Arensman - Pediatric Surgery

LANDES LANDES LANDES
BIOSCIENCE V ad e me c u m BIOSCIENCE BIOSCIENCE V ad e me c u m
V ad eme c um
Table of contents
Pediatric
(excerpt)
1. Preoperative Care 12. Inguinal Hernia and Hydrocele

2. Immediate Postoperative Care 13. Variocele
14. Testicular Torsion
Surgery
3. Anemia
4. Genetics and Prenatal 15. Cryptochidism:
Diagnosis in Pediatric Surgery The Undescended Testes (UDT)
5. Vascular Access 16. Circumcision
6. Fluids and Electrolytes 17. Hemangiomas and Vascular
Pediatric Surgery
7. Nutrition and Metabolism Malformations
8. Respiratory Failure 18. Branchial Cysts, Sinuses
and Support in Children and Fistulas
9. Hypovolemic Shock 19. Thyroglossal Duct Cyst
and Resuscitation and Sinus
10. Blood Component Therapy 20. Umbilical Anomalies
11. Perioperative Infections 21. Foreign Bodies
and Antibiotics of the Gastrointestinal Tract
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Arensman
Robert M. Arensman
Bambini
Almond
All titles available at I SBN 1- 57059- 499- 6
www.landesbioscience.com Daniel A. Bambini

P. Stephen Almond
v a d e m e c u m
Pediatric Surgery
Robert M. Arensman, M.D.

Northwestern University
Chicago, Illinois, U.S.A.
Daniel A. Bambini, M.D.

P. Stephen Almond, M.D.

LANDES
BIOSCIENCE
GEORGETOWN, TEXAS
U.S.A.
VADEMECUM
Pediatric Surgery
LANDES BIOSCIENCE
Georgetown, Texas U.S.A.
Copyright ©2000 Landes Bioscience

All rights reserved.
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Landes Bioscience, 810 S. Church Street, Georgetown, Texas, U.S.A. 78626
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ISBN: 1-57059-499-6
Library of Congress Cataloging-in-Publication Data
Arensman, Robert M.
Pediatric surgery / Robert M. Arensman, Daniel Bambini, P. Stephen
Almond.
p. ; cm. -- (Vademecum)
Includes bibliographical references and index.
ISBN 1-57059-499-6
1. Children--Surgery. I. Bambini, Daniel. II. Almond, P. Stephen. III.
Title. IV. Series.
[DNLM: 1. Surgical Procedures, Operative--Child. 2. Surgical
Procedures, Operative--Infant. WO 925 A681p 2000]
RD137.A74 2000
617.9´8--dc21 00--311658
While the authors, editors, sponsor and publisher believe that drug selection and dosage and
the specifications and usage of equipment and devices, as set forth in this book, are in accord
with current recommendations and practice at the time of publication, they make no
warranty, expressed or implied, with respect to material described in this book. In view of the
ongoing research, equipment development, changes in governmental regulations and the
rapid accumulation of information relating to the biomedical sciences, the reader is urged to
carefully review and evaluate the information provided herein.
Dedication
To children: whose endurance of suffering, whose
courage in the face of congenital malformations and
childhood cancer, and whose smiles over tears inspire
all who work with them to overcome childhood
maladies.
Contents
Section I: Assessment of the Pediatric Surgical Patient ........... 1
1. Preoperative Care .............................................................. 2
Robert M. Arensman
Consultation ............................................................................................... 2
Physical Examination .................................................................................. 2
Diagnostic Studies and Laboratory Investigations ....................................... 3
Pain Management ....................................................................................... 3
Blood Donation .......................................................................................... 4
Presurgical Visitation and Teaching ............................................................. 4
2. Immediate Postoperative Care........................................... 5
Daniel A. Bambini
Wound and Dressing Care .......................................................................... 5
Extubation and Transfer .............................................................................. 5
Postoperative Orders ................................................................................... 5
Pain Management ....................................................................................... 7
3. Anemia .............................................................................. 9
Robert M. Arensman and Lars Göran Friberg
Definition ................................................................................................... 9
Physiologic Anemia .................................................................................... 9
Iron Deficiency ........................................................................................... 9
Hereditary Spherocytosis ............................................................................ 9
Sickle Cell Anemia .................................................................................... 10
Other Anemias ......................................................................................... 10
4. Genetics and Prenatal Diagnosis in Pediatric Surgery..... 11
Lars Göran Friberg
Prenatal Diagnostic Studies and Tests ........................................................ 11
Indications for Prenatal Diagnosis ............................................................. 13
Section II: Perioperative Management and Critical Care...... 15
5. Vascular Access ................................................................ 16
Marleta Reynolds
Blood Sampling ........................................................................................ 16
Venous Access ........................................................................................... 16
Central Venous Access .............................................................................. 17
Umbilical Vessel Access ............................................................................. 17
Intraosseous Access ................................................................................... 17
6. Fluids and Electrolytes .................................................... 19
John R. Wesley
Fluids ........................................................................................................ 19
Electrolytes ............................................................................................... 20
Dehydration ............................................................................................. 22
7. Nutrition and Metabolism .............................................. 23
John R. Wesley
Introduction ............................................................................................. 23
Administering Parenteral Nutrition Solutions ........................................... 24
Monitoring ............................................................................................... 27
Complications .......................................................................................... 28
Pediatric Nutritional Assessment ............................................................... 30
Transition from Parenteral to Enteral Nutrition ........................................ 30
8. Respiratory Failure and Support in Children.................. 31
Marybeth Madonna
Novel Approaches for Respiratory Support in Children ............................ 33
9. Hypovolemic Shock and Resuscitation ........................... 35
Matthew L. Moront
Definition ................................................................................................. 35
Clinical Indicators of Inadequate Tissue Perfusion .................................... 35
Treatment of Shock ................................................................................... 36
10. Blood Component Therapy ............................................ 39
Richard Fox
Blood Component Preparation ................................................................. 39
Screening .................................................................................................. 39
Indications for Transfusion ........................................................................ 39
Transfusion Reactions ............................................................................... 41
11. Perioperative Infections and Antibiotics ......................... 43
Riccardo Superina
Classification and Incidence of Postoperative Wound Infection ................ 43
Etiology .................................................................................................... 43
Clinical Presentation ................................................................................. 44
Diagnosis .................................................................................................. 44
Treatment ................................................................................................. 45
Infection of a Central Venous Catheter or Port ......................................... 48
Yeast Infections ......................................................................................... 48
Summary .................................................................................................. 48
Section III: Common Pediatric Surgical Problems ................ 49
12. Inguinal Hernia and Hydrocele....................................... 50
Juda Z. Jona
Incidence .................................................................................................. 50
Etiology .................................................................................................... 50
Diagnosis .................................................................................................. 51
Treatment ................................................................................................. 52
Outcomes ................................................................................................. 52
Special Considerations .............................................................................. 52
13. Varicocele ........................................................................ 55
Juda Z. Jona
Incidence .................................................................................................. 55
Etiology .................................................................................................... 55
Pathophysiology ........................................................................................ 55
Treatment ................................................................................................. 56
Outcomes ................................................................................................. 56
14. Testicular Torsion ............................................................ 57
Juda Z. Jona
Incidence .................................................................................................. 57
Etiology .................................................................................................... 57
Diagnosis .................................................................................................. 58
Pathophysiology ........................................................................................ 58
Treatment ................................................................................................. 58
Outcomes ................................................................................................. 59
15. Cryptorchidism: The Undescended Testes (UDT) ........... 60
Juda Z. Jona
Incidence .................................................................................................. 60
Etiology .................................................................................................... 60
Treatment ................................................................................................. 61
Outcomes ................................................................................................. 62
16. Circumcision ................................................................... 63
Lars Göran Friberg and Juda Z. Jona
Male Circumcision ................................................................................... 63
Female Circumcision ................................................................................ 63
Phimosis ................................................................................................... 64
17. Hemangiomas and Vascular Malformations .................... 65
Maureen Sheehan and Daniel A. Bambini
Incidence .................................................................................................. 65
Etiology .................................................................................................... 65
Diagnosis .................................................................................................. 66
Pathology .................................................................................................. 67
Treatment ................................................................................................. 67
18. Branchial Cysts, Sinuses and Fistulas .............................. 69
Daniel A. Bambini
Incidence .................................................................................................. 69
Etiology .................................................................................................... 69
Diagnosis .................................................................................................. 70
Pathology/Pathophysiology ....................................................................... 70
Treatment ................................................................................................. 71
Outcome .................................................................................................. 71
19. Thyroglossal Duct Cyst and Sinus .................................. 72
Daniel A. Bambini
Incidence .................................................................................................. 72
Etiology .................................................................................................... 72
Diagnosis .................................................................................................. 74
Pathology .................................................................................................. 74
Treatment ................................................................................................. 74
Outcomes ................................................................................................. 74
20. Umbilical Anomalies ....................................................... 75
Daniel A. Bambini
Incidence .................................................................................................. 75
Etiology .................................................................................................... 75
Clinical Presentation, Diagnosis, and Treatment ....................................... 75
Outcomes ................................................................................................. 78
21. Foreign Bodies of the Gastrointestinal Tract ................... 79
John R. Wesley
Incidence .................................................................................................. 79
Etiology .................................................................................................... 79
Diagnosis .................................................................................................. 80
Treatment ................................................................................................. 80
Prevention ................................................................................................ 84
22. Hypertrophic Pyloric Stenosis ........................................ 85
Richard Fox and Daniel A. Bambini
Incidence .................................................................................................. 85
Etiology .................................................................................................... 85
Diagnosis .................................................................................................. 86
Pathology .................................................................................................. 86
Treatment ................................................................................................. 86
Outcomes ................................................................................................. 88
23. Intussusception ............................................................... 89
Vinh T. Lam
Incidence .................................................................................................. 89
Etiology .................................................................................................... 89
Pathology/Pathophysiology ....................................................................... 89
Diagnosis .................................................................................................. 91
Treatment ................................................................................................. 92
Outcomes ................................................................................................. 93
24. Disorders of the Spleen ................................................... 94
Harry T. Papaconstantinou and Dai H. Chung
Anomalies ................................................................................................. 94
Splenectomy ............................................................................................. 94
Hematologic Disorders ............................................................................. 95
Cysts and Tumors ..................................................................................... 97
Hypersplenism .......................................................................................... 97
Postsplenectomy Sepsis ............................................................................. 97
25. Rectal Prolapse and Anal Disorders ................................ 99
Steve Szczerba
Constipation ............................................................................................. 99
Rectal Prolapse ........................................................................................ 101
Anal Fissure ............................................................................................ 104
Perianal and Perirectal Abscess ................................................................ 105
Fistula-In-Ano ........................................................................................ 106
Hemorrhoids .......................................................................................... 106
Condyloma Acuminata ........................................................................... 107
Section IV: Pediatric Trauma .............................................. 108
26. Initial Assessment and Resuscitation............................. 109
Matthew L. Moront and Fawn C. Lewis
Organization ........................................................................................... 109
Primary Survey ....................................................................................... 110
Reassessment ........................................................................................... 113
Transition Phase ...................................................................................... 113
Secondary Survey .................................................................................... 113
Tertiary Survey ........................................................................................ 113
27. Soft Tissue and Extremity Trauma ................................ 115
Daniel A. Bambini and P. Stephen Almond
Incidence ................................................................................................ 115
Etiology .................................................................................................. 115
Clinical Presentation ............................................................................... 115
Diagnosis ................................................................................................ 115
Management ........................................................................................... 115
28. Facial Injuries ............................................................... 120
Bahram Ghaderi and Diane Dado
Incidence ................................................................................................ 120
Etiology .................................................................................................. 120
Diagnosis ................................................................................................ 121
Pathophysiology ...................................................................................... 121
Treatment ............................................................................................... 121
29. Head and Spinal Cord Injuries in Children .................. 123
David Bentrem
Head Injuries .......................................................................................... 123
Spinal Cord Injuries ................................................................................ 125
30. Abdominal Trauma ....................................................... 128
John Hijjawi and Daniel A. Bambini
Classification .......................................................................................... 128
Assessment .............................................................................................. 128
Diagnostic Evaluation ............................................................................. 129
Splenic Injuries ....................................................................................... 129
Liver Injuries .......................................................................................... 130
Pancreatic Injuries ................................................................................... 131
Intestinal Injuries .................................................................................... 131
31. Genitourinary Trauma .................................................. 132
Kate Abrahamsson and Fawn C. Lewis
Renal Trauma .......................................................................................... 132
Ureteral Trauma ...................................................................................... 133
Bladder Trauma ...................................................................................... 133
Urethral Trauma ..................................................................................... 133
Scrotal Trauma ........................................................................................ 134
Labial Trauma ......................................................................................... 134
Penile Trauma ......................................................................................... 135
Sexual Abuse ........................................................................................... 135
32. Thoracic Trauma ........................................................... 136
Introduction ........................................................................................... 136
Immediately Life Threatening Injuries .................................................... 136
Potentially Life Threatening Injuries ....................................................... 138
33. Vascular Injuries ............................................................ 141
Daniel A. Bambini
Incidence ................................................................................................ 141
Etiology .................................................................................................. 141
Diagnosis ................................................................................................ 142
Treatment ............................................................................................... 142
Outcomes ............................................................................................... 143
34. Burns............................................................................. 144
P. Stephen Almond and Heron E. Rodriguez
Incidence ................................................................................................ 144
Etiology .................................................................................................. 144
Management ........................................................................................... 145
Airway, Breathing, Circulation ................................................................ 145
Secondary Survey .................................................................................... 145
Fluid Resuscitation ................................................................................. 145
Burn Wound Care .................................................................................. 146
Nutrition ................................................................................................ 146
Pain Control ........................................................................................... 148
35. Bites and Stings ............................................................. 150
Heather Haukness and David Bentrem
Animal Bites ........................................................................................... 150
Snake Bites ............................................................................................. 151
Spider Bites ............................................................................................. 152
Stings ...................................................................................................... 152
36. Neonatal Trauma and Birth Injuries ............................. 153
Daniel A. Bambini
Incidence ................................................................................................ 153
Head Injuries .......................................................................................... 153
Spine and Cord Injuries .......................................................................... 155
Facial Fractures ....................................................................................... 155
Eye Injuries ............................................................................................. 155
Nerve Injuries ......................................................................................... 155
Pneumothorax ........................................................................................ 156
Abdominal Trauma ................................................................................. 157
Skeletal Fractures .................................................................................... 157
Iatrogenic Perforation of the Pharynx or Esophagus ................................ 157
37. Child Abuse .................................................................. 159
Definitions ............................................................................................. 159
Incidence ................................................................................................ 159
Diagnosis ................................................................................................ 160
Patterns of Injury .................................................................................... 160
Burns ...................................................................................................... 162
Section V: Pediatric Tumors................................................ 163
38. Renal Tumors ................................................................ 164
P. Stephen Almond
Incidence ................................................................................................ 164
Etiology .................................................................................................. 164
Diagnosis ................................................................................................ 165
Pathology ................................................................................................ 165
Staging .................................................................................................... 167
Treatment ............................................................................................... 167
Outcome ................................................................................................ 168
39. Neuroblastoma .............................................................. 169
Marybeth Madonna
Background and Etiology ........................................................................ 169
Pathology ................................................................................................ 169
Staging .................................................................................................... 171
Diagnosis ................................................................................................ 172
Treatment ............................................................................................... 173
Outcomes ............................................................................................... 174
40. Liver Tumors ................................................................. 177
P. Stephen Almond
Incidence ................................................................................................ 177
Etiology .................................................................................................. 177
Diagnostic Studies .................................................................................. 177
Pathology ................................................................................................ 178
Classification and Staging ....................................................................... 179
Treatment ............................................................................................... 179
Outcomes ............................................................................................... 179
41. Teratomas ...................................................................... 182
P. Stephen Almond
Incidence ................................................................................................ 182
Etiology .................................................................................................. 182
Sacrococcygeal Teratoma ......................................................................... 182
Ovarian Teratoma ................................................................................... 184
Retroperitoneal Teratomas ...................................................................... 185
Mediastinal Teratomas ............................................................................ 185
Head and Neck Teratomas ...................................................................... 185
Testicular Teratomas ............................................................................... 186
42. Ovarian Masses ............................................................. 188
Robert M. Arensman
Incidence ................................................................................................ 188
Etiology .................................................................................................. 188
Diagnosis ................................................................................................ 188
Treatment ............................................................................................... 189
43. Testicular Tumors .......................................................... 193
Daniel A. Bambini
Incidence ................................................................................................ 193
Etiology .................................................................................................. 193
Diagnosis ................................................................................................ 193
Pathology ................................................................................................ 194
Treatment ............................................................................................... 194
Outcomes ............................................................................................... 195
44. Gastrointestinal Tumors ................................................ 197
Ambrosio Hernandez and Dai H. Chung
Esophagus ............................................................................................... 197
Stomach .................................................................................................. 197
Small Intestine ........................................................................................ 198
Appendix ................................................................................................ 198
Large Intestine ........................................................................................ 199
45. Mediastinal Masses ....................................................... 201
Vicky L. Chappell and Dai H. Chung
Etiology and Embryology ....................................................................... 201
Diagnosis ................................................................................................ 203
Anterior and Superior Mediastinum ....................................................... 203
Middle Mediastinum .............................................................................. 204
Posterior Mediastinum ............................................................................ 204
Treatment ............................................................................................... 204
Outcome ................................................................................................ 205
46. Breast Lesions ............................................................... 206
Vinh T. Lam and Daniel A. Bambini
Breast Development ................................................................................ 206
Diagnostic Approaches to Breast Lesions ................................................ 206
Congenital Breast Anomalies .................................................................. 207
Gynecomastia ......................................................................................... 207
Breast Asymmetry ................................................................................... 208
Nipple Discharge .................................................................................... 208
Fibroadenoma ......................................................................................... 208
Breast Infection ....................................................................................... 209
Cystosarcoma Phylloides ......................................................................... 209
Breast Cancer .......................................................................................... 209
47. Hodgkin’s Lymphoma ................................................... 211
Lars Göran Friberg and Daniel A. Bambini
Incidence ................................................................................................ 211
Etiology .................................................................................................. 211
Diagnosis ................................................................................................ 212
Classification .......................................................................................... 212
Staging .................................................................................................... 212
Treatment ............................................................................................... 213
Outcomes ............................................................................................... 214
48. Non-Hodgkin’s Lymphoma ........................................... 215
Incidence ................................................................................................ 215
Etiology .................................................................................................. 215
Classification .......................................................................................... 215
Diagnosis ................................................................................................ 217
Staging .................................................................................................... 217
Treatment ............................................................................................... 217
Outcomes ............................................................................................... 219
49. Rhabdomyosarcoma and Other Soft Tissue Tumors ..... 220
Marleta Reynolds
Incidence ................................................................................................ 220
Diagnosis ................................................................................................ 220
Pathology ................................................................................................ 221
Treatment ............................................................................................... 221
Outcomes ............................................................................................... 221
Lipomatous Tumors ................................................................................ 221
Fibrous Tumors ....................................................................................... 222
50. Thyroid Masses ............................................................. 224
Robert M. Arensman
Incidence ................................................................................................ 224
Etiology .................................................................................................. 224
Diagnosis ................................................................................................ 224
Classification .......................................................................................... 225
Treatment ............................................................................................... 226
Outcomes ............................................................................................... 227
Section VI: Gastrointestinal Hemorrhage ............................ 228
51. Rectal Bleeding in Infancy ............................................ 229
Daniel A. Bambini
Incidence ................................................................................................ 229
Etiology .................................................................................................. 229
Diagnosis ................................................................................................ 231
Treatment ............................................................................................... 231
52. Polyps of the Gastrointestinal Tract .............................. 232
Riccardo Superina
Incidence ................................................................................................ 232
Etiology and Pathology ........................................................................... 232
Diagnosis ................................................................................................ 233
Classification .......................................................................................... 233
Treatment ............................................................................................... 235
53. Peptic Ulcer Disease and Gastritis................................. 237
Heron E. Rodriguez
Incidence ................................................................................................ 237
Diagnosis ................................................................................................ 239
Medical Treatment .................................................................................. 239
Surgical Treatment .................................................................................. 239
54. Portal Hypertension ...................................................... 241
Kimberly Brown
Anatomy and Physiology ........................................................................ 241
Etiology .................................................................................................. 241
Incidence ................................................................................................ 242
Diagnosis ................................................................................................ 242
Treatment ............................................................................................... 243
Outcome ................................................................................................ 244
55. Meckel’s Diverticulum .................................................. 245
Richard Fox
Incidence ................................................................................................ 245
Etiology .................................................................................................. 245
Diagnosis ................................................................................................ 247
Pathology/Pathophysiology ..................................................................... 247
Treatment ............................................................................................... 247
Outcomes ............................................................................................... 248
Section VII: Anomalies of the Gastrointestinal Tract ........... 249
56. Intestinal Obstruction in the Neonate .......................... 250
Daniel A. Bambini
Incidence ................................................................................................ 250
Etiology .................................................................................................. 250
Diagnosis ................................................................................................ 252
Treatment ............................................................................................... 253
Outcomes ............................................................................................... 253
57. Pyloric and Duodenal Obstruction ............................... 254
Daniel A. Bambini
Incidence ................................................................................................ 254
Etiology .................................................................................................. 254
Differential Diagnosis ............................................................................. 255
Diagnosis ................................................................................................ 255
Pathology and Classification ................................................................... 255
Treatment ............................................................................................... 258
Outcomes ............................................................................................... 259
58. Malrotation and Volvulus.............................................. 260
Vinh T. Lam
Incidence ................................................................................................ 260
Etiology .................................................................................................. 260
Classification .......................................................................................... 261
Diagnosis ................................................................................................ 261
Treatment ............................................................................................... 262
Outcomes ............................................................................................... 264
59. Atresia and Stenosis....................................................... 265
P. Stephen Almond
Incidence ................................................................................................ 265
Etiology .................................................................................................. 265
Diagnosis ................................................................................................ 266
Treatment ............................................................................................... 266
Outcomes ............................................................................................... 267
60. Meconium Ileus ............................................................ 268
Vinh T. Lam
Incidence ................................................................................................ 268
Etiology .................................................................................................. 268
Classification .......................................................................................... 269
Diagnosis ................................................................................................ 270
Treatment ............................................................................................... 270
Outcomes ............................................................................................... 271
61. Hirschsprung’s Disease ................................................. 272
Robert M. Arensman
Incidence ................................................................................................ 272
Etiology .................................................................................................. 272
Diagnosis ................................................................................................ 273
Treatment ............................................................................................... 274
62. Colonic Atresia .............................................................. 276
P. Stephen Almond
Incidence ................................................................................................ 276
Etiology .................................................................................................. 276
Diagnosis ................................................................................................ 276
Classification .......................................................................................... 276
Treatment ............................................................................................... 277
63. Gastrointestinal Duplications and Mesenteric Cysts ..... 278
Riccardo Superina and Daniel A. Bambini
Gastrointestinal Duplications .................................................................. 278
Mesenteric Cysts ..................................................................................... 281
Section VIII: Peritonitis in Infancy ..................................... 284
64. Necrotizing Enterocolitis .............................................. 285
Fawn C. Lewis
Incidence ................................................................................................ 285
Etiology .................................................................................................. 285
Classification .......................................................................................... 285
Diagnosis ................................................................................................ 287
Treatment ............................................................................................... 287
Outcomes ............................................................................................... 290
65. Gastrointestinal Perforation in the Newborn ................ 293
Daniel A. Bambini
Incidence ................................................................................................ 293
Etiology and Pathophysiology ................................................................. 293
Diagnosis ................................................................................................ 294
Treatment ............................................................................................... 294
Outcomes ............................................................................................... 294
66. Neonatal Ascites ............................................................ 295
Incidence ................................................................................................ 295
Urinary Ascites ....................................................................................... 295
Biliary Ascites ......................................................................................... 296
Chylous Ascites ....................................................................................... 297
Outcomes ............................................................................................... 298
Section IX: Jaundice in Infancy and Childhood ................. 299
67. Biliary Atresia ............................................................... 300
Riccardo Superina
Incidence ................................................................................................ 300
Etiology .................................................................................................. 300
Diagnosis ................................................................................................ 301
Pathology ................................................................................................ 301
Treatment ............................................................................................... 302
Outcomes ............................................................................................... 302
68. Choledochal Cysts......................................................... 304
Riccardo Superina
Incidence ................................................................................................ 304
Etiology .................................................................................................. 304
Diagnosis ................................................................................................ 305
Pathology ................................................................................................ 305
Classification .......................................................................................... 305
Treatment ............................................................................................... 307
Outcomes ............................................................................................... 307
Section X: Respiratory Distress .......................................... 309
69. Upper Airway Obstruction in the Newborn .................. 310
Daniel A. Bambini
Incidence ................................................................................................ 310
Etiology .................................................................................................. 310
Diagnosis ................................................................................................ 312
Treatment ............................................................................................... 312
70. Vascular Rings ............................................................... 314
Robert M. Arensman
Incidence ................................................................................................ 314
Etiology .................................................................................................. 314
Diagnosis ................................................................................................ 315
Treatment ............................................................................................... 315
71. Tracheoesophageal Fistula and Esophageal Atresia ....... 318
Daniel A. Bambini
Incidence ................................................................................................ 318
Etiology .................................................................................................. 318
Classification .......................................................................................... 318
Diagnosis ................................................................................................ 321
Treatment ............................................................................................... 321
Outcomes ............................................................................................... 322
72. Diaphragmatic Anomalies ............................................. 325
Daniel A. Bambini
Incidence ................................................................................................ 325
Etiology .................................................................................................. 325
Diagnosis ................................................................................................ 326
Treatment ............................................................................................... 330
Outcomes ............................................................................................... 331
73. Congenital Malformations of the Lung ......................... 333
Marleta Reynolds
Congenital Lobar Emphysema ................................................................ 333
Pulmonary Sequestration ........................................................................ 333
Congenital Cystic Adenomatoid Malformation (CCAM) ....................... 335
Bronchogenic Cyst .................................................................................. 337
74. Foreign Bodies in the Air Passages and Esophagus ....... 339
Marleta Reynolds
Foreign Bodies in the Esophagus ............................................................. 339
Foreign Bodies of the Air Passages ........................................................... 341
75. Chylothorax and Diseases of the Pleura ........................ 344
Chylothorax ............................................................................................ 344
Empyema ............................................................................................... 348
Spontaneous Pneumothorax .................................................................... 349
76. Patent Ductus Arteriosus .............................................. 352
Samer Kanaan and Daniel A. Bambini
Incidence ................................................................................................ 352
Etiology .................................................................................................. 352
Diagnosis ................................................................................................ 354
Treatment ............................................................................................... 354
Outcomes ............................................................................................... 354
Section XI: Congenital Malformations of the Chest Wall,
Abdominal Wall and Perineum ..................................... 356
77. Chest Wall Deformities ................................................. 357
Marleta Reynolds
Pectus Excavatum ................................................................................... 357
Pectus Carinatum ................................................................................... 359
Poland’s Syndrome .................................................................................. 359
Sternal Clefts and Ectopia Cordis ........................................................... 359
78. Abdominal Wall Defects ................................................ 361
Grant H. Geissler
Incidence ................................................................................................ 361
Omphalocele .......................................................................................... 361
Treatment ............................................................................................... 363
Outcomes ............................................................................................... 365
79. Anorectal Malformations .............................................. 366
P. Stephen Almond
Incidence ................................................................................................ 366
Etiology .................................................................................................. 366
Classification .......................................................................................... 366
Diagnosis ................................................................................................ 367
Treatment ............................................................................................... 367
Outcomes ............................................................................................... 371
80. Urogenital Sinus, Cloaca, and Cloacal Exstrophy ......... 372
Robert M. Arensman
Definitions ............................................................................................. 372
Embryogenesis ........................................................................................ 372
Incidence ................................................................................................ 372
Associated Anomalies .............................................................................. 372
Diagnosis ................................................................................................ 373
Treatment ............................................................................................... 374
Results .................................................................................................... 374
Section XII: Functional and Acquired Disorders
of the Esophagus ........................................................... 375
81. Gastroesophageal Reflux ............................................... 376
Grant H. Geissler
Introduction and Incidence ..................................................................... 376
Embryology and Anatomy ...................................................................... 376
Diagnostic Studies .................................................................................. 377
Medical Therapy ..................................................................................... 378
Surgical Therapy and Long-Term Results ................................................ 378
82. Achalasia ....................................................................... 380
David Bentrem
Incidence ................................................................................................ 380
Etiology .................................................................................................. 380
Diagnosis ................................................................................................ 381
Treatment ............................................................................................... 381
83. Caustic Esophageal Injury and Perforation ................... 383
Christina L. Dial and Daniel A. Bambini
Incidence ................................................................................................ 383
Etiology .................................................................................................. 383
Classification and Pathophysiology ......................................................... 384
Treatment ............................................................................................... 384
Outcomes ............................................................................................... 385
Section XIII: Gastrointestinal Diseases
of the Older Child......................................................... 387
84. Appendicitis .................................................................. 388
Steve Szczerba
Incidence ................................................................................................ 388
Etiology .................................................................................................. 388
Diagnosis ................................................................................................ 390
Treatment ............................................................................................... 391
Outcomes ............................................................................................... 392
85. Adhesive Intestinal Obstruction .................................... 393
Todd R. Vogel
Incidence ................................................................................................ 393
Etiology .................................................................................................. 393
Diagnosis ................................................................................................ 393
Treatment ............................................................................................... 394
86. Gallbladder Disease in Childhood ................................ 395
Fawn C. Lewis
Incidence ................................................................................................ 395
Etiology .................................................................................................. 395
Diagnosis ................................................................................................ 396
Treatment ............................................................................................... 398
Outcomes ............................................................................................... 398
87. Superior Mesenteric Artery (SMA) Syndrome ............... 400
Todd R. Vogel
Incidence ................................................................................................ 400
Etiology .................................................................................................. 400
Diagnosis ................................................................................................ 400
Treatment ............................................................................................... 401
88. Inflammatory Bowel Disease ........................................ 402
Christopher Mascio and Daniel A. Bambini
Incidence and Etiology ........................................................................... 402
Ulcerative Colitis .................................................................................... 402
Crohn’s Disease ....................................................................................... 405
89. Disorders of the Pancreas .............................................. 408
Todd R. Vogel
Pancreatic Embryology and Anatomy ..................................................... 408
Acute Pancreatitis .................................................................................... 408
Chronic Relapsing Pancreatitis ................................................................ 410
Pancreatic Cysts ...................................................................................... 410
Congenital Pancreatic Abnormalities ...................................................... 411
Pancreatic Neoplasms ............................................................................. 412
Section XIV: Endocrine Disorders ...................................... 414
90. Pheochromocytoma ...................................................... 415
Richard Fox
Incidence ................................................................................................ 415
Diagnosis ................................................................................................ 416
Treatment ............................................................................................... 417
Outcomes ............................................................................................... 419
91. Hyperparathyroidism .................................................... 420
P. Stephen Almond
Incidence ................................................................................................ 420
Etiology .................................................................................................. 420
Diagnosis ................................................................................................ 421
Treatment ............................................................................................... 421
Outcomes ............................................................................................... 422
92. Neonatal Hypoglycemia ................................................ 424
Daniel A. Bambini
Incidence ................................................................................................ 424
Etiology .................................................................................................. 424
Diagnosis ................................................................................................ 426
Treatment ............................................................................................... 426
Outcomes ............................................................................................... 427
93. Intersex ......................................................................... 428
Daniel A. Bambini
Incidence ................................................................................................ 428
Development of the Gonads and Genitalia ............................................. 428
Classification and Etiology ...................................................................... 429
Diagnosis ................................................................................................ 431
Treatment ............................................................................................... 431
Outcomes ............................................................................................... 433
Section XV: Miscellaneous Pediatric Surgical Topics .......... 434
94. Short Bowel Syndrome ................................................. 435
Fawn C. Lewis and Daniel A. Bambini
Incidence and Etiology ........................................................................... 435
Initial Treatment ..................................................................................... 437
Late Treatment ........................................................................................ 437
Outcomes ............................................................................................... 438
95. Conjoined Twins ........................................................... 439
Robert M. Arensman
Incidence ................................................................................................ 439
Etiology .................................................................................................. 439
Classification .......................................................................................... 439
Diagnosis ................................................................................................ 440
Treatment ............................................................................................... 440
Outcomes ............................................................................................... 440
96. Minimally Invasive Pediatric Surgery ............................ 443
Indications .............................................................................................. 443
Equipment .............................................................................................. 443
General Considerations ........................................................................... 444
Appendectomy ........................................................................................ 445
Fundoplication and Gastrostomy ............................................................ 445
Splenectomy ........................................................................................... 446
Pyloromyotomy ...................................................................................... 446
Contralateral Inguinal Exploration .......................................................... 447
Nonpalpable Testis .................................................................................. 447
Pull-Through for Hirschsprung’s Disease ................................................ 447
Thoracoscopy ......................................................................................... 448
Summary ................................................................................................ 449
Appendix I: Common Drugs and Dosages Used
in Pediatric Surgical Patients ........................................ 450
Index ............................................................................. 459
Editors
Robert M. Arensman, M.D.
Surgeon-in-Chief
Division of Pediatric Surgery
Lydia J. Fredrickson Professor of Pediatric Surgery
Children's Memorial Hospital
Chapters 1, 3, 42, 50, 61, 70, 80, 95
Daniel A. Bambini, M.D.

Fellow in Pediatric Surgery
Chapters 2, 17-20, 22, 27, 30, 33, 36, 43, 46-48,
51, 53, 56, 57, 63, 65, 66, 69, 71, 72, 76, 83, 88, 92-94
P. Stephen Almond, M.D.

Assistant Professor of Surgery
Director of Trauma
Divisions of Pediatric Surgery and Transplantation
Chapters 27, 34, 38, 40, 41, 59, 62, 79, 91
Contributors
Kate Abrahamsson Kimberly Brown
Divisiionen för barn-och Department of General Surgery
ungdomssjukvård Kirurgi Loyola University Medical Center
Queen Silvia Children's Hospital Maywood, Illinois, U.S.A.
Sahlgrenska Universitetssjukhuset/Östra Chapter 54
Göteborg, Sweden
Chapter 31 Vicky L. Chappell
Department of Surgery
David Bentrem University of Texas
Department of General Surgery Medical Branch at Galveston
Northwestern Memorial Hospital Galveston, Texas, U.S.A.
Northwestern University Chapter 45
Chapters 29, 35, 82
Dai H. Chung Ambrosio Hernandez
Division of Pediatric Surgery Department of Surgery
University of Texas University of Texas
Medical Branch at Galveston Medical Branch at Galveston
Galveston, Texas, U.S.A. Galveston, Texas, U.S.A.
Chapters 24, 44, 45, 75, 96 Chapter 44
Diane Dado John Hijjawi

Division of Plastic Surgery Department of Plastic Surgery
Loyola University Medical Center Northwestern Memorial Hospital
Maywood, Illinois, U.S.A. Northwestern University
Chapter 28 Chicago, Illinois, U.S.A.
Chapter 30
Christina L. Dial
Department of General Surgery Juda Z. Jona
St. James Hospital and Medical Center Division of Pediatric Surgery
Midwestern University Evanston Hospital
Olympia Fields, Illinois, U.S.A. Evanston, Illinois, U.S.A.
Chapter 83 Chapters 12-16
Richard Fox Samer Kanaan

Office of Graduate Medical Education Department of General Surgery
Loyola University Medical Center Northwestern Memorial Hospital
Maywood, Illinois, U.S.A. Northwestern University
Chapters 10, 22, 55, 90 Chicago, Illinois, U.S.A.
Chapter 76
Lars Göran Friberg
Divisiionen för barn-och Vinh T. Lam
ungdomssjukvård Kirurgi Children's Surgical Associates
Queen Silvia Children's Hospital Orange, California, U.S.A.
Sahlgrenska Universitetssjukhuset/Östra Chapters 23, 46, 58, 60, 66
Göteborg, Sweden
Chapters 3, 4, 16, 47, 48 Fawn C. Lewis
Grant Geissler Children's Memorial Hospital
Department of Pediatric Surgery Chicago, Illinois, U.S.A.
Children's Memorial Hospital Chapters 31, 37, 64, 86, 94
Chicago, Illinois, U.S.A. Marybeth Madonna
Chapters 78, 81 Division of Pediatric Surgery
Bahram Ghaderi Chicago, Illinois, U.S.A.
Division of Plastic Surgery Chapters 8, 39
Loyola University Medical Center
Maywood, Illinois, U.S.A. Christopher Mascio
Chapter 28 Department of General Surgery
Loyola University Medical Center
Heather Haukness Loyola University
Department of Pediatrics Maywood, Illinois, U.S.A.
Children's Memorial Hospital Chapter 88
Chapter 35
Matthew L. Moront Riccardo Superina
Specialty Surgeons of Pittsburgh Division of Pediatric Surgery
Pittsburgh, Pennsylvania, U.S.A. Children's Memorial Hospital
Chapters 9, 26, 32, 37, 94 Chicago, Illinois, U.S.A.
Chapters 11, 52, 63, 67, 68
Harry T. Papaconstantinou
Department of Surgery Steve Szczerba
University of Texas Department of General Surgery
Medical Branch at Galveston Northwestern Memorial Hospital
Galveston, Texas, U.S.A. Northwestern University
Chapters 24, 75, 96 Chicago, Illinois, U.S.A.
Chapters 25, 84
Marleta Reynolds
Division of Pediatric Surgery Todd R. Vogel
Children's Memorial Hospital Department of General Surgery
Chicago, Illinois, U.S.A. Robert Wood Johnson University Hospital
Chapters 5, 49, 73, 74, 77 University of Medicine and Dentistry
of New Jersey
Heron E. Rodriguez New Brunswick, New Jersey, U.S.A.
Catholic Health Partners Chapters 85, 87, 89
Columbus Hospital
Chicago, Illinois, U.S.A. John R. Wesley
Chapters 34, 53 Medical Director and Vice President
of Medical and Professional Affairs
Maureen Sheehan Baxter Healthcare
Department of General Surgery Roundlake, Illinois, U.S.A.
Loyola University Medical Center Chapters 6, 7, 21
Loyola University
Maywood, Illinois, U.S.A.
Chapter 17
Preface
This modest manual of pediatric surgery has been prepared as a

ready reference for information on the common surgical problems of
childhood.
It represents basic information that is reasonably known or
proven with little if any theory or speculation. It is intended to pro-
vide information needed to diagnose, to choose diagnostic studies or
to begin treatment.
The information contained herein is a point of departure that
leads on to the further study of problems or conditions that afflict
our children.
The Editors
Chicago, Illinois
Section I: Assessment of the Pediatric
Surgical Patient
CHAPTER 1
Preoperative Care
Robert M. Arensman
Consultation
Prior to surgery for any patient, surgical consultation occurs. This provides a
meeting and introduction between child and surgeon and proceeds to a complete
history and physical examination. Since many children meet a surgeon for the first
time on referral, the results of a prior history and physical examination are often
available. If that is the case the previous findings are reviewed, verified, and further
information is sought that may elucidate the diagnosis.
The meeting may be brief but creates the foundation for further interaction
between surgeon and child. It is certainly an opportunity for the surgeon to create a
friendship, or at least trust, between a frightened child and the person who will
ultimately perform surgery. This meeting also is the chance to create communica-
tion and trust with anxious parents. Consequently, child and parents must be given
adequate opportunity to present their understanding of the diagnosis, raise ques-
tions concerning surgery and in-hospital care, and discuss worrisome questions such
as pain control, postoperative management, ultimate outcome and long-term results.
If a crowded clinic schedule precludes adequate time to cover all aspects of
anticipated surgery, it is quite appropriate to schedule further visits or simply to
arrange time for phone conferences with all concerned. Frequently, the surgeon can
include other significant family members (grandparents, aunts, and uncles, siblings)
by arranging for evening phone conversations.
Since many presurgical patients have already undergone diagnostic testing, it is
important to review these tests and share the surgeon’s interpretation with the child
and family. Sometimes this entails consultation with other specialists at the children’s
hospital (radiologist, pathologists, and pediatric subspecialists). The results of these
consultations will generally not be available at the time of the surgical consultation,
but the results or discussion can easily be shared with child and family members via
phone, e-mail, or fax. Communication at present is one of the easier, but most
important, aspects of patient care.
Physical Examination
Abnormal findings on physical examination are often reported to a surgeon prior
to the patient encounter. This does not preclude another examination at the time of
the consultation visit. Additional findings may be demonstrated, and certainly one
wishes to confirm the previously reported findings. Such simple matters as hernias
or hydroceles are often confused and need the careful reexamination of the pediatric
Pediatric Surgery, edited by Robert M. Arensman, Daniel A. Bambini, and P. Stephen Almond.
©2000 Landes Bioscience.
Preoperative Care 3
surgeon to clarify. In addition, associated findings, well known to the pediatric sur-
geon, may not be common knowledge to the referring pediatrician or family practi-
tioner. Therefore, a good physical examination is always advisable prior to surgical 1
intervention.
Diagnostic Studies and Laboratory Investigations
The need for diagnostic studies varies from none to extensive. In the case of a
child with a reducible inguinal hernia, a good, but simple, physical examination
constitutes the best diagnostic study. Further tests, radiographs, blood examina-
tions, etc. are invasive, bothersome, expensive and unwarranted unless there are
other findings or complaints. However, the child who presents with severe, recur-
rent abdominal pain without any physical findings may need extensive studies to
demonstrate the causative pathology (or lack thereof ). Suffice to say, diagnostic studies
are chosen and done that are needed to completely and safely make a diagnosis and
sufficient to advise a child and family concerning the need for surgical intervention.
It is clear that a healthy child on a standard diet requires nothing as far as preop-
erative testing if the surgical problem is simple, can be done under outpatient gen-
eral anesthetic without hospital stay. For example, a two-year-old child with
uncomplicated bilateral inguinal hernias, eating until a few hours before surgery,
whose cheeks and lips betray no sign of anemia can and should undergo operative
repair without diagnostic testing. Careful questioning of the family history adequately
excludes inherited diseases and bleeding dyscrasias. Examination of the child pro-
vides all the further information needed to make a correct decision concerning the
need for further tests. In contrast, a two-year-old child with previous diagnosis of
biliary atresia with unsuccessful Kasai procedure and progressive biliary cirrhosis
clearly needs a very complicated and extensive diagnostic evaluation to allow a
determination as to whether he can undergo hepatic transplantation.
In summary, the diagnostic regimen is designed to be sufficiently brief or thor-
ough to correctly and adequately identify the surgical problem(s) and formulate the
best and safest surgical plan.
Pain Management
One of the greatest concerns for child or parents when approaching a surgical
event is the problem of postoperative pain control. Most children are not particu-
larly concerned about the technical details of the surgery they will undergo, but they
are greatly fearful of the pain that they endure in the postoperative period. Knowl-
edge that this can be controlled in a variety of ways provides some comfort. Knowl-
edge that they will also be in the company of their parents during this period of time
is also vitally important.
Consequently, the consultation visit or phone conferences include a thorough
discussion of postoperative pain management. Commonly used methods of pain
control include intraoperative local anesthetic administration, intravenous narcot-
ics, patient controlled analgesia, caudal blocks, epidural blocks and continuous epi-
dural anesthesia. Although these can all be discussed before the surgical event, it is
generally best to provide at least one to two hours in the preanesthetic room so this
can be discussed a second time with the anesthesia staff. This is the time of the final
decision concerning the exact pain control methods to be used. Since this is often
4 Pediatric Surgery
tailored to fit the anesthesia used during the operative event, the anesthesiologist is
included in this decision.
1 Blood Donation
Due to the tremendous information on the hazards of blood transfusion, most
parents want to discuss possible transfusion thoroughly. Since transfusion is a rare
event, discussion can be limited to acknowledgement that transfusion is most unlikely,
and so much so that blood is not routinely prepared for the operation anticipated.
In the event that transfusion is a possibility, discussion centers on the use of banked
blood versus donor directed blood. This is both a controversial and emotional sub-
ject so it is sometimes necessary to involve the director of the blood bank service to
fully elucidate the questions posed. Parents must fully understand that blood samples
will be necessary from child and donors prior to the surgical date. Furthermore, they
need to fully understand that all donor directed blood is subjected to the same
testing required for all other blood donations. Finally, parents need to understand
that type match does not necessarily predict cross match and that fulfillment of all
these requirements requires adequate time before the surgery date.
Presurgical Visitation and Teaching
Most children’s hospitals provide a presurgical visitation and teaching program.
These programs allow children to visit all portions of the operative suite prior to
surgery. They become familiar with the holding area, the operating room, and the
postanesthesia recovery area. They have an opportunity to try on “scrubs”, gowns,
masks and caps. The nurses from the various areas answer questions, reassure the
children of their parent’s nearness and participation in the entire process, and par-
ticularly address concerns about postoperative pain. In our particular hospital, the
children conclude the visit by making a mural that is taped to the entry hall wall. As
the children pass to the operating rooms, they can look for their previous artwork.
We hope it lessens their anxiety and certainly endorse the use of these programs if
available.
Selected Readings
1. Puri P, Sweed Y. Preoperative assessment. In: Puri P, ed. Newborn Surgery. Oxford:
Butterworth-Heineman 1996; 41–51.
2. Albanese CT, Rowe MI. Preoperative and postoperative management of the neo-
nate. In: Spitz L, Coran AG, eds. Operative Surgery. London: Butterworth 1995;
5-12.
CHAPTER 2
Immediate Postoperative Care

Daniel A. Bambini
The postoperative care of surgical neonates and children begins upon comple-
tion of wound closure. The level of postoperative care administered is dependent
upon the procedure performed but some general guidelines are provided below.
Specific guidelines for postoperative management of many pediatric surgical condi-
tions are provided throughout this handbook.
Wound and Dressing Care
Prior to the removal of the sterile surgical drapes, the skin surrounding the surgi-
cal wound is cleansed with warm saline-soaked sponges or lap pads to remove any
debris, blood, or prep solutions surrounding the wound. The area is gently padded
dry and a sterile towel or dressing is placed over the wound to prevent contamina-
tion at the time of drape removal. The type of dressing applied to surgical wounds is
selected according to surgeon preference, the type of wound created, and the method
of closure. For clean procedures, a dry, sterile dressing (i.e., gauze, steristrips, Opsite®,
Tegrederm®) is suitable. Antibiotic ointments and other wound applicants are gen-
erally not necessary. To minimize the stress and pain of later dressing removal, dress-
ings are secured in position with the minimal amount of tape or occlusive barrier
that achieves coverage of the wound.
Extubation and Transfer
Intraoperative monitoring devices should be left in place until after extubation.
A physician member of the surgical team should be present at the time of extubation
and assist in the transfer of the pediatric surgical patient to the postanesthesia care
unit or appropriate intensive care unit. If respiratory rate or inspiratory tidal vol-
umes are inadequate, the child should be observed in the OR until breathing has
improved. Special attention to body temperature and measures to prevent hypoth-
ermia after drape removal should be instituted including infrared heating lights,
wrapping with warm blankets, and increasing the ambient room temperature.
Postoperative Orders
The postoperative orders are individualized for each patient. In general, outpa-
tient procedures will require only simple postoperative care and specific wound care
instructions for the parents. Arrangements for office follow-up visits are discussed.
A general outline for writing postoperative orders in postsurgical pediatric patients
is provided below.
6 Pediatric Surgery
Admission Order
Specific information regarding the type of bed and/or location within the hospi-
tal to which the patient goes after recovery is listed. Arrangements for intensive care
unit beds are made preoperatively. If observation status or discharge from the recov-
ery unit is desired, specific instructions regarding wounds, medications, and antici-
2 pated clinical course/problems are provided to the parents or primary caregiver.
Attending Physician and Consultants
List the attending physician and all consultants who will participate in the care
of the patient. In addition, one specifies which physician(s) and/or service(s) will be
the primary providers of postoperative care and orders. The nursing staff must be
clearly informed regarding who is contacted for questions about care and for any
problems that arise.
Diagnosis
List the primary diagnosis and/or the procedure that has been performed.
Allergies
List any known drug allergies or other sensitivities (i.e., latex, tape, antibiotics,
pain medications, etc.)
Admission Weight
The patient’s preoperative weight is specified. This is the weight that is used to
calculate medication dosages, fluids, nutritional requirements, etc.
Vital Signs
Provide instructions for the frequency at which vital signs are monitored and
recorded. Parameters for changes in vital signs that require notification of the surgi-
cal team are clearly specified.
Monitoring Equipment
List any special monitoring devices that are appropriate for postoperative care
including pulse oximetry, apnea and/or cardiac monitors, etc.
Ventilator Settings and Respiratory Care
For patients requiring postoperative ventilatory support, specific instructions
regarding ventilator mode, tidal volume, peak inspiratory pressure, inspired oxygen
concentration, etc. are provided. If other respiratory interventions (i.e., nebulizers,
chest physiotherapy, frequent suctioning) are required, specific written orders are
made.
Intravenous Fluids
Maintenance and replacement fluid orders are provided. Specific information
regarding postoperative fluid and electrolyte management is provided in Chapter 7.
Diet
Special diets (i.e., clear liquids, general diet) or oral restriction (i.e., NPO) are
specified, including orders for initiation of enteral tube feedings when applicable.
Immediate Postoperative Care 7
Activity
Level of activity and/or restriction (i.e., bedrest, ambulation, etc.) is specified.
Physical therapy may be helpful to some hospitalized patients and is initiated when
appropriate.
Medications 2
All medications including doses, routes of administration, and frequencies of
administration are recorded clearly and accurately. Analgesic and antiemetic medi-
cations are ordered when appropriate. Doses are calculated on a per weight basis.
Wound Care
Special instructions for dressing care or surgical wounds are provided when
applicable.
Drains
Drain care orders include specific requests for suction, stripping, frequency of
emptying, and quantification of output. Nasogastric tubes are placed to suction or
gravity drainage according to attending surgeon preference. Foley catheters are placed
to gravity drainage.
Special Studies
Any radiographic exams or follow-up studies are specified, and the radiology
department and/or attending radiologist should be notified of all requests. Chest
radiographs are obtained in the recovery room or intensive care unit for all patients
who remain intubated or who had intraoperative placement of central venous lines
or catheters.
Laboratory Tests
Routine laboratory testing is often not necessary in pediatric surgical patients,
especially those who have procedures in the surgicenter and are discharged shortly
after surgery. Specific laboratory studies are obtained if the results are expected to
alter clinical management of the patient. Laboratory tests are often indicated in
children who undergo extensive and complicated procedures.
Pain Management
Achieving adequate pain relief is important in children, although children often
do not or cannot complain specifically of pain. Pain may adversely affect recovery of
infants since painful stimuli may result in decreased arterial saturation and increased
pulmonary vascular resistance. Effective pain control allows earlier ambulation and
faster recovery in older children.
Local anesthetics administered in the operating room can provide prolonged
pain control. Local wound infiltration or regional nerve blocks with bupivicaine
(Sensorcaine®) provide pain control for 4-6 hours following an operation. The
maximum dose is 3 mg/kg given as a 0.25–0.75% solution.
For larger operations, intravenous narcotics provide excellent pain control. Lib-
eral use of patient controlled analgesia devices and epidural catheters improve post-
operative pain control after many abdominal or thoracic operations.
8 Pediatric Surgery
Selected Readings
1. Filston HC, Izant, Jr. RJ. The surgical neonate: Evaluation and care, 2nd edition.
Norwalk, CT: Appleton-Century-Crofts 1985.
2 Raffensperger JG. Immediate postoperative care. In: Raffensperger JG ed. Swenson’s
Pediatric Surgery, 5th edition. Norwalk, CT: Appleton & Lange 1990; 27–28.
2
CHAPTER 3
Anemia
Robert M. Arensman and Lars Göran Friberg
Unlike many Chapters of this handbook that deal with a specific surgical
condition, this short Chapter touches on a physiologic state that has great importance
to the surgeon. Anemia denotes a state in which a patient has less than normal
hemoglobin. In this situation, decreased oxygen transport may decrease wound
healing, may increase cardiac stress during or after surgical event, and may predispose
to a variety of postoperative complications. Fortunately, all these anemia problems
are less likely in the pediatric patient, but still one must consider carefully the presence
of anemia, its probable cause, whether it should be corrected (how and how quickly),
and its chance of seriously affecting surgical outcome.
Definition
Generally, anemia is defined as hemoglobin less than 10 grams/deciliter. The
normal value for adults and older children is 12-16 grams/deciliter. However, this
value may be higher in the newborn and will characteristically fall below this normal
range during the first 1-2 months of life.
Physiologic Anemia
Babies rapidly lower their hemoglobin in the neonatal period. Values often fall
to the 9-10 g/dl level with corresponding hematocrits of 25-30%. This change is
normal and reflects a slow initiation of hematopoesis by the neonatal bone marrow.
If surgery is necessary during this period, the surgical and anesthesiological staff
must decide whether the transfusion of blood outweighs risks of transfusion and
delay of hematopoesis onset.
Iron Deficiency
Iron supplies are transferred to a neonate late in intrauterine life. These supplies
may be low in preterm children, just as the supply of other nutrients, vitamins,
minerals are low in early children. If there is no compelling reason to correct the
anemia quickly, the infant is given iron orally. This is absorbed in the duodenum
and proximal jejunum and nicely corrects the problem. Parental iron or transfusion
are the alternatives if this deficiency must be corrected quickly.
Hereditary Spherocytosis
This is an autosomal dominant disease process that prevents red cells from
assuming their characteristic biconcave shape. The elliptical red blood cells do not
move easily through the capillary bed or the pulp of the spleen. Red cells thus
10 Pediatric Surgery
entrapped are more rapidly destroyed, resulting in splenomegaly, jaundice, and

anemia. The presence of a family history consistent with this disease and the
observation of spherocytes and reticulocytes on a peripheral blood smear confirm
the diagnosis. Further confirmation involves demonstration of increased cellular
fragility in the osmotic fragility test. These children are highly prone to the
development of gallstones and concomitant biliary tract disease. Thus, they need
full evaluation of those structures if they are coming to splenectomy to control the
3 spherocytosis.
Sickle Cell Anemia
This is the most common inherited disorder of the African American population.
Up to 10% of this population is affected. This disease is an autosomal recessive trait
and requires the homozygous state for expression of the full-blown disease. Most of
these patients have anemia, leukocytosis, jaundice, and perhaps splenomegaly early.
By teenage years, the spleen usually shrinks from progressive infarction and fibrosis.
However, these children by then often also have biliary stones and biliary tract disease.
In severe forms of this disease, children have painful crises that involve bone
pain, severe right and left upper abdominal pain, strokes, and pulmonary infarctions.
Many of these children develop osteomyelitis and leg ulcers.
A peripheral smear demonstrates sickle shaped red blood cells, especially when
crisis is occurring. However, today most of these children are quickly diagnosed at
the time of birth through mandated state screening programs. Hemoglobin
electrophoresis confirms the presence of hemoglobin S and indicates the zygosity.
Prenatal diagnosis is possible through amniocentesis and DNA analysis.
Although surgeons are not generally asked to manage children with this disease,
they are frequently asked to consult for abdominal pain. When surgery is necessary
for appendicitis, biliary problems, etc., it is important that the surgeon know how to
manage these children to optimize outcome. Preoperative suppressive transfusions,
exchange transfusions, meticulous hydration and prevention of hypoxia all are
important aspects of preoperative, intraoperative, and postoperative care.
Other Anemias
Diverse other anemic states more rarely come to the attention of pediatric
surgeons. Generally, the request is to assist with a complication of the anemia, most
often splenomegaly or biliary complications such as cholelithiasis. Care should be
used to correct the anemia to the degree possible before operation. If this is not
possible, the surgeon must try to optimize care to prevent postoperative complications
associated with low red blood volume and decreased oxygen transport.
Selected Readings
1. In: Behrman LE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics.
Philadelphia: W.B. Saunders Company, 16th edition, Chapters 452-471. 2000;
481-490.
2. Oski FA. The erythrocyte and its disorders. In: Nathan DG, Oski FA, eds. Hema-
tology of Infancy and Childhood, 3rd edition. Philadelphia: W.B. Saunders 1987.
3. Seeler RA, Shwiaki MZ. Acute splenic sequestration crises (ASSC) in young chil-
dren with sickle cell anemia. Clin Pediatr 1972; 11:701.
4. Rennels MB et al. Cholelithiasis in patients with major sickle hemoglobin-opathies.
Am J Dis Child 1983; 138:66.
CHAPTER 4
Genetics and Prenatal Diagnosis

in Pediatric Surgery
Lars Göran Friberg
Serious malformations occur in 1.5% of all births. For half of these children the
etiology is unknown; the other half have a documented genetic or teratogenic cause.
The importance of prenatal diagnosis lies in its ability to give parents several options.
If there are malformations or conditions incompatible with life, the pregnancy can
be terminated. If the malformation is correctable at term, surgery can take place
after delivery. For some malformations, progressive adverse effects in utero may
warrant consideration of early delivery or fetal surgery.
Modern obstetric care includes close monitoring during pregnancy and various
prenatal screening-tests, such as alpha-fetoprotein screening and prenatal ultrasonog-
raphy. Fetal ultrasonography is routinely performed around the 18th gestational
week. Occasionally, there are indications for more invasive screening tests, such as
chorionic villus sampling, amniocentesis, fetoscopy, fetal sampling, and percutane-
ous umbilical blood sampling.
Early detection of congenital defects in utero allows for both parental counseling
and referral to a perinatal center where further investigation of the condition can be
undertaken and monitoring of the high-risk pregnancy can be done. The perinatal
center should advise when, where and how delivery will take place. Appropriate
centers must have a skilled neonatal intensive care facility and pediatric surgical
expertise.
Prenatal Diagnostic Studies and Tests
Chorionic Villus Sampling (CVS)
CVS allows biopsy of fetal cells for chromosomal, enzymatic or DNA analysis in
the first trimester (9-12 weeks gestation). Cells are obtained by direct biopsy of
chorion, either transcervically or transabdominally, preferably under ultrasound
guidance The major disadvantage of this procedure is the associated 3% increased
rate of spontaneous abortion. CVS is the preferred prenatal diagnostic test for many
high risk conditions (i.e., cystic fibrosis, sickle cell disease, Duchenne’s dystrophy, etc.)
Alpha-Fetoprotein (AFP) Screening
AFP is one of the major proteins in fetal serum. Screening should be offered to
women at 16-18 weeks of gestation. AFP in maternal serum is of fetal origin. Increased
levels of AFP are found in fetuses with neural tube defects, anencephaly, Turner’s
syndrome, omphalocele, sacrococcygeal teratoma, intestinal obstruction and missed
abortion. Low AFP levels are observed in intrauterine growth retardation, Trisomy 18,
Trisomy 21, and other conditions. However, a normal AFP level does not rule out
trisomy. Women with a maternal age exceeding 35 years are at high risk for having
babies with Downs Syndrome. Consequently, this group and indeed most pregnant
women are now offered assay for both AFP and human chorionic gonadotropin
(hCG). Human chorionic gonadotropin levels are frequently elevated in fetuses with
Trisomy 21.
4 Amniocentesis
Amniocentesis involves sampling and analysis of amniotic fluid to detect pres-
ence of metabolic disorders and chromosomal defects. Amniocentesis can be safely
performed between 12-18 weeks gestation and usually takes 14 days to obtain results.
The safety of this procedure is somewhat better than CVS. The risk of miscarriage
following amniocentesis is less than 1%.
Fetoscopy and Fetal Sampling
When appropriate, these procedures are performed between 15 and 21 weeks of
gestation. Analysis of fetal blood can reveal many conditions including Wiskott-
Aldrich syndrome, hemophilia A and B, hemoglobinopathies, alpha 1-antitrypsin
deficiency, and chronic granulomatous disease. Conditions in which the genetic
defect is not expressed in the amniotic fluid can be discovered by sampling from the
skin and liver. Fibroblastic cell culture can be performed to reveal mosaicism. The
risk of miscarriage is around 5%.
Percutaneous Umbilical Blood Sampling
Percutaneous aspiration of umbilical cord blood can be safely performed under
ultrasound guidance. The blood samples can reveal hematologic abnormalities
including isoimmunization. This procedure is usually done between 18 and 20 weeks
of gestation. The risk of miscarriage is about 2%. Results of analysis are usually
available within 2-3 days.
Prenatal Ultrasound
Sonography is the most important method of fetal screening. It is useful to de-
termine gestational age of the fetus, single or multiple births, the amniotic fluid
volume, growth in high risk pregnancies, and a significant number of fetal anoma-
lies. Ultrasonography is the best noninvasive method for determining both func-
tional and anatomic abnormalities in the fetus. Evaluation of amniotic fluid volume
is most important. When performed in the 4th month of gestation, the finding of
normal amounts of amniotic fluid suggests normal swallowing and renal function.
A finding of reduced amniotic fluid volume, or no fluid at all, is a sign of impaired
renal function, such as obstruction, multicystic kidneys or renal agenesis. Too much
amniotic fluid (more than 2000 ml = polyhydramnios) suggests impaired fetal swal-
lowing (neurologic abnormality, anencephali), proximal alimentary tract obstruc-
tion, or compression of the esophagus due to diaphragmatic hernia or congenital
lung malformation.
Genetics and Prenatal Diagnosis in Pediatric Surgery 13
Although a single ultrasound study can provide a wealth of information, serial

studies over time can provide even more. Functional evaluation of kidney, heart,
and lungs is considerably more accurate with repeated exams.
The heart and the great vessels are easily visualized with ultrasonography or fetal
echocardiography. The dynamic function can be evaluated. The four chambers and
the two great vessels should be visualized and allows the diagnosis of anomalies such
as tetralogy of Fallot, tricuspid atresia, hypoplastic left heart, aortic valve stenosis/
atresia and double outlet right ventricle.
Cerebral malformations, encephaloceles, and hydrocephalus are readily identi-
fied by prenatal ultrasound. Intraabdominal structures like hepatic neoplasms
(hemangioma), neuroblastoma, enteric duplications and atresias of the gut can also
4
be detected. The differentiation of omphalocele and gastroschisis is especially
important because the prognosis in omphalocele is so much worse, compared to
gastroschisis, because of the serious associated anomalies and chromosomal defects.
Indications for Prenatal Diagnosis
General Risk Factors
Maternal age more than 35 years (increased risk for fetal chromosomal
abnormality).
Elevated or reduced serum AFP
Increased serum hCG.
Specific Risk Factors
Previous still birth or neonatal death.
Previous child with a structural defect or chromosomal abnormality.
Structural abnormality in any of the parents.
Balanced translocation in any of the parents.
Inherited disorders (i.e., cystic fibrosis, metabolic disorders, sex-linked
disorders)
Medical disease in the mother (i.e., diabetes mellitus)
Exposure to teratogens (i.e., ionizing radiation, anticonvulsant medicine,
alcohol, etc.)
Infections (i.e., rubella, toxoplasmosis, cytomegalovirus)
Treatment of the Fetus
Hydrops fetalis can occur secondary to isoimmunization induced hemolysis.
Prenatal treatment of this condition may include erythrocyte transfusion in utero.
Transplacental treatment can be administered for cardiac arrhythmias (especially
supraventricular tachycardia) leading to hydrops. The most common prenatal treat-
ment used for the fetus is steroid (i.e., glucocorticoid ) administration to increase
pulmonary surfactant in the lungs of the preterm infants.
Fetal Surgery
The indication for fetal surgery is malformations that interfere with fetal organ
development, which if alleviated would allow normal organogenesis. Pioneering work
is now carried out in a few centers for highly selected cases; however, these procedures
involve significant risks for both the mother and the fetus (i.e., infection, premature
labor, etc.).
Although no large series have proven any long term benefits, work continues
(and should continue at a few centers with close supervision) on the use of fetal
surgery for:
1. vesicoamniotic shunt for severe bilateral hydronephrosis with pulmonary
hypoplasia
2. congenital diaphragmatic hernia with prenatal prosthetic patch repair or
tracheal plugging
4 3. lobectomy for congenital cystic adenomatoid malformation
4. thoracoamniotic shunt for fetal chylothorax
5. ventriculoamniotic shunt for severe obstructive hydrocephalus
6. resection of sacrococcygeal teratoma to prevent cardiac failure secondary
to arteriovenous fistula
7. correction of critical aortic stenosis to prevent severe left ventricular
hypoplasia
Selected Readings
1. Harrison R. The fetus as a patient. In: O’Neill Jr. JA, et al, ed. Pediatric Surgery, 5th
Edition St. Louis: Mosby, 1998; 33-41.
2. Zackai EH, Robin NH. Clinical Genetics. In: O’Neill Jr. JA et al, eds. Pediatric
Surgery, 5th Edition. St. Louis: Mosby, 1998; 19-31.
3. Caniano DA, Baylis F. Ethical considerations in prenatal surgical consultation.
Pediatr Surgery Int 1999; 15:303-9.
4. Milner R, Adzick NS. Perinatal management of fetal malformations amenable to
surgical correction. Curr Opin Obstetr Gyn 1999; 11:177-83.
Section II: Perioperative Management
and Critical Care
CHAPTER 5
Vascular Access
Marleta Reynolds
Blood Sampling
Current microtechniques of chemical analysis allow small samples of blood to be
taken from children. Capillary tubes can be used for obtaining blood by “heel-stick”.
If more blood is needed an antecubital or scalp vein can be used. An assistant will be
needed to restrain the child. A 21 or 23 gauge scalp needle (butterfly) with preattached
plastic tubing and a small syringe is used to penetrate the skin and enter the vein.
Blood will flow immediately and can be aspirated gently by the assistant. Peripheral
arterial blood can be sampled in a similar fashion.
Under extreme conditions an experienced physician may use a femoral vein for
blood sampling. The child will need to be adequately restrained and the skin pre-
pared with antibacterial solution. The femoral artery is palpated and a small scalp
vein needle is inserted just medial to the femoral artery.
Venous Access
Access for infusion therapy can be obtained by percutaneous insertion of steel
needles or plastic catheters or by cutdown on peripheral veins. When placing a per-
cutaneous catheter make a small nick in the skin at the insertion site with a separate
needle to eliminate skin traction on the plastic catheter and avoid damage to the tip
of the catheter. A local anesthetic can be injected to raise a skin wheal at the inser-
tion site. If time allows a topical anesthetic cream can be applied. The needle and
plastic catheter are inserted until blood returns. The catheter can then be advanced
over the needle into the vein. The catheter is secured by a plastic dressing and tape to
allow monitoring of the insertion site and catheter tip site. Phlebitis is the most
common complication of peripheral intravenous catheters.
Cutdowns for peripheral venous access are being used less frequently. The cepha-
lic vein at the wrist and the saphenous vein at the ankle are good sites because of
their superficial and constant location. Meticulous care should be taken in restrain-
ing the extremity and maintaining sterile technique. A vertical incision over the vein
provides for greater exposure and the incision can be extended proximally if more
length of the vein is needed. A plastic catheter can be placed in the vein by making
an oblique venotomy. If the vein is very small the catheter can be passed over a
needle. The catheter is secured with absorbable suture and the wound is closed. A
sterile dressing is placed and the extremity is immobilized. Peripheral arteries can be
cannulated using a similar technique.
Vascular Access 17
Central Venous Access

Central venous access can be obtained by cutdown or percutaneous technique.
“PIC” lines or “PCVCs” are small silastic catheters advanced into the central circu-
lation via a peripheral vein. These central lines can be placed with or without ultra-
sound guidance. These lines cannot be maintained indefinitely but are ideal for
several days and up to several weeks. Catheter related sepsis occurs in 2.7-6% of
patients with these catheters. Venous thrombosis has been reported in 0.3%.
When short-term, multiple port or large bore access is needed, a percutaneous
central line can be placed via the subclavian, external or internal jugular vein. For
prolonged parenteral nutrition, blood samplings, or chemotherapy, a tunneled silastic
catheter with or without a venous reservoir is preferred. The catheter can be placed
with a percutaneous technique or by cutdown utilizing the subclavian, external jugu-
lar, internal jugular or saphenous veins. Fluoroscopy should be used during place- 5
ment of any central line to confirm correct placement. If the subclavian vein has
been accessed, a chest x-ray should be obtained to identify an associated pneumothorax
or other thoracic complication.
Umbilical Vessel Access
Central venous and arterial access can be obtained through the umbilical cord in
a newborn. The distal cord is amputated after the area is prepped with an aseptic
solution. The umbilical vein is large and thin-walled and a 5 French plastic catheter
can be advanced through the ductus venosus into the right atrium. A 3.5 French soft
plastic catheter can be advanced into either of the paired umbilical arteries and
positioned in the thoracic or abdominal aorta. The catheter should be positioned
above the diaphragm or below the level of the renal arteries. The position of either
catheter must be verified by x-ray. Heparin is added to the infusate to prevent throm-
bosis. Because of the high associated complication rate both of these catheters should
be removed as soon as possible.
Intraosseous Access
In emergency situations intravenous access may not be easily or rapidly attain-
able in an infant or small child. The intraosseous route may be used for infusion of
fluid, drugs and blood. Bone marrow needles, short (18-22 gauge) spinal needles or
large (14-16 gauge) hypodermic needles can be used. The knee is supported and the
tibia prepared with antimicrobial solution. The needle is placed in the midline of
the anterior tibia on the flat surface 1-3 cm below the tibial tuberosity. The needle is
directed inferiorly at a 60-90% angle and advanced until marrow content is aspi-
rated. The fluid should flow freely into the intramedullary space. The needle is
stabilized with a supported dressing to prevent dislodgement. Placement may be
checked with a miniature C-arm imaging device.
It is contraindicated to use the intraosseous route in children with diseases of the
bone or with ipsilateral extremity fractures. Needle dislodgement with subperiosteal
or subcutaneous infiltration of fluid is the most common complication. Compart-
ment syndrome and osteomyelitis have been reported. The infection rate is not
higher using this technique. Fears over potential injury to the tibial growth plate
have not been substantiated. It is generally advisable to remove an intraosseous needle
as soon as possible.
Selected Readings
1. In: Simon RR, Brenner BE, eds. Emergency Procedures and Techniques, 3rd edi-
tion. Baltimore: Williams & Williams 1994; 418-419.
2. Guy J, Haley K, Zuspan SJ. Use of intraosseous infusion in the pediatric trauma
patient. J Pediatr Surg 1993; 28(2):158-61.
3. Donaldson JS, Morello FP, Junewick JJ et al. Peripherally inserted central venous
catheters: US guided vascular access in pediatric patients. Radiology 1995;
197(2):542-4.
4. Dubois J, Garel L, Tapiero B et al. Peripherally inserted central catheters in infants
and children. Radiology 1997; 204(3):622-6.
5
CHAPTER 1
CHAPTER 6
Fluids and Electrolytes

John R. Wesley
Paramount to successful treatment of infants and children with surgical disease

is the establishment of fluid and electrolyte balance as expeditiously as possible,
preferably preoperatively. Adequate vascular access must be established (see previous
section) and careful attention given to keeping the infant or child warm, and reduc-
ing insensible losses. Special attention must be given to estimating and correcting
pre-existing dehydration, and special note taken of the physiologic status of the
patient.
Most neonates are born with 10% fluid excess secondary to high levels of antidi-
uretic hormone (ADH) that limit excretion of fluid during the first 24 hours of life.
Overaggressive administration of fluid and electrolytes will interfere with normal-
ization of the physiologic process. Fluid overload is linked with the development of
patent ductus arteriosus, respiratory difficulty, and has been linked as a contributing
factor to necrotizing enteral colitis.
Fluid loss is composed of sensible water (urine, feces, sweat) and insensible water
loss (respiratory and transepidermal).
Fluids
Insensible Water Loss
Respiratory water loss increases with low humidity of inspired air and increases
in minute ventilation (increased metabolic rate, fever, congestive heart failure, and
respiratory distress syndrome).
Transepidermal Water Loss Is Affected by:
Skin keratin thickness (e.g., thin in VLBW, thick in postmature infant)
Surface area/body mass
Postnatal age
Activity level
Body temperature
Postural changes
Ambient humidity
Ambient temperature
Air currents (e.g., open bed)
Phototherapy
Radiant heat
Other Sources of Fluid Imbalance (Sensible Water Loss)

Third space (e.g., NEC burns)
Diarrhea
Diabetes insipidis
SIADH
Renal failure
Congestive heart failure (e.g., from PDA)
Hyperglycemia (osmotic diuresis)
Estimated Maintenance Fluid Requirements for Premature to
Term Infants(ml/kg/d):
Premature Term
Day < 1250 g > 1250 g
6 1 100 75 60-75
2 100-120 75-100 75-85
3 120-up 100-up 100
Note: The above table is only an estimate of fluid requirements. Careful

monitoring of fluid status is essential. Some VLBW infants require very large
amounts (e.g., 250-300 ml/kg/d) of fluid. Patients under warmers or receiving
phototherapy may require an additional 15-25 ml/kg/d.
Maintenance Fluid rRequirements for Term Infants

and Older Children
Weight Daily Fluid requirements
0-10 kg 100 cc/kg/day or 4 cc/kg/hr
10-20 kg 1000 cc + 50 cc/kg/day >10 kg or 40 cc + 2 cc/kg/hr >10 kg
> 20 kg 1500 cc + 20 cc/kg/day > 20 kg or 60 cc + 1 cc/kg/hr > 20 kg
Electrolytes
Maintenance Electrolytes for Premature Infants:
Sodium
Maintenance: 2-4 mEq/kg/d for infants > 30 weeks gestation; 3-5 for infants
< 30 weeks gestation
Generally not given in the first 24 hours
In VLBW infants and infants born with gastroschisis and omphalocele, check
base line sodium (electrolytes) at birth
Bicarbonate is a sodium salt: 1 mEq NaHCO3 = 1 mEq Na
Potassium
Maintenance: 2 mEq/kg/d
Generally not in first 24 hours of age, or until infant has urinated
Decrease need with renal compromise or extensive tissue breakdown (e.g.,
NEC, burns)
Increase need with diuretics and certain drugs (e.g., Amphotericin B)
Fluids and Electrolytes 21
Maintenance electrolytes for term infants and children up to 20 kg
Component Supplied As Amount Required Comments

Na NaCl; Na acetate 2-4 mEq/kg/day The acetate salt should be used
in hyperchloremic patients.
When used as a phosphate
source, each millimole of Na
phosphate provides approx.
1.3 mEq Na.
K KCl; K phosphate 2-4 mEq/kg/day Each millimole of K phosphate

K acetate provides approximately 1.5 mEq
potassium.
Ca Ca Gluconate 0.5-3.0 mEq/kg/day Premature infants require more

10% 10 mL (l g) provides calcium than full-term infants or
4.8 mEq children. An initial dose of 1 mEq
/kg/day should be adjusted on 6
basis of serum calcium and PO4
measurements. Precipitation fac-
tor should be calculated and
should not exceed a factor of 3.
Calcium- [(Calcium mEq/kg) + (Phosphate mM/kg)] x Wt(kg) x 100 Adjunct Calcium or

Phosphate ≤3 Phosphate to maintain
Precipitation Precipitation Factor
Factor ≤ 3 (per 100ml).
Total Infusion Volume per Bottle
PO4 K phosphate 0.5-1.5 mM/kg/day Order only to provide mainten-

Na Phosphate nance phosphorus, the major
anion of intracellular fluids,
important in the formation of
ATP, ADP, and creatine phos-
phate. Due to valence change
with pH, PO4 is ordered in milli-
moles rather than millequiva-
lents. The normal serum level for
term newborns is 3.5-8.6 mg/dL;
for premature newborns during
the first week only it is 5.4 to
10.9 mg/dL, and declines toward
term newborns in 304 weeks.
Mg MgSO4 0.5-1.0 mEq/kg/day A major cation in the body acting

as a catalyst for many intracellu-
lar enzymatic reactions.
Electrolyte ranges are for patients up to 10 kg. Heavier (and older) patients should
be given electrolytes based on standard replacement solutions (0.5 NS or NS)
supplemented according to serum electrolyte measurements.
Dehydration
Add to maintenance fluids any losses from dehydration:
% Weight H2O Na Cl K
loss cc/kg mEq/kg mEq/kg mEq/kg
5 50 4 3 3
10 100 8 6 6
15 150 12 9 9
For practical purposes, mild to moderate dehydration should be corrected with
IV D5-1/2 NS + 20 mEq KCl/L; and severe dehydration should be corrected with
Ringers Lactate or normal saline (NS) + 20 mEq/KCl/L.
On the first day after birth, maintenance fluids are usually started with D10W
and blood sugar maintained between 60-80 mg/dL. More fluid is required for
insensible and/or third space losses. On the second day after birth, sodium and
potassium may be added in accordance with fluid status and electrolyte determina-
6 tion. On the third day after birth, IV fluids are gradually increased, dependent on
clinical status.
Sodium and potassium may be required within the first day after birth if fluid
losses after surgery are high. Sodium should not be added to the IV fluid until
serum sodium is < 135 mg%, and there is no evidence of edema or other over-
hydration. The acetate form of sodium and potassium are given to small for gesta-
tional age (SGA) neonates. Usually sodium and potassium chloride can be given to
surgical patients if the base excess is > 0 and urine pH is greater than 7.
If the patient is unable to take oral nutrition by the third day of age, parenteral
nutrition should be started at that time, once fluid and electrolyte balance has been
attained.
Selected Readings
1. Wesley JR, Khalidi N. Faubion WC et al. The University of Michigan Medical
Center Parenteral and Enteral Nutrition Manual, Sixth Edition. North Chicago:
Abbott Laboratories, 1990.
2. Nelson WE, Behrman RE, Kliegman RM et al. Fluid and Electrolyte Therapy. In:
Joe Editor et al eds. Textbook of Pediatrics. Philadelphia: WB Saunders Co. 1996;
206-222.
3. Bell EF, Oh W. Fluid and electrolyte balance in very low birth weight infants. Clin
Perinatol 1979; 6:139–150.
4. Rowe MI. Fluid and electrolyte management. In: Welch KJ et al, ed. Pediatric
Surgery, 4th Edition. Chicago : Year Book Medical Publishers 1986; 22–27.
CHAPTER 1
CHAPTER 7
Nutrition and Metabolism

John R. Wesley
Introduction
Several considerations make parenteral nutrition in the premature neonate, the
infant, and young child, significantly different from that in the adult. These include
smaller body size, rapid growth, highly variable fluid requirements, and in newborn
infants, the immaturity of certain organ systems, especially the liver, kidney, lung,
and gastrointestinal tract. Infants and children have markedly decreased energy stores
when compared with the adult, and are much more rapidly affected by inability to
eat, and nothing by mouth (NPO) orders that frequently accompany the onset of
severe illness and subsequent diagnostic studies. An infant or child stressed by major
infection, severe trauma, or major surgery is frequently unable to tolerate enteral
nutrition. Inadequate nutritional support may result in weakening of respiratory
muscles, depression of central nervous system function, apnea, increased difficulty
in weaning from mechanical ventilation, and increased susceptibility to infection.
The disordered nutrient metabolism encountered during severe systemic stress re-
sults in altered nutrient requirements. A traumatized or septic patient has signifi-
cantly increased fluid and electrolyte requirements, as well as increased energy needs.
Other important nutrients in relatively short supply, such as water-soluble vitamins
and some fat-soluble vitamins, are used at a more rapid rate, and if unrecognized
and unreplaced, become rate-limiting factors without which recovery and wound
healing cannot proceed. Minor metabolic stress can be met and overcome with rela-
tive ease; but with children, especially neonates, there is never much margin for
error. Major stress demands a much more sophisticated understanding of host
response, altered organ physiology, and a detailed finely tuned plan for effective
intervention.
Because so many medical and surgical problems are made worse by malnutri-
tion, parenteral nutrition should be initiated as early as possible once a physician
determines that an infant or small child is malnourished, or unlikely to tolerate
enteral nutrition within a 3-5 day period. Most normal newborns establish positive
nitrogen balance with weight stabilization or weight gain by the second to fourth
postpartum day. In infants unable to take adequate enteral nutrition, sufficient
nutrients can be provided with peripheral parenteral nutrition by infusing glucose-
amino acid solutions concomitantly with fat emulsion. Central venous access may
be more appropriate in infants who require fluid restriction, or in infants with lim-
ited peripheral vein access and with a need for prolonged parenteral nutrition.
Every infant receiving parenteral nutrition (PN) goes through a period of physi-
ologic adjustment which can be divided into two stages. The first stage is a time of
increasing tolerance to the PN solution as reflected by the serum and urine glucose
levels. During this time the glucose and lipid dose should be gradually increased
until a sufficient number of calories are provided or until other factors, such as the
volume of fluid tolerated, limit further increases. The time required for this initial
adjustment phase is extremely variable. Immature infants, or those with severe stress
due to infection or respiratory insufficiency, will require a longer period for stabili-
zation than the more mature infant. The second stage marks the beginning of the
period during which the infant is receiving an adequate number of calories for weight
gain and electrolyte balance is stable. Optimal weight gain for newborns during this
phase should be 15-25 grams per day, or 1-2% of total body weight in kg/day in
older patients. Weight gain greater than this may reflect excess fluid administration
and fluid retention. Inadequate weight gain may reflect an underlying metabolic
insult, such as sepsis. During both phases, it is very important to keep accurate
intake and output records, and obtain daily weights at the same time and with the
same scale each day. Urine output should run 1 mL/kg/hour or more, with urine
7 specific gravity between 1.005 and 1.015 in the absence of glucosuria.
PN solution is ordered for 24 hour periods, at a specified hourly flow rate. The
patient’s daily weight must be provided so that the pharmacist can check the appro-
priateness of the orders for the components being compounded. Volume, amino
acids, lipid emulsion, and all electrolytes should be ordered per kilogram. (Chap-
ter 6, Fluid and Electrolytes) Dextrose, amino acid, and fat emulsion calories along
with the nonprotein calories-to-gram nitrogen ratio should be calculated to ensure
the appropriate balance of PN components. This ratio should always be in the range
of 150-300 nonprotein calories per gram of nitrogen.
The following tables summarize the essential components of a complete set of
PN orders (Tables 7.1 and 7.2).
Administering Parenteral Nutrition Solutions
Infants should be started on half-strength solutions (4-8 mg/kg/min of dextrose)
and advanced to 3/4 and full-strength (10-14 mg/kg/min maximum) over the ensu-
ing 24-48 hours, with rates adjusted according to urine and blood glucose determi-
nations. Total volume can then be increased as tolerated to further increase caloric
intake. Because of the high rate of phlebitis with hyperosmolar solutions, peripheral
PN concentration should not exceed 12.5% dextrose and 2.5% amino acids.
In low birth weight and critically ill infants, umbilical artery (UA) and vein
(UVC) catheters are usually present. Central strength formulations (greater than
10%) can be infused through a UVC once placement of the catheter tip above the
diaphragm is confirmed. Concentrations of dextrose administered though a UAC
should usually be limited to a maximum of 12.5%.
It is safe to begin fat emulsion at a rate of 0.5-1 g/kg/day on the first day of TPN.
Lipids can be advanced at a rate of 0.5 g/kg/day to a maximum of 3 g/kg/day in
premature infants, and 4 g/kg/day in full term infants.
Central PN solutions may be administered alone, or as a 3-in-1 solution with
intravenous fat. Peripheral formulations are generally given concomitantly with
intravenous fat to reduce the osmolarity of the final solution and to keep the total
Nutrition and Metabolism 25
Table 7.1. Pediatric PN: Macronutrients

Fluid Combination of 60-150 mL/kg/day See Chapter on fluid and electro-

items below lytes. Monitor intake and output.
Aim for urine output of 1-2 mL/kg
/hr with urine Sp Gr. 1.005-1.015.
Calories Protein 4.0 kcal/g 45-120 kcal/kg/day Maintenance and Normal Growth:
CHO 3.4 kcal/g Age (yrs): Kcal/kg:
Fat 9.0 kcal/g 0-1* 90-120
(IV Fat emulsion 12-18 75-90
10% = 1kcal/mL; 12-19 60-75
20% = 2 kcal/mL. 12-20 45-60
Calorie requirement increased by
one or more of the following: 12%
increase for each degree of fever
above 37°C. 20-30% increase with
major surgery. 40-50% increase
with severe sepsis. 50-100%
7
increase with long-term growth
failure. *Premature infants should
start at 80 kcal/kg and increase as
needed for appropriate weight
gain.
Protein Amino Acid To provide essential In neonates, protein should be
solution and nonessential initiated at 1 g/kg and advanced
5% amino acids to a maximum of 2.5 g/kg/d.
7% 1.7-2.5 protein For every gram of nitrogen given,
g/kg/day 150-300 nonprotein calories
should be provided as carbohy-
drate or fat to maintain positive
nitrogen balance. 1 g protein =
0.16 g nitrogen.
Carbohydrate D5, D10, D25, To provide neces- Use D10 + A2.0 for peripheral PN
D50 sary calories (0.4 kcal/mL) Nonprotein-calorie/
Rate: gN ratio 85:1. Add fat emulsion for
0.4-1.5 g/kg/hr. additional nonprotein calories.
D25 + A3.5 for Central PN
(1.0 kcal/mL) Nonprotein-calorie/
gN ratio 155:1. In neonates up to
D12.5 can be administered in
peripheral lines and through umbi-
lical artery catheters. D25 can be
infused through umbilical venous
catheters if proper line placement
in the right atrium is confirmed.
volume within manageable limits. Ideally, the daily intravenous calorie budget should
approximate normal calorie distribution in a balanced enteral pediatric diet: 45%
carbohydrate, 40% fat and 15% protein. In actual practice, only enough fat need be
given to prevent essential fatty acid deficiency. Most importantly, the sum of the
Table 7.2. Pediatric PN: Micronutrients

Vit B12 Cyanocabalamin 1 mcg is provided in Important coenzyme function

the Pediatric Multivi- related to growth, red and
tamin Solution white blood cell maturation.
Included in multivitamin
solution.
Folic Acid 140 mcg is provided Important factor in cellular

in the Pediatric Multi- growth, especially in the
vitamin Solution maturation of red and white
blood cells. Included in multi-
vitamin solution.
Multivitamins Pediatric Multi- 2-3 mL/day* Each 3 mL vial contains:

vitamin Solution Vitamin A – 2300 IU
(Astra) Vitamin D – 400 IU
Ascorbic Acid – 80 mg
7 Thiamine (B1) – 1.2 mg
Riboflavin (B2) – 1.4 mg
Niacinamide – 17 IU
Pyridoxine – 1 mg
Pantothenic Acid – 5 mg
Vitamin E – 7 IU
Folic Acid – 140 mcg
Cyanocobalamin (B12) – 1 mcg
Phytonadione (k1) – 200 mcg
Biotin – 20 mcg
*Neonates and infants under
1.75 kg: 2 mL/day
Infants and children 1.75-30 kg:
3 mL/day
Iron RBCs or Imferon® 2 mg/day Start at 5 weeks of age. Higher
requirement with unreplaced
blood loss or chronic iron
deficiency. A test dose of
Imferon is necessary before
instituting therapy.
Phytonadione AquaMephyton® 200 mcg are pro- Important in the production of

(Vit K1) vided in the Pediatric certain coagulation factors (pro-
Multivitamins thrombin, VII, IX, X). Deficiency
and hemorrhagic diathesis may
develop rapidly in neonates
who are not being fed enterally.
Included in multivitamin
solution.
continued on next page
Trace elements Each 0.3 mL of trace elements

Less than 10 kg mixture contains:
Zn 300.0 mcg/kg/day 300.0 mcg Zn
Cu 20.0 mcg/kg/day 20.0 mcg Cu
Mn 10.0 mcg/kg/day 10.0 mcg Mn
Cr 0.2 mcg/kg/day 0.2 mcg Cr
Se 0.8 mcg/kg/day 0.8 mcg Se
Each patient (1-9 kg) should
receive 0.3 mL/kg/day of the
mixture.
Trace elements Each 0.1 mL of trace elements

10-30 kg mixture contains:
Zn 100.0 mcg/kg/day 100.0 mcg Zn
Cu 20.0 mcg/kg/day 20.0 mcg Cu
Mn 10.0 mcg/kg/day 10.1 mcg Mn
Cr 0.2 mcg/kg/day 0.2 mcg Cr
Se .8 mcg/kg/day 0.8 mcg Se
Each patient (10-30 kg) should
receive 0.1 mL/kg/day of the
mixture. In patients with weight 7
greater than 30 kg, use adult
trace element solution,
1 mL/day.
Heparin 1 IU/mL Helps prevent platelet thrombi

and clots from forming at the
catheter tip. Heparin may be
deleted from the TPN of
neonates and children on
ECMO therapy.
nonprotein calories should be sufficient to provide a total nonprotein-calorie-to-

gram-nitrogen ratio in the range of 150:1 to 300:1. This range is necessary to achieve
the optimal utilization and protein-sparing effect of the administered PN solution.
Excessive or unbalanced protein intake has been associated with metabolic acidosis
in small premature infants.
Tables 7.1 and 7.2 are designed to provide a guide for parenteral nutrition in
newborn infants and small children. The nutritional requirements for children over
age 3 years and teenagers will not be dealt with separately except to reiterate their
increased caloric requirements due to rapid growth and development. In addition,
each pediatric unit or service may have guidelines for specific application of PN for
problems unique to their patients.
Monitoring
Infants on PN must be carefully monitored. In addition to accurate daily intake,
output, weight, and weekly length and head circumference, judicious use of blood
tests is very important in infants and children due to their small total blood volume.
Table 7.3 outlines recommended tests and frequency of monitoring. Careful atten-
tion to the values will alert the physician to potential metabolic complications and
ensure optimal benefit from PN therapy.
Table 7.3. Blood values monitored routinely during parenteral nutrition

Frequency of monitoring
At start of therapy At start of therapy
and biweekly* and weekly As indicated
Na, K, Cl SGOT, LDH, alkaline Copper

phosphatase
Creatinine
Urea Bilirubin direct/total Zinc
Glucose Triglycerides Iron
Hgb, Hct, WBC, Magnesium Ammonia
platelets Albumin or Prealbumin Osmolarity
Calcium, phosphorus pH
*Serum levels should be monitored more frequently in the premature infant.
Complications
7
Technical Complications
The incidence of technical complications due to placement and position of cen-
tral lines in infants and children has been greatly reduced in recent years by careful
attention to aseptic technique and x-ray conformation after catheter insertion. The
introduction of nonreactive silicone catheters in place of polyvinylchloride catheters
has reduced the incidence of foreign body reaction and subclavian vein or vena cava
thrombosis. The incidence of cardiac arrhythmias due to irritation from the catheter
has been greatly reduced by placing the tip of the catheter at the junction of the
superior vena cava and the right atrium rather than in the heart. Suturing the cath-
eter to the skin at the catheter-cutaneous junction and checking to be sure that the
catheter is secure at each 72 hour dressing change has greatly reduced the frequency
of catheter dislocation. The complications arising from administration of PN through
an umbilical artery catheter (UAC) in neonates are associated with the UAC place-
ment, e.g., vasospasm, thrombosis, embolization, hypertension, hemorrhage, and
necrotizing enterocolitis.
Almost all of the technical complications inherent in central PN can be avoided
by use of peripheral PN administration. Phlebitis and superficial skin slough are the
most common complications in patients receiving peripheral PN. The incidence of
phlebitis is reduced in patients receiving concomitant intravenous fat emulsion.
Simultaneous infusion of fat reduces the osmolarity and increases the pH of the PN
solution, and although still slightly hypertonic, the fat emulsion appears to protect
the vein from phlebitis. If an infiltrated IV site is identified quickly, it is usually
benign, and the extravasated fluid is rapidly reabsorbed. This process is enhanced by
warm moist dressings to the area and silver sulfadiazine dressings in those few cases
where skin slough occurs. Very rarely a skin slough site will require skin grafting.
Metabolic Complications
Almost every conceivable metabolic complication has been reported during total
parenteral nutrition. Table 7.4 lists the more common metabolic complications,
and although serious consequences may ensue if metabolic complications go
Table 7.4. Potential metabolic complications from PN
1. Electrolyte Imbalance
a. Hyper/hyponatremia
b. Hyper/hypokalemia
c. Hyper/hypochloremia
d. Hyper/hypocalcemia
e. Hyper/hypomagnesemia
f. Hyper/hypophosphatemia
2. Carbohydrate Administration
a. Hyper/hypoglycemia
b. Hyperosmolarity and associated osmotic diuresis with dehydration,
leading to nonketotic hyperglycemic coma.
3. Protein Administration
a. Cholestatic jaundice
b. Azotemia
4. Lipid Administration
a. Hyperlipidemia
b. Alteration of pulmonary function
c. Displacement of albumin-bound bilirubin by plasma free fatty acid 7
d. “Overloading syndrome” – characterized by hyperlipidemia, fever,
lethargy, liver damage, and coagulation disorders. This has been reported
in adults but has been recognized rarely in children.
5. Trace Element Deficiencies
a. Zinc deficiency
b. Copper deficiency
c. Chromium deficiency
6. Essential Fatty Acid Deficiency (EFAD)
EFAD occurs if lipid emulsions are not used; the major clinical manifestation
is a desquamating skin rash.
undetected for any length of time, careful clinical monitoring and appropriate
adjustment of the PN solution results in most patients tolerating parenteral nutri-
tion infusion quite well.
Infectious Complications
Sepsis continues to be the major complication of centrally infused parenteral
nutrition in infants and children, and the protocol for work-up of possible catheter
sepsis is the same as that for the adult. Placement of catheters under strict aseptic
conditions and meticulous care of the catheter site with 72-hour standardized dress-
ing changes greatly reduces the incidence of septic complications. In addition, strict
avoidance of the use of the PN catheter for blood drawing, administration of medi-
cation, or blood products, minimizes the risks of contamination and mechanical
failure.
Peripheral PN administration has the advantage of eliminating most of the sep-
tic and technical complications inherent with central catheters. However, the avoid-
ance of frequent infiltration and local infection or skin slough that may accompany
peripheral IV infusion is dependent on the same careful attention to sterile technique
of insertion and occlusive dressings that are important in central line management.
Pediatric Nutritional Assessment

Nutritional assessment of the pediatric patient differs from that of the adult. The
pediatric patient, especially the infant, does not have the reserves of an adult and
must be provided additional calories for growth. Thus, nutritional inadequacies are
seen more quickly and can be more devastating in the pediatric age group. In addi-
tion to standard biochemical parameters, such as albumin and total protein, body
weight should be obtained daily, and height/length and head circumference should
be measured at least weekly on all patients where adequate nutrient intake is ques-
tioned.
Whenever possible, enteral nutrition should be employed to support the pediat-
ric patient, and supplemented with PN to ensure adequate calories. Even a small
amount of enteral nutrition will reduce or prevent septic complications stemming
from bacterial translocation and breakdown of normal host mucosal barriers to bac-
teria, fungi, and endotoxin.
Transition from Parenteral to Enteral Nutrition
One of the most important, and frequently overlooked, phases of the pediatric
7 patient’s recovery from a severe medical or surgical illness is the transition period
from parenteral to enteral nutrition. Successful reintroduction of enteral nutrition
requires an understanding of how parenteral nutrition affects the functional capaci-
ties of the gastrointestinal tract and the limitations of the child’s immature physiol-
ogy. To manage a plan for successful transition of the pediatric patient from parenteral
to enteral support, the clinician must select an appropriate formula, design a feeding
regimen, and taper the parenteral support appropriately. The selection of a formula
is based on the child’s age, clinical pathology, and the caloric density, osmolarity,
protein content, carbohydrate and fat source, and the nutrient complexity of the
formula. The transition feeding regimen is designed to allow for adaptive increases
in digestive enzymes and surface area within the gut. Small advances in the volume
of enteral feedings are made first; increases in concentration of the formula follow
later. A systematic method for the progression of enteric support and the tapering of
parenteral nutrition is preferred.
Selected Readings
1. In: Suskind RM ed. Textbook of Pediatric Nutrition, Second edition. New York:
Raven Press 1993.
2. Wesley JR, Khalidi N, Faubion WC et al. The University of Michigan Medical
Center Parenteral and Enteral Nutrition Manual, Sixth Edition. North Chicago:
Abbott Laboratories, 1990: 54-69.
3. Wesley JR. Nutrient metabolism in relation to the systemic stress response. In:
Fuhrman BP, Zimmerman JJ eds. Pediatric Critical Care, 2nd edition. St. Louis:
C.V. Mosby 1998; 799-819.
4. Bachman AL, Klish WJ. Handbook of Nutritional Support. Baltimore, William
& Wilkins 1997: 73-91.
5. Han-Markey T, Wesley JR. Pediatric critical care. In: ASPEN, eds. Silver Springs,
MD: The ASPEN Nutrition Support Practice Manual 1998; 34:1-10.
6. Braunschweig CL, Wesley JR, Clark SF et al. Rationale and guidelines for transi-
tional feeding in the 3-30 kg child. J Amer Diet Assoc 1988; 88:479-482.
CHAPTER 1
CHAPTER 8
Respiratory Failure and Support

in Children
Marybeth Madonna
Respiratory failure in children can occur for a variety of reasons. In neonates, the
usual final common pathway is persistent pulmonary hypertension (PPH) and per-
sistent fetal circulation. Infants are born with shunts in place between the systemic
and pulmonary circulation, namely the patent ductus arteriosus and the patent
foramen ovale. In conditions with increased pulmonary vascular resistance and pul-
monary hypertension, these shunts allow blood to bypass the lungs and return to
the body prior to oxygenation. Many conditions predispose to this final pathophysi-
ology. In the preterm infant the most common is respiratory distress syndrome (RDS)
that is due to immaturity of the lungs which have deficient surfactant production.
In term infants, respiratory failure occurs due to pneumonia, sepsis, or aspiration,
most commonly of meconium but also of blood or amniotic fluid. In addition,
those babies born with congenital abnormalities of the heart or lungs such as con-
genital diaphragmatic hernia, lobar emphysema, or anomalies of venous return may
have respiratory distress secondary to PPH.
In children, the common end physiology of respiratory failure is acute respira-
tory distress syndrome (ARDS). In this condition, inflammatory mediators are
released after a stress. These mediators make the respiratory epithelium leaky and
thick, this decreases gas exchange. Again, a variety of conditions predispose to this
final pathway. Most common is pneumonia or pneumonitis caused by respiratory
syncitial virus (RSV) infection. Patients can also have a variety of other viral and
bacterial pneumonias. In addition, sepsis, stress, massive transfusions, near drown-
ing and inhalation injuries all predispose to ARDS.
Treatment of the neonate or child with respiratory failure is similar to treatment
of adults, namely providing adequate oxygenation and ventilatory support. How-
ever, there are some distinct differences. The airways of the child are smaller than
those of adults and therefore, airway conductance is less. The anterior-posterior
diameter of the glottis in infants and small children is less than one third that of
adults. In children the narrowest part of the airway is the subglottis, unlike adults
where it is the glottis. Due to these conditions, uncuffed endotracheal tubes are used
in infants and smaller children. When these tubes are used, there is a leak of ventila-
tory pressures. The respiratory rate in children is much faster than in adults, and
children tend to increase respiratory rate rather than tidal volume in times of stress.
Additionally, the inspiratory time is much shorter (as low as 0.4-0.5 seconds). Children
have lower tidal volumes than adults (despite a similar ratio of tidal volume to body
weight), with neonates having tidal volumes as low as 20 ml. All of these differences
must be considered when managing respiratory failure in children.
The characteristics of ventilator support must be understood. Historically, infants
and small children were treated with pressure control ventilation with a
nonsynchronized respiratory rate because the ventilators did not have a high enough
sensitivity to measure the small inspiratory effort of children. With the computer
era, this has changed. The variety of conventional ventilatory support available for
children today is great.
The components of the support are similar to those in adults. Volume cycled
(volume control) ventilation delivers a set tidal volume to the patient with each
breath, regardless of the pressure required to achieve that volume. In pressure cycled
ventilation (pressure control) the breath is terminated when a set peak pressure is
reached regardless of the volume delivered. With time-cycled ventilation, manda-
tory inspiration ends when a preset time has passed regardless of airway pressure or
volume delivered. Today, a combination of these modes can be used with the more
sophisticated ventilators available. This allows volume control, pressure limited ven-
tilation during which a set tidal volume is delivered to the patient as long as a preset
8 pressure is not exceeded. In the various modes of ventilation, the percentage of
inspired oxygen and respiratory rate is set. In addition, the positive end expiratory
pressure (PEEP) is set.
In comparison to tidal volume, there is a large dead space in the ventilator circuit
of children. Consequently, assistance of spontaneous respiratory effort is required.
Synchronized mandatory ventilation delivers a set number of breaths but the man-
datory breaths are synchronized with the patient’s own respiratory efforts so only a
portion of the breaths are assisted by the ventilator (the preset rate). Another aid to
spontaneous respiration is pressure support ventilation. The spontaneous inspira-
tion is sensed in this mode and a variable flow of gas is delivered until the airway
pressure reaches a preset pressure. This pressure is then actively sustained during the
patient’s inspiration. Thus, the work of inspiration is much less.
The child’s disease condition and the physician’s familiarity with the equipment
determine which of the above ventilatory modes is used. No matter what mode is
used, the physician tries to minimize the pulmonary damage caused by the ventila-
tor. Ventilator induced lung injury is a significant complication of respiratory sup-
port in children. One of the important components of the support that needs
limitation is the fraction of inspired oxygen. Oxygen toxicity is a real problem.
Increased oxygen content in airways causes increased free radical formation that in
turn causes damage to respiratory epithelium. In addition, premature infants can
suffer other long-term sequelae from high oxygen content, most importantly retin-
opathy of prematurity (ROP).
In most pulmonary disease states, a child loses functional residual capacity (FRC)
of the lung due to alveolar collapse. Higher ventilatory pressures are required to
overcome this problem. The higher peak airway (inspiratory) pressures (PIP) subse-
quently causes damage to the lungs themselves (barotrauma), setting up a vicious
cycle. Recently, it has been shown that the volume of the breath (“volutrauma”) is
also implicated in lung damage. Since it is now believed that the opening and closing
Respiratory Failure and Support in Children 33
of the alveoli is the main problem causing airway damage, the pressure and volume
of breaths are important. Pressure and volume are limited whenever possible. To
achieve this, the physician may allow permissive hypercapnea (i.e., accept PaCO2 of
60-80 as long as the pH is over 7.2). This method of respiratory support decreases
mortality in both infants and children with respiratory failure due to a variety of
conditions.
To prevent lung damage from alveolar recoil, adequate pressures are needed to
prevent the alveoli from collapsing. To achieve this, a higher level of positive end-
expiratory pressure (PEEP) is used. The use of PEEP increases functional residual
capacity (FRC) and also increases ventilation perfusion matching, thereby increas-
ing oxygenation. Higher PEEP also decreases alveolar edema.
To assist ventilation/perfusion matching, prone positioning is sometimes used.
The benefit from prone positioning is thought to result from blood flow redistribu-
tion to the dependant areas of the lung (anterior in prone positioning) or more
homogenous distribution of ventilation.
Novel Approaches for Respiratory Support in Children
Inverse ratio ventilation is occasionally used in an attempt to enhance alveolar
distension and reduce hypoxemia and pulmonary shunting. In this type of ventila-
tion, more time is spent in inspiration than expiration (usually 2:1). There is a risk 8
of incomplete expiration, which can cause auto PEEP, a condition in which the true
PEEP is much higher than that set on the ventilator.
High frequency ventilation delivers respiratory support at high rates with tidal
volumes near anatomic dead space. This is very similar to the panting of dogs. Jet
ventilation provides small bursts of gas though a jet port in the endotracheal tube at
a rate of 240-600 breaths per minute (BPM). In oscillatory ventilation, a piston
pump drives a diaphragm that delivers small volumes at frequencies of 180-900 BPM.
When using high frequency ventilation, oxygenation is manipulated by changing
the mean airway pressure (MAP) delivered and the FiO2. The ventilation is pro-
vided by the change in pressure around the MAP (also called DP). In high fre-
quency ventilation, axial streams are developed in the airways with a prograde central
core and streaming in the opposite direction at the periphery thereby transporting
particles rapidly to the terminal airways. The goal of high frequency ventilation is to
apply a MAP that recruits alveoli and maintains oxygenation while limiting the
amplitude (DP) to provide adequate chest wall movement and CO2 elimination.
Intratracheal pulmonary ventilation (ITPV) uses an infusion of fresh gas into
the trachea via a cannula placed at the tip of the endotracheal tube. This gas flow
replaces central airway dead space with fresh gas during the expiratory phase of
ventilation and functions to reduce dead space, thereby increasing CO2 elimina-
tion. Experience with this mode of ventilation in children with respiratory failure is
limited.
Surfactant is a phospholipid that is produced in the lungs by the Type II
pneumatocytes. It functions to reduce surface tension in the lungs, thereby increas-
ing compliance and FRC. It is most effective when administered with its associated
proteins. This agent has significantly reduced the mortality of premature infants
due to respiratory distress syndrome (RDS) because these infants are naturally defi-
cient in surfactant. It has also improved outcomes in full term infants and children
with respiratory failure complicated by a relative surfactant deficiency.
Liquid ventilation is a mode of ventilation in which gas exchange is achieved in
the lungs through a liquid medium. The liquid must have a low surface tension and
high solubility to oxygen and carbon dioxide and must be nontoxic. A present, the
perflurocarbons are the only commercially available liquid ventilation medium and
are only used experimentally. Either total liquid ventilation, requiring a special ven-
tilator or partial liquid ventilation where the lungs are filled to FRC with the liquid
and then conventional ventilation is used can be attempted. Phase I trials have shown
improved oxygenation and pulmonary mechanics in adults treated with this mode
of ventilation.
Nitric oxide is an endogenously produced substance that induces vascular smooth
muscle relaxation. It is generated by nitric oxide synthase (NOS) through oxidation
of L-arganine to citrulline. It stimulates guanylate cyclase to produce increased cGMP
that reduces intracellular calcium causing relaxation of vascular smooth muscle. Nitric
oxide, when administered as a gas, is a potent selective pulmonary vasodilator. This
agent has proven very useful in neonates with pulmonary hypertension. It may also
benefit older children perhaps through improved ventilation/perfusion matching.
Extracorporeal membrane oxygenation (ECMO) has been used in neonates since
8 1975. In this form of therapy, a membrane oxygenator provides oxygenation and
ventilation. The membrane is made of silicone which is very permeable to oxygen
and carbon dioxide but impermeable to blood and nearly impermeable to water.
Access is obtained through a venotomy or arteriotomy in the infant or child. The
blood is then pumped to the oxygenator and then back to the patient. Most com-
monly the neck vessels are used for access.
There are two forms of therapy. Venovenous ECMO uses only the jugular vein
and relies on a double lumen catheter to both remove and return blood to the patient.
This provides only support of oxygenation and ventilation but relies on the patient’s
cardiac output to deliver the oxygenated blood. In venoarterial ECMO, the jugular
vein and carotid artery are cannulated and both cardiac and respiratory support are
provided because the patient’s heart is partially bypassed. ECMO is a highly effec-
tive form of therapy with survival rates of over 90% for some diagnoses (such as
meconium aspiration syndrome) treated. However, it is a very invasive therapy with
significant risks, mostly due to bleeding from the anticoagulation therapy required.
Therefore, it is used with caution and only in centers with knowledge and experi-
ence with this therapy.
Selected Readings
1. Arensman RM, Statter MB, Bastawrous AL et al. Modern treatment modalities for
neonatal and pediatric respiratory failure. Am J Surg 1996; 172:41-47.
2. Hirschl RB. Respiratory failure: Current status of experimental therapies. Sem
Pediatr Surg 1999; 8:155-170.
3. Shanley CJ, Hirschl RB, Schumaker RE et al. Extracorporeal life support for neo-
natal and respiratory failure: a 20 year experience. Ann Surg 1994; 220:269-282.
CHAPTER 1
CHAPTER 9
Hypovolemic Shock and Resuscitation

Matthew L. Moront
Definition
Shock can be defined in a variety of ways. In general, shock exists when there is
evidence of multisystem organ hypoperfusion. This evidence is gathered during the
initial clinical assessment and supported by laboratory tests and monitoring sys-
temic acid-base balance. On a cellular level shock is characterized as an imbalance
between oxygen delivery and oxygen consumption. This imbalance leads to a failure
of tissue perfusion to meet metabolic demands of the cell and results in anaerobic
metabolism, metabolic acidosis, the release of inflammatory mediators, and eventu-
ally multisystem organ failure. Implicit in this definition is that inadequate perfu-
sion can be caused by decreased oxygen supply, increased oxygen demand, or a
combination of both of these factors.
Children manifest a shock state differently than adults. Perhaps the most strik-
ing difference between adults and children is the degree to which cardiac output can
fall without exhibiting systemic hypotension. The intrinsic compensatory mecha-
nisms of children allow a loss of 40-45% of the intravascular volume before systemic
blood pressure can no longer be maintained. At the point where compensatory mecha-
nisms are no longer able to maintain blood pressure, children often decompensate
with a precipitous drop in blood pressure.
Clinical Indicators of Inadequate Tissue Perfusion
Tachycardia
Tachycardia is one of the earliest signs of shock but is not specific. An increased
heart rate is also caused by other factors such as fear, anxiety and pain. The response
of the heart rate to a fluid challenge provides insight as to ongoing fluid losses or the
degree of volume deficit.
Altered Mental Status
Mental status changes are observed when cerebral perfusion is compromised as a
result of hypovolemia. Unfortunately, children with head injuries present in a simi-
lar fashion. An example of altered mental status is a child who exhibits a minimal
response to blood draws or placement of an intravenous catheter. Another example
is a child who initially appears combative or somnolent after losing blood from a
femur fracture or deep laceration and undergoes a significant improvement in men-
tal status after 20-40 cc/kg fluid challenge.
Decreased Diastolic Pressure

The diastolic pressure should normally be two thirds of the systolic pressure. A
decrease in diastolic pressure of 20 mm Hg or greater indicates significant intravas-
cular volume loss. This finding can be subtle and is also one of the early indicators of
inadequate tissue perfusion.
Mottled Cool Extremities
One of the body’s compensatory mechanisms to counter the effects of hypov-
olemia is to shunt blood away from the less critical areas in the periphery to the
essential internal organs. The result is a mottled appearance of the skin beginning in
the extremities and, in severe shock states, extending onto the torso. Peripheral per-
fusion is frequently measured by evaluating capillary refill at the nail bed, which is
normally less than 2 seconds. Children in shock frequently have measurable delays
in capillary refill. A more subjective measure of the central shunting of blood is in
the assessment of the quality of peripheral versus central arterial pulses. Children in
shock frequently exhibit thready distal pulses when compared to a femoral or carotid
arterial pulse. In severe shock states distal pulses may not be palpable. The distal
pulses will return after appropriate fluid resuscitation and the peripheral pulses will
subjectively feel as strong as the central pulsations.
Decreased Systolic Blood Pressure
In children with hypovolemic shock, decreased systolic blood pressure is a late
9 finding and indicates severe intravascular volume loss of over 40% of circulating
blood volume with vascular decompensation. A normal systolic blood pressure is
approximately 80 mm Hg plus two times the age in years. For example, a child who
is 4 years old should have a systolic blood pressure of 80 + (4 years X 2) = 88 mm Hg.
A decreased systolic blood pressure indicates all of the body’s intrinsic compensatory
mechanisms are unable to maintain adequate perfusion to the vital organs. This
situation is referred to as uncompensated shock and requires immediate attention to
prevent cardiorespiratory arrest and death.
Urine Output
A decrease in urine output represents diminished organ perfusion and is a find-
ing in children with intravascular volume loss which usually indicates a deficit of
between 25-40% of blood volume. Accurate hourly assessment of urine output
requires a bladder catheter. Another frequently used urine measurement is the spe-
cific gravity. Children with a high specific gravity (1.010-1.030) have concentrated
urine which is suggestive of a volume deficit. It must be emphasized that both of
these indicators are late findings and should only confirm the other signs of inad-
equate tissue perfusion and volume deficit.
Treatment of Shock
Priorities
Establishing a secure airway with protection of the cervical spine occupy the
highest priority in the primary survey. Ensuring adequate ventilation and oxygen-
ation are also essential initial steps prior to assessing for signs of inadequate tissue
Hypovolemic Shock and Resuscitation 37
perfusion. Once these tasks are accomplished, attention is directed at evaluation of

shock and restoration of adequate circulating volume.
Intravenous Access
The placement of sufficient intravenous access is a considerable challenge in a
seriously ill infant or young child. A systematic stepwise approach is essential in
accomplishing this difficult task.
Two large bore catheters placed above and below the diaphragm are optimal. No
more than 90 seconds or two attempts should be made at peripheral intravenous
(IV) access before moving on to alternate methods for children in hypovolemic
shock. In children under 6 years of age, an intraosseous infusion device can be placed
in the proximal tibia or distal femur. This route is only used in an unconscious
victim in extremis.
Older children in whom peripheral IV access cannot be obtained require a saphe-
nous vein cutdown at the ankle or groin. This procedure should be performed by a
surgeon or someone skilled in this method of securing IV access.
Failure of the previously described methods mandates placement of a central
venous line. Possible insertion sites include the subclavian, internal jugular, and the
femoral veins. For children in severe shock, the preferred site is the femoral approach.
This leaves the head and torso free for reassessment and other procedures and avoids
life-threatening complications (i.e., pneumothorax, hemothorax).
Fluid Resuscitation 9
After assessing for signs of inadequate perfusion and securing intravenous access,
administration of a 20 cc/kg crystalloid fluid bolus is appropriate. A careful reassess-
ment following the bolus will provide information as to the need for further fluid
challenges. If the heart rate decreases significantly, the mental status clears, or other
signs of poor perfusion disappear then no additional fluid boluses are needed. If the
child’s status is either only slightly improved or unchanged, a second challenge of
20 cc/kg is required. Reassessment after the second bolus using the same evaluation
criteria usually reveals a restoration of adequate circulating intravascular volume.
Evidence of persistent hypovolemia requires the clinician to conduct a careful search
for sources of ongoing or unrecognized hemorrhage. A third crystalloid bolus is
initiated and 10 ccs/kg of crossmatched packed red blood cells (PRBCs) delivered
via a rapid fluid warmer. If insufficient time has passed for a full crossmatch,
unmatched type specific cells, or else O-negative packed red blood cells (PRBCs)
are given.
Thermoregulation
The maintenance of the body’s core temperature is an essential component in
restoring homeostasis to an injured child. Hypothermia, defined as a core tempera-
ture less than 36˚C causes coagulopathy and acidosis. All fluids are warmed to as
near body temperature as possible via in-line warming devices. This is especially true
for blood and blood products, which are normally stored at 4°C. The temperature
in the resuscitation area is kept high and the child kept covered unless exposure is
necessary for examination or intervention. It is much easier to keep an injured child
warm than it is to re-warm a child who has become hypothermic.
Selected Readings
1. American College of Surgeons Committee on Trauma. Advanced Trauma Life Sup-
port Course. Chicago, IL: American College of Surgeons 1997.
2. American Heart Association. Pediatric Advanced Life Support. Dallas, TX: Ameri-
can Heart Association, 1988.
3. Walley KR, Wood LDH. Shock. In: Hall JB, Schmidt GA, Wood JDH eds. Prin-
ciples of Critical Care, 2nd Edition. New York: McGraw Hill 1992; 277–301.
9
CHAPTER 1
CHAPTER 10
Blood Component Therapy

Richard Fox
Blood component therapy has revolutionized the ability to care for patients with
both acute and chronic medical conditions. However, as with the administration of
any medication, inherent risks exist. These risks include immunologic, infectious or
metabolic derangements. So the medical practitioner must weigh the benefits and
risks. To do so, it is important to understand the following information.
Blood Component Preparation
Whole blood is the source from which all other blood components are derived.
A preservative solution is added and the mixture is centrifuged. The resulting prod-
uct includes packed red blood cells (PRBCs) and a plasma/platelet mixture. The
plasma/platelet fraction is further centrifuged to obtain two further preparations: a
platelet/clotting factor (except factor VIII) fraction and plasma. Plasma, when fro-
zen and then thawed to 4˚C, results in cryoprecipitate and protein fractions.
Whole blood consists of red blood cells and plasma plus a preservative solution.
Packed red blood cells consist of red blood cells, minimal amounts of plasma and a
storage solution. Platelet fractions contain variable amounts of white blood cells and
plasma plus preservative. Fresh frozen plasma contains all coagulation factors and
plasma plus a storage solution. Cryoprecipitate consists of factor VIII, XIII, fibrino-
gen, fibronectin, and von Willebrand’s factor. Granulocyte fractions contain white
cell, plasma, and storage solution components. Plasma protein consists primarily of
albumin with lesser amounts of alpha and beta globulin (but no gamma globulin).
In addition, pure albumin solutions (5% and 25%) are commercially available.
Screening
All donor blood products must be labeled indicating the ABO grouping, and
when possible, the Rh type. The Food and Drug Administration (FDA) has man-
dated that all units for allogeneic transfusion be screened and found negative for
antibodies to human immunodeficiency virus (anti-HIV), hepatitis C virus (anti-
HCV), hepatitis B core antigen (anti-HBc) and human T-cell lymphotropic virus
(anti-HTLV) as well as hepatitis B surface antigen (HBs) and human immunodefi-
ciency virus (HIV-1) antigen. A serologic test is also performed for syphilis.
Indications for Transfusion
Few absolute criteria for transfusion exist. A better understanding of the risks
and benefits of each type of blood component enable clinicians to individualize
transfusion therapy upon established guidelines rather than old “transfusion trig-
ger” principles.
Rarely is whole blood indicated. Furthermore, few institutions maintain an active
stock of whole blood. It is reserved for acute blood loss >15-30% of total blood
volume. For cases less than this, similar results can be obtained with crystalloid/
colloid and packed red cell therapy. Packed red cell therapy is reserved for patients
with symptomatic anemia and a hemoglobin value < 6 g/dL. With autologous trans-
fusion, the criteria may be more liberal. One unit of PRBCs contains 250 cc-300 cc
and in adults raises the hemoglobin by 1g/dl or the hematocrit by 3%. In neonates
10 cc/kg is the usual initial transfusion amount which raises the hematocrit about
10%.
Platelet therapy is reserved for patients with postoperative bleeding and platelet
counts < 50,000/µL, as well as cancer/ chemotherapy patients with rapidly falling or
low platelet counts < 10,000/µL. Of note, platelet transfusions are usually ineffec-
tive in patients with thrombocytopenia secondary to destruction or circulating
autoimmune disorders, such as immune thrombocytopenic purpura (ITP) or throm-
botic thrombocytopenic purpura (TTP). One unit (approximately 50 cc) raises the
platelet count between 5-10,000 plts/µL in a 70 kg adult, and 20,000 plts/µL in an
18 kg child.
Fresh frozen plasma can be used for rapid coumadin reversal when insufficient
time is available for vitamin K reversal (approximately six hours). Other indications
include:
1. unidentifiable coagulation factor defects or coagulation factor deficien-
cies for which specific factor component therapy is unavailable,
10 2. prothrombin time > 1.5 times normal with microvascular bleeding,
3. massive transfusion with subsequent coagulopathy, and
4. conditions such as TTP. It is not used for plasma volume expansion.
Cryoprecipitate is used for specific factor deficiencies, i.e., factors VIII, XIII,
fibrinogen, fibronectin and von Willebrand’s factor. In particular, it is second line
therapy for patients with:
1. von Willebrand’s disease unresponsive to desmopressin therapy, or
2. Factor VIII:C (hemophilia A) deficiency when specific Factor VIII con-
centrate is unavailable.
Cryoprecipitate may occasionally be indicated when serum fibrinogen levels are below
80-100 mg/dL. The indications for use in patients with fibronectin deficiency are
not well defined. Another use of cryoprecipitate is to make a “fibrin glue” by mixing
it with topical thrombin to enhance hemostasis.
Albumin and plasma protein fractions are reserved purely for volume expansion.
There is currently little support for its use as a nutritional supplementation.
Granulocyte transfusions are available for patients with neutropenia and active
infection unresponsive to antibiotic therapy. A recombinant granulocyte colony stimu-
lating factor (gCSF) is also currently available.
A variety of other factors are available for specific deficiencies. Viral inactivated
Factor VIII exists for treatment of hemophilia A and von Willebrand’s disease. Fac-
tor IX (prothrombin complex concentrate) may be used for coumadin reversal and
specific factor deficiency. Activated prothrombin complex concentrate (composed
Blood Component Therapy 41
of Factors II, VII, IX, and X) is available for hemophilia A and those with Factor
VIII antibody. Rh immune globulin is available for those receiving Rh(D) positive
platelet concentrates, or pregnant/postpartum females to minimize isoimmuniza-
tion. Intravenous immune globulin (IVIG) is available to treat patients with immu-
noglobulin deficiency or TTP.
Transfusion Reactions
Immediate and delayed immunologic and nonimmunologic reactions to blood
products are well described. Immediate immunologic complications include:
1. hemolytic transfusion reactions,
2. immune-mediated platelet destruction,
3. febrile nonhemolytic reactions, and
4. allergic reactions.
Hemolysis most commonly occurs secondary to ABO incompatibility with intra-
vascular destruction of donor RBCs followed by complement activation, hypoten-
sion, diminished renal blood flow and very rarely disseminated intravascular
coagulation with multi system organ failure. Symptoms include fever, tachycardia,
chills, dyspnea, chest and back pain, and abnormal bleeding. Treatment includes
stopping the transfusion, fluid resuscitation and pressor support as necessary. In
addition, specimens are sent to the lab to check for hemoglobinuria and hemoglo-
binemia. Urine is also analyzed for Coomb’s direct antibody.
Platelet counts may be refractory to platelet transfusion. Often this occurs sec-
ondary to alloantibody directed against human leukocyte antigen (HLA) or platelet
specific antigens. It most commonly occurs in patients who have received multiple
previous transfusions. Diagnosis is suggested by a poor response noted on 10
posttransfusion platelet levels. Treatment requires HLA matched donor platelets.
Febrile nonhemolytic reactions occur secondary to antibody in the donor or
recipient blood directed against white blood cells or cytokine activation. Such reac-
tions occur in 1% of all transfusions and treatment/prevention requires antipyretics
or future transfusion with washed red cells.
Allergic reactions are heralded by the appearance of urticaria or even anaphy-
laxis. Treatment involves preadministration of antihistamine for minor reactions
and possibly even epinephrine and corticosteroids when severe.
Delayed immunologic reactions include hemolysis, alloimmunization, and graft-
versus-host disease (GVHD). Delayed hemolysis occurs from 2-14 days after trans-
fusion as a result of previous alloimmunization to red blood cell antigen. Transfused
cells may remain in the circulation for an extended period of time thus provoking an
amnestic response with fevers, diminished blood counts and development of a posi-
tive Coomb’s antibody test. Treatment is observation and the course is self-limited.
Alloimmunization is an amnestic response mediated by IgM on secondary exposure
to an antigen present on donor red blood cells. GVHD occurs when viable T-lym-
phocytes in donor blood engraft and destroy host tissue antigen. Severely
immunocompromised hosts are the most seriously threatened (i.e., fetus, bone mar-
row transplant and organ transplant patients, and those with immunodeficiency
syndromes). Symptoms include fever, rash, nausea, vomiting and diarrhea with an
increase in liver function assays and drop in cell counts. A fatality rate of up to 90%
is described. GVHD cannot be fully prevented but its risk is minimized by using
gamma irradiated blood products.
Nonimmunologic complications include infectious disease transmission such as:
cytomegalovirus (CMV), hepatitis, HIV, and rarely babesia, bartonella, borrelia,
brucella, leishmania, parvovirus, plasmodia, toxoplasma, and trypanosome. Bacte-
rial contamination occurs secondary to both gram positive or negative organisms.
Symptoms include fevers, chills, hypotension and shock. Treatment involves stop-
ping the transfusion, administering antibiotics and vasopressors, and obtaining cul-
tures. Circulatory overload syndromes present with pulmonary edema most
commonly in patients with chronic severe anemia (because of their low RBC mass
with elevated plasma volumes). These complications are avoided by regulating the
transfusion rate between 2-4 cc/kg/hr.
Massive transfusion of cold blood products can produce hypothermic complica-
tions that present with cardiac arrhythmia and arrest. These are best prevented by
using fluid warming devices < 42˚C. Metabolic complications of transfusion therapy
include: alkalosis or acidosis, citrate toxicity with subsequent hypocalcemia, hyper-
kalemia from prolonged blood storage, hypokalemia from alkalosis, diminished
2-3 DPG with subsequent leftward shift of the oxygen dissociation curve, and
hemosiderosis from chronic transfusions.
Selected Readings
1. Wolf CFW. Blood component therapy. In: Barie PS, Shires GT eds. Surgical
Intensive Care. Boston : Little Brown & Company 1993; 723-739.
2. Hutchinson RJ. Surgical implications of hematologic disease. In: O’Neill Jr. JA et
al, eds. Pediatric Surgery, 5th edition. St. Louis: Mosby 1998l 157-170.
10 3. Valeri RC. Physiology of blood transfusion. In: Barie PS, Shires GT eds. Surgical
Intensive Care. Boston: Little Brown & Company 1993; 681-721.
CHAPTER 1
CHAPTER 11
Perioperative Infections and Antibiotics

Riccardo Superina
Perioperative infections in surgical patients include postoperative wound infec-

tions and other nosocomial infections. In the following Chapter, only those infec-
tions that occur in the wound or at the operative site will be discussed. Nosocomial
infections such as hospital acquired gastroenteritis, postoperative pneumonia, or
infections at peripheral intravenous catheter sites are not considered.
Classification and Incidence of Postoperative Wound
Infection
Wound classification is used as a guide to the expected incidence of wound in-
fections. The risk for surgical wound infection depends greatly upon the degree of
contamination encountered at the time of operation.
Clean Wounds
Clean wounds are those that result after procedures that have no preoperative
infection and during which no mucosal surface is breached. Wound infections in
clean wounds are very uncommon and overall should be less than 0.1%.
Clean-Contaminated Wounds
Clean-contaminated wounds result from operations in which a mucosal barrier
has been breached but in which no infection or acute inflammation has been
encountered. The wound infection rate is less than 1%.
Contaminated Wounds
Wounds that result from operations done in the presence of acute inflammation
or active infection are considered contaminated. Wound infections occur in less
than 5% of cases.
Dirty Wounds
Dirty wounds are those wounds resulting from operations done in the presence
of pus or gross fecal contamination. Wound infection occurs in about 20-30% of
these wounds.
Etiology
Bacteria are responsible for most surgical wound infections. These pathogens
may be either endogenous or exogenous to the host. Postoperative wound infections
in clean cases usually originate from operating room personnel or from the skin of
the patient. For this reason they are usually caused by gram-stain positive organisms
such as Staphylococcus aureus or Staphylococcus epidermidis.
In the other types of surgical wounds, the bacteria causing wound infections
usually originate in the host and more specifically, in the organ or organs being
operated upon. For this reason, operations on the intestines are complicated by
postoperative infections caused by gram-negative bacteria. Wound infections fol-
lowing appendicitis are frequently caused by aerobic or anaerobic colonic organ-
isms. Respiratory flora may be present in wounds after pulmonary resections.
Patients who have been in a hospital for prolonged periods of time become colo-
nized with bacteria that have acquired resistance to many antibiotics. Infections
with multi-resistant organisms are more difficult to treat. Examples of such organ-
isms include methicillin-resistant Staphylococcus aureus (MRSA) and Enterococci
with resistance to vancomycin and ampicillin.
Moderate or severely ill patients acquire defects in neutrophil function that play
a permissive role in the development of postoperative infections. These defects include
abnormalities in neutrophil migration, intracellular killing and phagocytosis. Fac-
tors such as malnutrition, sepsis and trauma all impair host neutrophil defenses. B
and T lymphocyte function is also impaired in very sick patients and contributes to
the higher than expected incidence of postoperative sepsis observed in these patients.
Newborns are considered immunologically challenged hosts. Neonates have
immature bacteriostatic and bactericidal defense mechanisms which place them at
greater risk for postoperative infections than older sick children.
Clinical Presentation
Wound Infections
11 The findings of redness, tenderness, heat, and swelling at the operative site all
suggest postoperative wound infection. Fever may or may not be present. Typically
wound infections develop 3-5 days after operation, but this is quite variable. Viru-
lent anaerobic streptococcal infections may cause exquisite pain at the operative site
within 24 hours after operation, whereas slow growing Staphylococci may present a
week or more after surgery.
Deep Infections
Deeper infections in the chest and abdominal cavity present with fever and pain.
In children, accurate description of subjective complaints is not often possible, and
reliance on the parents’ interpretation of a child’s behavior and mood is often a very
useful guide to assessing a child’s recovery after surgery.
A postoperative ileus that does not resolve after an abdominal procedure should
lead one to suspect infection. Abdominal tenderness or redness may indicate an
underlying infection. Persistent cough, pleuritic chest pain and tachypnea may indi-
cate an intrathoracic infection after chest procedures.
Diagnosis
Culture
If a deep postoperative infection is suspected, the diagnosis is established by
careful culturing of peripheral blood or wound fluid at the operative site. Postoperative
Perioperative Infections and Antibiotics 45
drains such as chest tubes and intra-abdominal drains may provide samples that if
cultured act as a guide to antibiotic selection.
Laboratory Tests
Biochemical and hematologic tests support but do not prove the presence of an
infection. An elevated white blood cell count or “left shift” (less mature leukocytes
in the differential white blood cell count) support the diagnosis of infection. How-
ever, the absence of these indicators does not mean an infection is not present.
Diagnostic Imaging
Plain films sometimes provide valuable data regarding infected postoperative
fluid collections. Chest films of patients with intrathoracic infections often show
pleural effusions, air fluid levels in the chest, or subpulmonic collections. Plain films
are less helpful at localizing collections in the abdomen but may demonstrate other
nonspecific signs such as abnormal gas patterns (i.e., ileus), air-fluid levels, or dis-
tended loops of bowel.
Ultrasonography and computed tomography (CT) provide accurate informa-
tion regarding the presence of fluid collections in the abdomen. Ultrasonography
can accurately determine size and complexity of intra-abdominal fluid collections.
The echogenic characteristics of the fluid can often indicate whether infection in
the fluid is likely. Computed tomography (CT) with intravenous contrast may show
contrast enhancement at the periphery of a fluid. Contrast enhancement is com-
mon with infected fluid collections and abscesses. Radionucleide scans such as gal-
lium scans may be helpful when all other modalities fail but are rarely necessary.
Treatment
Antibiotic Prophylaxis 11
Antibiotic prophylaxis as a means of preventing postoperative infection is a con-
cept that was introduced and developed in the 1960s by Sir Ashley Miles. In theory,
an early critical period of a wound infection exists that determines the outcome of
the infection. The conditions in the wound present at the time of the inoculation
with bacteria determine the fate of the infection. If antibiotics are administered so
that tissue levels are present at the time bacteria are introduced into a wound, then
infection can be prevented. If bacteria are inoculated into a site where there is no
protection and in which conditions are suitable for growth, then infection and sepsis
ensue.
Prophylactic antibiotics to prevent wound infections must be administered prior
to the operation so that adequate bacteriostatic levels will be present at the time
bacteria are introduced. It is recommended that intravenous antibiotics be adminis-
tered at least 30-60 minutes before the incision is made. The choice of antibiotics is
determined by:
1. the site of the operation and
2. the type of bacteria which are likely to be encountered.
Prophylaxis for clean operations has never been proven to be beneficial except in:
1. immunocompromised hosts,
2. patients at risk for endocarditis, and
3. patients in whom an artificial device such as a vascular prosthesis will be

implanted.
Recommendations for prophylactic antibiotic therapy to prevent postoperative
infection are listed in Table 11.1.
Wound Closure for Dirty Cases
In adult general surgery, it is customary to leave the skin and subcutaneous tis-
sues open after completion of a dirty or contaminated case. In pediatric cases, it is
less customary to do so. Children have fewer postoperative wound infections than
adults. This may be mainly because children have less subcutaneous fat and better
vascularity to subcutaneous area.
Established Wound Infections
Treatment of established wound infections requires drainage and, in some cases,
antibiotic administration. For infections in which there is copious pus and little
surrounding inflammation, drainage may be all that is necessary. The host has already
contained the infection. If the wound is already partially opened, drainage may
already be started and no additional manipulation may be needed. For closed wounds,
aspiration with a narrow gauge needle may be attempted under mild sedation and
infiltration with local anesthesia. If pus is aspirated, then a portion of the wound
may be opened and pus expressed. Irrigation and packing may be necessary to pro-
mote growth of granulation tissue and secondary wound closure.
Surgical wounds with purulent drainage, tissue edema, and cellulitis, are at least
partially opened and broad-spectrum antibiotics are started. Necrotizing wound
infections are always kept in mind. If symptoms do not improve after 12-24 hours
as manifest by decrease in the area of redness and swelling, then wound exploration
under anesthesia is considered. All necrotic tissue is removed and proper cultures are
11 taken from deep inside the infected area. In the most severe cases, widespread resec-
tion of infected areas as well as high-dose antibiotic therapy are necessary for control
of the infection.
Deep Infections
The treatment of deeper postoperative infections is guided by the location of the
infection, the threat it poses to the host, and the presence of drainable infected fluid.
When an abscess has formed, no matter where, drainage is mandatory. Localized
collections may be drained by interventional radiologists who can also insert cath-
eters for continued drainage. Postoperative peri-appendiceal infections may be treated
very well using this technique and have all but eliminated the need for repeated
laparotomies after appendectomy. Drainage will also permit the collection of samples
for culture. Antibiotic therapy is recommended even when there is a drainable col-
lection until fever has subsided and the white blood cell count is returning to the
normal range.
Treatment of postoperative infections with antibiotics implies that the infection
is serious and will spread unless treated. Therefore, broad-spectrum antibiotic therapy
is recommended until there has been a response or until cultures have identified an
organism allowing sensitivity testing to determine choice of drugs.
Perioperative Infections and Antibiotics 47
Table 11.1. Recommendations for prophylactic antibiotic therapy in surgical

patients
Site of Operation Recommended Antibiotics
or Antibiotic Therapy
Head and Neck penicillin and gentamicin

Lungs and Trachea cefazolin
Biliary Tree and Liver cefazolin
Small Bowel ampicillin and gentamicin OR
cefoxitin
Large Bowel ampicillin, metranidazole, and
gentamicin OR cefoxitin
Genitourinary Tract ampicillin
Operations on Newborn Babies ampicillin and gentamicin for 48

hours prior to operation
Patients at Risk for Endocarditis penicillin and gentamicin before
surgery and for 2 doses following
surgery
Subcutaneous Reservoir Placement cefazolin before surgery and for 2
doses after surgery
Abdominal infections originating from the gastrointestinal tract are treated with
antibiotics against gram-positive and gram-negative organisms as well as anaerobes.
A useful combination is so called “triple therapy” with metranidazole, ampicillin
and gentamicin. Clindamycin is preferred by some instead of metranidazole because
of its better gram-positive coverage. For patients with renal impairment, it may be
better to substitute a third generation cephalosporin instead of an aminoglycoside
(i.e., gentamicin) to cover gram-negative bacteria. 11
Biliary tract sepsis requires coverage against Enterococci as well as Enterobacter
species and therefore includes ampicillin and an aminoglycoside. Vancomycin is
substituted for cases in which ampicillin-resistant organisms are possible until sensi-
tivity results are available.
Thoracic postoperative infections are less prevalent than abdominal ones, prin-
cipally because the intestines are a vast reservoir of bacteria. Infections following
lung resections for bronchiectasis or in patients with cystic fibrosis may be difficult
to treat. Postoperative empyemas require drainage with large bore chest tube(s) and
prolonged treatment with antibiotics. Antibiotic therapy is tailored to specific cul-
ture results and sensitivities.
Surgical Re-Exploration
Postoperative infections, no matter what location, may require re-operation if
more conservative measures fail. Failure of conservative treatment is indicated by:
1. continued fever,
2. persistent leukocytosis, and
3. lack of clinical improvement in the patient. A continued septic state in
association with radiologic evidence of undrained fluid usually mandates
re-exploration.
Infection of a Central Venous Catheter or Port

Once established, infection of a catheter with or without a subcutaneous port is
difficult to eradicate without removal of the device. Urokinase can be administered
to facilitate resolution of the infection by dissolving clot at the catheter (nidus of
infection). Then treatment with antibiotics for 2–3 weeks may eradicate infection if
the patient remains afebrile and blood cultures are negative during the period of
treatment. In-patients who receive chemotherapy and have leukopenia are always
prone to infection. At least some efforts should be made to salvage intravascular
devices in these high risk patients, since removal requires another operation and
offers no guarantee that a new infection will not occur. Prophylactic antibiotics
should always be considered before insertion or implantation of a central venous
catheter and subcutaneous port.
Yeast Infections
While uncommon, yeast infections are not rare in pediatric surgical patients.
Patients who are malnourished, debilitated and often treated with a number of pow-
erful antibiotics are at risk for development of yeast infections. Candida is the most
common yeast isolated in the pediatric surgical patients. Candida is much more
difficult to culture than bacteria, and therapy is commonly delayed for that reason.
Any patient suspected of having a serious postoperative infection, yet not im-
proving on antibiotic therapy, should be examined for Candida infection. Cultures
of the wound, drains and urine are done. Ophthalmologic examination of the fun-
dus and ultrasound examination of the ureters and kidneys may also be done in an
attempt to detect evidence of Candida infection.
Amphotericin remains the treatment of choice for serious Candida infections.
Serious infections should be treated for 14-21 days. Fluconazole may be used in less
11 serious cases, particularly if renal function is already impaired. Liposomal amphot-
ericin formulation is also available for use in selected patients with impaired renal
function.
Summary
Children are very resistant to wound infections. Antibiotics are a powerful ally
in the treatment of infections, but must be used judiciously and only when neces-
sary. Antibiotic therapy is based on culture results whenever possible, but are not
withheld if clinical signs indicate an infection that cannot be proven or characterized.
Selected Readings
1. Nichols RL. Postoperative infections in the age of drug-resistant gram-positive
bacteria. Amer J Med 1998; 104:11S–16S.
2. Hunt TK, Hopf HW. Wound healing and wound infection: what surgeons and
anesthesiologists can do. Surg Clin N Amer 1997; 77:587–606.
3. Nichols RL. Surgical infections: Prevention and treatment—1965 to 1995. Amer
J Surg 1996; 172:68–74.
Section III: Common Pediatric Surgical
Problems
CHAPTER 12
Inguinal Hernia and Hydrocele

Juda Z. Jona
Incidence
Hernias and hydroceles are among the most common pediatric surgical prob-
lems. The incidence of indirect inguinal hernia in the term neonate is 3.5-5%. Pre-
mature infants have a higher incidence of approximately 9-11%. Inguinal hernias
are more common in boys (male: female ratio is 5:1 to10:1). Sixty percent of inguinal
hernias occur on the right side, while about 30% occur on the left. Ten percent
occur as bilateral hernias. Bilateral hernias are more common in premature infants
(45-55%) and females. Indirect inguinal hernias and hydroceles are known to have
familial tendencies, but true heredity factors have not been clarified.
Etiology
The processes vaginalis is an elongated diverticulum of the peritoneum which
accompanies the testicle upon its descent into the scrotum. It pierces the anterior
abdominal wall at the deep (internal) inguinal ring which is located just lateral to
the deep inferior epigastric blood vessels. In most individuals, the processes obliter-
ates during the ninth month of intrauterine life or soon after birth. If that channel
remains open, intraperitoneal fluid will slowly accumulate in the structure forming
a communicating hydrocele (also known as hernia/hydrocele) If the processes is
wide enough, intestines, ovaries, or omentum can herniate into the inguinal canal
forming an indirect hernia. Should the processes vaginalis obliterate near its origin
but remain patent distally fluid may accumulate forming a noncommunicating
hydrocele. If the processes obliterates proximally and distally but remains patent in
its mid portion then it is known as hydrocele of the cord .
Direct inguinal hernias are occasionally identified in children. The abdominal
wall defect is in the floor of the inguinal canal within the confinement of Hasselbach’s
triangle. Anatomically, Hasselbach’s triangle is that area bordered superolaterally by
the inferior epigastric vessels, inferiorly by the inguinal ligament, medially by the
rectus abdominus muscle. Direct inguinal hernias are believed to occur secondary to
structural weakness. Femoral hernias occur inferior to the inguinal ligament within
the femoral canal, just medial to the femoral vessels and are extremely rare in children.
A bulge (swelling) in the groin which at times may extend into the scrotum is by
far the most frequent sign. The bulge may appear and then disappear with some
regularity especially during straining, crying, or coughing. Although sharp pain is
Inguinal Hernia and Hydrocele 51
usually not associated with herniation, discomfort that occurs in some babies is
easily overlooked. Occasionally constipation, “colicky-baby” syndrome, and even
regurgitation are present.
In the very young, the initial presentation may be an episode of incarceration. In
this scenario, the baby is more symptomatic, the bulge is firm and tender to touch,
the groin and scrotum may be erythematous, and vomiting or poor feeding are
frequent. A history of recurring groin swelling which the parents or the pediatrician
can reduce is a strong indication that a hernia is present.
Diagnosis
The physical examination in many is so characteristic that only observation is
necessary to make the diagnosis. The examiner palpates the cord to ascertain if bowel
or other structures are present. Diagnostic confirmation is made when the contents
of the hernia are reduced into the peritoneal cavity. Hydroceles, even the communi-
cating variety, are difficult to reduce though many reduce spontaneously when the
child is recumbent over several hours. Many physicians resort to transillumination
to distinguish a hydrocele from a hernia containing bowel. However, this may be
misleading, particularly in infants, since a hernia with gas-filled bowel loops may
transilluminate quite well. If the child presents with a reduced hernia, secondary
changes can be found that suggest presence of a hernia. Palpation of the cord may
elicit the “silk glove” sign (rubbing together of the opposing peritoneal membranes
of the empty sac). The cord may feel thickened in comparison to the contralateral
side. Increases in intra-abdominal pressure (i.e., coughing, crying, exhaling against
an occlusion such as thumb in mouth, blowing bubbles, etc) may help demonstrate
the hernia.
In the face of a suggestive history but no concrete findings, repeated examina-
tion in 2-3 weeks is recommended. The parents should continue observation, be
taught how to reduce the hernia and, at times, even resort to photographing the
hernia so that a definite diagnosis can be established.
Complete history and physical examination may reveal other unrecognized con- 12
ditions. The genitalia and the testicles must be carefully examined. At times, a retractile
testis presents as an inguinal bulge and appears to be a hernia. Undescended testes
are commonly (85%) associated with indirect hernia, and the two conditions are
repaired at the same time. Occasionally, the differential diagnosis includes inguinal
or femoral adenopathy. If an adenitis has progressed to an abscess, the findings may
be difficult to distinguish from an advanced stage of incarcerated inguinal hernia. In
this scenario, immediate surgical exploration is undertaken for both diagnosis and
treatment.
When incarceration is suspected, plain abdominal films are helpful and demon-
strate an obstructive/ileus bowel gas pattern and intestinal gas in the groin and scro-
tum. Plain films are also useful to distinguish between an acute hydrocele, for which
an operation can be delayed, and incarceration which requires immediate attention.
When in doubt—operate! Untreated incarceration leads to bowel necrosis and/or
testicular ischemia.
Treatment
The reason for repairing an inguinal hernia is to prevent incarceration. Since the
incidence of incarceration is inversely related to age, the younger the patient—the
sooner the repair. Premature babies should have their hernias repaired just prior to
discharge from the hospital. Asymptomatic school age children can be repaired when
school is in recess. The timing of repair is less clear with hydroceles. In most centers,
hydroceles are not repaired until the baby is 12-18 months or older. Approximately
90-95% of all hydroceles resolve spontaneously in the first few months of life. If a
hydrocele becomes very large and tense, earlier repair can be considered. If a hydro-
cele cannot be differentiated from a hernia, operation is indicated.
The operation is an outpatient procedure performed under general anesthesia.
Infants less than 60 weeks postconception and children with associated conditions
(i.e., cystic fibrosis, hemophilia, etc.) Need admission for 24 hours of observation.
The recommended steps of inguinal hernia repair are listed in Table 12.1.
Postoperative care is straightforward. Since intradermal absorbable sutures are
used for wound closure, most of these patients can start routine bathing within
24-48 hours. No restriction on diet or activities is given. Tylenol for analgesia is all
that is required. In the older children, ibuprofen or codeine may be necessary. Patients
with long-standing hernias, large hydroceles or formation of fibrous adhesive tissue
around the cord may experience induration in the operative area that eventually
subsides. These children deserve extended follow-up as occasionally the testicle on
the affected side may be drawn out of the scrotum and requires secondary orchidopexy.
Outcomes
Recurrent herniation is rare and is seen in less than 1% of cases. More often,
residual or posttraumatic hydroceles may be noted. If they do not resolve after sev-
eral months, aspiration of their contents may expedite resolution.
Special Considerations
12
Contralateral Exploration (CLE)
The issue regarding the merits and objections to routine CLE in pediatric inguinal
hernia is still not resolved among pediatric surgeons. Since the incidence of bilateral
involvement in the first year or two of life is high (20-40%), most surgeons at pedi-
atric centers perform CLE. Some surgeons extend CLE to mid-childhood (6 years
old). The author’s experience and many studies support exploration in all preschool
children. In certain instances (i.e., strong family history, complicating circumstances
like hemophilia or cystic fibrosis), exploration is offered to even older children.
Resistance to this practice has been voiced by some European surgeons who report
unacceptable complication rates. Either way, the family must understand the pros
and cons and be an integral part of the decision making.
Operation for Incarcerated Hernias
If clinically the patient presents with peritonitis and the picture of sepsis, stran-
gulation must be suspected. Antibiotics and massive preoperative resuscitation (rapid)
is done. Initially, standard inguinal exposure is carried out, although the abdomen is
prepped widely. The sac that is commonly edematous is freed from the cord structures.
Inguinal Hernia and Hydrocele 53
Table 12.1. Guidelines for inguinal hernia repair

1. The surgical prep includes the lower 1/2 of the abdomen, genitalia, and
upper thighs.
2. The incision is relatively short and is positioned in an inguinal skin crease at
the level of the internal inguinal ring.
3. The aponeurosis of the external oblique is exposed and followed inferiorly
until definite identification of the inguinal ligament is made.
4. The inguinal ligament is followed medially towards the pubic tubercle and
the external inguinal ring is clearly identified.
5. The external oblique is divided along its fibers so as to transect the external
ring, thus exposing the contents of the inguinal canal. (In small children this
step may be omitted and the hernia repaired without opening the ring.)
6. Near the pubic tubercle, the cord and hernia sac are elevated with atraumatic
forceps and encircled. Care is taken not to pierce the inguinal floor and
produce a direct hernia.
7. A curved Kelly clamp is passed under the cord and sac upon which they now
rest.
8. Careful separation of any creamasteric fibers allows identification of the sac
that is gently grasped and elevated. With the sac pulled up and medially, the
vas and vessels are exposed and gently teased off the sac. The vessels come
off easily. The vas is quite intimate with the sac and is always an extraperi-
toneal structure.
9. Once freed, the cord structures are encircled with a narrow penrose drain in
older children (or an Allis clamp in the very young) to protect these structures.
10. If the sac is empty, it can be divided between clamps and each section freed
separately. The proximal sac is held at some tension and with gentle pull of
the cord structures the areolar tissue between the two is identified and
cleared either bluntly with forceps or sharply with scissors. As one reaches
the internal ring, the lip of the internal oblique muscles is retracted so that
high ligation of the sac can be done.
11. The neck of the sac is suture ligated with either an absorbable or nonabsorb-
able suture. The floor of the canal is inspected. On rare occasions, additional
sutures are required to narrow the internal ring. 12
12. The distal sac is opened. If a hydrocele of the testis is present it is opened.
The testicle is drawn back into the scrotum by gentle pull on the scrotum.
13. Once hemostasis is assured, the external ring, if opened, is reconstructed and
the external oblique is gently approximated. At this point, 0.25% marcain is
instilled into the wound and subcutaneous tissue for postoperative pain
control Make sure that the testicle is repositioned in the scrotum properly.
14. The skin and scarpa’s fascia are closed.
The sac is opened and the bowel inspected. If viability is established (immediately or
after a period of observation), the bowel is returned to the peritoneal cavity. Widen-
ing of the internal ring may be required at times. If strangulation is the case, a
Laroque type (RLQ) incision is made and the peritoneum entered. It is wise to
isolate the bowel entering and exiting the hernia such that the intestinal spillage is
minimized. Formal resection requires direct anastomosis in most instances unless
the patient is most unstable. The hernia ring can be closed either from the inguinal
or the peritoneal side. Antibiotics are administered for 48 hours or until the ileus
resolves.
Femoral Hernias
Femoral hernias occur inferior to the inguinal ligament, along the femoral canal,
medial to the femoral vessels. Femoral hernias are extremely rare in childhood but
are more common in females. The diagnosis is most difficult in the very young and
in those with excessive adipose tissue. In the lean and cooperative patient, a bulge is
noted below the inguinal crease. Unfortunately, most are diagnosed after a routine
inguinal exploration in which only a small indirect hernia or no hernia was found.
In these cases, the femoral canal must be explored. This is accomplished by incising
the transversalis fascia in the Hasselbach’s triangle close to the Poupart ligament.
The hernia will be identifiable medial to the femoral vein. The Cooper’s ligament
repair is the treatment of choice. If the diagnosis is made preoperatively, a subin-
guinal (femoral canal) approach can be used in which the space medial to the vessels
is eliminated after reduction of the hernia and its contents.
Selected Readings
1. Lloyd DA, Rintala RJ. Inguinal hernia and hydrocele. In: O’Neill Jr. JA et al, eds.
Pediatric Surgery, 5th Edition St. Louis: Mosby 1998; 1071-1086.
2. Potts WJ, Riker WL, Lewis JE. The treatment of inguinal hernia in infants and
children. Ann Surg 1950; 132:566.
3. Rowe MI, Marchildon MB. Inguinal hernia and hydrocele in infants and children.
Surg Clin North Am 1981; 51:1137.
4. Surana R, Puri P. Is contralateral exploration necessary in infants with unilateral
inguinal hernia? J Pediatr Surg 1993; 28:1026.
5. Rajput A, Gauderer MWL, Hack M. Inguinal hernias in very low birth weight
infants: incidence and timing of repair. J Pediatr Surg 1992; 27:1322.
12
CHAPTER 1
CHAPTER 13
Varicocele
Juda Z. Jona
Incidence
Varicocele results from dilation of the testicular veins within the pampiniform
venous plexus. It is rare in children less than 10 years of age. At adolescence, the
incidence is 10-15%.
Etiology
Varicocele occurs because of increased hydrostatic pressure within the gonadal
veins. This may be due to either incompetent venous valves or venous obstruction.
Its relative greater occurrence on the left side is related to the fact that the left go-
nadal venous drainage is to the left renal veins rather than directly to the IVC as
occurs on the right side.
Varicocele occurs predominately in postpubertal teenagers and young adults and
occurs almost exclusively on the left side (80-90%). Most boys describe enlarge-
ment of the cord and testicle after physical activities, coughing or straining. The
lesion is a complex elongated “cord,” at times, described as a “tangle of worms.”
Standing and straining may demonstrate the varicocele. Tenderness is not a com-
mon finding. With the patient supine and resting, the swelling will recede. The
differential diagnosis includes hernia, hydrocele, and tumor. Diagnostic testing is
rarely required for varicocele.
Younger children with Wilms’ tumor, neuroblastoma, or hydronephrosis may
present with a varicocele which is caused by obstruction of venous return from the
testis. In cases of right-sided varicocele (1-7%), the presence of a retroperitoneal
mass should be considered. Bilateral lesions occur in 2-20% of patients.
Pathophysiology
A varicocele increases blood surrounding the testis, thereby raising the testicular
temperature as heat dissipates from the venous blood. Spermatogenesis is decreased
with an increase in testicular temperature. In addition, hormone-like substances
secreted by the affected testicle may adversely influence contralateral testicular
function.
Fig. 13.1. The tortuos, venous channels of a varicocele are easily visualized in the
left scrotal sac. This child proved to have a Wilms’ tumor involving the left kidney.
Treatment
Operative interruption of the gonadal vein is curative. Traditionally, this is car-
ried out through an inguinal exposure. Recent reports suggest that these patients
can be successfully treated laparoscopically. Using this technique, the main gonadal
vein trunk is interrupted in the retroperitoneum. Some surgeons ligate the gonadal
artery as well (with the hope of reducing the recurrence rate) and rely on the collat-
eral blood supply (perivas, gubernacular and pudendal vessels) to maintain testicu-
lar viability. This technique is known as the Fowler-Stevens maneuver, which is
commonly used to treat intra-abdominal cryptorchidism.
13
Outcomes
Most varicocele operations are successful and in many fertility is preserved or
restored. The risk of recurrence after operative treatment is between 5% and 45%.
Reactive hydrocele occurs in about 10-35%. Other surgical complications include
testicular atrophy, nerve injury, and injury to the vas deferens. If recurrence occurs,
contrast radiography or ultrasound may be required to demonstrate the site of re-
currence.
Selected Readings
1. Palomo A. A radical cure of varicocele by a new technique: Preliminary report. J
Urol 1949; 61:604.
2. Hutson JM. Undescended testis, torsion, and varicocele. In: O’Neill Jr. JA et al.
eds. Pediatric Surgery, 5th Edition. Vol. 2. St. Louis: Mosby 1998; 1101-1105.
CHAPTER 1
CHAPTER 14
Testicular Torsion
Juda Z. Jona
Incidence
The incidence of this condition is hard to estimate, since many occur antenatally.
Torsion can strike at any age, however, it is most prevalent in the early teen years.
Etiology
There are two variations of this condition. Extravaginal torsion is more common
in fetuses and neonates and denotes torsion of the spermatic cord along its course
but outside the tunica vaginalis. Most babies born with an absent (“vanishing”)
testes are victims of this type of prenatal torsion. Absence of testicular fixation is
believed to predispose the testicle to this type of torsion. The other variety, intrav-
aginal torsion, is more common in childhood and occurs within the tunica vaginalis.
In these cases, fixation of the tunica vaginalis is to the cord and not the testicle. The
testicle is suspended within the tunica vaginalis causing a “Bell-Clapper” deformity.
Contraction of the cremasteric muscle initiates testicular rotation in these predis-
posed testicles.
Prenatal testicular torsion will present with an empty scrotum. Exploration will
reveal an interrupted spermatic cord and absent testes. Neonates with unilateral
cryptorchidism may have had an in utero torsion. Perinatal testicular torsion will
present as a tender mass in the scrotum. Others may present with a red, firm, tender
mass of the groin area which may not be distinguishable from an incarcerated her-
nia. Older children and teenagers will present with a sudden onset of severe testicu-
lar pain followed by local swelling and firmness with radiation of pain to the ipsilateral
groin and lower abdomen. A history of similar short-lived events may suggest previ-
ous episodes of torsion that spontaneously resolved.
On examination, the child is restless and in obvious pain. On inspection, the
involved testicle is “high-riding”(Fig. 14.1). Later the scrotum becomes swollen, red
and tender. Careful and gentle palpation of the cord and scrotal contents may yield
a clue to the diagnosis. In epididymitis, the tenderness will be selectively located in
the posterior zone of the testicle and may extend along the distal spermatic cord
(best examined against the pubic tubercle). In addition, pyuria may be found. In
cases of torsion of the appendix testes, transillumination may demonstrate a “blue
Fig. 14.1. Left-sided testicular torsion demonstrated by swelling and high position-
ing of the usually lower left testis.
dot” sign on the superior aspect of the testicle. This site will be tender, but other-
wise, the testicle and the cords are uninvolved.
Diagnosis
In most instances, history and gentle examination will yield the correct diagno-
sis. If the diagnosis is unclear, ultrasonography with doppler and/or technetium
scanning are indicated. Ultrasonography is portable, ubiquitous and inexpensive.
Unfortunately, it requires pressure on the tender scrotum and is very operator
dependent. Radioisotope scanning is the most sensitive test, but may not be readily
available, is time consuming and is relatively expensive. Both tests confirm the diag-
nosis of torsion by demonstrating cessation of blood flow to the involved gonad.
Nuclear scans are more specific and can distinguish testicular torsion from inflam-
14 mation, epididymitis or torsed appendix testes.
Pathophysiology
Torsion results in testicular ischemia. A rotation of 270˚ or greater will impair
blood supply to the gonad. If treatment is not promptly instituted (i.e., within
6-8 hours), ischemic gangrene of the testicle can occur. Spermatagonia are more
sensitive to blood flow and oxygen deprivation than Sertoli/Leydig cells, so that in
subacute cases androgen production may be preserved but spermatogenesis impaired.
Treatment
Immediate surgical exploration is the treatment of choice. Delay for confirma-
tory ultrasound or radionucleotide scanning is not indicated. Exploration is through
a scrotal, median raphe incision. The affected testicle is inspected, detorsed, and
placed in a warm sponge. Up to 30 minutes observation is acceptable. If doubt
Testicular Torsion 59
remains regarding viability, the testis is incised to determine the presence of bleed-
ing. The flow of the tunica may be restored to some extent while the parenchyma
remains underperfused. Debate ranges regarding the treatment of marginally viable
testicle. Some replace it into the scrotum and follow testicular size. If atrophy or
hypoplasia ensue, the testicle is removed. Others, fearing adverse effects on the nor-
mal testicle (i.e., autoimmunization to spermatogonia), will perform orchiectomy
at initial exploration. In all cases, the contralateral testicle is fixed in the scrotum
with 4 sutures, also through a scrotal incision. In infants, an inguinal incision may
afford some advantages in cases of questionable diagnosis (i.e., incarcerated hernia)
or if the torsion has occurred in an undescended testicle.
Outcomes
Postoperative care in these children is not complicated; however, a torsed testicle
may atrophy with time. Long-term follow-up is imperative. Testicular salvage rates
are directly proportional to duration of torsion. For torsion less than 6 hours, 85-
97% can be salvaged. If the duration of torsion exceeds 24 hours, the chance of
salvaging the testes is less than 10%.
Selected Readings
1. Noseworthy J. Testicular torsion. In: Ashkraft KW, Holder TM, eds. Pediatric
Surgery, 2nd Edition. Philadelphia: W.B. Saunders Company 1993; 505-600.
2. Hutson JM. Undescended testis, torsion, and varicocele. In: O’Neill Jr. JA et al,
eds. Pediatric Surgery, 5th Edition, St Louis: Mosby 1998; 1099-1101.
14
CHAPTER 15
Cryptorchidism: The Undescended Testes

(UDT)
Juda Z. Jona
Incidence
Undescended testes afflicts about 1% of males.
Etiology
The testes develop from the primitive urogenital ridge and differentiate into
gonads. Then they descend, migrating in the retroperitoneum toward the internal
inguinal ring guided by hormones and the gubernaculum. At about 20 weeks gesta-
tion, the testes emerge from the external canal. By 36-38 weeks, most “arrive” in the
scrotum. Failure of descent is due to mechanical obstacles, improper gubernacular
attachment or function, or intrinsic abnormality of the testis. UDT is seen with
other anomalies including anorectal and genitourinary anomalies.
Empty scrotum and inguinal mass are common presenting complaints. It is im-
portant to know if the testis was seen or felt in the scrotum. The examination must
be performed in a comfortable and warm environment. Visual inspection is particu-
larly helpful, since any tactile stimulation of the lower abdomen, upper thighs or
genitalia may evoke cremasteric muscle response and retract the testes out of the
scrotum. If a mass is seen in the groin, it should be assessed carefully for size, shape
and mobility. With constant mild traction, a retractile testis can be coaxed back into
the scrotum. The abdomen, upper thighs and perineum must be palpated to ascer-
tain the presence of an ectopic testis. If no gonad is perceived, more careful exami-
nation against the pubic tubercle may demonstrate a “cord” (vas) which suggests an
atrophic testis. If no remnant of testicular structures are found, abdominal ultra-
sound may show the absence of the ipsilateral kidney. If absent, the testes is assumed
to be absent and surgical exploration is deferred. The evaluation of a phenotypic
male without any obvious testes may denote bilateral cryptorchidism or an intersex
problem. These infants require more urgent evaluation including chromosome analy-
sis, hormone and steroid assays, and more advanced imaging. Laparoscopy is par-
ticularly useful in these situations for diagnosis and initiation of therapy (Fig. 15.1).
Cryptorchidism: The Undescended Testes (UDT) 61
Fig. 15.1. Laparoscopic view of left undescended testis (large arrows) near the in-
ternal ring (smallest arrows). The vas is seen medially (medium arrows) and the
umbilical artery is seen along the anterior abdominal wall (curved arrow).
Treatment
Unless bilateral, cryptorchidism can be addressed when the baby has passed his
first birthday. However, an associated inguinal intervention (85%), may mandate
earlier intervention. In such situations, UDT should be corrected at the time of
hernia repair. Definitive correction of UDT best done between 1-2 years of age. By
that time, delayed descent can be noted, precise examination is easier to perform,
and a period of observation by parents and pediatricians will determine that the
problem is UDT rather than retractile testis. Delay beyond two years of age is un-
warranted.
The indications for surgery include:
1. the prevention of torsion,
2. reducing the risk of trauma,
3. repair of an associated hernia,
4. improving fertility, 15
5. examination for tumor, and
6. cosmesis.
Operation is outpatient with general anesthesia. If the testicle is not papable,
laparoscopy should be performed (Fig. 15.2). If the testicle is at or near the internal
ring, inguinal exploration is performed. If the testicle is intra-abdominal, the tes-
ticular vessels may be divided and the testicle is brought down as a second stage. The
important steps of orchiopexy include adhesion lysis, herniorraphy, mobilization/
transposition of the vessels, and formal orchiopexy.
If the testis cannot reach the scrotum comfortably, it should be sutured to the
pubic tubercle and a second stage orchidopexy is performed at 6-12 months.
Fig. 15.2. Laparoscopic view of left undescended testis (single arrow) with testicu-
lar artery and vein clipped (curved arrows) in preparation for subsequent pull-
down procedure.
Outcomes
Postoperative visits ascertain wound healing, presence of infection or hematoma,
and follow the size and position of the testicle. The parents and pediatrician are
instructed to perform frequent examinations. Complications of orchidopexy include
injury to the vas deferens or testicular vessels (1%), testicular atrophy (< 8%) and a
high-riding testicle (5-10%). Fertility after orchidopexy for unilateral UDT is 95%,
but only 70% for bilateral UDT. The risk of testicular malignancy in patients with
a history of cryptorchidism is 5-10 times greater than the normal population. The
risk is greater for bilateral cryptorchidism and intra-abdominal testes.
Selected Readings
1. Hutson JM. Undescended testis, torsion, and varicocele. In: O’Neill, Jr. JA et al,
eds. Pediatric Surgery, 5th Edition. St. Louis: Mosby 1998; 1087-1089.
15 2. Fonkalsrud EW, Mengel W. The undescended testes. Chicago: Year Book Medical
Publishers 1981.
3. Scorer CG. The descent of the testis. Arch Dis Child 1964; 39:605.
CHAPTER 1
CHAPTER 16
Circumcision
Lars Göran Friberg and Juda Z. Jona
Few topics generate as much controversy as whether a child should undergo

circumcision or not. In a global sense, this is the most common surgical procedure
performed on children.
Male Circumcision
In the male child, circumcision means resection of the penile foreskin via an
encircling skin incision made in the foreskin proximal to the corona. The indication
for this is cultural (often religious) or rarely to relieve obstruction due to phimosis.
Although it is essentially a cosmetic procedure, considerable effort has been under-
taken to prove the benefits of circumcision in the newborn period. The results of all
these studies are scientifically very questionable. Complications include bleeding,
infection, and meatal stenosis.
The role of the male foreskin is to protect the glans and the meatus. After pu-
berty the foreskin lessens the friction during sexual intercourse. These properties are
lost after circumcision. The care of the male foreskin should be no touch except for
the boy himself or his future sexual partner(s). There is no need to cleanse the inside
of the foreskin before puberty. The same goes with girls, where there is no need at all
to clean the vaginal cavity.
There have been a considerable number of reports published concerning the
retractibility of the male foreskin at different ages. At birth the foreskin seldom
retracts but if the meatus is clear allowing free urination, there is no problem. Sepa-
ration begins when infants experience erection and continues with increasing fre-
quency to puberty. By teenage years, retraction should be sufficient to allow good
hygiene, free and easy urination, and comfortable intercourse. Full retractibility is
by nature desired only when reproduction starts.
Female Circumcision
In the female child, circumcision means resection of the external genitalia. This
can be done in different ways. In the mildest form, only the tip of the clitoris is
resected, but in the Faraonic circumcision the whole clitoris, the minor labia and the
major labia are extirpated and the opening is sewn together just permitting voiding
and passing of menstrual blood. The indication for female circumcision is for cul-
tural initiation, to be a part of the female society. It is predominantly performed in
some parts of Africa.
There is no medical indication for female circumcision. The complication rate is

unknown, but bleeding is the most common postoperative complication and is some-
times fatal. The disadvantages are difficulties with future sexual life and birthing, at
which time the Faraonic circumcision must be mechanically opened. After giving
birth, there is often a desire to be restored to the circumcised status.
To remember: no child has ever asked for circumcision!
Phimosis
Incidence
Phimosis is a condition of circumferential scarring at the tip of the foreskin,
which prevents retraction. Incidence is about 1% or 2%.
Etiology
Infection, balanopostitis, or forceful retraction maneuvers lead to scaring of the
prepuce. The phimosis usually presents late in infancy and occasionally there is his-
tory of urinary tract infection. Physical examination shows obstruction of urinary
flow and inability to retract the foreskin. Treatment is plastic surgical procedure to
open the prepuce, or in severe cases circumcision.
Selected Readings
1. Rowe MI et al. Male external genitalia. In: Rowe MI, O’Neill Jr JA, Grosfeld JL et
al, eds. Essentials of Pediatric Surgery. St. Louis: Mosby-Yearbook Inc. 1995;
769-770.
2. International Code of Medical Ethics. Adopted 35th World Medical Assembly.
Venice, Italy, Oct 1983.
3. AAP Task Force on Circumcision: Report of the Task Force on Circumcision.
Pediatrics 1989; 84:388.
4. Baskin LS, Canning DA, Snyder HM et al. Treating complications of circumci-
sion. Pediatr Emer Care 1996; 12:62-68.
16
CHAPTER 1
CHAPTER 17
Hemangiomas and Vascular Malformations

Maureen Sheehan and Daniel A. Bambini
Incidence
Vascular anomalies are relatively common in neonates and infants. Hemangio-
mas are the most common benign tumor of infancy yet account for less than one
third of congenital vascular lesions. Congenital hemangiomas occur in only 1.1-2.6%
of term infants; however, that incidence increases to almost 30% in premature in-
fants weighing less than 1000 grams. Although few are evident at birth, 70-90% of
hemangiomas are apparent by one month of age. Females are affected 3-5 times
more than males. Incidence also varies with ethnic origin. Caucasians have the high-
est incidence of hemangiomas accounting for 10% of patients; the incidence is much
lower in children of African-American or Asian descent.
Vascular malformations, unlike true hemangiomas, are always present at birth
although not always apparent. They affect females and males equally. The incidence
of each type of vascular malformation is quite variable. Capillary malformations are
the most common affecting 3-6 per 1,000 infants. Both clinical management and
outcome vary with the type of lesion present.
Etiology
Hemangiomas are neoplastic growths that most often occur sporadically although
10% are familial. The exact etiology remains unknown. The growth consists of a
mass of endothelial cell proliferation interspersed with vascular lumens and chan-
nels. Hemangioma formation may represent faulty embryonic development of
peripheral blood vessels in which endothelial cells undergo neo-vascularization and
canalization.
Vascular malformations result from developmental errors of arteries, veins, cap-
illaries, or lymphatics. During the third week of fetal life, mesenchymal cells differ-
entiate into primitive capillary clusters and by the 48th day of gestation the capillary
clusters connect with feeding arteries and draining veins. Lymphatics are formed
from buds off the veins forming a parallel drainage system. Vascular malformations
occur as a result of hypoplasia, hyperplasia, or aplasia of any (or a combination) of
the developing vascular structures.
Hemangiomas most frequently occur on the head and neck (60%); the trunk is
the next most frequent site of occurrence followed by the extremities. Hemangio-
mas are frequently identified after a period of rapid growth and continue to enlarge
disproportionate to the child’s growth over the first 6-8 months of life. During the
rapid growth phase, superficial lesions appear bright red while deeper lesions have a
purple or blue hue. The period of rapid growth is followed by a stationary phase and
ultimately by a period of involution (usually beginning by 18 months of age). The
stages are not mutually exclusive, and frequently involution and proliferation occur
simultaneously within different areas of the same lesion. During the involution stage,
hemangiomas often fade to a blue-gray color and acquire an area of central pallor.
The time required for full involution varies: 50% involute by age 5, 70% involute
by age 7, and 90% involute by age 9. Despite complete resolution, some skin changes
(i.e., pallor, atrophy, or skin redundancy) may persist in 10-20% of cases. Scarring
usually does not occur unless the lesion has areas of ulceration.
Vascular malformations are present at birth but not necessarily obvious. The
presentation of the lesion differs depending on the etiology of the lesion and loca-
tion. High flow lesions such as arteriovenous malformations usually become appar-
ent on physical exam with noted warmth, palpable thrill, audible bruit, or visible
pulsation. Capillary vascular malformations appear to follow sensory nerve distribu-
tion and have a purplish hue. Venous malformations undergo gradual dilation that
give the appearance of a growing lesion sometimes mistaken for a hemangioma.
Venous malformations are easily compressible and swell with dependent position-
ing. Lymphangiomatous lesions often require the assistance of gravity to become
apparent and frequently lead to increased limb circumference. Lymphangiomas rap-
idly enlarge when they become infected and seldom undergo full regression after
treatment.
Diagnosis
Hemangiomas (Fig. 17.1) are most often diagnosed based on history and physi-
cal exam; further diagnostic testing is usually not warranted. The differential diag-
nosis of the hemangiomas varies with its stage of growth. During the pre-eruptive
state, hemangiomas may be confused for a nevus, port wine stain, or focal dermal
hypoplasia. At later stage, they may appear similar to spider angiomas,
angiokeratomas, or pyogenic granulomas.
Computed tomography (CT) and magnetic resonance imaging (MRI) are very
useful to define the nature of vascular lesions. For hemangiomas, the appearance on
CT varies depending on the stage of growth. During the proliferative phase, heman-
giomas appear well circumscribed with homogenous enhancement. Hemangiomas
undergoing involution appear more lobulated and heterogeneous. The CT appear-
ance of vascular malformations varies depending upon their origin and location.
Venous malformations typically have heterogeneous enhancement and sometimes
contain calcifications. Lymphatic malformations appear as multiloculated cysts with
septa. Musculoskeletal changes such as hypertrophy, distortion, or destruction can
also be identified by CT of vascular malformations
17 MRI and magnetic resonance angiography (MRA) are perhaps the most accu-
rate radiologic studies to evaluate vascular lesions. MRI/MRA differentiate heman-
giomas from vascular malformations, easily distinguished high flow lesions from
low flow lesions, and correctly identify lymphatic malformations.
Hemangiomas and Vascular Malformations 67
Fig. 17.1. Raised, spongy mass typical of a cavernous capillary hemangioma.
Pathology
When hemangiomas in the proliferative phase are studied with electron micros-
copy, a multilaminate basement membrane and an abundance of mast cells are promi-
nent features. Mast cells release heparin which modulates angiogenic factors and
promotes blood vessel formation. The endothelial cells of proliferating hemangio-
mas appear flattened. Uptake of 3H-thymidine, a marker for rapid cellular prolif-
eration, is much increased.
Vascular malformations have an increased ratio of endothelial cells to smooth
muscle cells of 214:1 as compared to a ratio of 10:1 to 62:1 found in normal vessels.
Histologic features include:
1. ectatic capillaries, veins, or lymphatics,
2. thin basement membranes and
3. absence of rapid endothelial turnover.
Lymphatic malformations are sometimes classified as microcystic, macrocystic,
or combined. When viewed under the light microscope, these lesions are composed
of abnormal vesicles or ectactic channels filled with lymphatic fluid. Capillary mal-
formations are composed of a network of dilated capillaries and venules in varying
densities.
Treatment
The treatment course of hemangiomas is one of observation which allows 70-90%
17
complete involution. Vascular crises require a more aggressive approach as does the
rare hemangioma that:
1. involves the visual axis which may cause permanent amblyopia,
2. causes airway obstruction,
3. causes bilateral auditory canal obstruction,

4. causes congestive heart failure,
5. is associated with Kasabach-Merritt syndrome. Surgery to assure a good
cosmetic result is considered in patients with pedunculated lesions, bulky
lesions (such as on the nasal tip), or ulcerated lesions. Cryotherapy is
occasionally used to ablate hemangiomas but frequently leaves hypo-pig-
mented lesions and atrophic scarring. For large lesions that infiltrate vital
structures, corticosteroid therapy for 4-6 weeks is advocated. The dose is
high, involution occurs rapidly if at all (< 30% of cases), and the lesion
may regrow when the steroids are tapered. Alpha interferon will also pro-
duce regression in many hemangiomas that produce risk to life. The treat-
ment however requires months and is slow to produce results.
Treatment for vascular malformations varies dependent on the etiology. Port
wine stains are best ablated using laser photocoagulation. Venous malformations are
sometimes treated using laser therapy, but may be amenable to compression stock-
ings, sclerotherapy, or surgical removal. Debulking, when appropriate, is effective
for lymphatic malformations, however, this treatment is frequently limited by the
proximity of lymphatics to important anatomic structures. Antibiotics are necessary
to treat lymphatic malformations if they become infected.
Arteriovenous malformations require surgical excision. Simple ligation is inef-
fective and embolization has generally failed for large lesions but is useful when
performed within 24 hours preceding operation to reduce blood loss at the time of
excision.
Selected Readings
1. Wahrman JE, Honig PJ. Hemangiomas. Peds in Review 1994; 15(7):266-271.
2. Low DW. Hemangiomas and vascular malformations. Sem Ped Surg 1994;
3(2):40-61.
3. Silverman RA. Hemangiomas and vascular malformations. Ped Derm 1991;
38(4):811-833.
4. Filston HC. Hemangiomas, cystic hygromas, and teratomas of the head and neck.
Sem Ped Surg 1994; 3(3):147-159.
17
CHAPTER 1
CHAPTER 18
Branchial Cysts, Sinuses and Fistulas

Daniel A. Bambini
Incidence
The exact incidence of branchial remnants in children is not known but these
lesions occur less commonly than thyroglossal duct cysts and slightly more com-
monly than cervical vascular or lymphatic malformations. Second branchial rem-
nants and cysts are much more common than those of first branchial origin. Lesions
derived from the lower branchial apparatus are extremely rare.
Etiology
All branchial cysts, sinuses or fistulae are congenital lesions arising from defec-
tive development of the branchial apparatus. The branchial apparatus is identifiable
in the 4-8 week old human embryo as a series of 6 branchial arches with intervening
clefts (ectodermal) and pouches(endodermal). There is normally no communica-
tion between the clefts and pouches in the human embryo which are separated by
pharyngeal membranes. Many of the final structures of the head and neck are all
derived from the branchial arches, clefts and pouches. Branchial fistulas occur when
there has been breakdown or abnormal development of the pharyngeal membranes.
Defects in first branchial arch development result in cleft lip/palate deformities,
abnormally shaped external pinna, and malformation of the malleus and incus (deaf-
ness). Aural atresia and microtia result from failure of the first branchial cleft to
develop normally. First branchial cysts and sinuses/fistulae occur near or behind the
anterior border of the upper third of the sternocleidomastoid, under the angle of the
jaw, or just below the ear. Sinuses or fistulae of first branchial origin extend from an
external opening at the posterior submandibular triangle to the external auditory
canal. Complete fistulas with an opening at the external auditory canal only occur
in 30% of these rare cases. The tract courses very near the facial nerve.
Second branchial defects are most often complete fistulas. The second branchial
fistula opens externally along the lower third of the anterior border of the sterno-
cleidomastoid. The tract ascends form this opening along the platysma and courses
along the carotid sheath, over the hypoglossal and glossopharyngeal nerves passing
between the bifurcation of the carotid artery. The tract passes behind the posterior
digastric and stylohyoid muscles and opens on the lateral pharyngeal wall at the
level of the tonsillar pillar. Cysts and/or cartilaginous remnants of second branchial
pouch or cleft origin occur along the same tract. These cysts may or may not have an
associated sinus tract.
Third and fourth branchial anomalies occur extremely rarely and usually occur
in the left neck. The external opening of third branchial abnormalities would be
expected along the anterior edge of the sternocleidomastoid at the level of the clavicle.
The tract passes posterior to the internal carotid artery and then superior to the
adjacent 11th cranial nerve to connect with the piriform sinus. Fourth branchial
anomalies also would have an opening at the lower neck and a tract extending pos-
terior to the sternocleidomastoid, inferior to the subclavian artery (right) or aortic
arch (left), and then cephalad toward the cervical esophagus.
Second branchial fistulas are more likely to present in the neonate but the external
skin opening often goes unnoticed. The parent may first notice a mucoid drainage
from the external opening or the area may present as a localized infection. Infection
is less common in fistulas and sinus tracts than in cysts. Branchial cysts often go
unnoticed until later childhood or adolescence/early adulthood and present as
gradually enlarging masses along the anterior border of the sternocleidomastoid.
Approximately 10-15% of branchial remnants occur bilaterally. There may be a
family history of similar branchial anomalies in 10% of these children. Twenty-five
percent of branchial cysts initially present with signs of acute inflammation includ-
ing tenderness and erythema.
First branchial cysts present as swellings near the ear lobe within the subman-
dibular triangle in children of all ages. There is often a history of recurrent infections
and either spontaneous or surgical drainage. These lesions are in close proximity to
the parotid gland and underlying facial nerve.
In addition to cysts and/or sinus tracts, these anomalies may include ectopic
cartilage and/or skin tags.
Diagnosis
The diagnosis of branchial remnants including cysts, fistulas, sinuses, tags and
cartilage is made by physical exam and history. The location of each lesion is fairly
typical. The differential diagnosis of branchial cysts and remnants is that of the
lateral neck mass in children. The differential diagnosis of first branchial remnants
includes cat scratch disease and other causes of cervical lymphadenitis such as tuber-
culosis, atypical mycobacterium, histoplasmosis and actinomycosis. Preauricular cysts
or sinuses (Fig. 18.1), although in a similar location, are usually anterior to the ear
and are not of branchial origin.
Second branchial anomalies must be distinguished from lymphangioma, lym-
phadenitis, atypical mycobacterium infection, malignant lymph nodes, dermoids,
parotid lesions, tumors of the sternocleidomasotoid and/or torticollis, cat scratch
disease, actinomycosis and hemangioma. The differential diagnosis of first bran-
chial abnormalities. Mycobacterium stains, immune titers, skin testing and cultures
may all be useful to exclude these other possibilities. Ultrasonography is occasion-
ally useful to distinguish neck masses as cystic or solid and determine precisely the
relation between lesions and the surrounding vital structures of the neck.
18
Pathology/Pathophysiology
The tissue lining branchial remnants and cysts is the same for both first and
second branchial derivatives. Ninety percent are lined with stratified squamous
Branchial Cysts, Sinuses and Fistulas 71
Fig. 18.1. Typical appearance of preauricular pits.
epithelium, but columnar epithelium with or without cilia is possible. Skin append-
ages are sometimes present as are acute and chronic inflammatory changes when
infection has occurred.
Treatment
All branchial remnants should be completely excised prior to infection. Bran-
chial sinuses require excision to prevent recurrent infection and chronic drainage.
Acute infections of branchial cysts are treated with incision and drainage, antibiotics
and warm compresses. Excision is performed after the acute inflammatory response
has subsided. Second branchial cysts, sinuses or fistulas must be resected with cau-
tion to avoid injury to the glossopharyngeal nerve and carotid artery. The facial
nerve must be carefully identified and preserved when first branchial remnants are
excised. Nerve injury is best avoided by dissecting as close to the tract as possible
during the resection.
Outcome
Recurrence is rare unless the epithelialized tract is not completely excise. Previ-
ous infection increases the risk of recurrence. Neoplastic degeneration of branchial
remnants can occur and is another reason for early, complete resection.
Selected Readings
1. Telander RL, Deane SA. Thyroglossal and branchial cleft cysts and sinuses. Surg
Clin North Am 1977; 57:779.
2. Smith CD. Cysts and sinuses of the neck. In: O’Neill Jr. JA et al, eds. Pediatric
Surgery, 5th Edition. St. Louis: Mosby 1998; 757-772. 18
CHAPTER 19
Thyroglossal Duct Cyst and Sinus

Daniel A. Bambini
Incidence
Thyroglossal duct cysts or remnants are the most common midline cervical mass
of childhood. While the exact incidence is difficult to estimate, they occur 3 times
more commonly than branchial remnant cysts and sinuses. The sex incidence is
approximately equal.
Etiology
In the normal embryo, the thyroid anlage develops as an endodermal thickening
that creates a diverticulum in the floor of the pharynx between the first and second
pharyngeal grooves. Initially the developing thyroid is in close contact with the
developing aortic arch. As the embryo elongates, the thyroid diverticulum descends
in the anterior neck in close proximity to the developing hyoid bone. A connection
to the foramen cecum at the base of the tongue persists as the thyroglossal duct until
the thyroid attains its final position in front of the trachea. The upper portion of the
thyroglossal duct then disappears during the 5th week of intrauterine life, however
the lower portion of the duct may persist as the pyramidal lobe of the thyroid. If all
or part of the thyroglossal duct persists beyond fetal life, a cyst or sinus tract can
develop.
Thyroglossal duct cysts are rarely present at birth. Two thirds of thyroglossal
duct cysts will become clinically apparent in childhood; the majority appearing by
2-6 years of age. The most common presentation is an asymptomatic midline cervi-
cal mass with normal overlying skin (Fig. 19.1). Less than 1% will be lateral to
midline. Occasionally the cyst can become infected with mouth flora, leading to
spontaneous or even surgical drainage. A chronic draining sinus tract may develop.
Physical examination most commonly reveals a nontender, unilocular, rounded
firm mass at or near the hyoid bone. However, it can occur anywhere in the midline
of the neck between the base of the tongue and the thyroid gland. The mass may be
tender if the cyst is infected. The mass, which is tethered to the hyoid, will move in
the vertical axis as the patient swallows. The mass will also move with protrusion of
the tongue.
The differential diagnosis includes ectopic thyroid, pyramidal lobe of thyroid,
dermoid or sebaceous cyst, lymphadenitis, thyroid goiter and lipoma.
Thyroglossal Duct Cyst and Sinus 73
Fig. 19.1. Midline neck mass over hyoid bone characteristic of thyroglossal duct
cyst (other common lesion in this location is a midline dermoid cyst).
Fig. 19.2. Thyroglossal duct cyst as visualized at the time of surgical excision which
includes resection of the central portion of the hyoid bone (Sistrunk procedure).
19
Diagnosis
The diagnosis of thyroglossal duct cyst is made primarily on the basis of physical
findings. Rarely, if midline ectopic thyroid cannot be excluded, thyroid scan or aspi-
ration biopsy may be helpful. Dermoid cysts are superficial lesions that remain at-
tached to the dermis and are easily distinguished from thyroglossal duct cysts during
excisional biopsy.
Pathology
The majority of thyroglossal duct cysts are lined by psuedostratified ciliated co-
lumnar or stratified squamous epithelium. Twenty percent will contain some recog-
nizable thyroid tissue. Chronically infected cysts and sinuses may be composed mostly
of granulation tissue or fibrous tissue.
Treatment
Treatment of a thyroglossal duct cyst is by complete excision of the cyst and its
tract. Resection should include the central portion of the hyoid bone and complete
excision of the tract to the base of the tongue (Sistrunk procedure). Thyroglossal
duct cysts should be removed shortly after discovered and before infection occurs.
Infected cysts should be treated with antibiotics and aspiration or drainage with
surgical excision delayed until local inflammation subsides. Postoperative care is
outpatient and if a drain is used, it is removed at 24-48 hours.
Outcomes
Recurrence is infrequent (< 5%) if the central portion of the hyoid bone is resected
en bloc. In the past, over 95% of recurrent cases were associated with failure to
resect the central hyoid bone. The risk of recurrence following resection of a previ-
ously infected or drained thyroglossal duct cyst is increased by 50%. Malignant
degeneration can occur in thyroglossal duct remnants which may give rise to adeno-
carcinoma or squamous carcinomas later in life.
Selected Readings
1. Sistrunk WE. Technique of removal of cyst and sinuses of the thyroglossal duct.
Surg Gynecol Obstet 1928; 46:109-112.
2. Soper RT, Pringle KC. Cysts and sinuses of the neck. In: Welch K, et al, eds.
Pediatric Surgery, 4th edition. Chicago: Year Book Medical Publishers 1986;
539-551.
3. Tapper D. Head and neck sinuses and masses. In: Holder TM, Ashcraft KW eds.
Pediatric Surgery, 2nd Edition. Philadelphia: WB Saunders 199; 923-934.
19
CHAPTER 1
CHAPTER 20
Umbilical Anomalies
Daniel A. Bambini
Disorders of the umbilicus are not unusual in newborns and infants. In this
Chapter, the common anomalies of the umbilicus are discussed including ompha-
lomesenteric (vitelline) duct remnants, urachal remnants, and umbilical hernia.
Omphalocele and gastroschisis, which also involve the umbilicus, are defects of the
abdominal wall and are discussed in Chapter 77.
Incidence
Umbilical disorders are frequently encountered in pediatric patients. Umbilical
hernia is probably the most common hernia in children and occurs in about 40% of
African-American children and in 3-4% of Caucasian children. It is more common
in premature infants. Urachal remnants are fairly uncommon affecting approximately
one of every 5000 newborns. Omphalomesenteric remnants are less rare lesions.
Meckel’s diverticulum (Chapter 55) is the most common and is identifiable in 1-2%
of all autopsies while a patent omphalomesenteric duct affects 1in 15,000 live births.
Etiology
Some disorders of the umbilicus arise from the persistence of normal fetal struc-
tures (i.e., omphalomesenteric duct, urachus). Early in gestation, the embryo is con-
nected to the chorion (developing placenta) by a connecting stalk of extraembryonic
mesoderm. The omphalomesenteric duct connects the extraembryonic coelom to
the intestine (ileum) and normally obliterates and involutes by the sixth week of
gestation. Incomplete obliteration can result in variety of omphalomesenteric defects
(see below). The urachus is a tubular, fetal structure that connects the developing
urinary bladder to the allantoic stalk. Later in gestation, the urachus also obliterates
to form the median vesico-umbilical ligament. Urachal remnants can persist if the
obliterative process is incomplete but may also occur in association with obstructive
lesions of the distal urinary tract (i.e., urethral stenosis, urethral atresia, posterior
urethral valves). Umbilical hernias result from failed closure of the facial ring at the
umbilicus.
Clinical Presentation, Diagnosis, and Treatment
Omphalomesenteric Duct Defects
The five omphalomesenteric duct defects are:
1. Meckel’s diverticulum (in which the proximal duct has remained patent,
2. umbilical polyp with blind pouch (in which the distal duct has remained
patent),
20 3. vitelline duct cyst (midportion of duct has remained patent),
4. patent omphalomesenteric duct, and
5. persistent fibrous cord. Although the clinical presentation varies with the
specific lesion present, all except the persistent fibrous cord present with
symptoms specific to the umbilicus. If the omphalomesenteric duct per-
sists as a fibrous cord or band, it can act as a fulcrum for midgut volvulus
or herniation of a loop of bowel between the band and the abdominal
wall. The clinical picture in this case is intestinal obstruction and/or vol-
vulus.
The patent omphalomesenteric duct (POD) is present from birth but is often
(40%) not noticed until the infant is beyond one month of age. Boys are affected
more than girls at a ratio of about 7:1. The symptoms are usually drainage of foul-
smelling material, bowel contents, feces, or gas from the umbilicus. Inspection of
the umbilicus in children with POD or umbilical polyp reveals a “rosette” of pink,
bowel mucosa at the umbilical surface. If the omphalo-ileal fistula is large and wide,
the ileum can prolapse through it. Vitelline duct cysts frequently present with signs
of infection including purulent umbilical drainage and periumbilical erythema,
induration, and tenderness. A mass deep to the umbilicus is occasionally palpable.
The differential diagnosis includes urachal remnants and umbilical granuloma. Ab-
dominal x-ray with contrast administered via the umbilical opening may show com-
munication with the intestine confirming the diagnosis and excluding the possibility
of a urachal lesion. The treatment of patent omphalomesenteric duct, umbilical
polyp, and vitelline duct cyst is complete surgical resection and umbilical recon-
struction.
Urachal Remnants
Urachal remnants are classified into five distinct groups:
1. completely patent (communicates between umbilical surface and bladder),
2. urachal sinus (opens to umbilical surface),
3. urachal diverticulum (opens to bladder),
4. urachal cyst, and
5. urachal chorda (persists as a cord). Urachal remnants are often lined by
transitional epithelium but are frequently composed primarily of granu-
lation tissue.
Children with urachal remnants most frequently present in infancy and early
childhood, but rarely present in the neonatal period. The usual presenting symptom
in infants with a persistent patent urachus is a report of a thin, watery discharge
from the umbilicus. Mucosal prolapse may be visible. The other urachal remnants
most commonly present as infection with erythema and purulent drainage from the
umbilicus. Low midline abdominal mass and pain are sometimes the presenting
features.
The diagnosis of urachal cyst is most commonly confirmed by ultrasound that
also determines its size, its relation to the umbilicus and bladder, mobility, and its
location within the abdominal wall. Cystoscopy is helpful to identify urachal diver-
Umbilical Anomalies 77
20
Fig. 20.1. Meckel’s diverticulum with large omphalomesenteric cyst at the distal tip.
ticula. Fistulogram if an umbilical puncta is present demonstrates a completely patent

urachus or urachal sinus. The differential diagnosis includes the various forms of
omphalomesenteric duct remnants, umbilical granuloma, and a variety of inflam-
matory intra-abdominal processes.
Treatment of these lesions depends on the exact type of remnant and the extent
of associated infection and/or inflammation. Urachal fistulas, cysts, and diverticula
should be completely resected. Urachal diverticula of the bladder are associated with
high risk for chronic urinary tract infection, stone formation, and malignancy. Infected
urachal remnants are initially treated with broad-spectrum antibiotics (7-10 days)
followed by resection. In some cases, initial incision and drainage may be preferable.
Complete resection is then performed after the acute inflammatory reaction subsides.
Umbilical Hernia
Umbilical hernias are present at birth when the fascial ring of the umbilicus has
not completely closed. Eighty-five percent of umbilical hernias will close spontane-
ously by 6 years of age. The diagnosis is made simply by clinical observation. The
hernia wall consists of skin and underlying peritoneum and bulges with increases in
intra-abdominal pressure. Depending upon the size of the hernia, bowel loops may
be contained within it. The size of the fascial defect is variable.
Most surgeons wait until the child is at least 4-5 years of age prior to repair to
allow adequate time for spontaneous closure (85%). Hernias with especially large
fascial defects may be an exception to the wait-and-see approach as they are less
likely to close spontaneously. Complications of pediatric umbilical hernia repair are
infrequent. Infection and hematoma are the most prevalent but occur in less than
2% of cases.
Outcomes
Recurrence after complete resection of noninfected omphalomesenteric or urachal
20 remnants is extremely uncommon. The recurrence rate after resection of infected
urachal cysts in near 30%. Urachal remnants have the potential for malignant
degeneration to adenocarcinoma or transitional cell carcinoma bases on a few case
reports but the frequency of this occurrence is not truly established.
Selected Readings
1. Jona JZ. Umbilical anomalies. In: Raffensperger JG ed. Swenson’s Pediatric Sur-
gery, 5th Edition. Norwalk: Appleton & Lange 1991; 189-198.
2. Shaw A. Disorders of the umbilicus. In: Welch KJ, Randolph JG, Ravitch MM et
al, eds. Pediatric Surgery, Fourth Edition. Chicago: Yearbook Medical 1986;
731-739.
3. Rich RH, Hardy BE, Filler RM. Surgery of the anomalies of the urachus. J Pediatr
Surg 1983; 18:370-372.
4. Lassaletta L, Fonkalsrub EW, Tovard JA et al. The management of umbilical her-
nias in infancy and childhood. J Pediatr Surg 1975; 10:405.
5. Haller Jr. JA, Morgan Jr. WW, Stumbargh S. Repair of umbilical hernias in child-
hood to prevent adult incarceration. Am Surg 1971; 37:245.
CHAPTER 1
CHAPTER 21
Foreign Bodies
of the Gastrointestinal Tract
John R. Wesley
Incidence
We have no idea of how many gastrointestinal foreign bodies are ingested annu-
ally. It is probable that most children accidentally swallow a foreign body that passes
undetected sometime during childhood. However, the National Safety Council esti-
mates that approximately 2,000 people in the United States die each year from the
complications of inhaled or ingested foreign bodies. Fatal accidents in the home
comprise the majority of this total, and over half of these involve children newborn
to age 5 years. Foreign bodies in the tracheal-bronchial tree and esophagus are cov-
ered in Chapter 74. The present Chapter deals with diagnosis and management of
foreign bodies of the stomach, intestine, rectum, and genital-urinary tract.
Etiology
Infants and children, as a normal part of immature, exploratory activity, or because
of curiosity and their developmental need to taste new objects, place a variety of
foreign bodies into their mouths or other orifices. These include small toys, eating
utensils, pen tops, peanuts, cocktail hot dogs, other chunky or particulate foods,
batteries, coins, pins, buttons, screws, ornaments, bones, and beads. If the child is
startled, falls, or simply becomes distracted, the foreign body is swallowed.
As with esophageal and intestinal foreign bodies, objects in the rectum are due to
both unintentional and intentional actions. In general, the only object commonly
found in the rectum of children is a thermometer secondary to squirming while a
rectal temperature is taken. Other foreign objects are nearly always a consequence of
sexual behavior that is either auto-erotic or performed by a playmate, older child, or
adult bent on sexual abuse. In most cases, these rectal foreign bodies have a phallic
shape and include bottles, hot dogs, zucchini, broomsticks, and light bulbs. Foreign
bodies of the vagina or penile urethra frequently occur as a result of children playing
“doctor” or as a result of the desire to satisfy dares by “friends”. The types of objects
are sometimes imaginative but generally possess a long and thin shape such as pipe
cleaners, pens, pencils, metal springs, paper clips, screws, sticks and leaves, paint
chips, and swizzle sticks. In younger girls, early exploration and genital stimulation
frequently lead to vaginal insertion of foreign objects including toilet paper, toys, or
other small, rounded objects.
Diagnosis
A careful history is the keystone to making the correct diagnosis. Children under
the age of 2 years are usually accompanied by a responsible adult who has witnessed
an acute choking episode or the swallowing of a now missing object. Children between
the ages of 2 and 8 years are not constantly under adult supervision, and an acute
21 choking episode or ingestion of an object may pass unobserved. Children beyond
the age of 8 years can usually be relied upon to accurately describe the choking
episode, report ingestion of an object, and understand their significance. Following
the initial suspicious incident, there is often a symptom-free interval. Once objects
reach the stomach or intestine, they cause only vague or intermittent symptoms or
cause no further symptoms at all.
Treatment
Gastrointestinal Foreign Bodies
Once in the stomach, 95% of all ingested foreign bodies pass safely through the
gastrointestinal tract and exit without difficulty, usually within 24-48 hours. The
size, shape, and characteristics of the object dictate initial management: either con-
tinued observation or efforts at immediate retrieval.
In general, round or cuboidal objects without sharp edges or projections pass
through the stomach and intestine easily causing little difficulty and concern. Objects
of this type most commonly include coins, buttons, marbles, closed safety pins, and
small toys (Fig. 21.1) These patients need no hospitalization but should return if
they develop abdominal pain, vomiting, bloody stools, or if the object has not been
identified in the stools in 4-5 days. Roentgenograms document the progress of the
object, or whether it has passed unnoticed. Surgical removal is indicated for contin-
ued abdominal pain, vomiting, significant bleeding, or failure of the object to pass
in 4-5 weeks.
Of greater concern are those patients who have swallowed elongated, slender, yet
relatively blunt objects. These include pencils, pens, bobby pins, long nails, small
tools, and chicken bones. Although most of these pass without difficulty, failure of
passage is highest in this category. Problems occur at fixed points or sites of anatomi-
cal narrowing or angulation such as the pylorus, the C-loop of the duodenum, the
ligament of Treitz, and the ileocecal valve. The rigid nature of the object makes it
difficult to negotiate these areas. The ends of the objects, even though not sharply
pointed, may impinge on the bowel wall causing damage or perforation. These pa-
tients may be admitted to the hospital or observed carefully as outpatients. Progress
of the object is followed by serial roentgenograms (Fig. 21.2) Operative removal is
indicated if significant abdominal pain, tenderness, or bleeding is present, or if the
object fails to change position.
Of greatest concern are those patients who have swallowed foreign bodies with
sharp edges or points. These include pins, needles, tacks, jacks, open safety pins and
pieces of glass. Such a patient should be hospitalized and followed carefully, with
daily roentgenograms to monitor the progress of the object and frequent physical
examinations for tenderness. The patient’s stools are strained for the object and
tested for blood. Abdominal pain, tenderness, elevated temperature, and rectal bleed-
ing are warning signals, but continued progress of the foreign body is still a good
Foreign Bodies of the Gastrointestinal Tract 81
21
Fig. 21.1. Examples of foreign bodies commonly swallowed or aspirated by children.
Fig. 21.2. Spoon handle

swallowed by a re-
tarded child; passed
without difficulty in 3
days.
reason for nonoperative management. Surgical intervention is reserved for patients

with significant bleeding or signs of peritonitis. Operation is also indicated when
the object has failed to move over several days. Fortunately, most straight pins, needles,
and open safety pins will pass uneventfully.
Finally, battery ingestion poses a special problem, and is becoming increasingly
21 more common as “button-size” batteries in watches, hearing aids, cameras, and cal-
culators are more accessible to the pediatric population (Fig. 21.3). Because batter-
ies may leak or burst within 24 hours of ingestion and cause corrosive alkali burns or
poisoning from mercuric salts, they are removed endoscopically while still in the
stomach. Once they have passed into the duodenum, they are removed surgically if
they do not progress rapidly through the intestine as indicated by roentgenograms
taken at 6-hour intervals. Intestinal perforation has been reported from a small bat-
tery lodged in a Meckel’s diverticulum, and death in a 16-month old infant from
corrosive esophageal perforation has followed ingestion of an alkaline battery. Dietary
aids to protect mucosa and help passage are generally not effective and not recom-
mended. An exception is the use of mineral oil to possibly aid the passage of a
particularly bulky object. Cathartics are always contraindicated.
Rectal, Penile, and Vaginal Foreign Bodies
A few but nevertheless surprising number of objects find their way into the rec-
tum of teenagers and young adults. Dildos, coke bottles, light bulbs, and umbrellas
have been removed (Fig. 21.4) and there are undoubtedly other equally surprising
objects that go unreported. The basic tenant in dealing with these situations is to
expect the unusual. Pain, bleeding, obstipation, and embarrassment usually bring
the patient to the emergency room. The history is either unbelievably scant, or
inappropriately complex, and frequently associated with sexual experimentation,
hazing, and alcohol intoxication. Sigmoidoscopy with rectal lubrication and dilata-
tion is the keystone to extraction. Occasionally a light general anesthetic is required.
Perforation may require a temporary diverting colostomy.
A variety of small elongated objects have been reported in the penile urethra
including pipe cleaners, thermometers, and swizzle sticks (Fig. 21.5). The same his-
torical and etiological factors hold true as described for rectal foreign bodies. A
pelvic roentgenogram will demonstrate the object. Cystoscopy is generally required
for removal.
Vaginal foreign bodies should be considered whenever evaluating a prepubertal
girl with lower genital tract symptoms. An intermittently bloody, foul-smelling vaginal
discharge is the classic complaint of the patient with a vaginal foreign body. Small
wads of toilet paper are the most common foreign bodies, but objects such as pencil
erasers, pins, beads, nuts and leaves are also found. Physical examination is best
carried out with the child in the knee-chest position unless anesthesia is required.
Gentle vaginal lavage with saline solution can remove bits of toilet paper. In general,
small objects can be most easily palpated on rectal examination and removed if the
examiner places his finger in the rectum and then applies gentle outward pressure.
However, if the object is large or sharp, or simple maneuvers fail, the patient will
require examination and removal of the foreign body under general anesthesia.
A forgotten tampon is the most common intravaginal foreign body in menar-
chial females. Obviously the patient’s age and hormonal status should be considered
Foreign Bodies of the Gastrointestinal Tract 83
21
Fig. 21.3. Examples of ingestable batteries with dime to indicate relative sizes.
Fig. 21.4. Umbrella

inserted into the rectum
under obscure circum-
stances. Reprinted with
permission from Dav-
enport HW. Physiology
of the digestive tract,
3rd edition. Chicago:
Year Book Medical
Publishers, 1971.
first in the differential diagnosis of vaginal discharge. Although physicians are often
reluctant to raise the question, every child with genital complaints (and his/her
parents) should be asked directly whether there is a possibility of any sexual contact
or sexual abuse. If the history provides few diagnostic clues, then the physical exami-
nation and cultures will be the physician’s best guide to proper management.
21
Fig. 21.5. A pipe cleaner inserted into the penile urethra removed by cystoscopy.
Prevention
The best approach to the management of foreign bodies is prevention. Children
are great imitators who frequently see adults holding pins, needles, or nails in their
mouths or talking, laughing, and walking around while eating—all practices that
should be condemned. Infants and children under the age of 5 should be denied
access to buttons, screws, coins, beads, pins, small toys, and jewelry. All toys should
be inspected for loose parts, and bean shooters, dart guns, and similar playthings
should be prohibited. All nuts, seeds, carrots, and popcorn should be withheld from
the diet of children under the age of 4 years. Food for children should never contain
small bones; cherry, plum, prune, or peach pits; watermelon, orange, or grape seeds;
or stems from fruits. Physicians should be alert for loose deciduous teeth prior to
any surgery. When foreign body aspiration or ingestion does occur, an understand-
ing of the principles of management and a willingness to move ahead swiftly with
the appropriate therapy greatly reduces the mortality and morbidity of this com-
mon health problem.
Selected Readings
1. Kosloske AM. Foreign bodies. In: Buntain, WB, ed. Management of Pediatric
Trauma. Philadelphia: W.B. Saunders Co. 1995; 459-477.
2. Wesley JR: Management of foreign bodies in various orifices. Part I: Etiology and
Foreign bodies of the ears, nose, larynx, trachea, bronchi, and esophagus. Emer
Med Rep 1984; 14:101-108.
3. Wesley JR: Management of Foreign bodies in various orifices. Part II: Foreign
bodies of the intestine, rectum, urethra, vagina, and prevention. Emer Med Rep
1984; 15:109-116.
4. Temple DM, McNeese MC. Hazards of battery ingestion. Pediatr 1983;
71:100-103.
5. Paradise JE. Vaginal discharge. In: Fleisher G, Ludwig S eds. Pediatr Emer Med.
Baltimore: Williams and Wilkins 1983; 262-265.
CHAPTER 1
CHAPTER 22
Hypertrophic Pyloric Stenosis

Richard Fox and Daniel A. Bambini
Incidence
Hypertrophic pyloric stenosis (HPS) is a disease of newborns with an incidence
of 1 in 300 to 1 in 900 live births. It is most commonly identified in Caucasians of
northern European descent. Throughout the world, HPS appears to be less com-
mon in Africans, African Americans, and Asians. HPS is more common in infants
with blood types B and O. Seasonal variation in the incidence of HPS has been
reported; more infants present during the spring and fall months.
Although a genetic predisposition to HPS is suspected, the exact mode of inher-
itance is unknown. Males are affected four times as often as females, and first born
males are at highest risk. Family history is relevant. When parents (mother or father)
have had HPS it occurs in 5-20% of their male children but in only 3-7% of their
female children. Children whose mothers had HPS develop pyloric stenosis 3-4
times more frequently than children whose fathers had HPS. Finally, monozygotic
twins are more likely to be concordant for HPS than are dizygotic twins.
Etiology
Despite extensive clinical and laboratory research, the cause of pyloric stenosis
remains unknown. Researchers have investigated the role of many factors (i.e., gan-
glion cells, maternal factors, gastric acidity, nutritional factors, prostaglandins, nitric
oxide, hypergastrinemia, etc.) suspected as contributors to pyloric muscle hypertro-
phy, yet no conclusive etiology has been described.
Infants with HPS generally present between the second or third week of life to
two months of age. Rare cases have been reported throughout childhood and into
adult life. Symptoms begin as mild regurgitation that gradually progresses to
nonbilious vomiting. With time, the emesis becomes more frequent and forceful
(i.e., “projectile”). Infants are generally consolable and hungry after vomiting epi-
sodes. In 3-5% of cases, the vomitus will be brown or even bloody secondary to an
associated esophagitis /gastritis.
In HPS, dehydration, weight loss, and failure to thrive are the result of uncor-
rected fluid losses and inadequate nutrition caused by a nearly complete gastric out-
let obstruction. Gastric secretions contain significant quantities of potassium,
hydrogen ion, and chloride. Although the kidney can initially compensate for mild
electrolyte losses, with prolonged emesis and dehydration, a hypokalemic,
hypochloremic, metabolic alkalosis develops. Indirect hyperbilirubinemia is observed

in 1-2% of patients and is caused by a decrease in hepatic glucuronyl transferase
probably as a consequence of starvation.
Physical examination often reveals a hungry child with signs of dehydration (i.e.,
sunken fontanelles, pale mucosal membranes, poor skin turgor, lethargy, hypoto-
nicity, poor capillary refill). Visual inspection of the abdomen may reveal peristaltic
waves traversing from left to right across the upper abdomen. Nasogastric or orogastric
22 decompression of the stomach offers symptomatic relief and empties the stomach of
retained formula/milk and mucous facilitating palpation of a hypertrophied pylorus
(“olive”). A meal of sugar water can be administered to satiate the crying infant. As
the child relaxes, palpation of the pyloric mass is often easier.
The differential diagnosis of HPS is extensive and includes all causes of nonbilious
emesis in the neonate. Anatomic anomalies that mimic pyloric stenosis include duode-
nal stenosis, antral/pyloric webs or duplications (those 10-15% with obstruction
proximal to the ampulla of Vater). Functional problems in the differential include
gastroenteritis, gastroesophageal reflux with/without hiatal hernia and achalasia. The
metabolic differential includes congenital adrenal hyperplasia (CAH) and inborn
errors of metabolism. Intracranial pathologies, including intracerebral bleeding and
hydrocephalus are also associated with “projectile” vomiting.
Diagnosis
The hypertrophied pylorus (“olive”) is palpable on clinical exam in about 75-90%
of infants with HPS. If the “olive” cannot be felt or the diagnosis is in doubt, an
abdominal ultrasound is beneficial. A pyloric muscle thickness greater than 4 mm
or pyloric channel length greater than 14mm confirms the diagnosis.
Ultrasonographic detection of pyloric stenosis has a false negative rate of 5-10%. If
ultrasonography is nondiagnostic, an upper gastrointestinal study (UGI) can be
performed. UGI may be beneficial to exclude reflux, distal obstruction and malrota-
tion as sources of emesis. The radiographic findings on UGI that suggest HPS include:
the “string sign” from the narrowed pyloric channel, the “double track” sign from
the folding of the rugal folds, and the pyloric “beak” or “shoulder” signs from the
pyloric bulge into the antrum.
Pathology
Gross pathologic findings include a firm, bulbous pylorus with a smooth and
shiny serosal surface (Fig. 22.1). On cut section, the mucosa is pushed inward effec-
tively obliterating the pyloric channel. The lumen of the duodenum attains its full
size immediately distal to the hypertrophied pylorus unlike the proximal gastric
lumen which demonstrates progressive narrowing. Microscopic examination of the
pylorus reveals hypertrophy and hyperplasia of the circular pyloric muscle fibers.
Edema and nonspecific inflammatory changes are observed in the mucosa and
submucosa.
Treatment
The mainstay of treatment is surgical pyloromyotomy, a procedure formalized
by Ramstedt in 1912. Initial management of infants with HPS includes fluid resus-
citation appropriate to the degree of dehydration and severity of electrolyte
Hypertrophic Pyloric Stenosis 87
22
Fig. 22.1. Hypertrophied pyloric muscle characteristic of a neonate with hyper-

trophic pyloric stenosis.
abnormalities (i.e., hypochloremia, hypokalemia, alkalosis). A typical resuscitation

plan includes initial rehydration with normal saline (10-20 cc/kg boluses) until urine
output is established, followed by gradual potassium replenishment (i.e., D51/2 NS
with 20 meq KCl/l at 1.5-2 times maintenance rate) until electrolyte abnormalities
are corrected. Most infants can be operated upon within 24 hours of admission.
Pyloromyotomy is performed under general anesthesia. The traditional incision,

as described by Ramstedt, is a transverse right upper quadrant incision. Periumbilical
incisions are occasionally used and cosmetically superior, but have higher risk for
wound infection. The stomach is identified and the pylorus is delivered through the
incision. The hypertrophied pylorus is incised from the gastroduodenal junction
proximally to just beyond the extent of the tumor, being careful not to violate the
duodenal or gastric mucosa. The incised muscle is split further by dividing the
22 remaining circular muscle fibers using the blunt edge of a knife handle or other
spreading device. The intact mucosa bulges between the divided muscle edges. The
divided pylorus is grasped on each side of the pyloromyotomy and gently manipu-
lated to and fro to confirm complete separation of the muscle fibers. The pylorus is
replaced into the abdomen after the mucosa is closely reinspected for leak or bleeding.
Feeding is started 6-8 hours postoperatively. A pyloric feeding regimen is used to
gradually initiate and advance enteral intake. Most regimens begin with sugar water
followed by increasing concentrations and volumes of the child’s formula. Occa-
sionally, infants will continue to have small amounts of emesis when feedings are
initiated postoperatively. Parents should be forewarned and reassured regarding this
potential, usually self-limited, postoperative problem. The feeding volume is advanced
every few hours. The infant is discharged when po intake is adequate to maintain
hydration and meet estimated nutritional needs.
Outcomes
Infants tolerate pyloromyotomy very well. Average hospital length of stay is 1-3
days. Overall mortality is approximately 0.3%. Two uncommon complications of
pyloromyotomy are gastric/duodenal perforation and incomplete separation of muscle
fibers. Although perforations are easily repaired at the time of surgery, an unrecog-
nized duodenal perforation is a devastating complication presenting as diffuse peri-
tonitis and/or intra-abdominal abscess. Incomplete pyloromyotomy results in
prolonged postoperative feeding intolerance. Recurrence of HPS is extremely rare.
Selected Readings
1. Ramstedt C. Zur Operation der angelorenen Pylorus Stenose. Med Klinik 1912;
8:1702.
2. Schwartz MZ. Hypertrophic pyloric stenosis. In: O’Neill Jr. JA et al, eds. Pediatric
Surgery, 5th edition. St. Louis: Mosby 1998; 1111-1117.
3. Benson CD, Lloyd JR. Infantile pyloric stenosis: a review of 1120 cases. Am J Surg
1964; 107:429-433.
CHAPTER 1
CHAPTER 23
Intussusception
Vinh T. Lam
Incidence
Intussusception primarily affects infants and toddlers, although it can also occur
prenatally or during the neonatal period. Intussusception rarely occurs in adults.
The estimated incidence in the United States is about 1.5-4 cases per every 1,000
live births. Males are affected more than females at a ratio of 3:2. Male predomi-
nance is even greater in the 6-9 month age group.
Incidence peaks during two seasons of the year: spring/summer and middle of
winter. This seasonal variation correlates with times of increased number of cases of
viral gastroenteritis and upper respiratory infection.
Etiology
Intussusception is most commonly idiopathic and no anatomic lead point can
be identified. Several viral gastrointestinal pathogens (rotavirus, reovirus, echovirus)
may cause hypertrophy of the Peyer’s patches of the terminal ileum which may
potentiate bowel intussusception. Ileocolonic intussusception is the most common
type of intussusception in children (Fig. 23.1).
A recognizable, anatomic lesion acting as a lead point is only found in 2-12% of
all pediatric cases. The most commonly encountered anatomic lead point is a Meckel’s
diverticulum. Other anatomic lead points include polyps, ectopic pancreatic or gas-
tric rests, lymphoma, lymphosarcoma, enterogenic cyst, hamartomas (i.e., Peutz-
Jeghers syndrome), submucosal hematomas (i.e., Henoch-Schonlein purpura),
inverted appendiceal stumps, and anastomotic suture lines. Children with cystic
fibrosis are at increased risk of intussusception possibly due to thickened inspissated
stool.
Postoperative intussusception accounts for 1.5-6% of all pediatric cases of intus-
susception. Most of these patients develop small bowel intussusception following
operations that include retroperitoneal dissection as part of the procedure. Postop-
erative intussusception (Fig. 23.2) is the most common cause of intestinal obstruc-
tion in the first postoperative week.
The pathophysiology of the intussusception is that of bowel obstruction and
progressive bowel ischemia. As the intussuseptum becomes invaginated within the
intussuscipiens, the bowel wall and mesentery of the intussuseptum is compressed
causing venous and lymphatic occlusion, venous stasis, and edema. As the edema
23
Fig. 23.1. Ileocolonic intussusception (most common form of intussusception) at

laparotomy after barium reduction had failed.
Fig. 23.2. The rather rare enteroenteric intussusception, often the cause of an im-
mediate postoperative obstruction, possibly secondary to disordered peristalsis as
the postoperative ileus resolves.
Intussusception 91
increases and venous outflow becomes obstructed, arterial inflow is compromised.

Inadequate perfusion leads eventually to ischemic bowel necrosis.
Intussusception is primarily a disorder of infancy and occurs most commonly
between 5–10 months of age. Two thirds of children with intussusception are less
than 1 year of age at presentation. The principle signs and symptoms of intussuscep-
tion are:
1. vomiting (85%),
2. abdominal pain (83%),
3. passage of blood or bloody mucous per rectum (53%), 23
4. a palpable abdominal mass, and
5. lethargy. The classic triad of pain, vomiting, and bloody mucous stools
(“red current jelly”) is present in only one third of infants with intussus-
ception. Diarrhea may be present in 10-20% of patients.
The abdominal pain of intussusception is frequently acute in onset, severe, and
intermittent. During episodes of pain the infant will often draw his/her knees up to
the abdomen, scream inconsolably, and become pale and diaphoretic. Between pain
episodes, which may last only briefly, the child may be quiet and appear well. With
time, the child may become more ill and appear lethargic with increasing abdominal
distention, vomiting, and progression to shock with cardiovascular collapse.
Physical exam of the abdomen occasionally identifies a “sausage-shaped” mass at
the right upper quadrant or mid-abdomen. The right lower quadrant may feel empty
and the cecum may not be palpable in the right iliac fossa (sign of Dance). Rectal
exam may reveal a palpable mass if the intussusception has passed far enough dis-
tally. Prolapse of the intussusceptum from the anus is a rare event (1-3%). Fever and
leukocytosis are common findings. Tachycardia becomes more prominent as hypo-
volemia ensues.
The differential diagnosis includes intestinal colic, gastroenteritis, acute appen-
dicitis, incarcerated hernia, internal hernia, and volvulus.
Diagnosis
If the diagnosis is suggested by history and physical exam, several radiographic
studies can confirm the diagnosis. Early in the course of the illness, abdominal plain
x-ray may show a normal or nonspecific bowel gas pattern. Later, abdominal films
will show a more obvious pattern of small bowel obstruction with a relative absence
of gas in the colon. In 25-60%, abdominal plain films demonstrate a right upper
quadrant soft tissue density that displaces air-filled loops of bowel.
Ultrasonography of the abdomen is a reliable means to identify intussusception.
Two ultrasonographic signs of intussusception are:
1. the “doughnut” or “target” sign on transverse views, and
2. the “pseudokidney” sign on longitudinal views.
Barium or air contrast enema is the “gold-standard” diagnostic study for infants
with suspected intussuception (Fig. 23.3). It is both diagnostic and therapeutic in
identifying and reducing intussusception (see below).
23
Fig. 23.3. Ileocolonic intussusception within the transverse colon as demonstrated

by barium enema.
Treatment
Once a presumptive diagnosis of intussusception is made, the child should have
1. an intravenous line placed for rehydration,
2. a nasogastric tube placed for decompression, and
3. intravenous antibiotics started. A complete blood count, chemistry panel,
and type and screen are obtained.
Hydrostatic barium enema or pneumatic enema is used to confirm the diagnosis
and to reduce the intussusception. Hydrostatic reduction is contraindicated if the
child has signs of peritonitis or gangrenous bowel. A surgeon should be present at
the time of attempted reduction. In performing barium enema to reduce intussus-
ception, the barium column should be no higher than three feet above the patient.
Each attempt should persist until reduction of the intussusception fails to progress
for a period of 3-5 minutes. A maximum of three attempts should be made. Success-
ful complete reduction of the intussusception can be observed when the intussus-
ceptum passes through the ileocecal valve producing free flow of contrast into the
distal ileum. For pneumatic reduction, air is delivered into the colon via a transanally
placed foley catheter. An initial pressure of 80 mmHg is raised to a maximum pres-
sure of 120 mmHg. Reflux of air into the terminal ileum, seen flouroscopically,
signifies reduction of the intussusception.
If the intussusception is successfully reduced, the child is admitted for overnight
observation. Oral diet is resumed on the next morning. If the intussusception can-
not be completely reduced, operative intervention is indicated.
Surgery is indicated in children with:
1. clinical evidence of dead bowel,
2. peritonitis,
Intussusception 93
3. septicemia,
4. evidence of an anatomic/pathologic lead point,
5. failed enema reduction.
Surgical exploration for intussusception is performed through a right lower quad-
rant transverse incision. Retrograde pressure is applied by squeezing the intussus-
ceptum within the intussucipiens in a proximal direction. No “pulling” attempts
should be made at the ileal end. Following successful reduction, it is important to
assess bowel viability and search for anatomic lead points. Appendectomy is usually
performed but optional. Local or segmental resection is indicated if:
1. the intussusception cannot be reduced, 23
2. the segment of bowel appears infarcted or nonviable, or
3. a lead point is identified. Primary anastomosis can usually be performed
with minimal morbidity.
Fever, probably related to cytokine release and/or bacterial translocation, com-
monly occurs following reduction of intussusception whether performed surgically
or nonoperatively and should be anticipated.
Outcomes
Hydrostatic barium enema can successfully reduce intussusceptions in 50-75%
of cases. Success with air insufflation for reduction is even better and may be as high
as 95%. The recurrence rate of intussusception after successful reduction (whether
hydrostatic or surgical) is about 5-7%. Recurrence may be slightly lower with reduc-
tion using air insufflation. The mortality rate of intussusception is less than 1%.
Mortality increases with delay in diagnosis, inadequate fluid resuscitation, perfora-
tion, and surgical complications.
Selected Readings
1. Young DG. Intussusception. In: O’Neill, Jr. JA et al, eds. Pediatric Surgery, 5th
Edition. St. Louis: Mosby 1998; 1185-1198.
2. Ravitch MM. Intussusception in infancy and childhood: an analysis of seventy-
seven cases treated by barium enema. N Engl J Med 1958; 259:1058.
3. Hirschsprung H. Tilfaelde af Subakut Tarminvagination. Hospitals-Tidende 1876;
3:321.
4. Stringer MD, Pablot SM, Brereton RJ. Paediatric intussusception. Br J Surg 1992;
46:484.
5. Raffensperger JG, Baker RJ. Postoperative intestinal obstruction in children. Arch
Surg 1967; 94:450.
CHAPTER 24
Disorders of the Spleen

The spleen has several major functions. Hemopoietic production of the fetal
spleen continues until approximately 5 months of infancy, and the spleen is a stor-
age, as well as removal site for pathologic erythrocytes, leukocytes and platelets. The
splenic white pulp, which has the largest collection of lymphoid tissue, plays an
important immune function by producing immunoglobulins (IgM) as well as
opsonizing proteins (tuftsin and properdin). These proteins enhance neutrophil
phagocytosis and stimulate complement production. Circulation through the red
pulp allows splenic phagocytes to remove opsonized microorganisms with or with-
out specific antibodies.
Anomalies
Accessory spleens are present in 16% of the children undergoing splenectomy
and 25% of autopsy series. They are usually located near the splenic hilum, the tail
of the pancreas, the greater curvature of the stomach and, less frequently in the
splenocolic or splenorenal ligaments, the greater omentum, and the bowel mesen-
tery. An aggressive approach to remove all accessory splenic tissues is taken in those
patients undergoing splenectomy for hematologic and autoimmune diseases. Missed
accessory spleens can hypertrophy and manifest hypersplenism as late as 25 years
after splenectomy.
Asplenia, congenital absence of the spleen, is a syndrome often associated with
cyanotic congenital heart disease (i.e., transposition of the great vessels, truncus
arteriosus, anomalous venous return) and intestinal malrotation. Circulating eryth-
rocytes contain nuclear remnants (called Howell-Jolly bodies) usually removed by
the spleen. These patients have an increased susceptibility to serious infection.
Polysplenia is characterized by a normally functioning, multilobed spleen.
Polysplenia syndrome often accompanies absence of the inferior vena cava,
preduodenal portal vein, midgut malrotation, aberrant hepatic artery, situs inversus,
and bilary atresia. In fact about 10% of infants with biliary atresia have polysplenia
syndrome.
Splenectomy
Childhood diseases sometimes treated with splenectomy include congenital
hemolytic anemia (spherocytosis), chronic autoimmune disorders, hypersplenism,
splenic masses (cysts and tumors), and splenic trauma. The most frequent indica-
tions for splenectomy in childhood, excluding trauma, are hereditary spherocytosis
and refractory idiopathic thrombocytopenic purpura (ITP). Splenectomy for staging
Disorders of the Spleen 95
Hodgkin’s disease was performed routinely in the past; however, the practice is now
controversial due to the development of sensitive radiographic diagnostic tools and
the association of secondary acute myeloid leukemia (AML) in splenectomized
Hodgkin’s patients receiving chemotherapy.
Splenectomy can be readily performed through a left upper transverse abdomi-
nal incision. First, the ligamentous attachments of the spleen are divided. The spleen
is then delivered through the wound and the short gastric vessels are divided.
Reflecting the spleen laterally, the splenic hilum is mobilized from the tail of the
pancreas, and the splenic artery and vein are individually ligated and divided. Recently,
laparoscopic splenectomy has become a common approach with possibly decreased
morbidity compared to the open procedure. The common complications following
splenectomy in the acute period include atelectasis, pancreatitis, and hemorrhage.
The presence of Howell-Jolly bodies on peripheral smear reflects total splenectomy. 24
Hematologic Disorders
Hereditary Spherocytosis
Hereditary spherocytosis, the most common congenital hemolytic anemia, is
transmitted as an autosomal dominant trait. In this disease, red blood cells have an
abnormal spherical shape due to the deficiency of ankyrin, that is required for assembly
of the structural plasma membrane protein spectrin. This structural deficiency results
in membrane rigidity. Lack of the biconcave red cell shape leads to trapping and
destruction in the splenic pulp.
Clinically, patients present with varying degrees of anemia, jaundice, fatigue and
splenomegaly. The chronic anemia is usually mild, but infection can lead to a crisis
of rapidly developing severe anemia with generalized symptoms of headache, nausea
and abdominal pain. Jaundice tends to parallel the severity of anemia. Children
with long standing severe symptoms may exhibit signs of growth failure and cholelithi-
asis as a result of chronic hemolysis. The development of biliary tract calculi increases
with age and may reach 50% in adolescents.
Diagnosis is strongly suggested when there is a family history of spherocytosis
and a child presents in an anemic crisis. Peripheral blood smear reveals the presence
of spherocytes in combination with an increased reticulocyte count (5-20%) and a
negative Coombs test. Red blood cells exhibit an increased osmotic and mechanical
fragility in hypotonic saline. Infusion of labeled 51Cr demonstrates decreased red
blood cell trapping and destruction in the spleen.
Hereditary spherocytosis is the most common indication for elective splenec-
tomy. Splenectomy is performed soon after diagnosis if symptoms are marked or
there has been a hypoplastic crisis. However, unless clinical symptoms are severe,
splenectomy should be deferred until 4-6 years of age because of the increased sus-
ceptibility to sepsis. Neonates with severe hemolytic anemia and high bilirubin lev-
els may require urgent splenectomy to prevent brain damage due to kernicterus.
The gallbladder is examined by ultrasound preoperatively. If stones are present, com-
bined cholecystectomy with splenectomy is performed.
Idiopathic Thrombocytopenic Purpura

Idiopathic thrombocytopenic purpura (ITP) is an immune-mediated hemor-
rhagic syndrome in which antibody-sensitized platelets are destroyed in the
reticuloendothelial system. In children, the peak incidence is between 4 and 8 years
of age, with a prevalence of 1-13 per 100,000 patients. This is a generally benign,
self-limiting illness that occurs a few weeks after a viral upper respiratory tract infec-
tion. Patients with ITP have increased circulating platelet-associated IgG, and the
spleen is both the source of antiplatelet antibodies and the site of increased platelet
destruction.
Patients with the acute form of ITP usually present with the sudden onset of
ecchymosis, petechiae, and less frequently with epistaxis, bleeding gums, and hema-
turia. Central nervous system hemorrhage occurs in 1-3% of patients and is an
24 ominous sign with poor outcome. In the chronic form of ITP, the onset is insidious,
with cyclic remissions and exacerbations of symptoms, such as easy bruising and
petechiae. Splenomegaly is unusual in children with chronic ITP (2%) and is usu-
ally a manifestation of another underlying disease such as lymphoma. Bone marrow
aspirates are not indicated in these children, but if performed, show an increase in
the number of large megakaryocytes without platelet budding. Circulating antigens
or antibodies resulting from an infection may alter the platelet membrane, or im-
mune complexes may adsorb to the platelet surfaces resulting in opsonization and
destruction of immature platelets.
Approximately 75% of children with acute ITP will experience spontaneous
remission with normal platelet counts within 3 months of diagnosis. Ten to 20% of
patients may progress to the chronic form, which is defined as lasting > 6 months.
Acute ITP is primarily treated with limitation of antiplatelet drugs and pharmaco-
logic treatment with corticosteroids. Intravenous gammaglobulin (IVIG) may be
used in patients at higher risk for hemorrhage. Oral prednisone is administered at
2 mg/kg/day in divided doses for 1 to 3 weeks. Corticosteroids function by:
1. preventing phagocytosis of antibody-coated platelets,
2. diminishing binding of IgG to the platelet,
3. enhancing platelet production, and
4. decreasing antiplatelet antibody synthesis.
Infusion of IVIG, 400 mg/kg/day for 5 days, leads to a rapid rise in platelet
count in children with both acute and chronic disease, and functions to saturate
immune binding sites on mononuclear phagocytes, thereby inhibiting clearance of
platelets bound with autoantibodies. Chronic ITP patients should be worked up
thoroughly for other autoimmune diseases and/or connective tissue disorders (i.e.,
lupus erythematosis). Treatment is based on the severity of symptoms and thrombo-
cytopenia. Corticosteroids and IVIG are also the primary medical therapy for chronic
ITP. Plasmaphoresis, anti-Rh(D), and α-interferon may also be beneficial therapies.
Approximately 18% of children with chronic ITP will require splenectomy for
refractory disease. Preoperatively, the patients receive vaccinations against encapsu-
lated organisms. Long term remission nears 80% postsplenectomy but the remain-
ing 20% require administration of further medication to include cytotoxic
immunosuppressive drugs.
Disorders of the Spleen 97
Thalassemia
β-thalassemia, which is also known as Mediterranean anemia or erythroblastic
anemia of childhood, is a condition in which anemia results from a defect in the
erythrocyte. Frequent blood transfusions are required and patients often present
with splenomegaly. Splenectomy is indicated when the need for blood transfusions
increases markedly along with persistent elevation of reticulocyte count as well as
severe hypersplenism.
Cysts and Tumors
Benign tumors are rare and include splenic hamartomas, hemangiomas, adenomas
and lipomas. If symptomatic, partial splenectomy may be indicated. Lymphomas
are the most common malignant tumors involving the spleen. In children, lym-
phoma is far more commonly found as metastatic disease to the spleen. Angiosar- 24
coma is the primary malignant tumor of the spleen and frequently is metastatic at
the time of presentation.
The various splenic cysts include congenital cysts (Fig. 24.1), pseudocyst (gener-
ally arising after trauma), and parasitic cysts commonly caused by Echinococcus
granulosus. For all these conditions, symptoms indicate the need for splenectomy or
partial splenectomy.
Hypersplenism
Anemia, leukopenia, and thrombocytopenia due to increased destruction of cells
characterize hypersplenism. Patients have splenomegaly and evidence of bone mar-
row hyperplasia. In most cases there is some condition that produces splenomegaly
and finally hypersplenism Spherocytosis, ITP, Gaucher’s disease, sarcoidosis,
Hodgkin’s disease, portal hypertension, and parasitic infections (i.e., schistosomia-
sis, visceral leishmaniasis, malaria) are all examples of disease processes complicated
by hypersplenism. In most instances, the indications for splenectomy are relative
and require careful judgment.
Postsplenectomy Sepsis
After splenectomy, individuals are more susceptible to fulminant bacteremia after
splenectomy due to the following changes:
1. decreased clearance of bacteria from the blood,
2. decreased levels of IgM, and
3. decreased opsonic activity.
Overwhelming postsplenectomy sepsis (OPSI) is characterized by septicemia with
frequent meningitic involvement. The risk of sepsis is greatest in young children
and 80% of cases occur within 2 years after surgery. The overall incidence of OPSI
is 4.25%, but varies with age and underlying disease. Splenectomy for trauma and
incidental operative injuries carries the lowest risk (1.5-2%). The highest risk occurs
in patients with thalassemia and reticuloendothelial disorders (10-11%). OPSI risk
is 2-8% in patients with congenital hemolytic anemias or ITP. Regardless of the
incidence, mortality rates of OPSI approach 50%. Death can occur within 12-24
hours after the onset of symptoms and is frequently associated with adrenal hemor-
rhage (Waterhouse-Friderichsen syndrome). Prodromal signs are minimal; the patients
typically present with cardiovascular collapse and high fever.
24
Fig. 24.1. Large, posttraumatic splenic cyst shown at surgical excision
Streptococcus pneumoniae is responsible for the majority (50%) of reported OPSI

cases. Hemophilus influenzae, Meningococcus, Escherichia coli, and Staphylococcus are
the next most common offending bacteria.
Prevention is the key to overcoming problems related to OPSI. All patients un-
dergoing splenectomy require immunization with polyvalent capsular polysaccha-
ride antigens of pneumococci (Pneumovax), H. influenzae, and meningococci. The
vaccines should be administered 2 weeks prior to operation; however, if splenec-
tomy is urgent or emergent, they may be given in the postoperative period.
Pneumovax, which provides protection against 85% of all pneumococcal infections,
can provide adequate antibody levels for 3-5 years. Prophylactic penicillin is also
given to asplenic patients for several years, especially in younger children with addi-
tional prophylaxis required at the time of high-risk procedures such as dental work.
Selected Readings
1. Holdworth RJ, Irving Ad, Cuschieri A. Postsplenectomy sepsis and its mortality
rate; actual versus perceived risks. Br J Surg 1991; 78(9):1031-1038.
2. Imbach P, Kuhne T. Immune thrombocytopenic purpura ITP. Vox Sang 1998;
74(Suppl 2):309-314.
3. Lane PA. The spleen in children. Curr Opin Pediatr 1995; 7:36-41.
CHAPTER 1
CHAPTER 25
Rectal Prolapse and Anal Disorders

Steve Szczerba
Anorectal problems ranging from simple constipation to chronic and intractable

constipation, fistulae, fissure, prolapse, etc. are very common and bothersome in
childhood. This Chapter deals briefly with the more frequently encountered problems.
Constipation
Childhood constipation is definable as delay or difficulty in defecation to the
point of distress in the child. Encopresis is defined as overflow incontinence or
repeated soiling of underwear with stool that occurs in a child over 5 years of age.
Incidence
Soiling is reported in 3% of children older than 4 years of age. Most studies
reveal a male predominance (6:1 ratio) and 50% familial incidence.
Etiology
Various disorders can cause constipation and the most common etiologies are
very age dependent. No organic etiology is identifiable in most children (Table 25.1).
In infants less than 1 year of age anatomical and dietary factors predominate. In
older children, behavioral and dietary factors predominate. In children with neuro-
muscular disorders, constipation is a very common problem. Contributing factors
often include:
1. lack of coordination between abdominal muscle contraction and anal
relaxation,
2. difficulty passing stool in a supine position,
3. decreased fluid intake.
Chronic constipation develops typically between 2-4 years of age. In 50%, con-
stipation develops before toilet training. Episodes of constipation often increase when
the toddler is beginning to gain control over defecation. Typically, children with
constipation report chronic, recurring, nonspecific abdominal pain. Further ques-
tioning may reveal problems such as poor toilet training, enuresis, stools of very
large caliber, and soiling. In cases of typical, functional constipation, soiling occurs
during periods of activity. The child often reports no sensation of fullness or urgency.
Chronic encopresis may occasionally present as chronic diarrhea. The child mistakenly
Table 25.1. Causes of constipation in children
Common Causes: Normal variation

Dehydration
Excess cow’s milk
Dietary change: Change of formula
Change to cow’s milk
Introduction of solids
Anal fissure
Perianal streptococcal infection
Dysuria
Reluctance to use an unfamiliar bathroom
Anatomical Conditions: Anterior anus or rectum

Hirschsprung’s disease
Congenital rectal stenosis
25 Colonic stricture (after NEC)
Imperforate anus
Spina bifida occulta
Spinal cord tumor
Tethered spinal cord
Systemic Disorders: Hypothyroidism

Diabetes mellitus
Lead poisoning
Hypercalcemia
Diabetes insipidus
Cystic fibrosis
Neurologic immaturity
Miscellaneous: Meconium plug

MEN IIb
Intestinal psuedoobstruction
Visceral myopathy
Child abuse (Munchausen’s by proxy)
Chagas disease
Neurofibromatosis
Medications: Diuretics
Anticonvulsants
Supplemental Iron
appears to be straining to have a bowel movement when he or she is actually strain-

ing to retain stool.
Physical exam reveals a slightly distended, nontender abdomen. Stool is easily
palpable in the lower left quadrant. Inspection of the perineum is performed to
evaluate for anal fissures, cellulitis, anterior ectopic anus, or other anorectal disor-
ders. Digital rectal exam often reveals a shortened anal canal with decreased sphinc-
ter tone. The rectum is dilated and full of stool. The differential diagnosis includes
all etiologies listed in Table 25.1. Hirschsprung’s disease is considered, especially in
children with failure to pass meconium within the first 24-48 hours of life.
Rectal Prolapse and Anal Disorders 101
Diagnosis
Primarily, history and physical examination make the diagnosis. An abdominal
x-ray should be done to evaluate the intestinal gas pattern and rule out vertebral
anomalies. A contrast enema is useful to delineate anatomic or functional causes of
constipation. To be most useful, it should be performed without a bowel prep or a
digital rectal exam. Functional constipation will cause the colon and rectum to dilate
to the rectal verge. CT, MRI and ultrasound can sometimes be useful adjuncts to
evaluate anatomic abnormalities but are not used routinely.
Anal manometry with electromyography helps differentiate functional constipa-
tion from neurological problems. Electrostimulation of the perineum is sometimes
useful to determine the location of the anus relative to the sphincter complex in
constipated children suspected of having an abnormally positioned or anteriorly
displaced anus. Tissue biopsy or may be necessary to rule out Hirschsprung’s disease.
Pathophysiology 25
Functional constipation usually begins with a painful bowel movement from a
large stool, anal fissure, or a perianal or perirectal abscess. The child then fears dis-
comfort and stool is voluntarily held in the rectum by the external anal sphincter. As
the rectum dilates around the bolus of feces, the urge to defecate disappears. The
cycle of stool withholding causes rectal relaxation, reflex relaxation of the internal
anal sphincter, and dilation of the rectosigmoid. Continence then depends on con-
scious contraction of the external anal sphincter. When the child is involved in play
or is distracted, liquid stool passes around the fecal bolus and leaks past the external
anal sphincter and stains underclothes (encopresis).
Treatment
The treatment for chronic or acute constipation in the older child is a three-step
process that is outlined in Table 25.2. In summary, the treatment for constipation is
removal of stool in the rectum and the use of a combination of laxatives and behav-
ior training to keep the rectum empty and prevent stool reaccumulation. This allows
the rectum to return to its normal size and regain normal sensory and muscular
function. Adjunctive pharmacologic therapies can include oral administration of
cisapride, a prokinetic agent, and mineral oil.
Outcome
Approximately 60-70% respond during the first few months of treatment. Main-
tenance treatment for chronic constipation must be continued for at least 6 months
to a year.
Recurrence is common. The 5-year relapse period for children with chronic con-
stipation is approximately 20-40%.
Rectal Prolapse
Incidence
Rectal prolapse is a relatively common, self-limited problem in young children.
An exact childhood incidence is not known. Boys and girls are equally affected.
Table 25.2. Management plan for constipated child
Step 1. Eliminate impacted stool

Mild to moderate impaction
1. Bicosydyl suppository
2. Enemas of saline, mineral oil, or hyperphosphate
3. Oral mineral oil as a stool softener
4. Increase of dietary fiber with dietary adjustments and supplements
(Metamucil, etc.)
Severe impaction
1. Digital disimpaction
2. General anesthesia and disimpaction
3. Combination therapy as for mild impaction
4. Polyethylene glycol-electrolyte solution by nasogastric tube
Step 2. Establish a bowel regimen
Achieve a bowel movement every day.
25 1. Oral mineral oil
2. Increased dietary fiber.
3. Increase supplemental liquids (water, juices etc.)
4. Utilize the gastro-colic reflex by sitting on toilet after meals.
5. Use a foot stool if feet dangle
6. Repeat enemas or suppository if the child goes 2 days without a bowel
movement
Step 3. Maintain a healthy pattern for 6 months
1. Once the proper dose of laxative is established and the child has a soft,
comfortable bowel movement daily, maintain the laxative and bowel
regimen for 6 months.
2. If symptoms return, return to Step 1.
Etiology
There are two types of rectal prolapse: mucosal or full-thickness. Most cases of
rectal prolapse are idiopathic. Unlike adults, prolapse is only rarely associated with
chronic debilitating illness, traumatic injury, or malnutrition. However, rectal pro-
lapse is a well-known complication of cystic fibrosis (see below), and this diagnosis
should be excluded in all children presenting with this problem. In addition, chil-
dren with neuromuscular problems or bladder exstrophy often develop prolapse.
The peak age of occurrence of rectal prolapse occurs during the years 1-3. It is
frequently an intermittent problem, therefore a thorough history from the parent is
required. Most parents note the prolapse after defecation, however crying, coughing
or straining may also precipitate episodes of rectal prolapse. In some children, pro-
lapse occurs with each bowel movement, but abnormal patterns of defecation, such
as a constipation or diarrhea, often contribute. Mucosal bleeding from the surface of
the prolapsed bowel is not uncommon.
Upon examination of the anal region, rectal prolapse appears as a swollen rosette
of tissue that is slightly longer posteriorly than anteriorly. Sigmoid intussusception
can also have a similar appearance but is distinguishable by digital rectal exam. Pro-
lapse of mucosa alone may present with radial mucosal folds at the anal junction.
When the prolapse is full thickness, circular folds are seen in the prolapsed mucous.
25
Fig. 25.1. Rectal prolapse with substantial mucosal edema shown in the prolapsed
segment.
A rectal exam should be performed after the prolapse is reduced. If there is a history
of rectal bleeding, proctoscopy is indicated.
In addition to sigmoid intussusception, conditions to be considered in the dif-
ferential diagnosis are cystic fibrosis and conditions associated with tenesmus, such
as parasites, polyps, inflammatory bowel disease, and proctitis. Approximately 20%
of children with cystic fibrosis will develop some degree of rectal prolapse.
Diagnosis
The diagnosis is made by history and physical alone. Occasionally, colonoscopy
or contrast enema may be required to evaluate for lead points such as polyps. All
children should be tested for parasites (stool specimens for ova and parasites) and
cystic fibrosis (sweat test for chloride analysis).
Pathophysiology
At younger ages, anatomical and social factors may contribute to the develop-
ment of rectal prolapse. In infants and toddlers, the rectal mucosa is loosely adher-
ent to the underlying muscles. There is increased demand on the perineal musculature
for continence and toilet training. Additionally, flattening of the developing sacrum
redirects intraabdominal pressure toward the anus.
Treatment
Acute prolapse can easily be reduced by pushing the tip of the herniated bowel
into the anus. Once edema has developed, a gentle squeezing pressure may be required.
Parents must be taught to reduce the prolapse promptly if there is recurrence. Tap-
ing of the buttock has been used but is not always effective. To limit recurrence, one
must treat the precipitating cause and limit straining. Improvement in diarrhea or
constipation, delaying or limiting toilet training and medical treatment for parasites
or cystic fibrosis usually resolve the problem in 1-2 months. Rarely, if rectal prolapse
persists after conservative management, surgical intervention may be required. Scle-
rosing injections under general anesthesia in four quadrants linearly into the rectal
submucosa is one technique. Rarely, full thickness rectal prolapse may be resistant to
sclerosing techniques and an operation is indicated. Cerclage, creating a temporary
anal stenosis of the external anal sphincter, is a simple and usually effective tech-
nique. Posterior presacral rectopexy is a more invasive surgical treatment but is
effective. Surgical intervention should be reserved for only the most severe cases
refractory to simpler interventions.
Outcomes
Sclerosing techniques are up to 90% effective. Bleeding, infection, stricture and
abscess formation may complicate attempted sclerosis or surgical interventions.
25 Recurrence rates after surgery are low, however, constipation may be a complication.
Anal Fissure
Incidence
An anal fissure is a tear in the mucosa and the anoderm lining the anal canal.
These lesions are common in infancy and are the most common cause of bright red
blood per rectum in that age group (see Chapter 53).
Etiology
Anal fissures occur in the setting of constipation and passage of large, hard stools
that cause a mechanical tear of the anal mucosa. Diarrhea can cause a chemical
irritation from stool alkalinity. Pain associated with anal fissures may potentiate
constipation and seems to be related to hypertonicity of the anal sphincters.
Anal fissures in children most commonly occur during infancy. The usual pre-
senting symptom in that age group is bright red blood per rectum. Crying with
bowel movements and hard stool streaked with bright-red blood are the common
findings observed by the parents.
Gently spreading the anus (also having the older child bear down), exposes the
dentate line and the longitudinal tear comes into view. Fissures are most commonly
located in the posterior midline and distal to the dentate line. An unhealed fissure
may become infected and evolve into a chronic ulcer. If this occurs a sentinel skin
tag forms distal to the fissure, and the anal papilla may hypertrophy. Fissures are
sometime multiple and may occur anteriorly. Fissures located laterally suggest Crohn’s
disease or immunodeficiency states. Chronic anal fissures in older children may
indicate inflammatory bowel disease.
Diagnosis and Treatment
Anal fissures are diagnosed from history and physical. Acute fissures respond to
gentle anal dilation, stool softeners, laxatives, and Sitz baths. If fissures are second-
ary to underlying conditions, treatment is directed to these conditions as well. Fissures
associated with inflammatory bowel disease may be treated with metronidazole.

Topical anesthetic ointments after each bowel movement reduce sphincter spasm
and pain. A hypertonic anal sphincter may be treated with botulinum toxin and
topical nitroglycerine or a lateral subcutaneous internal sphincterotomy. Chronic
anal ulcers are surgically excised eliminating granulation/scar tissue while preserving
the sphincters. Leukemia and chronic immunosuppression are contraindications to
surgical intervention since such fissures fail to heal until these problems are addressed.
Outcomes
Most acute fissures respond to conservative measures and heal within 10-14 days.
Recalcitrant fissures respond to lateral internal sphincterotomy. This procedure
quickly relieves symptoms in 95% of cases and recurrence is less than 5%.
Perianal and Perirectal Abscess
Infants are commonly affected with infections and abscesses in the perianal area.
Infected diaper rash is the most common cause of superficial abscesses. Staphylococ- 25
cal or gram negative enteric organisms are the most common organisms involved.
Deeper abscesses of the anal canal or perirectal tissues arise from crypt infections.
These infections are usually caused by enteric and anaerobic organisms.
Girls and boys are equally affected when less than 1 year of age. With children
older than 1 year, males are more commonly affected. Parents frequently report the
presence of a perianal mass and sitting intolerance. For perianal abscesses, examina-
tion of the anus reveals a tender, erythematous mass lateral to the anus. Perirectal
abscesses are frequently associated with fever and malaise in addition to sitting intol-
erance.
On careful digital exam even deep perirectal abscesses may be palpable as a fluc-
tuant mass. Crohn’s disease may present as a perirectal abscess and should always be
considered. Rarely, infected rectal duplications or dermoid cysts can present clini-
cally as perirectal abscesses. Type III saccrococcygeal teratomas have been mistak-
enly identified and treated as perirectal abscesses. If the diagnosis is not obvious, CT
scan of the abdomen and pelvis with oral, rectal and IV contrast will demonstrate
most perirectal abscesses.
Treatment
Superficial perianal abscesses can be treated with Sitz baths. Typically antibiotics
are not required. If the area becomes fluctuant, incision and drainage may be offered.
Deeper infections require immediate drainage under general anesthesia along with
intravenous antibiotics.
Outcomes
One third of superficial perianal abscesses resolve without surgery but the major-
ity require surgical drainage. One third of these abscesses recur. Nearly 30-50% of
infants presenting with perianal abscesses actually have fistula-in-ano. Deeper infec-
tions heal well after incision and drainage. As with the superficial lesions, recurrence
is not uncommon.
Fistula-In-Ano
Although crypt abscesses are the usual cause of a fistula-in-ano, perianal ab-
scesses may be the inciting infection. In patients with perianal abscesses, up to 50%
will develop a fistula.
Children with fistula-in-ano have pain with bowel movements and frequently
have recurrent perianal infections that drain mucus. When the mucus stops drain-
ing, a small, indurated pustule will become evident. Occasionally dark stool may be
seen inside the tract. The clinical scenario and the physical exam are adequate to
make the diagnosis.
Classically, the cause of fistula-in-ano is a crypt abscess that extends to the peria-
nal skin. The fistula tract is usually intersphincteric (tracking between the internal
and external sphincters) or transphincteric (penetrating through the external sphincter
25 muscle tissue) connecting the crypt to the external perianal skin. In infants, the
fistula almost always extends straight radially from the involved crypt and opens on
the skin laterally. Goodsall’s rule does not apply in infants.
Treatment
Treatment is surgical. Antibiotics are used if there is associated cellulitis. Most
surgeons perform a fistulotomy to open the track over its entire length. A lacrimal
probe is passed through the tract and the overlying tissue is opened to the fibers of
the sphincter muscle. The tract lining is curetted and the wound is left open. Post-
operatively, Sitz baths, wound care and stool softeners are used. Rarely, a seton is
used for high, transsphincteric fistulas. Typically an 0-silk or a rubber band is pulled
through the tract with the probe. The seton is tightened or manipulated over the
course of a few weeks. The muscle fibers are slowly cut, allowing the fistula to be
unroofed without risking incontinence. If the tract is well developed with granula-
tion tissue, a fistulectomy is an alternative to fistulotomy.
Hemorrhoids
Hemorrhoids in children are unusual. Four to five percent of children with por-
tal hypertension may develop symptomatic bleeding rectal varices. The clinical pre-
sentation is variable with thrombosis occurring most frequently in teenagers.
Hemorrhoid thrombosis is frequently associated with heavy physical activity. Symp-
toms may be a report of a perianal mass that prolapses, rectal bleeding, or perianal
itching. Children with hemorrhoids frequently have an anal ulcer with a prominent
skin tag, rectal prolapse, or rectal polyp. Rectal duplications can rarely present as an
external hemorrhoid. Physical exam and history are usually adequate to establish the
diagnosis.
Treatment
When bleeding occurs in children with portal hypertension, sclerotherapy may
be attempted, however, direct oversewing is the definitive therapy. In children with
thrombosed hemorrhoids therapy depends on timing of presentation. If seen within
the first 24 hours of symptoms, incision and clot removal provides immediate relief
if pain is the presenting symptom. After the first day, spontaneous resolution is
underway. Rest, analgesics, stool softeners, and Sitz baths are then the treatment of
choice. Hemorrhoidectomy, the surgical procedure of choice, is reserved for chronic

hemorrhoids that do not respond to medical therapy. It has a low recurrence rate
(< 0.5%). Hemorrhoid surgery is contraindicated in most children who are
immunocompromised. Sclerotherapy or rubber-band ligation are recommended over
formal hemorrhoidectomy in children with hemorrhoids and concomitant inflam-
matory bowel disease.
Condyloma Acuminata
Perianal condyloma acuminata are caused by human papillovirus subtypes 6, 11,
16, and 18. Sexual abuse is associated in 60-90% of cases, however, vertical trans-
mission from nongenital skin of mother to infant is a possibility. Perianal warts
appear anytime from infancy to adulthood. Condylomata are found mostly on the
moist perineum in the perianal area. They appear as single or multiple sessile or
pedunculated, red, papillary excrescencies. The diagnosis is made merely by clinical
appearance.
Topical treatment with podophylline or bichloroacetic acid is the treatment of
25
choice for a small number of warts. Podophylline is diluted 1:4 with a tincture of
benzoin and applied to the individual warts on the perineum weekly. It should not
be applied to warts in the anal canal. The perineum needs to be washed at 2 hours
after application. Bicholoracetic acid may be used in the anal canal and is also applied
weekly. The warts turn white after application. Dilute sodium bicarbonate solution
is used to neutralize excess acid after application. When warts are numerous or in-
volve the anal canal, surgical therapy is more effective. Fulguration with electrocau-
tery, carbon dioxide laser ablation, or excision are the commonly used approaches.
One must be careful to minimize the loss of normal epithelium. Despite therapy,
the recurrence rate of condylomata is very high.
Selected Readings
1. Squires RH Jr. Common problems in pediatric gastroenterology. Comprehensive
Therapy 1996; 22(12):767-75.
2. Abrahamian JF, Lloyd-Still JD. Chronic constipation in children: a longitudinal
study of 186 patients. J Pediatr Gastroenterol Nutr 1984; 3:460.
3. Stafford PW. Other disorders of the anus and rectum, anorectal function. In: O’Neill
JA Jr., Rowe MI, Grosfeld, JL et al, eds. Pediatric Surgery, 5th ed. St. Louis: Mosby-
Year Book1998; 1449-1460.
4. Sempsky WT, Rosenstein BJ. The cause of rectal prolapse in children. Am J Dis
Child 1989; 142:338.
5. Ashcraft KW, Garred JL, Holder TM. Rectal prolapse—17 year experience with
the posterior repair and suspension. J Pediatr Surg 1990. 25:92.
6. Wyllie GG. The injection treatment of rectal prolapse. J Pediatr Surg 1979; 14:62.
7. Abercrombie JF, George BD. Perianal abscesses in children. Ann R Coll Surg Engl
1992; 74:385.
8. Poenaru D, Yazbeck S. Anal fistula in infants: etiology, features, management. J
Pediatr Surg 1993; 28:1194.
9. Shafer AD, McGlone TP, Flanagan RA. Abnormal crypts of Morgagni: The cause
of peri-anal abscess and fisutula-in-ano. J Pediatr Surg 1987; 22:203.
10. Heaton ND, Davenport M, Howard ER. Symptomatic hemorrhoids and anorec-
tal varices in children with portal hypertension. J Pediatr Surg 1992; 27:833.
Section IV: Pediatric Trauma
CHAPTER 1
CHAPTER 26
Initial Assessment and Resuscitation

Matthew L. Moront
Organization
The initial assessment is organized into three distinct phases of care:
1. the primary survey,
2. the transition phase, and
3. the secondary survey.
Primary survey
The purpose of the primary survey is to rapidly identify immediately life threat-
ening injuries and prioritize the management of these injuries. Life threatening prob-
lems identified in the primary survey are addressed immediately as they are identified.
The use of a systematic, standardized series of steps allows everyone involved to
anticipate and participate in an organized manner without a need for lengthy expla-
nations and without duplication of effort.
The primary survey is conducted expediently in the following sequence:
1. Airway and C-Spine Stabilization
2. Breathing
3. Circulation
4. Disability (Neurologic)
5. Exposure and protection from hypothermia
Transition Phase
The transition phase bridges the gap between the Primary and Secondary sur-
veys. This is the time for reassessment of the patient’s status. Many essential tasks are
accomplished during this phase. Consultants are contacted and trauma radiographs
are obtained. If transfer of the child to a trauma center is indicated, this process is
begun immediately.
Interventions such as gastric and bladder decompression, venipuncture for blood
type and crossmatch, and additional intravenous access procedures are performed.
If hemodynamic or clinical instability occurs at any time during the evaluation and
treatment process, a complete re-evaluation from the beginning is performed.
Secondary Survey
The secondary survey is a comprehensive evaluation of the patient to identify
and initiate treatment for all injuries.
Primary Survey
Airway
Establishing and maintaining a secure airway is the highest priority in the care of
an injured child. Protection of the cervical spine through proper immobilization is
essential until an injury can be excluded.
Assessment
Airway assessment begins as the child arrives by noting the child’s color, respira-
tory rate, mental status, and chest wall movement. Children with head injuries or an
altered level of consciousness (i.e., Glasgow coma scale (GCS) score of 8 or less) are
considered unable to protect their airways and require immediate intubation.
Treatment
All injured children receive high-flow supplemental oxygen and cardiorespira-
tory monitoring. Initial airway interventions include maneuvers as simple as clear-
ing the mouth and hypopharynx of secretions or foreign bodies. Other measures to
26 secure a protected airway include the jaw thrust maneuver, nasal or oral airway
placement, and endotracheal intubation. With careful assessment and early inter-
vention, a surgical airway is rarely necessary. A surgical airway is required when all
other interventions have failed or in cases of severe maxillofacial trauma.
Endotracheal intubation is best performed in a controlled setting by clinicians
experienced in pediatric airway management. Medications commonly used to pro-
vide amnesia, analgesia, sedation, and muscle relaxation for intubation include:
1. Atropine (0.01mg/kg) (min dose-0.1mg)
2. Lidocaine (1 mg/kg) head injuries
3. Thiopental (3-5 mg/kg) sedation/amnesia
4. Fentanyl (2 mcg/kg) analgesia
5. Vecuronium (0.1mg/kg) paralysis
Children with serious head injuries require lidocaine prior to intubation. Hypoxia
and hypotension contribute to secondary brain injury and must be avoided.
Gastric decompression and bladder catheterization are necessary in intubated
and paralyzed children and are accomplished as soon as possible following intuba-
tion. A portable chest radiograph confirms tube placement and excludes hemotho-
rax or pneumothorax.
Reassessment
The ability of an injured child to protect and maintain his/her airway must be
constantly reconfirmed. The initial assessment provides stimulation that helps main-
tain a satisfactory level of consciousness. A careful reassessment is required after the
initial resuscitation, but before leaving the trauma bay to ensure no change in the
child’s ability to protect or maintain his/her airway. Airway edema, anemia, hypov-
olemia, and increasing intracranial pressure can all cause delayed airway compro-
mise if not recognized and treated early.
Initial Assessment and Resuscitation 111
Breathing
Assessment
Assessment of breathing includes an evaluation of respiratory mechanics to insure
adequate ventilation. As with the airway assessment, this begins with a visual inspec-
tion of the child for signs of increased work of breathing or asymmetrical chest wall
movement. Other visual clues suggesting respiratory compromise include anxious-
ness due to hypoxia, nasal flaring, chest wall retractions, tachypnea, or a flail seg-
ment. The chest is palpated for signs of crepitus, penetrating injuries, tenderness,
rib fractures or chest wall instability.
Auscultation of the chest follows which includes evaluation for symmetrical breath
sounds in all lung fields. Normal heart tones and good air movement without stri-
dor or wheezing suggests adequate respiratory mechanics for oxygenation and
ventilation.
The chest wall of a child is more pliable than that of an adult. The ribs are more
cartilaginous, there is less musculature, and the mediastinum is less well fixed. Sig-
nificant underlying parenchymal injury can occur in the absence of rib fracture or
chest wall contusion. 26
Treatment
Treatment of the most common thoracic injury, pulmonary contusion, is largely
symptomatic and supportive. The same is true for rib fractures, even those associ-
ated with a flail chest. Careful monitoring combined with aggressive pain manage-
ment is frequently all that is necessary. Children with large flail segments that cause
respiratory compromise may require endotracheal intubation and mechanical venti-
lation if pain control does not allow adequate respiratory effort. Other common
thoracic injuries include pneumothorax and hemothorax. Both are treated with a
thoracostomy tube placed in the fifth intercostal space along the anterior or mid-
axillary line.
A tension pneumothorax compromises venous return of blood to the heart if not
immediately decompressed. Placement of a 16 or 18 gauge needle in the second or
third intercostal space along the mid-clavicular line is lifesaving in this situation.
This must be quickly followed by a thoracostomy tube to prevent reaccumulation of
the pneumothorax.
Thoracostomy tube placement for hemothorax may return a large amount of
blood requiring surgical hemostasis. Initial chest tube output of greater than 20 cc/kg
or sustained output of greater than 2 cc/kg/hr for more than four hours may require
surgical exploration for hemorrhage control.
Reassessment
Any intubated child requires repeated reassessments to insure proper endotra-
cheal tube position. Auscultation is performed after any patient movement for addi-
tional testing such as computed tomography scans, radiographs or operative
intervention.
Circulation
Assessment
Assessment of circulation involves evaluation for indicators of inadequate tissue
perfusion and identification of sites of active hemorrhage. Unlike adults, a child can
maintain a normal blood pressure despite losing 25-40% of his blood volume. Other
signs that indicate reduced circulating volume include:
1. an altered level of consciousness,
2. cool mottled extremities,
3. delayed capillary refill,
4. narrowed pulse pressure,
5. tachycardia, and
6. tachypnea.
Treatment
As a rapid assessment is being carried out, 2 large bore peripheral intravenous
catheters are placed. No more than 90 seconds is taken to secure peripheral venous
26 access. Children in shock often exhibit peripheral vasoconstriction so that obtaining
intravenous access can be a formidable challenge. A systematic stepwise approach is
required to prevent unnecessary delays in fluid resuscitation and restoration of cir-
culating volume. It is ideal if blood samples can be drawn for a type and crossmatch
and labs at the time that the venous access is placed.
Alternative access opportunities include saphenous vein cutdown, femoral vein
access via Seldinger technique or direct visualization, and placement of an interosseous
catheter for unconscious children in extremis. Lacerations demonstrating signifi-
cant hemorrhage are controlled with direct pressure.
Fluid resuscitation begins with a 20 cc/kg bolus of Lactated Ringers solution,
followed by a reassessment of vital signs, mental status, distal perfusion, etc. A sec-
ond bolus of 20 cc/kg of crystalloid is administered if signs of inadequate tissue
perfusion persist. A third bolus of Lactated Ringers solution can be given if neces-
sary, but further fluid requirements are met with a bolus of 10 cc/kg of type specific
packed red blood cells. Persistent fluid requirements, acidosis, or hemodynamic
instability suggest ongoing hemorrhage.
Disability
Assessment
A rapid evaluation of mental status, and examination for signs or symptoms of
head injury or gross peripheral sensorimotor deficit is made. The Glasgow Coma
Score (GCS) is noted. The cervical spine cannot be fully evaluated in children with
altered mental status; it must be protected until adequate evaluation can be made.
Treatment
Patients with findings or a scene report of diminished consciousness, loss of
consciousness, seizures, or posturing are evaluated as soon as possible by computed
tomography (CT) of the brain after initial assessment and reevaluation are complete
and the patient is stabilized.
Initial Assessment and Resuscitation 113
Reassessment
Careful reassessment is mandatory in children with head injury. The neurologic
exam can evolve during the primary assessment. This is also true for children with
spinal cord injuries. Children with suspected head injury require reevaluation every
15-30 minutes. Vital signs are carefully monitored. Hypotension and hypoxia are
avoided. Pupils are rechecked, and level of consciousness confirmed. Careful docu-
mentation and timing of the findings is extremely important.
Exposure
Assessment
After stabilization, all children brought to the resuscitation area must be care-
fully and completely examined for injury. All items of clothes including shoes, socks,
and undergarments are removed and the child is carefully rolled to each side while
spinal protection is maintained. All areas not easily visualized are exposed. This is
especially true of the back, perineum, occiput, and posterior cervical spine. Care is
taken not to miss injuries to poorly visualized areas such as limbs splinted to protect
intravenous lines or fractures, the area under the cervical collar, and the inside of the 26
mouth.
Quickly after a careful examination is conducted, the child is covered with warm
blankets. Care is taken not to prevent child’s temperature to fall below 36.5˚C. An
axillary temperature is taken upon arrival as part of the vital signs and reassessments
are made at intervals and when needed. Warmed fluids and blood are used through-
out the resuscitation.
Transition Phase
Studies
Reassessment of vital signs is followed by placement of additional intravenous
access if needed, placement of a nasogastric tube and urinary catheter, and ordering
labwork and initial radiographs of the chest, pelvis, and c-spine. Assistants are asked
to call consultants or initiate procedures to transfer the child to a trauma center.
Antibiotics and additional analgesia are administered if indicated. Immunization
against tetanus is administered.
Secondary Survey
In the secondary survey, a systematic and thorough examination of the patient
from head to toe is made to detect any peripheral, minor, or occult injury not ini-
tially recognized on the primary survey. Elements of the primary survey are re-evalu-
ated in order to gain a sense of clinical improvement or deterioration. Splints are
placed on injured extremities, nasogastric and bladder tubes are placed if not done
already, antibiotics, ultrasounds, CT scans, and other tests are ordered as indicated.
Tertiary Survey
The tertiary survey is performed to ensure that an injured child is not discharged
with an injury that has gone unrecognized. This is a final check of the child’s ability
to perform activities of daily living and appropriate movements that were not evalu-
ated previously. Prior to hospital discharge a member of the trauma team goes to the
bedside and conducts an age appropriate physical examination. In a toddler this

may simply be watching him put his shirt on or get out of bed. It is essential to
examine any extremity that may have been splinted for IV protection. Similarly, the
child must be seen to eat and ambulate normally prior to discharge. This survey may
identify nonserious injuries that parents would have been concerned about after
discharge. By conducting a tertiary survey many simple questions can be answered
which will reduce parent anxiety and eliminate frequent phone calls.
Selected Readings
1. American College of Surgeons Committee on Trauma. Advanced Trauma Life Sup-
port Course. Chicago, IL: American College of Surgeons 1997.
2. Glynn L, Statter MB. The ABCs of pediatric trauma. In: Arensman RM ed. Pedi-
atric Trauma: Initial Care of the Injured Child. New York: Raven Press 1995; 7-18.
3. Moore EE. Resuscitation and evaluation of the injured patient. In: Zuidema GD,
Rutherford RB, Ballinger WF eds. The Management of Trauma. Philadelphia:
W.B. Saunders 1985; 5.
26
CHAPTER 1
CHAPTER 27
Soft Tissue and Extremity Trauma

Daniel A. Bambini and P. Stephen Almond
Incidence
Soft tissue and extremity injuries are common and account for 25% of pediatric
trauma. Typically, these injuries involve skin, muscle, tendon, nerve, and/or bone.
Most fractures occur as isolated injuries, but frequently skeletal fractures and soft
tissue injuries occur in multiply injured children. Male children sustain fractures
twice as often as females.
Etiology
Soft tissue and extremity injuries are the result of blunt or penetrating trauma.
Motor vehicle accidents, falls, and assaults are the most common blunt mechanisms.
Guns, knives, impalement and animal bites (see Chapter 35) are the most common
penetrating mechanisms.
Children with soft tissue and/or extremity trauma present with a variety of symp-
toms. Injuries limited to the skin and subcutaneous tissue present with localized
pain and skin disruption. Injuries involving the underlying muscle or tendon may
present with hemorrhage or motor deficits. Vascular injuries present with hemor-
rhage and signs of ischemia (pain, pallor, paresthesias, paralysis, pulselessness, and
poikilothermia).
Diagnosis
Extremity injuries are diagnosed during the secondary survey. The extremity is
completely exposed and visually inspected for deformities, abnormal angulation,
penetrations, abrasions, shortening, swelling, and ecchymosis. Pulse, blood pres-
sure, sensory (light touch and two-point discrimination) and motor examination of
the injured extremity is performed and compared to the contralateral extremity.
Each joint is passively and then actively moved through its full range of motion.
Radiographs are obtained to confirm a fracture and must include at least two views
(anteroposterior and lateral views) and the joint above and below the fracture. If
there is a questionable finding, contralateral x-rays are indicated.
Management
Emergencies
There are five musculoskeletal emergencies:
1. open fractures,
2. open joint injuries,
3. dislocations,
4. vascular injuries, and
5. neurologic injuries.
Children with open fractures and open joints need antibiotics and operative
irrigation and debridement within 6 hours. Dislocations need to be reduced to pre-
vent neurovascular and bone injury. Vascular injuries need to be repaired within 4-6
hours to prevent ischemic injury to muscles and nerves. Fasciotomies should be
considered in cases of prolonged ischemia. Neurologic deficits need early attention
to determine the cause. Deficits due to ischemia require immediate vascular evalua-
tion whereas deficits due to nerve transection are less emergent.
Wound Management
The principles of wound management include pain relief, irrigation and debri-
dement, and wound closure. Factors influencing decision-making include the mecha-
nism and extent of injury, wound location and degree of contamination (i.e., clean,
dirty, foreign body), associated injuries, and patient age. Factors associated with a
higher incidence of infection and complications include large wounds (vs. small
27 wounds), high velocity gunshot wounds (vs. stab wounds and low velocity gunshot
wounds), clean wounds (vs. contaminated wounds), and wounds closed within 4-6
hours (vs. > 6 hours). Anesthetics are given topically and/or subcutaneously. Lidocaine
1% [with or without epinephrine (1: 100,000-200,000)] is given via 25 gauge needle
through the open wound. Antibiotics are given to children with the following inju-
ries/conditions:
1. high-energy mechanism of injury,
2. valvular heart disease,
3. lymphedematous tissue,
4. signs of wound infection,
5. contaminated wounds that are closed primarily, and
6. wounds with devitalized tissue.
Muscle and Tendon Injuries

Most muscle injuries are minor (strain) and complete disruption of the muscle
fascicles is unusual. Completely severed muscles require meticulous surgical repair.
Most muscle injuries are treated by an initial short period of immobilization fol-
lowed by active range of motion exercises. Overly aggressive rehabilitation and physical
therapy may increase fibrosis and precipitate myositis ossificans.
Tendon injuries and lacerations are best treated by early surgical repair. The
diagnosis of tendon injuries in the child with a lacerated hand or wrist can be diffi-
cult or impossible because the child is frequently crying, frightened, and uncoopera-
tive. Physical findings are frequently unreliable and delayed diagnosis is common.
Primary repair is the treatment of choice. If this cannot be accomplished, the skin
should be closed and an elective secondary repair is performed within 2-3 days.
Soft Tissue and Extremity Trauma 117
Fractures
Children have anatomic and physiologic features that alter the pattern of frac-
tures observed when compared to adults. The primary anatomic difference is the
presence of growth centers that vary with age of development. Pediatric bone is
relatively soft making it resistant to fracture but more prone to compression or col-
lapse as seen with “greenstick” or “elevation (torus)” fractures. The periosteum is
thicker and more elastic in children than in adults, which also contributes to a rela-
tively greater number of fractures demonstrating periosteal elevation rather than
displacement. Healing of fractures occurs at a faster rate in children than in adults.
Fractures involving active growth centers (see Epiphyseal and Physeal Injuries) in
children may cause growth failure, severe shortening, or abnormal angulation. In
most pediatric fractures, growth is stimulated. Children between 2 and 14 years of
age with long bone fractures are at risk of limb overgrowth.
Initial management of fractures includes splinting and immobilization. Immo-
bilization reduces pain and minimizes or prevents further soft tissue injury. Splint-
ing should be performed in the emergency department prior to transport for
additional studies or procedures. Tight circumferential bandages are avoided in trau-
matically injured extremities. Pain associated with fractures is often severe and nar-
cotic analgesia is usually necessary, particularly prior to reduction. If large doses of
narcotics are used, the child must be monitored for signs of respiratory depression. 27
Orthopedic surgeons most often provide definitive treatment of pediatric frac-
tures and early consultation is advisable. Two-view radiographs (AP and lateral) to
include joints both proximal and distal to the fracture site are obtained. Prior to
contacting the orthopedic consultant, it is useful to obtain information regarding
the neurovascular status of the involved limb, the complexity of the fracture (i.e.,
open vs. closed, simple vs. comminuted, displaced vs. nondisplaced), and mecha-
nism or energy/force of injury. Joint involvement should also be assessed.
Most fractures in childhood are treated with cast immobilization. Healing in
children is rapid and joint immobilization is less of a problem in children than
adults. Displaced fractures that are in close proximity to joints commonly require
closed reduction or open reduction with internal fixation. Fractures on both sides of
a joint frequently require internal fixation. Femur fractures in children are currently
managed using several treatment options including:
1. simple casting,
2. skeletal traction,
3. external fixation,
4. plate and screw fixation,
5. intramedullary (IM) rod insertion, and
6. others.
IM rod insertion is avoided in children less than 10 years of age to prevent dam-
age to the greater trochanteric physis.
Open fractures are always treated in the operating room under general anesthe-
sia. Wound cultures are obtained and intravenous antibiotics are started early.
Operative management of open fractures includes meticulous debridement, thor-
ough irrigation, and fixation of the fracture. Delayed wound closure at 42-72 hours
after the initial debridement is recommended.
Childhood fractures require close radiographic follow-up to assure that reduc-

tion is maintained. Casts may need to be changed often to maintain appropriate
alignment of the healing fracture. All fractures should be followed at least 6-12
months after injury to identify potential growth disturbance.
Epiphyseal and Physeal Fractures
The Salter-Harris classification is the radiographic classification system most
commonly used to describe physeal injuries in children (Fig. 27.1). Type 1 fractures
are usually the result of shearing or avulsion forces and are identifiable as separation
of the epiphysis from the metaphysis. This type of injury is most commonly observed
in young children but may be found in neonates as a birth-related injury. Type 1
fractures are relatively easy to treat, reduce easily, and heal quickly. Long-term prog-
nosis is excellent because the vascular supply to the epiphysis and physis is not dis-
rupted. Growth is not impaired.
Type 2 fractures are the most common physeal fracture of childhood. The frac-
ture line extends along the physis and crosses the metaphysis producing a metaphy-
seal fragment. This injury also results from shearing or avulsive force injury. Type 2
fractures are most commonly identified in children beyond 10 years of age. Like
Type 1 fractures, the blood supply remains intact and healing is rapid. Although
27 rare, growth arrest can occur with this type of injury. The treatment for Type 1 and
Type 2 fractures is closed reduction and casting.
In Type 3 fractures, the fracture line extends along the physis and crosses through
the epiphysis to involve the articular surface of the epiphysis. This type of fracture
results from shearing or avulsion forces applied to a ligament attached to the epi-
physeal fragment. This type of fracture frequently disrupts the vascular supply to the
epiphysis and can lead to partial growth arrest. Open reduction and fixation is com-
monly required with Type 3 injuries.
Type 4 fractures extend through the epiphysis, physis and metaphysis. Like Type
3 fractures, this is an intra-articular fracture. The fracture fragments are often com-
pletely separated which may cause the physeal plate to be offset. The blood supply is
frequently compromised which may lead to delayed healing or nonunion. Open
reduction is necessary to reduce the chance of growth disturbance or premature
physeal arrest.
Type 5 fractures the result of crush injury to the physis from axial loading forces.
These injuries are easily missed because they are clinically stable and may have no
associated radiographic findings. They are frequently associated with an ipsilateral
diaphyseal fracture. Growth arrest at the involved physis frequently leads to defor-
mity and mismatched extremity length. Treatment may require extensive procedure
including bone lengthening, contralateral bone arrest, or bone shortening procedures.
Tetanus Prophylaxis
Tetanus infection, although rare, is most likely to occur following puncture
wounds, dirty lacerations, and crush injuries in children with incomplete immuni-
zation. In the United States, children currently receive the tetanus immunization
series before 7 years of age. Children with clean minor wounds who have completed
the primary series of tetanus toxoid or have received a tetanus booster dose within
10 years require no additional therapy. If the immunization history is uncertain or
Soft Tissue and Extremity Trauma 119
Fig. 27.1. Salter-Harris classification of physeal injuries and fractures.
unknown or more than 10 years have elapsed, a booster dose of tetanus toxoid is
administered. For serious or contaminated wounds, a booster dose of tetanus toxoid
is administered if one has not been administered within the previous five years. If
the immunization history is unknown, children with puncture wounds or contami- 27
nated wounds should receive tetanus immune globulin in addition to a booster dose
of tetanus toxoid.
Selected Readings
1. Sullivan CM, Stasikelis P, Warren FH. Extremity trauma. In: Arensman RM et al,
eds. Pediatric Trauma: Initial Care of the Injured Child. New York: Raven Press
1995; 139-157.
2. Salter RB, Harris WR. Injuries involving the epiphyseal plates. J Bone Joint Surg
1963; 45A:587-622.
3. Rowe MI, O’Neill Jr. JA, Grosfeld JL et al. Musculoskeletal injuries. In: Rowe MI,
O’Neill Jr. JA, Grosfeld JL et al, eds. Essentials of Pediatric Surgery. St. Louis:
Mosby-Year Book 1995; 214-219.
4. Shafi S, Gilbert JC. Minor pediatric injuries. Pediatr Clin North Amer 1998;
45:831-851.
5. Kao SC, Smith WL. Skeletal injuries in the pediatric patient. Radiol Clin North
Amer 1997; 35:727-46.
CHAPTER 28
Facial Injuries
Bahram Ghaderi and Diane Dado
Incidence
Maxillofacial trauma in the pediatric population presents many challenges.
Although the management principles are the same for all age groups, the recon-
struction techniques required for children must take into consideration their devel-
oping anatomy, rapid healing, emotional immaturity and their long term potential
for deformity as a consequence of altered facial growth.
Numerous studies report the incidence of facial trauma in children as lower than
in adults. Pediatric facial fractures account for 1.5-15% of all facial fractures. Most
studies suggest an overall male preponderance, especially among adolescents. Chil-
dren under 5 years of age have a significantly lower risk of facial fracture only
accounting for 1-1.5% of the total. However, most data was collected prior to wide
availability of computed tomography (CT). It is likely that the incidence of pediat-
ric facial fractures is significantly higher than reported.
The nose and the mandible are the two most prominent features of the pediatric
face and are the structures most often fractured in children. Midfacial fractures are
rare in children. Associated injuries are common which reinforces the importance of
a complete initial assessment.
Etiology
There are many causes of pediatric facial injuries. They include traffic related
accidents, falls, sports injuries, and child abuse. Child abuse is a rare cause but should
be considered in every case, especially in children with recurrent injuries.
The diagnosis of pediatric facial fractures can be problematic. The clinician must
have a high index of suspicion, especially when other major injuries are present.
Children are frequently uncooperative with history and physical exam. At times
general anesthesia is needed to properly examine and evaluate a pediatric trauma
patient. History must often be obtained from parents or observers. Photographs of
the child before the incident are sometimes helpful, as are dental records, including
any models, from the child’s dentist or orthodontist.
Survey of the head and neck should proceed in an orderly fashion. First, the
neurological status of the child is assessed by evaluation of the neck, cervical spine,
inspection of the eyes, otoscopy, rhinoscopy and finally examination of the face and
oral cavity. Important parts of this include assessing function of the fifth nerve and
Facial Injuries 121
motor function of the seventh nerve. Facial skeleton examination begins with in-
spection followed by manual palpation. Facial asymmetry with edema, ecchymosis,
periorbital swelling, trismus, and malocclusion are all signs that suggest facial frac-
ture. A complete ophthalmologic and neurologic evaluation is important because of
a high incidence of associated injuries to the eyes and nerves of the face.
Diagnosis
With the advent of the CT scan pediatric facial fractures are no longer difficult
to identify. Plain x-rays consistently underdiagnose pediatric facial fractures. Because
of higher ratio of cancellous to cortical bone in children, there is higher incidence of
“greenstick” type fractures. CT provides detailed information on soft tissue and
bony structure with the added capability for three-dimensional reconstruction.
Pathophysiology
Unlike the adult, the pediatric facial skeleton is a dynamic and evolving struc-
ture. A child’s face has protective anatomic characteristics that reduce the likelihood
of facial fractures. Eighty percent of cranial growth occurs during the first years of
life. Although facial growth is also rapid during this phase, only after age 2 does the
face begin to grow faster than the skull. The orbit and the brain nearly complete
their growth by age 7. Lower facial growth continues into early adulthood. Because
children have a high craniofacial ratio, the incidence of skull fractures is higher than
that of severe midfacial fractures. In children the facial bones are not weakened by
the development of the paranasal sinuses, adding further relative protection from 28
facial fracture. Immature bone has greater elasticity which explains the higher inci-
dence of greenstick fractures in children as compared with adults. The pediatric
mandible and maxilla are also rendered more resistant to fractures by the presence of
unerupted teeth.
Treatment
The basic principles of trauma management in the initial assessment of children
with facial fractures is followed. Hypovolemic shock can result from blood loss from
the highly vascular face of a child. This is a double threat because much of the blood
can be lost in the airway. A variety of airway interventions are available. Oral intuba-
tion is favored over cricothyrotomies and emergent tracheotomies in the emergency
department. If necessary, tracheotomies should be done in the operating room. Once
the patient is stabilized from life threatening injuries, a definitive operative plan can
be made for reconstruction of facial injuries.
Rigid fixation has greatly improved results in adult facial fractures. Unfortu-
nately, this technique can not be applied indiscriminately in children as it may impair
facial growth. There is evidence that the pediatric skeleton has considerable ability
to remodel after injury. Conservative management of most facial injuries is war-
ranted. Rigid fixation should be used with caution and reserved for fractures that
cause the original features to be difficult to restore by other methods.
Wide versus limited exposure is another controversial issue. Complete exposure
of the fracture site is one of the basic tenets of maxillofacial trauma, but some sug-
gest that wide subperiosteal undermining may contribute to restriction of bone growth
from a scarred soft-tissue envelope. Until more experience is accumulated it is rea-

sonable to limit exposure to a minimum to decrease any potential for further injury.
Alloplastic materials are not recommended for use in children. Autogenous bone
harvested from the cranium provide excellent material for reconstruction. The tim-
ing of bone grafting in children is controversial and should be reserved for severe
deformity. It is important in the setting of a severe fracture to discuss with the par-
ents the possibility of secondary reconstructive procedures.
The mixed dentition in children presents a unique challenge for reconstruction.
The mandible and the maxilla are filled with tooth buds in various stages of erup-
tion. Injuries to this region can result in maldevelopment of permanent dentition.
Extreme care must be taken to avoid further injury by limiting debridement and
manipulation.
Selected Readings
1. Kolati PT, Rabkin D. Management of facial trauma in children. Pediatr Clin North
Am 1996; 43(6):1253-1275.
2. Bantz ML. Pediatric facial fractures. In: Pediatric Plastic Surgery. Appleton & Lange,
1997.
3. Ehrlich FE, Heldrich FJ, Tepas III JJ. Craniofacial Injuries. In: Pediatric Emer-
gency Medicine Aspen: 1987.
28
CHAPTER 1
CHAPTER 29
Head and Spinal Cord Injuries in Children

David Bentrem
Head Injuries
Incidence
Trauma accounts for nearly one third of all pediatric surgical admissions, and
approximately one half of these children will have sustained a head injury. One
child in 10 will suffer a significant head injury during school years, of which one
third require hospitalization. Boys are injured at a rate almost twice that of girls. The
peak age incidence for traumatic head injury occurs in teenagers.
Over 85% of brain injuries sustained in childhood are not life threatening. The
most common mechanism of head injury in children is falls. The two major causes
of severe brain injuries in children are motor vehicle accidents and child abuse.
Child abuse is the predominant mechanism of head injury in children less than two
years of age. Nonaccidental trauma is suspected and fully evaluated whenever a child
presents with brain injury inconsistent with the reported mechanism of injury.
The hallmark of traumatic brain injury is an altered level of consciousness. Both
the duration of unconsciousness and the duration of posttraumatic amnesia corre-
late with injury severity and outcome. The most widely used coma scoring system
for head injury is the Glasgow coma scale (GCS). This scale is useful for all age
groups, but the verbal score must be modified for children younger than four years
(Table 29.1). Traumatically injured children with a GCS of 8 or less have usually
sustained severe brain injuries. Children with a score of 9-12 often have moderate
brain injuries and are at risk for neurologic deterioration. The majority of head
injured children present with a GCS of 13-15 and only mild brain injuries, but still
there remains a small, but significant, risk for neurologic deterioration. Cranial nerve
abnormalities can result from fracture of the skull base. Clinical signs of basilar skull
fracture include “raccoon eyes” ecchymosis, hemotympanum, and cerebrospinal fluid
leak (i.e., otorrhea, rhinorrhea).
Children who have sustained a minor impact with no loss of consciousness, and
who are appropriately responsive shortly thereafter, are unlikely to have a serious
brain injury and can be released to a responsible and informed guardian after a
period of observation. However, the presence of other signs or symptoms, including
lethargy, irritability, vomiting, headache, or scalp swelling require further assess-
ment of injury. The lack of an adequate history favors hospital admission for
observation.
Table 29.1. Modified Glasgow coma scale (GCS) for age < 4
Score
Eye Opening (E)

Spontaneous 4
To speech 3
To pain 2
None 1
Best Motor Response (M)
Obeys commands 6
Localizes pain 5
Normal flexion (withdrawal) 4
Abnormal flexion (decorticate) 3
Extension (decerebrate) 2
None (flaccid) 1
Verbal Response (V)
Appropriate words, social smile, fixes and follows 5
Cries, but consolable 4
Cries, persistently irritable 3
Restless, agitated 2
None 1
Coma Score = E + M + V
Diagnosis
Radiographic studies provide essential information for evaluation of the severity
29 of injury. For clinically well-appearing patients, computed tomography (CT) can be
used to identify anatomic lesions as determine risk for deterioration (i.e., subdural
hematoma, epidural hematoma, etc.). Early and aggressive intervention can dra-
matically improve the outcome when instituted before clinical deterioration. It is
prudent to obtain CT scans on all children admitted with the diagnosis of head
injury. A normal CT scan after head injury is a significant indicator of good outcome.
Treatment
An awake child is admitted to an area where close monitoring of level of con-
sciousness, vital signs, and movement of extremities can be performed. Some chil-
dren will require intravenous fluids secondary to persistent vomiting. The intake,
output, and electrolytes of these children should be measured and monitored closely.
A syndrome of inappropriate antidiuretic hormone (SIADH) release and less com-
monly diabetes insipidus are sometimes observed in children following head injury.
Confusion, alteration in level of consciousness, and seizures result from severe
hyponatremia.
The initial management of the child with a severe head injury requires establish-
ing a stable, controlled airway. Endotracheal intubation with a nondepolarizing muscle
relaxant is performed using in-line cervical traction. All children with severe head
injuries are assumed to have a spine injury and maintained in cervical collars until
proven otherwise. Serial blood gases are acquired to verify adequate oxygenation
and ventilation. Tissue hypoxia and hypercarbia compound any intracranial insult.
Head and Spinal Cord Injuries in Children 125
Hyperventilation leads to cerebral vascular constriction and is a relatively safe and

effective, short-term method to rapidly reduce intracranial pressure.
Acute, expanding extracerebral hemorrhagic lesions causing mass effect are neu-
rosurgical emergencies. Epidural hematomas occur following injury of the middle
meningeal artery. They occur more commonly in young people because the dura is
not as tightly adherent to the skull. Neurologic outcomes following craniotomy for
epidural hematomas correlate with the presence or absence of coma at the time of
surgery. Acute subdural hematomas develop from tearing of cortical bridging vessels
that frequently result in expanding intracranial masses. As with epidural hemato-
mas, increased intracranial pressure (ICP) often necessitates immediate surgical evacu-
ation. Penetrating injuries and compound skull fractures are also neurosurgical
emergencies. As a general rule, skull fractures depressed the thickness of the skull or
more are reduced operatively.
For nonsurgical lesions systemic and intracranial pressure monitoring are impor-
tant. An arterial line allows for monitoring of systemic arterial pressure so that cere-
bral perfusion pressure (the difference between mean arterial pressure and ICP) can
be calculated. A cerebral perfusion pressure of 50-60 mm Hg is ideal. Elevated
intracranial pressures frequently require therapeutic intervention to prevent second-
ary brain injury. Unconscious patients and patients with open depressed skull frac-
tures are started on prophylactic anticonvulsant therapy.
The administration of osmotic agents such as mannitol indiscriminately to all
patients with severe head injuries is contraindicated. Hyperosmolar agents can
dehydrate the normal brain and may allow hematomas or edematous brain to expand.
Mannitol is used when there is clinical or radiological suspicion of mass effect, and
the patient is clinically deteriorating. 29
Most importantly, treatment begins with prevention. The majority of brain injuries
in children are preventable. Proper use of seat belts in motor vehicles and wearing of
helmets for bicycle riding, in-line skating, skiing and other activities reduces the risk
of serious head injuries in children.
Spinal Cord Injuries
The child with a spinal cord injury presents similar management issues as the
severely head injured patient. Not only can anoxia and ischemia make the primary
injury worse, but failure to provide proper initial management may extend the level
of irreversible neurologic damage.
Incidence
In the United States, fewer than 10% of the 8,000 yearly spinal cord injuries
occur in children. Approximately 50% of pediatric spinal injuries result from motor
vehicle accidents and 25% result from diving-related accidents. The incidence of
spinal cord injury increases with age, particularly after the age of 12. Male children
are more frequently injured than females (1.6:1). Younger children have dispropor-
tionately increased cervical spine injuries secondary to the relative largeness of a
child’s head compared to torso. Older children have a distribution of spinal injuries
that is similar to adults. Thoracolumbar or lumbar spine injuries are uncommon in
children and are most frequently associated with lap belt injuries.
Spinal cord injury presents as neurologic dysfunction below the level of sus-
pected injury. Complete or severe injuries result in a symmetrical flaccid paresis or
paralysis with accompanying sensory loss. Lesser injuries may present with transient
neurologic dysfunction. Any history of neurologic dysfunction involving the limbs
or bowel and bladder is strongly suggestive of spinal injury. Cervical spinal cord
injuries sometimes cause hypotension and bradycardia from disruption of sympa-
thetic tone.
Diagnosis
The radiographic evaluation of children with a suspected spine injury poses spe-
cial problems, and alignment is maintained at all times. Any untoward movement
may precipitate permanent neurologic damage and dysfunction. It is extremely
important to adequately visualize all seven cervical vertebrae when evaluating for
cervical spine injury. In unconscious trauma patients with potential for spine injury,
the entire spine is evaluated radiographically. Anteroposterior and lateral view plain
films are the initial diagnostic screening test. Almost 2/3 of spinal cord injuries in
children have no radiographic abnormality on plain films and clinical exam with
consideration of the mechanism of injury are important. Prevertebral soft tissue
swelling on the lateral cervical spine x-ray is an important and often subtle sign of
injury even when no fracture is apparent. Pseudosubluxation, a normal anatomical
variant with anterior displacement of C-2 on C-3, is present in 40% of children
younger than seven years of age. It can easily be confused with a cervical fracture/
dislocation. Lateral lumbosacral plain films are indicated in all children with lap belt
29 injuries before immobilization is removed. CT scan of the spine is helpful for detecting
subtle fracture, soft tissue swelling, and rotary subluxations. CT is also useful to
evaluate for suspected C-7 and T-1 injuries where plain radiographs often fail to
visualize fractures clearly. Magnetic resonance imaging (MRI) is useful to evaluate
the extent of the parenchymal cord injury and the relationship of the cord to sur-
rounding structures.
Treatment
Spinal cord perfusion is optimized by restoring and maintaining normal sys-
temic blood pressure and euvolemia. Central venous pressure monitoring is helpful.
Gastric decompression and elective intubation is performed in patients with respira-
tory compromise. Early administration of high dose glucocorticoids may improve
neurologic outcome in both complete and incomplete spinal cord injuries. Methyl-
prednisolone is administered as a 30 mg/kg bolus followed by a 23 hour infusion of
5.4mg/kg/hr.
The injured spine is maintained in alignment throughout care. The halo device
offers an excellent alternative means of managing cervical spine instability in young
children. Surgical therapy for reduction and stabilization is necessary in the pres-
ence of complex unstable fractures, irreducible subluxations, and penetrating injury.
The most important factor determining the subsequent outcome of spinal cord
injury is the initial extent of the injury. Other than preventing further injury, there
is little evidence that any surgical or pharmacological treatment improves outcome.
Head and Spinal Cord Injuries in Children 127
Selected Readings
1. American College of Surgeons Committee on Trauma: Advanced Trauma Life Sup-
port Instructor Manual, American College of Surgeons. Chicago 1997.
2. McLone DG, Yoon SH. Head and spinal cord injuries in children. In: Raffensperger
JG ed. Swenson’s Pediatric Surgery, 5th edition. Norwalk: Appleton & Lange 1990;
261–275.
3. Bell WO. Pediatric head trauma. In: Arensman RM ed. Pediatric Trauma: Initial
Care of the Injured Child. New York: Raven Press 1995; 101-118.
4. Leberte MA, Dunham WK. Thoracolumbar spine injuries in children. In: Arensman
RM ed. Pediatric Trauma: Initial Care of the Injured Child. New York: Raven
Press1995; 119-138.
29
CHAPTER 30
Abdominal Trauma
John Hijjawi and Daniel A. Bambini
Classification
Abdominal trauma is either blunt or penetrating. Blunt abdominal trauma rep-
resents about 84-95% of pediatric abdominal trauma. The most common mecha-
nisms of injury are motor vehicle accidents and falls. The most commonly injured
organs are the kidneys, spleen, and liver. Penetrating trauma is less common (5-15%),
usually occurs in adolescents and teenagers, and is more common in urban areas.
The most common mechanisms of injury are stab wounds, gunshot wounds, and
impalement injuries. The most commonly injured organs are liver, small bowel, and
colon.
Assessment
A team approach is the most efficient means to assess (Chapter 28) and stabilize
a critically injured child. The team consists of a pediatric surgeon (team leader), a
pediatric anesthesiologist (airway management), and two nurses. The team is
assembled in the trauma room prior to the arrival of the patient. While waiting, the
team leader contacts the transport team, assigns resuscitation duties, and insures
that all team members observe universal precautions. Important prehospital infor-
mation includes time of the accident, mechanism of injury, condition of other vic-
tims, estimated blood loss at the scene, vital signs, a list of possible injuries, and any
treatment given en route. The team leader should verify allergies, medications, past
medical history, the child’s last meal, and events surrounding the injury.
Dividing the abdomen into three nonanatomic areas allows the surgeon to gen-
erate a list of potential organ injuries and the need for diagnostic tests. The
intraabdominal abdomen is defined by the anterior axillary line laterally, the costal
cartilages superiorly, and the pubis inferiorly. It contains portions of the large and
small bowel, hepatobiliary system, spleen, stomach, and urinary bladder. The
intrathoracic abdomen is between the 4th intercostal space superiorly and the costal
margin inferiorly. It contains segments of the liver, spleen, stomach, and colon. The
retroperitoneal space is defined by the posterior axillary lines laterally and the fourth
intercostal space superiorly. It contains the great vessels, duodenum, pancreas,
ascending and descending portions of the colon and the genitourinary system
The abdominal exam begins with visual inspection looking for evidence of pen-
etrating injuries, seatbelt marks, abrasions, or retained projectiles. The perineum is
inspected for ecchymosis, hematomas and blood at the urethral meatus. The flanks,
back and buttocks are also inspected. Lacerations and/or blood near the vagina or
Abdominal Trauma 129
anus raises the suspicion of abuse. Palpation begins in an area without obvious injury.
The surgeon is sensitive to subtle signs of tenderness, rebound, or guarding
The child is log-rolled and the back, spine, buttocks, and anus visually inspected
and palpated for tenderness or deformities. The rectal examination is performed
last. The purpose of this examination is to assess sphincter tone, mobility and posi-
tion of the prostate, rectal wall integrity, and for the presence of gross blood.
Diagnostic Evaluation
Unless an indication for immediate celiotomy exists (Table 30.1), definitive
diagnosis requires imaging. Computed tomography (CT) is the preferred radio-
graphic examination for blunt trauma. It is relatively quick, noninvasive, and very
specific for solid organ injuries. However, CT requires the child to leave the trauma
room, be exposed to radiation, and cooperate. CT is essential to successful
nonoperative management of blunt pediatric abdominal trauma.
Ultrasonography is used in the trauma room to screen for solid organ injury.
Free, intraabdominal fluid suggests solid organ injury or intestinal perforation and
requires an abdominal CT and admission. Compared to CT, U/S is portable, faster,
less expensive, easy to repeat, and has no radiation. However, U/S is operator-
dependent and nonspecific.
Although the indications for diagnostic peritoneal lavage (DPL) are the same for
children and adults, DPL is performed less frequently in children. The technique
and interpretation is the same for both groups. Warm Ringer’s lactate (10 mL/kg up
to a maximum volume of one liter) is instilled, drained from the peritoneal cavity,
and sent for cell count, bilirubin, and amylase. Indication for laparotomy are the
same as adults (Table 30.2). Compared to CT, DPL is quicker and more sensitive
test for intraabdominal injuries. However, it is invasive, nonspecific, does not evalu-
ate the retroperitoneum, and leads to an increase in nontherapeutic laparotomies.
30
Splenic Injuries
Splenic injuries are the most common cause of intraperitoneal bleeding. The
severity of the injury is graded on CT scan. Grade I is a subcapsular or
intraparenchymal hematoma without capsular disruption. Grade II is a parenchy-
mal fracture outside of the hilum. Grade III is a fracture that enters the hilum and
grade IV is a shattered spleen. Most children with an isolated splenic injury can be
managed nonoperatively. This requires 24-48 hours of monitoring in the pediatric
intensive care unit, serial physical examinations, serial hemoglobins, and blood
replacement. The child is transferred to the floor when he is hemodynamically stable
and not requiring blood transfusion. On the floor, the child is kept on bed rest and
started on a diet. Most children are ready for discharge between 7 and 10 days.
Indications for laparotomy include blood transfusion > 40 cc/kg (or half the child’s
blood volume) and a suspected intestinal perforation. At operation, splenic salvage
(i.e., splenorraphy, partial splenectomy) is possible in over 50-60% of children.
Indications for splenectomy include patient instability, associated life-threatening
injuries, and grade IV injuries. Splenectomized children are vaccinated against Pneu-
mococcus and Hemophilus influenza and placed on penicillin to decrease the risk of
OPS (overwhelming postsplenectomy sepsis). In addition, parents are told about
the risk of OPS and to seek medical attention at the first sign of infection.
Table 30.1. Indications for laparotomy

1. Refractory hypotension despite adequate fluid resuscitation.
2. Blood transfusion requirements totaling half the patient’s estimated blood
volume.
3. Pneumoperitoneum.
4. Positive DPL. (see Table 30.2)
5. Obvious peritonitis on initial or subsequent physical examination.
6. Abdominal distension with associated hypotension.
7. Diaphragmatic injury.
8. Evidence of intraperitoneal bladder rupture on cystography.
9. Evidence of abdominal penetration with gunshot wound to abdomen. No
attempt should be made to predict missile trajectory.
10. Evidence of posterior fascial penetration on local exploration of abdominal
stab wounds.
Table 30.2. Interpretation of diagnostic peritoneal lavage (DPL)
Positive lavage if one or more are present:

1. Aspiration of more than 10 cc gross blood.
2. Grossly bloody lavage fluid.
3. Amylase level of greater than 175u/dl
4. RBC count: >100,000/mm3 (blunt trauma) or
>50,000/mm3 (penetrating trauma).
5. Presence of bile, stool, or bacteria.
Liver Injuries
30 Liver injuries are the second most common cause of intraperitoneal bleeding
and a leading cause of mortality in children with blunt abdominal trauma. Injury
severity is graded on CT into one of 6 grades. Grade I is a subcapsular hematoma
that is < 10% of the liver surface area or a nonbleeding, < 1 cm laceration of the
liver. Grade II is a subcapsular hematoma that covers 10-50% of the liver or a small
(< 2 cm), nonexpanding, intraparenchymal hematoma. Grade III is a subcapsular
hematoma > 50% of the liver, a bleeding subcapsular hematoma, an intraparenchymal
hematoma > 2 cms or a laceration of the parenchyma > 3 cms. Grade IV is a rup-
tured central hematoma or laceration involving 25-75% of one lobe. Grade V is a
laceration involving > 75% of a lobe or hepatic vein injury. Grade VI is hepatic
avulsion. Like splenic injuries, most pediatric liver injuries are less severe (Grades I,
II, III) and can be managed using a nonoperative approach. Indications for opera-
tion include > 50% blood volume replacement and hemodynamic instability. The
surgical principles used in the management of complex (Grade III, IV, and IV) liver
injuries include maintenance of large bore IV access, prompt replacement of blood
products, the use of steroids (30 mg/kg bolus), maintenance of normothermia, manual
compression of the injury to control blood loss, occlusion of the porta hepatis (Pringle
maneuver), finger fracture of devitalized liver to allow direct ligation of bleeding
vessels, debridement of devitalized tissue, and abdominal packing with re-explora-
tion in 24-48 hours for uncontrollable, life-threatening bleeding.
Abdominal Trauma 131
Pancreatic Injuries
Pancreatic injuries are also uncommon and frequently occur in association with
other injuries. Blunt trauma (70%) is more common and usually the result of motor
vehicle accidents, handle-bar injuries, or sharp blows to the epigastrium. Penetrat-
ing injuries are less common (30%). Pancreatic injuries are graded as contusions
(grade I), minor lacerations (grade II), suspected pancreatic ductal injury (grade III),
or severe crush injuries (grade IV). Grade I and II injuries are managed nonoperatively.
Grade III injuries are investigated with preoperative ERCP to rule out a ductal in-
jury. Partial disruptions can be managed nonoperatively, but complete disruptions
usually require distal pancreatectomy. Grade IV injuries require debridement of
devitalized tissue, external drainage, and treatment of associated injuries. Rarely,
Roux-en-Y pancreaticojejunostomy or pancreaticoduodenectomy are required.
Intestinal Injuries
Intestinal injury is seen in blunt (up to 18%) and penetrating (> 60%) abdomi-
nal trauma. Signs and symptoms include abdominal pain, abdominal distension
and tenderness, and vomiting. However, 16% of children are asymptomatic. Labo-
ratory tests are not helpful and up to 60% of children do not have free air on plain
film or CT. The presence of free fluid in the abdomen without solid organ injury
suggests intestinal injury and has been used as an indication for operation in adults.
Another option is to admit the child for serial examinations. Indications for opera-
tion include signs of peritonitis, extravasation on UGI or CT, free air, or a positive
DPL.
Selected Readings
1. Svoboda JA et al. Severe liver trauma in the face of coagulopathy: A case for tempo-
rary packing and re-exploration. Am J Surg 1992; 144:717-721.
2. Haller JA. The current status of nonoperative management of abdominal injuries 30
in children and young adults. Am Surg 1998; 64(1):24-27.
3. Jerby BL et al. Blunt intestinal injury in children: The role of the physical exami-
nation. J Pediatr Surg 1997; 32(4):580-4.
4. Patrick DA et al. Ultrasound is an effective triage tool to evaluate blunt abdominal
trauma in the pediatric population. J Trauma 1998; 45(1):57-63.
5. Almond PS et al. Abdominal trauma in children. In: Arensman RM, et al eds.
Pediatric Trauma: Initial Care of the Injured Child. New York: Raven Press 1995;
79-100.
6. Stylanios S. Controversies in abdominal trauma. Sem Ped Surg 1995; 4(2):116-19.
CHAPTER 31
Genitourinary Trauma
Kate Abrahamsson and Fawn C. Lewis
Renal Trauma
Incidence
About 90% of all renal injuries in children are due to blunt injury and approxi-
mately 10% of all pediatric blunt abdominal trauma involves the kidney.
Symptoms
Contusion is the most common renal injury in children. Flank pain or tender-
ness and hematuria (microscopic or gross) are the most common findings. Abdomi-
nal tenderness, flank mass, hematoma over the flank, or fractured ribs are important
signs suggesting the presence of renal trauma.
Hematuria is not always present despite severe renal injury. Children with renal
pedicle injuries and/or pedicle disruptions sometimes present without hematuria.
Thus, the degree of hematuria does not correlate with the severity of the injury.
Diagnosis
The most sensitive and specific test to evaluate blunt renal trauma is computed
tomography (CT) with intravenous contrast. If the patient is unstable or requires
immediate surgery, a ”one shot” intravenous pyelogram, is helpful to provide infor-
mation about a suspected renal injury and/or contralateral renal function.
Treatment
Microscopic hematuria in a single urinalysis without associated clinical findings
is followed with repeat urinalysis. Persistent microscopic hematuria requires evalua-
tion. Gross hematuria as a result of blunt trauma is evaluated by CT, and if there is
no extra-renal indication for operation, the patient is admitted, placed at bedrest,
and followed for resolution of the hematuria. Serial abdominal CT scans or ultra-
sound is helpful to evaluate for stability and resolution of hematomas. Short term
complications (secondary bleeding, abscesses, urinomas) and long term complica-
tions (arteriovenous fistulae, encysted hematomas, hypertension) are most often seen
after injuries with devascularization of segments of parenchyma, extensive hemor-
rhage and urinary extravasation. Extensive renal injuries sometimes require opera-
tive intervention (i.e., repair, nephrectomy, drainage).
Genitourinary Trauma 133
Ureteral Trauma
Ureteral trauma in children is an uncommon injury. Severe flexion or decelera-
tion is the usual mechanism for ureteropelvic avulsion and children with these inju-
ries are evaluated urologically by CT scan with intravenous contrast. It is important
to remember that hematuria is rarely seen with ureteral injury. The treatment of this
lesion is immediate surgical repair.
Bladder Trauma
Incidence
Bladder rupture is seen in 10-15% of all patients with pelvic fracture but also
occurs without pelvic fracture after severe blunt force to the abdomen with a dis-
tended urinary bladder.
Symptoms
The usual symptoms and signs of bladder rupture are diffuse lower abdominal
pain and tenderness and microscopic hematuria. If blood is present at the urethral
meatus, injury of urethra or bladder neck is considered and retrograde urethrography
is performed prior to insertion of a catheter into the bladder.
Diagnose
Bladder rupture is diagnosed by cystogram. Oblique and postevacuation views
are required.
Treatment
Extraperitoneal bladder rupture is managed by catheter drainage. Cystography is
performed after 7-10 days. If there is no extravasation, the catheter can be removed.
If there still is extravasation the catheter is left in place an additional 7 days and
cystography is repeated. If a laparotomy is performed for other intra-abdominal and
pelvic injuries, a suprapubic catheter is placed through a small cystostomy incision
to allow decompression and drainage. Intraperitoneal bladder rupture requires lap- 31
arotomy. This injury is usually occurs in association with injury of other intraperito-
neal organs. Intraperitoneal urine is rapidly absorbed leading to azotemia and acidosis.
A suprapubic catheter and a urethral catheter are inserted. Cystography is performed
after 7-10 days. If there is no extravasation the urethral catheter is removed and the
suprapubic catheter is left until normal voiding is established.
Urethral Trauma
Incidence
Urethral trauma is divided into posterior and anterior urethral injuries. Posterior
urethral trauma is associated by severe blunt trauma. About 5% of males with pelvic
fractures have injuries to the posterior urethra and 10-30% of these patients also
have bladder rupture. Any child with known or suspected pelvic fracture must also
be suspected of having injury to the lower urinary tract. Anterior urethral injuries
are rarely associated with pelvic fracture but most often occur following straddle
injuries or direct trauma to the perineal area where bulbous urethra is compressed
against the ischial rami.
Symptoms
Children with disruption of the urethra usually have a palpable bladder and are
unable to void. Frequently, blood is identified at the urethral meatus. For posterior
urethral injuries, rectal examination may reveal a pelvic hematoma. Anterior ure-
thral injuries are frequently associated with perineal or scrotal swelling and perineal
hematoma.
Diagnosis
Urethral injuries are evaluated with retrograde urethrocystogram. No further
instrumentation of the urethra is performed in boys. In females, the urethra and the
bladder neck are best evaluated by cystoscopy.
Treatment
A suprapubic catheter is inserted to drain the urinary bladder and antibiotics are
administered. Urethral reconstruction is generally not performed until the acute
inflammatory process and hematoma have resolved. Complications of urethral injury
include stricture, incontinence and impotence.
Scrotal Trauma
Incidence
Trauma to scrotum occurs infrequently and severe injuries are unusual because
of the size and mobility of the testes in boys. Injury occurs when the scrotum is
compressed against the inferior pubic ramus.
Symptoms
The diagnosis is obvious as pain and swelling of scrotum occur quickly. Testicu-
lar torsion can cause symptoms similar to those observed after scrotal trauma. Other
causes for painful scrotum are torsion of appendix of the testis, epididymitis, contu-
sion of the scrotal wall and scrotal hematocele with or without rupture of the testis.
31
Diagnosis
Scrotal ultrasonography is helpful but can never absolutely exclude the diagnosis
of testicular torsion.
Treatment
For scrotal wall contusion without testicular injury, symptomatic treatment is
recommended. If the testicle is ruptured or a large hematocele is observed or, explo-
ration is indicated to repair the torn tunica albuginea, control bleeding, drain any
hematocele and salvage the testis if possible. Testicular exploration is also indicated
if testicular torsion is suspected. Antibiotics are administered to avoid secondary
infection. Penetrating injuries require debridement if tissue damage is extensive.
Labial Trauma
Straddle injuries may cause severe hematomas and labial trauma. Anesthesia is
often required to perform an adequate investigation of vagina and/or urethra. When
the hematoma is extensive, there is a risk for urinary retention and urethral catheter
drainage is indicated.
Genitourinary Trauma 135
Penile Trauma
Injury to the penis in children is unusual and is most commonly seen after zip-
per injuries or after the toilet seat falls while the boy is voiding. Operation is rarely
needed. Gentle cleansing three times a day is usually the only treatment needed to
prevent secondary infection. Severe injuries to the penis are rare and an individual-
ized surgical approach is needed sometimes requiring microvascular repair and skin
grafting.
Sexual Abuse
Sexual abuse is always suspected in children with injuries to the perineal area.
Signs in boys include contusion and laceration of penis or scrotum and crush inju-
ries to the testis in boys. In girls, the signs of sexual abuse are labial contusion with
introital laceration and bleeding.
Selected Readings
1. Gonzales ET, Guerriero WG. Genitourinary trauma. In: Kelalis PP, King LR,
Belman AB eds. Clinical Pediatric Urology, 2nd Edition. Philadelphia: W.B.
Saunders 1985; 1125–1156.
2. Guice III SL. Urologic Trauma. In: Arensman RM ed. Pediatric Trauma: Initial
Care of the Injured Child, New York; Raven Press 159–172.
3. Livne PM, Gonzales ET. Genitourinary trauma in children. Urol Clin North Am
1985; 12:53–65.
31
CHAPTER 32
Thoracic Trauma
Matthew L. Moront
Introduction
Although thoracic injury is uncommon in children, it is associated with mortal-
ity rates of 20-30%. Sixty to 80% of children sustaining thoracic trauma have asso-
ciated injuries and nearly half of these children have concomitant head injury. There
is a dramatic difference in the mortality rate of children who sustain thoracic trauma
(10-15%) compared to those without thoracic trauma (1-2%). The mortality rate
for children sustaining isolated chest trauma is approximately 5%. The majority of
children with thoracic injury who die do so as a result of traumatic brain injury.
There are several differences in the types of chest injuries sustained by children as
compared to adults. The bones in children are more cartilaginous and pliable, and
thus will bend farther before they break. Therefore, children fracture ribs less com-
monly than adults but are twice as likely to sustain pulmonary contusions. Pulmo-
nary contusion is the most common thoracic injury in children. While pneumothorax
is common in both adults and children, the incidence of tension pneumothorax is
much higher in children because the mediastinum is more mobile and shifts more
easily.
Immediately Life Threatening Injuries
Pneumothorax
Pneumothorax occurs when air enters the potential space between the visceral
and parietal pleuras. Air enters this space from the inside due to a violation of the
visceral pleura. Air enters from the outside when the parietal pleura is violated. As
air accumulates in the pleural space the lung becomes compressed and the mediasti-
num shifts away from the side of pneumothorax. With severe mediastinal shift, the
venous return to the heart is impaired causing hemodynamic instability and even-
tual cardiac arrest.
Simple pneumothorax occurs in 30-40% of pediatric thoracic trauma victims.
Pneumothorax is most commonly identified in association with a rib fracture but
also occurs after blunt or penetrating chest injuries without an associated fracture.
Initial symptoms include ipsilateral chest pain, dyspnea, tachypnea, and restless-
ness. Pulse oximetry is frequently normal despite the presence of a large
pneumothorax.
Physical examination reveals absent or decreased breath sounds and hyperreso-
nance to percussion on the affected side and tracheal shift away from the side of the
pneumothorax. Jugular venous distention is sometimes observed with tension
Thoracic Trauma 137
pneumothorax, however, this sign is frequently not present in children with hypo-
volemia. The diagnosis of a tension pneumothorax is clinical; treatment is not delayed
while awaiting radiologic confirmation.
Immediate treatment of a tension pneumothorax is needle decompression in the
second intercostal space at the mid-clavicular line. Definitive treatment for any pneu-
mothorax is tube thoracostomy in the fourth or fifth intercostal space at the anterior
axillary line.
Hemothorax
Hemothorax occurs in 10-15% of pediatric thoracic injuries. The most com-
mon cause of a hemothorax is injury to a systemic vessel (i.e., intercostal vessel,
internal mammary artery, etc.). Other causes include hemorrhage from the great
vessels or the pulmonary hilum (often fatal) and bleeding from the lung paren-
chyma (5%). As with the diagnosis of pneumothorax, the anterior-posterior radio-
graph is very helpful for diagnosis. Treatment includes tube thoracostomy which
evacuates the blood from the pleural space and expands the lung to tamponade the
bleeding.
Children with large hemothoraces require special consideration. Evacuation some-
times reverses the tamponade effect of a large hematoma and results in vascular
collapse. Placement of 2 large bore intravenous lines with fluid warmers and the
immediate availability of type specific blood and an autotransfusion device is rec-
ommended prior to tube thoracostomy. Immediate evacuation of greater than
20 cc/kg of blood or the sustained loss of greater than 2 cc/kg/hr of blood over four
or more hours requires exploratory thoracotomy for hemostasis. Failure to evacuate
the majority of blood in the pleural space sometimes results in empyema or fibrothorax
(“trapped lung”) and requires prolonged hospitalization and thoracotomy for treatment.
Aortic Injury
Thoracic aortic injury is an uncommon injury in children and is almost always
due to severe deceleration or crush type injury. Injury to the aorta accounts for
approximately 2% of the unintentional deaths in children.
Although the risk of traumatic aortic rupture is higher in adults than children,
the risk of death from this injury is higher in children. The most common location
32
of aortic injury due to blunt trauma is similar in both children and occurs immedi-
ately distal to the takeoff of the left subclavian artery. The descending aorta is fixed
at this point, therefore, the shear stress encountered during a sudden deceleration is
greatest at this point
Aortic injuries in children are frequently accompanied by multi-system trauma
and 75% of the victims do not survive to reach a hospital. Of those children who
reach the hospital alive, over 50% will die within 24 hours. The overall mortality for
this injury is 90%.
The diagnosis of aortic injury is suspected when there is a history of significant
deceleration or crush mechanism of injury accompanied by physical exam findings
of profound shock, chest pain, and possible paraplegia. Other signs include hoarse-
ness, dysphagia or spinal injury. Chest radiograph findings suggestive of aortic injury
include:
1. mediastinal widening,
2. prominent aortic knob,
3. first rib fracture or scapular fracture,

4. elevated left mainstem bronchus,
5. deviated esophagus (deviated NGT),
6. left pleural effusion, and
7. obliterated AP window.
Children suspected of having aortic injury undergo immediate aortography.
Recently, dynamic thoracic computed tomography is gaining increased acceptance
in some centers as a sensitive diagnostic modality. Treatment includes emergent
thoracotomy, usually through a left posterolateral incision, and direct suture repair.
Pericardial Tamponade
Pericardial tamponade is very rare in children; a history of a penetrating wound
or of a severe deceleration is common. Physical signs include tachycardia, hypoten-
sion, muffled heart tones and distended neck veins. Children who present in hypo-
volemic shock will not manifest distended neck veins until very late in the
presentation, if at all. Although pulsus paradoxus is a prominent feature in adults
with this condition, it is often difficult to assess in an injured child. The diagnosis of
pericardial tamponade is suggested by an abnormally elevated or steadily increasing
central venous pressure. In the hemodynamically stable child transthoracic
echocardiogram confirms the diagnosis. The chest radiograph frequently demon-
strates a left pleural effusion or an abnormal cardiac silhouette. In the unstable child,
pericardiocentesis provides dramatic relief of symptoms and provides definitive
diagnosis. Aspiration of blood that does not easily clot confirms the diagnosis and
produces rapid clinical improvement.
Flail Chest
Flail chest occurs when two or more adjacent ribs fractured in two or more
places causing the injured segment to move paradoxically in relation to the remain-
der of the chest wall during respiration. Flail chest is a rare condition in children and
most commonly occurs as a result of direct blow to the chest wall. Most children
sustaining flail chest injuries also have severe underlying parenchymal injuries and
32 hemorrhage. Physical examination reveals an obvious chest wall deformity with pal-
pable crepitus and discordant chest wall movement. Ecchymosis and severe chest
wall tenderness are common. Chest radiograph confirms the diagnosis, although
the underlying pulmonary parenchymal injury is not always visible on the initial
CXR. Treatment requires aggressive and continuous pain control. Endotracheal
intubation is sometimes needed in children who are unable to maintain adequate
ventilation or oxygenation. Significant amounts of positive end expiratory pressure
(PEEP) helps to stabilize the flail segment and treat the underlying parenchymal
injury.
Potentially Life Threatening Injuries
Pulmonary Contusion
Pulmonary contusion is an injury to the lung parenchyma resulting from direct
trauma that causes hemorrhage, edema, and dysfunction. Pulmonary contusion is
the most common thoracic injury in children. In children, compliant chest walls,
decreased thoracic musculature, and cartilaginous ribs allow a significant transfer of
Thoracic Trauma 139
kinetic energy to the lung parenchyma without overlying rib or chest wall injury.
The pathophysiology of pulmonary contusion includes alveolar, vascular, and
epithelial disruption resulting in pulmonary edema, desquamative alveolitis, and
the release of inflammatory mediators. Clinically, children with pulmonary contu-
sion exhibit hypoxia, ventilation-perfusion mismatch, and atelectasis.
Extrathoracic injuries associated with pulmonary contusion include splenic lac-
eration and closed head injury. Associated intrathoracic injuries include mainly
hemothorax and pneumothorax which occur in over 50% of the children with sig-
nificant pulmonary contusion. Children with severe pulmonary contusion are tachy-
pneic, hypoxic, and dyspneic. Yet, the initial physical exam is often misleading and
these findings are absent in over 50% of cases. The initial chest radiograph usually
reveals patchy infiltrates or a small pleural effusion. The radiographic findings typi-
cally worsen over the ensuing 48 hours and correlate with the clinical findings.
Treatment is primarily supportive and involves aggressive pain management and
pulmonary toilet. Most children with pulmonary contusion do not require intuba-
tion or mechanical ventilation. Children who do require mechanical ventilation
have a 2-fold increased risk of pneumonia and an increased incidence of respiratory
distress syndrome.
Diaphragmatic Injury
Traumatic diaphragmatic injury is very uncommon in children and accounts for
less than 2% of all pediatric thoracic injuries. Over 90% of blunt injuries to the
diaphragm occur on the left side. Associated injuries are common, especially to the
abdominal viscera and pelvis. In children with blunt diaphragmatic rupture, there is
also an increased incidence of head injuries. Diaphragmatic injury is suspected and
ruled out in all cases of penetrating trauma below the level of the tip of the scapula
or the nipple line.
Radiologic findings suggestive of diaphragmatic rupture include:
1. the tip of the nasogastric tube above the diaphragm,
2. bowel gas or gastric bubble in the chest, and
3. obscured or elevated left hemidiaphragm.
Ultrasonography, computed tomography, and contrast studies have all been used to 32
make the diagnosis of diaphragmatic perforation, but all have high false-negative
rates for small perforations. Diagnostic peritoneal lavage, thoracoscopy, or laparo-
tomy are the most sensitive methods to identify diaphragmatic injuries. Treatment
is surgical repair.
Traumatic Asphyxia
Traumatic asphyxia is a syndrome consisting of cervicofacial cyanosis and sub-
conjunctival and petechial hemorrhages associated with varying degrees of central
nervous system and pulmonary dysfunction. It is rare and accounts for only about
one of every 18,000 trauma admissions. It is caused by a sudden and forceful ante-
rior-posterior compression of the chest against a closed glottis. A sudden increase in
intrathoracic pressure causes rapid retrograde flow through the valveless jugular sys-
tem with dilation of capillaries and venules. Neurologic symptoms, including
disorientation and agitation, usually clear within 24 hours and permanent disability
is unusual. Temporary visual loss secondary to retinal hemorrhages may occur but is
rarely permanent. These children have a characteristic cyanotic hue with marked
disparity between the cutaneous appearance of the head, neck, and upper extremities
as compared to the remainder of the body. Despite its alarming appearance the
survival rate for isolated traumatic asphyxia is over 90%. Associated injuries (i.e.,
pulmonary contusion, intra-abdominal injuries) are responsible for the majority of
deaths. Management includes airway stabilization, head elevation, and prevention
of hypoxia. The prognosis for the majority of children with this injury is excellent.
Tracheobronchial Rupture (TBR)
Tracheobronchial rupture occurs in less than 2% of children with thoracic trauma.
TBR affects older children with males greatly outnumbering females. The mortality
rate in children with TBR is approximately 30% and over half of these children
sustain severe associated injuries. The mechanism of injury is thought to be either a
sudden shearing force or compression causing a rapid increase in transverse thoracic
diameter and disruption of the tracheobronchial tree at fixed points near the carina
and cricoid. Nearly 80% of TBR occurs within 2cm of the carina and another 15%
occur in the more proximal trachea. The immediate management of children with
TBR includes securing the airway, ensuring adequate ventilation, tube thoracos-
tomy. Definitive treatment will usually require surgery. In cases with severe air leak
compromising ventilation, a double lumen endotracheal tube or selective contralat-
eral mainstem intubation is often helpful.
Esophageal Perforation
Esophageal perforation is a rare injury in children and occurs as a result of pen-
etrating trauma, foreign bodies, or iatrogenic injury. Children with esophageal perfo-
ration at the cervical level may present with torticollis, excessive salivation, and refusal
to eat. Eighty-three percent of penetrating esophageal injuries occur in the cervical
esophagus and 63% have an associated tracheal injury. Other physical signs include
subcutaneous emphysema, fever, shock, and a mediastinal crunch on auscultation
(Hamman’s sign). Cervical and chest radiographs identify foreign bodies and may
show pneumothorax, pleural effusion, or pneumomediastinum. Children with in-
tra-abdominal perforation commonly present with rigidity and tenderness. The di-
32 agnosis and treatment is usually surgical although nonoperative management is
sometimes possible. Overall mortality is approximately 15% but increases substan-
tially if the diagnosis is delayed beyond 24 hours.
Selected Readings
1. Peclet MH, Newman KD, Eichelberger M et al. Thoracic trauma in children: an
indicator of increased mortality. J Pediatr Surg 1990; 25:961-6.
2. Eichelberger MR. Patterns of thoracic injury. In: Eichelberger MR ed. Pediatric
Trauma: Prevention, Acute Care, Rehabilitation. St Louis: Mosby-Year Book, Inc.
1993.
3. Eichelberger, MR. Trauma to the airway and thorax. Pediatric Annals 1987;
16:307-316.
4. Borgen PI, Arensman RM. Pediatric Thoracic trauma: Six immediately life-threat-
ening injuries. In: Arensman RM ed. Pediatric Trauma: Initial Care of the Injured
Child. New York: Raven Press 1995; 53–63.
5. Hancock BJ, Wiseman NE. Tracheobronchial injuries in children. J Pediatr Surg
1991; 29:1316–1319.
6. Ali IS, Fitzgerald PG, Gillis DA et al. Blunt traumatic disruption of the thoracic
aorta: a rare injury in children. J Pediatr Surg 1992; 27:1281-1284.
CHAPTER 1
CHAPTER 33
Vascular Injuries
Daniel A. Bambini
Incidence
Pediatric vascular injuries are rare. The exact incidence is unknown. Approxi-
mately 1% of patients listed in the Pediatric Trauma Registry have suffered major
vascular injuries. Eighty percent of these children are greater than 5 years of age. For
penetrating vascular injury, the ratio of males: females is 3:1. Eighty percent of these
children are greater than five years old at the time of injury. Only about 50% of
these injuries will require surgical repair. The distribution of penetrating vascular
injuries by most common site is: upper extremity (50%), lower extremity (31%),
visceral (10%), neck (7%). Seventy percent of blunt vascular injuries in children are
lower extremity injuries. One half of these are popliteal artery injuries. Brachial
artery injury is the most common upper extremity blunt vascular injury.
Etiology
Pediatric vascular injuries are often the result of iatrogenic trauma. For infants
under 2 years of age, catheter related vascular injuries are most common. The pre-
dominant mechanism in this group is arterial injury during placement of catheters
or diagnostic cardiac catheterization.
For older children and adolescents, vascular injuries result from a wide variety of
blunt and penetrating injuries. Penetrating trauma is the mechanism of vascular
injury in about 75% of this group. Broken glass lacerations, gun shot wounds, and
knife injuries are the three most common types of penetrating injuries leading to
major vascular damage. Vascular injury from blunt mechanism occurs less com-
monly and a high index of suspicion is required to identify these injuries. Crush
injuries, displaced fractures, and joint dislocations are the blunt mechanisms most
often associated with vascular injuries. About 30% of long bone fractures in chil-
dren have associated vascular injury. The specific fracture/dislocations having the
greatest risk for vascular injury are: mid/distal femur fractures, elbow/knee disloca-
tions, tibial plateau injuries, and midshaft humerus fractures.
Vascular injury is suspected in all children with penetrating injury near or in
proximity to major vessels or severe blunt injuries to the extremities ( i.e., crush,
fracture/dislocation at joints, long bone fracture, etc.). The clinical signs of vascular
injury are pain, pallor, pulselessness, parasthesia, and paralysis. Massive bleeding
from an open penetrating wound is also highly suggestive. Absent pulse(s) distal to
the site of injury is the most conclusive sign of major vascular injury, yet distal pulses
remain palpable in at least 20% of children so injured. Delay in diagnosis is com-
mon (25%) with vascular injuries of the extremities in children but chronic ischemia
is rare (6%) as is the need for amputation (3-4%). Blunt or penetrating trauma can
result in a spectrum of pathologic vascular problems including: contusion with vas-
cular spasm, intimal tear, complete or partial transection, pseudoaneurysm, arterio-
venous fistula, entrapment, thrombosis. The most common blunt vascular injury is
an intimal tear with thrombosis.
Diagnosis
Although arteriography is commonly used in adults to evaluate suspected vascu-
lar injuries, it is infrequently used or necessary in pediatric patients. The complica-
tion rate of diagnostic angiography in young children is high; and it should be used
very selectively. The diagnosis of vascular injury is for the most part based on clinical
judgment; the extent of vascular injury is determined at surgical exploration as indi-
cated.
In general, patients with wounds associated with vigorous arterial bleeding, pulse
deficits, or expanding hematomas are taken to the OR expeditiously to control hem-
orrhage and repair major vascular injury. A penetrating wound in “proximity” to
major vessel is not of itself an indication for surgical exploration. Angiography is
useful in this group to identify vascular injuries that require operative intervention.
In addition angiography is indicated and commonly used to evaluate for major vas-
cular injury with:
1. penetrating zone I or II neck injuries,
2. pelvic fractures with massive bleeding,
3. failure to regain distal pulses after reduction of long bone fracture,
4. multiple penetrating extremity wounds,
5. severe crush injuries,
6. fractures/dislocations at the elbow or knee.
Treatment
The specific surgical intervention required to treat major vascular injuries de-
pends upon the type of lesion, anatomic location, as well as presence of other asso-
ciated injuries. The principles of general vascular surgery apply as well to children as
33 to adults. Vessels are repaired primarily whenever possible, but autologous vein or
PTFE conduits are necessary or useful at times. Small, nonvital vessels often are
simply ligated. Obviously, surgical repair is performed as expeditiously as possible to
restore blood flow quickly and limit ischemic tissue damage.
For extremity vascular injuries requiring operative repair, fasciotomy is often
necessary to treat or prevent compartment syndrome. Compartment syndrome de-
velop acutely in the postoperative period secondary to ischemia-reperfusion injury
of muscle/soft tissue. Early signs of compartment syndrome after vascular repair
include increasing severity of extremity pain, increased swelling and tenderness be-
low area of injury, pain increased with passive movement of toes/fingers, compart-
mental pressures measured greater than 30 mmHg. Fasciotomy should be performed
as soon as possible to treat compartment syndrome to limit soft tissue loss and
myonecrosis. Early fasciotomy is considered for cases of:
Vascular Injuries 143
1. combined artery and vein injury,

2. arterial injury with severe soft tissue damage,
3. progressive postoperative edema,
4. prolonged (> 5 hr) cold ischemia time,
5. early signs of compartment syndrome.
Outcomes
Penetrating vascular injuries in children result in amputation in only 3-4% of
cases. Blunt extremity vascular injuries are often more difficult to identify and de-
layed diagnosis is frequent. Amputation rates for blunt vascular injuries of the ex-
tremities are much higher than that of penetrating injuries: lower extremity (25%)
vs. upper extremity (17%). Vascular injury is a marker for overall increased severity
of injury. The mortality rate in traumatized children with major vascular injuries is
about 20% compared to a rate of 2-3% in all other injured children without vascu-
lar injury.
Selected Readings
1. King DR, Wise W. Vascular injuries. In: Buntain WL ed. Management of Pediat-
ric Trauma, 1st Edition. Philadelphia: W.B. Saunders 1994; 265-276.
2. Evans WE, King DR, Hayes JP. Arterial trauma in children: Diagnosis and man-
agement. Ann Vasc Surg 1988; 2:268-270.
3. Richardson JD, Fallat M, Nagaraj HS. Arterial injuries in children. Arch Surg
1981; 116:685-690.
33
CHAPTER 34
Burns
P. Stephen Almond and Heron E. Rodriguez
Incidence
Two million people are injured and 12,000 die each year in the United States
due to burn injuries. About 500,000 are evaluated in emergency rooms, 75,000 are
hospitalized and 25,000 are admitted to burn centers. One third are children under
6 years with most being less than 2. Males and those in lower socioeconomic groups
are at higher risk.
Etiology
Burn injuries are caused by thermal energy, electricity, or chemicals. Thermal
injuries are the result of scald (85%) or flame (13%) burns. Electrical and chemical
burns are less common (about 2%). Child abuse is documented in about 8% of
burn injuries and is suspected but not proven in another 8%.
Pathophysiology
The body’s response to a burn can be divided into an ebb phase and a flow phase.
The ebb phase is initiated by the burn. The burn destroys the skin, disrupting its
thermoregulatory and barrier function. Evaporative and heat losses are increased
and bacteria have direct access to the blood stream. It is estimated that 200 ml per
m2 of burned body surface area are lost every hour. In addition, inflammatory
mediators including interleukin (IL)-1, IL-6, tumor necrosis factor (TNF),
prostanoids, and oxygen free radicals are released resulting in increased capillary
permeability, edema, and further loss of intravascular volume. The flow phase is
characterized by the body’s homeostatic response to the burn. The hypothalamic
temperature regulatory center is reset, leading to an increase in metabolic rate,
hyperthermia, and a hyperdynamic state. Activation of the limbic system by pain,
fear and thalamic relay of nocioceptive stimuli results in an increase in circulating
cathecolamines. Cortisol and ACTH levels are increased leading to increased gluco-
neogenesis, proteolysis and lipolysis. Glucagon secretion is increased and facilitates
gluconeogenesis, glycogenolysis, lipolysis and ketogenesis. Peripheral resistance to
insulin can also occur. Antidiuretic hormone (ADH) secretion is increased and the
renin-angiotensin-aldosterone system is reset at an elevated level that is burn-size-
dependent. The result is fluid retention, edema, and dilutional hyponatremia.
Increased glomerular filtration and diuresis start 3-4 days after the injury.
Burns 145
Management
Burn patients should be evaluated and treated according to ATLS protocols,
with several special considerations. First, the burn process is halted by removing all
clothing and any objects that can retain heat, chemicals, or act as a tourniquet.
Chemical burns should be copiously irrigated with water. Neutralizing agents can
be dangerous and, in general, should not be used. Second, hypothermia is prevented
and pain diminished by providing a warm, draft-free environment and covering the
child with a clean dry sheet. Finally, the possibility of an inhalational injury or
carbon monoxide poisoning is considered in any child with prolonged smoke expo-
sure, loss of consciousness, carbonaceous sputum, singed facial hair, or signs of ther-
mal injury to the oropharynx. In these children, carbon monoxide levels drawn and
early intubation is strongly considered.
Airway, Breathing, Circulation
Burn victims are evaluated in warm, draft-free environments by physicians
observing universal precautions. All jewelry and clothing are removed, and humidi-
fied 100% oxygen is administered. Patients are evaluated for signs (facial burns,
carbonaceous sputum, singed nasal hair and tachypnea) or symptoms (burned within
a confined space, altered level of consciousness, and hoarseness) suggestive of an
inhalation injury or carbon monoxide poisoning. Fiberoptic bronchoscopy is diag-
nostic for an inhalational injury and can be used as an aid to intubation. Airway
patency, however, does not guarantee adequate oxygenation or ventilation. Carbon
monoxide and smoke inhalation interfere with oxygenation and are treated by
manipulations of FiO2 and PEEP (positive end expiratory pressure). Circumferen-
tial, third degree chest burns restrict ventilation and are treated with escharotomies.
Two large bore intravenous lines are started in the upper extremities, preferably (but
not necessarily) through nonburned areas. Lower extremity lines have a higher rate
of infection.
Secondary Survey
A thorough head-to-toe evaluation is performed. The eyes are inspected for cor-
neal injury. All skin is inspected and a neurovascular exam of the extremities is
performed to rule out vascular insufficiency or compartment syndrome due to a
circumferential, third degree burn. The child is log-rolled to inspect the back.
Fluid Resuscitation
The Parkland (Ringers lactate at 4 ml/kg/%BSA) and Shriners (Ringers lactate 34
at 5000 ml/m2/BSA burned plus 2000 ml/m2/BSA total/day) formulas provide
guidelines for fluid resuscitation. One half the calculated fluid is given during the
first 8 postburn hours and the second half over the following 16 hours. These for-
mulas serve only as guides and are adjusted based on hemodynamic status and urine
output (minimum 1 cc/kg/hr). In electrical injuries with extensive muscular injury,
myoglobinuria requires alkalinization of the urine (by adding sodium bicarbonate
to the IV fluids), osmotic diuretics (mannitol), and a higher urine output to prevent
the myoglobin from crystallizing in the renal tubules. Only rarely is invasive hemo-
dynamic monitoring required.
Burn Wound Care

The wounds are gently cleansed and ruptured blisters debrided. After the initial
debridement, the burn diagram is completed. The depth (Fig. 34.1) and size of the
burn are determined using either the rule of nines or the Lund-Browder chart
(Fig. 34.2). Transfer to a burn center is determined by American Burn Association
criteria (Table 34.1).
Topical agents are used to control microbial wound colonization and reduce
burn wound sepsis. Silver sulfadiazine is easily applied, causes no pain, and is the
mostly widely used agent. Its disadvantages are the rapid appearance of plasmid-
related resistance to sulfonamides and other antibiotics, limited eschar penetration
and occasional transient neutropenia. Silver nitrate is an effective antimicrobial with
no gram-negative resistance but, induces electrolyte imbalances and does not pen-
etrate eschar. Mafenide acetate penetrates the burn eschar rapidly. It is a strong
inhibitor of carbonic anhydrase and frequently causes metabolic acidosis and its
application can cause pain.
If circumferential burn eschars act as a constricting tourniquet, escharotomies
should be performed. Full thickness burns covering 25% BSA or less should be
excised and grafted without delay. For deep burns too extensive to be excised and
grafted in one procedure, staged excisions can be performed. Another alternative is
early complete excision with the use of a temporary skin substitute to cover the
wounds. Deep partial thickness burns can be treated with excision and grafting or
with expectant therapy with similar long-term results.
The diagnosis of burn wound infection is clinical. Focal brown or black discol-
oration and conversion of a partial-thickness injury to full-thickness necrosis are the
most reliable signs of burn wound infection. The diagnosis is confirmed by wound
biopsy. The wound is cleansed of all antimicrobial preparations. The biopsy includes
eschar and underlying viable tissue. A portion is put in formalin and sent to pathol-
ogy and the remainder is sent for culture. More than 100,000 bacteria per gram of
tissue and bacteria within viable tissue are suggestive of burn wound infection. A
concurrent positive blood culture for the same organism suggest burn wound sepsis.
Identification and treatment of burn wound infections minimizes the depth of the
burn wound and graft loss.
Nutrition
The hypermetabolism of the postburn period requires aggressive nutritional sup-
port. To meet the increased energy expenditures, supplemental caloric intake is
34 needed. Metabolic energy expenditure (MEE) by indirect calorimetry is the best
method for determining caloric requirements. MEE multiplied by a factor of 1.3
gives the number of calories required to maintain pediatric burn patients. Alterna-
tively, several formulas can estimate with fair accuracy the caloric intake needed (see
Chapter 7). In general, 20% of total calories should be provided as protein, 20% as
fat and the rest as carbohydrates. To achieve positive nitrogen balance, the recom-
mended protein intake for children less then 1 year of age, is 3-4 gm protein/kg and
1.5-2.5 gm protein/kg for older children. Randomized trials show aggressive pro-
tein feeding decreases infection rates and improves neutrophil function. Also, burn
patients receiving 9% of protein as arginine had lower infection rates and decreased
hospital stay. Diets containing 15-20% of nonprotein calories as fat appear to be
Burns
Fig. 34.1. Burn depth classification and characteristics. The initial classification of burn depth is based upon clinical criteria and the surface
characteristics of the wound. The initial estimation of burn depth is frequently revised because whether a burn is ultimately partial or full
thickness often requires several hours to days. Adapted with permission from Uitvlugt ND, Ledbetter DJ: Treatment of pediatric burns. In:
Arensman RM, ed. Pediatric Trauma. Initial care of the injured child, New York: Raven Press 1995; 179.
147
34
Fig. 34.2. Modified Lund-Browder chart for estimating and recording the total ex-
tent of partial and full thickness burns. Adapted with permission from Uitvlugt ND,
Ledbetter DJ. Treatment of pediatric burns. In: Arensman RM ed. Pediatric Trauma.
Initial care of the injured child. New York: Raven Press 1995; 179.)
Table 34.1. American Burn Association criteria for referral to a burn center
Partial-thickness and full-thickness burns greater than 10% BSA in patients
under 10 years old
Partial-thickness and full-thickness burns greater than 20% BSA in older
children
Partial-thickness and full-thickness burns involving face, eyes, ears, hands, feet,
genitalia, perineum, or overlying major joints
Full-thickness burns greater than 5% BSA at any age
Significant electrical burns including lightning injury
Significant chemical burns
34 Inhalation injury
Burn injury in patients with preexisting illnesses or conditions that could
complicate recovery or increase mortality and morbidity
Any burn patient in whom concomitant trauma poses an increased risk of
mortality and morbidity should be treated at a trauma center until stable before
transfer to a burn center
Children with any burn seen in a hospital without qualified personnel or
appropriate equipment for their care
Suspected child abuse or neglect, or patients requiring special social, emo-
tional, or rehabilitative support
Burns 149
optimal, with at least 4% of the total calories in the form of linoleic acid to prevent
essential fatty acid deficiency. Larger amounts of fat can have an immunosuppres-
sive effect by stimulating the release of arachidonic acid.
The oral or enteral route or maintaining adequate nutrition is preferred, but if
the oral route is not possible, or if the total BSA burned is > 20%, an alternative
route should be considered. Enteral nutrition increases gut blood flow, preserves
mucosal integrity, maintains bowel function, and prevents bacterial translocation. It
has a lower incidence of metabolic imbalances, avoids the potential complications
of catheter insertion and catheter-related infections and is significantly less expen-
sive than TPN (total parenteral nutrition). Immediate intragastric feedings after
burn injury (within 6-24 hr) have been shown to be safe and effective. Burn patients
receiving nasoduodenal tube feedings throughout the operative and perioperative
period, have a lower infection rate than those whose feedings are held. Complica-
tions related to the enteral nutrition are rare. Increased mortality has been associated
with the use of TPN in patients with severe burn injuries.
Pain Control
Pain control in the pediatric population is often under emphasized. Adequate
pain relief, especially in burn patients, is essential. For severe burn injuries, this may
require the assistance of an anesthesiologist or pain service.
Suggested Readings
1. Duarte AM. Environmental skin injuries in children. Curr Opin Pediatr 1995;
7:423-30.
2. Germann G, Cededi C, Hartmann B. Postburn reconstruction during growth and
development. Pediatr Surg Int 1997; 12:321-6.
3. Wolf SE, Debroy M, Herndon DN. The cornerstones and directions of pediatric
burn care. Pediatr Surg Int 1997; 12:312-20.
4. Pruitt BA Jr., McManus AT, Kim SH et al. Burn wound infections: current status.
World J Surg 1998; 22:135-45.
5. Uitvlugt ND, Ledbetter DJ. Treatment of pediatric burns. In: Arensman RM ed.
Pediatric Trauma. Initial care of the Injured Child. New York: Raven Press 1995;
173–199.
6. Lund CC, Browder NC. The estimation of area of burns. Surg Gynecol Obstet
1944; 79:52-8.
7. Bowser BH, Caldwell FT. The effects of resuscitation with hypertonic vs. hypo-
tonic vs. colloid on wound and urine fluid and electrolyte losses in severely burned
children. J Trauma 1983; 23:916-923.
34
CHAPTER 35
Bites and Stings

Heather Haukness and David Bentrem
Animal Bites
In 1992, there were an estimated 52.4 million dogs and 54.6 million cats kept as
pets. These large numbers of household pets account for most of the approximately
5 million Americans bitten annually. Animal bites overall account for up to 1% of
summer emergency department visits. Animal bite injuries are most often due to
dog bites (80%), but cat bites (6%) and human bites (1-3%) are also commonly
encountered. Bite wounds that require attention are often to the extremities, espe-
cially the dominant hand. Significant cosmetic and functional impairment accom-
pany severe bites. Facial bites are more frequent in children under 10 years old and
lead to 5-10 deaths per year secondary to exsanguinating hemorrhage.
Animal bite wounds are frequently contaminated with multiple strains of aero-
bic and anaerobic bacteria. Between 2-30% of wounds seen in the emergency
department become infected. Puncture wounds become infected more frequently
than avulsion-type injuries. Osteomyelitis is an infrequent, but severe complication
of bite wounds and is always considered when there is joint pain near the site of
injury. Patients with a history of asplenia, liver disease or other forms of
immunocompromise are at particularly high risk for infection.
Cat bites carry a much higher incidence of infection than dog bites, 35% vs. 4%.
Human bite injuries have an even higher infection and complication rate than either
cat or dog bite wounds. In general, anaerobic bacteria are recovered from over 50%
of all infected bite wounds. Pasteurella multocida, Staphylococcus aureus, and strepto-
cocci are common pathogens in infected dog and cat bites. Streptococci sp., Staphylo-
coccus aureus, Bacteroides sp., and Eikenella sp. are the most likely organisms recovered
from infected human bite wounds.
The medical history that is obtained regarding bite injuries includes:
1. the type of animal,
2. situation in which the bite occurred, and
3. the time course of the injury.
Child abuse must be considered in all cases of human bite injury. In addition,
concerns regarding potential exposure to viral diseases such as rabies (animal bites),
HIV, hepatitis B, and hepatitis C should be explored. If the bite injury is associated
with extensive tissue damage or is older than 8 hours, gram stain and aerobic/anaerobic
cultures should be obtained prior to starting antibiotic therapy. All wounds are co-
piously irrigated and devitalized tissue is surgically debrided. Immobilization and
Bites and Stings 151
elevation of affected limbs helps reduce swelling and hasten resolution of associated
cellulitis. Empiric antibiotic therapy should is given in all cases of:
1. puncture bite wounds (i.e., the majority of cat bites seen in the emer-
gency department),
2. head, hand, foot, or genital bite injuries;
3. bites with associated crush injury, and
4. bites in immunocompromised patients.
The drugs of choice are either oral amoxicillin/clavulanic acid or intravenous
ampicillin/sulbactam. In the penicillin-allergic patient, trimethoprim/
sulfamethoxazole in combination with clindamycin is an alternative regimen. Tetra-
cycline, although effective against P. multocida, is not used in children. Wounds in
proximity to bone should have baseline radiographs taken. A tetanus booster is ad-
ministered if the original 3-dose series is complete and a booster injection has not
been given in the previous five years. The primary series tetanus vaccine and tetanus
immunoglobulin is administered if the child has not completed the primary 3-dose
series.
Wound management options include primary repair or delayed primary closure.
Many physicians advise against primary closure, except with facial wounds. Punc-
ture wounds are best allowed to heal by secondary intention. Therapeutic failure is
usually from poor compliance with wound care, inappropriate antibiotic choice, or
failure to recognize joint penetration. For outpatient therapy, follow-up appoint-
ments are mandatory and wounds are seen and re-evaluated within 48 hours. Ulti-
mately, prevention is the best treatment of bite injuries. Children’s interactions with
pets should be closely supervised to prevent bite wounds. Stray animals should be
reported to local authorities.
Snake Bites
Venomous snakes, usually pit vipers (rattlesnakes, copperheads, or cotton mouths)
bite approximately 8,000 people in the United States yearly. Fifty percent of these
injuries occur in individuals less than 20 years of age. Snake bites occur more fre-
quently in males, usually on the distal extremity. The highest incidence of snake bite
is in the rural Southeast. Envenomization can cause extensive tissue destruction that
predisposes to infection. Symptoms are dependent on the dose of venom, the loca-
tion of the envenomation, size of the child, and whether the venom was delivered
subcutaneously or intravenously. Severe pain and edema of the affected area is often
associated with systemic symptoms such as nausea, vomiting, and diarrhea; or neu-
rologic symptoms such as fasciculations and paresthesias. Diagnostic laboratory tests
include creatinine phosphokinase, prothrombin time, fibrinogen, fibrin split prod-
ucts, and platelet count. Local pressure, immobilization, and a proximal constrict- 35
ing band is applied to impede lymphatic and superficial venous flow without
compromising arterial blood flow. The child is monitored closely and large bore
intravenous access is established. Antibiotics, analgesics, and a tetanus booster are
indicated. If the use of antivenom is anticipated, the child is tested for hypersensitiv-
ity to horse serum prior to administration.
Spider Bites
Arachnid bites, from the black widow or brown recluse spider in particular, are
treated with the accepted mainstays of wound care including tetanus prophylaxis
and patient monitoring. The decision to use antivenom is based on the severity or
rapidity by which symptoms progress. The bite itself is rarely painful. Paresthesias,
diaphoresis, and facial edema often occur along with generalized muscle cramps.
Muscle spasm can be treated with intravenous calcium or diazepam.
Stings
Hymenoptera (bees, wasps, & ants) venom is not very potent, however, children
are at special risk for severe reaction because of their small size, and therefore, rela-
tively higher systemic concentration. Bees are responsible for one half of stings, and
the yellow jacket is most likely to produce anaphylaxis. Reactions range from a direct
local tissue response, to serum sickness, to generalized anaphylaxis. The most fre-
quent systemic symptoms are urticaria, syncope, and respiratory obstruction. It is
estimated that 8 of every 1000 humans are allergic to insect stings.
Antihistamines, systemic corticosteroids, intravenous epinephrine, and airway
protection (i.e., intubation) are often necessary in severe reactions. Otherwise, cold
compresses and analgesics provide reasonable symptomatic relief. The stinger is
removed by gentle scraping. Sensitized patients should always carry a kit containing
tweezers and self-injectable epinephrine. Desensitization has been useful in children
with history of severe reaction to stings.
Selected Readings
1. American Academy of Pediatrics. Bite wounds. In: Peter G ed. 1997 Red Book:
Report of the Committee on Infectious Disease. 24th ed. Elk Grove Village, IL: 1997.
2. Golladay ES. Animal, snake, and spider bites. In: Buntain WL ed. Management of
Pediatric Trauma. Philadelphia: W.B. Saunders 1995; 478-493.
35
CHAPTER 1
CHAPTER 36
Neonatal Trauma and Birth Injuries

Daniel A. Bambini
Incidence
Newborns are most likely to be injured during birth if labor is difficult, presen-
tation is breech, or a forceps delivery technique is used. Traumatic injury accounts
for 1-2% of neonatal deaths. Birth trauma is the sixth leading cause of neonatal
mortality and causes about 25 deaths per every 100,000 live births in the United
States. For every neonatal death that occurs as a result of trauma, another 20 neo-
nates will suffer a significant birth-related injury. This chapter describes several of
the birth-related injuries that may be encountered in neonates.
Head Injuries
Cephalohematoma
Cephalohematoma occurs in about 1% of all live-born infants. The risk for
developing cephalohematoma is greatest in large, male infants born to primigravid
mothers. Forceps delivery is perhaps the greatest risk. Midforceps delivery results in
cephalohematoma in about 30% of cases while low forceps and nonforceps delivery
have a much lower risk (3.5% and 1.7% respectively). Cephalohematoma is believed
to result from shearing forces that disrupt vessels in the plane between the calvarium
and periosteum. The edges of the hematoma are usually sharply demarcated and do
cross beyond suture lines. An underlying skull fracture (usually linear) is present in
5-25% of cases, therefore x-rays are indicated. Subgaleal hemorrhage mimics
cephalohematoma but often is larger extending beyond suture line boundaries into
surrounding soft tissues of the head and neck.
Most cephalohematomas resorb spontaneously within a few weeks to months.
Rarely an infant develops anemia (requiring transfusion) or hyperbilirubinemia as a
result of cephalohematoma. If the hematoma is extremely large or if ulceration of
the overlying scalp appears likely, needle aspiration and decompression of the
hematoma is performed using sterile technique. If this is unsuccessful, open drain-
age is indicated to prevent the disfiguring calcification and skull deformity that may
occur.
Skull Fractures
Calvarial fractures in newborns are almost always associated with forceps deliv-
eries. The most common site of fracture is the parietal bone. Most fractures are
asymptomatic although fractures may lead to seizures or intracranial hemorrhage.
Many neonates with skull fractures have an overlying cephalohematoma or the scalp
is ecchymotic. A depression may be palpable on exam.
Neonatal skull fractures are typically classified as linear or depressed. Linear frac-
tures generally heal within 2 months of injury. Skull x-rays should be obtained at
3-4 months of age to exclude the presence of a leptomeningeal cyst. Leptomeningeal
cysts occur after injury to the dura and frequently lead to seizure activity. Neurosur-
gical intervention is required to treat these lesions.
Depressed skull fractures in neonates are often called “ping-pong” fractures because
there is “buckling” of the bone inward without an actual break in calvarial continu-
ity. Computed tomography is indicated to identify penetrating bone fragments or
an underlying intracranial hemorrhage. Small fractures are safely observed. Many
depressed fractures are elevated using thumb compression. Vacuum extraction may
also be successful. The indications for surgical intervention are:
1. penetrating bony fragments,
2. neurologic deficits,
3. signs of increased intracranial pressure,
4. cerebrospinal fluid collection beneath the galea, and
5. failed attempt at closed manipulation.
Intracranial Hemorrhage (ICH)

Infants born after prolonged or difficult labor are at risk for intracranial hemor-
rhage. Other known risk factors include cephalopelvic disproportion, breech deliv-
ery, and mid or high forceps delivery. Frequently the onset of symptoms and signs of
ICH (irritability, high-pitched cry, seizures, increased head circumference, bulging
fontanelle, etc.) is insidious and may not be appear until 24-48 hours after birth.
The three common types of ICH are subarachnoid hemorrhage, subdural hemor-
rhage, and epidural hemorrhage. Head CT and cranial ultrasound are the diagnostic
tests used to differentiate the three.
Subarachnoid bleeding in neonates is not uncommon. Many neonates will have
erythrocytes in their CSF after birth but the majority will be asymptomatic. In
premature neonates, subarachnoid hemorrhage is usually caused by asphyxia. In
term babies, subarachnoid bleeding is more likely to be secondary to trauma. The
typical presenting feature of significant subarachnoid hemorrhage is an acute onset
of seizures usually around 24–36 hours of age. Treatment consists of seizure control,
correction of coagulopathies, and transfusion as indicated.
Neonatal subdural hemorrhage is very rare. Vaginal breech deliveries are at the
highest risk for producing subdural hemorrhage in newborns. Prolonged deliveries
with face/brow presentations may cause excessive cranial molding and subsequent
bleeding. The severity of symptoms depends on the amount and location of bleed-
ing. Bleeding is usually from rupture of the superficial cerebral veins. Cerebral con-
tusion is present in up to one half of these infants. Epidural hemorrhage is the rarest
36 type of ICH encountered in neonates. Only about 60-70% of these cases will have
an associated fracture of the temporal bone. When the bleeding source is arterial, it
causes the infant to rapidly deteriorate with increased intracranial pressure within
the first few hours of life. Surgical intervention must be initiated as soon as possible.
Neonatal Trauma and Birth Injuries 155
Spine and Cord Injuries

Spine injuries in neonates almost always occur at the cervical spine. Nearly 75%
of birth related c-spine injuries are associated with breech or footling presentation at
time of delivery. Breech injuries usually occur at the C6-7 or C7-T1 levels and result
from traction on the body while the head remains engaged within the maternal
pelvis. High cervical injuries (C1-2) can occur with difficult vertex deliveries due to
extreme rotational forces. In neonates, complete cord transection can occur even
when the dura and bony structure or the cervical spine remains intact with no radio-
logic evidence of fracture. If a fracture or dislocation is observed on radiologic stud-
ies, severe cord injury (i.e., transection) can be anticipated. Depending upon the
severity or completeness of the lesion, these newborns may be awake and alert at
birth but often have apnea, spinal shock, severe flaccidity, and respiratory failure.
Abdominal wall paralysis and areflexia are frequent early findings. Later, the origi-
nally flaccid extremities become spastic and hyper-reflexic.
The prognosis is difficult to determine initially and gradual return of neurologic
function, sometimes complete, can occur over weeks to months. Therapy is nonspe-
cific and conservative. All efforts should be made to maintain neck immobilization
to prevent further injury.
Facial Fractures
The most common facial fracture in neonates is subluxation of the nasal septum.
This lesion only occurs in 2-3% infants but can cause significant respiratory distress
in these obligate nasal breathers. The respiratory distress is frequently aggravated or
worsened by oral feedings. The treatment is early reduction and fixation. Mandible
fractures are also occasionally observed in infants and also require early identifica-
tion, reduction and immobilization. If undiagnosed, mandible fractures ultimately
result in malocclusion, cosmetic deformity, feeding problems, and speech difficulties.
Eye Injuries
Minor eye injuries (abrasions, conjunctival hemorrhage, etc) are common and
occur in 20-25% of normal deliveries. Serious eye injuries occur in less than 0.25%
of live births and are almost always a result of forceps delivery. Subconjunctival and
retinal hemorrhages are the most common eye injuries. Most resorb within the first
2–3 days of life and are benign. Hyphema, blood in the anterior chamber of the eye,
is more serious and requires careful observation and follow-up. If rebleeding occurs
or the blood persists beyond 7 days of life, medical treatment with acetazolamide
and/or surgical evacuation of the anterior chamber may be necessary to prevent later
glaucoma. Corneal cloudiness or haziness is normal in the newborn, but if it persists
beyond one week a rupture of the posterior corneal membrane (Descement’s mem-
brane) should be suspected. If left untreated, this can result in permanent leukoma,
astigmatism, strabismus, and other problems with vision.
36
Nerve Injuries
The most often observed neonatal nerve injuries include injuries to the facial
nerve, the recurrent laryngeal nerve, brachial plexus, and the phrenic nerve. Facial
nerve palsy is the most common birth-related nerve injury and is usually associated
with forceps delivery. Nearly 75% of facial nerve injuries occur on the left side.
Facial nerve injury usually occurs secondary to pressure applied to the nerve at the
site where it exits the stylomastoid foramen or where it crosses over the ramus of the
mandible. Signs of injury may be subtle and include flattening of the ipsilateral
nasolabial fold, a persistently opened eye, and an inability of the neonate to move
the corner of the mouth. Spontaneous recovery usually occurs within 1-3 weeks but
may take longer. The cornea should be protected with an eye patch and eye drops
(1% methyl cellulose) instilled every 3-4 hours.
Vocal cord paralysis is the principal clinical manifestation of recurrent laryngeal
nerve injury which is uncommon in neonates. The left nerve is more commonly
injured than the right in part due to its longer length and course through the neck.
Bilateral vocal cord paralysis in neonates is usually secondary to CNS damage (i.e.,
hypoxia, hemorrhage). The signs of unilateral vocal cord paralysis include inspira-
tory stridor and hoarseness with crying. There are typically no symptoms or signs at
rest. The diagnosis is made by direct laryngoscopy. Small frequent feedings are rec-
ommended to reduce the risk of aspiration. Most of these injuries are reversible and
resolve spontaneously within 4-6 weeks.
Brachial plexus injuries and phrenic nerve injuries in neonates are usually due to
stretching (traction) injury of nerve roots. Erb’s palsy is the most common newborn
brachial plexus injury, affects C5-6 nerve roots, and accounts for 60-90% of cases.
The signs of Erb’s palsy are:
1. arm internally rotated at the shoulder,
2. arm extension at the elbow,
3. forearm pronated,
4. flexion at the wrist, and
5. absent biceps and brachioradialis reflexes.
Klumpke’s paralysis is a rare neonatal brachial plexus injury affecting the C8-T1
nerve roots that is frequently associated with a Horner syndrome (ptosis, miosis,
anhidrosis, enophthalmos). The prognosis for recovery of neurologic function after
brachial plexus injury is quite variable although full recovery is usual in 80-90% of
cases. Complete recovery, if forthcoming, usually occurs within 3-6 months. Neo-
natal phrenic nerve paralysis results from traction injury to the C3-C5 nerve roots
and almost always occurs in association with a brachial plexus injury. Eighty percent
are right side lesions; less than 1% are bilateral. Phrenic nerve injury may cause
diaphragmatic paralysis, respiratory distress, and an elevated hemidiaphragm and/or
eventration (Chapter 72). Most of these phrenic nerve injuries resolve spontane-
ously within 6 weeks. Diaphragmatic plication may become necessary if improve-
ment does not occur by 2 months or life threatening pulmonary complications
(pneumonias, respiratory failure, aspiration) ensue.
36 Pneumothorax
Pneumothorax in neonates usually results from the barotrauma of aggressive
positive pressure ventilation or overinflation of the lungs. If respiratory distress is
significant, tube thoracostomy is performed. Small pneumothoraces in
uncompromised, nonmechanically ventilated infants can be observed and often
Neonatal Trauma and Birth Injuries 157
resolve spontaneously in 24-48 hours. In mechanically ventilated neonates, tube

thoracostomy is indicated for almost all pneumothoraces.
Abdominal Trauma
Abdominal trauma in neonates is very rare. Liver injuries are the most common
yet occur in far less than 1% of newborns. Abdominal trauma usually occurs in
babies with history of a difficult delivery. The most commonly injured organs are
the liver, adrenal gland, spleen and kidney. The risk of injury is increased in the
presence of pre-existing organomegaly. The clinical presentation is that expected
with intra-abdominal bleeding and includes signs of pallor, irritability, abdominal
distention, anemia, and hemodynamic instability. Neonatal liver injuries are extremely
difficult to control surgically, and many of these injuries are lethal.
Skeletal Fractures
The most commonly encountered birth-related fractures are of the clavicle,
humerus, and femur. Most of these injuries are either midshaft fractures or epiphy-
seal separations. Less than 1% of all newborn infants sustain a fracture during deliv-
ery. Most fractures are easily confirmed by x-ray examination. Clavicle fractures
account for 90-93% of neonatal fractures. All infants with clavicle fracture should
be evaluated for possible coexisting injuries of the brachial plexus, the cervical spine,
and the humerus. Clavicle fractures in neonates usually heal rapidly with complete
union at 7-10 days. Specific therapy is usually not necessary.
Seventy-five percent of extremity fractures in newborn infants occur in associa-
tion with breech delivery. Infants with long-bone fractures usually have a tender,
swollen limb that often hangs limply with no voluntary movement. Crepitus is vari-
ably present. Epiphyseal injuries of the humerus and femur are usually Salter-Harris
type I lesions.
Iatrogenic Perforation of the Pharynx or Esophagus
Pharyngoesophageal perforations occur after difficult endotracheal or esophageal
intubations. Traumatic perforations also occur with vigorous passage of orotracheal
suction tubes or feeding tubes. The clinical findings associated with pharyngeal per-
foration with a nasogastric tube closely mimic those of esophageal atresia. In patients
with esophageal atresia, the nasogastric tube passes to about 11 cm before reaching
the distal end of the blind esophageal pouch. If the tube passes to a shorter or longer
distance with some difficulty, the diagnosis of esophageal atresia is considered. Blood
at the end of the passed nasogastric tube is common with perforation but not expected
with esophageal atresia. A chest radiograph may reveal a pneumothorax, pneumo-
mediastinum, feeding tube within the chest, or a unilateral infiltrate with an abnor-
mal extrapleural air collection. Nonoperative therapy with close observation,
broad-spectrum antibiotics, TPN, and selected tube thoracostomy, is usually effec-
tive. Clinical deterioration with signs of infection or mediastinitis mandates prompt 36
surgical exploration and drainage.
Selected Readings
1. Perlow JH, Wigton T, Hart J et al. Birth trauma. A five-year review of incidence
and associated perinatal factors. J Reprod Med 1996; 41:754-760.
2. Krasna IH, Rosenfeld D, Benjamin BG et al. Esophageal perforation in the neo-

nate: an emerging problem in the newborn nursery. J Pediatr Surg 1987;
22:784-790.
3. Marchildon MB, Doolin EJ. Birth Injuries. In: Buntain WL ed. Management of
Pediatric Trauma. Philadelphia: WB Saunders Company 1995; 494-513.
4. Raffensperger JG. Trauma in the neonate. In: Raffensperger JG ed. Swenson’s
Pediatric Surgery, 5th Edition. Norwalk: Appleton & Lange 1991; 339-341.
5. Medlock MD, Hanigan WC. Neurologic birth trauma: intracranial, spinal cord,
and brachial plexus injury. Clin Perinat 1997; 24:845-57.
36
CHAPTER 1
CHAPTER 37
Child Abuse
Definitions
The maltreatment of children can be subdivided into 2 major categories:
1. abuse and
2. neglect.
The abuse of a child may be either physical or sexual. Physical abuse is defined as
“harm or threatened harm to a child through nonaccidental injury as a result of the
acts or the omissions of acts of those persons responsible for the child’s care.” Sexual
abuse is defined as “the commission of any sexual offense with or to a child as a
result of the acts or omissions by the child’s caretaker.”
Neglect is a more subtle form of maltreatment and is defined as “the harm that
occurs through the failure of the caretaker to provide adequate food, shelter, medical
treatment, or other provisions necessary for the child’s health and welfare.” Neglect
is further subdivided into emotional, physical, or educational neglect.
Incidence
In 1992 there were almost 3 million cases of possible child abuse or neglect
reported in the United States. Substantiation rates varied from 13-72% (average
40%) documenting that over 1 million children were confirmed victims of abuse or
neglect during that one-year period, giving an incidence of approximately 2 per
1000 children. The actual prevalence is unknown. In 1992, over 1200 children died
as a result of neglect or abuse.
Although both sexes suffer abuse, girls are slightly more affected due to the higher
incidence of sexual abuse in female children. Contrary to many published reports
children under 2 years of age are the least abused group of children. However, more
serious injuries and fatalities occur among this younger age group. Although there
are no significant racial or ethnic differences in the incidence of abuse or neglect,
socioeconomic factors are important. When families are stratified into those with
household incomes greater or lesser than 15,000 dollars annually, families in the
lower income bracket demonstrated 4-8 times the amount of abuse or neglect inflicted
by families in the higher income bracket.
When a child is seen with injuries, abuse is suspected if any of the following are
present:
1. there is a delay in presentation or the history given is inconsistent with
the injuries sustained,
2. parents attempt to explain injuries with mechanisms that are unlikely

given the child’s developmental status,
3. the primary caregiver is not present,
4. there is a history of unexplained injuries,
5. parents understate the seriousness of the child’s injuries and are reluctant
to allow hospital admission for further diagnostic testing,
6. parents have visited several area hospitals and arrive at the emergency
department in the early morning hours, hoping to draw less attention to
the child’s injuries, or
7. parents questioned about the specifics of the injury, become hostile and
defensive.
In addition, concern arises when the child’s physical examination reveals a vari-
ety of injuries often in various stages of healing. Table 37.1 lists some of the com-
mon physical and clinical findings associated with child abuse.
Diagnosis
The diagnosis of child abuse or neglect can be difficult to make with certainty.
The clinician’s role is not one of judge or jury, but rather child advocate. Thus, every
effort must be made to remain impartial and collect the facts as accurately and
completely as possible. Careful documentation of the exact words parents and care-
takers use to describe the events surrounding the suspected abuse is essential. The
decision to evaluate a child for abuse is based not only on the history and physical
examination, but also on the radiologic findings and the constellation of injuries
found. The degree to which these injuries correspond to recognized patterns of injury
in abused children influences the medical conclusions drawn.
The most common radiologic findings are those of occult fractures and soft
tissue injuries. Fractures occur in nearly one third of physically abused children and
over half of all fractures in children less than one year of age are the result of abuse.
Occult fractures are identified in various states of healing. Periosteal or new bone
growth appears 7-10 days following injury. Soft and hard callus formation occurs at
2 and 3 weeks, respectively. At 6-8 weeks following injury, the original fracture line
is completely obscured by callus formation.
Spiral fractures are commonly seen in the tibia, femur, radius, and humerus of
abused children and result from a strong twisting of the extremity. Abuse is sus-
pected when the history does not suggest a large amount of rotational force. Rib
fractures, especially in children less than 2 years of age, alerts the clinician to the
possibility of intentional injury. Rib fractures resulting from intentional injury are
usually posterior and occur secondary to the violent compression of the chest.
Patterns of Injury
Head Injury
Head injuries are the leading cause of fatal child abuse, and 80-90% of these
37 deaths affect children under 2 years of age. Complex skull fractures, bilateral frac-
tures, or fractures that cross suture lines are suggestive of intentional injury.
Shaken impact syndrome (SIS), first described by Caffey in 1974, is character-
ized by subdural or subarachnoid hemorrhages, retinal hemorrhages, and minimal
Child Abuse 161
Table 37.1. Clinical and physical signs of child abuse
Head:
Retinal hemorrhages in the absence of thoracic trauma
Unexplained dental trauma or torn frenulum of the upper or lower lip.
Bilateral black eyes with a history of a single fall or injury
Traumatic hair loss or swollen ears
Severe central nervous system injury with a history inconsistent with the extent
of injury.
Skin and Soft Tissue Injuries:
Bruises located in areas normally protected, such as the thighs, upper arms,
back, and abdomen. Bruising normally occurs over bony prominences
(elbows, knees, etc.)
Bruising of various colors denoting injuries in various states of healing.
Bruises that resemble the outline of specific objects such as belts, hands,
whipping cords, etc.
Burns that follow a stocking or glove pattern, located on the perineum, or that
are well demarcated such as from being dipped into a too-hot tub, or from a
hot iron, cigarette, curling iron, etc.
Human bites of any type with an inconsistent history
Abdominal Injuries:
Evidence of abdominal wall hematoma.
Bilious vomiting or reports of emesis or diarrhea witnessed only by the parents.
Injuries to the perineum or genitals. Physical exam consistent with a sexually
transmitted disease.
Chronic weight loss or child small for gestational age.
Skeletal Injuries:
Rib fractures in a child under 2 years of age, particularly posterior rib fractures
that often result from direct blows. Lateral rib fractures can occur secondary
to anterior posterior crushing.
Femur fractures in children less than 3 years of age, particularly if the child is
preambulatory.
Fractures located at the metaphyseal-epiphyseal junctions in the long bones.
Spiral fractures of the femur
Fractures in various states of healing
Fractures of the scapula, sternum, spinous process, or lateral clavicle.
Complex skull fracture after a fall of less than 3 feet in height.
or absent signs of external trauma. Originally thought to occur due to violent shak-
ing of an infant without cranial impact, recent evidence suggests that the rotational
forces caused by shaking alone are not severe enough to cause the severity of injury
frequently observed in these children. A combination of violent shaking and sudden
deceleration caused by cranial impact is necessary to generate the angular accelera-
tion required to cause significant intracranial injury. The SIS is rarely observed in
children over 3 years of age. Clinical findings include lethargy, irritability, vomiting,
apnea, feeding intolerance, and seizures (40-70%). Retinal hemorrhages are present
in 70-95% and are often bilateral or associated with retinal detachment. Retinal
hemorrhage is very rarely seen in other clinical situations (i.e., recent vehicular trauma,
37
birth trauma), is not associated with recent cardiopulmonary resuscitation, and is
not attributable to birth trauma if the child is beyond 2 months of age.
Intra-Abdominal Injuries
Abdominal injuries are the second most common cause of death in the abused
child. Bruising of the anterior abdominal wall suggests potential visceral injury. The
organs overlying the spine are particularly vulnerable, especially the duodenum and
pancreas.
Duodenal hematomas occur as a result of compression against the vertebral col-
umn and present as either a partial or complete bowel obstruction with vomiting
and mid-epigastric pain. Upper gastrointestinal contrast studies may reveal a “coiled
spring” appearance. The pancreas is also easily compressed against the spine and
injured. Blunt trauma to the pancreas causes pancreatitis, pancreatic transection, or
pancreatic pseudocyst. Over 50% of pancreatic pseudocysts identified in children
are posttraumatic.
Injuries to the solid organs (i.e., liver, spleen, kidney) are less occasionally seen
and include rupture, contusion, and subcapsular hemorrhage. Tears to the bowel
mesentery and gastrointestinal perforations are also rarely encountered.
Burns
Burn injuries account for approximately 10% of intentional injury cases. Inflicted
burns commonly show distinctive patterns of distribution. Burns to the perineum
and buttocks often occur as a form of punishment for a child having difficulty toilet
training. Burn wounds that have a “stocking” or “glove” distribution most often
result from immersion in hot water and are nearly pathognomonic for intentional
injury. Deep, repeated, and well-circumscribed burn wounds (i.e., branding inju-
ries) are common and are frequently inflicted with cigarettes, curling irons, and
cooking utensils.
Treatment
Management of children with intentional injuries is the provision of both physi-
cal and emotional treatment. Medical specialists, social service agents, physical thera-
pists, and psychological support personnel are helpful to manage these children.
Child abuse is a family problem and requires a multidisciplinary team to be effec-
tive. It is important to answer any questions the child may have concerning what is
happening to them and to provide reassurance. Intentionally injured children must
be reassured that what has happened is not their fault.
Despite the requirement that all physicians report any suspected cases of child
abuse or neglect, formal reporting occurs in only half of these cases. Failure to report
is due to concerns over harming reputations, producing undue stress on the family,
or discouraging offenders from voluntarily seeking treatment. Good faith reporting
laws protect physicians from litigation in unproven cases.
Selected Readings
1. Gonzalez-Ibrahim E. Diagnostic points in child abuse. Resident and Staff Physi-
cian 1996; 42(12):12-16.
2. Wissow LS. Child abuse and neglect. NEJM 1995; 332(21):1425-1431.
37 3. Duhaime AC, Christian CW, Rorke LB et al. Nonaccidental head injury in infants—
the “shaken baby syndrome.” NEJM 1998; 338(25):1822-1829.
4. Berkowitz CD. Pediatric Abuse—New Patterns of injury. Emerg Med Clin N Am
1995; 13(2):321-341.
Section V: Pediatric Tumors
CHAPTER 38
Renal Tumors
P. Stephen Almond
Incidence
The five most common pediatric renal tumors are Wilms’ tumor, clear cell
sarcoma, rhabdoid tumor, congenital mesoblastic nephroma, and nephro-
blastomatosis. Wilms’ tumor is by far the most common with an incidence of 8 per
million or about 350 cases per year. Children with Wilms’ tumor, clear cell sarcoma,
or rhabdoid tumor are entered in the National Wilms’ Tumor Study Group (NWTS).
Wilms’ tumors make up 90-95% of NWTS followed by clear cell sarcomas (6%),
and rhabdoid tumors (2%). Congenital mesoblastic nephroma is the most common
renal tumor of infancy, but less than 100 cases of congenital mesoblastic nephroma
have been reported. Nephroblastomatosis or nephrogenic rests are found in 1% of
neonatal autopsies, 40% of Wilms’ tumors, 8% of kidneys with obstructive uropa-
thy, and 7% of multicystic dysplasic kidneys.
Etiology
The majority of Wilms’ tumors are sporadic. However, deletions in chromo-
somes 11p13 (10% of cases), 11p15, and 16q have been reported in Wilms’ tumor
patients and are associated with increased risk of developing the tumor. The 11p13
gene has been named Wilms’ tumor gene 1 (WT1); the 11p15 gene is called Wilms’
tumor gene 2 (WT2); and the 16q gene has been named the Wilms’ tumor gene 3
(WT3). The gene product of WT 1 is a DNA-binding protein found on fetal kid-
ney and genitourinary tissues. The gene products of WT 2 and WT 3 are unknown.
The etiology of clear cell sarcoma, rhabdoid tumors, and congenital mesoblastic
nephroma are unclear.
The majority of children with Wilms’ tumor present between the ages of 2 and 4
years with an asymptomatic abdominal mass. Other symptoms include hematuria
(10%), hypertension (20%), anorexia, fever, and weight loss. Associated conditions
can be divided into syndromic and nonsyndromic. There are four syndromic asso-
ciations. Denys-Drash syndrome is male pseudohermaphroditism and degenerative
renal disease leading to end-stage renal disease within the first year of life. The risk of
Wilms’ tumor is 90% and the associated chromosomal deletion is 11p13 or WT 1.
Klippel-Treneunay syndrome includes superficial vascular anomalies, deep vascular
anomalies, and limb overgrowth. Beckwith-Wiedemann syndrome includes mac-
rosomia, macroglossia, omphalocele, ear fissures, facial hemangioma, and mental
Renal Tumors 165
retardation (5%). The risk of Wilms’ tumor is 5% and the associated chromosomal
deletion is 11p15 or WT 2. WAGR syndrome is the association of Wilms’ tumor,
aniridia, genitourinary anomalies, and mental retardation. The risk of Wilms’ tumor
is 30% and the genetic defect is a chromosomal deletion of WT 1.
There are three nonsyndromic lesions associated with Wilms’ tumor:
1. aniridia,
2. hemihypertrophy, and
3. genitourinary anomalies.
The incidence of aniridia in Wilms’ tumor patients is < 1%. It is due to a dele-
tion of the short arm of chromosome 11. Children with aniridia should undergo
physical examination, abdominal ultrasound and urinalysis every 3 months until
the age of 5-8 years. The incidence of hemihypertrophy in Wilms’ tumor patients is
3%. The most common genitourinary anomalies in Wilms’ tumor patients are cryp-
torchidism (1%) and hypospadias (5.2%).
Children with clear cell sarcoma present between 1 and 2 years of age. Bone
metastasis are present in 40% of these children. Children with rhabdoid tumor also
present younger (average age 17 months) than those with Wilms’ and have a high
incidence of associated brain tumors (13%). Children with mesoblastic nephroma
present within the first four months of life. Otherwise, the clinical presentation of
these three lesions is identical to that of Wilms’ tumor.
Diagnosis
The diagnostic workup includes a chest x-ray and an abdominal ultrasound. The
chest x-ray is done to evaluate for the presence of pulmonary metastasis. The
abdominal ultrasound shows:
1. the tumors’ organ of origin,
2. the consistency of the tumor,
3. tumor in the contralateral kidney, and
4. the extension of tumor into the renal vein and/or inferior vena cava (IVC).
Additional studies include urinalysis, abdominal plain film, and computed
tomography (CT) (Fig. 38.1). The urine may contain red blood cells (20% of cases)
or hyaluronic acid. The abdominal x-ray may show “eggshell” calcifications. This is
in contrast to the “speckled” calcifications seen in neuroblastoma and the “popcorn”
calcifications seen in teratomas. CT scan of the chest and abdomen are frequently
used to determine:
1. the presence of pulmonary metastasis,
2. the tumors’ organ of origin,
3. the condition of the contralateral kidney, and
4. the presence of tumor in the renal vein/IVC.
Pathology
The histology of Wilms’ tumor (Fig. 38.2) is either favorable or unfavorable.
Favorable histology is more common (89%) and is characterized by the presence of
three components:
1. stroma,
2. blastema, and 38
3. epithelial elements.
Fig. 38.1. Computed tomography revealing left renal tumor replacing most of the
medial portion of the organ (Wilms’ tumor).
Fig. 38.2. Pathological specimen shows a solid, homogeneous tumor replacing

most of the superior pole of a kidney (Wilms’ tumor).
38
Renal Tumors 167
Unfavorable histology is less common (11%) and defined by the presence of

anaplasia, clear cell sarcoma, or rhabdoid tumor.
Mesoblastic nephroma is a benign, white to yellow-tan lesion that gradually gives
way to normal appearing kidney. Microscopically, spindle cells are seen instead of
the normal kidney parenchyma. Although generally considered a “benign” or hama-
rtomatous lesion, mesoblastic involvement of the renal vein and a pulmonary
metastasis have been reported.
Nephroblastomatosis or nephrogenic rests are microscopic areas of blastema
adjacent to normal kidney parenchyma. The natural history of these rests is poorly
understood. At present it is thought that these lesions may regress or develop into
Wilms tumor.
Staging
NTWS has five stages. Stage I lesions are confined to the kidney and completely
excised. Stage II lesions extend beyond the kidney but are completely excised. Local
spillage confined to the flank and preoperative biopsy are acceptable. Stage III lesions
are confined to the abdomen but cannot be completely resected. Peritoneal spillage
of tumor automatically makes the tumor Stage III. Stage IV lesions have hematog-
enous metastases to lung, liver, bone, or brain. Stage V indicates bilateral disease.
Treatment
These children undergo laparotomy and abdominal exploration unless Stage IV
or V disease is confirmed. The presence of bloody ascites suggests tumor rupture
and stage III disease. Before removing the tumor, the contralateral kidney is manu-
ally and visually inspected. Any and all suspicious lesions are biopsied. If the con-
tralateral kidney is normal, an ipsilateral radical nephroureterectomy is performed
being careful not to rupture the tumor. Paraaortic lymph nodes are sampled and
titanium clips are placed along the margins of resection. If there is bilateral disease
and wedge/partial resection of all tumors will result in removal of greater than 1/3 of
the total renal mass, biopsy is done and the patient is closed. Afterward, the child is
treated with chemotherapy and re-explored in 6 weeks.
All children with Wilms’ tumor receive postoperative chemotherapy. Those with
stage I and II lesions and favorable histology are treated with actinomycin-D and
vincristine. Children with stage III and IV lesions and favorable histology are treated
with actinomycin-D, vincristine, and adriamycin (doxorubicin) and postoperative
radiation therapy. Children with stage I lesions and unfavorable histology are treated
with actinomycin-D and vincristine. Children with stage II, III, and IV lesions and
unfavorable histology are treated with three (actinomycin, vincrisitne, adriamicin)
or four (actinomycin, vincrisitne, adriamycin, cyclophosphamide) drugs and post-
operative radiation therapy.
Indications for postoperative radiation therapy include gross residual tumor, tumor
spill, positive lymph nodes, peritoneal involvement, stage IV disease, and unfavor-
able histology. Indications for preoperative chemotherapy include stage V disease,
IVC involvement above the hepatic veins, massive tumor, and cytoreductive therapy
if other organs are at risk.
Children with congenital mesoblastic nephroma do not require adjuvant therapy.
38
Outcome
The overall survival for children with favorable histology is 90%. Children with
favorable histology, stage I lesions have a 97% long-term survival. This drops to
60% for children with favorable histology, stage IV lesions. Overall survival for
children with unfavorable histology is 50%. Children with unfavorable histology,
stage I lesions have an 89% long-term survival. This drops to 14-55% for children
with unfavorable histology and any other stage disease.
Congenital mesoblastic nephroma is a benign lesion with an excellent prognosis.
Selected Readings
1. Renal tumors. In: Rowe MI, O’Neill JA, Grosfeld JL et al, eds. Essentials of Pedi-
atric Surgery. St. Louis: Mosby-Year Book, Inc. 1995.
2. Shochat SJ. Wilms’ Tumor: Diagnosis and Treatment in the 1990s. Sem Pediatr
Surg 1993; 2:59-68.
38
CHAPTER 1
CHAPTER 39
Neuroblastoma
Marybeth Madonna
Background and Etiology
Neuroblastoma is a tumor derived from neuroblasts. Neuroblasts are derived
from neural crest cells and migrate during fetal development to form the autonomic
nervous system. There are two paths of migration:
1. along developing nerves to form the sympathetic plexuses, where they
form ganglion cells and
2. to the adrenal gland to form the medulla.
Tumors of the neuroblasts can be either malignant or benign. The tumors are
named ganglioneuromas, ganglioneuroblastoma and neuroblastoma depending on
the degree of malignant potential with ganglioneuromas being completely benign
and neuroblastoma being malignant with the ganglioneuroblastoma as an interme-
diate tumor.
Neuroblastoma is the most common extracranial solid tumor in children
accounting for 8-10% of all childhood cancers. This tumor occurs in approximately
1 in 7000-18,000 live births. There are about 550 new cases each year in the United
States. There is no ethnic prevalence. There is a slight male predominance with a
ratio of 1.2:1 (male to female). The median age at diagnosis is 22 months, and 97%
of neuroblastomas are diagnosed in children less than ten years of age. The inci-
dence is biphasic with a peak at less than one year of age and a second peak at 2-4
years. Prenatal or postnatal exposure to drugs, chemicals or radiation has not been
unequivocally demonstrated to increase the incidence of neuroblastoma.
Although most cases of neuroblastoma are thought to be sporadic, there is a
subset of patients that exhibit a predisposition to develop disease in an autosomal
dominant pattern. About 22% of neuroblastomas are thought to be the result of a
germinal mutation. The hereditary form of the disease has an earlier mean age at
diagnosis (9 months versus 22 months) and has a higher incidence of bilaterality
and multifocal tumors (20%). Neuroblastoma is thought to follow a two-mutation
hypothesis of tumorogenesis.
Pathology
Neuroblastomas arise from the primitive pluripotential sympathetic cells that
are derived from neural crest cell and normally differentiate to form tissues of the
sympathetic nervous system. All fetuses have neuroblastic nodules between 17-20
weeks gestational age. Most regress before birth or shortly thereafter. Neuroblas-
toma in situ is frequently found in infants three months or younger dying from
other causes. Therefore the cells that form neuroblastoma may be fetal remnants
that fail to regress.
39 Neuroblastomas belong to a group of tumors classified as the “small round blue
cell” tumors. Others in this category include Ewing’s sarcoma, non-Hodgkin’s lym-
phoma, primitive neuroectodermal tumors (PNETs) and undifferentiated soft tis-
sue sarcomas such as rhabdomyosarcoma. Neuroblastomas can be differentiated from
other tumors in this category by using immunohistochemistry. These tumors are
positive for the marker neuron specific enolase. Neuroblastoma cells have neuritic
processes called neuropil. Homer-Wright pseudorosettes are formed by neuroblasts
surrounding areas of eosinophilic neuropil. Ganglioneuromas, the benign variety of
this tumor, are composed of mature ganglion cells, neuropil and Schwann cells.
Ganglioneuroblastomas are a heterogeneous group of tumors with varying degrees
of mature ganglion cells. These cells may be focal or diffuse with the diffuse variety
associated with less aggressive behavior. These tumors show dense neurosecretory
granules as well as microfilaments on electron microscopy (EM).
There have been attempts to determine prognosis based on histologic criteria.
The Shimada classification compares patient age and
1. the presence or absence of Schwann cell stroma,
2. degree of differentiation and
3. mitosis-karyorrhexis index (MKI) to differentiate tumors into favorable
or unfavorable prognosis.
The Joshi classification considers the presence of calcifications and mitotic rate.
A low mitotic rate (< 10 per high power field) and the presence of calcifications
predicts a favorable outcome (grade 1). Grade 3 tumors have neither feature and are
associated with a poor prognosis.
Several cellular and molecular characteristics have prognostic significance in
patients with neuroblastoma. Tumor cells produce varying amounts of DNA. The
measurement of this is called the DNA index. For normal cells, the DNA content is
diploid and the DNA index is 1. Some neuroblastomas have a high DNA content
and are called hyperdiploid (DNA index >1). In younger children, tumors that are
hyperdiploid are more likely to have a lower stage of tumor and be responsive to
cyclophosphamide and doxirubicin. Hyperdiploid tumors in older children do not
have the same favorable outcome. The most consistent specific genetic abnormality
identified in children with neuroblastoma is a deletion of the short arm of chromo-
some 1(1p). This most likely represents a deletion of a tumor suppressor gene.
Neuroblastomas may also show n-myc amplification. This region of amplifica-
tion is located on the distal short arm of chromosome 2 and contains the N-myc
protoconcogene. Amplification of n-myc occurs in 25% of patients with neuroblas-
toma and is associated with advanced stage of disease, rapid tumor progression, and
poor prognosis. Molecular studies have demonstrated a correlation between the 1p
deletion and n-myc amplification. An important pathway in normal differentiation
of neuroblasts involves a differentiation factor called nerve growth factor (NGF)
and its receptor (trk A). Most neuroblastoma cell lines are not responsive to NGF.
Tumors that have high trk A are associated with a good biological response to therapy
and a favorable prognosis. Trk A expression is inversely correlated with n-myc am-
plification.
Neuroblastoma 171
Tumors arise anywhere there are sympathetic nerves from the brain to the pelvis.
Most primary tumors occur in the abdomen (65%) (Fig. 39.1). The frequency of 39
adrenal tumors is slightly higher (40%) in children compared to infants (25%).
Infants have more thoracic and cervical primary tumors. The clinical presentation
depends on the location of the primary. Often the symptoms are few and general.
The patients often appear ill and fail to thrive. Those with cervical tumors (Fig. 39.2)
present with a mass in the neck or with Horner’s syndrome (meiosis, anhydrosis and
ptosis). Those with thoracic primaries are diagnosed after a mass is found on a rou-
tine chest radiograph. The parents often find abdominal tumors when they are bathing
the child. Abdominal tumors are more irregular than Wilms’ tumors and more often
cross the midline. Pelvic tumors may result in obstructive symptoms (urethral or
colonic). Rarely they compress or infiltrate the iliac veins and/or arteries and present
with lower extremity edema. Tumors that extend intraspinal in any of these loca-
tions may present with neurologic symptoms.
Two specific syndromes sometimes occur in patients with neuroblastoma.
Opsoclonus-myoclonus is a constellation of symptoms including polymyoclonia,
cerebellar ataxia with gait disturbance, and opsoclonus (“dancing eyes”). More that
50% of children with this syndrome have primary tumors located in the thorax.
Although this syndrome is often associated with a favorable outcome, the symptoms
may or may not resolve after tumor removal. Rarely, patients with neuroblastoma
present with profuse watery diarrhea if the tumor secretes vasoactive intestinal pep-
tide (VIP). The diarrhea resolves once the tumor is removed.
Ninety to ninety-five percent of tumors are biologically active secreting
vanillymandelic acid (VMA) or homovanellic acid (HVA) or other catecholamine
metabolites. HVA represents degradation products of the dopamine pathway. More
differentiated tumors produce norepinephrine and epinephrine that give rise to VMA.
The VMA: HVA ratio has some prognostic implication with levels > 1 indicating
tumors with a more favorable prognosis. Ten percent of neuroblastomas secrete ace-
tylcholine and not catecholamines; these tumors tend to be more malignant.
There are two main patterns of metastatic spread in patients with neuroblas-
toma. The first is lymphatic spread. Thirty-five percent of children with apparently
localized disease have lymph node metastasis at the time of presentation. Spread of
tumor to lymph nodes outside the cavity of the primary tumor is considered dis-
seminated disease but these patients may have a better outlook than those with
other forms of disseminated disease. The other form of metastatic spread is
hematogenous. The most common sites of metastasis are bone marrow, bone, liver
and skin. Only rarely does neuroblastoma metastasize to lung or brain and these are
usually manifestations of end-stage disease.
Staging
Patients with neuroblastoma are staged based on the extent of primary disease
and the presence or absence of metastases. Completeness of surgical resection is also
factored into the staging system. There have been two main staging systems in the
past but recently a new international staging system has been devised which com-
bines the previous systems (Table 39.1).
39
Fig. 39.1. Left adrenal neuroblastoma of large size and with marked calcification
demonstrates medial aortic displacement and inferior displacement of the left kidney.
Diagnosis
The diagnosis of neuroblastoma is based on the clinical signs and symptoms
discussed previously. Once there is suspicion that a child has a neuroblastoma con-
firmatory testing is done. The child’s urine is sent for HVA and VMA. The primary
tumor is usually assessed with computed tomography (CT) scan which helps deter-
mine the extent of disease, invasion into surrounding structures, and lymph node
involvement. In children with a primary abdominal tumor, involvement of the liver
with tumor can also be assessed. For patients who present with neurologic symp-
toms, magnetic resonance imaging (MRI) can be helpful to define involvement of
the spinal canal or cord. Metaiodobenzylguanidine (MIBG) is a compound resem-
bling norepinephrine that binds to norepinephrine sites and is stored in neural crest
cells. When this substance is labeled with 123I or 131I it can define the tumor or
identify metastases even in those few patients with normal catecholamine levels. To
assess for metastases, a bone scan and a plain chest radiograph are obtained. Com-
puted tomography of the chest is only warranted if there are suspicious findings on
a chest radiograph. Bone marrow aspirates are done from bilateral iliac crests as are
trephine (core) bone marrow biopsies.
The diagnosis of neuroblastoma is confirmed by:
1. Biopsy with unequivocal diagnosis of neuroblastoma by light microscopy
or
2. Bone marrow biopsy or aspirate with unequivocal tumor cells and increased
serum or urine catecholamines.
Because of the heavy dependency of treatment plans on tumor biology, there is a
strong rationale for sampling tumor in most cases.
Neuroblastoma 173
39
Fig. 39.2. Large white arrows indicate cervical neuroblastoma well shown by mag-
netic resonance imaging.
Treatment
Treatment for patients with neuroblastoma involves a combination of surgery,
radiation and chemotherapy. The goal of the initial surgical intervention in patients
with neuroblastoma is to establish a diagnosis, provide tissue for biological studies
(1-5 grams of tissue), stage the tumor surgically and excise completely those tumors
where this is feasible. Complete excision should only be undertaken when there is
not a concern for undue morbidity to vital organs or the patient. Sacrifice of major
organs such as the kidney or spleen should be avoided, especially in children less
than one year of age. If there are known distant metastases then the most accessible
tissue is obtained for diagnosis and biological studies.
For thoracic tumors, a posterior-lateral thoracotomy is generally used. Attach-
ments to the sympathetic chain and intercostal nerves are often found but en bloc
excision of the chest wall is not required. Dumbbell shaped tumors that enter the
neural foramina are generally treated initially with chemotherapy. These tumors
were historically treated with radiation and laminectomy but had a higher rate of
spinal column sequelae than those treated with chemotherapy.
For abdominal tumors, a generous transverse incision is usually employed. Liga-

tion of feeding vessels is attempted early but care must be taken as larger tumors can
39 rotate the aorta and distort the celiac, superior mesenteric and renal vessels. Lymph
node sampling is performed regardless of the gross appearance of the nodes as
inspection only has been shown to be in error 25% of the time. For sampling, non-
contiguous nodes above and below the tumor are sampled. For those tumors in the
abdomen and pelvis, contralateral lymph nodes are important. For infants less than
one year of age, liver biopsy may be indicated if stage 4S (Table 39.1) disease is
suspected.
For those patients who have incomplete resection initially, a delayed attempt at
resection of residual tumor is undertaken at the end of induction chemotherapy
(12-24 weeks after diagnosis). Surgery is not indicated for those patients who have
progressive disease at this time. If there has been some response, the goal is complete
resection of residual disease. The efficacy of eradication of the primary tumor at this
time is not proven, but survivors have had complete resection more often than
nonsurvivors. For patients with stage 4 and 4S, there is a 30% relapse rate if there is
complete excision of the primary tumor and a greater than 90% rate in those with-
out excision of the primary tumor.
Complications of surgical intervention include atelectasis, infection, ileus, and
complications specific to the resection of the primary tumor. Overall the complica-
tion rate is low, estimated at 5-25%. Patients with localized tumors have lower rates
of complication. Complications occur most frequently in infants after attempted
excision of large tumors.
Chemotherapy is usually multiagent therapy. The most frequently used drugs
include cyclophosphamide, cisplatin, doxirubicin and epipodophyllotoxins
(teniposide-VM-26 and etoposide-VP-16). In general, drugs are combined so that
the noncell cycle specific agents are given followed by the cell cycle specific agents.
Neuroblastoma is considered a radiosensitive tumor but the role of radiation
therapy in patients with this tumor is now minimal due to the newer chemotherapy
agents. Presently radiation therapy s used for regional lymph node metastasis if com-
plete response is not achieved with chemotherapy, for infants with 4S disease who
have respiratory distress from hepatomegaly secondary to tumor involvement, and
in those patients who require total body irradiation for bone marrow transplantation.
Bone marrow transplantation is currently considered an investigational therapy
for Stage 3 and 4 patients. Autologous bone marrow is given to the patients with or
without purging to remove the neuroblastoma cells. Long-term results survival rates
approach 40%. In these patients, recurrence most commonly occurs at the primary
site of the tumor or in the bone or bone marrow.
Outcomes
When considered alone, the two most important clinical variables for predicting
outcome in neuroblastoma patients are the disease stage and the patient age at diag-
nosis. Disease free survival of all patients with stages 1, 2, or 4S is 75-90%. Infants
less than one year of age with stage 3 and 4 have cure rates of 80-90% and 60-75%,
respectively. Those children older than one year of age with stage 3 and 4 disease
have 3-year survivals of 50% and 15%, respectively.
Neuroblastoma 175
Table 39.1. Staging of neuroblastoma

Children’s Cancer Study Pediatric Oncology International Neuroblastoma 39
Group (CCSG) System Group (POG) System Staging System
Stage I: confined to the Stage A: Complete gross Stage 1: Localized tumor

organ or structure of resection of primary confined to the area of origin;
origin tumor, with or without complete gross excision, with
microscopic residual or without microscopic
disease; intracavitary residual disease; identifiable
lymph nodes not ipsilateral and contralateral
adherent to primary lymph nodes negative
tumor; nodes microscopically
adherent to the surface
of or within the primary
tumor positive
Stage II: Tumor extending Stage B: Grossly unre- Stage 2A: Unilateral tumor
in continuity beyond the sected primary tumor; with incomplete gross
organ or structure of ori- nodes and nodules the excision; identifiable
gin, but not crossing the same as in stage A ipsilateral and contralateral
midline; regional lymph lymph nodes negative
nodes on the ipsilateral microscopically
side possibly involved
Stage 2B: Unilateral tumor
with complete or incomplete
gross excision; with positive
ipsilateral regional lymph
nodes; identifiable contralat-
eral lymph nodes negative
microscopically
Stage III: Tumor extending Stage C: Complete or Stage 3: Tumor infiltrating
in continuity beyond the incomplete resection of across the midline with or
midline; regional lymph primary tumor; intra- without regional lymph node
nodes possibly involved cavitary nodes not adhe-involvement; or unilateral
bilaterally. rent to primary tumor tumor with contralateral
histologically positive regional lymph node involve-
for tumor; liver as in ment; or midline tumor with
stage A bilateral lymph node
involvement.
Stage IV: Remote disease Stage D: Dissemination Stage 4: Dissemination of
involving the skeleton, of disease beyond intra- tumor to distant lymph nodes,
bone marrow, soft tissue, cavitary nodes (i.e., bone, bone marrow, liver or
and distant lymph node extracavitary nodes, other organs (except as
groups liver, skin, bone defined in stage 4S)
marrow, bone, etc.)
Stage IV-S: As defined in Stage DS: Infants less Stage 4S: Localized primary
stage I or II, except for the than one year of age tumor as defined for stage 1
presence of remote dis- with stage IV-S disease. or 2 with dissemination
ease confined to the liver, limited to liver, skin, or bone
skin or marrow (without marrow
bone metastases)
Presently, trials are underway to randomize patients into low, intermediate and
high risk groups based on age, stage, histology and biologic markers as discussed in
39 previous sections. The therapies would then be streamlined based on the risk group
in an attempt to minimize morbidity from therapy while maximizing survival. Low
risk patients may get no or minimal chemotherapy, intermediate risk patients would
get moderately aggressive chemotherapy with or without a consideration for radia-
tion therapy. High-risk patients, in whom survival has not improved in the past
three decades, would get chemotherapy, radiation therapy followed by bone marrow
transplantation.
Selected Readings
1. Grosfeld JL. Neuroblastoma. In: O’Neill Jr. JA et al. eds. Pediatric Surgery, 5th
2. Shimada H et al. Identification of subsets of neuroblastoma by combined histo-
pathologic and N-myc analysis. J Natl Cancer Inst 1995; 87:1470.
3. Haase GM. Head and neck neuroblastomas. Semin Pediatr Surg 1994; 3:194.
4. Black CT. Neuroblastoma. In: Andrassy RJ ed. Pediatric Surgical Oncology. Phila-
delphia: W.B. Saunders Company 1998; 175-211.
CHAPTER 1
CHAPTER 40
Liver Tumors
P. Stephen Almond
Incidence
Pediatric liver tumors are a sufficiently diverse and uncommon group of tumors
that determine the true incidence of each is difficult. Together, they make up about
2% of all pediatric tumors and the majority (75%) are malignant (Table 40.1). This
Chapter focuses on hepatoblastoma, hemangioendothelioma, hepatocellular carci-
noma, embryonal sarcoma, focal nodular hyperplasia, and mesenchymal hamartoma.
The incidence of each tumor varies with age and gender (Table 40.1). In infancy,
hemangioendothelioma is more common followed by hepatoblastoma and mesen-
chymal hamartoma. Thereafter, hepatoblastoma is more common followed by
hemangioendothelioma, hepatocellular carcinoma, sarcoma, and mesenchymal
hamartoma.
Etiology
The etiology of most hepatic tumors is obscure. Hepatoblastomas arise from
embryonal tissue and have been associated with trisomy 2, trisomy 20, and chromo-
some 11 abnormalities. Hemangioendotheliomas occur due to abnormal mesen-
chymal development leading to large, multiple, thin-walled collections of vessels
within the liver. Hepatocellular carcinoma is associated with many chronic liver
diseases that are characterized by a continuous cycle of injury and repair, suggesting
recurrent injury or faulty tissue repair as etiologies. Embryonal sarcomas and mes-
enchymal hamartomas are also of mesodermal origin. The etiology of focal nodular
hyperplasia (FNH) is unclear.
Most children present with an abdominal mass, abdominal distension, or gas-
trointestinal symptoms (i.e., vomiting, anorexia).
Diagnostic Studies
The preoperative work-up determines the tumor’s organ of origin, the extent of
the primary tumor, and presence of metastatic disease. Laboratory studies include a
complete blood count, prothrombin time, partial thromboplastin time, platelet count,
liver function tests, hepatitis profile, and an alpha-fetoprotein level. Children with
liver tumors frequently are anemic (60%), have abnormal platelet counts and, in
hepatoblastoma and hepatocellular carcinomas, an elevated alpha-fetoprotein level.
Table 40.1. Incidence of liver tumors for infants (< 1 year old) and children
(< 15 years old) and the male to female ratio of each
Age
< 1 year < 15 years Female/Male
40 Hepatoblastoma 26 % 28% 1:1.6
Hemangioendothelioma 62 % 27% 1.6:1
Hepatocellular carcinoma 0.6 % 16% 1:1.8
Embryonal sarcoma 0.6 % 7.7% 1.7:1
Focal nodular hyperplasia 1% 5% 3.4:1
Mesenchymal hamartoma 8% 7% 1:1
Other 1.8 % 9.3%
Radiographic studies should include a chest x-ray, an abdominal ultrasound and

a computed tomography (CT) scan of the chest, abdomen, and pelvis. The chest
x-ray and CT of the chest are done to rule out pulmonary metastasis. The abdomi-
nal ultrasound is done to determine the organ of origin, the size, location, consis-
tency (solid vs. cystic), and vascularity of the tumor, and the patency of the portal
and hepatic veins. A CT scan of the abdomen further defines the extent of the
tumor and its relationship to other intraabdominal organs.
Pathology
Hepatoblastoma (Fig. 40.1) can involve the right (58%), left (15%), or both
lobes (27%) of the liver. Grossly, the tumor is usually singular, large, brown, and
with areas of hemorrhage and necrosis. Microscopically, they are classified as epithe-
lial, mesenchymal, or anaplastic. Epithelial tumors have both fetal and embryonal
cells where mesenchymal tumors have fetal, embryonal, and mesenchymal tissue.
The small cell, or anaplastic hepatoblastoma, is characterized by small, blue cells,
with little cytoplasm and hyperchromatic nuclei.
Hepatocellular carcinomas are large, multicentric, dark tumors that frequently
(up to 80%) involve both lobes of the liver. Microscopically, large, dark cells with
many nucleoli and tumor giant cells characterize hepatocellular carcinomas. In con-
trast, fibrolamellar hepatocellular carcinoma is usually a single mass with a
pseudocapsule characterized by tumor cells that are divided into lobules by fibrous
stromal bands and containing eosinophilic inclusions.
Embryonal sarcomas are usually singular, large, soft, yellow-colored lesions
involving the right lobe (75%). The lesion has cystic and solid components and
frequently exudes a light colored, gelatinous material when cut. Microscopically,
there is a pseudocapsule surrounding spindle-shaped cells suspended in an “acidic,
mucopolysaccharide-rich ground substance.” The majority of tumor cells contain
round, PAS positive, intracellular inclusions.
Hemangioendotheliomas are well-circumscribed, single or multicentric tumors
of various sizes. Microscopically, they are lined by endothelial cells and separated
from each other by connective tissue. Mesenchymal hamartomas (Fig. 40.2) are
large, complex (cystic and solid) tumors that usually involve the right lobe (75%).
Microscopically, the bulk of the tumor is mesenchyme but may also contain cysts,
Liver Tumors 179
40
Fig. 40.1. Intraoperative photograph of an intrahepatic hepatoblastoma involving

much of the left hepatic lobe.
Fig. 40.2. Infrahepatic nodular tumor of the left lobe that proved after excisional
biopsy to be a mesenchymal hamartoma.
bile ducts, hepatocytes, and inflammatory cells. Focal nodular hyperplasia is a well-
circumscribed, irregularly shaped, firm, light-colored tumor that occurs with equal
frequency in both lobes. The surrounding capsule frequently contains large blood
vessels. Microscopically, the tumor consists of normal appearing hepatocytes that
are subdivided into smaller lesions by fibrous connective tissue septa.
40
Classification and Staging
The Children’s Oncology Group (COG) uses the Intergroup Staging System for
the staging of hepatoblastomas and hepatocellular carcinomas. In children with stage
I disease, the tumor is confined to the liver and totally resected. In children with
stage II disease, the primary liver tumor is resected but there is evidence of micro-
scopic residual disease in the remaining liver or outside the liver. Children with stage
III disease have either gross tumor left behind, positive nodes, or tumor spill at
operation. Children with stage IV disease have metastatic disease.
Treatment
All children with solid lesions of the liver are explored through a transverse upper
abdominal incision. Ascitic fluid is sent for cytology. Suspicious lesions outside the
liver are biopsied and sent for frozen section. If possible, the primary tumor is removed
and the margins marked with titanium clips. If the tumor is not resectable (i.e.,
tumor in both lobes, tumor invading the portal vein, or tumor at the hepatic veins),
the abdomen is explored to confirm that the tumor is confined to the liver. Children
with unresectable tumors confined to the liver should be considered for
transplantation.
All children with a malignant tumor receive postoperative chemotherapy. There-
fore, a long-term, central venous catheter is placed at the end of the procedure.
Children with hepatoblastoma and hepatocellular carcinoma are entered into the
COG study group and treated with cisplatin, vincristine, and 5-FU. Children with
embryonal sarcomas are treated with vincristine, actinomycin-D, and cyclophos-
phamide.
Children with hemangioendotheliomas are not routinely explored as the major-
ity spontaneously involute in the first few years of life. In the interim, they are
followed closely for signs/symptoms of congestive heart failure, respiratory compro-
mise, and thrombocytopenia (Kasabach-Merritt syndrome). Congestive heart fail-
ure is caused by the large arteriovenous shunt within the liver and is treated with
digoxin and Lasix. Respiratory compromise is due to compression of the thoracic
cavity by the tumor. In these cases the child is supported (i.e., intubated if necessary)
while efforts are made to decrease the size of the tumor. Steroid, radiation, alpha-
interferon, and embolization have been used for this purpose. Thrombocytopenia is
due to platelet trapping within the hemangioendothelioma and has been treated
with aspirin, alpha-interferon, and steroids. Indications for operation include car-
diac decompensation and suspicion of malignancy. At operation, the hepatic artery
and even branches of the portal vein may require ligation.
Liver Tumors 181
Outcomes
For benign liver tumors, complete surgical resection is curative. Survival for chil-
dren with hemangioendothelioma varies from 32-75%. For malignant tumors, sur-
vival is better in children with stage I and stage II disease (vs. stage III and stage IV
disease) and hepatoblastoma (vs. hepatocellular carcinoma and embryonal sarcoma). 40
The 2-year survival for children with stages I or stage II hepatoblastoma is 90% vs.
67% for stage III disease. The overall survival for children with hepatocellular carci-
noma and embryonal sarcomas is 20%.
Selected Readings
1. Rowe MI, O’Neill Jr. JA, Grosfeld JL et al. Liver tumors. In: Rowe MI, O’Neill Jr.
JA, Grosfeld JL et al, eds. Essentials of Pediatric Surgery. St. Louis: Mosby-Year
Book 1998.
2. JM Becker, MS Heitler. Hepatic hemangioendotheliomas in infancy. SGO 1989;
168:189-200.
3. Chandra SR, Stocker JT. Hepatic neoplasms. In: Stocker JT, Dehnerj LP eds. Pedi-
atric Pathology, Chapter 19: The liver, gallbladder, and biliary tree. Philadelphia:
JB Lippincott Company 1992; 753-776.
4. Urban CE, Mache CJ, Schwinger W et al. Undifferentiated (Embryonal) Sarcoma
of the Liver in Childhood. Cancer 1993; 72:251-6.
5. King DR. Liver tumors. In: O’Neill Jr. JA et al, eds. Pediatric Surgery, 5th Edition.
St. Louis: Mosby 1999; 421-430.
CHAPTER 41
Teratomas
P. Stephen Almond
Incidence
Teratomas are interesting but uncommon lesions, probably occurring in 1 in
20,000-40,000 live births. The exact incidence is difficult to ascertain. The ana-
tomic distribution of these lesions varies between reporting institutions, but the
sacrococcygeal lesion appears to be most common.
Sacrococcygeal teratomas account for the majority of cases (45-65%). The next
most common locations are: gonadal (10-35%), mediastinal (10-12%), retroperito-
neal (3-5%), cervical (3-6%), presacral (3-5%), and central nervous system (2-4%).
Etiology
Teratomas arise from germ cells or other totipotential cells. Primordial germ
cells appear during the third week of gestation in the wall of the yolk sac near the
allantois. They move along the dorsal mesentery of the hindgut, reaching the genital
ridges by about the sixth week of gestation. Germ cells that do not complete this
journey can develop into teratomas. While the totipotential nature of germ cells and
their path of migration explain the location and pathology of the more common
teratomas (sacroccygeal and gonadal), intracranial and mediastinal locations are more
difficult to explain.
Sacrococcygeal Teratoma
Sacrococcygeal teratoma (Fig. 41.1)is the most common solid tumor in the neo-
nate. Prenatal discovery by ultrasound is becoming common. Polyhydramnios,
placentomegaly, and gestational age less than 30 weeks are associated with a poor
prognosis. The lesions vary in size, shape, location, and extension. Interestingly,
75% occur in females. On examination, the visible portion of the lesion is skin
covered and posterior to the anus. In some patients all or part of the lesion may be in
the retrorectal space and/or the retroperitoneum. In these cases, patients will present
with rectal pain, constipation, and/or a mass. The differential diagnosis is quite long
and includes lipoma, myelocystocele, pilonidal cysts, sacral dimple, diastematomy-
elia, meningocele, epidermal sinus, sacral agenesis, fetus in fetu, parasitic twin, hama-
rtoma, hemangioma, neuroblastoma, chordoma, rectal duplication, and sarcoma.
Associated anomalies occur in 10-15% of cases and include imperforate anus,
anorectal stenosis, anorectal agenesis, sacral hemivertebra, absence of the sacrum
Teratomas 183
41
Fig. 41.1. View of the most common teratoma of childhood, the sacrococcygeal
teratoma. This tumor occurs predominantly in females and generally arises be-
tween the tip of the coccyx and the anus.
and coccyx, and anterior meningocele. Currarinos triad is the association of a pre-
sacral mass with anorectal stenosis and a sacral deformity.
Diagnosis
The diagnosis of saccrococcygeal teratoma is usually made by physical examina-
tion. A chest xray is obtained to rule out metastatic disease. An abdominal film may
demonstrate calcifications within the mass or displacement of the bowel by the mass.
Ultrasonography is useful to determine the nature of the lesion (solid vs. cystic), the
presence of an intraabdominal component, and the presence of liver involvement.
Alpha-fetoprotein (AFP) and beta human chorionic gonadotropin (beta-hCG) are
serum tumor markers associated with teratomas and should be obtained preopera-
tively and followed postoperatively.
Classification
Saccrococcygeal teratomas are classified based on the location of the lesion. Type I
lesions are external with a small presacral component (45%). Type II lesions have an
external and a significant presacral component (34%). Type III lesions have a small
external component with the majority of the tumor being retroperitoneal (9%).
Type IV lesions have no external component, being entirely presacral (10%).
Treatment
Sacrococcygeal teratomas should be completely excised. Type I and II lesions can
be approached posteriorly through either an inverted chevron or sagittal incision.
Type III and IV lesions require an additional transverse lower abdominal incision.
Essential components of the procedure include complete removal of the intact tumor,
ligation of the middle sacral artery, and excision of the coccyx with the tumor. If the
lesion is benign (97%), no further therapy is indicated. These patients should be
41 evaluated every 3 months for the first two years with emphasis on rectal examina-
tion and AFP levels. If the lesion is malignant , adjuvant chemotherapy with cisplatin,
bleomycin, and vinblastine is indicated.
Ovarian Teratoma
Ovarian and sacrococcygeal teratomas occur with near equal frequency in infants.
In older children, however, ovarian teratomas are more common and account for
50% of all pediatric ovarian tumors. Abdominal pain, mass, and vomiting are the
most common presenting complaints. With the child supine, the mass is often vis-
ible and movable. If torsion has occurred, the abdomen may be tender. The differ-
ential diagnosis includes pregnancy, ovarian torsion, omental or mesenteric cyst,
lymphangioma, and lymphoma.
Diagnosis
Abdominal xrays may show displacement of the normal gas pattern and/or calci-
fications within the tumor. Ultrasonography can determine the organ of origin,
assess the contralateral ovary, and determine whether the lesion is solid or cystic.
Serum AFP and beta-hCG levels should be measured.
Treatment
The indications for operation include an ovarian lesion > 5 cms, a complex ova-
rian mass, and torsion. At operation, the surface of the affected ovary should be
inspected to insure that the capsule is intact and smooth. In addition, the perito-
neum and contralateral ovary should be evaluated to rule out metastasis and bilat-
eral lesions, respectively. If the affected ovary has a smooth, intact surface, the
contralateral ovary is normal, and there is no ascites or evidence of metastasis, the
tumor should be removed. Since 50% of ovarian tumors are teratomas and > 90%
of these are benign, an attempt should be made to preserve any remaining ovarian
tissue. If there are bilateral teratomas, both should be enucleated, with preservation
of ovarian tissue. If there is evidence of metastatic disease, diaphragmatic scraping,
peritoneal washes, and biopsies should be obtained. If there are immature elements
in the tumor, peritoneal implants, or peritoneal glial implants, the tissue should be
graded according to the Norris classification system. Grade II and III lesions require
postoperative cisplatin-based chemotherapy.
Teratomas 185
Retroperitoneal Teratomas
Retroperitoneal teratomas occur with equal frequency between the genders.
Children usually present with gastrointestinal symptoms or abdominal mass. In
addition to chest xray, abdominal plain films, and ultrasound, a CT scan may be
useful to determine the relationship of the tumor to other retroperitoneal structures
and distinguish it from a primary renal or adrenal tumor. The differential diagnosis 41
includes those listed for ovarian teratomas as well as Wilms’ tumor, neuroblastoma,
and sarcoma.
Treatment
Retroperitoneal teratomas should be removed. The vast majority are benign and
require no further treatment. Patients with malignant lesions and those with high-
grade immature elements should be treated with cisplatin-based chemotherapy.
Mediastinal Teratomas
Mediastinal teratomas may arise in the mediastinum, the pericardium, or the
heart. The latter two are mentioned only for completeness. Mediastinal teratomas
occur with equal frequency between the genders. They usually present with respira-
tory symptoms or chest pain. A small portion of boys with mediastinal teratomas
may present with precocious puberty as a result of a beta-hCG secreting tumor. The
differential diagnosis includes thymoma, parathyroid adenoma, bronchogenic cysts,
cystic hygroma, duplications, aneurysms, lymphoma, lipoma, myxoma, and thy-
roid goiter.
Diagnosis
Chest radiograph and CT scan are necessary to confirm the presence of a mass
and define its relationship with other intrathoracic structures. AFT and beta-hCG
levels should be drawn preoperatively and followed in the postoperative period. Boys
with beta-hCG secreting mediastinal teratomas should have chromosomal analysis
as there is an association between these lesions and Klinefelter’s syndrome.
Treatment
The treatment is surgical resection. Children with malignant lesions and those
with tumors containing high-grade immature elements also receive cisplatin-based
chemotherapy.
Head and Neck Teratomas
Intracranial Teratomas
Intracranial teratomas have a bimodal distribution occurring in infants < 2 months
and children 12-16 years. They usually originate from the pineal gland and increase
41
Fig. 41.2. Tonsilar teratoma invading the sinuses and cheek of a newborn female.
the intracranial pressure. In the newborn this is manifest as obstructive hydroceph-

alus and in the child as headaches, visual changes, seizures, and vomiting. On CT or
MRI, teratomas are typically calcified, supratentorial, midline lesions. In newborns,
most of these lesions are benign. In older children, the majority are malignant.
Treatment
Complete resection, although rarely possible, is the only chance for long-term
survival. Partial resection will provide palliation of symptoms in some cases.
Other Head and Neck Teratomas
Teratomas involving the neck or areodigestive tract are rare, gender nonspecific
lesions. They can obstruct the oropharynx leading to polyhydramnios and pulmo-
nary hypoplasia in the fetus and respiratory distress in the newborn. They may involve
the neck (thyroid teratoma), the oropharynx (Fig. 41.2), or the nasopharynx. The
differential diagnosis includes cystic hygroma, lymphangioma, branchial cleft cyst,
goiter, and neuroblastoma. Plain films show calcifications, while CT and/or MRI
demonstrate the extent of the lesion. The majority are benign and complete resec-
tion is the treatment of choice.
Testicular Teratomas
Testicular teratomas occur in infants and young adults and account for 7% of all
teratomas. They usually present as a painless testicular mass. Ultrasound may show
calcifications. Alpha-fetoprotein and beta-hCG should be drawn preoperatively. The
Teratomas 187
treatment of choice is high ligation of the cord. A retroperitoneal lymph node dis-
section is not indicated. Patients with malignant teratomas should receive chemo-
therapy.
Selected Readings
1. Azizkhan RG, Caty MG. Teratomas in childhood. Curr Opin Pediatr 1996;
8:287-292.
2. Pringle KC. Sacrococcygeal teratoma. In: Puri P ed. Newborn Surgery. Oxford:
Butterworth Heinemann1996; 505-511. 41
3. Altman RP, Randolph JG, Lilly JR. Saccrococcygeal teratoma: American Acadamy
of Pediatrics Surgical Section Survey-1973. J Pediatr Surg 1974; 9:389.
4. Currarino G, Coln D, Votteler T. Triad of anorectal, sacral, and presacral anoma-
lies. Am J Roentgenol 1981; 137:395.
CHAPTER 42
Ovarian Masses
Robert M. Arensman
Incidence
Ovarian masses—solid or cystic—are rare occurrences in childhood. With the
advent of ultrasonography, the detection of these rare events has occurred much
more frequently, especially for cystic lesions. Nonetheless, the total occurrence of
these lesions remains low.
Etiology
Exact etiologic causes of either cystic or solid ovarian masses remain unknown.
In neonates the appearance of cystic lesions is clearly related to the influence of
maternal steroid production. These simple follicular cysts generally regress quickly
and completely once the neonate is no longer within the milieu of high maternal
hormone influence. The appearance of cystic lesions later in life is likely due to
problems of hormonal regulation and balance, but the exact mechanisms are un-
known at this time. Solid ovarian masses are predominantly benign or malignant
tumors and are often associated with chromosomal anomalies. The most common
of these anomalies occur on chromosomes 1 and 12, but problems with 5, 7, 9, 17,
21, and 22 have all been reported. The exact biochemical significance of these
abnormalities has not yet been elucidated.
Ovarian masses may go undetected in many cases when they remain small and
produce no symptoms. With increase in size, pain is the most common complaint.
The pain is usually chronic but generally is not severe. If torsion occurs, the pain is
severe, constant, and often associated with signs of peritoneal irritation on physical
examination.
In children, parental discovery of an abdominal mass is often the first complaint.
Acts of bathing or dressing often lead to abdominal examination and notice of the
mass. In cases of endocrinologically active masses, the first noticeable abnormality
may be premature menarche or isosexual precocity.
Diagnosis
Ultrasonography has become the principle method to diagnose ovarian pathol-
ogy. This modality quickly, accurately, noninvasively localizes the ovarian structures
without the use of radiation. Ultrasonography accurately characterizes ovarian masses
Ovarian Masses 189
as cystic, solid, or mixed, and demonstrates the anatomic relationships to other

pelvic structures.
Computed tomography (CT) coupled with intravenous contrast infusion may
provide additional information. CT is particularly useful especially to demonstrate
variations in solid tissue density, such as intra-ovarian fat planes so characteristic of
teratomas.
Serum markers such as alpha fetoprotein and beta human chorionic gonadotro-
pin may be particularly helpful for both diagnosis and eventual monitoring in cases
of ovarian germ cell tumors (i.e., endodermal sinus tumor, choriocarcinoma).
Classification and Staging 42
The ovarian masses are first distinguished as cystic or solid. The cystic lesions
can be further classified as simple or complex. If there is any solid component within
the ovarian mass it should be classified within the types of ovarian tumors (Table 42.1).
Malignant ovarian tumors are staged by the gross pathologic findings at laparotomy
and according to the cellular origin of the tumor (Table 42.2).
Treatment
Simple cystic lesions are often serendipitous findings of antenatal ultrasound or
neonatal examinations done as part of an evaluation of congenital anomalies. The
majority of these lesions are simple follicular cysts associated with high maternal
steroid production. When reasonably small (< 7 cm) torsion is unlikely, regression
occurs rapidly, and serial ultrasound examination to monitor disappearance is suffi-
cient. If larger than 7 cm, surgical exploration with cystectomy is generally safer
(Fig. 42.1). Clearly, large cysts should be removed to relieve pressure and its conse-
quent pain, to prevent torsion, and to insure that it is not part of a large cystic
teratoma. In all these situations, attempt should be made to preserve normal and
functioning ovarian tissue.
Solid ovarian tumors need full surgical removal with an attempt to assure mar-
gins of resection that harbor no residual tumor if they are malignant. This may
mandate removal of Fallopian structures, removal of peri-ovarian structures, or radi-
cal resection of other pelvic structures involved with the tumor. Careful inspection
of contralateral structures is mandatory because solid ovarian tumors, especially ter-
atomas (Fig. 42.2), have been reported bilaterally in up to 10% of cases. Few sur-
geons would suggest a need to bivalve the contralateral ovary if inspection is normal
although this approach has previously been recommended.
In conjunction with surgical extirpation, a thorough search for metastatic dis-
ease is indicated—both radiologically and by careful physical examination at the
time of surgery. This allows for appropriate staging and directs further therapy. Dis-
ease extending beyond the ovary will absolutely require adjunctive chemotherapy.
Today this will generally involve the use of a platinum agent coupled with either
bleomycin, etoposide, or vinblastine. Currently, multi modal therapy can achieve
60-80% survival rates up to five years after diagnosis.
Table 42.1. Classification of ovarian tumors in children

Germ cell tumors
Germinoma Most primitive germ cell tumor

Embryonal Carcinoma
Endodermal sinus Tumor Most common malignancy of
ovarian origin in childhood.
Elevated levels of alpha
fetoprotein
42 Choriocarcinoma Tumor resembling placental
tissue; elevated levels of beta
human chorionic gonadotropin
Gonadoblastoma Tumor with features of
dysgenetic gonadal tissues
Teratomas and Germ cell origin, generally
Teratocarcinomas represent the three distinct cell
lines of mature tissue origin
Stromal tumors
Granulosa-Thecal Tumor Usually produces estrogen,

isosexual precocity
Androblastoma Usually produces testosterone
and virilization
Table 42.2. Ovarian tumor staging

Germ cell tumors Epithelial Tumors
I Limited to ovaries IA One ovary; capsule intact

Negative peritoneal washings IB Both ovaries, capsules intact
Markers decrease after surgery IC One or both ovaries; capsular
rupture; positive washings
II Microscopic residual disease II One or both ovaries with pelvic
Lymph nodes positive (< 2 cm) extension
III Gross residual tumor III One or both ovaries, metastases
Lymph nodes positive (> 2cm) outside pelvis or positive nodes
Contiguous visceral involvement
Positive peritoneal washings
IV Distant Metastases IV Distant Metastases
Ovarian Masses 191
42
Fig. 42.1. Large left ovarian cyst in an infant. Large arrow points to midline uterus
while the small arrows indicate the fimbriated ends of the Fallopian tube. Left
ovary is massively distended with a simple cyst.
Fig. 42.2. Large ovarian teratoma after surgical resection. Tortuous vessels and ir-
regularity of surface suggest malignancy but pathological slides revealed benign
ovarian teratoma with predominantly glial elements.
Selected Readings
1. Lovvorn HN 3rd, Tucci LA, Stafford PW. Ovarian masses in the pediatric patient.
AORN Journal 1998; 67:568-84.
2. Lazar EL, Stolar CJ. Evaluation and management of pediatric solid ovarian tu-
mors. Semin Pediatr Surg 1998; 7:29-34.
3. Helmrath MA, Shin CE, Warner BW. Ovarian cysts in the pediatric population.
Semin Pediatr Surg 1998; 7:19-28.
4. Hawkins EP. Germ cell tumors. Am J Clin Path 1998; 109:S82-8.
5. Rescorla FJ. Germ cell tumors. Semin Pediatr Surg 1997; 6:29-37.
42
CHAPTER 1
CHAPTER 43
Testicular Tumors
Daniel A. Bambini
Incidence
Testicular tumors account for less than 2% of pediatric solid tumors. In the
United States, the incidence in males less than 15 years of age is 0.5 per 100,000.
Only 2-5% of all testicular tumors occur in children. Racial differences are present;
the greatest incidence occurs in Asian populations.
Etiology
The etiology of testicular tumors is unknown, yet there is an association with the
dysplastic undescended testicle. Orchidopexy may or may not protect against
malignant degeneration if performed early. Children with undescended testicle remain
at high risk after surgery. Almost one quarter of cryptorchid-associated testicular
tumors occur in the contralateral descended testicle.
Peak incidence in children is 2 years with a second increase in frequency during
puberty. Testicular tumors in children present as painless, nontender masses often
discovered incidentally during evaluation of an inguinal hernia. One third of patients
will have an associated hydrocele. Hormonal effects from Sertoli or Leydig cell tumors
may produce the initial symptoms. Leydig cell tumors are the most common gonadal,
stromal tumor in children and may cause macrogenitosomia, gynecomastia, or pre-
cocious puberty. Sertoli cell tumors also may present as precocious puberty and are
associated with cardiac myxoma, endocrinopathies, and gynecomastia.
Physical examination reveals a painless, firm mass that fails to transluminate.
Asymmetry increases the suspicion of a testicular tumor. Physical examination should
also include a close evaluation of the abdomen and contralateral testicle. The differ-
ential diagnosis includes inguinal hernia, hydrocele, orchitis, testicular torsion, trau-
matic contusion, and tumor. Meconium peritonitis rarely causes a scrotal mass in
neonates, and splenogonadal fusion may also resemble a testicular tumor.
Diagnosis
The definitive diagnosis of testicular tumor is made from surgical exploration
and biopsy. Radiographic evaluation should also include scrotal and abdominal
ultrasound which further define the lesion and may identify other genitourinary
anomalies or retroperitoneal lymphadenopathy. CT scan is the preferred method for
evaluation of the chest and abdomen for metastatic disease. If disease appears local-
ized, CT scans are not performed until after the diagnosis of malignancy is con-
firmed. Serum levels of human chorionic gonadotropin (HCG)and alpha fetoprotein
(AFP) are obtained preoperatively.
Pathology
The majority (70%) of childhood testicular tumors are of germ cell origin.
Embryonal carcinomas and yolk sac (endodermal sinus) tumors account for
approximately 40%. Yolk sac tumors are the most common testicular tumors of
childhood and the mean age at presentation is three years. Almost all children with
yolk sac tumors will have elevated serum AFP levels (normal < 20 ng/ml).
Seminomas are very rare in children, but it is the most common malignancy to
43 develop in the undescended testicle. The overall risk of malignancy in undescended
testicle(s) is 5-30 times greater than that of a normally descended testicle.
Nongerminal cell tumors account for approximately 30% of all testicular tumors
in children. Sarcomas account for 33% of nongerm cell testicular tumors and rhab-
domyosarcoma is the most common of these.
Testicular tumors are classified as tumors of germ cell origin or as tumors arising
from the supporting stromal tissue within the testis (Table 43.1). Clinical staging
(Table 43.2) is based upon physical examination, surgical exploration, and radio-
graphic findings (i.e., U/S, CT, CXR, etc.)
Treatment
Exploration is via an inguinal incision. A transscrotal approach to biopsy or
excision increases the risk of local recurrence and should not be used. The inguinal
canal is entered and the cord structures are encircled. The venous and lymphatic
drainage are occluded to prevent tumor spread during manipulation of the tumor.
The testicle along with the cremasteric muscle, gubernaculum and the fascia envel-
oping the testicle is delivered into the surgical field (Fig. 43.1). If a mass is identi-
fied, wedge biopsy with frozen section is performed. Malignant tumors are treated
by radical orchiectomy. Benign lesions can be treated by enucleation or simple or-
chiectomy. In addition, children with Stage II or III disease require systemic chemo-
therapy and modified retroperitoneal lymphadenectomy. Teratocarcinomas have
lymph node involvement in 30% of children at the time of presentation; modified
radical lymph node dissection is recommended.
For yolk sac tumors, lymph node disease is absent in 80-90% of children at the
time of diagnosis. However, 10-20% with Stage I tumors will develop relapse which
can be identified by an increasing AFP. A serum AFP level is drawn 3-4 weeks fol-
lowing resection. If the level is normal and there is no evidence of metastatic disease,
radical orchiectomy alone is adequate treatment. Monthly AFP level and CXR are
obtained for two years. A persistent or rising AFP level is an indication for chemo-
therapy. The addition of modified radical lymph node dissection is controversial.
Testicular Tumors 195
Table 43.1. Classification of testicular tumors and relative incidence
Germ Cell Tumors 71%

Yolk Sac (Endodermal sinus) 15%
Embryonal Carcinoma 21-40%
Teratoma 19-26%
Teratocarcinoma 10-21%
Mixed Germ Cell 5-10%
Seminoma 2-3%
Choriocarcinoma < 1%
Sex Cord-Stromal Tumors 9% 43
Sertoli Cell 3%
Leydig Cell 5%
Granulosa Cell 1%
Mixed or Other 1%
Other Tumors 20%
Paratesticular (Rhabdomyosarcoma, Sarcoma, etc.) 13%
Lymphoma/Lymphoid 5%
Adrenal Rests <1%
Other Tumor or Tumor-like Lesions 2%
Table 43.2. Staging system for pediatric testicular tumors
Stage Description
I Tumor confined to the testis and completely excised by
radical inguinal orchiectomy
II Transscrotal orchiectomy with gross tumor spill, microscopic
residual in scrotum or less than 5 cm from proximal spermatic
cord, lymph nodes less < 2cm
III Retroperitoneal lymph nodes > 2 cm
IV Distant metastasis (above diaphragm, liver)
Rhabdomyosarcoma is the most common paratesticular neoplasm. Initial treat-

ment includes radical orchiectomy, unilateral pelvic and retroperitoneal lymphadenec-
tomy, and systemic chemotherapy. Radiation therapy is administered for residual
tumor or if nodal disease is present.
Outcomes
In general, children with Stage I testicular tumors have a 3-year disease-free sur-
vival rate near 85%. Overall survival form yolk sac tumors is around 70-90%. Most
43
Fig. 43.1. An example of the rather rare testicular tumors of childhood. This small,
ovoid tumor in a child with marked virilization proved to be a Leydig cell tumor.
metastasis and recurrences develop within 2 years of initial treatment. The overall
2-year survival of children with paratesticular rhabdomyosarcoma is approximately
75%.
Selected Readings
1. La Quaglia MP. Genitourinary tract cancer in childhood. Seminar Ped Surg 1996;
5(1):49-65.
2. Raffensperger JG. Tumors of the testicle. In: Raffensperger JG ed. Swenson’s Pedi-
atric Surgery, 5th Edition. Norwalk: Appleton & Lange 1990; 397-399.
CHAPTER 1
CHAPTER 44
Gastrointestinal Tumors
Ambrosio Hernandez and Dai H. Chung
Gastrointestinal (GI) tract tumors are rare in children; the majority of pediatric
GI tumors are usually lymphatic or stromal in origin. Pediatric GI tumors are found
throughout the GI tract and consist of various histologic subtypes, but the predomi-
nant type is non-Hodgkin’s lymphoma of the distal small bowel, cecum, and appen-
dix. Colorectal cancer is extremely rare in children; but it is the distant second most
common malignancy of the GI tract.
Esophagus
Extrinsic compression of the esophagus by other lesions can occasionally mimic
tumor-like findings, but malignant tumors of the esophagus in children are extremely
rare. Patients with long-standing pathologic gastroesophageal reflux (GER) may be
at risk for the development of glandular metaplasia of the distal esophagus, known
as Barrett’s esophagus. Identification and treatment of Barrett’s esophagus have been
reported in fewer than 200 pediatric patients. However, Barrett’s esophagus in chil-
dren is suspected to be more common than previously reported, and its prevalence
may increase secondary to an increasing number of children with GER. Barrett’s
esophagus is at risk for developing dysplasia and subsequent adenocarcinoma of the
esophagus. Antireflux procedures control reflux symptoms, but long-term surveil-
lance is needed to detect cases of dysplasia or esophageal carcinoma before transmu-
ral infiltration occurs.
Smooth muscles make up the predominant tissue of the esophagus. Benign and
malignant smooth muscle tumors (leiomyoma and leiomyosarcoma) are found in
less than 5% of all esophageal tumors in children. These tumors usually present as
multiple or diffuse lesions and are frequent in syndromic patients. The presenting
symptoms are those of esophageal obstruction: dysphasia, emesis of undigested food,
and chest pain.
Contrast esophagram identifies these tumors, and esophagoscopy reveals a
mucosa-covered constrictive mass, suggestive of stricture. Surgical resection for treat-
ment may require extensive dissection due to the diffuse nature of these tumors, and
large esophageal resections sometimes require esophageal substitution.
Stomach
Gastric teratomas represent less than 1% of all childhood teratomas. They are
large benign tumors that usually present early in life (first 3 months), frequently
along the greater curvature of the stomach in male infants. Malignant gastric ter-
atoma has not been reported. Plain abdominal radiographs often demonstrate
calcification within the tumor, and ultrasound or computed tomography can fur-
ther clarify the characteristic features of teratomas which are comprised of tissues
derived from multiple germ cell layers. Common presenting symptoms include
abdominal mass, distention, emesis, hematemesis or lethargy. Needle biopsy for
diagnosis is not accurate or necessary. Treatment is excision of the tumor and recon-
struction of the stomach. Patients undergoing partial or total gastrectomy must be
carefully followed for potential complications (i.e., pernicious anemia).
Small Intestine
The distal small bowel is a common site for gastrointestinal non-Hodgkin’s lym-
phoma in children. This type of tumor is more common in males who frequently
present with symptoms of abdominal mass and intermittent crampy abdominal pain.
These symptoms occasionally mislead clinicians to proceed with an appendectomy
44 only to encounter a large terminal ileal mass at the time of operation. Non-Hodgkin’s
lymphomas can also present with intestinal obstruction, perforation, or hemorrhage.
Treatment options for GI lymphomas include chemotherapy, radiation therapy
and surgical resection. The role of tumor debulking or complete surgical resection
for GI lymphoma is controversial, since data are inconsistent when comparing the
various modalities of treatment. However, those patients who present with intesti-
nal obstruction, perforation, or hemorrhage are probably best treated by resection.
The mean survival rate for stage II patients is 80% at 4.5 years of follow-up.
Appendix
Carcinoid tumors in the GI tract are most commonly found in the appendix.
Most tumors are asymptomatic and are discovered incidentally as part of the appen-
dectomy specimen. About half of the patients may present with acute symptoms
suggestive of acute appendicitis. These tumors are extremely rare in very young chil-
dren (< 4 years of age) due to the lack of enterochromaffin cells within the intestinal
tract in this age group. The incidence in children between 10 and 15 years of age is
0.4 per 100,000 per year in boys and is twice as common in girls.
Approximately 60% of carcinoid tumors are confined to the bowel at the time of
diagnosis. All carcinoid tumors are potentially malignant; however, their tendency
toward metastasis depends upon the site of origin. Appendiceal carcinoids are almost
never cancerous, whereas 70% of ileal and cecal carcinoids are metastatic.
The carcinoid syndrome, which consists of symptoms of flushing, diarrhea, or
bronchial constriction, occurs in 15-25% of patients who have hepatic or more
distant metastasis. These clinical manifestations are the result of various substances
(VIP, histamine, serotonin, bradykinin, prostaglandins) secreted by the tumor. The
diagnosis is confirmed by identifying elevated urine levels of 5-hydroxyindoleacetic
acid (5-HIAA), the by-product of serotonin.
Appendectomy is the treatment of choice for appendiceal carcinoid. In the small
bowel and stomach, carcinoid tumors tend to be multicentric and require wide
resections to include regional lymph nodes. Octreotide, a long-acting somatostatin
analog, has been effective in controlling clinical symptoms.
Gastrointestinal Tumors 199
Large Intestine
The majority of colonic polypoid lesions are benign and occur either spontane-
ously or in association with inherited polypoid diseases. Several polyposis syndromes
are described in children: juvenile polyposis, Cronkhite-Canada syndrome, Peutz-
Jeghers syndrome, and familial adenomatous polyposis coli (Gardner’s syndrome,
and Turcot’s syndrome).
Juvenile polyps are common benign polyps that are usually located within 10 cm
of the anus. They frequently present as painless rectal bleeding or as prolapsed masses.
These smooth round masses are hamartomas and have no malignant potential. In
asymptomatic patients, they may be observed, but larger (> 1cm) or symptomatic
polyps are removed endoscopically.
Juvenile polyposis syndrome refers to patients with multiple juvenile polyps that
are distributed throughout the GI tract. Symptoms include chronic bleeding and
hypoproteinemia. These polyps are considered premalignant by some because there
may be a family history of adenomatous polyposis and adenocarcinoma of the colon. 44
The incidence of cancer is 6%. Treatment options include endoscopic polyp removal
or surgical resection. Cronkhite-Canada syndrome represents juvenile polyposis com-
bined with skin hyperpigmentation and alopecia. These juvenile polyps are at risk
for malignant degeneration and should be monitored closely. Surgical resection is
considered for the segment of intestine with a high density of polyps.
Peutz-Jeghers syndrome is associated with hamartomatous or adenomatous GI
polyps and altered pigmentation of the mouth and skin. These polyps are often
large and pedunculate, and the incidence of colon cancer is 2-3%. Asymptomatic
small polyps are simply observed. Endoscopic polypectomy is the treatment of choice
for symptomatic polyps, and occasionally, laparotomy with open polypectomy or
bowel resection for extensive polyps may be required.
Familial adenomatous polyposis coli is an autosomal dominant polyposis syn-
drome in which hundreds of adenomatous polyps are found throughout the colon.
The median age for patient presentation is 16 years, and colorectal cancers appear at
around 36 years of age. Gardner’s syndrome combines colonic polyps with extra-
colonic soft tissue tumors, and Turcot’s syndrome is associated with brain tumors. A
positive family history mandates diagnostic colonoscopy no later than early in the
second decade. Common symptoms are bloody diarrhea, malnutrition, and ane-
mia. Patients require total colectomy because of the potential for malignancy. Tradi-
tionally, surgical therapy is total colectomy with ileoproctostomy or abdominoperineal
resection with permanent ileostomy. Alternatively, total colectomy with rectal
mucosectomy and ileoanal pull-through (J- or S-ileal pouch) eliminates the risk for
malignancy and provides a chance for continence without permanent ileostomy.
Patients with inflammatory bowel disease are at greater risk for developing colon
carcinoma. The overall rate of carcinoma in ulcerative colitis patients is 2-5%. These
patients are 20 times more likely to develop cancer than the general population.
After the first 10 years with ulcerative colitis, the chance of cancer development
increases by 1% per year. Cancers in this patient population are also very aggressive
and infiltrative. The patients who have had the disease for a minimum of 7 years
should undergo surveillance endoscopy with multiple random biopsies at least every
other year. Any evidence of mucosal dysplasia or severe clinical symptoms should
prompt patients to undergo proctocolectomy. The surgical options are similar to

those discussed for familial adenomatous polyposis coli.
In addition to polyposis syndrome and inflammatory bowel disease, there are
several other predisposing factors for the development of the colon cancer: a diet
high in fat and cholesterol and low in fiber, exposure to environmental chemicals,
radiation therapy, and chronic irritation from infection or ureterosigmoidostomy.
Colorectal carcinoma in children is sporadic in 75% of cases, and usually occurs in
the second decade of life. Common presenting symptoms include abdominal pain,
nausea, vomiting and change in bowel habits. Physical findings most commonly
seen are abdominal mass, distention, tenderness, rectal bleeding and weight loss.
Patients frequently present with an intestinal obstruction or an acute abdomen. If
plain radiographs demonstrate a mass lesion in the colon, a contrast enema can
identify the point of obstruction. Endoscopy allows tissue biopsy and confirmation
of diagnosis. Children with colorectal carcinoma have relatively evenly distributed
44 lesions throughout the colon, in contrast to adults. More than 80% of the tumors
are Dukes’ stage C or D at the time of diagnosis in children, and a frequent delay in
diagnosis in children may contribute to this finding. Additionally, an aggressive
mucinous type of adenocarcinoma is found in more than one half of the children
with colorectal tumors. The combination of the advanced stage of disease at the
time of diagnosis and the increased frequency of a mucinous subtype contribute to
the poor prognosis in children.
The primary treatment for colon cancer in children is wide surgical resection to
include the involved mesentery along with the lymphatic drainage area. Only about
50% of pediatric patients can have resection for cure. The ovaries and the omentum
are common sites of metastasis. There are no specific adjuvant therapy protocols
available for these children, but most patients will receive chemotherapy. Radiation
therapy is used selectively. The overall cure and survival rates of children with colon
carcinoma are poor; less than 5% of patients survive 5 years.
Selected Readings
1. Ford EG. Gastrointestinal tumors. In: Andrassy RJ ed. Pediatric Surgical Oncol-
ogy. Philadelphia: W.B. Saunders 1998.
2. Skinner MA, Plumley DA, Grosfeld JL et al. Gastrointestinal tumors in children.
Ann Surg Oncol 1994; 1(4):283-289.
3. Bethel CA, Bhattacharyya N, Hutchinson C et al. Alimentary tract malignancies
in children. J Pediatr Surg 1997; 32(7):1004-1009.
4. LaQuaglia MP, Heller G, Filippa DA et al. Prognostic factors and outcome in
patients 21 years and under with colorectal carcinoma. J Pediatr Surg 1992;
27(8):1085-1090.
CHAPTER 1
CHAPTER 45
Mediastinal Masses
Vicky L. Chappell and Dai H. Chung
The classic anatomic description divides the mediastinum, represented by the

substernal central thoracic space bounded laterally by the parietal pleura, into four
compartments: superior, anterior, middle and posterior. The superior mediastinum
lies between the thoracic inlet superiorly and the level of the fourth thoracic verte-
brae inferiorly, and contains the thymus, lymphatics and vascular structures. The
anterior mediastinum is the space below the superior compartment, bounded by the
pericardium and diaphragm. The middle mediastinum contains the pericardium,
heart, origins of the great vessels, trachea, main-stem bronchi and lymphatics. The
posterior mediastinum is bounded by the great vessels anteriorly and the vertebral
bodies posteriorly. The normal structures in this space include the esophagus, sym-
pathetic ganglia, thoracic duct, vessels and lymphatics. Understanding of the ana-
tomical subdivisions of the mediastinum and the relative frequency of specific
pathology in these subdivisions aids in the differential diagnosis (Table 45.1).
Etiology and Embryology
In addition to classification by location, mediastinal masses can also be catego-
rized as developmental, neoplastic, or inflammatory. It is presumed that incomplete
separation and tubulization of the esophagus and trachea after the proliferative phase,
which normally occurs by the fifth week of gestation, results in foregut duplication.
Additionally, these duplication cysts can communicate with the spinal canal, and
are referred to as neuroenteric cysts. The thymus develops as paired primordium
from the ventral third pharyngeal pouch and descends to an area anterior to the
aortic arch during the seventh week of gestation. Incomplete descent or obliteration
of its tract may result in a cystic or ectopic thymus in the neck. In the middle
mediastinum, bronchogenic cysts develop from abnormal budding of the tracheal
diverticulum or ventral portion of the foregut. Pericardial cysts occur from the fail-
ure of disconnected lacunae in the mesenchyme to coalesce to form the pericardial
sac.
The most common neoplasm of the anterior mediastinum in children is lym-
phoma, accounting for up to 45% of pediatric mediastinal masses. Germ cell tu-
mors (25%), mesenchymal tumors (15%), and thymic tumors (17%) comprise the
remainder. Most of these tumors are malignant. Neurogenic lesions, which com-
prise approximately 20% of mediastinal tumors, are usually located in the posterior
mediastinum.
Table 45.1. Mediastinal masses in the pediatric population

Compartments Pathology Incidence (%)
Superior & Anterior Lymphomas 54

Non-Hodgkin’s
Hodgkin’s
Germ cell tumors
Teratoma
Seminoma
Thymic lesions
Hyperplasia
Thymic cyst
Thymoma
Cystic Hygroma
Middle Lymphomas 26
Bronchogenic cyst
Granuloma
45 Pericardial cyst
Hamartoma
Posterior Neurogenic 20
Neuroblastoma
Ganglioneuroblastoma
Ganglioneuroma
Neurofibroma
Enteric (Duplication) Cyst
Acute infection of the mediastinum is most often seen following esophageal per-
foration, cardiac operations, or penetrating chest trauma. Many developmental con-
ditions of the mediastinum (i.e., thymic cysts, enteric cysts, bronchogenic cysts, and
cystic hygroma) are at increased risk for acquired infection.
Most mediastinal masses are asymptomatic, often discovered incidentally on chest
radiographs taken for other indications. However, clinical symptoms are frequently
the result of mass effects on normal structures within a particular compartment.
Large masses in the anterior and middle mediastinum are particularly significant for
their potential influence on respiratory tract symptoms, including airway obstruc-
tion. Symptoms may range from noisy, stridorous breathing in infants to cough,
chest pain, dyspnea, and orthopnea in older children. Cardiac compression may
result in cyanosis, syncope, and dysrhythmias. Great vessel compression can lead to
superior vena cava syndrome, characterized by venous engorgement along with head
and neck swelling. By contrast, posterior mediastinal masses can be quite large and
yet asymptomatic. However, posterior mediastinal masses, especially those that enter
neural foramina, can cause symptoms of spinal cord compression.
Certain pathology presents with specific symptoms. Hodgkin’s lymphomas may
have concomitant cervical or supraclavicular nodes as well as fever, night sweats and
Mediastinal Masses 203
weight loss (‘B’ symptoms) in one-third of patients. Neuroblastoma in the upper

mediastinum involving the stellate ganglion produces Horner’s syndrome, charac-
terized by ptosis, miosis, and anhydrosis. Although rare, a pediatric patient with
thymic neoplasia may present with myasthenia gravis or hypoplastic anemia.
Diagnosis
Two-view chest radiographs often confirm the presence of mediastinal masses.
Ultrasound study can differentiate the cystic nature of the mass, but contrast-enhanced
computed tomography (CT) provides far more information about the mass and its
relationship to the surrounding mediastinal structures. Magnetic resonance imag-
ing (MRI) is helpful to define spinal involvement or vascular lesions. However, the
sedation required for adequate MRI study in pediatric patients may compromise
the airway in patients with large anterior mediastinal masses. An esophagogram or
echocardiogram also provides additional information to further define mediastinal
masses.
Ultrasound or CT-guided percutaneous needle biopsy of solid mediastinal mass
can be performed to establish tissue diagnosis. Complications such as pneumotho-
rax, bleeding, perforation, or tumor seeding occur infrequently. Tissue diagnosis 45
may also be obtained from sites alternative to the tumor itself, such as lymph node
or bone marrow. Incisional or excisional biopsies of the tumor are performed using
thoracoscopy, mediastinoscopy or mini-thoracotomy. However, the goal of the pre-
operative work-up is to help define the optimal surgical approach for resection, and
open direct tissue biopsy of masses is rarely indicated in pediatric patients.
Tumor markers such as serum β-human chorionic gonadotropin (β-HCG) or
α-fetoprotein may help in the diagnosis and follow-up of malignant mediastinal
tumors. Since more than 90% of patients with neuroblastoma produce high levels
of catecholamines, quantification of the corresponding by-products (vanillylmandelic
acid, homovanillic acid) in urine over a 24-hour period can confirm the diagnosis.
Anterior and Superior Mediastinum
The common tumors in order of decreasing frequency are lymphomas, terato-
mas, germ cell tumors, lymphangioma (cystic hygromas), and thymic tumors.
Malignant lymphomas present most frequently in older children, and sometimes,
diagnosis can be sought from nonmediastinal areas such as bone marrow and nodal
tissues. Among Non-Hodgkin lymphomas, the lymphoblastic subtype is most likely
to present in the mediastinum. This is a diffuse, fast-growing tumor of T cell and
pre-B cell origin.
Teratomas are the second most common tumors of the anterior mediastinum.
They are derived from multiple germ cell layers and can have both cystic and solid
components. Teratomas frequently have calcifications, and only 25% of teratomas
are malignant in pediatric patients. β-HCG and a-fetoprotein can also help to dif-
ferentiate various germ cell tumors and are especially important postoperatively as
an early marker of recurrence. Seminomatous germ cell tumors are responsive to
radiation therapy and should be distinguished from other types.
Lymphangiomas in the mediastinum frequently present as a mediastinal exten-
sion of a cervical lesion. They demonstrate extensive endothelial-lined buds within
tissue planes and complete resection is required to avoid recurrence. Large
lymphangiomas may be treated with slcerosing injections (i.e., OK-432, a mono-

clonal antibody produced by Streptococcus pyogenes) but the value and efficacy of this
therapy is not established.
Thymic cysts are usually asymptomatic but can become infected. Large lesions
produce symptoms due to mass effect. Thymolipoma is benign, but along with
thymic cysts, resection is indicated for proper diagnosis and prevention of complica-
tions. Thymomas originating in the thymic epithelium are usually aggressive, but
account for less than 1% of mediastinal tumors. Thymomas associated with myas-
thenia gravis may produce autoantibodies to acetylcholine receptors which leads to
progressive muscle weakness. Resection produces some improvement in symptoms
for 30-50% of these children.
Middle Mediastinum
Pericardial cysts are benign, fluid-containing cysts lined with mesothelium. They
are usually asymptomatic and CT imaging can provide accurate diagnosis. When
the diagnosis is uncertain or the cysts become too large, thoracotomy or thoraco-
scopic resection or evacuation of the cysts should be performed. Pericardial effusion
45 may, on rare occasions, represent underlying pathology such as cardiac hemangioma
or rhabdomyoma. Bronchogenic cysts develop from abnormal budding of the tra-
cheal diverticulum or ventral portion of the foregut. These mucus filled cysts are
lined with bronchial epithelium and are frequently located at paraesophageal,
paratracheal, or perihilar regions. Excision is performed electively to avoid the com-
plications of infection, hemorrhage, or problems due to mass effects. Complete
resection via thoracotomy or video-assisted thorocoscopy is the preferred treatment
of bronchogenic cysts; recurrence and malignancy are extremely rare.
Posterior Mediastinum
The posterior mediastinum is the common site of benign and malignant neuro-
genic tumors. Sixty percent are malignant, most often neuroblastoma or
ganglioneuroblastoma, and thirty percent are benign tumors such as ganglioneuroma,
neurofibroma or schwannoma. The remaining 10% represent miscellaneous mesen-
chymal tumors or granulomas. Enteric duplication cysts may occur in this location,
are lined by esophageal or gastric epithelium, and occasionally communicate with a
viscus lumen. Most are asymptomatic and benign. Treatment is usually complete
resection, but stripping of the mucosal lining of a foregut duplication may be adequate
for long tubular duplications. Neuroenteric cysts are foregut duplications that also
have connections to the spinal canal. They often present as an intraspinal mass.
MRI or CT with myelogram should be considered when a posterior mediastinal
mass is associated with vertebral anomalies. Total excision is recommended with
simultaneous laminectomy as necessary.
Treatment
For cysts, regardless of symptoms, removal should be seriously considered to
prevent future complications of infection, bleeding or compression on adjacent nor-
mal structures. For similar reasons, benign mediastinal tumors should also be resected.
Ganglioneuromas and neurofibromas often remain encapsulated and can be easily
removed. The role of surgery regarding malignant tumors spans the spectrum from
Mediastinal Masses 205
diagnostic procedures to complete resection or debulking of the tumor mass to relieve

complications. Patients with Non-Hodgkin’s lymphoma and bulky anterior medias-
tinal involvement may require surgical intervention for respiratory symptoms, pleu-
ral effusion or superior vena cava syndrome. Neuroblastomas, when found in early
stages (I or II), are considered for complete primary resection. Seminomatous tumors
are treated with chemotherapy and radiation, while germ cell tumors of other origin
are treated with resection or debulking followed by chemotherapy.
In general, the surgical approach to mediastinal masses depends on location, size
and pathology. Thoracoscopy can be useful for resection or biopsy in approachable
lesions, such as foregut duplications (enteric cysts and duplications, bronchogenic
cysts, neurenteric cysts) and simple solid masses. Large anterior mediastinal masses
are best approached through a median sternotomy.
Outcome
Prognosis depends on the underlying pathology. Patients with benign cysts and
tumors have excellent outcomes with complete recovery. Recent protocols for
Hodgkin’s disease have improved the overall 5-year survival, which is now approaching
90%. Although, the youngest patients have the best prognosis, overall prognosis for 45
neuroblastoma remains poor.
Selected Readings
1. Philippart Al, Farmer DL. Benign mediastinal cysts and tumors. In: Oneill JA, Jr.,
Rowe MI, Grosfeld JL et al, eds. Pediatric Surgery, ed 5, St. Louis: Mosby 1998.
2. Grosfeld JL, Skinner MA, Rescorla FJ et al. Mediastinal tumors in children: Expe-
rience with 196 cases. Ann Surg Oncol 1994; 1(2):121-127.
3. Saenz NC, Schnitzer JJ, Eraklis AE et al. Posterior mediastinal masses. J Pediatr
Surg 1993; 28(2):172-176.
4. Kern JA, Daniel TM, Tribble CG et al. Thorascopic diagnosis and treatment of
mediastinal masses. Ann Thorac Surg 1993; 56(1):92-96.
5. Robie DK, Gursoy MH, Pokorny WJ. Mediastinal tumors-airway obstruction and
management. Semin Pediatr Surg 1994; 3(4):259-266.
CHAPTER 46
Breast Lesions
Breast lesions in children and adolescents are not uncommon. This Chapter
reviews normal breast development and describes several of the abnormal breast
conditions that may be encountered in pediatric patients.
Breast Development
The mammary glands are a modified and specialized type of sweat gland. Breast
development begins during the sixth week of gestation as a solid down growth of
epidermis into the underlying chest wall mesenchyme. In the embryo, male and
female breast development is identical. At birth the rudimentary mammary glands
of newborn males and females are similar, and no significant changes occur until the
pubertal period.
The breast of the newborn has characteristically poorly-formed, inverted nipples.
Shortly after birth, the nipples appear more raised above the underlying mammary
pits due to proliferation of the surrounding connective tissue of the areola. Neo-
nates frequently have secretion of colostrum-like fluid (called ‘witches’ milk’) from
the nipples in response to late gestational maternal hormones.
The average age of female thelarche is approximately 11 years of age (range 8-15
years). Breast growth and development is stimulated by the production of estrogen
and progesterone at the onset of puberty. Estrogen is the primary hormone that
produces ductal and stromal growth within the breast tissue. Progesterone initiates
alveolar budding, stimulates lobular growth, and contributes to secretory develop-
ment of the breast. Premature thelarche (Fig. 46.1) begins before 8 years of age and
occurs without other evidence of precocious puberty. Premature thelarche presents
as enlargement of one or both breasts and is a benign process. Biopsy is absolutely
contraindicated and may result in a small, underdeveloped breast. Precocious puberty
implies premature breast development in association with the presence of other sec-
ondary sex characteristics (pubertal hair, etc.) and generally indicates the need to
search for tumor producing estrogens.
Diagnostic Approaches to Breast Lesions
Evaluation of breast-related symptoms in children begins with a thorough his-
tory and physical examination. In adolescents, details regarding menstrual history
(onset, etc.) and the relation of symptoms to the menstrual cycle are obtained. A
family history of similar problems or other breast disease is investigated.
Breast Lesions 207
Fig. 46.1. A 7-year-old female with premature thelarche presenting as benign en- 46
largement of the left breast tissue with no other signs of puberty.
Diagnostic tests are chosen relative to the presenting clinical features and include
breast ultrasound, fine-needle aspiration, and very rarely open biopsy. Most of these
diagnostic tests are reserved for older children and adolescents presenting with a
breast mass. Ultrasound is particularly useful to distinguish cystic from solid lesions.
Mammography has practically no use in pediatric or young adult patients because
the dense fibrous tissue in these patients often precludes visualization of mammo-
graphic abnormalities (i.e., calcifications).
Congenital Breast Anomalies
Approximately 1-2% of females have abnormalities of breast development pre-
senting as extra breast tissue including polymastia (extra breast) and polythelia (extra
nipple). Supernumerary nipples are also relatively common in males. An extra breast
or nipple usually develops along the nipple line just inferior to an otherwise normal
breast. They can rarely appear in the axillary or abdominal wall regions. Amastia or
athelia are rarer occurrences and often accompany other chest wall anomalies. For
example, Poland’s syndrome includes amastia, aplasia of the pectoral muscles, rib
deformities and upper extremity defects.
Gynecomastia
Gynecomastia refers to benign enlargement of the male breast and is due to
proliferation of the glandular portion of the breast. However, this condition can be
confused with general obesity or pectoral muscle hypertrophy. Gynecomastia in
boys is most often due to pubertal changes (25%) but is idiopathic in just as many
(25%). Gynecomastia is also caused by drugs (10-20%), cirrhosis or malnutrition
(8%), primary hypogonadism (8%), testicular tumors (3%), secondary hypogonadism

(2%), hyperthyroidism (1.5%), and renal disease (1%). Management of pubertal
gynecomastia in adolescent boys includes careful history (i.e., drugs, kidney disease,
liver disease) and a thorough exam to include a careful testicular examination. If the
exam and history are negative, reassurance and periodic follow-up are all that is
needed. For those who absolutely refuse to outwait the enlargement (regression in
6-24 months), simple mastectomy and liposuction produce satisfactory immediate
cosmetic relief.
Breast Asymmetry
Breast asymmetry is common in pediatric patients, affecting as much as 25% of
the population. Unilateral hypomastia is most often due to inadequate end-organ
response to hormonal stimulation. Bilateral hypomastia may indicate an endocrine
or genetic abnormality that requires additional investigation (i.e., karyotyping, etc).
Generally, asymmetry is mild and transient. Seldom is cosmetic reconstruction
required.
Nipple Discharge
Serous or bloody nipple discharge in prepubertal patients is sometimes associ-
ated with infantile mammary duct ectasia, intraductal papilloma or cyst, or chronic
46 cystic mastitis. Galactorrhea, when not associated with pregnancy or nursing, is
abnormal and may be neurogenic, caused by hypothalamic or pituitary dysfunction
or other endocrine abnormalities. Prolactinomas are exceptionally rare in children.
Some drugs can cause galactorrhea, but many cases will remain idiopathic. These
forms of drainage require investigation, usually resolve spontaneously, and rarely
require surgery.
An intraductal papilloma most often presents as a subareolar lesion producing
sanguinous or serosanguinous nipple discharge. The differential diagnosis includes
fibroadenoma, cystosarcoma phylloides, papillary carcinoma, and mammary duct
ectasia. Ductography can occasionally help localize these lesions. Treatment is surgi-
cal excision of the entire involved duct. Most young women with this lesion are
postpubertal and have sufficient breast development to allow surgery without fear of
damaging an underdeveloped breast bud. Isolated papillomas confer no increased
risk for later breast malignancy, but diffuse juvenile papillomatosis is associated with
a greater risk of breast cancer.
Fibroadenoma
Fibroadenoma is the most common breast lesion in adolescent females. These
lesions are benign and occur as encapsulated, nontender mobile masses. The mor-
phology of fibroadenomas is quite variable. Most can be observed safely, but many
are removed in patients because of progressive enlargement, family history of malig-
nant breast disease, or because a malignant lesion can not be absolutely excluded. In
general, fibroadenomas are not considered a risk for future breast malignancy, but
patients with complex fibroadenomas, family history of complex fibroadenomas, or
associated proliferative disease are at increased risk (20% will develop breast cancer
within 25 years of diagnosis).
Breast Lesions 209
Giant (juvenile) fibroadenoma are greater than 5 cm in size and frequently double
in size within 3-6 months. Most frequently teens with this lesion present with a
rapidly enlarging, encapsulated breast mass. Treatment is surgical excision. Occa-
sionally reduction mammoplasty may be required to achieve acceptable cosmetic
results.
Breast Infection
Neonatal breast hyperplasia occasionally is complicated by infection, perhaps
initiated by over-manipulation. In neonates and prepubertal children, mastitis usu-
ally involves the entire breast complex and is caused most often by gram positive
bacteria (i.e., staphylococcus, streptococcus, etc). Systemic antibiotics are required
for the treatment of most breast infections in children. Incision and drainage may
be necessary in cases developing breast abscess. This is best performed through a
circumareolar incision whenever possible.
Cystosarcoma Phylloides
Originally believed to arise from a pre-existing adenoma, this lesion is now known
to occur de novo from breast parenchyma as a slow-growing painless mass. Approxi-
mately 20% of adolescents with this lesion will present with axillary adenopathy.
Histologically, there are three types:
1. benign,
46
2. intermediate,
3. malignant.
Size has no relation to whether malignancy is present or not. In malignant lesions,
the lung is the most common site of metastasis. Treatment is total excision of the
tumor mass. Initial radical resection (i.e., mastectomy) is not warranted unless evi-
dence of metastasis is present. Mastectomy is indicated for malignant lesions, very
large lesions (cosmetic consideration), and in cases of local recurrence of borderline
or malignant lesions.
Breast Cancer
Primary breast cancer is very rare in childhood. It accounts for less than 1% of all
childhood cancers and less than 0.1% of all reported breast cancers. Most cases
described are carcinomas, but rhabdomyosarcomas, sarcomas, and non-Hodgkin’s
lymphoma have occurred as primary malignancies in the breast of children. In
premenarchal children, treatment is by wide local excision. In postmenarchal chil-
dren, breast cancer should be managed as it is in adult patients. A full discussion of
breast cancer treatment is beyond the limits of this text.
The breasts of pediatric oncology patients should be examined regularly for signs
of metastatic disease. Metastatic leukemia and rhabdomyosarcoma present in the
breast. Complete metastatic work-up is indicated in addition to incisional/excisional
biopsy of the mass. Fine needle aspiration can frequently successfully make the diag-
nosis avoiding open biopsy. Secondary malignancy developing in patients treated
for other malignancies, especially children with previous Hodgkin’s disease who
received upper mantle radiation, is increasingly common. Radiation-induced breast
sarcoma has also been reported after treatment of Wilms’ tumor, and adrenal
carcinoma.
Selected Readings
1. Ciftci AO, Tanyel FC, Buyukpamukcu N et al. Female breast masses during child-
hood: a 25-year experience. Euro J Pediatr Surg 1998; 8:67-70.
2. Kaste SC, Hudson MM, Jones DJ et al. Breast masses in women treated for child-
hood cancer: incidence and screening guidelines. Cancer 1998; 82:784-92.
3. Pacinda SJ, Ramzy J. Fine-needle aspiration of breast masses. A review of its role in
diagnosis and management in adolescent patients. J Adol Health 1998; 23:3-6.
4. Sher ES, Migeon CJ, Berkovitz GD. Evaluation of boys with marked breast devel-
opment at puberty. Clin Pediatr 1998; 37:367-71.
5. West KW, Rescorla FJ, Scherer LR 3rd et al. Diagnosis and treatment of symptom-
atic breast masses in the pediatric population. J Pediatr Surg 1995; 30:182-7.
46
CHAPTER 47
Hodgkin’s Lymphoma
Hodgkin’s lymphoma is a malignant disease of the lymphatic system character-

ized by the presence of Reed-Sternberg cells in the lymphomatous tissue. The origin
of the Reed-Sternberg cells is unknown, but these cells are speculated to arise from
dendritic cells within the lymph node that function as antigen presenting cells.
Incidence
Hodgkin’s disease accounts for about 5% of childhood malignancies. It is rare
before the age of five years and has its peak incidence in the third decade of life. It is
more common in boys, but after the age of 12-13 years the sex ratio is shifted. It
may be slightly more common in black American children; geographic and socio-
economic forces may affect the incidence as well. The incidence in children is
increasing and currently is about 1.6 per 100,000 children per year.
Etiology
The etiology of Hodgkin’s disease is unknown. It is likely associated with the
Epstein-Barr virus, but a causal relationship has not been established.
The most common childhood presentation of Hodgkin’s lymphoma is painless
cervical or supraclavicular adenopathy. The nodes are firm, generally painless, and
often matted. Mediastinal lymphadenopathy is present in 50% of the patients at the
time the cervical findings are noticed. Greater than 85% of children with Hodgkin’s
disease will have disease within the chest at the time of presentation. The presence of
facial swelling or distended cervical veins alerts the physician to mediastinal disease
and the possibility of airway obstruction. Axillary and inguinal lymph nodes can be
involved but primary axillary or inguinal tumors are uncommon.
Systemic symptoms occur in approximately one third of children at the time of
diagnosis and indicate advanced disease. Systemic symptoms include:
1. recent (< 6 months) loss of greater than 10% body weight,
2. drenching night sweats, and
3. fever exceeding 38°C. Pruritis is no longer considered an important indi-
cator of extensive disease. Splenomegaly and/or hepatomegaly also indi-
cate more advanced disease.
Respiratory distress due to mediastinal lymph node enlargement is much less

common in children with Hodgkin’s disease than in children with Non-Hodgkin’s
lymphoma (NHL) but should always be considered prior to general anesthesia and
biopsy. The differential diagnosis includes systemic infections, infectious lymphad-
enopathy, NHL, Langerhan’s histiocytosis, mononucleosis, and other causes of gen-
eralized or localized lymphadenopathy.
Diagnosis
Children suspected of having Hodgkin’s disease receive a careful history and
physical examination. Posteroanterior and lateral chest x-rays and computed
tomography (CT) of the neck, chest and abdomen are routinely obtained. Gallium
scan and magnetic resonance imaging also provide accurate information regarding
the extent of disease and may be necessary to fully stage a patient. Bone marrow
aspiration and biopsy is also indicated.
Diagnosis depends on histologic evaluation and identification of Reed-Sternberg
cells, so biopsy is mandatory. If possible, several lymph nodes are evaluated histo-
logically. In the absence of palpable peripheral lymph nodes in a child with medias-
tinal disease, mediastinoscopy or thoracotomy are necessary to obtain tissue for
diagnosis.
Classification
The Rye classification includes four histopathologic subtypes of Hodgkin’s disease:
47 1. lymphocyte predominant,
2. mixed cellularity,
3. nodular sclerosing, and
4. lymphocyte depleted.
The relative ratio of normal lymphocytes to malignant (Reed-Sternberg) cells de-
creases progressively between lymphocyte predominant and lymphocyte depleted
subtypes. Overall, the most frequent subtype in children (< 16 years old) is nodular
sclerosing (> 65%). However, younger patients (< 10 years old) have an increased
relative proportion of the lymphocyte predominant and mixed cellularity subtypes.
Staging
Clinical staging is dependent on:
1. involvement of single or multiple lymph node regions,
2. involvement of single or multiple extra lymphatic organs, and
3. disease present on one or both sides of the diaphragm.
The stage is determined by a combination of clinical parameters including:
1. history,
2. physical exam,
3. x-rays,
4. CT scans of chest, abdomen and pelvis,
5. gallium scan, and
6. laboratory studies.
The Ann Arbor staging classification is the clinical staging system most com-
monly used (Table 47.1). The clinical stage (CS) is amended to pathologic stage
(PS) once the histologic results of the biopsy and staging laparotomy (if necessary)
Hodgkin’s Lymphoma 213
Table 47.1. Ann Arbor staging classification of Hodgkin’s lymphoma

Stage Description
I Involvement of a single lymph node region (I)
OR
Involvement of a single extralymphatic site or organ (IE)
II Involvement of 2 or more lymph node regions on same side of diaphragm
(II)
OR
Localized involvement of an extralymphatic organ or site and lymph node
involvement on the same side of the diaphragm (IIE)
III Involvement of lymph node regions on both sides of the diaphragm (III)
OR
Involvement of lymph node regions on both sides of the diaphragm with:
1.Extension to an extralymphatic site (IIIE)
OR
2. Involvement of the spleen (IIIS)
OR
3. Both 1) and 2) above (IIIES)
IV Diffuse or disseminated (multifocal) involvement of 1 or more extra-
lymphatic organs or tissues with or without lymph node enlargement
OR
Isolated extralymphatic organ involvement with distant (nonregional) nodal
involvement.
Subclassifications: A No symptoms
B Symptoms of fever, night sweats, and weight loss. 47
E Extranodal involvement *
S Splenic involvement
+ Pathologic confirmed involvement of extranodal site or
organ
* The subclassification E denotes minimal, localized, extralymphatic disease

resulting from direct extension from an adjacent lymph node area and can be
encompassed by a potentially curative radiation portal.
are known. Absolute pathologic staging requires laparotomy, splenectomy, and

biopsies of abdominal lymph nodes, liver, and bone marrow. Historically, staging
laparotomy changes the stage in 14-56% of children with Hodgkin’s disease. How-
ever, staging laparotomy is not now commonly performed and is only considered in
unique cases where it may significantly alter therapy (i.e., radiation being consid-
ered as only therapy). Subdiaphragmatic involvement is seen in up to 40% of patients
with cervical disease.
Treatment
The treatment of Hodgkin’s disease is determined by the stage of disease and
where it is located. For older patients with stage I or IIA disease, radiation therapy
alone to the involved area and contiguous lymphoid structures is successful. Low
stage patients with bulky mediastinal disease are at higher risk for recurrence and
therefore receive systemic chemotherapy in addition to mantle radiation. For patients
with Stage IIB or higher disease, multi-drug chemotherapy or a combination of
chemotherapy and radiation are used. Chemotherapeutic drug regimens include
MOPP (mechlorethamine, vincristine, procarbazine, prednisone) and ABVD
(adriamycin, bleomycin, vinblastine, dacarbazine). Combinations of the MOPP and

ABVD with lower radiation doses may decrease overall toxicity. Combined chemo-
therapy and lower dose radiation therapy is effective and achieves local control in
over 95% of cases. Although mantle radiation has proven very effective for localized
disease, combination therapy is used to control higher stages of disease and has been
successful in children with relapse.
Outcomes
Newer protocols give overall five-year survival in children close to 90%. The
youngest patients have the best prognosis. Histologically, the lymphocyte depleted
subtype has the worst prognosis, but this is very unusual in children. The lympho-
cyte predominant subtype has the best prognosis. Stage I patients have relapse free
and overall survival rates of 91% and 95%, respectively. Children with IIA disease
have a relapse free survival rate of about 80% and an overall survival rate of 92% at
5 years.
There are significant complications of treatment related to both chemotherapy
and radiation. The complications of chemotherapy, depending on the specific agents
used include myelosuppression, cardiovascular changes, pulmonary problems, gonadal
problems, and neurologic impairment. Most cases of testicular azospermia occur in
patients that have received six cycles of MOPP and often this problem is irreversible.
The use of ABVD diminishes the risk of sterility.
Mantle radiation causes adverse effects such as hypothyroidism, myelosuppression,
47 pericarditis, pneumonitis, nephritis, skeletal hypoplasia, bone osteonecrosis, gonadal
dysfunction and growth retardation. Impairment of growth is more common in
children less than 13 years of age who have received doses greater than 35Gy to large
areas of the body. The risk of thyroid dysfunction in children receiving more than
25Gy to the neck is almost 80%.
Secondary neoplasms (acute nonlymphocytic leukemia, thyroid carcinoma, par-
athyroid adenoma, soft tissue sarcoma, osteogenic sarcoma, non-Hodgkin’s lym-
phoma, etc.) are probably radiation induced. The incidence of a secondary leukemia
in children treated with MOPP is about 5-7% and increases to about 10% if radia-
tion therapy is administered. The peak incidence for leukemia following treatment
for Hodgkin’s disease occurs at 5 years following therapy. Solid tumors develop in
about 4-5% of patients.
Selected Readings
1. Azizkhan RG, Dudgeon DL, Buck JR et al. Life-threatening airway obstruction as a
complication to the management of mediastinal masses in children. J Pediatr Surg
1985; 20:816-22.
2. Levanthal BG. Management of stage I-II Hodgkin’s disease in children. J Clin Onc
1990; 8:1123-24.
3. Oberlin O. Present and future strategies of treatment in childhood Hodgkin’s lym-
phomas. Ann Onc 1996; 7(Suppl 4):73-78.
4. Beaty O, Hudson MM, Greenwald C et al. Subsequent malignancies in children and
adolescents after treatment for Hodgkin’s disease. J Clin Onc 13:603-609.
5. Cooper DL, DeVita VT. Hodgkin’s disease: Current therapy and controversies. Adv
Onc 1994; 10:17-24.
6. Whalen T. Hodgkin’s and Non-Hodgkin’s lymphoma. In: Andrassy RJ ed. Pediatric
Surgical Oncology. Philadelphia: WB Saunders Company 1998; 267-277.
CHAPTER 1
CHAPTER 48
Non-Hodgkin’s Lymphoma
Incidence
Lymphomas are the third most common pediatric malignancy. They are divided
into non-Hodgkin’s lymphoma (NHL) and Hodgkin’s lymphoma. NHL is less com-
mon than Hodgkin’s lymphoma and therefore accounts for less than 5% of pediatric
malignancies. Males are affected more than females by a ratio of 3:1.
Etiology
The etiology of NHL is unknown. However, the link between Epstein-Barr virus
(EBV) and endemic forms of Burkitt’s lymphoma observed in Africa and New Guinea
is well known. EBV deoxyribonucleic acid (DNA) and nuclear antigens have been
identified in 95% of African Burkitt’s lymphoma tumor cells. A sporadic form of
Burkitt’s lymphoma occurs in Europe and America. Although histologically similar
to the Burkitt’s lymphoma of Africa, only 15% have tumors with identifiable EBV
DNA. In addition, many cases of Burkitt’s lymphoma have a translocation of a
segment of the long arm of chromosome 8 containing the c-myc protooncogene.
The segment is frequently translocated to the long arm of chromosome 14. The
MIC2 gene, a commonly expressed gene in many small-, round-cell tumors of child-
hood, is expressed in many childhood lymphoblastic lymphomas.
The Epstein-Barr virus is also associated with other large-cell lymphomas found
in immunosuppressed patients (HIV, Wiscott-Aldrich syndrome, Bloom syndrome,
ataxia telangiectasia, X-linked lymphoproliferative syndrome, and organ transplant
recipients on immunosuppression).
Classification
In children with NHL, more than 90% of tumors belong to three histological
subtypes:
1. lymphoblastic lymphoma,
2. small noncleaved cell lymphoma (including Burkitt’s and non-Burkitt’s
lymphomas), and
3. large cell lymphoma (histiocytic). Each of these categories is subdivided
on the basis of histology and/or immunophenotype.
Lymphoblastic lymphomas account for 30% of childhood NHL and are
mostly tumors of thymocyte (i.e., T-cell) origin. These tumors consist of
lymphoblasts that are morphologically identical to acute leukemia T lymphoblasts.
Cells from lymphoblastic lymphomas are most often positive for the enzyme terminal
deoxynucleotidyl transferase (TdT) and have a T-cell immunophenotype. Rare

tumors are positive for the tumor marker CD10. Chromosomal abnormalities are
infrequently identified.
Small noncleaved cell lymphomas (Burkitt’s and non-Burkitt’s) account for
40-50% of childhood NHL. Small noncleaved cell lymphomas originate from B-cells.
Most cells express surface immunoglobulin M (either kappa or lambda light chain)
but they do not express terminal deoxynucleotidyl transferase (TdT). Almost all
tumors are CD10 positive. Chromosomal translocation (usually t(8;14), sometimes
t(8;22) or t(2;8)) are characteristic of these tumors. The translocation places the
c-myc gene, a cellular proliferation gene, near the immunoglobulin regulatory locus,
which often results in inappropriate expression of c-myc.
Large cell lymphomas (LCL), formerly histiocytic lymphoma, account for 20-25%
of childhood NHL. There are three subtypes based on reactivity to T- or B-cell
antibodies: B lineage, T lineage, or indeterminate lineage. B-lineage large cell lym-
phoma is further divided into large cleaved, large noncleaved, and immunoblastic
lymphoma; the distinction is probably not of major clinical significance. B-Cell
LCL are often immunotypically similar to small noncleaved lymphomas and chro-
mosomal abnormalities (i.e., t(8;14)) are sometimes present.
T-lineage large cell lymphomas are divided into two groups:
1. CD 30 positive anaplastic LCL, and
2. other T-cell lymphomas.
Over 90% of anaplastic large cell lymphomas are CD 30 positive and the major-
ity are of T-cell immunophenotype. Many CD 30 positive anaplastic LCL have a
characteristic translocation (2;5) (p23;q35). LCLs of indeterminate lineage are most
48 often CD 30 positive, usually classified as anaplastic LCLs, and have clinical out-
come similar to LCL of T-cell lineage.
In children, NHL occurs predominantly in the chest and abdomen. Sometimes
the disease presents with generalized lymphoid and extranodal involvement. All NHL
tumors grow rapidly and have a tendency for widespread systemic dissemination.
All lymphatic tissue may be involved including that of lymph nodes, Peyer’s patches,
the thymus, Waldeyer’s ring, pelvic organs, liver, and spleen. Extralymphoid
involvement can be seen in the skin, testis, bone and central nervous system (CNS).
Nearly 75% of children with lymphoblastic lymphoma present with a large,
bulky, anterior mediastinal mass which may compress the trachea or create superior
vena caval obstruction. Presenting symptoms include dyspnea, wheezing, stridor,
swelling of the head and neck, and sometimes dysphagia. Pleural effusions (often
bilateral) are frequently present. In patients with generalized lymphadenopathy and/or
hepatosplenomegaly, bone marrow and CNS involvement should be suspected. Bone
marrow involvement may cause diagnostic confusion in determining whether the
child has NHL with bone marrow involvement or leukemia. Although somewhat
arbitrary, patients with greater than 25% bone marrow blasts are considered to have
leukemia. Children with less than 25% bone marrow blasts are considered to have
lymphoma.
Small, noncleaved cell lymphoma most often (up to 90%) present in the abdo-
men as a fast growing tumor. Other sites of involvement include Waldeyer’s ring,
Non-Hodgkin’s Lymphoma 217
testis, nasal sinuses, bone, bone marrow, the central nervous system, peripheral lymph
nodes, and skin. However, the African endemic form often appears at the orbit or in
the jaw (>70%); the sporadic form almost always presents as abdominal disease. The
endemic form has a peak incidence at around seven years of age; the sporadic form
affects children from a broader age distribution. More than half of abdominal cases
involve the small bowel and probably originate in the Peyer’s patches. The present-
ing symptoms are usually abdominal pain, anorexia, or tenderness. Rarely the pre-
sentation is intestinal obstruction caused by intussusception with a lymphoma as a
lead point.
Large-cell lymphoma usually occurs at extranodal sites and is frequently widely
disseminated at the time of presentation. It seldom involves the gastrointestinal tract.
Primary sites include the skin, testis, eye, tonsils, soft tissues and sometimes the
mediastinum. The peak incidence of the large-cell lymphoma occurs in puberty.
Diagnosis
The heterogeneity of this disease makes it essential to perform diagnostic biopsy
to identify the histopathology. Immuno-phenotyping and cytogenic studies are
essential to establish the diagnosis. Approximately 20% of these children have bone
marrow involvement at the time of presentation; therefore, bone marrow biopsy
and aspiration are required. Lumbar puncture is used to identify cerebrospinal
involvement.
Radiographic imaging (i.e., computed tomography, chest x-ray, etc.) is the prin-
cipal means to evaluate the extent of the primary tumor. At most institutions, clini-
cal staging relies almost exclusively on the results of computed tomography. Computed
tomography of the chest, abdomen, and pelvis are routinely obtained to identify 48
and document the presence of disease above and below the diaphragm. Depending
on the site of the primary tumor (i.e., neck, spine, etc) other radiographic tests may
be necessary. Although staging laparotomy provides the highest diagnostic accuracy,
it is no longer routinely performed in children in most institutions.
Staging
Although several staging schemes exist, the most widely used staging system is
the one used at the St. Jude’s Children’s Hospital (Table 48.1). This staging system
distinguishes children with limited disease (i.e., single mass or locoregional disease)
from those with more extensive intra-abdominal or thoracic disease. Children with
a localized, nonabdominal, nonmediastinal disease (i.e., Stage I) have an overall
better prognosis than children with primary tumors in central nervous system,
mediastinum, thymus, epidural and paraspinal locations. The prognosis is unfavor-
able when disease exists on both sides of the diaphragm or when the disease is dis-
seminated (Stage III/IV).
Treatment
Treatment of NHL is based upon both histology, immunophenotype, and clini-
cal stage. All children with NHL are considered for entry into a clinical trial. NHL
in children is considered to be widely disseminated from the beginning, even when
the disease appears localized. Combination chemotherapy is recommended for all
patients. Radiation therapy is sometimes added for children with:
Table 48.1. St. Jude’s staging system for childhood non-Hodgkin’s lymphoma
Stage Description
Stage I Single tumor or nodal area outside the abdomen or mediastinum
Stage II Single tumor with regional node involvement
Two or more tumors or nodal areas on one side of the diaphragm
Primary gastrointestinal tumor (resected) with or without regional
node involvement
Stage III Tumors or lymph node areas on both sides of the diaphragm
Any primary intrathoracic disease or extensive intra-abdominal
disease
Any paraspinal or epidural tumors
Stage IV Any bone marrow* or CNS involvement regardless of other sites of
involvement
1. primary NHL of the bone,

2. central nervous system involvement ( sometimes emergency therapy),
3. testicular involvement,
4. severe mass effect ( i.e., superior vena caval compression or airway ob-
struction).
Children with localized disease do not benefit from radiation therapy, which, in
this setting, only increases toxicity. Typical multiagent chemotherapy includes treat-
ment with doxorubicin, vincristine, cyclophosphamide and prednisone followed by
oral 6-mercaptopurine and weekly methotrexate. Children with head and neck tu-
48 mors receive intrathecal therapy as prophylaxis for CNS disease.
The role of surgery in the initial management of NHL is mostly for incisional
biopsy to establish the diagnosis. Complete surgical resection of well-localized tu-
mors, particularly if confined to the bowel, may be beneficial to overall survival and
reduces complications such as tumor lysis syndrome or bowel perforation following
chemotherapy.
Children with large mediastinal tumors are at significant risk of cardiac or respi-
ratory arrest during general anesthesia. For this reason, the least invasive procedure
possible is used to establish the diagnosis of lymphoma. Bone marrow biopsy and
aspiration often provides the diagnosis and should be done early. Peripherally in-
volved lymph nodes are biopsied using local anesthetic and light sedation in most
instances. Pleural effusions aspirated via thoracentesis also can provide the correct
histologic diagnosis. If less invasive measures are not able to make the diagnosis,
computed tomography guided core needle biopsy of the mediastinal mass is consid-
ered. If necessary, mediastinoscopy, thoracoscopy, or anterior mediastinotomy are
the surgical procedures used to establish the diagnosis. If the risk of anesthesia or
heavy sedation is too great, preoperative treatment with radiation therapy or ste-
roids is considered. The diagnostic biopsy is obtained as soon as possible, once the
risk of general anesthesia is acceptably lowered. Preoperative treatment with steroids
and/or radiation therapy may affect the ability to obtain an accurate diagnosis.
Non-Hodgkin’s Lymphoma 219
Outcomes
Tumor burden at the time of diagnosis is the most important prognostic factor.
Children with Stage I disease have 90-95% long-term survival with multiple drug
chemotherapy with or without radiation therapy. Stage II patients have a 75% sur-
vival rate. In advanced cases, a multiple drug program offers about 70% relapse-free
survival. Patients with refractory disease or relapses are treated by autologous or
allogeneic bone marrow transplantation.
A major complication of therapy is tumor lysis syndrome that results from rapid
breakdown of malignant cells. Hyperuricemia compromises renal function. Initial
overhydration and pretreatment with allopurinol may prevent the adverse effects of
rapid tumor cell lysis. Gastrointestinal problems of bleeding, obstruction, and rare
perforation are also part of this syndrome.
Selected Readings
1. Hutchison RE, Berard CW, Shuster JJ et al. B-cell lineage confers a favorable out-
come among children and adolescents with large-cell lymphoma: A Pediatric On-
cology Group study. J Clin Onc 1995; 13:2023-2032.
2. Murphy SB, Fairclough DL, Hutchison RE et al. Non-Hodgkin’s lymphomas of
childhood: an analysis of histology, staging, and response to treatment of 338 cases
at a single institution. J Clin Onc 1989; 7:186-93.
3. Azizkhan RG, Dudgeon DL, Buck JR et al. Life-threatening airway obstruction as
a complication to the management of mediastinal masses in children. J Pediatr
Surg 1985; 20:816-22.
4. Magrath I. Malignant non-Hodgkin’s lymphomas in children. In: Pizzo PA, Poplack
DG. Principles and Practice of Pediatric Oncology, 2nd Edition. Philadelphia: JB
Lippincott 1993; 537-75. 48
5. Whalen T. Hodgkin’s and Non-Hodgkin’s Lymphoma. In: Andrassy RJ ed. Pediat-
ric Surgical Oncology. Philadelphia: W.B. Saunders Company 1998; 267-277.
CHAPTER 49
Rhabdomyosarcoma
and Other Soft Tissue Tumors
Marleta Reynolds
Incidence
Rhabdomyosarcoma is the third most common malignancy of childhood com-
prising 5% of childhood malignancies. Four to 7 cases per million population are
reported per year. The peak age of incidence is between 3 and 5 years but these
tumors have been found in neonates. The male-female ratio is 1:1.4. The head and
neck (40%), genitourinary tract (20%), extremities (18%) and trunk (7%) are the
most common sites. The retroperitoneum (7%), perineum and other sites (8%)
have also been involved. Bladder and vaginal rhabdomyosarcoma usually occur in
infants. Extremity rhabdomyosarcomas are most common in adolescents.
Clinical presentation varies with organ of origin. Eighty percent of patients present
with local or regional disease at time of diagnosis. Tumors that develop in the na-
sopharynx or auditory canal present with bloody or purulent drainage. If arising
from the muscles of head and neck, an asymptomatic firm swelling is apparent.
Orbital tumors often cause pain and swelling about the eye with proptosis and diplo-
pia. Bloody vaginal discharge or the presence of a red polypoid friable lesion at the
introitus are highly suggestive of rhabdomyosarcoma of the vagina. Bladder tumors
may mimic an infection with urinary frequency or other difficulties with urination.
Prostatic lesions can block the urinary flow necessitating catheterization. Rectal exam
is usually diagnostic as the prostatic lesion bulges into the rectum. Lesions of the
trunk or extremity present as a firm fixed mass beneath the subcutanteous tissue.
Differentiating these lesions from benign nodules or tumors is often impossible
without a biopsy. A family history should be taken because of the higher incidence
of soft tissue sarcomas found in some families manifesting the Li-Fraumeni syn-
drome. The Li-Fraumeni syndrome is a familial clustering of rhabdomyosarcoma,
soft tissue tumors in children, adrenocortical adenocarcinoma and early breast can-
cer in adults within families found to have a germline mutation of the p53 tumor
suppressor gene.
Diagnosis
Ultrasound, CT and MRI can be used to better define the anatomic relation-
ships of the primary tumor. Metastatic evaluation includes chest radiographs,
Rhabdomyosarcoma and Other Soft Tissue Tumors 221
abdominal and chest CT scan, bone scan, skeletal survey, bone marrow aspirate and
biopsy.
Diagnosis is made by incisional biopsy with frozen section analysis to be certain
that enough representative tissue has been sampled. Fresh tissue should be analyzed
for cell surface markers and chromosomes. Special stains can be used to differentiate
the small round blue cells of rhabdomyosarcoma from neuroblastoma, lymphoma
and Ewing’s sarcoma. Regional lymph nodes should be sampled in children with
limb primaries.
Pathology
There are three subtypes of rhabdomyosarcoma: pleomorphic, alveolar and
embryonal. The pleomorphic subtype is rarely diagnosed today and consists of sheets
of anaplastic cells. It carries a poor prognosis. There are conflicting opinions about
the differentiation between alveolar and embryonal rhabdomyosarcoma and the clini-
cal significance of establishing the histologic subtype. Newer classification systems
propose that any alveolar pattern identified in a tumor would group that tumor in
the alveolar subtype. Twenty to 30% of newly diagnosed rhabdomyosarcoma are of
the alveolar subtype. The embryonal subtype constitutes two thirds of newly diag-
nosed rhabdomyosarcoma. Molecular techniques to differentiate these subtypes are
being used more frequently and may clear up some of the confusion in the future.
The staging system used for the Intergroup Rhabdomyosarcoma Study (IRS)
with the results is outlined in Table 49.1.
Treatment
Complete resection of the primary tumor with wide margins can be accom-
plished in some cases. In lesions of the head and neck or pelvis this may not be 49
possible and a second look operation following chemotherapy can be performed.
Chemotherapy with vincristine, dactinomycin, and cylophosphanide (VAC) has
proven efficacy in low-stage tumors. Other drug combinations continue to be evalu-
ated to treat patients in the poor prognostic Stages II-IV and include etoposide and
ifosfamide. Radiation therapy is recommended for residual disease
Outcomes
The overall prognosis for survival in children with rhabdomyosarcoma is depen-
dent upon site of origin. Orbit lesions have the best overall prognosis with a 5-year
survival of 94%. The 5-year survival of other head and neck lesions is considerably
less at 50%. Survival at five years for paratesticular, vaginal and limb/trunk lesions is
81%, 67%, and 44%, respectively.
Lipomatous Tumors
Benign lipomas so often seen in adults are uncommon in children. Infiltrating
lipomas and lipoblastomas are the more common benign fatty tumors found in
children. Complete resection is indicated to prevent recurrence but may not be
feasible depending on location. Liposarcomas are very rare in children and wide
resection is indicated for cure.
Table 49.1. Rhabdomyosarcoma staging: Intergroup rhabdomyosarcoma study
Stage Description 5-Year

Survival
I Complete Excision Except Regional Disease 85%
II Excision with Microscopic Residual
(Margins or Regional Nodes) 40%
III Gross Residual Disease (Primary Site or Regional Nodes) 40%
IV Distant Metastases 35%
Dissatisfaction with this staging system has prompted the suggestion that the TNM
staging system be applied. This staging system is outlined in Table 49.2.
Table 49.2. TNM classification and staging: Rhabdomyosarcoma
Stage Sites T N M
I Orbit
Head and Neck (Excludes Parameningeal) T1 or T2 N0,N1,Nx M0
Genitourinary (Not Bladder or Prostate) a or b
II Bladder/Prostate T1 or T2 N0, Nx M0
Extremity a
Cranial, Parameningeal
Other
III Bladder/Prostate T1 or T2 a N1 M0
Extremity b N0,N1,Nx M0
Cranial, Parameningeal
Other
49
IV All T1 or T2 N0, N1 M1
a or b
T1-Confined to anatomic site of origin
T2 – Extension a < 5 cm diameter
b > 5 cm diameter
N-Regional Nodes
M-Distant Metastases
Fibrous Tumors
There is a wide spectrum of lesions represented in the category of fibrous tu-
mors. Differentiating the benign from malignant can be very difficult. Benign fi-
broma is a rare small lesion in the subcutaneous tissue that is treated with wide local
excision. The fibromatoses can have benign histology but behave aggressively be-
cause of location or invasiveness. Cure can only be obtained by wide local excision.
Mutilating surgery is not recommended until the tumor becomes “aggressive.”
Fibrosarcoma can present at any age. The most common locations are the trunk,
extremities, face and neck. The tumor presents as a rapidly growing, firm, painless
mass. Imaging of the primary site with CT or MRI is combined with metastatic
evaluation using chest radiograph and chest CT. Complete surgical excision is re-
quired for cure. Chemotherapy and radiation therapy have no proven efficacy.
Rhabdomyosarcoma and Other Soft Tissue Tumors 223
Selected Readings
1. Lobe TE, Weiner ES, Hays DM et al. Neonatal Rhabdomyosarcoma: The IRS
Experience. J Pediatr Surg 1994; 29(8):1167-1170.
2. Womer RB, Sinniah D. Soft Tissue Sarcomas. In: D’Angie GJ, Sinniah D, Mead-
ows AT et al, eds. Practical Pediatric Oncology. New York: Wiley-Liss 1992;
318-324.
3. Cofer BR, Weiner ES. Rhabdomyosarcoma. In: Andrassy RJ ed. Pediatric Surgical
Oncology. Philadelphia: W.B Saunders Company 1998; 221-237.
4. Malkin D, Li FP, Strong LC et al. Germline p53 mutations in a familial syndrome
of breast cancer, sarcomas and other neoplasms. Science 1990; 250:1233.
49
CHAPTER 50
Thyroid Masses
Robert M. Arensman
Incidence
Thyroid masses are reasonably rare in childhood. If all children are examined for
thyroid lesions, at most 1-3% will demonstrate a goiter or mass. Tumors, benign or
malignant, are not common until mid life. The use of iodized salt in the United
States has eliminated the prevalence of goiter that accounted for the large number of
thyroid masses in inland regions during the last century and the early part of this
century. Ionizing radiation is no longer used for trivial disease processes of young
children so postradiation tumors have decreased greatly.
Etiology
In cases of iodine deficiency or Grave’s disease the development of goiter is easily
explained due to hyperfunction to compensate for lack of substrate or overproduc-
tion of hormone. In the other thyroid lesions there is generally a lack of etiologic
causation with the exception of chronic lymphocytic thyroiditis that is associated
with antibodies to thyroidal tissue and appears to be an autoimmune disorder. Within
the United States previous radiation exposure, except in those who had a previous
malignant tumor, is now quite rare as a cause of thyroid abnormalities. Finally, there
is a small group of families that have the multiple endocrine neoplasia (MEN) syn-
dromes who develop medullary carcinoma as part of the pattern of their disease.
For most children, a neck mass is the presenting symptom (Fig. 50.1). The mass
occurs within the location generally held by the thyroid, rises and falls with swallow-
ing, and may be accompanied by nodes in the cervical chains. Toxic goiter will
obviously be associated with those problems occurring with increased thyroid hor-
mone: weight loss, change in skin or hair texture, heat intolerance, nervousness, etc.
Occasionally pain is associated with a thyroid mass, especially in cases of thyroiditis.
If a thyroid mass is sufficiently large, a child may have trouble with breathing or
swallowing, but such extreme cases are rare indeed.
Diagnosis
Generally, history and physical examination, thyroid hormonal testing, ultra-
sonography and thyroid scintigraphy will establish a diagnosis. History and physical
establish the location, possibility of hypo/hyper function, and associated abnormals
such as cervical adenopathy. Hormonal testing of blood demonstrates euthyroid, or
Thyroid Masses 225
Fig. 50.1. Large and slightly eccentric thyroid mass that proved to be an adenoma
(see Fig. 50.2) of the right thyroid lobe.
hypo/hyper functioning states consistent with the various goiters or toxic condi-
tions. Scintigraphy locates masses within the thyroid substance and also demon-
strates degree of function (cold vs toxic nodules); and ultrasonography is excellent
for localizing masses and demonstrating cysts.
Fine needle aspiration has proven very reliable in establishing diagnoses in adults.
There is much less experience with this technique in children, but the demonstra-
tion of clearly cystic lesions associated with normal cytology may help avoid need-
less biopsy in the younger patients also.
50
Pathophysiology
In toxic states, excessive thyroid hormone increase the metabolic rate to high
and ultimately dangerous levels. These can be initially controlled with hormonal
blocking drugs but ultimately a permanent form of therapy is required. When the
condition is simply a mass there may be no pathophysiologic consequence if the
mass is benign. Thus simple, small cysts may be apparent visually but of no conse-
quence to good health. However, nodules that are malignant pose threat to life and
will eventually metastasize to cervical nodes or the lungs if left untreated.
Classification
Thyroid masses are classified as benign or malignant. The more common causes
of thyroid masses in children are listed in Table 50.1.
Although thyroid masses are rare within childhood years, almost 20% are malig-
nant, especially if the nodule is “cold” on scintigraphy and occurs in a female.
Malignancies are most often papillary histology unless associated with the multiple
endocrine neoplasia (MEN) syndromes. Mutations in the RAS and RET proto-
oncogenes have been reported frequently in association with thyroid cancers,
particularly the follicular and medullary types.
Table 50.1. Causes of thyroid masses in children
Benign Malignant
Goiter Papillary Carcinoma
Thyroiditis Follicular Carcinoma
Grave’s Disease Medullary Carcinoma
Adenoma
Toxic
Nonfunctioning
Cyst
50
Fig. 50.2. Intraoperative view of a thyroid adenoma. Note large vessels on the
surface supplying blood to this greatly enlarged thyroidal tissue.
Treatment
Treatment rests on correct determination of the pathologic diagnosis. Small cys-
tic lesions of the thyroid may be aspirated for cytology and observed. Goiters due to
deficiency of substrate are treated with hormone replacement. Grave’s disease is treated
with blocking therapy until a euthyroid state is achieved or the best control possible
is achieved. Surgical resection (Fig. 50.2) can then be offered, especially in young
children for whom systemic radiation should be avoided. In chronic thyroiditis,
surgery offers little other than a cosmetic reduction of a neck mass unless airway or
swallowing problems are present.
In cases of malignancy, thyroidectomy with lymph node sampling is critical to
establish diagnosis and disease extent. Removal of the thyroid facilitates treatment
with radioactive iodine and future surveillance for recurrent or residual disease.
Thyroid Masses 227
Outcomes
Children with partial or total thyroidectomy for benign disease do very well.
They recover quickly from surgery, generally need close observation for compliance
in taking replacement or suppressive thyroid hormone, but do well in long term
studies. Children with thyroid malignancies have long term survival with very low
mortalities at 5, 10, and 15 year follow-up. However, eventual appearance of meta-
static disease is reported in up to 50-80% of these children with ultimate early
termination of life in these patients.
Selected Readings
1. Rallison ML, Dobyns BM, Meikle AW et al. Natural history of thyroid abnormali-
ties: Prevalence, incidence, and regression of thyroid diseases in adolescents and
young adults. Am J Med 1991; 124:225.
2. Epstein FH. The molecular basis of thyroid hormone action. N Engl J Med 1994;
33:847.
3. Gorlin JB, Sallan SE. Thyroid cancer in childhood. Endrocrinol Metab Clin North
Am 1990; 19:649.
4. Raab SS, Silverman JF, Elsheikh TM et al. Pediatric thyroid nodules: Disease de-
mographics and clinical management by fine needle aspiration biopsy. Pediatrics
1995; 95:46.
5. Newman KD. The current management of thyroid tumors in childhood. Semin
6. Smith MB, Xue H, Strong L et al. Forty-year experience with second malignancies
after treatment of childhood cancer: analysis of outcome following the develop-
ment of the second malignancy. J Pediatr Surg 1993; 28:1342.
50
Section VI: Gastrointestinal Hemorrhage
CHAPTER 1
CHAPTER 51
Rectal Bleeding in Infancy

Daniel A. Bambini
Incidence
Rectal bleeding, although not a frequently encountered presenting symptom in
neonates or infants, causes significant parental anxiety and should be regarded seri-
ously. Because rectal bleeding may result from several different diagnoses, its exact
occurrence is difficult to quantitate. Blood loss is usually minor; massive rectal hem-
orrhage is uncommon.
Etiology
Rectal bleeding in infancy can occur as a result of hemorrhage at upper or lower
gastrointestinal sites. Upper gastrointestinal bleeding is defined as hemorrhage that
occurs from a source proximal to the ligament of Trietz. Lower gastrointestinal bleed-
ing occurs distal to the ligament of Treitz. Although many common etiologies of
rectal bleeding in infancy have been described (Table 51.1), clinical diagnosis may
not be possible in nearly one half of these children.
Clinical presentation depends almost exclusively upon the underlying etiology.
The history and exam are important to narrow the field of possible etiologies. The
presence or absence of related symptoms and/or signs are often helpful to establish
the cause. Rectal bleeding may be in the form of hematochezia, melena, or occult
blood. Hematochezia or bright red blood from the rectum, although usually from a
distal gastrointestinal lesion, can occur from either upper or lower gastrointestinal
sources. Melena or tarry stools only occurs when the bleeding occurs from a lesion
proximal to the ligament of Trietz.
Perhaps the most common presentation of rectal bleeding in infancy, an other-
wise healthy-appearing infant will be noted by the parent to have a small amount of
bright red blood on the diaper or on the outside of stool. An anal fissure (Chap-
ter 27) is the most likely etiology in this scenario, and is often identifiable as a small
tear or ulceration at the anal verge usually located posteriorly. Anal fissures are very
unusual in breast-fed infants.
Necrotizing enterocolitis (Chapter 67) may also produce rectal bleeding in in-
fancy (usually in premature infants), although rectal bleeding is seldom the primary
symptom. The diagnosis is suggested by history which may include prolonged gas-
tric emptying, feeding intolerance, apnea, jaundice, abdominal distention, vomit-
ing, thrombocytopenia, leukocytosis, other signs of sepsis. Recurrent rectal bleeding
Table 51.1. Causes of rectal bleeding in infancy

Age
Location of Bleeding Source Neo- 1mo 1 yr
nate to to
(< 1mo) 1 yr 2 yr
Upper Gastrointestinal:
Hemorrhagic disease +
Swallowed maternal blood +
Esophagitis or Gastritis (see Chapter 55) + ++
Peptic Ulcer Disease (see Chapter 55) ++
Gastric teratoma
Esophageal or Gastric varices (see Chapter 56) +
Lower Gastrointestinal:
Anal fissure (see Chapter 27) +++ ++
Necrotizing enterocolitis (see Chapter 67) ++
Gangrenous bowel ++
Malrotation with midgut volvulus (see Chapter 60) ++
Hirschsprung’s Disease with enterocolitis (see Chapter 64)
Allergic proctocolitis
Intussusception (see Chapter 25) ++ +
Prolapse (see Chapter 27)
Polyps (see Chapter 54) ++ +
Meckel’s Diverticulum (see Chapter 57) ++
Lymphonodular hyperplasia
Enteritis (i.e., campylobacter, Yersinia, salmonella)
Inflammatory Bowel Disease (see Chapter 94) ++
Intestinal duplication (see Chapter 66)
GI vascular malformation
(+++ most common cause, ++ more common cause, + relatively more common)
after recovery from necrotizing enterocolitis (NEC) suggests recurrent NEC or a

51 post-NEC gastrointestinal stricture.
Intussusception (Chapter 25) occurs most commonly in infants between 6 and
18 months of age. Rectal bleeding associated with intussusception is classically de-
scribed as having a “current-jelly” appearance, which is probably only apparent in
about a third of cases. Intermittent abdominal pain is the usual distinguishing symp-
tom. A palpable sausage-shaped abdominal mass helps establish the diagnosis. A
barium or pneumatic enema is performed to both confirm and reduce the intussus-
ception.
An acute onset of melena and bilious emesis in an otherwise healthy baby sug-
gests malrotation with midgut volvulus (Chapter 60). At onset, the physical exami-
nation may be unremarkable. With time, the abdomen will become progressively
distended and tender to palpation. An upper gastrointestinal contrast study should
be obtained immediately to confirm the diagnosis. Emergent laparotomy is indi-
cated. Gangrenous bowel from midgut volvulus or other causes (segmental small
bowel volvulus, internal hernia, sigmoid volvulus, etc.) is the second most common
of rectal bleeding in infants between one and 12 months of age.
Rectal Bleeding in Infancy 231
Diagnosis
The exact origin of gastrointestinal hemorrhage remains undiagnosed in about
30-50% of neonates and infants with rectal bleeding. For most of these infants, the
blood loss is minor, self-limited, and seldom recurs. Diagnosis begins with a thor-
ough history and physical examination. Diagnostic evaluation of significant or re-
current hemorrhage may include upper and/or lower gastrointestinal endoscopy and
radiographic procedures including contrast enema, enterolysis, tagged red cell stud-
ies, arteriography, etc.. The choice of diagnostic tests and the urgency of the diag-
nostic work-up should be based upon the most likely etiologic lesion as well as the
severity of bleeding. Minor bleeding may resolve spontaneously and require no fur-
ther evaluation. Major bleeding (i.e., shock, transfusion) requires aggressive evalua-
tion.
Melena per rectum suggests upper gastrointestinal hemorrhage. The initial diag-
nostic maneuver for suspected upper GI hemorrhage is to place a nasogastric tube.
Aspiration of gross blood or “coffee ground” appearing fluid confirms the presence
of upper gastrointestinal bleeding. Absence of bile in an otherwise nonbloody gas-
tric aspirate does not exclude the possibility of upper gastrointestinal hemorrhage
arising distal to the pylorus of the stomach.
Treatment
The treatment of rectal bleeding in infants depends upon accurate identification
of the source of bleeding. In many infants, if not most, diagnosis is not possible and
reassurance to the parents is all that can be offered. Given the large array of entities
which can cause rectal bleeding in infants, a detailed discussion of treatment for
each lesion is beyond the scope of this Chapter. Information regarding the treat-
ment of many of these entities is provided elsewhere in this book (Table 51.1).
Selected Readings
1. Sherman N, Clatworthy H Jr. Gastrointestinal bleeding in neonates: a study of 94
cases. Surgery 1967; 62:614-619.
2. Spencer R. Gastrointestinal hemorrhage in infancy and childhood: 476 cases. Sur-
gery 1964; 55:718-734.
3. Arensman R. Gastrointestinal bleeding. In: O’Neill Jr. JA et al. eds. Pediatric Sur- 51
gery, 5th Edition. St. Louis: Mosby 1253-1256.
CHAPTER 52
Polyps of the Gastrointestinal Tract

Riccardo Superina
Incidence
Intestinal polyps are much less common in children than in adults, and their
association with syndromatic clusters is very common. Malignant transformation
except in the syndromatic cases is less than in adults, and the approach to manage-
ment is more expectant. Approximately 1% of children have asymptomatic intesti-
nal juvenile polyps which are benign. Other types of polyps are much rarer.
Etiology and Pathology
The etiology of polyps in children is multifactorial and depends on the type of
polyp. Etiologies and pathologic features will be discussed individually in the classi-
fication section.
Bleeding
Lower intestinal bleeding is the hallmark presentation of most polypoid condi-
tions. The bleeding is frequently associated with crampy abdominal pain. The blood
is usually red, indicating its origin in the lower gastrointestinal tract, and small in
quantity, unlike bleeding from duplications or Meckel’s diverticula with peptic ul-
ceration. If the bleeding is from polyps in the small bowel, the blood will appear
darker. In the rare cases of duodenal or gastric polyps, rectal bleeding may appear
black (i.e., melena).
Pain
Crampy abdominal pain is a frequent symptom along with bleeding. The pain
does not necessarily occur with the bleeding.
Intussusception
Traction on a polyp may cause intussusception anywhere it occurs. Usually,
colocolonic intussusception occurs only when a colonic polyp serves as a lead point.
The symptoms include crampy, intermittent pain, bleeding from venous engorge-
ment of the mucosa, and signs of intestinal obstruction (i.e., vomiting, distention,
obstipation). Unlike idiopathic intussusception, intussusception from a polyp occurs
in older children and may not be reduced with contrast enema.
Polyps of the Gastrointestinal Tract 233
Diagnosis
The diagnosis of polypoid lesions depends primarily on two modalities: intesti-
nal contrast studies and endoscopy. Endoscopy is advantageous as it can be both
diagnostic and therapeutic. Contrast studies for colonic polyps can be very accurate
and can be used to follow polyps for changes in number and size. Upper intestinal
polyps in the stomach and duodenum are also accurately visualized with contrast
studies.
Small bowel polyps may be difficult to image even with small bowel enemas.
Small bowel polyps are notoriously difficult to diagnose, and thankfully, occur only
very rarely. Diagnoses is most often made at the time of laparotomy when bleeding
or obstructive symptoms have prompted an operation.
Classification
Benign
Isolated Juvenile polyps
These are the most common polypoid lesion of infancy and childhood. The
peak age of incidence is between the ages of 3 and 10 years. As with most polyps,
crampy abdominal pain and bleeding with bowel movements are the presenting
symptoms. Juvenile polyps are hamartomatous excrescencies of the intestinal mu-
cosa. They appear to lengthen from traction caused by peristalsis and the flow of
intestinal contents. There is no malignant potential, and juvenile polyps naturally
auto-amputate if given enough time. Seventy-five percent of juvenile polyps occur
in the rectum and sigmoid colon, but juvenile polyps may occur in the right colon
as well.
Peutz-Jeghers Syndrome
This well-known syndrome causes polyps predominantly in the small bowel. Its
hallmark distinguishing feature is the pigmented lesions observed on the buccal
mucosa and lips of these patients. Malignant degeneration can occur, and lifelong
surveillance is necessary.
Adenomatous Polyp
This lesion is rare but known to occur in childhood (Fig. 52.1). Malignant de- 52
generation can occur as in the adult-type lesion. Familial adenomatous polyposis
(FAP) is a syndrome that results in multiple colorectal polyps (see below). Tradition-
ally, the presence of at least one hundred individual polyps is required to make this
diagnosis.
Hemangiomatous Polyps
Hemangiomatous polyps cause profuse bleeding and occur predominantly in
the distal small bowel. Profuse bleeding may require excision if it occurs repeatedly.
They tend to regress with time as do most hemangiomas after the age of two years.
Fig. 52.1. Two sessile, adenomatous polyps in a young girl whose mother and four
sisters all had familial polyposis.
Malignant
Juvenile Polyposis and Familial Adenomatous Polyposis (FAP)
Juvenile polyposis is an autosomal dominant disorder which causes polyps pre-
dominantly in the large and small bowel. The lesions resemble adenomatous polyps
individually but are actually mucous-retention polyps. These polyps can occur any-
where along the gastrointestinal tract. It is considered a premalignant condition and
6% of these children will eventually develop malignancy.
Familial adenomatous polyposis is characterized by hundreds of adenomatous
polyps in the rectum and colon causing diarrhea and bleeding. It also shares an
autosomal dominant pattern of inheritance. Malignant degeneration in one or more
polyp is virtually certain before the age of twenty years.
Adenocarcinoma
Although rare, isolated colonic or small bowel adenocarcinoma can occur in
52 childhood. It can be mistaken for a juvenile polyp until it has advanced beyond the
stage where it can be excised completely. Adenocarcinomas usually arise from villous
adenomas.
Lymphoma
Small bowel lymphoma is usually a non-Hodgkin’s B cell lymphoma. The two
most common gastrointestinal sites of non-Hodgkin’s lymphoma are the distal small
bowel and the stomach. Proximal gastric lesions may be visualized and biopsied
endoscopically although the lesion originates in the submucosa. In the small bowel,
CT scanning can usually image the lesion if it has attained sufficient size to cause
symptoms. Lymphoma of the bowel is rare in infancy, but the incidence increases
with advancing age peaking in adolescence. Bleeding is the main symptom from
gastric lesions. Small bowel lymphomas cause crampy abdominal pain, and may
Polyps of the Gastrointestinal Tract 235
result in intussusception. Some may erode and perforate into the free abdominal
cavity presenting as gastrointestinal perforation.
Treatment
The treatment of polyps of the GI tract in children can vary from simple obser-
vation of benign lesions to wide excision and chemotherapy for malignant ones.
Juvenile polyps should be removed endoscopically once diagnosed. This is done
to stop the symptoms as well as establish the diagnosis. Most are within easy reach of
a flexible sigmoidoscope and can be snared around the stalk. There is rarely more
than one lesion and removal is curative. Cecal or ascending colonic polyps can be
observed as long as the lesions do not grow and exceed 2 centimeters in diameter.
However, with the widespread use of colonoscopy in pediatrics, endoscopic removal
of these lesions is now the treatment of choice in this previously hard to reach area.
The overall guiding principle in the treatment of Peutz-Jeghers polyps should be
one of bowel conservation. Since the entire gastrointestinal tract can be affected,
excision of all affected areas could easily result in intestinal insufficiency. Regular
surveillance through endoscopy or small bowel contrast studies should be done to
detect polyps which are growing and could be malignant. Large polyps or those
causing significant symptoms should be removed. Endoscopic removal should be
attempted first. For lesions normally beyond the range of normal endoscopy, a com-
bined endoscopic-surgical approach can be attempted where the surgeon at laparo-
tomy guides the endoscopist through the small bowel for visualization and removal
of large lesions. Resection of segments of bowel should be reserved only for cases
where cancer has developed and invaded the submucosa, or in areas where polyps
are particularly dense and causing severe symptoms. Duodenal polyposis may be a
very difficult problem. Although most polyps can be excised endoscopically or sur-
gically, diffuse duodenal involvement with bleeding has been treated with pan-
creaticoduodenectomy.
Lymphomatous lesions are surgically removed. Perforations or areas of intussus-
ception are resected. Radical surgery with lymph node dissection is unnecessary as
all these patients require systemic chemotherapy. Staging with CT scanning and
bone marrow sampling are necessary, and most patients require insertion of subcu-
taneous reservoirs for long-term chemotherapy. The outlook for small bowel lym-
phoma depends on the staging and cellular subtype. Prognosis for this disease has
been steadily improving with the advent of more effective chemotherapeutic regimens. 52
Treatment of children with familial adenomatous polyposis (FAP) involves not
only the individual involved but should also extend to others in the family. Family
members should be screened and referred for genetic counseling and genetic analy-
sis. Definite genetic markers have been identified in this family of disorders, which
may include other syndromes such as Gardner’s syndrome (multiple osteomas,
fibromas, epidermoid cysts). The surgical treatment of FAP requires planning the
process with the family and the patient. It is customary to do a complete colonic and
rectal removal with a sphincter saving operation in early adolescence. This generally
occurs at a time when the patient understands and can participate in treatment
planning and execution.
A Soave type operation (endorectal pullthrough) is the one most commonly
used. The mucosa of the distal rectum is stripped and the terminal ileum is pulled
through. Both straight pull throughs and reservoir operations in the form of a J or S
pull through have been advocated. Patients have attained the size where a stapling
device can be used to construct both the reservoir and perform the lower anastomo-
sis. It is customary to protect the pouch and the anastomosis with a loop ileostomy
which is then closed 4-6 weeks later after all the suture lines have healed and a
contrast study of the pouch has demonstrated no leaks.
The prognosis when the disease is treated in a timely fashion is excellent. Peri-
odic studies to inspect the remaining native anal mucosa is essential for the early
detection of new lesions which can be easily ablated. Genetic counseling is essential
so that all family members can be screened and so that all patients affected by the
disease can consider the risks to their own children.
All other lesions are exceptionally rare in children including the adenomatous
polyp or frank carcinoma. Treatment always includes endoscopic removal whenever
possible, saving surgical resection for cases where this is impractical or contra-indi-
cated (i.e., invasive cancer).
Selected Readings
1. Lelli Jr. JL, Coran AG. Polypoid disease of the gastrointestinal tract. In: O’Neill Jr.
JA et al, eds. Pediatric Surgery, 5th Edition. St. Louis: Mosby 1998; 1283-1296.
2. Gardner EJ, Richards RC. Multiple cutaneous and subcutaneous lesions occurring
simultaneously with hereditary polyposis and osteomatosis. Am J Hum Genet 1953;
5:139.
3. Raffensperger JG. Polyps of the gastrointestinal tract. In: Raffensperger JG ed.
Swenson’s Pediatric Surgery, 5th Edition. Norwalk: Appleton & Lange 1990;
463-472.
52
CHAPTER 1
CHAPTER 53
Peptic Ulcer Disease and Gastritis

Heron E. Rodriguez
Acid-peptic injury to the mucosa of the stomach and duodenum in the form of
inflammation, erosions and ulcerations represents the most common disease affect-
ing the upper gastrointestinal tract. Although reasonably rare in childhood, peptic
ulcers and stress gastritis are still the most common forms of acid peptic disease
(APD).
In children, gastroduodenal ulcers can be classified as either primary or second-
ary. Primary ulcerations are the result of an intrinsic ulcer diathesis. Secondary ulcers,
also called stress ulcers, are related to critical illness, sepsis, shock or major physical
or thermal injury. Among these, two types of ulcers have been given eponyms. The
“Cushing ulcer” occurs in association with head injury, encephalopathy, or major
surgery. The “Curling ulcer” occurs after extensive burns.
Incidence
The true incidence of APD in children is unknown. Estimates from the pre-
endoscopic era suggest that there are 3.5-14.7 new cases per 100,000 children each
year. Contrary to the trend in adults, the incidence of APD in children appears to be
increasing. Although this may be in part related to the increased use of ulcerogenic
drugs and the increase in the number of children surviving major illnesses and trauma,
it appears that the true prevalence of APD is increasing. Overall the incidence in
boys is 2-3 times higher than in girls, but an equal sex incidence is noted in infants
and very young children. Of children presenting with ulcer disease, 33-56% will
have first and/or second-degree relatives with APD.
Pathophysiology
The process of peptic ulceration results from the offensive interaction of hydro-
chloric acid, pepsin, and the defensive cellular mechanisms attempting to preserve
the integrity of the gastroduodenal mucosa. A causal relationship between APD and
Helicobacter pylori has been established.
Basal acid output in children with APD is not significantly different than that of
control subjects. Although acid and pepsin are necessary for the development of
ulcers, acid hypersecretion is only rarely the cause of APD. The Zollinger-ellison
syndrome (hypergastrinemia secondary to gastrinoma) is exceptionally rare in chil-
dren. The increased frequency of APD in children with chronic renal failure is
attributed to elevated gastrin levels. G-cell hyperplasia, systemic mastocytosis and
hyperparathyroidism (also seen with chronic renal failure) are rare conditions asso-
ciated with increased hyperacidity.
The most common mechanism for ulcer formation involves a decrease in the
ability of the mucosa to protect itself against acid injury. Normally, a thick mucus
layer in the surface of the stomach provides an effective barrier to the action of acid.
In addition, continuous cell turnover in the gastric mucosa assures epithelial integ-
rity and cell regeneration after injury. Cholinergic agonists, prostaglandins, and
cytokines stimulate the release of mucus and are protective. However, mucosal is-
chemia reduces mucus production increasing the ability of hydrogen ions to diffuse
back into the mucosa. A low arterial pH of the blood supplying the stomach may
also impair the ability of the mucosa to protect itself. The effects of low perfusion
states, steroids, and anti-inflammatory medications impair all of these defense mecha-
nisms, leaving the mucosa prone to acid injury. Inadequate gastric emptying may
also promote mucosal injury by increasing the time that the mucosa is exposed to
acid.
Helicobacter pylori is a fastidious, spiral-shaped, gram-negative rod whose redis-
covery has changed traditional concepts about the pathogenesis and treatment of
APD. H. pylori gastritis is the most common cause of chronic gastritis in children.
Nearly all patients with duodenal ulcers have H. pylori gastritis. About 85% of patients
with gastric ulcers are infected with H. pylori. If acid suppression is used as the only
form of treatment, a relapse rate of 90% per year can be expected. When H. pylori is
eradicated with antibiotics, the relapse rate falls to 10% annually. Interestingly, the
majority of individuals infected by H. pylori develop neither gastritis nor ulcer dis-
ease. Other cofactors are clearly important for development of peptic ulcer disease.
The symptoms of gastritis, peptic ulcer and duodenitis are similar and nonspe-
cific. In the first month of life, perforation and bleeding are the usual presentations.
Up to two years of age, the major symptoms are:
1. recurrent vomiting,
2. refusal of feedings,
3. persistent crying,
4. slow growth, and
5. gastrointestinal hemorrhage.
In preschool children, postprandial pain, vomiting and hemorrhage are the com-
mon presenting features. In older children, the presentation is similar to that of
53 adults. The pain of peptic disease is often vague and difficult for young patients to
describe. It may be temporally related to meals or relieved by eating. Nocturnal
awakening caused by episodes of pain is a common feature and may differentiate
organic from psychogenic pain. Anorexia, nausea, early satiety, eructation and vom-
iting all are common symptoms.
APD is confirmed in only 15-20% of all children presenting with symptoms of
dyspepsia. Psychogenic abdominal pain accounts for the vast majority of cases. The
differential diagnosis of dyspepsia in children with APD includes esophagitis, chole-
cystitis, liver disease, pancreatitis, and infectious gastroenteritis. In children present-
ing with upper gastrointestinal hemorrhage (i.e., hematemesis), the differential
Peptic Ulcer Disease and Gastritis 239
diagnosis should include APD, esophagitis, Mallory-Weis tear, esophageal or gastric

varices, and other causes of gastrointestinal hemorrhage.
Diagnosis
Barium upper gastrointestinal radiography (UGI) has been extensively used in
the evaluation of APD. It only detects 75% of duodenal ulcers and fewer than 40%
of gastric ulcers in children. In addition to its low sensitivity and specificity in iden-
tifying ulcers, another important disadvantage in the evaluation of suspected APD
is the inability of UGI to detect the presence of H. pylori. UGI is useful in the
evaluation of early satiety and vomiting when obstructive conditions such as malro-
tation or gastric outlet obstruction need to be ruled out.
Upper GI flexible endoscopy offers several advantages in the evaluation of APD.
The presence of ulcers can be determined or excluded with great accuracy. Direct
visualization or biopsies of the affected areas of the mucosa can determine the cause
of ulcer disease and gastritis. With endoscopic biopsy, H. pylori can be identified by
histologic study, rapid urease testing, or tissue culture. Alternatively, H. pylori infec-
tion can be identified by the serum urease test, the carbon-labeled urea breath test,
and H. pylori-specific serum immunoglobulin G (IgG) measurement. Finally, endo-
scopic methods (i.e., electrocautery, sclerotherapy) provide therapeutic means to
obtain hemostasis in cases of significant, acute upper gastrointestinal hemorrhage.
Medical Treatment
Antacids effectively heal peptic ulcers when compared with placebo (75% com-
pared with 40%). Nevertheless, compliance is disappointing and the side effects of
diarrhea and constipation are not uncommon. The dominant pathway of parietal
cell activation is paracrine stimulation of the histamine-2 (H2) receptor by hista-
mine. H2 receptor antagonists (i.e., cimetidine, ranitidine, famotidine) inhibit pari-
etal cell responses to all secretagogues and are the main form of therapy for APD. H2
antagonists are effective agents against APD, with relapse rates of less than 20%.
Other medical treatment options include omeprazole, sucralfate, and prostag-
landin therapies. Omeprazole inhibits gastric secretion at the final common path-
way, the H+–K+ adenosine triphosphatase pump. It is effective in healing ulcers in
95% of patients within 4 weeks. Sucralfate forms a protective coat on the gastric
mucosa. The negative charge of the sulfated disaccharide aluminum salt of sucralfate
adheres to the positive protein charge of the injured gastric mucosa. Sucralfate also
seems to enhance mucosal microvascular flow, mucus production, and prostaglan-
din secretion. Prostaglandin replacement (misoprostol) has been shown to be effec- 53
tive in the prevention and treatment of ulcers associated with the use of nonsteroidal
anti-inflammatory drugs (NSAIDs).
Eradication of H. pylori substantially reduces ulcer recurrence. Amoxicillin and
metronidazole, usually in combination with bismuth, are added to conventional
therapy for 2 weeks. Because ulcers tend to recur, follow-up endoscopy to prove
healing and eradication of H. pylori is considered.
Surgical Treatment
Children with primary APD have a high rate of recurrence throughout child-
hood and into adult life. In the past, early surgical intervention for these children
has been recommended. At present, surgery is limited almost exclusively to the treat-
ment of APD complications.
In the majority of cases, bleeding responds well to nasogastric decompression,
volume replacement and transfusion therapy. For major or recurrent bleeding,
endoscopic treatment modalities are available. These include therapeutic injections
(i.e., hypertonic NaCl, epinephrine, absolute ethanol) and cauterization with heater
probe, bipolar coagulator or laser. If medical and endoscopic treatments fail, surgery
is indicated. Acute perforation or gastric outlet obstruction are both complications
of APD that require surgical intervention.
The surgical procedures used to treat peptic ulcer disease include:
1. simple closure of a localized perforation,
2. gastrotomy with oversewing of the base of a bleeding ulcer,
3. partial and subtotal gastrectomies,
4. vagotomy with pyloroplasty, and
5. highly-selective vagotomy.
Vagotomy and pyloroplasty is the traditional approach that provides good long-
term results causing minimal disturbance of growth and development. The choice
of the operation used to treat APD should be individualized, taking into account
the likelihood of recurrence, the comorbid factors, and the nutritional and develop-
mental needs of the growing child.
Selected Readings
1. Scherer III LR. Peptic ulcer and other conditions of the stomach. In: O’Neill Jr. JA
et al, eds. Pediatric Surgery, 5th edition. St. Louis: Mosby 1998; 1119-1125.
2. Raffensperger JG. Stress bleeding and peptic ulcer. In: Raffensperger JG, ed.
Swenson’s Pediatric Surgery, 5th edition. Norwalk: Appleton & Lange 1991;
473-477.
3. Mezoff AG, Balistreri WF. Peptic ulcer disease in children. Pediatrics in Review
1995; 16:257.
53
CHAPTER 1
CHAPTER 54
Portal Hypertension
Kimberly Brown
Anatomy and Physiology
The portal venous system drains blood from the stomach, pancreas, gallbladder,
spleen, and intestines into the liver. The portal vein arises in the embryo as the left
and right vitelline veins, which form numerous anastomoses among the developing
hepatocytes. Following gut rotation, the left vitelline vein is obliterated, and the
right vitelline vein persists as the main portal vein. Portosystemic anastomoses exist
in four main areas,
1. the gastro-esophageal veins via the cardiac vein and perforating esoph-
ageal veins,
2. the retroperitoneum via the pancreaticoduodenal veins and the retroperi-
toneal-paravertebral veins,
3. gastrorenal-splenorenal vein, and
4. the hemorrhoidal plexus.
The portal venous system lacks valves, which means the flow of blood is entirely
dependent upon the pressure gradient within the system. Normal flow toward the
liver is termed hepatopedal, while significantly increased portal venous resistance
can result in hepatofugal flow away from the liver. Normal portal venous pressure is
5-10 mm Hg greater than central venous pressure. Portal hypertension is defined as
elevation of the portal venous-IVC pressure gradient above 10-12 mm Hg.
Etiology
Portal hypertension in children can be divided into two major categories based
upon the anatomic location of the increased portal resistance. Extrahepatic portal
hypertension (EHPH) is most commonly the result of portal vein obstruction due
to thrombosis. Risk factors for portal vein thrombosis include umbilical vein cath-
eterization, neonatal sepsis, blunt abdominal trauma, and omphalitis; idiopathic
cases are also reported. The thrombosis frequently recanalizes which results in a
cavernous transformation of the portal vein into numerous smaller channels.
Intrahepatic portal hypertension (IHPH) is typically associated with congenital
liver or biliary diseases in children. Biliary atresia is by far the most common cause
of IHPH in children. Other etiologies include sclerosing cholangitis, familial
cholestatic syndromes, cystic fibrosis, α-1 antitrypsin deficiency, hemochromatosis,
Wilson’s disease, viral hepatitis, autoimmune hepatitis, or idiopathic neonatal hepa-
titis. The manifestations of liver disease appear long before the sequelae of portal
hypertension in most of these children. Congenital hepatic fibrosis (CHF) is a rare

cause of intrahepatic portal hypertension in children. Hepatocyte function is nor-
mal in livers affected by CHF.
Incidence
Liver disease in children is relatively rare, with an incidence of 1 in 5000-7000.
EHPH is approximately twice as common as IHPH in children. The most common
cause of IHPH, biliary atresia, occurs at a rate of 1 in 15,000 live births. This distri-
bution is distinctly opposite that seen in adults, who most commonly develop intra-
hepatic portal hypertension as a result of alcoholic cirrhosis.
Children with IHPH usually present between several months to one year of life
with severe hepatic dysfunction, manifested by jaundice, hepatic encephalopathy,
and malnutrition complicated by poor growth and increased susceptibility to infec-
tions. While the child’s liver disease may dominate the clinical picture, it is impor-
tant to remember that portal hypertension is present in these patients and to be
aware of its potential complications.
Extrahepatic portal hypertension most commonly presents in the first decade of
life with gastrointestinal hemorrhage from esophageal varices. Bleeding is often pre-
cipitated by a respiratory or gastrointestinal febrile illness, and aspirin is frequently
implicated. Increased portal pressure causes splenic congestion, resulting in sple-
nomegaly and hypersplenism. Portal hypertension, therefore, is suspected in any
child with splenomegaly, unexplained thrombocytopenia, leukopenia, ascites or gas-
trointestinal hemorrhage.
Diagnosis
In a child presenting with an initial episode of gastrointestinal bleeding, abdominal
ultrasonography is used to confirm the etiology. The presence of portal vein throm-
bosis, the extent of collateral formation, and the direction of portal vein flow is
established by this noninvasive and relatively inexpensive diagnostic exam. Like-
wise, in a child with evidence of chronic liver disease, ultrasonography confirms the
presence of portal hypertension and evaluates the liver for abnormalities such as
nodular cirrhosis, fibrosis, or ductal dilatation. The presence of spontaneous porto-
systemic shunts and esophageal varices as indicated by thickened vessels in the lesser
omentum is also documented.
Upper endoscopy is used to identify and quantitate esophageal varices. This pro-
cedure is ideally performed in the operating room under general anesthesia to pro-
vide a controlled environment for a thorough study and possible therapeutic
54 intervention (i.e., sclerotherapy, banding). Documentation of varices includes size,
location and presence of cherry red spots or red walls. Identification of other poten-
tial sites of bleeding is also important, as many children with portal hypertension
also have gastric varices, peptic ulcer disease, esophagitis, or a portal hypertensive
gastropathy or enteropathy.
Angiography is a much more rarely used diagnostic and potentially therapeutic
modality used in certain cases of portal hypertension. The venous phase of a celiac
axis injection demonstrates the anatomy of the portal venous system, while a
Portal Hypertension 243
percutaneous transhepatic approach allows for direct entry into the portal vein and
therapeutic dilation of portal vein strictures especially liver transplant patients.
Treatment
The most common, clinically significant complication of portal hypertension is
gastrointestinal bleeding from esophageal varices. In the acute setting, massive hem-
orrhage is managed with intensive care monitoring, transfusion of red blood cells
and fresh frozen plasma, and potentially intubation and sedation to minimize agita-
tion that increases variceal pressure. Octreotide infusions effectively control acute
hemorrhage in the vast majority of children with portal hypertension and variceal
bleeding. Endoscopic variceal banding or sclerotherapy is occasionally used in cases
where hemorrhage does not resolve with supportive care. Once the patient has stabi-
lized, endoscopy with sclerotherapy or banding is employed to prevent repeat epi-
sodes of hemorrhage.
Gastrointestinal hemorrhage in children with extrahepatic portal hypertension
tends to be less severe than bleeding that occurs in children with IHPH due to the
presence of a normally functioning liver. In addition to coagulation abnormalities,
many patients with IHPH also have significant malnutrition which contributes to
the greater morbidity and mortality of gastrointestinal bleeds in this group.
Medical treatment of portal hypertension is aimed at relieving the pressure in
the portal system. Pharmacotherapy, consisting primarily of β-blockade, reduces
cardiac output and therefore portal venous pressure. Octreotide, a long-acting syn-
thetic somatostain analogue, may also be used to decrease blood flow in the portal
and azygous veins, as well as to reduce collateral blood flow.
Surgical treatment of portal hypertension can be either direct, which involves
ligation of the varices themselves, or indirect, in which the portal venous system is
decompressed with a surgical shunt. A nonselective portosystemic shunt diverts the
majority of portal blood to the caval system, causing a higher incidence of hepatic
encephalopathy. Examples include portocaval, mesocaval and central splenorenal
shunts. Selective portosystemic shunts shunt a portion of portal blood into the sys-
temic circulation, with distal splenorenal shunt being the most common. Recently,
selected children with EHPH due to portal vein thrombosis have been successfully
treated by surgical creation of a mesenterico-portal venous bypass (Rex shunt).
Recurrent bleeding, shunt thrombosis and hepatic encephalopathy are the most com-
mon complications of surgical shunting.
Treatment of intrahepatic portal hypertension focuses on the primary liver dis-
ease. In biliary atresia (Chapter 67), surgery to decompress the biliary tract is ideally
performed within the first three months of life. In the Kasai procedure, the atretic
segments of the extrahepatic bile ducts are excised and a Roux loop is anastomosed
to the porta hepatis. If performed early, before significant liver injury and cirrhosis,
54
the Kasai procedure can delay liver failure and the need for transplantation in as
many as two thirds of patients. For advanced cirrhosis and other intrahepatic sources
of portal hypertension, liver transplantation is the definitive treatment. Consequently,
surgical interventions prior to transplant should not incur significant risk to the
patient, nor should they interfere with the ability to perform a subsequent liver
transplant in the patient.
Outcome
Patients with biliary atresia experience jaundice, poor nutrition and rapidly
declining liver function due to biliary cirrhosis. Currently, if the biliary tract is diverted
before 3 months of age, one third of these patients will survive without requiring
transplant, while one third will survive to require transplant in childhood and the
remainder will die from liver failure.
For patients with EHPH, sclerotherapy is effective in the treatment of acute
variceal bleeding in up to 75% of patients. However, several follow-up sessions are
necessary to obliterate the varices, and a rebleeding rate of 5-25% is expected. Per-
sistent variceal bleeding as well as hypersplenism may require a surgical shunt. Selec-
tive shunts have proven successful for the control of bleeding, thrombocytopenia,
and leukopenia, without creating great risk of encephalopathy.
Selected Readings
1. Bambini DA, Superina RA, Almond PS et al. Experience with the Rex shunt in
children with extrahepatic portal hypertension. J Pediatr Surg 2000; 35:13-19.
2. Shilyansky J, Roberts EA, Superina RA. Distal splenorenal shunts for the treat-
ment of severe thrombocytopenia from portal hypertension in children. J
Gastrointest Surg 1993; 3:167-172.
3. Alonso EM, Hackworth C, Whitington PF. Portal hypertension in children. Clin-
ics in Liver Disease 1997 May; 1(1):201-221.
4. Watanabe FD, Rosenthal P. Portal hypertension in children. Curr Opin Pediat
1995; 7:533-538.
5. Hassall E. Nonsurgical treatments for portal hypertension in children. Gastrointes-
tinal Endoscopy Clinics of North America 1994; 4(1):223-258.
6. Rowe MI, O’Neill JA Jr., Grosfeld JL et al. Essentials of pediatric surgery. St. Louis:
Mosby-Year Book Inc. 1995; 1075-1086.
7. Raffensperger JG. Swenson’s Pediatric Surgery, 4th edition. New York: Appleton-
Century-Crofts 1980; 443-452.
54
CHAPTER 1
CHAPTER 55
Meckel’s Diverticulum
Richard Fox
Incidence
Meckel’s diverticulum is the most common gastrointestinal tract anomaly. It
occurs with a worldwide prevalence of approximately 2-4%. For the majority of
patients, this remnant remains clinically silent throughout life. Its presence is not
suspected unless complications arise or it is identified as an incidental finding at
autopsy or laparotomy. In asymptomatic patients, there is no gender predilection.
However, in symptomatic patients, males are affected approximately 2-4 times more
frequently than females. Meckel’s diverticulum is known as the disease of “two’s.”
Two percent of the population is affected, it occurs twice as frequent in males, it is
located two feet from the ileocecal valve, it is two inches long and two centimeters
wide, and two types of mucosa often exist. Furthermore, the majority of symptom-
atic patients present by two years of age.
Etiology
During the third week of fetal development, the midgut opens into the yolk sac,
remaining temporarily connected by the vitelline duct (or yolk stalk). This duct
divides the cranial and caudal portions of the midgut (distal duodenum, jejunum
and proximal ileum, distal ileum and cecum, appendix, ascending colon, and proxi-
mal two-thirds of the transverse colon, respectively.) As intestinal maturation pro-
ceeds, the vitelline duct narrows. By the third month of embryogenesis, the duct
disappears as the physiologic intestinal herniation spontaneously reduces. If com-
plete regression fails to occur, an omphalomesenteric duct-related anomaly results
(Chapter 22). The anomaly that results is solely dependent upon the stage of devel-
opmental arrest.
A complete diverticulum, the most clinically significant anomaly, extends directly
from the antimesenteric border of the ileum. In approximately 75% of patients, the
tip remains free and unattached within the abdominal cavity. In the remaining 25%,
the Meckel’s diverticulum remains attached to the anterior abdominal wall. The
connection consists of either a solid fibrous cord (arterial or ductal remnant) or
omphalo-ileal fistula extending from the umbilicus to the ileum. Vitelline duct cysts
result if a cyst forms in the midportion of the cord (Chapter 22).
The yolk sac is initially supplied by the paired vitelline arteries. As fetal matura-
tion proceeds, the left vitelline artery disappears, leaving the right vitelline artery to
form the superior mesenteric artery. Meckel’s diverticulum is commonly supplied
from a branch arising from the ileal artery. Infrequently, the vascular supply arises
directly from an ileocolic arterial branch or from the mesentery directly. The artery
is always an end-artery.
Symptoms are usually absent unless complications occur. The most commonly
encountered complications of Meckel’s diverticulum are bleeding, obstruction, or
inflammation (with or without perforation). Meckel’s diverticuli occasionally iden-
tified in association with symptoms of umbilical fistulas or hernia. Infrequently,
tumors can develop in Meckel’s diverticuli. Carcinoid is the most common tumor,
though adenocarcinoma, leiomyoma, lymphoma occur as well. The incidence of
complications diminishes with increasing age.
Bleeding typically occurs in children less than five years of age, with two being
the average age. Meckel’s diverticulum is the most common cause of a massive pedi-
atric lower gastrointestinal hemorrhage. Bleeding is characteristically episodic and
painless. It recurs frequently and is often severe enough to require multiple blood
transfusions for resuscitation. However, some children may present only with mel-
ena and mild anemia, corresponding with a slower, more controlled blood loss.
Bleeding almost always occurs at the junction between normal ileal mucosa and the
heterotopic (usually gastric or pancreatic) diverticular mucosa. Less common sites
include the tip of the diverticulum or the mesenteric border of the ileum. Bleeding
is usually secondary to peptic ulceration caused by hydrochloric acid secreted by
heterotopic parietal cells. The differential diagnosis includes juvenile polyps,
inflammatory bowel disease, peptic ulcer disease of the stomach or duodenum, and
blood dyscrasias.
Obstruction occurs from intussusception, herniation, kinking or volvulus.
Intussusception is the etiology in nearly 50% of cases. It commonly presents before
ten years of age. The diverticulum may act as the leadpoint causing an ileo-ileal
intussusception with rapid progression to an ileo-colic presentation. The patient
presents with early vomiting, abdominal pain, and oftentimes, a palpable abdomi-
nal mass. If untreated, intestinal vascular compromise and ischemic necrosis can
occur. Volvulus is the second most common mechanism of obstruction and occurs
in 25% of cases. Obstruction can also occur secondary to internal herniation around
an aberrant right vitelline artery or fibrous band. Intestinal prolapse through a patent
vitelline duct also causes intestinal obstruction. Infrequently, obstruction occurs when
a Meckel’s diverticulum protrudes through an indirect inguinal hernia (Littre’s her-
nia). Most obstructions due to Meckel’s diverticuli are not diagnosed until laparotomy.
Meckel’s diverticulitis presents similar to acute appendicitis. The diagnosis is
rarely made prior to laparotomy. Obstruction of the lumen of the diverticulum
occurs secondary to any number of objects, ranging from a fecalith, parasite or even
an ingested fish bone. Inflammation follows obstruction. Pressure builds within the
closed-off diverticulum which eventually compromises venous outflow. Arterial inflow
55 is diminished, bacterial invasion ensues, and gangrenous changes in the diverticular
wall ultimately lead to perforation. Unlike perforated appendicitis, Meckel’s diver-
ticulum is more likely to present with free air and diffuse peritonitis because of its
precarious location away from the lateral abdominal wall. Pain symptoms are very
similar to those of acute appendicitis. Periumbilical pain occurs secondary to hollow
Meckel’s Diverticulum 247
Fig. 55.1. Meckel’s diverticulum in the usual antimesenteric position with aberrant
tissue in the distal tip responsible for symptoms.
viscus distension. Somatic pain localization depends upon the location of the diver-
ticulum and the surrounding peritonitis.
Diagnosis
Prior to the advent of the radio-labeled technetium scan in 1970, children with
massive lower gastrointestinal bleeding were presumed to have Meckel’s diverticu-
lum and taken for immediate laparotomy. Currently the Meckel’s scan has a false
negative rate of 1.7%, false positive rate of 0.05%, sensitivity of 85% and specificity
of 95%. To maximize sensitivity, three agents are available. Pentagastrin is used to
facilitate technetium uptake in gastric mucosal cells. H2-receptor blockers, such as
cimetidine, are used to reduce the gastric cellular excretion of technetium. Gluca-
gon may be used to inhibit intestinal peristalsis which lengthens the cells exposure
to the labeled technetium maximizing the time for uptake. A bladder catheter is
inserted prior to scanning to evacuate technetium excreted in the urine and to simplify
radiographic interpretation. False positive scans result from intussusception, hydro-
nephrosis, arteriovenous malformation, gastrointestinal duplication, inflammatory
bowel disease, neoplasms and other intestinal areas containing heterotopic gastric
mucosa.
Pathology/Pathophysiology 55
Meckel’s diverticulum is a true diverticulum. It contains all three normal bowel
layers: mucosa, submucosa, and muscularis propria. Vitelline duct cells are
pluripotential. As a result, 50% of all diverticula contain heterotopic gastric mucosa.
If symptomatic, the prevalence is closer to 75%. Heterotopic pancreatic tissue is
present in only 5%. Five percent contain both gastric and pancreatic tissue. Hetero-
topic colonic or enteric mucosa occur infrequently (i.e., 2% each). Many ulcers are
nonapparant on inspection and are thus best detected on microscopy. Of note, un-
like peptic ulceration of the stomach and duodenum (Chapter 55), ileal ulcers from
a Meckel’s diverticulum are not associated with Helicobacter pylori.
Treatment
Treatment of a symptomatic diverticulum requires operative intervention. Irre-
spective of the etiology, appropriate preoperative resuscitation with intravenous flu-
ids or blood products is paramount. For bleeding or diverticulitis, a right lower
quadrant incision is usually performed. If necessary, medial extension of the wound
allows greater exposure. The cecum is identified and followed proximally toward the
ileum. The diverticulum is noted, the arterial supply is identified and ligated, and
the diverticulum is removed. Surgical options include simple diverticulectomy with-
out ileal resection, a wedge resection to include immediately surrounding ileum, or
an ileal resection with primary anastomosis.
For cases presenting as intussusception, an initial barium enema is attempted for
immediate hydrostatic reduction. Diverticulectomy usually follows at a later date
unless intestinal ischemia is suspected which requires immediate laparotomy.
For patients who present with intestinal obstruction, rarely is the diagnosis of a
Meckel’s diverticulum made preoperatively. Accordingly, a midline laparotomy inci-
sion may be appropriate. This affords the most versatility in decompression and
potential resection.
For the myriad of persistent vitelline duct anomalies (Chapter 22), transverse
supraumbilical or infraumbilical incisions are appropriate. The anomaly is identi-
fied, careful dissection proceeds to identify and remove all involved structures.
The best management strategy for the asymptomatic or incidentally encoun-
tered Meckel’s diverticulum remains controversial. Excision is prudent in patients
with palpable heterotopic mucosa, obvious inflammation, abdominal wall attach-
ments, or unexplained abdominal pain. Other conditions require individual judge-
ment.
Outcomes
Upon resection of a Meckel’s diverticulum, cure from subsequent bleeding or
inflammation is expected. The most common postoperative complication is adhe-
sive small bowel obstruction which occurs in 5-10% of patients.
Selected Readings
1. Amoury RA, Snyder CL. Meckel’s diverticulum. In: O’Neill Jr. JA et al, eds. Pedi-
atric Surgery, 5th Edition. St. Louis; Mosby 1998; 1173-1184.
2. Mackey WC, Dineen PA: Fifty years experience with Meckel’s diverticulum. Surg
Gynecol Obstet 1983; 156:56.
55 3. St. Vil D et al. Meckel’s diverticulum in children: A 20-year review. J Pediatr Surg
1991; 26:1289.
4. Jewett TC, Duszynski DO, Allen JE. The visualization of Meckel’s diverticulum
with 99m-Tc-pertechnetate. Surgery 1970; 65:567.
5. Kusumoto H et al. Complications and diagnosis of Meckel’s diverticulum in 776
patients. Am J Surg 1992; 164:382.
Section VII: Anomalies
of the Gastrointestinal Tract
CHAPTER 56
Intestinal Obstruction in the Neonate

Daniel A. Bambini
Incidence
The overall incidence of neonatal intestinal obstruction is difficult to estimate
because it may result from such a variety of embryonic anomalies and functional
abnormalities. However, intestinal obstruction is the most common surgical emer-
gency of the newborn. The incidence of neonatal intestinal obstruction is approxi-
mately 1 case per every 500-1000 live births. Approximately 50% of these neonates
will have intestinal atresia or stenosis. Duodenal atresia and jejunal atresia occur in
approximately equal numbers, although some authors report that jejunolileal atresia
is the more common.
Etiology
Intestinal obstruction may be caused by several conditions in the neonate
(Table 56.1). The specific etiology of each condition is described in the appropriate
Chapter of the book devoted to that problem.
The majority of neonates with intestinal obstruction present shortly after birth,
yet prenatal diagnosis of obstructive gastrointestinal lesions is possible in selected
patients. Proximal obstructing lesions can produce proximal bowel dilation with
hyperperistalsis that is readily identifiable by prenatal ultrasonography. The classic
“double bubble” appearance of duodenal atresia can be identified in utero with
ultrasonography. Distal intestinal obstructions are less likely to cause polyhydram-
nios but on occasion dilated loops of bowel may be identified as anechoic masses. In
cases of meconium ileus, dilated loops of bowel filled with echogenic meconium
may be identified.
Five clinical findings suggest intestinal obstruction in the neonate: maternal poly-
hydramnios, excessive gastric aspirant, abdominal distention, bilious vomiting, and
obstipation. The presence or absence of each of these clinical findings depends largely
upon the level of gastrointestinal obstruction. Early recognition of intestinal ob-
struction is imperative if the complications of respiratory compromise and sepsis are
to be avoided.
Maternal polyhydramnios: Amniotic fluid is continuously ingested and ab-
sorbed within the intestine of the fetus. The ingested fluid is transferred to the
maternal circulation via the placenta to be excreted in the mother’s urine. Proximal
gastrointestinal obstruction interrupts this process and leads to accumulation of
Intestinal Obstruction in the Neonate 251
Table 56.1. Causes of neonatal intestinal obstruction
Common:
Malrotation (duodenal obstruction, volvulus, internal hernia)
Duodenal atresia, stenosis or annular pancreas
Jejunal atresia or stenosis
Ileal atresia or stenosis
Simple meconium ileus
Meconium ileus with perforation
Meconium plug syndrome
Hirschsprung’s disease
Drug-induced ileus
Hypertrophic pyloric stenosis
Uncommon:
Pyloric atresia or web
Tumors
Intussusception
Segmental intestinal dilatation
Small left colon syndrome
Milk bolus obstruction
Colonic atresia
Functional Intestinal obstruction
Intestinal Psuedo-Obstruction
Neuronal Intestinal Dysplasia
Megalocystis-Microcolon-Intestinal Hypoperistalsis Syndrome
Inguinal hernia
excess amniotic fluid. Distal small bowel or colonic obstructions do not usually
result in polyhydramnios.
Excessive gastric output: Passage of a nasogastric or orogastric tube is often
performed in premature infants and infants with a maternal history of polyhydram-
nios. If the initial volume of gastric aspirant is large (> 50 cc) or is bilious then
gastrointestinal obstruction should be considered.
Abdominal distention: Abdominal distension may not be apparent at birth but
develops over time as ingested air accumulates proximal to an obstruction. The time
of onset, degree and characteristic appearance of the distention may suggest the level
of obstruction. Gastric distention within a few hours may cause the epigastrium to
protrude indicating an obstruction of the stomach or duodenum. Gradual overall
abdominal distension occurring over a 12-24 hour period suggests a distal gastrointes-
tinal tract obstruction.
Bilious emesis: It is usual for healthy newborns to spit-up postprandially, but
bilious emesis in a term newborn is distinctly abnormal. Premature infants (< 35
weeks) occasionally have bilious emesis secondary to an immature or poorly func-
tioning pyloric sphincter but proximal gastrointestinal obstruction must still be con-
sidered. Sepsis with an associated paralytic ileus may also result in bilious emesis.
Vomiting begins soon after delivery if the lesion is proximal or complete, but may be
delayed in cases of distal or incomplete obstruction. 56
Failure to pass meconium: A normal newborn is expected to pass a large amount
of thick, dark green, shiny meconium usually within the first twelve hours of life
and almost always by 24 hours. Failed or delayed passage of meconium suggests

obstruction, but neonates with proximal obstructing lesions may pass a normal
amount of meconium. Because neonates with ileal atresia or distal small bowel
obstruction may pass several meconium stools on the first day of life, passage of
meconium does not exclude the possibility of obstruction. Preterm infants com-
monly have delayed passage of meconium. Approximately 20% will not pass stool
during the first 48 hours following birth.
Additional physical findings that suggest obstruction are the presence of intesti-
nal patterning and peristalsis that is visible through the intestinal wall. Distended
loops of bowel may be palpable as ill-defined tubular masses. Masses that feel hard
and with a “doughy” consistency can be felt especially in cases of meconium ileus.
The rectum may feel tight on exam if small and unused as in cases of distal bowel
obstruction. Lethargy and hypotonia are late signs of intestinal obstruction and the
resultant sepsis. Abdominal wall discoloration and ecchymosis suggest perforation
and/or necrosis.
Diagnosis
The diagnosis of neonatal intestinal obstruction is largely made on clinical
grounds. Flat and upright abdominal radiographs are obtained to confirm the diag-
nosis. Swallowed air serves as the contrast media to help delineate the level of
obstruction. A normal neonate swallows air from birth and has air within the proxi-
mal small bowel within 30 minutes. Air usually reaches the colon by 3-4 hours and
can be identified in the rectum by 6-8 hours. There should be no air-fluid levels in
an upright film of a normal newborn.
Specific radiograph abnormalities of the lesions causing obstruction are described
in other Chapters, however, when a complete obstruction exists the air pattern may
stop abruptly leaving the remainder of the bowel airless. Bowel loops proximal to
complete obstruction are dilated. Multiple dilated loops of bowel with “stepladder”
air fluid levels on the upright film is the pattern most often seen with distal intesti-
nal obstruction. However, air-fluid levels are not characteristic of the distal intesti-
nal obstruction due to meconium ileus. Partial obstructions as in stenosis may allow
small amounts of air to pass beyond the level of obstruction, but the paucity of
bowel gas in the bowel distal to dilated bowel segments can easily be identified as
abnormal. The abdominal films should always be inspected for peritoneal and/or
scrotal calcifications which may signify an intrauterine perforation with meconium
peritonitis.
Barium or gastrograffin contrast enema examination may be useful to distin-
guish between causes of distal bowel obstruction (ileal atresia, meconium ileus,
Hirschsprung’s, meconium plug, etc.) and in cases of meconium ileus or plug may
also be therapeutic. Upper gastrointestinal barium studies are generally not as useful
unless one is seeking patterns of abnormal bowel rotation. UGI contrast studies are
reserved for cases of partial obstruction that cannot be confirmed by plain radiographs.
56 Proximal intestinal obstruction as in cases of duodenal or pyloric atresia leads to
fluid loss that has a high concentration of hydrogen, potassium and chloride ions.
Hypochloremic alkalosis can develop if fluid losses are not replaced. Distal intestinal
Intestinal Obstruction in the Neonate 253
obstructions lead to fluid and electrolyte loss from both emesis as well as from fluid
sequestered into the lumen of dilated bowel loops. Fluid shifts and intravascular
volume depletion may lead to severe dehydration, oliguria, metabolic acidosis, and
inadequate peripheral perfusion. Prolonged intestinal obstruction leads to alterations
in intestinal motility, accumulation of gas and fluid, and bacterial overgrowth. Severe
abdominal distention in the neonate can easily impair diaphragmatic function caus-
ing respiratory acidosis. As plasma volume loss increases, alterations in blood flow
may result in bowel ischemia and necrosis.
Classification systems for specific obstructing lesions of the gastrointestinal tract
are presented in other Chapters of this book. Please refer to specific lesions for details.
Treatment
The specific management strategies and surgical considerations for the various
conditions causing intestinal obstruction in the neonate are described in other Chap-
ters of this book. However, the initial treatment of any suspected neonatal obstruc-
tion includes placement of a nasogastric tube to decompress the stomach and to
prevent vomiting/aspiration. Fluid and electrolyte replacement should be quickly
undertaken to resuscitate the infant and restore circulating blood volume in antici-
pation of the potential need for surgical intervention. Most obstructive lesions in
neonates will require surgical therapy and surgery should not be delayed once vol-
ume resuscitation is adequate. If an intestinal anastamosis is anticipated periopera-
tive antibiotics are indicated.
Outcomes
The outcomes from neonatal intestinal obstruction vary with the etiology of the
obstruction. Overall survival is generally good but often is influenced by the associ-
ated anomalies of each condition. Please refer to the appropriate Chapters for dis-
ease specific treatment results.
Selected Readings
1. Raffensperger JG, Seeler RA, Moncada R. Intestinal obstruction in the newborn.
In: Raffensperger JG et al, eds. The Acute Abdomen in Infancy and Childhood.
Philadelphia: JB Lippincott Company 1970; 1-19.
2. Haller, Jr. JA, Talbert JL: Gastrointestinal Emergencies. In: Haller, Jr JA, Talbert
JL, eds. Surgical Emergencies in the Newborn. Philadelphia: Lea & Febiger 1972;
86-111.
3. Filston HC. Other causes of intestinal obstruction. In: O’Neill, Jr. JA et al, eds.
Pediatric Surgery, 5th Edition. St. Louis: Mosby 1998; 1215-1221.
56
CHAPTER 57
Pyloric and Duodenal Obstruction

Daniel A. Bambini
Congenital obstructing lesions of the pylorus and duodenum include pyloric
atresia, duodenal atresia and stenosis, and annular pancreas. Hypertrophic pyloric
stenosis and malrotation, also present as proximal gastrointestinal obstruction and
are discussed in Chapters 24 and 60, respectively.
Incidence
Pyloric atresia is extremely rare, accounting for less than 1% of atresias or stenoses
of the gastrointestinal tract. The incidence of neonatal duodenal obstruction is esti-
mated at 1 in 5,000 to 10,000 live births. Seventy-five percent of intestinal stenoses
and 40% of atresias are found in the duodenum. Multiple atresias occur in 15% of
cases.
Etiology
Pyloric atresia is usually caused by a solid mucosal diaphragm obstructing the
pylorus and may result from in utero vascular or mechanical fetal injury. Familial
and autosomal recessive forms have been described in association with epidermoly-
sis bullosa.
Intrinsic duodenal obstructions vary from duodenal narrowing or webs to com-
plete discontinuity of the duodenum. The origin of duodenal and pyloric atresia
may be a defect in recanalization of the embryonic duodenum. Normally, the prolif-
erating epithelial lining occludes the duodenal lumen during the fifth to sixth gesta-
tional week. Vacuolation and recanalization of the duodenum are completed by the
eighth to tenth week.
Annular pancreas and preduodenal portal vein may cause extrinsic duodenal
obstruction. Annular pancreas results when the anterior and posterior anlages of the
pancreas fuse to become a ring of pancreatic tissue surrounding the atretic or stenotic
3rd portion of the duodenum.
Pyloric Obstruction
The newborn with complete pyloric atresia or prepyloric antral web presents
shortly after birth with persistent, nonbilious vomiting and possibly epigastric
distension. Respiratory findings secondary to aspiration are common and include
dyspnea, tachypnea, and cyanosis. Excessive salavation and poor weight gain may
also occur. Prepyloric antral diaphragms may also present as an acquired lesion in
Pyloric and Duodenal Obstruction 255
older children. Symptoms in older children may include epigastric abdominal pain,
vomiting, or postprandial fullness. Thirty percent of these infants have associated
anomalies, epidermolysis bullosa being the most common.
Duodenal Obstruction
Fifty percent of neonates with duodenal atresia are born premature and are of
low birth weight. Maternal polyhydramnios is present in up to 75% of cases. Bilious
vomiting on the first day of life is the usual presenting feature. Vomiting may be
nonbilious in cases of preampullary atresia (20%). Abdominal distention may or
may not be present since obstruction is very high in the gastrointestinal tract. Meco-
nium is usually passed in the first 24 hours followed by constipation. Incomplete
obstruction may delay the onset of symptoms.
The anomalies associated with duodenal obstruction in order of greatest fre-
quency are Down’s syndrome (30%), malrotation, congenital heart disease, esoph-
ageal atresia, urinary tract malformation, and anorectal malformation. Vertebral
anomalies are present in about one third of cases. Duodenal atresia is associated with
the VACTERL syndrome/VATER association.
Differential Diagnosis
The differential diagnosis of pyloric and duodenal obstruction includes malrota-
tion, pyloric atresia, pyloric web, hypertrophic pyloric stenosis, duodenal atresia,
annular pancreas, and preduodenal portal vein.
Diagnosis
A plain x-ray of the abdomen is useful to confirm the diagnosis in cases of sus-
pected duodenal or pyloric obstruction in the newborn. In the rare cases of pyloric
atresia, abdominal films will typically demonstrate gas in a dilated stomach with no
gas beyond the pylorus. Three radiologic signs confirm the diagnosis of pyloric atresia:
1. single gas bubble sign,
2. pyloric dimple sign,
3. absence of a ‘beak’ sign (found in hypertrophic pyloric stenosis). Ultra-
sonography will demonstrate an absence of the normal echo pattern of
the pyloric channel and surrounding pyloric muscle.
An antral web gives the appearance of a membranous septum projecting into the
antral lumen, perpendicular to the antral wall, located 1-2 cm proximal to the pylorus.
A central aperture is present in 90% of cases.
Abdominal plain films in neonates with duodenal atresia (Figs. 57.1 and 57.2)
will demonstrate dilated stomach and duodenum giving the characteristic ‘double
bubble’ sign with no gas distal to the duodenum. If partial duodenal obstruction is
present, some air is usually present in the distal intestine. Ultrasound examination
can also identify the double bubble sign characteristic of duodenal atresia (Fig. 57.3).
Pathology and Classification
Pyloric atresia occurs as one of three types: membranous pyloric obstruction
(Type A), segmental atresia (Type B), pyloric aplasia (Type C). the approximate
distribution of each type is 55%, 35%, and 10% respectively.
57
Fig. 57.1. Double bubble sign demonstrated only with swallowed air shortly after
birth. Two arrows point to the stomach and the duodenum.
57
Fig. 57.2. A similar demonstration of the double bubble sign reversed in a patient
with situs inversus and duodenal obstruction.
Duodenal obstruction can be secondary to intrinsic or extrinsic lesions. Intrinsic

duodenal obstruction may be caused by duodenal atresia, diaphragm with or with-
out perforation (wind-sock web), or stenosis. In addition, duodenal obstruction
may be found as proximal and distal segments separated by a gap or joined by a
fibrous cord. Extrinsic duodenal obstruction can be caused by annular pancreas,
malrotation, or preduodenal portal vein. Annular pancreas constricts the 3rd por-
tion of the duodenum, but the obstruction is usually due to a concomitant duode-
nal atresia or stenosis. Duodenal obstruction occurs distal to the ampulla of Vater in 57
80% of cases.
Fig. 57.3. Ultrasound demonstration of double bubble sign associated with the
various types of duodenal obstruction (atresia, stenosis, membrane, or annular pan-
creas). Such demonstrations have even been done prenatally.
Treatment
Perioperative Management
Newborns admitted within 1-2 days with pyloric or duodenal atresia are usually
in good physical condition unless it is a case of epidermolysis bullosa associated with
a pyloric atresia. Gastric distension is relieved by nasogastric decompression. Intra-
venous hydration to correct dehydration, metabolic alkalosis, and electrolyte imbal-
ance is appropriate preoperative therapy.
Indications for Surgery
Surgical intervention is generally indicated in all forms of pyloric or duodenal
obstruction. Medical treatment consisting of thickened feeds and antispasmodics
can be used in the rare case of antral web without significant obstruction, but surgi-
cal therapy is preferred. Transgastric excision of the membrane without pyloroplasty
has been described.
Pyloric Obstruction
Pyloroplasty is the treatment of choice for membranous pyloric obstruction
(Type A) and short pyloric atresia (Type B). The membrane is excised through a
longitudinal incision across the pylorus and the mucosa is reapproximated. A cath-
eter must be passed distally to ensure there is no distal atresia. The longitudinal
57 incision in the pylorus is closed transversely. Long pyloric atresias and pyloric apla-
sia are surgically treated by resection and end-to-end gastroduodenostomy.
Duodenal Obstruction
In neonates with duodenal atresia, stenosis, or annular pancreas the surgical treat-
ment of choice is a ‘double diamond’ duodenoduodenostomy. A transverse incision
is made in the distal end of the proximal duodenal segment and a longitudinal
incision is made in the distal segment. An end-to-end anastamosis is performed.
Duodenal webs are excised through a longitudinal duodenotomy over the site of the
obstruction with special attention to locate and preserve the ampulla. The
duodenotomy is closed transversely after the distal patency of the duodenum is
confirmed.
Postoperative Care
Nasogastric decompression is continued postoperatively along with fluid and
electrolyte replacement. Patients with duodenal atresia often have prolonged bilious
drainage from the nasogastric tube due to ineffective peristalsis of the dilated duode-
num. Oral feedings may be delayed for several days to 2+ weeks until the volume of
gastric drainage decreases. Central venous access for hyperalimentation is often
desirable. Persistent dysfunction and deformity of the dilated proximal duodenum
occasionally requires duodenoplasty.
Outcomes
Early diagnosis and surgical intervention have improved survival of neonates
with pyloric or duodenal obstruction. Pyloric atresia is associated with an overall
mortality of 45% with the majority of deaths occurring in cases with epidermolysis
bullosa and multiple intestinal atresias. Mortality in infants with duodenal obstruc-
tion is approximately 15-20% and is attributable to a high incidence of associated
anomalies, prematurity, and low birth weight in these infants.
Selected Readings
1. Dalla Vecchia LK, Grosfeld JL, West KW et al. Intestinal atresia and stenosis: A
25-year experience with 277 cases. Arch Surg 1998; 133:490-497.
2. Raffensperger JG. Pyloric and duodenal obstruction. In: Raffensperger JG ed.
509-516.
3. Muller M, Morger R, Engert J. Pyloric atresia: Report of four cases and review of
the literature. Pediatr Surg Int 1990; 5:276.
4. Stauffer UG, Schwoebel M. Duodenal atresia and stenosis-annular pancreas. In:
O’Neill Jr. JA et al, eds. Pediatric Surgery, 5th Edition. St. Louis: Mosby 1998;
1133-1143.
57
CHAPTER 58
Malrotation and Volvulus

Vinh T. Lam
Incidence
From autopsy studies it appears that the incidence of malrotation in the general
population is between 0.2-0.5%. However, the generally accepted incidence based
on clinical presentation at the major children’s hospitals is one per every 6000 live
births. It is clear that this condition can be present throughout life without causing
any clinical problems. However, those afflicted generally present within the first six
months of life with smaller numbers occurring each year throughout childhood.
Presentation after teenage years or in adult life is quite rare.
Etiology
The growth rate of the gastrointestinal tract in early gestation is faster than that
of the body. Therefore, intestinal development occurs through the umbilical ring
within the physiological umbilical hernia (fourth week of gestation). During the
tenth week of gestation the intestine begins an orderly migration back into the
abdominal cavity. This includes a counterclockwise rotation of two intestinal seg-
ments: the duodenojejunal segment and the cecolic segment.
The duodenojejunal limb returns first and rotates 270˚ to the right of the supe-
rior mesenteric artery (SMA), passing beneath the artery into the left upper quad-
rant. At the completion of this process, the duodenojejunal junction is fixed to the
retroperitoneum at the ligament of Treitz.
The rotation of the cecocolic limb is also counterclockwise. It rotates from a
position left of the SMA, around SMA axis to attain its final position in the right
lower quadrant. By the twelfth week of gestation this process is complete, and the
colon becomes fixed to the retroperitoneum.
A broad based mesentery provides a stable position for the small intestine within
the peritoneal cavity. It begins in the right iliac fossa at the ileocecal junction and
runs obliquely upward across the abdomen to the ligament of Treitz. The duode-
num is fixed securely to the retroperitoneum as the C-loop. The ascending and
descending colon are fixed to the right and left retroperitoneum, and the transverse
colon is fixed at either end as it drapes across the superior mesentery vessels. If
fixation does not take place, the intestine is suspended by a thin stalk containing the
superior mesenteric vessels and is susceptible to midgut volvulus.
Children with congenital diaphragmatic hernia, omphalocele or gastroschisis
will have malrotation as a matter of course since the bowel cannot undergo normal
Malrotation and Volvulus 261
rotation and fixation. However, other gastrointestinal problems, including intestinal

atresias and Hirschsprung’s disease, have occurred in association with malrotation.
Classification 58
During the course of rotation and fixation, three conditions develop that make
the gut susceptible to volvulus:
Nonrotation
This is the most common anomaly. It occurs when neither duodenojejunal or
cecocolic limb have undergone correct rotation. The duodenojejunal and ileocecal
junctions lie close together while the midgut hangs on a thin stalk containing the
superior mesenteric vessels. The entire gut is poorly stabilized due to the narrow
base of the mesentery.
Abnormal Rotation of the Duodenojejunal Limb
Nonrotation of the duodenojejunal limb followed by normal rotation and fixa-
tion of the cecocolic limb results in duodenal obstruction caused by abnormal
mesenteric (Ladd’s) bands extending from the colon across the anterior duodenum.
The risk of midgut volvulus in this condition is lower because there is a relatively
broad mesenteric base between the duodenojejunal junction and the cecum.
Abnormal Rotation of the Cecocolic Limb
Normal rotation of the duodenojejunal limb with nonrotation of the cecocolic
limb results in the same potential for midgut volvulus as complete nonrotation. In
this condition, the cecum and the first part of the colon lie close to the midline
against the third portion of the duodenum over the superior mesenteric vessels. The
mesenteric base is narrow and stability is poor. Volvulus easily develops.
Reverse rotation of either limb create rarer forms of malrotation presenting as
colonic obstruction, entrapment in a paraduodenal hernia, or midgut volvulus.
Presentation varies depending on the specific mechanism of obstruction and the
extent of vascular compromise. Symptoms may be either mild and intermittent or
severe and catastrophic with complete obstruction and vascular occlusion.
The most common symptom of malrotation with volvulus is vomiting (95%).
Initially, the vomitus is gastric or bilious in nature but may become grossly bloody if
bowel compromise is present. Abdominal distention follows with bloody diarrhea
(28%) indicating bowel ischemia or necrosis. Children with volvulus appear severely
ill and complain of generalized abdominal pain if they can speak. Lethargy, grunting
respirations, dehydration, peritonitis and shock follow as the bowel ischemia per-
sists or worsens.
Diagnosis
The diagnosis of malrotation with or without volvulus usually rests upon radio-
graphic confirmation. Plain abdominal radiographs may demonstrate obstruction
and abdominal distention. The radiographic appearance of a “gasless” abdomen occurs
when the volvulus has created a closed-loop obstruction from which the intralumi-
nal air has been absorbed and replaced with fluid.
Contrast radiographic studies can easily demonstrate the site of obstruction and
the presence of the malrotation. In simple malrotation, the upper gastrointestinal
58 series shows the incomplete rotation of the duodenojejunal loop (Fig. 58.1). If vol-
vulus has occurred, there is frequently an abrupt cut-off to passage of barium described
as a “bird’s beak” in the third portion of the duodenum. Alternatively, duodenal
obstruction may be only partial and have a spiral or corkscrew appearance.
Barium enema can identify the position of an abnormally placed cecum suggest-
ing malrotation. However, the position of the cecum is highly variably in small
children, and about 15% of children with malrotation will have a normally placed
colon.
Ultrasonography has proven to be very reliable in making the diagnosis of mal-
rotation at some centers. The relative position of the superior mesenteric artery and
vein is normally quite constant and characteristic with the vein to the right of the
artery. Absence of this normal relationship strongly suggests malrotation.
Treatment
Midgut volvulus is a surgical emergency. A child with this condition requires
intravenous access, a nasogastric tube, expeditious fluid resuscitation followed by an
emergent laparotomy. Malrotation without volvulus is a relatively nonemergent con-
dition that allows more time for preoperative decision making.
The operative management (Ladd procedure) of malrotation involves six prin-
ciples. These can be applied to those with uncomplicated malrotation as well as to
those with midgut volvulus.
Evisceration
Generally a supra-umbilical transverse incision is made. The bowel is eviscerated
and the malrotation is confirmed (Fig. 58.2). Ascites (chylous from obstruction or
bloody from necrosis) is frequently encountered and drained.
Untwisting of the Volvulus
The volvulus is untwisted by rotating it counterclockwise. If there is no bowel
compromise, the operation is continued. If the bowel is edematous and hemor-
rhagic, improvement may occur with untwisting. If the bowel appears necrotic or
nonviable, a second look operation 24-36 hours later may help to determine viabil-
ity and preserve bowel.
Division of Ladd’s Bands
Ladd’s bands create a duodenal obstruction as they pass over the malpositioned
second and third portion of the duodenum. These peritoneal folds are divided freely
mobilizing the cecum.
Widening the Mesenteric Base
After untwisting the volvulus and lysis of Ladd’s bands, it is generally possible to
widen the base of the mesentery. This is achieved by placing the small bowel along
the right gutter and positioning the colon to the left with the ileocecal valve facing
in the opposite direction.
Malrotation and Volvulus 263
58
Fig. 58.1. Upper gastrointestinal series x-ray demonstrating failure of the duode-
num to complete rotation and reach a proper position in the left upper quadrant at
the ligament of Trietz.
Fig. 58.2. Intraoperative photograph of malrotation. Large arrow indicates the ceph-
alad direction. Smaller arrows demonstrate the backward orientation of the ileoce-
cal valve with the cecum and appendix clearly unfixed and in the wrong position.
Relief of Duodenal Obstruction

Lysis of Ladd’s bands will relieve most obstructions but occasionally further dis-
58 section is needed to fully mobilize and straighten the duodenum. In addition,
intraluminal duodenal obstruction (i.e., atresia, stenosis) may coexist and must be
identified and corrected. A soft tube can be passed through the bowel along its
entire length to verify its patency.
Incidental Appendectomy
Since the colon and the attached appendix generally lie in an abnormal position
both before and after an operation for malrotation, it is advisable to remove the
appendix to prevent confusion if the child were to develop appendicitis in the future.
Outcomes
Recurrent volvulus has been reported in up to 10% of children having the Ladd
procedure. In addition, these children have the 5-6% chance of postoperative bowel
obstruction secondary to adhesions. Complications of atelectasis, wound infection,
postoperative bleeding, and prolonged ileus are all common. Many children have
protracted hospital stays, particularly if a volvulus progressed to long segments of
intestinal necrosis requiring resection. In fact, 18% of children with short bowel
syndrome have this condition secondary to malrotation with midgut volvulus. In
children who present with peritonitis, shock and sepsis, multiple organ failure with
death may occur despite aggressive resuscitation and surgical intervention.
Selected Readings
1. Ladd WE. Congenital Obstruction of the Duodenum in Children. N Engl J Med
1932; 206:277-283.
2. Touloukian RJ, Smith EI: Disorders of Rotation and Fixation. In: O’Neill, Jr. JA et
al, eds. Pediatric Surgery, 5th Edition. St. Louis: Mosby 1998; (2)1199-1214.
3. Snyder WH, Chaffin L. Embryology and pathology of the intestinal tract: Presen-
tation of 48 cases of malrotation. Ann Surg 1954; 140:368.
CHAPTER 1
CHAPTER 59
Atresia and Stenosis

P. Stephen Almond
Incidence
Jejunal atresia is the most common gastrointestinal atresia and occurs in about
one per 2,000 live births.
Etiology
In, 1955 Louw and Barnard presented convincing evidence that small bowel
atresias were secondary to an in-utero occlusion of all or a portion of the blood
supply to the small bowel, i.e., the superior mesenteric artery. The affected bowel
scars down to a fibrotic remnant or may be totally reabsorbed.
The bowel is shortened in all but type I lesions. Peristalsis in the proximal, bul-
bous tip may be abnormal. Bowel proximal and distal to the atresia may be deficient
in acetylcholinesterase activity and cholinergic ganglia. The muscle layers are often
replaced with scar tissue.
Four types of atresias have been described. In type I, continuity of the bowel wall
is preserved with obstruction being caused by an intraluminal diaphragm or ‘windsock’
of mucosa. This accounts for about 20% of atresia. In type II, continuity of the
bowel wall is preserved only by a solid cord of tissue between the proximally dilated
and distally collapsed bowel. This accounts for 35% of atresias. Type III (Fig. 59.1)
has been divided into types IIIa and IIIb. In type IIIa, a portion of bowel and its
associated mesentery are missing leaving two blind ends of bowel. Type IIIb is char-
acterized by an extensive loss of small bowel and a large mesenteric defect. The
blood supply for the remaining small bowel is supplied retrograde by the ileocolic
artery. As there is no mesentery, the bowel spirals tightly around this artery giving
the appearance of a spiral staircase or ‘apple peel.’ Multiple atresias are classified as
type IV.
The infant is usually referred to a pediatric surgeon within the first 24-48 hours
of life with bilious vomiting, abdominal distention, and failure to pass meconium
(70%). The differential diagnosis includes meconium disease, Hirschsprung’s dis-
ease, small left colon syndrome, malrotation, and intussusception. Conditions
59
Fig. 59.1. Intraoperative photograph of a jejunal atresia, type III, with muscular
discontinuity and mesenteric loss. Three arrows demonstrate the bulbous end of
the proximal atresia, while two arrows show the resumption of the bowel beyond
the segment of loss.
associated with jejuno-ileal atresia include other intestinal atresias (10-15%),

Hirschsprung’s disease (xx%), biliary atresia, polysplenia syndrome (situs inversus,
cardiac anomalies, biliary atresia, intestinal atresia), and cystic fibrosis (10%).
Diagnosis
In jejunal atresia, abdominal films show dilated loops of bowel with air fluid
levels. If perforation has occurred, free air and/or peritoneal calcifications may be
present (12% of cases). Barium enema demonstrates a small unused colon and often
shows reflux of contrast into a collapsed terminal ileum.
Treatment
The infant should be placed under a radiant warmer until normothermic. A
nasogastric tube and IV are placed and the infant is resuscitated with boluses
(20 cc/kg) of normal saline until an adequate urine output (2 cc/kg/hr) is achieved.
A hemoglobin, capillary blood gas, and blood crossmatch are sent to the lab. After
these things are done, the infant may be taken to the radiology suite for diagnostic
studies as indicated.
At operation, a supraumbilical, transverse incision is made. The duodenum is
identified and the dilated small intestine is followed to the point of obstruction. The
distal bowel is then opened and irrigated to rule out distal atresias. The colon, spleen,
and biliary tree are likewise inspected to rule out associated atresias, polysplenia or
asplenia syndromes, and biliary atresia. If possible, the dilated bulbous tip should be
resected or tapered and intestinal continuity re-established with primary anastamosis.
Atresia and Stenosis 267
Postoperatively, the infant should be maintained on peripheral hyperalimentation,

nasogastric suction, and antibiotics. All patients should be tested for cystic fibrosis
at least twice. If bowel function does not return in a timely fashion, the possibility of
Hirschsprung’s disease or missed atresia should be considered.
Outcomes 59
Survival in infants with jejunal atresia is greater than 95%.
Selected Readings
1. Dalla Vecchia LK, Grosfeld JL, West KW et al. Intestinal atresia and stenosis: A
25-year experience with 277 cases. Arch Surg 1998; 133:490-497.
2. Janik JP, Wayne ER, Janik JS et al. Ileal atresia with total colonic aganglionosis. J
Pediatr Surg 1997; 32:1502-3.
3. Rescorla FJ, Grosfeld JL. Intestinal atresia and stenosis: analysis of survival in 120
cases. Surgery 1985; 98:668-675.
CHAPTER 60
Meconium Ileus
Vinh T. Lam
Meconium ileus is one of the common causes of intestinal obstruction in the

newborn. It specifically refers to the distal small intestinal obstruction caused by
inspissated meconium in newborns. Approximately 80-90% of these infants will
have cystic fibrosis (mucoviscidosis). Meconium peritonitis occurs when meconium
ileus is complicated by volvulus and/or perforation. The clinical features and pre-
sentation of meconium ileus in the newborn can be quite variable.
Incidence
The incidence of cystic fibrosis ranges from 1 per every 1000-2000 live births.
The genetic defect is transmitted as an autosomal recessive trait; the carrier rate
among Caucasians is about 5-6%. Cystic fibrosis much more rarely occurs in chil-
dren of African or Asian decent. Meconium ileus occurs in 10-20% of infants with
cystic fibrosis.
Etiology
Cystic fibrosis results from a mutation of the cystic fibrosis gene located on the
long arm of chromosome 7. Although many types of mutations have been identi-
fied, nearly 70% of these patients have a 3-base deletion called the ∆F508 mutation.
The cystic fibrosis gene encodes for a cAMP-activated chloride channel protein that
helps regulate fluid balance across the apical surface of epithelial cells in many organs.
Ninety percent of children with cystic fibrosis will have pancreatic enzyme defi-
ciency and/or abnormal meconium composition. However, this meconium abnor-
mality is sufficiently severe in 10-20% of affected neonates to produce tenacious,
thick meconium that adheres to the distal ileal mucosa, producing distal intestinal
obstruction.
The protein concentration in the meconium of normal infants is about 7%. In
neonates with cystic fibrosis and meconium ileus, the protein content is nearly
80-90%. The abnormally viscous meconium results from both abnormal secretions
(pancreatic and intestinal) as well as abnormal proximal small intestinal concentrat-
ing mechanisms. Infants with cystic fibrosis and meconium ileus have mild pancre-
atic disease but intestinal glands are severely affected. The intestinal glandular
abnormality may be the primary pathologic entity leading to the development of
meconium ileus.
Meconium Ileus 269
In utero, the highly viscous, thick meconium adheres to the ileal mucosa and
inspissated “putty-like” pellets form within the lumen obstructing the terminal ileum
and proximal colon. The ileum proximal to the obstruction dilates to several centi-
meters, while the entire colon distal to the inspissated meconium is small in caliber
(microcolon). The dilated, meconium-filled ileal segment is prone to volvulus.
Classification
Meconium ileus is classified as simple (uncomplicated) or complicated. In simple 60
meconium ileus, the abnormal meconium causes distal intestinal obstruction sec-
ondary to inspissated “pellets” at the terminal ileum. The “pellet”-containing ileum
is small in caliber while the proximal ileum is dilated. Microcolon is also present.
Complicated meconium ileus is believed to be the result of volvulus in the proxi-
mal dilated intestinal segment. As with other forms of volvulus, intestinal ischemia,
necrosis, or perforation can occur. Depending upon the timing and evolution of the
volvulus, complicated meconium ileus may result in intestinal atresia, perforation(s)
and meconium peritonitis with ascites. If perforation occurs after birth, bacterial
peritonitis can occur.
Infants born with meconium ileus generally present with three cardinal signs:
1. generalized abdominal distention developing within the first 24-48 hours
of life,
2. initially clear then bilious emesis/gastric aspirate, and
3. failure to pass meconium in the first 24-48 hours.
Maternal polyhydramnios occurs in about 20% of cases. A family history of
cystic fibrosis is present in 10-30% of cases. In both simple or complicated cases,
some of these infants tolerate feedings for several hours before signs and symptoms
of bowel obstruction or perforation develop. Infants with complicated meconium
ileus tend to develop symptoms earlier. Many present shortly after birth with severe
abdominal distention, bilious emesis, and occasionally respiratory distress. Hypov-
olemia and hemodynamic instability are also common presenting features of com-
plicated meconium ileus.
Physical examination reveals a distended abdomen. At times it is possible to
palpate meconium-impacted bowel in the right lower quadrant that feels “doughy”
or “rubbery.” In female babies with meconium peritonitis, meconium can occasion-
ally be observed in the vagina having passed through the fallopian tubes and uterus.
In boys with meconium peritonitis, the scrotum may appear black as a result of
meconium passing through a patent process into the scrotum. Meconium in the
scrotal sac as in the peritoneal cavity may be calcified.
The differential diagnosis includes the many causes of neonatal bowel obstruc-
tion (see Chapter 56). The most common causes of distal bowel obstruction that
mimic meconium ileus are: ileal atresia, Hirschsprung’s disease, small left colon syn-
drome, meconium plug syndrome, and colon atresia. Sepsis also presents with
abdominal distension and feeding intolerance and is included in the differential
diagnosis.
Similar to meconium ileus, meconium plug syndrome is a transient neonatal
colonic obstruction. Twenty-five percent of infants presenting with meconium plug
syndrome have cystic fibrosis. The other cases of this syndrome are caused by a
transient motility disorder of the distal colon related to immaturity or a maternal
history of diabetes (50%). All infants with this syndrome should be screened for
cystic fibrosis and Hirschsprung’s disease (Chapter 61).
Diagnosis
Plain radiographs of the abdomen are necessary in cases of suspected neonatal
60 bowel obstruction; meconium ileus is no exception. In simple meconium ileus, plain
films demonstrate dilated loops with relative absence of air fluid levels (precluded by
the thick, viscus meconium). A “ground glass” or “soap bubble” appearance is often
appreciated in the right lower quadrant corresponding to bowel loops filled with
thick meconium mixed with air. In cases of complicated meconium ileus radio-
graphic findings may include:
1. calcifications (as a result of perforation with extravasation of meconium
in the peritoneal cavity),
2. massive bowel dilation (i.e., ileal atresia),
3. mass effect (i.e., giant cyst, etc)
4. more impressive air–fluid levels, and
5. ascites.
If simple meconium ileus is suspected, barium or water-soluble contrast enema
is performed to confirm the diagnosis. The contrast radiographic findings that help
confirm the diagnosis include:
1. a small and unused microcolon,
2. inspissated meconium “pellets” in the terminal ileum.
Microcolon is also seen in cases of atresia with or without meconium ileus. Long
segment Hirschsprung’s disease and total colonic aganglionosis can frequently present
with a clinical and radiologic picture identical to that of meconium ileus and should
always be considered.
The chloride sweat test (pilocarpine iontophoresis) is a simple noninvasive test
that is used to identify infants with cystic fibrosis. In this test, an electrical current is
used to stimulate sweat production at a localized area of skin treated with pilo-
carpine. The sweat is collected and the chloride content is measured. A sweat chlo-
ride level above 60 mEq/L identifies neonates with cystic fibrosis with high accuracy.
The test should be performed in neonates beyond 2-3 days of life. Genetic testing
confirms the diagnosis of cystic fibrosis.
Treatment
The initial management of neonates with bowel obstruction (i.e., meconium
ileus) includes resuscitation with intravenous fluids, stomach decompression with a
gastric tube, and administration of broad spectrum antibiotics. Simple meconium
ileus can frequently be managed nonoperatively with gastrograffin enemas performed
in the radiology suite under fluoroscopic guidance. Dilute gastrograffin (3:1 or 4:1
water to gastrograffin) is a contrast agent that is administered per rectum and intro-
duced into the terminal ileum. This hyperosmolar agent draws fluid into the bowel
lumen effectively clearing thick, viscus, and often inspissated meconium. The
gatrograffin enema may need to be repeated 2 or 3 times over a 24-72 hour period
to be effective. Oral N-acetylcysteine (mucomyst) in concentrations of 5-10% can
Meconium Ileus 271
also be given to help clear the thick meconium which usually begins to pass within
12-18 hours following enema.
Surgical intervention is required in:
1. most neonates with complicated meconium ileus, and
2. neonates with simple meconium ileus having 2 or 3 unsuccessful
gastrograffin enemas. In patients with uncomplicated meconium ileus
requiring surgery, the procedure is enterotomy with irrigation and removal
of the obstructing meconium. 60
Irrigation with various solutions (i.e., saline, gastrograffin, N-acetylcysteine, etc.) is
helpful to dissolve the thick inspissated meconium. The appendiceal stump is fre-
quently the site chosen to instill these solutions and evacuate the meconium.
In neonates with meconium ileus complicated by ileal atresia or volvulus, surgi-
cal management usually includes resection of the dilated segment, distal irrigation,
and primary anastamosis to restore bowel continuity. In cases of perforation or giant
cystic meconium peritonitis, the preferred procedure is debridement, cyst resection,
and temporary enterostomy. Ostomy closure is typically performed 4-6 weeks later.
Other surgical options for complicated meconium ileus include the use of special
ostomy procedures (i.e., Bishop-Koop, Mikulicz, Santulli-Blanc).
Outcomes
Recent one-year survival rates for infants with simple meconium ileus are
92-100%. For infants with complicated meconium ileus, one-year survival is lower
at 75-89%. Overall survival in these infants has benefited from improved long-term
management of patients with cystic fibrosis. Pulmonary complications are respon-
sible for the majority of late deaths in these children. Late complications associated
with cystic fibrosis include:
1. distal intestinal obstruction syndrome (2-11%),
2. appendicitis (4-5%),
3. intussusception (1-2%),
4. rectal prolapse (11-30%),
5. colon strictures, and
6. gall bladder disease.
Selected Readings
1. Rescorla FJ, Grosfeld JL. Contemporary management of meconium ileus. World J
Surg 1993; 17:318.
2. Bishop HC, Koop CE. Management of meconium ileus: Resection, Roux-en-Y
anastamosis and ileostomy irrigation with pancreatic enzymes. Ann Surg 1957;
145:410.
3. Santulli TV, Blanc WA. Congenital atresia of the intestine: Pathogenesis and treat-
ment. Ann Surg 1961; 154:939.
4. Ziegler MM. Meconium ileus. Curr Prob Surg 1994; 31:736.
5. Rescorla FJ. Meconium ileus. In O’Neill Jr. JA et al, eds. Pediatric Surgery, 5th
CHAPTER 61
Hirschsprung’s Disease
Robert M. Arensman
Hirschsprung’s disease or congenital megacolon is a developmental anomaly
caused by migratory failure of neural crest cells. When these primitive neurogenic
cells fail to take up positions in the submucosal and intermyenteric plexi of the
bowel from lips to anus, motility disturbances result that most routinely present as
chronic constipation in a newborn child.
Incidence
Incidence is approximately 1:5000 live births with males affected four times as
frequently as females (4:1 males to females).
Etiology
Reasons for migratory failure are unknown. Nevertheless, the pattern of neural
crest migration with localization in parasympathetic plexi from the proximal bowel
to the distal bowel is well described. Characteristically, the distal extent of migration
in children with Hirshsprung’s disease is the rectosigmoid region. However, females
and children with Down’s syndrome often have longer segments of aganglionosis.
Cases of total colonic aganglionosis and very rarely extensive small bowel aganglionosis
are well reported.
Familial occurrence can be documented in 3-30% of reported cases, with a greater
familial incidence in longer segment disease. Chromosomal mutations have been
identified on chromosomes 10 and 13 in some families.
Ninety-eight percent of babies pass meconium within 24-48 hours of birth. In
babies with Hirschsprung’s disease 90% fail to achieve this passage. The disease
progresses to abdominal distension, bilious vomiting, and possibly obstructive
enterocolitis.
If a neonate leaves the hospital without diagnosis, he will generally reappear with
chronic constipation within two years. This constipation often accompanies a dietary
change such as the change from breast milk to formula or formula to solid foods.
Rarely, children escape diagnosis until more advanced ages when chronic constipa-
tion and failure to thrive are seen.
The most common physical findings are abdominal distension, visible bowel
loops with peristalsis, and poor muscle development secondary to poor nutrition.
Rectal examination reveals a spastic rectum with little or no stool since the stool
bolus is high and beyond the examining finger.
Hirschsprung’s Disease 273
61
Fig. 61.1. Lateral radiograph of the rectum from a neonatal barium enema demon-
strating the narrow caliber rectum and sudden transition to dilated colon.
Examination should include a check for associated syndromes: Down’s syndrome,

trisomy 18, Waardenburg syndrome, von Recklinghausen’s syndrome, type D brachy-
dactyly, and Smith-Lemli-Opitz syndrome.
Diagnosis
When history and physical exam suggest Hirschsprung’s disease, diagnosis has
always rested on barium enema, anorectal manometry, and rectal biopsy. The first
two can suggest the diagnosis but only the biopsy can conclusively confirm the
diagnosis.
Barium enema reveals a spastic, poorly compliant rectum with eventual passage
through a cone shaped transition zone into dilated colon (Fig. 61.1). These findings
are often more apparent in older children who have had sufficient time to develop
greater dilation. Delayed films often reveal slow clearance of contrast and may reveal
the cone shaped transition zone better than the retrograde injection (Fig. 61.2).
In normally innervated colon, distention of the rectum produces reflex relax-
ation of the internal sphincter which is easily assessed with diagnostic manometry.
Failure to demonstrate this normal reflex strongly suggests Hirschsprung’s disease
although the accuracy in neonates is an unsettled controversy at the present time.
Rectal biopsy (suction for neonates and infants; punch or strip for older chil-
dren) demonstrates absence of ganglion cells and nerve hypertrophy inpatients with
Hirschsprung’s disease. Special stains can further delineate acetylcholinesterase activity
in abnormal quantities or a variety of neuronal markers. Biopsies can be repeated
internally to establish the exact length of the aganglionic segment.
61
Fig. 61.2. Transition zone at splenic flexure demonstrating the change from bowel
with ganglionic cells to the aganglionic distal segment.
Pathophysiology
Long-term obstruction produces colonic dilation, often poor nutritional absorp-
tion or utilization, occasional small bowel dilation, and rarely vomiting. The result
is a “sickly” child who fails to do well socially or educationally. In 20-40% of chil-
dren with Hirschsprung’s disease, an obstructive enterocolitis develops: fever, vom-
iting, severe diarrhea, shock and sepsis. Mortality is high in this group of children
despite aggressive resuscitation and antibiotics.
Treatment
Suction rectal biopsy is the first treatment modality since it establishes the diag-
nosis and allows further and definitive therapy. If the child has passed the neonatal
or infant period, it may be necessary to do a punch or strip biopsy to obtain a
sufficiently deep specimen to make the diagnosis of aganglionosis.
Historically, these children were managed with initial colostomies in a normal
piece of bowel followed by reconstructive surgery when they were older and larger.
Today neonatal repair has been demonstrated safe and effective without use of an
antecedent colostomy. Children first seen as older individuals can be managed by
rectal irrigation and primary repair if the colon is not massively dilated. In that case,
Hirschsprung’s Disease 275
a decompressing colostomy followed by elective repair after regression of colon size

remains the best alternative.
Colonic pull down procedures of slightly different variation are the current
definitive therapy. All have slight advantages and disadvantages but have been shown
to give good, reliable and repetitive results. In all operations the goal is to eliminate
or greatly reduce the aganglionic portion of bowel while bringing a piece of normal
bowel close to the anal sphincter. Recent advances have allowed most of these proce-
dures to be performed laparoscopically as well as via laparotomy.
In cases of total colonic aganglionosis, procedures similar to those used for
inflammatory bowel disease have been used with fair results to achieve bowel func-
tion, continence, and sphincter control.
61
Selected Readings
1. Sherman JO, Snyder ME, Weitzman JJ et al. A 40 year multinational retrospective
study of 880 Swenson procedures. J Pediatr Surg 1989; 24:833.
2. Nixon HH. Hirschsprung’s disease: progress in management and diagnosis. World
J Surg 1985; 9:189.
3. Swenson O, Sherman JO, Fisher HG. Diagnosis of congenital megacolon: an analy-
sis of 501 patients. J Pediatr Surg 1973; 8:587.
4. Swenson O, Bill AH. Resection of rectum and rectosigmoid with preservation of
sphincter for benign spastic lesions producing megacolon. Surgery 1948; 24:212.
5. Duhamel B. A new operation for the treatment of Hirschsprung’s disease. Arch
Dis Child 1960; 35:38.
6. Soave F. Hirschsprung’s disease: A new surgical technique. Arch Dis Child 1963;
39:16.
CHAPTER 62
Colonic Atresia
P. Stephen Almond
Incidence
Colonic atresia is a rare disorder of the newborn. The incidence is estimated at
one per every 20,000 to 40,000 live births. It is the least common intestinal atresia
accounting for only 5-10% of reported cases.
Etiology
Although the exact etiology of colonic atresia is unknown, it is believed by many
to result from in-utero vascular occlusion of the large bowel.
Infants with colonic atresia present within the first 24 hours of life with abdomi-
nal distension, bilious vomiting, and failure to pass meconium. Intestinal loops are
often both visible and palpable through the distended abdominal wall. On rare
occasions, infants with colonic atresia present very ill with volvulus or with peritoni-
tis secondary to perforation of the proximal dilated bowel.
The differential diagnosis includes malrotation with volvulus, small bowel atre-
sia, meconium disease, and Hirschprung’s disease. Drug-induced ileus, megacystis
hypoperistalsis syndrome, and hypoplastic left colon are other conditions to con-
sider. Colonic atresia has been reported in infants with Hirschsprung’s disease, small
intestinal atresias, abdominal wall defects (i.e., gastroschisis, omphalocele), anorec-
tal malformations, and other major anomalies ( renal, cardiac, ocular). Polydactyly
and syndactyly are also associated with colonic atresia.
Diagnosis
Abdominal films demonstrate air-fluid levels and often a huge dilated loop just
proximal to the obstruction. Barium enema shows a small unused colon that ends
abruptly at the level of obstruction before refluxing in the small bowel. If associated
with a small bowel atresia, the diagnosis is often not made until laparotomy.
Classification
The classification of colonic atresias is the same as that of small intestinal atresias.
An intraluminal membrane obstructing an otherwise intact colon wall is a Type I
lesion. In type II lesions, the bowel segments remain connected via a cord-like band
and there is no mesenteric defect. Type III atresias lack a connection between the
bowel segments and often have large mesenteric defects. Atresias isolated to the
Colonic Atresia 277
62
Fig. 62.1. Photograph of a child with colonic atresia. Small arrow indicates the
appendix and the large arrows demonstrate the right colon with abrupt end at the
hepatic flexure. Transverse and descending colon were normal and ganglion cells
were present throughout (excluding Hirchsprung’s disease).
colon are equally distributed by type and location. However, type III atresias are the
most common lesions if ileocolonic atresias are also included. Type IV lesions con-
tain multiple atresias.
Treatment
The infant should be brought to the operating room euvolemic and normother-
mic. At operation, proximal and distal atresias should be identified (Fig. 62.1) and
colonic biopsies taken to assess for the presence of Hirschsprung’s disease
(aganglionosis). In infants with Hirschsprung’s disease, a leveling colostomy should
be performed. Infants without Hirschsprung’s disease can be managed in one of two
ways. In one method, the proximal bulbous tip is either resected or tapered and a
primary anastomosis is performed. Alternatively, an end colostomy can be performed.
Selected Readings
1. Potts WJ. Congenital atresia of the intestine and colon. Surg Gynecol Obstet 1947;
85:14.
2. Powell RW, Raffensperger JG. Congenital atresia of the intestine. J Pediatr Surg
1982; 17:166.
3. Oldham KT. Atresia, stenosis and other obstructions of the colon. In: O’Neill Jr.
JA et al, eds. Pediatric Surgery, 5th Edition. St. Louis: Mosby 1998; 1361-1368.
CHAPTER 63
Gastrointestinal Duplications
and Mesenteric Cysts
Riccardo Superina and Daniel A. Bambini
Gastrointestinal Duplications
Incidence
Duplications of the alimentary tract occur infrequently. Although an exact inci-
dence is unknown, duplications are identifiable in 1 of every 4500-5000 autopsies
performed. Two types of gastrointestinal duplications exist: tubular and cystic. The
tubular kind are relatively uncommon compared to the cystic type.
Gastric duplications account for only 3-4% of all gastrointestinal duplications,
occur twice as often in females than males, and occur along the greater curvature of
the stomach in over 50% of cases. Intestinal duplications account for 45-55% of all
gastrointestinal duplications. Most occur at the terminal ileum. Duodenal duplica-
tions are slightly more common than gastric duplications representing 5-6% of the
total seen. Colon duplications are the rarest reported. Rectal duplications are also
exceptionally rare.
Etiology
Split Notochord Mechanism
Early in embryonic life, the neurenteric canal is formed connecting the primitive
neural tube with the developing intestine. During closure of the neurenteric canal,
remnants of developing intestine may be deposited anywhere from the intraspinal
space to the chest and abdominal cavities. Intestinal duplications originating in this
fashion are usually of the tubular type.
Incomplete Recanalization
After the intestine goes through its solid phase, recanalization yields a long con-
tinuous tube. Errors in this process can leave cystic structures composed of intestinal
remnants beside the normal gut. These types of duplications are usually of the cystic
type.
Incomplete Twinning
Duplications of long segments of the intestinal tract can be secondary to an
aborted twinning process. This type of duplication most commonly involves the
hindgut causing duplications of the colon, rectum and anus.
Gastrointestinal Duplications and Mesenteric Cysts 279
Intestinal Obstruction
Duplications most commonly come to clinical attention through the develop-
ment of obstructive symptoms. Obstruction may be caused by three predominant
mechanisms:
1. The mass of an expanding duplication can compress the adjacent intes-
tine and narrow the lumen enough to cause colicky pain, vomiting and
distension if low enough in the intestinal tract.
2. The duplication may act as the lead point of an intussusception.
3. The duplication may cause a segmental volvulus with closed loop
obstruction.
Obstructive symptoms vary, depending on the location of the duplication and
whether it is cystic or tubular. In the chest, they may cause difficulty in swallowing.
Duplications should always be suspected when a child presents with a picture of 63
intestinal obstruction if there has been no previous surgery and intussusception is
ruled out.
Gastrointestinal Bleeding
Tubular duplications often contain ectopic gastric mucosa. Acid secretion can
cause peptic ulcers in the adjacent bowel with which they communicate. Since
duplications can occur anywhere along the gastrointestinal tract, bleeding may be of
the upper type with hematemesis and melena, or it can present as bright red blood
per rectum. Bleeding is often very noticeable and requires transfusion of blood for
patient stabilization.
Perforations
Another presentation of duplications that secrete acid is perforation. Peptic
ulceration and perforation of adjacent bowel occurs by a mechanism similar to gas-
tric peptic ulcer disease.
Neurologic Symptoms
Not infrequently, tubular duplications of the abdomen and chest are associated
with intraspinal cysts with which they may communicate via a patent channel, or
through an atretic fibrous cord extending through the adjacent vertebral body. These
intraspinal cysts may also contain intestinal mucosa and cause symptoms of muscle
weakness or paralysis before the extra-spinal cysts have declared themselves.
Asymptomatic Masses
With the increasing use of ultrasound for the diagnosis of genitourinary symp-
toms, asymptomatic cystic lesions may sometimes be observed. Prenatal diagnosis
of cystic lesions of the intestine is also being made more frequently.
Diagnosis
A Meckel’s scan is usually positive for tubular duplications that contain ectopic
gastric mucosa. They appear as large areas which take up tracer and may be located
in areas where a Meckelís diverticulum would not normally be found such as the
chest.
Esophageal duplications, if large enough, can often be observed as posterior

mediastinal radiopaque bodies on plain radiographs of the chest. Esophageal dupli-
cations are frequently associated with vertebral anomalies that if present on the plain
film confirm the diagnosis. The esophageal lumen is compressed on a barium swal-
low study. The most definitive study is the CT scan, which can clearly outline the
size, position and density of a paraesophageal mass.
Diagnosis of a duplication in the abdomen may be more difficult. Patients with
intestinal obstruction by any mechanism show dilated intestinal loops and air fluid
levels. Vertebral anomalies should be carefully looked for when duplications in the
abdomen are suspected. Abdominal ultrasound often successfully demonstrates a
fluid filled cystic duplication. Frequently, however, intra-abdominal duplications
are only diagnosed at the time of laparotomy.
Pathology and Pathophysiology
Intestinal duplications may contain epithelium of the bowel to which they asso-
63 ciated, or they may contain ectopic intestinal or respiratory epithelium. Spinal cysts
contain intestinal epithelium that secretes mucous The cyst expands slowly until it
causes compressive symptoms of the spinal cord. Gastric mucosa, when present,
secretes acid. Normal adjacent bowel with which the duplication communicates
may develop peptic ulceration with potential for bleeding or perforation.
Classification
Cystic
Cystic duplications are the more common type. As their name implies, cystic
duplications are rounded, hollow intestinal segments located most commonly in the
distal ileum. They can occur anywhere along the intestinal tract. There is usually no
communication with the adjacent bowel, so that the natural history is for the dupli-
cation to grow until it causes symptoms since there is no way for the duplication to
empty. Cystic duplications usually involve only a short segment of bowel and can
therefore be easily removed surgically. Cystic duplications share a muscular layer
with the adjacent bowel but have a separate mucosal layer. The mucosal lining is
similar to that of the adjacent bowel.
Tubular Duplications
Tubular duplications are often long segments of bowel and can occur anywhere
from the esophagus to the rectum. They run parallel to the normal bowel. If they
communicate with the normal bowel at the distal end, they do not distend and
cause obstruction. Tubular duplications often have ectopic gastric mucosa and can
present with bleeding or perforation. Tubular duplications also are commonly asso-
ciated with fibrous cords to thoracic vertebrae, intraspinal cysts, and anomalies of
vertebral bodies reflecting their origins in the neurenteric canal.
Multiple Duplications
Defective neurenteric canal development may result in multiple duplications in
the same patient along the obliterated tract of the canal. Duplications have been
described crossing from the chest into the abdomen, communicating with the gall-
bladder, or replacing most of the bowel. Symptoms can be bizarre and initially puz-
zling until the embryological origins of these structures is understood.
Treatment
The treatment for symptomatic gastrointestinal duplications is surgical removal.
Small cystic duplications can be removed quite easily with the adjoining bowel.
Since intestinal duplications are situated on the mesenteric side of the intestine, it is
not possible to resect them without also compromising the blood supply to the
adjacent normal bowel. Removal of short segments of bowel is not usually a prob-
lem. Large esophageal duplications can be resected leaving the esophageal mucosa
intact, and closing the resultant muscular defect.
Long tubular duplications can also be removed, unless the amount of adjacent
bowel that has to be sacrificed is considered too much for the welfare of the patient.
In these cases, the seromuscular layer of the duplication can be opened and the
mucosa can be stripped from the entire length of duplication. The seromuscular
cuff can be resected and closed over the area of denuded epithelium. Resection can 63
be limited to the area where the duplication communicates with the intestine. Alter-
natively, the duplication and adjoining bowel can be anastomosed over a long length
to ensure free drainage.
Outcomes
The outcome after removal of most duplications is very favorable. When bowel
loss is kept to a minimum, the effect on absorptive capacity is negligible. Major loss
of bowel length occurs when the bowel is affected by very long tubular duplications,
or when diagnosis and treatment is delayed with resulting ischemic loss of bowel
from closed loop obstruction or intussusception.
Treatment of long tubular duplications through mucosal ablation and marsupi-
alization of the cyst remnant is usually successful. In some cases, despite best efforts,
the patient may be left an intestinal cripple and may require intestinal rehabilita-
tion, special enteric feeds and parenteral nutrition.
Neurologic damage from intraspinal duplications is often at least partially irre-
versible.
Mesenteric Cysts
Incidence
Like duplications, mesenteric cysts are very uncommon. At least 25% of all these
lesions occur in children less than 10 years old. Mesenteric cysts are responsible for
about 1 of every 15,000-20,000 admissions to pediatric hospitals.
Etiology and Classification
Mesenteric cysts have been classified in the past based on their presumed etiol-
ogy. Mesenteric cysts can result from infectious, neoplastic, traumatic, or abnormal
embryonic developmental processes. Congenital cysts are the most commonly
encountered mesenteric cysts in children and are better classified based on histo-
logic findings (Table 63.1). Approximately 90% of mesenteric cysts encountered in
the neonate are lymphangiomas. Mesothelial cysts are the next most common.
Table 63.1. Classification of mesenteric cysts by histology
Type of Mesenteric Cyst Histologic Findings

1. Lymphangioma Lined by endothelium
2. Mesothelial cyst Lined by mesothelium
3. Enteric cyst * Lined by enteric mucosa, no muscle
layers present *
4. Pseudocyst (nonpancreatic) Fibrous wall with epithelial lining.
* lack of muscle layer distinguishes it from intestinal duplication
Clinical presentation can be quite variable. Classically, children with mesenteric
cysts present with signs of partial bowel obstruction (pain, nausea, vomiting, anor-
63 exia, distention) and have a palpable, free-moving intra-abdominal mass. Occasion-
ally, volvulus with intestinal ischemia or infarction can occur. Symptoms may have
an acute onset if rapid enlargement of the cyst occurs secondary to hemorrhage. The
differential diagnosis of a mesenteric cyst includes ovarian cyst, choledochal cyst,
pancreatic cyst, enteric duplication, loculated ascites, renal or splenic cyst, hydro-
nephrosis, cystic teratoma, and hepatic or omental cysts.
Diagnosis
Radiographic studies are helpful to confirm the diagnosis. Abdominal
ultrasonagraphy helps localize cystic lesions within the abdomen and can determine
whether they are complex or simple, unilocular or multilocular, and single or multiple.
Fig. 63.1. Arrows demonstrate bowel path across the superior aspect of a large,
multiloculated mesenteric cyst.
Most mesenteric cysts are single and multilocular (Fig. 63.1). CT scan does not add
much additional information but occasionally can exclude other abdominal organs
as the source of the cystic mass.
Treatment
The treatment of mesenteric cysts is complete resection. Bowel resection may be
required in over 50% of children. If complete resection is not possible, partial exci-
sion with marsupialization of the remaining cyst remnant with sclerosis of the lining
may prove successful. Partial excision alone is associated with a high recurrence rate.
Outcomes
Recurrence rate after resection is around 6-13%. Mortality is extremely rare and
generally associated with cysts complicated by volvulus and ischemic bowel necrosis.
Selected Readings
1. Bond SJ, Groff DB. Gastrointestinal duplications. In: O’Neill Jr. JA et al, eds. 63
Pediatric Surgery, 5th edition. St. Louis: Mosby 1998; 1257-1267.
2. Grosfeld JL, OíNeill JA, Clatworthy HW. Enteric duplications in infancy and
childhood: an 18-year review. Ann Surg 1970; 172:83.
3. Norris RW et al. A new surgical approach to duplications of the intestine. J Pediatr
Surg 1986; 21:167.
4. Ricketts RR. Mesenteric and omental cysts. In: O’Neill Jr. JA et al, eds. Pediatric
Surgery, 5th edition. St. Louis: Mosby 1998; 1269-1275.
5. Chung MA et al. Mesenteric cysts in children. J Pediatr Surg 1991; 26:1306.
Section VIII: Peritonitis in Infancy
CHAPTER 1
CHAPTER 64
Necrotizing Enterocolitis
Incidence
The incidence of necrotizing enterocolitis (NEC) in the United States is 1-3
cases per thousand live births, or 25,000 cases per year. NEC is the most serious and
frequent disorder of low-birth-weight infants with an incidence of approximately
6% in infants below 1500 g. NEC is primarily, but not exclusively, a disease of
premature infants born in nations with well-developed neonatal intensive care sys-
tems. Among the most developed countries, the incidence of NEC is unequal with
the United States, Canada, United Kingdom, and Australia having the highest rates
of disease. NEC is rare in Switzerland, Scandinavia, and Japan.
Etiology
There is no single identified cause of necrotizing enterocolitis. Currently, there
are three main factors that are present and seem to contribute to the development
and progression of NEC in infants:
1. intestinal ischemia (thrombotic, embolic, or selective as in the diving
reflex),
2. bacterial colonization of the intestine, and
3. substrates in the gut lumen.
Prematurity is the major predisposing factor to NEC development. Premature
newborns have immature guts in which the gastrointestinal barrier defense mecha-
nisms are limited by inadequate production of mucus, complement, immunoglo-
bulins (i.e., IgA, IgM), and poor phagocyte function. Exposure to antibiotics,
pathogenic bacteria, and formula feeds create a luminal environment suitable for
bacterial overgrowth. With intestinal ischemia, bacteria breach the mucosal layer
and NEC begins. Many additional factors may contribute to the development of
NEC (Table 64.1).
Classification
Necrotizing enterocolitis follows a variable clinical course. The stages of NEC
are commonly classified as outlined in Table 64.2. However, clinical distinction
between each stage is often difficult.
Table 64.1. NEC associated factors or conditions

Umbilical catheters
Hypotension
Enteral feeds
Pneumonia
Maternal cocaine use
Hyperosmolar formula feedings
Vasoconstrictive medical therapy (indomethacin)
Patent ductus arteriosus
Table 64.2. Clinical classification of NEC and survival

Stage Clinical Radiographic Treatment Survival
Findings Findings
I: Emesis, mild Ileus pattern medical 100%

Suspected distention, evaluation,
64 NEC intolerance treat for NEC,
to feeds sepsis
evaluation.
II: Bilious emesis or Ileus, pneuma- aggressive
Definite gastric drain tosis intestinalis, medical 96%
NEC output, marked portal resuscitation
abdominal disten- vein gas and therapy
tion, occult or for NEC
gross GI hemorrhage
III: Bilious gastric Ileus, pneuma- Surgical 50%
Advanced output, abdominal tosis intestinalis,
NEC distention, occult portal vein gas,
or gross GI hemor- pneumo-
rhage, abdominal peritoneum,
wall erythema, ascites
deterioration of vital
signs, septic shock
NEC can involve any segment of the intestine, but the most commonly affected
region is the ileocecal area (45%) supplied by the most distal branches of the superior
mesenteric artery. Isolated small intestinal involvement is noted in 30% of cases.
NEC is limited to the colon in 25% of cases, and the splenic flexure is the most
common site of colonic involvement. Pan-necrosis (involvement of more than 75%
of the bowel) occurs in 14-30% of cases. NEC occurs as a single continuous lesion
in only about half of cases.
Grossly, the affected bowel is distended. The intestinal wall is thinned with hem-
orrhagic or graying areas. Subserosal or intravascular gas is observed in 50% of cases.
White colored bowel indicates areas of nonperfusion. Histologically, coagulation
Necrotizing Enterocolitis 287
necrosis of the mucosa is the predominant feature. Full or partial thickness involve-
ment of the subserosa and muscular layers is also common. Viable areas of bowel
demonstrate features of acute and chronic inflammation. Granulation tissue, fibro-
sis, and epithelial regeneration are signs of an extended duration of injury and recovery.
Although the clinical presentation can be quite variable, early NEC presents as
intestinal ischemia. An ileus is often present producing abdominal distention, tac-
hypnea, lethargy, feeding intolerance, gastric distention, and bilious or nonbilious
emesis. Gross or occult blood in the stool is identified in 25-55% of patients. As the
disease progresses, clinical indicators of shock and sepsis become evident including
temperature instability, increased lethargy, apnea, bradycardia, and oliguria. Increasing
oxygen requirement and a need for intubation and mechanical ventilation are also
signs of disease progression.
Abdominal exam is notable for distention, diminished bowel sounds, and ten-
derness. Initially the abdomen is soft but often become firm and increasingly tender
with erythema, discoloration, abdominal wall edema, and crepitance. Tympany on
abdominal percussion is frequently present in cases with perforation and free intra- 64
peritoneal air. Peripheral perfusion is diminished.
Laboratory testing reveals leukocytosis or leukopenia and thrombocytopenia that
occur as a response to gram negative bacterial septicemia. Metabolic acidosis occurs
as a result of sepsis as fluid lost into the interstitial space causes intravascular volume
depletion and hypoperfusion. Prothrombin (PT) and activated partial thrombo-
plastin time (PTT) are frequently prolonged due to disseminated intravascular co-
agulopathy (DIC).
Diagnosis
The diagnosis of NEC should be suspected in premature or low birth weight
infants with abdominal distention, an increasing need for respiratory support, feed-
ing intolerance, or lethargy. Initial evaluation of an infant suspected of having NEC
includes a careful physical exam, laboratory evaluation, and plain abdominal radio-
graphs. On a flat anterioposterior view, bowel distension is the earliest and most
common radiographic finding of NEC (Fig. 64.1). Intramural bowel gas (pneuma-
tosis intestinalis) occurs in almost all patients with NEC. However, pneumatosis
intestinalis is not a specific finding and has been reported in several other diseases
including Hirschsprung’s disease with enterocolitis, pyloric stenosis, and carbohy-
drate intolerance. Other plain film findings include portal venous gas, pneumoperi-
toneum, ascites, or fixed and persistently dilated bowel loops. Only 63% infants
with intestinal perforations due to NEC demonstrate pneumoperitoneum on pre-
operative abdominal films (Fig. 64.2).
Treatment
As is true for most diseases, the best treatment is prevention. Prenatal care helps
to decrease the number of premature births. NEC is rare among infants receiving
breast milk, when compared to formula fed infants in the neonatal intensive care
unit. The frequency of NEC in babies whose feeding schedule is advanced quickly is
not different from that observed in infants whose feedings are advanced more slowly.
64
Fig. 64.1. Extensive pneumatosis intestinalis consistent with severe necrotizing

enterocolitis.
Table 64.3. Medical management of NEC

Cultures: Blood cultures are necessary.
Urine/sputum/CSF as indicated
Orogastric or nasogastric decompression of stomach.
Intravenous fluid resuscitation to restore tissue perfusion and renal function
Antibiotics: synergistic coverage for gram positives and gram negatives, with
additional coverage for anaerobes. A penicillin, and amino-
glycoside, and either clindamycin or metronidazole are most
commonly used.
Correction of anemia and coagulopathy: transfuse packed red blood cells and
platelets. Fresh frozen plasma is used as coagulation parameters indicate.
Abdominal supine and gravity-dependent (cross-table or left lateral decubitus)
radiographs, repeated serially as clinical picture indicates.
Frequent repeat abdominal exams by the same physician at least every 6 hours
until infant stable, then as clinical picture indicates.
Surgical intervention if the infant worsens, fails to improve on intensive
nonsurgical therapy, or for advanced NEC with perforation and gangrene.
64
Fig. 64.2. Premature neonate (800 grams) with massive free air from necrotizing
enterocolitis. Arrows outline hepatic edge on decubitus radiograph.
Medical therapy is instituted immediately once the diagnosis of NEC is sus-

pected (Table 64.3). The goals of medical management include restoration of tissue
perfusion, control of infection or sepsis, and careful observation for evidence of
gangrene or perforation. An orogastric or nasogastric tube is placed to decompress
the stomach and diminish further gastrointestinal distention. Aggressive volume
resuscitation with isotonic fluids restores intravascular volume and helps improve
organ perfusion as indicated by reversal of hypotension, oliguria, and acidosis. Because
ischemic bowel sequesters large amounts of fluid in its walls and lumen, surprisingly
large resuscitation volumes are often required.
Systemic antibiotics for control of bacteremia are given, targeting broad cover-
age of enteric bacteria (i.e., gram-positive, gram-negative, anaerobic). Many centers
use ampicillin, gentamicin, combined with metronidazole or clindamycin. In some
institutions with a high prevalence of coagulase negative staphylococcus, vancomy-
cin is used to provide broader gram-positive coverage.
After initial resuscitation, surveillance includes serial abdominal exams with serial
evaluation for leukopenia, thrombocytopenia, anemia, acidosis, and hypoxia.
Abdominal films are repeated when there is a clinical change or increased suspicion
of gastrointestinal perforation. Indicators of intestinal gangrene or perforation and

potential need for operative intervention include:
1. an inability to resuscitate the infant,
2. subsequent deterioration of vital signs and hematologic indices (i.e., throm-
bocytopenia, leukopenia),
3. septic shock,
4. intestinal hemorrhage,
5. increasing ascites,
6. radiographic evidence of a persistent, fixed, dilated loop of intestine and
7. pneumoperitoneum.
Of these, pneumoperitoneum is probably the only absolute indication for surgi-
cal intervention.
The morbidity and mortality of laparotomy in septic, premature neonates is
high. The ideal timing of surgical intervention is often difficult to identify during
the progressive course of NEC; however, it is important to operate once it is clear
that a perforation has occurred. If nonsurgical therapy is ineffective after 4-6 hours
64 of intensive treatment, strong consideration for surgical intervention is warranted.
The main surgical options for an infant with NEC include:
1. peritoneal drainage,
2. laparotomy with resection and stoma(s),
3. laparotomy with resection and primary anastamosis,
4. laparotomy with proximal diversion, or
5. a combination of 1-4.
In cases of severe pan-necrosis, the surgeon (and family) may choose to explore
the abdomen and close with no further surgical intervention. In this scenario, sur-
gery is performed to confirm the diagnosis and to allow the provision of comfort
care to infants with no chance of survival. The choice of procedure is individualized
for each patient based on size, severity of illness, and presence of other comorbid
factors (i.e., intraventricular hemorrhage (IVH), etc.). Regardless of the procedure
chosen, great care is taken to minimize evaporative heat and water losses during
surgery (i.e., warming pads, plastic coverings, warmed fluids, and efforts to keep the
intestine inside the abdominal cavity whenever possible).
For laparotomy, the usual incision is a right-sided, transverse supraumbilical
incision which allows careful examination of the entire intestine. White-appearing
areas often represent full-thickness ischemic necrosis. Transparent areas of bowel
indicate areas of mucosal necrosis penetrating through to the muscularis and are at
high risk of perforation. Other areas of dull greenish-black or purple discolored
intestine may or may not recover. Bowel segments with questionable viability are
left in place and a “second look” operation is planned to re-evaluate the integrity of
these segments. Frankly necrotic segments require resection. If multiple necrotic
segments are resected, the surgeon must decide whether to bring out multiple sto-
mas and/or mucous fistulas or to create distal anastomoses. This decision depends
on the clinical status of the patient, the number of bowel segments, the individual
bowel segment lengths, the total length of remaining viable bowel, the presence/
absence of an ileocecal valve, and the ability of bowel ends to reach the anterior
abdominal wall. The length of the remaining viable intestine, its location, and the
presence or absence of the ileocecal valve are carefully noted in the medical record.
Peritoneal drainage is often performed in infants weighing less than 1000 gm
and in some larger infants who are physiologically unstable. A drain (e.g., penrose)
is placed into the peritoneal cavity usually via a right lower quadrant incision. Some
infants improve with this therapy and do not require emergent laparotomy.
Approximately one third of infants with NEC and weight < 1000 gm treated with
peritoneal drainage require no further surgical intervention. However, subsequent
exploration may be indicated if there is no improvement.
After recovery from a NEC episode, enteral feeding is introduced slowly after
about 10-14 days of medical therapy. Enteral feeds are slowly advanced toward goal
rates and concentration; nutrition is supplemented by intravenous hyperalimentation
(TPN) as needed. After recovery from NEC, 25% of infants develop intestinal stric-
tures resulting from circumferential scarring of nonperforated intestinal segments.
Enteral nutritional goals are frequently not met due to feeding intolerance related to
post-NEC stricture formation. Strictures are usually treated surgically by resection
and primary anastamosis well after the recovery phase and after significant growth
has occurred. 64
Infants with stomas are allowed to feed and grow well before these stomas are
closed. An arbitrary time or size such as two months or five kilograms is sometimes
used as a goal. Intestinal continuity is restored earlier if:
1. the intestinal segment proximal to the stoma is short causing failure to
thrive or difficult water and/or salt loss problems, or
2. stomal complications occur (i.e., stenosis, prolapse).
Patients with resections leaving insufficient absorptive intestinal length have short
bowel syndrome (SBS) (Chapter 95) and require long- term TPN. In some of these
infants, SBS is a temporary problem that resolves as intestinal length and diameter
increase with age, but central venous catheter infections and complications (i.e.,
cholestatic liver disease, etc.) are sometimes serious, life-threatening problems.
Outcomes
Overall survival from NEC is improving, especially in those infants weighing
less than 1000 gm. Overall survival has increased from near 50% in the 1980s to
approximately 80% in the 1990s. Early diagnosis and treatment are important. Infants
who progress to intestinal perforations have nearly a 65% perioperative mortality,
whereas infants without perforation at the time of surgery have a 30% mortality.
Survival rates for surgically treated NEC are similar between infants receiving lap-
arotomy and those receiving peritoneal drainage except in very low birth weight
infants (< 1000 gm) in which laparotomy results in only a 22% survival rate com-
pared to a 69% survival rate following peritoneal drainage.
Of infants surviving acute NEC, 25% will develop a late circumferential intesti-
nal stricture. Recurrent NEC occurs in about 6% of infants and typically occurs 3-5
weeks after the first episode. NEC also occurs as an infrequent postoperative com-
plication, most commonly following gastroschisis or myelomeningocele repairs. The
mortality rate of postoperative NEC is 46-67%.
Significant neurological impairment is observed in 15-30% of infants that sur-

vive NEC, but this rate is similar to the rate expected in premature hospitalized
infants of comparable size without NEC. Other gastrointestinal sequelae include a
10% incidence of short bowel syndrome (SBS) and malabsorption.
Selected Readings
1. Bell MJ, Ternberg JL, Feigin RD. Neonatal necrotizing enterocolitis; therapeutic
decisions based upon clinical staging. Ann Surg 1978; 187:1-7.
2. Rickets RR, Jerles ML. Neonatal necrotizing enterocolitis: experience with 100
consecutive surgical patients. Wld J Surg 1990; 14:600-615.
3. Ein SH, Shandling B, Wesson D, Filler RM. A 13 year experience with peritoneal
drainage under local anesthesia for necrotizing enterocolitis. J Pediatr Surg 1990;
25:1034-1037.
4. Morgan JL, Shochat SJ, Hartman GE. Peritoneal drainage as definitive manage-
ment of perforated NEC in the very low birth weight infant. J Pediatr Surg 1994;
29:30-34.
5. Mollitt DL, Golladay ES. Postoperative neonatal necrotizing enterocolitis. J Pediatr
Surg 1982; 17:757-763.
64 6. Grosfeld JL, Cheu H, Schlatter M et al. Changing trends in necrotizing entero-
colitis: experience with 302 cases in two decades. Ann Surg 1991; 214:300-307.
CHAPTER 1
CHAPTER 65
Gastrointestinal Perforation
in the Newborn
Daniel A. Bambini
Incidence
Spontaneous perforations of the gastrointestinal (GI) tract occur in the stomach,
duodenum, small intestine or colon. The problem is encountered only infrequently;
so, the exact incidence is unknown. Male infants are affected more often than females
(approx. 4:1). Although neonatal gastric perforation is a well-recognized entity, other
spontaneous GI perforations are much rarer and often incorrectly attributed to other
disease processes (i.e., necrotizing enterocolitis).
Etiology and Pathophysiology
Almost all spontaneous perforations of the GI tract are considered to be the
result of ischemic necrosis. The perforation is the end result of “selective circulatory
ischemia,” a defense mechanism of the neonate to hypoxia, physiologic stress, and
shock. Microembolic phenomena may also play a role. In response to physiologic
stress (hypoxia, hypovolemia, etc.), blood is selectively shunted away from mesen-
teric vessels to the more vital heart and brain. Local mesenteric ischemia can progress
to microvascular thrombosis and subsequent gastrointestinal wall necrosis and per-
foration. Although ischemia is likely the underlying problem, other factors includ-
ing bacterial colonization, hyperosmolar feeds, and an immature neonatal immune
system may also contribute. Indomethacin may also play a causal role in spontane-
ous GI perforations particularly in preterm infants as it does in the etiology of
necrotizing enterocolitis. Risk factors for neonatal gastrointestinal perforation include
all causes of severe fetal distress(abruption, emergent c-section, etc.).
Most infants with spontaneous gastrointestinal perforation present within the
first week of life (usually 4-5 days) with an abrupt onset of abdominal distention
and associated tachycardia, hypovolemia, and poor systemic perfusion. With severe
pneumoperitoneum, respiratory function is compromised requiring urgent intuba-
tion. Typically, the abdomen is markedly distended and tympanitic to percussion.
Pneumoperitoneum is usually present in these infants. The clinical course of neo-
nates with spontaneous gastrointestinal perforation may mimic those of NEC or
other diseases associated with perforation.
Diagnosis
The diagnosis is confirmed by plain abdominal x-rays (flat and decubitus views)
that demonstrate free intraperitoneal air. Additional laboratory evaluation includes
blood cultures, blood leukocyte and platelet counts, arterial blood gases, and serum
pH. Serial abdominal films are to be obtained if perforation cannot be demon-
strated initially but remains highly suspected.
Treatment
Treatment commences as soon as possible, simultaneous to the diagnostic work-
up. Rapid deterioration is anticipated and prevented with aggressive fluid resuscita-
tion, intravenous antibiotics, correction of acid-base disturbances, and nasogastric
decompression. Intubation and ventilatory support is required in infants with respi-
ratory distress. Aspiration of the massively distended pneumoperitoneum can be
helpful in infants with severe life-threatening respiratory compromise. Surgical
exploration is indicated. The site of perforation is identified although in up to 10%
of cases the perforation site has sealed spontaneously and cannot be identified. Sur-
gical treatment is dictated by the infants physiologic condition and the findings at
laparotomy (i.e., site of perforation, tissue condition, soilage, etc) and include pri-
mary repair, resection with external diversion, resection with anastamosis, drainage,
65 etc. Obstruction distal to the site of perforation is excluded whenever possible.
Outcomes
Survival in neonates with spontaneous gastrointestinal perforation is approxi-
mately 30-60%. Infants with isolated stomach perforations have the best overall
survival. The prognosis is adversely affected by prematurity, the presence of other
anomalies, and a delay in diagnosis.
Selected Readings
1. Grosfeld JL et al. Gastrointestinal perforation and peritonitis in infants and chil-
dren: Experience with 179 cases over ten years. Surgery 1996; 120:650.
2. Touloukian RJ. Gastric ischemia: the primary factor in neonatal perforation. Clin
Pediatr 1973; 12:219.
3. Rosser SB, Clark CH, Elechi EN: Spontaneous neonatal gastric perforation. L
Pediatr Surg 17:390.
4. Weinberg G, Kleinhaus S, Boley SJ. Idiopathic intestinal perforations in the new-
born: An increasingly common entity. J Pediatr Surg 1989; 24:1007-1008.
CHAPTER 1
CHAPTER 66
Neonatal Ascites
Incidence
Ascites in the newborn is an uncommon condition that can occur as a result of
three major medical problems. The exact incidence of ascites in the newborn is not
known. The most common surgical conditions that lead to accumulation of fluid
within the peritoneal cavity of the neonate are:
1. obstructive uropathy (urinary ascites),
2. spontaneous perforation of the biliary tree (bile ascites), and
3. lymphatic obstruction (chylous ascites).
Ascites from hepatocellular failure ( neonatal hepatitis, inborn errors of metabo-
lism, alpha 1- antitrypsin deficiency, biliary atresia, etc.) can occur in neonates but
more commonly occur in older infants and children.
Urinary Ascites
Etiology
Urinary ascites, the most common cause of neonatal ascites, usually occurs as a
complication of posterior urethral valves. Other conditions that cause urinary ascites
include:
1. ureteropelvic junction (UPJ) obstruction,
2. distal ureteral stenosis,
3. ureterocoele,
4. urethral atresia,
5. neurogenic bladder,
6. bladder neck obstruction,
7. ureterovessicle obstruction, and
8. spontaneous bladder rupture.
Urinary ascites rarely occurs in the absence of urinary tract obstruction.
Ascites occurs secondary to extravasation of urine from a perforation in the uri-
nary tract with accumulation of urine in the peritoneal cavity. Perforations, most
often in the upper tracts, are identifiable in about 64% and occur proximal to the
urinary tract obstruction. Rupture of a dilated renal pelvis or the renal parenchyma
allows a perirenal collection of urine to accumulate in the retroperitoneum. Perfora-
tion through the peritoneum allows the fluid to enter the peritoneal cavity.
Neonates with urinary ascites are mostly males (male:female 6:1). Gross abdominal
distension and ascites is usually present from birth. Abdominal distention is fre-
quently so severe that it causes respiratory distress. Other clinical features include
oliguria, hyponatremia, and hyperkalemia.
Diagnosis
Plain abdominal films demonstrate diffuse opacification with centrally located
“floating” intestines. The lower rib cage may appear widened. Contrast studies may
help to locate the site of extravasation. Computed tomography with intravenous
contrast or intravenous pyelography (IVP) may demonstrate extravasation of con-
trast into the perirenal space that produces the characteristic “halo” sign. In males
with posterior urethral valves, voiding cystourethrogram identifies the site of
extravasation and demonstrates the underlying cause of urinary ascites.
Paracentesis returns urine. A sample is sent to the laboratory for measurement of
creatinine, urea nitrogen, and potassium. All are elevated when compared to simul-
taneously obtained serum values.
Treatment
Large volume paracentesis is performed to relieve respiratory distress. Electrolyte
and metabolic imbalances are identified and corrected appropriately. The obstructed
urinary tract is decompressed by either nephrostomy or catheter drainage of the
66 urinary bladder depending upon the level of perforation. When the general condi-
tion of the patient is satisfactory, surgical correction of the underlying cause of uri-
nary obstruction is performed.
Biliary Ascites
Etiology
Neonatal biliary ascites is a rare condition that occurs most commonly second-
ary to spontaneous perforation of the extrahepatic bile ducts. The site of perforation
is most often at the junction of the cystic duct and the common bile duct. The
etiology of spontaneous perforation is not known. Viral infections, congenital mal-
formation, or weakness of the bile duct wall have been proposed as explanations,
but the current theory suggests that vascular compromise produces an area of
segmental ischemia at the junction. Rarely, distal biliary obstruction can lead to
secondary perforation of the biliary tree.
Bile leakage into the peritoneal cavity causes progressive abdominal distension
and accumulation of ascites. In some cases, the bile leak may lead to diffuse perito-
nitis with hypovolemia and cardiovascular collapse. In other cases, a pseudocyst
forms rather than generalized ascites.
Biliary ascites usually occurs between one week and three months of age. Bile
duct perforations are most frequent between the ages of 4-12 weeks. The clinical
course varies between a chronic, indolent disease to one with an acute, fulminating
Neonatal Ascites 297
illness progressing rapidly to shock and cardiovascular collapse. Usually, these infants
develop progressive abdominal distension, ascites, jaundice, and acholic stools.
Occasionally, biliary ascites becomes infected and the infant is acutely ill with jaun-
dice, fever, and sepsis.
Diagnosis
Abdominal paracentesis is useful to make the diagnosis. The ascitic fluid has a
markedly elevated bilirubin level. Hepatobiliary radioisotope scanning is an effec-
tive, accurate, and noninvasive method to make the diagnosis of biliary ascites. The
isotope will collect diffusely in the peritoneal cavity rather than in the duodenum
and small bowel.
Treatment
The treatment of biliary ascites is surgical. Surgical therapy includes intra-opera-
tive cholangiogram performed through the gallbladder wall to identify the leak and
possibly a site of biliary obstruction. Treatment options range from simple drainage
to biliary diversion procedures. If the bile duct is not obstructed and simple drain-
age is selected, cholecystostomy is performed to provide access to the biliary tree for
postoperative study. The drains remain in place until a cholangiogram demonstrates
no leak or obstruction. Roux-en-Y or other biliary reconstruction is necessary in
cases of distal bile duct obstruction.
Chylous Ascites 66
Etiology
Chylous ascites is caused by lymphatic obstruction. The source of lymphatic
obstruction can be:
1. congenital malformation of the lymphatic ducts (39%),
2. trauma,
3. occlusion of mesenteric lymphatics by extrinsic forces (i.e., malrotation,
peritoneal bands, incarcerated hernia),
4. injury to the cysterna chyli,
5. inflammation (5%), and
6. neoplasm (3%).
Congenital malformations of the lymphatics that lead to ascites include lacteal
or cisterna chyli atresias, mesenteric cysts, and lymphangiomatosis. However, the
exact cause of chylous ascites in many neonates is never known with certainty.
Lymphatic fluid accumulates in the peritoneal cavity causing progressively
increased abdominal distention and girth. Chylous ascites becomes milky white in
color after oral feeding is initiated due to high fat content.
Most of these infants present with abdominal distention and ascites at birth or
within the first days of life. If a mechanical occlusion of lymphatics is present (i.e.,
malformation, etc), there may be symptoms of intestinal obstruction. Peripheral
lymphedema of the extremities is present in 10% of newborns with chylous ascites.
Diagnosis
Abdominal x-rays demonstrate an opaque, fluid-filled abdomen with centrally
located intestines. Paracentesis obtains fluid that has a high triglyceride content
(> 1500 mg/dl) and high lymphocyte count (differential 70-90% lymphocytes) con-
firming the diagnosis.
Treatment
Chylous ascites in the newborn is usually managed nonoperatively. The majority
of patients respond to gastrointestinal rest and central hyperalimentation. An enteral
diet that is high in protein and contains medium-chain triglycerides is often helpful
to control chylous ascites. If the ascites is severe, progressive, intractable, and com-
promises respiration, laparotomy is performed to exclude correctable situations. If
no fixable lesion is identified, a peritoneovenous shunt can be used successfully to
control ascites.
Outcomes
The outcome from treatment of neonatal ascites depends heavily upon the
underlying etiology. Urinary ascites responds well to correction of the underlying
urologic problem. The mortality from urinary ascites is near zero. Similarly for bil-
iary ascites, external drainage is effective in greater than 80% and no additional
surgical intervention is required. Eighty percent of bile duct perforations heal within
2-3 weeks. Unfortunately, chylous ascites does not respond so favorably. For chylous
66 ascites, nonsurgical management is successful in 60-70% of patients. The death rate
from chylous ascites is about 25-30%.
Selected Readings
1. Lilly JR, Weintraub WH, Altman RP. Spontaneous perforation of the extrahepatic
bile duct and bile peritonitis in surgery. Surgery 1974; 75:664-673.
2. Stringel G, Mercer S. Idiopathic perforation of the biliary tract in infancy. J Pediatr
Surg 1983; 18:546.
3. Unger SW, Chandler JG. Chylous ascites in infants and children. Surgery 1983;
93:455.
4. Mann CM, Leape LL, Holder TM. Neonatal urinary ascites: a report of 2 cases of
unusual etiology and a review of the literature. J Urol 1974; 111:541.
Section IX: Jaundice in Infancy and Childhood
CHAPTER 67
Biliary Atresia
Riccardo Superina
Extra hepatic biliary atresia (EHBA) is an acquired disorder of the bile ducts. It
affects babies in the first month of life. As its name implies, it is a progressive oblitera-
tive process primarily involving the extra hepatic bile ducts. The onset of the disease
is heralded by progressive jaundice which is often mistaken for hemolytic jaundice
of the newborn or a breast milk induced cholestasis. It is the most common cause of
obstructive jaundice in the newborn period.
Incidence
Worldwide, biliary atresia occurs at a rate of approximately 0.8-1.0 per every
10,000 live births. Females are slightly more affected than males (female:male ratio
near 1.4:1). There are no apparent racial differences in the incidence of this disease.
Etiology
The etiology of EHBA is uncertain. There is evidence which supports a viral
etiology, including the presence of giant cells and electron microscopic appearance
of viral like particles in some studies. EHBA may be a part of a more global develop-
mental disorder which is suggested by the frequent presence of associated anatomi-
cal abnormalities. The more frequent of these include venous anomalies such as
preduodenal portal vein, interrupted vena cava and azygous continuation of the
portal vein. Other commonly associated anomalies include polysplenia, situs inversus
abdominis, and intestinal rotational anomalies. Occasionally, it has been associated
with congenital absence of the portal vein. An ischemic intrauterine event which
causes progressive obliteration of the extra hepatic biliary tree has been proposed as
a mechanism for the occurrence of so called “correctable atresia”.
The obliterative process which affects the extra hepatic ducts does not necessar-
ily affect all the extra hepatic ducts at the same rate. The gall bladder can remain
patent and communicate with an abnormal albeit patent distal duct in continuity
with the duodenum, or with a patent proximal duct connected to the intrahepatic
biliary tree. This does not imply that the patent ducts are normal, but that the
process, which is a progressive one, has not yet reached completion.
Jaundice with acholic stools during the first month of life are the most common
presenting symptoms. There is rarely any other significant medical history. On
examination, scleral icterus and jaundice are usually obvious. Stools are pale and
Biliary Atresia 301
colorless. The urine is usually dark. The liver may be enlarged and indurated if the
diagnosis is made beyond the first eight weeks of life. Splenomegaly is another com-
mon physical finding but generally occurs quite late in the clinical course. Another
late sign of EHBA is the appearance of enlarged abdominal veins signaling the onset
of portal hypertension.
Jaundice secondary to EHBA in the newborn period is often mistaken for other
less ominous reasons for hyperbilirubinemia. Parents may be reassured that the jaun-
dice is a normal physiological phase even though the time is well beyond what would
be considered an acceptable time period. ABO antigen incompatibility and “breast
milk” jaundice are the two most common diagnostic errors. Diagnostic inaccuracy
is increased when a baby has a normal period of unconjugated hyperbilirubinemia
that persists beyond what would normally be considered acceptable. Any jaundice
in a baby beyond 14 days of life should not be discounted as “physiologic”.
Diagnosis
Liver function tests show a conjugated or direct hyperbilirubinemia with a nor-
mal or slightly raised unconjugated fraction. Transaminase levels also show moder-
ately raised values, usually above 100 IU/dL. Serum GGTP levels are elevated far
above normal levels, consistent with an obstructive jaundice.
Ultrasonography is the principle radiologic test used to evaluate persistent neo-
natal juandice. In EHBA, ultrasound findings are often limited to identifying a
hypoplastic or absent gallbladder. In the early stages of the disease, before complete
obliteration of the extra hepatic ducts, cystic lesions may be identifiable in the area
of the hepatic hilum, leading to the erroneous diagnosis of a choledochal cyst. The
spleen may be enlarged, and the liver consistency often shows increased echogenicity 67
secondary to fibrosis. At times, ultrasonography may show no identifiable abnor-
malities.
Radionucleide imaging studies (HIDA or DESIDA) scans show failure of tracer
excretion into the intestine after 24 hours. Failure of excretion may also be seen in
cholestatic conditions such as severe neonatal hepatitis, but when it is found in
combination with the appropriate clinical and biochemical profile, it is a very accu-
rate predictor of biliary atresia. Phenobarbital given for 5 days prior to the scan may
decrease the false negative rate.
A liver biopsy is an extremely accurate way of diagnosing EHBA. Typical find-
ings include bile plugging in major bile ducts, bile ductule proliferation, and increased
fibrosis (see Pathology). When all signs point to EHBA, the diagnosis is confirmed
by open biopsy and cholangiogram. If the diagnosis of EHBA is entertained early,
many of the diagnostic radiological and histological features may not be as well
defined. Careful observation and early repetition of the tests may be necessary. Liver
biopsy early in the course of the disease may show more inflammatory than cholestatic
changes and may be mistaken for neonatal hepatitis. Therefore, whenever the diag-
nosis is considered, it must be confirmed with operative cholangiogram.
Pathology
Histologically, EHBA is characterized by bile ductule proliferation, fibrosis origi-
nating in the portal triads, and bile plugging in the major ducts. In advanced cases
there may be evidence of cirrhosis. The process may also be accompanied by a lobular
inflammatory response resulting in the formation of giant cells, syncytial giant cells,
and in more severe cases, bridging hepatocyte necrosis.
EHBA can be classified into two main types: correctable or uncorrectable. Cor-
rectable atresia (uncommon) can be characterized as an intact intrahepatic biliary
tree and a short segment of dilated extrahepatic duct that communicates with the
bile ducts in the liver but comes to an abrupt stop. Uncorrectable atresia is more
common and characterized by progressive sclerosis and obliteration of the intra- and
extrahepatic biliary tree. No recognizable bile ducts can be discerned at the time of
exploration.
Treatment
The only treatment possible is a surgical attempt at reestablishing bile drainage
into the intestine. If the diagnosis is the correctable type of atresia, then a simple
choledochoenterostomy establishes long term bile drainage. If exploration and
operative cholangiography confirms ‘uncorrectable’ biliary atresia, the usual course
of action is to proceed with dissection of the liver hilum in order to transect proxi-
mal ductules which may still be patent and drain bile. This procedure is called the
Kasai procedure after Morio Kasai who first described this dissection for the treat-
ment of uncorrectable atresia. The fibrotic tissue, which may still contain remnants
of the extra hepatic biliary tree, is dissected off the portal vein and hepatic arteries
and followed up to the portal plate where it is transected. A loop of intestine (usually
Roux-en-Y) is then brought to the hilum of the liver and fixed to the capsule of the
67 liver (portoenterostomy). Following portoenterostomy, ursodeoxycholic acid is often
started to promote bile secretion and fat-soluble vitamin supplementation is begun.
Outcomes
Unfortunately, the Kasai procedure is not a cure for biliary atresia. In approxi-
mately one third of cases, no improvement in bile drainage is seen, and progressive
liver dysfunction ensues. In most of these cases, liver failure or death from complica-
tions of portal hypertension takes place between the ages of one and two years.
In one third of babies, bilirubin returns to normal. Liver function tests, however,
continue to demonstrate a cholestatic picture with a modest but persistent abnor-
mality in serum transaminase values. Serum bile acids are also elevated above nor-
mal values. These children may demonstrate relatively normal growth and
development for indefinite periods of time. Portal hypertension may develop despite
normal synthetic hepatic function and has to be addressed if complications develop.
One third of children have an improvement in serum bilirubin after
portoenterostomy but jaundice persists. These children may have a few years of
relative good health, but liver function deteriorates progressively and death ensues
from liver failure in early childhood.
Cholangitis is a frequent complication following portoenterostomy and requires
admission and treatment with broad-spectrum intravenous antibiotics. Frequent
episodes of cholangitis may cause deterioration in children who have achieved good
biliary drainage.
Biliary Atresia 303
When bile drainage after successful portoenterostomy stops, re-exploration has

been advocated to try and re-establish bile drainage. This approach has lost propo-
nents in the era of liver transplantation because of the low chances of prolonged
benefit and causes increased scarring which may further complicate transplantation.
Liver transplantation has become the treatment of choice in children with failed
Kasai operations. The timing of referral for transplantation is a critical issue to ensure
that children with unsuccessful operations receive transplants in a timely manner.
Selected Readings
1. Kasai M et al. Surgical treatment of biliary atresia. J Pediatr Surg 1968; 3:665.
2. Altman RP: The portoenterostomy procedure for biliary atresia: a five-year experi-
ence. Ann Surg 1978; 188:357.
3. Vazquez-Estevez et al. Biliary atresia: early determination of prognosis. J Pediatr
Surg 1989; 24:48.
4. Okazaki T et al. Long-term postsurgical outcome of biliary atresia. J Pediatr Surg
1999; 34:312-315.
5. Bates MD et al. Biliary atresia: pathogenesis and treatment. Semin Liver Dis 1998;
18:281-293.
6. Ryckman FC et al. Biliary atresia: surgical management and treatment options as
they relate to outcome. Liv Transpl Surg 1998; 4:S24-33.
67
CHAPTER 68
Choledochal Cysts
Riccardo Superina
Incidence
Choledochal cysts are one of the more common bile duct anomalies in children
and occur in approximately one in 10,000 live births. Racial and geographic differ-
ences exist. The malformation is more common in areas of Asia, notably Japan. It is
also more common in girls.
Etiology
The cause of choledochal cysts is uncertain and several theories have been pro-
posed. Choledochal cysts have been associated with a long common channel of the
terminal bile duct and pancreatic duct, as visualized on transhepatic or retrograde
studies of the common duct. A long common channel is defined as a junction of the
two ducts at least one centimeter prior to the sphincter in the wall of the duodenum.
This theory implies pancreatic juices with their proteolytic enzymes reflux into the
distal common bile duct and weaken the integrity of the duct tissue leading to pro-
gressive dilatation and cyst formation. Common channels, however, are not detect-
able in all cases.
A genetic basis for the disease is possible or even likely since choledochal cysts
occur in concert with other organ system dysfunction. Cysts are commonly identi-
fied in patients with congenital hepatic fibrosis, intrahepatic biliary cysts, and poly-
cystic kidney disease.
Some choledochal cysts may occur as a result of congenital duct wall anomalies.
It is likely that isolated weakening in the common bile duct wall from ischemic
events can lead to duct wall outpouching. Choledochoceles may result from abnor-
mal development of the hepato-biliary bud in the embryonic foregut.
Classically, choledochal cysts present with jaundice, right upper quadrant pain
and a palpable mass. Pain is usually the predominant symptom. Jaundice with serum
bilirubin levels in the 2-5 mg/dl range is also common. A mass is rarely palpable.
The serum amylase or lipase levels may also be elevated in children presenting with
acute abdominal pain and choledochal cysts.
Asymptomatic choledochal cysts are being identified on a more regular basis as
ultrasound examination of the abdomen has become a more frequently used modal-
ity for the investigation of numerous symptoms. Children with polycystic kidney
disease should have ultrasound examination of the biliary tree to rule out small or
Choledochal Cysts 305
asymptomatic choledochal cysts. Prenatal ultrasound examination can also detect

cystic masses near the liver in the fetus. Infants with prenatally diagnosed chole-
dochal cysts can be examined and evaluated in more detail after birth.
Diagnosis
If a choledochal cyst is suspected, the diagnosis is confirmed by blood chemistry
studies and abdominal ultrasonography. All liver function tests are elevated during
periods of acute pain: direct hyperbilirubinemia, elevated GGT (gamma-
glutamyltransferase), and modest elevation of transaminase values. Serum amylase
and lipase can be elevated if there is an accompanying pancreatic inflammation.
Abdominal ultrasound is the imaging test of choice for making the diagnosis of
choledochal cyst. A cystic dilatation of the common bile duct and gall bladder is the
most common finding. The dilatation can extend into the common hepatic duct,
but typically does not extend into the hepatic ducts.
Idiopathic pancreatitis with secondary biliary duct dilatation can be confused
with choledochal cysts. Continued observation in cases of pancreatitis will often
demonstrate resolution of the ductal dilatation, whereas true choledochal cysts will
not resolve spontaneously.
Pathology
Examination of the cyst wall demonstrates chronic inflammatory changes and
often complete denudation of the epithelium. In undiagnosed or incorrectly treated
cysts, chronic epithelial inflammation rarely leads to malignant degeneration.
Cholangiocarcinoma has been reported in choledochal cysts first diagnosed in adults
and in cyst remnants not resected after childhood diagnosis. The usual malignancies
are adenosquamous carcinoma or small cell carcinoma.
Classification 68
Type 1
This is by far the most common form of cyst (Fig. 68.1). It involves most of the
common bile duct, cystic duct, gall bladder and common hepatic duct. Type 1 cho-
ledochal cysts do not typically extend into the hepatic ducts. They frequently com-
municate with the duodenum through a lumen so small that it can barely be perceived
at surgery. These cysts are sometimes subclassified as either cystic (Type 1c) or fusi-
form (Type 1f ).
Type 2
This type of cyst is an outpouching or diverticulum of the common bile duct,
involving all layers of the duct wall. It typically involves a short segment of an oth-
erwise normal duct. It does not affect the gallbladder. It is extremely rare.
Type 3
This type is called a choledochocele and is located at the distal end of the com-
mon duct. Often it is completely contained within the duodenal wall. It causes
obstruction through compression of the normal duct and is sometimes considered a
duplication cyst. The rest of the ductal system is normal.
68
Fig. 68.1.Demonstration of the most common type of choledochal cyst (type 1): a
fusiform dilation of the common and common hepatic ducts with only minor dila-
tion of the intrahepatic ducts.
Type 4
This cystic lesion involves both the intrahepatic and extrahepatic biliary system.
The intrahepatic portion consists of areas of normal ducts interspersed with areas of
saccular dilatation.
Choledochal Cysts 307
Type 5
Type 5 lesions are single or multiple intrahepatic biliary cysts. There is no extra-
hepatic component in this type. The intrahepatic biliary cysts may be localized to
one lobe or segment, but it is usually a bilateral, diffuse process. Type 5 (and some-
times Type 4) lesions are often called Caroli’s disease, in which there are multiple,
irregular segmental dilatations of the intrahepatic bile ducts. Caroli’s often occurs in
association with hepatic fibrosis and polycystic kidney disease. Cirrhosis and portal
hypertension can develop in both type 4 and 5 lesions.
Treatment
Treatment consists of cyst excision and biliary reconstruction with a Roux-en-Y
choledochojejunostomy for all type 1 and type 4 cysts. In the past, anastomosis of a
loop of bowel to either the gallbladder or cyst wall to re-establish bile drainage was
considered adequate treatment. The realization that malignancies can develop in
cyst remnants has led to the recommendation that as much of the cyst as can be
safely removed be resected.
For type 2 cysts, an attempt at simple resection should be made with primary
repair of the common duct. Because of the small size of the duct, postoperative
strictures may complicate the recovery, but these can usually be successfully dilated.
Type 3 cysts are resected via a transduodenal approach. Alternatively, marsupial-
ization of the cyst into the duodenum is another acceptable treatment if the cyst
cannot be resected without causing further damage to the distal common duct.
Leaving cyst wall remnants in type 3 cases does not seem to have the same potential
for malignant degeneration as for type 1 cysts.
Type 5 (and some type 4) lesions pose a more difficult treatment problem. For
intrahepatic cysts localized to one lobe or segment, hepatic resection is only rarely
beneficial. Many of these patients may eventually require liver transplantation (see 68
below).
For cases complicated by severe cholangitis and advanced inflammatory changes,
cyst drainage and biliary decompression either operatively or with the assistance of
an interventional radiologist may be necessary before any attempts at cyst resection.
Prophylactic cystectomy may sometimes be necessary. In patients with polycys-
tic disease and renal failure, dealing with the cyst before renal transplantation may
be wise. Development of cholangitis is more serious in an immunosuppressed host,
and cyst excision before transplantation all but eliminates that possibility.
Prenatal diagnosis of choledochal cysts is becoming more common. Cysts rarely
cause problems in the newborn period, and therefore it is almost never necessary to
operate right after birth. A period of 4-6 weeks for observation is usually a good
idea. Cysts may regress, and there is no significant risk for an acute cyst-related
complication. Additionally, a waiting period allows the baby to grow and may lower
anesthetic and surgical risks.
Outcomes
Type 1 cysts completely excised have an excellent prognosis. Periodic evaluation
of liver function tests and ultrasonographic examination of the biliary tree should be
performed for the first 5 years as indicated. If everything appears normal, only rarely
is additional follow-up testing needed. Type 2 and 3 cysts may have complications
of biliary strictures in the short-term postoperative period. However, long-term out-

look is excellent.
The outcome for type 4 and 5 cysts is much more guarded. These children will
suffer from intermittent bouts of cholangitis and experience progressive fibrosis of
the liver. Ultimately, liver failure may ensue, and liver transplantation may be indi-
cated. Further palliative surgery to improve intrahepatic strictures is rarely indi-
cated. Radiological dilatations of dominant strictures may add years of life to children
with these difficult problems and may delay the need for transplantation.
In patients with congenital hepatic fibrosis and choledochal cysts, long term
problems related to portal hypertension and liver failure are likely to develop. Cys-
tectomy may, however, significantly add to the quality of life as well as provide
improved bile drainage to decrease the risk for development of biliary cirrhosis.
Selected Readings
1. Todani T, Watanabe Y, Narusue M et al. Congenital bile duct cysts. Classification,
operative procedure and review of 37 cases including cancer arising from a chole-
dochal cyst. Am J Surg 1977; 134:263-269.
2. Caroli J. Diseases of the intrahepatic bile ducts. Israel J Med Sci 1968; 4:21-35.
3. Chijiiwa K, Tanaka M. Late complications after excisional operation in patients
with choledochal cyst. J Am Coll Surg 1994; 179:139.
4. Tugo-Vicente HL. Prenatally diagnosed choledochal cysts: observation or early
surgery. J Pediatr Surg 1995; 30:1288.
68
Section X: Respiratory Distress
CHAPTER 69
Upper Airway Obstruction in the Newborn

Daniel A. Bambini
The principle features of upper airway obstruction in infants are stridor and
cyanosis. Neonatal airway obstruction most commonly originates in the larynx but
may be caused by nasal or postnasal lesions, pharyngeal pathology, or narrowing of
the trachea or major bronchi. Respiratory distress in the newborn is an acute emer-
gency and requires rapid assessment and treatment. This Chapter discusses the fea-
tures of upper airway obstruction in the neonate.
Incidence
The exact incidence of upper airway obstruction in the newborn is difficult to
estimate due to the large array of conditions that may cause this problem. The inci-
dence of some of the more common etiologies are listed in Table 69.1.
Etiology
Airway obstruction can occur at any level from the nose to the tracheobronchial
tree. Table 1 lists the common etiologies by level of obstruction. A full discussion of
the embryologic, genetic, and anatomic basis of each lesion is beyond the scope of
this Chapter. Some lesions are discussed in detail within other Chapters of this book.
Newborn infants are obligatory nose breathers. Conditions that obstruct the
nasal passages or pharynx cause obstructive apnea. Supraglottic lesions tend to cause
inspiratory stridor, while those of the trachea or bronchi cause an expiratory or
biphasic stridor. Lung lesions that cause respiratory distress usually do not produce
obstructive signs. Although the severity of stridor can be mild and self-limited in
many instances, severe airway obstructions may be lethal or cause hypoxic brain
injury.
Stridor is the sound produced as air flows across a narrowed airway. As air flows
through a tube, the lateral forces holding the tube open decrease (Venturi principle).
At narrowed segments, the walls may collapse and touch causing vibration of the
walls which is acoustically appreciable as a stridor. The pitch of the stridor is depen-
dent more upon the thickness of the vibrating wall than the anatomic level of
obstruction.
Upper Airway Obstruction in the Newborn 311
Table 69.1. Causes of airway obstruction in the newborn

Nose and Oropharynx
Nasal agenesis or atresia

Traumatic nasal deformities (septal hematoma or
dislocation, nasal fracture, etc
Stuffy nose syndrome (turbinate hypertrophy)
Choanal atresia (unilateral or bilateral)
Nasopharyngeal mass (encephaloceles, teratoma,
glioma,adenoids
Oral Cavity and Oropharynx
Craniofacial abnormalities (Apert’s, Crouzon’s,

Treacher-Collins, etc.)
Micrognathia with glossoptosis: Pierre Robin Sequence
Treacher-Collins syndrome
Macroglossia (hypertrophy, tumor, lingual thyroid, thyro-
glossal duct cyst)
Oropharyngeal tumors (dermoid, epignathus or teratoma,
tongue duplication, etc.)
Hypertrophy of tonsils or adenoids
Peritonsilar or retropharyngeal abscess/mass
Larynx
Laryngomalacia
Vocal cord paralysis
Congenital or acquired subglottic stenosis
Laryngeal webs (glottic, interarytenoid) or atresia
Neoplasms (hemangioma, lymphangioma, papillomatosis)
Congenital laryngeal cleft
Laryngeal trauma from endotracheal intubation
Tracheobronchial Tree
Tracheomalacia
69
Vascular rings and slings
Innominate artery compression syndrome
Tracheoesophageal fistula with esophageal atresia
(secondary tracheomalacia)
Tracheal agenesis, webs, and stenosis
Compression from bronchogenic cyst, esophageal
duplication, sequestration, etc
The clinical presentation of an upper airway obstruction in a neonate is propor-
tional to both the degree and anatomic level of the obstructing lesion. Cyanosis and
severe respiratory distress (dyspnea, retractions, agitation, wheezing, stridor) are the
hallmark signs of upper airway obstruction but are also common to the presentation
of other lesions of the pulmonary, gastrointestinal, and cardiovascular systems. The
first indication of a potential airway problem may be “noisy respirations” observed
by the family or nurses in the neonatal unit. The onset and duration of stridor
should be documented and information regarding possible trauma, relationship to
feeding, possible aspiration, or congenital malformation is noted.
Stridor that is present from birth is most often caused by congenital
laryngomalacia. Considerably less likely are subglottic stenosis, vocal cord paralysis,
or a vascular ring. The stridor associated with a vocal cord paralysis is frequently
louder when the infant is awake.
Progressively increasing stridors may be secondary to neoplastic lesions causing
gradual compromise of the airway. Subglottic hemangiomas usually occur between
1 and 3 months of age (85% before 6 months) and 50% of these infants will have
associated skin hemangiomas.
Diagnosis
After careful history and physical examination, radiographic evaluation of the
upper airway is indicated. Anteroposterior and lateral plain cervical radiographs are
obtained to view the soft tissues of the neck and chest. The films are obtained dur-
ing inspiration if possible. Fluoroscopy is useful to localize the level of airway
obstruction if inspiratory films are not obtainable. Barium swallow allows assess-
ment of the pharynx for obstructing lesions and can identify vascular rings encroach-
ing on the esophagus and/or trachea. Ultrasound can be useful to identify vocal cord
paralysis. Computed tomography and magnetic resonance imaging are occasionally
used to localize and assess size of soft tissue lesions and aberrant vessels.
Endoscopy (flexible nasolaryngoscopy, fiberoptic or rigid bronchoscopy,
esophagoscopy) confirms the diagnosies suggested by radiologic studies. The phar-
ynx, trachea, larynx, vocal cords, and bronchi are carefully visualized. Supraglottic
airway collapse, vocal cord paralysis, and laryngomalacia are all best visualized in
spontaneously breathing infants. In neonates with stridor, the endoscopic evalua-
tion is most safely performed in the operating room. Resuscitation equipment, small
endotracheal tubes (2.5-3.5 mm), and tracheotomy instruments should be immedi-
69 ately available.
Treatment
Prior to administering anesthesia, the surgeon caring for the infant with upper
airway obstruction must select proper sized equipment (bronchoscope, etc.) and be
absolutely sure that all necessary equipment (suction, lenses, etc.) is functional. After
careful laryngoscopy, a ventilating bronchoscope is passed through the glottis under
direct vision. The identification of the lesion and its location and the ability to pass
a bronchoscope are key determinants for the ability to perform safe endotracheal
intubation. If bronchoscopy cannot be performed (tracheal diameter < 2.5 mm),
tracheostomy or anterior cricoid split may be necessary. After intubation, other en-
doscopic procedures (esophagoscopy, nasopharyngoscopy, etc.) can be performed to
evaluate for masses, fistulas, or foreign bodies. Postoperative care after bronchos-
copy should include close observation for possible delayed swelling and/or need for
intubation. Racemic epinephrine nebulizers and dexamethasone may help prevent
delayed airway swelling.
Upper Airway Obstruction in the Newborn 313
The definitive treatment of upper airway obstruction is guided by the underly-

ing etiology. A full discussion of all lesions is beyond the limits of this Chapter.
Selected Readings
1. Holinger LD. Upper airway obstruction in the newborn. In: Raffensperger JG ed.
669-682.
2. Holinger PH. Neonatal respiratory tract obstruction. Cardiopulmon Dis 1962;
4:285.
3. Benjamin B. Airway obstruction: Part 1. Laryngeal Obstruction. In: Freeman NV
et al, eds. Surgery of the Newborn. Edinburgh: Churchill Livingstone 1994;
409-424.
69
CHAPTER 70
Vascular Rings
Robert M. Arensman
Vascular rings and slings are a series of anatomical variations in the formation of
the aortic arch, ductus arteriosus or pulmonary artery that can produce vascular
compression of the trachea or esophagus.
Incidence
These anomalies are reasonably rare and may go undetected in the general popu-
lation since many are asymptomatic. The aberrant right subclavian artery is the
most common sling, possibly present in 1:200 individuals. The other sling (aberrant
pulmonary artery sling) and two most common forms of rings (double aortic arch,
right arch with left ductus) are seen much less frequently and present for surgical
correction only a few times each year, even at large metropolitan children’s hospitals.
Etiology
The cause of branchial vessel persistence or malformation is not identified, but
all rings and slings represent some variation on maldevelopment of branchial vessels
three, four and six. The double aortic arch forms a complete vascular ring and results
from persistence of the double fourth branchial vessels. In a right aortic arch with
left ductus arteriosus, the right fourth arch vessel persists rather than the left yet the
ductus forms normally, encircling the trachea and esophagus.
In the aberrant pulmonary artery sling, the sixth arch vessel develops abnor-
mally, passing over the right main bronchus between the trachea and esophagus.
Normally the pulmonary artery passes in front of both and divides into its right and
left branches anterior to the trachea. In cases of aberrant right subclavian sling, the
third branchial vessel fails to form the typical innominate vessels. Instead, the right
subclavian artery arises directly from the descending aorta, passes to the right and
behind the esophagus, or very rarely between trachea and esophagus.
Children, 1-2 months to 2 years of age, present with biphasic stridor and/or
dysphagia (dysphagia lusoria). Dysphagia occurs much less commonly than stridor.
Onset of symptoms is often insidious. As the child grows, the trachea and/or the
esophagus are gradually compressed producing and then worsening the symptoms.
Examination reveals stridor that is often worse with agitation or anger. Minimal
respiratory infections often exacerbate the problem and dramatically worsen symp-
toms. An affected child may have respiratory retractions and use accessory muscles
Vascular Rings 315
to breathe. Signs of thinness, malnutrition or frank failure to thrive may be present

if dysphagia and vomiting are severe.
Severe respiratory infections, croup, obstructing airway lesions, and gastroesoph-
ageal reflux are the most commonly considered problems in the differential diagno-
sis. The more bizarre forms of rings and slings have a high incidence of congenital
cardiovascular anomalies that should be identified prior to an attempt at repair.
Diagnosis
Severe and recurrent tracheal/esophageal symptoms indicate the need for chest
x-ray and barium esophagram (Fig. 70.1). A characteristic set of indentations,
narrowings, and deviations allow almost all these anomalies to be diagnosed with
these two radiographic studies.
Arteriography is avoided because it is highly invasive, can permanently damage
tiny arteries, requires dyes and radiation, and is largely unnecessary. However, mag-
netic resonance imaging (Fig. 70.2) and computed tomography with contrast are
now frequently used and provide excellent delineation of the anatomy without inva-
sion and with much less radiation.
Endoscopy can be done at the time of operative correction to document the
degree of tracheomalacia but is not needed for diagnosis or therapy.
Pathophysiology
Tracheal compression producing tracheomalacia and airway compromise explains
the problem. Interestingly, the esophageal compromise is much less commonly seen,
and most children continue to eat and thrive long after the stridor appears.
Rarely, neonates present with bilateral pulmonary hyperinflation (air block syn-
drome). These infants have sufficient compression to prevent normal expiration and
develop a pattern of obstruction that looks like bilateral extensive congenital lobar
emphysema. Intubation with tube placement below the ring will usually stabilize
these neonates until surgical division is accomplished.
Treatment
If a vascular ring or sling presents with symptoms, surgical repair is indicated.
Preoperative management entails sufficient study to define the anatomy correctly 70
and obtaining blood for type and cross-match.
Left thoracotomy is the normal approach for all these lesions except the aberrant
left pulmonary artery sling which is approached via median sternotomy. For the
vascular rings, division of the nondominant ring and ductus or the ductus alone
usually suffices. The aberrant left pulmonary artery sling was originally repaired
without cardiopulmonary bypass but today most centers would choose bypass to
insure stability while the left artery is switched. Rarely aortopexy is added to more
readily stabilize tracheomalacia, but this is only seldom necessary.
Families should be warned that stridor persists for 6-24 months after correction.
Feeding problems, if present before surgery, abate quickly. Short and long-term out-
comes are excellent in virtually all these children. Intraoperative mortality and
perioperative morbidity are exceedingly low.
Fig. 70.1. Typical double indentation of esophagus on barium swallow in an infant

with a double aortic ring. Notice that one indentation often occurs somewhat higher
than the other does.
70
Fig. 70.2. MRI demonstrating complete encirclement of the espophagus and trachea
by a double aortic ring.
Vascular Rings 317
Selected Readings
1. Gross RE. Surgical relief for tracheal obstruction from a vascular ring. N Engl J
Med 1945;233:586.
2. Potts WJ, Holinger PH, Rosenblum AH. Anomalous left pulmonary artery caus-
ing obstruction to right main bronchus: Report of a case. JAMA 1954; 155:1409.
3. Nikaidoh H, Riker WL, Idriss FS. Surgical management of “vascular rings.” Arch
Surg 1972; 105:327.
4. Backer CL, Ilbawi MN, Idriss FS et al. Vascular anomalies causing tracheoesoph-
ageal compression. Review of experience in children. J Thorac Cardiovasc Surg
1989; 97:725.
70
CHAPTER 71
Tracheoesophageal Fistula
and Esophageal Atresia
Daniel A. Bambini
Incidence
The incidence of congenital tracheoesophageal malformations in the U.S. is
approximately one in every 4,500 births. In some areas of the world (i.e., Finland),
the incidence may be as high as one in 2,440 births. Tracheoesophageal fistula and
esophageal atresia affects males slightly more commonly than females (1.26:1).
Although most cases of tracheoesophageal anomalies are sporadic, familial pat-
terns of inheritance are reported. About 6% of infants with tracheoesophageal mal-
formations are twins. Parents with one affected child have a 0.5-2% chance of a
tracheoesophageal anomaly affecting subsequent offspring. If more than one off-
spring is affected, the risk is 20%. Newborns with a parent having history of TEF/EA
are affected 3-4% of the time.
Etiology
Although the association of this disease with many other congenital abnormali-
ties suggests that these lesions occur after a major disturbance in embryogenesis, the
exact cause remains unidentified. Environmental teratogens have been implicated
but specific causal relationships are unproven. At 22-23 days of gestation, the esopha-
gus and trachea are recognized as a common diverticulum of the foregut. Division
into separate tubular structures occurs primarily in the fourth week of development
and is complete at 34-36 days. Although several embryonic theories have been pro-
posed to explain the formation of tracheoesophageal malformation, none fully explains
all of the anatomic variants of the anomaly that have been described. A high inci-
dence of coincidental anomalies suggests generalized damage to mesenchymal tissue
during the 4th week of gestation.
Classification
Although many anatomic variations have been described, only five types of tra-
cheoesophageal anomalies occur commonly, accounting for 98% of the lesions en-
countered. The Gross-Vogt classification is the most commonly used anatomic
classification system (Table 71.1). Type A lesions are isolated esophageal atresia with-
out a tracheoesophageal fistula and are frequently associated with a “long gap” between
the proximal and distal esophageal segments. Type B lesions are esophageal atresias
Tracheoesophageal Fistula and Esophageal Atresia 319
Table 71.1. Anomalies associated with tracheoesophageal fistula and esophageal

atresia
Cardiovascular 35%
Ventricular septal defect (most common), tetrology
of Fallot, atrial septal defect, patent ductus arteri-
osus, coarctation of the aorta (1-1.5%)
Genitourinary 20%
Hypospadias, cryptorchidism, renal agenesis,
renal hypoplasia, cystic renal disease, hydro-
nephrosis, vesicoureteral reflux, ureteric dupli-
cation, pelvicoureteral or vesicoureteral obstruc-
tion, urachal anomalies, intersex abnormalities,
cloacal or bladder exstrophy, megalourethra,
urethral duplication, posterior urethral valves
Gastrointestinal 24%
Anorectal atresia, duodenal atresia, ileal atresia,
malrotation, annular pancreas, pyloric stenosis
Neurologic 10%
Hydrocephalus 5.2%
Neural tube defects 2.3%
Holoprosencephaly 2.3%
Anophthalmia or microphthalmia 2.3%
Microcephaly
Skeletal 13%
Vertebral anomalies, radial limb deformities
VACTERL 25%
association
CHARGE 3%
association
Choanal atresia 5.2%
Facial cleft 7.2%
Abdominal 4.3%
wall defect
Diaphragmatic 2.9%
71
hernia
Tracheobronchial 40+ %
anomalies
Unilateral pulmonary agenesis, ectopic or absent
right upper lobe bronchus, congenital bronchial
stenosis, decreased cartilaginous: membranous
trachea ratio, laryngotracheoesophageal cleft
Cleft lip or palate
Deafness
Other syndromes: Downs syndrome, Fanconi’s syndrome,
Townes-Brock syndrome, Bartsocas-Papas
syndrome, McKusick-Kaufman syndrome
in association with a proximal tracheoesophageal fistula and are very rare, accounting
for only about 1% of lesions. Type C lesions constitute the most common congeni-
tal esophageal anomaly (85-89%) and include a blind-ending proximal esophageal
pouch with a distal tracheoesophageal fistula. In Type D anomalies, there are two
tracheoesophageal fistulas, one each from the proximal and distal esophageal seg-
ments. In Type E anomalies, a tracheoesophageal fistula is present without an atresia
(H-type fistula). Type F anomalies, congenital esophageal stenosis, are exceptionally
rare (i.e., 1 in 25,000-50,000 births)
For most type C lesions, the proximal esophagus ends blindly with a 1-2 verte-
bral body gap between it and the distal esophagus (Fig. 71.1). The distal esophagus
opens into trachea via an end-to-side fistula located approximately 1 cm above the
carina. Rarely the fistula may be to the right or left main bronchus.
Esophageal obstruction prevents the fetus from swallowing amniotic fluid in utero.
In cases of pure atresia of the esophagus, polyhydramnios is usually present (85%).
Polyhydramnios is present in only about 30% of mothers with fetuses having esoph-
ageal atresia and distal TEF since the fluid can reach the neonatal gut via the fistula.
In the postnatal period, the infant will be unable to swallow his own secretions,
saliva, or feedings. If appropriate precautions are not taken, spillover into the airway
and lung parenchyma occurs causing respiratory compromise.
The distal fistula is usually narrow but allows free passage of air from the trachea
into the gastrointestinal tract. Gastroesophageal reflux in newborns with TEF/EA is
common and occurs in part due to immaturity of the lower esophageal sphincter
and poor lower esophageal motility. Reflux of gastric acid or bile into the respiratory
tract via the fistula causes a chemical pneumonitis. The tracheas in infants with
TEF/EA have a relatively reduced amount of cartilage and relatively increased amount
of muscle in the tracheal wall. As a consequence, the tracheas of these neonates are
prone to collapse (i.e., tracheomalacia). The presence of a dilated, hypertrophied
proximal esophageal pouch may also contribute to tracheomalacia by direct external
compression on the trachea.
Infants with esophageal atresia and tracheoesophageal fistula most commonly
71 become symptomatic within the first few hours of life. However, prenatal diagnosis
is sometimes suspected if prenatal ultrasonography demonstrates a small or absent
stomach bubble in association with maternal polyhydramnios. Occasionally, the
dilated blind upper esophageal pouch is also identifiable.
The symptom that occurs shortly after birth is excessive salivation. Excessive
salivation results from pooling of secretions in the proximal esophageal pouch and
posterior pharynx. Feeding frequently results in regurgitation, choking, gagging, or
cyanosis. Tachypnea, atelectasis, and respiratory distress result from reflux of gastric
contents into the airway or aspiration from the proximal blind pouch. These events
cause a chemical pneumonitis. Abdominal distension results from inspired air entering
the gastrointestinal tract via the fistula and causes worsening respiratory distress and
pulmonary compromise. Symptoms may be less apparent in children with tracheoe-
sophageal fistula without esophageal atresia (H-type fistula).
Fig. 71.1. Plain x-ray in child with a Type C tracheoesophageal fistula and esoph-
ageal atresia. The nasogastric tube is coiled in the blind proximal pouch (solid
arrow) and air present in the bowel (white arrow) confirming the presence of a
distal fistula.
The incidence of other associated congenital anomalies is between 50-70%.

Infants with esophageal atresia without tracheoesophageal fistula (Type A) are the
group most likely to have other anomalies, while infants with H-type fistula (Type E)
are least likely to have other lesions. While cardiovascular anomalies predominate
(35%), genitourinary (20%), gastrointestinal (24%), skeletal (13%) and neurologic 71
anomalies (10%) are also common (Table 71.2). Tracheoesophageal anomalies are
sometimes identified in infants with a broad range of associated malformations known
as the VACTERL association, that includes vertebral, anorectal, cardiac, tracheoe-
sophageal, renal, and radial limb deformities. Esophageal atresia is also identified in
infants with the CHARGE association that includes coloboma, heart defects, choanal
atresia, retardation, genital hypoplasia, and ear deformities (i.e., deafness).
Esophageal atresia occasionally occurs in infants with DiGeorge sequence, Pierre-
Robin sequence, Holt-Oram syndrome, polysplenia syndrome, cleft lip and palate,
omphalocele, and even more rarely with Schisis association.
Table 71.2. Gross-Vogt classification of tracheoesophageal anomalies

Type A Isolated Esophageal atresia 7.8%
Type B Esophageal atresia + Proximal TEF 0.8%
Type C Esophageal atresia + Distal TEF 85.8%
Type D Esophageal atresia + Double TEF 1.4%
Type E Isolated TEF (H-type fistula) 4.2%
Type F Esophageal Stenosis *
* not included
Diagnosis
The diagnosis of esophageal atresia is strongly suggested when there is difficulty
or inability to pass (or repass) a nasogastric or orogastric tube. Resistance is typically
encountered when the tube is passed to about 11-12 cm. A babygram x-ray fre-
quently confirms the nasogastric tube coiling within the proximal esophageal pouch.
To estimate the “gap” or distance between the esophageal segments, a nasogastric
tube is passed until resistance is encountered and a chest x-ray is obtained. The
distance between the tip of the tube and the carina estimates the “gap.” A distance of
less than 2-2.5 vertebral bodies is favorable.
The abdominal portion of the babygram is inspected for the presence of distal
bowel gas (confirmation of a fistula), a “double bubble sign (i.e., suggesting an asso-
ciated duodenal atresia), or other dilatations.
The diagnostic evaluation of infants with tracheoesophageal anomalies includes
screening for other associated congenital defects. Physical examination identifies
defects of the VACTERL and CHARGE associations. Echocardiography and renal
ultrasonography are obtained to identify cardiovascular defects, define cardiac and
aortic arch anatomy, and identify genitourinary malformations. Chromosomal analy-
71 sis is also indicated.
Treatment
Initial treatment of infants with tracheoesophageal anomalies includes measures
to prevent aspiration and pneumonitis. A sump catheter (i.e., Replogle tube) is
positioned in the proximal esophageal pouch to continuously aspirate saliva. The
infant is positioned in an upright or head-up prone position to minimize gastroe-
sophageal reflux and prevent aspiration pneumonitis. An H2-blocker and broad-
spectrum antibiotics are empirically started. Routine endotracheal intubation is
avoided since ventilated air entering through the tracheoesophageal fistula can cause
gastric perforation and worsening respiratory distress secondary to abdominal
distension.
The surgical approach depends on the exact TEF/EA anomaly present. For Type C
lesions, division of the fistula and primary anastomosis of the esophagus is the pro-
Table 71.3. Waterston classification (1962) and current survival

Group Description Survival (%)
A Birthweight > 5.5 lbs. and otherwise well 100

B Birthweight 4 – 5.5 lbs. and otherwise healthy 85
Or
Birthweight > 5.5 lbs. with moderate pneumonia
C Birthweight < 4 lbs. or higher with severe 65
pneumonia or severe cardiac anomalies
cedure of choice. The operation is usually performed via a right posterolateral

thoracotomy (4th interspace) unless a right-sided aortic arch has been identified pre-
operatively in which case a left thoracotomy is preferred. Most pediatric surgeons
use an extrapleural approach. For isolated tracheoesophageal fistulas (Type E), a
cervical approach is possible in most cases and the fistula is divided via a right-sided,
low cervical incision. Esophageal replacement (i.e., colonic interposition, gastric
pull-up, etc.) is sometimes required in cases of “long gap” esophageal atresia, esoph-
ageal atresia without fistula, or when attempted primary repair and anastamosis has
failed.
Outcomes
Overall survival in modern series is around 85-95%. Infants with other major
associated anomalies have a poorer prognosis. The original Waterston’s classification
(Table 71.3) grouped patients based on birthweight, associated congenital anoma-
lies, and presence/absence of pneumonia; these factors predicted infants with risk of
poor survival and helped guide surgical therapy. With the development of modern
neonatal critical care, more low birth weight infants with anomalies are surviving.
Today, the infants at highest mortality risk include those with:
1. birthweight < 1500 grams,
2. major congenital heart disease,
3. severe associated anomalies, and
4. ventilator dependency.
The Spitz classification (Table 71.4) stratifies infants by birthweight and the
71
presence of major cardiac disease and is currently the most commonly used means
to predict survival. Of course,, infants with tracheoesophageal atresia and features of
the VACTERL association have a higher mortality rate (20-25%).
Complications after repair of tracheoesophageal anomalies include anastomotic
leak (14-16%), recurrent tracheoesophageal fistula (3-14%), esophageal stricture
(20-40%) or dysmotility, gastroesophageal reflux (40-70%)), tracheal obstruction,
and tracheomalacia (10-20%). Recurrent laryngeal injury following repair of tra-
cheoesophageal fistula, particularly H-type, is an uncommon but potentially devas-
tating complication.
Table 71.4. Spitz classification and survival

Group Description Percent of Survival
Total (%) (%)
I Birthweight > 1500 g without major congenital 79 97

cardiac defect
II Birthweight < 1500 g or major congenital 19 59
cardiac defect
III Birthweight < 1500 g and major congenital 2 22
cardiac defect
Selected Readings
1. Waterston DJ, Bonham-Carter RE, Aberdeen E. Esophageal atresia: Tracheoesoph-
ageal fistula. A study of survival in 218 infants. Lancet 1962; 1:819.
2. Spitz L, Kiely E, Brereton RJ. Esophageal atresia: Five year experience with 148
cases. J Pediatr Surg 1987; 22:103.
3. Harmon CM, Coran AG. Congenital anomalies of the esophagus. In: O’Neill Jr.
JA et al, eds. Pediatric Surgery, 5th edition. St. Louis: Mosby 1999; 941-967.
4. Haight C, Towsley H. Congenital atresia of the esophagus with tracheoesophageal
fistula: extrapleural ligation of fistula and end-to-end anastamosis of esophageal
segments, Surg Gyn Obstetr 1943; 76:672.
5. Spitz L. Esophageal atresia: past, present, and future. J Pediatr Surg 1996; 31:19.
71
CHAPTER 1
CHAPTER 72
Diaphragmatic Anomalies
Daniel A. Bambini
Incidence
Congenital diaphragmatic hernia (CDH) refers to a congenital defect in the
posterolateral diaphragm at the “foramen of Bochdalek.” It is a relatively common
cause of neonatal respiratory distress with an overall incidence between 1:2000 and
1:5000 live births. CDH accounts for about 90% of congenital diaphragmatic de-
fects. Eighty to ninety percent of congenital diaphragmatic hernias occur on the left
side. A hernia sac is only present 20% of the time. Retrosternal hernias (Morgagni)
are much less common and only account for 2-6% of congenital diaphragmatic
defects. Diaphragmatic eventration is even rarer but is a postoperative complication
in 1-2% of children undergoing surgery to repair congenital heart defects.
Etiology
The specific etiology of CDH is unknown but it is believed to result from a
defective formation of the pleuroperitoneal membrane. In the early weeks of devel-
opment, the pleural and peritoneal cavities communicate via the paired
pleuroperitoneal canals. During the 8th week, the pleural cavity becomes separated
from the peritoneal cavity by the developing pleuroperitoneal membrane. If the
pleuroperitoneal membrane fails to develop, closure of the pleuroperitoneal canal is
incomplete and a posterolateral diaphragmatic defect results. A newer hypothesis
has arisen from the nitrofen rat model of CDH. Electron microscopy of these nitrofen
exposed rat embryos suggests that CDH results from a defective development of the
“posthepatic mesenchymal plate” which also contributes to closure of the
pleuroperitoneal canal. Although familial cases are reported, most cases of CDH are
sporadic. CDH is associated with trisomies 18, 21, and 23 but a specific genetic
etiology has yet to be identified.
Morgagni hernias result from failure of the sternal and crural portions of the
diaphragm to fuse at the site where the superior epigastric artery traverses the dia-
phragm. Morgagni hernias are associated with congenital heart disease and trisomy
21. A variant of the retrosternal hernia is associated with the pentalogy of Cantrell
which includes: omphalocoele, inferior sternal cleft, severe cardiac defects (includ-
ing ectopia cordis), diaphragmatic hernia and pericardial defects. The diaphrag-
matic defect results when the septum transversum fails to develop in the embryo.
Eventration of the diaphragm may be either a congenital or acquired lesion.
Neonatal eventration may be due to defective central development or enervation of
the diaphragm. It may also result from a traction injury to the nerve roots of the
phrenic nerve during traumatic delivery. Eventration most often results from iatro-
genic phrenic nerve injury complicating cardiac or mediastinal surgery.
Thirty percent of fetuses with CDH will be stillborn. If born alive, neonates
with CDH usually present with respiratory distress. The onset of respiratory distress
can be immediate at the time of delivery or may be delayed for 24-48 hours. Only
10% of patients with CDH present beyond the neonatal period. These children (or
adults) may present with vague gastrointestinal symptoms or may be completely
asymptomatic discovered only as an incidental finding. Rarely, an older child with
CDH may present with life-threatening respiratory and cardiopulmonary distress.
Hemodynamic instability may result from severe mediastinal shift caused by a mas-
sively distended, intrathoracic stomach. Volvulus and intestinal obstruction are ex-
ceedingly uncommon, but reported, presentations of CDH beyond the neonatal
period.
The initial signs of CDH in the neonate include tachypnea, grunting respira-
tions, chest retractions, cyanosis, and pallor. Physical exam may reveal a scaphoid
abdomen, shifting of the heart sounds to the right (i.e., left hernia), and bowel
sounds within the chest. Breath sounds are decreased bilaterally, but are often more
diminished on the side of the hernia. Disparity between preductal and postductal
pulse oximetry may confirm the presence of right-to-left shunting and persistent
fetal circulation. The differential diagnosis of CDH includes cystic adenomatoid
malformation, cystic teratoma, pulmonary sequestration, bronchogenic cyst, neu-
rogenic tumors and primary lung sarcoma.
The majority of children with Morgagni (retrosternal) hernias are asymptom-
atic. Diagnosis is often not made until adulthood. Children with this lesion may
present with recurrent respiratory infections, coughing, vomiting or epigastric pain/
discomfort. Intestinal obstruction and bowel ischemia/necrosis may result from in-
carceration of bowel within the hernia sac.
Eventration in the neonate can be asymptomatic but most present with tachyp-
nea, respiratory distress and pallor. Chest physical signs include ipsilateral dullness
to percussion and unilateral or bilateral diminished breath sounds. The point of
maximal cardiac impulse is shifted away from the side of the lesion. Neonates with
diaphragmatic eventration have difficulty sucking and tire easily with feedings. This
combination often causes inadequate weight gain. Older children may present with
recurrent pneumonia or upper gastrointestinal symptoms. The differential diagno-
sis of eventration includes tumors, bronchogenic cysts, pulmonary sequestration,
pulmonary consolidation, and pleural effusion.
72
Diagnosis
Prenatal diagnosis of CDH can be made by fetal ultrasonography as early as 25
weeks gestation. CDH suspected in a newborn infant with respiratory distress is
confirmed by “babygram” performed simultaneously with resuscitation. The com-
mon radiographic findings of left-sided CDH include air/fluid filled loops of bowel
in the left chest, mediastinal shift, and the stomach gas bubble within the chest
(Fig. 72.1). A nasogastric tube may appear to coil in the chest if the stomach lies
Diaphragmatic Anomalies 327
Fig. 72.1. Typical chest x-ray of neonate with left-sided diaphragmatic hernia
(Bochdalek type). Small arrows outline loops of bowel within the left chest while
large arrows demonstrate nasogastric tube turning back toward the chest. Note
also the heart displaced into the right hemithorax.
within the thorax. Right-sided CDH (Fig. 72.2) is often more difficult to discern
and on x-ray examination may resemble lobar consolidation, fluid within chest, or
diaphragmatic eventration.
For Morgagni hernias, posteroanterior and lateral chest radiographs often dem-
onstrate an air-fluid filled structure located immediately posterior to the sternum
(Fig. 72.3). This diagnosis is frequently made when gastrointestinal symptoms lead
to a contrast study that demonstrates herniated stomach, small bowel or colon within
the chest.
Chest radiographs in patients with eventration demonstrate an elevated
hemidiaphragm although this finding can be obscured by intubation and positive
pressure ventilation. The diagnosis is confirmed by ultrasonographic or fluoroscopic
demonstration of paradoxic diaphragmatic motion. Occasionally, computed tomog-
raphy may be necessary to distinguish eventration from other mass lesions.
Pathology/Pathophysiology 72
Congenital diaphragmatic hernia has a complex pathophysiology. Lung hypo-
plasia occurs as a direct consequence of progressive compression of the developing
lungs by herniated viscera. The severity or degree of pulmonary hypoplasia depends
upon both the duration and timing of visceral herniation into the chest. Hypoplasia
is most severe on the ipsilateral side but occurs on both sides. Gas exchange within
these grossly small lungs is limited by a reduced functional area, decreased number
of bronchial divisions, a reduced number of mature alveoli, and surfactant defi-
ciency. Alveoli of CDH lungs are immature and have thickened intra-alveolar septa.
72
Fig. 72.2. Neonatal chest x-ray demonstrating the much rarer right-sided diaphrag-
matic hernia with bowel entering the right hemithorax around the liver. Small arrows
indicate bowel loops, large arrows demonstrate nasogastric tube and endotracheal
tube, while medium arrows indicate the cannulae of an extracorporeal life support
system.
Fig. 72.3. Lateral chest x-ray demonstrating the central, anterior diaphragmatic
herniation generally referred to as a hernia of Morgagni.
The pulmonary vasculature is best characterized by the presence of increased

muscularization of the pulmonary arterioles. The abnormally muscularized and
reactive pulmonary vasculature bed contributes to persistent fetal circulation, pul-
monary hypertension and acute respiratory failure. Left ventricular hypoplasia is 72
also present in CDH and may adversely affect cardiopulmonary function.
The hypoplastic lungs in patients with CDH are functionally immature and
have limited capability for gas exchange. In many cases, alveolar function is inad-
equate. Hypoxemia, hypercarbia and acidosis can quickly develop causing further
deterioration in pulmonary function. The overly muscularized pulmonary artery
tree quickly vasoconstricts in response to reduced oxygen tension and acidosis. This
vasoconstrictive response is both exaggerated and sustained causing pulmonary hy-
pertension. Pulmonary hypertension in the newborn with CDH may effect a return
to the fetal pattern of circulation with right-to-left shunting across both the ductus
arteriosus and foramen ovale. Intrapulmonary shunting also occurs. Right-to-left
shunting further limits gas exchange exacerbating hypoxia, hypercarbia and acido-
sis. A viscous cycle ensues which can rapidly progress to hypotension, shock and
cardiorespiratory failure/arrest.
Morgagni hernias do not typically produce the pathophysiologic problems
encountered with the posterolateral diaphragmatic defect. Gastrointestinal obstruc-
tion or ischemia and their associated pathophysiologic changes are the presenting
features of this lesion when symptomatic.
Unilateral diaphragmatic eventration results in abnormal respiratory mechanics.
Ventilation may be ineffective due to a paradoxic motion of the ipsilateral diaphragm
during inspiration. Contralateral lung ventilation is also impaired. During inspira-
tion, the mediastinum shifts toward the contralateral side reducing the effective
tidal volume on that side.
Treatment
The treatment of CDH depends upon the time of diagnosis and the clinical
presentation. Institutional expertise and the availability of advanced life support
techniques (i.e., extracorporeal membrane oxygenation (ECMO)) also influence the
management strategy of these infants. Once considered a surgical emergency, CDH
is now managed by a delayed surgical approach. Preoperative stabilization and con-
trol of pulmonary hypertension is advised. Mechanical ventilation techniques which
avoid barotrauma are helpful. High frequency and oscillatory ventilation, nitric ox-
ide administration, surfactant replacement, and ECMO are interventions and thera-
pies readily employed to manage these infants. Fetal interventions (i.e., fetal repair,
tracheal ligation), liquid ventilation and lung transplantation are currently experi-
mental therapies investigated at only a few specialized centers.
Neonates with CDH may present with severe respiratory distress that requires
aggressive resuscitation to include endotracheal intubation, neuromuscular block-
ade, and positive pressure ventilation. Initial ventilation should attempt to maintain
preductal saturation at or above 90% using the lowest airway pressures capable of
providing oxygenation. Barotrauma to the hypoplastic lungs must be minimized.
Orogastric or nasogastric decompression is used to minimize bowel distension
which can further compromise respiratory function. Echocardiography is performed
to evaluate for associated cardiac anomalies and assess the severity of pulmonary
hypertension and shunting. Inotropic agents are used to augment left ventricular
function and to raise systemic pressure minimizing right-to-left ductal shunting.
Hypervolemia and hypovolemia must be avoided. Hypoxemia, hypercarbia and aci-
dosis must be identified and promptly corrected when present. Bicarbonate may be
72 used to treat acidosis.
Several pharmacologic interventions may be useful in the perioperative manage-
ment of neonates with CDH. Inhaled nitric oxide, a potent pulmonary vasodilator,
may successfully control refractory pulmonary hypertension. Surfactant replacement
therapy for CDH is controversial but may be beneficial in improving gas exchange.
ECMO is indicated for infants with CDH and respiratory failure that cannot be
managed with conventional therapy. Candidates must have a reasonable chance for
survival with no major, nonreversible anomalies. ECMO can be used as a preoperative
or postoperative therapy. Some centers perform diaphragmatic repair while on

ECMO.
Surgical repair of congenital diaphragmatic hernia is delayed until after preop-
erative stabilization and resolution of pulmonary hypertension. The repair should
be performed efficiently and expeditiously to minimize operative stress. The
abdominal viscera are reduced from the chest via a transabdominal approach. The
diaphragmatic defect is closed primarily if possible, or a prosthetic patch may be
inserted for larger defects. Tube thoracostomy is optional. The abdominal wall is
stretched prior to closure to increase the capacity of the abdominal cavity. Rarely,
abdominal closure is achieved with a prosthetic silo or by creating skin flaps and a
ventral hernia.
Postoperative ventilator management can be difficult. Chest compliance is
decreased after repair and surgical stress can precipitate intense pulmonary vasocon-
striction and pulmonary hypertension with recurrent fetal circulatory pattern. Res-
piratory failure can occur abruptly and ECMO may be required as a rescue therapy.
Postoperative ventilation strategies should attempt to minimize barotrauma while
maintaining normal PO2, PCO2 and pH.
Morgagni hernias are surgically repaired via a transabdominal approach. Pri-
mary closure of small defects is preferred, but larger defects may require prosthetic
patch closure. The treatment of symptomatic eventration is also surgical. The dia-
phragm on the affected side is plicated via either a transthoracic or transabdominal
approach. Plication effectively immobilizes the flaccid diaphragm, reducing the para-
doxical movement and mediastinal shift that occurs with respiration.
Outcomes
Despite the many therapeutic options available to manage patients with CDH,
the overall survival remains about 60%. Institutional variation in survival is great
and ranges from 25-80%. CDH accounts for 4-10% of neonatal deaths occurring
as a result of congenital anomalies. ECMO improves survival by 15-20% at institu-
tions employing this therapy.
Most survivors of CDH are generally healthy and are without respiratory prob-
lems. Long-term respiratory status is dependent on the severity of pulmonary hypo-
plasia at birth and the degree of lung injury sustained during the perinatal period.
Gastroesophageal reflux is common in survivors of CDH repair; surgical interven-
tion may be required in 10-15%. Survivors of CDH are at increased risk for
neurodevelopmental delays. The overall incidence of neurologic abnormalities is
10-45%.
Surgical results from repair of Morgagni hernias are in general excellent. Com-
plication rates are low. Morbidity and mortality is usually due to associated cardiac
anomalies which are frequently found in these children.
72
The perioperative morbidity and mortality of diaphragmatic plication for even-
tration is low. Complications are mostly secondary to prolonged mechanical venti-
lation and/or cardiac dysfunction associated with an underlying cardiac pathology.
Plication results in immediate improvement in pulmonary mechanics but long-term
respiratory function depends on lung damage prior to plication surgery.
Selected Readings
1. Glick PL, Irish MS, Holm BA eds. New Insights into the Pathophysiology of Con-
genital Diaphragmatic Hernia. Clin Perinatol 1996; 23(4):625-907.
2. Puri P. Congenital diaphragmatic hernia. Current Probl Surg 1994; 31(10):784.
3. Kluth D, Keijzer R, Hertl M et al. Embryology of congenital diaphragmatic her-
nia. Semin Pediatr Surg 1996; 5(4):224.
72
CHAPTER 1
CHAPTER 73
Congenital Malformations of the Lung

Marleta Reynolds
Within this section, four congenital malformations of the lung are discussed. All
of these lesions are quite rare. Even in large metropolitan children’s hospitals, these
lesions are seen only a few times each year. The exact etiology of each of these con-
ditions is unknown although there are multiple causation theories. To facilitate the
study of this information, they are presented together with a short vignette on pre-
sentation, diagnosis and current treatment.
Congenital Lobar Emphysema
Congenital lobar emphysema is an isolated hyperinflation of one lobe of the
lung in the absence of extrinsic bronchial compression. Some cases are found to
have abnormal bronchial cartilage. Symptoms of respiratory distress including tachy-
pnea, chest wall retractions, and wheezing usually develop in infancy. Upper respira-
tory infections may precipitate severe symptoms. Physical exam reveals decreased
breath sounds on the affected side with hyperresonance. The trachea and mediasti-
num are shifted to the contralateral side. A chest radiograph reveals hyperinflation
of the affected lobe, atelectasis of the adjacent lobes, and mediastinal shift (Fig. 73.1).
A chest CT may be helpful to rule out a perihilar bronchogenic cyst causing bron-
chial obstruction. The left upper lobe is affected most often followed by the right
middle lobe.
Positive pressure ventilation can potentially cause overinflation of the involved
lobe. Overinflation of the diseased lung can cause cardiovascular collapse and must
be avoided. At surgery, the chest is opened and the lobe herniates out of the chest
and allows safe ventilation (Fig. 73.2). The mediastinum should be inspected for
lesions that could cause bronchial obstruction. Lobectomy is performed and the
remaining lung expands to fill the space.
Pulmonary Sequestration
A pulmonary sequestration is a segment or lobe of lung which has no bronchial
communication with the tracheobronchial tree. The sequestered lung tissue has sys-
temic arterial blood supply that may arise from the thoracic or abdominal aorta.
Blood return from a pulmonary sequestration is usually via the pulmonary venous
system. Occasionally the venous drainage will be to systemic veins. A sequestration
probably develops from an aberrant lung bud that pinches off from the caudal foregut
with its own blood supply.
Fig. 73.1. Radiograph of a child with congenital lobar emphysema. This x-ray dem-
onstrates hyperlucency of the left chest, spread ribs on the left, mediastinal shift,
and a collapsed left lower lobe.
73
Fig. 73.2. Congenital lobar emphysema of the left upper lobe at the time of surgical
resection. The left lung and lingula are herniating through the thoracotomy inci-
sion due to hyperexpansion of the lobe.
Congenital Malformations of the Lung 335
Extralobar Sequestration
An extralobar sequestration is triangular in shape and is usually found in the
posterior costophrenic angle adjacent to the aorta or esophagus. It can be found
anywhere in the chest and upper abdomen. It is often associated with a congenital
diaphragmatic hernia. Most children with extralobar sequestration are asymptom-
atic and the lesion is discovered as an incidental finding on chest radiograph. In a
newborn or infant, an ultrasound can be diagnostic, but in older children CT of the
chest is usually diagnostic. Very occasionally, angiography will be needed to identify
the anomalous blood supply.
Intralobar Sequestration
An intralobar sequestration is found within normal lung parenchyma (Fig. 73.3).
The most common sites are the right lower lobe and left lower lobe. In the majority
of cases, the venous return is to the pulmonary venous system.
Infection within the sequestered lung generally leads to the diagnosis. Chronic
infection and lung abscess may prompt a chest radiograph and chest CT(Fig. 73.4).
Angiography is seldom indicated. Degenerative arteriosclerotic changes of the anoma-
lous arterial vessel may develop and lead to hemoptysis.
The involved lobe should be resected. Careful exploration and identification of
the arterial supply of the affected lobe with suture ligation of the vessel will avoid
exsanguinating hemorrhage from the systemic vessel that may retract into the abdo-
men if it is divided before control is obtained.
Congenital Cystic Adenomatoid Malformation (CCAM)
The etiology of congenital cystic adenomatoid malformation is unknown, but
the lesion is represented by a combination of solid and cystic components with an
overgrowth of terminal bronchiolar-type tubular structures and a lack of mature
alveoli (Fig. 73.5). The predominantly solid lesions are found in stillborn or prema-
ture infants and are associated with fetal anasarca, ascites and maternal polyhydram-
nios. The solid-cystic lesions can produce respiratory distress in the near-term infant.
The cystic lesions usually do not present until late infancy or early childhood and
occasionally into adulthood. The cystic lesions become secondarily infected and are
identified after a bout of pneumonia or after recurrent pneumonias.
Prenatal ultrasound can identify this anomaly of the lung. When diagnosis is
made prenatally the only predictor of poor outcome is the presence of hydrops.
Fetal intervention has included placement of thoracoamniotic shunt or lobectomy.
After birth, the chest radiograph can be diagnostic of a congenital cystic adenomatoid
malformation but can be confused with a congenital diaphragmatic hernia. The
lesion with few cystic spaces may be confused with a parenchymal bronchogenic
cyst. CT scan may be very helpful in differentiating the pathology. 73
The abnormal histology found in the lesion consists of an adenomatoid increase
of terminal respiratory bronchiolar-like structures lined with ciliated columnar epi-
thelium. Cysts with bronchial-type epithelium may have polypoid overgrowths and
can be interspersed with the solid tissue. There are no bronchial mucoserosal glands
or cartilage plates. Alveolar cysts may be lined with mucous-secreting cells. Malig-
nancy has been reported in more than 10 cases.
Fig. 73.3. An intrapulmonary lobar sequestration without bronchial communica-

tion, infection, or abscess formation.
73
Fig. 73.4. An infected intrapulmonary sequestration that has developed into a tho-
racic abscess with an air-fluid level.
Congenital Malformations of the Lung 337
Fig. 73.5. Cystic adenomatoid malformation with macrocystic changes easily

discernable by simple examination.
Surgical resection of the involved lobe is indicated. Exploration for an anoma-

lous systemic arterial supply should be performed because an overlap in diagnosis
between congenital cystic adenomatoid malformation and intrapulmonary seques-
tration has been reported.
Bronchogenic Cyst
A bronchogenic cyst (Fig. 73.6) develops as a result of abnormal budding from
the developing tracheo-bronchial tree. It occurs at the glandular stage of bronchial
development. The cysts can be found in the hilum of the lung, the mediastinum,
the posterior sulcus and within the pulmonary parenchyma. Parenchymal cysts present
with fever and other signs of pulmonary sepsis. By compressing the airway, medias-
tinal cysts may produce wheezing, signs of airway obstruction, and may create distal
pulmonary infection and its associated symptoms of fever and cough.
Diagnosis can be made by chest radiograph for parenchymal cysts and chest CT
for mediastinal cysts. MRI may provide additional information about the cysts. The
73
parenchymal cyst can be confused with a cystic adenomatoid malformation. The
cysts can be filled with air or mucous. If the cyst becomes infected, the air and pus
may create an air-fluid level on chest radiographs.
The cysts are lined with ciliated columnar or cuboidal epithelium on a fibromus-
cular base. If infected the cyst’s epithelial lining may be destroyed. The cyst wall is
thin and may contain cartilage and bronchial glands. Association with lower lobes is
frequent, and there is rarely a communication with the tracheobronchial tree. A
Fig. 73.6. Bronchogenic cyst after surgical removal with characteristic thin-walled
cystic structure.
single case of adenocarcinoma has been reported in an 8- year old girl with a bron-
chogenic cyst.
Surgery is indicated for mediastinal and parenchymal bronchogenic cysts. If
pulmonary sepsis has developed it should be aggressively treated prior to surgical
resection. The mediastinal cyst requires simple excision and may be amenable to
thoracoscopic removal. The posterior wall of the trachea may need to be patched
with a piece of pericardium if it is resected with the cyst. A lobectomy is usually
required to treat parenchymal cysts. Incomplete resection of the cyst may lead to
recurrence. Anesthetic management must be individualized but positive pressure
ventilation and nitrous oxide can be used for most patients.
Selected Readings
1. Savic B et al. Lung sequestration: Report of seven cases and review of 540 pub-
lished cases. Thorax 1979; 34:96.
2. Reynolds M. Congenital lesions of the lung. In: Shields TW ed. General Thoracic
Surgery, 4th Edition, Vol. 2. Williams & Wilkens, Media 1994; 859-87.
3. Miller JA, Corteville JE, Langer JC. Congenital cystic adenomatoid malformation
73 in the fetus: Natural history and predictors of outcome. J Pediatr Surg 1996;
31(6):805-8.
4. Cass DL, Crombleholme TM, Howell LJ et al. Cystic lung lesions with systemic
arterial blood supply: A hybrid of congenital cystic adenomatoid malformation
and bronchopulmonary sequestration. J Pediatr Surg 1997; 32(7):986-90.
5. Suen HC, Mathiesen DJ, Grillo HC. Surgical management and radiologic charac-
teristics of bronchogenic cysts. Ann Thorac Surg 1993; 55(2):476-81.
CHAPTER 1
CHAPTER 74
Foreign Bodies in the Air Passages

and Esophagus
Marleta Reynolds
Foreign Bodies in the Esophagus
Incidence
Children between the ages of 1 and 5 years are at risk for accidental ingestion of
a foreign object. Most children in this age range swallow some toy, coin, or button at
one time or another. The vast majority of swallowed foreign objects are probably
never known, even to parents.
Most ingested objects pass through the gastrointestinal tract without difficulty.
However, a foreign object may become lodged proximal to a congenital or acquired
stricture of the esophagus. In addition, objects such as coins, toys, bones and open
safety pins can become lodged in the hypopharynx or at one of three levels of physi-
ologic narrowing of the esophagus: the cricopharyngeus, the aortic arch and gas-
troesophageal junction. Children may complain of dysphagia, develop sialorrhea or
may develop respiratory symptoms from compression of the membranous trachea.
Diagnosis
Posterior and lateral neck radiographs and a chest radiograph (Figs. 74.1A and
74.1B) are needed to identify the location of the object and evaluate for extravasated
air in the mediastinum, subcutaneous tissue, or chest. Water soluble contrast may
be used to identify the object when plain films are negative and clinical suspicion is
high.
Pathophysiology
Complications of esophageal foreign bodies include perforation, aspiration,
retropharyngeal abscess, mediastinitis, pericarditis, pneumothorax, pneumomedi-
astinum and vascular injury. Complications that develop well after the ingestion
include respiratory compromise, extraluminal migration, esophageal stricture, tra-
cheoesophageal fistula and recurrent pneumonia.
Treatment
Small blunt objects are of little concern but large or sharp objects may need to be
removed. The urgency of removal will depend on the type of foreign body, the
74
Figs. 74.1A and 74.1B. (opposite) Coin and open safety pin within the esophagus.
Since these foreign bodies are seen “en face” we know they are in the esophagus
and not in the trachea (where foreign bodies generally are seen on edge). The
safety pin is probably caught in the cricopharyngeus muscle while the coin is caught
at the aortic arch.
Foreign Bodies in the Air Passages and Esophagus 341
length of time the object has been in the esophagus and the patient’s symptoms. An
algorithm for coin ingestion is included (Fig. 74.2) Foreign objects below the
cricopharyngeus are removed under general anesthesia using a rigid or flexible esopha-
goscope and appropriate grasping forceps or baskets.
Outcomes
Perforation and bleeding are the most frequent complications of extraction and
occur in 2-13% of cases. Most foreign bodies that reach the stomach will pass and
outpatient observation with instructions to return if abdominal complaints arise is
indicated.
Foreign Bodies of the Air Passages
Incidence
Aspiration of a foreign body into the air passages usually occurs in older infants
and toddlers. Boys are affected more than girls in a ratio of 2:1.
A caretaker may witness the child placing a toy or coin in the mouth and then
choking, gagging or developing paroxysms of coughing and wheezing. Once the
object becomes lodged in the airway, an asymptomatic period may follow. The object
then may precipitate erosion or infection. The child may develop fever, malaise,
cough and/or hemoptysis. Atelectasis, pneumonia, or lung abscess may result.
Diagnosis
Posteroanterior and lateral chest radiographs (Fig. 74.3) are obtained to evaluate
a child suspected of aspirating a foreign body. Posteroanterior and lateral soft-tissue
neck radiographs are useful for identifying tracheal foreign bodies. Inspiratory and
expiratory chest radiographs can also be helpful. On expiration the air is trapped
behind the obstruction causing emphysema of the involved lobe or lung and medi-
astinal shift to the contralateral side.
Treatment
General anesthesia is used to remove foreign bodies of the airway. The laryngo-
scope is used to expose the larynx and spray topical lidocaine before the broncho-
scope is introduced. The rigid ventilating bronchoscope is used to visualize the foreign
body (Fig. 74.4). The grasping forceps are introduced into the bronchoscope. The
object is removed through or with the bronchoscope. Humidity, bronchodilators
and steroids may be helpful in decreasing postoperative edema. Racemic epineph-
rine may also be administered. A postoperative chest radiograph should be obtained
to identify a pneumothorax or mediastinal air complicating foreign body removal.
74
Fig. 74.2. Management algorithm for coin ingestion by a child.
74
Fig. 74.3. Screw within the trachea, blunt end pointed downward (most common
orientation), and passing into the right mainstem bronchus (always the most com-
mon location for an aspirated foreign body).
Foreign Bodies in the Air Passages and Esophagus 343
Fig. 74.4. Foreign body within the main trachea that proved on extraction to be
part of a crayon.
Selected Readings
1. Quinn PG, Conners PJ. The role of upper gastrointestinal endoscopy in foreign
body removal. Gastrointestinal Endoscopy Clin N Amer 1994; 4(3):571-593.
2. Holinger LD. Foreign bodies of the airway & esophagus. In: Holinger LD, Lusk
RP eds. Pediatric Laryngology & Bronchoesophagology 1997; 233-251.
3. Healy GB: Management of tracheobronchial foreign bodies in children: an up-
date. Ann Oto Rhin Laryn 1990; 99:889-891.
4. Webb WA. Management of foreign bodies of the upper gastrointestinal tract. Gas-
troenterology 1988; 94:204-216.
74
CHAPTER 75
Chylothorax and Diseases of the Pleura

Chylothorax
Incidence
Chylothorax is an accumulation of lymphatic fluid in the pleural space. Most
cases involve the right side, although bilateral chylothoraces do occur. Males are
affected twice as frequently as females. In general, chylothorax occurs as a direct
result of surgical trauma. Nontraumatic chylothorax is less common, and when
bilateral, it is usually a result of venous hypertension in the brachiocephalic system
due to superior vena caval thrombosis.
The etiology of chylothorax can be classified into four groups:
1. congenital,
2. traumatic,
3. neoplastic, and
4. spontaneous (Table 75.1).
Congenital chylothorax occurs in the neonatal period (Fig. 75.1) and is associ-
ated with multiple congenital anomalies such as lymphangectasia. Trauma to the
thoracic duct during delivery is possible due to difficult extraction during breech
presentations; hyperextension of the spine may rupture the delicate lymphatic ves-
sels lying over the vertebral bodies. However, intraoperative trauma to the thoracic
duct is the most common cause of chylothorax in all age groups. Surgical procedures
in the anatomic region of the thoracic duct, which is quite small and has many
tributary lymphatic channels, may disrupt the thoracic duct and result in chylotho-
rax. The most common of these procedures include congenital diaphragmatic her-
nia repair, tracheoesophageal fistula repair, PDA ligation, and especially cardiac
procedures. Tumors such as lymphoma, neuroblastoma and other metastatic malig-
nancies may cause chylothorax by obstructing the lymphatic flow in the thoracic
duct. Causes of spontaneous chylothorax include forceful straining, coughing or
vomiting, as well as subclavian or superior vena caval thrombosis. An increase in
intraductal pressure due to elevated central venous pressure or increased chyle trans-
port after a meal, coupled with an increase in intrathoracic pressure may result in a
“blowout” or rupture of the thoracic duct.
Chylothorax and Diseases of the Pleura 345
Table 75.1. Etiology of chylothorax
CONGENITAL
• Lymphangiomatosis
• Lymphangiectasia
• Down’s syndrome
• Noonan’s syndrome
• Turner’s syndrome
TRAUMA
• Operative
• Cardiothoracic/esophageal procedures
• Diaphragmatic hernia repair
• Subclavian or left heart catheterization
• Blunt trauma
• Birth trauma
• Physical abuse
• Penetrating trauma
NEOPLASTIC
• Lymphoma
• Neuroblastoma
SPONTANEOUS
• Forceful straining, coughing or vomiting
• Subclavian/superior vena cava thrombosis
Fig. 75.1. Right hemithorax

opacification from neonatal
chylothorax.
75
Anatomy
The thoracic duct originates in the abdomen as the cisterna chyli located over
the second lumbar vertebrae. The duct passes through the aortic hiatus and extends
upward into the posterior mediastinum on the right. At the level of the fifth verte-
bral body, the thoracic duct crosses the midline to the left hemithorax, where it
continues its ascent posterior to the aortic arch. The thoracic duct empties into the
venous circulation at the junction of the subclavian and internal jugular veins
(Fig. 75.2).
Normal chyle flow ranges between 50-200 ml/hr and varies widely depending
on volume of fat digestion and central venous pressure. Chyle contained in the
thoracic duct transports nearly 75% of the ingested fats from the small intestine to
the systemic circulation. Chyle is also rich in protein and lymphocytes. Consequently,
prolonged loss of chyle from a thoracic duct fistula can result in malnutrition,
hypoproteinemia, fluid and electrolyte imbalance, metabolic acidosis and immuno-
deficiency.
Symptoms and Diagnosis
Chylous compression produces acute respiratory distress symptoms: dyspnea,
tachypnea and cyanosis. The involved hemithorax contains intrapleural fluid that
produces percussion dullness and diminished breath sounds. If severe, the fluid pro-
duces mediastinal and tracheal shift to the contralateral side. There are other causes
of pleural effusion and respiratory distress, but the chylothorax fluid has multiple
characteristic physical and laboratory features that confirm the diagnosis (Table 75.2).
The presence of polyhydramnios, hydrops fetalis, and pleural effusion on fetal
ultrasound is highly suggestive of congenital chylothorax. Congenital chylothorax is
considered seriously in all cases of nonimmune hydrops fetalis in which pleural
effusions develop early. In neonates with traumatic birth, symptoms of respiratory
embarrassment observed in combination with pleural effusion are highly suggestive
of chylothorax. Symptoms are usually rapid in onset with 50% of cases occurring
within 24 hours of delivery.
Evidence of significant pleural effusion following thoracic surgery alerts the phy-
sician to the possibility of thoracic duct injury. Patients with chylothorax resulting
from obstruction of the thoracic duct by a mass along with cervical or supraclavicu-
lar lymphadenopathy indicates the need to identify malignancy.
Treatment and Outcome
The primary therapy for chylothorax is thoracostomy tube drainage. This allows
quantitation of the daily chyle leak, promotes pulmonary reexpansion, and allows
the leak to seal. In cases of congenital chylothorax, this drainage may be achieved
with intrauterine thoracentesis or insertion of a pleuroamniotic shunt. In addition,
dietary manipulations are useful to reduce lymph flow through the thoracic duct.
Large-chain triglycerides are primarily transported to the systemic circulation by the
cisterna chyli and thoracic duct; therefore restriction to medium- and short-chain
75 triglycerides, which are absorbed directly into the portal venous circulation, results
in reduced lymph flow through the thoracic duct and promotes fistula closure. Patients
refractory to thoracostomy drainage and dietary manipulations require cessation of
oral feedings and the implementation of total parenteral nutrition.
Fig. 75.2. Schematic drawing of the thoracic duct anatomy. Reprinted with permis-
sion from Cohen RG. The pleura. In: Sabiston DC, Spencer FC eds. Surgery of the
Chest. 6th ed. W.B.Saunders, 1995.
Table 75.2. Characteristic features of chylous fluid

Color Cloudy if patient on oral diet
Serosanguinous if NPO
Specific gravity 1.012 – 1.025
Protein > 5 g/2L
Fat > 400 mg/ 2L
Triglycerides > 220 mg/2L 75
Lymphocytes > 90% of total cells
Gram stain Negative
Sudan Red stain Chylomicrons/fat globules
The rate of chyle leak from a thoracic duct fistula can be massive with significant
volume and nutritional losses; they must be replaced appropriately. The immune
status of the patient must also be monitored closely due to a steady decline in circu-
lating lymphocytes and potential risks for sepsis. It is generally accepted that a 7-10
day trial of thoracostomy tube drainage and dietary manipulation is justified in
patients with chylothorax. This regimen is successful in 70-80% of cases. However,
when drainage exceeds 180 ml/day/year of age in a child (maximum 500 ml/day),
surgical correction is required. Ingestion of cream or milk products prior to surgery
will engorge the lymphatic vessels of the thoracic duct and facilitate visualization of
ductal injury for suture ligation. Application of fibrin glue may also help seal the
leak. Thoracoscopic management with suture ligation along with pleurodesis is an
alternative intervention. When operative options fail or are exhausted, the
pleuroperitoneal shunt may provide prolonged symptomatic relief.
Outcome
Conservative management of chylothorax in the pediatric population is success-
ful in 70-80% of cases, with the majority of the remaining patients successfully
managed by operative intervention. Long-term follow-up in newborns with con-
genital chylothorax demonstrates less encouraging results due to the underlying
pulmonary malformations in many of these children.
Introduction of total parenteral nutrition and enteral formulas containing
medium-chain triglycerides as well as the operative thoracic duct ligation for chy-
lothorax have significantly improved the overall outcome for postcardiotomy patients.
However, mortality remains close to 10% in children who develop chylothorax fol-
lowing surgery for congenital heart disease. Most deaths are due to overwhelming
bacterial and fungal infections, presumably as a consequence of excess loss of lym-
phocytes in chylous drainage.
Empyema
Empyema is the accumulation of infected fluid in the pleural space. In children,
empyema is generally a sequelae of severe pneumonia and occurs in about 1% of
cases. Other less common causes include trauma, intrathoracic perforation of the
esophagus, or infection of the retropharyngeal or mediastinal spaces. The most com-
mon organisms identified in childhood cases of empyema are Staphylococcus aureus,
Hemophilis influenzae, and Streptococcus pneumoniae. The development of empyema
is described in three stages. The early stage or exudative stage (24-72 hours) is char-
acterized by an accumulation of thin pleural fluid with low cellular content. This is
followed by the fibrinopurulent stage (7-10 days) during which the infected pleural
fluid consolidates, fibrinous material accumulates, and loculation formation results
in decreased lung mobility. Finally, the organization phase ensues (2-4 weeks) and
the involved lung frequently becomes entrapped by a pleural peel which forms as a
result of fibroblast proliferation and fibrin deposition.
75
Children with empyema present with fever, cough, respiratory distress, and chest
pain. A recent history of pneumonia may be identified. Physical exam reveals decreased
breath sounds, dullness to percussion, and tactile fremitus on the involved hemitho-
rax. Irritation of the pleura results in a friction rub on auscultation. Chest x-ray
(Fig. 75.3) typically identifies thickened pleura in association with a pleural effu-
sion. Ultrasound may be used to determine the presence of loculations; however,
computerized tomography is the most sensitive study to determine the degree of
pleural thickness, the presence and number of loculations, and presence of lung
consolidation (Fig. 75.4). Thoracentesis with fluid analysis can occasionally con-
firm the diagnosis. The gross appearance of the fluid is turbid and thick. Laboratory
data consistent with empyema include pH < 7.2, glucose level < 40 mg/dL, protein
> 3 g/dL, LDH > 200 U/L, and WBC > 15,000/mm3. Gram stain and culture of the
pleural fluid is important to help guide antibiotic therapy.
Successful treatment of empyema depends on early diagnosis, with administra-
tion of appropriate antibiotics in combination with pleural drainage and mainte-
nance of lung expansion. Diagnostic thoracentesis is occasionally therapeutic in the
early exudative phase of empyema. Thoracostomy with closed drainage and intrave-
nous antibiotics are necessary to treat fibrinopurulent stages of empyema. Failure of
antibiotics and thoracostomy tube drainage is usually a result of inadequate drain-
age of loculated fluid or lung entrapment in the fibrotic peel. Simple loculations can
be lysed by urokinase (20,000 IU of diluted urokinase, three installations per day)
or streptokinase administered through a preexisting chest tube; however, if unre-
sponsive, thoracoscopic pleural debridement and decortication can provide compa-
rable clinical results to traditional open thoracotomy techniques. Lung abscess may
require wedge resection, or even lobar resection. Mortality associated with empy-
ema in children is less than 3%. Pulmonary function after recovery is usually nor-
mal, although mild restrictive or obstructive disease on follow-up spirometry has
been reported.
Spontaneous Pneumothorax
Pneumothorax is a collection of air in the pleural space. Pneumothorax occurs in
0.5-2.0% of all infants, and particularly in patients receiving high continuous posi-
tive airway pressure for treatment of hyaline membrane disease, meconium aspira-
tion syndrome, or congenital diaphragmatic hernia. Uneven ventilation, poor
pulmonary compliance, high viscosity of lung fluid, and high surface tension lead to
increased intraalveolar pressure which often results in alveolar over-distention and
rupture. The dissection of air through the parenchyma causes pulmonary interstitial
emphysema. Once the visceral pleura is perforated, pneumothorax results.
Spontaneous pneumothorax may occur in children with no known underlying
disease or may result from an underlying condition such as a congenital bleb, cystic
adenomatoid formation, or cystic fibrosis. Typically, patients are adolescent males
with ectomorphic features. Recurrence rates are 50% after the first episode, 62%
after the second, and 83% after the third. 75
Fig. 75.3. Empyema demonstrated with lower thorax opacification and air fluid
levels (arrows).

The most common presenting symptoms of spontaneous pneumothorax are
ipsilateral chest pain and dyspnea. Other symptoms include tachypnea, intercostal
retractions, cyanosis, and grunting. Physical exam reveals decreased breath sounds
in the ipsilateral hemithorax. If a tension pneumothorax is present, the trachea is
usually shifted to the contralateral side. Diagnosis is confirmed by chest x-ray, and
radiographic findings are enhanced if films are taken at end expiration.
Spontaneous unilateral pneumothorax of 15-20% in size may be monitored by
75 serial chest x-rays if the patient is asymptomatic. Pleural air can be reabsorbed at a
rate of 1.25% per day, and there are recurring reports, poorly documented or ex-
plained, that this rate may be increased by administration of 100% oxygen. If the
patient is symptomatic or the size of the pneumothorax increases, a thoracostomy
Fig. 75.4. Computed tomography shows the size and extent of an empyema with
multiple air and fluid filled spaces.
tube must be inserted. In tension pneumothorax, a 14-gauge angiocatheter can be

placed in the second intercostal space anteriorly to immediately relieve hemody-
namic compromise. Continuous suction on the chest tube is maintained until the
air leak ceases. Persistence of air leak may be seen in patients with a chronic under-
lying condition such as cystic fibrosis, bronchopulmonary dysplasia, lung cysts, or
blebs. Treatment of these patients then requires resection of the diseased lung along
with pleurodesis. Pleurodesis is usually performed by the administration of chemi-
cal irritants (talc, tetracycline) or mechanical rub through the thoracostomy tube,
thoracoscope, or at thoracotomy. Talc is the agent of choice since it incites less pain
and is more effective in treating pneumothorax in patients with cystic fibrosis.
Pleurodesis is considered in any teenager after two episodes of spontaneous pneu-
mothorax because of the high recurrence rate. Overall outcome is excellent after
surgical treatment for pneumothorax.
Selected Readings
1. Allen EM, van Heeckeren DW, Spector ML et al. Management of Nutritional and
Infectious Complications of Postoperative Chylothorax in Children. J Pediatr Surg
1991; 26:1169-1174.
2. Rothenberg SS. Thoracoscopy in infants and children. Semin Pediatr Surg 1994;
3(4):277-282.
3. Merry CM, Bufo AJ, Shah RS et al. Early definitive intervention by thoracoscopy 75
in pediatric empyema. J Pediatr Surg 1999; 34(1):178-180.
4. Chan W, Keyser-Gauvin E, Davis GM et al. Empyema thoracis in children: A 26-
year review of the Montreal Children’s Hospital experience. J Pediatr Surg 1997;
32(6):870-872.
CHAPTER 74
Patent Ductus Arteriosus

Samer Kanaan and Daniel A. Bambini
Incidence
Patent ductus arteriosus (PDA) is the persistence in postnatal life of the ductus
arteriosus. Overall, the incidence of PDA in term infants is approximately 1 in 2,000
live births. PDA, as an isolated lesion, accounts for about 5-10% of congenital heart
disease. PDA affects girls twice as often as boys and frequently affects siblings sug-
gesting a genetic component to etiology. PDA is particularly common in babies
whose mothers contract rubella during the first trimester of pregnancy.
A high percentage of preterm infants have prolonged PDA after birth. The fre-
quency of occurrence varies with gestational age and birth weight (BW). The inci-
dence of PDA in premature infants born at 28-30 weeks of gestation is about 77%.
Preterm infants born at gestational ages beyond 31 weeks have a lower incidence of
PDA (i.e., 44% for 31- 34 weeks vs. 21% for 34-36 weeks). Neonates born at weights
less than 1000g have a high incidence (83%) of PDA, whereas, 47% of neonates
with birth weights between 1000-1500 grams and 27% with birth weights between
1500-2000 grams have a PDA. However, only about half of PDAs in low birth
weight infants (i.e., BW less than 1750 g) become hemodynamically significant.
Etiology
The ductus is derived from the distal embryologic left sixth arch. In the normal
fetal cardiovascular system, the ductus arteriosus connects the upper descending
thoracic aorta and the proximal left pulmonary artery allowing oxygen-rich placen-
tal blood to bypass the fetal pulmonary circulation. In the normal fetal cardiovascu-
lar system, ductal flow is considerable and is directed exclusively from the pulmonary
artery to the aorta. Although patent at birth, the ductus arteriosus spontaneously
closes shortly after birth. Postnatal closure occurs in two stages. In full-term infants,
the first stage is usually completed within 10-15 hours of birth as smooth muscle
within the ductal wall contracts. The second stage of closure is usually completed by
2-3 weeks and results from diffuse fibrous proliferation of the intima. The ductus
arteriosus is completely closed by 8 weeks in 88% of infants with an otherwise
normal cardiovascular system. When closure is delayed or fails, patent ductus arte-
riosus results.
Ductus closure is mediated by:
1. release of vasoactive substances,
2. variations in pH,
Patent Ductus Arteriosus 353
3. increased oxygen tension, and

4. prostaglandins (PGE1, PGE2, PGI2).
Rising oxygen tension and prostaglandins produce opposite effects; increased
partial pressure of oxygen (PO2) causes smooth muscle constriction while prostag-
landins cause relaxation. The relative effects of oxygen and prostaglandins on the
ductus varies at different gestational ages; the ductus is more sensitive to oxygen in
the mature fetus and is more sensitive to prostaglandins in the premature fetus.
PDA occurs in four distinct forms:
1. an isolated cardiovascular lesion in an otherwise healthy infant,
2. an isolated cardiovascular lesion in a premature infant,
3. an incidental finding associated with more significant structural cardio-
vascular defects, and
4. a critical compensatory structure in cyanotic or left-sided obstructive
lesions.
The signs and symptoms in PDA are related to left-to-right shunting. The mag-
nitude of the shunt depends upon the size of the ductus and relative systemic and
pulmonary vascular resistances. PDA is categorized as large, moderate, and small
lesions.
For large PDAs, aortic and pulmonary artery pressures are essentially equal. After
birth, the systemic vascular resistance remains fairly constant, therefore, the magni-
tude and direction of shunting depends mostly upon changes in pulmonary vascu-
lar resistance. As the pulmonary vascular resistance falls, left-to-right shunting rapidly
develops and infants develop signs of severe congestive heart failure usually within a
month. Signs include tachypnea, tachycardia, sweating, irritability, poor feeding,
and slow weight gain. Pulmonary edema, pneumonia, or recurrent respiratory
infections frequently occur. On examination, the precordium is “overactive” and
associated with a systolic murmur and/or thrill (often continuous) that is maximal
in the pulmonary area. Cardiac enlargement is suggested by a thrusting left ven-
tricular apical impulse. The pulse pressure is widened and palmar pulses are fre-
quently palpable. If the heart failure is severe, sometimes no murmur is heard.
Hepatomegaly, jugular venous distention, and basilar rales can occur. If left untreated,
cyanosis develops (usually by five years of age) as pulmonary vascular resistance
increases above the systemic vascular resistance (Eisenmenger syndrome). Differen-
tial cyanosis may be noted with blueness of the feet and left hand, but not the face or
right hand.
In moderate-sized PDA the shunt is regulated primarily by the size of the ductus
arteriosus and the pulmonary artery pressure is only moderately elevated. As postna-
tal pulmonary vascular resistance falls, the shunt increases and heart failure occa-
sionally occurs. Usually, compensatory left ventricular hypertrophy leads to clinical
improvement and stabilization of symptoms by the second or third month of life.
Physical developmental delay, breathlessness, and fatigue sometimes occur, but most
children with moderate-sized PDA remain asymptomatic until the second decade of
life. On examination, the pulse is jerky, the precordium is mildly overactive, and the
apex of the left ventricle is palpable suggesting cardiac enlargement. The classical 76
continuous murmur is usually present by age 2-3 months. Eisenmenger syndrome

does not routinely develop with this lesion.
In small-sized PDA the left-to-right shunt is small and pulmonary vascular resis-
tance falls to normal after birth with no subsequent left ventricular failure. Symp-
toms are absent in infancy and childhood, but may appear much later in life as a
murmur on physical exam. Physical development is normal, the pulse is normal,
and the precordium is not overactive. The quality of the continuous murmur varies
greatly and is sometimes only detectable when the patient sits or stands upright.
The differential diagnosis includes truncus arteriosus, aortopulmonary window,
anomalous origin of the left or right pulmonary artery from the aorta, peripheral
pulmonary stenosis, venous hum, and a centrally positioned arteriovenous
malformation.
Diagnosis
Patent ductus arteriosus suspected on physical exam can be reliably documented
with echocardiography. Evaluation of left-sided chamber sizes and flow characteris-
tics can provide an estimate of shunt size. The electrocardiogram is nonspecific but
sometimes suggests left ventricular strain and hypertrophy, left atrial enlargement,
and right ventricular hypertrophy. The chest radiograph shows cardiomegaly, plethora
with or without interstitial pulmonary edema, or an enlarged ascending aorta.
Treatment
In a term infant with a large PDA, spontaneous closure is extremely unlikely and
treatment to physically close the PDA is indicated at the time of diagnosis. If symp-
toms are present, the procedure is done immediately. If no symptoms are present,
elective closure is planned within three months. Because indomethacin and other
medical interventions are not effective in term infants, some type of mechanical
closure is needed. The usual approach is open surgical ligation, but recently other
effective forms of therapy including transluminal placement of occlusive devices or
thoracoscopic occlusion using metal clips have become more popular.
In premature infants, early closure of PDA is beneficial. Aggressive intervention
is indicated once the diagnosis is established. The treatment for premature infants
includes supportive therapy with nutritional support, volume restriction, diuretics,
inotropic support, afterload reduction, ventilator support, and blood transfusion as
indicated. Oral or intravenous indomethacin is then administered unless contrain-
dicated. Failure to achieve closure of the ductus with indomethacin necessitates sur-
gical ligation via a high left lateral thoracotomy. Tube thoracostomy following the
procedure is optional.
Potential complications of PDA ligation include residual PDA, recurrent laryn-
geal or vagus nerve injury, pneumothorax, lung injury, chylothorax, and rarely liga-
tion of wrong structure (i.e., aortic isthmus, left pulmonary artery, or left bronchus).
Recurrent PDA following successful ligation is rare.
Outcomes
In term infants, life expectancy is normal after surgical closure of an uncompli-
cated PDA during infancy or childhood. For children who are operated upon in
76 infancy or childhood, the probability of early postoperative death is near zero. When
Patent Ductus Arteriosus 355
moderate or severe pulmonary vascular disease has developed preoperatively, late

deaths occur due to progression of the pulmonary disease.
In preterm infants, overall surgical mortality (30 day) ranges from 10-30%. The
mortality is not related to the interval between birth and operation but is more
related to birth weight and gestational age. Only about one-half of premature hospi-
tal survivors of PDA ligation are alive and well 1-5 years later. About one-third have
bronchopulmonary dysplasia and approximately one-sixth have severe complica-
tions of prematurity such as retrolental fibroplasia, blindness, and cerebral palsy.
The death rate from untreated PDA is estimated to be 30% in the first year of
life. The risk of death (usually secondary to congestive heart failure) is highest in the
first few months of life. Children with large PDAs who survive infancy without
treatment frequently die in the second or third decade from acute or chronic right
heart failure. For patients with untreated moderate-sized PDA, congestive heart fail-
ure causes death from the third and fourth decade onward. Subacute bacterial en-
docarditis sometimes occurs late in patients with small-sized PDAs.
Selected Readings
1. Gross RE, Hubbard JP. Surgical ligation of a patent ductus arteriosus. Report of
first successful case. JAMA 1939; 112:729.
2. Castaneda AR, Jonas RA, Mayer JE et al. Patent ductus arteriosus. In: Castaneda
AR et al, eds. Cardiac Surgery of the Neonate and Infant. Philadelphia: W.B.
Saunders 1994; 203-293.
3. Kirklin JW, Barratt-Boyes BG. Patent ductus arteriosus. In: Kirklin JW, Barratt-
Boyes BG eds. Cardiac Surgery: Morphology, Diagnostic Criteria, Natural His-
tory, Techniques, Results and Indications. New York: Churchill Livingstone 1993;
841-859.
76
Section XI: Congenital Malformations
of the Chest Wall, Abdominal Wall
and Perineum
CHAPTER 1
CHAPTER 77
Chest Wall Deformities

Marieta Reynolds
Pectus Excavatum
Incidence and Etiology
Of the congenital chest wall deformities, pectus excavatum (Figs. 77.1A and
77.1B) is the most common and occurs in approximately 0.2% of the population.
Boys are affected more frequently than girls at a ratio of 4 to 1. The deformity
usually presents at birth and becomes more prominent during the first few years of
life. The defect can become more pronounced between 8 and 10 years and again
during puberty. Children with severe deformities may have associated kyphosis and
some degree of scoliosis. Pectus excavatum is also associated with congenital heart
disease, lung cysts, Ehler-Danlos syndrome, Marfan’s syndrome and some muscu-
loskeletal anomalies. There is a familial tendency.
Symptoms related to a pectus excavatum vary depending on the severity of the
lesion. There is some evidence that the moderate to severe deformities can compro-
mise cardiorespiratory function. The symptoms of exertional dyspnea and tachycar-
dia begin in the teens and become progressively worse. Decrease in exercise tolerance
can develop.
Diagnosis
A child with a moderate to severe pectus excavatum should be evaluated with
baseline pulmonary function studies, an echocardiogram to diagnose associated mitral
valve prolapse and aortic root widening, and a CT of the chest with 3D reconstruc-
tion to demonstrate the full extent of the deformity.
Treatment
After the initial evaluation, return office visits are scheduled on a yearly basis to
quantify changes in the severity of the deformity and consider the most appropriate
time for correction. Children with mild deformities are followed yearly until the
lesion progresses or they reach their maximal growth in the late teens. Children with
moderate or severe deformities may undergo surgical correction after 6 years of age.
Surgical correction before 4 years of age has been shown to arrest chest wall growth
causing cardiorespiratory symptoms in some children.
Fig. 77.1A and 1B. Two examples

of pectus excavatum. Both are
moderate in depression and in
77 patients without symptoms other
than complaints about the cos-
metic appearance.
Chest Wall Deformities 359
A new technique to repair a pectus excavatum deformity has been described. It

entails placement of a bar behind the sternum through two small incisions. The bar
is then flipped over and the deformity corrected. The bar is removed 2 years later. 77
This technique shows promise and should be considered especially in children six to
twelve years of age.
The standard operation to correct the pectus excavatum deformity includes
subperichondrial resection of the deformed cartilage and an osteotomy of the ster-
num. Some type of temporary fixation of the sternum can be performed using stain-
less steel bars, Kirschner wires and other materials. Postoperative complications are
rare and include wound infection, seroma or hematoma and recurrence of the
deformity.
Pectus Carinatum
Pectus carinatum or “pigeon” or “chicken” breast occurs much less frequently
than pectus excavatum. Few physiologic studies have been performed on patients
with pectus carinatum deformities. Preoperative evaluation is identical to that for
pectus excavatum.
The surgical technique for repair of the carinatum deformity is tailored to the
child’s anatomy. The cartilages are removed and at least one osteotomy of the ster-
num made. A wedge resection of the sternum may aid in bringing the sternum in
line with the remainder of the chest. The intercostal muscle bundles may be reefed
up to pull the sternum backward. Some surgeons use a metal strut or other device to
fix the sternum in place. Complications and results are similar to the pectus excavatum
repair.
Poland’s Syndrome
Poland’s syndrome is manifested by congenital absence of the sternocostal head
of the pectoralis muscle, hypoplasia of the breast, absent nipple, missing ribs and
muscle, and syndactyly or absent fingers on the affected side. Preoperative evalua-
tion should include a chest CT with 3D reconstruction; occasionally an arteriogram
should be considered to evaluate the blood supply of local or distant combined
flaps. The chest wall reconstruction is performed using rib grafts or local muscle
flaps. The contralateral latissimus dorsi muscle can be used as a free tissue transfer. A
breast prosthesis can be inserted to complete chest wall reconstruction. Follow-up
MRI with 3D reformation can be used to evaluate the result.
Sternal Clefts and Ectopia Cordis
The rare cleft, fissure or split of the sternum can be classified as superior, inferior
or complete. These defects result from a failure of fusion of the sternum. Moderate
size clefts can be primarily repaired. Larger defects may require rib grafts or artificial
materials.
Ectopia cordis involves complete absence of the sternum with exposed heart.
Few survivors have been reported since it is often associated with complex congeni-
tal heart disease. A rare successful repair was accomplished using a two-stage proce-
dure. Initially, skin coverage of the heart was obtained with bilateral pectoral skin
flaps. The second stage included placement of methyl methacrylate struts which
were covered with bilateral pectoralis major muscle. Another repair was performed
in one stage using polytetrafluoroethylene membrane. Neither patient had associ-

ated congenital heart disease. In those patients with complex congenital heart dis-
77 ease, the skin coverage is done in the neonatal period and the cardiac defect is repaired
at a later date.
Selected Readings
1. Quigley PM, Haller, Jr. JA et al. Cardiorespiratory function before and after cor-
rective surgery in pectus excavatum. J Pediatr 1996; 128:638-43.
2. Haller JA, Jr., Colombani PM, Humphries CT et al. Chest wall constriction after
too extensive and too early operations for pectus excavatum. Ann Thor Surg 1996;
61(6):1618-24.
3. Nuss D. A 10-year review of a minimally invasive technique for the correction of
pectus excavatum. J Pediatr Surg 1998; 33(4):545-52.
4. Beer GM, Kompatscher P, Hergan K. Poland’s syndrome and vascular malforma-
tions. Br J Plastic Surg 1996; 49(7):482-4.
5. Knox L, Tuggle D, Knott-Craig CJ. Repair of congenital sternal clefts in adoles-
cence and infancy. J Pediatr Surg 1994; 29(12):1513-6.
6. Kim KA, Vincent WR, Muenchow SW et al. Successful repair of ectopia cordis
using alloplastic materials. Ann Plastic Surg 1997; 38(5):518-22.
7. Amato JJ, Zelen J, Talwalkar NG. Single-stage repair of thoracic ectopia cordis.
Ann Thorac Surg 1995; 59(2):518-20.
8. Hornberger LK, Colan SD, Lock JE et al. Outcome of patients with ectopia cordis
and significant intracardiac defects. Circulation 1996; 94(9 Suppl):1132-7.
CHAPTER 1
CHAPTER 78
Abdominal Wall Defects

Grant H. Geissler
Abdominal wall defects represent unique challenges to pediatric surgeons and

offer insight into normal fetal development. Omphalocele and gastroschisis are the
major anomalies encountered in the neonate. The first description of an abdominal
wall defect dates back to 1634, when Ambroise Paré first described an omphalocele—
a defect in abdominal wall musculature and skin with protrusion of abdominal vis-
cera contained within a membranous sac. Later, Calder described a child with
gastroschisis—the defect in the abdominal wall was displaced to the right of the
umbilicus and eviscerated bowel was not covered by a membrane. Survival of an
infant born with an abdominal wall defect, especially gastroschisis, was unusual
before the advent of modern antibiotics, nutritional support, and neonatal intensive
care capabilities.
Incidence
An accurate estimate of the incidence of gastroschisis and omphalocele is diffi-
cult to obtain due to the under reporting of stillbirths, confusion in defining rup-
tured omphaloceles as gastroschisis defects, and the voluntary terminations of
pregnancies with sonographic evidence of abdominal wall defects. Reports from the
United States suggest a combined incidence of 1 in 2000 live births, while estimates
from Liverpool and British Columbia are closer to 1 in 4,000 live births. In the
1960s and 1970s, omphaloceles outnumbered gastroschisis 3:1, but over the last 20
years gastroschisis defects have predominated 2-3:1. This increase in incidence may
represent increased selective termination of pregnancies of omphaloceles, more ac-
curate classification of defects, or an actual increase in the gastroschisis birth rate.
Males and females are equally affected by gastroschisis, and there may be a slight
male predominance in omphalocele. No predilections based on race, maternal age,
parity or geography have been substantiated.
Etiology
No specific etiologies for abdominal wall defects have been identified in humans;
however, an understanding of their embryology lends support to several theories.
Omphalocele
At approximately the fourth week of gestation, the fetal abdominal wall is sepa-
rated only by somatopleure, a thin membrane of ectoderm and mesoderm that is
later replaced by simultaneous ingrowth of four mesodermal tissue folds in cranial,
caudad, and lateral to medial directions. Cranial downgrowth forms the thoracic
and epigastric wall; caudad ingrowth forms the hypogastrium, bladder, and the hind-
gut. By the sixth week, further ingrowth occurs from lateral paravertebral myotomes.
These ingrowths flatten medially to form the rectus abdominus muscles and fuse in
the midline to complete enclosure of the abdominal cavity. It is theorized that the
78 failure of ingrowth of lateral mesoderm gives rise to an arrested somatopleure defect
characteristic of a standard omphalocele, and failure of ingrowth of cranial and caudad
elements accounts for the spectrum of defects which accompany pentalogy of Cantrell,
exstrophy of the urinary bladder, cloacal exstrophy, and imperforate anus. These
fusion defects are thought to occur within the first 4-7 weeks of gestation and can be
associated with other first trimester developmental anomalies and a 10-30% inci-
dence of chromosomal anomalies (i.e., autosomal trisomies of 12, 18, and 21).
Gastroschisis
Gastroschisis appears to arise from a specific weakness in the abdominal wall
with secondary rupture and herniation of abdominal viscera. More rapid dissolu-
tion of the right umbilical vein occurs outside the standard period of organogenesis,
leaving an area of relative weakness in the mesenchyme through which bowel or
abdominal viscera can herniate and eventually rupture. This theory explains the
95% incidence of the defect being to the right of the umbilicus. Gastroschisis is
usually an isolated mechanical defect and typically not associated with an increased
incidence of other developmental anomalies.
Omphalocele
At birth, omphalocele (Fig. 78.1) is recognized as a central defect of the abdomi-
nal wall beneath the umbilical ring. It is greater than 4 cm (defects less than 4 cm are
generally called hernias of the cord) and is covered by a membranous sac or amnion.
The umbilical cord inserts directly into the sac in an apical or occasionally lateral
position. The sac may rupture in utero in 10-18% or from the delivery process in
4%. If the sac is intact, it contains normal appearing abdominal viscera including
the liver in 48%. Giant omphaloceles have sacs that replace most of the abdominal
wall, contain most of the intra-abdominal viscera, and have associated underdevel-
oped peritoneal cavity and pulmonary hypoplasia.
The incidence of associated major congenital anomalies has previously been
estimated at 35%, but with improvement in neonatal survival, data collection, and
imaging techniques, more recent series report associated anomalies in up to 81%.
Cardiovascular defects are present in 20% of the patients and include tetralogy of
Fallot, ASD, and VSD most commonly. Defects associated with cranial fold failure
include congenital heart disease, diaphragmatic hernia, ectopia cordis, sternal cleft,
and when all of these elements are present represent the pentalogy of Cantrell. Defects
of caudad fold ingrowth may include imperforate anus, genitourinary malforma-
tions, bladder or cloacal exstrophy, colon atresia, sacral and vertebral anomalies, and
meningomyelocele.
Abdominal Wall Defects 363
78
Fig. 78.1. Modest omphalocele with completely intact membrane and cord at infe-
rior margin.
Syndromes associated with omphalocele include autosomal trisomies 13, 18,

and 21 in up to 30%. Also associated is the Beckwith-Weideman syndrome consist-
ing of macroglossia, hypoglycemia, and visceromegaly.
Gastroschisis
At birth, gastroschisis (Fig. 78.2) is recognized by an isolated opening in the
abdominal wall to the right (95%) of the umbilicus with free evisceration of abdominal
contents. In utero exposure of bowel to amniotic fluid eventually causes thickening,
shortening, and the development of a thick fibrous peel which seems accentuated
with prolonged exposure and term deliveries. Associated defects are uncommon and
relate mechanically to the abdominal wall defect. Evisceration of the bowel leads to
malrotation. Constriction of the base of the herniated intestines may cause intesti-
nal stenosis, atresia, and volvulus (usually ileal in location). Undescended testicles
are also more common with this defect. Upon repair, intestinal edema, thickening,
and the fibrous peel eventually resolve coinciding with resolution of a prolonged
ileus that may last up to 4 weeks. Late appearance of necrotizing enterocolitis (NEC)
has been reported in up to 17% of patients following standard repairs. Patients with
gastroschisis are more likely to be born preterm or small for gestational age (SGA)
compared to omphalocele patients who are usually term, large babies.
Treatment
Prenatal diagnosis of an abdominal wall defect is not in itself an indication for a
cesarean delivery. Prenatal management calls for and evaluation for associated anoma-
lies including a complete fetal ultrasound and possible amniocentesis. For gastroschisis
78
Fig. 78.2. Gastroschisis without any signs of a membrane and demonstrating the
inflammatory reaction and thickening of the bowel serosa that occurs on exposure
to amniotic fluid.
patients, serial ultrasound examinations showing progressive dilatation and thick-

ening of bowel has been correlated with poor intestinal function and is used in some
centers as an indication for induction of labor.
Neonatal management begins with the preservation of eviscerated bowel or intact
amnion with sterile moistened saline gauze dressings, transparent film wrap, or bowel
bags. Intravenous fluids at 1.25-1.5 times maintenance and antibiotics are adminis-
tered. Great care is taken to conserve body heat. A thorough examination for associ-
ated anomalies is performed including:
1. plain radiographs of the chest, spine and pelvis,
2. echocardiogram,
3. renal ultrasonography, and
4. chromosomal analysis.
Omphalocele
Primary closure of the small to medium sized omphalocele is preferred. This
includes excision of the sac, possible correction of the associated malrotation, and
general inspection of the abdominal contents. Intraoperative alternatives to primary
closure include prosthetic patch closure, simple closure of mobilized skin flaps, or
placement of a silo for sequential tightening and staged closure. Giant omphaloceles
or patients who are not suitable candidates for anesthesia may be treated with topi-
cal application of Betadine® ointment or silver sulfadiazine to the intact sac. This
allows secondary eschar formation and eventual epidermal ingrowth. Residual
abdominal wall hernias are then repaired at one year of age.
Abdominal Wall Defects 365
Gastroschisis
Primary closure of gastroschisis usually begins with enlarging the abdominal wall
defect to allow for the reduction of intestinal contents. The abdominal wall is gently
stretched to enlarge the peritoneal cavity, and preoperative enemas may be helpful
to decompress the colon. Severe matting of the bowel or peel formation may preclude
an immediate repair of associated atresia or stenosis. These can be reduced and repaired
78
in 6-8 weeks when the bowel injury/peel has resolved. In cases of volvulus and necrosis,
nonviable bowel is resected. Bowel continuity is restored primarily or more rarely
proximal enterostomies are performed as needed. Complete reduction of bowel con-
tents under minimal pressure is preferable, but a prosthetic patch, skin flaps, or
staged closure may be necessary. Parenteral nutrition is begun postoperatively and
continued until adequate oral nutrition is attained. Careful postoperative fluid man-
agement, postoperative antibiotics, and adequate ventilatory support contribute to
successful outcomes.
Outcomes
Morbidity and mortality rates with omphalocele closure are closely tied to pre-
maturity, large-sized defects, and major associated anomalies. Most modern series
report survival rates form 71-93%. Children with gastroschisis often have a pro-
longed ileus, but once bowel function returns, these children thrive and ultimately
do well since they have few associated anomalies. At present, survival rates are gen-
erally 90-95%.
Once children in both groups survive the neonatal surgery and achieve adequate
levels of oral nutrition and growth, long-term survival depends almost exclusively
on the presence of other anomalies and their associated morbidity rates.
Selected Readings
1. Vermeij-Keers C, Hartwig NG, van der Werff JF. Embryonic development of the
ventral body wall and its congenital malformations. Sem Pediatr Surg 1996; (5):82-9.
2. Dykes EH. Prenatal diagnosis and management of abdominal wall defects. Sem
Pediatr Surg 1996 ; (5):90-4.
3. Snyder CL. Outcome analysis for gastroschisis. J Pediatr Surg 1999 ; (34):1253-6.
4. Rinehart BK, Terrone DA, Isler CM et al. Modern obstetric management and
outcome of infants with gastroschisis. Obstet Gynecol 1999 ; (94):112-6.
5. Schuster SR. A new method for staged repair of large omphaloceles. Surg Gynecol
Obstet 1967 ; (124):297-300.
6. Rowe MI et al. Abdominal wall defects. In: Rowe et al, eds. Essentials of Pediatric
Surgery. St. Louis: Mosby 1995; 431-440.
CHAPTER 79
Anorectal Malformations
P. Stephen Almond
Incidence
The incidence of imperforate anus is one in every 5,000 live births, with cloaca
malformations accounting for 10%. Males (58%) are more commonly affected than
females (42%).
Etiology
In the third week of gestation, the embryo consists of an amniotic cavity and a
larger yolk sac separated by a trilaminar disc consisting of ectoderm (amniotic side),
mesoderm (middle), and endoderm (yolk sac side). The disc then begins a cranio-
caudal folding process that tubularizes a portion of the endoderm into what will
eventually become the hindgut. The hindgut joins the allantois and the mesonephric
ducts to form the cloaca. At the end of the cloaca, endoderm of the cloaca is in direct
contact with surface ectoderm creating the cloacal membrane. During development,
this membrane moves posteriorly and inferiorly.
Cloacal division into rectum and urogenital tract is initiated by the caudal move-
ment of tissue between the allantois anteriorly and the hindgut posteriorly. This
cranio-caudal movement stops at the verumontanum. At seven weeks, cloacal divi-
sion is completed by lateral ingrowth of mesenchyme, thereby completing the uro-
genital septum and forming the perineum. The perineum divides the cloacal
membrane into the urogenital membrane anteriorly and the anal membrane poste-
riorly. Mesenchymal swellings then surround the anal membrane. The anal pit, a
depression in the ectoderm at the anal membrane, develops in the eighth week and
the membrane perforates in the ninth week.
Anorectal malformations occur when this process fails. The exact etiology of
failure is currently unknown.
Classification
Previously, infants with imperforate anus were classified based on the relation-
ship of the rectal terminus to the levators. This was determined by inverting the
infant and taking a transpelvic xray (invertogram). Rectal termini above the pubo-
coccygeal line (a line drawn between the pubis and coccyx) are above the levators
and designated high lesions. Termini between the pubococcygeal line and the lowest
quarter of the ossified ischium (the “I” point) are translevator and designated inter-
mediate lesions. Those below the “I” point traverse the levators and are designated
low lesions.
Anorectal Malformations 367
More recently, a treatment-based classification system was proposed. In this sys-

tem, infants are separated into two groups based on their need for a colostomy.
Infants with a cutaneous fistula, anal stenosis, or anal membranes can undergo pri-
mary repair without protective colostomy. In contrast, those with rectourethral fis-
tula, rectovesical fistula, anorectal agenesis without fistula, rectal atresia, vestibular
fistula, vaginal fistula, or cloacal malformations require protective colostomy before
primary repair.
79
Most infants with imperforate anus are referred because no anal opening is iden-
tified (Fig. 79.1) on the newborn screening exam or because of failure to pass meco-
nium. Although most are healthy, full-term infants, associated congenital anomalies
are common. These anomalies include vertebral anomalies, limb anomalies, heart
defects, and Downs syndrome. The presence of any one of these mandates ruling
out the others. The two most common cardiac anomalies associated with anorectal
malformations are tetralogy of fallot and ventricular septal defects. Duodenal atresia
and Hirschsprung’s disease (2%) are occasionally identified in infants with anorectal
malformations.
Diagnosis
Imperforate anus is a clinical diagnosis. Inspect the perineum for meconium
and/or a perineal fistula (Figs. 79.2 and 79.3). Determine gluteal and gluteal cleft
development. Determine the presence or absence of an anal pit and the extent of
sphincter development by eliciting an anal wink. This can be done by gently scratching
the perianal skin. Preoperative tests are aimed at determining the presence of a fis-
tula, the location of the rectal terminus, and if there are any associated lesions. To
determine the presence and location of a cutaneous fistula, observe the newborn for
24-48 hours. Meconium on the perineum confirms a cutaneous fistula and a low
lesion (Figs. 79.4 and 79.5). If there is no meconium, place a radiopaque marker on
the perineum and obtain a prone, cross-table lateral xray of the pelvis. Air, acting as
a contrast medium, will delineate the rectal terminus and differentiate a low vs. high
lesion. To determine the relationship of the urinary tract to the rectum, strain the
urine with diaper or gauze and obtain a urinalysis. Meconium in the urine docu-
ments a communication between the bowel and the urinary tract. A voiding cys-
tourethrogram (VCUG) will document the fistula. Passage of a nasogastric tube
(esophageal atresia), cardiac echo (cardiac anomaly), abdominal ultrasound (renal
agenesis), plain films (vertebral anomalies), spine ultrasound and lumbar magnetic
resonance imaging (tethered cord) will rule out associated anomalies. Female infants
with a single perineal opening, or cloaca, need urgent evaluation of the urinary
tract.
Treatment
The infant is kept NPO, on peripheral hyperalimentation and antibiotics until
colostomy or primary repair is performed. Infants with a cutaneous fistula, anal
stenosis, or anal membrane undergo a minimal posterior sagittal anorectoplasty
(PSARP) or a transposition anoplasty (Pott’s anoplasty). Infants with a flat bottom,
meconium in the urine, or other fistula (i.e., urethral, vaginal, vestibular) undergo
79
Fig. 79.1. High imperforate anus in a male. Median raphe is flat and without any
signs of meconium extrusion
Fig. 79.2. Young

female with ante-
rior ectopic anus.
Arrows mark the
posterior edge of
the vaginal open-
ing and the anus.
These two struc-
tures are too
close, and the
anal opening lies
outside the anal
dimple.
Fig. 79.3. Low imperforate

anus in a female with fistula
visible at the posterior
fourchette (vestibular fistula).
79
Fig. 79.4. Low imperforate

anus in a male. Well devel-
oped raphe that will prob-
ably demonstrate fistula
with meconium extrusion
over first 1-2 days of life.
Fig. 79.5. Low imperforate anus

in a male. “Bucket handle”
shown at site of covered anus
with small amount of meconium
extruding (arrow).
79
colostomy. Females with a cloaca also undergo colostomy, and if necessary,

vaginostomy and/or urinary diversion. Postoperatively, the mucous fistula is irri-
gated (to remove impacted meconium) and a distal colostogram is performed to
show the distal rectum and fistula (Fig. 79.6). The child is then followed closely to
insure weight gain and adequate colostomy function. If all goes well, a PSARP is
performed between 2 and 12 months of age.
Throughout the operation, an electrical stimulator is used to determine the ex-
act location of the external sphincter and insure that dissection stays in the midline.
The muscle complex and levators are divided, the rectum is identified and opened,
and the fistula is identified. Because there is no dissection plane between the urethra
and rectum at the fistula, a small portion of the anterior wall of the rectum is left on
the urethra, and the rectum is separated from the urethra superiorly. The fistula is
closed; the rectum is mobilized and pulled down to the perianal skin. The perineal
body is reconstructed anteriorly and the rectum is secured within the muscle com-
plex fibers. The anoplasty is completed with interrupted absorbable suture.
The first morning following the reconstruction, the child is fed and converted to
oral pain medication. The Foley catheter and IV antibiotics are continued for five
days. The child is discharged after spontaneous voiding. At two weeks, the anus is
sized with a Hagar dilator in the office and the mother/caregiver is instructed on
home dilatations. The colostomy may be closed six weeks later.
79
Fig. 79.6. High imperforate anus in a male undergoing colostogram. Arrows trace
the path of the colourethral fistula.
Outcomes
Results depend upon the level of the lesion and the sacrum. In general, infants
with low lesions have an excellent outcome with constipation (40%), soiling (13%),
and diarrhea (4%) accounting for the majority of complications. Infants with high
lesions have a higher incidence of these complications: constipation (35%), soiling
(54%), diarrhea (12%).
Selected Readings
1. Pena A. Anorectal malformations. Seminars in Pediatric Surgery 1995; 4:35-47.
2. Pena A. Surgical management of anorectal malformations: A unified concept. Pediatr
Surg Int 1988; 3:82-93.
3. Kiely EM, Pena A. Anorectal malformations. In: O’Neill Jr. JA et al, eds. Pediatric
Surgery, 5th edition. St. Louis: Mosby, 1998; 1425-48.
4. Shaul DB, Harrison EA. Classification of anorectal malformations’ initial approach,
diagnostic tests, and colostomy. Sem Pediatr Surg 1997; 6:187-195.
CHAPTER 80
Urogenital Sinus, Cloaca, and Cloacal

Exstrophy
Robert M. Arensman
Definitions
These three rare congenital anomalies represent various stages of arrested devel-
opment of abnormal embryogenesis. In infants with urogenital sinus, final develop-
ment and separation of the urinary and genital structures fail to occur so that the
urethra and vagina share a common external orifice. In cloaca, the arrested develop-
ment produces a situation that has urethral, vaginal, and rectal openings all sharing
a common single external orifice. The faulty embryogenesis in cloacal exstrophy
compounds the problem by rupture onto the anterior abdominal wall. These chil-
dren have a central exposed bowel field with 1-4 orifices (ileum, colon, appendix(ces)),
divided hemibladder fields, an omphalocele, and imperforate anus.
Embryogenesis
Complex development of two membranes (cloacal membrane and urorectal sep-
tum) creates the separation of the anterior urogenital structures from the posterior
rectal structures. Arrested development of these processes appears to explain and
create urogenital sinus and cloaca. However, some further problem arises to create
cloacal exstrophy. The exact nature of this problem is currently unknown; and
although there are two prominent theories, neither has much proof for support at
this time.
Incidence
All three conditions are rare, even in large children’s medical centers. The inci-
dence varies from 1:150,000 to 400,000 live births. At present only 20-30 yearly
cases of cloacal exstrophy are expected in the United States. Male to female pre-
dominance has been recorded as high as 2.5:1.
Associated Anomalies
These children have normal intelligence and grow well unless the rare problem
of short bowel occurs. However, they all have a host of associated anomalies, espe-
cially in four areas: genitourinary, gastrointestinal, vertebral, and lower extremity. As
many as 70-85% of these infants have anomalies remote from the basic defect. Most
common are hydronephrosis or hydroureter, renal agenesis, pelvic kidney, duplica-
tions or crossed fused ectopia.
Urogenital Sinus, Cloaca, and Cloacal Exstrophy 373
80
Fig. 80.1. Typical cloacal exstrophy with: 1) omphalocele (black and white arrow),
2) divided bladder (large solid arrows), 3) central bowel mucosa (large white ar-
row) with intussucepted small bowel, and 4) labia (small black arrows).
The associated gastrointestinal anomalies consist of malrotation, atresia/steno-

sis, duplications, Meckel’s diverticula, absent or double appendix, and rarely short
bowel syndrome. Vertebral anomalies include myelodysplasia absent segments, and
hemivertebrae. Central nervous system disorders other than myelodysplasia are rather
rare. Finally, lower extremity malformations are present in up to 25% of these chil-
dren and include such things as clubfeet, dislocated hips, or missing portions of a
lower limb.
Diagnosis
Most of these infants can be correctly diagnosed on physical examination alone.
In females, failure to confirm three perineal orifices with proper anatomic relation-
ships allows a very accurate preliminary diagnosis. Diagnostic studies are needed to
document extent of urogenital and rectal fusion and the presence of associated anoma-
lies. In most cases, ultrasonography is done initially to seek evidence for obstruction
in the urinary system or vaginal atresia with hydrocolpos. This is followed by con-
trast injections via orifices. Skeletal radiographs are done, and the spine is imaged
with ultrasound and/or magnetic resonance imaging (MRI) to evaluate for possible
tethered cord.
Treatment
The first phase of treatment depends on making an accurate diagnosis and
assessing whether the infant is strong, has favorable anatomy and a lack of associated
anomalies. If these conditions are met, single stage repair may be undertaken in the
neonatal period. If these conditions are not met, it is advisable to plan for a multi-
stage repair perhaps extending over several years. In any event, there are reliable
operations for each of the anomalies comprising these conditions. Most of the con-
ditions can be corrected or greatly improved. In general, the principle of repair is to
separate the various systems and repair them as well as possible. Continence in uri-
nary or bowel function cannot be guaranteed but is frequently improved. If control
is not satisfactory for the future, reasonable forms of diversion allow functionally
acceptable results.
80 Results
Survival and good neurological outcome are common for these children. Most
long-term problems are associated with urinary and bowel function. Results are
mixed with only about half of these children achieving satisfactory control through
sphincter use. The rest are reconstructed with drainage procedures, pouches, or per-
manent ostomies that allow a functional status in society.
Selected Readings
1. Spencer R. Exstrophia splanchnica (exstrophy of the cloaca). Surgery 1964;
57:751-766.
2. Hendren WH. Repair of cloacal anomalies: Current techniques. J Pediatr Surg
1986; 21:1159-76.
3. Hendren WH. Further experience in reconstructive surgery for cloacal anomalies.
J Pediatr Surg 1982; 17:695-717.
4. Pena A, deVries PA. Posterior sagittal anorectoplasty: Important technical consid-
erations and new applications. J Pediatr Surg 1982; 17:796-811.
5. Warner BW, Ziegler MM. Exstophy of the cloaca. In: Ashcraft KW, Holder TM
eds. Pediatric Surgery, 2nd Edition. Philadelphia: W.B. Saunders Company 1993;
393-401.
Section XII: Functional and Acquired
Disorders of the Esophagus
CHAPTER 81
Gastroesophageal Reflux
Grant H. Geissler
Introduction and Incidence
Although all infants and children vomit occasionally, the term gastroesophageal
reflux is reserved for more severe, prolonged or symptomatic emesis not related to
anatomic obstruction or acute illnesses. Isolated gastroesophageal reflux occurs most
frequently in the first 9-12 months of life but can arise anytime during childhood.
Infantile gastroesophageal reflux frequently improves spontaneously around one year
of age as
1. the lower esophageal sphincter (LES) tone improves,
2. the child adopts a more upright posture,
3. the child uses abdominal muscles as accessory muscles of respiration less,
and
4. the child progresses to a general diet.
Specific diagnostic evaluations, medical and surgical treatments are usually reserved
for patients with pathologic reflux, defined as reflux which causes injury to another
organ system (i.e., esophagitis, pneumonia) or causes failure of the infant or child to
thrive. Approximately 1 in 300-1000 children have excessive, passive reflux across
an incompetent LES and require medical or surgical therapy.
Embryology and Anatomy
The esophagus is composed of cervical, thoracic, and intra-abdominal segments
and arises from the embryologic foregut. Its separation from the respiratory system
and relation to the stomach and diaphragm is completed between the fourth to
seventh week of fetal gestation. The esophagus travels through the esophageal hiatus
in the diaphragm bounded laterally by the diaphragmatic crura. The esophagus en-
ters the stomach forming an acute angle known as the angle of HIS.
The lower esophageal sphincter is a physiologic high pressure zone located in the
intra-abdominal esophagus adjacent to the body of the stomach. LES dysfunction,
widening of the angle of HIS, paraesophageal or sliding hiatal hernias, or decreased
gastric mobility may all contribute to the development of pathologic gastroesoph-
ageal reflux. In addition, decreases in distal esophageal motility may impair clear-
ance of refluxed gastric contents.
Children with neurological impairments are more commonly afflicted with patho-
logic gastroesophageal reflux.
Gastroesophageal Reflux 377
Clinical symptoms of gastroesophageal reflux arise from prolonged exposure of
esophageal squamous mucosa to gastric acid, aspiration of gastric contents into the
airway, or growth impairment secondary to inability to hold down adequate nutri-
tion. The younger patients tend to have more respiratory complications of patho-
logic gastroesophageal reflux, while older patients more commonly present with
esophageal symptoms.
Respiratory symptoms may be difficult to attribute solely to reflux but include
bronchospasm, laryngospasm, hoarseness, pneumonia, apnea, and choking spells.
Reflux has been suspected as a contributing factor to sudden infant death syndrome
(SIDS) or acute life threatening event (ALTE) apnea. Multichannel recording of
esophageal pH, heart rate, respiratory rate, and EEG have linked drops in esoph-
ageal pH immediately preceding apneic events. Recurrent aspiration pneumonia 81
may follow either an upper posterior or lower posterior lobe pattern, and broncho-
scopy may disclose laryngeal edema and airway washings filled with lipid-laden
macrophages. Coughing, choking, or wheezing symptoms may worsen at night when
the child is recumbent, and gastric acid is not buffered by food. Poor sleep patterns,
dental caries, and recurrent otitis media has been associated with severe gastroe-
sophageal reflux in children.
Esophageal symptoms arise from the prolonged contact of esophageal mucosa
with gastric acid. Esophagitis may be manifest as irritability in infants and heart-
burn in older patients. Esophagitis may be mild and only discovered by esophageal
biopsy or may progress to gross inflammation, ulceration, and eventual stricture
formation. Columnar metaplasia is an adaptive response to repetitive esophageal
irritation and is commonly known as Barrett’s esophagitis. This metaplasia may
predispose to adenocarcinoma of the esophagus, especially when evidence of dyspla-
sia is present. Hematemesis, heme positive stools, and chronic iron deficiency anemia
also arise from ongoing esophagitis. Sandifer’s syndrome refers to voluntary arching
of the back and neck to improve peristalsis and improve esophageal emptying. Chil-
dren with neurological impairment, diffuse foregut dysmotility, or esophageal atre-
sia may have esophagitis from motility disturbances which alter LES function and
impair clearance of refluxed acid from the esophageal mucosa. Delayed gastric mo-
tility may also contribute to pathologic reflux, especially in patients with neurologi-
cal impairment.
Nutrition lost from repetitive vomiting may be so profound and cause growth
disturbances. Patients are particularly prone to vomit with coughing, exertion, cry-
ing, or after feeding. Since eating promotes reflux, some children with esophagitis
and pain avoid eating voluntarily to reduce discomfort.
Diagnostic Studies
Once pathologic gastroesophageal reflux is suspected, diagnostic evaluation usu-
ally begins with a barium upper GI examination. The value of this study is to explore
anatomic problems (esophageal stricture, hiatal hernia), evaluate esophageal posi-
tion, stomach size, and to exclude outflow obstructions such as duodenal malforma-
tions or malrotation. Since barium is not a physiologic medium and the test is of
limited duration, the absence of demonstrable reflux does not exclude it and the
study may be normal in up to 50% of the known refluxers. Esophageal pH monitoring
Fig. 81.1. Severe case of gastro-

esophaeal reflux with wide open lower
esophageal sphincter (curved arrow)
and reflux to the thoracic inlet (short
arrow).
81
is a more accurate study to document suspected gastroesophageal reflux. A pH probe

is placed in the distal esophagus and continuous monitoring of esophageal pH over
a 24 hour period is performed. The frequency of reflux episodes (esophageal pH ≤
4) and specific duration of episodes is quantified.
Nuclear medicine scans may be useful in diagnostic and operative planning.
Esophageal scintiscans are performed by placing a known quantity of radioisotope
in the stomach. Serial scans quantitate the amount of isotope refluxed into the esopha-
gus, thereby documenting gastroesophageal reflux. Gastric scintiscans employ simi-
lar isotopes, but the rate of gastric emptying is measured as the isotope travels distally
out of the stomach. Significant delays in emptying may warrant additional prokinetic
medication or a gastric drainage procedure.
Esophagoscopy is a final diagnostic that is used to evaluate patients with gastroe-
sophageal reflux. Esophageal biopsies confirm esophagitis, quantitate degrees of se-
verity, or detect presence of columnar metaplasia. Anatomic problems such as
ulcers, strictures, or hiatal hernias can also be documented.
Medical Therapy
Once a diagnosis of pathologic gastroesophageal reflux is secured, medical therapy
is instituted. This includes dietary modification (i.e., formula changes, thickened
feeds), adoption of an upright posture for eating and in the postprandial period, and
avoidance of positions that increase intra-abdominal pressure. Administration of
Gastroesophageal Reflux 379
antacids minimizes the inflammatory effect of gastric acid on esophageal or bron-

chial mucosa. Prokinetic medications (i.e., cisapride, metoclopramide) may improve
esophageal peristalsis, increase gastroesophageal tone, improve gastric emptying, and
have been helpful in controlling gastroesophageal reflux when combined with other
medical modalities. A combination of these modalities controls reflux in up to 90-95%
of children.
Surgical Therapy and Long-Term Results
Surgical therapy is employed to control gastroesophageal reflux when medical
therapy of appropriate dose and duration fails, or when severe life threatening events
preclude a medical approach. The goal of surgical therapy is to restore the normal
anatomic relationship of the gastroesophageal junction within the abdomen, repair
hiatal hernias if present, recreate the angle of His, and improve gastric emptying if 81
necessary.
Fundoplication procedures are the current operation of choice for surgical con-
trol of gastroesophageal reflux. Two approaches are commonly employed, the Nis-
sen or Thal fundoplication. A large variety of other surgical repairs are rarely used in
children.
A Nissen fundoplication involves a retroesophageal crural repair, followed by a
360˚ wrap of the gastric fundus around the esophagus. The fundus is sutured to
itself and to the esophagus anteriorly. Care is taken to avoid vagal nerve trunks. A
“loose” Nissen wrap is preferred. A Maloney dilator is placed within the esophagus
to fully dilate it and prevent narrowing while the wrap is constructed. Nissen wraps
control reflux in up to 92% of patients, but may require revision in 12% (i.e., slip-
ping or loosening with recurrent reflux), and eventually in up to 30% of patients
who are neurologically impaired.
A Thal fundoplication also begins with a crural repair, but then the gastroesoph-
ageal junction is mobilized and pulled downward into the stomach. The fundus is
sutured to the esophagus and hiatus circumferentially. This approach may allow
more patients to burp and vomit to some degree postoperatively, provides greater
than 90% control of reflux symptoms, but may have a higher recurrence rate over
time.
Reoperative surgery may be performed if reflux symptoms recur following previ-
ous successful fundoplication. Redo fundoplication rates range from 5-20% rou-
tinely and are higher in patients with neurologic impairment.
Selected Readings
1. Fonkalsrud EW, Ament ME, Berquist W. Surgical management of the gastroe-
sophageal reflux syndrome in childhood. Surgery 1985; 97:42.
2. Ashcraft KW, Holder TM, Amoury RA. Treatment of gastroesophageal reflux in
children by Thal fundoplication. J Thorac Cardiovasc Surg 1981; 82:706.
3. Pearl RH et al. Complications of gastroesophaeal antireflux surgery in neurologi-
cally impaired versus neurologically normal children. J Pediatr Surg 1990; 25:1169.
4. Wheatley MJ et al. Redo fundoplication in infants and children with recurrent
gastroesophageal reflux. J Pediatr Surg 1991; 26:758.
5. Boix-Ochoa J, Rowe MI. Gastroesophageal reflux. In: O’Neill Jr. JA ed. Pediatric
Surgery, 5th Edition. St. Louis: Mosby 1998; 1007-1028.
CHAPTER 82
Achalasia
David Bentrem
Incidence
Achalasia is a functional disorder in which the lower esophageal sphincter (LES)
fails to relax with swallowing. The incidence of achalasia in children is one case per
10,000. Consequently, only 2-5% of all reported cases of achalasia occur in chil-
dren. In contrast to adults, boys are more commonly affected than girls (1.6:1).
Most major pediatric centers encounter less than one case per year.
Etiology
The etiology of achalasia is poorly understood in either children or adults. Gan-
glion cell defects within the myenteric plexus of the lower two thirds of the esopha-
gus and a lack of nitric oxide synthase activity in the lower esophagus, cardia and
gastric fundus are both postulated. But neither theory is confirmed, and a direct
cause/effect relationship has not been established.
Less commonly, secondary achalasia results from diseases of the vagal dorsal motor
nuclei (i.e., polio, diabetic autonomic neuropathy, amyloidosis, sarcoidosis). In
Chagas’ disease, Trypanosoma cruzi destroys the myenteric plexus of the esophagus
causing symptoms and clinical findings similar to achalasia.
Pathophysiology
Normally, the LES remains tonically constricted (with an intraluminal pressure
of about 30 mmHg) in order to prevent reflux of highly acidic gastric contents into
the esophagus. With swallowing, “receptive relaxation” of the LES precedes the peri-
staltic wave. In achalasia the musculature of the lower esophagus remains spastically
contracted, and the LES fails to relax as food approaches. Over time, the esophagus
becomes tremendously dilated. Chronic inflammation and ulceration of the mu-
cosa from stasis, causes severe pain, and puts the child at risk for rupture.
The onset of symptoms is usually before 15 years of age with a mean of 8-9 years.
The primary symptoms are:
1. progressive dysphagia of first liquids and then solids,
2. vomiting, and
3. retrosternal pain.
As the disease progresses and the proximal esophagus becomes distended, chil-
dren vomit retained food and liquid. The onset of symptoms is often insidious.
Achalasia 381
Because the disease is so rare, the diagnosis is frequently delayed, and the symptoms
are attributed to psychological problems. Younger children fail to gain weight, and
teenagers lose weight over a period of several months. Nocturnal regurgitation may
result in recurrent pneumonias.
Diagnosis
A dilated esophagus with an air-fluid level on plain chest radiograph is suspi-
cious for achalasia. The same radiograph may also demonstrate signs of recurrent
aspiration pneumonitis. A barium swallow outlines a dilated esophagus that nar-
rows concentrically to a “beak” at the cardioesophageal junction. Fluoroscopy can
demonstrate disordered and retrograde peristalsis in the dilated proximal esophagus.
Endoscopy is used to exclude a congenital cause for the abnormally dilated esopha-
gus. Esophagoscopy demonstrates concentric narrowing of the distal esophagus, of-
ten without signs of esophagitis. The LES will relax to allow passage of the scope
into the stomach, ruling out congenital stricture, cartilaginous remnants of the esoph-
ageal wall, or stenosis secondary to gastroesophageal reflux disease (GERD). Defini- 82
tive diagnosis is made by esophageal manometry. Manometric studies confirm the
three major abnormalities of achalasia:
1. aperistalsis,
2. incomplete relaxation of the LES with swallowing, and
3. increased resting tone of the LES.
Hyperperistalsis, disorganized peristalsis and retrograde peristalsis are occasion-
ally observed.
Treatment
The goal of treatment is to relieve the functional obstruction. Pharmacologic
treatment is based on relaxing smooth muscle. Isosorbide dinitrite and nifedipine
have been used with some success in adolescents: however, side effects and transient
responses have limited the use of drugs. Balloon dilatation is sometimes used as first
line therapy in all ages. Dilatation offers temporary relief with improvement in 50%,
but long term results have been unsatisfactory in children with recurrence rates as
high as 25-30%. Repeated dilations creates increasing risk of esophageal perfora-
tion. Injection of botulin toxin into the LES has been tried in small series of adults
and older children with good results, but requires repeated injections. Larger series
are needed to confirm the initial success rate.
The basis of all surgical procedures is the cardiomyotomy described in 1914 by
Heller. The Heller myotomy provides excellent long-term relief of achalasia but is
complicated by gastroesophageal reflux in up to 6% of cases. Preoperative
esophagoscopy is done to ensure complete evacuation of retained food. Then, the
standard Heller operation is carried out through a left thoracotomy (7th intercostal
space. Upper transabdominal, laparoscopic, and thoracocopic approaches are all pos-
sible and increasingly reported. An antireflux procedure may be done with the Heller
myotomy.
To perform the myotomy, the distal esophagus is mobilized, encircled with a
tape, and freed at the esophageal hiatus, so that the gastroesophageal junction is
adequately visualized. The muscle fibers are separated longitudinally down to the
underlying mucosa. The myotomy incision extends from the middle/distal esophagus
Fig. 82.1. Massive esoph-

ageal dilation secondary to
achalasia, spasticity of the
lower esophagus that pre-
vents successful swallow-
ing.
82
down onto the stomach. A gastric flap (greater curvature) can be sutured over the
esophageal mucosa or some form of antireflux procedure may be used to cover the
myotomy. In an international survey of pediatric surgeons, an antireflux procedure
was performed in 75% of patients with a transabdominal myotomy but in only
17% with a transthoracic myotomy.
Manometry done after the operation confirms dramatically decreased sphincter
tone and improved esophageal motility in most patients. In children with achalasia,
esophageal myotomy has a 95% overall success rate with relief of symptoms and
weight gain.
Selected Readings
1. Raffensperger JG ed. Achalasia. In: Swenson’s Pediatric Surgery, 5th edition. New
York: Appleton & Lange 1990.
2. Heller E. Extramukose cardioplastik vein chronischen cardiospasmus mit dilitation
des oesophagus. Mitt Grenzgeb Med Chir 1913; 27:141-8.
3. Lelli JL, Drongowski RA, Coran AG. Efficacy of the transthoracic modified Heller
myotomy in children with achalasia—a 21-year experience. J Pediatr Surg 1997;
32(2):338-41.
CHAPTER 1
CHAPTER 83
Caustic Esophageal Injury and Perforation

Christina L. Dial and Daniel A. Bambini
Incidence
Caustic ingestion results in approximately 5,000-15,000 esophageal injuries per
year in the United States. Most caustic esophageal injuries (50-80%) occur in chil-
dren and 39% occur in children under age 5. Caustic ingestions in the young chil-
dren are considered “accidental,” but ingestions in adolescents and young adults are
often intentional (i.e., suicide attempt, “dare”). Boys are more frequently affected in
children less than 5 years; whereas, girls are the more likely victims during adolescence.
Etiology
The type and extent of injury resulting from ingestion of a caustic solution depends
upon:
1. the type of agent,
2. its physical state (i.e., liquid, solid),
3. concentration,
4. the amount ingested, and
5. the duration of contact with the esophageal or gastric mucosa.
The most common substances implicated in corrosive burns of the esophagus
are alkaline caustics, household bleaches, and acid or acid-like corrosives. Alkaline
caustics containing sodium hydroxide, potassium hydroxide, or sodium bicarbonate
are found in household lyes (granular or liquid), drain cleaners, and washing soda.
Dishwashing detergent and household cleaners frequently contain sodium metasili-
cate and ammonia. Acids (i.e., sulfuric, oxalic, hydrochloric) are found in batteries,
paint thinners, and other solvents. Hair dye solutions often contain potassium per-
manganate which also causes injury to the esophagus.
Alkalines, including dishwashing detergents, household bleaches, and laundry
detergents, are the most frequently ingested caustic agents. Most cause only limited
injury to the esophageal mucosa without extensive necrosis or subsequent sequelae.
Strong alkaline products (i.e., drain cleaners) containing lye are odorless, tasteless,
and can lodge in the oropharynx or upper esophagus causing severe damage, necro-
sis, or perforation. If the alkaline solution is a viscous liquid, the oropharynx may be
spared but the damage may extend from the mid-esophagus distally to the
esophagogastric junction.
Strong acids frequently have a bitter taste, emit a strong odor, and burn on con-
tact, which often results in rapid expulsion after ingestion. When swallowed, acids
usually causes significant damage to the stomach and variable mucosal damage to
the esophagus. The duodenum and proximal small bowel are relatively well-pro-
tected by the pylorus.
Classification and Pathophysiology
Injury to the mucosal surfaces occurs within seconds of the insult. Caustic inju-
ries to the esophagus are classified similarly to thermal burn injuries of the skin
(Table 83.1). The nature of the injury differs between acid and alkali ingestion.
Alkali ingestion causes liquefaction necrosis with destruction of the epithelium, sub-
mucosa and sometimes the muscularis. Even though an eschar forms and neutral-
ization occurs, continued destruction of the deeper layers is still possible following
alkali ingestion. Following acid ingestion injuries, coagulation necrosis occurs and a
hard eschar is formed. This may limit the damage to the mucosa.
Caustic ingestion injuries evolve through three phases:
1. the acute phase,
2. the subacute phase, and
3. the cicatrization phase.
83 The acute phase (0-4 days) is characterized by the presence of inflammation,
edema, thrombosis, eschar formation, and necrosis. The subacute phase or repara-
tive phase occurs between 5 and 14 days following the injury. During this phase
necrotic tissue is sloughed, fibroblasts are deposited, and neorevascularization begins.
The esophageal wall is weakest during the subacute phase and prone to perforation.
The cicatrization phase occurs between 3 and 6 weeks following injury. Fibrous
tissue replaces the submucosa and muscularis forming dense scar that results in stric-
tures, obliteration, or shortening of the esophagus.
The clinical presentation of children following caustic ingestion is highly vari-
able. Signs and symptoms include drooling, burning pain in the mouth and lips,
odonyphagia, dysphagia, hoarseness, stridor, and aphasia. Esophageal perforation is
often associated with symptoms of severe retrosternal, back, or upper abdominal
pain. Fever, tachycardia, and hypotension are signs of severe esophageal injury and
often indicate massive ingestions. Physical signs of caustic ingestion include ulcer-
ation or discoloration of the oropharyngeal mucosa, cervical crepitance, hematemesis,
and peritonitis. A lack of external physical findings does not exclude the possibility
of caustic ingestion.
Treatment
The first priority of initial management is always airway protection and control.
Vascular access, fluid resuscitation, and identification of the caustic agent are also
important aspects of early treatment. Nasogastric intubation is avoided and gastric
lavage is contraindicated to prevent propagation of injury beyond the pylorus or
iatrogenic perforation. Plain chest and abdominal x-ray examinations are indicated
to evaluate for mediastinal or free intraperitoneal air indicating perforation.
Once respiratory and hemodynamic stability are achieved, the severity of the
injury is determined. Fiberoptic endoscopic examination of the entire airway and
esophagus is the preferred method to determine the extent of injury. Esophagoscopy
Caustic Esophageal Injury and Perforation 385
is performed within 24-48 hours of ingestion to the upper limit of any full-thick-
ness injury encountered. Endoscopic classification of caustic esophageal injuries is
given in Table 83.1.
Once the grade of injury is determined (Table 83.1), the suggested management
is as follows:
Grade I
Children with grade I injuries are admitted for observation and intravenous fluid
administration. A clear liquid diet is started at 36-48 hours and advanced as toler-
ated to a general diet. A contrast esophagram is performed 2-3 weeks following
injury in any child with residual symptoms or dysphagia.
Grade II/III
Children with grade II or III injuries are denied oral intake for several days (some-
times weeks). Parenteral nutrition, either peripheral or central, is mandatory to pro-
vide adequate nutritional support. Intensive care unit observation is necessary to
monitor for signs of worsening or more complicated injury (i.e., esophageal perfora-
tion, gastric perforation, tracheoesophageal fistula, mediastinitis). Severe grade III 83
injuries with esophageal perforation mandate surgical intervention. Oral intake is
withheld until patients can tolerate swallowing their saliva. A liquid diet is started
initially and advanced to a general diet as tolerated. A barium esophagram is per-
formed at 2-4 weeks following injury to identify early stricture formation.
The use of steroids (i.e., prednisolone, dexamethasone) and antibiotics in initial
management of caustic esophageal injuries is controversial. Steroids inhibit the
inflammatory process and may reduce granulation and stricture formation. Unfor-
tunately, steroids are also immunosuppressive and may contribute to infectious com-
plications and morbidity. Antibiotics may reduce bacterial overgrowth that occurs
in Grade II/III injuries.
Other forms of therapy include esophageal stenting, dilatation, and early enteral
feeding by jejunostomy, gastrostomy or nasogastric tube feedings. A nasogastric tube
may function to keep the esophageal lumen patent. H2 blockers, proton pump
inhibitors and antacids are frequently used, but their benefits have not been proven.
Immediate surgical intervention is indicated in those patients with uncontrol-
lable hemorrhage or perforation (i.e., mediastinal air, intraperitoneal air, or perito-
nitis). Esophageal resection can be performed via either thoracotomy or laporotomy
(i.e., transhiatal). After resection, gastrostomy or jeujunostomy tube is placed and
the proximal esophagus is diverted as a cervical esophagostomy. Reconstruction of
the alimentary tract is delayed for at least 2-3 months or until all acute problems are
resolved. The mortality associated with esophageal perforation following caustic
ingestion is 20-25%.
Outcomes
The most common complication of caustic ingestion is stricture formation.
Although rare in Grade I injuries, strictures occur in 20-30% of Grade II injuries
and 90-95% of Grade III injuries. Although several treatments have been employed
to prevent stricture formation (i.e., steroids, bougionage, esophageal stents, etc.),
none has been highly successful. Once a stricture has developed, dilatation becomes
Table 83.1. Endoscopic classification of esophageal injuries

Classification Depth of Mucosal Involvement
Grade I Superficial mucosal hyperemia, edema, and sloughing
Grade II Transmural involvement with exudates, ulceration, and

muscle involvement and pseudomembrane formation
Grade III Eschcar formation, obliteration of lumen, and deep

ulceration
Erosion through the esophagus into the periesophageal
tissue, mediastinum, pleural or peritoneal cavities
necessary and is usually started at 6-8 weeks after injury. Weekly dilatation is contin-
ued until the stricture softens and a bouginage dilator 2-3 times the diameter of the
esophagus can be easily passed i.e., 32-36 FR in toddlers, 38-44 FR in children 5-10
83 years old and 46-54 FR in children more than 10 years old. The risk of esophageal
perforation with dilatation is relatively low but this is the most common complica-
tion. When the interval between dilatations fails to increase or actually decreases,
long-term failure is probable. These children are candidates for surgical reconstruc-
tion such as colonic or jejunal substitution, reversed gastric tubes, or gastric pull-up
procedures.
Caustic ingestion is associated with an increased risk of esophageal carcinoma.
The middle portion of the esophagus is most often affected, and the tumors are
usually squamous cell in origin. The incidence of esophageal carcinoma in patients
after caustic ingestion is estimated to be 500-1,000 times greater than the incidence
in the general population. The latency period between initial injury and develop-
ment of esophageal carcinoma varies from 10-50 years. Lifelong follow-up and screen-
ing endoscopy is recommended.
Selected Readings
1. Andreoni B, Farina ML, Biffi R et al. Esophageal perforation and caustic injury:
Emergency management of caustic ingestion. Dis Esoph 1997; 10:95-100.
2. Lovejoy FH Jr. Corrosive injury of the esophagus in children. NEJM 1990;
323:668-9.
3. Christensen HBT. Epidemiology and prevention of caustic ingestion. Acta Pediatr
1994; 83:212.
4. Raffensperger JG. Caustic esophageal burns. In: Raffensperger JG ed. Swensen’s
Pediatric Surgery. Appleton-Lange 1991; 827-832.
5. Anderson KD, Rouse TM, Randolph JG. A controlled trial of corticosteroids in
children with corrosive injury of the esophagus. NEJM 1990; 323:637.
6. Gaudreault P et al. Predictability of esophageal injury from signs and symptoms: A
study of caustic ingestion in 378 Children. Pediatrics 1983; 71:767-770.
Section XIII: Gastrointestinal Diseases
of the Older Child
CHAPTER 84
Appendicitis
Steve Szczerba
Incidence
Acute appendicitis is the most common surgical emergency in children and ado-
lescents. Overall there are about 250,000 cases of appendicitis in the United States
annually, and the majority occur in children 6-10 years of age. Appendicitis affects
males more often than females (M:F ratio 3:2) and the lifetime risk for each group is
8.6% and 6.7%, respectively. Caucasians are affected more commonly than other
racial groups. Acute appendicitis occurs more frequently during the summer months.
Etiology
Appendicitis is caused by obstruction of the appendiceal lumen that leads to
vascular congestion, ischemic necrosis, and subsequent infection. The most com-
mon cause of the obstruction is a fecalith or inspissated fecal matter. Fecaliths are
identifiable in about 20% of children with appendicitis. Other causes of appen-
diceal obstruction include:
1. lymphoid follicle hyperplasia,
2. carcinoid or other tumors,
3. foreign bodies (i.e., pins, seeds, etc.), and
4. rarely parasites.
Appendicitis can affect any age group. Although exceptionally rare in neonates
and infants, acute appendicitis does occasionally present at that young age, and
diagnosis may be extremely difficult and delayed. In slightly older children, the
presenting clinical signs and symptoms are quite variable in pattern and order of
appearance. Pain is usually the first symptom. It frequently begins as a dull, vague
periumbilical pain but with time may localize to the right lower abdomen. Patients
typically report a gradual increase in pain intensity as the disease process progresses.
Anatomical variability in the location of the appendix (i.e., retrocecal, pelvic) is
common and can alter the pain symptoms accordingly. In children with a retrocecal
or pelvic appendix, pain may start in the right lower quadrant without any early
periumbilical pain. Flank pain, back pain, and referred testicular pain are also com-
mon symptoms in children with retrocecal or pelvic appendicitis. If the inflamed
appendix is in proximity to the ureter or bladder, symptoms may include urinary
frequency, pain with micturition, or discomfort from urinary retention and bladder
distension.
Appendicitis 389
Anorexia, nausea and emesis usually develop within a few hours of pain onset.
Emesis is usually mild. Diarrhea may occur secondary to inflammation and irrita-
tion of the terminal ileum or cecum. Severe gastrointestinal (GI) symptoms that
develop prior to the onset of pain usually indicate a diagnosis other than acute
appendicitis. However, mild GI complaints such as indigestion or change in bowel
habits occasionally precede pain symptoms in children with appendicitis.
Typically, patients with uncomplicated appendicitis have low-grade fever. Tem-
peratures above 38.6˚C suggest perforation. Children with appendicitis avoid move-
ment and tend to lie still in bed. Frequently, these children lie quietly on their sides
or with their knees flexed. Children with appendicitis sometimes walk with a limp
favoring the right leg.
The signs of appendicitis elicited by physical exam are often very subtle. Hyper-
esthesia of the skin can be elicited by gently touching the skin of the patient with a
stethoscope. Bowel sounds, although an unreliable predictor, are often decreased or
absent.
The abdominal tenderness associated with acute appendicitis varies with the
time-course of the disease and the anatomic location of the appendix. During the
initial stages, tenderness can be mild and only vaguely localized in the lower abdo-
men. When the parietal peritoneum becomes irritated over the site of the appendix,
localized tenderness can be elicited. “McBurney’s point”, an area one-third the dis- 84
tance from the anterior superior iliac spine to the umbilicus, is the most common
site of maximal tenderness in appendicitis that has progressed beyond 12-24 hours.
Retrocecal appendicitis may cause tenderness midway between the 12th rib and the
posterior superior iliac spine. Pelvic appendicitis produces rectal tenderness. In chil-
dren with acute appendicitis and malrotation, tenderness occurs well away from the
usual location in the right lower quadrant. If the disease process has progressed
beyond 24-36 hours, perforation may cause an abrupt but temporary decrease in
pain symptoms and tenderness as the intraluminal pressure of the distended, inflamed
appendix is released.
Peritonitis is manifest as muscle wall rigidity, guarding, and rebound tenderness.
“Rovsings’ sign” (palpation of left lower quadrant producing right lower quadrant
pain) is a reliable indicator of acute appendicitis in children. The “psoas sign” (retro-
cecal appendicitis) and the “obturator sign” (pelvic appendicitis) are difficult to elicit
in smaller children. Rectal exam may reveal a palpable, tender extrinsic mass or
abscess.
Acute appendicitis can mimic just about any intra-abdominal process. The dif-
ferential of acute appendicitis is extensive and includes gastroenteritis, Crohn’s dis-
ease, mesenteric adenitis (i.e., Campylobacter, viruses, Yersinia, etc.), pancreatitis,
peptic ulcer disease, cholelithiasis, cholecystitis, Meckel’s diverticulitis, constipation,
intussusception, and many other conditions. Systemic disorders that are in the dif-
ferential of acute abdominal pain and appendicitis include porphyria, sickle cell
crisis, Henoch-Schonlein purpura, hemolytic uremic syndrome, diabetic ketoacido-
sis, measles, Lupus erythematosus, and parasitic infections. In females, ectopic preg-
nancy, ovarian torsion, ovarian cysts, and pelvic inflammatory disease must also be
considered. Urinary tract disease (i.e., renal stones, pyelonephritis, cystitis) can also
mimic acute appendicitis. Pneumonia, particularly of the right lower lobe, is a fre-
quent nonabdominal source of lower abdominal pain in children that must be
considered. In children less than 3 years old, gastroenteritis and ileocolic intussus-
ception are the two most common conditions included in the differential diagnosis.
Acute appendicitis is associated with several other conditions. Patients with
enterocolitis (Yersinia, Salmonella, Shigella, etc.) or parasitic infections (Entamoeba,
Strongyloides, Enterobius, Schistosoma, Ascaris) can develop appendicitis second-
ary to both local or generalized lymphoid hyperplasia and obstruction of the appen-
diceal lumen. Viral infections with measles, chicken pox, or cytomegalovirus (CMV)
have been linked with appendicitis.
Children with cystic fibrosis have a higher incidence of acute appendicitis due to
abnormal mucous that becomes inspissated and obstructs the lumen of the appen-
dix. Hirschsprung’s disease should be considered in any neonate that presents with
appendicitis.
Diagnosis
The principal means of diagnosis is history and physical examination. Serial
examinations by the same examiner are perhaps the most accurate diagnostic tool.
Leukocyte count (WBC) above 10,000 is observed in greater than 90% of children
with acute appendicitis. A left shift is usual but not an absolute finding. Urinalysis is
helpful to differentiate pyelonephritis or renal calculus from appendicitis, however,
84 mild hematuria and pyuria can be seen when the inflamed appendix is near the
ureter.
Plain film radiography has limited value in children suspected of having appen-
dicitis. A radiopaque fecalith can be seen in only 5% of patients with acute appendi-
citis. The more subtle plain film findings are:
1. sentinel loop in the right lower quadrant,
2. lumbar scoliosis concave to the right lower quadrant,
3. mass effect from a pelvic abscess,
4. loss of the psoas shadow, and
5. loss of the preperitoneal fat stripe.
A chest radiograph is obtained to evaluate children with history “atypical” for
appendicitis and suspected of having a pneumonia.
Barium enema is not usually performed in children suspected of having acute
appendicitis but is frequently chosen to evaluate for intussusception. Barium enema
signs of appendicitis include:
1. incomplete filling of the appendix,
2. wall irregularities of terminal ileum or cecum, and
3. mass effect on the terminal ileum or cecum.
Ultrasonography has about 85% sensitivity and greater than 90% specificity in
the diagnosis of acute appendicitis. The main sonographic criterion for diagnosis is
demonstration of a noncompressible appendix larger than 7 mm in diameter. Iden-
tification of an appendicolith or periappendiceal fluid is also helpful. As with other
radiographic studies, the value of ultrasound may be to exclude other diagnoses,
particularly in female patients. Computed tomography (CT) is a reliable test for
acute appendicitis but is reserved for situations when the diagnosis is unclear. Reported
sensitivity and specificity are approximately 95-98%. In severely obese patients and
patients presenting late and suspected of having abscesses, CT may be the diagnos-
tic test of choice.
Appendicitis 391
Appendicitis begins with obstruction of the appendiceal lumen. The obstructed
appendix continues to secrete mucus causing the appendix to distend. Distension
activates visceral nerve pain fibers that cause pain symptoms referred to the perium-
bilical area (T-10 dermatome). As the intraluminal pressure increases, lymphatic
drainage is impaired causing further edema and intramural pressure within the
appendix. As the pressure continues to rise, venous outflow is compromised which
leads to decreased arterial perfusion and ischemic necrosis. Tissue infarction, gan-
grene with bacterial infection, and perforation follow if the condition remains
untreated. Pain localizes to the right lower quadrant when surrounding inflamma-
tion irritates the parietal peritoneum activating somatic pain fibers. After perfora-
tion, localized abscess or diffuse peritonitis can occur. Diffuse peritonitis is common
in young children and infants whose omentum is proportionately smaller and less
able to contain an advancing suppurative process.
Treatment
For most patients, immediate surgical intervention is not considered mandatory.
The patient with appendicitis is resuscitated with intravenous fluids, started on broad-
spectrum IV antibiotics, and kept NPO. Although spontaneous resolution can occur,
appendectomy is still the treatment of choice for all patients suspected of having 84
acute appendicitis. Complication rates and perforation rates are the same for patients
undergoing surgery within 6 hours of admission as those undergoing surgery between
6-16 hours after admission. All patients with appendicitis and generalized peritoni-
tis require expedient resuscitation and urgent exploration.
For children presenting late (i.e., several days or weeks) with well-localized peri-
appendiceal abscess or phlegmon, prolonged IV antibiotic therapy (2-3 weeks) and
CT-guided percutaneous abscess drainage is often a better therapeutic option. Inter-
val appendectomy is usually performed 4-6 weeks later but may not be totally
necessary.
At surgery, the abdomen is explored via a transverse or oblique right lower quad-
rant incision. The peritoneal cavity is entered and the appendix is delivered into the
wound if possible. The appendix is assessed for signs of inflammation, gangrene,
and/or perforation. Cultures are frequently obtained but are of questionable value.
Appendectomy is all that is needed in cases of acute appendicitis whether perfor-
ated, nonperforated, or gangrenous. In rare cases, when the cecal wall is involved in
a gangrenous, inflammatory process, limited ileocecal resection with primary anas-
tomosis may be necessary. For gross contamination, the abdomen and pelvis are
irrigated with saline solution. The wound closure is standard and in children the
skin incision is almost always closed regardless of the pathologic findings.
If a normal appendix is found at laparotomy (5-15% of cases), the abdomen is
systematically inspected for evidence of inflammatory bowel disease, a Meckel’s
diverticulum, mesenteric adenitis, peptic ulcer disease, and other pathology. In
females, the ovaries should be identified and inspected. If Crohn’s disease is encoun-
tered, the appendix should be removed unless the disease process grossly involves
the base of the appendix.
Postoperative care includes continued broad-spectrum antibiotic therapy. Ampi-

cillin, gentamycin, and clindamycin or metronidazole, are the traditional “gold stan-
dard” antibiotics for treatment of children with acute appendicitis. Antibiotic therapy
must provide activity against the common pathogens associated with appendicitis:
E. coli, Bacteroides, Enterococcus, and Klebsiella. Alternative antibiotics, such as
ampicillin/sulbactam, and others, can be considered and are probably equally effec-
tive. A switch to oral antibiotics is sometimes made when the patient is afebrile and
tolerating a diet. For perforated or gangrenous appendicitis, a 5-day course is the
usual recommended therapy but many surgeons stop postoperative antibiotics when
the recovering patient is afebrile with a normal WBC (including differential). For
children with nonperforated appendicitis, postoperative antibiotics are usually con-
tinued for only 24 hours.
Outcomes
Complication rates after appendectomy vary with the severity of the appendici-
tis. Wound infection, overall observed in 5-10% of patients, is the most common
complication. Abscess formation and bowel obstruction occur in less than 5% and
usually affect those with perforated appendicitis. Surgical morbidity from perfora-
tion is approximately 10% and includes wound infection, wound dehiscence, abscess,
84 and bowel obstruction. Other complications such as tubal infertility, abscess forma-
tion secondary to retained fecalith or partial appendectomy, and suppurative pyle-
phlebitis are also uncommonly reported after complicated cases of acute appendicitis.
Selected Readings
1. Anderson R et al. Indications for operation is suspected appendicitis and incidence
of perforation. Br Med J 1994; 308:107.
2. Bennion RS, Thomson JE. Early appendectomy for perforated appendicitis in chil-
dren should not be abandoned. Surg Gynecol Obstet 1987; 165:95.
3. Brender JD et al. Childhood appendicitis: factors associated with perforation.
Pediatrics 1985; 76:301.
4. Sarfati MR et al. Impact of adjunctive testing on the diagnosis and clinical course
of patients with acute appendicitis. Am J Surg 1993; 166:660.
5. Stringel G. Appendicitis in children: A systematic approach for a low incidence of
complications. Am J Surg 1987; 154:631.
6. Anderson KD, Parry RL. Appendicitis. In: O’Neill Jr. JA et al, eds. Pediatric Sur-
gery, 5th Edition. St. Louis: Mosby-Year Book 1998; 1369-1380.
7. Rao PM et al. Effect of computerized tomography of the appendix on the treat-
ment of patients and use of hospital resources. N Engl J Med 1998; 338:141.
CHAPTER 1
CHAPTER 85
Adhesive Intestinal Obstruction

Todd R. Vogel
Incidence
The incidence of postoperative adhesive obstruction after laparotomy is about
2%. The procedures which have highest risk for adhesive McBurney’s point in pedi-
atric patients are:
1. subtotal colectomy,
2. resection of symptomatic Meckel’s diverticulum,
3. Ladd’s procedure, and
4. nephrectomy.
Etiology
The causes of postoperative McBurney’s point include adhesions, intussuscep-
tion, hernia, and tumor. Adhesions are fibrous bands of tissue that form between
loops of bowel or between the bowel and the abdominal wall after intraabdominal
inflammation. Obstruction occurs when the bowel is “caught” within one of these
fibrous bands in a kinked or twisted position, twists around an adhesive band, or
herniates between a band and another fixed structure within the abdomen.
Children with a mechanical obstruction present with cramping abdominal pain,
distension, and vomiting. For prolonged McBurney’s points the vomitus becomes
bilious or even feculent. Inspection of the abdomen may reveal obvious dilated loops
of bowel and distension. If observed early in the clinical course, the patient’s vital
signs are within the normal range and the abdomen is not tender. In contrast, chil-
dren with compromised bowel or a prolonged obstruction, present with abdominal
pain, vomiting, fever, tachycardia, decreased blood pressure, abdominal tenderness,
and leukocytosis.
Diagnosis
The differential diagnosis is ileus versus mechanical obstruction. Nonsurgical,
inflammatory and metabolic conditions that may result in ileus must be considered.
Blood is drawn and sent for Hbg, WBC and differential, amylase (pancreatitis),
liver function tests (hepatitis) and bilirubin (biliary tract disease). Urinalysis (uri-
nary tract infection, nephritis, stones), blood cultures (systemic infection), and stool
cultures (colitis, rotavirus) may also be indicated. Upright posteroanterior and lat-
eral chest xrays are obtained to exclude pneumonia or the presence of free intraperi-
toneal air. Flat and upright abdominal films are also obtained. In a child with a
complete bowel obstruction, abdominal films will show dilated loops of small bowel
with multiple air fluid levels and little or no air in the rectum and/or distal to the
obstructing lesion. Ultrasound is occasionally useful to rule out a postoperative
intussusception.
Treatment
Nonoperative management includes resuscitation with isotonic saline solutions,
nasogastric decompression, correction of electrolyte abnormalities, IV antibiotics,
and serial examinations. Within 24 hours, children with ileus and simple mechani-
cal obstruction will improve as indicated by a return of bowel function, a normaliza-
tion of vital signs and a normal WBC. Indications for operation include obstipation
for 24 hours, continued abdominal pain with fever and tachycardia, decreased blood
pressure, increasing abdominal tenderness, and leukocytosis despite adequate resus-
citation and medical treatment.
The abdomen is opened through a previous incision, if present, and midline, if
not. The cecum is identified and the collapsed ileum is followed proximally until
dilated bowel and the point of obstruction is identified. The offending adhesive
bands are disrupted and the abdomen is closed. Laparoscopic lysis of adhesions is
another option and may allow a shorter postoperative recovery and hospital stay.
Postoperatively, nasogastric decompression and intravenous fluids are continued
until return of bowel function and the volume of gastric aspirate decreases.
85 Selected Readings
1. Akgur FM et al. Adhesive small bowel obstruction in children: The place and
predictors of success for conservative treatment. J Pediatric Surg 1991; 26(1):37-41.
2. Raffensperger J ed. Swenson’s Pediatric Surgery. Norwalk: Appleton & Lange 1990;
855-857.
3. Filston HC. Other causes of McBurney’s point. In: O’Neill, Jr. JA et al, eds. Pedi-
atric Surgery, 5th Edition. St. Louis: Mosby 1998; 1215-1218.
CHAPTER 1
CHAPTER 86
Gallbladder Disease in Childhood

Fawn C. Lewis
Incidence
Gallbladder disease is uncommon in infants and children and is generally classi-
fied as congenital or acquired. Considering all children with gallbladder problems,
congenital anomalies are identifiable in 14%. Thirty percent have neonatal cholestasis
syndromes and 40% have calculous disease. The few remaining childhood problems
are rare and include biliary obstruction from fibrosing pancreatitis, sclerosing cho-
langitis, or other lesions such as metastatic tumor. The most common congenital
anomalies of the gallbladder are:
1. agenesis,
2. duplication,
3. ectopic location,
4. bilobate or multiseptated gallbladder, and
5. stenosis of the cystic duct.
Primary gallbladder tumors are usually benign adenomas but may have malig-
nant potential. Gallbladder carcinoma is extremely rare in children.
The overall prevalence of cholelithiasis in neonates and young children is
approximately 0.15-0.22%. This percentage increases in older children and teenag-
ers. The genders are affected equally until adolescence, when cholelithiasis is more
common in females. Native Americans, especially Pima Indians, have a much greater
risk of cholelithiasis-with a prevalence approaching 100% by age 40 in the females
of some tribes. Gallstones are more common in Caucasian children than children of
African-American descent. Children with hemoglobinopathies or hemolytic diseases
are at a great risk for cholelithiasis. Gallstones are identifiable in 12-40% of teenage
children with sickle cell disease. Biliary sludge (without stones) is detectable in an-
other 10-16%. Although biliary sludge progresses to cholelithiasis in 66-100% of
children with sickle cell disease, resolution may occur in up to 20%. Approximately
14% of children with sickle cell disease undergo cholecystectomy during childhood.
Etiology
Congenital Anomalies
There are no specific causative factors associated with congenital anomalies of
the gallbladder. Annular pancreas has been rarely associated with agenesis of the
gallbladder.
Polyposis
Metachromatic leukodystrophy (ML) has been associated with gallbladder poly-
posis in children (3 cases reported in the literature). Polyposis may precede the diag-
nosis of ML by 6 months.
Lithogenesis
There are two general types of gallstones: cholesterol and pigment stones. For-
mation of cholesterol gallstones depends upon the relative concentrations of choles-
terol, lecithin, and bile salts. Three factors are said to be necessary for stone formation:
1. increased cholesterol saturation of the bile,
2. bile stasis, and
3. the presence of nucleating factors as a nidus of stone formation.
Black pigment stones are usually formed in a setting of hemolysis, ileal resection,
or total parenteral nutrition (TPN). Pigment stones are more prevalent in Asians.
Acalculous Cholecystitis
Several factors may predispose patients to develop acalculous cholecystitis
including:
1. dehydration,
2. adynamic ileus,
3. gallbladder stasis,
4. total parenteral nutrition,
5. hemolysis, and
6. massive transfusions.
86 These conditions are frequently encountered in children with severe critical ill-
ness (i.e., multisystem trauma, burns, pneumonia, sepsis, severe infection).
Several factors associated with gallbladder disease in children are listed in
Table 86.1.
The typical presentation of any gallbladder malady involves right upper abdomi-
nal pain, nausea, and emesis. If infection is present, fever, leukocytosis, or Murphy’s
sign (an inspiratory pause due to patient discomfort when the examiner holds mild
pressure in the right upper quadrant) may be present. If obstruction to bile flow
occurs, jaundice or acholic stools are seen. It is critically important to identify the
etiology of the jaundice in order to provide proper treatment. If stones are present,
surgery will correct the problem. However, more serious causes of jaundice (i.e.,
biliary atresia, choledochal cyst) must be excluded. Neonates and younger infants
frequently have an associated clinical condition (i.e., prolonged TPN, prematurity,
cystic fibrosis (CF), prolonged fasting) that may contribute to cholestasis and jaundice.
Diagnosis
In addition to physical exam, laboratory evaluation of the patient’s leukocyte
count, electrolytes, serum glucose, liver function tests (AST, ALT, alkaline phos-
phatase, bilirubin, and albumin), and amylase help formulate the differential diag-
nosis for any patient with abdominal pain and emesis. If the child is jaundiced,
serum albumin level, prothrombin time (PT), and partial thromboplastin time (PTT)
Gallbladder Disease in Childhood 397
Table 86.1. Factors associated with cholelithiasis or cholecystitis

Drugs/Treatments Association
Exchange Transfusion Stones

Furosemide Stones, Sludge
Phototherapy Stones, Sludge
Morphine Stasis
Ceftriaxone Pseudolithiasis
Chemotherapy for Wilms’ tumor, Stones
Neuroblastoma, Hodgkin’s Disease,
Non-Hodgkin’s Lymphoma
Diseases/Conditions Association
Obesity Stones
Sickle Cell Disease Stones, Sludge
Polycythemia Stones, Sludge
Hereditary Spherocytosis Stones, Sludge
Kawasaki’s Disease Hydrops
Byler’s Disease (progressive familial Hydrops
intrahepatic cholestasis)
Hepatitis A Edema,
Wall Thickening
Epstein-Barr Virus Hydrops, Sludge
Ileal Resection Stones
Short-Gut Syndrome Stones
Cystic Fibrosis Stones
Infection Sludge then Stones 86
Dehydration Stones, Sludge
Leptospirosis Stones, Sludge
are checked to assess nutritional status, hepatic synthetic function, and the possible
surgical risk of hemorrhage.
Transabdominal ultrasonography is both sensitive and specific to identify dila-
tion of the intra- or extra-hepatic biliary tree, gallbladder distension, and occasion-
ally the pancreatic duct. Gallbladder wall thickness or edema, pericholecystic fluid,
cholelithiasis, biliary sludge, or polyps are easily identified with this rapid, noninvasive
test.
As in adults, evidence of dilation of the common bile duct or intrahepatic biliary
system necessitates further evaluation of the biliary tree. Depending on the size of
the child, endoscopic retrograde cholangiopancreatography (ERCP) or percutane-
ous transhepatic cholangiography (PTCA) are used to study the biliary system.
If there is no evidence of cholelithiasis or biliary dilation, nuclear studies are
infrequently used to assess the function of the biliary tract. Cholescintigraphy is a
nuclear medicine scan in which a technetium-99 labeled isotope is injected intrave-
nously and concentrated and excreted in the bile. Visualization of the gallbladder
usually rules out cholecystitis; excretion into the duodenum rules out complete com-
mon bile duct obstruction. Cholescintigraphy may yield false positive results in:
1. fasting patients,
2. patients on TPN, and
3. patients with acalculous cholecystitis.
Gallbladder contractility and emptying can be assessed by cholescintigraphy with
simultaneous injection of cholecystokinin (CCK). A gallbladder ejection fraction of
less than 35% has been reported to be associated with dyskinesia or stasis.
Delay in diagnosis is not uncommon, particularly in patients with cystic fibrosis
(CF), and average delays of 8 months are common. Consequently, a high level of
suspicion for gallbladder disease is necessary when CF patients present with abdominal
pain.
In the neonate, acalculous cholecystitis with a gangrenous gallbladder can mimic
necrotizing enterocolitis. If initial medical therapy is unsuccessful, this diagnosis
should be considered and exploration may be necessary.
Treatment
Treatment of incidentally found gallstones or sludge is expectant. Follow-up
ultrasound to evaluate for disease progression is indicated if symptoms occur. In the
case of children with sickle-cell disease, biliary sludge is followed regularly with
ultrasound. Identification of cholelithiasis should lead to prophylactic elective chole-
cystectomy especially if the child has abdominal complaints that suggest cholecysti-
tis or abdominal crises that can be confused with recurrent bouts of gallbladder
inflammation. Elective cholecystectomy is performed in children with sickle cell
disease and symptomatic biliary sludge. Preoperative preparation of the sickle-cell
patient consists of suppressive blood transfusions (10 ml/kg 2-3 times over 2-3 weeks)
86 to decrease the percentage of circulating Hb-S to less than 30% and to suppress
bone marrow production of Hb-S. During surgery, care is taken to avoid hypother-
mia, hypovolemia, or acidosis since these problems can initiate a sickle crisis. In an
emergent setting, preoperative transfusion to a hemoglobin of 12 and very careful
management to minimize hypovolemia, hypothermia, and acidosis are critically
important.
A child undergoing splenectomy due to a hemolytic disease, should also undergo
prophylactic cholecystectomy if there is cholelithiasis by ultrasound.
The preferred treatment of uncomplicated biliary colic today is laparoscopic
cholecystectomy. There is no standard age or size limit for this procedure, but the
experience of the surgeon directs appropriate choice of “open” or “minimally inva-
sive” technique (see Section XVI). The absolute and relative contraindications to
laparoscopic cholecystectomy in children are similar to those in adults. Absolute
contraindications include inability to safely perform the dissection or clearly iden-
tify the anatomy. Relative contraindications include multiple previous surgeries, bleed-
ing disorders, and previous right upper quadrant surgery.
Outcomes
Most children undergoing laparoscopic cholecystectomy are discharged within
24 hours; overall morbidity is 1-2%, and mortality is extremely rare. Sickle cell
patients are typically hospitalized longer, especially for pain management. Morbid-
ity from laparoscopic cholecystectomy in children with sickle cell disease is reported
to be around 6%; pain crises and pulmonary infections are the leading postoperative
Gallbladder Disease in Childhood 399
problems. The mortality rate associated with cholecystectomy in children with sickle
cell disease is about 2-4%.
Selected Readings
1. Frexes M, Neblett WW 3rd, Holcomb GW Jr. Spectrum of Biliary Disease in
Childhood. South Med J 1986; 79(11):1342-9.
2. Emond JC, Whitington PF. Selective surgical management of progressive familial
intrahepatic cholestasis (Byler’s disease). J Pediatr Surg 1995; 30(12):1635-41.
3. Holcomb GW 3rd. Laparoscopic cholecystectomy. Semin Pediatr Surg 1993;
2(3):159-67.
4. Ware RE, Filston HC. Surgical management of children with hemoglobinopa-
thies. Surg Clin North Am 1992; 72(6):1223-36.
5. Moir CR, Donohue JH, van Heerden JA. Laparoscopic cholecystectomy in chil-
dren: Initial experience and recommendations. J Pediatr Surg 1992; 27(8):1066-70.
6. Newman KD, Marmon LM, Attorri R et al. Laparoscopic cholecystectomy in
pediatric patients. J Pediatr Surg 1991; 26(10):1145-7.
7. Rowe MI, O’Neill JA Jr., Grosfeld JL et al, eds. Essentials of Pediatric Surgery. St.
Louis: Mosby-Year Book Inc. 1995; 656-62.
86
CHAPTER 87
Superior Mesenteric Artery (SMA)

Syndrome
Todd R. Vogel
Incidence
SMA syndrome is a rare cause of small bowel obstruction in the pediatric popu-
lation.
Etiology
The duodenum passes between the aorta and the SMA at the level of the third
thoracic vertebra. The SMA arises at the level of the first lumbar vertebra at an angle
of 45-60˚. Compromise of this angle secondary to weight loss and resorption of
retroperitoneal fat may lead to extrinsic compression of the duodenum. Symptoms
present when the third portion of the duodenum is intermittently compressed by
the overlying superior mesenteric artery (usually at an angle of 15˚ or less). Ana-
tomic features include a short aortomesenteric distance together with sagittal paral-
lelism between the aorta and the SMA. Predisposing factors include a rapid weight
loss, prolonged supine positioning, and use of spinal orthosis. Spinal orthosis or
body casts are associated with hyperextension of the spine allowing for SMA com-
pression. Familial clustering of SMA syndrome and genetic predisposition have been
suggested.
Most patients present with a history of weight loss from dieting or illness, a
history of minimal weight loss but with rapid vertical growth, or a history of immo-
bilization. Nausea, intermittent bilious vomiting are the principal symptoms. On
examination, the patients are usually thin and the abdominal examination is unre-
markable.
Diagnosis
The differential diagnosis includes ileus, malrotation, and anorexia nervosa.
Abdominal films are usually unrevealing. Upper GI study demonstrates a dilated
proximal duodenum with minimal or no passage of contrast past the vertebral col-
umn. Turning the patient prone or on the left side results in passage of contrast into
a decompressed bowel. The gold standard for diagnosis is a lateral aortogram with
concomitant ingestion of a barium meal.
Superior Mesenteric Artery (SMA) Syndrome 401
Treatment
Conservative treatment with an oral diet of frequent, small volume, high calorie
liquids results in weight gain and complete recovery in most patients. Positioning
the patient in the left lateral position may allow for passage of intestinal contents.
Alternatively, continuous nasojejunal feedings may be used until the retroperitoneal
fat has been restored. If enteral feedings are unsuccessful, hyperalimentation is indi-
cated. If weight gain is not established in 5-7 days, surgical intervention should be
considered.
Surgical options include:
1. mobilization of the duodenum from underneath the SMA and placing it
to the right of the spine or
2. duodenojejunostomy.
Selected Readings
1. Burrington JD. Superior Mesenteric Artery Syndrome. In Raffensperger, JG ed.
867-870.
2. Burrington JD. Superior Mesenteric Artery Syndrome in Children. Am J Dis Child
1976; 130:1367.
3. Ylinen P et al. Superior Mesenteric Artery Syndrome. A follow-up study of 16
operated patients. J Clin Gastroenterol 1989; 11(4):386-391.
87
CHAPTER 88
Inflammatory Bowel Disease

Christopher Mascio and Daniel A. Bambini
Pediatric inflammatory bowel disease (IBD) is somewhat different from adult

IBD since it is typically associated with a more severe clinical course, concurrent
emotional problems, growth and pubertal delay, and increased risk of colon cancer
from long-standing disease. IBD includes both ulcerative colitis and Crohn’s dis-
ease. Although considered distinct entities, clinical differentiation between the two
is not always clear-cut. Clinical presentation, radiographic evaluation, and patho-
logic findings are all given careful consideration to achieve an accurate diagnosis.
The incidence of pediatric IBD is estimated to be between 3-10 per 100,000
children per year. Males and females are affected equally and approximately 25% of
patients have a family history of IBD. Both ulcerative colitis (UC) and Crohn’s
disease occur more frequently in the Jewish population than other ethnic groups.
IBD is uncommon in Asian and African-American children; it affects white chil-
dren 4-5 times more frequently than black children.
The exact cause of IBD is currently unknown but a multifactorial etiology is
likely. Children with ulcerative colitis often have a characteristic genotype
(HLA-W27) suggesting genetic factors predispose to the development of this dis-
ease. One theory suggests UC develops as an immunologic response to an unidenti-
fied colonic antigen. The proposed antigen may be of bacterial, viral, or autologous
origin.
Conditions associated with the development of Crohn’s disease are allergic
hypersensitivity, vasculitis, and autoimmune disease. Genetic or environmental fac-
tors (i.e., infection, smoking, second hand smoke inhalation) may also contribute to
the pathogenesis of Crohn’s disease. Pathologic specimens from patients with Crohn’s
disease frequently have lymphangiectasis and mesenteric adenopathy suggesting that
obstructive lymphangitis plays a causal role.
Ulcerative Colitis
Most children with ulcerative colitis develop symptoms between the ages of 10-20
years. Only 4% of these patients develop symptoms before the age of 10 years. The
most common presenting symptoms are diarrhea and rectal bleeding. The typical
scenario is a episode of diarrhea followed by the appearance of bloody mucus or pus
Inflammatory Bowel Disease 403
in the child’s stools. Other symptoms and signs include tenesmus, crampy abdominal
pain, anorexia, weight loss, growth retardation, and anemia (67%). Although the
onset of symptoms is usually insidious, 15% of children present with acute fulmi-
nant colitis (i.e., severe abdominal pain, profuse bloody diarrhea, fever, sepsis)
requiring aggressive medical (and sometimes surgical) therapy. Of these children,
5% develop toxic megacolon. Extracolonic manifestations of UC include sclerosing
cholangitis, fatty liver, arthralgias (25%), arthritis, uveitis (< 2%), osteoporosis,
erythema nodosum, pyoderma gangrenosum, nephrolithiasis (8%), and aphthous
stomatitis.
Diagnosis
Because ulcerative colitis usually affects the rectum (95%), flexible sigmoidos-
copy or colonoscopy is the best initial diagnostic study. Endoscopic findings of UC
include mucosal friability, pseudopolyps, and ulcers. Biopsies are obtained to con-
firm the diagnosis. Barium enema (BE) is an effective means to evaluate the entire
colon but occasionally worsens or precipitates an episode of acute colitis. BE find-
ings that suggest a diagnosis of ulcerative colitis include ulcerations, pseudopolyps,
mucosal “thumbprinting,” loss of haustra, and a shortened, narrowed, rigid-appear-
ing colon. The BE exam may be entirely normal in the early stages of ulcerative
colitis.
Recently, serological assays have been developed which help distinguish types of
inflammatory bowel disease in children (Table 88.1). Assays for perinuclear
antineutrophil cytoplasmic antibodies (pANCA) are positive in children with UC
with over 90% specificity. While some patients with Crohn’s disease have a positive
assay for pANCA, their clinical presentations often resemble that of ulcerative coli-
tis. Children with UC remain positive for pANCA even after resection.
Ulcerative colitis usually develops first at the rectum and progresses proximally.
It affects only the large intestine, and 95% of patients have rectal involvement. The
severest cases involve the entire colon (pancolitis), but the greatest amount of
inflammation and pathologic changes are always within the rectosigmoid colon.
Chronic inflammation and ulceration of the mucosa and submucosa lead to the
88
formation of pseudopolyps (Fig. 88.1). Crypt abscesses are the most distinguishing
microscopic feature of ulcerative colitis.
The risk of colon carcinoma in children with UC is approximately 2-4% after
10 years of active disease. The risk increases by 15-20% in each subsequent decade.
If colonic mucosal biopsies demonstrate dysplasia, the risk of carcinoma is very
high.
Treatment and Outcomes
Primary medical therapy includes systemically and locally administered corti-
costeroids, sulfasalazine, and oral metronidazole. The use of other immunosuppres-
sive agents (i.e., azothioprine, cyclosporin) is controversial. The child’s nutritional
status is assessed and supplemental multivitamins and/or iron are administered as
necessary. During acute flare-ups, hospitalization with bowel rest, intravenous fluids,
parenteral nutrition, and intravenous steroids are often required to control symptoms.
Table 88.1. Serological assays for the diagnosis of pediatric inflammatory bowel
disease
Assay Ulcerative colitis Crohn’s disease

Perinuclear antineutrophil
cytoplasmic antibodies (pANCA) ↑↑↑ ↑
Anti-Saccharomyces cerevisiae
antibodies (ASCA) — ↑↑↑
Fig. 88.1. Total colectomy specimen showing extensive ulcerative colitis and loss
of large sections of colonic mucosa and pseudopolyps.
88
Indications for elective surgical intervention include chronicity, anemia, growth
retardation, failure to thrive, and an unacceptable quality of life. Emergent surgical
therapy is occasionally required in cases with severe hemorrhage, perforation, or
toxic megacolon that fails to respond promptly to medical therapy. Total
proctocolectomy provides a cure since ulcerative colitis affects only the colon and
rectum. Reconstructive options include:
1. permanent ileostomy,
2. endorectal ileal pullthrough (i.e., straight ileoanal, J-pouch, S-pouch, etc.),
and
3. continent ileal reservoir (i.e., Koch pouch).
A diverting ileostomy is used to protect the reconstruction and reduce the risk of
pelvic infection. Ileostomy closure is generally performed at 3-4 months following
proctocolectomy.
Complications following proctocolectomy and pull-through procedures in chil-

dren with UC include chronic reservoir inflammation (pouchitis), anastomotic stric-
ture, diarrhea, increased stool frequency and urgency. Most children experience 3-6
bowel movements per day. Long-term, less than 5% experience daytime soiling,
although 15% will have occasional nocturnal soiling.
Crohn’s Disease
Although Crohn’s disease usually affect young adults, 15-20% of patients de-
velop symptoms before age 15 years. The most common presenting signs and symp-
toms of Crohn’s disease in children are:
1. anorexia with weight loss (90%),
2. abdominal pain (70%),
3. diarrhea (67%),
4. anemia, and
5. fever.
Extraintestinal manifestations of Crohn’s disease include conjunctivitis, growth
retardation, arthritis, nephrolithiasis, cholelithiasis, and digital clubbing.
Although Crohn’s disease can affect any portion of the gastrointestinal tract,
ileocolitis is the most common form. Isolated colonic involvement occurs in 30-35%,
while isolated small bowel disease only occurs in 10% of children.
Diagnosis
A complete radiographic evaluation includes all portions of the gastrointestinal
tract. Contrast studies (i.e., upper gastrointestinal series with small bowel follow
through, barium enema) provide the most information. Radiographic features of
Crohn’s disease include thickened mucosal folds, linear ulcerations, “cobblestoning”
of the mucosa, intestinal fistulas, sinus tracts, and strictures. In children suspected
of having an intra-abdominal abscess, computed tomography (CT) is useful. Tissue
samples are obtained by upper and/or lower endoscopy to confirm the diagnosis.
Laboratory findings consistent with Crohn’s disease include anemia, elevated
erythrocyte sedimentation rate (ESR), prolonged prothrombin time, and hypoalbu-
88
minemia. Stool cultures are generally negative for pathologic organisms. Serological
assays for anti-Saccharomyces cerevisiae antibodies (ASCA) are elevated and highly
disease specific to Crohn’s disease. Titers for ASCA decrease toward normal levels
following resection.
In contrast to ulcerative colitis, Crohn’s disease is a transmural inflammatory
process. The intestinal wall is thickened with areas of submucosal edema, fibrosis,
and granuloma formation. The granulomas affect all bowel layers, contain multi-
nucleated giant cells, and are present in about 60% of children with the disease.
Skip areas of intestinal involvement separated by normal segments of bowel are a
distinguishing feature of Crohn’s disease. Transmural bowel wall inflammation com-
monly leads to fistula formation to the skin, urinary bladder, vagina, and other
portions of small and/or large bowel. Mucosal ulcers produce the characteristic
“cobblestone” appearance.
88
Fig. 88.2. Intraoperative demonstration of inflamed, thickened terminal ileum with

thickened mesentery and “fat creep” onto bowel.
Treatment
Initial medical treatment includes dietary modification (i.e., high calorie, high
protein), administration of azulfidine (sulfasalazine). More severe cases may require
the use of 5-aminosalicylic acid compounds, prednisone, cyclosporin, azathioprine,
or methotrexate either alone or in combination. Metronidazole also helps relieve
and control symptoms. Hospitalization is required for severe symptoms or contin-
ued malnutrition despite outpatient therapies. Total parenteral nutrition, bowel rest,
and electrolyte repletion are sometimes required.
Surgery is noncurative for Crohn’s disease but may impart clinical improvement
and/or remission. In addition, resection allows reduction or even discontinuation of
some medications for variable periods of time. The usual indications for operation
include three complications: fistula, abscess, and obstruction. Failure to thrive is a
less common indication for surgical therapy. The benefit of surgical intervention is
carefully weighed against the high risk of recurrence after each operation. The over-
all surgical goal is to preserve bowel length. Bowel-preserving techniques include:
1. repairing multiple strictures with enteroplasties,
2. avoiding segmental resection whenever possible, and
3. taking small margins of normal bowel when resection and anastamosis is
required.
Despite periods of remission, the incidence of recurrence is as high as 70% at
one year, even with complete resection of all grossly involved bowel. The incidence
of reoperation increases as these children are followed throughout life.
Selected Readings
1. Pettei MJ, Davidson M. Extra-gastrointestinal manifestations of inflammatory bowel
disease. J Pediatr Gastroenterol Nutr 1985; 4:689.
2. Castile RG et al. Crohn’s disease in children: assessment of the progression of dis-
ease, growth, and prognosis. J Pediatr Surg 1980; 15:462.
3. Coran AG, Klein MD, Sarahan TM. The surgical management of terminal ileal
and right colon Crohn’s disease in children. J Pediatr Surg 1983; 18:592.
4. Dehn TCB et al. Ten-year experience of strictureplasty for obstructive Crohn’s
disease. Br J Surg 1989; 76:339.
5. Coran A. A personal experience with 100 consecutive total colectomies and straight
ileoanal endorectal pull-throughs for benign disease of the colon and rectum in
children and adults. Ann Surg 1990; 212:242.
6. Fokalsrud EW, Loar N. long-term results after colectomy and endorectal ileal
pullthrough procedure in children. Ann Surg 1992; 215:57.
7. Ruemmele FM, Targan SR, Levy G et al. Diagnostic accuracy of serological assays
in pediatric inflammatory bowel disease. Gastroenterology 1998; 115:822.
88
CHAPTER 89
Disorders of the Pancreas

Todd R. Vogel
The most common pancreatic disorders encountered in infants and children are:
1. pancreatitis,
2. congenital anatomic lesions,
3. carcinoma, and
4. hypoglycemia.
The first three of these entities are discussed in this section. Hypoglycemia is
discussed in Chapter 99.
Pancreatic Embryology and Anatomy
The pancreas develops in the 4th week of gestation and begins as two buds,
dorsal and ventral, from the endoderm of the duodenum. The growing dorsal por-
tion of the developing pancreas spans across the hepatic diverticulum while the
ventral portion lies below moving more distal. The dorsal and ventral portions fuse
in week ten. The distal portion will create the duct of Wirsung while the proximal
portion may obliterate or form the duct of Santorini. 10% of the population will
have a double collecting system in the pancreas. Fetal insulin production begins in
the fifth gestational month and the exocrine function is present at birth.
The pancreas is a retroperitoneal organ located at the vertebral L1-L2 level. The
head of the pancreas lies to the right of the vertebral column and, along with the
uncinate process, is intimately adherent to the duodenum. The body of the pancreas
lies anterior to the superior mesenteric artery and vein, and the portal vein. The
arterial supply of the pancreas is derived from the gastroduodenal artery, superior
mesenteric artery, and the splenic artery. The head of the pancreas receives arterial
blood via the four pancreaticoduodenal arteries (i.e., anterior superior, anterior infe-
rior, posterior superior, and posterior inferior). Venous drainage is via the splenic
and portal vein.
Acute Pancreatitis
Incidence
Acute pancreatitis is an uncommon disease in children but has higher morbidity
and mortality than in adults.
Disorders of the Pancreas 409
Etiology
The vast majority of cases of pancreatitis in children are from blunt abdominal
injury. In the pediatric population, nearly 40% of cases of traumatic pancreatitis are
attributable to bicycle-related injury. After trauma, the most common causes of pan-
creatitis in children are drug therapy (corticosteroids, azathioprine, thiazides, furo-
semide, tetracyclines, and valproic acid), viral infection (Epstein-Barr, Coxsackie,
enterovirus, and mumps), and bacterial infection. Cystic fibrosis, biliary disease,
vasculitic diseases (systemic lupus, Henoch-Schonlein purpura), and type I and V
hyperlipidemias are also associated with acute pancreatitis in the pediatric popula-
tion.
Children present with vague abdominal pain which is exacerbated by eating.
The classic symptom of pain radiating to the back is rarely observed in the pediatric
population. Nausea and vomiting may be present. Rarely, patients may present with
a small bowel obstruction or young women may present with salpingitis secondary
to pancreatitis.
Diagnosis
Serum amylase, trypsinogen, and lipase levels are useful to establish the diagno-
sis of acute pancreatitis. An elevated serum amylase is the usual biochemical abnor-
mality associated with acute pancreatitis. Because amylase production occurs from
other nonpancreatic sources (i.e., salivary gland), elevated serum amylase is rela-
tively nonspecific. Calculation of the amylase clearance may be helpful and is nor-
mally less than 5%. Trypsinogen and lipase are produced almost exclusively by the
pancreas; elevated serum levels are more specific for pancreatitis.
Computed tomography (CT) is the best radiographic study to image the pan-
creas in cases of severe or complicated pancreatitis. Abdominal CT is often obtained
as part of the trauma evaluation. Ultrasound is sometimes useful, but often only
provides limited visualization of the pancreas due to its retroperitoneal location and
interposed bowel gas which further limits the study. Endoscopic retrograde
cholangiopancreatography (ERCP) is an invasive test that can accurately delineate 89
pancreatic ductal anatomy. ERCP causes pancreatitis in 5-10% of cases and is gen-
erally avoided during the early phases of acute pancreatitis.
Treatment
Medical management is the mainstay of treatment for pancreatitis. Volume
resuscitation is essential to counter retroperitoneal third space fluid losses. Nasogastric
decompression is recommended to avoid gastric distention and patients are initially
maintained NPO with nasogastric decompression. Pain management is essential.
Meperidine is preferred because it does not cause sphincter of Oddi contraction like
morphine does. Hyper-alimentation may be necessary if the course of pancreatitis is
prolonged. Enteral feeding distal to the ligament of Treitz via duodenal feeding tube
is the preferred method of providing nutrition in refractory cases. The majority of
cases of pancreatitis are self-limited and resolve spontaneously with supportive therapy.
In severe cases (i.e., necrotizing pancreatitis, infected pancreatic necrosis), surgi-

cal intervention may be necessary for irrigation and/or debridement of the pancreas.
The morality rate in this scenario approaches 15%.
Chronic Relapsing Pancreatitis
Incidence
Chronic relapsing pancreatitis in children is rare.
Etiology
Chronic relapsing pancreatitis is usually associated with a hereditary disease such
as hyperlipidemia types I and V, aminoaciduria, or hyperparathyroidism. It has also
been described in association with familial pancreatitis, congenital anomalies, and
posttraumatic pancreatitis. The disease process causes changes in the pancreatic pa-
renchyma including calcification, nodularity, and fibrosis.
Children most commonly present with intractable abdominal pain. Some may
have pancreatic exocrine insufficiency. Endocrine insufficiency is exceedingly rare,
but can develop.
Diagnosis
The diagnosis is suggested by a history of recurrent episodes of acute pancreati-
tis. ERCP is recommended to determine ductal anatomy and identify abnormalities
and/or strictures. ERCP is mandatory before any operative procedure can be con-
sidered.
Treatment
Surgical therapy may be indicated in cases of severe unremitting pain or if ductal
strictures are present that cannot be managed endoscopically (i.e., stent, dilatation,
etc). Surgical treatment has also been suggested as a possible means to prevent the
progression of exocrine and endocrine insufficiency. Surgical therapy is guided by
the findings of the ERCP. Options include internal drainage (i.e., Roux-en-Y, lateral
89 pancreaticojejunostomy (Puestow procedure) and/or pancreatic resection.
Pancreatic Cysts
Etiology and Classification
Pancreatic cysts are broadly classified based on etiology. These are simply catego-
rized as:
1. congenital,
2. retention,
3. pseudocycts,
4. neoplastic, or
5. parasitic.
Congenital Pancreatic Cysts

Congenital cysts of the pancreas are a rare finding in children. The cysts may be
unilocular or multilocular and are most commonly found in the body or tail of the
pancreas. These cysts are lined with true epithelium and most commonly contain
nonenzymatic fluid. The majority of these lesions are asymptomatic unless they are
large. Symptomatic cysts are excised.
Retention Cysts
Pancreatic retention cysts occasionally occur in children and are associated with
chronic obstruction of the pancreatic ductal system. These cysts are filled with enzyme
containing fluid. Surgical treatment is by excision or internal drainage.
Pseudocysts
Approximately 90% of pseudocysts occur secondary to trauma. This condition
is more common in males (nearly 3:1). Patients present with symptoms (most com-
mon to least) of vomiting, abdominal pain, abdominal mass, fever, and anorexia.
Pseudocysts are usually located in the lesser sac and the cyst wall consists of granula-
tion tissue. If the cyst communicates with the pancreatic ductal system, high amy-
lase levels are measurable with the cyst fluid. Useful diagnostic tests include serum
amylase, ultrasound, and CT. Surgical treatment is indicated for large, persistent, or
infected/symptomatic cysts. The surgical options include external drainage,
cystgastrostomy, cystjejunostomy, or excision. Surgical therapy is associated with
low mortality, minimal morbidity, and low recurrence.
Congenital Pancreatic Abnormalities
Annular Pancreas
Annular pancreas is a rare congenital anomaly that occurs due to abnormal rota-
tion of the pancreatic ventral bud. It is the most common of the congenital pancre-
atic abnormalities. The annular pancreas usually completely encircles the second
portion of the duodenum. This anomaly is associated with Down’s syndrome,
abnormalities of rotation, duodenal atresia (see Chapter 59), and biliary atresia.
Seventy percent of children with this lesion are symptomatic and will present with 89
high intestinal obstruction. The emesis is most often bilious but can be nonbilious
as well. Surgical therapy (see Chapter 59) consists of bypass anastomosis:
duodenoduodenostomy or duodenojejunostomy. Division of the pancreatic tissue
is not recommended due to the high association of fistula formation. Gastrojejunos-
tomy is not recommended due to associated growth problems and the risk of marginal
ulcers.
Pancreatic Divisum
Pancreatic divisum is a congenital anomaly in which the dorsal and ventral pan-
creatic tissue fail to fuse in utero. Pancreatic divisum is identifiable in 10-15% of the
population. This condition is usually asymptomatic, however there may be an
increased incidence of pancreatitis due to the inability of the accessory duct to
adequately drain the pancreatic tissue. Diagnosis is made exclusively with ERCP.
Treatment, if necessary, is by endoscopic or surgical sphincterotomy of the accessory
ampulla.
Fig. 89.1. Small, antimesenteric remnant of aberrant tissue that proved to be a

pancreatic rest.
Ectopic Pancreas
Ectopic pancreatic tissue (Fig. 89.1) is frequently identified in the duodenum,
colon, pylorus, appendix, or a Meckel’s diverticulum. Ectopic pancreas can cause
local inflammation and bleeding and is noted in approximately 3% of postmortem
examinations.
Pancreatic Neoplasms
Neoplasms are an extremely rare surgical problem in infants and children. Only
about 70 cases have been reported in the literature. Neoplasms may be cystic or
89 solid masses. Solid masses are more likely to be malignant. The most common
malignant tumors are:
1. adenocarcinoma,
2. islet cell carcinoma,
3. undifferentiated carcinoma, and
4. ductal cell carcinoma.
Tumors may be endocrinologically active or silent. Patients present with various
clinical symptoms including abdominal pain, hypoglycemia, abdominal mass, and
jaundice. The treatment is surgical excision with encouraging long term survival.
Of the endocrinologically active tumors, insulinoma is the most common.
Insulinoma is a benign tumor usually seen in children greater than 4 years of age.
Patients usually present with Whipple’s triad. Gastrinoma is the second most com-
mon of the endocrinologically active tumors. Gastrinoma is associated with
hypergastrinemia and peptic ulcer disease. The majority of gastrinomas are malignant.
Selected Readings
1. Ghishan et al. Chronic relapsing pancreatitis in children. J Pediatrics 1983;
102(4):514-518.
2. Jaksic T et al. A 20 year review of pediatric pancreatic tumors. J Pediatr Surg 1992;
27(10):1315-1317.
3. Petersen C et al. Surgical therapy and follow up of pancreatitis in children. J Pediatr
Gastroent 1997; 25(2).
4. Raffensperger JG. Pancreatitis. In: Raffensperger JG ed. Swenson’s Pediatric Sur-
gery, 5th edition. Norwalk: Appleton & Lange 1990; 891-894.
5. Roberts I. Disorders of the Pancreas in Children, Gastroenter Clinics Nor Am
1990; 19:No. 4.
89
Section XIV: Endocrine Disorders
CHAPTER 1
CHAPTER 90
Pheochromocytoma
Richard Fox
Incidence
Pheochromocytoma is an uncommon neoplasm of childhood. It presents with
symptoms of catecholamine excess that can be cured with prompt diagnosis and
treatment. In the general population, pheochromocytoma may occur in 1 per 50,000.
Of the patients with nonessential hypertension, 0.1-0.2% of adults and 1% of children
have pheochromocytomas. Ten percent of reported tumors occur in childhood of
which 90% occur sporadically. A strong familial relationship is demonstrated by the
high concordance rates among both children and adults, 5-10% and 2.5%
respectively. Four percent of tumors are associated with various neurocutaneous syn-
dromes, including Von Recklinghausen’s neurofibromatosis, Sturge Weber, Von
Hippel Lindau and the MEN (multiple endocrine neoplasia) IIa and MEN IIb
syndromes. Bilateral tumors are present in 70% of children which is nearly 10 fold
greater than the bilaterality rate observed in adults. Bilateral tumors occur most
frequently in children afflicted with familial tumor syndromes (i.e., MEN). Bilater-
ality is less prominent in those with nonsyndromatic familial tumors. The right
adrenal gland is affected twice as frequently as the left. Hormonal influences may
play a role in the development of pheochromocytoma. Males are affected more often
than the females during the preadolescent years while the opposite occurs after the
onset of puberty.
Pheochromocytoma typically presents in children between the ages of age 8-14
years. In contrast to adults, who generally present with paroxysms of hypertension,
children often demonstrate repeated sustained episodes of elevated blood pressure.
Symptoms often present acutely without prodrome in children. Common com-
plaints include headache, sweating and nausea. Others note polydipsia or polyuria,
visual disturbances and seizures, or even weight loss despite a voracious appetite.
Rarely, other children complain of vague somatic or bony pains, often signifying the
presence of metastatic disease. In any event, symptoms that remain unrecognized
may result in congestive heart failure, hypertensive retinitis, encephalopathy and
ultimately death.
Physical exam often demonstrates elevated blood pressure, tachycardia, flushing
and diaphoresis. Occasionally, extremity exam evaluation reveals puffy, cyanotic,
mottled hands.
Nearly every organ system is included in the differential diagnosis. Diabetes

mellitus, hyperthyroidism, Conn’s or Cushing’s disease, and adrenogenital syndrome
are the major endocrinopathies to be considered. These conditions all result from
excess tumor steroid production. Bilateral femoral pulses are assessed to exclude the
diagnosis of coarctation of the aorta. Renal artery stenosis and renal parenchymal
diseases, including pyelonephritis, glomerulonephritis, and neoplasm, are all poten-
tially suspect. Cerebrovascular disease, psychiatric conditions, and lead poisoning
also mimic the findings observed in patients with pheochromocytoma. Finally, in
children who demonstrate paroxysms of hypertension, familial dysautonomia (Riley-
Day syndrome) is a consideration.
Diagnosis
Urinalysis is the most sensitive diagnostic test. Urinary free catecholamines,
vanillamandelic acid (VMA) and metanephrines (MN) are measured. Classically, a
twenty-four hour urine collection is preferred, however, an overnight sample suf-
fices. Finally, new and improved radiographic studies (i.e., MIBG (meta-
iodobenzylguanidine) scan) facilitate tumor localization prior to laparotomy.
Metanephrines are by far the most reliably measured breakdown product. A false
negative rate of 4% is acceptable. VMA analysis carries a false positive rate of nearly
25%. This is secondary to variation in daily creatinine clearance or ingestion of raw
fruit, coffee or tea, that alter its value. However, VMA level often correlates directly
with tumor size which is a definite prognostic advantage. In patients with elevated
homovanillic acid (HMA) levels, a diagnosis of either neuroblastoma or rarely, a
pure dopamine secreting tumor, is suspected.
Radiographic modalities available include ultrasound, computed tomography
(CT) scan, magnetic resonance imaging (MRI), and MIBG nuclear scan. Tests that
have fallen out of favor include selected venous sampling and arteriography, as well
as provocative testing utilizing histamine or phentolamine. These tests all carry sig-
nificant risk for morbidity and mortality from potential precipitation of catechola-
mine crisis. Furthermore, the current radiographic studies are far superior in safety,
simplicity and diagnostic accuracy.
Ultrasound is a highly sensitive diagnostic tool in skilled hands. Some institu-
tions boast a 100% accuracy rate with this modality. Success is entirely operator
dependent based heavily upon the operator’s level of expertise.
90 CT scan detects 95% of tumors. Missed tumors include very small lesions
(1-1.5cm), extra-adrenal lesions, adrenal hyperplasia, and residual or recurrent tumors.
Similarly, MRI is also excellent in diagnosis of pheochromocytoma. Its advantage
over CT is in superior adrenal imaging with adjustment of T1 and T2 weighted
images.
MIBG scanning excels in detection of extra-adrenal tumors that CT or MRI
fails to detect. MIBG may also play a significant role in detection of bony metastasis
with accuracy that complements bone scanning. MIBG inhibits catecholamine uptake
by chromaffin cells and subsequently blocks adrenergic neurons. It is stored in vesicles
as a norepinephrine analogue. Its uptake is proportionate to the number of secretory
granules in adrenal or tumor cells. MIBG’s main drawback is the relative inability to
distinguish normal adrenal gland from tumor, especially in children with MEN
Pheochromocytoma 417
who have hyperplastic adrenal glands. False negative results occur in the presence of
medications that interfere with vesicular uptake of norepinephrine. Examples include
oral decongestants, antipsychotics, tricyclic antidepressants, calcium channel blockers,
cocaine, and some beta blockers.
Pheochromocytoma is named after the predominant cell from which it derives,
the pheochromocyte (black cell). Catecholamines are stored in intracellular vesicles
that when oxidized by dichromate fixative, result in a characteristic black appear-
ance (the chromaffin reaction). Tumors are yellow-brown in color and often appear
well circumscribed (Fig. 90.1). Tumors average 3-6 cm in diameter and usually weigh
less than 50 grams. Histologically, cells line up in discrete rows or nests called
“zellballen,” meaning balls of cells.
The sympathetic chain develops from totipotential neural crest cells that emi-
grate from the cervical aorta caudally along all major aortic branches toward the
pelvis. These precursor cells form the major paraganglia and adrenal medulla.
Accordingly, extra-adrenal tumors are located in these areas. Vasoactive amines are
secreted by these cells causing the characteristic symptoms of pheochromocytoma.
Seventy percent of tumors are located within the adrenal medulla, whereas up to
30% are found in an extra-adrenal location. Ninety-five percent of all tumors are
located below the diaphragm. The most common site of extra-adrenal tumor is
within the bladder. In these cases, hematuria is often an initial presenting symptom.
Other sites include the “organ of Zuckercandl” (i.e., paraganglionic tissue at the
aortic bifurcation), renal hilum, chest or mediastinum, carotid body, and prostate.
Rarely, tumor is noted in both intra- and extra-adrenal locations. If tumors are iden-
tified in places not commonly inhabited by chromaffin tissue, a careful evaluation
for metastatic disease is indicated. Unlike most tumors, histology fails to distinguish
benign from malignant lesions. Clinical behavior alone makes this determination.
In children, 3-6% of tumors are malignant compared to 10% of those in adult
patients.
Tumors arising from adrenal medulla secrete both epinephrine and norepineph-
rine. Extra-adrenal tumors primarily secrete norepinephrine because they lack the
enzyme phenylethanolamine-N-methyltransferase. Incidentally, dopamine is found
in many tumors. However, it is rarely secreted, nor is it responsible for symptoms.
Excess catecholamine activates both alpha and beta receptors. Specifically, norepi- 90
nephrine activates alpha receptors. Epinephrine, on the other hand, stimulates both
alpha and beta receptors resulting in diastolic hypertension from peripheral vaso-
constriction. Reflex bradycardia is occasionally observed.
Treatment
Treatment of pheochromocytoma is primarily surgical. Paramount for a success-
ful outcome is adequate preoperative blood pressure control and fluid management.
The goal of therapy is to optimize patients hemodynamically in an effort to avoid
intraoperative cardiovascular crisis. Adequate alpha blockade (and sometimes beta
blockade) for 1-2 weeks is often necessary to reduce blood pressure. Beta blockade is
added when children:
Fig. 90.1. Pathological specimen of a well-circumscribed, suprarenal gland tumor

that was cytologically a pheochromocytoma.
1. demonstrate signs or symptoms of cardiac arrhythmia,

2. experience tachycardia greater than 140 beats per minute, or
3. have pure epinephrine secreting tumors.
Alpha blockade is always given prior to beta blockade to avoid potential cat-
echolamine-induced cardiomyopathy from either a direct toxic effect to the myo-
cardium or from ischemic changes from intense, unopposed vasoconstriction. Of
note, beta blockade may interfere with analysis of urinary metanephrines. If blood
pressure is not critically elevated, phenoxybenzamine is administered orally and
titrated to normotension over a period of 1-2 weeks. This medication works slowly.
If critically elevated blood pressures are present, rapid intravenous correction with
phentolamine (regitine) or nitroprusside is recommended. Predictors of a favorable
90 surgical outcome are the ability to successfully normalize blood pressure and/or relieve
symptoms with medical management. An even more sensitive indicator is the nor-
malization of urinary metanephrines.
Preoperatively, nutritional supplementation is often necessary to replenish energy
stores lost to hypermetabolism. A hypermetabolic state is the direct result of cat-
echolamine excess. In general, patients with elevated basal metabolic rates perform
and recover poorly overall.
Prior to induction of general anesthesia, appropriate volume resuscitation is cru-
cial. On average, approximately 15% of the circulating volume may need replace-
ment. This is due to chronic catecholamine-induced vasoconstriction with subsequent
loss of plasma and red blood cell mass. This is especially important prior to induc-
tion of general anesthesia where global vasodilation may precipitate cardiovascular
crisis from hypovolemia.
Pheochromocytoma 419
Two phases of general anesthesia are encountered. First is the hypertension phase.
This includes all events prior to vascular control and excision of tumor and results
from catecholamine surge. After tumor excision, circulating catecholamine level may
precipitously drop. This begins the second phase, the hypotension phase. In addi-
tion to blood pressure changes, blood sugars must also be cautiously monitored as
hypoglycemia ensues. Medications must be immediately available to treat any of the
above conditions as they arise. Useful medications include sodium nitroprusside,
phentolamine and diazoxide. Anti-arrhythmics, such as lidocaine, magnesium sul-
fate or bretyllium, should also be readily available.
Since 95% of tumors are intra-abdominal, a subcostal or transverse upper
abdominal incision is traditionally employed. In certain circumstances (i.e., obese
children), a flank approach might facilitate resection. Furthermore, in cases with
tumors less than 10 centimeters, a laparascopic approach is a consideration. Regard-
less of method used, the critical step in tumor resection is careful and rapid venous
control of the tumor. Resection includes the involved adrenal gland unless an extra-
adrenal tumor is the problem. Most intraoperative bleeding is encountered on the
right side resulting from injury to the shorter, right adrenal vein. Prior to closure, a
careful exploration of the contralateral adrenal gland, periaortic areas, mesentery,
and retroperitoneum, is performed to identify either synchronous or metastatic dis-
ease. A few surgeons advocate a contralateral adrenal biopsy.
Outcomes
Children with benign tumors who undergo resection do remarkably well. Surgi-
cal morality is less than 5%. Recurrence does unfortunately occur and usually does
so within 5 years of initial resection. Patients with MEN II are at highest risk for
recurrence. Accordingly, many surgeons advocate routine bilateral adrenalectomy in
syndromatic patients on initial exploration. However, others find this approach
excessively radical and propose that partial contralateral adrenalectomy or even rou-
tine observation suffice. This eliminates the need for longterm steroid replacement.
If blood pressure does not normalize within 2 weeks postoperatively, a search for a
second primary tumor or metastasis is required. Metastases occur in tissues where
chromaffin cells are otherwise absent; lymph nodes, liver, lung and bone. In patients
with metastatic disease, combined modality chemotherapy or MIBG therapy are
options. Radiation therapy is also considered. Few studies are available regarding
long-term outcomes and treatment results in pediatric patients with malignant pheo- 90
chromocytoma.
Selected Readings
1. Doski JJ, Robertson FM, Cheu HW. Endocrine tumors. In: Andrassy RJ ed. Pedi-
atric Surgical Oncology. Philadelphia: W.B. Saunders Company 365-403.
2. Caty MG, Coran AG, Geagen M et al. Current diagnosis and treatment of pheo-
chromocytoma in children. Arch Surg 1990; 125:978.
3. Ein SH, Weitzman S, Thorner P et al. Pediatric malignant pheochromocytoma. J
4. Turner MC, Lieberman E, DeQuattro V. The perioperative management of pheo-
chromocytoma in children. Clin Pediatr 1992; 10:1248.
CHAPTER 91
Hyperparathyroidism
P. Stephen Almond
Incidence
Hyperparathyroidism is uncommon in the pediatric population.
Etiology
In the fourth week of gestation, the rostral end of the fetus is characterized by six
branchial arches, each with its own artery, nerve, and mesenchymal core. Externally,
these arches are separated by clefts (branchial clefts) and internally by pouches (bran-
chial pouches). The third and fourth branchial pouches each develop a dorsal and
ventral wing. In the fifth week, the dorsal wing of each differentiates into parathy-
roid tissue while the ventral wings differentiate into thymus (third branchial pouch)
and the ultimobranchial body (fourth branchial pouch). Due to its connection with
the thymus, the parathyroid tissue of the third branchial pouch is pulled below that
of the fourth to become the inferior parathyroid glands. The parathyroid tissue of
the fourth pouch becomes the superior parathyroid.
Parathyroid hormone is an 84 amino acid, polypeptide chain secreted by the
parathyroid glands in response to low serum calcium. The hormone is cleaved into
a biologically active amino-terminal fragment and an inactive, but easily measured,
carboxyl-terminal fragment. The active fragment binds to receptors on bone and
renal tubule cells, stimulating adenylate-cyclase and the generation of cyclic AMP
(cAMP). Within bone cells, an increase in intracellular cAMP causes resorption of
bone with release of calcium and phosphate. Within the renal tubule, increased
intracellular cAMP in response to PTH causes calcium resporption, phosphaturia,
and magnesium resorption. In addition, PTH increases intestinal absorption of cal-
cium by stimulating the conversion of 25-hydroxycolecalciferol vitamin D to the
more potent, 1,25-dihydroxycholecalciferol vitamin D.
Hyperparathyroidism is either hereditary or sporadic and may result from
hyperplasia, adenoma, or malignancy. Neonatal hyperparathyroidism is rare, auto-
somal recessive in some cases, and due to four gland hyperplasia. Familial hyperpar-
athyroidism is more common, autosomal dominant or autosomal recessive in some
cases, and due to chief-cell hyperplasia. Parathyroid adenoma is the most common
cause of hyperparathyroidism in children. Only two cases of parathyroid cancer
have been reported in children.
Hyperparathyroidism 421
The hyperparathyroid state affects the nervous, genitourinary, gastrointestinal,
and musculoskeletal systems. Therefore, the presenting symptoms are often vague,
nonspecific, and difficult to substantiate. In neonatal hyperparathyroidism, the new-
born presents with failure to thrive, dehydration, respiratory distress, and poor feed-
ing. On examination, the infant may appear lethargic, underweight, and have
decreased muscular tone. The vast majority of older children with hyperparathy-
roidism are sporadic cases. These children present with bone pain, weakness, vague
gastrointestinal symptoms, nephrocalcinosis, and nephrolithiasis. Frequently, the
physical examination is unremarkable.
Children with familial hyperparathyroidism [MEN type I (pituitary adenoma,
hyperparathyroidism, and pancreatic neoplasm), MEN type IIA (medullary carci-
noma of the thyroid, hyperparathyroidism, and pheochromocytoma), and familial
hypocalciuric hypercalcemia] are usually identified during screening of previously
diagnosed family members. Nevertheless, history and physical examination should
be aimed at identifying familial cases.
Diagnosis
The diagnosis of hyperparathyroidism is based on elevated serum calcium
(> 11 mg/dl) and PTH levels. In > 90%, the serum calcium to phosphorus ratio is
> 33. Serum creatinine and BUN are measured to assess volume status and renal
function. A 24-hour urine collection for calcium is sent to rule out familial
hypocalciuric hypercalcemia. A urine calcium level < 100 mg/24 hours is suggestive
of this diagnosis.
Hyperparathyroidism is due to hyperplasia, adenoma, or malignancy. Adenoma
is the most common cause in children. Chief cell hyperplasia is the cause in neonatal
hyperparathyroidism and familial hyperparathyroidism. Malignancy is extremely
rare.
Treatment
Once the diagnosis of primary hyperparathyroidism is established, surgical
exploration of the neck is indicated. Preoperatively, the serum calcium should be
lowered into the normal range with saline infusion and Lasix. If this is unsuccessful,
mithromycin may be used.
At operation, the skin and platysma are divided transversely along a curviliniar 91
line 1 cm above the clavicles and extending between the sternomastoid muscles. The
strap muscles are separated in the midline and retracted laterally. The thyroid gland
is inspected, palpated, and mobilized by dividing the middle thyroid veins and the
superior thyroid arteries. The lobes of the thyroid are rotated medially and all four
glands are visualized and biopsied. If all four glands are not identified, consideration
should be given to:
1. exploring the carotid sheath,
2. exploring the retroesophageal space,
3. exploring the thymus, and

4. performing thyroid lobectomy on the affected side. Sternotomy is gener-
ally not performed at the first operation.
The amount of parathyroid tissue to be removed depends on the preoperative
diagnosis and the appearance of the glands at operation. Neonatal hyperparathy-
roidism and MEN I are associated with chief cell hyperplasia and therefore, require
either total parathyroidectomy with autotransplantation of one gland or three and
one-half gland resection. Due to an increased risk of Hypoparathyroidism, children
with MEN IIA should have only enlarged glands removed. MEN IIB patients do
not require parathyroidectomy. Single adenomas should be removed after confirm-
ing three other normal glands. Meticulous hemostasis is obtained prior to wound
closure.
Outcomes
Complications of parathyroidectomy include:
1. damage to the recurrent laryngeal nerve (< 1%),
2. persistent or recurrent hyperparathyroidism (< 5%),
3. Hypoparathyroidism, and
4. hematoma (rare).
Recurrent laryngeal nerve injury may be secondary to transection, ligation, or
blunt trauma to the nerve. Although immediate repair is the treatment of choice for
a transected nerve, delayed repair, anastomosis to the vagus nerve, burying the nerve
in the posterior cricoarytenoid muscle have been described. Injury to the nerve causes
the affected vocal cord to assume a more medial position and can be identified with
laryngoscopy at the end of the procedure. Reversible injuries to the nerve usually
resolve within ten weeks.
Persistent hyperparathyroidism means that the patient remains hypercalcemic
postoperatively. This is usually due to misdiagnosis or retained hyperplastic or
adenomatous tissue. In recurrent hyperparathyroidism, the patient has a period of
normocalcemia followed by a return of hypercalcemia. This may be seen with par-
athyroid cancer, familial hyperparathyroidism, and familial hypocalciuric hypercal-
cemia. In the later two cases, the pathology is chief cell hyperplasia. At operation,
however, only enlarged glands are removed under the erroneous assumption that
they are adenomas.
Hypoparathyroidism may be permanent or transient. Clinical signs of hypocal-
cemia include circumoral numbness and tingling, peripheral paresthesias, hyperac-
91 tive tendon reflexes, positive Chvostek’s and Trousseu’s signs, muscle cramps, tetany,
and prolonged Q-T interval. Symptomatic patients are placed on a cardiac monitor
and treated with IV calcium gluconate infusion and Calcitrol (1,25 dihydroxy-
cholecalciferol vitamin D). As symptoms resolve and the calcium is corrected, cal-
cium supplementation is converted to oral calcium carbonate (i.e., Caltrate, TUMS)
and Calcitrol.
Hyperparathyroidism 423
A neck hematoma may present as life-threatening, respiratory distress. In this

scenario, the wound should be opened at bedside followed by immediate explora-
tion in the operating room.
Selected Readings
1. Allo M, Thompson NW, Nishiyama R. Primary hyperparathyroidism in children,
adolescents, and young adults. World J Surg 1982; 6:771.
2. Thompson NW, Carpenter LC, Kessler DL. Hereditary neonatal hyperparathy-
roidism. Arch Surg 1978; 113:102.
3. Harman CR, van Heerden JA, Farley DR et al. Sporadic primary hyperparathy-
roidism in young patients: a separate disease entity? Arch Surg 1999; 134:651.
91
CHAPTER 92
Neonatal Hypoglycemia
Daniel A. Bambini
Incidence
Although hypoglycemia in the newborn period is relatively common, hyperin-
sulinism only accounts for about 1% of cases of hypoglycemia in neonates. None-
theless, neonatal hyperinsulinism is the most common cause of persistent
hypoglycemia in the newborn, identifiable in about 50% of this group.
Etiology
Hypoglycemia in the newborn can result from hyperinsulinism, inborn errors of
hepatic metabolism, hormonal deficiencies and a variety of other causes. The most
common causes of neonatal hypoglycemia are listed in Table 92.1. In the neonate or
infant under 1 year of age, the most common cause of hyperinsulinism is
nesidioblastosis. Beyond one year, pancreatic adenoma is more common.
Nesidioblastosis
All cells of the pancreas are believed to arise from primordial duct cells.
Nesidioblastosis refers to the process by which islet cells bud from the pancreatic
ducts and is a normal part of fetal pancreatic development. The hyperinsulinism of
early infancy has often been attributed to the persistence of “nesidioblasts” within
the pancreas, either focal or diffuse, beyond the fetal period. Because nesidioblasts
are often identifiable in normal newborns as well, the pathophysiologic problem is
now believed to be secondary to abnormal mechanisms of insulin storage and re-
lease. The pathologic findings in pancreatic specimens taken from these infants are
often completely normal but sometimes demonstrate:
1. focal nesidioblastosis,
2. diffuse nesidioblastosis,
3. islet cell hypertrophy,
4. nuclear hypertrophy,
5. cellular dysplasia, or
6. adenomatosis.
The pathologic findings do not correlate well with the clinical severity of disease.
Neonatal Hypoglycemia 425
Table 92.1. Causes of neonatal hypoglycemia

Cause Transient Hyperinsulinism Persistent
or
Recurrent
Birth asphyxia +
Starvation +
Sepsis and/or hypothermia +
Congenital heart disease +
Low birth weight +
Interruption of venous infusion +
Excess exogenous insulin + +
Infant of diabetic mother + +
Erythroblastosis fetalis + +
Beckwith-Wiedemann Syndrome + +
Insulinoma + +
“Nesidioblastosis” + +
Leucine-sensitive hypoglycemia + +
Adrenal disease/cortisol deficiency * +
Growth hormone deficiency +
Hypopituitarism * +
Inborn error of hepatic metabolism* +
* associated with decreased glucose production
Islet Cell Adenoma

Insulin-secreting islet cell adenomas generally occur in older children (> 3-4
years) not infants. Seventy-five percent of adenomas arise in the body and tail of the
pancreas. Approximately 15% of these tumors are multicentric. Malignant
insulinomas are extremely rare in children. Grossly, the tumors appear “pink”, are
usually well-encapsulated (pseudocapsule), and are sometimes found in association
with nesidioblastosis. 92
Hypoglycemia in the newborn is associated with nonspecific, often subtle, clini-
cal findings. It should be expected or anticipated in neonates that are premature or
small for gestational age. Infants born to diabetic mothers are at risk for hypoglyce-
mia and often have transient hyperinsulinism (due to high glucose levels in utero).
These babies are frequently large for gestational age.
The presenting symptoms and/or signs of neonatal hypoglycemia include apnea,

cyanosis, limpness, irritability, tremors, seizures, coma, lethargy and hypothermia.
In older children, hypoglycemia may commonly present as nervousness, fatigue, or
seizures. As expected, symptoms are relieved by eating. Physical findings are usually
unremarkable, except in infants with other associated conditions. Most infants have
a high birth weight and hepatomegaly (from excess glycogen storage). The differen-
tial diagnosis of neonatal hypoglycemia is vast. Broadly speaking, hypoglycemia usu-
ally arises from hyperinsulinism, endocrine disorders, or inborn errors of hepatic
metabolism.
The Beckwith-Wiedeman syndrome is highly associated with hypoglycemia.
Features of this syndrome include macroglossia, gigantism, omphalocele. Some of
these infants may have nesidioblastosis but the hypoglycemia usually resolves with
medical therapy.
Diagnosis
The diagnostic work-up may be fairly urgent to avoid cerebral damage and men-
tal retardation associated with inadequately treated neonatal hypoglycemia. Endo-
crinologic and metabolic evaluation is indicated for hypoglycemia that:
1. lasts greater than one week after birth,
2. is refractory to glucose infusion, or
3. is acquired after discharge from the nursery.
The diagnostic work-up should include simultaneous measurements of serum
glucose and assay for serum insulin level. A normal or elevated insulin level in the
setting of hypoglycemia indicates hyperinsulinism.
The normal expected values for serum glucose in neonates is age-dependent.
Term infants less than 24 hours old should have serum glucose levels exceeding
35 mg/dl. Beyond 24 hours, the serum glucose should remain above 45 mg/dl.
Early after birth (< 72 hrs) premature/LBW infants should have serum glucose lev-
els greater than 25 mg/dl. Hypoglycemia, during the first three days of life, is de-
fined as a serum glucose less than 30 mg/dl in full term neonates or less than 20 mg/
dl in low birth weight infants. Beyond 72 hours of age, hypoglycemia is defined as a
serum glucose less than 40mg/dl.
The principle diagnostic criteria for hyperinsulinism are:
1. serum insulin > 10 mU/ml with simultaneous serum glucose < 50 mg/dl,
and
2. glucose requirement to maintain serum glucose above 35 mg/dl exceed-
ing 10 mg/kg/min.
Other indicators of hyperinsulinism are low serum concentrations of free fatty
92 acids and ketones and a glycemic response to parenterally administered glucagon.
Plasma or urinary C peptide levels are elevated.
Treatment
Prompt identification and treatment of hypoglycemia is essential to prevent per-
manent central nervous system damage. Initial medical therapy includes providing
intravenous glucose at concentrations of 15-20% dextrose at a rate sufficient to
maintain normoglycemia. Sometimes infusions of 10-25 mg glucose/kg/min are
required to maintain normoglycemia. Central venous catheterization is required.
Neonatal Hypoglycemia 427
Frequent feeds can provide additional glucose. Refractory hypoglycemia may be

treated by several pharmacologic agents including diazoxide, octreotide, and gluca-
gon. Other agents that have been used are mesoxalyl urea, corticosteroids and alpha-
adrenergic agents. Medical therapy controls hypoglycemia in about 50-75% of
patients. Neonates that cannot maintain a fast despite optimal medical manage-
ment should be considered for surgery.
Surgical therapy reduces insulin secretion by resecting pancreatic mass contain-
ing the beta cells. It is unusual to find any macroscopically visible lesion (i.e., adenoma)
within the pancreas at the time of laparotomy. The amount of pancreatic tissue that
should be resected is a controversial point. Subtotal (75%) pancreatectomy results
in a treatment failure rate of at least 50%. A spleen-sparing 95% pancreatectomy is
recommended by many but occasionally this results in postoperative endocrine
insufficiency. In the 95% resection, the pancreatic head, body and uncinate process
are resected leaving only the rim of pancreatic tissue on the common bile duct and
duodenum.
Outcomes
Postoperative complications include:
1. persistent or recurrent hypoglycemia,
2. mental retardation,
3. diabetes mellitus.
Hyperglycemia after 95% resection is usually only transient but late diabetes
mellitus has been reported in some patients as they enter adolescence. In patients
having 75% resections, almost 30% may require an additional resection to control
hypoglycemia. A 95% pancreatectomy does not absolutely prevent hypoglycemia as
5% of these patients may eventually require a second resection of pancreas which
hypertrophies. Another 10% may require diazoxide to control hypoglycemia, while
7-8% may need long-term insulin to control hyperglycemia. The mortality rate for
95% pancreatectomy is around 2.5%.
Selected Readings
1. Shilyansky J, Cutz E, Filler RM. Endogenous hyperinsulinism: Diagnosis, man-
agement, and long-term follow-up. Sem Pediatr Surg 1997; 6(3):115-120.
2. Spitz L, Bhargava RK, Grant DB et al. Surgical treatment of hyperinsulineamic
hypoglycemia in infancy and childhood. Arch Dis Child 1992; 67:201-105.
3. Jaffe R, Hashida Y, Yunis EJ. Pancreatic pathology in hyperinsulinemic hypoglyce-
mia of infancy. Nature Med 2:2344-2347.
4. Leibowitz G, Glaser B, Higazi AA et al. Hyperinsulinemic hypoglycemia of infancy
(nesidioblastosis) in clinical remission: High incidence of diabetes mellitus and
persistent beta-cell dysfunction at long-term follow-up. J Clin Endocrinol Metab
1995; 80:386-392.
92
CHAPTER 93
Intersex
Daniel A. Bambini
Sex assignment at birth is usually a straight forward anatomical decision. Confu-

sion may occur if an anomaly of genital development occurs. Disorders of intersex
occur when the phenotypic sex of an infant or child is discordant with his/her gonadal,
genotypic, or hormonal sex.
Incidence
Intersex anomalies are exceedingly rare; a precise overall incidence is not known.
Approximately 4-6 of every 10,000 births have some form of genital ambiguity. In
the United States and Western Europe, female pseudohermaphroditism is the most
common intersex disorder. Congenital adrenal hyperplasia, the most common cause
of female pseudohermaphroditism, occurs between 1 in 5,000-15,000 live births.
Testicular feminization syndrome, a form of male pseudohermaphroditism, occurs
between 1 in 20,000-64,000 live male births. True hermaphroditism is the rarest of
these disorders; less than 500 cases have been reported in the world literature this
century.
Development of the Gonads and Genitalia
Normal gonadal development is a complex and precise series of events. The
undifferentiated gonad is bipotential and the Y chromosome is essential for devel-
opment of the testes. The SRY gene produces a protein that initiates development of
the gonad into the testes. Presence of the SRY gene at fertilization may be the primary
determinant of gonadal sex. Two X chromosomes are required for ovarian differen-
tiation. During the fifth week of gestation, germ cells with XX or XY chromosomes
migrate from the yolk sac to the retroperitoneum to form germal epithelium at the
anteromedial urogenital ridge. Gonadal induction begins at the sixth week with
growth of these germ cells into the underlying mesenchyme. During the seventh
week of gestation, sex cords and seminiferous tubules in males. Sertoli cells begin to
produce Mullerian inhibiting substance (MIS), during the eighth week of gestation.
In females, primary follicles develop but usually not until the 9th-10th week of
gestation. Female development occurs in the absence of ovaries or gonads. Male
development only occurs if testosterone (androgen) is present.
By the sixth week of gestation there are two pairs of genital ducts in the embryo
whether male or female. The Wolfian (mesonephric) ducts are located posterome-
dial to the urogenital ridge and drain the mesonephric kidney. Under the influence
Intersex 429
of testosterone (Leydig cell), Wolfian ducts develop into the male genital ducts in-
cluding the vas deferens, seminal vesicles, and epididymis. The Mullerian ducts arise
anterolateral to the urogenital ridge. Mullerian ducts give rise to the uterus, fallo-
pian tubes, and vagina in the female. In males, MIS (Sertoli cell) prevents develop-
ment of the Mullerian structures that regress and disappear. Testosterone, produced
by the Leydig cells, stimulates Wolfian duct development and formation of the vas
deferens, seminal vesicles, and epididymis. In females, MIS is absent and the Mulle-
rian ducts develop into the uterus, fallopian tubes, and vagina.
Development of the external genitalia normally occurs during the 9th through
12th weeks of gestation. The genital tubercle and labioscrotal folds are identifiable
in the 4th week of gestation. In the absence of testosterone, the genital tubercle will
develop into the female clitoris and the labioscrotal folds become the labia minora
and majora. Development of male external genitalia requires reduction of testoster-
one to dihydroxytestosterone (DHT) by 5-reductase. Under the influence of DHT,
the genital tubercle enlarges to form the penis, the labioscrotal folds fuse in the
midline to form the scrotum, and the urogenital sinus closes.
Classification and Etiology
The four major categories of intersex anomalies are:
1. male pseudohermaphroditism,
2. female pseudohermaphroditism,
3. true hermaphroditism, and
4. mixed gonadal dysgenesis. Multiple etiologies have been identified for
each.
Male pseudohermaphroditism results from incomplete masculinization or com-
plete feminization in a genetic male (XY karyotype). Etiologies include:
1. inadequate biosynthesis of testosterone by the Leydig cell,
2. inability to convert testosterone to dihydroxytestosterone, or
3. absent/impaired binding of testosterone to the androgen receptor.
Enzymatic defects in 20-22 desmolase, 3-hydroxysteroid dehydrogenase, 17-
hydroxylase, 17-20 desmolase, or 17-ketosteroid reductase can cause testosterone
deficiency. Adrenal insufficiency is also common in first three. Testicular feminiza-
tion is an X-linked recessive syndrome that results from a defect in androgen recep-
tor function. Although external genitalia are normal and female, MIS function is
also normal, and there are no internal Mullerian structures present. Another variant
of male pseudohermaphroditism is the retained Mullerian duct syndrome. In this
syndrome, MIS production is defiant or the receptor for MIS is abnormal, yet test-
osterone metabolism and function is normal. In males so affected, the testes will be
undescended and Mullerian structures will persist (i.e., uterus, fallopian tubes, vagina).
Female pseudohermaphroditism is definable as abnormal masculinization in a
genetic female (XX karyotype). Female pseudohermaphroditism is usually caused
by exposure to endogenous or exogenous androgens in utero. Masculinization is 93
often severe. The most common cause is congenital adrenal hyperplasia (CAH) as a
result of an enzymatic deficiency in the biosynthesis of corticosteroid. The enzy-
matic deficiency responsible for 95% of cases CAH is 21-hydroxylase deficiency.
Salt wasting occurs in 75% of those with 21-hydroxylase deficiency secondary to
mineralcorticoid insufficiency. Deficiencies of 11-hydroxylase or 3-hydroxysteroid

dehydrogenase can also cause CAH and female pseudohermaphroditism.
True hermaphroditism usually occurs in individuals with a 46XX karyotype, yet
rare individuals with 46XY or mosaic karyotypes have been reported. Many 46XX
hermaphrodites have detectable HY antigen suggesting translocation of the short
arm of the Y chromosome containing a retained SRY segment. The 46 XX/46XY
mosaics are considered chimeras resulting from fusion of two fertilized ova and usu-
ally have an ovary on one side with a testes on the contralateral side. In these cases,
the internal ducts are consistent with their ipsilateral gonad. The majority of true
hermaphrodites have an ovotestis. The testicular tissue of either the unilateral testis
or ovotestis may be dysgenetic. True hermaphroditism is defined by the presence of
both ovarian and testicular tissue.
Mixed gonadal dysgenesis results from inadequate induction and formation of
the gonad. Most commonly, these individuals have sex chromosomal mosaicism
(45X/46XY) and a dysgenetic testis on one side with a streak gonad on the other.
Other individuals in this group may have unilateral gonadal agenesis or bilateral
streak gonads. About 40% of this group have a pure 46XY karyotype. Presence of a
Y chromosome increases the risk of tumor formation
Intersex anomalies are generally apparent at birth in the delivery room or on
initial physical examination because of genital ambiguity. Accurate gender assign-
ment may initially be impossible. Occasionally, genital ambiguity is overlooked in
the neonate, and these children with intersex anomalies may present at adolescence.
Any male with bilateral inguinal hernias and hypospadius is considered to poten-
tially have an intersex disorder until proven otherwise.
Evaluation of the intersex infant should include elucidation of history of mater-
nal exposure to drugs including alcohol, androgens and progesterone. Maternal
ingestion of hydantoin-based drugs or phenobarbital may effect cytochrome p450
enzymes essential for fetal steroid synthesis and metabolism. A thorough physical
examination is essential. The genitalia should be inspected for:
1. gonadal symmetry,
2. scrotal position relative to the penis,
3. darkened genital or areolar coloration,
4. rogation of the labioscrotal folds,
5. size and degree of chordee of the phallus,
6. position of the urethral meatus
7. dehydration consistent with salt wasting.
Physical findings that suggest an intersex problem include:
1. clitoromegaly,
2. penoscrotal or perineoscrotal hypospadius with bilateral cryptorchidism,
93 3. penoscrotal or perineoscrotal hypospadius with a unilateral palpable gonad,
and
4. micropenis without palpable gonads.
Dysmorphic features (i.e., shield chest, wide spaced nipples, web neck, etc.) sug-
gest an underlying chromosomal abnormality. Rectal exam/bimanual exam to assess
for a midline positioned uterus is required.
Intersex 431
Diagnosis
Preliminary diagnosis of intersex disorders can be made with 90% accuracy using
two criteria:
1. presence/absence of gonadal symmetry,
2. presence/absence of Barr body or chromatin mass on the buccal smear
(Table 93.1).
Evaluation of intersex infant begins with a careful physical examination of gonadal
symmetry. Gonadal symmetry refers to the relative position of one gonad to the
other above/below the external inguinal ring. The majority of intersex anomalies are
able to be classified relatively quickly with reasonable accuracy.
Laboratory testing that is indicated in the evaluation of intersex infants includes
the buccal smear with Y fluorescence to identify Barr body/chromatin mass (i.e.,
second X chromosome). Additional blood specimens should be obtained to mea-
sure electrolytes (Na, K, glucose) and serum levels of gonadotropins LH and FSH,
dihydrotestosterone (DHT), testosterone, 17-hydroxyprogesterone,
17-hydroxypregnenolone, androstendione, and dehydroepiandrosterone (DHEA),
11-deoxycortisol. Assay for MIS should also be performed. Measurement of test-
osterone levels in response to human chorionic gonadatropin (hCG) stimulation is
particularly useful to determine the presence of testicular tissue and distinguish
between hypogonadism or end-organ unresponsiveness. Blood should be sent for
leukocyte culture to obtain karyotype.
Radiologic studies may be useful to define urogenital anatomy and confirm results
from other studies. A genitogram may demonstrate the anatomy of internal ducts.
Magnetic resonance imaging is preferred to ultrasound or computed tomography to
further delineate internal anatomy because of its superior soft-tissue contrast
resolution.
Surgical evaluation of the intersex infant may also include cystoscopy, laparos-
copy, and gonadal biopsy. In cases with a persistent urogenital sinus, cystoscopy can
help determine whether the vagina enters the urogenital sinus proximal or distal to
the external urethral sphincter. Gonadal biopsy is usually necessary to complete the
diagnostic work-up, except in those with congenital adrenal hyperplasia in which
the diagnosis can be obtained from serum markers. Gonadal biopsy should be per-
formed in a longitudinal orientation. Both gonads are biopsied in cases of gonadal
asymmetry. Tissue should be studied to determine androgen receptor levels. Tissue
culture may provide fibroblasts which can be used to determine 5-reductase levels.
Treatment
Infants and children with intersex disorders should be treated as psychosocial
emergencies. Expeditious evaluation and treatment is indicated. After the initial
diagnostic evaluation is completed, sex assignment is determined and surgical
reconstruction is planned. Sex assignment is based upon many factors including:
1. anatomy, 93
2. diagnosis,
3. fertility potential,
4. age at time of diagnosis,
5. gonadal sex,
Table 93.1. Classification and characterization of the common intersex

abnormalities
Category Gonadal Barr Body Karyotype Comment
of Intersex Symmetry
Female Pseudo- Symmetric Present 46 XX CAH, excessive

hermaphroditism androgen present
Male Pseudo- Symmetric Absent 46 XY inadequate
hermaphroditism masculinization
True Hermaph- Asymmetric Present 46 XX, testicular
roditism mosaics and ovarian
tissue coexist
Mixed Gonadal Asymmetric Absent 45 X,46 XY abnormal
Dysgenesis or 46 XY gonads
Fig. 93.1. Marked clitoromegaly (virilization) in an infant female with congenital

adrenal hyperplasia.
6. genetic sex,
7. parental desires.
Anatomic potential is possibly the most important determinant of sex assign-
93 ment. The majority of patients with intersex anomalies will be raised as female. All
female pseudohermaphrodites with CAH are potentially fertile and should be raised
as females if recognized early. All male pseudohermaphrodites with testicular femi-
nization and most of those with partial androgen insensitivity syndrome should be
raised as females. Male pseudohermaphrodites with 5-reductase deficiency have
Intersex 433
normal testes capable of inducing significant masculinization at puberty. These

individuals should be raised as males if recognized early.
Once the decision of sex assignment had been made, external anatomy can be
either feminized or masculinized and discordant hormone production can be stopped.
Early surgical and hormonal therapy are important to developing concordant gen-
der identity. The goal of genitoplasty is to create external genitalia that are, as near
possible, normal in appearance and function. Feminizing genitoplasty is performed
in the neonatal period as soon as gender assignment has been determined; masculin-
izing genitoplasty is usually delayed until 1-2 years of age. Several surgical proce-
dures are available to assist these patients including:
1. reduction clitoroplasty,
2. labioscrotal reduction,
3. low vaginoplasty,
4. flap vaginoplasty or vaginal pull through,
5. perineal reconstruction,
6. hypospadius repair,
7. descent of intraabdominal gonad,
8. removal of retained gonad.
In patients given male sex assignment, discordant ductal structures should be
removed.
Decisions regarding management of the gonads requires consideration of poten-
tial for concordant or disconcordant hormone production/effects, and the risk of
developing subsequent malignancy. The presence of a Y chromosome or HY antigen
dramatically increases the risk of tumor formation. Patients with mixed gonadal
dysgenesis have a 30-50% risk of developing gonadoblastoma; the risk is highest at
and beyond puberty. Wilms’ tumors occur in about 8% of patients with male
pseudohermaphroditism. All streak gonads and testicular tissue that cannot be
brought in to the scrotum should be removed. Streak gonads are at high risk for
developing seminoma and dysgerminoma.
Hormonal and steroid replacement therapy must be monitored carefully to attain
normal secondary sex characteristics and to achieve pubertal growth.
Outcomes
For patients with CAH, immediate diagnosis with correct medical, surgical, and
psychosocial treatment may lead to nearly normal sexual function. There is only
limited information available about the psychosexual development of karyotypic
females raised as males or karyotypic females raised as male. Masculinization or
feminization of the brain is thought to occur very early in development. As adults,
sexual orientation may not remain consistent with sex assignment, particularly when
virilized neonates are assigned and raised as females.
Selected Readings 93
1. Hughes IA. Intersex. In: Freeman NV et al, eds. Surgery of the newborn. New
York: Churchill Livingstone 1994; 781-789.
2. Donahoe PK, Schnitzer JJ. Evaluation of the infant who has ambiguous genitalia,
the principles of operative management. Semin Pediatr Surg 1996; 5:1.
3. Donahoe PK, Schnitzer JJ. Ambiguous genitalia in the newborn. In: O’Neill JA et
al, eds. Pediatric Surgery, 5th edition. St. Louis: Mosby 1998; 1797-1817.
Section XV: Miscellaneous Pediatric
Surgical Topics
CHAPTER 1
CHAPTER 94
Short Bowel Syndrome

Short bowel syndrome (SBS) is a clinical condition in which the surface area of
the small bowel is inadequate for the absorption of sufficient nutrients. It most
commonly occurs as a result of massive small bowel resection. While preservation of
the ileocecal valve may allow a larger resection without developing clinical features
of small bowel syndrome, SBS has occurred following resections of as little as 40%
of the small intestine, but most cases occur when more than 50% of the patient’s
total bowel length has been lost.
The true incidence of SBS is unknown. The main conditions that result in SBS
in infants and young children include:
1. atresia (32%),
2. volvulus (29%),
3. necrotizing enterocolitis (19%), gastroschisis (12%), and other condi-
tions requiring massive bowel resection (7%). In older children, the pri-
mary causes of SBS are midgut volvulus and Crohn’s Disease.
Pathophysiology
Because intestinal mucosal function is site specific, resection of different seg-
ments leads to an array of different problems. Jejunal mucosa secretes cholecystoki-
nin (CCK) and secretin; its brush border is rich in carbohydrate digesting enzymes.
The jejunum absorbs calcium, magnesium, and iron. The ileum absorbs carbohy-
drates, proteins, water, and electrolytes and is the primary absorption site of bile
acids (i.e., enterohepatic circulation), vitamin B12, and fat-soluble vitamins (i.e., A,
D, E, and K). The major functions of the colon are to absorb water and sodium. The
colon excretes potassium and bicarbonate. As a result, combined resection of small
bowel and colon leads to greater water loss, dehydration, hypokalemia, hypo-
magnesemia, and hyponatremia similar to that observed in patients with end jejun-
ostomies. The major consequences of small bowel resection are listed in Table 94.1.
Following small bowel resection, nutrients within the gut lumen stimulate trophic
hormone production (Table 94.2), stimulate release of trophic pancreatic and bil-
iary secretions, and provide a direct source of nutrients to the enterocytes. As the
number of enterocytes increases, villous hypertrophy occurs and the small bowel
Table 94.1. Consequences of massive small intestinal resection

Dehydration proportional to length resected-especially if colon is resected
Nutrient Malabsorption: fat soluble vitamin deficiency (A, D, E, K), starch,
disaccharides, iron, folate, B12, Zinc
Fat malabsorption: steatorrhea, impaired absorption of Calcium, Magnesium, and
Zinc
Electrolyte loss: Sodium, Potassium, Calcium, Magnesium, Zinc
Bile Acid Malabsorption
Bacterial overgrowth of remaining small bowel, especially with ileocecal
resection.
Gastric Acid Hypersecretion, usually not seen unless there is a > 66% resection
D-Lactic Acidosis caused by bacterial fermentation of lactose to lactate
Nephrolithiasis
Cholelithiasis
TPN complications: both hepatic and catheter-related
Table 94.2. Stimulants for reactive intestinal hypertrophy

Glutamine Preferred fuel for small bowel mucosal cells
Butyrate Preferred fuel for colonic mucosa
Hormones enteroglucagon, bombesin, epidermal growth factor,
and stimulants glucocorticoids, prostaglandin E2, ornithine decarboxylase
dilates and lengthens. This compensatory response does not occur if nutrients are
not present in the gut lumen.
In general, the loss of a portion of the jejunum is better tolerated than a similar
loss of ileum although the ileum responds with more villous hyperplasia than does
the jejunum. The permeability of intracellular tight junctions in the ileum is less,
allowing for an increased ability to concentrate lumenal contents. In addition, the
ileum and ascending colon are much better at absorbing sodium chloride against a
gradient than are the other segments of bowel. However, resection of the ileum leads
to decreased reabsorption of bile salts and loss of the ileocecal valve allows bacterial
overgrowth within the small intestine.
The possibility of SBS is considered whenever an infant or child has undergone
94 a major intestinal resection. The first clinical indicator of SBS is frequently diarrhea.
Electrolyte instability commonly follows, as do fatty acid and vitamin deficiencies,
weight loss and growth retardation. The diagnosis is mostly clinical.
Short Bowel Syndrome 437
As a general guideline, the total small bowel length in an individual is approxi-

mately equal to 3-5 times the height of the individual. When estimating bowel
length following resections performed on premature or low birth weight infants, the
gestational age is considered because the intestine lengthens significantly over the
last 17 weeks of gestation (Fig. 94.1). In theory, it is possible for the intestine in a
30-week premature infant to double in length by the time the infant reaches 40
weeks of gestational age.
Initial Treatment
Nutrients are required in the intestinal lumen to decrease mucosal atrophy and
stimulate reactive hyperplasia. The amino acid glutamine is directly used by the
enterocytes so its introduction enterally helps preserve mucosal mass. Serum levels
of Vitamins A, D, E, and K are measured and supplemented as necessary. Bacterial
overgrowth is treated by periodic courses of oral antibiotics (i.e., metronidazole,
vancomycin, trimethoprim/sulfamethoxizole, or ciprofloxacin). Encephalopathy and
lactic acidosis are caused by elevated serum levels of D-Lactate and also respond to
oral antibiotic therapy.
Intravenous nutrition is required to supplement calories while the intestinal
mucosa hypertrophies and adapts. Enteral feeds are introduced and advanced slowly
and methodically to deliver maximally tolerated enteral calories and protein. Tight
control of osmolality, volume, and composition is maintained. Peptides are the major
protein source and glucose polymers are well tolerated as the initial carbohydrate
source. Long chain triglycerides and short chain fatty acids are added gradually and
are also trophic to the mucosa. Medium chain triglycerides (MCTs) are directly
absorbed and do not require lipase or bile acids for absorption. Unfortunately, MCTs
are not trophic for the gut. Later, fiber (pectin) is gradually added as are Vitamins
B12, E, D, and A.
Additional therapies include histamine-2 (H2) receptor antagonists to control
gastric acid hypersecretion, somatostatin to reduce pancreatic and biliary secretions,
loperamide to slow gut motility, and cholestyramine to decrease diarrhea from bile
salt malabsorption. Cholestyramine does not improve steatorrhea, or diarrhea that
results from fat malabsorption. Growth hormone (GH) may also play a role in hy-
pertrophy and GH supplementation is being evaluated as an aid to recovery. The
goal of therapy is to improve bowel recovery and function so that the intestine is
eventually capable of absorbing required nutrients and the patient is no longer
dependent on parenteral nutrition
Late Treatment
After optimal medical therapy for 2-3 years, the intestine adapts to its full poten-
tial. If nutrient malabsorption remains, multiple surgical options are available but
most have had limited success. The Bianchi procedure is perhaps the most success-
ful method used to increase intestinal length. In this procedure, the vessels supply-
ing each side of the bowel are separated into two leaves right at the mesenteric
border. The bowel is divided longitudinally into two halves, each supplied by one
set of the mesenteric vessels. The two segments of bowel are reconstructed as tubes 94
Fig. 94.1. Intestinal length by gestational age.
and then anastomosed end-to-end, effectively doubling the length of the intestine.
Although this procedure has gained both successes and supporters, enthusiasm for it
has waned in the era of small bowel transplantation. Yet, small bowel transplanta-
tion continues to have high mortality and morbidity. Overall, the results of pediat-
ric small bowel transplantation are improving with recent advances in antirejection
therapy.
Outcomes
An overall survival of 82% is reported for patients with SBS. Of these, 62%
adapt to survive on enteral nutrition alone. Patients surviving and adapting with an
ileocecal valve are reported to have an average of 18.5 cm of small intestine (range
10-35 cm). Patients surviving and adapting without an ileocecal valve in place are
reported to have an average of 19.4 cm of small intestine (range 10-25 cm). The
ability to achieve adaptation may be independent of the presence of the ileocecal
valve.
Selected Readings
1. Bianchi A. Intestinal loop lengthening—a technique for increasing small intestinal
length. J Pediatr Surg 1980; 15:145-151.
2. Touloukian RJ, Smith GJ. Normal intestinal length in preterm infants. J Pediatr
Surg 1983; 18:720-723.
3. Grosfeld JL, Rescorla FJ, West KW. Short bowel syndrome in infancy and child-
hood: analysis of survival in 60 patients. Am J Surg 1988; 151:41-46.
4. Collins JB, Georgeson KE, Vicente Y. Short bowel syndrome. Sem Pediatr Surg
94 1995; 4:60-72.
CHAPTER 1
CHAPTER 95
Conjoined Twins
Robert M. Arensman
Incidence
Twinning is reasonably rare and conjoined twining is extremely rare. The exact
incidence is not completely known. Based on various demographic reports, occur-
rence varies from 1: 25,000 to 1: 200,000 births, but the latter figure more accu-
rately reflects live born conjoined twins since there is a high rate of stillbirth (>60%).
Interestingly, about 75% of conjoined twins are female.
Etiology
The stimulus to incomplete twinning is not known, but these twins appear to be
monozygotic, monchorionic twins who fail to make complete separation in the
blastula stage of embryonic development (12-16 days after fertilization). Very rarely,
conjoined twins may result from fusion of separate embryos. In the few documented
cases, one of the fused embryos appears to be a parasitic appendage on the other.
Classification
Conjoined twins are classified based on the site of union. The descriptive names
are derivations of the Greek work “pagos” which means “that which is fixed.”
Many variations on these standard types exist, including the variation known as
“two headed monster,” a particularly unfortunate group with two heads and upper
spines that quickly fuse into one body with two arms, two legs and a common lower
spine. Generally each head controls half of the body so each twin controls only one
arm and leg.
These general types can be further described to indicate number of limbs. One
often sees words such as tripus (three legs) or tetrapus (four legs) appended to the
major descriptor; hence a description such as “ischiopagus tetrapus” describes a pair
of conjoined twins joined at the pelvis with a combined total of 4 lower extremities.
The condition is apparent at birth. Simple inspection confirms the diagnosis
and generally allows for broad classification. Diagnostic studies are chosen to iden-
tify and document the exact type and number of anomalies. This process allows
final classification and formulation of a plan for surgical separation if possible.
Table 1. Classification of conjoined twins

Type Site of Union Relative Incidence
Thoracopagus Chest 40%

Omphalopagus Abdomen 30%
Pyopagus Sacrum 20%
Ischiopagus Pelvis 5%
Craniopagus Head 2%
Diagnosis
Ultrasonographic studies document number of hearts, union of hearts, intracar-
diac anomalies, genitourinary anomalies, and gynecologic anomalies. Computed
tomography and contrast studies are extensively used to locate organs, study spinal
and cranial unions, and sort out gastrointestinal anatomy. Portions of the gastrointes-
tinal tract are often combined or shared by both twins.
Preoperative evaluation and documentation to the greatest extent possible allows
good surgical planning. It also allows realistic counseling with parents regarding
chance for survival, life-long disabilities, and quality of life.
Treatment
In the past, surgical separation has usually been attempted with the hope of
improving quality of life. However, surgical separation results in 30-40% mortality
of one or both twins. If the surgical separation becomes emergent, mortality in-
creases to 70%. At present, longer periods of observation and evaluation are com-
mon, and in some cases families have rejected surgery when risk of surgery is high or
sacrifice of one twin is certain. Survival in the conjoined state is possible with nor-
mal life expectancy. Furthermore, thoracopagus twins with a conjoined heart(s) have
never been separated successfully (i.e., no single survivors).
Outcomes
With careful preoperative evaluation and conscientious intraoperative and pe-
rioperative care, up to 60% of conjoined twins can be successfully separated and
approximately 60% of these separated twins will survive and have reasonably suc-
cessful lives. The survival group includes many children who have life-long ostomies
for urinary or gastrointestinal diversion, have artificial extremities, and require re-
petitive surgeries to correct ongoing cosmetic or functional defects. Nevertheless,
this situation is far from hopeless. Each set of conjoined twins should be carefully
studied and evaluated to provide the best long-term care available.
95
Conjoined Twins 441
Fig. 95.1. Conjoined twins with complete union below the high thoracic level.
These twins share a single conjoined heart and have fusion of the spine posteriorly.
Separation of this type of union is currently not feasible.
95
Selected Readings
1. Hoyle RM: Surgical Separation of Conjoined Twins. Surg Gyn Obst 170: 549-
562, 1990.
2. Wilcox DT, Quinn FM, Spitz L et al. Urological problems in conjoined twins. Brit
J Uro 81(6): 905-910, 1998.
3. Cywes S, Millar AJ, Rode H et al. Conjoined twins—the Cape Town experience.
Ped Surg Int 12(4): 234-248, 1997.
4. Spencer R. Conjoined twins: theoretical embryologic basis. Teratology 45(6): 591-
602, 1992 (25 references).
95
CHAPTER 1
CHAPTER 96
Minimally Invasive Pediatric Surgery

In the early 1970s, pediatric surgeons first used peritoneoscopy and thoracos-
copy. Subsequently, the recent development of improved monitors, telescopes, and
miniaturization of instruments has created explosive growth in the use of laparo-
scopic procedures in infants and children. The reported advantages of the laparo-
scopic technique are better operative exposure, faster patient recovery, and shorter
hospital stay; however, the disadvantages include lack of tactile sensation, inconclu-
sive long-term results, and increased operative time.
Indications
Commonly performed laparoscopic pediatric procedures include diagnostic lap-
aroscopy for abdominal pain or trauma, appendectomy, cholecystectomy, pyloromyo-
tomy, fundoplication, gastrostomy, splenectomy, evaluation for contralateral inguinal
hernia, localization of nonpalpable testis, pull-through for Hirschsprung’s disease,
and thoracoscopic lung biopsy or decortication for empyema. Other procedures
that are less frequently performed by laparoscopy include Ladd’s procedure, Meckel’s
diverticulectomy, resection of foregut duplications, staging for cancer, and ligation
of patent ductus arteriosus. In reality, the only absolute contraindications to lap-
aroscopy are those that would mitigate against any surgical procedure, such as
coagulopathy and hemodynamic instability.
Equipment
The development of instruments designed specifically for pediatric patients has
been the most significant progress in pediatric endoscopic surgery over the past
several years. Reusable 3-mm trocars are economical and associated with decreased
postoperative pain; however, frequent trafficking of instruments may lead to unin-
tentional dislodgement. The trocars can be secured to the abdominal wall using a
1cm sleeve of an 18 Fr. rubber catheter over the shaft of the trocar, which is then
sutured to the skin. Larger 5-mm expandable trocars (Innerdyne®) are very useful
in maintaining a tight seal with the surrounding tissue and can accommodate larger
instruments such as harmonic scalpel or retractors.
The Hopkins rod lens telescopes come in a variety of sizes, lengths, and lens
angles. The 0˚ scopes provide the least amount of disorientation and are easy to use
for simple procedures. The 30˚ scopes are more useful for advanced laparoscopic
procedures and in some situations provide better exposure of the viscera. The most
commonly used scope size is 5mm, but smaller diameter (1.2-4 mm) scopes with
excellent lighting and optics are available for smaller patients. Three-chip cameras
produce superior images and advances in digital cameras will continue to improve
96 the image quality of the operative fields.
Instruments with smaller diameters (1.7-3 mm), as well as shorter shafts, are
available in various styles of tips and handles. Advances in product technology have
created miniaturization of instruments without the sacrifice of feel and durability.
Sutures also come in a variety of size and shape: ski-needles are easy to pass through
the trocars and to manipulate by retaining some of the mechanical advantages of
curved needles. Development of the harmonic scalpel and endoscopic ultrasound
has also contributed significantly to recent progress in laparoscopy. The harmonic
scalpel, which is now available in 5 mm size, can coagulate vessels up to a few milli-
meters in diameter and allows easier and safer dissection of vascular tissues. Diag-
nostic endoscopic ultrasound is useful in defining vascular anatomy and identifying
lesions in the parenchyma of solid organs. It may occasionally be useful in laparo-
scopic staging cancer.
General Considerations
General patient preparation for operations do not change because of the use of
minimally invasive surgical techniques. Operating room personnel, as well as the
patient’s family, are prepared for potential conversion of any laparoscopic procedure
to an open case. The primary surgeon stands facing the patient with the operative
area in line with the video monitor. A Foley catheter is placed only for anticipated
lengthy procedures, otherwise the bladder is emptied by the Crede’ maneuver. The
stomach is decompressed by orogastric suction. Prophylactic antibiotics are consid-
ered to prevent trocar site infection, and fascial defects resulting from 5-mm or
larger trocars are closed with sutures to prevent incisional hernias.
Two important considerations in pediatric laparoscopy are:
1. abdominal cavities are smaller in size and
2. the abdominal wall is much more elastic and thinner.
It is easier to injure abdominal viscera during trocar placement in pediatric patients;
therefore, some authors favor open technique for trocar placement. However, with
diligence and a careful approach, trocars can be placed safely using closed technique,
even in the smallest infants. The umbilicus is an ideal initial trocar site for tele-
scopes. Especially in infants, the normal small fascial defects at the umbilicus can be
easily enlarged to enter the peritoneal cavity for subsequent insufflation without the
need for Veress needle placement. All trocars are placed under direct visualization.
Large instruments are maneuvered under visual guidance as they can easily injure
viscera.
General anesthetics are used in pediatric patients undergoing minimally invasive
surgery. Caudal blocks and infiltration of long-acting local anesthetics at the trocar
sites are helpful in providing postoperative analgesia. Absorption of CO2 and an
increase in abdominal pressure due to insufflation may present physiologic chal-
lenges. Visceral absorption of CO2 may lead to hypercapnia requiring an increase in
minute ventilation, particularly in infants with chronic lung disease such as bron-
chopulmonary dysplasia. Abdominal insufflation results in increased intra-abdomi-
nal pressure that may cause a decrease in lung compliance and tidal volume. Venous
return to the heart is sometimes decreased compromising cardiovascular function.
Minimally Invasive Pediatric Surgery 445
Older children can tolerate 14 mmHg insufflation pressures, while smaller infants
require pressure ranging from 8-12 mmHg.
Appendectomy
96
Many centers now routinely use laparoscopic appendectomy. The benefits of less
postoperative pain and faster return to full activity are reported but unproven. Lap-
aroscopic appendectomy is particularly beneficial in cases of obese patients as well as
teenage girls with abdominal pain in whom the diagnosis of appendicitis is ques-
tionable. Laparoscopy allows thorough examination of the entire peritoneal cavity
to search for pathology when a normal appendix is encountered.
The operating surgeon stands on the patient’s left and the umbilical trocar is
initially placed for the telescope. Two additional trocars are placed, one in the left
lower quadrant and the other in a midline suprapubic location. Trendelenburg posi-
tion is used to gravity-retract the small bowel from the right lower quadrant for
better exposure of the cecum and pelvis. The mesoappendix is divided using cautery
or hemoclips depending on vessel size. The appendix is ligated using a pretied
endoloop. Alternatively, an endostapler can divide the appendix and mesoappendix
simultaneously. The specimen is most easily delivered through the umbilical 10-mm
port. This route may reduce the incidence of wound infection. Occasionally, if the
thickened inflamed appendix cannot be removed through the trocar, an endoscopic
retrieval bag is used. In cases of ruptured appendicitis, the fecalith is sought and
removed if possible and irrigation of the abdominal cavity considered. Although the
subcutaneous wound infection rate is much lower with laparoscopic approach, the
incidence of postoperative abscess is similar to open cases.
Fundoplication and Gastrostomy
Laparoscopic fundoplication is now a common technique for antireflux proce-
dures. The indications and diagnostic work-up for laparoscopic fundoplications are
the same as those for open procedures. Occasionally, a preoperative enema is neces-
sary to ensure the colon is decompressed. A distended transverse colon, especially in
small infants, can limit the operative exposure making the procedure more difficult.
The bladder is decompressed by the Credé maneuver and an esophageal dilator is
placed. The patient is placed at the foot end of the table, with the lower extremities
taped in a cross-legged position or, in the case of older children (weight > 20 kg),
supported in stirrups. The patient is placed in reverse Trendelenburg with the left
side raised slightly, providing gravity retraction of the viscera away from the upper
abdomen.
Generally, five trocars are used, and in small infants, they should be placed lower
in the abdomen to create a longer working space and thus allow better maneuver-
ability. Use of a 30˚ or 45˚ angle scope is important to facilitate adequate operative
exposure. First, dissection is completed to create proper intra-abdominal esophageal
length. The short gastric vessels are divided with cautery, hemoclips, or harmonic
scalpel depending on vessel size. A complete 360˚ wrap (Nissen) is the most com-
mon fundoplication performed by laparoscopy, but partial fundoplications (Toupet
and Thal) can also be performed. The decision algorithm for which type of wrap to
create should be the same as in open cases.
Gastrostomy is placed in indicated patients. A site on the anterior stomach is

grasped and pulled up. Two U-stitches are placed through the abdominal wall to
96 suspend the anterior wall of the stomach. Using the Seldinger technique, a needle is
introduced through the abdominal and anterior stomach wall. After placing a guide
wire, graduated dilators (8 to 20 Fr.) are used to enlarge the tract, and a MicKey®
gastrostomy button is placed. Postoperatively, the gastrostomy tube is placed to gravity
drainage overnight and then small amounts of enteral feeding are started the next
day. The feeding volume is then gradually increased to target over 2-3 days. Patients
are discharged from hospital by postoperative day 2.
Splenectomy
The harmonic scalpel and endoscopic stapling devices have facilitated the devel-
opment of laparoscopic splenectomy. The major indications for laparoscopic sple-
nectomy are hematologic disorders such as hereditary spherocytosis and idiopathic
thrombocytopenic purpura. Other conditions that occasionally require splenectomy
include sickle cell disease, β-thalassemia and Hodgkin’s disease. All patients receive
preoperative vaccinations against Pneumococcus, H. influenzae, and Meningococcus.
Proper patient positioning is instrumental to this operation, and most surgeons
prefer the lateral approach. The left side is elevated approximately 45˚ with a bean
bag or gel roll. For trocar placement, the table is tilted to the patient’s left side, and
for splenectomy, the table is tilted to the right so that the patient is in near lateral
decubitus position. The scope is placed through the umbilical trocar, and three
additional ports are usually necessary. First, the splenocolic and gastrosplenic liga-
ments are divided with cautery, and then a careful search is made for accessory
spleen. The reported incidence of missed accessory spleen is variable but similar to
open cases, ranging up to 20%. The short gastric vessels are divided using cautery or
a harmonic scalpel. Maintaining phrenic attachments at the superior pole allow
easier maneuvering of the spleen during hilar dissection. Then, using the stapling
device via a 12-mm port, the hilar vessels are carefully divided to avoid injury to the
tail of the pancreas. An endobag is introduced directly through the largest trocar
fascial defect, and the spleen is guided into the bag. Placing the spleen into the bag
can at times be the most difficult part of the operation, especially in small patients.
The neck of the sac is exteriorized and the spleen is fractured with morcellator,
surgical clamps or surgeon’s finger. Operating time has consistently been longer for
laparoscopic approach, but it has been shown to be a safe alternative to open
splenectomy.
Pyloromyotomy
Despite some enthusiasm for less postoperative ileus and improved cosmesis, the
benefits of laparoscopic pyloromyotomy remain unknown. A single trocar is placed
at the umbilicus for insufflation and 3-mm telescope. Two stab wounds are created
with 11-scalpel blade for 3-mm instruments without the need for trocars. Left-hand
grasper exposes the pylorus, and then the pyloromyotomy incision is made with a
retractile arthrotomy knife. A pyloric spreader is used to perform the myotomy until
the two sides of the split pyloric tumor move independently. Careful inspection for
absence of mucosal injury is confirmed, and then the umbilical fascia is closed.
Postoperative feedings can be started within 2-4 hours. Reported series demonstrate
an average operating time of 25 minutes with 3% perforation and 0.8% inadequate

myotomy rates.
Contralateral Inguinal Exploration
96
Routine contralateral inguinal exploration at the time of symptomatic hernia
repair for infants is the standard practice based on the high incidence of contralat-
eral patent processus vaginalis (4-65%). However, the potential complications of
injury to the vas deferens (1.6%) and testicular atrophy (2%) exist, even in the
hands of experienced pediatric surgeons. Although there are numerous diagnostic
methods (e.g., ultrasound evaluation of the inguinal canal or simple insufflation of
the abdominal cavity) for determining the presence of contralateral inguinal hernia,
laparoscopic inspection is the most simple and reliable method.
The symptomatic hernia sac is dissected to the level of the internal ring, and a
3-mm trocar is introduced into the peritoneal cavity. After insufflation, a 70˚ tele-
scope is placed to inspect the patency of the contralateral processus vaginalis. Modi-
fication of this technique by insufflating the abdomen via the hernia sac on the
symptomatic side and then placing 1.2-mm scope through a 14-gauge angiocatheter
in line with the contralateral internal inguinal ring has resulted in more accurate
assessment of patent processus vaginalis. Once the presence of a contralateral hernia
is determined, it is repaired in standard fashion by high ligation of the sac. Recently,
several authors have even performed transperitoneal laparoscopic hernia repairs.
Several reported series show no surgical complications related to laparoscopy
with false negative rate of less than 1%. Authors consistently emphasize that the
laparoscopic contralateral exploration is the most accurate means to determine the
patency of processus vaginalis.
Nonpalpable Testis
When a testis is not palpable on thorough inguinal exam after induction of gen-
eral anesthesia, diagnostic laparoscopy is performed via a small umbilical trocar. If
testicular vessels are atrophic and end blindly before the internal inguinal opening,
the procedure is stopped due to absence or nonviable testis. However, if normal
appearing vessels pass into the inguinal canal or a testis is seen at the orifice, inguinal
exploration and orchidopexy is indicated. In cases where the testis is primarily located
in the abdominal area, the testicular vessels are not of sufficient length to allow
primary orchidopexy. The two-stage Fowler-Stephens approach is the most popular
technique used for intra-abdominal testis. Ligation of the testicular vessels is per-
formed laparoscopically, and then followed by inguinal exploration and orchidopexy
months later when collateral vascularity of the testis is achieved. Many authors report
an excellent outcome without significant complications using this technique. The
average operating time is less than 10 minutes. Recently, some authors have advo-
cated single-stage laparoscopic orchidopexy with promising initial results.
Pull-Through for Hirschsprung’s Disease
Traditionally, Hirschsprung’s disease has been surgically treated in two or three
stages; leveling colostomy followed by a colonic pull-through with either simulta-
neous or subsequent colostomy closure. One-stage pull-through has also been advo-
cated for infants with satisfactory results. In addition, laparoscopy-assisted single-stage
colonic endorectal pull-through for Hirschsprung’s disease has recently been

performed at many centers with an excellent outcome. The enthusiasm for the lap-
96 aroscopic approach stems from a hope for decreased morbidity along with equiva-
lent functional results compared to open cases. Infants do not require diverting
colostomy, and they may have an opportunity to achieve normal fecal control at an
earlier age.
All patients undergo the same diagnostic work-up for Hirschsprung’s disease as
in open operation. Patients with severe enterocolitis are generally excluded for the
laparoscopic approach since these children seem to benefit from a period of diver-
sion and colon rest prior to reconstruction. Frequent preoperative rectal irrigation
with saline is helpful to decompress the colon. Infants are positioned transversely on
the operating table and are prepped entirely below the nipple line. The initial trocar
is placed just below the liver margin for a small angled scope (3-5 mm). Two work-
ing ports of 3 or 5 mm are placed in the left upper and right lower quadrants. An
additional suprapubic trocar is used in some patients for retraction. The transition
zone is identified grossly and seromuscular biopsy is obtained with scissors to con-
firm the presence of ganglionic cells. Mesenteric dissection is accomplished with
cautery in small infants, but requires hemoclips or harmonic scalpel in older chil-
dren with larger vessels. Preservation of the marginal artery is vital to this portion of
the operation. The aganglionic segment of distal colon is dissected circumferentially.
Perineal endorectal dissection is started at 1-2 cm above the dentate line after plac-
ing several traction sutures to evert the anus and rectum. The submucosal plane is
developed with cautery and dissection is continued proximally using blunt and sharp
dissection until the colorectum turns inside out. The rectal sleeve is then opened
and a full-thickness specimen is sent for frozen section for additional confirmation
of ganglion cells at the proximal margin. After posterior myotomy, coloanal anasto-
mosis is completed with interrupted absorbable sutures. Oral feeding is started on
postoperative day 1, and the average length of hospital stay is 2-3 days. Reported
experiences of 80 patients from several centers demonstrate an average operating
time of 147 minutes with minimal blood loss. Only two patients required conver-
sion to open procedures, and enterocolitis and chronic diarrhea were each noted in
8% of the patient group. Despite the lack of long-term results, laparoscopy-assisted
colonic endorectal pull-through for Hirschsprung’s disease appears to be a satisfac-
tory technique.
Thoracoscopy
A wide variety of conditions are amenable to thoracoscopic approach. Biopsy of
lung nodules and early drainage of empyema are commonly performed thoraco-
scopic procedures. Access to mediastinal masses is also easily achieved
thoracoscopically, and resection without a large painful thoracotomy incision can
be accomplished. Other recent innovative applications of minimally invasive tech-
niques in thoracic surgery include ligation of patent ductus arteriosus (PDA) and
anterior spinal fusion for severe scoliosis. Single lung ventilation is generally diffi-
cult to achieve in small patients weighing less than 30 kg due to the lack of a small
sized double-lumen endotracheal tube. However, selective mainstem intubation of
the contralateral bronchus or use of a bronchial blocker can be of great help in
maintaining operative exposure. A low-pressure (3-5 mm Hg) CO2 infusion can

also assist in keeping the lung decompressed.
Summary
96
There now exist extensive applications of the laparoscopic technique in infants
and children. The list of operations appears to be getting longer as we witness the
development of improved technology. However, one must exercise caution in utiliz-
ing these innovative techniques and be aware of potential complications associated
with each procedure. The benefits of minimally invasive techniques must be care-
fully compared to the potential increased cost, operating time, and lack of long-
term results. Most importantly, laparoscopic surgery should be regarded in terms of
applying minimally invasive techniques to perform the same operations as in open
approach.
Selected Readings
1. Georgeson KE. Minimally invasive pediatric surgery: Current Status. Semin Pediatr
Surg 1998 (series); 7(4):193-238.
2. Lobe TE. Laparoscopic surgery in children. Curr Prob Surg 1998; 35(10):859-950.
3. Holcomb GW III: Laparoscopic Pediatric and Fetal Surgery. Semin Lap Surg 1998
(series); 5(1):1-66.
Index 459
INDEX
A Bilious emesis 251, 269

Bites 115, 150-152, 161
Aberrant pulmonary artery 312, 314 Bladder rupture 130, 133
Index
Abscess 45, 46 Blood donation 4
Abuse 120, 123, 129, 135, 144, 148, Blunt abdominal trauma 128, 130,
150, 159-162 132
Acalculous cholecystitis 396, 398 Bochdalek (Foramen of ) 325, 327
Achalasia 380-382 Brain injury 110, 123, 125, 136
Adenoma 420-422, 424, 425, 427 Branchial arches 69
Adenomatous polyps 234 Branchial remnant 69-72
Adhesions 393, 394 Breast 206-209, 212, 220
Airway 310-313, 315, 320, 330, 337, Bronchogenic cyst 185, 201, 202,
341, 343, 349, 377, 384 204, 205, 311, 326, 333, 335,
Alpha interferon 68 337, 338
Amniocentesis 10-12 Bronchoscopy 312, 377
Anal fissure 100, 101, 104 Burkitt’s lymphoma 215
Anemia 3, 9, 10 Burns 144-149, 161, 162
Ann Arbor staging 212, 213
Annular pancreas 395, 411 C
Anorectal malformation 366, 367
Antibiotic therapy 40, 46-48 Calories 24, 25, 27, 30
Anus 91, 100, 101, 103-105, 107 Cantrell (pentalogy of ) 362
Aortomesenteric 400 Carcinoid 198
Apnea 310, 377 Caroli’s disease 307
Appendicitis 246, 388-392 Cat bite 150, 151
Apple peel lesion 265 Catecholamine 171, 172, 415-419
ARDS 31 Caustic esophageal injury 383
Arteriography 142 Cephalohematoma 153, 154
Ascites 286, 287, 290, 295-298 Cerebral perfusion 35
Aspiration 312, 320, 322, 339, 341, Chagas’ disease 380
349, 377, 381 CHARGE association 321
Asplenia 266 Chloride channel 268
Atresia 250-252, 254, 255, 257-259, Cholangiocarcinoma 305
261, 264-267, 269-271, 276, Cholecystitis 238, 389, 396-398
277, 396, 411 Choledochal cyst 301, 304, 305, 307,
308
B Cholelithiasis 389, 395, 397, 398,
405
Barium esophagram 315, 385 Chorionic villus sampling 11
Battery ingestion 82 Chylothorax 344-346, 348, 351, 354
Beckwith-Wiedemann syndrome 164, Chylous ascite 295, 297, 298
363, 426 Circumcision 63, 64
Bell-Clapper deformity 57 Clear cell 164, 165, 167
Biliary atresia 241-244, 300-302, 396, Cloaca 366, 367, 370, 372
411 Cloacal exstrophy 362, 372
Coins 79, 80, 84 Emesis 85, 86, 88
Colon atresia 269 Empyema 348-351
Colon carcinoma 403 Endodermal sinus 189, 190, 194
Compartment syndrome 142, 143, Enteral nutrition 23, 30
145 Epidural hematoma 124, 125, 154
Congenital adrenal hyperplasia 428, Esophageal atresia 311, 318-323, 377
Index
429, 431, 433 Esophageal perforation 339, 341, 348,

Congenital cystic adenomatoid 381, 384-386
malformation (CCAM) 335, Esophagitis 376-378, 381
337 Esophagoscopy 312, 378, 381, 384
Congenital diaphragmatic hernia 325, Eventration 325-327, 330, 331
327, 331, 332, 335, 344, 349 Exchange transfusion 10
Congenital hemangioma 65 Extracorporeal membrane oxygenation
Congenital lobar emphysema 315, (ECMO) 27, 34, 330, 331
333, 335 Extremity trauma 115
Conjoined twin 439, 440 Extubation 5
Constipation 99-102, 104, 107, 371
Corticosteroids 68, 96 F
Craniofacial ratio 121
Crohn’s disease 389, 391, 402-405, Facial nerve 69-71
407, 410 Facial trauma 110, 120, 121
Croup 315 Fecalith 246, 388, 390, 392
Cryoprecipitate 39, 40 Female pseudohermaphroditism
Cryotherapy 68 428-430
Cryptorchidism 56, 57, 60-62 Femoral hernia 50, 54
Cushing ulcer 237 Fertility 56, 61, 62
Cystic fibrosis 241, 266-271, 390, Fetal surgery 11, 13, 14
396-398, 409 Fibroadenoma 208, 209
Cystography 130, 133 Fissure 229, 230
Fistula-in-ano 105, 106
D Fistulas 69-71, 77
Fluid imbalance 20
Dehydration 19, 22, 29, 85, 86, 100 Fluid requirements 20, 23
Diagnostic studies 3, 11 Fluid resuscitation 112, 130, 145
Dislocation 116, 126, 141, 142, 155 Fluids 21, 22, 37
Dog bite 150 Follicular carcinoma 226
Double aortic arch 314 Follicular cyst 188, 189
Ductus arteriosus 314, 330, 352-354 Foramen cecum 72
Duodenal hematoma 162 Foreign bodies 79-82, 84
Duodenal web 259 Fundoplication 379
Duodenoduodenostomy 259, 411
Duplications 278, 279, 280, 281, G
283, 395
Ganglioneuroblastoma 169, 170
E Gastric teratoma 197, 198
Gastritis 230, 237-239
Electrolytes 19-21, 24 Gastroesophageal reflux 315, 320,
Embryonal sarcoma 177, 178, 180, 322, 323, 331, 376-379, 381
181, 221 Gastroschisis 361-363, 365
Index 461
Germ cell tumor 189, 190, 192, 194, Incarceration 51, 52

201-203, 205 Inguinal hernia 50-52
Glasgow coma scale 110, 112, 123, Insulinoma 412
124 Intersex 428-432
Greenstick fracture 117, 121 Intestinal injury 131
Gross-Vogt classification 318, 322 Intestinal obstruction 232, 246, 248,
Index
Growth retardation 403-405 250-253, 268, 269, 271, 279,
Gynecomastia 193, 207, 208 280, 393, 411
Intra-abdominal testicle 61, 62
H Intraosseous 17, 37
Intravenous access 17, 37
Head injury 112, 113, 123, 124, 136, Intussusception 89-93, 102, 103, 230,
139, 160 232, 235, 246-248, 389, 390,
Helicobacter pylori 237, 238, 248 393, 394
Hemangioendothelioma 177, 178, Ischiopagus 439, 440
180, 181 Islet cell adenoma 425
Hemangioma 65-68, 70, 97
Hematuria 132, 133 J
Hemorrhage 111, 112, 115, 132,
137-139, 142, 150, 153-156, Jaundice 300-302, 304
160-162, 229, 231, 238, 239, Jejunal atresia 250, 251, 265-267, 269
242, 243, 246, 397, 404 Joint injuries 116
Hemorrhoids 106, 107 Juvenile polyps 232, 233, 235, 246
Hemothorax 110, 111, 137, 139
Hepatoblastoma 177-181 K
Hepatocellular carcinoma 177, 178,
180, 181 Kasabach-Merritt syndrome 68, 180
Hereditary spherocytosis 9 Kasai procedure 302
Hirschsprung’s disease 251, 252, 261, Kyphosis 357
265-267, 269, 270, 272-274,
276, 277, 390
L
Hodgkin’s disease 202, 205, 209, Ladd’s procedure 261, 262, 264, 393
211-214 Laparoscopic cholecystectomy 443
Hydrocele 50-52, 55, 56 Laparoscopic pull-through procedure
Hydrops fetalis 13 443, 447, 448
Hydrostatic reduction 92 Laparoscopic splenectomy 446
Hyperinsulinism 424-426 Laparoscopy 431, 443-445, 447, 448
Hypersplenism 94, 97 Large cell lymphoma 215, 216
Hypertrophic pyloric stenosis 251, Lead point 89, 93
254, 255 Leiomyoma 197
Hyperventilation 125 Ligation 330, 335, 344, 348, 354, 355
Hypoparathyroidism 422 Lipids 24
Hypovolemia 35-37 Liquid ventilation 34
Liver biopsy 301
I Lymphoma 184, 185, 194, 197, 198,
Idiopathic thrombocytopenic purpura 201, 209, 211-219, 221
(ITP) 94, 96, 97
Imperforate anus 362, 366, 367,
369-372
M O
Maintenance 20-22, 25, 37 Omphalocele 361-365, 372
Malrotation 251, 254, 255, 257, Omphalomesenteric duct 75-77
260-262, 264, 265, 276, 389, Omphalopagus 440
400 Open fracture 116, 117
Index
Manometry 381, 382 Opsoclonus-myoclonus 171

McBurney’s point 389, 393, 394 Orchidopexy 52, 61, 62, 193
Meckel’s diverticulum 230, 245-248, Ovarian teratoma 184, 185, 190
391, 393, 412 Ovary 184, 189-191
Meconium 250-252, 255, 265, 268, Oxygen 32, 34, 35, 42
269-272, 276
Meconium ileus 250-252, 268-271 P
Mediastinum 185, 201-204, 217, 218
Medullary carcinoma 224, 226 Packed red blood cells 37, 39
Melena 229-232, 246 Pain management 3, 7
MEN syndrome 415, 416, 419, 421, Pancreatic cyst 410
422 Pancreatitis 238, 389, 393, 395,
Mesenteric cyst 281-283, 297 408-411
Mesoblastic nephroma 164, 165, 167, Papillary carcinoma 208, 226
168 Paralysis 110, 115, 126, 141, 155,
Metabolic alkalosis 86 156
Metabolism 23, 35 Parathyroid 420, 422
MIBG scanning 416, 419 Parenteral nutrition 17, 22-24, 27-30
Mixed gonadal dysgenesis 429, 430, Parkland formula 145
433 PCVC 17
Morgagni 325-327, 329-331 Pectus carinatum 359
MRSA 44 Pectus excavatum 357, 359
Myotomy 381, 382 Perforation 287-291, 293-296, 298
Perianal abscess 105, 106
N Pericardial cyst 201, 202, 204
Peritoneal drainage 290, 291
Necrotizing enterocolitis (NEC) 229, Peutz-Jeghers syndrome 199, 235
230, 285-293, 398, 435 Pheochromocytoma 415-417, 419,
Neglect 148, 159, 160, 162 421
Neonatal hepatitis 295, 301 Phimosis 63, 64
Nephroblastoma 164, 167 PIC 17
Nesidioblastosis 424-426 Plasma 29, 39, 40, 42
Neuroblastoma 165, 169-176, 182, Pneumoperitoneum 287, 290, 293,
185, 186 294
Nevus 66 Pneumothorax 110, 111, 136, 137,
Non-Hodgkin’s lymphoma 170, 197, 139, 140, 156, 157
198, 203, 205, 209, 212, 214, Poland’s syndrome 359
215, 218 Polyhydramnios 250, 251, 255, 269
Nonoperative management 129, 140 Polyposis 233-235, 396
Nonrotation 261 Polyps 199, 230, 232-235, 246, 397
Nutrition 22, 23 Polysplenia 266
Port wine stain 66, 68
Portal hypertension 241-243
Index 463
Portoenterostomy 302, 303 Sarcoma 164, 170, 177, 182, 185,

Portosystemic anastomoses 241 194, 209, 214, 221
Posterior sagittal anorectoplasty 367 Scoliosis 357
Postsplenectomy sepsis 97 Scrotum 134, 135
Potassium 21, 22 Seminoma 194, 202, 203, 205
Premature thelarche 206, 207, 212 Sequestration 311, 326, 333, 335,
Index
Prematurity 285, 294 337, 338
Prenatal diagnosis 10, 11 Shaken impact syndrome (SIS) 160,
Prenatal ultrasound 13 161
Preoperative management 3, 6, 10 Shock 35-37, 42
Presurgical visitation 4 Short bowel syndrome (SBS) 435,
Primary survey 109, 110, 113 436, 438
Proctocolectomy 404, 405 Sickle cell 11
Prostaglandin 353 Sinus 69-72, 74, 76, 77
Protein preparations 25, 27, 29, 30, Sistrunk procedure 73, 74
39, 40 Skin grafting 135
Pull-through procedure 405 Skull fracture 123, 125, 153, 154,
Pulmonary artery sling 314, 315 160, 161
Pulmonary contusion 111, 136, Slings 314, 315
138-140 Sodium 20, 22
Pulmonary hypertension 329-331 Spherocytosis 94, 95, 97
Pyloric stenosis 85-87 Spider bite 152
Spleen 94-97
R Splenectomy 94-98
Splenic cysts 97
Rectal biopsy 273, 274 Splenic laceration 139
Rectal bleeding 229-232, 402 Splenomegaly 301
Rectal prolapse 101-104, 106 Stings 152
Rectopexy 104 Stridor 310-312, 314, 315, 384
Rectum 79, 82, 83, 91, 100, 101, 104 Stromal tumor 190, 193, 194
Recurrent hernia 52 Subdural hematoma 124, 125, 154,
Renal injury 132 160
Respiratory distress 310, 311, 320, Superior mesenteric artery syndrome
322, 325, 326, 330, 333, 335, (SMA) 400, 401
346, 348
Resuscitation 36, 37, 41 T
Retinopathy of prematurity 32
Rex shunt 243 Teratoma 165, 182-187, 189, 194,
Rhabdoid 164, 165, 167 202
Rhabdomyosarcoma 170, 194-196, Testicle 50-52, 55, 57-59, 61, 62
209, 220-223 Testicular feminization 428, 429, 432
Riley-Day syndrome 416 Thermal injury 145
Rings 311, 312, 314, 315 Thermoregulation 37
Rye classification 212 Thoracic duct 344, 346, 348
Thoracoscopy 443, 448
S Thoracotomy 315, 323, 335, 349,
351, 354, 381, 385
Saccrococcygeal teratoma 183 Thyroglossal duct 69, 72-74
Salter-Harris 118, 119, 157 Thyroid carcinoma 214
Thyroid scan 74 V
Torsion 57-59, 61
Trace elements 27 VACTERL association 319, 321-323
Tracheoesophageal fistula 311, Varices 230, 239, 242-244
318-323, 339, 344, 385 Varicocele 55, 56
Tracheomalacia 311, 315, 320, 323 Vascular injury 141-143
Index
Transfusion 39-42 Vascular malformation 65-69

Vasoactive intestinal peptide 171
U Venomous snake bite 151
Ventilator support 32
Ulcerative colitis 402-405, 410 Vitamins 23, 26
Ulcers 237-239, 248, 403, 405, 411 Vitelline duct 245-248
Ultrasonography 70, 86, 91 Volvulus 251, 260-262, 264, 268,
Umbilical fistula 246 269, 271, 276, 279, 282, 283
Umbilical hernia 75, 77
Umbilical vessel 17 W
Umbilicus 75, 76, 77
Undescended testes 51 WAGR syndrome 165
Urachal remnant 75-78 Wilms tumor 167
Urachus 75-77 Wind-sock 257
Urine output 24, 25, 36 Wound care 5, 7
Urogenital ridge 60 Wound closure 46, 52
Urogenital sinus 372 Wound infection 43-46, 48
Uropathy 295
Z
Zollinger-Ellison syndrome 237
LANDES LANDES LANDES
BIOSCIENCE V ad e me c u m BIOSCIENCE BIOSCIENCE V ad e me c u m
V ad eme c um
Table of contents
Pediatric
(excerpt)
1. Preoperative Care 12. Inguinal Hernia and Hydrocele

2. Immediate Postoperative Care 13. Variocele
14. Testicular Torsion
Surgery
3. Anemia
4. Genetics and Prenatal 15. Cryptochidism:
Diagnosis in Pediatric Surgery The Undescended Testes (UDT)
5. Vascular Access 16. Circumcision
6. Fluids and Electrolytes 17. Hemangiomas and Vascular
Pediatric Surgery
7. Nutrition and Metabolism Malformations
8. Respiratory Failure 18. Branchial Cysts, Sinuses
and Support in Children and Fistulas
9. Hypovolemic Shock 19. Thyroglossal Duct Cyst
and Resuscitation and Sinus
10. Blood Component Therapy 20. Umbilical Anomalies
11. Perioperative Infections 21. Foreign Bodies
and Antibiotics of the Gastrointestinal Tract
This is one of a new series of medical handbooks.

It includes subjects generally not covered in other handbook series, especially
many technology-driven topics that reflect the increasing influence of technology
in clinical medicine.
The name chosen for this comprehensive medical handbook series is Vademecum,
a Latin word that roughly means “to carry along”. In the Middle Ages, traveling
clerics carried pocket-sized books, excerpts of the carefully transcribed canons,
known as Vademecum. In the 19th century a medical publisher in Germany, Samuel
Karger, called a series of portable medical books Vademecum.
The Vademecum books are intended to be used both in the training of physicians
and the care of patients, by medical students, medical house staff and practicing
physicians. We hope you will find them a valuable resource.
Arensman
Robert M. Arensman
Bambini
Almond
All titles available at I SBN 1- 57059- 499- 6
www.landesbioscience.com Daniel A. Bambini

P. Stephen Almond

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LANDES LANDES LANDES

BIOSCIENCE V ad e me c u m BIOSCIENCE BIOSCIENCE V ad e me c u m

1. Preoperative Care 12. Inguinal Hernia and Hydrocele

This is one of a new series of medical handbooks.

All titles available at I SBN 1- 57059- 499- 6

www.landesbioscience.com Daniel A. Bambini

Robert M. Arensman, M.D.

Daniel A. Bambini, M.D.

P. Stephen Almond, M.D.

Copyright ©2000 Landes Bioscience

Please address all inquiries to the Publisher:

Library of Congress Cataloging-in-Publication Data

Daniel A. Bambini, M.D.

P. Stephen Almond, M.D.

Diane Dado John Hijjawi

Richard Fox Samer Kanaan

This modest manual of pediatric surgery has been prepared as a

Immediate Postoperative Care

entrapped are more rapidly destroyed, resulting in splenomegaly, jaundice, and

Genetics and Prenatal Diagnosis

Although a single ultrasound study can provide a wealth of information, serial

Central Venous Access

Fluids and Electrolytes

Paramount to successful treatment of infants and children with surgical disease

Other Sources of Fluid Imbalance (Sensible Water Loss)

Note: The above table is only an estimate of fluid requirements. Careful

Maintenance Fluid rRequirements for Term Infants

Maintenance electrolytes for term infants and children up to 20 kg

Component Supplied As Amount Required Comments

K KCl; K phosphate 2-4 mEq/kg/day Each millimole of K phosphate

Ca Ca Gluconate 0.5-3.0 mEq/kg/day Premature infants require more

Calcium- [(Calcium mEq/kg) + (Phosphate mM/kg)] x Wt(kg) x 100 Adjunct Calcium or

PO4 K phosphate 0.5-1.5 mM/kg/day Order only to provide mainten-

Mg MgSO4 0.5-1.0 mEq/kg/day A major cation in the body acting

Nutrition and Metabolism

Table 7.1. Pediatric PN: Macronutrients

Fluid Combination of 60-150 mL/kg/day See Chapter on fluid and electro-

Table 7.2. Pediatric PN: Micronutrients

Vit B12 Cyanocabalamin 1 mcg is provided in Important coenzyme function

Folic Acid 140 mcg is provided Important factor in cellular

Multivitamins Pediatric Multi- 2-3 mL/day* Each 3 mL vial contains:

Phytonadione AquaMephyton® 200 mcg are pro- Important in the production of

Trace elements Each 0.3 mL of trace elements

Trace elements Each 0.1 mL of trace elements

Heparin 1 IU/mL Helps prevent platelet thrombi

nonprotein calories should be sufficient to provide a total nonprotein-calorie-to-

Table 7.3. Blood values monitored routinely during parenteral nutrition

Na, K, Cl SGOT, LDH, alkaline Copper

*Serum levels should be monitored more frequently in the premature infant.

Table 7.4. Potential metabolic complications from PN

Pediatric Nutritional Assessment

Respiratory Failure and Support

Hypovolemic Shock and Resuscitation

Decreased Diastolic Pressure

perfusion. Once these tasks are accomplished, attention is directed at evaluation of

Blood Component Therapy

Perioperative Infections and Antibiotics

Perioperative infections in surgical patients include postoperative wound infec-

3. patients in whom an artificial device such as a vascular prosthesis will be

Table 11.1. Recommendations for prophylactic antibiotic therapy in surgical