OnLine Pediatric Surgery HANDBOOK

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Content

I. INTRODUCTION
A. Neonatal Physiologic Characteristics
---1. Water Metabolism
---2. Fluid and Electrolytes Concepts
B. Variations in Individual Newborns
---1. Types of Newborns Infants
---2. Metabolic and Host Defenses
---3. Surgical Response of Newborns
II. HEAD AND NECK LESIONS
A. Cervical Lymphadenopathy
B. Congenital Torticollis
C. Thyroglossal Duct Cyst
D. Branchial Cleft Fistula
E. Cystic Hygromas
III. OBSTRUCTIVE PROBLEMS
Neonatal Intestinal Obstruction Logical Approach
A. Esophageal Atresia
B. Gastro-Duodenal Anomalies
---1. Gastric Anomalies
---2. Pyloric Stenosis
---3. Duodenal Malformations
C. Malrotation and Volvulus
D. Intestinal Atresia
E. Meconium Ileus
F. Hirschsprung's disease
---1. Total Colonic Aganglionosis
G. Imperforate Anus
H. Duplications
I. Intussusception
J. Appendicitis
K. Chronic Intestinal Pseudo-obstruction
L. Bezoars
M. Neuronal Intestinal Dysplasia
IV. HERNIAS AND ABDOMINAL WALL DEFECT
A. Diaphragmatic Hernias
---1. Congenital Diaphragmatic Hernia (Bochdalek)
---2. Morgagni Hernia
---3. Esophageal Hernias
B. Processus Vaginalis Remnants
---1. Inguinal Hernias
---2. Hydroceles
C. Undescended Testis
D. Umbilical hernias
E. Omphalocele
F. Gastroschisis
V. GASTROINTESTINAL BLEEDING

A. Upper GI bleeding (Newborn)

B. Lower GI bleeding (Newborn)
---1. Necrotizing Enterocolitis (NEC)
C. Upper GI bleeding (Older child)
D. Lower GI bleeding (Older child)
---1- Anal Fissure
---2- Meckel's Diverticulum
---3- Juvenile Polyps
VI. PANCREATIC AND BILIARY DISORDERS
A. Pancreatitis
B. Biliary Atresia
C. Choledochal Cyst
D. Cholelithiasis
E. Idiopathic Perforation Bile Ducts
VII. TUMORS
A. Wilms tumor & Genetics
B. Neuroblastoma & Genetics
C. Rhabdomyosarcoma & Genetics
D. Liver Tumors
E. Sacrococcygeal Teratoma
F. Ovarian Tumors
G. Thyroid Nodules
H. Burkitt's Lymphoma
VIII. GYNECOLOGIC CONSIDERATIONS
A. Labial Adhesions in Infants
B. Ovarian Cyst
C. Breast Disorders
IX. PRENATAL CONGENITAL MALFORMATIONS
A. Fetal Surgery
B. Fetal Intestinal Obstruction
C. Prenatal CCAM
X. PEDIATRIC LAPAROSCOPY
A. Physiology of the Pneumoperitoneum
B. Laparoscopic Cholecystectomy
C. Laparoscopic Appendectomy
D. Laparoscopy for the Undescended Testis
E. Groin Laparoscopy
F. Laparocopic Splenectomy
G. Laparoscopic Fundoplication
XI. SUGGESTED READING
A. Specific Reading
B. General Reading
Created: March 1996
Last updated: March 2001

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I. INTRODUCTION
A. Neonatal Physiologic Characteristics
1. Water metabolism
Water represents 70 to 80% of the body weight of the normal neonate and
premature baby respectively. Total body water (TBW) varies inversely with fat
content, and prematures have less fat deposits. TBW is distributed into
extracellular fluid (ECF) and intracellular fluid (ICF) compartment. The ECF
compartment is one-third the TBW with sodium as principal cation, and
chloride and bicarbonate as anions. The ICF compartment is two-third the
TBW with potassium the principal cation. The Newborn's metabolic rate is high
and extra energy is needed for maintenance of body temperature and growth.
A change in body water occurs upon entrance of the fetus to his new
extrauterine existence. There is a gradual decrease in body water and the
extracellular fluid compartment with a concomitant increase in the intracellular
fluid compartment. This shift is interrupted with a premature birth. The
newborn's body surface area is relatively much greater than the adults and
heat loss is a major factor. Insensible water loss are from the lung (1/3) and
skin (2/3). Transepithelial (skin) water is the major component and decreases
with increase in post-natal age. Insensible water loss is affected by gestational
age, body temperature (radiant warmers), and phototherapy. Neonatal renal
function is generally adequate to meet the needs of the normal full-term infant
but may be limited during periods of stress. Renal characteristics of newborns
are a low glomerular filtration rate and concentration ability (limited urea in
medullary interticium) which makes them less tolerant to dehydration. The
neonate is metabolically active and production of solute to excrete in the urine
is high. The kidney in the newborn can only concentrate to about 400 mOsm/L
initially (500-600 mOsm/L the full-term compared to 1200 mOsm/L for an adult),
and therefore requires 2-4 cc/kg/hr urine production to clear the renal solute
load. The older child needs about 1-2 cc/kg/hr and the adult 0.5-1 cc/kg/hr.
Back to Index

2. Fluid and Electrolytes Concepts

Cellular energy mediated active transport of electrolytes along membranes is
the most important mechanism of achieving and maintaining normal volume
and composition of fluid compartments. Infants can retain sodium but cannot
excrete excessive sodium. Electrolytes requirements of the full-term neonate

are: Sodium 2-3 meq/kg/day, potassium 1-2 meq/kg/day, chloride 3-5

meq/kg/day at a rate of fluid of 100cc/kg/24 hrs for the first 10 kg of weight. As
a rule of thumb, the daily fluid requirements can be approximated too:
prematures 120-150cc/kg/24 hrs
neonates (term) 100cc/kg/24 hrs
Infants >10kg 1000cc+ 50cc/kg/24 hrs.
Special need of preterm babies fluid therapy are: conservative approach,
consider body weight changes, sodium balance and ECF tonicity. They are
susceptible to both sodium loss and sodium and volume overloading. High
intravenous therapy can lead to patent PDA, bronchopulmonary dysplasia,
enterocolitis and intraventricular hemorrhage. Impaired ability to excrete a
sodium load that can be amplify with surgical stress (progressive renal
retention of sodium). Estimations of daily fluid requirements should take into
consideration:
(1) urinary water losses,
(2) gastrointestinal losses,
(3) insensible water losses, and
(4) surgical losses (drains).
Blood Volumes estimates of help during surgical blood loss are:
premature 85-100 cc/kg,
term 85 cc/kg,
and infant 70-80 cc/kg.
The degree of dehydration can be measured by clinical parameters such as:
body weight, tissue turgor, state of peripheral circulation, depression of
fontanelle, dryness of the mouth and urine output. Intravenous nutrition is one
of the major advances in neonatal surgery and will be required when it is
obvious that the period of starvation will go beyond five days. Oral feeding is
the best method and breast is best source. Newborn infants requires 100-200
calories/kg/day for normal growth. This is increased during stress, cold,
infection, surgery and trauma. Minimum daily requirement are 2-3 gm/kg of
protein, 10-15 gm/kg of carbohydrate and small amount of essential fatty acids.
Back to Index

B. Variations in Individual Newborns

1. Types of Newborns Infants

a) The full-term, full-size infant with a gestational age of 38 weeks and a body
weight greater than 2500 grams (TAGA)- they received adequate intrauterine
nutrition, passed all fetal tasks and their physiologic functions are predictable.
b) The preterm infant with a gestational age below 38 weeks and a birth weight
appropriate for that age (PreTAGA);
c) The small-for-gestational-age infant (SGA) with a gestational age over 38
weeks and a body weight below 2500 grams- has suffered growth retardation
in utero.
d) A combination of (b) and (c), i.e., the preterm infant who is also small for
gestational age.
The characteristic that most significantly affects the survival of the preterm
infant is the immature state of the respiratory system. Between 27 and 28
weeks of gestation (900-1000 grams), anatomic lung development has
progressed to the extent that extrauterine survival is possible. It is only after
30 to 32 weeks of gestation that true alveoli are present. Once there is
adequate lung tissue, the critical factor that decides extrauterine adaptation
and survival of the preterm infant is his capabilities to produce the
phospholipid-rich material, surfactant that lines the respiratory epithelium.
Back to Index

2. Metabolic and Host Defenses

Handling of the breakdown products of hemoglobin is also a difficult task for
the premature infant. The ability of the immature liver to conjugate bilirubin is
reduced, the life span of the red blood cell is short, and the bilirubin load
presented to the circulation via the enterohepatic route is increased.
"Physiologic" jaundice is, therefore, higher in the preterm infant and persists
for a longer period. Unfortunately, the immature brain has an increased
susceptibility to the neurotoxic effects of high levels of unconjugated bilirubin,
and kernicterus can develop in the preterm baby at a relatively low level of
bilirubin. Other problems affecting the baby include the rapid development of
hypoglycemia (35 mg%), hypocalcemia and hypothermia. Newborns have a
poorly developed gluconeogenesis system, and depends on glycolysis from
liver glycogen stores (depleted 2-3 hrs after birth) and enteral nutrition.
Immature infants can develop hyperglycemia from reduced insulin response to
glucose causing intraventricular hemorrhage and glycosuria. The preterm and
surgical neonate is more prone to hypocalcemia due to reduced stores, renal
immaturity, and relative hypoparathyroidism (high fetal calcium levels).
Symptoms are jitteriness and seizures with increase muscle tone. Calcium
maintenance is 50 mg/kg/day. Human beings are homeothermic organisms
because of thermoregulation. This equilibrium is maintained by a delicate
balance between heat produced and heal lost. Heat production mechanisms
are: voluntary muscle activity increasing metabolic demands, involuntary
muscle activity (shivering) and non-shivering (metabolizing brown fat). Heat

loss occurs from heat flow from center of the body to the surface and from the
surface to the environment by evaporation, conduction, convection and
radiation. There is an association between hypothermia and mortality in the
NICU's. The surgical neonate is prone to hypothermia. Infant produce heat by
increasing metabolic activity and using brown fat. Below the 35C the newborn
experiences lassitude, depressed respiration, bradycardia, metabolic acidosis,
hypoglycemia, hyperkalemia, elevated BUN and oliguria (neonatal cold injury
syndrome). Factors that precipitate further these problems are: prematurity,
prolonged surgery, and eviscerated bowel (gastroschisis). Practical
considerations to maintain temperature control are the use of humidified and
heated inhalant gases during anesthesia, and during all NICU procedures use
radiant heater with skin thermistor-activated servo-control mechanism. The
newborn's host defenses against infection are generally sufficient to meet the
challenge of most moderate bacterial insults, but may not be able to meet a
major insult. Total complement activity is 50% of adults levels. C3,C4,C5
complex, factor B, and properdin concentration are also low in comparison to
the adult. IgM, since it does not pass the placenta, is absent.
Back to Index

3- Surgical Response of Newborns

The endocrine and metabolic response to surgical stress in newborns (NB) is
characterized by catabolic metabolism. An initial elevation in cathecolamines,
cortisol and endorphins upon stimulation by noxious stimuli occurs; a defense
mechanism of the organism to mobilize stored energy reserves, form new
ones and start cellular catabolism. Cortisol circadian responsiveness during
the first week of life is diminished, due to inmaturation of the adrenal gland.
Cortisol is responsible for protein breakdown, release of gluconeogenic
aminoacids from muscle, and fat lipolysis with release of fatty acids. Glucagon
secretion is increased. Plasma insulin increase is a reflex to the hyperglycemic
effect, although a resistance to its anabolic function is present. During
surgical stress NB release glucose, fatty acids, ketone bodies, and amino
acids; necessary to meet body energy needs in time of increase metabolic
demands. Early postoperative parenteral nutrition can result in significant rate
of weight gain due to solid tissue and water accumulation. Factors correlating
with a prolonged catabolic response during surgery are: the degree of
neuroendocrinological maturation, duration of operation, amount of blood
loss, type of surgical procedure, extent of surgical trauma, and associated
conditions (hypothermia, prematurity, etc.). They could be detrimental due to
the NB limited reserves of nutrients, the high metabolic demands impose by
growth, organ maturation and adaptation after birth. Anesthetics such as
halothane and fentanyl can suppress such response in NB.
Back to Index

II. NECK LESIONS

A. Cervical Lymphadenopathy

An enlarged lymph node is the most common neck mass in children. Most are
anterior to the sternocleidomastoid muscle. Infection is the usual cause of
enlargement; viral etiology and persist for months. Acute suppurative
submandibular adenitis occur in early childhood (6 mo-3 yrs), is preceded by
pharyngitis or URI, the child develops erythema, swelling and cellulitis, and
management is antibiotics and drainage. Chronic adenitis: persistent node (> 3
wk., tonsillar), solitary, non-tender, mobile and soft. Generally no tx if < 1 cm,
for nodes above 2 cm sizes with rapid growth, clustered, hard or matted do
biopsy. Other causes are: (1) Mycobacterial adenitis- atypical (MAIS complex),
swollen, non-tender, nor-inflamed, positive skin test, excision is curative,
chemotx is of no value. (2) Cat-Scratch adenitis- caused by A. Fellis,
transmitted by kittens, positive complement fixation test, minimally tender,
fluctuant regional nodes, spontaneous resolution. (3) Hodgkin's disease
mostly teenager and young adults, continuing growth, non-tender node,
associated to weight loss, biopsy is diagnostic.
Back to Index

B. Congenital Torticollis
Congenital muscular torticollis is a disorder characterize by shortening of the
cervical muscles, most commonly the sternocleidomastoid (SCM) muscle, and
tilting of the head to the opposite side. This is the result of endomysial fibrosis
of the SCM muscle. There is a relationship between birth position and the side
affected by the contracture. Congenital torticollis causes: plagiocephaly (a
craniofacial deformity), fascial asymmetry (hemihypoplasia), scoliosis and
atrophy of the ipsilateral trapezius muscle if not corrected. Torticollis can
develop at any age, although is more common during the first six months of
life. The SCM muscle can be a fibrous mass, or a palpable tumor 1-3 cm in
diameter within the substance of the muscle is identified by two to three weeks
of age. Management is conservative in most cases using early physiotherapy
exercises' a mean duration of three months to achieve full passive neck range
of motion. The severity of restriction of motion is the strongest predictor of
treatment duration. Those children with failed medical therapy or the
development of fascial hemihypoplasia should undergo surgical transection of
the SCM muscle.
Back to Index

C. Thyroglossal Duct Cysts

Thyroglossal duct cyst (TDC) is the most common congenital anterior midline
neck mass usually (2/3 of cases) presenting before the second decade of life.
Symptoms appear at an average age of four with the sudden appearance of a
cystic mass at the angle of neck level moving with tongue protrusion and
swallowing. Males are more commonly affected than females. TDC is an
embryologic anomaly arising from epithelial remnant left after descent of the
developing thyroid from the foramen cecum. The lining is cuboidal, columnar
or pseudostratified epithelium. TDC is associated to discomfort, infection and
a slight probability of malignancy. A legally protective requirement is to

document that the mass is not ectopic thyroid gland. Diagnosis is physical.
Sonograms will show a cyst between 0.4 and 4 cm in diameter, with variable
sonographic appearance and no correlation with pathological findings of
infection or inflammation. Once infected surgical excision is more difficult and
recurrence will increase. Management is Sistrunk's operation: Excision of cyst
with resection of duct along with the central portion of hyoid bone (a minimum
of 10-15 mm of hyoid bone should be removed) and some muscle surrounding
the proximal ductules (the length of single duct above the hyoid bone spreads
into many ductuli as it approach the foramen cecum). Extensive dissection can
cause pharyngodynia. The greatest opportunity for cure is surgery at initial
non-inflamed presentation. Inadequate excision is a risk factor for further
recurrence.
Back to Index

D. Branchial Cleft Fistulas

Branchial cleft fistulas (BCF) originate from the 1st to 3rd branchial apparatus
during embryogenesis of the head and neck. Anomalies of the 2nd branchial
cleft are by far the most commonly found. They can be a cyst, a sinus tract or
fistulas. Fistulas (or sinus tract if they end blindly) display themselves as small
cutaneous opening along the anterior lower third border of the
sternocleidomastoid muscle, communicates proximally with the tonsillar
fossae, and can drain saliva or a mucoid secretion. Management consists of
excision since inefficient drainage may lead to infection. I have found that
dissection along the tract (up to the tonsillar fossa!) can be safely and easily
accomplished after probing the tract with a small guide wire in-place. This will
prevent injury to nerves, vessels and accomplish a pleasantly smaller scar.
Occasionally a second stepladder incision in the neck will be required. 1st
BCF are uncommon, located at the angle of the mandible, and communicating
with the external auditory canal. They have a close association with the fascial
nerve. 3rd BCF are very rare, run into the piriform sinus and may be a cause of
acute thyroiditis or recurrent neck infections.
Back to Index

E. Cystic Hygroma
Cystic hygroma (CH) is an uncommon congenital lesion of the lymphatic
system appearing as a multilocular fluid filled cavity most commonly in the
back neck region, occasionally associated with extensive involvement of
airway or vital structures. The etiology is intrauterine failure of lymphatics to
communicate with the venous system. Prenatal diagnosis can be done during
the first trimester of pregnancy as a huge neck tumor. Differential diagnosis
includes teratomas, encephalocele, hemangiomas, etc. There is a strong
correlation between prenatal dx and Turner's syndrome (> 50%), structural
defects (Noonan's syndrome) and chromosomic anomalies (13, 18, 21). Early
diagnosis (< 30 wk gestation) is commonly associated to those anomalies,
non-immune hydrops and dismal outcome (fetal death). Spontaneous
regression is less likely but can explain webbed neck of Turner and Noonan's

children. Prenatal dx should be followed by cytogenetic analysis: chorionic

villous sampling, amniocentesis, or nuchal fluid cell obtained from the CH
itself to determine fetal karyotype and provide counseling of pregnancy. Late
diagnosis (>30 wks) should be delivered in tertiary center prepare to deal with
dystocia and postnatal dyspnea of newborn. The airway should be secured
before cord clamping in huge lesions. Intracystic injection of OK432
(lyophilized product of Streptococcus pyogenes) caused cystic (hygromas)
lymphangiomas to become inflamed and led to subsequent cure of the lesion
without side effects.
Back to Index

III. OBSTRUCTIVE PROBLEMS

LOGICAL APPROACH TO NEONATAL INTESTINAL OBSTRUCTION
By: Jordan J. Weitzman, MD
Los Angeles, California, USA

Signs and Symptoms

1. Bilious vomiting is always abnormal.
2. Abdominal distention (scaphoid abdomen possible).
3. Delayed, scanty or no passage of meconium.
4. Polyhydramnios in mother.
5. Down's syndrome
6. Family history
a. Hirschsprung's disease
b. Diabetic mother
c. Jejunal atresia
Work-up (Logical approach)
1. While the infant is being studied, it must be kept in mind that the problem may be
"non-surgical".
a. Sepsis of the newborn with associated ileus is the most important cause of non-surgical
bilious vomiting and abdominal distention.
b. Intracranial lesions
i. Hydrocephalus
ii. Subdural hemorrhage
c. Renal disease associated with uremia.
i. Renal agenesis
ii. Polycystic disease
iii. Other urinary tract anomalies which may be associated with severe hydronephrosis.
2. Plain roentgenograms of the abdomen.
a. Diagnostic in complete high intestinal obstruction- no gas in distal small bowel.
i. Double bubble in duodenal obstruction.
ii. Few gas filled loops beyond duodenum indicates jejunal atresia.
b. Many gas filled loops (requires 24 hours) indicates some form of low intestinal
obstruction.
i. Ileal atresia
ii. Meconium ileus (an unfortunate misnomer)- obstruction of the distal small intestine by
thick undigested meconium.
iii. Meconium plug syndrome - obstruction of colon by a plug of meconium.
iv. Small left colon syndrome.
v. Hirschsprung's disease - congenital aganglionosis of colon starting with the rectum.
vi. Colonic atresia.
c. May be nonspecific in instances of malrotation of the intestines. This diagnosis must
always be considered in neonates with unexplained bilious vomiting.
d. Calcifications - at some time during fetal life meconium was (is) present in the abdomen.

3. Contrast enema will differentiate the various types of low intestinal obstruction.
a. Microcolon - complete obstruction of the small bowel.
b. Meconium plug syndrome - colon dilated proximal to an intraluminal mass.
c. Hirschsprung's disease - although it may appear to be diagnostic, not reliable in the
newborn.
d. Small left colon syndrome - colon dilated to the splenic flexure, then becomes narrow.
4. Upper G.I. series - the procedure of choice in diagnosing malrotation of the intestines. In the
past a contrast enema was thought to be the diagnostic test of choice in instances of
malrotation but the cecum and ascending colon can be in normal position in an infant or child
with malrotation of the intestines.
5. Rectal biopsy - a pathologist competent in reading the slides is essential and should not be
taken for granted.
a. Suction biopsy of the rectal mucosa and submucosa- best screening procedure to rule
out Hirschsprung's disease (ganglion cells are present in the submucosa), and is diagnostic in
experienced hands.
b. Full thickness biopsy of the rectal wall may be necessary if the suction biopsy is
non-diagnostic or if the pathologist is unwilling or unable to make the diagnosis of
aganglionosis on a suction biopsy specimen. This procedure is difficult in the small infant and
has been replaced by the suction biopsy in most centers.
c. All newborns who have delayed passage of meconium associated with a suspicious
contrast enema should have a suction biopsy of the rectal mucosa and submucosa. With this
technique, Hirschsprung's disease will be diagnosed early before it is complicated with
enterocolitis. If delayed passage of meconium is "cured" by rectal stimulation(suppository,
thermometer, or finger), it must be kept in mind that the diagnosis of Hirschsprung's disease
is still a possibility. Whether or not a suction biopsy of the rectum is done before the infant
goes home depends on the clinical setting but the safe course of action is to do the rectal
biopsy before discharge. Parents may not call before the infant gets into trouble with
enterocolitis.
d. Suction biopsy of the rectum is probably indicated in all cases of so called meconium
plug syndrome or small left colon syndrome. If the suction biopsy is not done, the infant must
be observed for recurrent gastrointestinal symptoms. A breast-fed infant who has
Hirschsprung's disease can "get by" for a prolonged period of time.
6. Concluding comments:
The newborn suspected of having intestinal obstruction should be studied in a logical step
by step manner. It is important that it be definitely established that the infant has a surgical
problem before surgery is performed. This is usually not difficult in instances of complete high
small bowel obstruction or when plain films of the abdomen show calcification and/or a distal
small bowel obstruction with the contrast enema showing a microcolon or a definite
malrotation of the colon (cecum in upper mid-abdomen or left upper quadrant).
When plain films are suggestive of a high small bowel obstruction but there is gas in the
distal small bowel, an upper GI series rather than a contrast enema should be performed. It is
critically important that the diagnosis of malrotation of the intestines be always considered
and ruled out in a neonate with bilious vomiting. Prompt recognition and treatment of
malrotation of the intestines which is often associated with a midgut volvulus avoids the dire
consequences of the problems associated with a massive small bowel resection.
Mistakes are frequently made when the contrast enema is interpreted as normal, meconium
plug syndrome, small left colon syndrome or Hirschsprung's disease. In all of these clinical
situations, a suction biopsy of the rectum is an excellent screening procedure. If ganglion
cells are present, Hirschsprung's disease is ruled out and the infant probably has a
non-surgical diagnosis. If ganglion cells are absent, the next step depends on the clinical
picture and setting. If the pathologist is experienced and confident of the interpretation, the
diagnosis of Hirschsprung's disease can be made with confidence. If there is any doubt about
the absence of ganglion cells in the suction biopsy, a full thickness biopsy of the rectum (a
difficult technical procedure requiring a general anesthetic) can be done to settle the issue. If
Hirschsprung's disease is believed to be the problem, it must be diagnosed histologically
before the infant is operated upon because at the time of surgery the site of obstruction may
not be apparent and the abdomen may be closed because no obvious site of obstruction is
found.
Hypothyroidism in the first two to three months of life can mimic Hirschsprung's disease in

all aspects except for a normal rectal biopsy.

Another important point to remember is that duodenal atresia is a different disease from
jejunal or ileal atresia in terms of their cause. Jejunal and ileal atresia occur as a result of a
vascular accident in the small bowel mesentery during fetal life. Consequently, there is a
relatively low incidence of other congenital anomalies except for cystic fibrosis.
Duodenal atresia is a different disease in that there is a very high incidence of associated
anomalies-- (Down's syndrome, imperforate anus, renal anomalies, congenital heart disease,
etc.).
Malrotation of the intestines and Hirschsprungs disease must be ruled out before a
newborn with unexplained bilious vomiting and/or abdominal distention is sent home. It can
be unsafe to rely on parents to observe their infant for problems resulting from the above
conditions. If diagnosed late, malrotation of the intestines or Hirschsprungs disease can
become life threatening or result in life long problems.
Back to Index
A. Esophageal Atresia
Esophageal atresia (EA) with distal tracheo-esophageal fistula (TEF) is the most common
congenital
anomaly of the esophagus, followed by EA without TEF also known as pure esophageal
atresia and
pure TEF. Incidence is one in every 2500 live births. The trachea and esophagus initially begin
as a ventral diverticulum of the foregut during the third intrauterine week of life. A proliferation
of endodermal cells appears on the lateral aspect of this growing diverticulum. These cell
masses will divide the foregut into trachea and esophageal tubes. Whether interruption of this
normal event leads to tracheo-esophageal anomalies, or during tracheal growth atresia of the
esophagus results because of fistulous fixation of the esophagus to the trachea remians to be
proven. Polyhydramnios is most commonly seen in pure EA. EA causes excessive salivation,
choking, coughing, regurgitation with first feed and inability to pass a feeding tube into the
stomach. Contrast studies are rarely needed and of potential disaster (aspiration). Correct
dehydration, acid-base disturbances, respiratory distress and decompress proximal
esophageal pouch (Reploge tube). Evaluate for associated conditions such as VACTERL
association. Correct dehydration, acid-base disturbances, respiratory distress and
decompress proximal esophageal pouch (Reploge tube). Evaluate for associated conditions
such as VACTERL association (3 or more):
-Vertebral anomalies i.e. hemivertebrae, spina bifida
-Anal malformations i.e. imperforate anus
-Cardiac malformations i.e. VSD, ASD, Tetralogy Fallot
-Tracheo-Esophageal fistula (must be one of the associated conditions)
-Renal deformities i.e. absent kidney, hypospadia, etc.
-Limb dysplasia
Early surgical repair (transpleural or extrapleural) is undertaken for those babies with
adequate arterial blood gases, adequate weight (>1200 gm) and no significant associated
anomalies. Delayed repair (gastrostomy first) for all other patients. Repair consists of
muscle-sparing thoracotomy, closure of TEF and primary anastomosis. Esophagogram is
done 7-10 days after repair. Most important predictors of outcome: birth weight, severity of
pulmonary dysfuntion, and presence of major congenital cardiac disease. Complications after
surgery: anastomotic leak, stricture, gastroesophageal reflux, tracheomalacia and recurrent
TEF. Increase survival is associated with improvements in perioperative care, meticulous
surgical technique and aggressive treatment of associated anomalies.
Congenital isolated tracheo-esophageal fistula (TEF) occurs as 4-6% of the disorders of the
esophagus bringing problems during early diagnosis and management. More than H-type is
N-type, due to the obliquity of the fistula from trachea (carina or main bronchi) to esophageal
side (see the figure) anatomically at the level of the neck root (C7-T1). Pressure changes
between both structure can cause entrance of air into the esophagus, or esophageal content
into the trachea. Thus, the clinical manifestation that we must be aware for early diagnosis
are: cyanosis, coughing and choking with feedings, recurrent chest infections, persistent
gastrointestinal distension with air, and hypersalivation. Diagnosis is confirmed with a
well-done esophagogram, or video-esophagogram (high success rates, establish level of the
TEF). Barium in the trachea could be caused by aspiration during the procedure. Upon
radiologic doubt bronchoscopy should be the next diagnostic step. Any delay in surgery is
generally due to delay in diagnosis rather than delay in presentation. Management consists of

surgical closure of the TEF through a right cervical approach. Hint: a small guide-wire
threaded through the fistula during bronchoscopy may be of some help. Working in the
tracheo-esophageal groove can cause injury to the recurrent laryngeal nerve with vocal cord
paralysis. Recurrence after closure is rare.aggressive treatment of associated anomalies.
The three most common anastomotic complications are in order of frequency: stricture,
leakage and recurrent TEF. Recurrent TEF after surgical repair for esophageal atresia occurs
in approximately 3-15% of cases. Tension on the anastomoses followed by leakage may lead
to local inflammation with breakage of both suture lines enhancing the chance of recurrent
TEF. Once established, the fistula allows saliva and food into the trachea, hence clinical
suspicion of this diagnosis arises with recurrent respiratory symptoms associated with
feedings after repair of esophageal atresia. Diagnosis is confirmed with cineradiography of the
esophagus or bronchoscopy. A second thoracotomy is very hazardous, but has proved to be
the most effective method to close the recurrent TEF. Either a pleural or pericardial flap will
effectively isolate the suture line. Pericardial flap is easier to mobilize, provides sufficient
tissue to use and serves as template for ingrowth of new mucosa should leakage occur. Other
alternatives are endoscopic diathermy obliteration, laser coagulation, or fibrin glue deposition.
B. Gastro-Duodenal Anomalies
B.1 Gastric Anomalies
Congenital gastric outlet obstruction is extremely rare. It occurs either in the pyloric or antral
region. Antral membranes (web or diaphragm) are thin, soft and pliable, composed of
mucosa/submucosa, and located eccentric 1-3 cm proximal to pyloro-duodenal junction. They
probably represent the developmental product of excess local endodermal proliferation and
redundancy. The diagnosis should rely on history, contrast roentgenology studies and
endoscopic findings. Symptoms are those of recurrent non-bilious vomiting and vary
according to the diameter of aperture of the membrane. There is a slight male predominance
with fair distribution between age groups in children. Associated conditions: pyloric stenosis,
peptic ulcer and cardiac. History of polyhydramnios in the mother. Demonstration of a
radioluscent line perpendicular to the long axis of the antrum is diagnostic of a web.
Endoscopy corroborates the diagnosis. Management can be either surgical or non-surgical.
Surgical Tx is successful in symptomatic pt. and consist of pyloroplasty with incision or
excision of the membrane. Other alternative is endoscopic balloon dilatation or transection of
the web. Non-obtructive webs found incidentally can be managed medically with small curd
formula and antispasmodics. The presence of an abnormally dilated gastric bubble in prenatal
sonography should alert the physician toward the diagnosis of congenital antro-pyloric
obstruction.
B.2 Pyloric Stenosis
Is an abnormality of the pyloric musculature (hypertrophy) causing gastric outlet obstruction
in early infancy. The incidence is 3 per 1000 live births. The etiology is unknown, but
pylorospasm to formula protein cause a work hypertrophy of the muscle. Diagnostic
characteristics are: non-bilious projectile vomiting classically 3-6 weeks of age, palpable
pyloric muscle "olive", contrast studies are not necessary when the pyloric muscle is
palpated, enlarged width and length in ultrasonography. The treatment consist in correction
of hypochloremic alkalosis and state of dehydration and performing a Fredet-Ramstedt
modified pyloromyotomy. Post-operative management consist of: 50% will have one to several
episodes of vomiting, usually can feed and go home in 24-36 hours, initial feeds start 8-12
hours after surgery.
B.3 Duodenal Malformations
Can be intrinsic (Atresia, Stenosis, Webs) or extrinsic (Annular pancreas, Ladd's bands).
Occur distal or proximal to the ampulla of Vater. Most commonly distal to ampulla and
therefore bilious vomiting is present. (Note: Bilious vomiting is surgical until proven otherwise
in a baby).
"Windsock" webs have clinical importance because of their tendency to be confused with
distal duodenal obstruction and because of the frequent occurrence of an anomalous biliary
duct entering along their medial margin. Embryology: The first major event in the
differentiation of the duodenum, hepatobiliary tree, and pancreas occurs at about the third

week in gestation, when the biliary and pancreatic buds form at the junction of the foregut and
the midgut. The duodenum at this time is a solid cord of epithelium, which undergoes
vacuolization followed by recanalization and restitution of the intestinal lumen over 3-4 weeks
of normal development. Failure of recanalization of the second part of the duodenum results in
congenital obstruction of the lumen, often in conjunction with developmental malformation of
the pancreatic anlagen and the terminal part of the biliary tree. In support of this concept is the
high incidence of annular pancreas observed, believed to represent a persistence of the
ventral pancreatic anlage in association with intrinsic duodenal obstruction.
Congenital partial obstruction of the duodenum can be either intrinsic (membrane, web or
pure) or
extrinsic (Ladd's bands, annular pancreas). A significant group (25-33%) is born with Down's
syndrome.
This does not entail a higher risk of early mortality unless associated with cardiac
malformations. Other
associated conditions are malrotation (midgut volvulus is rare due to absent bowel distension
and
peristalsis), biliary tract anomalies and Meckel's diverticulum. The diagnosis is suggested in
utero by
the double-bubble image on ultrasound. Vomiting is the most frequent presenting symptom.
UGIS is
diagnostic, showing a dilated stomach and first duodenal portion with scanty passage of
contrast
material distally. Management varies accordingly to the type of stenosis: Ladd's bands are
lysed. Pure
stenosis is opened longitudinally and closed transversely (Heineke-Mickulicz). Membranous
stenosis is
resected. Successful endoscopic membranectomy of duodenal stenosis has been reported.
Duodeno-duodenostomy is the procedure of choice for annular pancreas. Diaphragms can
rarely be
double. Anastomotic malfunction requiring prolonged intravenous nutrition and
hospitalization has
prompted development of a diamond shape larger stoma. Tapering or plication of the dilated
duodenum
is another effective method of improving disturbed transit. Other complications after surgery
are
megaduodenum with blind loop syndrome, biliary reflux, cholestatic jaundice, delayed transit
and bowel
obstruction. Early mortality is associated to prematurity and associated malformations.
Long-term
follow-up is warranted to identify late problems.
The diagnostic characteristics are: bilious vomiting, history of polyhydramnios in mother,
KUB with classic "Double-bubble" appearance, a microcolon in barium enema study or
malrotation.
Treatment consist in: (1) duodeno-duodenostomy bypass for atresias, annular pancreas, and
some stenosis. (2) duodenoplasty for webs, and stenosis, and (3) lysis of ladd's bands and
Ladd's procedure for malrotation. Associated anomalies are: Down's syndrome (20-30%),
VACTERL syndrome, CNS anomalies and cardiac anomalies.
C. Malrotation and Volvulus
The rotation and normal fixation of the intestinal tract takes place within the first three months
of fetal life. In the earliest stages when the intestinal tract is recognizable as a continuous
tube, the stomach, small intestine, and colon constitute a single tube with its blood supply
arising posteriorly. The midgut portion of this tube, from the second portion of the duodenum
to the mid-transverse colon, lengthens and migrates out into an extension of the abdomen,
which lies at the base of the umbilical cord. Here this loop of bowel undergoes a 270-degree
counterclockwise twist at its neck. In the center of the twisted loop lie the blood vessels that
will become the superior mesenteric artery and vein. After rotation, the small intestine quite
rapidly withdraws into the abdominal cavity, with the duodenum and the proximal jejunum
going first. During this process the duodenojejunal junction goes beneath and to the left of the
base of the superior mesenteric vessels. This leaves the upper intestine, including the
stomach and the duodenum, encircling the superior mesenteric vessels like a horseshoe with
its opening on the left side of the embryo. The small intestine then follows into the abdomen,

and withdrawal of the right half of the colon takes place so that it lies to the left. At the next
step, the cecum and the right colon begin to travel across the top of the superior mesenteric
vessels and then down to the right lower quadrant. The colon now lies draped across the top
of the superior mesenteric vessels, again like a horseshoe, with its opening placed inferiorly.
The duodenojejunal loop is said to attach to the posterior abdominal wall soon after its turn,
whereas the mesenteric attachments of the entire colon and of the remaining small bowel
gradually adhere after they arrive in their normal positions. In malrotation the right colon can
create peritoneal attachments that include and obstruct the third portion of the duodenum
(Ladd's bands).
The diagnostic hallmarks are: bilious vomiting (the deadly vomit), abdominal distension and
metabolic acidosis. A UGIS is more reliable than barium enema, most patients present in first
month of life (neonatal), but may present at any time.
The treatment is immediate operation; volvulus often means strangulation. Needs fluid and
electrolyte replacement. Ladd's procedure consist of: reduce volvulus with a
counterclockwise rotation, place small bowel in right abdomen, lysed ladd's bands, place
large bowel in left abdomen, do an appendectomy. In cases of questionable non-viable bowel
a second look procedure is required.

D. Intestinal Atresias
Intestinal atresias are the product of a late intrauterine mesenteric vascular accident (blood
supply was not received by a portion of bowel) as attested by Louw and Barnard in 1955. They
are equally distributed from the ligament of treitz to the ileocecal junction. Colonic atresias are
very rare. There is proximal bowel dilatation, with distal (unused) micro-bowel. The diagnosis
is suspected with maternal history of polyhydramnios (the higher the atresia), bilious
vomiting, abdominal distension and obstipation. KUB shows "thumb-size" dilated bowel
loops, and barium enema a microcolon of disuse. Louw classified them into: Type I: an
intraluminal diaphragm with seromuscular continuity. Type II: cord-like segment between the
bowel blinds ends. Type IIIA: atresia with complete separation of blind ends and V-shaped
mesenteric defect (see figure), the most commonly found. Type IIIB: jejunal atresia with
extensive mesenteric defect and distal ileum acquiring its blood supply entirely from a single
ileocolic artery. The distal bowel coils itself around the vessel, giving the appearance of an
"apple peel"deformity. Type IV: multiple atresias of the small intestine. After preoperative
stabilization (GI decompression, electrolytes disturbances' correction, antibiotherapy, and
normothermia), treatment consists of exploratory laparotomy, resection of proximal dilated
intestine, and end to oblique anastomosis in distal jejuno-ileal atresias. Tapering jejunoplasty
with anastomosis is preferred in proximal defects.
Back to Index
E. Meconium Ileus
Meconium ileus is a neonatal intraluminal intestinal obstruction caused by inspissated
meconium blocking the distal ileum. Occurs in 10-15% of all patients with cystic fibrosis, and
85-95% of patients with meconium ileus have cystic fibrosis. The meconium has a reduced
water, abnormal high protein and mucoproteint content, the result of decreased pancreatic
enzyme activity and prolonged small bowel intestinal transit time. Meconium Ileus is classified
into two types: (1) Simple meconium ileus: The distal small bowel (10-30 cm of distal ileum) is
relatively small, measuring less than 2 cm in diameter and contains concretions of gray,
inspissated meconium with the consistency of thick glue or putty. It is often beaklike in
appearance, conforming to the shape of the contained pellets. Proximally, the mid-ileum is
large, measuring up to 7 cm in diameter. It is greatly distended by a mass of extremely thick,
tenacious, dark green or tarry meconium. The unused small colon (microcolon) contains a
small amount of inspissated mucus or grayish meconium. (2) Complicated meconium ileus:
usually occurs during the prenatal period associated to volvulus, atresias, gangrene,
perforation or peritonitis. A cystic mass or atresia of the bowel may occur. The degree of
obstruction varies, may be cured in mild cases by rectal irrigations. Failure to pass meconium,
abdominal distension and vomiting are seen in more severe cases. The diagnosis is
suspected with findings of: multiple loops of dilated small bowel and coarse granular
"soap-bubble" appearance on plain abdominal films. Some cases may show calcifications in
the peritoneum (Meconium peritonitis). The Sweat Test is diagnostic of cystic fibrosis (value
over 60 meq/L of sweat sodium or chloride are diagnostic). This test is not useful in infant

during first weeks of life. Therapy is either: (1) Nonoperative- should be tried first. It consist of
a careful gastrograffin enema after the baby is well-hydrated. Gastrograffin is a hyperosmolar
aqueous solution of meglumine diatrizoate containing 0.1% polysorbate-80 (tween-80, a
wetting agent) and 37% iodine. Its success is due to the high osmolarity (1700 mosm/liter)
which draws fluid into the bowel and softens and loosens the meconium. (2) Surgical therapy
that has included: ileostomy with irrigation, resection with anastomosis, and resection with
ileostomy (Mikulicz, Bishop-Koop, and Santulli). Post-operative management includes: 10%
acetylcysteine p.o., oral feedings (pregestimil), pancreatic enzyme replacement, and
prophylactic pulmonary therapy. Long-term prognosis depends on the degree of severity and
progression of cystic fibrosis pulmonary disease.
Back to Index
F. Hirschsprung's Disease
Hirschsprung's is the congenital absence of parasympathetic innervation of the distal
intestine. The colon proximal to the aganglionic segment, in an effort to overcome the partial
obstruction, becomes distended and its wall markedly thickened because of muscle
hypertrophy. Occurs 1 in 1000-1500 live births with a 4:1 male predominance. 96% are TAGA.
4% prematures.
The parasympathetic ganglion cell network located between the circular and longitudinal
muscle layers is referred to as Auerbach's plexus, whereas Meissner's plexus is the
submucosal layer of ganglion cells just beneath the muscularis mucosa. In Hirschsprung's
disease, ganglion cells are absent from all layers. That aganglionic segment usually involves
the terminal intestine, i.e. the rectum or rectosigmoid. The aganglionic segment may, however,
include the entire large bowel and even small bowel.
Hirschsprung's disease (HD) is characterized by lack of enteric ganglion cells, hyperplasia
of abnormal nerve fibers and a non-propulsive, non-relaxing segment of bowel. Classically the
etiology is attributed to a failure of cranio-caudal migration of parasympathetic neural crest
cells to the distal bowel. A plausible explanation for the failure of relaxation of the bowel
involved is a deficiency of enteric inhibitory nerves that mediates the relaxation phase of
peristalsis. This nerves are intrinsic to the gut and are classify as non-adrenergic and
non-cholinergic. Nitric oxide (NO) has recently been implicated as the neurotransmitter which
mediates the relaxation of smooth muscle of the GI tract in HD. It's absence in aganglionic
bowel might account for the failure of relaxation during peristalsis. Besides, adhesions
molecules (absent in aganglionic bowel) during early embryogenesis might restrict the
neuro-ectodermal origin involved in the initial contact between nerves and muscle cell
(synaptogenesis) suggesting that developmental anomaly of innervated muscle and absent
NO causes the spasticity characteristic of HD.
Symptoms usually begin at birth, frequently with delayed passage of meconium. Any
newborn who fails to pass meconium in the first 24-48 hours of life should be evaluated for
possible Hirschsprung's disease. In some infants, the presentation is that of complete
intestinal obstruction. Others have relatively few symptoms until several weeks of age, when
the classic symptom of constipation has its onset. Diarrhea is not uncommon but differs from
the usual infantile diarrhea in that it is associated with abdominal distension. Occasionally the
patient will go many years with mild constipation and diagnosis will be delayed.
The diagnosis is first suspected based on history and physical examinations
(characteristically there is no stool in rectum and abdominal distension is painless). Initial
evaluation includes an unprepped barium enema (the first enema should be a barium enema!).
The aganglionic rectum appears of normal caliber or spastic, there is a transition zone and
then dilated colon proximal to the aganglionic segment. 24-hrs delayed films shows poor
emptying with barium throughout the colon, as opposed to the child with psychogenic stool
holding in whom the barium generally collects in the distal rectosigmoid. Rectal suction
biopsy is then performed. This can be done without anesthesia and the submucosal plexus is
examined for ganglion cells. With experience, a good pathologist (should be an expert!), can
identify the presence or absence of ganglion cells in this specimen without a full thickness
biopsy. Difficulty in interpreting the specimen or not enough to include several slides of
submucosa would require a full-thickness biopsy for definitive diagnosis generally done under
general anesthesia. Some centers employ manometry, histochemical studies or special stains
for diagnosis. These special studies are only as good as the person performing them and
interpreting the results.
The initial treatment requires performing a "leveling" colostomy in the most distal colon
with ganglion cells present. This requires exploration with multiple seromuscular biopsies of

the colon wall to determine the exact extend of the aganglionosis. The colostomy is placed
above the transition zone. Placement of the colostomy in an area of aganglionosis will lead to
persistent obstruction. Once the child has reached an adequate size and age (6-12 months; 20
pounds or more), a formal pull-through procedure is done. Some of this are: Swenson,
Duhamel and Soave procedures. Current preference is for Soave procedure (modified
endorectal pull-through) and consist of resection of the majority of aganglionic bowel except
for the most distal rectum, the mucosa and submucosa of this rectum is excised and the
normally innervated proximal bowel is pulled through the seromuscular coat of retained
rectum and suture immediately above the dentate line. Recently a laparoscopic procedure
without colostomy use is being done earlier in life with promising results.
Back to Index
F1. Total Colonic Aganglionosis
by: Peter Sacher, MD *
Total colonic aganglionosis (TCA) is found in approx. 2 to 13% of patients with
Hirschsprung's disease (2). There are three critical phases for patients with TCA (15).
The first period comprises the time from birth until correct diagnosis. Patients with TCA
present with a large variety of symptoms. Several authors have outlined the diagnostic
problems in patients with TCA (2,4,18). Atypical symptoms may lead to excessively delayed
diagnosis. Festen et al. report a delay in diagnosis up to 160 days after birth (4). Patients
present with either ileus or symptoms as in typical Hirschsprung's disease but additionally
with recurrent vomiting. In patients presenting with ileus, diagnosis may be delayed for
several weeks because causative factors like volvulus or meconium ileus do not primarily
warrant investigations for aganglionosis. Furthermore, TCA may be associated with other
anomalies of the gastrointestinal tract. Only a few reports of TCA associated with small bowel
atresia and volvulus can be found (3, 7). Lally et al. report anastomotic failure following repair
of ileal atresia due to underlying extensive aganglionosis (11). In cases of midgut volvulus
without malrotation, aganglionosis has to be ruled out. Stringer et al. recently published a
series of seven full-term infants with meconium ileus due to extensive intestinal aganglionosis
(18). Neonatal appendicitis, a very rare disease, may be the leading symptom of TCA.
Therefore, rectal biopsies are mandatory in those cases. Ratta et al. state that lack of
awareness of the condition may lead to delay in diagnosis and inappropriate treatment (15).
Additional to the diagnostic problems due to atypical and heterogenic symptoms,
histochemical examination of rectal biopsies may prove negative or equivocal because
increased acetylcholinesterase activity may not be present in TCA (5,10,12). Furthermore,
there is no typical radiographic pattern (13,17). Plain abdominal radiographs usually suggests
low bowel obstruction whereas barium enema usually does not show pathognomonic
features.
If no mechanical obstruction is found at laparotomy in neonates presenting with ileus, it is
suggested to resect the appendix to rule out TCA. If rectal mucosal biopsies are negative or
equivocal, biopsies should be repeated or a formal sphincterectomy for thorough analysis is
done.
The second period lasts from the raising of stoma to its closure, including the definite
surgical procedure. Failure to thrive and excessive fluid losses have been reported in patients
with ileostomies (2). Post-ileostomy complications, however, have been eliminated after the
importance of oral sodium supplementation to maintain the enteral co-transport system has
been realized (16). In the series of Cass & Myers, ileostomy dysfunction was common with a
20% prolapse rate and 25% rate of persistent excessive losses (2). Interestingly, right
transversostomies may show a good function even in cases of TCA. Therefore, frozen section
biopsies are mandatory when raising a stoma.
The definitive surgical procedure has been debated (2,5,8,9,15). Colonic patch graft
procedures were the first proposals for surgical management of TCA (14). The rational behind
were to use the distinctive resorptive function of part of the aganglionic colon (6). Use of the
right colon has the theoretical advantage of improved water resorption. However the colon
patch procedures have significant complications, e.g., enterocolitis, ulceration of the
aganglionic pouch, perforations and extreme dilatation. Multiple modifications of the
technique have been reported but none were superior (1,8,9,18). Actually, a modified
Duhamel's pull-through procedure seems favorable in the treatment of TCA (2,15).
The third critical phase begins with closure of the stoma. Complications in this period are
predominantly recurrent episodes of sub-ileus and diarrhea or nocturnal incontinence. The
cause for sub-ileus is a raised tone in the residual sphincter. Repeated manual anal dilatations

may be mandatory. The treatment of diarrhea may be managed by diets and/or Loperamide
often in large doses. Side effects of large doses of Loperamide are mental irritability and
dyskinesia.
Significantly better survival of the patients with TCA nowadays is mainly attributed to more
accurate diagnosis and improved management of infants with ileostomies.
References:
1. Boley SJ: A new approach to total aganglionosis of the colon. Surg Gynecol Obstet 159:481-484, 1984
2. Cass DT, Myers N: Total colonic aganglionosis: 30 years' experience. Pediatr Surg Int 2:68-75, 1987
3. Fekete CN, Ricour C, Martelli H: Total colonic aganglionosis (with or without ileal involvement): A review of 27 cases. J
Pediatr Surg 21:251-254, 1986
4. Festen C: Total colonic aganglionosis: A diagnostic problem. Z Kinderchir 27:330-337, 1979
5. Festen C, Severijner R, v.d. Staak F: Total colonic aganglionosis: Treatment and follow-up. Z Kinderchir 44:153-155, 1989
6. Heath AL, Spitz L, Milla PJ: The absorptive function of colonic aganglionoic intestine: Are the Duhamel and Martin
procedures rational? J Pediatr Surg 20:34-36, 1985
7. Ikeada K, Goto S: Total colonic aganglionosis with or without small bowel involvement: An analysis of 137 patients. J
Pediatr Surg 21:319-322, 1986
8. Kimura K, Nishijima E, Muraji T: A new surgical approach to extensive aganglionosis. J Pediatr Surg 16:840-843, 1981
9. Kimura K, Nishijima E, Muraji T: Extensive aganglionosis: Further experience with the colonic patch graft procedure and
long-term results. J Pediatr Surg 23:52-56, 1988
10. Kurer MH, Lawson JON, Pambakian H: Suction biopsy in Hirschsprung's disease. Arch Dis Child 61:83-84, 1986
11.Lally KP, Chwals WJ, Weitzman JJ: Hirschsprung's disease: A possible cause of anastomotic failure following repair of
intestinal atresia. J Pediatr Surg 27:469-470, 1992
12. Lister J, Tam PKH: Hirschsprung's disease. In: Neonatal Surgery, Hrsg. Lister J, Irving IM, Burrterworth, London,
p.523-546, 1990
13. Louw JH: Total colonic aganglionosis. Can J Surg 21:397-405, 1971
14.Martin L: Surgical management of Hirschsprung's disease involving the small intestine. Arch Surg 97:183-189, 1968
15.Ratta BS, Kiely EM, Spitz L: Improvements in the management of total colonic aganglionosis. Pediatr Surg Int 5:30-36,
1990
16. Sacher P, Hirsig J, Gresser J et al: The importance of oral sodium replacement in ileostomy patients. Progr Pediatr Surg
24:226-231, 1989
17. Swenson O, Sherman JO, Fisher JH: Diagnosis of congenital megacolon: an analysis of 501 patients. J Pediatr Surg
7:587-594, 1973
18. Stringer MD, Brereton RJ, Drake DP: Meconium ileus due to extensive intestinal aganglionosis. J Pediatr Surg
29:501-503, 1994

* Author:
Peter Sacher, MD
Pediatric Surgical Department
University Children's Hospital
Zurich Switzerland
Back to Index
IMPERFORATE ANUS
by: Adrian M. Viens, MS
University of Toronto
Imperforate anus (IA) is a congenital anomaly in which the natural anal opening is
absent. Diagnosis of IA is usually made shortly after birth on routine physical examination.
The incidence of IA is approximately 1 in 4000-5000 live births and it is more common in
males. Its etiology is unknown and it runs equally through all racial, cultural and
socio-economic groups. There is preliminary evidence (> 5 case reports) of the existence of
autosomal inheritance (both dominant and recessive) in patients with anorectal
malformations.
IA is classified as either "high" or "low" depending on the termination of the distal
rectum. When the rectum ends above the levator muscles the malformations are classified as
high, and when the rectum ends below the levator muscles the malformations are classified as
low. High lesions are more frequent in males, low ones in females. Determination of the level
of the lesion (by abdominal x-ray or perineal ultrasound) is critical for appropriate
management. Children who have IA may also have other congenital anomalies. The acronym
VACTERL describes the associated problems that infants with IA may have: Vertebral defects,
Anal atresia, Cardiac anomalies, Tracheoesophageal fistula, Esophageal atresia, Renal
anomalies, and Limb anomalies.
Repair of low IA is relatively simple and is usually treated with perineal anoplasty;

however, repair of high IA is more complex. Patients are initially given a temporary colostomy
and time is given to allow the child to grow. A pull-through operation is completed at a later
date. Independent of the level of the lesion, the goal of the surgery is the creation of adequate
nerve and muscle structures around the rectum and anus to provide the child with the
capacity for bowel control.
MALES: most important decision in the initial management of Imperforate Anus (IA)
male patient during the neonatal period is whether the baby needs a colostomy and/or another
kind of urinary diversion procedure to prevent sepsis or metabolic derangements. Male
patients will benefit from perineal inspection to check for the presence of a fistula (wait 16-24
hours of life before deciding). During this time start antibiotherapy, decompress the GI tract,
do a urinalysis to check for meconium cells, and an ultrasound of abdomen to identify
urological associated anomalies. Perineal signs in low malformations that will NOT need a
colostomy are: meconium in perineum, bucket-handle defect, anal membrane and anal
stenosis. These infants can be managed with a perineal anoplasty during the neonatal period
with an excellent prognosis. Meconium in urine shows the pt has a fistula between the rectum
and the urinary tract. Flat "bottom" or perineum (lack of intergluteal fold), and absence of anal
dimple indicates poor muscles and a rather high malformation needing a colostomy. Patients
with no clinical signs at 24 hours of birth will need a invertogram or cross-table lateral film in
prone position to decide rectal pouch position. Bowel > 1 cm from skin level will need a
colostomy, and bowel < 1 cm from skin can be approach perineally. Those cases with high
defect are initially managed with a totally diverting colostomy. Diverting the fecal stream
reduces the chances of genito-urinary tract contamination and future damage.
FEMALES: most frequent defect in females patient with imperforate anus (IA) is
vestibular fistula, followed by vaginal fistulas. In more than 90% of females cases perineal
inspection will confirm the diagnosis. These infants require a colostomy before final
corrective surgery. The colostomy can be done electively before discharge from the nursery
while the GI tract is decompressed by dilatation of the fistulous tract. A single orifice is
diagnostic of a persistent cloacal defect usually accompany with a small-looking genitalia.
Cloacas are associated to distended vaginas (hydrocolpos) and urologic malformations. This
makes a sonogram of abdomen very important in the initial management of these babies for
screening of obstructive uropathy (hydronephrosis and hydroureter). Hydrocolpos can cause
compressive obstruction of the bladder trigone and interfere with ureteral drainage. Failure to
gain weight and frequents episodes of urinary tract infections shows a poorly drained urologic
system. A colostomy in cloacas is indicated. 10% of babies will not pass meconium and will
develop progressive abdominal distension. Radiological evaluation will be of help along with a
diverting colostomy in this cases. Perineal fistulas can be managed with cutback without
colostomy during the neonatal period.
The most important prognostic characteristic is the severity of the IA. Patients with low
IA have a good probability of having normal stool patterns. Patients with high IA report more
problems such as fecal incontinence and constipation. For patients who cannot maintain
normal bowel function, the use of a special diet, underpants liners, enemas and drugs have
ameliorated their lives. Long-term follow up (with both qualitative and quantitative quality of
life considerations) of these patients is very important. In addition, the use of anal
endosonography and/or manometry can be used as objective measurements of anorectal
pressure and sphincter function.
References
1- Chen CJ: The treatment of imperforate anus: experience with 108 patients. J Pediatr Surg 34(11):1728-32, 1999.
2- Landau D, Mordechai J, Karplus M, Carmi R: Inheritance of familial congenital isolated anorectal malformations: case
reports and review. Am J Med Genetics 71:280-282, 1997.
3- Rintala RJ, Lindahl HG, Rasanen M: Do children with repaired low anorectal malformations have normal bowel function? J
Pediatr Surg 32(6):823-826, 1997.
4- Pea A: Anorectal malformations. Semin Pediatr Surg 4(1):35-47, 1995
5- Pea A: Management of anorectal malformations during the newborn period. World J Surg 17(3):385-92, 1993
6- Pea A: Posterior sagittal approach for the correction of anorectal malformations. Adv Surg 19:69-100, 1986
7- Pea A: Surgical treatment of high imperforate anus. World J Surg 9(2):236-43, 1985
8- deVries PA, Pea A: Posterior sagittal anorectoplasty. J Pediatr Surg 17(5):638-43, 1982

Back to Index

H. Duplications

Duplications of the gastrointestinal tract are considered uncommon congenital

anomalies usually diagnosed or unexpectedly encountered intraoperatively
during the first two years of life. The duplicated bowel can occur anywhere in
the GI tract, is attached to the mesenteric border of the native bowel, shares a
common wall and blood supply, coated with smooth muscle, and the epithelial
lining is GI mucosa. May contain ectopic gastric or pancreatic tissue. Most are
saccular, other tubular. Theories on their origin (split notochord syndrome,
twining, faulty solid-stage recanalization) do not explain all cases of duplicated
bowel. Three-fourth are found in the abdomen (most commonly the ileum and
jejunum), 20% in the thorax, the rest thoraco-abdominal or cervical. Symptoms
vary according to the size and location of the duplication. Clinical
manifestations can range from intestinal obstruction, abdominal pain, GI
bleeding, ulceration, or mediastinal compression. Ultrasound confirms the
cystic nature of the lesion (muscular rim sign) and CT the relationship to
surrounding structures. Management consist of surgical excision avoiding
massive loss of normal bowel and removing all bowel suspect of harboring
ectopic gastric mucosa.
Back to Index
I. Intussusception
Although intussusception can occur at any age, the greatest incidence occurs in infants
between 4-10 months of age. Over half of the cases are in the first year of life. Frequently
occurs after a recent upper respiratory infection, by Adenovirus type 3 that causes a reactive
lymphoid hyperplasia that act as lead point (of Peyer's patch).
A definite lead point is identified in about 5% of patients. These include: Meckel's
diverticulum, polyps, Henoch's Schonlein purpura, hematoma, lymphoma, foreign bodies, and
duplications. Most children have no lead point and it is felt that enlarged mesenteric nodes or
swollen Peyer's patches may be the cause. The baby has intermittent periods of severe
discomfort with screaming, stiffening and drawing up of the legs, followed by periods of rest.
Vomiting may occur and bloody, mucoid (currant jelly) stool may be passed. The baby may
become dehydrated and appear acutely ill. Frequently, lethargy may be an early sign. The
diagnosis is made by barium enema, and hydrostatic reduction of the intussusception with
barium is successful in approximately 50% of cases. To be successful, the barium must reflux
into the terminal ileum. The surgeon should be notified before an attempt at barium reduction,
and should be present at the time of study. Recently the use of gas enema reduction has been
successful in patients with: (1) symptoms less than 12 hours, (2) no rectal bleeding, (3)
absence of small bowel obstruction, and (4) normally hydrated. Ultrasonography can be used
as a rapid sensitive screening procedure in the initial diagnosis of intussusception. Previous
adverse clinical features that precluded barium reduction can be replaced during gas
reduction. Predictors of failure of reduction are: (1) ileocolic intussusception, (2) long duration
of symptoms, (3) rectal bleeding, and (4) failed reduction at another institution. Air reduction
(pneumocolon) is a very effective alternative method since it brings less radiation (shorter
flouroscopy time), less costs and less morbidity in cases of perforations.
Failure of hydrostatic reduction requires urgent operation through a right lower quadrant
horizontal incision. The intussusception is reduced by pushing on the distal bowel like a tube
of toothpaste rather than pulling the proximal bowel. Most cases are ileo-colic
intussusception, and a few are jejuno-jejunal or ileo-ileal intussusception.
The traditional method of diagnosing and managing ileo-colic intussusception is barium
enema contrast reduction. In China where this is the most common surgical emergency in
childhood, pneumatic reduction has been used for more than 25 years. A recent tendency
toward this approach is seen in recent years in Occident. This consist of rectal insufflation of
oxygen at a flow rate of 2 L/min, controlling pressure by adjusting the height of the mercury
column, and using maximal pressures of 80 mm Hg. Small bowel aeration is a sign of
complete reduction. Series are successful in 70-90% of cases. Gas enema reduction is very
successful in patients with: (1) symptoms less than 12 hours, (2) no rectal
bleeding, (3) absence of small bowel obstruction, and (4) normally hydrated. Ultrasonography

can be
used as a rapid sensitive screening procedure in the initial diagnosis of intussusception.
Previous
adverse clinical features that precluded barium reduction can be replaced during gas
reduction.
Predictors of failure of reduction are: (1) ileocolic intussusception, (2) long duration of
symptoms, (3)
rectal bleeding, and (4) failed reduction at another institution. Air reduction (pneumocolon) is a
very
effective alternative method since it brings less radiation (shorter flouroscopy time), less
costs and less
morbidity in cases of perforations.
J. APPENDICITIS
By: Antonio Carlos Sansevero Martins, MD *
INTRODUCTION
The most common cause of emergency laparotomies in all age groups, acute appendicitis
remains a diagnostic challenge, with high perforation rates at laparotomy, and, curiously, high
negative laparotomy rates. With current standard approach and use of antimicrobial
medication the overall mortality is almost zero, and major complications are gradually
declining.
ETIOLOGY
Appendiceal lumen obstruction by a fecalith, lymphoid hyperplasia, carcinoid tumor,
anomalous vessels, or intestinal parasites as Ascaris lumbricoides leads to acute increase in
intraluminal pressure with consequent ischemia and mucosal damage. In association with
bacterial proliferation this may lead to gangrene and perforation.
DIAGNOSIS
Early diagnosis is the key for success in management of appendicitis, and it is based solely
upon history and physical findings. Image and laboratory studies are of relative value.
Signs and Symptoms
Abdominal pain is the chief complaint of appendicitis. It is usually periumbilical at onset,
colicky due to lumen obstruction and distension (visceral referred pain), and in a few hours
shifts its location to the RLQ changing to a continuous and intense pattern, explained by
parietal irritation caused by the inflamed appendix exudate. Special concern must be given to
patients with initial periumbilical pain who localize it into uncommon sites. One must always
remember that the localized pain in appendicitis is directly related to where it lays, so we can
have flank or back pain in retrocecal appendicitis, dysuria in retro vesical cases, etc. Vomiting,
generally after the pain, is usually present in appendicitis. Bowel habits are generally
unchanged in early cases, but diarrhea can be present initially in up to 10% of cases, usually
associated to pelvic appendicitis.
Physical Examination
Simple examination can predict appendicitis when we see the child walking, bent over his
right hip, and stepping carefully to avoid minimum shaking. The apprehensive little patient lies
on the examining table in a supine position, with a slight flexion of the right leg. When asked
to show the pain site, he points out with a finger. Abdominal examination should be done
gently, divided in quadrants, and beginning distant from the site of complaint. It always helps
if the patient is asked to help in the examination, the surgeon palpating over his hand, to
reduce voluntary guarding. Point tenderness and/or appendiceal masses at RLQ are
diagnostic, but unfortunately, they are not present in a great deal of cases. Peritoneal irritation
signs can be assessed without performing the classic, Blumberg maneuver, just by soft right
heel percussion, or gentle Mc Burney's point finger percussion. In advanced cases with
"wooden abdomen" and clear infectious signs, the diagnosis is obvious.
Image and Laboratory
Image resources are poor in diagnosing appendicitis. Plain abdominal films can show a
fecalith (20%), a dilated and fixed ileal loop (sentinel loop) and indirect signs such as scoliosis,
psoas line and/or pre-peritoneal line blurring. Ultrasound, very good to assess collections, has
gained popularity, as far as more experience has been achieved, and modern devices have
been developed. In some series, its accuracy has been proved in 90% of cases, but it is still an
operator-dependent exam, with a fail in at least 10 to 15% of negative findings, even with color
Doppler scans. No lab test can confirm the diagnosis of appendicitis. Dueholm et al could
predict 100% normal appendices when WBC and C-reactive protein were normal, but no
inverse relation could be proved. The urinalysis is important to assess possible infection or

calculi migration. Massive pyuria usually preannounces urinary infection, but discrete findings
can be due to appendicitis.
TREATMENT
Surgical Management
Standard appendectomy through a Babcock-Davis muscle splitting approach is a safe, quick
and efficient technique to manage appendicitis. The stump can be inverted, but nowadays
simple ligation with electrocauterization is the procedure of choice. In complicated cases, with
perforation and peritonitis, exhaustive cavity lavage with saline should be accomplished. No
improvement is shown by cavity antibiotic irrigation. Drainage is controversial and we do not
use drains for appendicitis. Abdominal wall is approximated with Smith-Jones sutures.
Primary skin closure is performed even in advanced cases. Laparoscopic appendectomy has
gained force in the 90's as a minimal invasive technique, easy visualization of the structures
even in difficult sites, e.g., retrocecal appendices, and accurate suction of purulent material
and irrigation of the cavity with very low postoperative complication rates ( less than 1.5%).
Operative time, costs, intraoperative complications such as a bleeding, stump leakage and
visceral puncture are some limitations to be reduced with time and experience. A subgroup of
children (obese, female, athletic) benefit from the use of this surgical technique.
In patients with appendiceal mass or abscess, with no signs of peritonitis or systemic sepsis
"cooling the process" with antibiotic therapy, followed by interval appendectomy (operating
on the patient on an elective basis free from inflammation) can be done with relative security.
Antibiotic Therapy
Prophylactic antibiotic is given one hour before surgery and continued for 24 hours in
uncomplicated appendicitis, and must be continued for at least five to seven days, in cases of
a complication ( perforation, peritonitis, abscess ). Triple combination of ampicillin (200
mg/Kg/day), gentamicin( 5 mg/Kg/day) and clindamycin ( 40 mg/Kg/day) has shown the best
results in treating infection and preventing septic complications.
COMPLICATIONS
Major complications as intra abdominal abscesses, small bowel obstruction and sepsis, have
declined to an overall rate of 7%, in well controlled studies. Minor ones such as wound
infection to a rate of less than 2%.
THE "NORMAL APPENDIX"
It has been common knowledge that a diagnostic error of 10 to 15% could be acceptable when
compared with the risks of appendicitis, and some series stills carry this unacceptable rate,
with no decrease in the complication rates, compared with other series. In the first 24 hours,
close attention, and short period reexaminations, added to critical interpretation of image and
lab studies will help decrease unnecessary surgery with no increase in complication rates.
* AUTHOR
Antonio Carlos Sansevero Martins, MD - Pediatric Surgeon - Pediatric Emergency Unit
Coordinator - Pediatric Surgery Resident Program Coordinator - Department of Pediatrics,
Hospital de Clnicas de Uberlandia Universidade Federal de Uberlandia - Minas Gerais - Brasil
REFERENCES
1. Cloud DT: Appendicitis. In Aschcraft KW , Holder TM( eds):Pediatric Surgery. W.B. Saunders
Company, Mexico City, pp 470-477, 1993.
2. El Ghoneimi et al: Laparoscopic appendectomy in children: report of 1,379 cases. J Ped
Surg. 29(6):786-789,1994.
3. Gilchrist BF et al: Is there a role for laparoscopic appendectomy in pediatric surgery? J Ped
Surg 27(2):209-214, 1992.
4. Kellnar St, Trammer A, Till H, Lochbhler H: Endoscopic appendectomy in childhood technical aspects. Eur J Pediatr Surg 4: 341-343, 1994
5. Lund DP, Murphy EU: Management of perforated appendicitis in children: a decade of
aggressive treatment. J Ped Surg 29(8):1130-1134, 1994
6. Martins ACS, Abreu N, Oliveira Jr DF et al. Apendicite aguda: experiencia de 7 anos em um
Hospital Universitrio. Presented at theXVI Congresso Brasileiro de Cirurgia Peditrica.
November, 1995. Vitoria, ES, Brazil.
7. Mosdell DM, Morris DM, Fry DE: Peritoneal cultures and antibiotic therapy in pediatric
perforated appendicitis. Am J Surg 167( March): 313-316, 1994.
8. Putnam TC, Gagliano N, Emmens RW: Appendicitis in children. Surg Gyn Obst 170(June):
527-532, 1990.
9. Quillin SP, Siegel MJ: Appendicitis: efficacy of color Doppler sonography. Radiology 191:
557-560, 1994.
10. Varlet F, Tardieu D, Limonne B, Metafiot H, Cavrier Y: Laparoscopic versus open
appendectomy in children- comparative study of 403 cases.
11. Wang Y, Reen DJ, Puri P: Is a histologically normal appendix following emergency

appendectomy always normal? Lancet 347(20): 1076-1079, 1996.

12. Wong ML, Casey SO et al: Sonographic diagnosis of acute appendicitis in children. J Ped
Surg 29(10): 1356-1360, 1994.
Last update: September 1996
Back to Index

K.Chronic Intestinal Pseudo-Obstruction

Chronic Intestinal Pseudo-Obstruction is a rare disorder of intestinal motility
in infants and children characterized by recurrent attacks of abdominal pain,
distension, vomiting, constipation and weight loss in the absence of obvious
mechanical lesions. The disease can be familial or sporadic. Suggested
etiology is degeneration of enteric nervous or smooth muscle cells. The
diagnosis is based on history, physical exam, radiographies and motility
studies. X-Ray hallmarks are: absent strictures, absent, decreased or
disorganized intestinal motility, and dilated small/large bowel loops.
Associated conditions identified in 10-30% of patients are bladder dysfunction
(megacystis) and neurological problems. Histologic pattern portrayed:
myenteric plexus hyperplasia, glial cell hyperplasia, and small ganglion cells
(hypoganglionosis). Management is primary supportive: intestinal
decompression (NG), long-term TPN and antibiotic prophylaxis. Motility agents
are unsuccessful. Venting gastrostomy with home parenteral nutrition has
shortened the high hospitalization rate associated to this disease process. A
similar condition can be seen in early fed prematures due to immaturity of
intestinal motility.
Back to Index

L.Bezoars
Bezoars are rare foreign body concretions formed in the stomach and small
bowel composed mainly of hair (tricho), vegetable matter (phyto) or milk curds
(lacto). Most cases are females children, 6-10 years old, with bizarre appetite
(trichophagia) and emotional disturbances. Originally the mass forms in the
stomach and can move to the small bowel by fragmentation, extension or total
translocation. Diagnosis can be confirmed by UGIS, CT-Scan or endoscopy.
The child can develop an asymptomatic palpable abdominal mass, pain,
obstruction or perforation. Other children will reduce intake and develop
weight loss. Predisposing conditions to bezoar formations are: gastric
dymotility and decreased acidity. Management can consist of mechanical or
pulsating jet of water fragmentation via the endoscope, operative extraction,
shock-wave lithotripsy (ESWL) with subsequent evacuation, or dissolution by
oral ingestion of proteolytic enzymes (papain, acetylcysteine, cellulase). With
ESWL the shock wave pressure needed is less than half used for urolithiasis
cases.
Back to Index
M. Neuronal Intestinal Dysplasia
Intestinal Neuronal Dysplasia (IND) is a colonic motility disorder first described in 1971 by
Meier-Ruge associated to characteristic histochemical changes of the bowel wall (hyperplasia

of submucous & myenteric plexus with giant ganglia formation, isolated ganglion cells in
lamina propia and muscularis mucosa, elevation of acetylcholinesterase in parasympathetic
fiber of lamina propia and circular muscle, and myenteric plexus sympathetic hypoplastic
innervation), also known as hyperganglionosis associated to elevated acetylcholinesterase
parasympathetic staining. The condition can occur in an isolated form (either localized to
colon or disseminated throughout the bowel), or associated to other diseases such as
Hirschsprung's (HD), neurofibromatosis, MEN type IIB, and anorectal malformations. It is
estimated that 20-75% of HD cases have IND changes proximal to the aganglionic segment.
Clinically two different types of isolated IND have been described: Type A shows symptoms of
abdominal distension, enterocolitis, bloody stools, intestinal spasticity in imaging studies (Ba
Enema) since birth, is less common and associated with hypoplasia of sympathetic nerves.
Type B is more frequent, symptoms are indistinguishable from that of HD, with chronic
constipation, megacolon, and repeated episodes of bowel obstruction. Management depends
on clinical situation; conservative for minor symptoms until neuronal maturation occurs
around the 4th year of life, colostomy and resectional therapy for life threatening situations.
Back to Index

IV. HERNIAS AND ADBOMINAL WALL DEFECTS

A. Diaphragmatic Hernias
A.1 Congenital Diaphragmatic Hernia (Bochdalek)
The most common congenital diaphragmatic hernia (CDH) is that which occurs through the
postero-lateral defect of Bochdalek. It is caused by failure of the pleuroperitoneal membrane
to develop adequately and close before the intestines returning to the abdomen at the tenth
week of gestation. The intestines then enter the pleural cavity and cause poor lung
development leading to pulmonary hypoplasia (a reduced number of alveoli per area of lung
tissue). This defect is postero-lateral in the diaphragm and may vary in size. Stomach, liver or
spleen may be partly in chest as well. Frequency is 1:2000 live births and the natural history in
prenatally diagnosed CDH is that 60% will die. The clinical presentation is that the newborn
becomes rapidly cyanotic, acidotic, and has poor ventilation. Major findings relate to the
degree of pulmonary maldevelopment. Chest films will show intestines in the chest. Left sided
hernias are more common than right (90% on left). Placement of a radiopaque nasogastric
tube may show the tube coiled in the lower left chest. Higher risk factors are: early appearance
of symptoms in life, prematurity and associated anomalies. Treatment consist of rapid
intubation and ventilation with use of muscle relaxants, placement of a nasogastric tube to
prevent gaseous distension of the intestines and preoperative stabilization of arterial blood
gases and acid-base status. Surgery can be undertaken when one of the following objectives
are met: (1) blood gases normalize with no significant changes between preductal and
postductal samples, (2) echocardiogram demonstrate reduce pulmonary pressure and
pulmonary peripheral resistance.
Operative management consist of abdominal approach, closure of hernia by primary repair or
use of mesh, and correction of malrotation. Postoperative management is very difficult. Due to
hypoplastic lungs, there is frequently pulmonary hypertension leading to right-to-left shunting
and progressive hypoxemia, hypercarbia, and acidosis that worsens the pulmonary
hypertension. The use of chest tubes may cause overstretching of the already hypoplastic
alveoli causing: increase pulmonary hypertension, reduce functional residual capacity and
reduce lung compliance. Postoperatively, the infant should be kept paralyzed and ventilated
and only very slowly weaned from the ventilator. The severity of pulmonary hypoplasia, both
ipsilaterally and contralaterally, is the main determinant of outcome. ECMO (extracorporeal
membrane oxygenator) has come to reduce somewhat the mortality of this condition.
The mortality of CDH is directly related to the degree of lung hypoplasia associated. Death is
caused by persistent pulmonary hypertension and right ventricular failure. Prospective studies
of prenatally diagnosed fetus prior to 25 wk. gestation has shown that 60% will die despite
optimal postnatal care. This unsolved problem has prompted investigators to develop new
treatment options such as preoperative stabilization, jet-frequency ventilation, and ECMO.
Another area of development is intrauterine fetal surgical repair. To achieve success fetal
surgery should: (1) pose no risk to the mother (innocent bystander) or her future reproductive

capacity; (2) tocolytic therapy in the post-op weeks should proved effective to avoid prenatal
stillbirths; and (3) the procedure should be superior to conventional therapy. Intrauterine
repair has meet with limited success due to herniation of the fetal liver into the chest through
the defect. Disturbance of the umbilical circulation during or after liver reduction causes fetal
death. Positive-pressure ventilation after birth reduces the liver before the baby comes for
surgical repair. Dr. Harrison (USFC Fetal Treatment Center) has devised separate fetal
thoraco-abdominal incisions to deal with this problem ("two-step dance"), reducing or
amputating the left lateral segment of the liver. Another less invasive approach is enlarging
the hypoplastic lungs by reducing the normal egress of fetal lung fluid with controlled tracheal
obstruction called PLUGS (Plug Lung Until it Grows).
Delayed presentation beyond the neonatal period is rare, estimated to occur in 4-6% of cases.
Infants and children will present with either respiratory or gastrointestinal symptoms such as:
chronic respiratory tract infection, vomiting, intermittent intestinal obstruction, and feeding
difficulty. Occasionally the child is asymptomatic. The small size of the defect protected by
either the spleen or the liver and the presence of a hernial sac may delay the intestinal
herniation into the chest. A rise intrabdominal pressure by coughing or vomiting transmitted
to any defect of the diaphragm makes visceral herniation more likely. Diagnosis is confirmed
by chest or gastrointestinal contrast imaging. Management consists of immediate surgery
after preop stabilization. Most defects can be closed primarily through an abdominal
approach. Chest-tube placement in the non-hypoplastic lung is of help. Surgical results are
generally excellent. A few deaths have resulted from cardiovascular and respiratory
compromise due to visceral herniation causing mediastinal and pulmonary compression.
A.2 Morgagni Hernias
First described in 1769, Morgagni Hernias (MH) are rare congenital diaphragmatic defects
close to the
anterior midline between the costal and sternal origin of the diaphragm. They occur
retrosternally in the
midline or more commonly on either side (parasternally) of the junction of the embryologic
septum
transversum and thoracic wall (see the figure) representing less than 2% of all diaphragmatic
defects.
Almost always asymptomatic, typically present in older children or adults with minimal
gastrointestinal
symptoms or as incidental finding during routine chest radiography (mass or air-fluid levels).
Infants
may develop respiratory symptoms (tachypnea, dyspnea and cyanosis) with distress. Cardiac
tamponade due to protrusion into the pericardial cavity has been reported. The MH defect
contains a
sac with liver, small/ large bowel as content. Associated conditions are: heart defects, trisomy
21,
omphalocele, and Cantrells' pentalogy. US and CT-Scan can demonstrate the defect.
Management is
operative. Trans-abdominal subcostal approach is preferred with reduction of the defect and
suturing of
the diaphragm to undersurface of sternum and posterior rectus sheath. Large defects with
phrenic
nerve displacement may need a thoracic approach. Results after surgery rely on associated
conditions.
A.3 Esophageal Hernias
Two types of esophageal hernia recognized are the hiatal and paraesophageal hernia.
Diagnosis is
made radiologically always and in a number of patients endoscopically. The hiatal hernia (HH)
refers to
herniation of the stomach to the chest through the esophageal hiatus. The lower esophageal
sphincter
also moves. It can consist of a small transitory epiphrenic loculation (minor) up to an
upside-down
intrathoracic stomach (major). HH generally develops due to a congenital, traumatic or
iatrogenic factor.

Most disappear by the age of two years, but all forms of HH can lead to peptic esophagitis
from
Gastroesophageal reflux. Repair of HH is determined by the pathology of its associated reflux
(causing
failure to thrive, esophagitis, stricture, respiratory symptoms) or the presence of the stomach
in the
thoracic cavity. In the paraesophageal hernia (PH) variety the stomach migrates to the chest
and the
lower esophageal sphincter stays in its normal anatomic position. PH is a frequent problem
after
antireflux operations in patients without posterior crural repair. Small PH can be observed.
With an
increase in size or appearance of symptoms (reflux, gastric obstruction, bleeding, infarction or
perforation) the PH should be repaired. The incidence of PH has increased with the advent of
the
laparoscopic fundoplication.
PROCESUS VAGINALIS REMNANTS
B.1 Inguinal Hernias
A hernia is defined as a protrusion of a portion of an organ or tissue through an abnormal
opening. For groin (inguinal or femoral) hernias, this protrusion is into a hernial sac. Whether
or not the mere presence of a hernial sac (or processus vaginalis) constitutes a hernia is
debated. Inguinal hernias in children are almost exclusively indirect type. Those rare instances
of direct inguinal hernia are caused by previous surgery and floor disruption. An indirect
inguinal hernia protrudes through the internal inguinal ring, within the cremaster fascia,
extending down the spermatic cord for varying distances. The direct hernia protrudes through
the posterior wall of the inguinal canal, i.e., medial to deep inferior epigastric vessels,
destroying or stretching the transversalis fascia. The embryology of indirect inguinal hernia is
as follows: the duct descending to the testicle is a small offshoot of the great peritoneal sac in
the lower abdomen. During the third month of gestation, the processus vaginalis extends
down toward the scrotum and follows the chorda gubernaculum that extends from the testicle
or the retroperitoneum to the scrotum. During the seventh month, the testicle descend into the
scrotum, where the processus vaginalis forms a covering for the testicle and the serous sac in
which it resides. At about the time of birth, the portion of the processus vaginalis between the
testicle and the abdominal cavity
obliterates, leaving a peritoneal cavity separate from the tunica vaginalis that surrounds the
testicle.
Approximately 1-3% of children have a hernia. For infants born prematurely, the incidence
varies from 3-5%. The typical patient with an inguinal hernia has an intermittent lump or bulge
in the groin, scrotum, or labia noted at times of increased intra-abdominal pressure. A
communicating hydrocele is always associated with a hernia. This hydrocele fluctuates in size
and is usually larger in ambulatory patients at the end of the day. If a loop of bowel becomes
entrapped (incarcerated) in a hernia, the patient develops pain followed by signs of intestinal
obstruction. If not reduced, compromised blood supply (strangulation) leads to perforation
and peritonitis. Most incarcerated hernias in children can be reduced.
The incidence of inguinal hernia (IH) in premature babies (9-11%) is higher than full-term
(3-5%), with a dramatic risk of incarceration (30%). Associated to these episodes of
incarceration are chances of: gonadal infarction (the undescended testes complicated by a
hernia are more vulnerable to vascular compromise and atrophy), bowel obstruction and
strangulation. Symptomatic hernia can complicate the clinical course of babies at NICU ill with
hyaline membrane, sepsis, NEC and other conditions needing ventilatory support. Repair
should be undertaken before hospital discharge to avoid complications. Prematures have:
poorly developed respiratory control center, collapsible rib cage, deficient fatigue-resistant
muscular fibers in the diaphragm that predispose then to potential life-threatening post-op
respiratory complications such as: need of assisted ventilation (most common), apnea and
bradycardia, emesis, cyanosis and re-intubation (due to laryngospasm). Independent risk
factors associated to this complications are (1) history of RDS/bronchopulmonary dysplasia,
(2) history of patent ductus arteriosus, (3) low absolute weight (< 1.5 Kg), and (4) anemia (Hgb
< 10 gm- is associated to a higher incidence of post-op apnea). Postconceptual age (sum of
intra- and extrauterine life) has been cited as the factor having greatest impact on post-op

complications. These observation makes imperative that preemies (with post conceptual age
of less than 45 weeks) be carefully monitored in-hospital for at least 24 hours after surgical
repair of their hernias. Outpatient repair is safer for those prematures above the 60 wk. of
postconceptual age. The very low birth weight infant with symptomatic hernia can benefit from
epidural anesthesia.
At times, the indirect inguinal hernia will extend into the scrotum and can be reduced by
external, gentle pressure. Occasionally, the hernia will present as a bulge in the soft tissue
overlying the internal ring. It is sometimes difficult to demonstrate and the physician must rely
on the patient's history of an intermittent bulge in the groin seen with crying, coughing or
straining.
Elective herniorrhaphy at a near convenient time is treatment of choice. Since risk of
incarceration is high in children, repair should be undertaken shortly after diagnosis. Simple
high ligation of the sac is all that is required. Pediatric patients are allowed to return to full
activity immediately after hernia repair. Patients presenting with incarceration should have an
attempt at reduction (possible in greater than 98% with experience), and then admission for
repair during that hospitalization. Bilateral exploration is done routinely by most experienced
pediatric surgeons. Recently the use of groin laparoscopy through the hernial sac permits
visualization of the contralateral side.
Approximately 1% of females with inguinal hernias will have the testicular feminization
syndrome. Testicular feminization syndrome (TFS) is a genetic form of male
pseudohermaphroditism (patient who is genetically 46 XY but has deficient masculinization of
external genitalia) caused by complete or partial resistance of end organs to the peripheral
effects of androgens. This androgenic insensitivity is caused by a mutation of the gene for
androgenic receptor inherited as an X-linked recessive trait. In the complete form the external
genitalia appear to be female with a rudimentary vagina, absent uterus and ovaries. The infant
may present with inguinal hernias that at surgery may contain testes. Axillary/pubic hair is
sparse and primary amenorrhea is present. The incomplete form may represent undervirilized
infertile men. Evaluation should include: karyotype, hormonal assays, pelvic ultrasound,
urethrovaginogram, gonadal biopsy and labial skin bx for androgen receptor assay. This
patients will never menstruate or bear children. Malignant degeneration (germ cell tumors) of
the gonads is increased (22-33%). Early gonadectomy is advised to: decrease the possible
development of malignancy, avoid the latter psychological trauma to the older child, and
eliminate risk of losing the pt during follow-up. Vaginal reconstruction is planned when the
patient wishes to be sexually active. These children develop into very normal appearing
females that are sterile since no female organs are present.
B.2 Hydroceles
A hydrocele is a collection of fluid in the space surrounding the testicle between the layers of
the tunica vaginalis. Hydroceles can be scrotal, of the cord, abdominal, or a combination of the
above. A hydrocele of the cord is the fluid-filled remnant of the processus vaginalis separated
from the tunica vaginalis. A communicating hydrocele is one that communicates with the
peritoneal cavity by way of a narrow opening into a hernial sac. Hydroceles are common in
infants. Some are associated with an inguinal hernia. They are often bilateral, and like hernias,
are more common on the right than the left. Most hydroceles will resolved spontaneously by
1-2 years of age. After this time, elective repair can be performed at any time. Operation is
done through the groin and search made for an associated hernia. Aspiration of a hydrocele
should never be attempted. As a therapeutic measure it is ineffective, and as a diagnostic tool
it is a catastrophe if a loop of bowel is entrapped. A possible exception to this is the
postoperative recurrent hydrocele.
C. Undescended Testis
The undescended testis is a term we use to describe all instances in which the testis cannot
be manually manipulated into the scrotum. The testes form from the medial portion of the
urogenital ridge extending from the diaphragm into the pelvis. In arrested descent, they may
be found from the kidneys to the internal inguinal ring. Rapid descent through the internal
inguinal ring commences at approximately week 28, the left testis preceding the right.
Adequate amounts of male hormones are necessary for descent. The highest levels of male
hormones in the maternal circulation have been demonstrated at week 28. Thus, it appears
that failure of descent may be related to inadequate male hormone levels or to failure of the
end-organ to respond. The undescended testes may be found from the hilum of the kidney to
the external inguinal ring. A patent processus vaginalis or true hernial sac will be present 90%

of the time.
The undescended testis found in 0.28% of males can be palpable (80%; most at inguinal
canal), or
non-palpable (20%). Testes that can be manually brought to the scrotum are retractile and
need no
further treatment. Parents should know the objectives, indications and limitations of an
orchiopexy: that
the testis could not exist (testicular vanishing syndrome), even after descend can atrophy, that
it cannot
be fixed and removal is a therapeutic possibility. To improve spermatogenesis (producing an
adequate
number of spermatozoids) surgery should be done before the age of two. Electron microscopy
has
confirmed an arrest in spermatogenesis (reduced number of spermatogonias and tubular
diameter) in
undescended testis after the first two years of life. Other reasons to pex are: a higher
incidence of
malignancy, trauma and torsion, and future cosmetic and psychological problems in the child.
The
management is surgical; hormonal (Human Chorionic Gonadotropin) treatment has brought
conflicting
results except bilateral cases. Surgery is limited by the length of the testicular artery. Palpable
testes
have a better prognosis than non-palpable. Laparoscopy can be of help in non-palpable testis
avoiding
exploration of the absent testis.
D. Umbilical Hernias
by: Adrian M. Viens, MS
University of Toronto
An umbilical hernia is a small defect in the abdominal fascial wall in which fluid or abdominal
contents protrude through the umbilical ring. The presence of a bulge within the umbilicus is
readily palpable and becomes more apparent when the infant cries or during defecation. The
actual size of the umbilical hernia is measured by physical examination of the defect in the
rectus abdominis muscle, and not by the size of the umbilical bulge. The size of the fascial
defect can vary from the width of a fingertip to several centimetres.Embryologically, the cause
of an umbilical hernia is related to the incomplete contraction of the umbilical ring. The
herniation of the umbilicus is a result of the growing alimentary tract that is unable to fit within
the abdominal cavity. Umbilical hernias are more prevalent in females than in males and are
more often seen in patients with African heritage. The increased frequency of umbilical
hernias has also been attributed to premature babies, twins and infants with long umbilical
cords. There is also a frequent association with disorders of mucopolysaccharide metabolism,
especially Hurler's Syndrome (gargoylism).Most umbilical hernias are asymptomatic; the
decision to repair the umbilical hernia in the first years of life is largely cosmetic and is often
performed because of parental request, not because of pain or dysfunction. In the past, some
parents use to tape a coin over the umbilical bulge, however, manual compression does not
have an effect on the fascial defect.Treatment of umbilical hernia is observation. Most
umbilical hernias spontaneously close by age two, with 90% closed by age three and 95%
closed by age five. However, surgical repair is recommended if the hernia has not closed by
the age of five.If a large defects (> 2cm) remains after the age of 2, spontaneously repair in
unlikely and may be closed surgically. The incidence of incarceration (trapped intestinal loop)
is rare, even in larger defects. Females should especially have their umbilical hernia corrected
before pregnancy because of the associated increased intra-abdominal pressure that could
lead to complications. The procedure is simple and incidence of complication such as
infection is extremely rare. The repair is usually done as outpatient surgery under general
anesthetic.
References:
1-Skandalakis JE, Gray SW (eds): Embryology for surgeons: the embryological basis for treatment of congenital anomalies 2nd edition. Williams & Wilkins, Baltimore, pp. 563-67, 1994
2- Skinner MA, Grosfeld JL: Inguinal and umbilical hernia repair in infants and children. Surg Clinics of North Am 73(3):
439-49, 1993

3- Raffensperger JG (ed): Swenson's Pediatric Surgery - 5th edition. Appleton & Lange, Norwalk, Connecticut, pp.195, 1990
4- Moore KL: The developing human - 4th edition. Philadelphia, WB Saunders, pp. 231-34, 279-81, 1988
5-Woods GE: Some observations on umbilical hernias in infants. Arch Dis Child 28:450-62, 1953
6- Evans AG: The comparative incidence of umbilical hernias in colored and white infants. J Natl Med Assoc 33:158-60, 1944

E. Omphalocele
The three most common abdominal wall defect in newborns are umbilical hernia,
gastroschisis and
omphalocele. Omphalocele is a milder form of primary abdominoschisis since during the
embryonic
folding process the outgrowth at the umbilical ring is insufficient (shortage in apoptotic cell
death).
Bowel and/or viscera remains in the umbilical cord causing a large abdominal wall defect.
Defect may
have liver, spleen, stomach, and bowel in the sac while the abdominal cavity remains
underdeveloped in
size. The sac is composed of chorium, Wharton's jelly and peritoneum. The defect is centrally
localized
and measures 4-10 cm in diameter. A small defect of less than 2 cm with bowel inside is
referred as a
hernia of the umbilical cord. There is a high incidence (30-60%)of associated anomalies in
patients with
omphalocele. Epigastric localized omphalocele are associated with sternal and intracardiac
defects (i.e.,
Pentalogy of Cantrell), and hypogastric omphalocele have a high association with
genito-urinary
defects (i.e., Cloacal Exstrophy). All have malrotation. Cardiac, neurogenic, genitourinary,
skeletal and
chromosomal changes and syndromes are the cornerstones of mortality. Antenatal diagnosis
may
affect management by stimulating search for associated anomalies and changing the site,
mode or
timing of delivery. Cesarean section is warranted in large omphaloceles to avoid liver damage
and
dystocia. After initial stabilization management requires consideration of the size of defect,
prematurity
and associated anomalies. Primary closure with correction of the malrotation should be
attempted
whenever possible. If this is not possible, then a plastic mesh/silastic chimney is fashioned
around the
defect to cover the intestinal contents and the contents slowly reduced over 5-14 days.
Antibiotics and
nutritional support are mandatory. Manage control centers around sepsis, respiratory status,
liver and
bowel dysfunction from increased intraabdominal pressure.
F. Gastroschisis
Gastroschisis is a congenital evisceration of part of the abdominal content through an
anterior
abdominal wall defect found to the right of the umbilicus. The protruding gut is foreshortened,
matted,
thickened and covered with a peel. In a few babies (4 to 23%) an intestinal atresia (IA) further
complicates the pathology. IA complicating gastroschisis may be single or multiple and may
involve the
small or large bowel. The IA might be the result of pressure on the bowel from the edge of the
defect
(pinching effect) or an intrauterine vascular accident. Rarely, the orifice may be extremely
narrow
leading to gangrene or complete midgut atresia. In either case the morbidity and mortality of
the child is
duplicated with the presence of an IA. Management remains controversial. Alternatives

depend on the
type of closure of the abdominal defect and the severity of the affected bowel. With primary
fascial
closure and good-looking bowel primary anastomosis is justified. Placement of a silo calls for
delayed
resection performing a second look operation at a later stage to save intestinal length. Angry
looking
dilated bowel prompts for proximal diversion, but the higher the enterostomy the greater the
problems
of fluid losses, electrolyte imbalances, skin excoriation, sepsis and malnutrition. Closure of
the defect
and resection with anastomosis two to four weeks later brings good results. Success or
failure is
related to the length of remaining bowel more than the specific method used.

V. GASTROINTESTINAL BLEEDING
A. Upper GI bleeding (Neonate)
Initially do an Apt test to determine if blood comes from fetal origin or maternal origin (blood
swallowed by the fetus). If its of fetal origin then consider a coagulation profile (PT,PTT). If this
coagulation profile is normal the possibilities are either stress gastritis or ulcer disease. If the
coagulation profile is abnormal then consider hematologic disease of the newborn and
manage with vitamin K. The apt test is performed by mixing 1 part of vomitus with 5 part H2O,
centrifuge the mixture and remove 5 ml (pink). Then add 1 ml 1% NaOH, wait 2 minutes and if it
remains pinks is fetal blood, if it turns brown-yellow its maternal blood.
B. Lower GI bleeding (Neonate)
Again start with an Apt test, if its positive its maternal swallow blood, if its negative do a PT,
PTT. If the coagulation profile is abnormal give Vit K for hematologic disorder of newborn. If
it's normal do a rectal exam. A fissure could be the cause, if negative then consider either
malrotation or Necrotizing enterocolitis.
B.1 Necrotizing Enterocolitis (NEC)
Although the exact pathogenesis of NEC is not known, the most widely held theory is that of
perinatal stress leading to selective circulatory ischemia. The stress includes prematurity,
sepsis, hypoxia, hypothermia, and jaundice. These babies frequently have umbilical artery,
vein catheters, have received exchange transfusions or early feeds with hyperosmolar
formulas. The intestinal mucosal cells are highly sensitive to ischemia and mucosal damage
leads to bacterial invasion of the intestinal wall. Gas-forming organisms produce pneumatosis
intestinalis (air in the bowel wall readily seen on abdominal films). Full-thickness necrosis
leads to perforation, free air and abscess formation. These usually premature infants develop
increased gastric residuals, abdominal distension, bloody stools, acidosis and dropping
platelet count. The abdominal wall becomes reddened and edematous. There may be
persistent masses and signs of peritonitis. Perforation leads to further hypoxia, acidosis and
temperature instability. The acid-base status is monitored for worsening acidosis and hypoxia.
Serial platelet counts are obtained and, with increasing sepsis, the platelet count drops
<50,000, indicating intravascular coagulation and decreased bone marrow production. The
white blood cell count may be high, low or normal and is not generally of help. Serial
abdominal films are obtained to look for evidence of free abdominal air, a worsening picture of
pneumatosis intestinalis, or free portal air. Therapy consist initially of stopping feeds,
instituting nasogastric suctioning and beginning broad-spectrum antibiotics (ampicillin and
gentamycin). Persistent or worsening clinical condition and sepsis or free air on abdominal
films require urgent surgical intervention. Attempts to preserve as much viable bowel as
possible are mandatory to prevent resultant short gut syndrome.
Complicated NEC is the most common neonatal surgical emergency of modern times, has
diverse etiologies, significant mortality and affects mostly premature babies. The use of
primary peritoneal drain (PPD) in the management of NEC dates from 1977. The technique is
used in the very low birth weight premature infant (<1500 gm) with pneumoperitoneum,
metabolic and hemodynamic instability. Consist of a right lower quadrant incision and
placement of a drainage (penrose or catheter) under local anesthesia with subsequent

irrigation performed bedside at the NICU. Initially used as a temporizing measure before
formal laparotomy, some patient went to improvement without the need for further surgery
(almost one-third). They either had an immature (fetal type) healing process or a focal
perforation (not associated to NEC?) which healed spontaneously. Those babies not improved
by PPD either die (20%), go on to laparotomy and half die (20%) or develop complications
(24%). Some suggestion made are: PPD should be an adjunct to preop stabilization, before
placing drain be sure pt has NEC by X-rays, persistent metabolic acidosis means uncontrolled
peritoneal sepsis, do not place drain in pts with inflammatory mass or rapid development of
intraperitoneal fluid, the longer the drainage the higher the need for laparotomy.
C. Upper GI Bleeding (Older Children)
In the initial evaluation a history should be obtained for bleeding disorders, skin lesions, and
aspirin or steroid ingestion. The physical exam for presence of enlarged liver, spleen, masses,
ascites, or evidence of trauma or portal hypertension. Labs such as bleeding studies and
endoscopy, contrast studies if bleeding stops. Common causes of Upper GI bleeding are:
1. Esophagus
(a) Varices- usually presents as severe upper gastrointestinal bleeding in a 2-3 year old who
has previously been healthy except for problems in the neonatal period. This is a result of
extrahepatic portal obstruction (portal vein thrombosis most commonly), with resulting
varices. The bleeding may occur after a period of upper respiratory symptoms and coughing.
Management is initially conservative with sedation and bedrest; surgery ir rarely needed.
(b) Esophagitis- this is a result of persistent gastroesophageal reflux leading to inflammation
and generally slow, chronic loss of blood from the weeping mucosa. Treatment consist of
antacids, frequent small feeds, occasionally medications and if not rapidly improved, then
surgical intervention with a fundoplication of the stomach.
(c) Mallory-Weiss- this is a tear of the distal esophagus and/or gastroesophageal junction
secondary to severe regurgitation. This was thought to be uncommon in children because it
was not looked for by endoscopy. It probably occurs more often than previously thought.
Treatment initially is conservative and, if persistent, oversewing of the tear through an incision
in the stomach will be successful.
(d) Duplication cysts- Rare cause, they are seen on the mesenteric side of the intestine
anywhere from the esophagus to the anus. They bleed when there is ectopic gastric mucosa
present. Total excision is curative.
2. Stomach
(a) Gastric Erosions- managed medically in most cases.
(b) Ulcer- treated medically unless there is persistent hemorraghe, obstruction or perforation.
(c) Hematoma- usually secondary to trauma or bleeding disorders.
3. Duodenum
(a) Duodenitis- associated to acid peptic disease.
(b) Hematobilia- secondary to blunt or penetrating abdominal injury. Occasionally requires
surgical intervention with local repair or ligation of hepatic vessels.
D. Older GI Bleeding (Older Child)
D.1 Anal Fissure
Anal fissure is the most common cause of rectal bleeding in the first two years of life.
Outstretching of
the anal mucocutaneous junction caused by passage of large hard stools during defecation
produces a
superficial tear of the mucosa in the posterior midline. Pain with the next bowel movement
leads to
constipation, hardened stools that continue to produce cyclic problems. Large fissures with
surrounding bruising should warn against child abuse. Crohn's disease and leukemic
infiltration are
other conditions to rule-out. The diagnosis is made after inspection of the anal canal. Chronic
fissures
are associated with hypertrophy of the anal papilla or a distal skin tag. Management is directed
toward
the associated constipation with stool softeners and anal dilatations, warm perineal baths to
relax the
internal muscle spasm, and topical analgesics for pain control. If medical therapy fails

excision of the
fissure with lateral sphincterotomy is performed.
D.2 Meckel's Diverticulum
Meckel's diverticulum (MD), the pathologic structure resulting from persistence of the
embryonic
vitelline duct (yolk stalk), is the most prevalent congenital anomaly of the GI tract. MD can be
the cause
of: gastrointestinal bleeding (most common complication), obstruction, inflammation and
umbilical
discharge in children and 50% occur within the first two years of life. Diagnosis depends on
clinical
presentation. Rectal bleeding from MD is painless, minimal, recurrent, and can be identified
using
99mTc- pertechnetate scan; contrasts studies are unreliable. Persistent bleeding requires
arteriography
or laparotomy if the scan is negative. Obstruction secondary to intussusception, herniation or
volvulus
presents with findings of fulminant, acute small bowel obstruction, is diagnosed by clinical
findings and
contrast enema studies. The MD is seldom diagnosed preop. Diverticulitis or perforation is
clinically
indistinguishable from appendicitis. Mucosal polyps or fecal umbilical discharge can be
caused by MD.
Overall, complications of Meckel's are managed by simple diverticulectomy or resection with
anastomosis. Laparoscopy can confirm the diagnosis and allow resection of symptomatic
cases.
Removal of asymptomatic Meckel's identified incidentally should be considered if upon
palpation there
is questionable heterotopic (gastric or pancreatic) mucosa (thick and firm consistency)
present.
D.3 Juvenile Polyps
Childhood polyps are usually juvenile (80%). Histology features a cluster of mucoid lobes
surrounded
by flattened mucussecreting glandular cells (mucous retention polyp), no malignant potential.
Commonly seen in children age 310 with a peak at age 56. As a rule only one polyp is present,
but
occasionally there are two or three almost always confined to the rectal area (within the reach
of the
finger). Most common complaint is bright painless rectal bleeding. Occasionally the polyp may
prolapse
through the rectum. Diagnosis is by barium enema, rectal exam, or endoscopy. Removal by
endoscopy
is the treatment of choice. Rarely colotomy and excision are required.

VI. PANCREATIC AND BILIARY DISORDERS

A. Pancreatitis
Uncommon disorder in childhood. Trauma (compressed injury against spinal
column) and biliary tract disorders (choledochal cyst, cholelithiasis) are most
common cause of pancreatitis. The most common congenital ductal anomaly
leading to pancreatitis is pancreas divisum. Most common complaint is
mid-epigastric abdominal trauma associated with nausea and vomiting.
Diagnosis is confirmed with elevated levels of amylase and lipase. Ultrasound
is useful to determine degree of edema and presence of pseudocyst formation.

Treatment consists of: NPO, NG decompression, decrease acid stimulation

(H-2 blockers), aprotinin, glucagon, and anticholinergics. Pain is relieved with
meperidine. When pancreatic serum enzymes level return to near normal level
patient is started in low-fat diet. Antibiotic prophylaxis use is controversial.
Surgery is indicated for: abscess formation and pseudocyst. Pseudocysts are
the result of major ductal disruptions or minor lacerations. Observation allows
spontaneous resolution in 40-60% of cases. Percutaneous aspiration and
catheter drainage is another alternative in management. Follow-up studies
permit determine if cavity is decreasing in size. This can be done outpatient
teaching parents to irrigate the catheter at home to assure patency.
Persistency beyond 6 months may need resectional therapy. Additional option
is internal drainage (cyst-gastrostomy, cyst-jejunostomy). Abscess should be
drained and debride. Pancreatic pseudocyst formation is an uncommon
complication of pancreatic inflammatory disease (pancreatitis) or trauma in
children. More than half cases are caused by blunt abdominal trauma.
Ultrasound is the most effective and non-invasive way of diagnosing
pancreatic pseudocysts. Acute pseudocysts are managed expectantly for 4-6
wk. until spontaneous resolution occurs. 25-50% will undergo spontaneous
resolution. Medical therapy consists of decreasing pancreatic stimulation and
giving nutritional support. Rupture is the major complication of conservative
management. Chronic pseudocysts (> three mo.) will benefit from prompt
operation and internal drainage since resolution is rare. Percutaneous catheter
drainage under local anesthesia using Ultrasound or CT guided technique is
an appropriate method of first-line therapy for non-resolving (chronic) or
enlarging pancreatic pseudocysts. The approach is transgastric or
transcutaneous. Those cysts that fail to resolve with percutaneous drainage
should go investigation of ductal anatomy to rule out disruption of the main
pancreatic duct. The need for further surgery (drainage or resectional) will
depend on the status of the duct of Wirsung.
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B. Biliary Atresia
Persistent conjugated hyperbilirubinemia (greater than 20% of total or 1.5
mg%) should be urgently evaluated. Initial evaluation should include a
well-taken history and physical exam, partial and total bilirubin determination,
type and blood group, Coomb's test, reticulocyte cell count and a peripheral
smear. Cholestasis means a reduction in bile flow in the liver, which depends
on the biliary excretion of the conjugated portion. Reduce flow causes
retention of biliary lipoproteins that stimulates hypercholesterolemia causing
progressive damage to the hepatic cell, fibrosis, cirrhosis and altered liver
function tests. Biliary Atresia (BA) is the most common cause of persistently
direct (conjugated) hyperbilirubinemia in the first three months of life. It is
characterized by progressive inflammatory obliteration of the extrahepatic bile
ducts, an estimated incidence of one in 15,000 live births, and predominance
of female patients. The disease is the result of an acquired inflammatory
process with gradual degeneration of the epithelium of the extrahepatic biliary
ducts causing luminal obliteration, cholestasis, and biliary cirrhosis. The
timing of the insult after birth suggests a viral etiology obtained

transplacentally. Almost 20% of patients have associated anomalies such as:

polysplenia, malrotation, situs inversus, preduodenal portal vein and absent
inferior vena cava. Histopathology is distinguished by an inflammatory
process in several dynamic stages with progressive destruction, scar
formation, and chronic granulation tissue of bile ducts. Physiologic jaundice
of the newborn is a common, benign, and self-limiting condition. In BA the
patient develops insidious jaundice by the second week of life. The baby looks
active, not acutely ill and progressively develops acholic stools, choluria and
hepatomegaly. Non-surgical source of cholestasis shows a sick, low weight
infant who is jaundiced since birth. The diagnostic evaluation of the
cholestatic infant should include a series of lab tests that can exclude
perinatal infectious (TORCH titers, hepatitis profile), metabolic
(alpha-1-antitrypsin levels), systemic and hereditary causes. Total bilirubin in
BA babies is around 6-10 mg%, with 50-80% conjugated. Liver function tests
are nonspecific. Lipoprotein-X levels greater than 300 mg% and Gamma
Glutamyl Transpeptidase (GGT) above 200 units% suggest the diagnosis. The
presence of the yellow bilirubin pigment in the aspirate of duodenal content
excludes the diagnosis of BA. Ultrasound study of the abdomen should be the
first diagnostic imaging study done to cholestatic infants to evaluate the
presence of a gallbladder, identify intra or extrahepatic bile ducts dilatation,
and liver parenchyma echogenicity. The postprandial contraction of the
gallbladder eliminates the possibility of BA even when nuclear studies are
positive. Nuclear studies of bilio-enteric excretion (DISIDA) after
pre-stimulation of the microsomal hepatic system with phenobarbital for 3-5
days is the diagnostic imaging test of choice. The presence of the
radio-isotope in the GI tract excludes the diagnosis of BA. Percutaneous liver
biopsy should be the next diagnostic step. The mini-laparotomy is the final
diagnostic alternative. Those infant with radiographic evidence of patent
extrahepatic biliary tract has no BA. Medical management of BA is uniformly
fatal. Kasai portoenterostomy has decreased the mortality of BA during the
last 30 years. Kasai procedure consists of removing the obliterated
extrahepatic biliary system, and anastomosing the most proximal part to a
bowel segment. Almost three-fourth of patients will develop portal
hypertension in spite of adequate postoperative bile flow. They will manifest
esophageal varices, hypersplenism, and ascites. Hepatic transplantation is
reserved for those patients with failed portoenterostomy, progressive liver
failure or late-referral to surgery.
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C. Choledochal Cyst
Choledochal cyst is a rare dilatation of the common bile duct, prevalent in
oriental patients (Japan), where >60% of patients are less than 10 years old.
The etiology is related to an abnormal pancreatic-biliary junction (common
channel theory) causing reflux of pancreatic enzymes into the common bile
duct (trypsin and amylase). Symptoms are: abdominal pain, obstructive
jaundice, a palpable abdominal mass, cholangitis, and pancreatitis. Infants
develop jaundice more frequently, causing diagnostic problems with Biliary
Atresia. Older children may show abdominal pain and mass. Jaundice is less

severe and intermittent. Diagnosis is confirmed with Ultrasound and

corroborated with a HIDA (or DISIDA) Scan. Choledochal cysts are classified
depending on morphology and localization. Management is surgical and
consist of cyst excision and roux-en-Y hepatico-jejunostomy reconstruction.
Cyst retention penalties paid are: stricture, cholangitis, stone formation,
pancreatitis, biliary cirrhosis, and malignancy. Long-term follow-up after
surgery is advised.
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D. Cholelithiasis
With the increase use of sonography in the work-up of abdominal pain,
cholelithiasis is diagnosed more frequent in children. Gallstones occur as
consequence of loss of solubility of bile constituents. Two types are
recognize: cholesterol and bilirubin. Those of cholesterol are caused by
supersaturation of bile (lithogenic) by cholesterol overproduction or bile salt
deficiency. Bilirubin stones occur due to hemolysis (Sickle Cell, thalassemia)
or bacterial infection of bile. Other etiologies include: Ascaris Lumbricoides
infestation, drug-induced (ceftriaxone), ileal resection, TPN. etc. Gallbladder
sludge is a clinical entity that when it persists can be a predisposing factor for
cholelithiasis and cholecystitis. Laparoscopic Cholecystectomy (LC) has
become the procedure of choice for the removal of the disease gallbladder of
children. The benefit of this procedure in children is obvious: is safe, effective,
well tolerated, it produces a short hospital stay, early return to activity and
reduced hospital bill. Several technical differences between the pediatric and
adult patient are: lower intrabdominal insufflation pressure, smaller trocar size
and more lateral position of placement. Complications are related to the initial
trocar entrance as vascular and bowel injury, and those related to the
procedure itself; bile duct injury or leak. Three 5 mm ports and one 10 mm
umbilical port is used. Pneumoperitoneum is obtained with Veress needle
insufflation or using direct insertion of blunt trocar and cannula.
Cholangiography before any dissection of the triangle of Calot is advised by
some workers to avoid iatrogenic common bile duct injuries during dissection
due to anomalous anatomy, it also remains the best method to detect common
bile ducts stones. Treatment may consist of: (1) endoscopic sphincterotomy,
(2) opened or laparoscopic choledochotomy, or (3) transcystic
choledochoscopy and stone extraction. Children with hemolytic disorders, i.e.
Sickle cell disease, have a high incidence of cholelithiasis and benefit from LC
with a shorter length of postop stay and reduced morbidity. Acalculous
cholecystitis (AC) is more commonly found in children than adults. Two-third
of cases appear as a complication of other illness: trauma, shock, burns,
sepsis, and operative procedures. Contributing causes mentioned are:
obstruction, congenital tortuosity or narrowing of the cystic duct, decreased
blood flow to the gallbladder, and long-term parenteral nutrition. Males are
more commonly affected than females. Fever, nausea, vomiting, diarrhea,
dehydration and marked subhepatic tenderness are the most common
symptoms. Other less common sx are jaundice, and abdominal mass. Labs
show leucocytosis and abnormal liver function tests. Recently (APSA 95), two
distinct forms of this disease have been recognized: acute, with symptom

duration less than one month and chronic, with sx greater than three months.
US is diagnostic by demonstrating hydrops of gallbladder, increase wall
thickness and sludge. HIDA scan with CCK stimulation may help diagnose
chronic cases. In both situations management consist of early
cholecystectomy which can be executed using laparoscopic techniques.
Back to Index

E. Idiopathic Perforation of Bile Ducts

Spontaneous perforation of the common bile duct is the second cause of
surgical jaundice in infants. The perforation is generally identified at the
junction of the cystic and common bile ducts. Most infants develop slowly
progressive bilious ascites, jaundice, and clay-colored stools. Other patients
develop an acute bile peritonitis. Diagnosis is by ultrasound or HIDA scan
showing extravasation. Paracentesis confirms the nature of the ascitic fluid.
Management consist of intraoperative cholangiogram (to demonstrate area of
leak), and adequate simple drainage of area. Periportal inflammation precludes
vigorous surgical efforts that could be disastrous. Tube cholecystostomy
placement help for post-op follow-up studies. The leak generally seals
spontaneously during the ensuing 2-3 weeks. Prognosis is good with no long
term biliary sequelae.
Back to Index

VII. TUMORS
A. Wilms Tumor & Genetics
Wilms tumor (WT) is the most common intra-abdominal malignant tumor in children
affecting more than 400 children annually in the USA. It has a peak incidence at 3.5 years of
age. WT present as a large abdominal or flank mass with abdominal pain, asymptomatic
hematuria, and occasionally fever. Other presentations include malaise, weight loss, anemia,
left varicocele (obstructed left renal vein), and hypertension. Initial evaluation consists of:
Abdominal films, Ultrasound, IVP, urinalysis, Chest-X-rays and Computed Tomography. The
presence of a solid, intrarenal mass causing intrinsic distortion of the calyceal collecting
system is virtually diagnostic of Wilms tumor. Doppler sonography of the renal vein and
inferior vena cava can exclude venous tumor involvement. Metastasis occurs most commonly
to lungs and occasionally to liver. Operation is both for treatment and staging to determine
further therapy.
Following NWTSG recommendation's primary nephrectomy is done for all but the largest
unilateral tumors and further adjuvant therapy is based on the surgical and pathological
findings. Important surgical caveats consist of using a generous transverse incision,
performing a radical nephrectomy, exploring the contralateral kidney, avoiding tumor spillage,
and sampling suspicious lymph nodes. Nodes are biopsied to determine extent of disease. WT
staging by NWTSG consists of:
Stage I- tumor limited to kidney and completely resected.
Stage II- tumor extends beyond the kidney but is completely excised.
Stage III- residual non-hematogenous tumor confined to the abdomen.
Stage IV- hematogenous metastasis.
Stage V- bilateral tumors.
Further treatment with chemotherapy or radiotherapy depends on staging and histology
(favorable vs. non-favorable) of the tumor. Non-favorable histologic characteristics are:
anaplasia (three times enlarged nucleus, hyperchromatism, mitosis), sarcomatous or rhabdoid
degeneration. The success in managing WT has been remarkable the result of stratification,

registry and study from the NWTSG. Disease-free survival is 95% for Stage I and
approximately 80% for all patients. Prognosis is poor for those children with lymph nodes,
lung and liver metastasis.
WT occurs either sporadic (95%), familial (1-2%) or associated with a syndrome (2%). Such
syndromes predisposing to WT are WAGR (Wilms, aniridia, genitourinary malformation and
mental retardation), Beckwith-Wiedemann Syndrome (gigantism, macroglossia, pancreas cell
hyperplasia, BWS), and Denys-Drash Syndrome (male pseudohermaphrodite, nephropathy
and Wilms tumor, DDS). They tend to occur in younger patients. Sporadic WT is associated in
10% of cases with isolated hemihypertrophy or genitourinary malformations such as
hypospadia, cryptorchidism and renal fusion. Bilateral synchronous kidney tumors are seen in
5-10% of cases. Routine abdominal ultrasound screening every six months up to the age of
eight years is recommended for children at high risk for developing WT such as the
above-mentioned syndromes.
It was originally thought that WT developed after the two-hit mutational model developed
for retinoblastoma: When the first mutation occurs before the union the sperm and egg
(constitutional or germline mutation) the tumor is heritable and individuals are at risk for
multiple tumors. Nonhereditary WT develops as the result of two-postzygotic mutations
(somatic) in a single cell. The two-event hypothesis predicts that susceptible individuals such
as familial cases, those with multifocal disease and those with a congenital anomaly have a
lower median age at diagnosis than sporadic cases. It is now believed that several genes'
mutations are involved in the overall WT pathogenesis.
Loss of whole portions of a chromosome is called loss of heterozygosity (LOH), a
mechanism believed to inactivate a tumor-suppressor gene. WT has been found 50% of the
time to contain LOH at two genetic loci: 11p13 and 11p15. WT will develop in 30% of WAGR
children. Children with the WAGR association shows a deletion in the short arm of
chromosome 11 band 13 (11p13) but a normal 11p15 region. Up to a third of sporadic WT have
changes in the distal part of chromosome 11, a region that includes band p13. The region of
the deletion has been named the WT1 gene, a tumor suppressor gene that also forms a
complex with another known tumor-suppressor, p53. WT1 gene express a regulated
transcription factor of the zinc-finger family proteins restricted to the genitourinary system,
spleen, dorsal mesentery of the intestines, muscles, central nervous system (CNS) and
mesothelium. WT1 is deleted in all WAGR syndromes cases. The important association of WT1
mutation and WAGR syndrome with intralobar nephrogenic rests immediately suggest that
WT1 expression be necessary for the normal differentiation of nephroblasts. Only five to 10%
of sporadic WT have thus far been shown to harbor WT1 mutations. Inactivation of WT1 only
affects organs that express this gene such as the kidney and specific cells of the gonads
(Sertoli cells of the testis and granulosa cells of the ovary). WT1 has been shown to cause the
Denys-Drash syndrome. Most of the mutations described in DDS patients are dominant
missense mutations.
A small subset of BWS has a 11p15 duplication or deletion. The region 11p15 has been
designated WT2 gene and is telomeric of WT1. Beckwith- Wiedemann form of WT is also
associated with IGF2, an embryonal growth-inducing gene. This might prove that two
independent loci may be involved in tumor formation. Candidates genes include insulin-like
growth factor II gene (IGFII) and the tumor suppressor gene H19. A substantial fraction of WT
(without LOH at the DNA level) has been found to have altered imprints with resultants over
expression of IGFII and loss of expression of the tumor suppressor H19. IGFII may be
operating like an oncogene by perpetuating nephroblast and may account for the perilobar
rests observed in BWS patients.
A gene for a familial form (FWT1) of the tumor has also been identified in chromosome 17q.
There also might be a gene predisposing to Wilms tumor at chromosome 7p, where
constitutional translocations have been described. Mutation in p53 is associated with tumor
progression, anaplasia and poor prognosis. Most WT are probably caused by somatic
mutations in one or more of the increasing number of WT genes identified.
A few chromosomal regions have seen identified for its role in tumor progression. LOH at
chromosome 16q and chromosome 1p has been implicated in progression to a more
malignant or aggressive type Wilms' tumor with adverse outcome. This occurs in
approximately 20% of patients with WT. These children have a relapse rate three times higher
and a mortality rate twelve times higher than WT without LOH at chromosome 1p. p53 is also
associated with the so called anaplastic unfavorable histology.
Patients with WT and a diploid DNA content (indicating low proliferation) have been found
to have an excellent prognosis. Hyperdiploidy (high mitotic activity) is a poor prognostic
feature of Wilms tumor, rhabdomyosarcoma and Osteosarcoma.
Nephrogenic rests are precursor lesions of WT. Two types are recognized: perilobar

nephrogenic rests (PLNR) limited to the lobar periphery, and intralobar nephrogenic rests
(ILNR) within the lobe, renal sinus or wall of the pelvocaliceal system. The strong association
between ILNR, aniridia and Denys-Drash syndrome where the WT1 zinc finger gene has been
implicated suggests that this locus might be linked to the pathogenesis of ILNR. Also the
association between BWS and some cases of hemihypertrophy with abnormalities of more
distant loci on chromosome 11p raises the possibility that the putative WT2 gene might be
more closely linked to PLNR.
An advantage of genetic testing is that children with sporadic aniridia, hemihypertrophy or
the above discussed syndromes known to be at high risk for developing WT can undergo
screening of the germline DNA. This might identify if they harbor the mutation and need closer
surveillance for tumor development.
B. Neuroblastoma & Genetics
Neuroblastoma (NB) is the most common extracranial solid tumor in infants. More than 500
new cases are diagnosed annually in the United States. Most neuroblastomas (75%) arise in
the retroperitoneum (adrenal gland and paraspinal ganglia), 20% in the posterior mediastinum,
and 5% in the neck or pelvis. NB is a solid, highly vascular tumor with a friable pseudocapsule.
Most children present with an abdominal mass, and one-fourth have hypertension. Other have:
Horner's syndrome, Panda's eyes, anemia, dancing eyes or vaso-intestinal syndrome.
Diagnosis is confirmed with the use of simple X-rays (stipple calcifications), Ultrasound, and
CT-Scan. Work-up should include: bone marrow, bone scan, myelogram (if there is evidence
of intraspinal extension), and plasma/urine tumor markers level: vanillylmandelic acid (VMA),
homovanillic acid ( HVA) and dopamine (DOPA).
Management of NB depends on the stage of disease at diagnosis. Localized tumors are
best managed with surgical therapy. Partially resected or unresectable cases need
chemotherapy a/o radiotherapy after establishing a histologic diagnosis. Independent
variables determining prognosis are age at diagnosis and stage of disease. The Evans
classification for NB staging comprised:
Stage I - tumor confined to an organ of origin.
Stage II - tumor extending beyond an organ of origin, but not crossing the midline. Ipsilateral
lymph nodes may be involved.
Stage III - tumor extending beyond midline. Bilateral lymph nodes may be involved.
Stage IV - remote disease involving skeleton, bone marrow, soft tissue or distant lymph
nodes.
Stage IVS - same as stage I or II with presence of disease in liver, skin or bone marrow.
Young children with stage I/II have a better outcome. A poor outcome is characteristic of
higher stages, older patients and those with bone cortex metastasis. Other prognostic
variables are: the site of primary tumor development, maturity of tumor, presence of positive
lymph nodes, high levels of ferritin, neuron-specific enolase, and diploid DNA.
Neuroblastoma is a malignant tumor of the postganglionic sympathetic system that
develops from the neural crest: sympathetic ganglion cells and adrenal gland. In vitro three
cell types have been identified:
1- neuroblastic (N-type) cells that are tumorigenic. These cells are responsible for producing
cathecolamines and vasoactive substances which help in diagnosis and follow-up therapy.
2- the Schwannian or substrate-adherent (S-type) cells that are non-tumorigenic, and the
3- intermediate I-cells.
NB can behave seemingly benignly and undergo spontaneous regression, mature into a
benign ganglioneuroma or most commonly progress to kill its host. This disparate behavior is
a manifestation that we are dealing with related tumors with differently genetic and biological
features associated with a spectrum of clinical behaviors.
Conclusive associations with environmental factors have not been proved in NB. Hereditary
factors are important in NB since a few cases exhibit predisposition following a dominant
pattern of inheritance. The most characteristic cytogenetic abnormality of neuroblastoma is
deletion of the short arm of chromosome 1 in locus 36 (1p36) occurring in 50 to 70% of primary
diploid tumors. LOH of the short arm of chromosome 1 is also associated with an unfavorable
outcome, suggesting that a tumor suppression gene may be found in this region. The common
region of deletion or LOH resides at the distal end of the short arm of chromosome 1 from
1p36.2 to 1p36.3. Loss or inactivation of a gene at this site is critical for progression of
neuroblastoma. A few candidate genes from this site have been mapped. LOH in chromosome
14 long arm (14q) has also been identified in 25-50% or neuroblastoma cells studied but no
clinical behavior has been identified with this finding. Gain of chromosome 17 is associated
with more aggressive tumors.

Another consistent chromosomal aberration identified in 25 to 30% of NB cells is the

presence of double-minute chromosomes, small fragments of chromatin containing multiple
copies of the oncogene N-myc produced by amplification. N-myc protooncogene is found on
chromosome 2p and its activation results in tumor formation. The amplified N-myc sequence
is found on extrachromosomal double minutes (DM) or on homogeneous staining regions
(HSR) involving different chromosomes in neuroblastoma (N-type) cell lines. N-myc
amplification is strongly associated with advance stages of disease, rapid tumor progression
and poor outcome independent of the stage of the tumor or the age of the patient. NB tumors
associated with N-myc amplification needs aggressive therapy. N-myc amplification
associated with deletion of 1p is correlated with a poor outcome. Deletion of the long arm of
chromosome 1 (1q-) is also a poor prognostic sign.
Though most NB cells are diploid, a good number of them are hyperdiploid or triploid.
Hyperdiploidy is a good prognostic feature of NB and embryonal rhabdomyosarcoma in
infants, while diploid tumors at any age and hyperdiploid in older patients carry a worse
prognosis requiring more intensive treatment.
Neuroblast cells needs nerve growth factor (NGF) for proper differentiation. NB tumor cells
do not respond to NGF or do not express the receptor. This receptor consists of three
transmembrane tyrosine kinase receptors (TRK-A, TRK-B, and TRK-C), known together as the
TRK receptor. TRK-A is detected in 90% of NB cells and correlates inversely with N-myc
expression. High TRK-A levels correlate strongly with improved survival and plays a role in the
propensity for tumors to regress or differentiate into a more benign nature. TRK-B is
associated with more matured tumors and TRK-C with lower stage tumors. Alteration in the
NGF receptor function or expression promotes tumorigenesis. In conclusion, high levels of
TRK expression are associated with better prognosis, earlier stage, lower patient age and lack
of N-myc expression.
Neuroblastomas in newborns, cystic tumors, bilateral tumors in infants, and infants less
than one year of age with neuroblastoma stage IV-S can undergo neuronal cell differentiation
with spontaneous regression. It is thought that high level of TRK-A found in this cases might
explain differentiation and regression as high level of this glycoprotein is associated with a
favorable prognosis. Regression might be associated with non-affected tumor cell apoptosis.
Other biological markers associated with NB are the multidrug resistance-related protein
(MRP) gene, telomerase activity and bcl-2 gene activity. MRP shows a strong correlation with
an advanced clinical stages and poor prognosis. High telomerase activity is associated with
poor prognosis and high N-myc amplification. The bcl-2 gene produces a protein that prevents
neuronal cell death (apoptosis) and promotes tumor progression. Bcl-2 expression is
associated with a poor outcome. Apoptosis in NB may result in tumor progression.
The RET proto-oncogene is a protein tyrosine kinase gene (Ret protein) expressed in the
cells derived from the neural crest. The activation of RET involves a chromosomal inversion of
the long arm of chromosome 10 that juxtaposes the tyrosine kinase encoding domain of RET
to the amino terminal sequences of at least three unrelated genes. Germline mutations in the
RET gene have been associated with neuroblastoma, pheochromocytoma, multiple endocrine
neoplasia (MEN) 2, familial medullary thyroid carcinoma (MTC), radiation-induced thyroid
papillary carcinoma, and recently Hirschsprung's disease. RET analysis is a suitable method
to detect asymptomatic children with MEN at risk to develop MTC allowing us to consider
thyroidectomy at a very early stage of neoplasm development (C-cell hyperplasia) or
prophylactically.
High levels of neuron specific enolase and serum ferritin levels are associated with a poor
prognosis in NB. Nm-23 and ganglioside GD2 are still other tumor markers associated with
poor outcome, active disease and tumor progression.
C. Rhabdomyosarcoma & Genetics
Rhabdomyosarcoma (RMS), the most common soft tissue sarcoma in infants and children,
represents about 5-15% of all malignant solid lesions. It has a peak incidence before the age of
five years, and a second surge during early adolescence. Head, neck and pelvic malignancies
are more prevalent in infancy and early childhood, while trunk, extremity and paratesticular
sites are largely a disease of adolescents. RMS arises from a primitive cell type and occurs in
mesenchymal tissue at almost any body site excluding brain and bone. The predominant
histologic type in infants and small children is embryonal rhabdomyosarcoma, occurring in
the head and neck, genitourinary tract and retroperitoneum. Embryonal RMS is associated
with a favorable prognosis. Botryoid RMS is a subtype of the embryonal variety, which
ordinarily extends into body cavities such as bladder, nasopharynx, vagina, or bile duct. The
alveolar cell type, named for a superficial similarity to the pulmonary alveoli, is the most

common form found on the muscle masses of the trunk and extremities, and is seen more
frequently in older children and young adults. Alveolar RMS is associated with a poor
prognosis. This unfavorable prognosis is the result of early and wide dissemination, bones
marrow involvement and poor response to chemotherapy.
Clinical findings, diagnostic evaluation and therapy depend upon location of the primary
tumor and are beyond the scope of this review. Head and neck RMS are most common and
occur in the orbit, nasopharynx, paranasal sinuses, cheek, neck, middle ear, and larynx. Most
are treated by simple biopsy followed by combined therapy or preoperative chemotherapy and
radiation followed by conservative resection. Operations for extremity lesions include wide
local excision to remove as much of gross tumor as possible. The trend in management is
more chemotherapy with conservative surgical therapy. Survival has depended on primary
site, stage of disease, and treatment given.
Most RMS occurs sporadically. Approximately 5% are associated to syndromes such as
Beckwith-Wiedemann with LOH at the 11p15 locus, Li-Fraumeni, the neurofibromatosis-NF1
gene located on 17q11, Basal Cell Nevus, and the Fetal Alcohol syndrome. Other risk factors
in the development of RMS include maternal use of marijuana and cocaine, exposure to
radiation, and maternal history of stillbirth.
Alveolar and embryonal RMS are the most genetically studied sarcomas in children. The
expression of a number of human paired box-containing (PAX) genes has been correlated with
various types of RMS. In alveolar RMS novel fusion genes encoding chimeric fusion proteins
have been identified. The most consistent genetic mutation identified in more than 70% of
alveolar RMS is translocation of chromosomes 2 and 13, t(2;13)q35-37;q14). The PAX3 loci in
chromosome 2 fuses to the FKHR (fork head in rhabdomyosarcoma) domain of chromosome
13 creating a powerful chimeric PAX3-FKHR gene that encodes an 836 amino acid fusion
protein. This information is obtained using reverse transcriptase PCR assays of alveolar RMS
or by protein immunoprecipitation with PAX3 and FKHR antisera. The PAX3-FKHR protein is
an active transcription factor. The t(2;13) activates the oncogenic potential of PAX3 by
dysregulating or exaggerating its normal function in the myogenic lineage and affecting the
cellular activities of growth, differentiation and apoptosis. Another of the reported
translocation is t(1;13)(p36;q14) involving chromosome 1 and 13 in 10% of alveolar RMS. In
this variant, Chromosome 1 locus encoding PAX7 fused to FKHR in chromosome 13 resulting
in another chimeric transcript PAX7-FKHR. PAX7-FKHR tumors tend to occur in younger
patients, are more often in the extremity, are more often localized lesions and are associated
with significantly longer event-free survival. Still, a small subset of alveolar RMS does not
contain either fusion mutation. The PAX3-FKHR and the variant PAX7-FKHR fusions are
associated with distinct clinical phenotypes. Identification of fusion gene status by PCR is a
useful diagnostic tool in differentiating RMS from other round cell tumors.
Embryonal RMS contains frequent allelic loss on chromosome 11 (11p15), a genetic feature
specific for this type of tumor. Allelic loss is manifested by the absence of one of the two
signals in the tumor cells indicating a genetic event such as a chromosome loss, deletion, or
mitotic recombination that eliminates one allele and the surrounding chromosomal region. The
smallest affected region has been localized to chromosomal region 11p15.5. The presence of a
consistent region of allelic loss is often indicative of the presence of a tumor suppressor gene
inactivated in the associated malignancy. The mechanism for inactivation of tumor suppressor
genes is postulated to be a two-hit scenario in which both copies of the gene are sequentially
inactivated: a small point mutation inactivates one of the two alleles, preferably the maternal
side allele, and the allelic loss event inactivate the second allele (the paternal allele). This
leads to over expression of the insulin-like growth factor II gene known to play a role in the
development of embryonal tumors.
Other alterations associated with embryonal RMS are distinct patterns of chromosomal
gains (chromosomes 2,7,8,12,13,17,18, and 19) in contrast with alveolar RMS which shows
genomic amplification of chromosomal region 12q13-15 in 50% of cases. Notably, these
distinct changes predominantly involved chromosomes 2, 12, and 13 in both subtypes.
Additionally embryonal RMS cases shows mutations of members of the RAS gene family, a
second proto-oncogene. Both tumors share alterations in the p53 gene at the germline level
contributing to increase susceptibility to other tumors characteristics of the Li-Fraumeni
syndrome. There is also greater over expression of c-myc in alveolar RMS when compared
with embryonal RMS. All this favors a multi-step origin of RMS tumors generating phenotypic
changes of growth autonomy, abnormal differentiation and motility.
The Li-Fraumeni familial cancer syndrome is manifested by increased susceptibility of
affected relatives to develop a diverse set of malignancies during early childhood. The major
features of the syndrome include breast cancer, osteosarcoma, rhabdomyosarcomas of soft
tissue, glioblastoma, leukemia and adrenal cortical carcinoma. More than one-half of the

cancers overall and nearly one-third of the breast cancers were diagnosed before 30 years of
age. Among females, breast cancer is the most common. Germline mutations within a defined
region of the p53 gene have been found in families with the Li-Fraumeni syndrome.
Persistence of the mutation in the germline suggests a defect in DNA repair in the family
member first affected. Asymptomatic carriers of p53 germline mutation needs closed
evaluation and follow-up for early detection and treatment in case neoplasia develops.
D. Hepatic Tumors: Hepatoblastoma
Hepatoblastoma (HB) is the most common primary malignant neoplasm of the liver in children
mostly
seen in males less than four year of age. Diagnostic work-up (US, Scintigraphy, CT-Scan)
objective is
predicting resectability and tumor extension. Diagnostic laparotomy will decide resectability.
Markers
associated to this tumor are: alpha-fetoprotein and gamma-glutamyltransferase II. Only
reliable chances
of cure is surgical excision although half are unresectable at dx. Unresectable tumors can be
managed
with preop chemotx. Disadvantages of preop chemotx are: progressive disease, increase
morbidity,
post-op complications, and toxicity. Advantages are: decrease in tumor size, covert
three-fourth cases
into resectable, although extent of surgery is not decreased. Tumor necrosis is more
extensive in pt.
receiving preop chemotx. Osteoid present in tumors after chemotx may represent an inherent
ability of
the tumor to maturate and differentiate. Diploid tumors on DNA flow cytometry show a better
prognosis.
E. Sacrococcygeal Teratoma
Sacrococcygeal teratoma (SCT) is the most common extragonadal germ cell tumor in
neonates with an
incidence of one in 30-40,000 live births. Three-fourth are females. SCT present as a large, firm
or more
commonly cystic masses that arise from the anterior surface of the sacrum or coccyx,
protruding and
forming a large external mass. Histology consist of tissue from the three germ cell layers. SCT
is
classified as: mature, immature, or malignant (endodermal sinus) and produces alpha-feto
protein (AFP).
Prenatal sonographic diagnostic severity criteria are: tumor size greater than the biparietal
diameter of
the fetus, rapid tumor growth, development of placentomegaly, polyhydramnios and hydrops.
Large
tumors should benefit from cesarean section to avoid dystocia or tumor rupture. Management
consist of
total tumor resection with coccyx (recurrence is associated with leaving coccyx in place).
Every
recurrence of SCT should be regarded as potentially malignant. Malignant or immature SCT
with
elevated AFP after surgical resection will benefit from adjuvant chemotherapy. Survival is 95%
for
mature/immature tumors, but less than 80% for malignant cases. Follow-up should consist of
(1)
meticulous physical exam every 3-6 months for first three years, (2) serial AFP determination,
(3)
fecal/urodynamic functional studies. Long term F/U has found a 40% incidence of fecal and
urinary
impairment associated to either tumor compression of pelvic structures or surgical trauma.
F. Ovarian Tumors

Ovarian tumors are uncommon childhood malignancies (1%) characterized by recurrence and
resistance to therapy. Aggressive surgery is limited to avoid compromising reproductive
capacity and
endocrine function. Low incidence and need of mulitinodal therapy encourages referral to
centers
dealing with effective cancer therapy.The most common histology is germ cell:
dysgerminoma,
teratoma, and endodermal sinus tumor. This is followed by the sex-cord stroma tumors with a
low
incidence of malignancy. They can cause feminization (granulosa-theca cell) and
masculinization
(androblastoma). Other types are: epithelial (older adolescent), lipid-cell, and gonadoblastoma.
Ovarian
tumors present with acute abdominal symptoms (pain) from impending rupture or torsion.
They also
cause painless abdominal enlargement, or hormonal changes. Preop work-up should include:
human
chorionic gonadotropin (HCG) and alpha-fetoprotein ( AFP) levels. Imaging studies:
Ultrasound and
CT-Scan. The most important prognostic factor in malignant tumors is stage of disease at time
of
diagnosis. Objectives of surgery are: accurate staging (inspection of peritoneal surfaces and
pelvic
organs, lymph node evaluation), washing and cytology of peritoneal fluid, tumor removal, and
contralateral ovarian biopsy if needed. Chemotherapy consists of: bleomycin, cis-platinum,
and
vinblastine. Radiotherapy is generally not effective, except in dysgerminoma. Elevation of
tumor
markers (AFP or HCG) after therapy signals recurrence.
G. Thyroid Nodules
The need to differentiate malignant from benign thyroid nodules is the most challenging
predicament in management.Present diagnostic work-up consists of ultrasonography (US),
radionuclear scans (RNS) and fine-needle aspiration biopsy (FNAB). After reviewing our
ten-year experience with twenty-four pediatric thyroid nodules we found nineteen benign and
five malignant lesions. Benign nodules were soft, movable, solitary and non-tender. Malignant
nodules were found during late adolecence, characterized by localized tenderness, a
multigandular appearance and fixation to adjacent tissues. US and RNS were of limited utility
since malignancy was identified among cystic and hot nodules respectively. Suppressive
thyroid hormone therapy was useless in the few cases tried. FNAB in
eighteen cases did not limit the number of thyroid resections. It showed that the probability
that a
malignant nodule had suspicious or frankly malignant cytology was 60%. The specificity was
90%. This
is the result of a higher number of patients with follicular cell cytology in the aspirate. No
attempts
should be made to differentiate follicular adenoma from carcinoma since capsular and
vascular invasion
cannot be adequately assessed by FNAB.The physical exam findings, persistence of the
nodule,
progressive growth and cosmetic appearance were the main indications for surgery. FNAB is
a safe
procedure that plays a minor role in the decision to withhold surgery. Its greatest strength is
to
anticipate in case of malignancy that a more radical procedure is probably needed. FNAB, US
and RNS
should not replace clinical judgement or suspicion as the most important determinants in
management.
In spite of presenting with advanced, multicentric and larger tumors children have a better
survival than adults. Populations at risk: past radiation to head and neck, nuclear waste
radiation, MEN II kindred. Clinical presentation is a solitary cervical mass or metastatic lymph

node. Diagnostic work-up should include: sonogram (cystic or solid), thyroid scan (cold or
hot), Fine-needle aspiration cytology(FNA), and Chest-X-Ray (lung metastasis 20% at dx).
Pathology of tumors: papillary (majority, psammomas bodies), follicular (vascular or capsular
invasion), medullary (arise from C-cells, multicentric, locally invasive), anaplastic (rare,
invasive and metastatic). Management is surgical. Complications of surgery increase with
decreasing age of patient: temporary hypoparathyroidism, recurrent nerve injury. Prognostic
factors associated to higher mortality are: non-diploid DNA, psammomas bodies, over 2 cm
diameter nodule, and anaplastic histology. Follow-up for recurrence with serum thyroglobulin
level and radioisotope scans. Adjunctive therapy: thyroid suppression and radio-iodine for
lymph nodes and pulmonary metastasis.
H. Burkitt's Lymphoma
Burkitt's lymphoma (BL) is a highly malignant tumor first described during the late 50's in
African children (jaw), endemic in nature, and composed of undifferentiated lympho-reticular
cells with uniform appearance. The American BL variety is non-endemic, mostly attacks
children between 8-12 years of age, predominantly (>75%) with abdominal disease such as
unexplained mass, pain, or intussusception. The head and neck region follows. The tumor can
appear as a localized, diffuse (multifocal, non-resectable) or metastatic abdominal mass (bone
marrow and CNS). It's considered the fastest growing tumor in humans with a doubling time
around 12-24 hrs. Chemotherapy is the primary treatment modality due to its effectiveness in
rapidly proliferating cells. The role of surgery is to establish the diagnosis (using open
biopsy), stage the tumor, remove localized disease, relieve intestinal obstruction and provide
vascular access. Complete resection whenever possible offers the patient improved survival.
Is more readily accomplished in patients with localized bowel involvement operated on an
emergency basis due to acute abdominal symptoms. The only predictor of event free survival
is extent of abdominal disease at diagnosis. Debulking (cytoreductive) procedures increases
morbidity and delays initiation of chemotherapy worsening prognosis. Extensive tumors
should be managed with minimal procedure and immediate chemotherapy (a/o radiotherapy).
Bone marrow and CNS involvement are ominous prognostic signs.
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VIII. GYNECOLOGIC CONSIDERATIONS

A. Labial Adhesions in Infants
Minor labial adhesions is a common pediatric gynecologic problem
occasionally confused with imperforate hymen. Most cases are in children 2-6
y/o and involve labial adhesions secondary to diaper rash. The process
causing fusion is a natural one: two normally covered surfaces with squamous
epithelium in contact with each other is traumatized eventually forming a
fibrous tissue union (agglutinate) between them when healing occurs. A small
opening near the clitoris is always present through which urine escapes.This
seldom causes symptoms except recurrent UTI if it covers the urethral meatus.
Treatment consists of applying estrogenic creams (0.1%) for two weeks.
Manual separation can be painful and adhesion recurs. Unless the urethral
meatus is covered, there is no reason to be further aggressive in management.
Prolonged use of estrogenic cream can cause precocious isosexual
development.
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B. Ovarian Cysts
Ovarian cysts in fetus and infants are usually follicular in nature and less than

2 cm in size. They are commonly diagnosed between the 28th and 39th wk. of
gestation by sonography. Hypotheses on etiology are: (1) Excessive fetal
gonadotropic activity, (2) enzymatic abnormalities of the theca interna, and (3)
abnormal stimulation by the mother HCG. Obstetric management consists on
observation and vaginal delivery. After birth, diagnostic assessment and
management will depend on the size and sonographic characteristics of the
cyst. Simple anechoic cysts, and those less than 5 cm in size can be observed
for spontaneous resolution. Cyst with fluid debris, clot, septated or solid
(complex nature), and larger than 5 cm should undergo surgical excision due
to the higher incidence of torsion, perforation and hemorrhage associated to
them. Percutaneous aspiration of large simple cysts with follow-up
sonography is a well-accepted therapy, preserving surgery for recurrent or
complicated cases. Surgical therapy is either cystectomy or oophorectomy
that can result in loss of normal ovarian tissue.
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C. Breast Disorders
Most breast disorders in children of either sex are benign. Congenital lesions
are: absent or multiple breast. Transplacental hormonal influence in neonates
may cause hyperplasia of breast tissue with predisposition to infection
(Mastitis neonatorum). Premature hyperplasia (thelarche) in females is the
most common breast lesion in children. It occurs before the age of eight as a
disk-shaped concentric asymptomatic subareolar mass. Remains static until
changes occur in the opposite breast 6-12 mo later. It can regress
spontaneously or stay until puberty arrives. Biopsy may mutilate future breast
development. On the contrary, discrete breast masses in males cause concern
and excision is warranted. Gynecomastia is breast enlargement cause by
hormonal imbalance, usually in obese pre-adolescent boys. If spontaneous
regression does not occur, it can be managed by simple mastectomy. Virginal
hypertrophy is rapid breast enlargement after puberty due to estrogen
sensitivity. If symptomatic, management is reduction mammoplasty.
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IX. PRENATAL CONGENITAL MALFORMATIONS

A. Fetal Surgery
Poor survival with neuroblastomas, diaphragmatic hernias and necrotizing
enterocolitis requires efforts during the next few years to reduce mortality
rates. These areas will require extensive investigation as to etiology, unique
characteristics and better management. Certain lesions such as
hydrocephalus, hydroureteronephrosis and diaphragmatic hernias may benefit
from intrauterine correction.
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B. Fetal Intestinal Obstruction

The fetal gastrointestinal tract (foregut, midgut and hindgut) undergoes ventral
folding between 24-28 days' gestation. By the 5-6th wk the stomach rotates to
the right and the duodenum occludes by cell proliferation. Recanalization of
the duodenum occurs around the 8th wk. The midgut rotation takes place
during the 6-11th wk and the final peritoneal closure by 10th wk. The fetal GI
tract begins ingestion and absorption of amniotic fluid by the 14th wk. This
fluid contributes to 17% effective nutrition; proximally obstructed gut can
cause growth retardation. Fetal intestinal obstruction is caused by: failure of
recanalization (duodenal atresia), vascular accidents (intestinal atresias),
intrauterine volvulus, intussusception, or intraluminal obstruction (meconium
ileus). Esophageal obstruction causes polyhydramnios, absent visible
stomach and is related to tracheo-esophageal anomalies. Duodenal
obstruction seen as two anechoic cystic masses is associated to aneuploidy
(trisomy 21) and polyhydramnios. Jejuno-ileal obstruction produces dilated
anechoic (fluid-filled) serpentine masses and bowel diameter of 1-2 cm. Large
bowel obstruction is most often caused by meconium ileus, Hirschsprung's
disease or imperforate anus. The colon assumes a large diameter and the
meconium is seen echogenic during sonography. In general the method of
delivery is not changed by the intrauterine diagnosis of intestinal obstruction.
Timing can be affected if there is evidence of worsening intestinal ischemia
(early delivery recommended after fetal lung maturity).
Back to Index

C. Prenatal CCAM
Congenital cystic adenomatoid malformation is a lung bud lesion characterize
by dysplasia of respiratory epithelium caused by overgrowth of distal
bronchiolar tissue. Prenatally diagnosed CCAM prognosis depends on the size
of the lung lesion and can cause: mediastinal shift, hypoplasia of normal lung
tissue, polyhydramnios, and fetal hydrops (cardiovascular shunt). Classified in
two types based on ultrasound findings: macrocystic (lobar, > 5 mm cysts,
anechoic, favorable prognosis) and microcystic (diffuse, more solid,
echogenic, lethal). Occurs as an isolated (sporadic) event with a low rate of
recurrence. Survival depends on histology. Hydrops is caused by vena caval
obstruction, heart compression and mediastinal shift. The natural history is
that some will decrease in size, while others disappear. Should be follow with
serial sonograms. Prenatal management for impending fetal hydrops has
consisted of thoraco-amniotic shunts (dislodge, migrate and occlude), and
intra-uterine fetal resection (technically feasible, reverses hydrops, allows lung
growth). Post-natal management consist of lobectomy.
Back to Index

X. PEDIATRIC LAPAROSCOPY
A. Physiology of the Pneumotperitoneum

The concept behind minimally invasive surgery is that the size of the wound has a direct
correlation with the metabolic and endocrine response to surgical trauma. The greater the
cutting of fascia, muscle and nerve the higher the catecholamine and catabolic response of
the body to surgical trauma.
A potential working space during video-laparoscopic abdominal procedures in children is
established with the help of a carbon dioxide pneumoperitoneum. The most popular technique
used in children for developing a pneumoperitoneum is the open (Hasson) technique, usually
in children less than two years of age. Closed or percutaneous (Veress needle) technique is
mostly practice in older children and adolescents. Insufflation by either technique will cause
an increase in intrabdominal pressure (IAP). Studies during congenital abdominal wall defects
closure such as gastroschisis and omphalocele has shown that the rise in IAP may cause
decrease venous return, decrease renal perfusion, low splanchnic flow, and increased airway
pressures. In addition, abdominal distension causes pulmonary function abnormalities such
as decreased functional residual capacity, basilar alveolar collapse, and intrapulmonary
shunting of deoxygenated blood. The cardiac afterload will increase, an effect that may be
magnified by hypovolemia.
Hypotension during the establishment of the pneumoperitoneum is a very feared
complication. It could be the result of vascular injury, arrhythmia, insufflating too much carbon
dioxide, impending heart failure, gas embolism or the development of a pneumothorax. We
generally insufflate a three-kilogram baby with ten millimeters of mercury of intra-abdominal
pressure and a 70-kilogram child with a maximum of fifteen mm of Hg.
Increase awareness of the intrinsic effects carbon dioxide insufflation may cause in the
child abdominal cavity is necessary. Carbon dioxide is absorbed by the diaphragmatic
surfaces and cause hypercapnia, respiratory acidosis, and pooling of blood in vessels with
decrease cardiac output. This effect is usually controlled by the anesthesiologist increasing
minute ventilation by 10% to 20% to maintain normocapnia. Increase dead space or decrease
functional residual capacity caused by the Tredelenberg position and administration of volatile
anesthetic agents can increment this problem. High risk children where this effect can be
potentiate further are those with pre-existent cardio-respiratory conditions causing increase
dead space, decrease pulmonary compliance and increase pulmonary artery pressure and
resistance. It is estimated that carbon dioxide accumulates primarily in blood and alveoli due
to the decrease muscular components to buffer the excess absorbed gas present in children.
After the procedure, the combination of residual carbon dioxide in the diaphragmatic surface
and water forms carbonic acid that upon absorbtion by the lymphatics produces referred
shoulder pain. There is always a small risk of ventricular dysrhythmia with insufflation of
carbon dioxide in children.
Some contraindications for performing laparoscopy during the pediatric age are: history of
severe cardio-pulmonary conditions, uncorrectable coagulopathy, prematurity, distended
abdomen with air or ascites, and multiple abdominal scars from previous operative
procedures.
Back to Index

B. Laparoscopic Cholecystectomy
Laparoscopic Cholecystectomy (LC) has become the procedure of choice for the removal
of the disease gallbladder of children. The benefit of this procedure is obvious: safe, effective,
and well tolerated. It produces a short hospital stay, early return to activity and reduced
hospital bills. Several technical differences between the pediatric and adult patient are: lower
intrabdominal insufflation pressure, smaller trocar size and more lateral position of placement.
Complications are related to the initial trocar entrance as vascular and bowel injury, and those
related to the procedure itself, i.e., bile duct injury or leak. Three 5 mm ports and one 10-mm
umbilical port are used. Pneumoperitoneum is obtained with Veress needle insufflation or
using direct insertion of blunt trocar and cannula. Cholangiography before any dissection of
the triangle of Calot using a Kumar clamp is advised by some workers to avoid iatrogenic
common bile duct (CBD) injuries during dissection due to anomalous anatomy, and the best
method to detect CBD stones. Treatment of CBD stones may consist of:
1- endoscopic sphincterotomy followed by LC,
2- open (conventional) or laparoscopic choledochotomy, or
3- transcystic choledochoscopy and stone extraction.

Children with hemolytic disorders, i.e., Sickle cell disease, have a high incidence of
cholelithiasis and benefit from LC with a shorter length of postop stay and reduced morbidity.
San Pablo Medical Center performed 4439 cholecystectomies from January 1990 to July
1995; 83 (1.8%) of them in children. LC was found superior to the open conventional procedure
reducing the operating time, length of stay, diet resumption, and use of pain medication. The
child is more pleased with his cosmetic results and activities are more promptly established.
We also found that CBD stones can be managed safely with simultaneous endoscopic
papillotomy and costs of LC are further reduced employing re-usable equipment and selective
cholangiographic indications.
Back to Index

C. Laparoscopic Appendectomy
Semm, a gynecologist, is credited with inventing laparoscopic appendectomy in 1982. With
the arrival of video-endoscopic procedures the role of laparoscopic appendectomy in the
management of acute appendicitis in children has been studied and compared with the
conventional open appendectomy. General advantages of laparoscopic appendectomy
identified are: ease and rapid localization of the appendix, ability to explore and lavage the
entire abdominal cavity, decrease incidence of wound infection, less cutaneous scarring, more
pleasing cosmetically, and a rapid return of intestinal function and full activity. There is
certainly some advantage in doing laparoscopic appendectomy in the obese child, teenage
female with unclear etiology of symptoms, for athletes, children with chronic right lower
quadrant abdominal pain, and cases requiring interval appendectomy. Disadvantages are:
expensive instrumentation, time-consuming and tedious credentialing, and the major benefit
is in the postop period.
Analyzing the results of several series that compare laparoscopic vs. conventional
appendectomy in the management of acute appendicitis we can conclude that laparoscopy
produces no difference with open appendectomy in respect to operating room complications
and postoperative morbidity, has a longer operating and anesthesia time, higher hospital
costs, a shorter length of stay, less postop pain, less pain medication requirement, and
shorter convalescence. One series warned that complicated cases of appendicitis done by
laparoscopy could increase the postoperative infectious rate requiring readmission.
Otherwise, they all favored laparoscopic appendectomy in the management of appendicitis.
Still, unresolved issues in my mind are: Does laparoscopic appendectomy reduce
postoperative adhesions? , Is it necessary to remove a normal looking appendix during a
negative diagnostic laparoscopy performed for acute abdominal pain? , Will the increase
intrabdominal pressure alter the diaphragmatic lymphatic translocation of bacteria favoring
higher septic rates in complicated cases? Experimental evidence in animal models favors
higher rates of systemic sepsis after sequential development of pneumoperitoneum.
Back to Index

D. Laparoscopy for the Undescended Testis

The undescended testis identified in 0.28% of males can be palpable (80%) or non-palpable
(20%). It is difficult to determine either location or absence of the non-palpable undescended
testis by clinical examination. Imaging studies (Ultrasound, CT Scan, Magnetic resonance,
gonadal venography) are not reliable in proving its absence. Diagnostic laparoscopy is reliable
in finding the non-palpable undescended testis or proving its absence. Furthermore it can be
combine to provide surgical management. After reviewing several series, with non-palpable
undescended testes managed by laparoscopy the following three findings were identified:
1- The testis is present; in either an intra-abdominal (38%) or inguinal position (12%).
Intrabdominal testes can be managed by first stage laparoscopic internal spermatic vessel
clipping and cutting (Stephen-Fowler's), followed by second stage vas-based standard
orchiopexy six to nine months later. Inguinal testes are managed by standard inguinal
orchiopexy.
2- The testis is absent (vanishing testicular syndrome) as proven by blind ending vas and
testicular vessels (36%). These children are spare an exploration. If the vas and vessels exit
the internal ring, inguinal exploration is indicated to remove any testicular remnant as
histologic evidence, although I have found useful removing the testicular remnant by the

laparoscopic approach. The presence of a patent processus vaginalis may suggest a distal
viable testis.
3- The testis is hypoplastic, atretic, or atrophic (26%), in which case is removed
laparoscopically.
Exact anatomical localization of the testis by laparoscopy simplifies accurate planning of
operative repair; therefore, is an effective and safe adjunct in the management of the
cryptorchid testis.
Back to Index

E. Groin Laparoscopy
The issue of contralateral exploration in the pediatric inguinal hernia patient has been hotly
debated. Proponents of routine contralateral exploration cite the high percentage of
contralateral hernia a/o potential hernia found at exploration, the avoidance of the cost of
another hospitalization, psychological trauma and anxiety to the child and parents over a
second operation, and the added risk of anesthesia of a second procedure. Most pediatrics
surgeons habitually explore the contralateral side. They disagree in opinions about
exploration depending upon the primary site of inguinal hernia, age, sex and the use of
herniography or some intra-operative technique to check the contralateral side.
Recently the use of groin laparoscopy permits visualization of the contralateral side. The
technique consists of opening the hernial sac, introducing a 5.5-mm reusable port,
establishing a pneumoperitoneum, and viewing with an angle laparoscope the contralateral
internal inguinal ring to decide the existence of a hernia, which is repaired if present. Requires
no additional incision, avoids risk of vas deferens injury in boys, is rapid, safe and reliable for
evaluating the opposite groin in the pediatric patient with unilateralinguinal hernia. Children
less than two years of age have a higher yield of positive contralateral findings.
Back to Index

F. Laparoscopic Splenectomy
Laparoscopic splenectomy is another safe and technically feasible video-endoscopic
procedures in children. Indications are usually hematological disorders such as Idiopathic
thrombocytopenic purpura, spherocytosis, and Hodgkin's staging. Technical considerations
of the procedure are based on anatomical facts such as the variability in the splenic blood
supply, the ligaments anchoring the organ and the size of the diseased spleen. Generally the
avascular splenophrenic and colic ligaments are cauterized, the short gastric and hilar vessels
are individually ligated with metallic clips or gastrointestinal staplers, and the spleen is placed
in a plastic bag, fracture or morzelized until it is removed through the navel.
Comparing the laparoscopic procedure with the conventional splenectomy, the advantages
are: improved exposure, decreased pain, improved pulmonary function, shortened
hospitalization, more rapid return to normal activities and excellent cosmetic appearance.
Disadvantages are longer operating time, higher costs and the need to open 5-20% of cases
due to technical uncontrolled hemorrhage, such as bleeding from the splenic artery.
Back to Index

G. Laparoscopic Fundoplication
Fundoplication for the management of symptomatic gastroesophageal reflux (GER) is
another procedure that has evolved recently taking advantage of minimally invasive technique.
Indications for performing either the open or laparoscopic fundoplication is the same, namely:
life threatening GER (asthma, cyanotic spells), chronic aspiration syndromes, chronic
vomiting with failure to thrive, and reflux induced esophageal stricture. Studies comparing the
open versus the laparoscopic technique in the pediatric age have found a reduced mean
hospital and postoperative stay with laparoscopy. The lap procedure seems similar to the
open regarding efficacy and complication rates. Costs are not excessive, they are even lower
if we take into consideration the shorter length of stay. Lower rate of adhesions, pulmonary
and wound complications are another benefit of the lap technique suggested. Percutaneous

laparoscopic gastrostomy can be done concomitantly for those neurologically impeded

children refer with feeding problems and GER.
Whether to do a complete (Nissen) or partial (Toupee, Thal, or Boix-Ochoa) wrap relies on
the experience of the surgeon with the open procedure. He should continue to do whatever
procedure he used to perform using open surgery. Long-terms results of complications or
recurrence of GER after laparoscopic fundoplication are still pending publication.
Back to Index

XI. SUGGESTED READING

A. Specific Readings
1- Lugo-Vicente HL: Pediatric Surgery Update, Monthly Newsletter, San Juan,
Puerto Rico. Vol 1-7, 1993, 1994, 1995, 1996
2- Coran AG: Vascular Access and Infusion Therapy. Seminars in Pediatric
Surgery 1(3): 173-241, 1992
3- Altman RP: Liver and Biliary Tract Diseases. Seminars in Pediatric Surgery
1(2):97-171, 1992
4- Gans SL, Grosfeld JL: Current Concepts of Pediatric Oncology. Seminars in
Pediatric Surgery 2(1):1-82, 1993
5- Lugo-Vicente HL: Colangiopatas Infantiles. En: Jos Ma. Valoria Villamartn,
editor. Ciruga Peditrica. Madrid: Diaz de Santos, 1994; pags. 210-220
Back to Index

B. General Reading
1- Welch KJ, Randolph JG, Ravitch MM, O'Neill JA, Rowe MI: Pediatric Surgery.
4th edition. Chicago. Year Book Medical Publishers. 1986
2- Ashcraft KW, Holder TM: Pediatric Surgery. 2nd edition. Philadelphia. W.B.
Saunders Co. 1993
3- Grosfeld JL: Common Problems in Pediatric Surgery. 1st edition. St Louis.
Mosby Year Book. 1991
4- Ashcraft KW, Holder TM: Pediatric Esophageal Surgery. 1st edition.
Orlando. Grune & Stratton, Inc. 1986
5- Seeds JW, Azizkhan RG: Congenital Malformations: Antenatal Diagnosis,
Perinatal Management and Counseling. 1st edition. Maryland. Aspen
Publishers, Inc. 1990
6- Puri P (ed): Congenital Diaphragmatic Hernia. Mod Probl Paediatr. Basel,
Karger, Vol 24, 1989

Back to Index
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PERIODICAL ELECTRONIC NEWSLETTER of Interest to Primary Physicians, Pediatricians, Surgeons, Residents,
Medical Students, Nurses, and Health-related professionals dealing with current issues and reviews in the practice of
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Vol. 16 No. 03 MARCH 2001 Issue:

Congenital Pouch Colon
Adrenal Hemorrhage
Neonatal Laparoscopy
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PEDIATRIC SURGERY
UPDATE
Index /Database
| A|B|C|D|E |F|G | H|I|J |K|L|M|N|O|P| Q |R|S |T|U|V |W|X |Y |Z|

A
Abdominal Compartment Syndrome
Acalculous Cholecystitis
Achalasia
Acute Pancreatitis
Adrenal Hemorrhage
Adreno Cortical Carcinoma
Airway Foreign Bodies
Alarming Hemangiomas
Anal Fissures
Ankyloglossia
Annular Pancreas
Appendicolith
Askin Tumor
Asplenia
Asymtomatic Malrotation

B
Baker Cysts
Barrett's Esophagus
Beckwith-Wiedemann Syndrome
Bezoars
Bilateral Cryptorchidism
Bile Duct Injury
Bile Duct Rhabdomyosarcoma
Bile-Plug Syndrome
Bile Peritonitis
Biliary Atresia
Biliary Hypoplasia
Bladder Rhabdomyosarcoma
Bleeding Nipple
Blue Rubber Bleb Nevus Syndrome
Bone Marrow Transplant - Central Venous Catheters
Bowel Management
Branchial Cleft Fistulas
Breast
Breast Rhabdomyosarcoma
Bronchogenic Cysts
Burkitt's Lymphoma
Byler's Disease

C
Candidemia
Carcinoids
Carcinoid Syndrome
Caroli's Disease
Castleman's Disease
Cat Scratch Disease
CDH-Delayed Presentation
Cervical Lymphadenopathy
Central Venous Catheter Sepsis
Christmas Tree Deformity
Cholecystokinin
Choledochal Cyst
Chronic Intestinal Pseudo-Obstruction
Chylothorax
Chylous Ascites
Cloacal Exstrophy
Congenital Adrenal Hyperplasia
Congenital Diaphragmatic Hernia: Intrauterine Repair
Congenital Diaphragmatic Hernia Study Group
Congenital Hydrocele: When to do Surgery?
Congenital Lobar Emphysema
Congenital Mesoblastic Nephroma
Congenital Pouch Colon
Congenital Pre-Pyloric Antral Membrane
Congenital Torticollis
Currarino Triad
Cystic Fibrosis Prenatal Diagnosis
Cystic Neuroblastoma
Cytokines
Cystosarcoma Phylloides

D
Deep Venous Thrombosis in Central Venous Lines
Denys-Drash Syndrome
Dermoids Cysts
Diaphragmatic Eventration
Did You Know...
Dumping Syndrome
Duodenal Stenosis
Duplications
Dysgerminoma

E
Echo in CDH
Ectopic Pancreas
Epignathus
Esophageal Atresia and Tracheo-esophageal Fistula
Esophageal Diverticulum
Esophageal Hernias
Esophageal Perforation

Esophageal Polyps
Ewing's Sarcoma

F
Familial Adenomatous Polyposis
Fetal Abdominal Wall Defects
Fetal Intestinal Obstruction
Fetal Neuroblastoma
Fibrolamellar Carcinoma
Fibrosarcoma
Fibrous Hamartoma
Fistula-in-ano
Fournier's Gangrene
Frantz Tumor

G
Gallbladder Dyskinesia
Gallstones
Gaucher's
Gallbladder Disorders
Gallstone Pancreatitis
Gastric Duplication
Gastric Volvulus
Gastric Perforation
Gastro-Esophageal Reflux Disease
Gastroesophageal Reflux and Neurological Impairment
Gastroesophageal Reflux and Respiratory Symptoms
Gastroschisis with Intestinal Atresia
Giant Cystic Meconium Peritonitis
Golytely: How much to give?
Grave's Disease
Groin Laparoscopy for Inguinal Hernias

H
Hemangiopericytoma
Henoch-Schnlein Purpura
Hepatic Cysts
Hepatic Cysts: Sclerotherapy
Hepatic Hemangioendothelioma
Hepatoblastoma
Heterotaxia Syndrome
Hirschsprung's Associated Enterocolitis
Hirschsprung's Disease
Hirschsprung's Disease: Colostomy?
Hydrometrocolpos
Hypothermia

Idiopathic Perforation Bile Ducts

Idiopathic Thrombocytopenic Purpura
Infantile Myofibromatosis
Inoperable Wilms' Tumor
Imperforate Anus: Female Concepts
Imperforate Anus: Males Decisions
Imperforate Hymen
Incidental Appendectomy
Inguinal Hernia in Prematures
Insulinoma
Internet: Overall
Internet Part 2
Intestinal Atresias
Intestinal Leiomyosarcomas
Intestinal Neuronal Dysplasia
Intestinal Non-Hodgkin's Lymphoma
Intra-Abdominal Lymphangiomas
Intussusception: Air or contrast reduction?
Ivemark's Syndrome

J
Juvenile Polyps

K
Klippel-Trenaunay Syndrome

L
Labial Adhesions in Infants
Laparoscopic Adrenalectomy
Laparoscopic Appendectomy
Laparoscopic Cholecystectomy
Laparoscopic Cholecystectomy vs Open Cholecystectomy
Laparoscopic Fundoplication
Laparoscopic Ladd's Procedure
Laparocopic Pyloromyotomy
Laparoscopic Splenectomy
Laparoscopy for Non-palpable Undescended Testis
Laryngo-Tracheal Clefts
Left Hypoplastic Colon Syndrome
Li-Fraumeni Syndrome
Lipoblastoma
Liver Focal Nodular Hyperplasia
Lumbar Hernias
Lymphedema
Luteinizing Hormone-Releasing Hormone

Macklin Effect
Malone Stoma
Malrotation
Manometry in Imperforate Anus
Meckel's Diverticulum
Meconium Ileus
Meconium Ileus-like Syndrome
Meconium Peritonitis
Mediastinal Cysts
Medullary Thyroid Carcinoma
Megacystis, Microcolon Intestinal Hypoperistalsis Syndrome
Mesenteric Cysts
Mesocolic Hernias
Microgastria
Midline Neck Masses
Mirizzi's Syndrome
Mixed Gonadal Dysgenesis
Morgagni Hernias
Mllerian Duct Syndrome
Mllerian Inclusions
Multiple Endocrine Neoplasia
Multiple Endocrine Neoplasia Type I
Munchausen by Proxy
Muscle Biopsy
Myofibromatosis

N
NEC and Primary Peritoneal Drainage
Neck Adenopathy
Neonatal Jaundice
Neonatal Laparoscopy
Neonatal Ovarian Cysts
Neonatal Testicular Torsion
Nephrectomy in Neuroblastoma
Nesidioblastosis
Net Address!
Neuroblastoma
Neuroblastoma - Genetics
Neuroblastoma - Stage III
Neuroblastoma - Stage IV
Neurenteric Cysts
Neurofibromatosis
Newborn Ostomy
New ECMO System
Nodular fasciitis

O
OK-432
Omphalocele
Omphalo-Mesenteric Remnants
Omphalopagus
On Hirschsprung's Disease Etiology
Ovarian Tumors
Overwhelming Post-Splenectomy Infection

P
Pancreas Development
Pancreas Divisum
Pancreatic Pseudocysts
Pancreatic Trauma
Pectus Carinatum
Perforated Appendicitis
Peutz-Jeghers Syndrome
Pheochromocytoma
Physiology of Pneumoperitoneum
Pilomatrixoma
Pilonidal Sinus
Postoperative Intussusception
PRAPS
Preauricular Sinus
Preduodenal Portal Vein
Prenatal Congenital Cystic Adenomatous Malformation
Prenatal Choledochal Cyst
Prenatal Cystic Hygroma
Prenatal Inguinal Hernia
Prenatal Ovarian Cysts
Primary Hyperparathyroidism
Primary Immunedeficiency
Profile Surgery Section AAP
Pulmonary Sequestration
Pure Tracheo-Esophageal Fistula
Pyloric Stenosis Revisited
Pyocele
Pyriform Sinus

R
Ranula
Rhabdomyosarcoma
Rhabdomyosarcoma - Genetics
Rectal Duplication
Rectal Prolapse
Recurrent Abdominal Pain
Recurrent Intussuception
Recurrent Tracheo-Esophageal Fistula
References?
RET oncogene
Retroperitoneal Teratomas

S
Sacrococcygeal Dimples
Sacrococcygeal Teratoma

Segmental Volvulus
Septate Gallbladder
Short Bowel Syndrome
Sigmoid Volvulus
Spherocytosis
Splenic Cysts
Splenic Trauma
Splenoptosis
Splenosis
Submit!
Superior Mesenteric Artery Syndrome
Superior Vena Cava Syndrome
Surgical Response in Newborns

T
Testicular Feminization Syndrome
Testicular Teratoma
The Pediatric Inguinal Hernia: Is Contralateral Exploration Justified?
Thymic Cysts
Thyroglossal Duct Cyst
Thyroid Cancer
Thyroid Nodules: FNAB
Tongue Cysts
Total Colonic Aganglionosis
TPN Induced Cholestatic Jaundice
Tracheomalacia
Trans-Anal Pull-Through Video
Typhlitis

U
Umbilicus
Undescended Testis: Early Surgery
Urachal Remnants

V
VACTERL
Vaginal Agenesis
Vaginal Rhabdomyosarcoma
Vascular Access: Neonates

W
Warning!
Water in Neonates
Wound Infections
Wilms Tumor and Hyaluronic Acid
Wilms Genetics

Z
Last updated: March 2001

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PEDIATRIC SURGERY UPDATE

VOL 16 NO 02 FEBRUARY 2001
Neuroblastoma Stage IV
Stage IV Neuroblastoma (metastatic NB) refers to high risk group of children with the primary tumor in the adrenal
gland, mediastinum or pelvis associated with disease progression in other sites (bone marrow, cortical bone, liver,
lymph node). Role of surgery in stage IV NB is controversial. Cure will require control of the primary tumor and
elimination of metastatic disease. For infants with metastatic NB a more than 95% resection has been found adequate
surgical treatment either initially or after effective chemotherapy. Adding ipsilateral lymph node dissection does not
appear to affect survival. Delayed surgery after several courses of chemotherapy may be as effective as initial
resection and is associated with fewer complications statistically. Resection without induction chemotx results in
significant blood loss. High risk NB usually invades blood vessels and surrounding structures precluding resection.
Intensive preop chemotherapy reduces tumor size and invasiveness allowing surgical removal. A fibrotic capsule
forms with less blood supply to the tumor. Stage IV NB is best managed with initial chemotx until distant metastasis
are controlled followed by primary gross tumor removal (even in the face of significant tumor reduction) and
completion chemotx. Gross complete resection is best accomplished when a good partial response is obtained.
Radiotx is added to unresectable lesions. Even when chemotx changes the tumor histology (Shimada) from
unfavorable to favorable this does not improve overall outcome. Resection is not confounded by biology of the tumor
(n-myc status). Survival is improved with kidney preservation during surgery. Local control of disease is a
prerequisite for successful bone marrow transplantation.

References:
1- DeCou JM, Bowman LC, Rao BN, Santana VM, Furman WL, Luo X, Lobe TE, Kumar M: Infants with metastatic
neuroblastoma have improved survival with resection of the primary tumor. J Pediatr Surg 30(7): 937-941, 1995
2- La Quaglia MP, Kushner BH, Heller G, Bonilla MA, Lindsley KL, Cheung NKV: Stage 4 neuroblastoma diagnosed at more
than 1 year of age: Gross total resection and clinical outcome. J Pediatr Surg 29(8): 1162-1166, 1994
3- Shamberger RC, Allarde-Segundo A, Kozakewich HPW, Grier HE: Surgical management of stage III and IV neuroblastoma:
Resection before or after chemotherapy? J Pediatr Surg 26(9): 1113-1118, 1991
4- Haase GM, O'Leary MCO, Ramsay NKC, Romansky SG, Stram DO, Seeger RC, Hammond GD: Aggressive surgery
combined with intensive chemotherapy improves survival in poor-risk neuroblastoma. J Pediatr Surg 26(9): 1119-1124
5- Shorter NA, Davidoff AM, Evans AE, Ross AJ 3rd, Zeigler MM, O'Neill,JA Jr: The role of surgery in the management of
stage IV neuroblastoma: a single institution study. Med Pediatr Oncol 24(5):287-91, 1995
6- Tsuchida Y, Yokoyama J, Kaneko M, Uchino J, Iwafuchi M, Makino S, Matsuyama S, Takahashi H, Okabe I, Hashizume K,
et al: Therapeutic significance of surgery in advanced neuroblastoma: a report from the study group of Japan. J Pediatr Surg
27(5):616-22, 1992

Incidental Appendectomy
Removing a normal appendix incidentally during a surgical procedure done for reasons other than abdominal pain is
associated with a small but definite increase in adverse postoperative outcome. In this respect incidental
appendectomy has been found to increase the incidence of postoperative septic complications (wound infection). It
is neither cost-effective as an estimated 36 incidental procedures would be needed to prevent one case of
appendicitis. As any procedure it increases adhesion formation from surgical manipulation in the right lower
quadrant fossa. In potentially contaminated primary procedures the addition of incidental appendectomy does not
increase operative morbidity or mortality. Incidental appendectomy is indicated in procedures where a potential
diagnostic pitfall can occur such as Ladds procedure for malrotation, diagnostic laparoscopy for right quadrant pain
and surgically reduced ileo-colic intussusception.

References:
1- Wen SW, Hernandez R, Naylor CD: Pitfalls in non-randomized outcomes studies. The case of incidental appendectomy with
open cholecystectomy. JAMA 274(21):1687-91, 1995
2- Miranda R, Johnston AD, O'Leary JP: Incidental appendectomy: frequency of pathologic abnormalities. Am Surg
46(6):355-7, 1980
3- Mulvihill S, Goldthorn J, Woolley MM: Incidental appendectomy in infants and children. Risk v rationale. Arch Surg
118(6):714-6, 1983
4- Sugimoto T, Edwards D: Incidence and costs of incidental appendectomy as a preventive measure. Am J Public Health
77(4):471-5, 1987
5- Addiss DG, Shaffer N, Fowler BS, Tauxe RV: The epidemiology of appendicitis and appendectomy in the United States. Am

J Epidemiol 132(5):910-25, 1990

6- Ritchey M, Haase GM, Shochat SJ, Kelalis PP: Incidental appendectomy during nephrectomy for Wilms' tumor. Surg
Gynecol Obstet 176(5):423-6, 1993

Currarino Triad
Congenital caudal anomalies that include anorectal malformation, sacral bony abnormality and a presacral mass is
known as the Currarino Triad (CT); an autosomic dominant hereditary syndrome described in 1981 caused by
abnormal separation of neuroectoderm from endoderm. The anorectal malformation associated with CT is stenosis
(or agenesis) of the distal rectum causing intractable constipation (chief complaint of this triad) or intestinal
obstruction. Sacral agenesis and abnormalities of the os sacrum (scimitar sacrum, hemisacrum with preserved first
sacral vertebra) are the most common bony anomalies identified. Most frequently the presacral mass in CT is
reported to be an anterior meningocele, a benign teratoma, enteric, dermoid cyst or a combination. Though prenatal
diagnosis can be made, most cases are diagnosed postnatally in the first decade of life. Routine pelvic x-rays should
be done in all cases of anorectal stenosis. Pelvic ultrasound and x-rays in patients with history of chronic
constipation since early childhood will suggest the diagnosis. MRI is the study of choice detecting the presacral
mass and any anomalies of the spinal canal (tethered cord syndrome caused by the presacral mass). Management
consists of excision of the presacral mass and repair of the anorectal malformation. A gene associated with CT has
been mapped to the terminal portion of the long arm of chromosome 7 (7q36).

References:
1- Kirks DR, Merten DF, Filston HC, Oakes WJ: The Currarino triad: complex of anorectal malformation, sacral bony
abnormality, and presacral mass. Pediatr Radiol 14(4):220-5, 1984
2- Kochling J, Pistor G, Marzhauser Brands S, Nasir R, Lanksch WR: The Currarino syndrome--hereditary transmitted
syndrome of anorectal, sacral and presacral anomalies. Case report and review of the literature. Eur J Pediatr Surg
6(2):114-9, 1996
3- Lee SC, Chun YS, Jung SE, Park KW, Kim WK: Currarino triad: Anorectal malformations, sacral bony abnormality, and
presacral mass - A review of 11 cases. J Pediatr Surg 32(1): 58-61, 1997
4- Gudinchet F, Maeder P, Laurent T, Meyrat B, Schnyder P: Magnetic resonance detection of myelodysplasia in children with
Currarino triad. Pediatr Radiol 27(12):903-7, 1997
5- Zia-ul-Miraj M, Brereton RJ: Currarino's triad: an unusual cause of constipation in children. Pediatr Surg Int 13(5-6):437-9,
1998
6- Samuel M, Hosie G, Holmes K: Currarino triad-Diagnostic dilemma and a combined surgical approach. J Pediatr Surg
35(12):1790-1794, 2000

Submit!
We are inviting all of you to submit contribution of your own to the newsletter.
Credit will be given to the authors keeping his or her name, institution and
E-mail address on their work.
A list of contributors will appear in the internet homepage.
All works should be send to Dr. H. Lugo-Vicente to the e-mail: titolugo@coqui.net

*Edited by: Humberto L. Lugo-Vicente, MD, FACS, FAAP

P.O. Box 10426, Caparra Heights Station, San Juan, Puerto Rico 00922-0426
Tel (787)-786-3495 Fax (787)-720-6103 E-mail: TITOLUGO@COQUI.NET
Internet Address:HTTP://HOME.COQUI.NET/TITOLUGO
Copyright Office of the Library of Congress 1997

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Pediatric Surgery Update ISSN 1089-7739
Last updated: March 2001

ROLE OF INTERNET IN PEDIATRIC

SURGERY
by : Humberto L. Lugo-Vicente, MD, FACS, FAAP *
San Juan, Puerto Rico

Abstract
Internet, the largest network of connected computers, is becoming the ultimate frontier to
access information for health providers. This review focus on how developments of this
communication technology have become a useful educational resource in Pediatric
Surgery, and describes modest ideas in computer network use.
Internet basic resources are electronic mailing (E-mail), discussion groups, file transfer,
and browsing the World Wide Web (WWW). E-mail brings physicians with common
interest together. Surgeons employ it as a communicating tool. Legal and social
responsibility is bounded with its use. Discussion groups permits debate including
clinical cases, operations, techniques, research, career opportunities, and meetings. File
transfer provides the opportunity of retrieving archives from public libraries. The WWW is
the most resourceful tool due to its friendly interface and ease of navigation.
The average physician needs to know almost nothing on how computers work or where
they came from to navigate through this pandemonium of information. Click and play with
today graphical applications encourage the computer illiterate to connect. Establishing
the connections envelops the need of hardware, software and a service provider.
Future development consists of online journals with new ideas in peer-review and
authentication, telemedicine progression, international chatting, and centralization of
pediatric surgery cyber space information into database or keyword search engines.
INDEX WORDS: pediatrics, surgery, internet

Introduction
Internet is the largest network of connected computers. More than 30 million computers
exchanging physical links through a standard protocol of communication. A super avenue
of information and transactions (1). The Net is affecting every aspect of life and
dissemination of information relevant to medicine for the health community is not immune
to this technology.
The busy surgeon who invests little time searching the literature could find himself with a
clinical practice that does not keep pace with recent medical advances. Informatics option
to stay updated in the discipline of Pediatric Surgery includes access to printed periodical
publications, regular meetings, congress assistance, digital database storage, and Internet
resources.
Text, journals, and books are usually outdated by the time they reach the regular subscriber.
Not to mention cost of subscription, printing and storage capabilities needed. Meeting and
congress dynamic regular sessions can be costly, and access to the full written report is
almost never achieved until print publication of the paper is obtained usually six months to
one year later. Digital databases (i.e., CD-ROM) store large amount of information, but prices
of CD are overwhelming. An additional driver is needed as hardware for reading the stored
material. Information is becoming an unlimited commodity, we can have as much as we
want at no cost, but are limited by our storage capacity (2).
By agreeing to a set of operating protocols, users have developed innovative techniques to

seek out information from different databases accessible via the network along with
methods for sharing documents. Internet provides immediate downloadable information and
dynamic information on every aspect of life. Still the idea that it represents a frustrating
educational event in computing persists. The average person needs to know almost nothing
on how computers work or where they came from to navigate through this network. Click
and play with today graphical applications encourage the computer illiterate to connect.
The purpose of this review is to highlight how newly ways of communication using Internet
navigational technology can be useful educational resources in Pediatric Surgery, and
clarify concepts of network communication for future use by physicians.

History of Internet
After the postwar years military intelligence was searching for strategic forms of
communication in the after-match of a nuclear holocaust, a system that would defeat current
centralized tendencies in communication. The notion of creating several nodes of super
computers that convey each other through standard telephones line was developed.
Sending the information in small packages that would meet at the other end of the line using
a uniform protocol of communication and regrouping (TCP/IP). These nodes would be
created around different parts of the world divided in either top level geographical or
institutional domains like: government (gov), commercial (com), educational, (edu), military
(mil), network resources (net), and other organizations (org).
Scientists were the first to use this system in an effort to consolidate research and establish
electronic communication in the flow of new projects. This created an atmosphere of social
behavior and effective long distance communication as more nodes grew in each country.
Curiously, the initial electronic discussion group developed among scientists was called the
Science-Fiction list (3, 4).
World Wide Web (WWW), the crowning glory of the Internet, is developed in Geneva,
Switzerland in 1989. The WWW provides a user friendly interface with the capacity to send
and receive information through Internet using text, graphics, audio and video utilizing a
protocol of marked language (5). Seen today as the best resource to post information that
can reach and be accessed in almost every corner of the planet.

Uses of Internet for the Pediatric Surgery community

Internet basic resources are:
electronic mailing (E-mailing),
discussion groups (news groups and list servers),
file transfer,
and the WWW.
Through the initial effort of scientists to establish communication using electronic text
mailing convenience over the postal service was foreseen. E-mailing is faster than postal
mail. It takes an average of two to eight minutes for messages to arrive to another computer
node in very distant geographical zones. The message is stored by the internet service
provider (ISP) until the electronic box owner retrieves the message. You do not have to pay
extra for e-mailing, and is global in scope. Files can be attached to messages up to half a
megabyte in size (a megabyte represents one million characters).
News groups and list servers with discussion interest have developed both in pediatrics and
surgery. Messages posted by authors to the list or discussion group are automatically
mailed to all subscribers. Posting growth to such lists includes United States, Central and
South America, Europe, Middle East, Africa, and Australasia to mention a few. List servers
for different surgery and pediatric sub-specialties exist: NICU-Net, PICU-Net, cardiology,
gastroenterology, neurology, emergency medicine, critical care, Pediatric pain, etc. (6, 7).
A popular list among Pediatric Surgeons worldwide is called the Pediatric Surgery List.
Originally developed by Thomas Whalen for topics discussion that includes clinical cases,
operations, techniques, research, career opportunities, and meetings. Intended for pediatric
surgeons and interested general surgeons and residents (8). Although the list is in
embryological phase, growth will inevitably create a medium of international discussion

without precedent. A constant forum for exchange of ideas, difficult cases, consensus on
management, and development of our specialty.
File transfer provides the unique opportunity of retrieving archives from public file libraries.
Free software is also available. Downloading of data into the hard disk of your computer is
very straightforward. Anti-viral programs are available to monitor each access file that can
become part of your system whenever you download them from Internet.
Recent poll of the Pediatric Surgery Internet list server members regarding what resource of
the Net they use most of the time was done. Almost one-fourth (23%) of the list population
(58/246) answered the survey. Electronic mailing (personal and list server/discussion
groups) occupied 83% of resources, web browsing 16%, and long distance computing 1%.
Pediatric surgeons with access to the Net use it mostly as a communication tool. WWW
browsing is slowly developing as a second alternative probably due to absent access to a
web browser connection.

E-mailing uses and responsibility

Electronic mailing is the most useful resource of Internet. Mailing lists bring people with
common interest together (9). Through it physicians have developed news, chat, and list
group discussion. This creates the perfect environment to consult colleagues on a clinical
problem, send draft of a paper for peer revision, read journals without paying subscription
rates, maintain your continuing medical education credits, and retrieve anything the same
day that it is published (1). It will become an essential tool in medical research, teaching
medical students, clinical practice, postgraduate studies, and continuing medical education.
The lack of a traditional peer-review process and author identification might prevent E-mail
text from being taken as authoritative (10).
The common user of the Net is a professional. Environmental motivations have created an
informal code of conduct known as net-etiquette. By this is meant politeness in replying.
Along with accessibility, identification and social responsibility (11).
Netters (defined as common user of the Net), resent several iatrogenic web disorders: not
waste the carrying capacity of the Net (bandwidth), posting unsolicited advertising
(spamming), and observing inappropriate online behavior (1). Chain E-mail letters can
overcrowd your electronic site. Other problems related to the nature of e-mailing that we
must be aware are: sign your posting so that we can know who is writing, do not reply
publicly to the whole group when answering privately to one person, and avoid including the
entire text of the original message in your reply.
A hot debate among frequent E-mail list servers involves being careful when answering or
replying, specially when the answer will hit many members of a list server group. The
inclusion of your name and address at the end of your E-mail text represents a legal
signature for all aspect of the law: the author name type in ASCII characters (10). Simple
rules to observe are: avoid using patients' names, address, record numbers or institutional
demographics. When personally responding to electronic medical consultation by an
unknown online patient ask yourself: Is he your patient behind the monitor? Have you
examined him or review his past medical record? Will my answer be used as possible legal
evidence in case this is unintended? The potential for abuse while looking at this
information will always exist.
Disclaimers notice stating the medico-legal responsibility behind frequent response to
complex medical problems are being asked for to list server administrators (12). This if a
response from some member commentator triggers a change in diagnosis or therapy in a
given discussion case that causes ultimate damage to the patient involved. The
commentator cannot be held responsible of his answer in as much as he had no clear
physician-patient relationship, was not paid for this service, or had the opportunity to
examine the patient or his medical charts. The disclaimer should include that particular
consultation or advice was not the idea of the answer, reliance on this comments should not
be done, and printed versions of the E-mail should not appear in any patient medical record
(12).

WWW and Pediatric Surgery

WWW can be seen as an endless book, where each page has a divergent story. The web
page is the basic unit of information and hypertext provides links to other pages in the same
directory, or in different domain sites of the network (5). What makes the WWW the most
resourceful area of Internet is the ability to watch text, images, video, audio, and real time
pictures embedded in web pages.
Each page has a unique address, also known as uniform resource locator (URL). URL
essential ingredients are protocol, domain name, and directory. For example the URL of
Pediatric Surgery Update' is: http://home.coqui.net/titolugo/index.htm. This means that the
protocol is http, the domain name /home.coqui.net/, and the file "index.htm" is the web index
page under "/titolugo/" directory.
Pediatric Surgery Update', the periodical electronic newsletter started on July 1993 as a
print form. Initially covered short issues and reviews in the discipline of pediatric surgery.
December 1995 marked its development as a web site. The WWW introduction of the print
version permitted development of further areas such as: review articles with images, graphs
and tables, survey section, technical innovation area, a Pediatric Surgery Online Handbook
for residents and medical students, and an area for medical students to developed research
and writing skills (13).
Departments and Section of Pediatric Surgery have developed their own web page in the
WWW. Through them we have access to such content as: faculty members, facilities,
research programs, interests, residency and fellowship programs, and other pediatric and
medically relevant links. Continuous medical education credits are part of some web site
offering.

Page in the Net, HTML and graphic applications

A web page, the basic unit of the WWW consists of all one medium, such as text, or can
include multiple media including graphics, sounds, animation, and video (14). The web page
is built using a mark language that is plain text tagged with handles <text>. This is known as
hypertext mark-up language (HTML). HTML can easily be learned for later production of a
web page. The National Center of Supercomputing Application (NCSA) Beginner's Guide to
HTML is used by many to start to understand the hypertext markup language used on the
WWW. It is an introduction and does not pretend to offer instructions on every aspect of
HTML (15). Computer applications developed as web editors provide the functionality
needed to construct a web page with little knowledge of HTML. They are called WYSISYG
(What You See Is What You Get) Editors.
Movement between web pages is accomplished by links to other universal resource locators
or Internet address. Links can be in different color or underlined text where the cursor of
your pointer device changes as though sensing an executable movement. By either clicking
the device or hitting the return key, you will be moving to that link. Some links are just
libraries composed of downloadable files.
Web editors can be downloaded from different suppliers in the Net. Some are free but most
can be obtained as shareware to try them for a limited period. For a list of HTML editing
tools or programs available the reader is referred to URL:
http://sdg.ncsa.uiuc.edu/~mag/work/HTMLEditors/windowslist.html

Establish the Connection

To gain access to Internet you will need hardware, software, and a service provider.
Hardware is your computer. This includes a monitor, central processing until (CPU) and
keyboard. Macintosh and Widows operating systems ease of use graphical environments
have prevailed during the last years over the more text-based disk operating system (DOS).
A modem is another piece of hardware needed that will provide the telephone line
communication.

Computer software that help you navigate the web is known as web browser. Web browsers
are in essence a navigational aid for moving around and between the various nodes and
links of the WWW (14). Some web browsers are non-graphic like Lynx, and graphical like:
Mosaic, Netscape, and MS Internet Explorer. Netscape is the most widely used and industry
standard full-features web browser. Latest versions of this software can be downloaded free
from their respective site (URL) in the web (16,17).
Internet service providers (ISP) are either private or universities based. The service provider
will give you access to the Net using a local or toll-free telephone number. Some may
include web space with the monthly rate offer. A university-based ISP usually provides
service for a nominal or none rate. Electronic addresses of such users usually end in the
suffix -edu. Most physicians with Internet access have it through academic affiliation (9)
Once connected, the Net is a pandemonium of information with no central index. The user
will rely on automated index or search engines. Search engines collect database, retrieve
programs, or harvest them (2). A collection of search engines can be found at URL:
http://www.webcom.com/webcom/power/index.html (The WebCom Power Index). Specific
search engines in the field of medicine will help create an atmosphere of librarian resource.
Editors like Spooner's Ped-Info and Lehmann's Points of Pediatric Interest, have developed
web sites with collection of information specifically oriented toward pediatric content. The
web site has been maintained as a set of WWW pages through which you can link to:
Departments of Pediatrics, professional organizations, pediatric practices, Children
Hospitals, medical and surgical subspecialties, on-line publications, and pediatric software
of interest. Criteria for entry into the database are that the resources must have appeal to
pediatricians, and specific pediatric content. Both web sites allow easy access to pediatric
information on the WWW for health care professionals and parents (18, 19).

Future of the Net in Pediatric Surgery

The future of Internet will be an unbounded multimedia circus. Real time video and audio
technology will permit us view recorded in vivo sessions made in another location paid for
through a local phone call. In vivo videos of laparoscopic procedures have already taken
place between two continents (United States and Argentina). With the use of a personal
computer and a modem, they have transmitted the surgical procedure through live
broadcast, via the Net, for the nominal price of a local phone call. Resident surgeons
watched and interacted during the surgical procedure. This opens an area by which
academic medicine can be telecasted to other parts of the world with least bearing on
economical resources (20).
Since anyone can publish in the Net online, electronic journals will develop with new
peer-review concepts. Editors, reviewers, and authors will need to adjust to the use of this
information technology. Online publication in Pediatric Surgery will increase as printed form
of actual journal joins the cyberspace domain. Less paper work on publishing companies
may mean a reduction in subscription price, with e-mailing guarantees of providing
manuscript of written and published articles.
Cyber citations as proposed by the American Psychological Association or Modern
Language Association have yet to be standardized by the American Medical Association to
be used as bibliographical style (21). Authors that use Online references will need to keep
printed or digital file copy of such articles, since there is no way to avoid drastic changes or
movement done to this domain address (22).
International chatting is another area of future development for our pediatric surgery
community. Using simple downloadable application like mIRC (internet relay chat) you can
connect to an undernet organization channel and chat with groups of people at the same
time (23). The International Pediatric Chat channel developed by J. Edlavitch use two weekly
sessions to maintain the group online (24).
Telemedicine refers to the use of telecommunication technology to simplify health care
delivery or distribute medical informatics. Some specific projects represented by this
technologic are: Multi campuses linking of hospitals and research centers, linkages
between rural health clinics and central hospital, physician-to-hospital links for transfer of

patient information, diagnostic consultations, patient scheduling, research, literature

searches, video program distribution for public education on health care issues, use of
video and satellite relay to train health care professionals in widely distributed or remote
clinical settings, and transfer of diagnostic information such as electrocardiograms or
X-rays. Some benefits are improved access to areas in needs of health service, reduce cost
of traveling, reduces professional isolation, and improving the quality of care given.
Development of the infrastructure needed along with cost containment issues are two of the
problems faced by this technological advance (25).
Most Pediatric Surgery Organizations (Surgical section AAP, APSA, CAPS, BAPS, etc.) will
find themselves generating web sites of their own during the next few years. This will add to
the pandemonium of information already established. A future trend in development will be
the need to gather all this information in a Pediatric Surgery Cyber Web site with database
keyword access. This way a centralized path will exist to organize the varied information
buried in the Net.
We must be aware of the negative effects of expansion of computerized information. The
WWW can be an intoxicating and seductive place. Long hours glued to the small screen,
surfing the cyberspace, and reading E-mail can cause social degradation, increasing
disparity and isolation of the individual. Fragmentation of knowledge can be the result.
Users must continue to maintain an equilibrium to avoid such side-effects (26,27).

Conclusions
The exponential growth of Internet in the disciplines of Pediatric Surgery will cause a
change in patient care, teaching and research. Changes in our specialty will be nurtured
through the international use of information posted in the Net. Main use by contemporary
pediatric surgeons is as a communicating tool using electronic mailing with WWW browsing
slowly growing.
Future developments consist of online journals with new concepts in peer-review and
authentication, telemedicine, international chatting, and centralization of cyber space
information into database or keyword search engines. Marketing is another frontier in the
development of medical informatics technology.

References
1- Pallen M: Guide to the Internet: Introducing the Internet. BMJ 311: 1422-1424, 1995
2- Thomas P. Copley (September 17, 1996) Make the Link Workshop: Tutorial Number Five
[Online] Available: http://www.crl.com/~gorgon/links.html [September 20, 1996]
3- Johnson Phillip (May 8, 1996) History of the Internet [Online] Available:
http://dragonfire.net/~Flux/ihistory.html [September 15, 1996]
4- Sterling Bruce (July 5, 1994) Short History of the Internet [Online] Available:
http://www.forthnet.gr/forthnet/isoc/short.history.of.internet [September 15, 1996]
5- Pallen M: Guide to the Internet: The World Wide Web. BMJ 311: 1552-1556, 1995
6- Christopher U. Lehmann (1996) Point of Pediatric Interest E-mail Discussion Lists [Online]
Available: http://www.med.jhu.edu/peds/neonatology/elists.html#elists [September 6, 1996]
7-Tarczy-Hornoch P: NICU-Net: An Electronic Forum for Neonatology. Pediatrics 97(3):
398-399, 1996
8- PEDIATRIC SURGERY Mail List Subscription Address: Majordomo@UMDNJ.edu
Subscription Message: Subscribe PEDSURG-L Human administrator: Thomas V. Whalen,
Professor, Robert Wood Johnson Medical School, E-mail: whalen@umdnj.edu
9- Spooner SA: On-Line Resources for Pediatricians. Arch Pediatr Adolesc Med 149:
1160-1168, 1995

10- Elliot SJ, Elliot RG: Internet List Servers and Pediatrics: Newly Emerging Legal and
Clinical Practice Issues. Pediatrics 97(3); 399-400, 1996
11- Frisse ME, Kell EA, Metcalfe ES: An Internet Primer: Resources and Responsibilities.
Academic Medicine 69(1): 20-24, 1994
12- De Ville, K.A. Internet Listservers and Pediatrics: Newly Emerging Legal and Clinical
Practice Issues II. Pediatrics 98: 453-454, 1996
13- Lugo-Vicente HL (September 16, 1996) Pediatric Surgery Update [Online] Available:
http://home.coqui.net/titolugo/index.htm [September 18, 1996]
14- Thomas P. Copley (September 17, 1996) Make the Link Workshop: Tutorial Number Four
[Online] Available: http://www.crl.com/~gorgon/links.html [September 20, 1996]
15- NCSA (September 1996) A Beginner's Guide to HTML [Online] Available:
http://www.ncsa.uiuc.edu/General/Internet/WWW/HTMLPrimer.html#A1.10.1 [September 18,
1996]
16- Microsoft Corp (1996) Internet Explorer Home [Online] Available:
http://www.microsoft.com/ie/ [September 21, 1996]
17- Netscape Communication Corp (1996) Download Netscape Navigator Software [Online]
Available: http://home.netscape.com/comprod/mirror/client_download.html [September 21,
1996]
18- Spooner SA (August 1994) Ped Info a Pediatric Web Server [Online] Available:
http://www.uab.edu/ped-info [September 16, 1996]
19- Lehmann C (1996) Points of Pediatric Interest [Online] Available:
http://www.med.jhu.edu/peds/neonatology/poi.html [September 22, 1996]
20- Telbelian A (September 1996) NOMC broadcasts live surgery via the Internet [Online]
Available: http://laparoscopy.com/pictures/vdconf.html [September 17, 1996]
21- Li X., Crane N. (May 20, 1996) Bibliographic Formats for Citing Electronic Information
[online]. Available: http://www.uvm.edu/~xli/reference/estyles.html [September 15, 1996]
22- Aruzen MA: Cyber Citations: Documenting Internet Sources Presents Some Thorny
Problems. Internet World Sept 1996, pag 72-74
23- mIRC Co. Ltd. (1996) Homepage of mIRC [Online] Available:
http://www.geocities.com/SiliconValley/Park/6000/ [September 22, 1996]
24- Edlavitch Julius (September 1996) Home Page of International Pediatric Chat [Online]
Available: http://www.pedschat.org/ [September 19, 1996]
25- William Frederick, Moore Mary (August 1995)Telemedicine: Its Place on the Information
Highway [Online] Available: http://naftalab.bus.utexas.edu/nafta-7/telepap.html [September
7, 1996]
26- Thomas P. Copley (September 17, 1996) Make the Link Workshop: Tutorial Number Two
[Online] Available: http://www.crl.com/~gorgon/links.html [September 20, 1996]
27- Gravanis MB: Computers and social isolation [Letter to the editor] The Pharos 59 (3): 50,
1996
____________________________
* Associate Professor in Pediatric Surgery, University of Puerto Rico School of Medicine.
Member of SCOT (Section of Computers and Other Technologies) Section of the American
Academy of Pediatrics.
Address reprints request to: Humberto Lugo-Vicente, MD- P.O. Box 10426, Caparra Heights

Station, San Juan, Puerto Rico 00922-0426 Tel. 787 / 786-3495 Fax 787 / 720-6103 E-mail:
titolugo@coqui.net
Date posted: March 1, 1997

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Submit your contribution...

We are inviting all of you to submit contribution (REVIEWS OR ARTICLES) of
your own to the newsletter.
This is free, on a voluntary basis, and credit will be given to the authors
keeping his or her name, institution and E-mail address on their work.
A list of contributors will appear on the homepage.

Simple Rules to submit work:

1- REVIEWS or ARTICLES should deal with Pediatric Surgery.
2- Academically oriented (Remember: medical students, residents, fellows,
primary physicians, pediatric physicians, and surgeons are reading your
work).
3- Back-up by references, i.e., J Pediatr Surg 30(9):1288-1289, 1995.
4- For REVIEWS: the text should be between 150 and 250 words long. Short
title or abbreviated i.e., CDH: ECMO Pitfalls.
5- Summary of recent work to-be/or published are good.
6- Send your name, institution and E-mail address with the work.
7- Minor editing changes will be made (to be sure they fit the page) and send
back to you for approval.
8- Authors will be sole proprietors (Copyright) of their work.
9- To complete a section of the Pediatric Surgery HANDBOOK: use less than 1000 words text.
Images or graphs should be send as *.GIF or *.JPG format.

10- All works should be e-mail to:TITOLUGO@COQUI.NET

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new techniques in pediatric surgery

Excision of Branchial Cleft Fistulas
Those branchial cleft fistulas that originate from the 2nd branchial cleft are by far the most commonly
found. They display themselves as small cutaneous opening along the anterior lower third border of
the sternocleidomastoid muscle, communicates proximally with the tonsillar fossae, and can drain
saliva or a mucoid secretion. Management consists of excision since inefficient drainage may lead to
infection.
I have found that dissection along the tract (up to the tonsillar fossa!) can be safely and easily
accomplished after probing the tract with a small guide wire (0.018 in.) in-place. This will prevent
injury to nerves, vessels and accomplish a pleasantly smaller scar.
Technique: Make an elliptical incision around the pit area. Hold still the skin around the pit area with
several silk 5-0 sutures. Gently introduce the straight part of the guide wire up until it stops. Ask the
anesthesiologist to determine if the guide wire can be felt in the oral cavity (rare). Tie the guide wire to
the silk sutures holding the removed skin so that it won't dislodged during dissection. Using a needle
tip cautery and keeping traction on the tied skin-wire dissect the tract staying as near as possible to
the fistulous tract until it can be safely removed. Occasionally a second stepladder incision in the neck
will be required depending on the length of the tract.

Esophago-Myotomy for Achalasia

Achalasia in children is an uncommon esophageal motor disorder distinguished by clinical,
radiological and manometric features. Clinical presentation is characterized by progressive
dysphagia, regurgitation, weight loss, chest pain and nocturnal cough. Diagnosis is established by
barium swallow and confirmed by manometry and motility studies. Primary therapy is surgical
(Heller's modified esophagomyotomy), and results are similar after a transabdominal or thoracic
approach.
I prefer to do the esophagomyotomy through the abdomen. Children above the age of four benefit
from a midline upper incision. Below that age a transverse supra-umbilical incision gives better
exposure, safer closure, and good cosmetic results.
Technique: After mobilizing circumferentially the distal esophago-gastric junction a purse string
suture is placed in the proximal fundus of the stomach near the G-E junction. A 16-G foley catheter is
threaded through the gastrostomy into the distal esophagus and once in the thoracic esophagus the
balloon is inflated. The foley is slowly brought toward the stomach, the balloon will hold in the stenotic
part of the distal esophagus. A longitudinal incision in the anterior esophageal wall is made avoiding
the vagus nerve, dividing the muscle fibers (preserving the submucosa), and knowing that the
procedure creates an adequate esophageal lumen while passing the inflated foley catheter
concomitantly. This maneuver can be repeated as needed to be sure that the myotomy was
completed. The gastrotomy is closed, crura approximated and the surgeon decides whether to
construct an antireflux procedure or not depending on his preference.

CVC Placement in VLBW infants

Very low birth weight (VLBW) infants (less than 1500 gms) needs multiple venous access to meet all
the fluid, antibiotic and nutritional requirements during periods of intensive supportive care. Central
Venous Catheter (CVC) placement has improved care and survival of these sick infants.
Percutaneous placement of subclavian catheters carries the risk of pneumo/hemothorax, easy
dislodgement, and a significant failure rate of cannulation. An alternative is placement of a 4.2 Fr or
2.7 Fr Broviac catheter through the external jugular vein (EJV) or internal jugular vein (IJV).
Technique: The procedure can be done at the NICU or a nearby OR using either sedation or general

anesthesia. The right side of the neck and retro auricular area is exposed, prep and draped. A small
transverse incision is done over the EJV at the base of the neck. The Broviac catheter is tunneled to
exit somewhere behind the earlobe. Proximal limb of the catheter is threaded through a venotomy into
the superior vena cava/right atrial junction. Alternatively, if the EJV is non-useful, the incision can be
extended medially and the IJV used instead.
Submitted: 04/21/97

TREATMENT OF GASTROESOPHAGEAL REFLUX WITH A GASTRIC TUBE

CARDIOPLASTY
by: Oktay Mutaf MD
Gastroesophageal reflux is common in small children and either is asymptomatic or can usually be
controlled with conservative maneuvers. Nevertheless, in some special group of patients, like the
neurologically impaired and acquired GER after an esophageal injury due to ingested caustic
substances etc. will inevitably need antireflux surgery some time during their early childhood. The
most popular antireflux surgical techniques used in children are Nissen, Thal and Boix-Ochoa
procedures. These techniques have a significant recurrence rate even in the best hands. The reason
for this is inherited in the techniques themselves. In all above mentioned antireflux procedures fundic
or crural muscles are sutured to the distal esophagus to create an acute angle of His with various
degrees of fundic wraps (180 - 360 degrees) around the esophagus. The fundus of the stomach or
the crura have very heavy and strong muscles when compared with the poor esophageal
musculature. In a small child this difference is much more obvious when compared with that of an
adult. As a result strong diaphragmatic and/or fundic contractions can be enough to tear off a wrap
shortly after the operation or may be, as the child gets older and taller, the wrap may slip from the
distal esophagus ending up with an obtuse angle of His. The aim is to try to find a way of creating a
relative relapse proof antireflux barrier for GER patients.
Technique: Reflux esophagitis is first controlled with use of adequate antacid therapy before
operative interventions (Omeprazole 1-2 mg/kg/day). After instituting general anesthesia the patients
were esophagoscoped, dilated as necessary and a 10-mm outer diameter PVC tube is placed
trans-orally into the stomach. The patent in supine position is laparotomized via a mid line abdominal
incision. The triangular ligament is divided and the liver retracted laterally. An opening is made in the
gastroepiploic membrane 2 cm long to the left of the esophagus. The lower jaw of a TA 60-3.5
stapling instrument (Auto Suture; US Surgical Corp., Norwalk, CT) is placed through this opening
along the posterior wall of the stomach. The intragastric PVC tube is positioned in the lesser
curvature and the stapler is placed next to the tube vertically parallel to the lesser curvature. The
instrument is closed and the titanium staples fired. The instrument and the PVC tube are removed. As
a result, a 6 cm long gastric tube is created starting from the esophagogastric junction and traveling
along the lesser curvature down the stomach. A nasogastric tube is placed and the incision closed.
Details: http://www.med.ege.edu.tr/~pedsurg/om-plasty.htm
Submitted: 10/13/00

Submit your technique

If you have developed a technique in Pediatric Surgery that would like to share with our surgical
community, please don't hesitate to send it to Dr. Humberto Lugo-Vicente for inclusion in this
section of Pediatric Surgery Update.
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Last updated: March 2001

HUMBERTO L. LUGO-VICENTE, MD, FACS, FAAP

Editor-in-Chief
Pediatric Surgery Update
Associate Professor of Pediatric Surgery
University of Puerto Rico, School of Medicine
Associate Director - Department of Pediatric Surgery
University Pediatric Hospital, Medical Science Campus, Rio Piedras, Puerto Rico
Chief- Section of Pediatric Surgery
San Pablo Medical Center, Bayamn, Puerto Rico

Curriculum Vitae
Weekly Schedule
Physical
Address:
Suite 309, Santa Cruz Medical Bldg
Santa Cruz # 73
Bayamon, Puerto Rico 00961

Postal
Address:
P.O. Box 10426
Caparra Heights Station
San Juan, Puerto Rico 00922-0426

Tel/Fax:
Private office (787) 786-3495
University Children Hosp (787) 777-3535 Ext 7242 and 7240
Fax (787) 720-6103

E-mail:
TITOLUGO@COQUI.NET
Last updated: March 2001

'Pediatric Surgery Update' is registered in

the United States Copyright Office of the Library of Congress .

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Pediatric Surgery Update Review

Articles
Index
BILIARY ATRESIA: An Overview
IMPACT OF MINIMALLY INVASIVE SURGERY IN CHILDREN
NEURONAL INTESTINAL DYSPLASIA: A Role for Surgery?
ROLE OF INTERNET IN PEDIATRIC SURGERY
THE PEDIATRIC INGUINAL HERNIA: Is Contralateral Exploration Justified?
TRICHOBEZOAR IN A 11-YEAR OLD GIRL: A Case Report
DELAYED DIAGNOSIS AND MANAGEMENT OF BRONCHIAL RUPTURE FOLLOWING BLUNT
THORACIC TRAUMA - Carlos E. Prieto-Velhote, Manoel C. Prieto-Velhote, Tais F.de Oliveira-Velhote
Last updated:March 2001

SUBMIT! SUBMIT! SUBMIT!

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NEURONAL INTESTINAL DYSPLASIA:

A Role for Surgery?

by: Humberto L. Lugo-Vicente, MD. FACS, FAAP*

Pediatric Surgeon
During the past two decades motilities disorders of bowel function in infants and children
have been distinguished with the help of histochemical, radiographics and manometric
studies performed to patients. The surgeon is occasionally asked to render care to many of
this unfortunate children in the hope that sphincterotomy, enterostomies, resections, and
pull-through surgery can yield a cure or improvement of symptoms.
Motilities disorders plague the clinical picture of the child with recurrent bouts of constipation,
diarrhea, enterocolitis, bloody stools, abdominal distension, colicky abdominal pain, and
encopresis. Symptoms can be present since birth, or develop later in the child's life. There is
progressive constipation and megacolon formation in many of this infants.
Normal propulsive motility of the gastrointestinal tract is dependent on normal anatomy,
musculature and innervation of the bowel wall. Control is exerted by extrinsic and intrinsic
nerves, chemoreceptors and mechanoreceptors located in the bowel wall that unfortunately
are practically inaccessible for investigation and experimentation (1).
Gastrointestinal motility disorders are viewed as those about the esophagus (i.e. achalasia),
gastro-pyloric area (i.e. hypertrophic pyloric stenosis), small bowel, colonic (Hirschsprung's
disease, neuronal intestinal dysplasia), and pseudo-obstructive problems such as the
adynamic bowel of prematures and chronic intestinal pseudo-obstruction (CIPO) (1).
Using colorectal biopsy specimens patients with inborn errors of colonic innervation have
been classified as: aganglionosis (52%), hypoganglionosis (5%), and neuronal intestinal
dysplasia (43%). An additional half of the biopsies will not fit this classification due to
moderate malformation such as dysganglionosis, hypogenetic and heterotopic nerve cell
characteristics (2).

NID
Neuronal intestinal dysplasia (NID), first described by Meier-Ruge in 1971, is a poorly
understood colonic motility disorder of neuronal structure in the bowel wall (3). NID is
characterized by several histochemical and pathological findings such as:
1- hyperplasia of submucous and myenteric plexus with formation of giant ganglia,
2- isolated ganglion cells in the mucosal lamina propia and between muscle layers of
muscularis mucosa,
3- moderate elevation of acetylcholinesterase in the parasympathetic fibers of mucosal lamina
propia and circular muscle, and
4- hypoplastic or aplastic myenteric plexus sympathetic innervation (4,5).
The most characteristic alteration identified is acetylcholinesterase elevation of
parasympathetic fibers, and the less reliable diagnostic feature, the giant ganglion cells. Not
all patients demonstrate the whole spectrum of histological traits depicted above.
The etiology of NID has eluded us. The development of NID in previously normal bowel, the
association with other intestinal malformations, and the clinical heterogeneity of this patients
suggest that NID is a reaction of the neural intestinal system caused by congenital obstructive
factors or inflammatory disease (6). Major histocompatibility complex II expression has been
found markedly elevated in Hirschsprung's Disease (HD) and NID cases (normally nerve tissue
is deficient of the antigen). This has lent support that the bowel may be highly susceptible to
an abnormal response of immune origin (7).
Initially described as a localized and disseminated form of disease, Fadda in 1983 re-classify it

into two types ( A and B), with a common clinical feature in both: chronic constipation and
megacolon (8). In type A the disease is confined to the colon causing a functional bowel
obstruction with acute onset. Symptoms are present since birth and comprise: constipation,
ulcerative colitis, painful straining, and bloody stools. Contrast studies of the colon display
rigid, spastic segmental contraction of bowel, ulcer, erosions, and no peristalsis. Manometric
studies will show absent recto-inhibitory reflex (4,5,8,9,10). Histologically there is aplasia or
hypoplasia of myenteric sympathetic innervation and increase acetylcholinesterase activity in
lamina propia, circular muscle and muscularis mucosa (5,9). Ganglion cells are present,
excluding the diagnosis of Hirschsprung's disease (HD).
Type B NID is more common, symptoms commence around six month of life, there is
constipation and adynamic distal bowel with megacolon undistinguishable from
Hirschsprung's disease. Histology is characterized by dysplastic parasympathetic submucous
plexus with giant ganglion cells and hyperplasia, elevated acetylcholinesterase levels, and
isolated ganglia in lamina propia (5,8,9,10). This type is more commonly found associated to
HD, anorectal malformations, MEN IIB syndrome and CIPO (8). Manometry shows that these
patients have non-proportional relaxation of the internal anal sphincter, anorectal
hyperexcitability, and increase amplitude of anorectal fluctuations (11).

NID and HD
It is estimated that isolated NID is eight time rarer, and affects longer segments of bowel than
Hirschsprung's Disease (HD) (5,12). Both disease process have been reported in 20-75% of
patients (10). This has shifted the attention to patients with HD who persists with clinical
problems after adequate pull-through resection.
HD is characterized by lack of enteric ganglion cells, hyperplasia of abnormal nerve fibers and
a non-propulsive, non-relaxing segment of bowel. Classically the etiology is attributed to a
failure in cranio-caudal migration of parasympathetic neural crest cells to the distal bowel.
Factors leading to failure of differentiation after migration of neural crest cells could be
responsible for HD complex etiology. A plausible explanation for the failure of relaxation of the
bowel involved is a deficiency of enteric inhibitory nerves that mediates the relaxation phase
of peristalsis. This nerves are intrinsic to the gut and are classified as non-adrenergic and
non-cholinergic. Nitric oxide (NO) has recently been implicated as the neurotransmitter that
mediates the relaxation of smooth muscle of the GI tract in HD. It's absence in aganglionic
bowel might account for the failure of relaxation during peristalsis. Besides, adhesions
molecules (absent in aganglionic bowel) during early embryogenesis might restrict the
neuro-ectodermal origin involved in the initial contact between nerves and muscle cell
(synaptogenesis) suggesting that developmental anomaly of innervated muscle and absent
NO causes the spasticity characteristic of HD (1,2,7,13,14.,15). Initial management consist of
leveling colostomy in ganglionic bowel with later pull-through surgery.
Patients with symptoms of obstruction (constipation, enterocolitis) persisting after surgery for
HD could be hastened by: mechanical (anastomotic stricture) reasons, functional (NID,
residual aganglionosis) problems, and infectious etiology (C. Difficile) (16). Sonographic
follow-up analysis of colonic motility in patient with HD and NID after corrective surgery for HD
shows that with time the dysmotility changes of the ganglionic NID colon improves (17).
Retrospectives studies evaluating the influence of retained NID colon in patients with repaired
HD have identified that the NID segment of bowel can be preserved without increasing the risk
of morbidity or mortality to them (18). The actual incidence of NID associated with HD could be
explored by monitoring the histological findings of the proximal bowel during initial colostomy
construction. Biopsy of this colostomy segment should warned us of the presence of NID
changes.

NID and CIPO

Chronic Intestinal Pseudo-Obstruction (CIPO) is a rare disorder of intestinal motility in infants
and children characterized by recurrent attacks of abdominal pain, distension, vomiting,
constipation and weight loss lacking obvious mechanical lesions. The disease can be familial
or sporadic. Suggested etiology is degeneration of enteric nervous or smooth muscle cells.
The diagnosis is based an history, physical exam, radiographies and motility studies. X-Ray

hallmarks are: absent strictures, absent, decreased or disorganized intestinal motility, and
dilated small/large bowel loops. Associated conditions identified in 10-30% of patients are
bladder dysfunction (megacystis) and neurological problems. Histologic pattern portrayed:
myenteric plexus hyperplasia, glial cell hyperplasia, and small ganglion cells
(hypoganglionosis) (19,20,21). Management is primary supportive: intestinal decompression
(NG), long-term TPN and antibiotic prophylaxis (22,23). Motility agents are unsuccessful.
Venting gastrostomy with home parenteral nutrition has shortened the high hospitalization
rate associated to this disease process24. A similar condition can be seen in early fed
prematures due to immaturity of intestinal motility.
NID accounts for 30% of patients with CIPO symptoms as attested by histologic sections (25).
It is illogical to relate symptoms of dysmotility to submucous plexus changes seen on biopsy,
and almost entirely ignore the myenteric plexus that is ultimately concerned with colonic
motor activity.

Management of NID
Management has switched from a more aggressive attitude to a more conservative approach.
Repeated bouts of obstructive episodes have been an indication for colostomy, and more
serious symptoms have ended with resection (5). The indications for surgery in NID cannot be
sustained alone on the histochemical picture of the biopsy specimen, but on the clinical
situation of the patient, since NID is best a histopathologic condition rather than an unique
clinico-pathological entity (26,27,28).
Type A NID with its acute fulminant course during the neonatal period has an unfavorable
progression with early indications for surgery. Colostomy is reserved for neonatal obstruction
with associated severe enterocolitis. It is probable that many sick infants will show a clinical
picture similar to severe necrotizing enterocolitis before being diagnosed the condition. A
word of caution should be exerted against extensive colonic resections for this disease
process. This could impair colonic water absorption, stool consistency and may overwhelm
fecal incontinence problems (28).
Type B NID runs a more chronic path and management is more conservative. There is clinical
evidence gathered that the colonic motility disturbance associated matures and improves by
the fourth year of life of the child (5,10). If problems persists beyond the fourth year of life
more aggressive management is warranted. In general patients can be managed with saline
colonic irrigations, TPN, high dose lactulose, and paraffin oil until clinical improvement and
normalization of biopsy results are obtained (28,29). Prokinetic agents (cisapride) can be of
help in some groups of patients, with the addition of neostigmine in clinically resistant
cases16. Surgery is rarely deemed necessary in this subgroup of patients.

Conclusions
Neuronal intestinal dysplasia is a poorly understood colonic motility disorder with
characteristic histopathological findings and clinical presentation. It is often associated with
Hirschsprung's disease and can constitute a cause of failure of clinical improvement after
adequate resectional pull-through surgery. Other conditions associated with NID are: CIPO,
anorectal malformations and MEN II syndrome patients.
To increase the diagnostic yield of NID the pathologist should be aware and use
histochemistry evaluation of the rectal biopsy specimen in patients with history of
constipation or unexplained bouts of diarrhea. Adequate sampling of the temporary proximal
colostomy done to HD patients should be examined for NID pathological changes.
Treatment has centered around the clinical picture with most cases managed medically with
prokinetic agents, colonic irrigations, and bowel cathartics until improvement and
normalization of histology occur. There is evidence of progressive maturation of the enteric
nervous system with time. Surgery is indicated for patients with severe clinical deterioration
after failed medical management.

References

1- Molenar JC, Tibboel D, van der Kamp AWM, et al: Diagnosis of Innervation-Related Motility
Disorders of the Gut and Basic Aspects of Enteric Nervous System Development. Prog Pediatr
Surg 24:173-185, 1989
2- Meier-Ruge W: Classification of malformations of colorectal innervation. Verh Dtsch Ges
Pathol 75:384-385, 1991
3- Meier-Ruge W: Ueber ein Erkrankungsbild des Kolos mit Hirschsprung-Symptomatik. Verh
Dtsch Ges Pathol 55:506, 1971
4- Scharli AF, Meier-Ruge W: Localized and Disseminated Forms of Neuronal Intestinal
Dysplasia Mimicking Hirschsprung's Disease. J Pediatr Surg 16(2):164-170, 1981
5- Munakata K, Okabe MI, Sueoka H: Clinical and Histologic Studies of Neuronal Intestinal
Dysplasia. J Pediatr Surg 20(3):231-235, 1985
6- Sacher P, Briner J, Hanimann B: Is neuronal intestinal dysplasia (NID) a primary disease or a
secondary phenomenon? Eur J Pediatr Surg 3(4):228-230, 1993
7- Hirobe S, Doody DP, Ryan DP, et al: Ectopic Class II Major Histocompatibility Antigens in
Hirschsprung's Disease and Neuronal Intestinal Dysplasia. J Pediatr Surg 27(3):357-363, 1992
8- Rintala R, Rapola J, Louhimo I: Neuronal Intestinal Dysplasia. Prog Pediatr Surg 24:186-192,
1989
9- Fadda B, Maier WA, Meier-Ruge W, et al: Neuronal Intestinal Dysplasia: Critical 10-years'
analysis of Clinical and Biopsy Results. Z Kinderchir 38(5):305-311, 1983
10- Pistor G: Functional Colonic Ultrasonography: Normal Findings of Colonic Motility and
Follow-Up in Neuronal Intestinal Dysplasia. Prog Pediatr Surg 24:155-164, 1989
11- Krebs C, Silva MC, Parra MA: Anorectal Electromanometry in the Diagnosis of Neuronal
Intestinal Dysplasia in Childhood. Eur J Pediatr Surg 1(1):40-44, 1991
12- Briner J, Oswald HW, Hirsig J, et al: Neuronal Intestinal Dysplasia- Clinical and
Histochemical Findings and its association with Hirschsprung's Disease. Z Kinderchir
41(5):282-286, 1986
13- Robey SS, Kuhajda FP, Yardley JH: Immunoperoxidase Stains of Ganglion Cells and
Abnormal Mucosal Nerve Proliferations in Hirschsprung's Disease. Human Pathol
19(4):432-437, 1988
14- Gittes GK, Kim J, Yu G, et al: Severe Constipation with Diffuse Intestinal Myenteric
Hyperganglionosis. J Pediatr Surg 28(12):1630-1632, 1993
15- Cuffari C, Rubin SZ, Krantis A: Routine Use of the Nitric Oxide Stain in the Differential
Diagnosis of Hirschsprung's Disease. J Pediatr Surg 28(9):1202-1204, 1993
16- Moore SW, Millar AJW, Cywes S: Long-Term Clinical, Manometric, and Histological
Evaluation of Obstructive Symptoms in the Postoperative Hirschsprung's Patient. J Pediatr
Surg 29(1):106-111, 1994
17- Pistor G, Hofman- von Kap-herr S: Functional Colon Sonography in Neuronal Intestinal
Dysplasia. Fortschr Med 102(14):397-400, 1984
18- Hanimann B, Inderbitzin D, Briner J, et al: Clinical Relevance of Hirschsprung-associated
Neuronal Intestinal Dysplasia. Eur J Pediatr Surg 2(3):147-149, 1992
19- Bindl L, Emons D, Haverkamp F, et al: Megacystis Microcolon Intestinal Hypoperistalsis
Syndrome: A Neuropathy? Z Kinderchir 44(4):249-252, 1989

20- Gil-Vernet JM, Casasa JM, Boix-Ochoa J, et al: Intestinal dysmotility pseudo obstruction.
Cirugia Pediatrica 5(2):87-95, 1992
21- Peck SN, Altschuler SM: Pseudo-obstruction in Children. Gastroenterol Nursing
14(4):184-188, 1992
22- Fonkalsrud EW, Pitt HA, Berquist WE, et al: Surgical Management of Chronic Intestinal
Pseudo-obstruction in Infancy and Childhood. Prog Pediatr Surg 24:221-225, 1989
23- Ament ME, Vargas J: Diagnosis and Management of Chronic Intestinal Pseudo-obstruction
Syndromes in Infancy and Childhood. Arquivos de Gatroenterologia 25(3):157-165, 1988
24- Pitt HA, Mann LL, Berquist WE, et al: Chronic Intestinal Pseudo-obstruction. Management
with Total parenteral nutrition and a venting enterostomy. Arch Surg 120(5):614-618, 1985
25- Milla PJ, Smith VV: Intestinal Neuronal Dysplasia. J Pediatr Gastroenterol Nutr 17:356-357,
1993
26- Schofield DE, Yunis EJ: Intestinal Neuronal Dysplasia. J Pediatr Gastroenterol Nutr
12:182-189, 1991
27- Scharli AF: Neuronal Intestinal Dysplasia. Pediatr Surg Int 7(1):2-7, 1992
28- Koletzko S, Ballauff A, Hadziselimovic F, et al: Is Histological Diagnosis of Neuronal
Intestinal Dysplasia Related to Clinical and Manometric Findings in Constipated Children?
Results of a Pilot Study. J Pediatr Gastroenterol Nutr 17:59-65, 1993
29- Simpser E, Kahn E, Kenigsberg K, et al: Neuronal Intestinal Dysplasia: Quantitative
Diagnostic Criteria and Clinical Management. J Pediatr Gastroenterol Nutr 12(1):61-64, 1991
*Associate Professor in Pediatric Surgery, Department of Surgery, University of Puerto Rico,
School of Medicine, and Universidad Central del Caribe, School of Medicine.
Address reprints to: Humberto L. Lugo-Vicente, MD- P.O. Box 10426, Caparra Heights Station,
San Juan PR 00922-0426. Tel (787) 786-3495 Fax (787) 720-6103 E-mail: titolugo@coqui.net

Published in: Boletin Asociacin Medica de Puerto Rico 87(3-4): 60-63, 1995

>>>BACK TO INDEX<<<

BILIARY ATRESIA: An Overview

by: Humberto L. Lugo-Vicente, MD, FACS, FAAP
Pediatric Surgeon*
Biliary atresia (BA) continues to originate controversy and despair among
physicians and patients alike. With the development of liver transplant and
new techniques in small size orthotopic replacement, a new insight in BA

surgical care has come forth. Where do we stand today in therapy?, What
causes this terrible disease?, and What should be the guidelines in
management of the cholestatic infants? are some issues reviewed in this
monograph.
This condition is the most common cause of persistently direct (conjugated)
hyperbilirubinemia in the first three months of life. Kasai portenterostomy and
liver transplantation battle hand in hand to become today's leading therapy.
One thing is for sure, results after portoenterostomy are decided by the
promptness of the initial work-up and referral to surgery. More than 80% of BA
babies have satisfactory bile flow after hepatic-portoenterostomy if the
procedure is done before their 8th week of life. It's certain, we must view
newborns with persistent cholestasis as urgent cases that need immediate
assessment and management of their condition.

History
Biliary atresia is characterized by progressive inflammatory obliteration of the
extrahepatic bile ducts, an estimated incidence of one in 15,000 live births, and
predominance of female patients (1). The first comprehensive paper was
written by J. Thompson of Edimburg in 1882. J.B. Holmes in 1916 classified
cases as correctable (10-15%), and non-correctable (85-90%) depending on the
pathologic structures identified at the porta hepatis area, and Dr. William Ladd
in 1928 ventures into the first successful bile-enteric anastomosis (2). In 1959
Kasai and Suzuki described a new procedure for biliary atresia that
transformed management during the following 30 years (3). By 1980 most
infants with biliary atresia were managed with the Kasai procedure.

Etio-pathological Considerations
Although much has been written of BA, its pathogenesis remains speculative.
The original theory of an embryogenic accident that settled in occlusion of the
extrahepatic biliary tree, was challenged by the absence of jaundice at birth,
and histologic evidence of patent biliary ducts that progressively disappeared
during the first months of life (4). Findings in the obstetric history of older
parents, high use of drugs, associated illness, and fetal loss suggested the
possibility of exposure to a noxious agent during the reproductive processn
(5).
The disease is the result of an acquired inflammatory process with gradual
degeneration of the epithelium of the extrahepatic biliary ducts causing
luminal obliteration, cholestasis, and biliary cirrhosis (6). The timing of the
insult after birth suggests a viral etiology obtained transplacentally; Reovirus
type 3 has been implicated (7). Up to 68% of infants with BA show antibodies
to Reovirus type 3 in serum, although no viral particle has been isolated (8).
Oral and intraperitoneal administration of reovirus to experimental animals
causes hepatitis and biliary tract inflammation with fibrosis similar to BA
(4,7,9,10).
Almost 20% of patients have associated anomalies such as: polysplenia,
malrotation, situs inversus, preduodenal portal vein and absent inferior vena
cava. This raises the possibility of a genetic mutation and the hypothesis of

laterality with defective development of one side of the body when compared
with the contralateral image side (11,12).
Histopathology is distinguished by an inflammatory process in several
dynamic stages with progressive destruction, scar formation, and chronic
granulation tissue of bile ducts (13). The pathologist cannot be categoric in the
diagnosis of BA, since liver changes are compatible with an extrahepatic
mechanical obstruction. Two changes merits mention: portal tract interlobular
ducts proliferation, and cholestatic histology. Other findings are: giant cell
transformation, focal hepatic cell necrosis, interlobular space and portal tract
fibrosis. Conditions displaying a similar histology are: alfa-1-antitrypsin
deficiency, Alagille's syndrome (hypoplasia of bile ducts), and TPN induced
cholestasis. Immunohistochemistry of the portal ducts can show the presence
of the epithelial membrane antigen in large ducts, changes specific for BA
(13,14).
Three types of microscopic biliary structures have been identified in the most
proximal aspect of the extrahepatic remnant removed surgically near the area
of the porta hepatis. These are: bile ducts with a mean diameter of 500 and
bile in the lumen, collecting ductules of biliary glands with a mean diameter of
250 , and biliary glands without bile and a mean diameter of 100 (15).
Postoperative biliary flow after Kasai correlates with the presence and size of
bile ducts and collecting ductules exclusively (15,16,17,18). Electron
microscopy can exhibit canalicular biliary membrane filaments whose volume
and appearance correlates with adequate bile flow (19). The degree of hepatic
fibrosis associated also relates with post-op biliary flow. Kasai
portoenterostomy relies on the realization that the microscopic structures in
the porta hepatis will act as micro-conduits of bile as an internal biliary fistula
is created with a segment of bowel. All will eventually merged into one or two
ducts.
BA classification is based on findings upon operative cholangiography and the
macroscopically specimen morphology as shown inFigure 1 (percent's are
obtained from the National Biliary Atresia Registry (20)).

Clinical Manifestations and Diagnosis

Physiologic jaundice of the newborn is a common, benign, and self-limiting
condition. Persistent conjugated hyperbilirubinemia (greater than 20% of total
or 1.5 mg%) should be urgently appraised. Initial evaluation should include a
well-taken history and physical exam, partial and total bilirubin determination,
type and blood group, Coomb's test, reticulocyte cell count and a peripheral
smear (21).
Cholestasis means a reduction in bile flow in the liver, which depends on the
biliary excretion of the conjugated portion. Reduce flow causes retention of
biliary lipoproteins that stimulates hypercholesterolemia causing progressive
damage to the hepatic cell, fibrosis, cirrhosis and altered liver function tests
(22). The etiology of the cholestatic infant is classified in several groups as
depicted inTable 1 (23). Those structurally related etiologies are surgical
causes of jaundice.
In BA the patient develops insidious jaundice by the second week of life. The
baby looks active, not acutely ill and progressively develops acholic stools,

choluria and hepatomegaly (24). Non-surgical source of cholestasis shows a

sick, low weight infant who is jaundiced since birth. The diagnostic evaluation
of the cholestatic infant should include a series of lab tests that can exclude
perinatal infectious (TORCH titers, hepatitis profile), metabolic
(alpha-1-antitrypsin levels), systemic and hereditary causes (25,26).
Total bilirubin in BA babies is around 6-10 mg%, with 50-80% conjugated. Liver
function tests are nonspecific. Lipoprotein-X levels greater than 300 mg% and
Gamma Glutamyl Transpeptidase (GGT) above 200 units% suggest the
diagnosis (13). The presence of the yellow bilirubin pigment in the aspirate of
duodenal content excludes the diagnosis of BA.
Ultrasound study of the abdomen should be the first diagnostic imaging study
done to cholestatic infants to evaluate the presence of a gallbladder, identify
intra or extrahepatic bile ducts dilatation, and liver parenchyma echogenicity.
BA sonographic characteristics are: absent, or small gallbladder that does not
contract upon hormonal stimuli, and increased liver echogenicity (27). The
postprandial contraction of the gallbladder eliminates the possibility of BA
even when nuclear studies are positive. This changes are consistent with
neonatal hepatitis (27,28,29).
Nuclear studies of bilio-enteric excretion (DISIDA) after pre-stimulation of the
microsomal hepatic system with phenobarbital for 3-5 days is the diagnostic
imaging test of choice (30). BA patients will show an increase hepatic uptake
during early injection without significant bilio-enteric excretion in delayed
films (24 hrs.) The presence of the radio-isotope in the GI tract excludes the
diagnosis of BA. Hepatocellular causes of jaundice will show poor
concentration of isotope in the liver associated to delayed or absent excretion.
Percutaneous liver biopsy should be the next diagnostic step if previous
studies suggest BA and the infant has no associated coagulopathy (31).
Findings of BA are: bile duct proliferation and fibrosis. Unfortunately this
changes are nonspecific of BA and can be found in neonatal hepatitis (2). The
mini-laparotomy is the final diagnostic alternative. Through a small right
subcostal incision a gallbladder cholangiogram and liver biopsy is done.
Those infant with radiographic evidence of patent extrahepatic biliary tract has
no BA. Small, hypoplastic ducts are associated to Alagille's syndrome (32). In
BA the gallbladder can be a fibrous remnant, present but filled with white bile
(hydrops), with no communication with the biliary tree, or with distal
extrahepatic communication. Once the diagnosis of BA is established
intraoperative, a Kasai enterostomy is constructed.
The prenatal diagnosis of BA was first reported in 1986. Antenatal sonography
showed a cystic structure in the fetal abdomen confirmed as BA 76 hours later
(33). Recently, diagnostic laparoscopy has been found useful in the evaluation
of the cholestatic infant (34).

Management
Medical management of BA is uniformly fatal.
Kasai portoenterostomy has decreased the mortality of BA during the last 30
years. The procedure done before the first 6-8 weeks of life will yield biliary
flow in 75-80% of infants.
Kasai procedure consists of removing the obliterated extrahepatic biliary

system, and anastomosing the most proximal part to a bowel segment.

Adequate illumination and magnifying loupes are essential. The initial
mini-laparotomy incision is extended once the diagnosis is confirmed. The
gallbladder, cystic duct and extrahepatic remnant is mobilized, the distal
common bile duct is ligated proximally and the dissection goes proximally
toward the porta hepatis. At the porta hepatis the remnant looks like a fibrous
cord with the shape of a cone. At this point the cord is transected
perpendicularly to the liver level and the specimen send to the pathologist.
The raw surface left over is anastomosed to a defuntionalize limb of jejunum in
a roux-en-Y fashion using small suture bites. It is estimated that 10-15% of
cases have distal patency of the extrahepatic bile ducts and the gallbladder
can be used as conduit, instead of bowel. This variation in the procedure
eliminates the possibility of developing cholangitis, but increases the
incidence of anastomotic leak from ischemia during dissection
(2,13,24,30,35,36).
Table 2 shows complications associated to Kasai procedure. Cholangitis is
probably the most common, serious, and occur in 90% of patient who drain
bile during the first year of life. Clinically they manifest fever, leukocytosis,
elevated bilirubin in serum, and deterioration of liver function tests.
Management consists of antibiotics. Main cause is ascending infection
through the interposed bowel segment associated to destruction of lymphatic
drainage. Recurrent attacks of cholangitis cause progressive hepatic damage
(37,38). Sonography can show cystic intrahepatic bile duct dilatations in
children with repetitive attacks. This cysts are either non-communicating or
communicating. Management may consist of percutaneous transhepatic
drainage, and sclerosis of non-communicating cysts (39). To reduce the
incidence of cholangitis the prophylactic uses of antibiotics during the first
18-24 months of life is recommended. Constant attacks of cholangitis are
associated to sudden cease of bile flow needing steroid therapy or
re-operation.
In pursuit of reducing the episodes of cholangitis Kasai procedure was
modified providing an external conduit to diminish the intraluminal pressure
and secretory liver gradient. This enabled the measurement of bile volume and
concentration. This external conduits did not reduce the incidence of
cholangitis. Furthermore they were plagued with several complications such
as: enterostomal varices, bile salts deficiency, electrolytes disturbances, and a
later procedures to close the enterostomy (40,41). Not exteriorized interposed
segments of bowel with intussuscepted valves and jejunal interposition
between porta hepatis and duodenum has proved to reduce the incidence of
cholangitis increasing survival (42).
Almost three-fourth of patients will develop portal hypertension in spite of
adequate postoperative bile flow. They will manifest esophageal varices,
hypersplenism, and ascites. Factors associated to this complication are:
history of cholangitis, older infant during surgery, re-operations, and elevated
portal pressure during initial surgery (43). Esophageal varices usually develop
2-8 years after portoenterostomy and are managed with endoscopic
sclerotherapy effectively. Secondary hypersplenism can be managed with
partial embolization of the splenic artery. Ascites will need salt restriction and
diuretics. Later in life a reduction in this complications is linked to the

spontaneous development of portosystemic shunts.

Essential fatty acids malabsorption leading to caloric and nutritional
deficiencies should be managed with high concentration medium chain
triglyceride formulas. This malabsorption could lead to A, D, K, and E fat
soluble vitamin deficiency. Maternal milk is insufficient to provide caloric
needs, and formula supplementation should be provided, even with forced
tube feedings. The objective is to provide 150 calories and 3-4 grams of
protein daily per kilogram of weight, along with vitamin supplementation.
Pruritus is difficult to manage. Antihistamines are first line of treatment due to
their tranquilizing effect. Other drugs used are cholestyramine to reduce
enterohepatic circulation, phenobarbital to increase biliary flow and rifampin.
The new era of liver transplantation, better surgical techniques, smaller donor
accessibility and new inmunosuppresor agents (cyclosporine) has brought
second thoughts to the use of Kasai enterostomy. Major liver transplant
centers see Kasai as a complementary procedure of historic interest. From
75-80% of patient with BA will be candidates for orthotopic liver transplant
(44,45). Some recommendations for those patient initially undergoing a Kasai
procedure who could become future candidates for liver transplantation are:
Use oblique incisions to reduce adhesions to the liver capsule, the roux-en-Y
of jejunum should not be long (40 cm) to be reused later avoiding problems
with cyclosporine absorption, and avoid enterostomies (22).

Results
Without surgical management survival of infants with BA is 8-12 months, most
dying of irreversible liver failure.
Results of portoenterostomy are associated to a group of prognostic factors
studied (seeTable 3). The two most important factors are: age at surgery and
histologic liver changes (46,47). Other factors are: caucasian race,
morphologic type of BA, size of ductal structures at porta hepatis, postop bile
flow, degree of hepatic fibrosis, surgical technique used, type of surgical
reconstruction, incidence of cholangitis, and development of symptomatic
portal hypertension (20,26).
Age at surgery is probably the most determinant factor of survival after Kasai
procedure. Ideally it should be done before 60 days of life. The presence of bile
ducts in the extrahepatic remnant and the degree of liver fibrosis correlates
with the age of the patient. The older the patient the lesser the possibility of
findings adequate size ducts and the worst the hepatic fibrosis. The degree of
hepatic fibrosis and degeneration of intrahepatic ducts correlates directly with
prognosis irrespective of the size of the ductal micro-structures identified in
the porta hepatis area (47,48).
General results of Japanese series fare better when compared to Occident.
This is attributed to a racial influence associated. Caucasian race has a worst
prognosis than oriental children (20). Morphologically those patient with
patent distal extrahepatic ducts and a gallbladder Kasai constructed do better.
This is the result of a more physiologic conduit, a reduced number of
cholangitis episodes, and a low level inflammatory process (49).
The type of surgical reconstruction has no relationship to survival. Continual
attacks of cholangitis will progress to hepatic fibrosis and irreversible damage

related with early development of portal hypertension (20). Postoperative bile

flow correlates with improved immediate survival, with no guarantee of long
term survival.
Results reported by several series can be appreciated inTable 4. Up to 80% of
children undergoing portoenterostomy before 60 days of age will drain bile
and jaundice will improve. At five years survival will be 29-60%, 25-35% at ten
years and 8% at twenty years (13,20,30,49-52).
The greatest mortality in BA occurs during the first two years after
portoenterostomy. Long-term follow-up of children living more than ten years
after Kasai shows moderate hepatic dysfunction, controlled portal
hypertension, a normal intellectual coefficient, and a good quality of life
(49,53).

Conclusions
Persistent jaundice in the newborn must be managed urgently. A diagnosis
should be established early in the life of the child and Kasai portoenterostomy
offered to those infants before their eighth week of life. This will allow that
more than one-third of BA children survive this terrible disease.
Hepatic transplantation is reserved for those patients with failed
portoenterostomy, progressive liver failure or late-referral to surgery. Liver
transplant indications should include patients with bilirubin levels above 10
mg%, low albumin levels, weight loss and uncontrolled ascites.

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51- Akiyama H, Saeki M, Ogata T: Congenital biliary atresia: Our operative

method and the operative results. Jpn J Pediatr Surg 10:673, 1978
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* Associate Professor in Pediatric Surgery, Department of Surgery, University
of Puerto Rico School of Medicine. Assistant Professor Surgery, Universidad
Central del Caribe School of Medicine. Chief- Section of Pediatric Surgery,
Department of Surgery, HURRA and Hospital San Pablo.
Address reprints to: Humberto L. Lugo-Vicente, MD, P.O. Box 10426, Caparra
Heights Station, San Juan PR 00922-0426. Tel. (809)-786-3495 Fax
(809)-720-6103 e-mail: titolugo@coqui.net
Published in Boletn Asociacin Mdica de Puerto Rico. Vol 87 (7-8-9): 147-153, 1995

>>>BACK TO INDEX<<<

THE PEDIATRIC INGUINAL HERNIA:

Is contralateral exploration justified?

by: Humberto L. Lugo-Vicente, MD, FACS, FAAP

Pediatric Surgeon*

From the 1950's until the present, the issue of contralateral exploration in the
pediatric inguinal hernia patient has been fairly hotly debated. Proponents of
routine contralateral exploration cite the high percentage of contralateral
hernia a/o potential hernia (patent processus vaginalis) found at routine
exploration, the avoidance of the cost of a second hospitalization,
psychological trauma and anxiety to the child and parents over a second
operation, the added risk of anesthesia of a second procedure, and the
possibility of development of an incarcerated hernia at a later date (1).
Opponents of routine contralateral exploration states that many needless
procedures are performed to avoid the development of only a few clinical
hernias, that operation lengthened by the contralateral exploration may
augment the risks and cite the increase risk of damage to the cord or gonad by
the procedure itself (2).
A survey by Rowe and Marchildson in 1981, showed that 80% of pediatrics
surgeons habitually explore the contralateral side in male patients, and 90% do
so in females patients (3). Surgeons disagree in opinions about exploration
depending upon the primary site of hernia, age, sex and the utilization of
herniography or some intra-operative technique to check the contralateral
side.
With such a broad variance of opinions regarding routine contralateral
exploration we decided to study our experience and examined 161 successive
patients who underwent bilateral hernia repair by the author during a 30
months period.
MATERIAL AND METHODS
The medical charts of all infants and children who underwent consecutive repair of inguinal
hernias by the same surgeon (HLV) from July 1985 to December 1987 at the Ramon Ruiz Arnau
University Hospital (HURRA) and Hospital San Pablo (HSP), were retrospectively reviewed.
During this period 248 patients were identified, 87 who underwent a unilateral procedure and
161 patients with bilateral inguinal procedures. This last groups of patients comprise the study
group. The charts were reviewed for sex, age at operation, gestational age, diagnostic
characteristic, associated conditions, pre- and postoperative complications, findings during
surgery, and outcome.
The findings during surgery were then compared in the sex, gestational age and age at
operation subgroups to decide the effect that this variable had on the results using chi-square
analysis. A p < 0.01 was considered significant.
The surgical procedure was performed under general endotracheal anesthesia using 3.5x
magnifying loupes. A bilateral transverse inguinal crease incision was done and scarpa's
fascia opened. The external spermatic ring was identified and without opening the external
oblique fascia the cord was brought forth to the wound area. The hernia sac or processus
vaginalis was carefully dissected free from the cord structures and ligated high with silk 000.
No further dissection attempts were done if an obliterated processus vaginalis was identified.
All specimens were submitted for pathological exam. Scarpa's reaproximated with
polyglycolic acid 0000 suture and skin approximated with subcuticular chromic catgut 0000
suture.

RESULTS
There were 161 patients who underwent bilateral inguinal exploration and repair, 81 patients
came from HURRA and 80 from HSP. Males were 89 and females 72 for a 1.2:1 ratio.
Age at operation is shown inTable 1, showing that almost two-thirds (61%) were infants
younger than two years of age, generally the population of children referred to a pediatric
surgeon. In only 110 pts. of the study group we could retrieve the data on gestational age; 89
pts (81%) were at term and 21 (19%) were premature babies.
Table 2 displays the initial clinical mode of presentation of the patients, 69 pts presented with
a right inguinal hernia (RIH), 47 with a left inguinal hernia (LIH), and 45 pts with bilateral
inguinal hernias (BIH). Males and females were fairly distributed between the group.
Table 3 shows the associated conditions: 25% of our patients had past history of some kind of
airway disease process, most commonly bronchial asthma. All cases with undescended testis
were pexed concomitantly. None of the umbilical hernias underwent simultaneous repair. A
group of 25 patients (16%), suffered an episode of incarceration preop, all were successfully
reduced manually and repaired promptly, their mean age was 4.2 mo. No patient suffered from
strangulation or testicular edema. At that time 102 (63%) procedures were done as one day
surgery, and 59 (37%) as outpatient and the mean operating time was 20+/-8 minutes.
Operative findings during surgery were recorded as a hernia sac (HS), a patent processus
vaginalis (PPV), or an obliterated processus vaginalis (OPV). In that group of patients with an
initial diagnosis of BIH we found 85 (95%) hernias, 4 (4%) PPV and 1 (1%) OPV, they will not be
considered further. Those patients with a unilateral (RIH or LIH) hernia are shown inTable 4. A
positive finding (either a hernial sac or a patent processus vaginalis) was identified in 74% RIH
and 72% LIH patients when the contralateral side was explored. All hernias were of the indirect
type.
The postop complications are listed inTable 5, the most common were two patients with
residual scrotal hydrocele that resolved spontaneously six months after surgery. A premature
infant with a postconceptual age of 46 weeks had an episode of apnea in the immediate
postop period, requiring mechanical ventilation for one day. Another patient needed inhalation
therapy for a postintubation croup condition. We did not find testicular damage or hernia
recurrence after a mean follow-up of six years, neither a wound infection was recorded. There
was no mortality reported in the present study.
When the contralateral findings during surgery were compared and analyzed within the three
subgroups of patients (sex, gestational age and age at operation), we found that females had a
higher probability of having positive findings than males, as seen inTable 6. No difference was
obtained whether the patients had history of prematurity or not. Those infants younger than
two months also had the highest probability of having positive findings. We also obtained
statistic significance in patients above the two years old, probably the result of the higher
frequency of females over males in these subgroups of patients.
DISCUSSION
If routine contralateral exploration of the unilateral pediatric hernia is to prevail is because it
has a high yield of positive findings (HS and PPV), a low complications rate and can be
expeditiously accomplished (4).
From this study, there is a high percentage of positive contralateral operative findings (72% in
our series), and a very low incidence of significant morbidity following contralateral repair.
Our data favors a strong reasoning to justify routine contralateral exploration of infants and
children with unilateral hernia by pediatric surgeons. We agree with McGregor et al (5), that we
live in a litigious society and gonadal morbidity whether related to the hernia operation or not
can eventually result in litigation. There are still a large proportion of infant and child hernia
operations that are not performed by pediatric surgeon and perhaps we should highlight that
education, confidence, and informed consent is the hallmark of our recommendations. It
should be noted that the bilateral procedures took a mean time of 20 minutes. Time should not
be spent in tedious dissections of the cord structures so as to increase the yield of PPV
identified, this could certainly be a factor in the past experience of other surgeon with regards

to gonadal or vas deferens trauma. Nowadays most cases are done as outpatients
procedures.
Previous reports have shown a higher incidence of positive contralateral findings in young
infants, females, prematures, and when the presenting hernia is on the left side (6,7,8). Our
data confirms that young infants and females do have a higher yield of positive findings (92%
and 94% respectively). We could not demonstrate that prematurity or left-sided hernias were
associated with a higher positive rate (64% and 68% respectively) as substantiated by our
statistical analysis. The older children group (2-5 y/o and >6 y/o) with a higher positive yield
could be biased since females were represented most commonly. As other authors have
stated, the major benefit of contralateral exploration of the pediatric hernia is that it allows
discovery and elimination of a patent processus vaginalis so that an indirect inguinal hernia
cannot develop (2).
We conclude by establishing some criteria to justify routine contralateral exploration of the
pediatric hernia: the surgeon should be experienced in child surgical care, associated
conditions should not increase the surgical risks significantly, time-consuming dissections of
the cord structures should be discouraged and the operating time should be kept to a
minimum.
Acknowledgement
A special thanks to Professor Iris Parrilla of the Family Medicine Department, Universidad
Central del Caribe, School of Medicine for her assistant in the statistical analysis of the data.
REFERENCES
1. Sparkman RS: Bilateral exploration of inguinal hernia in juvenile patients, ,Surgery 51; 393,
1962
2. Rowe MI, Lloyd DA: Inguinal Hernia, In Welch KG, Randolph JG, Ravitch MM, O'neill JA,
Rowe MI, (eds): Pediatric Surgery. Chicago: Yearbook, l986, pp779-793
3. Rowe MI, Marchildson MB: Inguinal hernia and hydrocele in infants and children. Surg Clinic
North Amer 61: 1137, l981
4. Menton JP, Clatworthy HW: Incidence of patency of the processus vaginalis. Ohio State Med
J 53:530-532, l961
5. Mc Gregor DB, Halverson K, Mc Vay CB: The unilateral pediatric inguinal hernia: Should the
contralateral side be explored? J Ped Surg 15(3):313-317, l980
6. Gilbert M, Clatworthy HW: Bilateral operations for inguinal hernias and hydroceles in
infancy and childhood. Am J Surg 97:255, 1959
7. Bock JE, Sobye JW: Frequency of contralateral inguinal hernia in children.Acta Chir Scand
136:707, l970
8. Recorta FJ, Grosfeld JL: Inguinal hernia repair in the perinatal period and early infancy:
Clinical considerations J Ped Surg 19(6):832-837, l984

* Associate Professor in Pediatric Surgery, Department of Surgery, University

of Puerto Rico School of Medicine. Assistant Professor Surgery, Universidad
Central del Caribe School of Medicine. Chief- Section of Pediatric Surgery,
Department of Surgery, HURRA and Hospital San Pablo.
Address reprints to: Humberto L. Lugo-Vicente, MD, P.O. Box 10426, Caparra
Heights Station, San Juan PR 00922-0426. Tel. (809)-786-3495 Fax
(809)-720-6103 e-mail: titolugo@coqui.net
Published in Boletn Asociacin Mdica de Puerto Rico. Vol 87(1):8-11, 1995

>>>BACK TO INDEX<<<

TRICHOBEZOAR IN A 11-YEAR OLD GIRL:

A Case Report

Carlos A. Santiago-Snchez, MD*

Priscila Garau-Daz, MD**
Humberto L. Lugo-Vicente, MD, FACS, FAAP***

Bezoars are masses of solidified organic or non-biological material commonly

found in the stomach and small bowel. They have been known for centuries
and nowadays continues to be a challenging therapeutic dilemma for surgeons
and gastroenterologists alike.
The identification, therapy, and long-term management of patients with
bezoars depend on accurate classification and knowledge on the
pathophysiology of formation. Four types of bezoars have been described
based on their composition: phytobezoars, trichobezoars, lactobezoars, and
miscellaneous (1).
We describe a case of a gastric trichobezoar in a pediatric patient managed
successfully with surgery.
CASE HISTORY
An 11-year old white girl, 6th grade student, was admitted on September 4, 1994 to the
University Pediatric Hospital complaining of a sensation of fullness at the epigastrium, vague
feeling of epigastric distress, nausea and anorexia. One day before admission a plain
abdominal film done at the Local Health Center showed a large radiopaque image filling the
stomach and suggesting an intra-abdominal tumor. The patient was transferred to our
supra-tertiary institution for further evaluation and management. Computerized Abdominal
Tomography using oral and intravenous contrast material showed a large gastric bezoar
(seeFigure 1). Further questioning of the child revealed epigastric complains for months and
she confirmed "eating hair when nervous". The family and social history uncovered that her
mother was a psychiatry patient and the father an alcoholic with frequent domestic fights,
claiming the child responsible for the household crisis. Furthermore the mother menaced the
child by telling her "she was going to kill her". Psychiatry evaluation revealed a depressed,
frightened, neglected child that relieved her anxiety by eating her hair (trichophagia).
Physical examination revealed a skinny girl with pale conjunctiva. A large, firm, oval shaped,
non-tender and mobile mass was palpable at the left upper quadrant of the abdomen. The
mass extended from the distal margin of the left rib cage to approximately 2 cm above the
navel. On the right side the mass was palpable beyond the midline to the right nipple line.
There was no guarding, rigidity or tenderness. No alopecia was noted in the child. The rest of
the physical examination was essentially negative.
Laboratory work-up upon admission exhibited a mild hypochromic microcytic anemia
(hemoglobin 11.9 gm/dl, and hematocrit 35.6%). Normal coagulation profile, urinalysis,
electrolytes, amylase, lipase, and liver function tests. A plain chest film was normal.
The upper gastrointestinal series displayed a large intraluminal space occupying mass lesion
with a honeycomb appearance that filled the stomach contour with extension into the proximal
duodenum (seeFigure 2).
Upper endoscopy showed a normal esophageal mucosa. The stomach contained a very large,
black, hairy ball extending through the pylorus. The gastric mucosa appeared normal without
evidence of ulceration. A significant foul, nauseating smell was noted. Biopsy confirmed the
hair-nature of the bezoar.
Although fragmentation with Extracorporeal Shock Wave Lithotripsy was considered, the
huge size of the bezoar along with the proximal extension to the duodenum contraindicated its
use and no further attempt was done. The child was taken to the operating room and the
bezoar removed without difficulty using an anterior longitudinal gastrotomy incision. The
mass had the shape of the stomach and proximal part of the duodenum, a brilliant surface and
a putrefactive odor (seeFigure 3). The gastric mucosa was normal and not adhered to the
mass.
Oral feedings were resume on the 6th postoperative day. The child discharged home after
adequate psychiatry assessment and therapy.

DISCUSSION
The word 'bezoar', comes either from the Arabic word "bedzehr", or the Persian word
"padzhar", meaning protecting against a poison or an antidote (2,3). In ancient times the solid
mass occasionally found in the stomach of a goat or an antelope was thought to have magical
healing powers and even rejuvenating properties (4). Medicinal qualities and omens of good
luck were also attributed to bezoars (2). In modern medicine, however, the concretion found in
the stomach and intestine of humans and referred by the term bezoar is known to be
associated not with such positive effects, but with significant morbidity and even mortality (5).
In children four types have been described based on their composition:
1- phytobezoars composed mainly of vegetable or fruit fiber,
2- trichobezoars, comprise mainly of hair,
3- lactobezoars made of milk curd, and
4- miscellaneous (medicational or food bolus) bezoars (5,6).
Phytobezoars are the most common type of bezoars. They consist of vegetable material and
indigestible cellulose fiber (7). Persimmons seed and other fruit products are frequent
reported factors in their formation. Most develop in adults patients with impaired digestion
and previous gastric surgery causing dysmotility disorders such as post-gastrectomy cases
for peptic ulcer disease. Ailments other than gastric surgery that has been noted to cause
impaired gastric emptying includes: diabetic gastroparesis, myotonic dystrophy, and
autovagotomy secondary to tumor invasion (8). When associated with gastric surgery the
stomach exhibits a diminished ability to digest, produce acid, pepsin activity, and
mechanically reduce food (9).
The classically described bezoar, usually involving psychologically disturbed individuals is
the trichobezoar or "hair-ball" bezoar. The trichobezoar is a concretion of hair found in the
alimentary tract of animals, especially ruminants, and occasionally in man. Over the centuries
these bezoars have been associated with children and emotionally disturbed adult females
who ingest hair (trichophagia), carpet, rope, string, etc. The classic pediatric case is that of a
partially bald child with a mass in the stomach (3). Hair strand become retained and attached
in the folds of the gastric mucosa because the friction surface is insufficient for propulsion by
peristalsis (10).
Trichobezoar are seen almost exclusively in female children, 6-10 years old, with bizarre
appetite (trichophagia) and emotional disturbances (1). They may produce multiple clinical
manifestations such as: large firm movable epigastric mass, fullness, bloating, regurgitation,
nausea, vomiting, epigastric pain, hematemesis, and tiredness (2). Originally the mass
develops in the stomach and can move to the small bowel by fragmentation of a portion,
extension or total translocation (3). Many patients complain of early satiety, and weight loss.
Other children will reduce intake and develop failure to thrive. If untreated, chronic obstruction
may result in death from malnutrition or other complication such as ulceration, hemorrhage or
perforation. Symptoms are intermittent and absent for many years. Rapunzel syndrome is
ascribed to those gastric bezoars that have a tail-like extension of twisted hair reaching the
ileocecal valve (2).
Lactobezoars have been noted during the last two decades, corresponding to the period of
improved neonatal salvage. These bezoars are described in low birth weight neonates fed a
highly concentrated formula. Milk products like casein congeal forming the lactobezoar (11).
There is a miscellaneous group of bezoars consisting of medications glues, antiacids, and
food bolus. Food bolus that are incompletely chewed contain nuts and fiber or are trapped in
narrow gastric segments (12).
Bezoars are diagnosed in most cases by conventional radiological examination, i.e. plain
abdominal films, upper gastrointestinal series, ultrasonography, or computerized abdominal

tomography (13). When an upper gastrointestinal series is performed with the use of barium,
an intragastric mass with a honey-comb like surface around which the contrast medium flows
may readily be observed, as seen in our experience. Gastric endoscopy is one of the most
sensitive means to diagnosed bezoars, will confirm the diagnosis and determine their nature.
Also, is utilized to obtain biopsy specimen to confirm their composition (2,14).
Bezoars can be managed by various means, depending on their underlying nature and
location. Prior to 1959 the prevailing therapy for gastric or intestinal bezoars was surgical
excision. This carried a high morbidity and mortality. Emergency laparotomy may still be
necessary if the bezoar is associated with acute intestinal obstruction. Currently, non-surgical
techniques of management of gastric bezoars may include: dissolution, suction, lavage,
mechanical endoscopic fragmentation using pulsating jet of water, and fragmentation with
extracorporeal shock wave lithotripsy (ESWL) (15,16,17). With ESWL the shock wave needed
is half than required by urolithiasis cases (17). Intragastric administration of enzymes (papase,
pancrelipase, and cellulase) or drugs (metoclopramide, tagamet, bicarbonate, acetylcysteine)
has also been reported in the literature (18,19). If those methods fail, gastrotomy and manual
removal is the only means of reliving the patient. Large bezoars will generally need surgery for
removal (20).
Besides dissolution or removal, treatment should focus on prevention of recurrence, since
elimination of the mass will not alter the conditions contributing to bezoar formation.
Psychiatry follow-up may be necessary to reduce the frequency of recurrence.
In summary, the accepted therapies for patients with gastric bezoars include:1- observation, 2medical dissolution, 3- fragmentation, and 4- laparotomy with gastrostomy. The treatment
modality will depend on the type of bezoar involved. Treatment should not only focus on
resolution of the established mass, but also prevention of recurrence, since the underlying
condition contributing to bezoar formation will not be altered by elimination of the mass.
REFERENCES
1-Debakey M, Ochsner A: Bezoars and Concretions- A Comprehensive Review of the
Literature with an Analysis of 303 Collected Cases and a Presentation of 8 additional cases.
Surgery 4:934-963, 1938; 5:132-160, 1939
2-Deslypere JP, Praet M, Verdonk G: An Unusual Case of the Trichobezoar. The Rapunzel
Syndrome. Am J Gastroenterol 77(7):467-470, 1982
3-Sharma V, Sharma ID: Intestinal Trichobezoar with Perforation in a Child. J Pediatr Surg
27(4):518-519, 1992
4-Budd DC, Mc Crany ML: Gastric Phytobezoar: Still postgastrectomy syndrome Ann Surg
44(2):104-107, 1978
5-Andrus C, Ponsky J: Bezoars: Classification, Pathophysiology, and Treatment. Am J
Gastroenterol 83:476-478, 1988
6-Rao PLNG, Mitra SK, Pathak IC: Trichobezoars in Children. Int Surg 66:63-65, 1981
7-Stanten A, Peters HE: Enzymatic Dissolution of Phytobezoars. Am J Surg 130:259-261, 1975
8-Goldstein SS, Lewis TH, Roth Stein R: Intestinal Obstruction Due to Bezoars Am J
Gastroenterol 79:313-318, 1984
9-Kirks DR, Szemes GC: Autovagotomy and Gastric Bezoar. Gastroenterol 61:96-97, 1971
10-Reisfeld R, Dammert W, Simpson JS: Trichobezoar: An Uncommon Pediatric Problem. Can
J Surg 21(3):251-252, 1978
11-Usmani SS, Levenbrown J: Lactobezoar in a full-term breast-fed infant Am J Gatroenter
84(6):647-649, 1989
12-Henke TA, Stillworthy RE Bin Stadt DU et al: Medication Bezoar in 2 Neonates. Am J Diseas
Child 136:72-73, 1982
13-Newman B, Girdany BR: Gastric trichobezoars-Sonographic and Computed Tomographic
Appearance Ped Radiol 20(7):526-527, 1990
14-Dietrich ND, Gau FE: Postgastrectomy Bezoars; endoscopic Diagnosis and Treatment. Ann
Surg 170:432-435, 1985
15-McKechnie JC: Gastroscopic Removal of a Phytobezoar. Gatroenterol 62(5):1047-1050,
1972
16-Lange V: Gastric Phytobezoar: An Endoscopic Technique for Removal. Endoscopy
18:195-196, 1986
17-Benes T, Chmel J, Jodl J, et al: Treatment of a Gastric Bezoar by Extracorporeal Shock
Wave Lithotripsy. Endoscopy 23:346-348, 1991
18-Pollard HB, Block GE: Rapid Dissolution of Phytobezoar by Cellulase Enzyme. Am J

Surgery 116:933-935, 1968

19-Winkler WP, Saleh J: Metoclopramide in the Treatment of Gastric Bezoar. Am J
Gatroenterol 78(7):403-405, 1983
20-Robles R, Parrilla P, Escamilla C, et al: Gastrointestinal Bezoars. British J Surgery
81:1000-1001, 1994

* General Surgery Resident, Department of Surgery, University of Puerto Rico,

School of Medicine.
** Assistant Professor of Pediatrics, Section of Pediatric Gatroenterology,
University Pediatric Hospital, University of Puerto Rico, School of Medicine.
*** Associate Professor of Pediatric Surgery , Section of Pediatric Surgery,
University Pediatric Hospital, and Department of Surgery, University of Puerto
Rico, School of Medicine.
Address reprints to: Humberto L. Lugo-Vicente, MD, P.O. Box 10426, Caparra
Heights Station, San Juan PR 00922-0426. Tel. (809)-786-3495 Fax
(809)-720-6103 e-mail: titolugo@coqui.net

>>>BACK TO INDEX<<<

IMPACT OF MINIMALLY INVASIVE SURGERY

IN CHILDREN
Humberto L. Lugo-Vicente, MD, FACS, FAAP*
Pediatric Surgeon

ABSTRACT
An important medical technological progress of this century corresponds to the application of
minimal invasive surgical techniques in adults and children. Laparoscopic surgery is causing
an impact in the results of many procedures done during the pediatric age.
Within this review we explore the development of laparoscopic abdominal surgery in children
along with basic physiology and complications of establishing a potential working space
(pneumoperitoneum). Indications, results, and where we are headed in the management of
various of the most common surgical conditions of children are issues discussed.
Laparoscopic surgery has proven safe, efficient, technically feasible and well tolerated in most
children. Produces early return to activities, reduced hospital stay, less hospital bills, and
better cosmetic results when compared to open (conventional) procedures.
HISTORY
For almost 150 year's physician has struggle to develop techniques of minimal invasive
surgery. Unfortunately, the medium, optics and instrumentation of earlier times were archaic.
Development of the fiber optic transmission of light in 1928, the rod-shaped lens of Hopkins in
the early 60's and video improvement during the late 70's renew interest in accessing the body
cavities by minimally invasive technique using the laparoscope. Our fellow physicians, the
gynecologists dominated this field for ten years (1).
The revolution occurred in France in 1987, this time Province of Lyon, when the gallbladder of
a lady is removed successfully using laparoscopic technique. Since then, the rest has been
evolution (2).
PEDIATRIC LAPAROSCOPY
Pediatric laparoscopy grew slowly and lag behind. The reason is that children usually do well
and procedures are of short duration. The optics is of paramount importance when the
abdominal cavity is small, and instrumentation should be tailored to body size. We wanted to
see how general surgeons did before applying this technique in children. Credentialing
became very tedious and time consuming if we consider that two cholecystectomies are done
in children for every 100 performed by general surgeons in adults (3). Other Pediatric
surgeons thought of this as a Nintendo game or making a ship in a bottle.

The concept behind minimally invasive surgery is that the size of the wound has a direct
correlation with the metabolic and endocrine response to surgical trauma. The greater the
cutting of fascia, muscle and nerve the higher the catecholamine and catabolic response of
the body to surgical trauma.
A potential working space during video-laparoscopic abdominal procedures in children is
established with the help of a carbon dioxide pneumoperitoneum. The most popular technique
used in children for developing a pneumoperitoneum is the open (Hasson) technique, usually
in children less than two years of age (4). Closed or percutaneous (Veress needle) technique
is mostly practice in older children and adolescents (5, 6). Insufflation by either technique will
cause an increase in intrabdominal pressure (IAP). Studies during congenital abdominal wall
defects closure such as gastroschisis and omphalocele has shown that the rise in IAP may
cause decrease venous return, decrease renal perfusion, low splanchnic flow, and increased
airway pressures (7). In addition, abdominal distension causes pulmonary function
abnormalities such as decreased functional residual capacity, basilar alveolar collapse, and
intrapulmonary shunting of deoxygenated blood. The cardiac afterload will increase, an effect
that may be magnified by hypovolemia.
Hypotension during the establishment of the pneumoperitoneum is a very feared complication.
It could be the result of vascular injury, arrhythmia, insufflating too much carbon dioxide,
impending heart failure, gas embolism or the development of a pneumothorax (8, 9). We
generally insufflate a three-kilogram baby with ten millimeters of mercury of intra-abdominal
pressure and a 70-kilogram child with a maximum of fifteen mm of Hg as can be appreciated
in Graph 1.
Increase awareness of the intrinsic effects carbon dioxide insufflation may cause in the child
abdominal cavity is necessary. Carbon dioxide is absorbed by the diaphragmatic surfaces and
cause hypercapnia, respiratory acidosis, and pooling of blood in vessels with decrease
cardiac output. This effect is usually controlled by the anesthesiologist increasing minute
ventilation by 10% to 20% to maintain normocapnia. Increase dead space or decrease
functional residual capacity caused by the Tredelenberg position and administration of volatile
anesthetic agents can increment this problem. High risk children where this effect can be
potentiate further are those with pre-existent cardio-respiratory conditions causing increase
dead space, decrease pulmonary compliance and increase pulmonary artery pressure and
resistance. It is estimated that carbon dioxide accumulates primarily in blood and alveoli due
to the decrease muscular components to buffer the excess absorbed gas present in children
(10). After the procedure, the combination of residual carbon dioxide in the diaphragmatic
surface and water forms carbonic acid that upon absorbtion by the lymphatics produces
referred shoulder pain. There is always a small risk of ventricular dysrhythmia with insufflation
of carbon dioxide in children (3, 11, 12).
Some contraindications for performing laparoscopy during the pediatric age are: history of
severe cardio-pulmonary conditions, uncorrectable coagulopathy, prematurity, distended
abdomen with air or ascites, and multiple abdominal scars from previous operative
procedures (12).
We have already gone through Four Congress of Endosurgery in Children, and what has been
the impact? The indications from either diagnostic or therapeutic laparoscopy has grown fairly
as can be gathered from Table 1.
I have managed to gather the results of some of the most common laparoscopic procedures
done in children and will discuss them. These are: cholecystectomy, appendectomy, groin
laparoscopy, in pursuit of the non-palpable undescended testis, splenectomy, and
fundoplication.
RESULTS
Laparoscopic Cholecystectomy
Laparoscopic Cholecystectomy (LC) has become the procedure of choice for the removal of
the disease gallbladder of children. The benefit of this procedure is obvious: safe, effective,
and well tolerated. It produces a short hospital stay, early return to activity and reduced
hospital bills (3). Several technical differences between the pediatric and adult patient are:

lower intrabdominal insufflation pressure, smaller trocar size and more lateral position of
placement. Complications are related to the initial trocar entrance as vascular and bowel
injury, and those related to the procedure itself, i.e., bile duct injury or leak. Three 5 mm ports
and one 10-mm umbilical port are used. Pneumoperitoneum is obtained with Veress needle
insufflation or using direct insertion of blunt trocar and cannula. Cholangiography before any
dissection of the triangle of Calot using a Kumar clamp is advised by some workers to avoid
iatrogenic common bile duct (CBD) injuries during dissection due to anomalous anatomy, and
the best method to detect CBD stones (13).
Treatment of CBD stones may consist of:
1- endoscopic sphincterotomy followed by LC,
2- open (conventional) or laparoscopic choledochotomy, or
3- transcystic choledochoscopy and stone extraction.
Children with hemolytic disorders, i.e., Sickle cell disease, have a high incidence of
cholelithiasis and benefit from LC with a shorter length of postop stay and reduced morbidity
(3).
From April 1992 to 1995 Avils, Mas & Lugo managed to do 40 cholecystectomies at the
University Pediatric Hospital. Twenty-four were done laparoscopically with one conversion
and 16 open as can be seen in Table 2 (14).
San Pablo Medical Center performed 4439 cholecystectomies from January 1990 to July 1995;
83 (1.8%) of them in children (Table 3).
Both series stress the issue that LC is superior to the open conventional procedure reducing
the operating time, length of stay, diet resumption, and use of pain medication. The child is
more pleased with his cosmetic results and activities are more promptly established. We also
found that CBD stones can be managed safely with simultaneous endoscopic papillotomy and
costs of LC are further reduced employing re-usable equipment and selective
cholangiographic indications (3).

Laparoscopic Appendectomy
Semm, a gynecologist, is credited with inventing laparoscopic appendectomy in 1982. With the
arrival of video-endoscopic procedures the role of laparoscopic appendectomy in the
management of acute appendicitis in children has been studied and compared with the
conventional open appendectomy. General advantages of laparoscopic appendectomy
identified are: ease and rapid localization of the appendix, ability to explore and lavage the
entire abdominal cavity, decrease incidence of wound infection, less cutaneous scarring, more
pleasing cosmetically, and a rapid return of intestinal function and full activity. There is
certainly some advantage in doing laparoscopic appendectomy in the obese child, teenage
female with unclear etiology of symptoms, for athletes, children with chronic right lower
quadrant abdominal pain, and cases requiring interval appendectomy (15). Disadvantages are:
expensive instrumentation, time-consuming and tedious credentialing, and the major benefit
is in the postop period.
Analyzing the results of several series that compare laparoscopic vs. conventional
appendectomy in the management of acute appendicitis we can conclude that laparoscopy
produces no difference with open appendectomy in respect to operating room complications
and postoperative morbidity, has a longer operating and anesthesia time, higher hospital

costs, a shorter length of stay, less postop pain, less pain medication requirement, and
shorter convalescence. One series warned that complicated cases of appendicitis done by
laparoscopy could increase the postoperative infectious rate requiring readmission.
Otherwise, they all favored laparoscopic appendectomy in the management of appendicitis
(15-19).
Still, unresolved issues in my mind are: Does laparoscopic appendectomy reduce
postoperative adhesions? , Is it necessary to remove a normal looking appendix during a
negative diagnostic laparoscopy performed for acute abdominal pain? , Will the increase
intrabdominal pressure alter the diaphragmatic lymphatic translocation of bacteria favoring
higher septic rates in complicated cases? Experimental evidence in animal models favors
higher rates of systemic sepsis after sequential development of pneumoperitoneum (20).
Groin Laparoscopy
The issue of contralateral exploration in the pediatric inguinal hernia patient has been hotly
debated. Proponents of routine contralateral exploration cite the high percentage of
contralateral hernia a/o potential hernia found at exploration, the avoidance of the cost of
another hospitalization, psychological trauma and anxiety to the child and parents over a
second operation, and the added risk of anesthesia of a second procedure. Most pediatrics
surgeons habitually explore the contralateral side. They disagree in opinions about
exploration depending upon the primary site of inguinal hernia, age, sex and the use of
herniography or some intra-operative technique to check the contralateral side (21).
Recently the use of groin laparoscopy permits visualization of the contralateral side. The
technique consists of opening the hernial sac, introducing a 5.5-mm reusable port,
establishing a pneumoperitoneum, and viewing with an angle laparoscope the contralateral
internal inguinal ring to decide the existence of a hernia, which is repaired if present. Requires
no additional incision, avoids risk of vas deferens injury in boys, is rapid, safe and reliable for
evaluating the opposite groin in the pediatric patient with unilateralinguinal hernia. Children
less than two years of age have a higher yield of positive contralateral findings (12,22,23).
Diagnostic Laparoscopy for the Non-palpable Undescended Testis
The undescended testis identified in 0.28% of males can be palpable (80%) or non-palpable
(20%). It is difficult to determine either location or absence of the non-palpable undescended
testis by clinical examination. Imaging studies (Ultrasound, CT Scan, Magnetic resonance,
gonadal venography) are not reliable in proving its absence. Diagnostic laparoscopy is reliable
in finding the non-palpable undescended testis or proving its absence. Furthermore it can be
combine to provide surgical management. After reviewing several series (12, 24-36), with
non-palpable undescended testes managed by laparoscopy the following three findings were
identified:
1- The testis is present; in either an intra-abdominal (38%) or inguinal position (12%).
Intrabdominal testes can be managed by first stage laparoscopic internal spermatic vessel
clipping and cutting (Stephen-Fowler's), followed by second stage vas-based standard
orchiopexy six to nine months later. Inguinal testes are managed by standard inguinal
orchiopexy.
2- The testis is absent (vanishing testicular syndrome) as proven by blind ending vas and
testicular vessels (36%). These children are spare an exploration. If the vas and vessels exit
the internal ring, inguinal exploration is indicated to remove any testicular remnant as
histologic evidence, although I have found useful removing the testicular remnant by the
laparoscopic approach. The presence of a patent processus vaginalis may suggest a distal
viable testis.
3- The testis is hypoplastic, atretic, or atrophic (26%), in which case is removed
laparoscopically.
Exact anatomical localization of the testis by laparoscopy simplifies accurate planning of
operative repair; therefore, is an effective and safe adjunct in the management of the
cryptorchid testis.

Laparoscopic Splenectomy
Laparoscopic splenectomy is another safe and technically feasible video-endoscopic
procedures in children. Indications are usually hematological disorders such as Idiopathic
thrombocytopenic purpura, spherocytosis, and Hodgkin's staging. Technical considerations
of the procedure are based on anatomical facts such as the variability in the splenic blood
supply, the ligaments anchoring the organ and the size of the diseased spleen. Generally the
avascular splenophrenic and colic ligaments are cauterized, the short gastric and hilar vessels
are individually ligated with metallic clips or gastrointestinal staplers, and the spleen is placed
in a plastic bag, fracture or morzelized until it is removed through the navel.
Comparing the laparoscopic procedure with the conventional splenectomy, the advantages
are: improved exposure, decreased pain, improved pulmonary function, shortened
hospitalization, more rapid return to normal activities and excellent cosmetic appearance.
Disadvantages are longer operating time, higher costs and the need to open 5-20% of cases
due to technical uncontrolled hemorrhage, such as bleeding from the splenic artery (37, 38).
Laparoscopic Fundoplication
Fundoplication for the management of symptomatic gastroesophageal reflux (GER) is another
procedure that has evolved recently taking advantage of minimally invasive technique.
Indications for performing either the open or laparoscopic fundoplication is the same, namely:
life threatening GER (asthma, cyanotic spells), chronic aspiration syndromes, chronic
vomiting with failure to thrive, and reflux induced esophageal stricture. Studies comparing the
open versus the laparoscopic technique in the pediatric age have found a reduced mean
hospital and postoperative stay with laparoscopy.
The lap procedure seems similar to the open regarding efficacy and complication rates. Costs
are not excessive, they are even lower if we take into consideration the shorter length of stay.
Lower rate of adhesions, pulmonary and wound complications are another benefit of the lap
technique suggested. Percutaneous laparoscopic gastrostomy can be done concomitantly for
those neurologically impeded children refer with feeding problems and GER (39-43).
Whether to do a complete (Nissen) or partial (Toupee, Thal, or Boix-Ochoa) wrap relies on the
experience of the surgeon with the open procedure. He should continue to do whatever
procedure he used to perform using open surgery. Long-terms results of complications or
recurrence of GER after laparoscopic fundoplication are still pending publication.
CONCLUSIONS
Video-Laparoscopic procedures are safe and efficient, technically feasible and well tolerated
by children. Opening a child is not a complication. The future of pediatric laparoscopy may
involve the use of intrauterine therapeutic fetoscopy.
REFERENCES
1- Marlow J: History of Laparoscopy, Optics, Fiberoptics, and Instrumentation. Clin Obstet
Gynecol 19: 261-275, 1976
2- Ko ST, Airan MC: Review of 300 consecutive laparoscopic cholecystectomies: development,
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14- Unpublished results.
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of 1,379 cases. J Pediatr Surg 1994 Jun;29(6):786-9
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20- Bloechle C. Emmermann A, Treutl, et al: Effect of peritonitis induced by gastric ulcer
perforation in the rat. Surg Endosc 9(8): 898-901, 1995
21- Lugo-Vicente HL: The Pediatric Inguinal Hernia: Is Contralateral Exploration Justified?
Boletin Asoc Med PR 87(1):8-11, 1995
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a contralateral patent procesus vaginalis. Pediatric Surgery 8: 385-388, 1993
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Nonpalpable Testis. Pediatric Annals 22: 678-684, 1993
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Sep;93 Suppl 2:S103-8
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therapeutic trends. Pediatr Med Chir 1994 Nov-Dec;16(6):513-6
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vaginalis. J Urol 1994 Aug;152(2 Pt 2):776-8

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treatment of non-palpable testicular cryptorchism. Prog Urol 1994 Apr;4(2):206-13
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Mar;73(3):310-3
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role of laparoscopy. Urology 1994 Feb;43(2):222-7
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prospective assessment of accuracy. J Urol 1994 Mar;151(3):728-31
32- Holcomb GW 3rd, Brock JW 3rd, Neblett WW 3rd, et al: Laparoscopy for the nonpalpable
testis. Am Surg 1994 Feb;60(2):143-7
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(1988-90). Aust N Z J Surg 1993 Jun;63(6):451-3
34- Rappe BJ, Zandberg AR, De Vries JD, et al: The value of laparoscopy in the management of
the impalpable cryptorchid testis. Eur Urol 1992;21(2):164-7
35-Diamond DA, Caldamone AA:The value of laparoscopy for 106 impalpable testes relative to
clinical presentation. J Urol 1992 Aug;148(2 Pt 2):632-4
36- Heiss KF, Shandling B: Laparoscopy for the impalpable testes: experience with 53 testes.
J Pediatr Surg 1992 Feb;27(2):175-8; discussion 179
37- Janu PG, Rogers DA, Lobe TE: A Comparison of Laparoscopic and Traditional Open
Splenectomy in Childhood. J Pediatr Surg 31(1): 109-114, 1996
38- Gigot JF, de Goyet JDV, Van Beers BE, et al: Laparoscopic splenectomy in adults and
children: Experience with 31 patients. Surgery 119(4): 384-389, 1996
39- Lobe TE, Schropp KP, Lunsford K: Laparoscopic Nissen Fundoplication in Childhood. J
Pediatr Surg 28(3): 358-361, 1993
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Gastrostomy and Fundoplication in 120 Patients. J Pediatr Surg 30: 1065-1071, 1995
41- Collard JM, de Gheldere CA, Kock MD, et al: Laparoscopic Antireflux Surgery: What is real
progress? Ann Surg 220(2): 146-154, 1994
42- Weerts JM, Dallemagne B, Hamoir E, et al: Laparoscopic Nissen Fundoplication: detailed
analysis of 132 patients. Surg Laparosc Endosc 3(5): 359-364, 1993
43- Lobe TE: Laparoscopic Nissen fundoplication in paediatric patient. Gaslini 27(1): 73-79,
1995

* Associate Professor of Pediatric Surgery , Section of Pediatric Surgery,

University Pediatric Hospital, and Department of Surgery, University of Puerto
Rico, School of Medicine.
Address reprints to: Humberto L. Lugo-Vicente, MD, P.O. Box 10426, Caparra
Heights Station, San Juan PR 00922-0426. Tel. (809)-786-3495 Fax
(809)-720-6103 e-mail: titolugo@coqui.net
This paper was presented at the 46th Meeting of the American College of
Surgeon, Puerto Rico Chapter, Hotel Marriot, Condado, Puerto Rico, February
1996.

DEDICATION
To those great masters that taught me minimally invasive surgical techniques:
Thom Lobe, Keith Georgeson, Douglas Olsen, Zoltan Czabo, Manuel Daz-Vargas, and Manuel
Ms

>>>BACK TO INDEX<<<

ROLE OF INTERNET IN PEDIATRIC

SURGERY
by : Humberto L. Lugo-Vicente, MD, FACS, FAAP *
San Juan, Puerto Rico

Abstract
Internet, the largest network of connected computers, is becoming the ultimate frontier to
access information for health providers. This review focus on how developments of this
communication technology have become a useful educational resource in Pediatric
Surgery, and describes modest ideas in computer network use.
Internet basic resources are electronic mailing (E-mail), discussion groups, file transfer,
and browsing the World Wide Web (WWW). E-mail brings physicians with common
interest together. Surgeons employ it as a communicating tool. Legal and social
responsibility is bounded with its use. Discussion groups permits debate including
clinical cases, operations, techniques, research, career opportunities, and meetings. File
transfer provides the opportunity of retrieving archives from public libraries. The WWW is
the most resourceful tool due to its friendly interface and ease of navigation.
The average physician needs to know almost nothing on how computers work or where
they came from to navigate through this pandemonium of information. Click and play with
today graphical applications encourage the computer illiterate to connect. Establishing
the connections envelops the need of hardware, software and a service provider.
Future development consists of online journals with new ideas in peer-review and
authentication, telemedicine progression, international chatting, and centralization of
pediatric surgery cyber space information into database or keyword search engines.
INDEX WORDS: pediatrics, surgery, internet

Introduction
Internet is the largest network of connected computers. More than 30 million computers
exchanging physical links through a standard protocol of communication. A super avenue
of information and transactions (1). The Net is affecting every aspect of life and
dissemination of information relevant to medicine for the health community is not immune
to this technology.

The busy surgeon who invests little time searching the literature could find himself with a
clinical practice that does not keep pace with recent medical advances. Informatics option
to stay updated in the discipline of Pediatric Surgery includes access to printed periodical
publications, regular meetings, congress assistance, digital database storage, and Internet
resources.
Text, journals, and books are usually outdated by the time they reach the regular subscriber.
Not to mention cost of subscription, printing and storage capabilities needed. Meeting and
congress dynamic regular sessions can be costly, and access to the full written report is
almost never achieved until print publication of the paper is obtained usually six months to
one year later. Digital databases (i.e., CD-ROM) store large amount of information, but prices
of CD are overwhelming. An additional driver is needed as hardware for reading the stored
material. Information is becoming an unlimited commodity, we can have as much as we
want at no cost, but are limited by our storage capacity (2).
By agreeing to a set of operating protocols, users have developed innovative techniques to
seek out information from different databases accessible via the network along with
methods for sharing documents. Internet provides immediate downloadable information and
dynamic information on every aspect of life. Still the idea that it represents a frustrating
educational event in computing persists. The average person needs to know almost nothing
on how computers work or where they came from to navigate through this network. Click
and play with today graphical applications encourage the computer illiterate to connect.
The purpose of this review is to highlight how newly ways of communication using Internet
navigational technology can be useful educational resources in Pediatric Surgery, and
clarify concepts of network communication for future use by physicians.

History of Internet
After the postwar years military intelligence was searching for strategic forms of
communication in the after-match of a nuclear holocaust, a system that would defeat current
centralized tendencies in communication. The notion of creating several nodes of super
computers that convey each other through standard telephones line was developed.
Sending the information in small packages that would meet at the other end of the line using
a uniform protocol of communication and regrouping (TCP/IP). These nodes would be
created around different parts of the world divided in either top level geographical or
institutional domains like: government (gov), commercial (com), educational, (edu), military
(mil), network resources (net), and other organizations (org).
Scientists were the first to use this system in an effort to consolidate research and establish
electronic communication in the flow of new projects. This created an atmosphere of social
behavior and effective long distance communication as more nodes grew in each country.
Curiously, the initial electronic discussion group developed among scientists was called the
Science-Fiction list (3, 4).
World Wide Web (WWW), the crowning glory of the Internet, is developed in Geneva,
Switzerland in 1989. The WWW provides a user friendly interface with the capacity to send
and receive information through Internet using text, graphics, audio and video utilizing a
protocol of marked language (5). Seen today as the best resource to post information that
can reach and be accessed in almost every corner of the planet.

Uses of Internet for the Pediatric Surgery community

Internet basic resources are:
electronic mailing (E-mailing),
discussion groups (news groups and list servers),
file transfer,
and the WWW.
Through the initial effort of scientists to establish communication using electronic text
mailing convenience over the postal service was foreseen. E-mailing is faster than postal
mail. It takes an average of two to eight minutes for messages to arrive to another computer

node in very distant geographical zones. The message is stored by the internet service
provider (ISP) until the electronic box owner retrieves the message. You do not have to pay
extra for e-mailing, and is global in scope. Files can be attached to messages up to half a
megabyte in size (a megabyte represents one million characters).
News groups and list servers with discussion interest have developed both in pediatrics and
surgery. Messages posted by authors to the list or discussion group are automatically
mailed to all subscribers. Posting growth to such lists includes United States, Central and
South America, Europe, Middle East, Africa, and Australasia to mention a few. List servers
for different surgery and pediatric sub-specialties exist: NICU-Net, PICU-Net, cardiology,
gastroenterology, neurology, emergency medicine, critical care, Pediatric pain, etc. (6, 7).
A popular list among Pediatric Surgeons worldwide is called the Pediatric Surgery List.
Originally developed by Thomas Whalen for topics discussion that includes clinical cases,
operations, techniques, research, career opportunities, and meetings. Intended for pediatric
surgeons and interested general surgeons and residents (8). Although the list is in
embryological phase, growth will inevitably create a medium of international discussion
without precedent. A constant forum for exchange of ideas, difficult cases, consensus on
management, and development of our specialty.
File transfer provides the unique opportunity of retrieving archives from public file libraries.
Free software is also available. Downloading of data into the hard disk of your computer is
very straightforward. Anti-viral programs are available to monitor each access file that can
become part of your system whenever you download them from Internet.
Recent poll of the Pediatric Surgery Internet list server members regarding what resource of
the Net they use most of the time was done. Almost one-fourth (23%) of the list population
(58/246) answered the survey. Electronic mailing (personal and list server/discussion
groups) occupied 83% of resources, web browsing 16%, and long distance computing 1%.
Pediatric surgeons with access to the Net use it mostly as a communication tool. WWW
browsing is slowly developing as a second alternative probably due to absent access to a
web browser connection.

E-mailing uses and responsibility

Electronic mailing is the most useful resource of Internet. Mailing lists bring people with
common interest together (9). Through it physicians have developed news, chat, and list
group discussion. This creates the perfect environment to consult colleagues on a clinical
problem, send draft of a paper for peer revision, read journals without paying subscription
rates, maintain your continuing medical education credits, and retrieve anything the same
day that it is published (1). It will become an essential tool in medical research, teaching
medical students, clinical practice, postgraduate studies, and continuing medical education.
The lack of a traditional peer-review process and author identification might prevent E-mail
text from being taken as authoritative (10).
The common user of the Net is a professional. Environmental motivations have created an
informal code of conduct known as net-etiquette. By this is meant politeness in replying.
Along with accessibility, identification and social responsibility (11).
Netters (defined as common user of the Net), resent several iatrogenic web disorders: not
waste the carrying capacity of the Net (bandwidth), posting unsolicited advertising
(spamming), and observing inappropriate online behavior (1). Chain E-mail letters can
overcrowd your electronic site. Other problems related to the nature of e-mailing that we
must be aware are: sign your posting so that we can know who is writing, do not reply
publicly to the whole group when answering privately to one person, and avoid including the
entire text of the original message in your reply.
A hot debate among frequent E-mail list servers involves being careful when answering or
replying, specially when the answer will hit many members of a list server group. The
inclusion of your name and address at the end of your E-mail text represents a legal
signature for all aspect of the law: the author name type in ASCII characters (10). Simple
rules to observe are: avoid using patients' names, address, record numbers or institutional
demographics. When personally responding to electronic medical consultation by an

unknown online patient ask yourself: Is he your patient behind the monitor? Have you
examined him or review his past medical record? Will my answer be used as possible legal
evidence in case this is unintended? The potential for abuse while looking at this
information will always exist.
Disclaimers notice stating the medico-legal responsibility behind frequent response to
complex medical problems are being asked for to list server administrators (12). This if a
response from some member commentator triggers a change in diagnosis or therapy in a
given discussion case that causes ultimate damage to the patient involved. The
commentator cannot be held responsible of his answer in as much as he had no clear
physician-patient relationship, was not paid for this service, or had the opportunity to
examine the patient or his medical charts. The disclaimer should include that particular
consultation or advice was not the idea of the answer, reliance on this comments should not
be done, and printed versions of the E-mail should not appear in any patient medical record
(12).

WWW and Pediatric Surgery

WWW can be seen as an endless book, where each page has a divergent story. The web
page is the basic unit of information and hypertext provides links to other pages in the same
directory, or in different domain sites of the network (5). What makes the WWW the most
resourceful area of Internet is the ability to watch text, images, video, audio, and real time
pictures embedded in web pages.
Each page has a unique address, also known as uniform resource locator (URL). URL
essential ingredients are protocol, domain name, and directory. For example the URL of
Pediatric Surgery Update' is: http://home.coqui.net/titolugo/index.htm. This means that the
protocol is http, the domain name /home.coqui.net/, and the file "index.htm" is the web index
page under "/titolugo/" directory.
Pediatric Surgery Update', the periodical electronic newsletter started on July 1993 as a
print form. Initially covered short issues and reviews in the discipline of pediatric surgery.
December 1995 marked its development as a web site. The WWW introduction of the print
version permitted development of further areas such as: review articles with images, graphs
and tables, survey section, technical innovation area, a Pediatric Surgery Online Handbook
for residents and medical students, and an area for medical students to developed research
and writing skills (13).
Departments and Section of Pediatric Surgery have developed their own web page in the
WWW. Through them we have access to such content as: faculty members, facilities,
research programs, interests, residency and fellowship programs, and other pediatric and
medically relevant links. Continuous medical education credits are part of some web site
offering.

Page in the Net, HTML and graphic applications

A web page, the basic unit of the WWW consists of all one medium, such as text, or can
include multiple media including graphics, sounds, animation, and video (14). The web page
is built using a mark language that is plain text tagged with handles <text>. This is known as
hypertext mark-up language (HTML). HTML can easily be learned for later production of a
web page. The National Center of Supercomputing Application (NCSA) Beginner's Guide to
HTML is used by many to start to understand the hypertext markup language used on the
WWW. It is an introduction and does not pretend to offer instructions on every aspect of
HTML (15). Computer applications developed as web editors provide the functionality
needed to construct a web page with little knowledge of HTML. They are called WYSISYG
(What You See Is What You Get) Editors.
Movement between web pages is accomplished by links to other universal resource locators
or Internet address. Links can be in different color or underlined text where the cursor of
your pointer device changes as though sensing an executable movement. By either clicking
the device or hitting the return key, you will be moving to that link. Some links are just
libraries composed of downloadable files.

Web editors can be downloaded from different suppliers in the Net. Some are free but most
can be obtained as shareware to try them for a limited period. For a list of HTML editing
tools or programs available the reader is referred to URL:
http://sdg.ncsa.uiuc.edu/~mag/work/HTMLEditors/windowslist.html

Establish the Connection

To gain access to Internet you will need hardware, software, and a service provider.
Hardware is your computer. This includes a monitor, central processing until (CPU) and
keyboard. Macintosh and Widows operating systems ease of use graphical environments
have prevailed during the last years over the more text-based disk operating system (DOS).
A modem is another piece of hardware needed that will provide the telephone line
communication.
Computer software that help you navigate the web is known as web browser. Web browsers
are in essence a navigational aid for moving around and between the various nodes and
links of the WWW (14). Some web browsers are non-graphic like Lynx, and graphical like:
Mosaic, Netscape, and MS Internet Explorer. Netscape is the most widely used and industry
standard full-features web browser. Latest versions of this software can be downloaded free
from their respective site (URL) in the web (16,17).
Internet service providers (ISP) are either private or universities based. The service provider
will give you access to the Net using a local or toll-free telephone number. Some may
include web space with the monthly rate offer. A university-based ISP usually provides
service for a nominal or none rate. Electronic addresses of such users usually end in the
suffix -edu. Most physicians with Internet access have it through academic affiliation (9)
Once connected, the Net is a pandemonium of information with no central index. The user
will rely on automated index or search engines. Search engines collect database, retrieve
programs, or harvest them (2). A collection of search engines can be found at URL:
http://www.webcom.com/webcom/power/index.html (The WebCom Power Index). Specific
search engines in the field of medicine will help create an atmosphere of librarian resource.
Editors like Spooner's Ped-Info and Lehmann's Points of Pediatric Interest, have developed
web sites with collection of information specifically oriented toward pediatric content. The
web site has been maintained as a set of WWW pages through which you can link to:
Departments of Pediatrics, professional organizations, pediatric practices, Children
Hospitals, medical and surgical subspecialties, on-line publications, and pediatric software
of interest. Criteria for entry into the database are that the resources must have appeal to
pediatricians, and specific pediatric content. Both web sites allow easy access to pediatric
information on the WWW for health care professionals and parents (18, 19).

Future of the Net in Pediatric Surgery

The future of Internet will be an unbounded multimedia circus. Real time video and audio
technology will permit us view recorded in vivo sessions made in another location paid for
through a local phone call. In vivo videos of laparoscopic procedures have already taken
place between two continents (United States and Argentina). With the use of a personal
computer and a modem, they have transmitted the surgical procedure through live
broadcast, via the Net, for the nominal price of a local phone call. Resident surgeons
watched and interacted during the surgical procedure. This opens an area by which
academic medicine can be telecasted to other parts of the world with least bearing on
economical resources (20).
Since anyone can publish in the Net online, electronic journals will develop with new
peer-review concepts. Editors, reviewers, and authors will need to adjust to the use of this
information technology. Online publication in Pediatric Surgery will increase as printed form
of actual journal joins the cyberspace domain. Less paper work on publishing companies
may mean a reduction in subscription price, with e-mailing guarantees of providing
manuscript of written and published articles.

Cyber citations as proposed by the American Psychological Association or Modern

Language Association have yet to be standardized by the American Medical Association to
be used as bibliographical style (21). Authors that use Online references will need to keep
printed or digital file copy of such articles, since there is no way to avoid drastic changes or
movement done to this domain address (22).
International chatting is another area of future development for our pediatric surgery
community. Using simple downloadable application like mIRC (internet relay chat) you can
connect to an undernet organization channel and chat with groups of people at the same
time (23). The International Pediatric Chat channel developed by J. Edlavitch use two weekly
sessions to maintain the group online (24).
Telemedicine refers to the use of telecommunication technology to simplify health care
delivery or distribute medical informatics. Some specific projects represented by this
technologic are: Multi campuses linking of hospitals and research centers, linkages
between rural health clinics and central hospital, physician-to-hospital links for transfer of
patient information, diagnostic consultations, patient scheduling, research, literature
searches, video program distribution for public education on health care issues, use of
video and satellite relay to train health care professionals in widely distributed or remote
clinical settings, and transfer of diagnostic information such as electrocardiograms or
X-rays. Some benefits are improved access to areas in needs of health service, reduce cost
of traveling, reduces professional isolation, and improving the quality of care given.
Development of the infrastructure needed along with cost containment issues are two of the
problems faced by this technological advance (25).
Most Pediatric Surgery Organizations (Surgical section AAP, APSA, CAPS, BAPS, etc.) will
find themselves generating web sites of their own during the next few years. This will add to
the pandemonium of information already established. A future trend in development will be
the need to gather all this information in a Pediatric Surgery Cyber Web site with database
keyword access. This way a centralized path will exist to organize the varied information
buried in the Net.
We must be aware of the negative effects of expansion of computerized information. The
WWW can be an intoxicating and seductive place. Long hours glued to the small screen,
surfing the cyberspace, and reading E-mail can cause social degradation, increasing
disparity and isolation of the individual. Fragmentation of knowledge can be the result.
Users must continue to maintain an equilibrium to avoid such side-effects (26,27).

Conclusions
The exponential growth of Internet in the disciplines of Pediatric Surgery will cause a
change in patient care, teaching and research. Changes in our specialty will be nurtured
through the international use of information posted in the Net. Main use by contemporary
pediatric surgeons is as a communicating tool using electronic mailing with WWW browsing
slowly growing.
Future developments consist of online journals with new concepts in peer-review and
authentication, telemedicine, international chatting, and centralization of cyber space
information into database or keyword search engines. Marketing is another frontier in the
development of medical informatics technology.

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24- Edlavitch Julius (September 1996) Home Page of International Pediatric Chat [Online]

Available: http://www.pedschat.org/ [September 19, 1996]

25- William Frederick, Moore Mary (August 1995)Telemedicine: Its Place on the Information
Highway [Online] Available: http://naftalab.bus.utexas.edu/nafta-7/telepap.html [September
7, 1996]
26- Thomas P. Copley (September 17, 1996) Make the Link Workshop: Tutorial Number Two
[Online] Available: http://www.crl.com/~gorgon/links.html [September 20, 1996]
27- Gravanis MB: Computers and social isolation [Letter to the editor] The Pharos 59 (3): 50,
1996
____________________________
* Associate Professor in Pediatric Surgery, University of Puerto Rico School of Medicine.
Member of SCOT (Section of Computers and Other Technologies) Section of the American
Academy of Pediatrics.
Address reprints request to: Humberto Lugo-Vicente, MD- P.O. Box 10426, Caparra Heights
Station, San Juan, Puerto Rico 00922-0426 Tel. 787 / 786-3495 Fax 787 / 720-6103 E-mail:
titolugo@coqui.net
Date posted: March 1, 1997

>>>BACK TO INDEX<<<

Delayed Diagnosis and Management

of Bronchial Rupture following Blunt Thoracic Trauma
Carlos E. Prieto-Velhote, Manoel C. Prieto-Velhote, Tais F.de Oliveira-Velhote
From the Division of Pediatric Surgery, Hospital Carlos Chagas, So Paulo, Brazil.
Address reprints request to: Carlos E. Prieto-Velhote, M.D. - Rua Carlos Weber 1389 - Apto.114
- CEP 05303-000 So Paulo Brazil. E-mail: cevelhote@mandic.com.br Fax:
55-021-11-64402706
ABSTRACT
The authors discuss delayed diagnosis of bronchial rupture following blunt thoracic
trauma. They report two patients suffering lung atelectasis forty days and two years after blunt
thoracic trauma respectively. Bronchoscopic examination was crucial in diagnosis. Both
cases managed with bronchial anastomosis doing well eighteen months and four years after
surgery.
KEY WORDS: thoracic trauma, blunt thoracic trauma, bronchial rupture, lung atelectasis,
bronchial reconstruction.
Bronchial rupture following blunt thoracic trauma is becoming a frequent event (1).
Although bronchial rupture accounts for almost 3% of all cases of blunt thoracic trauma (2)
the true incidence of bronchial disruption is unknown since most patients die before arriving
to a trauma center (3). About 3% of people dying from accidents have tracheobronchial
disruption (4). Most of the bronchial lesions are the rsult of motor vehicle accidents, though
other etiological agents may be incriminated (5,6). Early diagnosis is important to avoid
serious complications of delayed treatment as permanent bronchial stenosis, parenchyma
destruction by chronic infection and consequent pulmonic resection (7,8,9).
Bronchial rupture has two main forms of clinical presentation. During the early phase
symptoms of dyspnea, cyanosis and thoracic pain are common. Upper rib fractures and
pneumothorax are frequently found on chest x-ray examination in these cases. The other main
form is associated with minor complaints, no signs of acute respiratory distress or
pneumothorax, being frequently misdiagnosed at the emergency room. This second group of
patients with delayed diagnosis includes 24% to 68% of all patients suffering traumatic
rupture (4). The diagnosis of bronchial rupture is done some time later during routine

radiological examination of the thorax or after developing symptoms from the associated
atelectasis (4).
This paper discusses two patients managed at our hospital for bronchial rupture after
delayed diagnosis.
CASE REPORTS
Two patients were referred to the emergency room of the Hospital Carlos Chagas with
lobar atelectasis and history of blunt thoracic trauma.
The first patient is a six-year-old boy with history of a motor vehicle accident. In the initial
evaluation at the emergency room he had moderate thoracic pain and dyspnea. A left clavicle
fracture and left lung contusion were diagnosed. Stayed in the hospital under medical
observation for five days and was sent home with no symptoms of respiratory distress.
Progressively develop exercise dyspnea and forty days later during chest x-ray examination
total opacification of the left hemithorax is observed. Bronchoscopic examination confirmed
the diagnosis of complete rupture of the left main bronchus.
The second patient is a five-year-old girl admitted with mild fever, cough and dyspnea.
The chest x-ray examination reveals opacity of the right hemithorax (Fig. 1). History reveals
she sustained thoracic trauma after a television set fell over her chest two years previously.
Since then, two admissions to different hospitals with diagnosis of pneumonia were reported.
A thoracic CT-Scan revealed total atelectasis of the left lung (Fig. 2). Total rupture of the left
main bronchus was detected during bronchoscopic examination.
Resection of the bronchial scar with bronchial anastomosis using absorbable synthetic
5-0 sutures resulted in progressive recovery of the affected lung of both patients (Fig. 3). They
are both now free of symptoms, four years and eighteen months after surgery respectively.
DISCUSSION
Although prompt diagnosis and management of bronchial rupture secondary to blunt
thoracic trauma is desirable, delayed surgical reconstruction of the main bronchus may be
achieved without gross compromise of lung function (10). Frequently a "silent" rupture of the
main bronchus may be misdiagnosed. Those cases demonstrates mild symptoms of
respiratory insufficiency after trauma, and no pneumothorax or rib fractures at the
radiological examination of the chest. Days or even years may go by before the diagnosis is
made. Partial or total lung atelectasis and extensive infiltrates in lung parenchyma diagnosed
during routine radiological examination may be suspicious of bronchial rupture after history of
trauma. Thoracic CT-Scan or Magnetic Resonance Imaging may help in diagnosis (11,12). With
suspicion of bronchial rupture, bronchoscopy confirms the diagnosis (2,7,13,14). Both
patients had their diagnosis confirmed after fiberoptic bronchial examination which is of
paramount importance in patients suffering blunt trauma having symptoms of respiratory
distress, pleural air leaks, lobar atelectasis or persistent pneumothorax (7,9).
Immediate surgical correction of bronchial rupture could reduce the incidence of late
complications such as secondary tissue infection and persistent bronchial stenosis ( 9, 15). In
children, atelectatic lungs heal within a few weeks after bronchial reconstruction if no
secondary infection is present (15). Bronchial reconstruction must always be attempted. Lung
resection is restricted to those patients with tissue necrosis secondary to infection (9). Our
two patients with delayed diagnosis of bronchial rupture had no symptoms of infection. They
were successfully managed with resection of the bronchial scar and anastomosis of the main
bronchus with absorbable synthetic sutures. After uneventful surgical recovery they had
follow-up bronchoscopic examination six moths later with total permeability of the main
bronchus, a chest x-ray with good ventilation of the affected lung and minimal elevation of the
diaphragmatic dome.
BIBLIOGRAPHY
1.Hartley C, Morrit GN: Bronchial rupture secondary to blunt chest trauma. Thorax 48:183-184,
1993
2. Goh SH, Tan SM, Chui P, Low BY: Traumatic bronchial rupture - A case report. Ann Acad
Med Singapore 24: 883-886, 1995
3. Baumgartner F, Sheppard B, de Virgilio C, Esrig B, Harrier D, Nelson RJ, Robertson, JN:
Tracheal and main bronchial disruptions after blunt chest trauma: presentation and
management. Ann Thor Surg 50:569-574, 1990
4. Frimpong-Boateng K, Amoah AGB: Delayed diagnosis and repair of total bronchial rupture:
A report of two cases. East Afr Med J 74:114-115, 1994
5. Roux P, Fisher RM: Chest injuries in children: na analysis of 100 cases of blunt chest
trauma from motor vehicles accidents. J Pediatr Surg 27:551-555, 1992

6. Lin MY, Wu MH, Chan CS, Lai WW, Chou NS, Tseng YL: Bronchial rupture by blunt chest
trauma. Ann Emerg Med 25:412-415, 1995
7. Amauchi W, Birolini D, Branco PD, de Oliveira MR: Injuries to the tracheobronchial tree in
closed trauma. Thorax 38: 923-928, 1983
8. Sirbu H, Herse B, Schorn B, Httermann U, Dlichau H: Successful surgery after complete
disruption of the right bronchial system. Thorac Cardiovasc Surgeon 43: 239-141, 1995
9. Jones WS, Mavroudis C, Richardson JD, Gray LA Jr, Howe WR: Management of
tracheobronchial disruption resulting from blunt trauma. Surgery 95: 319-323, 1984
10. Taskinen SO, Salo J, Haltunen PE, Sovijarvi AR: Tracheobronchial rupture due to blunt
chest trauma: a follow-up study. Ann Thor Surg 48: 846-849, 1989
11. Huson H, Sais CJ, Amendola MA: Diagnosis of bronchial rupture with MR imaging. J Magn
Reson Imaging 3: 919-920, 1993
12. Wan YL, Tsai KT, Yeow KM, Tan CF, Wong HF: CT findings of bronchial transection. Am J
Emerg Med 15:176-177, 1997
13.Parat S, Bidat E, Chevallier B, Lacombe P, Azoulay R, Legardere B, Bouledroua MS: Late
diagnosis of a bronchial fracture after thoracic injury. Ann Pediatr (Paris) 36:205-207, 1989
14. Rossbach MM, Johnson SB, Gomez MA, Sako EY, Miller OL, Calhoonn JN: Management of
major thacheobronchial injuries: a 28-year experience. Ann Thorac Surg 65:182-186, 1998
15. Gaebler C, Mueller M, Schramm W, Eckesberger F, Vecsei V: Tracheobronchial ruptures in
children. Am J Emerg Med 14: 279-284, 1996

Figure 1 - Radiological examination of the thorax with massive atelectasis on the left lung after thoracic trauma two
years before.
Figure 2 - A CT scan of the same patient showing the trachea (arrowhead) and an amputated left main bronchus
(arrow).
Figure 3 - Radiological examination done two months after surgery with good ventilation of the left lung.

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Department of Pediatric Surgery

UNIVERSITY PEDIATRIC HOSPITAL
Puerto Rico Medical Center, Health Science Campus, Rio Piedras, Puerto Rico
00936
The UNIVERSITY PEDIATRIC HOSPITAL is an institution
dedicated to care, research and education of our sick children...
ADMINISTRATION

DEPARTMENT OF PEDIATRIC SURGERY

NEONATAL INTENSIVE CARE UNIT
PEDIATRIC INTENSIVE CARE UNIT
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Administration of the University Pediatric Hospital

Tel (787)-777-3535
Ms. Sylvia Mercado - Executive Director
Mr. Rafael Prez - Administrator
Dr Concepcin Quiones de Longo - Medical Director
Dr Luisa Burgos - Associate Medical Director
Dr Luisa Burgos - President Medical Faculty

Department of Pediatric Surgery

Tel (787)-777-3535 Ext 7242 & 7240
Section of General Surgery
Dr Enrique Marquez - Professor / Director
Dr Humberto Lugo-Vicente - Associate Professor/Associate Director
Dr Teresita Avils- Professor
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Dr Aurelio Segundo - Assistant Professor

Section of Burns
Dr Albert Surez-Director/Associate Professor

Section of Cardiac Surgery

Dr Enrique Mrquez- Chief/Professor

Section of Hand Surgery

Dr Hernan Cestero - Associate Professor
Section of Neurological Surgery
Dr Nathan Rifkinson- Director/Professor

Section of Ophtalmology
Dr Townsend- Director/Professor

Section of Orthopedics
Dr Fernndez-Filiberty- Director/Professor

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Dr Norma Cruz- Director/Associate Professor

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Dr Antonio Puras- Director/Associate Professor

Neonatal Intensive Care Unit of the University

Pediatric Hospital
Tel (787)-777-3535
Dr Marta Valcarcel- Director/Professor
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Pediatric Intensive Care Unit of the University

Pediatric Hospital
Tel (787)-777-3535
Dr Felcita Gotay- Director/Associate Professor
Dr Alicia Fernndez Sein- Associate Director/Associate Professor
Dr Rafael Rodrguez Santana- Associate Professor

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Upcoming meetings and courses...

NOVEMBER 2000
November 2-6: Annual Conference of Indian Association of Paediatric Surgeons
Chandigarh, INDIA
Contact: Prof. K.L.N.Rao, Head of Dept of Paediatric Surgery, Post Graduate Institute of
Medical Education & Research, Chandigarh 160012, India. email: klnrao@hotmail.com
November 23-25: ANNUAL CONGRESS OF THE PORTUGUESE PEDIATRIC SURGERY
SOCIETY
Coimbra, PORTUGAL
Contact: Associao de Sade Infantil de Coimbra (ASIC)- Hospital Peditrico de CoimbraAv. Bissaya Barreto - 3000-076 Coimbra, PORTUGAL TEL.+351 239 480335; FAX 239 484464
E-mail: asic@mail.telepac.pt
November 26-29: 17th Panamerican Congress of Pediatric Surgery
Temuco, CHILE
Contact: DRA. GALICIA MONTECINOS LATORRE, Presidente, Asociacin Panamericana de
Ciruga Peditrica, gmontecinos@usa.net , Fono-Fax: 56 - 45 314857, P.O. Box 734, Temuco,
Chile o Cristina Miranda Gonzlez, Asistente de Informacin Mdica, Centro Saval Temuco
Manuel Montt N 116, Fono-Fax: (45) 216303-232361, E-mail: temco@saval.cl, Temuco, Chile

JANUARY 2001
January 29-31: XXX. INTERNATIONAL SYMPOSIUM OF PEDIATRIC SURGERY
Obergul, Tyrol, AUSTRIA
Contact: Prof. Dr. Ernst Horcher - General Hospital Vienna - University Hospital - Clinical
Department of Pediatric Surgery - Whringer Grtel 18-20 -A-1090 Wien -AUSTRIA Tel: +43/1/
40400-6836 Fax: +43/1/ 40400-6838 e-mail: Ernst.Horcher@akh-wien.ac.at

FEBRUARY 2001
February 14-18: 51st Annual Meeting -American College of Surgeons - Puerto Rico Chapter
San Juan, PUERTO RICO
Contact: Maritza Negroni & Associates Meeting Planners - 10-17 Cordoba Street Torrimar
Guaynabo, PR 00966. Tel (787) 783-0027 / (787) 782-8436. Fax (787) 793-0516.
E-mail: mnegroni@coqui.net
February 24-25: HIRSCHSPRUNGS CON
Coimbatore, INDIA
Contact: Dr Ramkumar Ragupathy - Organizing Secretary - 81 Valluvar Street, Sivananda
Colony, Coimbatore, 641 012. India E-mail: Ramkumar@md3.vsnl.net.in

MARCH 2001
March 22-24: THE 10TH ANNUAL CONGRESS FOR ENDOSURGERY IN CHILDREN
sponsored by IPEG (International Pediatric Endosurgery Group).
Brisbane, AUSTRALIA
Contact: IPEG Registrar - 2716 Ocean Park Blvd, Suite 2030, Santa Monica, CA
90405 USA. Tel:(310) - 314-2500. Fax: (310) - 314-2535. Email: registration@ipeg.org
Website: http://www.ipeg.org/meeting.html

APRIL 2001

April 4-8: 34th Annual Meeting, Pacific Association of Pediatric Surgeons and XIth Council
Meeting, World Federation of Association of Pediatric Surgeons
Kyoto, JAPAN
Contact: Professor Takeshi Miyano, department of Pediatric Surgery, Juntendo University
School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421, Japan. Fax: 81-3-5802-2033;
E-mail: takeshi@med.juntendo.ac.jp
April 26-28: 2nd World Congress of the Pediatric Thoracic Disciplines
Izmir, TURKEY
Contact: Ege University Faculty of Medicine, Pediatric Surgery Department, Bornova 35100
Izmir TURKEY , Fax: +90 232 375 12 88 , E-mail: omutaf@med.ege.edu.tr

MAY 2001
May 3-5: IVth European Congress of Pediatric Surgery
Budapest, HUNGARY
Contact: Andrew B. Pintr, Department of Pediatric Surgery, 7623 Pcs, Hungary. Tel:
00-36-72-310-144; Fax: 00-36-72-314-937; E-mail: pedsurg@apacs.pote.hu
May 8-11:V Brasilian Congress of Endosurgery (with a section of Pediatric Endosurgery)
Rio de Janeiro, BRASIL
Contact: Dr. Gladys Mariani - Pediatric Surgery Section Coordinator. Fax:55 21 4302010 or
55 21 4306496. E-mail:gladys@antares.com.br
May 20-23: American Pediatric Surgical Association - 32nd Annual Meeting
Naples, Florida, USA
Contact: APSA Meeting Manager, The Sherwood Group, 60 Revere Dr., Suite 500,
Northbrooks, IL 60062. Tel (847) 480-9576; Fax (847) 480-9282.

JUNE 2001
June 11-15: PEDIATRIA 2001 - XXIV Congreso Nacional de Pediatria , V Congreso Nacional
de Cirugia Pediatrica , V Congreso Nacinal de Terapia Intensiva Neonatal y Pediatrica, III
Congreso Internacional " La salud del Nio menor de 5 aos" y el II Congreso " La salud del
nio caribeo en los inicios del sigloXXI.
Habana, CUBA
Contact: Profesor Dr. Enzo Dueas Telefono (537)55-2559/55-2560 Fax (537) 55-2558 e-mail
cnscs@infomed.sld.cu
Lic. Zosima L opez Telefonos:(537)28-5199 y 22-6011 al 19
ext1510 Fax (537) 28-7996 / 28-3470 /22-8382 e-mail:zosima@palco.get.cma.net

JULY 2001
July 18-21: British Association of Pediatric Surgeons - 48th Annual International Congress
London, ENGLAND
Contact: Honorary Secretary Mr. L Rangecroft, BAPS Office, Royal College of Surgeons of
England, 35-43 Lincoln's Inn Fields, London WC29 3PH, England.

SEPTEMBER 2001
September 9-14: The 23rd International Congress of Pediatrics, (The IPA World Congress of
Pediatrics) and The 2nd International Congress on Pediatric Nursing
Beijing, CHINA
Contact: Mr. Joe Jia - Congress Coordinator, Secretariat - 23rd International Congress of
Pediatrics (23rd ICP) - C/o Foreign Relations Dept., Chinese Medical Association - 42 Dongsi
Xidajie, Beijing 100710, China. Tel: (86 10) 65250394 / 65134885, Fax: (86 10) 65123754 /

65250394
Email: adc@public.bta.net.cn
September 19-21: 2001 Annual Meeting of Egyptian Pediatric Surgical Association (EPSA)
Cairo, EGYPT
Contact: Prof Alaa Hamza, Secretary of EPSA , E-mail: shamza@idsc.gov.eg

OCTOBER 2001
October 5-6: 14th International Symposium on Pediatric Surgical Research
Conference aimed at gathering people interested in any field of research related to pediatric
surgical disease. Free papers and lectures. Deadline for abstracts: June, 1st, 2001
Madrid, SPAIN
Contact: Prof. Dr. Juan A. Tovar, Hospital Universitario La Paz, P.Castellana 261 28046
MADRID. Tel: +34 91 727 70 19, Fax: +34 91 727 70 33 , e-mail: jatovar@hulp.insalud.es

NOVEMBER 2001
November 4-10: IV CONGRESO DE CIRUGIA PEDIATRICA DEL CONO SUR DE AMERICA
(CIPESUR)
Montevideo, URUGUAY
Contact: Dr Alex Acosta
November 13-16: CIRENDOSC-2001: 1st Cuban Congress and International Workshop in
Endoscopic Surgery
Habana, CUBA
Contact: Prof. Julin Ruiz Torres - President Organizing Committee, Dr. Rafael Torres Pea Promotional Coordinator, Centro de Ciruga Endoscpica - Hospital Universitario "General
Calixto Garca" Calle G y 27, Ciudad de La Habana, Cuba. Tel: (53-7) 33-4190, 55-2161,
55-2162 Fax: (53-7) 33-3319, 33-4190 E-mail: congreso2001@cce.sld.cu

FEBRUARY 2002
February 13-16: 52nd Annual Meeting - American College of Surgeons - Puerto Rico Chapter
San Juan, PUERTO RICO
Contact: Maritza Negroni & Associates Meeting Planners - 10-17 Cordoba Street Torrimar
Guaynabo, PR 00966. Tel (787) 783-0027 / (787) 782-8436. Fax (787) 793-0516.
E-mail: mnegroni@coqui.net
Last updated: January 2001

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New Released VIDEO:

Trans-Anal Modified Endorectal Pull-Through (Soave-Boley) Procedure for Hirschsprung's
Disease without Abdominal Incision in a Neonate
Figure 1:
Barium enema depicting transitional zone in recto-sigmoid colon.

Type: VSH
TITLE
"Primary Trans-Anal (Incision-less) Endorectal Pull-Through for Hirschsprung's Disease in a Newborn"
HISTORY
3 week-old TAGA baby girl born with constipation and painless abdominal distension.
Barium enema shows a transitional zone in recto-sigmoid colon (see figure 1 above).
Suction rectal biopsy demonstrated no ganglion cells. Rectal irrigation's until 4 kg of weight.
Single stage trans-anal modified endorectal pull-though (Soave-Boley) done without abdominal incision. Duration of
procedure - 90 minutes. Send home one day after surgery. Doing well.
Technique in Detail
Diagnosis of HD is made with the help of clinical manifestations, barium enemas findings and suction rectal biopsy. This technique pertains to cases with classic
recto-sigmoid aganglionosis although we believe long-segments cases can also be managed similarly.
The child receives oral electrolyte solution and bowel cleansing the day before surgery. Preoperative prophylaxis with broad-spectrum antibiotics is used. In a
modified lithotomy position the abdomino-perineal area is prepared and draped. The anus is gently dilated manually. Stay sutures placed around the ano-cutaneous
junction permits eversion of the entrance of the anus. Alternatively you can use a circumferential retractor system (1). Around one centimeter above the pectinate line the
area is infiltrated with an epinephrine-saline 1:200,000 solution. Multiple fine silk traction sutures are placed circumferentially in the mucosa where the dissection will
commence. Using sharp dissection with small curved scissors separation of the submucosa layer from the circular internal sphincteric fibers is performed bringing forth
the mucosa-submucosa tube of tissue. Needle tip cauterization of small vessels may be needed.
Once the dissection goes smoothly the submucosal plane meets the peritoneal reflection. The transanal submucosal dissection is extended above the extra
muscular plane (2). At this point the bowel wall is opened in its most anterior portion and entrance into the peritoneal cavity is obtained. The seromuscular coat is cut
circumferentially and the bowel can be mobilized into the perineal area further by coagulating the vessels and ligamentous attachments near the bowel wall. Once the
transitional zone has reached without tension to the anocutaneous junction a piece of bowel wall is send for frozen section confirmation of ganglion cells. The bowel is
amputated and a single layer colo-anal anastomosis using polyvycryl 0000 alternated with 00000 interrupted sutures is done.
No postoperative bowel decompression is needed (NG). After two hours in recovery room the child is sent to the ward and po fluid liquids are given six hours after
surgery. Prophylactic antibiotics are completed for no more than twenty-four after surgery. Once bowel function returns (generally twelve to eighteen hours later) the child
is sent home. Two weeks after surgery a rectal exam is done.

References
1- Saltzman DA, Telander MJ, Brennon WS, Telander RL: Transanal Mucosectomy: A Modification of the Soave Procedure for Hirschsprung's Disease. J Pediatr Surg
31(9): 1272-1275, 1996
2- Georgeson KE, Fuenfer MM, Hardin WD: Primary Laparoscopic Pull-Through for Hirscsprung's Disease in Infants and Children. J Pediatr Surg 30(7): 1017-1022, 1995

AUTHORS
Ivn Figueroa-Otero, MD FACS FAAP
Humberto Lugo-Vicente, MD FACS FAAP
Section of Pediatric Surgery, Department of Surgery, University of Puerto Rico School of Medicine and the University
Pediatric Hospital, San Juan, Puerto Rico.

Duration of video: 18 min (Voiceless)

Cost: $ 40.00 US dollars (shipping and handling included)

To obtain a copy of the video send check or money order to:

Humberto Lugo-Vicente, MD
P.O. Box 10426
Caparra Heights Station
San Juan, Puerto Rico 00922 USA
***********
Tel (787) 786-3495
Fax (787) 720-6103
Sorry, no credit cards.
Allow 2-4 weeks for delivery.

Home
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Last updated: March 2001

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