Hypertrophic Pyloric

Stenosis
Pembimbing : dr. Henny Maisara Sipahutar, Sp.Rad
PPDS Pembimbing : dr. Randy Harry
Rahel Imelda Panggabean
190131143
Definition
 A condition characterised by hypertrophy of the two circular
muscle layers of the pylorus.
 Resulting in constriction and obstruction of gastric outlet.

 Described by Hirschprung in 1888

Epidemiology
 The incidence of HPS is approximately 2 to 5 per 1,000 births per year in
most white populations.
 Less common in India, and among black and Asian populations, with a
frequency that is one- third to one-fifth that in the white population.
 The male-to-female ratio is approximately 4:1.

Etiology
 IDIOPATHIC
 GENETIC
 ETHNIC ORIGIN (more in whites): more commonly
seen in Caucasians
 ENVIRONMENTAL
Erythromycin or azithromycin exposure
Transpyloric feeding of premature babies
Pathophysiology
 Hypertrophied muscles
 Gastric outlet obstruction
 Non bilious projectile vomiting
 Gastric fluid loss
 Hypochloraemic hypokalaemic alkalosis
 Paradoxical aciduria
Pathophysiology
Pathophysiology
Clinical Presentation
 ONSET at 2 to 8 weeks of age
(commonly at around one month
of age)

 SYMPTOMS
 Projectile ,forcible, frequent
episodes of non- bilious coffee
ground vomiting 30 to 60
minutes after feeding.
 Weight loss
 Persistent hunger
 Lethargy
 Constipation or
 hunger diarrhoea
Clinical Presentation
 SIGNS
 Palpable,olive shaped, mobile, smooth, firm mass (1.5 to
2 cm) with all borders well made out, moves with
respiration, with impaired resonance on percussion to
right of epigastric area.(95% cases)
 Visible gastric peristalsis from left upper quadrant to
epigastrium (golf ball waves)
 Signs of dehydration
 Jaundice (2%) (due to decreased hepatic glucuronosyl
transferaseassociated with starvation)
 Diagnosis  If the olive is not palpable in
an infant who has a clinical
 Initially suggested by picture suggestive of HPS,
the typical clinical further studies are
presentation. warranted.
 The mass is firm, mobile,
approximately 2 cm, best  Ultrasonography is used to
palpated from the left, located in measure the thickness of the
the mid- epigasrtrium beneath
the liver edge. pyloric wall and the length of
 Palpation of the hard muscle mass the pyloric canal.
or olive is diagnostic in – Normal wall thickness
conjunction with a typical history.
<2mm, HPS >4 mm
 Diagnosis by palpation of olive only
successful 49% of cases in recent – Normal length of
years vs. 78% 30 years ago. the pyloric canal
 Palpation requires a calm infant with
relaxed abdominal musculature, <10 mm, HPS >14
which is difficult in these hungry mm
babies.  Sensitivity and
specificity as high as
100%
Radiology Features
Radiology Features
 ABDOMEN X RAY (erect posture)
• upper abdominal gas bubble in the stomach.
 ABDOMINAL ULTRASONOGRAPHY
(Gold standard at present)
Doughnut sign or cervical
pyloric sign
•pyloric muscle thickeness >4 mm
•pyloric length >16mm in presence of functional
•gastric outlet obstruction
 Radiology Features

BARIUM MEAL/ Fluoroscopy
 Peristaltic waves (caterpillar sign)
 Delayed gastric emptying

 Elongated and narrow pyloric canal- String sign /

Railroad track sign
 The pylorus indents the contrast-filled antrum
(shoulder sign) or base of the duodenal bulb
(mushroom sign)
Radiology Features
• The barium may outline •Bulge in the distal antrum with
crowded mucosal folds as streak of barium pointing
parallel lines - DOUBLE towards pyloric canal- BEAK
TRACT SIGN SIGN

D o u b l e tract s i g n Beak s i g n
Management
 Medical but not a surgical emergency
 RESUSCITATION
 MEDICAL TREATMENT –
Atropine methyl nitrate orally is tried to relax the pylorus
muscle.
Pre Operative Preparation
 Resuscitation with IV rehydration.
 Correct hypovolaemia with 10 ml/kg 0.9 % saline.
 Correct hypochloraemic alkalosis and hypokalaemia (over
24-48 hrs): 0.45% NaCl in 5% dextrose with added KCl at a
rate of 120-150 mL/kg/24hr.
 Nasogastric tube drainage to prevent aspiration of vomited
secretions.

Surgery should take place when:

 Dehydration corrected
 Normal serum Na a n d K
 Chloride ion >90mmol/l
 Bicarbonate ion < 28 mmol/l
Surgery

 FREDET-RAMSTEDT’s PYLOROMYOTOMY>>>
conventional open procedure

 LAPARASCOPIC PYLOROMYOTOMY>
 Case Report
• A male newborn (NB), from the second pregnancy of a 27-year-old mother.
She denies drug addiction. Three normal obstetric ultrasounds. Discharge of
clear transvaginal fluid for 30h. Baby born vaginally with Apgar 8-9, Capurro
“B” 35 weeks, gestation, weight 2550g, height 48cm.
Management starts with fasting, oxygen therapy, ampicillin and amikacin for
seven days due to premature membrane rupture. Insidious respiratory and
infectious evolution. Sudden onset of postprandial non-biliary vomiting and
increase of indirect bilirubin. Given the possibility of nosocomial infection, he
is started on cefepime plus dicloxacillin. X-ray of chest and abdomen shows
gastromegaly (Fig. 1). Oral ingestion is resumed, with enteral stimulation, but
vomiting persists so he continues to fast, a central line is installed and
parenteral nutrition is started. Gastric hyperperistalsis and palpation of pyloric
mass. Blood gas shows normochloraemic metabolic alkalosis. Ultrasound
shows pylorus 18.8mm long, with slices showing muscle wall thickness of up to
4.3mm (Fig. 2), confirming the diagnosis of HPS.
• Surgical finding: 2cm pylorus with pearly appearance. Oral intake resumed
24h after surgery, well tolerated and accepting milk increments. Antibiotics
suspended as there is no evidence of infection. Discharged home with follow-
up as outpatient.
 Case Report
• Figure 1.X-ray of abdomen and • Figure 2.Pyloric ultrasound.
chest. Note the presence of Note increased length and
gastromegaly. thickness of muscle wall.
 Discussion
• Hypertrophic pyloric stenosis is a very rare condition in preterm infants. In full-term
newborns it appears from the second to the seventh week after birth, and its incidence
rate is much higher than in pre-terms. Predominance of the male gender has a genetic
component, as the greater risk of gastrointestinal malformations in that gender is well
documented, as is the risk of rotavirus infections in childhood. This suggests considerable
differences in the development, maturity and function of the gastrointestinal tract
between men and women.
• Mutations in chromosomes and even in chromosome x play an important role in the
development of HPS. However, despite a large variety of studies, it has not been possible
to document a Mendelian inheritance pattern.
• Other risk factors associated to HPS are: caesarian birth and mothers smoking during
pregnancy.  However, caesarian birth as a risk factor is not fully clear, as in some series the
birth method is consistent with maternal infection. Vaginal birth has also been described
as beneficial, as it prevents hormonal cascades and stress factors in newborns. Our patient
was born vaginally and therefore not affected by such a risk factor.
 Discussion
• The clinical presentation often consists of vomiting that could initially be mistaken for
reflux, yet episodes often lead to metabolic alkalosis due to loss of hydrogen ions and
chlorine. This is consistent with our patient's symptoms. This presentation in pre-term
newborns is often diagnosed and treated as gastroesophageal reflux, as premature babies
have multiple risk factors such as the use of methyl-xanthines and immaturity of the upper
oesophageal sphincter. This makes it difficult to consider HPS as the primary origin of
vomiting.
• Our patient was initially treated with anti-reflux measures, using widely recommended
drugs at standard doses.
• The patient was examined, palpating a pyloric mass, a pathognomic sign of the condition.
• Abdominal ultrasound is considered to be a highly sensitive (91%) and specific (100%) test,
which also makes it possible to distinguish duodenal membranes or other malformations
as differential diagnoses. In our patient, the ultrasound showed the findings described in 
Table 1 and Fig. 2, which are consistent with the literature. Three factors have been
suggested for the condition's diagnosis: the presence of delayed gastric output by
ultrasound, pyloric sphincter muscle thickness of ≥2.5mm and muscle length ≥14mm.
 Discussion
Table 1.
Pathognomic findings in abdominal ultrasound.

a) Pyloric diameter from 15 to 18mm 

b) Muscle thickness from 3 to 4mm and length more than 17mm 
c) Narrow intestinal span with characteristic “double track” appearance 

• Laparoscopic, or minimally invasive, surgery is increasingly recommended in the literature,

but requires appropriate medical instruments and equipment to tackle the physiology of
pre-term infants and their reduced surgical fields, with small incisions through the
umbilical scar and excellent results, starting food intake the following day.
References
 Ohshiro and Puri. Pathogenesis of infantile hypertrophic pyloric stenosis: recent progress.
Pediatr Surg Int (1998)13:243-252.
 Macadesi and Oates. Clinical diagnosis of pyloric stenosis: a declining art. BMJ
1993;306:553-555.
 Heller, et al. Application of new imaging modalities to the evaluation of common pediatric
conditions. J Pediatr 1999; 135(5): 632-639.
 Yagmurlu, et al. Laparoscopic pyloromyotomy: a concurrent single institution series. J
Pedatr Surg 2004;(39)3:292-296.
 Schwartz. Hypertrophic pyloric stenosis. Dalam: Coran AG, editor (penyunting). Pediatric
Surgery. Edisi ke-7. Elsevier Saunders; 2012.p.1020-31.
 Rogers IM. New insights on the patoghenesis of pyloric stenois of infancy. a review with
emphasis on the hyperacidity theory. Open Journal of Pediatrcs. 2012;2:97-105.
 Hernanz-Schulman. Infantile hypertrophic pyloric stenosis. Radiology. 2003.hlm.319-31.
 Aspelund G, Langer JC. Current management of hypertrophic pyloric stenosis. Seminar in
Pediatric Surgery. Elsevier. 2007;16:27-33.
 L.P. Islas-Dominguez, et al. Hypertrophic pyloric stenosis in a preterm infant: a case report.
Revista Medica Del Hospital General De Mexico. Elsevier,2018; 81 (4): 217-220.
Thank You