CONGENITAL

(INFANTILE)
HYPERTROPHIC PYLORIC
STENOSIS
ANN MARIA MATHEW
19M3754
 DEFINITION

• Hypertrophy of musculature of pyloric antrum, especially

the circular muscle fibres, causing primary failure of
pylorus to relax.
ETIOLOGY

The exact cause of IHPS remains unknown, a lack of nitric oxide synthase in
pyloric tissue has been implicated.
• There is increased risk of developing the condition if newborn gets
erythromycin or azithromycin in first 14 days after birth.
EPIDEMIOLOGY

Common in first born males (4:1)

• Incidence is 4 in 1000 births
• It is familial
• It is seen between 2nd and 8th weeks of age of an infant, the time taken by
the hypertrophied muscle to cause complete obstruction
 CLINICAL FEATURES

Hypertrophy of the circular muscle of the pylorus results in constriction and obstruction of the gastric
outlet, leading to nonbilious, forcible, projectile emesis.

Visible gastric peristalsis (VGP)- wave of contractions from left upper quadrant to epigastrium
• Palpable lump of hypertrophied pylorus which is better felt from left side, olive like mass. It is the
most important clinical feature (95%).
• Constipation
• Loss of hydrochloric acid secondary to persistent emesis leads to hypokalemic, hypochloremic,
metabolic alkalosis, and dehydration.
DIFFERENTIAL DIAGNOSIS

Duodenal atresia (Bilious vomiting is present since birth)

• High intestinal obstruction (e.g. Volvulus neonatorum)
DIAGNOSIS

Clinical examination- palpation of olive mass is pathognomic

Pyloric muscle thickness of more than 3 to 4 mm or length of more than 15

to 18 mm on ultrasound in the presence of functional gastric outlet
obstruction is diagnostic.
• Barium meal shows obstruction. Contrast study is not commonly done; but
when it is done, it shows ‘string sign’ or ‘mushroom sign’ or ‘double track
sign’ with pyloric obstruction.
 MANAGEMENT

• Preoperatively, resuscitation and correction of electrolyte abnormalities are essential. The infant must be
resuscitated with IV fluids to establish an adequate urine output in order to restore acid-base balance. If not,
postoperative apnea can occur due to a propensity to compensate for metabolic alkalosis by retaining
respiratory carbon dioxide.
• The serum bicarbonate level should be normalized to a value of less than 30 mEq/L prior to surgery.
• The dehydration and alkalosis can be corrected by giving 150–180 mL/kg per day of 0.45 per cent saline with
0.15 per cent KCl in 5 per cent glucose. Oral feeding is discontinued and the stomach emptied with a
nasogastric tube; ongoing gastric losses should be replaced with normal saline containing potassium chloride.
SURGERY

• Ramstedt’s operation- hypertrophied muscle is cut along the whole length adequately
until the mucosa bulges out. Mucosa should not be opened (pyloromyotomy). If mucosa
is injured, it should be sutured horizontally using interrupted vicryl or silk sutures.
• The end result should be an intact bulging submucosa from duodenal fornix to gastric
antrum.
• Laparoscopic pyloromyotomy is becoming popular.
RAMSTEDT PYLOROMYOTOMY
COMPLICATIONS OF SURGERY

• Surgical complications are uncommon and avoidable and includes

• duodenal perforation
• hemorrhage
• wound infection and wound dehiscence
REFERENCES

• Bailey and Love 26th edition

• Sabiston Textbook of surgery
• SRB’S manual of surgery