PYLORIC STENOSIS

KABERA Ren,MD PGY III Resident Family and Community Medicine National University of Rwanda

INTRODUCTION
Pyloric stenosis classically presents with the gradual onset of nonbilious projectile vomiting after 3 weeks of age. First described by Hirschsprung in 1888 Ramstedt described an operative procedure to alleviate the condition in 1907 the procedure used to this day to treat pyloric stenosis 20% of infants are symptomatic from birth, and most are symptomatic within the first 2 months after birth.

ANATOMY

INTRODUCTION
Genetics: Multifactorial inheritance risk; recurrence risk 39% if first degree relative affected 1/300-1/1000 live births (male 1/150 live births; female 1/750 live births) . Predominant age: Infancy; onset usually at 3-4 weeks of age, rarely in the newborn period or as late as 5 months of age. Predominant sex: Male > Female (5:1).

ETIOLOGY
Hypertrophy is after birth, leading to gastric outlet obstruction. The pathogenesis remains unclear but postnatal infusion of gastrin produces an identical lesion in newborn puppies. Hypergastrinemia may play an important etiologic role. Gastrin levels are known to be elevated in the newborn. Prostaglandin E2 infusion, which is used to maintain a patent ductus arteriosus in certain cardiac anomalies, has been linked to a higher incidence of infantile hypertrophic pyloric stenosis.

ETIOLOGY
Recent studies show a relative lack of nitric oxide (a smooth muscle relaxant) synthase innervation. Decreased density of interstitial cells of Cajal (ICC) Increased synthesis of epidermal growth factor in the hypertrophied muscle, Primary underlying cause of the disorder is still unclear.

SIGNS AND SYMPTOMS

Epigastric distention Visible gastric peristalsis, sometimes retrograde Palpable tumor (olive) in right upper quadrant Diagnosis traditionally made by palpation of mass Firm, movable, approx 2 cm in length, olive shaped and best palpated from the left. Mass located above and to the right of the umbilicus in the midepigastrum beneath the liver edge

SIGNS AND SYMPTOMS

Jaundice: unconjugated hyperbilirubinemia may be observed, which is thought to result from inadequate glucose absorption and an inability to maintain glucuronyl transferase activity. Diminished stools Prolonged vomiting may lead to dehydration, weight loss, and development of hypochloremic alkalosis. Hypokalemia Intermittent, non-bilious, projectile vomiting of increasing frequency and severity.

DIAGNOSIS
CLINICAL:OLIVE LABORATORY: Early - evidence hypochloremic alkalosis, with low serum chloride and high bicarbonate Later - may have acidosis with low bicarbonate and low potassium Elevated unconjugated bilirubin level PATHOLOGICAL FINDINGS: Concentric hypertrophy of pyloric muscle.

DIAGNOSIS
IMAGING: Upright plain film of abdomen may reveal dilated stomach (filled with fluid and/or air) and relative lack of air in intestines. Ultrasound (first choice if available) shows thickened and elongated pyloric muscle Barium swallow (performed only when diagnosis is not clinically clear) reveals strong gastric contractions and elongated, narrow pyloric canal (string sign); now rarely performed if ultrasound available.

DIFFERENTIAL DIAGNOSIS
Inexperienced or inappropriate feeding Gastro esophageal reflux Gastritis Congenital adrenal hyperplasia Pyloric diaphragm Pylorospasm

MANAGEMENT
In infants who present with dehydration, fluid resuscitation begins before diagnostic procedures are initiated. Correction of alkalosis and hypokalemia is essential before treatment, which is non emergent and performed only after the patient is stabilized. The Ramstedt pyloromyotomy is the surgical procedure of choice, being curative and having mortality rates of 0.0 to 0.5% and an incidence of recurrence of 1%. A longitudinal incision divides the serosal muscle on the anterior surface of the pylorus down to the submucosa.

Thank you