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Hypertrophic Pyloric Stenosis
Hypertrophic pyloric stenosis is obstruction of the pyloric lumen due to pyloric muscular hypertrophy.
Hypertrophic pyloric stenosis may cause almost complete gastric outlet obstruction. It affects 2 to 3 out of 1000 infants and is more common among males by a 5:1 ratio, particularly firstborn males. It occurs most often between 3 wk and 6 wk of age and rarely after 12 wk. The exact etiology is uncertain, but a genetic component is likely because siblings and offspring of affected people are at increased risk, particularly monozygotic twins. Maternal smoking during pregnancy also increases risk. Proposed mechanisms include lack of neuronal nitric oxide synthase, abnormal innervation of the muscular layer, and hypergastrinemia. Infants exposed to certain macrolide antibiotics (eg, erythromycin) in the first few weeks of life are at significantly increased risk.
Symptoms typically develop between 3 wk and 6 wk of life. Projectile vomiting (without bile) occurs shortly after eating. Until dehydration sets in, children feed avidly and otherwise appear well, unlike many of those with vomiting caused by systemic illness. Gastric peristaltic waves may be visible, crossing the epigastrium from left to right. A discrete, 2- to 3-cm, firm, movable, and olive-like pyloric mass is sometimes palpable deep in the right side of the epigastrium. With progression of illness, children fail to gain weight, and dehydration develops (see Dehydration in Children : Symptoms and Signs).
Hypertrophic pyloric stenosis should be suspected in all infants in the first several months of life with projectile vomiting. Diagnosis is by abdominal ultrasonography showing increased thickness of the pylorus (typically to ≥ 4 mm; normal, < 2 mm) along with an elongated pylorus (> 16 mm). If the diagnosis remains uncertain, ultrasonography can be repeated serially or an upper GI series can be done, which typically shows delayed gastric emptying and a string sign or railroad track sign of a markedly narrowed, elongated pyloric lumen. In rare cases, upper endoscopy is required for confirmation. The classic electrolyte pattern of an infant with pyloric stenosis is that of hypochloremic metabolic alkalosis (due to loss of hydrochloric acid and simultaneous hypovolemia—see Metabolic Alkalosis). About 5 to 14% of infants have jaundice, and about 5% have malrotation (see Malrotation of the Bowel).
Initial treatment is directed at hydration and correcting electrolyte abnormalities. Definitive treatment is a longitudinal pyloromyotomy, which leaves the mucosa intact and separates the incised muscle fibers. Postoperatively, the infant usually tolerates feeding within a day.
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