Pocket Pediatrics: The Massachusetts General Hospital for Children Handbook of Pediatrics (Pocket Notebook Series), 2 Ed.

PYLORIC STENOSIS

Definition (Pediatr Rev 2000;21:249; Adv Pediatr 2011;58:195)

• Gastric outlet obstruction 2/2 hypertrophy and edema of pyloric canal, and antropyloric distension and muscle spasm → vomiting, dehydration, and hypochloremic, hypokalemic, metabolic alkalosis; thought to be an acquired condition

Epidemiology

• Incidence of 1 in 250–1,000, M 4–8× > F, Caucasian predilection

Clinical Manifestations

• Mean age 3 wk, but anytime birth to 5 mo; begins w/ regurgitation → nonbilious vomiting +/− projectile vomiting

• Classically w/ “olive-like” mass palpated in epigastric region, pathognomonic; not always appreciable; exam enhanced by NGT decomp and prone positioning

Diagnostic Studies

• U/S is study of choice, pyloric thickness >4 mm, length >16 mm w/ sens 89%, spec 100%; may need repeat U/S given operator variability

• Most common cause of metabolic alkalosis in infancy; hypochloremic metabolic alkalosis 2/2 acid loss from vomiting and decreased HCO3 secretion

• Excess bicarb can be excreted in urine w/ obligate Na loss, followed by H2O, also 2° hyperaldo 2/2 inc renin release causes distal H+ secretion and paradoxic aciduria

• Can have low or nml K but usually total body K depletion

Treatment

• Reverse metabolic derangements and volume status

• Pyloromyotomy is curative, incision through serosa and mucosal layer. Essentially 100% success rate



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