Hirschspung Disease

Hirschsprung's disease is a congenital absence of enteric ganglion cells in the submucosal and myenteric plexuses, resulting in a functional bowel obstruction where the aganglionic segment remains tonically contracted and fails to relax during normal peristalsis. The condition predominantly affects males at a 4:1 ratio and classically presents in neonates with delayed passage of meconium beyond 48 hours, progressive abdominal distension, and bilious vomiting, with the potentially life-threatening complication of Hirschsprung-associated enterocolitis occurring in up to 50% of patients. Medical students must recognize this diagnosis early because definitive surgical management through pull-through procedures is curative, while missed or delayed diagnosis can lead to toxic megacolon, sepsis, and significant mortality.