ABSITE Practice Exam Prep: American Board of Surgery In-Training Examination Study Guide

In patients with Hirschsprung's disease, the leading cause of death is enterocolitis. Hirschsprung's disease is characterized by a congenital absence of ganglion cells in the distal bowel, leading to a lack of peristalsis in the affected segment. This condition results in a functional obstruction, which can cause fecal stasis and bacterial overgrowth in the dilated proximal bowel.

Enterocolitis arises due to the combination of stasis and infection, potentially resulting in severe inflammation, tissue necrosis, and the risk of perforation. This complication can develop acutely and is particularly dangerous in neonates and young children. The clinical presentation typically includes abdominal distension, fever, diarrhea, and in severe cases, signs of sepsis.

The other potential causes of death in children with Hirschsprung's disease, such as sepsis, intestinal obstruction, and malnutrition, can occur but are secondary to the overwhelming complication of enterocolitis. While malnutrition may arise from prolonged obstruction or inability to feed due to severe symptoms, it is not recognized as the leading immediate cause of death. Similarly, sepsis can be a consequence of enterocolitis but is not the primary issue directly attributable to Hirshsprung's itself. Intestinal