Hirschsprung Disease

Overview

Hirschsprung disease is a rare congenital disorder characterized by the absence of ganglion cells in a segment of the distal bowel, extending proximally to varying lengths. The affected segment cannot relax or propel stool, causing functional obstruction. Most cases are diagnosed in the neonatal period.

Symptoms

• Abdominal distension
• Intractable constipation
• Difficulty passing stool
• Vomiting

Treatment

Treatment usually requires surgical removal of the aganglionic bowel segment, with preoperative decompression enemas and gradual restoration of bowel function afterward.

Dietary advice

After surgery, progress gradually from liquids to an easily digestible diet, adding dietary fiber and fluids to prevent constipation.