Hirschsprung disease is a congenital condition caused by the absence of nerve cells (ganglion cells) in a segment of the large bowel. Without these nerve cells, the affected segment cannot relax to allow normal passage of stool, causing functional bowel obstruction.
Newborns typically present with failure to pass meconium within 48 hours of birth, abdominal distension, and vomiting.
Older infants and children may present with severe, persistent constipation that does not respond to standard treatment.
Diagnosis is confirmed by suction rectal biopsy, which demonstrates the absence of ganglion cells in the affected segment.
Surgical treatment involves removing the aganglionic segment and joining the normal bowel to the anus. This may be performed in a single stage or with an initial defunctioning stoma depending on the child's condition.
Hirschsprung disease requires lifelong follow-up after surgery to monitor bowel function and screen for enterocolitis, a potentially serious complication. I manage this condition at Vatsalya Children Hospital with a structured long-term follow-up protocol.
Written by Dr Greeshma Suresh, MBBS (NEIGRIHMS), MS General Surgery (Silchar Medical College), MCh Pediatric Surgery (Institute of Medical Sciences, BHU, Varanasi), Paediatric Surgeon, Vatsalya Children Hospital, Mahavir Road, Orderly Bazar, Varanasi, Uttar Pradesh 221002.
