Hirschsprung’s Disease

Abstract

Hirschsprung’s disease (HD) is characterised by an absence of ganglion cells in the distal bowel extending proximally for varying distances. The absence of ganglion cells has been attributed to failure of migration, proliferation, and survival of neural crest cells. The earlier the arrest of migration, the longer the aganglionic segment. The aganglionosis is confined to the rectosigmoid in 80–85% of patients and 15–20% of patients have longsegment aganglionosis or total colonic aganglionosis. Delayed passage of meconium is the cardinal symptom in neonates with HD. The diagnosis is confirmed on examination of suction rectal biopsy specimens. Of patients with HD, 80–85% have rectosigmoid disease. A one stage pull-through operation can be performed successfully in these patients using a transanal endorectal approach without opening the abdomen. The procedure is associated with excellent clinical results, permits early postoperative feeding, early hospital discharge and no visible scars. During the past two decades, a variety of one stage laparoscopic pull-through procedures have been performed in infants with HD with excellent results. The major benefits of laparoscopic approach are reduced adhesion formation, excellent visualization of the pelvic structures and shorter postoperative recovery time.