ABSTRACT
Hirschsprung’s disease (HD) is a relatively common cause of intestinal obstruction in the newborn. It is characterized by absence of ganglionic cells in the distal bowel beginning at the internal sphincter and extending proximally for varying distances. The aganglionosis is confined to rectosigmoid in over 80% of patients. In the remaining patients, the aganglionosis extends beyond rectosigmoid involving descending colon and transverse colon, or it may involve the entire colon along with a short segment of terminal ileum. Total intestinal aganglionosis with absence of ganglion cells from the duodenum to the rectum is the rarest form of HD and is associated with high morbidity and mortality. 1 – 4
