A rare association of ileal atresia and total colonic Hirschsprung's disease

Abstract

Total colonic aganglionosis is the most rare form of Hirschsprung's disease and is defined as the absence of ganglion cells in both myenteric and submucosal plexus extending from the anus to at least the ileocecal valve. Intrauterine intussusceptions, on the second hand, is also extremely rare. It may occur in late stage of pregnancy and cause impairment of blood supply to a segment of intestine leading to its resorption and atresia. The coexistence of total colonic Hirschsprung's disease, intrauterine intussusception and ileal atresia has yet to be described in the literature. We present this rare case of intrauterine intussusception causing an association of ileal atresia and total colonic Hirschsprung's disease. The diagnosis was performed per-operatively.