Abstract

Intrauterine intussusception is an extremely rare cause of intestinal atresia. It may occur at late stage of pregnancy and cause impairment of blood supply to a segment of intestine leading to its resorption and atresia. Of same, the aganglionosis caused by an intrauterine vascular accident has been shown. . The coexistence of Hirschsprung's disease, intrauterine intussusception and ileal atresia has yet to be described in the literature. We reported a rare case of 2-day old newborn presented with acute bowel obstruction. An abdominal radiograph identified distended loops of bowel with no air in the rectum. An emergency surgery was performed. At laparotomy, an ileal atresia associated to ileocecal intussusception were found. Distally, there was an unused colon. A terminally anastomosing resection were performed. The child had developed a postoperative functional obstruction requiring a surgical revision. A corrective anastomsis was found with a dilated proximal small bowel and an unused colon. An ileostomy and multi-level biopsy were performed. Histology confirmed the diagnosis of total colonic Hirschsprung disease. The last surgery was performed at the age of two months. It consists of a total colonic resection and Sovae ileorectal pull-through