A Rare Case of Extensive Intestinal Atresia Involving Small and Large Bowel

Hereditary multiple intestinal atresia ( HMIA) is the rarest form of intestinal atresia, first reported by Winter and Zeltzerin 1956. In this case report we are presentinga pre-term male baby born at 35 weeks of gestation. The patient did not pass meconium. He had dark green vomiting and epigastric fullness. There was rectal atresia atabout 3 cm from the anal verge. On laparotomythere was atresia at and beyond the Duodenojejunal junction, the distal bowel being extremely small in caliber and the whole intestine was absolutely solid with no lumen, involving both the small and large intestine right up to the anal canal. The bowel was non-canalized and solid with no mucosa at all. Enterotomy was done at different levels to check the patency of the gut but everywhere the there was no lumen and even the large bowel including the rectum was atretic. Biopsy report revealed multiple microscopic lumens with all the microscopic layers. Immunological work up revealed severe immunodeficiency.