Neuronal Intestinal Dysplasia type B with Massive Small Intestinal Hemorrhage: Report of One Case

Neuronal intestinal dysplasia type B is characterized by poor intestinal motility that usually results in constipation. Massive gastrointestinal hemorrhage is very unusual as the initial presentation in this disease entity. We report on a 3-year-and-7-month-old boy who had experienced two episodes of life-threatening lower gastrointestinal hemorrhage and high fever. Technetium-99m labeled red blood cell scan and the Meckel's diverticulum scan failed to demonstrate the bleeding point. The results of panendoscopy and colonoscopy were negative. Laparotomy revealed a 70 cm-long thick-walled intestine (from 100 cm to 170 cm below the Treitz ligament) with cord like material formed by mucosa debris, blood clot and mucus. Pathology report revealed hyperplasic nerve bundles in submucosal plexus with giant ganglion formation, consistent with neuronal intestinal dysplasia type B.