Fatal course of idiopathic chronic ulcerative enteritis with panenteritis and perforation: a case report and review of literature

Idiopathic chronic ulcerative enteritis (ICUE) is a very rare disease with high mortality. Because of clinical rarity, several small case reports have been published and there is a lack of large sample study. Preoperative definite diagnosis is difficult. Although definite treatment for ICUE is radical surgical resection, surgical decision in operative field is difficult. A 77-year-old man came to the emergency department with complaints of a 1-day history of abdominal pain and abdominal distension. Abdominal computed tomography revealed ileus and focal free air. Laparotomy revealed multiple small bowel tiny perforations in the ileum. The serosa surface in the whole small bowel had small multiple yellowish tiny discolored lesions. Despite the presence of multiple mucosal ulcers in entire small bowel, the ileum including perforation site was resected segmentally. Microscopically, mucosal ulcers in resected small bowel demonstrated transmural inflammation, no granuloma, and no lymphoid aggregates. These features were consistent with a diagnosis of ICUE with panenteritis and perforation. After surgery, the patient's general condition gradually aggravated. Unfortunately, the patient died of multiple organ failure on post-operative day 14. Surgically, the decision including resection range, anastomosis or enterotomy becomes difficult in ICUE with panenteritis. According to recent 40 year's revised data, the post-operative mortality of ICUE is about 53.4%. Although ICUE is rare, its recognition is important for appropriate diagnosis and treatment. Retrospective multicenter case studies are required to determine proper treatment and improve prognosis.