S2891 Cryptogenic Multifocal Ulcerous Stenosing Enteritis: A Rare Finding Causing Recurrent Small Bowel Obstructions

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INTRODUCTION: We present a case of cryptogenic multifocal ulcerous stenosing enteritis (CMUSE), an exceedingly rare disorder, specifically targeting the small bowel. CASE DESCRIPTION/METHODS: A 47 year-old female was referred for recurrent small bowel obstructions (SBO) and opioid-induced constipation. She denied bloody stool, mouth ulcers, rashes, joint pain, and use of NSAIDs. She reported a 20-pound weight loss over a few years. She had no significant tuberculosis risk factors and no family history of IBD or celiac disease. She had a surgical history of appendectomy, cholecystectomy, and abdominoplasty. Physical exam was only notable for surgical scars. Labs were pertinent for iron deficiency anemia and elevated inflammatory markers. She was negative for HIV, HCV, and celiac disease. Patient had 3 episodes of SBO conservatively managed. CT of the abdomen/pelvis showed wall thickening of mid-small bowel loops, SBO, and multiple enlarged mesenteric lymph nodes (3.1 × 3.7cm). Upper endoscopy and colonoscopy showed no inflammation or ulcerations. Video capsule endoscopy could not be performed, due to patency capsule retention. MR enterography (MRE) showed angulation of mid-small bowel loops with areas of wall thickening, narrowing, and distention, no abnormalities of distal and terminal ileum, and enlarged mesenteric lymph nodes. Interventional radiology could not access the lymph nodes to rule out lymphoma. She ultimately underwent a diagnostic laparoscopy, partial small bowel resection, and mesenteric lymph node biopsy. Pathology showed areas of superficial and circumferential ulceration with submucosal fibrosis and thickened muscularis mucosa, favoring CMUSE. DISCUSSION: The differential diagnosis for this presentation included Crohn’s disease and NSAID induced enteropathy. However, based on the clinical features, MRE, and histology, CMUSE is the most likely diagnosis. CMUSE typically has a prolonged presentation, over decades, notably with exclusive small intestine involvement without colonic or gastric pathology or extraintestinal manifestations. As seen in this case, ulcers are predominantly in the ileum, while sparing the terminal ileum. Histology is remarkable for absence of severe inflammation, even in the ulcerated areas, without typical Crohn’s features. Genetic basis and pathogenesis remains unknown, with mutations being investigated. Both surgical management as well as immunomodulators are used, though data remains limited.Figure 1.: Specimen site: small bowel. Several pauci-inflammatory, well delineated areas of ulceration with expansion and fibrosis of the underlying submucosa and thickening of the muscularis mucosae. The muscularis propria and subserosa are normal. The bowel immediately adjacent to the ulcerated areas is not affected. Typical features of Crohn's disease are absent.