eP402 - A Case of Cryptogenic Multifocal Ulcerous Stenosing Enteritis

Purpose/Background: Cryptogenic Multifocal Ulcerous Stenosing Enteritis (CMUSE) can present with similar symptoms as Crohn’s disease. We aim to describe the diagnosis and management of an uncommon pathology.Methods/Interventions: A 24-year-old male presented to the office after hospitalization for small bowel obstruction. He reported a history of chronic, recurrent abdominal pain, diarrhea, anemia, arthritis, and obstructive episodes since childhood. He was previously evaluated for Crohn’s disease, but a colonoscopy with biopsy was negative. His gastroenterologist treated him with prednisone, iron transfusions, and biologics. A Computed tomography (CT) scan showed multiple small bowel strictures. Magnetic resonance imaging (MRI) revealed nonspecific inflammation in the ileum. To further evaluate the etiology of his small bowel obstruction, a capsule endoscopy, push enteroscopy, and colonoscopy were performed. The capsule endoscopy showed multiple areas of chronic-appearing strictures. Biopsy was performed by push enteroscopy. The colonoscopy revealed a grossly normal-appearing colon and distal ileum. The most proximal and distal aspects of stenosis were tattooed in anticipation of surgical management.Results/Outcomes: The biopsy revealed mucosal ulcers with fibrinopurulent exudate. The cause of these ulcers remained unclear, and the patient was consented for a diagnostic laparoscopy with possible bowel resection and strictureplasty. Diagnostic laparoscopy was performed and showed no signs of Crohn’s disease or adhesions. The umbilical incision was extended to externalize the small bowel for further evaluation. An enterotomy was made through one of the strictures to allow passage of a pediatric colonoscope to the proximal and distal tattoos. Strictures that could not be passed by the colonoscope were managed with strictureplasty or resection. Postoperatively, the patient initially suffered an ileus but recovered well and in follow up reported improved symptoms. Final pathology showed superficial ulcerations and submucosal fibrosis without transmural disease, indicative of cryptogenic multifocal ulcerous stenosing enteritis (CMUSE). His case was discussed at a multidisciplinary inflammatory bowel disease conference, and he was prescribed lansoprazole for mucosal protection and surveillance with MRI.Conclusion/

Discussion: First described in 1964, there are approximately 60 cases of CMUSE reported in the literature, most presenting in the fifth decade of life. Diagnostic criteria include unexplained small bowel strictures, chronic abdominal pain with recurrent obstruction, superficial ulcers without signs of systemic disease, and persistent occult blood loss. CMUSE is associated with mutations in SLCO2A1, a prostaglandin transporter, and PLA2G4A, a protein that catalyzes the release of arachidonic acid. The differential diagnosis includes inflammatory bowel disease, non-steroidal anti-inflammatory drug-induced enteropathy, tuberculosis enteritis, and viral etiologies. Push enteroscopy and capsule endoscopy are the diagnostic modalities of choice, with pathology providing definitive diagnosis. Most providers use steroids in their treatment but risk subjecting the patient to lifelong steroid dependence. Some have demonstrated improvement through use of biologics or hormonal therapy. Ultimately, surgery with resection and strictureplasty can provide definitive diagnosis and treat refractory cases. Despite these measures, many patients recur.Images and Pathology of CMUSEA. Coronal view of the CT scan taken on presentation for small bowel obstruction
B. Capsule endoscopy image of stricture
C. Push enteroscopy image of stenosis
D. Histopathology of the small intestine mucosa, submucosa and muscularis propria of stricture at 2x magnification
E. Histopathology at 4x magnification
F. Histopathology at 20x magnification showing lymphocytes and plasma cells in the submucosa