Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE): a 20-year single-center clinical and radiologic experience

Abstract

The purpose of this article is to describe clinical and imaging characteristics of confirmed cases of cryptogenic multifocal ulcerous stenosing enteritis (CMUSE). Retrospective review of electronic medical records identified patients considered for a diagnosis CMUSE over 20-years in a single large tertiary center. Clinical data were abstracted and diagnosis was confirmed based on published criteria. Two GI radiologists reviewed CT and MR enterography (CTE/MRE) exams in consensus of confirmed patients to characterize the imaging features of CMUSE. Eight patients with confirmed CMUSE diagnosis were included for image review, and 9 CTEs and 1 MRE were analyzed. Most patients were males (75%) with a median age at diagnosis of 59.5 years (25–71) presenting with iron deficiency anemia (75%). Patients were commonly refractory (87.5%) to their first therapy, including steroids, with half being refractory to surgical intervention. Major imaging features included multiple (≥ 5; 88%; 7/8), short (< 2 cm; 100%; 8/8), circumferential (100%; 8/8) strictures with moderate wall thickening (6–9 cm), and stratified hyper enhancement (100%; 8/8) located in the ileum (100%; 8/8). Median proximal small bowel dilation was 2.95 cm (2.5–4.1 cm). No CMUSE cases demonstrated penetrating disease (e.g., abscess, fistula). CT and MR enterography are invaluable tools in the multidisciplinary diagnostic evaluation of CMUSE, a rare cause of small bowel strictures with overlapping clinical and imaging features of Crohn’s disease and NSAID enteropathy.