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Cryptogenic multifocal ulcerative stenosing enteritis (CMUSE): a rare case and a review of the literature
Lauren Hudson1, Niamh Grayson2, Desmond Toomey3
1Surgical Intern, University College Dublin, Midlands Regional Hospital Mullingar, Longford Rd, Robinstown (Levinge), Mullingar, Co. Westmeath, N91 W237, Ireland.
Abstract:
Cryptogenic multifocal ulcerative stenosing enteritis (CMUSE) is a rare enteropathy of unknown aetiology and pathophysiology. It is characterized by chronic and recurrent ulceration and stenosis of the small intestine. We present the case of a 33 year old female who had multiple presentations to our hospital with symptoms of small bowel obstruction. Diagnostic laparoscopy revealed extensive serosal adhesions with mixed transmural inflammation. The mucosa was extensively ulcerated and the bowel wall was congested and haemorrhagic, with hypertrophy of the muscularis propria. Ileoscopy revealed multiple strictures. Following treatment with balloon dilatation and surgical resection, she responded to treatment with immunomodulators and has remained in remission. CMUSE is a rare but important differential of benign SI strictures. The mainstays of treatment are steroids, immunomodulators, balloon dilatations, and surgical resection. Treatment should be aimed at reducing steroid dependence and preserving small bowel length, resorting to surgical resection in cases of occluding strictures.
Insights
Cryptogenic multifocal ulcerative stenosing enteritis (CMUSE) is a rare small bowel disease. Early diagnosis and treatment with steroids, immunomodulators, and procedures can lead to remission and preserve bowel length.
Area of Science:
- Gastroenterology
- Internal Medicine
- Pathology
Background:
- Cryptogenic multifocal ulcerative stenosing enteritis (CMUSE) is a rare enteropathy.
- It presents with chronic, recurrent small intestine ulceration and stenosis.
- The etiology and pathophysiology remain largely unknown.
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