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Cryptogenic multifocal ulcerous stenosing enteritis: Radiologic features and clinical behavior

 Jiyoung Hwang  ;  Jin Sil Kim  ;  Ah Young Kim  ;  Joon Seok Lim  ;  Se Hyung Kim  ;  Min Ju Kim  ;  Mi Sung Kim  ;  Kyoung Doo Song  ;  Ji Young Woo 
 WORLD JOURNAL OF GASTROENTEROLOGY, Vol.23(25) : 4615-4623, 2017-07 
Journal Title
Issue Date
Abdominal Pain / diagnostic imaging ; Abdominal Pain / etiology ; Adolescent ; Adult ; Anemia / diagnostic imaging ; Anemia / etiology ; Chronic Disease ; Constriction, Pathologic / complications ; Constriction, Pathologic / diagnostic imaging* ; Constriction, Pathologic / pathology ; Crohn Disease ; Diagnosis, Differential ; Enteritis / complications ; Enteritis / diagnostic imaging* ; Enteritis / pathology ; Female ; Follow-Up Studies ; Gastrointestinal Hemorrhage / diagnostic imaging ; Gastrointestinal Hemorrhage / etiology ; Humans ; Intestinal Obstruction / complications ; Intestinal Obstruction / diagnostic imaging* ; Intestinal Obstruction / pathology ; Intestine, Small / diagnostic imaging ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Rare Diseases / diagnostic imaging* ; Rare Diseases / pathology ; Recurrence ; Republic of Korea ; Retrospective Studies ; Tomography, X-Ray Computed ; Ulcer / complications ; Ulcer / diagnostic imaging* ; Ulcer / pathology ; Young Adult
Computed tomography ; Cryptogenic multifocal ulcerous stenosing enteritis ; Diagnosis ; Small bowel series ; Small intestine
Aim: To investigate the characteristic radiologic findings of cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) which can be differentiated from other similar bowel disease and to assess their clinical behavior.

Methods: Twenty pathologically and clinically confirmed CMUSE patients (males:females = 8:12; mean age: 40.4 years) between March 2002 and August 2015 from seven academic centers in South Korea were retrospectively reviewed. We evaluated small bowel series (SBS; n = 25), computed tomography (CT) enterography (n = 21), magnetic resonance (MR) enterography (n = 2), and abdominopelvic CT (n = 18) images, focusing on enteric and perienteric manifestations. Any change in radiologic features during follow-up period was recorded. We evaluated clinical data including presenting symptoms, laboratory finding and presence of relapse from electronic medical records. Histopathologic findings were also evaluated.

Results: The main symptoms were abdominal pain (n = 12) and anemia (n = 10). All patients showed small bowel strictures (n = 52, mean: 2.6 per patient) on initial CT/MR, located in the ileum (n = 47) or jejunum (n = 5). Strictures showed short-length (mean: 10.44 mm) and circumferential bowel wall thickening (mean: 5.56 mm) with layered enhancement (n = 48) that were also noted on initial SBS (n = 36) with shallow ulcers (n = 10). Some ulcerative lesions or wall thickening progressed into strictures on follow-up SBS/CT, and some strictures revealed recurrent ulceration on follow-up SBS. There were no penetrating disease features like fistula or abscess and no gastrointestinal tract involvement except the small bowel. Nine patients experienced disease recurrence (median relapse-free period: 32 mo) even post-operatively. Histopathologic features of surgically resected specimens were characterized as multiple superficial ulcerations confined to mucosa or submucosa and multiple strictures.

Conclusion: Under characteristic radiologic findings with multiple short-segmental strictures and/or shallow ulcers of the small intestine, CMUSE should be considered when assessing patients with recurrent abdominal pain and anemia.
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1. College of Medicine (의과대학) > Dept. of Radiology (영상의학교실) > 1. Journal Papers
Yonsei Authors
Lim, Joon Seok(임준석) ORCID logo https://orcid.org/0000-0002-0334-5042
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