Small bowel involvement in blue rubber bleb nevus syndrome: MR imaging features

Abstract

Blue rubber bleb nevus syndrome (BRBNS) is a rare condition which is characterized by multiple venous malformations that may affect several organs. Gastrointestinal lesions, which mostly involve the small bowel and distal colon, may be responsible for gastrointestinal bleeding, causing more frequently chronic anemia. We report herein two cases of BRBNS causing gastrointestinal bleeding which were investigated by means of MR imaging. All venous malformations showed markedly high signal intensity on fat-suppressed T2-weighted MR images. In addition, extraintestinal venous malformations displayed high signal intensity similar to that of small bowel venous malformations. Fat-suppressed T2-weighted TSE images show both small bowel and colonic involvement, thus providing useful information relative to the most appropriate therapeutic approach to be used. MR imaging is especially helpful when capsule endoscopy or enteroscopy are contraindicated or not feasible. In addition, because of lack of radiation, MR imaging can be performed repeatedly to assess the evolution of the disease before a planned surgery or endoscopic resection. Also, because BRBNS is by essence a multifocal condition with extraintestinal involvement, MR imaging appears as an appropriate imaging technique to investigate the multiple locations of the disease in one session, thus replacing multiple examinations.