| Literature DB >> 25631979 |
Gayatri Amit Deshpande1, Inian Samarasam, Sam Varghese George, Sudhakar Chandran.
Abstract
Blue rubber bleb nevus syndrome (BRBNS) is a rare condition characterised by venous malformations in the skin, gastrointestinal tract and other parts of the body. Its presentation is usually sporadic, although cases of autosomal dominant inheritance have been reported. Usually seen in children, BRBNS presentation in adults is rare. Symptoms at presentation depend on the organs involved; patients with BRBNS may present with acute or chronic gastrointestinal bleed. We herein report a rare presentation of BRBNS in an adult who suffered from intermittent abdominal pain and melaena for three years. Contrast-enhanced computed tomography revealed a jejunojejunal intussusception with a vascular malformation as the lead point. The patient underwent laparotomy with resection of the intussuscepted bowel segment. Recovery was uneventful. In spite of a wide range of therapeutic options for the management of BRBNS described in the literature, the efficacy of those available therapies, including surgical excision, is not well established.Entities:
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Year: 2014 PMID: 25631979 PMCID: PMC4294001 DOI: 10.11622/smedj.2014164
Source DB: PubMed Journal: Singapore Med J ISSN: 0037-5675 Impact factor: 1.858