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Jornal Português de Gastrenterologia

Print version ISSN 0872-8178

Abstract

FERREIRA, M. et al. Manifestações gastrointestinais na síndrome blue rubber bleb nevus: relato de um caso. J Port Gastrenterol. [online]. 2009, vol.16, n.3, pp.115-119. ISSN 0872-8178.

Blue Rubber Bleb Nevus Syndrome (SBRBN) is a rare disease characterized by several vascular malformations of the skin and gastrointestinal tract, being able to afect other organs less frequently. Gastrointestinal bleeding is the more frequent clinical manifestation and treatment is conservative in the majority of the cases, surgery or endoscopic treatment are indicated in the most serious cases. The authors present the clinical case of a 71 years-old woman, with hemangiomatous lesions in the lips, orofaringe, esophagus and brain which are consistent with the diagnosis of SBRBN. Ultrasound endoscopy was decisive in the diagnosis because established the angiomatous nature of the esophageal lesion. The authors reviewed the literature emphasizing the clinical and therapeutic aspects of gastrointestinal complications of this syndrome.

Keywords : Blue rubber bleb nevus syndrome; vascular malformation; hemangioma.

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