Manifestações gastrointestinais na síndrome blue rubber bleb nevus: relato de um caso

 

M. Ferreira¹, S. Mendes¹, J. Torres¹, R. Ferreira¹, C. Agostinho¹, J.M. Pontes¹, E. Panão¹, M. J. Campos¹

 

 

RESUMO

A Síndrome de Blue Rubber Bleb Nevus (SBRBN) é um distúrbio raro caracterizado pela presença de múltiplas malformações vasculares da pele e tracto gastrointestinal, podendo afectar menos frequentemente outros órgãos. A hemorragia gastrointestinal é a manifestação clínica mais frequente, sendo o tratamento conservador na maioria dos casos, estando indicado em casos mais graves terapêutica endoscópica ou cirúrgica. Os autores apresentam o caso clínico de uma paciente do sexo feminino, de 71 anos, cuja conjugação de lesões hemangiomatosas nos lábios, orofaringe, esófago e cérebro permitiram o diagnóstico de SBRBN. O contributo da ecoendoscopia foi decisivo no diagnóstico ao estabelecer a natureza angiomatosa da lesão esofágica. Faz-se uma revisão da literatura enfatizando-se os aspectos clínicos e terapêutica das complicações gastrointestinais deste distúrbio.

Palavras-chave: Síndrome de Blue Rubber Bleb Nevus, malformação vascular, hemangioma.

 

 

SUMMARY

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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Bibliografia

 

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¹Serviço de Gastrenterologia, Departamento de Medicina do Centro Hospitalar de Coimbra, EPE , Serviço de Gastrenterologia IPO Coimbra, EPE2

Correspondência:

Margarida Ferreira

Serviço de Gastrenterologia Centro Hospitalar de Coimbra EPE

Quinta dos Vales, São Martinho do Bispo, Coimbra

Tel: 936488695

E-mail: magaidaferreira@gmail.com

Recebido para publicação: 30/04/2008

Aceite para publicação: 26/09/2008