S. Mão de Ferro1,3, M. Salazar1,3, R. Tato Marinho1, H. Glória1, H. Cortez Pinto1, N. Fatela1, A. Marques2, F. Aldeia4, F. Ramalho1, M. Carneiro de Moura1,2
Resumo
Caso Clínico: Homem de 73 anos. Colecistectomia há 9 anos. Colangite com 5 dias de evolução. Ecografia abdominal e CPRM mostraram dilatação e litíase das vias biliares intra-hepáticas esquerdas, sugestivas de doença de Caroli segmentar. Medicado com Imipenem. Quatro dias após a alta novo internamento por colangite. Efectuada CPRE com esfincterotomia e remoção dos cálculos da via biliar intra-hepática esquerda. Por recidiva e doença segmentar, submetido a hepatectomia esquerda. Comentários: A doença de Caroli é uma doença congénita, frequentemente assintomática nos primeiros 5 a 20 anos e raramente durante toda vida. Consiste na dilatação das vias biliares intra-hepáticas, em 20% dos casos com envolvimento segmentar. Litíase intra-ductal e colangites de repetição são complicações frequentes. A cirurgia corrige a doença segmentar.
Segmental Caroli's Disease in a 73 years male
Summary
Case Report: A 73 year old male was admitted with cholangitis. Abdominal ultrasound and magnetic resonance cholangiopancreatography showed sacular dilation and stones in the left intrahepatic biliary tree, compatible with segmental Caroli's disease. He was treated with Imipenem. Four days after discharge he was readmitted with cholangitis and underwent a ERCP with sphincterotomy and removal of the stones. Due to recurrence of the symptoms a left hepatectomy was performed. Comments: Caroli's disease is a congenital condition, frequently
asymptomatic before age 20 years or more rarely throughout the entire lifespan. It is characterised by intrahepatic ductal dilations. Segmental involvement is present in 20% of cases. Intraductal stones and cholangitis are frequent complications. Surgery is the treatment of choice for segmental disease.
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Bibliografia
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(1) Unidade de Hepatologia, Serviço de Gastrenterologia, Hospital de Santa Maria, Lisboa, Portugal.
(2) Serviço de Gastrenterologia, Hospital de Santa Maria, Lisboa, Portugal.
(3) Serviço de Gastrenterologia, Instituto Português de Oncologia de Lisboa Dr. Francisco Gentil, EPE, Lisboa, Portugal.
(4) Serviço de Cirurgia III, Hospital de Santa Maria, Lisboa, Portugal.
Recebido para publicação: 14/03/2006
Aceite para publicação: 24/11/2006
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