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Biliopatia portal hipertensiva : a propósito de um caso clínico
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A trombose da veia porta, na ausência de recanalização, e com a existência concomitante de cavernoma, constitui uma das causas de hipertensão portal. Associado à hipertensão portal existe um conjunto de complicações possíveis das quais se destacam as alterações biliares.
A biliopatia portal hipertensiva refere-se às alterações da árvore biliar provocadas pela presença de cavernoma da veia porta. A sua patogénese não é completamente conhecida. No entanto, a compressão extrínseca provocada pelo cavernoma e a isquemia associada são mecanismos possíveis e amplamente aceites. Apesar da maioria dos doentes apresentar alterações biliares precoces, apenas uma pequena minoria apresenta sintomas. Quando presentes, estes estão associados a episódios de obstrução biliar com ou sem colangite.
A análise bioquímica hepática, a ultrassonografia com doppler, a tomografia computorizada (TC), a colangio-ressonância magnética (RM) e a colangiopancreatografia retrógrada endoscópica (CPRE) são as várias modalidades disponíveis para avaliação da biliopatia portal hipertensiva.
O tratamento é limitado aos casos sintomáticos e deverá ter uma abordagem personalizada. As opções terapêuticas centram-se no tratamento endoscópico (esfincterotomia, limpeza das vias biliares com extração de cálculos e colocação de próteses) e tratamento cirúrgico com criação de “shunt” porto-sistémico.
Apresentamos o caso de um doente de 46 anos com antecedentes de trombose da veia porta com posterior transformação cavernomatosa, de etiologia não esclarecida, que desenvolveu um quadro de icterícia obstrutiva. A investigação realizada e os antecedentes pessoais do doente suportaram o diagnóstico de biliopatia portal hipertensiva. Destacamos a realização de CPRE, após a qual se colocou esta hipótese diagnóstica. Relatamos os achados clínicos, laboratoriais e imagiológicos e a abordagem terapêutica instituída neste doente.
Thrombosis of the portal vein and, in the absence of recanalization, the occurrence of cavernoma is one of the causes of portal hypertension. Associated with this there is a set of possible complications, where the biliary changes are found. Hypertensive portal biliary disease refers to changes in the biliary tree caused by the presence of a portal vein cavernoma. The pathogenesis that accounts for its onset is not fully understood, however, extrinsic compression caused by the cavernoma and associated ischemia are possible and widely accepted mechanisms. Although most patients have early biliary changes, only a small minority have symptoms. When present, these are associated with episodes of biliary obstruction with or without cholangitis. Hepatic biochemical analysis, Doppler ultrasonography, Computed Tomography, Magnetic Resonance imaging with cholangiography, and ERCP are the various modalities available for the evaluation of portal biliopathy. Treatment is limited to symptomatic cases and should have a personalized approach. Therapeutic options focus on endoscopic treatment (sphincterotomy, cleaning of the bile ducts with extraction of stones and placement of prostheses) and surgical treatment with port-systemic shunt. We present the case of a patient of a 46-year-old patient with a history of portal vein thrombosis and subsequent cavernomatous transformation, whose etiology wasn’t clarified and has developed obstructive jaundice. The investigation and personal history supported the diagnosis of portal biliopathy. We emphasize the realization of ERCP, after which this diagnostic hypothesis was placed. We report the clinical, laboratory and imaginary findings and the therapeutic approach instituted in this patient.
Thrombosis of the portal vein and, in the absence of recanalization, the occurrence of cavernoma is one of the causes of portal hypertension. Associated with this there is a set of possible complications, where the biliary changes are found. Hypertensive portal biliary disease refers to changes in the biliary tree caused by the presence of a portal vein cavernoma. The pathogenesis that accounts for its onset is not fully understood, however, extrinsic compression caused by the cavernoma and associated ischemia are possible and widely accepted mechanisms. Although most patients have early biliary changes, only a small minority have symptoms. When present, these are associated with episodes of biliary obstruction with or without cholangitis. Hepatic biochemical analysis, Doppler ultrasonography, Computed Tomography, Magnetic Resonance imaging with cholangiography, and ERCP are the various modalities available for the evaluation of portal biliopathy. Treatment is limited to symptomatic cases and should have a personalized approach. Therapeutic options focus on endoscopic treatment (sphincterotomy, cleaning of the bile ducts with extraction of stones and placement of prostheses) and surgical treatment with port-systemic shunt. We present the case of a patient of a 46-year-old patient with a history of portal vein thrombosis and subsequent cavernomatous transformation, whose etiology wasn’t clarified and has developed obstructive jaundice. The investigation and personal history supported the diagnosis of portal biliopathy. We emphasize the realization of ERCP, after which this diagnostic hypothesis was placed. We report the clinical, laboratory and imaginary findings and the therapeutic approach instituted in this patient.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2017
Palavras-chave
Biliopatia portal hipertensiva Cavernoma da veia porta Icterícia obstrutiva