Severe polycystic liver disease: An unsual cause of chronic Budd-Chiari Syndrome
<p>Autosomal dominant polycystic kidney disease is a hereditary disease, characterized by the development of cysts in the renal parenchyma with extra-renal manifestations. Liver damage is rarely a source of complications. A Budd-Chiari syndrome could occur following the compression of the supr...
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| Main Authors: | , , , , , |
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| Format: | Book |
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Archives of Clinical Gastroenterology - Peertechz Publications,
2022-10-29.
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| Summary: | <p>Autosomal dominant polycystic kidney disease is a hereditary disease, characterized by the development of cysts in the renal parenchyma with extra-renal manifestations. Liver damage is rarely a source of complications. A Budd-Chiari syndrome could occur following the compression of the supra-hepatic veins by the cysts. It is an exceptional mechanical complication. </p><p>We present a case of a 54 year old woman, with a diagnostic of an asymptomatic autosomal dominat polycystic kidney disease since childhood was admitted to our hospital due to significantly increased abdominal girth. The physical examination showed grade III ascites. A paracentesis for relief at admission disclosed an exudative fluid. A abdominal computed tomographic scan showed multiples cystic lesions in the kidneys and liver, with a large hepatic cyst responsible for compression of the suprahepatic veins and the inferior vena cava resulting in chronic Budd-Chiari syndrome. The treatment was radiological drainage followed by percutaneous sclerosis of cysts to alleviation of the compression. Unfortunately, the patient died a few days after an intraperitoneal cystic rupture. </p> |
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| DOI: | 10.17352/2455-2283.000113 |