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- Title
Functional Budd-Chiari Syndrome Associated With Severe Polycystic Liver Disease.
- Authors
Miranda de Menezes Neves, Precil Diego; Pedroso Balbo, Bruno Eduardo; Hitoshi Watanabe, Elieser; Rocha-Santos, Vinicius; Andraus, Wellington; Carneiro D'Albuquerque, Luiz Augusto; Onuchic, Luiz Fernando
- Abstract
A 50-year-old woman with end-stage renal disease secondary to autosomal dominant polycystic kidney disease was referred to a quaternary care center due to significantly increased abdominal girth. Her physical examination revealed tense ascites and abdominal collateral veins. A 10-L paracentesis improved abdominal discomfort and disclosed a transudate, suggestive of portal hypertension. A computed tomographic scan revealed massive hepatomegaly caused by multiple cysts of variable sizes, distributed throughout all hepatic segments. Contrast-enhanced imaging uncovered extrinsic compression of hepatic and portal veins, resulting in functional Budd-Chiari syndrome and portal hypertension. Although image-guided drainage followed by sclerosis of dominant cysts could potentially lead to alleviation of the extrinsic compression, the associated significant risk of cyst hemorrhage and infection precluded this procedure. In this scenario, the decision was to submit the patient to a liver-kidney transplantation. After 1year of this procedure, the patient maintains normal liver and kidney function and refers significant improvement in quality of life.
- Subjects
BUDD-Chiari syndrome; PORTAL hypertension diagnosis; THROMBOSIS diagnosis; HEPATOMEGALY; CHRONIC kidney failure; COMPUTED tomography; KIDNEY transplantation; LIVER transplantation; PARACENTESIS; PHYSICAL diagnosis; POLYCYSTIC kidney disease; PORTAL vein; QUALITY of life; HEPATIC veins; DIAGNOSIS
- Publication
Clinical Medicine Insights: Gastroenterology, 2017, Issue 10, p1
- ISSN
1179-5522
- Publication type
Article
- DOI
10.1177/1179552217713003