Liver cirrhosis caused by chronic Budd–Chiari syndrome

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Abstract

Chronic Budd–Chiari syndrome (BCS) is a rare cause of liver cirrhosis (LC) and tends to be misdiagnosed in clinical practice. In order to characterize LC caused by chronic BCS, we conducted this retrospective observational study. Medical records of all patients who were initially diagnosed as chronic BCS with LC when discharged from our department from January, 2011 to October, 2016 were reviewed. Cirrhotic patients with known causes and cases lacked key data were excluded. Data of remaining patients was collected and analyzed. A total of 15 cases were included in this study. Patients with LC caused by chronic BCS were characterized by preserved liver function and prominent portal hypertension (PH). Abdominal distention and edema of lower extremities were most common initial manifestations. Intra- or extrahepatic collaterals on imaging studies were of great importance for differential diagnosis. Most of these patients received interventional angioplasty followed by anticoagulation with warfarin and survived without obvious complications of PH. Chronic BCS was a rare but important cause of LC and should always be considered in patients with chronic liver disease and so-called cryptogenic LC. Early diagnosis and timely treatment may improve outcome. Correct interpretation of imaging examinations was fundamental to avoiding misdiagnosis.

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