Clinical characteristics and outcome of budd-chiari syndrome at a tertiary care hospital in Pakistan

Objective: To determine the clinical characteristics of Budd-Chiari syndrome (BCS), its causes and outcome at a tertiary care hospital. Study Design: An observational study. Place and Duration of Study: The Aga Khan University Hospital, Karachi, from 2004 to 2014. Methodology: A retrospective analysis of data was conducted. A predesigned questionnaire was filled from medical records of patients with BCS. Clinical features, etiology, management and outcome was noted from 2004 to 2014. Descriptive statistics were determined. Results: Forty-five patients' charts were reviewed; 26 (57.8%) were male patients. The median (IQR) age at diagnosis was 26.0 (20.5 to 34.5) years. Primary BCS was seen in 27 (60.0%) patients. The most frequent clinical features included ascites (82.2%), abdominal pain (55.6%), and hepatomegaly (31.1%). A combined hepatic vein/inferior vena cava block was found in 25 (55.6%) patients. Out of the 28 tested patients protein C and protein S deficiencies were detected in 22 (78.6%) and 17 (60.7%) patients, respectively. Antithrombin III deficiency was detected in 14 (58.3%) of those tested patients. Anticoagulants were used in 24 (53.3%) patients. TIPS was done in 11 (24.4%) patients. Mortality was 6.7% (n=3). Conclusion: Congenital thrombophilia was a major causal factor. Age, clinical features, biochemistry and management are important factors in survival.