Budd-Chiari Syndrome: clinical patterns and therapy

Area of Science:

• Hepatology
• Internal Medicine
• Vascular Surgery

Background:

• Budd-Chiari syndrome (BCS) is a rare condition characterized by hepatic venous outflow obstruction.
• Diagnosis and management of BCS present significant challenges due to its varied presentation.

Purpose of the Study:

• To analyze the clinical characteristics, diagnostic challenges, and outcomes of Budd-Chiari syndrome.
• To evaluate the effectiveness of different treatment modalities for BCS.

Main Methods:

• Retrospective analysis of 36 patients diagnosed with Budd-Chiari syndrome between 1971 and 1980.
• Review of patient demographics, clinical presentation, diagnostic methods (including liver scintiscanning), and treatment outcomes.

Main Results:

• The study included 36 patients (25 women, 11 men) with a wide age range at presentation.
• Oral contraceptive use was noted in 6 of 11 pre-30-year-old female patients.
• A high incidence of classical symptoms and severe liver function abnormalities was observed in patients with shorter illness duration.
• Liver scintiscanning showed characteristic caudate lobe uptake in only 46% of cases.
• Mistaken diagnoses occurred in 8 cases, leading to deterioration in 4.
• One-year survival was 58.3% and was not influenced by age, sex, or etiology.
• Fibrinolytic therapy showed limited benefit, while hepatic transplantation offered promising results in a small cohort.

Conclusions:

• Budd-Chiari syndrome exhibits diverse clinical presentations and diagnostic difficulties.
• Hepatic transplantation appears to be the most effective surgical treatment for Budd-Chiari syndrome.
• Further research is needed to improve early diagnosis and treatment strategies for BCS.