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Recognition and management of Budd-Chiari syndrome. Experience with 143 patients
Insights
Early surgical intervention for Budd-Chiari syndrome improves patient outcomes. Tailoring operative procedures to individual pathology is crucial for better results in this liver vascular disease.
Area of Science:
- Hepatology
- Vascular Surgery
- Gastroenterology
Background:
- Budd-Chiari syndrome is a rare liver disease characterized by obstruction of hepatic venous outflow.
- Common complications include intractable ascites, esophageal varices, and upper gastrointestinal bleeding.
- Lesions can involve hepatic veins or the inferior vena cava (IVC).
Purpose of the Study:
- To classify Budd-Chiari syndrome based on lesion location and extent.
- To evaluate the efficacy and outcomes of surgical treatment versus conservative management.
- To determine the impact of disease severity and stage on operative risk and mortality.
Main Methods:
- Classification of 143 Budd-Chiari syndrome patients into eight categories based on lesion characteristics.
- Grading of disease severity into four stages to assess operative risk.
- Surgical intervention for 104 patients and conservative treatment for 39 patients.
- Follow-up ranging from 1 to 66 months.
Main Results:
- Operative mortality was 9.6% overall, with 0% in Stages I and II, 9% in Stage III, and 21% in Stage IV.
- 73% of surgically treated patients had good outcomes.
- Conservative treatment resulted in a 33.3% mortality rate among Stage IV patients.
Conclusions:
- Early treatment of Budd-Chiari syndrome leads to significantly better outcomes.
- Surgical procedures should be individualized based on etiology and underlying pathology.
- Surgical intervention offers improved survival rates compared to conservative management, especially in advanced stages.
Abstract:
Of 143 patients with Budd-Chiari syndrome treated from December 1982 to August 1988, 100 had intractable ascites, 78 had esophageal varices and 39 had upper gastrointestinal bleeding. In these patients, 49 had membranous obstruction, 134 had occlusive lesions of the inferior vena cava (IVC) above the confluence of the hepatic veins including 50 membranous obstructions, and 9 had occlusion of the hepatic veins. According to the location and extent of lesions, we classified this disease into eight categories; the severity of the disease and corresponding operative risk were used to divide the disease into four grades. One hundred and four patients (73%) were operated upon. There was no death in Stage I and II patients, but in Stage III and IV patients the mortality was 9% and 21% respectively. The overall operative mortality was 9.6%. Follow-up for 1 to 66 months revealed that 73% (76/104) had good results except 4 who died from other causes. Among 39 patients receiving conservative treatment, 13 (33.3%) died of late complications. They all belonged to Stage IV patients. We conclude that the earlier the treatment the better the results, and the operative procedure must be tailored to the etiology and underlying pathology.