[Experiences with surgical therapy of Budd-Chiari syndrome]. / Erfahrungen mit der chirurgischen Therapie des Budd-Chiari-Syndroms.

ABSTRACT

The obstruction of the hepatic venous outflow tract with or without involvement of the inferior vena cava results in the Budd-Chiari syndrome (BCS). With its very heterogenous etiology and variable epidemiology the rare disease either takes a chronic or an acute foudroyant clinical course. In general the prognosis is poor. Together with the clinical signs the diagnosis is based on radiological measures and the histology of the hepatic parenchyma. The exact etiological investigation of the BCS is of great significance. Typical findings are discussed and a diagnostic scheme is developed. Between 1979 and 1991, altogether 16 operations were carried out in 13 patients with a BCS. Predominantly there were undertaken a porto-systemic shunt procedure or an orthotopic liver transplantation, respectively, in 6 cases each. The need for an always individually tailored therapeutic strategy of the BCS is underlined by a case history. An overview analyzes the different therapeutic modalities of the BCS and their differential indications.