Budd-Chiari Syndrome: clinical patterns and therapy.

Retrospective analysis of 36 patients (25 women, 11 men) with the Budd-Chiari syndrome diagnosed between 1971 and 1980 showed a wide range in aged at presentation (12 to 68 years) with the peak incidence in the third decade for women and in the fourth for men. The 11 patients below the age of 30 were women and six of these had been taking oral contraceptive preparations. There was also a wide range in duration of illness before establishment of diagnosis ranging from eight weeks or less in 20 patients, to up to six years in the other 16. Those in the former group had a high incidence of the 'classical' clinical features (tender hepatomegaly with ascites) and most severe abnormalities in liver function tests. Liver scintiscanning showed the characteristic pattern of maximum colloid uptake in the caudate lobe in only 46 per cent of patients. Mistaken diagnosis in eight cases led to early exploratory laparotomy with serious deterioration in four. One year survival of 58.3 per cent was unrelated to age, sex, or aetiology and little benefit was seen in the few cases treated early with fibrinolytic agents. Of the surgical measures employed only hepatic transplantation has proven worthwhile with three out of four cases alive at 14, 16 and 52 months respectively from the time of operation.