Thrombosis of portal vein in absence of hepatic disease.

Of 602 patients referred for portal hypertension during a 20 year period, 18 children (mean age of 5.0 years) and 29 adults (mean age of 26.5 years) had thrombosis in the portal bed with a healthy liver (7.8 per cent incidence). In 29 of the 47 patients we studied, the causative factor remained obscure. Variceal hemorrhage occurred in all and was the indication for our observation and treatment. In 11 adult patients (mean age of 22.1 years), surgical treatment was not performed because of reluctance of the patient or because of technical reasons: nine of the patients rebled and one patient died because of massive bleeding. Direct procedures on esophageal varices had a high rate of rebleeding (seven of 11 patients) independent of the age of the patient when the operation was performed. In 13 patients, shunting offered a good chance of long term protection: three of the 13 rebled at a mean follow-up study of 5.7 years. No recurrence of bleeding was observed in 15 patients when variceal eradication had been obtained after chronic endoscopic sclerotherapy at one to eight years of follow-up study (mean of 3.9 years). The data from this study indicate that sclerotherapy should be the first choice of treatment to control recurrent bleeding in extrahepatic portal hypertension caused by thrombotic obstruction, particularly in the young patient.