Non-cirrhotic portal hypertension entity in South Africa? A report of 6 cases

Six patients with portal hypertension and well-preserved liver function as well as classic features of non-cirrhotic portal hypertension on histological examination of biopsy specimens are described. Three of these patients also had extrahepatic portal vein occlusion. All patients had varying degrees of portal hypertension and hypersplenism. Three patients underwent splenectomy and the remainder had sclerotherapy to control variceal bleeding. The overall prognosis is excellent if variceal bleeding can be controlled by appropriate measures. Hence recognition of this infrequently diagnosed entity is important for an accurate prognosis and appropriate therapeutic interventions