Non-cirrhotic intrahepatic portal hypertension: A long term follow-up study

The clinico-pathological features and prognosis of 59 cases of non-cirrhotic portal hypertension seen at one hospital over a 30-year period are described. Diagnosis was made on operative wedge liver biopsy in most cases. All but two had clinical features of portal hypertension and 47 had bled from oesophageal varices. In 53, portal decompression was performed without operative death or variceal rebleeding. Subsequent liver histology in 16 cases up to 18 years after diagnosis showed no change in the majority. The relatively static morphology was reflected in the clinical course. Twenty-two patients have died but only 11 as a result of liver disease or its treatment. Actuarial analysis shows the five year survival to be 90 per cent and the 30-year survival 55 per cent in strong contrast to the grim prognosis for cirrhosis. The infrequency of operative liver biopsies and unfamiliarity with the disease lead to underdiagnosis.