"Endemic" idiopathic portal hypertension: report on 32 patients with non-cirrhotic portal fibrosis.

The clinical features, surgical management, and long term follow up of 32 patients from Iran with idiopathic portal hypertension are reported. Many features of the disease are similar to those reported from India and Japan. The unsuspected finding was a 46% history of marked pica of clay (geophagia) in a subset of 26 patients. In addition, 81% of our patients had a prolonged prothrombin time, despite otherwise normal to minimally abnormal liver function tests. Liver biopsies revealed intrahepatic periportal fibrosis with subintimal thickening of terminal branches, and in many specimens a striking peri-ductular fibrosis was seen in the adjacent bile ducts. The spleen was very large with a dilated artery (external diameter: 11 mm to 15 mm). Portal venous pressure (PVP) was measured intra-operatively before and after clamping the splenic artery (SA). Clamping the SA consistently caused a decreased in PVP which ranged from 2.0 to 18.2 cm water with the mean +/- SEM of 9.7 +/- 1.5 cm water (p < 0.001, paired t-test). It was equivalent to 32.3 +/- 3.6% decrease in PVP. Fifteen selected patients (Group I) were managed with splenectomy with excellent short and long term results. The selection criteria for splenectomy included a decrease in PVP to < 24 cm of water after clamping the SA. Three patients from this group were re-examined 10 to 12 years following splenectomy. Cirrhosis had not developed, but the minimal abnormalities in the liver function tests had persisted.(ABSTRACT TRUNCATED AT 250 WORDS)