Hepatic Dysfunction in Patients with Extrahepatic Portal Venous Obstruction

Background: Extrahepatic portal venous obstruction (EHPVO) developing due to thrombotic occlusion of the portal vein in children is generally considered a benign disease. Whether hepatic dysfunction develops in these patients in the absence of a gastrointestinal bleed has not been well studied.

Materials And Methods: Forty-three patients with EHPVO who had not bled in the last 3 months were studied. Patients were divided into those with (group I) or without ascites (group II). Matched cirrhotic patients with ascites (group III) served as controls. Clinical, biochemical, ultrasonographic, and histopathological evaluation was carried out. Portal biliopathy was assessed in five patients in group I and in 12 patients in group II by cholangiography.

Results: Of 43 EHPVO patients, ascites was seen in nine (21%) patients (group I). Thirty-four patients had no ascites (group II). Serum ALT (54 +/- 24 vs. 34 +/- 10 IU/l, P < 0.01), albumin (3.2 +/- 0.3 vs. 3.7 +/- 0.4 g/dl, P < 0.01), and prothrombin time difference (9.0 +/- 4.5 vs. 2.4 +/- 1.9 s, P < 0.05) were deranged in patients in group I compared with group II. Patients in group I were 4 years older, and the duration of portal hypertension was longer than in group II (11.5 vs. 5.6 year, P < 0.05). Portal biliopathy changes were significantly more severe in group I than in group II patients. Ascites was high gradient in all the patients in group I and the serum-ascitic albumin gradient was comparable between groups I and III. None of the EHPVO patients, but four cirrhotic patients, developed spontaneous bacterial peritonitis during a follow-up of 11 +/- 4 months.

Conclusions: Hepatic dysfunction in the form of ascites and deranged liver functions is not uncommon in patients with EHPVO, more so in patients with prolonged portal hypertension. Based on our data it would be worthwhile to study whether prolonged portal vein thrombosis in EHPVO patients could lead to progressive liver disease.