Primary extrahepatic portal vein obstruction in adults: a single center experience

Extrahepatic portal venous obstruction (EHPVO) is a heterogenous disease with regards to etiology, pathogenesis, age, and geographical location. This study analyzed our experience with EHPVO in adults aged >20 years. EHPVO associated with pancreatitis and abdominal lymph node tuberculosis compressing the portal vein was excluded.

Retrospective analysis of the hospital database from January 2000 to December 2009 was done. All patients with liver disease who are attending our department were also prospectively evaluated with Doppler ultrasound for the portal venous system to study the prevalence of EHPVO. Clinical, biochemical, and imaging findings; work up for thrombophilia; treatment given; and follow up were evaluated.

In the retrospective analysis, primary EHPVO in adults was seen in 108/10,095 (1 %), and in the prospective analysis, it was seen in 16/2,188 (0.73 %). The main clinical presentations were abdominal pain and variceal bleed. Imaging findings included portal cavernoma, portal biliopathy, gallbladder calculi, collaterals, and ascites. The major causative factors identified were hyperhomocysteinemia, antiphospholipid antibodies, and myeloproliferative disorders, while, in a third of patients, none of these risk factors could be identified. Twelve patients were subjected to surgery, while the remaining patients were managed either by medical, endoscopic, or interventional radiological techniques. More than 2 years of follow up was available in 90 patients; two patients died due to uncontrolled bleeding, seven patients required surgery, seven patients showed deterioration in liver function, and one patient developed hepatocellular carcinoma. In the prospective study, three patients were subjected to surgery, and the others were managed medically.

Primary EHPVO is an uncommon cause of portal hypertension in adults in India, and its etiological spectrum is comparable to the West.