Objective: To evaluate the clinical presentation, possible etiological factors, management and
outcome of patients in our hospital with extrahepatic portal vein obstruction (EHPVO). Materials
and Methods: This study included patients with EHPVO followed up in our department during
last 10 years. Patients of cirrhosis with EHPVO were excluded. Patients’ clinical presentation,
etiology of EHPVO, management and outcome results were analyzed. Results: Of 30 patients,
19 (67.9%) were males. Median age was 12 years. Of 14 patients who underwent liver biopsy
9 had histological activity index stage of 1/6. History of omphalitis and pulmonary tuberculosis
was present in one case each. Of 22 patients with the available thrombophilia profile, nine
patients had a deficiency of protein C, five patients had a deficiency of protein S, one each
had reduced level S of anti-thrombin III and factor V mutation. The predominant presenting
symptom was hematemesis (15 patients, 53.6%). Seven patients (25%) had splenomegaly.
Three patients (10.7%) had no esophageal varices on endoscopy. Three patients underwent
splenectomy due to severe pancytopenia. Endoscopic retrograde cholangipancreatography
was performed in four patients (14.3%) due to portal biliopathy. Common bile duct stenting was
performed in all four patients. Of them, one patient underwent splenorenal shunt operation for
indication of hemobilia. One patient died at the age of 40 years, due to cholangitis and sepsis.
Conclusions: Results from this study show that the anticoagulant deficiency is a common
cause of EHPVO in our setup. Hematemesis is a common presenting symptom. Some of these
patients have symptomatic portal biliopathy.