Cavernous transformation of the portal vein: etiology determines the outcome

BACKGROUND/AIMS: Cavernous transformation of the portal vein is a rare condition with various etiologies and diverse clinical presentations. We aim to assess the disease outcome of different etiologic groups. METHODOLOGY: We retrospectively scrutinized the clinicopathologic characteristics of 35 patients with cavernous transformation of the portal vein, placing special emphasis on the disease extent, progression, and outcome after stratification into different etiologic groups. RESULTS: There were 20 males and 15 females with a mean age of 40.4 years. The etiologies of these patients could be classified into four groups including hematologic disease (n=12, 34.2%), malignant disease (n=8, 22.9%), non-malignant disease (n=6, 17.2%), and idiopathic (n=9, 25.7%). The hematologic disease group had 9 patients with myeloproliferative disease and 3 patients with protein C or protein S deficiency. The hematologic disease group survived well after medication or surgical management. Hepatocellular carcinoma (n=6) was the leading cause in the malignant diseases group, which had a significantly poorer survival rate than the rest (p<0.05). CONCLUSIONS: Cavernous transformation of the portal vein is a long-term sequele of portal vein thrombosis in which the etiology determines the outcome. More aggressive management including long-term anticoagulation in patients with myeloproliferative disorder or underlying prothrombotic states and endoscopic therapy in controlling variceal bleeding may be a more effective treatment for these patients.