An Unusual Case of Portal Hypertension Secondary to Primary Hypoplasia of the Portal Vein

Abstract

Primary hypoplasia of the portal vein with secondary portal hypertension and acquired portosystemic collateral circulation is infrequently reported in the veterinary literature. Diagnosis of this condition requires documentation of abnormal hepatocellular function, the lack of intrahepatic or extrahepatic macroscopic congenital portosystemic shunts, and liver histopathology demonstrating portal hypoperfusion in the absence of hepatic inflammation or nodular regeneration. Due to a perceived poor prognosis, many patients with this condition are euthanized; however, those that are spared can be medically managed, in some cases for years. This case report describes the diagnosis and management of a patient with primary hypoplasia of the portal vein and secondary portal hypertension that presented with the severe but typical clinical manifestations of ascites and hepatic encephalopathy, normal liver enzyme concentrations, and normal serum bile acid concentrations.