Non-Cirrhotic Portal Hypertension of Unknown Etiology, May Be from Thrombosis of the Extrahepatic Portal Vein

Abstract

Purpose: 55-year-old adult female with h/o colon cancer 3 years ago had right hemicolectomy and chemotherapy, presented with GERD symptoms and found to have large esophageal varices. Liver function is preserved. Screening tests for specific liver diseases including HBsAg, autoantibodies, serum ferritin, ceruloplasmin were all normal. CT of abdomen revealed extra hepatic portal vein thrombosis; hematology evaluation to evaluate other causes of hypercoaguable state is negative. She has multiple cysts throughout the liver on imaging and later patient developed esophageal variceal hemorrhage. After ultrasonographic and biopy evaluation, a diagnosis of idiopathic non-cirrhotic portal hypertension was made. Percutaneous liver biopsy showed non-specific changes and did not provide definitive diagnostic histologic evidence of either hepatic venous outflow obstruction or of pathology involving the major portal vein. Endoscopy showed extensive oesophageal varices with stigmata of bleeding. She has undergone esophageal variceal ligation several times electively and another time for a bleed. She saw a liver surgeon regarding whether she might be a candidate for a shunt procedure such as a distal splenorenal shunt. Conclusion: Noncirrhotic portal hypertension results from thrombosis of the extrahepatic portal vein. Multiple etiologies may cause the disorder, although nearly half are idiopathic. In this case, it's not certain whether or not the large number of liver cysts decreased liver parenchyma contributed to increased portal pressure and esophageal varices. Endoscopic management of esophageal variceal bleeding is the preferred therapy. However, when endoscopic treatment fails to control variceal hemorrhage, a distal splenorenal shunt is likely to be the most successful operation.