Non-Cirrhotic Portal Hypertension

Abstract

Non-cirrhotic portal hypertension refers to causes of portal hypertension (PHT) other than cirrhosis which are pre-sinusoidal in nature. The major causes are extra-hepatic portal vein obstruction (EHPVO) and congenital hepatic fibrosis (CHF). Non-cirrhotic portal fibrosis also constitutes a small proportion of paediatric PHT. EHPVO is characterized by obstruction of the extra-hepatic portal vein with or without involvement of the intra-hepatic portal veins or splenic or superior mesenteric veins. What causes EHPVO is unknown however; various theories like infection, umbilical sepsis and hypercoagulable states have been postulated. Endoscopic management is highly successful in controlling acutevariceal bleeding and eradication of esophageal varices. At present the focus in EHPVO has gradually shifted to the management of long-term problems of bleeding from gastric and ectopic varices, growth retardation, portal biliopathy, massive splenomegaly with poor quality of life, hypersplenism and minimal hepatic encephalopathy. Improved outcomes of surgical management of EHPVO along with newer surgical procedures have changed the outlook towards surgery. Congenital hepatic fibrosis has a good long-term outcome in the absence of renal cysts and recurrent cholangitis due to biliary cystic disease. Non-cirrhotic portal fibrosis is seen in adolescents and has a good outcome with endoscopic and surgical management.