Non Cirrhotic Portal Fibrosis - Current concepts

Abstract

Non cirrhotic portal fibrosis (NCPF) is characterized by perivenular fibrosis of the small and medium branches of the portal vein resulting in portal hypertension (PHT) while the liver structure and function remain normal. This results in an increased portal venous pressure gradient in the absence of a known cause of liver disease. It has been commonly described from India and Japan, and is being increasingly recognised in the West. The etiology of NCPF in unclear but postulated to be chronic infections, exposure to medication or toxins, thrombophilia, immunological disorders or genetic disorders. Multifactorial etiology can also be encountered. The majority of patients present with signs or complications of (PHT), mainly well controlled episodes of variceal bleeding and splenomegaly. They usually have preserved liver function. Patients can be misdiagnosed as having liver cirrhosis, but awareness of this condition (presence of portal hypertension with disproportionately large splenomegaly but preserved liver functions) along with a diligent search to rule out other causes of non cirrhotic PHT, can identify this subset of patients who have a better prognosis than cirrhotic patients with similar symptoms. The recommendations for prophylaxis and treatment of variceal bleeding are similar to cirrhotic patients. While endotherapy can successfully manage most of the varieal bleeding episodes, surgical portocaval shunts are indicated in patients with failure of endotherapy, bleeding from sites not amenable to endotherapy and symptomatic splenomegaly. Since NCPF is a benign disorder with overall good prognosis except for need for frequent endoscopic surveillance and therapy, there is deep interest in role of prophylactic surgery to prevent the first bleeding episode all together and to offer a onetime permanent solution to patients who have recovered from a bleeding episode. But shunt surgery is not entirely innocuous and technically demanding, so the debate continues.