Face says it all: a curious case of non-cirrhotic portal fibrosis

Abstract

Non cirrhotic portal fibrosis is a disease of obscure etiology and it causes portal hypertension, massive splenomegaly and well tolerated episodes of variceal bleeding young adults due to obliterative portovenopathy in young adults from low socioeconomic backgrounds, having normal or close to normal hepatic functions. It is also called as idiopathic portal fibrosis, though the etiology of NCPF is unclear but thought to be from chronic infections, exposure to medications and toxins, thrombophilia, immunological disorders are genetic factors. In India it accounts for 30 percent of variceal bleeds in non-cirrhotic portal fibrosis. The characteristic portal hemodynamics include intrahepatic perisinusoidal portal hypertension, increased splenic and portal vein blood flow and increased intrahepatic portal resistance. Patients can be misdiagnosed as having liver cirrhosis, but awareness of this condition by looking at the face and general condition along with diligent search of non-cirrhotic portal hypertension can identify this subset of patients who have better prognosis then cirrhotic patients with similar symptoms.