Portal vein thrombosis leading to pre-sinusoidal non-cirrhotic portal hypertension resulting in decreased synthetic function of the liver

摘要

Non-cirrhotic portal hypertension (NCPH), defined as elevated portal pressures in the absence of cirrhosis, is a relatively rare cause of elevated portal pressures in western countries. In NCPH decompensated liver disease is common, but complications are often mitigated by appropriate medical therapy. Liver synthetic function loss is uncommon. We present a unique case of a patient with biopsy proven NCPH, who eventually developed progressive loss of hepatic synthetic function in the setting of long standing portal hypertension. This loss of synthetic function corresponded with the interval development of incomplete septal cirrhosis (ISC), and progression of previously noted nodular regenerative hyperplasia in biopsies performed 7 years apart. Our patient’s clinical course was complicated by multiple hospitalizations for gastrointestinal hemorrhage. Patients with ISC have higher rates of bleeding varices when compared to patients with macronodular cirrhosis. While patients with NCPH typically have better overall survival and fewer bleeding complications than cirrhotic patients, this is typically attributed to the former having preserved synthetic function. It appears that the presence of ISC may be a poor prognosticator in patients with NCPH.