Liver Transplantation for Severe Intrahepatic Noncirrhotic Portal Hypertension

作者：Alyssa M. Krasinskas, Bijan Eghtesad, Patrick S. Kamath, Anthony Jake Demetris, Susan C. Abraham · 发表于：Liver Transplantation · 年份：2005 · DOI：10.1002/lt.20431 · 被引用次数：179 · 研究领域：Liver Disease and Transplantation、Organ Transplantation Techniques and Outcomes、Liver Disease Diagnosis and Treatment

Intrahepatic noncirrhotic portal hypertension can be idiopathic or associated with known toxic, developmental, vascular, or biliary tract diseases. Most patients are successfully managed medically or with shunting procedures. The goal of this study was to explore the reasons some patients require orthotopic liver transplantation (OLT). The clinical features, gross and microscopic liver explant pathology, and posttransplantation course in 16 patients who underwent OLT for intrahepatic noncirrhotic portal hypertension were studied. There were 11 men and 5 women with a mean age of 47 years. Clinical manifestations included gastrointestinal varices (n = 12), ascites (n = 8), encephalopathy (n = 3), and hepatopulmonary syndrome (n = 3). Cirrhosis was misdiagnosed clinically, radiographically and/or histologically in 13 patients (81%). Grossly, liver explants weighed a mean of 1,100 g, and 12 had a nodular appearance. Histologically, all 16 livers had portal tract vascular abnormalities, 15 had nodular regenerative hyperplasia (NRH), and 9 had incomplete septal cirrhosis. After OLT, mild NRH features were noted in 2 patients, and 1 of these patients developed evidence of portal hypertension. This study demonstrates that a subset of patients with intrahepatic noncirrhotic portal hypertension have severe symptoms requiring OLT. Accurate pre-OLT diagnosis is frequently difficult at advanced stages of the disease; 81% of our patients carried a diagnosis of cirrhosis. Morphologically, t...