Liver transplantation for severe intrahepatic noncirrhotic portal hypertension

Abstract

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, the explanted livers showed evidence of vascular abnormalities, NRH, and increased fibrosis, but not cirrhosis. Importantly, however, a diagnosis of cirrhosis is not required in this group of patients to qualify them for OLT, and these patients have good long-term graft function after OLT. (Liver Transpl 2005;11:627-634.)

Acute Deterioration of Idiopathic Portal Hypertension Requiring Living Donor Liver Transplantation: A Case Report

“…Four patients died in the early posttransplantation period, due to herpes zoster encephalitis, suicide, sudden rupture of an unsuspected splenic aneurysm, and pneumonia. 2,8,11) Three patients showed evidence suggestive of recurrent with IPH in the graft liver. One patient developed symptoms one year after OLT; 11) 2 patients were asymptomatic, though their liver biopsy findings after OLT suggested recurrence.…”

“…Collagen diseases and autoimmune disorders are known as associated with IPH. 10,11) Immune system plays a role in IPH, so infection may be one of progressive factors for IPH. Though decrease of hepatic blood flow is considered to relate to IPH progression, it is unclear whether decrease of hepatic blood flow affected acute deterioration in this case.…”

“…Several case reports dealing with OLT for severe IPH are summarized in Table 1 [2][3][4][5][6][7][8][9][10][11] . Taking into account these previous reports and the present, there were 40 patients (30 men, 10 women) with a mean age at transplantation of 45.5 years (range: 20-65 years).…”

“…Most IPH patients have a good prognosis with treatment for their esophagogastric varices, but some IPH cases develop end-stage liver failure despite various medical treatments [2][3][4][5][6][7][8][9][10][11] . Such end-stage liver failure is an indication for liver transplantation, but details of such cases have not been fully reported in the literature.…”

Association of celiac disease and portal hypertension: Cirrhotic or noncirrhotic

“…In India, noncirrhotic portal fibrosis (NCPF) is an important cause of portal hypertension (PHTN) seen in up to 18 % cases [5]. NCPF presents in the third and fourth decade and in 20 % to 30 % of cases the liver slowly undergoes parenchymal atrophy with subsequent decompensation [6,7]. In a study of liver explants, which were initially thought to be having CC, NCPF was found to be an important cause of end-stage CLD considered for liver transplantation [8].…”