Obliterative portal venopathy: Difference between revisions
No edit summary |
|||
| Line 4: | Line 4: | ||
==Overview== | ==Overview== | ||
'''Hepatoportal sclerosis (HPS)''' is a rare disorder characterized by sclerosis of the intrahepatic portal veins, non-cirrhotic portal hypertension, asymptomatic splenomegaly and recurrent variceal bleeding. | '''Hepatoportal sclerosis (HPS)''' is a rare disorder characterized by sclerosis of the intrahepatic portal veins, non-cirrhotic portal hypertension, asymptomatic splenomegaly and recurrent variceal bleeding. The prevalence of this disease is unknown, and the distribution is worldwide, with many cases reported in Asia. The disease can occur at any age, and both males and females are equally affected. HPS typically manifests with overt splenomegaly with pancytopenia, portal hypertension and mild abnormalities in liver function tests. HPS patients may present as persons with generally good health, but with ruptured esophagogastric varices. Most commonly, the condition is detected in investigating a fortuitous finding of hypersplenism or splenomegaly. Ascites, hepatic encephalopathy and jaundice are uncommon. The main histopathologic findings are periportal fibrosis, occlusion of small portal veins, sclerosis of the portal venous system, and proliferation of small vascular channels within/around portal tracts. The disease is slowly progressive. Liver failure, ascites, or encephalopathy may rarely occur in some patients with advanced stages of the disease. The risk of extrahepatic portal vein thrombosis is high. The ætiology of HPS is unclear. Exposure to toxic substances (arsenic or vinyl chloride) or drugs (vitamin A, 6-mercaptopurine), autoimmune and connective tissue diseases, systemic or intraabdominal infections, and clotting abnormalities have been proposed as possible causes of hepatoportal sclerosis.HPS is usually a diagnosis of exclusion. Differential diagnosis from liver cirrhosis is crucial and may be difficult. A large liver biopsy specimen, together with expertise and awareness of the examiner, are requested for optimal diagnosis. Variceal bleeding is the main cause for morbidity and mortality. Treatment of active bleeding and its prophylaxis can follow the recommendations for patients with cirrhosis. In considering splenectomy, the high risk of thrombosis in the portal venous system should be taken into account. Current overallprognosis is good (80% survival at ten years after the diagnosis). Male patients and patients with disease onset before 40 years of age show a poorer prognosis. | ||
==References== | ==References== | ||
Revision as of 19:58, 27 July 2012
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Hepatoportal sclerosis
Overview
Hepatoportal sclerosis (HPS) is a rare disorder characterized by sclerosis of the intrahepatic portal veins, non-cirrhotic portal hypertension, asymptomatic splenomegaly and recurrent variceal bleeding. The prevalence of this disease is unknown, and the distribution is worldwide, with many cases reported in Asia. The disease can occur at any age, and both males and females are equally affected. HPS typically manifests with overt splenomegaly with pancytopenia, portal hypertension and mild abnormalities in liver function tests. HPS patients may present as persons with generally good health, but with ruptured esophagogastric varices. Most commonly, the condition is detected in investigating a fortuitous finding of hypersplenism or splenomegaly. Ascites, hepatic encephalopathy and jaundice are uncommon. The main histopathologic findings are periportal fibrosis, occlusion of small portal veins, sclerosis of the portal venous system, and proliferation of small vascular channels within/around portal tracts. The disease is slowly progressive. Liver failure, ascites, or encephalopathy may rarely occur in some patients with advanced stages of the disease. The risk of extrahepatic portal vein thrombosis is high. The ætiology of HPS is unclear. Exposure to toxic substances (arsenic or vinyl chloride) or drugs (vitamin A, 6-mercaptopurine), autoimmune and connective tissue diseases, systemic or intraabdominal infections, and clotting abnormalities have been proposed as possible causes of hepatoportal sclerosis.HPS is usually a diagnosis of exclusion. Differential diagnosis from liver cirrhosis is crucial and may be difficult. A large liver biopsy specimen, together with expertise and awareness of the examiner, are requested for optimal diagnosis. Variceal bleeding is the main cause for morbidity and mortality. Treatment of active bleeding and its prophylaxis can follow the recommendations for patients with cirrhosis. In considering splenectomy, the high risk of thrombosis in the portal venous system should be taken into account. Current overallprognosis is good (80% survival at ten years after the diagnosis). Male patients and patients with disease onset before 40 years of age show a poorer prognosis.