Portal hypertension is classified as idiopathic (IPH) when all other causes have been excluded, and it is also known as noncirrhotic portal fibrosis. The most specific finding is tapering of the third and higher order portal vein branches due to periportal fibrosis, a picture that has been termed obliterative port-venopathy.
IPH presents with all the signs and symptoms of portal hypertension of any other cause. However, presentation is heavily skewed towards variceal bleeding and splenomegaly at the expense of ascites, hepatic encephalopathy, or other complications, which are distinctly rare. There is a reported female preponderance of 2:1 to 3:1 with an average age at presentation of 45 to 50 years. The vast majority of patients will suffer at least one episode of variceal bleeding from gastroesophageal varices (90%). Splenomegaly is also a very common initial complaint (> 70%).
As mentioned above, IPH is a diagnosis of exclusion. In the presence of clear signs of portal hypertension and when clinical history, physical examination, imaging and laboratory studies, and biopsy rule out other causes, only then one can diagnose IPH. Biopsy invariably shows concentric fibrosis around the small portal vein branches without cirrhosis. Some authors report preferential involvement of the left lobe with associated right lobe atrophy. Liver-associated laboratory tests are normal or near normal and serve only to exclude other causes of portal hypertension. Anemia and thrombocytopenia are also common findings in IPH, the latter likely due to the significant splenomegaly that characterizes the disease.
It is crucial to properly diagnose IPH from a prognostic and management point of view. With proper management of variceal bleeding and splenomegaly and its associated thrombocytopenia, life expectancy is no different from healthy individuals. Its course is stable over many years and not associated with cirrhosis or hepatic neoplasms. There is a high initial success rate for the management of bleeding varices whether banding or sclerotherapy is used, which approaches 90 to 95%. Rebleeds are uncommon (3%) but are managed equally successfully. Emergent portosystemic shunts are rarely required (< 10% of acute bleeds), with TIPS having proven to be an effective approach. Splenomegaly can be also treated effectively with percutaneous intra-arterial embolization of a portion of the spleen (initially 25 to 50% with reevaluation).
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