[Natural history of portal cavernoma without XXXX . A single centre retrospective study of 32  cases].

@entity1 @entity556 @entity66 @entity5 @entity75 @entity843 @entity409 @entity3181 @entity3562 @entity2395
PURPOSE: Portal cavernoma follows a chronic occlusion of the portal vein. The long-term consequences of portal cavernoma are not well known. The objective of this study was to report the aetiology of the portal cavernoma and its natural course after excluding @entity409 causes. METHODOLOGY: A single centre retrospective study based on the data collected from the radiology department of the Clermont-Ferrand hospital was conducted from 2000 to 2011. All the @entity1 for whom an imagery found a portal cavernoma have been looked for excluding the @entity1 having a @entity409 whatever the aetiology and the @entity3181 . RESULTS: Thirty-two cases (18  @entity1 and 14  @entity1 ) were selected. The mean age at diagnosis was 54.2  years and the mean follow-up period was 5.4  years. The discovery of a portal cavernoma was incidental for 8  cases. An aetiology was found for 24  cases: it was an haematological aetiology in 15  cases (10  myeloproliferative syndromes, 2  antiphospholid syndromes, 1  @entity2395 major, 1  @entity3562 , 1  @entity556 gene mutation), a general aetiology in 2  cases (1  @entity66 , 1  pancreatic @entity5 ), and a local inflammation in 7  cases. A dysmorphic aspect of the liver was noticed on medical imaging for 11 out of the 32  cases. A liver biopsy was performed in 4  @entity1 and was normal for all of them. Sixteen @entity1 developed oesophageal varices, 4  @entity1 developed @entity843 , 3  developed asymptomatic biliary compression by the portal cavernoma, and the @entity1 who had been followed for the longest time (15  years) developed an @entity75 . CONCLUSION: In addition to its underlying etiology, the prognosis of portal is mainly related to the occurrence of oesophageal varices that may develop during the follow-up of the @entity1 .
