Outcome of Extrahepatic Portal Vein Obstruction in Children
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- Category: Vol. 77, December 2009
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Outcome of Extrahepatic Portal Vein Obstruction in Children,MONA FAHMY, MANAL EL-HAWARY, AZZA MOSTAFA and NEHAL EL-KOOFY
Abstract
Background: Extrahepatic portal vein obstruction (EHP-VO) developing due to thrombotic occlusion of the portal vein in children is generally a benign disease. The aim of this retrospective study was to describe clinical presentation, risk factors, complications and treatment in children with EHPVO presented to the Pediatric Hepatology Unit, Cairo University Children’s Hospital, Egypt.
Materials and Methods: The medical records of 145 children (89 male, median age 10 years, range 7 months -18 years) presenting with EHPVO between 1988 and 2008 were reviewed retrospectively.
Results: Clinical presentation included bleeding in 59% and splenomegaly in 100%. Prolonged prothrombin time was seen in 26% and elevated liver enzymes in 6%. On first endoscopy, oesophageal varices were present in 131 (90%) patients; of those subjects, 101 (77%) received sclerotherapy, 7 (5.3%) had band ligation and 14 (10.6%) received both. Thrombophilia investigation was performed in 40 patients revealed: factor V Leiden mutation in 12, protein C deficiency in 11, factor II mutation in 6 and antithrombin III deficiency in 1 patient. Liver biopsies were performed in 6 patients and revealed mixed portal cirrhosis in two and chronic hepatitis with minimal fibrosis in four patients.
Conclusion: The aetiology of EHPVO in the majority of patients remains unknown. The overall prognosis is good. Sclerotherapy and banding are effective treatments for bleeding varices with good long term outcome. Prophylactic beta-blocker decreased the risk of esophageal bleeding.