Approaches to the management of pediatric portal hypertension: results of an informal survey

  • Benjamin L. Shneider


Portal hypertension and its related complications are major clinical issues in pediatric hepatology. The leading cause of significant liver disease in children is biliary atresia. The biliary injury in this disorder is associated with prominent portal hypertension at disease stages where synthetic liver function is typically intact. Thus the relative contribution of complications of portal hypertension to morbidity and mortality in liver disease is greater in children than in adults. In children with biliary atresia, prominent portal hypertension is often present at the time of corrective hepatoportoenterostomy (Kasai procedure) and it progresses over relatively short periods of time1. The rate of progression of the portal hypertension is related to outcome after hepatoportoenterostomy, with more accelerated progression in children with failed procedures or with recurrent cholangitis2. Complications that are a direct result of this portal hypertension are common. Variceal hemorrhage occurs in a large percentage of children, often within the first 5 years of life2, 3, 4, 5, 6, 7. Ascites, hepatopulmonary syndrome, rectal varices/colopathy, stomal hemorrhage, and pulmonary hypertension have also been described in children with biliary atresia8, 9, 10, 11, 12, 13.


Portal Hypertension Esophageal Varix Biliary Atresia Variceal Hemorrhage Primary Prophylaxis 
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Copyright information

© Springer Science+Business Media Dordrecht 2004

Authors and Affiliations

  • Benjamin L. Shneider
    • 1
  1. 1.Division of Pediatric HepatologyMount Sinai Medical CenterNew YorkUSA

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