Non-transplant Management of Portal Hypertension in Children

Chapter

Abstract

Liver transplantation changed the care of children with portal hypertension. The causes of pediatric portal hypertension can be both cirrhotic and noncirrhotic and were initially thought to be primarily attributed to extrahepatic portal venous obstruction. The experience with the broad clinical spectrum of pediatric diseases producing PH has evolved according to the management strategies used to treat the complications of this disease and has impacted our understanding of its associated pathophysiology. Portal hypertension is an abnormally high blood pressure in this system (>10 mmHg) and can be a consequence to increased resistance and/or increase in blood flow. Diseases causing PH in children can be classified into two principle groups, those with associated significant liver disease and those associated with no significant liver disease. The management of PH in children has, until recently, focused on the initial control of established bleeding; longer-term strategies are guided by the determination of the presence or absence of liver disease. It is critically important to assess the nature of disease resulting in PH; the goal of managing the bleeding complication hinges on resuscitation and stabilization, with endoscopic control remaining the cornerstone of therapy.

Keywords

Liver transplantation Portal hypertension Extrahepatic portal venous obstruction Portosystemic shunt Hepatic vein pressure Cirrhosis Biliary atresia Hepatocellular carcinoma Constrictive pericarditis EHPVO Budd Chiari Hypersplenism Hepatopulmonary syndrome Portopulmonary Sengstaken Blakemore tube Splenorenal shunt Rex shunt 

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Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Division of Transplant Surgery, Department of SurgeryUniversity of Washington, Director of Transplant Services, Seattle Children’s HospitalSeattleUSA

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