Portal hypertension is a source of significant morbidity and mortality among children, with unique manifestations in the pediatric age groups. Different underlying etiologies, primarily extrahepatic portal vein thrombosis and biliary atresia, lead to different approaches in management. The greater availability of organ transplants for children with liver failure means more pediatric patients with severe underlying liver disease will undergo liver transplantation, altering management of associated portal hypertension. For patients with persistent portal hypertension, pharmacotherapy and endoscopic surveillance/treatment are specialized, due to the altered physiology of children combined with a lack of primary data directing effective management. In comparison to adults, surgical shunt placement is usually pursued in children due to the age-related benefits of durable portal blood flow normalization and unique anatomical considerations. Endovascular therapies, including transhepatic portal systemic shunt placement (TIPS), portal vein thrombolysis and recanalization, balloon-occluded retrograde transvenous obliteration (BRTO) of gastric varices, also play a role in management; however, data is lacking, and the smaller size of the patient can make endovascular treatment more technically challenging.

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Pediatric Portal Hypertension

  • Wes Joseph Albright,
  • Joseph J. Gemmete

摘要

Portal hypertension is a source of significant morbidity and mortality among children, with unique manifestations in the pediatric age groups. Different underlying etiologies, primarily extrahepatic portal vein thrombosis and biliary atresia, lead to different approaches in management. The greater availability of organ transplants for children with liver failure means more pediatric patients with severe underlying liver disease will undergo liver transplantation, altering management of associated portal hypertension. For patients with persistent portal hypertension, pharmacotherapy and endoscopic surveillance/treatment are specialized, due to the altered physiology of children combined with a lack of primary data directing effective management. In comparison to adults, surgical shunt placement is usually pursued in children due to the age-related benefits of durable portal blood flow normalization and unique anatomical considerations. Endovascular therapies, including transhepatic portal systemic shunt placement (TIPS), portal vein thrombolysis and recanalization, balloon-occluded retrograde transvenous obliteration (BRTO) of gastric varices, also play a role in management; however, data is lacking, and the smaller size of the patient can make endovascular treatment more technically challenging.