Abstract
Background Extrahepatic portal vein thrombosis (EHPVT) is an important cause of portal hypertension in children. Rex shunt has been used successfully to treat these patients. Methods We report our experience in 19 infants and children (5 months to 14 years) with HPVT eligible for a mesenteric-portal surgical shunt with left internal jugular vein autograft. Eight children had idiopathic EHPVT, nine had post–umbilical catheterization EHPVT, one had portal vein agenesis, and one had posttransplant EHPVT. Results It was possible to perform the Rex shunt in all patients except for 8 of 9 cases in the post–umbilical catheterization EHPVT group. A Warren procedure was performed in 4 of those patients and a proximal splenorenal shunt in 1. Current follow-up ranges from 3 to 26 months. Shunt thrombosis occurred in one patient with portal vein agenesis and associated cardiac anomaly. Portal hypertension has significantly improved after surgery. None of our patients have experienced new bleeding episodes until now. Conclusions The Rex shunt should be considered in the treatment of children with idiopathic EHPVT experiencing repeated gastrointestinal bleeding episodes refractory to endoscopic treatment. Nevertheless, the role of this operation for children with post–umbilical catheterization EHPVT is yet to be clearly evaluated.
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