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Extrahepatic portal vein thrombosis after umbilical catheterization: is it a good choice for Rex shunt?
Nelson Elias Mendes Gibelli1, Ana Cristina Aoun Tannuri, Maria Lúcia Pinho-Apezzato
1Instituto da Criança, Hospital das Clínicas, Pediatric Surgery and Liver Transplantation Division, Department of Pediatrics, University of São Paulo Medical School, São Paulo, Brazil. nelson.gibelli@icr.usp.br
Insights
The Rex shunt is effective for treating pediatric extrahepatic portal vein thrombosis (EHPVT) causing portal hypertension, especially in idiopathic cases. Its role in post-catheterization EHPVT requires further evaluation.
Area of Science:
- Pediatric surgery
- Hepatology
- Vascular surgery
Background:
- Extrahepatic portal vein thrombosis (EHPVT) is a significant cause of portal hypertension in children.
- The Rex shunt procedure has shown success in managing these pediatric patients.
Purpose of the Study:
- To evaluate the efficacy and outcomes of the Rex shunt in pediatric patients with EHPVT.
- To assess the suitability of the Rex shunt for different etiologies of EHPVT.
Main Methods:
- A retrospective review of 19 infants and children (5 months to 14 years) with EHPVT who underwent a mesenteric-portal surgical shunt using a left internal jugular vein autograft.
- Patients were categorized by EHPVT etiology: idiopathic, post-umbilical catheterization, portal vein agenesis, and post-transplant.
Main Results:
- Rex shunt creation was successful in most cases, except for 8 out of 9 patients with post-umbilical catheterization EHPVT, where alternative shunts (Warren procedure, proximal splenorenal shunt) were performed.
- One instance of shunt thrombosis occurred in a patient with portal vein agenesis and a cardiac anomaly.
- Significant improvement in portal hypertension was observed post-surgery, with no new gastrointestinal bleeding episodes reported during the 3-26 month follow-up.
Conclusions:
- The Rex shunt is a recommended treatment for children with idiopathic EHPVT and refractory gastrointestinal bleeding.
- The effectiveness of the Rex shunt for EHPVT secondary to umbilical catheterization needs further investigation.
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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