Management of portal vein anastomotic stenosis after pediatric liver transplantation: Evaluation of single center experience

Introduction: Late onset portal vein anastomotic stenosis (PVAS) is a frequent complication in pediatric liver transplantation. For clinical relevant PVAS percutaneous transluminal angioplasty (PTA) is the first treatment option. When PTA is not feasible or not successful to obtain portal vein patency, a surgical Meso-portal shunt (MPS) can be performed. Our aim was to evaluate the clinical and biochemical characteristics of pediatric patients with post-transplant PVAS and the results of our management of these patients. Methods: We retrospectively studied all patients aged >18 years that underwent a therapeutic intervention procedure between 2014 and 2018, either PTA or MPS after pediatric liver transplantation including living and deceased liver donors. We evaluated primary patency of the PTA and MPS and secondary patency after the complete therapeutic intervention process. Results: PVAS was diagnosed by ultrasound and/or CT-scan prior to the first intervention. At our center, 18 (11%) of 165 patients underwent a therapeutic intervention procedure for PVAS after liver transplantation. Of the patients with PVAS, 72% had biliary atresia as the primary diagnosis, 72% were transplanted under 1 year of age and 67% received a living donor graft. All PVAS patients presented with splenomegaly. Thrombocyte counts were below 150 in only 67% of PVAS patients before the first intervention. Of all PVAS patients 44% had at least one episode of a gastro-intestinal bleeding. In 12 patients endoscopy was performed and all had esophageal varices. The median post-procedural follow-up time was 1.4 years. Twelve patients underwent a primary PTA, of whom one patient underwent two PTA's and one patient three PTA's in total. 10 patients received a MPS of whom four patients had a MPS after PTA. Primary patency was 67% for the first PTA, 0% for the second and third and patency after MPS was 80%. Secondary patency was 89% for the complete therapeutic approach. Conclusion: In 11% of our cohort of pediatric transplantation patients portal vein anastomotic stenosis is a significant problem especially in recipients with biliary atresia, transplantation below 1 year of age and after living donor transplantation. Based on our results we do not support a second PTA if the first PTA fails. Our combined treatment strategy with PTA and MPS has a good clinical outcome with a secondary patency of 89%