P0961 - Early percutaneous intervention in pediatric chronic extrahepatic portal vein occlusion is feasible and safe

Background/Purpose: The Baveno VI consensus workshop recommended that children with chronic extrahepatic portal vein (EHPV) occlusion and normal liver function should be considered for early meso-Rex bypass (MRB). Percutaneous EHPV recanaliztion has primarily been reported in adults with underlying liver disease. We report our experience utilizing a multidisciplinary treatment algorithm for treating EHPV occlusion with percutaneous balloon angioplasty followed by surgical shunt if necessary.

Methods: This is an IRB-approved retrospective review of seven patients at a single institution who presented with symptomatic chronic EHPV occlusion between 2014 and 2019. All patients were treated via the algorithm (figure), which included a multidisciplinary evaluation and an endovascular attempt at anatomy delineation and percutaneous EHPV recanalization prior to any attempted surgical intervention. Primary end point was portal vein patency.

Results: We evaluated seven patients (5 male), ages ranged from one week to twelve years (median 1.08 years). History of an umbilical vein catheter was present in five patients. EHPV’s were successfully recanalized in 71% (5/7), and 80% (4/5) have remained patent with repeat angioplasties. The other patient required a splenorenal shunt following recurrent EHPVO after abdominal neuroblastoma excision. Shunts were performed in four patients (2 MRB and 2 splenorenal). MRB thrombosis occurred in one patient, but successful EHPV recanalization was achieved during an elective interventional procedure performed for MRB stenosis. Splenomegaly and platelet counts improved in 60% of recanalized patients. There were no episodes of recurrent gastrointestinal bleeding. Complications included one case of prolonged intubation due to respiratory superinfection and one blood transfusion. Postoperative bleeding in one surgical patient necessitated reoperation.

Conclusion: Early treatment as recommended by the Baveno VI guidelines can be achieved with percutaneous intervention. EHPV patency can be successfully maintained with interventional procedures with minimal complications, with surgery reserved for patients that fail percutaneous recanalization or have unfavorable anatomy.