A Novel Combined Surgical and Interventional Radiology Vascular Reconstruction, With Stenting of Meso-Rex Bypass, to Successfully Manage Recurrent Portal Venous Flow Obstruction Post Segmental Liver Transplant in a Pediatric Patient.

BACKGROUND

Portal vein complications can be difficult to manage in the context of challenging anatomy and porto-systemic collaterals.

METHODS

This case illustrates a child with biliary atresia and associated laterality (situs inversus, polysplenia) features, who underwent segmental liver transplantation with portal venous conduit at 4 years of age for end-stage liver disease due to failed Kasai portoenterostomy. He subsequently developed portal venous conduit thrombosis requiring early surgical and interventional procedures. This was thought to be due to angulation of the conduit versus venous steal through multiple large portosystemic collaterals. Despite repeat angioplasty and embolization of portosystemic collaterals, he developed recurrent portal hypertension with upper gastrointestinal bleeding and persistent portal stenosis. Graft function remained well preserved. A meso-Rex bypass was therefore created 2-years 9-months post-transplant. Initial flow was excellent, but follow-up imaging revealed diminished flow and stricture at the left portal vein anastomosis. This was again thought to be secondary to steal through portosystemic collaterals and was refractory to angioplasty. Thus, a combined surgical-interventional radiology revision of the Meso-Rex bypass with laparotomy and liver mobilization, placement of an uncovered stent, portosystemic collaterals surgical ligation, and splenectomy of the main right-sided spleen was performed.

RESULTS

There were no complications and, 2 years 8 months post procedure, there is excellent shunt patency and graft function with no signs of portal hypertension.

CONCLUSION

This case report highlights the critical utility of a multidisciplinary approach and innovative solutions by an experienced program in the management of complex surgical complications following pediatric liver transplantation.