Cavernous Transformation of the Portal Vein in a 26-Month Old Boy Treated by Transjugular Intrahepatic Portosystemic Shunt: A Case Report

Approaches to the management of portal hypertension and variceal hemorrhage in pediatrics remain controversial, in large part because they are not well informed by rigorous clinical studies. Fundamental biological and clinical differences preclude automatic application of approaches used for adults to children. On April 11-12, 2015, experts in the field convened at the first Baveno Pediatric Satellite Meeting to discuss and explore current available evidence regarding indications for MesoRex bypass (MRB) in extrahepatic portal vein obstruction and the role of primary prophylaxis of variceal hemorrhage in children. Consensus was reached regarding MRB. The vast majority of children with extrahepatic portal vein obstruction will experience complications that can be prevented by successful MRB surgery. Therefore, children with extrahepatic portal vein obstruction should be offered MRB for primary and secondary prophylaxis of variceal bleeding and other complications, if appropriate surgical expertise is available, if preoperative and intraoperative evaluation demonstrates favorable anatomy, and if appropriate multidisciplinary care is available for postoperative evaluation and management of shunt thrombosis or stenosis. In contrast, consensus was not achieved regarding primary prophylaxis of varices. Although variceal hemorrhage is a concerning complication of portal hypertension in children, the first bleed appears to be only rarely fatal and the associated morbidity has not been well characterized.

Transjugular intrahepatic portosystemic shunt (TIPS) is a valuable tool in managing complications of severe portal hypertension (PH) in adults. In children, TIPS is regarded as a temporary and technically demanding procedure. We report the first paediatric series of TIPS and review its feasibility and efficacy in children. Children with severe PH that is unresponsive to nonselective β-blockers and endoscopic treatment were considered candidates for TIPS placement with an expanded polytetrafluoroethylene-covered Viatorr stent graft. Indications were cirrhotic PH, noncirrhotic PH, and portal vein thrombosis, in both native and transplanted livers. Thirteen children were selected for TIPS between 2005 and 2010. The indications were bleeding in 8 (61.5%) and ascites in 5 (38.5%). TIPS was successfully placed in 11 children, median age 9.8 years (range 2.2-18) and median weight 30 kg (11.5-96). Two patients had a portal cavernoma and 3 had received split liver transplantation. Following TIPS, the portosystemic gradient decreased to 10 mmHg (5-15 mmHg) (P < 0.00001); PH complications resolved in 10 of 11 patients (91%); no patient developed clinical encephalopathy; and 3 patients required a TIPS revision. All of the shunts were patent at last follow-up (20.4 months, range 0.2-67) in 7 or liver transplantation (6 months, 1.5-33) in 4. Our results suggest that TIPS is feasible and effective in children with ascites or gastrointestinal bleeding unresponsive to medical and endoscopic treatment. TIPS should become part of the armamentarium that is used to manage PH complications in children, both in native livers and in transplanted grafts, as a bridge to transplantation and for long-term management.

To evaluate the feasibility and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) for extrahepatic portal venous obstruction with recurrent variceal bleeding in children.