Purpose: Variceal bleeding in children generally presents in the setting of cirrhosis but may also occur in the absence of liver disease and after successful liver transplantation. The authors reviewed their experience with portosystemic shunting in children to better define its efficacy in sclerotherapy failures, as primary therapy, and its role before and after liver transplantation.
Methods: Between 1983 and 1997, 21 children with recurrent variceal bleeding underwent portosystemic shunting at the authors' institution. Patients were divided into two groups: 13 patients in group I presented with intrinsic liver disease (cirrhosis) as a cause for portal hypertension; eight patients in group II with no liver disease had extrahepatic portal venous thrombosis, five of which occured after successful liver transplantation. The mean age was 8.6 years (range, 3 to 18). Shunt procedures were semielective, and were performed successfully in all children without any operative morbidity or mortality. Follow-up ranged from 6 months to 15 years.
Results: Shunt procedures included splenorenal (n = 15), splenocaval (n = 1), side-to-side splenorenal (n = 1), inferior mesenteric vein to renal vein (n = 1), mesocaval (n = 1), and transcutaneous intrahepatic portosystemic shunt (TIPS; n = 2). All patients in group II are alive and well with no further bleeding or occlusions. Of the 13 patients in group I, three had bleeding postshunt, and three patients went on to require liver transplantation because of worsening liver disease. One child died of liver failure with encephalopathy 4 years after shunting. There were two shunt occlusions.
Conclusions: Portosystemic shunting is effective therapy in patients with intrinsic liver disease, with salvage amenable by liver transplantation in the treatment failures. It is definitive therapy in patients with extrahepatic venous thrombosis.