Surgical Portosystemic Shunts and the Rex Bypass in Children: a Single-centre Experience

Objectives: This study aimed to illustrate the indications for, and types and outcomes of surgical portosystemic shunt (PSS) and/or Rex bypass in a single centre.

Methods: Data were collected from children with a PSS and/or Rex bypass between 1992 and 2006 at Mount Sinai Medical Center, New York.

Results: Median age at surgery was 10.7 years (range 0.3-22.0 years). Indications included: (i) refractory gastrointestinal bleeding in portal hypertension associated with (a) compensated cirrhosis (n= 12), (b) portal vein thrombosis (n= 10), (c) hepatoportal sclerosis (n= 3); (ii) refractory ascites secondary to Budd-Chiari syndrome (n= 3), and (iii) familial hypercholesterolaemia (n= 4). There were 20 distal splenorenal, four portacaval, three Rex bypass, two mesocaval, two mesoatrial and one proximal splenorenal shunts. At the last follow-up (median 2.9 years, range 0.1-14.1 years), one shunt (Rex bypass) was thrombosed. Two patients had died and two had required a liver transplant. These had a patent shunt at last imaging prior to death or transplant.

Conclusions: Portosystemic shunts and Rex bypass have been used to manage portal hypertension with excellent outcomes. In selected children with compensated liver disease, PSS may act as a bridge to liver transplantation or represent an attractive alternative.