Surgical portosystemic shunts and the Rex bypass in children: a single-centre experience

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.

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

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.

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.