The Roux-en-Y procedure in congenital hepato-biliary disorders.

Background: The use of the Roux-en-Y procedure is limited in paediatric surgery practice, and is performed mainly in congenital hepatobiliary disorders either as an initial or permanent treatment. In this 18-year retrospective study, we present our experience of the Roux-en-Y procedure in childhood cases of biliary atresia (BA) and congenital choledochal cyst (CCC).

Methods: Twenty-eight children (18 females and 10 males; age 25 days-12 years) with hepatobiliary disorders were treated in our clinics between 1986-2004. Twenty patients suffered from BA (11 females, 9 males) and eight from CCC (seven females, one male). The surgical approach in the patients with BA (mean age 2.1 months) was Roux-en-Y hepatic portoenterostomy (Kasai procedure) and in the patients with CCC (mean age 7.2 years) was cyst excision with Roux-en-Y hepaticojejunostomy. The mean follow up period was 9.3 years.

Results: The children with BA developed the follow postoperative complications: 12 cholangitis, 6 portal hypertension and 5 hepatic cirrhosis. Among the children with CCC, two presented post-operative cholangitis, which was treated conservatively, and one developed anastomotic stricture and underwent reoperative reconstruction. At the end of the follow-up period among the children with BA 6 had died, 3 had undergone liver transplantation, and 5 were on a waiting list for transplantation. All children with CCC were alive without sequelae.

Conclusions: Roux-en-Y in BA, with timely diagnosis, is preferred as an initial procedure, followed by liver transplantation in cases with no bile drainage and is the only possible reconstruction in cases of CCC after excision of the biliary cyst.