Abstract

Background: Children with biliary atresia (BA) and polysplenia syndrome (PS)
have always been considered as a high risk liver transplant recipients due to
technical problems during transplant surgery. We report a single-centre experience with the liver transplantation in children with this syndrome.
Material/Methods: Between 2000 and 2008 330 liver transplants were performed in our centre in children, among them there were five with end stage liver insufficiency, with BA and PS, following the Kasai procedure. They underwent liver transplantation from living (4 patients) and deceased donors
(1 patient). All patients demonstrated additional malformations like: absence  of retrohepatic vena cava (1), intestinal malrotation (2), preduodenal portal vein (1), hepatic artery (2) and cardiac anomalies (2). No situs inversus occurred. Transplantations were performed at the age ranging from 8 months to 11 years. We did not meet serious technical problems during operation, as well as never had to use vascular conduits for graft revascularization.
Results: All patents are alive and well with follow up between 9 and 105 months after transplantation, although one is with persistent hepatitis C and  one has symptoms of portal hypertension after portal thrombosis which occurred during PTLD treatment one year after transplantation.
Conclusions: Results of liver transplantation in children with BA and PS syndrome are as good as for other indications for non-syndromic BA in experienced paediatric liver transplant centre.

Keywords: Liver Transplantation