title: Liver transplantation in children with biliary atresia and polysplenia syndrome.
abstract: BACKGROUND
  Children with biliary atresia and polysplenia syndrome (BA-PS) have always been considered as high risk liver transplant recipients due to technical problems during transplant surgery. We report single-center experience with liver transplantation in children with this syndrome.
  
  
  METHODS
  Between 2000 and 2010, 401 liver transplantations were performed in 358 children, including 6 patients with BA-PS, who underwent living (5 patients) or deceased (1 patient) donor liver transplantation. Patients demonstrated various malformations: absence of retrohepatic vena cava (3), intestinal malrotation (3), preduodenal portal vein (1), hepatic artery anomalies (3), cardiac anomalies (2), and situs inversus (1). Transplantations were performed at the patient age of 8 months to 11 years.
  
  
  RESULTS
  There were no serious technical problems during the operations, and we did not have to use vascular conduits for graft revascularization in any case. All patents were alive at follow-up between 14 and 123 months after transplantation (mean 75 months). We observed, however, increased incidence of PV thrombosis and biliary complications in these patients, which did not influence patient and graft survival. In 1 child with graft failure due to chronic rejection after discontinuation of immunosuppression due to PTLD, retransplantation was performed.
  
  
  CONCLUSIONS
  Results of liver transplantation in children with BA-PS are as good as for other indications and non-syndromic BA in an experienced pediatric liver transplant center. Although there were no serious technical problems during deceased or living related donor transplantation in these children, close observation for possible vascular complications should be the routine in the postoperative period.
authors: Department of Pediatric Surgery and Organ Transplantation, Children's Memorial Health Institute, Warsaw, Poland. dorbro1@poczta.onet.pl
authors: Dorota
authors: D
authors: Broniszczak
authors: Artur
authors: A
authors: Apanasiewicz
authors: Piotr
authors: P
authors: Czubkowski
authors: Piotr
authors: P
authors: Kaliciński
authors: Hor
authors: H
authors: Ismail
authors: Anna
authors: A
authors: Ostoja-Chyzynska
authors: Malgorzata
authors: M
authors: Markiewicz-Kijewska
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery; D001656:Biliary Atresia / Q000601:surgery*; D002648:Child; D005260:Female; D006801:Humans; D007223:Infant; D008099:Liver / Q000002:abnormalities / Q000601:surgery; D016031:Liver Transplantation / Q000009:adverse effects; D008297:Male; D011169:Portal Vein; D012086:Reoperation; D012857:Situs Inversus / Q000601:surgery; D013154:Spleen / Q000002:abnormalities* / Q000601:surgery*; D013577:Syndrome; D020246:Venous Thrombosis / Q000209:etiology