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Liver transplantation in children with biliary atresia and polysplenia syndrome
Dorota Broniszczak1, Artur Apanasiewicz, Piotr Czubkowski
1Department of Pediatric Surgery and Organ Transplantation, Children's Memorial Health Institute, Warsaw, Poland. dorbro1@poczta.onet.pl
Insights
Liver transplantation in children with biliary atresia and polysplenia syndrome (BA-PS) is safe and effective. Despite potential complications, outcomes are comparable to other pediatric liver transplant recipients.
Area of Science:
- Pediatric Hepatology
- Transplant Surgery
- Medical Genetics
Background:
- Biliary atresia and polysplenia syndrome (BA-PS) presents unique challenges for pediatric liver transplantation due to associated congenital anomalies.
- Historically, children with BA-PS were considered high-risk recipients, necessitating careful evaluation of surgical techniques and outcomes.
Observation:
- A single-center study evaluated 6 pediatric patients with BA-PS undergoing liver transplantation between 2000 and 2010.
- Patients presented with diverse malformations including absence of retrohepatic vena cava, intestinal malrotation, and cardiac anomalies.
- Transplantations utilized living or deceased donors, with patient ages ranging from 8 months to 11 years.
Findings:
- No significant technical difficulties or need for vascular conduits were encountered during transplantation procedures.
- All patients survived the follow-up period (mean 75 months), with comparable graft survival rates to other indications.
- An increased incidence of portal vein thrombosis and biliary complications was observed but did not impact overall survival.
Implications:
- Liver transplantation in children with BA-PS yields outcomes comparable to non-syndromic biliary atresia in experienced centers.
- While technical challenges are minimal, vigilant postoperative monitoring for vascular complications is crucial in BA-PS patients.
- This study supports the feasibility and success of liver transplantation for BA-PS, improving long-term prognosis.
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.
Material/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.