Broniszczak Dorota, Apanasiewicz Artur, Czubkowski Piotr, Kaliciński Piotr, Ismail Hor, Ostoja-Chyzynska Anna, Markiewicz-Kijewska Malgorzata
Department of Pediatric Surgery and Organ Transplantation, Children's Memorial Health Institute, Warsaw, Poland.
Ann Transplant. 2011 Jan-Mar;16(1):14-7.
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.
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.
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.
由于移植手术中的技术问题，患有胆道闭锁和多脾综合征（BA-PS）的儿童一直被视为肝移植的高风险受者。我们报告了本综合征患儿肝移植的单中心经验。
材料/方法：2000年至2010年期间，358名儿童接受了401例肝移植，其中6例BA-PS患儿接受了活体（5例）或尸体（1例）供体肝移植。患者表现出各种畸形：肝后下腔静脉缺如（3例）、肠旋转不良（3例）、十二指肠前门静脉（1例）、肝动脉异常（3例）、心脏异常（2例）和内脏反位（1例）。移植手术在患儿8个月至11岁时进行。
手术过程中没有严重的技术问题，在任何情况下我们都无需使用血管导管进行移植物再血管化。所有患者在移植后14至123个月（平均75个月）的随访中均存活。然而，我们观察到这些患者门静脉血栓形成和胆道并发症的发生率增加，这并未影响患者和移植物的存活。1例因PTLD停用免疫抑制后发生慢性排斥反应导致移植物功能衰竭的患儿接受了再次移植。
在经验丰富的儿科肝移植中心，BA-PS患儿的肝移植结果与其他适应证及非综合征性BA的结果一样好。尽管这些患儿在尸体或活体亲属供体移植过程中没有严重的技术问题，但术后密切观察可能的血管并发症应成为常规操作。
