Liver transplantation in children with biliary atresia and polysplenia syndrome.

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.

[Treatment of lymphangioma with OK432 in children]. / Leczenie naczyniaków chlonnych u dzieci preparatem OK-432.

From 1998 to 2003 6 children with lymphangioma of cervico-facial region were treated with OK-432 in Polish Mother Health Institute. Three of them had nonresectable leasion and two required tracheostomy during first months of life. Each patient received from 1 to 10 sessions of obliteration. In total 23 sessions were performed. The youngest patient treated with OK-432 was 2 weeks and the oldest 2 years old. The response to treatment was very good in 4, good in 1 and fairly good in 1 case. In three patients criodestruction was applied as an additional treatment. In two children residual lesions were subsequently removed surgically. There were no serious complications. After obliteration sessions only 2-3 day temperature increase and local inflammation occurred. We consider obliteration with OK-432 as a treatment of choice in lymphangiomas in children.