RESUMEN
BACKGROUND: Biliary atresia (BA) is the most common indication for pediatric liver trans plantation, although portoenterostomy is usually performed first. However, due to the high failure rate of portoenterostomy, liver transplantation has been advocated as the primary procedure for patients with BA. It is still unclear if a previous portoenterostomy has a negative impact on liver transplantation outcomes. AIM: To investigate the effect of prior portoenterostomy in infants un dergoing liver transplantation for BA. METHODS: This was a retrospective cohort study of 42 pediatric patients with BA who underwent primary liver transplantation from 2013 to 2023 at a single tertiary center in Brazil. Patients with BA were divided into two groups: Those under going primary liver transplantation without portoenterostomy and those undergoing liver transplantation with prior portoenterostomy. Continuous variables were compared using the Student's t-test or the Kruskal-Wallis test, and categorical variables were compared using the χ2 or Fisher's exact test, as appropriate. Multivariable Cox regression analysis was performed to determine risk factors for portal vein thrombosis. Patient and graft survival analyses were conducted with the Kaplan-Meier product-limit estimator, and patient subgroups were compared using the two-sided log-rank test. RESULTS: Forty-two patients were included in the study (25 [60%] girls), 23 undergoing liver transplantation without prior portoenterostomy, and 19 undergoing liver transplantation with prior portoenterostomy. Patients with prior portoenterostomy were older (12 vs 8 months; P = 0.02) at the time of liver transplantation and had lower Pediatric End-Stage Liver Disease scores (13.2 vs 21.4; P = 0.01). The majority of the patients (35/42, 83%) underwent living-donor liver transplantation. The group of patients without prior portoenterostomy appeared to have a higher incidence of portal vein thrombosis (39 vs 11%), but this result did not reach statistical significance. Prior portoenterostomy was not a protective factor against portal vein thrombosis in the multivariable analysis after adjusting for age at liver transplantation, graft-to-recipient weight ratio, and use of vascular grafts. Finally, the groups did not significantly differ in terms of post-transplant survival. CONCLUSION: In our study, prior portoenterostomy did not significantly affect the outcomes of liver transplantation.
RESUMEN
eHAT is one of the most dreaded post-LT complication. Treatment approaches include retransplantation, revascularization, or observation. Systemic thrombolytic therapy is used in pediatric patients with thromboembolic events. However, there is no previous study reporting on the use of systemic r-tPA to treat eHAT. The treatment strategies used in patients with eHAT are described, focusing on two children who failed SR and were treated with systemic heparinization plus systemic r-tPA infusion. r-tPA-RP consists of intravenous systemic infusion at a dose of 0.3 mg/kg/h during 6 hours, for 5 days. First case (3-year) was transplanted with a whole liver, and second case (6-year) received a LLS from a living donor. HAT was diagnosed by doppler US and confirmed by angioCT scan in both patients in the first day after LT. They underwent SR and were clinically stable. Re-thrombosis occurred in both patients the day after, and r-TPA-RP was started-one patient required two r-TPA-RP for HAT recurrence. They presented minor bleeding, without repercussion. Hepatic artery recanalized after 10 and 3 days in the first and second patient, respectively. Retransplant was avoided, and one developed biliary strictures, successfully managed in the follow-up. r-TPA-RP avoided retransplantation after eHAT in these cases. To our knowledge, this is the first report of the use of systemic r-TPA to treat eHAT in children. This strategy may compose an algorithm to treat eHAT that failed SR in stable patients.
