Kidney Transplant Outcomes in Prune Belly Syndrome: A Retrospective Analysis of UNOS Data

To compare death-censored graft survival and patient survival after kidney transplantation between recipients with prune belly syndrome (PBS), a rare congenital disorder with unique anatomical challenges, and propensity score-matched controls using national registry data.

Using the UNOS STAR database, we conducted a retrospective matched cohort study of first-time, single-organ kidney transplants from January 2010 to September 2024. PBS recipients (n=269) were matched 1:4 to controls (n=1,076) using propensity scores based on recipient, donor, and transplant-era characteristics. Survival was analyzed using Kaplan-Meier curves, stratified log-rank tests, and Cox models stratified by matched set.

Matching achieved standardized mean differences <0.1 for all covariates (median age 10 years; 97.8% male in the PBS group). Death-censored graft survival at 5 and 10 years was 92.6% and 69.8% in PBS recipients versus 91.1% and 75.4% in controls (hazard ratio [HR] 1.08, 95% CI 0.69-1.69; p=0.73). Patient survival at 10 years was 94.3% versus 92.8% (HR 1.38, 95% CI 0.65-2.96; p=0.40). Findings were consistent across age, era, and donor-type subgroups, although confidence intervals were wide.

No significant differences in graft or patient survival were detected between PBS recipients and matched controls, supporting evaluation of PBS recipients for kidney transplantation on the same basis as other candidates. Confidence intervals do not exclude a modest difference, particularly at longer follow-up.