Objective: The aim of this study was to describe long-term outcomes of kidney replacement therapy (KRT) in Australians with prune belly syndrome in comparison to a control group of congenital kidney disease.

Methods: We identified all Australians treated with KRT between 1977 and 2021 with a diagnosis of PBS from the Australia and New Zealand Dialysis and Transplant Registry.

Results: We identified 37 males (no females) who commenced KRT at a median age of 17 years (range 1-45). At initiation of KRT treatment, 54% of patients were on haemodialysis, 30% on peritoneal dialysis and 16% received a pre-emptive kidney transplant. Forty-eight kidney transplants (35 first, 11 second and 2 third grafts) occurred, of which 48% were from deceased donors. Median age at first transplant was 21 years (range 2-47). Graft survival at 1, 5 and 10 years for first grafts was 91%, 71% and 51%, respectively (range 6 days to 36 years). Three men reported parenthood at median age 35 years. There were 10 deaths reported at a median age of 37 years (range 17-49). Reported aetiology was cardiac death (50%), malignancy (20%), dialysis cessation (10%) and uncertain cause (20%). Compared to an age and gender-matched control group of people with congenital kidney dysplasia, Australians with PBS had equivalent peritoneal dialysis technique survival, but slightly better transplant graft and overall survival.

Conclusions: Prune belly syndrome has marked variation in outcomes from KRT, but overall, these were equivalent or better than a matched control group with congenital kidney disease, including use of peritoneal dialysis (despite lack of abdominal wall musculature).