Long-term outcomes of kidney replacement therapy in Australians with prune belly syndrome

Aim

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.

Conclusion

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).