Deceased-donor kidney transplantation in prune belly syndrome

Abstract

Objectives: To present the long-term outcome of deceased-donor kidney transplantation in prune belly syndrome (PBS). All the PBS transplant patients had undergone extensive pretransplant urologic tract preparation. We also compared the patient and graft survival in an age-matched control group who underwent transplantation for end-stage renal disease due to a nonposterior urethral valve in our urology department.

Methods: From 1988 to 2003, 11 kidneys were transplanted in 8 male patients with PBS. The mean age was 13.5 years (range 4 to 32). Patient and graft survival were assessed and compared with that of a group of 103 deceased-donor kidney transplantations performed in 86 age-matched controls who underwent transplantation because of end-stage renal disease due a nonposterior urethral valve (mean age 13.9 years, range 1.7 to 20).

Results: In the PBS group, a total of 23 pretransplant urologic procedures were performed. One operation was performed in 1 patient, two in 2, three in 2, and four in 3 patients. In the PBS group, after transplantation, adequate bladder function was maintained by intermittent catheterization in 4 patients and timed voiding in 3; 1 patient had undergone pretransplant ileal conduit formation. Up to 10 years after transplantation, we found no difference in patient and graft survival between the two groups (P = 0.466 and P = 0.838, respectively).

Conclusions: Aggressive pretransplant urologic tract preparation and keeping the postvoid residual urine volume to a minimum are needed in patients with PBS. With such management, the outcome of kidney transplantation in our patients with PBS was not different from that of other patients who underwent transplantation for other causes of end-stage renal disease in our department.