Medium and Long-Term Clinical Outcomes of Kidney Transplantation in Patients With Prune Belly Syndrome: A Single-Centre Experience

Abstract

To present the medium and long-term outcomes of kidney transplantation performed in patients with Prune Belly Syndrome (PBS) which is one of the congenital anomalies of the kidney and urinary tract (CAKUT). Thirteen kidney transplantations were performed in nine patients with PBS from January 1994 to December 2020. Demographics, surgical features, graft and patient survival rates were analyzed. Survival outcomes was compared with 106 age- and donor-type matched controls who underwent transplantation because of nonurological causes. The median ages of PBS patients at the time of first and second transplantation were 19 and 34 years old, respectively. One patient had postoperative Clavien grade 3a complication. Eight of 13 transplants are still functional at the last follow-up. Eight patients underwent pretransplant urological operations in preparation for kidney transplantation, while 4 patients underwent urological operations in the post-transplant period. Graft survival for PBS transplants at 5, 10, and 15 years were 90.9%, 57.7%, and 38.5%, respectively. Graft survival for control cohort at 5, 10, and 15 years were 89.9%, 80%, and 74%, respectively. The patient and graft survival rates were similar between PBS and control groups (P=.449, P=.134, respectively). This single-center study showed that the medium-term graft survival rates in patients with PBS are encouraging and comparable to non-CAKUT transplant patients, however long-term graft survival rates were found to be proportionally inferior. To help determine the reasons for long-term transplant kidney function deterioration and how to improve them we suggest that multicenter international collaboration will be needed.