Introduction:
Prune belly syndrome (PBS) presents with both renal dysplasia and urinary tract abnormalities. When performing kidney transplantation in PBS patients with kidney failure, extensive pretransplant urinary tract preparation may be necessary.
Presentation of case:
We report the case of a 36-year-old man with PBS who underwent living-related kidney transplantation with urinary diversion using the Mitrofanoff principle. The patient had a bilateral loop ureterostomy for a urethral obstruction. Cystourethrography before the operation showed tortuous and dilated ureters with vesicoureteral reflux (VUR), and complete occlusion of the urethra. Before transplantation, we created a catheterizable urinary conduit with the patient's own malformed left ureter in accordance with the Mitrofanoff principle. The folding procedure was selected for ureteroplasty to preserve good blood supply. Extravesical detrusorrhaphy was performed as an antireflux procedure. V-quadrilateral-Z ureterostomy for catheterization was performed. Then, we performed living-related kidney transplantation from the patient's mother. Postoperative cystourethrography did not show left VUR. The patient performed clean intermittent self-catheterization without complications, and had good graft function.
Discussion:
The appendix and ileum are currently the most commonly used options for urinary conduits based on the Mitrofanoff principle. However, the patient had complications of diarrhoea and constipation, so we used the patient's own malformed ureter. We performed a folding procedure to avoid ureteral stenosis and VUR, and used the V-quadrilateral-Z flap technique to avoid stoma stenosis CONCLUSION: When performing kidney transplantation in patients with PBS, urinary tract anomalies should be thoroughly evaluated. Our surgical procedure will help to reduce complications after kidney transplantation.