Living-related kidney transplantation with catheterizable urinary conduit in prune belly syndrome: A case report

Abstract

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.

Keywords: Kidney transplantation; Mitrofanoff principle; Prune belly syndrome; Urinary diversion.

Fig. 1

Preoperative cystography. Cystography was performed via the right ureteral ureterostomy. Left vesicoureteral reflux appeared when 40-ml contrast agent was injected. The bladder capacity was 180 ml. (a) left dilated ureter, (b) bladder, (c) right ureter.

Fig. 2

Left ureteroplasty using the folding procedure. (i) (a) left ureter, (b)bladder. (ii) For smooth catheterization, intermittent sutures were placed over the 14-Fr catheter before folding the dilated ureter.

Fig. 3

Postoperative cystography. Cystography was performed via the urinary conduit. Left vesicoureteral reflux was not observed. Self-catheterization allowed bladder emptying.