The Clinical Outcome of Renal Transplantation in Cloacal Exstrophy: A Case Report

Purpose: Cloacal exstrophy is a primary abdominal wall defect associated with genitourinary, gastrointestinal, musculoskeletal, and neurological system defects. Scarce research is available regarding anatomic considerations, surgical techniques, and outcomes regarding renal transplantation in this patient population. Herein, we discuss a case of renal transplantation in a patient with cloacal exstrophy. We aim to provide awareness of surgical techniques, endocrine changes, and outcomes for transplant physicians when presented with this patient population. Materials and Methods: As a child, the patient underwent penectomy and bilateral orchiectomy. Subsequently, cloacal exstrophy repair, pelvic sagittal osteotomy, colostomy, vaginoplasty, bilateral ureteral implantation (unknown tunneled or nontunneling), and appendicovesicostomy creation were performed. However, the patient developed end-stage renal disease secondary to hypertensive nephrosclerosis and recurrent urinary tract infections. She made 1600 mL urine per day, catheterizing 4 times daily using Mitrofanoff, and underwent hemodialysis 3 times weekly for 10 months before renal transplant. Results: The patient underwent deceased donor renal transplantation to right iliac fossa. Owing to significant adhesions, peritoneum was entered, and a peritoneal window was created. Mitrofanoff was noted to be more superficial and superior than the anatomic location of the bladder. For ureteroneocystotomy, dome of the bladder was successfully encountered without complication or involvement of native ureteral reimplants. She had an uncomplicated postoperative course with immediate graft function. At 18-month follow-up, the patient continued to make the same urine output with creatinine 1.1, glomerular filtration rate 63.9, and urine protein-creatinine ratio 0.26 mg/g. She completed immunoprophylaxis 6 months after transplant and has not had recurrent urinary tract infections or pyelonephritis. Postoperative endocrine workup showed low testosterone and luteinizing hormone but elevated follicle stimulating hormone. Conclusion: Patients with cloacal exstrophy can have successful renal transplant outcomes. This provides another avenue of evidence regarding surgical techniques and long-term kidney outcomes in this patient population.