Living-Donor Kidney Transplant in a Patient With Type B Mayer-Rokitansky-Küster-Hauser Syndrome, Reconstructed Vagina, and Abnormal Pelvic Vessels: A Case Report.

ABSTRACT

Mayer-Rokitansky-Küster-Hauser syndrome is a rare disorder consisting of vaginal aplasia and other müllerian duct abnormalities. Urinary tract malfor-mations possibly leading to renal failure are also common. For these patients, kidney transplant remains the best option. However, aberrant anatomy and scarring from previous operations may actually preclude successful implantation of the graft. In this setting, careful pretransplant evaluation with high-resolution imaging studies and multidisciplinary planning are mandatory. We report on a patient with type B Mayer-Rokitansky-Küster-Hauser syndrome, left renal agenesis, right pelvic kidney, grade 3 cystocele, reconstructed vagina, and abnormal vasculature of the pelvis who developed end-stage renal disease due to chronic pyelonephritis. After a thorough preoperative assessment, she eventually underwent simultaneous right pelvic nephrectomy and living-donor kidney transplant. Despite the complexity of the procedure, there were no intraoperative or postoperative complications. After 1 year of follow-up, she is doing well with excellent graft function.