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.
Key words : Cystocele, End-stage renal disease, Neovagina, Renal transplantation
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare disorder consisting of vaginal aplasia and other müllerian duct abnormalities. The incidence of the syndrome is 1:4500 female newborns.1 The molecular basis of the disease is basically unknown. However, WNT4 mutations have been detected in 2 patients with müllerian duct defects.2,3 Two variants of the disease have been described: type A and type B. Type B MRKH syndrome is often associated with urologic, ovarian, skeletal, or otologic malformations.4-7 Common urinary tract anomalies include unilateral kidney agenesis (43%), unilateral kidney agenesis plus pelvic kidney (18%), and pelvic kidney (10%).8
We herein report a patient with type B MRKH syndrome, right pelvic kidney, reconstructed vagina, grade 3 cystocele, and abnormal vasculature of the pelvis who underwent successful living-donor kidney transplant.
In 1992, a 31-year-old female patient was referred to our Low Clearance Clinic due to nycturia, trace proteinuria, and raised serum creatinine concentration. The patient had been diagnosed with type B MRKH syndrome 10 years earlier after investigations for primary amenorrhea. Associated malformations were left renal agenesis and pelvic right kidney. Her past medical history also included appendectomy plus right-sided ovariectomy, laparotomy plus adhesiolysis, creation of a neovagina by the Vecchietti procedure,9 and recurrent urinary tract infections due to vesicoureteral reflux. Aggressive medical treatment could not stop the deterioration of her renal function, and, after 23 years, she eventually developed end-stage renal disease.
Her husband came forward as a potential donor, and a thorough pretransplant assessment was promptly conducted. An angio-computed tomography scan (Figure 1) showed a right-sided pelvic kidney with a single artery originating from the anterior-lateral aspect of the aorta, close to the iliac bifurcation. The renal artery was normal in size (maximum diameter = 4.7 mm) and divided into 3 branches just before entering the renal hilum. Two renal veins were also detected, both joining the lower distal part of the inferior vena cava. The aorta and the iliac artery were heavily calcified and tortuous. The right common iliac artery was also kinked and retracted toward the midline. The bladder was prolapsed. A grade 3 cystocele was lately confirmed by a voiding cystogram. The main issues to consider before we could proceed with surgery were the need for right nephrectomy, the aberrant vascular anatomy, and the site for the ureteral anastomosis. After multidisciplinary discussion, we opted for a pelvic nephrectomy followed by a right-sided kidney transplant.
The procedure was performed in July 2015. The donor kidney was medium in size and had 1 artery, 1 vein, and 1 ureter. The pair was blood group compatible, with 3 HLA antigens mismatched. The direct microcytotoxicity crossmatch was negative. As immunosuppression, the patient was given basiliximab 20 mg intravenously on day 0 and day 4 (Simulect, Novartis Pharmaceuticals Corporation, Basel, Switzerland), tacrolimus 0.1 mg/kg/day orally (Prograf, Astellas Pharma Inc., Tokyo, Japan), mycophenolate mofetil 2000 mg/day orally (CellCept, Genentech Inc., San Francisco, California), and steroids.
The recipient was placed in a supine position. A right-sided Gibson incision was conducted; after medial mobilization of the peritoneum, the retroperitoneal space was entered. The pelvic kidney was tenaciously attached to the surrounding structures, making it extremely difficult to mobilize. Nephrectomy was performed, preserving as much as possible of the native kidney vascular supply and ureter (Figure 2). The allograft was put inside the abdomen, and the vessels were anastomosed as follows. The donor artery was connected end-to-end to the main renal artery of the recipient. The donor vein was anastomosed end-to-side to the right common iliac vein. After it was declamped, the graft perfused homogeneously and urine production began immediately. The transplant ureter was anastomosed end-to-end to the remnant native ureter on a 7Fr JJ indwelling stent. The entire procedure took 230 minutes. The warm ischemia time was 35 minutes, and the cold ischemia time was 130 minutes. The intraoperative blood loss was 150 mL. The postoperative course was uncomplicated. Serum creatinine concentration reached 1 mg/dL on day 3, and the patient was discharged on day 10. After 1-year follow-up, she is doing well with excellent graft function.
To the best of our knowledge, this is the first report of a patient with type B MRKH syndrome who underwent simultaneous nephrectomy and living-donor kidney transplant after complex vaginal reconstruction.
In 2000, Fedele and associates performed a laparoscopic creation of a neovagina in a woman with a kidney transplant, but the overall procedure was described as standard.10 Our patient was relatively young and fit. However, scarring from previous surgery, aberrant vascular anatomy, need for simultaneous pelvic nephrectomy, underlying cystocele, and short length of the donor vessels did represent difficult challenges. Careful imaging evaluation and meticulous surgical planning were key factors to avoid unexpected findings, reduce the duration of the operation, and safely perform the procedure. With complex recipients, a living-donor transplant is the best option as it gives the chance to proceed in an elective setting, reduces the risk of complications, and ensures the best long-term outcomes.
We decided to engraft the organ on the right side for several reasons. First, we had to remove the native kidney to prevent recurrent infections after transplant. Second, the risk of urinary complications arising from the cystocele suggested that we avoid a direct anastomosis between the graft and the bladder. Therefore, an end-to-end anastomosis between the transplant ureter and the remnant of the native ureter was considered the safest option. Moreover, the complex anatomy of the recipient, the aortic and iliac calcification, and the short length of the donor vessels forced us to find an alternative site for vascular reconstruction. The native renal artery represented a valid candidate for implantation as it was long enough and properly sized.
Type B MRKH syndrome is a complex disease. In patients with end-stage renal disease, kidney transplant remains the best option. However, associated malformations, scarring from previous surgical procedures, and vascular anomalies should be expected to represent a technical challenge. Careful pretransplant evaluation and multidisciplinary planning are recommended to reduce complications and optimize outcomes.
Volume : 17
Issue : 2
Pages : 266 - 268
DOI : 10.6002/ect.2016.0220
From the 1Nephrology and Dialysis Unit and the 2Renal Transplant Unit,
Fondazione I.R.C.C.S. Ca’ Granda - Ospedale Maggiore Policlinico, Milan, Italy;
and the 3Department of Clinical Sciences and Community Health, University of
Milan, Milan, Italy
Acknowledgements: The authors did not receive any funding for the present study, and they have no conflicts of interest to declare.
Corresponding author: Evaldo Favi, Renal Transplant Unit - Fondazione I.R.C.C.S. Ca’ Granda - Ospedale Maggiore Policlinico, Via della Commenda n. 15 - 20122 - Milan, Italy
Phone: +39 0250320393
Figure 1. Preoperative Abdomen and Pelvis Computed Tomography Scan with 3-Dimensional Reconstruction Showing a Pelvic Kidney Located in the Right Iliac Fossa
Figure 2. Intraoperative Image of the Native Pelvic Kidney Showing the Renal Artery (single arrow), Renal Veins (double arrows), and Ureter (triple arrows)