Case Presentation
This case presentation is of a 34-year-old married woman with Type I Mayer-Rokitansky-Küster–Hauser syndrome. She presented with no known comorbidities and an unremarkable past surgical history. She had undergone dilator therapy to acquire an appropriate-caliber vagina estimated to be 8cm long. Furthermore, she had received controlled ovarian stimulation twice, utilizing an antagonist technique as part of in vitro fertilization.
Uterine transplantation (UTx) is the only suitable intervention that recovers reproductive anatomy and functionality. Especially in women with absolute uterine factor infertility (AUFI). UTx involves transplanting of the uterus, cervix, surrounding ligamentous tissues, related blood vessels, and a vaginal cuff. AUFI is the alternative to adoption and surrogacy.
The donor of the uterus was the patient’s 40-year-old sister. She had no known comorbidities or medical conditions except that she was CMV IgG positive. In addition, all other serum virology was unremarkable. Ultrasound, computed tomography, and magnetic resonance imaging all revealed typical pelvic architecture and vascularity. In addition to a duplex right kidney with two proximal ureters and a normal left kidney and ureter.
The HLA mismatch was 1-1-1, and the calculated reaction frequency was 78%. Despite low-level HLA class II DSA, including a self-reactive antibody against one product of the same haplotype (a potential HLA-DR4, -7, -9 epitope) and the entirely allogeneic HLA-DR52, all cross-matching was negative.
Uterus Transplant Procedure
The donor retrieval procedure was carried out via a Maryland incision. A modified radical hysterectomy was performed, with significant retrieval of the round and uterosacral ligaments and a considerable amount of bladder peritoneum with lengthy vascular pedicles. However, the vascular pedicles consisted of the uterine arteries to the anterior division of the internal iliac on the left and the lateral uterine arteries on the right, which were doubled in this case. A bilateral salpingectomy was performed with the preservation of both ovaries.
The recipient operation began one hour before the planned uterine explant. The procedure was carried out through an infra-umbilical midline incision. Furthermore, the anastomosis of the external iliac arteries and veins was prepared by skeletonizing them. The fallopian tubes and uterine remnants were subsequently excised, leaving the vagina closed. Doctors placed the graft in the pelvis in an orthotopic position with successful vascular anastomosis. However, the effort between the utero-ovarian vein and the external iliac vein was unsuccessful. It was worsened by vascular tearing during the anastomosis. Therefore, left-sided venous drainage was achieved between the utero-ovarian vein and the inferior epigastric vein.
The vagina was then opened, and the vaginal anastomosis was performed with interrupted sutures. Following reperfusion, the uterus transformed from a dark pale tone to a pink and well-perfused look. Doctors administered appropriate immunosuppressants, antibiotics, and antifungal prophylaxis.
The patient was discharged on the 10th postoperative day. Moreover, doctors proceeded with conservative management for her excessive lymphatic drainage after the incision.
Follow Up
She was extensively monitored after discharge, twice weekly for the first postoperative month and then weekly thereafter. At each visit, a clinic examination of the vagina with speculum and high vaginal swabs was undertaken, with blood culture and urine microscopy culture. Cervical biopsies were collected on postoperative day 5, 2 weeks, 1 month, and then monthly thereafter. According to the histopathological grading criteria employed in the Dallas Uterus Transplant Study (DUETS), all biopsies were classified as normal, with no indications of rejection.
Transabdominal ultrasound scans revealed consistent uterus size and echogenicity throughout the menstrual cycle, evolving endometrial thickness, and normal uterine artery and intramyometrial Doppler waveforms. She had her first period two weeks after the surgery. At the time of reporting, 10 weeks postoperatively, she had her third menstrual cycle and was doing well.
Discussion
This case has several innovative aspects. First, it is the first prosperous case where successful vascular reconstruction was attempted. Due to unusual vasculature, where there were two right-sided uterine arteries, an uncommon variant, they were pantalooned to produce a common stem, which was anastomosed to the recipient’s external iliac artery.
The second novelty of this case is the venous drainage used, as this is the first case that has used patients’ inferior epigastric veins for drainage. A feared complication that is minimized in the patient is the CMV infection transmission from the seropositive donor. For this, the patient was given a complete 6 months of antiviral prophylaxis before 3 months of CMV PCR surveillance to make sure she remained infection-free before transferring the embryo
Moreover, this is the first case to use alemtuzumab as an immunosuppressant for induction when the majority of cases have used the polyclonal antibody anti-thymocyte globulin (ATG). Alemtuzumab, on the other hand, is routinely utilized in kidney and pancreas transplantation and is standard induction therapy for the majority of solid organ transplants in our centre. When compared to ATG, it is associated with decreased biopsy-proven rejection and has been shown to reduce post-transplantation immunosuppression and steroid exposure.
