Creation of a Neovagina with Single Staged Uterine Anastomosis.

Mullerian agenesis, commonly known as Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome, affects 1 in 4500 to 5000 genotypically XX females. 1 While the condition most frequently entails complete vaginal agenesis accompanied by an absent or rudimentary uterus, it may also occur in isolation with a fully developed uterine body and/or cervix. 2 Removal of uterine tissue is traditionally recommended in order to prevent endometriosis from retrograde menstruation, 3 however in the rare case of a fully formed uterus, this results in permanent loss of ability to carry a pregnancy. Techniques to create a vaginal canal are established and include utilization of a skin graft held in place with a vaginal mold (McIndoe), 4 creation of a vaginal canal via peritoneal transposition (Davydov), 5 and less commonly via upward abdominal traction on the introital dimple or utilization of a segment of sigmoid colon. 6 Case reports exist of anastomosis of a neovagina to the uterine corpus with reports of spontaneous conception following vaginoplasty. 7 However, prior attempts at anastomosis have all included delayed surgery with anastomosis to the uterus several months following neovagina creation. 8 , 9 This case demonstrates a single stage creation of a peritoneal neovagina with anastomosis to a functional uterine corpus. The patient was a 17-year-old female with a medical history notable for ectopic ureters requiring bilateral reimplantation at age 4 due to recurrent urinary tract infections in the setting of ureteral reflux. She presented with primary amenorrhea at age 14, four years after thelarche. After noting a blind ending vagina and a 46XX karyotype, ultrasound and MRI revealed an anatomically normal uterus with questionable presence of a cervix, and polycystic ovaries, which were presumed to have accounted for the patients minimal hematometra. A multidisciplinary team including gynecology, urology, plastic surgery, and colorectal surgery was organized for creation of a peritoneal neovagina. This team had extensive experience with peritoneal neovagina creation in both cis and transwomen. Given the appearance of a normal uterus, the patient and her parents were counseled on attempted uterine preservation with anastomosis of the neovagina to the uterine cavity, and they were consented for surgery with videography for the purposes of education. Surgery began with cystoscopic evaluation, placement of bilateral ureteral catheters into the reimplanted ureters, and injection of indigo cyanine green dye to facilitate ureteral identification during the laparoscopic dissection. The abdomen was entered laparoscopically in the left upper quadrant. Four robotic ports and one assistant port was placed. The robot was docked with a monopolar scissor, bipolar, tip up grasper, and 30°F camera. Pelvic survey revealed a normal appearing uterus and fallopian tubes with polycystic ovaries. The space between the bladder and vaginal remnant was dissected robotically with a combination of sharp dissection and electrocautery, taking care to stay in the avascular plane between the two structures, to the level of the bladder neck. With one surgeon palpating at the vaginal dimple, the location on the obliterated vaginal tissue was confirmed, and the rectovaginal space was dissected downwards toward the perineum. The space between the blind ending vagina and peritoneal cavity was incised. The peritoneum was mobilized and confirmed to reach the perineal incision for what would become the inferior margin of the neovagina. An incision was made in the midline of the uterine fundus with monopolar scissors to maintain hemostasis, and this incision was carried downward until endometrial tissue was visualized. A bougie was inserted transfundally to determine the lower bound of the patent endometrial cavity and/or cervical canal. In this case, no tissue consistent with cervical stroma was identified. With digital assistance from below, a laparoscopic grasper was inserted pushed through the lower uterus, and a foley catheter was brought up into the uterine corpus to allow the uterine cavity to drain into the neovagina and remain patent postoperatively. Attention was returned to the neovagina. An intervening band of obliterated tissue was incised in the midline. The peritoneum over the rectovaginal septum was mobilized and connected to the posterior vaginal introitus with delayed absorbable suture. Anteriorly, the freed edge of peritoneum was brought downward to the anterior vaginal introitus and then sutured transvaginally. The lateral edges of the obliterated vaginal tissue were rotated 90 degrees and sutured to the anterior and posterior aspects of the peritoneal neovagina flaps to become the lateral edges of the neovagina ( Figure 1 ). The peritoneum overlying the rectum was carefully mobilized in an inverted U shape, then flipped to create the upper bound of the posterior neofornix. This did incorporate bilateral uterosacral ligaments and obliterate the rectovaginal space ( Figure 2 ). Peritoneum over the dome of the bladder was carefully dissected away from the bladder in a similar inverted U and transposed to create the anterior vaginal fornix. The peritoneal neovagina was successfully connected to the uterus at the level of the isthmus when a normal cervical canal could not be identified. A schematic of the final anatomy is depicted in Figure 3 , with blue indicating the existing uterine body and the native introital tissue, and green depicting mobilized peritoneum. The band of tissue from the obliterated vaginal space was dissected in the midline then rotated to become the lateral edges of the neovagina. The serosa of the anterior lower rectum was sutured to the uterosacral ligaments and posterior uterus, obliterating the Pouch of Douglas and becoming the upper posterior vagina. The final anatomical outcome: blue indicates the existing uterine body and the native introital tissue, and green depicts the mobilized peritoneum. Oral estradiol was prescribed to facilitate endometrial proliferation and patency of the canal. A foley catheter in the uterine cavity remained in place 11 days after surgery. Vaginal packing in the neovagina was changed on postoperative day (POD)5 and removed on POD11. The patient discharged from the hospital on POD11 and immediately began dilation three times daily. She began to report light bleeding on POD17. Evaluation on POD20 revealed a healing vagina with a patent os at the vaginal apex. Diagnostic hysteroscopy on POD45 confirmed a patent os with an estrogenized uterine cavity. Three months after surgery, the patient presented to gynecology clinic and noted ongoing use of vaginal dilators with monthly bleeds during the placebo week of her oral contraceptive pills, which were continued given her polycystic ovarian syndrome.

In the presence of vaginal agenesis with a functional upper reproductive tract, a peritoneal neovagina may be successfully anastomosed to the lower uterus in a single staged robotic approach. Prior reports of treating this rare condition have focused on staged approaches, with creation of a neovagina at the first stage and later anastomosis to the uterine body. At the onset of menstruation, accumulation of obstructed menses (hematocolpos) can cause significant patient discomfort. In addition to reducing morbidity from multiple procedures, the single stage technique described here has the benefit of allowing immediate relief of hematocolpos, with the added advantage egressing menstrual blood provides in maintaining patency of the new vaginal canal. Our technique involved a modified Davydov procedure as our team has had better outcomes with this method, but a single staged approach could theoretically be accomplished with a modified McIndoe graft followed by abdominal entry for uterine reanastamosis. This technique, and any technique for creation of a neovagina, should be limited to high volume centers with experience in treating vaginal agenesis, as repeat reconstructive surgery is difficult and morbid. Our patient had marked polycystic ovaries and obesity, which likely prevented normal menstrual cycling and earlier diagnosis of her uterine outflow obstruction. Additional consideration should also be taken to evaluate for associated renal and skeletal abnormalities, which are concurrently present in 53% of patients. 10 After surgery, she required oral contraceptives to maintain menstruation. Without regular menstruation, occlusion of the anastomosis is a significant concern and thus a limitation of both the single stage technique as well as a staged reanastomosis. Additionally, all techniques for neovagina creation depend on regular postoperative dilation, often for life, to maintain a functional vaginal orifice. Selection of patients who can adhere to these postoperative requirements is essential. However, in the appropriately selected patient, Mullerian agenesis with a functional uterine body can be successfully treated in a single stage, fertility maintaining approach by a multidisciplinary team.