Management of partial Müllerian agenesis: staged McIndoe procedure for the creation of a neovagina and utero-neovaginal unification.

The patient did well postoperatively. She was educated on the importance of a dilation regimen to ensure preserved patency of the neovaginal canal. Since her procedure, she has elected to discontinue hormonal suppression, has experienced six episodes of monthly unobstructed menstruation, and has plans to follow-up closely.

We started by stenting the bilateral duplicated ureteral system for better delineation during the procedure. The left medial orifice was first cannulated with a sensor wire followed by a 5-French open-ended catheter. This allowed us to perform a left retrograde pyelography, which confirmed a patent left ureter and a normal-appearing renal pelvis. The double-J stent was then deployed in the usual fashion. The same procedure was repeated on the right medial orifice, during which normal right renal anatomy was confirmed. Both lateral ureteral orifices were also stented in a similar fashion. Attention was then turned to the vagina. An external examination showed normal labia minora bilaterally, a normal clitoris and urethral opening, and a normal anus. The Malecot drain was then exposed to facilitate dissection around the tract created at the vaginal apex. This dissection was performed bluntly to further develop the neovaginal space. A rectal finger and a bladder retractor were used intermittently to ensure a safe dissection. Cautery and 3–0 polyglactin stitches were used along the way to ensure hemostasis. This process was continued until the pericervical peritoneal reflection was encountered. A cervix was palpably present, but it was noted to be significantly flushed with the lower uterine segment likely secondary to chronic inflammation from hematometra. The Malecot drain’s exit point at the vaginal apex was isolated and two 2–0 monofilament sutures were placed to allow for anchoring the epithelialized tract to a window in the graft. The neovaginal space was packed with eight Raytec sponges to maintain patency and hemostasis while the skin graft was harvested. The patient was then placed in the right lateral decubitus position. She was re-draped and the left lateral thigh was prepped with betadine. The skin graft harvest site was demarcated with a marking pen. To harvest the skin graft, an Aesculap dermatome was set up with a 10-cm blade set at 0.017 in (0.04318 cm), and gentle pressure was applied along the marked area to harvest a 20-cm graft. The graft was placed in sterile saline and the harvest site was dressed with epinephrine-soaked laparotomy sponges. The skin graft was draped over a silicone mold measuring 4×11 in (10.16×27.94 cm), paying close attention to leaving the skin’s raw side outward. The graft was folded lengthwise, and the ends were brought together on the lateral sides with a series of interrupted 2–0 polyglactin stitches followed by a running 4–0 polyglactin stitch, which led to even coverage of the silicone mold. The harvest site was then exposed and dressed with Xeroform gauze strips, Telfa, and Tegaderm. The patient was then placed in the dorsal lithotomy position and was prepped and draped once again. The Malecot drain was placed through the upper aspect of the mold and graft. Using a free needle, the two 2–0 monofilament anchoring sutures that were previously placed around the Malecot drain’s exit point were passed through the vaginal graft and around the Malecot drain. After removing the eight Raytec sponges, the silicone mold with the encircling graft was lubricated and inserted into the neovagina using gentle pressure and with the help of a retractor. The mold was then inflated with 100mL of air, bringing together the raw edges of the skin graft to the neovaginal walls. The labia majora were closed over the mold with three interrupted 2–0 polyglactin stitches to keep it in place. The patient did well postoperatively and presented 1 week later for her stage 2 McIndoe procedure. We started by cutting the three polyglactin sutures on the labia majora. The Malecot drain was then lateralized, and the silicone mold was eased out of the neovagina, paying careful attention to avoid any disruption of the graft. The Malecot drain was visualized at the vaginal apex and using a Kelly clamp, it was removed in its entirety. Upon inspection, the skin graft was noted to be well applied to the neovaginal walls. The mold was then reinserted with the plan to educate the patient on how to remove it and replace it periodically. Finally, the graft harvest site was inspected and redressed.

In conclusion, we present a case of isolated vaginal agenesis in which menstrual suppression was appropriate initially but was not sufficient for the patient’s long-term goals. A staged McIndoe procedure with utero-neovaginal unification is a viable option for the creation of a neovagina in the presence of a functional uterus.

The purpose of this video is to discuss a technique for the creation of a neovagina and utero-neovaginal unification in a patient with partial Müllerian agenesis. Müllerian duct anomalies occur because of abnormal development of the uterus, cervix, and upper two-thirds of the vagina during embryogenesis [ 1 ]. The incidence of such anomalies has been estimated to be as high as 6.7% and even higher in patients with a history of infertility and recurrent pregnancy loss [ 2 – 4 ]. Mayer–Rokitansky–Küster–Hauser syndrome is a common variation of Müllerian anomalies; it typically presents as upper vaginal aplasia and uterine atresia or aplasia. However, the variable degree of expressivity seen with Müllerian anomalies leads to a spectrum of anatomical differences [ 5 ]. Amenorrhea, cyclic pain, dyspareunia, and infertility are common presenting complaints in patients with Müllerian anomalies [ 6 ]. Initial workup includes imaging such as pelvic ultrasound and/or MRI with situational evaluation of karyotype and endocrinological bloodwork. Screening for additional malformations is commonly done as other organ systems can be affected in patients with Müllerian anomalies [ 1 ]. Treatment ranges from conservative options to complex surgical approaches. We present the case of a nulliparous female with no significant medical, surgical, or family history, who was found to have a Müllerian anomaly in the form of isolated vaginal agenesis with a functional uterus. She was initially diagnosed in early puberty after presenting with cyclic pelvic pain and primary amenorrhea, despite normal development of secondary sexual characteristics. Workup by the patient’s gynecologist and an reproductive endocrinology and infertility subspecialist revealed appropriate Tanner Stage development and a 1-cm-long vaginal dimple. Imaging showed a normal uterus with an indeterminate cervix and normal ovaries and kidneys. Her primary diagnosis was thought to be a transverse vaginal septum versus cervico-vaginal agenesis. Given that she had no desire for intercourse at the time, she opted to pursue menstrual hormonal suppression with combined birth control pills. At the age of 23, she expressed interest in intercourse and also reported worsening abdominal pain, refractory to hormonal suppression. Repeat MRI revealed an enlarged, blood-filled uterus with a distended candidate cervix. An initial procedure revealed a distal vaginal pouch and a single, engorged uterus with a normal fundus, fallopian tubes, and ovaries. No hemoperitoneum or evidence of endometriosis were seen. Cystoscopy showed a duplicated ureteral system bilaterally with urine spillage from all four ureteral orifices. Subsequently, a mini-laparotomy via a Pfannenstiel incision was used to perform a small fundal hysterotomy and allow for drainage of 400cc of hematometra. A uterine sound was guided through the fundal hysterotomy down to the vaginal apex using tactile feedback of the surgeon’s fingers in the rectum and vagina, creating a communication between the uterus and vaginal pouch. A Malecot drain was threaded through this tract to allow for continued drainage of blood and epithelialization over time. The patient was on oral antibiotics for 2 weeks post-drain placement. Six weeks later, the patient underwent a staged McIndoe procedure.