This video involves a rare case of vaginal prolapse diagnosed and treated in a 19-year-old with Meyer-Rokitansky-Kuster-Hauser syndrome. While previous case reports in the literature have described vaginal prolapse in MRKH patients after creation of a neovagina, to our knowledge there are no previous reports of de novo vaginal pouch prolapse in this patient population. Here we describe a patient presenting with MRKH and symptomatic prolapse of her vaginal pouch. We illustrate a native tissue transvaginal repair that was used to successfully resolve her condition. The patient presented to our division for evaluation of a vaginal lump she noticed with wiping. She had been symptomatic for the prior year, complaining of constant pressure, irritation of the prolapsed skin, and significant anxiety and unhappiness over this change in her anatomy. The patient was not previously or currently sexually active. She had been diagnosed with Meyer-Rokitansky-Kuster-Hauser syndrome approximately two years prior during an evaluation of primary amenorrhea, and the condition was confirmed by MRI. On examination in our office, she was found to have a blind vaginal pouch that was approximately four centimeters long. During Valsalva straining, a significant prolapse of her vaginal pouch was demonstrated. For maximum repetitive strain, there was complete aversion of the vaginal pouch. Several management strategies were discussed with the patient, with a strong focus on non-surgical options like simple observation, watch and wait, or an attempt at a pessary fitting. Regarding surgery, the patient's anatomy allowed us to avoid the traditional neovagina approach, which is associated with well-known long-term challenges. We believed that the starting length and width of her vaginal pouch would improve with the use of vaginal dilators, and eventually might enable a standard native tissue vaginal vault suspension. It was explained to the patient that this would require her to utilize dilators to extend the vaginal length until a standard fixation point, like the sacrospinous ligament, could be reached. Whereas the option of sacrocopepxy was discussed and offered, we advised against the implantation of mesh at such a young age, and recommended this be kept in mind as a future option if native tissue repair were to fail. It was emphasized that prolapse surgery is entirely elective, and we discussed the important considerations of age, sexual activity, and the impact of prolapse symptoms on her quality of life. After contemplating her options, the patient decided to proceed with the use of vaginal dilators in preparation for a native tissue repair. She was prescribed estradiol vaginal cream, and was referred to a pelvic floor physical therapist to provide assistance with the dilators, and to generally improve her pelvic muscle awareness and tone. While the patient was eager to proceed quickly with surgical correction, due to the psychological strain of constantly feeling the bulge, she understood the significance of achieving full vaginal dilation beforehand. She used the combination of vaginal dilators, estradiol cream, and physical therapy for four months, at which point the vagina fully extended to the level of the ischial spines and sacrospinous ligaments. After further counseling, we agreed to proceed with surgery. We recommended a suture-based sacrospinous ligament fixation, and explained that the specific vaginal incision, and whether to utilize a unilateral or bilateral approach, would be determined intraoperatively, once the mobility and quality of tissues could be fully assessed under anesthesia. Here we demonstrate the complete aversion of her vaginal pouch. Alice clamps were used to grasp the apex of the vagina, and a vertical incision was made. Based on assessment of the vaginal compartments, a decision was made to utilize a posterior approach to the sacrospinous ligaments. The edges of the vaginal walls were grasped with Alice clamps. The rectovaginal fascia was then dissected from the overlying vaginal epithelium. This dissection was eventually carried out laterally to the levator ani muscles, proximally to the apex, and distally to the perineal body. Here we see blunt dissection toward the ischial spine and sacrospinous ligament. Careful attention was paid here to maintaining an avascular dissection plane, lateral and adjacent to the levator ani muscles, reflecting the rectum medially. The sacrospinous ligament was then adequately cleared using blunt dissection. A suture capture device was then used to place a single suture into the mid-portion of the right sacrospinous ligament, about 2 cm from the ischial spine. The same process was repeated on the left side. Whereas both permanent and absorbable sutures can be utilized for sacrospinous suspension, with no clear evidence of superiority for one suture over another, in this particular case we selected permanent o-gortec suture due to markedly thin vaginal epithelium. A tapered free needle was then used to pass the suture through the subepithelial layer of each ipsilateral vaginal corner. The enteroseal space was then closed using an o-vicral purse-string suture. The posterior coporaphy was then completed in the traditional fashion. The vaginal incision was then closed using a 2-o-vicral suture, in a running locked fashion. Finally, the bilateral sacrospinous sutures were tied down, resulting in what appeared to be optimal suspension of the vaginal apex. Each side of the vaginal apex comfortably reached the ipsilateral sacrospinous ligament fixation point, with no suture bridge or excess tension noted. Here we see the final result of the pelvic reconstruction. This surgery achieved a vaginal length of approximately 6 cm after the repair. The patient was admitted overnight for her recovery. She had minimal pain and was able to spontaneously void on postoperative day number 0. She did not require the use of a Foley catheter. She was discharged to home on postoperative day number 1. She required pain medication for about 7 days postoperatively. The patient has been seen in the office for a 3-month follow-up and will be followed for several years to further assess her status. To date, she is extremely happy with her surgical outcome and feels that all of her presenting symptoms have been resolved.

vaginal prolapse

Meyer-Rokitansky-Kuster-Hauser syndrome

non-surgical options

surgery

sacrospinous ligament fixation