CASE REPORT

A Novel Approach to Recurrent Vaginal Vault Prolapse in a Patient With Müllerian Agenesis Tamara V. Toidze, MD, Karolynn T. Echols, MD, and Ricardo Caraballo, MD Background: Vaginal agenesis is a congenital disorder affecting 1 in 4000 to 1 in 10,000 females. Lack of normal vaginal support structures may lead these patients to develop prolapse of the distal rudimentary vagina. Presently, there is no consensus regarding the best surgical option. However, the goal of surgery is to restore normal anatomy and establish normal sexual function. Case: We report the case of a 28-year-old woman with müllerian agenesis, diagnosed at age 19 years, who presented with complaints of dyspareunia and vaginal eversion. One year before presentation to our practice, the patient underwent an anterior and posterior repair with mesh and a transobturator sling. After careful evaluation, the patient underwent excision of mesh and a second procedure to elongate and suspend her vagina. Her vaginal length was extended from 4 to 8 cm. Conclusions: Prolapse of the neovagina in müllerian agenesis is a rare event; it can occur after nonsurgical or surgical treatments. Although there have been reported cases of abdominal or laparoscopic sacrocolpopexy to correct vaginal vault prolapse in these patients, there is a scarcity of information addressing the vault suspension in cases lacking healthy vaginal tissue. Our case shows how successful anatomical functional correction can be achieved with a simpler technique using an allograft to achieve a normal vaginal length in combination with a sacrocolpopexy. Key Words: müllerian agenesis, Mayer-Rokitansky-Küster-Hauser syndrome, vaginal vault prolapse, neovagina, abdominal sacrocolpopexy (Female Pelvic Med Reconstr Surg 2015;21: e33–e35)

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aginal agenesis is a congenital disorder affecting 1 in 4000 to 1 in 10,000 females.1 Otherwise known as müllerian aplasia or agenesis or Mayer-Rokitanski-Kuster-Hauser syndrome, these patients are unable to have vaginal intercourse without treatment. Vaginal dilators are the preferred method of treatment. In patients where dilation is not successful or contraindicated, multiple procedures for construction of the vagina have been developed. Lack of normal vaginal support structures may lead these patients to develop vaginal vault prolapse. Surgical options for the vault prolapse include a sacrospinous ligament suspension or an abdominal sacrocolpopexy. Presently, there is no consensus regarding the best surgical option.2,3 However, the goal of surgery is to restore normal anatomy and establish normal sexual function. Prolapse of the neovagina in müllerian agenesis is a rare event, and it can occur after nonsurgical or surgical treatments. Although there have been reported cases of abdominal or laparoscopic sacrocolpopexy to correct vaginal vault prolapse in these patients,4–6 there is a scarcity of information addressing the vault suspension in cases lacking vaginal length. Most cases use the McIndoe, Vecchietti, or Davydov procedures7–9; these operations can be tedious and complex, and there is no consensus regarding the best surgical treatment for these patients. Our case shows how successful anatomical and functional correction can be achieved From the University Urogynecology, Cooper Medical School at Rowan University, Camden, NJ. Reprints: Ricardo Caraballo, MD, Cooper University Hospital/Cooper Medical School at Rowan University, 6012 Main Street, Voorhees, NJ 08043. E-mail: [email protected]. The authors have declared they have no conflicts of interest. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. DOI: 10.1097/SPV.0000000000000168

with a simpler technique using an allograft to achieve a normal vaginal length in combination with a sacrocolpopexy.

CASE REPORT A 28-year-old patient with a history of müllerian agenesis, diagnosed at age 19 years, presented to an outlying specialist with primary amenorrhea and the inability to have intercourse. Initial gynecologic evaluation revealed her to have a 46 XX karyotype as well as normal ovarian function, average height, and average build. Her secondary sexual characteristics were normal, with well-developed breasts, axillary hair, and pubic hair. She had a 3-cm vaginal length. Evaluation of the renal system was normal. Diagnostic laparoscopy revealed absence of the uterus with fused fallopian tubes in the midline. The patient used vaginal dilators and was able to have intercourse with some satisfaction. There was no documentation of her vaginal length after the use of dilators. Eight years after the use of vaginal dilators, the patient presented to her local urologist with complaint of protrusion and urinary incontinence. She was diagnosed with complete vaginal eversion and stress urinary incontinence. Vaginal length was not documented. She subsequently underwent an anterior and posterior repair with mesh using Elevate and a transobturator sling. One year postoperatively, the patient presented to our practice for the first time with recurrence of her protrusion, dyspareunia, and decreased sexual satisfaction. She had no complaint of urinary incontinence. On pelvic examination, vaginal length was 4 cm with total eversion and severe scarring from previous mesh placement. Pelvic organ prolapse quantification points were as follows: Aa, +3; Ba, +3; C, +3; Gh, 4; Pb, 3; TVL, 4; Ap, +3; and Bp, +3. Both anterior and posterior meshes were easily palpable, and the patient was uncomfortable to mild pressure. Rectal examination revealed an asymptomatic stricture from the posterior arms, on the left more than the right (Fig. 1). Our approach to this patient required 2 surgical interventions. First, surgery was performed to remove vaginal mesh anteriorly and posteriorly. The patient tolerated the procedure well and continued regular vaginal dilation. Postoperative pelvic examination revealed no palpable mesh. Eight months after mesh removal procedure, the patient presented with desire to have a full-length functional vagina. She was extensively counseled about her treatment options and elected to have a sacrocolpopexy with elongation of her vagina using biological grafts as needed. Surgeon preference dictated that the abdominal part of the procedure be performed via open laparotomy. Laparoscopic or robotic route would have been adequate. The abdomen was opened by Pfannenstiel incision 2 cm above the symphysis pubis. Once the abdominal cavity was entered, an end to end anastomosis sizer reusable sizer was placed in the vagina to help identify the vaginal apex intra-abdominally. Once the apex was identified, a 2-cm rudimentary uterus was noted and removed to clear the apex from surrounding tissue. Once the apex was exposed, the anterior and posterior portions of the vagina were dissected away from the bladder and the rectum, respectively. A Y-shaped polypropylene mesh was attached to the anterior and posterior portions of the vagina with interrupted 2-0

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Toidze et al

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FIGURE 3. Mesh attached to vaginal vault and ready to be attached to longitudinal ligament.

FIGURE 1. Vaginal length 4 cm with total eversion.

polydioxanone sutures. Attention was then directed to the vagina. A scalpel was used to make a transverse incision at the midlevel of the vagina encompassing the posterior and lateral walls. To minimize the chance of mesh exposure, care was taken to ensure that the vaginal incision was not over the area covered by the sacrocolpopexy mesh. The vaginal mucosa was then undermined proximally and distally. Gentle cephalad traction and lysis of the remaining connective tissue along the posterior vaginal incision resulted in several centimeters of added vaginal length. An AlloDerm thin implantable graft was trimmed to the shape of the posterior defect and attached to the surrounding portion of the vaginal wall using 2-0 polyglactin 910 (Fig. 2). After completion of the vaginal portion of the surgery, attention was turned back to the abdominal cavity, where the previously prepared polypropylene mesh anchoring the vaginal apex was attached without tension to the anterior longitudinal ligament with CV-2 Gore-Tex sutures. The mesh was retroperitonealized with 3-0 polyglactin 910. Cystoscopy revealed no injuries to the bladder or the urethra and bilateral efflux of indigo carmine dye from both ureteral orifices. Intraoperatively, the vaginal length was extended from 4 to 8 cm (Fig. 3). After her discharge home, the patient was placed on a regimen of estrogen cream and twice-daily dilator use until the onset of vaginal intercourse 3 months postoperatively. Six months after surgery, the patient developed vaginal bleeding caused by diffuse

FIGURE 2. AlloDerm® placed in posterior compartment.

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granulation tissue without mesh exposure in the posterior apical vaginal wall. She underwent excision and fulguration of the granulation tissue. The patient used dilators for an additional 3 months. She reported sexual activity with satisfaction. Sexual function was not quantitatively measured. The pelvic organ prolapse quantification points, 1 year after surgery, were Aa, −3; Ba, −3; C, −5; Gh, 3.5; Pb, 3; TVL, 7.5; Ap, −2; and Bp, −2.

DISCUSSION The etiology of prolapse of the neovagina in müllerian agenesis is unknown. The lower third of the vagina is derived from the urogenital sinus and lacks apical support. Surgical procedures designed to construct a neovagina do not typically establish normal anatomic support for the full-length vagina. It stands to reason that these patients may be at increased risk for prolapse of the neovagina. Recently, abdominal sacrocolpopexy has been used to correct prolapse of the neovagina. In 1990, Peters and Uhlir5 described the case of a patient with a mechanically created neovagina with complete prolapse, who was treated with abdominal sacrocolpopexy. In 2002, Schaffer et al6 reported 2 cases of women with Mayer-Rokitansky-Küster-Hauser syndrome and vaginal vault prolapse, who were successfully treated with abdominal sacrocolpopexy with polyester mesh, Halban culdoplasty, and paravaginal repair. In 1 case, the total vaginal length remained 6 cm before and after the procedure, and in the other case, the vaginal length was elongated from 4 to 5 cm after the procedure. Most recently, Christopoulos et al,10 in 2011, reported the case of a 23-year-old patient with Mayer-Rokitansky-KüsterHauser syndrome, who was initially treated with vaginal dilators, and her vaginal length upon completion of therapy was 7.5 cm from 3 cm before treatment. The patient was treated with laparoscopic sacrocolpopexy, which corrected the prolapse and elongated the vaginal length to 9 cm. Sacrospinous ligament fixation has also been reported as a treatment of the prolapsed neovagina. Muir and Walters,11 in 2004, described a case of sacrospinous ligament fixation, but the vaginal length was not long enough to be directly sutured to the sacrospinous ligament, and a fascia lata allograft was used to attach the anterior and posterior vaginal walls to the sacrospinous ligament. Calcagno et al,4 in 2010, described a case of vaginal elongation with the use of vaginal dilators, followed by sacrospinous ligament suspension to correct vaginal eversion. Lastly, Burns et al,12 in 2012, reported a case of a 21-year-old patient who was treated with a sacrospinous ligament suspension using a mesh implant. She developed a mesh erosion 6 months © 2015 Wolters Kluwer Health, Inc. All rights reserved.

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Female Pelvic Medicine & Reconstructive Surgery • Volume 21, Number 3, May/June 2015

after the procedure, which was later corrected with mesh excision and the use of a biological graft to repair the defect. Patients with eversion of the neovagina pose a special challenge to the operating surgeon because of the shortened and scarred vagina often associated with dyspareunia. A 2010 Cochrane review by Maher et al13 showed comparison of abdominal sacrocolpopexy versus sacrospinous colpopexy in women after hysterectomy. Abdominal sacrocolpopexy was associated with lower rate of recurrent prolapse and less dyspareunia than was vaginal sacrospinous colpopexy. This observation may be relevant when choosing treatment options for patients with vaginal agenesis and prolapse. Our case is unique in that it shows how successful anatomical and functional correction can be achieved via abdominal sacrocolpopexy and creation of a neovagina with a biological graft even after severe scarring due to previous transvaginal mesh placement. REFERENCES 1. Evans TN, Poland ML, Boving RL. Vaginal malformations. Am J Obstet Gynecol 1981;141:910–920. 2. Müllerian agenesis: diagnosis, management, and treatment. Committee Opinion No. 562. American College of Obstetricians and Gynecologists. Obstet Gynecol 2013;121:1134–1137. 3. Laufer MR. Congenital absence of the vagina: in search of the perfect solution. When, and by what technique, should a vagina be created?Curr Opin Obstet Gynecol 2002;14:441–444.

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Vaginal Prolapse in Müllerian Agenesis

4. Calcagno M, Pastore M, Bellati F, et al. Early prolapsed of neovagina created with self-dilatation and treated with sacrospinous ligament suspension in a patient with Mayer-Rokitansky-Kuster-Hauser syndrome: a case report. Fertil Steril 2010;93:267.e1–e4. 5. Peters WA 3rd, Uhlir K. Prolapse of neovagina created by self-dilatation. Obstet Gynecol 1990;76(pt 2):904–906. 6. Schaffer J, Fabricant C, Carr BR. Vaginal vault prolapsed after nonsurgical and surgical treatment of müllerian agenesis. Obstet Gynecol 2002;99(pt 2):947–949. 7. Borruto F, Chasen ST, Chervenak FA, et al. The Vecchietti procedure for surgical treatment of vaginal agenesis: comparison of laparoscopy and laparotomy. Int J Gynaecol Obstet 1999;64:153–158. 8. Davydov SN, Zhvitiashvili OD. Formation of vagina (colpopoiesis) from peritoneum of Douglas pouch. Acta Chir Plast 1974;16:35–41. 9. McIndoe AH, Banister JB. An operation for the cure of congenital absence of the vagina. J Obstet Gynaecol Br Commonw 1938;45:490–494. 10. Christopoulos P, Cutner A, Vashisht A, et al. Laparoscopic sacrocolpopexy to treat prolapsed of the neovagina created by vaginal dilation in Rokitansky syndrome. J Pediatr Adolesc Gynecol 2011;24:e33–e34. 11. Muir TW, Walters MD. Surgical management of vaginal vault prolapse in a woman with neovagina and pelvic kidneys. Obstet Gynecol 2004;104 (pt 2):1199–1201. 12. Burns E, Naim M, Badawy MD. Mulerian agenesis with vaginal vault prolapsed following mechanically created neovagina. J Pediatr Adolesc Gynecol 2012;25:e75–e76. 13. Maher C, Feiner B, Baessler K, et al. Surgical management of pelvic organ prolapsed in women. Cochrane Database Syst Rev 2010;(4):CD004014.

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A novel approach to recurrent vaginal vault prolapse in a patient with müllerian agenesis.

Vaginal agenesis is a congenital disorder affecting 1 in 4000 to 1 in 10,000 females. Lack of normal vaginal support structures may lead these patient...
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