Mayer-Rokitansky-Kuster-Hauser Syndrome Treatment & Management
- Author: Andrew J Kirsch, MD, FAAP, FACS; Chief Editor: Andrea L Zuckerman, MD more...
Although Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome (also referred to as Mayer-Rokitansky syndrome or Rokitansky-Küster-Hauser syndrome) has psychologically devastating consequences, its physiologic defects can be surgically treated. Following diagnosis, surgical intervention allows patients to have normal sexual function. Reproduction may be possible with assisted techniques.
Evaluation of patients with MRKH syndrome can be performed on an outpatient basis; however, surgical repair requires admission. Medical and surgical care are essential for capacity for sexual intercourse, and genital appearance. In rare cases, fertility may be possible. Using assisted reproductive techniques, women with MRKH syndrome can become pregnant by having oocytes harvested, fertilized, and implanted in a surrogate; however, outcome data in the literature are less than optimal.
The ideal repair provides the patient with an unscarred vagina that allows sexual functioning. There is no unanimity on which procedure is preferred in which circumstances, but Torres-de la Roche et al published an evidence-based expert opinion paper on the decision-making process.
Frank technique or perineal dilation
The only nonoperative option, this technique is successful in motivated patients willing to spend time to create a neovagina using the molds. Patients apply progressive pressure to the perineum using a bicycle-seat stool to hold a dilator in place. They need to be frequently examined to be sure they are dilating the vagina and not the urethra.
Because this technique is self-administered, compliance may be poor in patients with a vaginal dimple or no vagina because these patients may experience discomfort and abandon the dilator. However, this is often an indication of future compliance with vaginal dilators and molds in the postoperative period.
The surgical procedure most commonly employed for vaginal reconstruction is the McIndoe technique. A split-thickness skin graft is the most popular tissue for vaginal replacement. Skin from the thigh or buttocks is preferable. Sometimes, knowing the patient's tan lines is helpful in hiding the donor graft site.
With blunt dissection, a pocket is created between the urethra and the rectum. A cylindrical stent is covered with the skin graft and then placed into the potential space. The graft is fixed in place by attaching cut edges of the skin incision to recreate the introitus. The labia majora are then sutured loosely together to hold in the mold. A Foley catheter is placed, and the patient is given a low-residue diet and placed on modified bed rest.
About 1 week later, the stent is removed. The neovagina is irrigated, and any areas of granulation tissue are cauterized with silver nitrate sticks. The patient is instructed to use a mold or dilator in the neovagina every day and night for 3 months, then nightly for an additional 3 months to prevent contraction.
Disadvantages of this procedure include scarring at the donor site, neovaginal stenosis, and the need for long-term dilation.
In a Williams vaginoplasty, a vulval flap is used to make a vaginal tube. Although this simple procedure does not damage the urethra or the rectum, dilation is needed for a lengthy period, and the neovagina has a physiologically abnormal angle.
Rotational flap procedures
Rotational flap procedures use pudendal thigh, gracilis myocutaneous, labia minora, and other fasciocutaneous flaps. Disadvantages of these techniques include extensive skin scarring at the donor graft site and the need for patient diligence in postoperative dilation.
This technique uses an isolated segment of bowel to create a neovagina. The isolated segment retains its vascular supply via an intact mesentery. Sigmoid is generally the preferred bowel segment because it can most easily be mobilized to the perineum in a tension-free manner. Patients who have undergone this reconstructive technique report a high degree of satisfaction.
This procedure exerts continuous progressive pressure through passage of an acrylic olive through the potential neovaginal space and the abdominal wall. A traction device is placed into the peritoneal cavity and gradually draws the olive upward over a period of days to weeks. This gradually lengthens the vaginal vault. This technique is now performed laparoscopically.[7, 12]
Potential complications of surgical treatment of MRKH syndrome include the following:
Excessive skin scarring at the donor graft site
Postoperative rectovaginal and urethrovaginal fistulas
Vaginal discharge and dyspareunia
Inadequate vaginal lubrication
Poor patient compliance with the postoperative dilation schedule
The following consultations may be helpful:
Endocrinologist - A reproductive endocrinologist is best in this case; alternatively, a pediatric and adolescent gynecologist may be consulted
Monitor how well patients comply with postoperative dilation regimens.
Recommend condoms to help prevent human papillomavirus (HPV) infection.
Regularly examine patients to assess for stenosis.
Londra L, Chuong FS, Kolp L. Mayer-Rokitansky-Kuster-Hauser syndrome: a review. Int J Womens Health. 2015. 7:865-70. [Medline].
Morcel K, Guerrier D, Watrin T, Pellerin I, Leveque J. [The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome: clinical description and genetics]. J Gynecol Obstet Biol Reprod (Paris). 2008 Oct. 37(6):539-46. [Medline].
Edmonds DK, Rose GL, Lipton MG, Quek J. Mayer-Rokitansky-Küster-Hauser syndrome: a review of 245 consecutive cases managed by a multidisciplinary approach with vaginal dilators. Fertil Steril. 2012 Mar. 97(3):686-90. [Medline].
Carrard C, Chevret-Measson M, Lunel A, Raudrant D. Sexuality after sigmoid vaginoplasty in patients with Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril. 2012 Mar. 97(3):691-6. [Medline].
Hensle TW, Chang DT. Vaginal reconstruction. Urol Clin North Am. 1999 Feb. 26(1):39-47, vii. [Medline].
Hensle TW, Shabsigh A, Shabsigh R, Reiley EA, Meyer-Bahlburg HF. Sexual function following bowel vaginoplasty. J Urol. 2006 Jun. 175(6):2283-6. [Medline].
Fedele L, Bianchi S, Frontino G, Fontana E, Restelli E, Bruni V. The laparoscopic Vecchietti's modified technique in Rokitansky syndrome: anatomic, functional, and sexual long-term results. Am J Obstet Gynecol. 2008 Apr. 198(4):377.e1-6. [Medline].
Rall K, Eisenbeis S, Henninger V, Henes M, Wallwiener D, Bonin M, et al. Typical and Atypical Associated Findings in a Group of 346 Patients with Mayer-Rokitansky-Kuester-Hauser Syndrome. J Pediatr Adolesc Gynecol. 2015 Oct. 28 (5):362-8. [Medline].
Hall-Craggs MA, Williams CE, Pattison SH, Kirkham AP, Creighton SM. Mayer-Rokitansky-Kuster-Hauser Syndrome: Diagnosis with MR Imaging. Radiology. 2013 Aug 13. [Medline].
Friedler S, Grin L, Liberti G, Saar-Ryss B, Rabinson Y, Meltzer S. The reproductive potential of patients with Mayer-Rokitansky-Küster-Hauser syndrome using gestational surrogacy: a systematic review. Reprod Biomed Online. 2016 Jan. 32 (1):54-61. [Medline].
Torres-de la Roche LA, Devassy R, Gopalakrishnan S, de Wilde MS, Herrmann A, Larbig A, et al. Plastic neo-vaginal construction in Mayer-Rokitansky-Küster-Hauser syndrome: an expert opinion paper on the decision-making treatment process. GMS Interdiscip Plast Reconstr Surg DGPW. 2016. 5:Doc08. [Medline]. [Full Text].
Folgueira G, Perez-Medina T, Martinez-Cortes L, et al. Laparoscopic creation of a neovagina in Mayer-Rokitansky-Küster-Hauser syndrome by modified Vecchietti's procedure. Eur J Obstet Gynecol Reprod Biol. 2006 Aug. 127(2):240-3. [Medline].
Frega A, Scirpa P, Sopracordevole F, Biamonti A, Bianchi P, De Sanctis L, et al. Impact of human papillomavirus infection on the neovaginal and vulval tissues of women who underwent surgical treatment for Mayer-Rokitansky-Kuster-Hauser syndrome. Fertil Steril. 2011 Oct. 96(4):969-73. [Medline].