Mayer-Rokitansky-Kuster-Hauser Syndrome Treatment & Management

Updated: Aug 11, 2020
  • Author: Andrew J Kirsch, MD, FAAP, FACS; Chief Editor: Andrea L Zuckerman, MD  more...
  • Print
Treatment

Approach Considerations

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. There is interest in the possibility of uterine transplantation for women with uterine factor infertility (UFI), including those with UFI from MRKH syndrome. [17, 18]

Next:

Medical Care

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, as well as for 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. [19]

Previous
Next:

Surgical Care

There is no consensus across specialities between nonsurgical versus surgical management for vaginal agenesis. [3]  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. [20]

Excision of uterine anlage can also prevent endometriosis and resultant ovarian function impairment. [4, 5]  Removal of the rudimentary uterus is suggested if hematometra develops.

Frank technique or Ingram modification

The only nonoperative option, this technique is successful in motivated patients who are 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. [6] 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.

McIndoe technique

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.

Williams vaginoplasty

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.

Intestinal neovagina

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. [7] Patients who have undergone this reconstructive technique report a high degree of satisfaction. [8]

Vecchietti technique

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. [9, 21, 10]

Wharton-Sheares-George method

The Wharton-Sheares-George neovaginoplasty has been suggested as a a simple and effective minimally invasive surgical approach to the creation of a neovagina. [11]  In this procedure, the müllerian ducts are dilated incrementally by pushing dilators in the direction of the pelvic axis,  the median raphe is intersected, and a a vaginal mold is inserted into the newly created cavity and held in position by two sutures. No allogenic or autologous transplants are used, nor is there a requirement for traction devices or specialized surgical equipment.

Previous
Next:

Complications

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
  • Vaginal stenosis
  • Inadequate vaginal lubrication
  • Poor patient compliance with the postoperative dilation schedule
Previous
Next:

Consultations

The following consultations may be helpful:

  • Endocrinologist - A reproductive endocrinologist is best in this case; alternatively, a pediatric and adolescent gynecologist may be consulted
  • Geneticist
  • Orthopedic specialist
  • Urologist
  • Psychiatrist
  • Audiologist
Previous
Next:

Long-Term Monitoring

Monitor how well patients comply with postoperative dilation regimens.

Recommend condoms to help prevent human papillomavirus (HPV) infection. [22]

Regularly examine patients to assess for stenosis.

Previous