Mayer-Rokitansky Syndrome Treatment & Management

• Although evaluation of patients with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome can be performed on an outpatient basis, 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 Mayer-Rokitansky-Kuster-Hauser syndrome can become pregnant by having oocytes harvested, fertilized, and implanted in a surrogate.

The ideal repair provides the patient with an unscarred vagina that allows sexual functioning. Excision of uterine anlage can also prevent endometriosis and resultant ovarian function impairment.^{[3, 4]}

• Frank technique or perineal dilation

  • The only nonsurgical 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.
• McIndoe technique

  • The most common surgical procedure used for vaginal reconstruction is the McIndoe technique. The technique involves the following steps:
    • A split-thickness skin graft is the most popular tissue for vaginal replacement. Skin from the thigh or buttocks is preferable. Sometimes knowing the patients tan lines is helpful in hiding the donor graft site.
    • Using a blunt dissection, create a pocket between the urethra and rectum.
    • After covering a cylindrical stent with the skin graft, place it into the potential space. Fix the graft into 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 one week later, remove the stent. Irrigate the neovagina and cauterize any areas of granulation tissue with silver nitrate sticks.
    • Have the patient use a mold or dilator in the neovagina every day and night for 3 months, followed by nightly insertion for 3 more 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

  • Williams vaginoplasty uses a vulval flap to make a vaginal tube.
  • Although this simple procedure does not damage the urethra or rectum, dilation is needed for a lengthy period, and the neovagina has a physiologically abnormal angle.
• Rotational flap procedures

  • Rotational flap procedures use the 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 postsurgical dilation.
• Intestinal neovagina

  • This technique uses an isolated segment of bowel for vagina
  • The isolated segment retains its vascular supply via intact mesentery.
  • Sigmoid is generally the preferred bowel segment, as it can most easily be mobilized to the perineum in a tension-free manner.^[5]
  • Patients who have undergone this reconstructive technique report a high degree of satisfaction.^[6]
• Vecchietti technique

  • Exerts continuous progressive pressure by an acrylic olive passed 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]

• Endocrinologist: A reproductive endocrinologist is best in this case or a pediatric and adolescent gynecologist.
• Geneticist
• Orthopedic specialist
• Urologist
• Psychiatrist
• Audiologist