Vaginal Agenesis Treatment

What is vaginal agenesis?

Vaginal agenesis or Müllerian agenesis is the congenital absence of the vagina, the uterus or both due to Mayer-Rokitansky-Kuster-Hauser Syndrome. Agenesis or atresia means that an organ has not developed in the embryo and it is absent.

How often is vaginal agenesis seen?

Vaginal agenesis is seen in 1 per 4500-5000 females.

What is the most common cause of vaginal agenesis?

Vaginal agenesis is caused by embryologic underdevelopment of the Müllerian duct, causing agenesis of the vagina, uterus or both.

What causes a girl to be born without a uterus?

A girl can be born without a uterus due to a congenital condition called MRKH Syndrome in which the Müllerian duct is underdeveloped in the embryo, leading to absence of the vagina, uterus or both.

Gynecological examination reveals that the vagina is absent and pelvic ultrasonography may be followed by MRI to confirm if the uterus, tubes and ovaries exist. MRI can also diagnose any concommitant abnormalities related to the urinary system.

Karyotyping is necessary in patients with vaginal agenesis to examine if they have XX or XY chromosomes. In rare cases XY or XO karyotype may exist in patients with vaginal agenesis.

Differential diagnosis of MRKH syndrome includes isolated vaginal atresia found in various syndromes such as Mc Kusick-Kaufman Syndrome, androgen insensitivity syndrome (with XY karyotype),Mullerian aplasia and hyperandrogenism (46 XX) and renal-genital-middle ear anomalies.

How is the management of vaginal agenesis?

The following steps are important in the effective management of patients with vaginal atresia:

• Correct diagnosis of the underlying condition,
• Evaluation for associated congenital anomalies,
• Psychological counseling,
• Treatment or intevention to correct the anatomical genital anomalies,
• Discussion of options for future pregnancy.

Is vaginal atresia curable?

Vaginal atresia is a curable condition. Vagina can be reconstructed using several surgical and non-surgical techniques to make penetrative sexual intercourse possible.

How is vaginal atresia treated?

The first step in the management of patients with vaginal atresia is the non-surgical vaginal elongation method by using vaginal dilators. If patients are emotionally well prepared, most of them can achieve functional vaginal length by this non-surgical approach.

If this approach fails, patients should be referred to centers with expertise in the surgical management of vaginal atresia.

• Mc Indoe procedure.
• Peritoneal vaginoplasty-uses peritoneum (tissue covering abdominal organs) to create the neovagina done by laporoscopic surgery. The peritoneum is lined into the newly opened vaginal space.
• Intestinal vaginoplasty-uses a section of sigmoid colon to create the neovagina, usually done as a laporoscopic surgery.

What is Mc Indoe Procedure?

Mc Indoe Procedure is the creation of neovagina by using skin graft usually harvested from the groin area. The skin graft is placed on a vaginal mould and the mould is placed into the space created for neovagina. The patient needs to use the mould regularly so that the vagina stays open.

The disasdvantage of this method compared to the intestinal vaginoplasty and peritoneal vaginoplasty is that skin is not a mucosal tissue and it does not lubricate and there is higher risk that the vaginal opening will close.

Can people with müllerian agenesis get pregnant?

People with müllerian agenesis do not have a uterus so they can only get pregnant by a very sophisticated method called uterine transplantation which is an experimental method applied only in special centers in the world.

Can you get pregnant without a uterus but still have ovaries?

You can not get pregnant without a uterus but your eggs may be used in IVF treatment to create an embryo and the embryo can be transferred into the surrogate mother’s uterus.

Can someone with MRKH have kids?

Patients with MRKH syndrome can have kids either by gestational surrogacy or adoption. If the patient has ovaries, her eggs can be retrieved by in vitro fertilisation treatment, embryo may be formed by ICSI and the embryo may be transferred to the surrogate mother’s uterus.

Do people with MRKH have eggs?

Typically women with MRKH syndrome have ovaries and eggs so that they ovulate. They may have symptoms related to ovulation such as mid-cycle pain and premenstrual syndrome but no menstrual bleeding.

Do girls with MRKH ovulate?

Since girls with MRKH syndrome have ovaries, they do ovulate.

What happens to eggs with MRKH?

Women with MRKH have ovaries and the eggs will be expelled into the abdominal cavity during ovulation, the same way as in other patients who have a uterus. The difference is that the eggs will not be able form an embryo in the fallopian tube, and there is no uterus for an embryo to be implanted.

How do MRKH pee?

MRKH syndrome does not affect urination, because the urethral anatomy is normal. So MRKH patients can pee normally.

Can you have an orgasm with MRKH?

Orgasm in MRKH syndrome patients can be achieved by stimulation of the clitoris. Women do not necessarily need to have a vagina to achieve orgasm. After vaginal agnesis treatment, vaginal intercourse will also make it possible to have an orgasm through penetration.