By Dr Rajesh Vasu; HOD&Sr. Consultant Plastic& Aesthetic Surgeon; Fellowship in Aesthetic Surgery, Fellowship in Hair Restoration Surgery,Continental Hospitals

MRKH syndrome is a common name for Mayer-Rokitansky-Kuster-Hauser Syndrome. It is a condition where the affected girls /women are born either without a vagina or uterus or with an underdeveloped vagina or uterus. It comes as a surprise that this condition is present in 1: 10000 female births.

What is MRKH syndrome?

Women born with this syndrome have normal external genitalia and are brought up as normal girls as they have normal secondary sexual features. They may have an underdeveloped vagina which may appear as a “vaginal dimple” or may have a complete absence of it. However women with MRKH syndrome may have normally functioning ovaries.

Due to their normal looking appearance, parents discover that there is something wrong only when their daughter fails to get her first menstrual period. Another way that this syndrome is discovered is when they try and have a failed vaginal intercourse.

In rare instances, patients afflicted with MRKH syndrome may have an involvement of kidneys, urinary tract, skeletal and cardiac systems.

Diagnosis

This condition is most often diagnosed when a young woman fails to get her first menstrual period. A proper examination performed at that time by the gynecologist is likely to reveal the deformed vagina and uterus. Further diagnosis is aided by an Ultrasound, MRI or a Laparoscopic Surgery.

Treatment

MRKH syndrome can be treated either surgically or non-surgically. Non-surgical treatment uses dilators to slowly create a new vagina. While this works in some patients, the other patients may become non-compliant to this treatment as this takes a long time stretching up to a few years to achieve a normal vagina says Dr Rajesh Vasu – HOD & Sr. Consultant Plastic & Aesthetic Surgeon; Fellowship in Aesthetic Surgery at Continental Hospitals.

There are several surgical options for increasing vaginal depth or creating a vagina. The Vecchietti procedure though appearing simple, is fraught with failures and non-compliance.

Vaginoplasty

Women can also undergo a surgical vaginoplasty. A vaginoplasty involves a creation of a cavity and then lining it with a tissue to make a neovagina. The tissues used for lining usually use a skin graft or a cheek lining in some cases. This technique will need the patients to dilate the neovagina to maintain the cavity and prevent it from shrinking and collapsing in the long run.

With further surgical advances, bowel vaginoplasty has become the standard of care wherein a vascularized conduit of large or small intestine is swung down to create the neovagina. As this tissue is vascularized it doesn't involve any long term maintenance in the long run like the use of dilators. And as the tissue used is the bowel, issues like lubrication aren’t a cause of worry due to the inherent potential to secrete mucus.

Providing psychological support is very important for this subset of patients as this can cause serious problems causing lack of self-esteem. Patients may need psychological support not only from their parents and relatives but also from the medical community dealing with this issue. The services of a gynecologist, Plastic Surgeon go a long way in treating this complex problem and bringing back a smile to the young women in her marital life.

Is it possible to bear children?

If one is born with an incomplete vagina but have a normal size uterus, it is likely that she will be able to become pregnant and carry a baby. If one were born without a uterus or if the uterus is tiny, then she will not be able to carry a pregnancy. Since the ovaries are normal and make eggs, these can be fertilized with partner’s or a donor’s sperm. Adoption is another choice for some couples.

Surrogacy: Someone else such as your sister, friend, or another person you choose, could potentially be a gestational carrier. Gestational carriers are women who agree to carry a pregnancy for a couple.

Also Read: Medical Breakthroughs in 2018: Designer Babies, Child From Dead Woman’s Womb!