Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome—also known as Müllerian agenesis or uterovaginal agenesis—is a rare condition that causes underdevelopment of the uterus, cervix and upper vagina. MRKH patients are born with this condition (congenital), which is diagnosed in childhood or adolescence. MRKH syndrome affects 1 in 4,500 people assigned female at birth.
There are two MRKH syndrome types.
In MRKH Type 1, MRKH syndrome includes underdevelopment of the uterus, cervix and upper vagina.
In MRKH Type 2, the same underdevelopment of these structures occurs. But people with MRKH Type 2 also have other differences in the kidneys, spine and, on rare occasions, hearing and the heart. For example, these patients may have just one kidney or a kidney in a unique location such as the pelvis. Patients may also have conditions like scoliosis.
The cause of MRKH syndrome is unknown in many cases. Research into the specific gene changes that lead to MRKH syndrome continues to grow.
The genetics of MRKH are difficult to study due to its impact on the development of the uterus, which leads to the inability to carry a pregnancy.
One of the first symptoms of MRKH syndrome is a lack of a menstrual period (amenorrhea) by age 15. People with MRKH are affected by uterine factor infertility due to the underdevelopment of the uterus and upper vagina. As a result, people with MRKH do not become pregnant spontaneously and cannot carry a pregnancy. But since they have normal ovaries, they can have biological children through other means. MRKH also impacts the length of the vaginal canal.
While people with MRKH do not have periods, some may have abdominal pain. This can happen due to cramping of the underdeveloped uterine remnant muscle or due to a buildup of endometrial lining within the underdeveloped uterine remnant muscle. This type of pain occurs monthly and can worsen over time. People with MRKH ovulate and may also have cramping around the time of ovulation.
Around half of people with MRKH have related congenital (present since birth) conditions. Those with MRKH may also have:
Doctors diagnose most people with MRKH around 15 years old when they do not start having periods after an otherwise normal puberty.
To diagnose MRKH syndrome, doctors will take a health history and complete an exam. They will do bloodwork to look at puberty hormones. They will also conduct pelvic imaging, including an ultrasound or magnetic resonance imaging (MRI). Genetic testing will be done to confirm female sex chromosomes.
Once a diagnosis of MRKH syndrome is made, doctors also will screen for kidney, spine and hearing variations.
Because MRKH syndrome impacts the development of the uterus, fallopian tubes, cervix and upper vagina, people experience infertility (uterine factor) and a shortened vaginal canal.
Treatment for MRKH should extend beyond gynecologic care to include psychosocial support related to the diagnosis, pelvic health care including screening for bowel/bladder symptoms, and relevant care related to associated congenital changes (such as renal, spine, cardiac and hearing variations). This may also include sexual health counseling, grief counseling and family support.
While MRKH impacts the development of the uterus and ability to conceive and carry a pregnancy, it does not impact the health of the ovaries or eggs (oocytes). People with MRKH may choose to build a family through adoption or through in vitro fertilization (IVF) with a gestational carrier. There are also clinical trials for uterine transplantation that may allow people with MRKH to carry up to two pregnancies.
While MRKH impacts the development of the upper vagina and cervix, the lower vaginal canal and vulva are not affected. Due to underdevelopment of the upper vagina, MRKH affects the length of the vaginal canal. Some people with MRKH choose to lengthen their vagina through dilation or surgery.
There is no rush to decide whether to pursue vaginal lengthening—the timing and treatment are entirely up to the person.
Many people with MRKH chose not to lengthen the vaginal canal.
Most often, people with MRKH do not need to have surgery to create a functional vaginal canal.
For people with MRKH who choose vaginal lengthening, the first line of treatment is dilation. Dilation is recommended first because it is low risk.
Most dilation is done at home following education and coaching from the care team. The vaginal canal can be lengthened through use of a dilator, or through coital (sex) dilation.
For those who choose to pursue treatment for vaginal lengthening, the following summarizes many of the options available:
Dilation (using a dilator to stretch and lengthen the vaginal tissue) is most successful with consistent dilation. It can take three to 12 months. Vaginal dilation is successful for more than 90% of people who do it consistently.
Our gynecologist, pelvic health physical therapist, and clinical psychologist provide coaching and techniques to support people with MRKH through the dilation process to improve their experience and outcomes, including methods to minimize pain related to the pelvic floor muscles.
Patients who do not achieve functional vaginal length with self-dilation may consider surgical options.
Surgery is considered the second line option for vaginal lengthening due to its risks.
All surgical options require dilation/use of a vaginal stent during recovery after surgery. People considering surgery must be comfortable with the process of dilation prior to surgery. At Cincinnati Children’s, we offer several types of surgical procedures for vaginal lengthening. The umbrella term for these procedures is vaginoplasty. The goal is to lengthen the vagina surgically.
The Vecchietti is an inpatient surgery involving two procedures under anesthesia. The first procedure involves a laparoscopy, cystoscopy and placement of a small dilator against the vaginal opening.
During the procedure, the vaginal dilator is attached by strings (sutures) through the abdomen. The strings are then attached to a traction device that is placed on the abdomen. Patients stay in the hospital and must lay flat in bed during this time. Daily adjustments to the traction device create tension on the dilator to gradually lengthen the space over about a week. The constant tension creates a longer vaginal canal.
After about one week, a second procedure is needed to remove the abdominal traction device. The small dilator is replaced by a wearable dilator/stent. The vaginal dilator/stent is worn during recovery for several weeks to allow healing of the vaginal canal and to help prevent scarring.
A buccal graft vaginoplasty is an inpatient surgery involving two procedures under anesthesia. The buccal graft uses tissue from the patient’s mouth (inner cheek) to line the surgically created vaginal space and is most helpful to provide a small amount of additional vaginal length after dilation.
During the first procedure, incisions (cuts) are made inside the cheek to get a small amount of buccal mucosa (lining of the inside of the cheek), which is then wrapped around a dilator. It is placed inside a new vaginal space that is created during surgery between the bladder and rectum. Patients stay in the hospital and lay flat in bed wearing the vaginal stent, which is held in place by stitches.
After about one week, a second procedure is needed to remove the vaginal stent and replace it with a wearable dilator/stent. The vaginal dilator/stent is worn during recovery for several weeks to allow healing of the vaginal canal and to help prevent scarring (stenosis).
A McIndoe vaginoplasty is an inpatient surgery involving two procedures under anesthesia. During the first procedure, a skin graft is taken from the buttocks, thigh or another area on the person’s body. The skin graft is then wrapped around a dilator. It is placed inside a new vaginal space that is created during surgery between the bladder and rectum. Patients stay in the hospital and lay flat in bed wearing the vaginal stent, which is held in place by stitches.
After about one week, a second procedure is needed to remove the vaginal stent and replace it with a wearable dilator/stent. The vaginal dilator/stent is worn during recovery for several weeks to allow healing of the vaginal canal and to help prevent scarring (stenosis).
Because skin is used to line the space, there are potential long-term risks of skin cancers or hair growth. Another risk is vaginal dryness, as skin is not self-lubricating.
This procedure uses a segment of bowel to line the surgically created vaginal space. Because bowel is used to line the space, there are potential long-term risks of bowel disease (polyps, cancers, inflammatory bowel disease/colitis).
Often, patients who chose to have a bowel graft vaginoplasty have bothersome discharge and odor. Because the bowel graft is taken from the patient’s body, there is also risk of scarring of the graft site (bowel obstruction or stricture) that can result in the need for additional surgery.
Regardless of the type of vaginoplasty surgery, consistent dilation after surgery is needed to maintain the vagina. This involves wearing a vaginal dilator/stent initially and later using a dilator to maintain the canal. The goal is to prevent scarring/narrowing of the vaginal canal, particularly in the initial healing phase. Once healed, consistent dilation (or sexual intercourse) is important to maintain vaginal length.
There is no standard surgical option. Options vary by a surgeon’s expertise. Associated congenital changes in the kidneys or anatomy can also impact which vaginoplasty would be safest for a patient. It’s important that dilation or surgical lengthening is only done when the person chooses to and feels ready. Choosing not to lengthen the vagina is also an option.
MRKH syndrome treatment also includes routine gynecologic care, including breast, vulvar and vaginal exams, screening for gynecologic and pelvic floor conditions (dysmenorrhea, ovulation pain, endometriosis, urinary incontinence and prolapse), and testing for sexually transmitted infection (STI).
People with MRKH syndrome can expect to live a full life. There is currently no data on early menopause since ovarian function is not affected by MRKH.
The MRKH Care Center at Cincinnati Children’s offers coordinated, compassionate care for patients with MRKH syndrome. Our multidisciplinary team provides personalized treatment, education and emotional support—helping patients and families navigate diagnosis and thrive at every stage of their journey.
Last Updated 03/2026
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