| Category | Mullerian Agenesis |
Mullerian Agenesis
Mullerian agenesis, also known as Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome), is a congenital malformation affecting the female reproductive system. It occurs due to abnormal development of the Müllerian ducts, which are embryonic structures that normally form the uterus, fallopian tubes, cervix, and upper vagina. In individuals with Mullerian agenesis, these structures are either absent or incompletely formed. This condition causes the vagina and uterus to be underdeveloped or absent, although external genitalia are normal. Affected individuals usually do not have menstrual periods due to the absence of a uterus. Often, the first noticeable sign of MRKH syndrome is that menstruation does not begin by age 16 (primary amenorrhea). People with MRKH syndrome have a female chromosome pattern (46,XX) and normally functioning ovaries. . Dr. (Smt) Sanju Agrawal is the Best Mullerian Agenesis Doctor in Indore MP!
Symptoms Of Mullerian Agenesis
Mullerian agenesis often goes unnoticed until females reach their teens, but don't menstruate (amenorrhea). Other signs of puberty usually follow typical female development.
Vaginal agenesis may have these features:
The genitals look like a typical female.
The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located.
There may be no uterus or one that's only partially developed. If there's tissue lining the uterus (endometrium), monthly cramping or chronic abdominal pain may occur.
The ovaries typically are fully developed and functional, but they may be in an unusual location in the abdomen. Sometimes the pair of tubes that eggs travel through to get from the ovaries to the uterus (fallopian tubes) are absent or do not develop typically.
Types of Mullerian Agenesis
MRKH Type 1
The main distinguishing feature of MRKH Type 1 is that only the uterus and upper vagina are underdeveloped or impacted. All other organs remain unaffected. Common symptoms of Type 1 Mullerian Agenesis include:
Diminished vaginal depth and width
Painful sexual intercourse
MRKH Type 2
In MRKH Type 2, other organs are affected. The signs and symptoms of this form of MRKH syndrome include:
Kidney complication or failure
Skeletal malformations
Slight hearing loss
Heart defects
How is Mayer-Rokitansky-Küster-Hauser syndrome diagnosed?
Healthcare providers diagnose most cases of MRKH syndrome when a teenager doesn’t get their first menstrual period.
The first step in diagnosing the condition is to perform a physical exam. Your healthcare provider will insert a gloved finger into your vagina to measure its depth and width. MRKH is associated with a shortened vagina, which your provider will likely discover through this exam. They’ll order imaging tests like an ultrasound or magnetic resonance imaging (MRI) to see if your uterus, fallopian tubes, kidneys or other organs are affected. Your provider may order blood tests to check hormone levels.
MRKH is a complex condition with several different treatment options. These options include surgical and nonsurgical procedures based on the individual characteristics of a woman’s condition. While some treatments have been well-studied than others, no MRKH syndrome treatment is 100% guaranteed to be effective.
Surgical treatments include:
Vaginoplasty – Surgery to make a functioning vagina, for example, by using a skin graft.
Uterus transplant
Nonsurgical treatments include:
Self-dilation – This treatment involves a female patient using a series of incrementally larger dilators (small, round rods) to expand her existing vagina over the course of several months.
Vaginal dilation through intercourse – Frequent intercourse may also help some women with MRKH (and with willing partners) to expand their existing vaginas over time. However, artificial lubricant is often needed as side effects may be discomfort and bleeding.
FAQ
Do I need a yearly GYN exam?
General gynaecologic care is important but your visit may vary depending on your symptoms and history. Pelvic examinations may be important if there are concerns about complications or symptoms such as vaginal narrowing or discharge. Yearly screening may also be important if you are sexually active and therefore at risk of sexually transmitted infections.
Is it harmful if I wait to create a vagina until I’m older?
The choice to have treatment, how, when, etc., is UP TO YOU! Like any other important decision, it’s essential that you get all the information before you make the decision to have treatment. Remember, you can create a vagina AT ANY TIME or not at all and still feel complete!
What are the health complications of MRKH syndrome?
People with type 2 MRKH syndrome may have issues with other organs like their kidneys, spine or heart. Other health complications common in people with type 2 MRKH syndrome are:
Vertebral issues.
Scoliosis.
Renal anomalies like renal fusion or renal agenesis (missing one or both kidneys).
Increase risk for kidney stones, urinary tract infections and ureteral obstructions.
Hearing loss.
Heart problems.
How can I prevent MRKH?
There is no way to prevent MRKH syndrome. It can occur in people with no family history of the condition, or it can be genetic (run in your family). No one gene causes MRKH syndrome.
Is there any treatment for MRKH syndrome?
Treatment for MRKH depends on your goals and symptoms. There are surgical and nonsurgical treatment options, including vaginoplasty, vaginal dilation and a uterine transplant.
How is MRKH syndrome diagnosed?
Diagnosis typically involves a physical exam, pelvic ultrasound, and possibly MRI scans. Hormone testing may also be done to assess ovarian function.
Can people with MRKH syndrome get pregnant?
People with MRKH syndrome cannot carry a pregnancy naturally due to the absence of a uterus. However, they may still be able to have biological children through assisted reproductive technologies (ART) like egg retrieval and gestational surrogacy.
Can I still get sexually transmitted infections?
YES! Please remember that you should always use barrier protection such as an internal (female) or external (male) condom every time you have sex to prevent HIV and other STI.
A pelvic ultrasound is usually the first test to check to see if a uterus or womb is present. This test can also confirm that you have two ovaries and two kidneys. Sometimes a very tiny uterus can be seen. A tiny uterus is called a “uterine horn or remnant”. You may need to have an MRI so that your doctor can see your female organs in more detail.
If you were born with an incomplete vagina but have a normal size uterus, it is likely that you will be able to become pregnant and carry a baby. If you were born without a uterus or if your uterus is tiny, you will not be able to carry a pregnancy. Since you have ovaries and make eggs, one of your eggs can be fertilized with your partner’s or a donor’s sperm.
How to book an appointment with Dr. Sanju Agrawal for MRKH Treatment in Indore?
Struggling with Müllerian Agenesis? Find Hope and Support with Dr. (Smt) Sanju Agrawal Best Müllerian Agenesis Doctor in Indore MP in Indore! MRKH affects many women, leaving them with questions and concerns about reproductive health and well-being. Dr. Sanju Agrawal is a leading gynaecologist and obstetrician with an illustrious background holding an MBBS, M.S., and D.N.B. She has extensive experience in managing MRKH, utilizing the latest advancements in surgical and non-surgical approaches. Each woman's journey is unique; she tailors treatment plans to your individual needs and goals. Don't let MRKH define your future. Take control of your health and well-being. Schedule an appointment with Dr. Sanju Agrawal today!
