Mayer Rokitansky Kuster Hauser (Mrkh) Syndrome
Description
A congenital abnormality consisting of failure of all or part of the vagina to develop as well as lack of development of the uterus and fallopian tubes. The disorder occurs in 1 in 4000-5000 women and is the second most common cause of primary amenorrhea (no menstruation). The ovaries that produce estrogen and other female hormones are present and the outward appearance of the affected women is otherwise normal. The disorder is usually diagnosed when a patient presents in her teenage years with a failure to start menstruating. Surgery can usually improve the symptoms of painful sexual intercourse but most patients are not able to become pregnant. Experimental uterine transplant procedures are being investigated in Europe.
Symptoms
Lack of a menstrual cycle (amenorrhea), painful sexual intercourse, inability to become pregnant (infertility).
Tests
A history and physical exam will be done. A gynecological exam will usually demonstrate the abnormal vagina and lack of a cervix. A pelvic ultrasound can detect the absence of a uterus, or a only partially formed uterus.
- Ultrasound
Treatment
Surgery can repair the abnormal uterus and enable normal sexual intercourse. Rarely the ability to become pregnant can be restored.
Related Videos
Share Your Experience
Do you have experience with this disease? Do you or someone
you know suffer from Mayer Rokitansky Kuster Hauser (MRKH) syndrome?
Please share you experience with other members of the iTriage community.