Oncology and Fibroids
In which of the following conditions ovaries function normally (AIIMS Nov. 2011)
|A||Turner s syndrome|
|B||Mayer rokitansky kuster hauser Syndrome|
|C||Androgen insensitivity syndrome|
Mayer rokitansky kuster hauser Syndrome
Müllerian agenesisis a congenital malformation in women characterised by a failure of the Müllerian ducts to develop, resulting in absent uterus and variable malformations of the vagina. It is the second most common cause of primary amenorrhea.
Signs and symptoms:
1. A woman with this condition is hormonally normal, that is she will enter puberty with development of secondary sexual characteristics including thelarche and adrenarche. Her chromosome constellation will be 46,XX. Ovulation usually occurs.
2. Typically, the vagina is shortened and intercourse may in some cases be difficult and painful. Medical examination supported by gynecologic ultrasonography demonstrates a complete or partial absence of the cervix, uterus, and vagina.
3. Ovaries are present and function normally. This syndrome is characterised by congenital absence of vagina, primary amenorrhoea, rudimentary cornua uteri or absent uterus and morphologically normal ovaries and rudimentary Fallopian tubes (or fallopian tubes may also be absent) situated on the pelvic sidewall.
4. Normal ovulation; normal breast development; normal body and hair. The woman is amenorrhoeic and infertile. Frequently associated with urinary tract anomalies, skeletal abnormalities, congenital heart conditions, and inguinal hernia.
5. Women with MRKH typically discover the condition when, during puberty years, the menstrual cycle does not start.
6. Other, less common symptoms include kidney problems, hearing loss, and bone malformations. Renal anomalies occur in 25-35% of females with mullerian agenesis.
7. The estimated prevalence is 1 in 5000 women.
8. Although there are treatments to increase the comfort in sexual intercourse, there are none to let the woman become pregnant. Since the women do have ovaries, women with this condition can have genetic children through IVF & Surrogacy.
Ovarian dysgenesis/Turners syndrome is the MC cause of primary amenorrhea
Swyer syndrome, or XY gonadal dysgenesis, is a type of hypogonadism in a person whose karyotype is 46, XY. The person is externally female with streak gonads, and left untreated, will not experience puberty.