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Reproductive Physiology, Endocrinology, and Infertility

Question
52 out of 56
 

Which of the following is true about Mayer Rokitansky Küster Hauser? (AIIMS May 2009)



A Ovary uterus tubes absent

B Uterus absent, tubes and ovary present

C Uterus present, tubes and ovary absent

D Uterus, tubes and ovary present

Ans. B

Uterus absent, tubes and ovary present

1. Müllerian agenesis is 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.

2. It is the second most common cause of primary amenorrhea.

3. The condition is also called MRKH or Mayer-Rokitansky-Küster-Hauser Syndrome, named after August Franz Joseph Karl Mayer, Carl Freiherr von Rokitansky,Hermann Küster, and G.A.Hauser.

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.

2. Typically, the vagina is shortened and intercourse is difficult and painful.

3. Gynecologic Ultrasonography demonstrates a complete or partial absence of the cervix, uterus, and vagina.

4. This syndrome is characterized by congenital absence of vagina, primary amenorrhea, 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.

5. Normal ovulation; normal breast development; normal body and hair. The woman is amenorrheic and infertile.

6. Frequently associated with urinary tract anomalies, skeletal abnormalities, congenital heart conditions, and inguinal hernia.

7. Due Since there is no uterus, women with MRKH cannot carry a pregnancy.

8. Women with MRKH typically discover the condition when, during puberty years, the menstrual cycle does not start.

Treatment

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 with embryo transfer to a gestational carrier. Some women also choose to adopt.

Reproductive Physiology, Endocrinology, and Infertility Flashcard List

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