Header banner

Monday, May 23, 2016

NO VAGINA, NO WOMB IS CALLED ?



Hehe....see stuff oooo.....Müllerian agenesis, also called Mayer-Rokitansky-Küster-Hauser syndrome or MRKH, named after August Franz Joseph Karl Mayer, Carl Freiherr von Rokitansky, Hermann Küster, and G. A. Hauser, is a congenital malformation characterized by a failure of the Müllerian duct to develop, resulting in a missing uterus and variable degrees of vaginal hypoplasia of its upper portion. Müllerian agenesis (including absence of the uterus, cervix and/or vagina) is the etiology in 15% of cases of primary amenorrhoea


An individual with this condition is hormonally normal; that is, they will enter puberty with development of secondary sexual characteristics including thelarche and adrenarche (pubic hair). Their chromosome constellation will be 46,XX. Ovaries are intact and ovulation usually occurs. 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.

If there is no uterus, people with MRKH cannot carry a pregnancy. However, it is possible for them to have genetic offspring by in vitro fertilization (IVF) and surrogacy. People with MRKH typically discover the condition when, during puberty years, the menstrual cycle does not start (primary amenorrhoea). Some find out earlier through surgeries for other conditions, such as a hernia.

Treatment

A number of treatments have become available to create a functioning vagina, yet in the absence of a uterus currently no surgery is available to make pregnancy possible. Standard approaches use vaginal dilators and/or surgery to develop a functioning vagina to allow for penetrative sexual intercourse. A number of surgical approaches have been used. In the McIndoe procedure,] a skin graft is applied to form an artificial vagina. After the surgery, dilators are still necessary to prevent vaginal stenosis. The Vecchietti procedure has been shown to result in a vagina that is comparable to a normal vagina in patients. In the Vecchietti procedure, a small plastic “olive” is threaded against the vaginal area, and the threads are drawn through the vaginal skin, up through the abdomen and through the navel using laparoscopic surgery. There the threads are attached to a traction device. The operation takes about 45 minutes. The traction device is then tightened daily so the olive is pulled inwards and stretches the vagina by approximately 1 cm per day, creating a vagina approximately 7 cm deep in 7 days, although it can be more than this.[12] Another approach is the use of an autotransplant of a resected sigmoid colon using laparoscopic surgery; results are reported to be very good with the transplant becoming a functional vagina.]
Uterine transplantation has been performed in a number of people with MRKH, but the surgery is still in the experimental stage. Since ovaries are present, people with this condition can have genetic children through IVF with embryo transfer to a gestational carrier. Some also choose to adopt. In October 2014 it was reported that a month earlier a 36-year-old Swedish woman became the first person with a transplanted uterus to give birth to a healthy baby. She was born without a uterus, but had functioning ovaries. She and the father went through IVF to produce 11 embryos, which were then frozen. Doctors at the University of Gothenburg then performed the uterus transplant, the donor being a 61-year-old family friend. One of the frozen embryos was implanted a year after the transplant, and the baby boy was born prematurely at 31 weeks after the mother developed pre-eclampsia.
Promising research include the use of laboratory-grown structures, which are less subject to the complications of non-vaginal tissue, and may be grown using the person's own cells as a culture source.The recent development of engineered vaginas using the patient's own cells has resulted in fully functioning vaginas capable of menstruation and orgasm in a number of patients showing promise of fully correcting this condition in some of the sufferers. other problems are discussed below
Developmental disorders of the female reproductive tract are problems in a baby girl's reproductive organs that occur while she is growing in her mother's body.
Female reproductive organs include the vagina, ovaries, uterus, and cervix.

Causes

A baby starts to develop its reproductive organs between weeks 4 and 5 of pregnancy. This development continues until the 20th week of pregnancy.
The development is a complex process. Many different things can interrupt the process. How severe your baby's problem is depends on when the interruption occurred. In general, the earlier development problems occur in the womb, the more serious the problem.
Problems in the development of a girl's reproductive organs may be caused by:
  • Broken or missing genes (genetic defect)
  • Use of certain drugs during pregnancy
For example, some babies may have a genetic defect that prevents their body from producing a substance called 21-hydroxylase. If a developing baby girl lacks this substance, she will be born with a uterus, ovaries, and fallopian tubes, but her external genitals will look like those found on boys. See: Congenital adrenal hyperplasia
Certain drugs that the mother takes can pass into the baby's bloodstream and interfere with organ development. One drug known to do this is diethylstilbestrol (DES). Doctors once prescribed this medicine to pregnant women to prevent miscarriage and early labor. However, scientists learned that baby girls born to women who took this drug had an abnormally shaped uterus. The drug also increased the daughters' chances of developing a rare form of vaginal cancer.
Sometimes, a developmental disorder can be seen as soon as the baby is born. It may cause life-threatening conditions in the newborn. Other times, the condition is not diagnosed until the girl is older.
The reproductive tract develops close to the urinary tract and kidneys. It also develops at the same time as several other organs. As a result, developmental problems in the female reproductive tract sometimes occur with problems in other areas, including the urinary tract, kidneys, and lower spine.
Developmental disorders of the female reproductive tract include intersex and ambiguous genitalia. See the specific articles for information on these conditions.
Other developmental disorders of the female reproductive tract include:
  • Cloacal abnormalities -- The cloaca is a tube-like structure. In the early stages of a baby's development, the urinary tract, rectum, and vagina all empty into this one tube. But, later in development, the three areas separate and have their own openings. If the cloaca persists as a baby girl grows in the womb, all the openings do not form. For example, a baby may be born with only one opening on the bottom of the body near the rectal area. Urine and feces cannot drain out of the body. This can cause severe pain, stomach swelling, and breathing problems. Some cloacal abnormalities may cause a baby girl to look like she has a male's penis. These birth defects are rare.
  • Problems with outer (external) genitals -- Developmental problems may lead to a swollen clitoris or fused labia (when the folds of tissue around the opening of the vagina are joined together). Most other problems of the outer genitals are related to intersex or ambiguous genitalia.
  • Imperforate hymen -- The hymen is a thin tissue that partly covers the opening to the vagina. An imperforate hymen completely blocks the vaginal opening. This often leads to painful swelling of the vagina. Sometimes, the hymen has only a very small opening or tiny small holes. This problem may not be discovered until puberty. Some baby girls are born without a hymen.
  • Ovarian problems -- A baby girl may have an extra ovary, extra tissue attached to an ovary, or structures called ovotestes that have both male and female tissue.
  • Uterus and cervix problems-- A baby girl may be born with an extra cervix and uterus, a half-formed uterus, or a blockage of the uterus. Usually, girls born with half a uterus and half a vagina are missing the kidney on the same side of the body.
  • Vaginal problems-- A baby girl may be born without a vagina or have the vaginal opening blocked by a layer of cells that are higher up in the vagina than where the hymen is. A missing vagina is most often due to Mayer-Rokitansky-Küster-Hauser syndrome. In this syndrome, the baby is missing part or all of the internal reproductive organs (uterus, cervix, and fallopian tubes). Other abnormalities include having two vaginas or a vagina that opens into the urinary tract, a heart shaped uterus, and a uterus with a wall in the middle of the cavity

Symptoms

Symptoms vary according to the specific problem. They may include:
  • Breasts do not grow
  • Cannot empty the bladder
  • Lump in the stomach area, usually due to blood or mucus that cannot flow out
  • Menstrual flow that occurs despite using a tampon (a sign of a second vagina)
  • Monthly cramping or pain, without menstruation
  • No menstruation (amenorrhea)
  • Pain with sex
  • Repeated miscarriages or preterm births (may be due to an abnormal uterus)

Exams and Tests

The doctor may notice signs of a developmental disorder right away. Such signs may include:
  • Abnormal vagina
  • Abnormal or missing cervix
  • Bladder on the outside of the body
  • Genitals that are hard to identify as a girl or boy (ambiguous genitalia)
  • Labia that are stuck together or unusual in size
  • No openings in the genital area or a single rectal opening
  • Swollen clitoris
The belly area may be swollen or a lump in the groin or abdomen may be felt. The doctor may notice the uterus does not feel normal.
Tests may include:


No comments:

Post a Comment