Center for Congenital Anomalies of the Reproductive Tract
Congenital Anomalies of the Vagina
The Center for Anomalies of the Reproductive Tract at Boston Children's Hospital treats various congenital anomalies of the vagina, including:
- transverse vaginal septum
- vertical or complete vaginal septum
- vaginal agenesis
- agenesis of the lower vagina
obstructed hemi-vagina with ipsilateral renal agenesis (OVIRA)
Transverse vaginal septum
A transverse vaginal septum is a horizontal "wall" of tissue that has formed during embryologic development and essentially creates a blockage of the vagina. It can occur at many different levels of the vagina.
A large percentage of women with a transverse vaginal septum have a small hole, or fenestration, within the transverse vaginal septum so they may have regular menstrual periods, although the periods may last longer than the normal 4-7 day cycle.
During an examination, a woman may find that she has a normal hymeneal opening and a lower vagina. As one enters the vagina, there will be a fibrous wall of tissue which is the transverse vaginal septum. Above the transverse vaginal septum, there is a normal vagina.
If there is a complete obstruction without a hole within the transverse vaginal septum, when a woman is having menstrual cycles there will be a blockage of blood which will collect in the upper vagina.
A transverse vaginal septum will most likely require a surgical procedure to resect the fibrous septal tissue.
The gynecologist who performs the surgery must be familiar with the risk of complications of a transverse vaginal septum because there can be stenosis or scarring of the vagina in the area of the transverse vaginal septum which can create an "hour-glass" effect in the vagina. After resection of the transverse vaginal septum, the woman may be required to use a vaginal dilator in order to avoid this "hour-glass" effect of the healing process.
Once the transverse vaginal septum has been surgically corrected, the woman should be able to have normal sexual relations and should also have no long-term effects on reproductive function and the ability to have a child.
Vertical or complete vaginal septum
The vagina normally forms as two tubes meeting in the midline with fusion resulting in the creation of a single vagina. At times, there are fusion abnormalities which result in a complete vaginal septum which is a wall running vertically up the vagina, essentially creating two vaginas.
A woman may identify that she has a complete vaginal septum when she uses a tampon and sees that she still has blood coming from the vagina. She may, therefore, elect to use two tampons - one in each vagina. In other cases, she may notice that the vaginal orifices are too small to insert a tampon.
Other women have no symptoms from a complete vaginal septum. With sexual activity, for example, the vaginal septum may be torn creating one vagina. Other women notice that during sexual activity a penis will go towards one side or the other due to the fact that one side of the vagina may be larger than the other side.
A complete vaginal septum can be surgically resected. During a resection of a complete vaginal septum, the entire fibrous wall of the septum will be removed. The gynecologist should be aware that if the entire septum is not removed there may be a fibrous band of tissue running on the top and bottom of the vagina. This could result in discomfort with sexual activity. Therefore, the entire septum is removed and the normal vagina on both sides of the preexisting septum are brought together to create a normal texture to the vagina.
Women with a complete vaginal septum also have duplication of the upper reproductive tract and have two uteri and two cervices (see congenital anomalies of the uterus and congenital anomalies of the cervix).
Vaginal agenesis, or absence of the vagina, is a congenital disorder of the female reproductive tract. It affects approximately 1 in every 5,000 female infants. The cause of vaginal agenesis is unknown. A woman with complete vaginal agenesis may have this abnormality based on the fact that the vagina did not grow during embryologic development - this is called Mayer-von Rokitansky-Küster-Hauser's (MRKH) syndrome.
There are many variations of this syndrome. A woman may have no vagina and no uterus but have normal ovaries. Or she may have no vagina and may have a single midline uterus and no cervix.
Women with a midline uterus and vaginal agenesis have options for correction of this problem which include:
Stopping the retrograde menses (backward period): women take an oral contraceptive pill continuously. Their uterus is preserved in the midline so they can potentially carry a pregnancy by using assisted reproductive technologies and a planned abdominal delivery (cesarean section).
- Surgery: women undergo a procedure to create a communication between a vagina which is created and the upper uterus. There have been cases where this has been successful, although there have been numerous cases where this has resulted in an infection and the need for a hysterectomy, and a few reported deaths.
At the Center for Congenital Anomalies of the Reproductive Tract, we recommend that a woman with vaginal agenesis with a midline uterus and no cervix goes through a procedure for creating a functional vagina for sexual relations and maintains her uterus by taking a continuous dose of oral contraceptive pills to suppress retrograde menses (backward period) and the risk of endometriosis.
Women with vaginal agenesis can also have small rudimentary uterine horns which are lateral to the midline. (Uterine horns are small uteri that did not form into a single midline regular size uterus. Normally, the uterus always starts as two and comes together, fuses and becomes one uterus.) These uteri can also function, if they contain an endometrial stripe. Women with rudimentary uterine horns can also be managed in a similar fashion to women with a single midline uterus.
Women with vaginal agenesis from Mayer-von Rokitansky-Küster-Hauser's (MRKH) syndrome have a karyotype of 46, XX which is the most common for women. Obtaining a karyotype, therefore, can also be helpful in making a definitive diagnosis; additionally a testosterone level can also be helpful in making a diagnosis. Women with MRKH syndrome will have a testosterone level in the "normal female range" and women with androgen insensitivity will have a testosterone level in the "normal male range."
Creating a vagina
Women with vaginal agenesis will need to create a vagina to have normal sexual function. There are numerous options for creation of a vagina, including:
Vaginal dilators: the dilator is pushed against the area where the vagina should be located; with constant pressure on a daily basis, a woman can create a functional vagina. It should be noted that a woman with vaginal agenesis can have normal orgasmic function as the clitoris and the external genitalia are formed normally. The vagina should have normal vaginal lubrication. The process can take between six months and two years, depending on the frequency of the utilization of the dilators.
Skin grafts: this is called a McIndoe procedure. A split thickness skin graft is taken from the buttock and a space is created for the placement of vaginal mold with the skin graft attached to it. This is a surgical procedure and requires hospital bed rest for seven days. At the end of the seven-day period, the woman is taken back to the operating room for removal of the mold which is used to create the vagina. Once the mold is removed, the woman uses a vaginal dilator. The skin graft can result in a normal, functional vagina. Some women find that they need to utilize water-based lubricants for sexual activity, as the skin of the newly created vagina may be dry.
- Bowel use: the bowel is used to create a vagina. This procedure requires a laparotomy and a resection of a piece of bowel with a reapproximation of the intestine. The bowel is placed in the area which is created for the vagina. Some women with a "bowel vagina" complain of a chronic vaginal discharge as the gastrointestinal mucosa of the bowel constantly produces mucus so they must always wear a pad. Concerns also exist about the risk of sexually transmitted diseases because the bowel is a poor protective barrier against STD's.
MRKH (Mayer-von Rokitansky-Küster-Hauser's Syndrome) is a congenital condition of the female reproductive system that affects approximately 1 out of every 5,000 females. Girls diagnosed with MRKH have vaginal agenesis, which refers to an absent or incomplete vagina. The uterus is also very small or absent. It is important to understand that young women with this syndrome are genetic females. They have normal ovaries and will experience puberty without having periods. MRKH is a syndrome that may or may not be associated with renal (kidney), skeletal and hearing problems.
Agenesis of the lower vagina
A woman can be born without a lower vagina. She may have a normal uterus, cervix and upper vagina but there is a blockage with the absence of the lower vagina. If there is agenesis of the lower vagina and there is a normal, functional upper vagina and uterus, the upper vagina will fill with blood during menstruation. This can create a large pelvic mass which is the distended upper vagina.
The Center performs a pull-through vaginoplasty when the upper vagina is filled with blood so that the upper vagina can be brought down to the area where a normal vaginal opening should occur.
If the pull-through vaginoplasty is not performed when the upper vagina is filled with blood, there may not be enough vaginal tissue to bring it down to the area of the hymeneal opening. If this is the case, we may use a skin graft or section of bowel in order to create a normal length to the vagina.
Once a normal vagina has been created, a vaginal dilator may need to be worn in order to decrease the risk of scar tissue. Once corrected, a woman with agenesis of the lower vagina should have normal reproductive function and fertility
Obstructed hemi-vagina with ipsilateral renal agenesis (OVIRA)