VOLGOGRAD STATE MEDICAL UNIVERSITY

DEPARTMENT OF GYNAECOLOGY

VAGINAL AGENESIS/HYPOPLASIA

NIK MAWADDAH NIK DIN

5TH YEAR, FACULTY OF GENERAL MEDICINE

GROUP 33

INTRODUCTION

Vaginal agenesis and vaginal hypoplasia are part of a much broader group of congenital

malformations of the female reproductive tract. Congenital means “present at birth”.

Agenesis means “absence of” or “failure to develop”. Hypoplasia means “less

development than usual”.

Vaginal agenesis occurs in approximately 1 in every 5,000 to 7,000 female births. It

results from a problem during the very early development of the reproductive system of

a fetus while in her mother’s uterus.

There are many different conditions that may cause such a developmental problem and

lead to vaginal agenesis. By far the most common, representing more than 90% of

patients with vaginal agenesis, is Rokitansky-Mayer-Küster-Hauser syndrome, named

after the doctors who first discovered and reported this medical condition (also refered

to as RMKH, Rokitansky-Mayer syndrome, Rokitansky syndrome).

Rokitansky-Mayer-Küster-Hauser syndrome is a condition that includes

maldevelopment or absence of the vagina, fallopian tubes, cervix, and/or uterus. Some

women have incompletely developed uterine remnants, or horns. The ovaries and

external genitalia are normal, and the chromosome makeup of individuals is 46XX

(normal female).

Other symptoms involved to a varying degree are kidney abnormalities, skeletal

problems and hearing loss. The exact cause of these developmental problems is not yet

known.

Approximately 7% to 8% of the remaining patients with vaginal agenesis have a more

unusual genetic abnormality and have a condition known as androgen insensitivity

syndrome (AIS).

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Genetically, these patients are 46XY, and lack a vagina, cervix, uterus, fallopian tubes,

and ovaries. External genitalia may have a normal female appearance, or may lie

anywhere along the spectrum from male to female. Even fewer patients with vaginal

agenesis have other rarer conditions and more complex genetic and physical

abnormalities.

Regardless of the cause, the concerns surrounding vaginal agenesis are shared by all

who have this condition. The remainder of this article will help address some of these

issues.

Double uterus, complete or incomplete vaginal obstruction, and ipsilateral renal agenesis. A. Complete

vaginal obstruction. B. Incomplete vaginal obstruction. C. Complete vaginal obstruction with a lateral

communicating double uterus. (Rock JA, Jones HW Jr: The double uterus associated with an obstructed

hemivagina and ipsilateral renal agenesis. Am J Obstet Gynecol 138:340, 1980)

A. Isolated congenital cervical atresia with normal vaginal

development. B. Congenital cervical atresia with complete

vaginal agenesis. (From Fujimoto VY, Miller JH, Klein NA et al:

Congenital cervical atresia: Report of seven cases and review of

the literature. Am J Obstet Gynecol 177, 1419, 1977.)

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DIAGNOSIS

The diagnosis of vaginal agenesis or hypoplasia is most commonly made between the

ages of 15 and 18 years. Most young women first present to their physicians with

concerns because they have not started menstruating. Women with RMKH syndrome

have normal functioning ovaries and have usually gone through puberty and have

normal breast and pubic hair by this time.

Women with AIS or other conditions may have additional concerns over sexual

development. In either case, vaginal agenesis is the most common cause for failing to

start menstruating.

Diagnosis of vaginal agenesis is usually made with a physical examination by a

qualified physician. Other tests, including hormonal and genetic tests can help

determine what, if any, the exact cause of this condition is. Since there may be

associated kidney and uterine abnormalities, imaging studies such as an ultrasound,

MRI, or intravenous pyelogram may be useful. Patients are usually referred to a

pediatric and adolescent gynecologist for specialized care.

TREATMENT

After being evaluated by a specialist, questions usually arise about treatment. It is

important to know that a variety of treatment options are available for vaginal agenesis.

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Both non-surgical and surgical techniques are available to reconstruct the vagina. The

non-surgical approach involves expanding and enlarging the tissue already present at

the vaginal entrance by applying pressure (pressure dilation) over an extended period of

time. Plastic surgical techniques involve constructing a new vagina out of tissue from

various donor sites.

Although there are a variety of treatment approaches, the results of pressure dilation

are considered superior to those of surgical construction of a vagina. It is also important

to realize that dilation is also required after most surgical methods of creating a vagina,

and this postoperative dilation can often be more painful. It is therefore generally

recommended that pressure dilation should always be the first intervention tried, with

surgery utilized as a last resort.

1. Pressure Dilation Techniques

As described above, pressure dilation involves expanding and enlarging the tissue

present at the vaginal entrance by applying pressure over a period of time. After

dilation, the vagina naturally develops a mucosal lining very similar to that of a normal

vagina, and has a more natural sensation. Two approaches to this method are

available: the intermittent pressure (Frank Method) and the continuous pressure

(Vecchietti Procedure).

The intermittent pressure method (known as the Frank Method after the doctor who first

advocated it) is performed by the girl herself at home. It involves gentle pressure

application of rod-shaped appliances at the vaginal opening. This is typically done once

or twice per day for 20-30 minutes. The dilators are increased in size as dilation of the

vagina is achieved. The time necessary to complete treatment varies from less than one

month to over a year. A variety of different vaginal dilators are available, and some

refinements of the technique have been made. A specialized stool (Ingram Method) is

available that allows dilation to be carried out while clothed and in a sitting position.

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In the continuous pressure method (Vecchietti Procedure), pressure is applied in the

vaginal area by a dilation “olive”, a plastic bead through which traction sutures or

threads are threaded.

A surgical procedure is necessary to set up this method, since the traction sutures run

through the abdomen and the traction device is placed on the outside of the abdomen.

Following the surgery, the vagina is stretched over the course of 7-10 days.

2. Plastic Surgical Techniques

A variety of surgical methods have been developed for constructing a new vagina. All of

these procedures should be delayed until after puberty so that dilation can be tried first,

and because surgical creation of a vagina in childhood usually has poor results. The

most common surgical technique used is the Abbe-McIndoe Method. In this surgery, a

skin graft is used to create a vagina. The skin is usually taken from the buttocks of the

patient, and is then molded to form the vagina. The main problem of this procedure is

the tendency of the vagina to contract, necessitating the use of dilators after surgery.

Other surgical techniques are available, such as using a length of colon (gut) or

peritoneum (the membrane lining the inside of the abdominal cavity) to create a vagina.

VAGINOPLASTY

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A sagittal section through the pelvis demonstrates congenital absence of the vagina. The site of dissection will be in the areolar tissue between the bladder and rectum. The space is exaggerated here for illustration. The dissection should continue cephalad to 1-2 cm before the level of the peritoneum. Deeper dissection may allow the development of an enterocele.

In this view of the introitus in congenital absence of the vagina, a slight indentation or dimple may exist where the urogenital sinus in the fetus failed to invaginate and fuse with the Mullerian duct to develop a normal vagina.

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A second cause of absence of the vagina is surgical removal for oncologic reasons.

The air-powered dermatome is preferred because of the uniform thickness and size of the graft produced. The setting on the air-powered dermatome can be adjusted to 18/1,000ths or 24/1,000ths of an inch. STSG identifies the split-thickness skin graft.

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Two proposed paddle-shaped full-thickness skin grafts over the inguinal ligaments from the anterior superior iliac spine to the pubic tubercles are depicted. These paddles should be approximately 10 cm in length and 6 cm in width.

Harvesting of the full-thickness skin graft is performed with the standard scalpel after marking the shape of the paddle in ink on the skin.

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The full-thickness skin grafts (FTSG) are placed in a graft pan and moistened with saline. The same is true for the split-thickness skin graft (STSG) if that is preferred to the full-thickness skin graft.

After harvesting the full-thickness skin graft from the inguinal ligaments, if closure of the wound is on tension, the new SureClosure skin-stretching system can be utilized as shown. The stabilization needles are inserted in the subcutaneous space parallel to the line of the incision. Two, 4, or 6 of these can be utilized as necessary. Usually, no more than 2 are required. The skin-stretching device is anchored into

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the needle, and the ratchet on the skin-stretching device is activated until the skin is stretched to close the incision. Presently, a subcuticular suture of 3-0 delayed synthetic absorbable suture is utilized to close the wound.

A block of ordinary foam rubber, such as that used in the upholstery industry, is sterilized by gas autoclaving. The shape of the proposed vaginal form is outlined with brilliant green solution. The split-thickness skin graft is removed from the adhesive tape of the dermatome and placed in a sterile pan filled with saline solution. Ordinary contraceptive condoms that have been sterilized by gas autoclaving are used. Generally, two condoms are used to ensure against leakage of fluid into the foam rubber form.

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The foam rubber block is held in one hand and, with curved Mayo scissors, is shaped into the desired vaginal form.

The condom is slipped over the foam rubber vaginal form. The condom can be removed partially or completely if the size of the vaginal form needs to be adjusted in diameter or length. When the size of the vaginal form is satisfactory, the end of the condom is tied with a 2-0 synthetic monofilament permanent suture.

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The vaginal form is laid on the epidermal side of the split-thickness skin graft. The split-thickness skin graft is folded over the vaginal form and sutured along its seam with interrupted 4-0 synthetic absorbable suture. Excess graft is trimmed away.

The full-thickness, paddle-shaped skin grafts are placed over a foam rubber condom, and the margins are sutured together with synthetic absorbable suture.

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The dimple in the introital area is identified. The labia are retracted with Allis clamps, and a transverse incision is made in the epithelium.

Blunt dissection with the fingers opens the space between the bladder and rectum. Once the correct plane is reached, i.e., below the pubovesical cervical fascia under the bladder and superior to the perirectal fascia over the rectum, gentle blunt dissection is all that is needed to create an adequate cavity.

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A sagittal section shows the dissection carried approximately 2 cm from the peritoneum. This will reduce the incidence of enterocele in these patients.

Meticulous hemostasis should be maintained throughout the cavity.

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The skin-covered form is inserted into the cavity.

A sagittal view of the pelvis shows the skin-covered form inserted into the new vaginal canal.

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To hold the form in place for 12 days, the labia are sutured in the midline with interrupted 0 nylon sutures without tension.

After the foam rubber vaginal form has been removed on the 12th postoperative day, the new vagina

should be thoroughly inspected and irrigated with normal saline solution. A permanent vaginal form made

of soft rubber or silicone should be fitted and inserted. Care should be taken that the new vaginal form

does not protrude beyond the introitus. Protrusion will erode the introital area and cause pain,

discouraging the patient from continuing to use the form.

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GENDER, SEXUAL IDENTITY AND FUNCTIONING, AND MOTHERHOOD

These concerns are common and natural when confronted with the diagnosis of vaginal

agenesis. Gender and sexual identity issues may be even more troubling for patients

with AIS or other more complicated genetic and physical conditions. These concerns

are best addressed with the guidance of a qualified physician and professional

counselor. The important thing to remember is that no one can tell that a patient has

vaginal agenesis. And, following treatment, with dilators or surgery, no one will be able

to tell that the patient has had a reconstructive procedure.

Patients also wonder about sexual functioning and sexual pleasure. Much of the sexual

pleasure comes from stimulation of the clitoris, and not from the vagina. Therefore, after

creation of a vagina, and the decision to become sexually active, a patient will have

normal sexual sensations and enjoyable sexual relations.

Motherhood is also a very important concern. The question of “Will I be able to have

children?” depends on the individual patient. In women with RMKH syndrome that were

born with a normal sized uterus, patients may become pregnant and deliver a baby. In

those women that were born without a uterus or if the uterus is small, patients may not

be able to carry a pregnancy. However, since these patients have normal functioning

ovaries, other options are available. In vitro fertilization can allow an egg to be fertilized

by a partner’s sperm, and then the pregnancy carried by a surrogate mother. Adoption

is another choice for some couples that are not able to have their own children. It is

important to realize, however, that not being able to have your own children does not

mean that you cannot become a mother.

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