Vaginal atresia is a condition that occurs when the urogenital sinus does not give rise to the lower third of the vagina. There are various causes for absence of the vagina, including complete Mullerian agenesis and androgen insensitivity syndrome, both of which are characterized by absence of the female reproductive structures.
These are not usually classified with vaginal atresia, however, as the Mullerian structures are typically normal in the latter condition. The uterus, cervix, ovaries and tubes are all present as well as the upper two-thirds of the vagina.
The treatment of vaginal atresia is therefore directed at creating or restoring the functionality of the lower third of the vagina. Various grades are seen, including imperforate hymen, transverse vaginal septum, and vaginal atresia. Trauma, inflammation, or tumors of the vagina may result in acquired vaginal atresia.
There is no single approach which is universally successful in treating all grades of this condition.
Dilation procedures are recommended as first-line treatment for lower grades of vaginal agenesis rather than atresia per se. These are based upon tissue expansion, if the vagina is present beyond a dimple, but are not suitable in the presence of skin scarring as may follow previous surgery. They are mentioned here as intermittent self-dilation is also necessary following most surgical corrections to keep the vagina patent.
Also known as Frank’s procedure, this employs a graduated series of vaginal dilators which are inserted into the vaginal dimple using pressure, to expand the potential space between the rectum and bladder. It is performed by the patient and requires no anesthesia or surgery. It may take a few months, and compliance is essential. Psychological support should be available. The overall success rate is 80%. When this fails, surgery may be indicated.
Vecchietti procedure employs continuous abdominal traction on a vaginal acrylic olive to create a passage lined by native vaginal epithelium within 7-10 days. The only risk is of pain and potentially of vault prolapse in the long term.
When the atresia is due to urogenital anomaly, resulting in imperforate hymen, transverse vaginal septum, or complete atresia of the lower vagina, this is usually corrected by surgery, using the perineal approach. The vaginal pull-through method is used. Failure of this technique is followed by the adoption of vaginoplasty techniques.
In type 1 atresia the presence of a hematocolpos makes surgery easier and provides more vaginal mucosa for the pull-through. A needle is passed into the mass under ultrasound guidance if required, and blood is drawn to confirm the location. The closed space is now expanded with forceps, the blood is drained, and the excessive fibrous tissue trimmed away. The proximal vaginal mucosal edge is now brought down and attached to the hymeneal ring near the introitus. Molds are used to keep the vagina patent until it is epithelialized over the whole extent. Until regular sexual intercourse begins, molds may be repeatedly used at intervals. Digital dilatation must be supplemented if the neovagina is too narrow.
Type 2 atresia is accompanied by other serious anomalies of the reproductive tract. The chances of successful pregnancy are small even with successful correction, which requires major and complex surgery. The percentage of patients who eventually undergo hysterectomy is very high. For this reason vaginoplasty and cervicoplasty, with reconnection to the introital region, is considered only if the uterus is normal.
These procedures employ a variety of techniques and tissues to create a functional and cosmetically acceptable vagina. They may be based upon split-thickness skin grafts, full-thickness skin grafts, amniotic membrane, bowel grafts, or peritoneum. Each has its advantages and disadvantages. Many of them have now been replaced by laparoscopic procedures which make them less invasive, with shorter recovery times and avoids large abdominal incisions, potentially averting many operative complications. It also helps to prevent adhesions and rectal injury. Robotic surgery is now becoming increasingly popular.
Vaginal pull through procedure is used to correct an imperforate hymen or transverse vaginal septum. It involves cutting through the obstructing fibrous material, until the normal vagina is reached. At this point the collected blood is drained. The normal mucosa is then pulled through and attached at the hymeneal ring just above the introitus, and kept patent until re-epithelialization occurs.
William’s vaginoplasty in which the labia majora are fused to form a short neovagina. It was the first technique to be widely used but fell out of favor because of the very short vagina that resulted. Various modifications have therefore been made such as the Creatsas modification using perineal as well as vulval tissue, which yields better results with the length of the neovagina being up to 12 cm, and has been associated with sexual satisfaction in almost 95% of the patients.
The McIndoe-Reed procedure consists of expanding the rectovesical space and lining it with a split-skin thickness graft taken usually from the buttocks, and stretched over a mold, to create an artificial vagina. The presence of scars and possible occurrence of vaginal dryness and strictures are possible complications. It has a success rate of above 90%.
Amnion-lined neovagina using the same basic technique has now fallen out of use due to practical difficulties such as the possibility of transmission of various infections including HIV, as well as obtaining and storing appropriate tissue
The Davydov procedure uses the same space lined by peritoneum, but is associated with the risk of peritonitis, and sometimes of dryness and pain, as well as the potential for prolapse.
Intestinal vaginoplasty consists of using a pedicled loop of colon or ileum of which the distal end is sutured to the perineum. This requires a combined abdomino-perineal approach. The new vagina is well-lubricated and capacious, but the surgical procedure carries significant risks of peritonitis. The mucous discharge may be annoyingly excessive, leading to the need for continuous wear of a tampon or sanitary pad. Follow-up is required for both skin grafts and intestinal grafts to rule out the occurrence of carcinomas in the long term. It has an 80% success rate but is usually indicated only in the presence of severe perineal scarring.
In general, surgical correction is undertaken only when the patient is old enough to comply with post-surgical care, but may be performed earlier if indicated by a large or painful hematocolpos causing vaginal outflow obstruction, abdominal or pelvic pain or factors indicating the risk of endometriosis.
The goals of surgical intervention are to provide relief from pain, ensure normal sexual intercourse and to preserve fertility.
The choice of surgery depends upon the grade and type of atresia, the associated anomalies, the skill of the surgical team, the history of previous surgical attempts, the presence of scarring in the genital or abdominal area, and appropriate post-surgical and psychological care.