Pediatric urologists are frequently called upon to perform vaginoplasty for congenital or acquired female genitourinary anomalies at any age. There is a wide spectrum of disorders that might potentially be treated with a vaginoplasty.
Congenital female anomalies can be classified by which perineal structures are affected with the vagina. They include:
Isolated vaginal anomalies (figure 1) can exist with or without affecting the uterus and include partial or complete vaginal duplication, agenesis, or obstruction. A relatively common example associated with unilateral renal agenesis that presents with primary amenorrhea due to complete vaginal and uterine agenesis is the Mayer-Rokitansky-Küster-Hauser Syndrome.
Figure 1. Isolated vaginal anomalies, including agenesis, can exist with or without affecting the uterus. The external genitalia and anorectum are normal. (Photo courtesy of Linda A. Baker, MD)
Urogenital sinus anomalies (figure 2) can exist with or without external genital ambiguity in infancy and consist of internal fusion of the vagina and urethra. Urogenital sinus is most commonly due to congenital adrenal hyperplasia. The vagina and urethra drain onto the perineum via one shared orifice and the anorectum is normal.
Cloacal anomalies (figure 3) are the most severe neonatal genitourinary fusion anomalies wherein there is internal fusion of the vagina, urethra, and anorectum. The vagina, urethra, and anorectum drain onto the perineum via one shared orifice.
Acquired female genitourinary anomalies are secondary to other treatments. Examples include:
The commonality shared by all these conditions is the need for some form of vaginal reconstruction surgery. This article discusses current surgical options, with a focus on autologous buccal mucosa vaginoplasty.
Given there are many anatomic variants in need of vaginoplasty, many vaginoplasty techniques have been described. In order to guide technique choice, several preoperative surgical questions should be answered:
Is the vaginoplasty a primary procedure (no prior vaginal or perineal operation) or a secondary procedure (vaginal or perineal reoperation) fraught with extensive scarring?
Is the quantity and quality of native vaginal tissue available for use in the vaginoplasty sufficient to correct the anomaly?
Where is the healthy native vaginal tissue located (if any is present)?
Figure 2. Urogenital sinus anomalies can exist with or without external genital ambiguity and consist of internal fusion of the vagina and urethra. Here is a case of congenital adrenal hyperplasia demonstrating ambiguous external genitalia (clitoromegaly), a urogenital sinus, and normal anorectum. (Photo courtesy of Linda A. Baker, MD)
If the available native vaginal tissue is totally sufficient for vaginal reconstruction and the vaginal introitus is stenotic, then perhaps a simple pulldown procedure with scar excision could be performed. However, if there is some usable native tissue available but the amount is insufficient for reconstruction, a composite vaginoplasty must be performed. A composite vaginoplasty would consist of native vaginal tissue augmented by neovaginal tissue taken from a donor site.
Lastly, if there is a complete absence of the vagina, a total neovagina must be constructed from donor tissue. If neovaginal tissue is needed, then a decision for the surgeon is the choice of the best donor tissue source.
In situations where there is total absence of the vagina (figure 1), a variety of techniques have been described for the creation of a total neovagina, all with inherent advantages and disadvantages. The following is a short, selected list provided for illustrative purposes. For a more exhaustive review, see Human Reproduction Update (2014; 20:775-801) and others.
Figure 3. Cloacal anomalies, the most severe neonatal genitourinary fusion anomalies, are marked by internal fusion of the vagina, urethra, and anorectum. This infant female has only one perineal orifice, the cloaca. (Photo courtesy of Linda A. Baker, MD)
The Frank method. A nonoperative procedure first described in 1938 (Am J Obstet Gynecol 1938; 35:1053–5), the patient utilizes increasing sized sequential dilators to intermittently manually apply pressure on the perineal vaginal dimple where the vagina should have formed. Although clearly the least invasive and most cost-effective procedure, it requires substantial patient motivation, patient effort, and a significant time investment. Unfortunately, younger patients often show limited success, especially in cases with only a minimal skin dimple with which to start.
The Vecchietti procedure or traction vaginoplasty. Originally described in 1965 (Attual Ostet Ginecol 1965; 11:131–47) and later improved with a minimally invasive laparoscopic approach, the Vecchietti procedure establishes traction on a perineally positioned bead with sutures passing through the prevesical space to the traction tension device on the ventral abdominal wall. Continuous upward pressure is applied by the traction bead to the vaginal agenesis dimple, which in turn stretches, dissects, and elongates a mucosal cavity. There are some disadvantages, including pain with continued tightening of the traction device, potential abdominal or pelvic complications with traction thread placement, and discomfort and inconvenience of wearing an indwelling vaginal form at night for months after the vaginoplasty to prevent vaginal contraction and stenosis.
Intestinal vaginoplasty. A short segment of bowel (sigmoid colon, ileum, or jejunum) is harvested and attached as a vaginal opening on the perineum. Advantages of the procedure include natural lubrication through bowel mucus production and utility even if the patient has had previous perineal surgery. Potential drawbacks of the procedure include the inherent risks of an abdominal surgery and intestinal anastomosis, visible abdominal scarring (which can be minimized if performed laparoscopically), bowel vaginoplasty loss, stenosis or prolapse, and excessive discharge of odorous bowel mucus requiring daily pad usage.
Myocutaneous flap vaginoplasty. Bilateral flaps are created on the vascular pedicle with the underlying muscle (ie, gracilis) and used for reconstruction. If the blood supply to the flap remains strong, flap loss or contracture are minimized and vaginal dilation may not be required. Disadvantages include high rates of contracture and flap loss, potential for hair growth in the vagina, and significant disfiguring scars produced at the donor site.
Figure 4. Postoperative vaginal stenosis is an acquired female genitourinary anomaly. This teenage female with congenital adrenal hyperplasia had genital surgery as an infant and now has vaginal stenosis. (Photo courtesy of Linda A. Baker, MD)
The McIndoe procedure. Autologous split-thickness skin grafts are harvested from the buttocks or thigh area (Br J Plast Surg 1950; 2:254-67) and immobilized with a vaginal mold to help prevent stenosis or graft loss. This procedure avoids the complications of abdominal surgery, the grafted tissue typically takes well, and the occurrence of vaginal prolapse is minimal. Unfortunately, the resulting scars at the donor site are significant and often disfiguring, there is still a lack of lubrication as the grafted skin is keratinized, and there is the potential for hair growth in the vagina.
Alternative graft materials are needed, wherever possible, to minimize previously described adverse complications and expand therapeutic options. In addition, from the patient’s view with older vaginoplasty techniques, cosmesis is often poor and function is often suboptimal, owing to the lack of lubrication and dyspareunia from vaginal stenosis. This places additional social and psychological burdens on patients who are already concerned with their sexuality, genital appearance, and fertility.
In my experience, buccal mucosa is an excellent graft alternative affording a host of advantages. The tissue is thick and, with its underlying layer of dense elastic fibers, provides a high degree of strength with distensibility. Buccal mucosa is a non-keratinized, non-hair-bearing secretory epithelium, providing endogenous lubrication without the unpleasant and excessive bowel mucus and is an excellent color and texture match to the native vaginal epithelium. The graft harvest site heals rapidly, with no visible scarring. As the donor site is well hidden inside the patient’s mouth, its presence is likely only evident if the patient decides to share that fact.
Briefly, a 12- to 24-cm2 section of full-thickness mucosa can be harvested from the inner cheek-bilaterally, if needed-along the mandible, from the angle of the jaw toward the lip. The orifice to Stensen’s duct should be unharmed. The autologous buccal mucosa is then manually fenestrated to enable neovascularization and surface area expansion if needed and also to prevent hematoma or seroma accumulation beneath the graft.
After incisions for a neovaginal vault or vaginal stenosis repair are made, the graft tissue is then inserted and anastomosed to the native vaginal tissue if present. A vaginal mold is placed and immobilized by suturing the labia minora across it.
Inpatient care requires pain management, strict bed rest for 7 days with log rolling, minimal hip flexion, and an indwelling Foley catheter. Beginning in the recovery room, the patient must do hourly mouth exercises consisting of maximally opening their jaws then stretching their cheeks bilaterally with fingers and/or tongue to prevent oral contracture. The exercises should be continued throughout the entire hospital stay. At day 7, the urethral catheter and vaginal mold are removed at the bedside and the first dilation is performed.
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At home, vaginal discharge should be expected for up to 7 days. Dilation exercises should continue, starting with the largest tolerated Syracuse vaginal dilator and advancing sequentially through larger sizes. Sexual intercourse should not be attempted for at least 4-6 weeks, depending on healing times.
Since beginning in 2004, I have had excellent results with both composite and total vaginoplasties in over 65 primary and secondary surgeries (J Pediatr Urol 2006; 2:486-8; Pediatr Surg Int 2014; 30:533-5; Obstet Gynecol 2014; 123:947-50), with limited complications. Of the follow-up surveillance biopsies performed, no evidence of dysplasia or malignant degeneration has been detected (J Urol 2011; 185[suppl]:e103, abs. 257). Interestingly, in all biopsy cases the pathologist’s report has been unable to differentiate the tissue sample as being buccal mucosa rather than vaginal mucosa.
There is clearly a need for improved vaginoplasty methods. When native vaginal tissue is in limited quantity, vaginoplasty is more complex with increased risks. I have found autologous buccal mucosa vaginoplasty gives excellent early results when used in both simple and complex repairs.
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