Reconstruction of Congenital Defects of the Vagina

Abstract

Congenital absence of the vagina is a relatively rare condition most commonly associated with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Historically, several reconstructive techniques have been described to provide for functional vaginal reconstruction on these patients, both operative and nonoperative. Although there are many advantages and disadvantages of the various procedures, one experience with the use of split thickness skin grafts to reconstruct the vagina has produced acceptable functional results with limited donor site morbidity. Careful planning and timing of this form of reconstruction can produce predictable results in patients who are nearing sexual maturity.

Complete absence of the vagina is an uncommon congenital condition. Yet, the most common congenital deformity of the female pelvis is absence of the vagina. The condition most frequently associated with agenesis of the upper two-thirds of the vagina is müllerian aplasia also known as Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome.^1,2,3,4 The reported frequency ranges from 1 in 4,000 to 1 in 80,000 live births. These women have a normal female genotype (46XX), phenotype, and most commonly normal ovarian function with either an absent or rudimentary uterus (2–7% of individuals). MRKH syndrome may be an isolated gynecologic deformity (type I), but may also be associated with anomalies of the urinary tract and skeletal system (rib, vertebral), and to a lesser extent auditory and cardiac anomalies (type II). Urinary tract abnormalities may be found in up to 25–50% of affected individuals, and include dilatations, duplications, agenesis, and renal ectopy. Hence, preoperative assessment of the urinary tract system by either an intravenous pyelogram or abdominal and pelvic ultrasound is essential in these patients.

Commonly, primary amenorrhea is the presenting symptom encountered by the clinician, usually between the ages of 14–16 years. Secondary sexual characteristics are normal as these patients typically have normal ovarian function (Fig. 1). Simple physical examination will reveal the absence of a vagina with normal appearing external genitalia, suggesting the presence of this syndrome. Once the final diagnosis of MRKH syndrome is made (physical exam, abdominal/pelvic ultrasound, and karyotyping), discussion with the patient and her family is performed to appropriately plan the reconstructive process. Along these lines, the management of vaginal agenesis in MRKH syndrome constitutes a considerable challenge as its outcomes affect both the psychosocial and physical health of the patient.

Figure 1.

Typical appearance of a patient with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Note the normal appearance of the labia majora and minor with absence of a vaginal canal.

EMBRYOLOGY

The female reproductive organs are composed of the ovaries, uterus, and fallopian tubes. The internal genitalia develop from the paired müllerian ducts (paramesonephric ducts), which first emerge around 5 weeks postfertilization. These ducts are invaginations of the dorsal coelomic epithelium that develop lateral to the primitive renal system, the wolffian ducts (mesonephric ducts). Cranially, the paramesonephric ducts remain unfused and open into the peritoneal cavity; ultimately they become the fallopian tubes. Caudally, the ducts fuse and subsequently canalize, forming a single cavity that becomes the uterus. The caudal tip of the fused paramesonephric ducts projects into the urogenital sinus. Bilateral outgrowths of the urogenital sinus and vaginal plate grow and extend upward separating the uterus from the urogenital sinus. This movement and subsequent canalization of these tissues results in the formation of the vagina. It is hypothesized that a developmental arrest at this juncture results in the deformities associated with MRKH syndrome. The hymen is the result of vaginal epithelial proliferation at the point of contact between the vagina and the urogenital sinus.⁵

SURGICAL TECHNIQUE FOR THE RECONSTRUCTION OF VAGINAL AGENESIS

General Principles

Probably the most important aspect with regard to reconstruction of these patients is the timing of the initial procedure. Ideally, the patient should be nearing the age for sexual intercourse, highly motivated, and emotionally mature. Patients who are relatively immature despite their chronological age are at great risk for poorer outcomes, which can lead to unfavorable healing. Failure of complete, unimpeded healing can result in the need for multiple revisionary procedures, the consequences of which may result in various degrees of sexual dysfunction. Thus, preoperative patient assessment and careful psychological preparation before any surgical intervention is crucial. Failure to do so will most probably influence the success of any surgical or nonsurgical intervention.

Functional vaginal reconstruction must accomplish several essential requirements: (a) adequate length of the vaginal wall, (b) sufficient transverse dimensions of the introitus and vaginal pouch, (c) healing without cicatricial contracture, and (d) the vaginal lining should be durable enough to withstand the forces associated with intercourse. All of these elements are essential to allow for adequate and pain-free intercourse.

Maintaining fertility is rare due to the mal-developed uterus that is usually associated with these conditions. However, in the rare cases in which the uterus and cervix are functional, they should be maintained during the reconstructive procedure to allow the potential for future childbearing. The ability to bear children remains possible in most cases due to the recent advances with in-vitro fertilization. Regardless of the presence or absence of a functional uterus, one must remember that the majority of these patients have functioning ovaries, which would therefore allow for such in-vitro procedures through the use of a female surrogate. Regardless of the technique chosen, the surgeon must be experienced with the procedure and with its complications because the first attempt at reconstruction is the one with the highest likelihood of success.

Reconstructive Techniques

The description of techniques to correct vaginal agenesis dates back to the mid 1800s.⁶ The fact that there are many operations for the correction of vaginal agenesis suggests that no one procedure is capable of fully replicating a natural vagina. There remains no consensus in the literature regarding the best option, thus we will attempt to describe the more commonly performed procedures, with an emphasis on our favored technique.

The least invasive procedures are based on the gradual dilatation of the rudimentary vaginal pouch. First pioneered by Frank⁷ and later modified by Ingram,⁸ the graduated introduction of serial dilators over a period of months allows for some degree of lengthening to the vaginal canal. It has been suggested that this nonsurgical method should be the first-line method for treatment in cases in which the existing vaginal canal is more than 3 cm deep.⁹ An alternative technique combining gradual dilatation and surgery was originally described by Veccehietti,¹⁰ and later modified to be performed laparoscopically.¹¹ The latter two techniques have mainly gained popularity in Europe. The major drawback to all the above-mentioned techniques is inadequate patient compliance. To achieve suitable vaginal depth, the dilatation must be performed several times a day over a period of months to a year. Thus, high failure rates are noted, particularly in the younger patients. Our experience with the use of gradual dilatation has been universally disappointing.

The most commonly performed operation, and our preferred approach, is the Abbe-McIndoe procedure.⁹ This procedure involves the use of a split thickness skin graft (STSG) secured over a vaginal mold, which is then placed into a surgically dissected space between the rectum and bladder (recto-vesicular space) to create a neo-vagina (Fig. 2). This technique was pioneered by Abbe in 1898, and later popularized by McIndoe.¹² The main advantages of this technique are the relative technical ease of the operation, and the minimal donor site morbidity. This is especially true when the skin graft is harvested from the suprapubic region (Fig. 3). This preferred donor site has many inherent advantages. The STSG can be easily harvested as a single sheet graft in the supine position, produces acceptable cosmetic results, and allows for preparation of the graft while the perineal pocket is being created. It is critically important to harvest the STSG as a single sheet, thus allowing for a single suture line to be sewn around the stent, which reduces the potential for cicatricial contracture of the reconstruction (Fig. 4). The main drawbacks of this procedure are (a) the need for prolonged use of vaginal stent for up to 6 months postoperatively or until regular sexual activity commences, (b) the postoperative immobilization to improve skin graft take, and (c) lack of lubrication when the neo-vagina is constructed with skin. This technique is thus suited for the more mature and motivated patient. The most common complication of this procedure is partial graft take, which is often treated conservatively. Complete graft failure may be related to premature graft motion (lessened by strict bed immobilization for a week) or collections of blood or fluid beneath the skin graft. Reported reoperation rates range from 1 to 65%,^13,14 though in our experience the rates are less than 10% percent. On rare occasions, the surrounding structures may be inadvertently penetrated possibly resulting in fistula formation. Preoperative bowel prep is typically performed to lessen the consequence of inadvertent enterotomy.

Figure 2.

McIndoe procedures. (A) A pocket is created between the bladder anteriorly and the rectum posteriorly. (B) A split thickness skin graft is then sutured around a vaginal mold and then (C) secured in position using permanent sutures.

Figure 3.

The preferred donor site in the suprapubic region is easily accessible and can be used even in patients with previous surgical scars (A). Tumescent fluid is injected subcutaneously prior to graft harvest to ensure that a single sheet graft is obtained (B,C).

Figure 4.

(A) The stent is left in place for one week at which time the stent is removed under general anesthesia and the split thickness skin graft inspected. (B) Postoperative stenting of the reconstruction is necessary for long-term graft take and the avoidance of contracture.

Williams popularized vulvovaginoplasty in 1964,¹⁵ as a simple less-invasive means of recreating a vagina, by avoiding the dissection in the rectovaginal space. The original Williams procedure, which involved a horseshoe incision along the border of the labia majora and minora, has fallen out of favor due to an unnatural angle to the neo-vagina and risk of urine collection behind the resultant high perineum. Recent modifications of the Williams procedure claim to have addressed these problems, resulting in a quick, safe, technically less-demanding surgery, resulting in a satisfactory neo-vagina in a correct axis.¹⁶

Several local fasciocutaneous flaps have been described for creating a neo-vagina. These flaps are mainly based on the external and internal pudendal vessels. Probably the most common of these is the neurovascular pudendal thigh flap, also known as the “Singapore” flap.¹⁷ Supplied by the external pudendal artery system, this pedicled axial flap is a reliable, simple, and reproducible flap that has an easily hidden donor site scar. The main drawbacks of this technique are that these flaps are raised from a hair-bearing area and the fact that two flaps are necessary for total vaginal reconstruction. Recently, a pedicled unilateral external pudendal perforator flap was described for reconstruction of the congenital vagina agenesis.¹⁸ This flap offers some refinements to the original “Singapore” flap, which basically are the use of a unilateral flap, over the less hair-bearing skin region of the medial thigh.

An altogether different approach to vaginoplasty is the use of pelvic peritoneum from the pouch of Douglas. First described by Rothman¹⁹ and later modified by Tamaya and Imai²⁰ this technique entails the use of a pelvic peritoneal flap accessed through a laparotomy incision to recreate a neo-vagina lined with peritoneum. In a more recent modification, the neo-vagina is recreated using pelvic peritoneum in a one-stage transvestibular procedure with laparoscopic or laparotomy assistance.²¹ The use of pelvic peritoneum has potential advantages: little risk of flap necrosis or graft failure, no hair growth or odor as might occur with skin grafts, and minimal associated subcutaneous tissue that could lead to vaginal vault narrowing in case of weight gain—a potential problem with fasciocutaneous flaps.²¹

Colonic interposition vaginoplasty is a well-described technique more commonly utilized in the pediatric population or in cases of gender reassignment surgery.^22,23,24 The proposed advantages these techniques have to offer are adequate vaginal length and width maintained over time without the need for stenting or dilatation, very low risk of vaginal stricture, spontaneous mucus production facilitating sexual intercourse, and texture and appearance similar to that of the natural vagina. The disadvantages of this approach are significant. These include the need for major abdominal surgery with possible complications relating to the donor site (bowel obstruction, adhesions, strictures, fistula formation, and donor site scaring), mucosal prolapse, dyspareunia, and introital stenosis.⁵ The aforementioned advantage of neovaginal lubrication in our experience is often a troubling issue. Although native lubrication is useful during intercourse, the downside of persistent vaginal drainage is cumbersome for many patients and often leads to significant daily hygiene issues.