Vaginal Atresia Treatment & Management

Treatment options for vaginal agenesis include creation of a neovagina. Nonsurgical creation using Frank dilators is the best first-line approach. Patients use the dilators, increasing in length and diameter, with spandex underwear as they sit on a stationary racing bicycle seat for 30-120 min/day. This process uses pressure to create the vagina. Care must be taken to avoid dilating the urethra.

If this treatment option fails, surgical intervention should be considered.

In the patient with functional ovarian tissue but an absent uterus and vagina, reconstruction of the genital tract is not medically urgent. In the absence of ambiguous genitalia, gender assignment is not an issue, and involving the patient in the decisions regarding future surgery is prudent.

Girls who are offered the McIndoe operation require a certain level of psychological and sexual maturity to be motivated and compliant with the dilation (mold) regimens necessary for a successful outcome. Surgical intervention is usually delayed until the late teens so the patient is mature enough to comply with postoperative care. Surgical or medical intervention must be started sooner than this if a patient presents with vaginal outflow obstruction, abdominal or pelvic pain, or a risk for secondary endometriosis.

The primary goals of surgical intervention in patients with vaginal atresia are to relieve obstruction and pain, to restore a normal sex life, and to preserve the patient's reproductive potential. The timing of surgery depends on the patient's anatomic configuration and on the presence or absence of functional endometrial tissue.

The decision to perform surgery to correct vaginal atresia must be made in the context of the patient's overall condition. If a patient has a lethal or complex congenital anomaly that might complicate anesthesia or surgical management, reconstruction of the uterovaginal outflow tract should be carefully considered. Alternative decompression or resection techniques may be preferable.

Patients with clinically significant neurodevelopmental delay should be reevaluated for anatomic reconstruction within the context of future sexual maturation. A complex perineal reconstruction that does not improve a patient's daily function and that requires the use of postoperative dilators could be imprudent because of physical and psychological trauma.

The complexities of the regulation of developmental processes are now becoming sufficiently clear to allow speculation regarding future intervention with specific modulators (eg, clinical evaluation of the effects of intervention by using müllerian-inhibiting substance [MIS]).

An alternative strategy may rely on advances in surgical techniques and on the application of homografts derived from advances in tissue engineering, which allow for reconstruction by using site-specific tissues. ^[23]

The role of medical management of vaginal atresia is limited. In patients with functioning endometrium and an obstructed outflow tract, temporary hormonal manipulation of the menstrual cycle may be necessary until a patent genital tract can be created.

Most patients with functional endometrial tissue do not have a fully functioning uterus. In patients who have a patent cervix an absent vagina (very rare), creation of a neovagina will allow for outflow of the menstrual blood. On the other hand, the creation of a cervix in patients without a patent cervix is associated with a very high rate of infection and mortality. Therefore, most patients with some functioning endometrium in the müllerian bulb need to undergo bulb resection.

In 1938, Frank described a nonsurgical technique developed especially for patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. ^[24] In this clinical situation, absence of the uterus and proximal vaginal tract obviates complex reconstructive operations. The goal is to create a blind-ending vaginal pouch.

The technique involves forceful dilation of a shallow rudimentary vaginal pit with the sequential application of progressively wider and longer dilators. In 1980, Hendren and Donahoe reported that seven of eight patients treated with this technique achieved a pouch within 2-4 months. ^[25] In another study, vaginal dilation was attempted in 37 of 51 patients with vaginal agenesis, with a 92% success rate. ^[26]

Success depends on the presence of at least a vaginal dimple and requires a highly motivated patient who, wishing to avoid extensive surgical procedures, is willing to continue long-term dilation. Problems of stenosis, dyspareunia (20%), and decreased vaginal lubrication have made this option unattractive for many patients.

As for other congenital anomalies, the multiple reconstructive options available for vaginal reconstruction indicate that no single technique has gained uniform approval. ^{[27, 28, 29]} Pediatric surgeons, gynecologists, and plastic surgeons have traditionally contributed to the literature on the subject, and specialists in each field continue to be involved in the treatment of patients with vaginal atresia. A multidisciplinary approach is recommended to best meet the challenge of providing good cosmetic results with optimal functional and reproductive outcomes.

Choice of surgical approach

The various surgical approaches can be classified according to the tissues used to create a neovagina. Reconstruction involves the use of either extra-abdominal tissues or tissues from the abdominal cavity.

Reconstruction by using extra-abdominal tissues

Split-thickness skin grafts are the basis for many reconstructive procedures, such as the Abbe-McIndoe operation. ^[30] With this procedure, a split-thickness skin graft is taken from the buttock and used to create the neovagina. Afterward, a mold is inserted to shape the vagina. Advantages are related to the low morbidity and mortality of this type of reconstruction. Disadvantages include the tendency to develop vaginal stenosis, the lack of natural lubrication, the possible development of fistulas, and the need for continuous mold placement if the vagina is not used.

Musculocutaneous flaps provide a reliable means of creating a neovagina. ^[31] Although they provide a sensate surface, they are bulky. The rectus and gracilis muscles are the primary muscle groups used. As with fasciocutaneous and subcutaneous pedicled flaps, operations involving these flaps tend to cause scarring and use hair-bearing skin. ^[32]

Bilateral flaps have been used to create a neovagina for vaginal atresia, but they are primarily reserved for reconstruction after ablative oncologic surgery. A unilateral flap is often appropriate for reconstruction after repair of rectoneovaginal fistulas or urethral defects.

In vulvovaginoplasty, tissue expanders are used to increase the available skin from the labia. This tissue is used to create a posteriorly directed pouch. Functional assessment reportedly has yielded unsatisfactory results. Several groups report the use of amnion as a homograft, without evident rejection. This technique maintains a vaginal space for future dissection, but it has not gained wide acceptance.

Successful use of laparoscopic assisted biomaterial grafts for reconstruction of congenital atresia of the vagina and cervix has been reported. ^{[33, 34]}  

Reconstruction by using tissues of abdominal cavity

Intestinal segments, typically derived from the sigmoid colon, ^{[35, 36]} may provide advantages over the skin grafts used in the Abbe-McIndoe operation, in that the former do not routinely require postoperative dilation. Sections of the ileum, cecum, and rectosigmoid colon have also been used.

The advantages are principally related to the distensibility and self-lubricating nature of the conduit and the reduced natural contraction resulting in neovaginal stenosis, which allow the patient to avoid the discomfort of long-term use of dilators. Disadvantages are related to the potential complications encountered in laparotomy and bowel resection and to the continuous production of mucous secretions that may require the use of an absorptive pad or tampon.

The peritoneum has been used to maintain a cavity until the surfaces become epithelialized.

The laparoscopic Vecchietti procedure has gained a degree of acceptance in the management of vaginal atresia. ^[37] It aims to create a neovagina by invagination by using an acrylic olive that is placed against the vaginal dimple. The olive is attached to a traction device mounted on the abdomen with laparoscopically placed subperitoneal sutures. Then, traction is applied to the olive to produce 1-1.5 cm of invagination per day, creating a neovagina in approximately 7-9 days. After the neovagina is created, active dilation is required until regular sexual activity is started.

One advantage of this technique over the Frank procedure is that uninterrupted traction is applied. In addition, prolonged hospitalization is unnecessary because the traction can be completed on an outpatient basis. However, this is a relatively new technique, and the long-term results have yet to be reported.

Another laparoscopic approach is an adaptation of the Davydov procedure. This technique is characterized by a three-stage operation that includes dissection of the rectovesical space, abdominal mobilization of the peritoneum to create the vaginal fornices, and attachment of the peritoneum to the introitus. With use of the laparoscopic approach, the abdominal end of the neovagina is closed with a purse-string suture.

This laparoscopically assisted operation lowers the rate of intraoperative complications, shortened operating times and hospital stays, and minimizes external scars. After surgery, sexual function (composite score for desire, arousal, lubrication, orgasm, satisfaction, pain) approaches that of matched controls without gynecologic disorders.

Robotic-assisted repair of vaginal atresia has been described. ^{[38, 39]}

At present, no consensus has been reached regarding the ideal method for creating a functional vagina. At present, the most common operation is McIndoe vaginoplasty. Relatively noninvasive laparoscopic surgery seems promising. ^[40] However, additional data about results and long-term complications are required for it to gain general acceptance.

Procedural details

Psychological preparation of the patient is paramount. A well-established patient-physician relationship must be established.

Standard mechanical and antibiotic bowel preparation is appropriate when colon resection is contemplated for vaginal reconstruction. When extra-abdominal tissues are used for reconstruction, preoperative use of enemas may be prudent to evacuate the rectum. Perioperative antibiotics should be provided.

Wharton-Sheares-George operation

This method is suitable for a flat perineum with no pouch. ^{[41, 42, 43]} Two dimples at the lower end of the vestigial müllerian ducts are identified in between the two labia just below the urethral orifice located within the normal hymen. Hegar dilators are gently pushed through the dimples with increasing size. The tunnels created along the vestigial müllerian ducts appear like double-barrel tunnels with a central septum.

The central septum is then excised to form a neovagina. A vaginal mould is placed in the neovagina for 2 months with repeated washing and cleaning, after which patients are advised intermittent self-dilatation until active sexual function.

Abbe-McIndoe operation

With the patient in the lithotomy position, an H- or Z -shaped incision is made on the perineum. Sharp and blunt dissection of the tissues interposed between the urinary tract and the rectum results in a cavity 10-12 cm in length. A Foley catheter in the urethra guides the dissection away from this structure. The proximity to the rectum is best gauged by placing an examining finger or Hegar dilator in the rectum.

A split-thickness skin graft is harvested from the inner thigh or buttocks prior to the perineal incision. Although a full-thickness graft tends to limit contraction, it transfers undesirable epithelial appendages. The authors know of no controlled prospective evaluation performed to determine the benefits of meshed grafts versus unmeshed grafts to guide this decision.

A suitably shaped piece of polyethylene foam approximately 5 cm wide and 15 cm long serves as a mold for the neovagina. This is covered with adhesive plastic. Also used is a mentor mold, which is inflated with saline once placed in the neovaginal space. The skin graft is sutured to the mold with Vicryl, with the skin surface facing the obturator. This construct is inserted into the soft-tissue pouch. The labia are sewn closed with silk sutures. The Foley catheter is left in place, and the patient is given stool softeners.

Postoperatively, the patient is kept in the hospital for 1 week; the mold is then removed in the operating room. The neovagina is irrigated, and any areas of granulation tissue are cauterized. The patient is then discharged with a mold (eg, a Young dilator), which is removed upon voiding and defecation.

Correction of vaginal atresia using tissue from intestine

The patient is placed in a lithotomy position to afford access to the abdomen and perineum. The abdomen is entered through a Pfannenstiel incision, and the status of the uterine remnant is evaluated. For the patient with müllerian agenesis, rudimentary uterine horns may be present with remnant fallopian tubes. The value of these structures, in terms of subsequent fertility, must be determined. The ovaries are typically normal and undisturbed. Patients with androgen insensitivity syndrome have male gonads, which are removed to prevent malignant transformation.

A segment of the sigmoid colon is chosen, with a major vascular pedicle supplying the mesenteric arcade. This segment is divided, and the adjacent intestinal tract is placed in continuity. Then, whether the graft is moved to the perineum in an isoperistaltic or a reverse peristaltic manner is decided on the basis of the length of the mesenteric pedicle.

The chosen proximal end is closed in two layers. The perineal dissection requires a circular or cruciate incision at the hymenal ring. Then, blunt dissection from below toward the peritoneal reflection allows this incision to be opened and the sigmoid to be passed into the newly created tract. A single-layer anastomosis is created to the hymenal regions by using absorbable sutures. Attempts are made to extraperitonealize the sigmoid. A petroleum jelly pack is placed in the neovagina to maintain apposition to the dissected tissues. The use of closed-suction drains is optional.

In patients presenting with variants of urogenital sinus, imperforate anus, and cloaca, a single-stage reconstructive pull-through procedure is an option. Because this procedure is not used in patients with pure vaginal agenesis, it is not discussed in detail in this article. ^{[44, 25]}

Management after colon interposition vaginoplasty entails the routine postoperative care performed after bowel resection. Vaginal packing covered with a sheet of petroleum gauze is left in place for approximately 48 hours. Radhakrishnan recommended examination performed under anesthesia after 1 week to separate any synechiae. ^[45] Progressive dilations are begun 3 weeks after surgery to prevent stricture at the distal anastomosis.

Postoperative care after an Abbe-McIndoe operation differs because no intra-abdominal concerns are present. The mold maintaining the neovaginal cavity remains in place for 7-10 days to optimize graft adherence. It is then removed in the operating room, and the patient is fitted with a mold (eg, a Young dilator). Examination can often be accomplished in lithotomy stirrups without the need for general anesthesia.

Creation of a neovagina by using skin grafts requires long-term use of vaginal dilators, molds, or sexual activity to avoid stenosis. Dilator trauma places the posterior wall abutting the rectum at some risk for neovaginal-rectal ulcers and fistulas that may require additional surgery.

Schult et al reported a standardized surgical evaluation and repair technique that avoids a diverting colostomy in most patients. ^[46] A perineal approach to fistulectomy with two-layer closure of the rectum and levatorplasty resulted in good functional outcomes in this series. The mean hospital stay was 13 days, and only two patients required additional surgery for late relapse of a fistula.

The potential for malignant transformation of the neovaginal epithelium is always present. This complication has been documented in at least one case report of incidental squamous cell carcinoma manifesting as a fistula. ^[47]

Complications related to the sigmoid neovagina tend to be related to excess mucous drainage and the potential for prolapse.

Follow-up care should emphasize maintenance of an adequate vaginal opening and length. Reassessment and progressive sizing of the dilators are carried out until the patient is regularly sexually active.