Transverse vaginal septum is a rare cause of congenital vaginal obstruction. Presenting symptoms are identical to imperforate hymen, but diagnosis is frequently delayed in the setting of a normal vulvovaginal exam. Transverse vaginal septum is diagnosed on pelvic ultrasound with hematocolpos identified cephalad to the septum. Concomitant uterine and renal malformations have been described. Magnetic resonance imaging (MRI) may be helpful in identifying the anatomy. Joki-Erkkila and Heinonen in 2003 identified 26 women with obstructive vaginal malformations. Thirteen underwent incision of an imperforate hymen and 3 excision of a complete transverse vaginal septum, with a mean follow-up period of 13 years. The remaining 10 had obstructive hemivagina and incision of a “longitudinal” vaginal septum with a mean follow-up period of 16 years. The women with transverse obstruction (imperforate hymen or transverse vaginal septum) were diagnosed within a month from their primary symptoms compared with 27 months for those with longitudinal obstruction. None with imperforate hymen required reoperation, but 2/3 with transverse vaginal septum did for vaginal constriction, and 3/10 with longitudinal vaginal septum had reexcision of their septum. All of the 10 women with longitudinal obstruction had uterine and renal malformations, whereas in those with a transverse vaginal obstruction, only 6 underwent renal evaluation, and of these, 2 had double ureters. Dysfunctional uterine bleeding was reported by 19% of those in the transverse group and 40% of those in the longitudinal obstruction group, dyspareunia was reported in 30% of the transverse and none in the longitudinal, and dysmenorrhea was reported in 19% of transverse and 20% of longitudinal. No endometriosis was found in women who subsequently had a laparotomy or laparoscopy (18/26). In the 14 who were attempting to conceive, difficulty with fertility was not diagnosed. Twenty-five (89%) out of twenty eight pregnancies ended in delivery, the live birth rate of the longitudinal group being 82%, and 94% in those with transverse obstruction. The authors concluded that accurate diagnosis, along with adequate treatment, can reduce the need for reoperations and that no specific longterm clinical gynecologic symptoms were identified in these women with obstructing vaginal anomalies.

Female genital mutilation (FGM) is defined by the World Health Organization as “all procedures that involve partial or total removal of the external female genitalia, or other injury to the female genital organs for non-medical reasons.” The WHO estimates that more than 100 million worldwide have undergone some type of FGM. It is estimated that approximately 250,000 girls and women with FGM are currently living in the United States. Female genital mutilation is practiced in Africa, Southeast Asia, and the Middle East, and is most commonly performed in puberty. Late complications of FGM include strictures, obstruction, and fistula formation. Complete obstruction of the vagina resulting in hematocolpos and hematometra has been reported. Women with FGM may seek gynecologic consultation to alleviate dyspareunia or dysmenorrhea or in preparation for childbirth. Defibulation is a vertical incision made to open scarring and reconstruct external genitalia. In a study by Nour et al. at the African Women’s Health Center at the Brigham and Women’s Hospital, 40 women were identified as having undergone defibulation between 1995 and 2003. Indications for defibulation were pregnancy (30%), dysmenorrhea (30%), apareunia (20%), and dyspareunia (20%). Almost 50% were found to have an intact clitoris underneath the scar. More than 90% of women stated they would highly recommend the procedure to others. 100% were satisfied with appearance and sexual function. The American Congress of
Obstetricians and Gynecologists has prepared a toolkit for clinicians caring for patients with FGM that includes photographs and detailed instructions.

Sexually ambiguous external genitalia defects of the UGS are remarkably constant in appearance, regardless of the etiology of the anomaly. Such genitalia differ only in their degree of malformation and occupy a range of positions somewhere intermediate to the genitalia of a normal female and that of a normal male. These anomalies can be anatomically identical to each other, whether their etiologic factor is congenital adrenal hyperplasia (CAH), male hermaphroditism, true hermaphroditism, or some other intersex syndrome. External genitalia proceeds along the female lines except in the presence of some virilizing influence acting on the developing embryo (i.e., androgens). The conversion of testosterone to dihydrotestosterone by 5α-reductase activity occurs in the skin of the external genitalia and UGS in early gestation. Masculinization of the external genitalia ensues in the presence of functional androgens regardless of genetic sex. In the case of female pseudohermaphroditism— XX chromosomes in the presence of a virilizing influence—the fusion of the scrotolabial folds may be sufficient to obscure or conceal the vagina from the outside or even to entirely suppress its formation. The urethra can be formed for varying distances or along the entire length of the phallus. Therefore, the operative procedure for reconstruction of ambiguous genitalia into feminine genitalia does not vary in its essential elements, regardless of the cause of the intersexuality. The common goals for the female reconstruction of ambiguous genitalia include reduction of clitoral size, creation of labia minora, and exteriorization of the vagina.

Any reconstruction of the external genitalia with the objective of producing normal female appearance and function requires a full understanding of the surgical anatomy. It is essential to accurately identify the site of communication of the vagina with the UGS. In their classic paper in 1969, Hendren and Crawford recognized the variability of the communication of the vaginal insertion into the UGS. Figure 24.4 illustrates the spectrum of vaginal communication with the urethra, with Figure 24.4A representative of a low distal communication (infrasphincteric) and Figure 24.4B representative of a high proximal communication (suprasphincteric). In 95% of cases, the vaginal communication is in relation to the caudal UGS derivatives (infrasphincteric) with the vagina communicating with that portion of the UGS that in a man gives rise to the membranous portion of the male urethra and that in the woman becomes the vaginal vestibule. If this usual relation is confirmed at surgery, the persistent, anomalous UGS may be incised to the vaginal communication without fear of disturbing the urinary sphincter. In less than 5% of cases, the vagina communicates high, with the portion of the UGS that becomes the prostatic urethra in the man or the entire urethra in the woman (suprasphincteric). Knowledge of the possible variants in communication of the vagina with the UGS is critical before entertaining surgical correction. Preoperatively, genitography showing the relationship of the UGS, urethra, vagina, and bladder may be helpful. Contrast is injected retrogradely through the perineal meatus of the UGS. Delineation of this anatomy can be elucidated at the time of surgery with the use of endoscopy to evaluate where the vagina communicates with the UGS. In 1989, Bargy and colleagues described the anatomic lesions in the intersexual states based on clinical and anatomic observations.

One objective of the reconstruction procedure for external genitalia is to delay the procedure until the anomalous structures are of a size to permit easy identification of all structures. As observed by Azziz and coworkers, vaginal repair may be delayed until menarche, when maturity and the desire for sexual activity are usually well established. There is present debate in the need for early reconstruction for the sole purpose of cosmesis to prevent embarrassment or anxiety to the patient’s family. Crouch and Creighton revisited the longstanding dictum of early surgical correction of ambiguous genitalia for intersex conditions. They report that some advocate the “one-stage” procedure in infancy, but that others advocate deferral of vaginal surgery until after puberty, especially given that many patients require further surgery at adolescence. It has been believed that the intersex child may be psychologically damaged by the “appearance” of uncorrected external genitalia if not performed at infancy, but unfortunately to date little research on this exists.

Most hermaphrodites reared as girls have a vagina or vaginal pouch, although in some instances, it is rudimentary. Only rarely is there no vagina, despite ambiguity of the external genitalia. The choice of operative procedure must conform to the observed anatomy. Thus, these choices are considered in the context of several categories based on anatomic structure of the anomaly.

The basic operation is, in essence, a modification of one described at length by Young that was previously performed successfully by various surgeons, notably in Europe. Patients with adrenal hyperplasia usually require only reconstruction of the external genitalia. However, when exploratory laparotomy is necessary to remove contradictory sex structures in other types of intersexuality or to establish the diagnosis, reconstruction of the genitalia may be considered at the same operation.

If an operation is deemed necessary at a very young age, the structures can be so small that it is impossible to introduce a finger into the UGS, and all tissues must be grasped throughout the operation with fine delicate tissue forceps. Operating loupes (2.5 to 3) are of great benefit to the surgeon. Small bipolar forceps and microscissors are also useful. Fine 5-0 or 6-0 synthetic absorbable sutures on an atraumatic needle are used throughout the procedure.

In cases of simple labial fusion, a cutback vaginoplasty (Fig. 24.5) would be sufficient to restore “normal” female genital anatomy. In cases of low vaginal confluence with the UGS (Fig. 24.4A), reconstruction may be done either by freeing the posterior vaginal wall and suturing up to the perineal external opening (Fig. 24.6) or—if a patient has copious subcutaneous fat and difficulty exists in approximating the vagina to the perineal skin—by use of a posterior flap technique, as used by Fortunoff and coworkers (Fig. 24.7).

Initially, the UGS may be thoroughly investigated with a small McCarthy panendoscope to determine accurately the position and size of the vaginal communication. If a sound or catheter can be easily introduced into the meatus of the UGS and into the vagina, use of the endoscope may be omitted. Special care is needed not to introduce the sound into the urethra. A sound accidentally introduced into the urethra poses the danger of incising the distal urethral meatus. After the UGS is incised (to within 2 or 3 cm of the anus), the urethral orifice may be identified (Fig. 24.6A, B). A small Foley catheter may then be introduced through the urinary meatus for purposes of identification throughout the remainder of the operation. To attach the edges of the vagina to the skin, it is usually necessary to free the vagina posteriorly and laterally to secure sufficient mobilization so that these structures meet with no tension. It is unnecessary to free the vagina anteriorly, because this requires its separation from the urethra. Sufficient mobilization can ordinarily be obtained by lateral and posterior dissection. When sufficient freedom has been attained, the edges of the vagina may be secured to the skin with interrupted 5-0 sutures on an atraumatic reverse cutting needle. In the infant, four or five sutures around the edge of the vagina are usually sufficient. The edges of the incised sinus membrane may then be sutured to the skin anteriorly (Fig. 24.6D-G). A small sponge impregnated with petroleum jelly may be introduced into the vagina to maintain its patency during the healing process. The indwelling catheter may be left in place for a few days until edema of the surrounding structures has subsided. An indwelling catheter is particularly useful in children with metabolic disorders that require accurate urine collection. A pressure dressing for 24 hours reduces the incidence of incisional hematoma.

Figure 24.7 illustrates vaginoplasty with a posterior flap as advocated by Fortunoff and is useful in cases with anticipated difficulty in bringing the vaginal orifice to the outside. Briefly, a posterior-based U-flap is drawn, with corners on either side of the perineal body near the rectum (Fig. 24.7A-C). This flap must be wide enough for tension-free anastomosis. This posterior flap is dissected in the midline and is carried out between the rectum and UGS.

Sutures are individually placed through the posterior-based flap and into the split posterior vagina. Sutures are tied after all have been placed. Because the anterior wall is not disturbed, no anterior flap is required. Finally, the phallic skin is divided in the midline and moved inferiorly to create the labia minora.

Surgical reconstruction of an enlarged clitoris has undergone significant evolution. Traditionally, the clitoris was simply amputated, and a nonfunctioning cosmetic clitoris was fashioned. Although several children so treated now have normal adult sexual function, the literature is lacking in followup data on large patient groups. Surgical efforts now focus on concealment, plication, resection, and reduction, with an attempt to provide a normal cosmesis without sacrificing sensation or vascularity of the glans.

The clitoral flap technique has provided a somewhat better cosmetic result than simple amputation. This procedure attempts to preserve a shell of the glans on a pedicle flap. The shaft of the clitoris is subtotally resected, and the stumps are reanastomosed (Fig. 24.8). The nerve supply to the glans is severed during this procedure, with the result that sensation in the glans is diminished. Sexual function, however, seems to be satisfactory.

Rajfer and colleagues have suggested a dorsal approach to the subtotal resection of the corpora (Fig. 24.9), which has the advantage of preserving the ventral nerve supply and which should preserve sensation in the glans. This approach is theoretically desirable and can be recommended for suitable cases. As mentioned earlier, however, lack of clitoral sensation does not seem to significantly affect the later sexual behavior of patients treated by procedures that sever the dorsal nerves to the glans.

In 1999, Baskin and colleagues described the anatomic studies of the human clitoris. As in the human penis, the nerves in the clitoris form an extensive network surrounding the tunica


of the corporeal body with a nerve-free zone at the 12-o’clock position. The normal clitoris has corporeal bodies that are smaller but analogous to those of the penis. The surgeon should be mindful of their function if extensive resection is considered with care to preserve the dorsal aspect of the glans.

Rink and Adams in 1998 reviewed feminizing genitoplasty. They advocated clitoral reduction without sacrificing sensation or vascularity of the glans, recommending a subtunical reduction of erectile tissue as described by Kogan and colleagues in the 1980s. The glans is preserved with its neurovascular supply intact along Buck fascia and the dorsal tunic of the corpora, yet the cavernous erectile tissue is excised. Figure 24.10 illustrates this surgical management; additionally, the phallus is degloved, and this skin is used to create the labia minora. An incision is made around the corona of the phallus and continued inferiorly around the urethral meatus. Preservation of this meatal plate improves cosmesis and increases blood supply to the glans. The neurovascular bundle is identified, with lateral incisions into the tunica of the corpora along the phallus from the glans backward proximal to the corporal bifurcation. The cavernous erectile tissue is dissected from the inferior aspect of the dorsal tunic and excised, and the proximal and dorsal corpora are suture ligated. The glans is secured to the inferior aspect of the pubis or to the corporal stumps.

In a pilot study of six women in 2003, Crouch and colleagues reported on genital sensation after feminizing genitoplasty for women with CAH. These women were assessed for thermal, vibratory, and light touch sensory thresholds in the clitoris and vagina using a genitosensory analyzer and Von Frey filaments. Highly abnormal results for sensation in the clitoris were found in the six women studied. In three who had an introitus capable of admitting a vaginal probe, the vaginal sensory data was considered normal. Given an abnormal clitoral sensation and normal vaginal sensation, the authors hypothesize that the abnormal clitoral findings may result from the surgical reconstruction rather than an effect of CAH. A self-administered sexual function assessment revealed that these women had sexual difficulties, particularly in the areas of infrequency of intercourse and anorgasmia. The authors voiced their concerns with the stated superiority of modern surgery. They stated that the vascularity of the glans remains vulnerable and that the risk of damage to the neurovascular bundles is inherent in any technique that
entails their separation from the corporal tissue. They concluded that even after seemingly successful clitoral reduction, the sensory function is significantly impaired, and this should be taken into consideration during counseling. Larger studies are presently under way to further evaluate these findings.