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Abstract Surgical management of intersex states con- nadal dysgenesis or true hermaphrodites. A third group
tinues to evolve such that today's eorts achieve near of patients warrant discussion: girls born with pure
normal cosmetic and functional results. In this article we urogenital sinus abnormalities, although these patients
discuss the reconstruction of the female genitalia in those may have nearly normal external genitalia.
that have had signi®cant androgen stimulation as well as Successful reconstruction is dependent upon an accu-
those with near normal external genitalia but signi®cant rate preoperative de®nition of the anatomy, particularly
urogenital sinus anomalies. The initial evaluation as well with the urogenital (UG) sinus (vaginal) abnormalities.
as postoperative follow-up management is addressed. Initial evaluation begins with a careful history and must
be followed by a detailed physical examination, with any
phallic enlargement or prominence of the corporal bodies
being noted. Labial anatomy and the location of the UG
One of the more devastating problems that can befall sinus opening in relation to the genitalia and rectum is
new parents is the ®nding that their child has ambiguous noted. Anterior displacement of the rectum is not un-
genitalia. The psychological implications can be over- common in the more severe forms of UG sinus and is
whelming. This is truly an emergency necessitating a related to the persistent cloaca. The size, location, and
team approach by the neonatologist, endocrinologist, consistency of any gonadal tissue must be noted.
geneticist, and pediatric urologist. This team should Prior to any operative procedure the child should
work quickly to identify the genetic sex and biochemical undergo an abdominal and pelvic ultrasound examina-
makeup of the child. The causes of genital ambiguity are tion to identify the uterus, vagina, and gonads. The key
varied, with congenital adrenal hyperplasia being by far consideration in surgical reconstruction is the anatomy
the most common. Today the genitalia of these children of the UG sinus. This is delineated ®rst by genitography,
can be reconstructed to achieve a nearly normal male or whereby contrast is injected retrogradely through the
female phenotype. This paper discusses only the recon- perineal meatus of the UG sinus. The level of the con-
struction of female genitalia. Patients being recon- ¯uence of the urethra and vagina should be demon-
structed along the female line generally fall into two strated. (Fig. 1). At times in the severely masculinized
groups: those with excessive androgen stimulation female with a high con¯uence the vagina may not be
(usually congenital adrenal hyperplasia) and those af- identi®ed radiographically and may be noted only on
¯icted with other intersex conditions such as mixed go- careful endoscopy by a few small punctuate openings at
or near an apparently ¯attened verumontanum [2].
Endoscopy is very critical in the de®nition of this anat-
R. C. Rink (&) omy. With experience, however, the location of the
Pediatric Urology, vaginal entrance into the UG sinus can usually be
James Whitcomb Riley Hospital for Children,
Indiana University School of Medicine, identi®ed. If the vagina has not been identi®ed on ge-
702 Barnhill Drive, Room 1739, nitography, a small catheter can be left in the vagina at
Indianapolis, IN 46202, USA the time of endoscopy for later contrast studies. These
Tel.: +1-317-274-7472; Fax: +1-317-274-7487 UG sinus abnormalities occur in a spectrum (Fig. 2).
e-mail: rrink@iucc.iupui.edu
Powell et al. [75] have classi®ed these into four types: I ±
M. C. Adams labial fusion; II ± distal con¯uence; III ± high, proximal
Pediatric Urology,
Vanderbilt University Medical Center,
con¯uence; and IV ± absent vagina. Although not every
435 Medical Arts Building, patient ®ts neatly into these groupings, for example,
Nashville, TN 37212-8591, USA those patients with duplicate vaginas, it can be helpful
213
for the surgeon to consider the level and nature of the pairs utilize a posteriorly based perineal ¯ap as described
con¯uence when planning the surgical management. by Fortuno et al. in 1964 [4] and preserve the phallic
The state of the art in reconstruction for ambiguous skin for labia minora construction as described by
genitalia has evolved over many years, with numerous Marberger [10]. In their classic paper in 1969, Hendren
surgeons making signi®cant contributions. The compo- and Crawford [8] recognized the variable anatomy of
nents of reconstruction involve clitoral reduction when the UG sinus. They noted that the vagina may insert
necessary, use of skin for labial reconstruction, and va- proximal to the external sphincter (suprasphincteric
ginoplasty. Nowhere is this evolution more evident than con¯uence) or distal to the external sphincter (inf-
in the history of surgery for the prominent clitoris. Early rasphincteric). Their description of the vaginal pull-
reconstruction involved simple amputation of the clito- through procedure for the high con¯uence was a major
ris. Since then, eorts at concealment, plications, reces- advance and remains the basis for reconstruction today.
sion, and reduction have evolved. Currently, every eort Unfortunately, this procedure as initially described can
is made to provide a normal cosmetic appearance be quite complex and, at times, leaves an isolated vagina
without sacri®cing sensation or vascularity of the glans. with some tendency toward stenosis; therefore, several
This is achieved by subtunical reduction of erectile tissue modi®cations have been described [16]. Vaginal recon-
as described by Kogan et al. [9] or by one of its modi- struction has evolved toward four basic procedures:
®cations. This allows preservation of the glans with its
1. The cut-back vaginoplasty (Fig. 3) should be used only
neurovascular supply intact along Buck's fascia and the
with simple labial fusion.
dorsal tunic of the corpora, yet the cavernous erectile
2. In the ¯ap vaginoplasty the posterior wall of the UG
tissue is excised to improve cosmetics and prevent later
sinus and the vagina are opened but the vagina is not
painful erections.
detached anteriorly. The procedure has been appro-
Similarly, many urologists have contributed to the
priately applied to those sinuses with an infrasph-
current state of reconstruction of the vagina. Many re-
incteric, low con¯uence.
Fig. 2a,b Spectrum of vaginal con¯uence with the urethra. a High (suprasphincteric) con¯uence. b Low (infrasphincteric) con¯uence
Fig. 3 Cut-back vaginoplasty
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Fig. 5a Initial outline for the skin incision in patients with clitoral
Operative techniques hypertrophy. b The glans is secured to the pubis or corporal stumps.
The proposed incision into the posterior wall of the UG sinus
Preoperatively the patients undergo an enema and the
medium-to-high vaginal con¯uence patients undergo
complete bowel preparation with Golytely. They receive plate between these lines (Fig. 5A). The incision is then
preoperative antibiotics. Endoscopy is performed and a made along these lines and carried around the corona of
Fogarty catheter may be left in place through the UG the phallus. Incisions are continued inferiorly around
sinus into the vagina with the balloon in¯ated. A the urethral meatus. The preservation of this urethral
marked sponge is placed into the rectum. In all but the plate in our experience has allowed improved cosmesis
most minor procedures we now prepare with povidone- and provides more blood supply to the glans (Fig. 6A).
iodine the entire lower half of the child from the xiphoid The phallus is then degloved, leaving the preputial skin
process to the toes. The legs are wrapped and the entire intact. The neurovascular bundle is identi®ed along
patient is placed through an aperture in the drapes. This Buck's fascia, and lateral incisions into the tunica of the
allows complete access to the perineum and abdomen; corpora are made along the phallus from the glans
the patient can easily be turned from the supine to the backward proximal to the corporal bifurcation. The
prone position (Fig. 4). We ®rst address any clitoral cavernous erectile tissue is then dissected from the infe-
hypertrophy. rior aspect of the dorsal tunics and is excised (Fig. 6B).
The proximal and distal ends of each corporal body are
then suture-ligated. This allows the glans to remain at-
Surgical management of the clitoris and low vaginal tached to the intact Buck's fascia with its neurovascular
con¯uence bundle and to the ventral plate. DeJong and Boemers [1]
have destroyed the proximal corporal tissue by dilating
In patients with ambiguity and signi®cant clitoral hy- the corpora with a metal sound. We have not found this
pertrophy we would ®rst repair the phallus prior to to be necessary. The glans can at this time be secured to
proceeding with vaginoplasty. With the patient in the the inferior aspect of the pubis or to the corporal
supine position the proposed incision is outlined with a stumps. Care should be taken not to place the glans too
skin scribe. The phallus is retracted by a stay suture, and high on the pubis.
parallel lines are drawn on either side of the clitoris, Following completion of the clitoral reduction, at-
leaving what would be the equivalent of the urethral tention is turned to the vaginoplasty. With the child
215
remaining in the supine position, a posteriorly based the labia minora (Fig. 7B). These two wings of tissue are
U-¯ap is drawn with its corners on either side of the sutured on either side of the ventral urethral plate and
perineal body near the rectum (Fig. 5A). The ¯ap length inferiorly alongside the new vaginal opening. The su-
in this situation is determined by the distance necessary to periorly placed labial scrotal folds can be mobilized and
reach the posterior vagina. It must be made wide enough moved inferiorly as a Y-V plasty to create more normal-
and long enough to allow a tension-free anastomosis appearing labia majora. These labia are sutured to the
without compromising its blood supply or the perineal new labia minora medially and to the inferior aspect of
body. After the incision is made to develop this posterior the lateral edges of the perineal ¯ap. If the glans appears
¯ap, it is retracted and dissection is carried out strictly in to be too prominent, glans reduction can be carried out.
the midline between the rectum and the UG sinus. In the This should be done by excision of the central ventral
reconstruction for a distal, low con¯uence the posterior tissue, leaving the two lateral aspects intact, as it has
aspect of the UG sinus is opened widely (Fig. 5B). It been shown that the neurovascular bundle enters later-
must be noted that the distal one-third of the vagina is ally and then converges medially at the tip [13]. This will
nearly always narrowed and the vagina must be opened avoid loss of the blood supply or of sensation.
proximal enough to enter into its normal caliber. In this
situation, sutures are individually placed through the
posteriorly based ¯ap and then into the split posterior Reconstruction of the high, or supersphincteric, vaginal
wall of the vagina. After all of these sutures have been con¯uence
placed, they are then tied (Fig. 7A). In such cases of low
con¯uence the anterior wall is not disturbed and, We would agree with Hendren that the high vaginal
therefore, no anterior ¯ap is necessary. Thus, the phallic takeo is best treated by complete separation of the
skin can be divided in the midline, similar to Byar's ¯aps vagina from the UG sinus and that some modi®cation of
for hypospadias repair, and moved inferiorly to create a vaginal pull-through procedure should be completed.
We would, however, extend this procedure to include
those that join in the midaspect of this spectrum. Sep-
aration of the vagina can be a very dicult dissection,
Fig. 7a The posterior ¯ap is sewn in place, which opens the UG sinus
widely. Proposed incision into the phallic skin. b The phallic skin is Fig. 8a,b Proposed incisions. a A posterior U-shaped ¯ap is created in
split in the midline to create the labia minoria. Proposed incisions at the pure UG sinus with the patient in the prone position. b For
the base of the labia majora to allow inferior placement alongside the ambiguity the clitoral recession is ®rst performed with the patient in
vagina the supine position
216
Fig. 9a±d Posterior prone approach. a The posterior ¯ap is elevated quires reconstruction of the rectum and sphincteric
and dissection proceeds in the midline, exposing the UG sinus. b A mechanism and, more importantly, temporary colos-
Deavor retractor elevates the intact rectum from the UG sinus and the
proposed incision into the posterior wall of the UG sinus is noted. c tomy. Hendren and Atala [7] have described lateral
The UG sinus is opened through the distal atretic vagina . The mobilization of the rectum to increase exposure but have
retractor, now in the vagina, exposes its anterior wall con¯uence with found this to be too dicult. Hendren [6] later described
the urethra. d The vagina is completely mobilized and the UG sinus is mobilization of the rectum in the prone jackknife posi-
closed in 2±3 layers as the neourethra. (Reprinted with permission
from Rink et al. [16])
tion, followed by its retraction posteriorly to expose the
high vagina. We have recently described a midline peri-
Fig. 10a The posterior vaginal wall is opened. b The anterior vaginal neal prone approach to the high UG sinus that requires
wall is opened. (Reprinted with permission from Rink et al. [16])
neither division nor mobilization of the rectum yet allows
excellent exposure for the high vagina, even in infants
particularly the separation of the anterior wall of the [16]. The following is a description of that approach.
vagina from the UG sinus, urethra, and bladder. It is For a pure UG sinus abnormality with normal ex-
made even more challenging by the limited exposure. ternal genitalia the procedure is started with the patient
With inaccurate or inadequate separation due to poor in the prone position with the legs spread and the pelvis
exposure, complications such as stricture, ®stula, or di- elevated on rolls. A posteriorly based U-¯ap is again
verticulum formation from a retained distal vagina may created and the ¯ap is retracted away from the UG si-
occur. Vaginal stenosis may also be a problem. Because nus. In the patient with ambiguity and a high vaginal
of these diculties, many surgeons have recommended takeo the procedure is begun with the child in the su-
delayed vaginoplasty. pine position as described above for the low con¯uence
Recently, Pena et al. [14] have recommended ap- until clitoral reduction has been completed and the
proaching these patients through a midsagittal posterior posteriorly based ¯ap has been mobilized (Fig. 8). At
incision, splitting the entire rectum in the midline to this point the child is rotated to the prone position, and
provide improved exposure of the UG sinus. This re- dissection is carried out in the midline along the UG
217