Operative Technique in Plastic Surgery – Kevin C.Chung

TECH FIG 3 • Distal hypospadias repair with TIP
urethroplasty. A. Preoperative examination reveals
coronal hypospadiac meatus (white arrow) and blind
urethral dimple (black arrow). B. Incision of urethral
plate (arrow). C. Urethroplasty repair (arrow). D.
Harvesting of dartos flap. E. Overlying urethral repair
with dartos flap. F. Hypospadias closure.
■ The dartos flap is used as an additional layer of coverage over the urethral
repair and can be harvested from either the ventral or dorsal penis, but
typically, the dorsal dartos is more supple and better vascularized (TECH
FIG 3D,E).
■ Rotate the dartos flap over the urethral repair and suture in place using 7-0
Vicryl sutures.
■ Close the glans in two layers. Use 6-0 PDS (Ethicon, Somerville, NJ) to
close the deep layer and 7-0 Vicryl (Ethicon, Somervill, NJ) to close the
superficial layers.
■ For skin closure, transpose the mucosal collar flaps ventrally, excise the
redundant tissue, and suture together with 7-0 Vicryl (TECH FIG 3F).
■ If there is inadequate ventral skin for closure, mobilize flaps from the
excessive dorsal preputial skin, transpose the flaps ventrally, and suture in
place with 7-0 Vicryl.
■ Secure the stent to the glans with two 6-0 Vicryl sutures.
■ Midshaft Hypospadias Repair

■ Transverse Preputial Island Flap (TPIF)
■ Performed in a single-stage repair.
■ The urethral plate is preserved as the posterior urethral wall and a TPIF is
used to make up the anterior wall of the neourethra.
■ Harvest a strip of skin from the inner preputial skin along its native blood
supply.
■ Rotate the flap ventrally and position over the urethral plate to form the
anterior urethral wall.
■ Suture the skin flap to the lateral edges of the urethral plate using a running
7-0 Vicryl suture.
■ Complete hypospadias repair as previously discussed (glanuloplasty, skin
coverage, etc.).
■ Inner Preputial Inlay Graft (TECH FIG 4)
■ An inner preputial graft is used to augment the urethral plate by placement of
the graft into the defect that results following incision of the urethral plate.
■ Measure the dimensions of the existing urethral plate to assess the length and
width of an inlay graft needed for tubularization.
■ Harvest an inner preputial graft of appropriate size.
■ Defat the graft.
■ Apply the tourniquet to reduce bleeding.
■ Make a deep incision within the native urethral plate from the hypospadiac
meatus to the tip of the penis.
■ Elevate the urethral plate off the underlying corporal bodies.
■ Place the inner preputial graft along the vascular bed and quilt it with 7-0
Vicryl sutures.
■ Mature the graft to the surrounding urethral plate tissue with interrupted 7-0
Vicryl sutures.
■ Fenestrate the graft with a micro knife to prevent hematoma formation
beneath the graft.
■ Following placement of the graft, one can perform a single-stage repair in
which the lateral aspect of the urethral plate on each side of the graft is
tubularized. Alternatively, one can perform a two-stage repair in which
tubularization is delayed for 6 months later to allow maturation and

neovascularization of the graft prior to creation of the neourethra. At our
institution, we generally favor a two-stage approach in this setting.
■ Rotate the mucosal collar flaps ventrally alongside the urethral plate and
suture with 7-0 Vicryl sutures.
■ The graft is allowed to heal for 6 months and then is tubularized during the
second stage.
■ If there is greater than 30-degree chordee such that dorsal plication is
insufficient to correct the curvature, the hypospadias repair should be
approached similarly to that of a proximal hypospadias.
TECH FIG 4 • Proximal hypospadias repair with inner
preputial graft (first stage). A. Proximal hypospadiac
meatus (arrow), narrow unhealthy urethral plate,
moderate depth of glans groove. B. Degloving penis
while preserving mucosal collars (arrows). C.
Harvesting inner preputial graft. D. Deep incision of
urethral plate (arrow). E. Elevation of lateral urethral

plate (arrow) off the corporal bodies. F. Preputial graft
overlying incised urethral plate. G. Graft sutured into
place.
■ Proximal Hypospadias Repair
■ Both one- and two-stage repairs for proximal hypospadias have been
described.
■ In most cases, there is significant (greater than 30 degrees) chordee that
cannot be corrected with dorsal plication. Consequently, a two-stage repair is
most often needed for proximal hypospadias repair.
■ One-stage repair is reserved for the rare cases in which there is minimal
chordee that can be corrected with dorsal plication.
■ If the urethral plate is healthy and sufficiently wide (greater than 7 mm), a
TIP urethroplasty can be performed.
■ If the urethral plate is healthy but too narrow to tubularize, a one-stage repair
can be performed with a transverse preputial island flap or an inner preputial
inlay flap, as described for midshaft hypospadias repair.
■ Two-stage repair is recommended for patient with significant chordee and/or
an unhealthy urethral plate.
■ The goal of the first stage is to correct the chordee and/or augment the
urethral plate for tubularization at the second stage.
■ Stage 1: Correction of Chordee
■ If there is greater than 30-degree chordee, dorsal plication is usually
insufficient and the following techniques should be used to correct the
curvature.
Division of Urethral Plate
■ Make a transverse incision in the urethral plate at the level of the maximal
curvature.
■ Elevate the urethra off the corporal bodies.
■ Excise dysgenetic tissue away from the corporal bodies.
■ Repeat artificial erection. If chordee is now less than 30 degrees, proceed
with dorsal plication.

Deep Transverse Incisions of Tunica Albuginea (DTITA)
■ If significant chordee still remains that is more than 30 degree despite
division of the urethral plate, the next step is to make multiple nearly full-
thickness incisions in the tunica albuginea at the level of the maximal
curvature.
■ No full-thickness incisions into the erectile tissue should be made and thus
should not affect future erectile function.
■ For severe chordee, DTITA is often insufficient and corporal body grafting is
necessary.
TECH FIG 5 • Division of urethral plate with corporal
body grafting. A. Significant chordee after degloving.
B. Chordee persists despite division of urethral plate.
C. Corporal body incision (arrow) with subsequent
defect. D. SIS grafting (arrow) of corporal body defect.
Corporal Body Grafting (TECH FIG 5)
■ Make a full-thickness ventral incision through the tunica albuginea at the
area of greatest curvature and extend to the lateral borders of the
neurovascular bundles.
■ Elevate the corporal wall off of the underlying erectile tissue to create a

diamond-shaped defect.
■ Graft the defect with either a dermal graft (harvested from the inguinal area)
or small intestine submucosal (SIS) (off-the-shelf xenograft material).
■ Suture the graft in place with a running 6-0 PDS.
Bridging of Urethral Plate Defect
■ Following division of the urethral plate and correction of chordee, it is
necessary to bridge the gap between the proximal and distal native urethral
plates.
■ Traditionally, this is performed utilizing Byars flaps. However, at our
institution, we favor an inner preputial skin flap.
■ Byars flaps (TECH FIG 6)
■ Make a midline dorsal incision in the preputial skin.
■ Rotate each preputial skin flap (Byars flap) ventrally along with their
respective dartos vascular pedicles.
TECH FIG 6 • Byars flaps using ventrally rotated
preputial flaps.
■ Bring the flaps together in the midline and interpose the combined Byars
flaps between the proximal and distal urethral plate. If the urethral plate in
the glans is flat and insufficient, the plate can be incised and the Byars flaps
can be placed into the defect with extension of the flaps out to the tip of
glans.
■ Suture the flaps in place utilizing 7-0 interrupted Vicryl sutures.
■ At the time of the second stage, the Byars flaps are tubularized to form the

urethra.
■ Because of the nature of the preputial skin used for Byars flaps, the
resultant tubularized urethra can be more prone to be hypermobile or
difficult to catheterize. Furthermore, in our experience, the patient is more
prone to urethral diverticula formation. Thus, we have favored use of an
inner preputial skin flap for bridging of the urethral defect.
■ Inner preputial skin flap (TECH FIG 7)
■ Harvest an appropriate-sized flap from the dorsal inner preputial skin while
preserving the blood supply from the dartos fascia.
■ Defat the distal one-third of the flap while maintaining the blood supply to
the proximal two-thirds.
■ Incise the glans urethral plate.
■ Inlay the defatted distal one-third of the flap into the incised distal urethra.
■ The proximal two-thirds of the flap is positioned along the shaft of the
penis.
■ The flap is quilted to the tunica albuginea utilizing interrupted 7-0 Vicryl
sutures.
■ Fenestrate the flap to reduce subcutaneous hematoma formation.
■ The urethra is tubularized at the time of the second-stage repair.

TECH FIG 7 • Division of urethral plate with preputial
inlay flap. A. This curvature of more than 30 degrees
required division of the urethral plate. B. Harvested
preputial inlay flap. C. Defatted distal third of preputial
flap (arrow). D. Preputial inlay flap quilted to tunica
albuginea.
■ Stage 2: Tubularization of the Urethra and
Glanuloplasty
■ Tubularization
■ Deglove the penis.
■ Induce artificial erection and ensure that any previous significant chordee
has been corrected.
■ Make parallel incisions along the inlay graft and residual urethral plate from

the level of the hypospadiac meatus to the distal aspect of the glans.
■ Tubularize the urethral plate over a 6 or 8 French stent and close in two
layers, with interrupted 7-0 PDS for the first layer and a running subcuticular
7-0 PDS for the second layer.
■ If available, harvest a vascularized dartos flap from the dorsum and
transpose over the repair with interrupted 7-0 PDS.
Tunica Vaginalis Flap
■ If a vascularized dartos flap is insufficient, harvest a tunica vaginalis flap.
■ Make a transverse scrotal incision over either the left or right scrotum.
■ Create a subdartos pouch.
■ Deliver the testicle.
■ Harvest a rectangular tunica vaginalis flap large enough to completely cover
the length of the repair, ensuring not to disrupt its proximal blood supply
along the cord structures.
■ Create a tunnel between the scrotal incision and the penoscrotal junction.
■ Deliver the tunica vaginalis flap and cover the entire repair.
■ Suture the tunica vaginalis flap in place with interrupted 7-0 Vicryl sutures.
■ Place the testicle into the developed subdartos pouch, and pexy it in place
with interrupted 4-0 chromic sutures at the 3 o’clock and 9 o’clock positions.
Completion
■ Perform glanuloplasty.
■ Close in two layers. Use 7-0 PDS to close the deep layer and 7-0 Vicryl to
close the superficial layers.
■ Mobilize and transpose the mucosal collar flaps ventrally and suture with 7-
0 Vicryl sutures.
■ Mobilize skin flaps as necessary for skin closure.
■ Place a catheter into the bladder for drainage purposes.
■ Buccal Mucosa Graft for Redo Hypospadias
Repair
■ Unfortunately, not all primary hypospadias repairs are successful. As a
result, some patients may require a redo hypospadias repair.
■ In instances of a redo hypospadias repair, the urethral plate is often no longer

healthy and requires excision.
■ In addition, inner preputial skin is typically not available for redo
hypospadias repairs.
■ As a result, a hypospadias repair using a buccal mucosa graft is required.
■ Harvest a buccal mucosa graft from either cheek or the upper/lower lip.
■ Mark out the edges of the desired graft.
■ Infiltrate the edges of the graft with 1% lidocaine with epinephrine.
■ Harvest the graft using scissors while preserving the underlying fatty tissue
(avoid dissecting too deep as this will risk injuring the underlying
muscles/nerves).
■ Control bleeding from the graft site using bipolar cautery.
■ Defat the graft.
■ Excise the scarred urethra and surrounding scar tissue to create a healthy
vascular bed along the surface of the corporal bodies. Make the buccal graft
as wide as possible.
■ Tack down the buccal mucosal graft to the location of the urethral plate with
7-0 Vicryl sutures in a quilted fashion.
■ Fenestrate the graft with a micro knife to prevent hematoma formation
beneath the graft.
■ Allow at least 6 months for the buccal graft to mature.
■ In a second stage, the buccal mucosa graft is tubularized and the
hypospadias repair is completed (TECH FIG 8).
TECH FIG 8 • Second stage of proximal hypospadias
repair with buccal mucosa. A. Healed first-stage repair
with buccal mucosa. B. Tubularization and closure of

buccal mucosa urethral plate.
PEARLS AND PITFALLS
Indications
■ Proximal and middle hypospadias should be repaired to improve
urinary and sexual function.
■ Distal hypospadias typically will not impact overall function but
correction can be performed to improve the appearance.
Approach
■ The approach for each hypospadias repair should be dictated by
the intraoperative assessment of the chordee and the urethral plate.
■ Counsel the patient and/or parents preoperatively regarding the
possibility of needing a staged repair.
Technique
■ Know the limitations of the tissue available for repair. Attempting
to do too much in a single stage may result in failure of the repair
and increased morbidity for the patient.
■ Use all available tissue to cover the urethral repair to reduce the
risk of urethrocutaneous fistula.
Complications
■ If a urethrocutaneous fistula or urethral diverticulum forms, the
distal urethra and meatus must be interrogated. If a stricture or
meatal stenosis is present, correction of the fistula or diverticulum
without addressing the distal obstruction will inevitably lead to
recurrence.
POSTOPERATIVE CARE
■ Dressing
■ At the conclusion of the surgery, a slightly compressive dressing is typically
placed around the penis to reduce swelling and promote hemostasis.
■ At our institution, we place a benzoin-soaked Owen gauze around the
incisions followed by Coban gauze secured with plastic tape.
■ Care should be taken to ensure that the dressing is not too tight as this can
decrease blood flow to the glans.
■ The dressing is removed by a physician or nurse 4 to 5 days postoperatively.
■ After removal of the dressing, a topical antibiotic ointment should be applied
to the incisions routinely for 1 to 2 weeks as the incisions continue to heal.
■ Urinary diversion

■ As mentioned previously, a urethral stent is sutured in place at the end of the
surgery.
■ Urinary diversion with a urethral stent allows for proper urethral healing and
prevents postoperative urinary retention.
■ Some physicians utilize suprapubic tubes for urinary diversion as these may
limit bladder spasms. In patients with a history of severe bladder spasms, a
suprapubic tube should be considered as strong bladder spasms can expel the
urethral catheter.
■ The urethral stent is typically removed 1 to 2 weeks postoperatively.
■ Postoperative antibiotics
■ Historically, postoperative antibiotics were routinely prescribed while the
urethral stent was in place.9 However, it is currently unclear whether
postoperative antibiotics confer a benefit.
■ While awaiting more conclusive studies, we routinely prescribe
postoperative antibiotics (ie, Bactrim, Keflex) while the urethral stent is in
place.
■ Postoperative evaluation
■ Take a thorough history of the patient’s urinary stream (direction, force,
urethral bulging during urination, leakage of urine from along the repair, etc.).
■ Carefully examine the repair and look for evidence of urethrocutaneous
fistula (early signs consist of erythema or inflammation along the urethra
proximal to the meatus).
■ Examine the meatus for evidence of meatal stenosis.
■ If concern for meatal stenosis or urethral stricture, uroflowmetry can help
gauge the strength of the stream.
OUTCOMES
■ A successful hypospadias repair should meet the following primary goals:
■ Patent urethra with a good functional urinary stream
■ Intact sexual function (ie, erections, ejaculation)
■ Good appearance
■ Patient satisfaction
■ Success of a hypospadias repair is directly correlated with the severity of the
hypospadias. Patients with a history of a proximal hypospadias and/or
significant chordee requiring a multistaged repair with multiple grafts are more
likely to have diminished function or worse appearance.
■ Patients with a history of more proximal hypospadias are more likely to have a

shorter phallus, more voiding dysfunction, and lower maximum urinary flow
compared to matched controls.10 Patients are also more likely to report erectile
dysfunction and decreased sexual quality of life as adults.11
■ However, a study of patient-reported outcomes found that patients with a
history of a successful hypospadias repair were more satisfied with the
appearance of their phallus and fewer voiding symptoms than compared to
patients who had uncorrected hypospadias.12
COMPLICATIONS
■ Complication rates following hypospadias repair are also directly related to the
severity of the hypospadias and the complexity of the repair.13
■ In expert hands, distal hypospadias using the TIP urethroplasty can be
performed with few complications.14 Most series quote a less than 10%
incidence of complications for distal hypospadias repair.
■ Short-term complications
■ Glans dehiscence is the most common acute postoperative complication seen
following urethroplasty repair and is typically due to closure of the glans
under too much tension or by too much ventral pressure from the urethral
stent. If the patient has a functional urinary flow and direction, the decision to
correct the glans dehiscence typically depends on the patient’s or parents’
concern for appearance.
■ Hematoma formation and infection are other potential short-term
complications.
■ Long-term complications
■ Urethrocutaneous fistula formation is the most commonly seen long-term
complication and typically presents within 1 year of repair.15
• Rates of urethrocutaneous fistula formation with proximal hypospadias
repairs have been reported as high as 30% to 50%.16,17
• The patient or parents typically notices urine leaking from a site proximal to
the meatus.
• Often, there is an associated area of inflammation or wound breakdown.
• A fistula is unlikely to close on its own with conservative management and
reoperation is typically necessary.
• In general, it is recommended to wait 6 to 12 months prior to reoperation to
allow inflammation and edema to resolve.
• Options for repair include primary closure vs redo urethroplasty.

• For primary closure, the fistula tract is excised and the urethra is closed with
multiple layers of well-vascularized tissue. For primary closures, it is
essential to calibrate the distal urethra to ensure no obstruction that will
increase risk of recurrence.
■ Meatal stenosis
• The rates of meatal stenosis range from 0% to 17%.18
• Obstruction created by meatal stenosis will increase the risk of
urethrocutaneous fistula and urethral diverticula formation.
• At follow-up appointments, it is important to query the patient and/or his
family regarding urine stream and to closely examine the meatus.
• If evidence of meatal stenosis, a simple meatotomy performed in the
operating room is typically sufficient.
■ Recurrent chordee
• If the patient develops recurrent significant ventral curvature of the penis,
the patient may require reoperation if there is concern that it will affect
urinary or sexual function in the future.
• For minor recurrent chordee (less than 30 degrees), dorsal plication is
usually sufficient.
• For significant chordee (≥30 degrees), repeat hypospadias repair may be
necessary (although this is uncommon).
■ Urethral diverticulum
• Patients with a urethral diverticulum typically describe a bulging of the
ventral aspect of the penis during urination. At completion of urination,
compression of the bulge will cause the expulsion of more urine.
• Urethral diverticula typically warrant correct. At the time of repair, excision
of the excess urethra is typically sufficient. However, it is crucial to evaluate
the distal urethra as there is likely an obstruction, which contributed to the
initial diverticulum formation.
■ Urethral stricture
• Patients who present with urethral strictures often complain of a long, slow
stream or having to strain to empty their bladder.
• Diagnosis is typically performed by cystourethroscopy.
• For mild, thin strictures, endoscopic management with direct visual incision
of the urethra (DVIU) can be attempted; however, in many instances, the
stricture will recur.

• For denser strictures or strictures that have failed endoscopic management,
reoperation is indicated.
REFERENCES
1. Canon S, Mosley B, Chipollini J, et al. Epidemiological assessment of
hypospadias by degree of severity. J Urol. 2012;188(6):2362-2366.
2. Paulozzi LJ, Erickson JD, Jackson RJ. Hypospadias trends in two US
surveillance systems. Pediatrics. 1997;100(5):831-834.
3. Cox MJ, Coplen DE, Austin PF. The incidence of disorders of sexual
differentiation and chromosomal abnormalities of cryptorchidism and
hypospadias stratified by meatal location. J Urol. 2008;180(6):2649-2652.
4. Shih EM, Graham JM. Review of genetic and environmental factors leading
to hypospadias. Eur J Med Genet. 2014;57(8):453-463.
5. Fichtner J, Filipas D, Mottrie AM, et al. Analysis of meatal location in 500
men: wide variation questions need for meatal advancement in all pediatric
anterior hypospadias cases. J Urol. 1995;154(2 Pt 2):
833-834.
6. Schlomer B, Breyer B, Copp H, et al. Do adult men with untreated
hypospadias have adverse outcomes? A pilot study using a social media
advertised survey. J Pediatr Urol. 2014;10(4):672-679.
7. Timing of elective surgery on the genitalia of male children with particular
reference to the risks, benefits, and psychological effects of surgery and
anesthesia. American Academy of Pediatrics. Pediatrics. 1996;97(4):590-594.
8. Wright I, Cole E, Farrokhyar F, et al. Effect of preoperative hormonal
stimulation on postoperative complication rates after proximal hypospadias
repair: a systematic review. J Urol. 2013;190(2):
652-659.
9. Hsieh MH, Wildenfels P, Gonzales ET. Surgical antibiotic practices among
pediatric urologists in the United States. J Pediatr Urol. 2011;7(2):192-197.
10. Örtqvist L, Fossum M, Andersson M, et al. Long-term followup of men born
with hypospadias: urological and cosmetic results. J Urol. 2015;193(3):975-
981.
11. Chertin B, Natsheh A, Ben-zion I, et al. Objective and subjective sexual
outcomes in adult patients after hypospadias repair performed in childhood. J
Urol. 2013;190(4 suppl):1556-1560.
12. Keays MA, Starke N, Lee SC, et al. Patient reported outcomes in
preoperative and postoperative patients with hypospadias. J Urol. 2016;195(4

Pt 2):1215-1220.
13. Arlen AM, Kirsch AJ, Leong T, et al. Further analysis of the Glans-Urethral
Meatus-Shaft (GMS) hypospadias score: correlation with postoperative
complications. J Pediatr Urol. 2015;11(2):71.e1-71.e5.
14. Cheng EY, Vemulapalli SN, Kropp BP, et al. Snodgrass hypospadias repair
with vascularized dartos flap: the perfect repair for virgin cases of
hypospadias? J Urol. 2002;168(4 Pt 2):1723-1726.
15. Snodgrass W, Villanueva C, Bush NC. Duration of follow-up to diagnose
hypospadias urethroplasty complications. J Pediatr Urol. 2014;10(2):208-211.
16. Snodgrass W, Yucel S. Tubularized incised plate for mid shaft and proximal
hypospadias repair. J Urol. 2007;177(2):698-702.
17. Mcnamara ER, Schaeffer AJ, Logvinenko T, et al. Management of proximal
hypospadias with 2-stage repair: 20-year experience. J Urol.
2015;194(4):1080-1085.
18. Wilkinson DJ, Farrelly P, Kenny SE. Outcomes in distal hypospadias: a
systematic review of the Mathieu and tubularized incised plate repairs. J
Pediatr Urol. 2012;8(3):307-312.

69
CHAPTER
Vaginal and Vulvar
Reconstruction
Elizabeth B. Yerkes and Julia Corcoran
DEFINITION
■ Neovaginal creation or augmentation of the vaginal canal may be requested in
several congenital conditions and in acquired vaginal stenosis. This chapter
will address vaginal and vulvar reconstruction in emerging and young adults. It
does not include vaginoplasty or labioplasty techniques that may be applied in
reconstruction of urogenital sinus in cases of ambiguous genitalia, intersex
states, and differences of sex development.
■ Vaginal agenesis or congenital vaginal atresia (Mayer-Rokitansky-Küster-
Hauser syndrome)
■ Vaginal agenesis or insufficiency in the setting of complete androgen
insensitivity and intersex/differences of sex development
■ Classic bladder exstrophy or cloacal exstrophy
■ Vaginal stenosis after early childhood surgery (congenital adrenal
hyperplasia, intersex/differences of sex development, or persistent cloaca)
■ Secondary changes after pelvic malignancy or graft versus host disease
(GVHD)
■ Vulvar reconstruction in the form of monsplasty and labioplasty may be
requested to align the laterally displaced or offset mons tissue and pubic hair
and to reconfigure and stabilize the labia minora after pubertal development in
congenital conditions such as exstrophy-epispadias and its covered variants.
■ Reduction labioplasty may be requested to address physically bothersome bulk
of the labia minora.
ANATOMY
■ The paired Müllerian (paramesonephric) bodies fuse in the midline and contact

the urogenital sinus. The sinovaginal bulb is induced and the vaginal plate is
formed.
■ Müllerian development occurs within first 10 weeks of gestation.
■ The labia minora are formed from the urogenital folds and the labia majora
and mons from the labioscrotal folds (FIG 1).
PATHOGENESIS
■ Absent or incomplete development of the Müllerian bodies results in absence
or atresia of the female genital tract.
■ Ovaries have a different embryologic development and are spared.
■ Failure of the fusion of the Müllerian bodies results in duplication of the
vagina or unilateral obstruction or atresia.
■ Anomalous descent of the urorectal septum in dividing the cloaca, as in
anorectal malformations, may interfere with fusion of the Müllerian bodies.
■ Renal development and a portion of skeletal development occur at a similar
time to Müllerian development, so anomalies may coexist.
■ Pelvic diastasis associated with abdominal wall defects such as bladder
exstrophy and cloacal exstrophy also results in nonfusion of the labial and
mons tissues. Bifid clitoris also occurs in these conditions.
■ Thickening of the labia minora with puberty results in extensive variation in
size and shape. Sufficient hypertrophy may occur that some clothing and
physical activity are physically bothersome.
■ Hygiene and tissue health may be a concern in young women with limited
mobility or ability for self-care.
NATURAL HISTORY
■ Vaginal agenesis or atresia does not resolve over time, but maturation of the
vulvar tissues and proximal vaginal segment, if present, may facilitate the
reconstruction.
■ If a uterus and proximal vagina are present, judiciously allowing the vagina to
distend with menstrual products may bring it closer to perineum or expand to
create more tissue for pull-through reconstruction.
■ Local tissue flaps or nongenital grafts can be used to bridge the distance to
the perineum.
■ Surgical benefits of this natural resource must be balanced against pain and
the potential for retrograde menstrual flow and peritoneal irritation and
deposits that could compromise future fertility.

PATIENT HISTORY AND PHYSICAL FINDINGS
■ Patient age and autonomy as well as family values and motivations will impact
discussions about goals and surgical options.
■ General discussions may have already involved the parent due to the
professional relationship from infancy.
■ Once ready to discuss interventions, private discussions with the patient are
essential but the parent must also be included for surgical discussions in
minors.
■ Patient readiness and goals for long-term results must be ascertained.
■ Readiness to undertake daily vaginal dilations for indefinite period.
■ Goal may be simply unobstructed menstrual flow or use of tampon.
■ Understanding of need for progressive dilations, and potential additional
procedures, thereafter for functional canal
■ Readiness or anticipated timeline for intimate sexual contact.
FIG 1 • Sagittal view of pelvic anatomy depicting
relationship of urethra, vagina, and rectum in typical
development.
■ Maturity level to be able to discuss with partner

■ Desire for heterosexual penetrative intercourse vs nonpenetrative sexual
activities
■ Anticipated life events (graduation, prom, marriage) that could be
complicated by surgical recovery or complications
■ Conflicts between family values/guarantor values and patient’s desire for
surgery
■ Anatomical and patient factors that may inform management
■ Quality of distal UG sinus/vaginal pouch and vulvar skin
• Presence of functional uterus and proximal vaginal segment
• Degree of estrogenization and maturation of tissues
■ Location of hair bearing tissues
■ Prior pelvic or genital surgery
■ Prior abdominal, urinary, or rectal surgical procedures
■ Prior chemotherapy or regional radiation therapy in setting of pelvic
malignancy
■ Inflammatory bowel disease
■ Dermatologic conditions
■ Continence status for urine and stool
■ Patient factors that may impact perioperative care and success
■ Resilience
■ Willingness to ask for and accept support in the perioperative period
■ Compliance
■ Lack of privacy to complete required care (college dorm)
■ Timing relative to next expected menstrual period
■ History of hypercoagulable state or bleeding diathesis
■ Increased surgical risk in terms of bleeding after deep dissection or from graft
donor site
■ Increased risk of perioperative thromboembolic complications after pelvic
surgery and prolonged bed rest
■ Gastrointestinal or dermatologic conditions
■ Continence status for urine and stool
IMAGING
■ Imaging is not specifically required for surgical planning, although pelvic
ultrasound or MRI is often obtained in the course of establishing certain
diagnoses and individual anatomy.

NONOPERATIVE MANAGEMENT
■ Daily serial dilation program can achieve a functional vaginal canal in
properly selected and motivated candidates.
■ Preferred initial therapy in vaginal agenesis, CAIS, and other
intersex/differences of sex development with a vaginal pit or pouch to guide
positioning of dilator.
■ May be used to progressively dilate vaginal stenosis.
■ Tissue health and surgical scarring may limit success.
■ Assess maturity and commitment to goals of program
■ Program is voluntary and timing should be dictated by patient.
■ Ongoing office support is important to verify technique and to enhance
success.
■ Daily or twice daily dilation for 10 to 20 minutes
■ Goal is to achieve and maintain vaginal depth while increasing caliber.
■ Dilations may cease when consistently sexually active but may otherwise
need maintenance dilations.
SURGICAL MANAGEMENT
■ Vaginoplasty
■ The individual anatomic situation will dictate whether neovagina
construction or augmentation of the caliber of the vaginal canal is required.
■ Skin or mucosal grafts, nongenital pedicled flaps (bowel), and local tissue
flaps may be incorporated in the vaginal reconstruction.
■ Choice of donor tissue may be based upon surgeon experience, patient
expectations and preferences, donor tissue availability, harvest site morbidity
and scarring, and donor tissue properties.
■ Monsplasty and labioplasty
■ Inferomedial rotation of mons tissue in bladder exstrophy or cloacal
exstrophy
• Addresses soft tissue asymmetry
• Allows for concomitant midline abdominal scar revision, with or without
umbilicoplasty if desired, and removal of non–hair-bearing midline tissue
• Melds with labia majora to create greater privacy for the clitoris, labia
minora, and vaginal vestibule
• Supports concomitant revision of asymmetric or mobile labia minora tissue
and/or vaginoplasty

■ Mobilization of tissue flaps to allow cosmetic and functional coverage of
glans clitoris if overexposed after prior feminizing genitoplasty
■ Reduction labioplasty
■ Excision or reconfiguration of physically bothersome hypertrophic labia
minora
Preoperative Planning
■ Review of prior operative notes in individuals with prior pelvic reconstructive
procedures.
■ A thorough pelvic examination with or without cystoscopy and vaginoscopy is
often helpful in surgical planning and patient preparation and counseling.
■ Offer anesthesia or sedation due to the invasive and physically or emotionally
uncomfortable nature of the examination.
■ Schedule one or more detailed consultations with patient to review all surgical
options in the context of individual anatomy and goals.
■ Surgical scheduling with consideration of menstrual cycle
■ Avoid menses on day of surgery and for first postoperative week when
vaginal stent or mold is used.
■ Continuous menstrual suppression may be considered perioperatively if
cycles predictably unpredictable.
■ Mechanical bowel preparation in patients with anorectal anomaly and in those
who will have bowel vaginoplasty. Enema advised in other vaginal cases.
Positioning
■ Sequential compression devices in place prior to positioning
■ Position in dorsal lithotomy with all pressure points carefully padded (FIG 2).
■ Adequate padding on bed and/or protective barrier on sacral bony
prominence, if present
■ Gentle support of atypical lumbosacral anatomy, if present
■ Attention to limited range of motion at hip or lower extremity with some
diagnoses
■ Standard to low dorsal lithotomy is preferred.
■ Minimizes stress on the spine and nerves in those with pre-existing
pathology.

FIG 2 • Dorsal lithotomy positioning. This depicts the
highest and widest positioning recommended. Position
should accommodate either two surgeons between legs
or one surgeon assisting patient’s hip. Functional status
and hip joint flexibility need to be considered for some
diagnoses.
• Acute neurologic change may occur with more exaggerated or prolonged
lithotomy.
■ Allows participation of cosurgeon from step stool at patient’s hip.
T E C H N I Q U E S
■ Augmentation of Caliber of Vaginal Canal:
Vaginoplasty and Introitoplasty
■ This technique is not neovagina or vaginal replacement but rather
augmentation of the existing vaginal canal caliber or circumference (TECH
FIG 1).
■ Introital, distal, or mid vaginal stenosis
■ Globally narrow but patent vaginal canal
■ Angulation of vaginal canal at introitus
• Anteriorly positioned and narrow introitus relative to axis of vaginal canal
after having undergone exstrophy repair techniques without deep

mobilization of the urogenital complex
• Laterally positioned and angulated introitus in vaginal duplication of
cloacal exstrophy
TECH FIG 1 • Narrow vaginal caliber can be
augmented with graft techniques. The relationship
between the posterior vaginal wall and rectum may be
closer than natural relationship in setting of prior
reconstruction.
■ Remarkably increases vaginal caliber but cannot increase vaginal length
due to presence of uterus.
• Relevant to exstrophy where cervix may sit more distally even when well
supported.
Enlargement of the Vaginal Canal Caliber
■ Radial incisions made at 3:00 and 9:00 with electrocautery (TECH FIG 2).
■ Lateral location of incisions least likely to injure urethra, bladder base, or
rectum.
■ Incisions sequentially deepened to expand vaginal caliber without
compromising support.

■ Desired caliber for penetrative intercourse: two fingers, spread to 2.5 to 3
fingerbreadths.
■ For tight stenosis or atresia or with anterior introitus/angulated canal, the
authors prefer oral (buccal) mucosa free graft.
TECH FIG 2 • Perineal approach to augmentation
procedures. Incisions at 3 and 9 o’clock minimize risk
of injury to urethra and rectum, more of a concern in
complex anomalies. The incisions are extended
cranially into healthy proximal vagina so that even and
adequate caliber established.
■ For isolated introital stenosis, a local perineal skin flap can be utilized, alone
or in combination with free graft, if it does not deform or compromise
surrounding anatomy.
Resurfacing With Native Tissues: Buccal Mucosa
■ Grafts are harvested from one or both cheeks, depending upon amount of
substrate required.
■ Graft is defatted and meshed by hand with strokes of scalpel. Alternatively,
it may be intermittently temporarily folded and incised with fine scissors
(TECH FIG 3A).
■ Triangular defects will be created bilaterally by the radial incisions.

TECH FIG 3 • A. Harvested buccal tissue has been
defatted and hand meshed to cover wider defect. For
use in bilateral defects, it is divided obliquely into two
triangular grafts to advance into defect. B,C. Two uses
of buccal for augmentation procedures. B. Triangular
grafts quilted in place bilaterally. Note that native
vaginal mucosa remains along posterior wall. C.
Young adult with deferred repair of urogenital sinus.
The vagina was mobilized toward the perineum and
spatulated. Buccal mucosa is being inset to bridge to
gap and to improve introital caliber. D. Vaginal mold
carefully inserted. Labia sutured across to prevent
expulsion.
■ The rectangular meshed buccal graft is cut obliquely into two triangles.
■ 4-0 Vicryl suture is preplaced in apex of graft and advanced to apex of the
triangular incision.
■ The meshed graft is expanded to fill the defect and quilted into the dissected
bed with resorbable suture (TECH FIG 3B,C). With the introitus and labia at
rest, vagina appears normal, not gaping.
■ Insert vaginal mold (TECH FIG 3D).
■ An inflatable vaginal mold with central drainage port is not currently
commercially available, but the concept elements are ideally applied to
employed substitute.
■ Graft stability without undue pressure on urethra; sterile; impermeable;
central drainage port for mucus and blood

■ Neovagina Construction
Perineal Dissection
■ Perineal dissection of rectovesical space to create adequate vaginal vault.
■ In the expected region of a vaginal opening, there may be a short pouch of
supple tissue or flat, firm granular tissue.
■ Selection of skin incisions allows the vestibular tissue to be incorporated as
local flaps to minimize risk of introital stenosis (TECH FIG 4).
TECH FIG 4 • Vaginal agenesis. Incisions avoid
infringing upon adjacent anatomy and can preserve
tissue to advance into the neovagina. Tissue in this
region is not uniformly good quality, however.
■ After elevation of flaps and initial electrocautery, the remainder of the
dissection occurs bluntly with judicious use of cautery.
■ Care is taken to maintain awareness of urinary tract and rectum during
dissection.
■ Desired caliber for penetrative intercourse: two fingers, spread to 2.5 to 3
fingerbreadths and at least 8 cm depth. Deep dissection will push peritoneum
away but not enter it.
Graft Substrates and Techniques
■ After the neovaginal bed has been dissected to allow adequate depth and
caliber and a natural axis, the skin or mucosal graft is harvested and prepared.
■ Historically, the McIndoe skin graft vaginoplasty was the preferred

approach.
■ Harvest classically is a split-thickness graft from the buttocks, although
full-thickness grafts have been described.
■ Graft is sewn over a vaginal mold with dermal side facing outward. The
mold and form are inserted into the neovaginal cavity and held in place for
1 week to allow graft to take.
■ Contracture, lack of natural lubrication, and visible harvest site are the
primary liabilities.
TECH FIG 5 • Healed buccal mucosa neovagina in
case of vaginal agenesis. Note the pliable but not lax
introitus and nice match to surrounding tissues.
■ Oral (buccal) mucosa free graft is a newer concept for neovagina.
■ It has excellent take and histologic characteristics more similar to vaginal
mucosa than skin1 (TECH FIG 5).
■ Neovagina creation and more extensive defects will require maximal
harvest from both cheeks.2
■ Graft is inset to line the cavity with interrupted resorbable sutures.
■ Vaginal mold carefully inserted and secured by sutures across labia.
■ Oral care: Chlorhexidine mouthwash and frequent cheek exercises to
prevent harvest site contracture during secondary healing.
■ Strict bed rest with log rolling and HOB less than 30 degrees for 6 to 7
days with vaginal mold in place, along with Foley catheter.
■ Peritoneal flap neovagina
■ Laparoscopic Davydov procedure3 involves multiple peritoneal flaps

mobilized and pulled through to the perineum to create a neovaginal
scaffold.
• Subsequent epithelialization by squamous metaplasia to have vaginal
properties.
■ Single peritoneal flap modification4 involves laparoscopically raising a 10
× 10 cm supravesical peritoneal flap, leaving an intact pedicle
infraumbilically.
• The flap is pulled through to the perineum and tubularized over a mold.
• The apex of the neovagina is created laparoscopically with a purse-string
suture.
• The pelvic peritoneum is run closed transversely.
• Stenting is required continually for 3 months until epithelialized, and then
nightly until consistently having penetrative intercourse.
Pedicled Flap Techniques
Sigmoid Colon and Ileum
■ Bowel vaginoplasty principles
■ Segment of small or large bowel is harvested on its mesentery and pulled
through the dissected rectovesical space for anastomosis at the perineum.
■ A 12- to 15-cm segment of sigmoid colon is isolated on a branch of the
inferior mesenteric artery via open or laparoscopic abdominal approach. The
distal most branches may be ligated to allow more mobility of the mesentery
and sigmoid segment5 (TECH FIG 6A).
■ The segment is maintained isoperistaltic. The sigmoid is reconstituted.
■ The segment is pulled through to the perineum and secured with interrupted
anastomosis of resorbable sutures.
■ The apex of the neovagina is oversewn in two layers and fixed to the sacral
promontory to minimize prolapse.
■ Monti principle
■ Described in small series, with limited follow-up, for sigmoid and ileum.
■ Bowel segment is isolated and opened and retubularized longitudinally.
• Sigmoid Monti: An 8- to 10-cm segment of ileum is opened near anterior
mesentery, such that mesentery forms a cap at apex. This configuration
most favorable to allow vagina to reach perineum and to support apex of
neovagina6 (TECH FIG 6B,C).
• Ileal Monti: 12-cm segment of ileum opened on antimesenteric border.

Two segments may be combined end to end.7
TECH FIG 6 • A. Harvest of sigmoid colon for
vaginoplasty. Knowledge of vascular supply to colon is
essential. Maintaining isoperistaltic orientation may
support mucus efflux. Support of the apex minimizes
global prolapse, but redundancy may also result in
prolapse. B. In sigmoid Monti construction, the
isolated 8- to 10-cm segment of sigmoid is unfurled to
its longest by opening close to the mesenteric border.
C. The sigmoid is then retubularized in the opposite
plane to create the neovagina. The neovagina is then
pulled down through the neovaginal bed created via
perineal and abdominal dissection.
■ Neovagina caliber based upon length of bowel segment harvested. Length
of neovagina determined by circumference of bowel segment. Sigmoid has
advantage in that regard.
■ Advantages over nonreconfigured sigmoid or ileum are the length of

segment required and the more mobile and less obtrusive mesentery.
■ Postoperative stenting is recommended with ileal Monti to maintain caliber
while the neovaginal cavity heals.7 A dilation program then ensues.
■ A petroleum jelly gauze roll splint may be left for 24 to 48 hours after
sigmoid neovagina. Longer-term stenting is not required with sigmoid
neovagina, likely due to bulk of the tissue and natural interior caliber, but
dilation of the introitus may be advantageous to prevent stenosis.
■ Both segments provide self-lubrication, but the mucus from colonic
neovagina may be more problematic in terms of quantity and odor,
particularly if redundant segment or introital stenosis.
■ Prolapse is uncommon if the bowel segment is designed to limit
redundancy and if well supported. Redundant tissue may be excised, and
dilations are advisable while healing.
■ Diversion colitis related to isolation of the colonic segment from the
remainder of the GI tract may result in pain, bleeding, and discharge.
Primary gastrointestinal conditions, including malignancy, may also afflict
the segment.
Pudendal Thigh (Singapore) Flaps
■ This is a fasciocutaneous axial sensate flap based on posterior labial vessels
and terminal branches of the pudendal nerve8,9 (TECH FIG 7A,B).
■ Donor area usually has not been involved in previous urogenital and lower
abdominal wall reconstructions.
■ Donor site closes in a primary manner and hides parallel to the labia majora
in the groin/leg sulcus.
■ Does not need postoperative stenting or routine dilation.
■ Sensation is from the local neural plexus.
■ Vagina will be lined with epithelium. Desquamation leads to secretion of
sebaceous material. When used to create a total neovagina, lubrication is
required for penetrative sexual activity.
■ Flap is designed lateral to the labia majora, raised distal to proximal
including the deep fascia.
■ Rotation into an expanded vaginal canal or introitus occurs most naturally
at the 4 and 8 o’clock positions (rather than 3 and 9 o’clock vaginal
incisions) with minimal tension on the point of rotation (TECH FIG 7C).
■ Inset with resorbable suture.
■ Close donor in layers, also with resorbable suture material. No need for

drains.
■ For total vaginal reconstruction, bilateral flaps can be raised, 15 × 6 cm, and
sewn to each other with epithelial side lining the tube, which becomes the
neovaginal canal. The neovagina is then situated into the dissected space
between bladder and rectum and suspended well to the most superior point to
maintain length and prevent prolapse. Drainage is recommended in this
situation.
TECH FIG 7 • A. The fasciocutaneous pudendal thigh
flap is based on posterior labial vessels and branches
of the pudendal nerve. B. The plane of dissection for
harvest of the flap, as well as the posterior cutaneous
relaxing incision that allows tunneling of the graft. C.

Tunneling and union of the paired pudendal thigh flaps
in complete vaginal replacement. The flaps must be
well anchored to the apex of the prepared neovaginal
bed to maintain length and decrease risk of prolapse.
■ Monsplasty and Labioplasty
■ Surgeon must determine well in advance of planned procedure whether the
mons tissue is adequate for rotational flaps or whether tissue expansion is
required.
■ Goals:
■ Remove midline shiny hairless skin, revising adjacent midline surgical scar
■ Rotate hair-bearing tissue to midline
■ Create a natural contour of mons and labia majora to allow the clitoris,
labia minora and vaginal vestibule to enjoy less overt exposure.
• Interestingly, the inferomedial rotation to cover the genitalia in females is
opposite of the goal in males of evening up the pubic hairline and tissue
while visually increasing penile stature through upward rotation of tissues.
■ Pubic hair can be clipped but not shaved prior to marking the hair-bearing
rotational flaps.
■ If vaginoplasty will be performed as well, it will be completed prior to the
monsplasty and labioplasty.
■ Rotation flaps are marked with relaxing incisions (TECH FIG 8A). Midline
scar excision and umbilicoplasty incisions are planned as well, if part of the
requested procedure.
■ The labia minora may require advancement or partial excision for improved
cosmesis or patient comfort/hygiene (TECH FIG 8B).
■ Planning occurs along with the monsplasty but the actual labial work is
easier to visualize prior to recreating the mons.
■ A semilunar incision creates a non–hair-bearing skin flap just cranial to the
labial complex. This is previously operated tissue (TECH FIG 8C).
■ Resultant flap will be turned down to become the underside of the mons-
labial confluence and will form part of a hood for clitoral unit.
■ The semilunar flap is turned down and rolled to deepen the “hood” or to line
the anterior fusion of the labia majora and mons (TECH FIG 8D).

■ The hair-bearing mons flaps are rotated toward the midline and sutured to
each other in layers with resorbable sutures. The inferior or caudal edge of
the flaps is sutured to the “hood”/ledge with resorbable suture (TECH FIG
8E).
■ Expanded flaps require postoperative drains for 4 to 6 days until the flap is
well seated.
TECH FIG 8 • The surgical steps are depicted from
two different patients. A. Exposed urethra and genital
tissues to be better concealed with monsplasty.
Rotational flaps are marked in preparation for
monsplasty. B. Advancement of mobile or anteriorly
mounded labia minora for improved concealment of
clitoris and urethra after childhood repair of exstrophy.
C. Semilunar incision is raised from previously
operated midline hairless tissue. Tissue expanders
have been removed and midline closure preplanned
with staples. This hairless flap will be turned down to
conceal the more intimate anatomy and to line the
underside of the monsplasty. D. Semilunar flap turned
down. Note that the flap has been trimmed in the

midline and closed vertically to conceal the upper
labia minora. E. Final appearance after monsplasty
flaps rotated and approximated. Scar revision,
umbilicoplasty and vaginoplasty were also completed
in this patient.
■ Reduction Labioplasty
■ Goal is to restore comfort rather than to address cosmetic misgivings (TECH
FIG 9). Reassurance regarding wide spectrum of “normal” is central.
■ Defer any intervention until adequate time for adjustments has occurred, as
well as development one or more years beyond menses. Potential to “regrow”
bulk if development incomplete.
■ For whichever is used of the three techniques described below, adhere to the
following:
■ Place traction sutures in the tips of the labia minora to gauge tissue to be
excised.
■ Mark inner and outer surface of labia minora.
■ Mark both sides for symmetry prior to revising either labia.
■ Perform a two-layer closure.
TECH FIG 9 • Marked, physically bothersome
hypertrophy of the labia minora is seen with the labia
at rest.

Simple Excision and Closure
■ This method offers easiest recovery and least chance to redevelop bulk.
There is no potential for flap dehiscence.
■ Greater risk of exposing clitoris or creating irregularity anteriorly, due to
more difficulty blending into the naturally folded clitoral hood contour
(TECH FIG 10A).
■ Skin is incised sharply and dartos divided with electrocautery.
■ Dartos is run longitudinally with resorbable suture.
■ Skin is also closed with continuous resorbable suture.
■ Suture line is anterior and may result in more friction during healing but this
is not generally symptomatic (TECH FIG 10B,C).
Wedge Excision With Advancement
■ This method is more prone to postoperative edema and discomfort, more
prone to redevelop bulk, and more prone to dehiscence without layered
closure.
■ Wedge excision and advancement is a full-thickness resection of the central
portion of the hypertrophied labia.
■ The apex of the flap will advance to the bottom of the wedge incision
(TECH FIG 11A).
■ First layer closure with running resorbable suture begins in corner of wedge
incision, gradually anchoring the flap.
■ Skin closure is hidden on lateral and medial surfaces of the labia minora.
Longer lasting resorbable suture is used on the cutaneous layer at the apex of
the flap (TECH FIG 11B,C).
TECH FIG 10 • A. Retracted labia marked for simple
excision. Care must be taken to taper onto the clitoral

hood to avoid cranial irregularity and to avoid
exposing glans clitoris. B. Labia at rest after simple
excision. C. Immediate postoperative appearance with
labia spread. The anterior suture line is not typically
irritating, as it is concealed by the labia majora.
TECH FIG 11 • A. Retracted labia marked for wedge
resection and advancement. The running closure of
the dartos begins in the apex and progresses down to
the tip of the flap. Longer lasting resorbable sutures
are recommended at the tip. B. Postoperative
appearance in a different patient. Note that the suture
line is anterior with simple excision but here is hidden
in the inner and outer labial surfaces. C. The
appearance is similar at rest to simple excision.
Hybrid Approach
■ Useful in cases in which the labia minora are globally enlarged with
posterior insertion.
■ Offers benefits of hidden suture line and natural blending with clitoral hood,
with a less extensive flap recession than the wedge excision (TECH FIG 12).
■ Rationale for two-layer closure, regardless of technique:
■ Finlike rather than flat contour of labia
■ Hemostasis, decreased risk of hematoma. Insurance against dehiscence in
advancement cases.

TECH FIG 12 • Retracted labia marked for hybrid
excision with partial advancement and partial simple
closure. Again, the running closure of the dartos
begins in the apex and progresses down to the tip of
the flap. In this approach, the flap is shorter so the
posterior aspect of the closure will be simple two-layer
closure.
PEARLS AND PITFALLS
Patient
readiness
■ Older teens may profess readiness for vaginal reconstruction with
immediate goal of ease of tampon use and desire to prepare for
future sexual activity, but they may primarily desire to “get it over
with.”
■ Unless imminently prepared for sexual activity and willing to
accept support, they may lack the emotional maturity for the
procedure and to adhere to a postoperative dilation program.
Operative
preparation
■ A strong understanding of pelvic anatomy and potential congenital
or postsurgical variations is relevant when dissecting space for
neovagina or widening the vaginal canal.
■ Injury to the urethra, bladder, or rectum significantly changes the
operative experience and potentially long-term functionality.
Hemostasis
■ Address vault bleeding with packing rather than blind coagulation
in deep cavity.
■ Remain alert to adjacent pelvic structures.

Progression
of
postoperative
therapies
■ Even with a highly motivated and mature patient, frequent office
visits are recommended early postoperatively.
■ Provider reinforces or redirects patient regarding adequacy of axis
and depth of daily use of dilators.
Patient
selection for
reduction
labioplasty
■ Reduction of prominent labia minora for cosmetic purposes is
strongly discouraged.
■ Reassure about normal variations in prominence of labia minora in
girls and women.
■ For physical bother due to bulky tissue and friction/irritation from
clothing:
■ Instruct in trial of manually tucking redundant tissue in after
bathing and toileting.
■ Instruct in application of bland ointment nightly to manage
irritation. Ointment in daytime may increase bother.
■ Defer surgical management until estrogenization and maturation of
tissues is secure.
■ Surprising “regrowth” of tissues may occur if maturation in early
evolution.
Labioplasty
and perineal
care
■ Dehiscence of labial flaps can result in prolonged healing,
unfavorable cosmetic result, or asymmetry.
■ Two-layer closure of the labioplasty, particularly at the tip of the
labia, reduces risk of separation.
■ Moist perineal environment and oral or topical antibiotic use
increase risk of candidal infection and healing concerns. Nystatin
powder to groin creases may be protective.
POSTOPERATIVE CARE
■ Vaginal packing and vaginal stents/molds
■ Inflatable and solid forms have been utilized. None currently commercially
available.
■ Not required for pudendal thigh flaps.
■ Required for approximately 7 days for graft take with skin or oral mucosa.
■ Required all day and night for 3 months in peritoneal flaps due to
requirement of squamous metaplasia.
■ Mouth care after buccal mucosa harvest
■ Donor site heals by secondary intention and forms fibrinoid plaque within 2
days.
■ Chlorhexidine mouthwash rinses several times daily.

■ Mouth exercises minimize likelihood of function-limiting donor contraction.
Open wide. Puff check. Mindful manual massage of site by tongue.
■ Home dilation program: as described above for each technique. Frequent visits
to reinforce technique and improve compliance.
■ Activity restrictions
■ Avoid lifting, straddle stance or activity, squatting, or kneeling with perineal
pressure for 3 to 4 weeks. Driving restrictions based upon approach.
■ No tampon use or sexual activity until adequate healing determined by
surgeon.
OUTCOMES
■ A paucity of data exists regarding objective short- and long-term surgical
outcomes as well as validated patient-reported outcomes.
■ Female Sexual Function Inventory scores compared favorably to controls in
large peritoneal flap series.4
■ Long-term results after sigmoid vaginoplasty report high level of success and
patient satisfaction.5,10
• However, decreased sexual function with abnormal depression indices may
occur highlighting importance of pre- and postoperative counseling and
support.11
■ Tissue quality and match and adequacy of vaginal caliber are very good after
buccal mucosa vaginoplasty.1,2
■ Sexual activity is allowed by successful maintenance dilations. If pregnancy is
achieved, consultation with maternal fetal medicine and caesarean delivery is
recommended in neovagina, regardless of type.
COMPLICATIONS
■ Injury to surrounding structures: urethra, bladder base/ureter, or rectum may
occur with dissection of the neovaginal cavity
■ Vaginal contracture or stricture
■ Introital stenosis
■ Prolapse of pedicled tissues
■ Bothersome discharge (sigmoid)
■ Secondary changes within donor substrate (eg, chronic inflammation,
inflammatory bowel, malignancy)
■ Unsatisfactory cosmetic or functional result

REFERENCES
1. DaJusta D, Granberg C, Baker LA. Vaginoplasty with autologous buccal
mucosa graft: a close histologic match. J Urol. 2011;185:e103.
2. Nambiar A, Arevalo MK, Grimsby G, et al. Autologous buccal mucosa
vaginoplasty in 22 patients with congenital adrenal hyperplasia. Presented at
the Pediatric Urology Fall Congress, Montreal, Canada, September 2017.
3. Fedele L, Frontino G, Ciappina N, et al. Creation of a neovagina by
Davydov’s laparoscopic modified technique in patients with Rokitansky
syndrome. Am J Obstet Gynecol. 2010;202:33.e1-33.e6.
4. Zhao X, Ma J, Wang Y, et al. Laparoscopic vaginoplasty using a single
peritoneal flap: 10 years of experience in the creation of neovagina inpatients
with Mayer-Rokitansky-Kuster-Hauser syndrome. Fertil Steril. 2015;104:241-
247.
5. Nowier A, Esmat M, Hamza RT. Surgical and functional outcomes of sigmoid
vaginoplasty among patients with variants of disorders of sex development. Int
Braz J Urol. 2012;38:380-388.
6. Garcia-Roig M, Castellan M, Gonzalez J, et al. Sigmoid vaginoplasty with a
modified single Monti tube: a pediatric case series. J Urol. 2014;191:1537-
1542.
7. Trombetta C, Liguoria G, Siracusano S, et al. Transverse retubularized ileal
vaginoplasty: a new application of the Monti principle—preliminary report.
Eur Urol. 2005;48:1018-1024.
8. Joseph VT. Pudendal-thigh flap vaginoplasty in the reconstruction of genital
anomalies. J Pediatr Surg. 1997;32:62-65.
9. Monstrey S, Blondeel P, VanLanduyt K, et al. The versatility of the pudendal
thigh flap used as an island flap. Plast Reconstr Surg. 2001;107:719-725.
10. Kim SK, Park JW, Lim KR, Lee KC. Is rectosigmoid vaginoplasty still
useful? Arch Plast Surg. 2017;44:48-52.
11. Djordjevic M, Bizic M, Kojovic V, et al. Sigmoid vaginoplasty and its impact
on psychosocial and sexual life in patients with vaginal agenesis. J Urol.
2016;195:e791.

70
CHAPTERAmbiguous Genitalia
Deborah L. Jacobson and Elizabeth B. Yerkes
DEFINITION
■ Individuals born with ambiguous genitalia have external genitalia that are not
fully typical male or female in appearance. Others have developmental
differences that are not externally apparent but may impact future function or
well-being. These groups have some degree of discordance between genetic,
gonadal, and phenotypic sexual characteristics.
■ Medical nomenclature used over the decades was well intentioned but has
been viewed as pejorative. Accordingly, nomenclature with multiple
subterminologies has evolved with the goal of correcting this situation.
■ Hermaphroditism, pseudohermaphroditism, intersex state/disorder, and
disorders of sex development (DSD) have been used to describe the state of
having ambiguous or nontypical genitalia with or without chromosomal or
gonadal anomaly.
■ Even the most recent nomenclature, which originated from a consensus of
experts, affected individuals, and advocates, is not uniformly accepted by the
community. “Disorder” is not widely embraced as a desired description.
■ Whereas genital ambiguity and differences of sex development have
historically been considered pathologic and in need of surgical correction,
contemporary viewpoints acknowledge many phenotypic variations on the
continuum of human genital development.
■ The authors understand that surgical reconstruction of ambiguous genitalia is a
controversial topic and do not presuppose that surgical intervention is
necessary for, or would be preferred by, any given individual.
■ This chapter will cover the technical aspects of genital reconstruction;
however, surgical management should only be undertaken after extensive

counseling and shared decision-making. Longitudinal multidisciplinary care
for the individual and family is strongly encouraged.
■ This chapter will focus on feminizing genital procedures and the diagnoses
that could involve decision-making for these types of procedures.
ANATOMY
■ Clitoris
■ The corpora cavernosa (erectile bodies) of the clitoris originate proximally as
paired crura, swell medially to form the clitoral bulbs, and unite distally.
■ The paired cavernosa support a glans covered by a prepuce of labia minora.
MRI imaging studies performed by O’Connell et al demonstrate that the glans
lies caudal to the corporal bodies, leading to a natural hairpin curve.1
■ Autonomic input is via the paired cavernous nerves, which originate from the
vaginal plexus at the 10 and 2 o’clock positions along the vagina and course
dorsally along the proximal urethra.2 The nerves travel with the cavernous
arteries and enter the clitoral bodies under the pubic arch.
■ Somatic supply is via the paired dorsal nerves of the clitoris, which originate
from the pudendal nerves, run dorsolaterally along the crura and ischiopubic
rami at approximately the 11 and 1 o’clock positions, and enter the glans at
the corona. The nerves branch, fanning out along the clitoral bodies dorsally
and laterally. Nerves are absent in the dorsal midline and deficient ventrally.
■ The cavernous arteries originate from the internal pudendal artery, travel with
the cavernous nerves, and enter the clitoral body under the pubic arch.
Dissection between the crura at the pubic arch should be avoided.
■ Variations from typical clitoral anatomy should be expected in patients with
virilization of female genital tissue.
■ Labia
■ The labia majora embryologically correspond to the male scrotum. They are
separated by the vulvar cleft, which contains the labia minora and clitoris.
Together the structures conceal the vestibule.
■ The labia minora and urethral plate correspond to the male anterior urethra.
The labia join superiorly to frame the glans clitoris as the clitoral hood.
■ Vagina
■ The vagina consists of a rugated mucous membrane fixed to a bilayered
muscular invagination. It is suspended from the levator ani ventrally, the
rectovaginal septum dorsally, and the cervix apically.
■ Arterial supply is via uterine, vaginal, and internal pudendal branches

coursing along the anterolateral surface of the vagina.
■ Innervation originates anterolaterally via autonomic branches of the
uterovaginal plexus and somatic branches of the pudendal nerve.
■ The ureters pass adjacent to the lateral margins of the vagina and insert into
the base of the urinary bladder.
PATHOGENESIS
■ Cloacal development
■ The cloacal membrane folds with differential embryonic growth beginning in
week 4, and the cloaca partitioned into anterior (urogenital sinus—UG sinus)
and posterior (hindgut) compartments with the descent of the urorectal
septum.
■ Disruption of normal cloacal development can lead to persistent cloaca or
UG sinus anomalies.
■ Development of the external genitalia
■ Fetal androgenization leads to elongation of the genital tubercle, fusion of the
urethral folds, and fusion of the labioscrotal folds.
■ Development of the abdominal wall
■ Failure of mesodermal ingrowth or premature rupture of the cloacal
membrane leads to bladder or cloacal exstrophy.
■ Development of the UG sinus
■ Caudal paramesonephric (Müllerian) duct deficiencies may lead to agenesis
of the proximal vagina and variable anatomy of the uterus/fallopian tubes
(Mayer-Rokitansky-Küster-Hauser syndrome).
■ Lateral paramesonephric (Müllerian) fusion anomalies may lead to uterus
didelphys, bicornuate uterus, or unicornuate uterus.
■ Deficiency of the distal UG sinus may lead to vaginal atresia.
■ Incomplete vaginal canalization may lead to an imperforate hymen or
transverse vaginal septum.
NATURAL HISTORY
■ The natural history of an individual in this population, or the population as a
whole, is not entirely known.
■ For androgenized individuals, it is unclear what the prenatal hormonal milieu
(both in terms of absolute androgen levels and the response of developing
genital/nervous tissues) portends for future gender identity or sexuality.
■ For virilized females (such as patients with congenital adrenal hyperplasia) in

whom medical control is irregular, continued irreversible changes may occur in
the genital tissues.
■ The psychosocial outcomes of early vs delayed surgery remain unclear, as
does the definition and likelihood of surgical success.
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Antenatal course: complications, history of prematurity, and other anomalies
■ Evidence of maternal exposure to endogenous or exogenous androgen
■ Severe acne, abnormal hair growth, clitoral enlargement, or Cushingoid
appearance
■ Drugs, virilizing tumors, or adrenal disorders
■ Family history:
■ Known chromosomal anomalies
■ Fetal/neonatal demise
■ Genital ambiguity or urologic anomalies
■ Infertility or consanguinity
■ Physical examination
■ General: overall appearance, dysmorphic features, and general infant well-
being
■ Spine: gross deformity or neurocutaneous marks
• Anomalous findings may indicate genetic syndromes or fetal exposures.
• Tethered cord or occult spinal cord anomalies are common with select
diagnoses.
■ Abdomen: marked distention, hernias, suprapubic masses, and wall
abnormalities
• May indicate bladder outlet obstruction, hydrometrocolpos, or urinary
ascites
■ Perineum
• Labioscrotal folds: prominence, rugation, pigmentation, and presence of
gonads
• Clitorophallic structure: dorsal stretched length, glans width, and corporal
consistency
• Perineal orifices: number, location, and patency
• Gonads: presence by palpation, location, and consistency
• Palpable gonadal tissue suggests presence of at least some ipsilateral
testicular tissue

IMAGING AND SPECIALIZED EXAMINATION
■ Modalities are employed selectively based upon clinical findings and the
active differential diagnosis.
■ Ultrasonography: Evaluation of the adrenals, kidneys, ureters, bladder,
Müllerian structures, and gonads
■ Dilation of genital ducts or duplications or unclear anatomy may lead to
additional radiographic or operative investigations.
■ Genitography: Delineation of internal genitourinary anatomy
■ A Foley catheter or occlusive nipple is used to occlude the UG sinus orifice.
■ Retrograde administration of contrast material defines the proximal anatomy
including sinus length and level of confluence.
■ The vaginal confluence may be missed if the system is not well distended.
■ Voiding cystourethrography (VCUG)
■ The catheter may be advanced for VCUG after genitogram if indicated or if
the sinus is not opacified. Vesicoureteral reflux and bladder anatomy is
assessed during filling, at bladder capacity, during voiding, and after voiding
is complete.
■ VCUG alone may not opacify the vagina, so it is less useful study for
assessing the UG sinus.
■ Endoscopy: Direct visualization of patient anatomy
■ Because general anesthesia is required, endoscopy may be performed at the
time of definitive reconstruction unless additional anatomic evaluation is
required for surgical decision-making.
■ MRI: Delineation of pelvic anatomy and associated spinal anomalies
■ MRI often requires anesthesia in young children and would therefore be
considered when other imaging studies are inconclusive.
■ Diagnostic laparoscopy: very selectively used in infancy if additional
information required
■ Laparoscopy is utilized to clarify internal anatomy and gonadal
characteristics.
■ Gonadal biopsy may be necessary in select cases.
DIFFERENTIAL DIAGNOSIS
This list includes only conditions in which patients could potentially be
candidates for feminizing procedures.
■ Differences in gonadal differentiation

■ Pure gonadal dysgenesis
■ Mixed gonadal dysgenesis
■ Ovotesticular DSD (previously “true hermaphroditism”)
■ Differences in androgenization
■ Adrenal sources
• Congenital adrenal hyperplasia (in 46 XX patients)
• 21-Hydroxylase deficiency
• 11β-Hydroxylase deficiency
• 3β-Hydroxysteroid dehydrogenase deficiency
• 17β-Hydroxysteroid dehydrogenase deficiency (late virilization in 46 XY
phenotypic female)
■ Androgen-secreting tumor
■ Maternal sources
• Drugs
• Androgen-secreting tumor
• Placental sources
■ Placental aromatase deficiency
■ Androgen receptor dysfunction
• Variations in activity of androgen receptor in various tissues (46 XY
patients)
• Complete androgen insensitivity syndrome (external genitalia
phenotypically female, lacking Müllerian structures due to intact MIS)
• Partial androgen insensitivity (diverse external phenotype, degree of
virilization at puberty and innate gender identity not predictable)
■ Differences in embryonic development
■ Persistent cloaca
■ Exstrophy-epispadias and cloacal exstrophy
■ Müllerian anomalies
• Müllerian agenesis (Mayer-Rokitansky-Küster-Hauser syndrome)
• Vaginal atresia
• Fusion anomalies
• Vaginal duplication
• Uterine duplication
• Vaginal septum variations

• Urogenital sinus anomalies
• High vaginal confluence
• Low vaginal confluence
NONOPERATIVE MANAGEMENT
■ Create a multidisciplinary team incorporating experienced pediatric
urologists/pediatric surgeons/pediatric gynecologists, endocrinologists,
psychiatrists/psychologists, geneticists, and nurses/social support members to
work with the parents and child to guide patient care. Consider formal ethics
consultations when appropriate.
■ There is a broad spectrum of “normal” female external genitalia. Referring
providers, parents, and patients may be reassured by education on range of
“normal.” Appearance and future functionality are important to consider in
the pediatric patient if surgery is requested.
■ The timing and indications for feminizing genital reconstruction are currently
controversial, and there may be long-term consequences of both action and
inaction in this arena.
■ Performing feminizing surgery in prepubertal children precludes autonomous
self-determination in surgical decision-making and may lead to regret.
■ Consider the psychosocial consequences of genital ambiguity, and provide
appropriate counseling for patient and parent.
■ Patients deserve long-term psychological support, with or without surgical
intervention.
■ Extended support networks may be found both within the hospital and in the
patient’s own community.
■ Discuss patient reproductive potential, optimizing patient sexual development
and fertility when possible. Administration of androgens or estrogens may be
recommended in longer term, depending on the underlying anatomy and
function.
■ Consider the potential for gonadal malignancy, which varies significantly with
gonadal histology and patient pathophysiology. Whenever possible, gonadal
preservation should be considered.
SURGICAL MANAGEMENT
Preoperative Planning
■ Diagnostic laboratory evaluation may include karyotype, serum electrolytes,

17-hydroxyprogesterone, testosterone, luteinizing hormone, and follicle-
stimulating hormone levels. Additional adrenal and sex hormone testing is used
more selectively but is essential to make a diagnosis in some cases.
■ A thorough examination under anesthesia should be completed, including
inspection of external genitalia, palpation for gonads, cystoscopy, vaginoscopy
(if applicable), and genitography.
■ Consider the need for gonadal biopsy or gonadectomy at the time of
intervention, along with possible gonad cryopreservation.
■ Patients undergoing vaginoplasty require bowel preparation (enema vs
polyethylene glycol solution) prior to surgical intervention.
Positioning
■ Isolated genitoplasty with or without repair of a low UG sinus is generally
performed in frog-leg (infants, young children) or dorsal lithotomy (older
patients) position with padding of all pressure points.
■ For reconstruction of a high UG sinus, most patients will require both prone
and supine positioning, so total lower body preparation is utilized. This
preparation may be advantageous in infants and young children for sterility of
the field, whether high or low confluence.
■ Ensure there are no IVs in lower extremities.
■ A single-shot caudal block is reasonable in eligible patients, but a continuous
caudal catheter is not utilized intraoperatively.
■ After endoscopy and catheter placement, keep the catheters sterile and move
the patient up the table for second prep. Prep the lower body circumferentially
from the xiphoid to the toes.
■ Place feet and legs in a stockinette.
■ Drape to allow for lower body repositioning from supine to prone within the
drape (FIG 1).
■ Position the patient with stacked towels under the pelvis. Initial prone vs
supine positioning will depend upon the planned procedures.
Approach
■ Primary components of the feminizing genitoplasty include the following:
■ Clitoral degloving
■ Elevation of the urethral plate and mobilization of the common UG sinus
■ Clitoral reduction
■ Urogenital sinus reconstruction and vaginoplasty