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10 Surgical Management ofMale Classic Bladder Exstrophy: TheToronto Approach
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187
Modified Perineal Approach
intheManagement ofFemale
11
Epispadias
JoaoLuizPippiSalle, RodrigoL.P.Romao,
andMichaelChua
Introduction
Isolated female epispadias (IFE) is a rare congenital anomaly, considered the mildest form of the female exstrophy/epispadias complex, with an occurrence of 1in
480,000 newborn girls [1]. Factors such as Caucasian race, advanced parental age,
and in-vitro fertilization have been associated with it [9, 16].
Inheritance patterns in familial cases suggest a complex genetic trait, occurring
in less than 1% of cases [9, 16]. The prior study identied three severity levels of
IFE: mild (bid clitoris and patulous meatus) (Fig.11.1a, b), intermediate (dorsal
urethral split, patulous and opened bladder neck) (Fig.11.2a, b), and severe (complete urethral cleft involving the sphincter, wide diastasis and opened bladder neck,
leading to constant incontinence; prolapse in some cases simulating bladder exstrophy) [16] (Fig.11.3a, b). Treatment aims to achieve good cosmesis, preserve the
upper urinary tract, and ensure full urinary continence [5].
Supplementary Information The online version contains supplementary material available at
https://doi.org/10.1007/978- 3- 031- 91238- 2_11.
J. L. P. Salle
Division of Urology, SickKids Hospital and Co-Director of the Center for Pelvic
Reconstruction, Toronto, Canada
e-mail: pippi.salle@sickkisd.ca
R. L. P. Romao (
Division of Pediatric Urology, Division of Pediatric General and Thoracic Surgery, Hospital
for Sick Children, University of Toronto, Toronto, ON, Canada
e-mail: rodrigo.romao@sickkids.ca
M. Chua
Division of Pediatric Urology, Hospital for Sick Children, University of Toronto,
Toronto, ON, Canada
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2025
A. R. Shukla, R. S. Joshi (eds.), Bladder Exstrophy and Epispadias,
https://doi.org/10.1007/978-3-031-91238-2_11
*)
189
190
Fig. 11.1 The mucosal
strip between the two
hemi-clitoris is marked to
be further incised
Fig. 11.2 Dissection of
the mucosal strip which is
used as a handle to retract
and expose the BN
J. L. P. Salle et al.
Typically, the upper genitourinary tract in IFE patients is normal. Common associated anomalies include a bid clitoris, an open and patulous urethra, and pubic
diastasis; some cases also present with vesicoureteral reux (VUR). The defect
extends to the bladder neck, which is usually incompetent, leading to urinary
11 Modied Perineal Approach intheManagement ofFemale Epispadias
Fig. 11.3 Incision of the
intersymphyseal bands to
optimize exposure of the
BN and anterior
bladder wall
191
incontinence [4]. Diagnosis is generally straightforward, but some cases may be
subtle, despite a noticeable bid clitoris. Misdiagnosis with ambiguous genitalia
can occur, and some girls present later with persistent urinary incontinence [1].
Background
The traditional treatment involves urethral and genital reconstruction within the rst
year, followed by bladder neck reconstruction Young-Dees-Leadbetter cervicoplasty (YDL) when the child reaches the age of social continence [14]. In 2009,
Manzoni and Ransley proposed a single-stage technique for repairing the urethra,
bladder neck, and clitoris through a perineal approach, without tailoring the bladder
neck to enhance outlet resistance [12]. This technique was later modied to include
bladder neck tailoring (BNT) via the perineal approach, with studies conrming its
feasibility and favorable outcomes [3, 5, 11]. Benets of this method include earlier
surgery, improved continence, reduced morbidity, and potentially fewer additional
surgeries.
Modified Perineal Approach Surgical Technique
All patients receive prophylactic antibiotics prior to the procedure. Initially, a cystoscopy is conducted to evaluate the bladder neck and ureteric orices. The procedure begins with the placement of ne holding stitches into the prepuce of each
hemi-clitoris to retract and expose the mucosal strip between them. The mucosal
strip between both hemi-clitorises is marked, incised, and dissected from the pubic
attachments (Fig.11.1). Once free, this strip serves as a handle for retracting and
exposing the anterior bladder wall (Fig.11.2). It will eventually be excised along
192
J. L. P. Salle et al.
with the approximation of the edges of the hemi-clitoris during the nal steps of the
genitoplasty. The symphyseal pubic band is exposed and fully incised to enhance
the visualization of the bladder neck (BN) and anterior bladder wall (Fig.11.3). A
retro-pubic dissection extending approximately 5cm is performed to expose the BN
and anterior bladder wall. The mucosal strip is further divided in the midline all the
way through the BN and extended to approximately 5cm into the anterior bladder
wall (Fig.11.4). Two longitudinal incisions, 1.5cm apart, are made in the urethral
plate (Fig.11.5), which is then tubularized over an 8-Fr catheter with interrupted
absorbable sutures (after demucolization of its lateral excess) (Fig. 11.6). The
demucolized urethral plate is wrapped around the neourethra to reinforce it and to
prevent stulas (Fig.11.7). The anterior bladder wall and BN are incised, tailored,
and sutured to create a funneling conguration. Similarly, the lateral excess of the
BN and anterior bladder wall are de-epithelialized, separated from the pubic
Fig. 11.4 The mucosal
strip is further divided in
the midline all the way
through the BN and
extended to approximately
5cm into the anterior
bladder wall, allowing
wide exposure of the
bladder neck and the
lateral walls of the bladder
which will be funneled
11 Modied Perineal Approach intheManagement ofFemale Epispadias
Fig. 11.5 The anterior
bladder wall and neck
retracted, providing
excellent exposure
193
attachments, and their muscle is wrapped around to reinforce the funneled bladder
outlet. The symphyseal pubic bands are approximated, and the suprapubic area is
closed in layers with absorbable sutures. Genitoplasty and monsplasty are carried
out by lateral mobilization of the suprapubic fat, allowing medial rotation of the
diverted labia majora and reconstructing the mons pubis. This mobilization allows
both hemi-clitorises to be brought together at the midline, as we previously described
[6]. In most cases, a Foley catheter is left in place for 7days, but in some cases, a
suprapubic tube is left for 2–3weeks when it is clamped and urination is observed.
If urination is adequate, it can be removed [15].
The following link depicts the surgical technique and important technical details
to perform this procedure:
https://www.dropbox.com/scl/fi/6jigdqamlmn51llj5m3cl/PerinealUrethrocervicoplasty- for- Female- Epispadias- with- audio- and- credits.mp4?rlkey=k
mwvtcj07ylk7r5wf49aghz59&st=ubgrxi3t&dl=0
194
Fig. 11.6 Marking of the
mucosa of the urethra
(1.5cm apart), BN, and
lateral walls of the bladder
which will be funneled and
tailored
J. L. P. Salle et al.
Fig. 11.7 The lateral
aspects of the funneled
bladder, BN, and urethra
are demucosalized. The
bladder and urethra are
closed with interrupted
stitches
11 Modied Perineal Approach intheManagement ofFemale Epispadias
195
Institutional Data onModified Perineal Technique
A total of nine cases of IFE underwent a modied perineal cervicoplasty and were
included in our initial study and compared to three patients historically treated with
the classic YDL procedure. Seven of the nine patients (77.8%) were diagnosed at
birth, while two were identied later during investigations for refractory urinary
incontinence, including one teenage girl. All cases presented with bid clitoris on
the initial examination, and pubic diastasis was noted in every patient. Associated
anomalies included three cases with vesicoureteral reux (VUR), one case of spina
bida occulta, and one anorectal malformation. The mean age at the time of surgery
was 4.3years (range: 1–17years).
No bladder augmentations or osteotomies were performed in this group. In contrast, all patients who underwent YDL needed augmentation cystoplasty. Ureteric
reimplantation was also not performed in the group undergoing perineal cervicoplasty who had an uneventful postoperative course. The mean follow-up duration
was 6years (range: 1–10years).
Outcomes for urinary continence revealed that four out of seven children (57%),
who were old enough for evaluation, achieved continence following the initial procedure. Three incontinent patients received postoperative injections of dextranomer/hyaluronic acid as a bulking agent (Deux, Q-Med AB, Sweden), resulting in
continence for two of them, while one continued to experience stress incontinence.
Interestingly, two of these three patients were initially dry but later developed progressive stress incontinence, requiring endoscopic injection of bulking material at
the BN to improve outlet resistance [10].
Most patients (7 out of 9) demonstrated volitional voiding, though two were still
too young for toilet training assessment but showed signs of cycling their bladders
with dry intervals between diaper changes. Subjectively, the cosmetic results were
deemed satisfactory from both the surgeon’s and parents’ perspectives. Sexual function could not be assessed in these patients.
Preliminary ndings suggest that the perineal approach is effective for all IFE
cases and compares favorably with the traditional YDL technique, supporting previous reports [5]. Our approach, with modied BNT and reinforcement of the bladder
neck, achieved continence with voluntary voiding in most patients, eliminating the
need for bladder augmentation and ureteral reimplantation [2]. For the perineal
approach, complete incision of the inter-symphyseal pubic bands is crucial to facilitate tapering of the anterior bladder wall and bladder neck reinforcement (BNR).
Unlike the YDL technique, which can be overly retentive, the modied approach
creates a funnel-shaped BN, allowing voluntary voiding. None of the patients
required construction of a catheterizable channel. BN reinforcement with bladder
tissue, after removing mucosa and urethral, likely enhances outlet resistance,
improving continence as demonstrated in this series. Notably, two patients experienced incontinence recurrence years later, highlighting the need for long-term follow-up. Both were successfully treated with BN injections.
One case was diagnosed late in puberty after years of attending voiding dysfunction clinics and unsuccessful pharmacological treatments. This underscores the
196
J. L. P. Salle et al.
importance of genital and back examinations in incontinent patients to avoid prolonged suffering and social consequences. Most cases in this series were diagnosed
at birth, which could improve psychosocial outcomes and allow earlier treatment.
Achieving adequate cosmesis is important for girls with epispadias and exstrophy. Unfortunately, many women with prior repairs have unsatisfactory genital
appearance, persistent bid clitoris, absent mons, and severe midline scarring. The
present technique aimed to improve cosmesis through effective monsplasty and clitoroplasty, receiving positive feedback from parents and the medical team.
Discussion
VUR affects 30–75% of IFE cases [7, 16]. In this series, ureteric reimplantation was
not performed during the perineal approach, and no complications related to VUR
were observed at long-term follow-up. Patients did not develop recurrent febrile
UTIs and were not routinely given antibiotic prophylaxis. If reimplantation is
needed, the perineal approach may need to be reconsidered due to inadequate exposure for such a procedure [3].
Bladder capacity in IFE patients is typically low, likely due to poor outlet resistance [11]. Thus, early modied BNT is recommended to increase outlet resistance,
allowing the bladder to develop capacity and reducing the need for future augmentation cystoplasty [4, 13, 16].
In this small series, initial YDL approach results were poor, necessitating signicant redo surgeries, including augmentation, to achieve dryness. This may be attributed to the retentive nature of the YDL technique and the surgeons’ experience
with it.
Due to the rarity of cases, data comparing perineal versus classical YDL procedures is limited. The only other study, by Cheikhelard etal., also found favorable
results for the perineal approach in achieving continence without the need for augmentation in most patients.
Interestingly, some incontinent IFE patients post-perineal approach could be rendered continent after BN injections. This suggests that injections into a reduced BN
can achieve adequate coaptation of its walls, unlike in non-operated patients with a
wide-open BN.Similar experiences have been reported by others [8].
Acknowledgement: We acknowledge the work of Dr. Elaine Reck Sangalli, who did the illustra-
tions for our chapter.
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11 Modied Perineal Approach intheManagement ofFemale Epispadias
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