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166
ab
cd
S. N. Kureel et al.
If bladder capacity permits, ureters are mobilized and reimplanted either in a
submucosal or extramucosal fashion [14, 15].
In preparation for anatomical bladder neck reconstruction without double breasting, three or four 4-0 polygalactin sutures are placed across the urethral plate over
an 8 or 10 French feeding tube, incorporating the denuded detrusor at the bladder
neck and tying it off (Fig.9.31b, c) [16]. The bladder is closed with tubes for urethral drainage, bladder drainage, and bilateral ureteral drainage (Fig. 9.31d).
Glansplasty is done by maintaining coronal alignment and fashioning a slit-like
meatus after urethral tubularization (Fig.9.32a).
The open limbs of the rhabdosphincter on both sides are anchored to the tubularized urethra, and the corpora are approximated in the midline dorsally (Fig.9.32b, c).
Three sutures of polygalactin number 1-0 or number 1 are now placed across the
cartilage of the pubic bone (Fig.9.32d). A retropubic drain is placed, and the pubic
bones and recti are approximated in the midline (Fig.9.33a). The umbilicoplasty
ap is tubularized, and the lowermost abdominal crease is approximated (Fig.9.33b).
The skin cover is completed by dorsal midline approximation of the bifurcated
penile skin dartos and distal suturing to the dorsal subcoronal collar (Fig.9.34a). A
penoscrotal angle is created (Fig.9.34b). Reverse advancement of the inner preputial skin provides cover to the ventral surface as shown in Fig.9.34c. Lateral edges
of inner preputial aps and outer preputial skin strips are sutured (Fig.9.34d). The
scrotal median raphe is re-approximated (Fig.9.35).
Fig. 9.32 (a) Glansplasty maintaining corporal alignment. (b) Open limbs of rhabdosphincter on
both sides anchored to tubularized urethra. (c) Corpora approximated dorsal to tubularized urethra.
(d) Polygalactin sutures preplaced across pubic bones
ab
cd
9 Scrotoperineal Approach toBladder Exstrophy Repair
167
Fig. 9.33 (a) Pubic bones
and recti approximated in
the midline, (b)
Umbilicoplasty ap
tubularized and lower
abdominal skin crease is
aligned
a
b
Fig. 9.34 (a) Bifurcated penile skin-dartos aps approximated in dorsal midline providing dorsal
cover to penile shaft, (b) Penoscrotal angle is created on ventral side, (c) Reverse advancement of
inner preputial skin to provide ventral skin cover, (d) Joining the edges of dorsal and ventral aps
completes the penile skin cover
168
Fig. 9.35 Abdominal skin
and scrotal skin
reapproximated in midline
S. N. Kureel et al.
Postoperative Management
Postoperative analgesia is maintained by bupivacaine via epidural catheter as and
when needed.
Ureteric catheters are removed by 12–14days; suprapubic catheter is removed by
day 18–19; and 48hours after that, the urethral catheter is removed. Broad-spectrum
injectable antibiotics are used for 10days. After removal of the urethral catheter, spontaneous voiding occurs. In rare cases, the patient may need a sitz bath for 1–2days.
Postoperative penopubic stula occurs in about 50% of cases but usually heals
spontaneously except for 1–2% who need repair after 6 months.
Follow-Up
Follow-up evaluation at 3months, 6months, and then yearly includes ultrasound for
upper tract dilatation or thickening of the bladder and post-void residual urine volume. Yearly evaluation of serum creatinine and GFR is done and compared with
preoperative baseline values. Continent patients who present with increased frequency are evaluated for urinary tract infection (UTI) with urine microscopy and
culture and treated accordingly. An urodynamic study is done for the evaluation of
bladder pressure. Based on the clinical status and urodynamic ndings, oxybutynin
and imipramine may be used to improve continence and preserve the upper tract by
relaxing the bladder. Chemoprophylaxis is prescribed for those in whom antireux
surgery could not be done due to bladder plate condition.
Results
From 2004 to 2024, 241 cases of exstrophy-epispadias complex, ranging in age
from 6weeks to 24years, were operated on. These included 173 male and 68 female
patients. Out of these, 123 male patients with classical exstrophy and a good bladder
9 Scrotoperineal Approach toBladder Exstrophy Repair
169
plate underwent single-stage total reconstruction using a midline scrotoperineal
approach with radical corporal detachment, innervation-preserving sphincteroplasty, and bladder neck repair. Ureteric reimplantation was done whenever the
supratrigonal area was not studded with multiple polyps. Forty cases of incontinent
epispadias with good bladder capacity on cystogram (>60ml) were also managed
with the same protocol.
Ninety patients with a trapezoid-shaped urogenital diaphragm on 3-D CT pelvis
received ischiopubic osteotomy, while 33 patients in whom the urogenital diaphragm was rectangular or square with a very wide gap between the pubic bones
received transverse innominate osteotomy.
The outcome parameters assessed were:
1. Intraoperative complications
2. Immediate postoperative complications
3. Late complications
4. Urinary continence at 5-year follow-up
5. Upper tract status
6. Cosmetic outcome
1. Intraoperative Complications
The transverse perinei muscle was found to be absent in seven patients. In one
of these patients, minor invasion of the anterior wall of the rectum occurred,
which was identied and repaired without subsequent complications.
After approximation of pubic bone, compression of the corpora cavernosa
was noted in three patients, which was identied intraoperatively, and the lowermost approximation suture of pubic bone was released to relieve the
compression.
After corporal-urethral separation, the urethral plate was usually adequate to
take the meatus to the tip; however, in four patients, an unstretchable urethral
plate mandated its division and the creation of a hypospadiac meatus.
2. Immediate Postoperative Complications
In two patients, reexploration for intestinal obstruction was done around the
7th postoperative day. Postoperative ileoileal intussusception was the culprit,
which was reduced, and the further course was uneventful.
Two patients developed epididymo-orchitis, seven had supercial wound
dehiscence, and penopubic stula developed in 35 patients. All cases healed with
conservative management, without the need for intervention.
Three patients developed a ventral hernia secondary to the disruption of the
midline-approximated linea alba; a secondary suturing without using a mesh
was undertaken after 6months. The subsequent appearance of the abdominal
wall was normal with no recurrence.
3. Late Complications
Recurrent UTI was noted in 40 patients in whom ureteric reimplantation was
not done. After eradication of infection, a micturating cystourethrogram (MCU)
revealed bilateral grade 2–3 reux in all 40 patients. In one of these patients, left
renal function as assessed on nuclear scan was reduced, necessitating ureteric
reimplantation.
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S. N. Kureel et al.
Mild hydroureteronephrosis was noted in 30 patients on follow-up ultra-
sound. But it was stable, and renal function did not deteriorate. These patients
were started on oxybutynin.
There was no case of glans loss or penile loss.
4. Urinary Continence
In 130 patients who have completed at least 5years of follow-up after the
repair, continence was evaluated with a voiding diary, dry interval at nighttime, dry interval at daytime, frequency of voiding, capacity to hold midstream micturition, uroflow, and video recordings of micturition captured
on mobile by parents or patients themselves. Out of 130 patients operated
on for bladder exstrophy and incontinent epispadias, 96 are continent with
a dry interval of more than 2hours in the daytime and 5hours at night.
Midstream holding and reinitiation of micturition could be demonstrated in
70 patients. Stress incontinence was seen in 60 patients and managed with
medication.
5. Bladder Capacity
Bladder capacity as assessed on ultrasound ranged from 80 to 110 ml in
patients with dry intervals of more than 2hours. Bladder capacity measured on
MCU (done after eradication of infection in those patients who had recurrent
UTI) ranged from 60 to 70ml.
6. Upper Tracts
Upper tracts were evaluated with follow-up ultrasound for hydronephro-
sis, bladder capacity, bladder wall thickness, and post-void residual urine
volume at 3months, 6months, and yearly. A radionuclear scan was obtained
yearly and compared with the preoperative baseline. Out of 163, unilateral
deterioration of the upper tract was noted in one patient due to vesicoureteric reflux. There was no deterioration in GFR in the other 162 patients.
The appearance of new cortical scarring was noted in six of the patients
having vesicoureteric reflux, and modification of chemoprophylaxis
was needed.
7. Cosmetic Outcome
The aesthetic score was determined on a scale of 0–10, where a normal
appearance of the lower abdominal wall and genitalia was scored 10 and boys
with unoperated classic bladder exstrophy scored zero. Aesthetic scores ranged
from 7 to 8in 145 patients, while the parents of 18 patients were unsatised with
the small size of the phallus.
Conclusion
Using the scrotoperineal approach, bladder exstrophy reconstruction can be
done in a manner that preserves all the vascularity and innervation, with the
creation of a dynamic outlet resistance and low-pressure bladder reservoir.
Satisfying aesthetic and functional outcomes can be seen in more than twothirds of patients.
9 Scrotoperineal Approach toBladder Exstrophy Repair
171
References
1. Brock J III, O’Neill J Jr. Bladder exstrophy. In: O’Neill J, editor. Pediatric surgery. Philadelphia:
WB Saunders; 1998. p.1709–32.
2. Trendelenberg F.The treatment of ectopia vesicae. Ann Surg. 1906;44:981–9.
3. Gearhart JP, Forschner DC, Jeffs RD, etal. A combined vertical and horizontal pelvic osteotomy approach for primary and secondary repair of bladder exstrophy. J Urol. 1996;155:689–93.
4. Tomaszewski R, Pethe K, Koszutski T.Salter pelvic osteotomy in operative treatment in
patients with bladder extrophy. Chir Narzadow Ruchu Ortop Pol. 2010;75:126–8.
5. Sunil K, Kureel SN, Gupta A, etal. IschioPubic osteotomy, a simple and effective technique
for pelvic ring closure in repair of classic exstrophy bladder. J Indian Assoc Pediatr Surg.
2018;23(1):27–31.
6. Kureel SN, Gupta A, Kumar S, etal. A novel midline scroto-perineal approach facilitating
innervation preserving sphincteroplasty and radical corporal detachment for reconstruction of
exstrophy-epispadias. Urology. 2011;78(3):668–74.
7. Kureel SN, Gupta A, Singh CS, etal. A novel skin management scheme in surgery of epispadias undergoing Cantwell-Ransley repair: a technique to improve the aesthetics and minimize
complications. Urology. 2013;82(6):1400–4.
8. Kureel SN, Rashid KA, Rawat J.Tubularized trapezoid ap neoumbilicoplasty—simple technique for umbilical reconstruction in bladder exstrophy. Urology. 2009;73(1):70–3.
9. Sponseller PD, Bisson LJ, Gearhart JP, etal. The anatomy of the pelvis in the exstrophy complex. J Bone Joint Surg Am. 1995;77:177.
10. Kanoujia Sunil, etal. Study of variations in conguration of bony pelvis and magnitude of
pubic diastasis in exstrophy-epispadias in relation to type of anomaly in spectrum of exstrophy-epispadias complex- a prospective study– thesis submitted and accepted in 2017 for MCh
Pediatric Surgery, King George’s Medical University, Lucknow.
11. Stec AA, Pannu HK, Tadros YE, etal. Evaluation of the bony pelvis in classic bladder exstrophy using 3 D CT—further insights. Urology. 2001;58:1030.
12. Kureel SN, Gupta A, Gupta RK.Surgical anatomy of urogenital diaphragm and course of its
vessels in exstrophy-epispadias. Urology. 2011;78(1):159–63.
13. Kureel SN, Gupta A, Singh CS, etal. Surgical anatomy of penis in exstrophy-epispadias: a
study of arrangement of fascial planes and supercial vessels of surgical signicance. Urology.
2013;82(4):910–6.
14. Gupta A, Kureel SN, Wakhlu A, etal. Bladder exstrophy: comparison of anatomical bladder
neck repair with innervation preserving sphincteroplasty versus Young-Dees-Leadbetter bladder neck reconstruction. J Indian Assoc Pediatr Surg. 2013;18(2):69–73.
15. Gupta A, Kureel SN.Creation of effective antireux mechanism without creation of submucosal tunnel in surgical correction of vesicoureteric reux: myth or reality?-an experimental
study. J Indian Assoc Pediatr Surg. 2014;19(1):17–21.
16. Sunil K, Gupta A, Chaubey D, etal. Technique of antireux procedure without creating submucosal tunnel for surgical correction of vesicoureteric reux during bladder closure in exstrophy. J Indian Assoc Pediatr Surg. 2018;23(2):70–3.
Surgical Management ofMale Classic
Bladder Exstrophy: TheToronto
10
Approach
JoaoLuizPippiSalle, RodrigoL.P.Romao,
andMichaelChua
Introduction
Bladder exstrophy represents one of the most challenging congenital anomalies within pediatric urology, often requiring multiple, meticulous surgical
interventions and experience in order to achieve favorable long-term outcomes. Over the decades, the technique for the initial surgical closure of the
exstrophy bladder has evolved, and modifications have been introduced, leading to significant improvements in the management and long-term prognosis
of this complex condition.
The Toronto approach emerges as the accumulation of many advancements, aiming to address some of the shortcomings of previous surgical techniques and enhance
the quality of life for affected individuals.
Supplementary Information The online version contains supplementary material available at
https://doi.org/10.1007/978- 3- 031- 91238- 2_10.
J. L. P. Salle
Division of Urology, SickKids Hospital and Co-Director of the Center for Pelvic
Reconstruction, Toronto, Canada
e-mail: pippi.salle@sickkids.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
e-mail: michael.chua@sickkids.ca
© 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_10
*)
173
174
J. L. P. Salle et al.
Historical Context andEarly Attempts at Primary Closure
The journey toward the development of effective surgical management for bladder
exstrophy began with early attempts at primary closure. These initial endeavors
often resulted in poor outcomes, predominantly due to the high incidence of postoperative infections [24]. The complexity of the condition, coupled with limited surgical expertise and technology at the time, contributed to the challenging nature of
these early interventions.
Staged Repair andtheJeffs Proposal
Recognizing the limitations of primary closure, Dr. Robert D. Jeffs and Dr. J
Cendron proposed a staged repair technique. At the initial primary closure, the
approach was characterized by a deliberate decision not to attempt to create urethral
resistance, opting instead for an open bladder neck to avoid the potential deterioration of the upper urinary tract [8, 17].
Following this protocol, males underwent epispadias repair around the age of
one, followed by continence procedures as they reached school age, usually around
5 years. The treatment of epispadias involved internal rotation to correct dorsal
penile curvature and the application of Byars’ aps for skin closure, laying the
groundwork for subsequent interventions [25]. The patients remained incontinent
until school age, when bladder neck reconstruction, following principles described
by Young-Dees-Leadbetter, was performed [4]. They recognized early the importance of developing adequate bladder capacity after the initial primary closure and
epispadias repair in order to achieve success [2, 15, 35].
The Mitchell andGrady Proposal: CPRE
Advances in neonatal medical and surgical care stimulated Michael E.Mitchell
and Richard W.Grady to propose a simultaneous primary bladder closure and
epispadias repair early in life, a procedure they called Complete Primary Repair
of Exstrophy (CPRE) [13]. Despite its name, CPRE is almost never “complete,”
as it often results in hypospadias, which requires at least two additional surgical
steps for the urethroplasty [24, 27, 33]. Initially, only minimal attempts to repair
the bladder neck were made, a step later modied by the introduction of bladder
outlet tailoring in order to increase urethral resistance and enhance bladder
capacity [3, 14, 34].
This approach did, however, mirror the staged repair in terms of epispadias
treatment and addressing incontinence, if present. A signicant concern with
CPRE is the risk of penile ischemia due to compartment syndrome; although rare,
this is a catastrophic complication [9, 10, 21, 31]. Additionally, some patients
experienced upper tract deterioration, particularly those with some degree of
10 Surgical Management ofMale Classic Bladder Exstrophy: TheToronto Approach
urethral resistance and improved bladder capacity. This deterioration can be
accompanied by severe vesicoureteral reux (VUR), hydronephrosis, and symptomatic UTIs [12].
175
The Toronto Approach: AStaged, Comprehensive Strategy
Aiming forImproved Outcomes
The Toronto approach was developed as a modication of the staged repair and in
response to some of the issues observed with CPRE.
Bilateral ureteral reimplantation (BUR) is performed at the time of exstrophy
closure in a cephalad direction as previously described [7]. Tailoring/funneling of
the bladder neck, aimed at generating some degree of early outlet resistance without
signicant obstruction as observed in patients with the Young-Dees-Leadbetter procedure and increasing the chances of continence in the future, is also undertaken.
BUR signicantly eliminates or at least downgrades reux [30], reducing the
chances of severe febrile UTIs and protecting the upper urinary tract [16]. Epispadias
repair is carried out in a delayed fashion.
This approach prevents complications like penile ischemia seen with CPRE, protects the upper urinary tract, and potentially enhances bladder capacity [10].
Testosterone stimulation is administered before epispadias repair, which is performed later, around 9months to 1year of age. Epispadias repair is aimed at correcting the dorsal curvature with external corporal rotation, performing the
urethroplasty and penile skin closure using a rotational penile skin ap rather than
Byars’ aps, thereby avoiding midline dorsal scars, which can increase chances of
recurrent dorsal curvature [26].
Rationale andStrategic Pillars
This approach is predicated on the understanding that bladder exstrophy is not a
singular anomaly but a spectrum of disorders affecting the lower urinary tract, anterior abdominal wall, and pelvic bones. Reconstruction has a substantial impact on
both lower and upper urinary tract function. The Toronto approach is designed to
improve functional and cosmetic outcomes, offering a roadmap for reconstructing
the bladder and external genitalia while preserving renal function and achieving
urinary continence.
The rationale for early BUR is to provide room for gentle tailoring/funneling of
the bladder neck to generate some degree of outow resistance, following the natural striations observed in this area. Additionally, avoiding VUR reduces the risk of
signicant febrile UTIs [6, 16, 21]. Furthermore, urine stays in the bladder longer
rather than reuxing freely into the upper urinary tract [18]. This coupled with
increased outlet resistance potentially improves bladder capacity and continence in
the long run [19, 30].
176
J. L. P. Salle et al.
Technical Details andInnovations
Preoperative Assessment andPlanning
Preoperative assessment includes baseline imaging studies, such as ultrasound,
X-ray, and DMSA scans, to evaluate kidney, bladder, and pelvic bone anatomy. The
orthopedic surgery team reviews pelvic X-rays in preparation for pelvic osteotomies.
While the Toronto approach was initially described in the neonatal period, it
is currently performed electively, usually around 3–4months of life. Although
there are controversies about prolonged exposure of the bladder plate [1, 20, 22,
28, 36], many centers have switched to an elective surgical approach in recent
years based on the following factors: exstrophy closure is not an emergency; the
importance of bonding between family and baby allows breastfeeding to be
established; take advantage of the early testosterone boost; time to assemble an
experienced, consistent multidisciplinary team for surgical management; and
increased risk of surgical and anesthetic complications in the neonatal period
[29, 32].
Neonates with classic bladder exstrophy are admitted initially, and the bladder
plate is covered with a plastic lm (Saran Wrap). Prophylactic antibiotics are not
given. The baby is allowed to feed early and can usually be discharged after a period
of short observation and after the parents have been counseled about the care of the
bladder plate and understand the future plans.
Surgical Procedures
First Stage: Bladder Closure, Ureteral Reimplantation, Bladder
Neck Repair, Pelvic Osteotomy, Bilateral Inguinal Hernia Repair
andUmbilicoplasty
Surgical Intervention, Lateral Dissection, andApproach
tothePubovesical Bands
Initially, the bladder plate characteristics are measured, including the area and depth
of the defect, as well as the position of the ureteral orices and the length of the
urethral plate. The presence of polyps and bladder elasticity is documented. Large
polyps should be removed.
After carefully incising the mucocutaneous junction, dissection is carried
down laterally until the pre-vesical fat is identied on both sides. The dome is
then dissected off the peritoneum using sharp and blunt dissection. The ligaments/bands between the urethro-vesical complex and the pubic bones are
divided on both sides; this is preceded by proactive identication of the proximal
aspect of the neurovascular bundle on either side to avoid its injury (Video 10.1).
The future incisions for bladder neck tailoring are outlined with cautery (does
not fade away with the manipulation) before proceeding with the ureteral reimplantation (Fig.10.1).