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10 Surgical Management ofMale Classic Bladder Exstrophy: TheToronto Approach
Fig. 10.1 The markings
for future bladder neck
tailoring
177
Bilateral Ureteral Advancement Reimplantation
Bilateral ureteral reimplantation is then performed to correct vesicoureteral reux,
a common issue in bladder exstrophy patients. The ureters are dissected circumferentially, and a submucosal tunnel is created in a cephalad direction, as previously
described [7]. The anastomosis is performed with a ne, absorbable suture; one of
the stitches is anchored to the detrusor for support (Fig.10.2).
One must ensure that a feeding tube (3.5 or 5fr.) advances easily into the ureter
after the reimplantation is completed. In some infants, the stent does not advance to
the pelvis due to the presence of ureteral folds, which kink it and cause obstruction.
It is essential to have good drainage; therefore, we obtain a retrograde pyelogram to
ensure adequate stent position in the renal pelvis. The detrusor and mucosa are reapproximated at the original orice site. Ureteral stents are inserted and externalized
through the lateral bladder wall. They are usually secured in place with a pursestring suture to the bladder wall.
Bladder Neck Tailoring andRepair andBladder Closure
Following BUR, attention is turned to the bladder neck. The goal is to create a funneled continence mechanism that effectively empties urine, aiming to increase
resistance without obstruction. By doing so, there will be an increase in intra-vesical
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J. L. P. Salle et al.
a
b
c
Fig. 10.2 Bilateral ureteral reimplantation at the time of primary exstrophy closure. (a) Ureters
mobilized for reimplantation. (b) Submucosal tunnel developed in a cephalad orientation. (c)
Reimplanted ureters improving exposure for tailoring and closing the bladder neck
pressure that stimulates bladder growth. This effect is achieved through meticulous
reconstruction of the bladder neck, tailoring it following the striations often seen at
the future outlet (Figs.10.3 and 10.4).
The bladder neck is approximated using a long-standing absorbable suture with
interrupted sutures over an 8fr urethral catheter. The sutures are laid in without tying
to allow good visualization (Fig.10.5). Each stitch is placed to carefully include a
good muscle bite with a small amount of mucosa for apposition. The detrusor overlying lateral areas denuded of the mucosa is released from its attachments and used
10 Surgical Management ofMale Classic Bladder Exstrophy: TheToronto Approach
Fig. 10.3 Panoramic and zoomed-in view of the bladder neck striations and verumontanum prior
to bladder neck tailoring
Fig. 10.4 Bladder neck
tailoring has been marked
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J. L. P. Salle et al.
Fig. 10.5 Diagram and intraoperative picture of the bladder neck repair
to cover and reinforce the tailored bladder neck as a second layer (Fig.10.6 and
Video 10.2).
After a suprapubic catheter is inserted, the bladder is closed with interrupted
absorbable sutures (Fig.10.7). During the rst surgery, a neo-umbilicoplasty is
also performed using a rhomboid skin ap. Bilateral inguinal hernia repairs are
routinely performed in boys by undermining the subcutaneous tissue of the original exstrophy incision.
Pelvic Osteotomy
Pelvic osteotomies are performed routinely to bring the pubic bones together in the
midline, allowing for deep placement of the bladder in the pelvis and tension-free
closure of the abdominal wall. We prefer to utilize bilateral anterior oblique osteotomies as originally described by Salter and modied by Wedge [23]. Infants are kept
on modied Bryant’s (gallows) traction for 2–3weeks postoperatively to allow for
the osteotomies to heal (Fig.10.8).
10 Surgical Management ofMale Classic Bladder Exstrophy: TheToronto Approach
Fig. 10.6 Diagram of the
reconstructed bladder neck
in two layers and closed
bladder
181
Fig. 10.7 Final aspect of
the closed bladder and
repaired bladder neck with
catheters in place
182
Fig. 10.8 Modied
Bryant’s traction in a
5-month-old child
following classic exstrophy
closure
J. L. P. Salle et al.
Second Stage Repair: Epispadias Repair
andPenile Reconstruction
Preoperative Testosterone Administration
Epispadias and penile reconstruction take place after 9–12months. Intramuscular
injection of testosterone, 2mg/kg per dose, is administered at 90, 60, and 30days
prior to the epispadias repair.
Epispadias Repair
The repair of epispadias is aimed at correcting the urethral opening and dorsal
chordee to achieve a more typical urethral meatus location and a straight penis
following bladder closure. The penis is carefully degloved, and initial dissection
of the urethra is performed ventrally, separating it entirely from both corpora
and allowing it to achieve a ventral position. Both corpora should be dissected
until entirely free from the urethra, allowing for natural external rotation when
the penis is pulled in a downward direction [35] (Video 10.3). After external
rotation, a double layer of interrupted non-absorbable sutures is used to approximate and keep the externally rotated corpora in the midline, both dorsally and
ventrally.
The urethroplasty is completed with interrupted absorbable sutures, intended to
avoid an accordion effect and allow tissue stretching for the neourethra repair. In
most cases, the urethra will reach the tip of the glans or at least a subcoronal location. In some situations where the urethra is too short, it may be necessary to leave
it as a hypospadias, which will require further reconstruction. We do not excise the
ventral preputial skin when this is anticipated, leaving it available for future staged
hypospadias repair.
Penile Skin Reconstruction
Penile skin reconstruction is important, both from functional and cosmetic standpoints. We favor the use of a rotational penile skin ap from the ventral aspect of the
penis; this usually provides adequate coverage of the penile shaft (Fig.10.9). It
avoids a dorsal midline scar and ensures a natural appearance with a well-dened
10 Surgical Management ofMale Classic Bladder Exstrophy: TheToronto Approach
a
183
b
Fig. 10.9 Skin closure after epispadias repair. (a) Diagram of the ventral rotational skin ap. (b)
Harvesting of the skin ap. (c) Final aspect of the repair. Note absence of dorsal midline scar
c
penopubic angle [26]. However, as emphasized above, if the urethra is left as a hypospadias, the ventral prepuce can be left intact for a future staged urethroplasty.
Girls born with exstrophy follow the same principles described for boys except
for the genitalia, requiring a feminizing genitoplasty (clitoroplasty—usually
approximation of both hemi-clitoris); introitoplasty, Y-V vaginoplasty and monsplasty as described by Pippi Salle [11].
Further Stages: Continence Procedures andFinal Touches
Continence Enhancement
Additional procedures may be considered for patients who do not achieve urinary
continence following the bladder neck repair. In cases where the bladder neck is not
wide open, it is reasonable to attempt its coaptation using bulking materials. Such
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J. L. P. Salle et al.
attempts are more successful when a reasonable bladder capacity is present. We
always give the bladder time to develop capacity and, ultimately, continence/dryness with or without intermittent catheterization. Bladder augmentation and neck
closure are the last resources that, unfortunately, will be necessary in a proportion
of patients [37].
Cosmetic andFunctional Refinements
The nal stage may also involve cosmetic and functional renements to the genitalia and bladder, tailored to the individual patient’s outcomes from previous surgeries. It is important to achieve an adequate cosmetic appearance of the external
genitalia. In many surveys, females resent having a scarred suprapubic area and
an abnormal labial appearance [5]. They also care about having a reconstructed
umbilicus and a normal mons venus, elements often neglected in the past. This
stage is critical for addressing any residual issues and enhancing overall quality of
life [11].
Single Institution Experience withtheToronto Approach
Aware of the increased bladder capacities achieved when using the CPRE, we
decided to make the modications described above, aiming to safely maintain these
advantages. In 2019, a comparative study was performed at The Hospital for Sick
Children (SickKids), aimed at evaluating the outcomes of a single (CPRE) versus a
staged (Toronto approach) repair of bladder exstrophy [10]. It is important to note
that all patients undergoing CPRE in this series also underwent BUR and bladder
neck tailoring, similar to what is performed in the Toronto approach staged repair.
A total of 21 patients were analyzed, with 10 undergoing CPRE-BUR and 11 undergoing the Toronto approach.
In terms of intra-operative complications, the CPRE-BUR group experienced
two cases (20%) of penile ischemic injury, one of which led to signicant tissue
loss. In addition, all patients remained with hypospadias, requiring staged oral
mucosa urethroplasty later. Penile ischemia was not observed in the Toronto
approach group, highlighting its safety prole in preserving penile integrity during
surgery. The two groups had similar complications, such as stulae, strictures,
stones, and UTIs.
The study also reported on long-term surgical outcomes and the need for additional procedures. For the CPRE group, 7 out of 10 patients (70%) required further
surgical interventions, whereas in the staged Toronto approach group, 6 out of 11
patients (54.5%) underwent additional surgeries. This data suggests a slightly lower,
although not statistically signicant, rate of additional surgeries in the staged repair
group compared to the CPRE group.
The subjective assessment indicated better penile length and cosmetic outcomes
in the Toronto approach group, suggesting an advantage in postoperative
10 Surgical Management ofMale Classic Bladder Exstrophy: TheToronto Approach
185
appearance and patient satisfaction. This nding aligns with the surgical aim to
address functional issues and consider the cosmetic outcomes important for the
patient’s psychological well-being.
In summary, the study presents the Toronto approach as a safer alternative to
CPRE-BUR, especially in minimizing the risk of severe intra-operative complications like penile ischemic injury. While long-term outcomes regarding the
urinary system’s functionality and health appeared comparable between the
two groups, the Toronto approach demonstrated potentially better cosmetic
results and a slightly reduced need for additional surgeries. Figure10.5 illustrates the long-term outcomes of genitalia operated with the Toronto approach
(Fig.10.5).
Finally, it is noteworthy that the 1st stage of the Toronto approach had a signicantly shorter duration compared to CPRE (median 580 vs. 367 minutes). The
authors postulate that this impacts the surgeon’s stamina and accuracy when two
advanced reconstructive procedures are performed back-to-back (bladder closure
and epispadias repair) in the case of CPRE.When the duration of both procedures
was added up in patients undergoing the Toronto approach, there was no substantial
difference compared to CPRE.However, admittedly, patients undergo two general
anesthetics from the outset [10].
Addressing Challenges andLimitations
While the Toronto approach represents a signicant advancement in the surgical
management of bladder exstrophy, it is not devoid of challenges. The staged repair
introduces the potential for increased scarring at the proximal urethra, which may
create additional difculties during the epispadias surgery. However, as the bladder
neck was previously addressed, most cases do not require much dissection in
that area.
Conclusion
The surgical management of male bladder exstrophy via the Toronto approach
is characterized by a nuanced and staged application of various surgical techniques, each meticulously designed to address the distinct phases of treatment
required for this complex condition. The approach leverages a deep understanding of bladder exstrophy’s anatomical and functional aspects, aiming to restore
normal anatomy, preserve renal function, achieve urinary continence, and
improve cosmetic outcomes. It is paramount to maintain strict follow-up protocols for patients treated for bladder exstrophy regardless of the technique used
for primary closure. Aspects like lower and upper urinary tract function, cosmesis, transition into adulthood, sexuality, and fertility need to be taken into
account for the life continuum of children born with this challenging congenital
malformation.
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J. L. P. Salle et al.
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