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- •Foreword
- •Foreword
- •Preface
- •Contents
- •Contributors
- •Introduction
- •Epidemiology
- •Etymology
- •Bladder Exstrophy Pathophysiology
- •Conclusion
- •References
- •Normal Development
- •Introduction
- •Prenatal Imaging
- •Prenatal Counseling
- •Epispadias
- •Classic Bladder Exstrophy
- •Cloacal Exstrophy
- •BEEC Variants
- •Prenatal Management
- •Genetic Counseling
- •Conclusion
- •References
- •3: Bladder Exstrophy Genetics: Our Current Understanding
- •Bladder Exstrophy Genetics
- •Copy Number Variant (CNV) Studies
- •Gene Expression Studies
- •Genome-wide Association Study (GWAS)
- •Future Directions
- •References
- •4: Prenatal and Postnatal Imaging of the Bladder Epispadias-Exstrophy Complex
- •Introduction
- •Prenatal Imaging Findings
- •Bladder Exstrophy
- •Cloacal Exstrophy
- •Isolated Epispadias
- •Exstrophy Variants
- •Postnatal Imaging Findings
- •Urinary System
- •Musculoskeletal System
- •Spine
- •Conclusions
- •References
- •Introduction
- •Bladder Growth
- •Urinary Continence
- •Conclusions
- •References
- •6: Complete Primary Repair of Bladder Exstrophy and Epispadias
- •Bladder Neck Reconstruction, Bladder/Urethral Closure
- •Pubic Bone Closure
- •Umbilicoplasty
- •Immobilization
- •Urethral Plate Dissection
- •“Grady Monsplasty”
- •Complications
- •Conclusion
- •References
- •Introduction
- •Prenatal Diagnosis
- •Anatomic Anomalies
- •Immediate vs Delayed Closure
- •Surgical Reconstruction
- •Immobilization Techniques
- •Epispadias Repair
- •Achieving Urinary Continence
- •Proposed Follow-Up
- •Future Directions
- •Conclusion
- •References
- •8: The Kelly Procedure
- •Introduction
- •Tension-Free Bladder Neck Construction
- •Postoperative Management
- •References
- •Introduction
- •Anesthesia
- •Incision
- •Bladder Plate Mobilization
- •Radical Corporal Detachment
- •Osteotomy
- •Ischiopubic Osteotomy
- •Transverse Innominate Osteotomy
- •Corporal-Urethral Separation
- •Reconstruction
- •Postoperative Management
- •Follow-Up
- •Results
- •Conclusion
- •References
- •Introduction
- •Surgical Procedures
- •References
- •Bilateral Ureteral Advancement Reimplantation
- •Pelvic Osteotomy
- •Preoperative Testosterone Administration
- •Epispadias Repair
- •Penile Skin Reconstruction
- •Continence Enhancement
- •Conclusion
- •Introduction
- •Background
- •Modified Perineal Approach Surgical Technique
- •Discussion
- •References
- •Introduction
- •Posterior Iliac Osteotomies
- •Anterior/Double Iliac Osteotomies [3, 14]
- •Anterior Oblique Iliac Osteotomies [5, 11]
- •Anterior Bilateral Superior Pubic Rami Osteotomies [4]
- •Postoperative Immobilization
- •Complications/Long-Term Outcomes
- •References
- •Ureteral Reimplantation
- •Inguinal Hernia
- •Monsplasty
- •Umbilicoplasty
- •References
- •Introduction
- •Ureterosigmoidostomy
- •The Sigma-Rectum Pouch (Mainz Pouch II)
- •The Cologne Pouch
- •Conclusion
- •References
- •15: Cloacal Exstrophy
- •Introduction
- •Epidemiology
- •Embryologic Etiology
- •Prenatal Findings
- •Urinary
- •Gastrointestinal
- •Neurologic
- •Musculoskeletal
- •Genital
- •Management
- •Neonatal
- •Surgical Reconstruction
- •Secondary Procedures
- •Outcomes
- •Urinary Continence
- •Renal
- •Fecal Continence
- •Gender Rearing
- •Nutrition
- •Mobility
- •Psychosocial Outcomes
- •Conclusion
- •References
- •16: Male Epispadias
- •Embryology
- •Anatomic Features
- •Epispadias Repair
- •Pelvic Osteotomy
- •Modified Cantwell-Ransley Repair
- •Urethral Reconstruction
- •Bladder Neck Reconstruction
- •The Mitchell Repair
- •Initial Dissection
- •Penile Disassembly
- •Proximal Dissection
- •Bladder Neck Reconstruction
- •Primary Closure
- •Skin Closure
- •Outcomes
- •Fistula Formation
- •Urethral Stricture
- •Residual Curvature
- •Urinary Continence
- •Sexual Function
- •Renal Function
- •Female Epispadias
- •Outcomes
- •Conclusion
- •References
- •Introduction
- •Pre-operative Factors
- •Technical Aspects
- •Management
- •Failed Genital Reconstruction
- •Ureterosigmoidostomy
- •Augmentation Cystoplasty
- •References
- •Background
- •Preoperative
- •Monitoring
- •Intraoperative Management
- •Postoperative Management
- •Conclusion
- •References
- •Mental Health Concerns
- •Local Priority
- •Resources
- •Clinical Care
- •Capacity Building
- •Research
- •General Principles
- •References
- •Introduction
- •Defining Continence
- •Continence versus Dryness
- •Dry Interval: How Long Is Long Enough?
- •Dry Intervals: What Is Meaningful
- •Diversion Versus Continence
- •Timing
- •Challenging Dogma
- •References
- •Introduction
- •Preoperative Counseling
- •Bladder Neck Bulking Agent Injection
- •Artificial Urinary Sphincter
- •Bladder Neck Reconstruction
- •Bladder Neck Closure
- •Continent Catheterizable Channel: Mitrofanoff Principle
- •Augmentation Cystoplasty
- •Continent Urinary Diversion
- •References
- •22: Urinary Reconstruction for Bladder Exstrophy in the Developing World: Special Consideration and Technique
- •Introduction
- •Operative Technique
- •The Final Reconstruction
- •Young-Dees-Leadbetter Bladder Neck Plasty
- •Bladder Neck Closure
- •Operative details
- •Discussion
- •Conclusion
- •References
- •Introduction
- •Nephrology Evaluation
- •Measuring Kidney Function
- •Evaluating Blood Pressure
- •Imaging Studies
- •Transplant
- •References
- •Introduction
- •Post-operative Nursing Care
- •Pain Control
- •Immobilization
- •Orthopedic Care
- •Parental Teaching
- •Conclusion
- •Bibliography
- •Introduction
- •Pelvic Floor Musculature
- •Physical Therapy Evaluation
- •Participation
- •Activity
- •Impairment
- •Physical Therapy Intervention
- •Pre-toilet Training
- •Toilet Training
- •Post-toilet Training
- •Day Versus Night
- •Constipation
- •References
- •Pediatric Psychology
- •Infancy
- •Childhood
- •Adolescence
- •Adulthood
- •Future Directions
- •References
- •Females
- •Males
- •Erectile Function
- •Ejaculatory Function
- •Recommendations
- •Literature
- •Gynecologic Anatomy
- •Puberty
- •Pelvic Organ Prolapse
- •Fertility
- •Obstetric Considerations
- •Conclusions
- •References
- •Introduction
- •Patient Advocacy
- •Peer Support
- •Local Support Groups
- •Medical Advisory Council
- •Annual Conferences
- •Global Health Inequities
- •Global Health Initiatives
- •Advocacy Considerations
- •Patient-Directed Research
- •Patient Advisory Councils
- •Conclusion
- •References
- •Index

282
R. S. Joshi and J. Ramji
upto the levator hiatus with the urogenital bres lateral to the vesicourethral unit. This
is important to prevent the anterior displacement when the pubes are brought together
[22]. Gearhart etal. emphasise that during the primary closure, the bladder neck and
posterior urethra must be long enough and tight enough to prevent bladder prolapse/
eversion of bladder mucosa, at the same time allowing unimpeded egress of urine [6].
Vesicocutaneous stulae are technically considered failures, as the management
would necessarily imply a redo surgery [23].
Bladder Neck andOutlet
Bladder outlet obstruction is another troublesome form of failed closure. The severe
consequences of outlet obstruction on outcomes are main reasons why no formal
bladder neck reconstruction is attempted at the primary repair in multi stage repair of
exstrophy (MSRE); rather, a bladder outlet is created, which is incontinent, yet provides enough resistance to stimulate bladder growth [14]. In CPRE also, although the
epispadias repair is done simultaneously, there is no bladder neck reconstruction.
The symphyseal suture may contribute to stricture formation due to erosion into the
posterior urethra. Gearhart and associates have reported a high incidence of strictures
with the use of long paraexstrophy aps during bladder closure in MSRE, which are
prone to ischaemic necrosis. Ureteric stents exiting from the neourethra may be
another culprit, hence the importance of proper placement of tubes and their postoperative management. The neourethra must be calibrated before removing the
suprapubic tube [2, 24].
Outlet obstruction has also been observed in CPRE and Kelly’s repair, in form of
complete obliteration of the urethra. It is unclear whether this is due to technical
problems during extensive mobilisation causing ischaemia to the urethra [2].
The possibility of narrowing of the outlet can be suspected if there are episodes
of febrile urinary tract infections or development of hydronephrosis on follow-up
[6]. Bladder stones, difcult catheterisation, prolonged dry intervals, straining to
void with residual urine or recurrent episodes of epididymitis may be some other
presentations indicating outlet obstruction [24].
Complications like loss of urethral plate, glans, corporal body and clitoris may
happen in isolation or along with major modes of failure, constituting a ‘complex
failure’. Such soft tissue loss may be because of the radical dissection, more so in
CPRE [25]. The penile disassembly technique done incorrectly, accentuated by
vagaries in the blood supply of the penis, may contribute to an ischaemic injury to
the phallus [22]. Other reasons for the soft tissue injury could be arterial spasm,
venous congestion subsequent to compartment syndrome after a tight pubic closure
or an abnormal blood supply [25, 26]. Atrophy of one or both clitoral bodies has
also been observed, a probable consequence of ischaemic insult [27]. Partial or
complete loss of the urethral plate could result from damage to the terminal arteries
during dissection. Inadvertent injury to the bulbar artery as it exits from the common penile artery into the corpus spongiosum can lead to complete loss of the urethral plate [25]. In girls, loss of the urethrovaginal septum has been reported
secondary to the use of para-exstrophy aps [26] (Fig.17.2).

17 The Failed Exstrophy Repair: Technique andImplications
283
a
c
b
d
ef
Fig. 17.2
Complete dehiscence in a female. (c) Failed CPRE—bladder dehiscence with intact epispadias
repair. (d) Bladder prolapse in a male. (e) Prolapsing bladder in a female. (f) Vesicocutaneous stula. (g) Failed epispadias repair. (h) Stenosed hypospadiac meatus after CPRE causing outlet
obstruction. (i) Complex failure—loss of corpora. (j) Complex failure—loss of hemiglans
Various modes of failed exstrophy repair. (a) Complete dehiscence in a male. (b)

284
ij
R. S. Joshi and J. Ramji
g
h
Fig. 17.2 (continued)
Consequences ofFailed Repairs
A failed repair has negative long-term impacts on the patient and the healthcare
system [11]. The consequences of one or more failed repairs are increased scar formation and collagen deposition, decreased bladder growth, decreased capacity and
decreased rates of voided urethral continence [28]. A successful primary bladder
closure is the cornerstone to achieve sufcient bladder capacity and eventually continence. Each failed repair decreases the potential to gain adequate bladder capacity
[29]. Failure of the primary closure translates as a failure to get adequate bladder
capacity in over 50% patients. The MSRE uses the bladder capacity as the main
determinant for BNR.Kasprenski M etal. have reported that BNR alone as a continence procedure was successful in only 53.2% of the patients who were eligible for
this procedure [11]. Thus, only half of those who acquire reasonable capacity are
continent subsequently, suggesting that other factors like bladder compliance also

17 The Failed Exstrophy Repair: Technique andImplications
Fig. 17.3 Small capacity
bladder with scarred
abdominal wall after failed
repairs
285
play an important role [26]. The chances of continence with volitional voiding go
down to 30% with two closures and further to 20% with three or more closures [2].
Repeated surgical insults can also lead to brosis and contracture of the bladder
plate. This leads to a low-volume stiff bladder after reclosure. Peppas etal. have
shown the ratio of collagen to smooth muscle to be much higher in bladders that
have been subjected to multiple closures. The increased collagen formation
decreases the elasticity and compliance of the bladder, preventing the bladder from
reaching its full capacity with growth [16]. Hanna MK etal. report changes of cystitis cystica, cystitis glandularis and squamous metaplasia on bladder biopsy in
cases of failed repairs [30].
The scarring from previous surgery also increases the difculty of subsequent
repair, particularly the bladder neck reconstruction. It has been suggested by many
that 4–6months should be allowed for the wound to mature. However, waiting for
too long a period may aggravate the chronic inammation of the exposed bladder
plate and make it stiff and brotic [16] (Fig.17.3).
Consequences of Stricture and Obstruction Posterior urethral stricture and subsequent outlet obstruction impair the success of any repair signicantly. This is a
potentially dangerous complication as it poses a serious threat to the upper tracts
and slowly leads to deterioration of renal function [2, 22, 24] (Fig.17.4).
Redo surgeries and other salvage procedure after multiple failures are complicated, requiring longer surgical and post-operative recovery time [31]. The cost to
the patient, resource utilisation and burden on healthcare system is compounded
signicantly when the primary repair fails.
The afore-mentioned clinical and economic consequences of failure have raised
the question of centralisation of bladder exstrophy care. Patient outcomes may be
better optimised if the surgeries are done exclusively by teams with expertise earned
with large volume of cases. The improved outcomes at such high-volume centres of
excellence would probably offset the travel obstacles and costs [11, 31]. Long-term

286
R. S. Joshi and J. Ramji
a
c
b
Fig. 17.4 Effects of outlet obstruction. (a) High pressure bladder with reux. (b) Stone forma-
tion. (c) Compromised renal function

17 The Failed Exstrophy Repair: Technique andImplications
287
multi-institutional collaboratives involving experts from high-income countries
combined with local experience at well-equipped dedicated centres in middle- and
low-income countries are proving to a feasible model for alleviating the disease
burden in these areas [32].
Management
The initial management of a failed repair is conservative with debriding the devitalised tissue, removal of foreign bodies and treating wound infection with cultureappropriate antibiotics. There should be no attempts at immediate repeat closure. A
minimum interval of 4–6months is considered optimal before attempting any surgical procedure [3, 11]. This allows time for the wound to mature and the inammation to subside.
Before planning any surgical procedure for failure, a complete evaluation is mandatory. This should include examination under anaesthesia, pelvic x-rays, ultrasound and renal scans. Cystograms and cystoscopy are indicated in cases of prolapse,
vesicocutaneous stula or bladder outlet obstruction, while CT scans or MRI is
recommended when previous osteotomies have been done.
The options for a surgeon managing a failed exstrophy repair are delayed redo
bladder closure, redo bladder closure with epispadias repair, redo bladder closure
with concomitant bladder augmentation, excision of the bladder and continent urinary diversion, creating a urinary conduit [6, 33]. The precise management needs to
be individualised to correct the factors culpable for the initial failure. It is also necessary to consider the socio-economic circumstances, quality of life, and perception
of body image. In adolescents presenting with failure, continent urinary diversion
may be planned early [29].
Although a failed repair decreases the chances of a compliant, good capacity and
continent bladder, all failed primary repairs should be given a chance for redo repair,
if the bladder plate is healthy and genital reconstruction is possible.
Redo Exstrophy repair may be undertaken as a single-stage CPRE or as
MSRE.The basic tenets of a redo repair include: (1) radical dissection of the bladder and posterior urethra, (2) deep placement of the bladder, bladder neck and urethra as one unit in the pelvis, (3) osteotomy with external xators or traction to
secure post-operative immobilisation, (4) adequate post-operative sedation, pain
relief and control any distracting forces and (5) pre-operative intramuscular testosterone, which by increasing phallic length and girth may reduce likelihood of inadvertent injury [26].
The redo repair outlined below is what the authors follow and is much the same
as the complete primary repair described in another chapter in this textbook. The
challenge at the outset is to enter the correct surgical planes between the bladder
detrusor and rectus fascia circumferentially around the bladder plate. The scarred
and brous tissue from the previous failed repair will often render these planes difcult to identify, so care must be taken to ensure that the detrusor muscle of the
bladder is preserved. Once the planes are identied, the goal is to radically dissect

288
R. S. Joshi and J. Ramji
the vesicourethral unit, releasing all the adhesions from the previous surgery. The
planes between the detrusor muscle and fascia are often easiest to visualise laterally,
where the dissection begins to identify the medial border of the anterior rectus
sheath. The idea is to enter the perivesical fat and dissect the bladder superiorly and
inferiorly on either side. The supercial ligaments of the bladder are divided, but it
is recommended not to continue with the deep pelvic dissection at this point to avoid
injury to the corpora cavernosa that may not be fully visualised in this anterior plane.
Rather, the superior dissection at the dome of the bladder is performed, separating the peritoneum and supercial adhesions from the dome. In redo cases, sometimes it is impossible to avoid entering the peritoneum. If this occurs, the
peritoneotomy may even facilitate lateral dissection when the planes are especially
obliterated from previous surgery.
Once this lateral dissection is completed in an antegrade direction up to the lateral intersymphyseal bands, we proceed to the penile skin dissection. Regardless of
whether a previous epispadias repair has been done, the penile skin is degloved, the
neurovascular bundles are identied all the way to their course underneath the bone
at the corporal insertion into the pubic bone. Often this dissection is particularly
difcult as the brosis from the previous surgery can cause the bundles to be displaced from their original position. Once the bundles are safeguarded, the deep
intersymphyseal bands are accurately identied and divided under vision (Fig.17.5).
The radical division of all the bands in this area between the bladder neck, urethra
and the pubis helps to completely free the bladder plate, which can then be easily
lifted up or pushed into the pelvis without any restriction. It would be worth bearing
in mind that enthusiastic medial dissection at this point could compromise the width
of the bladder neck.
Often, a failed repair may also have a failed epispadias repair, or it might be a
failure of the rst stage of bladder closure in a MSRE, where the epispadias repair
had not been undertaken. It is invariably necessary to dissect the urethral plate or the
reconstructed tube off the corpora and redo the epispadias repair to adequately
mobilise the vesicourethral unit. The modied Cantwell-Ransley technique is
applied, separating the urethral plate rst ventrally and then dorsally starting at
Fig. 17.5 Lateral
dissection—identication
and division of deep
intersymphyseal bands

17 The Failed Exstrophy Repair: Technique andImplications
289
midshaft and moving proximally and distally. The broses from the previous surgery make this dissection tedious. The proximal prostatic and bladder neck dissection is tricky, where the urethra may get thin or narrow. Similarly, the distal dissection
from the glans may be troublesome. The tip of the urethral plate is kept connected
to the glans. Sometimes, however, the proximal radical mobilisation may shorten
the urethra, or the distal plate may not be supple enough, or may have been damaged
in the previous surgery. In such cases, it is more advisable to give a hypospadiac
meatus, as described by Mitchell and Bagli’s modication with a complete disassembly [22]. The pre-operative use of testosterone has been advocated as a means
to enhance the growth of the urethral plate, increase its pliability and vascularity and
minimise the incidence of hypospadias, especially in redo cases and older children
[26, 34]. The authors have also been able to obtain an orthotopic meatus in most
cases with the use of pre-operative testosterone enanthate intramuscular injections
(2mg/kg at 5weeks and 3weeks before surgery) combined with topical application
of testosterone cream.
Concomitant bilateral ureteric reimplantation may be possible in some redo
repairs based on the size and quality of the bladder plate. A cephalotrigonal or crosstrigonal method may be used. The safety and efcacy of reimplantation during
CPRE has been well established [35].
The closure of the vesico-urethral unit begins at the bladder neck. There is no
formal bladder neck reconstruction, but an attempt to funnel the bladder neck area
is made to increase the outlet resistance without causing obstruction. This is done
by marking an area of approximately 18 to 20mm width depending on the age and
clinical appearance, denuding lateral triangular strips of mucosa for creating a
10mm length of funnelled wall. Three or four sutures with 4–0 or 5–0 PDS are
taken, to be tied later after the bladder closure.
The bladder closure is started after bringing out and securing the ureteric catheters, suprapubic tube and a per-urethral stent. The closure is done in single or double
layer with 3-0 PDS, with simple or mattress sutures, as per the quality of the bladder
and surgeon’s preference. The urethroplasty is done preferably with interrupted
sutures, leaving the glansplasty for after pubic bone approximation.
As discussed earlier, osteotomy is imperative for a successful redo closure. An
adequate osteotomy will allow the surgeon to place the bladder and posterior urethra in the pelvis while the hip bones are internally rotated to bring the pubes in
close approximation to bridge the intersymphyseal gap. Depending on the severity
of the diastasis, expertise of the orthopaedic surgeon and whether a previous osteotomy has been done, the osteotomy best suited for the patient can be performed.
Sometimes a combination of transverse innominate and vertical iliac osteotomy
may be necessary, if the diastasis cannot be overcome by a single osteotomy. The
authors have found the modied Salter’s osteotomy to be adequate in most cases.
Usually, a single strong 1-0 PDS suture securely knotted on the outside is sufcient
to keep the approximation in place. Sometimes an additional suture may be necessary. Such a snug approximation can sometimes cause vascular compression and
create a penile compartment syndrome. Active bleeding from the glans and any
glans congestion should be checked for before being satised with the symphyseal

290
Fig. 17.6 Leg traction and
external xators for secure
immobilisation
R. S. Joshi and J. Ramji
closure. It may be necessary to release the suture and re-approximate with a small
gap between the pubic bones to ensure that there is no vascular compromise.
The glansplasty is now completed; the urethra is shifted ventrally, and the corpora are approximated dorsally in external rotation with 4-5 sutures of 5-0 PDS, as
demonstrated by Pippe Salle [36]. Depending on the availability and quality of skin,
the penile skin cover can be done by different techniques: a ventral rotational skin
ap described by Pippe Salle, dorsal transposition of a ventral ap (reverse Salle
technique), a transverse island ap or reverse Byar’s aps. Except for the reverse
Byar’s, all the others aim at avoiding a dorsal suture line, which improves the cosmesis [37, 38].
The abdominal wall closure can now be done in a tension-free manner. In rare
instances, lateral relaxing incisions can be given, to decrease undue tension. It is
vital to ensure that the tubes are all securely xed before the patient is shifted. Our
preferred method of immobilisation for stabilising the osteotomy is the use of external xators for 4weeks. The addition of leg traction like modied Bryant’s may
provide extra security (Fig.17.6).
Post-operative care focuses primarily on immobilisation, adequate pain relief
with epidural analgesia, wide spectrum antibiotics and antispasmodics to prevent
bladder spasms, along with the prevention of any other causes of raised abdominal
pressure such as distension and constipation. Ureteric stents are left in for 2weeks
and suprapubic drainage is maintained for 6weeks (Fig.17.7).

bc
17 The Failed Exstrophy Repair: Technique andImplications
a
291
Fig. 17.7 Redo repair in failed exstrophy. (a) Failed repair with complete dehiscence. (b) Redo
CPRE with osteotomies. (c) Outcome on 6-month follow-up
Use ofRectus Muscle Flaps
The bladder neck region and intersymphyseal area are the most vulnerable areas for
dehiscence or stula formation. The use of rectus muscle pedicled aps has been
described to reinforce the closure in the inter-pubic gap in redo bladder closures and
repair of vesicocutaneous stulae; as well as in bladder neck closures and bladder
augmentation in failed repairs [13, 39, 40] (Fig.17.8).
We have similarly used the rectus muscle ap in some redo repairs for reinforcing the closure, and sometimes also as an alternative to osteotomy in older patients.
The rectus muscle is separated off the anterior and posterior rectus sheaths, taking
care not to damage the anterior sheath. The muscle is divided at the upper end of the
incision and mobilised till its attachment to the pubic bone and preserving its vascular supply from the inferior epigastric vessels. The muscle ap can now be turned
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