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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

16 Male Epispadias
Recently, Weiss et al. reported results from the Multi-Institutional Bladder
Exstrophy Consortium (MIBEC). Of 8 patients with glandular epispadias, 5 were
continent (dry for 3h or more), 1 had intermediate continence (dry for 2h or less),
and 1 was incontinent. Of 8 patients with penile epispadias, 6 were continent and 2
had intermediate continence. Of 20 patients with penopubic epispadias, 10 were
continent, 2 of whom required clean intermittent catheterization, 5 had intermediate
continence with one of them on clean intermittent catheterization, and 5 were wet.
This series also showed that the proximal repairs will require a bladder neck reconstruction to achieve continence but that even the more distal defects may have bladder neck incompetence and persistent incontinence [34].
271
Sexual Function
The cosmetic appearance and sexual function of males undergoing isolated epispadias repair are not robust. Penile length, quality of erections, and ejaculatory function all play a role in sexual function. Reddy etal. reported that 73% of the males
with epispadias reported being engaged in sexual activity, yet 67% (10/15)reported
having one or more issues related to sexual function [32]. These included abnormal
ejaculation, difcult orgasm, decreased sensation, difculty maintaining erections,
penile appearance, and curved erections.
Of Sunil etal. series of patient with male epispadias, only 15 (20%) completed
the questionnaire. Although 12 patients (80%) reported overall satisfactory sexual
intercourse, 11 (73%) admitted to 1 or more problems with sexual function, including abnormal ejaculation (53%), diminished sensation (20%), and difculty maintaining an erection (20%) [35].
Of 31 epispadias patients in Bencic et al. series, 7 (22.6%) completed the
International Index of Erectile Function (IIEF) questionnaire. The average score
was 21.3 (19–26) for the erectile function domain and 8.7 (7–10) for the overall
satisfaction domain. Only 2 patients (28.6%) had lower scores in the erectile function domain. Three of the 7 patients had lower scores in the orgasmic domain [29].
Renal Function
Bladder neck reconstruction may place patients with epispadias at risk for renal
compromise over time. This risk is lower than in bladder exstrophy as bladder
capacity is near-normal in most children with isolated epispadias.
In a series by Joshi etal. from the International Bladder Exstrophy Consortium
of 12 patients with isolated epispadias, eGFR was normal in all. Vesicoureteral
reux was present in one patient who also developed renal scarring. This suggests
that patients with vesicoureteral reux are at risk for pyelonephritis and possible
renal scarring, and they should be followed closely after repair [36].

272
P. A. Merguerian
Female Epispadias
Primary female epispadias is rare and occurs in an estimated prevalence of 1:160,000
to 1:480,000 live births [37]. Some of these patients present later on in life when
they are unable to be toilet-trained.
In this condition, the dorsal aspect of the urethra is absent and the urinary sphincter including the bladder neck is decient. The clitoris is bid, the perineal body is
broadened, and the vagina is anterior. The mons pubis is absent, and the anterior
labia are laterally displaced but are fused in the midline posteriorly. The vagina may
be stenotic [1]. The internal genital organs (uterus, fallopian tubes, and ovaries) are
usually unaffected.
The goals in managing these patients include the following:
1. Creation of a straight urethra
2. Good cosmetic appearance with reapproximation of the genitalia in the midline
3. Reconstructing the bladder neck for continence
4. Maintaining clitoral function
The traditional surgical approach was a staged reconstruction with the second
stage being the bladder neck reconstruction [38]. Manzoni and Ransley presented
an alternative single-stage technique in 2009, where the urethra, bladder neck, and
clitoris were repaired by a perineal approach, but without tailoring the bladder neck
to enhance outlet resistance [39]. This approach was then modied to perform a
bladder neck tailoring through a perineal approach with favorable outcomes [40,
41]. The advantages of using this approach include improved continence and
reduced morbidity. The radical soft-tissue mobilization described by Kelly has been
applied for female primary epispadias [42].
With the complete primary epispadias repair, the bladder neck, urethra, and
vagina are mobilized as a unit. Osteotomies may be necessary when the pubic diastasis is wide. The intersymphyseal bands are deeply incised to allow the bladder
neck and urethra to move posteriorly. The bladder neck is then reconstructed and the
urethra tubularized. The symphysis pubis is reapproximated.
The Richard Grady Monsplasty is then performed. This is a vertical Z-plasty
closure to provide a 2-layer closure of the mons that decreases tension and improves
cosmetic appearance by recreating a clitoral hood that provides a more normal
appearance of the external genitalia for girls with bladder exstrophy. This has been
described in the article by Weiss DA etal. that also includes a video of the technique [43].
The perineum is described elsewhere in this book by Pippi Salle etal.
The Kelly repair of female epispadias is described by Leclari MD etal. [42].
Supplementary video related to this article can be found at https://doi.org/10.1016/j.
jpurol.2017.08.017.
Through a short transpubic midline incision, the lateral aspect of the bladder is
exposed, giving access to the superior aspect of the pelvic oor. The most anterior

16 Male Epispadias
273
bers of the levator ani muscle are incised at the level of their insertion on the internal obturator muscle fascia, providing exposure to the ischiorectal fat and the urogenital diaphragm. Perineal dissection allows adequate exposure to the corpora
cavernosa. A muscle stimulator is used to identify the bulbospongiosus, ischiocavernosus, and transverse perineal muscles. The periosteum of the ischiopubic rami is
peeled away to allow mobilization of both corpora cavernosa, until the neurovascular pedicle exiting from the Alcock’s canal is identied. All striated muscular structures, part of the urogenital diaphragm, linking the urogenital complex to the
anterior pelvic ring are detached from the bone. Further dissection, on the medial
aspect of the corpora, allows separation of the corpora from the urethral plate, which
eventually allows repositioning of the bladder neck deep into the pelvis.
Reconstruction included ureteral reimplantation if necessary, bladder neck funneling reconstruction, and urethral reconstruction with tubularization over an 8 or 10F
catheter. Muscular bers detached from the pelvis and bers of the bulbospongiosus
muscle are wrapped around the bladder neck and the upper urethra whenever possible. A corporoplasty is performed by approximating both corpora cavernosa ventrally to the neo-urethra on the midline, and both glans and clitoris are covered by a
common clitoral hood.
Outcomes
Alyami etal. reported on the long-term outcomes comparing the traditional YoungDees- Leadbetter repair versus the modied perineal approach. They had a total of
12 patients with isolated female epispadias, three of whom underwent the traditional repair and 7 underwent the perineal repair. None of the 3 patients who underwent the traditional repair were continent, while 4 of 7(57%) who underwent the
perineal approach were continent.
Leclair et al. compared the perineal approach to the Kelly repair [42]. They
included 16 patients in their study. Seven females underwent the perineal approach,
and of these, 5 of 7 were continent during the day and 4 of 5 were also continent at
night. They state that 3 of the 5 required bladder neck injection to achieve continence. Of the 9 patients undergoing the Kelly repair, 8 were evaluable and all
achieved diurnal continence and 3 of 8 were fully continent though one required
clean intermittent catheterization for obstructive micturition.
There are signicant limitations to the above studies including the small number
of patients in the surgical group. The comparison groups may also not have been
similar as the more severe forms of epispadias most likely required a more radical
approach. Because of the rarity of this condition, multi-institutional and longer term
studies are required to determine the benet of the complete primary repair versus
the perineal repair versus the Kelly repair.

274
P. A. Merguerian
Conclusion
Epispadias represents one end of the spectrum of the bladder exstrophy-epispadias
complex. Repair of epispadias is a challenging operation even in expert hands. The
incidence is rare, and repair should be performed in centers of excellence in order to
provide the best possible outcome.
As this condition is rare, published long-term outcomes related to continence and
sexual function are sparse. Studies show that continence can be achieved in the
majority of patients with epispadias but over 80% require multiple procedures to
achieve social continence. Erectile and ejaculator functions are satisfactory, yet
there is continued dissatisfaction with genital appearance.
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8. Arap S, Nahas WC, Giron AM, etal. Continent epispadias: surgical treatment of 38 cases. J
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12. Gearhart JP, Leonard MP, Burgers JK, Jeffs RD.The Cantwell-Ransley technique for repair of
epispadias. J Urol. 1992;148:851–4.
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14. Grady RW, Mitchell ME.Management of epispadias. Urol Clin N Am. 2002;29:349–60.
15. Gearhart JP, Jeffs RD. The use of parenteral testosterone therapy in genital reconstructive
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16. Makedonsky J.The use of Human Chorionic Gonadotropin (HCG) for penile reconstruction in
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19. Gearhart JP, Sciortino C, Ben-Chaim J, Peppas DS, Jeffs RD.The Cantwell-Ransley epispadias repair in exstrophy and epispadias: lessons learned. Urology. 1995;46:92–5.
20. Bar-Yosef Y, Sofer M, Ekstein MP, Binyamini Y, Ben-Chaim J.Results of Epispadias repair
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21. Kramer SA, Jackson IT.Bilateral rhomboid aps for reconstruction of the external genitalia in
epispadias-exstrophy. Plast Reconstr Surg. 1986;77:621–31.
22. Pippi Salle JL, Elifranji M, Abbas T, Alhadi A, Elkadhi A, Leslie B, Vallasciani S.Technical
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https://doi.org/10.1016/j.urolvj.2022.100127.
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24. Pippi Salle JL, Jednak R, Capolicchio JP, Franca IMP, Labbie A, Gosalbex R.A ventral rotational skin ap to improve cosmesis and avoid chordee recurrence in epispadias repair. BJU
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275

The Failed Exstrophy Repair: Technique
andImplications
RakeshS.Joshi andJaishriRamji
Introduction
Over the past several decades, there has been a dramatic evolution in the management of bladder exstrophy. Advanced surgical techniques and improved postoperative management have contributed to better results in functional and cosmetic
outcomes. Successful primary closure is a key factor for bladder growth and eventually continence. In the modern era, contemporary success rates of exstrophy closure
have improved signicantly, and the long-term continence rates range around
45–60% depending on the type of repair done [1].
With increasing experience and knowledge of the surgical anatomy, the incidence of complete dehiscence has decreased from 13% to 1% [2]. Yet failures in
different forms still occur frequently. A failed exstrophy closure is a devastating
setback to subsequent genitourinary reconstruction, urinary continence and longterm impact on the patient and healthcare system.
The occurrence of bladder prolapse, complete dehiscence, vesicocutaneous stula, outlet obstruction or any combination of factors along with loss of urethra,
corpora or glans/clitoris constitutes a failed exstrophy closure. Although repeat closure is often feasible, there are negative implications on the fate of the urinary tract.
The chances of achieving continence after failed and multiple closures decrease
dramatically. Children with one or more failed closures are ultimately an extremely
challenging subset with poorer outcomes.
17
Supplementary Information The online version contains supplementary material available at
https://doi.org/10.1007/978- 3- 031- 91238- 2_17.
R. S. Joshi (*) · J. Ramji
Department of Pediatric Surgery, B. J. Medical College and Civil Hospital,
Ahmedabad, Gujarat, India
© 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_17
277

278
In this chapter, we will try to outline the possible reasons for failed closures, the
types of outcomes that constitute failure, the early and late management of failed
closure, including principles of redo closures, and the outcomes of redo exstrophy
closures.
R. S. Joshi and J. Ramji
Reasons forFailure ofPrimary Closure
Regardless of choice of surgery, the objectives of surgery should aim at tension-free
closure of bladder, pelvic ring and abdominal wall; protection of the upper tracts by
avoiding outlet obstruction and pyelonephritis; and preservation of the genital and
urethral soft tissues. Consequently, a failed closure results when any one of these
objectives is not accomplished adequately.
Pre-operative, technical or post-operative causes can be the attributable factors
for failure, either in isolation, or more commonly, multifactorial.
Pre-operative Factors
Various patient-related factors may contribute to failure of the primary repair; a
wide pubic diastasis, an ill or undernourished infant and an unprotected or improperly preserved bladder plate, which delays the primary surgery. Technical precision
and the surgeon and institutional experience, along with lack of concomitant osteotomy, are also known to impact the outcomes [3].
Technical Aspects
From a technical point of view, adequate vesicourethral mobilisation is a key factor
contributing to successful closure. Experienced surgeons advocate aggressive posterior repositioning of the bladder, bladder neck and the urethra. This is based on the
principle that exstrophy represents an anterior bladder herniation. The posterior
placement is facilitated by the simultaneous dissection of bladder and urethral plate
and division of the intersymphyseal band. Failure to release this band increases the
chances of dehiscence [4, 5]. Surer etal. and EL Sherbiny etal. have showed that
this principle helps prevent signicant complications even in repair of failed closures [4].
The rate of dehiscence may also be correlated with exiting the drainage catheters from the neo- urethra, rather than a suprapubic exit site [6]. This manoeuvre
may cause ischaemia, especially if paraexstrophy aps are used. The aps can
necrose and cause the inter-pubic stitch to erode into the urethra, with resultant
infection, dehiscence or urethral stricture. Currently the role of paraexstrophy
aps is limited mostly to staged repairs when there is severe shortening of the
urethra [7].

17 The Failed Exstrophy Repair: Technique andImplications
279
The Role ofOsteotomy
Studies have proven the importance of osteotomy in the surgical treatment of bladder exstrophy. The major role of osteotomy is in relaxing the tension on the bladder
closure and the abdominal wall closure to allow for tension-free, adequate healing.
In the absence of osteotomy, the abnormal separation of the pubic bones and the
external rotation of the innominate bones place a signicant tension on the soft tissues during reconstruction of the bladder, urethra and the anterior abdominal wall.
It has been documented that the use of osteotomy has improved the success rates of
soft tissue closure and urinary continence and resolved the gait abnormalities [8].
Some surgeons believe that when the primary repair is done in newborns, ligamental laxity allows the pelvis to be closed without signicant tension. Osteotomy
becomes mandatory once exstrophy repair is delayed beyond 72h, in cases of
poorly malleable pelvis and when the pubic diastasis is more than 4cm [9, 10]. A
rm anterior pelvic ring resulting from osteotomy supports the urethral suspension
and contributes in preventing bladder prolapse or dehiscence [8]. Gearhart has
reported in his study of 170 failed repairs that osteotomy had been done in only 29%
at the time of the primary repair [11]. The rate of failure of abdominal wall closure
and bladder closure has diminished to less than 5% at experienced centres with the
incorporation of osteotomy in the repair of bladder exstrophy. Various types of osteotomies have been described, like posterior iliac, anterior osteotomy of the superior
pubic rami, anterior diagonal iliac, modied Salter’s innominate and vertical iliac
ramotomy [8]. The combined transverse innominate and vertical iliac osteotomy
have been shown to have lower rates of dehiscence and prolapse compared to other
osteotomies [10]. The benets of osteotomy far outweigh the complication rate of
4% shared collectively by the various osteotomies [8]. At the authors’ institute, all
primary repairs are done after 3months of age with osteotomy in all cases. The
dehiscence rate has been less than 2%, and there have been very few
complications.
On the other hand, certain authors believe that osteotomy is not an essential element of the exstrophy repair. The downsides are the added morbidity, complications
and requirement of an expert orthopaedic surgeon. Even after the osteotomy, the
pubes eventually drift apart over a period of time; and hence the osteotomy itself
may not be a long-term contributor to continence [12]. Bhatnagar etal. and Sudipta
etal. have reported good results with the use of rectus aps to facilitate the closure
instead of osteotomy and believe that it is not necessary either for the closure or for
the achievement of continence [10, 13].
When talking about failed repairs and redo closures, the pendulum swings greatly
in favour of osteotomy. There is ample evidence of osteotomy increasing the success rates of reclosures as reported by various authors [8, 14–16]. The greatest success rates in reclosures have been achieved when the osteotomy was combined with
secure post-operative immobilisation with external xators and modied Buck’s
traction. Conversely, the outcomes were universally poor regardless of the type of
immobilisation, if the osteotomy was not done [15] (Fig.17.1).

280
ab
Fig. 17.1 Complete dehiscence within days of closure—no osteotomies done. (a) Male.
(b) Female
R. S. Joshi and J. Ramji
Post-Operative Sources ofWound Tension
Successful closure depends as much on reducing the tension on the closure as on
maintaining the tension-free closure in the post-operative period. Inadequate analgesia, poor immobilisation, abdominal distension and poor urinary drainage are
some of the factors that can increase the wound tension in the post-operative period
and lead to a failed repair [14].
In the initial post-operative period, the focus should be on controlling these
adverse forces aggressively. Epidural analgesia with hourly pain assessments and
adjunctive sedation whenever needed is crucial to keep the child still and comfortable. The nasogastric tube should be removed only when the child is stooling adequately, and abdomen is soft.
Post-operative immobilisation plays a crucial role in successful closure. It allows
the tension-free closure enabled by osteotomy to heal adequately. The use of
mummy wraps and spica casts has been shown to be rife with complications, with
little benet [15]. Modied Buck’s traction or modied Bryant’s traction has been
advocated with satisfactory outcomes [10, 11]. The best results have been reported
with the external xators along with modied Buck’s traction for 6–8weeks. The
leg traction may be the most effective way to control the movement of the legs as
well as keep the buttocks and pelvis at on the bed [15].
Proper management of the outlet of the reconstructed bladder is of prime importance. Early dislodgement of the urinary drainage tubes poses a signicant risk for
failure [17]. Special attention should be given to securing the tubes and leaving
them in for an adequate period. Generally, we leave the ureteric catheters for
14days, the per-urethral stent for 21days and the suprapubic tube for 6 weeks.
However, there may be institutional variations in the protocol [6, 12, 13]. The closure can also be compromised by wound infection, ultimately leading to dehiscence
or a stula. Hence the importance of broad-spectrum intravenous antibiotics and
nutritious diet cannot be overemphasised.

17 The Failed Exstrophy Repair: Technique andImplications
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Modes ofFailure
Failure of primary exstrophy repair can manifest as dehiscence, prolapse of bladder,
vesicocutaneous stula, urethral stricture or bladder neck obstruction. A complex
failure constitutes a combination of one or more of these along with corporal, urethral or glans loss.
Kasprenski etal. have reviewed the outcomes of 170 patients with a failed closure. They documented bladder prolapse and dehiscence as the commonest forms
(around 35%) and complex failures comprised another 11% [11].
Modes of primary closure failure
Prolapse 61 35.9
Dehiscence 60 35.3
Vesicocutaneous stula 05 2.9
Obstruction 06 1.8
Complex 19 11.2
Unknown 15 8.8
Others 07 4.1
n
%
Complete dehiscence of the bladder and abdominal wall often occurs early in the
post-operative period. The causes for this disastrous occurrence have been described
in the previous section. One of the most important causes is the technical failure.
Irrespective of the type of repair done, radical mobilisation of the vesicourethral
unit for it to be placed deep in the pelvis is the critical step [4, 18]. Gearhart and
Jeffs emphasise the need for this radical dissection along with a secure pelvic ring
closure in MRSE.A study by Gearhart compared the success rates with and without
osteotomy and with different methods of immobilisation. Success rate of 75% was
documented with osteotomy and went up to 96% with immobilisation by external
xators or modied Buck’s traction. Spica cast and mummy wrapping were found
to be far less effective [15].
Complete primary repair of exstrophy (CPRE) as described by Grady and Mitchell
also targets at returning the anatomy to more normal location, allowing pelvic diaphragm to assist in continence. The dissection of the intersymphyseal band, the anterior part of the perineal membrane from the pubis, is essential for deep placement of
the vesicourethral unit. The bladder, bladder neck and urethra are mobilised and
closed as one unit, using penile disassembly and partial bladder neck tailoring [4, 5,
19]. Proponents of CPRE report good early outcomes with low rates of failures in
form of dehiscence, stula and soft tissue loss. The failure rates go up to 58% in
cases done without an osteotomy [20, 21].
Another common form of failure is bladder prolapse often not recognised in the
early post-operative period as it is less obvious. The abdominal wall is intact as is the
bladder closure, but the bladder prolapses inferiorly through a separated pubic diastasis or out the bladder neck. (3) Insufcient dissection of the bladder and urethra from
the inferior ramus of the pubis moves the outlet cephalad and increases the chances of
bladder prolapse. Similarly, the dissection must be adequate posteriorly and inferiorly
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