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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

12 Osteotomies forBladder Exstrophy
219
rate with a comprehensive approach involving osteotomies, external xation, and
Buck’s traction [21]. However, attributing the success solely to osteotomies proves
challenging due to inherent patient differences between those who receive osteotomies and those who do not. Nonetheless, cases of primary closure failure documented in the literature often lacked osteotomies. Long-term orthopedic outcomes
generally lean toward favorability, with most patients achieving normal activity levels and mobility comparable to the general population. However, potential longterm adverse outcomes may include hip dysplasia, osteoarthritis, and excessive
external rotation foot progression angle [7].
Complications associated with orthopedic reconstruction are generally rare.
According to Okubadejo etal., there is a reported 4% rate of orthopedic complications, excluding pin site problems [15]. Although nerve palsies may occasionally
occur following osteotomies, they tend to be transient in nature. Non-union at the
osteotomy site is infrequent but may arise with larger corrections. Wound complications, especially those linked with posterior incisions, are not uncommon.
Additionally, pressure sores resulting from casts or traction devices require vigilant
monitoring and appropriate management, as they represent the most prevalent
orthopedic issue [15].
References
1. Baird AD, Sponseller PD, Gearhart JP.The place of pelvic osteotomy in the modern era of
bladder exstrophy reconstruction. J Pediatr Urol. 2005;1(1):31–6. https://doi.org/10.1016/j.
jpurol.2004.09.001. PMID: 18947531.
2. Cardin S, Herrera-Soto J, Marrero P, Rich M, Swana H, Langford J.Pelvic ring stabilization
using anterior subcutaneous internal xation in bladder exstrophy repair. J Pediatr Orthopaedic
Soc North Am. 2020;2(3):132.
3. de Mattos CB, Mendes PH, Boechat PR, Júnior JL, da Silva Guimarães L. Bilateral anterior
pelvic osteotomy for olosure of bladder exstrophy: description of technique. Rev Bras Ortop.
2015;46(1):107–13.
PMCID: PMC4799192.
4. Frey P, Cohen SJ. Anterior pelvic osteotomy. A new operative technique facilitating primary bladder exstrophy closure. Br J Urol. 1989;64(6):641–3. https://doi.org/10.1111/
j.1464- 410x.1989.tb05327.x. PMID: 2627639.
5. Giordano M, Di Lazzaro A, Guzzanti V, Careri S, Bagolan P, Zaccara A, Toniolo R.Oblique
pelvic osteotomy in the treatment of bladder exstrophy in neonates. J Pediatr Orthop
B. 2019;28(3):207–13. https://doi.org/10.1097/BPB.0000000000000614.
6. Grady RW, Mitchell ME.Complete primary repair of exstrophy. J Urol. 1999;162(4):1415–20.
PMID: 10492227.
7. Jani MM, Sponseller PD, Gearhart JP, Barrance PJ, Genda E, Chao EY.The hip in adults with
classic bladder exstrophy: a biomechanical analysis. J Pediatr Orthop. 2000;20(3):296–301.
PMID: 10823593.
8. Jones D, Parkinson S, Hosalkar HS.Oblique pelvic osteotomy in the exstrophy/epispadias
complex. J Bone Joint Surg Br. 2006;88(6):799–806.
X.88B6.17712. PMID: 16720777.
9. Kenawey M, Morakis E, Cervellione R, Keene D, Kelley SP.The true pelvic volume change
with various corrective osteotomy techniques for exstrophy-epispadias complex spectrum: the
https://doi.org/10.55275/JPOSNA- 2020- 132. ISSN 2768-2765.
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value of computer-assisted virtual surgery. J Pediatr Orthop B. 2023. https://doi.org/10.1097/
BPB.0000000000001145. Epub ahead of print. PMID: 38189741.
10. Lloyd-Roberts GC, Williams DI, Braddock GT.Pelvic osteotomy in the treatment of ectopia vesicae. J Bone Joint Surg Br. 1959;41-B:754–7.
X.41B4.754. PMID: 13853691.
11. McKenna PH, Khoury AE, McLorie GA, Churchill BM, Babyn PB, Wedge JH.Iliac osteotomy: a model to compare the options in bladder and cloacal exstrophy reconstruction. J
Urol. 1994;151(1):182–6; discussion 186–7.
PMID: 8254810.
12. Meldrum KK, Baird AD, Gearhart JP. Pelvic and extremity immobilization after bladder
exstrophy closure: complications and impact on success. Urology. 2003;62(6):1109–13.
https://doi.org/10.1016/s0090- 4295(03)00791- x. PMID: 14665365.
13. Mundy A, Kushare I, Jayanthi VR, Samora WP, Klingele KE.Incidence of hip dysplasia associated with bladder exstrophy. J Pediatr Orthop. 2016;36(8):860–4. https://doi.org/10.1097/
BPO.0000000000000571. PMID: 26090968.
14. Nhan DT, Sponseller PD. Bilateral Anterior Innominate Osteotomy for Bladder Exstrophy.
JBJS Essent Surg Tech. 2019;9(1):e1. https://doi.org/10.2106/JBJS.ST.18.00018. PMID:
31086719; PMCID: PMC6485763.
15. Okubadejo GO, Sponseller PD, Gearhart JP. Complications in orthopedic management of
exstrophy. J Pediatr Orthop. 2003;23(4):522–8. PMID: 12826954.
16. O’Phelan EH. Iliac osteotomy in exstrophy of the bladder. J Bone Joint Surg Am.
1963;45:1409–22. PMID: 14069780.
17. Schmidt AH, Keenen TL, Tank ES, Bird CB, Beals RK.Pelvic osteotomy for bladder exstrophy. J Pediatr Orthop. 1993;13(2):214–9. PMID: 8459014.
18. Shnorhavorian M, Song K, Zamilpa I, Wiater B, Mitchell MM, Grady RW. Spica casting
compared to Bryant’s traction after complete primary repair of exstrophy: safe and effective in a longitudinal cohort study. J Urol. 2010;184(2):669–73. https://doi.org/10.1016/j.
juro.2010.03.057. Epub 2010 Jun 19. PMID: 20639033.
19. Shultz WG.Plastic repair of exstrophy of bladder combined with bilateral osteotomy of ilia. J
Urol. 1958;79(3):453–8.
20. Sponseller PD, Bisson LJ, Gearhart JP, Jeffs RD, Magid D, Fishman E.The anatomy of the
pelvis in the exstrophy complex. J Bone Joint Surg Am. 1995;77(2):177–89. https://doi.
org/10.2106/00004623- 199502000- 00003. PMID: 7844123.
21. Sponseller PD, Jani MM, Jeffs RD, Gearhart JP. Anterior innominate osteotomy in
repair of bladder exstrophy. J Bone Joint Surg Am. 2001;83(2):184–93. https://doi.
org/10.2106/00004623- 200102000- 00005. PMID: 11216679.
22. Stec AA, Pannu HK, Tadros YE, Sponseller PD, Fishman EK, Gearhart JP. Pelvic oor
anatomy in classic bladder exstrophy using 3-dimensional computerized tomography: initial
insights. J Urol. 2001;166(4):1444–9. PMID: 11547108.
23. Suson KD, Sponseller PD, Gearhart JP. Bony abnormalities in classic bladder exstrophy:
the urologist’s perspective. J Pediatr Urol. 2013;9(2):112–22. https://doi.org/10.1016/j.jpu-
rol.2011.08.007. Epub 2011 Nov 21. PMID: 22105005.
24. Vining NC, Song KM, Grady RW. Classic bladder exstrophy: orthopaedic surgical considerations. J Am Acad Orthop Surg. 2011;19(9):518–26. https://doi.
org/10.5435/00124635- 201109000- 00002. PMID: 21885697.
25. Wakim A, Barbet JP.Connections of the bladder plate and bladder neck with the bony pelvis
in a fetus with classic bladder exstrophy. Urology. 2002;60(1):142–6; discussion 146. https://
doi.org/10.1016/s0090- 4295(02)01715- 6. PMID: 12100941.
26. Wild AT, Sponseller PD, Stec AA, Gearhart JP.The role of osteotomy in surgical repair of
bladder exstrophy. Semin Pediatr Surg. 2011;20(2):71–8. https://doi.org/10.1053/j.semped-
surg.2010.12.002. PMID: 21453849.
https://doi.org/10.1016/S0022- 5347(17)66294- 3. PMID: 13514903.
https://doi.org/10.1016/s0022- 5347(17)34912- 1.
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27. Yazici M, Sözübir S, Kilicoglu G, Bernay F, Incesu L, Ariturk E.Three-dimensional anatomy
of the pelvis in bladder exstrophy: description of bone pathology by using three-dimensional
computed tomography and its clinical relevance. J Pediatr Orthop. 1998;18(1):132–5. PMID:
9449115.
28. Yazici M, Kandemir U, Atilla B, Eryilmaz M. Rotational prole of lower extremities in bladder exstrophy patients with unapproximated pelvis: a clinical and radiologic
study in children older than 7 years. J Pediatr Orthop. 1999;19(4):531–5. https://doi.
org/10.1097/00004694- 199907000- 00022. PMID: 10413008.
221

Common Adjuvant Surgeries
inExstrophy/Epispadias Care
ElizabethRoth, TravisGroth, andJohnKryger
Ureteral Reimplantation
Among the many considerations for surgeons at the time of bladder exstrophy repair
is whether or not to perform ureteral reimplantation at the time of initial closure. Due
to the anterior herniation of the bladder in bladder exstrophy, the ureter inserts posteriorly into the bladder and closer to the bladder neck. There is a signicantly shorter
intramural tunnel. VUR is present in a large proportion of bladder exstrophy patients
after initial bladder closure, with estimates of >75% prevalence postoperatively,
Dickson [9]. Recurrent pyelonephritis within this population typically affects 20–50%
of patients in the 90-day postoperative period, even with the use of continuous antibiotic prophylaxis, Braga [3], Grady [14]. In certain surgical approaches, such as YoungDees-Leadbetter bladder neck reconstruction or Kelly radical soft tissue mobilization,
ureteral reimplantation may be required to facilitate reconstruction without undue risk
of ureteral obstruction, Dickson [9], Tourchi [25]. With complete primary repair of
exstrophy (CPRE), there is better bladder urinary cycling due to enhanced sphincter
tone at the bladder neck. However, longer periods of urine holding and bladder pressure can also result in worsening VUR.This results in a pressure pop-off mechanism
for the bladder and can compromise bladder distension during cycling and can result
in worsening hydronephrosis. There may be a theoretical improvement in bladder
cycling from bilateral ureteral reimplant (BUR) to facilitate better bladder distension
during the dry intervals between voiding. Ureteral reimplant has also reduced hydronephrosis that is secondary to VUR.Lastly, correction of VUR will reduce the incidence of UTI.There is some evidence for larger bladder capacity at age 4years in
children closed with a CPRE-BUR combined approach, Ramji [22].
13
E. Roth · T. Groth · J. Kryger (*)
Pediatric Urology, Children’s Wisconsin, Medical College of Wisconsin,
Milwaukee, WI, USA
e-mail: Eroth@childrenswi.org; Tgroth@childrenswi.org; Jkryger@childrenswi.org
© 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_13
223

224
E. Roth et al.
Cephalo-trigonal or cross-trigonal approaches may be considered and should be
driven by the general shape of the bladder plate and which approach will achieve the
most optimal tunnel length. With either technique, the procedure starts by identifying each ureteral orice and cannulating with a small stent or feeding tube (3.5–5
Fr). Each ureteral orice is circumscribed and dissected free of the intramural tunnel. The trajectory of the ureter will be more medial and caudal than in primary
VUR, and there is an increased risk for inadvertent ureteral injury if the altered
trajectory is not appreciated. Once the ureter is adequately freed from the intramural
tunnel, a new trajectory can be plotted.
Cephalo-trigonal Technique The cephalo-trigonal approach to ureteral reimplan-
tation is a bladder exstrophy-specic surgical technique rst described by Canning
etal. [4]. It utilizes the known altered trajectory of the ureter in exstrophy patients.
There is a posterior insertion of the ureter that enters near the bladder neck. It naturally angles in a cephalad direction. The reimplant is further complicated by a very
small bladder. This technique advances the orice cranially. This allows advancement and elongation of the intramural tunnel while preserving the laterality of the
orice and minimizes risk for extravesical kinking of the ureter that would lead to
obstruction.
After dissection of the ureter, a submucosal tunnel is sharply created from the
native entry site in a cranial direction on the ipsilateral side of the bladder (Fig.13.1).
As in other ureteral reimplantation techniques, the tunnel length should ideally be
3–5 times the width of the ureter to ensure optimal resolution of VUR postoperatively. Ideal tunnel length may not be possible with a small bladder plate or with a
bladder plate with modest craniocaudal dimensions. Care must also be exercised to
consider directional shifts of the ureteral trajectory that may occur with corresponding bladder closure. After passing the ureter through the tunnel to the neo-orice,
the ureter is secured to the adjacent mucosa with an absorbable suture in an interrupted fashion. The neo-orice is then assessed by passing the stent through the
reimplanted ureter to ensure a gentle trajectory that passes easily in a retrograde
direction. The process is then repeated with the contralateral ureter.
Advantages of a cephalo-trigonal technique include minimization of extravesical
ureteral obstruction by taking advantage of the unique trajectory of the ureter in
bladder exstrophy and maintaining the laterality of each ureter relative to the midline. Disadvantages include limited tunnel length with a small bladder plate as well
as difculty accessing the ureters cystoscopically through the urethra due to the
cranial location and caudal trajectory of the intramural tunnel.
Cross-trigonal Technique (Cohen) First described as a technique for primary
VUR, the Cohen cross-trigonal technique is also well suited for use in bladder
exstrophy patients, especially in those where the transverse dimensions of the bladder plate exceed the craniocaudal dimensions, Cohen [6]. With this approach, the
ureter is mobilized as above, but the submucosal tunnels are created sharply to
advance the right ureter across the midline to exit on the left and the left ureter

13 Common Adjuvant Surgeries inExstrophy/Epispadias Care
225
Fig. 13.1 Cephalo-trigonal ureteral reimplant
across the midline to exit on the right (Fig.13.2). The surgeon will have to choose
which ureter to place cranially based on the trajectory and size of the ureters overall.
A 1:3–5 ratio of ureteral width to tunnel length is again recommended. The ureteral
orice is secured to the adjacent mucosa at the new site with an absorbable suture.
A small-caliber catheter or stent is again placed retrograde to ensure a smooth trajectory of the ureter. Care must be taken to adequately mobilize the ureter to ensure
that there is a smooth trajectory for the proposed new ureteral hiatus to avoid issues
with extravesical kinking leading to ureteral obstruction. A small segment of the
excess ureter may need to be excised to facilitate a smooth trajectory of the reimplanted ureter. Surgeons must also consider how bladder closure may alter ureteral
trajectory and avoid advancing the tunnel signicantly up the bladder sidewall to
avoid obstruction.

226
Fig. 13.2 Cross-trigonal
ureteral reimplant
E. Roth et al.
Advantages to the Cohen cross-trigonal approach include maximizing tunnel
length in patients with limited craniocaudal bladder plate dimensions, better cystoscopic access to the ureters for future urologic surgical needs (but it can still be
difcult), and familiarity of the technique to most pediatric urologists and surgeons.
Limitations include increased risk for extravesical ureteral obstruction unless familiar with technical modications for exstrophy and frequent need to resect a portion
of distal ureter to ensure an appropriate ureteral trajectory.
Outcomes Multiple surgical groups have published clinical outcomes from blad-
der exstrophy patients who underwent ureteral reimplantation at the time of initial
bladder closure, indicating that it is safe and technically feasible in experienced
hands, Dickson [9], Braga [3], Ramji [22], Garat [13], Jarosz [17]. Documented
clinical benets include decreased incidence of febrile UTI, Braga [3], decreased
renal scar on nuclear scintigraphy, Dickson [9], and decreased need for subsequent
ureteral surgery, Dickson [9], Braga [3], Jarosz [17]. While the rate of VUR is
decreased in reported cohorts, the overall rate of postoperative VUR in exstrophy
patients is signicantly higher than in primary VUR literature (0–40% versus <5%),
Dickson [9], Braga [3], Jarosz [17].
Other proposed benets of early ureteral reimplantation are improved bladder
cycling efciency, potentially leading to the development of greater bladder capacity. The international group based in India noted increased bladder capacity at age
4years in patients who underwent concurrent BUR at initial closure, though this
nding is likely multifactorial, Ramji [22]. In our experience, we have found BUR
at the time of initial closure to be helpful in patients with large bladder plates, both
male and female.
In carefully selected patients, reimplantation can facilitate improved bladder
cycling, protect against recurrent pyelonephritis in the postoperative period, and
possibly improve capacity and potential for volitional voiding. However, this must
be weighed against the additional operative time, increased tissue handling and

13 Common Adjuvant Surgeries inExstrophy/Epispadias Care
227
mobilization required, and additional surgical complexity added to an already complex surgical problem. Thus treating surgeons must individualize the decision on
whether bilateral ureteral reimplantation is warranted on a case-by-case basis.
Inguinal Hernia
It has been well established that classic bladder exstrophy patients have a signicantly higher risk of developing inguinal hernias when compared to the general
population. Connolly [7] The historically reported incidence of inguinal hernias in
classic bladder exstrophy ranges from 56% to 86% in males and 8% to 15% in
females. There have been several theories for this, which include innate weakness
of the abdominal wall, lack of obliquity of the inguinal canal, along with the increase
in intra-abdominal pressure after bladder exstrophy closure, Husmann [16],
Muecke [21].
Several studies have assessed risk factors for inguinal hernias in classic bladder
exstrophy patients. Male gender is a signicant risk factor for developing an inguinal
hernia. Most recently in 2021, Lee etal. reported that 64.2% (34 of 53) of males developed inguinal hernias and only 5.3% of (2 of 38) females developed inguinal hernias
after CPRE in patients with bladder exstrophy, Lee [19]. It has been previously
reported by Lavien etal. that pelvic osteotomy is associated with a decreased incidence of inguinal hernia. In this series, 25% of patients developed an inguinal hernia
if an osteotomy was performed versus 46% if an osteotomy was not performed. In
their study, both female sex and osteotomies were associated with decreased risk for
development of an inguinal hernia along with decreased risk for recurrence. Age at
bladder closure was not associated with hernia formation, Lavien [18].
Inguinal hernias in classic bladder exstrophy patients are at risk for incarceration. Lee etal. assessed the timing of inguinal hernias following CPRE. In their
study, 17% of patients presented with incarcerated hernias. The greatest risk for
inguinal hernia formation was within the rst 6 months following CPRE.They also
noted decreased rates of development of inguinal hernias after 12months of age,
Lee [19]. Ellison et al. reported that simultaneous hernia closure at the time of
CPRE was safe and decreased the risk of future development of inguinal hernias. In
their study, 36% (9 of 25) of patients developed inguinal hernias if not repaired at
the time of closure versus 11% (2 of 18) who developed inguinal hernias if previously repaired at the time of CPRE, Borenstein [2].
The recurrence rate for inguinal hernias is signicantly higher in the bladder
exstrophy population than in the general pediatric population. Previous studies have
demonstrated a recurrence rate of 17–34% following inguinal hernia repair following bladder exstrophy closure versus a 0.3–1.1% recurrence rate in the general pediatric population, Stringer [23], Lavien [18], Borenstein [2], Ein [10].
Several different approaches for inguinal hernia repairs have been described in
bladder exstrophy patients. This includes a pre-peritoneal approach by Connely
etal., which is typically performed at the time of bladder closure and standard open
inguinal approach, Connolly [7], Ellison [11].

228
E. Roth et al.
Monsplasty
Another important adjuvant surgical step is the monsplasty. Patients with bladder
exstrophy experience signicant diastasis of the pubic rami. This results in the
separation of the soft tissues above the pubic rami, which is the mons pubis. Even
after surgical approximation of the pubic rami, there is separation of the mons
pubis in the midline. Often there can be a deep cleft in the pubic midline. This gap
can slowly worsen over time as the bony pelvis grows and the pubic diastasis widens.
It has been especially signicant in the appearance of the female genitalia that
appears more open at the introitus, with a at or concave mons pubis and bid clitoris and clitoral hood. Long-term data have shown that women with exstrophyepispadias complex report dissatisfaction with their genital appearance, Mathews
[20], Woodhouse [27]. The appropriate reconstruction of the mons pubis and clitoral structures can greatly enhance the cosmetic appearance of the genital area for
girls and boys.
After the approximation of the pubic bones at initial closure, then the monsplasty is undertaken, Cook [8]. This can also be performed in subsequent surgeries at any age. If it is being performed at a later stage of surgery, then the rst step
is to excise the scar in the midline along its length and excise the non-hair-bearing
skin. This will create the natural appearance of the escutcheon at nal closure.
The lipoma of the mons pubis is then mobilized from each side of the pubis by
dissecting the cephalad and lateral and anterior edges until each lipoma can be
freely mobilized to the midline. The lipomas are approximated in the midline with
layers of 4–0 or 5–0 absorbable interrupted sutures. This creates one central mons
above the pubic bones. Sometimes they are sufciently generous in size to allow
them to overlap in the midline and further reduce future separation. The skin
edges are excised as much as tension allows to remove non-hair-bearing skin to
create the natural appearance of the escutcheon. The skin is approximated cosmetically with absorbable sutures and often Steri-Strips to reduce tension on the
skin edges during healing.
Prior to completing the skin closure of the mons, the clitoral hood is reconstructed. The clitoral bodies can be approximated in the midline, and the medial
edges of the glans clitoris can be de-epithelialized and approximated with ne
absorbable monolament sutures. But we often do not perform direct approximation of the clitoral bodies on newborn closure to avoid neurovascular injury. Then
each clitoral hood can be easily approximated in the midline to enhance the cosmetic natural appearance of the genitalia. The technique described by Grady is a
vertical z-plasty, Weiss [26]. Each hemi-clitoral hood is incised along it’s medial
edge to create two layers. The inferior layers are approximated in the midline with
ne absorbable monolament suture to form the posterior layer of the clitoral hood.
Then the superior layers are approximated along their medial edges.
These steps to enhance the mons pubis and clitoral bodies are rather easy steps
that add considerable improvement to the pubo-genital appearance.

13 Common Adjuvant Surgeries inExstrophy/Epispadias Care
229
Umbilicoplasty
Due to the anterior abdominal wall defect in bladder exstrophy, affected children are
born with an abnormally low-set umbilical cord at the insertion of the urachus on
the exposed bladder dome, rather than the typical location approximately 2/3 of the
way between the xiphoid and the pubic symphysis. The umbilicus plays an important aesthetic landmark on the abdomen. The surgical reconstruction of a neoumbilicus should be performed in conjunction with other exstrophy surgeries to create an
orthotopic neoumbilicus.
A variety of techniques have been described, Sumfest [24], Cervellione [5],
Feyaerts [12], Barroso [1], and Hanna [15]. The site of the neoumbilicus is selected
by tracing a line across the anterior abdominal wall between the anterior superior
iliac crests. The neoumbilicus is centered on this axis in the midline. Next, a ap or
free graft of adjacent skin is selected. The area under the graft or ap is defatted
down to the anterior abdominal fascia, and the skin is secured in this location to
form an indented neoumbilicus. Three examples of neo-umbilicoplasty (NU) techniques are diagrammed below: Free graft, spiral-ap (S-ap), and rhomboid ap.
We conducted an unpublished patient satisfaction survey of our MIBEC patients
who were at least 3years postoperative from umbilicoplasty. The preference was for
free graft NU (Figs.13.3, 13.4, 13.5, 13.6, 13.7, 13.8, and 13.9).
Fig. 13.3 Free Graft
NU.A circular graft is
harvested from the
intended neo-umbilical site
which is located at the
intersection of the
midsagittal line and the
level of the iliac crest.
After the free graft and
underlying fascia have
been defatted, the free graft
is centrally secured to the
exposed fascia with
permanent or absorbable
monolament
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