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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

Exstrophy asaGlobal Health Concern:
Strategies forAddressing theBurden
19
ofDisease
KatelynA.Spencer, AseemR.Shukla, andRakeshS.Joshi
Global Health, Global Surgery, andTheir Role Within Treating
Bladder Exstrophy
The World Health Organization (WHO) has dened health to be “a state of complete physical, mental, and social well-being, and not merely the absence of disease
or inrmity” since the advent of the organization in 1948 [1]. This denition pushed
health beyond the binary of solely sick versus well and enshrined the idea that health
is a state which can be promoted. This opened the door for global health advocates
to work to improve the health of people around the world and across dened country
borders.
Global health is a concept that has changed in its denition and scope over the
years. It is most commonly dened as “an area for study, research, and practice
that places a priority on improving health and achieving equity in health for all
people worldwide” [2]. Of note, global health is distinctly different from that of
public health or international health according to current denitions. Public health
has traditionally been constrained by national borders, while global health focuses
on issues that transcend borders. Additionally, conversely to international health,
K. A. Spencer . A. R. Shukla
Children’s Hospital of Philadelphia, Philadelphia, PA, USA
Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
e-mail: ShuklaA@chop.edu
R. S. Joshi (
Department of Paediatric Surgery, Ahmedabad 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_19
*)
313

314
K. A. Spencer et al.
which was initially developed through the lens of colonialism with knowledge,
services, and interventions exclusively coming from high-income countries
(HICs) to low- and middle-income countries (LMICs), global health attempts to
shift the power imbalance. It aims to promote partnership or pooling of expertise
between high-, middle-, and low-income countries in addition to reducing health
inequity by advocating for social equity. Global health also works to include population-level prevention without neglecting the importance of individual-level
clinical care through multidisciplinary strategies that draw on health and social
sciences in addition to elds like engineering, urban planning, climate science,
and others.
Despite global health being a large eld that encompasses many domains, surgery has not always been considered a primary element [3]. As the eld has traditionally been dened by specic populations (i.e., mothers, children, etc.) and
diagnoses (i.e., HIV), surgery is a hard entity to fund, as it intersects all patient
populations and includes a variety of disease pathologies. There has been a more
recent push to dene global surgery and include it within the umbrella of global
health [4]. This includes supporters of the addition such as Jim Kim, the 12th
President of the World Bank, who has stated that “surgery is an indivisible, indispensable part of health care and of progress toward universal health coverage” [5].
The addition of surgery within the eld is especially important since common
surgical procedures are cost-effective and comparable to other widely accepted
global health interventions (i.e., antiretroviral therapy) when performed in
LMICs [6].
There is a large burden of surgical disease worldwide and a disproportionate
allocation of resources currently. Three-fourths of all surgeries take place in the
wealthiest third of the world [7]. As a result, there are two billion people who do not
have access to surgical care [8]. This is all due to differing levels of resource availability and capacity. For example, HICs have 10 times as many operating rooms [8]
and 11 times as many trained surgeons [9] per person as LMICs.
Conversely, the burden of disability-adjusted life-years from surgical conditions
is concentrated within LMICs [10]. Approximately 94% of complex congenital
anomalies that require surgical intervention occur in LMICs [11]. When specically
examining genitourinary conditions, they are incredibly common [12]. They are ve
to ten times more likely than cleft lip and/or palate and are the third most common
non-chromosomal anomaly behind congenital heart and limb defects. While rare
compared to other genitourinary anomalies, bladder exstrophy epispadias complex
(BE) occurs in 1in 50,000 live births [13]. As such, it can account for a signicant
number of cases, especially in places with higher birthrates. It is a common trend
within LMICs to have higher birth rates than HICs, and this can contribute to an
increased burden of disease [14]. Global surgery endeavors to treat conditions like
BE and should not be viewed as too costly [6] or complex because the burden of
disease within these countries is signicant. Outside of cost, from a moral standpoint, there is an obligation that exists to provide the highest level of care possible
for these children and their families.

19 Exstrophy as a Global Health Concern: Strategies for Addressing the Burden…
315
Unique Challenges ofBladder Exstrophy inLMICs
Outside of the standard challenges of surgical conditions within LMICs, including
lack of access to care, decreased resource availability, etc., BE provides unique
challenges to patients and providers designing interventions to treat this population.
Delay inDiagnosis andClosure
BE is primarily diagnosed prenatally via ultrasound in HICs. As a result, parents can
be counseled on what to expect, and surgical planning about closure can be done
before delivery. However, access to and equity of prenatal care within and between
countries impacts early detection of BE [15, 16]. In LMICs there is less access to
prenatal ultrasounds, and so, less of these children are diagnosed prior to delivery.
This then creates a delay in closure because surgical planning is delayed.
Additionally, due to the rare nature of BE, diagnosis can be further delayed as parents are sent through the referral network until they nally reach a physician with
knowledge of the condition. Then, there is further time required for parents to reach
a physician who can perform the closure operation.
Delayed closure for BE also comes with its own challenges. Closing children
with BE at older ages, which is common in LMICs because of all of the reasons
listed above, is associated with a higher failure rate [17], greater challenges with
continence, and the need for repeat closures and other operations throughout the
lifetime of the child [18]. All of these challenges contribute to worsening the
sequelae of mental health challenges that come with BE.
Complex Level ofMedical andSurgical Care Required
forBladder Exstrophy
Treating BE successfully requires a high degree of technical skill and experience.
While the timing and type of closure for BE remain a debated topic among pediatric
urologists [19], there is denitive support that well-performed initial closures are
more successful for patients than redo closures after a failed initial attempt [18]. In
order to achieve this, the initial closure should be performed by well-trained urologists with the assistance of orthopedic surgeons. This creates a challenge in LMICs,
where the access to and availability of highly trained surgeons is low in general.
There is a high rate of failure when less experienced surgeons attempt to perform
the initial closure, and this creates future challenges if the child needs a repeat procedure due to the presence of scar tissue and altered anatomy.
Additionally, these children need dedicated and specic postoperative care and
long-term follow-up to facilitate a successful closure and good quality of life. This
includes having well-trained nurses postoperatively who are familiar with the
unique needs of these children. These children will also need continued follow-up

316
K. A. Spencer et al.
care to monitor their long-term outcomes, including continence, renal function, fertility, and sexual function. All of this is complicated in the LMIC setting, where
access to care and medical specialties is limited.
Mental Health Concerns
Children with BE, irrespective of their location, are at a dramatically increased risk
of psychosocial adjustment issues, including suicidal ideation and behaviors,
depression, and anxiety compared to their anatomically intact peers [20–25]. This is
complicated by the fact that children in LMICs have less access to mental health
services [26].
The mental health concerns of this population also extend to the caregivers of
these children. It has been documented that parents of children with BE have stress
levels comparable to the parents of children with other long-term diagnoses such as
type one diabetes [27]. Parents, in general, are concerned about the long-term
impacts of BE, including helping with hygiene and uncertainty about their child’s
future [28]. Primarily these parents are concerned about their child’s ability to get
married, have children, achieve independence, and obtain complete continence, and
these worries increase with subsequent surgeries.
Mental health within LMICs for this patient population is uniquely complicated
by the cultural traditions and beliefs surrounding religion within those countries.
Specically, in many LMICs around the world, there is an increased rate of multigenerational housing [29], and living within close quarters creates challenges for
families with children with BE due to the constant leakage of urine. This can contribute to perceived and experienced stigma from family, school classmates, and
community members [28]. All of this is compounded by lower nancial resources
for disposable diapers, mental health services, etc., While some of these challenges
are not unique to the LMIC setting, they can compound to create different challenges for patients and their caregivers that must be understood and addressed
within interventions created to address this need.
Strategies toDesign aGlobal Surgery Collaboration
In order for a global surgery collaboration to be successful, it must address the challenges the specic population of interest has holistically and treat all aspects of the
disease [4]. The focus cannot just be on creating anatomic integrity and then leaving, as might be seen within many “short-term surgical missions.” These programs,
which have become common within the eld of global surgery, are typically focused
on increasing surgical volume over a short timeframe and do not always lead to
improved surgical capacity for host institutions [30]. They are not designed to cultivate long-term follow-up for patients and have been known to result in higher complication rates that are outside of the local health system’s capacity to address [31].
Furthermore, when volunteer teams are not well integrated with the local hosts, they

19 Exstrophy as a Global Health Concern: Strategies for Addressing the Burden…
317
can burden local providers, stress hospital resources, and create negative opinions of
HIC volunteers [32]. While conceived and executed with the best of intentions,
these global surgery programs can cause more harm than good for local
populations.
In response to these programs, the American Surgical Association Working
Group (ASAWG) on Academic Global Surgery created a set of guiding principles
for ethical engagement within global surgery. They are as follows [10]:
1. Local priority: The interests of the local partners and patients should take prece-
dence in all decision-making.
2. Resources: Programs should not hinder existing infrastructure, and obtained
HIC resources should be focused on the host site and partners.
3. Clinical care: HIC surgeons and staff should create a plan for complication man-
agement and long-term follow-up.
4. Capacity building: Training should be applicable to the LMIC setting in which
host partners will function, focus on local as opposed to visiting surgeons, and
not contribute to out-migration.
5. Research: Research should be mutually collaborative and equitably attribute
authorship.
6. General: All HIC partners should practice cultural sensitivity and uphold the
standards of care and practice that exist in their HIC.
The Ahmedabad Model ofExcellence
Successful global surgery collaborations employ the above strategies and put an
emphasis on capacity building. An example of a robust global surgery collaboration, irrespective of disease of interest, is the International Bladder Exstrophy
Consortium (IBEC) based in Ahmedabad, Gujarat, India. The program began in
2009 when surgeons from two academic research centers in the United States
joined with the Department of Pediatric Surgery at the Civil Hospital and BJ
Medical College in Ahmedabad, Gujarat, India, to form a multi-institutional collaboration. Since then, the partnership has grown to include the Children’s Hospital
of Philadelphia, Cincinnati Children’s Hospital, Seattle Children’s Hospital, surgeons from the Hospital for Sick Children in Toronto, Canada, and a pediatric
anesthesiologist specialized in pediatric pain management. The Civil Hospital, a
government-nanced public hospital offering free medical care to a primary catchment area of 60 million people, serves as the host institution. The collaboration
includes at least one annual visit from the same team of surgeons, during which
new patients are evaluated and care plans are made, while all previous patients are
recalled for long-term follow-up. Patients undergo yearly urodynamic evaluation,
cystogram, ultrasound, DMSA scan, and bloodwork. The collaboration also
evolved toward a more holistic approach to care, including mental health screenings, educational sessions with family members, and community-building activities for the entire cohort.

318
The IBEC is an example of successfully putting the criteria outlined by the
ASAWG into practice in order to exact long-term benets for patients. The following sections will outline the unique benets of the collaboration and how they have
been implemented according to the ASAWG principles.
K. A. Spencer et al.
Local Priority
The IBEC has a conscious focus on maintaining local priority throughout all aspects
of the collaboration. The coordinators from both the host and visiting teams frequently discuss the priorities of local partners throughout the planning process. This
includes focusing on the host team’s goals and adjusting the collaboration so it best
serves the local patient cohort. For example, the rst few years of the collaboration
identied a greater need for patient education, so the collaboration added novel
education initiatives that included a doll representative of a BE patient to better
explain to patients, their caregivers, and nursing staff what they should expect after
surgery [33].
A gap in care and support for the caregivers was also identied, and research
endeavors were added to focus on the patients’ quality of life and strategies to best
support them [28]. Patient- and family-centered educational lectures were strengthened based on the gathered data, and social workers and psychologists were added
to the group to offer support. Patients and families who attend the collaboration
annually can gain several ancillary psychosocial benets, including group support
through a family-coordinated WhatsApp group, one-on-one counseling, and social
resources.
Resources
The collaboration has focused on leveraging the existing infrastructure to provide
more care to a greater number of patients as the geographic catchment area of the
collaboration has increased [34]. Collaboration funding is prioritized for the host
institution’s needs, including purchasing necessary resources for the collaboration
and providing services to patients. Importantly, funds are not overwhelmingly dedicated to travel for the visiting team.
Clinical Care
The clinical care plan for complication management for the collaboration was
devised together with the host and visiting providers and is based on pathways produced over several years [17]. The clinical care aspect of the collaboration is centered on an annual commitment of the primary surgeons from HICs to return for
patient follow-up. Additionally, all interval follow-up care is completed by the host

19 Exstrophy as a Global Health Concern: Strategies for Addressing the Burden…
319
team with support from the teams from HIC through regular teleconference
communication.
Long-term follow-up is an essential element of the collaboration to create the
continuity of care required to treat such a complex condition. Specically for BE,
consistent follow-up is required to ensure that the long-term consequences of even
successful BE closures are captured within data and addressed either through medical or surgical management. For many global surgery programs, including shortterm surgical missions, there is little data on long-term outcomes because there is
not a large emphasis placed on coordinating follow-up [35]. As a result, these programs are hard to evaluate. Previous work has found that the postoperative followup for many of these programs typically stops after 6months. For the small number
of programs that do follow patients for longer than 6 months, their follow-up rate is
on average 56% with a 22.3% complication rate [35]. Conversely, the IBEC has
continued to follow patients years after their original procedure and has a consistent
follow-up rate of 76% [17]. This is despite the various challenges within the LMIC
setting.
This is a testament to the original inception of the IBEC.It was prospectively
constructed to include a long-term commitment between the host and visiting surgeons. The collaboration includes rigorous preoperative counseling and setting
expectations of care with patients to ensure that they not only return for follow-up
but also undergo a comprehensive examination.
Capacity Building
All aspects of the collaboration are committed to capacity building. All repairs are
performed with at least one resident of the host institution participating in the surgery. Emphasis is placed on knowledge sharing between host and visiting stakeholders, with a focus on training local residents and attending physicians to create
autonomous surgeons that have the capacity to perform the CPRE with the same
outcomes as the teams from the HIC academic research centers. As a result, the
host team has increased experience and greater competency in performing a
CPRE.Over the last 5years, almost as many closures have been performed outside
of the time of annual collaboration as during the set window, with comparable
results. This is the ultimate showcasing of capacity building, as the host institution
is now able to independently provide an extremely high level of care for this specic patient population.
Also, essential to capacity building for the IBEC was the selection of the host
institution that had the ability to house the collaboration and expand with it as it
grew. The IBEC purposefully selected the second-largest hospital in Asia to partner
with. Civil Hospital has a pediatric surgery department that oversees 100 beds and
performs 2500–3000 surgical procedures per year. When selecting a host institution, Civil Hospital had demonstrated through these parameters that it possessed the
capacity to care for postoperative patients with BE.

320
K. A. Spencer et al.
Research
The collaboration has been focused on research and, as such, has produced several
collaborative manuscripts [17, 28, 34, 36–38]. Each of these projects has equitably
credited host partners with rst, co-rst, or corresponding authorship.
General Principles
Cultural sensitivity is of primary importance for the collaboration, so local partners
lead all patient interactions, and all visiting partners are educated on cultural sensitivity prior to attending the collaboration.
Equally as important is the commitment to providing care to the IBEC cohort of
patients that is equivalent to the standard of care within the visiting surgeons’ home
institutions. The IBEC has reported outcomes that are comparable with those seen
in HICs [17].
Overall, the IBEC offers a model for a sustainable, long-term global surgery collaboration. It has generated positive, long-term benets for local partners and
patients with surgical outcomes on par with HICs. By focusing on surgical capacity
over volume, the collaboration’s scope has widened to increase the geographic
catchment area for patients [34]. But, like all things in the eld of global health,
there is always more work to be done to address gaps in care. Even well-designed
interventions, like the IBEC, are still trying to address the unique challenges of BE
to bring earlier referrals, operate on children at younger ages, and provide strategies
to make the collaboration even more equitable for individuals of all social classes
within the region [34].
Despite the continued work that is ongoing, the IBEC offers a template for global
surgery collaboration when focused on addressing the burden of BE.Such projects
must embody the principles of the ASAWG and should push the eld forward by
maintaining its commitment to a sustainable, long-term collaboration. While this
specic program deploys a large volume of resources for a rare disease such as BE,
it serves as a template for other pathologies. BE, being a global genitourinary deformity, mastery of the procedure enables downstream expertise in other genitourinary
reconstructive procedures such as hernia repairs, reimplantation of ureters, hypospadias, bladder neck reconstruction, and more. By using BE and the IBEC as a
model for global collaboration, we submit that there are a multitude of benets for
many more children with less rare conditions.
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