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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5514_Библиотеки_им_академика_М_И_Перельмана.pdf
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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

8 The Kelly Procedure
135
from the venous plexuses at the base of the prostate, which is easily controlled with
bipolar cautery.
Tension-Free Bladder Neck Construction
The bladder is opened in the midline proximally from the urethral neo-meatus. The
ureteric orices are cannulated with a 4 or 6 Fr feeding tubes, and if required a
Cohen type ureteric reimplantation is performed. The location for the construction
of the bladder neck is just proximal to the verumontanum, an area that exhibits
characteristic longitudinal linear folds (Fig.8.5). Triangles of bladder mucosa are
marked and removed, and a 1- or 2-layer tension-free orthotopic bladder neck
reconstruction is performed over an 8 Fr silicone stent with interrupted 4/0 Monocryl
sutures. We do not use a measure of the width of the remaining urethral plate, nor
do we measure the nal circumference of the reconstructed bladder neck. Instead,
we use our judgement and experience to aim that rst layer of sutures captures an
adequate amount of detrusor muscle to ensure that the urethral stent can be moved
with a degree of resistance. The proximal end of the catheter is secured within the
bladder with a 6/0 Monocryl suture. A 10 or 12 Fr suprapubic catheter is inserted
into the bladder for postoperative drainage. The bladder is closed with a single layer
with interrupted 4/0 Monocryl sutures. Ureteric stents, when used, can be brought
out through the midline closure or laterally through separate puncture sites.
Fig. 8.5 Operative image
showing orthotopic
location of bladder neck
construction, with marked
mucosal triangles for
excision

136
Fig. 8.6 Original hand
drawing by Justin Kelly
showing the IPGAM
procedure
N. S. Johal et al.
Urethral Reconstruction andGlans Closure
The urethral plate is tubularised with interrupted 5/0 or 6/0 PDS sutures on the existing 8Fr silicone urethral stent. An IPGAM procedure is performed distally to form
a more ventrally placed urethral meatus (Fig.8.6). Triangles of mucosa are excised
from the dorsal aspect of the glans, lateral to the central urethral plate. The glans is
reconstructed over the urethra with 1 or 2 layers of interrupted 6/0 PDS sutures. The
urethra is retracted ventrally, and the corporal bodies are approximated in the midline with 1 or 2 layers of interrupted 5/0 PDS sutures, either with or without a rotation of the corpora to correct the dorsal chordee.
Wound Closure andPenile Skin Reconstruction
The rectus muscle closed in layers commencing with interrupted 2/0 Vicryl for the
rectus muscle and 0 or 1 Vicryl for symphyseal region. The skin is closed in 2 layers, with Vicryl for the subcutaneous fat and Monocryl for skin. An umbilicoplasty
can be performed at the skin closure stage if requested by the family. Skin coverage
of the penis is always a challenge, and excision of the dartos tissue from the penile

8 The Kelly Procedure
skin can facilitate this step. Anchoring sutures between the dartos tissue and the
base of the corpora can enhance the visible penile length. The urethral stent is
secured to the glans penis with 4/0 Prolene. A compression dressing is placed
around the reconstructed penis.
137
Kelly Procedure inFemales (Video 2)
The basic tenets of the procedure are the same as in the male patient. The incision
continues inferiorly to expose both the clitoral corporal bodies leaving intact the
labia minora skin aps. This enables subsequent clitoral hood skin reconstruction
following the bladder neck and urethral reconstruction. As in the male, the pelvic
muscular oor is exposed in the extraperitoneal plane to access the neurovascular
bundle, the obturator internus muscle and the superior aspect of the levator ani
below and lateral to the vagina. Each clitoral body is separated from the surrounding
fat of the labium majus. As in the male, the clitoral bodies can be detached from the
ischiopubic ramus with identication and mobilisation of the pudendal neurovascular bundles. However, this step is not always necessary and is determined by the
degree of pubic diastasis, which if signicant can hinder a tension free bladder neck
construction and approximation of the clitoral bodies. After the bladder neck is
reconstructed, the muscle of the urogenital diaphragm below and lateral to the
vagina can be used as a wrap over the bladder neck and urethral repair. The abdomen is closed as in the male. The mons pubis is constructed by approximation of the
subcutaneous fat from either side to achieve a good cosmetic appearance.
Postoperative Management
Postoperatively the patient can return to the in-patient ward and can commence
feeds almost immediately. Postoperative analgesia is delivered via an indwelling
epidural catheter for the initial 4–5days, which we have previously shown results in
a reduced hospitalisation following primary closure [5]. Broad spectrum intravenous antibiotics such as co-amoxicav are continued for 48h and then converted to
an oral dose for a total of 7 days after which prophylactic dose antibiotics are
administered. If the patient has undergone ureteric reimplantation, the ureteric catheters are removed after 7days. The penile compression dressing is removed on day
7, the urethral stent on day 14 and the suprapubic catheter after day 21 once effective urethral voiding is established.
Complications andOutcomes
In our experience, major complications following a Kelly procedure are rare. Glans
ischaemia and atrophy are widely muted as a potential complication, supposedly
related to the mobilisation of the pudendal neurovascular bundle. Over the past

138
N. S. Johal et al.
20years, the exstrophy team has performed in excess of 400 Kelly procedures and
has witnessed hemi-glans atrophy in 2 patients. Less serious and more common
complications would include urethrocutaneous stula formation, penile skin dehiscence, infrapubic incisional hernia and urethral meatal stenosis. Factors responsible
for good outcomes are a high volume of cases (related to centralisation of BEX
surgery to 2 centres in United Kingdom), collaborative working arrangements, specialist anaesthesia and postoperative analgesia, and highly experienced specialist
urology nursing care.
Our institution made a conscious decision 25years ago to seek an alternative
surgical strategy for children with BEX, having concluded that the staged approach
of neonatal bladder closure, Cantwell-Ransley epispadias repair and Young-Dees
bladder neck reconstruction was not conferring good outcomes. The Kelly procedure was introduced and became the operation of choice for all BEX cases within a
few years and has remained so to this day. We are highly satised with the improved
penile cosmetic outcomes, reduced bladder augmentation rates and a signicant
increase in the number of patients who can achieve spontaneous urethral voiding
without the need for continence pads or underwear. We are required to submit
annual outcome data to the national funding organisation for highly specialised services, such as BEX care, and the number of children who are dry by day and voiding
per urethra at 5years of age is 50–60%. We know that this gure increases with age
and specically with biofeedback therapy, which commences at around 8years of
age. We remain condent that the decision we made to adopt the Kelly procedure
for our BEX patients was correct and that it has provided our cohort of patients with
improved outcomes.
It is wrong in our view to simplify urinary continence in terms of dryness by day
and night, because in BEX patients it is a much more complex issue, that should
take into account voiding intervals and uid intake. There is a wide diversity of
phenotypes in BEX, and outcomes are unpredictable and at times unexpected. To
compare the outcomes of BEX surgeries and institutions by means of urinary continence is divisive and overlooks many other health outcome measures, which are
equally and, in some cases, more important.
The Kelly procedure is not complex, but does require a high surgical skillset,
courage and a large cohort of patients to become procient. We are condent that
the centralisation of BEX care that we have witnessed in the United Kingdom and
that is now underway with the establishment of consortia in the United States will
continue to enhance our understanding of what are the right goals and aspirations in
the management of BEX.

8 The Kelly Procedure
139
References
1. Siffel C, Correa A, Amar E, Bakker MK, Bermejo-Sanchez E, Bianca S, Castilla EE, Clementi
M, Cocchi G, Csaky-Szunyogh M.Bladder exstrophy: an epidemiologic study from the inter-
national clearinghouse for birth defects surveillance and research, and an overview of the lit-
erature. Am J Med Genet. 2011;157:321–32.
2. Burki T, Hamid R, Duffy P, Ransley P, Wilcox D, Mushtaq I.Long-term followup of patients after
redo bladder neck reconstruction for bladder exstrophy complex. J Urol. 2006;176(3):1138–41.
discussion 1141–2
3. Kelley JH, Eraklis AJ.A procedure for lengthening the phallus in boys with exstrophy of the
bladder. J Pediatr Surg. 1971;6(5):645–9.
4. Kelly JH, Taghavi K, Mushtaq I, Justin H.Kelly and his procedure for bladder exstrophy and
epispadias. J Pediatr Surg. 2022;57(2):314–21.
5. Mushtaq I, Garriboli M, Smeulders N, Cherian A, Desai D, Eaton S, Duffy P, Cuckow P.Primary
bladder exstrophy closure in neonates: challenging the traditions. J Urol. 2014;191(1):193–7.

Scrotoperineal Approach toBladder
Exstrophy Repair
S.N.Kureel, ArchikaGupta, andKanoujiaSunil
Introduction
Bladder exstrophy is one of the most serious urological birth defects involving
the lower abdominal wall, bladder, urethra, and penis with certain associated
anomalies and mostly normal upper tracts. The classic description of this birth
defect was given by O’Neill as “if one blade of a pair of scissors were passed
through the urethra of a normal person; the other blade was used to cut through
the skin, abdominal wall, anterior wall of the bladder and urethra, and the symphysis pubis; and the cut edges were then folded laterally as if the pages of a
book were being opened” [1] (Fig.9.1). Trendelenburg in 1906 described that
despite the severity of the defect, all neurophysiological components necessary
for continence are present in exstrophy [2]. Therefore, any strategy/approach for
reconstruction must aim to create a low-pressure reservoir of adequate capacity,
create an outlet, reconstruct the bladder neck with internal sphincter, and reconstruct the striated muscle of the external sphincter with preserved innervation.
While aiming for satisfactory continence, it is of paramount importance that
upper tracts do not suffer any damage from high pressure or the inevitable vesicoureteric reux. Reconstruction of the outlet must be aimed at creating dynamic
resistance with capacity to relax on command of the pontine micturition center
and remain contracted in the lling phase to avert leakage of urine (Fig.9.2).
Therefore, outlet reconstruction should be accomplished such that innervation
9
Supplementary Information The online version contains supplementary material available at
https://doi.org/10.1007/978- 3- 031- 91238- 2_9.
S. N. Kureel (*) · A. Gupta
Department of Pediatric Surgery, King Geroge’s Medical University, Lucknow, India
K. Sunil
Chandan Institute of Pediatric and Neonatology, Lucknow, 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_9
141

142
Fig. 9.1 Classic exstrophy
bladder (male) showing
open bladder plate with
epispadias with preputial
hood on ventral aspect of
glans penis
S. N. Kureel et al.
remains preserved. Other desirable goals are preservation of the cavernosal
nerve and an adequately sized phallus (Fig.9.3). The technique of radical corporal detachment for penile lengthening must ensure absolute preservation of
the neurovascular bundle of corpora and vessels supplying the glans.
The task of bridging the abdominal wall defect is achieved by bringing the recti
toward the midline after doing the osteotomy [3–5]. The aesthetic appearance is
further enhanced with umbilicoplasty, abdominoplasty, and penile skin coverage
(Fig.9.3).
The midline scrotoperineal approach of bladder exstrophy reconstruction with
innervation-preserving sphincteroplasty and radical corporal detachment realizes
all the aforementioned goals [6–8].

9 Scrotoperineal Approach toBladder Exstrophy Repair
Fig. 9.2 Schematic
diagram to show salient
points of normal
micturition. The lling
phase under control of
sympathetic (red) and
emptying under control of
parasympathetic (green). It
is controlled by paracentral
lobule of cerebral cortex
through sacral micturition
center (S2, 3, 4)
143
A brief review of the relevance of surgical anatomy, especially in the context of
bladder exstrophy here, would be useful in understanding the technical nuances of
the procedure. This would include:
1. Anatomy of the bony pelvis
2. Anatomy of the urogenital diaphragm in exstrophy
3. Anatomy of fascial planes and vessels in the epispadiac penis
4. Relevant anatomical details of open bladder-plate

144
Fig. 9.3 Appearance after
reconstruction of bladder
exstrophy showing
adequate-sized phallus,
umbilicus, and wellaligned lower abdominal
skin crease
S. N. Kureel et al.
Anatomy ofBony Pelvis
The bony conguration of the pelvis in bladder exstrophy has been described by
Sponseller and associates using computerized tomography (CT) pelvis and threedimensional reconstruction [9]. In a cross-sectional view of the bony pelvis at the
level of acetabulum and triradiate cartilage, the intertriradiate distance is the length
of an intertriradiate line joining the triradiate cartilage of the right and left sides
across the pelvic cavity. This line also divides the pelvis into anterior and posterior
segments (Fig.9.4). There has been shown to be a mean external rotation of 12
degrees in the posterior pelvis on each side, retroversion of the acetabulum, a 31%
increase in intertriradiate distance, a mean 18° external rotation of the anterior pelvis, and a 30% shortening of superior pubic rami compared to the control population. This shortening of superior pubic rami along with external rotation of the
pelvis contributes to a varying degree of diastasis of the pubic symphysis. Our study
on diastasis of the pubic bone in patients with bladder exstrophy has demonstrated
that the shape of the gap between the ischiopubic rami, where the urogenital diaphragm is located, varies from rectangular to trapezoid [10] (Fig.9.5a, b). Stec etal.
have described that the sacrum in exstrophy patients has a 42.6% larger volume and
23% more surface area compared to the control population [11].

9 Scrotoperineal Approach toBladder Exstrophy Repair
145
12˚
Fig. 9.4 Shows the magnitude of external rotation of pelvis, retroversion of acetabulum, increased
intertriradiate distance, and external rotation of anterior pelvis with shortened superior pubic rami.
Abnormal anatomy (yellow) is superimposed with normal anatomy of cross-section of pelvis
(ochre yellow)
Fig. 9.5 (a) Three-
dimensional image of CT
pelvis perineal view
showing rectangular shape
of space between
ischiopubic rami. I—
medial and lowermost
point of ischial tuberosity;
P—Superior and
medialmost point of pubic
bone. (The cartilaginous
segment of ischiopubic
ramus is not ossied.). (b)
Three-dimensional image
of CT pelvis perineal view
showing trapezoid shape of
space between ischiopubic
rami. I—Medial and
lowermost point of ischial
tuberosity; P—Superior
and medialmost point of
pubic bone
30%
31%
a
b
Anatomy ofUrogenital Diaphragm inBladder Exstrophy
It has been conclusively demonstrated that all the normal muscles of the urogenital
diaphragm are present in patients with bladder exstrophy [12]. Normally, the urogenital diaphragm between bilateral ischiopubic rami and the base of the urogenital
triangle is triangular. However, in bladder exstrophy, the urogenital diaphragm may
be trapezoid or rectangular depending upon the degree of pubic diastasis [10]. The
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