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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

21 Surgical Options forRefractory Urinary Incontinence inClassic Bladder Exstrophy
343
the patients, 15 already had undergone AC, 12 had undergone a Mitrofanoff procedure and 12 had been treated with BNI in an attempt to achieve continence. Four
patients had undergone more than 1 bladder neck procedure. Postoperatively 28
patients were using CIC to empty the bladder (5 per urethra, 23 via Mitrofanoff).
Although redo BNR can render a signicant number of patients dry, it was only
effective if performed in conjunction with AC.Ashraf Hafez of Mansoura, Egypt,
described his experience with the Mitchell BNR and detrusor wraparound aps in 28
children with CBE [68]. His manuscript was described as mini-trigonal tubularization and bladder neck wraparound with a demucosalized detrusor ap. With a mean
follow-up of 2.5years, all 13 patients with augment on CIC via reconstructed outlets
were continent which was signicantly higher than the non-augmented cohort.
Complete dryness was achieved in 20 (71%) of 28 patients.
The use of non-detrusor muscular aps as an additional substrate for coaptation/
compression of the bladder outlet with or without BNR has been described. The
pyramidalis, rectus abdominis, and gracilis muscles have been harvested as wraparound aps of reconstructed bladder outlet to augment resistance and improve continence [69–71].
Bladder Neck Closure
Persistent incontinence after BNR as described can be challenging to achieve urinary continence. Repeat BNR may denervate the posterior urethra and distal trigone, and ultimately impair any potential for spontaneous voiding. In patients who
have undergone multiple surgical procedures involving the bladder neck with
intractable urinary incontinence, the muscle quality may be questionable or extensive scarring may exist. This clinical scenario does not lend itself to reconstructive
efforts. BNC or transection may be the singular way to achieve urethral continence
albeit at the expense of alternative bladder drainage in the form of CIC or urostomy.
Patient selection is paramount as with any lower urinary tract reconstruction but
may be magnied as BNC is most frequently paired with the creation of a CCC
using the Mitrofanoff principle. Compliance with CIC is critical to avoidance of
bladder perforation and upper urinary tract preservation.
Standard BNC is accomplished by approximating the anteroposterior bladder
neck edges in two layers. The distal urethra may or may not be closed, and omentum
or a rectus muscle ap is usually interposed between the closed bladder neck and
urethra [72–74]. Despite these measures, BNC is often associated with such complications as leakage or stula. Khoury etal. described a modication of the standard
technique of BNC that involves extensive mobilization of the posterior edge of the
bladder neck, elevating it at least 2cm. off of the vagina or rectum in female and
male patients, respectively [72]. This free edge of the posterior bladder neck is
rolled anteriorly and, hence, the anastomosis is placed anterior and not directly
inferior. When concomitant enterocystoplasty is performed, the anterior suture line
is begun in the midline by suturing the edge of the detubularized and recongured
bowel plate to the anteriorly rolled edge of the bladder base.

344
B. A. VanderBrink
Many series of BNC have been reported with success rates of 40–100% [72,
75–81]. Bladder perforation is the most feared complication because of inadequate
drainage and excessive pressures but does not seem to occur at higher rates than
BNR.Nguyen and Baskin reported high calculus formation and stula rates (40%)
in 20 patients (n=12 CBE patients) who underwent AC and BNC, largely attributable to poor compliance with CIC [78]. They also found that incomplete division of
the bladder neck portended stula formation. At 3months after surgery, 40% of the
patients were completely dry, 20% had leakage via the stoma and 40% had a urethral stula. After additional surgeries, 85% of the patients were completely continent 2years following BNC (15% declined further surgeries or had an ileal conduit).
Of the 15 patients with follow-up longer than 3years, only 40% remained completely dry, and leakage via the stoma developed in 47% after being dry for 1year.
Repeat urodynamic studies did not show any adverse changes in bladder dynamics
in the incontinent patients. Stomal stenosis of the CCC occurred in 30% of the
patients and bladder stones developed in 40%. Novak etal. described 76 patients
(n=42 CBE patients) who underwent AC with BNC [79]. After a mean follow-up
of 44months, continence was achieved in 98% of patients and there were no reports
of bladder perforation. Complications included two bladder neck stulas and ve
patients with stomal leakage. Landau et al. described 12 patients (n = 5 CBE
patients), who underwent BNC with a 10-year follow-up [80]. Continence was
achieved in all patients, and upper tract dilation was noted in the only patient who
did not concurrently receive AC.There were no reports of bladder perforation.
Continent Catheterizable Channel: Mitrofanoff Principle
CIC through the reconstructed urethra in the CBE patients either with or without
BNR may not allow for easy, reliable performance of it. Therefore, an alternative
conduit for CIC can be quite advantageous, and in 1980, Paul Mitrofanoff published
his clinical series utilizing the appendix as the conduit for a CCC for CIC [81]. The
Mitrofanoff principle is based on the implantation of a supple tube within a submucosal tunnel with seromuscular support. The urinary reservoir pressure will rise
during lling and coapts the lumen of the CCC to prevent retrograde leakage through
the CCC and provide continence. The seromuscular layers of detrusor in a bladder
or gastrointestinal reservoir, when the bladder is absent or insufcient, can be utilized for the site of implantation of the conduit.
Although this Mitrofanoff principle has been applied frequently with the appendix, other substitutes have included transversely tubularized bowel segments (YangMonti) or ureteral remnants. Careful patient selection and ensuring a low-pressure
urinary reservoir are critical elements to achieving the successful outcome of the
CCC with a high rate of channel continence. Failure to regularly catheterize and
completely empty the bladder or reservoir can lead to urinary tract infection, hydronephrosis, or reservoir perforation.
When the appendix is utilized for the CCC, assessment of the appendiceal length
and its mesenteric vascular anatomy is important. The appendix can be removed

21 Surgical Options forRefractory Urinary Incontinence inClassic Bladder Exstrophy
345
with a cuff of cecum to provide length and a wider stoma to help minimize stenosis
at the skin level. The mesoappendix should be mobilized until the desired stomal
location can be reached without tension. As with ureteral reimplantation, creation of
the antireux mechanism can be accomplished in an intravesical or extravesical
fashion. For intravesical technique, the bladder can be opened in the sagittal plane
to allow for concomitant reconstructive procedures such as bladder outlet procedure
and/or AC as indicated. The distal end of the appendix can be tunneled into the bladder through neohiatus with a minimal submucosal tunnel length of 3cm [81, 82].
Securement of the appendix to the outer bladder wall at the entry into the neohiatus
into the submucosal tunnel with 3-0 absorbable suture assists to minimize channel
retraction. Bladder immobilization using abdominal wall hitch stitch minimizes
catheterization angulation and false passages. For the extravesical technique, the
bladder is distended via the indwelling catheter and a 3cm incision is made in the
detrusor and seromuscular aps are created on the anterior-lateral bladder wall [83].
The bladder mucosa is not entered during this extravesical dissection. At the distal
aspect of the trough, a mucosotomy is made and the bladder mucosa to appendiceal
mucosa anastomosis is performed circumferentially using absorbable 4–0 suture.
The seromuscular aps of the trough are brought anterior to the channel and sutured
to each other. Incorporating the serosa of the appendix with the suture used for seromuscular ap closure sutures helps maintain the length of the antireux tunnel
as well.
Implantation of the CCC into the detrusor muscle is the desired method however
in the CBE patients this may present a unique challenge if the native bladder is too
small or otherwise unsuitable for CCC tunneling. In these cases, a “serosal trough”
strategy can be used to implant the CCC into a bowel segment used for augmentation [84, 85]. Two parallel incisions through the augmented bowel serosa are created
to expose the muscularis mucosa and submucosa. A full-thickness insertion orice
for the channel is made at the distal end of these incisions. The channel is laid into
the tunneled serosal “trough” and implanted into the augmented bladder orice. The
serosal aps of the parallel incisions are used to overlay the tunneled channel
segment.
It is important regardless of the implantation technique used, the Mitrofanoff
channel is intra-operatively tested on multiple occasions, and each time any operative step is completed, for ease of catheterizability and stomal continence while the
bladder is lled. This will permit the surgeon to identify which step introduced any
change or difculty and adjust accordingly. Depending on stomal location, guide
the appendiceal base to the umbilicus or through the rectus muscle after creating a
cruciate incision in the rectus fascia. Mature the stoma at the skin level by spatulating the proximal end of the appendix and securing a wide-based V- or U-shaped skin
ap at the apex of the spatulation. Key technical points are to maintain the shortest
channel possible to facilitate ease of catheterization; avoid kinking; and if possible,
xation of the bladder to the undersurface of the abdominal wall.
When the appendix is absent, insufcient in its length, or other factors preclude
its use for Mitrofanoff, an alternative substrate is needed for Mitrofanoff. Colon or
ileum is most frequently used and the latter is favored due to the more robust blood

346
B. A. VanderBrink
supply of the small bowel relative to the large bowel. After assessment of mesenteric vascular anatomy, a 2–3cm segment of ileum is isolated from gastrointestinal
continuity on a vascularized pedicle. This segment is opened transversely along the
antimesenteric border according to the technique described by Monti [86].
Tubularization of the recongured intestinal segment in a longitudinal fashion over
a 12- to 14-Fr catheter is performed in two layers using a 5-0 absorbable monolament suture. Implantation of the recongured channel into the bladder and stomal
maturation occurs using the aforementioned surgical techniques as with the appendix. Another alternative technique for creation of a CCC is the Macedo technique
which mandates concomitant AC due to the technique [87]. The CCC is constructed
from the same bowel segment that AC occurs. From 35cm of ileum, a 3cm width
ap from the anterior and posterior wall of ileum in the mid part of the isolated segment. The continence mechanism of the CCC is based on angulation and a serouslined tunnel created with three to four nonabsorbable seromuscular sutures.
CCC has been utilized frequently in the CBE population due to the aforementioned challenges of CIC through the reconstructed bladder outlet or following
BNC.Kasprenski etal. from the Hopkins team described the simultaneous creation
of a CCC at the time of BNR in a series of 24 EEC (n=18 CBE) patients who were
felt not to be candidates of BNR by the authors due to their institution’s eligibility
criteria [53]. The median bladder capacity of these 18 CBE patients was 220mL
and 2 had prior failed bladder closure. For patients with greater than 6months of
follow-up time (n=17), 12 (71%) had dry intervals greater than 3h. Of the 10
patients who were completely dry, 7 (58%) achieved volitional voiding per urethra
with residual urine emptied through their CS.The remaining 5 (42%) were continent by stomal catheterization alone and did not achieve voiding per urethra. Of the
5 patients, 29% did not achieve continence following a BNR-CCC procedure. Three
of the 5 patients (60%) established eventual continence following BNC.In the
Maruf series of 350 patients with EEC where continence outcomes were evaluated,
a CCC was used in 157 patients as part of BNC and continent diversion with 60
additional patients as part of BNR with (n=30) or without (n=30) AC [3]. This
series reported that in those patients who underwent BNR with AC with CCC,
61.3% were continent, with all of these patients performing CIC alone without voiding. Of those who underwent BNC and continent diversion, all 133 patients were on
CIC via CCC.Ulman etal. reported their experience with six CBE patients who had
undergone YDL-BNR with an adjuvant CCC [88]. Four patients had a breakdown
of their reconstructed bladder necks due to CIC via urethra. AC was performed in
all six patients at the same setting as the creation of the CCC.Szymanski etal. as
part of the multicenter study of ve North American pediatric centers from 1980 to
2016 examined the use of CIC in 216 CBE patients [8]. At the last follow-up, 67.4%
of all patients performed CIC.Among 95 patients with an intact native bladder,
30.5% performed CIC (72.4% via CCC). Among those without BNR or with an
AUS, 14.8% performed CIC, compared to 54.8% after BNR and 100% after
BNC.Among 107 patients who underwent AC, all performed CIC, typically via
CCC (86.9%). Older patients on CIC were more likely to use a CCC, increasing
from 61.5% for those younger than 10years to 88.3% for patients >18years. Of 76

21 Surgical Options forRefractory Urinary Incontinence inClassic Bladder Exstrophy
347
adults without a diversion, 85.5% performed CIC.The CIC rate was 100% in adults
who underwent AC and 31.3% in adults with a native bladder. These clinical series
emphasize the use of CIC via CCC as a frequently utilized intervention in the CBE
population.
Complications from CCC arise with short-term and extended follow-up. Stomal
stenosis at the skin level is the most commonly reported problem with a reported
incidence between 10% and 40% [89–92]. False passages can also occur from
repeated erroneous catheterizations or excessive angulation within the channel and
can usually be managed with a temporary indwelling catheter [93]. Stomal inconti-
nence is infrequent and may be due to a short intravesical tunnel, persistently elevated reservoir pressures, or a stula between the channel and the bladder. Lowering
elevated detrusor pressures either medically or surgically can resolve the stomal
incontinence when secondary to abnormal storage pressures. For inadequate continence mechanism due to shortened antireux tunnel, endoscopic management with
Deux has been reported [94]. A formal subfascial revision with redo implantation
to address the inadequate antireux mechanism of the channel is another surgical
intervention performed as well for stomal incontinence.
Augmentation Cystoplasty
The chapter thus far has focused on surgical techniques to address incompetent
bladder outlets and alternative bladder emptying conduits in the form of
CCC. However, the urinary incontinence in CBE patients may be secondary to
impaired bladder capacity with or without abnormal storage characteristics resulting in an insufcient bladder reservoir. Use of pharmacotherapies (i.e. anticholinergics and/or beta-3 agonists) should be used prior to surgical procedures to improve
bladder storage characteristics. When medical management is ineffective, AC is a
highly effective procedure to simultaneously decrease elevated detrusor end-lling
pressures, improve bladder compliance, and increase bladder capacity.
The use of which gastrointestinal segment is utilized for AC varies by patient
anatomy and surgeon preference. Gastric, ileal, and colonic segments have all been
used in CBE patients [8, 95, 96]. Gastric segments are less utilized for AC compared
to other segments due to the typical normal small and large intestine anatomy of
CBE patients. Advantages of sigmoid colon for AC are its anatomic proximity to the
bladder and the thick muscular taenia of the colon may serve as a more robust substrate for ureteral or CCC implantation than the thinner seromuscular layer of ileum.
This author preferentially uses ileum for AC because of its mesenteric mobility,
relative abundance compared to sigmoid colon, and tendency to generate less
mucous than the colon. Detubularization and reconguration of the intestinal segment used for AC into a U- or W-shape provide multiple advantages, including
maximization of the volume achieved for any given surface area, blunting of bowel
contractions, and improvement of overall capacity and compliance. This augment
patch is then sutured to the bladder after making a sagittal incision to provide wide
enterovesical anastomosis.

348
B. A. VanderBrink
AC has been performed in varying rates of CBE published series. Szymanski
etal. in a multicenter study over three decades retrospectively reviewed 212 CBE
patients who underwent primary bladder closure where 108 (50.9%) underwent
augmentation, 10 (4.7%) diversion (1 with a previous augmentation), and 95
(44.8%) neither [8]. After controlling for follow-up time in this series using survival
analysis, the probability of AC was 14.9% by age 5years, 50.7% by 10years, and
70.1% by 18 years. After controlling for follow-up time, this probability varied
signicantly by treatment center; the probability at 10years ranged between 26.7%
and 64.5%, depending on the center. Among 87 children who underwent a bladder
outlet procedure without simultaneous AC for persistent urinary incontinence after
primary closure, the probability of future AC was 46.4% at 5years and 60.7% at
10years after the bladder outlet procedure and did not differ among centers. Maruf
etal. published that out of 432 CBE cohorts treated at Johns Hopkins, 76 and 165
patients underwent AC with BNR or BNC, respectively (56%, 241/432) [3].
Cervellione etal. reviewed 32 CBE patients who underwent bladder closure using a
staged repair technique that underwent a salvage procedure for continence [97]. AC
was performed in 23/32 (72%) and the remaining 9 patients had either neobladder
or urinary diversion procedures.
The complete primary repair technique for CBE has been touted to normalize
anatomy at the initial repair, thereby allowing early bladder lling and cycling
which may inuence bladder function, capacity, and possibly the need for AC.In a
multicenter study of CBE patients, Weiss etal. showed 54 CBE patients who underwent bladder closure using a complete primary repair technique reviewed at three
centers prior to the creation of a clinical consortium between the centers over a
20-year period (1993–2013) [7]. The median age of patients at the last follow-up
was 12years, and they reported AC was performed in 10/54 (19%) with BNR or
BNC.Ellison etal. and DiCarlo etal. reported their own single-institution experience with continence outcomes and procedures following complete primary repair
technique in 29 and 61 CBE patients, respectively [1, 51]. Ellison etal. reported that
6/29 (20%) underwent AC which was very similar to 12/61 (19%) in the DiCarlo
series undergoing AC.No study has been published to date to discern if the CPRE
proves to carry a lower risk for AC.
Surgical technique is critical when performing AC to minimize complications.
Adequate length of the intestine (20cm of either the large or small intestine) utilized will minimize the risk of re-augmentation [98]. Benz etal. reviewed a total of
166 patients with CBE who underwent AC and 17 (10%) patients underwent a reaugmentation. The length of the bowel used for AC was predictive of the need for
re-augmentation. In the re-augmentation cohort, the mean amount of bowel used
during the rst AC procedure was 12cm compared to the mean amount of bowel
used for non re-augmentations which was 20.8cm. There is also a need to widely
open the bladder to minimize the risk of postoperative hourglass deformity contributing to poor drainage and persistent abnormal bladder storage characteristics.
Bladder perforation is once again the most feared complication of AC and in large
series has been reported to occur 5–10% in large clinical series of patients with CBE
and other diagnoses [99–101]. Detubularization and retubularization are useful for

21 Surgical Options forRefractory Urinary Incontinence inClassic Bladder Exstrophy
349
minimizing the risk of bladder perforation [102]. The most commonly reported
need for additional surgery after AC is the presence of bladder calculi and occurs in
15–30% of patients [102–105] There is a high recurrence rate observed in patients
with AC that initially developed a stone-acquiring second bladder calculi within a
short period of time of their rst occurrence. Recurrence of the bladder stone in the
AC patient was independent of the method of removal and found to be similar for
both open or endoscopic extraction [106, 107].
Continent Urinary Diversion
In the rst half of the twentieth century, standard care of the CBE patients was not
predicated on bladder preservation as closure and functional use of the exstrophic
bladder was met with poor success rates [108, 109]. Urinary diversion, with cystectomy, was near universal and this frequently involved urorectal anastomosis for
CBE patients as rst described by Simon in 1852 [110]. Coffey in 1930 rened the
ureteral anastomosis technique to reduce the risk of reux and ureterosigmoidostomy was pursued as a standard of care for CBE patients [111]. The ureterosigmoidostomy was accompanied by a high incidence of metabolic acidosis and intestinal
dysfunction. This was noted in patients who underwent ureterosigmoidostomy with
extended follow-up and improved survival, an increased risk of colorectal carcinoma which dampened the enthusiasm for this technique [112–115]. At the same
time as the initial reports describing neoplastic complications following ureterosigmoidostomy became more published, Eugene Bricker popularized the ileal conduit
as an incontinent form of urinary diversion—the so-called “Bricker Bladder” [116]
To overcome the disadvantages of the classical ureterosigmoidostomy and
reduce the number of postoperative upper urinary tract complications as well as to
improve the continence rates, Fisch and Hohenfellner introduced the rectum-sigma
pouch (Mainz Pouch II), which transformed the high-pressure segment of the rectosigmoid into a low-pressure reservoir by detubularization and reconguration [117].
Fixation of the rectosigmoid junction at the promontory as well as parallel ureteral
implantations guarantee a straight ureteral path and prevent kinking and subsequent
obstruction. The antimesenteric opening of the bowel creates a low-pressure reservoir, which is expected to be essential for good day and night continence and confers advantages for the upper urinary tract. The group from Mainz described their
technique for minimizing the upper tract complications with antireux ureteral
reimplantation whether the ureter was normal caliber or dilated [118]. D’elia etal.
utilized the Mainz II pouch in 123 patients over 10years (n=26 with EEC) with
reported continence rates day and night of 97% and 95%, respectively [119]. 70%
of the patients were on alkalinizing oral medications to address subclinical acidosis
seen on blood gas to prevent the development of clinical hyperchloremic acidosis.
Mingin etal. [17] described ve patients with bladder exstrophy who underwent the
Mainz pouch II procedure between 1996 and 1998 to create a rectosigmoid pouch,
allowing urine to drain into and be eliminated via the rectum [120]. Three patients
required oral sodium bicarbonate to correct metabolic acidosis, but upper tracts

350
B. A. VanderBrink
remained nondilated, all patients were continent during daytime and nighttime, and
there were no episodes of pyelonephritis during follow-up, which was between
about 1 and 3years.
Despite the surgical and technical advances for the modern staged reconstruction
of the bladder involving the preservation of the bladder early in life, there is a subset
of CBE patients where the bladder is not deemed a suitable substrate for reconstruction utilizing many of the lower urinary tract reconstruction previously discussed in
this chapter such as BNR, CCC, and AC.Cystectomy of the exstrophic bladder is
rarely performed in contemporary practice where the modern staged repair is the
preferred surgical philosophy. Ko etal. described the Johns Hopkins experience in
18 patients of whom (n=15 with CBE), six were performed primarily with diversion and twelve were performed secondarily. Of the patients with primary cystectomy this occurred at age ranges of 1–9years due to what authors state were “small,
brotic, noncompliant, polyp-covered bladders” of poor quality unsuitable for
immediate closure [121]. Bladder template size and tissue quality were followed in
these patients before determining that they were “unsuitable for closure.” [121] The
authors concluded from this series and their extensive experience that cystectomy
should be considered in select CBE patients where the bladder template is of inadequate size or of too poor quality to permit primary closure, patients with low bladder capacity after prior closure either due to inadequate growth or loss of capacity,
particularly at the time when a continence procedure is being considered.
When rectal diversion is not pursued an incontinent conduit with ileum or colon
into abdominal wall stomal appliance has been employed. The relative simplicity of
the procedure compared to continent reconstructive techniques has been offset by
the long-term complications from a urostomy stoma (retraction/stenosis) as well as
ureterointestinal anastomotic complications. Deterioration of the upper urinary tract
and renal function, calculi formation, and stomal stenosis are observed as late complications at rates of 50–86% with ileal conduits in pediatric patients [122, 123].
With ileum, there is typically a reuxing ureteral anastomosis while colon affords
the opportunity of non-reuxing ureterocolic anastomosis. The group from Mainz
reported on 105 pediatric patients (n= 16 CBE) with colonic conduit with nonreuxing ureteral anastomosis with a mean follow-up of 16years in 84 patients
[124]. Early and late stenosis at the ureterocolic anastomosis and stomal stenosis
occurred in 7.6 and 15.5% of the cases, respectively. Eight kidneys without function
after recurrent pyelonephritis, calculi, or stenosis at the ureterocolic anastomosis
were removed during follow-up. A total of 31 patients had complications during
follow-up and 18 underwent conversion to continent urinary diversion.
When the use of closed continent urinary diversion is preferred or selected over
a conduit, and the bladder deemed unusable, there are various continent urinary
reservoirs (CURs) that have been described in the literature [82, 87, 125–128]. As
with AC, the creation of CUR is predicated on detubularized and recongured
bowel segment with typical nonreuxing ureteroenteric anastomosis. The CUR
relies upon a continent catheterizable efferent limb created from a variety of substrates such as in situ appendix, tapered ileum, or recongured bowel. The appendiceal lumen can inuence the maximal diameter of the catheter used for drainage.

21 Surgical Options forRefractory Urinary Incontinence inClassic Bladder Exstrophy
351
Wiesner etal. retrospectively compared the intussuscepted ileal nipple and in situ,
submucosally embedded appendix as continence mechanisms in 400 patients who
underwent cutaneous ileocecal pouch (Mainz pouch I) [129]. Continence mechanisms were nearly equal between the two techniques. 36% of the entire cohort
required intervention for a stomal complication with a higher rate of stomal stenosis seen (32% v 17%) in an in situ, submucosally embedded appendix compared to
intussuscepted ileal nipple, most likely due to the smaller diameter of the appendix. However, stone complication was seen in 20% of the ileal nipple group compared to 10% in the appendiceal group likely related to the use of metal staples to
create the intussuscepted ileal nipple. Continence rates of the two outlets were very
good (82% in ileal and 93% for appendix). The complications associated with
CUR increase with increased follow-up and are not dissimilar to that of AC; such
as reservoir calculi, reservoir perforation, metabolic disturbances, and
gastrointestinal [130].
As long-term follow-up of reconstructive procedures in CBE patients such as
conduits, AC, or CUR have revealed a considerable complication rate; interest in
ureterosigmoidostomy as a simple and appliance-free technique of continent urinary diversion has rekindled. Whether bladder reconstruction techniques performed for continence that preserves the exstrophy bladder, which frequently are
dependent on CIC, are superior to the rectal urinary diversion which can be emptied voluntarily via the natural rectal sphincter and is performed at a single operation, will continue to be the subject of much controversy and discussion
over time.
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B. A. VanderBrink
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