Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5514_Библиотеки_им_академика_М_И_Перельмана.pdf
Incontinence inExstrophy-Epispadias:
Should WeChange Our Thinking?
DanaA.Weiss
Introduction
When a child is born with the bladder outside the body, an open bladder neck, and
an open urethra, with a short phallus or bid clitoris, there are multiple goals for the
future. These goals focus initially on restoring normal anatomy with an internalized
bladder and reconstructing a more normal appearance for the genitalia. Next, the
focus shifts to urinary function, optimizing the bladder to hold urine at safe storage
pressures and to empty at low pressures to prevent urinary tract infections and to
preserve renal function. The next phase naturally aims to achieve dryness, again
while ensuring protected renal function. Finally, as a child shifts into adulthood, the
focus shifts to optimizing sexual function and fertility.
While these aims can be approached separately, each is interrelated. Closure of
the bladder and bladder neck must be achieved with just the right amount of resistance. If the resistance is too low, the kidneys might remain safe, as there will be no
urine stasis or elevated bladder pressures, but gross incontinence may mean that the
bladder will not grow and cycle. If the bladder outlet resistance is too high, the bladder will overwork, become hypertrophic, and transmit elevated pressures to the kidneys, leading to chronic kidney disease or even renal failure. Next, if a bladder neck
reconstruction is done with the goal of dryness through intermittent catheterization,
and especially if the bladder is augmented, then the child and later the teen and adult
are committed to a consistent and regimented catheterization schedule with attendant risks of infections, bladder stones, damage to the kidneys, and even more catastrophic events, such as perforation of the augment and death. Thus, it is critical to
gauge interest and buy-in both from a family wishing their child to be “normal” and
20
D. A. Weiss (*)
Children’s Hospital of Philadelphia, Philadelphia, PA, USA
Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
e-mail: Weissd1@chop.edu
© 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_20
323
324
from the child, who needs to want to engage in these practices not just for the present, but also throughout the teenage years, when often there is resistance to participating in any xed routine.
Other chapters in this book are focused on how to achieve continence with a
surgical approach. This chapter is intended to bring focus to the questions of what,
when, and why to achieve continence.
D. A. Weiss
Defining Continence
Since continence is one of the key outcome measures in exstrophy, one would think
that there would be a clear denition. However, one of the challenges in interpreting
results across time and institutions is the lack of a consistent denition. Moreover,
as there are some general themes that are shared among the various denitions, the
question then arises as to where these came from. Did patients participate in the
assessment and creation of these denitions, or are they physician-made?
Continence versus Dryness
Most studies and outcomes distinguish continence with the ability to void from
continence with the need to catheterize. One way to clarify these disparate outcomes is perhaps in the wording: the word continence means the ability to hold
urine and volitionally empty the bladder via the urethra, while the word dryness
would indicate the ability to hold urine but with the requirement of a catheter to
empty the bladder or the use of a drainage bag. Going forward in this chapter, the
words continence and dryness will be used to differentiate between these two groups.
Dry Interval: How Long Is Long Enough?
The next variation in outcomes reported is the time interval required to be considered continent, or dry. The most commonly accepted interval is 3hours. A review of
the literature by Lloyd etal. in 2012 determined that out of 87 studies from 2000 to
2012, the most common denition used to assess continence was “dry with voiding/
catheterization every 3 hours” [1]. This is important because the most common denition did not separate voiding vs. catheterization; however, the time interval was
the same.
The time interval for dening continence or dryness in patients with exstrophy
has not been uniform. In 1978, the denition used for continence for exstrophy was
“being completely dry for a minimum of 2 hours while the patient was awake without pharmacological or mechanical aids” [2]. That 2-hour interval was used in the
setting of patients undergoing a combined Young-Dees urethroplasty and MarshallMarchetti-Krantz bladder neck suspension, with assessment of outcomes at an average age of 14years (range 7.5–24.5years).
20 Incontinence inExstrophy-Epispadias: Should WeChange Our Thinking?
325
In a follow-up of 207 patients from 1945 to 1985 treated at Columbia, which was
then a major exstrophy center, continence was dened as “excellent” if the criteria
were met that “the patient maintained a daytime dry interval of at least 3 hours with
rare episodes of nighttime wetting and stress incontinence” [3]. This study also
included an intermediate continence marker, which is helpful for those who may be
quite satised with voiding a little more frequently: “A good result was dened as a
postoperative daytime dry interval of 1–3 hours…” Finally, “a poor result was a dry
interval of less than 1 hour” [3].
Dry Intervals: What Is Meaningful
While 3hours seems to have become the gold standard for being considered “continent” or “dry,” there have not been enough studies on what these dry intervals
mean for the patient. How different is it for a child—or adult—to have to void every
2hours compared to every 3hours? And should the timing be different if they have
to catheterize vs. if they can void? And at what price is it worth it?
In the adult literature, there has been an increasing number of investigations
concerned with the degree of and bother of incontinence in both males after prostate
surgery and females with stress urinary incontinence. For some men, who have
spent a lifetime not having to think about their bladders, even a drop of urine, or the
need for the use of a pad, is a signicant burden, while healthy women may be much
more prone to having 1–2 leaks per 24-hour period, ranging from small to larger
volumes, even at a young age [4].
A challenge in reviewing the literature regarding the importance or signicance
of specic dry intervals concerns the condition being evaluated. The literature on
patients with spina bida and patients with dysfunctional voiding who are otherwise
anatomically normal tend to focus on incontinence episodes rather than dry intervals. This is difcult to correlate to BEEC, since for most children with BEEC the
question is how long they can stay dry between voiding. Even in the spina bida
literature, the denition of continence has changed over time. For example, initially
continence was dened by the survey question of “dry, with or without interventions, during the day.” This denition shifted to a question stating “Quantify frequency of bladder incontinence during the day over the last month (when not having
a UTI),” and for this question, the answers of “Never” or “Less than once per month”
t the classication of “continent” [5].
Diversion Versus Continence
In the age of incontinent diversion, there was a different balance to consider, as the
child would be destined for either a stoma bag or leakage per urethra. Each camp
had its advocates—one placing emphasis on dryness of the genitalia for later normal
sexual activities, despite the presence of a stoma bag, while the other advocated for
management of urethral incontinence and living without a stoma bag [6]. Even at
326
D. A. Weiss
that time, Lattimer, a leader in exstrophy care, felt that waiting for continence was
better than upfront diversion. In his study, he worked with pediatric psychiatrists to
evaluate the long-term (over 20years) outcomes of patients and concluded, “Our
psychological studies have indicated that the wait several years for the possibility of
urinary continence does not leave these patients with insurmountable psychological
trauma.” While the trade-off that he spoke of was closing the bladder and waiting
compared to diversion with an abdominal stoma, one could extrapolate that he may
take this trade-off even for complex continent diversions and all the complications
associated with those procedures.
Timing andCost ofContinence
Timing
The next question, that follows after assessing the interval of dryness needed to
dene continence or dryness, is the timing at which dryness or continence can or
should be achieved. While on average children toilet train between 2 and 3years of
age [7], there are some children that remain wet through their fourth year, and a
pediatric urology clinic sees children through school age and into high school with
urgency, frequency, and even wetting; some manage with voiding every 2hours to
avoid leaks. So, should a child who is born with an open bladder that never has had
the normal developmental chance to cycle and grow be expected to achieve the
same bladder capacity and develop continence at the same age as a child with a
normal bladder? If we allow for children to void every 2hours in our dysfunctional
voiding clinic, why should voiding every 2hours for a child with exstrophy be considered not good enough? Children with exstrophy are often very well adjusted to
their lives and many times do not have signicant bother by being in a pull-up even
into school-age years. The distress usually comes from external inuences, such as
parent and teacher concerns. We see this often in the setting of nocturnal enuresis,
where the child, even until 10–12years of age, does not mind being wet at night, but
the wetness—and often more than the wetness, the lack of distress from the child—
causes the greatest distress to the parent. To this end, encouraging a 5- or 6-year-old
to take on the challenges of continence, to demand timed voiding or catheterization
on a set schedule when they would much rather be carefree and time-free, perhaps
adds undue stress to the otherwise happy child.
In children with BEEC, studies have indeed found that continence develops later
in life, often developing after puberty [8]. Development of continence requires a
combination of factors: enlarging growth of the bladder to provide a large enough
reservoir to hold the urine and adequate bladder outlet resistance in the form of a
competent bladder neck and external sphincter complex. Even for patients who
undergo a formalized bladder neck reconstruction in the setting of a staged repair,
bladder capacity greater than 100cc has been found to be a predictor of the development of continence [9–12]. Khandage etal. recently demonstrated that there was a
50% chance of reaching this goal of 100cc by 5.7years of age. However, they also
20 Incontinence inExstrophy-Epispadias: Should WeChange Our Thinking?
327
showed that bladder capacity continues to increase over time, and up to 80% of
patients had achieved 100cc capacity by 8years of age [11]. While the focus of
these studies is to demonstrate that 50% would have an adequate bladder capacity
for BNR by age 5–6, the data also indirectly supports the concept that over time, the
bladder grows in the setting of gentle outlet resistance, and with continued bladder
growth, there is continued potential to develop longer dry intervals. Perhaps in
another 20years, as trends shift from proceeding with reconstruction at 5–6years
of age to 8–9 and even older past puberty, we will nally know the real numbers
who have the potential to achieve continence over time.
While the data are still immature, we have anecdotally seen children develop
gradually increasing dry intervals over time. In children who were closed as a newborn, we have seen continence increase by 20–30minute intervals year after year.
In some children who had the bladder closed at a later age, sometimes 10–11years,
as part of our international surgical collaboration, we have even found that they
achieve 2–3hour dry intervals within a year. Thus, by looking at trends and gradual
changes for each child, physicians can provide personalized expectations and treatment plans.
Cost ofContinence
The next question in the calculus of achieving dryness or continence is to what
lengths a child and family want to go to achieve dryness vs. how long they are willing to wait to see if continence will develop with time. This varies signicantly
between families but also varies signicantly based on external inuences and discussions with surgeons and care givers. In centers where the routine is to give time
to gradually develop growth of the bladder and improve sphincteric control, families are more likely to tolerate and manage the wet child and to give time for the
achievement of continence, gaining motivation from each gradual increase in dry
interval. On the other hand, in centers where from the start, the plan is to progress
to dryness, either through bladder neck reconstruction or bladder neck closure, the
family will be gearing up from birth for that day when dryness is achieved, and for
some, that time cannot come soon enough.
The goal for early dryness comes from the motivation to normalize children
among their peers and to get them out of diapers and pull-ups as soon as they are
willing to participate in a catheterization regimen. This early dryness comes with
the cost of further surgeries, the need to catheterize on a strict regimen, and the risk,
especially in the setting of an augmentation or bladder neck closure, of signicant
complications. These costs are multiplied over a lifetime. Lastly, the promise of
dryness even with a bladder neck reconstruction is not a guarantee after just one
surgery. From one of the largest experiences in BNR, almost 41% of children progressed to other procedures after an initial BNR, and even nearly 28% of those who
underwent BNR with augmentation and/or catheterizable channel went on to further
surgeries [13]. Moreover, families must know that even a bladder neck closure is not
a 100% guarantee for continence, as 9/124 patients in this large series remained
328
D. A. Weiss
incontinent even after BNC and continent diversion [13]. Probably with enough
surgery, these all can become dry, but again, when the question is what is the cost of
dryness, each surgery adds up.
The ip side of the argument is that while childhood dominates the present and
the desire to be dry is real, ultimately childhood—including the teenage years—is
only a small fraction of a lifetime. If there is the potential to achieve voiding with
continence during the 50+ years of adulthood, is it worth being wet, to some degree,
for the rst 10–20years of life?
Physical Therapy forContinence
To follow along with the question of “At what cost dry?”, we must also look at what
it takes to be continent. We don’t presume that the majority will be magically dry
when they hit their 13th birthday. The thought is that over time, with gradual development of the external sphincteric muscles in parallel with the gradual growth of the
bladder, a child will be able to hold more and more urine. As detailed in another
chapter, the role of pelvic oor physical therapy is coming to the forefront in all
areas of urinary health, and none more so than in the management of bladder exstrophy. Much of this therapy involves intensive work by the child and the family. This
starts with bladder training to really focus on the bladder, spending a concentrated
effort, both at home and during therapy sessions, on feeling and responding to the
bladder, voiding on a strict timed schedule, and tracking improvements. In addition,
at times, the improvements will be directly related to the intensity of the training: at
times of rigorous training, the person may be dry, but when they lessen their intensity, wetness recurs. This can be frustrating.
Challenging Dogma
The goal of this chapter is to challenge dogma. If we don’t do that, we will never
advance the care of our patients. So the questions I propose are: What is magical
about 3hours? Why is the drive to be dry at 5years, or to be dry by kindergarten,
driving our medical care? Where does freedom and independence without worrying
about the bladder play a role?
Many studies use an all-or-nothing approach to dry intervals; more than or less
than 3hours without the use of any pads. Others offer an “intermediate” success
outcome: a dry interval of 2hours. But what if a child is happy and functional being
continent if they void every 1.5–2hours, then that should count for something—and
that child should be considered continent. If a child or young adult can wear a pad
or liner for minor leaks and peace of mind, they may still be considered “wet,” but
they may be functionally happy. So, some children and families might decide that
the benet of becoming completely dry is not worth the cost.
While each management decision needs to be based on a discussion between the
patient/family and the physician, there are now outside inuences that are much
20 Incontinence inExstrophy-Epispadias: Should WeChange Our Thinking?
329
more prevalent than 15years ago. Social media has allowed for ongoing discussions and conversations at best, or passive observations at worst, that tell a parent
what they should be doing, how they should be thinking, and what choices they
should make for their child. Some may be pressured to seek dryness by age 5–6
and may consider anything later than that as a failure. But what if the child
doesn’t care?
We have made patience the hallmark of our approach to continence in bladder
exstrophy. We lay the foundation for this from the earliest meeting—be it prenatally or in the rst visit in clinic. Our goal is to keep each child safe and healthy
and allow the best chance for them to void normally. While we hope that with
newer techniques of closure we will see increasing numbers of children who
achieve continence with voiding after only a single surgery, even the prospect that
20% will be able to be continent with voiding is enough to keep families working
toward the goal. Even some older children who have not achieved continence are
now minimally bothered by the wetting that even when offered a procedure, they
decline.
References
1. Lloyd JC, Spano SM, Ross SS, Wiener JS, Routh JC.How dry is dry? A review of denitions
of continence in the contemporary exstrophy/epispadias literature. J Urol. 2012;188:1900–4.
https://doi.org/10.1016/j.juro.2012.07.017.
2. Toguri AG, Churchill BM, Schillinger JF, Jeffs RD.Continence in cases of bladder exstrophy.
J Urol. 1978;119:538–40.
3. Connor JP, Hensle TW, Lattimer JK, Burbige KA.Long-term followup of 207 patients with
bladder exstrophy: an evolution in treatment. J Urol. 1989;142:793–5. https://doi.org/10.1016/
S0022- 5347(17)38890- 0.
4. Lukacz ES, Falke C, Geynisman-Tan J, Wyman JF, Mueller ER, Markland AD, etal. Healthy
bladder storage and emptying functions in community-dwelling women measured by a 2-day
bladder health diary. Neurourol Urodyn. 2023;42:725–35. https://doi.org/10.1002/nau.25169.
5. Wiener JS, Suson KD, Castillo J, Routh JC, Tanaka ST, Liu T, Ward EA, Thibadeau JK, Joseph
DB; National Spina Bida Patient Registry. Bladder management and continence outcomes in
adults with spina bida: results from the national spina bida patient registry, 2009 to 2015.
J Urol. 2018;200(1):187–94. https://doi.org/10.1016/j.juro.2018.02.3101. Epub 2018 Mar 26.
PMID: 29588216; PMCID: PMC6919313.
6. Lattimer JK, Beck L, Yeaw S, Puchner PJ, Macfarlane MT, Krisiloff M.Long-term followup
after exstrophy closure: late improvement and good quality of life. J Urol. 1978;119:664–6.
https://doi.org/10.1016/S0022- 5347(17)57583- 7.
7. Vermandel A, Van Kampen M, Van Gorp C, Wyndaele J. How to toilet train healthy children? A review of the literature. Neurourol Urodyn. 2008;27:162–6. https://doi.org/10.1002/
nau.20490.
8. Taskinen S, Suominen J, Mäkelä E.Development of late continence in bladder exstrophy
and epispadias patients. Urology. 2020:S0090429520307937. https://doi.org/10.1016/j.
urology.2020.06.034.
9. Bar-Yosef Y, Savin Z, Ekstein M, Ben-David R, Dekalo S, Bar-Yaakov N, etal. Preoperative
bladder capacity predicts social continence following bladder neck reconstruction in children
born with exstrophy–epispadias complex. Eur J Pediatr Surg. 2023;33:510–4. https://doi.
org/10.1055/a- 2003- 1823.
https://doi.org/10.1016/S0022- 5347(17)57541- 2.
330
10. Chan DY, Jeffs RD, Gearhart JP. Determinants of continence in the bladder exstrophy population: predictors of success? Urology. 2001;57:774–7. https://doi.org/10.1016/
S0090- 4295(00)01102- X.
11. Khandge P, Morrill CC, Wu WJ, Harris KT, Haffar A, Maruf M, etal. Achieving goal capacity
for continence surgery: a cumulative event analysis of bladder exstrophy patients. J Pediatr
Urol. 2023;19:563.e1–8. https://doi.org/10.1016/j.jpurol.2023.04.039.
12. Massanyi EZ, Shah BB, Baradaran N, Gearhart JP. Bladder capacity as a predictor of
voided continence after failed exstrophy closure. J Pediatr Urol. 2014;10:171–5. https://doi.
org/10.1016/j.jpurol.2013.08.003.
13. Maruf M, Manyevitch R, Michaud J, Jayman J, Kasprenski M, Zaman MH, etal. Urinary continence outcomes in classic bladder exstrophy: a long-term perspective. J Urol. 2020;203:200–5.
https://doi.org/10.1097/JU.0000000000000505.
D. A. Weiss
Surgical Options forRefractory Urinary
Incontinence inClassic Bladder
21
Exstrophy
BrianA.VanderBrink
Introduction
The congenital defect in classic bladder exstrophy (CBE) results in signicant aberrations in typical urinary tract anatomy. Despite a successful primary repair, regardless of the technique employed, there is frequent urinary incontinence in this patient
population [1–4]. The urinary symptoms contribute to a decreased patient-reported
quality of life [5, 6]. Therefore, interventions aimed at improving the urinary symptoms are pursued, each with their own varying degrees of effectiveness and
invasiveness.
The social consequences of urinary incontinence begin to manifest as the child
approaches primary school age when most of their peers are toilet trained and not
using protective undergarments. The timing of active interventions for urinary
incontinence differs amongst patients and their families and may be dependent upon
their respective psychosocial circumstances and physician biases. Many nonsurgical methods have been recommended in the form of surveillance and psychosocial
support, pelvic oor therapy, pharmacotherapy, and clean intermittent catheterization (CIC) via urethra prior to additional surgery undertaken for purposes of achieving continence. This chapter is aimed at discussing and reviewing the surgical
options for refractory urinary incontinence in CBE patients following primary
surgery.
B. A. VanderBrink (*)
Division of Pediatric Urology, Cincinnati Children’s Hospital Medical Center,
Cincinnati, OH, USA
e-mail: Brian.vanderbrink@cchmc.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_21
331
332
B. A. VanderBrink
Preoperative Counseling
It is extremely helpful to set realistic expectations for the CBE patients and their
family about observed urinary continence outcomes after primary bladder surgery
in the surgeon’s personal experience, as well as published series. In the author’s
opinion, this message can never be communicated too early nor too often to CBE
patients and their families/care providers. They should be informed that the prospect of volitional voiding accompanied by urinary continence both day and night is
the exception rather than the norm in CBE population based upon existing literature. Large retrospective reviews in single and multiple institutions have seen this
“holy grail” outcome in CBE patients of urinary continence while voiding, with or
without additional bladder neck surgery, and not catheterizing in only 20–25% of
patients [3, 7, 8]. Therefore to achieve social continence, the majority of CBE
patients will likely require additional lower urinary tract reconstructive surgery following primary bladder closure.
An emphasis on proper patient selection for any of the continent reconstruction
surgeries discussed in this chapter cannot be understated. Assessment of the degree
of bother from the urinary continence will undoubtedly inuence the intensity of
and desire for further intervention. Bother can be quantied with patient-reported
outcomes utilizing validated quality of life (QOL) questionnaires or also more simply by asking the patient or parent during the clinical encounter [9]. But it must be
underlined that parent-reported (or proxy-reported) responses as to their child’s
QOL is an observers’ impression of a child’s internal experience: it is not interchangeable with the child/patient-reported QOL.Parents, healthcare providers, and
other proxies typically underestimate patents’ QOL [10]. Therefore, a single visit
and response may not be adequate prior to complex urologic reconstruction for
incontinence to discover or understand these nuances of patient/family preferences
or social determinants of health.
Due to the frequent need for additional procedures following and complications
associated with a complex lower urinary tract reconstruction discussed in this chapter [11], it is this author’s practice pattern to not schedule surgery upon rst meeting
the patient or family despite obvious indications/presence of bothersome urinary
incontinence. The Johns Hopkins team has advocated for an intense pre–bladder
neck repair program conducted by a senior urology nurse practitioner, senior clinical nurses, and a child psychologist well versed in voiding issues prior to bladder
reconstruction [12]. These sessions are begun for both child and parents at least
6months before the proposed bladder outlet procedure with successful completion
of the program required before surgery is scheduled. Bladder capacity has been
observed to be a predictor of continence after bladder neck reconstruction (BNR)
alone. [13, 14] However, additional nonphysiological considerations such as emotional maturity of the patient, adherence to lifestyle modications, and adequate
family support may have an impact on urinary continence [3]. As such, patient
selection, which represents a collective consideration of all of these factors, likely
remains the most important tool for achieving continence after reconstructive
surgery.