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374
S. Sen et al.
The benets achieved from surgical reconstruction must be maintained throughout
the life of the child, and most major reconstructions fail in this regard, especially as
regards reliable adult life continence and protection of the kidneys. The reconstructed exstrophy, sans BA, has or develops urodynamic abnormality, which, coupled with VUR (persistent or recurrent), takes a toll on renal function [8]. The
results of bladder neck surgery and bladder capacity achieved are less than ideal.
Thus a degree of dryness achieved is often lost in teenagers/adults, partly due to
greater expectations beyond childhood and continuing urodynamic deterioration. In
a recent American study of long-term results in exstrophy, several relevant issues
came to light: Reliable continence was mainly achieved in augmented bladders with
a catheterizable stoma, which, however, was achieved after many surgical procedures in the individual patient [9].
Management of exstrophy has challenges that, though applicable universally,
have special relevance in a developing country. Many children have “failed exstrophy,” including dehiscence or redehiscence of attempted bladder turn-in, poorly
executed epispadias repair, neglected exstrophy with a small polypoid bladder,
continuing incontinence after bladder turn-in, failed bladder neck surgery, and
deteriorating upper tracts. Some of them with a stenotic bladder outlet and small
bladder capacity with HUN with dilating VUR are of considerable concern in the
long term. Meanwhile, these children are unable to attend school, the use of diapers is becoming prohibitively expensive, and their troubles are compounded by
UTIs, calculi, and progressive renal failure (20% in this report). Many are hypertensive in adult life.
Repeated surgery without BA fails to bring relief to these patients, with attendant
costs and hopelessness. We believe that BA, a reliable Mitrofanoff, ureteric reimplantation, and bladder neck surgery (YDL-3/4 and BCN in ¼) are reliable reconstructions in these children.
Children who come to us without any previous reconstruction are offered bladder
turn-in, epispadias repair, and fasciomuscular closure with the help of a RAM ap
in infancy, avoiding the rst 3months of life. The rst 3months are avoided, as
thereafter the RAM becomes more robust. The bladder outlet, bladder neck, and
urethra are repaired in continuity with no attempt at “tightening” of the bladder
outlet. Ureteric reimplantation is not attempted at this stage, and the child is kept on
prophylaxis. A few children develop a moderate amount of continence (holding on
until they reach the toilet to void and not wet). They are kept on careful follow-up
for UTI.Most children, however, are offered BA at around age 5years as a more
realistic and safe long-term option. Contact and social interaction with other children and families help to reassure parents, especially as regards CIC via a catheterizable stoma. Thus, these children have a two-stage reconstruction, completed by
school-going age.
In older children with a neglected/dehisced bladder plate, we have incorporated
BA into the open and mobilized bladder plate, achieving abdominal closure in a
single stage.
The YDL bladder neck plasty, originally described for allowing dryness with
continent voiding, fails to provide reliable results in this regard. Instead, there are
22 Urinary Reconstruction for Bladder Exstrophy in the Developing World: Special…
375
undesirable effects on the unaugmented bladder as the procedure consumes some of
the already small bladder capacity as well as inicts undesirable urodynamic
changes in the bladder that remains. This results not only in poor continence but
also upper tract pathology, as shown by HUN and diminished eGFR.BA offers a
reliable solution, although it necessitates to lifelong CIC.Large low-pressure storage capacity is created, providing better continence, better protection, and recovery
of upper tracts. This also allows a “longer” and narrower YDL plasty, as the residual
capacity of the native bladder is not a concern. The YDL allows for the greater
safety of pop-off if CIC is delayed, but not for voiding via naturalis.
Exstrophy is associated with VUR, and when the bladder is urodynamically
abnormal, there is a dilating VUR.Lowering bladder pressure by BA does not
(unlike in augmented neurogenic or valve bladder) reliably eliminate VUR in many
children. This can be a cause of repeated UTIs and renal scars. Thus, a ureteric
reimplantation becomes necessary. Unlike some centers, we have not undertaken
ureteric reimplantation at the time of bladder turn-in, as these bladders are often
unhealthy and ureters are of ne caliber in an infant. Ureteric reimplantation has
been done at the time of BA when the bladder plate improves in quality after bladder
turn-in. Some were done in the colonic segment of the donut augment when the
bladder was very small.
These children will need lifelong CIC, which should be easy and painless, and
allow a 12 Fr catheter for draining urine and mucus. A catheterizable stoma rather
than periurethral CIC is essentially preferable, generally using the appendix
implanted into the bladder plate. The “in situ” appendix in the “doughnut” ileocecal
augmentation with its ileal periappendiceal cuff provides a non-reuxing, wellvascularized channel without any implantation into the already small bladder. It also
provides catheter drainage to the “dependent” area of the bladder–augment cavity
without impinging on the sensitive trigone.
The above reconstruction shows excellent results, with dryness with 3–4
hourly CIC in 95%, resolution of VUR in 165 of 194 renal units, and resolution
of persisting HUN.There was a signicant improvement in eGFR (p=0.036) in
children with subnormal eGFR, indicating a degree of reversibility in
renal damage.
The supply and cost of catheters is an important factor for an individual with
lifelong CIC.We insist on a clean and dry catheter, which could be used for even
6months. The family is supplied with a cloth catheter bag with multiple channels in
which a set of catheters (feeding tube or Nelcath) is kept after cleaning with tap
water. This allows the catheter to dry before the next use and is not a source of UTI
[9]. Additionally, the child is encouraged to keep the catheter “in situ,” with its outlet plugged, between CIC sessions during the rst year or so after surgery. This
makes intermittent bladder drainage easy at school and also prevents skin-level stomal stenosis. Daily bladder washouts using drinking water are insisted upon. The
low incidence of calculi is possibly the outcome [10, 11] (Fig.22.12).
376
Fig. 22.12 Cloth catheter
bag with catheters in place
drying in the sun
S. Sen et al.
Conclusion
We describe a method of treating exstrophy by an effective and safe two-step reconstruction including BA and Mitrofanoff CIC as an integral part of the reconstruction. This has yielded reliable results for more than 90% of the children treated. The
chief hurdle is the crossing of the psychological barrier of accepting CIC, which is
accepted either after many failed attempts at reconstruction or, preferably, by counseling from the very start, helped by contact with other reconstructed children.
References
1. James Sam C, Kurian JJ, Kishore R, Arunachalam P, Sen S.Management and outcome in
dehisced exstrophy with a simplied bladder re-closure and further reconstruction. J Pediatr
Urol. 2020;16(6):836.e1–8.
2. Gearhart JP, Leonard MP, Burgers JK, Jeffs RD.The Cantwell-Ransley technique for repair of
epispadias. J Urol. 1992;148(3):851–4.
3. Barla RK, Sen S.Skin cover in epispadias repair by dorsal transposition of a ventral island
ap: a modication of the Pippi Salle technique. Pediatr Surg Int. 2015;31(11):1099–102.
4. Sen S, Zachariah N, Chacko J, Thomas G.Buttressing the divided bladder neck by a rectus
abdominis muscle ap to prevent urethral recanalisation in paediatric urinary incontinence.
Pediatr Surg Int. 2003;19(1–2):124–6.
5. Sen S, Arunachalam P, Sam CJ.Non-obstructive, non-reuxing uretero-colonic anastomosis
by colonic sero-muscular excision and extra mural peri-ureteric wrap– a new technique in 17
children. J Pediatr Urol. 2021;17(1):99.e1–7.
6. Babu R, Sudipta S.Augmentation in exstrophy. In: Babu R, editor. Dysfunctional and neurogenic bladder, medical and surgical management. 1st ed. Singapore: Notion Press; 2021.
p.181–92.
22 Urinary Reconstruction for Bladder Exstrophy in the Developing World: Special…
7. Sudipta S, Viju J. “Doughnut Augmentation”- ieocaecal segment with in-situ appendix used
as a simple but effective bladder augment/substitute in very small bladders. J Pediatr Urol.
2008;4(1):S40.
8. Diamond DA, Bauer SB, Dinlenc C, Hendren WH, Peters CA, Atala A, Kelly M, Retik
AB.Normal urodynamics in patients with bladder exstrophy: are they achievable? J Urol.
1999;162(3):844–5.
9. Harris KT, Villela NA, Alam R, Wu WJ, Artigas P, DiCarlo HN, Gearhart JP.The exstrophy
experience: a national survey assessing urinary continence, bladder management, and oncologic outcomes in adults. J Pediatr Urol. 2023;19(2):178.e1–7.
10. Sam CJ, Jagadeesan CT, Sen S, Arunachalam P, Appalaraju B, Das PT.Urinary tract infection in
pediatric patients on clean intermittent catheterization via a mitrofanoff port with reused catheters– any association with catheter sterility? J Indian Assoc Pediatr Surg. 2020;25(2):91–5.
11. Kisku S, Sen S, Karl S, Mathai J, Thomas RJ, Barla R.Bladder calculi in the augmented bladder: a follow-up study of 160 children and adolescents. J Pediatr Urol. 2015;11(2):66.e1–6.
377
Kidney andBladder Exstrophy:
Considerations andCaution
SangeetaHingorani
Introduction
The worldwide prevalence of chronic kidney disease (CKD) in children is 15–74.7
cases per million children [1]. The most common cause of CKD in children is congenital abnormalities of the kidney and urinary tract (CAKUT), which accounts for
29–40% of cases of CKD depending on region and database [2]. CKD is a state of
irreversible damage to the kidneys with reduction of kidney function and represents
a continuum of disease from mild kidney abnormalities with normal metabolic
function and solute handling to end-stage kidney disease (ESKD).
Given that CAKUT, which includes obstructive uropathy, neurogenic bladder,
and hypoplastic and dysplastic kidneys with or without vesicoureteric reux, is the
major cause of CKD in children, understanding the diagnosis, need for follow-up
and ongoing monitoring, and prevention of progression is important for all practitioners caring for these children. This chapter will focus primarily on the exstrophyepispadias complex, of which bladder exstrophy (BE) is the most common type,
accounting for 71–83% of cases [3]. These complex malformations involve the
lower abdominal wall, bladder, and pelvic oor and often necessitate multiple
reconstructive surgeries and procedures. Many of the procedures are designed to
obtain urinary continence, normal appearance of the genitalia, sexual function, and
to preserve kidney function. Though initial thoughts were that children with exstrophy had normal kidneys, as kidney function measured by serum creatinine was normal, we have now learned that CAKUT-associated abnormalities are increased in
prevalence in children with BE.Baseline abnormalities are then compounded by
23
S. Hingorani (*)
University of Washington School of Medicine, Department of Pediatrics,
Division of Nephrology, Seattle Children’s Hospital, Seattle, WA, USA
e-mail: sangeeta.hingorani@seattlechildrens.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_23
379
380
S. Hingorani
reconstructive surgeries and obstructive complications and urinary tract infections,
which can further impact an already abnormal kidney parenchyma [3].
Summary ofLiterature
There are few large studies of patients with BE evaluating long-term kidney function in a systematic and comprehensive way. And comparisons between studies are
also difcult given varying surgical techniques used and varying modalities to evaluate kidney function (Tables 23.1 and 23.2).
In a retrospective cross-sectional study from a single-referral center in Tel Aviv
of patients followed by pediatric urology from birth to the present time, investigators evaluated kidney function, including serum creatinine and urine protein to creatinine ratio; calculated an estimated glomerular ltration rate (GFR) using the
Schwartz formula or 24-hour urine collection for creatinine clearance; and measured blood pressure [3]. Imaging studies included a kidney ultrasound and DMSA
scan. Forty-three patients were included in this study, of whom 39 were diagnosed
with BE. Kidney evaluation occurred at a median of 9 years of age (range of
1–24years). Overall, 26 patients (60%) completed kidney evaluations. The median
estimated GFR was 127.5 mL/min/1.73 m2 with an interquartile range of
103–140/1.73m2; a decreased GFR was present in only 12% of patients. Elevated
blood pressures were recorded in 29% of patients at follow-up, and 36% (10) of
patients had albuminuria or proteinuria, and the latter were more common in patients
who had a bladder augmentation. Imaging studies revealed scarring in 61% of
patients evaluated, and abnormalities on ultrasound, including urinary tract dilatation, were seen in 46% of patients. The overall prevalence of any type of kidney
abnormality, whether structural or functional, was seen in 32 (74%) of patients
evaluated. Risk factors identied for scarring on DMSA scans were the presence of
urinary tract dilatation on US and persistent infections or a history of kidney stones.
None of the risk factors studied were associated with structural or functional
declines or changes in kidney function [3].
Another recent study by Joshi etal. of children with BE cared for as part of a
US-India Multi-Institutional Bladder Exstrophy Collaboration at a Civil Hospital in
Table 23.1 Normal glomerular ltration rate (GFR) in children and adolescents [12]
Age Mean GFR±SD (mL/min/1.73m2)
1week (males and females) 41±15
2–8weeks (males and females) 66±25
>8weeks (males and females) 96±22
2–12years (males and females) 133±27
13–21years (males) 140±30
13–21years (females) 126±22
23 Kidney andBladder Exstrophy: Considerations andCaution
381
Table 23.2
Authors and
year of
publication
Demaria
etal., 1979
Husmann
etal., 1988
Mesrobian
etal., 1988
Grady and
Mitchell
1999
Bolduc
etal., 2002
Gargollo
etal., 2008
Ebert etal.,
2009
Comparison of kidney outcomes from 1979 to 2021 [4]
Surgical
technique used
to repair
bladder
exstrophy
MSRE 22 (22
MSRE 68 (68
MSRE 103 (32
CPRE 24 (18
MSRE 57 (49
CPRE 32 (32
One stage
reconstruction
Sample
size
BE)
BE)
had BE
repair, 71
diverted)
BE,
6CE)
BE,
8CE)
BE)
17 (16
BE, 1 PE)
Measurement
of renal injury
(IVP, RUS,
VCUG,
DMSA, eGFR)
Imaging—IVP
Labs—
Creatinine
Imaging—IVP,
VCUG
Labs—
Creatinine
Imaging—IVP,
VCUG, and
RUS
Labs—
Creatinine
Imaging——
RUS, VCUG
Labs—
Creatinine
(only available
in 5/24 Pts)
Imaging—
RUS, VCUG,
DMSA
Imaging
only—RUS,
DMSA, VCUG
Imaging—
RUS, DMSA
Labs—eGFR
Mean
length of
follow-up
(Years)
8.7 3/22 (14%) had
12.7 8/68 (13%) had
8 6/24 (25%)
4 98% of the renal
4.5 14/57 (24%) had
5 9/32 (28%) had
18.7 4/17 (24%) had
Renal outcomes
renal ndings on
IVP
100% had normal
Creatinine
renal scarring
3/68 (4%) had
renal
insufciency
hydronephrosis
100% normal
Creatinine
units have no
evidence of
deterioration on
serial RUS
renal scarring
2/57 (3.5%) renal
insufciency
up to 4 episodes
of pyelonephritis
7/32 (22%) had
hydronephrosis
6 (19%) had
cortical defects
UTIs
3/17 (18%) had
unilateral smaller
kidney
100% normal
renal function
(continued)
382
S. Hingorani
Table 23.2
Authors and
year of
publication
Schaeffer
etal., 2012
Ellison
etal., 2017
Giron
etal., 2017
Sinatti
etal., 2021
BE bladder exstrophy, CE cloacal exstrophy, PE penile epispadias, MSRE modern staged repair of
exstrophy, CPRE complete primary repair of exstrophy
(continued)
Surgical
technique used
to repair
bladder
exstrophy
MSRE 53 (53
CPRE 30 (30
One stage
reconstruction
Multiple
techniques
(Single stage
and two stage
repair)
Sample
size
BE)
BE)
15 (15
BE)
48 (38
BE, 7 PE
and 3CE)
Measurement
of renal injury
(IVP, RUS,
VCUG,
DMSA, eGFR)
Imaging—RUS
Labs—eGFR
Imaging—RUS
Labs—eGFR
Imaging—
RUS, VCUG
Labs—
Creatinine
Imaging—
RUS, VCUG
Labs—eGFR
Mean
length of
follow-up
(Years)
15.1 1/53 (1.8%) had
9.7 16/30 (53%) had
10.3years 100% normal
18years 36/48 (75%) had
Renal outcomes
CKD stage 2
hydronephrosis
4/30 (13%) had
CKD stage 2
kidneys
100% normal
Creatinine
recurrent UTIs
2/48 (4%) had
hydronephrosis
1/48 (2%) had
CKD stage 2
Ahmedabad, India were prospectively followed from 2010 to 2020, reported more
extensively on kidney outcomes 1–10years after BE surgery [4]. Of the 72 patients
included in this study, 60 had either primary BE surgery or a redo of their BE surgery. Of these, 4 patients had an eGFR <90mL/min/1.73m2, and 36 had an eGFR
>120mL/min/1.73m2. Twenty children had kidney scarring seen on DMSA scans.
In this study, neither urinary reux nor the presence of kidney scars was associated
with a decline in eGFR after adjustment for age, sex, and duration of follow-up.
Additionally, abnormal DMSA scans were not associated with microalbuminuria or
urinary reux. This population had a surprisingly low rate of urinary tract infections, precluding analysis of any associations with exposures or outcomes [4].
A study of 42 patients born with BE between 1937 and 1968, of whom 25 were
contacted at a median of 50years (range 37–69years), found that 15 of 25 (60%) of
patients had normal creatinine levels or mild CKD based on eGFR with a mean
eGFR of 66mL/min/1.73m2 and 3 patients were on kidney replacement therapy:
(1) on hemodialysis and (2) had a kidney transplant (PMID 19136304). Childhood
surgical and clinical courses were reviewed, and the ESKD was thought to be from
progressive scarring of their upper tracts due to progressive hydronephrosis from
bladder outlet obstruction, kidney stones, and a history of febrile urinary tract
23 Kidney andBladder Exstrophy: Considerations andCaution
383
infections after closure. MRI imaging showed scarring in the kidneys of varying
degrees from mild to severe in 20 of the 25 patients. Dilatation of the ureter and/or
kidney pelvis was seen in 11 patients. Ten patients had recurrent kidney stones
throughout their adult life [5].
Smaller, single-center studies have reported varying degrees of kidney function, from normal in all 16 study patients with BE with a mean serum creatinine
of 0.85mg/dL (0.8–1.0) after a mean follow-up of 19.4years. Fourteen patients
had normal kidney US, while only 3 had a smaller kidney and stable DMSA
scan without any evidence of scarring [6]. Other studies have found that only 1
child of the 27 with normal GFR at the time of bladder neck reconstruction
developed stage 2 CKD after 10years of follow-up. Imaging studies in this
cohort of patients demonstrated mild hydronephrosis (2 patients), moderate (5
patients), and severe (2 patients), with the remaining US being normal. No correlation was found between the presence or severity of hydronephrosis and
eGFR [7]. In a study of 23 adult patients aged 18–57years who had exstrophyepispadias complex and presented to an adult urology practice, 10 of 23 were
noted to have CKD stage 2 or higher, though only one patient had CKD stage
5 and 7 had hydronephrosis on US.Most patients had a history of kidney stones
and pyelonephritis [8].
Nephrology Evaluation
The pediatric urologic patient may present to the nephrologist at any time during their clinical course. Ideally, those with a pre-natal diagnosis of bladder
exstrophy or CAKUT will be known to nephrology, or nephrology will be
involved early in their diagnosis and treatment course. Close communication
between urology and nephrology is essential to provide a collaborative approach
in the evaluation, prevention, and management of kidney disease. A thorough
history and physical examination with a particular focus on risk factors for preexisting kidney disease, birth weight, gestational age, family history of kidney
disease, dialysis, or transplants, and a review of systems elucidating kidney and
urinary symptoms (edema, dysuria, nocturia, enuresis, and hematuria) should be
performed. Also, a review of the patient’s urologic diagnosis, medications, past
and planned surgical treatment courses, history of urinary tract infections, and
kidney stones is essential to understanding risk factors for the development and
progression of CKD.Additional consideration should be given to whole genome
sequencing in patients with CAKUT, as approximately 25% of patients will
have an identied genetic mutation [9].
384
Screening and ongoing monitoring of kidney outcomes in patients with bladder exstrophy
Monitoring parameter
eGFR Serum creatinine
Kidney size, growth,
architecture
Albuminuria/
proteinuria Normal
albuminuria <30mg/g
creatinine
Normal proteinuria
<300mg/g creatinine
or <0.2
Growth Height and weight Yearly and/or at each visit Pediatrician,
Blood pressure Oscillometric BP or
Kidney stones Urinalysis; urine
Electrolyte
abnormalities
Prevention of kidney
injury and urinary
tract infections
Avoidance of
ibuprofen/NSAIDs
and other nephrotoxic
medications
Good voiding and
stooling habits
Consider prophylactic
antibiotics
Healthy lifestyle
habits
Monitoring studies Interval for monitoring
and cystatin C
Kidney ultrasound Baseline, annually
Urinalysis and spot
urine albumin/
protein to creatinine
ratio
manual BP and
ABPM when
>8years of age
calcium to
creatinine ratio;
24-hour urine stone
risk prole
Ultrasound or Fast
CT
Serum levels of Na,
K, Cl, HCO3,
glucose, calcium,
and phosphorus
Urinalyses and
urine cultures when
indicated
VCUG and
urodynamic studies
Nutrition and
activity counseling
Baseline, and then yearly Urology or
through 3years and then
every 3–5years based on
kidney function or other
complications;
recommended during
adolescence growth spurt
Yearly Urology or
At each visit, yearly Pediatrician,
If there is a suspicion of
stones
Baseline, yearly if
presence of CKD or
failure to gain weight and
grow
Baseline, yearly or when
needed
At each visit adjusted
based on weight, blood
pressure, albuminuria
S. Hingorani
Care provider
doing the
monitoring
nephrology
Urology or
nephrology
nephrology
urology,
nephrology
urology,
nephrology—24hour ABPM study
Urology or
nephrology
Pediatrician,
urology, or
nephrology
Pediatrician,
urology,
nephrology
Pediatrician at
well child visits,
urology and
nephrology