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Primary Bladder Exstrophy Closure in Neonates:
Challenging the Traditions
I. Mushtaq,* M. Garriboli,* N. Smeulders, A. Cherian, D. Desai, S. Eaton,
P. Duffy and P. Cuckow
From the Department of Pediatric Urology, Great Ormond Street Hospital for Children NHS Trust (IM, MG, NS, AC,
DD, PD, PC) and Department of Pediatric Surgery, UCL Institute of Child Health (MG, SE), London, United Kingdom
Purpose: We describe a novel approach to neonatal bladder exstrophy closure
that challenges the role of postoperative immobilization and pelvic osteotomy.
Materials and Methods: We reviewed the primary management of bladder
exstrophy at our institutions between 2007 and 2011. In particular we compared
postoperative management in the surgical ward using epidural analgesia to
muscle paralysis and ventilation in the intensive care unit. Clinical outcome
measures were time to full feed, length of stay, postoperative complications and
redo closure. Cost-effectiveness was also evaluated using hospital financial data.
Data are expressed as median (range). Significance was explored by Fisher exact
test and unpaired t-test.
Results: A total of 74 patients underwent primary closure without osteotomy.
Successful closure was achieved in 70 patients (95%). A total of 48 cases (65%)
were managed on the ward (group A) and 26 (35%) were transferred to the
intensive care unit (group B). The 2 groups were homogeneous for gestational
age (median 39 weeks, range 27 to 41) and age at closure (3 days, 1 to 152).
Complications requiring surgical treatment were noted in 4 patients (8.3%) in
group A and 3 (11.5%) in group B (p ¼0.609). Length of stay was significantly
shorter for the group managed on the ward (11 vs 18 days, p <0.0001). Median
costs were $42,732 for patients admitted to the intensive care unit and $16,214
for those admitted directly to the surgical ward (p <0.0001).
Conclusions: Primary closure of bladder exstrophy without lower limb immobi-
lization and osteotomy is feasible. Postoperative care on the surgical ward using
epidural analgesia results in shorter hospitalization.
Key Words: bladder exstrophy, osteotomy, urologic surgical procedures
MANAGEMENT of bladder exstrophy
remains one of the most significant
challenges in pediatric urology.
1
Care
of these patients is becoming central-
ized to a smaller number of centers
worldwide (2 centers in England),
although the techniques used and
their results vary widely.
2e4
It is universally agreed that suc-
cessful initial bladder closure is the
cornerstone of development of bladder
capacity and continence.
5
A range of
techniques is used to achieve suc-
cessful primary closure supported
by pelvic immobilization with or
without pelvic osteotomy. The most
common techniques described for
postoperative pelvic immobilization
are Bryant traction, modified Bryant
traction, Buck traction and spica
Abbreviations
and Acronyms
BE ¼bladder exstrophy
CBEX ¼classic bladder exstrophy
ICU ¼intensive care unit
Accepted for publication July 15, 2013.
Supported by the Fondazione Alberto
Mascherpa Onlus and OBM Onlus (MG), and the
Great Ormond Street Hospital Charity (SE).
* Equal study contribution.
See Editorial on page 13.
0022-5347/14/1911-0193/0
THE JOURNAL OF UROLOGY
®
©2014 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION AND RESEARCH,INC.
http://dx.doi.org/10.1016/j.juro.2013.07.020
Vol. 191, 193-198, January 2014
Printed in U.S.A. www.jurology.com j193
casting.
6,7
At those centers where osteotomy is used
an external fixation device may also be applied.
Most of these techniques require prolonged hospi-
talization, often longer than 1 month, and still
might not result in a favorable outcome.
8e10
In addition to clinical outcomes, evidence of cost-
effectiveness in case management is becoming a
fundamental part of care and is now a crucial point
in the evaluation of therapy and management. We
describe an approach to primary bladder exstrophy
closure that does not require pelvic osteotomy or
prolonged pelvic immobilization.
MATERIALS AND METHODS
We retrospectively studied all new cases of bladder ex-
strophy managed by primary closure at our institution
between January 2007 and December 2011. Only patients
with CBEX were included. Patients with CBEX who un-
derwent primary closure elsewhere were excluded from
the study. Surgical management of bladder exstrophy
consists of initial bladder closure in the neonatal period. A
radical soft tissue procedure is performed at age 9 to 12
months. This second stage, commonly referred to as the
Kelly procedure, is performed in all cases regardless of
bladder capacity, and incorporates reconstruction of the
bladder neck and epispadias reconstruction. All patients
were followed for a minimum of 1 year, and complete
records were available for all.
The medical records for this cohort were reviewed and
data were collected regarding patient demographics, sur-
gical findings, postoperative analgesia techniques, loca-
tion of postoperative care (urology ward/intensive care
unit), duration of hospital stay, time required to establish
oral feeding, epidural catheter related complications,
surgical complications and need for redo closure. Suc-
cessful exstrophy closure was defined as an intact repair
that did not require a redo closure.
Primary closure was performed by any member of our
team of 4 pediatric urologists. Anesthesia was adminis-
tered by a pediatric anesthetist with expertise in neonatal
epidural analgesia.
A similar surgical technique was used by all 4 sur-
geons. This procedure consists of mobilization and sepa-
ration of the bladder plate from the rectus sheath and
umbilicus. Dissection of the bladder plate ends distally
adjacent to the verumontanum, and the umbilicus is
excised in all cases. Ureteral stents (4Fr to 6Fr) are placed
routinely, and the bladder is closed with interrupted
absorbable monofilament sutures in a single layer. A sil-
icone urethral stent is left in situ. The rectus muscle is
closed with interrupted absorbable sutures, and the pubis
is reapproximated with at least 2 interpubic sutures. The
skin is closed in 2 layers using interrupted absorbable
sutures. We no longer use the frog plaster, mermaid
dressings or any form of lower limb traction.
11
Cases were managed postoperatively along 1 of 2
clearly defined pathways. The first pathway involves de-
livery of postoperative analgesia via an epidural catheter
placed at surgical closure with the patient returning to
the surgical ward. In addition, some patients required
nurse controlled analgesia. Patients were allowed to feed
orally immediately postoperatively, and breastfeeding
was actively encouraged. No form of pelvic immobilization
was used. The second pathway involved elective paralysis
and ventilation in the ICU for a median of 7 days (mean
8.5), followed by return to the surgical ward. Apart from
muscle paralysis, no additional pelvic immobilization was
used in this group. The second pathway was chosen based
on individual surgeon preference, closure under excessive
tension or failure to insert an epidural catheter.
Postoperative analgesia was managed by a dedicated
acute pain team that uses the FLACC (Face, Legs, Ac-
tivity, Cry, Consolability) and COMFORT scales for pain
assessment to maintain an adequate level of analgesia for
infants admitted to the ward and neonatal ICU, respec-
tively.
12,13
Urinary drainage in both groups was achieved
with ureteral stents, which were removed on post-
operative day 7, and a urethral stent, which was allowed
to dislodge spontaneously after postoperative day 7. If the
urethral stent had not dislodged by postoperative day 14,
it was actively removed. No patient had a suprapubic tube
or wound drain placed. Patients were discharged home
once the ureteral stents were removed, oral feeding was
established and surgical site healing was deemed
satisfactory.
Treatment dose antibiotics were administered to all
patients for 5 to 7 days. These were delivered initially by
intravenous route and then orally, with the usual choice
of antibiotic being amoxicillin/clavulanic acid. In addition,
patients were given oral antifungal prophylaxis for the
length of time that the ureteral stents were in situ.
Antibiotic prophylaxis was maintained for a minimum of 3
months. No patient received anticholinergic medication.
Outcome measures evaluated included postoperative
complications and requirement for redo closure. Data are
expressed as median (range). Fisher exact test was used
to compare proportions, while unpaired t-test was used to
compare normally distributed data, with p <0.05 being
considered significant.
Economic data were derived from data collected retro-
spectively from individual patient records. An economic
evaluation was performed based on actual time in theater,
days in ICU and days in surgical ward. Costs were
calculated using the template of the National Commis-
sioning Group costs for bladder exstrophy.
Economic outcome was calculated as the direct costs
associated with in-hospital treatment (primary admission
plus any readmission). The overall cost was subdivided
into the categories 1) operating theater, 2) ward/ICU, 3)
medical staff and 4) nonmedical staff (clinical nurse spe-
cialists, nurses, administrative workers).
RESULTS
A total of 74 neonates with CBEX were treated be-
tween January 2007 and December 2011. Primary
closure without osteotomy was performed in all
patients and was successful in 70 (95%). Those with
failed primary closure underwent redo closure
with bilateral pelvic osteotomy at age 4 months
(3 patients) or 8 months (1). These patients had
194 BLADDER EXSTROPHY CLOSURE IN NEONATES
undergone initial closure between days 2 and 4 of
life. Patient demographics are given in table 1.
Median gestational age was 39 weeks (range 27 to
41), and 42 patients (57%) were male.
Median age at closure was 3 days (range 1 to
152). All patients underwent primary closure
regardless of size of bladder plate or degree of pubic
diastasis. The pubis was approximated with
absorbable sutures in all patients without pelvic
osteotomy. Of the cohort 48 patients (65%) returned
postoperatively to the surgical ward with an
indwelling epidural catheter. A continuous local
anesthetic infusion was maintained for a median of
4 days (range 1 to 6) to deliver local analgesia. In
some patients this treatment was supplemented
with nurse controlled morphine infusion. The aim of
this strategy was to provide adequate analgesia
without sedation, thereby allowing for an early re-
turn to oral feeding, and allowing parents to nurse
and comfort their infant. No patient was placed in
any form of lower limb traction. Full oral feeding
was established at a median of 4.5 days following
surgical closure (table 2). Median hospital stay was
13.5 days. One patient remained in the hospital for
41 days due to social reasons.
Epidural related complications occurred in 9 pa-
tients (19.5%). Complications included transient
erythema, swelling and positive bacteriological
swab from the epidural site. No patient required
any specific treatment, and there were no clin-
ical sequelae.
A total of 26 patients (35%) were transferred to
the ICU immediately following surgery for elective
paralysis and ventilation. The indication for elective
ventilation was a tight approximation of the pubis
in 4 patients, surgeon preference in 21 and failure to
insert an epidural catheter in 1. Median stay in the
ICU was 7 days (mean 8.5), after which patients
returned to the general surgical ward. Oral feeding
was fully established by postoperative day 10, and
the majority of patients (89%) were discharged from
the hospital within 3 weeks of surgery.
Of the cohort 4 patients (5%) required a redo
closure. These redo procedures were divided equally
between the 2 treatment pathways and did not
include the 4 ICU cases with tight reapproximation
of the pubis. No evident cause of failure was iden-
tified. Other complications requiring surgical
treatment occurred in 8 children (10.8%) and
included bladder prolapse managed by laparoscopic
cystopexy in 1, urethral stenosis that resulted in
bladder rupture in 1 and urethral stenosis that
required dilation under general anesthesia in 6.
Comparing the 2 groups of patients, there was no
significant difference in the rate of complications or
redo surgery (Fisher exact test p ¼0.6). Time
required to reach full oral feed (3 days) and length of
stay (11 days) were significantly less for patients
admitted to the surgical ward compared to patients
electively ventilated in the ICU (11 and 18 days,
respectively, unpaired t-test p <0.0001).
Median costs, including readmission costs, for
patients admitted to the ICU were $42,732 (range
$30,395 to $87,532), compared to $16,214 ($10,630
to $46,675) for those admitted directly to the sur-
gical ward. This difference was highly significant
(p <0.0001, see figure).
DISCUSSION
Achieving successful primary closure of bladder
exstrophy remains one of the most elusive chal-
lenges in pediatric urology (table 3). The factors that
have been implicated in success include age at
closure, size of bladder plate, width of pubic dias-
tasis, use of pelvic osteotomy, osteotomy technique
and use of lower limb immobilization. The conse-
quences of a failed primary closure are serious not
only in terms of the requirement for a redo closure,
but also in terms of the potential negative impact on
Table 2. Complications, outcomes and costs
Group A Group B Totals/Av p Value
No. complications requiring surgery: 5 (10.4%) 3 (11.5%) 8 (10.8%) 0.583
Bladder rupture 1 0 1
Bladder prolapse 0 1 1
Urethral stenosis 4 2 6
No. redo closure (%) 2 (4.2) 2 (7.7) 4 (5) 0.609
Median days from closure to full oral feeding (range) 3 (2e13) 11 (6e27) 4.5 (2e27) <0.0001
Median days length of stay (range) 11 (6e17) 18 (14e41) 13.5 (6e41) <0.0001
Median cost of admission $16,214 $42,732 $21,558 <0.0001
No patient in either group died.
Table 1. Patient characteristics
Group A Group B Totals/Av p Value
No. prenatal diagnosis (%) 11 (23) 9 (35) 20 (27) 0.2891
Median wks gestational
age (range)
40 (27e41) 39 (28e41) 39 (27e41) 0.6267
No. males (%) 26 (54) 16 (62) 42 (57) 0.1114
Median days age at
closure (range)
3(1e87) 2.5 (1e152) 3 (1e152) 0.6594
BLADDER EXSTROPHY CLOSURE IN NEONATES 195
the future development of bladder capacity and
continence. For this reason elaborate and regi-
mented plans for postoperative management have
been described.
14
In a recent study Stec et al described a success
rate for primary closure of 95% among a cohort of
65 patients undergoing closure at their institu-
tion.
15
A third of the patients underwent pelvic
osteotomy at primary closure, and all patients
were maintained in lower limb traction for an
average of 30 days. Postoperative pain control was
achieved by epidural analgesia, supplemented by
an intravenous opiate infusion in 37% of patients.
Intravenous benzodiazepines, nonsteroidal medica-
tions and oral opiates were administered for sup-
plementary sedation. Most patients (90%) required
a stay in the ICU, with approximately half requiring
ventilation.
We reserve the use of pelvic osteotomy for those
patients who require redo closure and some infants
older than 6 months at primary closure. In the last
decade pelvic osteotomies have not been used
routinely in primary closure of bladder exstrophy,
and pubic approximation has been achieved in all
cases with an interpubic suture. Approximately a
third of our patients were maintained in the ICU for
a median of 7 days. In 4 patients the abdominal wall
closure was considered to be under significant
tension, and this was the indication for elective
paralysis and ventilation. In the remaining patients
in this group the decision to immobilize in the ICU
was based on surgeon preference rather than degree
of tension in the abdominal wall closure. Therefore,
the 2 groups of patients in this cohort (ICU vs ward)
are fairly comparable in terms of patient character-
istics (table 1). Thus, we can conclude that a suc-
cessful bladder exstrophy closure can be achieved in
the majority of cases without any form of pelvic or
lower limb immobilization. Our ward based
approach has a singular overwhelming advantage, ie
that the infant can be cared for on the general ward
in close proximity to the parents and can be breast-
fed, which facilitates early onset of intestinal
motility and permits good parental bonding with
the child.
The use of epidural analgesia allows delivery of
local analgesia to the surgical site, minimizing the
use of intravenous and oral opiate analgesia.
16
Therefore, the patient is more alert and comfort-
able and able to interact with the parents. The
minimal use of opiates may also decrease gastroin-
testinal motility disturbances and adverse effects
on respiration. Our data reveal that the majority
of patients maintained on the ward following sur-
gery establish full oral feeding by postoperative
day 3. Median length of hospital stay for the entire
cohort was 13.5 days, compared to 11 days for
those patients who returned to the ward following
closure.
Closure without routine admission to the inten-
sive care unit and postoperative pelvic immobiliza-
tion were cost effective in our study, as good
equivalent outcomes were achieved at a signifi-
cantly decreased cost. Transfer to the ICU may be
beneficial in cases with considerable tension in the
abdominal wall closure and/or pubic reapprox-
imation. However, the findings of this study have
resulted in a reduction in the number of cases
managed in the ICU.
We describe the postoperative management of
neonates subjected to primary closure of bladder
exstrophy in a high volume specialized unit. The
concentrated experience of such cases is likely to
Table 3. Literature review
Present Series Stec et al
15
Schaeffer et al
9
Gargollo et al
17
Technique Primary closure Primary closure Primary closure Complete primary repair
No. pts 74 65 194 32
Study length (yrs) 5 30 23 14
No. pts treated yearly 14.8 2.2 8.4 2.3
No. redo procedures (%) 4 (5) 3 (5) 6 (3) 1 (3)
No. other complications (%) 8 (10.8) Not reported 21 (10.8) 12 (38)
Followup (mos) 12e72 Greater than 12 Not reported 3e156
Median length of stay (days) 13.5 35.8 Not reported Not reported
Comparison of costs (British pounds sterling) between group A
(surgical ward) and group B (ICU).
196 BLADDER EXSTROPHY CLOSURE IN NEONATES
have a role in the favorable outcomes. The surgical
technique used for closure is conventional, and good
urine drainage was achieved with ureteral and
urethral catheters. The most important de-
terminants of success are effective local analgesia,
minimal sedation, early onset of enteral feeding,
and close parental contact and bonding.
CONCLUSIONS
Our results challenge conventional thinking in
neonatal exstrophy closure and demonstrate that
primary closure can be achieved without pelvic
osteotomy or pelvic/lower limb immobilization. Most
of all, we recommend epidural analgesia and ward
based care in these cases.
REFERENCES
1. Ebert AK, Reutter H, Ludwig M et al: The
exstrophy-epispadias complex. Orphanet J Rare
Dis 2009; 4: 23.
2. Grady RW and Mitchell ME: Complete primary
repair of exstrophy. J Urol 1999; 162: 1415.
3. Pippi-Salle JL and Chan PT: One stage bladder
exstrophy and epispadias repair in newborn
male. Can J Urol 1999; 6: 757.
4. Baird AD, Nelson CP and Gearhart JP: Modern
staged repair of bladder exstrophy: a contem-
porary series. J Pediatr Urol 2007; 3: 311.
5. Woodhouse CR, North AC and Gearhart JP:
Standing the test of time: long-term outcome of
reconstruction of the exstrophy bladder. World J
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6. Baird AD, Sponseller PD and Gearhart JP: The
place of pelvic osteotomy in the modern era of
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2005; 1: 31.
7. Shnorhavorian M, Song K, Zamilpa I et al: Spica
casting compared to Bryant’s traction after
complete primary repair of exstrophy: safe and
effective in a longitudinal cohort study. J Urol
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8. Meldrum KK, Baird AD and Gearhart JP: Pelvic
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cations of primary closure of classic bladder
exstrophy. J Urol, suppl., 2008; 180: 1671.
10. Purves JT and Gearhart JP: Complications of
radical soft-tissue mobilization procedure as a
primary closure of exstrophy. J Pediatr Urol 2008;
4: 65.
11. Nicholls G and Duffy PG: Anatomical correction
of the exstrophy-epispadias complex: analysis of
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12. Merkel SI, Voepel-Lewis T, Shayevitz JR et al:
The FLACC: a behavioral scale for scoring post-
operative pain in young children. Pediatr Nurs
1997; 23: 293.
13. Association of Paediatric Anaesthetists of Great
Britain and Ireland: Good practice in post-
operative and procedural pain management, 2nd
edition. Paediatr Anaesth, suppl., 2012; 22: 1.
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of bladder exstrophy in the modern era:
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15. Stec AA, Baradaran N, Schaeffer A et al:
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EDITORIAL COMMENTS
A challenge in sports is a triathlon. A challenge in
medicine should allegorically include 3 disciplines, ie
a well realized idea, and early and late results. Many
surgeons today still do not believe that neonatal
exstrophy closure can successfully be achieved
without osteotomy. Additionally, lack of osteotomy
is blamed for recurrent bladder dehiscence and
impaired continence results. However, it is known
that symphysis diastasis recurs after all commonly
used pelvic closure techniques.
1
Exstrophy closure
has been done at age 6 to 8 weeks without osteotomy
since 1983 at our institution. We have not had
any failures of initial closure, and few cases have
needed minor additional surgery at that stage.
However, on long-term followup symphysis diastasis
is comparable to available post-osteotomy data.
2
So
what is even more important than osteotomy or
immobilization? Adequate pain management with
epidural catheter is a mainstay of care (reference 16
in article). This factor improves surgical outcome
and ensures stabilization of the parent-child rela-
tionship, which is already endangered by early sur-
gery, and may have lifelong consequences for the
mental and physical well-being of these children.
A. K. Ebert
Department of Urology and Pediatric Urology
Ulm University
Ulm
and
W. H. R€
osch
Department of Pediatric Urology
University Medical Center Regensburg
Regensburg, Germany
BLADDER EXSTROPHY CLOSURE IN NEONATES 197
REFERENCES
1. Satsuma S, Kobayashi D, Yoshiya S et al: Comparison of posterior and anterior pelvic osteotomy for bladder exstrophy complex. J Pediatr Orthop B 2006; 15: 141.
2. Ebert A, Kertai M, Hirschfelder H et al: Morphologic and functional hip long-term results after exstrophy repair. J Pediatr Urol, suppl., 2012; 8: S06.
This intriguing study challenges some of the tradi-
tional concepts regarding BE closure. The authors
make a compelling case for regionalization and
referral of uncommon and rare anomalies such as
BE to a limited number of centers. Several other
studies have also found higher success and lower
morbidity rates for procedures performed at high
volume centers.
The authors have convincingly shown that
traditional prolonged postoperative immobilization
and urine drainage by cystostomy tube for 4 weeks
are unnecessary. Neonatal classic bladder ex-
strophy closure was successfully achieved in 95% of
cases without any form of immobilization. They
have also demonstrated the feasibility of a short
period of urine drainage by ureteral stents for
7 days and a urethral catheter for 7 days with a
maximum of 14 days. Furthermore, postoperative
care in a pediatric unit with continuous analgesic
infusion through an epidural catheter and early
oral feeding were equally successful but much more
cost effective than postoperative admission to an
ICU. However, one cannot emphasize enough the
importance of postoperative pain control by a dedi-
cated pain management team.
This novel approach is a valuable contribution.
The authors have confirmed my belief that the
majority of neonatal bladder exstrophy closures
can be accomplished without osteotomy. During the
last 3 decades I observed 1 failure (breakdown)
among 106 bladder exstrophy closures without
osteotomy, which I reserved for older children
and reclosure.
I plan to adopt the policy of the authors in my
next neonatal BE closure, namely no postoperative
immobilization, and brief urinary drainage. How-
ever, if the closure appears to be under tension, I
will still use elective muscle paralysis and ventila-
tion with admission to the ICU for these unusual
cases. This article should change the postoperative
course for most neonatal BE closures.
Moneer K. Hanna
Department of Urology
New York-Presbyterian Hospital/Weill Cornell Medical College
New York, New York
REPLY BY AUTHORS
We are grateful for the encouraging comments and
personal perspectives, which provide further evi-
dence of the questionable role of pelvic osteotomy
and prolonged pelvic immobilization in primary
neonatal exstrophy closure. However, the excellent
results achieved at high volume centers might not
be reproducible at centers that manage only 1 or
2 bladder exstrophy cases yearly. In such circum-
stances a “belt and braces” approach would not seem
unreasonable.
198 BLADDER EXSTROPHY CLOSURE IN NEONATES