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To describe a novel approach to neonatal bladder exstrophy closure, which challenges the role of postoperative immobilisation and pelvic osteotomies. We reviewed the primary management of bladder exstrophy between 2007 and 2011. In particular we compared the post-operative management on the surgical ward, using epidural analgesia, with muscle paralysis and ventilation on the intensive care unit (ICU). Clinical outcome measures were: time to full feed, length of stay, postoperative complications and re-do closure. Cost-effectiveness has also been evaluated using hospital financial data. Data are expressed as median (range). Significance was explored by Fisher exact test and unpaired t test. 74 patients underwent primary closure without osteotomies. A successful closure was achieved in 70 patients (95%). 48 babies (65%) were managed on the ward (Group A), 26 were transferred to ICU (Group B). The two groups were homogeneous for gestational age: 39 weeks (27-41) and age at closure: 3 days (1-152). Complications requiring surgical treatment occurred in 4 children in Group A and 3 children in Group B (8.3% and 11.5%, p=0.609). Length of stay was significantly shorter for the group managed on the ward (11 days vs 18 days, p < 0.0001). Costs for patients admitted to ICU were median £27,025 compared with those admitted directly to the surgical ward £10,254 (p<0.0001). Primary closure of bladder exstrophy without lower limb immobilisation and osteotomies is feasible. Post-operative care on the surgical ward under epidural analgesia resulted in shorter hospitalisation.
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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:
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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
bladder exstrophy reconstruction. J Pediatr Urol
2005; 1: 31.
7. Shnorhavorian M, Song K, Zamilpa I et al: Spica
casting compared to Bryants traction after
complete primary repair of exstrophy: safe and
effective in a longitudinal cohort study. J Urol
2010; 184: 669.
8. Meldrum KK, Baird AD and Gearhart JP: Pelvic
and extremity immobilization after bladder ex-
strophy closure: complications and impact on
success. Urology 2003; 62: 1109.
9. Schaeffer AJ, Purves JT, King JA et al: Compli-
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
34 patients. Br J Urol 1998; 82: 865.
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.
14. Nelson CP, Dunn RL, Wei JT et al: Surgical repair
of bladder exstrophy in the modern era:
contemporary practice patterns and the role of
hospital case volume. J Urol 2005; 174: 1099.
15. Stec AA, Baradaran N, Schaeffer A et al:
The modern staged repair of classic bladder
exstrophy: a detailed postoperative management
strategy for primary bladder closure. J Pediatr
Urol 2012; 8: 549.
16. Rubenwolf PC, Koller B, Rubben I et al: Periop-
erative pain management in major reconstructive
surgery in pediatric urology: a plea for contin-
uous epidural anesthesia. Urologe A 2011; 50:
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17. Gargollo PC, Borer JG, Diamond DA et al: Pro-
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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
Article
Introduction Previous assumptions suggested that the technique of approximation without osteotomy in primary exstrophy repair (PER) could only be applied in newborns and anticipated poorer outcomes. Recent studies indicated that this technique can be successfully executed not only in immediate PER but also yields favorable long-term results. Therefore, we evaluated and compared the orthopaedic and radiological long-term outcomes after pubic symphysis approximation without osteotomy in immediate and delayed PER. Methods From March 2018 to December 2020, individuals with PER and approximation of the symphysis without osteotomy were recruited. Patients <12 years and with a history of orthopaedic surgery of the bony pelvis were excluded. Orthopaedic examinations and magnetic resonance imaging (MRI) of the bony pelvis including the hip joints were performed and pubic diastasis, the acetabulum angle (ACA), and the center-edge angle (CEA) were evaluated. Results Twenty-nine patients were included, 11 of them had an immediate and 18 had a delayed PER. Between the two groups, no significant differences could be observed concerning hip pain (p = 0.419), mobility impairment (p = 0.543), sports impairment (p = 0.543), hip impingement (p = 1.000), leg length discrepancy (p = 0.505), and width of the pubic diastasis as measured by MRI (p = 0.401). There were also no significant differences with regard to CEA right (median 30 degrees, p = 0.976), CEA left (median 31.5 degrees, p = 0.420), ACA right (median 19 degrees, p = 0.382), and ACA left (median 17 degrees, p = 0.880). Conclusion There were no significant differences in clinical orthopaedic or radiological long-term outcomes between bladder exstrophy patients after immediate and delayed bladder closure with symphysis approximation without osteotomy. Establishing core outcome sets is essential to get robust and comparable results, further advancing and substantiating our initial insights.
Article
Objective: To review the outcomes of classic bladder exstrophy closure without the use of osteotomy or lower extremity/pelvic immobilization. Study design: A prospectively maintained institutional approved exstrophy-epispadias complex database of 1487 patients was reviewed for patients with CBE who had undergone closure without osteotomy nor immobilization. All patients were referred to the authors' institution for reconstruction later in life or for failed closure. Results: Of a total of 1016 CBE patients, 56 closure events were identified that met inclusion with a total of 47 unique patients. 38 closures were completed prior to 1990 (67.9%). 45 closure events developed eventual failure (45/56, 80.4%) (Table 1). 13 closure events were secondary closures (13/56, 23.2%). The primary closure failure rate was 83.7% (36/43) while the secondary closure failure rate was 69.2% (9/13). Failures were attributed to one or more of: dehiscence, bladder prolapse, and vesicocutaneous fistula (25/45, 55.6%) (23/45, 51.1%) (6/45, 13.3%) respectively. 37 patients developed social continence (37/47, 78.7%), while only 8 patients developed spontaneous voided continence (7/47,17.0%) (Table 2). The most common methods of voiding were continent catheterizable channels (25/47, 53.2%) of which all were socially continent. Conclusion: These results illustrate the critical role osteotomy and post-operative immobilization can play in both primary and secondary exstrophy closure. While this is a historical case series, the authors believe that these results remain relevant to contemporary exstrophy surgeons.
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Exstrophy-epispadias complex (EEC) represents a spectrum of genitourinary malformations ranging in severity from epispadias (E) to classical bladder exstrophy (CEB) and exstrophy of the cloaca (EC). Depending on severity, EEC may involve the urinary system, musculoskeletal system, pelvis, pelvic floor, abdominal wall, genitalia, and sometimes the spine and anus. Prevalence at birth for the whole spectrum is reported at 1/10,000, ranging from 1/30,000 for CEB to 1/200,000 for EC, with an overall greater proportion of affected males. EEC is characterized by a visible defect of the lower abdominal wall, either with an evaginated bladder plate (CEB), or with an open urethral plate in males or a cleft in females (E). In CE, two exstrophied hemibladders, as well as omphalocele, an imperforate anus and spinal defects, can be seen after birth. EEC results from mechanical disruption or enlargement of the cloacal membrane; the timing of the rupture determines the severity of the malformation. The underlying cause remains unknown: both genetic and environmental factors are likely to play a role in the etiology of EEC. Diagnosis at birth is made on the basis of the clinical presentation but EEC may be detected prenatally by ultrasound from repeated non-visualization of a normally filled fetal bladder. Counseling should be provided to parents but, due to a favorable outcome, termination of the pregnancy is no longer recommended. Management is primarily surgical, with the main aims of obtaining secure abdominal wall closure, achieving urinary continence with preservation of renal function, and, finally, adequate cosmetic and functional genital reconstruction. Several methods for bladder reconstruction with creation of an outlet resistance during the newborn period are favored worldwide. Removal of the bladder template with complete urinary diversion to a rectal reservoir can be an alternative. After reconstructive surgery of the bladder, continence rates of about 80% are expected during childhood. Additional surgery might be needed to optimize bladder storage and emptying function. In cases of final reconstruction failure, urinary diversion should be undertaken. In puberty, genital and reproductive function are important issues. Psychosocial and psychosexual outcome depend on long-term multidisciplinary care to facilitate an adequate quality of life.
Article
Introduction Abnormalities of the bony pelvis in exstrophy-epispadias complex (EEC) and their possible relation to hip disease are well described. However, there is a lack of information about long-term orthopedic consequences and hip function in patients with EEC. Therefore, we investigated clinical and radiological results in an EEC patient cohort after long-term follow-up. Patients and Methods We conducted a cross-sectional study using standardized radiography, clinical investigation, and the Harris hip score. Seventeen postpuberty consecutive unselected EEC patients (3 female, 14 male; mean age 18.2 years) that presented to our clinic due to urological procedures or routine check-up from 2010 to 2011 were included. All had undergone symphysis approximation with a traction bandage without osteotomy in early childhood. Radiological analysis was conducted offline by two independent investigators. Results Radiological analysis showed a mean pubic diastasis of 5.1 cm (range 2.8–8.5 cm). Borderline hip dysplasia was present in four patients, one of them having had co-occurring developmental hip dysplasia in previous history. No severe dysplasia, subluxation, or luxation of the hip was found; however, one patient showed early hip arthrosis. Clinical examination revealed no relevant restriction of range of motion, although rotation and abduction were slightly altered in five patients. None of the EEC patients complained about pain or restriction in sports or daily activities. Harris hip score was perfect for all but one study participants. Conclusion Despite EEC-specific hip morphology, long-term hip function is not impaired in patients after symphyseal approximation without osteotomy in the newborn period. The symphysis diastasis after this procedure is comparable to available postosteotomy data. The large majority of EEC patients did not show dysplastic or degenerative hip disease. Functional hip score results confirmed reasonable age-related hip function in nearly all examined patients. However, postnatal ultrasound hip screening is recommended to prevent and adequately treat potential co-occurring developmental hip dysplasia.
Article
Regionale Anästhesieverfahren sind ein essentieller Bestandteil der modernen Kinderanästhesie. In unserer Abteilung ist die kontinuierliche lumbale Periduralanästhesie (PDA) ein etabliertes Verfahren im perioperativen Schmerzmanagement mittlerer und großer Eingriffe am unteren Harntrakt. Ziel der vorliegenden Arbeit war es, die PDA im Säuglings- und Kindesalter hinsichtlich ihrer Effizienz, Sicherheit und möglicher Vorzüge gegenüber der rein systemischen perioperativen Analgesie zu analysieren. Von 21 Säuglingen, die wegen einer kongenitalen Blasenekstrophie primär plastisch-rekonstruktiv versorgt wurden, erhielten 15 präoperativ eine PDA; 6 Kinder ohne PDA dienten als Vergleichsgruppe. Retrospektiv wurden der perioperative Analgetikabedarf, Intensivpflichtigkeit, Nachbeatmungsdauer, Darmmotilität, der Verbrauch an Anticholinergika sowie PDA-assoziierte Komplikationen zwischen beiden Gruppen verglichen. Kinder mit kombinierter Allgemeinanästhesie und PDA hatten einen um das 6- bis 10-fache niedrigeren intra- bzw. postoperativen Opiatbedarf, konnten zügiger extubiert (59 vs. 210 min) und früher von der Intensiv- auf die Normalstation verlegt werden (1,1 vs. 1,8 Tage). Der postoperative Verbrauch an Anticholinergika lag um 50% niedriger als bei Kindern ohne PDA. PDA-assoziierte Komplikationen traten nicht auf. Die lumbale PDA stellt im perioperativen Narkose- und Schmerzmanagement bei mittleren und großen plastisch-rekonstruktiven kinderurologischen Eingriffen im Kindesalter ein analgetisch hocheffektives und sicheres Verfahren dar. Durch den signifikant reduzierten Bedarf an Anästhetika und Analgetika begünstigt die PDA eine zeitnahe Extubation mit allen weiteren Vorteilen der postoperativen Versorgung.
Article
Successful primary bladder closure of classic bladder exstrophy sets the stage for development of adequate bladder capacity and eventual voided continence. The postoperative pathway following primary bladder closure at the authors' institution is quantitatively and qualitatively detailed. Sixty-five consecutive newborns (47 male) undergoing primary closure of classic bladder exstrophy were identified and data were extracted relating to immediate postoperative care. Overall success rate was utilized to validate the pathway. Mean age at time of primary closure was 4.6 days and mean hospital stay was 35.8 days. Osteotomy was performed in 19 patients (mean age 8.8 days), and was not required in 39 infants (mean age 2.9 days). All patients were immobilized for 4 weeks. Tunneled epidural analgesia was employed in 61/65 patients. All patients had ureteral catheters and a suprapubic tube, along with a comprehensive antibiotic regimen. Postoperative total parenteral nutrition was commonly administered, and enteral feedings started around day 4.6. Our success rate of primary closure was 95.4%. A detailed and regimented plan for bladder drainage, immobilization, pain control, nutrition, antimicrobial prophylaxis, and adequate healing time is a cornerstone for the postoperative management of the primary closure of bladder exstrophy.
Article
The radical soft-tissue mobilization procedure was developed as a component of the staged closure of classical bladder exstrophy to improve continency rates without having to perform pelvic osteotomies. The authors describe complications following this procedure and discuss possible etiologies and subsequent management. We extracted from an institutionally approved exstrophy database the records of patients evaluated for complications following radical soft-tissue mobilization repair from 1999 to 2002. Four patients were referred to our institution following closure of exstrophy with the radical soft-tissue mobilization technique; two boys and two girls. Complications included ischemic penile injuries in both males, failed exstrophy closure in one female, incontinence with need for bladder neck transection and diversion in two patients, and upper tract deterioration in two patients of whom one required cystectomy and incontinent diversion. Omission of osteotomies when employing the radical soft-tissue mobilization repair appears to result in complications that could otherwise be prevented. Additionally, the complex dissection of the pelvic musculature, innervation and vasculature performed during radical mobilization has great potential to injure the pelvic structures and genitalia, as has been seen with the cases presented herein.
Article
Regional analgesia is firmly established in modern pediatric anesthetic practice and its popularity continues to grow. In our department continuous epidural anesthesia (CEA) is a frequently used technique of pain management following major reconstructive procedures of the lower urinary tract. The aim of this study was to investigate the efficacy, safety, and potential benefits of CEA over standard analgesics.We retrospectively reviewed the records of 21 infants who underwent single-stage bladder exstrophy repair in our department. In 15 children an epidural catheter was placed preoperatively for CEA; 6 patients treated without CEA served as controls. Total doses of narcotics and analgesics, length of intensive care unit (ICU) stay and ventilatory assistance, time to first bowel activity, anticholinergic requirements, and CEA-related side effects were documented and compared for both groups.Children given epidural anesthesia required six- to tenfold lower doses of morphine intra- and postoperatively compared to those without CEA; ventilatory support upon completion of surgery was remarkably shorter (59 versus 210 min) in the CEA group as well as ICU stay (1.1 versus 1.8 days). The total consumption of anticholinergics was twice as high as in patients without CEA. There were no relevant CEA-related complications.Being a retrospective audit of practice in our institution with a small number of patients, our results are in line with previously published data on CEA in pediatric patients. CEA has been shown to significantly reduce the need for anesthetics and morphine and allows early extubation with all subsequent advantages for a speedy recovery post surgery. Thus, the technique is to be recommended as a safe and efficacious method for pain management following major reconstructive surgery in pediatric urology. Importantly, this type of anesthesia should be performed only by experienced anesthesiologists in institutions where appropriate equipment, staff, and monitoring are available.
Article
Bryant's traction is the most commonly used method for immobilization after bladder exstrophy repair. We hypothesized that spica casting is a safe and effective alternative to Bryant's traction after complete primary repair of exstrophy. Complete primary repair of exstrophy was performed for initial repair in 39 consecutive children by all surgeons at Seattle Children's Hospital since 1998. Three sequential cohorts were evaluated-Bryant's traction without osteotomy (13 patients), spica casting without osteotomy (14) and spica casting with osteotomy. These 3 sequential cohorts represent eras of care and an evolution of practice. Primary outcomes included major complications related to immobilization, dehiscence, urinary incontinence and length of stay. We defined complications of immobilization as nonunion of pelvic osteotomy, femoral nerve palsy, revision of spica cast requiring return to the operating room, infection at the osteotomy site and activity limiting pain at the osteotomy site. Fisher's exact test or t test was used to determine statistical significance. There was no difference in urinary continence (p = 0.09). Use of Bryant's traction was associated with double the length of stay (p >0.001). There was no correlation of major complications to the type of immobilization used. Spica casting compared to Bryant's traction is associated with shorter hospitalization following complete primary repair of exstrophy and does not have a significant difference in the rate of complications. In our longitudinal cohort study with long-term followup spica cast was safe and effective for patients with bladder exstrophy, and should be considered an acceptable method of immobilization.
Article
Many changes have occurred in the treatment of bladder exstrophy over the last few years and many repairs are now offered. The purpose of this study was to evaluate long-term outcomes in a select group of patients in whom modern staged repair (MSRE) was undertaken. From an institutionally approved database were extracted 189 patients who had undergone primary closure between 1988 and 2004. The records of 131 patients (95 males) who underwent MSRE with a modified Cantwell-Ransley repair by a single surgeon in 1988-2004 were reviewed with a minimum 5-year follow up. Sixty-seven patients with a mean age of 2 months (range 6 h to 4 months) underwent primary closure, and 18 underwent osteotomy at the same time. Mean age at epispadias repair was 18 months (8-24). Mean age at bladder neck reconstruction (BNR) was 4.8 years (40-60 months) with a mean capacity of 98 cc (75-185). Analysis of bladder capacity prior to BNR revealed that patients with a mean capacity greater than 85 cc median had better outcomes. Seventy percent (n=47) are continent day and night and voiding per urethra without augmentation or intermittent catheterization. Social continence defined as dry for more than 3h during the day was found in 10% (n=7). Six patients required continent diversion after failed BNR. Seven patients are completely incontinent. The mean time to daytime continence was 14 months (4-23) and the mean time to night-time continence was 23 months (11-34). No correlation was found between age at BNR and continence. Patients with a good bladder template who develop sufficient bladder capacity after successful primary closure and epispadias repair can achieve acceptable continence without bladder augmentation and intermittent catheterization.
Article
The place of pelvic osteotomy in reconstructing bladder/cloacal exstrophy has been debated for some time; the experience with 'combined' osteotomy in primary and re-operative exstrophy closure at this institution is presented, with a discussion of the historical and scientific place of osteotomy in managing this condition. Sixty-eight patients had bilateral vertical and transverse iliac osteotomy between 1992 and 2003, and with outcome data available. Of 58 patients with classic exstrophy, eight were newborns, eight were deliberately delayed primary closures, 36 were re-operative after previous failed closure and six were bladder neck reconstructions where the bladder outlet was very wide, such that bony closure was felt necessary for successful bladder neck coaptation. Of 10 patients with cloacal exstrophy, nine were primary closures and one was a re-operative closure. Data were collected relating to age at closure, complications and continence outcome. The mean (range) age (months) was 41 (5-179) for re-operative closures, 12.5 (3-32) for delayed primary closures, 64.1 (38-79) for bladder neck reconstruction, 51.4 (6-165) for cloacal exstrophy closure, and 15 (2-45) days for newborn exstrophy closure. There was a superficial wound infection in two patients, pin-site infection in one, loose pins in two, and two had transient femoral nerve palsy. In two patients the procedure failed and they required further re-operative closure with osteotomy. Sixteen patients are dry urethrally day and night, 12 have had and four are awaiting bladder augmentation, one has a colon conduit, and 35 are awaiting a definitive continence procedure. Osteotomy has a proven track record in the field of exstrophy reconstruction, and the benefit especially in re-operative closure is emphasized by the present results. The surgical morbidity with the 'combined osteotomy' is low, cosmetic results are excellent and the effect on success of closure is clearly advantageous.
Article
We report the urological, orthopedic and neurological complications of primary closure of classic bladder exstrophy using modern staged repair of exstrophy. An approved database identified 137 males and 57 females with classic bladder exstrophy who underwent primary repair by 1 of 2 surgeons in 23 years. A total of 185 patients underwent primary closure using modern staged repair of exstrophy with or without osteotomies, whereas 9 underwent delayed primary closure with epispadias repair at age 12 months. Of the patients 63 received osteotomies. Mean age at closure was 60 days and mean followup was 9 years. There were 14 major complications (11%) and 27 minor complications (14%). Major urological complications included bladder prolapse or dehiscence in 6 male patients (3%), which was successfully reclosed. Major orthopedic complications, including osteotomy nonunion in 2 cases, leg length inequality in 1 and persistent joint pain in 1, developed in 4 of the 63 patients (6%) who underwent osteotomy. Major neurological complications included femoral nerve palsy in 4 patients (2%). There were 21 minor urological complications (11%), including posterior bladder outlet obstruction in 4 cases, urethrocutaneous fistula in 2, suprapubic tube removal in 2, intrapubic stitch erosion in 4, febrile urinary tract infection in 6 and surgical site infection in 3. Six patients (3%) had minor orthopedic complications, including pelvic osteomyelitis in 1, pin site infection in 3 and a pressure sore from immobilization in 1. Closure of bladder exstrophy is a safe surgery with an acceptable risk of complications. A critical review of outcomes provides insight to further refine the technique and manage complications when they develop.