0022-5347/02/1684-1692/0 Vol.

168, 1692–1694, October 2002

THE JOURNAL OF UROLOGY® Printed in U.S.A.
Copyright © 2002 by AMERICAN UROLOGICAL ASSOCIATION, INC.® DOI: 10.1097/01.ju.0000027525.81696.02

Exstrophy

COMPLETE REPAIR OF EXSTROPHY: FURTHER EXPERIENCE WITH

NEONATES AND CHILDREN AFTER FAILED INITIAL CLOSURE
MOHAMED T. EL-SHERBINY, ASHRAF T. HAFEZ AND MOHAMED A. GHONEIM
From the Urology and Nephrology Center, Mansoura University, Mansoura, Egypt

ABSTRACT

Purpose: The surgical repair of bladder exstrophy remains challenging for the urologist.
Recently, complete primary repair has been used in neonates. We present our experience with
this approach in neonates and children after failed initial closure.
Materials and Methods: Between November 1998 and November 2000, 17 boys and 2 girls with
bladder exstrophy underwent complete repair. Complete primary repair was performed in the first 72
hours of life in 4 boys. Complete repair with osteotomy was at a mean age ⫾ SD of 23 ⫾ 21 months
(range 1 to 74) in 15 patients including 7 with failed initial closure. The bladder and urethra were
closed in continuity with epispadias repair by total penile disassembly. All patients were kept in spica
cast for 3 weeks. Ureteral stents and suprapubic tube were removed 10 and 14 days after surgery,
respectively. Ultrasound was performed before surgery and 3 months thereafter, and voiding cys-
tourethrography was obtained 3 months postoperatively and then annually in all cases.
Results: Complete closure resulted in hypospadias in 10 boys (59%). There was no major
complication. Mean followup ⫾ SD was 17 ⫾ 8 months (range 5 to 33). Temporary suprapubic
urinary leakage was noted initially in 10 cases (52%) but no patient had persistent fistula. Initial
postoperative renal ultrasound revealed hydronephrosis in 11 renal units (29%). However, at last
followup only 1 renal unit (2%) showed pelvicaliceal dilatation. Two patients (10%) had a febrile
urinary tract infection and were treated conservatively. Reflux was noted in 24 renal units (63%)
but did not require surgery. The 4 boys in whom the closure was performed at birth had regular
voiding with 60 to 90-minute dry intervals and mean bladder capacity ⫾ SD was 85 ⫾ 35 cc. The
15 older children had a mean bladder capacity of 74 ⫾ 37 cc and 5 (33%) had regular voiding with
30 to 90-minute dry intervals.
Conclusions: Complete repair of exstrophy is feasible in neonates and older children including,
those with failed initial closure with minimal morbidity. There is a short-term evidence of
favorable outcome in newborns. Longitudinal followup is required to determine the future need
of bladder neck reconstruction and augmentation in older patients.
KEY WORDS: bladder exstrophy; reconstructive surgical procedures; penis

The surgical management of bladder exstrophy is a diffi-
cult challenge for the pediatric urologist. The classic surgical
approach to correct bladder exstrophy is performed in stag-
es.1, 2 Primary bladder and abdominal wall closure is the
initial step, followed by epispadias repair at age 6 months to
1 year. Bladder neck function is not addressed until age 4 to
5 years when bladder capacity is adequate and the child is
ready to be dry. This approach was popularized by Jeffs, who
showed that renal preservation and satisfactory continence
rates can be achieved. However most reports on staged repair
were based on highly select groups of patients.3, 4 Gearhart et
al later combined epispadias repair with bladder closure in
children with failed initial closure.5 Combining the proce-
dures was not associated with increased morbidity and the
results were comparable to the staged approach.
Recently, Grady and Mitchell combined primary bladder
closure with epispadias repair in 1 stage in 18 neonates with
classic bladder exstrophy.6 The idea was to optimize the
chance for early bladder cycling and potentiate bladder de-
velopment. It may also obviate the need for multistage repair
of bladder exstrophy, including further bladder neck recon-
struction and penile reconstructive surgery. They used their
1692
technique of epispadias repair by total penile disassembly.7
Although acceptable short-term results were achieved, the
procedure has been criticized in view of 50% incidence of
antireflux surgery secondary to breakthrough urinary tract
infections. We previously demonstrated the feasibility of this
technique for bladder exstrophy reconstruction in neonates
and older children including those with failed initial bladder
closure with minimal morbidity.8 We report our further ex-
perience with this technique in 19 patients.
MATERIALS AND METHODS
Between November 1998 and November 2000, 17 boys and
2 girls underwent 1-stage reconstruction for bladder exstro-
phy. Complete primary repair was performed in the first 72
hours of life in 4 boys, and at a mean age of 23 ⫾ 21 months
(range 1 to 74) in 15 including 7 with failed initial closure.
In male patients bladder closure was combined with epis-
padias repair by the total penile disassembly as described by
Grady and Mitchell (see figure).6 In females the urethral
plate and vagina were mobilized as 1 unit and the whole unit
was positioned deep into the pelvis with Y-V advancement.
 COMPLETE REPAIR OF EXSTROPHY AFTER FAILED INITIAL CLOSURE
Dissection of bladder and urethral plate as 1 unit and closure. Note
that intersymphyseal bands were completely cut and penis was
totally disassembled.
Osteotomy was not performed in the 4 neonates who under-
went the procedure in the first 3 days of life. All older pa-
tients underwent bilateral anterior oblique iliac osteotomies
via separate incisions. Concomitant repair of inguinal hernia
was performed in 1 newborn. Neither local epinephrine in-
jection nor penile tourniquet was required. At the end of the
procedure ureteral stents and suprapubic cystostomy tube
were left indwelling in all cases.
Ureteral stents and cystostomy tube were removed 14 and
21 days, respectively, postoperatively. Parentertal antibiotics
were administered for 2 days followed by routine antibiotic
prophylaxis for reflux. All patients were maintained in spica
cast for 3 weeks. Weight bearing was not permitted for ad-
ditional 3 weeks for the older children who underwent osteot-
omy. Ultrasound was performed before surgery and every 3
months postoperatively, and voiding cystourethrography was
done 3 months after surgery and then annually in all cases.
RESULTS
All complete repairs were successful and there was no major
complication. Normal orthotopic urethral meatus was achieved
in 7 boys. The repair resulted in hypospadias in the remaining
10 boys (59%). Ten patients (52%) had temporary suprapubic
urinary leakage following removal of the cystostomy tube,
which was treated with a urethral catheter. Median time to
closure of the suprapubic fistula was 5 days (range 3 to 12). No
patient had dehiscence or persistent fistula. Early postopera-
tive complications and their management are listed in table 1.
Mean followup was 17 ⫾ 8 months (range 5 to 33). Initial
renal ultrasound at 3 months demonstrated grade II to III
hydronephrosis in 11 renal units (29%). However, serial ultra-
sound examinations showed normal renal growth in all patients
and only 1 had grade II hydronephrosis in 1 renal unit (2%) at
last followup. Single breakthrough febrile urinary tract infec-
tion occurred in only 2 patients (10%), which was managed
conservatively with appropriate antibiotics. Urethral meatal
stenosis in 1 of these 2 patients was treated with ventral meato-
tomy under local lidocaine and prilocaine cream.
TABLE 1. Early postoperative complications
No. Pts. (%) Complications Treatment
19 Total
1 (5) Prolonged ileus Nasogastric tube placement
10 (52) Suprapubic urinary leak Urethral catheter for 5 days
5 (26) Urinary candida infection Discontinuation of broad-spectrum
antibiotics and urine alkalization
1693
Voiding cystourethrography revealed grade II to IV vesi-
coureteral reflux in 24 of 38 (63%) renal units in 15 children.
none of whom required reimplantation at last followup. None of
the 4 patients whose bladders were closed at birth had reflux.
The 4 boys in whom closure was performed at birth had
regular voiding with 60 to 90-minute dry intervals and mean
bladder capacity was 85 ⫾ 35 cc (range 55 to 120). The 15
older children had a mean bladder capacity of 74 ⫾ 37 cc
(range 30 to 120) and 5 (33%) had regular voiding with 30 to
90-minute dry intervals. Bladder capacity based on voiding
cystourethrography and detailed continence status are
shown in table 2. Other procedures included inguinal herni-
orrhaphy in 6 patients performed as a separate operation.
DISCUSSION
The goals of bladder exstrophy reconstruction include pres-
ervation of kidney function, creation of urinary continence,
decreased episodes of urinary tract infection, and creation of
functionally and cosmetically acceptable external genitalia.
These goals have remained constant since the initial opera-
tions were proposed and attempted in the 1800s. Surgical
management of this condition has undergone dramatic evo-
lution during the last 30 years.
In the 1970s multiple trials aimed at planned 1-stage exstro-
phy reconstruction. However, the primary enthusiasm was fol-
lowed by disappointment due to poor outcome. Continence rate
was 20% with renal deterioration in most of the continent
patients.9 Planned staged approach gained wide popularity
with reported urinary continence rates as high as 91%.1, 2, 10
However, these results were based on highly select groups of
patients and others failed to achieve such results. Continence
rates of only 10% to 30% were reported with this approach.10, 11
Renal damage rates were reported to be 13% to 20%.10, 12
Recent efforts have focused on reconstruction of the bladder
in a primary 1-stage fashion based on the principle that exstro-
phy represents anterior bladder herniation.13 Therefore, repair
demands aggressive posterior repositioning of the bladder,
bladder neck and urethra. The bladder, bladder neck and ure-
thra are considered 1 unit and no initial effort is made at
bladder neck reconstruction. With these concepts in minds,
Grady and Mitchell combined bladder closure with epispadias
repair by penile disassembly in 1-stage in newborns.6 The
short-term results were encouraging. We have enthusiastically
embraced this approach with encouraging preliminary results.8
Our study confirms that this approach is feasible without
major complications. There was no dehiscence or persistent
fistula in any patient. We agree with Surer et al that simul-
taneous dissection of the bladder and urethral plate permits
deep placement in the pelvis, improving closure of the abdo-
men and minimizing the risk of urethrocutaneous fistula.14
Normal renal growth was observed in all patients and only 1
renal unit (2%) showed mild dilatation at last followup. Dur-
ing a mean followup of 17 months there was no need for
antireflux surgery although voiding cystourethrography
showed grade II to IV vesicoureteral reflux in 24 of 38 (63%)
renal units. The incidence of vesicoureteral reflux in this
series is similar to that noted by Grady and Mitchell.6 It is
interesting that in the 4 patients whose bladders were closed
at birth voiding cystourethrography did not show reflux.
Grady and Mitchell addressed the problem of insufficient
TABLE 2. Bladder capacity and continence
Older
Newborns
Children
No. pts. 4 15
No. toilet trained 2 8
No. dry intervals 4 5
No. continent (dry interval 90 mins. or greater) 2 3
Mean bladder capacity ⫾ SD (cc) 85 ⫾ 35 74 ⫾ 37
Mean expected bladder capacity for age ⫾ SD (cc) 87 ⫾ 22 153 ⫾ 53
1694 COMPLETE REPAIR OF EXSTROPHY AFTER FAILED INITIAL CLOSURE
urethral length to reach the glans when the complete penile
disassembly technique was used.6 This technique may create
hypospadias in some cases which may be corrected later. They
believe that this risk represents an inherent lack of length in
the urethral plate. Mitchell and Bagli applied the complete
penile disassembly technique to epispadias repair, and hypos-
padias resulted in 30% of cases.7 In our study complete repair of
exstrophy resulted in hypospadias in 10 of 17 boys (59%). We
believe that hypospadias occurred more often in our series be-
cause of the aggressive posterior mobilization of the bladder and
urethra that is usually required for exstrophy. However, this
high incidence of hypospadias may be minimized with preoper-
ative use of testosterone local cream in older children and re-do
cases to enhance the growth of the urethral plate.5 In male
newborns partial separation of the urethral plate and use of
interrupted sutures have been found to be helpful to obtain an
orthotopic meatus.15
Longer followup is required to define the continence issue
among these patients. However, the 4 patients in whom
bladder closure was performed at birth had 60 to 90-minute
dry intervals, and 2 are starting toilet training and favorable
outcome is expected. Of the remaining 15 older children 5
had documented dry intervals, and 3 of them are starting
toilet training with promising results. Longer followup will
determine whether the future need for bladder neck recon-
struction will be obviated with this approach. Combined ex-
strophy closure with epispadias repair has been promising in
older boys with failed or late initial closure.5
CONCLUSIONS
Complete repair of exstrophy is feasible in neonates and
older children after failed initial closure with acceptable mor-
bidity. There is no short-term evidence of upper tract deteri-
oration or increased incidence of febrile urinary tract infec-
tions. However, the repair results in hypospadiac opening in
two-thirds of male patients. There is short-term evidence of
favorable outcome in newborns. Longitudinal followup is re-
quired to determine the future need for bladder neck recon-
struction and augmentation in older patients.
REFERENCES
1. Chan, D. Y., Jeffs, R. D. and Gearhart, J. P.: Determinants of
continence in the bladder exstrophy population: predictors of
success? Urology, 57: 774, 2001
2. Gearhart, J. P.: Bladder exstrophy: staged reconstruction. Curr
Opin Urol, 9: 499, 1999
3. Capolicchio, G., McLorie, G. A., Farhat, W. et al: A population
based analysis of continence outcomes and bladder exstrophy.
J Urol, 165: 2418, 2001
4. Lottmann, H. B., Melin, Y., Cendron, M. et al: Bladder exstrophy:
evaluation of factors leading to continence with spontaneous
voiding after staged reconstruction. J Urol, 158: 1041, 1997
5. Gearhart, J. P., Mathews, R., Taylor, S. et al: Combined bladder
closure and epispadias repair in the reconstruction of bladder
exstrophy. J Urol, 160: 1182, 1998
6. Grady, R. W. and Mitchell, M. E.: Complete primary repair of
exstrophy. J Urol, 162: 1415, 1999
7. Mitchell, M. and Bagli, D.: Complete penile disassembly for epis-
padias repair: the Mitchell technique. J Urol, 155: 300, 1996
8. Hafez, A. T., El-sherbiny, M. T. and Ghoneim, M. A.: Complete
repair of bladder exstrophy: preliminary experience with neo-
DISCUSSION
Dr. John Gearhart. In your failed exstrophy group I am interested in knowing how you fixated the bones after
the osteotomy. In addition, I am not surprised with your outcome in the older children because a number of years
ago we found that if your first exstrophy closure fails, the child has only a 40% chance of having adequate bladder
capacity for bladder neck reconstruction within 5 years.
Dr. Mohamed El-Sherbiny. All older patients underwent an osteotomy, and the fixation of the pubis was
performed with a No. 1 polydioxanone suture.
nates and children with failed initial closure. J Urol, 165:
2428, 2001
9. Megalli, M. and Lattimer, J. K.: Review of the management of
140 cases of exstrophy of the bladder. J Urol, 109: 246, 1973
10. Connor, J. P., Hensle, T. W., Lattimer, J. K. et al: Long-term
followup of 207 patients with bladder exstrophy: an evolution
in treatment. J Urol, 142: 793, 1989
11. Woodhouse, C. R. and Redgrave, N. G.: Late failure of the recon-
structed exstrophy bladder. Br J Urol, 77: 590, 1996
12. Mesrobian, H. G., Kelalis, P. P. and Kramer, S. A.: Long-term
followup of 103 patients with bladder exstrophy. J Urol, 139:
719, 1988
13. Thomalla, J. V., Rudolph, R. A., Rink, R. C. et al: Induction of
cloacal exstrophy in the chick embryo using the CO2 laser.
J Urol, 134: 991, 1986
14. Surer, I., Baker, L. A., Jeffs, R. D. et al: Combined bladder neck
reconstruction and epispadias repair for exstrophy-epispadias
complex. J Urol, 165: 2425, 2001
15. Pippi-Salle, J. L. and Chan, P. T.: One stage bladder exstrophy and
epispadias repair in newborn male. Can J Urol, 6: 757, 1999
EDITORIAL COMMENT
El-Sherbiny et al have demonstrated that the complete primary
exstrophy repair technique can be applied safely to neonates and
salvage cases. Importantly, this article joins a series of reports on
this technique confirming its safety when performed by experienced
surgeons. Although Gearhart et al have reported on complications
associated with complete primary repair,1 surgeons from other insti-
tutions as well as our own have described few complications (refer-
ences 6 and 8 in article). The fact that the complication rate remains
low despite the fact that these surgeons have reported cases early in
their learning curve with this technique is impressive, especially in
comparison to the extraordinarily high complication rates associated
with primary repair techniques in the 1960s and early 1970s.2
Why the difference? We believe the increased margin of safety with
the complete primary repair technique comes from several principles.
The technique relies on dissection along anatomical planes to preserve
blood supply, and it focuses on returning anatomy to a “more normal”
location by placing the urethra, bladder neck and bladder deep into the
pelvis to allow the pelvic diaphragm to assist with later continence. No
specific surgical attention is directed to the bladder neck, thus avoiding
the temptation to overly narrow this area and produce obstructive
uropathy. El-Sherbiny et al describe only transient hydronephrosis in
29% of the renal units in their series. We have noted the same transient
phenomenon, likely demonstrating a temporary increased bladder stor-
age pressure as the bladder accommodates to its new role as a urinary
storage organ (reference 6 in article).
The complete primary repair technique appears as safe as the gold
standard—staged closure for exstrophy—popularized by Gearhart
and Jeffs. Long-term results from medical centers now using this
technique will demonstrate whether it is also consistent in producing
urinary continence and satisfactory sexual function for patients with
exstrophy as well.
Richard W. Grady
Division of Pediatric Urology
Children’s Hospital and Regional Medical Center
Seattle, Washington
1. Gearhart, J. P.: Complete repair of bladder exstrophy in the new-
born: complications and management. J Urol, 165: 2431, 2001
2. King, L. and Wendcl, E.: Primary cystectomy and permanent
urinary diversion in the treatment of exstrophy of the urinary
bladder. In: Current Controversies in Urologic Management.
Edited by R. Scott, Jr., H. Gordon, C. Carlton and P. Beach.
Philadelphia: W. B. Saunders Co., pp. 242–250, 1972