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

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.