|Year : 2016 | Volume
| Issue : 4 | Page : 158-163
The long-term outcomes after staged repair of exstrophy-epispadias complex
Sajni I Khemchandani
Department of Urology, Institute of Kidney Diseases and Research Center and Dr. H L Trivedi Institute of Transplantation Sciences, Civil Hospital Campus, Ahmedabad, Gujarat, India
|Date of Web Publication||19-Jul-2016|
Sajni I Khemchandani
No. 401, Sudarshan Flats, 13, Shantinagar Society, Usmanpura, Ahmedabad - 380 013, Gujarat
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Introduction: Classic bladder exstrophy (BE) is a rare malformation of the genito-urinary tract affecting 1:50,000 to 1:100,000 live births. The surgical reconstruction of the BE-epispadias complex is challenging for the most experienced pediatric urologists, surgeons, and orthopedists. Purpose: To assess the success of staged reconstruction of the BE and long-term effects on the upper urinary tract, renal function, and continence. Materials and Methods: This is retrospective study; between 1994 and 2013, 30 patients with BE have undergone stage 1 repair at the institute. Eighteen male patients have been operated for epispadias repair and thirteen patients have undergone Guy Leadbetter bladder neck reconstruction. Three patients required augmentation cystoplasty one child is continent after epispadias repair only and one child attained continence after single-stage repair. Results: Hence, out of 17 patients, 14 are socially continent, four patients require clean intermittent self-catheterization for bladder emptying. Four patients, who are coming for regular follow-up, are awaiting continence procedure. Two patients who underwent augmentation cystoplasty are on hemodialysis for renal failure and one child has altered renal function. Conclusion: In our experience, the modern staged repair offers a low risk of renal scarring with acceptable continence opportunity with acceptable cosmetic appearance of external genitalia in the males and females.
Keywords: Bladder exstrophy, epispadias, outcomes, reconstructive surgical procedure
|How to cite this article:|
Khemchandani SI. The long-term outcomes after staged repair of exstrophy-epispadias complex. J Indian Assoc Pediatr Surg 2016;21:158-63
|How to cite this URL:|
Khemchandani SI. The long-term outcomes after staged repair of exstrophy-epispadias complex. J Indian Assoc Pediatr Surg [serial online] 2016 [cited 2019 Sep 15];21:158-63. Available from: http://www.jiaps.com/text.asp?2016/21/4/158/186544
| Introduction|| |
Classic bladder exstrophy (BE) is a rare malformation of the genito-urinary tract affecting 1:50,000-1:100,000 live births. The male to female ratio ranges from 1.5 to 5:1.  The surgical reconstruction of the BE-epispadias complex has evolved significantly during the last 50 years but is still challenging for the most experienced pediatric urologists, surgeons, and orthopedists.
The upper tracts are almost always normal in the neonates with classic BE before abdominal wall, posterior urethral, and bladder closure. The upper tract changes or deterioration of renal function almost always occurs after reconstructive surgery.
The objectives of treatment are to achieve a secure closure of the bladder, pelvis, and the abdominal wall; preservation of renal function; urinary continence and creation of functional and cosmetically appealing genitalia.
Grady and Mitchell have described complete repair of classic BE with the combination of bladder closure and epispadias repair at birth.  However, the modern staged repair of exstrophy is still considered the gold standard. This involves bladder closure shortly after birth, followed by epispadias repair at 6-12 months of age and bladder neck reconstruction at 4-5 years of age.
To assess the success of staged reconstruction of the BE and its effect on the upper urinary tract, we have analyzed the renal function and continence in 30 BE patients.
| Materials and methods|| |
Between 1994 and 2013, 30 patients with BE have been managed at our institute in various stages of repair, 7 were females and 23 were male patients. Of 30 patients 27 patients underwent primary repair and 3 patients were referred for secondary BE closure. Twenty-one children presented before 1 year of age; four neonates presented before 72 h of birth, in whom bladder closure was done without osteotomy with hip spica only. Three of these patients, who were operated without osteotomy, had bladder dehiscence, hence have undergone secondary repair with bilateral anterior iliac osteotomy and immobilization with external fixators (EFs). Eight patients presented before 3 months of age; all underwent primary bladder closure with bilateral anterior iliac osteotomy and immobilization with hip spica. Nine patients presented between 4 months and 1 year of age, all were operated with primary or secondary exstrophy repair with bilateral anterior iliac osteotomy and immobilization with EFs. Two of these children referred with bladder dehiscence, had primary repair with posterior iliac osteotomy; hence, secondary exstrophy repair was carried out with bilateral anterior iliac osteotomy. Eight children presented between 1 and 5 years of age and one child presented at 9 years of age; all underwent bladder closure with bilateral anterior iliac osteotomy and immobilization with EFs. None of the children with anterior iliac osteototomy and EFs had bladder dehiscence. The mean and median age of exstrophy repair was 1.25 years and 4 months, respectively.
Eighteen male children have undergone modified Cantwell-Ransley epispadias repair. One patient had failure and six had penopubic or urethro-cutaneous (UC) fistulae. One child with failure underwent secondary epispadias repair with good result. Of six children with fistulae, three required repair and three healed spontaneously. The average age at the time of epispadias repair was 5 years (2-12 years).
Bladder neck reconstruction was been done by Guy Leadbetter technique. The triangular flaps of the bladder were incorporated into the bladder instead of de-epithelializing, and using these to buttress the bladder neck repair.
Thirteen children have undergone Guy Leadbetter bladder neck reconstruction, eleven males and two females. Eight children are continent for more than 2½ h with good nocturnal continence; one female child needs clean intermittent self-catheterization (CISC) every 3-4 h for bladder emptying. Four children are continent for 1-2 h only. One female who was continent for <2 h, has undergone augmentation cystoplasty. Two other children also have undergone augmentation cystoplasty and are on CISC every 3-4 h. One male child, who underwent exstrophy reconstruction at 9 years of age, has surprisingly developed continence after epispadias repair only.
Interestingly, one female child who presented at 3 years of age with good bladder plate was operated with bilateral cephalo-trigonal ureteric reimplantation, trigonal tubularization, and bladder and abdominal wall closure with bilateral anterior iliac osteotomy is continent for 3 h after single-stage repair only [Figure 1] and [Figure 2].
The average age at the time of bladder neck repair was 8 years (4-16 years) and the mean bladder capacity at the time of repair was 100 ml (80-200 ml).
| Results|| |
Thirty patients have undergone primary or secondary bladder closure, four neonates presenting before 72 h of life were operated without osteotomy, three of these developed dehiscence.
Hence, following this all children were operated with bilateral anterior iliac osteotomy either with hip spica in infants <3 months and EFs in all children older than 3 months [Table 1].
|Table 1: Primary bladder exstrophy repair/secondary bladder exstrophy repair|
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Eighteen children have undergone modified Cantwell-Ransley epispadias repair, one had a failure and was successfully repaired again. Six children had UC fistula postoperatively; three healed spontaneously and three required repair [Table 2].
Thirteen patients have undergone all stages of BE repair; nine are continent for more than 2½ h, and four children are continent for <2 h. One child is continent after single-stage repair; another child has developed continence after epispadias repair only. One child with failed bladder neck repair and two children whose bladder capacity was <80 ml have undergone augmentation cystoplasty. Hence, out of 17 patients; 14 are socially continent with good nocturnal continence. Two children, who had undergone augmentation cystoplasty, have developed chronic renal failure and are on hemodialysis. These children had progressive renal scarring with upper tract changes. These changes resulted from progressive hydronephrosis due to bladder outlet obstruction after bladder closure in one female patient and after urinary diversion in another male child. One female who is on CISC has developed altered renal functions. Five patients developed bladder calculi after BE or epispadias repair, and have developed renal scarring. These patients who developed renal scarring following closure had one or more documented febrile urinary tract infections. However, all were maintained on suppressive antibiotics from the time of initial closure. There was no correlation between age of bladder neck repair and urinary continence. Four patients who are coming for regular follow-up have still not developed adequate bladder capacity and are awaiting continence procedure [Table 3].
|Table 3: Bladder neck reconstruction uy Leadbetter bladder neck re - construction|
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| Discussion|| |
The modern staged approach to BE reconstruction has undergone significant changes since it was first advocated by Jeffs and Cendron as quoted by Gearhart.  This approach has been modified significantly since. However, the goal of upper tract preservation, obtaining urinary continence, and achievement of satisfactory functional and cosmetic external genitalia remains same. Nonetheless, this success is associated with some risk to the upper urinary tract.
Usually, these complications are seen after failed initial closure, which can be categorized as complete dehiscence, bladder prolapse, and outlet obstruction. In our series, three neonates who were operated without osteotomy had bladder dehiscence; other 27 children who were operated with bilateral anterior iliac osteotomy did not have bladder dehiscence. Hence, we will suggest bilateral anterior iliac osteotomy in all children. The most critical support for osteotomy is that it allows tension free closure, which prevents subsequent dehiscence and organ prolapsed.  Studies from Europe and North America have shown that patients requiring repeat closure after failed attempt are less likely to have undergone osteotomy at initial closure. , Children operated before 3 months of age had primary bladder closure with bilateral anterior iliac osteotomy and immobilization with hip spica. In Indian summers, it is difficult to maintain hygiene and avoid wound infection in hip spica. Hence, we propose EFs in all children for immobilization. It is very convenient to maintain hygiene and avoid wound infection.
Bhatnagar incorporates triangular flaps after urethral lengthening in the bladder repair; we also incorporate these triangular flaps for the bladder repair. This not only helps in utilizing bladder tissue for enhancing the bladder capacity but also improves the results of surgery with regard to urinary continence. 
The patients with outlet obstruction after initial closure are at risk of hydronephrosis, severe reflux, pyelonephritis, and renal scarring. One female child with outlet obstruction developed renal scarring and is on hemodialysis. In a review by Baker et al. of exstrophy database, 29 boys and 12 girls were found to have posterior urethral obstruction after closure in the neonatal period. 
In our patients, the most common method of presentation was urinary tract infection seen in eight patients; of these five had bladder calculi due to intersymphyseal stitch erosion into bladder and one presented with urinary retention. Three children with stones required open cystolithotomy, two required cystolitholapexy. Now we prefer to use Dexon suture for pubic symphyseal closure rather than prolene suture. Child with urinary retention required regular urethral dilation.
Husmann et al. have revealed an acceptable risk of renal deterioration with 14.7% of patients developing renal deterioration over a follow-up of 12.7 years. These authors suggest that most significant factors in the development of upper tract scarring after bladder closure are one or more febrile urinary tract infections, failure to use prophylactic antibiotics after bladder closure and presence of progressive hydronephrosis.  In Baker et al. series, 22% had upper tract deterioration, including two with chronic renal insufficiency.  In our series also, three patients (10%) have developed renal deterioration.
The above statistics clearly suggest that in an infant or child with BE, a febrile urinary infection should warrant a renal and bladder ultrasound to rule out hydronephrosis, hydroureter, and to assess bladder emptying. Since all patients with exstrophy have various degree of reflux, all should receive chemoprophylaxis. The prophylaxis should be continued until ureteric reimplantation and bladder neck reconstruction performed and reflux cured and appropriate bladder emptying proven on ultrasound.
The development of hydronephrosis after bladder neck repair can be due to ineffectual bladder emptying, a small inelastic bladder that is not capable of handling increasing volume of urine over time or ureteral obstruction secondary to reimplantation. This can occur any time after repair, hence the importance of constant regular follow-up evaluations.  In our series also, one child developed altered renal function due to small inelastic bladder not capable of handling the increasing volume of urine.
Although modified staged reconstruction is being performed at many institutes, Grady and Mitchell have proposed total reconstruction consisting of the bladder and urethral closure with penile disassembly in the newborn period. However, 50% of these patients required ureteric reimplantation after newborn period for febrile urinary tract infections.  Thus, these patients will also need careful, regular follow-up.
Baradaran et al. also stipulate that delayed primary repair of exstrophy does not compromise the rate of bladder growth. However, children born with smaller templates will have overall smaller capacities and are less likely to undergo bladder neck reconstruction. 
While bladder prolapse and dehiscence have been recognized as major complications of primary BE repair, posterior urethral outlet obstruction after bladder closure puts bladder and upper tracts at risk and markedly decreases the success rate of modern techniques of staged reconstruction.  These complications are more problematic than upper tract changes after bladder neck repair.
| Conclusion|| |
Therefore, whichever treatment modality is chosen for the newborn with BE; modern type of staged reconstruction, bladder, and urethral closure by penile disassembly in the newborn or urinary diversion, follow-up must be careful and into and including adulthood. In our experience, the modern staged repair offers a low risk of renal scarring with acceptable continence opportunity with acceptable cosmetic appearance of external genitalia in the males and females.
We are thankful to Dr. Raj Shah and Dr. Vinod Gautam for their support.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]
[Table 1], [Table 2], [Table 3]