classic bladder exstrophy ( be ) is a rare malformation of the genito - urinary tract affecting 1:50,000 - 1:100,000 live births .
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
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 .
the mean and median age of exstrophy repair was 1.25 years and 4 months , respectively .
one patient had failure and six had penopubic or urethro - cutaneous ( uc ) fistulae .
the average age at the time of epispadias repair was 5 years ( 212 years ) .
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 2 h with good nocturnal continence ; one female child needs clean intermittent self - catheterization ( cisc ) every 34 h for bladder emptying .
two other children also have undergone augmentation cystoplasty and are on cisc every 34 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 [ figures 1 and 2 ] .
female exstrophy child with good bladder plate micturating cystourethogram showing good bladder capacity the average age at the time of bladder neck repair was 8 years ( 416 years ) and the mean bladder capacity at the time of repair was 100 ml ( 80200 ml ) .
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 ] .
primary bladder exstrophy repair / secondary bladder exstrophy repair 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 ] .
cantwell - ransley epispadias repair thirteen patients have undergone all stages of be repair ; nine are continent for more than 2 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 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 .
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 .
four patients who are coming for regular follow - up have still not developed adequate bladder capacity and are awaiting continence procedure [ table 3 ] .
the modern staged approach to be reconstruction has undergone significant changes since it was first advocated by jeffs and cendron as quoted by gearhart .
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 .
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 .
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 .
now we prefer to use dexon suture for pubic symphyseal closure rather than prolene suture .
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 .
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 .
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 .
 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 . 