1. To review surgical and medical outcomes of the previous repaired bladder exstrophy cases. 2. To present recent developments in surgical techniques and care of these patients.
* Exstrophy-epispadias complex (EEC) is a spectrum of genitourinary malformations resulting from abnormal cloacal development . * CBE, the most common form with an incidence of 2.68 in 100,000 live births is characterized by an evaginated bladder plate, epispadias and an anterior defect of the pelvis, pelvic floor and abdominal wall * The single most important predictor of long-term bladder growth and continence is successful primary bladder closure . * The staged reconstruction of bladder exstrophy was proposed by Jeff and led to significant improvements in continence and renal function with excellent cosmetic results. * The initial staged management consisted of newborn bladder and posterior urethral closure followed by bladder neck reconstruction at 2 to 3 years of age and later epispadias repair. * The understanding that appropriately timed epispadias repair may lead to enhancement in bladder capacity led to performance of epispadias repair at 2 to 3 years of age followed by later bladder neck reconstruction. The current modification of the staged repair consists of secure bladder and posterior urethral closure in infancy followed by epispadias repair at 6 to 12 months of age and bladder neck reconstruction when the patient is able to cooperate in a good voiding program. * Complete repair surgery is performed in a single procedure that closes the bladder and the abdomen and repairs the urethra and outer sex organs * A failed classic bladder exstrophy (CBE) closure is defined by wound dehiscence, bladder prolapse, bladder outlet obstruction, or formation of a vesicocutaneous fistula.
Study Type
OBSERVATIONAL
Enrollment
10
primary complete bladder exstrophy repair
Evaluate results of the technique used for bladder exstrophy repair
complete bladder exstrophy repair
Time frame: base line
Present recent development in care and repair of these patients
update in surgical technique
Time frame: baseline
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