BrynLee's Condition

Bladder Exstrophy is a congenital anomality in which the urinary bladder is present outside the body. It is rare, occurring once every 50,000 live births with a 2:1 male:female ratio. The diagnosis involves a spectrum of anomalies of the lower abdominal wall, bladder, anterior bony pelvis, and external genitalia. It occurs due to failure of the abdominal wall to close during fetal development and results in protrusion of the posterior bladder wall through the lower abdominal wall.
Treatment is with surgical correction of the defect, but patients can still have long term issues with urinary tract infections and urinary incontinence.
The cause of bladder exstrophy is maldevelopment of the lower abdominal wall, leading to a rupture which causes the bladder to communicate with the amniotic fluid.

Modern therapy is aimed at surgical reconstruction of the bladder and genitalia, most often through a staged approach. The initial surgical management consists of bladder, posterior urethral and abdominal wall closure (with or without osteotomies). Bladder closure may be delayed if the bladder is deemed too small to close. The surgical management epispadius usually occurs at approximately 6–12 months of age. Reconstruction of the bladder neck is typically done at toilet training age (approximately age 4-5), once the child is able to and interested in participating in a bladder retraining program. In very carefully selected patients surgical management may be carried out in a single stage or in combined procedures.
Even with successful surgery, patients may have long-term problems with

• incontinence
• urinary reflux
• repeated urinary tract infections
• bladder adenocarcinoma
• colonic adenocarcinoma
• self-image
• uterine prolapse

Sexual function and libido are normal in exstrophy patients. Successful pregnancies and delivery in exstrophy patients have been reported.