Sometimes problems occur in utero with the way the bladder forms. Bladder exstrophy is a congenital birth defect that is the malformation of the bladder and urethra, in which the bladder is turned "inside out” and the lower portion of the bladder fails to form correctly.
The skin, muscle, and pelvic bones at the lower part of the belly are not joined. Instead of forming into its normal round shape, the bladder instead is flattened and exposed outside the body. The urethra and genitalia are not formed completely, and the anus and vagina appear anteriorly displaced.
This condition is rare and on average, it occurs in about 1 out of every 50,000 births. It is slightly more common in males than females by 2 to 1. Family history plays a role. Children born to a parent with bladder exstrophy have about a 1 in 70 chance of having it. If a family has one child with this condition, they have a 1 in 100 chance of having another child with it.
It is unknown what causes bladder exstrophy. The problem occurs somewhere between four and 10 weeks of pregnancy when various organs, tissues and muscles begin to form layers that separate, divide and fold.
Bladder Exstrophy Treatment
Each case is different and your doctor will decide which surgery and treatment plan is best for your child. Bladder exstrophy requires surgical repair usually involving staged "reconstruction." The primary goals for reconstruction are closure of the bladder and urethra, closure of the abdominal wall, preservation of kidney and sexual function, improved appearance of genitalia, and urinary continence. There are usually three stages of reconstruction.
- 1st Stage - Closure of bladder and abdomen (24-48 hours of life)
- 2nd Stage - Epispadius repair (2-3 years old)
- 3rd Stage - Achieve urinary continence (4-5 years old)
Other procedures your child may require are bladder augmentation, reimplantation of ureters or mitrofanoff stoma.
Will Bladder Exstrophy Affect My Child's Health and Development?
In the majority of cases the children are healthy with normal intelligence and normal physical and social development. The child may have a waddling gait which will become less obvious as he/she gets older. Extra care will be needed for urinary control.