What is it?
Bladder exstrophy is a congenital abnormality in the formation of the bladder and associated structures. “Exstrophy” is a Greek word, meaning “turn inside out”. This is a fairly ac Instead of having a normal, round shape it is flattened and and the bladder is left exposed to the external world. It is very rare and occurs in one of every 30,000 to 50,000 births. The probability of a family having more than one child with exstrophy is one in 100. Children born to a parent with exstrophy have a risk of one in 70 having exstrophy. Recent published evidence suggests that children born with the assistance of fertility techniques run the risk of having bladder exstrophy seven times higher than children conceived naturally.
How is it diagnosed?
Most of the time bladder exstrophy can be detected before birth with frequent ultrasounds, but usually the diagnosis is not made until the baby is born.
What causes it?
There is no exact known cause for exstrophy but it can develop as early as 4 to 5 weeks after conception. There are no known risk factors for this condition but studies have found that it is more common in boys and infants whose mothers were young when giving birth or had tobacco exposure .
Bladder exstrophy requires surgery. There are several surgical treatment options available for each child’s situation. Surgery needs to be done speedily within the first 48 hours of birth in order to prevent infection from damaging the kidneys. Surgical repair will involve closing the bladder wall and reconstructing the bladder neck to restore urinary continence while preserving kidney function and improving the appearance and function of the genitals in both males and females.
Surgery generally has three stages known as staged reconstructive surgeries. During the first stage the pelvic bones are brought together and the bladder is closed. The second stage involves reconstruction of the genitals to improve appearance and function of the male penis and external genitalia of the female.
The final stage entails reconstructing the bladder and urinary tract. Enlargement of the bladder is performed is necessary. Continence can be achieved in up to 73-78 percent of cases if the bladder is well developed with sufficient capacity. In some instances, the bladder is of poor quality and unfit for closure the regular way. In this case, other techniques will be used. With several techniques of modern reconstructive surgery available, it is extremely rare for a patient with exstrophy to reach adolescence without any treatment.
To know more about bladder exstrophy contact Dr. Grace Hyun, the best female pediatric urologist in NYC. Services include: