Bladder exstrophy is a condition in which the urinary bladder is outside of the body at the time of birth.
Exstrophy includes a spectrum of urologic malformations that range from mild to severe. Epispadias, the milder form, is a condition where the urethra (the tube that carries urine out of the body from th e bladder) opens on the top surface of the penis instead of on the tip. In classic bladder exstrophy, the bladder and related structures are open and located outside the body.
The pubic bones, which are normally joined to form the front of the pelvis, are separated. Cloacal exstrophy, the most complex and severe form of bladder exstrophy, also involves the bowel.
Bladder exstrophy is congenital (children are born with it) and rare, occurring in only three of every 100,000 live births. Although the causes of exstrophy are not known, there is some evidence that genetic factors may play a role. P>
The risk of recurrence of exstrophy in a given family is one out of 275 births. There is a one in 70 chance a parent with exstrophy will have a child with the same disorder.
Bladder exstrophy can sometimes be diagnosed with a prenatal ultrasound, but the diagnosis is usually made at the time of birth.
Regardless of the stage at which the diagnosis is made, it is important that the child be referred to a pediatric urologist with experience in treating and managing this rare condition.
Reconstructive surgery is required to treat bladder exstrophy. The complexity of the surgery depends on the extent of the malformation. The best results with classic bladder exstrophy have been achieved with staged reconstruction, a series of surgeries that take place over a number of years.
Bladder and pelvic closure are carried out in the newborn period. Epispadias repair in the male occurs during the first years of life, and an operation to correct urine flow and improve continence is carried out between ages 3 and 6.
Some infants will be candidates for newborn closure with epispadias repair at the same time. With appropriate management from the newborn period, the child has a much greater likelihood of having a functional urinary tract and an excellent qu ality of life.
The Department of Pediatric Urology at Childre n's Hospital of Pittsburgh of UPMC is the only one in Pittsburgh that specializes in the treatment and management of all forms of bladder exstrophy. Steven Docimo, MD, director of pediatric urology, worked in the bladder exstrophy clinic at the Johns Hopkins Hospital for many yea rs before coming to Pittsburgh.
Particular areas of expertise include reconstructive surgery for classic bladder exstrophy and reconstruction for failed exstrophy and epispadias repair. Adults with a history of bladder exstrophy and related issues are also seen.
Dr. Docimo was the first to apply laparoscopic-assisted reconstructive techniques to children and adults with bladder exstrophy, allowing major reconstruction with shorter hospital stays and smaller abdominal incisions.
For patient referral or consultations, contact the Department of Urology at 412-692-4100.
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