Bladder exstrophy is a condition in which the urinary bladder is outside of the body at the time of birth. Exstrophy includes a variety of urological malformations ranging from mild to severe. It can be identified before birth, but most children born with bladder exstrophy need surgical solutions.
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What Is Bladder Exstrophy?
Bladder exstrophy is a rare congenital condition in which the urinary bladder is on the outside of the body and turned inside out at birth. People who are born with the condition are at an increased risk of having children with exstrophy.
Bladder exstrophy includes a spectrum of urologic malformations ranging from mild to severe, which may affect the genitals, pelvic floor, and abdominal wall. Milder forms may include epispadias, a malformation causing the urethra to have an opening somewhere else along the upper side of the penis, rather than in the tip.
In the classic form of exstrophy, the bladder and related structures are open and turned outside of the body. The exstrophy can also cause a separation in the pubic bones, which normally join at the front of the pelvis.
Cloacal exstrophy, the most complex and severe form of exstrophy, involves complications of the bowel.
How common is bladder exstrophy?
Bladder exstrophy is a rare condition, affecting fewer than 50,00 people in the U.S. It is estimated that people with the condition have a one in 70 chance of having a child with exstrophy as well.
What causes bladder exstrophy?
Bladder exstrophy is caused by a mutation or change in DNA. These mutations can occur due to genetics, UV exposure, or viral infections.
Bladder exstrophy risk factors
Bladder exstrophy is a genetic disease and a rare one. There is little known about what causes bladder exstrophy or who is at risk of it, outside of the family connections to DNA mutations.
What Are the Signs and Symptoms of Bladder Exstrophy?
The symptoms of bladder exstrophy are recognizable at birth, although it may take some time to understand the full extent of the condition.
Exstrophy can affect the anus, abdominal wall, genitals, pelvic floor, and uterus.
When should I see a doctor about my bladder exstrophy symptoms?
If your child is born outside a hospital, you should consult your doctor immediately to begin preparing to treat your child’s exstrophy.
How Do You Diagnose Bladder Exstrophy?
The effects of bladder exstrophy can be recognized at birth as defects or malformations of the bladder and pelvic region. As it is a rare condition, it can take time for doctors to fully understand the extent of the exstrophy. Doctors will use imaging technologies and physical examinations to assess your child's condition.
How Do You Treat Bladder Exstrophy?
Typically, the only solution for the symptoms of bladder exstrophy is surgery. The complexity of the surgery is dependent on the severity of the malformations.
Bladder and pelvic closure are carried out in the newborn period. Epispadias repair in males typically occurs during the first few years of life, and an operation to correct urine flow and improve continence is performed between the ages of 3 and 6.
What happens during bladder exstrophy surgery?
Your doctors and surgeons will prepare a personalized plan to treat your child’s symptoms of exstrophy. Each case of this rare condition is unique, so your doctors will design a personalized treatment plan tailored to your child's specific needs.
How effective is treatment?
Treatment effectiveness depends on the severity of the condition. Exstrophy can affect many different structures and regions of the groin, which can complicate surgery or require additional surgeries.
Why Choose UPMC for Bladder Exstrophy Care?
The Department of Pediatric Urology at UPMC Children's Hospital of Pittsburgh is the only one in Pittsburgh that specializes in the treatment and management of all forms of bladder exstrophy. 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.
Doctors at UPMC Children's were 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.
