What is bladder exstrophy?
Bladder exstrophy is a complex, rare disorder that occurs early on while a fetus is developing in the womb. As the bladder is developing, the abdominal wall does not fully form, leaving the pubic bones separated and the bladder exposed to the outside skin surface through an opening in the lower abdominal wall. Because the bladder and urethra are not closed, the bladder cannot store urine. Urine produced by the kidneys drains into this open area.
Bladder exstrophy occurs in approximately 1 in every 50,000 live births and is slightly more common in males. The disorder may occur in varying degrees and may involve other organs, including the bowel, external genitalia and pelvic bones.
Treatment for bladder exstrophy includes surgical repair. The goal of treatment is to optimize urinary control, to preserve normal renal function, and to optimize the appearance and function of the external genitalia. If left untreated, normal urine continence does not occur and normal sexual function is compromised.
Why Choose Us?
The Division of Urology at Children's Hospital of Philadelphia (CHOP) is a world leader in pediatric urology, offering specialized care for a wide range of urologic disorders in children and adolescents.
At Children’s Hospital of Philadelphia (CHOP), your child will receive coordinated, specialized care from a multidisciplinary team with extensive experience treating children with bladder exstrophy.
Bladder exstrophy diagnosis and evaluation
Exstrophy of the bladder can usually be diagnosed by fetal ultrasound before an infant is born. Bladder exstrophy is suspected when ultrasound shows that the baby’s bladder is not filling and emptying normally.
If doctors believe your unborn baby has bladder exstrophy, we will schedule you for a comprehensive prenatal evaluation at the Center for Fetal Diagnosis and Treatment. Before your baby is born, you’ll also meet with the exstrophy team in the Division of Urology.
If bladder exstrophy is not prenatally diagnosed, the bladder defect is easily visible after birth.
Treatment for bladder exstrophy
The goals of bladder exstrophy treatment are to improve quality of life, continence, and body image for children born with bladder exstrophy.
Families who come to Children's Hospital of Philadelphia for bladder exstrophy repair will have access to the Multi-Institutional Bladder Exstrophy Consortium (MIBEC). Comprised of expert pediatric urologists from Children’s Hospital of Philadelphia, Boston Children's Hospital and Children's Hospital of Wisconsin, the goal of this multicenter team is to provide expert surgical care of babies born with bladder exstrophy and epispadias.
Complete primary repair of bladder exstrophy
Bladder exstrophy can be repaired with reconstructive surgery. If your child is treated at Children’s Hospital of Philadelphia, their surgery will be done using an approach called the complete primary repair of exstrophy (CPRE).
CPRE allows us to achieve the goals of surgical correction all in one operation:
- Bladder closure (moving it inside the body)
- Epispadias repair with reconstruction of the genitalia
- Bladder neck reconstruction (reconstruction of the urinary sphincter muscles)
- Pelvic osteotomy (closing the pelvic bones)
Closing the bladder early allows the bladder to cycle (fill and empty urine) which helps with bladder growth and development. Surgery is typically performed within six to 12 weeks after delivery. This gives parents an opportunity to bond with their newborn and allow the baby to grow healthy and strong.
After surgery, your child will be admitted to the hospital for approximately three weeks to allow him to heal. During this time your child will be in a hip spica cast that is split in half to allow access to the surgical incisions, skin and groin. Most children will be in the cast for about four weeks.