Exstrophy of the bladder (bladder exstrophy)

Bladder exstrophy—when a child is born with the bladder exposed on the outside of the lower abdominal wall—can seem particularly scary. The good news is that, although this is a rare condition, we have treated many national and international children with bladder exstrophy here at Boston Children’s Hospital Center for Bladder Exstrophy. Here’s a synopsis of the important aspects of the condition and information on the many support services available to your child and you.

• Bladder exstrophy is a complex congenital anomaly, meaning that your child was born with the condition.
• Children with the condition are born with the bladder exposed inside-out on the surface of the body.
• Bladder exstrophy is very rare: Only about 400 babies are born with bladder exstrophy each year in the United States
• It’s more common in boy babies than girl babies.
• In the healthy full-term baby, initial surgery is usually performed at 2-3 months of age.
• With treatment, your child should be able to enjoy an active childhood.
• Children with bladder exstrophy may need extra treatment with medication or surgery in order to have control over their bladders (urinary continence).

How Boston Children’s approaches exstrophy of the bladder

Although this is a rare condition, we have treated many children with bladder exstrophy from across the country and the world here at Boston Children’s Center for Bladder Exstrophy.

Unique approach to treatment

Some hospitals treat bladder exstrophy using a planned three-stage surgical approach called the Modern Staged Repair of Exstrophy (MSRE). The MSRE approach includes separate stages of initial bladder closure (first stage), epispadias repair (second stage) and a third stage surgical procedure when a child is about 5 years old called Bladder Neck Reconstruction (BNR). This last surgery is a standard operation performed in order to help your child become continent of urine (appropriately dry/without leaking urine).

Instead of this method, at Boston Children’s, we use an approach called the Complete Primary Repair of Exstrophy (CPRE). This involves an advanced single-stage surgical procedure during which the surgeon attempts to bring about all of the benefits of the three stages of the staged MSRE approach. Our single-stage surgical approach is usually performed within the first few months after a baby is born.

Family-based care

Our team is aware of the fact that the family is the most important source of emotional support and comfort for children, so we consider family members a vital component of each child's health care team. We encourage you to call any time to speak with your child's nurse or doctor as well as members of our psychosocial support team (a social worker, psychiatric nurse and Child Life specialist).

Network of support

At Boston Children’s, we’ve found it helpful to connect parents and create a support network of families grappling with the effects of exstrophy of the bladder.Additionally, our Exstrophy Support Group, created in 1992, offers programs for families, children and teens with the condition.

Our interdisciplinary team offers additional psychosocial supports, in addition to the services of a social worker, Child Life specialist and psychiatric nurse. The Center for Families, pastoral care, Interpreter Services and Case Management Services can also be key components of the care we extend to your family. Of note is our Center for Families’ Patient Family Housing Program, which can assist you with your accommodation needs if you’re traveling to Children’s from a distance.