When your child is treated in the Bladder Exstrophy Program here at Children’s Hospital of Philadelphia (CHOP), you can expect them to receive coordinated, specialized care from our team of experts. Physicians and surgeons from many of our hospital departments including urology, orthopedics, physical therapy, psychology and more all work together. They use their extensive experience treating children with bladder exstrophy to help your child heal and stay dry.

Early diagnosis and prenatal care

If you are an expectant family who has received a diagnosis of suspected bladder exstrophy, epispadias or cloacal exstrophy at a prenatal appointment, our team will evaluate you to confirm the diagnosis and determine a treatment plan, if needed. You can expect to have a series of outpatient appointments including:

• Antenatal ultrasound imaging and a fetal MRI with the team in our Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment to confirm and clarify the details around your diagnosis
• Meetings with our nurse practitioners and attending urologists who specialize in caring for bladder exstrophy, epispadias and cloacal exstrophy to help make sure you understand your diagnosis and the next steps
• Consultations to educate your family on every step of your child’s path from delivery to your initial stay in our Newborn/Infant Intensive Care Unit (N/IICU), your child’s surgery and care after surgery, and the lifelong expectations for your child’s care

Before surgery and after delivery

If your child was diagnosed before birth, your scheduled delivery may occur in our Garbose Family Special Delivery Unit. After your child is born and stabilized in our N/IICU:

• Our team will conduct additional imaging including kidney (renal) ultrasounds, pelvic X-rays and a hip ultrasound to check for separation between the pubic bones (pubic diastasis) and potential hip issues.
• We will teach you how to manage the part of the bladder visible on the outside of your child’s body (bladder plate) while at home.
• We will see you back in our multidisciplinary clinic when your child is around six weeks old to review their surgery plan.
• Many boys will receive testosterone injections six weeks and three weeks before the operation.
• We will schedule your child’s surgery once all pre-surgery testing is complete.
• If your child is born with cloacal exstrophy, they will undergo a surgery before they leave the hospital, as a first step in their long-term care. 

Your child’s surgery and hospital stay

When your child is between three and six months old, we will perform a procedure called a complete primary repair of bladder exstrophy (CPRE). The CPRE includes several carefully planned steps to rebuild and protect the bladder and surrounding areas:

• Our orthopedics team will make surgical cuts in your child's hip bones to help bring their pelvic bones together (bilateral iliac osteotomies).
• Our anesthesiologists will manage your child’s pain and monitor them during the entire procedure. This might include an epidural or central line (complex anesthesia).
• We will close your child’s bladder to restore its shape and position (bladder closure).
• We will repair the opening on the top side of your child's urethra so urine can flow normally (epispadias repair).
• We will rebuild your child's belly button for a natural appearance (umbilicoplasty).
• We also perform surgeries to repair only the end of the urethra (distal epispadias) or to correct isolated cases of epispadias when it is not part of a larger bladder condition.
• For children with cloacal exstrophy, the second surgery will occur much later, often between two and three years of age. 

Later, all children will have procedures to look inside the bladder (cystoscopy) to check healing and function. 

Some children may need follow-up surgeries, like redoing the bladder neck repair or repositioning the ureters in the bladder (ureteral reimplantation).

Caring for your child after surgery

After surgery, your child will be in a brace to keep their hips and legs in place (spica brace). They will stay in the hospital recovering for two to three weeks, then continue to recover at home. Their inpatient care will include:

• A two to five day stay in our N/IICU after their operation to make sure their pain is under control.
• Management of the tubes that will aid in their healing and help empty their bladder like ureteral stents, bladder suprapubic tubes and urethral stents.
• Patient and family education for taking care of tubes and changing your baby’s diapers when they can go home.
• An evaluation by our physical therapy team to make sure your car seat is placed safely in your car for transportation.
• After your child goes home, they will return for imaging once a week for three to four weeks, until the suprapubic tube can be removed. 

Long-term follow up

After discharge, your child will return to CHOP for follow-up appointments:

• Every three months for the first year
• Every six months for one year
• And then yearly

They will have a renal and bladder ultrasound at each visit, and a pelvic X-ray each year.

Second opinions

If your child was not delivered at CHOP and you were referred to us for a second opinion, you can schedule your first appointment with one of our exstrophy surgeons. Before your child’s scheduled visit, one of our team members will reach out to you to ask for your child’s medical history, to have their medical record transferred to CHOP and to have insurance forms filled out and returned. Please bring any images (ultrasounds, X-rays, etc.) previously done to your child’s first appointment.

As a first-time patient, you can expect a 30- to 60-minute visit. This appointment will include a physical exam, a consultation with a nurse practitioner or attending physician to discuss future care and home care and an opportunity to have your questions answered.

Our scheduling coordinators will schedule all follow-up appointments and testing prescribed by your child’s attending pediatric urology physician, pediatric orthopedic surgeon or nurse practitioner. Follow-up appointments will typically be shorter than the initial meeting, depending on the severity of your child’s condition.

Team members you meet with may include:

• A nurse practitioner to answer your questions, arrange your stay near the hospital if your family is from out of town and connect you to all the resources you need – including other families for support
• A psychologist who can provide counseling for your child and family both before and after surgery
• A physical therapist who will provide early exposure to pelvic floor therapy to ensure your child’s pelvic area develops properly, before and after surgery, so they can eventually stay dry (achieve continence)