What to Expect During Care for Bladder Exstrophy

Early diagnosis and prenatal care

Expectant families that come to our hospital for an evaluation after receiving a prenatal diagnosis of a suspected bladder exstrophy/epispadias/cloacal exstrophy will have a series of outpatient appointments that will include:

• 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 specific details around the diagnosis
• Meetings with one of our nurse practitioners and attending urologists who specialize in caring for bladder exstrophy/epispadias/cloacal exstrophy
• Consultations to educate families on every step of their child’s path from delivery to their initial stay in our Newborn/Infant Intensive Care Unit (N/IICU) to surgery and postoperative care, as well as lifelong expectations

Pre-operative course and post-delivery

Scheduled delivery often occurs in our Garbose Family Special Delivery Unit. The following care takes place after a child is born and stabilized in our N/IICU:

• Imaging will include renal ultrasounds and pelvic X-rays. Prior to surgery, our team will obtain a specialized, low-dose CT scan to assess pubic diastasis and potential hip issues.
• In some males, pre-operative testosterone is administered six weeks before the operation and then again three weeks before the operation.
• An inpatient operation will be scheduled. The ideal of age repair is between 4 weeks and 16 weeks of age.

Hospitalization and surgical procedure

The complete primary repair of bladder exstrophy (CPRE) procedure will be performed between 4 weeks and 16 weeks of age.

• The CPRE includes bilateral iliac osteotomies (orthopaedics team), complex anesthesia (epidural, central line), bladder closure, epispadias repair and umbilicoplasty.
• We also perform distal epispadias and isolated epispadias repairs.
• For cloacal exstrophy, first- and second-stage closures and complex reconstruction is done to achieve dryness. Secondary surgeries include redoing bladder neck procedures, ureteral reimplantation surgery and cystoscopy.

Postoperative care

After surgery, your child will be in a spica cast. They will stay in the hospital recovering for four to six weeks. Their inpatient care will include:

• A weeklong stay in the ICU post-operation
• Management of their many tubes (ureteral stents, bladder suprapubic tube, urethral stent, and drain)
• Follow-up about two weeks to a month after surgery

Long-term follow-up

After discharge, your child will return to CHOP for follow-up every three months.

Second opinions

First-time appointments

Typically, first-time appointments can be scheduled by speaking with a coordinator at one of our nine locations. Before your child’s scheduled visit, one of our team members will reach out to you to obtain your child’s medical history, have you’re their medical record transferred to CHOP, and have insurance forms filled out and returned.

First-time patients can expect a visit lasting 30 to 60 minutes. This appointment will include a physical exam, explanations/suggestions for future care and home care by the nurse practitioner and attending physicians, and an opportunity to have any questions you have answered.

Patient families should bring along any images (ultrasounds, X-rays, etc.) previously done.

Follow-up appointments

Follow-up appointments and testing prescribed by your child’s attending pediatric urology physician, pediatric orthopaedic surgeon or nurse practitioner will be scheduled with one of our scheduling coordinators.

Follow-up appointments will typically be shorter than the initial meeting, depending on the severity of the condition.

Team members you may meet with may include:

• Nurse practitioner who is available to answer all of 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 and other families for support.
• Psychologist who can provide counseling for children and families before and after surgery
• Physical therapist who will provide early exposure to pelvic floor focused therapy to ensure proper development to achieve continence