Bladder Exstrophy

There is no known cause for exstrophy. The problem happens during the first 4 to 8 weeks of pregnancy. This is when organs, muscles and tissues begin to form layers that separate, divide and fold.

Exstrophy is not caused by something the mother did or did not do while she was pregnant. It does not run in families (not hereditary).

While your baby is in the hospital, we avoid using products made with latex. You should also avoid using latex at home, especially in items that touch your baby’s mouth, nose, urethra or other mucous membrane. Products that might contain latex include some pacifiers and nipples for baby bottles.

Contact between mucous membranes and latex raises the risk of forming a latex allergy later in life. Babies with exstrophy are at greater risk for contact because they are born with some mucous membranes (their bladder and sometimes their intestines) outside, instead of inside, their body. Avoiding latex may prevent your child from forming a latex allergy.

After doctors make the diagnosis, your child may have surgery to: 

• Bring the pubic bones together in front
• Place the bladder into the pelvic cavity
• Tighten the muscles where the bladder connects to the urethra to improve control of urine flow
• Fix the tube (urethra) that carries urine from the bladder to the outside of the body if it is short and split (epispadias)
• Close the wall of the belly (abdominal wall)
• Create an opening from the intestines to outside the belly wall so poop (stool) can come out (the surgery is called a colostomy) 

For young babies, we do all these steps in a single surgery (complete primary repair), 3 to 4 months after birth. An orthopedic surgeon closes the pubic bones, and a urologist completes the other steps.

Doing everything at once is more complex than surgery in stages over several years. But 1-stage repair means your child will have better outcomes. The bladder is more likely to grow to normal or nearly normal size, giving your child more control over urine flow. Even so, 50% to 60% of children will need more surgery later in childhood. Your child may need surgery to help improve bladder control or stop backflow of urine from the bladder to the kidneys (vesicoureteral reflux).

Your baby will be in the hospital for 7 to 10 days when they have surgery. They will have: 

• A spica cast. Orthopedic surgeons will put on this special cast. It holds your baby’s pelvis in place until the bone sets. The cast goes from the ankles to the mid-chest, with a hole for the diaper area. This cast will stay on for 4 to 6 weeks or until your baby outgrows it. Your child’s nurses will teach you how to take care of the cast and give you tips for diapering. Occupational therapists will help you pick the right type of car seat to use while your baby is in the cast. You can get special car seats on loan.
• Catheters. Catheters are tubes that drain pee (urine). Your baby will have a catheter called a suprapubic (S-P) tube. The tube goes from their bladder through their belly wall. It drains pee from the bladder into a bag. This keeps the bladder empty so it can heal. Your baby will have the S-P tube for 3 to 4 weeks at home. Your baby may also have 2 ureteral catheters that come out through their urethra. These will fall out on their own in the hospital or the first week after your child leaves. Your child’s nurses will teach you how to take care of the catheters.
• Intravenous line (IV). Your baby will have a tube inserted into a vein to give fluids and antibiotics for several days.
• Heart and breathing monitor. Your baby’s heart rate, breathing and oxygen levels may be measured for several days.
• Antibiotics. Your child may be on a daily, low dose of antibiotics to help prevent urinary tract infections (UTIs).

Before your baby leaves the hospital, your child’s nurse will teach you how to care for your baby at home. You can call the Urology Clinic to ask questions at any time. 

• Your child will have weekly appointments in the Urology Clinic for 6 weeks after leaving the hospital, until the spica cast and bladder catheters are removed.
• After that, visits are every 2 to 3 months. Your baby will have X-rays or ultrasounds during many of the visits. 

Your child should see their regular doctor (pediatrician) for well-child checkups. Your child may get more UTIs than other children. Their pediatrician can check for and treat any infections.

When your child is school-age, they should see their urologist at least once a year. This doctor will check that their kidneys are growing well and will look for any signs of kidney problems, like swelling due to blockage or backup of urine. This is called hydronephrosis (PDF). If your child is having problems, they will need to see the urologist more often.

Some children who had surgery for exstrophy gain complete control over urine flow, and they have no other issues later. About 60% of children will need treatment later in childhood. Treatment may include: 

• If pee (urine) flows from your child’s bladder back to their kidneys (vesicoureteral reflux), they may need to take antibiotics, most likely for a year or more. This helps prevent urinary tract infections. As they get older, they may need treatment to reduce the backflow of urine. Treatment options include surgery or an injection of a gel (Deflux) into their bladder. This creates a bulge where the tube from the kidney enters the bladder and makes it harder for the urine to flow back up to the kidneys. Learn more about vesicoureteral reflux (PDF).
• If leaking of urine (incontinence) is a problem, your child may need surgery to tighten the group of muscles that connect the bladder to the urethra (bladder neck).
• Some children need surgery to enlarge their bladder. This is called bladder augmentation (PDF) (Dari) (Spanish).
• Some children need a tube placed so they can empty their bladder by putting a catheter through an opening in their belly wall. This is called a Mitrofanoff channel (PDF) (Dari) (Spanish).
• Boys may need surgery on the penis if the opening is on the underside, not at the tip. This is called hypospadias (PDF) (Amharic) (Russian) (Simplified Chinese) (Somali) (Spanish) (Vietnamese).
• Some boys or their parents may want surgery to make the penis look more typical. In boys with exstrophy, the penis is often a bit shorter than typical, but it works normally and has normal feeling. Our Differences in Sex Development team cares for babies, children and teens with all types of conditions that affect genital development. 

Surgeries can often be combined to reduce the number of operations on your child.

Lifelong follow-up care is important for good urinary health. For families in the Puget Sound area, close coordination between urologists at Seattle Children’s and at the University of Washington and Harborview Medical Center helps ensure proper care when our patients become adults.

As an adult, your child should have normal sexual feeling and function and potential for having a baby (fertility).

• Surgeons, doctors and nurses who have experience with all aspects of bladder exstrophy will take care of your child.
• We are the region’s only referral center for patients born with bladder exstrophy. Our surgeons are experts at repairing this condition in babies. They also are skilled at procedures children may need later in childhood.
• Based on the organs affected, your child’s urologist may involve other specialists through our Reconstructive Pelvic Medicine Clinic. Your child’s care team may include a gynecologist, gastroenterologist and orthopedic surgeon. Our team approach means your child gets coordinated care each step of the way from experts who know your child’s full treatment plan.
• We arrange your clinic visits so your child can see all the providers they need in one day, in one place, instead of having to make separate visits.

• A complete team cares for your child to meet their early and long-term needs. We provide the right treatment for your child at the right time, until age 21.
• If an ultrasound during pregnancy shows that your baby may have exstrophy, our Fetal Care and Treatment Center team can monitor your baby’s condition, coordinate with your birth hospital if needed and plan for their care. Finding a problem early gives you more time to make decisions and prepare.
• Many of our patients come to us as young babies for exstrophy repair. Our Level IV Neonatal Intensive Care Unit (NICU) is skilled and equipped to care for babies with the most complex conditions.
• We also treat many older children, including children who have had surgery somewhere else. We provide second opinions, too.
• After surgery to repair their bladder exstrophy, we continue to care for your child in our Bladder Exstrophy Clinic. We understand that problems affecting the genitals or urinary tract can be uncomfortable to deal with. We respect your family’s privacy and feelings as we evaluate and treat your child.
• Children don’t react to illness, pain, surgery or medicine in the same way as adults. They need – and deserve – care designed just for them. We offer educational, social and emotional support that fits your child or teen’s needs.

Our method of repairing bladder exstrophy with a single surgery was developed by Dr. Michael Mitchell, former chief of pediatric urology at Seattle Children’s. The procedure is called complete primary repair.

It allows surgeons to correct the condition in 1 stage, instead of in 3 surgeries over several years.

We perform 1-stage repair when your baby is 3 to 4 months old. Our approach: 

• Gives you time to bond with your baby before they have major surgery
• Lets your newborn grow and their organs develop before anesthesia and surgery
• Improves your child’s quality of life, giving them better control over the flow of urine

• Each child is different, and children’s needs change as they grow and develop. We tailor treatment to your child, working closely with your family through all stages of life. We help with concerns from toilet training to puberty to sexual function. We truly see this as a lifelong partnership.
• Our team takes time to explain your child’s condition and answer all your questions. We help you fully understand your treatment options and make choices that are right for your family.
• We help your child and your family through the challenges of this condition. You’ll get support from child life specialists, social workers and many others, like lactation consultants to help with breastfeeding your baby.
• We connect you to community resources, support groups and other families dealing with exstrophy. Our Reconstructive Pelvic Medicine Program also hosts a yearly family gathering. For more information, email our bladder exstrophy coordinator.
• At Seattle Children’s, we work with many children and families from around the Northwest and beyond. We can help with financial counseling, schooling, housing, transportation, interpreter services and spiritual care. Read about our services for patients and families.

• Seattle Children’s leads the way in research to provide evidence-based care. Dr. Paul Merguerian guides efforts to create clinical pathways to provide care the best way, the same way, to every child with a specific condition. The team is creating clinical pathways for bladder exstrophy and a broad range of urologic conditions, based on scientific research and expert opinion.
• As we care for children over the years, we keep careful records of patients who had surgery for bladder exstrophy. This helps us improve our surgical procedures and follow-up treatments. We publish our research so other urologists can benefit from what we learn.
• We help train surgeons worldwide. A Seattle Children’s pediatric urologist, Dr. Rich Grady, started efforts to teach surgeons in other countries to repair bladder exstrophy with a single surgery. The procedure was developed here at Seattle Children’s. Dr. Grady has passed away, but the work continues.