To understand what bladder exstrophy is, it is important to know something about the bladder. The bladder is a muscle sac in the pelvis that stores urine. Urine is made in the kidneys. The urine travels to the bladder down two tubes called ureters. When the bladder is full of urine, the bladder muscle squeezes the urine out of the body.
Bladder exstrophy is a complicated, rare condition that occurs before a child is born. As the bladder is developing, it forms outside the child’s belly. The word “exstrophy” means “outside.”
How Often Does This Occur?
Bladder exstrophy is rare. It occurs in about one out of every 50,000 live births. It is slightly more common in boys than girls.
Bladder exstrophy is not caused by anything a mother did or did not do during pregnancy. However, bladder exstrophy may happen more often when children are conceived with assisted fertility.
Family history also plays a role.
- There is an increased chance that the child will have the condition if a parent has bladder exstrophy.
- If a family has one child with this condition, there is an increased chance that another child will have it as well.
How Do I Know If My Child Has Bladder Exstrophy?
Children with bladder exstrophy can experience many signs and symptoms. They may include:
- Bladder problems: The bladder is outside the belly at birth. After the bladder is put back into the belly, it may leak urine.
- Split pubic bones: The pubic bones are not joined in the middle. Hips are turned outward.
- Epispadias: The urethra, which is the hollow tube that drains urine from the bladder to the outside of the body, is not formed completely. The hole that your child urinates from may be in a different place in their private area.
- Abnormal development of outer sex organs:
- Boys: The penis may appear shorter and curve upward (chordee). The testicles may not be in a normal position in the scrotum. A hernia may also be present.
- Girls: The clitoris and labia minora are split and spread apart. The vagina and urethra are shorter than usual. Usually the uterus, fallopian tubes and ovaries develop normally.
- Displaced or missing belly button and/or an umbilical hernia. The child’s umbilical cord inserts low in the belly, just above the bladder.
- Kidney problems: Some children may have extra kidneys or kidneys not in the usual place.
- Vesicoureteral reflux (VUR): Urine flows backward from the bladder up to the kidneys.
What Is Cloacal Exstrophy?
Cloacal exstrophy is the most severe form of bladder exstrophy. The child’s bladder and part of the intestines form outside the body.
Cloacal exstrophy affects the genitals and can affect the rectum, pelvis and spinal cord. Children with cloacal exstrophy may experience additional problems, including:
- Spina bifida: An opening in the spine
- Bowel problems: Part of the bowel is outside the belly at birth. The bowel is shorter than normal.
- Spinal lipoma or tethered spinal cord: This can cause problems with the legs, bladder and rectum because the nerves can be stretched.
> Learn more about cloacal exstrophy.
How Is Bladder Exstrophy Diagnosed?
Bladder exstrophy often can be diagnosed before birth through a routine prenatal ultrasound. It will show that the baby’s bladder is not filling and emptying normally.
Your doctor may refer you to a fetal care center for additional tests if your unborn child is diagnosed with bladder exstrophy.
> Learn about the Cincinnati Children's Fetal Care Center.
In many cases, bladder exstrophy is not diagnosed before birth. But the signs of this condition are visible once a baby is born. The bladder will clearly be seen outside of the baby’s belly. Additional tests may be done after birth to help the doctors determine the plan of care for your child.
How Is Bladder Exstrophy Treated?
Bladder exstrophy is treated with surgery by a pediatric urologist. A pediatric orthopedic surgeon will work with the urologist to put the child’s pubic bones in the proper place.
Different hospitals offer different approaches to surgery for bladder exstrophy. Sometimes the repair can take place in one surgery. At other times the repair takes several surgeries over months or years. The goal is to plan the surgical approach that will best meet your child’s needs as they grow.
What Happens after Surgery?
After surgery, children with bladder exstrophy will require lifelong follow-up care. This helps ensure the best health and quality of life possible.
Children with bladder exstrophy benefit greatly when they receive ongoing care from a variety of team members.
The care team will provide a personalized care plan. They will monitor your child throughout childhood and adolescence. Your child will be scheduled for regular follow-up visits and testing to ensure their bladder and kidneys continue to develop in a healthy way.
It is very common for children with bladder exstrophy to experience emotional issues related to their condition. Parents may struggle with complicated feelings about having a child with this condition. A psychologist can be part of the care team to help your family understand these feelings and deal with them together.
With surgery and other treatment, children with bladder and cloacal exstrophy can have a happy, productive life with normal life expectancy. Some males with bladder exstrophy grow up to experience fertility issues, but fertility assistance is available to help them conceive a child.