Bladder Exstrophy-Epispadias Complex

Bladder exstrophy epispadias complex describes a problem in the development of the lower abdominal wall, bladder, and urethra that results in the bladder being open and exposed to the outside of the body.  This problem may occur in boys or girls.  In boys, the penis is usually short, upwardly curved (dorsal chordee), and the urethra is split open and rests along the top of the penis (male epispadias).  In girls, the open urethra passes between the clitoris, which is separated by the urethral plate into two halves (female epispadias).  The bony pelvis is also split open so that the pubic bones, which usually come together just above the penis or clitoris, are separated (diastasis).  Although epispadias may occur without bladder exstrophy, these two problems usually occur together.   The most severe form of this problem is called cloacal exstrophy.  In this case, bowel segments are also open and present outside the body in addition to the bladder and urethra.

The incidence of bladder exstrophy is approximately 1 in 30,000, and the incidence of cloacal exstrophy is approximately 1 in 100,000.

What Is the Cause of Exstrophy and Epispadias?

These developmental problems occur around 4 to 8 weeks of gestation, but the cause remains unknown.

How Are Exstrophy and Epispadias Diagnosed?

Exstrophy may be diagnosed before delivery by prenatal ultrasounds.  When this diagnosis is suspected prenatally, a pediatric urologist is usually consulted so that parents can prepare and understand how their child will be cared for after delivery.  Initial management includes protecting the delicate open bladder and imaging the kidneys by ultrasound and plain pelvis films.  Abnormalities in kidney development or position may be present.  Children with cloacal exstrophy will also have spine imaging with ultrasound to look for spinal cord abnormalities.  The exposed bladder is protected with a thin plastic film (Saran wrap) until closure is performed.  It is important to avoid exposure of your child to latex to avoid allergies to latex later in life.

How Are Exstrophy and Epispadias Treated?

The goals of reconstructive surgery are to improve the appearance of the genitals, provide urinary control, protect kidney function, and allow sexual function.  This is achieved by closure of the bladder and urethra, reconstruction of the penis and clitoris, and closing the abdominal wall and pelvic bones in front of the bladder.  Closure is often accomplished as a single-stage procedure (complete primary repair for exstrophy, CPRE) within the first few days of life.  Less often, the closure is delayed to allow the baby to grow and develop, or the procedure may be done in stages.  Once the surgery is completed, babies with exstrophy are kept in a lower body (spica) cast for four weeks to protect the closure of the pelvic bones and allow healing.  A bladder catheter keeps the bladder empty while it is healing.  Instructions for wound care, antibiotics, and pain medications are given before discharge home.  The support of our urology team allows parents to feel comfortable with home care at the time of discharge.  Hospitalization is usually 2 to 4 weeks.  When feasible, CPRE may give the bladder a better chance to fill and empty at an early age, which seems to stimulate the growth and development of the bladder.

Although near-normal bladder function with regular voiding and continence are possible after a single procedure, most children require additional surgeries.  Reshaping the bladder neck to prevent unwanted urine leakage through the urethra may be necessary.  Occasionally, the bladder remains small and stiff, and it must be enlarged using a patch of bowel (bladder augmentation).  If bladder emptying by urination is not possible, then using a catheter to drain the bladder at regular intervals may be necessary.  Most often, children with exstrophy have vesicoureteral reflux (regurgitation of urine from the bladder to the kidneys), which will need to be corrected to prevent kidney infections.  Because there are many exstrophy variations, each patient’s care must be given according to their needs.

Children with corrected bladder exstrophy may remain at an increased risk for urinary tract infections. Regular urology follow-up is required to monitor kidney growth and identify problems that might increase the risk of infection. The long-term outcome for children with exstrophy is excellent.