Children's Hospital Colorado

Bladder Exstrophy

What is bladder exstrophy?

In babies with bladder exstrophy, the skin, muscle and pelvic hip bones of the lower belly are malformed, causing the bladder to protrude from the skin. The genitals can also be deformed and the muscles surrounding the bladder and urethra fail to function properly. Without normal muscle and neurological function, future incontinence – the inability to control urination – is at risk without surgery.

What causes bladder exstrophy?

This condition occurs in as few as 1 in 50,000 live births. However, a family history makes it more likely. Children born to a parent with bladder exstrophy have about a 1-in-70 chance of having it, and if parents have one child with this condition, they have a 1-in-100 chance of having another child with it.

Recent studies also suggest that children born as the result of fertility treatments are 7 times more likely to have bladder exstrophy than those conceived without fertility assistance. There is a slightly increased rate of bladder exstrophy in mothers who use progestins (a natural or synthetic steroid hormone, such as progesterone, to maintain pregnancy). Males are five times more likely than females to be diagnosed.

In some cases, bladder exstrophy is detected from a routine prenatal ultrasound. In other cases, it isn't diagnosed until birth, when physicians can clearly see the exposed bladder.

Prenatally, ultrasound and MRI together often assist in making this diagnosis.

At birth, the diagnosis of bladder exstrophy is made when:

  • Part of the bladder extends out beneath the umbilical cord
  • Abdominal muscles go in opposite directions
  • In male babies, the penis is short and wide with the tip of the penis split in the middle
  • In females, the mons pubis (the rounded mass of fatty tissue lying over the pubic bones), clitoris and labia are separated, and the opening of the vagina or anus may be pushed more towards the abdomen

Likely, this diagnosis will not affect your pregnancy, but delivery at the Colorado Fetal Care Center may be recommended. This way, our highly trained team will be available to care for your newborn child. A unique treatment plan will be created by your fetal care team to ensure the best outcomes for your child during and after pregnancy.

The treatment for fetal bladder exstrophy is different with each child. The Colorado Fetal Care Center offers the experience and expertise of our fetal care team to provide the widest range of treatment options for this condition.

Babies born with this condition generally receive at least one bladder exstrophy surgery, but most children require multiple operations. The first priority is to close the bladder, the back of the urethra and the pelvic bones, while ensuring that the internal organs are enclosed within the abdomen. Surgeons also rebuild the external sex organs and fix the bladder so it can provide urinary continence.

The number of surgeries depends on the details of each case, but physicians often follow a phased approach to bladder exstrophy surgery. This means they start at birth by closing the bladder and pelvis and repair the urethra and genitals later in infancy. Then, when a child is ready for potty training and his or bladder is large enough, physicians perform the bladder exstrophy surgery necessary for continence (usually around age 4).

What is the long-term outcome after surgery for bladder exstrophy?

Reconstructive surgery to correct the defects associated with bladder exstrophy have positive results, with most patients achieving functional use of their bladder, urethra, pelvic bones and genitals by potty-training-age. Even in more complex cases that require longer-term management, reconstructive surgery can allow patients to reach puberty with successful results.

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