Bladder Exstrophy and Epispadias

Also known as: bladder exstrophy, epispadias.

What are bladder exstrophy and epispadias?

Bladder exstrophy is a complex congenital (before birth) defect that develops in which the bladder is inside out and lies exposed on the outside of the abdomen. The disorder also usually involves abnormalities of the urinary tract, skeletal muscles and bones, and the intestinal system.  

Epispadias, usually seen with exstrophy of the bladder, is a defect in which the urethral opening (the tube that drains urine from the bladder to the outside) is found either on the top side of the penis (in boys) or in girls, is larger than normal and placed closer to the bladder. These defects frequently occur together.

What causes bladder exstrophy and epispadias? 

The exact cause of bladder exstrophy and epispadias is not clear. There may be a genetic component (its more common in boys), and while it may be seen in more than one member of a family, it’s rare to do so.

What are the symptoms of bladder exstrophy and epispadias?

The disorder may be mild to severe and is often associated with  wide pubic bones, abnormally rotated legs and feet, abnormal/weak abdominal muscles with an umbilical hernia where the umbilicus (belly button) sits above the bladder. In boys there is a short small penis with epispadias while in girls the vaginal opening is narrow with wide labia and a short urethra. Complications of bladder exstrophy and epispadias include incontinence, urinary reflux, infertility and an increased risk of infections.

What are bladder exstrophy and epispadias care options? 

Reconstructive surgery is typically undertaken within a few days after birth and may be performed in stages. Supportive care, both short and long term to ensure mother baby bonding and continuing through the following 4-5 years through the final surgery, is beneficial.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 3/30/2018 7:57:14 AM

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