Epispadias is a rare congenital (present at birth) anomaly involving the development of the urethra (the tube that empties urine from the bladder). The urethra does not develop into a full tube and the urine exits the body from an abnormal location. In boys with epispadias, the urethra generally opens on the top or side of the penis rather than the tip. From the meatal opening to the tip of the penis, the penis is split and is opened, forming a gutter. The penis also usually has some degree of curvature or chordee. In girls, the opening of the urethra is usually between the clitoris and the labia, but may be in the belly area.
The causes of epispadias are unknown at this time. It may be related to improper development of the pubic bone. Epispadias is associated with bladder exstrophy, an uncommon birth defect in which the bladder is inside out, and sticks through the abdominal wall. Nearly all boys with bladder exstrophy will also have epispadias. Most girls with exstrophy also have epispadias. Epispadias can occur in both boys and girls who are otherwise healthy with no other abnormalities.
Epispadias occurs in 1 in 117,000 newborn boys and 1 in 484,000 newborn girls. The condition is usually diagnosed at birth or shortly thereafter, based on a physical examination. Very mild cases may be missed at birth and not be diagnosed until later, if the child (usually female) leaks urine after toilet training.
Classification of epispadias is based on the location of the meatus (opening where the urine comes out) on the penis. It can be positioned on the glans (glanular), along the shaft of the penis (penile) or near the pubic bone (penopubic). The position of the meatus is important because it predicts the degree to which the bladder can store urine (continence). The closer the meatus is to the base of the penis, the more likely the bladder will not hold urine.
Surgical repair of epispadias is recommended in patients where the epispadias is more than mild. Our surgical team includes urologists and an orthopaedic surgeon who work together to do this repair. Our research has shown that having an orthopaedist do osteotomies during the first surgery makes a difference in long-term continence, especially for our patients with epispadias.
Leakage of urine (incontinence) is common in children with epispadias. A second operation or operations may be necessary to correct incontinence. In the Division of Urology, our comprehensive team of epispadias experts will help your family determine what is best for your child.
As your child nears adulthood, our dedicated Urology Transitional Care Program is here to help you and your child prepare for the transition from pediatric to adult medical care. For patients with complex urologic conditions like epispadias, it is especially important that the care they receive remains effective and streamlined. Learn more about how this collaboration between CHOP and the Hospital of the University of Pennsylvania (HUP) can support you through this process.
Reviewed by: Division of Urology
Date: May 2011