What is epispadias? During pregnancy, organ formation in the developing baby is a finely organized set of timed events. Epispadias represents a unique error in this process resulting in a malformation of the external genitalia. The problem can also include the urethra bladder and large intestine. The degree to which epispadias affects the genitalia varies. For instance, in boys, it can be just a small dimple on the tip of the penis above the normal opening or with girls, it could be a double clitoris. However, whenever the urethra or bladder are involved the epispadias usually more severe. This large spectrum of malformations is called the exstrophy-epispadias complex. Only rarely does epispadias occur as an isolated defect. How does epispadias affect boys? Fortunately, epispadias is quite rare, with only one in 117,000 males being affected. In affected boys, the penis is typically broad, shortened and curved toward the abdomen (dorsal chordee). The penis is attached to the pelvic bones, which are widely separated, resulting in a penis that is pulled back toward the body. Normally, the meatus is located at the tip of the penis; however, in boys with epispadias, it is located on top of the penis. From this abnormal position to the tip, the penis is split and is opened, forming a gutter. It is as if a knife was inserted into the normal meatus and the skin stripped away on the top of the penis. Classification of epispadias is based on the location of the meatus 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 in that it predicts the degree to which the bladder can store urine (continence). The closer the meatus is to the top base of the penis, the more likely the bladder will not hold urine. In most cases of penopubic epispadias, the bones of the pelvis do not come together in the front. In this situation, the bladder neck cannot close completely and the result is leakage of urine. Most boys with penopubic epispadias and approximately two-thirds of those with penile epispadias have leakage of urine with stress (e.g., coughing and strenuous effort). Ultimately, they may require reconstructive surgery of the bladder neck. Almost all of the boys with glanular epispadias have a good bladder neck. They can hold urine and toilet train normally. However, the penis abnormality (upward bend and abnormal opening) still requires surgical repair. How does epispadias affect girls? Epispadias is much more rare in girls, with only one of 484,000 affected. Those who are affected have pubic bones that are separated to varying degrees. This causes the clitoris not to fuse during development, resulting in two halves of the clitoris. Furthermore, the bladder neck is almost always affected. As a consequence, girls with epispadias invariably leak urine with stress (e.g., coughing and strenuous effort). Fortunately, in most cases, early surgical treatment can resolve these problems.