Epispadias is a rare congenital (present at birth) abnormality that involves the opening of the urethra (the tube from which urine exit the bladder). In boys with epispadias, the urethra opens in top of the penis rather than the tip. The space between this opening and tip of the penis appears like an open book (gutter). In girls with epispadias, the urethral opening is towards the clitoris or even belly area. This results in the external genitalia and urethra not forming or functioning well.
Most boys and girls are born with genitals that look normal and work well. But some children are born with a condition called epispadias. Epispadias can produce a penis or urethra that don't work well or look normal.
There are many types of surgery that pediatric urologists can use to fix this problem. Here we offer information to help you speak to your child's urologist about options.
Male urinary tract
Female urinary tract
What Happens under Normal Conditions?
The urinary tract is like a plumbing system. It has special "pipes" that allow waste to flow through. The urinary tract is made up of 2 kidneys, 2 ureters, the bladder, and the urethra.
The kidneys act as a filter system for the blood. They remove toxins and keep useful protiens, sugar, salts, and minerals. Urine is the waste product. It is made in the kidneys and flows down two, 10 to 12-inch-long tubes called ureters. The ureters are about a quarter inch wide and have muscled walls. They push urine into the bladder.
The bladder can swell to store the urine until you're ready to drain it. It also closes the path so urine can't flow back into the kidneys.
The tube that carries urine from the bladder out of the body is called the urethra.
In males, the urethra is long. It starts at the bladder and runs through the prostate gland, perineum (the space between the scrotum and the anus), and penis. In females, the urethra is much shorter. It runs from the bladder in front of the vagina and opens outside the body.
Your urethra has muscles called sphincters. The sphincter complex (also called the bladder neck) is a ring-shaped muscle wrapped around the urethra. They help keep the urethra closed so urine doesn't leak before you're ready. These sphincters open up when the bladder contracts so you can release urine.
How Does Epispadias Affect Boys?
Epispadias is quite rare, affecting only 1 in 117,000 males. In boys with epispadias, the penis tends to be broad, short, and curved up (“dorsal chordee”). The pelvic bones are widely separated. Since the penis is attached to these bones, it results in a penis that's pulled back toward the body.
Normally, the opening of the urethra (the “meatus”) is at the tip of the penis. But in boys with epispadias, it's on top of the penis.
From this opening, a groove runs along the top of the penis to the end. The epispadias is classified based on the location of the meatus on the penis.
- Glanular epispadias: It is found on the head of the penis
- Penile epispadias: It is found along the shaft of the penis
- Penopubic epispadias: it is found or near the pubic bone
The position of the meatus can help predict the how well the bladder stores urine. If the meatus is close to the base of the penis (and the abdominal wall), the bladder sphincter is likely affected and it won't hold urine.
In most cases of penopubic epispadias, the bones of the pelvis don't come together in the front. The bladder sphincter doesn't close all the way since it's shaped more like a horseshoe than a ring. Because of this, urine leaks out. Most boys with penopubic epispadias, and about 2 of 3 with penile epispadias leak urine with stress. (Things like coughing and strenuous effort.) Most will need to have the bladder neck fixed with surgery.
Almost all boys with glanular epispadias have a good bladder neck. They can hold urine and toilet train normally. Still, the bend and abnormal opening of the penis will need to be fixed with surgery.
How Does Epispadias Affect Girls?
Epispadias is much more rare in girls, with only 1 of 484,000 cases. Girls who are affected have pubic bones that are separated to different degrees. This keeps the clitoris from connecting in the middle, resulting in 2 halves of the clitoris. The bladder neck is also almost always affected. Girls with epispadias likely leak urine with stress. (Things like coughing and strenuous effort.) In most cases, early surgical treatment can fix these problems.