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Epispadias

Most boys and girls are born with fully functioning genitalia. But a congenital condition called epispadias can produce a penis or vagina that not only performs inefficiently, but also looks abnormal. Luckily, pediatric urologists have various surgical techniques at their disposal to repair the ill-placed urethral opening, hooded foreskin and curvature associated with this condition. The following information should help you speak to your child's urologist about diagnosis and treatment.

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.

How is epispadias diagnosed?

The diagnosis of epispadias is typically made at birth. Although, on occasion when the malformation may not be drastic, epispadias has been missed at birth and only becomes apparent when the child (usually female) remains wet after toilet training.

What are the risks associated with epispadias?

Presently, there are no studies that have documented that a child with epispadias is at increased risk for urinary tract infections (UTIs). However, if complete exstrophy-epispadias exists, the child will be at increased risk for UTIs due to a condition called vesicoureteral reflux. The standard of care for these children is to maintain them on antibiotics until the reflux is corrected.

Babies with epispadias are typically healthy and vigorous. They have a low incidence of abnormalities that affect organ systems other than the genitourinary system and the pelvic bones. Therefore, these children do not require extensive diagnostic radiographic studies. On the other hand, babies born with the more severe form of exstrophy-epispadias complex are at a slightly increased risk for the existence of associated malformations like enlarged ureter or vesicoureteral reflux.

Boys with penopubic epispadias or the exstrophy-epispadias complex have problems with their anatomy that may make them infertile. In normal young men, the bladder neck is closed when sperm moves from the testicles to the urethra. The bladder neck also assumes a closed position when sperm is ejaculated from that area. In men with penopubic epispadias or the exstrophy-epispadias complex, the bladder neck may not close completely during ejaculation. This allows the sperm to move backwards into the bladder (retrograde ejaculation). This situation may cause problems when a couple is trying to have children. Some of these patients may have poor sperm quality. Furthermore, dorsal chordee and a short, stubby penis may make sexual intercourse difficult. However, improvements in surgical repair have significantly reduced the severity of these problems. Females with epispadias usually are not at risk for infertility since their internal reproductive organs are normal.

What are some treatment options?

The primary goals of treatment of epispadias are to: maximize penile length and function by correcting dorsal bend and chordee; and create functionality and cosmetically acceptable external genitalia with as few surgical procedures as possible. If the bladder and bladder neck are also involved, surgical treatment is required to establish urinary continence and preserve fertility.

Surgical technique in males: There are two popular surgical techniques that achieve these objectives. The first is the modified Cantwell technique, which involves partial disassembly of the penis and placement of the urethra in a more normal position. The second technique and most recent evolution of the modern epispadias repair is the Mitchell technique. It involves complete disassembly of the penis into its three separate components — two corpora cavernosa and a single corpus spongiosum. Following disassembly, the three components are reassembled such that the urethra is in the most functional and normal position and dorsal chordee is corrected. Both techniques provide a straight urethra positioned on the underside of the penis, correction of chordee and an acceptable cosmetic result. The Mitchell technique has a lower complication rate and facilitates bladder and bladder neck repair. Sometimes, boys with the exstrophy-epispadias complex are born with a very small or severely underdeveloped penis. In these situations, surgical reconstruction of the penis is more difficult.

Surgical technique in females: Genital reconstruction in girls with bladder exstrophy is less complex compared to the reconstruction in boys. The urethra and vagina may be short and near the front of the body and the clitoris is in two parts. The internal female structures — uterus, fallopian tubes and ovaries — are normal. If diagnosed at birth, the two parts of the clitoris can be brought together and the urethra can be placed into the normal position. If repaired early enough, lack of urinary control (incontinence) may not be a problem. If the diagnosis is missed or if early repair is not performed, then incontinence can be surgically corrected at the time of diagnosis. If the vaginal opening is narrow in older girls or younger women, reconstruction can be performed after puberty.

Children with the exstrophy-epispadias complex may require surgery to improve the urethral resistance (bladder neck repair) however with newer methods of primary repair at birth almost 1/3 of the patients can achieve urinary control without the need for other procedures. If, however, the child does not potty train normally. There are several different methods to achieve normal urination function. An initial approach might involve injecting a bulking material around the bladder neck so that urine cannot leak from the bladder. Other surgical methods involve more complex procedures, like the creation of a longer urethral tube or wrapping various materials around the bladder neck.

What can be expected after treatment?

An abnormal hole in the urethral tube used to be quite common after a major epispadias repair. With today's technical improvements, this is fairly uncommon with a rate of formation as low as 6 percent. Persistent dorsal chordee is fairly typical with older reconstructive techniques for epispadias. It is now less common with newer treatment techniques. Epispadias repair does little harm to erectile function.

Frequently asked questions:

Can epispadias be prevented?

There is no known prevention.

What is the best time for surgical treatment?

Several leading reconstructive surgeons have championed early repair of epispadias, especially in boys with penopubic or penile epispadias and all girls with the condition. They have demonstrated that there is reasonable increase in the bladder capacity after epispadias reconstruction. A well-developed bladder is a prerequisite for satisfactory urinary control, and early bladder filling and emptying (cycling) may promote bladder growth and storage function. Therefore, if surgery is performed within the first few months of life, the child may have a better chance of having a normal bladder. Until recently epispadias reconstruction was delayed until one year of age, however, some surgeons are advocating very early repair in an effort to achieve better bladder development.



Reviewed: January 2011

Last updated: March 2013

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Epispadias Glossary
  • abdomen: Also referred to as the belly. It is the part of the body that contains all of the internal structures between the chest and the pelvis.

  • abnormality: A variation from a normal structure or function of the body.

  • anatomy: The physical structure of an internal structure of an organism or any of its parts.

  • antibiotic: Drug that kills bacteria or prevents them from multiplying.

  • bladder: The bladder is a thick muscular balloon-shaped pouch in which urine is stored before being discharged through the urethra.

  • bladder capacity: The amount (maximum volume) of urine that the bladder can hold. Often referred to as bladder volume.

  • bladder exstrophy: A congenital birth defect that is the malformation of the bladder and urethra, in which the bladder is turned "inside out" and exposed on the outside of the abdomen. Because the bladder and other structures are exposed to the outside of the body, urine constantly trickles onto the skin causing irritation.

  • bladder neck: Area of thickened muscle fiber where the bladder joins the urethra. Acting on signals from the brain, bladder neck muscles can either tighten to hold urine in the bladder or relax to allow urine out and into the urethra. These muscles also tighten during ejaculation to prevent backflow of semen into the bladder.

  • chordee: Abnormal downward bend of the penis during an erection.

  • clitoris: Sensitive female sex organ, which is visible at the front of the vagina.

  • congenital: Present at birth.

  • continence: The ability to control the timing of urination or a bowel movement.

  • corpora: Plural of corpus. The main portion of something, such as an organ or other body part, or a mass of tissue with a distinct function.

  • corpora cavernosa: Two cylinder-shaped bodies that lie side by side in the penis and that, when filled with blood, enlarge to cause the penis to swell and become erect.

  • corpus spongiosum: A column of erectile tissue in the center of the penis and surrounding the urethra. When filled with blood it enlarges and causes the penis to swell and become erect.

  • diagnosis: The process by which a doctor determines what disease or condition a patient has by studying the patient's symptoms and medical history, and analyzing any tests performed (e.g., blood tets, urine tests, brain scans, etc.).

  • dorsal chordee: An upward bend in the penis when the penis is erect(rigid).

  • ejaculate: The fluid that is expelled from a man's penis during sexual climax (orgasm). To release semen from the penis during an orgasm.

  • ejaculation: Release of semen from the penis during sexual climax (orgasm).

  • epispadias: A rare defect present at birth in which the opening of the urethra is in an abnormal location. In males, the opening is usually on the topside of the penis and not the tip.

  • erectile: Capable of filling with blood under pressure, swelling and becoming stiff.

  • exstrophy-epispadias: A congenital abnormality that affects the bladder, genitals and the pelvis.

  • external genitalia: The region of the body comprising of the penis and scrotum in males and the clitoris, vagina and labia in females.

  • fallopian tubes: There are two fallopian tubes, one on each side of the uterus. They transport an egg from the ovary to the uterus.

  • fertile: Able to produce offspring.

  • fertility: The ability to conceive and have children.

  • foreskin: The loose fold of skin that covers the head of the penis.

  • genitalia: External sexual organs.

  • genitourinary system: The parts of the body that play a role in reproduction and/or getting rid of waste products in the form of urine.

  • glans: The head of the penis.

  • incontinence: Loss of bladder or bowel control; the accidental loss of urine or feces.

  • infection: A condition resulting from the presence of bacteria or other microorganisms.

  • infertile: Physically incapable of conceiving children.

  • infertility: The diminished ability or the inability to conceive and have offspring.

  • intestine: The part of the digestive system between the stomach and the anus that digests and absorbs food and water.

  • ions: Electrically charged atoms.

  • meatus: The opening of the urethra.

  • ovaries: Female reproductive organs that produce eggs and also produce the sex hormones estrogen and progesterone.

  • pelvic: Relating to, involving or located in or near the pelvis.

  • pelvis: The bowl-shaped bone that supports the spine and holds up the digestive, urinary and reproductive organs. The legs connect to the body at the pelvis.

  • penis: The male organ used for urination and sex.

  • penopubic: Describing something located at the junction of the penis and the pubic.

  • pregnancy: The condition of being pregnant.

  • pubic bone: Also referred to as the pubis. Lower front of the hip bone.

  • pus: The yellowish or greenish fluid that forms at sites of infection.

  • radiographic: X-ray.

  • reflux: Backward flow of urine. Also referred to as vesicoureteral reflux (VUR). An abnormal condition in which urine backs up from the bladder into the ureters and occasionally into the kidneys, raising the risk of infection.

  • reflux: Backward flow.

  • retrograde: Backwards.

  • retrograde ejaculation: Cuased by the failure of the bladder neck to close during ejaculation allowing the ejaculate to be propelled into the bladder instead of out the penis.

  • retrograde ejaculation: Caused by the failure of the bladder neck to close during ejaculation allowing the ejaculate to be propelled into the bladder instead of out the penis.

  • shaft: Cylindrical part of the penis.

  • sperm: Also referred to as spermatozoa. Male germ cells (gametes or reproductive cells) that are produced by the testicles and that are capable of fertilizing the female partner's eggs. Cells resemble tadpoles if seen by the naked eye.

  • stent: With regard to treating ureteral stones, a tube inserted through the urethra and bladder and into the ureter. Stents are used to aid treatment in various ways, such as preventing stone fragments from blocking the flow of urine.

  • testicle: Also known as testis. Either of the paired, egg-shaped glands contained in a pouch (scrotum) below the penis. They produce sperm and the male hormone testosterone.

  • ureter: One of two tubes that carry urine from the kidneys to the bladder.

  • ureteral: Pertaining to the ureter. Also referred to as ureteric.

  • urethra: A tube that carries urine from the bladder to the outside of the body. In males, the urethra serves as the channel through which semen is ejaculated and it extends from the bladder to the tip of the penis. In females, the urethra is much shorter than in males.

  • urethral: Relating to the urethra, the tube tha carries urine from the bladder to outside the body.

  • urge: Strong desire to urinate.

  • urinary: Relating to urine.

  • urinary continence: Ability to control urination.

  • urinary tract: The system that takes wastes from the blood and carries them out of the body in the form of urine. Passageway from the kidneys to the ureters, bladder and urethra.

  • urinary tract infection: Also referred to as UTI. An illness caused by harmful bacteria, viruses or yeast growing in the urinary tract.

  • urination: The passing of urine.

  • urine: Liquid waste product filtered from the blood by the kidneys, stored in the bladder and expelled from the body through the urethra by the act of urinating (voiding). About 96 percent of which is water and the rest waste products.

  • urologist: A doctor who specializes in diseases of the male and female urinary systems and the male reproductive system. Click here to learn more about urologists. (Download the free Acrobat reader.)

  • urology: Branch of medicine concerned with the urinary tract in males and females and with the genital tract and reproductive system of males.

  • uterus: A hallow, muscular organ in the pelvis cavity of females in which the embryo is nourished and develops before birth.

  • UTI: Also referred to as urinary tract infection. An illness caused by harmful bacteria growing in the urinary tract.

  • vagina: The tube in a woman's body that runs beside the urethra and connects the uterus (womb)to the outside of the body. Sometimes called the birth canal. Sexual intercourse, the outflow of blood during menstruation and the birth of a baby all take place through the vagina.

  • vesicoureteral reflux: Also referred to as VUR. An abnormal condition in which urine backs up from the bladder into the ureters and occasionally into the kidneys, raising the risk of infection.

Epispadias Anatomical Drawings

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