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Epispadias

Pediatric – Epispadias

Normally, boys and girls are born with fully functioning genitals. A congenital condition called Epispadias can affect the functioning of the penis or vagina in small children. This affects the functioning of the genitalia and gives it an abnormal appearance.

Fortunately, with the advances in pediatric medical technology, urologists are able to correct the abnormally placed urethral opening, hooded foreskin and the curvature which develops due to this condition.

What is Epispadias?

During the pregnancy, the development of the fetus’ organs is an intricate and timely process. Epispadias causes the vaginal and penile structures to develop in an abnormal manner. This condition affects the normal formation of the genitalia in varying degrees.

In boys, the condition may result in the formation of a small dimple on the penile opening and may result in the formation of an additional clitoris in girls. When this condition is extensive, it can result in exposing the bladder and urethra. Collectively, these conditions are termed as ‘exstrophy-epispadias complex’. In rare cases, Epispadias is seen as an isolated condition.

How does Epispadias affect boys?

Epispadias is a fairly rare condition and is seen in 1 out of around 117,000 boys. In such cases, the penis is shaped broader than normal, shortened and curved upward (dorsal chordee). The penis may be attached to the pelvic bone in some cases. This causes the pelvic bone to form widely separated, causing the penis to be pulled towards the body.

In normal situations, the meatus is located at the tip of the penis. Boys with Epispadias have the meatus located on top of the penis. The penile shaft is split from the base to the tip on the upper side, forming a gutter.

The location of the meatus helps to determine the degree of Epispadias. The meatus may be located on the glans (glanular), along the shaft (penile) or near the pubic bone (penopubic). The position of the meatus determines the amount of urine which the bladder can store (continence). The closer the meatus is located to the upper part of the base of the penis, the lesser amount of urine will be stored in the bladder.

Most cases of penopubic Epispadias, the pelvic bones are widely separated. This results in the bladder’s neck to be affected. It causes the bladder’s neck to remain open which does not allow the storage of urine, resulting in a constant dribbling. Stress incontinence (from coughing and strenuous efforts) affects most boys with penopubic Epispadias and around 2/3rd of those affected by penile Epispadias.

Eventually, this requires reconstructive surgical procedure of the neck of the bladder. Almost all cases of glanular Epispadias have a fully functional bladder’s neck and do not affect the urinary continence. However, in case the penis is bent upward with an abnormal opening it may require surgical correction.

How does Epispadias affect girls?

Epispadias is comparatively rare in girls and affects 1 in about 565,000 children. Girl child affected by this condition have widely separated pelvic bones in varying degrees. This results in the clitoris to form abnormally and causes it to split into two sections. Also, the bladder’s neck is almost always affected by this disorder. As a result, girls with Epispadias are affected with stress incontinence.

In most cases, early surgical repair can help solve this problem efficiently.

How is Epispadias diagnosed?

Diagnosis of Epispadias is normally done at the time of birth. Occasionally, when the malformation is not significantly visible the condition may become noticeable when the girl child starts toilet training (constant moistness).

What are the risks associated with Epispadias?

There is no proof to show that Epispadias can result in UTI (urinary tract infection). However, in case the child is suffering from exstrophy-epispadias there is an increased risk of UTI as this condition results in vesicoureteral reflux (VUR).

The standard treatment for such cases involves regular antibiotics till the reflux is corrected.

Babies affected by Epispadias are normally healthy and full of vigor. Pelvic bones, genitourinary system and other organs are not affected. Hence, such children do not require extensive diagnostic radiographic tests. However, in case the baby is affected with a severe form of exstrophy-epispadias complex there is an increased risk of developmental malformation, including vesicoureteral reflux, horseshoe kidney and enlarged ureter.

Boys with penopubic Epispadias or extreme form of exstrophy-epispadias complex may face infertility problems due to malformed genital anatomy. Normally, young men have a bladder neck which is closed when the sperm is transferred from the testicles to the urethra and during ejaculation. When men are affected with penopubic Epispadias or extreme form of exstrophy-epispadias complex the bladder’s neck does not close properly during ejaculation and causes the sperm to flow backwards. This causes problems while trying to conceive. Such men also have lower sperm quality.

Today, surgical repairs are available that effectively correct this condition for men. In women with Epispadias, there is usually no risk of risk of infertility as the genitals are not affected.

What are the treatments for Epispadias?

The main aims of the treatment of Epispadias are to:

• Maximize penile length by treating curvature or the penis

• Create functioning and normal-looking genitalia with least number of surgical procedures

In case the bladder and the bladder’s neck are also affected, surgical treatment helps to restore normal urinary continence and improve fertility.

Surgical treatment for males

There are mainly two types of surgical methods for treating Epispadias in males:

• Cantwell technique (modified) – This involves dissembling the penis partially and restoring the urethra in a normal position.

• Mitchell technique (modern) – This involves dissembling the penis completely into its three components – two corpora cavernosa and single corpus spongiosum. After this, the three components are re-assembled enabling the urethra to function normally. The position of the dorsal chordee is also corrected. This technique has lower risk comparatively and gives better repair of the bladder and the bladder’s neck. If the boy is born with an abnormally small or underdeveloped penis surgical reconstruction of the penis becomes difficult.

Surgical treatment for females

Reconstruction of the genitalia in girls affected by bladder exstrophy is comparatively easier than boys as the urethra and vagina are usually short and near the front of the body with the clitoris sectioned into two parts. The internal genital structure – including uterus, ovaries and fallopian tubes – are also normally formed.

When this condition is diagnosed at birth, surgical repair can help to join the sections of the clitoris and urethra can also be positioned normally. Early repair can help prevent urinary incontinence also. In case the diagnosis is at a later stage in life then the urinary incontinence can be surgical repaired. Reconstruction of the narrowed vaginal opening in younger women or older girls is usually advised after puberty.

Children affected by exstrophy-epispadias complex require surgical treatment for improving urethral resistance. Several different treatment methods can be used for this and restore normal urinary function. Initial treatment may require injecting a bulking material around the bladder’s neck to help close it efficiently and prevent urinary incontinence. Other methods are somewhat more complex as these involve creating a longer urethral tube or wrapping various types of materials around the neck of the bladder.

What is the prognosis after treatment for Epispadias?

After a major Epispadias surgery an abnormal hole in the urethral tube is commonly seen. Today, with the technological advances in medical and surgical science this condition can be easily prevented (lowered to 6%). Epispadias repair does little or no harm to the erectile functioning of the penis.

Frequently asked questions:

Can Epispadias be prevented?

There are no known preventive measures for Epispadias.

What is the best time for surgical treatment of Epispadias?

Most expert surgeons and urologists advise on early repair of Epispadias, especially in boys with penile Epispadias or penopubic Epispadias and all girls with the condition. There is documented increase in regaining normal bladder capacity after Epispadias reconstruction. When surgery is performed at an early stage in the life of the child there are more chances of successful restoration of normal bladder function. Currently, Epispadias surgery is advised within the first 12 months of the child’s life.