What is Epispadias Repair (Female)?

Epispadias (also called bladder exstrophy) is a rare congenital defect in females in which there is a fissure in the upper wall of the urethra.

Effects of Epispadias Repair (Female)

Females with simple hypospadias also have normal lives, including conceiving and bearing children.

Candidates for Epispadias Repair (Female)

Several leading reconstructive surgeons have championed early repair of epispadias, especially in boys with penopubic or penile epispadias and all girls with the condition. If surgery is performed within the first few months of life, the child may have a better chance of having a normal bladder. Today, most patients undergo epispadias reconstruction at 12 months or even earlier.

Your Consultation

A child who is suspected to have epispadias is recommended to undergo tests such as the following:

  • CBC
  • Serum electrolytes
  • Pelvic x-ray
  • Intravenous pyelogram (IVP)
  • Ultrasound of the urogenital system

The Epispadias Repair (Female) Procedure

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.

Recovery

Surgical repairs generally produce both continence (the ability to control the flow of urine) and a good cosmetic outcome.

Risks

Persistent urinary incontinence can occur in some people even after multiple operations. Also upper urinary tract (ureter and kidney) damage as well as infertility may occur.

FAQs

How does epispadias affect girls?

Epispadias is much more rare in girls, with only one of 565,000 affected. Those who are affected have pubic bones that are widely 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 symptoms of epispadias?

The symptoms and signs in females are:

  • Abnormal opening from the bladder neck to the area above the normal urethal opening
  • Bladder exstrophy (may or may not be present)
  • Widened pubic bone
  • Bifid clitoris, rudimentary labia
  • Urinary incontinence
  • Reflux nephropathy
  • Urinary tract infections

How is the treatment approached?

Genital repair is often accomplished at different times for the boy and girl with exstrophy. In females, the genitalia and urethral repair can often be accomplished at the time of the initial bladder closure. In males, reconstruction of the penis and urethra (epispadias repair) is usually accomplished between 1 and 2 years of age. This surgery is also important in that it usually causes some urethral resistance, which in turn results in pooling of urine in the bladder which can stimulate bladder growth and capacity.

Can epispadias be prevented?

There is no known prevention.