Epispadias

Epispadias is a rare congenital urological defect that affects the urinary tract and alters the way urine exits the body. It is more common in boys than girls, about one in 100,000 males and about one in 480,000 females.

The urethra is a urinary tube that carries urine from the bladder out of the body. In boys with epispadias, the urethral opening is abnormally located on top of the penis rather than the tip, creating an open groove-like appearance. In girls, the urethra is located near the clitoris or the lower abdominal area.

Male Epispadias

In males, the urethral opening is located on the top of the penis, resulting in a groove-like appearance. Epispadias may also be associated with other urological or developmental problems, collectively called the exstrophy-epispadias complex.

About 10% of epispadias cases occur without additional problems. However, in 90% of cases, it is associated with:

• Visible urinary bladder through the abdominal wall
• Genital and urinary tract malformations
• Small genitalia and pelvic floor abnormalities
• Displaced anus and inguinal hernia

Boys with epispadias have a broad, short, and upward-curved penis. The pelvic bones are widely separated, pulling the penis towards the body. Based on the meatus location, it is classified as:

• Glanular Epispadias: Meatus is on the head but on top of the penis.
• Penile Epispadias: Opening lies along the shaft above the base.
• Penopubic Epispadias: Opening is near the pubic bone at the base of the penis.

In females, the urethra splits the clitoris abnormally, leading to urinary incontinence. It may be associated with short vaginal length, exposed bladder or urethra, and other urinary abnormalities.

It occurs due to abnormal urinary tract development during a specific stage of pregnancy. Most cases have no known maternal or prenatal cause.

It is often diagnosed at birth due to abnormal genital appearance. In mild cases, symptoms may be missed until toilet training. Symptoms include:

• Urine leakage or incontinence
• Stress incontinence (e.g., during coughing or sneezing)
• Urgency incontinence (difficulty holding urine)

Diagnosis is primarily by genital examination at birth. Additional tests may include:

• Kidney function tests
• Urine tests and imaging (ultrasound, CT, X-ray)
• Urological exams to check urinary tract anatomy
• Spinal cord or colon assessments if needed

• Urinary incontinence is common
• Chordee: Curved penis in boys
• Potential infertility in penopubic cases
• Possible issues with sperm quality
• Intercourse difficulty due to curvature or short penis
• Females generally retain fertility

Treatment involves surgical repair to restore appearance and functionality.

Before Surgery

Tests are done to determine if the defect affects other organs. Surgery may be staged depending on severity and associated complications.

Surgeries to Treat Male Epispadias

Two main surgical techniques are:

• Mitchell Technique
• Cantwell Technique

Surgery involves disassembling the penis, reconstructing the urethra (urethroplasty), correcting curvature, and reassembling the penis. A catheter is usually placed post-surgery.

• Restore penile function and appearance
• Preserve fertility and urinary control

Surgeries to Treat Female Epispadias

Surgery is simpler in girls. Reconstructive surgery may bring the clitoris together and reposition the urethra. Internal organs are usually unaffected.

Recovery

Most children recover in 1-2 days post-surgery. Complex cases may require longer stays.

Life After Surgery

Surgery helps achieve urinary continence and normal urinary function. Multiple procedures may be required depending on severity.

Sexual Function After Surgery

Most patients retain normal sexual function. Rare cases may experience low sperm count or volume, but fertility is generally preserved.

Further Treatment

Around 1 in 3 children gain urinary control after one surgery. Others may need bladder neck reconstruction or injection of bulking agents for urine control.

Q: Are children with epispadias more likely to get urinary tract infections?
A: No, epispadias alone does not increase UTI risk, but children with exstrophy-epispadias complex are at higher risk.

Q: Can epispadias be prevented?
A: There is currently no known method to prevent epispadias.

Q: What is the best time for surgical treatment?
A: Early surgery is preferred to enhance bladder development and urinary control.

Q: What is the difference between Epispadias and Hypospadias?
A: Both are birth defects. In epispadias, the urethral opening is on top of the penis; in hypospadias, it is on the underside.

Q: Can circumcision be done in children with epispadias?
A: Circumcision should be avoided until surgery, as foreskin may be needed for reconstructive procedures.