Epispadias

Epispadias occurs when the urethra (the tube through which a child urinates) does not close normally, and the inner lining of the urethra is exposed on the top surface of the penis in boys and between the clitoris in girls.

All children born with another birth anomaly called bladder exstrophy also have epispadias. In some cases, epispadias may be present on its own. In isolated epispadias, the bladder is closed and covered by the lower abdominal wall muscles and skin. Some children with epispadias may have bony-pelvis abnormalities that are similar to those seen in bladder exstrophy but less severe. Isolated epispadias is thought to be a mild form of exstrophy-epispadias complex, but many children with epispadias can have issues related to urinary incontinence, even after the exposed urethra is surgically repaired.

Epispadias is more common in boys than in girls. When it occurs in boys, the urethral opening can be located at any point along the top of the penis. Boys may have an upward curve of the penis. All girls with epispadias have a separated clitoris.

The exact cause of epispadias is unknown. Popular theories suggest that a structure known as the cloacal membrane might not develop normally. This may prevent appropriate tissue development, ingrowth, and the joining together of the urethra in the midline.

Epispadias is typically diagnosed immediately after birth during the initial physical examination. However, especially in females with epispadias, the diagnosis may not be made until challenges related to toilet training prompts a thorough genitourinary physical exam.

The primary goal of treatment for epispadias is to:

• Develop adequate bladder function and promote urinary continence
• Provide acceptable appearance and function of the external genitalia
• Ensure that your child has a typical and normal childhood

Epispadias repair typically occurs when a child is 6 to 12 months old. The type of surgical repair depends on the severity of the anomaly. If epispadias is mild, surgery may only involve the urethra and external genitalia. If epispadias is more severe, surgery may also involve the bladder neck and pelvic bones.

At Boston Children’s, we also evaluate many older children who have persistent urinary incontinence. We provide individualized care based on patients’ unique anatomies and individual needs. Many patients experience improvements in incontinence over time and with medications and physical therapy. In select patients, additional surgeries, such as bladder neck reconstruction, may be required to help achieve continence.

The experts in our Bladder Exstrophy Program provide exceptional pediatric care to children with epispadias and their families. Our team includes physician specialists in urology, nephrology, orthopedic surgery, anesthesiology, radiology, and nuclear medicine, as well as nurses, social workers, and child life specialists who will care and support you and your child at every step, from diagnosis through treatment, and even after your child returns home.

We offer a variety of resources for families, including access to the Fetal Care and Surgery Center (FCSC). The FCSC provides support through prenatal counseling, treatment, and follow-up for families with a confirmed or suspected fetal congenital anomaly. In addition, medical staff, social workers, child life specialists, and spiritual care services are available to support expectant parents.