EpispadiasCongenital defect - epispadias
Epispadias is a rare defect that is present at birth. In this condition, the urethra does not develop into a full tube. The urethra is the tube that carries urine out of the body from the bladder. The urine exits the body from the wrong place with epispadias.
The causes of epispadias are not known. It may occur because the pubic bone does not develop properly.
Epispadias can occur with a rare birth defect called bladder exstrophy. In this birth defect, the bladder is inside out and sticks through the wall of the abdomen. Epispadias can also occur with other birth defects.
The condition occurs more often in boys than girls. It is most often diagnosed at birth or soon afterward.
Males will have a short, wide penis with an abnormal curve. The urethra most often opens on the top or side of the penis instead of the tip. However, the urethra may be open along the whole length of the penis.
Females have an abnormal clitoris and labia. The urethral opening is often between the clitoris and the labia, but it may be in the belly area. They may have trouble controlling urination (urinary incontinence).
Exams and Tests
- Abnormal opening from the bladder neck to the area above the normal urethra opening
- Backward flow of urine into the kidney (reflux nephropathy, hydronephrosis)
- Urinary incontinence
- Urinary tract infections
- Widened pubic bone
Tests may include:
- Blood test
- Intravenous pyelogram (IVP), a special x-ray of the kidneys, bladder, and ureters
- MRI and CT scans, depending on the condition
- Pelvic x-ray
- Ultrasound of the urinary system and genitals
People who have more than a mild case of epispadias will need surgery.
Leakage of urine (incontinence) can often be repaired at the same time. However, a second surgery may be needed.
Surgery can help the person control the flow of urine. It will also fix the appearance of the genitals.
Some people with this condition may continue to have urinary incontinence, even after surgery.
Ureter and kidney damage and infertility may occur.
When to Contact a Medical Professional
Call your health care provider if you have any questions about the appearance or function of your child's genitals or urinary tract.
Elder JS. Anomalies of the bladder. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 541.
Gearhart JP, Mathews R. Exstrophy-epispadias complex. In: Wein AJ, Kavoussi LR, Partin AW, Peters CA, eds. Campbell-Walsh Urology. 11th ed. Philadelphia, PA: Elsevier; 2016:chap 139.
Review Date: 10/10/2017
Reviewed By: Jennifer Sobol, DO, urologist with the Michigan Institute of Urology, West Bloomfield, MI. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.