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Epispadias
Congenital defect - epispadias
Epispadias is a rare defect that is present at birth The urethra is the tube that carries urine out of the body from the bladder.
In this condition, the urethra does not develop into a full tube. The urine exits the body from the wrong place.
Causes
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.
Symptoms
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 clitorus 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
Signs include:
- Abnormal opening from the bladder neck to the area above the normal urethra opening
-
Backward flow of urine into the kidney (
reflux nephropathy
,
hydronephrosis
)
Reflux nephropathy
Reflux nephropathy is a condition in which the kidneys are damaged by the backward flow of urine into the kidney.
Read Article Now Book Mark ArticleHydronephrosis
Hydronephrosis is swelling of one kidney due to a backup of urine. This problem may occur in one kidney.
Read Article Now Book Mark Article -
Urinary incontinence
Urinary incontinence
Urinary (or bladder) incontinence happens when you are not able to keep urine from leaking out of your urethra. The urethra is the tube that carries...
Read Article Now Book Mark Article - Urinary tract infections
- Widened pubic bone
Tests may include:
- Blood test
-
Intravenous pyelogram
(IVP), a special x-ray of the kidneys, bladder, and ureters
Intravenous pyelogram
An intravenous pyelogram (IVP) is a special x-ray exam of the kidneys, bladder, and ureters (the tubes that carry urine from the kidneys to the bladd...
Read Article Now Book Mark Article - MRI and CT scans, depending on the condition
- Pelvic x-ray
- Ultrasound of the urinary system and genitals
Treatment
Patients 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.
Outlook (Prognosis)
Surgery can help the person control the flow of urine. It will also fix the appearance of the genitals.
Possible Complications
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.
References
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 RI. Exstrophy-epispadias complex. In: Wein AJ, Kavoussi LR, Partin AW, Peters CA, eds. Campbell-Walsh Urology . 11th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 139.
Review Date: 10/28/2015
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, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
