Hypospadias is a common birth defect in which the opening of the tube that carries urine from the body (urethra) develops abnormally, usually on the underside of the penis. This occurs in about 8/1000 male births. The incidence of hypospadias in the United States is increasing as well as is the percentage of more severe cases.
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Causes & Risk Factors
There are many potential causes of hypospadias. Theories include abnormal androgen (hormone) production by the fetal testicles, genetic factors and environmental factors. A familial link is found in 4% to 10% of hypospadias cases.
Symptoms & Types
Hypospadias results from abnormal development of the penis that leaves the urethral meatus (urine hole) closer to the scrotum than its its normal position on the head of the penis. There are varying degrees of severity of the hypospadias defect. In all cases a circumcision is not advised, as the foreskin is required for the hypospadias repair.
- Anterior hypospadias (50% of cases): This occurs when the urine hole is located on the head or just below the coronal rim of the penis. These are less severe defects.
- Middle hypospadias (30% of cases): This occurs when the urine hole is located along the shaft of the penis.
- Posterior hypospadias (20% of cases): This occurs when the urine hole is close to the scrotum. These cases represent a more severe defect.
Diagnosis & Tests
Diagnosis is made by physical exam. Most hypospadias cases involve 3 elements:
- an abnormally located urethral meatus;
- an incompletely formed and dorsally hooded prepuce (more foreskin on the top of the penis than the bottom); and
- a ventral chordee (downward curvature of the penis).
There are varying degrees of severity of each of these defects. Associated anomalies of undescended testicles and/or an inguinal hernia occur in about 9% of cases.
Treatment & Care
There is no medical management for hypospadias. It must be treated with surgery. In occasional cases where it is determined that the shorter penile length could complicate adequate surgical repair of hypospadias, pre-surgical testosterone injections can be considered.
The goal of the surgery is both functional -- a straight penis with urine hole (urethral meatus) at the end of the penis -- and cosmetic. There are many different types of hypospadias surgeries. More severe (proximal) cases sometimes require a 2 stage approach with each stage 6 months apart. With all boys it is advised to perform hypospadias repair between 6-18 months of age.
Living & Managing
After surgery, your child will usually be discharged home the same day of surgery. He will be prescribed pain medicine for his discomfort, but acetaminophen or ibuprofen is also helpful. Most boys are sent home with a urinary catheter in the penis for a couple of days. He will be prescribed a medication to prevent bladder spasms during this time as well as an antibiotic to prevent infection.
The most common complication of surgery is an urethrocutaneous fistula, or an opening between the urethra and penile skin. This occurs in about 5% of cases and is most often recognized by the parent seeing 2 separate urinary streams.