Overview

Hypospadias is a congenital (present at birth) condition in which the opening of the urethra (tube that carries urine and, in males, semen from the body) is located below the normal location. This is caused by incomplete development of the urethra in utero between 8 and 20 weeks of gestation.

Various degrees of hypospadias result in an opening located anywhere along the length of the urethra. Degrees of hypospadias are classified according to location, including anterior (50% of cases), middle (20% of cases), and posterior (30% of cases). Hypospadias may also cause a curvature of the penis (chordee). Chordee is sometimes evident only with an erection. Severe chordee may result in the inability to perform sexual intercourse.

Without surgical correction, severe hypospadias may result in the inability to urinate standing and infertility.

Incidence and Prevalence

Hypospadias occurs in approximately 1 in 300 males and 1 in 500,000 females in the United States. Worldwide incidence is rising, partially due to an increase in the number of minor cases being reported. The condition is more common in infants of Jewish and Italian descent.

Risk Factors and Causes

The cause of hypospadias is unknown but may include genetic, endocrinological, and environmental factors. Genetic factors are suggested by an increase in the condition in twins compared to a single birth. Human chorionic gonadotropin (hCG) is a hormone produced in early pregnancy that stimulates the production of estrogen and progesterone. In the case of twins, the production of hCG may not be sufficient to prevent incomplete urethral development. There is also a 20% chance that an infant born with hypospadias has a family member with the condition.

Endocrinological factors include low levels of androgens (e.g., testosterone, androsterone) and the infant’s cells’ inability to use these substances effectively may also result in hypospadias. Androgens are substances that stimulate the development of male characteristics.

Maternal exposure to increased levels of progesterone, common during in vitro fertilization (IVF), increases the risk for hypospadias in the infant.

Environmental exposure to estrogen during urethral development may also be a risk factor. Exposure can result when the mother ingests pesticides on fruits and vegetables and milk from pregnant cows.

Signs and Symptoms

Hypospadias results in a urethral opening located below the tip of the glans penis (bulbous end of the penis). This opening may be located on the glans, along the shaft of the penis, at the scrotum (pouch that contains the testicles), or in the perineum (area between the scrotum and the anus). The farther the opening is from the tip of the glans, the more likely chordee (curvature in the penis) is present.

Mild hypospadias results in a downward spray of the urine stream.

Common complications of severe hypospadias are undescended testicles and inguinal hernias (i.e., located in the groin). Other complications include upper urinary tract anomalies and vesicoureteral reflux (backflow of urine from the ureter to the bladder).

Diagnosis

Hypospadias is sometimes diagnosed by prenatal ultrasound, but it usually occurs in males at birth. Upon examination, the foreskin is usually incomplete and the misplaced urethral opening is located. Mild hypospadias may not be diagnosed unless circumcision (removal of the foreskin) is performed. Diagnosis of hypospadias in females requires more thorough physical examination.

Treatment

Cases of hypospadias in which the urethral opening is near the tip of the glans do not require treatment if the urine stream is straight and there is no chordee. Moderate or severe hypospadias requires surgery to achieve the following:

• Allow the patient to urinate standing
• Correct curvature of the penis (chordee)
• Present a cosmetically acceptable appearance
• Preserve fertility

Surgery is usually performed under general anesthesia when the child is between the ages of 6 and 18 months. Techniques vary considerably from case to case and may involve stages. Before surgery, testosterone injections or creams may be used to increase penis size and antibiotics are administered to lower the risk for infection. A catheter is inserted into the bladder to drain urine.

First, the anatomy is thoroughly assessed. The penile skin is retracted and any curvature is corrected by removing the hardened tissue or plicating (pinching) tissue to offset the curvature. Then, the urethra is extended using local tissue from the foreskin and the opening is repositioned at the tip of the glans. If an adequate amount of tissue is not available, tissue from inside the mouth may be used. Finally, the penile skin is replaced and sutured.

Surgical Complications

Local swelling and minor bleeding are common following surgery to repair hypospadias and usually are not severe. Antibiotics are continued after the procedure and infection is rare. Complications include adverse reactions to anesthesia and excessive bleeding that may require additional surgery.

Long-term complications that may require additional surgery include the following:

• Development of a fistula (abnormal opening from the urethra to the skin)
• Narrowing of the urethral opening (meatal stenosis) or the urethra (stricture)
• Formation of urethral diverticula (ballooning of the urethra)

Prognosis

The prognosis for hypospadias depends on the severity of the condition. Surgical correction for moderate or severe conditions is becoming increasingly successful. Surgical advancements (e.g., tissue glue, laser procedures, urethral substitutes) and antenatal (before birth) intervention that may improve the prognosis are being developed.

Prevention

Hypospadias cannot be prevented.

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