Overview

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Hypospadias is a condition that is present at birth (congenital) in which the opening of the
tube that carries urine and, in males, semen from the body (the urethra) 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 bulbous end of the penis
(the glans penis). This opening may be located on the glans, along the shaft of the penis, at the
pouch that contains the testicles (scrotum), or in the area between the scrotum and the anus
(perineum). The farther the opening is from the tip of the glans, the more likely curvature in
the penis (chordee) is present.

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

Common complications of severe hypospadias include undescended testicles and inguinal

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

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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 removal of the foreskin
(circumcision) is performed. Diagnosis of hypospadias in females requires 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 pinching (plicating) 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 an abnormal opening from the urethra to the skin (fistula)
• Narrowing of the urethral opening (meatal stenosis) or the urethra (stricture)
• Formation of ballooning of the urethra (urethral diverticula)

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 before birth (antenatal)
intervention that may improve the prognosis are being developed.

Prevention

Hypospadias cannot be prevented.

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