Hypospadius

 

Condition Description

Hypospadius (pronounced HY-po-SPAY-dee-us) is a common birth defect where a baby boy's urethra does not extend to the tip of the penis and instead opens on the underside of the penis or even below the penis (in the area called the perineum).

During fetal life between the 6 th and 14th week, the embryonic cells do not come together to fuse and close the urethral grove to form a tube for urine passage to the surface from the bladder. The opening can be anywhere along the underside but 85% of the time the opening is near the tip of the penis (in the glans or corona of the penis).

The cause of hypospadius is unknown but it is slightly more common in some families. There is a possible relationship between the mother being exposed to estrogens or endocrine-disrupting chemicals such as PCBs.

Prevalence

This is a common condition, occurring in 1 of every 300 baby boys. Rarely, (1 of 500,000) girls will have a misplaced urethra, typically opening into the vagina. Because it is so rare in girls, the term hypospadius generally refers to the condition in boys.

Common Associated Conditions

Hypospadius does not affect a boy's fertility but it can have a negative effect on sexual function. It also causes urine to spray downward so boys may have to urinate sitting on the toilet. Most boys will also have a condition called chordee where there is a fibrous band of tissue along the length of the penis that pulls it downward, especially apparent during erection. Some boys are also have undescended testicles, inguinal hernias or hydroceles.

Short-term Treatment and Outcomes

Affected boys should not be circumcised because the foreskin will be used in the surgical repair. It is important the surgeon be very experienced in hypospadius repair because there can be complications and each boy's penis and hypospadius is different. The surgery is typically performed between 6 and 18 months of age. The surgical correction of the chordee and the urethra is generally very successful, resulting in good function and appearance. If the opening is on the shaft of the penis multiple surgeries might be necessary. Typically, however, hypospadius repair is a one-stage surgery that is highly successful.

Long-term Treatment and Outcomes

Surgical repair is necessary for sexual function (normal erection and proper sperm deposition in intercourse). It is also necessary for voiding while standing. However it is generally not considered a threat to health but rather as a hindrance to reproduction that is best corrected during early childhood.

Common Complications

If not corrected the boy could be more prone to urinary tract infections. Some hypospadius corrections are more complex if the penis is short, the chordee is severe and there is not much foreskin tissue to use in the repair. In those situations multiple surgeries may be needed. In about 10% of boys, a fistula forms because of breakdown of the delicate tissue of the newly created passage for the urine flow. Repeated surgery may be necessary to repair the fistula and redirect the urine flow to the new opening (meatus). A second complication is scarring of the opening (meatus) causing a narrowing (stricture). A very quick surgical procedure will be necessary to open the scarred meatus.

Implications for Children's Development

The trauma of having surgery as a very young child can be difficult for the child and family. A child's fears following surgery may persist because of his memory of the discomfort. Pain control post-operatively is an important consideration as well as having the parent present for the surgical induction and during the hospitalization.

Beyond the immediate recovery period there should be no ill effects on the boy's development if the repair is successful. However if the penis remains unusual in appearance the boy may be self-conscious which could affect his self-image and self-confidence. Even as an adult, surgery might be helpful in improving the appearance of the penis and improving sexual function.

Children should be taught simple information about their condition and treatment, using terms that they can understand. Books and dolls can help children understand how their bodies work. They should be encouraged to ask questions about their bodies and the treatments and tests. Children do not need a great deal of detailed information but rather they require reassurance and explanations that are tailored to their age and understanding.

They need to be helped to understand that blood tests and uncomfortable procedures are necessary to help them feel better, even though they experience temporary pain or discomfort. Play therapy can assist young children to express their fears and feelings and can reveal misunderstandings they may have about their condition or treatment. Reliable information and support resources are available online.