Topic Resources
The urethra is the tube that drains urine from the bladder out of the body.
A Look Inside the Urinary Tract
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Types of Birth Defects of the Urethra
There are several types of birth defects of the urethra. The urethra may be
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Partially blocked
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In the wrong place
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Bulging out from its opening (prolapsed urethra)
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Duplicated (two or more urethras instead of just one)
These defects may
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Cause urine to come out from the wrong location
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Block the flow of urine
Any urethra defect that blocks or slows the flow of urine can cause urine to become stagnant, which can result in urinary tract infections
(UTIs). Blockage of urine flow also can raise the pressure inside the
bladder and/or kidneys and damage them over time. Frequent infections
also can damage the kidneys. Kidney damage can cause high blood pressure and rarely kidney failure.
Partially blocked urethra
Several birth defects partially block the urethra.
In posterior urethral valves, folds of abnormal tissue in
the urethra block the flow of urine from the bladder. Posterior urethral
valves occur only in boys. The blockage increases the pressure in the
bladder and can cause difficulty urinating and a weak urine stream. In
more severe forms, the blockage affects the developing fetus. The
increased urine pressure from the blockage can interfere with
development of the bladder and kidneys. The blockage also may reduce the
amount of urine the fetus releases into the amniotic fluid (the fluid
that surrounds the fetus in the uterus). If the fetus does not release
enough urine into the amniotic fluid, the amount of amniotic fluid is
reduced. If there is too little amniotic fluid, problems occur with the
development of the fetus's lungs, heart, and limbs.
Poor lung development can lead to death shortly before or after birth.
After birth, affected infants have symptoms of poor bladder drainage or
poor kidney function.
A urethral stricture is a narrowing of the urethra that is usually caused by an injury but sometimes is a birth defect. It is more common in boys.
In urethral meatal stenosis, the outside opening of the
urethra (meatus) is narrow and decreases and misdirects the flow of
urine. This occurs mostly in boys who previously had surgery on their
penis or were circumcised as newborns.
Urethra in the wrong place
The opening of the urethra may be in the wrong place.
In boys, the opening of the urethra may be on the underside of the penis (called hypospadias) or on the topside of the penis (called epispadias), instead of at the tip. Boys who have hypospadias often have another defect called chordee
(a downward bend of the penis) and a hooded foreskin (where the
foreskin has not grown together on the underside of the penis). Children
who have epispadias may have involuntary release of urine (urinary incontinence).
In girls, the opening of the urethra may be misplaced
between the clitoris and the labia, inside the opening of the vagina, or
rarely on the abdomen.
Urethral prolapse
Urethral prolapse occurs in girls. In this disorder, the inner
lining of the urethra sticks out through the opening of the urethra.
When urethral prolapse occurs, the opening of the urethra looks like a
small red and swollen donut. Urethral prolapse typically does not cause
symptoms, but the prolapsed tissue may bleed, causing blood spots on the
girl's diaper or underwear. This disorder is common among blacks.
Duplicated urethras (extra urethras)
Rarely, children are born with two or more urethras. Usually only
one of them is connected to the bladder, but sometimes they are
connected to each other.
Diagnosis
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Physical examination
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Sometimes voiding cystourethrography
Before birth, doctors sometimes discover defects of the urethra that cause significant urinary blockage during routine prenatal ultrasonography.
After birth, doctors often find defects of the urethra during a physical examination or a well-child examination. If doctors suspect a boy has posterior urethral valves, they do a test called voiding cystourethrography
(VCUG) before the boy is discharged from the hospital. For voiding
cystourethrography, a catheter is passed through the urethra into the
bladder, a liquid that shows up on x-rays (contrast agent) is put in through the catheter, and x-rays are taken before and after the child urinates.
Treatment
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Usually surgical repair
Defects of the urethra that cause symptoms, such as blockage or urinary reflux, usually need to be surgically corrected.
Children who have a blockage in the urethra have surgery to open
the blockage as soon as possible. Children whose urethra is abnormal,
narrow, or missing may need surgery to correct these defects.
Boys who have posterior urethral valves have surgery when they
are diagnosed. Surgery is done to relieve the blockage and prevent
further kidney damage. Even after surgery, the bladder may not function
normally and boys may need catheterization or additional surgery.
Catheterization is draining the bladder by inserting a thin, flexible
tube (catheter) through the urethral opening into the bladder.
Boys who have hypospadias may have surgery to repair the defect
and correct any other existing defects such as chordee depending on the
degree of severity.
Girls who have urethral prolapse may be given a cream that
contains estrogen to lessen symptoms. Urethral prolapse usually goes
away with time and rarely requires surgery.
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