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Urethra Defects

By Ronald Rabinowitz, MD, Professor of Urology and Pediatrics, University of Rochester Medical Center ; Jimena Cubillos, MD, Associate Professor of Clinical Urology and Pediatrics, University of Rochester School of Medicine and Dentistry

The urethra is the tube that drains urine from the bladder out of the body.

A Look Inside the Urinary Tract

Complications of defects of the urethra

There are several types of birth defects of the urethra. These defects may

  • Cause urine to come out from the wrong location

  • 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.

Types of Birth Defects of the Urethra

The urethra may be

  • Partially blocked

  • In the wrong place

  • Bulging out from its opening (prolapsed urethra)

  • Duplicated (two or more urethras instead of just one)

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 blocks 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 is not fused 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. 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 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.

Duplicated (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

  • Physical examination

  • 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 dye 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

  • 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, sterile tube (catheter) through the urethral opening into the bladder.

Boys who have hypospadias have surgery to repair the defect and correct any other existing defects such as chordee.

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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