Types of congenital defects of the urethra
The urethra may be
partially blocked
in the wrong position
protruding out of its opening (prolapsed urethra)
Duplex (two or more urethra instead of just one)
Partial obstruction of the urethra
Many birth defects cause partial obstruction of the urethra.
For posterior urethral valves, abnormal tissue folds in the urethra prevent urine from flowing from the bladder, and posterior urethral valves occur only in boys. The blockage increases pressure in the bladder and can cause difficulty passing urine and poor urine flow. In more severe forms, the obstruction affects the developing fetus. Increased urine pressure due to the obstruction can affect the development of the bladder and kidneys, and the obstruction may also reduce the amount of urine the fetus releases into the amniotic fluid (the fluid that surrounds the fetus in the womb). If the fetus does not release enough urine in the amniotic fluid, the amount of amniotic fluid decreases, and if there is too little amniotic fluid, problems occur in the development of the fetus’s lungs, heart and limbs, and poor lung development can lead to death before or after birth short. After birth, affected infants have symptoms of poor bladder drainage or poor kidney function.
Urethral stricture is a narrowing of the urethra usually caused by injury but sometimes a birth defect, and is more common in boys.
In meatal urethral stenosis, the external opening of the urethra (meatus) is narrow and blocks the urethra. It occurs most often in boys who have had penile surgery or were circumcised shortly after birth.
The urethra is in the wrong position
The urethral opening may be in the wrong place.
In boys, the urethral opening may be on the lower side of the penis (called the hypospadias) or on the upper side of the penis (called the epispadias), rather than at its apex. Boys with hypospadias often have another defect called chordee and hooded foreskin (in which the foreskin is not fused on the underside of the penis). Children with epispadias may have involuntary passing of urine (enuresis).
In girls, the urethral opening may be misplaced between the clitoris and the labia, inside the vaginal opening, or, rarely, on the abdomen.
urethral prolapse
Urethral prolapse occurs in girls, where the inner lining of the urethra protrudes through the opening of the urethra. When urethral prolapse occurs, the opening of the urethra looks like a red, swollen donut. Urethral prolapse usually does not cause symptoms, but the prolapsed tissue may bleed and cause bloody spots on a girl''s diaper or underwear.
Duplication or duplication of the urethra (accessory urethra)
In rare cases, babies are born with two or more urethra, usually only one of which is attached to the bladder, but sometimes they are connected to each other.
the diagnosis
Clinical examination
Sometimes voiding cystourethrogram
Before birth, doctors sometimes discover defects of the urethra that cause significant urinary obstruction during a routine prenatal ultrasound.
After delivery, doctors often find defects of the urethra during a physical exam or exam of a healthy baby. If doctors suspect that a boy has posterior urethral valves, they do a test called voiding cystourethrogram (VCUG) before the boy is discharged from the hospital. This procedure involves passing a catheter through the urethra into the bladder, an X-ray contrast agent is placed through the catheter, and doctors X-ray before and after the child urinates.
processing
Usually surgical repair
Defects of the urethra that cause symptoms, such as obstruction or urinary reflux, usually need to be corrected surgically.
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 with posterior urethral valves undergo surgery when diagnosed. Surgery is used to relieve blockages and prevent further damage to the kidneys. Even after surgery, the bladder may not function normally and boys may need additional catheterization or surgery. A catheter is used to drain the contents of the bladder by ing a thin, flexible, sterile tube (catheter) through the opening of the urethra into the bladder.
Boys who have hypospadias have surgery to repair the defect and to correct any other existing defects, such as curvature of the penis.
Girls who have urethral prolapse may be given a cream that contains estrogen to reduce symptoms. Urethral prolapse often goes away with time and rarely requires surgery.
