Posterior Urethral Valves

What are posterior urethral valves?

A posterior urethral valve (PUV) is a birth defect (congenital disorder) where tissue blocks the tube that pee (urine) travels through to leave your body (urethra). This means your bladder can’t empty properly, and pee can back up and cause damage to your bladder and kidneys.

PUV is a congenital condition, meaning you’re born with it. A healthcare provider might diagnose PUV during pregnancy, shortly after birth or during childhood. It only affects males. It’s also called congenital obstructive posterior urethral membrane (COPUM).

Are posterior urethral valves rare?

About 500 babies born each year have posterior urethral valves. It’s the most common cause of urinary tract blockages in boys.

What are the symptoms of posterior urethral valves?

Symptoms of posterior urethral valves can include:

• Weak or dribbling stream of pee
• Urinary tract infections (UTIs)
• Poor weight gain in infants
• Painful urination (dysuria)
• Difficulty peeing
• Low amniotic fluid (oligohydramnios)
• Kidney dilation (hydronephrosis), seen on prenatal ultrasound

What causes posterior urethral valves?

Extra tissue in your urethra near your bladder causes posterior urethral valves. It narrows your urethra so pee doesn’t flow through as easily as it should. This happens early in the development of a fetus. But providers aren’t sure what causes the extra tissue to form.

What are the complications of posterior urethral valves?

PUV can lead to:

• Pee backing up towards your kidneys (vesicoureteral reflux/VUR)
• Inability to empty your bladder completely (urinary retention)
• Kidney swelling
• Kidney failure

How are posterior urethral valves diagnosed?

Sometimes, a provider diagnoses posterior urethral valves with an ultrasound during pregnancy. At or after birth, a provider might use one or more of these tests to diagnose PUV:

• Kidney ultrasound. An ultrasound can help your provider see any blockages or differences in how your child’s kidneys, bladder and urinary tract look.
• Voiding cystourethrogram. This lets your provider see how well your child’s bladder fills and empties. This will also show the urethra and any areas of narrowing.
• Cystoscopy. Your provider uses a small, flexible tube with a light and a camera at the end to look inside your child’s urinary tract.
• Kidney function tests. Blood or urine (pee) tests can help to determine how well your child’s kidneys are working.

How are posterior urethral valves treated?

Treatment for posterior urethral valves is usually a procedure to remove or destroy the blockage. A provider often does this with fulguration — using electricity to destroy the tissue with heat.

Depending on any other issues, a provider may also treat your child with:

• Catheterization to empty their bladder
• Urethral dilation to widen their urethra
• Vesicostomy to treat VUR
• Antibiotics to treat infections
• Dialysis or kidney transplant for kidney failure
• Bladder relaxants (antispasmodics) to make peeing easier

In some rare cases, a specialist may perform surgery on the fetus while you’re still pregnant.

What’s the prognosis (outlook) for posterior urethral valves?

If your child has posterior urethral valves, they’ll probably need blood tests and ultrasounds to monitor their kidney function throughout their lifetime. They may have issues with leaking pee (urinary incontinence) either as a child or an adult. Studies suggest that about 15% of kids with posterior urethral valves progress to kidney failure.

When should I see a healthcare provider?

Contact a healthcare provider right away if your child has symptoms of posterior urethral valves. Left untreated, the condition can cause severe health issues.

What questions should I ask my doctor?

It might be helpful to ask a healthcare provider:

• What are the treatment options?
• How does this treatment work?
• What can I expect during recovery?
• How do I care for my child during recovery?
• When should we follow up with you?