What Is Periventricular Leukomalacia and What Does It Mean for Your Baby?

Periventricular leukomalacia is a type of brain injury that affects the white matter surrounding the ventricles in the brain. There is no cure, but treatment can manage symptoms and help with mobility.

Periventricular leukomalacia (PVL) is a type of brain injury that primarily affects infants born before 37 weeks of gestation.

With rising survival rates of premature infants, more and more children are being diagnosed with PVL each year. In fact, PVL may affect as many as 32% of children born prematurely.

Here’s what you need to know about PVL, what causes it, and what treatments may help.

Learn more about premature birth complications.

What is periventricular leukomalacia?

“Periventricular” means in or around the ventricles in the brain. The word “leuko” means white, and “malacia” means softening.

PVL is a brain injury disorder caused by the softening of white brain tissue surrounding the ventricles (periventricular area of the brain). The tissue softens and then dies off, leaving small holes in the brain and leading to various signs and symptoms.

Both fetuses and newborns may receive a PVL diagnosis. It’s most common in children who were born prematurely.

The developmental window with the highest risk of a periventricular white matter injury is between 23 and 32 weeks of gestation.

Low birth weight is another notable risk factor.

What are the symptoms of periventricular leukomalacia?

Symptoms of PVL may not show up immediately after delivery. Still, children are at risk of developing various issues that may be apparent within the first year.

It’s important to note that the type and severity of symptoms depend on the extent of brain damage and where it occurs.

Signs and symptoms may include:

• developmental delays
• learning disabilities
• hearing impairment
• vision impairment
• dystonia
• seizures or hypsarrhythmia
• muscle spasticity

If PVL is accompanied by excessive bleeding in the periventricular-intraventricular area of the brain, a person may develop a neurological disorder called cerebral palsy .

Cerebral palsy primarily affects a person’s movement and posture. It may lead to symptoms like tight muscles, weakness in one arm or leg, and involuntary movements called tremors .

What causes periventricular leukomalacia?

To understand PVL, it helps to understand brain anatomy .

The ventricular system consists of four ventricles in the brain. These ventricles produce and circulate cerebrospinal fluid (CSF) to cushion and protect the brain. The brain tissue (white matter) surrounding the ventricles allows nerve fibers to send messages from the brain to the muscles throughout the body.

While the exact cause of PVL isn’t fully known, experts believe a lack of oxygen, blood flow, or both to the ventricles at critical times in brain development may cause it. When the ventricles don’t get enough blood or oxygen, the surrounding brain tissue dies, resulting in brain damage.

Situations that may lead to blood flow and oxygen deprivation in the brain include:

• intracranial hemorrhage (bleeding in the brain)
• premature rupture of membranes (water breaking early in pregnancy)
• intrauterine (womb) infection during pregnancy

How is periventricular leukomalacia diagnosed?

At your appointment, your pediatrician will ask about your child’s symptoms, go over their health history, and perform a physical exam .

PVL mainly affects children who were born before 37 weeks of gestation. If your child has symptoms and was born prematurely, your doctor may order further testing.

Doctors can visualize the ventricles and any brain damage using minimally invasive imaging tests:

• MRI: MRI uses a magnetic field and radio waves to capture very detailed images of the brain and brain stem. While this procedure is painless, young children may need sedation to ensure they stay still for the duration of the test.
• Cranial ultrasound: This type of ultrasound uses sound waves to capture images inside the head. It’s particularly useful in children under 12 months old . There’s no need for sedation with ultrasound.

What’s the treatment for periventricular leukomalacia?

There’s no specific treatment for PVL. Instead, treatment is individualized and aimed at addressing a person’s symptoms or specific issues.

Treatment may include physical therapy, occupational therapy , and speech therapy to address developmental or learning delays.

Medications for seizures , assistive devices for mobility like wheelchairs and walkers, and continued vision and hearing care may also help improve a person’s quality of life.

What’s the outlook for people with periventricular leukomalacia?

The outlook for PVL depends on the extent of brain injury.

It’s not a progressive disorder, meaning it will not get worse over time. However, PVL may present challenges that affect a person’s life through adulthood. Your child will likely work with a team of doctors long term to help manage symptoms.

Some people with PVL may have a near typical life expectancy. Others, with complications like cerebral palsy, may have a shortened life expectancy.

That said, a 2019 study found that 22% of people with milder forms of cerebral palsy live to age 58 years.

Frequently asked questions

Can a baby with PVL live a normal life?

PVL symptoms range from mild to severe. One person may lead a relatively normal life, while another may experience significant symptoms .

Is periventricular leukomalacia curable?

No. There is no cure for PVL. Treatment is typically supportive and targeted at addressing symptoms, improving mobility, and supporting learning and behavioral health.

Can a baby recover from brain damage at birth?

Recovery after brain injury is individual and depends on the location and extent of the damage. With PVL, the damage is caused by brain tissue death, so it cannot be reversed.

Takeaway

Speak with your child’s doctor if you have concerns about PVL. Symptoms of this condition may not crop up right after birth, but your doctor can tell you what to be on the lookout for and when to get testing to aid with a diagnosis.

PVL affects people in different ways, so the signs and symptoms may vary. The good news is that treatment can help improve a person’s quality of life.

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