Periventricular Leukomalacia (PVL) in Children

What is periventricular leukomalacia (PVL) in children?

Periventricular leukomalacia (PVL) is a softening of white brain tissue near the ventricles. The ventricles are fluid-filled chambers in the brain. These are the spaces in the brain that contain the cerebrospinal fluid. The white matter is the inner part of the brain. It sends information between the nerve cells and the spinal cord, and from one part of the brain to another.

PVL occurs because brain tissue has been injured or has died. A lack of blood flow to the brain tissue before, during, or after birth causes PVL. It's rarely possible to tell when or why this happens. PVL is sometimes linked to bleeding inside the brain (intraventricular hemorrhage). PVL can occur in babies who are born early (preterm or premature).

With PVL, the area of damaged brain tissue can affect the nerve cells that control motor movements. As the baby grows, the damaged nerve cells cause the muscles to become tight or shaky (spastic) and hard to move. Babies with PVL have a higher risk for cerebral palsy. This is a disorder that causes problems with muscle control. A child with PVL may also have thinking or learning problems.

What causes PVL in a child?

It's not clear why PVL occurs. This area of the brain is very vulnerable to injury, especially in preterm babies whose brain tissues are more fragile. PVL may happen when the brain gets too little blood or oxygen. But it's not clear when PVL occurs. It may occur before, during, or after birth. Most babies who develop PVL are preterm, especially those born before 30 weeks. Other things linked with PVL include early bursting or rupture of membranes (amniotic sac) and infection in the uterus.

Which children are at risk for PVL?

PVL can happen in any baby. But the risk is higher in babies who are born preterm. Smaller, younger preterm babies are at higher risk.

What are the symptoms of PVL in a child?

In some mild cases, the condition causes no symptoms. PVL may not be apparent until months after birth. Symptoms can occur a bit differently in each baby. The most common symptom of PVL is spastic diplegia. This is a form of cerebral palsy that causes tight, contracted muscles, especially in the legs. In some cases, the symptoms may be similar to cerebral palsy. Or they can include learning problems or visual problems.

The symptoms of PVL can be like other health conditions. Make sure your child sees their healthcare provider for a diagnosis.

How is PVL diagnosed in a child?

The healthcare provider will ask about your child’s symptoms and health history. They will give your child a physical exam. Your child may also have tests, such as:

• Cranial ultrasound. This is a painless test that uses sound waves to make images of the tissues in the body. This test is used to view the baby's brain through the soft openings between the skull bones (the fontanelles). In a baby with PVL, the ultrasound shows cysts or hollow places in the brain tissue. Sometimes the condition can’t be seen with an ultrasound right away. So healthcare providers give babies at risk for PVL an ultrasound 4 to 8 weeks after birth.

• MRI. This test uses large magnets, radio waves, and a computer to make images of the inside of the body. MRI may show some of the early changes in the brain tissue that occur with PVL.

How is PVL treated in a child?

There is no treatment to cure PVL. Babies at risk for PVL may need special care after discharge from the hospital. Follow-up may include physical therapy, occupational therapy, and speech therapy. Symptoms of PVL, such as spasticity, can be treated with medicine and other therapy.

What are possible complications of PVL in a child?

PVL may lead to problems with physical or mental development. The severity of these problems varies. In these cases, the baby still needs to be checked from time to time for signs of problems. In the most severe cases, PVL can cause cerebral palsy or other serious physical and mental delays. Only time can tell how severe a child’s disability will be.

How can I help my child live with PVL?

If your child is diagnosed with PVL, they should be checked regularly by a developmental specialist. This can help find problems early so you and your child can get help early. If you or other family members need additional support, ask your healthcare providers about community resources or counseling services. Your child may also qualify for early childhood intervention programs through the public school system. Check with your school district about qualifications and available resources.

When should I call my child’s healthcare provider?

Call the healthcare provider if your child has:

• Symptoms that don’t get better, or get worse

• New symptoms

Key points about PVL in children

• Periventricular leukomalacia (PVL) is a softening of white brain tissue near the ventricles. The white matter is the inner part of the brain. It sends information between the nerve cells and the spinal cord, and from one part of the brain to another.

• A lack of blood flow to the brain tissue before, during, or after birth causes PVL.

• PVL can happen in any baby. But the risk is higher in babies who are born preterm.

• Babies with PVL have a higher risk for cerebral palsy. A child with PVL may also have thinking or learning problems. In some mild cases, the condition causes no symptoms.

• There is no treatment to cure PVL. Babies at risk for PVL may need special care after discharge from the hospital. Follow-up may include physical therapy, occupational therapy, and speech therapy.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.

• Before your visit, write down questions you want answered.

• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.

• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.

• Ask if your child’s condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if your child does not take the medicine or have the test or procedure.

• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your child’s healthcare provider after office hours. This is important if your child becomes ill and you have questions or need advice.