Periventricular Leukomalacia (PVL) and Cerebral Palsy

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Periventricular leukomalacia (PVL) is a type of brain damage that affects white matter. Babies with PVL have a higher risk of developing cerebral palsy. Although there is no current cure for PVL, several treatment options can minimize complications. Ongoing therapy helps children live with the condition.

What Is Periventricular Leukomalacia?

PVL is a form of brain damage that adversely affects the white matter of the infants’ brains. It can be caused during or shortly after birth, as well as while the baby is still in utero.

PVL is more common in preterm babies, and its development peaks between 24 and 32 weeks of gestation.[1]

When damage occurs to the white matter in the brain, the cells die, and in turn, holes and empty spaces are created in a part of the brain. The periventricular white matter sends nerve impulses that control motor function. Spasticity, cognitive impairment, and vision issues are often a result of PVL.

Infants with PVL are at a heightened risk of developing cerebral palsy. A significant percentage of babies with PVL will also be diagnosed with cerebral palsy.[1]

The name periventricular leukomalacia is derived from the medical terms:

• Periventricular: Around or near the ventricles
• Leuko: White
• Malacia: Softening

What Causes PVL?

Medical researchers have found that lack of oxygen or blood flow to the periventricular area of the brain is the primary reason the condition develops.

Lack of oxygen and blood flow can occur when the infant isn’t delivered in time, such as when a physician fails to perform an emergency C-section.

Babies born too early are at risk as well; premature infants born before 32 weeks gestation are especially susceptible to PVL. Maternal infections and rupture of the amniotic sac are other causes that can lead to PVL.

Other causes can include:

• Using drugs while pregnant
• Failure of physicians to perform an emergency C-section
• Umbilical cord inflammation
• Antepartum hemorrhage
• Carrying twins
• Fetal membrane inflammation
• Infections in the womb during pregnancy
• Asphyxia during pregnancy, labor, and delivery
• Prolonged resuscitation of the infant

PVL Symptoms

Each baby’s symptoms may be unique; sometimes, symptoms may not occur until months after birth. The most common symptom, however, is spastic diplegia, a medical term meaning tight, restricted muscles. Other symptoms may include:

• Loss of coordination
• Vision and hearing problems
• Cognitive impairment
• Impaired development

Spastic diplegia alone doesn’t always mean that babies have PVL. A complete diagnosis must be made first to rule other medical conditions out. According to the Great Ormond Street Hospital for Children, cerebral palsy is the primary symptom of PVL.

How Is PVL Diagnosed?

Since PVL symptoms can mimic other disorders, physicians perform tests for an accurate diagnosis. The first test is generally a cranial ultrasound in which sound waves are used to view the infant’s brain.

Magnetic resonance imaging (MRI) may provide a more in-depth, detailed view of the baby’s brain.[2]

Treatment Options for PVL

There are no specific treatment options for PVL, but the child may go through a series of assessments to diagnose conditions that may develop due to PVL, such as cerebral palsy.

Afterward, depending on any other medical conditions, treatment may consist of physical, speech, and occupational therapy, medications, and assistive devices, if needed.

Keep in mind that treatment for associated medical conditions is generally determined by:

• The infant’s age
• The severity of the medical condition
• How the infant handles (tolerates) certain medications and therapies
• The parents’ opinions and decisions on treatment options

What Is the Prognosis for PVL?

Some infants with PVL will have minor medical issues but otherwise remain healthy. On the other hand, babies with severe PVL may have severe, long-term disabilities, including cerebral palsy.

The prognosis depends on what other medical conditions, if any, the baby develops as a result of PVL.

Is There Any Way to Prevent PVL?

Since unknown factors can cause PVL, prevention can be difficult.

Regular medical checkups during pregnancy while avoiding alcohol and drugs are the best ways to prevent PVL. There is no guarantee, as sometimes PVL can still occur even with the best prenatal care.

In the meantime, researchers and scientists continue to research screening and testing to help better identify intrauterine infections as causes of PVL. Most of the research on PVL is to prevent the condition and find better ways to treat infants diagnosed with PVL.

However, the good news is that PVL is not progressive, meaning it will not worsen over time.