What is periventricular leukomalacia (PVL)? 

Periventricular leukomalacia (PVL) is characterized by the death or damage and softening of the white matter, the inner part of the brain that transmits information between the nerve cells and the spinal cord, as well as from one part of the brain to another.

• Periventricular means around or near the ventricles, the spaces in the brain containing the cerebrospinal fluid
• Leuko means white
• Malacia means softening

Why is periventricular leukomalacia a concern?

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 spastic, or tight, and resistant to movement. Babies with PVL have a higher risk of developing cerebral palsy (a group of disorders that prevent the child from controlling their muscles normally), and may have intellectual or learning difficulties. PVL may occur alone or in addition to intraventricular hemorrhage (bleeding inside the brain).

What causes periventricular leukomalacia?

It is not clear why PVL occurs. This area of the brain is very susceptible to injury, especially in premature babies whose brain tissues are fragile. PVL may happen when the brain receives too little oxygen. However, it is not clear when the trigger for PVL occurs--before, during, or after birth. Most babies who develop PVL are premature, especially those born before 30 weeks gestation. Other factors that may be associated with PVL include early rupture of membranes (amniotic sac) and infection inside the uterus.

What are the symptoms of periventricular leukomalacia?

PVL may not be apparent until later months. Each baby may experience symptoms differently. The most common symptom of PVL is spastic diplegia, a form of cerebral palsy characterized by tight, contracted muscles, especially in the legs. The symptoms of PVL may resemble other conditions or medical problems. Always consult your baby's doctor for a diagnosis.

How is periventricular leukomalacia diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for PVL may include:

• Cranial ultrasound. A painless test that uses sound waves to view the baby's brain through the fontanelles, the soft openings between the skull bones. With PVL, the ultrasound shows cysts or hollow places in the brain tissue.
• Magnetic resonance imaging (MRI). This test uses a combination of a large magnet, radio frequencies, and a computer to produce detailed images of internal structures. MRI may show some of the early changes in the brain tissue that occur with PVL.

Treatment for periventricular leukomalacia

There are no treatments for PVL. Management of the problems that can result from PVL will be determined by your baby's doctor based on:

• Your baby's gestational age, overall health, and medical history
• Extent of the condition
• Your baby's tolerance for specific medications, procedures, or therapies
• Expectations for the course of the condition
• Your opinion or preference

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.