Periventricular Leukomalacia (PVL)
Periventricular leukomalacia (PVL) is a form of brain damage that affects the white matter of brain, resulting in the cells in the white matter of brain either decaying or dying. In turn, an area of the brain is empty, resulting in fluid buildup.
What is Periventricular Leukomalacia
Periventricular Leukomalacia is a type of brain damage that most often affects premature infants. PVL causes small areas of brain tissue around the ventricles in the brain to die. This results in “holes” in the brain. “Periventricular” refers to the area of the brain surrounding the ventricles. “Leuko” refers to the white matter in the brain.
What Causes Periventricular Leukomalacia?
Periventricular leukomalacia causes cell damage to the brain’s periventricular tissue as well as a decrease in blood flow. These are the two primary reasons why this condition occurs. In addition, babies born prematurely, especially before 32 weeks gestation, have a heightened risk of PVL. Unfortunately, premature infants are also at the highest risk of death should they develop PVL.
Other possible causes of PVL include:
- Having twins
- Umbilical cord inflammation
- Infection around the time of delivery
- Antepartum hemorrhage
- Problems with the placental blood vessel
- Sepsis and other illnesses in which bacteria enters the bloodstream
- Oxygen deprivation to the periventricular area of the infant’s brain
Symptoms of Periventricular Leukomalacia
Unfortunately, PVL is extremely difficult to detect, especially in newborns and infants under 6 months of age. Since PVL mimics so many other medical conditions, and most infants show no outward signs of impairment, it may take months or even years before a formal diagnosis is made. However, there are certain signs and symptoms to look out for, including:
- Difficulties with coordination
- Intellectual and cognitive impairment
- Vision problems
- Hearing impairment
How Periventricular Leukomalacia Affects Children
It’s estimated that around 60-100 percent of all children who have PVL will also develop cerebral palsy (CP). In most cases, spastic diplegia is the most typical type of CP that develops due to PVL. Although uncommon, quadriplegia CP may also develop. Quadriplegic life expectancy, with or without PVL, may be lower than other types of CP.
Treatment for Periventricular Leukomalacia
If PVL is suspected, your child may need to undergo a series of development assessments in order to get an accurate diagnosis, as well as a cranial ultrasound, which is usually reserved until the infant is at least over 6 months of age, as performing an ultrasound too early may not detect PVL. Older than this age, an MRI is usually done.
Treatment will then depend upon the severity of the disorder, but typically includes a series of therapy plans, including physical, massage, and speech. If vision is impaired, your physician may recommended corrective vision treatment.
Periventricular Leukomalacia Prognosis
Prognosis greatly depends upon the severity of the disease. While some children will have relatively minor problems, others may have severe disabilities and deficits for life. The best course of action is to ensure you and your physician find and carry out the best treatment plan for your child’s situation.
How to Optimize Your Child’s Prognosis
In an ongoing effort to understand more about PVL and other types of beneficial treatments, the National Institute of Neurological Disorders and Stroke conduct regular research on PVL as well as other brain injury disorders. The NIH offers clinics and clinical trials across the nation. Furthermore, organizations such as the National Organization for Rare Disorders help research and study PVL in an attempt to treat and help find a cure. For more information or to get involved, contact the organization directly at 888-999-NORD.
For parents, participating in research and awareness is important. It is also important to work closely with your child’s healthcare team to routinely assess your child’s condition and treatment options that may be the most appropriate.