Periventricular Leukomalacia (PVL) Brain Injuries

Periventricular leukomalacia (PVL) is a specific type of injury to the brain which sometimes affects babies born prematurely. It is the most common birth-related brain injury that is caused by a lack of blood flow during labor and delivery.

What Is PVL?

PVL occurs when the white matter in the ventricles of the brain becomes damaged or decayed. White matter is very important because it facilitates electrical impulse signals that the brain uses to control the rest of the body. The cellular decay of the white matter tissue creates voids or holes within the brain. These holes eventually fill up with fluid which results in the condition known as PVL.

While there are many success stories, periventricular leukomalacia can result in permanent physical and mental disabilities and PVL can have many long-term effects, including cerebral palsy and epilepsy.

• PVL vs. Cerebral Palsy: PVL and cerebral palsy are related. Cerebral palsy is a cluster of neurologic movement disorders resulting from injury to a baby’s developing brain during childbirth or pregnancy. PVL is a very specific type of injury to the brain that develops during pregnancy. PVL frequently leads to cerebral palsy but the 2 conditions are different. The term “PVL CP” is sometimes used to classify cases of cerebral palsy that were originally triggered by periventricular leukomalacia.

Causes of PVL

Interruption or restriction of blood flow around the ventricles of the developing brain of a fetus during gestation is believed to be one of the principal causes leading to PVL. In the early stages of pregnancy, the fetus and particularly its brain ventricles are especially vulnerable to injury or disruption of normal development.

Any type of restriction or interruption of the supply of oxygenated blood from mother to baby to the baby’s brain during pregnancy can potentially damage white matter and result in PVL. Infections of the fetal membranes (chorioamnionitis) and other types of maternal infection are a common source of interruption to blood and oxygen flow.

PVL Symptoms

PVL is typically present when the baby is born but a formal diagnosis is normally not made until months later. Periventricular leukomalacia symptoms will often be different in each child, but the most common universal symptoms of PVL include:

• Abnormal developmental delays (failure to reach development milestones)
• Noticeably poor body control
• Spasticity, contractions, or tightness of the muscles (usually in the legs)
• Impaired vision and lack of eye control

Clinical seizures are also a common symptom of PVL in some babies with more severe injuries. Almost all of these common PVL symptoms are also common symptoms of other birth injuries. This can make differential diagnosis a challenge.

Getting to a formal diagnosis of PVL can be a long and complicated process involving countless exams and diagnostic brain tests to breakdown the child’s PVL symptoms.

A cranial ultrasound is often the first diagnostic imaging tool used in diagnosing PVL. A cranial ultrasound is similar to a pregnancy ultrasound. It is performed on the newborn’s head and gives doctors images of the brain. The image results of a cranial ultrasound are analyzed to spot abnormalities associated with PVL. A Computed Tomography (“CT”) scan and an MRI (Magnetic Resonance Imaging) are also used in the diagnosis of PVL.

PVL Treatment & Prognosis

There is currently no effective treatment for PVL. Once the damage occurs, the decayed white matter in the brain will never regenerate and cannot be repaired or restored. Although PVL is a permanent injury, careful monitoring and effective management of the condition can help limit its long term effects and symptoms.

Rehabilitative physical therapy and occupational therapy are typically utilized to help manage PVL. Medications can also be prescribed to address certain physical symptoms such as seizures.

Keep in mind what “no effective treatment” means. While there is no PVL specific treatment, as we said there are success stories. There are many children who have only mild PVL symptoms. Great improvement in long-term outcomes is often the result of physical and cognitive therapy (and loving your child with everything you have). The key? Find the best doctors you can. There are good doctors everywhere. But you are more likely than to not to get superior care at a teaching university than you would from a small town pediatrician.

PVL is not a progressive condition so its symptoms do not gradually worsen as the child gets older. The damage to the white matter occurs during brain development and does not continue to expand after birth. Although not progressive, the physical symptoms of PVL usually become more and more noticeable as the child ages.

What Is Difference Between PVL and White Matter Disease?

There are differences between white matter disease and PVL. White matter disease is a medical condition in adults caused by the deterioration of white matter in the brain over time. White matter disease differs from PVL in that it occurs in certain adults, not babies.

White matter serves as a protective coating for the nerve fiber network that connects the spinal cord and the brain. These nerve pathways are important because the brain uses them to transmit impulse signals through the spine to control body movement. White matter is comprised largely of a white fatty substance called myelin. White matter disease happens when the myelin in the white matter breaks down and decays away leaving nerve fibers exposed which causes interruption of the signals from the brain to the body.

Deterioration of white matter is typically something that occurs in elderly patients. It is known to be triggered by certain chronic health conditions including:

• chronic hypertension
• chronic inflammation of blood vessels
• cigarette smoking.

Genetic predisposition and diabetes are also risk factors for white matter damage. Like PVL, there is no effective cure for white matter disease. Damage to white matter cannot be restored or reversed. Treatment of white matter disease aims only to slow down or stop the continuing erosion of myelin in the white matter to prevent the condition from getting worse.

Medical Sources & Studies for PVL

Gotardo, Juliana Wendling, et al.: “Impact of peri-intraventricular hemorrhage and periventricular leukomalacia in the neurodevelopment of preterms: A systematic review and meta-analysis.” PLOS ONE 14.10 (2019): e0223427. (This study determined that any degree of periventricular leukomalacia and peri-intraventricular hemorrhage negatively impacts neurodevelopment.)

Huang, Jichong, et al.: “Association between perinatal hypoxic-ischemia and periventricular leukomalacia in preterm infants: A systematic review and meta-analysis.” PLOS ONE 12.9 (2017): e0184993. (This study looked at perinatal hypoxic-ischemia signs associated with periventricular leukomalacia. The researchers found that oligohydramniotic mothers, metabolic acidosis in preterm infants, infants with low Apgar scores, and infants with respiratory distress increased the PVL risk.)

Jalali, Ali, et al.: “Prediction of periventricular leukomalacia in neonates after cardiac surgery using machine learning algorithms.” Journal of Medical Systems 42.10 (2018): 1-11. (Study found that wavelet analysis and machine learning algorithms can predict PVL occurrence in infants who just underwent heart surgery)

Morioka, Chikako, et al.: “Neuroprotective effects of human umbilical cord-derived mesenchymal stem cells on periventricular leukomalacia-like brain injury in neonatal rats.” Inflammation and Regeneration 37.1 (2017): 1-10. (This study looked at whether mesenchymal stem cells from human umbilical cords can treat PVL-like brain injuries in four-day-old rats. Results were not conclusive.)

Papadimitriou, Ioanna, et al.: “Dystonia assessment in children with cerebral palsy and periventricular leukomalacia.” European Journal of Paediatric Neurology 32 (2021): 8-15. (This study assessed dystonia in children with periventricular leukomalacia and cerebral palsy. The researchers analyzed 31 patients’ motor functions, manual abilities, spasticity, and dystonia presence. They found that all of them experienced upper body dystonia. Twenty-nine percent of cases only involved the limbs and 71 percent involved multiple body parts. The researchers concluded that dystonia severity, not spasticity, was associated with motor function issues among children with CP and PVL.)