Periventricular Leukomalacia (PVL) and Birth Injury

“Periventricular” means “around the ventricles,” “leuko” means “white,” and “malacia” means “softening.” Therefore, periventricular leukomalacia (PVL) is a term that refers to damage (or softening) of the brain’s white matter around the ventricles, which are areas of the brain that are filled with cerebrospinal fluid (1). PVL is caused by oxygen deprivation around the time of birth (birth asphyxia). Other risk factors include brain bleeds and infections transmitted from mother to baby during birth (1, 2).

Like many birth injuries, PVL is more common in premature babies, because they are underdeveloped, fragile, and more susceptible to damage (1). However, PVL also can occur in full-term infants. Especially when improperly managed, PVL can lead to permanent motor disorders such as cerebral palsy (CP). It may also cause intellectual and developmental disabilities (I/DDs), seizure disorders, vision and hearing deficits, and other complications (3, 4).

The more premature a baby is, the more susceptible they are to developing PVL. Infants with the highest risk of PVL are those under 32 weeks gestational age (2). (PVL, however, can occur at any gestational age, including at term).

Besides prematurity, the following circumstances increase the likelihood that a baby will develop PVL:

• Birth asphyxia/hypoxic-ischemic encephalopathy
• Intraventricular hemorrhage (a brain bleed inside of the ventricles)
• Maternal infections such as chorioamnionitis
• Premature rupture of membranes (PROM) (1)
• Preeclampsia
• Hypocarbia (low carbon dioxide in the blood) (5)
• Very low birth weight (VLBW) baby
• Maternal obesity (6)

Newborns with PVL do not always manifest obvious signs of this condition, but the following issues may emerge over time:

• Motor control issues and tight/spastic muscles, especially in the legs (2)
• Neonatal seizures
• Intraventricular hemorrhages
• Respiratory distress syndrome (RDS) (5)
• Abnormalities in attention or behavior (6)

Often, signs and symptoms are not noticeable until the child begins to exhibit developmental delays. As the child ages and falls behind peers, the extent of problems caused by PVL becomes more apparent. However, as an infant, problems sucking or unusual stiffness should cause concern regarding PVL.

In diagnosing PVL, medical professionals should take a complete medical history and conduct a physical examination. Additionally, they may use the following tests:

• Cranial ultrasound: This allows them to view the baby’s brain through the fontanelles, or soft spots between skull bones. Babies with PVL may show signs of cysts in the brain tissue.
• Magnetic resonance imaging (MRI): This can show a detailed image of internal structures. It may reveal some of the early abnormalities in babies with PVL (1).

Delaying premature birth, or preventing decreased oxygenation during an inevitable premature birth, is the most important step in decreasing the risk of PVL. Additionally, strategies that emphasize the maintenance of adequate blood supply and flow in the brain should be utilized to help prevent PVL.

While no specific cure exists for PVL, therapeutic hypothermia (brain/body cooling) has proven very effective in treating infants with hypoxic-ischemic encephalopathy at or near term. Hypothermia treatment works by cooling the brain and decreasing the amount of cellular death. When administered very shortly after birth (most guidelines say within six hours), the treatment can prevent or reduce the severity of neurological injury associated with HIE, such as PVL.

Another neuroprotective agent shows promise. Some research has found that when a mother is given the steroid betamethasone during pregnancy, it can reduce the risk of PVL in the baby (7). Further research is needed in this area. Magnesium sulfate can also confer neuroprotective effects on babies likely to be born prematurely and may prevent PVL (8).

Babies with PVL should be monitored for the development of disorders such as cerebral palsy, intellectual impairment, visual problems, and epilepsy.

Once children with PVL are older, they are likely to miss certain developmental milestones due to motor problems and intellectual disabilities.  Visual-motor dysfunction is common and many children with PVL are diagnosed with spastic cerebral palsy.

Areas of negligence that may lead to PVL include the following:

• Failure to properly monitor the baby and recognize fetal distress
• Failure to diagnose and treat disorders that can lead to premature birth
• Failure to prevent issues that can cause hypoxic-ischemic injury
• Improper use of forceps or vacuum extractors
• Failure to obtain adequate informed consent, which includes advising the mother of the risks and alternatives of delivery methods, medication usage, etc.
• Failure to properly deliver the baby and follow standards of care when performing a C-section or vaginal delivery
• Over-ventilation of the newborn, causing hypocarbia
• Failure to timely and appropriately resuscitate.

When underlying conditions are not properly diagnosed and treated, it is negligence.  When conditions like hypoxic-ischemic encephalopathy and PVL result from this negligence, it constitutes medical malpractice.