Periventricular Leukomalacia (PVL) and Birth Injury*

Periventricular leukomalacia is brain injury to white matter, where the white matter softens and dies around the lateral ventricles, leaving fluid-filled cysts. PVL can occur preterm and term babies. PVL results from trauma, hypoxia or ischemia.

PVL occurs as the result of a ‘domino effect’ of inflammatory responses that occur after an oxygen deprivation-related injury to the blood-brain barrier. There are often few or no signs of PVL in newborns, and the issue can be missed entirely without routine screening. Problems suckling or unusual stiffness can be a sign of periventricular leukomalacia. Preliminary diagnoses are based on head imaging results, with the most effective method being an MRI.

The best way to treat PVL is to prevent it in the first place – neuroprotective drugs can be used in pregnancies at risk for PVL, and all steps should be taken to prevent premature births as well as hypoxia, ischemia, trauma, and overventilation. In eligible cases, hypothermia treatment must be used for infants who have hypoxic ischemic encephalopathy (HIE) that leads to PVL.

Medical Explanations

What Is Periventricular Leukomalacia (PVL)?

Periventricular leukomalacia (PVL) is a type of brain damage that leads to the development of cerebral palsy, epilepsy, developmental delays, intellectual impairment and visual disturbances.  Specifically, PVL refers to white matter brain injury, characterized by softening or necrosis (death) of white matter near the lateral ventricles, which are located in the top section of the brain and provide pathways for cerebral spinal fluid.

White matter helps transmit messages throughout the largest part of the brain.  When the white matter tissue softens and dies, cysts filled with fluid may be left behind. Lack of oxygen (hypoxia), and too little blood (ischemia) to the brain are the main causes PVL injuries.  PVL can develop prenatally, due to poor prenatal care; it can develop during birth due to birth injuries such as trauma, hypoxia and ischemia; and it can occur after birth, due to hypoxia / ischemia.

Causes of Periventricular Leukomalacia (PVL)

The younger and smaller a baby is, the more prone she is to developing PVL.  Infants at the highest risk of developing PVL are those under 32 weeks of gestational age.  PVL, however, can occur at any gestational age, including at term, especially if the baby suffered a hypoxic / ischemic insult.  Factors involved in the development of PVL are: 1.) decreased oxygen / blood flow to the periventricular region, and 2.) damage to the glial cells, which comprise most of the white matter and support neurons throughout the nervous system.  These factors can create a sequence of events that leads to the development of PVL.

There are a number of events that can cause hypoxia / ischemia and resultant PVL.  Fetal blood vessels have very thin walls, and the vessels providing nutrients to the periventricular region cannot maintain sufficient blood flow during periods of low oxygen.  In addition, low blood pressure (hypotension) caused by birth trauma, fetal distress, and post-birth hypocarbia and hypotension can lead to decreased blood and oxygen to the developing brain.  These hypoxic-ischemic events can cause damage to the blood brain barrier, a network of cells that regulates the flow of nutrients to the brain.  A damaged blood brain barrier can further contribute to greater levels of hypoxia.  Injury to the blood brain barrier also can occur as a result of maternal infection during pregnancy, fetal infections, or infection of the newly delivered baby.  Premature infants are especially susceptible to these insults because their cardiovascular and immune systems are not fully developed and they do not have the ability to auto-regulate cerebral blood flow.

When hypoxic-ischemic injury or infection causes damage to the blood brain barrier, there is a cascade of inflammatory responses.  One of these responses includes release of molecules called cytokines, which are toxic to the developing brain.  Cytokines perpetuate brain injury by damaging nearby areas that were not involved in the initial injury.  Further damage is caused by free radicals, which are molecules produced during ischemic episodes.  Additionally, when the glial cells become damaged, nearby neurons have little or no support.

Risk Factors for Periventricular Leukomalacia (PVL)

  • Prematurity
  • Hypoxic events during or soon after birth
  • Hypocarbia or overventilation (low carbon dioxide in the blood)
  • Moderate and severe intraventricular hemorrhage (IVH)
  • Lengthy resuscitation following birth
  • Maternal infections, such as chorioamnionitis and villitis
  • Fetal infections
  • Infections of the newborn, such as herpes encephalitis (HSV)
  • Respiratory distress
  • Pneumonia
  • Hypotension

Signs and Symptoms of Periventricular Leukomalacia (PVL)

Many times, PVL is silent and there are no signs, symptoms or encephalopathy.  In some cases, PVL will not manifest fully until about 8 weeks after birth.  Often, signs and symptoms are not noticeable until much later due to the fact that children with PVL typically exhibit motor problems, and newborns can’t perform many specific tasks.  As the child develops, the extent of problems caused by PVL can start to be identified.  As an infant, problems sucking or unusual stiffness should cause concern regarding PVL.

Other signs and symptoms of PVL include the following:

  • Delayed motor development (for example, infants may have all leg joints either flexing or extending)
  • Weakness or altered muscle tone, primarily in the lower extremities
  • Vision problems
  • Periods of apnea (breathing cessation)
  • Low heart rates
  • Seizures

Diagnosing Periventricular Leukomalacia (PVL)

PVL typically begins with numerous, small areas of necrosis, followed in more severe cased by cystic formation and more diffuse white matter injury.  Newborns typically exhibit few or no signs of white matter injury, and PVL can easily be missed if routine screening is not performed.  Furthermore, prematurity, extreme stiffness, and / or poor ability to suckle should alert the physician that there may be white matter damage.

The preliminary diagnosis of PVL often is made using head imaging technologies.  Soon after an at-risk baby is born, physicians typically order an ultrasound to determine if the baby has white matter damage.  However, the low sensitivity of an ultrasound allows for some white matter injury to be missed.  Magnetic resonance imaging (MRI) is much better than an ultrasound at identifying PVL.  MRI can show lesions that are less than 0.5 cm, as well as noncystic PVL, while an ultrasound typically cannot.  An MRI should be performed on infants who had a difficult course of development, especially if there was prematurity, maternal or fetal infection, a known hypoxic / ischemic event during or right after birth, or a traumatic head injury.

Computed tomography, or CT scanning, is less useful for the diagnosis of PVL in very preterm infants because it detects fewer lesions than does MRI or ultrasound.  In older infants, however, CT can identify the loss of periventricular white matter, as well as ventricular enlargementEven with the best head imaging, however, some white matter damage will be missed and films will be read as normal.

Modes and timing of diagnostic techniques include the following:

  • Although PVL often cannot typically be seen on an initial ultrasound scan in the first 24 hours after brain insult, the scan will usually show periventricular damage after 24 hours of life.  If there are cystic changes they will usually be seen after approximately 1 – 3 weeks.  This emphasizes the importance of serial scans.
  • Routine ultrasound screening should be performed on all infants with a gestational age less than 33 weeks, or with birth weight less than 1500 grams to identify abnormalities in the brain.  Screening should also be performed in all births where birth trauma is suspected.
  • Screening should be performed to detect IVH (brain bleeds), with an initial scan being performed on extremely low birth weight infants at risk for abnormalities.
  • When abnormalities are detected, more frequent examinations are performed to assess the progression of PVL.
  • MRI should be used for better assessment of the extent of white matter damage.  An MRI has better sensitivity and specificity than ultrasound and CT scans and can show injury at 1 day of age.

Prevention and Treatment of Periventricular Leukomalacia (PVL)

Delaying or preventing premature birth is considered one of the most important steps 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.  Low blood pressure, constricted blood vessels in the brain, and conditions that impair normal functioning of the blood vessels should be avoided.  It is therefore essential to prevent hypocarbia, hypoxemia and abnormal blood pressure.

While no specific cure exists for PVL, hypothermia (brain / body cooling) has proven very effective in treating infants with hypoxic ischemic encephalopathy, or HIE.  Hypothermia treatment works by cooling the brain and decreasing the amount of cellular death.  When administered within six hours of life, 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.  Further research is needed in this area.

In general, therapy for PVL focuses on teaching caregivers to handle, feed, dress, and toilet their children.  Babies with PVL should be monitored for the development of disorders such as cerebral palsy, intellectual impairment, visual problems and epilepsy

Periventricular Leukomalacia (PVL) and Medical Malpractice

Areas of negligence may 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, such as cord compression, nuchal cord, chorioamnionitis, premature rupture of membranes, delayed emergency C-section / delayed delivery, placental abruption, uterine rupture, improper use of pitocin and cytotec, and prolonged and arrested labor
  • 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 and medication usage
  • Failure to obtain adequate informed consent regarding the risks and alternatives of the use of delivery instruments, such as forceps and vacuum extractors
  • 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

It is crucial for physicians to closely monitor the mother and baby and prevent premature birth.  Standards of care must be followed at all times to avoid infection, hypoxic / ischemic insults, overventilation and resultant PVL.  It is essential for physicians to properly treat underlying conditions that can cause PVL.  When underlying conditions are not properly diagnosed and treated, it is negligence.  When the mother and baby are not properly monitored, or standards of care are not followed and hypoxia / ischemia occur, it also constitutes negligence.  If this negligence causes PVL, it is medical malpractice.

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