Periventricular leukomalacia

Periventricular leukomalacia (PVL) or white matter injury of prematurity affecting the periventricular zones, and typically results in cavitation and periventricular cyst formation. 

It is important to note that both periventricular and subcortical leukomalacia correspond to a continuous disease spectrum. Please refer to the article on patterns of neonatal hypoxic–ischaemic brain injury for a relation between perinatal brain maturation process and these lesions.

PVL is most common in premature neonates (less than 33/40) and <1500 grams at birth. 

PVL may manifest as cerebral palsy (> 50%), intellectual disability or visual disturbance. 

It likely occurs as a result of hypoxic-ischemic lesions resulting from impaired perfusion at the watershed areas, which in premature infants are located in a periventricular location. It is likely that infection or vasculitis also play a role in pathogenesis. 

  • early: periventricular white matter necrosis 
  • subacute: cyst formation
  • late: parenchymal loss and enlargement of the ventricles
Distribution

The white matter necrosis often occurs in a characteristic distribution with the pattern being dorsal and lateral to the lateral ventricles and with involvement of the centrum semiovale, the optic (trigone and occipital horns) and acoustic (temporal horn) radiations.

Ultrasound

Cranial ultrasound provides a convenient, non-invasive, relatively low-cost screening examination of the hemodynamically unstable neonate at the bedside. The examination also imparts no radiation exposure. Sonography is sensitive for the detection of hemorrhage, periventricular leukomalacia and hydrocephalus.

On ultrasound, hyperechoic areas are firstly identified in a distinctive fashion in the periventricular area, more often at the peritrigonal area and in an area anterior and lateral to the frontal horns (periventricular white matter should be less echogenic than the choroid plexus).

These are watershed areas that are sensitive to ischemic injury. Follow-up scans in the more severely affected patients may reveal the development of cysts in these areas, known as cystic PVL (when cystic PVL is present, it is considered the most predictive sonographic marker for cerebral palsy).

Classification

See: sonographic grading of PVL.

MRI

Initial MR images depict areas of T1 hyperintensity within larger areas of T2 hyperintensity.

Subsequent cavitation and periventricular cyst formation, features that are required for a definitive diagnosis of PVL, develop 2-6 weeks after injury and are easily seen on sonograms as localized anechoic or hypoechoic lesions. Progressive necrosis of the periventricular tissue with resulting enlargement of the ventricles is called end-stage PVL. CT and MR imaging findings of end-stage PVL include ventriculomegaly with irregular margins of the bodies and trigones of the lateral ventricles, loss of periventricular white matter with increased T2 signal, and thinning of the corpus callosum.

The differential for periventricular echogenicity in neonates on ultrasound include

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Article information

rID: 12858
Section: Pathology
Tag: refs
Synonyms or Alternate Spellings:
  • Leukomalacia
  • Periventricular leukomalacia (PVL)
  • Periventricular leucomalacia
  • Periventricular leucomalacia (PVL)
  • Periventricular leukomalacias

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