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Information

Nervous System Disorders

= HIE = HYPOPERFUSION INJURY

Frequency: 2–9÷1,000 live births

Cause:

  1. in utero← interruption of placental blood flow + gas exchange:
    • fetal factors: fetomaternal hemorrhage, thrombosis, bradycardia, disrupted umbilical circulation (tight nuchal cord, cord prolapse)
    • inadequate placental perfusion: maternal hypotension, preeclampsia, placental abruption, chronic vascular disease
    • impaired maternal oxygenation: asthma, pulmonary embolism, pneumonia, CO poisoning, severe anemia
  2. postnatally: severe hyaline membrane disease, pneumonia, meconium aspiration, CHD
  3. older child: cardiac arrest, near drowning, asphyxiation (strangling), barbiturate intoxication → circulatory / respiratory failure

Pathophysiology:

⇓cerebral blood flow (= ischemia) and ⇓ blood oxygenation (= hypoxemia) → shift in metabolism from oxidative phosphorylation to inefficient anaerobic oxidation → rapid energy depletion, acidosis, release of inflammatory mediators and excitatory neurotransmitters (particularly glutamate), free radical formation, calcium accumulation, lipid peroxidation → necrosis → cell death

Prognosis:

  1. full recovery
  2. neonatal death (20%)
  3. significant neurologic sequelae (25%): one of the most common causes of cerebral palsy (spastic quadriplegia, diplegia)

DDx: metabolic encephalopathy from inborn errors of metabolism (congenital lactic acidosis, urea cycle disorder, amino aciduria), congenital + neonatal CNS infections, congenital malformations, severe birth trauma

Temporal evolution and optimal imaging modality:

US:

CT:

MR:

Hypoperfusion Injury in Older Child!!navigator!!

Location:

  1. mild HIE: watershed zones
  2. severe HIE: gray matter of cerebral cortex, basal ganglia, hippocampi + sparing of brainstem and cerebral white matter

CT:

  • decreased attenuation of cortical gray matter (diffuse edema)
  • loss of normal gray matter–white matter differentiation
  • bilateral decreased attenuation of basal ganglia and thalami
  • “reversal” sign = higher attenuation of white matter
  • “white cerebellum” sign = lower attenuation of supratentorial brain with relative sparing of cerebellum + brainstem

MR:

  • increased signal intensity on DWI (after 2 hours)
  • subtle increased intensity + swelling of affected areas (after >24 hours)
  • diffuse T2 prolongation in subcortical white matter (= delayed postanoxic leukoencephalopathy)

Hypoperfusion Injury in Preterm Infant!!navigator!!

Gestational age:<36 weeks

Frequency: 5% of infants born <32 weeks EGA

Location:

  • periventricular white matter (mild hypotension)
  • thalami, brainstem, cerebellum = metabolically most active tissue (severe hypotension)

Consequence:

  1. Germinal matrix hemorrhage
  2. Periventricular leukomalacia

US:

  • globular hyperechoic change = PVL (early)
  • localized anechoic / hypoechoic lesions = cystic PVL (2–6 weeks later)
  • progressive periventricular necrosis + ventricular enlargement = end-stage PVL

MR:

  • hyperintense areas on T1WI within larger hyperintense area on T2WI (early)
  • ventricular enlargement with irregular margins of body + trigone of lateral ventricles (later):
  • loss of periventricular white matter with ↑ T2 signal
  • thinning of corpus callosum

Prognosis: germinal matrix hemorrhage upon reperfusion to ischemic tissues

Cx: cerebral palsy in up to 19% of infants <28 weeks EGA

  • 50% of cerebral palsy cases occur in infants born prematurely

Hypoperfusion Injury in Term Infant!!navigator!!

Gestational age:≥36 weeks

Location:

Mild to moderate hypoxic-ischemic injury causes lesions in watershed areas, parasagittal cortex, and subcortical white matter, while sparing brainstem, cerebellum, deep gray matter structures.

Severe hypoxic-ischemic injury involves the lateral + ventral thalamus, posterior putamen, perirolandic sensorimotor cortex, and corticospinal tracts.

Injury pattern:

  1. peripheral pattern (= parasagittal, watershed / borderzone) - more common
    MR:
    • restricted diffusion in parasagittal cortex and underlying subcortical white matter
    • cortical thinning + diminution of underlying white matter
    • ex vacuo dilatation of adjacent lateral ventricles in trigones and occipital horns
  2. basal ganglia–thalamus pattern
    MR:
    • bilateral abnormal T1 hyperintensity (days 3–7) in posterolateral putamen, ventrolateral thalamus, corticospinal tract
    • absent posterior limb sign = loss of normal mildly hyperintense T1 focus in posterior limb of internal capsule
    • indistinct / abnormally iso- or hyperintense T2 foci relative to adjacent gray matter instead of normal hypointense foci in posterolateral putamen, posterior limb of internal capsule, ventrolateral thalamus

Prognosis: 15–20% of neonatal mortality; significant developmental deficits in 25%


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