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Updated: Feb 13 2018

Chiari Malformation

  • Snapshot
    • An infant boy presents with trouble swallowing, bilateral arm and leg weakness, and stridor. The infant was born to a 28-year-old woman who did not seek prenatal care. On physical exam, the infant barely moves his arms and legs and has stridor. A meningomyelocele is appreciated in the lumbosacral area. Magnetic resonance imaging of the head shows downward displacement of the cerebellar vermis and tonsils through the foramen magnum. (Chiari II malformation)
  • Introduction
    • Clinical definition
      • Chiari malformations describes a
        • heterogenous group of neurological anatomic anomalies involving the
          • cerebellum, brainstem, and craniocervical junction along with
            • the cerebellum being downwardly displaced
    • Epidemiology
      • incidence
        • Chiari malformation type I is
          • the most common type
    • Pathogenesis
      • unclear
    • Associated conditions
      • meningomyelocele
      • spinal cavitations
      • Robin sequence
      • neurofibromatosis type I
      • Noonan syndrome
  • Chiari Malformations
      • Chiari Malformation
      • Type
      • Comments
      • Chiari I
      • Findings
        • typically asymptomatic in children
        • abnormally shaped cerebellar tonsils are
          • downwardly displaced¬†through theforamen magnum
      • Associated conditionsyringomyelia
      • Chiari II (Arnold-Chiari malformation)
      • Findings
        • typically symptomatic
        • cerebellar vermis and tonsil are 
          • downwardly displaced through the foramen magnum
      • Associated condition
        • spinal meningomyelocele
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