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Chiari Malformation

Topic updated on 09/19/17 8:14am

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 the foramen magnum
  • Associated condition
    • syringomyelia
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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