Updated: 12/25/2021

Arnold-Chiari Malformation

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  • Snapshot
    • A 9-month-old child presents with with a head circumfrence that is two standard deviations above the norm, frontal-bossing, and translucent skin. An MRI is ordered.
  • Introduction
    • Congenital structural defect of the cerebellum occuring during brain development including
      • noncommunicating hydrocephalus
        • most commonly associated with type II
      • spina bifida
      • meningomyelocele
      • elongated 4th ventricle with kinking of the brainstem
      • displacement of the brainstem and cerebellum into the spinal cord
      • CSF outflow obstruction
      • small posterior fossa
    • Three primary types of malformation are
      • type I
        • most common
        • minimal brainstem herniation, but usually no compression or CSF outflow obstruction
        • may not cause symptoms
        • usually identified by accident
      • type II
        • aka Arnold-Chiari malformation
        • usually accompanied by a myelomeningocele
          • form of spina bifida that occurs when the spinal canal and vertebral column do not close before birth
          • causing the spinal cord to protrude through an opening in the back
        • can result in partial or complete paralysis below the spinal opening
      • type III
        • most severe form
        • usually results in lethal neurological defects
    • Arnold-Chiari syndrome is often associated with other pathology including hydrocephalus, syringomyelia, and spinal curvature
  • Presentation
    • Symptoms
      • range widely depending on the degree of cerebellar and brain stem herniation
      • dizziness
      • muscle weakness
      • numbness
      • visual defects or blindness
      • headache
      • poor motor coordination
      • poor balance
    • Physical exam
      • neurological exam deficits
      • motor deficits
      • mental disability may be noted
      • others as above
  • IMAGING
    • CT/MRI
      • used to identify anatomic abnormality and determine severity of herniation
  • Differential
    • Other developmental defects (neural tube defects), intrauterine trauma, cerebral palsy,
  • Treatment
    • Prevention
      • no preventive measures are available at this time
      • prevent neural tube defects with adequate B12/folate in mothers diet
    • Medical management
      • symptom control
        • usually indicated for pain
    • Surgical intervention
      • indicated for correction of functional disturbances or halt the progression of herniation and damage
      • repeat operations may be required as child continues to grow
  • Complications
    • Permanent paralysis
    • Death as neonate or toddler
  • Prognosis
    • Range widely depending on type and severity of anatomic abnormality
    • Some children with type I function normally, while type III has low survival rate

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