Arnold-Chiari Malformation

Reviewed 06/2017
 


BASICS

DESCRIPTION

  • Chiari disorders involve the cerebellum and craniocervical junction and include alteration of CSF hemodynamics and herniation of the cerebellar tonsils into the spinal canal.

  • There ar...

DIAGNOSIS

HISTORY

  • Depending on the specific malformation, typical symptoms include headache, tinnitus, vertigo, nausea, weakness, dysphagia, neck pain, and dysesthesia.

  • Chiari I: These lesions cause sym...

TREATMENT

MEDICATION

Neuropathic pain may be managed with anticonvulsants and antidepressants in addition to traditional pain medications. 
  • Anticonvulsants for neuropathic pain (off-label) (2)[A]

    • Gabap...

ONGOING CARE

FOLLOWUP RECOMMENDATIONS

Follow-up with neurosurgery after hospital discharge. 

MONITORING

  • Cerebellar tonsils ascend with age. Some lesions presumably resolve spontaneously as children age (...

REFERENCES

Cheng JS, Nash J, Meyer GA. Chiari type I malformation revisited: diagnosis and treatment. Neurologist.  2002;8(6):357–362. [View Abstract on OvidInsights]
Fernández AA, Guerrero AI, ...

ADDITIONAL READING

  • Litvack ZN, Lindsay RA, Selden NR. Dura splitting decompression for Chiari I malformation in pediatric patients: clinical outcomes, healthcare costs, and resource utilization. Neuro...

CODES

ICD10

  • Q07.00 Arnold-Chiari syndrome without spina bifida or hydrocephalus

  • Q07.02 Arnold-Chiari syndrome with hydrocephalus

  • Q07.01 Arnold-Chiari syndrome with spina bifida

  • Q07.03 Arnold-Chiari syndro...

PEARLS

  • Chiari I malformations are the most common and can be treated conservatively in the absence of symptoms (2)[A].

  • Chiari II malformations are treated based on symptoms, typically by surgical decom...

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