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Arnold-Chiari Malformation

Brian P. Keene, DO and Michael T. Aguilar, DO Reviewed 06/2018
 


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.

  • Classifi...

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]

    • Gabapen...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Follow-up with neurosurgery after hospital discharge. 

Patient Monitoring

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

REFERENCES

1
McVige JW. Imaging of congenital malformations. Continuum (Minneap Minn).  2016;22(5):1480–1498. {L-End} [View Abstract on OvidMedline]
2
Cheng JS, Nash J, Meyer GA. Chiari type I malf...

ADDITIONAL READING

  • Everson R, Holly L, Batzdorf U. Chiari 1 malformation in the adult: part I. Contemp Neurosurg.  2017:39(4):1–7.

  • Everson R, Holly L, Batzdorf U. Chiari 1 malformation in the adul...

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...

CLINICAL PEARLS

  • Chiari I malformations are the most common and can be treated conservatively in the absence of symptoms (3)[A].

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

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