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Arnold-Chiari Malformation

Diksha Mohanty, MD Reviewed 06/2022
 


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

Asymptomatic patients can be managed conservatively, with routine clinical and MRI follow-up assessments. Prophylactic surgery is also recommended by some. 

GENERAL MEASURES

Symptomatic patien...

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.  [View Abstract]
...

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 surg...

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