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Arnold-Chiari Malformation

Bryan G. Beutel, MD and Minah Tariq, MS Reviewed 04/2024
 


BASICS

DESCRIPTION

  • Arnold-Chiari disorders are a collective group of malformations involving the hindbrain and posterior fossa. Pathological manifestations range from herniation of the cerebellar tonsi...

DIAGNOSIS

HISTORY

Symptoms, including cerebellar and myelopathic, are variable depending upon the specific malformation 
  • Chiari I (symptoms due to two mechanisms)

    • Direct compression of neural structures...

TREATMENT

Asymptomatic patients can typically be managed conservatively, with routine clinical and MRI follow-up assessments 

GENERAL MEASURES

Symptomatic patients require decompressive surgery and/or s...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Follow-up with neurosurgery after hospital discharge 

Patient Monitoring

  • Postoperatively, patients should avoid exercise/lifting for >4 weeks

  • Cerebellar tonsils a...

REFERENCES

1
Klekamp J. How should syringomyelia be defined and diagnosed? World Neurosurg. 2018;111:e729-e745.
2
Giammattei L, Borsotti F, Parker F, Messerer M. Chiari I malformation: surgical techniqu...

ADDITIONAL READING

SEE ALSO

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

  • Sahuquill...

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

  • MRI evaluation is the primary imaging modality for suspected Arnold-Chiari malformations

  • Chiari I malformations are the most common and can be treated conservatively in the absence of s...

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