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Chiari malformation

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The following summarized guidelines for the evaluation and management of chiari malformation are prepared by our editorial team based on guidelines from the Congress of Neurological Surgeons (CNS 2023) and the Canadian Cardiovascular Society (CCS 2020).
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Guidelines

1.Screening and diagnosis

Screening of siblings: do not obtain routine screening in asymptomatic siblings or first-degree relatives of patients with Chiari malformation type 1.
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2.Diagnostic investigations

Diagnostic imaging: consider obtaining further brain and spine imaging to evaluate for clinically relevant pathology, such as hydrocephalus or spine syrinx, in patients with Chiari malformation type 1 diagnosed with brain or cervical spine MRI.
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3.Nonpharmacologic interventions

Activity restrictions: do not advise activity restrictions in patients with asymptomatic Chiari malformation type 1 without syrinx, as there is no evidence of future harm prevention.
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4.Surgical interventions

Indications for surgery, asymptomatic patients: do not perform prophylactic surgery in patients with asymptomatic Chiari malformation type 1 without syrinx. Recognize that there is a small percentage of patients developing new or worsening symptoms in the future.
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  • Indications for surgery (symptomatic patients)

  • Intraoperative monitoring