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Related Experiment Video

Updated: Jun 2, 2026

Comprehensive Endovascular and Open Surgical Management of Cerebral Arteriovenous Malformations
14:58

Comprehensive Endovascular and Open Surgical Management of Cerebral Arteriovenous Malformations

Published on: October 20, 2017

A new entity: Chiari Zero malformation and its surgical method.

Nejat Isik1, Ilhan Elmaci, Mustafa Kaksi

  • 1M.H. Goztepe Education and Research Hospital, Department of Neurosurgery, Istanbul, Turkey. nejatisik@yahoo.com

Turkish Neurosurgery
|May 3, 2011
PubMed
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Long-term outcomes of surgical management in subtypes of Chiari malformation.

Neurological research·2021

Chiari Zero malformation, a condition with syringomyelia symptoms but minimal herniation, was successfully treated with craniovertebral decompression. This suggests a new surgical approach for this specific malformation.

Area of Science:

  • Neurosurgery
  • Neurology
  • Medical Science

Background:

  • Syringomyelia is a condition characterized by a fluid-filled cyst within the spinal cord.
  • Chiari malformation involves the displacement of cerebellar tonsils through the foramen magnum.
  • Chiari Zero malformation is a debated entity presenting syringomyelia symptoms with minimal or no cerebellar tonsillar herniation.

Observation:

  • A 38-year-old male patient presented with cervical syringomyelia, left leg monoparesis, and sensory loss below the T7 level.
  • Initial treatment with syringopleural shunting provided temporary relief, but syringomyelia recurred nine months post-surgery, leading to clinical deterioration.
  • The patient's condition was subsequently classified as "Chiari Zero Malformation" due to the absence of significant tonsillar herniation.

Findings:

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Last Updated: Jun 2, 2026

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  • Craniovertebral decompression was performed despite the lack of tonsillar herniation.
  • Six months post-surgery, the patient experienced complete resolution of syringomyelia and significant neurological improvement.
  • This outcome suggests craniovertebral decompression is an effective treatment for Chiari Zero Malformation.

Implications:

  • The concept of Chiari Zero Malformation is clinically significant and warrants further investigation.
  • Craniovertebral decompression may be a suitable and effective surgical intervention for managing Chiari Zero Malformation.
  • This case expands the understanding of surgical indications for syringomyelia in the absence of classic Chiari tonsillar herniation.