Chiari Zero: A Rare Case Of Chiari Zero With Syringomyelia

Résumé

Introduction: Chiari zero, initially dubbed syringohydromyelia without hindbrain herniation is a rare subset of Chiari malformations. The clinical presentation is usually due to the syringohydromyelia. The mode of management is mostly surgeon specific. Case report: We presented a 41-year-old man with an 8-year history of progressive numbness. Neurological exam revealed left sided upper limb hypopselaphesia, with diminished algesthesia and temperature sensation in the left upper limb and trunk. MRI noted cervico-thoracic syringohydromyelia with tonsillar herniation. We performed foramen magnum decompression with duraplasty. His syringomyelia reduced significantly and neurological status improved in the 3 month follow up. Conclusion: Chiari zero is a rare entity. Foramen magnum decompression has been the gold standard surgical modality of management. Recently, the concept of ‘central or axial’ atlantoaxial instability was introduced, paving way atlanto-axial stabilization as an alternative mode of management.