Case Report: Chiari Zero Malformation

Abstract

Introduction: Chiari zero, initially dubbed syringohydromyelia without hindbrain herniation, is a rare subset of Chiari malformations. The clinical presentation is usually due to syringohydromyelia. The mode of management is foramen magnum decompression. We report a case of Chiari 0 with multiseptated/multiloculated syringohydromyelia and review the literature. Case report: We presented a 41-year-old man with an 8-year history of progressive numbness. A neurological exam revealed left-sided upper-limb hypopselaphesia, diminished algesthesia, and temperature sensation in the left upper limb and trunk. MRI noted cervicothoracic syringohydromyelia with tonsillar herniation. We performed foramen magnum decompression with duraplasty. His syringomyelia reduced significantly, and his neurological status improved during the three-month follow-up. Conclusion: Chiari 0 is a rare entity thought to occur due to altered dynamics in CSF flow at the craniovertebral junction with resultant syringohydromyelia without tonsillar herniation. Foramen magnum decompression with duraplasty remains the most widely accepted surgical intervention.