Case Report: Chiari Zero Malformation
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Date
2023
Authors
Njau, Mwangi Ken
Nyangau, Nyaoncha Andrew
Arnold, Adili Wobenjo
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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
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Keywords
Chiari zero, Chiari-like malformation, syringomyelia, tight cisterna magna, atlantoaxial instability, foramen magnum decompression, atlantoaxial fusion
Citation
Mwangi, K. N., Nyangau, A. N., & Wobenjo, A. A. (2023). Case Report: Chiari Zero Malformation. East African Journal of Neurological Sciences, 2(1), 43-47.