Chiari malformation (CM) is a structural defect in the cerebellum, characterized by a downward displacement of one or both cerebellar tonsils through the foramen magnum and Syringomyelia is a chronic progressive degenerative disorder characterized by a fluid-filled cyst located in the spinal cord. Syringomyelia is often associated with type I Chiari malformation and is commonly seen between the C-4 and C-6 levels. The exact development of syringomyelia is unknown but many theories suggest that the herniated tonsils in type I Chiari malformations cause a "plug" to form, which does not allow an outlet of CSF from the brain to the spinal canal. Syringomyelia is present in 25% of patients with type I Chiari malformations. On the other side, intracranial epidermoid tumor is relatively common congenital lesion wherean inclusion of displaced dorsal midline ectodermal cell rests occurs during neural tube closure. We found a 14-year-old boy with spastic quadriparesis with difficulty in walking with poor balance for 6 months. Magnetic resonance imaging demonstrated a large epidermoid in left cerebellum and syringomyelia extending from C4 to D4 associated with caudal displacement of the cerebellar tonsil (chiari type -1 malformation). A Midline suboccipital craniectomy with extending left suboccipital craniectomy with foramen magnum decompression along with excision of posteior arch of C1 and complete removal of Epidermoid Tumour with expansile duroplasty was done.
After operation, resulted in symptomatic improvement of these symptoms with complete resolution of syrinx & chiari. After surveying the literature, we think, we have found the rarest association, an epidermoid tumor with chiari malformation type I & syringomyelia.
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2022: 6.012
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