Basilar invagination in a Chiari malformation associated with osteogenesis imperfecta in the pediatric population is a rare entity. We report a case of a seven-year-old female who presented with sudden-onset bilateral spastic quadriplegia and evidence of a basilar invagination on MRI. She underwent emergency decompression of the impinging odontoid via transoral approach followed by posterior wiring and fusion of the C1 and C2 vertebrae.
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