Osteogenesis imperfecta (OI) is a rare, autosomal inherited disorder due to adefect in collagen I synthesis leading to skeletal deformities with a characteristictendency to fracture bones easily, and ocular, otologic, cutaneous and dentalabnormalities1. Basilar invagination (BI) is one of its rare complications, occurringas the weight of the cranium progressively deforms the unusually soft skull base,producing invagination of the foramen magnum and translocation of the uppercervical vertebrae into the posterior cranial fossa2. This causes brainstemcompression leading to severe neurological disability, respiratory compromiseand even sudden death; anaesthetizing such a patient thus is quite a challenge.We report here a similar patient who underwent transoral odontoidectomy forrelief of neuraxis compression.
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