A 3.5 year old boy with Morquio's disease was referred with a persisting left hemiparesis four months after a fall and was found to have craniocervical junction compression due to atlantoaxial subluxation and significant anterior soft tissue compression. Transient unconsciousness at the time of the fall was probably due to medullary concussion as a result of hyperextension, not a head injury. Spinal cord compression due to atlantoaxial subluxation at the craniovertebral junction is a major cause of disability and death in these patients. Once cervical myelopathy appears, early posterior occipitocervical fusion has been advocated in order to arrest the progression of neurological disability and this is successful in most cases. This conventional approach was considered unsafe because of the significant anterior compression. A combined anterior transoral decompression with posterior fusion to deal with this particularly difficult problem is described.
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