Abstract
The authors report a case of Klippel-Feil syndrome associated with atlantoaxial dislocation and basilar impression treated with transoral odontoidectomy. An 8-year-old girl was admitted to the author's hospital with a two-year history of weakness in the upper and lower extremities and recent development of dyspnea. The neurological examination revealed spastic tetraparesis. Radiological examination showed a fused vertebrae at C2 and C3, atolantoaxial dislocation and basilar impression. The patient underwent atlantoaxial arthrodesis by the Brooks method. The atlantoaxial dislocation could not be reduced so the authors decompressed the foramen magnum, and performed C1 laminectomy and occipito-axial fusion. Consequently tetraparesis and dyspnea gradually improved. Eleven months after the second procedure, tetraparesis had gradually worsened and the patient was readmitted to hospital. The reason for the change in tetraparesis was attributed to progression of anterior compression of the medulla oblongata by the odontoid. The authors performed transoral odontoidectomy to achieve anterior decompression. The authors dislocated the bilateral mandible joints, opened the mouth for 4cm and released the soft palate, and removed the odontoid. Post operatively symptoms gradually improved.
