Dolichoodontoid is defined as hypertrophy of the apical portion of the odontoid process of the axis. We report the first case of a dolichoodontoid process in a pediatric patient with other congenital anomalies. This rare entity has had three previous citations in the literature. Our patient presented with scoliosis in which subsequent MRI demonstrated a Chiari I malformation, a small thoracic syrinx and a dolichoodontoid process. The diagnosis of this malformation should be entertained in cases of craniocervical anomalies.

Keywords:
Odontoid process, Dens, Craniocervical junction, Chiari I malformation, Turriodontoid
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© 2002 S. Karger AG, Basel
2002
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