Condylus tertius with atlanto-axial rotatory fixation: an unreported association

The "condylus tertius" or the "third occipital condyle" is an embryological remnant of the proatlas sclerotome. Anatomically, it is attached to the basion and often articulates with the anterior arch of the atlas and the odontoid apex; hence, it is also called the "median occipital condyle". It is a rare anomaly of the cranio-vertebral junction (CVJ) that can lead to instability and compression of important surrounding neurovascular structures. We report a case of a 16-year-old boy who presented with suboccipital neck pain, torticollis and right sided hemiparesis. Plain radiographs revealed an increased atlanto-dental interspace (ADI) with a retroflexed odontoid. Open mouth view showed asymmetry of the articular processes of the atlas with respect to the dens. Computed tomography (CT) of the CVJ delineated the third occipital condyle. Furthermore, on dynamic CT study, a type 3 atlanto-axial rotatory fixation (AARF) was clearly demonstrated. Magnetic resonance imaging (MRI) of the CVJ revealed severe right-sided spinal cord compression by the retroflexed and rightward deviated dens. It also revealed disruption of the left alar and transverse ligaments. The patient was treated with 8 weeks of cranial traction and reasonable alignment was obtained. This was followed by C1-C2 lateral mass screw fixation and C1-C2 interlaminar wiring to maintain the alignment. A review of the literature did not reveal any cases of condylus tertius associated with non-traumatic AARF. An accurate knowledge of the embryology and imaging features of this rare CVJ anomaly is useful in the prompt diagnosis and management of such patients.