Anat Cell Biol.  2020 Dec;53(4):522-526. 10.5115/acb.20.189.

Duplication of the odontoid process with other congenital defects of the craniocervical Junction: case report and review of the literature

• Zeoli T
• Iwanaga J
• Bui C
• Dumont AS
• Tubbs RS

• KMID: 2509702
• DOI: http://doi.org/10.5115/acb.20.189

Abstract

Duplication of the odontoid process remains a rare developmental pathology that is underrepresented in the current literature. As the pivot point for the craniovertebral junction, the odontoid process is vital for the integrity of the atlanto-axial joint and the ability of the head and cervical spine to rotate correctly. The pathogenesis being incompletely understood, it has been proposed that odontoid process duplication involves faulty sclerotome migration and disruption of the axis ossification center. Patients presenting with this pathology usually have associated structural abnormalities. A detailed anatomical and embryological understanding of the odontoid process is necessary for successful management and treatment of patients presenting with odontoid process duplication. We present a rare case of a patient with a duplicated odontoid process in association with C2–C3 fusion, incomplete anterior arch of C1, variant inferior bony process of the transverse process of C1, and enlarged right jugular foramen.

Keyword

Odontoid process duplication; Odontoid process; Axis; Duplicated odontoid process

Figure

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  Fig. 1 Three-dimensional reconstructed computed tomography of the craniocervical junction in the patient presented herein. Note the two ossification centers (arrow heads) of the duplicated odontoid process via a posterior view through the foramen magnum and from a posterior view (left). On this same image, the right jugular foramen (arrow) was significantly enlarged compared to the left. From an anterior view (right), note again the two ossification centers for the apical parts of the odontoid processes (arrow heads), the split anterior arch of C1 (asterisks), and the fused C2 and C3 vertebrae (black arrow). Also, note the right-sided inferior bony extension from the transverse process of C1 (white arrow).

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  Fig. 2 Schematic drawing of the various ossification centers of the axis. Note the DCS between the body and odontoid process and the NCS separating the odontoid process and body of the axis from the remaining parts of C2. Smaller sits of fusion include the synchondrosis between the apex of the odontoid process and its lower parts and the midline fusion of left and right sides of the odontoid process. DCS, dentocentral synchondrosis; NCS, neurocentral synchondrosis.

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  Fig. 3 Schematic drawing of the embryological derivations of the craniocervical junction. Note the early sclerotome contributions to this region. The first two occipital somites (yellow) give rise to the regions of the clivus at the skullbase and the third occipital somite (green) gives rise to parts of the occipital bone and region of jugular foramen. The proatlas (fourth occipital somite) gives rise to the apical region of the odontoid process, occipital condyle, and lateral mass of the atlas. The first cervical sclerotome gives rise to the anterior and posterior arches of the atlas, and the body of the odontoid process.

Reference

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