A rare unreported case of abnormal craniocervical segmentation and its clinical implications.

Anatomy and Functional Imaging 25.01.2022
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Section: Musculoskeletal system
Case Type: Anatomy and Functional Imaging
Patient: 43 years, male
Authors: Iacopo Chiavacci
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Details
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AI Report

Clinical History

The patient was a 43-year-old male, involved in a motorcycle accident at 80 mph. He was complaining of sternal, left shoulder and cervico-thoracic spinal pain. A full-body trauma CT was performed, including multiplanar soft tissues and bone window reconstructions.

Imaging Findings

A CT of the cervical spine revealed a complex anatomical anomaly of the craniocervical junction. The C1 ring was incomplete, with absence of its anterior arch and fusion of the left lateral masses of C1 and C2. The odontoid process of C2 was also dysplastic.

The left occipital condyle was significantly enlarged and extended infero-medially into the spinal canal to form an articulation with the posterior aspect of the odontoid process and left lamina of C2. The enlarged condyle also caused narrowing of the spinal canal and displacement of the spinal cord to the right, as seen on soft tissue windows.

Discussion

A wide variety of craniocervical junction anatomical variants are described in the literature, comprising but not limited to occipital anomalies (platybasia, basioccipital hypoplasia, occipital condyle hypoplasia, tuberculus paracondylaris and condyles terzius), atlas anomalies (occipital assimilation of the atlas, arch anomalies, ponticulus atlantis) and axis/odontoid anomalies (odontoid hypoplasia, ossiculum terminale, os odontoideum, os avis). [1 - 5]

Embryologically, the craniocervical junction is of mesodermal origin and it appears in the third gestational week. Cells from the embryonic plate condense to either side of the notochord and eventually separate to form segmental clusters called somites. The somites will differentiate into ventromedial sclerotomes and dorsolateral myotomes. The sclerotomes will develop into vertebral bodies, neural arches, ligaments and membranes.

The fourth occipital somite and the cranial part of the first cervical somite form the proatlas, which is the precursor of the craniocervical junction. The first three cervical somites combine to form the first and second cervical sclerotomes, which give rise to the odontoid process and the body of C2. [6]

The abnormality we describe has not been previously published in the literature and it is the result of aberrant development of the proatlas, C1 and C2 sclerotomes. [7]

Other than representing an interesting entity, this can lead to important clinical implications, for example in case of trauma or surgical intervention:

  • An enlarged occipital condyle extending into the cervical canal can lead to significant spinal canal stenosis and spinal cord compression.
  • Due to the chronic narrowing of the spinal canal, similarly to cases of degenerative cervical stenosis, patients with this anomaly are more likely to suffer from spinal cord damage and central cord syndrome in case of trauma.
  • Chronic instability due to abnormal craniocervical biomechanics can lead to exaggerated and premature degenerative changes.

From an imaging point of view, it is important for the Radiologist and Spinal Surgeon to be familiar with common anatomical variants and embryology, making it possible to interpret complex rare anomalies such as the one we described.

Written informed patient consent for publication has been obtained.

Differential Diagnosis List

Complex anatomical anomaly of the craniocervical junction
Os odontoideum
Pre-basioccipital arch
Ponticulus atlantis
Condylus tertius
Condylar hypertrophy

Final Diagnosis

Complex anatomical anomaly of the craniocervical junction

Figures

Partial C1-C2 fusion, cervical spine CT bone window

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Coronal view of the cervical spine through C2, showing a dysplastic odontoid process and fusion of the left lateral masses of
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Left paramedian sagittal view of the cervical spine demonstrating partial fusion of C1 and C2

Enlarged left occipital condyle, cervical spine CT bone window

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Coronal view of the cervical spine demonstrating an enlarged left occipital condyle extending inferiorly to articulate with t
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Sagittal view of the cervical spine demonstrating absence of the anterior arch of C1 and the left occipital condyle articulat

Spinal canal narrowing, cervical spine CT bone and soft tissue reconstructions

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Axial bone widow of the cervical spine demonstrating an enlarged left occipital condyle extending infero-medially and narrowi
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Axial soft tissue reconstructions of the cervical spine demonstrating displacement of the spinal cord to the right but no obv