• Jayesh Sardhara, Sindgikar Pavaman, Kuntal Das, Arun Srivastava, Anant Mehrotra, and Sanjay Behari.
• Sanjay Gandhi Post Graduate Institute of Medical Science, Lucknow, Uttar Pradesh, India.
• World Neurosurg. 2016 Nov 1; 95: 621.e1-621.e5.

BackgroundCongenital spondylolytic spondylolisthesis of C2 vertebra resulting from deficient posterior element of the axis is rarely described in the literature.Case DescriptionWe describe a unique case of agenesis of posterior elements of C2 with craniovertebral junction anomalies consisting of osseous, vascular, and soft tissue anomalies. A 26-year-old man presented with symptoms of upper cervical myelopathy of 12 months' duration. A computed tomography scan of the cervical spine including the craniovertebral junction revealed spondylolisthesis of C2 over C3, atlantoaxial dislocation, occipitalization of the atlas, hypoplasia of the odontoid, and cleft posterior C1 arch. Additionally, the axis vertebra was found devoid of its posterior elements except bilaterally rudimentary pedicles. Magnetic resonance imaging revealed tonsilar herniation, suggesting associated Chiari type I malformation. CT angiogram of the vertebral arteries displayed persistent bilateral first intersegmental arteries crossing the posterior aspect of the C1/2 facet joint. This patient underwent foramen magnum decompression, C3 laminectomy with occipito-C3/C4 posterior fusion using screw and rod to maintain the cervical alignment and stability.ConclusionWe report this rare constellation of congenital craniovertebral junction anomaly and review the relevant literature.Copyright © 2016 Elsevier Inc. All rights reserved.

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