Of note, the largest case series we found was recently published by Nehete et al. We included age of presentation, radiological findings and treatment performed in Table 1. We performed a literature search in the PubMed Database for “Congenital C1 Stenosis” and also cross-referenced articles. For this reason, we performed a literature review of the general considerations, diagnosis and management of C1 stenosis. The literature concerning C1 stenosis is generally based on anecdotal cases or small series. Congenital and acquired (due to transverse ossification or facet osteophytes, for instance) C1 stenosis is an unusual entity that may cause cervical myelopathy with a clinical presentation similar to the subaxial cervical compression. The spinal canal is generally large at the atlantoaxial region, which partially explains the low rate of neurological deficits at presentation in patients with odontoid fractures. Although cervical stenosis has been extensively studied in the subaxial spine, fewer studies evaluated the role and prevalence of stenosis at the level of the atlas, also known as C1 stenosis. Keywords: Atlas, Stenosis, Hypoplasia, Cervical myelopathyĭegenerative cervical spondylosis is the most common cause of myelopathy in elderly patients, and most frequently as a result from stenosis and spinal cord compression below C2. Surgical treatment with a C1 laminectomy is generally necessary and any atlantoaxial or occipito-atlanto instability must be treated with spinal stabilization and fusion. Although the diagnosis can be easily made with a cervical magnetic resonance imaging, the C3/2 spinolaminar test using a lateral cervical plain radiograph is a useful and sensitive tool for screening. It may also be more commonly associated with syndromic conditions such as Down or Turner syndrome. Isolated pure atlas hypoplasia leading to stenosis is quite rare and may be associated with other anomalies, such as atlas clefts or transverse ligament calcification. However, some subjects have anatomical anomalies of the atlas, which may cause stenosis and result in clinical symptoms similar to subaxial cord compression. The cervical canal is generally largest at C1/2, explaining the relatively rare incidence of neurological deficits in patients with odontoid fractures. The vast majority of cervical myelopathy occurs in the subaxial cervical spine. C1 stenosis is often an easily missed cause for cervical myelopathy.
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