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J Neurosurg Spine. 2013 Jun;18(6):558-63. doi: 10.3171/2013.2.SPINE1351. Epub 2013 Mar 29.

Isolated cervical spinal canal stenosis at C-1 in the pediatric population and in Williams syndrome.

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The Paul Randall Harrington Neuro-Spine Program and



Isolated cervical canal stenosis at the level of the atlas (C-1) is a rare cause of cervical myelopathy in the pediatric population. It has been associated with several genetic disorders including spondyloepiphysial dysplasia congenita, Down syndrome, and Klippel-Feil syndrome. The purpose of this study is to highlight the authors' experience with 4 additional pediatric cases, review the literature, and report a new association of this disease with Williams syndrome.


The medical records and radiological imaging studies of 4 patients treated at Texas Children's Hospital for symptomatic hypoplasia of the atlas were retrospectively reviewed. Pertinent patient demographic data, clinical presentation, imaging findings, and outcomes after surgery were recorded. A thorough literature review was performed, allowing the authors to compare and contrast their 4 cases to surgical cases already published in the literature.


There were 11 boys and 1 girl in the aggregate series. The average age of the patients was 7 years (range 13 months-14 years), and the duration of symptoms prior to presentation was 6 months (range 0-36 months). The mean sagittal diameter of the spinal canal at the level of the atlas measured from the posterior aspect of the dens to the anterior aspect of the arch of C-1 was 11.9 mm (range 8.3-16 mm) in the aggregate series. In 2 new pediatric patients with hypoplasia of the atlas the disease was associated with Williams syndrome, which has not been previously described. Patients in the aggregate series were followed for an average of 18 months (range 3-50 months). Laminectomy of C-1 provided neurological improvement in all patients who presented.


Isolated cervical spinal canal stenosis at the level of the atlas is a rare cause of cervical myelopathy. The authors hope that this report will prompt clinicians to consider it when searching for the origin of signs and symptoms of cervical myelopathy, especially in children.

[Indexed for MEDLINE]

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