{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T11:25:50Z","timestamp":1761823550579},"reference-count":21,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["SPI"],"published-print":{"date-parts":[[2013,6]]},"abstract":"\nObject<\/jats:title>\nIsolated 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.<\/jats:p><\/jats:sec>\n\nMethods<\/jats:title>\nThe 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.<\/jats:p><\/jats:sec>\n\nResults<\/jats:title>\nThere were 11 boys and 1 girl in the aggregate series. The average age of the patients was 7 years (range 13 months\u201314 years), and the duration of symptoms prior to presentation was 6 months (range 0\u201336 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\u201316 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\u201350 months). Laminectomy of C-1 provided neurological improvement in all patients who presented.<\/jats:p><\/jats:sec>\n\nConclusions<\/jats:title>\nIsolated 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.<\/jats:p><\/jats:sec>","DOI":"10.3171\/2013.2.spine1351","type":"journal-article","created":{"date-parts":[[2013,3,29]],"date-time":"2013-03-29T14:00:52Z","timestamp":1364565652000},"page":"558-563","source":"Crossref","is-referenced-by-count":13,"title":["Isolated cervical spinal canal stenosis at C-1 in the pediatric population and in Williams syndrome"],"prefix":"10.3171","volume":"18","author":[{"given":"Sohum K.","family":"Desai","sequence":"first","affiliation":[{"name":"The Paul Randall Harrington Neuro-Spine Program and"},{"name":"Division of Pediatric Neurosurgery, Texas Children's Hospital, Department of Neurosurgery, Baylor College of Medicine, Houston; and"},{"name":"Division of Neurosurgery, University of Texas Medical Branch, Galveston, Texas"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sudhakar","family":"Vadivelu","sequence":"additional","affiliation":[{"name":"The Paul Randall Harrington Neuro-Spine Program and"},{"name":"Division of Pediatric Neurosurgery, Texas Children's Hospital, Department of Neurosurgery, Baylor College of Medicine, Houston; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akash J.","family":"Patel","sequence":"additional","affiliation":[{"name":"The Paul Randall Harrington Neuro-Spine Program and"},{"name":"Division of Pediatric Neurosurgery, Texas Children's Hospital, Department of Neurosurgery, Baylor College of Medicine, Houston; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alison","family":"Brayton","sequence":"additional","affiliation":[{"name":"The Paul Randall Harrington Neuro-Spine Program and"},{"name":"Division of Pediatric Neurosurgery, Texas Children's Hospital, Department of Neurosurgery, Baylor College of Medicine, Houston; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrew","family":"Jea","sequence":"additional","affiliation":[{"name":"The Paul Randall Harrington Neuro-Spine Program and"},{"name":"Division of Pediatric Neurosurgery, Texas Children's Hospital, Department of Neurosurgery, Baylor College of Medicine, Houston; and"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1-spine1351","series-title":"AJNR Am J Neuroradiol","first-page":"249","article-title":"Congenital defects of the posterior arch of the atlas: a report of seven cases including an affected mother and son","volume":"15","author":"Currarino","year":"1994"},{"key":"b2-spine1351","series-title":"Pediatr Neurosurg","doi-asserted-by":"publisher","first-page":"214","DOI":"10.1159\/000121194","article-title":"Anomaly of arch of atlas\u2014a rare cause of symptomatic canal stenosis in children","volume":"26","author":"Devi","year":"1997"},{"key":"b3-spine1351","series-title":"Spine (Phila Pa 1976)","doi-asserted-by":"publisher","first-page":"785","DOI":"10.1097\/00007632-198807000-00013","article-title":"Myelopathy hand characterized by muscle wasting. 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