Symptomatic Atlas Hypoplasia in a Latin-American Patient: Case Report and Literature Review

BACKGROUND: Atlas hypoplasia is an infrequent cause of upper cervical stenosis. Only 24 cases in nonsyndromic adult population have been published. We are not aware of previous reports describing isolated fully formed atlas hypoplasia in a Latin-American patient. The purpose of this work was to report a case of an 80-year-old Argentinian woman with cervical myelopathy because of atlas hypoplasia and a literature review about this subject. METHODS: A clinical case and an extended review of the literature are presented. We assessed from each case: age, sex, posterior atlanto-dens interval, surgical treatment, outcomes, and follow-up period. RESULTS: Neurologic symptoms markedly improved after posterior decompression from severe to moderate in the Japanese Orthopaedic Association Scoring System and from four to three on the Nurick scale. Twenty-five patients were analyzed (mean 58.4 years, 32% female). The mean posterior atlanto-dens interval was 8.8 mm. Twenty-three patients underwent decompression alone, and two needed posterior fusion. All patients reported clinical improvement at an average follow-up of 13 months. CONCLUSION: Cervical myelopathy caused by fully formed atlas hypoplasia is not an exclusive pathology of far east population, and it may present in nonsyndromic patients. Surgical treatment by C1 laminectomy improved neurologic impairment. STUDY DESIGN: Case report and literature review.