Patients with type IV Ehlers-Danlos syndrome may be predisposed to atlantoaxial subluxation
Abstract
OBJECTIVE: To determine if atlantoaxial subluxation is a complication of Ehlers-Danlos Syndrome (EDS).
METHODS: Observational study of a selected cohort of patients with EDS attending a national meeting. Twenty-six patients with EDS (19 with type III; 3 with type IV; 1 each with types II and I; 2 with undefined subtypes) were radiologically evaluated. Lateral flexion-extension views of the cervical spine in each patient were read in blinded fashion by a radiologist and compared to unblinded interpretations made by a separate radiologist. Measurement was taken from the most inferior aspect of the anterior arch of C1 to the dens along a horizontal plane. The remainder of the radiograph was examined for abnormalities.
RESULTS: Two patients with EDS type IV had radiographic evidence of atlantoaxial subluxation (p = 0.013 Fisher's exact test). Evidence of horizontal translation between vertebral bodies below C2 was noted in 3 patients. Cervical arthrosis was present in 9.
CONCLUSIONS: Atlantoaxial subluxation may be a more common finding in persons with type IV EDS than previously thought. Examination of the cervical spine radiographically should be considered before administering general anesthesia to these patients.
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Evidence Hub reads this as a cohort study, done in people. Its evidence level is 3 of 5 (Moderate · trial / cohort / secondary analysis). Trials without a large randomized design, cohorts and secondary analyses give moderate evidence.
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