Patients with type IV Ehlers-Danlos syndrome may be predisposed to atlantoaxial subluxation.
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.
Halko
G J
GJ
Division of Rheumatic Diseases and Radiology, University of Connecticut Health Center, Farmington 06030-1310, USA.
Cobb
R
R
Abeles
M
M
D000328:Adult; D001268:Atlanto-Axial Joint / Q000293:injuries*; D002574:Cervical Vertebrae; D015331:Cohort Studies; D004198:Disease Susceptibility; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000000981:diagnostic imaging; D005260:Female; D006801:Humans; D004204:Joint Dislocations / Q000453:epidemiology / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011859:Radiography