PEDIATRICS Vol. 80 No. 4 October 1987, pp. 555-560
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Atlantoaxial Instability in Individuals With Down Syndrome: Epidemiologic, Radiographic, and Clinical Studies

Siegfried M. Pueschel MD, PhD, MPH1 and Francis H. Scola MD1

1 From the Child Development Center, Department of Pediatrics, and the Department of Radiology, Rhode Island Hospital, Brown University—Program in Medicine, Providence

Atlantoaxial instability is a relatively frequent finding in individuals with Down syndrome. We examined 404 patients with this chromosome disorder and observed their atlanto-dens intervals and spinal canal widths to be significantly different from children without Down syndrome. Significant differences were also noted between boys and girls with Down syndrome in spinal canal widths but not in atlanto-dens interval measurements. When different neck positions were compared, measurements obtained in flexion were significantly greater than in extension or in neutral position. In addition, more patients had ge5 mm atlanto-dens interval measurements in flexion than in extension or neutral. A total of 59 (14.6%) of 404 patients displayed atlantoaxial instability. Fifty-three (13.1%) patients had asymptomatic atlantoaxial instability, and special precautions will have to be taken with this group of children. Six (1.5%) patients had symptomatic atlantoaxial instability who underwent surgery to prevent further injury to the spinal cord. In our follow-up studies of 95 patients with Down syndrome, we did not find any significant changes during either clinical or radiographic reexaminations.

Key Words: Down syndrome • atlantoaxial instability

Accepted on November 10, 1986




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