PEDIATRICS Vol. 89 No. 6 June 1992, pp. 1194-1198
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A Longitudinal Study of Atlanto-Dens Relationships in Asymptomatic Individuals with Down Syndrome

Siegfried M. Pueschel MD, PhD, MPH1, Francis H. Scola MD2, and John C. Pezzullo PhD3

1 From the Child Development Center, Department of Pediatrics, Rhode Island Hospital, Brown University Program in Medicine, Providence, Rhode Island
2 From the Child Development Center, Department of Diagnostic Imaging, Rhode Island Hospital, Brown University Program in Medicine, Providence, Rhode Island
3 From the Department of Center for Information Technology Research, Rhode Island Hospital, Brown University Program in Medicine, Providence, Rhode Island

This study was designed to investigate the natural history of atlantoaxial instability in individuals with Down syndrome and to determine whether significant changes in C1-C2 relationship are taking place over time. Although more than 400 patients with Down syndrome who are presently followed at the Child Development Center had cervical spine radiographic examinations in the past, only 141 patients who had serial radiological examinations and whose radiographs were available for reevaluation participated in this study. The results of our investigations revealed that there were only minor changes (1 to 1.5 mm) of atlanto-dens interval measurements over time in 130 (92%) patients with Down syndrome. Eleven patients (8%) had changes of atlanto-dens interval measurements between 2 and 4 mm over time; however, none of these patients had any clinical symptoms. The analyses of data obtained from several subgroups (males and females, various age groups, and patients with and without atlantoaxial instability) did not show any significant changes of atlanto-dens interval measurements of successively obtained radiographs. Our recommendations for and rationale of routine screening for atlantoaxial instability and follow-up examinations are discussed in detail.

Key Words: Down syndrome • atlantoaxial instability • longitudinal study

Submitted on June 6, 1991
Accepted on October 23, 1991




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