Symptomatic atlantoaxial subluxation in persons with Down syndrome

In a prospective study 40 of 236 individuals with Down syndrome were found to have atlantoaxial instability. Whereas most of the 40 affected children were asymptomatic, seven patients exhibited various neuropathology. Historical data, neurologic findings, radiologic interpretations, and surgical man...

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Bibliographic Details
Published in:	Journal of pediatric orthopaedics Vol. 4; no. 6; p. 682
Main Authors:	Pueschel, S M, Herndon, J H, Gelch, M M, Senft, K E, Scola, F H, Goldberg, M J
Format:	Journal Article
Language:	English
Published:	United States 01-11-1984
Subjects:	Adolescent Adult Atlanto-Axial Joint - injuries Child Down Syndrome - complications Female Humans Joint Dislocations - diagnosis Joint Dislocations - etiology Joint Dislocations - surgery Joint Instability - diagnosis Joint Instability - etiology Joint Instability - surgery Male Middle Aged Prospective Studies Spinal Fusion
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Summary:	In a prospective study 40 of 236 individuals with Down syndrome were found to have atlantoaxial instability. Whereas most of the 40 affected children were asymptomatic, seven patients exhibited various neuropathology. Historical data, neurologic findings, radiologic interpretations, and surgical management of these seven patients are described in detail. In addition, case reports of other individuals with Down syndrome and symptomatic atlantoaxial subluxation from the literature were analyzed. We conclude that early identification of these patients and appropriate surgical management, including reduction of the subluxation and posterior cervical spine fusion, will in most instances result in a favorable outcome; however, patients with long-standing symptoms and marked neuronal damage show no or only little improvement postoperatively.
ISSN:	0271-6798
DOI:	10.1097/01241398-198411000-00005