• Fraser C Henderson, Peter C Rowe, Malini Narayanan, Robert Rosenbaum, Myles Koby, Kelly Tuchmann, and Clair A Francomano.
• Department Neurosurgery, University of Maryland Capital Region Health Center, Cheverly, Maryland, USA; Departments of Neurosurgery and Radiology, Doctors Community Hospital, Lanham, Maryland, USA; Metropolitan Neurosurgery Group LLC, Silver Spring, Maryland, USA. Electronic address: henderson@fraserhendersonmd.com.
• World Neurosurg. 2021 May 1; 149: e854-e865.

BackgroundThe proclivity to atlantoaxial instability (AAI) has been widely reported for conditions such as rheumatoid arthritis and Down syndrome. Similarly, we have found a higher than expected incidence of AAI in hereditary connective tissue disorders. We demonstrate a strong association of AAI with manifestations of dysautonomia, in particular syncope and lightheadedness, and make preliminary observations as to the salutary effect of surgical stabilization of the atlantoaxial motion segment.MethodsIn an institutional review board-approved retrospective study, 20 subjects (16 women, 4 men) with hereditary connective tissue disorders had AAI diagnosed by computed tomography. Subjects underwent realignment (reduction), stabilization, and fusion of the C1-C2 motion segment. All subjects completed preoperative and postoperative questionnaires in which they were asked about performance, function, and autonomic symptoms, including lightheadedness, presyncope, and syncope.ResultsAll patients with AAI reported lightheadedness, and 15 had refractory syncope or presyncope despite maximal medical management and physical therapy. Postoperatively, subjects reported a statistically significant improvement in lightheadedness (P = 0.003), presyncope (P = 0.006), and syncope (P = 0.03), and in the frequency (P < 0.05) of other symptoms related to autonomic function, such as nausea, exercise intolerance, palpitations, tremors, heat intolerance, gastroesophageal reflux, and sleep apnea.ConclusionsThis study draws attention to the potential for AAI to present with syncope or presyncope that is refractory to medical management, and for surgical stabilization of AAI to lead to improvement of these and other autonomic symptoms.Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.

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