Instability of the Craniovertebral Junction and Treatment Outcomes in Patients with Down's Syndrome

Derek A. Taggard, MD
Arnold H. Menezes, MD
Timothy C. Ryken, MD (Iowa City, IA)

Operative intervention for instability at the craniovertebral junction in patients with Down's syndrome has become controversial with reports of high surgical complication rates and suggestions that the incidence of neurologic abnormality associated with this abnormal motion may be low. In this report, the clinical and radiographic findings of 35 patients treated at our institution are described. Common presentations included neck pain (n=15), torticollis (n=12) and myelopathy manifest by hyperreflexia (n=23) or varying degrees of quadriparesis (n=13). Two patients suffered acute neurological insults following routine general anesthetics for minor surgical procedures and another pair following minor falls. Atlantoaxial instability was the most common radiographic abnormality (n=22). Occipitoatlantal instability (n=15) was also frequently observed and was coexistent with atlantoaxial luxation in 14 patients. A rotary component of the atlantoaxial luxation was present in 14 subjects. Seventeen individuals had bony anomalies, the most frequent of which was os odontoideum (n=12). Twenty–four patients underwent operative intervention with successful fusion achieved in 23. Six of 11 patients with basilar invagination were reduced with preoperative traction and thus avoided ventral decompressive procedures – the remaining five subjects underwent transoral resections of ventral pathology. No patients deteriorated with surgery and 22 patients had excellent or good outcomes. This series highlights the clinicopathologic phenomena of craniovertebral instability in Down's syndrome patients and suggests satisfactory outcomes can be achieved with low surgical morbidity.