Research
We present a patient with Down syndrome with neck pain and severe cervical myelopathy. Imaging revealed occipito-atlantal and atlanto-axial instability with severe spinal cord compression. There are no standardized radiological or clinical guidelines to aid in managing this unique subset of patients. We demonstrate a successful occiput–C3 internal fixation and fusion without complication. Due to the largely unknown natural history and ongoing management difficulties in this population, we demonstrate a case that may aid future decision making for this specialized field. We also discuss an approach to reduce this deformity, which, to our knowledge, has not been published before.
aPrince of Wales Hospital, Barker Street, Randwick, Sydney, New South Wales, Australia
bRoyal North Shore Hospital, Reserve Road, St Leonards, Sydney, New South Wales, Australia
Abstract
We present a patient with Down syndrome with neck pain and severe cervical myelopathy. Imaging revealed occipito-atlantal and atlanto-axial instability with severe spinal cord compression. There are no standardized radiological or clinical guidelines to aid in managing this unique subset of patients. We demonstrate a successful occiput–C3 internal fixation and fusion without complication. Due to the largely unknown natural history and ongoing management difficulties in this population, we demonstrate a case that may aid future decision making for this specialized field. We also discuss an approach to reduce this deformity, which, to our knowledge, has not been published before.
2012 Published by Elsevier Ltd.
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Keywords
Cranio-cervical fusion Cranio-cervical instability Cranio-vertebral junction Down syndrome
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