• Terence Verla, Marc Prablek, Alexander E Ropper, David S Xu, and Michael Raber.
• Department of Neurosurgery, Baylor College of Medicine, Houston, Texas, USA. Electronic address: verla@bcm.edu.
• World Neurosurg. 2020 Nov 1; 143: 18-22.

BackgroundPatients with Klippel-Feil syndrome may present with neurologic complaints such as neck pain, radiculopathy and gait instability. Here we describe surgical management of a patient with congenital fusion of the occipital-cervical region and also block circumferential fusion of dens to T3 with spinal cord compression. This report is the first of its kind with such extensive fusion.Case DescriptionOur patient was a 56 year-old female, who presented with neck pain and tingling in all extremities. On exam, she had a short neck, prominent jaw with extremely limited range of motion in neck and features of myelopathy. CT showed fusion of the dens to T3 vertebrae. Patient underwent sub-occipital craniectomy, C1 laminectomy and Occiput to T5 posterior fixation and fusion with neurologic improvement.ConclusionThis is the first reported case of Klippel-Feil syndrome with fusion of all cervical vertebrae down to T3. We recommend surgery for advanced cases of myelopathy or radiculopathy due to stenosis and spinal instability.Copyright © 2020 Elsevier Inc. All rights reserved.

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