Congenital Fusion of Dens to T3 Vertebra in Klippel-Feil Syndrome

• Published in: World neurosurgery 2020-11, Vol.143, p.18-22
• Main Authors: Verla, Terence, Prablek, Marc, Ropper, Alexander E., Xu, David S., Raber, Michael
• Format: Article
• Language: English
• Subjects: Atlanto-Occipital Joint - diagnostic imaging; Atlanto-Occipital Joint - surgery; Cervical myelopathy; Cervical Vertebrae - abnormalities; Cervical Vertebrae - diagnostic imaging; Cervical Vertebrae - surgery; Congenital fusion; Craniotomy; Decompression, Surgical; Female; Fused spinal vertebrae; Humans; Joint Instability - diagnostic imaging; Joint Instability - surgery; Klippel-Feil syndrome; Klippel-Feil Syndrome - complications; Klippel-Feil Syndrome - diagnostic imaging; Klippel-Feil Syndrome - physiopathology; Klippel-Feil Syndrome - surgery; Laminectomy; Magnetic Resonance Imaging; Middle Aged; Neurosurgical Procedures; Occipital Bone - abnormalities; Occipital Bone - diagnostic imaging; Occipital Bone - surgery; Occipital-cervical fusion; Odontoid Process - abnormalities; Odontoid Process - diagnostic imaging; Odontoid Process - surgery; Posterior cervical fusion; Spinal Fusion; Spinal Stenosis - diagnostic imaging; Spinal Stenosis - etiology; Spinal Stenosis - physiopathology; Spinal Stenosis - surgery; Thoracic Vertebrae - abnormalities; Thoracic Vertebrae - diagnostic imaging; Thoracic Vertebrae - surgery; Tomography, X-Ray Computed
• ISSN: 1878-8750; 1878-8769
• DOI: 10.1016/j.wneu.2020.06.232

Patients 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. Our 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. This 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.