Axial chordoma of the cervical spine managed by a trans-mandibular trans-oral approach

Chordomas are low-grade but locally aggressive malignant neoplasms derived from notochordal remnants. These lesions are best treated by en bloc however this is especially challenging in the upper cervical spine due to the proximity of vital structures. We present a 46 year old male whom presented to our unit complaining of progressive upper cervical axial neck pain aggravated by neck rotation, of 4 months duration. He had no symptoms of myelopathy. Examination revealed high posterior spinal tenderness and resistance to neck rotation however the patient's motor and sensory examinations were normal and he had normal gait. An inter-disciplinary team comprising Orthopedics, Neurosurgery, Maxillofacial surgery and Otorhinolaryngology took the patient for surgery. The first stage of the procedure comprised Otorhinolaryngology performing a tracheostomy and thereafter a combined Orthopedic Neurosurgical procedure involving a posterior instrumented C1-C3/C4 fusion and biopsy of the lesion. The second stage involved the maxillofacial surgeons dividing the patient's mandible to increase operative exposure. Thereafter the Orthopedic and Neurosurgical surgeons proceeded to perform a transmandibular transoral en bloc resection of the body and odontoid process of C2 which was replaced by a cage construct. The vertebral arteries were preserved and no dural breech occurred. Post operatively the patient showed no new neurology and was ambulant. He was maintained in a Philadelphia collar for 6 weeks until his fusion was complete. In conclusion this difficult case was managed successfully by thorough pre-operative planning and inter-departmental co-operation. Fortunately the lesion was isolated to the body and odontoid process of C2 which was in the patients favor lending the case towards a favorable outcome.