Forestier's disease associated with a retro-odontoid mass causing cervicomedullary compression

Introduction: Forestier's Disease (FD) is a progressive skeletal disorder characterized by massive anterior longitudinal ligament calcification forming a bridge on the anterior border of the thoracic and subaxial cervical spine. To our knowledge, retro-odontoid masses associated with FD have not been described. Methods: Five patients with FD were treated for retroodontoid masses and cervicomedullary compression. All patients had progressive neurological symptoms due to ventral compression of the cervicomedullary junction. Results: Four patients underwent combined transoral resection of the ligamentous mass followed by an occipitocervical fusion procedure. One patient with circumferential cervicomedullary compression underwent a posterior decompression and occipitocervical fusion. Follow-up ranged from 4 to 19 months and 4 patients had variable neurological improvement. One patient died 3 weeks post-surgery due to pulmonary complications. Conclusions: This previously undescribed entity should be considered for patients with FD presenting with progressive quadriparesis. Transoral decompression followed by posterior fusion is typically warranted.