Forestier disease associated with a retroodontoid mass causing cervicomedullary compression.

OBJECT

Forestier Disease (FD) is a progressive skeletal disorder affecting predominantly older men. It is also known as diffuse idiopathic skeletal hyperostosis (DISH) and is characterized by massive anterior longitudinal ligament calcification that forms a bridge on the anterior border of the thoracic and subaxial cervical spine. To the authors' knowledge, retroodontoid masses associated with FD have not been described.

METHODS

Five patients with FD and multilevel subaxial cervical fusion were treated for retroodontoid masses and cervicomedullary junction (CMJ) compression. There were four men and one woman (mean age 73 years, range 54-86 years). All patients suffered progressive neurological symptoms resulting from anterior compression of the CMJ. Four patients underwent combined transoral resection of the ligamentous mass followed by an occipitocervical fusion procedure. One patient with circumferential CMJ compression underwent a posterior decompression and occipitocervical fusion. Histopathological examination of the mass showed hypertrophic degenerative fibrocartilage. Early postoperative neurological improvement was noted in all patients. The follow-up period ranged from 4 to 19 months. At the end of the follow-up period, four patients experienced neurological improvement. One patient died 3 weeks postsurgery of pulmonary complications.

CONCLUSIONS

The osseous elements of the occipitoatlantoaxial complex are not directly affected by FD. The ligamentous structures of the odontoid process, however, are exposed to significantly altered biomechanics resulting from fusion of the subaxial cervical spine associated with FD. Stress-induced compensatory ligamentous hypertrophic changes at the craniovertebral junction cause CMJ compression and subsequent neurological deterioration. This previously undescribed entity should be considered in patients with FD or DISH who present with progressive quadriparesis. Transoral decompression and posterior fusion are often needed in patients with large masses and severe progressive neurological deficits. Selected patients with smaller masses and milder neurological symptoms may be treated with posterior fusion alone.