Cervical Paget disease of bone with spinal cord compression due to atlanto-axial instability: a case report and review of the literature.

Abstract

Case report. To describe a case of polyostotic Paget disease of bone (PDB), with axis, lumbar, and calcaneum involvement. PDB can involve cervical spine. This can lead to cervical cord compression and cervical myelopathy. A cranio-cervical instability has been rarely described in patients with PDB. We report about the case of a 65-year-old women presenting with a polyostotic PDB, with axis, lumbar, and calcaneum involvement. The C2 vertebral lesion was associated with an atlantoaxial instability due to a pathologic odontoid fracture and was responsible for progressive spinal cervical cord compression. The patient was operated on by a posterior approach allowing spinal cord decompression, C0-C4 stabilization, and pathologic tissue sampling. Pathologic examination confirmed the diagnosis of Paget disease. The bisphosphonate therapy was started. Radiologic follow-up showed a good cranio-cervical alignement and an unchanged axis lesion. Even if the treatment of pagetic spinal stenosis symptoms should start with medical therapy, surgery can sometimes be performed earlier to treat neural compression due to spinal instability, with favorable outcome.