Tessitore Enrico, Luzi Michele, Lobrinus Johannes A, Samaras Nicolaos, Trombetti Andrea
Neurosurgical Service, Department of Clinical Neurosciences, Geneva University Hospital, Geneva, Switzerland.
Spine (Phila Pa 1976). 2008 Feb 1;33(3):E85-9. doi: 10.1097/BRS.0b013e3181624bad.
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.
病例报告。
描述一例累及枢椎、腰椎和跟骨的多骨型佩吉特骨病（PDB）病例。
PDB可累及颈椎。这可导致颈髓受压和颈椎病。PDB患者中很少描述有颅颈不稳。
我们报告了一例65岁女性多骨型PDB病例，累及枢椎、腰椎和跟骨。C2椎体病变因病理性齿状突骨折导致寰枢椎不稳，并导致颈髓进行性受压。患者接受后路手术，实现脊髓减压、C0-C4固定和病理组织取样。病理检查确诊为佩吉特病。开始双膦酸盐治疗。影像学随访显示颅颈排列良好，枢椎病变无变化。
即使佩吉特病性椎管狭窄症状的治疗应以药物治疗开始，但有时为治疗因脊柱不稳导致的神经受压可更早进行手术，预后良好。
