Case ReportPolyostotic fibrous dysplasia involving the thoracic spine with myelopathy: case report and review of the literature
Introduction
Fibrous dysplasia is a genetic, noninherited, benign, pathologic condition characterized by the replacement of bone and marrow with poorly organized spicules of immature bone in fibrous connective tissue [1]. Fibrous dysplasia represents approximately 7% of all benign tumor-like bone lesions. The lesions most often present in the long bones of the legs, arms, ribs, and pelvis and in the craniofacial bones. The spine is involved in 1.4% to 5.5% of fibrous dysplasia lesions [2], [3], [4]. Before 2010, a total of 54 monostotic fibrous dysplasia cases involving the spine had been reported in the English-language literature [5].
Polyostotic fibrous dysplasia (PFD) involving the spine is relatively common. However, fibrous dysplasia rarely leads to neurologic deficit and few such cases have been reported. We reviewed the literature to delineate the clinical, radiographic, and pathologic features of PFD with neurologic deficit. We performed a thorough search of PubMed/MEDLINE for the terms “polyostotic fibrous dysplasia,” “spine,” and “neurological deficit.” We reviewed the retrieved abstracts of all articles for relevance. Additional studies were identified by reviewing the references listed in these articles.
Section snippets
Case report
A 38-year-old woman with severe PFD was referred to our hospital with back pain and weakness as well as numbness of the right leg. Her symptoms had progressed over a period of 1 year, and at presentation, she required crutches to walk.
Eighteen years earlier, she had had a pathologic fracture of the right femoral neck. She had undergone curettage and bone grafting at another hospital (Fig. 1), and the histopathologic diagnosis was fibrous dysplasia. At that time, no other lesion was noticed. No
Discussion
Our literature review identified seven cases of PFD involving the spine with myelopathy [6], [7], [8], [9], [10], [11], [12]. Details regarding these cases (and our case) are summarized in the Table. The cases were evenly distributed between men and women and occurred most often in the fourth to sixth decades of life (age range, 35–53 y). Patients most commonly presented with axial neck or back pain with progressive myelopathy. The cause of neurologic deficit was atlantoaxial instability in
Conclusions
In the spine, PFD may lead to pathologic fracture and myelopathy even after adolescence. Vertebroplasty with or without decompression and fixation may be the appropriate option for cases with myelopathy.
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Cited by (7)
Fibrous dysplasia of the spine: A rare cause of lytic lesions and deformity
2020, NeurochirurgieDiagnostic Imaging: Spine
2015, Diagnostic Imaging: SpineDiscontinuous polyostotic fibrous dysplasia with multiple systemic disorders and unique genetic mutations: A case report
2020, World Journal of Clinical CasesKyphoplasty in fibrous dysplasia
2017, Challenging Cases and Complication Management in Pain MedicineMonostotic fibrous dysplasia of the thoracic spine: A case report
2016, Journal of Back and Musculoskeletal Rehabilitation
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Author disclosures: FLW: Nothing to disclose. ZJL: Nothing to disclose. XGL: Nothing to disclose. SMY: Nothing to disclose. LJ: Nothing to disclose. FW: Nothing to disclose. MY: Nothing to disclose.
No funds were received in support of this work. No benefits in any form have been or will be received from a commercial party related directly or indirectly to the subject of this manuscript.