Monostotic fibrous dysplasia of the thoracic spine. A case report.

STUDY

DESIGN

This case report details the diagnosis and treatment of a 12-year-old boy with progressive paraparesis from monostotic fibrous dysplasia of the thoracic spine.

OBJECTIVES

The authors discuss their experience in the context of previous reports to recommend the optimal management of this disease.

SUMMARY OF BACKGROUND DATA: Isolated vertebral involvement with fibrous dysplasia is exceedingly rare, with only 15 case reports describing the clinical presentation and treatment of these patients. The various treatments reported have included biopsy, decompression, curettage, and excision with or without fusion. A consensus for management has not been achieved.

METHODS

The patient developed progressive neurologic deterioration from a combination of epidural extension of dysplastic tissue and a severe subluxation with kyphosis. Evaluation included plain radiographs, magnetic resonance imaging, computed tomography-assisted biopsy, and a bone scan. The patient was successfully treated with complete resection of both the bony and soft tissue components of the lesion by a single-stage combined approach involving posterior resection, instrumentation, and fusion to achieve rapid decompression and immediate stabilization followed by anterior resection with strut graft fusion to obtain a complete resection and prevent a delayed progressive kyphotic deformity.

RESULTS

The patient's neurologic symptoms and signs rapidly resolved postoperatively. Graft incorporation with normal alignment was radiographically demonstrated at 3 months and maintained at 12 months. He has no pain or disability at 17 months follow-up.

CONCLUSIONS

The authors recommend radical removal of all involved bone accompanied by internal fixation and bone grafting to achieve long-term stabilization in patients with monostotic fibrous dysplasia of the spine accompanied by neurologic deficits, instability or progressive pain.