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


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


BACKGROUND
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.
Przybylski
G J
GJ
Department of Neurosurgery, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pennsylvania, USA.
Pollack
I F
IF
Ward
W T
WT
D002648:Child; D005358:Fibrous Dysplasia, Monostotic / Q000175:diagnosis* / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D013122:Spinal Diseases / Q000175:diagnosis / Q000601:surgery; D013904:Thoracic Vertebrae / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D014057:Tomography, X-Ray Computed