title: Neurosurgical management of symptomatic thoracic spinal ossification in a patient with fibrodysplasia ossificans progressiva.
abstract: Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by heterotopic ossification of soft connective and muscle tissues, often as the result of minor trauma. The sequelae include joint fusion, accumulation of calcified foci within soft tissues, thoracic insufficiency syndrome, and progressive immobility. The authors report on a patient with FOP who developed severe spinal canal stenosis in the thoracic spine causing substantial myelopathy. He underwent a thoracic laminectomy and resection of a large posterior osteophyte. Unique considerations are required in treating patients with FOP, including steroid administration to prevent ossification and anesthetic technique. The nuances of neurosurgical and medical management as they pertain to this disease are discussed.
authors.affiliation: Department of Neurosurgery, New York University School of Medicine, New York, New York, USA.
authors.forename: Bartosz T
authors.initials: BT
authors.lastname: Grobelny
authors.forename: David
authors.initials: D
authors.lastname: Rubin
authors.forename: Peter
authors.initials: P
authors.lastname: Fleischut
authors.forename: Elayna
authors.initials: E
authors.lastname: Rubens
authors.forename: Patricia Fogarty
authors.initials: PF
authors.lastname: Mack
authors.forename: Matthew
authors.initials: M
authors.lastname: Fink
authors.forename: Dimitris G
authors.initials: DG
authors.lastname: Placantonakis
authors.forename: Eric H
authors.initials: EH
authors.lastname: Elowitz
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009221:Myositis Ossificans / Q000175:diagnosis / Q000601:surgery*; D013902:Radiography, Thoracic; D013904:Thoracic Vertebrae / Q000601:surgery*; D014057:Tomography, X-Ray Computed