Neurosurgical management of symptomatic thoracic spinal ossification in a patient with fibrodysplasia ossificans progressiva.

TitleNeurosurgical management of symptomatic thoracic spinal ossification in a patient with fibrodysplasia ossificans progressiva.
Publication TypeJournal Article
Year of Publication2012
AuthorsGrobelny BT, Rubin D, Fleischut P, Rubens E, Mack PFogarty, Fink M, Placantonakis DG, Elowitz EH
JournalJ Neurosurg Spine
Volume16
Issue3
Pagination285-8
Date Published2012 Mar
ISSN1547-5646
KeywordsAdult, Diagnosis, Differential, Humans, Magnetic Resonance Imaging, Male, Myositis Ossificans, Radiography, Thoracic, Thoracic Vertebrae, Tomography, X-Ray Computed
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.

DOI10.3171/2011.11.SPINE1164
Alternate JournalJ Neurosurg Spine
PubMed ID22176432