Title | Neurosurgical management of symptomatic thoracic spinal ossification in a patient with fibrodysplasia ossificans progressiva. |
Publication Type | Journal Article |
Year of Publication | 2012 |
Authors | Grobelny BT, Rubin D, Fleischut P, Rubens E, Mack PFogarty, Fink M, Placantonakis DG, Elowitz EH |
Journal | J Neurosurg Spine |
Volume | 16 |
Issue | 3 |
Pagination | 285-8 |
Date Published | 2012 Mar |
ISSN | 1547-5646 |
Keywords | Adult, 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. |
DOI | 10.3171/2011.11.SPINE1164 |
Alternate Journal | J Neurosurg Spine |
PubMed ID | 22176432 |