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Spinal Reconstruction with Pedicle Screw-Based Instrumentation and rhBMP-2 in Patients with Neurofibromatosis and Severe Dural Ectasia and Spinal DeformityReport of Two Cases and a Review of the Literature
Samuel K. Cho, MD1; Geoffrey E. Stoker, BS2; Keith H. Bridwell, MD2
1 Department of Orthopaedics, Mount Sinai School of Medicine, 5 East 98th Street, Box 1188, New York, NY 10029
2 Department of Orthopaedic Surgery, Washington University in St. Louis School of Medicine, One Barnes-Jewish Hospital Plaza, Suite 11300 West Pavilion, Campus Box 8233, St. Louis, MO 63110. E-mail address for K.H. Bridwell: bridwellk@wudosis.wustl.edu
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Disclosure: One or more of the authors received payments or services, either directly or indirectly (i.e., via his or her institution), from a third party in support of an aspect of this work. In addition, one or more of the authors, or his or her institution, has had a financial relationship, in the thirty-six months prior to submission of this work, with an entity in the biomedical arena that could be perceived to influence or have the potential to influence what is written in this work. No author has had any other relationships, or has engaged in any other activities, that could be perceived to influence or have the potential to influence what is written in this work. The complete Disclosures of Potential Conflicts of Interest submitted by authors are always provided with the online version of the article.

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Investigation performed at Spine Service, Department of Orthopaedic Surgery, Washington University School of Medicine, and the Shriners Hospital for Children, St. Louis, Missouri

Copyright © 2011 by The Journal of Bone and Joint Surgery, Inc.
J Bone Joint Surg Am, 2011 Aug 03;93(15):e86 1-8. doi: 10.2106/JBJS.J.01659
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Neurofibromatosis is one of the most common genetic disorders, with type-I neurofibromatosis having a global prevalence of one in 3000 individuals1-4. Inherited in an autosomal dominant manner, type-1 neurofibromatosis may be known best for its cutaneous manifestations. Café au lait spots and peripheral neurofibromas arise as a result of unchecked proliferation of neural crest-derived melanocytes and Schwann cells, respectively5,6. These superficial lesions are generally benign and are often considered to be purely a cosmetic issue7. In contrast, the osteopathological manifestations of type-1 neurofibromatosis are of far greater clinical concern. Spinal deformity, particularly kyphoscoliosis of the thoracic spine, is the most common abnormality (present in 10% to 60% of cases)4,8-10.
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