TY - JOUR T1 - 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 AU - Cho, Samuel K. AU - Stoker, Geoffrey E. AU - Bridwell, Keith H. Y1 - 2011/08/03 N1 - 10.2106/JBJS.J.01659 JO - The Journal of Bone & Joint Surgery SP - e86 1 EP - 8 VL - 93 IS - 15 N2 - 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. SN - 0021-9355 M3 - doi: 10.2106/JBJS.J.01659 UR - http://dx.doi.org/10.2106/JBJS.J.01659 ER -