RT Journal
A1 Cho, Samuel K.
A1 Stoker, Geoffrey E.
A1 Bridwell, Keith H.
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
JF The Journal of Bone & Joint Surgery
JO The Journal of Bone & Joint Surgery
YR 2011
FD August 3
VO 93
IS 15
SP e86 1
OP 8
DO 10.2106/JBJS.J.01659
UL http://dx.doi.org/10.2106/JBJS.J.01659
AB 
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.