Prevalence of scoliosis in neurofibromatosis

Experience in a comprehensive, multispecialty neurofibromatosis clinic leads the authors to suggest that the association of spinal deformity with neurofibromatosis is less frequent than usually assumed. Previously reported statistics may be partly attributed to preselection of patients seen by spinal surgeons, and to the predominant referral of patients having severe manifestations of neurofibromatosis. A diagnosis of neurofibromatosis 1, based on National Institutes of Health criteria, is confirmed for two hundred twenty patients at the clinic. Twenty-three of these patients have structural scoliosis. Nine patients have idiopathic type curves, eight have dystrophic scoliosis, four have dystrophic kyphoscoliosis, and two have dystrophic lordoscoliosis. The authors believe that 10% is representative of the true prevalence of spinal deformity in an otherwise unselected cross section of neurofibromatosis patients.