Neurofibromatosis hyperkyphosis: A review of 33 patients with kyphosis of 80° or greater

Thirty-three patients with hyperkyphosis secondary to neurofibromatosis were reviewed as to the natural history of the deformity, complications of the untreated deformity, and results of treatment. The kyphoses ranged from 80 to 180°, averaging 132°. The natural history was not good, six patients presenting with paraparesis and six with respiratory distress. Twenty-seven patients had operative treatment. Laminectomy was useless for the treatment of paraparesis due to angulated spine deformity. Combined anterior and posterior spine fusion using abundant amounts of autogenous bone for both, coupled with prolonged rigid immobilization, gave the best results. Halo traction and halo casts were invaluable for the more severe deformities. Instrumentation was often impossible due to the severe deformity. All patients operated on by the authors eventually achieved solid union.