Scoliosis in neurofibromatosis. The natural history with and without operation

Abstract

We reviewed 47 patients with neurofibromatosis and dystrophic spinal deformities; 32 of these patients had been untreated for an average of 3.6 years and in them the natural history was studied. The commonest pattern of deformity at the time of presentation was a short angular thoracic scoliosis, but with progression the angle of kyphosis also increased. Deterioration during childhood was usual but its rate was variable. Severe dystrophic changes in the apical vertebrae and in particular anterior scalloping have a poor prognosis for deterioration. The dystrophic spinal deformity of neurofibromatosis requires early surgical stabilisation which should be by combined anterior and posterior fusion if there is an abnormal angle of kyphosis or severely dystrophic apical vertebrae. Some carefully selected patients can be treated by posterior fusion and instrumentation alone.

Corpectomy and circumferential spinal fusion in dystrophic neurofibromatous curves

“…The malignant behavior of such sharp and short angulated curves may result in major cosmetic, cardiopulmonary, and spinal cord problems [2,3,15].…”

“…Spinal deformity is the most common musculoskeletal manifestation of neurofibromatosis, affecting 10-30% of cases [1][2][3][4]. It is grouped into dystrophic and non-dystrophic curves [2,5,6].…”

Dystrophic kyphoscoliosis in neurofibromatosis type I: A report of two cases and review of the literature

“…Nowadays we recognise two major groups of neurofibromatosis scoliosis: first, a dystrophic (classic) spinal deformity, and, second, a minor form behaving similarly to idiopathic scoliosis [4,10,15]. The dystrophic form is usually a short angular kyphoscoliosis located in the thoracic area [2,3,10]. The term "kyphoscoliosis" is used when the kyphosis dominates the scoliosis or when it exceeds 49 °.…”

“…The term "kyphoscoliosis" is used when the kyphosis dominates the scoliosis or when it exceeds 49 °. This entity is feared for its rapid progression and poor prognosis, especially when there are severe dystrophic changes such as pencilling of ribs, foraminal enlargement, scalloping, rotation and wedging of apical vertebrae [2,4,15]. Subluxation or dislocation with rotatory gliding of vertebrae, as seen in case 1, is a rare though severe complication in neurofibromatosis-scoliosis [9,13,14].…”

“…Often more than one procedure is necessary. A posterior fusion alone is insufficient and leads to failure [2,5,11,15]. A vascularised rib graft augmentation was used in case 1.…”