PATHOPHYSIOLOGY OF SPINAL DEFORMITIES IN NEUROFIBROMATOSIS - AN ANALYSIS OF 71 PATIENTS WHO HAD CURVES ASSOCIATED WITH DYSTROPHIC CHANGES

The findings in seventy-one patients who had previously untreated spin al deformities associated with dystrophic changes and who had neurofib romatosis were reviewed to identify the risk factors for progression o f the curve as well as the natural history of the dystrophic changes a nd curve patterns. Four different types of curves were evaluated. Two of them had the most severe progression: (1) kyphoscoliosis with angul ar kyphosis (gibbus) and marked dystrophic changes and (2) so-called k yphosing scoliosis (a scoliosis that has so much rotation [90 degrees] that progression is evident only on the lateral roentgenogram) with a round kyphosis. Risk factors for substantial progression of the curve were an early age of onset, a high Cobb angle at the first examinatio n, an abnormal kyphosis, vertebral scalloping, severe rotation at the apex of the curve, location of the apex of the curve in the middle to caudal thoracic area, penciling of one rib or more on the concave side or on both sides of the curve, and penciling of four ribs or more.