The Journal of Bone and Joint Surgery, Vol 76, Issue 5 692-700, Copyright © 1994 by Journal of Bone and Joint Surgery, Inc
Pathophysiology of spinal deformities in neurofibromatosis. An analysis of seventy-one patients who had curves associated with dystrophic changes
H Funasaki, RB Winter, JB Lonstein and F Denis
Minnesota Spine Center, Minneapolis 55454.
The findings in seventy-one patients who had previously untreated spinal
deformities associated with dystrophic changes and who had
neurofibromatosis were reviewed to identify the risk factors for
progression of the curve as well as the natural history of the dystrophic
changes and curve patterns. Four different types of curves were evaluated.
Two of them had the most severe progression: (1) kyphoscoliosis with
angular kyphosis (gibbus) and marked dystrophic changes and (2) so-called
kyphosing 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 examination, 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.