The Journal of Bone and Joint Surgery, Vol 58, Issue 5 695-702, Copyright © 1976 by Journal of Bone and Joint Surgery, Inc
Neurofibromatous scoliosis. Natural history and results of treatment in thirty-seven cases
JH Chaglassian, EJ Riseborough and JE Hall
Of 400 patients with the diagnosis of neurofibromatosis on their hospital
records, 141 actually had the disease. The presence of at least two of the
following features was considered diagnostic: positive family history;
positive biopsy; a minimum of six cafe-au-lait spots, each with a diameter
of at least 1.5 centimeters; and multiple subcutaneous neurofibromas.
Scoliosis was present in thirty-seven patients (26 per cent), most commonly
associated with cafe-au-lait spots (thirty-five patients). In many of the
patients with scoliosis there were associated medical and surgical
problems. Although no standard pattern of spinal deformity could be
identified, a sharp single right thoracic curve involving more than five
vertebrae was the most common. For the whole group the initial measurement
of the scoliosis averaged 42 degrees. Double curves were more sever, buth
kyphosis was uncommon and no cases of paraplegia were recorded. In patients
with progressive scoliosis, the best results were obtained with early
Harrington instrumentation and posterior spine fusion. Progression of the
scoliosis was observed both before treatment and postoperatively. The
amount of progression was not necessarily related to the severity of other
manifestations of neurofibromatosis, and was not significantly dependent on
the length of the curve.
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