1. Previous reports of generalized neurofibromatosis (von Recklinghausen's disease) associated with paraplegia are reviewed and eight additional cases are presented and discussed.
2. Paraplegia in these cases may result from spinal deformity (kyphoscoliosis, subluxation, or dislocation), tumor (neurofibroma, meningioma, or fibrofatty tissue), or a combination of tumor and deformity.
3. Myelography should be carried out in all neurofibromatosis patients with paraplegia to determine the site and cause of spinal cord compression.
4. Early spine stabilization should be considered when the potential for paraplegia exists.
5. Laminectomy may increase instability and contribute to paraplegia. When decompression is performed, stabilization of the spine by fusion is indicated as part of the treatment plan.
6. Anterior cervical-spine fusion may afford the best means of stabilization if the destruction is extensive, if laminectomy has already been performed, or if later decompression may be necessary.
From the Newington Children's Hospital, Newington, and Hartford Hospital, Hartford