Neurosis fibromatosis (von Recklinghausen's disease) of the vertebral column.

Although the occurrence of bone changes in von Recklinghausen's disease is uncommon, the incidence is difficult to determine, as mild cases are less likely to seek advice, particularly if the disease is already in the family. Holt and Wright (1948) thought it was greater than a previously estimated figure of 7%. Of the bony deformities, scoliosis appears to be the commonest. Weiss (1921) described 15 cases of neurofibromatosis, all of whom had scoliosis. Brooks and Lehman (1924) studied seven cases with bone changes and found that all had scoliosis. They suggested that the lesion began with a neurofibroma of the periosteal nerve, and that this set up a reaction in the vertebra with bone destruction and regeneration. If actively bone-forming periosteum covers the tumour, a thin shell of bone forms over it and gives a cystic appearance. Weber (1930) described further cases and discussed various aspects of neurofibromatosis. Hagelstam (1946) reviewed the literature and collected 37 cases with deformity of the spine: two were in the cervical region, 20 in the upper and mid-dorsal regions and 15 in the dorsolumbar spines. Holt and Wright (1948) reviewed 127 cases of neurofibromatosis of which 29% showed skeletal involvement. The bone lesions consisted of erosive defects, scoliosis, growth disorders, bowing and pseudoarthrosis of the lower leg, intraosseous cystic lesions and associated anomalies. McCarroll (1950) found 19 cases of scoliosis in 43 cases with bone involvement. Below are case reports of five children with neurofibromatosis of the spine.