Neurofibromatosis in childhood.

A study of 78 children with neurofibromatosis showed that 40% had an autosomal dominant form of inheritence and a wide variety of manifestations which developed at varying stages during childhood. The pattern of these manifestations differed in many respects from the pattern seen in adults with neurofibromatosis. As a result of our inability to predict the future appearance of these manifestations and the difficulty encountered in treating advanced lesions, it is suggested that a policy of early detection and treatment is advisable. This applies particularly to intrathoracic neurofibromas, tumours of the optic nerves, spinal cord and brain, and kyphoscoliosis.