Compromise of the spinal canal in Proteus syndrome.

We report on 2 children with spinal stenosis and neurologic sequelae. They illustrate the 2 ways in which spinal compromise may develop in Proteus syndrome--vertebral anomalies or tumor infiltration. In one patient, spinal stenosis resulted from an angular kyphoscoliosis. In the other, cord compression resulted from infiltration of a paraspinal, intrathoracic angiolipoma.