Pitfalls of spinal deformities associated with neurofibromatosis in children.

A study of 116 patients younger than 12 years of age conclusively diagnosed as having neurofibromatosis was undertaken to determine the incidence of significant orthopedic problems. Deformities of the spine comprised the most common skeletal problem. Seventy-four patients (64%) had spinal deformities. Forty-six patients were treated by posterior spinal fusion. Ten required exploration for pseudarthrosis; six were found to have pseudarthrotic defects in the fusion mass. Eight patients had more kyphosis than scoliosis. Only three patients with kyphoscoliosis obtained a solid posterior spinal fusion after multiple surgical procedures. Anteroposterior and lateral roentgenograms of the cervical spine are recommended at the time of initial evaluation of all spinal deformities. Four patients had severe cervical spine deformities, only one of whom was initially identified as having a cervical spine disorder while under treatment for scoliosis. Three of these patients were seen by other surgical services for neck masses. Following removal of posterior elements, the osseous structures were unstable. Only one patient developed spondylolisthesis. Because of the exceedingly high incidence of pseudarthrosis and spinal instability following attempts at spinal fusion, certain guidelines have evolved for the management of these deformities. High-volume computed tomographic myelography in the prone, lateral, and supine positions or magnetic resonance imaging should be performed on all patients prior to surgical treatment. Anterior disc excision and bone graft followed by posterior arthrodesis with instrumentation are indicated if the kyphotic angle is greater than 50 degrees or if scoliosis is greater than 80 degrees. Even combined anterior and posterior arthrodesis operations did not guarantee successful permanent spinal stability in young patients with neurofibromatosis.