Quadriplegia in a 10 year-old boy due to multiple cervical neurofibromas.

STUDY

DESIGN

A case report of a child with quadriplegia as a result of neurofibromatosis type I who had cervical laminectomy for spinal cord decompression followed by occipitocervical instrumentation is described.

OBJECTIVES

To describe the consequences of severe neurofibromatosis type I and an effective surgical technique of occipitocervical instrumentation.

SUMMARY OF BACKGROUND DATA: Neurofibromatosis type I is one of the most commonly inherited genetic disorders in the human population. Extensive intraspinal involvement by neurofibromas can cause significant distortion of normal spinal structure as well spinal cord compression. Extensive laminectomy (with subsequent risk of postsurgical kyphosis) is often required for adequate decompression of the spinal cord.

METHODS

The clinical and radiographic presentation of a child with severe neurofibromatosis type I resulting in quadriplegia is described. The severe neurologic deficit was caused by compression of the spinal cord by intraspinal neurofibromas. Extensive laminectomy was required to adequately decompress the spinal cord. Occipitocervical fusion from the occiput to C6 was done to stabilize the spine and prevent future kyphosis.

RESULTS

Decompression of the spinal cord led to complete neurologic recovery, and instrumentation of the cervical spine was successful in preventing the development of postlaminectomy kyphosis in this pediatric patient.

CONCLUSIONS

The reported case emphasizes the need for treating acute neurologic symptoms caused by spinal cord compression in neurofibromatosis type I as well as addressing the future risk of spinal deformity following laminectomy.