Acrodysostosis and spinal canal involvement.

BACKGROUND

Acrodysostosis is a rare syndrome characterized by peripheral dysostosis, nasal hypoplasia, and frequently mental retardation. Only two adult cases of acrodysostosis have been reported to have neurological symptoms.

CASE DESCRIPTION: We report one additional adult case that presented with signs of spinal cord compression from spinal stenosis, and make the first histologic description in the literature of the bony anomalies seen in acrodysostosis. The patient had a T3 to T5 laminectomy and experienced a complete recovery.

CONCLUSIONS

Special attention should be given to these patients to detect signs of spinal stenosis, as early decompression can lead to neurological recovery.