A case of myelopathy because of enchondromas from Maffucci syndrome with successful surgical treatment.

BACKGROUND CONTEXT

Maffucci syndrome is a rare disorder comprising multiple enchondromas associated with multiple hemangiomas. Less than 200 cases have been reported in the literature. Most reported cases comprised lesions of the long bone metaphyses, hands, and feet. No previous case of myelopathy due to enchondroma from Maffucci syndrome has been reported.

PURPOSE

To highlight an interesting and rare presentation of thoracic myelopathy because of enchondromas from Maffucci syndrome.

STUDY DESIGN

This is a case report of a single patient presenting with myelopathy in whom enchondromas from Maffucci syndrome were removed from the spinal canal with the return of normal function.

METHODS

Clinical examination, magnetic resonance imaging, surgical resection, and histologic analysis.

RESULTS

The patient experiencing myelopathy symptoms underwent a surgical resection of the offending lesions that resulted in return to normal function.

CONCLUSIONS

Maffucci syndrome is a rare condition; however, in affected patients the possibility of structural spinal abnormalities causing cord compression must be considered, as discrete surgical resection can result in a good outcome.