Neuro-ophthalmologic manifestations of Maffucci's syndrome and Ollier's disease

Patients with Oilier's disease (multiple skeletal enchondromas) and Maffucc i's syndrome (multiple enchondromas associated with subcutaneous hemangioma s) may develop skull base chondrosarcomas or low-grade astrocytomas as a de layed consequence of these disorders. We report three patients with Ollier' s disease and Maffucci's syndrome who bad diplopia as the initial manifesta tion of intracranial tumors. Since patients with Maffucci's syndrome and Oi lier's disease are at risk for the delayed development of brain and systemi c neoplasms, neuro-ophthalmologists must be aware of the need for long-term surveillance in patients affected by these conditions.