Enchondromatosis and hemangioma (Maffucci's syndrome) with orbital involvement.

Abstract

Maffucci's syndrome is a rare, congenital disease of unknown cause characterized by the development of multiple enchondromas and soft-tissue hemangiomas. We treated a 34-year-old man with Maffucci's syndrome, bilateral proptosis secondary to multiple intraorbital hemangiomas, corneal exposure secondary to a left facial nerve palsy, and multiple intra-abdominal tumors. The skeletal manifestations were not clinically apparent and were only discovered after a careful radiologic survey. Simultaneous bilateral orbital cavernous hemangiomas should alert the physician to the possibility of Maffucci's syndrome.