Enchondromatosis and hemangioma (Maffucci's syndrome) with orbital involvement.
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.
Johnson
T E
TE
Division of Oculoplastics, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia.
Nasr
A M
AM
Nalbandian
R M
RM
Cappelen-Smith
J
J
D000328:Adult; D001763:Blepharoptosis / Q000175:diagnosis / Q000209:etiology; D004687:Enchondromatosis / Q000150:complications* / Q000175:diagnosis / Q000000981:diagnostic imaging; D006225:Hand / Q000000981:diagnostic imaging; D006392:Hemangioma, Cavernous / Q000150:complications* / Q000175:diagnosis / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D009918:Orbital Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000000981:diagnostic imaging; D010009:Osteochondrodysplasias / Q000150:complications*; D012886:Skull / Q000000981:diagnostic imaging; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D014792:Visual Acuity