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 expos...

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Veröffentlicht in:American journal of ophthalmology 1990-08, Vol.110 (2), p.153-159
Hauptverfasser: Johnson, Thomas E., Nasr, Amin M., Nalbandian, Robert M., Cappelen-Smith, Jan
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Sprache:eng
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Zusammenfassung: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
ISSN:0002-9394
1879-1891
DOI:10.1016/S0002-9394(14)76984-3