Johnson T E, Nasr A M, Nalbandian R M, Cappelen-Smith J
Division of Oculoplastics, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia.
Am J Ophthalmol. 1990 Aug 15;110(2):153-9. doi: 10.1016/s0002-9394(14)76984-3.
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.
马富西综合征是一种病因不明的罕见先天性疾病,其特征为多发性内生软骨瘤和软组织血管瘤的形成。我们治疗了一名患有马富西综合征的34岁男性,他因多发性眶内血管瘤继发双侧眼球突出,因左侧面神经麻痹继发角膜暴露,以及患有多个腹腔内肿瘤。骨骼表现临床并不明显,仅在仔细的放射学检查后才被发现。双侧同时发生的眼眶海绵状血管瘤应提醒医生注意马富西综合征的可能性。