| 色素血管性斑痣性错构瘤及其研究进展 |
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| 引用本文： | 黄士通,杨扬帆,余敏斌.色素血管性斑痣性错构瘤及其研究进展[J].国际眼科纵览,2021,45(4):300-306. |
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| 作者姓名： | 黄士通 杨扬帆 余敏斌 |
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| 作者单位： | 中山大学中山眼科中心 眼科学国家重点实验室，广州 510060 |
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| 摘 要： | 色素血管性斑痣性错构瘤(phacomatosis pigmentovascularis，PPV)是以全身皮肤弥漫血管畸形合并全身皮肤弥漫色素增多为特征的先天性综合征，病变还可累及颅脑、眼部、全身静脉、骨骼等多个器官。PPV发病极为罕见，眼部表现特殊，可表现为青光眼、眼周皮肤鲜红斑、视网膜血管异常、脉络膜血管瘤、眼黑变、...
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| 关 键 词： | 色素血管性斑痣性错构瘤 青光眼 |
| 收稿时间： | 2020-12-15 |
Ocular manifestations and advances of phacomatosis pigmentovascularis |
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| Huang Shitong,Yang Yangfan,Yu Minbin.Ocular manifestations and advances of phacomatosis pigmentovascularis[J].International Review of Opthalmology,2021,45(4):300-306. |
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| Authors: | Huang Shitong Yang Yangfan Yu Minbin |
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| Institution: | State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou 510060, China |
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| Abstract: | Phacomatosis pigmentovascularis (PPV) is a congenital syndrome characterized by diffuse epidermal capillary malformation and pigmented nevus. PPV also have systemic involvement. Ocular manifestations of PPV include glaucoma, periocular erythema, retinal vascular abnormalities, choroidal hemangioma, ocular melanocytosis, iris hererochromia, iris mammillation and fundus hyperpigmentation. Extracutaneous complications of PPV are the key in the treatment. The treatment of its associated glaucoma is challenge and most still refer to the treatment of glaucoma complicated with Sturge-Weber syndrome.(Int Rev Ophthalmol, 2021, 45: 300-306) |
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| Keywords: | phacomatosis pigmentovascularis glaucoma |
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