Carr R E, Noble K G
Trans Am Ophthalmol Soc. 1977;75:255-71.
A family with pseudoinflammatory macular dystrophy (PMD) is presented. This dominantly inherited macular dystrophy has its onset in the 3rd to 5th decades with the earliest manifestation being a macular subretinal neovascular network. Visual function tests (ERG, EOG, visual fields, retinal sensitivity) in the early and late stages indicates this is local or geographic disease. This dystrophy should be differentiated from other hereditary causes for subretinal neovascularization (angioid streaks, vitelliform dystrophy, dominant drusen of Bruch's membrane, optic nerve drusen and myopia). It is suggested that treatment be directed at early obliteration of the subretinal neovascularization with intense photocoagulation since the outcome in virtually all cases of untreated PMD is legal blindness.
本文报告了一个患有假性炎症性黄斑营养不良(PMD)的家系。这种常染色体显性遗传的黄斑营养不良在30至50岁发病,最早的表现是黄斑视网膜下新生血管网。早期和晚期的视觉功能测试(视网膜电图、眼电图、视野、视网膜敏感度)表明这是一种局限性或地图状疾病。这种营养不良应与其他导致视网膜下新生血管形成的遗传性病因(血管样条纹、卵黄样营养不良、Bruch膜显性玻璃膜疣、视神经玻璃膜疣和近视)相鉴别。建议采用强激光光凝术早期封闭视网膜下新生血管,因为几乎所有未经治疗的PMD患者最终都会导致法定失明。