Imai M, Iijima H
Department of Ophthalmology, Yamanashi Medical College, Japan.
Nippon Ganka Gakkai Zasshi. 1990 Nov;94(11):1091-6.
The first Japanese autosomal dominant pedigree of familial retinal arteriolar tortuosity was presented with photographical documentation. The proband, a 7-year-old girl also had typical Coats' disease in her left eye. The fluorescein fundus angiogram revealed retinal telangiectasis and microaneurysms with dye leakage in her left peripheral eyeground and marked arteriolar tortuosity without dye leakage in posterior portions of both eyegrounds. Her father showed prominent retinal arteriolar tortuosity bilaterally, and her sister and paternal grandmother showed mild tortuosity of retinal arterioles in both eyes. The recently proposed hypothesis that the familial retinal arteriolar tortuosity is caused by the retinal microcirculatory disturbance consisting of increased resistance in the retinal capillary bed might explain the coincidental occurrence of retinal arteriolar tortuosity and Coat's disease in this report.
首次展示了伴有照片记录的日本常染色体显性遗传的家族性视网膜小动脉迂曲谱系。先证者是一名7岁女孩,其左眼还患有典型的科茨病。荧光素眼底血管造影显示,其左眼周边眼底有视网膜毛细血管扩张和微动脉瘤,伴有染料渗漏,而双眼后部有明显的小动脉迂曲但无染料渗漏。她的父亲双眼有明显的视网膜小动脉迂曲,她的姐姐和祖母双眼有轻度的视网膜小动脉迂曲。最近提出的假说认为,家族性视网膜小动脉迂曲是由视网膜微血管循环障碍引起的,这种障碍表现为视网膜毛细血管床阻力增加,这可能解释了本报告中视网膜小动脉迂曲与科茨病同时出现的现象。