網膜血管症retinal vasculopathyを伴つた網膜色素変性症

Two cases of retinitis pigmentosa associated with retinal vasculopathy

緒　言

　網膜色素変性症が種々の網膜血管病変を伴うことがある。その一つにZamorani^1）（1956）が記載したCoats病との合併がある。その後現在までに同様な症例が報告され^2〜14），網膜色素変性症の眼底に網膜血管の拡張と蛇行，小動脈瘤，新生血管などを伴う滲出性病変をきたし，網膜硝子体出血や滲出性網膜剥離がみられたとのべている。しかし両疾患が合併する機序に関しては未だ明らかでない。

　また網膜色素変性症，遺伝性嚢胞状黄斑部浮腫，cone-rod dystrophyなどretinal dystrophyをきたす疾患群において網膜血管の透過性が亢進し，網膜浮腫を生じることがわかつてきた^{11,15〜18）}。

We observed two cases of retinitis pigmentosa associated with bilateral retinal vasculopathy. Both were males and were 33 and 36 years of age, respectively. One had retinitis pigmentosa sine pigmento and the other had typical retinitis pig-mentosa.

The early stage of retinal vasculopathy was ob-served in three eyes, in which marked capillary dilation, numerous microaneurysms and abnormal A-V shunt vessels were seen in the macular area. Furthermore, preretinal neovascularizations were observed in the posterior and equatorial regions of the retina. Fluorescein angiography revealed dyeleakage from these abnormal vessels.