乳頭周囲領域の拡大と陥凹，硝子体中の索状物を認める乳頭部先天異常

Congenital anomalies with enlarged and excavated peripapillary area and intravitreal strand

　視神経乳頭周囲領域の拡大と陥凹および硝子体中の索状物を認める眼先天異常7例8眼について臨床的および電気生理学的検討を行った．乳頭部の所見は朝顔症候群，コロボーマ，乳頭周囲ぶどう腫を呈しており，また従来第一次硝子体過形成遺残と呼ばれている異常に一致する所見が認めらねた．これらは視力不良で小眼球や広範な網膜脈絡膜異常を多く伴い，ERGやVEPも不良であり，網膜剥離の合併が多いことから乳頭部先天異常のなかでもきわめて予後不良な群であると思われた．

We observed unusual congenital anomalies ofthe disc with intravitreal strand in 8 eyes of 7 cases. The strands ran from the disc to the lens and/or the ciliary body. They contained tiny vessels. These findings were compatible with persistent hy-perplastic primary vitreous. The optic disc appeared similar to morning glory syndrome, coloboma and peripapillary staphyloma combined together. All the cases manifested poor visual acuity. They also showed poor responses in electroretinogram and visual evoked potential. The visual prognosis was generally very poor because of microphthalmos, chorioretinal abnormalities and high incidence of retinal detachment as associated features.