定型的脈絡膜欠損を合併したpit-macular syndromeの1例

A case of pit-macular syndrome with a typical coloboma of the choroid

　定型的脈絡膜欠損を合併したpit-macular syndromeの1例を報告する．

　症例　は16歳男性で，右眼に乳頭陥凹耳側縁の小さなpitと乳頭へ達する黄斑部網膜剥離がみられ，乳頭下方には約2乳頭径大の定型的脈絡膜欠損がみられた．螢光造影にて，乳頭陥凹内のpit部から乳頭耳下側縁にかけて低螢光領域がみられ，pitは深く下掘れしていると考えられた．明らかな螢光漏出像は認められなかった．1週後，乳頭耳側縁に沿ってレーザー光凝固術を施行したが，網膜剥離は軽減するにとどまった．

　視神経乳頭pitの発生には，胎生裂閉鎖不全が密接に関連していると考えられた．また，網膜下液はくも膜下腔よりの髄液に由来すると推測した．

A 16-year-old male presented with recent blurring of vision in his right eye. Funduscopy showed an enlarged optic disc with a tiny pit at the temporal edge of the cup. There was a discrete retinal detachment of the macula toward the optic disc and a typical coloboma of the choroid inferior to the optic disc. He was diagnosed as pit-macular syndrome with a typical coloboma of the choroid. Fluorescein angiography revealed hypo-fluorescence from the pit toward the inferotemporal margin of the optic disc, so the optic disc was thought to be undermined deeply toward the inferotemporal margin. No leakage of dye was observed. One week later, photocoagulation was performed along the tem-poral edge of the optic disc, but was unsuccessful in reattaching the macula. These findings seemed to suggest that the subretinal fluid was derived from the cerebrospinal fluid via the pit. The pit of the optic nervehead was thought to be closely related to incomplete closure of the fetal fissure.

Rinsho Ganka (Jpn J Clin Ophthalmol) 40(12) : 1305-1308, 1986