網膜色素線条に脈絡膜新生血管を合併した家族例

Angioid streaks with choroidal neovascularization in siblings

要約　目的：網膜色素線条がある同胞3名の両眼に生じた脈絡膜新生血管（CNV）の経過の報告。症例：症例は59歳女性，55歳男性，63歳女性で，3名は同胞である。母は生前に弾性線維性仮性黄色腫（PXE）と診断されていた。所見：症例1にはPXEと，両眼に網膜色素線条とCNVがあった。11年間の経過観察中に，右眼に光線力学的療法を行い，左眼のCNVは瘢痕化した。最終視力は0.3と0.07である。症例2では5年間の期間中に左眼のCNVが自然に瘢痕化した。右眼には出血を伴うCNVが発症し，5回のベバシズマブ硝子体注射を行った。最終視力は0.4と0.3である。症例3では4年間の期間中に，右眼のCNVに対し2回のベバシズマブ硝子体注射を行い，左眼のCNVにラニビズマブ硝子体注射を行った。最終視力は0.04と0.4である。結論：網膜色素線条がある同胞3名の両眼にCNVが生じ，抗VEGF薬の硝子体注射などで瘢痕化した。

Abstract.　Purpose：To report three siblings who showed angioid streaks with choroidal neovascularization（CNV）in both eyes. Cases：The cases comprised a 59-year-old female, 55-year-old male, and 63-year-old female when seen initially. The three cases were siblings. Their mother had reportedly been diagnosed with pseudoxanthoma elasticum（PXE）. Findings：Case 1 had PXE and angioid streaks with CNV in both eyes. The right eye received photodynamic therapy. CNV in the left eye became scar tissue during the follow-up of 11 years. Case 2 had PXE and angioid streaks with CNV in both eyes. The right eye received 5 sessions of intravitreal bevacizumab. Case 3 received intravitreal bevacizumab in the right eye and ranibizumab in the left. Conclusion：CNV developed in both eyes of 3 siblings with angioid streaks. The lesion became scar tissue either spontaneously or after intravitreal injection of anti-VEGF agents.