Liang Licong, Yang Yiliu, Bu Shaochong, Lu Fang
Department of Ophthalmology, West China Hospital of Sichuan University, Chengdu, China.
Front Med (Lausanne). 2021 Dec 21;8:761398. doi: 10.3389/fmed.2021.761398. eCollection 2021.
Incontinentia pigmenti (IP) is a rare X-linked neuroectodermal dysplasia affecting multiple organs. One of its most significant ophthalmic manifestations is retinal neovascularization due to retinal ischemia, which has been traditionally treated with laser photocoagulation or cryotherapy. The application of anti-vascular endothelial growth factor (VEGF) has been reported for the treatment of retinopathy of IP with beneficial results. However, clinicians should be aware of the possible ocular and systemic side effects of the intravitreal injection of anti-VEGF agents. A 4-month-old female infant with IP was treated with intravitreal injection of conbercept in both eyes. However, cotton-wool spots were noticed in the left eye 1 week after the injection. Laser photocoagulation was performed as an adjunct treatment. The cotton-wool spots were absorbed 1 month after the first intravitreal injection and have eventually disappeared. The cotton-wool spots, after intravitreal injection of conbercept for the treatment of IP, indicated severe retinal ischemia resulting from the neutralization of excessive VEGF, which was shown on fundus photograph and fluorescent angiography. Anti-VEGF agents could cause retinal arteriolar vasoconstriction and artery occlusion on rare occasions. The administration of anti-VEGF agents in pediatric cases with severe neovascularization and retinal ischemia should be carefully considered.
色素失禁症(IP)是一种罕见的X连锁神经外胚层发育不良,可累及多个器官。其最显著的眼部表现之一是由于视网膜缺血导致的视网膜新生血管形成,传统上采用激光光凝或冷冻疗法进行治疗。已有报道应用抗血管内皮生长因子(VEGF)治疗IP相关性视网膜病变并取得了良好效果。然而,临床医生应意识到玻璃体内注射抗VEGF药物可能产生的眼部和全身副作用。一名4个月大的IP女婴接受了双眼玻璃体内注射康柏西普治疗。然而,注射后1周在左眼发现了棉絮斑。作为辅助治疗进行了激光光凝。首次玻璃体内注射后1个月棉絮斑吸收,最终消失。玻璃体内注射康柏西普治疗IP后出现的棉絮斑,在眼底照片和荧光血管造影上显示是由于过量VEGF被中和导致严重视网膜缺血所致。抗VEGF药物在极少数情况下可引起视网膜小动脉血管收缩和动脉阻塞。对于患有严重新生血管形成和视网膜缺血的儿科病例,使用抗VEGF药物应谨慎考虑。