白化病并发视网膜脱离1例报道  

Albinism complicated with retinal detachment:a case report

作　　者：李婷婷 沙倩 LI Ting-ting;SHA Qian(Department of Ophthalmology,Shanghai Pudong Hospital-Fudan University Pudong Medical Center,Shanghai 201399,China)

机构地区：[1]上海市浦东医院-复旦大学附属浦东医院眼科,上海201399

出　　处：《复旦学报（医学版）》2022年第5期824-827,共4页Fudan University Journal of Medical Sciences

基　　金：上海市浦东医院院级人才培养项目(PY201901);复旦张江临床医学创新基金(KP7202107)。

摘　　要：眼皮肤白化病(oculocutaneous albinism,OCA)患者由于虹膜、脉络膜等缺乏黑色素,一旦发生视网膜脱离,诊断和手术干预极其困难。OCA患者眼底黑色素缺失,“红色眼底”缺乏对比度,视网膜组织难以辨认,无论是眼底检查还是手术操作都非常困难。术后视网膜愈合时间长,处理并发症需要非常谨慎。本文对1例白化病患者孔源性视网膜脱离的发病、诊断及治疗过程进行报道。患者经过治疗,视网膜成功复位,随访3个月无复发。因为发生继发性青光眼,视神经损害,预后视力差。本例提示,对于OCA患者的视网膜脱离,尽管通过手术使视网膜成功复位,但预后视力仍不佳,因此需要继续探索更为有效的诊治策略。For the lack of pigment in oculocutaneous albinism(OCA),especially in the iris and choroid,the diagnosis and surgical intervention of retinal detachment in albinism patients is extremely difficult.Because of the loss of melanin in the fundus of OCA patients,the“red fundus”lacks contrast,and the retinal tissue is difficult to recognize,both fundus examination and surgical operation are very difficult.Due to the long time of postoperative retinal healing,the management of complications also needs to be very careful.This report introduces the pathogenesis,diagnosis and treatment of rhegmatogenous retinal detachment in an albinism patient.With treatment,the patient’s retina was successfully reattached and there was no recurrence during a 3-month follow-up.However,due to secondary glaucoma optic nerve damage,the patient ended up with a poor prognosis.The patient was eventually assessed for disability.This case suggests that the therapeutic effect of retinal detachment in albinism patients is still not satisfactory despite the most careful surgical treatment.In the future,clinicians should continue to explore more effective diagnosis and treatment strategies.

关 键 词：眼皮肤白化病(OCA) 黑色素缺失 视网膜脱离 手术 继发性青光眼 

分 类 号：R774.12[医药卫生—眼科] R758.54[医药卫生—临床医学]