[关键词]
[摘要]
目的:探讨6例早产儿家族性渗出性玻璃体视网膜病变(FEVR)的临床特征。
方法:收集2018-08/2019-01在上海交通大学附属新华医院眼科就诊的FEVR早产儿6例,均于全身麻醉下行荧光素眼底血管造影(FFA)检查确诊,回顾性分析患儿的病例资料及病情特点。
结果:患儿6例均有明确的早产病史,初始诊断为早产儿视网膜病变(ROP),曾接受过玻璃体腔药物注射治疗,其中2例患儿接受过2次玻璃体腔注射治疗,随访中眼底检查和FFA检查结果显示符合FEVR的特征。全身麻醉下2例患儿FFA检查后行激光光凝治疗; 1例行眼底激光光凝联合玻璃体腔药物注射治疗; 1例给予玻璃体腔药物注射治疗; 2例患儿FFA检查显示视网膜周边无血管区不伴渗漏,随访观察。
结论:临床上早产儿FEVR初始诊断容易误诊为ROP,随访观察视网膜周边持续存在无血管区或病情加重,需行FFA检查明确诊断。FEVR是终身疾病,在婴幼儿期病变迅速发展,早期正确的诊断可使患儿得到正确及时的治疗及遗传咨询建议。
[Key word]
[Abstract]
AIM: To describe the clinical characteristics of 6 premature infants diagnosed as familial exudative vitreoretinopathy(FEVR).
METHODS: From August 2018 to January 2019, the researchers collected six premature cases of FEVR from Xinhua Hospital Affiliated To Shanghai Jiao Tong University School of Medicine. All 6 infants born prematurely had examinations of fundus photography and fluorescein angiograms under anesthesia. Medical history and angiographic features were analyzed retrospectively.
RESULTS: Six infants born prematurely were initially misdiagnosed as retinopathy of prematurity ROP. All underwent injection anti-vascular endothelial growth factor(anti-VEGF)drug into vitreous body cavity subsequently, two of whom were treated with injection anti-VEGF drug into vitreous body cavity twice. Six infants born prematurely had follow-up examinations of fundus photography and fluorescein angiograms with the machine of Retcam digital imaging system under anesthesia, they were eventually diagnosed as FEVR. Then 2 cases were treated with laser photocoagulation, 1 case was treated with injection anti-VEGF drug into vitreous body cavity combined laser photocoagulation, 1 case was treated with injection anti-VEGF drug into vitreous body cavity, 2 cases maintain the follow-up visit.
CONCLUSION: Clinically, premature infants FEVR, tend to be misdiagnosed as ROP initially. If the demarcation line separating the avascular from the vascular retinal regions presents persistent or the condition turns to be worse, more examinations will be required to confirm the diagnosis such as fluorescein angiograms under anesthesia. FEVR is a lifelong disease, its symptoms, if present, typically take a progressive course during childhood and adolescence. Early diagnosis of FEVR is crucial due to its progressive nature and the genetic/familial underpinnings of the condition. The correct identification of those FEVR patients can help them receive timely treatment and genetic counseling for those of child-bearing age.
[中图分类号]
[基金项目]
襄阳市医疗卫生领域科技计划项目(重点项目)(No.2020YL27)