永存原始玻璃体增生症的临床特征及超声影像学表现  

Clinical and ultrasound features of persistent hyperplastic primary vitreous

作　　者：黄景[1] 文晓蓉[1] 张梅[1] 周琛云[1] 何英[1] HUANG Jing;WEN Xiaorong;ZHANG Mei;ZHOU Chenyun;HE Ying(Department of Ultrasound, West China Hospital, Sichuan University, Chengdu 610041, China)

机构地区：[1]四川大学华西医院超声科,四川成都610041

出　　处：《西部医学》2021年第8期1230-1233,1238,共5页Medical Journal of West China

基　　金：四川省科技厅课题(2018SZ0182)。

摘　　要：目的分析永存原始玻璃体增生症(PHPV)的临床特征及超声影像学表现。方法选取2015年1月~2020年6月我院疑诊为PHPV患儿28例(31只患眼)的临床资料,所有患眼均进行二维超声、彩色多普勒血流显像(CDFI)检查,观察患眼晶状体及玻璃体异常回声、血流信号,分析超声对PHPV的诊断价值。结果28例患儿中,男性17例,女性11例;月龄0~10月,平均(5.68±0.64)月;单眼患病25例,双眼患病3例,临床检查发现患眼眼球均有视力障碍,眼轴较短,15例(18患眼)晶状体异常,并发白内障,6例患眼晶状体向前突出,虹膜彭隆,前房浅,5例患眼双眼水平震颤,10例对光反射消失,5例斜视,散瞳后裂隙灯检查均可见患眼晶状体后部混浊,呈乳白色反光,眼压均正常;二维超声所见:眼球短缩、晶状体缩小,扁或显示不清,玻璃体内稍高回声,边界清楚,病变呈倒三角形或条状稍强回声,倒三角底部与晶状体贴近,尖端与视盘相连,CDFI显示玻璃体内条索样回声内查见动脉血流信号,由视乳头向晶状体后延伸,或晶状体后部及玻璃体前部查见稍强回声团,呈漏斗状,前端与睫状体及晶状体相连,后端连接视神经。以手术及散瞳眼底检查结果为评价标准,超声诊断PHPV的灵敏度、特异度、准确度分别为81.48%、75.00%、80.65%,误诊率与漏诊率分别为25.00%与18.52%,Kappa值=0.396。结论PHPV患儿具有一定的临床特征,PHPV的声像图及CDFI影像学表现有助于该病的鉴别诊断,可为临床制定治疗方案提供重要参考依据。Objective To analyze the clinical and ultrasound features of persistent hyperplastic primary vitreous(PHPV).Methods The clinical data of 28 children(31 eyes)with suspected PHPV in the hospital from January 2015 to June 2020 were enrolled.All affected eyes underwent two-dimensional ultrasound and color Doppler flow imaging(CDFI)examination.The lens and abnormal echo of vitreous,and blood flow signals of affected eyes were observed.The diagnostic value of ultrasound for PHPV was analyzed.Results Among the 28 children,there were 17 males and 11 females.Their monthly age was 0-10 months,with average age of(5.68±0.64)months.There were 25 cases with monocular disease and 3 cases with binocular disease.It was found by clinical examination that there was visual impairment and short eye axis.There were 15 cases(18 eyes)with abnormal lens(complicated with cataract),6 cases with lens protruding forward,iris protruding and shallow anterior chamber,5 cases with binocular tremor,10 cases without light reflex and 5 cases with strabismus.After mydriasis,there was back lens opacity by slit-lamp examination,showing milky white reflection,and the intraocular pressure was normal.The two-dimensional ultrasound showed eyeball shortening,reduced,flat or unclear display lens,slightly high echo in vitreous with clear boundary.The lesions showed inverted triangle or strip-like moderately strong echo,the bottom of the inverted triangle was close to the lens,and its tip was connected to the optic disc.CDFI showed arterial blood flow in strip-like echo,which extended from nipple to the back of lens,there was echoic light cluster at the back of lens and front of vitreous,showing funnel shape,and front end was connected to ciliary body and lens,while back end was connected to optic nerve.Taking the results of surgery and mydriatic fundus examination as the evaluation criterion,the sensitivity,specificity,accuracy,misdiagnosis rate,missed diagnosis rate and Kappa value of ultrasound in the diagnosis of PHPV were 81.48%,75.00%,80.65%,25.00%,18.

关 键 词：永存原始玻璃体增生症 临床特征 超声影像学 

分 类 号：R779.7[医药卫生—眼科]