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婴幼儿白内障合并玻璃体异常的临床特点和病因分析
引用本文:马燕,卢海. 婴幼儿白内障合并玻璃体异常的临床特点和病因分析[J]. 眼科, 2015, 24(2): 103-107. DOI: 10.13281/j.cnki.issn.1004-4469.2015.02.008
作者姓名:马燕  卢海
作者单位:100730.首都医科大学附属北京同仁医院 北京同仁眼科中心 眼科学与视觉科学北京市重点实验室
基金项目:首都临床特色应用研究(Z141107002514029);首都医科大学眼科学院开放课题(201206)
摘    要:目的 探讨婴幼儿白内障合并玻璃体异常的临床特点和病因。设计 回顾性、非对照病例研究。研究对象 84例(114眼)3岁以下眼部彩色多普勒超声检查提示玻璃体异常的婴幼儿白内障患儿。方法 回顾性总结2013年1月至2014年7月在北京同仁医院眼科接受手术治疗的3岁以下合并玻璃体异常的婴幼儿白内障患儿。记录患儿月龄、主诉、体征、超声检查及超声诊断、术后确定诊断、既往史、家族史和母亲孕产史,对婴幼儿白内障合并玻璃体异常的病因和临床特点进行分析。主要指标 月龄、症状、病程、体征、超声表现及超声诊断、确定诊断。结果 在84例(114眼)患儿中,发病月龄0~15个月,平均(2.7±3.0)个月,确诊月龄2~20个月,平均(7.8±4.2)个月。81例(96.4%)为家长发现异常,3例(3.6%)为体检查出异常。主要首发症状为白瞳39例(46.4%),不追物 16例(19.0%),斜视10例(11.9%),双眼不等大7例(8.3%)。白内障表现为全白内障75眼(65.8%),后极性白内障38眼(33.3%),膜性白内障1眼(0.9%),可伴有眼球震颤、斜视、小眼球、浅前房、继发性青光眼、瞳孔残膜、牵拉性视网膜脱离、脉络膜缺损等。超声诊断为永存原始玻璃体增生症(PHPV)111眼(97.4%),视网膜脱离 3眼(2.6%)。术后明确病因为 PHPV 76眼(66.7%),家族性渗出性玻璃体视网膜病变14眼(12.3%),先天性白内障24眼(21.0%)。69.3%患眼超声诊断与术后诊断一致。结论 婴幼儿白内障合并玻璃体异常患儿就诊时月龄较大,多以家长发现白瞳就诊,可伴有多种眼部异常,术前眼部彩色多普勒超声检查有助于发现玻璃体视网膜病变。(眼科,2015, 24: 103 -107)

关 键 词:先天性白内障  永存原始玻璃体增生症  家族性渗出性玻璃体视网膜病变  
收稿时间:2014-08-22

Etiology and clinical characteristics of infantile cataract combined with vitreous abnormities
MA Yan;LU Hai. Etiology and clinical characteristics of infantile cataract combined with vitreous abnormities[J]. Ophthalmology in China, 2015, 24(2): 103-107. DOI: 10.13281/j.cnki.issn.1004-4469.2015.02.008
Authors:MA Yan  LU Hai
Affiliation:Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Key Laboratory of Ophthalmology and Visual Sciences, Beijing 100730, China
Abstract:Objective To investigate clinical characteristics and etiology of infantile cataract combined with vitreous abnormalities. Design Retrospective and non-controlled case series. Participants From January 2013 to July 2014 at Beijing Tongren Eye Center eighty-four children (114 eyes) aged 3-year old or younger who were diagnosed as cataract and detected vitreous abnormality by ophthalmic color Doppler flow imaging (CDFI) ultrasound were enrolled. Methods Charts of these patients were reviewed. Age, chief complaints, signs, CDFI examination and suggested diagnosis, proven diagnosis, past history, family history, and pregnancy history of their mothers were recorded. Etiology and clinical characteristics were analyzed. Main Outcome Measures Age, symptoms, course, signs, CDFI manifestations and suggested diagnosis, proven diagnosis. Results Age at presentation varied from 0 to15 months (2.7±3.0 months). Age at diagnosis was 2~20 months (7.8±4.2 months). Abnormalities were noticed by parents in 81 cases (96.4%) and detected by eye examinations in 3 cases (3.6%). The first noticed symptoms were leukocoria in 39 cases (46.4%), unable to follow objects in 16 cases (19.0%), strabismus in 10 cases (11.9%), and binocular asymmetry in 7 cases (8.3%). The cataract was complete type in 75 eyes (65.8%), posterior polar type in 38 eyes (33.3%), and membranous type in 1 eye (0.9%). They could complicate with nystagmus, strabismus, microphthalmia, shallow anterior chamber, secondary glaucoma, persistent pupillary membrane, tractional retinal detachment and coloboma of choroid. CDFI suggested diagnosis included persistent hyperplastic primary vitreous (PHPV) in 111 eyes (97.4%), retinal detachment in 3 eyes (2.6%). Confirmed diagnosis after surgery included PHPV in 76 eyes (66.7%), family exudative vitreoretinopathy (FEVR) in 14 eyes (12.3%) and congenital cataract in 24 eyes (21.0%). CDFI suggested diagnosis and post-operative confirmed diagnosis were consistent in 69.3% cases. Conclusion Patients who were diagnosed as infantile cataract presumptively with abnormal vitreous were mostly at large month-old and mostly noticed as leukocoria by their parents, which could complicate with a variety of ocular abnormality. Pre-operative CDFI examination could detect vitreous and retinal disorders. (Ophthalmol CHN, 2015, 24: 103-107)
Keywords:congenital cataract  persistent hyperplastic primary vitreous  family exudative vitreoretinopathy  
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