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先天性视网膜脱离综合征

Congenital retinal disinsertion syndrome.

作者信息

Boniuk M, Hittner H M

出版信息

Trans Sect Ophthalmol Am Acad Ophthalmol Otolaryngol. 1975 Nov-Dec;79(6):OP827-34.

PMID:813347
Abstract

The congenital retinal disinsertion (CRD) syndrome refers to cases of retinal detachment with disinsertion and may be divided into two groups. Group 1 reported by Hovland and co-workers includes healthy children with bilateral detachments and giant tears nasally, lens coloboma, and center anterior and posterior cortical lens opacities. Group 2 includes healthy children with a unilateral detachment often associated with microphthalmos and catatract. The opposite eye may show a combination of changes including small central anterior and posterior cortical lens opacities, lens colobomas, and paving-stone degeneration. The patients may have an increased risk of developing a detachment in the second eye, and some of these eyes have been treated with cryopexy. Seven patients belonging to group 2 have been described and four of these had unilateral cataract and microphthalmos. Two other patients had some degree of unilateral microphthalmos. Small central anterior or posterior cortical lens opacities were found in five eyes, and lens colobomas were found in two eyes. Six out of seven patients had varying degrees of paving-stone degeneration temporally in the nondetached eye. The CRD syndrome was familial in two patients who were sisters. Pathologic studies were done in the eyes of three patients.

摘要

先天性视网膜脱离(CRD)综合征指伴有视网膜脱离的病例,可分为两组。霍夫兰德及其同事报告的第1组包括双侧视网膜脱离、鼻侧巨大裂孔、晶状体缺损、晶状体前皮质和后皮质中央混浊的健康儿童。第2组包括单侧视网膜脱离且常伴有小眼症和白内障的健康儿童。对侧眼可能表现出多种变化,包括晶状体前皮质和后皮质中央小混浊、晶状体缺损以及铺路石样变性。这些患者患对侧眼视网膜脱离的风险可能增加,其中一些眼睛已接受冷冻治疗。已描述了7例属于第2组的患者,其中4例有单侧白内障和小眼症。另外2例患者有一定程度的单侧小眼症。5只眼发现有晶状体前皮质或后皮质中央小混浊,2只眼发现有晶状体缺损。7例患者中有6例在未发生视网膜脱离的眼颞侧有不同程度的铺路石样变性。该CRD综合征在两名姐妹患者中呈家族性。对3例患者的眼睛进行了病理研究。

相似文献

1
Congenital retinal disinsertion syndrome.先天性视网膜脱离综合征
Trans Sect Ophthalmol Am Acad Ophthalmol Otolaryngol. 1975 Nov-Dec;79(6):OP827-34.
2
Persistent fetal vasculature and minimal fetal vascular remnants: a frequent cause of unilateral congenital cataracts.持续性胎儿血管系统及微小胎儿血管残余:单侧先天性白内障的常见病因
Ophthalmology. 2004 May;111(5):906-13. doi: 10.1016/j.ophtha.2003.07.019.
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[Stickler's syndrome (dystrophia vitreoretinalis hereditaria). Results of surgery for retinal detachment].[斯蒂克勒综合征(遗传性玻璃体视网膜营养不良)。视网膜脱离的手术结果]
Cesk Slov Oftalmol. 2001 May;57(3):147-54.
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Chorioretinal colobomas in a pediatric population.小儿人群中的脉络膜视网膜缺损
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[Vitreoretinal changes in siblings of two patients with Stickler syndrome. Study of two families].[两名Stickler综合征患者兄弟姐妹的玻璃体视网膜改变。两个家族的研究]
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引用本文的文献

1
Congenital lens coloboma and associated pathologies.先天性晶状体缺损及相关病变。
Doc Ophthalmol. 1993;83(4):313-22. doi: 10.1007/BF01204333.