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蝶形视网膜色素上皮营养不良患者的多模式眼底影像学特征
引用本文:郑志坤,张娟,黎铧,李娟娟,张利伟.蝶形视网膜色素上皮营养不良患者的多模式眼底影像学特征[J].国际眼科杂志,2022,22(5):863-869.
作者姓名:郑志坤  张娟  黎铧  李娟娟  张利伟
作者单位:中国云南省昆明市,云南大学附属医院眼科 云南省第二人民医院眼科 云南省眼科医院 云南省眼科疾病研究重点实验室,中国云南省昆明市,云南大学附属医院眼科 云南省第二人民医院眼科 云南省眼科医院 云南省眼科疾病研究重点实验室,中国云南省昆明市,云南大学附属医院眼科 云南省第二人民医院眼科 云南省眼科医院 云南省眼科疾病研究重点实验室,中国云南省昆明市,云南大学附属医院眼科 云南省第二人民医院眼科 云南省眼科医院 云南省眼科疾病研究重点实验室,中国云南省昆明市,云南大学附属医院眼科 云南省第二人民医院眼科 云南省眼科医院 云南省眼科疾病研究重点实验室
基金项目:国家自然科学基金项目(No.81860171); 云南省卫生健康委员会医学学科带头人培养计划(No.D-2019021); 云南省卫生健康委员会医学后备人才培养计划(No.H-2018020)
摘    要:目的:探讨蝶形视网膜色素上皮营养不良(BPD)患者的多模式眼底影像特征。

方法:回顾分析2016-01/2019-07经多模式影像检查确诊BPD的患者18例36眼的眼底彩照、红外照相、自发荧光、荧光造影、脉络膜造影、光学相干断层扫描(OCT)及眼底血流成像OCT(OCTA)等多模式眼底影像检查。

结果:典型的眼底彩色照相可显示色素异常沉着而呈现的蝶翅样病变外观,病变进展后蝶形外观不明显,而相应区域萎缩; 红外照相显示BPD患者的眼底黄色病灶,表现为白色高亮影像; 自发荧光可有斑片状、点状所勾勒的蝶翅表现,也可有以上特征重叠的表现,当病变进展萎缩明显时,自发荧光不能显示典型的蝶形改变,但可以反映出色素上皮细胞的破坏程度范围; 眼底荧光血管造影和脉络膜造影除了显示蝶形病变外,能更为精准地显示血管病变情况,尤其是脉络膜新生血管(CNV)的检查; OCT显示病变位于视网膜色素上皮(RPE)之上与光感受器之间。随病变进展,色素上皮表现相应扩大的损伤,继发CNV者可见突破RPE的高反射信号; OCTA显示病变对视网膜深层和浅层损伤不明显,但脉络膜血流信号不同程度丢失,可敏感检出CNV的血流影像。

结论:多模式眼底影像技术提供了BPD患者病变发生发展的影像特征,有助于临床医生更加深入地认识该病。

关 键 词:视网膜色素上皮图形状营养不良    蝶形    多模式影像    脂褐质    特征
收稿时间:2021/8/18 0:00:00
修稿时间:2022/4/6 0:00:00

Multimodal imaging features of butterfly-like pattern retinal pigment epithelial dystrophy
Zhi-Kun Zhen,Juan Zhang,Hua Li,Juan-Juan Li and Li-wei Zhang.Multimodal imaging features of butterfly-like pattern retinal pigment epithelial dystrophy[J].International Journal of Ophthalmology,2022,22(5):863-869.
Authors:Zhi-Kun Zhen  Juan Zhang  Hua Li  Juan-Juan Li and Li-wei Zhang
Affiliation:Department of Ophthalmology, Affiliated Hospital of Yunnan University;Department of Ophthalmology, the Second Hospital of Yunnan Province;Yunnan Eye Hospital;Key laboratory of Yunnan Province for Ophthalmic Disease Research, Kunming 650021, Yunnan Province, China,Department of Ophthalmology, Affiliated Hospital of Yunnan University;Department of Ophthalmology, the Second Hospital of Yunnan Province;Yunnan Eye Hospital;Key laboratory of Yunnan Province for Ophthalmic Disease Research, Kunming 650021, Yunnan Province, China,Department of Ophthalmology, Affiliated Hospital of Yunnan University;Department of Ophthalmology, the Second Hospital of Yunnan Province;Yunnan Eye Hospital;Key laboratory of Yunnan Province for Ophthalmic Disease Research, Kunming 650021, Yunnan Province, China,Department of Ophthalmology, Affiliated Hospital of Yunnan University;Department of Ophthalmology, the Second Hospital of Yunnan Province;Yunnan Eye Hospital;Key laboratory of Yunnan Province for Ophthalmic Disease Research, Kunming 650021, Yunnan Province, China and Department of Ophthalmology, Affiliated Hospital of Yunnan University;Department of Ophthalmology, the Second Hospital of Yunnan Province;Yunnan Eye Hospital;Key laboratory of Yunnan Province for Ophthalmic Disease Research, Kunming 650021, Yunnan Province, China
Abstract:AIM: To explore the multimodal imaging features of butterfly-like retinal pigment epithelial dystrophy(BPD)patients.

METHODS: Retrospective analysis of the multimodal imaging of 18 BPD patients(36 eyes)from January 2016 to July 2019, including fundus color photography, infrared photography, autofluorescence, fundus fluorescein angiography, indocyanine green angiography, optical coherence tomography(OCT), and optical coherence tomography angiography(OCTA).

RESULTS: A typical fundus color photography showed the appearance of a butterfly-like lesion caused by abnormal pigmentation. The appearance of the butterfly was not obvious after the lesion progressed, and the corresponding region shrinks; Infrared photography showed the yellow lesions clearly in the BPD patients, which are white highlight images; Autofluorescence showed patchy, spotted butterfly wing performance. When the lesion progressed, autofluorescence did not show typical butterfly changes, but it showed the damage of pigment epithelial cells; Fundus fluorescein angiography and choroidal angiography, in addition to showed butterfly lesions, can more accurately display vascular lesions, especially choroidal neovascularization(CNV); OCT showed lesions located between the retinal pigment epithelial(RPE)layer and the photoreceptors. As the lesion progressed, the pigment epithelium showed enlarged lesions correspondingly. Secondary CNV patients can be seen to break RPE; OCTA showed that the lesions were not obvious at the deep and superficial layers of the retina. But the choroidal blood flow signals were lost in some degrees in OCTA, and the blood flow images of CNV can be detected sensitively.

CONCLUTION: Multimode imaging technology can provide imaging features of progression in BPD patients, which helps clinicians to understand the disease more deeply.

Keywords:pattern retinal pigment epithelial dystrophy  butterfly-like pattern  multimodal imaging  lipofuscin  features
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