Scupola Andrea, Grimaldi Gabriela, Sammarco Maria G, Sasso Paola, Marullo Michele, Blasi Maria A
Department of Ophthalmology, Vitreoretinal Service, Catholic University of Rome, Rome, Italy.
Department of Ophthalmology, Ocular Oncology Unit, Università Cattolica del Sacro Cuore, Rome, Italy.
Eur J Ophthalmol. 2020 May;30(3):595-599. doi: 10.1177/1120672119831223. Epub 2019 Feb 14.
Combined hamartoma of the retina and retinal pigment epithelium is a rare benign tumor characterized by a variable combination of glial, vascular, and pigmented components. The purpose of our study was to analyze the features of combined hamartoma of the retina and retinal pigment epithelium on optical coherence tomography angiography.
Small case series of two cases of combined hamartoma of the retina and retinal pigment epithelium with macular and optic nerve involvement, evaluated with multimodal imaging including optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography.
On optical coherence tomography, combined hamartoma of the retina and retinal pigment epithelium is characterized by disruption of the inner neurosensory retina and a variable degree of involvement of the external retina. Optical coherence tomography angiography showed diffuse alterations of the retinal vessels of the superficial and deeper layers, extended to the peripapillary area. Vessel abnormalities included increased tortuosity and caliber of vessels, vascular traction, and vessel stretching within the lesion.
Optical coherence tomography angiography allows in-depth multilayer analysis of tumor vascular network, highlighting the fine abnormalities of retinal vasculature characteristic of combined hamartoma of the retina and retinal pigment epithelium.
视网膜和视网膜色素上皮联合错构瘤是一种罕见的良性肿瘤,其特征是神经胶质、血管和色素成分的多种组合。我们研究的目的是分析光学相干断层扫描血管造影上视网膜和视网膜色素上皮联合错构瘤的特征。
对两例累及黄斑和视神经的视网膜和视网膜色素上皮联合错构瘤的小病例系列进行了评估,采用了包括光学相干断层扫描、荧光素血管造影和光学相干断层扫描血管造影在内的多模态成像。
在光学相干断层扫描上,视网膜和视网膜色素上皮联合错构瘤的特征是神经感觉视网膜内层中断,外层视网膜受累程度不一。光学相干断层扫描血管造影显示浅层和深层视网膜血管弥漫性改变,延伸至视乳头周围区域。血管异常包括血管迂曲和管径增加、血管牵拉以及病变内血管伸展。
光学相干断层扫描血管造影可对肿瘤血管网络进行深入的多层分析,突出视网膜和视网膜色素上皮联合错构瘤特征性的视网膜血管细微异常。