Arjmand Parnian, Elimimian Elizabeth B, Say Emil A T, Shields Carol L
Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
Retin Cases Brief Rep. 2019 Fall;13(4):357-360. doi: 10.1097/ICB.0000000000000596.
To describe the optical coherence tomography angiography features of congenital simple hamartoma of the retinal pigment epithelium.
Case report.
A 14-year-old boy was referred for an asymptomatic fundus tumor in the left eye. Visual acuity was 20/20 in the right eye and 20/40 in the left eye. The right fundus was normal. The left fundus disclosed a gray-white nodular retinal mass of 2-mm diameter in the juxtafoveal region, protruding into the vitreous cavity and causing radial retinal folds. The mass demonstrated echodensity without calcification on ultrasonography, hypoautofluorescence on short-wavelength autofluorescence, and mixed hyporeflective and hyperreflective areas on near-infrared reflectance imaging. By fluorescein angiography, the mass showed absolute hypofluorescence through the arteriovenous phase and diffuse late hyperfluorescence and staining. Optical coherence tomography revealed an optically dense lesion with abrupt posterior shadowing, whereas optical coherence tomography angiography showed an extensive haphazard intratumoral vascular network with fairly large-caliber (100-200 microns) vessels occupying full-thickness tumor, demonstrating more vascular details than visualized on fluorescein angiography. Serial segmental evaluation of the optical coherence tomography angiography en face images confirmed an intratumoral vascular network for full tumor depth down to retinal pigment epithelium, not related to projection artifact. Final diagnosis was congenital simple hamartoma of the retinal pigment epithelium. Considering the benign tumor, observation was recommended.
Optical coherence tomography angiography of congenital simple hamartoma of the retinal pigment epithelium revealed an extensive intratumoral, haphazard, large-caliber vascular network, more evident than seen on fluorescein angiography. Optical coherence tomography angiography has added new insight into this rare tumor.
描述视网膜色素上皮先天性单纯性错构瘤的光学相干断层扫描血管造影特征。
病例报告。
一名14岁男孩因左眼无症状眼底肿瘤前来就诊。右眼视力为20/20，左眼视力为20/40。右眼眼底正常。左眼眼底在黄斑旁区域可见一个直径2毫米的灰白色结节状视网膜肿物，突入玻璃体腔并引起视网膜放射状皱褶。该肿物在超声检查中显示回声密度且无钙化，在短波自发荧光检查中显示低自发荧光，在近红外反射成像中显示混合性低反射和高反射区域。荧光素血管造影显示，该肿物在动静脉期均表现为绝对低荧光，晚期出现弥漫性高荧光和染色。光学相干断层扫描显示一个光学密度高的病变，其后部有突然的阴影，而光学相干断层扫描血管造影显示肿瘤内有广泛的杂乱血管网络，有相当大口径（100 - 200微米）的血管占据肿瘤全层，显示出比荧光素血管造影更多的血管细节。对光学相干断层扫描血管造影的正面图像进行连续节段评估证实，肿瘤全层直至视网膜色素上皮存在肿瘤内血管网络，与投影伪影无关。最终诊断为视网膜色素上皮先天性单纯性错构瘤。鉴于该肿瘤为良性，建议进行观察。
视网膜色素上皮先天性单纯性错构瘤的光学相干断层扫描血管造影显示肿瘤内有广泛的、杂乱的、大口径血管网络，比荧光素血管造影更明显。光学相干断层扫描血管造影为这种罕见肿瘤提供了新的见解。
