In this case series, FAF showed decreased autofluorescence of the spots consistent with focal retinal pigment epithelium atrophy or abnormal material blocking normal autofluorescence and areas of increased autofluorescence suggesting retinal pigment epithelium dysfunction. Fluorescein angiography showed early and stable hyperfluorescence of the spots without leakage.
![bear tracks retina bear tracks retina](https://imagebank.asrs.org/Content/imagebank/a16b8341-06fc-4cc8-a4c3-8b25729f4740.jpg)
Optical coherence tomography showed a disruption in signal from the hyperreflective layer corresponding to the inner and outer segment junction and increased signal backscattering from the choroid in the area of the spots. Fundus autofluorescence imaging of the spots showed decreased general autofluorescence and decreased peripheral autofluorescence surrounding central areas of retained or increased autofluorescence. One eye also had additional spots detected on FAF imaging that were not visible on stereo biomicroscopy or color fundus photographs. In all three eyes with congenital grouped albinotic spots, FAF imaging showed autofluorescent spots corresponding to the albinotic spots seen on stereo biomicroscopy. Three eyes of three patients with congenital grouped albinotic spots were evaluated with FAF and optical coherence tomography imaging to evaluate the nature of the albinotic spots.
#Bear tracks retina series#
The purpose of this study was to describe the findings of fundus autofluores-cence (FAF) and optical coherence tomography in a series of patients with congenital grouped albinotic spots. It is important to distinguish grouped CHRPE lesions from the POFLs associated with familial cancer syndromes. The combination of both pigmented and non-pigmented lesions occurring in the same individual with grouped CHRPE is rare. Fluorescein angiography demonstrated persistent hypofluorescence correlating with the clinically observed areas of hyperpigmentation and hypopigmentation. A unique, co-existing feature was the presence of non-pigmented, punctate lesions located within the maculae suggestive of grouped non-pigmented CHRPE. The appearance was consistent with grouped pigmented CHRPE. These plaque-like lesions were circumferential along the peripheral fundus and were associated with smaller foci of pigmentation oriented towards the posterior pole. To describe a unique presentation of grouped congenital hypertrophy of the retinal pigment epithelium (CHRPE) and to review the clinical features which differentiate grouped CHRPE from the pigmented ocular fundus lesions (POFLs) associated with familial cancer syndromes.Ī 28-year-old asymptomatic, Caucasian female demonstrated multiple small, flat, dark brown to black clusters of retinal pigment epithelium (RPE) hypertrophy on dilated fundus examination of both eyes. No intraretinal fluid was seen during the late phase of the angiogram (Figure 1). The arterial phase revealed a transmitted hyperfluorescent lesion (a window defect of the retinal pigment epithelium) that corresponded to the lesions seen during the ophthalmic examination. Fluorescein angiography showed a normal choroidal and retinal vascular perfusion. The size of the flecks was highly variable, from the diameter of one vessel to 4 times the diameter of the optic nerve but the flecks had a consistent color pattern: in the smaller lesions, the color was more homogeneous, and in the larger lesions, it was concentrated at the edge of the flecks (Figure 1).
![bear tracks retina bear tracks retina](https://i0.wp.com/entokey.com/wp-content/uploads/2020/03/9781626238336_c098_f003.tif_epub1.jpg)
Funduscopy of both eyes revealed multiple white and sometimes yellow flecks of variable size and configuration affecting all parts of the retina (periphery, equator, posterior pole, and fovea) (Figure 1). Slitlamp examination of the anterior segments and vitreous cavity was unremarkable. Visual acuity was 20/20 in both eyes with −0.50 cylinder correction in the left eye. She had occasional headaches but no specific ocular symptoms, and she was in excellent general health. A 19-year-old woman was referred for evaluation after her general ophthalmologist noted areas of retinal abnormality on ophthalmoscopy after a routine refraction.