Ординатура / Офтальмология / Английские материалы / Atlas of Fundus Autofluorescence Imaging_Holz, Schmitz-Valckenberg, Spaide, Bird_2007

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Fig. 9.2  Stargardt macular dystrophy/fundus flavimaculatus, late stage. Fundus photographs and fundus autofluorescence (FAF) montage of a 48-year-old man diagnosed with Stargardt disease more than 20 years previously. There is extensive multi-focal retinal pigment epithelium atrophy in the macula, showing levels of severe decreased FAF signal. The retinal periphery shows marked alteration of normal background signal with disseminated areas of increased and decreased intensity. Note that the FAF signal appears to be normal around the optic nerve head (peripapillary sparing), a typical finding in patients with Stargardt disease (see also Fig. 9.1) [3]

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Fig. 9.3  Best vitelliform macular dystrophy. Top row: Reflectance and fundus autofluorescence (FAF) image of the left eye of a 10-year-old boy (vitelliform stage). A well-defined white deposit centrally at the level of the retinal pigment epithelium (RPE) is observed. Visual acuity with the left eye was 6/6. FAF imaging shows an increased signal that corresponds with the deposit but also extends outside the lesion as seen on ophthalmoscopy [7, 8, 15, 18, 25, 27]. The surrounding fundus is relatively more fluorescent than age-matched normal subjects. Middle row: FAF images of both eyes in a 21-year-old man. Visual acuities were 6/6 for the right and 6/5 for the left. The right eye (vitelliruptive stage) shows levels of increased FAF inside the central lesion, particularly at the bottom. In the very center, decreased intensity can be observed, indicating incipient RPE atrophy. FAF imaging of the left eye discloses a level of increased FAF at the borders of the lesion with a very bright signal at the bottom (pseudo­ hypopyon stage). Bottom row: Fundus photograph and FAF image of the left eye of a 65-year-old woman (atrophic stage). Visual acuity with the left eye was 6/9. Previous electrophysiology had shown a normal electroretinogram but a pathological electro-oculogram with no light rise. The central area of RPE atrophy exhibits a much decreased FAF signal. Note that that there is a second lesion in the superior macula in the vitelliruptive stage showing a mottled increased FAF signal

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Fig. 9.4  Adult-onset vitelliform macular dystrophy. Top and middle rows: Fundus photograph, fundus autofluorescence (FAF) image, and earlyand latephase fluorescein angiogram of a 48-year-old man presenting with a gradual decrease in visual function over the previous years. Electrophysiology disclosed a borderline electro-oculogram but no typical findings for Best disease. There is whitish-yellow deposit at the central macula, which corresponds to marked increased autofluorescence [2, 19, 20, 23, 25]. Early angiography shows hypofluorescence due to masking effects. A late-phase angiogram disclosed hyperfluorescence with staining of the lesion. Furthermore, basal laminar deposits (“stars in the sky,” “Milky Way”) as hyperfluorescence spots can be identified in the outer macula. Bottom row: Reflectance and FAF image 12 months later. The central lesion has undergone disruption with mottled spots of marked increased FAF signal

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Fig. 9.5  Adult-onset vitelliform macular dystrophy. Top row: Left eye of a 75-year-old woman with adult vitelliform macular dystrophy with a well-de- fined white deposit at the level of the retinal pigment epithelium centrally and visual acuity of 6/18. She had no family history, and her vision had been stable for years. Very marked enhanced autofluorescence signal corresponds with the deposit. Middle row: Fundus photograph and fundus autofluorescence (FAF) image of the right eye of a 76-year-old woman, visual acuity 1/60. Vision in the left eye had recently deteriorated. Electrophysiology disclosed an abnormal pattern electroretinogram but a normal Ganzfeld electroretinogram and a normal electro-oculogram. FAF imaging shows levels of increased and decreased intensity inside the central lesion, implying the beginning of retinal pigment epithelium (RPE) atrophy. Bottom row: Reflectance and FAF images of a 53-year-old man with adult vitelliform macular dystrophy and an atrophic lesion at the level of the RPE centrally. Right eye visual acuity was 6/60. Autofluorescence imaging showed decreased signal that corresponded to the atrophic area. The surrounding fundus was relatively more fluorescent than in age-matched normal subjects

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Fig. 9.6  Dominant drusen/Doyne honeycomb maculopathy/malattia leventinese. Fundus autofluorescence (FAF) images and fundus photographs in patients with dominant drusen [14]. Autofluorescence imaging typically reveals levels of increased intensity corresponding to funduscopically visible drusen areas [26]. This observation is in contrast to hard and small drusen in patients with age-related macular degeneration, which usually show normal or slightly decreased autofluorescence (see Chap. 11). Top left: Areas of marked decreased intensity in patients with dominant drusen may be caused by atrophy of the retinal pigment epithelium. Bottom line: This patient was tested positive for fibulin- 3 gene mutation