DDx
Diagnostic Considerations
Differential diagnoses include the following:
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Other genetic macular disease: Stargardt disease, Best disease, pattern dystrophy, North Carolina macular dystrophy, among others
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Central serous chorioretinopathy
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Myopic degeneration
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Light or laser damage
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Toxic retinopathies: Hydroxychloroquine, tamoxifen, among others
Differential Diagnoses
Media Gallery
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A normal-appearing macula of the left eye. Note the even pigmentation of the retinal pigment epithelium and the absence of any yellow excrescences (drusen) in the fovea. The optic nerve has unrelated changes.
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In angiography, fluorescein dye is passed through a peripheral vein and transmits through the vascular system. The dye fluoresces in the vasculature, as seen here. No vascular prominences are seen in the macula or in any areas of dye pooling or staining. The abnormal vessels in the optic nerve, however, do show dye leakage.
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Moderate nonexudative age-related macular degeneration is shown with the presence of drusen (yellow deposits) in the macular region.
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Staining of drusen. Drusen absorb dye and, in the late frames of the angiogram, show hyperfluorescence. This staining is distinguished from the leakage that occurs when the dye spreads outside the boundary of the lesion.
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A more advanced case of nonexudative age-related macular degeneration (ARMD). This image shows drusen that are larger, more confluent, and soft. Soft drusen are defined as drusen that have indistinct borders. Such drusen are more likely to convert to wet ARMD. A few areas of atrophy are noted, where the retinal pigment epithelium (RPE) has lost pigmentation. The retinal cells overlying atrophic RPE are generally nonfunctional and result in a scotoma.
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The atrophic retinal pigment epithelium (RPE) demonstrates staining of the underlying choroidal vasculature. Normally, the intact RPE masks the presence of choroidal fluorescence. However, when the RPE atrophies, the underlying dye appears as an area of hyperfluorescence in the early stages of angiography. In the late stages, the drusen lose fluorescence in concert with (or with a small time lag) the rest of the retinal layers.
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A more advanced case of dry age-related macular degeneration. Several areas of atrophy are present, as are areas of significant pigment mottling in the macula. The large drusen inferior to fixation are poorly distinguished from each other.
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The atrophic areas are easily distinguished by the hyperfluorescence of the retinal pigment epithelium (RPE) in the mid phase of the angiogram. Hypofluorescence of dye, due to masking caused by the increased pigmentation, is seen. No areas of frank dye leakage or exudative age-related macular degeneration (ARMD) are apparent. A "hot cross bun" pattern of dry ARMD-related pigment changes is evident near the fovea.
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High-definition optical coherence tomography scan of a 67-year-old woman showing retinal pigment epithelium mottling and pigment epithelial detachments temporal to fixation consistent with dry macular degeneration.
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Fundus photo showing drusen in a 67-year-old woman with dry age-related macular degeneration.
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Fluorescein angiogram 4 minutes after injection of dye on 67-year-old woman showing pigment epithelial detachments.
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A later frame of the angiogram demonstrating the absence of dye leakage outside the lesion, with staining of the areas of atrophy (window defects) in the macular region.
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High definition optical coherence tomography right eye demonstrating retinal pigment epithelium atrophy and changes in the deeper layers of retina. The absence of intraretinal cysts, subretinal fluid, or sub-retinal pigment epithelium fluid indicates the absence of wet age-related macular degeneration.
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Spectral domain optical coherence tomography (SD-OCT) analysis: OCT B-scans show the presence of pigment epithelial detachment bilaterally in a patient with previously diagnosed dry age-related macular degeneration (AMD), which appeared relatively stable in comparison to previous scans. No subretinal fluid or retinal edema was detected.
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Fluorescein angiography: Fundus angiography in the same patient shows staining of drusen and window defects only in each eye. No active neovascularization was detected in either eye.
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Optical coherence tomography (OCT) shows an active neovascular network in the right eye as opposed to the nonvascularized pigment epithelial detachment found in the left eye. The spectral domain optical coherence tomography (SD-OCT) images in the lower panels confirm pigment epithelial detachment formation in each eye.
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Optical coherence tomography (OCT) shows an active neovascular network in the right eye as opposed to the nonvascularized pigment epithelial detachment found in the left eye. The spectral domain optical coherence tomography (SD-OCT) images in the lower panels confirm pigment epithelial detachment formation in each eye.
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