Case #23 – Congenital Hypertrophy of the RPE

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Case 6: Optomap® Fundus Image Comparison of Left Eye Histopathological features of isolated CHRPE include hypertrophic RPE cells engorged with melanosomes and minimal or absent lipofuscin in pigmented areas.1 The depigmented areas within the lesion where pigment granules are very sparse, known as lacunae, correspond to atrophic RPE cells with few melanosomes and

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Case 6: An asymptomatic 37-year-old presented with no remarkable medical history and BCVA 20/20 OU. A nearly five disc diameter dark lesion is imaged in the temporal fundus of the left eye.

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Case 5: Optomap® Fundus Image Comparison of Right Eye Note similar pigmented lesions superior to the disc in the right eye. As previously stated, CHRPE "bear tracks" are typically found in only one eye and if pigmented lesions are found bilaterally there is a higher risk the patient may have FAP.

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Case 5: Optomap® Fundus Image Comparison of Left Eye Familial adenomatous polyposis (FAP) has been linked to the appearance of CHRPE-like lesions in the retina. These CHRPE-like lesions are RPE abnormalities that occur bilaterally and have some degree of variability in size and shape.1 There are usually four or more lesions present

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Case 5: A 12-year-old boy with a confirmed diagnosis of familial adenomatous polyposis (FAP). Multiple flat even textured oval-shaped lesions with halos were observed in both eyes.

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Although the pigmented groups are CHRPE lesions, in this case the lesions are more widespread throughout the entire fundus instead of being found in only a single quadarant.

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Case 3: Optomap® Fundus Image Comparison of Left Eye Grouped CHRPE lesions have an increased number of pigment granules similar to isolated CHRPE, and these pigment granules are significantly larger than those observed in adjacent normal RPE cells.1,3

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Case 3: Optomap® Fundus Image Comparison of Left Eye Note the pigmented lesions become larger as they extend toward the periphery. The lesions are more obvious in the composite color and red separation optomap® images, but still somewhat visible in green separation.

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