Combined hamartoma of the retina and retinal pigment epithelium
Retinography (Visucam 500, Zeiss) A: Color, B: Green filter. C: Fundus autofluorescence. D: Red filter
Fluorescein angiography (FF450 IR plus, Zeiss): Screen effect due to lesion pigmentation in early stages, with marked vascular tortuosity and oozing in late stages.
Optical coherence tomography (Cirrus-HD 5000, Zeiss): Marked thickening of the macular area with total destructuring of layers, cystic spaces and posterior shadow.
Combined hamartoma of the retina and retinal pigment epithelium (HCREPR) is a benign tumor that can cause significant visual loss. HCREPR is usually a solitary, unilateral lesion located juxtapapillary or in the posterior pole. Its typical appearance is raised, vascularized, and pigmented glial tissue.
Comments
Tests:
Retinography (Visucam 500, Zeiss): Color: pigmented lesion with central whitish area. Green filter: Highlights vascular tortuosity. AF: Highlights hypoautofluorescence that delimits the lesion. Red filter: Highlights RPE alteration.
Fluorescein angiography (FF450 IR plus, Zeiss): Screen effect due to lesion pigmentation in early stages, with marked vascular tortuosity and oozing in late stages.
Optical coherence tomography (Cirrus-HD 5000, Zeiss): Marked thickening of the macular area with total destructuring of layers, cystic spaces and posterior shadow.
Indication
A 37-year-old woman with a long-standing unilateral pigmented lesion with marked vision loss since childhood. Diagnosed in her country of origin with a lesion caused by toxoplasmosis. She presented a pigmented macular lesion, with vascular tortuosity and a fibrotic macular epiretinal membrane. Multimodal imaging tests supported the diagnosis of HCREPR.
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