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.

Dr. Pablo Gili Manzanaro

Description

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.