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RETINAL ASTROCYTIC HAMARTOMA

Originally posted on @retina.rocks 04/27/2020

Our patient (courtesy of Tufts) has the classic mulberry appearance of a retinal astrocytic hamartoma. Early in life the inner retinal lesions have a fleshy white appearance and are often multifocal and bilateral. Later in life they develop the mulberry or fish egg-like appearance with significant calcification.

Fluorescein angiography (FA) shows the often angiomatous nature of these lesions. OCT shows the elevated, hyperreflective inner retinal lesion, with marked shadowing more posteriorly.

These tumors are generally are just observed. Rarely, the vascular component can cause intraretinal exudation requiring treatment.

Learning Points:
A retinal astrocytic hamartoma is a benign tumor composed of spindle-shaped fibrous astrocytes located in the nerve fiber layer, most commonly associated with tuberous sclerosis.

Tuberous sclerosis includes the classic triad of seizures, mental deficiency and fibroangiomas, but patients can have lesions throughout the body including the eyes, skin (ash-leaf spots), bone, nails, teeth, brain, heart, lungs and kidneys.