Optical coherence tomography angiography of astrocytic hamartoma demonstrates intrinsic vascularity

PURPOSE: To evaluate the findings of astrocytic hamartoma in the setting of gyrate atrophy, including details of optical coherence tomography angiography (OCTA). OBSERVATIONS: Multimodal imaging was obtained on a 20-year-old woman with genetically-confirmed gyrate atrophy. Dilated fundus exam was performed, followed by ultra-widefield color and green autofluorescence imaging and OCTA of bilateral peripapillary and optic disc lesions. Clinical and imaging findings were consistent with gyrate atrophy. The bilateral peripapillary and optic disc lesions had a glistening, translucent, and mulberry-like appearance. OCTA imaging of these lesions clearly demonstrated an intrinsic vascular network and hyporeflective spaces within the lesion, which could not be seen on routine examination. CONCLUSIONS AND IMPORTANCE: OCTA was used to noninvasively diagnose astrocytic hamartoma in this patient with gyrate atrophy by showing the intrinsic vasculature and hyporeflective spaces of the lesion. This imaging modality can help differentiate astrocytic hamartoma from other lesions that typically lack intrinsic vascularity, such as optic disc drusen.