INVISIBLE, HONEYCOMB-LIKE, CAVITARY RETINAL ASTROCYTIC HAMARTOMA

To report a patient with clinically invisible retinal astrocytic hamartomas (RAHs) detected on optical coherence tomography (OCT) and studied with OCT angiography (OCTA).

Case report.

An 11-year-old healthy girl was evaluated for reduced visual acuity in the right eye. On examination, best-corrected visual acuity was 20/50 in the right eye and 20/20 in the left eye. Fundus examination revealed blunted foveal reflex in the right eye and normal findings in the left eye. Optical coherence tomography disclosed an abruptly elevated, honeycomb-like macular mass with thin filamentous walls in the right eye, limited to the nerve fiber layer and measuring 756 μm in thickness. The numerous intralesional cavities (optically empty spaces) displayed no calcification. These findings were consistent with Type IV RAH. Optical coherence tomography of the left eye revealed intact foveola and focal nerve fiber layer thickening inferiorly without cavity, measuring 136 μm in thickness, consistent with Type I RAH. En face OCTA detected microvascular flow within the walls of the cavitary RAH in the right eye. Bilateral RAH in a child with no systemic evidence of tuberous sclerosis syndrome was established.

Multimodal imaging is important in revealing causes of visual loss and in the detection of subclinical fundus tumors. In this case, clinically invisible RAHs were detected only by OCT and surrounding vascular flow by OCTA.