Purpose: To describe the multimodal imaging, including en face optical coherence tomography (OCT) and OCT angiography, findings of a case of macular microcysts associated with neuromyelitis optica.
Methods: Findings on clinical examination, color fundus photography, fluorescein angiography, fundus autofluorescence, visual fields, and OCT including en face OCT and OCT angiography are presented.
Results: A 12-year-old African American boy presented with bilateral optic atrophy from neuromyelitis optica. Clinical examination was notable for bilateral optic nerve head pallor. Visual fields of both eyes showed generalized depression. Fluorescein angiography and fundus autofluorescence were unremarkable. Spectral domain OCT B-scan images showed characteristic paracentral, hyporeflective, microcystic lesions in the inner nuclear layer of both eyes, and en face OCT images demonstrated a corresponding pattern of large paracentral cysts radiating peripherally into smaller diffuse cysts. Optical coherence tomographic angiography of the superficial and deep retinal capillary plexuses was unremarkable.
Conclusion: Macular microcysts have been associated with various forms of optic atrophy, including neuromyelitis optica. Spectral domain and en face OCT imaging of the microcysts demonstrated a very characteristic pattern. Normal fluorescein and OCT angiography suggest that nonvascular causes, such as Müller cell degeneration, might contribute to the etiologic mechanism.