Abstract
PURPOSE: Presentation of a case of Leber hereditary optic neuropathy (LHON) with arterial sheathing eleven years after initial loss of vision. OBSERVATIONS: A 46-year-old female was referred for re-evaluation of Leber hereditary optic neuropathy. She first noticed rapid painless loss of vision eleven years prior. Fundus imaging performed at that time did not demonstrate arterial sheathing. Genetic testing revealed the presence of the LHON 11778 G-A mitochondrial mutation. Laboratory values were within normal limits save for angiotensin-converting enzyme elevated to 69 U/L. Eleven years later, visual acuity was count fingers at 12 feet with complete loss of color vision. Funduscopic examination of the optic nerve revealed bilateral pallor, sheathing of the retinal arteries, diffuse vessel narrowing, and tortuous retinal vessels. CONCLUSIONS AND IMPORTANCE: We present a case of LHON that demonstrates retinal arterial sheathing and possibly broadens the spectrum of LHON fundus findings.