This healthy 10YO female presented with these asymptomatic bilateral lesions. Vision was 20/20 OU.
Fundus photography of her right eye shows multiple bear track lesions scattered throughout the posterior pole. An interlacing pattern of whitish deep retinal lines courses through the macula. The pigmented lesions are hypo-autofluorescent. Similar findings were noted in the left eye (not shown).
Learning Points:
Group-type congenital pigmented nevi of the RPE (bear tracks) is a relatively rare congenital condition characterized by well-demarcated, hyperpigmented, flat, variably sized RPE lesions that resemble bear footprints. Histopathologically, these lesions are similar to congenital hypertrophy of the RPE (CHRPE), with increased numbers of pigment granules in normal-sized RPE cells. These lesions are benign and cause no visual symptoms.
The major fluorophore in fundus autofluorescence (FAF) is lipofuscin, which is intraliposomal RPE material generated as byproducts of outer segment metabolism. The outer retina overlying these bear track lesions shows varying degrees of atrophy. This leads to less lipofuscin production with hypo-FAF.
Although bear tracks and the typical CHRPE lesions (large, round, unilateral, with depigmented lacunae with age) are not associated with systemic abnormalities, the multifocal, often bilateral, and small comet-shaped lesions with familial adenomatosis are associated with colonic polyps and cancer (Romania et al Ophthalmology 1992;99:911-913).
Benign lobular inner nuclear layer proliferations were described by Sanfilippo et al in 2023 as a unique benign lesion often associated with CHRPE lesions (Ophthalmology 2023;130:265-273). They appear as white, multifocal, often lobular tumors centered in the posterior pole. On OCT, they appear in the inner nuclear layer at the border of the outer plexiform layer.
