Benign concentric annular macular dystrophy. Diagnosis in a pediatric case

AbstractPurpose We report a case of a benign concentric annular macular dystrophy diagnosed in childhood during a routine examination. We describe all the ophtlamologic features, complementary tests and the differential diagnosis.Methods A 5 year old female patient who went to her first ophthalmological examination; her visual acuity (VA) was normal, but we found in the funduiscopy of her right eye an image of annular pigment epithelial atrophy in the macula zone with mottled pigment in the lesion. We appreciated in the left eye funduscopy a less marked alteration of the macular pigment epithelium.Results The electrophysiological parameters were completely normal, appreciating a bilateral foveal atrophy in the macular optical coherence tomography. We didn’t perform perimetry and fluorescein angiography due to early age of the patient. The patient had no relevant family history. This suggests the diagnosis of benign concentric annular macular dystrophy.Conclusion This is a pattern dystrophy described by Deutman in 1974, with few cases in the literature so far. Inheritance is autosomal dominant and characterized by an hypopigmented ring with respected foveal center. Usually diagnosed in adulthood and initially has a respected VA, so it is important to make a differential diagnosis with other entities to submit a bull's‐eye maculopathy, as malarial retinopathy, cone dystrophy and Stargardt disease. May evolve toward greater loss of VA and electroretinogram abnormalities, it is therefore necessary routine checks in these patients.