BACKGROUND: Two patients presented with congenital rotated macula with good vision and binocular function. CASES: Two patients had retinal folds with an extremely rotated macula OD as the result of peripheral fibrous proliferation on the retina. Each macula was substantially rotated to the nasal border of the disc. OBSERVATIONS: A 3-year-old girl (case 1) with best-corrected visual acuity (VA) of 0.5 OD and 1.0 OS was treated for amblyopia, after which she successfully achieved 1.0 bilaterally as well as good stereopsis of 120 seconds of arc measured with the TNO test. A 6-year-old girl (case 2) obtained a VA of 0.7 OD and 1.0 OS, and her stereopsis was of 240 seconds of arc. CONCLUSIONS: Good VA and stereopsis may be achieved by adaptation in the brain and an extremely large fusional potential at an early infantile age or a gradual shift of the macula. Appropriate treatment of amblyopia should be performed in patients with rotated macula if the macula appears normal.
BACKGROUND: Two patients presented with congenital rotated macula with good vision and binocular function. CASES: Two patients had retinal folds with an extremely rotated macula OD as the result of peripheral fibrous proliferation on the retina. Each macula was substantially rotated to the nasal border of the disc. OBSERVATIONS: A 3-year-old girl (case 1) with best-corrected visual acuity (VA) of 0.5 OD and 1.0 OS was treated for amblyopia, after which she successfully achieved 1.0 bilaterally as well as good stereopsis of 120 seconds of arc measured with the TNO test. A 6-year-old girl (case 2) obtained a VA of 0.7 OD and 1.0 OS, and her stereopsis was of 240 seconds of arc. CONCLUSIONS: Good VA and stereopsis may be achieved by adaptation in the brain and an extremely large fusional potential at an early infantile age or a gradual shift of the macula. Appropriate treatment of amblyopia should be performed in patients with rotated macula if the macula appears normal.