PMC6637436_01
External photograph (A) reveals telecanthus, flare of the medial eyebrows and hypoplastic nasal alae. Slit lamp image of the right eye (B) reveals a sectoral hypopigmentation of the iris extending from 9' o'clock meridian to 1'30" o'clock meridian. Slit lamp image of the left eye (C) reveals a complete hypopigmentation of the iris imparting it a 'brilliant blue' appearance. Color fundus montage of the right eye (D) reveals no area of hypopigmentation. Color fundus montage of the left eye (E) reveals a partially hypopigmented area in the superior fundus. SS-OCT of the right eye (F) reveals a subfoveal choroidal thickness of 455 micro. SS-OCT of the left eye (G) reveals a subfoveal choroidal thickness of 569 micro, a choroidal thickness of 591 micro in the pigmented fundus, and a choroidal thickness of 457 micro in the hypopigmented fundus.
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F1
Gross examination revealed telecanthus, flare of the medial eyebrows, and hypoplastic nasal alae (Figure 1A (Fig. 1)). Anterior segment examination revealed a sectoral heterochromia iridum in the right eye with a hypopigmented 'brilliant blue' iris extending for 4 hours, superotemporally (Figure 1B (Fig. 1)). The left eye had a 'brilliant blue' iris with relatively poor pupillary dilation (Figure 1C (Fig. 1)). The right fundus had a variegated appearance without choroidal hypopigmentation (Figure 1D (Fig. 1)). The left fundus had choroidal hypopigmentation beyond the superotemporal arcade up to the periphery (Figure 1E (Fig. 1)). Topcon DRI OCT -1 Atlantis) and compared between the two eyes and between the pigmented and the vitiliginous areas in the left eye. The subfoveal choroidal thickness was 455 micro (Figure 1F (Fig. 1)) and 569 micro in the right and left eye, respectively (Figure 1G (Fig. 1)).