Acorea: A rare congenital anomaly.

A young male presented to our clinic with a history of diminution of vision in the left eye from childhood. His vision in the left eye was hand movement at 1 M distance. Slit-lamp examination of the left eye showed the absence of pupillary opening with folds of iris tissue at center [Fig. 1a]. Krimsky test measured 35 prism diopter of exotropia. No family history of similar condition was noted. Lens status and ultrasonography B-scan of the left eye was within normal limit. Anterior segment optical coherence tomography revealed an open-angle and absence of pupillary opening [Fig. 1b]. A diagnosis of acorea was made. Acorea can be associated with microphthalmos, cataract, and iridocorneal dysgenesis which is suspected to result from autosomal dominant mutation. If diagnosed early, a pupilloplasty can help prevent the development of stimulus deprivation amblyopia.

Figure 1

(a) Slit-lamp photograph of the left eye showing the absence of pupil with folds of iris tissue at the center. (b) Anterior segment optical coherence tomography of the left eye showing the absence of pupil

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