Abstract
Lens coloboma is a rare congenital anomaly that occurs due to failure of the foetal fissure to close completely causing an incomplete formation of the lens, so usually inferonasal. Temporal lens coloboma is extremely rare; reported cases were associated with ocular or systemic anomalies. We report a rare case of unilateral atypical temporal lens coloboma without any iris abnormality and posterior segment coloboma or any other ocular or systemic anomaly.
 An 18-year-old female presented with a gradually progressive painless diminution of vision in her left eye for two years. There was no history of trauma, and family history was non-contributory. The systemic examination was normal. Ocular examination revealed a vision of 6/6 in the right eye and 6/60 in the left eye improving to 6/24 with pinhole and unilateral atypical temporal coloboma of the crystalline lens in the left eye after dilatation. The rest of the anterior segment and fundus examination was normal. No abnormality was detected in the right eye. The best corrected visual acuity of 6/6 OD and 6/9 OS was achieved with spectacle correction at 6 months of follow-up. This case is a rare presentation of unilateral atypical coloboma located temporally without any iris abnormality and posterior segment coloboma or any other ocular or systemic anomaly. Early correction of refractive error and astigmatism results in good prognosis.
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