Unilateral giant coloboma of the upper eyelid associated with other congenital anomalies (33 years follow-up of surgical repair).

PURPOSE: To describe a case of congenital unilateral giant coloboma and its successful surgical repair with 33 years of follow-up.

CASE REPORT: A 6-year-old boy presented with a congenital unilateral giant coloboma of the right upper eyelid associated with madarosis of the eyebrows, microphthalmos, dystopia of the hair, and coloboma of the apex of the nose. The patient underwent surgical repair of the multiple anomalies in different steps.

DISCUSSION: A multiple-step, two-layer technique for the reconstruction of the right upper eyelid was performed in a 6-year-old boy with congenital unilateral giant coloboma associated with multiple ocular and facial anomalies. After 33 years of follow-up, the cosmetic results are excellent, although it has not been possible to preserve the visual function of the right eye, which had to be enucleated.