Congenital tarsal kink syndrome.

Congenital horizontal tarsal kink syndrome is a rare but well-defined form of congenital upper eyelid entropion, characterized by a horizontal tarsal fold causing inversion of the lid margin and lashes, with absence of a visible upper eyelid crease. Induced trichiasis may lead to corneal complications and a major risk of amblyopia if diagnosis and management are delayed. We report the case of a 4-week-old infant with right unilateral tarsal kink complicated by a central corneal ulcer and associated microphthalmia. This case was managed by an anterior approach with release of orbicularis adhesions, everting sutures, amniotic membrane graft and temporary tarsorrhaphy, achieving rapid corneal healing and sustained normal eyelid position. A systematic review of 38 prior published cases from 1894 to 2025 revealed a male predominance, mostly unilateral, and high frequency of corneal lesions. Surgery was required in 94.9% without reported recurrence, regardless of technique (open or closed, anterior or posterior). In this condition, early recognition and prompt surgical management can prevent corneal damage and amblyopia.