Abstract
Background: The congenital tarsal kink syndrome is a rare form of congenital upper eyelid entropion associated with cardiovascular, musculoskeletal or central nervous system disorders. This syndrome must be recognized and surgically treated as a perinatal emergency to avoid associated complications—corneal ulcer, corneal leucoma, secondary amblyopia and decreased vision among children. Methods: A literature review was conducted to clarify the diagnosis particularities and the corrective surgery options of the congenital entropion on the upper eyelid. Results: Four relevant studies were found by researching the Web of Science and PubMed databases up to November 2021 for “congenital tarsal kink syndrome” and “congenital upper eyelid entropion”. Conclusions: In this paper, we present a case of congenital unilateral entropion of the upper left eyelid in the context of a tarsal kink syndrome in a one-month old infant, manifested by the absence of eyelashes on the upper eyelid of the left eye, hyperlacrimation and conjunctival hyperemia. Essential in managing the upper eyelid entropion is protecting the cornea. Furthermore, correcting a tarsal kink is eminently surgical, choosing between open or closed procedures. Herein, we address the difficulty in the timely diagnosis of this uncommon condition and make formal recommendations based on all reported cases.
Highlights
IntroductionThe average age at the time of diagnosis varies between 1 and 60 weeks [2]
Physical examination revealed the presence of a horizontal tarsal kink determining the internal rotation of the upper eyelid margin, the cilia coming into contact with the ocular globe and causing secondary blepharospasm, hyperlacrimation, photophobia and corneal ulcerations [2,3]
Twenty-seven cases of congenital entropion have been identified in the context of tarsal kink syndrome, 16 male and 11 female (Table 1)
Summary
The average age at the time of diagnosis varies between 1 and 60 weeks [2] It mainly affects boys and is mainly unilateral, but it can be bilateral [2,3]. The condition must be recognized and surgically treated as an emergency to avoid associated complications—corneal ulcer, corneal leucoma and secondary amblyopia [6,7,8]. The congenital tarsal kink syndrome is a rare form of congenital upper eyelid entropion associated with cardiovascular, musculoskeletal or central nervous system disorders. This syndrome must be recognized and surgically treated as a perinatal emergency to avoid associated complications—corneal ulcer, corneal leucoma, secondary amblyopia and decreased vision among children.
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