Inward rotation of the lower or upper eyelid margin, present at or shortly after birth (Maman, Ann Plast Surg 2011)
- Proposed mechanisms of congenital upper eyelid entropion
- Direct mechanical pressure in utero
- Orbicularis spasm and hypertrophy from corneal abrasion
- Intrauterine tarsal deformity
- Proposed mechanisms of congenital lower eyelid entropion
- Vertical deficiency in the posterior eyelid lamella with or without disinsertion of the lower eyelid retractor muscles
- Horizontal laxity of the medial or lateral canthal tendons
- Facial nerve palsies
- Congenital lower eyelid entropion is more common than upper eyelid entropion; however, both presentations are extremely rare.
- In a case series by Alsuhaibani et al. (Eye 2012), children with isolated facial nerve palsy were more prone to develop lower eyelid entropion; in contrast, adults with facial nerve palsy typically develop ectropion.
- Sires et al. (Ophthal Plast Reconstr Surg 1999) reported a retrospective clinical series of 25 cases of congenital horizontal tarsal kink. The classic presentation was male, Caucasian, diagnosed at 7.2 weeks of age, bilateral, or right-sided. Corneal ulcers were seen in 50% of patients.
- Typically presents at birth with diagnosis based on clinical malposition of eyelids
- Infrequent opening of eyes
- Corneal epithelial defects
- Upper eyelid congenital entropion
- More severe form of congenital upper eyelid entropion, known as the tarsal kink syndrome, is due to a congenital malformation of the tarsal plate. On eversion, the posterior tarsus demonstrates a large horizontal kinking (scarring) of the tarsus, that results in inversion of the eyelid margin and lashes onto the ocular surface.
- Typically absent upper eyelid folds (Biglan, Am J Ophthalmol 1980).
- Has been associated with cutis laxa, a rare connective disorder with redundant skin (Al-Faky, Pediatr Dermatol 2014).
- Lower eyelid congenital entropion
- Much more common than upper eyelid congenital entropion.
- Easier to diagnosis as more obvious clinical presentation at birth
- Often confused with epiblepharon
Figure 1. Congenital entropion. Courtesy Rona Z. Silkiss, MD, FACS.