Congenital ectropion

Congenital ectropion is a rare developmental anomaly of the eyelid present at birth, in which the eyelid margin (most commonly the lower eyelid) is turned outward, exposing the inner conjunctival surface. This condition is classified under congenital malformations of the eyelid (ICD-10: Q10.1) and is distinct from acquired ectropion, which develops later in life due to aging, scarring, or other causes. Congenital ectropion primarily affects the ocular and periorbital structures. It may occur as an isolated finding or in association with other congenital conditions, including blepharophimosis syndrome, Down syndrome, ichthyosis (particularly harlequin ichthyosis and lamellar ichthyosis), or other craniofacial anomalies where skin tightness or structural abnormalities contribute to eyelid malposition. Key symptoms include outward turning of the eyelid margin visible at birth, chronic exposure of the conjunctiva, excessive tearing (epiphora), conjunctival injection and inflammation, and potential corneal exposure leading to dryness, keratopathy, or ulceration if left untreated. The exposed conjunctival tissue may become thickened and keratinized over time. In severe cases, chronic corneal exposure can threaten vision. Management depends on the severity of the condition and any associated disorders. Mild cases may be managed conservatively with lubricating eye drops, ointments, and protective measures to prevent corneal drying and damage. More significant cases typically require surgical correction, which may involve skin grafting, lateral tarsal strip procedures, or other eyelid-tightening techniques to restore normal eyelid position and protect the ocular surface. Early intervention is important to prevent complications such as corneal scarring and amblyopia. When congenital ectropion occurs as part of a syndromic condition, treatment of the underlying disorder (such as skin management in ichthyosis) is also essential.

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