Case of bilateral congenital ectropion

Recently I came across a case report published in the journal Indian Pediatrics by Surana I and Surana S. about bilateral congenital ectropion of the upper eyelids in a six month old infant. The child also had features of Down’s syndrome, a genetic disorder characterized by chromosomal abnormality of trisomy 21. Congenital ectropion is frequently associated with a condition called blepharophimosis, a condition in which there is bilateral shortening of the eyelid openings, both horizontally and vertically.

Ectropion of the lower lid - from NHS

Ectropion is a condition in which the eyelids are everted or turned away from the globe or the eyeball. It is characterized by epiphora or overflow of the tears. This happens because the lacrimal punctum, the opening through which the tear drains form the eye is situated on the lid margin. As the lid is turned away, the tears are no longer able to flow through their normal path. Instead they accumulate and subsequently overflow. It is interesting to note that in this case, epiphora would not be a prominent symptom. Why is it so? It is because  most of the tear is drained by the punctum of the lower eyelid as tears naturally tend to gravitate downwards. Only a small amount of tear is drained by the upper punctum and without the involvement of the lower punctum the drainage remains sufficient. Ectropion may also lead to keratitis (inflammation of the cornea) and corneal ulcer. This happens because the eye is continuously exposed to the environment; hence it is also termed as exposure keratitis.

Ectropion is subdivided according to its causes-

• Involutional or senile
• Cicatricial
• Paralytic
• Congenital
• Mechanical

Let us examine the rationale behind the diagnosis. Involutional ectropion is ruled out as the patient is an infant. Cicatricial and mechanical ectropion is also ruled out as there is not history of scar formation, injury or space occupying lesion. Paralytic ectropion follows the paralysis of orbicularis oculi, the muscle that closes the lid. It is supplied by the temporal and zygomatic branches of the facial nerve. So paralysis of the facial nerve may cause ectropion. In this case, facial nerve paralysis was ruled out. Hence we get the diagnosis of congenital ectropion.

Ectropion is one of the rarest ocular symptoms of Down’s syndrome. In this case, although there were features of Down’s syndrome, the diagnosis was not established. The management of ectropion usually aims at prevention of keratitis and corneal ulcer formation. This can be achieved initially by application of artificial tears and local antibiotics. In severe cases, surgical management is necessary to correct the defect. In this case, the condition of the patient was stable on treatment with artificial tears, hence surgery was not undertaken.

Links:

• Congenital Ectropion - Indian pediatrics