Congenital eyelid imbrication syndrome in a neonate: A rare case
Eyelid imbrication syndrome (EIS) is an extremely rare eyelid malposition disorder, in which the eversion of upper eyelids is seen, sometimes associated with floppy eyelid syndrome. In adults, eyelid imbrication is associated with floppy/lax eyelids, which is usually managed surgically. Till date, five cases have been reported. Here, we describe a case of congenital EIS (CEIS) in a normal healthy newborn presenting with overriding of both upper lids on lower while closure and spontaneous eversion while crying.
A full-term boy was born after an uneventful cesarean section at 39 weeks of pregnancy to primipara non-consanguineous parents, weighing 3000 g, who was referred for routine checkup. He was the first child of the family. The pregnancy course was normal, no antenatal drugs were used except for iron and calcium supplementation. No family history suggestive of any ocular malformations. There were no dysmorphic facies or neurocutaneous markers in the baby.
Ocular examination of the baby showed elongated upper lids [Figure 1a] and tarsal plates were overlapping the lower lid margins by more than 2 mm. The upper eyelids (left>right) could be everted with minimal effort due its floppiness. The upper eyelids had a tendency for spontaneous eversion on crying [Figure 1b]. The lids could be manually repositioned. The tarsal conjunctiva of both eyelids showed hyperemia. Pediatric ophthalmologist opinion was taken by teleconsultation and advised topical lubricants. After 48 h, when called for review, there was marked improvement in lid position with decreased overriding and no spontaneous eversion.
The scarce literature of CEIS was due to its spontaneous recovery. This congenital condition is rare in children and is self-limiting and is associated with lax eyelids, whereas acquired condition requires surgical correction. CEIS is frequently associated with congenital floppy eyelid syndrome (CFES).[3,4] There was found to be association with Down syndrome, but this baby did not have those features.
Our case was similar to that of the case reported by Odat and Hina in 2009. He thought that postnatal growth of the bony orbit may contribute to the spontaneous tightening of canthal tendons. However, the other researchers proposed that whole eyelids were bulky and floppy and underwent involutional changes under the influence of unknown effect in the 1st week of life and that resulted in tightening of laxed canthal tendons and normalization of tone and size of the upper eyelids. The classical feature of floppy eyelid syndrome is spontaneous eversion of the eyelid. In CEIS, spontaneous eversion is directly related to the amount of overriding of upper eyelid over lower. This relationship is documented in Odat and Hina. and Chandravanshi et al. Congenital lax upper eyelid syndrome can be used instead of CEIS/CFES.
CEIS is a rare, transient, self-limiting disorder of unknown cause. The combination of CFES and CEIS should be considered as one of the differential diagnoses of congenital eyelid malposition. A pediatrician should be familiar with CEIS. The floppy eyelid syndrome should be looked for in a case of CEIS.