A rare case of bilateral congenital upper eyelid eversion managed conservatively.

A 12-h-old female infant was referred to our tertiary care hospital from another hospital for the management of eversion of both upper eyelids. The full-term neonate was born by cesarean section for failed induction of labor. On ocular examination, her upper eyelids were totally everted (left eye more than the right eye) with severe conjunctival chemosis and greenish discharge suggestive of secondary infection [Fig. 1]. After instillation of 0.5% proparacaine eye drops and using Desmarre's lid retractor, the anterior segment was examined which was normal with negative fluorescein staining of the cornea. On laboratory testing, the neonate had C- reactive proteins (CRP) level of 5.1 mg/dL. Elevated total leukocyte count (17,000/μL) with raised total (10.2 mg/dL) and indirect bilirubin (9.46 mg/dL) levels. The neonate was admitted to the neonatal intensive care unit (NICU) for neonatal sepsis and hyperbilirubinemia. The baby was started on systemic antibiotics and phototherapy. For ocular pathology, manual eversion was tried without any success. After that, magnesium sulfate soaked dressings were given to reduce chemosis but there was no positive response. So, we tried 5% hypertonic NaCl soaked dressings every 6 h. This resulted in a reduction in chemosis from day 2 and on day 6 the chemosis was completely reduced with normal closure of the eyelid [Fig. 2]. Along with this, the neonate was also given 0.5% moxifloxacin eye drops and topical 1% carboxyl methylcellulose (CMC) eyedrops every 6 h.

Figure 1

At the time of presentation

Figure 2

Figrue 2: At day 6

Discussion

Bilateral congenital upper eyelid eversion is a rare disorder. Adams, in 1896, first described congenital eversion of both eyelid as “ double congenital ectropion.”[1] In 1992, Sellar reported 51 cases after reviewing the literature.[2] 7 more cases reported till now.[34567] This is seen frequently in infants with Down syndrome, collodion skin disease, and Black race.

The presentation of the disease can be unilateral or bilateral. The exact etiopathogenesis is not known but multiple theories have been proposed to describe its etiology. This includes birth trauma, hypotonia of orbicularis oculi muscle, anterior lamellar shortening and posterior lamellar widening of the eyelid, failure of the orbital septum and levator aponeurosis fusion, elongation of eyelid laterally, and inelastic lateral canthal ligament.[5] The orbicularis spasm leads to venous stasis and conjunctival chemosis thus preventing the cornea from infection and exposure.[8]

In conservative management, various treatment modalities available which include moist dressing, taping of the eyelid, pressure patching, hypertonic saline dressing, topical antibiotics, and lubricants.[89]

The surgical management options include scarification of exposed conjunctiva, temporary tarsorrhaphy, subconjunctival injection of hyaluronic acid, fornix sutures, and full-thickness upper lid skin graft.[5910] But these are used in cases not responding to conservative treatment. Also, lid manipulations can lead to stimulation of autonomic effects such as respiratory arrest in neonates.[3]

Our case was managed with hypertonic saline dressing, antibiotic and lubricating eyedrops. Early treatment can prevent complications like conjunctival scarring, epimerization, and secondary infection. This case report advocates the need for a strictly conservative approach in management.

The aim of this photo essay is to create awareness among those who are first-time viewers like health care professionals in ophthalmology and neonatology.

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Conflicts of interest

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