Jairos C. Fumpa1,2 and Kangwa I. M. Muma1,3
1Department of Ophthalmology, School of Medicine and Clinical Sciences, Levy Mwanawasa Medical University, Lusaka, Zambia
2Mukinge Mission Hospital, Kasempa, Zambia
3University Teaching Hospitals – Eye Hospital, Lusaka, Zambia
Congenital eversion of the upper eyelids (congenital ectropion) is a rare condition. Most of the cases are bilateral, but unilateral cases also have been reported. It does not seem to be caused by difficult labour through the birth passage. At Lumwana District Hospital, a day-old female neonate presented with ectropion on both eyes at birth. The neonate was born at term, by Spontaneous Vaginal Delivery from a multiparous mother. Birth history was uneventful, although bilateral upper eyelid ectropion was immediately noted. The neonate was taken to the eye clinic for further management.
Ectropion of eyelids refers to a condition where the eyelids are turned outwards away from the globe . This is a common occurrence in the elderly although there are a number of causes such as stroke, skin cancer, injury, scar tissue from injuries or burns, growths on the eyelid (either cancerous or benign) birth defects (due to genetic disorders such as Down syndrome) Bell’s palsy (a condition that damages the nerve that controls facial muscles) or other types of facial paralysis . In the case of a new born baby, it is more frequently associated with Down’s syndrome and ichthyosis .
Congenital bilateral ectropion of the upper eyelids is a rare, benign condition [3, 4, and 5]. The eversion usually presents at birth and resolves spontaneously within two weeks of birth [3, 4, 5]. Its etiology is unknown and several possible mechanisms have been proposed, however it is frequently associated with Down’s syndrome, ichthyosis, and newborns in the black population [1, 2, 4].
A one-day old neonate was brought to the eye clinic at Lumwana District Hospital by the mother with complaints of swelling of both eyes and outward turning of eye lids with reddening of both eyes since birth. Further history and relevant information was obtained from the attending mid-wife who reported that, APGAR score was 9, born at term with birth weight of 3.1kgs, from a primigravida aged 18 years of age. The attending mid-wife further reported that, the patient was delivered through spontaneous vagina delivery and the pregnancy was uneventful. There was no history of discharge from both eyes. Credé’s prophylaxis using Tetracycline Eye Ointment (TEO) was conducted by the midwife immediately after the neonate was born. Consent for photos and publication was obtained from the mother.
On examination, the general condition was good, afebrile (body temperature 370C), not pale, not jaundiced and had no respiratory distress. There were no other abnormalities noted in the neonate systemically. Brisk reflex was present on both eyes. The cornea and the rest of the anterior segment examination were found normal on both eyes. However both eyes had ectropion with subconjunctival haemorrhage and chemosis as shown in figure 1.
The neonate patient was admitted, and the mother was reassured and counselled on the diagnosis and prognosis. Apart from bilateral ectropion and associated findings, there was no evidence of any other abnormality on both eyeballs. The patient was treated conservatively. Tetracycline eye ointment (TEO) and systemic antibiotics were given as prophylactic management for subconjunctival haemorrhage and chemosis. To correct the ectropion, the eyelids put in the correct position, TEO 1% applied then padded with wet gauze.
After 48 hours, the bilateral ectropion resolved as shown in figure 2 and the neonate was discharged. Subsequent follow up review at 2 weeks later on showed that the ectropion had completely resolved with eyeballs noted to be normal and the neonate could fixate to light.
Figure 1: Clinical appearance of the patient’s eyes with ectropion
Figure 2: Clinical appearance of the patient’s eyes with resolved ectropion within 48hrs
Congenital ectropion of the upper eyelid is a rare abnormality that can threaten the cornea and vision if not treated early. At Lumwana, the neonate who presented with bilateral ectropion of upper eyelids was treated conservatively as other causes of congenital ectropion were ruled out. Congenital ectropion of the upper eyelids was first described by Adams in 1896 [3 -5]. Later, Gilbert and co-workers described two more cases associated with Down’s syndrome [6-7]. This rare condition has been reported more frequently in black infants [1-8] associated with ichthyosis [1-4] and in infants with trisomy 21 . Although the condition is generally bilateral and asymmetrical, some unilateral cases have been described . The neonate in this case report had bilateral but asymmetrical ectropion. Down’s syndrome encompasses numerous ocular abnormalities like myopia, keratoconus, nystagmus, epiblepharon, epicanthus, convergent strabismus, cataracts, blepharoconjuctivitis with the epicanthal folds, and the typical mongoloid slant to the eyelid fissures being the most obvious periocular findings . None of these reported ocular abnormalities were found in the neonate. Essentially this neonate was normal.
Although the pathophysiology of congenital upper eyelid ectropion is unknown, multiple factors have been implied, including absence of an effective lateral canthal ligaments, lateral elongation of the eyelid, hypotonia of the orbicularis, vertical shortening of the anterior lamella, and failure of the orbital septum to fuse with the levator aponeurosis [1-6]. Treatment of congenital upper eyelid ectropion is controversial. Surgical treatment options that can be employed in the management of severe cases of congenital ectropion include tarsorraphy only [2-7], tarsorraphy with excision of redundant conjunctiva [5,7], fornix suture , full-thickness skin graft [1-5], full-thickness horizontal lid shortening [2,6] and attachment of the orbital septum to the levator aponeurosis .
In this case under review, the patient did fulfill the conservative treatment parameters due to the absence of other congenital abnormalities of the eyelids that may occur in Down’s syndrome. Therefore, a simple and conservative management with lubricants / antibiotic ointment and moist swabs were enough to prevent desiccation of the exposed conjunctiva, reduction of conjunctival edema and to allow spontaneous inversion of the eyelid within 48 hours. The fact that, the child responded to conservative treatment within 48 hours, the risk of amblyopia was removed.
Therefore, in the presented case where the eyelids were repositioned mechanically, eyelids taped down, eye ointment applied and eyes padded, we recommend conservative treatment as opposed to surgical intervention.
Though congenital bilateral upper eyelid ectropion is unusual, when it occurs, it can be conservatively managed with full resolution within 2 weeks. Not all bilateral ectropion of both upper eyelids at birth can be associated with Down’s syndrome, ichthyosis and other causes known.
- Orbit, Eyelids and Lacrimal System, Section 7. Basic and Clinical Science Course, AAO, 2011-2012
- Fasina O. (2013): Management of bilateral congenital upper eyelid eversion with severe chemosis. J Ophthalmic Vis Res. Apr;8 (2):175–178
- Alvarez EV, Wakakura M, Alvarez EI. Surgical management of persistent congenital eversion of the upper eyelids. Ann Ophthalmol. 1988 Sep;20(9):353–4, 357
- Surgical management of congenital upper-eyelid eversion.Loeffler M, Hornblass A Ophthalmic Surg. 1990 Jun; 21(6):435-7.
- Maheshwari R, Maheshwari S. Congenital eversion of upper eyelids: case report and management. Indian J Ophthalmol. 2006 Sep;54(3):203–204
- Suliman S, Michie C. A case of bilateral congenital eublepharon or ectropion. West Lond Med J. 2010;2(4):37–41
- Omolase CO, Ogunleye OT, Omolase BO, Ogendengbe A. Conservative management of congenital eversion of the upper lid in a Nigerian child. Pak J Ophthalmol. 2012;28(4):222–223
- Congenital bilateral ectropion in lamellar ichthyosis. Chakraborti C, Tripathi P, Bandopadhyay G, MazumderDB ,Oman J Ophthalmol. 2011 Jan; 4(1):35-6.