Ophthalmic emergencies in infants and children

Assessment of the red reflex is absolutely fundamental and should be performed as soon as possible after birth. This is best done in a dimly lit room using a direct ophthalmoscope set at +4 diopters at a working distance of 30.48 cm to 45.72 cm (12 inches to 18 inches) to illuminate both pupils simultaneously (Figure 1). The reflex should be identical in each eye and any difference found is cause for immediate referral. Assessment in heavily pigmented infants may be difficult, in which case the pupils can be dilated with 2.5% phenylephrine drops (Mydfrin [Alcon, USA]) instilled twice, 5 min apart. Parents may also be instructed to photograph their babies in a dimly lit room with the red eye reduction feature of the camera turned off, and to report any differences immediately. Assessment of the red reflex in the operating room showing retinoblastoma The reason that timing is so crucial is that the window of opportunity to treat infants all but closes at three months of age. If the visual axis has not been cleared and optical rehabilitation begun by this age, irreversible deprivational amblyopia occurs in the visual pathways and cortex, dooming salvage of useful vision to failure. The following are some threatening conditions. Cataracts in an infant may present with a white pupil (leukocoria) (Figure 2), may be unilateral or bilateral, and are often noted early on by the parents as they feed their child. Urgent referral is indicated because timing is of the essence. Treatment consists of surgical removal of the cataract, subsequent treatment with a contact lens or spectacles and aggressive occlusion therapy of the sound eye in unilateral cases. This must all be underway by three months of age. A congenital cataract causing leukocoria Persistent hyperplastic primary vitreous, also called persistent fetal vasculature, is caused by a lack of differentiation of the tertiary vitreous in embryonic life. It is congenital, almost always unilateral and the affected eye is smaller, so any difference in the size of the globes is worrisome. Treatment is similar to a unilateral congenital cataract and includes a vitrectomy. Retinoblastoma may be present at birth but usually develops in the first two years of life (Figure 3). Ninety-two per cent of cases are unilateral and sporadic while 8% are bilateral and inherited as an autosomal dominant. The tumour usually presents with leukocoria, and often the eye is turned outward (exodeviated) and is frequently seen in photographs. Treatment consists of a combination of chemotherapy, photocoagulation or enucleation. Retinoblastoma Congenital corneal opacity presents with opacification of some or all of the cornea, and the three-month timeline also applies here. Treatment consists of corneal grafting, occlusion therapy of the good eye and possibly spectacles. Glaucoma and cataracts can be a concurrent problem with some forms of congenital corneal opacity. Congenital glaucoma may be sporadic or inherited as an autosomal dominant condition. Characteristically, it is not present at birth and the affected child is described as having ‘big beautiful eyes’ (Figure 4), which should sound an alarm, particularly if the eyes are of different sizes. These children present with constant tearing and photophobia caused by corneal edema, and may be intolerant even of ordinary room illumination. Discharge is not a feature. Treatment is usually surgical. Large eyes caused by congenital glaucoma Warning signs of serious ophthalmic disease in an infant, prompting urgent referrals, include: Difference in the red reflex, either congenital or acquired; Constant strabismus, notably exotropia; Leukocoria; A difference in the size of the globes, either congenital or acquired; and Tearing and photophobia without associated discharge.

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