The shining eye.

An otherwise healthy 4-year-old boy presented with white reflex in the left eye (OS) of 6 weeks duration [Figure 1]. Visual acuity in the right eye was 20/20 and light perception in OS. Anterior segment examination was unremarkable in both eyes. Intraocular pressures were 10 mm Hg in both eyes. Fundus examination OS revealed total exudative retinal detachment with findings as shown in Figure 1. Ultrasonography OS was done [Figure 2].

Figure 1

(a, b) Clinical photograph and RetCam Fundus image OS

Figure 2

Ultrasound B-scan OS

Questions

Describe fundus photograph

Describe B-scan (longitudinal scan)

Enumerate differential diagnosis for leukocoria in a 4-year-old child

How would you manage this case.

Answers

Fundus photograph (OS) displays total exudative retinal detachment with retinal telangiectasia, aneurysmal dilatation of retinal vessels, and subretinal lipid exudation

Ultrasonography (OS) shows a total exudative retinal detachment with no evidence of intraocular mass

Retinoblastoma, Coats disease, congenital cataract, ocular toxocariasis, retinopathy of prematurity, persistence hyperplastic primary vitreous, medulloepithelioma, congenital toxoplasmosis, and retinal hamartomas

Management would be an observation with the 6-monthly review. These eyes may eventually end up with phthisis bulbi. Enucleation is advised if the patient develops secondary glaucoma or cosmetic concern.

A diagnosis of Coats disease was thus established. Coats disease is the most common (40%) lesion simulating retinoblastoma.[1] Detailed observation of color of the reflex (yellow reflex (xanthocoria) in Coats disease compared to white reflex (leukocoria) in retinoblastoma) and clear vitreous in Coats disease (in contrast to vitreous seeds in retinoblastoma) are other clinical signs which aid in differentiating Coats disease from retinoblastoma.[1] Characteristically, dilated blood vessels with aneurysmal dilatations are seen in Coats disease which are apparent till peripheral retina before their disappearance into the ora serrata. Whereas in retinoblastoma, no aneurysmal dilatation of blood vessels is seen and the feeder vessels disappear beneath the tumor in exophytic variety. Relatively older age at presentation, characteristic vascular changes, and absence of intraocular mass in Coats disease are helpful in differentiating it from retinoblastoma.[12]

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