Coats' disease is a nonhereditary ocular disease, with no systemic manifestation, first described by George Coats in 1908. It occurs more commonly in children and has a clear male predominance (69%). Most patients present clinically with unilateral decreased vision, strabismus, or leukocoria
. The most important differential diagnosis is unilateral retinoblastoma, which occurs in the same age group and has some overlapping clinical manifestations., Coats' disease is an idiopathic condition characterized by telangiectatic and aneurysmal retinal vessels and chronic accumulation of subretinal exudates. It is predominantly unilateral occurring mostly in young males, and can cause severe visual loss resulting from exudative retinal detachment.
The most common sign of retinoblastoma is white pupillary reflex, known as leukocoria
. True or false?
Imaging modalities for leukocoria
include B-scan ultrasound (US), computed tomography (CT), and magnetic resonance (MR); however, MR is commonly employed for detailed evaluation of the intraorbital contents.
Clinical examination signs that should raise concern include leukocoria
(white pupil), strabismus, restriction of ocular motility, asymmetric eye position within the orbit, decreased vision, high pressure in the eye, inflammation of the eyelids or conjunctiva, pseudohypopyon (inferior whitish layer in the anterior chamber of tumor cells), vitreous hemorrhage or inflammation, and an afferent pupillary defect.
Problems indicated by this white reflex (leukocoria
) can include among other conditions cataracts, congenital conditions or eye cancer.
Signs and symptoms at presentation were available in all cases: 47 patients (61%) had leukocoria
, 30 patients (39%) had proptosis, 16 patients (20.8%) had strabismus, 8 patients (10.4%) had pain and redness, 5 patients (6.5%) presented with an orbital mass leading to proptosis, and 1 patient (1.3%) presented with hyphema (Figure 1, A and B).
An 11-year-old girl presented with unilateral leukocoria
. A CT scan of the brain revealed calcification involving the retina and posterior chamber of the left eyeball.
of Signs/Symptoms patients * (%) ([dagger]) Signs (n = 32) Subretinal granulomatous 20 (65) mass/scar Vitritis 16 (55) Scotoma 10 (50) Posterior pole granuloma 13 (42) Peripheral granuloma with 12 (39) traction bands Active chorioretinitis 11 (34) Retinal detachment 9 (28) Strabismus 8 (27) Anterior uveitis 8 (25) Leukocoria
4 (15) Diffuse nematode endophthalmitis 2 (6) Symptoms (n = 37) Vision loss 25 (83) Permanent 17 (68) Temporary 7 (28) Unknown 1 (<1) Floaters 13 (38) Eye redness 12 (32) Photophobia 10 (27) Eye pain 7 (19) * Because of missing data, the number of respondents for selected characteristics varies.
Fourteen (100%) presented with leukocoria
, classified as VB stage using Rees-Ellsworth classification.
Apart from restoring of his eyesight (retinopathy with leukocoria
and partial retinal detachment), Joel will have to undergo treatment for a hearing impairment and hernia, the spokesperson added.
Most babies/children present with a 'white' reflex, cat's eye, or leukocoria
as the light reflects off the tumour.
The most common symptom of retinoblastoma is leukocoria
, or white eye reflex.