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Leukocoria

Dr Yuranga Weerakkody and Dr Daksha T. Bhansali et al.

Leukocoria (also spelled as leucocoria / leukokoria) referes to an abnormal white reflection from the retina. Despite its colour, the reflection is related to the familiar red-eye effect. Usually, when a light is shone through the iris, the retina appears red to the observer. In leukocoria, the retina abnormally appears white.

Pathology

Causes

The top 4 causes of leukocoria are:

retinoblastoma : ~ 58%
persistent hyperplastic primary vitreous : ~ 28%
Coats disease : ~ 16%
larval granulomatosis : ~ 16%

As with anything, causes can be considered using a surgical sieve approach

Tumours

Developmental

persistent hyperplastic primary vitreous
Coats disease
retrolental fibroplasia
coloboma of choroid or optic disc
neurocutaneous syndromes
- tuberous sclerosis (TS) (angiomata)
- von Hippel Lindau syndrome (vHL) (choroidal ostema)

Infection

Degenerative

posterior cataract
senesent calcification
optic nerve drusen
phthisis bulbi

Trauma

Associations

Leukocoria can be seen in the normal-sized eye or associated with microphthalmia.

normal sized eye
- calcified : retinoblastoma; retinal astrocytoma
- non-calcified : toxocaral endophthalmitis; Coats disease
microphthalmia
- unilateral : persistent hyperplastic primary vitreous (PHPV)
- bilateral : retinopathy of prematurity; bilateral PHPV

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