with

hypopyon , an accumulation of white blood cells in the anterior chamber,

which may herald relapse or involvement of the central nervous system.
The lesion of juvenile xanthogranuloma (nevoxanthoendothelioma; see
Chapter 690 ) may occur in the eye as a yellowish fleshy mass or plaque of the
iris. Spontaneous hyphema (blood in the anterior chamber), glaucoma, or a red
eye with signs of uveitis may be associated. A search for the skin lesions of
xanthogranuloma should be made in any infant or young child with spontaneous
hyphema. In many cases, the ocular lesion responds to topical corticosteroid
therapy.

Leukocoria
This includes any white pupillary reflex, or so-called cat's-eye reflex. Primary
diagnostic considerations in any child with leukocoria are cataract, persistent
hyperplastic primary vitreous, cicatricial retinopathy of prematurity, retinal
detachment, retinoschisis, larval granulomatosis, and retinoblastoma (Fig. 640.7
). Also to be considered are endophthalmitis, organized vitreous hemorrhage,
leukemic ophthalmopathy, exudative retinopathy (as in Coats disease), and less-
common conditions such as medulloepithelioma, massive retinal gliosis, the
retinal pseudotumor of Norrie disease, the so-called pseudoglioma of the Bloch-
Sulzberger syndrome, retinal dysplasia, and the retinal lesions of the
phakomatoses. A white reflex may also be seen with fundus coloboma, large
atrophic chorioretinal scars, and ectopic medullation of retinal nerve fibers.
Leukocoria is an indication for prompt and thorough evaluation .

FIG. 640.7 Red reflex. Normal red reflex in the left eye and white reflex in the right eye.
This patient was later diagnosed with retinoblastoma in the right eye. (From Martin RJ,
Fanaroff AA, Walsch MC editors: Fanaroff & Martin's neonatal-perinatal medicine, ed
10, Vol 2, Philadelphia, 2015, Elsevier/Saunders, Fig. 103.7, p. 1739.)
 The diagnosis can often be made by direct examination of the eye by
ophthalmoscopy and biomicroscopy. Ultrasonographic and radiologic
examinations are often helpful. In some cases, the final diagnosis rests with a
pathologist.

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