LEUKOKORIA

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.
The top 4 causes of leukocoria are:
1.
2.
3.
4.

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

HETEROKROMIA
Sindroma Horner Konginetal
Reaksi Radang Sekunder
Clinical Diagnosis
The most common clinical sign of retinoblastoma is leukocoria, which occurs as
the presenting sign in 56% to 62% of cases diagnosed in large series. 78 The next
most common sign is strabismus (20% to 24%), which generally occurs because
of involvement of the macula by tumor or because of retinal detachment related to
tumor. In Abramsons series of 1256 patients, the next most common presenting
signs after leukocoria and strabismus were poor vision (7.7%) and positive family
history (6.8%).
Patients may also present with inflammatory signs, such as an erythematous eye,
or symptoms suggesting orbital cellulitis.78 Although primary care physicians
screen for retinoblastoma in the office by examining for a red reflex, many
patients with retinoblastoma have their leukocoria detected first by family or
friends.79 A dilated funduscopic examination by an ophthalmologist is quite
reliable.
Retinoblastomas may demonstrate a variety of growth patterns: (1) In endophytic
retinoblastoma, cell division and tumor growth take place in the internal retinal
layers and the tumor grows towards the vitreous, with a tendency towards vitreous
seeding. (2) In the exophytic growth pattern, cell division and tumor growth
occurs in the external retinal layers and tumor develops in the subretinal space
(between pigmented epithelium and the sensory epithelium). This growth pattern
often leads to retinal detachment. (3) Tumors may demonstrate a mixed pattern of

endophytic and exophytic growth. (4) Finally, 2% of retinoblastomas display a

diffuse infiltrating pattern, in which tumor grows as a flat layer on or beneath the
retina without obvious mass or calcification. These diffuse infiltrating tumors
progress towards the anterior chamber and may ultimately present with
pseudoinflammatory complications such as pseudohypopyon (simulating pus or
white blood cells in the anterior chamber).65
Penegakan diagnosis CT Scan
More than 90% of retinoblastomas show evidence of calcification on CT (see Fig.
9-30).81 Calcification may be small and single, large and single (Fig. 9-35),
multiple and punctate, or a few fine-speckled foci.82
DD
Coats disease (primary retinal telangiectasis) is a primary vascular anomaly of the
retina characterized by idiopathic retinal telangiectatic and aneurysmal retinal
vessels, with progressive deposition of intraretinal and subretinal proteinaceous
exudates that leads to massive exudative retinal detachment (exudative
retinopathy).121-123 The condition occurs more frequently in juvenile males than in
females. However, it can occur in adults, in whom it is almost always
unilateral.121,122,124,125 The formation of retinal telangiectasia, and the breakdown in
the bloodretinal barrier with leakage of a lipoproteinaceous exudate at the
telangiectasis, are the essential causes of the pathologic changes that occur in
Coats disease
Umur bisa sampe sebelum 20 tahun. Puncaknya pd umur 6-8
Persistent hyperplastic primary vitreous (PHPV) is characterized by a unilateral
leukocoria in a microphthalmic eye of a full-term baby. Rarely, PHPV may be
bilateral (Fig. 9-44).
In a study by Howard and Ellsworth 62 of 500 children with leukocoria, PHPV
accounted for 51 of the 265 nonretinoblastoma cases.
ROP (retrolental fibroplasia, retinal fibroplasia) is seen in premature low-birthweight infants. ROP is usually bilateral and fairly symmetric. The essential feature
of ROP appears to be prematurity. The smaller the infant, the greater the risk of
developing this disease. ROP usually develops as a response to prolonged
exposure to supplemental oxygen therapy.
Ocular toxocariasis is a chorioretinitis caused by an inflammatory response to the
nematode Toxocara canis.102 Infected puppies excrete worm ova that may survive
in soil for years. Ocular toxocariasis is usually unilateral and seen in older

children. Clinically, it may present as endophthalmitis with vitreous haze from a

profound inflammatory response or as a posterior or peripheral retinal granuloma