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Leukocoria, also known as amaurotic cats eye, is an abnormal white reflection from the retina of the eye.

Cataract Retinoblastoma Toxocariasis Coats disease ROP PHPV (Persistent Hyperplastic Primary Vitreus) Norries disease

Retinoblastoma is the most common intraocular tumor of childhood, accounting for 1% of childhood cancer deaths in the United States and 5% of blindness in children. The incidence is 1 in 15,000 to 1 in 20,000 live births.

A malignant tumor of the retina that appears as a white, nodular mass extending into the vitreous (endophytic), as a mass lesion underlying a retinal detachment (exophytic), or as a diffusely spreading lesion simulating uveitis (diffuse infiltrating).

Iris neovascularization is common. Pseudohypopyon and vitreous seeding may occur The eye is normal in size May be bilateral, unilateral, or multifocal Diagnosis is usually made between 12 and 24 months of age A family history may be elicited in about 10%.

CLINICAL MANIFESTATIONS Leukocoria (60%) Strabismus (20%)

OTHER- Uveitis, Orbital cellulitis, Hyphaema, Heterochromia, Glaucoma, Bupthalmos

The disease is bilateral in approximately 30% of cases. The average age at diagnosis is 18 months and 90% of patients are diagnosed before the age of 3 years. Less than 10% of retinoblastoma suffers have a family history of the disorder, 90% of cases are sporadic. Of the sporadic cases, the responsible mutation is in a germ cell in 25% of cases and in a somatic cell in 75% of cases

A nematode infection that may appear as a localized, white, elevated granuloma in the retina or as a diffuse endophthalmitis. Associated with localized inflammation of ocular structures, vitreous traction bands and related macular dragging, traction retinal detachment, and cataract.

It is rarely bilateral and is usually diagnosed between 6 months and 10 years of age. Paracentesis of the anterior chamber may reveal eosinophils; serum enzyme-linked immunosorbent assay (ELISA) test for Toxocara organisms is positive. The patient may have a history of contact with puppies or eating dirt

Toxocariasis is a unilateral disorder that presents as strabismus, leukocoria or decreased vision. Retinal damage is the result of the host's inflammatory response to the single infection nematode, which must usually be dead before the uveitis can develop. The posterior uveitis may be of severe intensity.

Definition:Congenital retinal telangiectasia with vascular anomalies that nearly always presents unilaterally and can lead to

exudation and eventually to exudative retinal

detachment. Symptoms: The early stages are characterized by loss of visual acuity, the later stages by leukocoria or unilateral strabismus, although the combination of leukocoria and strabismus is also possible.

A retinal vascular abnormality resulting in small multifocal outpouchings of the retinal vessels Leukocoria may develop secondary to an exudative retinal detachment or to extensive yellow intraretinal and subretinal exudate. Usually develops in boys during the first two decades of life more severe cases occur in the first decade of life. Coats disease is rarely bilateral No family history

Stage 1 is characterized by abnormal dilation of retinal blood vessels and can be treated with laser photocoagulation or cryotherapy. Vision loss is usually reversible. However, Coats Disease is infrequently diagnosed at Stage 1 because patients are often too young to articulate their symptoms.

Stage 2 is characterized by both telangiectasia and exudation, and is best treated with laser photocoagulation. Visual prognosis is contingent upon the degree of involvement of the fovea, where foveal sparing portends a favorable prognosis.

Exudative retinal detachment characterizes Stage 3. Total retinal detachment characterizes Stage 4. Visual prognosis at this point is poor, and some eyes diagnosed at this stage will eventually require enucleation. Finally, Stage 5 is characterized by irreversible blindness.

A developmental ocular abnormality consisting of a varied degree of glial and vascular proliferation in the vitreous cavity

It is usually associated with a slightly small eye. Typically there is a membrane behind the lens that may place traction on the ciliary processes. This is a progressive condition with a cataract present at birth or early in life. The membrane and lens may rotate anteriorly, shallowing the anterior chamber and resulting in secondary glaucoma. Retinal detachments may be seen. Rarely bilateral No family history.

Opacity of the lens present at birth; may be unilateral or bilateral. There may be a family history or an associated systemic disorder. Congenital cataracts that cause significant visual loss must be detected early Large, dense white cataracts may present as leukocoria (white pupil),

Unilateral infantile cataracts that are dense, central, and larger than 2 mm in diameter will cause permanent deprivation amblyopia if not treated within the first 2 months of life and thus require surgical management on an urgent basis. Symmetric (equally dense) bilateral cataracts may require less urgent management, although bilateral deprivation amblyopia can result from unwarranted delay.

Retinal vascularization is complete by 42 weeks gestational age; thus, the peripheral retina is not fully vascularized at birth. After birth, vascularization of the peripheral retina proceeds normally. However, if this process does not occur, anomalous vessels may grow and result in retinopathy of prematurity.

Predominantly occurs in premature children. Leukocoria is usually the result of a retinal detachment

SI- flat demarcation line with branching blood vessels up to line SII- ridge with volume, blood vessels enter ridge SIII- ridge + extraretinal fibrovascular proliferation SIV- retinal detachment- a (not involving the fovea), b (involving the fovea) SV- total RD

The progressive oculoacousticocerebral degeneration of Norrie, is a rare, X-linked recessive heritable disorder characterized by
Bilateral leukocoria caused by retinal detachment. Affected boys classically have a triad of blindness, deafness, and mental retardation. Apparent at birth or in early infancy, the ocular findings usually progress to phthisis bulbi. An identical disorder in a Maltese kindred is called Episkopi blindness.

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