LEUKOKORIA

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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. retinoblastoma : ~ 58% 2. persistent hyperplastic primary vitreous : ~ 28% 3. Coats disease : ~ 16% 4. 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 Abramson’s 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

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Transcript of LEUKOKORIA

LEUKOKORIALeukocoria(also spelled asleucocoria / leukokoria) referes to an abnormal white reflection from theretina. 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. retinoblastoma: ~ 58%2. persistent hyperplastic primary vitreous: ~ 28%3. Coats disease: ~ 16%4. larval granulomatosis: ~ 16%

HETEROKROMIASindroma Horner KonginetalReaksi Radang SekunderClinical DiagnosisThe most common clinical sign of retinoblastoma is leukocoria, which occurs as the presenting sign in 56% to 62% of cases diagnosed in large series.78The 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.78Although 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.79A 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 ScanMore than 90% of retinoblastomas show evidence of calcification on CT (seeFig. 9-30).81Calcification may be small and single, large and single (Fig. 9-35), multiple and punctate, or a few fine-speckled foci.82

DDCoats 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).121-123The condition occurs more frequently in juvenile males than in females. However, it can occur in adults, in whom it is almost always unilateral.121,122,124,125The 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 diseaseUmur bisa sampe sebelum 20 tahun. Puncaknya pd umur 6-8Persistent 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 Ellsworth62of 500 children with leukocoria, PHPV accounted for 51 of the 265 nonretinoblastoma cases.

ROP (retrolental fibroplasia, retinal fibroplasia) is seen in premature low-birth-weight 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 nematodeToxocara canis.102Infected 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