Leukocoria

Presented By Asa Rairisti I11108052

Definition
Leukocoria may be a common sign in various ocular conditions and may be characterized by a whitish pupillary reflex (leukos: white, kore: pupil) that differs from the normal red ocular reflex.

Shields JA, Shields CL, Parsons HM. Retina 1991;11:232-43

Congenital Cataract
Opacity of the lens at birth, either hereditary or acquired throughout the placenta Signs : Leukocoria Nystagmus Strabismus Blunted red reflex

etiology
Idiopathic (most common). Familial, autosomal dominant. Galactosemia: Cataract may be the sole manifestation when galactokinase deficiency is responsible. PHPV: Unilateral. The involved eye is usually slightly smaller than the normal fellow eye. Rubella: Pearly white nuclear cataract, salt-and-pepper chorioretinitis, microphthalmos, corneal clouding, and poorly dilating pupils. Associated hearing defects and heart abnormalities are common. Lowe syndrome (oculocerebrorenal syndrome): Opaque lens, congenital glaucoma, renal disease, and mental retardation. Xlinked recessive. Others: Chromosomal disorders, systemic syndromes, other intrauterine infections, trauma, drugs, other metabolic abnormalities, aniridia, anterior segment dysgenesis, radiation.

Type of Cataract

Coronary cataract : Single or multiple finger- or bowling pinshaped opacities that ring the peripheral cortex

Cerulean cataract : Small, bluish punctate opacities of the peripheral cortex (anterior, posterior or both)

Treatment
Referral to a pediatrician to treat any underlying disorder. Treat associated ocular diseases. Cataract extraction, Treat amblyopia in children younger than 9 to 11 years A dilating agent (e.g., phenylephrine 2.5%, t.i.d., homatropine 2% t.i.d., or scopolamine 0.25% q.d.) may be used as a temporizing measure, allowing peripheral light rays to pass around the lens opacity and reach the retina. This rarely is successful longterm.

Retinoblastoma
Retinoblastoma is a tumor that originates from immature retinoblasts in the neural retina; it is the most frequent intraocular tumor in children. The incidence ranges from 1/14,000 to 1/20,000 live births, depending on the country Over 90% of cases are diagnosed before age 5 years

Retinoblastoma2
Affects infants and children. Leukokoria in one or both eyes. White retinal tumor fed and drained by dilated, tortuous retinal blood vessels.

Well-established tendency to invade optic nerve and choroid, extend extrasclerally, invade the brain, and metastasize.
Unilateral (6070%) or bilateral (3040%) ocular involvement; most unilateral cases are unifocal, and most bilateral cases are multifocal in both eyes. Germinal (heritable, 40%) and somatic (nonheritable, 60%) forms. Autosomal dominant inheritance pattern in germinal cases. Attributable to loss or inactivation of both alleles of retinoblastoma gene, a tumor suppressor gene on the long arm of chromosome 13.

Clinical presentations
leukocoria (from 60 to 80% of cases) Strabismus red eyes enlarged ocular globe due to increased intraocular pressure loss of vision Less common forms are : aseptic orbit cellulitis due to tumor necrosis, altered color of the iris (heterochromia), intraocular bleeding and pseudouveitis.

Diagnosis
Family history Ophthalmoscopy A grayish white, vascularized retinal tumor CT scan detecting calcium within the tumor Ultrasoundthe size of the lesions and may find calcification within the tumor MRI extraocular involvement and invasion of the optic nerve

Treatment
Intravenou chemotherapy Enucleation Radiation therapy External beam radiation therapy Plaque radiotherapy Laser therapy Photocoagulation Transpupillary thermotherapy (TTT) Cryotherapy Observation (for spontaneously arrested retinoblastoma, retinoma)

RETINOPATHY OF PREMATURITY (ROP)

Vasoproliferative retinopathy affecting premature infants exposed to high oxygen ROP affects immature blood vessels of the retina. It occurs weeks after birth
INCIDENCE 47% in infants with birth weights between 1000 and 1251g , 81.6% for infants weighing less than 1000g (2lb3oz) at birth. 80% of infants born at less than 28 weeks gestational age, 60% of infants born at 28-31weeks developed ROP Oxygen duration

RETINOPATHY OF PREMATURITY (ROP)

LOCATION zone 1 - centred on disc, 2x disc to fovea distance zone 2 - outer limit equator temporally, ora nasally zone 3 - temporal peripheral crescent

1. Screening birth weight less than 1500g or a gestational age of 32weeks or less, as well as in infants weighing between 1500g and 2000g with an unstable clinical course and who are believed to be at high risk. 2. immature retinas (no ROP) vascularized into zone 2 or 3 may be examined at 2week intervals 3. type 2 prethreshold disease weekly or twice weekly exams 4. type 1 prethreshold disease peripheral laser ablation. 5. Stages 4 and 5: Surgical repair of retinal detachment by scleral buckling, vitrectomy surgery, or both

Coats Disease
A localized, congenital, retinal vascular disorder consisting of abnormal telangiectatic segments of blood vessels that result in leakage

Characteristic
Retinal telangiectasia Retinal capillary nonperfusion Dilated intercapillary spaces. Lipid exudate Leukokoria Subretinal fluid Usually unilateral Male predominance Fibrovascular macular scars

Diagnose
Clinical manifestation : poor vision Strabismus Leukokoria Examination : ophthalmoscopically vascular abnormalities are seen in association with lipid deposition and subretinal exudate Ancillary testingFluorescein angiography is a useful tool for delineating the nature and extent of the vascular abnormalities present in this disease.

Classification
Shield et al : Stage 1 : retinal telangiectasia only Stage 2 : telangiectasia & exudation

Stage 3 : exudative retinal detachment Stage 4 : total retinal detachment & glaucoma Stage 5 : advanced end-stage disease
A : Subtotal retinal detachment B : Total retinal detachment

A : extrafoveal exudation B : foveal exudation

Treatment The main goal : to obliterate the telangiectasiae to facilitate the reabsorption of exudates and maintain as much visual acuity as possible Laser photocoagulation or cryotherapy to destroy anomalous vasculature. intravitreal proliferation and traction detachment vitreous surgery may improve the clinical course

Prognosis
can be measured in terms of two endpoints: visual acuity and anatomical stability central visual acuity is frequently poor in macular lipid deposition. retaining anatomical integrity of the globe is much better. The worst outcomes are in juvenile cases of total retinal detachment.

PHPV jurnal
developmental malformation of the eye where the embryological primary vitreous and hyaloid vasculature fail to regress. Persistent hyperplastic primary vitreous (PHPV) is a rare,non-hereditary, It presents unilaterally in 90% of cases and can be associated with microphthalmia and cataract formation. It is usually not associated with other congenital defects. In the majority of cases the fibrovascular membrane, fed by the persistent hyaloid artery, adheres to the posterior capsule of the crystalline lens.

 A gray-yellow retrolental membrane may produce leukocoria, with the subsequent suspicion of retinoblastoma.

PHPV
Diagnostic Consideration Based on the characteristic clinical picture (symptoms and findings) and additional ultrasound studies (when the posterior segment is obscured by lens opacities)
Treatment Treatment options include cataract surgery and removal of the fibrovascular membrane.

Ocular Toxocariasis Handbook of Pediatric

Ocular toxocariasis is a unilateral disorder that presents as strabismus, leukocoria or decreased vision. May present as posterior chorioretinitis, peripheral chorioretinitis, papillitis, neuroretinitis, endophthalmitis, motile chorioretinal nematode or diffuse unilateral subacute neuroretinitis, Keratitis, conjunctivitis and lens involvement have also been described

INCIDENCE Toxocariasis is the most common nematode infection affecting the eye in the United States; however, the exact incidence of ocular toxocariasis in unknown.

ETIOLOGY Most cases of human toxocariasis are caused by infection with the dog intestinal roundworm Toxocara canis or, rarely, the cat.Ocular toxocariasis results from invasion of the eye by the second- or third-stage larva of the nematode.

Clinical Features
80% less than 16 years. Another common manifestation is chronic endophthalmitis. often present anterior uveitis, hypopyn, posterior synechiae, cyclitic membrane, vitritis, and retinal detachment. Leukocoria because of the severe inammatory reaction decreased visual Acuity amblyopia, strabismus as a result of damage to the macula.

Systemic manifestation
known as visceral larval migrans (VLM). occurs most commonly in children less than 6 years eosinophilia, fever hepatosplenomegaly If ocular disease and VLM are both present, u-like symptoms Less common manifestations pulmonary symptoms, splenomegaly, and seizures.

 The most common presentation is a unilateral granuloma of the posterior pole or peripheral retina typically round, elevated, andup to two disc diameters in size Vitritis Peripheral granuloma hazy white elevated lesions located in the peripheral retina. Peripheral granuloma located in the inferior retina may resemble pars planitis in some children Vitreous membranes are often visible radiating from the peripheral lesion and may form radial retinal folds extending to the optic nerve head.

Toxocara canis- elevated granuloma

Toxocara canis retinitis.

Diagnosis
Diagnosis is based upon clinical features observed in a young patient and should be confirmed at least by the presence of specific IgG in the serum (ELISA test, 90% specificity and 91% sensibility).

Treatment
based on : severity of inammation, macular involvement, and the visual potential of the eye. periocular or systemic corticosteroids active vitritis topical corticosteroids and cycloplegic agents anterior uveitis, Thiabendazole at 50mg/kg/day for 7 days may be considered in children who fail to respond to systemic corticosteroid therapy antihelminthic therapy potential toxicity Surgical procedures refractory vitritis, vitreous membranes, epiretinal membrane, and traction or rhegmatogenous retinal detachment.

Retinal detachment
Retinal detachment is a disorder of the eye in which the retina peels away from its underlying layer of support tissue incidence : 1:10 000 Variety of ocular and systemic disorders are associated with pathological vitreous liquefaction, premature vitreous detachment, and extensive sites of vitreoretinal adhesion

Pathophysiology
extensive liquefaction within the vitreous cavity reduction in both the shock-absorbing capabilities and the stability of the gel associated by aging Accelerated vitreous liquefaction : significant myopia post cataract surgery (40%) severe ocular trauma (10-15 %) intraocular inflammation a variety of other congenital, inherited, or acquired ocular disorders.

Pathology

Classical pathogenesis of rhegmatogenous retinal detachment. The detached vitreous gel has caused a retinal tear by exerting traction upon the retina at the site of a vitreoretinal adhesion. Liquid in the vitreous cavity passes through the break into the subretinal space.

 Rhegmatous retinal detachment Traction retinal detachment

Exudative retinal detachment

Combined retinal detachment

Treatment
scleral buckling techniques and the creation of a chorioretinal adhesion around each break to eliminate and counteract vitreoretinal traction Vitrectomy techniques Pneumatic retinopexy

Optic Disk Coloboma

An optic disk coloboma is the result of incomplete closure of the embryonic optic cup. The optic disk is enlarged with a funnel-shaped depression with whitish tissue and a peripapillary pigment ring. The retinal vessels extend outward across the margin of the disk in a radial pattern without a central trunk vessel. Patients with optic disk coloboma often have decreased visual acuity and visual field defects.

 retinochoroidal colobomas that has received particular attention CHARGE syndrome (coloboma, heart disease, atresia choanae, retarded growth, genital hypoplasia, ear anomalies, with or without deafness

Norrie disease
Norrie disease, or 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.

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