LEUKOCORIA

DEFINITIONLeukocoria

A white pupillary reflex (leukos: white, kore: pupil), pink-white, or yellow-white pupillary reflection.

Normal reflex red reflex

The red reflex refers to the reddish-orange reflection of light from the eye's retina that is observed when using an ophthalmoscope or retinoscope from approximately 30 cm / 1 foot. This examination is usually performed in a dimly lit or dark room.

Normal red reflex

Leukocoria bilateral Leukocoria unilateral

Diferential diagnosis of leukocoria: Cataract Retinoblastoma Toxocariasis Coat’s disease Retinopathy of prematurity (ROP)

Persistent hyperplastic primary vitreous (PHPV)

Retinal detachment Norrie’s disease

CATARACTCataract any opacity in the lens Factors : aging, trauma, toxins, systemic disease (such as diabetes), smoking, and heredity.

Childhood congenital cataractsign : leukocoria absent red pupillary reflex abnormal eye movements (nystagmus)

Strabismus

RETINOBLASTOMARetinoblastoma is the most common intraocular malignant neoplasm of childhood affecting 1:20,000 to 1:34,000 children. The tumor occurs most frequently within the first 2 years of life and may even be found at birth. Signs: leukocoria, strabismus, poor vision, spontaneous hyphema, or a red, painful eye.

Complication : Secondary glaucoma

RETINOBLASTOMAA B

C A. Leukocoria in the left eye

B. This surgically excised eye is almost filled by a cream-colored intraocular retinoblastom

C. retinoblastoma showing Flexner-Wintersteiner rosettes

RETINOBLASTOMA

Typical appearance of intraretinal retinoblastoma. Opaque, yellow-white macular tumor fed and drained by dilated, tortuous retinal blood vessels.

RETINOBLASTOMAA B

A. B-scan ultrasonography of retinoblastoma. Solid, posterior intraocular mass contains strong particulate reflections attributable to intralesional calcification.

B. Computed tomography of bilateral intraocular retinoblastoma. Intraocular masses appear bright because of intralesional calcification.

RETINOBLASTOMATreatment option:• Intravenous chemotherapy  •  Enucleation    • Radiation therapy    • Lasertherapy     • Cryotherapy

RETINOBLASTOMA - Chemotherapy

PRETREATMENTSame lesion after

two cycles of chemotherapy

TOXOCARIASISToxocariasis a nematode infection (Toxocara canis)

Retinal damage is the result of the host's inflammatory response (severe eosinophilic reaction) that becomes granulomatous

The patient may have a history of contact with puppies or eating dirt.

TOXOCARIASISPresent :Occular symptoms : redness, photophobia & pain.Leukocoria, strabismus, decreased vision.

Ophthalmic physical examination :A large peripheral (anterior) retinal or choroid inflammatory mass, associated with a vitreoretinal reaction secondary retinal detachment.

TOXOCARIASIS

A white intravitreous mass of a toxocaral eosinophilic granuloma

COAT’S DISEASECongenital retinal telangiectasis (Coats' disease) is an idiopathic retinal vascular disorder that usually affects young male patients unilaterally in their first or second decade of life. No tumor mass. Retinal telangiectasia.    Retinal capillary nonperfusion.    Dilated intercapillary spaces.    Lipid exudate.    Subretinal fluid Retinal detachment

COAT’S DISEASEA

A. Coat’s disease in childrenB. vascular abnormalities with

aneurysmal dilatation, telangiectasia, exudation, and severe lipid deposition in the macula.

B

COAT’S DISEASE - Treatment

Photocoagulation Cryotherapy

Enucleated later stage severe

Obliterate telangiectasia

RETINOPATHY OF PREMATURITY (ROP)

Vasoproliferative retinopathy affecting premature infants exposed to high oxygen

INCIDENCE Prematurity (<32/40 weeks) Birth weight (30% < 1000gm affected) Exposed to high oxygen 90% ROP regresses spontaneously, 5%

blindness

RETINOPATHY OF PREMATURITY (ROP)

early stages of ROP a band of glomeruloid capillaries proliferates at the junction between the peripheral nonperfused and the posterior perfused retina break through the internal limiting membrane and invade the vitreous, inciting fibrosis and contractionthe retina is folded on itself by the organized vitreous, forming a fibroneural mass that drags the macula and optic disc temporally The end stage total retinal detachment, leukocoria, blindness, and phthisis bulbi.

SCREENING Indications Almost all infants with ROP have a gestational age

of 31 weeks or less (regardless of birth weight) or a birth weight of 1250 g or less; these indications are generally used to decide whether a baby should be screened for ROP,

Any premature baby with severe illness in perinatal period (Respiratory distress syndrome, sepsis, blood transfusion, Intra ventricular haemorrhage, apnoeic episodes, etc.) may also be offered ROP screening.

Timing Retinal examination with scleral depression is

generally recommended for patients born before 30–32 weeks gestation, or 4–6 weeks of life, whichever is later. It is then repeated every 1–3 weeks until vascularization is complete (or until disease progression mandates treatment).

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

Staging (ROP) : 1

Stage 1:The flat, white border between avascular and vascular retina seen superiorly is called a demarcation line.

Staging (ROP) : 2

Stage 2:The elevated mesenchymal ridge has height. Highly arborized blood vessels from the vascularized retina dive into the ridge.

Staging (ROP) : 3

Stage 3: Vessels on top of the ridge project into the vitreous cavity. This extraretinal proliferation carries with it a fibrovascular membrane

RETINOPATHY OF PREMATURITY (ROP)

Treatment :• Laser photocoagulation• Cryotherapy• Vitrectomy surgery

PERSISTENT HYPERPLASTIC PRIMARY VITREOUS (PHPV)

Persistent hyperplastic primary vitreous (PHPV) is a congenital anomaly in which the primary vitreous fails to regress in utero. Highly vascular mesenchymal tissue nurtures the developing lens during intrauterine life. In PHPV, the mesenchymal tissue (fibrovascular) forms a mass behind the lens.

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

PERSISTENT HYPERPLASTIC PRIMARY VITREOUS (PHPV)Sign :

Leukocoria Small eye (microphthalmia) Episodes of intraocular

(intravitreous) hemorrhage. Retinal detachment traction

from fibrovascular mass.

NORRIE’S DISEASENorrie 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.

Norrie’s disease usually with PHPV bilateral.

RETINAL DETACHMENTA retinal detachment (RD) describes the separation of the neurosensory retina (NSR) from the retinal pigment epithelium (RPE) caused by a breakdown of the forces that attach the NSR to the RPE.

Toxocariasis, PHPV, ROP, Coat’s disease

RETINAL DETACHMENT

Treatment:•Cryotherapy• laser photocoagulation using a slit-lamp delivery system and• laser using the indirect ophthalmoscopic delivery system combined with scleral indentation.

CONCLUSION

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