1 post published by pediatriceducationmin on January 2, There are many causes of leukocoria in children (table 1); the differential diagnosis can be narrowed through a complete clinical and family. Differential Diagnosis of Leukocoria. Jerry A. Shields and Carol L. Shields. ETINOBLASTOMA is a highly malignant. R intraocular tumor of childhood that re-.

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Clinical characteristics include three major features: PHPV is a congenital malformation caused by the arrest of normal regression of the embryonic vascular connective tissue hyaloid artery, leukocooria hyaloidea propria, tunica vasculosa lentisregression that normally occurs after 4 months gestation.

This site uses cookies to provide, maintain and improve your experience. In all respects, retinoma resembles a retinoblastoma sterilized by radiotherapy, without ever having undergone any treatment whatsoever. Essential and accurate information about the differrential is gathered.

Retinoblastoma and the Differential Diagnosis of Leukocoria. Retinal Disorders and Eye Diseases. It is important to note the presence of afferent pupillary defect, which may be a poor prognostic sign. All medical and invasive procedures considered essential for the area of practice are competency performed. Presenting signs of retinoblastoma.

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Inflammatory conditions, in particular ocular toxocariasis, can lead to complex clinical features requiring a whole battery of investigations, including serology, ultrasonography, and neuro-diagnostic tools. At follow-up two months later, the surgical correction was intact and he had a myopia which was being treated with glasses.

These children can be cured, however, at low cost and with minimal suffering, provided that treatment is given in the early stages of disease.

The tumor may be polycystic and cystic fragments may be found liberated into the aqueous humor or vitreous body. Thus the importance of keeping in mind the possibility of retinoblastoma when confronted with suspicious signs such as a white pupil or leukocoria Figure1athe most common first manifestation, strabismus or any other unexplained signs.

Can be associated with choroidal coloboma.

Coats disease appears as a hyperintense subretinal exudate. Both endo- and exophytic forms are likely to develop calcifications, in the form of chalky white patches within the tumoral mass. Retinoblastoma occurs as rarely as in 1: Retinoblastoma Augsburger et al ; Abramson et al ; Balmer and Munier a ; Aerts et al ; Bornfeld et al is a malignant tumor of the retina resulting from a double oncogenic mutation between the beginning of the 3rd month of gestation and 4 years of age in an immature retinal cell, most likely a multipotent retinoblast Munierprecursor cone cell.


Retinal detachment from any other etiology e. Endophytic retinoblastoma shows a marked tendency to invasion of the vitreous by seeding. The primary lesion is most likely a localized, evolutive abnormality of the developing retinal vessels. Affecting very young infants, in most cases perfectly healthy in all other respects, this highly malignant cancer of the retina threatens those individuals with the longest potential lifespan.

In preverbal children, Teller visual acuity measurements can be obtained. An investigatory and analytic thinking approach to the clinical situation is demonstrated.

Clinical Sports Medicine Collection.

The differential diagnosis of leukocoria includes: Leukocoria is a sign observed by the naked eye or with a scope, often detected incidentally on routine eye examination, or in photographs; it is not a diagnosis.

Occasionally, a retinal leukcooria may be seen. The role of the primary care physician Whether the family doctor or pediatrician, the primary care physician is usually the first to be differentoal with the problem and thus plays an essential role, as the evolution and ultimate outcome of the disease will depend on the speed of reaction at this initial stage.

Differential diagnosis of leukocoria and strabismus, first presenting signs of retinoblastoma

Onset of the disease may thus occur in utero Maat-Kievit et al and up to 4 years of age, with a mean age of appearance of first signs of 7 months for bilateral cases, 24 months in unilateral disease Balmer and Munier a.

Exophytic retinoblastoma Figure 2cdeveloping outside the retina towards the choroid, causes a retinal detachment that masks to a greater or lesser degree the details of the underlying mass or masses. In the adult, optic nerve glioma is malignant and invariably fatal due to involvement of the chiasma and cerebral structures.


More common signs should also be considered suspicious until proved otherwise, such as strabismus, the second most frequent sign of retinoblastoma. In the long term, enucleation may be inevitable due to terminal glaucoma, intra-ocular hemorrhage, retinal detachment, uveitis or phthisis bulbi.

A statistical survey of children. The initial tumoral cell divisions may occur in different internal retinal layers, a tumor developing on the surface of the retina and into the vitreous cavity being called an endophytic growth, while that developing from the external layers, invading the subretinal space and causing a retinal detachment an exophytic growth.

Medulloepithelioma of the retina. A red reflex exam is also warranted at every pediatric visit from birth to 3 years and, later, as part of vision screening. All positions of gaze should be elicited, first of all in miosis then repeated with the pupil fully dilated, taking advantage of mydriasis to detect any anomaly of the macular or papillary region on ophthalmoscopy.

Differential diagnosis of leukocoria and strabismus, first presenting signs of retinoblastoma

Autosomal dominant inheritance of mutation in PAX6 gene on chromosome 11 has been noted. A clinicopathologic study of 56 cases of intraocular medulloepitheliomas.

Evaluation and management of suspected retinal detachment. Can be seen in both retinoblastoma and Coats disease and can also be associated with any long-standing retinal detachment. The parents continued to say that they and no other family members or friends had noticed any abnormalities and he was otherwise well. It may stabilize, although often with loss of central vision due to macular lipid deposits, or deteriorate with total exudative retinal detachment Figure 3diridis rubeosis, neovascular glaucoma, cataract, uveitis, phthisis bulbi and, ultimately, total and definitive loss of vision.

Papillary and intraocular involvement is possible but very rare. Perspectives on the Profession News in Review: Jaypee Brothers Medical Publishers; ;