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Differential Diagnosis of Leukocoria

Jerry A. Shields and Carol L. Shields

ETINOBLASTOMA is a highly malignant intraocular tumor of childhood that requires accurate diagnosis and prompt treatment.1-3 The child with retinoblastoma most often presents clinically with leukocoria or white pupillary reflex in the affected eye or eyes (Fig 1). A number of benign conditions can clinically simulate retinoblastoma, sometimes creating diagnostic difficulty for the Because the management of these various pseudoretinoblastomas differs considerably from the treatment of retinoblastoma, their clinical recognition is mandatory. The use of techniques such as indirect ophthalmoscopy, ultrasonography, and computed tomography (CT) has also helped to improve diagnostic accuracy.] Increasing referral of patients to clinicians who specialize in ocular oncology has further improved diagnostic accuracy and decreased the chances of misdirected therapy. The more important pseudoretinoblastomas can be divided into hereditary conditions, developmental abnormalities, inflammatory disorders, tumors, and miscellaneous entities (Table 1). Because some of the conditions listed in Table 1 are rare and not often encountered by the practicing physician, the list does not necessarily reflect the true clinical problem confronting the ophthalmologist. In order to obtain more accurate information on the true clinical problem, we reviewed 500 consecutive patients referred to us between 1974 and 1987 with the diagnosis of either retinoblastoma or possible retinoblastoma in order to determine the frequency of the various simulating lesions in a clinical practice.6 Of the 500 referred cases, 288 (58%) were found on subsequent examination to have retinoblastoma, and 212 (42%) had a variety of pseudoretinoblastomas. The final diagnoses in the 500 cases and a diagnostic breakdown of the pseudoretinoblastomas are shown in Table 2. The three conditions that most commonly simulated retinoblastoma in our series were persistent hyperplastic primary vitreous (PHPV) (28%), Coats disease (16%), and presumed ocular toxocariasis (16%).

CONDITIONS THAT SIMULATE RETINOBLASTOMA

Conditions that can simulate retinoblastoma can be divided into hereditary conditions, developmental abnormalities, inflammatory disorders, tumors, and miscellaneous categories.

Hereditary Conditions Nowies disease. Norries disease is an Xlinked recessive disorder that occurs almost exclusively in males and is clinically apparent at birth. It accounted for only 1of the 212 cases of pseudoretinoblastoma in our series6 (Table 2). It is characterized by bilateral leukocoria secondary to a detached gliotic retina, often associated with elongated ciliary processes. Norries disease differs from retinoblastoma by its mode of inheritance, its predilection for males, and the ophthalmoscopic appearance of a tractional retinal detachment rather than a tumor. In contrast to patients with retinoblastoma, patients with Norries disease frequently develop hearing loss and mental retardation early in life. Congenital retinoschisis. Congenital retinoschisis is a bilateral vitreoretinal dystrophy that develops early in life. Like Norries disease, it is an X-linked recessive disorder that occurs almost exclusively in males. It accounted for 4 (2%) of the 212 pseudoretinoblastomas in our seriesh (Table 2). Ophthalmoscopy shows a characteristic stellate pattern in the fovea and thin vascular membranes that resemble a detached atrophic retina. The retinal membranes and the underlying fluid are characteristically transparent in contrast to the opaque or translucent yellow-white color of retinoblastoma. These

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From the Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA. Supported by the Eye Tumor Research Foundation, Philadelphia, PA. Adapted and reprinted with permission from Shields CL, Shields JA. Intraocular Tumors: A Text and Atlas. Philadelphicr, PA: Saunders; 1992. Address reprint requests to Jeny A. Shields, MD, Director, Oncology Service, Wills Eye Hospital, 900 Walnut St, Philadelphia, PA 19107. Copyright 0 1993 by W B. Saunders Company 0882-0838193l0804-0011$5.0010
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Seminars / n Ophthalmology,Vol8, No 4 (December), 1993: pp 273-280

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Table 2. Conditions Simulating Retinoblastoma Among 500 Consecutive Patients with Leukocoria Referred to the Investigator
No. of patients Final diagnosis Retinoblastoma Pseudoretinoblastoma Breakdown of pseudoretinoblastomas PHPV Coats disease Ocular toxocariasis Retinopathy of prematurity Combined hamartoma Coloboma Vitreous hemorrhage Astrocytic hamartoma Familial exudative vitreoretinopathy Idiopathic retinal vascular hypoplasia Rhegmatogenous retinal detachment X-linked retinoschisis Medulloepithelioma Congenital cataract Retinal capillary hemangioma Circumscribed choroidal hemangioma Diffuse choroidal hemangioma Peripheral uveoretinitis Toxoplasmic retinitis Idiopathic endophthalmitis Norries disease lncontinentia pigmenti Morning-glory disc anomaly

500
NO/%

288158 212/42 59127.8 3411 1 .O 33115.6 1014.7 914.2 914.2 8/33 612.8 512.4 411.9 411 9 . 411.9 4 1.9 1 411.9 31 1.4 311.4 3 1.4 1 311.4 2/0.9 210.9 110.5 110.5 110.5

Fig 1. Leukocoria in a child with retinoblastoma.

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thin membranes consist histologically of partialthickness retina, created by splitting of the retina in the nerve fiber layers and the development of large cystic spaces between the layers.
Table 1. Classification of Conditions That Can Simulate Retinoblastoma
Hereditary Conditions Norries disease Congenital retinoschisis lncontinentia pigmenti Dorminant exudative vitreoretinopathy Developmental Abnormalities Persistent hyperplastic primary vitreous (PHPV) Congenital cataract Coloboma Retina dysplasia Congenital retinal fold Myelinated nerve fibers Morning glory syndrome Congenital corneal opacity Inflammatory Disorders Ocular toxocariasis Congenital toxoplasmosis Congenital cytomegalovirus retinitis Herpes-simplex retinitis Peripheral uveoretinitis Metastatic endophthalmitis Orbital cellulitis Tumors Retinal astrocytic hamartoma Medulloepithelioma Glioneuroma Choroidal hemangioma Retinal capillary hemangioma Combined retinal hamartoma Leukemia Miscellaneous Coats disease Retinopathy of prematurity Rhegmatogenous retinal detachment Vitreous hemorrhage Perforating ocular injury

Dehiscences frequently occur in the inner layer of the retinoschisis cavity, leading to rupture of unsupported retinal blood vessels and subsequent vitreous hemorrhage. The inner layer of this cavity immediately behind the lens may become gliotic, producing leukocoria. This condition can be easily differentiated from retinoblastoma by its transparency with indirect ophthalmoscopy, its typical stellate configuration in the fovea, the lack of a distinct mass with ultrasonography, and its X-linked inheritance ~attern.~ Incontinentia pigmenti. Incontinentia pigmenti is a hereditary disorder that may affect the eye, skin, skeletal system, heart, and central nervous system. Children with this condition develop leukocoria secondary to proliferative retinal vascular abnormalities in the peripheral fundus that can lead to total retinal detachment. Incontinentia pigmenti accounted for 1of the 212 pseudoretinoblastomas in our seriesh

DIFFERENTIAL DIAGNOSIS OF RB

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Dominant exudative vitreoretinopathy. Dominant exudative vitreoretinopathy (familial exudative vitreoretinopathy (FEV)) is a bilateral, often asymmetrical, affectation of the fundus characterized by peripheral retinal avascularity and yellow subretinal exudation usually most pronounced in the temporal quadrants. Secondary traction between the posterior retinal and the area of peripheral fibrovascular proliferation can lead to a retinal detachment. FEV accounted for 5 cases (2%) of the pseudoretinoblastomas in our series.6The tractional phenomena and exudation seen with FEV are not often seen in eyes with retinoblastoma.
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Developmental Abnormalities Persistent hyperplasticprimay vitreous (PHPV). In our series, PHPV was the most common lesion that simulated retinoblastoma, accounting for 59 of the 212 pseudoretinoblastomas (28%) (Table 2).6PHPV is a congenital, nonhereditary condition that is usually unilateral and is noted during the first few days or weeks of life. A white pupillary reflex is characteristically present in the involved eye, which is almost always microphthalmic and has a shallow anterior chamber (Fig 2). In the more advanced form of PHPV, anterior-segment examination with the pupil dilated often shows the ciliary processes to be drawn into a contracting retrolental mass attached to a cataractous lens. Microphthalmia, traction on the ciliary pro-

Fig 2. Leukocoria secondary to persistent hyperplastic primary vitreous.

cesses, and cataract are not features of eyes with retinobla~toma.~ Cataract, Infantile cataracts of any pathogenesis can produce leukocoria and should therefore be included in the differential diagnosis of retin~blastoma.~ Although cataracts in children are relatively common, they accounted for only 4 of the 212 cases (2%) of pseudoretinoblastoma in our series (Table 2).hMost congenital cataracts are accurately diagnosed today, and patients are not referred to us because of suspected retinoblastoma. Coloboma. Coloboma accounted for 9 of the 212 cases of pseudoretinoblastoma in our series (4%).6 A large coloboma of the retina and choroid can produce leukocoria. However, the use of indirect ophthalmoscopy should clearly define a flat or depressed lesion, rather than an elevated one. If the patient has an associated iris coloboma, the diagnosis may be more obvious. Iris colobomas are almost never seen in eyes with retinoblastoma. Retinal dysplasia. Retinal dysplasia is a histopathological term that should not be used to describe a clinical condition. It is characterized by a congenitally detached or malformed retina with dysplastic retinal rosette^.^ It is associated with the trisomy 13 syndrome, which is characterized by a variety of systemic and central nervous system abnormalities. Ocular findings may include bilateral leukocoria, colobomas, and microphthalmia. Congenital retinal fold. The congenital retinal fold, or falciform fold, consists of a ridge of abnormal retina, glial tissue, and blood vessels which extends from the optic disc to any quadrant of the peripheral f ~ n d u s Although the .~ fold sometimes is considered to be an abnormality of the primary vitreous, most cases are probably a result of ocular toxocariasis, trauma, dominant exudative vitreoretinopathy, or prematurity and other proliferative retinopathies in children. The typical elongated fold with dragging of the retina does not occur with retinoblastoma, and the clinical differentiation should not be diffic~lt.~ Myelinated nerve fibers. Myelinated nerve fibers in the retina are occasionally widespread enough to produce leukocoria and thereby suggest the diagnosis of retinobla~toma.~ Fundus examination shows the typical yellow-white peripapillary thickening of the nerve fiber layer with

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a fibrillated margin. In some cases, myelinated nerve fibers are associated with a coloboma of the optic disk. The typical clinical appearance should enable easy differentiation from retinoblastoma. Morning gloy disc anomaly. The morningglory disc anomaly is a congenital abnormality of the optic disc, characterized by a colobomalike malformation associated with epipapillary fibrous tissue, peripapillary pigmentary changes, and retinal vascular anomalies. If this lesion is large, it may produce leukocoria." Smaller lesions can be confused with a small retinoblastoma near the optic disc. Bilateral idiopathic retinal vascular hypopasia. Bilateral idiopathic retinal vascular hypoplasia is a recently reported entity that is characterized by persistence of the primary vitreous and congenital absence of many of the peripheral retinal vessels. A total retinal detachment can ensue. This condition accounted for 4 of the 212 pseudoretinoblastomas (2%).6 In contrast to retinoblastoma, no retinal mass can be detected clinically or with ultra~onography.~ Congenital corneal opacity. A corneal opacity may also appear white, but it should not be confused with retinoblastoma. External and slit-lamp examination should readily differentiate this "pseudoleukocoria" from the true leukocoria of retinoblastoma, in which the cornea is usually clear."

Fig 3. Leukocoria secondary to ocular toxocariasis (nematode endophthalmitis).

Infiammatoy Disorders Ocular toxocariasis. Presumed ocular toxocariasis accounted for 33 of the 212 cases of pseudoretinoblastoma referred to us because of suspected retinoblastoma ( 16%).h This condition is caused by infestation of the eye with the wandering second-stage larva of the dog ascarid, Toxocara canis, the organism that produces visceral larval rnigram8 Ocular toxocariasis can appear as a diffuse vitreoretinal inflammation (nematode endophthalmitis) or as a localized granloma, both of which can produce a white mass that simulates retinoblastoma (Fig 3). In contrast to a comparable-sized retinoblastoma, the acute lesion of nematode endophthalmitis produces a severe cellular reaction and vitreous traction bands. The tractional phenomena and secondary cata-

ract seen with ocular toxocariasis are extremely rare in eyes with retinoblastoma." Congenital toxoplasmic retinitis. Ocular involvement with congenital toxoplasmosis may produce yellow-white retinal inflammation with vitreous cells, which can resemble an endophytic retinoblastoma with vitreous seeding. The resultant atrophic macular scars can become large enough to produce leukocoria. In contrast to a retinoblastoma, the atrophic toxoplasmosis lesion is flat or depressed and has well-delineated pigmented margins. Extraocular clinical findings of congenital toxoplasmosis such as hydrocephalus, hepatosplenomegaly, and cerebral calcifications are unlikely in retinobla~toma.~ Congenital cytomegalovirus retinitis. Neonatal infection with cytomegalovirus also may produce a retinal and vitreous inflammatory reaction that can resemble an endophytic retinoblastoma with vitreous ~ e e d i n g The typical .~ areas of hemorrhagic retinal necrosis as well as signs of anterior-segment inflammation should help in the clinical differentiation from retinoblastoma. Herpes-simplex retinitis. Herpes-simplex retinitis can produce a clinical picture similar to that of toxoplasmosis or cytomegalovirus retinitis and may also resemble an endophytic retinoblastoma with vitreous seeding. The patient may have simultaneous encephalitis, which is extremely unlikely in patients with retinoblast~ma.~

DIFFERENTIAL DIAGNOSIS OF RB

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Peripheral uveoretinitis. Idiopathic peripheral uveoretinitis (pars planitis) is a chronic intraocular inflammation of unknown origin that is characterized clinically by the development of organized white tissue within the vitreous base and oral zone of the retina (snowbanks), cellular clumps in the anterior vitreous, and inflammatory sheathing of the peripheral retinal vessels. In contrast to retinoblastoma, peripheral uveoretinitis usually becomes evident in older children or young adults. In the active stage, peripheral uveoretinitis can resemble a peripheral endophytic retinoblastoma with vitreous ~ e e d i n g . ~ Metastatic endophthalmitis. The frequency of metastatic endophthalmitis in children has decreased since the advent of antibiotics. However, untreated severe bacterial septicemias can produce overwhelming intraocular inflammation, which may occasionally resemble vitreous seeding of an endophytic retin~blastoma.~ Orbital cellulitis. Severe orbital inflammation in a child can produce clinical findings that may suggest a retinoblastoma that has become necrotic and has invaded the orbit. Children with such inflammation usually have clinical and radiological evidence of ethmoidal sinusitis. Conversely, a necrotic retinoblastoma can also produce signs and symptoms suggestive of orbital ~ellulitis.~ However, children with retinoblastoma do not usually have signs of ethmoiditis. A complete ocular evaluation with indirect ophthalmoscopy, ultrasonography, and CT can readily distinguish these condition^.^,^ Tumors Several types of benign and malignant intraocular tumors can also resemble retinoblastoma. Retinal astrocytic hamartoma The astrocytic hamartoma of the retina can be very similar to retinoblastoma. It accounted for 6 of the 212 pseudoretinoblastomas in our series (Table 2).6 This yellow-white tumor arises from the inner layers of the sensory retina and may show calcification. However, the calcification within an astrocytic hamartoma is usually glistening yellow (Fig 4), whereas the calcium within a retinoblastoma is a dull, chalky white. Clinical evidence of tuberous sclerosis or neurofibromatosis in the affected patient lends support to the

Fig 4. Calcified retinal astrocytic hamartoma of tuberous sclerosis simulating a calcified retinoblastoma.

diagnosis of astrocytic hamartoma. In many instances, however, an astrocytic hamartoma can occur in a patient who has no evidence of these syndromes. In such instances, a period of observation for growth may be justified in order to determine whether the lesion represents a retinobla~toma.~ Medulloepithelioma. Intraocular medulloepithelioma is an embryonal tumor that arises from the undifferentiated medullary epithelium usually in the ciliary-body region. It accounted for 4 of the 212 pseudoretinoblastomas in our series (2%).6 Because it occurs in children and often produces a yellow-pink intraocular mass with leukocoria, it may be clinically confused with retinoblastoma (Fig 5). In contrast to retinoblastoma, however, it is almost always unilateral, is usually diagnosed at a slightly older age, is often multicystic, and usually involves the ciliary region and anterior chamber rather than the posterior f ~ n d u s . ~ Glioneuroma. Glioneuroma is an extremely rare tumor that arises from the anterior lip of the primitive optic cup. In contrast to retinoblastoma, it is almost always unilateral, involves the anterior chamber angle, produces iridocorneal adhesions, and usually spares the posterior segment! Choroidalhemangioma. Circumscribed choroidal hemangioma occasionally produces leukocoria because of secondary retinal detachment.

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Fig 5. Medulloepithelioma of the ciliary body. The ciliary body location and the clear cysts in the tumor would be very atypical for retinoblastoma.

for 9 of the 212 pseudoretinoblastomas in our series (4%).h Unlike retinoblastoma, the lesion is almost always unilateral, is dusky gray rather than white, and is usually associated with contraction of the internal limiting membrane and secondary stretching and tortuosity of the retinal vessels, which are findings not seen with retinoblastoma. The condition is not familial. Leukemia. In rare instances, leukemia in children can cause vitreous cells and an accumulation of white cells in the anterior chamber that can simulate an endophytic retinobla~toma.~ In most of these cases, the diagnosis of leukemia can readily be made on the basis of clinical examination and hematologic studies.

There were 3 such cases in our series of 212 pseudoretinoblastomas.6 In contrast to retinoblastoma, the circumscribed choroidal hemangioma almost always occurs unilaterally, and the color of the lesion is typically red-orange rather than white. There may be a serous detachment of the retina, but in contrast to retinoblastoma, the overlying retinal vessels are of normal caliber and distribution. If the patient has typical encephalofacial angiomatosis of the SturgeWeber syndrome, the diagnosis of diffuse choroidal hemangioma is more obvious. However, the diffuse choroidal hemangioma also accounted for 3 of the 212 pseudoretinoblastomas.6 Retinal capillaiy hemangioma. A retinal capillary hemangioma can occasionally become clinically apparent in infancy and simulate retinoblastoma. It accounted for 3 of the 212 pseudoretinoblastomas in our series.6 Like retinoblastoma, it is often transmitted by an autosomal-dominant mode of inheritance, it arises from the sensory retina, and it shows dilated afferent and efferent retinal-feeder vessels. In contrast to retinoblastoma, however, it has a red-orange color and is associated with yellow subretinal exudates and often a stellate macu10pathy.~ Combined retinal and retinalpigment epithelial hamartoma. A combined hamartoma of the sensory retina and retinal pigment epithelium is a relatively rare lesion that may be clinically detected in young children and sometimes may be confused with retinoblastoma.4 It accounted

Miscellaneous Conditions Coats disease. Coats disease is a relatively common retinal-vascular disorder of uncertain origin that is characterized by unilateral retinal telangiectasia, progressive intraretinal exudation, and exudative retinal detachment. The disorder occurs in young males in the first decade of life.4 It is frequently confused clinically with retinoblastoma, and it accounted for 34 of the 212 cases of pseudoretinoblastoma in our series.6 Although Coats disease can become clinically apparent at almost any age, it usually is diagnosed in patients who are between 4 and 10 years of age. The family history, birth history, and medical history usually provide no clue as to a precipitating factor. The great majority of patients with Coats disease are unilaterally affected males. In contrast, patients with retinoblastoma may have a positive family history and bilateral involvement. The disease has no sex predilection. There is usually no ocular pain or inflammation unless secondary glaucoma develops. The most striking early funduscopic findings are dilation or aneurysmal changes in the retinal blood vessels and secondary retinal detachment (Fig 6). These are usually most pronounced in the temporal quadrants. These sausage-like aneurysmal vascular changes are different from the uniformly dilated retinal vessels associated with retinoblastoma. Refractile particles and occasional hemorrhage can often be observed in the subretinal space. The refractile particles correspond to the choles-

DIFFERENTIAL DIAGNOSIS OF RB

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Fig 6. Total retinal detachment secondary t o Coats disease. Note the irregularly dilated retinal vessels.

terol crystals noted on histological examination. These crystals usually appear quite different from the dull, chalky-white calcium particles present in retinoblastoma. Eyes with advanced Coats disease can develop a total retinal detachment, iris neovascularization, and a spontaneous hyphema. These features also can occur frequently in eyes with advanced retinoblastoma. There are no specific clinical tests for Coats disease. Fluorescein angiography shows retinal telangiectasia and profuse leakage from the abnormal retinal vessels. Ultrasonography shows a retinal detachment but no distinct tumor. Particulate echoes of moderately high intensity, which probably correspond to the refractile particles seen clinically, can be shown in the subretinal space. However, these particles are much less reflective than the calcium particles present in retinoblastoma. Retinopathy of prematurity (ROP). Children with ROP may develop severe peripheral retinal neovascularization that may lead to vitreoretinal traction and a total retinal detachment. This can produce either unilateral or bilateral leukocoria. ROP accounted for 10 of the 212 pseudoretinoblastomas in our series (5%) (Table 2).h Although this condition is rather common, it is not often confused with retinoblastoma today, thus explaining the relatively small number of cases in our ~ e r i e s . ~

The child with ROP usually has a history of prematurity and has usually received supplemental oxygen therapy immediately after birth. Most patients with retinoblastoma had a normal birth weight and received no oxygen. Moderately severe cases show dragging of the sensory retina toward the fibrovascular mass in the peripheral fundus temporally, which produces leukocoria. The retrolental lesion appears cystic and gliotic in contrast to retinoblastoma, which appears dull white or gray.4 Rhegmatogenous retinal detachment. A rhegmatogenous retinal detachment in infants and young children can be confused clinically with retinoblastoma. In contrast to retinoblastoma, the rhegmatogenous retinal detachment shows a typical rippled appearance, and a retinal hole usually can be detected with indirect ophthalmo~copy.~ Vitreous hemorrhage. Vitreous hemorrhage in children, regardless of the cause, may superficially resemble retinoblastoma. When the hemorrhage undergoes degradation, it may become white or yellow, similar to vitreous seeding from an underlying tumor. However, indirect ophthalmoscopy and B-scan ultrasonography should show the intravitreal location without retinal inv~lvement.~ Perforating ocular injury. Perforating injuries to the sclera may lead to intraocular scar tissue, which may have an ophthalmoscopic appearance similar to that of a retinoblastoma. This may occur following both surgical and nonsurgical t r a ~ m a . ~
SUMMARY

A number of ocular disorders in infants and children can clinically resemble a retinoblastoma. Smaller lesions may have ophthalmoscopic features suggestive of a tumor, whereas larger or more advanced lesions produce a white pupillary reflex, or leukocoria. The three conditions that most often lead to difficulty in the clinical differentiation from a retinoblastoma are PHPV, Coats disease, and presumed ocular toxocariasis. Hereditary disorders included in the differential diagnosis of a retinoblastoma include Norries disease, congenital retinoschisis, dominant exudative vitreoretinopathy, and incontinentia pigmenti. In addition to PHPV, develop-

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mental lesions such as congenital cataract, coloboma, retinal dysplasia, congenital retinal fold, and myelinated nerve fibers can sometimes clinically resemble retinoblastoma. Although presumed ocular toxocariasis is the intraocular inflammatory process that most commonly produces leukocoria, congenital toxoplasmosis, cytomegalovirus retinitis, herpes simplex retinitis, peripheral uveoretinitis, and metastatic endophthalmitis of any cause can sometimes be difficult to differentiate from retinoblastoma. Other pediatric intraocular tumors to be considered in the differential diagnosis of retinoblastoma include retinal astrocytoma, medulloepithelioma, glioneuroma, choroidal hemangioma, retinal capillary hemangioma, and
1. Shields JA, Shields CL. Management and prognosis of retinoblastoma. In: Shields JA, Shields CL (eds). Intraocular Tumors. A Text and Atlas. Philadelphia, PA: Saunders; 1992~337-391. 2. Shields JA. Misconceptions and techniques in the management of retinoblastoma. The 1992 Paul Henkind Memorial Lecture. Retina. 1992;12:320-330. 3. Ellsworth RM. The practical management of retinoblastoma. Trans A m Ophthalmol Soc. 1969;67:462-534. 4. Shields JA, Shields CL. Differential diagnosis of retinoblastoma. In: Shields JA, Shields CL (eds). lntraocular Tumors. A Text and Atlas. Philadelphia, PA: Saunders; 1992:341-362. 5. Howard GM, Ellsworth RM. Differential diagnosis of

combined retinal hamartoma. The ophthalmoscopic features of the retinal astrocytoma may be very similar to a retinoblastoma, and, unless the patient has associated signs of tuberous sclerosis or neurofibromatosis, the clinical diagnosis may be quite difficult. The other tumors have rather specific features that should facilitate the correct diagnosis. Rhegmatogenous retinal detachment, vitreous hemorrhage, or perforating injuries to the sclera may produce a clinical picture similar to that of a retinoblastoma. Familiarity with the clinical features of these pseudoretinoblastomas combined with a thorough history and ocular examination should enable one to readily differentiate these simulating conditions from retinoblastoma.
retinoblastoma. A statistical survey of 500 children. I. Relative frequency of the lesions which simulate retinoblastoma. A m JOphthalmol. 1965;60:610-618. 6. Shields JA, Parsons HM, Shields CL, et al. Lesions simulating retinoblastoma.JPed Ophthalrnol Strabism. 1991; 28:338-340. 7. Shields JA, Shields CL, Parsons HM. Review: Differential diagnosis of retinoblastoma. Retina. 1991;11:232-243. 8. Shields JA. Ocular Toxocariasis. A review. Suw Ophthalmol. 1984;28:361-381. 9. Shields JA, Shields CL, Suvarnamani C, et al. Retinoblastoma manifesting as orbital cellulitis. A m J Ophthalrnol. 1991;112442-449.

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