Diagnostico Diferenciales de Leucocoria

8/3/2019 Diagnostico Diferenciales de Leucocoria

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Differential Diagnosis of LeukocoriaJerry A. Shields and Carol L. Shields

ET INO BL AS TO MA is a highly malignantR ntraocular tumor of childhood that re-quires accurate diagnosis and prompt treat-ment.1-3 Th e child with retinoblastoma mostoften presents clinically with leukocoria or w hitepupillary reflex in the affected eye or eyes (Fig1).A number of benign c ond itions can clinicallysimulate retinoblastoma, sometimes creatingdiagno stic difficulty for the Be-cause the managem ent of thes e various pseudo-retinoblastomas differs considerably from thetreatm ent of retinoblastoma , the ir clinical recog-nition is mandatory. Th e use of techniques suchas indirect ophthalmoscopy, ultrasonography,and computed tomography (CT ) has also helpedto improve diagnostic accuracy.] Increasing re-ferral of patients to clinicians who specialize inocular oncology has further improved diagnos-tic accuracy and decreased the ch ances of misdi-rected therapy.The more important pseudoretinoblastomascan be divided into he reditary conditions, devel-opmental abnormalities, inflammatory disor-ders, tumors, and miscellaneous entities (Ta-ble 1).Because some of the conditions listed inTable 1 are rare and not often encountered bythe practicing physician, the list doe s not neces-sarily reflect t he tru e clinical problem confront-ing the ophthalmologist. In order to obtainmore accurate information on the true clinicalproblem, we reviewed 500 consecutive patientsreferred to us between 1974 and 1987 with thediagnosis of either retinoblastoma or possibleretinoblastoma in order to determine the fre-quency of the various simulating lesions in aclinical practice.6Of th e 500 referred cases, 288(58%) were found on subsequent examinationto have retinoblastoma, and 212 (42%) had avariety of pseudoretinoblastom as. T he final di-agnoses in the 500 cases and a diagnosticbreakdown of the pseudoretinoblastomas areshown in Table 2. The three conditions thatmost commonly simulated retinoblastoma inou r series were persistent hyperplastic primaryvitreous (PHPV) (28%), Coats disease (16%),and presumed ocular toxocariasis (16% ).

CONDITIONS THAT SIMULATERETINOBLASTOMA

Conditions that can simulate retinoblastomacan be divided into hered itary conditions, devel-opmental abnormalities, inflammatory disor-ders, tumors, and m iscellaneous categories.Hereditary Conditions

Nowies disease. Norries disease is an X-linked recessive disorder that occurs almostexclusively in males and is clinically app are nt a tbirth. It accou nted for only 1of the 212 cases ofpseudo retinoblastoma in our series6 (Table 2).It is characterized by bilateral leukocoria secon d-ary to a detach ed gliotic retina , often associatedwith elongated ciliary processes. Norries dis-eas e differs from retinoblastom a by its mode ofinheritance, its predilection for males, and theophthalmoscopic appearance of a tractionalretinal detachment rather than a tumor. Incontrast to patients with retinoblastoma, pa-tients with Norries disease frequently d evelophearing loss and m ental reta rda tion early in life.Congenital retinoschisis. Congenital retinos-chisis is a bilateral vitreoretinal dystrophy thatdevelop s early in life. Like N orries disease, it isan X-linked recessive disorder that occurs al-most exclusively in males. It accounted for 4(2%) of the 212 pseudoretinoblastomas in ourseriesh (Table 2). Ophthalmoscopy shows acharacteristic stellate pattern in the fovea andthin vascular membranes that resemble a de-tached atrophic retina. The retinal membranesand the underlying fluid are characteristicallytranspa rent in contrast to the opa qu e or translu-cent yellow-white color of retinoblastoma. Thes e

From the Oncology Service, Wills Eye Hospital, ThomasJefferson University, Philadelphia, PA.

Supported by the Eye Tu mo r Research Foundation, Philadel-phia, PA .Adapted and reprinted with permission from Shields CL,Shields JA. Intraocular Tumors: A Text and Atlas. Philadel-phicr, PA: Saun ders; 1992.Address reprint requests to Jeny A. Shields, MD, Director,Oncology Service, Wills Eye H ospital, 900 W alnu t St, Philadel-phia, PA 19107.Copyright 0 993 by WB. Saunders Company

0882-08381930804-0011$5.0010Seminars /n Ophthalmology,V o l 8 , No 4 (December),1993: pp 273-280 273


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274 SHIELDS AND SHIELDS

Table 2. Conditions Simulating Retinoblastoma Amon g 500Consecutive Patients wit h Leukocoria Referred t o th e

Investigator

Fig 1. Leukocoria n a child with retinoblastoma.

thin m em bran es consist histologically of partial-thickness retina, created by splitting of theretina in the nerve fiber layers and th e develop-ment of large cystic spaces between the layers.

Table 1. Classification of Conditions That C an SimulateRetinoblastoma

Hereditary ConditionsNorries diseaseCongenital retinoschisislncontinentia pigmentiDorminant exudative vitreoretinopathyPersistent hyperplastic primary vitreous (PHPV)Congenital cataractColobomaRetina dysplasiaCongenital retinal foldMyelinated nerve fibersMorning glory syndromeCongenital corneal opacity

Inflammatory DisordersOcular toxocariasisCongenital toxoplasmosisCongenital cytomegalovirus retinitisHerpes-simplex retinitisPeripheral uveoretinitisMetastatic endophthalmitisOrbital cellulitisRetinal astrocytic hamartomaMedulloepitheliomaGlioneuromaChoroidal hemangiomaRetinal capillary hemangiomaCombined retinal hamartomaLeukemia

MiscellaneousCoats diseaseRetinopathy of prematurityRhegmatogenous retinal detachmentVitreous hemorrhagePerforating ocular inju ry

Developmental Abnorma lities

Tumors

No. of patientsFinal diagnosis

RetinoblastomaPseudoretinoblastomaPHPVCoats diseaseOcular toxocariasisRetinopathy of prematurityCombined hamartomaColobomaVitreous hemorrhageAstrocytic hamartomaFamilial exudative

vitreoretinopathyIdiopathic retinal vascular

hypoplasiaRhegmatogenous retinaldetachment

X-linked retinoschisisMedulloepitheliomaCongenital cataractRetinal capillary hemangiomaCircumscribed choroidalDiffuse choroidal hemangiomaPeripheral uveoretinitisToxoplasmic retinitisIdiopathic endophthalmitisNorries diseaselncontinentia pigmentiMorning-glory disc anomaly

Breakdown of pseudoretinoblastomas

hemangioma

500N O / %288158212/4259127.83411 .O33115.61014.7914.2914.28/33612.8512.4

411.941 1.9411.941 1.9411.9311.4311.431 1.4311.42/0.9210.9110.5110.5110.5

Dehiscences frequently occur in the inner layerof the retinoschisis cavity, leading to rup ture ofunsupported retinal blood vessels and subse-quent vitreous hemorrhage. The inner layer ofthis cavity immediately behind the lens maybecom e gliotic, producing leukoc oria. This con-dition can be easily differentiated from retino-blastoma by its transparency with indirect oph -thalmoscopy, its typical stellate configuration inthe fovea, the lack of a distinct mass withultrasonography, and its X-linked inheritance~ a t t e r n . ~

Incontinentia pigmenti. Incontinentia pig-menti is a hereditary disorder that may affectthe eye, skin, skeletal system, hea rt, and centralnervous system. Children with this conditiondevelop leukocoria secondary to proliferativeretinal vascular abnormalities in the peripheralfundus that can lead to total retinal detach-ment. Incon tinentia pigmenti accounted for 1ofthe 212 pseudoretinoblastomas in our se rie sh


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DIFFERENTIAL DIAGNOS IS OF RB 27 5

Do min ant exudative vitreoretinopathy. Domi-nant exudative vitreoretinopathy (familial exu-dative vitreoretinopathy (FEV)) is a bilateral,often asymmetrical, affectation of the funduscharacterized by peripheral retinal avascularityand yellow subretinal exudation usually mostpronounced in the temporal quadrants. Second-ary traction between the posterior retinal andthe area of peripheral fibrovascular prolifera-tion can lead to a retinal detachm ent. FE Vaccounted for 5 cases (2% ) of the p seudoretino-blastomas in ou r series.6Th e tractional phenom-ena and exudation se en with FE V a re not oftenseen in eyes with retinoblastom a.Developmental Abnormalities

Persistent hyperplasticpr im ay vitreous (PHPV).In our series, PHPV was the most commonlesion that simulated retinoblastoma, account-ing for 59 of the 212 pseudoretinoblastomas(28%) (Table 2).6PH PV is a congenital, nonh e-reditary condition that is usually unilateral andis noted during the first few days or weeks oflife. A white p upillary reflex is characteristicallypresent in the involved eye, which is almostalways microphthalmic and has a shallow ante -rior chamber (Fig 2). In the more advancedform of PHPV, anterior-segment examinationwith the pupil dilated often shows the ciliaryprocesses to be drawn into a contracting retro-lental mass attached to a cataractous lens.Microphthalmia, traction on the ciliary pro-

Fig 2. Leukocoria secondary to persistent hyperplasticprimary vitreous.

cesses, and cataract are no t fea ture s of eyes withr e t i n o b l a ~ t o m a . ~Infantile cataracts of any patho-genesis can produce leukocoria and shouldthe refo re be included in the differential diagno-s i s o f r e t i n~b l a s t oma . ~lthough cataracts inchildren a re relatively common, they accoun tedfor only 4 of the 212 cases (2% ) of pseudoretino-blastoma in ou r series (Table 2).hMost congeni-tal cataracts are accurately diagnosed today,and patients are not referred to us because ofsuspected retinoblastoma.Coloboma. Coloboma accounted for 9 ofthe 212 cases of pseudoretinoblastoma in ourseries (4%).6 A large coloboma of the retina andchoroid can produce leukocoria. However, theuse of indirect ophthalmoscopy should clearlydefine a flat or depressed lesion, rath er tha n anelevated one. If the patient has an associatediris coloboma, th e diagnosis may be mo re obvi-ous. Iris colobomas are almost never seen ineyes with retinoblastom a.Retin al dysplasia is a histo-pathological term that should not be used todescribe a clinical condition. It is characterizedby a congenitally de tach ed or malformed retinawith dysp lastic retin al rosette^. It is asso ciatedwith the trisomy 13 syndrome, which is charac-terized by a variety of systemic and centralnervous system abnormalities. Ocular findingsmay include bilateral leukocoria, colobomas,and m icrophthalmia. Th e congenital reti-nal fold, o r falciform fold, consists of a ridge ofabnorm al retina, glial tissue, and blood vesselswhich extends from the optic disc to any quad-r a nt of th e p erip he ra l f ~ n d u s . ~lthough thefold sometimes is considered t o be a n abnorm al-ity of the primary vitreous, most cases areprobably a result of ocular toxocariasis, trau ma ,dominant exudative vitreoretinopathy, or prem a-turity and other proliferative retinopathies inchildren. Th e typical elongated fold with drag-ging of the retina do es not occur with retinoblas-toma, and the clinical differentiation should notb e d i f f i c ~ l t . ~Myelinated nerve fibers. Myelinated nervefibers in the retina are occasionally widespreadenough to pro duc e leukocoria an d thereby sug-gest the diagnosis of ret inobla~toma.~undusexam ination show s the typical yellow-white peri-papillary thickening of the n erve fiber layer with

Cataract,

Retinal dysplasia.

Congenital retinal fol d.


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27 6 SHIELDSAND SHIELDS

a fibrillated margin. In some cases, myelinatednerve fibers are associated with a coloboma ofthe optic disk. The typical clinical appearanceshould en ab le easy differentiation from retino-blastoma. The morning-glory disc anomaly is a congenital abnormalityof the optic disc, characterized by a co loboma-like malformation associated with epipapillaryfibrous tissue, peripapillary pigmentary chan ges,and retinal vascular anomalies. If this lesion islarge, it may produce leukocoria." Smaller le-sions can be confused with a small retinoblas-toma n ear th e optic disc.Bilateral idiopathic retinal vascular hyp opa sia.Bilateral idiopathic retinal vascular hypoplasiais a recently reported entity that is character-ized by persistence of the primary vitreous andcongenital absence of many of the peripheralretinal vessels. A total retinal detachment canensue. This condition ac counted for 4 f the 212pseudoretinoblastomas (2%).6 In contrast toretinoblastoma, no retinal mass can be de tectedclinically or with ul t r a~ on og rap hy .~

A corneal opac-it y may also appea r white, but it should not beconfused with retinoblastoma. External andslit-lamp examination should readily differenti-at e this "pseudoleukocoria" from the true leu-kocoria of retinoblastoma, in which the corneais usually clea r."

Morning gloy disc anomaly.

Congenital corneal opacity.

Infiammatoy DisordersOcular toxocariasis. Presumed ocular toxoca-riasis accounted for 33 of the 212 cases ofpseudoretinoblastoma referred to us because ofsuspected retinoblastoma (16%).h This condi-tion is caused by infestation of the eyewith the

wandering second-stage larva of the dog asca-rid, Toxocara canis, the organism that producesvisceral larval rnigr am 8Ocular toxocariasis can appear as a diffusevitreoretinal inflammation (nematode endo-phthalmitis) or as a localized granloma, bo th ofwhich can produce a white mass that simulatesretinoblastoma (Fig 3). In contrast to a compa-rable-sized retinoblastoma, the acute lesion ofnematode endophthalmitis produces a severecellular reaction and vitreous traction bands.Th e tractional phenomena and secondary cata-

Fig 3. Leukocoria secondary to ocular toxocariasis (nem a-tode end ophthalmitis).ract seen with ocular toxocariasis are extremelyrar e in eyes with retinoblastom a."Congenital toxoplasmic retinitis. Ocular in-volvement with congenital toxoplasmosis mayproduce yellow-white retinal inflammation withvitreous cells, which can resemble an endo-phytic retinoblastoma with vitreous seeding.The resultant atrophic macular scars can be-come large enough to produce leukocoria. Incontrast to a retinoblastoma, the atrophic toxo-plasmosis lesion is flat or depressed and haswell-delineated pigmented margins. Extraocu-lar clinical findings of con genital toxoplasmosissuch as hydrocephalus, hepatosplenomegaly,and ce rebra l calcifications ar e unlikely in retino-b l a ~ t o m a . ~Congenital cytomegalovirus retinitis. Neona-tal infection with cytomegalovirus also mayproduce a retinal and vitreous inflammatoryreaction th at can resemble an endophytic retino-blastoma with v it reous ~ e e d i n g . ~he typicalareas of hemorrhagic retinal necrosis as well assigns of anterior-segment inflammation shouldhelp in the clinical differentiation from retino-blastoma.Herpes-simplex retinitis. Herpes-simplex reti-nitis can produce a clinical picture similar tothat of toxoplasmosis or cytomegalovirus retini-tis and may also resemble a n endop hytic retino-blastoma with vitreous seeding. The patientmay have simultaneous encephalitis, which isextremely unlikely in patients with retinoblas-t ~ m a . ~


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DIFFERENTIAL DIAGNOSIS OF RB 277

Peripheral uveoretinitis. Idiopathic periph-eral uveoretinitis (pars planitis) is a chronicintraocular inflammation of unknown originthat is characterized clinically by t he develop-ment of organized white tissue within the vitre-ous base and oral zone of the ret ina(snowbanks), cellular clumps in the anteriorvitreous, and inflammatory sheathing of theperipheral retinal vessels. In contrast to retino-blastoma, peripheral uveoretinitis usually be-comes evident in older children o r young adults.In the active stage, peripheral uveoretinitis canresemble a peripheral endophytic retinoblas-toma with vi treous ~e e d in g .~Metastatic endophthalmitis. The frequencyof metastatic endophthalmitis in children has

decreased since the adven t of antibiotics. How-ever, un trea ted severe bacterial septicemias canproduce overwhelming intraocular inflamma-tion, which may occasionally resemble vitreousseeding of an endophytic re t in ~b la s t om a. ~Orbital cellulitis. Severe orbital inflamma-tion in a child can produce clinical findings thatmay suggest a retinoblastoma that has becomenecrotic and has invaded the orbit. Childrenwith such inflamm ation usually have clinical an dradiological evidence of ethmoidal sinusitis.Conversely, a necrotic retinoblastoma can also

produce signs and symptoms suggestive of or-b ita l ~e l l u l i t i s . ~owever, children with retino-blastoma do not usually have signs of ethmoidi-tis. A comp lete ocular evaluation with indirectophthalmoscopy, ultrasonography, and CT canreadily distinguish the se condition^.^,^Tumors

Several types of benign and malignant intra-ocular tumors can also resemble retinoblas-toma.Retinal astrocytic hamartoma Th e astrocytichamartoma of the retina can b e very similar toretinoblastoma. It accounted for 6 of th e 212pseudoretinoblastomas in our series (Table 2).6This yellow-white tumor arises from the innerlayers of the sensory retina and may showcalcification. However, the calcification withinan astrocytic hamartoma is usually glisteningyellow (Fig 4), whereas the calcium within aretinoblastoma is a dull, chalky white. Clinicalevidence of tub ero us sclerosis or neurofibroma-tosis in the affected patient l end s sup po rt to the

Fig 4. Calcified retinal astrocytic ham artoma of tuberoussclerosis simulating a calcified retinoblastom a.

diagnosis of astrocytic hamartoma. In manyinstances, however, an astrocytic hamartomacan occur in a patient who has no evidence ofthese syndrom es. In such instances, a period ofobservation for growth may be justified in ord erto dete rmine whether the lesion represents ar e t i n o b l a ~ t o m a . ~Medulloepithelioma. Intraocular medulloepi-thelioma is an embryonal tum or tha t arises fromthe undifferentiated medullary epithelium usu-ally in th e ciliary-body region. It acc oun ted for 4of the 212 pseudoretinoblastomas in our series(2%).6 Because it occurs in children and oftenproduces a yellow-pink intraocular mass withleukocoria, it may be clinically confused withretinoblastoma (Fig 5 ) . In contrast to retinoblas-toma, however, it is almost always unilateral, isusually diagnosed at a slightly older age, is ofte nmulticystic, and usually involves the ciliary re-gion and anterior chamber rather than theposterior f ~ n d u s . ~

Glioneuroma. Glioneuroma is an extremelyrare tumor that arises from the anterior lip ofthe primitive optic cup . In contrast to retinoblas-tom a, it is almost always unilateral, involves th eanterior chamber angle, produces iridocornealadhesions, and usually spares the posteriorsegment!Choroidal hemangioma. Circumscribed cho-roidal hemangioma occasionally produces leuko-coria because of secondary retinal detach me nt.


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278 SHIELDS AND SHIELDS

Fig 5. Medulloepithelioma of the ciliary body. The ciliarybody location and the clear cysts in the tumor w ould be veryatypical for retinoblastoma.

There were 3 such cases in our series of 212pseudoretinoblastomas.6 In contrast to retino-blastoma, the circumscribed choroidal heman-gioma almost always occurs unilaterally, and thecolor of th e lesion is typically red-o rang e ra the rthan white. There may be a serous detachm entof the retina, but in contrast to retinoblastoma,the overlying retinal vessels are of normal cali-ber and distribution. If the patient has typicalencephalofacial angiomatosis of the Sturge-W eber syndrome, the diagnosis of diffuse choroi-dal heman gioma is more obvious. However, thediffuse choroidal hemangioma also accountedfo r 3 of the 212 pseudo retinoblastom as.6A retinal cap-illary hemangiom a can occasionally bec om e cli-nically appar en t in infancy and simulate retino-blastoma. I t accounted for 3 of the 212pseudoretinoblastomas in ou r series.6 Like reti-noblastoma, it is often transmitted by an auto so-mal-dominant mode of inheritance, it arisesfrom the sensory retina, and it shows dilatedafferent and efferent retinal-feeder vessels. Incontrast to retinoblastoma, however, it has ared-orange color and is associated with yellowsubretinal exudates and often a stellate macu-10pathy.~

Combined retinal and retinal pigment epithelialhamartoma. A combined hamartoma of thesensory retina an d retinal pigment epithelium isa relatively rare lesion that may be clinicallydetected in young children and sometimes maybe con fused with retinoblastoma.4 I t accounted

Retinal capillaiy hemangioma.

for 9 of the 212 pseudoretinoblastomas in ourseries (4%).h Unlike retinoblastoma, the lesionis almost always unilateral, is dusky gray ratherthan white, and is usually associated with con-traction of the internal limiting membrane andsecondary stretching and tortuosity of the reti-nal vessels, which are findings not seen withretinoblastoma. T he condition is not familial.Leukemia. In rare instances, leukemia inchildren can cause vitreous cells and an accum u-lation of white cells in the a nte rior chamb er tha tcan s imulate an endophytic re t in ob la~ to m a.~nmost of these cases, the diagnosis of leukemiacan readily be made on the basis of clinicalexamination an d hematologic studies.

Miscellaneous ConditionsCoats disease is a relativelycommon retinal-vascular disorder of uncertainorigin tha t is characterized by unilateral retinaltelangiectasia, progressive intraretinal exuda-tion, and exudative retinal detac hm ent. Th edisorder occurs in young males in the firstdecade of life.4It is frequently confused clini-cally with retinoblastom a, an d it accoun ted for34 of the 212 cases of pseudoretinoblastoma inou r series.6Although Coats disease can become clini-cally apparent at almost any age, it usually isdiagnosed in patients who a re between 4 and 10years of age. The family history, birth history,and m edical history usually provide no clue as toa precipitating factor. The great majority ofpatients with Coats disease are unilaterallyaffected males. In contrast, pa tients with retino-blastoma may have a positive family history andbilateral involvement. The disease has no sexpredilection. Th ere is usually no ocular pain o rinflammation unless secondary glaucoma devel-ops.Th e most striking early funduscopic findingsare dilation or aneurysmal changes in the reti-nal blood vessels and secondary retinal detach-ment (Fig 6). These are usually most pro-nounced in the temporal quadra nts. The sesausage-like aneurysmal vascular changes aredifferent from the uniformly dilated retinalvessels associated with retinoblastom a. R efrac-tile particles and occasional hemorrhage canoften be observed in the subretinal space. Therefractile particles correspond to the choles-

Coats disease.


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DIFFERENTIAL DIAGNO SIS OF RB 27 9

Fig 6. Total retinal detachment secondary to Coats dis-ease. Note the irregularly dilat ed retina l vessels.

terol crystals noted o n histological examination .These crystals usually appear quite differentfrom the dull, chalky-white calcium particlespresent in retinoblastoma. Eyes with advancedCoats disease can develop a total retinal detach-ment, iris neovascularization, and a spontane-ous hyphema. These features also can occur fre-quently in eyes with advanced retinob lastoma.Th ere a re n o specific clinical tests for Coa tsdisease. Fluorescein angiography shows retinaltelangiectasia and profuse leakage from theabnorm al retinal vessels. Ultrasono graphy showsa retinal detachment but no distinct tumor.Particulate echoes of mod erately high intensity,which probably correspond to the refractileparticles seen clinically, can be shown in thesubretinal space. However, these particles aremuch less reflective than the calcium particlespresent in retinoblastoma.Retinopathy of prematurity (ROP). Childrenwith ROP may develop severe peripheral reti-nal neovascularization th at may lead to vitreo-retinal traction and a total retinal detachment.This can produce either unilateral o r bilateralleukocoria. ROP accounted for 10 of the 212pseudoretinoblastomas in our series ( 5 % ) (Table2).h Although this condition is rather common,it is not often confused with retinoblastomatoday, thus explaining the relatively small num-ber of ca ses in ou r ~ e r i e s . ~

The child with ROP usually has a history ofprematurity and has usually received supplemen-tal oxygen therapy imm ediately aft er birth. Mostpatients with retinoblastom a had a normal birthweight and received no oxygen. Moderatelysevere cases show dragging of th e sensory retinatoward th e fibrovascular mass in the p eripheralfundus temporally, which produces leukocoria.T he retrolental lesion app ears cystic and glioticin contrast to retinoblastoma, which appearsdull white o r gray.4Rhegm atogenous retinal detachment. A rheg-matogenous retinal detach me nt in infants andyoung children can be confused clinically withretinoblastoma. In contrast to retinoblastoma,the rhegmatogenous retinal detachment showsa typical rippled ap pea ran ce, and a retinal holeusually can be detected with indirect ophthal-m o ~ c o p y . ~Vitreous hemorrhage. Vitreous hemorrhagein ch ildren, regardless of t he cause, may superfi-cially resemble retinoblastoma. When the hem-orrha ge undergoes degradation, it may becomewhite o r yellow, similar to vitreous seeding froman underlying tumor. However, indirect ophthal-moscopy and B-scan ultrasonography shouldshow the intravitreal location without retinali n v ~ l v e m e n t . ~

Perforating ocular injury. Perforating inju-ries to the sclera may lead to intraocular scartissue, which may have an ophthalmoscopicappe aranc e similar to that of a retinoblastoma.This may occur following both surgical andno nsurgical t r a ~ m a . ~SUMMARY

A number of ocular disorders in infants andchildren can clinically resemble a retinoblas-toma. Smaller lesions may have ophthalmo-scopic features suggestive of a tumor, whereaslarger or more advanced lesions produce awhite pu pillary reflex, o r leukocoria.The three conditions that most often lead todifficulty in the clinical differentiation from aretinoblastoma a re P HP V, Coats disease, andpresum ed ocular toxocariasis.Hereditary d isorders included in the d ifferen-tial diagnosis of a retinoblastoma includeNorries disease, conge nital retinoschisis, domi-nant exudative vitreoretinopathy, and inconti-nentia pigmenti. In addition to PH PV , develop-


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280 SHIELDS AN D SHIELDS

mental lesions such as congenital cataract,coloboma, retinal dysplasia, congenital retinalfold, and m yelinated nerve fibers can som etimesclinically resemble retinoblastoma.Although presumed ocular toxocariasis is theintraocular inflammatory process that most com -monly produc es leukocoria, congenital toxoplas-mosis, cytomegalovirus retinitis, herpe s simplexretinitis, peripheral uveoretinitis, and meta-static endophthalmitis of any cause can some-times be difficult to d ifferentiate from retinoblas-toma.Other pediatric intraocular tumors to beconsidere d in the differential diagnosisof retino-blastoma include retinal astrocytoma, medullo-epithelioma, glioneuroma, choroidal heman-gioma, retinal capillary hemangioma, and

combined retinal hamartoma. The ophthalmo-scopic features of the retinal astrocytoma maybe very similar to a retinoblastoma, and, u nlessthe patient has associated signs of tuberoussclerosis o r neurofibrom atosis, th e clinical diag-nosis may be quite difficult. The other tumorshave rath er specific feat ure s that shou ld facili-tate the correct diagnosis. Rhegmatogenousretinal detachment, vitreous hemorrhage, orperforating injuries to the sc lera may produce aclinical picture similar to that of a retinoblas-toma.Familiarity with the clinical fea ture s of thesepseudoretinoblastomas combined with a thor-ough history and ocular examination shouldenable o ne to readily differentiate these simulat-ing conditions from retinoblastom a.

REFERENCES1. Shields JA, Shields CL. M anageme nt and prognosis of

retinoblastoma. In: Shields JA, Shields CL (eds). Intraocu-lar Tumors. A Text and Atlas. Philadelphia, PA: Saunders ;2. Shields JA. Misconceptions and techniques in themanagement of retinoblastoma. The 1992 Paul HenkindMemorial Lecture. Retina. 1992;12:320-330.3. Ellsworth RM. The practical management of retino-blastoma. Trans A m Ophthalmol Soc. 1969;67:462-534.4 . Shields JA,Shields CL. Differential diagnosis of retino-blastoma. In: Shields JA, Shields CL (eds). lntraocular

Tumors. A Text and Atlas. Philadelphia, PA: Saunders ;5. How ard G M, Ellsworth R M. D ifferential diagnosis of

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retinoblastoma. A statistical survey of 500 children. I.Relative frequency of the lesions which simulate retinoblas-toma. A m JOphthalmol . 1965;60:610-618.6 . Shields JA, Parsons HM, Shields CL, et al. Lesionssimulating retinoblastoma .JPed Ophthalrnol Strabism. 1991;7 . Shields JA, Shields CL, Parson s HM . Review: Differ-ential diagnosis of retinoblastoma. Retina. 1991;11:232-243.8. Shields JA. Ocular Toxocariasis. A review. S u w Oph-thalmol. 1984;28:361-381.9. Shields JA, Shields CL, Suvarnamani C, et al. Retino-blastoma manifesting a s orbi tal cellulitis. A m J Ophthalrnol.

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Diagnostico Diferenciales de Leucocoria

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