adultvitelliform macular degeneration: diagnosis and ... · adult vitelliform macular degeneration...

British Journal of Ophthalmology, 1980, 64, 733-740

Adult vitelliform macular degeneration:diagnosis and natural history*GIL A. EPSTEIN AND MAURICE F. RABBFrom the Aaron Fox Retino- Vascular Laboratory, Department of Ophthalmology,Michael Reese Hopsital and Medical Center, Chicago, Illinois 60616, USA

SUMMARY True vitelliform dystrophy rarely appears in the adult population. We describe 10cases in adults of bilateral vitelliform lesions clinically mistaken for Best's disease. Fluoresceinangiography is a useful tool in distinguishing this dystrophy from Best's disease or other diseases.The angiographic findings suggest pigment epithelial disease. Adult vitelliform degeneration may

lead to dry atrophic macular degeneration in a similar fashion as macular drusen. Symptoms andvisual findings in these patients are fairly stable, and may be only slowly progressive in spite ofophthalmoscopic and fluorescein angiographic changes over a period of years. The electro-oculo-gram is useful in separating adult vitelliform macular degeneration from true vitelliform dystrophy.

For the past 70 years the 'poached egg' appear-ance of the macular lesion in vitelliform maculardystrophy has been described in various ways. Theexample presented by Best in 1905 has become thestandard and since has been known as Best'sdisease.' Other descriptive terms are vitelliform,2vitelliruptive macular degeneration,3 vitelline macu-lar degeneration,4 vitelliform dystrophy of thefovea,56 and inherited macular cyst.7The classic presentation of the disease is a bila-

teral and symmetrical maculopathy with an auto-somal dominant mode of inheritance.6 8 The vitelli-form lesion typically measures i to 3 discs diameters,and usually presents before the second decade oflife. Visual acuity is frequently normal but may beminimally decreased in the vitelliform stage ofBest's disease. A mild red-green dyschromatopsiais seen in later stages of the disease.

Fluorescein angiography typically shows hypo-fluorescence in the area corresponding to thevitelliform lesion in Best's disease.9 10 The electro-oculogram (EOG) findings are pathologically sub-normal. Deutman demonstrated subnormal EOGsin family members of patients with proved Best'sdisease, suggesting detection of a carrier state.86

Vitelliform macular dystrophy is believed to be adisease of retinal pigment epithelium.6 8 The intact

*This paper was presented at the Chicago OphthalmologicalSociety on 16 April 1979.

Correspondence to Dr Maurice F. Rabb, c/o Department ofOphthalmology, Michael Reese Hospital and MedicalCenter, 2929 South Ellis, Chicago, Illinois 60616, USA.

vitelliform lesion ultimately leads to chorioretinalatrophy, with the terms 'scrambled egg' and'pseudohypopyon' employed to describe stages inthe natural progression of Best's disease.Gass" 12 described 9 cases of what he termed

'peculiar foveolar macular dystrophy'. His patientshad bilateral, symmetrical, slightly raised yellowsubretinal lesions that were 1/3 disc diameter witha central pigment spot. The onset of the diseasewas primarily in the 30 to 50 year age group, withsymptoms of blurred vision and metamorphopsia.There was a slow progression of visual loss in thesepatients. Gass found normal to slightly abnormalEOG readings in these cases. Fluorescein angio-graphy showed either a hypofluorescent lesion or,more typically, a small ring of hyperfluorescencesurrounding a hypofluorescent foveolar spot. Patho-logical changes were noted in the retinal pigmentepithelium, and there was some question of therelationship of this entity to familial drusen.

In 1977 Fishman et al. described 3 patients withbilateral vitelliform-like lesions who had normalEOG findings.13 These authors used the term'pseudovitelliform macular degeneration' to des-cribe the condition. These patients were all olderthan 45 years of age and had a visual acuity of 20/100or better. Fluorescein angiography showed hyper-fluorescence surrounding the foveolar region, andthey considered this was due to leakage from theperifoveolar capillaries. The authors emphasisedthe use of EOG in differentiating pseudovitelliformdegeneration from Best's disease.Kingham and Lochen described 6 cases of vitelli-

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Physical examination revealed 20/30 vision bila-terally with her present correction of a +0 50sphere in each eye. However, a + 125 spherel~ S b;<Mw1P_improvedthe acuity to 20/25 + in the right eye anda +I1-00 sphere +0-50 cylinder axis 900 resulted in20/25+ vision in the left. The anterior segments,pupils, extraocular movements, and intraocularpressures were within normal limits. Amsler grid,Goldmann perimetry, and colour vision testinggave normal results. Fundus examination disclosednormal-appearing optic discs. However, in bothmacular regions there were i disc diameter, slightlyraised, vitelliform-like lesions (Fig. 1).

Fluorescein angiography showed early hyper-fluorescence in the macular region in a ring-like

Fig. 1 (Case 1) Left macula with vitelliform-like lesion. fashion in a window defect pattern OU. Minimalsubretinal leakage of dye was seen bilaterally in latestages of the angiogram (Fig. 2). The EOG was 2 1in the right eye and 1 7 in the left eye. Examinationof 6 family members failed to disclose any retinalor macular disease. Evaluation of the patient 1 yearlater revealed no change in ocular symptoms. Thebest visual acuity was 20/25+ OU, but her refrac-

7 tion changed to +0 50 sphere in the right eye and+0-50 sphere 4-025 cylinder axis 90° in the left.Amsler grid, colour vision tests, and visual fieldswere unchanged.

In both macular regions the vitelliform lesionshad evolved into flat 'scrambled' lesions secondaryto retinal pigment epithelial changes (Fig. 3). Thefluorescein angiogram showed hyperfluorescence in

-- ~~~~~~~~~~~~awindow defect pattern in both eyes.Fig. 2 (Case 1) Late venous phase angiogram of lefteye showing ring hyperfluorescence with minimalsubretinal leakage in macular region.

form macular degeneration with normal EOGs.t4Angiography in their cases revealed hyperfluores-cence due to pigment epithelial changes. Leakagein the late stages was thought to arise from thechoroid and not the perifoveal capillary network. _We investigated 10 cases of adult vitelliform

lesions. When initially seen, the diagnosis of vitelli- _form macular dystrophy was considered. However,the age of presentation and the results of the retinalfunction tests ruled out Best's disease. Emphasis isdirected to the diagnosis and natural history ofadult vitelliform macular degeneration.

Case reports

CASE 1A 45-year-old black female complained of a gradualprogression of blurring vision and metamorphopsiain the left eye for 3 years. There was no history of Fig. 3 (Case 1) Left macular region I year later.previous medical or ocular disease or ocular trauma. Notice scrambling of vitelliform lesion.

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Fig. 4 (Case 2) Right macular region with ( to314disc diameter raised adult vitelliform lesion (1974).

CASE 2

A 76-year-old white male complained of a gradualdecrease of vision in both eyes, primarily at near

vision, for the past 5 years. This patient was firstdescribed by Fishman et al. in 1977.13 The patientdenied any history of metamorphopsia, photopsia,photophobia, or previous ocular problems. He hadcardiac arrhthmias that were being treated with a

pacemaker. Visual acuity was best corrected to20/400 OU and Jaeger 10 in the right eye and Jaegar8 in the left. There were 2+ anterior cortical, and2+ nuclear sclerotic changes in both lenses, account-ing for some loss of vision. Intraocular pressures,pupils, and extraocular movements were withinnormal limits. Amsler grid studies showed smallareas of distortion in the inferotemporal quadrantin the right eye and in the superonasal quadrant inthe left eye. Colour vision tested by isochromaticplates showed a red-green colour deficiency in botheyes. When the patient was seen in 1974, funduscopicexamination revealed vitelliform-like lesions in botheyes that were approximately i to 3/4 disc diametersin size (Fig. 4). In addition, a I disc diameter pig-mented naevus was noted superior to the leftmacula in 1974. Fluorescein angiography showedlate subretinal leakage of dye in both eyes. AnEOG in 1977 showed a value of 1-86 in the right eyeand 2-08 in the left eye.

-In 1978 ophthalmoscopic examination disclosedhyperpigmentation of the left foveal region at thelevel of the retinal pigment epithelium. Surroundingthis was a ring of hypopigmentation. The naevuswas unchanged. The right eye had an appearance ofcentral areolar choroidal sclerosis (Fig. 5).

Angiographic study revealed ring-like hyper-fluorescence in a window defect pattern surroundingcentral hypofluorescence in the left macular region.

Fig. 5 (Case 2) Late venous angiogram right eyewith central areolar choroidal sclerosis pattern (1978).

In the right eye early hyperfluorescence and adiscrete circular pattern of central areolar choroidalsclerosis was noted (Fig. 4). There was no leakageor pooling of dye in either eye. Electrical studieswere not permitted at this time in view of thepatient's medical condition. Examination of thepatient's 2 children failed to disclose any retinal ormacular pathology.

CASE 3A 70-year-old white female gave a 7- to 8-yearhistory of progressive and gradual decrease ofvision in both eyes. She denied other ocular symp-toms, previous eye disease, or eye trauma. Pertinentmedical history included hypertension medicallycontrolled with a diuretic.

Ocular examination revealed a best correctedvision of 20/200 and 20/60 in the right and left eye,respectively. Near visions were Jaeger 8 in the righteye and Jaeger 3 in the left. The anterior segmentswere normal, except for 1 + nuclear scleroticchanges in botlh eyes. Applanation tonometry waswithin normal limits. Amsler grid showed supero-temporal and inferonasal areas of distortion in theright eye. The Arnsler grid in the left eye was withinnormal limits.Fundus examination from July 1975 revealed a

1/3 disc diameter vitelliform-like lesion that involvedboth macular regions. Fluorescein angiography in1975 disclosed a ring of early hyperfluorescence inboth macular regions. Late photographs demon-strated minimal subretinal leakage of dye.Ophthalmoscopic examination in May 1978

revealed bilateral normal appearing optic discs. Inthe right macula a i disc diameter vitelliform-likelesion was seen and appeared larger. A similar 1/3disc diameter lesion with a central pigment spot was

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Fig. 6 (Case 4) Right eye showing umbilicatedvitelliform lesion with central hyperpigmentation.

noted in the left eye. No other retinal or choroidalpathology was noted. Fluorescein angiography in1978 showed an increase in the central hypofluores-cence in the right macular region when comparedwith the 1975 study. However, a ring of earlyhyperfluorescence surrounding the fovea persisted,and late photographs showed minimal subretinalleakage of dye. Angiography of the left eye showedhyperfluorescence in the macular region in a windowdefect pattern with no leakage detected, consistentwith dry atrophic macular degeneration. An EOGwas not permitted. Examination of the patient'sson did not disclose macular pathology.

CASE 4A 63-year-old white male noted blurring of vision,and metamorphopsia in both eyes for the past 2years. He denied previous medical or ocular prob-lems and there was no history of ocular trauma. Hewas referred to our fluorescein angiography labora-tory in August 1977, and visual acuity was 20/30OU at that time. Ophthalmoscopically, i discdiameter umbilicated vitelliform-like lesions werenoted in both eyes (Fig. 6). Angiography revealedearly hyperfluorescence in a ring-like configuration,which persisted as a window defect.

Examination in 1978 showed a best correctedvisual acuity of 20/30+ in both eyes. The anteriorsegments, extraocular movements, pupils, andintraocular pressures were within normal limits.Amsler grid testing revealed distortion of thecentral 4 squares in both eyes. Colour vision andvisual field testing failed to disclose any abnormali-ties. The ophthalmoscopic and fluorescein examina-tion had not changed from the study done 1 yearpreviously. Examination of 2 family members didnot reveal macular disease. Electrical studies werenot permitted.

Fig. 7 (Case 6) Right eye with small raised vitelliformlesion.

CASE 5A 78-year-old white female gave a 6-month historyof blurred vision in both eyes. She denied otherocular symptoms or previous eye disease. Bestcorrected visual acuity was 20/50 bilaterally. Theexternal and anterior segment examinations of botheyes were normal. Ophthalmoscopy disclosed nor-mal optic disc, but 1/3 disc diameter vitelliform-likelesions were noted in both macular regions. Peri-macular drusen were seen bilaterally. Fluoresceinangiography showed minimal late leakage of dyebeneath the sensory retina in both macular regions,more noticeable in the left eye. The EOG was 2 22in the right eye and 2 40 in the left.

CASE 6A 70-year-old white female had a 6-month historyblurred vision in both eyes. She denied other ocularsymptoms or previous eye disease. Medical prob-lems were denied. Best corrected visual acuity was20/30 in the right eye and 20/40 in the left eye.Anterior segments, pupils, extraocular movements,and intraocul4r pressures were unremarkablebilaterally.Funduscopic examination revealed vitelliform-

like lesion in both macular regions (Fig. 7). Noevidence of peripheral retinal disease was noted.Fluorescein angiography showed early hyper-fluorescence in the macular regions which persistedas a window defect suggestive of retinal pigmentepithelial changes. The EOG was within normallimits bilaterally (2 19 OD, 2 58 OS).

CASE 7A 43-year-old white female described blurred visionin both eyes since 1961. She was given glasses atthat time, with resultant visual improvement. In1974 she had a decline in vision with metamorphop-

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Fig. 8 (Case 7) Right eye with i to 113 raised adultvitelliform lesion (1974).

pia in both eyes, the right eye being worse than theleft. Best corrected visual acuity at that time was10/40 in the right eye and 20/25 in the left eye.Ophthalmoscopic examination in 1974 revealedbilateral macular vitelliform-like lesions (Fig. 8).Angiography revealed a faint ring of hyperfluores-cence in the foveal region. In 1975 electrical studieswere normal, and the patient was told that shedid not have Best's disease.

In 1977 she had recurrent, blurred vision. Exami-nation at another institution revealed vision acuitiesof 20/50 in the right eye with a +2 00 sphere and20/30 in the left eye with a + 1-75 sphere. Theremainder of the ocular examination was withinnormal limits. Electro-oculograms at that timerevealed a light peak to dark trough ratio of 1-37in the right eye and 1-66 in the left eye. On thebasis of the EOG findings her condition was diag-nosed as hereditary vitelliform macular degeneration.

In March 1978 the patient had a persistent

Fig. 9 (Case 7) Right eye 41 years later showing anincrease in central hyperpigmentation of the macularlesion (1978).

decrease in visual acuity with metamorphopsia,primarily in the right eye. There were no medicalproblems or any use of medications. Her mother andsister also had a history of ocular problems. Thepatient's visual acuity was correctable to 20/40,Jaeger 3 with a -2 75 sphere +0 50 cylinder axis700 in the right eye, and 20/30, Jaeger I with a+1 75 sphere in the left. The Amsler grid showeddistortion of the central 4 squares in the right eye.However, no visual field defect could be detected byGoldmann perimetry. Amsler grid and visual fieldswere normal in the left eye. Colour vision studieswere normal bilaterally.

Ophthalmoscopically, bilateral normal optic discswere seen. However, in each macular region therewas an approximate 1/3 disc diameter vitelliform-like lesion. In the right foveolar region there was asmall focal area of cenitral hyperpigmentation atthe level of the retinal pigment epithelium that wasnot seen ophthalmoscopically in 1974 (Fig. 9).

Fluorescein angiography revealed more hyper-fluorescence, which persisted as a window-defectpattern in both eyes when compared with the 1974angiogram (Fig. 10). There was no evidence ofleakage or pooling of fluorescein. Repeated electricalstudies were performed. The EOCi was normal inthe left eye (1 85) and borderline low in the righteye (1-66).

CASE 8A 63-year-old white female, mother of case 7, gavea history of blurred vision and poor night vision inboth eyes since age 45, the right eye being worsethan the left. The symptoms had been stable for thepast 3 to 4 years. Previous medical problems inclu-ded Hashimoto's disease, which was treated medi-cally with levothyroxine sodium 0 1 mg/day. She

Fig. 10 (Case 7) Midvenous phase fluorescein angiogramwith multiple RPE window defects in right macula. Therewas no leakage present in later photographs (1978).

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denied previous ocular disease or history of eyetrauma.The ocular examination revealed a best corrected

vision of 20/80, Jaeger 4 in the right eye, and 20/40,Jaeger 2 in the left eye. Anterior segments, extra-ocular movements, pupils, and intraocular pressureswere all within normal limits. Colour vision byisochromatic colour plates was normal bilaterally.Amsler grid studies showed distortion of the central4 squares in the right eye. However, no field losscould be detected by Goldmann perimetry. Ophthal-moscopy revealed a I to 1/3 disc diameter, slightlyelevated, vitelliform-like lesion in the right eye. Theleft eye appeared more consistent with a dry atro-phic macular degenerative process.

Fluorescein angiography of the right eye disclosedan early hyperfluorescent ring type window defectin the macular region. Fluorescein angiography ofthe lesion in the left posterior pole revealed asimilar window defect type of hyperfluorescence.The EOG was normal bilaterally, with light to darktrough ratios of 2 01 in the right eye and 1 90 in theleft eye.

CASE 9A 78-year-old woman had a gradual onset of blurredvision and distortion in both eyes since 1969. Shehad no refractive change or decrease in her visualstatus for the past 2 years. Ocular trauma or pre-vious ocular disease was denied. Her medicalproblems included hypertension, atheroscleroticcardiovascular disease, and thyroid problems. Hermedications included digoxin and a diuretic.

Visual acuity was 20/80 Jaeger 3 in the right eye,and 20/60, Jaeger 3 in the left eye. Pupils, extraocularmovements, and intraocular pressures were allwithin normal limits. The lens showed early nuclearsclerotic changes in both eyes. Amsler grid, visual

Fig. 11 (Case 9) Right eye adult vitelliform lesion inmacula. Drusen are also present (1975).

field, and colour vision were within normal limitsbilaterally.Examination of the 1975 photographs revealed

bilateral 1/3 disc diameter, vitelliform-like lesions inboth eyes (Fig. 11). There was also diffuse retinalpigment epithelial thinning that exposed the under-lying choroidal vasculature. Multiple punctatehypopigmented lesions at the level of the retinalpigment epithelium were seen in the left eye thatwere suggestive of drusen.

Fluorescein angiography in 1975 showed earlyhyperfluorescence in a doughnut type fashion thatinvolved both macular regions. In addition smallpunctate lesions fluoresced in the midvenous phase,consistent with typical drusen. In the late angio-graphic stage there was a persistent window-defecttype of hyperfluorescence in the macular region,with no evidence of pooling or leakage of dye.Ophthalmoscopic examination in 1978 revealed

multiple drusen-like changes in the left macularregion that resembled atrophic macular degenera-tion. However, in the right eye, there was increasedpigmentation in the macular region quite differentfrom that in 1975 (Fig. 12). Fluorescein angiographysimilarly revealed early hyperfluorescence of bothmacular areas with evidence of perimacular drusen.Late fluorescein angiography revealed a ring-typewindow defect involving the right macular region.A similar pattern was seen in the left eye. Noevidence of pooling or leakage could be seen.Electrical studies were not permitted.

CASE 10A 63-year-old black female complained of blurredvision in both eyes since 1972. She denied previousocular or medical disease, and family history wasnegative. Best corrected visual acuity was 20/30,Jaeger 2 in each eye. The anterior segments, pupils,

Fig. 12 (Case 9) 3 years later the vitelliform lesionhas disappeared. Angiography was compatible with dryatrophic macular degeneration.

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Adult vitelliform maculardegeneratiol7

extraocular movements, and intraocular pressureswere normal bilaterally. Amsler grid, colour vision,and visual fields were normal in each eye. Ophthal-moscopy revealed bilateral, 1/3 disc diameter vitelli-form-like lesions. In addition there were scatteredparacentral drusen. Angiography revealed hyper-fluorescence in a window defect pattern similar tothat seen in dry atrophic macular degeneration.Electrical studies were not permitted.

Discussion

The differential diagnosis of vitelliform or egg yolklesions should include central serous choroidopathy,Best's disease, inflammatory aetiology (toxoplas-mosis), retinal pigment epithelial detachment,macular drusen, and other macular degenerations(pseudovitelliform and peculiar foveal maculardystrophy). Our 10 patients were unusual in thatthe vitelliform-like lesions were atypical of Best'sdisease: (1) The lesions were smaller in size andwith greater asymmetry. (2) Angiographically theydid not block fluorescence. (3) The lesions occurredin an adult population. (4) EOG findings werenormal. (5) The natural history differs.Symptomatology in adult vitelliform macular

degeneration consisted of decreased vision andmetamorphopsia. The visual acuity was found to besymmetrical in most cases. In 3 cases (1, 6, 7) amore hyperopic correction could improve thevision, which suggested a slight elevation of thesemacular lesions. In later stages of the disease therewas little change in visual acuity in spite of thechanging ophthalmoscopic and angiographic ap-pearance. Acuity was generally better than 20/80,but may slowly decline with severe degenerativechanges. Colour vision was found to be normal inmost instances, though late stages of the diseaseshowed a red-green anomaly.One cannot predict the evolutional genetic pat-

terns from our series of patients in spite of theexamination of family members. However, thereappeared to be a familial predisposition in 1 family(cases 7 and 8). We suggest that all family membersof patients with vitelliform-like lesions are examinedophthalmoscopically.

Fluorescein angiographic patterns may vary. Themost common angiographic finding in the initialpresentation of this entity is perifoveal hyper-fluorescence from pigment epithelial window de-fects, which is caused by focal pigment epithelialatrophy and dispersion. Pigment epithelial disper-sion is commonly associated with clumping ofRPE. The foveolar region remains physiologicallyhypofluorescent. Fishman et al., in their cases ofpseudovitelliform degeneration, indicated that peri-

foveolar capillary leakage accounted for hyper-fluorescence.13 Further follow-up and fluoresceinangiogram of case 2 by stereo angiography revealedthe leakage was subretinal rather than from theperifoveal capillary network. If retinal leakage ofdye did indeed occur, one may expect a cystoid typeconfiguration.As the adult vitelliform lesion matures, an in-

crease in subretinal exudation is noted. This ac-counts for the late subretinal leakage of dye pre-sumably from the choriocapillaris. Subretinalhaemorrhage or angiographic evidence of subretinalneovascularisation was not found in our cases. Thenatural history of the mature adult vitelliformlesion further varies. In I case (2) loss of RPE andchoriocapillaris led to a clinical and angiographicpicture of central areolar choroidal sclerosis.However, in 5 cases (3, 6, 8, 9, and 10) the adultvitelliform lesion disappeared and more closelyresembled dry atrophic macular degeneration.Three cases (5, 9, 10) had coexistent paracentraldrusen.

These clinical and angiographic changes did notcorreleate significantly with changes in visualstatus. Fluorescein angiography should be utilisedto differentiate other causes of vitelliform-likelesions as well as to ascertain the natural course.Gass described in his series a central hyperpig-

mented spot in the fovea, presumed to be secondaryto retinal pigment epithelial clumping.1' A similarfinding was found in our cases (1, 2, 3, 4, 7). Threeof these patients did not show this finding in theinitial examination. However, the central fovealhyperpigmentation did occur in the natural courseof the disease on an average of 2 to 3 years later.

Electrical studies are useful in separating Best'sdisease from adult vitelliform macular degeneration.Krill found cases of retinal pigment epithelium andsensory retinal detachment having normal to slightlysubnormal EOGs."5 In senile macular degenerationand dominant drusen he found normal EOGs. Thefindings in our patients were consistent with thosefound in dry atrophic macular degeneration. Anextinguished or markedly subnormal EOG, whichis found in Best's disease, was not found in ourcases. For those patients in whom a familial ten-dency is a consideration electrical studies furtherdelineate true vitelliform dystrophy from adultvitelliform macular degeneration (see Table 1).Without histopathological studies the location

and the natural course of events cannot be proved.The initial degenerative process may be in theretinal pigment epithelium or choriocapillaris. Gassfound histological evidence of depigmentation,degeneration, and disruption of retinal pigmentepithelium centrally." Also, periodic-acid-Schiff-

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Table 1 Differentiation of adult vitelliform maculardegeneration from vitelliform macular dystrophy

Vitelliformdystrophy

Age on onset Prior to 2nd decade

Size of lesion 1-3 disc diameters

Symmetry + +

Visual acuity Initially normal or

slightly subnormal

Variable declineeventual

Colour vision Red-green anomallyin late stages

Fluorescein Vitelliform lesionangiography hypofluorescent

EOG Subnormal

Genetic aspects Autosomal dominant

Adult vitellifornmdegeneration

Greater than 40 yearsof age

1/3-' disc diameters

Initially 20/30-20/80

Slow progression,vision visually stable

Red-green anomallyin late stages

Vitelliform lesion withring-like RPEwindow defect

Normal

No definitive pattern

positive subpigment epithelial deposition of eosino-philic material was seen paracentrally. We believethat the primary pathological process in adultvitelliform macular degeneration is at the level ofthe retinal pigment epithelium as shown by fluores-cein angiography. However, as the disease pro-gresses, there may be additional degeneration of thechoriocapillaris.The natural progression of the disease shows that

there is a change in the clinical as well as angio-graphic findings in most patients, especially in thelater years of life. The visual acuity, however,usually did not markedly decrease. The eventualclinical picture in 5 cases was indistinguishablefrom that of dry atrophic macular degeneration.Three patients had coexistent paracentral typicaldrusen. It is our belief that adult vitelliform maculardegeneration is a form of dry atrophic maculardegeneration. The adult vitelliform lesion may beequal to macular drusen in forecasting the eventual

clinical picture and natural history of dry atrophicmacular degeneration. However, none of our casesshowed exudative macular degenerative changesin spite of being followed up for periods of morethan 5 years.

We thank Mr Tom Quirk and Mr Burt Green for the excep-tional photography. We acknowledge Frank La Franco,MD, and Andrew Lewicky. MD, for their technical assist-ance. Thanks also to Debbi Campbell for typing the manu-script.

References

'Best F. Ueber eine hereditare Maculoaffektion. Beitragezur Vererbunglehre. Z Augenheilkd 1905; 13: 199-212.2Zanen.Rausen-kyste vitelliform congenital de la macula.Bull Soc Belge Ophtalmol 1950; 96: 544-9.3Krill AE, Morse PA, Potts AM, Klien BA. Hereditaryvitelliruptive macular degeneration. Am J Ophthalmol1968; 61: 1405-114.4Braley AE, Spivey BE. Hereditary viteiline macular degener-ation. Arch Ophthalmol 1964; 72: 743-62.5Deutman AF. Electro oculography in families with vitelli-form dystrophy of the fovea. Arch Ophthalmol 1969; 81:305-16.6Deutman AF. Hereditary Dystrophies of the Posterior Poleof the Eye. Assen: Van Gorcum, 1971.7Sorby A, Savory M, Davey JB, Fraser RJL. Macularcysts; A dominantly inherited affection with a progressivecourse. Am J Ophthalmol 1956; 40: 144-58.8Goldberg MF. Genetic and Metabolic Eye Disease. Boston:Little, Brown, 1974.9Morse PH, Maclean AL. Fluorescein fundus studies inhereditary vitelliruptive macular degeneration. Am JOphthalmol 1968; 66: 485-94.

"Curry HF Moorman LT. Fluorescein photography ofvitelliform macular degeneration. Arch Ophthalmol 1968;79: 705-9.

"Gass JDM. A clinicopathologic study of a peculiar foveo-macular dystrophy. Trans Am Ophthalmol Soc 1974; 72:139-56.2Gass JDM. Stereoscopic Atlas of Macular Diseases, 2nd ed.St Louis: Mosby, 1977.

"Fishman GA, Trimble S, Rabb MF, Fishman M. Pseudo-vitelliform macular degeneration. Arch Ophthalmol 1977;95: 73-6.

14Kingham JO, Lochen GP. Vitelliform macular degenera-tion. Am J Ophthalmol 1977; 84: 531-6.

"Krill AE. The electroretinographic and electro oculo-graphic findings in patients with macular lesions. Trans AmAcad Ophthalmol Otolaryngol 1966; 70: 1063-83.

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