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The Risk of Fellow Eye Visual Loss With Unilateral Retinal Pigment Epithelial Tears Gabrielle Schoeppner, M.D., Elaine L. Chuang, M.D., and Alan C. Bird, M.D. To identify the magnitude of risk of fellow- eye visual loss, we reviewed the records of 43 patients with unilateral central visual loss caused by a tear of detached retinal pigment epithelium seen in our institution over a peri- od of one to 13 years. Loss of vision because of complications of age-related macular disease occurred in 16 of 43 patients (37%) in the first year, seven of 23 patients (30%) in the second year, and eight of 16 patients (50%) in the third year. An additional five patients lost visual acuity between the third and eighth years of follow-up. The cumulative risk of loss of visu- al acuity was 37% in one year, 59% in two years, and 80% in three years. Visual loss occurred in 29 of the 36 eyes as a result of a complication of retinal pigment epithelial de- tachment. The magnitude of risk to the fellow eye was greater than has been documented in unselected age-related macular degeneration. IN AGE-RELATED MACULAR DISEASE, the risk of developing a lesion in the fellow eye in patients with visual loss in the first eye has been vari- ously estimated at a cumulative risk of 12% over four to 27 months, I 36% for one to four years,234% for 3% years," and as an annual risk of 12% to 15%4,6 and 3% to 7%.6 It has been suggested that the overall risk in age-related macular degeneration may not be uniform, and the risk has been correlated with the appear- ance, density of distribution, and fluorescence of the drusen. 3,4,6-11 In 1981, a newly recognized complication of age-related macular disease, tears of detached retinal pigment epithelium, was described." Accepted for publication Sept. 14, 1989. From the Department of Clinical Ophthalmology, In- stitute of Ophthalmology, Moorfields Eye Hospital, London, England. This study was supported by the Medical Research Council Grant G 8709312 N. Reprint requests to Alan C. Bird, M.D., Professorial Unit, Moorfields Eye Hospital, City Road, EC1, London, V 2PD United Kingdom. Since then, it has been determined that people with tears of the pigment epithelium in one eye have a particularly high risk of sustaining an identical lesion in the fellow eye." We had formed a clinical impression that the risk of visual loss in the fellow eye in patients with a pigment epithelial tear in one eye was much higher than in other forms of age-related macu- lar degeneration. We undertook this study to test this observation and to provide data that would be directly comparable to previous in- vestigations of fellow-eye visual loss in patients who had any form of exudative maculopathy in the affected eye. If the magnitude of risk in this subset of patients with age-related macular disease was found to be greater than the group as a whole, it would allow a more accurate prognosis to be given and support the view that differential risk exists within age-related macu- lar disease. It might also stimulate a search for potential prophylaxis. Subjects and Methods Records, including fluorescein angiograms, of the 84 patients who had tears of the retinal pigment epithelium were reviewed. These pa- tients were seen in our clinic between 1976 and 1987. Tears were identified by criteria previous- ly established." The diagnosis was considered acceptable in the acute stage if the bed of the tear was readily recognizable by biomicroscopy and fluorescein angiography with a well- defined margin between the bed of the tear and the hyperpigmented residual detachment. In some patients, the diagnosis was not evident until the second visit if blood obscured the bed of the tear at the time of first examination. A small number of patients were seen more than six months after the initial event with well- defined fibrosis next to hyperpigmented pig- ment epithelium. Forty-eight patients had a tear in one eye and a visual acuity of 20/40 or better in the other eye ©AMERICAN JOURNAL OF OPHTHALMOLOGY 108:683-685, DECEMBER, 1989 683

The Risk of Fellow Eye Visual Loss with Unilateral Retinal Pigment Epithelial Tears

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Page 1: The Risk of Fellow Eye Visual Loss with Unilateral Retinal Pigment Epithelial Tears

The Risk of Fellow Eye Visual Loss With UnilateralRetinal Pigment Epithelial Tears

Gabrielle Schoeppner, M.D., Elaine L. Chuang, M.D., and Alan C. Bird, M.D.

To identify the magnitude of risk of fellow­eye visual loss, we reviewed the records of 43patients with unilateral central visual losscaused by a tear of detached retinal pigmentepithelium seen in our institution over a peri­od of one to 13 years. Loss of vision because ofcomplications of age-related macular diseaseoccurred in 16 of 43 patients (37%) in the firstyear, seven of 23 patients (30%) in the secondyear, and eight of 16 patients (50%) in the thirdyear. An additional five patients lost visualacuity between the third and eighth years offollow-up. The cumulative risk of loss of visu­al acuity was 37% in one year, 59% in twoyears, and 80% in three years. Visual lossoccurred in 29 of the 36 eyes as a result of acomplication of retinal pigment epithelial de­tachment. The magnitude of risk to the felloweye was greater than has been documented inunselected age-related macular degeneration.

IN AGE-RELATED MACULAR DISEASE, the risk ofdeveloping a lesion in the fellow eye in patientswith visual loss in the first eye has been vari­ously estimated at a cumulative risk of 12%over four to 27 months, I 36% for one to fouryears,234% for 3% years," and as an annual riskof 12% to 15%4,6 and 3% to 7%.6 It has beensuggested that the overall risk in age-relatedmacular degeneration may not be uniform, andthe risk has been correlated with the appear­ance, density of distribution, and fluorescenceof the drusen. 3,4,6-11

In 1981, a newly recognized complication ofage-related macular disease, tears of detachedretinal pigment epithelium, was described."

Accepted for publication Sept. 14, 1989.From the Department of Clinical Ophthalmology, In­

stitute of Ophthalmology, Moorfields Eye Hospital,London, England. This study was supported by theMedical Research Council Grant G 8709312 N.

Reprint requests to Alan C. Bird, M.D., ProfessorialUnit, Moorfields Eye Hospital, City Road, EC1, London,V 2PD United Kingdom.

Since then, it has been determined that peoplewith tears of the pigment epithelium in one eyehave a particularly high risk of sustaining anidentical lesion in the fellow eye." We hadformed a clinical impression that the risk ofvisual loss in the fellow eye in patients with apigment epithelial tear in one eye was muchhigher than in other forms of age-related macu­lar degeneration. We undertook this study totest this observation and to provide data thatwould be directly comparable to previous in­vestigations of fellow-eye visual loss in patientswho had any form of exudative maculopathy inthe affected eye. If the magnitude of risk in thissubset of patients with age-related maculardisease was found to be greater than the groupas a whole, it would allow a more accurateprognosis to be given and support the view thatdifferential risk exists within age-related macu­lar disease. It might also stimulate a search forpotential prophylaxis.

Subjects and Methods

Records, including fluorescein angiograms,of the 84 patients who had tears of the retinalpigment epithelium were reviewed. These pa­tients were seen in our clinic between 1976 and1987. Tears were identified by criteria previous­ly established." The diagnosis was consideredacceptable in the acute stage if the bed of thetear was readily recognizable by biomicroscopyand fluorescein angiography with a well­defined margin between the bed of the tear andthe hyperpigmented residual detachment. Insome patients, the diagnosis was not evidentuntil the second visit if blood obscured the bedof the tear at the time of first examination. Asmall number of patients were seen more thansix months after the initial event with well­defined fibrosis next to hyperpigmented pig­ment epithelium.

Forty-eight patients had a tear in one eye anda visual acuity of 20/40 or better in the other eye

©AMERICAN JOURNAL OF OPHTHALMOLOGY 108:683-685, DECEMBER, 1989 683

Page 2: The Risk of Fellow Eye Visual Loss with Unilateral Retinal Pigment Epithelial Tears

684 AMERICAN JOURNAL OF OPHTHALMOLOGY December, 1989

at the time of their first visit. In the good eye,drusen were identified in the fundus, but therewas no evidence of exudative complications ofage-related macular degeneration. Of thisgroup, four patients were not available forfollow-up; two had left the country after theirinitial examination, and two died severalmonths after the initial event. A fifth patientcould not be included because the tear wasprecipitated by photocoagulation to an avascu­lar pigment epithelial detachment in the felloweye within a year of the occurrence of a tear inthe first eye. The 43 remaining patients wereincluded in the study.

Visual loss was defined as reduction of acuityin the fellow eye to a level of 20/80 or worsebecause of lesions known to complicate age­related macular disease, namely detachment ofthe retinal pigment epithelium, subretinal neo­vascularization, and geographic atrophy. Allpatients who had not lost vision were invitedfor follow-up examinations in 1986 and 1988. Atthe initial visit and each subsequent visit, thebest-corrected visual acuity was recorded andophthalmoscopy was undertaken, which in­cluded color photography and fluorescein angi­ography if there were new symptoms. Loss ofvisual acuity was recorded, and any new le­sions at the macula were identified.

Results

Of the 43 patients included in the study, twowere seen for only one year, and two more werewithdrawn from analysis at the end of one yearbecause they received prophylactic photocoag­ulation to Bruch's membrane at that time. Theprophylactic photocoagulation was undertakenin the good eye in an attempt to prevent pig­ment epithelial detachment. Thus 43 patientswere examined at intervals for one year, and 39patients were followed up thereafter. The aver­age age at the time of the initial tear was 70years; 27 patients were women, and 13 patientswere men.

Thirty-six patients were reexamined untilvisual loss occurred. No patients had visualloss from a cause other than age-related macu­lar disease, and in no patient was a macularlesion identified that was amenable to photoco­agulation. The seven patients who retainedgood visual acuity (20/40 or better) included thetwo who received prophylactic laser treatmentand the two who were followed up for only oneyear. The remaining three have been followed

up for seven, eight, and 13 years after the initialevent.

In the first year of follow-up, 16 of 43 patientslost visual acuity (37%) in the fellow eye. Visualacuity was lost in seven of the 23 patients (30%)in the second year, and in eight of the remain­ing 16 patients (50%) in the third year. Addi­tionally, one patient lost visual acuity in thefourth year, one in the fifth year, two in thesixth year, and one in the seventh year. Thecumulative loss of fellow-eye visual acuity was37% in the first year, 59% in two years, and 80%after three years.

Visual loss in the fellow eye was most fre­quently precipitated by detachment of the pig­ment epithelium. In 23 eyes, pigment epithelialdetachments developed. Of these, six had well­defined subpigment epithelial new vessels ap­parent on angiographic examination, and 11had indirect evidence of neovascularization asshown by subretinal hemorrhage or exudateand notching before visual loss. In 15 of these23 eyes, a tear supervened. An additional sixpatients had tears of detached pigment epithe­lium at the time of visual loss in the fellow eyewithout our seeing the initial lesion.

Two patients lost vision from primary subret­inal neovascularization, and an additional twohad disciform scars, making it impossible todistinguish between detachment of the pig­ment epithelium and choroidal neovasculariza­tion as the initiating event. Three patients de­veloped geographic atrophy; in each patient,we had observed shallow pigment epithelialdetachments associated with soft drusen beforevisual loss.

Discussion

We believe that the high risk in the fellow eyein patients with a tear in the first eye representsthe natural history of the disorder. The ascer­tainment of patients was complete with theexception of the two who died and the two wholeft the country. The criteria for inclusion inthis study allowed only patients without evi­dence of exudative maculopathy in the felloweye. Visual loss was defined as reduction ofvisual acuity to 20/80; in some patients, weobserved the lesion that caused visual loss forsome time before the visual acuity deterioratedto this level. It is unlikely, therefore, that therisk has been overestimated compared to otherstudies. There is considerable variability in the

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Vol. 108, No.6 Retinal Pigment Epithelial Tears 685

protocol of previous investigations. One studyestablished loss of visual acuity as the endpoint." whereas others used the developmentof a lesion likely to cause visual loss. 1,2,4-6 Somedata represent a cumulative risk over a setnumber of years, I,;! whereas others represent anannual risk.t" Our data imply a risk of fellow­eye involvement in this subset of patients thatis greater than twice that in patients with exu­dative maculopathy in general.

Complications of pigment epithelial detach­ment accounted for the visual loss in mostpatients, indicating that the lesion in the felloweye mirrored the lesion in the first. Most, butnot all, pigment epithelial detachments eventu­ally tore. This propensity for bilateral tears wasobserved previously."

The findings of this study are in accord withthe concept of differential risk in patients withage-related macular degeneration, which is de­termined by variation in the density, site, andchemical composition of drusen. 4,6-11 It has beenproposed that the characteristics of drusen de­termine the form of lesion that may occur inresponse to Bruch's membrane change. It hasalso been recognized that symmetry exists be­tween the two eyes in clinically detectableBruch's membrane changes3,4,6,7 despite the con­siderable variation that exists between theeyes. Thus it is not surprising that a correlationexists between the form of the lesion in oneeye, and the form and magnitude of risk in theother.

If the magnitude of risk for visual loss in thesubset of patients with pigment epithelial tearsis so high, there is a need to seek a logicalapproach to prophylactic therapy. It would bevaluable to ascertain the risk factors. An analy­sis of drusen in the fellow eye of patients withtears of the retinal pigment epithelium showedthem to be confluent and hypofluorescent onfluorescein angiography." It has been suggest­ed that the hypofluorescence implies extensivelipid deposition in Bruch's membrane, whichwould impede water flow and predispose thepigment epithelium to detach." These findingsimply that attempts at prophylaxis should bedirected toward reducing the volume of waterpumped by the pigment epithelium, or increas­ing the hydraulic conductivity of Bruch's mem­brane.

References

1. Teeters, V. W., and Bird, A. c.. The develop­ment of neovascularization of senile disciform macu­lar degeneration. Am. J. Ophthalmol. 76:1, 1973.

2. Chandra, S. R., Gragoudas, E. 5., Friedman,E., Van Buskirk, E. M., and Klein, M. L.: Naturalhistory of disciform degeneration of the macula. Am.I. Ophthalmol. 78:579, 1974.

3. Gass, J. D M.: Drusen and disciform maculardetachment and degeneration. Arch. Ophthalmol.90:206, 1973.

4. Gregor, A., Bird, A. c., and Chisholm, 1. H.:Senile disciform macular degeneration in the secondeye. Br. J. Ophthalmol. 61:141, 1977.

5. Bressler,S, B., Bressler, N. M., Fine, S. L.,Hillis, A., Murphy, R. P., Olk, R. J., and Patz, A.:Natural course of choroidal neovascular membraneswithin the foveal avascular zone in senile maculardegeneration. Am. J. Ophthalmol. 93:157, 1982.

6. Strahlman, E., Fine, S. L., and Hillis, A.: Thesecond eye of patients with senile macular degenera­tion. Arch. Ophthalmol. 101:1191, 1983.

7. Gragoudas, E. 5., Chandra, S. R., Freidman,E., Klein, M, L., and Van Buskirk, M.: Disciformdegeneration of the macula. II. Pathogenesis. Arch.Ophthalmol. 94:755, 1976.

8. Siddy, W. E., and Fine, S. L.: Prognosis of pa­tients with bilateral macular drusen. Ophthalmology91:271, 1984.

9. Bressler, N. M., Bressler, S. B., Seddon, I. M.,Gragoudas, E. 5., and Jacobson, L.: Clinicalcharacteristics of drusen in patients with exudativeversus non-exudative age-related macular degenera­tion. Retina 8:109, 1988.

10. Pauleikhoff, D., Marshall, J., and Bird, A. c..Histochemical and morphological correlation of ag­ing changes in Bruch's membrane. ARVO abstracts.Supplement to Invest. Ophthalmol. Vis. Sci. Phila­delphia, J. B. Lippincott, 1989, p. 153.

11. Bird, A. C,; and Marshall, J.: Retinal pigmentepithelial detachments in the elderly. Trans. Oph­thalmol. Soc. U.K. 105:674, 1986.

12. Hoskin, A., Bird, A. c., and Sehrni, K.: Tearsof detached retinal pigment epithelium. Br. J. Oph­thalmol. 65:417, 1981.

13. Chuang, E. L., and Bird, A. c.. Bilaterality oftears of the retinal pigment epithelium. Br. J. Oph­thalmol. 72:417, 1988.

14. --:Repair after tears of the retinal pigmentepithelium. Eye 2:106, 1988.

15. --:The pathogenesis of tears of the retinalpigment epithelium. Am. J. Ophthalmol. 105:285,1988.