senile reticular pigmentary degeneration

6
SENILE RETICULAR PIGMENTARY DEGENERATION WILLIAM T. HUMPHREY, M.D., RICHARD E. CARLSON, M.D., AND JAMES A. VALONE, JR., M.D. Norfolk, Virginia Of 104 consecutive patients with senile reticular pigmentary degen- eration (207 eyes), 85 patients (82%) were more than 60 years old (mean age, 69.2 ± 8.54 years). Forty-nine (47%) were men and 55 (53%) were women. Peripheral visual fields were not characteristically constricted. Al- though most eyes tested had visual acuities of 20/50 or better, 69 eyes (33%) had visual acuities of 20/100 or worse. A total of 136 eyes (66%) had senile macular degeneration at the time senile reticular pigmentary degeneration was first diagnosed, whereas only 43 control eyes (21%) from the same referral population also had senile macular degeneration (P < .001). Macular degeneration was the primary cause for reduced vision when it was noted. In no instance could reduced visual acuity or constricted visual fields be attributed to the senile reticular pigmentary degeneration alone. Senile reticular pigmentary degeneration on routine ophthalmoscopy should alert the clinician to the possibility of co-existing macular degenerative disease. Senile reticular pigmentary degenera- tion has been described as a benign pe- ripheral fundus change noted primarily in older individuals. It has been reported to occur in approximately one third of those who have macular degenerative dis- ease. 1 " 5 We undertook a clinical study to char- acterize this peripheral change further and to relate it to macular degenerative disease. SUBJECTS AND METHODS Between 1981 and 1983, we studied 207 eyes in 104 consecutive patients with Accepted for publication Aug. 21, 1984. From the Department of Ophthalmology, Eastern Virginia Medical School, Norfolk, Virginia. This study was supported by a research grant from Lions Eye Bank and Research, Inc., Norfolk, Virginia, and the Virginia Eye Foundation, Norfolk, Virginia. Reprint request to William T. Humphrey, M.D., 905 Redgate Ave., Norfolk, VA 23507. senile reticular pigmentary degenera- tion. Patients with other types of periph- eral pigmentary disturbance were ex- cluded from the study. Each patient underwent an ophthalmic examination with particular reference to the location of senile reticular pigmentary degenera- tion in the fundus and the presence or absence of co-existing macular degenera- tive disease. We obtained Snellen visual acuity measurements with best correction for all eyes. Goldmann perimetry with best cor- rection (using the II 4 e test object) was performed on 188 eyes. AOH-R-R color testing was performed on 165 eyes. A detailed retinal drawing was done for each eye. Some patients also underwent fluorescein angiography. Because we were particularly interest- ed in relating senile reticular pigmentary degeneration to macular degeneration, we thought it prudent to obtain informa- ©AMERICAN JOURNAL OF OPHTHALMOLOGY 98:717-722, 1984 717

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Page 1: Senile Reticular Pigmentary Degeneration

S E N I L E RETICULAR PIGMENTARY DEGENERATION

W I L L I A M T. H U M P H R E Y , M.D. , R ICHARD E. CARLSON, M.D. , AND JAMES A. V A L O N E , J R . , M.D.

Norfolk, Virginia

Of 104 consecutive patients with senile reticular pigmentary degen­eration (207 eyes), 85 patients (82%) were more than 60 years old (mean age, 69.2 ± 8.54 years). Forty-nine (47%) were men and 55 (53%) were women.

Peripheral visual fields were not characteristically constricted. Al­though most eyes tested had visual acuities of 20/50 or better, 69 eyes (33%) had visual acuities of 20/100 or worse. A total of 136 eyes (66%) had senile macular degeneration at the time senile reticular pigmentary degeneration was first diagnosed, whereas only 43 control eyes (21%) from the same referral population also had senile macular degeneration (P < .001). Macular degeneration was the primary cause for reduced vision when it was noted. In no instance could reduced visual acuity or constricted visual fields be attributed to the senile reticular pigmentary degeneration alone.

Senile reticular pigmentary degeneration on routine ophthalmoscopy should alert the clinician to the possibility of co-existing macular degenerative disease.

Senile reticular pigmentary degenera­tion has been described as a benign pe­ripheral fundus change noted primarily in older individuals. It has been reported to occur in approximately one third of those who have macular degenerative dis­ease.1"5

We undertook a clinical study to char­acterize this peripheral change further and to relate it to macular degenerative disease.

SUBJECTS AND METHODS

Between 1981 and 1983, we studied 207 eyes in 104 consecutive patients with

Accepted for publication Aug. 21, 1984. From the Department of Ophthalmology, Eastern

Virginia Medical School, Norfolk, Virginia. This study was supported by a research grant from Lions Eye Bank and Research, Inc., Norfolk, Virginia, and the Virginia Eye Foundation, Norfolk, Virginia.

Reprint request to William T. Humphrey, M.D., 905 Redgate Ave., Norfolk, VA 23507.

senile reticular pigmentary degenera­tion. Patients with other types of periph­eral pigmentary disturbance were ex­cluded from the study. Each patient underwent an ophthalmic examination with particular reference to the location of senile reticular pigmentary degenera­tion in the fundus and the presence or absence of co-existing macular degenera­tive disease.

We obtained Snellen visual acuity measurements with best correction for all eyes. Goldmann perimetry with best cor­rection (using the II4e test object) was performed on 188 eyes. AOH-R-R color testing was performed on 165 eyes. A detailed retinal drawing was done for each eye. Some patients also underwent fluorescein angiography.

Because we were particularly interest­ed in relating senile reticular pigmentary degeneration to macular degeneration, we thought it prudent to obtain informa-

©AMERICAN JOURNAL OF OPHTHALMOLOGY 98:717-722, 1984 717

Page 2: Senile Reticular Pigmentary Degeneration

718 AMERICAN JOURNAL OF OPHTHALMOLOGY DECEMBER, 1984

tion regarding the incidence of macular degeneration in the referral retinal prac­tice from which these data originated. Because most patients in this study with senile reticular pigmentary degeneration were more than 60 years old at the time of examination, and because all but one patient, a black, were white, we exam­ined 207 control eyes in 104 consecutive white patients more than 60 years old for macular degeneration.

Data concerning the incidence of co­existing ocular disease were analyzed by chi-square testing with the Yates correc­tion. We compared the age means with the z statistic.

RESULTS

General findings—Patients with senile reticular pigmentary degeneration ranged in age at initial diagnosis from 46 to 90 years (Fig. 1). Fewer than 10% of patients were younger than 55 years. Of the patients studied, 85 (82%) were more than 60 years old. The mean age of 69.2 ± 8.54 years differed insignificant­ly from that of the control group (66.9 ± 5.69 years). Senile reticular pig­mentary degeneration showed a relative­ly even distribution between the sexes.

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A«· Fig. 1 (Humphrey, Carlson, and Valone). Age

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Visual Acuity Fig. 2 (Humphrey, Carlson, and Valone). Visual

acuity of eyes with senile reticular pigmentary de­generation. CF, counting fingers; HM, hand move­ments; LPO, light perception only.

Of patients examined, 49 (47%) were men and 55 (53%) were women.

Visual acuity—The presence of senile reticular pigmentary degeneration did not necessarily indicate poor vision. In 119 eyes tested (57%), the visual acuity was 20/50 or better (Fig. 2). Yet, 69 eyes tested (33%) had visual acuities of 20/100 or worse at the time of their initial exami­nations. Reduced visual acuity (20/100 or worse) in most of these latter eyes was attributed to macular degeneration— either drusen, retinal pigment epithelial mottling, retinal pigment epithelial de­tachment, or subretinal neovascular membranes. In no instance could re­duced visual acuity be attributed to senile reticular pigmentary degeneration alone.

Color vision—Of 165 eyes tested with AOH-R-R color plates, 76 (46%) were normal. Some degree of dyschromatopsia was recorded in 61 eyes tested (37%). Color perception was not demonstrable with this test in 28 eyes evaluated (17%). Color defects occurred primarily in the eyes with macular degeneration. Al­though severe dyschromatopsia may well have been present in some eyes, we thought it likely that a number of eyes exhibiting defective color perception on the plate test did so because of severely defective visual acuity.

Page 3: Senile Reticular Pigmentary Degeneration

VOL. 98, NO. 6 SENILE RETICULAR PIGMENTARY DEGENERATION 719

Visual fields—Goldmann visual fields with best corrected vision were per­formed on 188 eyes. It was difficult to evaluate the effect of senile reticular pig­mentary degeneration upon the visual field. Of eyes tested, 165 (88%) had 70 degrees of visual field or better in the horizontal meridian. In the 23 eyes (12%) with less than 70 degrees of horizontal visual field, processes other than senile reticular pigmentary degeneration, nota­bly cataract and poor understanding and cooperation, were thought to be contrib­utory. Most patients with senile macular degeneration recognized varying degrees of central scotoma on visual fields. Unlike certain other pigmentary retinal degener­ations, therefore, senile reticular pig­mentary degeneration did not appear to affect the visual field directly.

Peripheral fundus findings—Examined eyes characteristically had a reticular honeycomb pattern of hyperpigmenta-tion in the equatorial region (Fig. 3). Small dot-like drusen were often inter­spersed throughout this peripheral pig­mentary disturbance. The pigmentary disturbance usually occurred in more

Fig. 3 (Humphrey, Carlson, and Valone). Fundus photograph of a patient with senile reticular pigmen­tary degeneration.

than one quadrant. The superior quad­rant was most frequently involved. The nasal quadrant was the second most com­monly involved. The temporal quadrant was least often involved (Figs. 4 and 5). In 89 eyes (43%), senile reticular pigmen­tary degeneration involved 360 degrees of the midperiphery. The vitreoretinal interface on scierai depression was char­acteristically smooth.

Other ocular conditions—Other ocular conditions among patients with senile re­ticular pigmentary degeneration were tabulated to determine whether a corre­lation existed between any of these con­ditions and senile reticular pigmentary degeneration (Table). Fifteen eyes (7%) were aphakic. Forty-eight eyes (23%) had lenticular changes sufficient to explain a reduction in visual acuity to 20/30. A total of 136 (66%) had macular degeneration, either drusen, nondrusen retinal pigment epithelial disease, subretinal neovascu-larization, or some combination of these. Drusen were noted in 92 eyes (44%). Other retinal pigmentary epithelial dis­ease was noted in 63 eyes (30%). Nine­teen eyes (9%) had evidence of leaking subretinal neovascular membranes. By contrast, among control eyes, only 43 (21%) exhibited any type of macular de­generation, 35 (17%) had drusen, and 25 (12%) had nondrusen pigment epithelial disease. Fourteen control eyes (7%) had evidence of subretinal neovascular mem­branes. Therefore, eyes with senile reticular pigmentary degeneration had co-existing macular degeneration significantly more often than did age-matched control eyes from the same prac­tice (P < .001). Although eyes with se­nile reticular pigmentary degeneration were, in particular, more likely than con­trols to have drusen (P < .001) and nondrusen pigment epithelial disease (P < .001), these eyes appeared no more likely to have subretinal neovascular membranes than control eyes.

Page 4: Senile Reticular Pigmentary Degeneration

720 AMERICAN JOURNAL OF OPHTHALMOLOGY DECEMBER, 1984

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Fig. 4 (Humphrey, Carlson, and Valone). Fundus painting demonstrating the characteristic location of the reticular changes in a patient with senile reticular pigmentary degeneration.

Fluorescein angiography—Fluorescein angiography was performed on 77 pa­tients with senile reticular pigmentary degeneration (74%). Certain changes were regularly observed. Areas of hyper-fluorescence within the center of the re­ticular network were thought to be relat­ed to the rearrangement or atrophy of the pigment epithelium, allowing a partial window defect. The "heaped-up" reticu­lar hyperpigmentation stood out as lines

of hypofluorescence, presumably result­ing from the relative blockage of underly­ing choroidal fluorescence. We found no persistent staining or leakage of fluores­cein in the areas of senile reticular pig­mentary degeneration.

DISCUSSION

A number of pigmentary disturbances in the peripheral fundus have been re­ported. Although some of these distur-

Page 5: Senile Reticular Pigmentary Degeneration

VOL. 98, NO. 6 SENILE RETICULAR PIGMENTARY DEGENERATION 721

Fig. 5 (Humphrey, Carlson, and Valone). Number of patients with senile reticular pigmentary degener­ation in each quadrant of the Hindus.

bances have been described in detail, one type, senile reticular pigmentary degen­eration, has received less attention.1"5

Senile reticular pigmentary degenera­tion is usually recognized in older indi­viduals, thus the term "senile." The term "reticular" is used to describe the charac­

teristic equatorial to postequatorial fish­net, honeycomb, or lacy pattern of the disturbed, rearranged pigment epitheli­um noted ophthalmoscopically. The con­dition has been referred to as benign, limited in extent, and signifying a gener­alized aging of the fundus.3 Postmortem studies have shown a reticular pattern of pigment aggregation at the borders of linearly arranged drusen. These changes have been noted primarily in the equato­rial region, the nasal and inferior quad­rants being more frequently involved. Histologie postmortem studies of these eyes have disclosed areas of heaped-up pigment epithelium surrounding the dru­sen.5

The differential diagnosis of senile re­ticular pigmentary degeneration includes pigmentary degenerations of the retinitis pigmentosa types, pigmentary changes secondary to trauma, tumor, inflamma­tion, the reticular dystrophy of Sjögren, and lattice degeneration.6"8 The intact vis­ual fields in our series argued against retinitis pigmentosa. The absence of trau­ma, co-existing tumor, or ophthalmic in­flammatory disease in any of the patients

TABLE SENILE RETICULAR PIGMENTARY DEGENERATION AND ASSOCIATED OCULAR DISEASES

Associated Diseases

Macular deneration Drusen Other retinal pigment

epithelial disease Subretinal neovascular

membrane Aphakia Cataract

Eyes With Senile Reticular Pigmentary Degeneration

(No. = 207) No.

136 92

63

19 15 48

%

66 44

30

9 7

23

P Value

<.001 <.001

<.001

.468

.019

.007

No.

43 35

25

14 31 26

Control (No. =

%

21 17

12

7 15 13

Eyes 207)

P Value

<.001 <.001

<.001

.289

.09

.396

Eyes With Senile

Reticular Pigmentary

Degeneration Undergoing Fluorescein

Angiography (No. = 153)

No. %

121 79 57 37

59 39

16 10.5 13 8.5 14 9.2

Page 6: Senile Reticular Pigmentary Degeneration

722 AMERICAN JOURNAL OF OPHTHALMOLOGY DECEMBER, 1984

examined argued against reticular pig­mentary change on these bases. The mean age of our patients and the absence of characteristic macular pigmentary changes argued against the reticular dys­trophy of Sjögren. The absence of a dis­turbed vitreoretinal interface on scierai depression argued against lattice degen­eration.

Our study reinforced a number of pre­viously reported observations. The aver­age age of 69.2 years, the absence of visual field constriction, and, in the ab­sence of co-existing macular disease, rela­tively good visual acuities suggested a benign age-related process. In regard to the location of the reticular lesions, both our clinical study and the postmortem study of Bastek and associates5 suggested primarily an equatorial location, with the nasal fundus more likely to be involved than the temporal fundus. However, the mild predilection for the superior fundus noted in our series was at variance with the inferior location noted postmortem.5

Although one third of patients with senile macular degeneration have been described as having co-existing senile re­ticular pigmentary degeneration,3 the in­creased frequency of co-existing macular degenerative lesions among individuals with senile reticular pigmentary degener­ation was unexpected and noteworthy. The lack of a corresponding increase in subretinal neovascularization among pa­tients with senile reticular pigmentary degeneration was also unexpected and, at this point, remains poorly explained. Per­haps the macular degeneration associated with senile reticular pigmentary degener­ation is less likely to progress to subreti­

nal neovascularization than other forms of age-related macular degenerative dis­ease.

Although senile reticular pigmentary degeneration itself may not necessarily be correlated with visual impairment, it may be more closely related to macular degeneration than previously thought. During routine indirect ophthalmoscopy, especially in eyes in which cataracts limit postequatorial detail, senile reticular pig­mentary degeneration may be more easi­ly visible than early macular degenerative change. Since macular degeneration is such a common cause of visual loss among older individuals, the presence of senile reticular pigmentary degeneration on routine indirect ophthalmoscopy may suggest a need to evaluate macular struc­tures and functions carefully.

R E F E R E N C E S

1. Deutman, A. F.: The Hereditary Dystrophies of the Posterior Pole of the Eye. Assen, Van Gorcum and Co., 1971, pp. 333-337.

2. Daicker, B.: Lineare Degenerationen des per-ipheren retinalen Pigmentepithels. Albrecht von Graefes Arch. Klin. Exp. Ophthalmol. 186:1, 1973.

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

4. Kanski, J. J.: Peripheral retinal degeneration. Trans. Ophthalmol. Soc. U.K. 95:173, 1975.

5. Bastek, J. V., Siegel, E. B., Straatsma, B. R., and Foos, R. Y.: Chorioretinal juncture. Pigmentary patterns of the peripheral fundus. Ophthalmology 89:1455, 1982.

6. Kurz, G. H., and Zimmerman, L. E.: Vagaries of the retinal pigment epithelium. Int. Ophthalmol. Clin. 2:441, 1962.

7. Deutman, A. F., and Rümke, A. M. L.: Retic­ular dystrophy of the retinal pigment epithelium. Arch. Ophthalmol. 82:4, 1969.

8. Rutnin, U., and Schepens, C. L. : Fundus ap­pearance in normal eyes. III. Peripheral degenera­tions. Am. J. Ophthalmol. 64:1040, 1967.