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Fundus Appearance in Normal Eyes
F U N D U S APPEARANCE IN NORMAL EYES IV.
RETINAL BREAKS AND OTHER FINDINGS
U T H A I R U T N I N , * M.D.,
AND CHARLES L. SCHEPENS,
M.D.
Boston, Massachusetts
The previous papers in this series were concerned with descriptions of the standard appearance of the fundus periphery, devel opmental variations and peripheral degener ations. This presentation is devoted to a de scription of retinal breaks in normal eyes and other findings such as pigment clump ing, sheathed retinal vessels, benign melano mas of the pigment epithelium, white with pressure and so-called pearls. RETINAL BREAKS
In a total of 102 patients, 18 retinal breaks were found in 16 eyes of 14 patients, four females and 10 males. The sex difference is not statistically significant. As the breaks occurred bilaterally twice, sub jects showing asymptomatic retinal breaks represented 13.73% of the total number of individuals and 7.84% of the eyes. Only two of 16 eyes showed multiple retinal breaks. The subjects were almost equally divided between those 40 years or less and those over 40 years. Excluded from the category of retinal breaks were 10 incomplete breaks, seen in the eyes of three subjects, in which the bot tom of the break appeared covered by a thin, gray film (fig. 1-A). Several suspected or doubtful retinal breaks and small—prob ably incomplete—retinal holes resulting from the rupture of the inner wall of a single cystoid cavity were also excluded. Of the 18 complete breaks, one was a horseshoe and 17 were round holes. Size
varied from pinpoint to 1.5 disc diameters; the round breaks were nearly all smaller than one-half disc diameter. Fifteen round breaks were in the oral re gion; the horseshoe tear and two round holes were in the equatorial region. Many of the retinal breaks were related to other visi ble changes in the fundus (table 1). Some changes pointed to possible etiologic factors but, in one instance, there seemed to be no other fundus alteration associated with the retinal break. Similar observations have been reported by others. 1 An attempt was made to classify the reti nal breaks found in normal subjects ac cording to probable etiology. In subjects under 20 years of age very small holes were seen. They were the size of a pinpoint, had smooth edges and were found in the middle of a round area of retinal detachment no greater than 0.25 disc diameter. These tiny holes were either unassociated with other visible chorioretinal disease or adjoined a tiny pigment clump. They were located near the ora serrata posterior to the area of cys toid degeneration. Young subjects with small holes accounted for one half of all in dividuals found to have a retinal break. Failure to observe similar retinal breaks in older subjects probably results from the fact that later in life these breaks change in character either by becoming demarcated, obliterated by chorioretinal degeneration, or by growing larger. For instance, four round holes approximately one-eight disc diameter across with smooth edges and seen in older adults were surrounded by a small area of retinal detachment demarcated by a circular pigmented line (fig. 2-C and E ) . They were not associated with other visible alterations and may represent a later stage of the tiny holes seen in young subjects. Retinal breaks associated with latticelike
From the Department of Retina Research of the Retina Foundation and the Retina Service of the Massachusetts Eye and Ear Infirmary. This work was supported by research grant B-3489 of the National Institute of Neurological Diseases and Blindness, United States Public Health Service. »Fellow of the Retina Foundation (1957-1963) ; Fellow in Ophthalmology, Massachusetts Eye and Ear Infirmary (1957-1961). 1063
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AMERICAN JOURNAL OF OPHTHALMOLOGY TABLE 1 APPEARANCE OF THE PERIPHERAL FUNDUS IN NORMAL EYES RELATION BETWEEN 18 RETINAL BREAKS AND OTHER CHANGES
Minimal retinal detachment Pigment ring Granular tissue Pigment clump Cystoid degeneration Lattice degeneration Chorioretinal atrophy Ora abnormality No visible change
6 4 4 5 3 7 1 1 1
degeneration were of three types. First, round or oval holes of one-fifth disc diameter, with smooth edges, were located inside the area of latticelike degeneration. The second type was represented by one horseshoeshaped tear which was found along the pos terior edge of an area of pigmented lattice like degeneration (fig. 2-A). The third type was seen in one eye where two small breaks were adjacent to the anterior border of an area of latticelike degeneration. Breaks caused by the separation of gran ular tissue from the retina or those located near granular tissue had irregular borders and were located near the ora serrata (figs. 1-B, 2-B, D, E ) . Size varied from one-fifth to one-half disc diameter. Most frequently a granular tag was torn and attached by one side to the edge of the break; sometimes a granular floater was either free in the vitre ous cavity or attached to the cortical vitre ous (fig. 1-B) and, occasionally, the granu lar floater could not be found (fig. 2-E). Five small crescentic retinal tears were seen as a result of a vitreoretinal adhesion to a pigment clump. FINDINGS OF OTHER AUTHORS AND DISCUSSION
The frequent relationship between retinal breaks and other fundus changes has been noted previously,2*»0 particularly the relation to granular tissue, cystoid degeneration, 2Mo anomalies of the ora serrata,4 small areas of pigment clumping 1 ·' and chorioretinal atrophy.··*»
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Retinal breaks were found in histologie specimens when looked for systematically. Hanssen ■·· collected 37 myopic globes with out retinal detachment and noted complete retinal breaks in three (8.2%) and incom plete breaks in four. In a study of 167 eyebank eyes from 101 patients who did not suffer from any known eye disease,2*·0 four retinal breaks were found in four eyes (2.4%). Another similar study was per formed on 494 eyes of 250 consecutive autopsies.**·0 Histologically proven retinal breaks were present in 12 eyes (4.8% of the subjects). No case of bilateral retinal breaks was observed. Adams 10 found a total of six retinal breaks in three of 11 eye-bank eyes. The existence of retinal breaks was rec ognized as early as 1853.11 De Wecker, in 1870, described them as the immediate cause of retinal detachment but failed to draw the logical therapeutic conclusions.12 Leber, in 1882, gave further evidence of the role played by retinal breaks in the pathogenesis of retinal detachment.18 This theory became widely accepted after 1921, when Gonin demonstrated the therapeutic results ob tained by closing retinal breaks.14 Recognition of the absolute need for clos ing the retinal breaks once a retinal detach ment is established led to the commonly ac cepted assumption that, in time, a retinal break necessarily leads to retinal detach ment. That this hypothesis is not completely correct is proven by instances in which the retina did not detach after a retinal break had formed7»·15-"·18··1· and by the fact that, in samples of the general population, the inci dence of asymptomatic retinal breaks is much higher than the occurrence of retinal detachment. It is, therefore, of vital interest to attempt distinguishing between retinal breaks which are likely to lead to retinal detachment and those which are less likely to do. so. For this purpose, we have compared our findings in normal eyes to observations made in other types of selected populations using the same
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the ora serrata, nonprogressive and occur rence does not seem to be related to the pa tient's age. T h e r e are also similarities in both types of subjects : breaks tend to occur near areas showing other pathology, are often located temporally and above and are more frequent in males than females.
method of ophthalmoscopy (table 2 ) . A sin gle break was found in 3 0 . 5 % of 131 eyes with subclinical retinal detachment 2 0 and in 17 to 3 2 % (depending on sex and age) of 2,016 patients with a clinical nontraumatic retinal detachment in one eye. 21 I n normal subjects these percentages were reversed, a single break being found in 14 of 16 eyes ( 8 7 . 5 % ) . I n addition, one half of the nor mal eyes with retinal breaks belonged to pa tients aged 4 0 or less (table 3 ) — a n age dis tribution which differs significantly from that found in unilateral nontraumatic retinal detachment. 22
OTHER
FINDINGS
O t h e r findings include changes of uncer tain origin, such a s : ( 1 ) pigment clumping, ( 2 ) changes in retinal vessels, ( 3 ) benign melanoma of the pigment epithelium, ( 4 ) white-with-pressure and ( 5 ) pearls.
I n o u r normal subjects, the number of retinal breaks per eye and of subjects af fected did not increase with age. O n e may surmise, therefore, that the retinal breaks found in normal individuals were present early in life and that the aging process affected them little. Another characteristic of our normal subjects is that eyes with breaks located near the ora serrata were six times more numerous than those with breaks near the equator. I n contradistinc tion, patients with unilateral retinal detach ment showed retinal breaks near the equator three times more frequently than breaks near the ora serrata in males and over four times more frequently in females. 21
1. P I G M E N T
CLUMPING
A localized area of increased pigmenta tion without any other changes around it was frequently seen in the fundus. T h e pig ment clump was small, seldom being as large as the disc (fig. 3 - A ) . A total of 29 clumps were seen in 21 eyes of 19 patients. T h e r e were more clumps in the equatorial region than in the oral region but the difference was not statistically significant. O n e spot was seen in the posterior fundus. T h e pigment sometimes invaded the retina, raising the retinal surface overlying it. I n one instance, pigment granules were seen in the vitreous over the spot. All quadrants a p peared equally involved, a n d the subjects affected were almost equally divided be tween those under and over the age of 40 years. Pigment clumps were found in 13 males and six females, a difference which is
I t m a y be concluded, therefore, that breaks observed in normal subjects differ statistically from breaks seen in patients with retinal detachment: breaks in normals are smaller, generally single, located near
TABLE 2 A P P E A R A N C E O F T H E PERIPHERAL F O N D U S I N NORMAL E Y E S
SINGLE AND MULTIPLE RETINAL BREAKS
Retinal Breaks per Eye
Type of Survey No. Normal subjects Subclinical retinal detachment (Schepens5) Ctinical retinal detachment (Schepens and Marden")
Total No. of Eyes
Multiple
Single %
No.
%
No.
%
14
87.S
2
12.5
16
100
40
30.5
91
69.5
131
100
1,386
100
375
27
1,011
73
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AMERICAN JOURNAL OF OPHTHALMOLOGY TABLE 3 APPEARANCE OF THE PERIPHERAL F U N D U S I N NORMAL E Y E S
AGE DISTRIBUTION OF PATIENTS WITH RETINAL BREAKS
Age Group 40 years and under
Over 40 years
No.
No.
%
Normal subjects 8 50 Unilateral nontraumatic retinal detachment (Schepens and Marden22) 494 24.5
%
8 50 1,522
75.5
The distribution of normal subjects between the two age groups differs significantly from that of patients with unilateral nontraumatic retinal de tachment.
not statistically significant. In five cases, an area of pigment clumping was associated with a retinal break ; in two instances a pig ment clump was adjacent to a spot of chorioretinal atrophy (fig. 3-C), and in two other cases, it was surrounded by an area of white-with-pressure. Findings by other authors and discussion. In a study of 304 myopic fundi in 175 sub jects, Rehsteiner found23 pigment clumping in 18 eyes. His cases 36, 46 and 77 are good illustrations of this type of change. In the absence of histologie studies, the nature of pigment clumping is uncertain. As noted by others,248 pigment proliferation is a fre quent response to many stimuli. Inflamma tion due to the mechanical disturbance of traction or secondary to a biochemical irritation could produce pigment clumping. It could also result from a developmen tal anomaly of the pigment epithelium with benign proliferation into the retina.25 Some authors have noted that retinal tears tend to form around pigment clumps located in the peripheral fundus.1·180 2. CHANGES IN RETINAL VESSELS
Peripheral vessels showed excessive pig mentation along a portion of their course in seven eyes of five patients. In three eyes, the vascular change was present in an area
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of latticelike degeneration, one of which also showed a horseshoe-shaped retinal break (fig. 2-A). All patients affected were over 50 years of age. Sheathed vessels, either arterioles or venules, were seen in 14 eyes of 11 patients, eight females and three males. The vessels either had the appearance of copper wire or were white. In all cases except one, the affected vessels were in an area of retinoschisis, marked cystoid degeneration or chorioretinal degeneration. In one exceptional case, a sheathed vessel was seen in the eye of a 20-year-old female with no other visible changes. Unexplained small intraretinal hemor rhages were seen once in the fundus periphery* (fig. 3-C). 3. BENIGN MELANOMAS OF PIGMENT EPITHELIUM
This change appeared as a sharply out lined and darkly pigmented area in the fun dus. The pigmentation was uniform and had a dark, grayish color with tiny and sharply outlined islands of normal fundus. The be nign melanomas were flat and the overlying retina seemed undisturbed. Their size varied between one-fifth of a disc diameter and an area of four to five disc diameters, the most frequent size being one-half to one disc di ameter. No visual field defect was found corresponding to their location. A total of 22 benign melanomas were found in 19 eyes of 19 patients. Their geographic distribution was rather constant as 63.2% were located near the horizontal meridian, and the num ber located in the central area was equal to that found in the periphery (fig. 4). There was a significant preference for the upper temporal quadrant. All age groups appeared equally affected, indicating a nonprogressive * Of 102 subjects, there were two others (59 and 64 years of age) who showed small hemor rhages near the disc without visible changes in the retinal vessels : one was round, one-eighth disc in diameter and the other was flame-shaped. Tiny punctate peripheral retinal hemorrhages were fre quently associated with vitreoretinal traction.
pig. I (Rutnin and Schepens). Retinal breaks seen in fundi considered normal. (A) Temporal fundus periphery with cystoid degeneration and an incomplete retinal break located above the course of a long ciliary nerve and on the disc side of a visible vitreoretinal adhesion. (B) Nasal fundus periphery with granular globule at the base of an ora tooth; the globule, which was torn causing a ret inal tear, is in the vitreous cavity and casts a shadow on the fundus. (The paintings are accurate reproductions of individual fundi.)
Pig. 2 (Rutnin and Schepens). Retinal breaks seen in fundi considered normal. (A) Horseshoe type retinal break with rolled edges on the posterior border of an area of pigmented latticelike degenera tion. (B) Temporal fundus periphery showing white-with-pressure, two pigment clumps, and two granular tags. One of the tags is associated with a small retinal tear surrounded by minimal retinal detachment. fC) Small retinal hole, with minimal retinal detachment, surrounded by demarcation line în an older subject. (D) Two granular patches on either side of which is a small retinal hole. Έ) Deep ora bay with granular globule surrounded by a thin pigment line. A tiny piece was probably torn from the globule, jsing a small retinal tear. caus during a Milan retina r icar. (F) Marked cystoid degeneration with a localized retinal detachment caused by a small retinal break. The latter probably resulted from the rupture of a cystoid cavity. (The paintings are accurate reproductions of individual fundi.)
Pig. 3 {Rutnin and Schepens). Other changes seen in the periphery of normal fundi. A) Composite picture showing shapes and sizes of pigment clumps. B) Composite picture of temporal periphery showing size, shape and location of pearls and emphasizing their relation to other variations near the ora serrata. (C) Temporal area showing cystoid degeneration, white-with-pressure, pigment clump adjacent to area of chorioretinal atrophy, and small intraretinal hemorrhages. (D) Moderate degree of cystoid degeneration, and white-with-pressure on its disc side; an oval area, from which whitewith-pressure is absent, superficially resembles a retinal break. (E) Marked cystoid degeneration bordered by odd shapes of white-with-pressure. {Paintings C, D, and E are accurate reproductions of individual fundi.)
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lesion acquired before birth or early in life. There was no significant sex difference and patients with a deeply pigmented fundus were no more affected than those with a blond fundus. Two benign melanomas were bordered by a small area of chorioretinal atrophy. The benign melanomas could be clearly distinguished from areas of pigment clump ing because they were slate gray, uniform in color, flat—never invading the retina—and had smooth borders. Pigment clumps, which were often smaller than the benign melano mas, were mottled in color and irregular in shape; they were often raised and the pig ment always invaded the retina and some times the vitreous. Grouped pigmentation or bear tracks24"'26 differ clinically from be nign melanomas by their number and ar rangement. The following are signs differentiating benign melanomas of the pigment epitheli um27 from benign melanomas of the choroid: in the pigment epithelium the edges of the changes observed were sharply out lined, whereas, benign choroidal melanomas
Fig. 4 (Rutnin and Schepens). Composite diagram representing the location and size of all the benign melanomas of the pigment epithe lium seen in the eyes of normal subjects. As the right and left eye* were equally affected, all benign melanomas were represented on the diagram of a left eye.
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have feathered edges; and the pigmented areas observed contained tiny and sharply demarcated islands of normal fundus which are not found in benign choroidal melanomas. However, the frequent location of the changes observed near the horizontal meridian re sembles that of benign choroidal melano mas.24b In the absence of microscopic sections, some doubt still remains as to the exact nature of the benign melanomas de scribed. 4. WHITE-WITH-PRESSURE
When scierai pressure was used, the ret ina sometimes appeared as if it was smooth ly detached with fairly sharp borders. White-with-pressure often formed a band parallel to the ora serrata located between equator and ora. Sometimes the ends of the band were fusiform or hornlike but all kinds of shapes were encountered (figs. 3-C, D, E, 5 and 6 ) . When a normal area was surrounded by white-with-pressure, it was sometimes difficult to differentiate the un affected area from a retinal break (fig. 3D). Subretinal changes such as chorioreti-
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1
*%[[§' ^ ί ^ ^ ^ ^ ^ ^ ^ 0
Fig. S (Rutnin and Schepens). Diagrammatic representation of white-with-pressure in normal fundi. (E) Equator. (O) Ora serrata. (I) Solid area of white-with-pressure bordering an area of marked cystoid degeneration. (II) White-with-pressure with a sharp border on the equatorial side and an indefinite border toward the ora serrata. nal degeneration and atrophy, pigment clump ing and the presence of a benign melanoma did not affect the configuration of white-withpressure (figs. 7, 8-1 and I I ) . Early lattice and cystoid degeneration always showed white-with-pressure along their borders (fig 8-II, I I I , I V ) . The borders of white-with-pressure were generally sharp and slightly wavy but some times indefinite in the anterior or posterior border (figs. 3-E and 5-II). Sizes varied from a small island one-eight of a disc di ameter to a band three disc diameters wide extending over one half of the fundus pe riphery. Sometimes the posterior border of a retinoschisis lost its snow flakes and other pathognomonic signs and became continuous with a band of white-with-pressure (fig. 8-III). In other cases, it seemed that the posterior flat portion of an early retinoschi sis exhibited white-with-pressure while its higher and more anterior part did not (fig. 8-IV). There were also instances when
white-with-pressure merged gradually in an area of white-without-pressure, the latter simply being an exaggeration of the first. White-with-pressure was seen in 65 eyes (31.9%) and both eyes were affected in 20 subjects, a significant propensity to bilater alism. All quadrants were equally involved except for the lower nasal quadrant which was relatively unaffected. Sex was not a factor but age was important, the incidence being 4.5% in the second decade and reach ing 66.7% in the eighth decade of life. Findings by other authors and discussion. White-with-pressure was first described by Schepens in 19525 when he noted that there were two forms of very flat (subclinical) retinal detachment in the extreme fundus periphery, one with retinal breaks and one without. He described the affected periph eral retina as giving a watered silk of moire reflection. In 1953, Okamura coined the term "white-with-pressure" to describe a flat peripheral retinal detachment without
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IV
Fig. 6 (Rutnin and Schepens). Diagrammatic representation of areas of white-with-pressure un related to other fundus changes in normal fundi. (E) Equator. (O) Ora serrata. (I) Odd-shaped white-with-pressure bordering moderate cystoid degeneration. (II) Equatorial islands of white-withpressure. ( I l l ) Linear area. (IV) Equatorial band.
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iv
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retinal break.28 Later, white-with-pressure was described as a sign of early retinoschisis.28 Histologically, one area of whitewith-pressure showed a localized attach ment of the collapsed vitreous and reti nal atrophy with absence of internal limiting membrane along the area of vitreoretinal adhesion.30 A number of authors feel that whitewith-pressure is due to a vitreoretinal adhe sion in an area of cystoid degeneration.31 It has been said that white-with-pressure is presented in almost every Negro eye, and that it is not always limited to the fundus periphery.31 From our limited observations of Negro eyes, we believe that white-withpressure is often confused with another condition nearly always present in deeply pigmented fundi : pseudo white-with-pres sure or watered-silk appearance, located on the disc border of the area depressed with a scierai depressor. This appearance moves with the displacements of the scierai depres sor over the globe's surface and lacks the fixed configuration of genuine white-withpressure. We found genuine white-withpressure around cystoid and latticelike de generation, on the disc edge of many areas of retinoschisis and in locations of the pe riphery where the retina appeared clinically normal. 5. PEARLS
Small pearl-like round or oval bodies have been seen in the oral region. Their size varied from pinpoint to pinhead. The small
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er ones appeared to be covered by ciliary epithelium or peripheral retina, whereas larger pearls protruded into the vitreous cavity. Of a total of S3 pearls, 46 (86.8%) were located on an ora tooth and the re mainder on a bay. There was a marked preference for the horizontal meridian and the upper temporal quadrant. In many cases, a pearl was related to granular tissue or to variations of the ora serrata (fig. 3-B). This fact and the absence of the influence of age seem to indicate that pearls are of developmental origin. When the enu cleated eye is opened, a pearl loses the shiny aspect it has by ophthalmoscopy. In a recent histologie study, pearls were described as drusenlike structures which are different from granular tissue and which show the staining properties of an acid carbohydrate.32 Tissue spheres described by Samuels33 and others may perhaps represent pearls. SUMMARY
Retinal breaks were found in 13.73% of 102 normal subjects (7.84% of the eyes). Multiple or bilateral breaks were rare; sub jects under and over 40 years of age were equally affected, and a majority of the breaks were round or oval, very small, lo cated near the ora serrata and related to other fundus changes. Pigment clumping was more frequent near the equator than near the ora serrata and it affected equally young and old sub jects. Perivascular pigmentation or sheathing of
Fig. 7 (Rutnin and Schepens). Diagrammatic representation of areas of white-with-pressure related to other fundus changes. (E) Equator. (O) Ora serrata. (I) White-with-pressure in which three other lesions were seen: two areas of chorioretinal atrophy (left) and one retinal break surrounded by minimal retinal detachment (B). (II) Marked cystoid degeneration and bandlike area of whitewith-pressure which appears unaffected by several small patches of chorioretinal atrophy. ( I l l ) Band like area of white-with-pressure with cystoid degeneration on its anterior border; the white-withpressure appears unaffected by a pigment clump located next to an area of chorioretinal atrophy at the equator. (IV) White-with-pressure unaffected by a large benign nevus of the pigment epithelium. On the disc side of the nevus is a narrow band of chorioretinal atrophy.
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the retinal vessels was most frequently re lated to latticelike degeneration, retinoschi sis, marked cystoid degeneration or chorioretinal degeneration. Benign melanomas of the pigment epi thelium were nearly always one disc diame ter or less in size ; they were most frequent ly located near the horizontal meridian and were equally distributed between the central and peripheral portions of the fundus. All ages appeared equally affected. White-with-pressure often had odd shapes and increased in extent with age. It was distinguished from pseudo white-withpressure, so frequent in Negro fundi. Reti nal changes such as cystoid degeneration and latticelike degeneration were always surrounded by a narrow rim of white-withpressure and retinoschisis frequently showed this change on its border. O t h e r types of chorioretinal degeneration did not seem to affect its extent or location. Pearls are spherical structures located near the ora serrata, particularly near the horizontal meridian, in the upper temporal quadrant and along ora teeth. 99 West Cedar Street (02114) ACKNOWLEDGMENT
We are grateful to O. Pomerantzeff, Dipl. Eng., and to I. Shefler, M.A., for their assistance in statistical evaluation. REFERENCES
1. Dumas, J. and Schepens, C. L. : Chorioretinal lesions predisposing to retinal breaks. Am. J. Ophth. 61:620, 1966.
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2. Teng, C. C. and Katzin, H. M. : An anatomic study of the periphery of the retina, (a) Part I. Nonpigmented epithelial cell proliferation and hole formation. Am. J. Ophth. 34:1237, 1951; (b) Part II. Peripheral cystoid degeneration of the retina; formation of cysts and holes. Am. J. Ophth. 36: 29, 1953; (c) Part III. Congenital retinal rosettes. Am. J. Ophth. 36:169, 1953. 3. Okun, E. : Gross and microscopic pathology in autopsy eyes, (a) Part I. Introduction and long posterior ciliary nerves. Am. J. Ophth. 50:424, 1960; (b) Part II. Peripheral chorioretinal atro phy. Am. J. Ophth. 50:574, 1960; (c) Part III. Retinal breaks without detachment. Am. J. Ophth. 51:369, 1961. 4. Schepens, C. L. : Examination of the ora ser rata region: its clinical significance. Acta XVI Cone. Oph. (Britannia), 1950, v. 2, p. 1384. 5. : Subclinical retinal detachments. Arch. Ophth. 47:593, 1952. 6. O'Malley, P., Allen, R. A., Straatsma, B. R. and O'Malley, C. C. : Paving-stone degeneration of the retina. Arch. Ophth. 73:169, 1965. 7. Arruga, H. : Etiologia y patogenia del desprendimiento de la retina. XIV Cone. Ophth. (Hispania), 1933. (a) v. 2 (1) p. 78-85; (b) v. 2 (1) p. 84, figs. 89, 90, 91. 8. Hanssen, R. : Beitrag zur Histologie des myopischen Auges, insbesondere zur Lückenbildung in der Retina und zur Entstehung der Netzhautablö sung. Klin. Mbl. Auengh. 63:295, 1919. 9. Hanssen, R. : Zur Entstehung der Netzhauta blösung. Klin. Mbl. Augenh. 75:344, 1925. 10. Adams, S. T. : Retinal breaks in eye-bank eyes. Arch. Ophth. 55:254, 1956. 11. Coccius, E. A.: Ueber die Anwendung des Augen-Spiegels nebst Angabe eines neuen Instru mentes. Leipzig, I. Müller, 1853, p. 131. 12. deWecker, L., and Jaeger, E. : Traité des maladies du fond de l'oeil et atlas d'ophtalmoscopie. Paris, Adrien Delahaye, Libraire-Editeur, 1870. 13. Leber, T. : Ueber die Entstehung der Netz hautablösung. Ber. Versamml. ophth. Gesellsch. (Stuttgart), 1882, v. 14, p. 18. 14. Gonin, J. : Le traitement du décollement rétinien. Ann. Ocul. 158:175, 1921. 15. Burch, F. E. : Extensive retinal tear. Am. J. Ophth. 21:669, 1938.
Fig. 8 (Rutnin and Schepens). Diagrammatic representation of areas of white-with-pressure related to other fundus changes. (E) Equator. (O) Ora serrata. (I) Oral and equatorial chorioretinal de generation did not seem to affect the shape or extent of white-with-pressure. (II) Islands of latticelike degeneration were always surrounded by a narrow rim of white-with-pressure; however, more ex tensive areas of white-with-pressure (left) appeared unaffected by the lattice. ( I l l ) Marked cystoid degeneration bordered by early rtinoschisis (represented by RS and horizontal hatching) ; the retin oschisis was itself bordered by white-with-pressure (vertical hatching). (IV) Marked cystoid degenera tion bordered by early retinoschisis (RS and horizontal hatching) ; the posterior border of the retin oschisis showed white-with-pressure which was continuous with retinoschisis; crosshatching indicates where retinoschisis and white-with-pressure appeared superposed.
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16. Colyear, B. H. and Pischel, D. K. : Clinical tears in retina without detachment. Am. J. Ophth. 41:773, 1956. 17. Jeandelize, P. and Baudot, R. : Aspects de déchirure rétinienne sans décollement appréciable: Barrage au décollement. Ann. Ocul. 170 :S1S, 1933. 18. Gonin, J. : Le décollement de la rétine, patho génie-traitement. Lausanne, Payot et Cie, 1934. (a) p. 77; (b) p. 93; (c) table xiv, fig. 2. 19. Lister, W. : Holes in the retina and their clinical significance. Brit. J. Ophth. 8:1, 1924. 20. Schepens, C. L. : Management of subclinical retinal detachment, XVIII Cone. Ophth. (Belgica), 1958, v. 1, p. 1019. 21. Schepens, C. L. and Marden, D. : Data on the natural history of retinal detachment. Further char acterization of certain unilateral nontraumatic cases. Am. J. Ophth. 61:213, 1966. 22. : Data on the natural history of retinal detachment: I. Age and sex relationships. Arch. Ophth. 66:631, 1961. 23. Rehsteiner, K. : Ophthalmoskopische Unter suchungen über Veränderungen der Fundusperiph erie in myopen und senilen Augen. Graefes Arch. Ophth. 120:282, 1928. 24. Hogan, M. J. and Zimmerman, L. E. (ed ) : Ophthalmic Pathology : An Atlas and Textbook. Philadelphia, Saunders, 1962, ed. 2. (a) p. 516; (b) p. 477. 25. Duke, J. R. and Maumenee, A. E. : An un
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usual tumor of the retinal pigment epithelium in an eye with early open-angle glaucoma. Am. J. Ophth. 47:311, 1959. 26. Duke-Elder, W. S. : Textbook of Ophthal mology. St. Louis, Mosby, 1938, v 2, p. 1396. 27. Reese, A. B. and Jones, I. S. : Benign melano mas of the retinal pigment epithelium. Am. J. Ophth. 42:207, 1956. 28. Grignolo, A. : Ophthalmoscopy and other methods of examination. Schepens, C. L. and Re gan, C. D. J. (ed.) : In Controversial Aspects of the Management of Retinal Detachment. Boston, Little, Brown and Co., 1965, p. 3. 29. Shea, M., Schepens, C. L. and von Pirquet, S. R. : Retinoschisis : I. Senile type : a clinical re port of one hundred seven cases, Arch. Ophth. 63:1, 1960. 30. Watzke, R. C. : The ophthalmoscopic sign "white-with-pressure ;" a clinicopathologic correla tion, Arch. Ophth. 66 :812, 1961. 31. Discussion on significance of white-withpressure. Schepens, C. L., and Regan, C. D. J. (ed.) In Controversial Aspects of the Management of Retinal Detachment. Boston, Little, Brown and Co., 1965, p. 51. 32. Lonn, L. I. and Smith, T. R. : Ora serrata pearls: Clinical and histological correlation. Arch. Ophth. 77:809, 1967. 33. Samuels, B. : Opacities of the vitreous. Tr. Ophth. Soc. U.K. 50:414, 1930.
VITAL STAINING OF THE CORNEA AND CONJUNCTIVA W I T H A M I X T U R E OF FLUORESCEIN AND ROSE BENGAL M.
S.
NORN,
M.D.
Copenhagen, Denmark Rose bengal is used less often in practice than fluorescein for staining of the cornea and conjunctiva. I n a series of previous in vestigations, I have tried several vital stains. 1 " 7 Some of these stains have been used before (fluorescein, rose bengal, méth ylène blue, trypan blue, scarlet red, Bismarck b r o w n ) , while others have not been tried for this purpose (alcian blue, Sudan I I I , bromothymol blue, litmus). T h e properties of these stains were evalu ated by slitlamp and by microscope as fol lows: after staining, cells were aspirated From the Department of Ophthalmology, Kommunehospitalet. (Heads: P. Braendstrup and M. S. Norn.) This study was aided by Copenhagen Kommunehospital Centenary Fund.
from the conjunctival fluid, epithelial scrap ings were taken, mucous threads from the inferior fornix were mounted on microscope slides and, in some cases, biopsies were taken. More than 1,000 eyes were examined. These investigations indicated that a mix ture of rose bengal and fluorescein with the addition of preservative may be used for clinical purposes. T h e composition is as fol lows: Rose bengal Fluorescein sodium Sodium chloride Phenylmercuric nitrate Distilled water ad
50 50 45 0.05 5
mg mg mg mg gm
About 10 μΐ of this mixture is instilled into the conjunctival sac. The easiest way is
RETINAL BREAKS AND OTHER FINDINGS
U T H A I R U T N I N , * M.D.,
AND CHARLES L. SCHEPENS,
M.D.
Boston, Massachusetts
The previous papers in this series were concerned with descriptions of the standard appearance of the fundus periphery, devel opmental variations and peripheral degener ations. This presentation is devoted to a de scription of retinal breaks in normal eyes and other findings such as pigment clump ing, sheathed retinal vessels, benign melano mas of the pigment epithelium, white with pressure and so-called pearls. RETINAL BREAKS
In a total of 102 patients, 18 retinal breaks were found in 16 eyes of 14 patients, four females and 10 males. The sex difference is not statistically significant. As the breaks occurred bilaterally twice, sub jects showing asymptomatic retinal breaks represented 13.73% of the total number of individuals and 7.84% of the eyes. Only two of 16 eyes showed multiple retinal breaks. The subjects were almost equally divided between those 40 years or less and those over 40 years. Excluded from the category of retinal breaks were 10 incomplete breaks, seen in the eyes of three subjects, in which the bot tom of the break appeared covered by a thin, gray film (fig. 1-A). Several suspected or doubtful retinal breaks and small—prob ably incomplete—retinal holes resulting from the rupture of the inner wall of a single cystoid cavity were also excluded. Of the 18 complete breaks, one was a horseshoe and 17 were round holes. Size
varied from pinpoint to 1.5 disc diameters; the round breaks were nearly all smaller than one-half disc diameter. Fifteen round breaks were in the oral re gion; the horseshoe tear and two round holes were in the equatorial region. Many of the retinal breaks were related to other visi ble changes in the fundus (table 1). Some changes pointed to possible etiologic factors but, in one instance, there seemed to be no other fundus alteration associated with the retinal break. Similar observations have been reported by others. 1 An attempt was made to classify the reti nal breaks found in normal subjects ac cording to probable etiology. In subjects under 20 years of age very small holes were seen. They were the size of a pinpoint, had smooth edges and were found in the middle of a round area of retinal detachment no greater than 0.25 disc diameter. These tiny holes were either unassociated with other visible chorioretinal disease or adjoined a tiny pigment clump. They were located near the ora serrata posterior to the area of cys toid degeneration. Young subjects with small holes accounted for one half of all in dividuals found to have a retinal break. Failure to observe similar retinal breaks in older subjects probably results from the fact that later in life these breaks change in character either by becoming demarcated, obliterated by chorioretinal degeneration, or by growing larger. For instance, four round holes approximately one-eight disc diameter across with smooth edges and seen in older adults were surrounded by a small area of retinal detachment demarcated by a circular pigmented line (fig. 2-C and E ) . They were not associated with other visible alterations and may represent a later stage of the tiny holes seen in young subjects. Retinal breaks associated with latticelike
From the Department of Retina Research of the Retina Foundation and the Retina Service of the Massachusetts Eye and Ear Infirmary. This work was supported by research grant B-3489 of the National Institute of Neurological Diseases and Blindness, United States Public Health Service. »Fellow of the Retina Foundation (1957-1963) ; Fellow in Ophthalmology, Massachusetts Eye and Ear Infirmary (1957-1961). 1063
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AMERICAN JOURNAL OF OPHTHALMOLOGY TABLE 1 APPEARANCE OF THE PERIPHERAL FUNDUS IN NORMAL EYES RELATION BETWEEN 18 RETINAL BREAKS AND OTHER CHANGES
Minimal retinal detachment Pigment ring Granular tissue Pigment clump Cystoid degeneration Lattice degeneration Chorioretinal atrophy Ora abnormality No visible change
6 4 4 5 3 7 1 1 1
degeneration were of three types. First, round or oval holes of one-fifth disc diameter, with smooth edges, were located inside the area of latticelike degeneration. The second type was represented by one horseshoeshaped tear which was found along the pos terior edge of an area of pigmented lattice like degeneration (fig. 2-A). The third type was seen in one eye where two small breaks were adjacent to the anterior border of an area of latticelike degeneration. Breaks caused by the separation of gran ular tissue from the retina or those located near granular tissue had irregular borders and were located near the ora serrata (figs. 1-B, 2-B, D, E ) . Size varied from one-fifth to one-half disc diameter. Most frequently a granular tag was torn and attached by one side to the edge of the break; sometimes a granular floater was either free in the vitre ous cavity or attached to the cortical vitre ous (fig. 1-B) and, occasionally, the granu lar floater could not be found (fig. 2-E). Five small crescentic retinal tears were seen as a result of a vitreoretinal adhesion to a pigment clump. FINDINGS OF OTHER AUTHORS AND DISCUSSION
The frequent relationship between retinal breaks and other fundus changes has been noted previously,2*»0 particularly the relation to granular tissue, cystoid degeneration, 2Mo anomalies of the ora serrata,4 small areas of pigment clumping 1 ·' and chorioretinal atrophy.··*»
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Retinal breaks were found in histologie specimens when looked for systematically. Hanssen ■·· collected 37 myopic globes with out retinal detachment and noted complete retinal breaks in three (8.2%) and incom plete breaks in four. In a study of 167 eyebank eyes from 101 patients who did not suffer from any known eye disease,2*·0 four retinal breaks were found in four eyes (2.4%). Another similar study was per formed on 494 eyes of 250 consecutive autopsies.**·0 Histologically proven retinal breaks were present in 12 eyes (4.8% of the subjects). No case of bilateral retinal breaks was observed. Adams 10 found a total of six retinal breaks in three of 11 eye-bank eyes. The existence of retinal breaks was rec ognized as early as 1853.11 De Wecker, in 1870, described them as the immediate cause of retinal detachment but failed to draw the logical therapeutic conclusions.12 Leber, in 1882, gave further evidence of the role played by retinal breaks in the pathogenesis of retinal detachment.18 This theory became widely accepted after 1921, when Gonin demonstrated the therapeutic results ob tained by closing retinal breaks.14 Recognition of the absolute need for clos ing the retinal breaks once a retinal detach ment is established led to the commonly ac cepted assumption that, in time, a retinal break necessarily leads to retinal detach ment. That this hypothesis is not completely correct is proven by instances in which the retina did not detach after a retinal break had formed7»·15-"·18··1· and by the fact that, in samples of the general population, the inci dence of asymptomatic retinal breaks is much higher than the occurrence of retinal detachment. It is, therefore, of vital interest to attempt distinguishing between retinal breaks which are likely to lead to retinal detachment and those which are less likely to do. so. For this purpose, we have compared our findings in normal eyes to observations made in other types of selected populations using the same
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FUNDUS APPEARANCE IN NORMAL EYES
the ora serrata, nonprogressive and occur rence does not seem to be related to the pa tient's age. T h e r e are also similarities in both types of subjects : breaks tend to occur near areas showing other pathology, are often located temporally and above and are more frequent in males than females.
method of ophthalmoscopy (table 2 ) . A sin gle break was found in 3 0 . 5 % of 131 eyes with subclinical retinal detachment 2 0 and in 17 to 3 2 % (depending on sex and age) of 2,016 patients with a clinical nontraumatic retinal detachment in one eye. 21 I n normal subjects these percentages were reversed, a single break being found in 14 of 16 eyes ( 8 7 . 5 % ) . I n addition, one half of the nor mal eyes with retinal breaks belonged to pa tients aged 4 0 or less (table 3 ) — a n age dis tribution which differs significantly from that found in unilateral nontraumatic retinal detachment. 22
OTHER
FINDINGS
O t h e r findings include changes of uncer tain origin, such a s : ( 1 ) pigment clumping, ( 2 ) changes in retinal vessels, ( 3 ) benign melanoma of the pigment epithelium, ( 4 ) white-with-pressure and ( 5 ) pearls.
I n o u r normal subjects, the number of retinal breaks per eye and of subjects af fected did not increase with age. O n e may surmise, therefore, that the retinal breaks found in normal individuals were present early in life and that the aging process affected them little. Another characteristic of our normal subjects is that eyes with breaks located near the ora serrata were six times more numerous than those with breaks near the equator. I n contradistinc tion, patients with unilateral retinal detach ment showed retinal breaks near the equator three times more frequently than breaks near the ora serrata in males and over four times more frequently in females. 21
1. P I G M E N T
CLUMPING
A localized area of increased pigmenta tion without any other changes around it was frequently seen in the fundus. T h e pig ment clump was small, seldom being as large as the disc (fig. 3 - A ) . A total of 29 clumps were seen in 21 eyes of 19 patients. T h e r e were more clumps in the equatorial region than in the oral region but the difference was not statistically significant. O n e spot was seen in the posterior fundus. T h e pigment sometimes invaded the retina, raising the retinal surface overlying it. I n one instance, pigment granules were seen in the vitreous over the spot. All quadrants a p peared equally involved, a n d the subjects affected were almost equally divided be tween those under and over the age of 40 years. Pigment clumps were found in 13 males and six females, a difference which is
I t m a y be concluded, therefore, that breaks observed in normal subjects differ statistically from breaks seen in patients with retinal detachment: breaks in normals are smaller, generally single, located near
TABLE 2 A P P E A R A N C E O F T H E PERIPHERAL F O N D U S I N NORMAL E Y E S
SINGLE AND MULTIPLE RETINAL BREAKS
Retinal Breaks per Eye
Type of Survey No. Normal subjects Subclinical retinal detachment (Schepens5) Ctinical retinal detachment (Schepens and Marden")
Total No. of Eyes
Multiple
Single %
No.
%
No.
%
14
87.S
2
12.5
16
100
40
30.5
91
69.5
131
100
1,386
100
375
27
1,011
73
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AMERICAN JOURNAL OF OPHTHALMOLOGY TABLE 3 APPEARANCE OF THE PERIPHERAL F U N D U S I N NORMAL E Y E S
AGE DISTRIBUTION OF PATIENTS WITH RETINAL BREAKS
Age Group 40 years and under
Over 40 years
No.
No.
%
Normal subjects 8 50 Unilateral nontraumatic retinal detachment (Schepens and Marden22) 494 24.5
%
8 50 1,522
75.5
The distribution of normal subjects between the two age groups differs significantly from that of patients with unilateral nontraumatic retinal de tachment.
not statistically significant. In five cases, an area of pigment clumping was associated with a retinal break ; in two instances a pig ment clump was adjacent to a spot of chorioretinal atrophy (fig. 3-C), and in two other cases, it was surrounded by an area of white-with-pressure. Findings by other authors and discussion. In a study of 304 myopic fundi in 175 sub jects, Rehsteiner found23 pigment clumping in 18 eyes. His cases 36, 46 and 77 are good illustrations of this type of change. In the absence of histologie studies, the nature of pigment clumping is uncertain. As noted by others,248 pigment proliferation is a fre quent response to many stimuli. Inflamma tion due to the mechanical disturbance of traction or secondary to a biochemical irritation could produce pigment clumping. It could also result from a developmen tal anomaly of the pigment epithelium with benign proliferation into the retina.25 Some authors have noted that retinal tears tend to form around pigment clumps located in the peripheral fundus.1·180 2. CHANGES IN RETINAL VESSELS
Peripheral vessels showed excessive pig mentation along a portion of their course in seven eyes of five patients. In three eyes, the vascular change was present in an area
DECEMBER, 1967
of latticelike degeneration, one of which also showed a horseshoe-shaped retinal break (fig. 2-A). All patients affected were over 50 years of age. Sheathed vessels, either arterioles or venules, were seen in 14 eyes of 11 patients, eight females and three males. The vessels either had the appearance of copper wire or were white. In all cases except one, the affected vessels were in an area of retinoschisis, marked cystoid degeneration or chorioretinal degeneration. In one exceptional case, a sheathed vessel was seen in the eye of a 20-year-old female with no other visible changes. Unexplained small intraretinal hemor rhages were seen once in the fundus periphery* (fig. 3-C). 3. BENIGN MELANOMAS OF PIGMENT EPITHELIUM
This change appeared as a sharply out lined and darkly pigmented area in the fun dus. The pigmentation was uniform and had a dark, grayish color with tiny and sharply outlined islands of normal fundus. The be nign melanomas were flat and the overlying retina seemed undisturbed. Their size varied between one-fifth of a disc diameter and an area of four to five disc diameters, the most frequent size being one-half to one disc di ameter. No visual field defect was found corresponding to their location. A total of 22 benign melanomas were found in 19 eyes of 19 patients. Their geographic distribution was rather constant as 63.2% were located near the horizontal meridian, and the num ber located in the central area was equal to that found in the periphery (fig. 4). There was a significant preference for the upper temporal quadrant. All age groups appeared equally affected, indicating a nonprogressive * Of 102 subjects, there were two others (59 and 64 years of age) who showed small hemor rhages near the disc without visible changes in the retinal vessels : one was round, one-eighth disc in diameter and the other was flame-shaped. Tiny punctate peripheral retinal hemorrhages were fre quently associated with vitreoretinal traction.
pig. I (Rutnin and Schepens). Retinal breaks seen in fundi considered normal. (A) Temporal fundus periphery with cystoid degeneration and an incomplete retinal break located above the course of a long ciliary nerve and on the disc side of a visible vitreoretinal adhesion. (B) Nasal fundus periphery with granular globule at the base of an ora tooth; the globule, which was torn causing a ret inal tear, is in the vitreous cavity and casts a shadow on the fundus. (The paintings are accurate reproductions of individual fundi.)
Pig. 2 (Rutnin and Schepens). Retinal breaks seen in fundi considered normal. (A) Horseshoe type retinal break with rolled edges on the posterior border of an area of pigmented latticelike degenera tion. (B) Temporal fundus periphery showing white-with-pressure, two pigment clumps, and two granular tags. One of the tags is associated with a small retinal tear surrounded by minimal retinal detachment. fC) Small retinal hole, with minimal retinal detachment, surrounded by demarcation line în an older subject. (D) Two granular patches on either side of which is a small retinal hole. Έ) Deep ora bay with granular globule surrounded by a thin pigment line. A tiny piece was probably torn from the globule, jsing a small retinal tear. caus during a Milan retina r icar. (F) Marked cystoid degeneration with a localized retinal detachment caused by a small retinal break. The latter probably resulted from the rupture of a cystoid cavity. (The paintings are accurate reproductions of individual fundi.)
Pig. 3 {Rutnin and Schepens). Other changes seen in the periphery of normal fundi. A) Composite picture showing shapes and sizes of pigment clumps. B) Composite picture of temporal periphery showing size, shape and location of pearls and emphasizing their relation to other variations near the ora serrata. (C) Temporal area showing cystoid degeneration, white-with-pressure, pigment clump adjacent to area of chorioretinal atrophy, and small intraretinal hemorrhages. (D) Moderate degree of cystoid degeneration, and white-with-pressure on its disc side; an oval area, from which whitewith-pressure is absent, superficially resembles a retinal break. (E) Marked cystoid degeneration bordered by odd shapes of white-with-pressure. {Paintings C, D, and E are accurate reproductions of individual fundi.)
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lesion acquired before birth or early in life. There was no significant sex difference and patients with a deeply pigmented fundus were no more affected than those with a blond fundus. Two benign melanomas were bordered by a small area of chorioretinal atrophy. The benign melanomas could be clearly distinguished from areas of pigment clump ing because they were slate gray, uniform in color, flat—never invading the retina—and had smooth borders. Pigment clumps, which were often smaller than the benign melano mas, were mottled in color and irregular in shape; they were often raised and the pig ment always invaded the retina and some times the vitreous. Grouped pigmentation or bear tracks24"'26 differ clinically from be nign melanomas by their number and ar rangement. The following are signs differentiating benign melanomas of the pigment epitheli um27 from benign melanomas of the choroid: in the pigment epithelium the edges of the changes observed were sharply out lined, whereas, benign choroidal melanomas
Fig. 4 (Rutnin and Schepens). Composite diagram representing the location and size of all the benign melanomas of the pigment epithe lium seen in the eyes of normal subjects. As the right and left eye* were equally affected, all benign melanomas were represented on the diagram of a left eye.
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have feathered edges; and the pigmented areas observed contained tiny and sharply demarcated islands of normal fundus which are not found in benign choroidal melanomas. However, the frequent location of the changes observed near the horizontal meridian re sembles that of benign choroidal melano mas.24b In the absence of microscopic sections, some doubt still remains as to the exact nature of the benign melanomas de scribed. 4. WHITE-WITH-PRESSURE
When scierai pressure was used, the ret ina sometimes appeared as if it was smooth ly detached with fairly sharp borders. White-with-pressure often formed a band parallel to the ora serrata located between equator and ora. Sometimes the ends of the band were fusiform or hornlike but all kinds of shapes were encountered (figs. 3-C, D, E, 5 and 6 ) . When a normal area was surrounded by white-with-pressure, it was sometimes difficult to differentiate the un affected area from a retinal break (fig. 3D). Subretinal changes such as chorioreti-
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AMERICAN JOURNAL OF OPHTHALMOLOGY
DECEMBER, 1967
E
1
*%[[§' ^ ί ^ ^ ^ ^ ^ ^ ^ 0
Fig. S (Rutnin and Schepens). Diagrammatic representation of white-with-pressure in normal fundi. (E) Equator. (O) Ora serrata. (I) Solid area of white-with-pressure bordering an area of marked cystoid degeneration. (II) White-with-pressure with a sharp border on the equatorial side and an indefinite border toward the ora serrata. nal degeneration and atrophy, pigment clump ing and the presence of a benign melanoma did not affect the configuration of white-withpressure (figs. 7, 8-1 and I I ) . Early lattice and cystoid degeneration always showed white-with-pressure along their borders (fig 8-II, I I I , I V ) . The borders of white-with-pressure were generally sharp and slightly wavy but some times indefinite in the anterior or posterior border (figs. 3-E and 5-II). Sizes varied from a small island one-eight of a disc di ameter to a band three disc diameters wide extending over one half of the fundus pe riphery. Sometimes the posterior border of a retinoschisis lost its snow flakes and other pathognomonic signs and became continuous with a band of white-with-pressure (fig. 8-III). In other cases, it seemed that the posterior flat portion of an early retinoschi sis exhibited white-with-pressure while its higher and more anterior part did not (fig. 8-IV). There were also instances when
white-with-pressure merged gradually in an area of white-without-pressure, the latter simply being an exaggeration of the first. White-with-pressure was seen in 65 eyes (31.9%) and both eyes were affected in 20 subjects, a significant propensity to bilater alism. All quadrants were equally involved except for the lower nasal quadrant which was relatively unaffected. Sex was not a factor but age was important, the incidence being 4.5% in the second decade and reach ing 66.7% in the eighth decade of life. Findings by other authors and discussion. White-with-pressure was first described by Schepens in 19525 when he noted that there were two forms of very flat (subclinical) retinal detachment in the extreme fundus periphery, one with retinal breaks and one without. He described the affected periph eral retina as giving a watered silk of moire reflection. In 1953, Okamura coined the term "white-with-pressure" to describe a flat peripheral retinal detachment without
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IV
Fig. 6 (Rutnin and Schepens). Diagrammatic representation of areas of white-with-pressure un related to other fundus changes in normal fundi. (E) Equator. (O) Ora serrata. (I) Odd-shaped white-with-pressure bordering moderate cystoid degeneration. (II) Equatorial islands of white-withpressure. ( I l l ) Linear area. (IV) Equatorial band.
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iv
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retinal break.28 Later, white-with-pressure was described as a sign of early retinoschisis.28 Histologically, one area of whitewith-pressure showed a localized attach ment of the collapsed vitreous and reti nal atrophy with absence of internal limiting membrane along the area of vitreoretinal adhesion.30 A number of authors feel that whitewith-pressure is due to a vitreoretinal adhe sion in an area of cystoid degeneration.31 It has been said that white-with-pressure is presented in almost every Negro eye, and that it is not always limited to the fundus periphery.31 From our limited observations of Negro eyes, we believe that white-withpressure is often confused with another condition nearly always present in deeply pigmented fundi : pseudo white-with-pres sure or watered-silk appearance, located on the disc border of the area depressed with a scierai depressor. This appearance moves with the displacements of the scierai depres sor over the globe's surface and lacks the fixed configuration of genuine white-withpressure. We found genuine white-withpressure around cystoid and latticelike de generation, on the disc edge of many areas of retinoschisis and in locations of the pe riphery where the retina appeared clinically normal. 5. PEARLS
Small pearl-like round or oval bodies have been seen in the oral region. Their size varied from pinpoint to pinhead. The small
1075
er ones appeared to be covered by ciliary epithelium or peripheral retina, whereas larger pearls protruded into the vitreous cavity. Of a total of S3 pearls, 46 (86.8%) were located on an ora tooth and the re mainder on a bay. There was a marked preference for the horizontal meridian and the upper temporal quadrant. In many cases, a pearl was related to granular tissue or to variations of the ora serrata (fig. 3-B). This fact and the absence of the influence of age seem to indicate that pearls are of developmental origin. When the enu cleated eye is opened, a pearl loses the shiny aspect it has by ophthalmoscopy. In a recent histologie study, pearls were described as drusenlike structures which are different from granular tissue and which show the staining properties of an acid carbohydrate.32 Tissue spheres described by Samuels33 and others may perhaps represent pearls. SUMMARY
Retinal breaks were found in 13.73% of 102 normal subjects (7.84% of the eyes). Multiple or bilateral breaks were rare; sub jects under and over 40 years of age were equally affected, and a majority of the breaks were round or oval, very small, lo cated near the ora serrata and related to other fundus changes. Pigment clumping was more frequent near the equator than near the ora serrata and it affected equally young and old sub jects. Perivascular pigmentation or sheathing of
Fig. 7 (Rutnin and Schepens). Diagrammatic representation of areas of white-with-pressure related to other fundus changes. (E) Equator. (O) Ora serrata. (I) White-with-pressure in which three other lesions were seen: two areas of chorioretinal atrophy (left) and one retinal break surrounded by minimal retinal detachment (B). (II) Marked cystoid degeneration and bandlike area of whitewith-pressure which appears unaffected by several small patches of chorioretinal atrophy. ( I l l ) Band like area of white-with-pressure with cystoid degeneration on its anterior border; the white-withpressure appears unaffected by a pigment clump located next to an area of chorioretinal atrophy at the equator. (IV) White-with-pressure unaffected by a large benign nevus of the pigment epithelium. On the disc side of the nevus is a narrow band of chorioretinal atrophy.
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the retinal vessels was most frequently re lated to latticelike degeneration, retinoschi sis, marked cystoid degeneration or chorioretinal degeneration. Benign melanomas of the pigment epi thelium were nearly always one disc diame ter or less in size ; they were most frequent ly located near the horizontal meridian and were equally distributed between the central and peripheral portions of the fundus. All ages appeared equally affected. White-with-pressure often had odd shapes and increased in extent with age. It was distinguished from pseudo white-withpressure, so frequent in Negro fundi. Reti nal changes such as cystoid degeneration and latticelike degeneration were always surrounded by a narrow rim of white-withpressure and retinoschisis frequently showed this change on its border. O t h e r types of chorioretinal degeneration did not seem to affect its extent or location. Pearls are spherical structures located near the ora serrata, particularly near the horizontal meridian, in the upper temporal quadrant and along ora teeth. 99 West Cedar Street (02114) ACKNOWLEDGMENT
We are grateful to O. Pomerantzeff, Dipl. Eng., and to I. Shefler, M.A., for their assistance in statistical evaluation. REFERENCES
1. Dumas, J. and Schepens, C. L. : Chorioretinal lesions predisposing to retinal breaks. Am. J. Ophth. 61:620, 1966.
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2. Teng, C. C. and Katzin, H. M. : An anatomic study of the periphery of the retina, (a) Part I. Nonpigmented epithelial cell proliferation and hole formation. Am. J. Ophth. 34:1237, 1951; (b) Part II. Peripheral cystoid degeneration of the retina; formation of cysts and holes. Am. J. Ophth. 36: 29, 1953; (c) Part III. Congenital retinal rosettes. Am. J. Ophth. 36:169, 1953. 3. Okun, E. : Gross and microscopic pathology in autopsy eyes, (a) Part I. Introduction and long posterior ciliary nerves. Am. J. Ophth. 50:424, 1960; (b) Part II. Peripheral chorioretinal atro phy. Am. J. Ophth. 50:574, 1960; (c) Part III. Retinal breaks without detachment. Am. J. Ophth. 51:369, 1961. 4. Schepens, C. L. : Examination of the ora ser rata region: its clinical significance. Acta XVI Cone. Oph. (Britannia), 1950, v. 2, p. 1384. 5. : Subclinical retinal detachments. Arch. Ophth. 47:593, 1952. 6. O'Malley, P., Allen, R. A., Straatsma, B. R. and O'Malley, C. C. : Paving-stone degeneration of the retina. Arch. Ophth. 73:169, 1965. 7. Arruga, H. : Etiologia y patogenia del desprendimiento de la retina. XIV Cone. Ophth. (Hispania), 1933. (a) v. 2 (1) p. 78-85; (b) v. 2 (1) p. 84, figs. 89, 90, 91. 8. Hanssen, R. : Beitrag zur Histologie des myopischen Auges, insbesondere zur Lückenbildung in der Retina und zur Entstehung der Netzhautablö sung. Klin. Mbl. Auengh. 63:295, 1919. 9. Hanssen, R. : Zur Entstehung der Netzhauta blösung. Klin. Mbl. Augenh. 75:344, 1925. 10. Adams, S. T. : Retinal breaks in eye-bank eyes. Arch. Ophth. 55:254, 1956. 11. Coccius, E. A.: Ueber die Anwendung des Augen-Spiegels nebst Angabe eines neuen Instru mentes. Leipzig, I. Müller, 1853, p. 131. 12. deWecker, L., and Jaeger, E. : Traité des maladies du fond de l'oeil et atlas d'ophtalmoscopie. Paris, Adrien Delahaye, Libraire-Editeur, 1870. 13. Leber, T. : Ueber die Entstehung der Netz hautablösung. Ber. Versamml. ophth. Gesellsch. (Stuttgart), 1882, v. 14, p. 18. 14. Gonin, J. : Le traitement du décollement rétinien. Ann. Ocul. 158:175, 1921. 15. Burch, F. E. : Extensive retinal tear. Am. J. Ophth. 21:669, 1938.
Fig. 8 (Rutnin and Schepens). Diagrammatic representation of areas of white-with-pressure related to other fundus changes. (E) Equator. (O) Ora serrata. (I) Oral and equatorial chorioretinal de generation did not seem to affect the shape or extent of white-with-pressure. (II) Islands of latticelike degeneration were always surrounded by a narrow rim of white-with-pressure; however, more ex tensive areas of white-with-pressure (left) appeared unaffected by the lattice. ( I l l ) Marked cystoid degeneration bordered by early rtinoschisis (represented by RS and horizontal hatching) ; the retin oschisis was itself bordered by white-with-pressure (vertical hatching). (IV) Marked cystoid degenera tion bordered by early retinoschisis (RS and horizontal hatching) ; the posterior border of the retin oschisis showed white-with-pressure which was continuous with retinoschisis; crosshatching indicates where retinoschisis and white-with-pressure appeared superposed.
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16. Colyear, B. H. and Pischel, D. K. : Clinical tears in retina without detachment. Am. J. Ophth. 41:773, 1956. 17. Jeandelize, P. and Baudot, R. : Aspects de déchirure rétinienne sans décollement appréciable: Barrage au décollement. Ann. Ocul. 170 :S1S, 1933. 18. Gonin, J. : Le décollement de la rétine, patho génie-traitement. Lausanne, Payot et Cie, 1934. (a) p. 77; (b) p. 93; (c) table xiv, fig. 2. 19. Lister, W. : Holes in the retina and their clinical significance. Brit. J. Ophth. 8:1, 1924. 20. Schepens, C. L. : Management of subclinical retinal detachment, XVIII Cone. Ophth. (Belgica), 1958, v. 1, p. 1019. 21. Schepens, C. L. and Marden, D. : Data on the natural history of retinal detachment. Further char acterization of certain unilateral nontraumatic cases. Am. J. Ophth. 61:213, 1966. 22. : Data on the natural history of retinal detachment: I. Age and sex relationships. Arch. Ophth. 66:631, 1961. 23. Rehsteiner, K. : Ophthalmoskopische Unter suchungen über Veränderungen der Fundusperiph erie in myopen und senilen Augen. Graefes Arch. Ophth. 120:282, 1928. 24. Hogan, M. J. and Zimmerman, L. E. (ed ) : Ophthalmic Pathology : An Atlas and Textbook. Philadelphia, Saunders, 1962, ed. 2. (a) p. 516; (b) p. 477. 25. Duke, J. R. and Maumenee, A. E. : An un
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usual tumor of the retinal pigment epithelium in an eye with early open-angle glaucoma. Am. J. Ophth. 47:311, 1959. 26. Duke-Elder, W. S. : Textbook of Ophthal mology. St. Louis, Mosby, 1938, v 2, p. 1396. 27. Reese, A. B. and Jones, I. S. : Benign melano mas of the retinal pigment epithelium. Am. J. Ophth. 42:207, 1956. 28. Grignolo, A. : Ophthalmoscopy and other methods of examination. Schepens, C. L. and Re gan, C. D. J. (ed.) : In Controversial Aspects of the Management of Retinal Detachment. Boston, Little, Brown and Co., 1965, p. 3. 29. Shea, M., Schepens, C. L. and von Pirquet, S. R. : Retinoschisis : I. Senile type : a clinical re port of one hundred seven cases, Arch. Ophth. 63:1, 1960. 30. Watzke, R. C. : The ophthalmoscopic sign "white-with-pressure ;" a clinicopathologic correla tion, Arch. Ophth. 66 :812, 1961. 31. Discussion on significance of white-withpressure. Schepens, C. L., and Regan, C. D. J. (ed.) In Controversial Aspects of the Management of Retinal Detachment. Boston, Little, Brown and Co., 1965, p. 51. 32. Lonn, L. I. and Smith, T. R. : Ora serrata pearls: Clinical and histological correlation. Arch. Ophth. 77:809, 1967. 33. Samuels, B. : Opacities of the vitreous. Tr. Ophth. Soc. U.K. 50:414, 1930.
VITAL STAINING OF THE CORNEA AND CONJUNCTIVA W I T H A M I X T U R E OF FLUORESCEIN AND ROSE BENGAL M.
S.
NORN,
M.D.
Copenhagen, Denmark Rose bengal is used less often in practice than fluorescein for staining of the cornea and conjunctiva. I n a series of previous in vestigations, I have tried several vital stains. 1 " 7 Some of these stains have been used before (fluorescein, rose bengal, méth ylène blue, trypan blue, scarlet red, Bismarck b r o w n ) , while others have not been tried for this purpose (alcian blue, Sudan I I I , bromothymol blue, litmus). T h e properties of these stains were evalu ated by slitlamp and by microscope as fol lows: after staining, cells were aspirated From the Department of Ophthalmology, Kommunehospitalet. (Heads: P. Braendstrup and M. S. Norn.) This study was aided by Copenhagen Kommunehospital Centenary Fund.
from the conjunctival fluid, epithelial scrap ings were taken, mucous threads from the inferior fornix were mounted on microscope slides and, in some cases, biopsies were taken. More than 1,000 eyes were examined. These investigations indicated that a mix ture of rose bengal and fluorescein with the addition of preservative may be used for clinical purposes. T h e composition is as fol lows: Rose bengal Fluorescein sodium Sodium chloride Phenylmercuric nitrate Distilled water ad
50 50 45 0.05 5
mg mg mg mg gm
About 10 μΐ of this mixture is instilled into the conjunctival sac. The easiest way is