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Retinal Pigment Epithelial Folds Associated with Retinal Pigment Epithelial Detachment in Macular Degeneration
Retinal Pigment Epithelial Folds Associated with Retinal Pigment Epithelial Detachment in Macular Degeneration HOWARD SCHATZ, MD, H. RICHARD McDONALD, MD, ROBERT N. JOHNSON, MD
Abstract: The authors reviewed six eyes that had a shallow retinal pigment epithelial detachment with the retinal pigment epithelium (RPE) thrown into folds. A contraction of subpigment epithelial fibrovascular tissue causes a folding of the overlying, intimately adherent RPE. Retinal pigment epithelial folds are a sign of subretinal neovascularization and are another manifestation of age-related macular degeneration. Ophthalmology 1990; 97:658-665
We reviewed six eyes in six patients with retinal pigment epithelial folds associated with a retinal pigment epithelium (RPE) detachment, which was also associated with macular degeneration. We herein describe the clinical findings and fluorescein angiographic characteristics of this clinical sign. We also propose a pathogenesis.
METHODS We reviewed the records of six patients with the diagnosis of retinal pigment epithelial folds. Each patient had a full history and ocular examination, including slit-lamp biomicroscopy, stereo fundus photography, and fluorescein angiography. Follow-up examinations, done at variable intervals depending on the clinical course, included slit-lamp biomicroscopy, fundus photography, and, when indicated, fluorescein angiography. Originally received: November 10. 1989. Revision accepted: January 25. 1990. Presented at the American Academy of Ophthalmology Annual Meeting. New Orleans. Ocl/Nov 1989. Supported by the Retina Research Fund of St. Mary's Hospital and Medical Center. San Francisco. Califomia. Reprint requests to Howard Schatz. MD. 1 Daniel Burnham Ct. #210C. San Francisco. CA 94109.
658
RESULTS Six eyes in six patients (5 women, I man) had retinal pigment epithelial folds. The patients ranged in age from 71 to 100 years (average, 81 years). The visual acuity of the involved eye at the time the diagnosis of the retinal pigment epithelial folds was made and at the last follow-up is shown in Table 1. In all six eyes, retinal pigment epithelial folds were present in the overlying shallow detachment of the pigment epithelium. Subretinal neovascularization, associated with the shallow detachment of the retinal pigment epithelium (RPE), was present in all six eyes (Figs 1, 2). Four of the eyes were not treated. Disciform scars developed in three eyes. In one patient (case 3), the follow-up was only 1 month because the patient was 100 years of age and too weak to return for reexamination. In one eye (case 5), the area of obvious subretinal neovascularization was treated with laser photocoagulation, but the remainder of the folded area was not treated; a disciform scar developed in this eye in 4 months (Fig 3). In one eye (case 6), the pigment epithelial folded area was located mostly juxtafoveal, with only a very small edge of the folded area in the fovea . All of the folded area outside of the central fovea was treated. This eye did well (Fig 4). In all six patients, the fellow eye showed signs of agerelated macular degeneration, including disciform scar (3 eyes) and drusen (3 eyes).
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Table 1. Visual Acuity of the Involved Eye at Diagnosis and Last Follow-up
Patient No.
Age (yrs)/ Race/Sex
Visual Acuity in Eye with Retinal Pigment Epithelial Folds
1
76/W/F 77/W/M 100/W/F 81/W/F 71 /W/F 76/W /F
20/100 3/200 20/100 20/70 20/40 20/50
2 3 4 5 6
W = white; SRNV
Laser Treatment
Length of Follow-up
Final Visual Acuity
No No No No To obvious SRNV To most of folded area
6 mos 1 yr 1 mo 1 yr 4 mos 3V2 yrs
10/200 3/200 20/100 3/200 2/200 20/50
Status of Macula Disciform scar Disciform scar No change Disciform scar Disciform scar Quiet laser scar; no recurrence of SRNV
Fellow Eye Disciform scar Drusen Drusen Disciform scar Drusen Disciform scar
= subretinal neovascularization.
Fig 1. Case I. A 76-year-old white woman with visual acuity of 20/ 100 in the left eye. A, red-free photograph of the left macula. Notice the dark and light lines in the central macula. The whole macula was detached. B, arteriovenous phase fluorescein angiogram of the left macula. There is a small patch of subretinal neovascularization in the central macula. This entire area is encircled by a detached and folded RPE. Notice the alternating hyper- and hypofluorescent curvilinear lines. C, late phase fluorescein angiogram. There is a shallow detachment of the RPE throughout the macula and a small detachment of the sensory retina centrally.
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Fig 2. Case 4. A 81-year-old white women with a visual acuity of 20170 in the right eye. A, red-free photograph of the right macula. Notice the curvilinear pigmented lines in the central macula. There is a patch of subretinal neovascularization temporally with subretinal hemorrhage. B, early arteriovenous phase fluorescein angiogram. Notice the hyperfluorescence from the subretinal neovascularization and the hypofluorescence from the hemorrhage in the temporal macula. The entire central macula shows folding of the RPE as evidenced by hyper- and hypofluorescent lines. Stereoscopic angiography showed that this whole area was a mounded-up and detached area of RPE. C, late phase fluorescein angiogram. Notice the hyperfluorescent leakage from the subretinal neovascularization temporally. The entire macula is detached from a shallow detachment of the RPE, the folds are evident centrally.
DISCUSSION Clinically, the retinal pigment epithelial folds appeared as fine lines of pigmentation alternating with pale, light-
orange lines. The folds were sometimes fairly straight but more often were curvilinear or irregular. They usually occupied an area of'approximately 0.5 to I disc diameter and, in all cases, were located in the macula. Approximately four to ten pigment epithelial folds were seen in )
Fig 3. Case 5. A 71-year-old white woman with visual acuity of 20/40 in the left eye. A, red-free photograph of the left macula. There is a shallow detachment of the retinal pigment epithelium (RPE) in the macula. Notice the fine RPE folds. There is a shallow sensory detachment inferonasally. B, arteriovenous phase fluoresce in angiogram shows a patch of subretinal neovascularization just inferonasal to the fovea. The remaining central macular area shows pigment epithelial folds in a detached RPE. C, later phase fluorescein angiogram shows leakage from the subretinal neovascularization inferonasally and hyperfluorescent staining of the retinal pigment epithelial folds and detachment. D, late phase fluorescein angiogram shows pooling of fluorescein (hyperfluorescence) under the detached and folded RPE. The patch of subretinal neovascularization inferonasally shows leakage and a hypofluorescent rim nasally that represents a fine line of subretinal hemorrhage. E, the patch of subretinal neovascularization, just inferonasal to the left fovea, was then treated with laser photocoagulation. The detached and folded RPE was not treated. Three weeks postoperatively, laser photocoagulation of the subretinal neovascularization 'was done. Arteriovenous phase fluorescein angiogram shows hypofluorescence in the area of the laser treatment. The retinal pigment epithelial folds continue to show alternating lines of hyper- and hypofluorescence. F, the patient was then followed and noted acute reduction in vision. The angiophotograph was taken 4 months after laser photocoagulation. Late phase fluorescein angiogram shows hyperfluorescent staining of the disciform scar.
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Fig 4. Case 6. A76-year-old white woman with visual acuity of 20/50 in the right eye. A, red-free photograph of the right macula. There is a shallow detachment of retinal pigment epithelium (RPE) involving the central and upper portions of the macula. B,arteriovenous phase fluorescein angiogram shows folded and detached RPE. Notice the alternating hyper- and hypofluorescent curvilinear lines that look like a fingerprint. C, late phase fluorescein angiogram. There is leakage of fluorescein under the detached and folded RPE. Notice the multiple hyperfluorescent spots surrounding this area. D, 4 months after laser photocoagulation. Visual acuity was 20/ 50. Notice the flat, quiet laser lesion in the upper half of the macula.
each case, although one case had more than ten fine folds (Fig I). On slit-lamp biomicroscopy or stereoscopic fundus photography, retinal pigment epithelial folds look like mounded-up, thick, wrinkled cloth. The folds are more readily apparent on fluorescein angiography than clinically and are best seen with stereoscopic fluorescein angiography. They appear as alternating white (hyperfluorescent) and dark (hypofluorescent) lines. They sometimes look like a fingerprint (Fig 4). Stereoscopically, they produce a remarkable picture of a 662
mounded-up, folded, or wrinkled clump of tissue (Figs 5, 6). In all six eyes, subretinal neovascularization was present, and the associated clinical and angiographic findings of subretinal neovascularization were seen. The RPE was shallowly detached and folded . Drusen also were often present. In some cases of macular degeneration, new vessels proliferate from the choriocapillaris through a break in Bruch's membrane and dissect between the RPE and Bruch's membrane. 1,2 With time, fibrous tissue is laid
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Fig 5. Case 5. Stereoscopic view of the left macula. Notice the elevated, heaped-up, folded, and detached retinal pigment epithelium in the macula. There is a patch of subretinal neovascularization nasal to the fovea.
Fig 6. Case 2. Stereoscopic view of the left macula. Notice the large area of retinal pigment epithelial detachment in the macula. Notice the multiple retinal pigment epithelial folds.
down, and a fibrovascular membrane occupies the space between the RPE and Bruch's membrane. The fibrous tissue is adherent to the underside of the RPE. The fibrovascular sUbpigment epithelial tissue shrinks and contracts and tears the RPE. 3- 10 This causes folding of the flap of the RPE (unpublished data; HS). Such shrinkage, contracture, tearing, and folding of the RPE can occur spontaneously or in association with laser photocoagulation treatment. 6,8,9,tt Retinal pigment epithelial folds also can occur without an associated RPE tear. This occurred in all six of the described eyes. A shallow detachment of the RPE may undergo spontaneous flattening. t2 , t3 A fibrovascular membrane was present under the RPE, and we hypothesize that this caused the RPE to fold or to flatten and fold but not to tear. The previously detached RPE is stretched, and when it flattens, it folds (Fig 7). This appears to have been the sequence of events in these six eyes in which retinal pigment epithelial folds were associated with a shallow detachment of the RPE with no tear. Four eyes did poorly; disciform scarring of the macula developed. In three, this occurred spontaneously, and in one, the scarring occurred after laser treatment of the subretinal neovascularization but not of the entire membrane. One eye could not be followed because the patient was too weak. Retinal pigment epithelial folds are a sign of a subpigment epithelial fibrovascular membrane, also known as
subretinal or choroidal neovascularization. Vessels within the fibrovascular membrane are probably located under most of the area of folded and detached RPE. Thus, if laser photocoagulation treatment is to be successful, and if the retinal pigment epithelial folded area and the subretinal neovascularization that is apparent are not subfoveal, then the entire folded area along with the vessels should probably be treated, if possible. In one patient, the entire juxtafoveal retinal pigment epithelial folded area was treated; a very small folded area was subfoveal and was not treated, This eye (case 6) did reasonably well (preoperative visual acuity, 20/50; postoperative visual acuity, 20/50 [3,5 years later]) (Fig 4). In one eye (case 5), pigment epithelial folds were seen adjacent to an obvious patch of subretinal neovascularization (Fig 3). Only the subretinal neovascularization was treated, and the pigment epithelial folds were left untreated. Within 4 months, the entire pigment epithelial folded area showed massive subretinal neovascularization and disciform scarring. It appears that if laser treatment is directed only to the subretinal neovascularization and not to the entire area or a major portion of the area of retinal pigment epithelial folds, the result will probably be poor because it is likely that most of the folded area, ifnot all of it, contains subretinal neovascularization, It should be noted that it may not be valid to make any recommendations regarding the appropriate use of laser photocoagulation based on the experience of only the two cases that were treated, 663
OPHTHALMOLOGY
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MAY 1990
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VOLUME 97
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--
~O OOOOOf'oo oq,--
;=> P ---
B
"",
C) C)AC) c:=) C)~
.._"'-
...-..
~
O>"OO~OOOO-.J.<-OOOO
C) C) C)'r"C)~C) QC)1q C ---'
Retinal pigment epithelial folds probably represent a contracted subpigment epithelial fibrovascular membrane in evolution toward a disciform scar. Most of the time, the retinal pigment epithelial folds are located within the fovea. Retinal pig'11ent epithelial folds appear to be distinct from choroidal folds. The term choroidal folds pertains to a folding of the choroid and includes a folding of the overlying Bruch's membrane and RPE. 14- 16 Choroidal folds are caused by any ~hortening of the arc of the eye, the choroid, Bruch's membrane, or RPE such as that caused by compression of the globe by orbital or ocular tumors, optic nerve head swelling, hypotony, or hyperopia. 15 , 16 Choroidal folds also have been seen in many conditions. 14,15,17, 18 In macular degeneration, choroidal folds are seen associated with disciform scarring and also with the scarring from laser photocoagulation treatment. 19 Choroidal folds have a characteristic clinical and angiographic appearance of alternating pigmented and depigmented, hyperfluorescent and hypofluorescent lines, usually in the posterior pole. In this manner, they do appear similar to retinal pigment epithelial folds. The retinal pigment epithelial folds, in our cases, could be disting'lished from choroidal folds not only by their general difference in appearance, but especially by stereoscopic examination or stereoscopic fluorescein angiography in
664
Fig 7. There is a fibrovascular membrane (subretinal neovascularization) under the RPE. B, the fibrovascular membrane has become extensive, elevating the RPE. C, the fibrovascular membrane has contracted, folding the RPE .
which the RPE could be seen readily not only mounded up, but also thrown into folds. In each of our cases, the RPE was lifted up, detached, and folded. Moreover, in the area where the RPE was not detached, it was not folded. Flat RPE was not folded in our cases-in other words, all of our cases showed folds only in detached RPE. With choroidal folds, RPE is flat on Bruch's membrane, and both are folded as a result of the folding of the choroid. In our cases of retinal pigment epithelial folds, the choroid was not folded but separated from the overlying, detached, and folded RPE.
REFERENCES 1. Green WR, McDonnell PJ, Yeo JH . Pathologic features o fsenile macular degeneration . Ophthalmology 1985; 92:615-27. 2. Kenyon KR , Maumenee AE, Ryan SJ, et al. Diffuse drusen and associated complications. Am J Ophthalmol 1985; 100: 119-28. 3. Hoskin A, Bird AC , Sehmi K. Tears of detached retinal pigment epithelium. Br J Ophthalmol 1981; 65:417- 22. 4. Green SN, Yarian D. Acute tear of the retinal pigment epithelium. Retina 1983; 3:16-20. 5. Krishan NR, Chandra SR, Stevens TS. DiagnosiS and pathogenesis of retinal pigment epithelial tears. Am J Ophthalmol1985; 100:698707.
SCHATZ et al
6. Cantrill HL, Ramsay RC, Knobloch WHo Rips in the pigment epithelium. Arch Ophthalmol1983; 101:1074-9. 7. Gass JDM. Pathogenesis of tears of the retinal pigment epithelium. Br J Ophthalmol1984; 68:513-9. 8. Sunakawa M, Tsukahara I. Tear of the retinal pigment epithelium and serous retinal detachment [Letter]. Am J Ophthalmol 1985; 100: 488-9. 9. Decker WL, Sanborn GE, Ridley M, et al. Retinal pigment epithelial tears. Ophthalmology 1983; 90:507-12. 10. Schatz H. Essential Fluorescein Angiography: A Compendium of 100 Classic Cases. San Anselmo: Pacific Medical Press, 1983. 11. Gass JDM. Retinal pigment epithelial rip during krypton red laser photocoagulation. Am J Ophthalmol1984; 98:700-6. 12. Braunstein RA, Gass JDM. Serous detachments of the retinal pigment epithelium in patients with senile macular disease. Am J Ophthalmol 1979; 88:652-60. 13. Meredith TA, Braley RE, Aaberg TM. Natural history of serous de-
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tachments of the retinal pigment epithelium. Am J Ophthalmol 1979; 88:643-51. 14. Cangemi FE, Trempe CL, Walsh JB. Choroidal folds. Am J Ophthalmol 1978; 86:380-7. 15. Kalina RE, Mills RP. Acquired hyperopia with choroidal folds. Ophthalmology 1980; 87:44-50. 16. Friberg TR. The etiology of choroidal folds. A biomechanical explanation. Graefes Arch Clin Exp Ophthalmol1989; 227:459-64. 17. Johnson RN, Schatz H, McDonald HR. Photic maculopathy: early angiographic and ophthalmoscopic findings and late development of choroidal folds [Letter]. Arch Ophthalmol1987; 105:1633-4. 18. Bullock JD, Egbert PRo The origin of choroidal folds. A clinical, histopathological, and experimental study. Doc Ophthalmol 1974; 37: 261-93. 19. Gass JDM. Radial chorioretinal folds. A sign of choroidal neovascularization. Arch Ophthalmol 1981; 99:1016-8.
665
Abstract: The authors reviewed six eyes that had a shallow retinal pigment epithelial detachment with the retinal pigment epithelium (RPE) thrown into folds. A contraction of subpigment epithelial fibrovascular tissue causes a folding of the overlying, intimately adherent RPE. Retinal pigment epithelial folds are a sign of subretinal neovascularization and are another manifestation of age-related macular degeneration. Ophthalmology 1990; 97:658-665
We reviewed six eyes in six patients with retinal pigment epithelial folds associated with a retinal pigment epithelium (RPE) detachment, which was also associated with macular degeneration. We herein describe the clinical findings and fluorescein angiographic characteristics of this clinical sign. We also propose a pathogenesis.
METHODS We reviewed the records of six patients with the diagnosis of retinal pigment epithelial folds. Each patient had a full history and ocular examination, including slit-lamp biomicroscopy, stereo fundus photography, and fluorescein angiography. Follow-up examinations, done at variable intervals depending on the clinical course, included slit-lamp biomicroscopy, fundus photography, and, when indicated, fluorescein angiography. Originally received: November 10. 1989. Revision accepted: January 25. 1990. Presented at the American Academy of Ophthalmology Annual Meeting. New Orleans. Ocl/Nov 1989. Supported by the Retina Research Fund of St. Mary's Hospital and Medical Center. San Francisco. Califomia. Reprint requests to Howard Schatz. MD. 1 Daniel Burnham Ct. #210C. San Francisco. CA 94109.
658
RESULTS Six eyes in six patients (5 women, I man) had retinal pigment epithelial folds. The patients ranged in age from 71 to 100 years (average, 81 years). The visual acuity of the involved eye at the time the diagnosis of the retinal pigment epithelial folds was made and at the last follow-up is shown in Table 1. In all six eyes, retinal pigment epithelial folds were present in the overlying shallow detachment of the pigment epithelium. Subretinal neovascularization, associated with the shallow detachment of the retinal pigment epithelium (RPE), was present in all six eyes (Figs 1, 2). Four of the eyes were not treated. Disciform scars developed in three eyes. In one patient (case 3), the follow-up was only 1 month because the patient was 100 years of age and too weak to return for reexamination. In one eye (case 5), the area of obvious subretinal neovascularization was treated with laser photocoagulation, but the remainder of the folded area was not treated; a disciform scar developed in this eye in 4 months (Fig 3). In one eye (case 6), the pigment epithelial folded area was located mostly juxtafoveal, with only a very small edge of the folded area in the fovea . All of the folded area outside of the central fovea was treated. This eye did well (Fig 4). In all six patients, the fellow eye showed signs of agerelated macular degeneration, including disciform scar (3 eyes) and drusen (3 eyes).
SCHATZ et at
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Table 1. Visual Acuity of the Involved Eye at Diagnosis and Last Follow-up
Patient No.
Age (yrs)/ Race/Sex
Visual Acuity in Eye with Retinal Pigment Epithelial Folds
1
76/W/F 77/W/M 100/W/F 81/W/F 71 /W/F 76/W /F
20/100 3/200 20/100 20/70 20/40 20/50
2 3 4 5 6
W = white; SRNV
Laser Treatment
Length of Follow-up
Final Visual Acuity
No No No No To obvious SRNV To most of folded area
6 mos 1 yr 1 mo 1 yr 4 mos 3V2 yrs
10/200 3/200 20/100 3/200 2/200 20/50
Status of Macula Disciform scar Disciform scar No change Disciform scar Disciform scar Quiet laser scar; no recurrence of SRNV
Fellow Eye Disciform scar Drusen Drusen Disciform scar Drusen Disciform scar
= subretinal neovascularization.
Fig 1. Case I. A 76-year-old white woman with visual acuity of 20/ 100 in the left eye. A, red-free photograph of the left macula. Notice the dark and light lines in the central macula. The whole macula was detached. B, arteriovenous phase fluorescein angiogram of the left macula. There is a small patch of subretinal neovascularization in the central macula. This entire area is encircled by a detached and folded RPE. Notice the alternating hyper- and hypofluorescent curvilinear lines. C, late phase fluorescein angiogram. There is a shallow detachment of the RPE throughout the macula and a small detachment of the sensory retina centrally.
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Fig 2. Case 4. A 81-year-old white women with a visual acuity of 20170 in the right eye. A, red-free photograph of the right macula. Notice the curvilinear pigmented lines in the central macula. There is a patch of subretinal neovascularization temporally with subretinal hemorrhage. B, early arteriovenous phase fluorescein angiogram. Notice the hyperfluorescence from the subretinal neovascularization and the hypofluorescence from the hemorrhage in the temporal macula. The entire central macula shows folding of the RPE as evidenced by hyper- and hypofluorescent lines. Stereoscopic angiography showed that this whole area was a mounded-up and detached area of RPE. C, late phase fluorescein angiogram. Notice the hyperfluorescent leakage from the subretinal neovascularization temporally. The entire macula is detached from a shallow detachment of the RPE, the folds are evident centrally.
DISCUSSION Clinically, the retinal pigment epithelial folds appeared as fine lines of pigmentation alternating with pale, light-
orange lines. The folds were sometimes fairly straight but more often were curvilinear or irregular. They usually occupied an area of'approximately 0.5 to I disc diameter and, in all cases, were located in the macula. Approximately four to ten pigment epithelial folds were seen in )
Fig 3. Case 5. A 71-year-old white woman with visual acuity of 20/40 in the left eye. A, red-free photograph of the left macula. There is a shallow detachment of the retinal pigment epithelium (RPE) in the macula. Notice the fine RPE folds. There is a shallow sensory detachment inferonasally. B, arteriovenous phase fluoresce in angiogram shows a patch of subretinal neovascularization just inferonasal to the fovea. The remaining central macular area shows pigment epithelial folds in a detached RPE. C, later phase fluorescein angiogram shows leakage from the subretinal neovascularization inferonasally and hyperfluorescent staining of the retinal pigment epithelial folds and detachment. D, late phase fluorescein angiogram shows pooling of fluorescein (hyperfluorescence) under the detached and folded RPE. The patch of subretinal neovascularization inferonasally shows leakage and a hypofluorescent rim nasally that represents a fine line of subretinal hemorrhage. E, the patch of subretinal neovascularization, just inferonasal to the left fovea, was then treated with laser photocoagulation. The detached and folded RPE was not treated. Three weeks postoperatively, laser photocoagulation of the subretinal neovascularization 'was done. Arteriovenous phase fluorescein angiogram shows hypofluorescence in the area of the laser treatment. The retinal pigment epithelial folds continue to show alternating lines of hyper- and hypofluorescence. F, the patient was then followed and noted acute reduction in vision. The angiophotograph was taken 4 months after laser photocoagulation. Late phase fluorescein angiogram shows hyperfluorescent staining of the disciform scar.
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Fig 4. Case 6. A76-year-old white woman with visual acuity of 20/50 in the right eye. A, red-free photograph of the right macula. There is a shallow detachment of retinal pigment epithelium (RPE) involving the central and upper portions of the macula. B,arteriovenous phase fluorescein angiogram shows folded and detached RPE. Notice the alternating hyper- and hypofluorescent curvilinear lines that look like a fingerprint. C, late phase fluorescein angiogram. There is leakage of fluorescein under the detached and folded RPE. Notice the multiple hyperfluorescent spots surrounding this area. D, 4 months after laser photocoagulation. Visual acuity was 20/ 50. Notice the flat, quiet laser lesion in the upper half of the macula.
each case, although one case had more than ten fine folds (Fig I). On slit-lamp biomicroscopy or stereoscopic fundus photography, retinal pigment epithelial folds look like mounded-up, thick, wrinkled cloth. The folds are more readily apparent on fluorescein angiography than clinically and are best seen with stereoscopic fluorescein angiography. They appear as alternating white (hyperfluorescent) and dark (hypofluorescent) lines. They sometimes look like a fingerprint (Fig 4). Stereoscopically, they produce a remarkable picture of a 662
mounded-up, folded, or wrinkled clump of tissue (Figs 5, 6). In all six eyes, subretinal neovascularization was present, and the associated clinical and angiographic findings of subretinal neovascularization were seen. The RPE was shallowly detached and folded . Drusen also were often present. In some cases of macular degeneration, new vessels proliferate from the choriocapillaris through a break in Bruch's membrane and dissect between the RPE and Bruch's membrane. 1,2 With time, fibrous tissue is laid
SCHATZ et al
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Fig 5. Case 5. Stereoscopic view of the left macula. Notice the elevated, heaped-up, folded, and detached retinal pigment epithelium in the macula. There is a patch of subretinal neovascularization nasal to the fovea.
Fig 6. Case 2. Stereoscopic view of the left macula. Notice the large area of retinal pigment epithelial detachment in the macula. Notice the multiple retinal pigment epithelial folds.
down, and a fibrovascular membrane occupies the space between the RPE and Bruch's membrane. The fibrous tissue is adherent to the underside of the RPE. The fibrovascular sUbpigment epithelial tissue shrinks and contracts and tears the RPE. 3- 10 This causes folding of the flap of the RPE (unpublished data; HS). Such shrinkage, contracture, tearing, and folding of the RPE can occur spontaneously or in association with laser photocoagulation treatment. 6,8,9,tt Retinal pigment epithelial folds also can occur without an associated RPE tear. This occurred in all six of the described eyes. A shallow detachment of the RPE may undergo spontaneous flattening. t2 , t3 A fibrovascular membrane was present under the RPE, and we hypothesize that this caused the RPE to fold or to flatten and fold but not to tear. The previously detached RPE is stretched, and when it flattens, it folds (Fig 7). This appears to have been the sequence of events in these six eyes in which retinal pigment epithelial folds were associated with a shallow detachment of the RPE with no tear. Four eyes did poorly; disciform scarring of the macula developed. In three, this occurred spontaneously, and in one, the scarring occurred after laser treatment of the subretinal neovascularization but not of the entire membrane. One eye could not be followed because the patient was too weak. Retinal pigment epithelial folds are a sign of a subpigment epithelial fibrovascular membrane, also known as
subretinal or choroidal neovascularization. Vessels within the fibrovascular membrane are probably located under most of the area of folded and detached RPE. Thus, if laser photocoagulation treatment is to be successful, and if the retinal pigment epithelial folded area and the subretinal neovascularization that is apparent are not subfoveal, then the entire folded area along with the vessels should probably be treated, if possible. In one patient, the entire juxtafoveal retinal pigment epithelial folded area was treated; a very small folded area was subfoveal and was not treated, This eye (case 6) did reasonably well (preoperative visual acuity, 20/50; postoperative visual acuity, 20/50 [3,5 years later]) (Fig 4). In one eye (case 5), pigment epithelial folds were seen adjacent to an obvious patch of subretinal neovascularization (Fig 3). Only the subretinal neovascularization was treated, and the pigment epithelial folds were left untreated. Within 4 months, the entire pigment epithelial folded area showed massive subretinal neovascularization and disciform scarring. It appears that if laser treatment is directed only to the subretinal neovascularization and not to the entire area or a major portion of the area of retinal pigment epithelial folds, the result will probably be poor because it is likely that most of the folded area, ifnot all of it, contains subretinal neovascularization, It should be noted that it may not be valid to make any recommendations regarding the appropriate use of laser photocoagulation based on the experience of only the two cases that were treated, 663
OPHTHALMOLOGY
•
MAY 1990
•
VOLUME 97
•
NUMBER 5
--
~O OOOOOf'oo oq,--
;=> P ---
B
"",
C) C)AC) c:=) C)~
.._"'-
...-..
~
O>"OO~OOOO-.J.<-OOOO
C) C) C)'r"C)~C) QC)1q C ---'
Retinal pigment epithelial folds probably represent a contracted subpigment epithelial fibrovascular membrane in evolution toward a disciform scar. Most of the time, the retinal pigment epithelial folds are located within the fovea. Retinal pig'11ent epithelial folds appear to be distinct from choroidal folds. The term choroidal folds pertains to a folding of the choroid and includes a folding of the overlying Bruch's membrane and RPE. 14- 16 Choroidal folds are caused by any ~hortening of the arc of the eye, the choroid, Bruch's membrane, or RPE such as that caused by compression of the globe by orbital or ocular tumors, optic nerve head swelling, hypotony, or hyperopia. 15 , 16 Choroidal folds also have been seen in many conditions. 14,15,17, 18 In macular degeneration, choroidal folds are seen associated with disciform scarring and also with the scarring from laser photocoagulation treatment. 19 Choroidal folds have a characteristic clinical and angiographic appearance of alternating pigmented and depigmented, hyperfluorescent and hypofluorescent lines, usually in the posterior pole. In this manner, they do appear similar to retinal pigment epithelial folds. The retinal pigment epithelial folds, in our cases, could be disting'lished from choroidal folds not only by their general difference in appearance, but especially by stereoscopic examination or stereoscopic fluorescein angiography in
664
Fig 7. There is a fibrovascular membrane (subretinal neovascularization) under the RPE. B, the fibrovascular membrane has become extensive, elevating the RPE. C, the fibrovascular membrane has contracted, folding the RPE .
which the RPE could be seen readily not only mounded up, but also thrown into folds. In each of our cases, the RPE was lifted up, detached, and folded. Moreover, in the area where the RPE was not detached, it was not folded. Flat RPE was not folded in our cases-in other words, all of our cases showed folds only in detached RPE. With choroidal folds, RPE is flat on Bruch's membrane, and both are folded as a result of the folding of the choroid. In our cases of retinal pigment epithelial folds, the choroid was not folded but separated from the overlying, detached, and folded RPE.
REFERENCES 1. Green WR, McDonnell PJ, Yeo JH . Pathologic features o fsenile macular degeneration . Ophthalmology 1985; 92:615-27. 2. Kenyon KR , Maumenee AE, Ryan SJ, et al. Diffuse drusen and associated complications. Am J Ophthalmol 1985; 100: 119-28. 3. Hoskin A, Bird AC , Sehmi K. Tears of detached retinal pigment epithelium. Br J Ophthalmol 1981; 65:417- 22. 4. Green SN, Yarian D. Acute tear of the retinal pigment epithelium. Retina 1983; 3:16-20. 5. Krishan NR, Chandra SR, Stevens TS. DiagnosiS and pathogenesis of retinal pigment epithelial tears. Am J Ophthalmol1985; 100:698707.
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