The Natural Course of Central Retinal Vein Occlusion Patricia M. Quinlan, M.D., Michael J. Elman, M.D., Amita Kaur Bhatt, M.D., Patrick Mardesich, M.D., and Cheryl Enger, M.S. We reviewed the records of 160 patients who had central retinal vein occlusion between 1980 and 1985. Of 168 eyes, 107 (64%) were classified as nonischemic types and 61 (360/0) were classified as ischemic types. Of 107 nonischemic eyes, ten (9%) converted to the ischemic variant. Of 107 nonischemic eyes, 33 (31%) lost three or more lines of visual acuity irrespective of initial visual acuity. A final visual acuity less than or equal to 20/200 was recorded in 57 of 61 (93%) of ischemic eyes and 53 of 107 (50%) of nonischemic eyes. CENTRAL RETINAL VEIN OCCLUSION may result in permanent, severe, visual impairment. We reviewed the records of all patients examined at the Retinal Vascular Center of the Wilmer Institute between January 1980 and December 1985 who had a diagnosis of central retinal vein occlusion to learn more of the history of this disease and to identify various risk factors and prognostic indicators.
Patients and Methods We selected patients on the basis of an unambiguous diagnosis of central retinal vein occlusion documented by fundus photography and fluorescein angiography. The characteristic criteria were as follows: scattered intraretinal hemorrhages, venous dilatation, and tortuosity Accepted for publication May 30, 1990. From the Retinal Vascular Center (Drs. Quinlan, Elman, Bhatt, and Mardesich), and Wilmer Biostatistical Center (Ms. Enger), Wilmer Ophthalmological Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland; and the Department of Ophthalmology, University of Maryland School of Medicihe, Baltimore, Maryland (Dr. Elman). This study was supported in part by core grant EY01765 from the National Eye Institute. Reprint requests to Michael J. Elman, M.D., Department of Ophthalmology, University of Maryland Hospital, 22 S. Greene St., Rm. N6W54, Baltimore, MD 21201.
118
in all quadrants centered on the disk; optic disk swelling; and delayed arteriovenous filling (> 15 seconds). Two independent, masked observers (P.M.Q. and M.J.E.) classified the fluorescein angiograms and color photographs of all eyes with central retinal vein occlusion as either ischemic or nonischemic. Disputed cases were jointly adjudicated. The features of ischemic central retinal vein occlusion were five or more contiguous disk areas of capillary nonperfusion, leakage or staining of any venule wall, or evidence of retinal or anterior segment neovascularization. Thus, by definition, all eyes with neovascularization at baseline were assigned to the ischemic group. We classified eyes with central retinal vein occlusion without any of these features at baseline examination as nonischemic. This system was weighted to place questionably ischemic eyes in the ischemic group. Conversion from the nonischemic type to the ischemic type was defined by either development of retinal or anterior segment neovascularization or development of fluorescein angiographic changes of ischemia. We reviewed the patients' medical histories, present illnesses, and medications currently used. If patients stated they had high blood pressure or were using an antihypertensive medication, they were considered to have systemic hypertension. We excluded patients who received photocoagulation before their first visit to the Retinal Vascular Center because we could not always verify the indications for such treatment. We included patients we treated with panretinal photocoagulation for retinal or anterior segment neovascularization, however, since this treatment represents the accepted standard of care for these patients. Where recent ophthalmic follow-up was inadequate, we contacted the referring ophthalmologist to provide current clinical information regarding visual acuity and the presence of neovascularization. Patients with less than six months of follow-up were excluded. Group comparisons were performed by using the chi-square test for independence. Kaplan-
©AMmuCAN JOURNAL OF OPHTHALMOLOGY
110:118-123,
AUGUST,
1990
119
Central Retinal Vein Occlusion
Vol. lID, No.2
Meier experimental survival analysis! was used to estimate cumulative risks over time. Change in visual acuity was measured according to visual acuity on a modified Bailey-Lovie chart by using the logmar technique."
Results A total of 226 eyes with central retinal vein occlusion were identified. Of these, 58 were excluded because of previous laser treatment (14 eyes), less than six months of follow-up (19 eyes), or ungradable baseline photographs (25 eyes). We studied a total of 168 eyes from 160 patients. Of these, 61 eyes (36%) were ischemic and 107 (64%) were nonischemic at the initial visit. Of the 160 patients, the mean age was 63 years (range, 14 to 93 years). Eighty-nine were male (56%) and 71 were female (44%). Of 168 eyes, 82 were right eyes (49%) and 86 were left eyes (51 %). The eyes were affected bilaterally in 16 patients. Follow-up ranged from six months to six years (mean, 22 months). Age, sex, race, and bilateral occurrence were not related to the severity of venous occlusion. Ten eyes with central retinal vein occlusion (9%) were converted from the nonischemic to the ischemic type between one and 36 months after the initial visit (median, eight months). No significant ophthalmic or systemic risk factor was identified in the converted group when compared to eyes remaining nonischemic. Of the 160 patients, 24 were under 50 years of age (16 males and eight females). Of these patients, the right eye was involved in eight cases and the left in 16 cases. Ten of these eyes were ischemic, two converted to the ischemic type, and 12 remained nonischemic. We compared the prevalence of systemic diseases in young and old patients (Table 1). Of young patients 13 (54%) compared to 40 (29%) of older patients had no identified systemic disorder (P = .02). Three (13%) of the young patients had a collagen vascular disorder compared to three (2%) in the older group (P = .01). None of the young patients had cardiovascular disease (P = .003) or cerebrovascular disease (P = .12). Ten (42%) of the young patients had hypertension (P = .15) compared to 78 patients (57%) in the older age group. At the time they were first examined at Wilmer, 25 patients had retinal vascular occlusive disease in the fellow eye. Of these patients, 16 had central retinal vein occlusion, eight had
branch retinal vein occlusion, and one had branch retinal artery occlusion. During the course of this study only one additional patient developed a branch retinal vein occlusion in the fellow eye. No other vascular occlusions in the fellow eye were noted during follow up. We failed to find any significant systemic risk factors associated with bilateral retinal vascular occlusive disease. Similarly, when comparing 134 patients who had central retinal vein occlusion in one eye only to 11 patients who had a history of central retinal vein occlusion in one eye and were examined by us to have central retinal vein occlusion in the fellow eye, the severity of disease in the examined eyes, complication rate, and visual outcome were similarly distributed in both groups. The numbers, however, were small in this subgroup. Baseline visual acuity in the nonischemic group varied from 20/15 to counting fingers (Fig. 1). In the nonischemic group, a final visual acuity of 20/200 or less was noted in six of the 28 eyes (21 %) with initial visual acuity better than or equal to 20/40, and in 30 of the 34 eyes (88%) with initial visual acuity less than or equal to 20/200. Only 16 of 107 nonischemic eyes (15%) gained three or more lines of visual acuity from baseline, whereas 33 eyes (31 %) lost three or more lines of visual acuity (Table 2). Overall, the poorer the initial visual acuity, the poorer the visual prognosis (chi-square for trend, P < .001). Of the 107 eyes in the nonischernie group, 53 (50%) had a final visual acuity less than or equal to 20/200; only six (6%) resolved with visual acuity, ophthalmoscopic, and angiographic features returning to normal TABLE 1 A COMPARISON OF SYSTEMIC DISEASE AND AGE IN 160 PATIENTS PATIENTS < 50
PATIENTS « 50
YEARS OF AGE
YEARS OF AGE
(NO.
= 24)
(NO.
= 136)
SYSTEMIC DISEASE
NO.(%)
NO.(%)
PVAWE
Diabetes Hypertension
2 (8) 10 (42)
25 (18) 78 (57)
.230 .150
0(0)
44 (32)
.003
o (OJ
18 (13)
.120
3 (13)
3 (2)
.010
13 (54)
40 (29)
.020
Cardiovascular disease Cerebrovascular disease Collagen vascular disorders No systemic disease
120
• CONVERTERS
o NON-CONVERTERS
20/25 ~
~
o
20/50
S ~ 20/100
o
0
..J
~ 20/200
0
III
~
20/400
Z ~
51200
Patients and Methods We selected patients on the basis of an unambiguous diagnosis of central retinal vein occlusion documented by fundus photography and fluorescein angiography. The characteristic criteria were as follows: scattered intraretinal hemorrhages, venous dilatation, and tortuosity Accepted for publication May 30, 1990. From the Retinal Vascular Center (Drs. Quinlan, Elman, Bhatt, and Mardesich), and Wilmer Biostatistical Center (Ms. Enger), Wilmer Ophthalmological Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland; and the Department of Ophthalmology, University of Maryland School of Medicihe, Baltimore, Maryland (Dr. Elman). This study was supported in part by core grant EY01765 from the National Eye Institute. Reprint requests to Michael J. Elman, M.D., Department of Ophthalmology, University of Maryland Hospital, 22 S. Greene St., Rm. N6W54, Baltimore, MD 21201.
118
in all quadrants centered on the disk; optic disk swelling; and delayed arteriovenous filling (> 15 seconds). Two independent, masked observers (P.M.Q. and M.J.E.) classified the fluorescein angiograms and color photographs of all eyes with central retinal vein occlusion as either ischemic or nonischemic. Disputed cases were jointly adjudicated. The features of ischemic central retinal vein occlusion were five or more contiguous disk areas of capillary nonperfusion, leakage or staining of any venule wall, or evidence of retinal or anterior segment neovascularization. Thus, by definition, all eyes with neovascularization at baseline were assigned to the ischemic group. We classified eyes with central retinal vein occlusion without any of these features at baseline examination as nonischemic. This system was weighted to place questionably ischemic eyes in the ischemic group. Conversion from the nonischemic type to the ischemic type was defined by either development of retinal or anterior segment neovascularization or development of fluorescein angiographic changes of ischemia. We reviewed the patients' medical histories, present illnesses, and medications currently used. If patients stated they had high blood pressure or were using an antihypertensive medication, they were considered to have systemic hypertension. We excluded patients who received photocoagulation before their first visit to the Retinal Vascular Center because we could not always verify the indications for such treatment. We included patients we treated with panretinal photocoagulation for retinal or anterior segment neovascularization, however, since this treatment represents the accepted standard of care for these patients. Where recent ophthalmic follow-up was inadequate, we contacted the referring ophthalmologist to provide current clinical information regarding visual acuity and the presence of neovascularization. Patients with less than six months of follow-up were excluded. Group comparisons were performed by using the chi-square test for independence. Kaplan-
©AMmuCAN JOURNAL OF OPHTHALMOLOGY
110:118-123,
AUGUST,
1990
119
Central Retinal Vein Occlusion
Vol. lID, No.2
Meier experimental survival analysis! was used to estimate cumulative risks over time. Change in visual acuity was measured according to visual acuity on a modified Bailey-Lovie chart by using the logmar technique."
Results A total of 226 eyes with central retinal vein occlusion were identified. Of these, 58 were excluded because of previous laser treatment (14 eyes), less than six months of follow-up (19 eyes), or ungradable baseline photographs (25 eyes). We studied a total of 168 eyes from 160 patients. Of these, 61 eyes (36%) were ischemic and 107 (64%) were nonischemic at the initial visit. Of the 160 patients, the mean age was 63 years (range, 14 to 93 years). Eighty-nine were male (56%) and 71 were female (44%). Of 168 eyes, 82 were right eyes (49%) and 86 were left eyes (51 %). The eyes were affected bilaterally in 16 patients. Follow-up ranged from six months to six years (mean, 22 months). Age, sex, race, and bilateral occurrence were not related to the severity of venous occlusion. Ten eyes with central retinal vein occlusion (9%) were converted from the nonischemic to the ischemic type between one and 36 months after the initial visit (median, eight months). No significant ophthalmic or systemic risk factor was identified in the converted group when compared to eyes remaining nonischemic. Of the 160 patients, 24 were under 50 years of age (16 males and eight females). Of these patients, the right eye was involved in eight cases and the left in 16 cases. Ten of these eyes were ischemic, two converted to the ischemic type, and 12 remained nonischemic. We compared the prevalence of systemic diseases in young and old patients (Table 1). Of young patients 13 (54%) compared to 40 (29%) of older patients had no identified systemic disorder (P = .02). Three (13%) of the young patients had a collagen vascular disorder compared to three (2%) in the older group (P = .01). None of the young patients had cardiovascular disease (P = .003) or cerebrovascular disease (P = .12). Ten (42%) of the young patients had hypertension (P = .15) compared to 78 patients (57%) in the older age group. At the time they were first examined at Wilmer, 25 patients had retinal vascular occlusive disease in the fellow eye. Of these patients, 16 had central retinal vein occlusion, eight had
branch retinal vein occlusion, and one had branch retinal artery occlusion. During the course of this study only one additional patient developed a branch retinal vein occlusion in the fellow eye. No other vascular occlusions in the fellow eye were noted during follow up. We failed to find any significant systemic risk factors associated with bilateral retinal vascular occlusive disease. Similarly, when comparing 134 patients who had central retinal vein occlusion in one eye only to 11 patients who had a history of central retinal vein occlusion in one eye and were examined by us to have central retinal vein occlusion in the fellow eye, the severity of disease in the examined eyes, complication rate, and visual outcome were similarly distributed in both groups. The numbers, however, were small in this subgroup. Baseline visual acuity in the nonischemic group varied from 20/15 to counting fingers (Fig. 1). In the nonischemic group, a final visual acuity of 20/200 or less was noted in six of the 28 eyes (21 %) with initial visual acuity better than or equal to 20/40, and in 30 of the 34 eyes (88%) with initial visual acuity less than or equal to 20/200. Only 16 of 107 nonischemic eyes (15%) gained three or more lines of visual acuity from baseline, whereas 33 eyes (31 %) lost three or more lines of visual acuity (Table 2). Overall, the poorer the initial visual acuity, the poorer the visual prognosis (chi-square for trend, P < .001). Of the 107 eyes in the nonischernie group, 53 (50%) had a final visual acuity less than or equal to 20/200; only six (6%) resolved with visual acuity, ophthalmoscopic, and angiographic features returning to normal TABLE 1 A COMPARISON OF SYSTEMIC DISEASE AND AGE IN 160 PATIENTS PATIENTS < 50
PATIENTS « 50
YEARS OF AGE
YEARS OF AGE
(NO.
= 24)
(NO.
= 136)
SYSTEMIC DISEASE
NO.(%)
NO.(%)
PVAWE
Diabetes Hypertension
2 (8) 10 (42)
25 (18) 78 (57)
.230 .150
0(0)
44 (32)
.003
o (OJ
18 (13)
.120
3 (13)
3 (2)
.010
13 (54)
40 (29)
.020
Cardiovascular disease Cerebrovascular disease Collagen vascular disorders No systemic disease
120
• CONVERTERS
o NON-CONVERTERS
20/25 ~
~
o
20/50
S ~ 20/100
o
0
..J
~ 20/200
0
III
~
20/400
Z ~
51200
