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Clinical Course of Younger Patients With Central Retinal Vein Occlusion
Franco M. Recchia, MD;
Cynthia A. Carvalho-Recchia, MD;
Tarek S. Hassan, MD
Arch Ophthalmol. 2004;122:317-321.
ABSTRACT
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Objective To describe the clinical course of patients 55 years and younger with central retinal vein occlusion (CRVO).
Design and Methods Retrospective, noncomparative case series. Medical records of 67 patients were reviewed for demographic, photographic, clinical, and visual acuity (VA) data. Data from 57 patients with at least 6 months of follow-up (mean, 29.2 months) were analyzed statistically.
Main Outcome Measures Best-corrected visual acuity and incidence of intraocular neovascularization.
Results Of 67 consecutive patients (55% men; mean age, 45 years), the median presenting VA was 20/50. Forty-five patients (67%) were found to have at least 1 systemic disease. In 57 patients with at least 6 months of follow-up, the final VA was 20/40 or better in 42%, 20/50 to 20/100 in 18%, and 20/200 or worse in 40%. Visual decline was most common within 3 to 6 months of CRVO onset. Visual improvement was uncommon after 12 months. Of the 22 patients with a presenting VA of 20/40 or better, 36% declined to 20/400 or worse at the most recent examination. Of the 10 patients with a presenting VA of 20/200 to 20/400, 8 improved to 20/60 or better. None of the 6 patients with a presenting VA of counting fingers or worse improved. Intraocular neovascularization was diagnosed at 1 to 9 months following CRVO in 10 patients (18%). Neovascularization of the anterior segment developed in 6 patients (11%), including neovascular glaucoma in 3 (5%). The occurrence of neovascularization appeared to be unrelated to sex, age, presence of associated disease, duration of symptoms, or presenting VA.
Conclusions Younger patients with CRVO have a variable clinical course. Presenting VA does not appear to be predictive of visual or anatomic outcome. As a significant number of patients with good vision at presentation develop legal blindness, therapeutic intervention during periods of visual decline may be considered in these patients.
INTRODUCTION
Central retinal vein occlusion (CRVO) is a significant cause of visual impairment and may afflict persons of any age.1-2 As new therapeutic options for patients with CRVO emerge, an understanding of the condition's clinical course is essential for appropriate timing of any potential intervention. Large prospective studies (such as the Central Vein Occlusion Study [CVOS] and the Eye Disease Case-Control Study)1-3 and retrospective case series4 have delineated the natural history of the condition and enumerated systemic risk factors. Overall, poor visual outcome (visual acuity [VA] worse than 20/200) and poor anatomic outcome (intraocular neovascularization [NV]) are correlated with poor VA at presentation. Most patients in these studies, however, were older than 55 years, and the conclusions regarding the clinical course of CRVO may not apply to younger patients. Several studies focusing exclusively on patients younger than 50 years have arrived at conflicting conclusions and are limited by small sample size, nonconsecutive case selection, or incomplete follow-up. The present study was undertaken specifically to evaluate the visual and anatomic outcomes of younger patients with CRVO and is, to our knowledge, the largest series published to date of such patients treated at a single institution.
METHODS
Following approval from the institutional review board of William Beaumont Hospital (Royal Oak, Mich), records of all patients seen at Associated Retinal Consultants P.C. from February 1992 through February 2001 with the diagnosis of CRVO were obtained. Only the records of patients aged 18 to 55 years at the time of presentation were studied further. In all eyes, the diagnosis of CRVO was made clinically at the time of patients' presentation based on venous dilation and tortuosity, 4 quadrants of intraretinal hemorrhage, and angiographic evidence of impaired venous return. The diagnosis of CRVO was confirmed for the present study in all cases by review of fluorescein angiograms or color fundus photographs.
The following data were obtained: age; sex; duration of visual impairment; medical history; results of systemic medical evaluation; best-corrected VA (baseline and at 1, 3, 6, and 12 months, and at the most recent follow-up); occurrence of intraocular NV; and results of treatment. Presumed date of onset of CRVO was calculated by the duration of patients' symptoms.
Visual acuity measurements were converted to logMAR values for statistical comparison. Counting fingers (CF) and hand motions (HM) were assigned logMAR values of 2.0 and 3.0, respectively. Means were compared using the unpaired t test. Frequencies were compared using the  2 or Fisher exact test as appropriate. Visual acuity trends were analyzed using the Mann-Whitney U test. A P value of .05 was considered statistically significant.
RESULTS
A total of 67 consecutive patients aged 18 to 55 years were identified in the 10-year study period (Table 1). The mean age was 45 years (range, 20-55 years; median, 48 years). Thirty-seven patients (55%) were men, and 30 (45%) were women. The mean duration of visual impairment was 26 days (range, 1 day to 6 months; median, 12 days).
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Table 1. Baseline Demographic and Clinical Characteristics of All Patients Studied*
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In 36 of the 67 patients (54%), a systemic disease was known at the time of presentation (Figure 1). All 67 patients were asked specifically about a known diagnosis of hypertension, diabetes mellitus, and hypercholesterolemia: 15 patients had hypertension; 8 had diabetes mellitus; and 4 had hypercholesterolemia. In the 31 patients with no known systemic disease at presentation, a systemic medical evaluation was performed in 30, and a systemic abnormality was discovered in 9 (30%). No patient had proliferative diabetic retinopathy.
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Flowsheet depicting systemic abnormalities in 67 consecutive younger patients with central retinal vein occlusion. ACE indicates angiotensin-converting enzyme.
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Fifty-seven patients (85%) had been followed-up for at least 6 months, and 50 patients (75%) had been followed-up for at least 12 months. There was no statistical difference in demographics, presenting VA, or incidence of systemic disease between the patients with at least 6 months of follow-up and those with shorter follow-up. Data and statistical analyses regarding visual and anatomic outcomes presented in this study pertain to the group of 57 patients followed-up for at least 6 months.
VISUAL OUTCOMES
The median presenting VA was 20/50. Twenty-two patients (39%) had a VA of 20/40 or better. Nineteen patients (33%) had a VA of 20/50 to 20/100, and 16 (28%) had a VA of 20/200 to light perception. There was no significant difference in age, sex, or incidence of systemic disease among these 3 groups. Patients with the worst vision tended to be older and female. Among all 57 patients analyzed, the final VA was 20/40 or better in 24 patients (42%), 20/50 to 20/100 in 10 patients (18%), and 20/200 or worse in 23 patients (40%). No patient had a final VA worse than light perception. Overall, VA improved by at least 3 lines in 13 (23%) of 57 patients and declined by at least 3 lines in 16 (28%). In the cohort of 25 patients with follow-up of more than 24 months, vision remained unchanged after the 12-month follow-up visit in 17 (68%). Only 2 (8%) experienced subsequent visual improvement (2 or more lines), and 6 (24%) experienced further decline (typically to levels of CF to light perception). The most common causes of visual decline were macular edema and vitreous hemorrhage.
In the cohort of 22 patients with initially good VA (20/40 or better), 13 (59%) retained VA in that range at the most recent follow-up (mean, 29.2 months), while 8 (36%) had worsened to the level of 20/200 or worse (Table 2). The loss of VA was a minimum of 8 lines. In 5 patients (23%), VA was CF or HM. As presented in Table 3, the most significant decline in VA occurred within 5 months of presumed CRVO onset (or within 3 months of presentation). Only 1 of 10 patients who had lost VA by the 3-month follow-up eventually regained vision. (In that patient, the VA was 20/40 at presentation, 20/80 three months later, 20/40 at the 12-month follow-up, and 20/30 at 33 months.)
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Table 2. Final Visual Outcomes of Patients Stratified by Presenting Visual Acuity (VA)
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Table 3. Serial Visual Acuity (VA) Measurements for Patients Seen With Initially Good Vision (20/40 or Better)*
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In the cohort of 25 patients with intermediate VA at presentation (20/50 to 20/200), 11 (55%) retained VA in the same range at 12 months, while 8 (40%) improved to 20/40 or better and 5% worsened to 20/400 or worse (Table 4). Visual acuity improved by at least 3 lines in 26% and declined by at least 3 lines in 37%. Four patients had a final VA of CF or HM. While the 3-month mark appeared to be significant in this group as well, the cadence of visual change thereafter was more variable. For example, of 6 patients who demonstrated visual improvement at 3 months' follow-up (or 3-4 months after the presumed onset of CRVO), 5 ultimately retained a VA of 20/15 to 20/30. In 12 patients with worse VA (20/100 or worse) at 3 months' follow-up, 5 ultimately regained VA of 20/50 to 20/80, and 7 declined further to levels of 20/200 or worse.
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Table 4. Serial Visual Acuity (VA) Measurements for Patients Seen With Initially Intermediate Vision (20/50 to 20/200)*
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In the cohort of 10 patients with initially poor VA (20/400 to HM), 30% regained at least 3 lines of VA by 12 months (Table 5). Two patients attained a VA of 20/40 or better, and another 2 patients improved to the 20/50 to 20/200 range. In the remaining 6 patients, VA did not improve beyond 20/400. Six patients presented with a VA of CF or HM, and all 6 remained at that level. By contrast, of the 10 patients with a VA of 20/200 or 20/400, 8 (80%) improved to 20/60 or better. Visual acuity improved during the interval of 1 to 9 months after CRVO onset and was unrelated to age, sex, or presence of a systemic medical disorder.
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Table 5. Serial Visual Acuity (VA) Measurements for Patients Seen With Initially Poor Vision (Worse Than 20/200)*
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INCIDENCE OF NV
Overall, 10 patients (18%) developed intraocular NV, diagnosed 1 to 9 months (mean, 5.9 months, median, 6.5 months) following the presumed onset of CRVO (Table 6). Neovascularization of the anterior segment developed in 6 patients (11%), including neovascular glaucoma in 3 (5%). Neovascularization of the posterior segment developed in 5 patients (9%). Nine of the 10 patients with NV were treated with panretinal photocoagulation. Two patients underwent subsequent pars plana vitrectomy for vitreous hemorrhage. One patient, who developed 1 clock-hour of NV of the iris was observed without complication.
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Table 6. Clinical Characteristics and Outcomes of Patients Who Developed Intraocular Neovascularization (NV)
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Of the 10 patients who developed NV, 4 had presented with an initial VA of 20/40 or better; 2, with an initial VA between 20/50 and 20/100; and 4, with an initial VA of CF or worse. One half of the patients were men, and 7 (70%) had an associated systemic disease. The final VA in all but 1 patient was 20/400 or worse.
Table 7 presents a comparison of the demographic and clinical parameters between the cohort of 10 patients who developed NV and the cohort of 47 patients who did not. No significant difference in age, sex, presence of associated disease, duration of visual symptoms, or presenting VA was detectable.
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Table 7. Statistical Comparison of Demographic and Clinical Parameters Between Patients Who Developed Intraocular Neovascularization (NV) and Those Who Did Not
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COMMENT
Central retinal vein occlusion remains a cause of significant visual morbidity. Although it is diagnosed much more frequently in older individuals, CRVO can affect patients of all ages, with devastating effects on vision.1-4 Hayreh5 suggested the term optic disc vasculitis to encompass the clinical spectrum of disc swelling, venous abnormalities, intraretinal hemorrhage, and exudates occurring in younger patients. In type I optic disc vasculitis (similar to the so-called papillophlebits reported by previous authors6-7), marked disc edema was the predominant finding, and the visual prognosis was excellent. In type II, more typical clinical and angiographic features of CRVO were observed, and the clinical course was more variable. Only patients with clinical and angiographic findings consistent with CRVO were included in the present study.
Several authors in the last 2 decades have reported on the visual and anatomic outcomes in younger patients with CRVO. Priluck et al8 described the long-term course of 42 patients drawn from a cohort of 63 patients, 40 years and younger, seen at the Mayo Clinic from 1949 to 1974. However, final VA was unavailable in 8 patients, and for 28 patients, the most recent ocular examination was performed elsewhere. Walters and Spalton9 reviewed the cases of 17 patients aged 40 years or younger, but final visual acuities were available in only 11 (65%). Fong et al10 combined 39 of their nondiabetic CRVO patients younger than 50 years with 64 such cases from other retinal specialists. They did not, however, differentiate visual and anatomic outcomes for patients with different presenting levels of VA.
In the present study, 67 consecutive patients aged 18 to 55 years with CRVO followed-up at a single institution were studied. The presenting VA was 20/40 or better in 42% and 20/200 or worse in 28%. These percentages are consistent with those reported by Walters and Spalton9 and by Fong et al10 in their aggregate of 103 cases. Priluck et al8 reported a higher incidence of poor presenting VA (50%).
The statistical analyses in the present study apply to the 57 patients who were followed-up for at least 6 months. Final VA was reduced to 20/200 or worse in 40%. This overall level of poor final VA is higher than that in previous reports (range, 9%-32%).8-10 The incidence of intraocular NV was 18% in the present series. The rate of neovascular glaucoma (5%) is consistent with previous reports, while the rate of NV of the posterior segment (9%) is higher. Intraocular NV was diagnosed later than 6 months following the presumed onset of CRVO in 50% of the patients in whom it occurred, irrespective of presenting VA. This result underscores the importance of regular and vigilant examination.
The major aim of the present study was to detail the clinical course of patients subdivided by presenting VA. Prognostic information available from presenting VA is valuable for patient counseling and treatment recommendations. In the CVOS, for example, among the cohort of patients with a VA of 20/40 or better, 65% retained VA in that range, and only 10% declined to levels worse than 20/200. Patients in the CVOS with a presenting VA of less than 20/200 were unlikely to experience visual improvement and were more likely to develop intraocular NV.3 Our results in younger patients, however, stand in contrast with those of the CVOS, in that presenting VA does not appear to be predictive of final VA or clinical course. In younger patients with initially good VA (20/40 or better), only 13 (59%) of 22 patients remained at that level while, more strikingly, more than one third worsened to 20/400 or less. Younger patients with initially poor VA (20/200 or worse) may not fare as dismally as expected, as one half improved, and a full one fourth attained a VA of 20/40 or better. Moreover, the incidence of NV was not significantly different between the cohorts of patients with initially good and initially poor VA (18% compared with 25%; P = .70 by the Fisher exact test).
While an increasing number of systemic factors (especially those related to thrombophilia)11-12 have been reported to be associated with CRVO in younger patients, the appropriate extent of systemic evaluation remains unclear. Conclusions regarding the advisability of systemic testing based on the current series should be drawn with caution, since testing of matched controls was not performed. Additionally, since practice patterns evolved with changes in contemporary knowledge and publications over the 10-year study period, testing was not uniform for all patients. It is notable, however, that 4 of 30 patients without known prior disease were found to have common conditions (anemia, hyperglycemia, and hypercholesterolemia) that both have systemic ramifications and are potentially treatable (Figure 1).
Limitations of the present study are mainly those inherent to any retrospective review. There is a possibility of selection bias, in that milder cases of CRVO may not be referred to retina specialists. Long-term data may be biased toward more severe cases, as patients with a benign course may not maintain regular examinations. This potential bias was assessed by comparing the group of 57 patients with at least 6 months of follow-up with the group of 10 patients with less follow-up; no significant differences in baseline parameters were found. Standardized fluorescein angiography, as established in the CVOS, was not performed in every patient. The quality of angiographic images and the range of funduscopic views provided were not consistently sufficient to allow meaningful interpretation, thus precluding a rigorous assessment of perfusion or ischemia. However, VA is correlated with extent of perfusion and offers a general indication of perfusion status.1 The present study may thus provide practical information for cases in which fluorescein angiography may not be readily available.
It is hoped that our results will aid in the counseling of younger patients with CRVO, especially with respect to the appropriateness and timing of therapeutic intervention. For example, a significant number of younger patients with a presenting VA of 20/200 to 20/400 may recover vision spontaneously, albeit slowly. On the other hand, it appears that a patient with initially good vision who loses vision after 3 months may be unlikely to recover spontaneously, and visual improvement in any patient is unlikely after 12 months. Thus, interventions may be justified at an early stage in such cases.
AUTHOR INFORMATION
Corresponding author and reprints: Franco M. Recchia, MD, 8018 Medical Center East, Vanderbilt University Medical Center, Nashville, TN 37232 (e-mail: franco.recchia{at}vanderbilt.edu).
Submitted for publication May 21, 2003; final revision received October 16, 2003; accepted November 6, 2003.
This research was supported in part by the Heed Ophthalmic Foundation Cleveland, Ohio, the AOS-Knapp Foundation,Cleveland, and the Ronald Michels Fellowship Foundation, Baltimore, Md (Dr Recchia).
This research was presented in part at the 106th annual meeting of the American Academy of Ophthalmology; October 23, 2002; Orlando, Fla.
From Associated Retinal Consultants P.C., William Beaumont Hospital, Royal Oak, Mich. Drs Recchia and Carvalho-Recchia are currently affiliated with the Department of Ophthalmology and Visual Sciences, Vanderbilt University Medical Center, Nashville, Tenn. The authors have no relevant financial interest in this article.
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