clinical course of younger patients with central retinal vein occlusion

5
Clinical Course of Younger Patients With Central Retinal Vein Occlusion Franco M. Recchia, MD; Cynthia A. Carvalho-Recchia, MD; Tarek S. Hassan, MD Objective: To describe the clinical course of patients 55 years and younger with central retinal vein occlu- sion (CRVO). Design and Methods: Retrospective, noncompara- tive case series. Medical records of 67 patients were re- viewed for demographic, photographic, clinical, and vi- sual acuity (VA) data. Data from 57 patients with at least 6 months of follow-up (mean, 29.2 months) were ana- lyzed statistically. Main Outcome Measures: Best-corrected visual acu- ity 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 ex- amination. 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 sig- nificant number of patients with good vision at presen- tation develop legal blindness, therapeutic intervention during periods of visual decline may be considered in these patients. Arch Ophthalmol. 2004;122:317-321 C ENTRAL RETINAL VEIN OC- clusion (CRVO) is a sig- nificant cause of visual im- pairment 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 inter- vention. Large prospective studies (such as the Central Vein Occlusion Study [CVOS] and the Eye Disease Case-Con- trol Study) 1-3 and retrospective case se- ries 4 have delineated the natural history of the condition and enumerated systemic risk factors. Overall, poor visual outcome (vi- sual acuity [VA] worse than 20/200) and poor anatomic outcome (intraocular neo- vascularization [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 focus- ing exclusively on patients younger than 50 years have arrived at conflicting con- clusions and are limited by small sample size, nonconsecutive case selection, or in- complete follow-up. The present study was undertaken specifically to evaluate the vi- sual and anatomic outcomes of younger patients with CRVO and is, to our knowl- edge, the largest series published to date of such patients treated at a single insti- tution. METHODS Following approval from the institutional re- view board of William Beaumont Hospital (Royal Oak, Mich), records of all patients seen at As- sociated Retinal Consultants P.C. from Febru- CME course available at www.archophthalmol.com CLINICAL SCIENCES 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. (REPRINTED) ARCH OPHTHALMOL / VOL 122, MAR 2004 WWW.ARCHOPHTHALMOL.COM 317 ©2004 American Medical Association. All rights reserved. Downloaded From: http://archopht.jamanetwork.com/ on 10/14/2013

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Clinical Course of Younger PatientsWith Central Retinal Vein Occlusion

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Page 1: Clinical Course of Younger Patients  With Central Retinal Vein Occlusion

Clinical Course of Younger PatientsWith Central Retinal Vein OcclusionFranco M. Recchia, MD; Cynthia A. Carvalho-Recchia, MD; Tarek S. Hassan, MD

Objective: To describe the clinical course of patients55 years and younger with central retinal vein occlu-sion (CRVO).

Design and Methods: Retrospective, noncompara-tive case series. Medical records of 67 patients were re-viewed for demographic, photographic, clinical, and vi-sual acuity (VA) data. Data from 57 patients with at least6 months of follow-up (mean, 29.2 months) were ana-lyzed statistically.

Main Outcome Measures: Best-corrected visual acu-ity 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 systemicdisease. 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 declinewas 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 ex-amination. Of the 10 patients with a presenting VA of 20/200 to 20/400, 8 improved to 20/60 or better. None of the6 patients with a presenting VA of counting fingers or worseimproved. Intraocular neovascularization was diagnosedat 1 to 9 months following CRVO in 10 patients (18%).Neovascularization of the anterior segment developed in6 patients (11%), including neovascular glaucoma in 3(5%). The occurrence of neovascularization appeared tobe unrelated to sex, age, presence of associated disease,duration of symptoms, or presenting VA.

Conclusions: Younger patients with CRVO have avariable clinical course. Presenting VA does not appearto be predictive of visual or anatomic outcome. As a sig-nificant number of patients with good vision at presen-tation develop legal blindness, therapeutic interventionduring periods of visual decline may be considered inthese patients.

Arch Ophthalmol. 2004;122:317-321

C ENTRAL RETINAL VEIN OC-clusion (CRVO) is a sig-nificant causeofvisual im-pairment and may afflictpersons of any age.1,2 As

new therapeutic options for patients withCRVO emerge, an understanding of thecondition’s clinical course is essential forappropriate timing of any potential inter-vention. Large prospective studies (suchas the Central Vein Occlusion Study

[CVOS] and the Eye Disease Case-Con-trol Study)1-3 and retrospective case se-ries4 have delineated the natural history oftheconditionandenumeratedsystemic riskfactors. Overall, poor visual outcome (vi-sual acuity [VA] worse than 20/200) andpoor anatomic outcome (intraocular neo-vascularization [NV]) are correlated with

poor VA at presentation. Most patients inthese studies, however, were older than 55years, and the conclusions regarding theclinical course of CRVO may not apply toyounger patients. Several studies focus-ing exclusively on patients younger than50 years have arrived at conflicting con-clusions and are limited by small samplesize, nonconsecutive case selection, or in-complete follow-up. The present study wasundertaken specifically to evaluate the vi-sual and anatomic outcomes of youngerpatients with CRVO and is, to our knowl-edge, the largest series published to dateof such patients treated at a single insti-tution.

METHODS

Following approval from the institutional re-view board of William Beaumont Hospital (RoyalOak, Mich), records of all patients seen at As-sociated Retinal Consultants P.C. from Febru-

CME course available atwww.archophthalmol.com

CLINICAL SCIENCES

From Associated RetinalConsultants P.C., WilliamBeaumont Hospital, Royal Oak,Mich. Drs Recchia andCarvalho-Recchia are currentlyaffiliated with the Departmentof Ophthalmology and VisualSciences, Vanderbilt UniversityMedical Center, Nashville,Tenn. The authors have norelevant financial interest inthis article.

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Page 2: Clinical Course of Younger Patients  With Central Retinal Vein Occlusion

ary 1992 through February 2001 with the diagnosis of CRVOwere obtained. Only the records of patients aged 18 to 55 yearsat the time of presentation were studied further. In all eyes, thediagnosis of CRVO was made clinically at the time of patients’presentation based on venous dilation and tortuosity, 4 quad-rants of intraretinal hemorrhage, and angiographic evidence ofimpaired venous return. The diagnosis of CRVO was confirmedfor the present study in all cases by review of fluorescein angio-grams or color fundus photographs.

The following data were obtained: age; sex; duration of vi-sual impairment; medical history; results of systemic medicalevaluation; best-corrected VA (baseline and at 1, 3, 6, and 12months, and at the most recent follow-up); occurrence of intra-ocular NV; and results of treatment. Presumed date of onset ofCRVO was calculated by the duration of patients’ symptoms.

Visual acuity measurements were converted to logMARvalues for statistical comparison. Counting fingers (CF) andhand motions (HM) were assigned logMAR values of 2.0 and3.0, respectively. Means were compared using the unpairedt test. Frequencies were compared using the �2 or Fisher exacttest as appropriate. Visual acuity trends were analyzed usingthe Mann-Whitney U test. A P value of .05 was considered sta-tistically significant.

RESULTS

A total of 67 consecutive patients aged 18 to 55 years wereidentified in the 10-year study period (Table1). The meanage was 45 years (range, 20-55 years; median, 48 years).Thirty-seven patients (55%) were men, and 30 (45%) werewomen. The mean duration of visual impairment was 26days (range, 1 day to 6 months; median, 12 days).

In 36 of the 67 patients (54%), a systemic diseasewas known at the time of presentation (Figure). All 67patients were asked specifically about a known diagno-

sis of hypertension, diabetes mellitus, and hypercholes-terolemia: 15 patients had hypertension; 8 had diabetesmellitus; and 4 had hypercholesterolemia. In the 31 pa-tients with no known systemic disease at presentation, asystemic medical evaluation was performed in 30, and asystemic abnormality was discovered in 9 (30%). No pa-tient had proliferative diabetic retinopathy.

Fifty-seven patients (85%) had been followed-up forat least 6 months, and 50 patients (75%) had been fol-lowed-up for at least 12 months. There was no statisti-cal difference in demographics, presenting VA, or inci-dence of systemic disease between the patients with atleast 6 months of follow-up and those with shorter follow-up. Data and statistical analyses regarding visual and ana-tomic outcomes presented in this study pertain to thegroup of 57 patients followed-up for at least 6 months.

VISUAL OUTCOMES

The median presenting VA was 20/50. Twenty-two pa-tients (39%) had a VA of 20/40 or better. Nineteen pa-tients (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 sig-nificant difference in age, sex, or incidence of systemicdisease among these 3 groups. Patients with the worstvision tended to be older and female. Among all 57 pa-tients analyzed, the final VA was 20/40 or better in 24patients (42%), 20/50 to 20/100 in 10 patients (18%), and20/200 or worse in 23 patients (40%). No patient had afinal VA worse than light perception. Overall, VA im-proved by at least 3 lines in 13 (23%) of 57 patients anddeclined by at least 3 lines in 16 (28%). In the cohort of25 patients with follow-up of more than 24 months, vi-sion remained unchanged after the 12-month follow-upvisit in 17 (68%). Only 2 (8%) experienced subsequentvisual improvement (2 or more lines), and 6 (24%) ex-perienced further decline (typically to levels of CF to lightperception). The most common causes of visual declinewere macular edema and vitreous hemorrhage.

Table 1. Baseline Demographic and ClinicalCharacteristics of All Patients Studied*

AllPatients

�6 moFollow-up

�6 moFollow-up

PValue

No. of patients 67 57 10 NAFollow-up, mo

Mean 29.2 1.1 .77Range 6-107 0-4

Age, yMean 45.1 45.5 43.8 .77Range 20-55 20-55 24-55

SexMale 37 (55) 32 (56) 5 (50) .72Female 30 (45) 25 (44) 5 (50)

Duration of symptoms, dMean 26.1 26.3 25.1 .90Range 1-180 1-180 1-70

Presenting vision�20/40 28 (42) 22 (39) 6 (60) .3020/50 to HM 39 (58) 35 (61) 4 (40)

Associated diseaseKnown at time of

examination36 (54) 33 (58) 3 (30) .28

Discovered aftersystemic evaluation

9 (13) 7 (12) 2 (20)

None known 22 (33) 17 (30) 5 (50)

Abbreviations: HM, hand motions; NA, not applicable.*Unless otherwise indicated, values are presented as number

(percentage).

67 Patients With CRVO

Associated Disease Known at Initial Examination (n = 36)

1 Disease Only (n = 26)Hypertension (11)Hypothyroidism (4)Glaucoma (3)Diabetes Mellitus (2)Cancer (2)Hypercholesterolemia (2)Asthma (1)Ankylosing Spondylitis (1)

>1 Disease (n = 10)Hypertension (10)Diabetes Mellitus (6)Coronary Artery Disease (4)Hypothyroidism (2)Hypercholesterolemia (2)Renal Insufficiency,

Nondiabetic (1)Cancer (1)

No Disease Known at Initial Examination (n = 31)

Systemic Evaluation Not Performed (n = 1)

Systemic Evaluation Performed (n = 30)

Systemic Disorder Found (n = 9)Anemia (2)Hyperglycemia (1)Hypercholesterolemia (1)Hyperfibrinogenemia (1)Hyperalbuminemia (1)Elevated ANA (1)Elevated ACE Level (1)Elevated Antithrombin III (1)

Systemic Disorder Not Found (n = 21)

Flowsheet depicting systemic abnormalities in 67 consecutive youngerpatients with central retinal vein occlusion. ACE indicatesangiotensin-converting enzyme.

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Page 3: Clinical Course of Younger Patients  With Central Retinal Vein Occlusion

In thecohortof22patientswith initiallygoodVA(20/40orbetter),13 (59%)retainedVAinthat rangeat themostrecent follow-up (mean, 29.2 months), while 8 (36%) hadworsened to the level of 20/200 or worse (Table 2). Theloss of VA was a minimum of 8 lines. In 5 patients (23%),VA was CF or HM. As presented in Table3, the most sig-nificantdeclineinVAoccurredwithin5monthsofpresumedCRVO onset (or within 3 months of presentation). Only 1of 10 patients who had lost VA by the 3-month follow-upeventually regained vision. (In that patient, the VA was 20/40 at presentation, 20/80 three months later, 20/40 at the12-month follow-up, and 20/30 at 33 months.)

In the cohort of 25 patients with intermediate VA atpresentation (20/50 to 20/200), 11 (55%) retained VA inthe same range at 12 months, while 8 (40%) improved to20/40 or better and 5% worsened to 20/400 or worse(Table 4). Visual acuity improved by at least 3 lines in26% and declined by at least 3 lines in 37%. Four patientshad a final VA of CF or HM. While the 3-month mark ap-peared to be significant in this group as well, the cadenceof visual change thereafter was more variable. For ex-ample, of 6 patients who demonstrated visual improve-ment at 3 months’ follow-up (or 3-4 months after the pre-sumed onset of CRVO), 5 ultimately retained a VA of 20/15to 20/30. In 12 patients with worse VA (20/100 or worse)at 3 months’ follow-up, 5 ultimately regained VA of 20/50to 20/80, and 7 declined further to levels of 20/200 or worse.

In thecohortof10patientswith initiallypoorVA(20/400toHM),30%regainedat least3linesofVAby12months(Table 5). Two patients attained a VA of 20/40 or better,and another 2 patients improved to the 20/50 to 20/200range. In the remaining 6 patients, VA did not improve be-yond20/400.SixpatientspresentedwithaVAofCForHM,and all 6 remained at that level. By contrast, of the 10 pa-tients with a VA of 20/200 or 20/400, 8 (80%) improved to20/60 or better. Visual acuity improved during the inter-val of 1 to 9 months after CRVO onset and was unrelatedto age, sex, or presence of a systemic medical disorder.

Table 2. Final Visual Outcomes of Patients Stratified by Presenting Visual Acuity (VA)

Presenting VA (Median)No. (%) ofPatients

MeanDuration, d

MeanAge, y M:F

MeanFollow-up, mo

MostRecent VA

No. (%)of Patients

VAImproved*

VADeclined*

All patients (20/50) 57 26.3 45.3 1.3:1 29.2 20/15 to 20/40 24 (42) 13 (23) 16 (28)20/50 to 20/100 10 (18)

20/200 to LP 23 (40)20/15 to 20/40 (20/25) 22 (39) 26 42.8 1.4:1 31.8 20/15 to 20/40 13 (59) 0 9 (41)

20/50 to 20/100 1 (5)20/400 to LP 8 (36)

20/50 to 20/100 (20/60) 19 (33) 18.8 48.8 1.4:1 37.4 20/15 to 20/40 7 (37) 5 (26) 7 (37)20/50 to 20/100 5 (26)

20/200 to HM 7 (37)20/200 to 20/400 (20/200) 10 (18) 45 40.8 2.3:1 15.0 20/15 to 20/40 4 (40) 8 (80) 0

20/50 to 20/100 4 (40)20/200 to HM 2 (20)

CF to HM (CF) 6 (11) 18.2 50.7 0.2:1 18.0 20/15 to 20/40 0 0 020/50 to 20/400 0

CF to HM 6 (100)

Abbreviations: CF, counting fingers; HM, hand motions; LP, light perception.*Three or more Snellen chart lines.

Table 3. Serial Visual Acuity (VA) Measurements forPatients Seen With Initially Good Vision (20/40 or Better)*

VAAt

PresentationAt 3 mo(n = 22)

At 6 mo(n = 22)

At 12 mo(n = 20)

Most Recent(�18 mo)(n = 12)

20/15 to 20/40 22 12 (55) 12 (55) 12 (60) 6 (50)20/50 to

20/2000 8 (36) 6 (27) 4 (20) 0

�20/200 0 2 (9) 4 (18) 4 (20) 6 (50)

*Unless otherwise indicated, values are presented as number (percentage) ofpatients.

Table 4. Serial Visual Acuity (VA) Measurementsfor Patients Seen With Initially Intermediate Vision(20/50 to 20/200)*

VAAt

PresentationAt 3 mo(n = 25)

At 6 mo(n = 25)

At 12 mo(n = 20)

Most Recent(�18 mo)(n = 12)

20/15 to 20/40 0 7 (28) 7 (28) 8 (40) 5 (42)20/50 to

20/20025 17 (68) 16 (64) 11 (55) 5 (42)

�20/200 0 1 (4) 2 (8) 1 (5) 2 (16)

*Unless otherwise indicated, values are presented as number (percentage) ofpatients.

Table 5. Serial Visual Acuity (VA) Measurementsfor Patients Seen With Initially Poor Vision(Worse Than 20/200)*

VAAt

PresentationAt 3 mo(n = 10)

At 6 mo(n = 10)

At 12 mo(n = 10)

Most Recent(�18 mo)

(n = 5)

20/15 to 20/40 0 0 (0) 1 (10) 1 (10) 1 (20)20/50 to

20/2000 2 (20) 1 (10) 2 (20) 1 (20)

�20/200 10 8 (80) 8 (80) 7 (70) 3 (60)

*Unless otherwise indicated, values are presented as number (percentage) ofpatients.

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Page 4: Clinical Course of Younger Patients  With Central Retinal Vein Occlusion

INCIDENCE OF NV

Overall, 10 patients (18%) developed intraocular NV, di-agnosed 1 to 9 months (mean, 5.9 months, median, 6.5months) following the presumed onset of CRVO(Table 6). Neovascularization of the anterior segmentdeveloped in 6 patients (11%), including neovascular glau-coma in 3 (5%). Neovascularization of the posterior seg-ment developed in 5 patients (9%). Nine of the 10 pa-t ients with NV were treated with panret inalphotocoagulation. Two patients underwent subsequentpars plana vitrectomy for vitreous hemorrhage. One pa-tient, who developed 1 clock-hour of NV of the iris wasobserved without complication.

Of the 10 patients who developed NV, 4 had pre-sented with an initial VA of 20/40 or better; 2, with aninitial VA between 20/50 and 20/100; and 4, with an ini-tial VA of CF or worse. One half of the patients were men,and 7 (70%) had an associated systemic disease. The fi-nal VA in all but 1 patient was 20/400 or worse.

Table 7 presents a comparison of the demo-graphic and clinical parameters between the cohort of 10patients who developed NV and the cohort of 47 pa-tients who did not. No significant difference in age, sex,presence of associated disease, duration of visual symp-toms, or presenting VA was detectable.

COMMENT

Central retinal vein occlusion remains a cause of signifi-cant visual morbidity. Although it is diagnosed much morefrequently in older individuals, CRVO can affect patientsof all ages, with devastating effects on vision.1-4 Hayreh5 sug-gested the term optic disc vasculitis to encompass the clini-cal spectrum of disc swelling, venous abnormalities, in-traretinal hemorrhage, and exudates occurring in youngerpatients. 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 thevisual prognosis was excellent. In type II, more typical clini-cal and angiographic features of CRVO were observed, andthe clinical course was more variable. Only patients withclinical and angiographic findings consistent with CRVOwere included in the present study.

Several authors in the last 2 decades have reported onthe visual and anatomic outcomes in younger patients withCRVO. Priluck et al8 described the long-term course of 42patients drawn from a cohort of 63 patients, 40 years andyounger, seen at the Mayo Clinic from 1949 to 1974. How-ever, final VA was unavailable in 8 patients, and for 28 pa-tients, the most recent ocular examination was performedelsewhere. Walters and Spalton9 reviewed the cases of 17patients aged 40 years or younger, but final visual acuitieswere available in only 11 (65%). Fong et al10 combined 39of their nondiabetic CRVO patients younger than 50 yearswith 64 such cases from other retinal specialists. They didnot, however, differentiate visual and anatomic outcomesfor patients with different presenting levels of VA.

Table 7. Statistical Comparison of Demographic and ClinicalParameters Between Patients Who Developed IntraocularNeovascularization (NV) and Those Who Did Not

NVDeveloped

(n = 10)

No NVDeveloped

(n = 47)P

Value

Age, yMean 48.0 44.7 .21*Range 38-55 20-55

Sex, No. (%)Male 4 (40) 27 (57) .51†Female 6 (60) 20 (43)

Hypertension or DM, No. (%)Present 4 (40) 16 (34) .99†Absent 6 (60) 31 (66)

Any associated disease, No. (%)Present 7 (70) 30 (64) .89†Absent 3 (30) 17 (36)

Duration of symptomsMean No. of days 24.4 26.8 .86*Range 4 d to 4 mo 1 d to 6 mo

Presenting VAMedian 20/70 20/50 .68‡Mean 20/215 20/70 .2*Range 20/15 to HM 20/15 to CF

Abbreviations: CF, counting fingers; DM, diabetes mellitus; HM, handmotions; VA, visual acuity.

*Unpaired t test.†Fisher exact test.‡Mann-Whitney U test.

Table 6. Clinical Characteristics and Outcomes of Patients Who Developed Intraocular Neovascularization (NV)

Age, y/SexPresenting

VA Associated DiseaseCRVO

Duration Type of NVOnset

of NV, moInitial

Rx Additional Rx Final VA Follow-up, mo

46/M 20/15 Hypertension 4 d NVA 7 PRP 20/400 1250/M 20/15 Hypertension 7 d NVG, NVD, NVE 7 PRP PPV CF 8149/F 20/30 Hypothyroidism 8 d NVD, NVE 9 PRP PPV 20/30 4451/M 20/40 None 2.5 wk NVE 5 PRP HM 1533/M 20/50 None 4 d NVD, NVE 2 PRP Oral prednisone CF 9952/F 20/100 Hypothyroidism 4 mo NVI 8 PRP CF 4754/F CF None 3 wk NVD 6 PRP HM 1938/M CF Hyperglycemia 4 wk NVI 8 None CF 1252/F CF Hypertension, DM 3 wk NVG 1 PRP HM 3555/F HM Hypertension, DM 2 wk NVG NR PRP HM 12

Abbreviations: CF, counting fingers; CRVO, central retinal vein occlusion; DM, diabetes mellitus; HM, hand motions, NR, not recorded; NVA, NV of the angle;NVD, NV of the disc; NVE, NV elsewhere; NVG, neovascular glaucoma; NVI, NV of the iris; PPV, pars plana vitrectomy; PRP, panretinal photocoagulation;Rx, treatment; VA, visual acuity.

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Page 5: Clinical Course of Younger Patients  With Central Retinal Vein Occlusion

In the present study, 67 consecutive patients aged 18to 55 years with CRVO followed-up at a single institutionwere studied. The presenting VA was 20/40 or better in42% and 20/200 or worse in 28%. These percentages areconsistent with those reported by Walters and Spalton9 andby Fong et al10 in their aggregate of 103 cases. Priluck etal8 reportedahigher incidenceofpoorpresentingVA(50%).

The statistical analyses in the present study applyto the 57 patients who were followed-up for at least 6months. Final VA was reduced to 20/200 or worse in 40%.This overall level of poor final VA is higher than that inprevious reports (range, 9%-32%).8-10 The incidence ofintraocular NV was 18% in the present series. The rateof neovascular glaucoma (5%) is consistent with previ-ous reports, while the rate of NV of the posterior seg-ment (9%) is higher. Intraocular NV was diagnosed laterthan 6 months following the presumed onset of CRVOin 50% of the patients in whom it occurred, irrespectiveof presenting VA. This result underscores the impor-tance of regular and vigilant examination.

The major aim of the present study was to detail theclinical course of patients subdivided by presenting VA.Prognostic information available from presenting VA isvaluable for patient counseling and treatment recommen-dations. In the CVOS, for example, among the cohort ofpatients with a VA of 20/40 or better, 65% retained VA inthat range, and only 10% declined to levels worse than 20/200. Patients in the CVOS with a presenting VA of lessthan 20/200 were unlikely to experience visual improve-ment and were more likely to develop intraocular NV.3 Ourresults in younger patients, however, stand in contrast withthose of the CVOS, in that presenting VA does not appearto be predictive of final VA or clinical course. In youngerpatients with initially good VA (20/40 or better), only 13(59%) of 22 patients remained at that level while, morestrikingly, 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 im-proved, and a full one fourth attained a VA of 20/40 orbetter. Moreover, the incidence of NV was not signifi-cantly different between the cohorts of patients with ini-tially good and initially poor VA (18% compared with 25%;P=.70 by the Fisher exact test).

While an increasing number of systemic factors (es-pecially those related to thrombophilia)11,12 have been re-ported to be associated with CRVO in younger patients,the appropriate extent of systemic evaluation remains un-clear. Conclusions regarding the advisability of sys-temic testing based on the current series should be drawnwith caution, since testing of matched controls was notperformed. Additionally, since practice patterns evolvedwith changes in contemporary knowledge and publica-tions over the 10-year study period, testing was not uni-form for all patients. It is notable, however, that 4 of 30patients without known prior disease were found to havecommon conditions (anemia, hyperglycemia, and hy-percholesterolemia) that both have systemic ramifica-tions and are potentially treatable (Figure).

Limitations of the present study are mainly those in-herent to any retrospective review. There is a possibilityof selection bias, in that milder cases of CRVO may notbe referred to retina specialists. Long-term data may be bi-

ased toward more severe cases, as patients with a benigncourse may not maintain regular examinations. This po-tential bias was assessed by comparing the group of 57 pa-tients with at least 6 months of follow-up with the groupof 10 patients with less follow-up; no significant differ-ences in baseline parameters were found. Standardized fluo-rescein angiography, as established in the CVOS, was notperformed in every patient. The quality of angiographicimages and the range of funduscopic views provided werenot consistently sufficient to allow meaningful interpre-tation, thus precluding a rigorous assessment of perfu-sion or ischemia. However, VA is correlated with extentof perfusion and offers a general indication of perfusionstatus.1 The present study may thus provide practical in-formation for cases in which fluorescein angiography maynot be readily available.

It is hoped that our results will aid in the counsel-ing of younger patients with CRVO, especially with re-spect to the appropriateness and timing of therapeuticintervention. For example, a significant number ofyounger patients with a presenting VA of 20/200 to 20/400 may recover vision spontaneously, albeit slowly. Onthe other hand, it appears that a patient with initially goodvision who loses vision after 3 months may be unlikelyto recover spontaneously, and visual improvement in anypatient is unlikely after 12 months. Thus, interventionsmay be justified at an early stage in such cases.

Submitted for publication May 21, 2003; final revision re-ceived October 16, 2003; accepted November 6, 2003.

This research was supported in part by the Heed Oph-thalmic Foundation Cleveland, Ohio, the AOS-Knapp Foun-dation,Cleveland, and the Ronald Michels Fellowship Foun-dation, Baltimore, Md (Dr Recchia).

This research was presented in part at the 106th an-nual meeting of the American Academy of Ophthalmology;October 23, 2002; Orlando, Fla.

Corresponding author and reprints: Franco M. Recchia,MD, 8018 Medical Center East, Vanderbilt University Medi-cal Center, Nashville, TN 37232 (e-mail: [email protected]).

REFERENCES

1. The Central Vein Occlusion Study Group. Baseline and early natural history re-port. Arch Ophthalmol. 1993;111:1087-1095.

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