Sv
D
V
cNyyorarvflcAt
H
Optometry (2007) 78, 402-408
1d
ystemic considerations in bilateral central retinalein occlusion
awn N. Tomasini, O.D., and Bhagya Segu, O.D., M.P.H.
A Hudson Valley Healthcare System, Optometry Service, Castle Point, New York.
AbstractBACKGROUND: Central retinal vein occlusion (CRVO) is a common cause of visual impairment andcan occur at any age. Nonetheless, 90% of patients with CRVO are older than 50 years, and only 10%of CRVO patients are younger than 40 years. Systemic vascular diseases, such as hypertension anddiabetes, are common risk factors for the development of CRVO. However, when a patient less than 50years of age has bilateral and simultaneous central retinal vein occlusions, a hyperviscosity syndromeor inflammatory condition is also suspected.CASE REPORT: This article presents the case of a 40-year-old man with bilateral ischemic CRVO andthe differential diagnoses considered, including systemic lupus erythematosus (SLE), antiphospholipidantibody syndrome (APAS), dysproteinemias, and hyperhomocysteinemia.CONCLUSION: When a CRVO is observed in a young patient, more obscure underlying etiologies mustbe explored. As primary care providers, optometrists need to consider common (and atypical) vascularrisk factors for vein occlusion to prevent further ocular morbidity and systemic complications.Optometry 2007;78:402-408
KEYWORDSAntiphospholipid
antibody syndrome;Central retinal vein
occlusion;Dysproteinemias;Hyperhomocysteinemia;Systemic lupus
erythematosus
rt
iadaerdbs2resri
Central retinal vein occlusion (CRVO) is a commonause of visual impairment and can occur at any age.1,2
onetheless, 90% of patients with CRVO are older than 50ears, and only 10% of CRVO patients are younger than 40ears.1,3 The central retinal vein is particularly susceptible tocclusion at the site of the lamina cribrosa, where the centraletinal vein and central retinal artery are in close proximitys they exit the eye. Arteriosclerosis of the adjacent centraletinal artery may result in compression of the central retinalein causing the vein to collapse, thereby impeding bloodow. Decreased blood flow results in increased pressure andan be thrombus forming, resulting in a vein occlusion.lternatively, increased intraocular pressure can accentuate
he external pressure difference as the vein exits the eye,
Corresponding author: Dawn N. Tomasini, O.D., VA Hudson Valleyealthcare System, Optometry Service, Castle Point, New York 12511.
rE-mail: [email protected]
529-1839/07/$ -see front matter © 2007 American Optometric Association. Alloi:10.1016/j.optm.2006.12.017
esulting in turbulent blood flow predisposing the vein to ahrombotic event.2
A CRVO can be classified as nonischemic (perfused) orschemic (nonperfused) depending on the degree of block-ge. Nonischemic CRVO is characterized by less than 10isc diameters of capillary nonperfusion with fluoresceinngiography, visual acuity better than 20/400, tortuosity andngorgement of retinal vessels, mild intraretinal hemor-hages in all 4 quadrants, optic disc edema, and variousegrees of macular edema. Ischemic CRVO is characterizedy at least 10 disc diameters of retinal capillary nonperfu-ion with fluorescein angiography, visual acuity worse than0/400, more extensive retinal hemorrhages in all 4 quad-ants, sheathed arteries with gross retinal and maculardema. Visual acuity is generally worse with ischemic CRVOecondary to more extensive macular edema.4 Furthermore,etinal hypoxia in ischemic CRVO can stimulate neovascular-zation of the anterior and posterior segment of the eye, often
esulting in neovascular glaucoma and retinal detachment.3rights reserved.
satbraPvtabt
C
AichmmLaao
rmemtdsluwroDsuc
(mp(tdrs
ctfssfiws
pplgcvbFas
vipg
Fflm
403Tomasini and Segu Clinical Care
Central retinal vein occlusions have been associatedtrongly with systemic disease. Therefore, when diagnosed,thorough investigation for an underlying systemic condi-
ion must be considered. Necessary testing may includelood work, chest x-ray, carotid ultrasound scan, and evenadiologic imaging. The etiology can be quite varied, butge can be helpful in determining the differential diagnosis.atients older than 50 years usually have common systemicascular conditions such as hypertension or diabetes that puthem at risk for a vascular occlusive event. However, whenCRVO occurs in a patient less than 50 years of age or is
ilateral in nature, a hyperviscosity syndrome or inflamma-ory condition is also suspected.1
ase report
40-year-old white man presented to the Veterans Admin-stration (VA) optometry clinic with the complaint of de-reased vision over the previous 3 weeks, which began inis right eye and progressed to his left eye. The patient’sedical history included type I diabetes with insulin treat-ent of 10 years’ duration. He had also been treated foryme disease 1.5 years before. His best-corrected visualcuities on presentation were 20/200 in the right eye (O.D.)nd 20/400 in the left eye (O.S.). Anterior segment andcular tensions were normal.
Dilated fundus examination found flame-shaped hemor-hages in all 4 quadrants, optic disc edema, and severeacular edema in both eyes (see Figure 1). Based on the
xamination findings, the patient had bilateral CRVO withacular edema diagnosed. Results of our initial serologic
esting confirmed that the patient had poorly controllediabetes. His hemoglobin A1C, blood urea nitrogen, anderum creatinine levels were all elevated. Increased levels ofiver enzymes also indicated a fatty liver likely caused byncontrolled diabetes. Erythrocyte sedimentation rate (ESR)as also elevated, and his antinuclear antibody (ANA) test
esult was positive, indicating a possible autoimmune dis-rder. Results of further testing for anti–double strandedNA were positive, which strongly supported a diagnosis of
ystemic lupus erythematosus. His lipid profile confirmedncontrolled hyperlipidemia with elevated levels of total
igure 1 At the initial exam, dilated fundus examination revealedame-shaped hemorrhages in all 4 quadrants, optic disc edema, andacular edema in both eyes.
holesterol, triglycerides, and low-density lipoproteins
LDL). The complete blood cell count with differential hadinor abnormalities including a low red blood cell count
ossibly indicative of mild anemia. Rapid plasma reaginRPR), angiotensin-converting enzyme (ACE), and lymeiter were all negative (see Table 1). In addition, carotiduplex ultrasound results were normal. The patient waseferred for a primary care and hematology consult andcheduled to return in 2 weeks.
By the 2-week follow-up the patient had seen a primaryare practitioner (PCP) at the VA hospital and had hyper-ension diagnosed, for which he was taking lisinopril andurosemide. In addition, the patient was started on atorva-tatin for hyperlipidemia. The PCP maintained a slidingcale schedule for the diabetes. The ocular examinationndings remained unchanged, and similar retinal findingsere reported with the exception of additional cotton-wool
pots, more apparent in the left eye than the right eye.Despite several attempts to contact the patient and the
atient’s next of kin, the patient was lost to follow-up. Theatient returned 3 months later after having had macularaser treatment to the left eye with a private ophthalmolo-ist. Visual acuities were now 20/200� O.D. and fingerounting at 3 feet O.S. Retinal examination showed neo-ascularization of the disc and persistent macular edema inoth eyes, with focal laser scars evident in the left eye (seeigure 2). The patient was referred for pan retinal photoco-gulation. After treatment, the patient moved to anothertate to be closer to his family.
The patient returned 4 years later to explore options forisual enhancement. He presented with unaided visual acu-ties of no light perception in both eyes (OU). However, theatient denied any ocular pain or discomfort and reportedood mobility with a walking cane. He reported that pan
Table 1 Initial blood tests
Test Value Reference range
Hemoglobin A1c 8.6 4.6-6.5Urea nitrogen 26 mg/dL 7-18Serum creatinine 1.0 mg/dL 0.6-1.3ESR 52 0-10RPR Nonreactive NonreactiveACE 53 8-52Total cholesterol 284 140-200Triglycerides 180 35-160HDL 34 29-71LDL 214 0-130CHL/HDL 8 3.3-11ANA Positive NegativeLYME Negative NegativeDNA AB Positive NegativeP-time 13.4 11.5-14.2INR 1.08 0.8-1.2PTT 31.8 23.3-36.4
LDL � low-density lipoprotein; HDL � high-density lipoprotein;CHL � combined hyperlipidemia; INR � international normalized ratio;PTT � partial thromboplastin time.
rw
cHspgai
tenpisrtchapsp
D
Aiasipctetobypc
vosm
De
Waaaieta
ottpetibaovcsRal
skr
FnT
404 Optometry, Vol 78, No 8, August 2007
etinal photocoagulation had been performed in both eyes asell as a vitrectomy in the left eye.Between visits, the patient suffered from a cerebrovas-
ular accident with residual left hemiparesis at the age of 43.is medical conditions included type I diabetes, hyperten-
ion, hyperlipidemia, anemia, and depressive disorder. Theatient was on a sliding scale insulin schedule (novolin andlargine) for diabetes; hydrocholorothiazide, clopidogrel,nd felodipine for hypertension; atorvastatin for hyperlip-demia; and aspirin.
Slit lamp examination found florid neovascularization ofhe iris in the left eye and dense lens opacification in bothyes. The patient’s ocular tensions were normal despite iriseovascularization in the left eye. Because no view of theosterior segment was obtained with dilated fundus exam-nation, a B-scan ultrasound scan was performed thathowed a possible retinal detachment or vitreous hemor-hage in the right eye, whereas the retina appeared intact inhe left eye. With a medical history that now included aerebrovascular accident at the age of 43, a more extensiveematology panel was ordered (see Table 2) with specificttention to disorders that cause a hypercoaguable state. Theatient was referred to a social worker for visually impairedervices and was educated to continue follow-up with hisrimary care provider.
iscussion
central retinal vein occlusion can be a visually devastat-ng event. However, when it occurs bilaterally it can be lifeltering. This patient’s visual prognosis was poor from thetart considering the entering visual acuities, the amount ofschemia, and gross macular edema, as well as the patient’soor compliance with follow-up appointments. Efforts wereoncentrated on diagnosing an underlying condition, so thatreatment could be started before any other adverse systemicffects occurred. The consulting rheumatologist was tenta-ive about the diagnosis of lupus because there were nother common lupus manifestations; the diagnosis wasased solely on the initial blood test results. However, 3ears after the initial visit, a more extensive hematologyanel suggested elevated homocysteine (HCT) level as a
igure 2 Retinal examination at the 3-month follow-up visit revealedeovascularization of the disc and persistent macular edema in both eyes.he patient was referred for subsequent pan retinal photocoagulation.
ontributing cause for the patient’s bilateral central retinal
ein occlusions and cerebrovascular accident. The hematol-gist initiated treatment for the elevated HCT level bytarting the patient on folic acid and vitamin B12 supple-entation.
ifferential diagnosis considered based onxamination findings
hen a central retinal vein occlusion is diagnosed bilater-lly in a patient less than 50 years of age, it is important thatll the possible differential diagnoses be considered. Inddition to the common underlying causes for CRVO,ncluding hypertension and diabetes, this patient was alsovaluated for systemic lupus erythematosus (SLE), an-iphospholipid antibody syndrome (APAS), dysproteinemia,nd hyperhomocysteinemia.
Based on this patient’s initial laboratory test results, SLEriginally was the primary diagnosis. Systemic lupus ery-hematosus is a chronic inflammatory autoimmune diseasehat affects multiple organ systems and is characterized byrominent autoantibody production. The course of the dis-ase is varied and tends to go through periods of exacerba-ions and remissions. More than 80% of cases of SLE occurn African-American or Hispanic women during their child-earing years. The prevalence in children and older adults ispproximately 1 per 100,000, with a male to female ratiof 3:1.5-7 The most common systemic manifestations in-olve the dermatologic and musculoskeletal systems, spe-ifically arthritis, myopathy, and arthralgia.8 The skin canhow signs of malar rash, discoid rash, alopecia, andaynaud’s phenomenon. Hematologic conditions such asnemia, leucopenia, and thrombocytopenia are also preva-ent.9
Ocular findings in SLE include anterior segment signsuch as keratoconjunctivitis sicca, superficial punctateeratitis, conjunctivitis, episcleritis and scleritis.9 Poste-ior segment manifestations of SLE include retinal mi-
Table 2 Subsequent hematology results (3 years later)
Test Value Reference range
Factor V leiden Negative NegativeProtein C 157 74-151Protein S 53 60-145Total cholesterol 208 mg/dL 140-200Triglyceride 97 mg/dL 140-200HDL 37 mg/dL 29-71Hemoglobin A1c 8.5 4.6-6.5ESR 23 mm/h 0-10Serum creatinine 2.1 mg/dL 0.6-1.3Anticardiolipin antibodiesIgG, IgA, IgM
Negative Negative
Methylmalonic acid 689 nmol/L 73-376Homocysteine 18.1 umol/L 5.8-11.9
HDL � high-density lipoprotein; ESR � erythrocyte sedimentationrate.
chpevcaivlfarnpsswcsa
HwantilltAbhbcmaaiaa6neTlclcsas
etb
OptlVbdc
vpfntnt
pcpddeOr
toaovtsmocgndo
tdodftdrkniitfw
405Tomasini and Segu Clinical Care
rovasculopathy such as cotton-wool spots and retinalemorrhages.10 These findings parallel the systemic diseaserocess and can appear and disappear with periods ofxacerbations and remissions, respectively. Large-caliberaso-occlusion (such as central retinal artery occlusion andentral retinal vein occlusion) has also been reported, withrterial occlusions occurring more frequently.11 Originally,t was believed that vascular occlusions were the result of aasculitis; however, histopathologic evidence has shownittle to no active inflammation. More recently it has beenound that lupus anticoagulant, anticardiolipin antibodies,nd other antiphospholipid antibodies, play an importantole in lupus vasculopathy. In this patient’s case, the anti-uclear antibody and the DNA antibody results were bothositive. The DNA antibody test is highly specific forystemic lupus erythematosus. Although this patient haderologic markers indicative of lupus, the rheumatologistas hesitant about the diagnosis because of the lack of
linical signs. Furthermore, the patient had no dermatologicigns of SLE and only vague symptoms of fatigue toccount for musculoskeletal manifestations.
Antiphospholipid antibody syndrome, also known asughes or sticky blood syndrome, is another diagnosis thatas considered. The association between antiphospholipid
ntibodies and vascular events, including arterial and ve-ous thrombosis, excessive blood clotting, recurrent spon-aneous abortion, cerebrovascular accidents, and myocardialnfarction in young people, has been strong. Antiphospho-ipid antibodies have been found in patients with systemicupus erythematosus as well as other autoimmune condi-ions such as leukemia and lymphoproliferative disorders.ntiphospholipids are circulating immunoglobulins thatind to phospholipids and autoantibodies, leading to aypercoagulable state and recurrent thrombosis. The anti-odies commonly found are IgG anticardiolipin, IgM anti-ardiolipin, and lupus anticoagulant. Patients with APASay have positive findings for an anticardiolipin antibody
nd not for lupus anticoagulant; therefore, testing for all 3ntibodies must be performed for a correct diagnosis. APASs defined by an episode of arterial or venous thrombosisnd either a medium or high titer anticardiolipin or lupusnticoagulant test result on 2 occasions separated by at leastweeks. One elevated level of anticardiolipin is not diag-
ostic of APAS because it is unclear if the levels arelevated because of the thrombotic episode or as a result.herefore, a second elevated level should be confirmed at
east 6 weeks later.12 Thrombosis has been found moreommonly with lupus anticoagulant than with anticardio-ipin antibodies. APAS can involve almost any organ andause a diverse range of disorders within any one organystem. If these antibodies are present without any associ-ted disorder, it is referred to as primary antiphospholipidyndrome (PAPS).9
The most common manifestation is venous thrombosis,specially deep venous thrombosis of the legs.12 Arterialhromboses occur less frequently but usually involve the
rain in the form of a stroke or transient ischemic attack. ncular retinal findings have been found in up to 88% ofatients with APAS and can include transient visual loss,ransient diplopia, ischemic optic neuropathy, retinal vascu-ar occlusions, and peripheral proliferative retinopathy.12,13
enous tortuosity occurs in 70% of APAS patients and haseen associated with multiple retinal vein occlusions. Opticisc edema may be the result of thrombosis of the posterioriliary arteries leading to an ischemic optic neuropathy.12
Because APAS is rare, it is an unlikely cause of retinalascular occlusion; however, if no other risk factors areresent, it has been suggested that these patients be testedor APAS. In our patient’s case, hematology results wereegative for anticardiolipin antibodies. In addition, the pa-ient’s complete blood count with differential showed aormal white blood cell and lymphocyte count, which madehe diagnosis of leukemia or lymphoma unlikely.
Dysproteinemia results from an abnormal amount ofrotein or an abnormal nature of protein in the blood. Thisondition can occur as a primary disease from neoplasticroliferation of reticuloendothelial cells or as a secondaryisease associated with cirrhosis, nephrosis, or collagenisease. Waldenstrom macroglobulinemia and multiple my-loma are 2 primary dysproteinemias that affect the eye.cular manifestations can include bilateral hyperviscosity
etinopathy and complete central retinal vein occlusion.8
Waldenstrom macroglobulinemia is a chronic indolentype of lymphoma characterized by malignant proliferationf plasma cells that invade the bone marrow, lymph nodes,nd the spleen, resulting in the secretion of large quantitiesf IgM. The excess IgM causes an increase in serumiscosity. The disease is more common in white men olderhan 65; however, it can occur in younger patients. Systemicymptoms usually include enlarged lymph nodes, spleno-egaly, headaches, weight loss, confusion, dizziness, loss
f coordination, weakness, fatigue, and epistaxis. In severeases, 30% to 67% of patients with Waldenstrom macro-lobulinemia have been found to have hyperviscosity reti-opathy and heart failure. Retinal changes include venousilation, which can eventually result in central retinal veincclusion.8
Multiple myeloma affects more than 50,000 patients inhe United States making it the most common form ofysproteinemia.14 The abnormally elevated protein in 70%f cases is IgG and IgA in 29% of cases. Median age atiagnosis ranges between 60 and 75 years, and it is rarelyound in patients younger than 40. Multiple myeloma is alsowice as likely to occur in patients of African-Americanescent compared with the white population. This conditionesults from the abnormal proliferation of plasma cells alsonown as myeloma cells. Plasma cells, when producedormally, are needed for the immune system to fight offnfection. However, an excess of these cells will result in anmpaired immune system, anemia, and poor kidney filtra-ion. Myeloma cells also weaken bones, resulting in boneractures and hypercalcemia, a condition often associatedith a loss of appetite, nausea, thirst, fatigue, muscle weak-
ess, restlessness, and confusion. The systemic manifesta-
tgi
a3wpeh
fa
ecpoavhlcd
bp
406 Optometry, Vol 78, No 8, August 2007
ions of multiple myeloma are similar to those of macro-lobulinemia, with the main difference being the bonenvolvement that occurs with multiple myeloma.8
Although dysproteinemias are unlikely in this patient’sge, a hematologist ordered blood tests at the initial and-year visit. The patient failed to show up for the bloodorkup at the initial visit; however, at the 3-year visit theatient’s hematology panel was negative for abnormal lev-ls of IgG, IgA, and IgM, suggesting the patient did notave a dysproteinemia.
Homocysteine is a naturally occurring sulfur amino acidormed during the metabolism of methionine, an essentialmino acid derived from dietary protein.15 Mild to moderate
Table 3 Systemic manifestations of differential diagnosis
Systemic lupuserythematosus
Antiphosantibodysyndrome
Musculoskeletal ArthritisArthralgiaMyalgia
Cutaneous Butterfly rashAlopeciaPhotosensitivityRaynaud
phenomenon
Skin ulceLivedo reSkin nod
Central Nervous System Personality disorderSeizuresMigraine headachesPseudotumor
cerebri
CerebralStrokeMigraine
headacEpilepsy
Cardiopulmonary PericarditisMyocarditisEndocarditis
Myocardiinfarct
Pulmonarhypert
ValvularRenal Proteinuria
Nephrotic syndromeRenal failure
Renal veithromb
Glomerulthromb
Malignanhypert
Vasculitis
Gastrointestinal Mild painDiarrheaAbnormal liver
enzymes
Gut ischeHematemLiver vein
thromb
Retinal HemorrhagesCotton-wool spots
Arterial oocclus
Venous tVasculitisCotton-wVascular
sheathThrombosis Venous
ArterialVenousArterial
levated homocysteine levels, also known as hyperhomo-ysteinemia, are found in only 5% to 7% of the generalopulation. Patients usually are asymptomatic until the thirdr fourth decade of life, at which time premature coronaryrtery disease may develop, along with recurrent arterial andenous thrombosis.16 Homocystinuria is a variant of hyper-omocysteinemia, characterized by severely elevated HCTevel that is subsequently excreted into the urine. Homo-ystinuria is associated with dislocated lenses, mental retar-ation, and thromboembolic events.17
A variety of environmental and systemic factors haveeen associated with elevated HCT.15 However, the 2 mostrevalent factors contributing to elevated HCT levels in
id
Dysproteinemias Hyperhomocysteinemia
WeaknessBone pain or tenderness
Deep vein thrombosisBone fractures
isPallor
ia HeadachesDizzinessVertigoSeizures
Depression in Parkinsonpatients
Alzheimer dementiaStroke
Congestive heart failureOrthostatic hypotensionDypsnea
Myocardial infarctionCoronary artery disease
Venous dilationHemorrhagesCompressions at A/V
crossing changesVenous beadingVenous occlusionDisc congestionPosterior pole exudates
Elevated serumcreatinine levels
Renal failure
Renal abnormalitiesRenal failureHepato-splenomegalyGastrointestinal
obstructionus
ty
ts
Venous occlusionArtery occlusion
Venous
pholip
rsticularules
ischem
hes
alionyensiondiseasenosis
arosistension
miaesis
osis
r venoionortuosi
ool spo
ing
ymHvtdi
detlcpvv
sCbgvtb
a
cfmvrsolf3mdlt
apr
M
Moiti4ecathupoicntn
407Tomasini and Segu Clinical Care
oung patients are diet and genetics. Homozygous autoso-al recessive mutations of the enzymes used to metabolizeCT can cause increase levels of HCT in the body.18 Folate,itamin B12, and vitamin B6 are necessary cofactors forhese enzymes to function effectively. Therefore, a dietaryeficiency in any of the cofactors can also contribute toncrease HCT levels in the body.15
Elevated HCT causes damage at the vascular level byecreasing production of the necessary elements needed forndothelial cell synthesis, releasing oxygen free radicalshat damage existing endothelial cells and causing the pro-iferation of vascular smooth muscle cells. The vascularhanges secondary to elevated HCT level increase theatient’s risk for a vascular occlusive event such as deepein thrombosis, stroke, myocardial infarction, and retinalein occlusion at a premature age.15,18
A retrospective chart review study conducted by Vine15
howed an association between hyperhomocysteinemia andRVO. The study documented that 55% of patients withilateral CRVO had elevated HCT levels. There is also areater risk for an ischemic CRVO resulting in severeisual loss in patients with total plasma HCT level abovehe 95th percentile. Retinal artery occlusions have alsoeen linked.15
Patients with HCT levels greater than 10-�mol/L unitsre considered to be at highest risk for systemic and ocular
Table 4 Laboratory tests for differential diagnosis
Condition Laboratory test
Systemic lupus erythematosus ● Anticardiolipin antibodies● Lupus anticoagulant● Antinuclear antibodies
Antiphospholipid syndrome ● Lupus anticoagulant● Anticardiolipin profile
Dysproteinemia ● IgG, IgA, IgMHomocysteine ● Plasma homocysteine
● Folate● Vitamin B6● Vitamin B12
Table 5 Central Vein Occlusion Study (CVOS) classification an
CVOS Nonischemic CRVO
Characteristics ● Perfused capillaries seen on fluoresceinangiogram
● Visual acuity usually better than 20/400● Relatively mild degree of intraretinal
hemorrhage● Reduction of visual acuity mostly from
macular edemaProgression ● Approximately 25% will convert to
ischemic occlusions within the first monthTreatment ● For macular edema—Grid photocoagulatio
reduced macular edema but did notimprove visual acuity
omplications.15 The hematology tests that should be per-ormed in patients suspected of having hyperhomocysteine-ia include determining HCT, folic acid, vitamin B6, and
itamin B12 levels. The treatment of elevated HCT levels iselatively simple with changes in dietary habits and vitaminupplementation. Studies have found that a 6-week coursef 400 �g of folic acid combined with vitamin B6 and B12owered HCT levels by 30%. Furthermore, 1 to 5 mg ofolic acid supplements also decrease HCT levels by up to0%.18 At our patient’s 3-year follow-up examination, aore extensive hematology panel was performed, and it was
iscovered that the patient had significantly elevated HCTevels. Therefore, the primary care physician involved inhis case initiated the patient on folic acid supplementation.
A comparison of the systemic manifestations of theforementioned etiologies for bilateral CRVO and appro-riate laboratory tests to order are listed in Tables 3 and 4,espectively.
anagement and treatment of CRVO
anagement of a central retinal vein occlusion, regardlessf the etiology, is determined by whether the occlusion isschemic or nonischemic. A perfused or nonischemic cen-ral retinal vein occlusion usually has a mild degree ofntraretinal hemorrhages and visual acuity better than 20/00. In a perfused central retinal vein occlusion, maculardema is responsible for loss of visual acuity. An ischemicentral retinal vein occlusion is defined by a fluoresceinngiogram that shows larger areas of capillary nonperfusionhan the nonischemic CRVO, with extensive intraretinalemorrhages and visual acuity of 20/400 or worse. Innilateral cases of ischemic CRVO, a relative afferentupillary defect may be present as a result of the destructionf the retina from nonperfusion. Management includes ret-nal evaluations every month for the first 4 months, withareful monitoring of the iris and anterior chamber angle foreovascularization. If neovascularization develops, referralo a retina specialist for pan retinal photocoagulation isecessary.3,4
ment19
Ischemic CRVO
● Large area (10 disc areas or more) of capillary nonperfusionon fluorescein angiogram
● Extensive intraretinal hemorrhages● Visual acuity of 20/400 or worse● Reduction of visual acuity from capillary nonperfusion in
macular region
● Up to 50% will develop iris neovascularization with 2 to 3months of onset
● Pan retinal photocoagulation at first sight of irisneovascularization
d treat
n
fvuelovntornwrvv
C
Cstuprdo
R
1
1
1
1
1
1
1
1
1
1
408 Optometry, Vol 78, No 8, August 2007
The Central Retinal Vein Occlusion Study Group setorth guidelines for proper management of central retinalein occlusions.12 The results of the study indicate that mac-lar grid laser treatment is not beneficial in eyes with maculardema and visual acuity of 20/50 or worse. Treatment withaser did show a reduction in the amount of macular edeman fluorescein angiography; however, no improvement inisual acuity was reported and therefore such treatment isot indicated. This study also examined whether prophylac-ic laser treatment would be effective in reducing the devel-pment of neovascularization in cases of ischemic centraletinal vein occlusion. Patients prophylactically treated hadeovascularization at almost the same rate as those thatere not treated. In addition, eyes without prophylactic laser
esponded better to pan retinal photocoagulation once neo-ascularization did develop.19 Ischemic and nonischemicein occlusions are compared in Table 5.
onclusion
entral retinal vein occlusion is generally associated withystemic vascular disease such as hypertension and diabe-es. However, when CRVO occurs bilaterally, more obscurenderlying etiologies must be explored. As primary careroviders, optometrists need to consider common vascularisk factors for vein occlusion, as well as the conditionsiscussed in this report in atypical cases, to prevent furthercular morbidity and systemic complications.
eferences
1. Lahey JM, Tunc M, Kearney J, et al. Laboratory evaluation ofhypercoagulable states in patients with central retinal vein occlu-sion who are less than 56 years of age. Ophthalmology 2002;109(1):126-31.
2. Lahey JM, Kearney JJ, Tunc M. Hypercoagulable states and central
retinal vein occlusion. Curr Opin Pulm Med 2003;9(5):385-92.3. Prisco D, Marcucci R. Retinal vein thrombosis: risk factors, pathogen-esis and therapeutic approach. Pathophysiol Haemost Thromb 2002;32(5-6):308-11.
4. Cooney MJ, Fekrat S, Finkelstein D. Current concepts in the manage-ment of central retinal vein occlusion. Curr Opin Ophthalmol 1998;9(3):47-50.
5. Arevalo JF, Lowder CY, Muci-Mendoza R. Ocular manifestations ofsystemic lupus erythematosus. Curr Opin Ophthalmol 2002;13(6):404-10.
6. Wong NN. Systemic lupus erythematosus. In: Thomann KH, MarksES, Adamcyzk DT (eds). Primary eyecare in systemic disease, 2nd ed.New York: McGraw Hill, 2001; 291-301.
7. Mills JA. Systemic lupus erythematosus. N Engl J Med 1994;330(26):1871-9.
8. Myers H. Dysproteinemias. In: Thomann KH, Marks ES, AdamcyzkDT (eds). Primary eyecare in systemic disease, 1st ed. Norwalk:Appleton & Lange, 1995; 526-32.
9. Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome.N Engl J Med 2002;346(10):752-63.
0. Song YH, Kim CG, Kim SD, et al. Systemic lupus erythematosuspresenting earlier as retinal vaso-occlusion. Korean J Intern Med2001;16(3):210-3.
1. Sawa M, Saito Y, Kameda C, et al. Ocular complications in systemiclupus erythematosus—choroidal and retinal pigment epithelialchanges. Nippon Ganka Gakkai Zasshi 2002;106(8):474-80.
2. Bolling JP, Brown GC. The antiphospholipid antibody syndrome. CurrOpin Ophthalmol 2000;11:211-3.
3. Glacet-Bernard A, Bayani N, Chretien P, et al. Antiphospholipidantibodies in retinal vascular occlusions. Arch Ophthalmol 1994;112:790-95.
4. Stadtmauer EA. Multiple myeloma, 2004—one or two transplants?N Engl J Med 2003;349(26):2551-3.
5. Vine AK. Hyperhomocysteinemia: a new risk factor for central retinalvein occlusion. Trans Am Ophthalmol Soc 2000;98:493-503.
6. Hasset AC. Hyperhomocysteinemia. The Institute for TransfusionMedicine. 2003: Issue 6. Available at: http://www.itxm.org/tmu2003/issue2003-6.htm. Last accessed April 4, 2007.
7. Yildirim C, Yaylali V, Tatlipinar S, et al. Hyperhomocysteinemia: arisk factor for retinal vein occlusion. Ophthalmologica 2004;218(2):102-6.
8. Brown BA, Marx JL, Thomas WP, et al. Homocysteine: a risk factorfor retinal venous occlusive disease. Am Acad Ophthalmol 2002;109:287-90.
9. The Central Vein Occlusion Study Group. Natural history and clinicalmanagement of central retinal vein occlusion. Arch Ophthalmol 1997;
115(4):486-91.Recommended
