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Systemic considerations in bilateral central retinal vein occlusion

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  • S latve

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    ein ocs, 90%

    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 50

    Central retinal vein


    Noyeyears.1,3 The central retinal vein is particularly susceptible toocc





    age. Nonischemic CRVO is characterized by less than 10


    Optometry (2007) 78, 402-408

    152doilusion at the site of the lamina cribrosa, where the centralinal vein and central retinal artery are in close proximitythey exit the eye. Arteriosclerosis of the adjacent centralinal artery may result in compression of the central retinalin causing the vein to collapse, thereby impeding bloodw. Decreased blood flow results in increased pressure and

    be thrombus forming, resulting in a vein occlusion.ternatively, increased intraocular pressure can accentuate

    external pressure difference as the vein exits the eye,

    disc diameters of capillary nonperfusion with fluoresceinangiography, visual acuity better than 20/400, tortuosity andengorgement of retinal vessels, mild intraretinal hemor-rhages in all 4 quadrants, optic disc edema, and variousdegrees of macular edema. Ischemic CRVO is characterizedby at least 10 disc diameters of retinal capillary nonperfu-sion with fluorescein angiography, visual acuity worse than20/400, more extensive retinal hemorrhages in all 4 quad-rants, sheathed arteries with gross retinal and macularedema. Visual acuity is generally worse with ischemic CRVOsecondary to more extensive macular edema.4 Furthermore,years 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

    occlusion;Dysproteinemias;Hyperhomocysteinemia;Systemic lupus


    Central retinal vein occlusion (CRVO) is a commonse of visual impairment and can occur at any age.1,2netheless, 90% of patients with CRVO are older than 50

    ars, and only 10% of CRVO patients are younger than 40

    resulting in turbulent blood flow predisposing the vein to athrombotic event.2

    A CRVO can be classified as nonischemic (perfused) orischemic (nonperfused) depending on the degree of block-ystemic considerations in biin occlusion

    wn N. Tomasini, O.D., and Bhagya Segu

    Hudson Valley Healthcare System, Optometry Service, Ca

    AbstractBACKGROUND: Central retinal vcan occur at any age. Nonetheles


    antibody syndrome;retizares

    Corresponding author: Dawn N. Tomasini, O.D., VA Hudson Valleylthcare System, Optometry Service, Castle Point, New York 12511.E-mail: [email protected]

    9-1839/07/$ -see front matter 2007 American Optometric Association. All rig:10.1016/j.optm.2006.12.017eral central retinal

    ., M.P.H.

    oint, New York.

    clusion (CRVO) is a common cause of visual impairment andof patients with CRVO are older than 50 years, and only 10%inal hypoxia in ischemic CRVO can stimulate neovascular-tion of the anterior and posterior segment of the eye, oftenulting in neovascular glaucoma and retinal detachment.3

    hts reserved.

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    403Tomasini and Segu Clinical CareCentral retinal vein occlusions have been associatedongly with systemic disease. Therefore, when diagnosed,horough investigation for an underlying systemic condi-n must be considered. Necessary testing may includeod work, chest x-ray, carotid ultrasound scan, and eveniologic imaging. The etiology can be quite varied, but

    e can be helpful in determining the differential diagnosis.tients older than 50 years usually have common systemicscular conditions such as hypertension or diabetes that putm at risk for a vascular occlusive event. However, whenRVO occurs in a patient less than 50 years of age or is

    ateral in nature, a hyperviscosity syndrome or inflamma-y condition is also suspected.1

    se report

    40-year-old white man presented to the Veterans Admin-ration (VA) optometry clinic with the complaint of de-ased vision over the previous 3 weeks, which began inright eye and progressed to his left eye. The patients

    dical history included type I diabetes with insulin treat-nt of 10 years duration. He had also been treated forme disease 1.5 years before. His best-corrected visualities on presentation were 20/200 in the right eye (O.D.)

    d 20/400 in the left eye (O.S.). Anterior segment andular tensions were normal.Dilated fundus examination found flame-shaped hemor-ges in all 4 quadrants, optic disc edema, and severecular edema in both eyes (see Figure 1). Based on the

    amination findings, the patient had bilateral CRVO withcular edema diagnosed. Results of our initial serologicting confirmed that the patient had poorly controlledbetes. His hemoglobin A1C, blood urea nitrogen, andum creatinine levels were all elevated. Increased levels ofer enzymes also indicated a fatty liver likely caused bycontrolled diabetes. Erythrocyte sedimentation rate (ESR)s also elevated, and his antinuclear antibody (ANA) testult was positive, indicating a possible autoimmune dis-er. Results of further testing for antidouble strandedA were positive, which strongly supported a diagnosis oftemic lupus erythematosus. His lipid profile confirmed

    controlled hyperlipidemia with elevated levels of totalolesterol, triglycerides, and low-density lipoproteins

    ure 1 At the initial exam, dilated fundus examination revealede-shaped hemorrhages in all 4 quadrants, optic disc edema, and

    cular edema in both eyes.DL). The complete blood cell count with differential hadnor abnormalities including a low red blood cell countssibly indicative of mild anemia. Rapid plasma reaginPR), angiotensin-converting enzyme (ACE), and lymer were all negative (see Table 1). In addition, carotid

    plex ultrasound results were normal. The patient waserred for a primary care and hematology consult andeduled to return in 2 weeks.By the 2-week follow-up the patient had seen a primarye practitioner (PCP) at the VA hospital and had hyper-sion diagnosed, for which he was taking lisinopril andosemide. In addition, the patient was started on atorva-tin for hyperlipidemia. The PCP maintained a slidingle schedule for the diabetes. The ocular examinationdings remained unchanged, and similar retinal findingsre reported with the exception of additional cotton-woolts, more apparent in the left eye than the right eye.Despite several attempts to contact the patient and the

    tients next of kin, the patient was lost to follow-up. Thetient returned 3 months later after having had macularer treatment to the left eye with a private ophthalmolo-t. Visual acuities were now 20/200 O.D. and fingerunting at 3 feet O.S. Retinal examination showed neo-scularization of the disc and persistent macular edema inth eyes, with focal laser scars evident in the left eye (seeure 2). The patient was referred for pan retinal photoco-

    ulation. After treatment, the patient moved to anotherte to be closer to his family.The patient returned 4 years later to explore options forual enhancement. He presented with unaided visual acu-s of no light perception in both eyes (OU). However, the

    tient denied any ocular pain or discomfort and reportedod mobility with a walking cane. He reported that pan

    able 1 Initial blood tests

    est Value Reference range

    emoglobin A1c 8.6 4.6-6.5rea nitrogen 26 mg/dL 7-18erum creatinine 1.0 mg/dL 0.6-1.3SR 52 0-10PR Nonreactive NonreactiveCE 53 8-52otal cholesterol 284 140-200riglycerides 180 35-160DL 34 29-71DL 214 0-130HL/HDL 8 3.3-11NA Positive NegativeYME Negative NegativeNA AB Positive Negative-time 13.4 11.5-14.2NR 1.08 0.8-1.2TT 31.8 23.3-36.4

    LDL low-density lipoprotein; HDL high-density lipoprotein;HL combined hyperlipidemia; INR international normalized ratio;TT partial thromboplastin time.

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    404 Optometry, Vol 78, No 8, August 2007inal photocoagulation had been performed in both eyes asll as a vitrectomy in the left eye.Between visits, the patient suffered from a cerebrovas-

    lar accident with residual left hemiparesis at the age of 43.s medical conditions included type I diabetes, hyperten-n, hyperlipidemia, anemia, and depressive disorder. Thetient was on a sliding scale insulin schedule (novolin andrgine) for diabetes; hydrocholorothiazide, clopidogrel,

    d felodipine for hypertension; a