CENTRAL RETINAL ARTERY OCCLUSION ASSOCIATED WITH HYPERTRIGLYCERIDEMIA Tushar M. Ranchod, MD, Alan J. Ruby, MD
Purpose: To report a case of central retinal artery occlusion associated with hypertriglyceridemia. Methods: A 46-year-old man presented with decreased vision and was found to have a central retinal artery occlusion. Laboratory testing demonstrated markedly elevated triglycerides with no other abnormalities. Tests for cardiac and carotid embolic sources and hypercoagulability were negative. Results: Dietary management and medication compliance resulted in dramatically decreased serum triglyceride levels. Vision remained poor during follow-up. Conclusion: Central retinal artery occlusion requires a medical evaluation for embolic and thrombotic sources. A fasting lipid panel is inexpensive to obtain and should be considered in the workup for central retinal artery occlusion. RETINAL CASES & BRIEF REPORTS 4:368–369, 2010
Hypertriglyceridemia is associated with retinal vascular abnormalities, including lipemia retinalis, intravascular lipid embolus deposition, and waxy periarteriolar sheathing.6 Hypertriglyceridemia has also been reported in association with branch retinal vein occlusion as well as a case of multiple branch retinal artery occlusions.7,8 We report a case of CRAO associated with markedly elevated serum triglycerides. To the best of our knowledge, this is the first report of CRAO associated with hypertriglyceridemia.
From the Associated Retinal Consultants, Royal Oak, Michigan.
C
entral retinal artery occlusion (CRAO) in older adults is most commonly associated with embolic disease. The most common etiologies include emboli from carotid or cardiac sources. The less common causes of CRAO include giant cell arteritis and hypercoagulability from deficiencies in antithrombin III, protein C, or protein S.1,2 Central retinal artery occlusion in young patients, in addition to embolic disease, has been associated with hyperhomocysteinemia, trauma, systemic lupus erythematosus, sickle cell disease, platelet aggregation abnormalities, and migraine.3–5 It is recommended that patients who present with new CRAO undergo medical and laboratory examination, including carotid Doppler ultrasound for carotid plaques, echocardiogram for cardiac vegetations or thrombi, and laboratory testing for possible nonembolic etiologies. Hypertriglyceridemia is a disease associated with marked elevation of serum triglycerides either with or without associated elevation of other serum lipids.
Case Report A 46-year-old man presented with 2 days of painless decreased vision in the right eye. Medical history was notable for hypertension and hypercholesterolemia with no known history of coronary artery disease or cerebral arterial occlusive disease. Medications included simvastatin, verapamil, hydrochlorothiazide, and triamterene. The patient reported consuming 1.5 packs of cigarettes and 1 drink of alcohol per day and denied any illicit drug use. Best-corrected visual acuity was counting fingers in the right eye and 20/25 in the left eye. Intraocular pressures were 13 mmHg in the right eye and 16 mmHg in the left eye. External examination was normal without xanthomatous lid changes. Slit-lamp examination was notable only for trace nuclear sclerotic cataracts bilaterally. Dilated fundus examination of the right eye revealed a cup-to-disk ratio of 0.1 with disk hyperemia as well as peripapillary and macular retinal whitening. Scattered cotton wool spots were seen throughout the posterior pole accompanied by
Neither author has a proprietary interest or conflict of interest in this report. Reprint requests: Alan J. Ruby, MD, 3535 W. 13 Mile Road, Suite 344, Royal Oak, MI 48073; e-mail: [email protected]
368
369
CENTRAL RETINAL ARTERY OCCLUSION
Discussion
Fig. 1. A 46-year-old man with severe hypertriglyceridemia presented with retinal whitening, cotton wool spots, and a cherry red spot as manifestations of a CRAO.
a cherry red spot (Figure 1). No intraretinal hemorrhages or intraretinal emboli were visible. Dilated fundus examination of the left eye revealed a healthy disk with a cup-to-disk ratio of 0.2, mild arteriovenous nicking and arteriolar tortuosity, and a normal macula and periphery. Fluorescein angiography revealed delayed arm-to-retina time of 30 seconds, delayed arteriovenous transit time as well as areas of capillary nonperfusion in the right eye (Figure 2). Blood pressure was 170/112 mmHg. Laboratory workup demonstrated a normal complete blood count, sedimentation rate, fibrinogen, international normalized ratio, prothrombin time, partial thromboplastin time, fasting glucose, antithrombin III, protein C, and protein S levels. Antiphospholipid antibody panel testing was normal, and the Factor V Leiden mutation was not present. A fasting lipid panel revealed total cholesterol of 410 mg/dL (normal ,200 mg/dL), triglycerides of 3,205 mg/dL (normal ,200 mg/dL), and highdensity lipoprotein of 28 mg/dL (normal 25—65 mg/dL). The primary care physician reported recent normal carotid ultrasonography and cardiac echography. The primary care physician also recounted a history of poor dietary choices and medication noncompliance. During the next 3 months, visual acuity remained hand motions in the right eye with the development of disk pallor and vascular sclerosis. With improved diet and medication compliance, the patient’s serum triglycerides improved to 649 mg/dL.
Fig. 2. Fluorescein angiography revealed delayed arm-to-retina time, delayed arteriovenous transit, and areas of capillary nonperfusion consistent with CRAO.
The presentation of CRAO in a young individual requires medical evaluation for embolic or thrombotic sources as well as evaluation for less common inflammatory or infectious etiologies. Numerous reports have recommended comprehensive hypercoagulability blood panels, which often include expensive tests, on the rationale that rare, but life-threatening, causes of CRAO should be uncovered and treated. A fasting lipid panel is inexpensive and may reveal potentially life-threatening abnormalities. In this case, a fasting lipid panel revealed triglycerides to be more than 10-fold higher than normal, presumably elevated enough to cause sludging and occlusion of the central retinal artery. To the best of our knowledge, this is the first report of CRAO associated with hypertriglyceridemia. Most published studies of CRAO have not routinely reported serum lipids, and it is possible that severe hyperlipidemia may have been present but undiagnosed in some of these cases. We recommend including a fasting lipid panel as part of the routine blood work for all patients presenting with CRAO. The test is less expensive than tests for coagulation cascade abnormalities or genetic mutations, and ophthalmologists are uniquely positioned to explain the tangible medical consequences of hypercholesteroleinia in the context of permanent vision loss. The diagnosis of CRAO provided a strong incentive to our patient, who dramatically lowered his serum triglycerides in the subsequent months with dietary improvements and medication compliance. Key words: central retinal artery occlusion, hypertriglyceridemia, hyperlipidemia. References 1. Bertram B, Remky A, Arend O, Wolf S, Reim M. Protein C, protein S, and antithrombin III in acute ocular occlusive diseases. Ger J Ophthalmol 1995;4:332–335. 2. Fineman MS, Savino PJ, Federman JL, Eagle RC Jr. Branch retinal artery occlusion as the initial sign of giant cell arteritis. Am J Ophthalmol 1996;122:428–430. 3. Brown GC, Magargal LE, Shields JA, Goldberg RE, Walsh PN. Retinal arterial obstruction in children and young adults. Ophthalmology 1981;88:18–25. 4. Greven CM, Slusher MM, Weaver RG. Retinal arterial occlusions in young adults. Am J Ophthalmol 1995;120:776–783. 5. Sharma S, Sharma SM, Cruess AF, Brown GC. Transthoracic echocardiography in young patients with acute retinal arterial obstruction. RECO Study Group. Retinal Emboli of Cardiac Origin Group. Can J Ophthalmol 1997;32:38–41. 6. Winder AF, Dodson PM, Galton DJ. Ophthalmological complications of the hypertriglyceridaemias. Trans Ophthalmol Soc U K 1980;100:119–122. 7. Dodson PM, Galton DJ, Winder AF. Retinal vascular abnormalities in the hyperlipidaemias. Trans Ophthalmol Soc U K 1981;101:17–21. 8. Kurz GH, Shakib M, Sohmer KK, Friedman AH. The retina in type 5 hyperlipoproteinemia. Am J Ophthalmol 1976;82:32–43.
Purpose: To report a case of central retinal artery occlusion associated with hypertriglyceridemia. Methods: A 46-year-old man presented with decreased vision and was found to have a central retinal artery occlusion. Laboratory testing demonstrated markedly elevated triglycerides with no other abnormalities. Tests for cardiac and carotid embolic sources and hypercoagulability were negative. Results: Dietary management and medication compliance resulted in dramatically decreased serum triglyceride levels. Vision remained poor during follow-up. Conclusion: Central retinal artery occlusion requires a medical evaluation for embolic and thrombotic sources. A fasting lipid panel is inexpensive to obtain and should be considered in the workup for central retinal artery occlusion. RETINAL CASES & BRIEF REPORTS 4:368–369, 2010
Hypertriglyceridemia is associated with retinal vascular abnormalities, including lipemia retinalis, intravascular lipid embolus deposition, and waxy periarteriolar sheathing.6 Hypertriglyceridemia has also been reported in association with branch retinal vein occlusion as well as a case of multiple branch retinal artery occlusions.7,8 We report a case of CRAO associated with markedly elevated serum triglycerides. To the best of our knowledge, this is the first report of CRAO associated with hypertriglyceridemia.
From the Associated Retinal Consultants, Royal Oak, Michigan.
C
entral retinal artery occlusion (CRAO) in older adults is most commonly associated with embolic disease. The most common etiologies include emboli from carotid or cardiac sources. The less common causes of CRAO include giant cell arteritis and hypercoagulability from deficiencies in antithrombin III, protein C, or protein S.1,2 Central retinal artery occlusion in young patients, in addition to embolic disease, has been associated with hyperhomocysteinemia, trauma, systemic lupus erythematosus, sickle cell disease, platelet aggregation abnormalities, and migraine.3–5 It is recommended that patients who present with new CRAO undergo medical and laboratory examination, including carotid Doppler ultrasound for carotid plaques, echocardiogram for cardiac vegetations or thrombi, and laboratory testing for possible nonembolic etiologies. Hypertriglyceridemia is a disease associated with marked elevation of serum triglycerides either with or without associated elevation of other serum lipids.
Case Report A 46-year-old man presented with 2 days of painless decreased vision in the right eye. Medical history was notable for hypertension and hypercholesterolemia with no known history of coronary artery disease or cerebral arterial occlusive disease. Medications included simvastatin, verapamil, hydrochlorothiazide, and triamterene. The patient reported consuming 1.5 packs of cigarettes and 1 drink of alcohol per day and denied any illicit drug use. Best-corrected visual acuity was counting fingers in the right eye and 20/25 in the left eye. Intraocular pressures were 13 mmHg in the right eye and 16 mmHg in the left eye. External examination was normal without xanthomatous lid changes. Slit-lamp examination was notable only for trace nuclear sclerotic cataracts bilaterally. Dilated fundus examination of the right eye revealed a cup-to-disk ratio of 0.1 with disk hyperemia as well as peripapillary and macular retinal whitening. Scattered cotton wool spots were seen throughout the posterior pole accompanied by
Neither author has a proprietary interest or conflict of interest in this report. Reprint requests: Alan J. Ruby, MD, 3535 W. 13 Mile Road, Suite 344, Royal Oak, MI 48073; e-mail: [email protected]
368
369
CENTRAL RETINAL ARTERY OCCLUSION
Discussion
Fig. 1. A 46-year-old man with severe hypertriglyceridemia presented with retinal whitening, cotton wool spots, and a cherry red spot as manifestations of a CRAO.
a cherry red spot (Figure 1). No intraretinal hemorrhages or intraretinal emboli were visible. Dilated fundus examination of the left eye revealed a healthy disk with a cup-to-disk ratio of 0.2, mild arteriovenous nicking and arteriolar tortuosity, and a normal macula and periphery. Fluorescein angiography revealed delayed arm-to-retina time of 30 seconds, delayed arteriovenous transit time as well as areas of capillary nonperfusion in the right eye (Figure 2). Blood pressure was 170/112 mmHg. Laboratory workup demonstrated a normal complete blood count, sedimentation rate, fibrinogen, international normalized ratio, prothrombin time, partial thromboplastin time, fasting glucose, antithrombin III, protein C, and protein S levels. Antiphospholipid antibody panel testing was normal, and the Factor V Leiden mutation was not present. A fasting lipid panel revealed total cholesterol of 410 mg/dL (normal ,200 mg/dL), triglycerides of 3,205 mg/dL (normal ,200 mg/dL), and highdensity lipoprotein of 28 mg/dL (normal 25—65 mg/dL). The primary care physician reported recent normal carotid ultrasonography and cardiac echography. The primary care physician also recounted a history of poor dietary choices and medication noncompliance. During the next 3 months, visual acuity remained hand motions in the right eye with the development of disk pallor and vascular sclerosis. With improved diet and medication compliance, the patient’s serum triglycerides improved to 649 mg/dL.
Fig. 2. Fluorescein angiography revealed delayed arm-to-retina time, delayed arteriovenous transit, and areas of capillary nonperfusion consistent with CRAO.
The presentation of CRAO in a young individual requires medical evaluation for embolic or thrombotic sources as well as evaluation for less common inflammatory or infectious etiologies. Numerous reports have recommended comprehensive hypercoagulability blood panels, which often include expensive tests, on the rationale that rare, but life-threatening, causes of CRAO should be uncovered and treated. A fasting lipid panel is inexpensive and may reveal potentially life-threatening abnormalities. In this case, a fasting lipid panel revealed triglycerides to be more than 10-fold higher than normal, presumably elevated enough to cause sludging and occlusion of the central retinal artery. To the best of our knowledge, this is the first report of CRAO associated with hypertriglyceridemia. Most published studies of CRAO have not routinely reported serum lipids, and it is possible that severe hyperlipidemia may have been present but undiagnosed in some of these cases. We recommend including a fasting lipid panel as part of the routine blood work for all patients presenting with CRAO. The test is less expensive than tests for coagulation cascade abnormalities or genetic mutations, and ophthalmologists are uniquely positioned to explain the tangible medical consequences of hypercholesteroleinia in the context of permanent vision loss. The diagnosis of CRAO provided a strong incentive to our patient, who dramatically lowered his serum triglycerides in the subsequent months with dietary improvements and medication compliance. Key words: central retinal artery occlusion, hypertriglyceridemia, hyperlipidemia. References 1. Bertram B, Remky A, Arend O, Wolf S, Reim M. Protein C, protein S, and antithrombin III in acute ocular occlusive diseases. Ger J Ophthalmol 1995;4:332–335. 2. Fineman MS, Savino PJ, Federman JL, Eagle RC Jr. Branch retinal artery occlusion as the initial sign of giant cell arteritis. Am J Ophthalmol 1996;122:428–430. 3. Brown GC, Magargal LE, Shields JA, Goldberg RE, Walsh PN. Retinal arterial obstruction in children and young adults. Ophthalmology 1981;88:18–25. 4. Greven CM, Slusher MM, Weaver RG. Retinal arterial occlusions in young adults. Am J Ophthalmol 1995;120:776–783. 5. Sharma S, Sharma SM, Cruess AF, Brown GC. Transthoracic echocardiography in young patients with acute retinal arterial obstruction. RECO Study Group. Retinal Emboli of Cardiac Origin Group. Can J Ophthalmol 1997;32:38–41. 6. Winder AF, Dodson PM, Galton DJ. Ophthalmological complications of the hypertriglyceridaemias. Trans Ophthalmol Soc U K 1980;100:119–122. 7. Dodson PM, Galton DJ, Winder AF. Retinal vascular abnormalities in the hyperlipidaemias. Trans Ophthalmol Soc U K 1981;101:17–21. 8. Kurz GH, Shakib M, Sohmer KK, Friedman AH. The retina in type 5 hyperlipoproteinemia. Am J Ophthalmol 1976;82:32–43.
