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Arch Ophthalmol. 2001;119:1552-1553.

INTRODUCTION
Cancer is known to be a hypercoagulable state as described initially by Trousseau in 1865.¹ The mechanism is not well understood. Among the thrombotic events that may accompany tumors, ophthalmic thrombosis, especially retinal vein occlusion, is a known but uncommon phenomenon. However, simultaneous bilateral central retinal vein occlusion is quiet rare and was never described in the literature as a symptom of cancer.

Report of a Case

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A 78-year-old white man with a medical history of hypercholesterolemia treated with simvastatin, and mild chronic obstructive lung disease, was examined in our outpatient ophthalmology clinic because of acute bilateral vision loss lasting for several days. Visual acuity was 20/100 OD and 20/150 OS. Intraocular pressure and anterior segment examination findings in both eyes were normal. Bilateral funduscopy revealed dilation and tortuosity of all branches of the central retinal vein with dot and flame hemorrhages in all quadrants of the retina. Retinal fluorescein angiography demonstrated prolongation of the retinal circulation time with capillary permeability, which suggested the diagnosis of simultaneous bilateral central retinal vein thrombosis. The patient was referred to the department of internal medicine for further work-up. Physical examination findings, including blood pressure, were unremarkable except for the visual disturbances. Initial laboratory findings revealed a marked elevated erythrocyte sedimentation rate (107 mm/h), normal complete blood cell count, normal lipid profile results, and normal liver and kidney function test results. Further work-up revealed normal findings on echocardiography, carotid doppler, and protein electrophoresis, negative antinuclear antibody and antineutrophilic cytoplasmic antibody levels, and normal C3 levels. Chest x-ray films and computed tomographic scan results for the abdomen, head, and orbits were normal. Carcinoembryonic antigen was found to be significantly elevated (54 ng/mL; normal, 0-5 ng/mL), while prostatic specific antigen and CA19-9 (tumor marker for pancreatic cancer) were normal. Laboratory test results for hypercoagulability, including obtained levels of anticardiolipin antibody, protein S, protein C, and antithrombin III were all negative. However, activated protein C resistance assay was positive with the presence of heterozygous factor V Leiden mutation. On follow-up, serum homocysteine level was normal. To further evaluate the elevated carcinoembryonic antigen, a colonoscopy was performed and revealed a nearly obstructive tumor mass in the sigmoid colon. The patient was referred to surgery. Histologically, the tumor was found to be an adenocarcinoma in Dukes stage C1. It should be noticed that the patient denied any abdominal discomfort, change in bowel habits, or weight loss.

Comment

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To the best of our knowledge, simultaneous bilateral central retinal vein thrombosis is a rare ocular thrombotic event. Theoretically, it may be assumed that our patient had experienced an unnoticeable unilateral thrombotic occlusion in the past and the second event occurred prior to his present admission. However, the patient's complaints of bilateral visual deterioration for several days, the funduscopic appearance, and the fluorescein angiography may support our assumption that the thrombotic occlusions occurred simultaneously, rather than as 2 unrelated events.

Acute occlusion of the central retinal vein is one of the most dramatic clinical events in ophthalmology. Occlusion of the central retinal vein is often a result of local or systemic causes.² Local causes, such as open- or narrow-angle glaucoma and trauma were excluded in our patient. Systemic causes can be divided into 4 main categories³: (1) atherosclerosis and other risk factors (mainly systemic hypertension and diabetes mellitus); (2) conditions associated with elevated central venous pressure, such as pulmonary hypertension, tricuspid regurgitation, and right atrial myxoma; (3) hypercoagulability states, such as paraproteinemias, platelet-coagulant abnormalities (activated protein C resistance, protein S, protein C, and antithrombin III deficiencies), and malignancies; and (4) collagen vascular diseases and vasculitides. More recently, the Central Vein Occlusion Study Group demonstrated that an increased risk of central retinal vein occlusion was found in persons with systemic hypertension, diabetes mellitus, and open-angle glaucoma, while increasing levels of physical activity and alcohol consumption were found to decrease the risk of occlusion.⁴

Disorders of coagulation are common in patients with cancer. Clinically significant hemostatic abnormalities, such as thrombosis and hemorrhage, may affect as many as 15% of cancer patients, and hemostatic complications are the second most common cause of mortality. The 2 principal tumor cell procoagulants are tissue factor, which initiates the extrinsic pathway of blood coagulation, and cancer procoagulant, which directly activates factor X.¹ There were 2 causes of the hypercoagulability state in our patient, which predisposed him to the development of this rare ocular thrombotic event. Activated protein C resistance, with or without the factor V Leiden mutation, is known to be a prethrombotic condition, with a direct relation to venous but not to arterial thrombotic disorder. However, our patient did not develop a thrombotic event until the growth of a colon tumor. This occurrence supports the theory that for a thrombotic event to occur, 2 hypercoagulability factors must act synergistically.⁵ In our patient, the first manifestation of a colon tumor was a unique bilateral central retinal vein occlusion.

AUTHOR INFORMATION

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Fani Segev, MD; Amit Segev, MD; Avraham Livne, MD; Ehud I. Assia, MD; Yoseph A. Mekori, MD
Kfar-Saba, Israel

Corresponding author and reprints: Fani Segev, MD, Department of Ophthalmology, Meir Hospital, Kfar-Saba 44281, Israel (e-mail: amitse{at}inter.net.il).

REFERENCES

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1. Goad KE, Gralnick HR. Coagulation disorders in cancer. Hematol Oncol Clin North Am. 1996;10:457-484. FULL TEXT | ISI | PUBMED 2. Sanborn GE, Magargal LE, Jaeger EA. Venous occlusive disease of the retina. In: Tasman W, Jaeger EA, eds. Duane's Clinical Ophthalmology. Vol 3. Philadelphia, Pa: Lippincott-Raven; 1995:1-29. 3. Central Vein Occlusion Study Group. Baseline and early natural history report: the central vein occlusion study. Arch Ophthalmol. 1993;111:1087-1095. ABSTRACT 4. The Eye Disease Case-Control Study Group. Risk factors for central retinal vein occlusion. Arch Ophthalmol. 1996;114:545-554. ABSTRACT 5. Lane DA, Mannucci PM, Bauer KA, et al. Inherited thrombophilia, part 1. Thromb Haemost. 1996;76:651-662. ISI | PUBMED

SECTION EDITOR: W. RICHARD GREEN, MD