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Bilateral central retinal vein occlusion in eisenmenger syndrome
tomy with mitomycin C, which controlled his pressure between 7 and 9 mm Hg. Retinal venous occlusive disease in leukemia is believed to result from increased serum viscosity, secondary to leukocytosis or thrombocytosis.2 We document the lack of hyperviscosity (in fact, low viscosity) in this patient, whose white blood cell count remained normal. Oncologists maintain that in any malignancy, in general, abnormalities in platelet activity, anticoagulant proteins, or the coagulation cascade may account for observed thrombotic complications.3 In chronic myelogenous leukemia, decreased antithrombin III and plasminogen activity may result in increased thrombosis, which have been postulated to explain central nervous system thrombosis in the absence of leukocytosis.4 Anticardiolipin phospholipid autoantibodies have been associated in rheumatologic disease without leukemia to occur with diffuse retinal venous occlusive disease often with neovascularization.5 This case is a dramatic occurrence of simultaneous bilateral central retinal vein occlusion associated with leukemia and anticardiolipin autoantibodies. These antibodies, believed to be produced either directly by the leukemic tumor cells or by the immune system directed against leukemic cell phospholipid antigen, have been associated with increased thrombosis in leukemic patients, through interactions with platelet or endothelial phospholipids or inhibition of endothelial anticoagulant activity of protein C or prostacyclin.6 This case suggests an additional mechanism other than hyperviscosity for bilateral central retinal vein occlusions in leukemic patients.
PURPOSE:
To report a case of bilateral central retinal vein occlusion in a patient with Eisenmenger syndrome. METHODS: Case report. A 60-year-old woman with Eisenmenger syndrome secondary to a congenital ventricular septal defect presented with a 2-week history of decreased vision in both eyes. RESULTS: Ophthalmic examination revealed bilateral central retinal vein occlusion. Laboratory findings included hematocrit of 50.3% and pO2 of 52 mm Hg while on O2 5 L/min per nasal cannula, despite having undergone phlebotomy 2 weeks earlier. CONCLUSION: Chronic hypoxia caused by Eisenmenger syndrome may result in polycythemia with resultant hyperviscosity and bilateral central retinal vein occlusion. (Am J Ophthalmol 2001;132:268 –269. © 2001 by Elsevier Science Inc. All rights reserved.)
P
REFERENCES
1. Guyer RD, Tiedeman J, Yannuzzi LA, et al. Interferonassociated retinopathy. Arch Ophthalmol 1993;111:350 –356. 2. Schachat AP, Dhaliwal RS. Leukemias and lymphomas. In: Ryan SJ, editor. Retina. 2nd ed. St. Louis: Mosby-Year Book, Inc., 1994:873– 890. 3. Nand S, Messmore H. Hemostasis in malignancy. Am J Hematol 1990;35:45–55. 4. Gralnick HR. Hemostasis and thrombosis in acute and chronic leukemia. In: Henderson ES and Lister TA, editors. Willaim Damshek and Frederick Gunz’s leukemia. 5th ed. Philadelphia: W.B. Saunders Company, 1990:711–731. 5. Cobo-Soriano R, Sanchez-Ramon S, Aparicio MJ, et al. Antiphospholipid antibodies and retinal thrombosis in patients without risk factors: a prospective case-control study. Am J Ophthalmol 1999;128:725–732. 6. Zuckerman E, Toubi E, Golan TD, et al. Increased thromboembolic incidence in anti-cardiolipin-positive patients with malignancy. Br J Cancer 1995;72:447– 451.
Accepted for publication Feb 12, 2001. From the Kresge Eye Institute, Wayne State University School of Medicine, Detroit, Michigan. Inquiries to Dean Eliott, MD, Kresge Eye Institute, Wayne State University School of Medicine, Detroit, MI 48201; fax: (313) 577-2905; e-mail: [email protected]
Bilateral Central Retinal Vein Occlusion in Eisenmenger Syndrome Natalia Rodriguez and Dean Eliott, MD 268
AMERICAN JOURNAL
ATIENTS WITH EISENMENGER SYNDROME HAVE CON-
genital cardiac defects (most commonly, atrial septal defect, ventricular septal defect, or patent ductus arteriosus) with left-to-right shunting of blood. The resultant increased pulmonary artery pressure creates pulmonary vascular fibrosis and occlusion of pulmonary capillary beds. This increased resistance eventually reverses the intracardiac shunting of blood from left-to-right to right-to-left, thus bypassing the pulmonary circulation. As blood continues to bypass the lungs, the patient becomes hypoxic and develops erythrocytosis and resultant hyperviscosity. The case presented herein describes an adult patient with Eisenmenger syndrome and secondary polycythemia who developed bilateral visual loss secondary to retinal venous occlusive disease. A 60-year-old Caucasian woman with Eisenmenger syndrome secondary to a large congenital ventricular septal defect presented with a 2-week history of decreased vision in both eyes. Medical history was notable for prominent pulmonary hypertension and severe shortness of breath, but no symptomatic coronary artery disease, dizziness, or syncope. The patient was oxygen dependent and unable to ambulate secondary to dyspnea. Medications included coumadin 2.5 mg daily. A 25 pack-year history of smoking existed. The patient developed secondary erythrocytosis with hematocrit levels between 56% and 62%, and 2 weeks before presentation, she had been treated with phlebotomy. Ophthalmic examination revealed corrected visual acuity of 20/100 in both eyes, normal intraocular pressure, no evidence of iris neovascularization, and bilateral mild nuclear sclerotic cataracts. Funduscopy demonstrated intraretinal hemorrhages, cotton-wool spots, and macular
OF
OPHTHALMOLOGY
AUGUST 2001
FIGURE 2. Digital erythema and severe clubbing.
FIGURE 1. Ophthalmoscopic appearance of intraretinal hemorrhages, cotton wool spots, and macular edema (A, right eye; B, left eye).
edema in both eyes (Figure 1). Fluorescein angiography showed delayed venous filling and late cystoid leakage in the macula. Physical examination revealed a harsh III/VI systolic murmur and a sternal heave. Extremities showed digital erythema and severe clubbing (Figure 2). Chest x-ray demonstrated cardiomegaly and pleural effusions. Electrocardiogram showed right axis deviation and right ventricular hypertrophy. Laboratory examination revealed hemoglobin 15.9 g/dl, hematocrit 50.3%, and pO2 52 mm Hg while on O2 5 L/min per nasal cannula. The patient underwent phlebotomy, with stabilization of vision and a reduction in hematocrit to 38%. Krarup described a patient with multiple cardiac defects and Eisenmenger syndrome who developed bilateral rubeosis iridis with spontaneous hyphemas and minimal retinal findings.1 An additional report by Harino and associates described two patients with atrial septal defects and Eisenmenger syndrome, whose findings included microaneurysms, blot hemorrhages, and capillary dilation in the temporal peripheral retina.2 One of these patients develVOL. 132, NO. 2
oped bilateral rubeosis iridis. Our patient developed bilateral visual loss secondary to central retinal vein occlusion, and we are unaware of any reports describing this association based on a computer search using MEDLINE. In adults, the most common causes of cyanotic congenital heart disease are tetrology of Fallot and Eisenmenger syndrome.3 Patients with cyanotic congenital heart disease have arterial oxygen desaturation resulting from the shunting of systemic venous blood to the arterial circulation. The hemostatic changes associated with Eisenmenger syndrome may lead to thromboembolic events, cerebrovascular complications, or the hyperviscosity syndrome.4 As erythrocytosis caused by arterial desaturation develops in patients with Eisenmenger syndrome, the blood viscosity increases commensurately, and blood flow and oxygen transport eventually decrease. Retinal venous occlusive disease is a known sequela of increased blood viscosity, and it has been documented in patients with multiple myeloma, Waldenstrom macroglobulinemia, cryoglobulinemia, leukemia/lymphoma, and primary and secondary polycythemia.5 As demonstrated by our patient, individuals with Eisenmenger syndrome and associated secondary polycythemia may develop bilateral retinal venous occlusive disease.
REFERENCES
1. Krarup JC. Atypical rubeosis iridis in congenital cyanotic heart disease. Report of a case with microhaemangiomas at the pupillary margin causing spontaneous hyphaemas. Acta Ophthalmol (Copenhagen) 1977;55:581. 2. Harino S, Motokura M, Nishikawa N, et al. Chronic ocular ischemia associated with Eisenmenger’s syndrome. Am J Ophthalmol 1994;117:302–307. 3. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. N Engl J Med 2000;342:334 –342. 4. Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med 1988;128: 745–755. 5. Lowenstein JI. Retinopathy associated with blood anomalies. In: Albert DM, Jakobiec FA, editors. Principles and practice of ophthalmology. Philadelphia: W. B. Saunders, 1994:995– 1000.
BRIEF REPORTS
269
PURPOSE:
To report a case of bilateral central retinal vein occlusion in a patient with Eisenmenger syndrome. METHODS: Case report. A 60-year-old woman with Eisenmenger syndrome secondary to a congenital ventricular septal defect presented with a 2-week history of decreased vision in both eyes. RESULTS: Ophthalmic examination revealed bilateral central retinal vein occlusion. Laboratory findings included hematocrit of 50.3% and pO2 of 52 mm Hg while on O2 5 L/min per nasal cannula, despite having undergone phlebotomy 2 weeks earlier. CONCLUSION: Chronic hypoxia caused by Eisenmenger syndrome may result in polycythemia with resultant hyperviscosity and bilateral central retinal vein occlusion. (Am J Ophthalmol 2001;132:268 –269. © 2001 by Elsevier Science Inc. All rights reserved.)
P
REFERENCES
1. Guyer RD, Tiedeman J, Yannuzzi LA, et al. Interferonassociated retinopathy. Arch Ophthalmol 1993;111:350 –356. 2. Schachat AP, Dhaliwal RS. Leukemias and lymphomas. In: Ryan SJ, editor. Retina. 2nd ed. St. Louis: Mosby-Year Book, Inc., 1994:873– 890. 3. Nand S, Messmore H. Hemostasis in malignancy. Am J Hematol 1990;35:45–55. 4. Gralnick HR. Hemostasis and thrombosis in acute and chronic leukemia. In: Henderson ES and Lister TA, editors. Willaim Damshek and Frederick Gunz’s leukemia. 5th ed. Philadelphia: W.B. Saunders Company, 1990:711–731. 5. Cobo-Soriano R, Sanchez-Ramon S, Aparicio MJ, et al. Antiphospholipid antibodies and retinal thrombosis in patients without risk factors: a prospective case-control study. Am J Ophthalmol 1999;128:725–732. 6. Zuckerman E, Toubi E, Golan TD, et al. Increased thromboembolic incidence in anti-cardiolipin-positive patients with malignancy. Br J Cancer 1995;72:447– 451.
Accepted for publication Feb 12, 2001. From the Kresge Eye Institute, Wayne State University School of Medicine, Detroit, Michigan. Inquiries to Dean Eliott, MD, Kresge Eye Institute, Wayne State University School of Medicine, Detroit, MI 48201; fax: (313) 577-2905; e-mail: [email protected]
Bilateral Central Retinal Vein Occlusion in Eisenmenger Syndrome Natalia Rodriguez and Dean Eliott, MD 268
AMERICAN JOURNAL
ATIENTS WITH EISENMENGER SYNDROME HAVE CON-
genital cardiac defects (most commonly, atrial septal defect, ventricular septal defect, or patent ductus arteriosus) with left-to-right shunting of blood. The resultant increased pulmonary artery pressure creates pulmonary vascular fibrosis and occlusion of pulmonary capillary beds. This increased resistance eventually reverses the intracardiac shunting of blood from left-to-right to right-to-left, thus bypassing the pulmonary circulation. As blood continues to bypass the lungs, the patient becomes hypoxic and develops erythrocytosis and resultant hyperviscosity. The case presented herein describes an adult patient with Eisenmenger syndrome and secondary polycythemia who developed bilateral visual loss secondary to retinal venous occlusive disease. A 60-year-old Caucasian woman with Eisenmenger syndrome secondary to a large congenital ventricular septal defect presented with a 2-week history of decreased vision in both eyes. Medical history was notable for prominent pulmonary hypertension and severe shortness of breath, but no symptomatic coronary artery disease, dizziness, or syncope. The patient was oxygen dependent and unable to ambulate secondary to dyspnea. Medications included coumadin 2.5 mg daily. A 25 pack-year history of smoking existed. The patient developed secondary erythrocytosis with hematocrit levels between 56% and 62%, and 2 weeks before presentation, she had been treated with phlebotomy. Ophthalmic examination revealed corrected visual acuity of 20/100 in both eyes, normal intraocular pressure, no evidence of iris neovascularization, and bilateral mild nuclear sclerotic cataracts. Funduscopy demonstrated intraretinal hemorrhages, cotton-wool spots, and macular
OF
OPHTHALMOLOGY
AUGUST 2001
FIGURE 2. Digital erythema and severe clubbing.
FIGURE 1. Ophthalmoscopic appearance of intraretinal hemorrhages, cotton wool spots, and macular edema (A, right eye; B, left eye).
edema in both eyes (Figure 1). Fluorescein angiography showed delayed venous filling and late cystoid leakage in the macula. Physical examination revealed a harsh III/VI systolic murmur and a sternal heave. Extremities showed digital erythema and severe clubbing (Figure 2). Chest x-ray demonstrated cardiomegaly and pleural effusions. Electrocardiogram showed right axis deviation and right ventricular hypertrophy. Laboratory examination revealed hemoglobin 15.9 g/dl, hematocrit 50.3%, and pO2 52 mm Hg while on O2 5 L/min per nasal cannula. The patient underwent phlebotomy, with stabilization of vision and a reduction in hematocrit to 38%. Krarup described a patient with multiple cardiac defects and Eisenmenger syndrome who developed bilateral rubeosis iridis with spontaneous hyphemas and minimal retinal findings.1 An additional report by Harino and associates described two patients with atrial septal defects and Eisenmenger syndrome, whose findings included microaneurysms, blot hemorrhages, and capillary dilation in the temporal peripheral retina.2 One of these patients develVOL. 132, NO. 2
oped bilateral rubeosis iridis. Our patient developed bilateral visual loss secondary to central retinal vein occlusion, and we are unaware of any reports describing this association based on a computer search using MEDLINE. In adults, the most common causes of cyanotic congenital heart disease are tetrology of Fallot and Eisenmenger syndrome.3 Patients with cyanotic congenital heart disease have arterial oxygen desaturation resulting from the shunting of systemic venous blood to the arterial circulation. The hemostatic changes associated with Eisenmenger syndrome may lead to thromboembolic events, cerebrovascular complications, or the hyperviscosity syndrome.4 As erythrocytosis caused by arterial desaturation develops in patients with Eisenmenger syndrome, the blood viscosity increases commensurately, and blood flow and oxygen transport eventually decrease. Retinal venous occlusive disease is a known sequela of increased blood viscosity, and it has been documented in patients with multiple myeloma, Waldenstrom macroglobulinemia, cryoglobulinemia, leukemia/lymphoma, and primary and secondary polycythemia.5 As demonstrated by our patient, individuals with Eisenmenger syndrome and associated secondary polycythemia may develop bilateral retinal venous occlusive disease.
REFERENCES
1. Krarup JC. Atypical rubeosis iridis in congenital cyanotic heart disease. Report of a case with microhaemangiomas at the pupillary margin causing spontaneous hyphaemas. Acta Ophthalmol (Copenhagen) 1977;55:581. 2. Harino S, Motokura M, Nishikawa N, et al. Chronic ocular ischemia associated with Eisenmenger’s syndrome. Am J Ophthalmol 1994;117:302–307. 3. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. N Engl J Med 2000;342:334 –342. 4. Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med 1988;128: 745–755. 5. Lowenstein JI. Retinopathy associated with blood anomalies. In: Albert DM, Jakobiec FA, editors. Principles and practice of ophthalmology. Philadelphia: W. B. Saunders, 1994:995– 1000.
BRIEF REPORTS
269