RETINAL ARTERIOLAR MACROANEURYSMS AND CENTRAL RETINAL VEIN OCCLUSION IN A PATIENT WITH COMPLETE PULMONARY ATRESIA Maria Elena Gregory, MRCOphth,* Kim Bibby, FRCS, FRCOphth†
Purpose: Retinopathy in cyanotic heart disease arises due to hypoxia and polycythaemia. We report the development of central retinal vein occlusion, and two arteriolar macroaneurysms in a normotensive patient with polycythaemia secondary to complete pulmonary atresia. Method: A 44-year-old woman with polycythaemia secondary to complete pulmonary atresia, presented with unilateral reduced vision of hand movements in the right eye. Ocular assessment and hematological investigations were performed. Management was conservative. Results: Visual acuity was hand movements in the right eye, 6/5 in the left eye. Fundus assessment found right central retinal vein occlusion, and vessel tortuosity in the left eye. Hematological tests revealed an elevated red blood count, hemoglobin, heamatocrit, but normal plasma viscosity. Three months later, two arteriolar macroaneurysms developed in the left superotemporal arcade. Vision of 6/5 was retained in the left eye, hence no laser treatment was given. Subsequent follow-up revealed gradual involution of both macroaneurysms. Conclusion: Multiple retinal arteriolar macroaneurysms may occur in association with cyanotic heart disease such as pulmonary atresia. They appear to involute spontaneously, hence conservative management is appropriate. RETINAL CASES & BRIEF REPORTS 4:109 –111, 2010
From the *Department of Ophthalmology, Gartnavel General Hospital, Glasgow, Scotland; and †Department of Ophthalmology, Leicester Royal Infirmary, Leicester, United Kingdom.
atresia, developed a central retinal vein occlusion in one eye, and two arteriolar macroaneurysms in the other. Case Report
R
etinopathy in cyanotic heart disease arises as a result of hypoxia and polycythaemia, with the severity closely related to decreased arterial oxygen saturation and hematocrit.1 A normotensive patient with polycythaemia secondary to complete pulmonary
A 44-year-old woman nonsmoker presented with unilateral reduced vision. Visual acuity was hand movements in the right eye, 6/5 in the left eye. She had dilated episcleral vessels in both eyes. Fundoscopy revealed right central retinal vein occlusion with vitreous hemorrhage, and dilated, tortuous left retinal vessels (Figure 1A). She had a plethoric complexion, and had polycythaemia secondary to complete pulmonary atresia with ventricular septal defect and multiple pulmonary collateral arteries. Her resting oxygen saturation was 74%, blood pressure was 155/60 mmHg with previous readings ranging 115 to 140 mmHg systolic, and 55 to 60 mmHg diastolic. Hematological tests revealed an elevated red blood count (7.79 ⫻ 1012/L), hemoglobin
From the Department of Ophthalmology, Leicester Royal Infirmary, Leicester, LE1 5WW, United Kingdom. Reprint requests: Dr. Maria Elena Gregory, Ophthalmology Department, Gartnavel General Hospital, Great Western Road, Glasgow G12 0YN; e-mail: [email protected]
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Fig. 1. A, Fundus photograph of the right eye showing ischemic central retinal vein occlusion. B, Left fundus photograph showing vascular tortuosity, fibrosed arteriolar macroaneurysm in the superotemporal arcade (top black arrow), a more distal aneurysm (white arrow), and an inferotemporal blot hemorrhage (bottom black arrow). C, Left fundus photograph, and D, late fluorescein angiogram showing the macroaneurysms arising from the superotemporal arcade in the left eye.
level (21 g/dL) and haematocrit (0.685 L/L). Plasma viscosity (1.62 cP), lipid profile and clotting profile were normal, lupus anticoagulant negative. Cerebral magnetic resonance imaging detected white matter ischemic changes. No pulmonary arteries were delineated on previous cardiac catheterization. Echocardiography confirmed a ventricular septal defect over-riding the aorta, mild aortic regurgitation, left aortic arch and absence of the main pulmonary artery. She had not undergone any previous surgery or palliative treatment and was currently taking 75 mg acetylsalicylic acid daily. Despite extensive pan-retinal photocoagulation her right eye lost vision due to rubeotic glaucoma. Three months later, 2 arterial macroaneurysms were detected in the left superotemporal arcade, the proximal aneurysm being surrounded by hard exudates (Figure 1B). Choroidal filling was first seen on fundus fluorescein angiography at 26 seconds, complete at 55 seconds, retinal arterioles at 24 seconds, and laminar venous flow seen at 30 seconds with complete venous filling at 1 minute 2 seconds. Within 10 months, the proximal macroaneurysm fibrosed, but the more distal one was surrounded by subretinal hemorrhage and a dense ring of exudates extending into the macular area (Figure 1, C and D). Vision of 6/5 was retained, and laser treatment was deferred. During close follow-up, both macroaneurysms involuted gradually and spontaneously obviating active intervention (Figure 2).
aemia vera include a dark fundus resulting from dilatation of the choriocapillaris and swelling of the choroid, and dilated and tortuous veins. Retinal and vitreous hemorrhages, and central retinal vein thrombosis, may ensue.1,2 Retinal arteriolar macroaneurysms occur primarily in elderly people, (women more than men), with hypertensive and generalized arteriosclerotic vascular disease3– 6 and normally resolve spontaneously. No
Discussion Pulmonary atresia with ventricular septal defect results in reduced oxygenation and subsequent polycythemia. Typical ophthalmologic changes in polycyth-
Fig. 2. The Figure shows gradual involution of both arteriolar macroaneurysms.
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RETINAL CHANGES IN PULMONARY ATRESIA
definite ocular risk factors for arterial macroaneurysms have been identified; however, macroaneurysms have been reported in association with several retinal vasculature diseases, including retinal telangiectasia, arterial emboli, and retinal vein occlusion.4 – 6 Arteriolar macroaneurysms in polycythaemia may arise through different mechanisms. Slowing of circulation with chronic venous stasis as a result of venous occlusion, may lead to local arteriolar thrombosis, endothelial damage, and aneurysm formation.4,5 Alternatively, local turbulent flow and arteriolar wall weakness may lead to a macroaneurysm forming at the origin of dilated collateral vessels arising secondary to a retinal branch vein occlusion.4 Hypertension, which is a common consequence of polycythaemia, exacerbates the risk.2 Increased circulatory volume may lead to arterial thromboembolisation to the eye facilitated by the presence of a ventricular septal defect, leading to focal arteriolar wall damage and subsequent dilatation.2,3 In a previous report, a retinal arteriolar macroaneurysm developed adjacent to an embolus in a patient with congenital heart disease in the absence of polycythaemia.3 No arteriolar emboli or venous occlusions were detected in our patient’s left eye.
To our knowledge this is the first case demonstrating the formation of arteriolar macroaneurysms as a result of secondary polycythaemia in cyanotic heart disease such as pulmonary atresia. They appear to involute spontaneously, as with idiopathic macroaneurysms, and conservative management under close observation may be appropriate. Key words: pulmonary atresia, polycythaemia, retinal arteriolar macroaneurysms, central retinal vein occlusion. References 1. 2. 3.
4.
5. 6.
Peterson RA, Rosenthal A. Retinopathy and papilledema in cyanotic congenital heart disease. Pediatrics 1972;49:243–249. Nagy G, Gat G, Racz M, Mailath L. The optic fundus in polycythaemia vera. Acta Med Acad Sci Hung 1969;26:351–355. Abu El-Asrar AM, Awad A, Tabbara KF. Retinal arterial macroaneurysm in a patient with congenital heart disease. Br J Ophthalmol 1993;77:606 – 607. Panton RW, Goldberg MF, Farber MD. Retinal arterial macroaneurysms: risk factors and natural history. Br J Ophthalmol 1990;74:595– 600. Lewis RA, Norton EW, Gass JD. Acquired arterial macroaneurysms of the retina. Br J Ophthalmol 1976;60:21–30. Palestine AG, Robertson DM, Goldstein BG. Macroaneurysms of the retinal arteries. Am J Ophthalmol 1982;93:164 –171.
Purpose: Retinopathy in cyanotic heart disease arises due to hypoxia and polycythaemia. We report the development of central retinal vein occlusion, and two arteriolar macroaneurysms in a normotensive patient with polycythaemia secondary to complete pulmonary atresia. Method: A 44-year-old woman with polycythaemia secondary to complete pulmonary atresia, presented with unilateral reduced vision of hand movements in the right eye. Ocular assessment and hematological investigations were performed. Management was conservative. Results: Visual acuity was hand movements in the right eye, 6/5 in the left eye. Fundus assessment found right central retinal vein occlusion, and vessel tortuosity in the left eye. Hematological tests revealed an elevated red blood count, hemoglobin, heamatocrit, but normal plasma viscosity. Three months later, two arteriolar macroaneurysms developed in the left superotemporal arcade. Vision of 6/5 was retained in the left eye, hence no laser treatment was given. Subsequent follow-up revealed gradual involution of both macroaneurysms. Conclusion: Multiple retinal arteriolar macroaneurysms may occur in association with cyanotic heart disease such as pulmonary atresia. They appear to involute spontaneously, hence conservative management is appropriate. RETINAL CASES & BRIEF REPORTS 4:109 –111, 2010
From the *Department of Ophthalmology, Gartnavel General Hospital, Glasgow, Scotland; and †Department of Ophthalmology, Leicester Royal Infirmary, Leicester, United Kingdom.
atresia, developed a central retinal vein occlusion in one eye, and two arteriolar macroaneurysms in the other. Case Report
R
etinopathy in cyanotic heart disease arises as a result of hypoxia and polycythaemia, with the severity closely related to decreased arterial oxygen saturation and hematocrit.1 A normotensive patient with polycythaemia secondary to complete pulmonary
A 44-year-old woman nonsmoker presented with unilateral reduced vision. Visual acuity was hand movements in the right eye, 6/5 in the left eye. She had dilated episcleral vessels in both eyes. Fundoscopy revealed right central retinal vein occlusion with vitreous hemorrhage, and dilated, tortuous left retinal vessels (Figure 1A). She had a plethoric complexion, and had polycythaemia secondary to complete pulmonary atresia with ventricular septal defect and multiple pulmonary collateral arteries. Her resting oxygen saturation was 74%, blood pressure was 155/60 mmHg with previous readings ranging 115 to 140 mmHg systolic, and 55 to 60 mmHg diastolic. Hematological tests revealed an elevated red blood count (7.79 ⫻ 1012/L), hemoglobin
From the Department of Ophthalmology, Leicester Royal Infirmary, Leicester, LE1 5WW, United Kingdom. Reprint requests: Dr. Maria Elena Gregory, Ophthalmology Department, Gartnavel General Hospital, Great Western Road, Glasgow G12 0YN; e-mail: [email protected]
109
RETINAL CASES & BRIEF REPORTSℜ
110
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2010
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VOLUME 4
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NUMBER 2
Fig. 1. A, Fundus photograph of the right eye showing ischemic central retinal vein occlusion. B, Left fundus photograph showing vascular tortuosity, fibrosed arteriolar macroaneurysm in the superotemporal arcade (top black arrow), a more distal aneurysm (white arrow), and an inferotemporal blot hemorrhage (bottom black arrow). C, Left fundus photograph, and D, late fluorescein angiogram showing the macroaneurysms arising from the superotemporal arcade in the left eye.
level (21 g/dL) and haematocrit (0.685 L/L). Plasma viscosity (1.62 cP), lipid profile and clotting profile were normal, lupus anticoagulant negative. Cerebral magnetic resonance imaging detected white matter ischemic changes. No pulmonary arteries were delineated on previous cardiac catheterization. Echocardiography confirmed a ventricular septal defect over-riding the aorta, mild aortic regurgitation, left aortic arch and absence of the main pulmonary artery. She had not undergone any previous surgery or palliative treatment and was currently taking 75 mg acetylsalicylic acid daily. Despite extensive pan-retinal photocoagulation her right eye lost vision due to rubeotic glaucoma. Three months later, 2 arterial macroaneurysms were detected in the left superotemporal arcade, the proximal aneurysm being surrounded by hard exudates (Figure 1B). Choroidal filling was first seen on fundus fluorescein angiography at 26 seconds, complete at 55 seconds, retinal arterioles at 24 seconds, and laminar venous flow seen at 30 seconds with complete venous filling at 1 minute 2 seconds. Within 10 months, the proximal macroaneurysm fibrosed, but the more distal one was surrounded by subretinal hemorrhage and a dense ring of exudates extending into the macular area (Figure 1, C and D). Vision of 6/5 was retained, and laser treatment was deferred. During close follow-up, both macroaneurysms involuted gradually and spontaneously obviating active intervention (Figure 2).
aemia vera include a dark fundus resulting from dilatation of the choriocapillaris and swelling of the choroid, and dilated and tortuous veins. Retinal and vitreous hemorrhages, and central retinal vein thrombosis, may ensue.1,2 Retinal arteriolar macroaneurysms occur primarily in elderly people, (women more than men), with hypertensive and generalized arteriosclerotic vascular disease3– 6 and normally resolve spontaneously. No
Discussion Pulmonary atresia with ventricular septal defect results in reduced oxygenation and subsequent polycythemia. Typical ophthalmologic changes in polycyth-
Fig. 2. The Figure shows gradual involution of both arteriolar macroaneurysms.
111
RETINAL CHANGES IN PULMONARY ATRESIA
definite ocular risk factors for arterial macroaneurysms have been identified; however, macroaneurysms have been reported in association with several retinal vasculature diseases, including retinal telangiectasia, arterial emboli, and retinal vein occlusion.4 – 6 Arteriolar macroaneurysms in polycythaemia may arise through different mechanisms. Slowing of circulation with chronic venous stasis as a result of venous occlusion, may lead to local arteriolar thrombosis, endothelial damage, and aneurysm formation.4,5 Alternatively, local turbulent flow and arteriolar wall weakness may lead to a macroaneurysm forming at the origin of dilated collateral vessels arising secondary to a retinal branch vein occlusion.4 Hypertension, which is a common consequence of polycythaemia, exacerbates the risk.2 Increased circulatory volume may lead to arterial thromboembolisation to the eye facilitated by the presence of a ventricular septal defect, leading to focal arteriolar wall damage and subsequent dilatation.2,3 In a previous report, a retinal arteriolar macroaneurysm developed adjacent to an embolus in a patient with congenital heart disease in the absence of polycythaemia.3 No arteriolar emboli or venous occlusions were detected in our patient’s left eye.
To our knowledge this is the first case demonstrating the formation of arteriolar macroaneurysms as a result of secondary polycythaemia in cyanotic heart disease such as pulmonary atresia. They appear to involute spontaneously, as with idiopathic macroaneurysms, and conservative management under close observation may be appropriate. Key words: pulmonary atresia, polycythaemia, retinal arteriolar macroaneurysms, central retinal vein occlusion. References 1. 2. 3.
4.
5. 6.
Peterson RA, Rosenthal A. Retinopathy and papilledema in cyanotic congenital heart disease. Pediatrics 1972;49:243–249. Nagy G, Gat G, Racz M, Mailath L. The optic fundus in polycythaemia vera. Acta Med Acad Sci Hung 1969;26:351–355. Abu El-Asrar AM, Awad A, Tabbara KF. Retinal arterial macroaneurysm in a patient with congenital heart disease. Br J Ophthalmol 1993;77:606 – 607. Panton RW, Goldberg MF, Farber MD. Retinal arterial macroaneurysms: risk factors and natural history. Br J Ophthalmol 1990;74:595– 600. Lewis RA, Norton EW, Gass JD. Acquired arterial macroaneurysms of the retina. Br J Ophthalmol 1976;60:21–30. Palestine AG, Robertson DM, Goldstein BG. Macroaneurysms of the retinal arteries. Am J Ophthalmol 1982;93:164 –171.
