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Show Journal of Clillical N" lIro- ophthalmology 12( 3): 207- 209. 1992. Myxoma Mix- Up A Case Report R. H. Taylor, F. R. C. S., F. C. Ophth. and J. Deutsch F. R. C. S., F. C. Ophth. We present a 63- year- old lady who had atrial myxoma. The diagnostic difficulties distinguishing this from giant cell arteritis are highlighted. In particular, both conditions cause choroidal and retinal infarcts, anterior ischaemic optic neuropathy. with raised acute phase reactants. The authors stress the importance of continued ophthalmoscopy as the fundal changes become more apparent. Key Words: Atrial myxoma- Giant cell arteritisDiagnosis. From Birmingham and Midland Eye Hospital. Birmingham. England. Address correspondence and reprint requests to Mr. J. Deutsch. Senior Registrar. Birmingham & Midland Eye Hospital. Church Street. Birmingham B3 2NS, England. 207 '\:. 1992 Raven Press, Ltd., New York Atrial myxoma can present in one or a combination of three ways: ( a) obstruction to cardiac flow, ( b) embolic phenomenona, 50% of which are to the brain, and ( c) systemic symptoms and signs, including fatigue, fever, weight loss, and skin rashes ( 1). Investigations may show an elevated erythrocyte sedimentation rate, elevated serum globulins, and reduced serum complement levels. We report a case of atrial myxoma, which was initially mistaken for giant cell arteritis. CASE REPORT A 63- year- old lady collapsed after swimming. On presentation she was unconscious, had a rightsided hemiplegia, and had facial palsy. Both plantar responses were extensor. The left pupil reaction was reduced and the optic disk on this side was pale. No cardiovascular abnormality was detected. Her only previous medical history was migraine for which she received Migril. The following investigations were normal: full blood count. biochemical profile, 12- 1ead electrocardiogram, and chest radiograph. The erythrocyte sedimentation rate was 53 mm/ h. On recovery to consciousness 5 days later, she reported reduced vision in the left eye. She also reported a headache which had predated the collapse by 1 week. This was associated with a 2- week history of malaise and fatigue. On examination the acuity of the right eye was 20/ 20. This eye was normal in all respects. The left eye perceived light only and had an afferent pupillary defect. The left optic disk was pale and swollen, and there was loss of retinal transparency most marked in the macular area and in a wedgeshaped area nasal to the optic disk. The fundus picture was consistent with anterior ischaemic op- 208 R. H. TAYLOR AND J. DEUTSCH tic neuropathy and short ciliary and central retinal artery occlusion. Cranial computerized tomography identified an infarct in the internal capsule. A temporal artery biopsy showed luminal narrowing due to intimal fibroelastic proliferation of the lumen. There was no inflammatory cell infiltrate and no giant cells were seen. A tentative diagnosis of giant cell arteritis was made and oral prednisolone, 60 mg/ day was commenced. The headache improved, but the vision in the left eye remained severely reduced. Six months following initial presentation she suffered a la- minute episode of loss of visiol' in her right eye. This was described as a sea of black filling up from the bottom of her vision. Examination of the eye failed to reveal any abnormality. The erythrocyte sedimentation rate was now 32 mm/ h. Following this episode, an echocardiogram was undertaken, revealing an atrial myxoma, which was successfully resected 1 week later. Following this procedure, steroid therapy was tailed off. The erythrocyte sedimentation rate has remained low. At routine follow- up, 2 months after surgery, FIG. 1. Fundus photographs of the left eye at presentation ( above), showing a pale swollen optic disk, retinal vascular occlusion and an area of choroidal infarct nasal to the disk. Four months later ( below) the choroidal infarct. demonstrates a wedgeshaped zone of pigment epithelial atrophy and hyperplgmentation. MYXOMA MIX- UP 209 ophthalmoscopy of the affected was markedly different. There was evidence of central retinal artery occlusion, with retinal arterioles occluded and several choroidal infarcts ( choroidal pigmentation seen in the fundus photograph), in addition to anterior ischaemic optic neuropathy. DISCUSSION Duke Elder ( 2) reminds us that the infrequency of central retinal artery emboli from cardiac sources is due to two right- angle bends, one as the artery leaves the carotid, and the other as the central retinal artery leaves the ophthalmic artery. Ophthalmic complications of atrial myxoma are rare, with 11 reported cases of central retinal artery occlusion. Choroidal infarcts were also noted in two of these cases ( 3), indicating the presence of multiple emboli, characteristic of myxoma. Our patient exhibited the three major diagnostic features for giant cell arteritis ( age greater than 55, positive response to steroid within 48 hours, history longer than 2 weeks) and, in the absence of a positive temporal artery biopsy, three of the minor features ( systemic symptoms) as classified by Ellis and Raison ( 4). The occurrence of central retinal artery occlusion, ischaemic optic neuropathy, and choroidal infarct in the same eye is especially suspicious of atrial myxoma, but has also been seen in giant cell arteritis ( 3). There is considerable symptomatic overlap between atrial myxoma and giant cell arteritis. Both cause malaise and fatigue and a high erythrocyte sedimentation rate. Both cause neurological disorders by their effect on the vascular system with few specific clinical signs. Both can cause visual loss. This patient caused considerable diagnostic confusion, her age being at the upper end of one condition and the lower end of the other. Ophthalmoscopic examination is important. Atrial myxoma usually causes a central retinal artery occlusion, giant cell arteritis anterior ischaemic optic neuropathy. The importance of continued ophthalmoscopy is stressed as possible coexisting choroidal infarcts become more prominent. Acknowledgments: We are grateful to Professor A. R. Fielder and Dr. R. A. Stockley for their advice and for allowing us to report this case. REFERENCES 1. Yufe R, Karpati G, Carpenter S. Cardiac myxoma: a diagnostic challenge to the neurologist. Neurology 1986; 26: 1060- 5 2. Duke Elder 5, Dobree J. Diseases of the retina. In: Duke Elder, S, ed. System of Ophthalmology. Vol 10. London: Kimpton, 1967: 83. 3. Cogan D, Wray S. Vascular occlusions in the eye from cardiac myxomas. Am I Ophtha/ mo/ 1975; 80: 396- 403. 4. Ellis M, Ralston S. The ESR in the diagnosis and management of the polymyalgia/ giant cell arteritis syndrome. Ann Rheum Dis 1983; 42: 168-- 70. JCiln Neuro- ophthalmol, Vol. 12, No. 3. 1992 |
