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AMERICAN JOURNAL OF OPHTHALMOLOGY
November, 1992
Development of a Retinal Artery Macroaneurysm at the Site of a Previously Detected Retinal Artery Embolus Robert A. Wiznia, M.D. Department of Ophthalmology and Visual Science, Yale University School of Medicine. Inquiries to Robert A. Wiznia, Μ.Ό., 850 Howard Ave., New Haven, CT 06519. Although the origin of acquired retinal artery macroaneurysms is not entirely certain, several conditions associated with focal arterial wall damage have been implicated. 1 Inherent weak nesses, such as arterial bifurcations and arteriovenous crossing sites, as well as general condi tions such as arteriosclerosis, aging, and hypertension have been noted in several se ries.1·2 Increased serum lipids, branch retinal vein occlusion, branch retinal artery occlu sion,2·3 and diabetes mellitus 3 are other diseases with an increased association. Emboli are frequently seen near macroaneu rysms.1·3 However, it is rare for an embolus to be noted initially in a retinal artery followed by development of a macroaneurysm at the same location. Arruga and Sanders 4 did not note such an occurrence in a series of 70 patients with retinal embolism. Two such cases have been described, one by Lewis, Norton, and Gass 1 and the other by Khalil and Lorenzetti. 5 I managed an additional case in which an embolus led to the development of a macroaneurysm at the same location in a previously normal cilioretinal artery. A 77-year-old white man was first examined by me because of age-related macular degener ation on April 22, 1986. Best-corrected visual acuity was R.E.: 20/400 and L.E.: 20/30. He had a hemorrhagic disciform lesion in the right macula and atrophie loss of retinal pigment epithelium in the left macula. Over the next 14 months, the disciform process reached an invo luted end stage with a best-corrected visual acuity of 20/200 in the right eye. His disease course remained stable until he was noted to have a small embolus in the cilioretinal artery, located at the inferonasal edge of the macula of the left eye on Jan. 7, 1991 (Fig. 1). Visual acuity in the left eye was 20/30. Duplex carotid ultrasonography disclosed mild bilateral calcific plaque formation in the com mon carotid bifurcation and origin of the inter nal carotid arteries without marked stenosis. On April 15, 1991, the patient noted blurred
Fig. 1 (Wiznia). Fluorescein angiogram delineates an embolus in the cilioretinal artery at the inferona sal edge of the macula in the left eye on Jan. 7, 1991. vision in his left eye, which was found to be caused by retinal hemorrhage associated with a retinal artery macroaneurysm (Fig. 2). Visual acuity was 20/70 in the left eye. By April 30, 1991, additional hemorrhage and edema were noted and visual acuity was 20/400 in the left
Fig. 2 (Wiznia). Retinal artery macroaneurysm is present at the previous site of the embolus as demon strated in the late venous-phase angiogram obtained on April 15, 1991.
Vol. 114, No. 5
Letters to The Journal
eye. The patient would not consent to laser treatment at any time during his treatment course. Unfortunately, further bleeding oc curred, which resulted in a dense vitreous hem orrhage. The left eye was monitored conservatively for several months, but showed no sign of clearing. Visual acuity in the right eye improved during this time to 20/70. On Aug. 28, 1991, the patient underwent pars plana vitrectomy in the left eye. The surgical procedure proceeded fa vorably, but marked preretinal fibrosis, retinal pigment epithelial atrophy, and metaplastic subretinal tissue were evident in the macula. Early preoperative cataract demonstrated pro gression that ultimately required extracapsular cataract and anterior chamber intraocular lens implantation, which were performed on Dec. 11, 1991. On Jan. 23, 1992, best-corrected visual acuity was R.E.: 20/40 and L.E.: 20/300. There was no clinical sign of hemorrhage in the left macula, but there was distortion caused by preretinal fibrosis and metaplastic subsensory retinal tis sue. Focal arterial wall damage is the likely pre cursor of retinal artery macroaneurysms. The rarity of emboli as the cause of this damage is evident from previous reports of macroaneu rysms. Some additional cases in which an embolus is noted near a macroaneurysm could have a similar origin. Palestine, Robertson, and Goldstein 3 noted that only one of ten cases had an embolus nearby. Thus, it is likely that most are not caused by embolie disease. Of course, most macroaneurysms are detected because of symptoms of loss in visual acuity. The appear ance of the retinal artery before the onset of the symptoms is not known in most cases, and the potential for an undetected embolus to have preceded the macroaneurysm cannot be exclud ed. Emboli have been demonstrated to be the definite cause of a retinal artery macroaneu rysm in three cases, including the present one. 1 · 5 The lack of macroaneurysms as a subse quent development in the large series of retinal artery emboli described by Arruga and Sand ers4 implies that most macroaneurysms are at tributable to other causes.
References 1. Lewis, R. A., Norton, E. W. D., and Gass, J. D. M.: Acquired arterial macroaneurysms of the retina. Br. J. Ophthalmol. 60:21, 1976.
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2. Cleary, P. E., Kohner, E. M„ Hamilton, A. M, and Bird, A. C: Retinal macroaneurysms. Br. J. Oph thalmol. 59:355, 1975. 3. Palestine, A. G., Robertson, D. M., and Gold stein, B. G.: Macroaneurysms of the retinal arteries. Am. J. Ophthalmol. 93:164, 1982. 4. Arruga, J., and Sanders, M.: Ophthalmologic findings in 70 patients with evidence of retinal embo lism. Ophthalmology 89:1336, 1989. 5. Khalil, M., and Lorenzetti, M. D.: Acquired reti nal macroaneurysms. Can. J. Ophthalmol. 14:163, 1979.
Nonbacterial Thrombotic Endocarditis and Cortical Blindness J a m e s A. R u s h , M . D . Department of Ophthalmology, University of South Florida College of Medicine. Presented at the IX International Neuro-Ophthalmological Symposium, Williamsburg, Virginia, June 30, 1992. Inquiries to James A. Rush, M.D., 508S Habana Ave., Suite 100, Tampa, Fl 33609. A 73-year-old man had a two-week history of stepwise painless progressive visual loss in both eyes. No focal nonvisual neurologic symp toms occurred; however, he experienced four weeks of headaches and two weeks of mild confusion. His medical history was unremarka ble. Visual acuity was R.E.: 20/200 and L.E.: counting fingers at 2 feet. He identified none of the ten City University color plates with either eye. Pupillomotor responses were brisk without afferent defect in both eyes. Results of the examination of the ocular fundi were normal bilaterally. Computed perimetry showed abso lute superior altitudinal field loss with involve ment of fixation, along with moderate genera lized depression inferiorly in both eyes. Results of a left superficial temporal artery biopsy and bilateral carotid ultrasonograms were normal. Cranial magnetic resonance images showed a lesion in the left occipital lobe of mixed signal, suggesting hemorrhage, and mild gyral en hancement of the right occipital cortex (Fig. 1). Progressive confusion and weight and appetite loss resulted in hospitalization and death 11 days later. A chest x-ray film disclosed a left upper lobe infiltrate found on autopsy study to be a mucin-secreting pulmonary adenocarcinoma. Nonbacterial thrombotic endocarditis was present on the aortic and mitral valves, as well
AMERICAN JOURNAL OF OPHTHALMOLOGY
November, 1992
Development of a Retinal Artery Macroaneurysm at the Site of a Previously Detected Retinal Artery Embolus Robert A. Wiznia, M.D. Department of Ophthalmology and Visual Science, Yale University School of Medicine. Inquiries to Robert A. Wiznia, Μ.Ό., 850 Howard Ave., New Haven, CT 06519. Although the origin of acquired retinal artery macroaneurysms is not entirely certain, several conditions associated with focal arterial wall damage have been implicated. 1 Inherent weak nesses, such as arterial bifurcations and arteriovenous crossing sites, as well as general condi tions such as arteriosclerosis, aging, and hypertension have been noted in several se ries.1·2 Increased serum lipids, branch retinal vein occlusion, branch retinal artery occlu sion,2·3 and diabetes mellitus 3 are other diseases with an increased association. Emboli are frequently seen near macroaneu rysms.1·3 However, it is rare for an embolus to be noted initially in a retinal artery followed by development of a macroaneurysm at the same location. Arruga and Sanders 4 did not note such an occurrence in a series of 70 patients with retinal embolism. Two such cases have been described, one by Lewis, Norton, and Gass 1 and the other by Khalil and Lorenzetti. 5 I managed an additional case in which an embolus led to the development of a macroaneurysm at the same location in a previously normal cilioretinal artery. A 77-year-old white man was first examined by me because of age-related macular degener ation on April 22, 1986. Best-corrected visual acuity was R.E.: 20/400 and L.E.: 20/30. He had a hemorrhagic disciform lesion in the right macula and atrophie loss of retinal pigment epithelium in the left macula. Over the next 14 months, the disciform process reached an invo luted end stage with a best-corrected visual acuity of 20/200 in the right eye. His disease course remained stable until he was noted to have a small embolus in the cilioretinal artery, located at the inferonasal edge of the macula of the left eye on Jan. 7, 1991 (Fig. 1). Visual acuity in the left eye was 20/30. Duplex carotid ultrasonography disclosed mild bilateral calcific plaque formation in the com mon carotid bifurcation and origin of the inter nal carotid arteries without marked stenosis. On April 15, 1991, the patient noted blurred
Fig. 1 (Wiznia). Fluorescein angiogram delineates an embolus in the cilioretinal artery at the inferona sal edge of the macula in the left eye on Jan. 7, 1991. vision in his left eye, which was found to be caused by retinal hemorrhage associated with a retinal artery macroaneurysm (Fig. 2). Visual acuity was 20/70 in the left eye. By April 30, 1991, additional hemorrhage and edema were noted and visual acuity was 20/400 in the left
Fig. 2 (Wiznia). Retinal artery macroaneurysm is present at the previous site of the embolus as demon strated in the late venous-phase angiogram obtained on April 15, 1991.
Vol. 114, No. 5
Letters to The Journal
eye. The patient would not consent to laser treatment at any time during his treatment course. Unfortunately, further bleeding oc curred, which resulted in a dense vitreous hem orrhage. The left eye was monitored conservatively for several months, but showed no sign of clearing. Visual acuity in the right eye improved during this time to 20/70. On Aug. 28, 1991, the patient underwent pars plana vitrectomy in the left eye. The surgical procedure proceeded fa vorably, but marked preretinal fibrosis, retinal pigment epithelial atrophy, and metaplastic subretinal tissue were evident in the macula. Early preoperative cataract demonstrated pro gression that ultimately required extracapsular cataract and anterior chamber intraocular lens implantation, which were performed on Dec. 11, 1991. On Jan. 23, 1992, best-corrected visual acuity was R.E.: 20/40 and L.E.: 20/300. There was no clinical sign of hemorrhage in the left macula, but there was distortion caused by preretinal fibrosis and metaplastic subsensory retinal tis sue. Focal arterial wall damage is the likely pre cursor of retinal artery macroaneurysms. The rarity of emboli as the cause of this damage is evident from previous reports of macroaneu rysms. Some additional cases in which an embolus is noted near a macroaneurysm could have a similar origin. Palestine, Robertson, and Goldstein 3 noted that only one of ten cases had an embolus nearby. Thus, it is likely that most are not caused by embolie disease. Of course, most macroaneurysms are detected because of symptoms of loss in visual acuity. The appear ance of the retinal artery before the onset of the symptoms is not known in most cases, and the potential for an undetected embolus to have preceded the macroaneurysm cannot be exclud ed. Emboli have been demonstrated to be the definite cause of a retinal artery macroaneu rysm in three cases, including the present one. 1 · 5 The lack of macroaneurysms as a subse quent development in the large series of retinal artery emboli described by Arruga and Sand ers4 implies that most macroaneurysms are at tributable to other causes.
References 1. Lewis, R. A., Norton, E. W. D., and Gass, J. D. M.: Acquired arterial macroaneurysms of the retina. Br. J. Ophthalmol. 60:21, 1976.
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2. Cleary, P. E., Kohner, E. M„ Hamilton, A. M, and Bird, A. C: Retinal macroaneurysms. Br. J. Oph thalmol. 59:355, 1975. 3. Palestine, A. G., Robertson, D. M., and Gold stein, B. G.: Macroaneurysms of the retinal arteries. Am. J. Ophthalmol. 93:164, 1982. 4. Arruga, J., and Sanders, M.: Ophthalmologic findings in 70 patients with evidence of retinal embo lism. Ophthalmology 89:1336, 1989. 5. Khalil, M., and Lorenzetti, M. D.: Acquired reti nal macroaneurysms. Can. J. Ophthalmol. 14:163, 1979.
Nonbacterial Thrombotic Endocarditis and Cortical Blindness J a m e s A. R u s h , M . D . Department of Ophthalmology, University of South Florida College of Medicine. Presented at the IX International Neuro-Ophthalmological Symposium, Williamsburg, Virginia, June 30, 1992. Inquiries to James A. Rush, M.D., 508S Habana Ave., Suite 100, Tampa, Fl 33609. A 73-year-old man had a two-week history of stepwise painless progressive visual loss in both eyes. No focal nonvisual neurologic symp toms occurred; however, he experienced four weeks of headaches and two weeks of mild confusion. His medical history was unremarka ble. Visual acuity was R.E.: 20/200 and L.E.: counting fingers at 2 feet. He identified none of the ten City University color plates with either eye. Pupillomotor responses were brisk without afferent defect in both eyes. Results of the examination of the ocular fundi were normal bilaterally. Computed perimetry showed abso lute superior altitudinal field loss with involve ment of fixation, along with moderate genera lized depression inferiorly in both eyes. Results of a left superficial temporal artery biopsy and bilateral carotid ultrasonograms were normal. Cranial magnetic resonance images showed a lesion in the left occipital lobe of mixed signal, suggesting hemorrhage, and mild gyral en hancement of the right occipital cortex (Fig. 1). Progressive confusion and weight and appetite loss resulted in hospitalization and death 11 days later. A chest x-ray film disclosed a left upper lobe infiltrate found on autopsy study to be a mucin-secreting pulmonary adenocarcinoma. Nonbacterial thrombotic endocarditis was present on the aortic and mitral valves, as well
