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Show J. Clin. Neuro-ophthalmol. 4: 59-67, 1984. Neuroradiological Clinical Pathological Correlations Neovascular Glaucoma and Carotid Bruits PAUL R. ROSENBERG, M.D. JOSEPH B. WALSH, M.D. ROBERT D. ZIMMERMAN, M.D. A 59-year-old hypertensive male complained of decreased acuity in his right eye for 3 weeks. On examination, there was finger-counting vision in the right eye and 20/20 in the left eye. Examination of the left eye was normal. Rubeosis iridis was present in the right eye, with an almost totally closed angle secondary to peripheral anterior synechiae (PAS). Intraocular pressure was 30 mm Hg in the right eye and 14 mm Hg in the left eye. The right fundus showed multiple cotton- wool spots in the posterior pole (Fig. 1). There was segmentation of the blood column in the major veins and scattered peripheral blot hermorrhages in all quadrants. On auscultation, bilateral carotid bruits were heard. Ophthalmodynamometry was 45 mm diastolic in both eyes as compared to a blood pressure of 135/80. Past medical history was positive for hypertension controlled with Diuril, and angina pectoris controlled with Isordil. On further questioning, he did mention transient, episodic weakness of his left arm and leg. An intravenous retinal fluorescein angiogram was done (Figs. 2a-2d). A workup was then initiated in the hospital, including digital intravenous angiography (DIYA) (Fig. 3) and cerebral arteriography (Fig. 4). Fluorescein angiography revealed striking delays in choroidal, retinal arterial, and venous per- From the Departments of Ophthalmology (PRR, JBW) and Radiology (RDl), Montefiore Medical CenterlAlbert Einstein College of Medicine, Bronx, New York. March 1984 fusion (Figs. 2a-2c). There was a diffuse leakage from all of the retinal vessels in the later stages and leakage from dilated vessels on the optic nerve head (Fig. 2d). The mvA was suboptimal due to motion artifact induced by involuntary swallowing by patient. It revealed atherosclerosis at the left carotid artery bifurcation and suggested right internal carotid artery occlusion (Fig. 3). Selective carotid arteriography revealed 50% stenosis of the right external carotid artery and 95% stenosis of the right internal carotid artery with marked diminution of cephalic flow in this vessel (Fig. 4). This patient underwent uncomplicated carotid endarterectomy on the right side. Two days later, the intraocular pressure in the right eye rose to 50 mm Hg, on maximal medical therapy. A repeat fluorescein angiogram showed moderate improvement in ocular blood flow. Cyclocryotherapy was performed to control the pressure. Three weeks later, the visual acuity remained at finger counting. There was a decrease in iris neovascularization and intraocular tension was 20 mm Hg on Timoptic. On funduscopic examination, there was resolution of the cotton-wool spots and no evidence of segmental venous flow (Fig. 5). Compared to the preoperative angiogram, fluorescein angiography showed improvement in retinal blood flow, but delayed perfusion was still present (Figs. 6a and 6b). A postoperative mvA was once again suboptimal due to motion degradation. However, it demonstrated improved flow in the right internal carotid artery. 59 Neovascular Glaucoma Figure 1. Fundus photograph of the posterior pole of the right eye shows narrowed arterioles; thickened. irregular venules; and scattered colton-wool spots. Discussion Carotid occlusive disease may have several clinical presentations. Oollery et a1. 1 classified them in the following way: 1) explosive occlusion with sudden stroke and death, 2) asymptomatic, 3) associated with transient ischemic episodes, and 4) associated with visual symptoms. Based on these modes of presentation, the neurologist or the ophthalmologist is likely to see these patients initially. The ocular manifestations of carotid occlusive disease are varied and include hemianopic field defects, amaurosis fugax, cotton- wool patches, ischemic optic neuropathy, neovascularization of the anterior segment with neovascular glaucoma, chronic ischemic retinopathy/ and embolic arterial occlusive disease. Asymmetric retinopathy with moderated involvement of the occluded side in diabetic) and hypertensive4 retinopathy has also been noted, with carotid occlusive disease. The frequency of ocular involvement in carotid occlusive disease varies, depending on the type of referral center: neurologic 40%' or ophthalmologic 88%.6 If one recognizes the high morbidity and mortality associated with stroke, and considers the recent improvements in surgical procedures such as carotid endarterectomy6.7 or superficial temporal arterylmiddle cerebral bypass, then the need for early and rapid diagnosis of carotid disease becomes evident. Keams and Hollenhorst2 reviewed 600 cases of intermittent insufficiency or thrombosis of the carotid arterial system. About 5% had changes compatible with chronic ischemic retinopathy. Two patients had neovascular glaucoma. It is felt that the presence of anterior segment neovascularization is due to ischemia of the anterior and/ or posterior segment. The patient we present had unilateral neovascular glaucoma, with cotton-wool spots and retinal vascular sludging. A carotid bruit was present. Although ophthalmodynamometry (OOM) is felt to be a good noninvasive test,9 the diastolic OOMs were not helpful in this case. Normal ODMs have been seen frequently in the face of carotid artery disease.s The delayed filling of the retinal and choroidal circulation as detected on fluorescein angiography strongly suggests occlusion of the ophthalmic or carotid artery or a branch of the aortic arch. The history of previous TIAs indicates clinically significant carotid occlusive disease. Traditionally, selective carotid arteriography, with or without aortic arch arteriography, has been the procedure of choice for neuroradiologic evaluation of cerebrovascular disease. Digital venous angiography (DIVA) is a recently introduced technique which uses computer manipulation of radiographic images, thus markedly increasing contrast resolution. 1o This, in tum, allows for good visualization of important vascular struc- Journal of Clinical Neuro-ophthalmology March 1984 Rosenberg, Walsh, Zimmerman (a) (b) Figures 2a-2d Fluorescein angiogram of patient in Fig. 1. (a) Delayed choroidal and arterial transit times-27.0 seconds. (b) Slow filling of the superotemporal arteriole-44.1 seconds. (e) Laminar flow in the inferior venules and absence of flow in superior venule branches58.0 seconds. (d) Diffuse leakage from retinal vasculature with leakage from. and staining of, the optic nerve head-98.2 seconds. 61 Neovaseular Glaucoma (el (d) Figure 2a-2d Continued Journal of Clinical Neuro-ophthalmology Rosenberg, Walsh, Zimmerman Figure 3. Right posterior oblique (RPO) view from digital venous angiogram (DIVA) demonstrates a 50% stenosis of the origin of the left internal carotid artery arrow). The proximal left internal carotid artery is visualized above and to the left (posterior) to the common carotid artery. The right carotid artery bifurcation is obscured by artifacts (curved arrow) which are visualized as irregular areas of decreased and increased density. Patient swallowing during the procedure produces this "misregistration" artifact. In the region where the right internal carotid artery should be visualized (arrowhead). no vessel is seen. tures (such as the carotid arteries) following noninvasive venous injection of contrast material. I I. 12 In some patients, DIVA may completely replace the more invasive arteriogram, but care must be taken to determine that the DIVA study ade- March 1984 quately visualizes the vascular anatomy in question. Suboptimal DIVA studies occur when the patient cannot remain motionless during the examination (swallowing is a constant problem since this creates artifacts that overlie the carotid 63 Neovascular Glaucoma Figure 4. Lateral view from selective right common carotid angiogram demonstrates severe atherosclerotic involvement of the carotid bifurcation with 95% stenosis of the origin of the internal carotid artery (arrow) and 50% stenosis of tbe external carotid artery origin. Note the small caliber of the internal carotid artery distal to the stenosis (the vessel is about one-half its nonnal diameter and approximates the size of external carotid branches in the vicinity) and the slow flow in this vessel (external carotid branches. such as the superfidaltemporal arteries. are filled prior to intracranial carotid branches, a reversal of the nonnal flow pattern). Journal of Clinical Neuro-ophthalmology Rosenberg, Walsh, Zimmennan Figure 5. Fundus photograph of patient in Fig. 1 after carotid endarterectomy, showing resolution of the cotton-wool spots and less prominence of the nerve fiber layer. bifurcation). 13 In addition, the nonselective nature of the procedure (all arteries are opacified simultaneously) often produces vascular overlap that may obscure focal, but significant areas of stenosis or ulceration. l3 · 14 When DIVA is suboptimal for any reason (as in this case), arteriography must be performed. March 1984 An interesting feature of this case was the marked elevation in intraocular pressure immediately postendarterectomy. Fluorescein angiography revealed a moderate increase in retinal blood flow. We postulated that this also caused improved perfusion of the ciliary circulation, with increased aqueous secretion in an eye with a 6S Neovascular Glaucoma (Il) (b) Figures 60 and 6b. Fluorescein angiogram 1 week after carotid endarterectomy. (a) Note that retinal arteriole and choroidal filling is essentially completed at 23.4 seconds. (b) Al 34.4 seconds. there is almost complete filling of the retinal vasculature. Journal of Clinical Neuro-ophthalmology markedly impaired drainage mechanism. The present case demonstrates a typical example of neovascular glaucoma secondary to carotid occlusive disease. Although the visual acuity and ultimate prognosis of the involved eye remain poor, treatment of the occlusion by endarterectomy was performed. Hopefully, this will diminish the possibility of a stroke. References 1. Wise, G.N., Dollery, C.T., and Henkind, P.: The Retinal Circulation. Harper & Row, New York, 1971, pp. 309-310. 2. Keams, T.P., and Hollenhorst, R.W.: Venous stasis retinopathy of occlusive disease of the carotid artery. Proc. Staff Meet. Mayo Clitl. 38: 304-312, 1963. 3. Gray, A.T., and Rosenbaum, AL.: Retinal artery pressure in asymmetric diabetic retinopathy. Arch. Ophthalmol. 75: 758-762, 1966. 4. Hollenhorst, R.W.: A symposium: Occlusive vascular disease: Carotid and vertebral basilar artery stenosis and occlusion: Their ophthalmologic considerations. Trans. Am. Acad. Ophthalmol. Otolaryngol. 66: 166-180, 1962. 5. Johnson, H.C. and Walker, AE.: Angiographic diagnosis of spontaneous thrombosis of internal common carotid arteries. J. Neurosurg. 8: 631-659, 1951. 6. Sandok, B.A., Fulan, AJ., Whisnant, J.P., and Sundt, J.M.: Guidelines for the management of TiNs. Mayo Clin. Proc. 53: 665-674, 1978. 7. Thompson, J.E., And Garrett, W.V.: Peripheral arterial surgery. N. Engl. J. Med. 302(9): 491-503, 1980. 8. Keams, T.P., Siekert, R.G., and Sundt, J.M.: The ocular aspects of carotid artery bypass surgery. Trans. Am. Ophthalmol. Soc. 76: 247-265, 1978. 9. Sanborn, G.E., Miller, N.R., Langham, M.E., and Kumar, AJ.: An evaluation of currently available noninvasive tests of carotid artery disease. Ophthalmology 87: 435-439,1980. March 1984 Rosenberg, Walsh, Zimmerman 10. Mistretta, C.A: Development of digital subtraction angiography. In Digital Subtraction Angiography: An Application of Computerized Fluoroscopy, C.A Mistretta, A.B. Crummy, C.M. Strother, and J.F. Sackett, Eds. Yearbook Medical Publishers Inc., Chicago, 1982, pp. 7-15. 11. Chilcote, W.A, Modic, M.T., Pavilcek, W.A., et al.: Digital subtraction angiography of the carotid arteries: A comparative study in 100 patients. Radiology 139: 287-295, 1981. 12. Hoffman, M.G., Gomes, A.S., and Pais, 5.0.: Pitfalls and limitations in the interpretation of intravenous carotid digital subtraction angiography (DSA). Presented at the 68th Annual Meeting of the Radiologic Society of North America, Chicago, Illinois, November 30, 1982. 13. Zimmerman, R.D., Goldman, M.J., Auster, M., Chen, c., and Leeds, N.E.: Aortic arch digital arteriography-An alternative technique to digital venous angiography and routine arteriography in the evaluation of cerebrovascular insufficiency. Presented at XI Symposium Neuroradiologicum, Washington, D.C., October 1982. (Accepted for publication: AJNR 1983). 14. McGinnis, B., Hesselink, J.R., Ackerman, R., Davis, K.R., and Taveras, J.M.: The use of subtraction angiography in the evaluation of cerebral transient ischemic attacks. Presented at the 68th Annual Meeting of the Radiologic Society of North America, Chicago, Dlinois, November 30, 1982. Acknowledgments The authors thank Drs. Frank Veith and Michael Swerdlow for their assistance in the management of this case. We also acknowledge the photographic assistance of Kenn Kostuk and Larry Egan, and the secretarial assistance of Alice Simonelli. Write for reprints to: Joseph B. Walsh, M.D., Montefiore Hospital and Medical Center, 111 East 210th Street, Bronx, ew York 10467. 67 |