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Show ]. Clin. Neuro-ophthalmol. 5: 95-98, 1985 Neovascular Glaucoma as a Complication of the Wyburn-Mason Syndrome LORRI EFFRON, M.D. Z. NICHOLAS ZAKOV, M.D. ROBERT L. TOMSAK, M.D., Ph.D. I(] 1985 Raven Press, New York Abstract A child with the Wyburn-Mason syndrome developed neovascular glaucoma in association with changes in the retinal arteriovenous malformation and signs of retinal and choroidal ischemia. The Wybum-Mason syndrome is a rare congenital anomaly consisting of arteriovenous malformations involving the retina and central nervous svstem. l The intracranial malformation may hemorrhage, but the retinal lesion is usually stable. Occasionally, retinal hemorrhage and exudation from the retinal arteriovenous communication occur. 2-5 Although in some cases capillary nonperfusion has been documented by intravenous fluorescein angiography, 2,6 to the best of our knowledge, iris neovascularization and glaucoma have not previously been reported. 3A,7 We describe what we believe to be the first case of rubeosis iridis and neovascular glaucoma in a patient with Wyburn- Mason syndrome who had severe ischemia of both the retinal and choroidal circulation. Case Report A 4-year-old white girl was referred to the Cleveland Clinic Foundation in March 1981. A diagnosis of amblyopia of the left eye had been made when the child was 3 years of age. Past medical and family histories were noncontributory. Initial ocular examination at the Cleveland From the Department of Ophthalmology, Cleveland Clinic Foundation, Cleveland, Ohio. Write for reprints to: R. L. Tomsak, M.D., Ph.D., Department of Ophthalmology, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44106, U.S.A. Clinic revealed right eye vision of 20/30 and left eye vision of 20/200. The complete examination was normal, except for a 3+ afferent pupillary defect on the left, 3.5 mm of proptosis of the left eye, and a vascular anomaly consisting of a large dilated vessel inferotemporally on the left disc (Fig. 1). Neurologic examination was remarkable only for a bruit over the left mastoid and supraclavicular areas. High resolution computed tomography (CT) scanning of the orbits and parasellar region with and without contrast enhancement demonstrated a dilated vascular mass in the posterior portion of the left orbit extending into the suprasellar cistern. Digital subtraction angiography showed a vascular mass in the left retro- orbital space. A conventional angiogram of the left internal carotid artery revealed an arteriovenous malformation in the posterior aspect of the left orbit, and suprasellar region supplied primarily by the ophthalmic artery (Fig. 2). Based on the clinical and angiographic findings, a diagnosis of Wyburn-Mason syndrome was made. On a routine follow-up examination 15 months later, visual acuity of the left eve and exophthalmometry measurements we're unchanged, Repeat fundus examination demonstrated a change in the appearance of the arteriovenous malformation, with further dilation of the arteriovenous loop inferiorly and newlv dilated vessels on the superior portion of the optic disc (Fig, 3). On November 15, 1982, the patient was seen for a red, painful left eye of 2 days' duration, Visual acuities were 20/20 in the right eve and counting fingers at 1 ft in the left eye. Exophthalmometry measurements were ~nchanged. Slit lamp examination showed diffuse miCrocystic corneal edema and mild anterior chamber reaction consisting of a few cells and 95 Nl'l)\',ISClIl,H CI,llKOIll,1 in Wvbllrn M,lson Syndrollll' Figure 1. Vascular anomalY on the Ie it optic disc (March llJHI). trace tlare, in addition to neovascularization of the iris. Applanation tension of the left eye was 52 mm Hg. Fundus examination of the left eye revealed disc edema, posterior pole retinal hemorrhages, beading and dilation of the retinal veins, extreme narrowing of the retinal arteries, and choroidal infarcts in the midperiphery (Fig. 4). The anomalous vessels on the optic disc were further dilated. Gonioscopy showed that the anterior chamber angle was closed almost 3600 with neovascularization. The bruit over the left mastoid and supraclavicular areas could no longer be heard. A diagnosis of neovascular glaucoma with both retinal and choroidal ischemia was made. The intraocular pressure was brought under control with intravenous Diamox, and topical Timoptic, epinephrine, and pilocarpine. Intravenous fluorescein angiography showed diffuse choroidal fluorescence in the posterior pole of the left eye, with complete filling of the anomalolls vessels on the optic disc but no filling of the remaining retinal vessels 15 min after injection. On November 18, 1982, pamelinal xenon laser photocoagulation was performed under general anesthesia. A repeat left internal carotid angiogram demonstrated no significant change in the orbital and suprasellar vascular malformation. On follow-up examination approximately 3 weeks later, the visual acuity was light perception. The iris rubeosis was markedly decreased and the applanation pressure was 10 mm Hg. A repeat tluorescein angiogram showed some decrease in size of the anomalous vessels on the optic disc and also diminished profusion of these (Fig. 5). On subsequent follow-up examinations, the e;.e has become prephthisical. Discussion In reviewing this case of W~'burn-Masonsyndrome, \\'e note t\\'o interesting features: the Figure 2. Subtracted Iell Illtern,11 (.1rolld angiogram showing an arteriovenous malformation along the course of the left ophthalml( artery (arrowlll',lds). 96 Journal of Clinical Neuro-ophthalmology Figure 3. Enl.H~,'nll'nt ,It till' ",Iscular ,1I111m,ll" (August IllS2) change in the size and wnfiguration of the retinal arteriovenous malformation and the development of rubeosis iridis and neovascular glaucoma. The visual acuity in the Wvburn-Mason syndrome may range 'from norm'al' to no light perception. s 'A homonymous hemianopia may occur as a result of the intracranial portwn ot the arteriovenous malformation.') The retinal portion may produce decreased vision either by Cystic retinal degeneration2 or vascular decompensation. ; The case we describe demonstrated spontaneous change in the size of the retinal arterio- Figure 4, Further enlargement l1f the vascular anomaly with hemorrhages and other signs l1f retll1allschemla (November 1982). June 1985 Effron d al. Figure 5. flul1rescein an~iogram shl1wing involution of the vdscular anomalv after xenon laser photocoagulation (Decl'mbl'r I '-is::!) venous malformation (Figs. 1, 3, and 4). Whether this change represented primary growth of the arteriovenous malfor~ation or a disturbance in vascular hemodvnamlCs WIth eIther increased or reduced flow is not known. Spontaneous regression and enlargement of these retinal arteriovenous malformatwns have been documented. III, II Augsburger and coworkers l2 described the changing appearance of a complex retinal arteriovenous malformation over a 17-year period. While one portwn ot the malformation underwent spontaneous obstruction, another portion underwent marked elongation, tortuosity, and dilation .. Another unusual teature of thIS case was the development of rubeosis iridis and neovascular glaucoma, which to our knowledge has not been previously reported in the Wvburn-Mason svndron1l'. We suggest that ocular IschemIa p"'ayed an important role in the devek1pment ot the iris neovasculariz,1tion in this caSt', as IS sometimes seen in central rdin,1l vein occlusion and diabetic rdinopath~'. Ashton 11 proposed that tht' ischemic retina produces a vasculogenic factor that can c!Ittuse anteriorlv to produce iris neov,1sculanzatwn. The chor~)ida' infarcts noted in our p,1tient may also have contributed to till' dt"'elopnwnt of rubeosis iridis. 14 To explain the presence of ocular ischemia in our patient, two mechanisms may have b:en opera tive. If the ,uteriovenous maltorma tion supplied part of the arterial circulation to th~ retina and choroid, then a partial thrombOSIS ot the malformation could have produced ocular ischemia. Alternatively, increased flow and an 97 Neovascular Glaucoma in Wyburn-Mason Syndrome enlarging arteriovenous malformation could have led to a change in hemodynamics, resulting in a "steal" phenomenon with decreased flow to the arteries supplying the retina and the choroid. This concept of steal with subsequent ocular ischemia and rubeosis iridis has previously been proposed by Huckman and Haas. 15 They reported two cases of internal carotid artery occlusion complicated by neovascular glaucoma in which retrograde flow in the ophthalmic artery was documented by carotid arteriography. We feel that it is important to note that the arteriovenous malformation of the WyburnMason syndrome may change in size, probably because of altered hemodynamics. Therefore, intravenous fluorescein angiography with specific attention to intraretinal circulation and choroidal flow patterns should be of help in detecting changes indicative of abnormal perfusion of the posterior pole and thus in managing and preventing rubeosis iridis and neovascular glaucoma. If the development of rubeosis iridis could be anticipated, it could be treated at an earlier stage. In summary, this case demonstrates that rubeosis iridis and neovascular glaucoma may occur in association with changes in the ocular arteriovenous malformation of the WyburnMason syndrome. References 1. Wyburn-Mason, R.: Arteriovenous aneurysms of mid-brain and retina, facial naevi and mental changes. Brain 66: 163-203, 1943. 2. Archer, D. B., Deutman, A., Ernest, J. T., and Krill, A. E.: Arteriovenous communications of the retina. Am. J. Ophthalmol. 75: 224-241, 1973. 3. Zion, V. M.: Phakomatoses. In Duane T. D., Ed., 98 Clinical Ophthalmology, vol. 5. Harper & Row, Philadelphia, 1983, chap. 36, pp. 7-9. 4. Font, R. L., and Ferry, A. P.: The phakomatoses. lilt. Ophthalmol. Clin. 12: 1-50, 1972. 5. Bernth-Petersen, P.: Racemose haemangioma of the retina: report of three cases with long term follow up. Acta Ophthalmol. 57: 669-678, 1979. 6. Peyman, G. A., Sanders, D. R., and Goldberg, M. F.: Principles and Practice of Ophthalmology, vol. 2. W. B. Saunders, Philadelphia, 1980, pp. 13121324. 7. Walsh, F. B., and Hoyt, W. F.: Clinical Neuro-ophthalmology, vol. 2 (3rd ed.). Williams & Wilkins, Baltimore, 1969, pp. 1692 & 1694. 8. Hopen, G., Smith, J. L., Hoff, J. T., and Quencer, R.: The Wyburn-Mason syndrome, concomitant chiasmal and fundus vascular malformations. J. Clin. Neuro. Ophthalmol. 3: 53-62, 1983. 9. Theron, J., Newton, T. H., and Hoyt, W. F.: Unilateral retinocephalic vascular malformations. Neuroradiology 7: 185-196, 1974. 10. Dekking, H. M.: Arteriovenous aneurysm of the retina with spontaneous regression. Ophthalmologica 130: 113-115,1955. 11. Gregersen, E.: Arteriovenous aneurysm of the retina. A case of spontaneous thrombosis and "healing." Acta Ophthalmol. 39: 937-939,1961. 12. Augsburger, J. J., Goldberg, R. E., Shields, J. A., Mulberger, R. D., and Magargal, L. E.: Changing appearance of retinal arteriovenous malformation. Albrecht von Graefes Arch. Klin. Exp. Ophthalmal. 215: 65-70, 1980. 13. Ashton, N.: Retinal vascularization in health and disease. Am. r. Ophthalmol. 44: 7-17,1957. 14. Brown, G. C, Magargal. L. E., Simeone, F. A., Goldberg, R. E., Federman, J. L., and Benson, W. E.: Arterial obstruction and ocular neovascularization. Ophthalmology 89: 139-146, 1982. 15. Huckman, M. S., and Haas, J.: Reversed flow through the ophthalmic artery as a cause of rubeosis iridis. Am. J. Ophthalnlal. 74: 1094-1099, 1972. Journal of Clinical Neuro-ophthalmology |
