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Show /llllTllal (If Clillical N,,"rcl-tII'IIII/IJ!JIIIII,'gl/ (,(2): /20·-/27. 198/,. Letters to the Editor Familial Giant Cell Arteritis To tlzl' Editor: We read with interest the article by Tanenbaum and Tenzel (1) in which biopsy-proven temporal arteritis occurred in two siblings. We have reported the occurrence of temporal arteritis in a 73year- old man and, 28 years later, in his daughter. In both cases, the clinical diagnosis was supported by pathologic material (2). The man developed the sudden onset of blindness and was found to have a bilateral ischemic optic neuropathy. Although a temporal artery biopsy was never performed, the patient subsequently died, and pathologic examination of the right eye revealed that the short ciliary arteries were involved by an occlusive vasculitis with a multinucleated giant cell reaction. The eye also had areas of choroidal infarction with loss of retinal pigment epithelium and outer retinal layers in these areas. The man's daughter developed a typical anterior ischemic optic neuropathy in her left eye associated with systemic evidence suggesting giant cell arteritis. She underwent a left temporal artery biopsy that showed intimal proliferation and fibrosis with resultant narrowing of the vessel lumen, areas of loss and fragmentation of the remaining internal elastic lamina, inflammatory cell infiltration with mononuclear cells, and multinucleated giant cells. We agree with Tanenbaum and Tenzel (1) that there is sufficient evidence to support the familial or inheritable nature of both giant cell arteritis and polymyalgia rheumatica. This evidence includes the geographic distribution, race, and histocompatibility antigens of such patients, and the probability that the association is not caused by chance alone. Michael A. Novak William R. Green Neil R. Miller Johns Hopkins University Baltimore, MD 21205 REFERENCES 1. Tanenbaum M, Tenzel J. Familial temporal arteritis. / Clin Neuro-ophtha/mol 1985;5:244-8. 2. Novak MA, Green WR, Miller NR. Ophthalmological manifestations: a case of familial giant cell arteritis. Md State Med /1984;33:817-20. /26 <01986 Raven Press, New York Neovascular Glaucoma and Ischemia To the Editor: I read with interest the article by Effron and coworkers: "Neovascular Glaucoma as a Complication of the Wyburn-Mason Syndrome" (1). The authors attribute the development of neovascular glaucoma in their patient's left eye to ocular ischemia and propose either one of two mechanisms to explain the presence of the ischemia: (a) partial thrombosis of the malformation which might be supplying part of the retinal and choroidal arterial circulation, or (b) a steal phenomenon secondary to a change in hemodynamics following enlargement of the malformation. Although I certainly cannot explain the enlargement of the malformation (weakening of the vascular wall, primary growth, or disturbance in hemodynamics), there certainly is a more plausible explanation for the neovascular glaucoma. Figure 4 in Dr. Effron's article is compatible with an ischemic central retinal vein occlusion (CRVO). It is possible that the growing malformation has compressed the central retinal vein (CRV), with or without the central retinal artery, leading either to closure of the CRY or to decreased flow and thrombosis of the CRY. Rubeosis iridis and neovascular glaucoma occur in about 60% of eyes with ischemic CRVO (2). Congenital arterial loops on or near the optic nerve head can be complicated by vascular occlusion secondary to thrombosis and/or embolization (3,4). A similar process might have occurred in this patient with a vascular malformation at the optic nerve head. Other local factors that can lead to CRVO are large drusen of the optic nerve head (4), papilledema, subdural cerebral hemorrhage, and optic nerve hemorrhage (5). All of these conditions compress the vein and presumably produce slowing and turbulence of flow that can result in endothelial change and thrombosis. I propose that the Wyburn-Mason syndrome be added to the list of local factors at the optic nerve head that lead to venous, with or without arterial, occlusion. Elias I. Traboulsi, M.D. The Wilmer Eye Institute Johns Hopkins Hospital Baltimore, MD 21205 [VBwyburnmason] |
