value in treatment of choroidal neovascularization, it hasnot yet been widely used for other retinal diseases. Neo-vascular membranes in idiopathic juxtafoveolar retinaltelangiectasis appear to originate from the retina, ratherthan from the choroid.1 One theoretical concern is thatthe photosensitizing drug may leak out of the retinalvessels in the macula and potentially cause harm. Theleakage in idiopathic juxtafoveolar retinal telangiectasis istypically slow, accounting for relatively preserved vision inpatients without subretinal neovascular membranes, andpossibly for the fact that no apparent retinal damage wasnoted to the area surrounding the subretinal neovascularmembrane in our patient. Larger studies with long-termfollow-up will be important to establish whether this newtreatment should be recommended.

REFERENCES

1. Gass JDM, Blodi BA. Idiopathic juxtafoveolar retinal telan-giectasis. Update of classification and follow-up study. Oph-thalmology 1993;100:1536–1546.

2. Lee AL. Bilateral subretinal neovascular membrane in idio-pathic juxtafoveolar retinal telangiectasis. Retina 1996;16:344–346.

3. Berger AS, McCuen BW, Brown GC, Brownlow RL. Surgicalremoval of subfoveal neovascularization in idiopathic juxtafo-veolar retinal telangiectasis. Retina 1997;17:94–98.

4. Treatment of Age-related Macular Degeneration by Photody-namic Therapy (TAP) Study Group. Photodynamic therapyof subfoveal choroidal neovascularization in age-related mac-ular degeneration with verteporfin. Arch Ophthalmol 1999;117:1329–1345.

5. Sickenberg M, Schmidt-Erfurth U, Miller JW, et al. Apreliminary study of photodynamic therapy using verteporfinfor choroidal neovascularization in pathologic myopia, ocularhistoplasmosis syndrome, angioid streaks, and idiopathiccauses. Arch Ophthalmol 2000;118:327–336.

Retinal and Choroidal ArterialOcclusion in Wegener’sGranulomatosisTomohiro Iida, MD, Richard F. Spaide, MD, andJeff Kantor, MD

PURPOSE: To describe a patient with Wegener’s granulo-matosis presenting with bilateral retinal and choroidalarterial occlusion.

METHODS: A 64-year-old male with Wegener’s granulo-matosis was examined using ophthalmoscopy and indo-cyanine green angiography.RESULTS: The patient had central retinal arterial occlu-sion in the right eye and branch retinal arterial occlusionin the left eye associated with bilateral choroidal infarcts.Early phase wide-angle ICG angiography of left eyerevealed widespread choroidal perfusion problems, pre-sumably from the confluence of multiple segmental arte-rial occlusions. Indocyanine green angiography of theright eye showed similar widespread choroidal arterialocclusions.CONCLUSIONS: This patient developed loss of vision frommultiple arterial occlusions of the retina and choroid.Retinal and choroidal ischemia of this patient may havebeen caused by vasculitis leading to occlusion of retinalarteries and multiple short posterior ciliary arteries.(Am J Ophthalmol 2002;133:151–152. © 2002 byElsevier Science Inc. All rights reserved.)

WEGENER’S GRANULOMATOSIS IS A DISORDER CHAR-

acterized by necrotizing granulomatous lesions ofthe upper and lower respiratory tracts, generalized focalnecrotizing vasculitis, and glomerulonephritis. Ocularmanifestations occur in 30%–50% of the patients.1 Orbitalinvolvement and scleritis are the common ocular manifes-tations; retinal and choroidal vascular occlusions are muchless frequent. In this report, we describe a case of Wegen-er’s granulomatosis presenting with bilateral retinal andchoroidal arterial occlusions.

● CASE: A 64-year-old male, who had been diagnosed ashaving Wegener’s granulomatosis 2 years previously andwas treated with corticosteroids, presented with a com-plaint of visual disturbance in the right eye for one year.His best corrected visual acuity was no light perception inthe right eye and 20/200 in the left eye. He had denseposterior subcapsular cataracts bilaterally. In his right eye,there was optic atrophy, central retinal artery obstructionwith sclerosis of the vessels and widespread triangularshaped areas of pigmentary clumping and atrophy (Figure1, left). In the left eye, he had obstruction of inferotem-poral branch retinal artery with sclerosis and chorioretinalatrophy with pigmentary changes of the inferior fundus(Figure 1, right). Because he had glomerulonephritis anddense cataracts, we performed only indocyanine green(ICG) angiography, which is metabolized by the liver andallows enhanced imaging of the retina and choroid even ineyes with opacity as compared with fluorescein angiogra-phy. Early phases of wide-angle ICG angiography of theleft eye using wide-angle contact fundus lens2 showed awidespread filling defect of choroidal arteries and chorio-capillaris in the inferior fundus and in segmental areas ofthe superior fundus (Figure 2). The choroidal veins inthese affected areas were smaller in caliber. Occlusion of

Accepted for publication Aug 31, 2001.From the LuEsther T. Mertz Retinal Research Center, Manhattan Eye,

Ear and Throat Hospital, New York, New York, and the Vitreous-Retina-Macula Consultants of New York, NewYork.

Supported by The Macula Foundation, Inc.The authors have no financial interest in any aspect of this study.Inquiries to Richard F. Spaide, MD, Vitreous-Retina-Macula Consult-

ants of New York, 519 East 72nd Street, Suite 203, New York, NY 10021;fax (212) 628-0698; e-mail: VRMNY@aol.com

BRIEF REPORTSVOL. 133, NO. 1 151

infratemporal retinal artery was also seen in the left eye.Indocyanine green angiography of the right eye alsoshowed similar widespread filling defect of choroidal arter-ies and choriocapillaris.

Our patient had central retinal arterial occlusion in theright eye and branch retinal arterial occlusion in the lefteye associated with bilateral choroidal ischemia. Earlyphase wide-angle ICG angiography of the left eye revealedwidespread choroidal perfusion problems. We believe thatthese choroidal perfusion problems resulted from the con-fluence of multiple segmental arterial occlusions becausethere were multiple triangular areas of pigmentary changeswith filling defects of choroidal arteries and choriocapil-laris, and the large area of involvement does not corre-spond to the distribution of the major branches of theophthalmic artery which come off either medially orlaterally. There were separated areas of triangular choroi-dal ischemia in the superior fundus, that could not havecome from a single artery occlusion and there was a lack oflarger and medium arteries visible during early fillingphase.

Ocular histopathology of a patient with Wegener’sgranulomatosis has shown lymphocytic infiltrationthroughout the choroid and marked sclerosis of choroidalblood vessels with thickening of the media and prominentendothelial cells.3 Retinal and choroidal ischemia in ourcase may have been caused by vasculitis leading to occlu-sion of central or branch retinal arteries, and multipleshort posterior ciliary arteries. Kinyoun and associates4 andMirza and associates5 reported respective cases with We-gener’s granulomatosis associated with bilateral choroidalinfarctions, but they had limited ability to state where thechoroidal occlusion occurred because they did not use ICGangiography. Wide-angle ICG angiography was useful inour case for visualizing the pathologic hemodynamicchanges of large areas of the fundus. This technique is

superior to manually assembling montages of photographs,which are, by default, taken at different times during theangiographic sequence.2

REFERENCES

1. Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA.Ocular complications of Wegener’s granulomatosis. Ophthal-mology 1983;90:279–290.

2. Spaide RF, Orlock DA, Herrmann-Delemazure B, et al.Wide-angle indocyanine green angiography. Retina 1998;18:44–49.

3. Cutler WM, Blatt IM. The ocular manifestations of lethalmidline granuloma (Wegener’s granulomatosis). Am J Oph-thalmol 1956;42:21–35.

4. Kinyoun JL, Kalina RE, Klein ML. Choroidal involvement insystemic necrotizing vasculitis. Arch Ophthalmol 1987;105:939–942.

5. Mirza S, Raghu Ram AR, Bowling BS, Nessim M. Centralretinal artery occlusion and bilateral choroidal infarcts inWegener’s granulomatosis. Eye 1999;13:374–376.

Multiple Retinal Vein Occlusions inEssential ThrombocythemiaMitsuhiro Imasawa, MD, andHiroyuki Iijima, MD

FIGURE 2. Early phase of wide-angle ICG angiography of theleft eye revealed a widespread filling defect of the choroidalarteries and choriocapillaris in the inferior fundus (arcuatearrow) and in the segmental areas of the superior fundus (*).Occlusion of infratemporal retinal artery is also seen.

FIGURE 1. A 64-year-old male with Wegener’s granulomato-sis. (Left) A fundus photograph of the right eye shows sclerosisof the central retinal artery and its branches. A subcapsularcataract obscures the visualization of the macula and temporalperiphery. (Right) Fundus photograph of his left eye showsobstruction of inferotemporal retinal artery with sclerosis of thevessels and chorioretinal atrophy with pigmentary changes ofthe inferior fundus.

AMERICAN JOURNAL OF OPHTHALMOLOGY152 JANUARY 2002