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Clinicopathologic Reports, Case Reports, and Small Case Series
September 2004

Choroidal Neovascularization After Central Retinal Vein Occlusion in a 47-Year-Old Man

Author Affiliations



Copyright 2004 American Medical Association. All Rights Reserved.Applicable FARS/DFARS Restrictions Apply to Government Use.2004

Arch Ophthalmol. 2004;122(9):1398-1401. doi:10.1001/archopht.122.9.1398
Report of a Case

A 46-year-old white man was referred to The Wilmer OphthalmologicalInstitute, Baltimore, Md, for evaluation of macular edema. He had been diagnosedas having a nonperfused central retinal vein occlusion (CVO) in the righteye 5 months previously, as documented by photographs and a fluorescein angiogramfrom his referring ophthalmologist (Figure1). Medical history was positive for hypercholesterolemia. Medicationsincluded aspirin and a lipid-lowering agent.

Figure 1.
Image not available

A, Red-free photograph showingthe posterior pole of the right eye 1 month after the onset of visual loss.Diffuse intraretinal hemorrhages and optic nerve head swelling are seen. Visualacuity was 3/200 at this time. B, Early arteriovenous phase of a fluoresceinangiogram 1 month after documentation of the central retinal vein occlusion.Multiple areas of hypofluorescence are seen, which correspond to the intraretinalhemorrhages shown in A, and many areas of nonperfusion. No retinal vesselanastomosis to a fibrovascular lesion or choroidal neovascularization is noted.

Visual acuity at the time of referral was 20/160 in the right eye and20/20 in the left eye. There was no iris or angle neovascularization. Intraocularpressure was 16 mm Hg in each eye. Ophthalmoscopic examination showed resolvingintraretinal hemorrhages in all 4 quadrants in the right eye, with fovealthickening and cystic edema (Figure 2).The patient was diagnosed as having a nonperfused CVO with macular edema.Observation by his referring ophthalmologist for development of any neovascularizationwas recommended. He was told to return to The Wilmer Ophthalmological Institutein approximately 6 months to determine whether he still had macular edemawith perfusion of macular capillaries. If edema persisted, laser photocoagulationmight be considered, because a trend toward an increased chance of stablevisual acuity after photocoagulation, compared with observation, had beendemonstrated in younger patients with perfused macular edema in the CentralVein Occlusion Study.1

Figure 2.
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Color photograph of the posteriorpole of the right eye 5 months after the initial documentation of the centralretinal vein occlusion. In addition to the resolving intraretinal hemorrhagesin all 4 quadrants, clinical examination showed cystoid macular edema.

Six months later, 11 months after the onset of the CVO, the patientreported a slight improvement in vision, although measured visual acuity remainedunchanged at 20/160 in the right eye. No neovascularization was seen in theanterior segment. Ophthalmoscopic examination showed resolution of the intraretinalhemorrhages. However, a new subretinal hemorrhage was seen in the macula withfibrovascular tissue that just extended into the fovea from the superonasalaspect of the macula. There was cystoid macular edema at the inferotemporalaspect of this lesion (Figure 3A).An early-phase frame from a fluorescein angiogram (Figure 3B) showed an anastomosis between a retinal arteriole andthe fibrovascular lesion, as well as an anastomosis between a retinal venuleand the lesion. Within the fibrovascular tissue, early, intense, and well-circumscribedhyperfluorescence with late leakage was seen. There also was late leakageof dye in a petalloid pattern at the inferotemporal aspect of the fibrovascularlesion corresponding to the cystoid macular edema (Figure 3C). A diagnosis of subfoveal, predominantly classic choroidalneovascularization (CNV) was made. Although the cause of the CNV was not clear,photodynamic therapy (PDT) was recommended because the lesion was subfovealand because it was predominantly classic. Extrapolation from the Treatmentof Age-Related Macular Degeneration With Photodynamic Therapy Investigation2 was judged applicable to this patient to reducethe risk of moderate and severe visual acuity loss compared with no treatment.The patient underwent PDT with verteporfin (Visudyne; Novartis Pharma AG,Basel, Switzerland), without complications.

Figure 3.
Image not available

A, Eleven months after the onsetof the central retinal vein occlusion, the diffuse intraretinal hemorrhageshave resolved. A new subretinal hemorrhage is seen with fibrovascular tissuethat extends just into the fovea from the superonasal aspect of the macula.Some cystoid macular edema is seen involving the inferotemporal aspect ofthe macula. B, Early-phase frame of a fluorescein angiogram showing an anastomosisof the retinal circulation with the fibrovascular lesion. C, Late-phase frameof the angiogram showing continued leakage of the choroidal neovascularization,with an additional area of hyperfluorescence inferotemporally, correspondingto cystoid macular edema.

Three months later, visual acuity was 20/250 in the right eye. Ophthalmoscopicexamination showed resolution of much of the subretinal hemorrhage but somesubretinal fibrosis (Figure 4A).Fluorescein angiography showed continued leakage from CNV and cystoid macularedema (Figure 4B and C). Becauseof the continued fluorescein leakage from the CNV, a second course of PDTwas performed. Seven months after the patient's first course of PDT and 18months after the recognition of his CVO, the visual acuity remained 20/250in the right eye. Ophthalmoscopic examination showed a decreased amount ofsubretinal fluid and more subretinal fibrosis. Some intraretinal hemorrhagesoverlay the subretinal fibrosis (Figure 5A). Angiography showed minimal fluorescein leakage from CNV, resolutionof the leakage from the cystoid macular edema, and staining around the fibrovascularlesion. The anastomoses between the fibrovascular tissue and the retina showedlittle flow of fluorescein (Figure 5Band C). With only little fluorescein leakage from the CNV, PDT was not repeated.

Figure 4.
Image not available

A, Color photograph of the posteriorpole of the right eye 8 months after central retinal vein occlusion and 3months after photodynamic therapy. The subretinal hemorrhage has largely resolvedand the choroidal neovascularization (CNV) is beginning to become fibrotic.B, Midphase frame of a fluorescein angiogram showing continued leakage fromthe CNV. C, Late-phase frame showing continued leakage from both the CNV andaccumulation of dye in a pattern of cystoid macular edema.

Figure 5.
Image not available

A, Color photograph of the posteriorpole of the right eye 18 months after central retinal vein occlusion and 7months after photodynamic therapy. There is decreased surrounding subretinalfluid; intraretinal hemorrhages overlay the subretinal fibrosis. B, Early-phaseframe of a fluorescein angiogram showing less obvious fluorescein fillingof the vessels anastomosing from the retina to the fibrovascular lesion. Surroundingthe hypofluorescent lesion is hyperfluorescence corresponding to atrophy ofthe retinal pigment epithelium. C, Late-phase angiogram showing only minimalleakage from the fibrovascular lesion, resolution of the cystoid macular edema,and staining around the lesion.


Documentation of development of macular CNV after CVO, with retinalarteriole or venous anastomosis to the CNV, is of interest because of therecent attention given to retinal vascular anastomoses to CNV lesions.37 Ourliterature search disclosed no previous reports of CNV occurring in youngpatients within a short time after a CVO. One report documented CNV occurringin an older individual years after a vein occlusion.8 Althoughthese 2 diseases (CNV and CVO) could occur in the same eye, independent ofeach other, a causal relationship appears to exist in this case. This is supportedby the following: (1) the absence of any other known causes of CNV in thisyoung patient; (2) the temporal relationship of developing CNV within 6 monthsafter documentation of the CVO; and (3) the retinal anastomoses to the CNVthat were observed at the initial presentation of the CNV.

What could be the relationship of the CVO to the CNV? One could postulatethat chronic edema of the retina led to progressive damage of the retinalpigment epithelium, with disruption to the Bruch's membrane, that was associatedwith the growth of CNV. It is also possible that some CNV lesions begin inthe retina and extend to the choroid, as has been documented previously inidiopathic parafoveal telangectasia9 andmore recently suggested in age-related macular degeneration.6 Increasedhydrostatic pressure in venovenous collaterals, secondary to the CVO, couldhave either initiated or potentiated the development of such anastomoses.As shown in Figure 3A, the retinalhemorrhages caused by the CVO largely disappeared at about the same time thatanastomoses between the fibrovascular lesion and the retina were first observed.This suggests the possibility that these spontaneously occurring anastomosesdecompressed the occluded retinal venous vasculature into the fibrovascularlesion and into the choroid. Such retinal venous decompression has been documentedtherapeutically, after intense laser photocoagulation designed to rupturethe Bruch's membrane and create anastomoses between the retina and choroid.10

Whatever the cause of the CNV, the PDT was associated with a diminutionof fluorescein leakage from CNV, despite the presence of anastomoses betweenthe retina and the fibrovascular tissue. The PDT appeared to have had littleimmediate effect on the retinal vessels, although the anastomotic vesselsbecame nonperfused over time. Regardless of the cause of the CNV after CVO,whether or not there is a causal relationship, we believe that this is thefirst well-documented case of CNV developing in a relatively young patientwith a preexisting CVO. However, retinal anastomoses and cystoid macular edemawere observed. In addition, PDT appeared to have a typical involutional effecton the fibrovascular lesion.

This study was supported by the Heed Foundation, Cleveland, Ohio (DrDiLoreto); the Ronald G. Michels Fellowship, Scarsdale, NY (Dr DiLoreto);the Herman Knapp Testimonial Fund, Cleveland (Dr DiLoreto); the Altsheler-DurrellFoundation, Louisville, Ky; and the Guerrieri Retinal Research Fund and WilmerMacular Degeneration Research Fund, The Johns Hopkins University, Baltimore,Md.

The Johns Hopkins University, Dr Bressler's employer, but not Dr Bressler,is paid for Dr Bressler to provide consultation to Novartis Ophthalmics andQLT Inc; neither company had any input into the design or completion of thisstudy. The terms of this institutional consultant agreement are managed byThe Johns Hopkins University in accordance with its conflict-of-interest policies.

Correspondence: Dr Bressler, The Wilmer Eye Institute, The JohnsHopkins University School of Medicine, 550 N Broadway, Suite 115, Baltimore,MD 21205-2002 (

Central Vein Occlusion Study Group, The Central Vein Occlusion Study Group M report: evaluation of gridpattern photocoagulation for macular edema in central vein occlusion. Ophthalmology. 1995;1021425- 1433
Treatment of Age-Related Macular Degeneration With Photodynamic Therapy(TAP) Study Group, Photodynamic therapy of subfoveal choroidal neovascularization in age-relatedmacular degeneration with verteporfin: two-year results of 2 randomized clinicaltrial—TAP Report 2. Arch Ophthalmol. 2001;119198- 207
Kuhn  DMeunier  ISoubrane  GCoscas  G Imaging of choroidal anastomoses in vascularized retinal pigment epitheliumdetachments. Arch Ophthalmol. 1995;1131392- 1398
Hartnett  MEWeiter  JJStaurenghi  GElsner  AE Deep retinal vascular anomalous complexes in advanced age-related maculardegeneration. Ophthalmology. 1996;1032042- 2053
Slakter  JSYannuzzi  LASchneider  U  et al.  Retinal choroidal anastomoses and occult choroidal neovascularizationin age-related macular degeneration. Ophthalmology. 2000;107742- 753discussion 753- 754
Yannuzzi  LANegrão  SIida  T  et al.  Retinal angiomatous proliferation in age-related macular degeneration. Retina. 2001;21416- 434
Axer-Seigel  RBourla  DPriel  EYassur  YWeinberger  D Angiographic and flow patterns of retina choroidal anastomoses in age-relatedmacular degeneration with occult choroidal neovascularization. Ophthalmology. 2002;1091726- 1736
Eandi  CMPiccolino  FCVentre  LGrignolo  FM Photodynamic therapy for choroidal neovascularization in eyes withvascular disorders. Retina. 2002;22495- 498
Gass  JDBlodi  BA Idiopathic juxtafoveolar retinal telangiectasis: update of classificationand follow-up study. Ophthalmology. 1993;1001536- 1546
Fekrat  SGoldberg  MFFinkelstein  D Laser-induced chorioretinal venous anastomosis for nonischemic centralor branch retinal vein occlusion. Arch Ophthalmol. 1998;11643- 52