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Clinicopathologic Reports, Case Reports, and Small Case Series
December 2002

Photodynamic Therapy in Adult-Onset Vitelliform Macular Dystrophy Misdiagnosed as Choroidal Neovascularization

Arch Ophthalmol. 2002;120(12):1761-1763. doi:

In adult-onset vitelliform macular dystrophy (AOVMD), the yellowish subretinal material accumulating at the fovea may cause late hyperfluorescence, both with fluorescein angiography and with indocyanine green angiography,1,2 sometimes simulating choroidal neovascularization (CNV). We report 3 cases of AOVMD misdiagnosed as classic CNV secondary to age-related macular degeneration that were treated with photodynamic therapy (PDT).

Report of Cases
Case 1

A 71-year-old woman reported vision loss in her right eye. Examination showed atrophy of the choriocapillaris, and yellowish subretinal material was seen in the left eye (Figure 1A). Best-corrected visual acuity was 20/200 OD and 20/30 OS. Fluorescein angiography showed early blocked fluorescence at the left macula, with perimacular stellate aspect and mild late hyperfluorescence (Figure 1B). Indocyanine green angiography also showed late hyperfluorescence that was also misinterpreted as CNV. Optical coherence tomography (OCT) revealed foveal hyperreflective elevation of the profile of the retinal pigment epithelium (RPE)–Bruch membrane–choriocapillaris complex (Figure 2A). The patient received PDT in her left eye. Three months later, the subretinal material had disappeared, leaving in place RPE mottling (Figure 1C); visual acuity was unchanged. Fluorescein angiography showed mild window defect at the left macula (Figure 1D). With OCT, the foveal profile was restored because of disappearance of the subretinal mound (Figure 2B). One year after PDT, visual acuity, as well as angiographic and OCT findings (Figure 1E and F, and Figure 2C), were substantially unchanged.

Figure 1.
A, Vitelliform submacular lesion
with red-free photograph in case 1 immediately before photodynamic therapy.
Middle-phase fluorescein angiogram shows a stellate hypofluorescent lesion
with mild foveal hyperfluorescence that was misdiagnosed as choroidal neovascularization,
B, Three months after photodynamic therapy, the vitelliform lesion had disappeared
(C and D); the picture was unchanged after 1 year of follow-up (E and F).
Visual acuity was 20/30 and remained stable throughout the follow-up.

A, Vitelliform submacular lesion with red-free photograph in case 1 immediately before photodynamic therapy. Middle-phase fluorescein angiogram shows a stellate hypofluorescent lesion with mild foveal hyperfluorescence that was misdiagnosed as choroidal neovascularization, B, Three months after photodynamic therapy, the vitelliform lesion had disappeared (C and D); the picture was unchanged after 1 year of follow-up (E and F). Visual acuity was 20/30 and remained stable throughout the follow-up.

Figure 2.
Case 1. Optical coherence tomography
before photodynamic therapy (A) shows a hyperreflective subretinal elevation
at the level of the fovea, with preservation of the physiological neuroepithelial
depression. Three months later, the subretinal deposits had disappeared, and
the retinal pigment epithelium profile was almost restored or rectilinear
(B), with no change after 1 year (C).

Case 1. Optical coherence tomography before photodynamic therapy (A) shows a hyperreflective subretinal elevation at the level of the fovea, with preservation of the physiological neuroepithelial depression. Three months later, the subretinal deposits had disappeared, and the retinal pigment epithelium profile was almost restored or rectilinear (B), with no change after 1 year (C).

Case 2

A 74-year-old woman had a vitelliformlike lesion in her left eye on clinical examination. The right eye displayed a roundish area of chorioretinal atrophy at the macula. The subretinal macular deposits showed early, stellate blocked fluorescence, and marked late leakage in the left eye. The left eye received PDT because of suspected CNV. Visual acuity in the left eye was 20/40 before PDT and throughout the following year, and no further PDT was applied. The angiographic aspect of the left macula was also stable.

Case 3

Bilateral macular RPE atrophy associated with reticular hyperpigmentation were seen in both fundi of a 65-year-old man. The left fovea had yellowish subretinal material causing early, stellate blocked fluorescence with fluorescein angiography, followed by abundant late leakage. Best-corrected visual acuity was 20/600 OD and 20/100 OS. The left eye received PDT because of suspected CNV. We obtained fluorescein angiograms as early as 1 month after PDT. Although the angiographic picture was substantially unchanged, we observed resorption of the yellowish subretinal material, and this aspect was maintained during the following months. Visual acuity was 20/100 soon after PDT, and was measured as 20/60 after 6 months. Thereafter, it declined to 20/400 because of dense subcapsular cataract. Cataract extraction was advised approximately 1 year after PDT.

Comment

Adult-onset vitelliform macular dystrophy is a subtype of pattern dystrophy that resembles Best dystrophy, in which smaller vitelliform lesions are usually seen in middle-aged or elderly individuals.1 Lipofuscin material on either side of the RPE, as well as basal laminar and basal linear deposits, were observed throughout the macula in a recent clinicopathologic correlation.3

We saw 3 patients in whom a vitelliform macular lesion had been treated with PDT because of misdiagnosed CNV. Photodynamic therapy did not seem to cause any adverse effects despite the fact that it was done in the setting of no CNV, no intraretinal fluid, and possibly compromised choriocapillaris. Visual acuity was stable or possibly improved in all 3 instances. In 2 of 3 cases, the vitelliform subretinal material disappeared within 1 to 3 months of PDT treatment. Although this may be seen in the long-term in some eyes with AOVMD, it could also be hypothesized that the rather rapid resorption of the subretinal material is due to RPE stimulation by PDT, which is known to cause RPE damage followed by regeneration experimentally.4,5

In conclusion, we showed that AOVMD can be misinterpreted as CNV. Only practitioners experienced in clinical examination and fluorescein interpretation of macular degeneration should be performing PDT.

Corresponding author: Ugo Menchini, MD, Department of Oto-Neuro-Ophthalmological Surgical Sciences, Eye Clinic II, University of Florence, Viale Morgagni 85, 50134 Florence, Italy (e-mail: ugo.menchini@unifi.it).

References
1.
Gass  JDM Pattern dystrophies of the RPE: adult-onset foveomacular vitelliform dystrophy. Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment 1 St Louis, Mo CV Mosby Co1997;316- 321
2.
Lanzetta  PVirgili  GMenchini  U Indocyanine green angiography in vitelliform macular lesions. Ophthalmologica. 1996;210189- 194Article
3.
Dubovy  SRHairston  RJSchatz  H  et al.  Adult-onset foveomacular pigment epithelial dystrophy: clinicopathologic correlation of three cases. Retina. 2000;20638- 649
4.
Schmidt-Erfurth  UHasan  TGragoudas  EMichaud  NFlotte  TJBirngruber  R Vascular targeting in photodynamic occlusion of subretinal vessels. Ophthalmology. 1994;1011953- 1961Article
5.
Lin  SCLin  CPFeld  JRDuker  JSPuliafito  CA The photodynamic occlusion of choroidal vessels using benzoporphyrin derivative. Curr Eye Res. 1994;13513- 522Article
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