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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
Report of Cases
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
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.
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).
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.
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
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
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: email@example.com).
Menchini U, Giacomelli G, Cappelli S, Giansanti F, Romani A. Photodynamic Therapy in Adult-Onset Vitelliform Macular Dystrophy Misdiagnosed as Choroidal Neovascularization. Arch Ophthalmol. 2002;120(12):1761–1763. doi:
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