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Marion Ronit Munk, Jesse J Jung, Kristin Biggee, Ursula Schmidt-Erfurth, William R Tucker, H Nida Sen, Amani A Fawzi, Lee M Jampol; Idiopathic multifocal choroiditis (MFC)/punctate inner choroidopthy (PIC) with acute photoreceptor loss out of proportion to clinically visible lesions. Invest. Ophthalmol. Vis. Sci. 2014;55(13):2856.
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To report 7 cases (8 eyes) of idiopathic multifocal choroiditis/punctate inner choroidopathy(MFC/PIC) with small discrete chorioretinal lesions surrounded by a larger zone of acute photoreceptor (PR) irregularity and loss.
A retrospective multimodal imaging case series
6 females and 1 male were included (mean age at disease-onset 31.7±6.4, range 23-42 years). Visual acuity at presentation was as severe as hand-motion in the setting of widespread central vision and visual field loss, not explained by the ophthalmoscopically visible lesions. No vitreous cells were present. SD-OCT demonstrated extensive loss/irregularity of the ellipsoid zone, interdigitation zone and the external limiting membrane (ELM) not limited to the immediate location of the MFC/PIC lesions. The corresponding areas of PR loss were hyper-autofluorescent on blue (488nm) fundus autofluorescence (FAF) and near-infrared (788nm)-FAF, while the MFC lesions were hypo-autofluorescent. Indocyanine and fluorescein-angiogram showed only the MFC/PIC lesions. Scotomata detected in visual field testing corresponded to the areas of PR changes. In 4 cases a full-field ERG was performed, which showed a markedly decreased cone and rod response. Four patients were treated with oral corticosteroids, 1 patient with intravitreal triamcinolone and 2 patients additionally with intravitreal bevacizumab injections. The photoreceptor status improved in a single case only, while another case experienced worsening of the visual field defect/PR loss during the observational period.
MFC/PIC can present with secondary permanent or transient PR loss. Severity and extent of MFC-associated changes are best evaluated using a multimodal imaging approach. This dramatic presentation of PR-loss is likely a variant of MFC with chorioretinal atrophy, recently described by Jung et al.
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