Idiopathic Multifocal Choroiditis/Punctate Inner Choroidopathy With Acute Photoreceptor Loss or Dysfunction Out of Proportion to Clinically Visible Lesions

Abstract

Purpose To report acute/subacute vision loss and paracentral scotomata in patients with idiopathic multifocal choroiditis/punctate inner choroidopathy (MFC/PIC) due to large zones of acute photoreceptor attenuation surrounding the chorioretinal lesions. Methods Multimodal-imaging case-series Results Six females and 2 males were included (mean age 31.5±5.8 years). Vision ranged from 20/20-1 to hand motion (mean 20/364). SD-OCT demonstrated extensive attenuation of the external limiting membrane (ELM), ellipsoid and interdigitation zones, adjacent to the visible MFC/PIC lesions. The corresponding areas were hyperautofluorescent on fundus-autofluorescence (FAF), and were associated with corresponding visual field defects. Full-field ERG (available in 3 cases) showed markedly decreased cone/rod response and multifocal ERG revealed reduced amplitudes and increased implicit times in 2 cases. Three patients received no treatment, the remaining were treated with oral corticosteroids (n=4), oral acyclovir/valacyclovir (n=2), intravitreal/posterior subtenon triamcinolone-acetate (n=3) and anti-VEGF (n=2). Visual recovery occurred in only 3 cases, of whom 2 were treated. Varying morphological recovery was found in 6 cases, associated with decrease in hyperautofluorescence on FAF. Conclusions MFC/PIC can present with transient or permanent central photoreceptor attenuation/loss. This presentation is likely a variant of MFC/PIC with chorioretinal atrophy. Associated changes are best evaluated using multimodal imaging.

New associations of classic acute macular neuroretinopathy

“…However one previous report detected AMN lesions presenting with subtle hyperautofluorescence on 488 nm FAF similar to our case 17. Although this finding is not characteristic for AMN, it can be explained by the reduction of filtering of blue light by decreased visual pigment density in outer segments, seen on OCT as loss/disruption of the IZ 18 19. Alternatively, as suggested by Lalezary et al ,15 this lesion may represent co-localisation of the deeper retinal haemorrhage and AMN.…”

Multifocal choroiditis with retinal vasculitis, optic neuropathy, and keratoconus in a young Saudi male

“…Recent reports have identified MFC/PIC variant as a separate nosologic entity. [ 3 6 ] Imaging studies have shown that MFC/PIC can have adjacent photoreceptor attenuation of variable severity including chorioretinal atrophic lesions, central photoreceptor loss, and outer (zonal) retinal atrophy. [ 7 ] Our case shares OCT features that would certainly fall into that entity.…”

“…The characteristic inflammatory changes of the choroid, choriocapillaris, and retinal pigment epithelium (RPE) are yellow-white foci beneath the retina. [ 1 2 ] Other ocular complications, including choroidal neovascular membrane[ 2 3 ] and optic neuropathy,[ 4 ] can further decrease vision. Herein, we describe an unusual association and treatment of idiopathic MFC with optic neuropathy, retinal vasculitis, and keratoconus in a young male from Saudi Arabia.…”

Idiopathic multifocal choroiditis

“…[13] Foveal sparing is common until late in the disease course in these patients, and the majority of patients maintain good visual acuity. Munk et al,[22] however, reported eight patients with idiopathic MFC with atrophy associated with adjacent photoreceptor attenuation, an acute/subacute onset of decreased visual acuity and scotomata with vision loss as severe as counting fingers/hand motion. Visual acuity fully recovered in only three of the eight cases after treatment with corticosteroids and/or systemic mycophenolate mofetil (MMF) but no other agents such as cyclosporine, methotrexate, azathioprine, infliximab, or tacrolimus were used.…”

“…Visual acuity fully recovered in only three of the eight cases after treatment with corticosteroids and/or systemic mycophenolate mofetil (MMF) but no other agents such as cyclosporine, methotrexate, azathioprine, infliximab, or tacrolimus were used. [22]…”