Unilateral acute maculopathy associated with adult onset hand, foot and mouth disease: case report and review of literature

Abstract

Background: Acute maculopathy is a rare condition of unknown aetiology and Coxsackie virus is known to be associated with this macular chorioretinitis.

Findings: We report a case of acute unilateral maculopathy in a 35-year-old woman with concurrent hand foot and mouth disease. Furthermore, we display multimodal imaging (colour fundus photographs, autofluorescence, spectral domain ocular coherence tomography, fluorescein angiography and indocyanine green angiography) charting the course of the disease. The source of the virus was thought to be the patient's child. Empirical treatment with oral corticosteroids was commenced and the inflammation resolved, leaving a residual macular scar.

Conclusions: We present this case combined with the review of literature of adult onset Coxsackie-virus-associated retinitis. This case reiterates the fact that Coxsackie virus is an uncommon but important consideration in the differential diagnosis of chorioretinitis and posterior uveitis with atypical retinopathy.

Keywords: Acute maculopathy; Autofluorescence; Coxsackie virus; Hand; Posterior uveitis; foot and mouth disease (HFMD).

Figure 1

Clinical profile and ancillary investigations at baseline. Color fundus photography demonstrates the presence of irregular, ill defined, circular area of white grey discoloration of the right macula with intraretinal haemorrhage, intraretinal thickening and retinal pigmentary changes temporal to the fovea (A). Infrared imaging showing the presence of a figure of eight or dumb-bell-shaped lesion with disruption of the inner segment-outer segment (IS-OS) junction and hyperreflective debris at the apical side of the retinal pigment epithelium on the corresponding spectral domain ocular coherence tomography (SD-OCT) (B). Fundus fluorescein angiography showing a large area of intense subretinal hyperfluorescence indicating significant macular leakage (C) along with a small central area of pooling (yellow arrow) with no extra-macular abnormalities and no vasculitis. Indocyanine green angiography demonstrating irregular heterogenous patches of blocked fluorescence in the corresponding area (D).

Figure 2

Follow-up investigations. Funds fluorescein angiography (A) demonstrates late staining with reduced leakage, and indocyanine green angiography (B) illustrates irregular ‘moth-eaten’ choroidal vasculature with tortous choroidal vessels around the foveal region. A residual scar remained in the right macula with intraretinal pigmentary change and resolution of subretinal fluid with a subretinal fibrotic scar on SD-OCT (C). There is a presence of disrupted irregular photoreceptor layer with hyperreflective debris on the apical side of the retinal pigment epithelium.

Figure 3

Autofluorescence images. Autofluorescence at baseline revealed a irregular hypo-autofluorescent area with a strip of hyperautofluorescence at temporal edge (A), which over the next 4 days became morphologically similar to a dumb-bell-shaped maculopathy with increasing hyperautofluorescence at the rim of hypo-autofluorescent lesion (B). The lesion showed more stippled hyperautofluorescence at 1 month (C) with constricting lesion and loss of background fluorescence on subsequent follow-up at 2 months (D) and at 4 months (E).