Multimodality diagnostic imaging in unilateral acute idiopathic maculopathy

Abstract

Objective: To describe the clinical features and imaging characteristics in unilateral acute idiopathic maculopathy.

Methods: Retrospective review of 4 patients with a diagnosis of unilateral acute idiopathic maculopathy. Clinical characteristics (age, symptoms, Snellen visual acuity, and funduscopic features) and images from spectral-domain optical coherence tomography, fundus autofluorescence, fluorescein angiography, and indocyanine green angiography were analyzed.

Results: The median (range) age at presentation was 31 (27-52) years. The median (range) interval between symptom onset and presentation was 4 (1-20) weeks. Associated systemic findings included a viral prodrome (50%), orchitis (50%), hand-foot-mouth disease (25%), and positive coxsackievirus titers (50%). The median (range) visual acuity at initial examination was 20/400 (20/70 to 1/400), which improved to 20/30 (20/20 to 20/60) at final follow-up. The median (range) follow-up time was 8 (8-13) weeks. Early in the disease course, the central macula developed irregular, circular areas of white-gray discoloration. Following recovery, the macula had a stippled retinal pigment epithelium characterized by rarefaction and hyperplasia. Fluorescein angiography demonstrated irregular early hyperfluorescence and late subretinal hyperfluorescence. Spectral-domain optical coherence tomography showed a partially reversible disruption of the outer photoreceptor layer. Fundus autofluorescence initially revealed stippled autofluorescence that eventually became more hypoautofluorescent. Indocyanine green angiography showed "moth-eaten"-appearing choroidal vasculature, suggestive of choroidal inflammation.

Conclusions: The imaging characteristics highlight the structural changes during the active and resolution phases of unilateral acute idiopathic maculopathy. The visual recovery correlates with structural changes and suggests that the pathogenesis involves inflammation of the inner choroid, retinal pigment epithelium, and outer photoreceptor complex that is partially reversible.

Figure 1

The color fundus photograph of the affected right eye (A) one day after symptom onset demonstrates an irregular, circular area of grey-white discoloration in the macula. (B) Late-phase fluorescein angiogram image shows a large area of intense subretinal hyperfluorescence consistent with leakage and a smaller central area of pooling coinciding with an exudative detachment of the neurosensory retina. (C) TD-OCT shows a small subfoveal neurosensory detachment with hyperreflective debris at the apical side of the retinal pigment epithelium.

Figure 2

(A) Fundus photography in patient 1 one week after symptom onset showing granular hyperpigmention of the retina and retinal pigment epithelium. (B) Fluorescein angiography demonstrates late staining and less leakage in the nasal macula compared to the patient’s prior angiogram. (C) ICG angiography shows irregular “moth-eaten” choroidal vascular appearance with dilated choroidal vessels underlying the perifoveal region. (D) FAF shows a stippled autofluorescense in the macula areas extending to the optic nerve. E. SD-OCT reveals disruption and irregularity of the photoreceptor layer, subtle hyporeflectivity at the foveal outer segment area, and hyper-reflective material on the apical side of the retinal pigment epithelium.

Figure 3

Color fundus photograph, FAF, and SD-OCT findings in patient 1 at one week (A) and two months (B) after symptom onset. There is a loss of the grey-white discoloration and an increase in retinal pigment hyperplasia later in the disease course. The FAF shows less stippled autofluorescent areas (A, upper right) that evolve to a more stellate shaped autofluorescence with loss of background autofluorescence (B, upper right). SD-OCT shows hyporeflectivity of the outer photoreceptor layer (A, arrows), that are partially restored (B, arrowheads) at the twomonth follow-up visit. Note that the external limiting membrane remains intact in both A and B, yet the apical debris on the RPE has diminished.

Figure 4

Six weeks after symptom onset in patient 2, there is increased retinal pigment hyperplasia (A) and late subretinal staining on FA (B and C). FAF (D) shows a stippled pattern of autofluorescence, as well as loss of background autofluorescence. The SD-OCT (E) reveals disruption and irregularity of the outer photoreceptors and debris on the apical side of the RPE.

Figure 5

Fundus imaging in patient 3 one week after symptom onset reveals a grayish discoloration of the retina within the macula (A) and subretinal staining on FA (B). Seven weeks after symptom onset, there is increased retinal pigment hyperplasia (C).