Multimodality diagnostic imaging in unilateral acute idiopathic maculopathy.
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.
Department of Vitreoretinal Surgery and Disease, Emory University, Atlanta, Georgia, USA.
Cecilia S
CS
Jung
John F
JF
Payne
Chris S
CS
Bergstrom
Blaine E
BE
Cribbs
Jiong
J
Yan
G Baker
GB
Hubbard
Timothy W
TW
Olsen
Steven
S
Yeh
D000208:Acute Disease; D000328:Adult; D002829:Choroid / Q000473:pathology; D004596:Electroretinography; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D007208:Indocyanine Green; D008266:Macula Lutea / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009887:Ophthalmoscopy; D012164:Retinal Diseases / Q000175:diagnosis*; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence; D014792:Visual Acuity / Q000502:physiology
D007208:Indocyanine Green