Unilateral Acute Idiopathic Maculopathy

KC is a 21 year old African-American male with no past ocular history who presented to Dr. Lundgren at VCS with acute painless loss of central vision in the right eye. The patient endorsed a recent history of exposure to Hand-Foot-Mouth Disease. Dr. Lundgren performed a complete ocular exam including dilated fundus examination. He noted an abnormal appearance of the macula on clinical exam and referred the patient to the retina subspecialty clinic for further evaluation and management.

At the time of the patient’s initial visit to the retina clinic, the patient’s visual acuity was 20/400 in the right eye and 20/20 in the left eye without correction. Intraocular pressures were within normal limits. Pupils were equal, round and reactive to light bilaterally with no APD. Extraocular motility, alignment, and confrontational visual fields were within normal limits. Anterior segment examination was normal in both eyes. Posterior segment exam of the right eye revealed a circumscribed area of pigmentary changes in the central macula (see image 1). Peripheral exam was within normal limits. The posterior segment exam of the left eye was unremarkable.

Macular OCT was ordered and revealed a loss of distinguishable the ellipsoid layer and cone outer-segment tips (COST) lines, as well as central heterogenous hyperreflective thickening at the level of the outer retina and retinal pigment epithelium in the right eye. Overall central retinal thickness was somewhat thinned, with an average measurement of 210 microns. OCT of the left eye was within normal limits, including normal central retinal thickness. Based on the above clinical findings and the patient’s recent diagnosis of Hand-Foot-Mouth Disease, a diagnosis of Unilateral Acute Idiopathic Maculopathy (UAIM) was made. Conservative management was recommended for this patient, including observation and maintaining good hydration.

UAIM was first described by Yannuzzi, et al. in 1991 in a case series of 9 patients presenting with acute unilateral central vision loss followed by spontaneous resolution. Visual acuity on presentation is typically 20/200 or worse, as was the case for our patient. Early funduscopic findings include unilateral circumscribed pigmentary changes in the central macula. These changes are sometimes associated with localized serous detachment of the neurosensory retina. UAIM has been found to be associated with coxsackie virus infection. Patient’s often report a flu-like illness prior to symptoms of UAIM. Hand-foot-mouth disease, orchitis, and epididymitis are the most common manifestations of coxsackie virus infection in young adults. UAIM is a rare disease that should be considered as part of the differential diagnosis in patients with acute monocular central vision loss and a recent history of flu like illness.

KC was seen 2 weeks after presentation, and again 4 weeks thereafter. Visual acuity improved to 20/20 in the affected eye. Persistent but improved pigmentary changes of the central macula were noted clinically. Additionally, follow-up macular OCT of the right eye demonstrated clearly distinguishable outer retinal layer architecture, though some degree of thickening and irregularity of the retinal pigment epithelial signal remained at last visit.