Open Journal of Ophthalmology, 2012, 2, 119-121
http://dx.doi.org/10.4236/ojoph.2012.24026 Published Online November 2012 (http://www.SciRP.org/journal/ojoph) 119
Optical Coherence Tomography Findings in a Case of
Unilateral Acute Idiopathic Maculopathy
José Andonegui*, José Ramón Maya, Ane Zurutuza, Mónica Pérez de Arcelus
Ophthalmology Department, Complejo Hospitalario de Navarra, Pamplona, Spain.
Received July 8th, 2012; revised September 18th, 2012; accepted October 5th, 2012
ABSTRACT
Purpose: To describe the clinical, angiographic, and Optical Coherence Tomography (OCT) findings in a patient with
Unilateral Acute Idiopathic Maculopathy (UAIM) and to discuss a possible pattern of OCT findings in patients with this
disease. Methods: Case r eport. Results: A 34-year-old man with UAIM was evaluated. During the acute phase fundus
examination revealed a yellow thickening in the macular area of the right eye, Fluorescein Angiography (FA) showed
early alternating patches of hypofluorescence and hyperfluorescence and late hyperfluorescence and OCT showed
thickening of the outer retina in the foveal zone. Six weeks later, the macula had pigmentary mottling, FA showed an
area of central hypofluorescence surrounded by a hyperfluorescent ring that remained unchanged during the angiogram
and the OCT examination was normal. Conclusion: OCT is a useful diagnostic tool for UAIM. OCT evaluation of pa-
tients with this disease will exhibit the heterogeneous hyperreflectivity and thickening of the outer retina and Retinal
Pigment Epithelium (RPE). A serous macular detachment may not be a prominent finding in UAIM.
Keywords: Unilateral Acute Idiopathic Maculopathy; Optical Coherence Tomography
1. Introduction
Unilateral Acute Idiopathic Maculopathy (UAIM), a rare
clinical entity of unknown etiology, affects otherwise
healthy young adults who present with sudden loss of cen-
tral vision sometimes preceded by a viral illness or flu-
like symptom s. Pat ient s usual ly experience complete spon-
taneous resolution without treatment within few weeks
after the onset [1,2]. Since the initial description of this
disease by Yanuzzi et al. in 1991, very few cases have
been reported in the ophthalmic literature, and most of
these cases were reported befo r e the introductio n of Opti-
cal Coherence Tomography (OCT) as a routine tool for
diagnosing retinal pathology. We describe the clinical,
angiographic, and OCT findings in a patient with UAIM.
A possible pattern of OCT findings in patients diagnosed
with UAIM is discussed.
2. Case Report
A 34-year-old man presented with a 4-day history of
Visual Acuity (VA) loss in his right eye. A few days pre-
viously he had had an upper respiratory tract infection.
The VA was 0.2 in his right eye and 1.0 in his left eye.
The anterior segment examination was unremarkable bi-
laterally. The fundus examination revealed a yellow
thickening in the macular area of the right eye (Figure 1)
and a normal macula in the left eye. Fluorescein An-
giography (FA) showed early alternating patches of hy-
pofluorescence and hyperfluorescence and late hyper-
fluorescence in the right eye (Figure 2) and a normal left
eye. OCT showed thickening of the outer retina in the
foveal zone of the right eye (Figure 3); the left eye was
unremarkable. A diagnosis of UAIM was established and
no treatment was initiated.
Six weeks later, the VA returned to 1.0 in th e right eye.
The macula had pigmentary mottling (Figure 1). FA
showed an area of central hypofluorescence surrounded
by a hyperfluorescent ring that remained unchanged dur-
ing the angiogram (Figure 2). The OCT examination was
normal (Figure 3).
3. Discussion
UAIM is an inflammatory process involving the outer re-
tina and Retinal Pigment Epithelium (RPE). The condi-
tion is considered to be idiopathic, although a possible
association with Coxsackie virus was hypothesized [3].
In most cases, the involvement is unilateral, but bilateral
lesions have also been report ed [2,4] .
The diagnosis of UAIM had been previously based on
fundus examination and FA findings. More recently,
OCT was introduced as a diagnostic tool in this entity.
*Corresponding a uthor.

Optical Coherence Tomograph y Findings in a Cas e of Unilateral Acute Idiopathic Maculopathy 120
Figure 1. (A) Yellow thickening of the macular area is seen
in the acute disease phase; (B) Pigmentary mottling of the
macula is seen 6 weeks after onset.
Figure 2. FA in the acute phase (A-C) and 6 weeks after (D,
E). (A) Early phase of the angiogram showing a central area
of hypofluorescence and a peripheral ring of hyperfluores-
cence; (B) A progressive increase of the fluorescence is seen;
(C) A late-phase of the angiogram shows marked hyper-
fluorescence; (D) An early-phase angiogram revealing a
central hypofluorescent area surrounded by a hyperfluo-
rescent ring; (E, F) The angiographic pattern remains un-
changed in the late phases.
Regarding the clinical findings on fundus examinations,
a serous neurosensory detachment with an irregular white,
yellow, or gray thickening of the overlying RPE has been
typically described in most patients [1,2]. Papillitis, sub-
retinal exudation, intraretinal hemorrhages, or vitreous
cells have also been observed [2]. After resolution, pig-
ment epithelial atrophic changes and irregular pigmenta-
tion in the area of the in itial macular involvement can be
detected.
Angiographic findings in UAIM have been reported as
early irregular hyperfluorescence alternating with areas
of hypofluorescence and late complete staining of the
lesion in the acute disease phase [1,2]. These angio-
Figure 3. (A) An OCT image in the acute phase showing
hyperreflectivity and thickening of the outer retina in the
foveal zone; (B) The OCT image is unremarkable 6 weeks
later.
graphic changes have been considered to be similar to the
angiographic features of occult choroidal neovascular
membranes [1]. Once improvement has occurred, the an-
giographic pattern appears as a central area of hypofluo-
rescence due to blockage by the RPE hyperpigmentation
surrounded by a hyperfluorescent zone of RPE atrophy.
This angiographic configuration corresponds to a bull’s-
eye maculopathy.
The descriptions of OCT findings in UAIM in the
ophthalmic literature have been scanty and heterogeneous.
A Medline search identified only seven cases of UAIM
evaluated using OCT [4-10]. Gupta et al. [5] reported a
patient with a prominent serous macular detachment, but
the fluorescein angiogram of this patient was not charac-
teristic of UAIM, because it lacked the characteristic
early hypofluorescence and hyperfluorescence and late
hyperfluorescen ce described in this con dition. Ghazi et al.
[4] also described an atypical case characterized by a
macular hole in one eye and a small extrafoveal serous
detachment of the neurosensory retina in the fellow eye.
This case also lacked the hyperfluorescence pattern in the
angiogram. Another case with serous detachment re-
ported by Xu et al. [6] also presented an atypical an-
giogram and the atypical feature of recurrence of the
disease. Only the patient reported by Haruta et al. [7] had
a serous detachment on OCT and a typical angiographic

Optical Coherence Tomograph y Findings in a Cas e of Unilateral Acute Idiopathic Maculopathy 121
pattern. The patients reported by Aggio et al. [8], de la
Fuente et al. [9] and Ooto et al. [10] closely resembled
the current patient. These patients exhibited the typical
clinical and angiographic features of UAIM, but no sub-
retinal fluid was seen on the OCT images.
OCT has helped to achieve a better understanding of
many retinal diseases including UAIM. This entity con-
sists of an inflammatory process of unknown etiology
involving the outer retina and RPE that breaks down the
inner or outer hemato-retinal barriers and intraretinal
exudation in the outer retina responsible for the visual
loss, the hyperfluorescence observed in FA, and the
thickening detected on OCT. According to the results of
the current OCT evaluation and the ones described by
Aggio et al. [8] de la Fuente et al. [9] and Ooto et al. [10],
we hypothesized that a neurosensory macular detachment
is not a prominent feature of UAIM. All these were typi-
cal cases of UAIM, and in all of them OCT showed
heterogeneous hyperreflectivity and thickening of the
outer retina and RPE without a sensory macular detach-
ment. Before introduction of OCT, a neurosensory ma-
cular detachment was reported in most patients with
UAIM. However, a serous macular detachment can be
difficult to detect during a fundus examination unless it is
very prominent. FA in these patients characteristically
showed early irregular hypofluorescence and hyperfluo-
rescence followed by late hyperfluorescence of the entire
lesion. This angiographic pa ttern is more characteristic of
an exudative process of the outer retina than of a serous
macular detachment, characterized by smooth early hy-
perfluorescence with a slight increase in the late phases.
We concluded that OCT is useful for di agnosi ng UAIM .
Even thought more cases are needed to confirm our hy-
pothesis, we believe that an OCT evaluation of patients
with this disease will exhibit the heterogeneous hyper-
reflectivity and thickening of the outer retin a and RPE. A
serous macular detachment may not be a prominent find-
ing in UAIM.
REFERENCES
[1] L. A. Yanuzzi, L. M. Jampol, M. F. Rabb, J. A. Sorenson,
C. Beyrer and L. M. Wilcox, “Unilateral Acute Idiopathic
Maculopathy,” Archives of Ophthalmology, Vol. 109, No.
10, 1991, pp. 1411-1416.
doi:10.1001/archopht.1991.01080100091049
[2] K. B. Freund, L. A. Yanuzzi, G. R. Barile, R. F. Spaide, S.
A. Milewski and D. R. Guyer, “The Expanding Clinical
Spectrum of Unilateral Acute Idiopathic Maculopathy,”
Archives of Ophthalmology, Vol. 114, 1996, pp. 555-559.
doi:10.1001/archopht.1996.01100130547007
[3] A. P. Beck, L. M. Jampol, D. A. Glasser and J. S. Pollack,
“Is Coxsackievirus the Cause of Unilateral Acute Idio-
pathic Maculopathy,” Archives of Ophthalmology, Vol.
122, No. 1, 2004, pp. 121-123.
doi:10.1001/archopht.122.1.121
[4] N. G. Ghazi, A. Daccache and B. P. Conway, “Acute
Idiopathic Maculopathy. Report of a Bilateral Case Mani-
festing a Macular Hole,” Ophthalmology, Vol. 114, No. 5,
2007, pp. e1-e6. doi:10.1016/j.ophtha.2006.08.055
[5] A. Gupta, S. Rogers and B. N. Matthews, “Unilateral
Acute Idiopathic Maculopathy,” British Journal of Oph-
thalmology, Vol. 93, No. 8, 2009, pp. 1073-1074.
doi:10.1136/bjo.2008.140335
[6] H. Xu and P. Lin, “Unilateral Recurre nt Acute Idiopat hic
Maculopathy,” Graefe’s Archive for Clinical and Experi-
mental Ophthalmology, Vol. 249, No. 6, 2011, pp. 941-
944. doi:10.1007/s00417-010-1589-1
[7] H. Haruta, M. Sawa, Y. Saishin, N. Ohguro and Y. Tano,
“Clinical Findings in Unilateral Acute Idiopathic Reti-
nopathy. New Findings in Acute Idiopathic Maculopa-
thy,” International Ophthalmology, Vol. 30, No. 2, 2010,
pp. 199-202. doi:10.1007/s10792-009-9299-6
[8] F. B. Aggio, M. E. Farah, R. L. Meirelles and E. C. de
Souza, “Stratusoct and Multifocal ERG in Unilateral
Acute Idiopathic Maculopathy,” Graefe’s Archive for Cli-
nical and Experimental Ophthalmology, Vol. 244, No. 4,
2006, pp. 510-516. doi:10.1007/s00417-005-1172-3
[9] M. A. de la Fuente and R. Cuadrado, “Unilateral Acute
Idiopathic Maculopathy: Angiography, Optical Coher-
ence Tomography and Microperimetry Findings,” Jour-
nal of Ophthalmic Inflammatio n and Infection, Vol. 1, No.
3, 2011, pp. 125-127. doi:10.1007/s12348-010-0014-6
[10] S. Ooto, M. Hangai and N. Yoshimura, “Photoreceptor
Restoration in Unilateral Acute Idiopathic Maculopathy
on Adaptive Optics Scanning Laser Ophthalmoscopy,”
Archives of Ophthalmology, Vol. 129, No. 12, 2011, pp.
1633-1635.doi:10.1001/archophthalmol.2011.345