Optical Coherence Tomography Findings in a Case of Unilateral Acute Idiopathic Maculopathy

doi:10.4236/ojoph.2012.24026

Paper Menu >>

Journal Menu >>

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.

Email: *[email protected]

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;

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