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Year : 2016  |  Volume : 28  |  Issue : 3  |  Page : 201-204

Diagnostic dilemma in a macular lesion

Department of Cataract and Uvea, Giridhar Eye Institute, Ponneth Temple Road, Kadavanthra, Cochin, Kerala, India

Date of Web Publication2-May-2017

Correspondence Address:
Dr. N Sandhya
4-C Amity Park, Skyline Builders, Changampuzha Samadhi Road, Edappally, Cochin - 682 024, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0976-6677.205436

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How to cite this article:
Sandhya N. Diagnostic dilemma in a macular lesion. Kerala J Ophthalmol 2016;28:201-4

How to cite this URL:
Sandhya N. Diagnostic dilemma in a macular lesion. Kerala J Ophthalmol [serial online] 2016 [cited 2021 May 6];28:201-4. Available from: http://www.kjophthal.com/text.asp?2016/28/3/201/205436

A 30-year-old male presented to us with blurring of vision in the left eye of 1-week duration. His best-corrected visual acuity was 6/6, N6 in the right eye and 6/24, and N12 in the left eye. He had a history of fever with sore throat 2 weeks prior to that.

Anterior segment and intraocular pressures of both eyes were normal. Dilated fundus examination of the right eye was normal. Fundus examination of the left eye revealed a grayish white patch inferior to fovea surrounded by a yellow border which was thicker inferiorly [Figure 1].
Figure 1: Fundus photograph showing greyish white lesion inferior to fovea surrounded by an yellow border

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Fundus fluorescence angiography shows hypofluorescence corresponding to the grayish white patch with hyperfluorescent rim corresponding to the yellow border in the early phase, with leakage in late phase simulating a choroidal neovascular membrane [Figure 2] and [Figure 3].
Figure 2: Early phase of FA showing hypofluo-rescence corresponding to the greyish white patch with hyperflourescent

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Figure 3: Late phase of FA showing leakage from the lesion simulating CNVM

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Optical coherence tomography (OCT) images of the same patient showed the presence of vitreous hyper-reflective dots suggestive of vitreous cells. There was thickening of the retinal pigment epithelium (RPE) with overlying intraretinal edema in the inferior section [Figure 4].
Figure 4: OCT image showing presence of vitreous cells and thickening of RPE with overlying retinal oedema

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Fundus autofluorescence (FAF) of the same patient revealed central hypoautofluorescence corresponding to the central gray patch visible clinically and area of RPE thinning on the OCT. Hyperautofluorescence is noted in areas surrounding the lesion which revealed thickening of RPE on OCT [Figure 5].
Figure 5: FAF picture shows hypoautofluorescence corresponding to the central grey patch with hyperautofluorescent rim

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  What Are the Diagnostic Possibilities and the Management Strategies in This Case? Top

Dr. Amala E. George

Senior Consultant, Department of Uveitis, Sankara Nethralaya, Chennai, Tamil Nadu, India

This case illustrates the classic features of acute idiopathic maculopathy (AIM) and has excellent multimodal documentation. AIM typically affects young individuals and is usually unilateral though bilateral cases have been reported. Several reports point to an association with Coxsackievirus infection. The patient in this case report had a febrile illness with sore throat which could have been related to Coxsackievirus-associated hand, foot, and mouth disease (HFMD).[1] Outbreaks of HFMD have been reported in South India, with Coxsackievirus A16 being the most common serotype implicated.

The lesion shows a bull's eye pattern with central hypofluorescence and a hyperfluorescent rim. OCT evaluation confirms the presence of RPE involvement with overlying retinal edema. There is also a small foveolar detachment. The autofluorescence image shows the involvement of RPE-photoreceptor complex and this modality can be useful in following up the lesion. The condition is usually self-limiting with full visual recovery. The final vision depends on the severity and duration of this disruption of the RPE-photoreceptor complex.[2] The integrity of the inner-outer segment (IS-OS) junction is also important. A short course of oral steroids may be indicated in cases of unusually severe features but this has to be decided on a case-to-case basis.

Dr. Vinitha G. Rao

Sr. Consultant, Giridhar Eye Institute, Ernakulam, Kerala, India

The fundus photograph shows a well-defined area of retinal pigment alteration. The OCT shows the presence of inflammation in the posterior vitreous. In addition, it also shows RPE thickening with the overlying retinal edema. The hyperfluorescence in the FAF confirms that the disease is involving the RPE. Based on these, in a young patient, it appears to be AIM, which is usually unilateral. Unilateral acute idiopathic maculopathy (UAIM) is known to have a viral etiology and can have flu-like symptoms preceding the ocular complaints. Although the disease is self-limiting, it may benefit with a short course of systemic steroids.

One of the diseases that resemble UAIM is acute retinal pigment epitheliitis (ARPE). However, in ARPE, the fundus shows typical fine pigment stippling in the macular area or clusters of small round macular lesions with pigment spots, and the spectral domain-OCT shows a very localized thickening of the RPE with often a striking elongation of the outer segment of the photoreceptor, whereas in UAIM, it tends to be more diffuse thickening of RPE.

Dr. Natasha

Associate Professor, Amrita Institute of Medical Sciences, Kochi, Kerala, India

From the history and clinical features, the most probable diagnosis is AIM. The points in favor of the diagnosis in this case are preceding history of fever, acute decrease in vision, young individual, unilaterality, and the typical perifoveal grayish area with early hypofluorescence and late hyperfluorescence on FFA. This condition manifests as sensory neural detachment when it mimics a central serous retinopathy and sometimes intraretinal edema as in this case when it mimics a choroidal neovascular membrane. These are the main differentials and should be differentiated based on FFA and OCT features.

This condition is idiopathic although Coxsackievirus has been implicated as a causative agent and flu-like illness precedes the ocular involvement. It is self-limiting and undergoes spontaneous resolution. The prognosis is usually good and patients make a full visual recovery.

  Concluding Remarks Top

Dr. N. Sandhya

Sr. Consultant, Cataract and Uvea Services, Giridhar Eye Institute, Ernakulam, Kerala, India

A young gentleman presented to us with unilateral sudden decrease in vision of short duration. The differential diagnosis includes lesions affecting the outer retina such as UAIM, ARPE, acute macular neuroretinopathy, and placoid lesion of acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Multimodal imaging helps to differentiate these conditions.

AIM classically presents with irregular, white, gray, or yellow thickening of the RPE. Rarely, neurosensory detachment has also been noted.[3],[4],[5] Mild vitritis may or may not be present. FFA shows irregular early-phase hypofluorescence with hyperfluorescent rim. Late phase shows hyperfluorescence with staining pattern. OCT reveals either a thin layer of hyper-reflective material or edematous RPE protruding into the IS-OS junction, pseudopod-like extension of the subretinal exudation, or retinal thickening overlying the lesion which reduces as exudation and edema get better. There can also be loss of IS-OS in perifoveal distribution and accumulation of hyper-reflective material at the level of RPE-photoreceptor complex [Figure 6]. ARPE shows multiple clusters of discrete, small, dark-gray spots at macular area at the level of RPE, usually in clusters of 1–4. Each spot is small and is surrounded by yellow-gray depigmented area. FFA may be normal or shows hypofluorescence corresponding to lesion surrounded by hyperfluorescence [Figure 7]. Acute macular neuroretinopathy shows wedge-shaped, reddish-brown lesions pointing toward the foveal center. Here, the FFA is usually normal [Figure 8]. APMPPE is usually bilateral and there are multiple, yellowish-gray placoid lesions in the outer retina. FFA shows the typical early hypofluorescence and late staining of placoid lesions [Figure 9]. Resolving fibrinous central serous chorioretinopathy and choroidal neovascular membrane in the young may also be confused with the fundus appearance. In these, there is no vitreous inflammation and the FFA findings are classic.
Figure 6: Fundus picture – unilateral acute idiopathic maculopathy

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Figure 7: Fundus showing acute retinal pigment epithelitis

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Figure 8: Fundus picture showing wedge-shaped reddish brown lesions of acute macular neuroretinopathy

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Figure 9: Fundus picture showing multiple yellowish grey placoid lesions of APMPPE

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In this case, the fundus picture, FFA and OCT findings were characteristic of AIM and the patient was given a short course of oral steroids. His visual acuity improved to 6/9, N6P after treatment.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Beck AP, Jampol LM, Glaser DA, Pollack JS. Is coxsackievirus the cause of unilateral acute idiopathic maculopathy? Arch Ophthalmol 2004;122:121-3.  Back to cited text no. 1
Jung CS, Payne JF, Bergstrom CS, Cribbs BE, Yan J, Hubbard GB 3rd, et al. Multimodality diagnostic imaging in unilateral acute idiopathic maculopathy. Arch Ophthalmol 2012;130:50-6.  Back to cited text no. 2
de la Fuente MA, Cuadrado R. Unilateral acute idiopathic maculopathy: Angiography, optical coherence tomography and microperimetry findings. J Ophthalmic Inflamm Infect 2011;1:125-7.  Back to cited text no. 3
Haruta H, Sawa M, Saishin Y, Ohguro N, Tano Y. Clinical findings in unilateral acute idiopathic maculopathy: New findings in acute idiopathic maculopathy. Int Ophthalmol 2010;30:199-202.  Back to cited text no. 4
Nicolo M, Rosa R, Musetti D, Musolino M, Traverso CE. Early swept-source optical coherence tomography angiography findings in unilateral acute idiopathic maculopathy. Ophthalmic Surg Lasers Imaging Retina 2016;47:180-2.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]


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