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PHOTO ESSAY
Year : 2017  |  Volume : 65  |  Issue : 9  |  Page : 865-866

Multimodal imaging in dominant cystoid macular dystrophy


Vitreoretina Sciences, Aditya Birla Sankara Nethralaya, Kolkata, West Bengal, India

Date of Submission29-Apr-2017
Date of Acceptance30-May-2017
Date of Web Publication14-Sep-2017

Correspondence Address:
Rupak Roy
Aditya Birla Sankara Nethralaya, No. 147, Mukundapur, EM Bypass, Kolkata - 700 099, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_328_17

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  Abstract 


Keywords: Dominant cystoid macular dystrophy, multimodal imaging, spectral domain optical coherence tomography


How to cite this article:
Roy R, Saurabh K, Bhattacharyya S, Thomas NR, Datta K. Multimodal imaging in dominant cystoid macular dystrophy. Indian J Ophthalmol 2017;65:865-6

How to cite this URL:
Roy R, Saurabh K, Bhattacharyya S, Thomas NR, Datta K. Multimodal imaging in dominant cystoid macular dystrophy. Indian J Ophthalmol [serial online] 2017 [cited 2020 Feb 16];65:865-6. Available from: http://www.ijo.in/text.asp?2017/65/9/865/214642

Dominant cystoid macular dystrophy (DCMD) is a rare inherited retinal disorder. It primarily affects the macula. It is a unique retinal dystrophy because the appearance of cystic spaces in the macula heralds its onset with the rest of the retina being essentially normal.[1] Herein, we describe multimodal imaging findings in a case of DCMD.


  Case Report Top


A 30-year-old male patient presented to us with gradual diminution of vision in both eyes for 1 year. There was no history of night blindness and no history of niacin intake in the past. Family history was not significant. Best-corrected visual acuity was 20/30; N6 in both eyes. Anterior segment was normal with clear lens. There was no evidence of inflammation in the anterior chamber. Vitreous was clear. In both eyes, discs were healthy, retinal vessel caliber was normal, and pars plana was clear. Both eyes had cystoid macular edema at the posterior pole [Figure 1]. Multicolor imaging showed a greenish hue with central orange reflectance suggestive of gross cystoid macular edema [Figure 2]. Both short wave autofluorescence and near infra-red autofluorescence (NIR-AF) showed central multispot hyperautofluorescence and an area of hyperautofluorescence involving the posterior pole and extending up to the arcades [Figure 3]. NIR-AF showed an additional area of hyperautofluorescence, nasal to the disc in both eyes [Figure 4]. Spectral domain optical coherence tomography showed cystoid spaces involving all the retinal layers in both eyes [Figure 5]. Fluorescein angiography (FA) showed faint petaloid leakage at the fovea in both eyes [Figure 6]. The patient refused electrophysiological tests. Based on history, clinical examination and imaging findings a diagnosis of DCMD was made.
Figure 1: Color fundus photograph of right (a) and left (b) eye showing cystoid macular edema at posterior pole

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Figure 2: Multicolor image of right (a) and left (b) eye showing greenish hue at posterior pole suggestive of retina thickening (white arrows) with central orange hyper reflectance at fovea (black arrows) suggestive of a cyst

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Figure 3: Short wavelength autofluroscence of right (a) and left (b) eye showing distinct multispot hyperautofluorescence at the fovea (white arrows) with surrounding larger area of hyperautofluorescence involving the posterior pole extending beyond the arcades (white arrowheads)

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Figure 4: Near infra-red autofluroscence of right (a) and left (b) eye showing distinct multispot hyperautofluorescence at the fovea (thin white arrows) with surrounding larger area of hyperautofluorescence involving the posterior pole extending beyond the arcades (thick arrow). Hyperautofluorescent areas noted nasal to disc in both eyes (arrowhead c and d)

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Figure 5: Spectral domain optical coherence tomography line scan through the fovea of both right (a) and left (b) eye showing cystoid spaces involving all retinal layers

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Figure 6: Fundus fluorescein angiography midphase of right (a) and left (b) eye showing very faint petaloid hyperfluorescence (white arrows) at the fovea

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  Discussion Top


DCMD is a rare disease. The disease usually has its onset in the third decade of life. As the disease progresses the cystic spaces diminish giving rise to chorioretinal atrophy with subsequent loss in vision.[2] It has been suggested that pathophysiologically, this disease is caused by both abnormal muller cells as well as retinal pigment epithelial (RPE) dysfunction.[1] The current report highlights the multimodal imaging signatures of this entity. Multicolor imaging helps in picking up the cystoid abnormality much efficiently as compared to the conventional color fundus photograph. Fundus autofluorescence imaging, especially NIR-AF unmasks a larger area of RPE disturbance not picked up either on clinical examination or FA; thus, giving credence to the RPE dysfunction hypothesis. NIR-AF will be extremely useful to prognosticate and in follow-up of these cases, replacing invasive investigations such as fundus fluorescein angiography.

Multicolor imaging has the potential to completely replace color fundus photography in the future because both morphological and functional definition of the retina is rendered into a single composite image with the added advantages that all required image acquisitions can be performed with a single machine and in a nondilated pupil. It may also serve as a valuable noninvasive modality to determine the natural course or response to treatment based on regression of the area of greenish-hue representative of macular edema in these patients.

Electrophysiological tests and genetic testing are necessary addendum to the above-mentioned imaging modalities for conclusive diagnosis.


  Conclusion Top


The present report describes the multimodal imaging including multicolor findings in DCMD and provides unique insight into its possible pathogenesis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Saksens NT, van Huet RA, van Lith-Verhoeven JJ, den Hollander AI, Hoyng CB, Boon CJ. Dominant cystoid macular dystrophy. Ophthalmology 2015;122:180-91.  Back to cited text no. 1
[PUBMED]    
2.
Ganesh A, Stroh E, Manayath GJ, Al-Zuhaibi S, Levin AV. Macular cysts in retinal dystrophy. Curr Opin Ophthalmol 2011;22:332-9.  Back to cited text no. 2
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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