Elsevier

Ophthalmology

Volume 117, Issue 11, November 2010, Pages 2162-2168
Ophthalmology

Original article
Outer Retinal Hyperreflective Spots on Spectral-Domain Optical Coherence Tomography in Macular Telangiectasia Type 2

https://doi.org/10.1016/j.ophtha.2010.02.014Get rights and content

Purpose

To analyze focal hyperreflective morphologic alterations in outer retinal layers in patients with type 2 idiopathic macular telangiectasia (MacTel type 2) using spectral-domain optical coherence tomography (SD OCT).

Design

Cross-sectional case-control study.

Participants

Forty-one patients with MacTel type 2.

Methods

Anatomic layers were evaluated and compared with those of controls of similar age. Simultaneous SD OCT scans were obtained with a combined confocal scanning laser ophthalmoscope for simultaneous tomographic and topographic in vivo imaging (Spectralis HRA+OCT; Heidelberg Engineering, Heidelberg, Germany).

Main Outcome Measures

Morphologic alterations in the retinal layers secondary to MacTel type 2.

Results

Hyperreflective spots in the outer retina of MacTel type 2 patients were detected in all stages of disease using the SD OCT. Their presence was confined to the foveolar and parafoveolar region. The phenomenon also was detected in a monozygotic twin in an eye with no typical angiographic sign of the disease. A hyperreflective haze was detected in the vicinity of a disruption of the hyperreflective OCT line that is assumed to represent the line between the photoreceptor inner and outer segments and interdigitation of the outer segments and the retinal pigment epithelium. No corresponding pathologic features could be identified by biomicroscopy, time-domain OCT, or confocal scanning laser ophthalmoscope imaging. Crystalline deposits and intraretinal migration of pigmented cells were distinguishable because of differences in shape, reflectivity, and location.

Conclusions

Hyperreflective spots were identified in outer retinal layers of patients with MacTel type 2 in all disease stages. It is suggested that this phenomenon represents an early sign of a neurodegenerative process. Secondary assumptions include extravasated deposits or vascular abnormalities. The pathologic features are indicative of an active disease process before the disease manifests by typical fluorescein angiographic signs.

Financial Disclosure(s)

Proprietary or commercial disclosure may be found after the references.

Section snippets

Patients and Methods

Forty-one patients with MacTel type 2 were recruited at the Department of Ophthalmology, University of Bonn, a tertiary referral center participating in the Macular Telangiectasia Project (http://www.mactelresearch.org). This international research program is being conducted in 7 countries with 5 basic science laboratories and 22 clinical centers to evaluate the natural history and pathophysiologic features of this condition. The study adhered to the tenets of the Declaration of Helsinki; the

Results

The patients' mean age was 65 years (range, 54–75 years). There were 22 females and 19 males. A total of 79 eyes were included, and 3 eyes were excluded because of a history of focal laser photocoagulation.

Discussion

This study demonstrated hyperreflective spots on high-resolution SD OCT scans in the outer retinal layers associated with MacTel type 2. Interestingly, such spots occur already in preclinical stages of disease and thus may be helpful in the early diagnosis and identification of affected individuals. The precise nature and molecular constituents of these spots are yet unknown. Various possibilities and pathways may be considered. The authors suggest that these hyperreflective spots and bands are

References (36)

  • M. Bolz et al.

    Optical coherence tomographic hyperreflective foci: a morphologic sign of lipid extravasation in diabetic macular edema

    Ophthalmology

    (2009)
  • P. Charbel Issa et al.

    Findings in fluorescein angiography and optical coherence tomography after intravitreal bevacizumab in type 2 idiopathic macular telangiectasia

    Ophthalmology

    (2007)
  • J. Stone et al.

    Mechanisms of retinal angiogenesis

    Prog Retin Eye Res

    (1997)
  • J.M. Provis et al.

    Development of the human retinal vasculature: cellular relations and VEGF expression

    Exp Eye Res

    (1997)
  • L.A. Yannuzzi et al.

    Idiopathic macular telangiectasia

    Arch Ophthalmol

    (2006)
  • P. Charbel Issa et al.

    Idiopathic macular telangiectasia

  • P. Charbel Issa et al.

    Microperimetric assessment of patients with type 2 macular telangiectasia

    Invest Ophthalmol Vis Sci

    (2007)
  • R.P. Finger et al.

    Reading performance is reduced by parafoveal scotomas in patients with macular telangiectasia type 2

    Invest Ophthalmol Vis Sci

    (2009)
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    Manuscript no. 2009-1212.

    Financial Disclosure(s): The author(s) have made the following disclosure(s):

    Heidelberg Engineering provided the Department of Ophthalmology, University of Bonn, with the Spectralis HRA+OCT free of charge.

    Professor Dr. med. Frank G. Holz, MD, received lecture and travel fees.

    Dr. med. Peter Charbel Issa, MD, FEBO and Dr. med. Steffen Schmitz-Valckenberg, MD, FEBO, received lecture fees.

    Supported by the Lowy Medical Research Institute, Sydney, Australia; a Marie Curie Intra-European Fellowship (no.: 237238), Seventh European Community Framework Program, European Commission, Brussels, Belgium; BONFOR Program, Faculty of Medicine, University of Bonn, Bonn, Germany (grant no.: O-137.0011).

    Spectralis HRA+OCT: Heidelberg Engineering GmbH, Tiergartenstraße 15, 69121 Heidelberg, Germany.

    The sponsors or funding organizations had no role in the design or conduct of this research. The contents of this publication reflect only the author's views and not the views of the funding organizations.

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