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Very early disease manifestations in macular telangiectasia type 2

Session Details

Session Title: Quick Fire Free Paper Session 01

Session Date/Time: Thursday 26/09/2013 | 08:30-10:30

Paper Time: 10:05

Venue: Hall G1 (Level 2)

First Author: T.Heeren GERMANY

Co Author(s):    F. Holz   P. Charbel Issa           

Abstract Details


To report the earliest retinal alterations in macular telangiectasia (MacTel) type 2 detectable using a multi-modal imaging approach.


Monocenter prospective cross sectional case series.


Patients were selected from a single center cohort of 110 patients with MacTel type 2. Inclusion was based on an asymmetric disease manifestation where only one eye clearly allowed the diagnosis based on ophthalmoscopy, fluorescein angiography (FA) and spectral domain optical coherence tomography (SD-OCT), whereas the other did not. The assessment included fundus photography, fluorescein angiography (Fl-A), recordings of fundus autofluorescence (FAF), macular pigment optical density (MPOD; determined by cSLO two-wavelengths measurements) and SD-OCT for analysis of macular cross sections and macular thickness mapping.


Six patients with a mean age of 64±11 years (range, 43-72 years) were identified. Five out of the six seemingly unaffected eyes had a visual acuity (VA) of 20/20 or better. One eye with vitreomacular traction had a VA of 20/32. All those eyes showed an asymmetric configuration of the foveal pit being thinnest temporal to the foveal center. Within a similar area, three eyes revealed a slight focal reduction of MPOD, resulting in an associated increased signal on FAF imaging due to less absorption of the excitation light. Only two eyes showed a minor nonspecific leakage on Fl-A untypical for MacTel type 2. No significant functional deficits were detected on microperimetry testing.


Loss of macular pigment as well as juxtafoveal thinning of the neurosensory retina may precede vascular alterations in a very early disease stage of MacTel type 2. These findings support previous studies showing that macular pigment loss exceeds the area of vascular alterations in later diseases stages, suggesting telangiectasia to occur secondarily to a degenerative disease process. Identification of affected patients or family members may be facilitated using herein presented but hitherto not applied diagnostic standards.

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