VASCULAR DISTORTION AND DRAGGING RELATED TO APPARENT TISSUE CONTRACTION IN MACULAR TELANGIECTASIS TYPE 2

To examine the alterations in retinal vascular morphology over an extended follow-up in eyes with macular telangiectasis Type 2 (MacTel2).

Eyes with high-quality digital photographs were evaluated. The geometric distortion in baseline images required to emulate the follow-up images was determined and vectors were made that represented the direction and magnitude of changes, to create a warp field. Optical coherence tomography and optical coherence tomography angiography evaluation of the retina was performed.

There were 7 eyes of 4 patients, who had a mean age of 70.25 years, which were followed for a mean of 8.8 years. The eyes showed increasing grayish opacification in the temporal macula with straightening and displacement of the macular vessels, even those in the nasal macula. The warp field vectors pointed to the temporal juxtafoveal macula. There was never any cavitation at the epicenter of the retinal distortion in any patient, although cavitations were found around this area. Optical coherence tomography imaging showed a circumscribed region of hyperreflectivity in the temporal macula. Optical coherence tomography angiography showed a deep angular condensed network of vessels within the hyperreflective region. One eye showed marked atrophic changes including full-thickness macular hole formation, but no increase in graying of the retina, loss of retinal laminations, pigmentary infiltration, or alteration in the retinal vessels.

Tissue contraction with retinal vascular displacement and contortion seem to be integral aspects of disease manifestation in MacTel2. The induced vascular changes may lead to secondary effects that increase morbidity in this disease.