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Mali Okada, Katie M Litts, Tjebo FC Heeren, Angelos Kalitzeos, Vincent Rocco, Rebecca Mastey, Navjit Singh, Thomas Kane, Melissa Kasilian, Michel Michaelides, Marcus Fruttiger, Joseph Carroll, Catherine Egan; Twelve-month longitudinal study of remnant cone structure in macular telangiectasia type 2. Invest. Ophthalmol. Vis. Sci. 2018;59(9):4628.
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© ARVO (1962-2015); The Authors (2016-present)
To evaluate ellipsoid zone (EZ) lesions in macular telangiectasia (MacTel) type 2 over time and to determine the presence of remnant cone structures using both confocal and split-detector adaptive optics scanning light ophthalmoscopy (AOSLO).
Nine eyes from 6 patients (53.5 ± 4.4 years) with discrete EZ lesions secondary to MacTel Type 2 had multimodal imaging with SD-OCT, confocal and split-detector AOSLO performed at baseline, 6 months and 12 months. Enface OCT was used to guide the location of the EZ lesions for AOSLO imaging. Comparisons were made to determine presence of remnant cone structures.
At baseline, the mean (± SD) area of EZ lesions on enface OCT was 0.39 ± 0.58mm2. Remnant cone structures were visible within or at the border of some EZ lesions on split-detector but not on confocal AOSLO. Cone density within or at the border of lesions remained stable (32,298 ± 13,056 cones/mm2 at baseline, 37,257 ± 4,795 cones/mm2 at 6 months and 34,069 ± 9,715 cones/mm2 at 12 months, p=0.89). Discrete EZ lesions were surrounded by relatively normal cone mosaic without evidence of a transition zone. At month 12, the mean change in EZ area was -0.038 mm2. In 3 of the 6 eyes that had imaging at 12 months, there was evidence of recovery of EZ reflectivity on OCT and a corresponding reduction in size of lesion seen on AOSLO (See figures). Recovery of EZ reflectivity, however, was not directly related to area of remnant cone structures seen on initial split-detector AOSLO.
In MacTel type 2, clusters of remnant cone structure may persist within outer retinal lesions despite not being visible on standard imaging techniques. In some eyes, spontaneous recovery or reduction of EZ lesions can occur and may represent a waxing and waning disease state. This has implications for therapeutic treatment trials where OCT endpoints may be used to monitor treatment efficacy.
This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.
SD-OCT in a 46-year old male with macular telangiectasia type 2. Imaging shows discrete foveal-involving EZ break at baseline (A) and spontaneous recovery in the reflectivity of this lesion at month 12 (B).
Assessment of cone structure within the lesion of the same patient in Figure 1. Enface OCT at baseline (A) and at month 12 (D) with reduction in size of the EZ lesion. Change on confocal (B, E) and split-detector AOSLO (C, F) with remnant cone structures visible at border of the lesion.
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