PMID- 32818114 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20221012 IS - 2164-2591 (Print) IS - 2164-2591 (Electronic) IS - 2164-2591 (Linking) VI - 9 IP - 4 DP - 2020 Mar TI - Longitudinal Assessment of Remnant Foveal Cone Structure in a Case Series of Early Macular Telangiectasia Type 2. PG - 27 LID - 10.1167/tvst.9.4.27 [doi] LID - 27 AB - PURPOSE: To determine the extent of remnant cone structure within early foveal ellipsoid zone (EZ) lesions in macular telangiectasia type 2 longitudinally using both confocal and split detector adaptive optics scanning light ophthalmoscopy (AOSLO). METHODS: Spectral domain optical coherence tomography (SDOCT), confocal and split detector AOSLO were acquired from seven patients (10 eyes) with small (early) EZ lesions on SDOCT secondary to macular telangiectasia type 2 at baseline, 6 months, and 12 months. The presence of cone structure on AOSLO in areas of EZ loss as well as cones at 1 degrees eccentricity, and their change over time were quantified. RESULTS: By split detector AOSLO, remnant cone structure was identified within and on the borders of all foveal EZ lesions. Within the extent of these lesions, cone spacing ranged from 4.97 to 9.95 microm at baseline, 5.30 to 6.10 microm at 6 months, and 4.99 to 7.12 microm at 12 months. Four eyes with significantly smaller EZ lesions showed evidence of recovery of EZ reflectivity on SDOCT B-scans. Remnant cone structure was identified in some areas where EZ reflectivity recovered at the following time point. Eyes that showed recovery of EZ reflectivity had a continuous external limiting membrane. CONCLUSIONS: Remnant cone structure can persist within small SDOCT-defined EZ lesions, which can wax and wane in appearance over time. AOSLO can help to inform the interpretation of SDOCT imaging. TRANSLATIONAL RELEVANCE: The absence of EZ in early macular telangiectasia type 2 and other retinal conditions needs careful interpretation because it does not always indicate an absence of underlying cone structure. The integrity of the external limiting membrane may better predict the presence of remnant cone structure and recovery of EZ reflectivity. CI - Copyright 2020 The Authors. FAU - Litts, Katie M AU - Litts KM AD - Ophthalmology & Visual Sciences, Medical College of Wisconsin, Milwaukee, WI, USA. FAU - Okada, Mali AU - Okada M AD - Royal Victorian Eye and Ear Hospital, Melbourne, Australia. FAU - Heeren, Tjebo F C AU - Heeren TFC AD - Moorfields Eye Hospital NHS Foundation Trust, London, UK. AD - University College London Institute of Ophthalmology, London, UK. FAU - Kalitzeos, Angelos AU - Kalitzeos A AD - Moorfields Eye Hospital NHS Foundation Trust, London, UK. AD - University College London Institute of Ophthalmology, London, UK. FAU - Rocco, Vincent AU - Rocco V AD - Moorfields Eye Hospital NHS Foundation Trust, London, UK. FAU - Mastey, Rebecca R AU - Mastey RR AD - Ophthalmology & Visual Sciences, Medical College of Wisconsin, Milwaukee, WI, USA. FAU - Singh, Navjit AU - Singh N AD - Moorfields Eye Hospital NHS Foundation Trust, London, UK. AD - University College London Institute of Ophthalmology, London, UK. FAU - Kane, Thomas AU - Kane T AD - Moorfields Eye Hospital NHS Foundation Trust, London, UK. AD - University College London Institute of Ophthalmology, London, UK. FAU - Kasilian, Melissa AU - Kasilian M AD - Moorfields Eye Hospital NHS Foundation Trust, London, UK. AD - University College London Institute of Ophthalmology, London, UK. FAU - Fruttiger, Marcus AU - Fruttiger M AD - University College London Institute of Ophthalmology, London, UK. FAU - Michaelides, Michel AU - Michaelides M AD - Moorfields Eye Hospital NHS Foundation Trust, London, UK. AD - University College London Institute of Ophthalmology, London, UK. FAU - Carroll, Joseph AU - Carroll J AD - Ophthalmology & Visual Sciences, Medical College of Wisconsin, Milwaukee, WI, USA. AD - Cell Biology, Neurobiology and Anatomy, Medical College of Wisconsin, Milwaukee, WI, USA. FAU - Egan, Catherine AU - Egan C AD - Moorfields Eye Hospital NHS Foundation Trust, London, UK. AD - University College London Institute of Ophthalmology, London, UK. LA - eng GR - C06 RR016511/RR/NCRR NIH HHS/United States GR - R01 EY017607/EY/NEI NIH HHS/United States GR - UL1 TR001436/TR/NCATS NIH HHS/United States GR - P30 EY001931/EY/NEI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20200330 PL - United States TA - Transl Vis Sci Technol JT - Translational vision science & technology JID - 101595919 MH - *Fovea Centralis MH - Humans MH - Ophthalmoscopy MH - Retinal Cone Photoreceptor Cells MH - Visual Acuity PMC - PMC7396184 OTO - NOTNLM OT - adaptive optics scanning light ophthalmoscopy OT - cones OT - ellipsoid zone OT - macular telangiectasia COIS- Disclosure: K.M. Litts, None; M. Okada, Allergan, Bayer; T.F.C. Heeren, Heidelberg Engineering (S); A. Kalitzeos, None; V. Rocco, None; R.R. Mastey, None; N. Singh, None; T. Kane, None; M. Kasilian, None; M. Fruttiger, None; M. Michaelides, None; J. Carroll, None; C. Egan, Heidelberg Engineering (R), Novartis (F), Haag-Streit (R) EDAT- 2020/08/21 06:00 MHDA- 2020/08/21 06:01 PMCR- 2020/03/30 CRDT- 2020/08/21 06:00 PHST- 2019/09/18 00:00 [received] PHST- 2020/01/13 00:00 [accepted] PHST- 2020/08/21 06:00 [entrez] PHST- 2020/08/21 06:00 [pubmed] PHST- 2020/08/21 06:01 [medline] PHST- 2020/03/30 00:00 [pmc-release] AID - TVST-19-1928 [pii] AID - 10.1167/tvst.9.4.27 [doi] PST - epublish SO - Transl Vis Sci Technol. 2020 Mar 30;9(4):27. doi: 10.1167/tvst.9.4.27. eCollection 2020 Mar.