SCOTOMA CHARACTERISTICS IN MACULAR TELANGIECTASIA TYPE 2: MacTel Project Report No. 7-The MacTel Research Group

Stela Vujosevic; Tjebo F C Heeren; Daniela Florea; Irene Leung; Daniel Pauleikhoff; Ferenc Sallo; Alan Bird; Tunde Peto

doi:10.1097/IAE.0000000000001693

SCOTOMA CHARACTERISTICS IN MACULAR TELANGIECTASIA TYPE 2: MacTel Project Report No. 7-The MacTel Research Group

Stela Vujosevic, Tjebo F C Heeren, Daniela Florea, Irene Leung, Daniel Pauleikhoff, Ferenc Sallo, Alan Bird, Tunde Peto

School of Medicine, Dentistry and Biomedical Sciences

Research output: Contribution to journal › Article › peer-review

12 Citations (Scopus)

Abstract

PURPOSE: To characterize scotomas in macular telangiectasia Type 2 (MacTel).

METHODS: Five of the 27 centers performed microperimetry as part of the MacTel Natural History Observation Study. Data were analyzed in the Moorfields Eye Hospital Reading Centre. The number of stimuli under a threshold of 12, 10, 8, and <0 dB were counted (thresholding) and compared with one another.

RESULTS: A total of 565 examinations were gradable, received from 632 eyes of 322 participants (age 61.1 ± 9.1 years, 62% females). The authors found absolute scotomas in 243 eyes (43%), 98% of these affected the temporal quadrant, and 99.5% were unifocal. Growth of absolute scotomas was limited to an extent of approximately 40 deg. Although transition from functionally unimpaired retina to absolute scotomas is generally steeply sloped, the larger a scotoma, the steeper it is.

CONCLUSION: Scotoma features were consistent throughout a large MacTel cohort. The temporal quadrant was confirmed as predominantly affected, which might result from vascular or metabolic asymmetry. Functional loss did not exceed an area of 5° × 8° however advanced the disorder. Different MacTel phenotypes seem likely and point toward different types of progression; identifying these would improve planning for clinical trials and might lead to better understanding patient outcome.

Original language	English
Pages (from-to)	1-6
Journal	Retina (Philadelphia, Pa.)
Early online date	22 May 2017
DOIs	https://doi.org/10.1097/IAE.0000000000001693
Publication status	Early online date - 22 May 2017

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Journal Article

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10.1097/IAE.0000000000001693Licence: Unspecified

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title = "SCOTOMA CHARACTERISTICS IN MACULAR TELANGIECTASIA TYPE 2: MacTel Project Report No. 7-The MacTel Research Group",

abstract = "PURPOSE: To characterize scotomas in macular telangiectasia Type 2 (MacTel).METHODS: Five of the 27 centers performed microperimetry as part of the MacTel Natural History Observation Study. Data were analyzed in the Moorfields Eye Hospital Reading Centre. The number of stimuli under a threshold of 12, 10, 8, and <0 dB were counted (thresholding) and compared with one another.RESULTS: A total of 565 examinations were gradable, received from 632 eyes of 322 participants (age 61.1 ± 9.1 years, 62% females). The authors found absolute scotomas in 243 eyes (43%), 98% of these affected the temporal quadrant, and 99.5% were unifocal. Growth of absolute scotomas was limited to an extent of approximately 40 deg. Although transition from functionally unimpaired retina to absolute scotomas is generally steeply sloped, the larger a scotoma, the steeper it is.CONCLUSION: Scotoma features were consistent throughout a large MacTel cohort. The temporal quadrant was confirmed as predominantly affected, which might result from vascular or metabolic asymmetry. Functional loss did not exceed an area of 5° × 8° however advanced the disorder. Different MacTel phenotypes seem likely and point toward different types of progression; identifying these would improve planning for clinical trials and might lead to better understanding patient outcome.",

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year = "2017",

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doi = "10.1097/IAE.0000000000001693",

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T2 - MacTel Project Report No. 7-The MacTel Research Group

AU - Vujosevic, Stela

AU - Heeren, Tjebo F C

AU - Florea, Daniela

AU - Leung, Irene

AU - Pauleikhoff, Daniel

AU - Sallo, Ferenc

AU - Bird, Alan

AU - Peto, Tunde

PY - 2017/5/22

Y1 - 2017/5/22

N2 - PURPOSE: To characterize scotomas in macular telangiectasia Type 2 (MacTel).METHODS: Five of the 27 centers performed microperimetry as part of the MacTel Natural History Observation Study. Data were analyzed in the Moorfields Eye Hospital Reading Centre. The number of stimuli under a threshold of 12, 10, 8, and <0 dB were counted (thresholding) and compared with one another.RESULTS: A total of 565 examinations were gradable, received from 632 eyes of 322 participants (age 61.1 ± 9.1 years, 62% females). The authors found absolute scotomas in 243 eyes (43%), 98% of these affected the temporal quadrant, and 99.5% were unifocal. Growth of absolute scotomas was limited to an extent of approximately 40 deg. Although transition from functionally unimpaired retina to absolute scotomas is generally steeply sloped, the larger a scotoma, the steeper it is.CONCLUSION: Scotoma features were consistent throughout a large MacTel cohort. The temporal quadrant was confirmed as predominantly affected, which might result from vascular or metabolic asymmetry. Functional loss did not exceed an area of 5° × 8° however advanced the disorder. Different MacTel phenotypes seem likely and point toward different types of progression; identifying these would improve planning for clinical trials and might lead to better understanding patient outcome.

AB - PURPOSE: To characterize scotomas in macular telangiectasia Type 2 (MacTel).METHODS: Five of the 27 centers performed microperimetry as part of the MacTel Natural History Observation Study. Data were analyzed in the Moorfields Eye Hospital Reading Centre. The number of stimuli under a threshold of 12, 10, 8, and <0 dB were counted (thresholding) and compared with one another.RESULTS: A total of 565 examinations were gradable, received from 632 eyes of 322 participants (age 61.1 ± 9.1 years, 62% females). The authors found absolute scotomas in 243 eyes (43%), 98% of these affected the temporal quadrant, and 99.5% were unifocal. Growth of absolute scotomas was limited to an extent of approximately 40 deg. Although transition from functionally unimpaired retina to absolute scotomas is generally steeply sloped, the larger a scotoma, the steeper it is.CONCLUSION: Scotoma features were consistent throughout a large MacTel cohort. The temporal quadrant was confirmed as predominantly affected, which might result from vascular or metabolic asymmetry. Functional loss did not exceed an area of 5° × 8° however advanced the disorder. Different MacTel phenotypes seem likely and point toward different types of progression; identifying these would improve planning for clinical trials and might lead to better understanding patient outcome.

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SN - 0275-004X

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JO - Retina (Philadelphia, Pa.)

JF - Retina (Philadelphia, Pa.)

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SCOTOMA CHARACTERISTICS IN MACULAR TELANGIECTASIA TYPE 2: MacTel Project Report No. 7-The MacTel Research Group

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