April 2011
Volume 52, Issue 14
ARVO Annual Meeting Abstract  |   April 2011
Incidence And Progression Of Pigment Clumping In Macular Telangiectasia Type 2 (mactel2)
Author Affiliations & Notes
  • Annal D. Meleth
    Ophthalmology, National Eye Institute, Bethesda, Maryland
  • Brian Toy
    Ophthalmology, National Eye Institute, Bethesda, Maryland
  • Nupura Krishnadev
    Ophthalmology, National Eye Institute, Bethesda, Maryland
  • Robert P. Murphy
    Retina Group of Washington, Fairfax, Virginia
  • Emily Y. Chew
    Ophthalmology, National Eye Institute, Bethesda, Maryland
  • Wai T. Wong
    Ophthalmology, National Eye Institute, Bethesda, Maryland
  • Footnotes
    Commercial Relationships  Annal D. Meleth, None; Brian Toy, None; Nupura Krishnadev, None; Robert P. Murphy, None; Emily Y. Chew, None; Wai T. Wong, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science April 2011, Vol.52, 4524. doi:
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      Annal D. Meleth, Brian Toy, Nupura Krishnadev, Robert P. Murphy, Emily Y. Chew, Wai T. Wong; Incidence And Progression Of Pigment Clumping In Macular Telangiectasia Type 2 (mactel2). Invest. Ophthalmol. Vis. Sci. 2011;52(14):4524.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose: : To determine incidence and progression of pigment clumping in patients with MacTel2.

Methods: : Patients with a diagnosis of MacTel2 in at least one eye and at least 12 months of follow-up were identified from two retinal clinics and reviewed retrospectively. Fundus photographs at baseline and subsequent visits were evaluated longitudinally for the presence and location of involvement of pigment clumping. Correlative analyses of pigment clumping with visual acuity, OCT findings, and microperimetry findings were also performed.

Results: : The study involved 54 eyes in 27 patients with a mean follow-up of 42.5±14.2 months (range 12-79 months). At the initial/baseline visit, 53 eyes (98%) were determined to have clinical signs of MacTel2. Of these, 18 eyes (34%) had evidence of pigment clumping. Mean baseline logMAR visual acuity was significantly lower in eyes with pigment clumping (n = 18) compared to eyes without (n = 35) (0.58±0.27 (approximately 20/76) versus 0.26±0.31 (20/36)); p <0.01, student’s t-test). Longitudinal review revealed that new onset of pigment clumping occurred in 12/35 (34%) eyes, with the majority of new pigment appearing in the temporal perifoveal region. Quantitative analysis revealed that pigment clumping in all eyes increased as a function of time. Neovascular changes were observed in7/53 (13%) eyes with MacTel2; in 3 eyes, neovascular changes preceded the onset of pigment clumping, while in 3 eyes, pigment clumping was present concurrently with neovascular changes and in the final eye, pigment was present prior to neovascular changes.

Conclusions: : Pigment clumping is a clinical feature commonly found in MacTel2 and may be associated with lower visual acuity in affected eyes. Pigment clumping arises in typical fundus locations and increases in total area during the natural history of the disease. No apparent association was detected between pigment clumping and the development of neovascular changes. Pigment clumping is a prominent feature in the progression of MacTel2 and may constitute a useful outcome measure for clinical study.

Keywords: clinical (human) or epidemiologic studies: natural history • degenerations/dystrophies • macula/fovea 

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