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Abstract

Small vertical cystoid voids have been found in the macular inner nuclear layer of patients with multiple sclerosis and were thought to indicate faster disease progression. Later it was found that this “microcystic macular edema” (MME) was caused indirectly by optic neuropathy and does not represent a primary multiple sclerosis lesion. MME has now been described in optic neuropathy of almost any known cause. It is associated with ganglion cell loss and thickening of the inner nuclear layer; it is located in a ring around the fovea, not affecting the fovea itself. Fluorescein angiography showed no leakage indicating a nonvascular origin of the edema. The combined changes have been summarized as retrograde maculopathy and can be readily distinguished from the longer known cystoid macular edema. The clinical significance of this novel clinical sign remains to be determined.

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References

  1. Gelfand JM, Nolan R, Schwartz DM, Graves J, Green AJ. Microcystic macular oedema in multiple sclerosis is associated with disease severity. Brain. 2012;135(Pt 6):1786–93.

    Article  PubMed Central  PubMed  Google Scholar 

  2. Saidha S, Sotirchos ES, Ibrahim MA, Crainiceanu CM, Gelfand JM, Sepah YJ, u. a. Microcystic macular oedema, thickness of the inner nuclear layer of the retina, and disease characteristics in multiple sclerosis: a retrospective study. Lancet Neurol. 2012;11(11):963–72.

    Google Scholar 

  3. Petzold A. Microcystic macular oedema in MS: T2 lesion or black hole? Lancet Neurol. 2012;11(11):933–4.

    Article  PubMed  Google Scholar 

  4. Gelfand JM, Cree BA, Nolan R, Arnow S, Green AJ. Microcystic inner nuclear layer abnormalities and neuromyelitis optica. JAMA Neurol. 2013;70(5):629–33.

    Article  PubMed  Google Scholar 

  5. Sotirchos ES, Saidha S, Byraiah G, Mealy MA, Ibrahim MA, Sepah YJ, u. a. In vivo identification of morphologic retinal abnormalities in neuromyelitis optica. Neurology. 2013;80(15):1406–14.

    Google Scholar 

  6. Balk LJ, Killestein J, Polman CH, Uitdehaag BMJ, Petzold A. Microcystic macular oedema confirmed, but not specific for multiple sclerosis. Brain. 2012;135(12):e226.

    Article  PubMed  Google Scholar 

  7. Abegg M, Zinkernagel M, Wolf S. Microcystic macular degeneration from optic neuropathy. Brain. 2012;135(Pt 12), e225.

    Article  PubMed  Google Scholar 

  8. Barboni P, Carelli V, Savini G, Carbonelli M, La Morgia C, Sadun AA. Microcystic macular degeneration from optic neuropathy: not inflammatory, not trans-synaptic degeneration. Brain. 2013;136(Pt 7), e239.

    Article  PubMed  Google Scholar 

  9. Abegg M, Zinkernagel M, Wolf S. Re: Rönnbäck et al.: Imaging of the macula indicates early completion of structural deficit in autosomal-dominant optic atrophy (Ophthalmology 2013;120:2672–7). Ophthalmology. 2014;121(6):e29–30.

    Google Scholar 

  10. Rönnbäck C, Milea D, Larsen M. Author reply: to Ophthalmology. 2014;121(6):e30–1.

    Google Scholar 

  11. Burggraaff MC, Trieu J, de Vries-Knoppert WAEJ, Balk L, Petzold A. The clinical spectrum of microcystic macular edema. Invest Ophthalmol Vis Sci. 2014;55(2):952–61.

    Article  PubMed  Google Scholar 

  12. Abegg M, Dysli M, Wolf S, Kowal J, Dufour P, Zinkernagel M. Microcystic macular edema: retrograde maculopathy caused by optic neuropathy. Ophthalmology. 2014;121(1):142–9.

    Article  PubMed  Google Scholar 

  13. Wen JC, Freedman SF, El-Dairi MA, Asrani S. Microcystic macular changes in primary open-angle glaucoma. J Glaucoma. 2014: Epub.

    Google Scholar 

  14. Kisimbi J, Shalchi Z, Mahroo OA, Mhina C, Sanyiwa AJ, Mabey D, u. a. Macular spectral domain optical coherence tomography findings in Tanzanian endemic optic neuropathy. Brain. 2013;136(11):3418–26.

    Google Scholar 

  15. Wolff B, Azar G, Vasseur V, Sahel J-A, Vignal C, Mauget-Faÿsse M. Microcystic changes in the retinal internal nuclear layer associated with optic atrophy: a prospective study. J Ophthalmol. 2014;2014:1–5.

    Article  Google Scholar 

  16. Sigler EJ. Microcysts in the inner nuclear layer, a nonspecific SD-OCT sign of cystoid macular edema. Invest Ophthalmol Vis Sci. 2014;55(5):3282–4.

    Article  PubMed  Google Scholar 

  17. Sigler EJ, Randolph JC, Charles S. Delayed onset inner nuclear layer cystic changes following internal limiting membrane removal for epimacular membrane. Graefes Arch Clin Exp Ophthalmol. 2013;251(7):1679–85.

    Article  PubMed  Google Scholar 

  18. Wolff B, Basdekidou C, Vasseur V, Mauget-Faÿsse M, Sahel J-A, Vignal C. Retinal inner nuclear layer microcystic changes in optic nerve atrophy: a novel spectral-domain OCT finding. Retina. 2013;33(10):2133–8.

    Article  PubMed  Google Scholar 

  19. Menke MN, Feke GT, Hirose T. Effect of aging on macular features of X-linked retinoschisis assessed with optical coherence tomography. Retina. 2011;31(6):1186–92.

    Article  PubMed  Google Scholar 

  20. Lujan BJ, Horton JC. Microcysts in the inner nuclear layer from optic atrophy are caused by retrograde trans-synaptic degeneration combined with vitreous traction on the retinal surface. Brain. 2013;136(11):e260.

    Article  PubMed Central  PubMed  Google Scholar 

  21. Brandt AU, Oberwahrenbrock T, Kadas EM, Lagrèze WA, Paul F. Dynamic formation of macular microcysts independent of vitreous traction changes. Neurology. 2014;83(1):73–7.

    Article  PubMed  Google Scholar 

  22. Bhargava P, Calabresi PA. The expanding spectrum of aetiologies causing retinal microcystic macular change. Brain. 2013;136(Pt 11):3212–4.

    Article  PubMed Central  PubMed  Google Scholar 

  23. Mahroo OA, Shalchi Z, Kisimbi J, Sanyiwa AJ, Mohamed MD, Plant GT. Re: Abegg et al.: Microcystic macular edema: retrograde maculopathy caused by optic neuropathy. Ophthalmology. 2014;121:142–9. Ophthalmology. 13. Mai 2014.

    Article  Google Scholar 

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Acknowledgments

The author received financial support from the Swiss National Science Foundation.

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Correspondence to Mathias Abegg MD, PhD .

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Abegg, M. (2016). Retrograde Maculopathy. In: Petzold, A. (eds) Optical Coherence Tomography in Multiple Sclerosis. Springer, Cham. https://doi.org/10.1007/978-3-319-20970-8_10

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  • DOI: https://doi.org/10.1007/978-3-319-20970-8_10

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-20969-2

  • Online ISBN: 978-3-319-20970-8

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