Macular Degeneration, Age Related


Macular degeneration comprises a group of diseases characterised by progressive deterioration of the retinal photoreceptors, retinal pigment epithelial cells and underlying choriocapillaris, causing progressive central vision loss. The most common form of macular degeneration is age‐related macular degeneration (AMD), the leading cause of irreversible blindness among elderly patients in developed countries. Other types of hereditary early onset macular dystrophies share clinical characteristics with AMD, but a monogenetic basis for many of these conditions has been found. Recent advances in clinical and genetic research have led to a better understanding of the genetics and pathophysiology of AMD, which is currently viewed as a complex disorder in which multiple susceptibility genes combined with environmental factors play a role. The management of neovascular AMD has changed dramatically with the introduction of anti‐VEGF drugs, but treatment options for other types of macular degeneration remain limited because the aetiology and pathogenesis are incompletely defined.

Key Concepts:

  • Macular degeneration refers to a group of diseases characterised by photoreceptor cell degeneration and death leading to central vision loss.

  • Recent advances in Optical Coherence Tomography (OCT) technology has led to its major role in diagnosis and management of macular degeneration.

  • Monthly follow‐up of patients with neovascular AMD is recommended for best visual outcome.

  • Regular anti‐VEGF therapy with intravitreal injections of ranibizumab or bevacizumab is currently considered to be the standard treatment for neovascular AMD.

  • Ongoing clinical trials are investigating the efficacy and safety of combination treatments for neovascular AMD and treatments for other forms of macular degeneration.

  • Research efforts are directed at prevention of AMD and stem cell and gene therapy treatments for Stargardt disease.

Keywords: age‐related macular degeneration; Stargardt disease; hereditary retinal degeneration; AMD genetics; vascular endothelial growth factor

Figure 1.

Colour fundus photograph (a) and fundus autofluorescence image (b) of the left eye in a patient with advanced age‐related macular degeneration complicated by geographic atrophy of the central macula. Note well‐delineated area of hypopigmentation of the retinal pigment epithelial (RPE) layer (a, arrowheads), corresponding to hypoautofluorescent signal noted in (b).

Figure 2.

(a) Colour fundus photograph, (b) frame of fluorescein angiogram and (c) Optical Coherence Tomography (OCT) cross‐section image from the left eye of a patient with neovascular age‐related macular degeneration. A gray subretinal lesion is noted superior to the fovea (a, arrowheads). The choroidal neovascularisation is more readily discerned as the discrete area of hyper‐fluorescence in (b). The location of the retinal cross‐section OCT image shown in (c) is indicated in (b) as vertical green line. The OCT image shows irregular elevation of the RPE superior to the fovea, associated with retinal thickening and small round areas of dark signal representing intraretinal cysts of fluid.

Figure 3.

(a) Colour fundus photograph and (b) frame of fluorescein angiogram of the left macula in a patient with Stargardt disease. Deep yellow flecks are visible in (a). The normal choroidal fluorescence is blocked by excess lipofuscin, creating the appearance of a dark choroid in (b).



Age‐Related Eye Disease Study Research Group (2001) A randomized placebo‐controlled clinical trial of high‐dose supplementation with vitamins C and E, beta carotene and zinc for age related macular degeneration and vision loss. AREDS Report No. 8. Archives of Ophthalmology 119: 1417–1436.

Bither PP and Berns LA (1988) Stargardt's disease: a review of the literature. Journal of the American Optometric Association 59: 106–111.

Brown DM, Kaiser PK, Michels M et al. (2006) Ranibizumab versus verteporfin for neovascular age‐related macular degeneration. New England Journal of Medicine 355: 1432–1444.

Christen WG, Glyim RJ, Manson JE et al. (1996) A prospective study of cigarette smoking and age‐related macular degeneration in women. Journal of the American Medical Association 276: 1147–1151.

van Driel MA, Maugeri A, Klevering BJ, Hoyng CB and Cremers FPM (1998) ABCR unites what ophthalmologists divide(s). Ophthalmic Genetics 19: 117–122.

Eye Diseases Prevalence Research Group (2004) Prevalence of age‐related macular degeneration in the United States. Archives of Ophthalmology 122: 564–572.

Holz FG, Bindewald‐Wittich A, Fleckenstein M et al. (2007) Progression of geographic atrophy and impact of fundus autofluorescence patterns in age‐related macular degeneration. American Journal of Ophthalmology 143: 463–472.

Klein R, Klein BEK, Jensen SC and Meuer SM (1997) The five‐year incidence and progression of age‐related maculopathy: the Beaver Dam Eye Study. Ophthalmology 104: 7–21.

London NJS, Chiang A and Haller JA (2011) The dexamethasone drug delivery system: indications and evidence. Advanced Therapy 28: 351–366.

Pascolini D, Mariotti SP, Pokharel GP et al. (2004) 2002 Global update of available data on visual impairment: a compilation of population‐based prevalence studies. Ophthalmic Epidemiology 11: 67–115.

Paskowitz DM, LaVail MM and Duncan JL (2006) Light and inherited retinal degeneration. Brazilian Journal of Ophthalmology 90: 1060–1066.

Radu RA, Yuan Q, Hu J et al. (2008) Accelerated accumulation of lipofuscin pigments in the RPE of a mouse model for ABCA4‐mediated retinal dystrophies following vitamin A supplementation. Investigative Ophthalmology & Visual Science 49: 3821–3829.

Rosenfeld PJ, Brown DM, Heier JS et al. (2006) Ranibizumab versus verteporfin for neovascular age‐related macular degeneration. New England Journal of Medicine 355: 1419–1431.

Seddon JM, Ajani UA, Sperduto Rd et al. (1994) Dietary carotenoids, vitamins A, C and E, and advanced age‐related macular degeneration. Journal of American Medical Association 272: 1413–1420.

Seddon JM, Willett WC, Speizer FE et al. (1996) A prospective study of cigarette smoking and age‐related macular degeneration in women. Journal of American Medical Association 276: 1141–1146.

Sunness JS, Gonzalez‐Baron J, Applegate CA et al. (1999) Enlargement of atrophy and visual acuity loss in the geographic atrophy form of age‐related macular degeneration. Ophthalmology 106: 1768–1779.

The Alpha‐tocopherol Beta Carotene Study Group (1994) The effect of vitamin E and beta carotene on the incidence of lung cancer and other cancers in male smokers. New England Journal of Medicine 330: 1029–1035.

The CATT Research Group (2011) Ranibizumab and bevacizumab for neovascular age‐related macular degeneration. New England Journal of Medicine 364: 1897–1908.

Weng J, Mata NL, Azarian SM et al. (1999) Insights into the function of Rim protein in photoreceptors and etiology of Stargardt's disease from the phenotype in abcr knockout mice. Cell 98: 13–23.

Further Reading

Berger JW, Fine SL and Maguire MG (eds) (1999) Age‐Related Macular Degeneration. St Louis, MO: Mosby.

Bressler NM, Bressler SB and Fine SL (2006) Neovascular (exudative) age‐related macular degeneration. In: Stephen J Rayn (ed.) Retina, 4th edn, pp. 1075–1113. Philadelphia: Mosby.

Bressler SB, Bressler NM, Sarks SH and Sarks JP (2006) Age‐related macular degeneration: nonneovascular early AMD, intermediate AMD, and geographic atrophy. In: Stephen J Ryan (ed.) Retina, 4th edn, pp. 1041–1074. Philadelphia: Mosby.

Chen Y, Roorda A and Duncan JL (2010) Advances in imaging of Stargardt disease. In: Anderson RE, LaVail MM and Hollyfield JG (eds) Retinal Degenerative Diseases, Advances in Experimental Medicine and Biology, vol. 664, pp. 333–340. doi: 10.1007/978‐1‐4419‐9_38.

DeAngelis MM, Silveira AC, Carr EA and Kim IK (2011) Genetics of age‐related macular degeneration: current concepts, future directions. Seminars in Ophthalmology 26: 77–93.

Fishman GA, Farber M, Patel BS and Derlacki DJ (1987) Visual acuity loss in patients with Stargardt's macular dystrophy. Ophthalmology 94: 809–814.

Kostas H, Grigoriadou M and Petersen MB (2011) Age‐related macular degeneration: genetic and clinical findings. Clinical Chemistry and Laboratory Medicine 49: 601–616.

Sharma RK and Ehinger B (1999) Management of hereditary retinal degenerations: present status and future directions. Survey of Ophthalmology 43: 427–444.

Zhangm K, Nguyen T‐HE, Crandall A and Donoso LA (1995) Genetic and molecular studies of macular dystrophies: recent developments. Survey of Ophthalmology 40: 51–61.

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Zayit‐Soudry, Shiri, and Duncan, Jacque L(Mar 2012) Macular Degeneration, Age Related. In: eLS. John Wiley & Sons Ltd, Chichester. [doi: 10.1002/9780470015902.a0001906.pub2]