Macular Degeneration, Age Related

Abstract

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).

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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.

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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. http://www.els.net [doi: 10.1002/9780470015902.a0001906.pub2]