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C H A P T E R 1 Anatomy and Physiology of the Retina

CLINICAL CORRELATION: RETINA

Acute branch retinal vein occlusion with flame-shaped retinal hemorrhages and cotton wool spots involving the nerve fiber layer.

Myelinated nerve fiber layer. Note the arcuate pattern of the nerve fiber layer around the macula. The larger retinal vessels are located within the nerve fiber layer.

Cherry red spot following a central retinal artery occlusion. The ischemic retinal whitening occurs in the inner retina of the macula where the ganglion cell and nerve fiber layers are thickest. The central red spot is a result of the normal choroidal circulation.

Lipid exudate in the macula following malignant hypertension. The lipid may form a star-pattern within the middle layers of the retina as it radiates from the center of the macula.

Cystoid macular edema following cataract surgery. The cyst-like spaces form in the outer plexiform layer of the retina (Henle’s layer). Fluorescein angiography reveals a classic “petaloid” pattern of hyperfluorescence.

Commotio retinae following a blunt trauma injury. The deep retinal whitening results from shearing of the outer segments of the photoreceptors. Note the normal retinal blood vessels overlying the retinal whitening.

C H A P T E R 1 Anatomy and Physiology of the Retina

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CLINICAL CORRELATION: RETINAL PIGMENT EPITHELIUM

Bull’s eye maculopathy in chloroquine toxicity. The hypopigmented region around the center of the fovea results from atrophy of the retinal pigment epithelium.

Transmission or “window” defect in age-related macular degeneration with geographic atrophy. Atrophy of the retinal pigment epithelium “unmasks” the underlying choroidal fluorescence.

Stargardt disease is characterized by abnormalities of the retinal pigment epithelium (RPE). Lipofuscin accumulation within the RPE results in a vermillion discoloration of the fundus. Atrophy of the RPE may result in a bull’s eye maculopathy.

Fluorescein angiography of the same patient demonstrates a “silent choroid” as a result of blockage of the normal choroidal fluorescence. The central hyperfluorescence is a result of atrophy of the retinal pigment epithelium.

Hyperplastic retinal pigment epithelium (RPE) in a patient with presumed ocular histoplasmosis. In response to disease, the RPE may become atrophic or hyperplastic.

Intraretinal pigment migration in a patient with retinitis pigmentosa. The classic triad of retinitis pigmentosa includes disc pallor, vessel attenuation, and peripheral pigmentary alterations.

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C H A P T E R 1 Anatomy and Physiology of the Retina

CLINICAL CORRELATION: BRUCH’S MEMBRANE AND CHOROID

Angioid streaks in a woman with pseudoxanthoma elasticum. Angioid streaks are breaks in the RPEBruch’s membrane complex. They are often seen radiating from the optic disc.

Choroidal ruptures are breaks in the RPE-Bruch’s membrane complex following blunt trauma injuries to the eye. They are usually located concentric to the optic disc.

Atrophy of the retinal pigment epithelium in a patient with Stargardt disease reveals the underlying choroidal vessels.

Fluorescein angiogram of a patient with choroideremia demonstrates hyperfluorescence of the choriocapillaris centrally and hypofluorescence in the areas of the retinal pigment epithelial and choriocapillaris atrophy. Note the larger choroidal vessels in the peripapillary and peripheral retina.

Classic choroidal neovascularization. The new vessel membrane extends from the choroid through Bruch’s membrane to the sub-RPE or sub-neurosensory retinal space.

Fluorescein angiogram demonstrating choroidal nonperfusion in a patient with giant cell arteritis. This results from occlusion of the posterior ciliary arteries.

C H A P T E R 1 Anatomy and Physiology of the Retina

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CLINICAL CORRELATION: VITREOUS, RETINAL ADHESION, AND BLOOD-RETINAL BARRIER

Asteroid hyalosis is characterized by numerous calcium deposits within the vitreous gel. Individuals may be asymptomatic or complain of floaters.

The vitreous provides a scaffold for the growth of new vessels in proliferative diabetic retinopathy. Contraction of the vitreous may cause vitreous hemorrhage or traction retinal detachment.

Rhegmatogenous retinal detachment results from a retinal tear or hole. The retinal tear or hole allows fluid to accumulate in the potential space between the neurosensory retina and the retinal pigment epithelium.

An exudative retinal detachment results from fluid accumulation between the neurosensory retina and the retinal pigment epithelium. This patient had atypical central serous choroidopathy. Note the protein deposits on the posterior surface of the neurosensory retina.

Fluorescein angiography of a patient with diabetic retinopathy demonstrates disruption of the inner bloodretinal barrier. The hyperfluorescence is the result of leakage from the retinal vessels.

Fluorescein angiography of a patient with toxemia of pregnancy. Leakage of fluid through the retinal pigment epithelium (the outer blood-retinal barrier) results in exudative neurosensory retinal detachments.

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C H A P T E R 1 Anatomy and Physiology of the Retina

SELECTED REFERENCES

1.Bernstein PS. Macular biology. In: Berger JW, Fine SL, Maguire MG, eds. Age-Related Macular Degeneration.

St. Louis: Mosby, Inc; 1999:1–16.

2.Blanks JC. Morphology and topography of the retina. In: Ogden TE, Hinton DR, eds. Retina. 3rd ed. St. Louis: Mosby, Inc; 2001:32–53.

3.Chen J, Flannery J. Structure and function of rod photoreceptors. In: Ogden TE, Hinton DR, eds. Retina. 3rd ed. St. Louis: Mosby, Inc; 2001:122–137.

4.Eagle RC Jr. Mechanisms of maculopathy. Ophthalmology. 1984;91:613–625.

5.Fundamentals and Principles of Ophthalmology. San Francisco: The Foundation of the American Academy of Ophthalmology; 2001:68–95.

6.Gass JD. Muller cell cone, an overlooked part of the anatomy of the fovea centralis: hypotheses concerning its role in the pathogenesis of macular hole and foveomacular retinoschisis. Arch Ophthalmol. 1999;117:821–823.

7.Guyer DR, Schachat AP, Green WR. The choroid: structural considerations. In: Ogden TE, Hinton DR, eds. Retina. 3rd ed. St. Louis: Mosby, Inc; 2001:21–31.

8.Hargrave PA, McDowell JH. Rhodopsin and phototransduction: a model system for G protein-linked receptors. FASEB J. 1992;6:2323–2331.

9.Harris A, Bingaman DP, Ciulla TA, Martin BJ. Retinal and choroidal blood flow in health and disease. In: Ogden TE, Hinton DR, eds. Retina. 3rd ed. St. Louis: Mosby, Inc; 2001:68–88.

10.Kandel ER, Schwartz JH, Jessell TM, eds. Principles of Neural Science. 4th ed. New York: McGraw-Hill; 2000:507–515.

11.Lagnado L, Baylor D. Signal flow in visual transduction. Neuron. 1992;8:995–1002.

12.O’Brien DF. The chemistry of vision. Science. 1982;218:961–966.

13.Stryer L. The molecules of visual excitation. Sci Am. 1987;257:42–50.

14.Thumann G, Hinton DR. Cell biology of the retinal pigment epithelium. In: Ogden TE, Hinton DR, eds. Retina. 3rd ed. St. Louis: Mosby, Inc; 2001:104–121.