Retina Prof.Andrea Facskó M.D., Ph.D. med. habil. Head of the Dept. Of Ophthalmology Cross section Ophthalmoscop Fluorescein angiogram (FLAG) The retina Fotoreceptors: rodes (150 mill.) for light perception cones (7 mill.) for color vision Macula region: very dens of receptors Human eye: like a 137 megapixel camera Human eye+brain: TRIDIMENSINAL view Human eye:from blue to red from 400 till 700 nm wave length light can be detected 10 layers of the retina OCT /Spectral domain optical coherence tomography/ B scan C scan OCT General remarks The retina and the optic nerve are parts of the central nervous system The retina transparent red color (from the blood in the choroidal vessels) choroidal vascular network is normally invisible arteries (arterioles) are end-arteries (without anastomoses) veins are dark red (the ratio of vein to artery diameters is 2:3) capillaries are normally not visible Diseases of the retina 1. Local circulatory disturbances a) Central retinal artery occlusion (emergency situation) (CAO) (ischemic infarct of the retina) Symptoms: sudden painless loss of vision Etiology: embolus from an arteriosclerotic aorta or carotid artery transient ischemic attack in the brain embolus from the valves of the heart a myxoma of the atrium fat - because of severe crushing injuries to the chest or abdomen temporal arteritis increased blood viscosity or dysproteinemia systemic circulatory disturbances with severe hypotension severe migraine, nicotine abuse, oral contraceptives Ophthalmoscopic picture: the disc: pale with blurred outlines milky white ischemic edema of the posterior pole in the fovea : cherry red spot the retinal arterioles: extremely thin Therapy: paracentesis, injection of vasodilators retrobulbar Prognosis:poor Central arterial occlusion Branch artery occlusion: a sector-shaped, milky white retinal opacity can be seen,corresponding to the area supplied by the corresponding arterial branch Diseases of the retina 1. Local circulatory disturbances b) Occlusion of the central retinal vein (CVO) (red infarct of the retina) Symptoms: painless reduction of visual acuity,visual field defect Etiology : arteriosclerosis cardiovascular, hypertension disturbances of coagulation diabetes systemic diseases of the blood vessels Predisposition factors: certain toxic substances (nicotine) areas, where the venous passages are narrow Ophthalmoscopic picture: optic nerve head: hyperemic,edematous, contains hemorrhages retinal veins: dark red, tortuous, dilated dot and flame-shaped retinal hemorrhages macular edema, (later secondary alterations: macular star, white deposits, cystoid macular edema) Therapy: unsatisfactory The underlying systemic disorder must be treated accompanying macular edema: systemic corticosteroid Venal occlusion Complications: neovascular glaucoma therapy: photocoagulation Prognosis depends on the condition of the central retinal artery: Venous stasis retinopathy : central retinal artery is not affected, visual field is full. The prognosis is good Hemorrhagic retinopathy : simultaneous,often transient occlusion of the central retinal artery. Visual field is constricted,visual acuity reduced. The prognosis is poor Branch vein occlusion: Hemorrhagic infarct corresponding to the sector which is drained by the corresponding, specific vein. Predisposing areas: arterio-venous crossings Vascular retinopathy Diseases of the retina 2. Retinal changes in systemic vascular diseases (status of retinal vessels allows a conclusions as to the status of the cerebral vessels) a. Arteriosclerosis Ophthalmoscopic picture: optic nerve head: pale, sharply outlined the wall of the arterioles becomes less transparent (reflex stripes appear) uneven vessel width: hyalinized irregular thickening of the vessel wall general and segmental attenuation of the blood columns Gunn’s sign: at the arteriovenous crossing the hardened artery obscures the underlying veins b. Circinate retinopathy Symptoms: visual disturbances Etiology: arteriosclerosis, diabetes Ophthalmoscopic picture: wreath-like arrangement of white ,hard exudates Ophthalmoscopic picture: a.: Anterior Ischemic Optic Neuropathy (AION) optic nerve head is swollen and shows superficial hemorrhageslater:flat atrophic excavation highly attenuated retinal arterioles b.: normal ophthalmoscopic finding: Posterior Ischemic Optic Neuropathy (PION) Therapy: high doses of corticosteroid Prognosis: poor CARDIOVASCULAR , HYPERTENSION General remarks Benign hypertension: organs remain normal, systolic blood pressure may rise up to 160 mmHg, diastolic up to 90 mmHg Malignant hypertension: a primary idiopathic hypertension or secondary to a cardiovascular, endocrin or neurogenic condition In cases of renal ischemia the ocular changes occur mainly with chronic glomerulonephrosis or with secondary nephrosclerosis. Ophthalmoscopic picture: Benign hypertension (stages I-II ) Stage I. deep red color of the entire fundus optic nerve head: normal arterioles: tortuous, widened, copper wire reflex, thickened vessel wall, Gunn-sign’s, blunt angle of the branching arterioles, veins: normal/irregular caliber, tortuous venules Stage II. optic nerve head: normal arterioles: increasing sclerosis, attenuation, streching, irregular caliber widened light reflexes hard exudates, small multiple retinal hemorrhages Hypertesion angiopathy b.: Malignant hypertension (stages III-IV) Stage III. optic nerve head: ischemic papilledema arterioles: silver wire with increased reflexes from the attenuated blood vessels, markedly thinned blood column cotton-wool patches (ischemic local retinal edema) flame-shaped superficial hemorrhages Malignant hypertonic angiopathy Stage IV. optic nerve head: advanced ischemic edema arterioles: general attenuation, irregular caliber,white sheating silver-wire reflexes, crossing phenomena cotton wool spots, macular star hard fatty exudates flame shaped and dot like hemorrhages, general ischemic pallor Immediate medical treatment is required! Stage:IV severe angiopathy Retinal changes in diabetes General remarks: Benign variety (background retinopathy, limited to the retina) and malign stage (proliferative retinopathy). Insulin dependent juvenile diabetes has a higher tendency to be complicated by proliferative retinopathy, than adult-onset diabetes. Diabetes mellitus is one of the most frequent cause of blindness! Ophthalmoscopic picture: a.: Background retinopathy Juvenile form: capillary aneurisms, ischemic areas, proliferation, hypertrophy of the retinal venules with a formation of a rete mirabile, saccular dilatation of venous branches Senile form: microaneurisms, dot- and blot-like hemorrhages deep yellow hard exudates, fatty deposits (arranged in the shape of wreath) cotton wool spots wessels are of varying caliber and of thickened walls Therapy: laser photocoagulation Prognosis: good Diabetic retinopathy, background Diabetic retinopathy, background FLAG: b.: Proliferative retinopathy newly-formed blood wessels sprout in front of the retina growing on the back surface of the detached vitreous hemorrhages in and beneath the vitreous membrane formation traction retinal detachment Therapy: vitrectomy Prognosis: poor Proliferative diabetic retinopathy Proliferative diabetic retinopathy Proliferative diabetic retinopathy Kimmelstiel-Wilson disease: proliferative diabetic retinopathy associated with cardiovascular hypertension and diabetic nephrosclerosis. Ophthalmoscopic picture: edema of the optic nerve head and the retina, macular star, attenuated arterioles, cotton wool spots, retinal hemorrhages Retinopathy of Prematurity / Retrolental fibroplasia (ROP) disruption of normal development of the retinal vasculature in preterm infants with birth weight less than 2500 gr Symptoms: leucocoria, strabismus, high myopia Etiology:preterm birth and exposure to oxygen Classification of retinopathy of prematurity Stage I. demarcation border between vascularized and nonvascularized retina Stage II. formation of a ridge (development of intraretinal proliferative tissue) Stage III. ridge with extraretinal proliferation Stage IV. subtotal retinal detachment Stage V. total retinal detachment Treatment: cryotherapy, laser photocoagulation, vitrectomy Prophylaxis: partial pressure of oxygen shoud be kept as low as possible ophthalmoscopic screening Early detection is particularly important! ROP ROP ROP Hematologic disease Hemoglobinopathies Pernicious anemia Secondary anemia Leukemia Hodgkin’s disease Polycytemia Dysproteinemia Macroglobulinemia DISEASES OF THE MACULA (AQUIRED OR INHERITED) Senile (age-related) macular degeneration (AMD) Symptoms: deterioration of central vision, good peripheral vision Etiology: degenerative changes or familial disease Ophthalmoscopic picture: a.: dry form of macula degeneration atrophic pale area in the center, foveal reflex is missing irregular areas of pigmentation, partial atrophy of the pigment epithelium drusen (excrescences of the pigment epithelium) Age related macular degeneration AMD non exud. AMD b.: wet form of macular degeneration (disciform macular degeneration, Kuhnt- Junius) serous than hemorrhagic subretinal fluid subretinal neovascularisation hemorrhagic detachment of the RPR) scar formation (gray-white) circinate retinopathy Age related macular degeneration AMD exud. Secondary maculopathies phototoxic effect of artificial light (operations) intensive light (at the eclipse of the sun) drug toxicity (cloroquine) Hereditary macular degenerations (dystrophies) 1. Stargardt’s disease Symptoms: progrediating decrease of visual acuity Ophthalmoscopic picture: a.: early atrophic ,sharply delineated area b.: yellowish circumscribed foci in the macular area and retinal periphery (Stargardt’s fundus flavimaculatus) 2. Vitelliform macular degeneration (Best ) Symptoms: visual loss, photophobia Ophthalmoscopic picture: stages: 1: egg yolk (lipofuscin deposit int he RPE) 2.: hypopyon like(deposit liquified and show a horisontal level) 3.: scrambled egg (the cyst bursts) 4.: scar formation 3. Cone dystrophy 4. Rod monochromatism 5.: X-linked juvenile retinoschisis Hereditary retinal degenerations (progressive,noninflammatory retinal diseases) 1. Retinitis pigmentosa (RP) Etiology: inherited disease Symptoms: night blindness, loss of peripheral vision, central visual loss Ophthalmoscopic picture: pale, waxy-yellow optic nerve head attenuated retinal arterioles (thickened wall) black or brown bone corpuscle-like pigmentation (sometimes:cystoid maculaedema, bull’s eye lesion or macular hole) Therapy : - Tumors of the retina Retinoblastoma (10% are hereditary! , Both eyes!) Signs: amaurotic cat’s eye: yellow-white or gray reflex in pupil strabismus greatly reduced vision Differential diagnosis: persistence and hyperplasia of the primary vitreous retrolental fibroplasia Coats’ disease Toxocara canis infestation metastatic ophthalmia congenital cataract the Thanks for the attention!