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Anatomy and Function of the Eye and Ocular Disorders Part II Developed by SKI-HI Institute Utah State University Winter 2011 For use in Training The Human Eye retina macula fovea optic nerve optic disc choroid vitreous humor La Clinica Oculistica Virtuale 1 Choroid • If deprived of its blood supply for more than a few minutes, will die • Cannot be regenerated • Nourished by blood vessels that enter eye near optic nerve and which spread across surface of retina • Choroid supplies retina with nourishment 2 Rod and Cone Cells Rods • More in the peripheral retina • Detect motion • Function at low luminance level…night vision • Gross forms and shadow Cones • Color vision • Detail, reading vision 3 Night Vision and Night Blindness http://www.sapdesiguild.org 4 Prematurity and ROP • Factors causing ROP are: excessive oxygen, prematurity, infection, excessive exposure to light National Eye Institute, National Institutes of Health • Babies born today are more premature and lower in weight • Aggressively treated and have a perilous neonatal period full of numerous life sustaining procedures • Medical technology has increased survival, but not the chances of ROP occurring • ROP a major cause of infant blindness in developed countries 5 The Disease Process in ROP • Baby born early, retina has not vascularized yet—blood vessels grow from optic nerve head to outer reaches of retina • Growth stops for a while • Then oxygen causes them to grow wildly, tangle and grow into vitreous, not to edges • Vessels hemorrhage, scar tissue forms and vitreous contracts, damaging retina and causing detachments National Eye Institute, National Institutes of Health 6 Stages of ROP Stage Description of Retina 0 No abnormality 1 Minor changes in periphery of retina; some irregular blood vessels; see white band between area of retina with and area without blood vessels 2 Same characteristics as stage 1; now area without blood vessels looks silvery gray and opaque; band has Increased in height and width 3 Band now forms a thick ridge; lots of blood vessels near the ridge and spreading into vitreous 4 Peripheral retina starts to pull away, moderate detachment 5 Advanced or total retinal detachment 7 Zones of ROP • Described as if retina were the face of a clock • ROP in zone 1 more serious as it may involve the macula, affecting central or reading vision • ROP in zone 1, at 1-2 o’clock position, retinal changes don’t involve the macula; are further away 8 Treatment of ROP Observation/Monitoring • Most babies with ROP get better on their own • For most, disease can get worse fast and without treatment, half will end up blind Surgical Treatments • May be used alone or in combination laser photocoagulation cryotherapy-freezing surgical repair • Length of time between detachment and surgery is crucial to restoring vision 9 Vision Impairment in ROP • Baby’s vision can vary from near normal to total blindness, depending on how changes had progressed prior to diagnosis and treatment • ROP sometimes spontaneously regresses or cures itself and retina returns to near normal; rarely happens after stage 3 • Children with ROP may later develop myopia or near sightedness, strabismus, cataracts, nystagmus, astigmatism, microphthalmia, or blue-yellow color deficits • In the adult years, may develop further myopia, cataracts, closed-angle glaucoma or further detachment 10 Other Disabilities and ROP 70 % of infants born under a pound in weight and 3 months premature have additional disabilities such as: • Brain damage • Cerebral Palsy • Seizures • Hearing Loss • Learning disabilities • Cortical/Cerebral Visual Impairment • Sensory Issues 11 Other Retinal Conditions • Macular Degeneration • Retinitis Pigmentosa • Ushers • Leber’s Amaurosis • Coloboma CHARGE • Rubella • CMV 12 Retinoblastoma • A slow growing malignant tumor that arises from retina • Hereditary or non-hereditary; one or both eyes • Both types gradually fill the eye then extend through the optic nerve to the brain or into sclera and surrounding tissue • Pupil appears white or glows, strabismus present, eye inflamed • Discovered before age of three; diagnosed by ultra-sound, MRI, CT • Treatment: may remove the eye; in some cases chemo or radiation is used; high survival rate • Cataracts may result from treatment 13 Traumatic Retinal Detachment/Hemorrhage Shaken Baby Syndrome (SBS) • Infant forcefully shaken • Retina detaches as result of direct traumatic injury or secondary complication of bleeding in the eye • Visual impairment due to retinal detachment, optic atrophy or damage to visual pathways in the brain Head Injury • Similar destruction as SBS, most commonly occurring in car accident 14 Other Infections Toxoplasmosis • Infection of eye that is the result of maternal exposure during pregnancy to a parasite in cat feces • Can affect the brain as well as the eyes Toxocara • Acute inflammatory response inside eye in response to a parasite in dog feces • Causes cataracts, inflammation of vitreous Herpes • Blood-born viral infection transmitted during pregnancy or delivery that can cause severe damage to brain and retina 15 Loss of Color Vision • More common in males than females • Three types of color photoreceptors in the retina—red, green, and blue cones; defect may lie in ay of these • Most common: an inability to distinguish reds and greens • Present in both eyes and remains constant over time • Occasionally accompanies retinal disease or exposure to toxic materials that damage cones • No known treatment 16 Loss of Color Vision Simulated Normal Red Blue (rare) Green graphics.cornell.edu 17 Normal vision Myopia or near sightedness eyeatlas.com 19 Hyperopia or far sightedness Astigmatism eyeatlas.com 20 Glasses Can’t Fix Every Eye Problem! Dr. Lea Hyvärinen, Helsinki, Finland 21 Eyes in Constant Motion Saccades of eye looking at a photo of a face Artlex.com • Human eye is in constant state of vibration, oscillating back and forth at rate of 60 per second • Serve to refresh the image cast on rods and cones at back of eye • Without these microsaccades, staring fixedly causes distortions • Rods and cones respond best to changes in color and luminance • These saccades are so small they are imperceptible to others • In many children with poor vision, these microsaccades are exaggerated, so we see them as nystagmus 21 Nystagmus • Involuntary rhythmic, jerky eye movement • Vertical, horizontal or rotary • May decrease with age • Slightly blurs vision, central vision decreased • May hold objects closer to eyes • May turn head slightly to use null point • Increases with fatigue, stress, excitement Dr. Lea Hyvärinen, Helsinki, Finland 23 Difficulties with Saccades When Reading • Children with conditions such as nystagmus or strabismus may have inefficient reading saccades • Eye movements may be bigger; less room for error • They may struggle and lose their place • Enlarging the print may help • Using a finger or line marker to keep their place may help • Presenting print on computer line by line or in shorter lines Use of finger to keep place Use of paper as line marker to keep place 23 Random or Roving Eye Movements • Sign of poor acuity; takes good acuity to fixate • As if eyes are trying to find a target, but can’t quite focus on it well 24 Medications That Cause Double or Blurred Vision • May include antidepressants, antidiabetic drugs, barbiturates, cortisones, sedatives, and tranquilizers • Contact physician if any changes are noticed when using such drugs • The side effects can interfere with vision • Many children with multiple disabilities are on seizure medications which can cause visual side effects (blurring, less alert, double vision). • Great handout on this on website: www.fpg.1unc.edu/~edin under Resources, Vision Module, Session 4, handout I 25 The Visual System Wps.prenhall.com 26 Normal Optic Nerve optic nerve macula National Eye Institute, National Institutes of Health 27 Hydrocephalus • Increased fluid in the ventricles or water spaces of the brain • Put pressure on optic nerve fibers Child with Shunt • Prolonged high pressure causes permanent damage www.mps1disease.com • Putting in shunt to drain fluid soon enough can minimize the damage • Decreased visual functioning can be a sign of shunt failure Optic nerve head in back of the eye of 2 year old child with hydrocephalus www.nature.com/ 28 Brain Bleeds Around the Ventricles • Optic radiations pass by and around the lateral ventricles Lateral Ventricle • Low birthweight premature babies may suffer brain bleeds in the ventricles Optic Radiations • These bleeds can damage the optic radiations, resulting in vision loss Lateral Ventricle (other views) • Severity of vision loss depends on the extent of the bleed, treatment, other medical issues, etc. Grade III Bleed in the Lateral Ventricals 29 Optic Nerve Atrophy (ONA) www.nature.com • Loss of blood supply to optic nerve with accompanying gradual vision loss • Optic disc is pale when examined • Change in visual acuity and peripheral field may occur before change in the disc is evident; nerve cells of retina may stop functioning before the nerve atrophies • Degenerative condition following normal function • Not normally diagnosed in infancy 30 Optic Nerve Hypoplasia (ONH) • Small optic nerves with normal size blood vessels • Varying degrees of visual acuity and field losses • Associated with CNS anomalies -agenesis of corpus callosum (failure of two halves of brain to connect) -endocrine disorders • Incidence increasing, possibly as result of drugs, alcohol, tobacco use of mother before birth while pregnant Booklet on ONH from the Blind Childrens Center in Los Angeles, CA 31 Septo Optic Dysplasia de Morsier syndrome • A congenital malformation syndrome manifested by: - hypoplasia (underdevelopment) of the optic nerve - hypopituitarism - absence of the septum pellucidum (a midline part of the brain) • In a severe case, this results in pituitary hormone deficiencies, blindness, and mental retardation emedicine.com • There are milder degrees of each of the three problems, and some children only have one or two of the three 32 Delayed Visual Maturation Type I: child with normal general/neurological development and no underlying pathology; by 3-6 months, child has a rapid improvement of vision to normal or near-normal levels Type II: associated with systematic disorders of mental retardation; vision usually improves, but takes longer and there may be continued loss of vision Type III: associated with other ocular disorders such as albinism, cataracts or aniridia; vision worse than expected from disease alone with mean age of recovery at 20 weeks; onset of nystagmus precedes recovery which is complete by 8 months; also depends on visual abilities and other characteristics of the child 33