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RETINAL DISEASES PRESENTING IN INFANCY 46  Growth of abnormal blood vessels in the retina of some premature infants RETINOPATHY In most premature infants, blood ves...

RETINAL DISEASES PRESENTING IN INFANCY 46  Growth of abnormal blood vessels in the retina of some premature infants RETINOPATHY In most premature infants, blood vessel OF growth continues after birth until the PREMATURITY entire retina contains normal vessels In ROP, blood vessel growth stops after birth, so normal blood vessels do not extend to the edges of retina -not just any premature infant gets ROP -stevie wonders has stage 5 ROP 47  Area of avascular retina needs nutrients and oxygen Usually provided by blood vessels Sends out signals that stimulate growth of new blood vessels that will supply nutrients ROP i.e. VEGF Neovascularization creates leaky vessels – leads to fluid into vitreous Fibrous tissue associated with neovascularization Contracts, pulls the retina away Retinal Detachment and vision loss -avascular retina sends off VEGF, VEGF causes leaky fibrous blood vessels that causes fluid accumulation and traction in the retina which can cause a fluid detachment and tractional detachment 48 RISK FACTORS Low birth weight Normal: 2500-4200g (5lb 8oz – 9lb 4oz) - LBW: less than 2499g (5lb 8oz) VLBW: less than 1500g (3lb 5oz) ELBW: less than 1000g (2lb 3oz) Premature Birth (32 weeks or earlier) 23-24 weeks: examine within 3-4 weeks 25-28 weeks: examine within 4-5 weeks After 29 weeks: examine before discharge Supplemental oxygen or mechanical ventilation >2 weeks after birth Severely ill at birth 49  ZONES Zones: Location of affected retina Zone 1: closest to the optic nerve and macula* Zone 2: mid-peripheral Zone 3: far periphery on temporal side -temporal peripheral retina is what gets vascularized last 50 -zone 3 is where most ROP is -zone 2 à severe ROP -zone 1 à really bad 51  Stage 1 Distinct border between vascularized and avascular retina Vascularized: pinkish and translucent Avascular: whitish and opaque Mildly abnormal blood vessel growth Resolves without treatment Stage 2 Elevated ridge of tissue forms between vascularized and avascular tissue Moderately abnormal blood vessel growth Neovascularization begins forming at ridge STAGES Resolves without treatment -stage 1 à mild -stage 5 à worst 52  STAGES Stage 3 Fibrovascular tissue extends from the ridge into vitreous Severely abnormal blood vessel growth May see thickened and tortuous blood vessels = “plus disease,” indicating a worsening of the disease Treatment is often needed -this is where treatment is required -treatment would be laser photo coagulation, anything to kill the blood vessels 53  STAGES Stage 4 Partial RD that may involve macula - Usually results in VA worse than 20/200 Treatment required Stage 5 Total RD May present as leukocoria Usually LP or NLP -treatment is very required -stage 5 à endstage 54 TREATMENT Cryotherapy Laser Photocoagulation CRYO-ROP study showed Thought to be as patients at stage 3 ROP effective as cryotherapy treated with cryotherapy but with fewer were 50% less likely to complications progress to severe vision May be preferred loss and RD compared to treatment those untreated 55  Poor BCVA Myopia Strabismus COMPLICATIONS Amblyopia Glaucoma Nystagmus Cataracts 56 LEBER’S CONGENITAL Severe visual impairment present at/ AMAUROSIS or within 2 months of birth Responsible for 10- 18% of congenital blindness Autosomal recessive inheritance 57  LEBER’S CONGENITAL AMAUROSIS Diagnosis o Visually inattentive infant o Nystagmus o Severely reduced visual acuity o Poor pupillary light response o Flat or severely reduced ERG o High hyperopia is common o Often associated with mental retardation or deafness -infant is not making eye contact -severely visually impaired 58 LEBER’S CONGENITAL AMAUROSIS Fundus appearance At birth often normal May have white flecks, pigment clumping, or macular pigment dysplasia By adolescence, Pigmentary retinopathy Vessel attenuation Optic atrophy 59  Night blindness, not progressive Variable vision loss usually not worse than 20/200 Normal fundus High myopia and nystagmus Inherited AR or X-linked presents in infancy with reduced vision, nystagmus, high myopia, strabismus, and paradoxical pupils CONGENITAL STATIONARY AD presents later with night blindness and normal NIGHT BLINDNESS visual acuity 60  Absence of functioning cones (AR) Rod Monochromatism VA about 20/200, complete color blindness, nystagmus, photophobia, normal fundus, high hyperopia common Vision better in dim illumination Characteristic OCT appearance ACHROMATOPSIA Incomplete achromatopsia (AR) Similar presentation but better prognosis - VA 20/80-20/120, some color perception Blue cone Monochromatism (X-linked recessive) Normal rod and blue cone function but no red or green cone function -no cones so no color and vision is bad 61  12 YO M REFERRED FOR LV -empty box appearance -no big foveal pit 62 FOVEAL HYPOPLASIA Reduced vision, nystagmus, poorly developed macula/fovea Found in albinism and aniridia May also be an isolated abnormality 63 -no fovea seen 64 -no foveal pit is developed 65