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Retina Duke Elder Exam Dr Mostafa Khalil – ST1 Ophthalmology, Glasgow 5 in UK Duke Elder Exam - 2017 FRCOphth part 1 - 2019 Co-founder of Bridging the Gap Academy – medical education platform, check it out Instagram and followLearning objectives •Understand Retinal Anatomy •Understand different medical and surgical retinal conditions relevant for the Duke Elder Exam. •Gain understanding of OCT, FFA, ICG, ERG and EOG •Pass the Duke Elder ExamVitreous This is a transparent gel made of water, type 2 collagen and hyaluronic acid. • Makes 80% of the eye volume (4.4ml) • Composed of central vitreous and peripheral/cortical vitreous. Posterior Vitreous Detachment (separation of posterior vitreous from neurosensory retina): • With age, the vitreous gel liquifies • Liquefied vitreous flows into retrovitreous space • This separated the posterior vitreous hyaloid from the neurosensory retina • Features: Floaters and flashes. No visual loss or change.Retina T opography The retina is a neuroepithelium that lines the posterior segment of the eye, attaching firmly to the optic nerve head and the ora serrata. • If the optic nerve is on the right side, this means it is the right eye and vice versa. • The Macula (central retina) is 5.5.mm and is located temporal to the optic nerve between the temporal vascular arcades • The Fovea is the central 1.5mm of the macula of Mikael Häggström 2014". WikiJournal of Medicine 1 (2). DOI:10.15347/wjm/2014.008. ISSN 2002-4436. Publicical gallery Domain.orBy Mikael Häggström, used with permission., CC0, via Wikimedia CommonsRetina T opography 2 Posterior Pole (Macula and Optic disc) • Between the temporal vascular arcades • The Macula (central retina) is 5.5.mm and is located temporal to the optic nerve between the temporal vascular arcades • The Fovea is the central 1.5mm of the macula • Central fovea is called the umbo and is an avascular zone. Peripheral Retina • Near peripheral retina – 1.5mm ring outside the temporal arcades • Equatorial retina • Peripheral Retina – area outside the equator Ora Serrata • Border between the retina and the ciliary body. Photograph: Danny Hope from Brighton & Hove, UKDiagram: User:Zyxwv99, CC BY 2.0 <https://creativecommons.org/licenses/by/2.0>, via Wikimedia CommonsRetina T opography 2 Posterior Pole (Macula and Optic disc) • Between the temporal vascular arcades • The Macula (central retina) is 5.5.mm and is located temporal to the optic nerve between the temporal vascular arcades • The Fovea is the central 1.5mm of the macula • Central fovea is called the umbo and is an avascular zone. Peripheral Retina • Near peripheral retina – 1.5mm ring outside the temporal arcades • Equatorial retina • Peripheral Retina – area outside the equator Ora Serrata • Border between the retina and the ciliary body. BruceBlaus. When using this image in external sources it can be cited as:Blausen.com staff (2014). "Medical gallery of Blausen Medical 2014". WikiJournal of Medicine 1 (2). DOI:10.15347/wjm/2014.010. ISSN 2002-4436., CC BY 3.0 <https://creativecommons.org/licenses/by/3.0>, via Wikimedia CommonsPhotoreceptor Rods Cones Number 120 million 6 million Location Highest density in the mid-peripheral retina (about 20 degrees from pointHighest density at the macula (especially the fovea) of fixation) Pigment Rhodopsin Iodopsin Wavelength of maximum 498 Three types: short (420), medium (534) and long (564) wavelength cones absorbance(nm) which are sensitive to blue, green and red light respectively. Bipolar connection One bipolar cell can receive stimuli from 50-100 rods Forms a 1:1 ratio with bipolar cells Function Sensitive in dark-dim illumination; responsible for night and peripheral Sensitive for bright light; responsible for central and colour vision visionRetina Histology Ganglion cells 10 layers of retina from two separates embryological cups, neural ectoderm • Retinal pigment epithelium from outer cup, responsible Bipolar cells for absorption of light. • Neurosensory retina from inner cup (9 layers) • Photoreceptors are the rods (120 million) and cones (6 million) Photoreceptors • Information passed from photoreceptors to bipolar cells (rods and cones) which transmit information to ganglion cells • Ganglion cells axons form optic nerve and pass information from the retina to the brain Retinal pigment epithelium <https://creativecommons.org/licenses/by-sa/3.0>, via Wikimedia Commons Retinal pigment epithelium •Functions: •Absorbs light and prevents the scattering of light within the eye. •Contains a blood-retinal barrier which provides a selectively permeable membrane to supply nutrients to the photoreceptors and maintain homeostasis. •Transport and storage of metabolites and vitamins.Blood supply to the retina • including photoreceptors and RPE are supplied by the short posterior ciliary artery (choroid). • The inner two-thirds of retinal layers are supplied by the central retinal artery. • layer of the macula may have a dualner blood supply by the cilioretinal arteries (branch of the short posterior ciliary artery). When present, central vision may be conserved in cases of CRAO.Choroid • Origin: Neural crest • Middle layer of the eye (between sclera and retina) • Highly vascular structure • Supplies nutrients and blood supply to retina • Thickest posteriorly (0.22mm), thinnest anteriorly (0.1mm) Detail: • 4 layers: Bruch membrane, choriocapillaris, Satter, Haller • 5 layers: basement membrane of RPE, inner cBruch membrane: ollagenous zone, middle central band of elastic fibres, outer collagenous zone, basement membrane of choriocapillarisRetina OCT Layers Nerve fibre layer (Ganglion cells) Photoreceptors (rods and cones) Retinal Pigment epithelium Wies6014, CC BY-SA 4.0 <https://creativecommons.org/licenses/by-sa/4.0>, via Wikimedia Commons ChoroidIntraretinal Fluid (within the NSR)Subretinal Fluid (between the NSR and RPE)Pigment epithelial detachment (Sub-RPE fluid) Revolutionised diagnosis and management in ophthalmology • Time Domain – OCT • Spectral Domain – OCT (new), black and white, allows more definition of photoreceptors or ellipsoid zone. OCT – Ocular • Uses near-infrared light interferometry Coherence interference between reflected reference waves and those reflected by tissue. Tomography • OCT angiography – allows visualization of vessels and assessment of choroidal neovascularizationDiabetes in the Eye • Hyperglycemia causes increased retinal blood flow and damage to endothelial walls and pericytes. • Endothelial dysfunction causes vascular permeability and hard exudates formation (lipoproteins in the outer plexiform layer) – hyper-reflectivity within NSR on OCT • Pericyte damage predisposes to the formation of microaneurysms – lead to dot blot haemorrhage • Infarct of retinal nerve fibre layer – cotton wool spots, seen as hyper-reflectivity within nerve fibre layer on OCTDiabetes in the Eye •Can see dot blot haemorrhage and cotton wool spotDiabetes in the Eye •Exudates in macula •Intraretinal fluid at Macula • Duration of Diabetes: Most important risk factor. • Poor diabetic control: • 37% reduction in progression rate of diabetic retinopathy with 1% reduction in HbA1c. • Rapid improvement of diabetic control may Risk Factors transiently worsen retinopathy. Ris Fac rs • Type 1 diabetics may exhibit a superior benefit with good glycaemic control than type 2. • Pregnancy. • Others: hypertension, hyperlipidemia and smoking. Diagnosis of diabetes • Absence of neovascularisation Non • Mild: 1 dot/blot haemorrhage in any proliferative quadrant •quadrants), venous beading (in 1 quadrant), or Diabetic mild intraretinal microvascular abnormality (IRMA) Retinopathy • Severe: Intraretinal haemorrhages in 4 quadrantVenous beading ≥2 quadrants. moderate IRMA ≥1 quadrantNon proliferative Diabetic Retinopathy Diagnosis of diabetes Proliferative Diabetic • Presence of neovascularisation Retinopathy • Characterized as NVD, NVE, rubeosis iridis, NVA, • Treatment: pan retinal photocoagulation • Advanced leads to tractional retinal detachment Diagnosis of diabetes Diabetic Maculopathy • Presence of macular oedema (IRF within macula on OCT). • Treatment: Anti-VEGF injection e.g. Eylea x3 monthly intervals. Anti-vascular endothelial growth factors Anti-VEGF A protein that induces angiogenesis, • Lucentis (Ranibizumab) vascular permeability,and • Molecular weight of 49kDA inflammation. • humanised IgG1 monoclonal antibody fragment that binds all isoforms of VEGF-A Anti-VEGF therapies reduce vascular hyperpermeability: • Eylea (Aflibercept) resolution of IRF,SRF. • Molecular weight of 115kDA • Fusion protein, binds VEGF-A with higher affinity than lucentis, Halt/delay progression of as well as binding of PIGF 1 and 2. neovascularisation. • Beovu (BROLUCIZUMAB) • 26kDa smaller anti-VEGF – smallest molecular weight • Humanised single-chain antibody fragment that targets VEGF-A. • ? Benefit of lesser injection needed • SE profile: uveitis (common 2.2%), retinal vasculitis 3.3%.Diabetes in the Eye •Diagnosis and treatment?Diabetes in the Eye •Diagnosis and treatment?Diabetes in the Eye • Non-proliferative moderate/severe (4-6 month review) in ophthalmology clinic • Screening: Pre-proliferative R2 – refer to ophthalmology. R1(mild) annual review • Proliferative DR – urgent PRP within 2 weeksDiabetes in the Eye Diagnosis and treatment?Vitreous haemorrhage • Causes: PDR, PVD, Retinal tears, trauma, RVO, Proliferative sickle cell retinopathy, nAMD, ROP, Eales disease…. • Management: • No fundal view: B-Scan USS to rule out detachment • Close monitoring, every 2 weeks e.g. • Check other eye for clues e.g. DR in other eye • Urgent PRP if process of neovascularisation and fundal view • Vitrectomy if persistent VHEales disease • Idiopathic peripheral retinal periphlebitis. • young Indian males. • Recurrent vitreous haemorrhages. • CRVO vs BRVO •Occlusion at or proximal to the lamina cribrosa of the optic nerve where the retina artery exits the eye leads to CRVO •Hypertension – most common association in BRVO • Ischaemic vs non ischaemic • Presentation: •Painless loss of vision. •Tortuosity and dilatation •dot/blot haemorrhage, macular oedema. •4 quadrants vs 1 (superotemporal most common). •Neovascularisation (NVI > NVD > NVE) •100 day glaucoma (rubeotic Retinglaucoma)cclusionRetinal vein Occlusion •Non-Ischaemic • if Va >6/96 + OCT macular oedema 🡪 anti-VEGF. • Ozurdex implant if high cardiovascular risk •Ischaemic Management • Monitor. Urgent PRP if neovascularisation, open angle • cyclodiode laser if neovascularisation and closed angle. Retinal artery occlusion • Central (Atherosclerosis. Others: GCA!) ; Branch (embolic) • Central • Sudden painless loss of vision. (CF unless Cilioretinal artery spared) • Marked RAPD • Pale retina + cherry red spot, arteriolar attenuation • Branch • Altitudinal field loss. Similar fundoscopy to CRAO. •OCT – thickened/oedematous outer retinal layersCystoid Macular Oedema ▪ Diabetic macular oedema ▪ CRVO and BRVO ▪ Age related macular degeneration ▪ Uveitis typically pars planitis but also occurs in anterior and posterior uveitis. ▪ Retinitis pigmentosa ▪ Post-cataract surgery: Irvine gass syndrome – 6 weeks ▪ Drugs - nicotinic acid - Prostaglandin analogues (e.g. latanoprost) - Epinephrine Age Related Macular Degeneration • Degeneration of the retina • Non-neovascular (progressive) • Drusen at the macula (yellow deposits between Bruch’s membrane and RPE) • RPE atrophy/loss/geographic atrophy • Subretinal fibrosis/disciform scar • Neovascular – ingrowth of choriocapillaris into RPE/retina • Choroidal neovascular membranes: subtypes 1-3 • Sub-RPE fluid (type 1), can be called occult CNMV on FFA • Sub-retinal fluid (type 2), can be called classic CNMV on FFA • Retinal angiomatous proliferation (RAP, type 3 CNVM), IRF, SRF, sub-RPE fluid. Age Related Macular Degeneration •Increasing age: most important risk factor •Genetics: CFH and ARMS2 genes •Smoking •Hypermetropia •Hypertension •Female •White race •Cell death - apoptosis Dry Age Related Macular Degeneration DrusenNeovascular Age Related Macular Degeneration Drusen CMONeovascular Age Related Macular Degeneration Drusen CMO Neovascular Age Related Macular Degeneration Drusenoid PED SRF •Dry – modifiable risk factors. Vitamin C & E, lutein, zeaxanthin and zinc. Note beta carotene increases lung cancer (not used). Management of ARMD •Wet – Anti-VEGF x 3 monthly intervals e.g. Eylea. •Low vision Aids: Magnifiers vs Telescopes (Galilean telescope)Polypoidal Choroidal Vasculopathy • Polypoidal choroidal vasculopathy – variant of wet AMD • Polypoidal dilation of choroidal vasculature. Middle aged Asian populations. Unilateral. • Usually peripapillary location, choroidal neovascular membranes. • Investigation of choice: ICG – focal hyper fluorescence spots. • Management: Photodynamic therapy. Other option to observe or anti-VEGF Sickle cell retinopathy •Disorder that affects the beta haemoglobin subunit of RBC •African-Caribbean origin. Non-proliferative Proliferative retinopathy (Goldberg classification): ▪ Intraretinal haemorrhages (‘salmon 1. Peripheral arteriolar occlusion patches’) 2. Arteriovenous anastomosis ▪ RPE hyperplasia (‘black sunbursts’) 3. ‘Sea fan’ neovascularization 4. Vitreous haemorrhage 5. Tractional/rhegmatogenous retinal Detachment https://imagebank.asrs.org/file/18293/sickle-cell-sea-fan-retinopa thy, American society of retina specialistsCentral Serous Chorioretinopathy • Presentation • Unilateral drop in VA, metamorphopsia, central scotoma • slow recovery from bright light • Middle aged Men. Type A personality. Corticosteroid use. • Investigations • OCT: Triangle-shaped subretinal fluid. Drusenoid appearance may be present. RPE dysfunction •Management • Observe then PDT if no recovery.Degenerative retinoschisis Degenerative retinoschisis is splitting of the retinal layers between the outer plexiform and inner nuclear layers. • Common in hypermetropes. • Commonly located bilaterally and symmetrically in infero-temporal quadrants. • Appearance: Microcystoid degeneration with smooth immobile convex elevation of retina with no demarcation line of chronicity. • Associated with absolute field defectMacular telangiectasia • outer nuclear layer and ellipsoid zone with loss of • Cystic intraretinal cavitation • Secondary CNV • Stable disease course – rarely poor vision • Signs: crystalline deposits, right angled venules. Good visual acuity • OCT: Loss of ellipsoid zone. Intraretinal cysts. A Pathological Myopia • High Myopia defined as -6D or worse • Posterior staphyloma – hallmark (A) • Complications • Chorioretinal atrophy • Lacquer cracks (breaks in Bruchs membrane) (B) • Choroidal neovascular membranes B with associated fibrosis (C) C CPathological Hypermetropia • High Hypermetropia defined as +5.25D or worse • Complications • Pseudopapilloedema • Crowded discs • Angle closure glaucoma • Microphthalmia/nanophthalm ia with uveal effusion syndrome (exudative RD). • Choroidal folds (also caused by tumours, hypotony (IOP<6), posterior scleritis, graves disease) - images Angoid Streak • Bilateral symmetrical irregular cracks in Bruch membrane that radiate from optic disc and surround it concentrically • Fundus examination shows peripapillary atrophy with multiple irregular streaks radiate in a circular pattern as brown irregular lines. • Idiopathic or associated with Pseudoxanthoma elasticum – • ‘Plucked chicken’ appearance of skin usually in the neck, inguinal folds, antecubital fossa. Albinism •Melanin synthesis of the eye only (ocular albinism- XL inheritance) or, more commonly, the eye, skin and hair (oculocutaneous albinism- AR inheritance). •Ocular features: •Symptoms: dVA due to foveal hypoplasia •Signs: nystagmus, strabismus and iris/fundal hypopigmentation resulting in a ‘pink eye’ appearance. •The optic chiasm contains more crossed fibers than normal. Hydroxychloroquine toxicity • Full ophthalmic examination within first year of starting then annual screening after 5 years of therapy. • Bulls eye maculopathy – late sign (differential includes Stargart disease, rod-cone dystrophy). • OCT – Early detection with parafoveal loss of ellipsoid zone • Fundus autofluorescence (FAF) – increased signal in parafoveal region. • 10-2 Humphries visual field – parafoveal loss • Esterman visual fields – binocular vision.Solar toxicity • Sungazing or eclipse viewing • Central/paracentral scotoma • OCT – focal loss of ellipsoid zone at the level of the foveaFundus autofluorescence (FAF) • Images detect fluorophores (e.g. lipofuscin) within the RPE. • Useful in Best disease, Stargart disease, hydroxychloroquine toxicity. Best disease •AD inheritance. BEST1 Mutation. •Degeneration of the macula associated with lipofuscin accumulation in the RPE and atrophy of the photoreceptor layer of the retina. •Features •Bilateral condition associated with hypermetropic patients. •egg Yolk / Vitelliform lesion in macula • In adults – called adult vitelliform disease. •FAF: hyperfluorescence •ERG: normal •EOG: Abnormal (Reduced Arden ratio)Stargart disease •Most common inherited macular dystrophy •AR inheritance. Mutation in the ABCA4 gene on chromosome 1 Signs • Early: yellow white ‘pisciform’ flecks in RPE. Most visible with autofluorescence. • Late: Beaten bronze appearance of the macula that can progress to geographic atrophy with a ‘bull’s eye pattern’ (Second and Third row (arrows)). •FFA: ‘dark choroid’ (accumulation of lipofuscin in pigment epithelium blocks choroidal fluoresence).How is this test performed? • Intravenous injection of sodium fluorescein. Fundus images are then taken 1second intervals FLUORESCENCE FUNDUS ANGIOGRAPHY (FFA) what are the wavelengths of light involved in this test? • Fluorescein is stimulated by blue light (490nm) and emits green light (530nm). Give 2 side effect of sodium fluorescence • Transient skin and urine discoloration. Anaphylaxis Give 2 emergency medications to stand by • 1:1000 Adrenaline 0.5mg. Hydrocortisone, Chlorphenamine. Contraindications? • Pregnancy, Allergy Explain the different types of hyperfluoresence • transmission defect (haemorrhage), filling defect (vein occlusion). Explain the reasons for hyperfluoresence • Window defect (RPE atrophy), Leakage of dye (CMO), pooling of the dye (retinal detachment)tolerance in photophobic patients, 4. better visualisation through media opacities. why choroid not visualised by FFA? RPE absorbs fluorosceine sodium, fluorosceine molecules rapidly extravasate from choroidal circulation. Why is leakage of dye slower in ICG than FFA? because ICG is more bound to plasma proteins (98%); FFA is 80% bound. Contraindications of ICG? Uraemia, seafood allergy, iodine allergy, pregnancy.,3. betterTumours •B-Scan USS first •Choroidal Naevus •Choroidal Melanoma •Choroidal Mets •Retinoblastoma •Retinal Astrocytic Hamartoma •Von Hippel Lindau syndrome USS Describe the underlying physical principle • Ultrasound scan is based on acoustic impedance of tissues. • A piezoelectric crystal transducer converts electric energy to high frequency sound waves that travel through tissues. • the reflected signal (EHCO) is detected by a transducer and its magnitude is measured. A - Scan: plots intensity of echo vs time delay. B -scan : 2D image from multiple transverse A scans.USS Retinal Detachment Choroidal Melanoma Choroidal Naevus • Most common intraocular tumour • More prevalent in Caucasians • malignant)nign (1:8000 become Features: • Smooth margins, uniform, no invasion, no necrosis. • AsymptomaticChoroidal Melanoma • Most common uveal tract melanoma. • Unilateral • Collar Stud appearance, dome shaped elevation • Spread to liver – first 3 years • Gene: Monosomy in chromosome 3 (aggressive form) • USS: low internal reflectivity, large dome shaped lesion (mushroom shape) • Features: • Vision loss • Elevated subretinal mass, associated SRF • Irregular shaped, colour.Choroidal Metastasis • Choroid most common site of metastasis • Highly vascular • Breast in females • Lung in Males • solitary, nonpigmented associated subretinal fluidRetinoblastoma •Most common intraocular malignancy in children •Arises from embryonal photoreceptor cells of the retina •Associated with a mutation in the tumour suppressor gene RB1 on the long arm of chromosome 13q14. •Pathological findings: Flexner Rosettes are classic, but Homer Wright rosettes and fleurettes may exist. Genetic classification: 1) Hereditary germline (usually bilateral) or 2) somatic nonheritable (unilateral).Retinoblastoma •Average age of diagnosis is 3. Parents often notice loss of red reflex on photographs. •Unilateral (sometimes bilateral) leukocoria + strabismus +/- red eye. •Fundoscopy: white round mass with either endophytic (towards vitreous) or exophytic growth (towards RPE/choroid)Retinal Astrocytic Hamartomas • Glial tumors of the retinal nerve fiber layer that arise from retinal astrocytes. • Commonly referred to a "mulberry lesions” - multinodular appearance • Features: • celevated lesions.circumscribed, • Associations • Tuberous sclerosis (TS) • Neurofibromatosis • Retinitis pigmentosa (non-calcified lesions) Credit to: Jesse Vislisel, https://webeye.ophth.uiowa.edu/eyeforum/atlas/pages/retinal-astrocytic-hamartomas.htm Von- Hippel Lindau • AD condition affecting VHL gene on short arm of chromosome 3. • Multisystem disorder affecting brain, spinal cord, retina, kidneys, adrenal gland and pancreas. • Extraocular conditions: • Pheochromocytoma • Hemangioblastoma • Renal cell carcinoma • Ocular Credit to: West coast retina, Robert N • Retinal Capillary Hemangioma – Diagnostic. Angioma with feeder vessels Johnson, characteristic of VHL. http://westcoastretina.com/feb-2011.htmlHereditary vitreoretinal degenerations •X-linked Retinoschisis •Stickler Syndrome •Wagner SyndromeX-linked retinoschisis •X-linked retinoschisis is due to intercellular adhesion abnormalities which leads to splitting of the inner retina at the nerve fibre layer. Problem with Muller support cells. •Features •Early childhood: hypermetropic boys present with reading difficulties at primary school. •X-linked retinoschisis on FFA.ination: Bilateral maculopathy, Foveal schisis with spoke like folds separating cystoid spaces. Resembling CMO without leakage •Investigation •Scotopic ERG (-ve ERG): normal a wave, loss of b wave with oscillatory potentials.Stickler syndrome The most common cause of inherited retinal detachment. This condition is autosomal dominant and is most commonly due to defect in type 2 collagen (COL2A1). •Features •Eyes: high Myopia, cataract, ectopia lentis, glaucoma, optically empty vitreous, giant retinal tears, retinal detachment (lifetime incidence 50%). •Ears: Sensoineural Hearing loss (>60%)Stickler syndrome Pierre robin features: Micrognathia (mandibular hypoplasia), glossoptosis, high cleft, high arch palate, flat nasal bridge, maxillary hypoplasia. •Marfanoid features. •Management ••vitrectomy if RD occurs. •(360 degrees’ retinal laser)Wagner syndrome •This AD disorder is similar to stickler syndrome but without the systemic features. •Features •Low Myopia, cortical cataract, optically empty vitreous. 50% develop Retinal detachment by the age of 60. •Investigation •Gross atrophy of choriocapillaris with non perfusion on Fundus Fluorescein angiography.Retinitis pigmentosa •Photoreceptor dysfunction (rods then cones) and degeneration of retinal tissue •Mutation in the rhodopsin gene in the long arm of chromosome 3 •Inheritance can be AD (most common, least severe), AR or XL inheritance (worst prognosis) •Clinical Features •Symptoms: nyctalopia (night blindness) and tunnel vision (peripheral vision loss).•Triad: Pale optic disc (waxy disc) + bone spicules + arteriolar attenuation.Retinitis pigmentosa related conditions Usher Syndrome Bardet-Biedl syndrome •AR inheritance. AR inheritance. Bull’s eye maculopathy (cone-rod •Most common inherited cause of deaf-blind. Associated sensorineural hearing loss. dystrophy is more common than RP-like retinopathy • Associated learning disability, polydactyly Refsum syndrome and obesity. •AR inheritance Bassen Kornzweig syndrome •Accumulation of phytanic acid •Associated anosmia, peripheral neuropathy and AR inheritance ichthyosis. Abnormal absorption of fat soluble vitamins (Vitamin A & E deficiency). Associated spinocerebellar ataxia, acanthocytosis.Retinopathy of Prematurity •Risk factors •Premature born infant (<32 weeks’ gestation) •Weight less than 1500g. •Extended oxygen treatment; for example, in neonatal respiratory distress syndrome. Systemic illness (sepsis, necrotising enterocolitis).Classification of ROP • Location (optic disc is the center): •Zone 1: 30° from optic disc to radius 2x distance from disc to fovea •Zone 2: Zone 1 to nasal ora serrata •Zone 3: from zone 2 to rest of retinaClassification of ROP Stage 1 (Photo A): White demarcation line separating vascular from avascular areas Stage 2 (B): Ridge: elevated and thickened line Stage 3 (C): Neovascularization Stage 4 (D): Partial Retinal detachment: 4a) Extrafoveal 4b) foveal Stage 5: Total Retinal detachment Plus disease (Photo E): Increased venous dilatation and/or Arteriolar tortuosity of posterior retinal vessels in 2 or more quadrants.Screening •All babies born less than 32-week gestation and/or weighing less than 1500g: •If born < 27-week gestation : screen at 30-31 weeks. •If born > 27-week gestation : screen after 4-5 weeks. •Screen weekly if stage 3 disease, plus disease or if vessels end at zone 1. Otherwise, screen 2-weekly.Management •Treatment is usually within 72 hrs with transpupillary diode laser. if: •Zone 1, any stage with plus disease •Zone 1 stage 3 without plus disease • Zone 2, stage 3 with plus diseaseCoat’s disease •This is a congenital, unilateral condition characterised by telangiectasia and neovascularisation. Commonly affects young boys (85%). •Features • Reduced VA, strabismus and leukocoria. •Retinal telangiectasia (commonly temporal) and ‘light bulb’ microaneurysms. •Intra/sub-retinal exudation. •Complications: exudative RD. 1 in 40 will • Abnormalities develop retinal in the breaks peripheral associated with retina. those abnormalities Lattice Degenerative degenerations retinoschisis • Peripheral Retinal Degenerations. Lattice degenerations • Thinning of neurosensory retina with vitreous liquefaction and vitreoretinal adhesions. • Characterised by circumferential zigzag white lines with round holes within the lesion. • It is the most important degeneration predisposing to retinal tears. • It is present in around 6-8% of the normal population and is more common in myopes. • Important to note that 30% of patients with Rhegmatogenous retinal detachment have lattice degenerations. • Treatment should only be offered to those patients with a retinal detachment in the contralateral eye in the form of laser Credit to: Eric chin and Stefani karakas, retinopexy. https://webeye.ophth.uiowa.edu/eyeforum/atlas/ pages/perivascular-lattice-degeneration.htm Posterior vitreous detachment(PVD) • Separation of the posterior vitreous cortex from the neurosensory retina (NSR). • Occurs commonly with age as the vitreous becomes more liquefied which results in empty pockets of fluid in a process called vitreous syneresis. • Eventually fluids escapes in the retro-vitreous space separating the posterior hyaloid from the NSR. • A simple PVD is associated with retinal tears in 10% of patients. • A PVD associated with vitreous haemorrhage is associated with retinal tear in >70% of patientsPVD Image 1: pockets of fluid formed Image 2: Separation of posterior vitreous in vitreous cavity hyaloid from NSR (PVD)Features & Management • Temporal photopsia (flashes) with associated floaters. • Visual acuity not affected. • vitreous cortex avulsed from optic disc. • negative.gn (tobacco dust) • If complicated with retinal tear, treat with laser retinopexy. • N.B. PVD impedes progression of diabetic retinopathyRetinal breaks • These are full thickness retinal defects • Treat if • High Myopia (>6D) • Giant retinal tear • Symptomatic U shaped retinal tear • Systemic disease such as stickler syndrome • Types 1. Retinal Tear 2. Retinal DialysisRetinal Tear • A full thickness U shaped defect due to traction from posterior vitreous detachment at a causing a teare NSR causing a tear. • Progression tordRD Traction at a point on NSR Retinal Tear cases. in 1/3 ofFeatures & Management • Flashes, floaters. Weiss ring, shafer sign +ve (tobacco dust). • All symptomatic U shaped retinal tears should be treated with laser retinopexy Image showing tobacco dust (shafer sign) characteristic of retinal tear. Retinal dialysis • A disinsertion of the retina at the ora serrata involving less than 3 clock hours. • Idiopathic dialysis are more common and located inferotemporally. • However if due to trauma, it is located superonasally. • retinal detachment in children and young adults • Laser retinopexy – If there is no associated RD http://retinagallery.com/displayimage.php?album= • Scleral buckle – If there is related RD. 384&pid=4084#top_display_mediaScleral buckle https://www.texomaretina.com/services-2/common-treatments/scleral-buckle/ Retinal Detachment Retinal detachment is the separation of the neurosensory retina (NSR) from the retinal pigment epithelium (RPE). 1. Rhegmatogenous retinal detachment 2. Tractional retinal detachment 3. Exudative retinal detachmentRhegmatogenous Retinal Detachment • Detachment of the NSR from the RPE. • Vitreous liquefaction 🡪 PVD 🡪 Retinal Tear 🡪 Shifting of fluid Retinal Tear Retinal Detachment into subretinal space 🡪 Retinal detachment.Features • Flashes and floater • Curtain like visual field loss (Relative field loss) • Decreasing visual acuity (if macula is involved) • RAPD, Weiss ring, shafer sign. • Retinal break: most commonly U-shaped retinal tear, most common location is supero-temporal (60%). • Fresh retinal detachment: (1) Convex and corrugated dome shaped surface with loss of RPE markings. Extend from ora serrata to optic disc. (4) They are progressive and may affect macula. • Chronic retinal detachment: Retinal thinning, demarcation lines, intraretinal cysts, proliferative vitreoretinopathy.Management • Indirect ophthalmoscopy with scleral depression to visualise the ora serrata • B-scan USS if there is no view of posterior pole such as dense cataract or Vitreous haemorrhage • Management • Pars Plana Vitrectomy Tractional Retinal Detachment • This type of retinal detachment is due to vitreoretinal traction. • Fibrovascular membranes cause progressive contracture over areas of adhesions eventually causing a detachment. • Causes: proliferative diabetic retinopathy (PDR) and retinopathy of prematurity (ROP). Features • Visual acuity affected once macula is threatened causing distortion of vision • Detachment: shallow immobile concave tenting of retina. • Associated relative visual field loss, signs of underlying disease e.g. PDR. Management • Surgery is difficult and therefore delayed till the macula is threatened: • Vitrectomy with membrane peel.• Fluid leakage occurs under the retina thereby separating the neurosensory retina (NSR) from the retinal pigment epithelium (RPE). • Occurs when the compensatory mechanisms of the RPE fail to pump the fluid back into the choroidal circulation. Causes • Tumors: Choroidal tumors • Idiopathic: Nanophthalmia leading to uveal effusion syndrome. • Vascular: Exudative age related macular degeneration, Coat’s disease, central serous chorioretinopathy. • Inflammatory: • Posterior uveitis: sympathetic ophthalmia and vogt koyangi harada disease (VKH) • Posterior scleritis, orbital inflammatory disease, post-op inflammation and extensive panretinal photocoagulation.Features & Management •Floaters if vitritis present; distortion of vision if macula affected. •RD: smooth convex that is shallow or bullous. Lack of PVD and retinal tears and evidence of traction. •Evidence of underlying condition. Management •Treatment of underlying cause treats the retinal detachment, surgery not usually indicated.Choroidal Detachment • This is detachment of the choroid from the sclera due to accumulation of fluid or haemorrhage in the suprachoroidal space. This can be serous or haemorrhagic. Causes • Hypotony: post glaucoma filtration surgery or cyclodestructive procedures • Trauma Features • Smooth convex dome of dark retinal colour. • Arise from periphery, Anteriorly the ciliary body to the vortex veins posteriorly. The ora serrata becomes easily visible. • Choroidal detachments may touch “kissing choroidals” leading to retinal detachment.Choroidal rupture • A break in the choroid may result from closed globe injury from blunt trauma. • Disruption of the choriocapillaries, bruch membrane and RPE, however, the neurosensory retina is unaffected. Features • a crescent shaped yellow subretinal streak is seen concentric to the optic disc. Credit to: Austin R Fox, Doreen Dykhuizen, https://webeye.ophth.uiowa.edu/eyeforum/atlas/page s/choroidal-rupture.htmlUveal effusion syndrome • This is most commonly seen in middle aged hypermetropic men and is associated with nanophthalmos. • This may be due to impaired trans-scleral fluid drainage from the choroid leading to choroidal effusion and exudative RD Features • Ciliochoroidal detachment followed by exudative retinal detachment • Leopard spot mottling of RPE (this occurs following resolution of the condition). Management • Full thickness sclerotomiesFull thickness Macular hole •Defined as a defect in the fovea with splitting of all neurosensory retinal layers from the internal limiting membrane to the RPE. Features •Central visual acuity is affected along with metamorphopsia. Classification IVTS classification Gass classification Vitreomacular adhesion Stage 0 Vitreomacular traction Stage 1 – impending macular hole Small or medium macular hole with VMT Stage 2 – small macular hole Medium or large macular hole with VMT Stage 3 – large macular hole Small, Medium or large macular hole Stage 4 – full thickness macular hole with PVD without VMTManagement •Stage 1 – Observe •Stage 2 - Vitrectomy or intravitreal Vitreolysis with Oculoplasmin. •Stages 3 and 4 - Vitrectomy, internal limiting membrane peel and gas tamponade.