W20: Other ocular and systemic conditions impacting on visual function

What is this condition

All RD

2nd pic=Tractional

3rd pic=Exudative-pockets where the retina has come off, fluid under retina in the subretinal space

What are the types and causes of retinal detachment?

• Rhegmatogenous Retinal Detachment (RRD)

  • Cause: retinal break

• Tractional (TRD, retina pulled away by contracting vitreoretinal membranes eg ERM)

  • Causes: proliferative DR, retinopathy of prematurity, toxocariasis, trauma, previous giant retinal tear

• Exudative (fluid derived from the choriocapillaries gains access to subretinal space)

  • Causes: neoplastic choroidal lesions, inflammatory disease, optic pit, choroidal coloboma, vascular disorders, nanophthalmos

What is this condition?

Rhegmatogenous Retinal Detachment

Colours appear to change/lose focus can’t see vessels as clearly

Name the condition

Retinal Tear/ Hole RRD

Retinal tear/hole RRD

• Risk factors

• Pathophysiology

• Risk Factors

  • Lattice degeneration

  • High myopia/ structural change in axial length

  • Prev HST/ RD

  • Aphakia

  • Pseudophakia

  • Age-related retinoschisis

  • Trauma

  • Hereditary disorders

    • Stickler’s Syndrome

    • Marfan’s Syndrome

• Pathophysiology

  • PVD- HST

  • Traction, sub retinal fluid

  • Tissue weakness, partic true in genetic conditions

Name the condition

Retinoschisis

Splitting of retina in any area-central/periphery may not be across the entire

What are the types of retinoschisis and management?

• Juvenile X linked retinoschisis

  • Foveal schisis: cystoid spaces with a “bicycle-wheel” pattern of radial striae

  • Peripheral schisis

  • Vitreous haemorrhage

• Age-related retinoschisis

  • Immobile dome shaped elevation of the retina typically inferotemporally

    • IT in older retinas

  • May progress circumferentially

  • High myopia

• Management

  • Retinal examination with sclera indentation to exclude retinal break

  • A fundus contact lens evaluation

    • Gonio lens to see up to ora serrata

  • No treatment for foveal schisis (stellate maculopathy)

  • Surgical repair of RD

What is diabetic retinopathy and its classifications?

• Preventable Cause of Blindness

• Several Classifications

  • Background

  • Pre-proliferative

  • Proliferative

  • Maculopathy

  • Rubeosis

    • Affects aq humour outflow →ACG or OAG

Can all affect VA,VF,colour vision,CS

Can lead to early cataract

Name this condition

Proliferative DR

Proliferative DR

• Clinical signs

• Fluorescein angiography

• Treatment

• Clinical signs

  • NVD, NVE, NVI

  • Fibrovascular tissue along the posterior surface of the vitreous

  • Fibrovascular tissue extending into the vitreous and adherent to the retina

  • Traction retinal detachment

  • Vitreous haemorrhage

• Fluorescein angiograpy

  • Highlights the neovascularisation

  • Hyperfluorescence due to leakage from neovascular tissue

• Treatment

  • PRP

  • Surgical treatment of retinal detachment

  • Pars plana vitrectomy in vitreous haemorrhage

  • Anti VEGF treatment with caution

  • Follow up every 4 weeks 7

Name this condition

Diabetic maculopathy And DME

1st pic=Dot-blot haems + exudates

2nd pic=OCT=Cystic spaces ,vitreous has alr come off

3rd pic=Wide field FA, various areas w/ hyperfluorescence where leakage present

Diabetic maculopathy And DME

• Clinical signs

• Fluorescein angiography

• Treatment

• Clinical signs

  • May be present and include signs of any of the stages listed

  • Well-circumscribed or diffuse retinal thickening

  • Complete or incomplete rings of perifoveal hard exudates

  • Dark-blot haemorrhages

• Fluorescein angiography

  • Ischemic-capillary non-perfusion at the fovea

  • Diffuse exudative – late diffuse hypefluorescence due to leakage

  • Focal exudative – focal hyperfluorescence, good macular perfusion

• Treatment

  • Focal/ Grid Laser if clinically significant macular oedema (CSMO)

  • Anti-VEGF

  • Periocualr/ intravitreal steroid

Name the condition

Retinal Vein Occlusions- CRVO, BRVO, Hemi-retinal Vein Occlusion

Describe the picture

BRVO

Only ½ of retina affected-macula affected

Depending on which venous branch is affected through the occlusion macula may be spared

Describe the picture

HRVO

CWS + haemorrhaging

Name the condition

CRVO

More flame shaped hems-w/in NFL layer (arcuate shape of nerve fibres across the retina)

Retinal Vein Occlusions- CRVO, BRVO, Hemi-retinal Vein Occlusion

• Acute features

• Late features

• Classification

• Painless loss of vision

• Acute features

  • Diffuse retinal haemorrhages

  • Dilated tortuous retinal veins

  • Cotton-wool spots

  • Optic disc oedema

  • Macular oedema

• Late features

  • Collateral venous channels

  • Hard exudates, Microaneurysms

  • NVE, NVD

  • NVI, Rubeotic glaucoma

  • Fibrous proliferation

    • →tractional RD

• Classification

  • BRVO

  • CRVO

    • Entire retina affected

  • Hemi retinal Vein Occlusion

    • ½ of retina affected

Name the condition

Central Retinal Artery Occlusions

Pale fundus with cherry red spot

Name the condition

Branch of central retinal artery affected

Low contrast area

Name the condition

Cherry red spot in the retina

Retinal Artery Occlusions

• Symptoms

• Clinical signs

• Causes

• Symptoms

  • Painless, abrupt loss of vision (central retinal artery occlusion -CRAO) or partial visual field (branch retinal artery occlusion-BRAO) within seconds

• Clinical signs

  • Superficial opacification or whitening of the retina

  • Cherry-red spot in the centre of the macula (in CRAO)

  • Narrowed retinal arterioles

  • Cotton-wool spots (in BRAO)

  • Afferent pupillary defect

  • Retinal arteriolar emboli

    • esp if BRAO

• Causes

  • Embolus

  • Thrombosis

  • Giant cell arteritis

  • Other collagen-vascular diseases

  • Hypercoagulation disorders

  • Carotid dissection

  • Trauma

Ask about cardiovascular conditions e.g prev thrombosis in other body parts, or diagnosed with carotid artery stenosis -build up of abnormal tissue could break loose

Name the condition

Sickle Cell Retinopathy

Sickle Cell Retinopathy

• Non proliferative retinopathy

  • Clinical signs

• Proliferative retinopathy

  • Clinical signs

• Management

• Non proliferative retinopathy

• Clinical signs

  • Tortuous veins

  • Silver-wiring of arterioles in the peripheral retina

  • Salmon patches-pink pre retinal haemorrhages

  • Black sunbursts-hyper pigmentation due to RPE hypertrophy

• Proliferative retinopathy

• Clinical signs

  • Stage 1: peripheral arteriolar occlusion

  • Stage 2: Peripheral arteriovenous anastomoses

  • Stage 3: New vessels from the anastomoses (“sea- fan” configuration)

  • Stage 4: Vitreous haemorrhage

  • Stage5: Fibrovascular proliferation and tractional RD

• Management

  • Control underlying disease

  • Laser treatment of neovascularisation

Name the condition

Retinal artery macroaneurysm

Some visual function can’t be recovered

Retinal artery macroaneurysm

• Clinical findings

• Work up

• Treatment

• Clinical Findings

  • “Hemorrhage in all three layers”

    • Sub retinal

    • Intra retinal

    • Pre-retinal

  • Aneurysmal area over vessel, usually white

• Work-up

  • Hypertension

  • Cerebrovascular syndrome

• Treatment

  • Argon

  • Yag

Name the condition

Macular holes

Top one=Still some intact macula tissue

2=Laminar hole

3=Operculum pulled off

4=Parting until bruch’s membrane

Describe the pic

• Macular hole

  • OCT cross section

  • Vitreous come off but the macula is pulling/still attached

  • Large cystic space underneath

  • Pt complaining of reduced central vision/blurriness

  • Older pt-May need to inc add/ varis

Describe the pic

• Macular hole

Macular Holes

• Symptoms

• Clinical signs

• Management

• Symptoms

  • Decreased vision

  • Central visual field defect

• Clinical signs

  • Early stage of macular hole – loss of foveolar depression and yellow spot in the centre of the macula

  • Late stage of macular hole- a round red lesion in the centre of the macula surrounded by a halo

• Management

  • Treatment not required in 50% (resolves spontaneously)

  • Vitrectomy

  • ILM peel

  • Intraocular gas tamponade, posture

  • Follow up every 6-12 months

Retinal detachments and holes

Basic retinal anatomy

• Individual retinal layers

• 2 blood circulations (retinal and choroidal circulation)

Helps to identify type of hole → link with appearance

Name the layers

Label the eye

Retinal detachment and holes

Vitreo-retinal anatomy

• 98 % Water

• Strongly adherent to retina at vitreous base, optic disc and along major vessels arcades

  • Danger if there’s a pull → bleeding into vitreous space/pre retinal space causing severe haemorrhaging

• Type II collagen t½ 40yr

• Type IX collagen t½ 11yr

→ clumping/reduction in vitreous size

Name the condition

• Retinal detachment and holes

• Vitro-macular traction Note: while this looks very serious, this is only “traction” and does not warrant referral.

• The patient is most likely not aware of it

Vitreous has come off on either side of the macula but it’s pulling the macula up, only classed as traction- wouldn’t refer at this point, not much effect on VA

What is the grading scale by Gass based on?

• Biomicroscopy findings + their clinical interpretations.

• Since the introduction of OCT, other authors have worked on improving clinical interpretation by using OCT images

Retinal detachment and holes

Classification scale

• Stage

• Biomicroscopy (Gass)

• Interpretation (Gass)

• OCT

• Stage 0 (MH):

  • Perifoveolar posterior hyaloid detachment with normal fovea or minor contour changes

• Stage 1A (Impending MH):

  • Central yellow spot, loss of foveolar depression, no vitreofoveal separation

  • Early serous foveolar detachment

  • OCT: perifoveolar hyaloid detachment + foveal cyst

• Stage 1B (Impending MH):

  • Yellow ring with bridging interface, loss of foveolar depression

  • Serous detachment or occult hole with bridging prefoveolar cortex

  • OCT: cyst extends into outer retina (“occult macular hole”)

• Stage 2 (Full-thickness MH):

  • Small eccentric/oval or crescent defect within yellow ring

  • Vitreous still attached (no full PVD)

  • May have pseudo-operculum

• Stage 3 (MH):

  • Central round defect ≥400 µm with elevated rim

  • No Weiss ring (no complete PVD)

  • Partial vitreous detachment

• Stage 4 (MH):

  • Full-thickness hole with complete posterior vitreous detachment

  • Weiss ring present

  • Posterior hyaloid no longer visible on OCT

• Key concepts:

  • Progression from foveal cyst → full-thickness hole

  • Increasing vitreous separation and traction changes across stages

Stages of a macula hole

Stage 1: Vitreous has come off in parts + little bit of pull

Stage 1A=Some tractional change + cystic spaces

More traction + tissue removed

No foveolar→ tissue has been pulled away (part of it) no stability in that region anymore

Posterior Vitreous Detachment

• Who is affected

• Symptoms

• Development and clinical signs

• Who is affected?

  • Typically people in their 50-60s

  • Can occur earlier in life especially in high myopes

• Symptoms?

  • Blurry vision, flashes and floaters

• Development and clinical signs?

  • Syneresis of vitreous

  • Pockets of syneretic fluid

  • Posterior hylaloid “breakthrough”

  • Weiss Ring

  • Haem/ Tobacco dust consider HST

• Note the Weiss Ring which is in focus, compared to the blurry (not focussed) ONH: this indicates that both are not at the same depth....i.e. the vitreous has detached from the retina

Name the condition

CSR

Central Serous Chorioretinopathy- CSCR/CSR

• Symptoms

• Clinical signs

• Investigations

• Management

• Symptoms

  • Decreased, blurred vision

  • Metamorphopsia

  • Central defect in the visual field

  • Colours appears washed-out

• Clinical signs

  • Usually unilateral sometimes bilat

  • Localised elevation of the retina in the macular region

  • Focal hyperpigmentation (sign of previous episodes)

• Investigations

  • Fluorescein angiography (“smoke- stack” , “ink-blot” leakage)

    • Neovasc

• Management

  • Usually not required (most cases [~80%] recover in ~6-9months)

  • Laser treatment may be considered

Name the condition

ERM

More visible with red free

Epiretinal Membrane- ERM Macular Pucker

• Symptoms

• Causes

• Clinical signs

• Symptoms

  • Largely asymptomatic

  • May present with decreased and distorted vision (glare)

    • Changes over time-memb is contractile-metamorphopisa at diff levels

  • Often bilat

• Causes

  • Idiopathic

  • Retinal break

  • Retinal detachment

  • Laser photocoagulation

  • Ocular surgery

  • Trauma

  • Intraocular inflammation

  • Diabetic retinopathy

  • Other retinal vascular diseases

• Clinical signs

  • Glistering membrane (cellophane maculopathy)

  • Thick grey membrane (macular pucker)

  • Retinal folds

• Management

  • Membrane peeling =more common in Germany than UK

    • risks=not done freq

Name this condition

Angioid streaks

Angioid streaks

Bilateral crack-like reddish bands usually radiating in a spoke-like pattern

• Causes

  • Pseudoxantoma elasticum

  • Paget disease

  • Sickle cell disease

  • Ehler-Danlos

  • Acromegaly

  • Marfan syndrome

  • Lead poisoning

  • Senile elastosis

• Complications

  • CNVM

  • Choroidal haemorrhage

  • Depending on the location of the streaks they can affect the macula

    • impact visual function signif

  • Foveal involvement by extended streak

• Management

  • Treat underlying condition

  • Follow up every 6 months

  • Treat CNV by laser photocoagulation

Some ocular conditions contain collagen, genetic conditions affects collagen in terms of elasticity and tensile strength

Name the condition

Retinitis Pigmentosa

Retinitis Pigmentosa

• Inheritance pattern

• Symptoms

• Clinical signs

• Investigations

• Inheritance pattern:

  • Autosomal dominant (common, the best prognosis)

  • Autosomal recessive (less common, intermediate prognosis)

  • X linked (least common, most severe)

    • Affects males more (XY), women can be carriers (2 X chromosomes)

• Symptoms

  • Night blindness and loss of peripheral vision

  • Late symptoms: poor central and colour vision

• Clinical signs

  • “bone spicule” retinal pigmentary changes starting from midperiphery and gradually extending anteriorly and posteriorly

  • Areas of depigmentation (RPE atrophy)

  • Narrowing of arterioles

  • Optic disc atrophy

• Investigations

  • VF testing

  • Electroretinography

  • Fundus photography

Retinitis Pigmentosa

• Management

• Exclude systemic associations:

  • Bassen-Kornzweig syndrome (deficiency in beta-lipoprotein)

  • Refsum disease (deficiency in the enzyme phytanic acid 2- hydroxylase)

  • Usher syndrome (combined deafness and RP)

  • Kearns- Sayre (mitochondrial cytopathy)-refer to cardiologist (danger of complete heart block)

  • Bardet-Biedl syndrome (mental handicap, polydactily, obesity, hypogenitalism, RP)

• No definitive treatment for RP: gene editing/ stem cell treatment ongoing trials with promising results (not all approved yet)

• Artificial retinal implants, show promise

  • Can send signals to retina to create some light perception→ can improve ability to read

Name the condition

Stargardt’s Disease Fundus Flavimaculatus

Stargardt’s Disease Fundus Flavimaculatus

• Symptoms

• Clinical findings

• Symptoms:

  • Bilateral decrease of vision in childhood, early adulthood

• Clinical findings:

  • Heavily pigmented RPE in the macula (early stage)

  • Yellow –white with fleck like deposits in the macular

  • Atrophic macular degeneration.

  • Dark FFA appearance

  • No treatment

    • Stem cell treatment, gene editing ??poss

Name the condition

Stickler’s syndrome

Pigmentary changes-tissue ,breaks,migration of RPE

Neovasc changes

What are the key features of Stickler’s syndrome?

• Autosomal dominant

• Collagenopathy

  • Type 2 and Type 11

• Characteristic faces

  • Flat faces/changes to ocular bones/present w/ hearing loss or joint problems/under developed bones in the middle of the face-cheek + nose

  • Implications when prescribing lenses/ bridge design

• Ophthalmology

  • Myopia

  • Paediatric cataract

  • Lattice - radial

  • RD

  • Glaucoma

Name the condition

Best’s Disease- Vitelliform maculopathy

Pigmentary changes-yellow/white-more where RPE/NFL loss -more light reflecting back from choroidal circulation

Best’s Disease- Vitelliform maculopathy

• Symptoms

• Clinical findings

• Complications

• Symptoms

  • May be asymptomatic or present with decreased vision

• Clinical findings

  • Yellow, round, subretinal lesion in the macula (“egg yolk”) or pseudo hypopyon

  • EOG

    • Assess level of visual function

• Complications

  • Macular CNV

  • Haemorrhage

  • Scarring

Name the condition

Familial dominant drusen

What are the key features of Familial dominant drusen?

• Autosomal dominant

• Multiple drusen

  • Scattered rather than concentrated in macula area

• Usually symptom free but may develop symptoms similar to AMD later on in life (i.e. may develop AMD)

• Younger pts (i.e. younger than your typical AMD patient)

  • 30’s or younger

• No treatment

  • Explain there could be a change in symptoms with aging

Name the condition

Drug induced retinopathies

Agents may bind to melanin in RPE

Could lead to a concentration of drugs + prolong their adverse effects

Duration of drug taken/dosage/

What are the main drug-induced retinopathies and their associated medications?

• Antimalarials (chloroquine) → bull’s eye maculopathy

• Phenothiazines (thioridazine, chlorpromazine)

• Toxic crystalline maculopathies (tamoxifen, canthaxanthin, methoxyflurane)

What is the optometrist’s role in managing patients with lifelong ocular conditions?

• Conditions have lifelong impact on vision and daily life

• Role includes managing stable conditions, providing support, low vision aids, and referrals as conditions progress

• Excellent communication is essential for information, empathy, and managing expectations

What are the key considerations when assessing and differentiating ocular conditions over time?

• Conditions can affect visual function throughout life (early, stable, or progressive)

• Increased risk of further abnormalities or earlier age-related changes (e.g. cataract)

• Similar presentations (e.g. AMD vs familial dominant drusen) → cannot rely on appearance alone; consider age, family history, and visual function