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WCS32 Ocular Manifestations Of Systemic Disease Flashcards

1
Q

Systems related to eyes

A
  1. Endocrinology
  2. Rheumatology
  3. CVS disease
  4. Skin and Paediatric conditions
  5. Drug-related
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2
Q

Diabetes Mellitus and Eyes

A
  1. Diabetic retinopathy
    - **Non-proliferative (NPDR) (Earlier stage)
    - **    Proliferative (PDR) (Later stage)
    —> may NOT affect central vision
  2. Diabetic maculopathy
    —> affect central vision
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3
Q

Symptoms of Diabetic retinopathy

A

記: MCV, HDI

  1. ***Microaneurysm
    - Hallmark of diabetic retinopathy
    - Earliest sign
  2. Retinal haemorrhage (***Dot-and-blot, flame-shaped)
    - usually visually insignificant
  3. ***Hard exudates
    - accumulation of lipoproteins leaked from vessels
  4. ***Cotton wool spots
    - focal areas of infarction of retinal nerve fibre
  5. ***Venous beading
    - increasing ischaemia
  6. Intra-retinal microvascular abnormalities (IRMA)
    - anastomosis between arterioles and venules
    - vs Neurovascularisation (    network-like) (IRMA: much smaller)
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4
Q

***Pathophysiology of Diabetic retinopathy

A

Retinal microangiopathy (e.g. Microaneurysm, Retinal haemorrhage) —>

  1. ***Breakdown of Blood-Retinal Barrier —> Macula edema
    - Hard exudates
  2. ***Retinal ischaemia —> Vasoproliferative substances (e.g. VEGF) (Neovascularistion) —> Macula edema
    - Cotton wool spots
    - Venous beading
    - IRMA
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5
Q

***Non-proliferative Diabetic Retinopathy (NPDR)

A

Mild:
- ***>= 1 Microaneurysm

Moderate:

  • ***Multiple microaneurysm
  • Dot-and-blot haemorrhages
  • Venous beading
  • +/- Cotton wool spots

Severe (***4-2-1 rule):

  • Diffuse haemorrhage + Microaneurysms in ***4 Quadrants
  • Venous beading in 2Q
  • IRMA in >=1Q
  • > 50% progress to PDR
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6
Q

***Proliferative Diabetic Retinopathy (PDR)

A
  1. ***Retinal neovascularisation
  2. ***Vitreous haemorrhage
  3. Pre-retinal fibrosis (***regression of neovascularisation)
  4. Rubeosis iridis (NVI)
    - impending Neovascular glaucoma
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7
Q

***Diabetic maculopathy

A

Symptoms

  1. Multiple exudate / cystic space
  2. Dull macula
  3. Macular edema —> Loss of depression at fovea
  4. Loss of central vision

Pathophysiology:
1. Breakdown of Blood-retinal barrier
2. Vasoproliferative substances (VEGF)
Both —> Macula edema

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8
Q

Complications of Diabetic retinopathy / maculopathy

A
  1. ***Cataract (usually cortical)
  2. ***Neovascular glaucoma (newly formed blood vessels —> fibrosis —> closes the angle)
  3. ***Tractional retinal detachment
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9
Q

Treatment of Diabetic Retinopathy / Maculopathy

A

Mild / Moderate NPDR
- ***Glycaemic control (diet, medication)

Severe NPDR / PDR
- ***Panretinal laser photocoagulation (PRP)
—> killing of retinal tissue to prevent progression + preserve macula
—> impair peripheral vision + night vision

Clinically significant macula edema (CSME)

  • Intravitreal ***anti-VEGF (Ranibizumab / Aflibercept)
  • Intravitreal ***steroid (Triamcinolone / Ozurdex)
  • ***Focal / grid laser if no macula ischaemia —> obliterate leaking microaneurysm + stimulate retinal pigmented epithelium to absorb more water in cystic space
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10
Q

Thyroid eye disease (TED) / Graves’ ophthalmopathy / Orbitopathy (GO)

A
  • Typically associated with Graves’ hyperthyroidism
  • Also occur with hypothyroidism / euthyroid individuals
  • Immune-mediated

Orbital signs:

  1. ***Exophthalmos / Proptosis
  2. ***Limited EOM / Squint

Eyelid / Ocular signs:

  1. Lid retraction
  2. Lid lag
  3. Lagophthalmos (incomplete closure of the eyelids)
  4. Lid puffiness / Erythema
  5. Conjunctival injection / Caruncle inflammation / Chemosis (conjunctival swelling)
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11
Q

Ocular complications in TED

A
  1. ***Compressive optic neuropathy
  2. ***Exposure keratopathy —> cannot close eyes
  3. Increased IOP / Glaucoma
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12
Q

CT Orbit in TED

A
  1. ***Tendon sparing muscle enlargement (Pathognomonic for TED)
  2. Look for ***apical crowding (Compression of the optic nerve at the apex of the orbit by enlarged extraocular muscles —> looking for complication)
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13
Q

Phases in TED

A
  1. Active phase:
    - Inflammatory (6-18 months)
  2. Quiescence phase:
    - Post-inflammatory
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14
Q

***Activity and Severity of TED

A 
Activity:
Clinical Activity Score (CAS)
Active: CAS >=3
1. Spontaneous retrobulbar pain
2. Pain on eye movements
3. Eyelid erythema
4. Conjunctival injection
5. Chemosis
6. Swelling of the caruncle
7. Eyelid edema / fullness

Severity (**Exophthalmos, **Lid retraction):

  1. Mild
    - minor lid retraction (<3mm)
    - transient / no diplopia
    - corneal exposure responsive to lubricants
  2. Moderate to Severe
    - lid retraction (>=2mm)
    - moderate / severe soft tissue involvement
    - exophthalmos >=3mm above normal for race + gender
    - inconstant / constant diplopia
  3. Sight threatening
    - Dysthyroid optic neuropathy (DON)
    - Corneal breakdown (Exposure keratopathy)
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15
Q

Management of TED

A

Mild:

  • ***Quit smoking
  • Normalise thyroid function
  • Symptomatic treatment (e.g. lubricants for dry eyes)

Moderate to Severe:

  • Active —> Immunosuppression with ***Steroid (weekly IV infusion Methylprednisolone 12 weeks)
  • Inactive —> Surgeries (***Orbital —> Squint —> Lid)

Sight-threatening:
- Immediate intervention needed
—> DON: IV steroid + **Orbital decompression (thinning of **medial wall + **floor (X Lateral wall + Superior roof))
—> **Exposure keratopathy: Meds + Tape +/- Decompression

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16
Q

Teprotumumab

A

Anti-IGF1 Ab

  • Insulin-like growth factor 1 receptor
  • Rapidly effective in decreasing disease activity
17
Q

Rheumatology and Eyes

A

Collagen + Vessels

Autoimmune diseases with ophthalmic manifestations

  1. Seronegative spondyloarthropathies (***HLA-B27, ANA and RF -ve)
    - Ankylosing spondylitis
    - IBD-associated
  2. RA
  3. SLE
  4. Sjögren’s syndrome
  5. Sarcoidosis
18
Q

Ocular manifestations of Rheumatology disease

A
  1. ***Uveitis
  2. ***Peripheral Ulcerative Keratitis (PUK)
  3. ***Keratoconjunctivitis sicca (Sjögren’s)
  4. ***Episcleritis / Scleritis
  5. Retinal vein occlusion
  6. ***Ischaemic optic neuropathy
19
Q

Anterior uveitis vs Panuveitis

A

Anterior uveitis:
- Mostly commonly isolated + idiopathic
- Commonest association is **Seronegative spondyloarthropathies
—> esp. with **HLA-B27 allele
—> Ankylosing spondylitis, Psoriatic arthritis, IBD associated, JIA
- Others: RA, SLE, Sarcoidosis

Panuveitis:

  • Anterior uveitis + ***Posterior segment inflammation (Chorioretinitis, Vitritis, Retinal vasculitis)
  • Much less common
  • Usually associated with ***Behcet disease
20
Q

Signs of Anterior Uveitis

A
  1. Ciliary flush
  2. Keratic precipitates (KP) —> inflammatory cellular deposit seen on corneal endothelium
  3. Anterior chamber cells
  4. Posterior synechiae (PS) —> Iris stick on the lens
  5. Hypopyon —> Exudate (death inflammatory cells) in lower part of eyes
21
Q

Uveitis treatment

A

Anterior uveitis:

  1. ***Topical steroid
    - intensive initially then taper gradually
  2. ***Topical cycloplegics (e.g. homatropine, atropine)
    - relieve pain due to ciliary spasm
    - prevent posterior synechiae
  3. If steroid resistant / dependent
    - AC tapping to rule out infection (HSV, VZV, CMV, TB)
    - Subtenon steroid for unilateral disease (Triamcinolone)

Panuveitis / Posterior uveitis
1. Treat underlying condition

  1. ***Systemic steroid +/- immunomodulator
  2. ***Intravitreal steroid (Triamcinolone, Ozurdex)
    - more for unilateral cases
  3. Biologics: ***TNF inhibitors (Infliximab, Adalimumab)
22
Q

Peripheral Ulcerative Keratitis (PUK)

A

Signs

  1. Crescent shape ulcer
  2. Active ulcer on peripheral scar
  3. Corneal thinning
  4. Perforation
  • Mainly associated with ***RA
    —> others: collagen vascular disease (SLE), IBD
  • Mooren’s ulcer if isolated

Treatment:

  1. Systemic (Inflammation control)
    - Systemic steroid (Prednisolone +/- IV Methylprednisolone)
    - Immunomodulators (MTX, Azathioprine, Cyclosporin A, Cyclophosphamide)
    - Biologics (Infliximab, Rituximab)
  2. Local (Damage control)
    - Preservative free lubricants
    - Topical antibiotics
    - Conjunctival recession
  3. Prevent perforation
    - protect with eye shield
    - corneal melting —> avoid topical steroid
  4. Small / impending perforation
    - Cyanoacrylate glue + Bandage contact lens (BCL)
  5. Larger perforation
    - Corneal patch graft
23
Q

CVS disease and Eyes

A

Hypertensive retinopathy
Grade 1: Barely detectable arterial narrowing
Grade 2: Obvious **arterial narrowing with focal irregularities
Grade 3: Grade 2 + **Retinal haemorrhage + Exudates + CWS + Retinal edema
Grade 4: Grade 3 + ***Papilloedema

24
Q

Skin + Paediatric conditions and Eyes

A

Herpes Zoster Ophthalmicus

  • reactivation of latent VZV in Trigeminal ganglia (V1, V2)
  • ***Hutchinson’s sign (Nasociliary nerve affected —> more likely to have Intraocular inflammation)

Ocular involvements:

  • Conjunctivitis
  • ***Keratitis (Microdendritic lesions) (Simplex virus: Large dendritic lesions)
  • ***Anterior uveitis
  • Episcleritis
  • Scleritis
  • Trabeculitis (↑ IOP)
  • Retinitis
  • Retinal vasculitis
  • Choroiditis
  • Optic neuritis
25
Q

Management and Sequelae of Herpes Zoster Ophthalmicus

A

Management:

  1. Airborne precaution until lesions crusted
  2. Oral ***Aciclovir 800mg 5x/day for 7-10 days
  3. +/- Ointment Aciclovir 5x/day
  4. Treatment for ocular complications

Potential sequalae:

  • Post-herpetic neuralgia
  • Skin scarring
  • Sequalae of ocular complications
26
Q

Marfan syndrome

A
  • Autosomal dominant trait
  • Defective ***FBN1 gene at chromosome 15
    —> Encoding fibrillin-1
  • **Ectopia lentis (Lens subluxation / dislocation)
  • Idiopathic
  • Ocular causes: trauma, pseudoexfoliation syndrome
  • Systemic causes: Marfan syndrome (most common, superotemporal subluxation), Homocystinuria (2nd most common, inferonasal subluxation)
27
Q

Neurocutaneous Syndrome (aka Phakomatoses)

A
  1. Sturge-Weber syndrome

2. Neurofibromatosis Type 1

28
Q
  1. Sturge-Weber syndrome
A

Vascular malformation involving Leptomeninges + Facial skin

Neuro: ***Leptomeningeal angioma

  • Developmental delay / Intellectual disability
  • Seizures / Epilepsy

Cutaneous: ***Port-wine stain

  • aka Nevus flammeus
  • Typically V1, V2 distribution

Ophthalmic problems:

  • Glaucoma
  • Diffuse choroidal hemangioma
29
Q
  1. Neurofibromatosis Type 1
A
  • aka ***Von Recklinghausen’s Disease
  • AD inheritance with high penetrance
  • Mutation / Deletion of ***NF1 gene at chromosome 7q
    —> encode neurofibromin

Diagnostic criteria:

  1. ***Plexiform neurofibroma
  2. ***Optic glioma
  3. > =1 Lisch nodules
  4. ***Sphenoid dysplasia (pulsatile proptosis)
30
Q

Drug-related ophthalmic conditions

A
  1. Bull’s eye maculopathy
    - ***Chloroquine&raquo_space; Hydroxychloroquine
    - Risk correlates to daily dose, duration of use and total accumulative dose
    - Other risk factors: Age, liver, renal impairment, obesity
  2. Vortex keratopathy
    - aka Cornea verticillata
    - Whorl-like pattern of golden brown / gray deposits in the inferior
    - ***Amiodarone
    - Other drugs: Chloroquine / Hydroxychloroquine, Indomethacin, Phenothiazine
  3. Eyelid hypertrichosis and Trichomegaly
    - Systemic medication: Eriotinib —> Prominent Trichomegaly with curling of lashes
    - Topical medication: Prostaglandin analogs (Bimatoprost, Latanoprost) —> Hypertrichosis > Trichomegaly, much less lashes curling
    - Hirsutism: Cyclosporine, Tacrolimus, Phenytoin, Interferon-α2b
31
Q

***Systemic steroid + Topical steroid

A

Systemic steroid:

  1. Central Serous Chorioretinopathy (CSC)
  2. Cataract (Posterior subcapsular)

Topical steroid:

  1. Partial ptosis
    - mild ptosis
    - Muller muscle atrophy
  2. Increased IOP / Glaucoma
    - also in systemic steroid use
  3. Cataract
    - Posterior subcapsular type
    - fast visual deterioration
32
Q

***Important points

A
  1. DM: Retinopathy vs Maculopathy
  2. Thyroid eye disease: Activity vs Severity
  3. Herpes Zoster Ophthalmicus
  4. Uveitis and associated autoimmune disease
  5. Steroid side effects

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