6.1.13 Recognises ocular manifestations of systemic disease.

Question 1

Hypertension

Answer 1

 Hypertension is a medical condition where the pressure inside the arteries is persistently elevated
 The initial response of retinal arterioles to systemic hypertension is vasoconstriction
 Prolonged HBP can lead to hardening of vessel walls, AV nipping and eventually increased vascular permeability

Question 2

Hypertensive retinopathy grading scale

Answer 2

 Grade 1 – arteriolar narrowing – 2:1 - GP
 Grade 2 – arteriolar narrowing / nipping – 1:3 - GP
 Grade 3 – narrowing, nipping, haemorrhages, exudates and CWS – HES
 Grade 4 – narrowing, haems, exudates, CWS, disc swelling, macular star - HES

Question 3

CRAO / BRAO

Answer 3

 Artery occlusion may occur from HBP / high cholesterol
 Emboli block the artery and causing lack of oxygen delivery
 Refer emergency if <24 hours; prognosis poor after 48hr window

Question 4

Central retinal artery occlusion (CRAO)

Answer 4

 Sudden painless monocular loss in vision
 VA < CF, complete loss – generally all areas of the VF (Unless a cilioretinal artery supplying a critical macular area preserves vision)
 RAPD
 White, oedematous retina; cherry ret spot at fovea; visible emboli/hollenhurst plaque?

Question 5

Branch retinal artery occlusion (BRAO)

Answer 5

 Sudden painless altitudinal or sectoral visual field loss; vision may be unaffected
 Signs confined to the region of the retina supplied by the affected branch
 VA is variable; RAPD often present
 Fundus signs may be subtle; whiteish, oedematous SECTOR of the retina (area of ischaemia)
 Altitudinal or sectoral VF defect – which may become permanent if left untreated

Question 6

CRVO / BRVO

Answer 6

 Hypertension – 2/3rds pxs
 Age, diabetes, smoking, glaucoma
 Caused by thrombus in the central retinal vein or branch of central retinal vein
 Urgent referral (CMGs) – monitor for glaucoma

BRVO
- VA variable
- Most commonly superior temporal
- Dilation/tortuosity, flame shaped & dot-blot haems

Ischaemic CRVO
- VA poor
- RAPD
- Dilation/tortuosity, flame shaped & dot-blot haems

Question 7

Explaining to a px CRVO / BRVO

Answer 7

• Systemic cause: High blood pressure (hypertension) is a condition where the pressure of the blood inside the arteries is higher than it should be, and so the heart has to work harder than normal to pump blood around the body
• Optical affect: The consequences of uncontrolled HBP can be severe i.e. loss of vision due to a blockage in the blood supply to the retina or optic nerve, loss of peripheral vision due to stroke or episodes of vision loss which come and go.
• Action plan: The condition can be improved by lifestyle changes such as improving diet, avoiding excess alcohol consumption, stopping smoking and increasing exercise, as well as medications such as beta-blockers, calcium channel blockers and vaso-dilators.

Question 8

Medications & ocular side effects

Answer 8

 Beta blocker - Propranolol = dry eye
 ACE inhibitors - Lisinopril & ramipril = may cause ciliary body oedema which leads to reduced accommodation and angle closure

Question 9

Hyperthyroidism

Answer 9

 Thyroid eye disease / Graves
 WET phase (myogenic) - EOMs (recti; IR affected first) swell up to 8-10x normal size (2-3 years)
 DRY phase (mechanical) - Fibrosis & secondary muscle contracture
 Connective tissue inflammation; redness, discomfort, peri-orbital swelling
 Corneal exposure; gritty/redness
 Diplopia and proptosis
 May cause visual loss due to ONH compression

Question 10

Marfans syndrome

Answer 10

 Autosomal dominant connective tissue disorder
 Tall, thin stature with long limbs
 Myopia, corneal abnormalities, ectopia lentis – dislocation of lens, retinal detachment

Question 11

Rheumatoid arthritis

Answer 11

 Dry eye disease / Sjogrens syndrome
 Iritis – 2nd episode; refer for systemic work up
 Episcleritis / scleritis – refer for systemic work up at 3rd episode

Question 12

Giant cell arteritis

Answer 12

 AAION (sudden profound unilateral vision loss, tender scalp, hardened non-pulsatile temporal artery, jaw claudication, malaise, VERY pale and swollen disc)

Question 13

MS

Answer 13

 Demyelinating disease affecting CNS
 Optic neuritis; mostly commonly retrobulbar

Question 14

Ocular manifestations of Hypertension

Answer 14

• Pathogenesis
  • Vasoconstrictive phase - autoregulation breaks down due to higher luminal pressure causing arteriolar narrowing; AV ratio affected
  • Sclerotic phase - persistent HBP leads to hyalinized walls & loss of muscle cells, causing arteriosclerosis. AV nipping & copper wiring result. Macro & micro aneurysms, CRA, CRV & ERM can form
  • Exudative phase - blood retinal barrier (barrier between retina & blood regulating movement of waste, protein, ions, water flux etc in & out of retina) disrupted due to loss of smooth muscles and endothelial cells. Haems, Oedema, CWS & exudates result. Macular star can result
• Signs & Grading
  • Grade 1 - Arteriolar narrowing
  • Grade 2 - Arteriolar narrowing, AV nipping, Copper wiring
  • Grade 3 - Grade 2 + Retinal haems (dot, blot, flame), Exudates (chronic oedema = macula star), CWSs, IRMA
  • Grade 4 - Grade 3 + ONH swelling i.e. malignant hypertension
  Classification is not closely related to severity of hypertension!!
• Vessel ChangesIn reality, just says there’s nipping

Question 15

Hypertensive choroidopathy

Answer 15

Severe hypertension - younger people

Associated with renal disease, pregnancy or collagen vascular diseases

Signs - Elschnig’s spots (RPE lesions), Siegrists streaks, Serous detachments

Question 16

Management of ocular hypertension

Answer 16

Mild
(Routine referral to GP if undiagnosed)
general* or arteriolar narrowing; arteriolar wall
opacification* ; arteriovenous nipping
Moderate
flame-shaped or blot-shaped; haemorrhages;
(Referral to GP / consider HES if diabetic, visual
microaneurysms; cotton-wool spots; hard
symptoms or vascular complications)
exudates
Severe / Malignant
some or all of the above
(Same day referral to HES / Emergency to A&E
& swelling of the optic disc
if BP >180/120)

Question 17

Uveitis

Classifications:

Answer 17

• Anterior uveitis – iris & anterior ciliary body. Anterior has ended up being iritis in many cases
• Intermediate uveitis - posterior ciliary body & peripheral retina
• Posterior uveitis – uveal tract behind the posterior border of the vitreous base
• Panuveitis – entire uveal tract

Question 18

Causes of uveitis:

Answer 18

1. Infections (Bacterial, parasitic, fungal and viral) like herpes zoster
2. Trauma – IOFB, perforating injury
3. Systemic diseases - syphilis, TB, sarcoidosis, toxocariasis, toxoplasmosis. Majority of people have ocular first, THEN systemic condition may be found after you refer them
4. Idiopathic

Question 19

Anterior: uveitis

Answer 19

• Symptoms - rapid onset of unilateral pain, blurred vision (depends on severity), photophobia, redness & watery discharge
• Signs - Ciliary Injection, Miosis, Cells & flare, KPs, Hypopyon, Iris nodules (Busacca - iris stroma, Koeppe - pupillary margin), PS causing pigment on the lens, Lowered IOP (function of ciliary body not as good) (can sometimes be raised if TM blocked by cells & flare)
• Management - Emergency, same day referral. Make sure to look at fundus to exclude posterior uveitis & if clear view not obtained, must make HES aware

Question 20

Intermediate: Uveitis

Answer 20

• Onset - Idiopathic or systemic linked. Age 15-40 so young! Bilateral but assymetric
• Symptoms - painless floaters, blurred vision, anterior uveitis sxs are rare
• Signs - vitreous cells more anteriorly (vitritis), vitreous haze, snowballs (inflammatory cells & exudate), snowbanking (inferior & after gravity makes snowballs go downward), periphlebitis (perivascular inflammation)

Question 21

Posterior

Answer 21

• Onset
  
  • Sarcoidosis, Tuberculosis, Toxoplasmosis, Toxocariasis, Acquired syphilis, Presumed Ocular Histoplasmosis syndrome (as examples)
• Symptoms - Painless Floaters, Blurred vision, Scotomas (patchy), Distortion (if macula affected), Photopsia
• Signs - cells & flare, possible PVD, choroidal & vasculature changes
  
  • Retinitis - whitish retinal opacities
  • Choroiditis - round, yellow nodule
  • Vasculitis - periarteritis (artery) or periphlebitis (vein). Grey-white/yellowish perivascular patchy cuffing (inflammation of the outer coat of a vein/artery or of tissues around a vein/artery.) Candle-wax drippings
• Management - same as anterior??

Question 22

Conditions associated with Uveitis:

Answer 22

Bacterial Uveitis = Tuberculosis, Acquired syphilis, Lyme disease, Cat scratch disease

Parasitic Uveitis = Toxoplasmosis, Toxocariasis

Fungal Uveitis = Presumed ocular histoplasmosis syndrome (POHS)

Viral Uveitis = Cytomegalovirus, Acute retinal necrosis, Herpes simplex anterior uveitis, Herpes zoster anterior uveitis

• Psoriatic arthritis
  
  • Psoriasis leading to arthritis
• Fuch’s uveitis syndrome
  
  • 4th decade, cause uncertain, non-granulomatous
  • Kanski page 438
  • Signs (mild condition) - AC shows mild cells & flare, Heterochromia, Small KPs, Iris nodules & atrophy (retroillumination), PSO, Vitritis, Glaucoma
    
    • Fundus not normally affected
• Juvenile Idiopathic Arthritis (JIA)
• Behcets disease
• Ulcerative colitis
• Crohn disease
• Sarcoidosis

Question 23

Sarcoidosis

Answer 23

• Growth of tiny collections of inflammatory cells (granulomas) in any part of your body
• Results in all types of uveitis
• Common signs:
  
  • Mutton fat KPs, Small KPs
  • Iris nodules
  • PAS
  • Vitritis, snowballs
  • Chorioretinal lesions
  • Periphlebitis
  • Optic disc nodules/granulomas - granulomas can be on retina (creamy patches - check elevation on OCT) & even outside the eye on adnexa

Question 24

Ankylosing spondylitis

Answer 24

• Ankylosing spondylitis
  
  • 3rd-4th decade, limitation of spinal movements
  • AAU, Scleritis, Episcleritis, Keratitis, Mechanical ptosis

Question 25

The manifestations of uveitis

Answer 25

- Reduced Visual acuity - Intraocular pressure may be increased or reduced - Lid Oedema - Ciliary injection - Chemosis - Keratic Precipitates(KPs)- deposits on the corneal endothelium composed of inflammatory cells such as lymphocytes, plasma cells, and macrophages. - Anterior chamber: - Cells in the anterior chamber - Hypopyon–whitish purulent exudate composed of myriad inflammatory cells in the inferior part of the anterior chamber forming a horizontal level under the influence of gravity. - Aqueous flare: haziness of the normally clear fluid in the anterior chamber due to protein in the aqueous present as a result of the breakdown of the blood-aqueous barrier. - Fibrinous exudates - Iris: - Iris nodules- including Koeppe nodules which are the site of posterior synechiae formation, Bussaca nodules which are a feature of granulomatous uveitis, and yellowish nodules seen in syphilitic uveitis. - Iris pearls- seen in lepromatous uveitis - Iris crystals - Posterior synechiae - Iris atrophy- seen in herpetic uveitis - Heterochromia iridis - Iris neovascularization - Cataract - Secondary glaucoma - Vitritis - Vitreous hemorrhage - White snowball like exudates near the ora serrata - Papillitis or Disc edema - Mild peripheral phlebitis - Macular edema - Exudates in the choroid and retina - Retinal hemorrhages - Choroidal Neovascularization - Retinal detachment

Question 26

Thyroid

Answer 26

- Description - the thyroid produces T3 & T4 which are hormones that regulate… - Bilateral but assymetrical condition - TED can occur in hyper or hypo active thyroid conditions

Question 27

# Thyroid Signs: (wet phase - myogenic)

Answer 27

- It starts with Mild Lid signs like… - Lid lag - person looks down but their upper lid is retracted - Lid retraction - person has a startled looking appearance due to proptosis - Scleral show - in primary position, upper limbus should not show but if it does then bad news - Soft tissue involvement… - Periorbital oedema, Swollen & red lids, Gritiness, Dryness, Bulbar hyperaemia - Later… - Corneal exposure - reduction in vision possibly - More severe oedema & inflammation of the orbit & the muscles - pain on eye movement - Enlarged recti muscles & increased orbital contents cause more proptosis - px struggles to close their eyes! - This can start to stretch the ON/compress it causing more bad news i.e. possible optic neuropathy!!

Question 28

# Thyroid Dry phase - mechanical

Answer 28

- Inflammation subsides, muscles become fibrotic - Normally IR affected most commonly. Next most common is MR, then… - E.g. if IR affected then it is essentially more stiff & hardened, causing it to stay down but not letting it more up to elevate the eye as he fibrosis is on top of the muscle - This might cause diplopia and possibily a unilateral limitation in eye movement

Question 29

Stroke

Answer 29

- This is when blood supply to the brain is stopped - Manifestations - Visual pathway defect like a hemianopia, Visual neglect, Eye movement problems - Eye movement - nystagmus, gaze palsy, trouble doing saccades or pursuit. May be diplopia - Visual neglect - unaware of objects to one side - Management - fresnel prism until stable, occlusion, scanning techniques, vertical text, typoscope Lecture on Stroke & its ocular consequences, worth reading as quite short:

Question 30

Sjogren’s syndrome

Answer 30

- Description - a chronic inflammatory disorder (autoimmune - mistakenly attacking the body) causing lymphocitic infiltration of the lacrimal & salivary glands, causing their dysfunction & hence dry eye, dry mouth etc - Severe aqueous tear deficiency - Signs & Sxs - Burning, Stinging, Gritiness, FB sensation, Itchiness, Soreness, Redness, Photophobia, Watering - Reduced TBUT, Reduced schirmer (<5mm), Reduced phenol red (<10mm) - NaFl staining, reduced tear volume (<0.18mm) - Rarely anterior uveitis, episcleritis - Management - Refer to college guidelines on dry eye