12.

KASPER NOTES 2020

OPHTHALMOLOGY
02.Acute angle closure glaucoma
03.Age related macular degeneration
06.Angioid retinal streaks
07.Anterior uveitis
08.Argyll-Robertson pupil
09.Blepharitis
10.Cataracts
13.Central retinal artery occlusion
14.Central retinal vein occlusion
15.Diabetic retinopathy
17.Herpes simplex keratitis
18.Herpes zoster ophthalmicus
19.Holmes-Adie pupil
20.Horner's syndrome
21.Hypertensive retinopathy
22.Keratitis
24.Lacrimal duct problems
25.Mydriasis
26.Optic atrophy
27.Optic neuritis
28.Orbital cellulitis
30.Key points

1
Acute angle closure glaucoma

Glaucoma is a group of disorders characterised by optic neuropathy due, in

the majority of patients, to raised intraocular pressure (IOP). It is now
recognised that a minority of patients with raised IOP do not have glaucoma
and vice versa.

In acute angle-closure glaucoma (AACG) there is a rise in IOP secondary to

an impairment of aqueous outflow. Factors predisposing to AACG include:

 hypermetropia (long-sightedness)
 pupillary dilatation
 lens growth associated with age

Features

 severe pain: may be ocular or headache

 decreased visual acuity
 symptoms worse with mydriasis (e.g. watching TV in a dark room)
 hard, red-eye
 haloes around lights
 semi-dilated non-reacting pupil
 corneal oedema results in dull or hazy cornea
 systemic upset may be seen, such as nausea and vomiting and even
abdominal pain

Management

 urgent referral to an ophthalmologist

 management options include reducing aqueous secretions with
acetazolamide and inducing pupillary constriction with topical
pilocarpine

2
Age related macular degeneration

Age-related macular degeneration is the most common cause of blindness in

the UK. Degeneration of the central retina (macula) is the key feature with
changes usually bilateral. ARMD is characterised by degeneration of retinal
photoreceptors that results in the formation of drusen which can be seen on
fundoscopy and retinal photography.

Traditionally two forms of macular degeneration are seen:

 dry (90% of cases, geographic atrophy) macular degeneration:

characterised by drusen - yellow round spots in Bruch's membrane
 wet (10% of cases, exudative, neovascular) macular degeneration:
characterised by choroidal neovascularisation. Leakage of serous fluid
and blood can subsequently result in a rapid loss of vision. Carries
worst prognosis

Recently there has been a move to a more updated classification:

 early age-related macular degeneration (non-exudative, age-related

maculopathy): drusen and alterations to the retinal pigment epithelium
(RPE)
 late age-related macular degeneration (neovascularisation, exudative)

Age-related macular degeneration (ARMD) is the commonest cause of

visual loss in elderly persons in the developed world. It affects 30-50 million
people worldwide.

Epidemiology

 population estimates suggest a male to female ratio of 1:2

 the average age of presentation is greater than 70 years of age

3
Risk factors

 Advancing age itself is the greatest risk factor for ARMD. The risk of
ARMD increases 3 fold for patients aged older than 75 years, versus
those aged 65-74.
 Smoking is another key risk factor in the development of ARMD,
current smokers are twice as likely as non-smokers to have ARMD
related visual loss, and ex-smokers have a slightly increased risk of
developing the condition, (OR 1.13).
 Family history is also a strong risk factor for developing ARMD. First
degree relatives of a sufferer of ARMD are thought to be four times
more likely to inherit the condition.
 Other risk factors for developing the condition include those
associated with increased risk of ischaemic cardiovascular disease,
such as hypertension, dyslipidaemia and diabetes mellitus.

Patients typically present with a subacute onset of visual loss with:

 a reduction in visual acuity, particularly for near field objects

 difficulties in dark adaptation with an overall deterioration in vision at
night
 fluctuations in visual disturbance which may vary significantly from
day to day
 they may also suffer from photopsia, (a perception of flickering or
flashing lights), and glare around objects

Signs:

 distortion of line perception may be noted on Amsler grid testing

 fundoscopy reveals the presence of drusen, yellow areas of pigment
deposition in the macular area, which may become confluent in late
disease to form a macular scar.
 in wet ARMD well demarcated red patches may be seen which
represent intra-retinal or sub-retinal fluid leakage or haemorrhage.

4
Investigations:

 slit-lamp microscopy is the initial investigation of choice, to identify

any pigmentary, exudative or haemorrhagic changes affecting the
retina which may identify the presence of ARMD. This is usually
accompanied by colour fundus photography to provide a baseline
against which changes can be identified over time.
 fluorescein angiography is utilised if neovascular ARMD is suspected,
as this can guide intervention with anti-VEGF therapy. This may be
complemented with indocyanine green angiography to visualise any
changes in the choroidal circulation.
 ocular coherence tomography is used to visualise the retina in three
dimensions, because it can reveal areas of disease which aren't visible
using microscopy alone.

Treatment:

 the AREDS trial examined the treatment of dry ARMD in 3640

subjects. It showed that a combination of zinc with anti-oxidant
vitamins A,C and E reduced progression of the disease by around one
third. Patients with more extensive drusen seemed to benefit most
from the intervention. Treatment is therefore recommended in patients
with at least moderate category dry ARMD.
 Vascular endothelial growth factor, (VEGF) is a potent mitogen and
drives increased vascular permeability in patients with wet ARMD. A
number of trials have shown that use of anti-VEGF agents can limit
progression of wet ARMD and stabilise or reverse visual loss.
Evidence suggests that they should be instituted within the first two
months of diagnosis of wet ARMD if possible. Examples of anti-
VEGF agents include ranibizumab, bevacizumab and pegaptanib,. The
agents are usually administered by 4 weekly injection.
 Laser photocoagulation does slow progression of ARMD where there
is new vessel formation, although there is a risk of acute visual loss
after treatment, which may be increased in patients with sub-foveal
ARMD. For this reason anti-VEGF therapies are usually preferred.

5
Angioid retinal streaks

Angioid retinal streaks are seen on fundoscopy as irregular dark red streaks
radiating from the optic nerve head. They are caused by degeneration,
calcification and breaks in Bruch's membrane .

Causes

 pseudoxanthoma elasticum
 Ehler-Danlos syndrome
 Paget's disease
 sickle-cell anaemia
 acromegaly

6
Anterior uveitis

Anterior uveitis is one of the important differentials of a red eye. It is also

referred to as iritis. Anterior uveitis describes inflammation of the anterior
portion of the uvea - iris and ciliary body. It is associated with HLA-B27 and
may be seen in association with other HLA-B27 linked conditions (see
below).

Features

 acute onset
 ocular discomfort & pain (may increase with use)
 pupil may be irregular and small
 photophobia (often intense)
 blurred vision
 red eye
 lacrimation
 ciliary flush
 hypopyon; describes pus and inflammatory cells in the anterior
chamber, often resulting in a visible fluid level
 visual acuity initially normal → impaired

Associated conditions

 ankylosing spondylitis
 reactive arthritis
 ulcerative colitis, Crohn's disease
 Behcet's disease
 sarcoidosis: bilateral disease may be seen

Management

 urgent review by ophthalmology

 cycloplegics (dilates the pupil which helps to relieve pain and
photophobia) e.g. Atropine, cyclopentolate
 steroid eye drops

7
Argyll-Robertson pupil

Argyll-Robertson pupil is one of the classic pupillary syndrome. It is

sometimes seen in neurosyphilis. A mnemonic used for the Argyll-
Robertson Pupil (ARP) is Accommodation Reflex Present (ARP) but
Pupillary Reflex Absent (PRA)

Features

 small, irregular pupils

 no response to light but there is a response to accommodate

Causes

 diabetes mellitus
 syphilis

8
Blepharitis

Blepharitis is inflammation of the eyelid margins. It may due to either

meibomian gland dysfunction (common, posterior blepharitis) or
seborrhoeic dermatitis/staphylococcal infection (less common, anterior
blepharitis). Blepharitis is also more common in patients with rosacea

The meibomian glands secrete oil on to the eye surface to prevent rapid
evaporation of the tear film. Any problem affecting the meibomian glands
(as in blepharitis) can hence cause drying of the eyes which in turns leads to
irritation

Features

 symptoms are usually bilateral

 grittiness and discomfort, particularly around the eyelid margins
 eyes may be sticky in the morning
 eyelid margins may be red. Swollen eyelids may be seen in
staphylococcal blepharitis
 styes and chalazions are more common in patients with blepharitis
 secondary conjunctivitis may occur

Management

 softening of the lid margin using hot compresses twice a day

 'lid hygiene' - mechanical removal of the debris from lid margins
o cotton wool buds dipped in a mixture of cooled boiled water
and baby shampoo is often used
o an alternative is sodium bicarbonate, a teaspoonful in a cup of
cooled water that has recently been boiled
 artificial tears may be given for symptom relief in people with dry
eyes or an abnormal tear film

9
Cataracts

A cataract is a common eye condition where the lens of the eye gradually
opacifies i.e. becomes cloudy. This cloudiness makes it more difficult for
light to reach the back of the eye (retina), thus causing reduced/blurred
vision. Cataracts are the leading cause of curable blindness worldwide.

Epidemiology

 Cataracts are more common in women than in men

 The incidence of cataracts increases with age. One study found that
30% of individuals aged 65 and over had a visually-impairing cataract
in either one or both eyes

Causes

 Normal ageing process: most common cause

Other possible causes

 Smoking
 Increased alcohol consumption
 Trauma
 Diabetes mellitus
 Long-term corticosteroids
 Radiation exposure
 Myotonic dystrophy
 Metabolic disorders: hypocalcaemia

Patients typically present with a gradual onset of:

 Reduced vision
 Faded colour vision: making it more difficult to distinguish different
colours
 Glare: lights appear brighter than usual
 Halos around lights

10
Signs:

 A Defect in the red reflex: the red reflex is essentially the reddish-
orange reflection seen through an ophthalmoscope when a light is
shone on the retina. Cataracts will prevent light from getting to the
retina, hence you see a defect in the red reflex.

Investigations:

 Ophthalmoscopy: done after pupil dilation. Findings: normal fundus

and optic nerve
 Slit-lamp examination. Findings: visible cataract

Classification

 Nuclear: change lens refractive index, common in old age

 Polar: localized, commonly inherited, lie in the visual axis
 Subcapsular: due to steroid use, just deep to the lens capsule, in the
visual axis
 Dot opacities: common in normal lenses, also seen in diabetes and
myotonic dystrophy

11
Management

 Non-surgical: In the early stages, age-related cataracts can be

managed conservatively by prescribing stronger glasses/contact lens,
or by encouraging the use of brighter lighting. These options help
optimise vision but do not actually slow down the progression of
cataracts, therefore surgery will eventually be needed.
 Surgery: Surgery is the only effective treatment for cataracts. This
involves removing the cloudy lens and replacing this with an artificial
one. NICE suggests that referral for surgery should be dependent upon
whether a visual impairment is present, impact on quality of life, and
patient choice. Also whether both eyes are affected and the possible
risks and benefits of surgery should be taken into account. Prior to
cataract surgery, patients should be provided with information on the
refractive implications of various types of intraocular lenses. After
cataract surgery, patients should be advised on the use of eye drops
and eyewear, what to do if vision changes and the management of
other ocular problems. Cataract surgery has a high success rate with
85-90% of patients achieving 6/12 corrected vision (on a Snellen
chart) postoperatively.

Complications following surgery

 Posterior capsule opacification: thickening of the lens capsule

 Retinal detachment
 Posterior capsule rupture
 Endophthalmitis: inflammation of aqueous and/or vitreous humour

A hypermature age-related cortico-nuclear cataract with a brunescent (brown) nucleus. Credit National Eye Institute, National Institutes of Health.

12
Central retinal artery occlusion

Central retinal artery occlusion

 causes sudden unilateral visual loss

 due to thromboembolism (from atherosclerosis) or arteritis (e.g.
temporal arteritis)
 features include afferent pupillary defect, 'cherry red' spot on a pale
retina

13
Central retinal vein occlusion

Central retinal vein occlusion (CRVO) may be due to thromboembolism

(from atherosclerosis) or arteritis (e.g. temporal arteritis).

Features

 sudden painless loss of vision

 severe retinal haemorrhages
 'cherry red' spot on a pale retina
 afferent pupillary defect

14
Diabetic retinopathy

Diabetic retinopathy is the most common cause of blindness in adults aged 35-65 years-
old. Hyperglycaemia is thought to cause increased retinal blood flow and abnormal
metabolism in the retinal vessel walls. This precipitates damage to endothelial cells and
pericytes

Endothelial dysfunction leads to increased vascular permeability which causes the

characteristic exudates seen on fundoscopy. Pericyte dysfunction predisposes to the
formation of microaneurysms. Neovasculization is thought to be caused by the
production of growth factors in response to retinal ischaemia

In exams you are most likely to be asked about the characteristic features of the various
stages/types of diabetic retinopathy. Recently a new classification system has been
proposed, dividing patients into those with non-proliferative diabetic retinopathy
(NPDR) and those with proliferative retinopathy (PDR):

15
Proliferative retinopathy

 retinal neovascularisation - may lead to vitrous haemorrhage

 fibrous tissue forming anterior to retinal disc
 more common in Type I DM, 50% blind in 5 years

Maculopathy

 based on location rather than severity, anything is potentially serious

 hard exudates and other 'background' changes on macula
 check visual acuity
 more common in Type II DM

16
Herpes simplex keratitis

Herpes simplex keratitis most commonly presents with a dendritic corneal

ulcer.

Features

 red, painful eye

 photophobia
 epiphora
 visual acuity may be decreased
 fluorescein staining may show an epithelial ulcer

Management

 immediate referral to an ophthalmologist

 topical aciclovir

17
Herpes zoster ophthalmicus

Herpes zoster ophthalmicus (HZO) describes the reactivation of the

varicella-zoster virus in the area supplied by the ophthalmic division of the
trigeminal nerve. It accounts for around 10% of case of shingles.

Features

 vesicular rash around the eye, which may or may not involve the
actual eye itself
 Hutchinson's sign: rash on the tip or side of the nose. Indicates
nasociliary involvement and is a strong risk factor for ocular
involvement

Management

 oral antiviral treatment for 7-10 days

o ideally started within 72 hours
o intravenous antivirals may be given for very severe infection or
if the patient is immunocompromised
o topical antiviral treatment is not given in HZO
 topical corticosteroids may be used to treat any secondary
inflammation of the eye
 ocular involvement requires urgent ophthalmology review

Complications

 ocular: conjunctivitis, keratitis, episcleritis, anterior uveitis

 ptosis
 post-herpetic neuralgia

18
Holmes-Adie pupil

Holmes-Adie pupil is a benign condition most commonly seen in women. It

is one of the differentials of a dilated pupil.

Overview

 unilateral in 80% of cases

 dilated pupil
 once the pupil has constricted it remains small for an abnormally long
time
 slowly reactive to accommodation but very poorly (if at all) to light

Holmes-Adie syndrome

 association of Holmes-Adie pupil with absent ankle/knee reflexes

19
Horner's syndrome

Features

 miosis (small pupil)

 ptosis
 enophthalmos* (sunken eye)
 anhidrosis (loss of sweating one side)

Distinguishing between causes

 heterochromia (difference in iris colour) is seen in congenital Horner's

 anhidrosis: see below

20
21
Keratitis

Keratitis describes inflammation of the cornea. Microbial keratitis is not like

conjunctivitis - it is potentially sight threatening and should therefore be
urgently evaluated and treated.

Aetiology

Causes

 bacterial
o typically Staphylococcus aureus
o Pseudomonas aeruginosa is seen in contact lens wearers
 fungal
 amoebic
o acanthamoebic keratitis
o accounts for around 5% of cases
o increased incidence if eye exposure to soil or contaminated
water
 parasitic: onchocercal keratitis ('river blindness')

Remember, other factors may causes keratitis:

 viral: herpes simplex keratitis

 environmental
o photokeratitis: e.g. welder's arc eye
o exposure keratitis
o contact lens acute red eye (CLARE)

22
Clinical features

Features

 red eye: pain and erythema

 photophobia
 foreign body, gritty sensation
 hypopyon may be seen

Evaluation and management

Referral

 contact lens wearers

o assessing contact lens wearers who present with a painful red
eye is difficult
o an accurate diagnosis can only usually be made with a slit-
lamp, meaning same-day referral to an eye specialist is usually
required to rule out microbial keratitis

Management

 stop using contact lens until the symptoms have fully resolved
 topical antibiotics
o typically quinolones are used first-line
 cycloplegic for pain relief
o e.g. cyclopentolate

Complications may include:

 corneal scarring
 perforation
 endophthalmitis
 visual loss

23
Lacrimal duct problems

Dacryocystitis is infection of the lacrimal sac

Features

 watering eye (epiphora)

 swelling and erythema at the inner canthus of the eye

Management is with systemic antibiotics. Intravenous antibiotics are

indicated if there is associated periorbital cellulitis

Congenital lacrimal duct obstruction affects around 5-10% of newborns.

It is bilateral in around 20% of cases

Features

 watering eye (even if not crying)

 secondary infection may occur

Symptoms resolve in 99% of cases by 12 months of age

24
Mydriasis

Causes of mydriasis (large pupil)

 third nerve palsy

 Holmes-Adie pupil
 traumatic iridoplegia
 phaeochromocytoma
 congenital

Drug causes of mydriasis

 topical mydriatics: tropicamide, atropine

 sympathomimetic drugs: amphetamines, cocaine
 anticholinergic drugs: tricyclic antidepressants

25
Optic atrophy

Optic atrophy is seen as pale, well demarcated disc on fundoscopy. It is

usually bilateral and causes a gradual loss of vision*. Causes may be
acquired or congenital

Acquired causes

 multiple sclerosis
 papilloedema (longstanding)
 raised intraocular pressure (e.g. glaucoma, tumour)
 retinal damage (e.g. choroiditis, retinitis pigmentosa)
 ischaemia
 toxins: tobacco amblyopia, quinine, methanol, arsenic, lead
 nutritional: vitamin B1, B2, B6 and B12 deficiency

Congenital causes

 Friedreich's ataxia
 mitochondrial disorders e.g. Leber's optic atrophy
 DIDMOAD - the association of cranial Diabetes Insipidus, Diabetes
Mellitus, Optic Atrophy and Deafness (also known as Wolfram's
syndrome)

*strictly speaking optic atrophy is a descriptive term, it is the optic

neuropathy that results in visual loss

26
Optic neuritis

Causes

 multiple sclerosis
 diabetes
 syphilis

Features

 unilateral decrease in visual acuity over hours or days

 poor discrimination of colours, 'red desaturation'
 pain worse on eye movement
 relative afferent pupillary defect
 central scotoma

Management

 high-dose steroids
 recovery usually takes 4-6 weeks

Prognosis

 MRI: if > 3 white-matter lesions, 5-year risk of developing multiple

sclerosis is c. 50%

27
Orbital cellulitis

Orbital cellulitis is the result of an infection affecting the fat and muscles
posterior to the orbital septum, within the orbit but not involving the globe.
It is usually caused by a spreading upper respiratory tract infection from the
sinuses and carries a high mortality rate. Orbital cellulitis is a medical
emergency requiring hospital admission and urgent senior review.
Periorbital (preseptal) cellulitis is a less serious superficial infection anterior
to the orbital septum, resulting from a superficial tissue injury (chalazion,
insect bite etc...). Periorbital cellulitis can progress to orbital cellulitis.

Epidemiology

 Mean age of hospitalisation 7-12 years.

Risk factors

 Childhood
 Previous sinus infection
 Lack of Haemophilus influenzae type b (Hib) vaccination
 Recent eyelid infection/ insect bite on eyelid (Peri-orbital cellulitis)
 Ear or facial infection

Presentation

 Redness and swelling around the eye

 Severe ocular pain
 Visual disturbance
 Proptosis
 Ophthalmoplegia/pain with eye movements
 Eyelid oedema and ptosis
 Drowsiness +/- Nausea/vomiting in meningeal involvement (Rare)

28
Differentiating orbital from preseptal cellulitis

 reduced visual acuity, proptosis, ophthalmoplegia/pain with eye

movements are NOT consistent with preseptal cellulitis

Investigations

 Full blood count – WBC elevated, raised inflammatory markers.

 Clinical examination involving complete ophthalmological
assessment – Decreased vision, afferent pupillary defect, proptosis,
dysmotility, oedema, erythema.
 CT with contrast – Inflammation of the orbital tissues deep to the
septum, sinusitis.
 Blood culture and microbiological swab to determine the organism.
Most common bacterial causes – Streptococcus, Staphylococcus
aureus, Haemophilus influenzae B.

Management

 admission to hospital for IV antibiotics

29
Key points:
1st line of treatment for blepharitis is hot compresses

Drusen = Dry macular degeneration

A relative afferent pupillary defect is when the affected and normal eye appears to
dilate when light is shone on the affected eye

Acute angle closure glaucoma is associated with hypermetropia, where as primary open-
angle glaucoma is associated with myopia

Angioid retinal streaks are a feature of pseudoxanthoma elasticum

Central retinal vein occlusion - sudden painless loss of vision, severe retinal
haemorrhages on fundoscopy

Dorzolamide - carbonic anhydrase inhibitor

Flashes + floaters are most commonly caused by a posterior vitreous detachment

Flashes and floaters - vitreous/retinal detachment

Fundoscopy reveals end organ damage in hypertension

Holmes ADIe = DIlated pupil, females, absent leg reflexes

Horner's syndrome - anhydrosis determines site of lesion:

 head, arm, trunk = central lesion: stroke, syringomyelia

 just face = pre-ganglionic lesion: Pancoast's, cervical rib

 absent = post-ganglionic lesion: carotid artery

Horner's syndrome - anhydrosis determines site of lesion:

 head, arm, trunk = central lesion: stroke, syringomyelia

 just face = pre-ganglionic lesion: Pancoast's, cervical rib

 absent = post-ganglionic lesion: carotid artery

30
Hypocalcaemia is a cause of cataracts

Latanoprost is a prostaglandin analog used in glaucoma. It works by increasing

uveoscleral outflow

Macular degeneration - smoking is risk factor

Monocular transient painless loss of vision (amaurosis fugax) should be treated as a TIA

Patients with orbital cellulitis require admission to hospital for IV antibiotics due to the
risk of cavernous sinus thrombosis and intracranial spread

Pilocarpine is a muscarinic receptor agonist

Red eye - glaucoma or uveitis?

 glaucoma: severe pain, haloes, 'semi-dilated' pupil

 uveitis: small, fixed oval pupil, ciliary flush

Relative afferent pupillary defect indicates an optic nerve lesion or severe retinal
disease

Retinal detachment is a cause of sudden painless loss of vision. It is characterised by a

dense shadow starting peripherally and progressing centrally

Retinitis pigmentosa - night blindness + tunnel vision

Scleritis is painful, episcleritis is not painful

Scleritis is painful, episcleritis is not painful

Treatment of acute glaucoma - acetazolamide + pilocarpine

Vitamin A toxicity is a rare cause of papilloedema

Vitreous haemorrhage is a cause of sudden painless loss of vision in the context of

diabetic retinopathy

31
THANK YOU SO MUCH

32