PASSMEDICINE NOTES

OPHTHALMOLOGY
CONTENTS
No Topic Page
1 SUDDEN LOSS OF VISION
2 TUNNEL VISION
3 ORBITAL CELLULITIS
4 RED EYE
5 ACUTE ANGLE CLOSURE GLAUCOMA
6 PRIMARY OPEN-ANGLE GLAUCOMA
7 PRIMARY OPEN-ANGLE GLAUCOMA: MANAGEMENT
8 ANTERIOR UVEITIS
9 BLEPHARITIS
10 LACRIMAL DUCT PROBLEMS
11 CATARACTS
12 KERATITIS
13 MYDRIASIS
14 RELATIVE AFFERENT PUPILLARY DEFECT
15 ARGYLL-ROBERTSON PUPIL
16 HOLMES-ADIE PUPIL
17 HORNER'S SYNDROME
18 AGE-RELATED MACULAR DEGENERATION
19 PAPILLOEDEMA
20 HYPERTENSIVE RETINOPATHY
21 DIABETIC RETINOPATHY
22 POSTERIOR VITREOUS DETACHMENT
23 OPTIC ATROPHY
24 RETINITIS PIGMENTOSA
25 CENTRAL RETINAL ARTERY OCCLUSION
26 CENTRAL RETINAL VEIN OCCLUSION
27 ANGIOID RETINAL STREAKS
28 OPTIC NEURITIS
29 HERPES ZOSTER OPHTHALMICUS
30 HERPES SIMPLEX KERATITIS
31 RHEUMATOID ARTHRITIS: OCULAR MANIFESTATIONS
SUDDEN LOSS OF VISION
Sudden loss is a frightening symptom for patients. It may represent an ongoing issue or only be
temporary. The term transient monocular visual loss (TVML) describes a sudden, transient loss of
vision that lasts less than 24 hours.

The most common causes of a sudden painless loss of vision are as follows:
 ischaemic/vascular (e.g. thrombosis, embolism, temporal arteritis etc). This includes recognised
syndromes e.g. occlusion of central retinal vein and occlusion of central retinal artery
 vitreous haemorrhage
 retinal detachment
 retinal migraine

Ischaemic/vascular
 often referred to as 'amaurosis fugax'
 wide differential including large artery disease (atherothrombosis, embolus, dissection), small
artery occlusive disease (anterior ischemic optic neuropathy, vasculitis e.g. temporal arteritis),
venous disease and hypoperfusion
 may represent a form of transient ischaemic attack (TIA). It should therefore be treated in a
similar fashion, with aspirin 300mg being given
 altitudinal field defects are often seen: 'curtain coming down'
 ischaemic optic neuropathy is due to occlusion of the short posterior ciliary arteries, causing
damage to the optic nerve

Central retinal vein occlusion

 incidence increases with age, more common than arterial occlusion
 causes: glaucoma, polycythaemia, hypertension
 severe retinal haemorrhages are usually seen on fundoscopy

Central retinal artery occlusion

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

Vitreous haemorrhage
 causes: diabetes, bleeding disorders, anticoagulants
 features may include sudden visual loss, dark spots

Retinal detachment
 features of vitreous detachment, which may precede retinal detachment, include flashes of light
or floaters (see below)

Differentiating posterior vitreous detachment, retinal detachment and vitreous haemorrhage

Posterior vitreous detachment
Flashes of light (photopsia) - in
the peripheral field of vision
Floaters, often on the temporal
side of the central vision
Retinal detachment Vitreous haemorrhage
Dense shadow that starts Large bleeds cause sudden
peripherally progresses towards the visual loss
central vision Moderate bleeds may be
A veil or curtain over the field of described as numerous dark
vision spots
Straight lines appear curved Small bleeds may cause
Central visual loss floaters

TUNNEL VISION
Tunnel vision is the concentric diminution of the visual fields

Causes
 papilloedema
 glaucoma
 retinitis pigmentosa
 choroidoretinitis
 optic atrophy secondary to tabes dorsalis
 hysteria
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)

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
RED EYE
There are many possible causes of a red eye. It is important to be able to recognise the causes which
require urgent referral to an ophthalmologist. Below is a brief summary of the key distinguishing
features

Acute angle closure glaucoma

 severe pain (may be ocular or headache)
 decreased visual acuity, patient sees haloes
 semi-dilated pupil
 hazy cornea

Anterior uveitis
 acute onset
 pain
 blurred vision and photophobia
 small, fixed oval pupil, ciliary flush

Scleritis
 severe pain (may be worse on movement) and tenderness
 may be underlying autoimmune disease e.g. rheumatoid arthritis

Conjunctivitis
 purulent discharge if bacterial, clear discharge if viral

Subconjunctival haemorrhage
 history of trauma or coughing bouts

Endophthalmitis
 typically red eye, pain and visual loss following intraocular surgery
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

PRIMARY OPEN-ANGLE GLAUCOMA

Glaucomas are optic neuropathies associated with raised intraocular pressure (IOP). They can be
classified based on whether the peripheral iris is covering the trabecular meshwork, which is
important in the drainage of aqueous humour from the anterior chamber of the eye. In open-angle
glaucoma, the iris is clear of the meshwork. The trabecular network functionally offers an increased
resistance to aqueous outflow, causing increased IOP. It is now recognised that a minority of
patients with raised IOP do not have glaucoma and vice versa.

Epidemiology
 affects 0.5% of people over the age of 40
 the prevalence increases with age up to 10% over the age of 80 years
 affects males and females equally
Primary open-angle glaucoma (POAG, also referred to as chronic simple glaucoma) is present in
around 2% of people older than 40 years. Other than age, risk factors include:
 genetics: first degree relatives of an open-angle glaucoma patient have a 16% chance of
developing the disease
 black patients
 myopia
 hypertension
 diabetes mellitus
 corticosteroids

POAG may present insidiously and for this reason is often detected during routine optometry
appointments. Features may include
 peripheral visual field loss - nasal scotomas progressing to 'tunnel vision'
 decreased visual acuity
 optic disc cupping

Fundoscopy signs of primary open-angle glaucoma (POAG):

1. Optic disc cupping - cup-to-disc ratio >0.7 (normal = 0.4-0.7), occurs as loss of disc substance
makes optic cup widen and deepen
2. Optic disc pallor - indicating optic atrophy
3. Bayonetting of vessels - vessels have breaks as they disappear into the deep cup and re-appear at
the base
4. Additional features - Cup notching (usually inferior where vessels enter disc), Disc haemorrhages

Diagnosis:
 Case finding and provisional diagnosis is done by an optometrist
 Referral to the ophthalmologist is done via the GP
 Final diagnosis is done by investigations as below

Investigations:
 automated perimetry to assess visual field
 slit lamp examination with pupil dilatation to assess optic neve and fundus for a baseline
 applanation tonometry to measure IOP
 central corneal thickness measurement
 gonioscopy to assess peripheral anterior chamber configuration and depth
 Assess risk of future visual impairment, using risk factors such as IOP, central corneal thickness
(CCT), family history, life expectancy

PRIMARY OPEN-ANGLE GLAUCOMA: MANAGEMENT

Glaucomas are optic neuropathies associated with raised intraocular pressure (IOP). They can be
classified based on whether the peripheral iris is covering the trabecular meshwork, which is
important in the drainage of aqueous humour from the anterior chamber of the eye. In open-angle
glaucoma, the iris is clear of the meshwork. The trabecular network functionally offers an increased
resistance to aqueous outflow, causing increased IOP.

Epidemiology
 affects 0.5% of people over the age of 40
 the prevalence increases with age up to 10% over the age of 80 years
 affects males and females equally

Causes
 increasing age
 genetics: first degree relatives of an open-angle glaucoma patient have a 16% chance of
developing the disease

Symptoms:
 characterised by a slow rise in intraocular pressure: symptomless for a long period
 typically present following an ocular pressure measurement during a routine examination by an
optometrist

Signs:
 increased intraocular pressure
 visual field defect
 pathological cupping of the optic disc1

Case finding:
 optic nerve head damage visible under the slit lamp
 visual field defect
 IOP > 24 mmHg as measured by Goldmann-type applanation tonometry
 if suspected full investigations are performed

Diagnosis:
 Case finding and provisional diagnosis is done by an optometrist
 Referral to the ophthalmologist is done via the GP
 Final diagnosis is done by investigations as below

Investigations:
 automated perimetry to assess visual field
 slit lamp examination with pupil dilatation to assess optic neve and fundus for a baseline
 applanation tonometry to measure IOP
 central corneal thickness measurement
 gonioscopy to assess peripheral anterior chamber configuration and depth
 Assess risk of future visual impairment, using risk factors such as IOP, central corneal thickness
(CCT), family history, life expectancy
The majority of patients with primary open-angle glaucoma are managed with eye drops. These aim
to lower intra-ocular pressure which in turn has been shown to prevent progressive loss of visual
field.

NICE guidelines:
 first line: prostaglandin analogue (PGA) eyedrop
 second line: beta-blocker, carbonic anhydrase inhibitor, or sympathomimetic eyedrop
 if more advanced: surgery or laser treatment can be tried2

Reassessment
 important to exclude progression and visual field loss
 needs to be done more frequently if: IOP uncontrolled, the patient is high risk, or there is
progression

Medication
Prostaglandin analogues
(e.g. latanoprost)
Beta-blockers (e.g.
timolol, betaxolol)
Sympathomimetics (e.g.
brimonidine, an alpha2-
adrenoceptor agonist)
Carbonic anhydrase inhibitors
(e.g. Dorzolamide)
Miotics (e.g. pilocarpine,
a muscarinic receptor agonist)
Mode of action Notes
Increases uveoscleral outflow Once daily administration
Adverse effects include brown
pigmentation of the iris, increased
eyelash length
Reduces aqueous production Should be avoided in asthmatics and
patients with heart block
Reduces aqueous production Avoid if taking MAOI or tricyclic
and increases outflow antidepressants
Adverse effects include hyperaemia
Reduces aqueous production Systemic absorption may cause
sulphonamide-like reactions
Increases uveoscleral outflow Adverse effects included a
constricted pupil, headache and
blurred vision

Surgery in the form of a trabeculectomy may be considered in refractory cases.

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
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
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

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

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

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

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
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

Anisocoria may result in apparent mydriasis, due to the difference with the other pupil.

RELATIVE AFFERENT PUPILLARY DEFECT

Also known as the Marcus-Gunn pupil, a relative afferent pupillary defect is found by the 'swinging
light test'. It is caused by a lesion anterior to the optic chiasm i.e. optic nerve or retina

Finding
 the affected and normal eye appears to dilate when light is shone on the affected

Causes
 retina: detachment
 optic nerve: optic neuritis e.g. multiple sclerosis

Pathway of pupillary light reflex

 afferent: retina → optic nerve → lateral geniculate body → midbrain
 efferent: Edinger-Westphal nucleus (midbrain) → oculomotor nerve
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

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
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

Pre-ganglionic
Central lesions lesions Post-ganglionic lesions
Anhidrosis of the face, arm and Anhidrosis of the face No anhidrosis
trunk
Stroke Pancoast's tumour Carotid artery dissection
Syringomyelia Thyroidectomy Carotid aneurysm
Multiple sclerosis Trauma Cavernous sinus
Tumour Cervical rib thrombosis
Encephalitis Cluster headache

*in reality the appearance is due to a narrow palpebral aperture rather than true enophthalmos
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

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.

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.
PAPILLOEDEMA
Papilloedema describes optic disc swelling that is caused by increased intracranial pressure. It is
almost always bilateral.

The following features may be observed during fundoscopy:

 venous engorgement: usually the first sign
 loss of venous pulsation: although many normal patients do not have normal pulsation
 blurring of the optic disc margin
 elevation of optic disc
 loss of the optic cup
 Paton's lines: concentric/radial retinal lines cascading from the optic disc

Causes of papilloedema
 space-occupying lesion: neoplastic, vascular
 malignant hypertension
 idiopathic intracranial hypertension
 hydrocephalus
 hypercapnia

Rare causes include

 hypoparathyroidism and hypocalcaemia
 vitamin A toxicity

HYPERTENSIVE RETINOPATHY
The table below shows the Keith-Wagener classification of hypertensive retinopathy

Stage Features
I Arteriolar narrowing and tortuosity
Increased light reflex - silver wiring
II Arteriovenous nipping
III Cotton-wool exudates
Flame and blot haemorrhages
IV Papilloedema
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):

Traditional classification New classification

Background retinopathy Mild NPDR

 microaneurysms (dots)  1 or more microaneurysm

 blot haemorrhages (<=3)
 hard exudates
Moderate NPDR

Pre-proliferative retinopathy
 cotton wool spots (soft exudates;
ischaemic nerve fibres)
 > 3 blot haemorrhages
 venous beading/looping
 deep/dark cluster haemorrhages
 more common in Type I DM, treat
with laser photocoagulation
 microaneurysms
 blot haemorrhages
 hard exudates
 cotton wool spots, venous beading/looping and
intraretinal microvascular abnormalities (IRMA)
less severe than in severe NPDR
Severe NPDR

 blot haemorrhages and microaneurysms in 4

quadrants
 venous beading in at least 2 quadrants
 IRMA in at least 1 quadrant

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
POSTERIOR VITREOUS DETACHMENT
Posterior vitreous detachment is the separation of the vitreous membrane from the retina. This
occurs due to natural changes to the vitreous fluid of the eye with ageing. Posterior vitreous
detachment is a common condition that does not cause any pain or loss of vision. However, rarely
the separation of the vitreous membrane can lead to tears and detachment of the retina. It is
important to rule out retinal tears or retinal detachment in anyone with suspected posterior
vitreous detachment, as they may result in permanent loss of vision.

Epidemiology:
 Occur in over 75% of people over the age of 65
 More common in females

Risk factors:
 As people age, the vitreous fluid in the eye becomes less viscous, and thus, does not hold its
shape as well. Therefore, it pulls the vitreous membrane away from the retina towards the
centre of the eye.
 Highly myopic (near-sighted) patients are also at increased risk of developing posterior vitreous
detachment earlier in life. This is because the myopic eye has a longer axial length than an
emmetropic eye.

Symptoms:
 The sudden appearance of floaters (occasionally a ring of floaters temporal to central vision)
 Flashes of light in vision
 Blurred vision
 Cobweb across vision
 The appearance of a dark curtain descending down vision (means that there is also retinal
detachment)

Signs:
 Weiss ring on ophthalmoscopy (the detachment of the vitreous membrane around the optic
nerve to form a ring-shaped floater).

Investigations:
 All patients with suspected vitreous detachment should be examined by an ophthalmologist
within 24hours to rule out retinal tears or detachment.

Management:
 Posterior vitreous detachment alone does not cause any permanent loss of vision. Symptoms
gradually improve over a period of around 6 months and therefore no treatment is necessary.
 If there is an associated retinal tear or detachment the patient will require surgery to fix this.
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
RETINITIS PIGMENTOSA
Retinitis pigmentosa primarily affects the peripheral retina resulting in tunnel vision

Features
 night blindness is often the initial sign
 tunnel vision due to loss of the peripheral retina (occasionally referred to as funnel vision)
 fundoscopy: black bone spicule-shaped pigmentation in the peripheral retina, mottling of the
retinal pigment epithelium

Associated diseases
 Refsum disease: cerebellar ataxia, peripheral neuropathy, deafness, ichthyosis
 Usher syndrome
 abetalipoproteinemia
 Lawrence-Moon-Biedl syndrome
 Kearns-Sayre syndrome
 Alport's syndrome

Fundus showing changes secondary to retinitis pigmentosa

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

CENTRAL RETINAL VEIN OCCLUSION

Central retinal vein occlusion (CRVO) is a differential for sudden painless loss of vision.

Risk factors
 increasing age
 glaucoma
 polycythaemia

Features
 sudden, painless reduction or loss of visual acuity, usually unilaterally
 severe retinal haemorrhages are usually seen on fundoscopy

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
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%
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

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
RHEUMATOID ARTHRITIS: OCULAR MANIFESTATIONS
Ocular manifestations of rheumatoid arthritis are common, with 25% of patients having eye
problems

Ocular manifestations
 keratoconjunctivitis sicca (most common)
 episcleritis (erythema)
 scleritis (erythema and pain)
 corneal ulceration
 keratitis

Iatrogenic
 steroid-induced cataracts
 chloroquine retinopathy