Notes & Notes

For MRCP part 1 & 11

By
Dr. Yousif Abdallah Hamad

Ophthalmology

Contains:
1/ Passmedicine 2015 (white & black fields)
2/ on examination 2015 (green fields)
3/ pastest 2015 (yellow fields)

Notes & Notes for mrcp

[Link] Abdallah Hamad

Preface
How do I get started? Which books should I read? Which are the best
Self-assessment questions bank? Passmedicine alone? Is it enough? or should I add other
source? Should I add on-examination or pastest?
These are the usual questions asked by the MRCP candidates. For all these questions, and
depending on thorough follow up of a lot of candidates results , feedbacks and comments, rather
than my personal experience, and after consultations of a wide range of mrcp experts, I decide
to create a very concise notes collected from the most popular three mrcp sources :
- Passmedicine 2015 (in black & white fields)
- Onexamination 2015 (in green fields)
And pastest 2015 (in yellow fields)
Occasionally, I add a few facts from previous exams, last guidelines and uptodate
source.
How to use this ''notes & notes''?
I recommend candidates to follow these steps:
- First off all go carefully through this notes 2 times aiming to build a bulky knowledge
of mrcp syllabus.
- then start to practice questions from Passmedicine , onexam and pastest , you will find
it very easy to answer , and if you face any difficult question you have to open your
''notes & notes'' and read the topic again before returns to question banks , this is
crustily helpful to fix the idea.
- In the last few days before your exam, return to this notes and read it once again.
You will find some information written by red or big font, those are the answers of
questions, which are tested in above sources and previous exams. I hope this
collection will be enough to help you get through mrcp part 1 and give you a good grip
before interring part 11.

Dr. Yousif Abdallah Hamad

Notes & Notes for mrcp

[Link] Abdallah Hamad

Acute angle closure glaucoma

Acute angle closure glaucoma is associated with hypermetropia.
Primary open-angle glaucoma is associated with myopia

Glaucoma is a group 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.

Notes & Notes for mrcp

[Link] Abdallah Hamad

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 [Link] oval shape is due to the iris sphincter ischaemia from the
high intraocular pressure.
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

Treatment of acute glaucoma - acetazolamide + pilocarpine

____________________________________________________________

Primary open-angle glaucoma

Glaucoma is a group 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
Primary open-angle glaucoma (POAG), is the most common type of glaucoma.
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:

family history
black patients
myopia
hypertension
diabetes mellitus

Notes & Notes for mrcp

[Link] Abdallah Hamad

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
ophthalmoscopic exam optic disc cupping

Primary open-angle glaucoma: management

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.

Medication

Mode of action

Notes

Prostaglandin analogues (e.g.

Latanoprost)

Increases
uveoscleral outflow

Once daily administration

Adverse effects include
brown pigmentation of the
iris

Beta-blockers (e.g. Timolol)

Reduces aqueous
production

Should be avoided in
asthmatics and patients with
heart block

Sympathomimetics (e.g.
brimonidine, an alpha2adrenoceptor agonist)

Reduces aqueous
production and
increases outflow

Avoid if taking MAOI or

tricyclic antidepressants
Adverse effects include
hyperaemia

Carbonic anhydrase inhibitors

(e.g. Dorzolamide)

Reduces aqueous
production

Systemic absorption may

cause sulphonamide-like
reactions

Miotics (e.g. pilocarpine, a

muscarinic receptor agonist)

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.

____________________________________________________________
Notes & Notes for mrcp

[Link] Abdallah Hamad

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.
Traditionally two forms of macular degeneration are seen:

dry (geographic atrophy) macular degeneration: characterised by drusen - yellow round

spots in Bruch's membrane
wet (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)

Risk factors

age: most patients are over 60 years of age

smoking
family history
more common in Caucasians
high cumulative sunlight exposure
female sex

Features

reduced visual acuity: 'blurred', 'distorted' vision, central vision is affected first
central scotomas
fundoscopy: drusen, pigmentary changes

Investigation and diagnosis

Notes & Notes for mrcp

[Link] Abdallah Hamad

optical coherence tomography: provide cross sectional views of the macula

if neovascularisation is present fluorescein angiography is performed

General management

stop smoking
high dose of beta-carotene, vitamins C and E, and zinc may help to slow down visual loss for
patients with established macular degeneration. Supplements should be avoided in
smokers due to an increased risk of lung cancer

Dry macular degeneration - no current medical treatments

Wet macular degeneration

photocoagulation
photodynamic therapy
anti-vascular endothelial growth factor (anti-VEGF) treatments: intravitreal ranibizumab

____________________________________________________________

Cataracts

Majority

age related
UV light

Notes & Notes for mrcp

[Link] Abdallah Hamad

Systemic

diabetes mellitus
steroids (Inhaled steroids can cause cataracts)
infection (congenital rubella)
metabolic (hypocalcaemia, galactosaemia) (but if the galactosaemia is treated, the cataract is
reversible).
myotonic dystrophy, Down's syndrome

Ocular

trauma
uveitis
high myopia
topical steroids

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

____________________________________________________________

Diabetic retinopathy

Diabetic retinopathy is the most common cause of blindness in adults aged 35-65 years-old.

Notes & Notes for mrcp

[Link] Abdallah Hamad

About 80% of patients with type I diabetes will have retinopathy 10 years after presentation.
By contrast, in type II diabetes, where the time of onset is uncertain, up to 25% of patients will
have retinopathy at the time of diagnosis.
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.

The earliest sign of diabetic retinopathy is the presence of microaneurysms on

fluorescein angiography.
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)
blot haemorrhages (<=3)
hard exudates

1 or more microaneurysm

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

Notes & Notes for mrcp

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

[Link] Abdallah Hamad

Proliferative retinopathy (urgent referral to an ophthalmologist for panretinal photocoagulation)

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
Maculopathy can be indicated by the presence of hard exudates at the macula.
The exudates can be arranged in a ring (circinate exudates) surrounding a point of capillary
leakage.
This can be shown on fluorescein angiography
responds to laser treatment at the point of leakage.

Prognosis
The approximate percentage of eyes that will lose useful vision irretrievably within 5 years if not
treated :

3% in those with background retinopathy

20% for those with exudative
30% for those with pre-proliferative,
50% for those with proliferative retinopathy.

Asymmetric diabetic retinopathy should always raise the suspicion that there is some other
cause of ocular ischaemia on the worst-affected side, such as unilateral or asymmetrical carotid
artery disease do Carotid Doppler
Asymmetric DM Retinopathy suspect ocular ischemia (carotid artery disease)

____________________________________________________________

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 (skin laxity with angioid streaks) is also known as GrnbladStrandberg syndrome.
Ehler-Danlos syndrome

Notes & Notes for mrcp

[Link] Abdallah Hamad

Paget's disease
sickle-cell anaemia
acromegaly

____________________________________________________________

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

____________________________________________________________

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

Abnormally dilated pupil (mydriasis) which does not constrict in response to

light, loss of deep tendon reflexes, and abnormalities of sweating.

Notes & Notes for mrcp

[Link] Abdallah Hamad

characterized by a tonically dilated pupil that reacts slowly to light but shows a more definite
response to accommodation (i.e., light-near dissociation)
frequently seen in females with absent knee or ankle jerks and impaired sweating

pathophysiology
-

Viral or bacterial infection causes damage to neurons in the ciliary ganglion,

located in the posterior orbit, that provides parasympathetic control of eye
constriction.

Additionally, patients with Holmes-Adie Syndrome can also experience problems

with autonomic control of the body caused by damage to the dorsal root ganglia of
the spinal cord.

Diagnosis testing with low dose (1/8%) pilocarpine may constrict the tonic
pupil due to cholinergic denervation supersensitivity. A normal pupil will not
constrict with the dilute dose of pilocarpine.
The affected pupil is usually larger than the other but may constrict with time.
The cause of the associated arreflexia is unknown.

____________________________________________________________

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
Notes & Notes for mrcp

[Link] Abdallah Hamad

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
Most cases of optic neuritis are retrobulbar and hence there are no abnormalities on
fundoscopy.
MRI with gadolinium of the brain will likely show enhancement of the optic nerve
Abnormal visual evoked potentials (VEP)

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%

____________________________________________________________

Retrobulbar neuritis
the patient sees nothing and the doctor sees nothing'

in retrobulbar neuritis it is said that 'the patient sees nothing and the doctor sees
nothing'. As the inflammation is behind the optic nerve head (ie retrobulbar)

Symptoms andsigns of retrobulbar neuritis

-

visual acuity will be reduced to a very variable degree,

afferent pupillary defect will be apparent during the 'swinging flashlight test'.
Colour vision will be reduced
Red desaturation ie when red looks paler to one eye than the other - is a sensitive sign of
optic nerve dysfunction.
Visual field defects will occur, typically a central scotoma, but the type of defect varies.

Notes & Notes for mrcp

[Link] Abdallah Hamad

Retrobulbar neuritis has the same systemic implications as optic neuritis, in that an
episode of optic or retrobulbar neuritis can contribute to a diagnosis of multiple sclerosis
The retrobulbar neuritis seen with ethambutol may be unilateral or bilateral; as such
unilateral symptoms do not preclude the diagnosis.

____________________________________________________________

Herpes simplex keratitis

Herpes simplex keratitis most commonly presents with a dendritic corneal ulcer
The herpes simplex virus may lie dormant in the trigeminal ganglion, reactivation can occur
giving rise to eyelid and periorbital vesicles.
Features

red, painful eye

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

Eczema herpeticum
In patients with atopic dermatitis or eczema herpes simplex infection can cause eczema herpeticum,
which may be further complicated by secondary staphylococcal infection.
This is treated by adding oral antibiotics, for example, flucloxacillin 500 mg q.i.d.
Eczema herpeticum is a serious and potentially life-threatening condition which may require medical
and dermatology input.

Management

immediate referral to an ophthalmologist

topical aciclovir

____________________________________________________________

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

Notes & Notes for mrcp

[Link] Abdallah Hamad

Management

oral antiviral treatment for 7-10 days, ideally started within 72 hours. Topical antiviral treatment
is not given in HZO
oral corticosteroids may reduce the duration of pain but do not reduce the incidence of postherpetic neuralgia
ocular involvement requires urgent ophthalmology review

Complications

ocular: conjunctivitis, keratitis, episcleritis, anterior uveitis

ptosis
post-herpetic neuralgia

____________________________________________________________

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

mechanical removal of the debris from lid margins - cotton wool buds dipped in a mixture of
cooled boiled water and baby shampoo is often used*
artificial tears may be given for symptom relief in people with dry eyes or an abnormal tear film

*an alternative is sodium bicarbonate, a teaspoonful in a cup of cooled water that has recently been
boiled

Notes & Notes for mrcp

[Link] Abdallah Hamad

Keratitis

Keratitis refers to inflammation of one or more of the three corneal layers, the most common of
which is epithelial keratitis. This is characterised by dendritic ulcers. Rarer forms involve the
stroma or endothelium.
Pseudomonas aeruginosa is commonly associated with contact lens related infections.
first line treatment of corneal ulcer Levofloxacin hourly
The management must also include advising the patient to discontinue wearing contact lenses
and referral to a specialist ophthalmic unit.
Keratitis describes inflammation of the cornea. There are a variety of causes.
Recurrence is common.

Features

red eye: pain and erythema (sharp ocular pain)

photophobia
epiphora, and blurred vision (in many cases).
foreign body, gritty sensation
hypopyon may be seen

Infective

Viral: herpes simplex keratitis Treated with topical antivirals, e.g. aciclovir 5 times per day.
Topical steroids or oral antivirals can be used in some cases.

bacterial: typically Staphylococcus aureus. Pseudomonas aeruginosa is seen in contact lens

wearers
Microbial keratitis, causing a white corneal infiltrate, is more likely if there is a history of
contact lens wear or trauma, such as a corneal abrasion or a corneal foreign body.

fungal
amoebic: acanthamoebic keratitis
parasitic: onchocercal keratitis ('river blindness')

Enviromental

photokeratitis: e.g. welder's arc eye

exposure keratitis
contact lens acute red eye (CLARE)

Notes & Notes for mrcp

[Link] Abdallah Hamad

Acanthamoeba keratitis

is a rare and serious keratitis associated with contact lens (CL) wear.
Risk factors for Acanthamoeba keratitis are exposure of CL to contaminated water, for
example, using tap water to clean CL or swimming in lenses.
Clinical features of Acanthamoeba keratitis include pain out of proportion to clinical signs, ring
infiltrates and radial keratoneuritis.
Brolene drops is used in the management of Acanthamoeba keratitis,

Marginal keratitis

Marginal keratitis is areas of peripheral corneal infiltrates/ulcers associated with blepharitis.

It classically causes an infiltrate near the limbal edge with an area of clear cornea. There may
be limbal vessels growing towards the lesion(s).
It is thought to be caused by a hypersensitivity reaction to staphylococcal exotoxins
fromStaphylococcus aureus present on the lid margins in blepharitis.
Treatment involves topical steroids for the keratitis and lid hygiene advice and topical
antibiotics to treat the underlying blepharitis. In severe cases oral doxycycline can also be
used.

____________________________________________________________

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

Notes & Notes for mrcp

[Link] Abdallah Hamad

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
sign on ocular examination Hypopyon (While a hypopyon usually implies infective
endophthalmitis (eg following cataract surgery), a sterile hypopyon can occur in cases of
severe iritis (eg Behcet's disease).

Iritis is associated with conditions such as:

-

Reiter's

Behcet's

Psoriatic arthropathy (about 20%)

and inflammatory bowel disease.

A chronic iritis is rarely described in association with Lyme disease.

Signs of anterior uveitis

Notes & Notes for mrcp

[Link] Abdallah Hamad

Keratic precipitates: (opaque aggregates of inflammatory cells deposited on the endothelium

in anterior uveitis. They are typically located inferiorly.

Cells +/- flare +/- fibrin in the anterior chamber

Ciliary injection - localised conjunctival injection (redness) around the limbus
Posterior synechiae - where part of the pupil margin becomes stuck to the lens
Hypopyon (in severe anterior uveitis).Hypopyon is refers to a collection of inflammatory
material in the anterior chamber resulting in an opaque fluid level visible inferiorly. It occurs in
severe cases of anterior uveitis or endophthalmitis.

Scleritis

Scleritis : inflammation that occurs throughout the entire thickness of the sclera,
severe pain (may be worse on movement) and tenderness
50% of cases are bilateral.
Pain often radiates to the forehead, brow and jaw. This pain worsens with movement of the
eye, and is classically worse at night.
There is associated watering, photophobia and a gradual decrease in vision (sometimes with
diplopia).
Systemic symptoms such as fever, headache and vomiting can occur.
may be underlying autoimmune disease e.g. rheumatoid arthritis
Around 50% of patients with scleritis have an underlying disease, of which the majority are
connective tissue disorders. Rheumatoid arthritis is the most common.

On examination the globe is tender, and the sclera can have a bluish tinge.
Application of topical phenylephrine 2.5% leads to blanching of episcleral vessels in
episcleritis but not in scleritis.
Management ultimately depends on the underlying cause, but includes NSAIDs and
prednisolone.
The patient should be referred urgently to the ophthalmology clinic

Conjunctivitis

purulent discharge if bacterial, clear discharge if viral

Viral conjunctivitis
-

causes redness, soreness and watering.

In severe cases it can cause a keratitis which may affect vision.

It is highly contagious so patients should be advised to practise strict hand hygiene, to

avoid sharing towels and to take time off work.

Notes & Notes for mrcp

[Link] Abdallah Hamad

It is a self-limiting disease which may take several weeks to resolve.

Patients are treated with topical lubricants and some ophthalmologists give topical
chloramphenicol to protect against secondary bacterial infections.

Subconjunctival haemorrhage

history of trauma or coughing bouts

____________________________________________________________

Posterior uveitis

Posterior uveitis describes inflammation of the choroid, which can involve the retinal vessels.
It presents with gradual visual loss and floaters, which is often bilateral.
Discomfort and erythema are rare.
Slit light examination can demonstrate inflammatory lesions on the retina or choroid, with
inflammation of the retinal vessels and oedema of the optic nerve.

____________________________________________________________
CMV Retinitis: causes hemorrhage at the edge of the area of retinal necrosis

____________________________________________________________

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

Notes & Notes for mrcp

[Link] Abdallah Hamad

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

____________________________________________________________

Retinitis pigmentosa
Retinitis pigmentosa primarily affects the peripheral retina resulting in funnel vision
Features

night blindness is often the initial sign

funnel vision (the preferred term for tunnel 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
mitochondrial myopathy

Notes & Notes for mrcp

[Link] Abdallah Hamad

Fundus showing changes secondary to retinitis pigmentosa

Notes & Notes for mrcp

[Link] Abdallah Hamad

CHRPE - congenital hypertrophy of the retinal pigment

epithelium.

These can be typical' or 'atypical'.

Typical CHRPE are grey or black, with depigmented lacunas, and are found in one quadrant
of one [Link] do not affect vision.
Atypical CHRPE have a white fishtail and are bilateral. They do not affect vision, but if there
are more than four atypical CHRPE in each eye, then familial adenosis polyposis or Gardner
syndrome might be suspected as an association. Colonoscopy and examination of all
family members would therefore be appropriate.
A referral to gastroenterology is the best next step.

____________________________________________________________

Sudden painless loss of vision

An elderly patient with acute visual loss has giant cell arteritis until proved otherwise

The most common causes of a sudden painless loss of vision are as follows:

ischaemic optic neuropathy (e.g. temporal arteritis or atherosclerosis)

occlusion of central retinal vein
occlusion of central retinal artery
vitreous haemorrhage
retinal detachment

Ischaemic optic neuropathy

may be due to arteritis (e.g. temporal arteritis) or atherosclerosis (e.g. hypertensive, diabetic
older patient)
due to occlusion of the short posterior ciliary arteries, causing damage to the optic nerve
altitudinal field defects are seen

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

Notes & Notes for mrcp

[Link] Abdallah Hamad

Vitreous haemorrhage

causes: diabetes, bleeding disorders. the most likely source of bleeding in a diabetic patient is
fragile neovascular tissue (Proliferative retinopathy)
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

peripherally progresses towards the
central vision
A veil or curtain over the field of
vision
Straight lines appear curved
Central visual loss

Large bleeds cause sudden visual

loss
Moderate bleeds may be described
as numerous dark spots
Small bleeds may cause 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

Notes & Notes for mrcp

[Link] Abdallah Hamad

Keratoconus
Keratoconus, meaning "cone shaped," describes a condition in which the cornea (the clear front window of
the eye) becomes thin and protrudes. This abnormal shape can cause serious distortion of visual images.

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Rheumatoid arthritis: ocular manifestations

Ocular manifestations of rheumatoid arthritis are common, with 25% of patients having eye problems
Ocular manifestations
Scleritis is painful, episcleritis is not painful

keratoconjunctivitis sicca (most common)

dry eyes
- do Tear-film integrity
- A reflex response to irritation of the corneal surface is epiphora, or watering.
- Symptoms will be worse when tear-film evaporation is greater,

episcleritis (erythema)
scleritis (erythema and pain)
corneal ulceration
keratitis

Iatrogenic

steroid-induced cataracts

'steroid cataract' is typically posterior subcapsular, and causes constant and gradually
progressive blur.
Steroids can cause raised blood glucose levels. Fluctuating blood sugar levels can cause
osmotic swelling of the lens in the eye, resulting in fluctuations in vision. However, diabetic
retinopathy will not affect vision unless maculopathy occurs or vitreous haemorrhage
occurs.
Steroids raised intraocular pressure,
chloroquine retinopathy

Notes & Notes for mrcp

[Link] Abdallah Hamad

Episcleritis

Results in ocular irritation with nodules.

It is acute in onset, with mild pain or discomfort / grittiness.
It can be unilateral or bilateral, with localised or diffuse red eye.
There may be mild photophobia and watering. The lack of photophobia and discharge,
and normal vision, makes episcleritis the most likely option

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Retinoblastoma

The incidence is 1 in 16,000 live births.

Genetic predisposition occurs in 20% of patients with unilateral disease, and 30% of patients
with bilateral disease.
The gene has been localised to 13q and the inherited form is associated with an increased risk
of malignancy such as osteosarcoma and pineal tumours. It may be inherited as autosomal
dominant.
The commonest presentation is leucocoria (yellowish white pupil reflex), and there may
be diminished or absent vision or strabismus.
Late symptoms are:
- pupil irregularity
- hyphema
- pain
- proptosis
- signs of raised intracranial pressure.
The tumours have rarely metastasised before they are detected.

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Ectopia lentis
Ectopia lentis/subluxation of the lens is associated with:

Ehlers-Danlos syndrome
Marfan's syndrome
Weill-Marchesani syndrome (short stature, skeletal abnormalities and ectopia lentis), and
Refsum's disease.

Notes & Notes for mrcp

[Link] Abdallah Hamad

Chronic progressive external ophthalmoplegia (CPEO)

CPEO is an eye movement disorder which is often associated with mitochondrial disease.

Although it typically presents in young adults, it can affect all ages.

Classically the initial sign is bilateral ptosis, which is often unnoticed by the patient until the lid
droops to the point of producing a visual field defect.

Ophthalmoplegia then develops, which is symmetrical and again therefore unnoticed until
ocular motility limits peripheral vision. All directions of gaze are affected, but downward gaze
often less so.

Weakness of extraocular muscle groups, such as orbicularis oculi and facial muscles, can be
present in 25% of patients.

Visual acuity is affected in 95% of patients, but the deficit is usually mild.

CPEO is diagnosed via muscle biopsy, which shows an accumulation of enlarged

mitochrondria. PCR can also determine a mutation of mitochondrial DNA.

There are no specific treatments currently, but surgery can be used to correct ptosis and
prisms can be used to help with ophthalmoplegia.

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Kearns-Sayre syndrome

This is a mitochondrial inherited disease, and as such is only passed on by mothers to

offspring.

It is a slowly progressive neuromuscular disorder associated with progressive external

ophthalmoplegia and heart conduction defect.

Ocular manifestations include ptosis and peripheral retinal bony spiculed appearances.

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Fundoscopic features

Cytomegalovirus (CMV) retinitis

-

secondary to human immunodeficiency virus (HIV)

Fundoscopy of the left eye revealed an extensive 'brushfire-like' lesion in the major
superior temporal arcade with a large patch of white fluffy lesion mixed with extensive
retinal haemorrhages.

Ocular histoplasmosis and syphilitic choroiditis would give a fundus picture of multiple
whitish lesions.

Syphilitic neuroretinitis would normally give a picture of a macular star exudation.

Tuberculous periphlebitis gives a picture of perivenous sheathing and minimal retinal

haemorrhages.

Notes & Notes for mrcp

[Link] Abdallah Hamad

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Bilateral internal carotid artery displacement can cause binasal incongruous hemianopia if
the optic nerves are compressed.

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Anisocoria
is a condition characterized by an unequal size of the eyes' pupils. Affecting 20% of the population, it
can be an entirely harmless condition or a symptom of more serious medical problems
The history of anisocoria, with headaches and diplopia should ring alarm bells, in that a lifethreatening posterior communicating artery aneurysm/berry aneurysm needs to be excluded
urgently.

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Eye signs in Systemic diseases

Lisch nodules of the iris are golden nodules occurring bilaterally in the teenage
years onwards in Neurofibromatosis type 1 (NF-1). Axillary freckles appear at 10
years of age, while cafe au lait spots increase in size and number throughout childhood.
Brushfield spots of the iris are found in people with Down syndrome.
Kayser-Fleischer rings are due to copper deposition in Descemet's membrane of the
cornea.
Band keratopathy is caused by calcium deposition in Bowman's layer of the
cornea. Patients who present with band keratopathy should have a serum calcium
and phosphate level
Ectopia lentis with aortic regurgitation Marfan syndrome (Lens dislocation
(classically upwards)).
Inferior dislocated lens consistent with a diagnosis of homocytinstinuria.
Roth's spots haemorrhages in the retina associated with subacute bacterial
endocarditis.

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Immune reconstitution uveitis

associated with recovery of the CD4 count in HIV. In this condition a granulomatous uveitis
appears as the immune system recovers as a result of autoimmune mechanisms.

Notes & Notes for mrcp

[Link] Abdallah Hamad

Hyphaema
Occurs when bleeding from iris vessels fills the anterior chamber with blood and if there is
enough blood
the main risk in the acute stage is of raised intraocular pressure (IOP).
Intravenous carbonic anhydrase inhibitors is the most appropriate

treatment
Strict rest is vital if a hyphaema is present, as there is an increased risk of a second bleed
in the initial period.
avoid drops that dilate the pupil (such as anticholinergics) the iris remains stable and a
second bleed is therefore less likely.

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Acute corneal hydrops

Acute corneal hydrops occurs in advanced keratoconus, which is the most common cause of
corneal ectasia.
The cornea is made of three main layers:
- Epithelium
- Stroma
- Endothelium
The transparency of the cornea is maintained by the endothelium which constantly pumps
water out from the stroma.
Descemet's membrane is a specialised basement membrane which lies between the
endothelium and stroma, which helps to provide structural integrity to the cornea.
In acute corneal hydrops the endothelium and Descemet's membrane split which allows
aqueous to enter the corneal stroma.
Stromal and epithelial oedema results in corneal opacification and formation of epithelial
bullae.
Patient present with painful loss of vision
cornea is opaque and appears to be protruding
Keratoconus is associated with atopic conditions (for example, asthma, hay fever, eczema)
and Down syndrome.

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Subtarsal foreign body

history of 'gritty pain' in the eye with pain on blinking.

The appropriate management would be to examine underneath the lid to search for and
remove a foreign body.
Further management would involve topical chloramphenicol ointment three to four times a day
for five days.

Notes & Notes for mrcp

[Link] Abdallah Hamad

Notes & Notes for mrcp

[Link] Abdallah Hamad