Emergency Acute and Rapid Access Ophthalmology Practical, Clinical and Managerial Aspects 2019 PDF

Emergency, Acute
and Rapid Access

Ophthalmology
Practical, Clinical and Managerial

Aspects
Josephine Duvall-Young
123
123
Emergency, Acute and Rapid Access
Ophthalmology
Emergency, Acute and Rapid

Access Ophthalmology
Practical, Clinical and Managerial Aspects
Ophthalmology Department
Wirral University Teaching Hospital NHS
Birkenhead
United Kingdom
ISBN 978-3-319-92368-0 ISBN 978-3-319-92369-7 (eBook)

https://doi.org/10.1007/978-3-319-92369-7
Library of Congress Control Number: 2018954715
© Springer Nature Switzerland AG 2019

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Contents
1 Introduction�� 1
1.1 What Is the Problem?�� 1
1.2 Defining the Problem�� 2
1.3 The Book�� 3
Part I Clinical Aspects

2 Emergency Ophthalmology: Trauma�� 7
2.1 Terrorist Attack or Major Incident�� 7
2.2 Globe Penetration and Rupture�� 9
2.3 Retrobulbar Haemorrhage�� 9
2.4 Orbital Fracture�� 11
2.5 Lid Injury�� 11
2.6 Chemical and Thermal Burns �� 12
2.7 Contusion�� 13
2.8 Minor Injuries �� 14
2.9 Local Anaesthesia �� 15
Further Reading �� 16
3 True Ophthalmic Emergencies: Non Traumatic�� 17
3.1 Central Retinal Artery Occlusion�� 17
3.2 Endophthalmitis�� 20
4 Urgent Eye Conditions�� 25
4.1 Acute Angle Closure Glaucoma�� 25
4.2 Secondary Glaucomas�� 28
4.3 Retinal Detachment�� 30
4.4 Anterior Ischaemic Optic Neuropathy�� 30
4.4.1 Giant Cell Arteritis�� 31
4.5 Orbital Cellulitis�� 34
v
vi Contents
5 Conditions Requiring Same Day Management�� 37

5.1 Corneal Ulcers�� 37
5.1.1 Herpes Simplex Keratitis�� 37
5.1.2 Herpes Zoster Ophthalmicus (HZO)�� 39
5.1.3 Adenoviral Keratoconjunctivitis �� 40
5.1.4 Bacterial Ulcer�� 40
5.1.5 Contact Lens Associated Corneal Disease�� 42
5.1.6 Acanthamoeba Keratitis�� 43
5.1.7 Fungal Ulcers�� 43
5.1.8 Non Infective Ulcers �� 44
5.1.9 Allergic Eye Disease�� 45
5.1.10 Keratoconus�� 46
5.1.11 Keratitis Secondary to Blepharitis�� 47
5.1.12 Idiopathic Keratitis�� 49
5.2 Scleritis �� 49
5.3 Uveitis �� 51
5.3.1 Acute Anterior Uveitis (AAU)�� 51
5.3.2 Identifiable Associations of AAU �� 54
5.3.3 Inflammatory Chorioretinopathies�� 56
5.3.4 Lyme Disease�� 57
5.3.5 Medical Evaluation of Uveitis Patients�� 57
5.3.6 Immunosuppression�� 59
5.4 Recent Onset or Progressive Ophthalmoplegias �� 59
5.5 Transient Ischaemic Attack (TIA)�� 63
6 Rapid Access: Neurology �� 65
6.1 Headache�� 65
6.1.1 Primary Headache�� 66
6.1.2 Secondary Headache�� 67
6.2 Papilloedema and Disc Swelling�� 69
6.3 Subacute or Self limiting Angle Closure�� 74
6.4 Neurological Visual Field defect�� 76
6.5 Optic Neuritis�� 78
6.5.1 Afferent Pupil Defect�� 80
6.6 Anisocoria �� 82
6.7 Bell’s Palsy�� 82
6.8 Proptosis�� 83
7 Rapid Access: Retina �� 87
7.1 Retinal Vein Occlusion�� 87
7.2 Central Serous Retinopathy (CSR) �� 90
7.3 Choroidal Infarction�� 90
7.4 Choroidal Haemangioma�� 91
7.5 Choroidal Naevus�� 91
Contents vii
7.6 Vitreous Haemorrhage�� 92

7.7 Paraneoplastic Syndromes�� 92
8 Referrals from Other Hospital Departments�� 93
8.1 Toxic Effects of Drugs�� 93
9 Paediatrics�� 95
9.1 Neonates�� 95
9.2 Infants and Toddlers�� 96
9.2.1 Poor Visual Responses�� 96
9.2.2 Developmental Glaucoma�� 97
9.2.3 Non Accidental Injury�� 97
9.2.4 Strawberry Naevus�� 97
9.2.5 Nystagmus�� 97
9.2.6 Strabismus�� 98
9.2.7 Anisocoria �� 99
9.3 School Age Children �� 99
9.3.1 Optometry Referrals�� 99
9.3.2 Blepharitis �� 100
9.3.3 Intracranial Tumour�� 100
9.3.4 Neurofibromatosis�� 100
10 Online Resources�� 103
Part II Leading an Emergency and Rapid Access Service

11 Introduction�� 109
12 Organisation and Management�� 113
12.1 Volume and Workload Planning �� 113
12.2 Workforce�� 115
12.3 Walk in or Prebooked Service?�� 116
12.3.1 Walk in Service �� 116
12.3.2 Sorting Categories for Walk in Service�� 117
12.3.3 Prebooked Service�� 117
12.4 Training�� 119
12.5 Equipment and Useful Reference Charts�� 121
12.5.1 Visual Acuity Recording �� 123
12.5.2 Visual Acuity Measured Using Gratings
with Preferential Looking (Infants)�� 123
12.5.3 Corneal Thickness Adjustments for IOP�� 123
12.6 Protocols and Guidelines/Standard Operating
Procedures (SOP)�� 124
12.7 Patient Information and Education �� 127
viii Contents
12.8 Networking �� 127

12.9 Audit �� 127
Index. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131
About the Author
Josephine Duvall-Young trained in general oph-

thalmology largely at the Princess Alexandra Eye
Pavilion in Edinburgh and took up a fellowship in
experimental eye research studying the mecha-
nisms of macular disease at the University of
Illinois under Professor MOM Tso. She was
appointed consultant ophthalmologist in the NHS
at Walton Hospital, now Aintree Hospitals,
Liverpool, and later at South Buckinghamshire
NHS Trust, developing a special interest in macular
and hereditary eye disease. She is currently a con-
sultant at Arrowe Park Hospital, Wirral University
NHS Trust. When she joined Arrowe Park in 2014,
she recognised the evolution of ophthalmology into
numerous subspecialties with the result that the
general ophthalmologist was a disappearing breed. She saw the need for a consul-
tant to take over the management of emergency and urgent patients and developed
an emergency and acute service within the ophthalmology department. Since then,
emergency and acute service has been recognised by the Royal College of
Ophthalmologists as a subspecialty of ophthalmology. Mrs. Duvall-Young has par-
ticipated in hospital management throughout her career and enjoys sharing her
managerial and clinical experience through teaching. She has been an examiner for
the FRCS(Ophth)Ed and the FRCOphth for over 20 years.
ix
List of Figures
Fig. 2.1 Icare rebound tonometer �� 10

Fig. 3.1 Referral form to rapid assessment TIA and stroke clinic�� 19
Fig. 5.1 Anatomy of the orbital apex �� 61
Fig. 5.2 Anatomy of the cavernous sinus �� 61
Fig. 6.1 OCT optic nerve transverse scan of an 18 year old patient
showing drusen outlined �� 70
Fig. 6.2 OCT of same patient as in Fig 6.1 showing that the nasal nerve
fibre layer thickness is within normal
limits despite the swollen appearance of the disc �� 71
Fig. 6.3 Autofluorescent image of the discs of a 14 year old showing
bilateral but asymmetric drusen�� 71
Fig. 6.4 The same patient as in Fig 6.3 showing drusen (dotted line)
protruding laterally creating a “boot shaped” SHS (solid line)�� 72
Fig. 6.5 OCT in raised ICP showing absence of SHS despite advanced
disc swelling�� 72
Fig. 6.6 OCT of a 37 year old patient with granular appearing drusen,
outlined �� 73
Fig. 6.7 Progressing disc swelling in IIH showing progression
of thickening of NFL particularly nasally between two scans�� 73
Fig. 6.8 Anterior segment OCT showing closeable angle�� 75
Fig. 6.9 Normal visual field�� 77
Fig. 6.10 Diagram of parasympathetic innervation of the pupil�� 81
Fig. 6.11 Diagram of sympathetic innervation of the pupil �� 81
Fig. 6.12 Schematic electrodiagnostic traces �� 85
Fig. 10.1 Screenshot of NICE evidence website home page�� 104
Fig. 10.2 Screenshot of Hospital Premium Collection literature search�� 105
xi
List of Tables
Table 2.1 Birmingham Eye Trauma Terminology (BETT)�� 8

Table 2.2 Ocular Trauma Score (OTS)�� 8
xiii
Chapter 1
Introduction
1.1 What Is the Problem?
In recent decades, ophthalmology has become increasingly superspecialised, with

the result that there is a degree of deskilling of ophthalmologists and a need to redis-
cover the general ophthalmologist, also sometimes referred to as the comprehensive
ophthalmologist. This is particularly a problem in countries where eye care is medi-
cally delivered and hospital based, notably the UK. The problem will however arise
if it has not already done so in other health care systems. This book is written to
address the demands and problems of the UK National Health Service (NHS) deliv-
ery of ophthalmology, but also has a more general target in ophthalmology
worldwide.
There are many excellent textbooks in the field of ophthalmology, taking
either a general or a more specialised approach. This book is not intended to be a
comprehensive referenced text, but more a guide to how to approach the provi-
sion of urgent and emergency eye care within a superspecialised hospital disci-
pline. Only the most significant literature relating to reviews, major trials or
guidelines will be referenced. Some topics will be mentioned briefly as a reminder
and to help when planning the training of ophthalmologists and allied health
professionals.
Referrals direct to hospital ophthalmology in the UK have been growing steadily
and rapidly since about 2006 due to a number of factors. The demographic changes
associated with an increasing population and greater longevity are part of all consid-
erations of health care provision. However, more specifically, at that time, the
change in the NHS contract with optometrists allowed them to refer patients directly
to hospital departments of ophthalmology, rather than to their GP. As optometrists
have acquired more and more technological equipment they are able to detect
abnormalities which they could not previously see using more basic instruments
such as a direct ophthalmoscope. The contract requires them to report any
© Springer Nature Switzerland AG 2019 1

J. Duvall-Young, Emergency, Acute and Rapid Access Ophthalmology,
https://doi.org/10.1007/978-3-319-92369-7_1
2 1 Introduction
a bnormality which could be significant to eyesight or general health, and since the
referral now goes straight to the hospital instead of the GP, the number of referrals
has exploded out of control. In conjunction with this change, lay access to online
information about abnormalities detected gives rise to sometimes unwarranted anxi-
ety. The internet as well as printed and broadcast media additionally feed the cul-
tural shift in health seeking behaviour which is well recognised in a society
encouraged to believe in entitlement to services. Apart from the very significant and
somewhat unanticipated effect of changing the optometrists’ contract, patients
themselves have altered their behaviour, and approximations for the annual increase
in self referred eye emergencies are of the order of 10%. These numbers are unsus-
tainable and the service delivery has to be managed. This is part of the role of the
subspecialty recently recognised by the Royal College of Ophthalmologists, cur-
rently termed emergency ophthalmology but more appropriately termed emergency
and rapid access ophthalmology.
1.2 Defining the Problem
An urgent eye condition is any eye condition that is of recent onset and is distressing
or is believed by the patient, carer or referring health professional to present an
imminent threat to vision or to the general health.
The problem discussed here specifically refers to the NHS UK although parallels
almost certainly exist in other countries. First it is necessary to define the terms used
by commissioners and providers of health care. There is confusion over the use of
these terms depending on the context. Primary care means the care delivered by the
health professional at first point of contact of the patient. That can be a community
pharmacist, a nurse a general medical practitioner, an optometrist or an accident and
emergency doctor. It is only an ophthalmologist in a minority of situations either a
community ophthalmic medical practitioner, or where there is direct walk-in access
to an ophthalmology department, although here the patient is more likely to encoun-
ter a nurse or an optometrist in the first instance. The ophthalmologist then delivers
secondary care if necessary. However many hospital departments have what they
term incorrectly a Primary Care service sometimes run by a consultant ophthal-
mologist but more usually by trainees or sub consultant grade ophthalmologists.
This will now be referred to as an Emergency and Rapid Access Service for the
remainder of this book. The confusion in terminology needs to be ironed out in
commissioning documents and ophthalmologists’ job titles. Whichever terms are
used, this book is targeted at the training of the next cohort of consultant ophthal-
mologists who will be responsible for emergency and rapid access ophthalmology,
although much of the content will be relevant and useful to primary care providers,
be they general practitioners, optometrists, nurses or comprehensive ophthalmolo-
gists in other health systems. Wherever possible the investigation and treatment
plans will be evidence based. Where there is no evidence base, the advice expressed
is the opinion of the author and is open to challenge.
1.3 The Book 3
1.3 The Book
The book is divided into two parts, the first the clinical aspects with emphasis on
common or difficult areas of practice, while the second explores organisation, man-
agement, triage and staff training relevant to providing an emergency and rapid
access service. In the first part, the discussion is largely based on diagnosis although
in practice, referrals are more commonly symptom based. This will be covered in
the second part when considering setting up triaging guidelines. Conditions will be
discussed to a level allowing the ophthalmologist to inform the patient of likely
further steps if referred on to a subspecialty, and to allow knowledgeable communi-
cation with colleagues in other disciplines. Detailed information regarding investi-
gations, treatment regimes and practical procedures are included with the intention
that this will be a useful day to day manual, with practical information sometimes
difficult to find elsewhere.
The emergency and rapid access ophthalmologist has to be a Jack of all trades,
with a good overall knowledge of medicine and ophthalmology. His aims should be
to;
1. Diagnose and treat straightforward ophthalmological conditions in at most two
visits.
2. Make a differential diagnosis and order initial investigations in more complex
conditions, and refer to the appropriate subspecialists, optical services or medi-
cal services.
3. Be available to visit the emergency department and occasionally other hospital
departments to advise on emergency management.
4. Be a point of contact for colleagues in other disciplines within the organisation
in which he is based, and accept internal referrals to assist in the management of
patients across disciplines, or to offer opinions on patients in whom there may be
diagnostic difficulties.
Rapid access ophthalmology can be stressful or exciting, depending on the
approach of each practitioner. The next patient has something new, sometimes triv-
ial and sometimes challenging. The role requires the doctor to recognise what the
patient is presenting with, and to have the knowledge and skill to manage it defini-
tively, or to initiate investigations or treatment before onward referral. He should be
able to give the patient confidence in being on the right pathway, and information
regarding the next steps on the journey. After over 30 years of experience the author
still encounters new presentations in the clinic, but sound basic knowledge helps to
tease out a problem.
Note: Throughout the text, patients and healthcare professionals are referred to
as he, although they could equally well be referred to as she.
Part I
Clinical Aspects
Chapter 2
Emergency Ophthalmology: Trauma
2.1 Terrorist Attack or Major Incident
Events in current times give rise to ever present fears of terrorist attack. Witnessing
or being called upon to respond to bombings in crowded areas are still rare events,
but medical personnel must be aware of the type of injuries to anticipate, how to tri-
age them and how to manage them. Terrorist bomb attacks tend to occur in enclosed
spaces giving rise to a very high incidence of facial injuries (over 50% have head and
neck injuries), while eye injuries affect in the region of 10% of survivors. The inju-
ries fall into three groups-penetrating injury, blast injury and burns. The prognosis
for vision is poorer in injuries caused by bombs than for other causes of eye injury.
Large medical centres have a major accident plan in place. Members of medical
staff whether on or off duty know how to respond and to whom they should report.
A senior member of the emergency department staff takes control and delegates as
many as necessary to triage victims into those with immediately life threatening
conditions and others. CT scanning is usually carried out initially to locate shrapnel
and bony injuries. The role of the ophthalmologist is firstly to examine the eyes of
all unconscious patients with head and neck injuries, and secondly the eyes of all
conscious patients with ophthalmic symptoms. The unconscious patients have to be
traced in the imaging departments, operating theatres and intensive care unit if the
triage process is already under way when he arrives. Any perforated globes should
be protected with an eye shield. Prioritisation of the repair of injuries dictates that
general and neurosurgeons operate first on patients with life threatening injuries,
then ophthalmological surgery follows and lastly orthopaedic and plastic surgery.
Blast injury is the response to the pressure waves generated by the explosion and
can result in intraocular haemorrhage, retinal oedema, globe rupture and orbital frac-
ture. Once material is airborne, the injuries can be anything from abrasion to major
perforation, intraocular foreign body (IOFB) including organic material originating
from other victims, orbital fracture, avulsion of the optic nerve, and lid lacerations.
Depending on the environment there is also a possibility of chemical and thermal burns.

https://doi.org/10.1007/978-3-319-92369-7_2
8 2 Emergency Ophthalmology: Trauma
Eye injuries are classified with standardised terminology according to the

Birmingham Eye Trauma Terminology (BETT). This is based on two features: (1)
whether the globe is open or closed and (2) whether the injury is caused by a sharp
or a blunt object. This does not add much for the ophthalmologist, except to stan-
dardise terminology, but is useful for instructing general emergency departments
(Table 2.1).
There is also a system for scoring ocular trauma, again not especially relevant in
most ophthalmologists’ practice in the UK, but used for large scale surveys of
trauma management and outcomes. It is mentioned here for completeness. The
Ocular Trauma Score (OTS) ranges from 1, the most severe to 5, the best prognosis
(Table 2.2). It can be used to predict prognosis but is only 80% accurate.
Table 2.1 Birmingham Eye Trauma Terminology (BETT)

Closed injury Contusion
Lamellar laceration
Open injury Rupture (blunt injury)
Laceration Penetrating (entry wound)
Intraocular foreign body (IOFB)
Perforating (entry and exit wound)
Blunt injury Rupture
Contusion
Sharp injury Partial thickness
Full thickness Penetrating
IOFB
Perforating
Table 2.2 Ocular Trauma Score (OTS)

Initial factor Points
VA NPL 60
PL to HM 70
CF 80
6/60–6/18 90
6/12 or better 100
Globe rupture −23
Endophthalmitis −17
Perforating injury −14
Retinal detachment −11
RAPD −10
Score _______
Score Grade Predicted outcome at 6 months
0–44 1 73% NPL
45–65 2 28% NPL, 26% PL to HM
66–80 3 28% CF, 44% 6/60–6/18
81–91 4 74% 6/12 or better
92–100 5 92% 6/12 or better
2.3 Retrobulbar Haemorrhage 9
2.2 Globe Penetration and Rupture
Globe penetration occurs with sharp injury while blunt injury causes globe rupture.
Typical blunt injury occurs in a fall against an object such as a door handle or a
piece of furniture and the globe usually ruptures in a circumferential pattern under
the insertions of the rectus muscles where the sclera is thinnest. These patients are
often elderly. Sports in which the projectile is small enough to fit within the orbital
rim, such as squash, golf and badminton also cause serious blunt injury.
Penetration with a sharp object is usually obvious from the history, often using
power tools or a consequence of an assault. The management is to protect the injured
globe from further injury by applying a shield, ascertaining whether there is an
intraocular foreign body, usually by CT scan unless direct visualisation is possible,
and preparing for surgical repair. In the heat of the moment, simple routines may be
forgotten, but it is important to take a history of previous ocular health including
surgery, tetanus immunisation and record the visual acuity of both eyes.
A patient with a ruptured globe or a penetrating injury complains of little pain. If
pain is a feature especially if associated with systemic symptoms including vomit-
ing, and if there is bradycardia, bony injury of the orbit with or without retrobulbar
haemorrhage must be suspected. These symptoms and signs indicate excitation of
the oculocardiac reflex, most commonly in association with orbital fracture. In this
case CT scan of the orbit is indicated.
If a patient presents with subconjunctival haemorrhage with intact conjunctiva,
and a history of a significant blow to the eye, measurement of the intraocular pres-
sure, best done with the least traumatic method available such as the Icare, an atrau-
matic rebound measuring device requiring no topical anaesthesia and very little skill
to operate (Fig. 2.1) will alert the doctor to a scleral rupture. Other signs are vitreous
haemorrhage and shallowing of the anterior chamber. Such injuries require urgent
repair as there is a risk of expulsive haemorrhage. The visual outcome in these inju-
ries however can often be surprisingly good.
The Icare tonometer is an excellent instrument for use in emergency and rapid
access clinics. Ideally the PRO model should be available as this is the only
one which allows readings in the supine position. No local anaesthetic and no
skill are required to use it. NICE has reported on its validity as a method of
measuring intraocular pressure. It is a useful fast screening tool, and within
the normal range it is accurate. If the measurement registers out of the normal
range, it requires to be checked by Goldman applanation tonometry.
2.3 Retrobulbar Haemorrhage
While thinking about haemorrhage, retrobulbar bleeding, either caused by orbital

fracture or a severe impact on the globe should be considered. This is potentially
Fig. 2.1 Icare rebound tonometer
blinding and a true emergency. The signs are diplopia, progressive proptosis, pain and
often vomiting, diminishing vision with red desaturation and loss of pupil responses.
In patients with a tight orbital septum the amount of proptosis may not be very remark-
able and the intraorbital pressure will rise quickly with compression and ischaemia of
the optic nerve. If haemorrhage is suspected the patient requires regular, that is ¼
hourly, observations of visual acuity, red desaturation and pupil responses and if there
is progression of signs, urgent orbital decompression is required. The intraorbital pres-
sure can be indirectly assessed by measuring the intraocular pressure but this is not
normally requested, unless the observations are being taken consistently by the same
ophthalmic nurse. The ideal instrument is one of the Icare type. The decompression
can be done in the emergency department by lateral canthotomy and cantholysis.
Emergency Lateral Canthotomy and Cantholysis

Local anaesthetic is introduced into the lateral canthus, following which the
tissues are crushed with an artery forceps. The crushed tissue is then cut with
a large scissors as far as the orbital margin. This is the canthotomy. Cantholysis
2.5 Lid Injury 11
involves cutting into the lower and if necessary the upper parts of the lateral
canthal ligament by introducing the scissors in the lateral part of the incision
at right angles to the lid margin, feeling the ligament with the scissors and
cutting such that the lower lid is freely mobile from the orbital margin. If
necessary the upper part of the ligament can also be cut. This is not a disfigur-
ing procedure and can be repaired later although it heals remarkably well
without surgery.
2.4 Orbital Fracture
Ophthalmologists are often asked to give an opinion on orbital fractures. Their

role is to check the integrity of the globe and the extraocular muscles. Blow out
fractures affect the floor with entrapment of orbital fat and sometimes the infe-
rior rectus, or the medial wall with sometimes medial rectus entrapment. There
will almost always be diplopia because of the presence of bleeding and swelling
but the maxillofacial surgeon needs to know if there is entrapment of muscle.
There is nothing to be gained by early intervention even if there is muscle entrap-
ment which should be suspected if there is nausea and vomiting, and decisions
can be made with cross consultation over 2 or 3 days if there is thought to be
entrapment. Orthoptic assessment with charting of diplopia is useful in assessing
progress and showing the effect of reducing oedema. Intervention is often unnec-
essary, but if there is late enophthalmos, a delayed procedure may be offered for
cosmesis.
2.5 Lid Injury
A severe blow to the face occasionally presents with a complete ptosis with no other
injury. This is alarming for the patient, and for the doctor if he has not encountered
the circumstance before. The mechanism is contusion to the levator muscle of the
eyelid and complete recovery usually follows within 6 weeks.
Lid lacerations require close apposition of the margin to prevent notching and
long term epiphora and can be treated in the emergency department by an ophthal-
mologist. Lacerations of the medial end of the lid occur in tearing injuries, typi-
cally with dog bites but also catching the lid on an object such as a hook or a
clothes hanger. Here there is damage to the canalicular system. There is contro-
versy as to the best management of these but there is no immediate need to operate
and reconstructive surgery can be planned by a lacrimal or a plastic surgeon either
in the ensuing days or after a delay of weeks. Tetanus toxoid and antibiotics should
not be forgotten. Conjunctival lacerations do not usually require surgery and heal
quickly.
Technique for Repair of Lid Lacerations

1. Check integrity of globe and orbital bones.
2. Give antibiotic and tetanus prophylaxis, particularly if there is a delay
planned for surgery- Augmentin (amoxicillin with clavulanate) for dirty
or bite wounds, Clindamycin if allergic to penicillin.
3. If medial canthus or canalicular tear, consider referral for deferred repair
by plastic surgeon.
4. Decide whether GA or LA appropriate. GA is usually preferable.
5. Skin prep with betadine until tissues bleed.
6. Save all tissue. The eyelid is highly vascular and heals well.
7. Remove any foreign material and irrigate well.
8. Local anaesthetic with 1:100,000 adrenaline may be infiltrated to help
control bleeding, but avoid injecting tissue which looks potentially
devitalised.
9. Repair tarsus with ¾ thickness bites using 5/0 Vicryl or catgut, and burry
knots in tarsus or under muscle. The conjunctiva does not need to be
sutured. At this point the lid margin should be restored with care.
10. Muscle is closed with 5/0 or 6/0 vicryl
11. Skin is closed starting at lid margin. Choice of suture depends on whether
the patient will return for suture removal. Cosmesis is better if the sutures
are removed (7/0 Nylon), but in children it may be preferable to use 7/0
vicryl. Closure starts at the lid margin, at the line of the Meibomian
glands with a mattress suture tying the knot to lie external to the lashes.
12. Removal of sutures should be planned for 6 days. If there is tension on the
lid margin or concern about healing, the lid margin suture should be left
for 14 days.
2.6 Chemical and Thermal Burns
Chemical injury can cause very severe scarring of the conjunctiva and cornea and
the causative agent must be identified. Alkali burns are more devastating because
alkali saponifies cell membranes allowing the chemical to penetrate more deeply
into the tissues, while acid coagulates protein creating a barrier to deeper penetra-
tion. The emergency treatment is copious irrigation, paying attention to the fornices
and checking the pH of the tears. The Morgan lens is a commercially available
irrigating system which is more comfortable for the patient and more effective, but
an intravenous giving set and a bag of saline is also adequate. Once irrigated, the
area of the burn is assessed by fluorescein staining and by recording the area of
blanching of the limbus and conjunctiva. The severity and prognosis relate to the
number of clock hours of limbal involvement. The immediate management is to
apply antibiotic ointment and cycloplegic drops with systemic analgesia. The intra-
ocular pressure often rises in alkali burns and needs appropriate management.
2.7 Contusion 13
Preservative free lubricants and steroid drops are also indicated initially, with
onward referral to a corneal clinic for further management which may include con-
junctival, amniotic membrane, stem cell or corneal grafting. Burns are complicated
by the amount of damage there may be also to the eyelids interfering with the pro-
duction and flow of tears and requiring complex long term management by a corneal
specialist in conjunction with a plastic surgeon.
Thermal burns are managed similarly.
2.7 Contusion
Severe contusional injury to the eye or the head can cause optic nerve damage or
even avulsion, commotio retinae, choroidal rupture and intraocular bleeding with or
without retinal detachment.
In optic nerve injury there may be no apparent injury to the eye other than a pupil
defect, and only later may the extent of the injury manifest itself. High doses of
steroids have been advocated in the management of these injuries on the basis of a
response observed in experimental spinal cord lesions, but there is no objective
evidence of a benefit in optic nerve injury. On the contrary, there is evidence of an
adverse effect of high dose steroids in head trauma, in that there is an increased
mortality rate, measurable but as yet unexplained, at 6 months after head injury
treated with high dose steroids. The treatment of optic nerve injury with high dose
steroids remains controversial. If the nerve is avulsed, clearly there is no dispute
about the prognosis, but if contused there is an unpredictable recovery over days to
weeks.
Commotio retinae represents bruising of the retina. The retina becomes opaque
as glial cells swell, possibly accompanied by some neuronal disruption. There can
be profound visual loss but usually good recovery within a month.
Traumatic choroidal rupture, recognised as a curvilinear defect in the retinal pig-
ment epithelium (RPE) usually concentric with the optic disc, is not usually associ-
ated with choroidal neovascularisation because a healthy retinal pigment epithelium
has an inhibitory effect on vascular endothelial cell proliferation. If it does occur, it
is managed in a similar way to neovascular membranes in age-related disease but
carries a better prognosis in that the resulting scar is limited in size. However if the
choroidal rupture involves the macular area there may be some long term visual
consequences.
Intraocular bleeding as a consequence of blunt trauma may be anterior or poste-
rior. Trivial hyphaema without any other evident significant damage should be
treated in the same way as a severe hyphaema. This is because of the risk of rebleed-
ing. The aqueous has an anticoagulant property, preventing the initial bleed from
clotting in the eye. The blood will be cleared from the eye through aqueous drainage
routes. The bleeding vessel in the iris is initially plugged by fibrin which takes
5 days to mature into a secure clot. If the patient is physically active during those
5 days, the fibrin clot may be dislodged and a secondary bleed will occur. This bleed
is more likely to clot in the anterior chamber as the initial bleed has exhausted the
anticoagulant effect of the aqueous, and will fail to clear. If as is usually the case,
this is associated with a rise in intraocular pressure, there is a risk of corneal blood
staining, as well as uncontrollable intraocular pressure, and surgical removal of the
clot may be required. For these reasons it is advisable not to dilate the pupil in a
patient with a hyphaema, and to advise them to rest for 5 days with minimal activity.
At review after 5 days if, as is usually the case, it has resolved, the intraocular pres-
sure is measured, gonioscopy is performed to diagnose the presence of angle reces-
sion, which if present indicates the need for follow-up. Angle recession indicates a
risk of secondary glaucoma, not directly because of the altered anatomy of the
drainage angle, but because recession is a measure of the severity of the injury. Such
patients should be followed up until the age of 40 when glaucoma screening is pro-
vided by the general optometric service in the community. At the 5 day visit the
pupil is dilated to fully assess the posterior segment for retinal tears. If the hypha-
ema has increased at 5 day review, there should be consideration of surgical inter-
vention with vitrectomy instrumentation.
Bleeding in the posterior segment, that is vitreous haemorrhage, may indicate
significant retinal damage. Pupil responses and light projection response are helpful
in establishing the extent of injury. B ultrasound scanning is indicated to look for
retinal detachment. If vitreous haemorrhage appears to be an isolated finding, the
blood can be expected to clear rapidly in a healthy eye as compared to a diabetic
eye. Review after a week of physical inactivity is adequate to perform a complete
posterior segment examination looking for retinal injury.
2.8 Minor Injuries
Finally and for the sake of completeness, more trivial trauma is included in this
discussion. Management of this type of injury would ideally be delegated to non-
medically trained team members. Detail given here may be helpful when preparing
training for non-medical staff. Trivial injuries relate to foreign bodies which can be
embedded in the cornea and require to be removed using short-acting anaesthetic
drops and a needle or a battery operated burr which is ideal for removing rust rings
at the initial treatment. If a burr is not available the patient may have to return in
3–5 days for the removal of the rust ring which by that time will have consolidated
and will be relatively easy to pick off. Subtarsal foreign bodies may be missed, but
the tell-tale superficial linear vertical scratches in the superior cornea which can be
highlighted by fluorescein staining make these easier to identify. This is particu-
larly the case if sand is blown into the eye, as the grains are transparent, but fluo-
rescein pools round the particle making it detectable, usually on the tarsal
conjunctiva close to the lid margin. After the removal the patient should use an
antibiotic ointment for a day until the adjacent epithelial cells slide over to seal the
defect.
2.9 Local Anaesthesia 15
Larger abrasions can be a consequence of a foreign body but also are commonly
associated with a scratch from a child’s fingernail catching its mother’s eye as it
reaches towards her face, or occasionally with clumsy handling of contact lenses.
These should also be treated with antibiotic ointment until the epithelial surface is
sealed. There is some controversy as to whether a pad on the eye is advisable. In a
large abrasion the pain is quite intense and a pad may give some relief. Mydriatics
may also be prescribed in this circumstance but local anaesthetic drops should not
be offered as these reduce the rate of epithelial healing. Non steroidal anti-
inflammatory drops such as diclofenac may be helpful.
The aim of treatment of abrasions is not only to relieve the symptoms of the
injury, but if possible to reduce the risk of future problems of recurrent erosion. In
this context, there may be a role for a bandage contact lens or for one of the newly
developing soluble collagen contact lenses. These are however expensive and most
dissolve too rapidly to be useful. This is a therapeutic area which may see further
development.
Recurrent erosion is a common problem causing significant morbidity and its
management has been the subject of a Cochrane review, but with no consensus on
the best treatment. Debridement of loose epithelium using a burr may be helpful.
Lubricating gel at night is also advised for a month after an abrasion, while the
epithelium is restored to its normal thickness, in an attempt to reduce the risk of
recurrence.
Cyanoacrylate glue is a relatively frequent cause of eye injury. The size of the
tube or bottle is similar to the size of a tube of eye ointment or a bottle of eyedrops
and the glue can be inadvertently put in the eye. The blink reflex usually prevents
contact with the cornea but the lids may be firmly adherent to each other. The glue
can sometimes be picked off the lashes but if a significant amount is applied it is
necessary to cut the lashes to allow the lids to open. There may be a corneal abrasion
which is treated in the usual way and the lashes will be restored in about 6 weeks.
Corneal flash burns are associated with the use of welding arc lights and are due
to the effect of ultraviolet light on the cornea. This same pathogenesis is implicated
in snow blindness, in the keratitis suffered by users of tanning lamps, and more
recently with the use of halogen desk lights. The keratitis is usually minor with
punctate staining with fluorescein, but can be severe with total loss of the corneal
epithelium. Fortunately it heals quickly with no residual damage. Whatever the
causative mechanism, the symptoms occur after a delay following exposure of
6–12 h. No treatment is required except analgesia with topical non steroidals and
mydriatics in severe cases.
2.9 Local Anaesthesia
In extensive injuries being repaired under local anaesthesia, toxicity of the agents
must be borne in mind.
Local Anaesthetics
Topical
Oxybuprocaine (benoxinate)
Proxymetacaine
Amethocaine
All have similar time to onset around 30 s, maximum effect around 5 min
and wear off at around 30 min. Proxymetacaine is the best tolerated by the
patient.
Injectable
Lignocaine
Marcaine
These are CNS suppressants and cause cardiac arrhythmias.
Inadvertent overdose may occur if injected intravenously in error, or if
multiple injuries repaired by local anaesthetic. The maximum dose of ligno-
caine for an adult of average build is 300 mg, or 500 mg if mixed with adrena-
line. 30 ml of 1% lignocaine or 15 ml of 2% contains 300 mg.
Maximum dose of marcaine is 175 mg or 35 ml of 0.5%.
Doses are adjusted for weight of patient.
Toxicity is greater at extremes of age.
Further Reading
Barak A, Verssano D, Halpern P, Lowenstein A. Ophthalmologists, suicide bombings and get-
ting it right in the emergency department. Graefe’s Arch Clin Exp Ophthalmol. 2008;246:
199–203.
Icare rebound tonometry to measure intraocular pressure. Medtech innovation briefing (MIB57),
2016.
Kuhn F, Morris R, Witherspoon CD, Mester V. The Birmingham eye trauma terminology system
(BETT). J Fr Ophthalmol. 2004;27:206–10.
Kuhn F, Morris R, Mester V, Witherspoon CD, Mann L. Predicting the severity of an eye injury: the
ocular trauma score (OTS) in Ocular Trauma. Berlin: Springer; 2008. p. 17–22.
Lim CHL, Turner A, Lim BX. Patching for corneal abrasion. Cochrane Database Syst Rev
2016;(2):CD004764.
Chapter 3
True Ophthalmic Emergencies:
Non Traumatic
There are very few circumstances in ophthalmology in which immediate action can
make a difference to the outcome of a condition. Two of these have already been
discussed in the section on trauma, namely chemical burns and retrobulbar haemor-
rhage. The others are retinal artery occlusion and endophthalmitis. Patients do not
however present with a diagnostic label and triage based on symptomatology and
presentation is key to dealing with emergencies appropriately. Triage will be dis-
cussed in part II of this book. However, in this section the topics will be discussed
on the basis of diagnosis to facilitate ease of reference.
3.1 Central Retinal Artery Occlusion
The presenting symptom here is sudden and profound loss of vision. Establishing
the duration of the visual loss is most important and not as straight forward as it
appears. Frequently, particularly in the elderly, the patient may become aware of a
blind eye suddenly, when inadvertently covering the fellow eye, when in fact the
visual loss is of much longer standing. If indeed there is sufficient suspicion that the
visual loss has occurred within hours, vigorous attempts to restore vision may be
worthwhile. In experiments on primates, the ischaemic tolerance of the retina has
been shown to be 2 hours, but in clinical settings visual improvement has been
reported up to 48 hours after the onset of the symptom. It is reasonable to consider
a cutoff point for useful intervention at 24 hours.
The physical signs of arterial occlusion are well recognised as an afferent pupil-
lary defect, whitening of the retina at the posterior pole where it is thickest and a
cherry red spot at the fovea. There may be breaking up of the blood column in the
retinal arteries and veins, sometimes referred to as box-carring. In about 25% of
people there is a separate cilioretinal artery supplying the macula, and the site of the
obstruction in these people will dictate the physical signs and outcome, but the
pathophysiology is the same. The white appearance of the retina may persist for

https://doi.org/10.1007/978-3-319-92369-7_3
18 3 True Ophthalmic Emergencies: Non Traumatic
some weeks after the event, but often the fundus looks remarkably normal, some-
times with discernable arteriolar narrowing and much later, optic atrophy.
If the obstruction is transient, the presentation is termed amaurosis fugax.
Whether transient or permanent, the cause is divided into the majority which are
non-arteritic, while about 5% are caused by an arteritic process, most notably giant
cell arteritis (GCA) but also systemic lupus erythematosis (SLE), polyarteritis
nodosa (PAN)and granulomatosis with polyangiitis (GPA, formerly Wegener’s
granulomatosis).
In non-arteritic cases the cause is embolic, from the heart or the carotid arteries,
usually in arteriopaths. In younger patients it is necessary to look further for heart
disease or hypercoagulability disorders. A patent foramen ovale which is present in
25% of the population allows thrombus from the venous circulation to reach the
arterial side is now well recognized as a cause of paradoxical embolic events.
Attempts to dilate retinal vessels using sublingual glyceryl trinitrate or isosor-
bide dinitrate, and rebreathing of CO2 are advised but with no supportive evidence.
It is possible to dislodge an embolus and move it more peripherally in the circula-
tion using firm massage with some success. To be effective, the massage has to be
firm enough to be uncomfortable, in repeated episodes of 15 seconds. This manage-
ment should be combined with measures to reduce intraocular pressure, such as
acetazolamide intravenously, mannitol intravenously and anterior chamber paracen-
tesis. Systemic thrombolytic treatment has been attempted but no trials showing it
to be effective have been published. A multicentre trial (EAGLE European assess-
ment group for lysis in the eye study, not to be confused with EAGLE effectiveness
in angle closure glaucoma of lens extraction study) published in 2010 did not show
any benefit from locally injected thrombolytic agents into the ophthalmic artery. It
may, however, be worth discussing thrombolytic treatment with the acute stroke
team if the patient presents within 6 hours, bearing in mind that the treatment carries
a risk of haemorrhagic stroke.
The risk of stroke is raised after a vascular occlusive event in the eye, most in the
first week and about 10 times higher than those people of the same age without
ocular events. All these patients should be reviewed by the stroke team or a physi-
cian as an arterial occlusion is a localised stroke and appropriate investigation and
discussion of ongoing prophylactic treatment and lifestyle changes fall into the
realm of physicians. The patient must understand that restoration of vision is not
possible and that further investigations and treatment are directed to prevent further
vascular events, be they strokes or heart attacks. The prognosis for visual improve-
ment after the initial event is very poor. An artery will recanalise in 48–72 hours but
after that period visual improvement is not expected.
History taking includes a detailed history of the presenting event, a detailed past
history and in younger patients a family history of cardiovascular disease, a smok-
ing history, and a drug history particularly contraceptives and including illegal
drugs (talc embolus). Always bear in mind the possibility of carotid artery dissec-
tion, although pain is a characteristic feature of this and is not expected in embolic
arterial occlusion. Investigation in the ophthalmology department for patients over
50 or known to be arteriopaths are limited to measuring blood pressure, testing for
3.1 Central Retinal Artery Occlusion 19
glycosuria and measuring ESR and CRP. They should then be referred to the stroke
team according to a predetermined pathway depending on risk. A proforma of the
type shown in Fig. 3.1 is useful. In younger patients, investigations for vasculitis or
PATIENT DETAILS DATE AND TIME

NAME OF REFERRING DOCTOR DEPARTMENT
PRESENTING SYMPTOMS Date and time of onset

Yes no Duration
Hemisensory disturbance
Speech disturbance/dysphasia
Visual loss one eye
Hemiparesis/limb weakness
Visual loss homonomous hemifield
Vertigo/cerebellar signs
Persistent symptoms
BRIEF HISTORY
RISK FACTORS
Raised bp Y/N AF Y/N IHD/Angina Y/N Drug history
raised cholesterol Y/N periph vasc
disease Y/N
Previous CVA/TIA Y/N Details
bp pulse Regular Y/N
ABCD2 Score SCORE POINTS

A=age >60 1
B=bp >140 systolic 1
and/or>90 diastolic
C=clinical features Unilateral weakness 2
Speech disturbance 1
Other 0
D=duration >60 minutes 2
10-59 minutes 1
<10 minutes 0
D =diabetes diabetic 1
TOTAL ABCD2 SCORE
If presentation within 4 hours of onset, refer to AED, as may be suitable for thrombolysis
If ABCD2 score 4 or more or more than 1 TIA in a week –HIGH RISK. IMMEDIATE REFERRAL
Score 2 or 3, e-mail referral. Patient will be seen in (check)
All patients on Warfarin with focal neurology require urgent CT scan-contact Stroke Coordinator
Fig. 3.1 Referral form to rapid assessment TIA and stroke clinic
Vascular Disease Investigations

FBC
ESR, CRP
Glucose, cholesterol, triglycerides, lipid panel
Hypercoagulation—Factor V Leiden, homocysteine, fibrinogen, antiphospholipid
antibodies, prothrombin time
Carotid ultrasound
hypercoagulability may be started or if being seen immediately by a physician,

deferred depending on local relationships. Iris rubeosis occurs in 20% of cases and
review at 6 weeks to look for this complication is appropriate. Disc neovascularisa-
tion is rare.
Arteritic arterial occlusion. The majority of cases of arteritic vascular occlu-
sion are due to giant cell arteritis (GCA), a vasculitis affecting the extracranial
branches of the carotid artery in people over 50, and also other medium and large
sized arteries throughout the body. However, GCA most commonly causes visual
loss by affecting the posterior ciliary arteries and causing ischaemic optic neuropa-
thy. An ESR or CRP or both should be measured in cases of central artery occlusion,
and if raised, the diagnosis of GCA should be explored further. The investigation
and management of GCA will be considered in more detail in chapter 4.
3.2 Endophthalmitis
Exogenous endophthalmitis, resulting from an infective organism being intro-

duced either by trauma or by surgical intervention is much commoner than endog-
enous which results from blood borne infection. The incidence of infective
endophthalmitis post surgery is around 0.1% of surgical cases and 0.05% of intra-
vitreal injections. Most often infection presents 3–5 days after the intervention with
increasing pain, reducing vision, redness and photophobia. However bacteria of
lower pathogenicity such as propionibacterium acnes, a skin commensal, can pro-
duce a more chronic inflammation. Fungal infection is also slower to develop, usu-
ally following injury with soil contamination. In post operative cataract cases, an
unexpectedly severe inflammatory response is suspected to be infective, but it can
be due to a reaction to a retained fragment of lens matter which may not be readily
visible except on gonioscopy.
The severity of the physical signs varies with the duration of symptoms but there
may be lid swelling, conjunctival injection, hypopyon, vitritis and corneal oedema.
Retinal phlebitis is said to be the earliest sign although presentation is very rare at
that point. The organism is usually Gram +ve in post operative cases, usually an
organism of low virulence such as coagulase negative staphylococcus, but may be
Gram -ve following non-surgical trauma. The importance of a rapid response cannot
3.2 Endophthalmitis 21
be overemphasised. The rate of development of the inflammatory reaction and the

deterioration of vision can be very rapid. At the earliest opportunity, an intravitreal
injection of antibiotic should be given, with or without vitrectomy and vitreous
sampling for microbiology. The Endophthalmitis Vitrectomy study (EVS) of 1995
is still quoted as guidance for management, although almost certainly outdated. It
concluded that there was a significant improvement in vision after vitrectomy, only
in eyes in which the vision had dropped to perception of light only, and that intrave-
nous and oral antibiotics are of no value because of poor penetration. The main
changes which have occurred since this study are that in the EVS amikacin, an
aminoglycoside, was used and has now been largely replaced by newer antibiotics,
principally ceftazadime a cephalosporin, which do not have such toxic effects on the
retina. Oral antibiotics still have very little role except possibly in low grade or
chronic cases when moxifloxacin and other newer antibiotics which penetrate the
blood eye barrier more effectively may be prescribed. Current opinion suggests that
vitrectomy should be undertaken much earlier in acute infective endophthalmitis,
and probably at the very earliest opportunity. Although there is no evidence support-
ing the use of oral, intravenous or topical antibiotic with the intravitreal dose, the
standard practice is variable but does include all these other routes. Until this topic
is submitted to another rigorous trial, this continues to be the case. The same applies
to topical and oral steroids.
The availability of a vitreoretinal surgeon varies in different localities. If there is
no surgeon available immediately, the vitreous biopsy should be taken and intravit-
real antibiotic should be given without delay. A clean room within the eye depart-
ment should be available for this procedure.
Technique for Vitreous Biopsy

Topical anaesthesia with drops
Periocular skin prep with povidone iodine
Eye prep with 5% aqueous povidone iodine or chlorhexidine 0.05% if allergy
to iodine
Sterile drape and speculum
Sub tenons injection of 2% lignocaine
Measure using a caliper 3.5 mm from limbus
Insert 23G needle on an insulin syringe through the conjunctiva, aiming for
centre of the globe to a depth of 5 mm
Aspirate vitreous, cap syringe and send immediately to microbiology
Inject antibiotic- vancomycin 1 mg in 0.1 ml and ceftazidime 2 mg in 0.1 ml
Prescribe G ofloxacin and cefuroxime hourly, and G dexamethasone 2 hourly
Prescribe ciprofloxacin 750 mg bd for up to 1 week
A delayed chronic form of postoperative endophthalmitis is recognised. This can

present months or even years after the surgery and is due to propionibacterium
acnes slowly proliferating in the eye, sometimes sequestered under a flap of capsule.
This presents with a red eye, sometimes with hypopyon and clumps of exudates on
the iris and pupil margin, corneal oedema and vitritis. Initially this may appear ster-
ile and steroid responsive, but when it fails to respond, an aqueous and vitreous
sample should be taken for microbiology, and if proven managed by vitrectomy
with removal of the capsule and intraocular lens.
Blebitis is an infection which occurs months or years following glaucoma sur-
gery. There is a short history of pain, redness and reduced vision and the area of the
bleb looks inflamed sometimes with pus visible in the bleb. This should be treated
intensively with topical and systemic antibiotic, and watched carefully as it can
progress to endophthalmitis.
Endogenous endophthalmitis is much rarer (less than 10% of all endophthal-
mitis cases). It occurs in patients who are immunocompromised such as the severely
debilitated, HIV AIDS patients, or in patients with an infective site such as those
with indwelling devices, or as a consequence of bacteraemia such as in intravenous
drug users, patients with diverticulitis, chronic urinary tract infection or endocardi-
tis. Blood cultures should be taken and if there are any meningeal signs, also a spi-
nal tap for culture. Many of these patients are extremely debilitated but in some
vitrectomy may improve the prognosis and also reduce the risk of phthisis bulbi.
The mortality rate for patients with endogenous endophthalmitis is high, particu-
larly if other infected sites are identified, or if the infecting agent is fungal.
Candida endophthalmitis occurs as a result of soil contamination in trauma, but
is more often endogenous in intravenous drug abusers and after abdominal surgery.
It is not associated with AIDS and tends to be low grade with less discomfort than
would be expected from the severity of the signs. There may be fluffy lesions in the
vitreous and retina and it may be bilateral. Candida infection generally has a better
prognosis for vision than Aspergillus, although both are poor.
Toxic anterior segment syndrome is a form of postoperative inflammation
which is sterile and is probably a response to irrigation fluids, residues on instru-
ments or intraocular penetration of drugs. Its differentiating features from infection
are that it comes on earlier, within 24 h of surgery and is relatively painless. The
condition is characterized by corneal oedema extending to the limbus, the intraocu-
lar pressure is elevated, the vitreous is clear, and the response to steroids is
dramatic.
Differential Diagnosis of Red Eye Post Operatively

Raised intraocular pressure secondary to inflammation or angle closure
Retained lens matter
Long complex procedure with increased inflammatory response
Toxic anterior segment syndrome
Infective endophthalmitis
Further Reading 23
Treatment of Exogenous Endophthalmitis

Intravitreal antibiotic should be administered, with or without vitrectomy;
vancomycin 1 mg covers the G +ve organisms, and ceftazadine 2 mg cov-
ers the G -ve organisms
Dexamethasone injected intravitreally with the antibiotic may be considered
although a Cochrane review found insufficient evidence to support steroid
use. However steroids do not appear to have any adverse effect on the
outcome
Cycloplegia
Treatment of Endogenous Endophthalmitis

Intravitreal antibiotics with or without vitrectomy as for exogenous
Systemic antibiotic for at least 3–4 weeks
Treatment of Fungal Endophthalmitis

Intravitreal voriconazole 100 μg in 0.1 ml or amphotericin B 10 μg in 0.1 ml,
with or without vitrectomy
Intravenous amphotericin B or oral voriconazole or fluconazole
Avoid steroids
Further Reading
Endophthalmitis Study Group. Results of the endophthalmitis vitrectomy study. A randomized

trial of immediate vitrectomy and of intravenous antibiotics for the treatment of postoperative
bacterial endophthalmitis. Arch Ophthalmol. 1995;113:1479–86.
Kim CH, Chen MF, Coleman AL. Adjunctive steroid therapy versus antibiotics alone for acute
endophthalmitis after intraocular procedure. Cochrane Database Syst Rev. 2017; https://doi.
org/10.1002/14651858.CD012131.pub2.
Schumacher M, Schmidt D, Jurklies B, Gall C, Wanke I, Schmoor C, et al. Central retinal artery
occlusion: local intra-arterial fibrinolysis versus conservative treatment, a multicenter random-
ized trial. (EAGLE study). Ophthalmology. 2010;117:1367–75.
Chapter 4
Urgent Eye Conditions
4.1 Acute Angle Closure Glaucoma
Full blown primary acute angle closure glaucoma is easy to recognise. The patient
presents with a red eye, reduced vision with haloes, severe pain and a middilated
fixed pupil. Problems however can arise if the systemic symptoms associated with
the rising pressure overwhelm the local symptoms and the patient is directed to the
wrong department. Such patients may be found by the ophthalmologist as the vom-
iting patient in gastroenterology, the severe headache patient in neurosurgery or the
confused photophobic patient in neurology, all with an apparently incidental red
eye. The outcome is dependent on the duration of the raised pressure before it is
controlled, rather than on the level of intraocular pressure recorded at the time of
diagnosis and for that reason prompt action is necessary for a good visual
outcome.
Typically the condition presents in the over 60s, but can present earlier in patients
who are significantly hypermetropic, with or without plateau iris. Anterior segment
OCT is useful to show the anatomical configuration of the angle and iris. The onset
of symptoms is classically in the evening, in winter, with haloes round lights and
some discomfort which settles down when the patient goes to sleep. After a few
episodes like this, the acute episode sets in which is not self limiting and brings the
patient to seek medical advice. If the patient is not in the expected age group and is
not hypermetropic, and does not have a closeable angle in the fellow eye on gonios-
copy, the diagnosis should be reconsidered. Other possibilities are rubeotic glau-
coma, ciliary block glaucoma or an intraocular tumour. After measuring the baseline
intraocular pressure (IOP), and if possible a gonioscopy, the patient is given 500 mg
of acetazolamide injected slowly intravitreously and 500 mg orally if tolerated. The
acetazolamide reduces aqueous production and is maximally effective at 4 hours.
Pilocarpine 2% is given topically as a single dose. Constriction of the pupil will
release some of the congestion in the drainage angle but if the iris is significantly
ischaemic, it will not work and may even aggravate the degree of pupil block. Lying

https://doi.org/10.1007/978-3-319-92369-7_4
26 4 Urgent Eye Conditions
the patient down will not only be welcomed by the unwell patient, but may help the
lens-iris diaphragm to drop posteriorly as the pressure drops, helping to break the
attack. The patient should be reassessed after an hour. The Icare tonometer is ideal
for non-traumatic repeated IOP meassurements. If there is a response in the pressure
and the pupil constricts, then the attack is under control.
The recommendation (Choong et al.) is to expect a 25% reduction in initial IOP,
or a pressure of less than 35 mmHg at 30 minutes. If this is not achieved, topical
pilocarpine 2% may be repeated. If after an hour the IOP has not dropped osmotic
agents are indicated. These act by shrinking the vitreous. Either oral 50% glycerol
1 g/kg (except in diabetics or if not tolerated) or intravenous 20% mannitol are pre-
scribed (2.5 ml/kg, an average of 150–200 ml, given over 30 min). Note that man-
nitol is contraindicated in renal failure, and should be used with caution in heart
failure but does not affect diabetic control.
Once the pressure is controlled, gonioscopy should be repeated, or done for the
first time if not possible initially, and laser peripheral iridotomy (PI) should be car-
ried out in the next 24 hours in the fellow eye and within 48 hours in the affected eye
depending on the clarity of the cornea. Note that if significant pressure is exerted on
the eye with a gonioscope, the angle may appear open when it is functionally closed
or closeable. Raised IOP due to plateau iris may or may not respond to PI alone. If
it does not, referral to a glaucoma surgeon for further laser or intraocular surgical
procedure, probably lens extraction, is necessary. Anterior segment OCT is the best
method for assessing the anatomical configuration.
If the IOP has not come down with treatment, there may be a very large lens or
subluxed lens causing pupil block, or ciliary block. Ciliary block occurs when aque-
ous is misdirected posteriorly with anterior displacement of the lens and iris. It
occurs after intraocular surgery, usually in eyes with a previously narrow angle. It is
treated with intensive dilatation with atropine 1% and phenylephrine 10%.
Pilocarpine must be avoided. Otherwise the same measures as used for acute angle
closure are aimed at reducing pressure, but if they fail, the anterior vitreous face
needs to be disrupted either by YAG laser or surgically.
Adequate IOP should be achieved within 3 hours and the patient should be sent
home on steroid drops.
If the IOP has dropped but remains higher than optimal, treatment with a beta
blocker (timolol) or an alpha agonist (brimonidine or aproclonidine) may be neces-
sary. If the IOP is not controlled, an urgent review in the glaucoma clinic should be
arranged and the patient kept on acetazolamide until a surgical solution can be orga-
nized. The patient should be warned of the side effects of acetazolamide, being a
metallic taste and tingling in the periphery. These wear off if the patient continues
the medication.
The majority of patients recover completely especially if treated early, but about
1/3 require ongoing topical medication to control IOP.
It is worth noting that pupil dilatation for medical examination carries an
extremely low risk of angle closure glaucoma. Tropicamide is universally consid-
ered safe. There are only 2 cases in the literature of ACG induced by tropicamide
and the risk of provoking ACG even in patients with a shallow anterior chamber is
less than 1%.
4.1 Acute Angle Closure Glaucoma 27
Protocol for Management of Acute Angle Closure

Record VA, baseline IOP and if possible gonioscopic findings.
Give a single drop of pilocarpine 2% to affected eye and 1% to fellow eye.
Set up iv access and give 500 mg iv acetazolamide and 500 mg oral
acetazolamide.
Check IOP at 30 minutes and repeat pilocarpine 2% if IOP not fallen by 25%.
Check IOP at 1 hour and if not fallen by 25% from presentation, give
osmotic agent.
Oral glycerol 1.5 g/kg or
Iv mannitol 2.5 ml/kg of 20% over 30 minutes
Review at 3 hours or earlier if responding well.
Start G prednisolone qid to affected eye.
Laser PI fellow eye when tolerated (within 24 h).
Laser PI affected eye when cornea adequately clear (within 48 h).
IOP should be controlled within 3 hours of start of treatment.
Send home on steroids qid and any necessary IOP controlling medication
(ideally none).
Acetazolamide 250 mg qid
G timolol 0.25% bd.
Brimonidine or aproclonidine.
Avoid prostaglandin analogues e.g. latanoprost.
Technique of YAG PI
The IOP is checked pretreatment. Ideally the eye is pretreated with topical steroid.
Local anaesthetic and pilocarpine 2% are instilled. An iridotomy contact lens is
used to focus the laser at the level of the iris and reduce the risk of corneal epithe-
lial laser damage. The position of the iridotomy is in the peripheral 1/3 of the iris,
but there is disagreement as to the best site to avoid optical side effects later. It is
reported that iridotomies at 3 and 9 o’clock cause less optical aberrations but the
laser treatment is more painful than at 11 to 1 o’clock. In blue eyes a crypt is cho-
sen. The spot size is 200 μ, power 4–8 mJ with 1–3 pulses or bursts. In dark eyes
a crypt may not be identifiable, and it may be necessary to thin the treatment area
of the iris with argon laser 50 μ spot size 900 mW, duration 0.03 sec. The YAG is
then fired at the centre of the thinned area at a lower power of 2–3 mJ. Success is
recorded when pigment is released and aqueous is seen flowing through the iri-
dotomy. This is important to record as subsequently it may not be possible to be
sure if a small iridotomy is patent or not. Although there may be transillumination,
there can still be a membrane of tissue bridging the defect in the pigment.
Aproclonidine is administered postoperatively and steroids are prescribed
for the next few days. The postoperative IOP is recorded. The patient will then
be followed up in a glaucoma service.
Icare NICE Medtech Innovation Briefing

A Medtech report has been published by NICE on the use of Icare rebound
tonometry.
It concludes that;
Normal readings are reliable
High readings should be checked with Goldman
It is very good for measuring IOP in children
Accuracy is questionable in very high readings.
4.2 Secondary Glaucomas
Some secondary glaucomas present acutely and need to be differentiated from pri-
mary angle closure. These are:
Neovascular (Rubeotic) glaucoma.
Lens induced
Phacomorphic
Phacolytic
Phacoantigenic (formerly phacoanaphylactic)
Ghost cell
Neovascular glaucoma may be detected and treated at an early stage in the
medical retinal service but if it goes unrecognised it may present with acute symp-
toms similar to those of acute angle closure but without reduction in visual acuity
since the vision has almost always deteriorated severely prior to the onset of acute
symptoms. Although typically this secondary glaucoma presents around 3 months
after a central retinal vein occlusion, it can present at any time, and is also associ-
ated with branch retinal vein occlusion, diabetic retinopathy and ocular ischaemic
syndrome. It has been reported following arterial occlusion, but is rare.
The emergency management is to control the pain with topical steroids and
cycloplegia. The level of the intraocular pressure rise is only relevant if there is
residual vision to preserve, but often at the time of presentation the eye is already
blind. Oral acetazolamide is the most effective means of reducing the intraocular
pressure, but beta blockers such as timolol, alpha agonists such as brimonidine and
aproclonidine and topical carbonic anhydrase inhibitors such as dorzolamide may
be prescribed. Prostaglandin analogues should be avoided. Patients with visual
potential in the affected eye should be referred on to the retinal service for pan reti-
nal photocoagulation if appropriate, and possibly for anti-VEGF injections. Pressure
uncontrolled medically in a potentially seeing eye or causing pain in a blind eye is
an indication for surgery, either drainage or a cyclodestructive procedure.
Ocular ischaemic syndrome. This occurs if there is severe compromise of ocu-
lar blood supply through disease of the internal carotid. It can also be a consequence
of GCA. The patient presents with sudden or gradual reduction in vision, with or
without dull pain. The signs are advanced cataract, rubeosis iridis, cells in the
4.2 Secondary Glaucomas 29
anterior chamber, reduced IOP unless there is rubeosis involving the angle, dot and
blot haemorrhages and cotton wool spots in the retina and neovascularisation of the
optic disc. The ESR and CRP should be measured to exclude GCA, and referral to
the vascular service for investigation of the carotid circulation is appropriate. The
diagnosis of ocular ischaemic syndrome carries a poor prognosis for life, with a
high incidence of stroke and heart disease.
The lens induced secondary glaucomas relate to the morphology of the lens or
the amount of anterior chamber activity. Phakomorphic glaucoma is an angle clo-
sure glaucoma, whereas the others occur in open angled eyes. After first controlling
intraocular pressure and the inflammatory response as much as possible, urgent
referral on for cataract surgery is required. Phakolytic glaucoma presents as a uve-
itis and is caused by leakage of lens matter through the capsule of a mature cataract.
Phakoantigenic (formerly known as phakoanaphylaxis) is a severe inflammatory
response to leaking lens material occurring up to 14 days following nonsurgical or
surgical trauma and characterised by keratic precipitates.
Ghost cell glaucoma can also present acutely. The cause is degenerate blood cells
from intraocular haemorrhage obstructing the trabecular meshwork. The raised pressure
is managed medically and the IOP returns to normal once the haemorrhage has resolved.
A digression here is to list the ever increasing list of available drops for controlling
intraocular pressure. There are many combinations, often referred to for ease by trade
names, a frowned upon practice by purists, mostly in other medical disciplines. A refer-
ence list is helpful, as although one practitioner may have his own favourites, patients
can appear in clinics on other preparations, often manifesting symptoms of intolerance.
Glaucoma Drop Treatment

Beta blockers. Timolol (Timoptol), carteolol (Teoptic), levobunolol (Betagan).
Well tolerated Side effects. Slow pulse. Contraindicated in asthma.
Selective beta blocker. Betaxolol (Betoptic) . Well tolerated.
Carbonic anhydridase inhibitor. Dorzolamide (Trusopt), brinzolamide
(Azopt). 10% intolerant. Bad taste but brinzolamide better tolerated.
Prostaglandin inhibitors. Latanoprost. Long dark lashes. Monopost and
tafluprost (Saflutan) are preservative free. Avoid in inflammation.
Travaprost (Travatan) said to be better tolerated.
Bimatoprost (Lumigan).
Alpha blocker. Brimonidine(Alphagan). 12% allergic or intolerant.
Parasympathomimetic. Pilocarpine.
Combination. Cosopt (timolol with dorzolamide). Available preservative free.
Azarga (timolol with brinzolamide).
Simbranza (brinzolamide with brimonidine).
Duotrav (travaprost and timolol).
Ganfort (timolol with bimatoprost).
Xalacom (timolol with latanoprost).
Combigan (timolol with brimonidine).
4.3 Retinal Detachment
Flashes and floaters are a very frequent presentation to primary care and the vast
majority are those occurring in older individuals and due to posterior vitreous
detachment (PVD). This occurs over age 50 and most commonly over 80, the
younger patients usually being myopic. If the flashes occur temporally and in dim
lighting and associated with a single large floater, PVD is the probable diagnosis.
However, if there is vitreous haemorrhage there is a 60% incidence of retinal tear
and if there is pigment in the vitreous, a 90% association with a tear.
Examination for a retinal tear includes intraocular pressure recording as the IOP
falls in the presence of retinal detachment. Careful examination with a 3-mirror
contact lens and binocular indirect ophthalmoscopy with indentation has been
largely replaced with the use of panfunduscopic lenses and ultrawidefield retinal
imaging (Optos).
Retinal tears need to be walled off with laser which should be applied on the day
of presentation, usually once referred to the retinal service. Superior tears are likely
to progress to detachment more quickly than inferior tears because of the effect of
gravity and are therefore more urgent. Round holes within areas of lattice degenera-
tion do not require treatment. Retinal detachments are similarly referred and should
be treated as soon as possible. If the macula is still attached, i.e. visual acuity is
maintained, surgery is urgent. If the macula is already detached, the visual outcome
is significantly better if surgery is performed within 5 days of the event, rather than
if it is deferred.
Non-rhegmatogenous detachment presents less acutely, as a consequence of
inflammation or tumour.
4.4 Anterior Ischaemic Optic Neuropathy
The presentation of anterior ischaemic optic neuropathy (AION) is painless, often

with the patient wakening with loss of vision in one eye. The circulatory disturbance
is in the short posterior ciliary arteries. The degree of visual loss is variable and
there may be an altitudinal field defect. The defect remains unchanged after the first
few days. There is an RAPD and examination shows the disc to be swollen often in
one sector and sometimes with cotton wool spots and splinter haemorrhages. AION
whether arteritic or non arteritic, has a tendency to affect small nerves with a small
or absent cup. Decision making is very much based on history which helps to dif-
ferentiate non-arteritic from arteritic disease. Non-arteritic cases present at any age
but usually over 40, with a history of arteriopathy, that is hypertension, diabetes,
smoking, heart attack, stroke or peripheral vascular disease. In younger patients,
there may be a history of sleep apnoea. Amiodarone is associated with a reversible
bilateral optic neuropathy which presents in a very similar way to non arteritic
AION, is seen usually after more than 9 months of treatment. Sildenafil (Viagra) is
associated with AION by causing episodic hypotension.
4.4 Anterior Ischaemic Optic Neuropathy 31
Investigations include fasting cholesterol, lipids, glucose, FBC, electrolytes and

calcium. Treatment is aspirin 75 mg/day in appropriate patients to reduce the risk of
CVA, control of ocular hypertension if present, and a medical review to establish
any treatable cause, and minimise the risk of CVA or MI. There is a 25% risk of the
fellow eye being similarly affected.) Referral to the stroke/TIA clinic is appropriate.
Overtreatment of systemic hypertension causing nocturnal hypotension should be
considered.
Arteritic cases are largely due to giant cell arteritis (GCA) which has a character-
istic history, but other vasculitic conditions may be involved, such as PAN, SLE and
syphilis.
4.4.1 Giant Cell Arteritis
The American College of Rheumatology (1990) has listed the criteria for the diag-
nosis of GCA, with temporal artery biopsy (TAB) being the definitive test, although
it is said to be negative in 15% of cases. The diagnosis requires 3 out of 5 major
criteria to be present. The major criteria are: age over 50, new onset of localised
headache, temporal artery tenderness or reduced pulse, ESR over 50 mm in first
hour and a positive biopsy. Although central artery occlusion may be a feature of the
presentation, more typically the posterior ciliary arteries are affected and the pre-
sentation is less acute with ischaemic optic neuropathy.
GCA is very rare under 50 years of age and affects predominantly women. The
pathological changes affect medium and large sized vessels, but particularly the
temporal and posterior ciliary arteries, and the presentation is strongly associated
with polymyalgia rheumatic (PMR), characterised by limb girdle pain. 30–40% of
GCA patients have PMR at some time but this can be before during or after the
episode of GCA. Visual loss can be almost completely prevented by high dose ste-
roids and therefore timely diagnosis is important. For this reason, patients suspected
of GCA may be referred directly to ophthalmology even if visual symptoms are
absent.
Current consensus is that biopsy (TAB) is essential although this may change if
ultrasound of the temporal artery becomes the usual practice. The ESR may be nor-
mal in up to 20% of patients particularly in the very old. The use of ESR combined
with C-reactive protein (CRP) (greater than 40 mg/L) increases sensitivity. CRP
rises earlier in most diseases and is more sensitive and specific than ESR and is less
affected by age, but ESR has been the traditional test. Unfortunately, it is the cases
with a poor inflammatory marker response which experience the more serious isch-
aemic manifestations. Most patients also have a mild normochromic normocytic
anaemia. Platelets are slightly elevated and if greater than 400,000/μl support a
diagnosis of GCA, and if normal may be more helpful than the ESR in ruling out the
diagnosis. Minor features in the history include general malaise, loss of appetite,
weight loss, scalp tenderness, tongue pain and jaw claudication. Note that this is
pain specifically brought on by chewing and must be differentiated from jaw pain.
These features are supportive of the diagnosis but not indicative. If other orbital
branches of the arteries are involved the patient may more rarely present with cra-
nial nerve palsies causing diplopia.
There are practical difficulties in performing a TAB within the advised time scale
of 2 weeks from the start of steroid treatment. The TABUL study now suggests that
colour duplex ultrasonography of the temporal artery may replace TAB in the future,
being more sensitive but less specific than TAB. The classic halo sign is the diagnos-
tic feature, but the test requires further validation before it can be considered as a
replacement for biopsy. The advised protocol is to perform ultrasound initially and
if there is an equivocal result to go on to TAB. The expected reduction in the number
of TABs required is suggested to be about 85%. Bilateral halo sign is 100% specific
for GCA, and ultrasound with TAB is 95% sensitive. The Royal College of
Rheumatology is expected to publish new guidelines on this subject in 2018.
Treatment. If the diagnosis is suspected, the patient should be started on 1 mg/kg
prednisolone daily up to 60 mg as a once a day dose. If the presentation is with acute
visual loss, hospital admission for intravenous methylprednisolone 1000 mg/day for
3 days is indicated. Systemic symptoms recover within 72 hours of initiating treat-
ment and if this is observed, strongly supports the diagnosis. The steroid regimen
recommended is 40–60 mg/day for 4 weeks reducing by 10 mg every 2 weeks to
20 mg, then by 2.5 mg every 2 weeks to 10 mg then by 1 mg every month depending
on symptoms and haematological response. The whole course runs for 1–2 years.
Steroids should be given with caution to patients on digoxin, which may require a
dose adjustment. Patients taking warfarin need to attend the anticoagulant clinic
more frequently as the effect of the warfarin may change. Steroids should be given
with care to diabetics as they will upset diabetic control, and they should be avoided
or given with close supervision in the presence of infection. The patient must be
instructed to avoid taking non steroidal antiinflammatories because of an increased
risk of gastric ulceration. The most common side effects are osteoporosis, adrenal
suppression, weight gain, diabetes cardiovascular disease psychiatric disturbance
and GI bleeding. The recommendation is that patients should also be on a proton
pump inhibitor such as omeprazole 20 mg/day and biphosphonate with calcium and
vitamin D for bone protection. The use of low dose aspirin is controversial, and
there is no evidence to support it being of benefit. There may be a role for biologics
and other immunosuppressants such as methotrexate and cyclophosphamide, but
these are not currently the first line of treatment. The ongoing treatment is usually
under the supervision of a rheumatologist or the general practitioner. It is important
for the ophthalmologist to make it clear to the referring GP or rheumatologist that
the ongoing supervision of care is being handed back once the diagnosis with or
without TAB results has been made, to avoid the patient falling into a gap between
services and not having continuous care.
If the TAB is reported to be of adequate length, that is 2 cm excised shrinking to
1.5 cm when fixed, to allow multiple levels to be examined and with no features
supporting the diagnosis of GCA, the steroids can be discontinued. At high doses,
there is a risk of adrenal suppression after only 1 week of treatment and therefore
4.4 Anterior Ischaemic Optic Neuropathy 33
the steroids cannot be discontinued abruptly. The recommendation published by

NICE is to drop fairly rapidly to a physiological level, about 7.5 mg of prednisolone,
and then more slowly, by 2.5 mg per week to discontinue. The rate of reduction
depends on the duration of treatment and the general health of the patient. Adrenal
crisis which can occur if the steroids are discontinued too rapidly, is characterized
by hypotension, lethargy, hypoglycaemia seizure and coma.
TAB Technique
Select the temple on the side on which the patient has visual symptoms, or if
none the side with maximum tenderness. Identify the frontal branch of the
superficial temporal artery by palpating and mark with a pen. Shave a small
area of hair. Select an area of tenderness or nodularity, if present, of the artery.
If the artery is not identifiable, the main trunk of the artery is found just ante-
rior to the tragus of the ear. Adrenaline should be avoided in the local anaes-
thetic as it makes the identification of the artery more difficult. A skin incision
is made over the artery and parallel with its path, into the subcutaneous fat,
and by blunt dissection the temporal fascia is identified. The artery lies just
under the temporalis fascia which should be incised. Note that the facial nerve
is deep to the artery in the region anterior to the tragus. The artery is bluntly
dissected to expose up to 5 cm in length. The two ends of the proposed biopsy
are ligated and the biopsy excised with a scalpel avoiding crushing the biop-
sied tissue. The specimen shrinks during processing and as long a section as
is reasonably possible is taken, the minimum length being 2 cm to take
account of skip lesions. The pathologist requires 1.5 cm which is the shrunken
length of an in situ artery of around 2 cm.
The histopathological features are stenosis of the lumen, thickening of the intima,
fragmentation of the internal elastic lamina (ILM) with inflammatory cells includ-
ing giant cells in the media. The inflammatory cells can persist for many months
after treatment with steroids has begun and the disruption of the ILM is also a per-
sisting feature, but ideally the biopsy should be taken within 2 weeks of the pre-
sumptive diagnosis to avoid an inconclusive result.
Histopathological Features of GCA

Fragmentation of ILM- persists after inflammatory features have resolved but
not specific to GCA.
Lymphocytes and macrophages, sometimes multinucleate (giant cells) in
media.
Hyperplasia of intima with occlusion of the lumen.
4.5 Orbital Cellulitis
Primary care professionals are very aware of the risk of orbital cellulitis in children
spreading to cause cerebral abscess or encephalitis, and for that reason a low thresh-
old for referral is appropriate. Most such referrals relate to infected lid cysts or mild
inflammatory conditions of the eyelid skin and adnexae, giving rise to preseptal
cellulitis. Not uncommonly, florid attacks of adenoviral conjunctivitis are misdiag-
nosed as preseptal cellulitis.
The majority of cases (90%) of orbital cellulitis are secondary to ethmoid
sinusitis. The medial wall of the orbit is paper thin (lamina papyraceae) allowing
fairly free communication of an inflammation in the ethmoid sinus into the sub-
periosteal space of the orbit. The remainder of cases are caused by spread of
infection from dacryocystitis, maxillary sinusitis and dental abscess. The history
of the patient will reveal any recent upper respiratory tract or dental symptoms. If
there are any atypical features the possibility of rhabdomyosarcoma should be
considered.
A patient with orbital cellulitis feels unwell and the most obvious physical signs
are fever, proptosis and ophthalmoplegia, with periorbital swelling and chemosis.
Other features are reduced vision, desaturation of red, and raised IOP. The differential
diagnosis includes, thyroid eye disease, orbital myositis, idiopathic orbital inflam-
mation (also termed orbital pseudotumour), granulomatosis with polyangiitis (GPA),
leukaemia, lymphoma, metastatic carcinoma, rhabdomyosarcoma, sarcoidosis,
caroticocavernous fistula and cavernous sinus thrombosis. The features associating
the presentation with sinusitis are usually evident and an MRI scan of the orbits is
likely to show a subperiosteal abscess on the medial orbital wall. Treatment is with
intravenous antibiotic according to local policy, and daily review of signs. Any pro-
gression of the signs may indicate the requirement for drainage of an abscess but the
management is handled jointly between ENT and ophthalmology.
Cavernous sinus thrombosis is very rare but has a similar presentation to orbital
cellulitis. Venous drainage of the middle third of the face is via the facial vein which
drains into the orbital veins, and can be a route of spread of infection from a skin
lesion on the face, resulting in cavernous sinus thrombosis. The patient is febrile
with headache which progressively worsens and affects the areas of innervation of
the first two divisions of the trigeminal nerve. Periorbital oedema develops and any
of the cranial nerves traversing the cavernous sinus may be affected, giving visual
disturbance and diplopia. The signs progress from being unilateral to bilateral. The
emergency management is to take blood for culture and then give broad spectrum
intravenous antibiotic, including anaerobic cover if the precipitating infection is
dental. Heparinisation is also advised to prevent propagation of the thrombus and
septic emboli. This is a rare but life threatening condition.
Acute dacryocystitis may be misdiagnosed as orbital cellulitis because the pre-
sentation is very acute with intense pain and localised swelling and inflammation
involving preseptal structures but does not usually progress to orbital cellulitis.
There is a hot inflamed swelling inferior to the medial canthal ligament, and if a
tense abscess is present, the patient will be immediately relieved of pain by incision
Further Reading 35
of the abscess allowing the pus to drain through the skin. If the patient is pyrexial,
take blood cultures. If the abscess has not formed, apply hot compresses. Oral
amoxicillin with clavulanate (Augmentin) 500 mg bd should be prescribed for
5 days and the patient should be told that they will require lacrimal surgery to pre-
vent recurrence, and appropriate onward referral should be made. If there are signs
of spread into the orbit, the management is as for orbital cellulitis of any cause.
How to Drain a Lacrimal Abscess

Apply local anaesthetic cream over the abscess e.g. EMLA 5% (prilocaine
with lignocaine) and wait 30 min.
Skin prep with antiseptic.
Make an oblique vertical incision superomedial to inferolateral about 1 cm
in length and express the purulent content.
Collect a specimen for microbiology.
Put a gauze wick in the wound with an adhesive dressing.
Prescribe antibiotic (Augmentin 500 mg bd) and review in 2 days for
removal of dressing and wick.
Further Reading
Choong YF, Irfan S, Menage MJ. Acute angle closure glaucoma: an evaluation of a protocol for
acute treatment. Eye. 1999;13:613–6.
Hunder GG, et al. The American College of Rheumatology 1990 Criteria for the classification of
Giant Cell Arteritis. Arthritis Rheum. 1990;33:1122–8.
Icare rebound tonometer to measure intraocular pressure. NICE Medtech innovation briefing
(MIB57), 2016
Liew G, Mitchell P. Fundoscopy: to dilate or not to dilate? The risk of precipitating glaucoma with
mydriatic eye drops is very small. BMJ. 2006;7:332–3.
Luqmani R, et al. The role of ultrasound compared to biopsy of temporal arteries in the diagnosis
and treatment of giant cell arteritis (TABUL): a diagnostic accuracy and cost-effectiveness
study. Health Technol Assess. 2016;20(90):1–238.
NICE Corticosteroids-oral. Clinical knowledge summaries, 2015.
Chapter 5
Conditions Requiring Same Day
Management
5.1 Corneal Ulcers
Corneal ulcers present with a painful red eye with photophobia and reduced vision.
The clinical features vary slightly depending on the aetiology. For example, bacte-
rial ulcers are more painful than viral, while acanthamoeba infection presents with
intense photophobia. Fungal ulcers tend to evolve slowly with a less acute inflam-
matory response, and should be considered in cases where there may have been
injury with plant material or in patients using steroids.
Classification of corneal ulcers

Infective Viral
Bacterial
Parasitic
Fungal
Non-infective Autoimmune
Neurotrophic
Allergic
Blepharitis related
Idiopathic e.g. Mooren’s
5.1.1 Herpes Simplex Keratitis
The majority of ulcers presenting as an emergency are infective. Any eye profes-
sional can recognise a typical herpes simplex ulcer, and treat it with acyclovir.
These all heal without scarring within 10 days. Key features are previous episodes,
unilaterality, except in severely atopic patients, and reduced corneal sensitivity.
Difficulties however may arise if the presentation is atypical, and particularly if the
patient is a contact lens (CL) wearer. As a rule, herpetic ulcers should not be diag-
nosed in CL wearers until acanthamoeba infection is ruled out. Healing abrasions

https://doi.org/10.1007/978-3-319-92369-7_5
38 5 Conditions Requiring Same Day Management
may also present a dendritic pattern and herpetic ulcers in the peripheral cornea
may excite sufficient anterior stromal infiltrate to cause misdiagnosis as marginal
ulceration.
HSV infection can produce different patterns of disease. Primary infection tends
to produce a cutaneous lesion in a child, possibly with a follicular conjunctivitis, but
can also cause corneal epithelial disease. Secondary disease manifests in age 40s or
later, when it affects the cornea, producing not only the typical dendritic pattern
resulting from viral replication within the epithelium, but also affects the stroma and
endothelium. The terminology here becomes confused, but is clearly explained in
the American Academy of Ophthalmology guideline published in its Compendium
of Evidence-based Eye Care in 2014. Variations in terminology are still widespread
and are shown in the table:
Anatomical layer Nomenclature Alternative terminologies

Epithelium HSV epithelial keratitis Dendritic ulcer
Geographic ulcer
Stroma HSV stromal keratitis without ulceration Non-necrotising keratitis
Interstitial keratitis
Immune stromal keratitis
HSV stromal keratitis with ulceration Necrotising stromal keratitis
Endothelium HSV endothelial keratitis Disciform keratitis
If the epithelium alone is infected there is an ulcer, either dendritic or geographic.

This is treated with topical acyclovir five times a day for 7–10 days.
Stromal keratitis may be without ulceration and this corresponds with the terms
used elsewhere as non necrotising, interstitial or immune keratitis. This is an
immune response to viral or host antigen and the virus is not active. Although ste-
roid is the main element of treatment in this form of reaction, it is advisable to also
give a prophylactic dose of antiviral. These cases are treated with 2 hourly G pred-
nisolone tapering over 3 months with acyclovir 400 mg bd.
Stromal keratitis with ulceration, alternatively termed necrotising stromal kera-
titis is an actively infected process with an additional damaging inflammatory
reaction. Here the management is G prednisolone twice a day and acyclovir
800 mg five times a day for 10 days and then 400 mg twice a day until the steroid
is stopped.
Endothelial keratitis also called disciform keratitis is an immune process, prob-
ably with an infective element, characterised by corneal oedema and keratic precipi-
tates with minimal anterior chamber activity. It is treated with g prednisolone 2
hourly and acyclovir 400 mg five times a day for 10 days then twice a day while
steroids continue. Monitoring progress is best achieved by visual acuity changes
and corneal pachymetry, the thickness reducing as the oedema resolves.
Endotheliitis may progress to anterior uveitis, trabeculitis and secondary
glaucoma.
If there is doubt as to the aetiology of the epithelial keratitis, infection can be
confirmed by sending a scraping for polymerase chain reaction (PCR) assay for the
virus, but there is no way to confirm stromal or endothelial disease.
5.1 Corneal Ulcers 39
Topical antivirals are toxic to normal epithelium and should not be used for more
than 14 days. Systemic treatment with antivirals is preferable in more complex forms
of herpetic keratitis. Patients with frequently recurrent disease may require long term
prophylactic oral antivirals. 400 mg acyclovir twice a day for a year is recommended
for patients with more than two episodes in a year. Systemic antivirals should be
used more cautiously in patients with renal impairment and in pregnancy.
Neurotrophic ulcer can develop in an anaesthetic cornea and it may be necessary
to protect the cornea with a bandage contact lens, a botulinum toxin induced ptosis
or a temporary tarsorrhaphy.
Children respond to HSV differently from adults. They are more likely to have
bilateral disease, are more likely to have a stromal reaction and are more likely to
have recurrent disease. There is a suggestion that this is because the diagnosis is
delayed in children whose symptoms may not be acted upon quickly. Because of
their brisk inflammatory response, children are at high risk of becoming amblyopic
and need close supervision.
5.1.2 Herpes Zoster Ophthalmicus (HZO)
Ophthalmologists are regularly asked to give an opinion on patients with HZO,

particularly those who are admitted as inpatients because of general debility. The
rash can look very alarming sometimes with pronounced periorbital oedema. The
most useful initial assessment is to look for any extension of the rash down the side
of the nose, as if this area of skin is involved the likelihood is that the intraocular
structures are also involved. Absence of a rash on the side of the nose does not how-
ever absolutely exclude ocular involvement, but does make it less likely. About 50%
of patients with a shingles rash involving the forehead develop ocular involvement.
The consequences of infection are a combination of a vasculitis and a neuritis. All
patients will have a conjunctivitis but this does not require any treatment other than
hygiene measures. Ocular involvement may be a keratitis with fine stellate pseudo-
dendrites, or anterior stromal infiltrates. There may be an anterior uveitis with KP
and secondary glaucoma. Less frequently choroiditis, retinitis (acute retinal necro-
sis and progressive outer retinal necrosis), optic neuritis or nerve palsies can occur.
Endothelial (disciform) keratitis affects about 10% of HZO patients and may pres-
ent about 3 weeks after the rash and is treated with topical steroids. The treatment is
topical steroids as appropriate, together with systemic antivirals, acyclovir 800 mg
five times daily for 7–10 days.. Newer antivirals requiring less frequent dosing will
replace the current commonly prescribed drugs, to achieve better patient compli-
ance with treatment. Topical antivirals have no role in HZO. The keratitis and uve-
itis may be recurring problems after the initial episode.
Oral acyclovir should be started as soon as the diagnosis is made or within 72 hours
of onset of symptoms, as it reduces the duration of the acute symptoms and there is
equivocal evidence that it prevents or decreases the severity of post herpetic neural-
gia. This effect can be demonstrated even if the antiviral is given later than the recom-
mended 72 hours after onset, so it is worth prescribing even in late presentations. Post
herpetic neuralgia is much more frequent in patients with HZO (over 50%) than those
with the rash at other sites. Neurotrophic keratitis affects up to 25% of patients.
5.1.3 Adenoviral Keratoconjunctivitis
Conjunctivitis is usually managed outside hospital ophthalmology services but ade-

noviral infection is an exception. Although a minor condition, the symptoms can be
quite severe with pain and photophobia. There is a follicular conjunctivitis associ-
ated with upper respiratory tract symptoms, preauricular lymphadenopathy, chemo-
sis and if the cornea is involved, there are superficial punctuate staining lesions.
Later, at about 2 weeks, subepithelial discrete circular opacities which represent an
immune reaction may appear. These can persist for months and cause some reduc-
tion in acuity with glare, but usually resolve without scarring. There is no specific
treatment but non-steroidal anti-inflammatory drops may be given for symptomatic
relief. This is a highly contagious condition and there is no indication to review
these patients who can be reassured that the symptoms will resolve in 1–3 weeks.
Careful hygiene is advised to prevent spread to others and also to prevent contami-
nation from one eye to the other. The highly infectious nature of the condition relates
to the virus being resistant to standard disinfection and remaining viable for up to
5 weeks. The virus sheds from the eye for 3 days prior to symptoms and for 14 days
after onset. If clinic personnel are infected they should be kept away from patient
contact for 2 weeks from the onset of symptoms. Swabs from the conjunctiva can be
taken for PCR analysis if there is doubt about the diagnosis or to monitor a cluster
outbreak. The differential diagnosis includes Chlamydia infection, and primary her-
pes simplex. There are commercially available kits which can identify adenovirus in
the clinic and these also may be a useful tool, especially in limiting outbreaks.
Chlamydia causes conjunctivitis in neonates infected during passage through an
infected birth canal, or as a sexually transmitted disease. Children may be infected
from the conjunctival secretions of an adult. The diagnosis should be considered in
a unilateral conjunctivitis, and is characterised by a mucopurulent discharge, con-
junctival follicles and papillae and preauricular lymphadenopathy. The course may
run many months. There may be a keratitis very similar to that seen in adenoviral
keratoconjunctivitis. Conjunctival swabs should be taken for Chlamydia culture and
if positive the patient and partner should be referred to the STD clinic. The treat-
ment is with systemic antibiotic for 3–6 weeks with tetracycline 500 mg qid or
doxycycline 100 mg bd. Pregnant or nursing mothers and children should be treated
with erythromycin 500 mg qid or the appropriate paediatric equivalent.
5.1.4 Bacterial Ulcer
Bacterial ulcers tend to be more severe and more symptomatic. The predisposing fac-
tors are trauma, blepharitis, poor ocular surface particularly in diabetics, and the most
feared, contact lens wear. Contact lens related keratitis will be considered separately.
Bacterial keratitis ranges from a small infiltrate to a large epithelial defect, with
symptoms depending on the severity of the inflammation. Infiltrates are collections
of white blood cells which have migrated from the limbus into the stroma in response
to an insult. If the epithelium overlying them breaks down, an ulcer forms. If there
is an ulcer, the lesion needs to be scraped for microbiological examination. The
corneal sensation is checked and the ulcer is measured and drawn carefully, and
remeasured after scraping. Recording should also be made of the degree of conjunc-
tival reaction, the characteristics of any discharge, the presence of an endothelial
reaction, the extent of stromal haze or oedema and the presence of anterior chamber
reaction or hypopyon.
How to Scrape an Ulcer

Instil local anaesthetic drops.
Prepare Petri dishes and transport media according to local microbiology
protocol (chocolate agar, blood agar, Sabouraud’s medium for fungi, thiogly-
colate for anaerobes, saline for acanthamoeba).
Position patient at slit lamp.
Put sterile glove on dominant hand and take a sterile needle or a scalpel
blade.
Keeping the lids and lashes away from the sampling instrument, scrape
margins of the ulcer and inoculate plates and transport media, dropping the
needle or blade into the last vial of transport medium.
Send to laboratory along with any contact lens or lens case if appropriate.
The cause of an ulcer can not be diagnosed from the appearance but there are some
differentiating indicative features. Absence of corneal sensation points to herpetic
keratitis or neurotrophic ulceration. Extensive necrosis with deep ulceration is likely to
be due to Gram negative infection, most commonly Pseudomonas aeruginosa, which
produces proteolytic enzymes and may perforate. Treatment should be started imme-
diately after scraping if bacterial infection is suspected, with intensive antibiotic. The
treatment depends on local protocols. G Levofloxacin hourly day and night is recom-
mended, with cyclopentolate twice daily. Daily review is needed to chart improvement
as shown by change in the dimensions of the ulcer and reducing pain. The density of
the infiltrate does not relate to response to treatment as it may become denser while
reducing in size with healing. Once this is evident, the overnight medication can be
discontinued. Overnight drop administration may require hospital admission, depend-
ing on social circumstances. Failure to respond to treatment indicates that the causative
organism is resistant. Fungi, MRSA and acanthamoeba must be considered.
There is controversy as to the use of steroids with antibiotic in treating bacterial
ulcers (SCUT trial). The rationale for using steroid is to reduce the inflammatory
reaction and tissue destruction caused by the infection, but the risk is that they allow
the infectious agent to continue to flourish, especially if the causative agent turns
out to be acanthamoeba or a fungus.
Rarely a patient will present with a Descemetocoele or a perforated ulcer. The
perforation is likely to be plugged by iris but the anterior chamber is flat or very
shallow. The patient is aware of pain and increased, sometimes copious watering.
These should be managed as an emergency with measures to plug the defect, or in
the case of a Descemetocoele to support the Descemet’s membrane and endothelial
layer which is maintaining the integrity of the globe. The most expedient method is
to use tissue glue, either cyanoacrylate or fibrin glue and antibiotic, with a contact
lens over it. Amniotic membrane patch is a possibility but there is a delay while
waiting for the tissue to be available. The definitive treatment may be corneal graft-
ing, but a good option in an eye with a poor visual prognosis is a Gunderson flap,
where a peritomy is made and the conjunctiva is pulled down over the corneal
defect. The conjunctiva adheres to the defect in the cornea and slides back from the
epithelialised areas. If the perforation has occurred with expulsion of intraocular
content, as may occur in severely diseased eyes with longstanding corneal disease,
there is no option but to eviscerate or enucleate the eyeball. The patient is usually
happy to accept that a chronically irritable or painful eye is no longer a problem.
Technique for Creating a Gunderson Flap

Anaesthetise with general or peribulbar anesthetic
Remove all corneal epithelium and necrotic tissue
Retract eyeball inferiorly
Perform 360° peritomy
Dissect superior conjunctiva carefully away from Tenon’s to superior fornix
avoiding buttonholing
Open conjunctiva parallel with the superior fornix 15 mm from limbus
Pull flap down over the cornea and anchor to limbus with 4 8/0 Vicryl sutures
Close peritomy by suturing to flap inferiorly
Suture superior edge of flap to remaining superior conjunctiva
5.1.5 Contact Lens Associated Corneal Disease
The most minor condition included here for completeness is contact lens associated
red eye, due to overwear. This should never present to a secondary care clinic. More
significant is infiltrative keratitis. Infiltrates are usually small, usually less than
1 mm and round in the peripheral 3 mm of the cornea. They are single or multiple
and the eye remains quiet. These are not infective but are an immune response to
bacterial antigens present on the lens or lid margin and possibly also hypoxia or
reactions to chemicals and preservatives. They are seen in patients wearing re-
usable and extended wear lenses, more often with poor hygiene, and they respond
to treatment with steroid drops along with discontinuation of contact lens wear for
a period and instructions regarding handling of the lenses. However, untreated,
symptoms resolve within 24 h and the infiltrates resolve within 3 weeks. If these
infiltrates progress to a degree that they break down, infection may ensue and the
treatment is for bacterial ulcers. If there is coexistent blepharitis, this should be
managed with hot compresses and lid scrubs. Contact lens related ulcers can present
late partly because wearing the lens may mask the symptoms and also because the
cornea in contact lens wearers is less sensitive. There should be constant alert to the
possibility of acanthamoeba infection.
5.1.6 Acanthamoeba Keratitis
Acanthamoeba is ubiquitous in water including tap water. The history of an infected

patient is a contact lens wearer who showers or swims in his lenses or cleans his
lenses in tap water. There may be a history of minor trauma. The eye become painful
photophobic and watery and the symptoms are typically out of proportion to the
signs in that the ulcer may be small and the eye may not look very inflamed but the
photophobia is intense. In the early stages the lesion may look dendritic and a useful
rule is never to diagnose herpes simplex keratitis in a contact lens wearer. A missed
dendritic ulcer is not a big problem, but a missed acanthamoeba ulcer can progress
to a severe vision threatening lesion which is difficult to treat. On slit lamp examina-
tion the lesion is small and branching with satellite lesions. Radial neuritis is a
manifestation of the infection following the corneal nerves. Later ring shaped infil-
trates are seen, but hopefully the diagnosis has been suspected before this stage
develops. If available, scanning confocal microscopy shows the cysts in the cornea
and is the gold standard for diagnosis prior to microbiological proof. In the early
stages treatment is with polyhexamethylene biguanide (PHMB) 0.02% or chlorhex-
idine 0.02% can be used in combination with propamidine isethionate and dibromo-
propamidine isethionate (Brolene). Initially treatment is hourly drops for 5 days,
reducing to qid but it may take 2 weeks before any response can be seen. Patients
should be referred to a corneal specialist as the treatment can be protracted over
many months, and the treatment, (especially PHNB) is toxic to the cornea. If treat-
ment is unsuccessful, the only alternative is penetrating keratoplasty. Cysts may
remain in the cornea after treatment and cause reinfection, so patients require follow
up for some time after resolution of signs, ideally 6 months.
Acanthamoeba keratitis is rare in non contact lens wearers. Note that finding acan-
thamoeba in a contact lens case does not prove infection but absence of it rules it out.
5.1.7 Fungal Ulcers
Fungal infection of the cornea is rare in developed countries but is associated with
corneal trauma contaminated with organic matter or sometimes in contact lens
wearers, neurotrophic corneas or immunocompromised patients. Patients with fun-
gal infection have less severe pain than those with bacterial infection. There are
infiltrates with feathery edges, sometimes brownish pigmentation, satellite lesions,
endothelial plaque and hypopyon. Fungus should be suspected in patients with
severe infective ulceration not responding to antibiotic treatment and in whom the
severity of the corneal lesion appears out of step with the severity of symptoms.
Initial treatment is hourly econazole drops. Amphoterecin B is the preferred treat-

ment for Candida infection. Treatment is typically for 6 weeks but can be longer.
5.1.8 Non Infective Ulcers
5.1.8.1 Autoimmune
Sterile corneal ulceration is associated with connective tissue diseases, notably

rheumatoid disease (RA), granulomatosis with polyangiitis (GPA), polyarteritis
nodosa (PAN), systemic lupus erythematosus (SLE), relapsing polychondritis and
Sjogren’s syndrome. The ulcers form peripheral gutters as a consequence of immune
complex infiltrates inducing an inflammatory reaction with breakdown of corneal
tissue. There is often an associated scleritis and pain is a significant feature. The
patient is almost certainly already known to the rheumatologists but investigations
which have not already been done should be ordered. These are FBC, U and E, ESR,
rheumatoid factor, antinuclear antibody (SLE and RA), anti double stranded DNA
(SLE), and ANCA (GPA), as well as tests for the great imitators, FTA-ABS for
syphilis and ACE for sarcoidosis.
Treatment is directed towards the systemic condition and requires joint manage-
ment with a rheumatologist. Topical treatments include lubricating preparations,
and possibly cyclosporine or tacrolimus. Steroids should be avoided as they can
cause progressive thinning of the cornea. Collagenase inhibitors such as N acetyl-
cysteine, doxycycline and oral vitamin C are helpful. Perforation, may be managed
with cyanoacrylate glue and a bandage contact lens. Ongoing management is in the
hands of the corneal specialist in partnership with the rheumatologist.
5.1.8.2 Neurotrophic ulcers
Occur in desensitised corneas such as are seen in patients who have had herpetic
keratitis or treatment for trigeminal neuralgia, acoustic neuroma or in diabetics. The
desensitised eye is susceptible to minor injury and decreased reflex tearing increases
the risk of epithelial breakdown. Denervation itself also affects the corneal defences
by reducing epithelial turnover. Exposure due to lid malposition or loss of blinking
causes similar but usually less severe ulceration.
Management is with lubricants, Botulinum toxin induced ptosis or tarsorrhaphy.
Technique of inducing ptosis using Botulinus toxin A

With a 26G insulin syringe, draw up 5 units of Botulinum toxin
Inject into levator palpebrae superioris, just below orbital rim at level of the
pupil
Repeat after 1 week if ptosis not adequate
Expect ptosis in 5–7 days, lasting 6–8 weeks
5.1.9 Allergic Eye Disease
Allergic eye disease is classified into acute and chronic disease.
Allergic Eye Disease
Acute Seasonal
Perennial
Chronic vernal Vernal
Atopic
The cornea is involved in the chronic forms of allergic eye disease, never in the
acute forms. Acute allergic conjunctivitis, i.e. seasonal or perennial conjunctivitis is
an IgE mediated Type I response, treated with antihistamines, whereas chronic, ie
vernal (VKC) and atopic (AKC) are manifestations of a more complex IgE and
Tcell response.
5.1.9.1 Vernal keratoconjunctivitis
Vernal keratoconjunctivitis is most frequently seen in young boys. There is often

a history of eczema or asthma and a family history of atopic disease. It is a severe
bilateral chronic inflammation, characterised by intense itching, foreign body sen-
sation, stringy discharge, photophobia and blurred vision. It is usually a seasonal
condition but in about 25% of cases can be perennial. The conjunctiva shows giant
papillae as well as diffuse injection. The limbal conjunctiva may be thickened and
gelatinous with focal white dots (Trantas dots) which are collections of eosino-
phils. Limbal involvement can result in stem cell loss, leading to pannus and
neovascularisation. Punctate erosions may coalesce usually in the superior cor-
nea, with an overlying plaque of mucus and fibrin to form an ulcerated lesion,
termed a shield ulcer. Shield ulcers without plaque heal more quickly and with
less scarring and vascularisation than if plaque is present. The disease usually
subsides at puberty. Treatment is with anti allergy drops such as olopatadine twice
a day, with steroid use kept to a minimum. There is a significant risk of VKC
patients developing glaucoma secondary to steroid eye drops, and non steroidal
drops such as diclofenac and ketorolac are helpful. In severe cases with ulcer-
ation, cyclosporine 0.5–2% drops qid are effective and steroid sparing. A simple
symptomatic means to relieve itching is to use ice or a cold compress. The child
should be encouraged not to rub the eyes as rubbing enhances the inflammatory
reaction and aggravates the symptoms. Advice about avoiding exposure to aller-
gens in the home is also useful.
5.1.9.2 Atopic keratoconjunctivitis
Atopic keratoconjunctivitis is relatively rare and presents later in association with

atopic dermatitis and often asthma and rhinitis. It shares some features with VKC
but the conjunctiva shows a small papillary reaction. Trantas dots may be present
and also scarring of the conjunctiva, symblepharon, and shield ulcers. The symp-
toms are worse in the morning due to overnight accumulation of immune mediators.
The condition is associated with keratoconus and premature development of poste-
rior subcapsular cataract. Anti allergy drops and steroids are the management
approaches, as well as topical or systemic cyclosporine and systemic antihista-
mines. Steroid drops should not be used for more than 14 days. In intractable cases
tacrolimus and mitomycin C drops have a role, particularly in inhibiting neovascu-
larisation. Again, avoiding exposure to allergens is helpful, as is avoiding exposure
to sunshine, wind and salt water, by wearing a hat, swimming goggles and
sunglasses.
Allergy Treatment
Mast cell stabalisers Sodium chromoglycate (Catacrom is a

preservative free preparation)
Lodoxamide
Dual action-Mast cell inhibitor and Olopatadine
antihistamine Ketotifen
Steroids Prednisolone
Fluoromethalone
Non steroidal Ketorolac
Dilofenac
Immune modulators Cyclosporine
Tacrolimus
Mitomycin C
5.1.10 Keratoconus
Keratoconus would not usually present to a rapid access service except if acute
hydrops develops. This happens when Descemet’s membrane fractures allowing
fluid to enter the cornea causing sudden deterioration of vision accompanied by
photophobia. Cyclopentolate and hypertonic saline drops may help relieve symp-
toms and the condition resolves in 2–3 months usually with residual scarring requir-
ing penetrating keratoplasty. Intracameral gas injection may reduce the symptoms
and delay the need for a graft.
5.1.11 Keratitis Secondary to Blepharitis
Longstanding staphylococcal anterior blepharitis is associated with superficial punc-

tate keratitis, marginal infiltrates and ulceration, as well as limbitis, peripheral cor-
neal thinning, pannus and phlyctens. The points at which the lids cross the limbus are
most frequently involved, that is 10 and 2 o’clock and 8 and 4 o’clock. The reaction
is a hypersensitivity response to staphylococcal toxins. Being an immune reaction
the treatment is with steroid, but if the ulcer is large or shows significant inflamma-
tion, it should initially be treated as an infective ulcer, that is it should be scraped for
microbiology and then treated with antibiotic for 24 hours before steroid is intro-
duced. The symptoms subside rapidly once the topical steroid is started, four times a
day usually as a combined preparation with antibiotic such as Betnesol N or Maxitrol,
and the keratitis usually heals within 2 weeks. The cause, that is the blepharitis, needs
to be addressed to prevent the condition being a frequently recurring problem.
5.1.11.1 Blepharitis
Any general Ophthalmologist will not be surprised to know that blepharitis accounts
for 5% of all primary care ophthalmology Primary care is where it should stay
unless lack of control results in corneal ulceration. Further details about the condi-
tion are included for completeness and to be used in educating patients and primary
care providers. The condition is constitutional, and if described to the patient in
such terms, the patient is more likely to understand that it is not a disease and there-
fore does not require a cure, but rather needs to be managed by the patient through-
out his life. The symptoms fluctuate in severity often in relation to weather, and also
in relation to general state of health. A useful website, www.eyecalm.org, is avail-
able to give patients access to relevant information.
The symptoms are bilaterally sore, itchy, irritable eyes, worse in the morning
with sticky discharge adherent to the lashes.
Blepharitis Classification
Anterior: characterised by hyperaemia, telangiestasia of the lid margin, col-
larettes round the base of the lashes, trichiasis, madarosis (loss of lashes),
poliosis (loss of lash pigment), tylosis (thickening and notching of tarsal bor-
der of the lid) and associated staphylococcal infection.
Combined anterior and posterior
Seborrheic: characterised by hyperaemia and sticky deposit on lashes, but less
inflamed and associated with scaly, oily skin elsewhere particularly scalp, ear
and forehead. Demodex mite infestation causes anterior and posterior blepha-
ritis by blocking follicles and glands.
Posterior: associated with Meibomian gland dysfunction with oil globules or
foam round gland openings and telangiectasia and tarsal thickening.
Always beware unilateral blepharitis and consider malignancy, particularly seba-

ceous carcinoma but also squamous and basal cell carcinoma.
Contact dermatitis may be a reaction to cleaning products, make up or topical
medication and can cause blepharitis but the pattern of the dermatitis is usually eas-
ily recognised. Sjogren’s syndrome may have an element of blepharitis exacerbat-
ing symptoms, and managing the blepharitis will improve eye comfort.
Phthiriasis palpebrarum (pubic lice) are a rarer cause, while Demodex mites are
extremely common as a cause or an exacerbating factor of blepharitis.
The aim of treatment for all forms of blepharitis is to remove any debris and skin
scales on the lid margin which may obstruct the flow of meibomian secretions, and
increase the flow of secretions. This is achieved with local application of heat either
with hot compresses, an eyebag (commercially available microwaveable heat pad)
or by steam (commercially available such as Blephasteam). The heat treatment
needs to be over 10–15 minutes to melt the buttery secretions into an oily form
which is then more easily expressed from the glands with a cotton bud rolled over
the lid margin on the external surface at the base of the eyelashes. Some practitio-
ners recommend lid scrubs with baby shampoo diluted 1:10 or bicarbonate of soda
in water, but the simpler the routine the more likely is the patient to carry it out daily
in the long term. Some patients like commercially prepared presoaked eye scrub
pads, but these are not necessary, although there is a vogue for marketing tea tree oil
scrubs as a way to eradicate Demodex mites. Vaseline or a gel type of eye ointment
is probably as effective in preventing the mites from moving from follicle to follicle
and may even kill them by suffocation.
Patients with marked meibomian gland disease benefit from a 6–12 week course
of a tetracycline, most commonly doxycycline 100 mg bd for a month then once a
day for some months or indefinitely. This acts not only against the staphylococci but
also alters the consistency of the sebum by changing the fatty acid composition and
increasing its solubility. It also inhibits collagenase activity and are therefore is
beneficial in corneal ulceration.
Rosacea is particularly associated with blepharitis and corneal ulcers. Triangular
fronds of vascularised pannus based at the limbus are highly suggestive of this diag-
nosis. It is best managed with systemic doxycycline at the subantimicrobial low
dose of 40 mg/day in a modified release preparation (Efracea) for 4–6 weeks, or
longer if necessary. It is important to explain to the patient that this is not only an
antibiotic, but is also immunomodulatory and an inhibitor of proteolysis, therefore
having an effect on the underlying pathological processes. Without this explanation,
the patient may choose to discontinue treatment as the course is much longer than
needed for its antibiotic properties. This should however be avoided in pregnancy,
breast feeding and in children under 12. Side effects are IIH, gastrointestinal upset
and vulval candidiasis.
Blepharitis can cause secondary conjunctivitis, tear film breakdown conjunctival
cysts concretions and keratitis. It is tempting (and very easy) to remove concretions
from the tarsal conjunctiva but this has no beneficial impact on symptoms and
should be avoided, as some patients then believe that their symptoms can be helped
by the procedure and will return requesting repeat treatment unnecessarily.
5.2 Scleritis 49
In cases where the lid margin becomes distorted with trichasis and distichiasis, it
is worth considering a diagnosis of mucous membrane pemphigoid amd referring
the patient to the external diseases or corneal clinic.
Children with blepharitis may present with episodes of conjunctivitis, superficial
punctuate keratitis (SPK), phlyctenules, marginal keratitis, or with recurrent styes
and meibomian cysts. They usually present under 10 years old, most commonly
around 5, with chronic itching, irritation, redness and photophobia. Symptoms usu-
ally subside by about age 8. The aims of treatment, as with adults are to improve
meibomian gland function with heat and massage, and to reduce the staphylococcal
load. Erythromycin 30–40 mg/kg/day in four divided doses or clarithromycin
15 mg/kg in two divided doses for 3 weeks is helpful. Erythromycin accumulates in
the meibomian glands and affects the composition of sebum, as does tetracycline in
adults. Treatment for dandruff with appropriate shampoo may help to control symp-
toms also. Surgical intervention for meibomian cysts in children should be avoided
despite often very considerable pressure from parents. Explaining that surgery
destroys several glands and that those glands have to last a lifetime to maintain a
healthy tear film and eye is usually dissuasive.
5.1.12 Idiopathic Keratitis
Mooren’s ulcer is a very rare peripheral ulcer, diagnosed by exclusion of all other
systemic causes of ulceration and not associated with scleritis. It is said to predomi-
nate in healthy young men although any age and either sex can be affected. Its
prominent characteristic is the intensity of pain and inflammation it causes. The
ulcer is progressive both circumferential and centrally involving the anterior half of
the stroma, and the central edge is described as undermined. Treatment is with
intensive topical steroids with cycloplegia and antibiotic to prevent infection. Other
treatments include conjunctival resection and immunosuppressants, but this type of
patient would be referred to a specialist corneal service. Despite its rarity trainee
ophthalmologists regularly add Mooren’s ulcer to their differential diagnosis of cor-
neal ulcer.
5.2 Scleritis
Scleritis presents in middle aged or older patients, with most being between 40 and
60 years, and may be bilateral. 50% are associated with systemic disease. 90% of
cases are anterior and 10% posterior. Of the anterior cases 75% are non necrotising
and associated with anterior uveitis. The 25% which are necrotising are the more
difficult to manage, and avascular areas can be seen on the sclera causing sclera
melting (scleromalacia perforans). The presentation is with deep seated pain, which
is moderate to severe and disturbs sleep. This is accompanied by photophobia,
watering, tenderness and reduced vision and an increasingly red eye over a few
days. The 10% which are posterior may present with a white eye, exudative retinal
detachment, and macular or disc oedema. On examination there is redness with a
purplish tinge (referred to classically as violaceous). The redness does not blanch
with phenylephrine as the engorged vessels are deep. Black or brown areas indicate
thinning or necrosis. A history of trauma or surgery may indicate a cause, but if
none, extensive medical investigation is required. In a significant number of cases
scleritis is the presenting feature of a potentially life threatening condition. The
ocular complications are ulcerative keratitis (13%), uveitis (42%), glaucoma (12%)
and cataract (10%).
Conditions associated with scleritis

local HZO
Trauma
Surgery
Generalised RA
GPA
SLE
PAN
Inflammatory bowel disease
Syphilis
TB
Ankylosing spondylitis
Psoriatic arthritis
Reactive arthritis
Gout
Polymyositis
Sjogren’s disease
Behcet’s disease
GCA
Investigations for Scleritis

ESR, FBC, CRP
Rheumatoid factor
ANA (SLE, RA, polymyositis)
ANCA (GPA, PAN)
HbsAg (PAN)
HLA B27 (ankylosing spondylitis, psoriatic arthritis, inflammatory bowel
disease)
Uric acid (gout)
Syphilis serology
Lyme disease serology
ACE (sarcoidosis)
5.3 Uveitis 51
Ultrasound is helpful in diagnosing posterior scleritis. There is flattening of the

posterior globe with thickening of the sclera and retrobulbar oedema. An MRI or CT
scan is required if there is associated proptosis, to exclude orbital inflammation or
tumour.
The first line of treatment is systemic non steroidal anti inflammatory drugs.
Beyond that point, a rheumatologist should work in conjunction with the ophthal-
mologist. The next step is immunosuppression with cyclophosphamide or metho-
trexate, and the biological response modifiers. Periorbital steroids should be avoided
especially if there is necrosis or corneal ulceration.
5.3 Uveitis
5.3.1 Acute Anterior Uveitis (AAU)
Acute anterior uveitis (AAU) is the commonest form of uveitis making up about
90% of all cases of uveitis, but again for the sake of completeness and teaching, its
place within the whole field of uveitis is considered. AAU resolves within 3 months.
It is considered chronic if an attack lasts beyond that time. The different types of
uveitis have a differing geographical and racial incidence, and large series pub-
lished from different countries reflect this. A recent study from a French group
(ULISSE) is a good reflection of the Western European picture. The mean age of
their patients was 46, with 70% anterior, 10% intermediate, 10% posterior and 10%
panuveitis. The lower proportion of anterior uveitis in their publication is a reflec-
tion of the incidence of more complex cases in tertiary referral centres, with many
anterior cases being managed without complication in community and general sec-
ondary care.
The cause or disease association of uveitis is only ever identified in about 50%
of cases and even if it is identified it may not affect management. For this reason,
the consensus now is not to investigate fully cases of uveitis, but to tailor the
investigations to the findings. Current advice is to offer FBC, ESR, CRP, syphilis
serology, ACE, HLA B27, urinalysis for diabetes and a chest X-ray to look for
evidence of TB or sarcoidosis at the second episode or in bilateral presentations.
In recurrent cases testing for HLA B27 is likely to be positive in 50% (2–8% in
the general population). This is useful in that the patient can be warned of likely
recurrences with advice to seek treatment early should recurrence occur, and pre-
vent complications. In patients who have had numerous recurrences, it is some-
times useful for them to keep an unopened bottle of steroid drops for use if the
recurrence occurs when it is difficult to get treatment or for example when on
holiday. This is not advisable unless the patient has had a number of well docu-
mented attacks. Sometimes a patient may have had a very mild or misdiagnosed
attack and returns to the clinic having treated their irritable eye themselves for a
few days, by which time the physical signs may have melted away, or may never
have been there. The patient is then given to believe that they have recurrent uve-
itis and will tell the next treating professional who may not realise that the diag-
nosis can be in doubt.
Investigations for Second Attack of AAU, or Bilateral Cases

FBC, ESR, CRP
Syphilis serology
ACE
HLAB27
Chest X-ray
HLA B27 associated uveitis can be mild or severe and mainly affects men with
the first episode age 20 to 40. The presentation is typically with a photophobia red
eye, and sometimes severe inflammation with posterior synechiae, fibrinous exu-
date and a hypopyon. The inflammation tends to be more severe in women who also
may have a worse clinical course. Each episode lasts 6–8 weeks and in 2/3 of
patient’s there is a recurrent pattern, the second episode occurring on average
2 years later and the third after another year. The prognosis is generally good
although complications of macular oedema, cataract and glaucoma may occur. 50%
of the patients will develop a seronegative arthropathy (ankylosing spondylitis,
Reiter’s disease or psoriatic arthropathy) or inflammatory bowel disease (Crohn’s
disease or ulcerative colitis). Incidentally, acute anterior uveitis occurs in 25% of
patients with HLA B27 associated arthropathy or inflammatory bowel disease.
There are theories why HLA B27 is associated with inflammation but the patient
needs to understand that it is an association, not a cause. HLA B27 negative AAU
present at a later average age.
At the first and every subsequent clinic visit, the visual acuity and intraocular
pressure must be recorded. If there are posterior synechiae on presentation, vigor-
ous attempts must be made to break these before the patient leaves the clinic. If
they are resistant, it is helpful to instil dilating drops (usually phenylephrine and
cyclopentolate), and sit the patient in the dark with a hot compress over the eye.
Repeat, the drop application after 30 min if not successful. If still not successful,
sub conjunctival mydricaine 0.3 ml (procaine, atropine and adrenaline) is required
given with subconjunctival dexamethasone 2.5 mg or betamethasone 2 mg in
0.5 ml. The patient should be warned that after a subconjunctival injection the eye
may ache severely, and 1 g paracetamol should be given prior to leaving the clinic.
If the vision is reduced at the first visit, an OCT of the macula to demonstrate any
macular oedema is useful for following progress of treatment. The patient is given
a prescription for hourly or 2 hourly steroid drops, either dexamethasone or beta-
methasone, and cyclopentolate 1% at night if no synechiae or twice a day if there
5.3 Uveitis 53
were synechiae at presentation and a review appointment in 1–2 weeks to check

for a rise in intraocular pressure. It is important to tell the patient that he will feel
better very quickly but he must continue the treatment as the drops only control
the symptoms while the inflammatory episode runs its own course over 6–8 weeks.
The drop treatment should be tapered according to physical signs at review rather
than by giving the patient a reducing schedule from the start. These instructions
apply to all patients on treatment for AAU. In patients with associated inflamma-
tory diseases, consultation with their physician regarding systemic dosage of
immunosuppressants may decrease the frequency of recurrences of eye
symptoms.
A note on topical steroids. Dexamethasone causes a rise in intraocular pressure
in about 10% of cases, prednisolone in 7%, and fluoromethalone (FML) in many
fewer. However FML has a less powerful anti inflammatory effect. Topical steroids
cause a rise in IOP in children more frequently, more rapidly and to a higher level
than in adults, and the younger the child the more the effect is observed. If topical
steroids are ineffective, usually because of poor compliance, tramcinolone (a long
acting depot preparation of steroid) can be injected into the orbital floor. A deep
transeptal injection of 20–40 mg of triamcinolone is a good option, and is less
likely to cause a rise in IOP than a subtenons injection. Patients who respond to
topical steroids with raised intraocular pressure do not necessarily respond in the
same way to periocular injections. The injection can be repeated after 2–3 weeks if
necessary.
Classification of uveitis
Anatomical
Anterior Iritis
Iridocyclitis
Anterior uveitis
Intermediate Pars planitis
Posterior cyclitis
Vitritis
Posterior Focal, multifocal or diffuse choroiditis
Chorioretinitis
Retinitis
Neuroretinitis
Panuveitis
Aetiological
Infectious Bacterial
Viral
Fungal
Parasitic
Non infectious Known systemic association
Unknown systemic association
Masquerade Neoplastic
Non neoplastic
5.3.2 Identifiable Associations of AAU
Identifiable Associations of AAU

HLA B27 associated inflammatory disease
Herpetic disease
Sarcoidosis
Fuch’s heterochromic cyclitis
Behcet’s disease
Posner-Schlossman syndrome
Herpetic anterior uveitis is caused by herpes simplex, herpes zoster and less
commonly cytomegalovirus infection. Herpes infection accounts for about 10% of
uveitis referrals to tertiary centres. If there is accompanying active skin or corneal
disease, the diagnosis is straight forward, but it may present later when the other
features of herpetic eye disease have settled. It is nearly always a unilateral condi-
tion with frequent recurrences. The amount of anterior chamber activity ranges
from mild to severe with hypopyon, any type of KP and commonly posterior syn-
echiae. The intraocular pressure is usually raised compared with most cases of non
herpetic AAU where the IOP is low. The other AAU types associated with raised
pressure at presentation are syphilitic, sarcoidosis and Posner-Schlossman syn-
drome (probably herpes virus trabeculitis). Other useful signs are reduced corneal
sensation, interstitial corneal scarring and sector or diffuse iris atrophy. Treatment is
with topical steroids and oral antivirals for as long as steroids are used. Acyclovir is
recommended at 400 mg five times a day for HSV and 800 mg five times a day for
HZV. Antihypertensive treatment with timolol, brinzolamide or aproclonidine (not
latanoprost) is also required, and a mydriatic, usually atropine 1% or cyclopentolate
1% with phenylephrine 2.5%. As with all cases of AAU, OCT is very useful in docu-
menting the response to treatment if there is associated macular oedema.
Sarcoidosis is one of the conditions which can present in a host of different
ways, the other notoriously being syphilis. Although granulomatous diseases mainly
affect the posterior segment of the eye, sarcoidosis can present with AAU.
Characteristics which can differentiate it from other anterior uveitides are mutton
fat KPs and iris nodules. (One of several fairly useless facts trainees learn for the
purposes of exams is that Koeppe nodules are small and at the pupil margin and
Busacca nodules are large and more peripheral on the iris.) Rarely there may be an
associated posterior uveitis with vitreous snowballs, retinal vasculitis or optic neu-
ritis. Serum ACE levels are elevated and the management is to treat the AAU and
refer the patient to a physician for management of systemic disease. ACE is also
elevated in other pulmonary diseases.
Other conditions associated with mutton fat KPs are also granulomatous and are
TB, syphilis, sympathetic ophthalmitis and the uveitis seen in patients who develop
5.3 Uveitis 55
MS. To complete the list, Vogt Koyanagi Harada disease is included but this is
extremely rare in Europe (fewer than 20 cases in the UK).
Fuch’s heterochromic cyclitis is a very mild form of anterior uveitis which is
painless, unilateral in 90%, and seen in a white eye. There may be associated loss of
iris pigment causing heterochromia. The condition causes no symptoms other than
perhaps some floaters, and does not require any treatment. Later there may be com-
plications with cataract or glaucoma. The cause is unknown.
Behçet’s disease can present with acute anterior uveitis, often with hypopyon.
This is a rare condition in the UK although more common in the Middle East and
Asia, presenting in men aged 20 to 40. The typical ocular manifestation, however, is
retinal vasculitis and a hypercoagulability state with retinal vein occlusion. Other
features are mouth ulcers and genital ulcers. HLA B51 is associated with the condi-
tion but is not diagnostic and not helpful in UK patients.
Posner-Schlossman syndrome is a condition which presents unilaterally with
very little if any anterior chamber activity, but with high IOP giving acute symp-
toms of blurred vision sometimes with haloes. Patients are usually men aged
20–50, who become very good at recognising an attack once they have had the
condition previously. Gonioscopy is obligatory to rule out angle closure, although
the anterior chamber is typically deep and the pupil reactive although it may be
sluggish. The IOP may rise to 40–60 mmHg. The condition is thought to be a tra-
beculitis, probably secondary to CMV infection. Treatment is with topical non
steroidal anti inflammatory drops and antiglaucoma drops, such as timolol, but not
latanoprost.
Other types of uveitis present less acutely but are discussed here for
completeness.
Anterior uveitis in children does not usually present acutely. The commonest
association is with juvenile idiopathic arthritis (JIA). The child may describe float-
ers, but more often is found to have anterior chamber cellular activity in a white
painless eye, at routine ophthalmic screening of JIA patients. If present, it is treated
vigorously in conjunction with the paediatric rheumatologist, because they are at
significant risk of developing glaucoma and cataract.
Intermediate uveitis, is usually a low grade inflammation which runs a
chronic course. It may present with floaters and mild blurring in children and
young adults. The fundus features include snowbanking and snowballs in the
vitreous. It is often bilateral, and is associated with granulomatous anterior uve-
itis in MS and sarcoidosis. 70% of cases are idiopathic, 20% associated with
sarcoidosis and 10% with MS. Other systemic associations are syphilis, inflam-
matory bowel disease and Lyme disease. The term pars planitis is used only for
idiopathic cases.
Although often a low grade inflammation, 30–50% will develop cystoid macular
oedema, about 20% will develop cataract and 15% glaucoma. Treatment is either
nothing if symptoms are minimal, or steroids, either periocular or systemic if there
is macular oedema. Topical steroids are of no benefit. Beware the diagnosis in an
older patient, in whom intraocular lymphoma is a more likely diagnosis. If an older
patient presents with what appears to be posterior uveitis which is worsening despite
treatment, lymphoma needs to be ruled out by vitreous biopsy.
Posterior uveitis and retinal vasculitis often coexist. Systemic causes of retinal
vasculitis are Behcet’s disease, syphilis, SLE, TB, sarcoidosis, GPA, toxoplasmosis,
CMV, PAN, candidiasis, MS, HZV, HSV, GCA, Lyme disease, Crohn’s disease and
large cell lymphoma. Ocular diseases are Eale’s disease, ARN, Birdshot chorioreti-
nopathy and toxoplasmosis. Posterior uveitis presents with blurring, visual loss,
floaters and scotomata. Acute retinal necrosis caused by herpes virus should be
considered in patients with severe granulomatous panuveitis and severe pain, or if
after an attack of HZO. The peripheral retina appears white with marked phlebitis.
The condition should be referred as an emergency to a uveitis specialist service and
treated with acyclovir iv 10 mg/kg tid.
Toxoplasma choroiditis may present acutely if there is reactivation of a lesion
near the fovea. The patient presents with blurring of vision and floaters and the
lesion can usually be easily visualised and the toxoplasma titre on serology is ele-
vated. Treatment is with sulfadiazine 1 g qid with pyrimethamine 50 mg loading
dose and 25 mg bd and folinic acid 15 mg three times a week. Alternative treatment
regimes are clindamycin 150–300 mg qid with sulfadiazine 1 g qid, or cotrimoxa-
zole bd.
5.3.3 Inflammatory Chorioretinopathies
These are characterised by multiple whitish yellow discrete lesions at the level of
the RPE and choriocapillaris. Conditions to consider are sarcoidosis, sympathetic
ophthalmitis (Dalen-Fuch’s nodules- another piece of not very useful detail) and
large cell lymphoma. A group of poorly understood conditions which can have an
acute onset often with bilateral visual symptoms is termed unimaginatively “white
dot syndromes”. These are MEWDS (multiple evanescent white dot syndrome) and
APMPPE (acute posterior multifocal placoid pigment epitheliopathy), serpiginous
choroiditis, multifocal choroiditis with panuveitis, PIC (punctuate inner choroidi-
tis) and diffuse subretinal fibrosis. The conditions are differentiated according to
age of onset and clinical course, MEWDS for example being self limiting and
acute, and multifocal choroiditis with panuveitis is more chronic and recurrent.
Some knowledge of these conditions is helpful when referring to medical retinal
and uveitis services. APMPPE and MEWDS are similar in that they affect young
women and are self limiting. Serpiginous choroiditis affects middle aged men. PIC
affects young female myopes with acute visual loss and photopsia, whereas multi-
focal choroiditis with panuveitis (MCP) affects older myopic women. Birdshot
retinopathy is less acute in onset but may be part of this group, affecting middle
aged women and being associated with HLA A29. Before diagnosing these condi-
tions it is important to exclude granulomatous disease, notably sarcoidosis, TB and
syphilis.
5.3 Uveitis 57
White dot syndromes Site Other features Prognosis

APMPPE Bilateral Prodromal illness Self limiting
Young adult Good
Serpiginous Unilateral or Middle aged male Self limiting
choroidopathy bilateral Good unless
CRNV
MEWDS Unilateral Young female Self limiting
Good
MCP Bilateral Myopic female age CRNV
30–50
PIC Spectrum of MCP Young myopic female
Diffuse subretinal fibrosis Spectrum of MCP
Birdshot retinopathy Bilateral HLA A29 Recurrent
Female age 30–50 Relatively poor
5.3.4 Lyme Disease
NICE has recently identified areas of southern England and the Scottish Highlands
as high risk areas for developing Lyme disease. There is an expectation that the
incidence of the disease will increase very rapidly in the coming years, largely due
to climate change and warm winters allowing the organism to reproduce. The num-
ber of reported cases in the UK has increased tenfold in the last 15 years. Lyme
disease is difficult to diagnose and has been termed “the new great mimicker”.
Small mammals such as mice carry the Borrelia organism in their blood, and ticks
which bite these mammals then carry the organism to a human, bitten usually while
walking in the countryside. The ticks are very small, described as the size of a pop-
pyseed. The bite may go unnoticed but after an incubation period of 2–4 weeks but
possibly months, the patient may develop a rash which is called erythema migrans
and has a bull’s eye appearance. The patient may then complain of flu like symp-
toms with fever, arthralgia, myalgia and lethargy and sometimes confusion, menin-
geal symptoms and heart arrhythmia. Uveitis may be a manifestation of the infection.
The diagnosis is confirmed serologically but the antibody response takes a few
weeks to develop and serology will be negative if tested too early in the evolution of
the illness. Treatment is with antibiotics, either doxycycline in adults or amoxicillin
in children for a period of up to a month.
5.3.5 Medical Evaluation of Uveitis Patients
Uveitis is a large topic with many possible avenues of investigation depending on

features available in the history. A thorough systems review is necessary and a tick
box questionnaire to be completed by the patient is a time saving and useful place
to start. The information gleaned is helpful when the patient is referred on to the
uveitis clinic.
Suggested Systems Review for Uveitis Patients

CNS
Headache—sarcoidosis, HZV, PAN, TB, Lyme disease, Behcet’s disease,
VKH.
Hearing symptoms—sarcoidosis, syphilis, GPA, VKH.
Cranial neuropathy—sarcoidosis, MS, syphilis, HSV, Lyme disease.
Psychosis—SLE, Behcet’s disease, VKH.
Symptoms associated with cerebral vasculitis—HSV, HZV, syphilis, Lyme
disease, Behcet’s disease, APMPPE.
ENT
Saddle nose—syphilis, GPA, relapsing polychondritis.
Mouth ulcers—Behcet’s disease, SLE, HSV, Reiter’s, ulcerative colitis.
Sinusitis—sarcoidosis, GPA.
Salivary gland—sarcoidosis, lymphoma.
Lymphadenopathy—lymphoma, HIV, Toxoplasmosis.
GI
Diarrhoea—Crohn’s disease, ulcerative colitis, Whipple’s disease.
Jaundice, hepatosplenomegaly—Brucellosis, CMV, sarcoidosis, hepatitis.
Pulmonary
Cough, breathlessness—sarcoidosis, TB, malignancy, GPA.
Nodules/lymphadenopathy—histoplasmosis, sarcoidosis, malignancy, TB.
Genitourinary
Ulcers—Behcet’s disease, Reiter’s syndrome, syphilis.
Haematuria—GPA, PAN, SLE.
Balanitis, discharge—Reiter’s syndrome, syphilis.
Nephritis—PAN, GPA, tubulointerstitial nephritis with uveitis.
Skin
Alopecia—VKH, syphilis.
Vitiligo, poliosis—VKH.
Nodules—sarcoidosis, SLE, Crohn’s disease, ulcerative colitis.
Rash—syphilis, Lyme disease, Reiter’s syndrome, sarcoidosis, HZV,
Behcet’s disease, psoriasis, SLE.
Erythema nodosum—Behcet’s disease, sarcoidosis, AMPPE.
Musculoskeletal
Arthralgia/arthritis—Behçet’s disease, sarcoidosis, SLE, JIA, Lyme dis-
ease, syphilis, psoriasis, Reiter’s syndrome, ulcerative colitis.
Constitutional
Fever—Reiter’s syndrome, Behcet’s disease, PAN, inflammatory bowel
disease, HIV, TB, Whipple’s disease.
Night sweats—TB, malignancy, sarcoidosis.
Flu like—AMPPE, MEWDS, Lyme disease.
5.4 Recent Onset or Progressive Ophthalmoplegias 59
5.3.6 Immunosuppression
A number of new therapeutic agents for treating inflammatory conditions have been
developed in recent years, and an overview of immunosuppresants is useful.
Therapeutic class Mode of action Indication

Antimetabolites
Methotrexate Inhibits DNA synthesis Sarcoidosis
Azathioprine Alters purine metabolism JIA
Mycophenolate Inhibits purine synthesis Intermediate uveitis
Alkylating agents
Cyclophosphamide Inhibit lymphocyte proliferation GPA
Chlorambucil PAN
Severe uveitis
Inhibitors of cell signalling
Cyclosporine Inhibit T cell activity Behcet’s
Tacrolimus VKC
Biologics
Infliximab Aggressive systemic disease
Adaliimumab Uveitis
Rituximab
Interferon
iv immunoglobulin
5.4 Recent Onset or Progressive Ophthalmoplegias
Diplopia is a frequent presentation to ophthalmologists. The first thing to clarify in

the history is whether the patient is describing a monocular or a binocular phenom-
enon. In monocular diplopia, the second image is a ghost, whereas in binocular, the
two images are similar.
Monocular diplopia may be due to a tear film disturbance, uncorrected refractive
error, keratoconus, multiple pupils or iridotomy, or developing cataract.
Metamorphopsia, a symptom of macular disease may be inaccurately described by
the patient as double vision.
Binocular double vision is the symptom which causes alarm if of sudden onset
or showing progression. The cause is divided into neural and muscular or mechani-
cal. Neural causes are divided into supranuclear, internuclear, cranial nerve lesions
and decompensation of preexisting phorias. Working in collaboration with an
orthoptist, as is standard UK practice is invaluable.
History taking includes the presenting event with its time frame, progression if
any, and accompanying pain or visual disturbance and also neurological systems
questioning, and general health particularly vascular related and smoking history.
Initial recordings require eye movements testing, including pursuit, saccades, con-
vergence, and dolls head movements (vestibuloocular reflex), pupil reactions and
cranial nerve examination. Specific questioning with regard to diabetes, myasthenia

gravis, thyroid disease and pernicious anaemia is also significant.
The cranial nerve palsies will be considered first. They are responsible for over
half of acute referrals for diplopia. Lesions of the III, IV and VI cranial nerves are
relatively common in adults. VI palsy makes up about half of the cases, with IV next
in frequency and then III. The majority of nerve palsies are vascular, due to athero-
sclerosis, diabetes or not to be forgotten GCA. An ischaemic cause can be assumed
if the patient is over 50 with a history of arteriopathy, with sudden onset and stable
symptoms until recovery begins at 6 weeks to 6 months, (average 3 months for VI,
5 months for III) and no other neurological findings. III nerve palsies begin with
diplopia but ptosis develops over 24 hours. Only if a III nerve palsy is complete, i.e.
ptosis and down and out displacement of the globe, with a normal pupil, should it
be assumed to be ischaemic. In IV nerve palsy, the eye is hypertropic, with an
increase in deviation on gaze towards the unaffected side. Congenital IV nerve palsy
is not uncommon and is accompanied by a head tilt and there is no torsion of the
globe. If this decompensates later in life with diplopia, the head tilt can be recog-
nised in old photographs. VI nerve palsy is recognised as an esotropia. All nerve
palsies which are not ischaemic should be imaged urgently with MRI and if a partial
III, an MR angiogram. Other required investigations are FBC, ESR, CRP, ACE,
ANA and chest X-ray.
Non ischaemic causes of cranial nerve palsies may be trauma (frequently IV
with closed head trauma, sometimes quite minor), MS, HZO, aneurysm classically
causing a III nerve palsy accompanied by pain and pupil involvement, and intra-
cranial tumour. IV nerve palsy is very rarely due to compression or aneurysm,
unless bilateral. VI palsy may be the presentation of a meningioma or a pituitary
tumour.
Multiple cranial nerve palsies are found in cavernous sinus disease (thrombosis
or fistula) and in orbital apex disease in which the optic nerve is affected as well as
V(i), V(ii) and the sympathetic supply to the pupil giving a Horner’s syndrome.
Cavernous sinus disease is rare and presents with diplopia, pain or altered sensation
in the area of innervation of the first two divisions of the trigeminal nerve and some-
times severe headache or in carotid-cavernous sinus fistula, a whooshing sensation
in the head. Thrombosis may be secondary to infection and is very rare in the cur-
rent era of antibiotics. Fistula is also rare and presents with chemosis and pulsating
exophthalmos. 25% of cases are older women with vascular disease, and 75% are
trauma associated, sometimes surgical. The eye will look congested possibly with
arterialisation of conjunctival and episcleral vessels, and raised IOP. These cases are
investigated by MRI and are managed in conjunction with neurosurgeons if due to
tumour or aneurysm, and with physicians if secondary to infection. Bilateral mul-
tiple cranial nerve palsies are seen in meningitis, Guillain-Barré syndrome, Miller
-Fisher syndrome, pernicious anaemia and Wernicke’s encephalopathy. It is worth
revising the anatomy of the orbital apex (Fig. 5.1) and the cavernous sinus to under-
stand the pattern of signs elicited (Fig. 5.2).
Higher neurological lesions causing diplopia include internuclear ophthalmople-
gia (INO), skew deviation and supranuclear palsy. These are distinguished from
5.4 Recent Onset or Progressive Ophthalmoplegias 61
SUPERIOR ORBITAL FISSURE

LEVATOR PALPEBRASE SUPERIORIS
SUPERIOR RECTUS
LACRIMAL NERVE (V)

SUPERIOR OBLIQUE
FRONTAL NERVE (V)
IV NERVE
MEDIAL RECTUS SUPERIOR OPHTHALMIC VEIN
III NERVE SUPERIOR DIVISION

COMMON NASOCILIARY NERVE (V)
TENDINOUS RING
III NERVE INFERIOR DIVISION
OPTIC FORAMEN
LATERAL RECTUS
OPTIC NERVE
INFERIOR OPHTHALMIC VEIN
INFERIOR RECTUS
VI NERVE
OPHTHALMIC ARTERY
Fig. 5.1 Anatomy of the orbital apex
DIAPHRAGMA SELLAE
III NERVE
IV NERVE
PITUITARY GLAND
INTERNAL V(i) NERVE
CAROTID ARTERY
VI NERVE
V(ii) NERVE
SPHENOID SINUSES
Fig. 5.2 Anatomy of the cavernous sinus
nerve palsies by the finding that they affect eye movements other than vergences,
that is they affect pursuit movements, convergence and vestibuloocular movements
to different degrees depending on the site of the lesion. INO is recognised by loss of
adduction on the affected side with nystagmus on abduction contralaterally but
maintained convergence. The causes are CVA, MS, tumour, Wernicke’s encepha-
lopathy and rarely pernicious anaemia. Skew deviation is a vertical deviation not
caused by a III or IV nerve palsy. There is a vertical misalignment which changes in
different gaze positions and is due to a lesion in the brain stem or cerebellum, usu-
ally a stroke, tumour or MS.
Supranuclear ophthalmoplegia predominantly affects saccades. Vestibuloocular

movements are the last to be affected. Recognised patterns are dorsal midbrain
syndrome, caused by pinealoma, hydrocephalus, stroke or MS where there is failure
of upgaze. Horizontal saccadic palsy is due to a pontine lesion. Parkinson’s disease
is a relatively commonly encountered cause of saccadic palsy, often vertical.
Muscular and mechanical causes of diplopia include thyroid eye disease, orbital
myosotis, orbital cellulitis, trauma, orbital tumour and myasthenia gravis. An acute
onset of pain, inflammation, proptosis and diplopia can be caused also by HZO,
GCA, GPA, sarcoidosis and idiopathic orbital inflammation (encompassing condi-
tions formerly termed pseudotumour and orbital myositis) These patients require
urgent MRI as well as the relevant blood tests.
Myasthenia gravis is a relatively rare condition affecting women at average age of
onset 28 years and men at average of 42 years. 50–80% have eye symptoms at the
onset and 80% of those develop generalised symptoms within 3 years. At onset the
symptoms may develop and progress rapidly over weeks. The disease eventually
burns out. The ocular symptoms are ptosis and diplopia worse towards the end of the
day and improving after rest. The patient has an expressionless face and the eye move-
ments may mimic INO. Note that the pupil is never affected. If the diagnosis is sus-
pected direct questioning regarding skeletal muscle fatigue and difficulty with speech
breathing and swallowing are important and if features of these are present, urgent
neurological referral is required. The ice test is particularly useful in ptosis. Anti ace-
tylcholine receptor antibody is useful but is only positive in 50% of eye patients. If all
results are negative but the diagnosis is still suspected referral to a neurologist is nec-
essary, and possibly a muscle biopsy. Medical treatment is with pyridostigmine.
How to Perform the Ice Test

Photograph the patient in the primary position.
Close eyes for 2 min.
Open and rephotograph.
Close and ice pack for 2 min.
Rephotograph.
If improvement of ptosis is greater than 2 mm, the result is positive.
Thyroid eye disease does not usually present as an emergency but is included
here because rarely it can present with progressive diplopia and failing vision.
Thyroid eye disease is a lymphocytic inflammation of the orbit associated with
autoimmune thyrotoxicosis and is commoner in women but tends to be more severe
in men and is aggravated by smoking. The orbit becomes progressively congested
as the muscles hypertrophy, fat accumulates and glycosaminoglycans are laid down.
Most patients are hyperthyroid biochemically.
5.5 Transient Ischaemic Attack (TIA) 63
Classification of thyroid eye disease

Mild Lid retraction
Epiphora
Lubrication symptoms
Superior limbal keratitis
Moderate Proptosis
Diplopia
Raised IOP
Severe Corneal ulceration
Optic neuropathy
The progression of symptoms is shown in the classification. Optic neuropathy is

characterised by initially loss of contrast sensitivity then blurring and reduced VA,
a field defect, RAPD, and disc oedema. Visual loss is due to ION or arterial
occlusion. Management involves controlling the thyroid status. Smokers should be
encouraged to stop as there is a strong link between smoking and disease activity.
Lubricants and non steroidal anti inflammatories are helpful for mild cases, immu-
nosuppression with steroids, steroid sparing drugs or biologics are required for
moderate cases, while severe disease requires high dose steroid, 500 mg/day meth-
ylprednisolone iv for 3 days, possibly combined with low dose external beam irra-
diation. There is a delay before the radiation has an effect and therefore it is of no
value alone. If this treatment is not seen to be sufficiently effective, surgical decom-
pression of the orbit is necessary in an attempt to avoid irreversible visual loss.
Decompensation of a childhood squint can present later in life with acute onset
diplopia. This is particularly the case in patients with a congenital IV nerve palsy
who may experience diplopia when tired. Decompensation also occurs after an ill-
ness or trauma. Older patients may develop diplopia secondary to atrophy of orbital
fat and degeneration of connective tissue. This is described as the “Sagging eye
syndrome” and is the probable cause of the very common referral of older patients
who are found by the optometrist to have muscle imbalance requiring prism correc-
tion, but with no neurological cause.
5.5 Transient Ischaemic Attack (TIA)
Amaurosis fugax is caused by cerebrovascular ischaemia, GCA or retinal arterial

emboli. The last are common and the embolus arises in the heart especially if there
is atrial fibrillation, or in the carorid arteries. Other causes of transient visual loss
are compressive optic neuropathy as in Grave’s disease, and raised intracranial pres-
sure with papilloedema. Migraine may also cause transient visual loss. Vertebrobasilar
insufficiency causes a transient visual disturbance which is bilateral. TIA is defined
as symptoms lasting less than 24 hours. Amaurosis fugax is usually described as the

vision failing in an altitudinal pattern over a few seconds or by coming in from the
periphery.
Examination may reveal an arterial embolus, a cardiac arrhythmia or a carotid
bruit and hypertension or glycosuria. Patients are usually elderly and require a med-
ical assessment for the risk of stroke including Doppler ultrasound of the carotids.
Patients should be referred to a stroke clinic according to the proforma in Fig. 3.1.
In younger patients a full blood count, antibody tests for connective tissue disease
and coagulopathy screening may be indicated. This is covered more fully in Chap.
7.1, retinal vein occlusion.
Further Reading
de Parisot A, Kodjikian L, Errera MH, et al. Randomised controlled trial evaluating a standardised
strategy for uveitis etiologic diagnosis ULISSE. Am J Ophthalmol. 2017;178:176–85.
NICE. EyeCalm for supporting diagnosis and management of dry eye syndrome. NICE Guidance
and advice list. Medtech innovation briefings. London: National Institute for Health and Care
Excellence; n.d.
Meduru A, Grenga PL, Kaufman SC. Herpes zoster ophthalmicus. Expert Rev Ophthalmol.
2009;4:537–45.
Palioura S, Henry CR, Amescua G, Alfonso EC. Role of steroids in the treatment of bacterial kera-
titis. Clin Ophthalmol. 2016;10:179–86.
Pane A, Miller NR, Burdon M. The neuro-ophthalmology survival guide. 2nd ed. Amsterdam:
Elsevier; 2018.
Rutar T, Demer JL. “Heavy Eye” syndrome in the absence of high myopia: a connective tissue
degeneration in elderly strabismic patients. J AAPOS. 2009;13:36–41.
Srinivasan M, Mascarenhas J, Rajaraman R, et al. Corticosteroids for bacterial keratitis: the steroid
for corneal ulcers trial (SCUT). Arch Ophthalmol. 2012;130:143–50.
White ML, Chodosh J. Herpes simplex virus keratitis: a treatment guideline. Washington, DC:
American Academy of Ophthalmology Compendium of Evidence-Based Eye Care; 2014.
Chapter 6
Rapid Access: Neurology
This part of this text covers some common areas of referral to the ophthalmologist.
Different referral patterns exist in different hospitals depending on the availability
of sub specialists and access to those services in urgent circumstances. Most if not
all UK departments now have a direct access service for acute macular disease and
this topic is not covered. Optometrists or community ophthalmologists recognise
the symptoms of sudden onset distortion of central vision, usually in elderly patients
and would refer them directly for urgent OCT, and appropriate management of age
related macular disease. Referral guidance protocols are in widespread use.
Some of the topics relevant to rapid access have been covered in the acute section
to avoid disjointed discussion.
6.1 Headache
Headache is a frequent presentation to the eye department. Very often headaches are
centred on the eye, orbit and periorbital area and it takes some skill to persuade
some patients that their symptom is not ophthalmological. Obviously if there are
ophthalmic symptoms or signs, there is less of a diagnostic problem.
Headaches are classified as primary or secondary, primary being those with nor-
mal neurological, including ophthalmological, examination and if indicated investi-
gation. The recommendation is NOT to order imaging investigations except in “red
flag” cases as incidental findings which are fairly common, may lead to further
unanswered questions rather than allaying anxiety. The practice of offering imaging
to anxious patients to eliminate their anxiety is therefore to be avoided. In various
studies of healthy individuals, an incidental finding of an abnormality occurs in
upwards of 10% of scans, with the incidence rising with age.
History taking is a key element in reaching a diagnosis in a headache patient and
requires time, particularly as patients with headache may have had unrewarding
encounters with other health professionals or specialties. The exact nature of the

https://doi.org/10.1007/978-3-319-92369-7_6
66 6 Rapid Access: Neurology
headache must be teased out with particular emphasis on the time frame and pattern.
How long has the patient had symptoms? Why is the patient presenting now? Is the
headache constant or episodic? If episodic, for how long does each episode last and
what is the interval between episodes? Is it diurnal? Does it disturb sleep? Does it
interfere with activities? Is there associated nausea or vomiting? Are there any
visual symptoms including diplopia, blurring of vision, altered colour vision, obscu-
rations, loss of visual field or migrainous aura? What makes it better? What makes
it worse? Is the pattern getting worse or better over time? Is there pulsatile tinnitus
or any neurological deficit?
A systematic enquiry and past medical history will reveal coexisting diagnoses
such as malignancy, or identify the symptoms of giant cell arteritis, and a family
history of migraine may be relevant. Drug history including illegal drugs, and alco-
hol intake are also significant.
Examination includes a blood pressure check as occasionally patients with
accelerated hypertension present through the eye department. Examination of the
eye will identify loss of visual acuity or visual field. Colour vision testing and
pupil responses may point to an optic nerve problem. Inflammatory disease and
acute glaucoma can be identified at this point as the cause of the headache and
examination of the optic nerve itself reveals the presence or absence of disc
swelling.
6.1.1 Primary Headache
Migraine is the commonest severe primary headache. The pain is usually recurrent,
unilateral, pulsating, associated with nausea and photophobia, aggravated by activ-
ity and disabling. There may be visual aura in the form of zigzags, flashes or dots,
usually bilaterally. Retinal migraine however is a rarer uniocular presentation of
visual loss in one eye only. In some patients the aura occur without a headache fol-
lowing on and these as well as the ones with pain centred on the eyeball are the
patients who may present initially to the ophthalmologist as well as those with neu-
rological manifestations of migraine such as transient field loss or nerve palsies.
The clue is the evolution of the symptoms, which progress over 5 or more minutes,
then resolve within an hour, and may be followed within an hour by a headache.
Aura are specifically defined by the International Headache Society as a recurrent
disorder developing over 5–29 minutes and lasting less than 1 hour. Once the diag-
nosis is made the long term management lies in the hands of the general practitio-
ner, or if severe, the neurologist.
Tension headaches are commoner than migraine but less severe, bilateral, often
described as a band round the head and do not usually affect the ability to continue
with activity.
Trigeminal autonomic cephalgias are rare and are associated with autonomic
signs such as meiosis, ptosis and tearing. Cluster headaches fall into this group, all
of which should be referred to a neurologist for management.
6.1 Headache 67
Medication overuse headache is recognised as a significant problem and the

patient may need to consider this as an element of their symptom.
The role of the ophthalmologist in the care of patients with primary headache is
to exclude secondary headache causes, and to identify the symptoms of ophthalmic
migraine which may be quite alarming on the first occasion. If primary headache is
the diagnosis the patient should be referred back to their general practitioner who
may wish the advice of a neurologist.
6.1.2 Secondary Headache
Red Flag Headaches

Postural
Caused by Valsalva manoeuvre (not just aggravated)
Wakens from sleep
New onset over 50 years
Thunderclap
Focal neurological symptoms
Non-focal neurological symptoms
Risk factors for cerebral vein thrombosis
Oestrogen contraception
Pregnancy
Hypercoagulability
Dehydration
History of malignant disease
There are a number of red flag features which are elicited from the history and must
be recognised. Headache which is worse on lying down may be due to raised intra-
cranial pressure, as is headache caused by the Valsalva manoeuvre. Note that the
headache is brought on by straining, not aggravated by it, as happens with most
headaches including primary headaches.
The history of giant cell arteritis cannot be overemphasised. This has been cov-
ered in Sect. 4.4, but the principal features to elicit are visual disturbance, jaw clau-
dication, fever malaise and weight loss. General malaise, weight loss and loss of
appetite are also indications of advanced malignancy with the possibility of second-
ary intracranial deposits.
Raised intracranial pressure (ICP) is associated with headache and is recognised
by the ophthalmologist by the presence of disc swelling. Specific headache types
associated with raised ICP are thunderclap, the headache of venous sinus thrombo-
sis and idiopathic intracranial hypertension (IIH). Headache is very rarely the sole
presenting symptom (<0.2%) of intracranial tumour but is a presenting symptom in
combination with another feature in many cases.
Thunderclap headache is the term reserved for the very severe headache which
reaches maximum intensity usually within 1 minute, but always within 5, and which
is associated with subarachnoid haemorrhage, carotid dissection, intracranial
venous sinus thrombosis or bleeding into a pituitary tumour, termed pituitary apo-
plexy. If there are accompanying visual symptoms, especially in pituitary apoplexy,
but sometimes in carotid dissection, the patient may be directed to the eye emer-
gency department in the first instance.
Although intracranial venous sinus thrombosis is one of the causes of thunder-
clap headache, more commonly the headache builds up over a few days and the
presentation is with neurological signs often mimicking a cerebrovascular accident.
The risk factors are an oestrogen containing contraceptive pill, pregnancy, dehydra-
tion and hypercoagulability states (see section on TIA, Chap. 3.1).
Drugs, both prescribed and recreational, can be a cause of headache. Very many
prescription drugs can cause headache, but tetracyclines including minocycline
used for the treatment of acne, and retinoids including vitamin A also used for treat-
ing skin conditions are associated with disc swelling and raised intracranial pres-
sure, producing a presentation very similar to that of IIH. These cases clearly would
be managed by stopping the medication provoking the response. Medication over-
use headache is the term used for the headache affecting patients who take excessive
quantities of oral analgesia. It is defined as a headache which is present for 15 or
more days in a month, and which has developed or been aggravated by excessive
use of analgesics. It is managed by weaning off the medication.
IIH typically affects obese women aged 15–45, often with a history of recent
weight change either up or down. They describe a headache with the characteristics
associated with raised intracranial pressure, progressing over weeks, and may also
describe visual obscurations, pulsatile tinnitus, and less frequently diplopia which is
found to be due to VI nerve palsy. Examination reveals disc swelling and there may
be an enlarged blind spot on visual field analysis. MRI of the brain is required to
exclude an intracranial mass lesion or Chiari malformation, and an MR venogram
to identify venous sinus thrombosis. A well performed lumbar puncture is necessary
to show the elevated opening pressure greater than 20 cm H2O. This may be chal-
lenging in obese patients and can be done with radiological guidance. The manage-
ment of IIH is shared between the neurophthalmologist and the neurologist but the
cornerstone of treatment is weight control.
Chiari malformation is fairly commonly reported as an incidental feature on an
MRI. This is a malformation in which the cerebellar tonsil protrudes through the
foramen magnum. Type I is associated with headache, but in the adult population is
usually an incidental finding. If detected in children, they should be referred to a
paediatric neurosuregeon for a management opinion.
Another fairly common incidental finding on an MRI is a deficient diaphragm sellae
with empty sella syndrome. This is associated with IIH but is usually of no significance.
If it causes symptoms, these may be related to downward displacement of the optic
chiasm causing field defects, or to reduced pituitary function and endocrine symptoms.
A history of recent head injury may point to a chronic subdural haematoma. The
patient may have no other symptoms and this is an easy diagnosis to miss as there
is no disc swelling. The pain is thought to be due to meningeal stretching rather than
raised intracranial pressure. Only later if the haematoma increases in size will the
6.2 Papilloedema and Disc Swelling 69
diagnosis be evident if the patient becomes drowsy or confused, prompting further

investigation with imaging.
Headache may also commonly be due to referred pain from musculoskeletal
abnormalities in the neck.
6.2 Papilloedema and Disc Swelling
The term papilloedema is defined by neurologists as disc swelling caused by raised

intracranial pressure, while all other types are referred to as disc swelling. This is
not a helpful distinction as clearly the cause of the swelling cannot be identified by
the appearance. Careful disc examination reveals features other than those described
in the Frisen classification. The earliest feature is disc hyperaemia, followed by blur-
ring of the nasal edge with obscuration of the finest vessels. Small nerve fibre layer
haemorrhages are most easily detected with red free light. Venous pulsation is usu-
ally described but is not particularly helpful because it is absent in 20% of normal
eyes. Late features are venous congestion, more peripapillary haemorrhages and
cotton wool spots and then retinal and choroidal folds. Occasionally, in difficult
cases, particularly when there is a question of Leber’s optic neuropathy swelling of
the optic nerve can be demonstrated by fluorescein angiography, with dilatation of
the peripapillary vessels and late leakage, examined at 30 min after fluorescein
injection, which is absent in Leber’s optic neuropathy. The differentiation between
swelling caused by raised ICP and other causes, those being inflammatory, vascular,
infiltrative or infective, is said to be that the visual acuity is unaffected in raised
ICP. However ophthalmologists recognise that this is misleading as for example, IIH
is associated with visual obscurations, and intracranial tumours cause visual dys-
function through various field defects, or later because of secondary optic atrophy.
Ophthalmologists are often called upon to confirm the presence of disc swelling,
although with increasing access to brain imaging, this role is less important than in
the past. Nevertheless, frequently patients, particularly children, are referred with
“funny discs”, having attended a routine optometry examination. Although they are
asymptomatic patients, on direct questioning by the optometrist, a history of head-
ache is elicited and alarm bells ring, causing variable and understandable amounts
of anxiety in patients and parents of children. Such patients have to be seen in rapid
access services although very infrequently is there serious disease. The disc may be
crowded, tilted, have buried drusen, or indeed be swollen.
Frisen Scale for Grading Papilloedema

Stage 1: nasal halo of disc blurring
Stage 2: ircumferential halo of swelling. Cup maintained
Stage 3: elevation of disc with obscuration of one or more segments of blood
vessel at disc margin
Stage 4: almost complete obscuration of major blood vessels on disc
Stage 5: partial or total obscuration of all disc vessels
In the rapid access clinic such patients require a recording of corrected visual
acuity together with a note of the refractive error, colour vision testing using Ishihara
plates or a similar screening method, recording of pupil responses and if possible
visual field analysis. Dilated fundus examination is required, as in adult patients
media opacities may impair accurate disc assessment, and in children the inability
to maintain fixation means that an adequate view cannot be guaranteed through an
undilated pupil.
The definitive differentiation of early disc swelling and optic nerve head drusen
is a topic of considerable debate, particularly in children. Optic nerve head drusen
occur in about 2% of the population and evolve from childhood when they are small
non calcified and deeply buried. They increase in size slowly, migrate anteriorly and
become calcified. When they are calcified and migrate to the surface of the nerve
they are relatively easy to diagnose by direct visualisation, CT scan (best avoided in
children because of the significant radiation dose), autofluorescence and B scan
ultrasound. They are not therefore a significant diagnostic problem in adults. When
they are small, deep and non calcified, they may give rise to disc elevation but be
very difficult to image. The definitive investigation is fluorescein angiography which
shows late disc leakage in disc swelling but not in pseudopapilloedema with drusen.
However fluorescein angiography is not a practical option for children in most
departments. OCT with an enhanced depth transverse view (Fig. 6.1) and a circum-
ferential thickness measurement (Fig. 6.2) and autofluorescence of the disc (Fig. 6.3)
are the preferred investigations for detecting buried drusen but are not either highly
sensitive or highly specific. Various descriptions have been published showing char-
Fig. 6.1 OCT optic nerve transverse scan of an 18 year old patient showing drusen outlined
Fig. 6.2 OCT of same patient as in Fig 6.1 showing that the nasal nerve fibre layer thickness is
within normal limits despite the swollen appearance of the disc
Fig. 6.3 Autofluorescent image of the discs of a 14 year old showing bilateral but asymmetric
drusen
Fig. 6.4 The same patient as in Fig 6.3 showing drusen (dotted line) protruding laterally creating
a “boot shaped” SHS (solid line)
Fig. 6.5 OCT in raised ICP showing absence of SHS despite advanced disc swelling
acteristic findings but the interpretation remains difficult. Drusen are said to be
hyperreflective and create a recognisable “boot shaped “subretinal hyporeflective
space (SHS) (Fig. 6.4) because of the presence of a nodular structure extruding from
the nerve towards the subretinal space. This is not seen in disc swelling (Fig. 6.5).
This is the case if the druse lies in that position but not if the druse is within the
nerve structure and the amount of swelling minimal. Sometimes the druse may have
a granular structure, possibly representing calcification (Fig. 6.6) or can be hypore-
flective. The feature of the SHS associated with disc swelling is only identifiable in
relatively advanced disc swelling when other clinical features make the diagnosis
easier. An additional sign described in raised intracranial pressure producing disc
swelling is that the points of termination of Bruch’s membrane and the RPE are
displaced anteriorly, whereas in disc swelling of other causes such as optic neuritis
or ischaemic optic neuropathy, as well as in drusen, the ends of Bruch’s membrane
remain aligned across the optic nerve viewed in transverse section. This is not a
particularly useful finding in the opinion of the author probably because the intra-
cranial pressure has to be very high to produce the effect, and the diagnosis is evi-
Fig. 6.6 OCT of a 37 year old patient with granular appearing drusen, outlined
Fig. 6.7 Progressing disc swelling in IIH showing progression of thickening of NFL particularly
nasally between two scans
dent from other signs. Nerve fibre thickness analysis may be helpful in that in early
disc swelling the nerve fibre layer is thickened but normal in drusen patients. This is
said to be more specific if the nasal margin is examined rather than the temporal, but
again not highly sensitive. This finding is more helpful if applied to serial examina-
tions (Fig. 6.7) which would be done in the presence of symptoms in a neurooph-
thalmology clinic, but not usually indicated in the rapid access setting in
asymptomatic patients. B scan ultrasound only detects calcified drusen which can be
easily visualised by autofluorescence, and does not add much to the investigation.
In children in whom there is doubt as to the interpretation of the disc findings, an
MRI is indicated, with strong reassurance to the parents that this is extremely likely
to be normal. Very rarely if ever is a significant intracranial lesion detected in an
asymptomatic patient. Vague symptoms such as insignificant headache may be a
result of suggestibility following the optometrist’s examination.
Newly developing techniques of OCT angiography may earn a place in the dif-
ferentiation of disc oedema from pseudo disc oedema, helping to end this
controversy.
The abnormality of disc morphology caused by drusen is termed pseudopapill-
oedema. Crowded discs seen in hypermetropes are the other main cause of
pseudopapilloedema.
Characteristics of drusen
Elevated irregular surface
Blurred disc margin
Obliteration of cup
Vessels—no obscuration but anomalous branching sometimes present
Field—enlarged blind spot or bundle defect
OCT circumferential scan may show nerve fibre layer thinning
OCT transverse scan may show obliteration of the cup and surface distur-
bance (usually due to blood vessels) or a deep profile. Sometimes “boot
shaped” SHS
May demonstrate autofluorescence
Characteristics of true disc swelling
Blurred nerve fibre layer with obscuration of vessels at disc margin.
Dilated veins
Flame shaped haemorrhages
Cup not obliterated
OCT thickening of nerve fibre layer on circumferential scan, most obvious on
nasal side
6.3 Subacute or Self limiting Angle Closure
Patients with headache are sometimes suspected of having subacute angle closure
glaucoma. Patients often become worried about their risk and relate this to a family
history of glaucoma which is most often chronic open angle glaucoma. Optometrists
could easily dispel this fear by explaining that the two types of glaucoma are related
to each other only by name, and by taking a short history of the condition in the
family member. If pain is not an element, acute angle closure is not the diagnosis.
6.3 Subacute or Self limiting Angle Closure 75
Fig. 6.8 Anterior segment OCT showing closeable angle
This seems very obvious but is rarely explained adequately to the anxious patient.
Angle closure can be suspected, intermittent, acute or chronic.
If headache is indeed due to intermittent angle closure, the history will be very
specific of headache coming on in the evening, and settling down over night and
then recurring in a pattern in the evenings. Many people with fairly shallow anterior
chambers and intermittently red eyes get headaches but few of them have intermit-
tent angle closure. Useful signs of previous episodes of self limiting angle closure
are patches of iris atrophy, radial fibre whorling on the iris, and glaukomflecken
(focal necrosis of the anterior lens capsule seen as small white opacities). The essen-
tial examination is gonioscopy. The inferior angle is nearly always open. The angle
is classified as closeable if more than 180° of trabecular meshwork is obscured.
Anterior segment OCT is useful to demonstrate the configuration of the iris inser-
tion as well as to help in explaining the condition to the patient (Fig. 6.8). The iris
may either be displaced forwards to narrow the angle or only the peripheral part of
the iris may be displaced giving the configuration of plateau iris, which is more
often the cause of an angle closure in younger patients (30–50 years).
The EAGLE study is cited in this circumstance to advocate phacoemulsification
rather than YAG laser iridotomy as a prophylactic procedure in patients who may
develop acute angle closure. The study shows that phacoemulsification is a more
effective treatment than YAG iridotomy in primary angle closure glaucoma. This
has been extrapolated to include potentially closable angles. YAG iridotomy carries
with it a risk of accelerating the development of cataract as well as causing inflam-
mation and potentially retinal detachment. The optical side effects are significant in
many patients, and in patients suspected of having subacute attacks of glaucoma,
lens extraction is perhaps the best advice, whereas in patients with narrow angles,
information about symptoms of acute angle closure with advice to seek a medical
opinion early is the preferred management.
Van Herrick’s Method of Assessing Drainage Angle

Narrow slit focussed on limbus at an angle of 60°
Estimate the width of the gap between posterior cornea and iris as a frac-
tion of the corneal thickness
Grade 4 Equal to cornea Wide open

Grade 3 ¼ to ½ Open
Grade 2 ¼ Narrow
Grade 1 <1/4 Closable
6.4 Neurological Visual Field defect
I nterpreting visual fields can be difficult and optometrists often refer patients with field
defects. If the defect falls into a particular recognised pattern, there is no problem, but
more often than not there are uncertainties. Theoretical visual field defects are easy to
describe but the added factors of patient understanding cooperation and concentration
and the technician’s attention to detail lead to artefacts. In theory, a defect in one eye
can only be caused by a prechiasmal lesion, if the defect is crossed, the lesion is in the
chiasm, and if it is homonomous it is post chiasmal. Anterior to the chiasm, the defects
may be altitudinal due to vascular causes, or central and centrocaecal (spreading to
include the blind spot) in macular lesions and optic neuropathies. Homonomous means
the same side of the field in each eye whereas congruous means the same shape in the
two visual fields. The more congruous a defect, the further back it is in the visual path-
way. Macular sparing is the rule in occipital lesions, although difficult to demonstrate.
Bitemporal defects are associated with pituitary defects, and begin in the superior quad-
rant. Binasal defects are unusual and could theoretically be caused by bilateral carotid
expansion. Usually the appearance of a binasal defect is an early bilateral arcuate sco-
toma due to glaucoma. A junctional scotoma is a central scotoma on one side and a
superotemporal defect on the other, indicating an anterior chiasmal lesion on the side of
the central scotoma. Intellectually, this information is interesting but with the detailed
features available using neuroimaging techniques, usually MRI, locating a lesion is not
particularly valuable and is included for completeness and as a reminder for teaching.
When interpreting field charts, first the quality of the investigation should be estab-
lished. The quality of fixation is important for reliability. This is poor in uncooperative
or poorly concentrating subjects or in “trigger happy” patients or if the blindspot has
been wrongly plotted. If the patient has poor central vision, the fixation point can be
adjusted. Fixation loss of greater than 20% means the result is unreliable. False nega-
tives are registered when a suprathreshold stimulus is presented again in an area
already eliciting a threshold response, but not recorded on a subsequent occasion. With
false negatives, the plot gives a result worse than it should be. False positives occur
when the patient responds to the sound of the target being moved. If the target is
moved but the stimulus not excited and the patient responds, this is a false positive.
Some of the causes of erroneous field plots are ptosis, dermatochalasis, poor head
positioning with the subject falling back from the head rest, and interference from the
trial frame often combined with a bad head position. Media opacities show up on the
sensitivity (total deviation) plot but do not affect the pattern deviation which should
show scotomata if present. A tilted disc can give the appearance of bitemporal field loss.
Malingering and conversion disorder (formerly called hysteria) can manifest as
visual field defects. Until there is proof of a visual response incompatible with the
field defect, functional causes should not be diagnosed. There are various supple-
mentary tests which orthoptists and optometrists are particularly good at, which can
prove a visual response. The field may present as a cloverleaf pattern, associated
with a high false negative rate. The cloverleaf pattern occurs because of the way the
computer programme tests the peripheral field. In such cases a Goldmann field may
show crossing isopters, crowded isopters or spiralling. A patient claiming to be
totally blind, if asked to sign his name can still do so if the cause is organic but will
6.4 Neurological Visual Field defect 77
probably be incapable if malingering. Optokinetic nystagmus is an involuntary

response which can be elicited if there is vision present. In unilateral blindness,
lenses in a trial frame can be manipulated to confuse the patient and elicit a response,
and similarly prisms may elicit a binocular response to diplopia, proving bilateral
visual function. These patients can be difficult to manage. In children there may be
a clear cause of psychological distress such as domestic upset or unhappiness at
school and referral to mental health services is helpful. In all cases it is worth reas-
suring the patient that their eyes are not seriously diseased and that you expect them
to recover within a period of weeks when review should be planned.
The organic causes of field loss require to be investigated according to the site of
the lesion, be it retina, optic nerve, or brain, with early onward referral to the appro-
priate specialty, preferably with initial investigations ordered.
For completeness, the essential normal values for visual fields and the field test-
ing programmes commonly used on the Humphrey analyser are given here.
Visual Field Programmes

Normal field 130° vertical 160° horizontal, from fixation up 60, temporal 100,
inferior 70, 60 nasal. Blind spot 12–15° temporal to fixation (Fig. 6.9).
10-2: central 10°
24-2: 24° temporal 30° nasal
30-2: 30° temporal and nasal
120 point: 120° full field used for neurological cases
All can be SITA fast- faster than SITA standard and slightly less sensitive.
SITA is a threshold test. Standard takes 7 min per eye, fast 4 min per eye
130˚
BLIND SPOT
60°
160°
N T
100°
60°
70°
Fig. 6.9 Normal visual field

6.5 Optic Neuritis
Having discussed disc swelling from intracranial cause, with at least initially nor-
mal acuity, the next area to consider is disc swelling with accompanying visual
defect. The most common presentation of optic neuritis is in women under 45 years
who present with progressive blurring of vision, usually unilaterally, typically with
a washed out perception of colour and pain on moving the eye. The onset is fairly
rapid and progressive over 2 weeks. There may be a history of a preceding viral ill-
ness. The visual loss may be exacerbated by heat or exercise (Uhthoff’s phenome-
non). Examination reveals reduced visual acuity of any degree from mild to
profound, a relative afferent pupillary defect, desaturation of colour best tested with
a red target and pain on moving the eye. The optic nerve head may or may not
appear swollen but if swollen there are no haemorrhages or exudates. There is usu-
ally a central or centrocaecal field defect but other patterns of defect are often seen.
The patient needs to be informed that the vision may deteriorate further before it
begins to improve but the expectation is that it will improve, and review in 6 weeks
is appropriate. No treatment is required. OCT of the nerve may be useful in record-
ing the evolution of the condition. MRI is not necessarily indicated but if ordered
may show periventricular white matter plaques. A visually evoked potential (VEP)
is useful to confirm the diagnosis of demyelination which shows a characteristic
delay in the P100. In the acute phase the P100 may be unrecordable but if present is
delayed, and the delay persists after symptomatic recovery. The patient should be
reviewed at about a month to confirm that the vision is following the expected pat-
tern of decline followed by recovery.
There may already be a diagnosis of a demyelinating disease or a neurological
deficit but optic neuritis is the presenting symptom in 25% of cases of MS. Some
cases of optic neuritis are unrelated to MS, and can occur after a viral illness or in
association with acute sinusitis. Only if the patient has had a previous episode,
another neurological symptom or atypical MRI lesion, need demyelination be dis-
cussed. The optic neuritis treatment trial found good recovery in 93% of cases
whether or not they were treated. Oral prednisolone alone was found to be harmful
in that it increased the risk of recurrence. However 1 g of intravenous methylpred-
nisolone daily (250 mg qid) for 3 days followed by 11 days of oral prednisolone
1 mg/kg daily speeds up recovery but has no effect on the long term outcome or
recurrence rate. The prognosis for a first attack is for 2/3 of women and 1/3 of men
to develop MS.
Optic neuritis can present in children when it is usually bilateral and is said to
carry a lesser risk of being a manifestation of MS and is thought to be post viral. The
advice is to treat children with intravenous methylprednisolone 1–2 mg/kg/day.
Neuromyelitis optica (NMO) must be differentiated from MS as it is much more
severe and is potentially fatal. The patient may have features of unilateral or bilat-
eral optic neuritis with transverse myelitis either preceding, occurring together with
or following the eye symptoms within a shorter timescale than seen in MS. The
aquaporin 4 antibody test is positive in most cases of NMO, but if a patient with
6.5 Optic Neuritis 79
optic neuritis is not improving in the expected time scale, the diagnosis of NMO
should be considered with urgent referral to a neurologist.
The differential diagnosis of a swollen disc with visual loss includes typical optic
neuritis, ischaemic optic neuropathy and pituitary tumour, and more rarely, sarcoid-
osis, lymphomatous infiltration, Leber’s optic atrophy, and optic nerve sheath
meningioma. If there is evidence of infection, the differential includes cat scratch
disease, sphenoid sinusitis and orbital cellulitis. Other considerations in the absence
of swelling are toxic optic neuropathy and functional visual loss. Sometimes the
swollen nerve is associated with stellate neuroretinitis in which there is a macular
star of hard exudates. This is not indicative of any aetiology of the optic neuritis
although is seen more often with infective causes and sarcoidosis, and is well rec-
ognised in accelerated hypertension.
Swollen Disc with Visual Loss

Typical optic neuritis
Ischaemic optic neuropathy
Pituitary tumour
Sarcoidosis
Lymphoma
Leber’s optic neuropathy
Optic nerve sheath meningioma
Cat scratch disease
Sphenoid sinusitis
Orbital cellulitis
Toxic optic neuropathy. This is a rather poorly understood collection of condi-

tions. Toxicity and nutritional deficiency may act independently or in combination.
Deficiency of Vitamin B12, other vitamin B complex vitamins (B1, riboflavin and
others) and folic acid occurs in pernicious anaemia and malabsorption syndromes
(occurring more frequently with increasing numbers of patients having bariatric
surgery) as well as in the undernourished. Tobacco may have a direct toxic effect on
the optic nerve. Alcohol is probably not toxic but leads to a lifestyle of poor nutri-
tion. Other drugs, fumes and solvents may be implicated. Patients present with a
progressive deterioration in central vision bilaterally. The presentation is acute and
severe in methanol poisoning. A number of therapeutic agents, most notably etham-
butol and amiodarone are known to have toxic effects on the optic nerve. On exami-
nation there is a colour vision defect, sluggish pupil responses and initially a
hyperaemic disc, progressing to pallor. The drug related causes sometimes improve
with withdrawal of the drug. Nutritional amblyopia responds to varying degrees to
improved nutrition, multivitamin supplements especially B vitamins and folate, and
cessation of smoking and drinking. Vitamin B12 injections appear to be beneficial
even if the blood levels recorded are in the normal range. This may be because vita-
min B12 is abnormally bound to transcobalamin, giving a normal blood level but a
reduced availability to the tissues. Investigations depend on the details of the pre-
sentation but an exhaustive list is shown in the box.
Investigations for Optic Neuropathy

MRI optic nerve and brain
ESR, CRP
ACE
ANA (non specific indicator of autoimmune disease)
Syphilis serology
NMOAb, Aquaporin 4
B12, folate
Leber’s optic atrophy genetic test (mitochondrial DNA)
Cat scratch PCR
Lyme disease serology
TB testing (tuberculin skin test or quantiferon blood test)
CXR (for sarcoidosis and TB)
6.5.1 Afferent Pupil Defect
Despite this being a very standard higher exam question for ophthalmologists, many
are still hazy about the testing of pupil responses. The anatomy and physiology is
worth revision.
A Revision of Pupil Testing

Innervation; Iris sphincter controlled by parasympathetic, dilator by sympathetic.
Parasympathetic innervations is carried from the Edinger Westphal nucleus
in the III nerve nuclear complex and travels with III nerve through the cavern-
ous sinus to the inferior division of III nerve in the orbit, synapsing in the cili-
ary ganglion. It is then relayed in the short ciliary nerves to the sphincter
pupillae muscle (Fig. 6.10).
Sympathetic supply follows the internal carotid artery from the superior
cervical ganglion. In the cavernous sinus the sympathetic supply joins the V
nerve and enters the orbit in the superior orbital fissure in the nasociliary
nerve (Fig. 6.11).
Pupil light reflex. Rods and cones synapse with ganglion cells. Axons pass
along the optic nerve and pupillary fibres leave before the lateral geniculate
ganglion to go to the superior colliculus and synapse in the pretectal nuclei
and then pass bilaterally to the Edinger-Westphal nucleus, part of the III nerve
complex. The efferent arm is in the pupillary fibres in III nerve.
6.5 Optic Neuritis 81
An afferent pupil defect (APD) is present when no pupil response either

direct or consensual is observed when a light is shone in the bad eye but there
is a consensual response when light is shone in the good eye.
A relative afferent pupil defect (RAPD) demonstrates that one optic nerve
is less responsive than the other. Light shone in either eye gives a direct and
consensual response. If the light is shone in the good eye, both pupils con-
strict, but if the light swings to affected eye, it dilates because the strength of
the stimulus in that eye is reduced. This is the swinging torchlight test. An
RAPD can be shown even if the affected eye has an immobile pupil, as when
the light swings to the affected eye the pupil of the unaffected eye will dilate.
EDINGER - WESTPHAL
NUCLEUS III NERVE NUCLEUS LEVATOR PALPEBRAE
SUPERIORIS
INF DIVISION III

NERVE SPHINCTER
MUSCLE
CILIARY NERVE
CILIARY GANGLION
Fig. 6.10 Diagram of parasympathetic innervation of the pupil
LEVATOR PALPEBRAE
SUPERIORIS
DILATOR MUSCLE
V (I) NERVE NASOCILIARY NERVE
SYMPATHETIC
PLEXUS
INTERNAL CAROTID
ARTERY
SUPERIOR CERVICAL
GANGLION
Fig. 6.11 Diagram of sympathetic innervation of the pupil

6.6 Anisocoria
Anisocoria may present as a new phenomenon, or may be reported as new by an

inexperienced optometrist. It is very often physiological, in which case the differ-
ence between the pupils is the same in the light and in the dark. If the difference is
greater in the dark, the diagnosis to exclude is Horner’s syndrome, which may be
accompanied by slight ptosis and lack of sweating on the same side of the face. The
diagnosis can be confirmed if necessary using aproclonidine 0.5%, an alpha agonist
to which the nerve endings are hypersensitive causing dilatation, but no effect on the
pupil of the fellow eye. The drops take 30–40 min to take effect and also reverse the
ptosis. (Formerly cocaine drops which dilate a normal pupil but not a Horner’s
pupil, were used and are still considered the gold standard, but are less accessible in
the general clinic setting.) Horner’s syndrome can be congenital in which case the
iris on the affected side is lighter in colour, but if of recent origin, imaging of the
lung apex and neck are indicated. Dissection of the aorta is easily missed.
If the anisocoria is worse in the light, Holmes-Adie pupil has to be differentiated
from an acquired III nerve lesion. In Holmes-Adie pupil, constriction to accommoda-
tion is preserved, but the abnormal innervation can be demonstrated using 0.1% pilo-
carpine which produces rapid constriction, reversing the anisocoria. Holmes-Adie
pupil is usually diagnosed in early adult life and is thought to be a post viral phenom-
enon. III nerve palsy if complete, is associated with ptosis and a divergent squint.
6.7 Bell’s Palsy
Anisocoria
Worse in dark Horner’s Hypersensitive to aproclonidine (dilates)

Worse in light III nerve
Holmes-Adie Preserved accommodation reflex
Hypersensitive to pilocarpine 0.1% (constricts)
Bell’s palsy is a facial palsy of unknown cause which usually recovers. Patients pres-
ent initially to the general emergency department where treatment is started (pred-
nisolone 1 mg/kg to a maximum of 60 mg for 6 days, tapering off over the next
4 days) before referral to the ophthalmologist for advice about the eye. The diagno-
sis is made if there is a fairly rapid evolution of the palsy over less than 48 hours with
no other cranial nerve lesion, and if there is recovery in 6–8 weeks. The palsy affects
the forehead and lower parts of the face. If the forehead is spared or there are other
neurological features such as limb weakness or diplopia, investigation for a stroke or
other intracranial lesion is indicated. If the symptoms are progressive, Guillain Barre
syndrome, Lyme disease and meningitis must be considered. The role of the oph-
thalmologist acutely is to advise about how to prevent exposure keratitis. The eye
does not close on blinking or forced closure and there is also reduced tear
6.8 Proptosis 83
production. If there is a good Bell’s phenomenon on attempted closure the cornea is

safe. If there are tears pooling in the lower fornix, the patient can be encouraged to
redistribute these over the cornea at regular intervals by rubbing the lower lid
upwards to prevent drying. Otherwise lubricating drops can be prescribed but these
are usually not necessary. Taped closure at night may be required for a short time.
6.8 Proptosis
Proptosis. This differs from most of the other entities described so far in that it is a
sign, rather than a diagnosis, and the underlying problem needs to be teased out by
careful history taking. Appropriate questions relate to duration, progression, pain
visual symptoms such as reduced VA, reduced colour perception, and diplopia, pos-
tural changes or changes associated with Valsalva manoeuvre and medical history
particularly in relation to thyroid symptoms or known malignancy.
Examination includes full eye examination, particularly optic nerve function
(pupil responses, VA, colour vision and visual fields), fundus examination for signs
of optic nerve pressure, examination of eye movements and measurement of propto-
sis using an exophthalmometer. The direction of displacement of the globe is also
recorded. The orbit can be palpated if the swelling extends to the eyelids and aus-
cultated in case of a vascular cause. If thyroid disease is implicated other features
such as lid retraction and lid lag may be identifiable.
Investigation is directed by the differential diagnosis, looking for haematological
evidence of inflammation or thyroid disease, and ordering an MRI to identify the
site and nature of the causative lesion.
Proptosis
Children
Infection: Cellulitis secondary to sinus infection
Neoplastic: Neuroblastoma, leukaemia, rhabdomyosarcoma, retinoblas-
toma, capillary haemangioma, optic nerve glioma (neurofibromatosis type I),
Langerhans cell histiocytosis
Developmental: dermoid
Syndromes: Apert’s, Crouzon
Pseudoproptosis: buphthalmos, myopia
Adults
Infection: cellulitis secondary to sinus disease
Pseudotumour, thyroid
Vasculitis: GPA
Neoplastic: lacrimal tumour, lymphoma, leukaemia, meningioma, glioma,
ossifying fibroma, metastasis, Langerhans cell histiocytosis
Vascular: varix, carotico cavernous fistula
Trauma: haemorrhage, fracture
In children, capillary haemangioma is the commonest benign cause, which can

be expected to involute spontaneously. The lesions are commonly seen on the lids
but can also involve the orbit. Optic nerve glioma usually presents before the age of
7 in NF type I patients and produces an axial proptosis. This is usually benign and
managed conservatively. Rhabdomyosarcoma is the commonest primary orbital
malignancy in childhood typically presenting at around 7 years with rapidly pro-
gressing non axial proptosis with pain, and can be misdiagnosed as orbital cellulitis.
Dermoids are usually in the superotemporal anterior orbit, growing slowly and
painlessly. These cases should be referred to an orbital surgeon.
Cavernous haemangioma is the commonest benign orbital tumour in adults, pre-
senting in early middle age with slowly progressive proptosis or often a refractive
change with increasing hypermetropia. Most do not require any treatment but sur-
gery is an option especially if the visual symptoms are progressive.
Sphenoidal ridge meningioma and optic nerve sheath meningioma both can pres-
ent with proptosis and reduced vision, sometimes with oculomotor palsies. There
may be associated headache. The symptoms are due to encroachment on the supe-
rior orbital fissure. Meningiomas are the commonest benign intracranial tumours,
accounting for over 35% of all primary intracranial lesions. They are associated
with neurofibromatosis type II and can present at any age, but increase in frequency
with age.
Update on Electrodiagnostic Tests (Fig. 6.12)
The ERG photoreceptor response is characterised by a negative a wave fol-

lowed by a larger b wave. This is a global photoreceptor response. Rods vastly
outnumber cones and therefore the response is maximal in the dark adapted
state. The a wave represents the photoreceptor response measured as the
implicit time and the b wave the response from the inner retina. Ideally the
recordings are made with a corneal electrode, a wire or a foil draped over the
lower lid. In children recordings can be made with a skin electrode but the
traces are smaller and less easy to interpret than with a corneal electrode. To
separate the rod response from the cone response, a dim blue flash in the dark
is used for the rod response and a red flicker to select out the cone response.
Multifocal ERG is a newer development and has a place in the testing of sus-
pected chloroquine retinopathy.
Pattern ERG (PERG) is a response from the ganglion cells and is generally
a measure of macular function because the ganglion cells are intensely con-
centrated at the posterior pole The key features are the P50 and N95. Both
features are reduced in macular disease whereas the N95 is disproportionately
reduced in optic nerve disease and the test in conjunction with a VEP aids in
the interpretation of a VEP.
The VEP shows the response between the optic nerve and visual cortex.
The stimulus is either a flash or a pattern reversal checkerboard with varying
check sizes. The measured point is the P100 (normally at 100 ms). Delay in
Further Reading 85
the P100 indicates an abnormality of transmission in the optic nerve, usually

demyelination. The amplitude of the wave is maximum at the smallest check
size perceived by the patient and can give evidence to support malingering.
However, if the patient intentionally fails to focus on the stimulus the response
will be poor. Flash stimulation is used in unconscious patients or in babies.
EOG tests the function of the RPE but is not often done because it is very
time consuming. The patient is fully dark adapted and then the change in the
potential when the light is turned on is recorded as a ratio of light to dark
(Arden ratio). The light rise is severely reduced or absent in Best’s disease and
may be useful in family studies of inherited retinal disease although genetic
investigations are taking over rapidly.
ERG PERG P50

4
b
60
AMPLITUDE (µV)
AMPLITUDE (µV)
0 N95
50 100 -4
a
100
TIME (msec) TIME (msec)
FLICKER ERG (40HZ) VEP
30 P100
AMPLITUDE (msec)
AMPLITUDE (µV)
20 40 60
N75 N135
100
TIME (msec) 0
TIME (msec)
Fig. 6.12 Schematic electrodiagnostic traces
Further Reading
Azuara-Blanco A, Burr J, Ramsay C, Cooper D, Foster P, et al. The effectiveness of early lens
extraction with intraocular lens implantation for the treatment of primary angle-closure glau-
coma (EAGLE): a randomised controlled trial. Lancet. 2016;388:1389–97.
Bassi ST, Mohana KP. Optical coherence tomography in papilledema and pseudopapilledema with
and without optic nerve head drusen. Indian J Ophthalmol. 2014;62:1146–51.
Chang MY, Velez FG, Demer JL, Bonelli L, Quiros PA, Arnold AC, Sadun AA, Pineles
SL. Accuracy of diagnostic imaging modalities for classifying pediatric eyes as papilledema
versus pseudopapilledema. Ophthalmology. 2017;124:1839–48.
Diagnosis and management of headache in adults A national clinical guideline. Scottish

Intercollegiate Guidelines Network; 2008
IHD-3 The international classification of headache disorders. 3rd ed. 2018
Rebolleda G, Kawasaki A, de Juan V, Oblanca N, Munoz-Negrete FJ. Optical coherence tomog-
raphy to differentiate papilledema from pseudopapilledema. Curr Neurol Neurosci Rep.
2017;17(74):1–13.
The clinical profile of optic neuritis. Experience of the optic neuritis treatment trial. Optic neuritis
study group. Arch Ophthalmol 1991;109:1672–1678.
Xue K, Hidebrand GD. Retinal imaging: what the neurologist needs to know. Pract Neurol.
2013;13:236–44.
Chapter 7
Rapid Access: Retina
Most if not all UK hospitals now have fast track access to the retinal service for
acute macular disease in the elderly. This service is not considered in this text for
that reason. The retinal conditions which may present acutely outside the scope of
that service largely are retinal tears and detachments already discussed, inflamma-
tory diseases which have been covered in the section on uveitis and vascular events.
7.1 Retinal Vein Occlusion
While arterial occlusion is an emergency, venous occlusion is less acute and often
presents as an incidental finding when the patient visits his optometrist for a routine
check. If the macula is not involved with haemorrhage or oedema there are minor or
no symptoms. The RCOphth guideline 2015 advises that the initial visit to an oph-
thalmologist should be no more than 2–4 weeks after first recognition of fundus
signs, acknowledging that in asymptomatic patients, the time since the event is
inderminate. It is important to appreciate that branch retinal vein occlusion (BRVO)
and central retinal vein occlusion (CRVO) are different entities and behave differ-
ently. Most, however, occur in patients over 65 years old. BRVO is commoner than
CRVO and is associated with atherosclerosis. Generally, the prognosis is better than
for CRVO. Ischaemia in BRVO causes retinal neovascularisation but rarely rubeo-
sis. Vision loss results from a moderate amount of macular oedema which may
improve spontaneously over 3 months.
CRVO is divided into ischaemic or more commonly, non ischaemic type.
Ischaemic CRVO may go on to produce rubeosis unless treated. Hemivein occlu-
sion behaves in a similar way to CRVO. Signs associated with ischaemia are poor
visual acuity, RAPD, deep intraretinal haemorrhage, multiple cotton wool spots and
retinal vein dilatation and tortuosity. The visual outcome for ischaemic CRVO is
generally poor while non ischaemic may make a complete recovery. The central
vein occlusion study group published results of a multicentre study in 1997 showing

https://doi.org/10.1007/978-3-319-92369-7_7
88 7 Rapid Access: Retina
that there is a strong correlation between the visual acuity at presentation and prog-
nosis. 65% of patients presenting with 6/12 or better had a good outcome, while
only 20% of those presenting with 6/60 or less had any significant visual improve-
ment. This is however an old study and newer treatments mean that the figures need
to be reassessed.
Signs of Retinal Ischaemia

Significantly reduced VA
RAPD
Deep intraretinal haemorrhage
Cotton wool spots
Vascular dilatation and tortuosity
The majority of cases are attributable to generalised cardiovascular disease,

hypertension, diabetes or hyperlipidaemia and glaucoma. Less common causes are
homocyseinaemia, hypercoagulability disorders, and vasculitis particularly Behcet’s
disease. Investigation is not necessary if risk factors for cardiovascular disease, i.e.
age over 50, hypertension, diabetes, smoking and obesity, or glaucoma are identi-
fied. The Royal College of Ophthalmologists recommends initial investigations to
be blood pressure measurement, blood glucose, ESR and FBC to exclude leukae-
mia, other blood disorders or inflammatory disease. Blood pressure over
140/90 mmHg is recommended by NICE as requiring treatment. A serum lipid pro-
file is commonly added to this.
Patients under 50 without a cardiovascular risk factor, and patients with simulta-
neous bilateral disease should be investigated for hypercoagulabiity and inflamma-
tion. The list of investigations is shown in the box. Possible conditions are leukaemia,
multiple myeloma, Waldenstrom’s macroglobulinaemia, cryoglobulinaemia, polycy-
thaemia, antiphospholipid syndrome, myelofibrosis, protein C pathway abnormality
and Factor V Leiden thrombophilia. (Factor V Leiden thrombophilia is associated
with a history of miscarriage.) Screening for inflammation includes Behcet’s dis-
ease, PAN, SLE, GPA, sarcoidosis, TB, syphilis and Goodpasture’s syndrome.
Initial investigations
Hypercoagulability
Increased coagulant factors Prothrombin, factor V Leiden, thrombin, fibrinogen
Decreased anticoagulant factors Protein C, protein S, antithrombin III
Decreased fibrinolysis Plasminogen, plasmin
Homocyseinaemia Homocysteine
Leukaemia FBC, ESR
Multiple myeloma FBC, ESR, CRP
7.1 Retinal Vein Occlusion 89
Waldenstrom’s FBC
macroglobulinaemia
Myelofibrosis FBC
Cryoglobulinaemia Refer to haematology
Polycythaemia FBC
Antiphospholoipid syndrome Antiphospholipid antibody, prothrombin time
Inflammation
Behcet’s FBC, ESR, HLA B5
PAN FBC, ESR, HepBsAg
SLE FBC, ESR, ANA (antinuclear antibody),
antiphospholipid antibody
GPA cANCA (antineutrophil cytoplasmic antibody)
Sarcoidosis ACE (angiotensin converting enzyme)
TB Skin test
Syphilis Serology
Goodpasture’s disease pANCA cANCA, anti GBM Ab (anti glomerular
basement membrane antibody
There is no urgent treatment required other than control of IOP if elevated. RVO is
not correlated with the risk of stroke or mortality, except perhaps in younger male
subjects. The RCOPhth recommendation is for younger male patients to have a
cardiovascular risk assessment and appropriate prophylactic treatment.
Once retinal vein occlusion has been diagnosed the patient should be referred for
fluorescein angiography and to the retinal clinic within a month. Macular oedema is
treated with anti-VEGF intravitreally or intravitreal steroid, as a long acting implant
e.g. Ozurdex, which reduces capillary permeability and inhibits VEGF and is more
effective if implanted early.
Neoascularisation is treated with laser retinal photocoagulation.
There have been numerous large clinical trials concerning the treatment of vein
occlusions. These are fully described in the RCOPhth guidelines in which the con-
clusions are published. These are that:
1. Treatment is not effective if macular oedema has been present for greater than
12 months.
2. The best results are achieved if the macular oedema is of less than 3 months’
duration, and less good if there is a delay before treatment of over 6 months.
3. Treatment of macular oedema reduces the risk of neovascularisation and delays
its onset.
There is also a Cochrane review supporting the use of anti-VEGF intravitreally
for the treatment of macular oedema secondary to CRVO.
Treatment Algorithm RVO RCOphth 2015

Non ischaemic No anterior segment neovascularisation plus OCT evidence of
CRVO CMO
If VA 6/96 or better intavitreal anti VEGF or Oxurdex
If VA less than 6/96 no treatment but observe for anterior segment
neovascularisation
If VA better than 6/12 observe
Ischaemic CRVO If anterior segment neovascularisation, urgent PRP (Pan retinal
photocoagulation)
If anterior segment neovascularisation with raised IOP, urgent PRP
with cyclodiode laser or shunt surgery
Non ischaemic If VA better than 6/12 observe for 3 months
BRVO If VA 6/12 or worse with macular oedema, order FFA
FFA
If no macular ischaemia observe for 3 months
Mild to moderate ischaemia anti VEGF or Ozurdex
Severe macular ischaemia no treatment but observe for new vessels
Ischaemic BRVO Watch for new vessels and sector laser when they appear.
Prophylactic laser is not beneficial
7.2 Central Serous Retinopathy (CSR)
CSR is a relatively common condition presenting to rapid access clinics. It is thought

to be related to the level of glucocorticoids in the blood, which may either be thera-
peutic taken in the preceding month, raised in pregnancy or a response to stress. It
is also associated with hypertension and obstructive sleep apnoea. Circulating cor-
tisol and adrenaline affect autoregulation in the choroid. The typical stress related
presentation is a 40–50 year old type A personality male, complaining of micropsia
or metamorphopsia. Resolution within 2 months is the rule although a minor symp-
tomatic defect may persist and there is a 50% risk of recurrence.
OCT is the obvious investigation to demonstrate the localized neuroretinal serous
detachment. FFA is no longer indicated although if resolution does not occur it may
be necessary to identify any other vascular lesion such as neovasularisation or cho-
roidal haemangioma.
7.3 Choroidal Infarction
Disturbance of blood flow in the choroid is less easy to recognise than in the
retinal circulation, and is mostly associated with accelerated hypertension when
it may cause serous retinal detachment if a large area of choroid is affected, or
7.5 Choroidal Naevus 91
small infarcts of the RPE, seen later as patches of pigmentary irregularity termed
Elschnig’s spots. Vasculitis affecting the posterior ciliary arteries as is seen in GCA
and GPA can be the underlying cause. The diagnosis of choroidal insufficiency
should be considered in patients with failing vision and no obvious identifiable
cause.
7.4 Choroidal Haemangioma
Choroidal haemangioma is a benign vascular hamartoma. The lesion may be cir-

cumscribed or diffuse. The circumscribed ones are sporadic and have a very similar
presentation to CSR, aged 20–50 with reduced vision and metamorphopsia. The
lesion in the choroid is orange with an overlying serous retinal detachment. The dif-
ferential diagnosis is amelanotic melanoma, metastasis, posterior scleritis, choroi-
dal osteoma, posterior uveitis and lymphoma as well as CSR. The features on
fluorescein angiography are described as a lacy network of vessels during choroidal
filling, better demonstrated with ICG.
Diffuse choroidal haemangioma is associated with Sturge-Weber syndrome.
Either of these conditions should be referred to the retinal service for management.
7.5 Choroidal Naevus
Pigmented lesions in the fundus may be described to a patient in a manner which

excites alarm and results in a rapid access referral although the usual pathway would
be as a non urgent case. Choroidal naevi are found in up to 10% of the population,
clearly making it an impossibility to follow them all in case of malignancy. The ones
which could be malignant therefore have to be differentiated from the vast majority
which are not. Roughly 1 in 10,000 naevi are thought to have malignant potential,
but some of the lesions diagnosed as benign may already be malignant, raising
slightly the risk. There are a number of features associated with malignant potential,
and the advice is that if the patient shows 1 or 2 of these features he should be moni-
tored 6 monthly and with 3 or more should be referred to a specialist centre. The
features are; thickness greater than 2 mm, presence of subretinal fluid, visual symp-
toms, orange pigment on the surface and situated within 2 disc diameters of the disc.
Although proximity to the disc is described as a risk factor, any malignant change
would encite the accumulation of subretinal fluid or distortion of the photoreceptors
giving rise to early symptoms. It is probably unnecessary to follow up patients on
the basis of this feature alone. Drusen on the surface and depigmentation surround-
ing the lesion are indicators of a benign lesion. A drop in VA sometimes with flashes
and floaters is likely to cause alarm but can present with benign naevi if they have a
small amount of subretinal fluid over them, an associated macular oedema or rarely
choroidal neovascularisation.
Indications to follow up choroidal naevi

Thickness greater than 2 mm
Subretinal fluid
Visual symptoms
Orange pigment on surface
Within 2 disc diameters of disc
7.6 Vitreous Haemorrhage
Vitreous haemorrhage can cause profound painless visual loss. It can be due to a
retinal tear involving a retinal blood vessel, posterior vitreous detachment, trauma,
probably with associated retinal tear or detachment, proliferative diabetic retinopa-
thy, secondary to neovascularisation in retinal vein occlusion, and in association
with subarachnoid haemorrhage (Terson’s syndrome). In young healthy patients a
traumatic vitreous haemorrhage settles quickly and these patients should be advised
to remain as immobile as is practical and to return in a week for thorough retinal
examination. In an acute traumatic case B scan ultrasound is sensitive in detecting
retinal detachment. Patients with a retinal vascular cause should be reviewed in a
retinal clinic.
7.7 Paraneoplastic Syndromes
A retinopathy caused by an antibody attack on the retina occurs in association with

some malignancies. The presentation is painless rapid deterioration of vision, often
with flashes described as sparkling, with photophobia and glare. The symptoms can
be similar to those described by retinitis pigmentosa (RP) patients. ERG shows
reduced rod and cone responses. There may be a role for corticosteroids and immu-
nosuppressives in treating the condition.
Further Reading
Braithwaite T, Nanji AA, Greenberg PB LK. Anti-vascular endothelial growth factor for mac-
ular oedema secondary to central retinal vein occlusion. Cochrane Database Syst Rev.
2014;5:CD007325.
Natural history and clinical management of central retinal vein occlusion. The central vein occlu-
sion study group. Arch Ophthalmol 1997;115:486–491.
Retinal vein occlusion (RVO) guidelines. RCOphth Clinical guidelines, 2015.
Chapter 8
Referrals from Other Hospital
Departments
Even in a hospital setting there is a misconception about the activity of ophthal-

mologists as compared to optometrists. It is not uncommon for referrals from other
departments, particularly medicine for the elderly to arrive in rapid access clinics
stating that the patient has mislaid their glasses, cannot see to read, or may have had
a fall due to poor vision. Although irritating, these referrals are appropriate if the
patient’s vision is one of the factors preventing discharge from hospital. Surgical
patients however, often arrive with blurred vision, most often secondary to post
operative analgesic medication. Psychiatric patients complain of blurred vision
sometimes induced by drug treatment and are at increased risk of developing angle
closure glaucoma.
Other departments may refer because of a possible diagnosis of GCA requesting
TAB, or screening examinations for uveitis in children with JIA. Opinions about
suspected swollen discs are also relatively common. Requests for conjunctival
biopsy in suspected sarcoidosis are best refused as blind biopsy is unhelpful.
8.1 Toxic Effects of Drugs
Specialists in other departments may refer a patient for a baseline eye examination
before prescribing drugs known to have ocular side effects. These patients need to
be seen in rapid access to allow their treatment to be started.
Ethambutol causes an optic neuropathy which is dose and duration dependant. It
can usually be identified after 4 months of treatment and not before 2 unless there is
renal insufficiency. The patient may notice dyschromatopsia and central progressive
blurring of vision. OCT screening is helpful as nerve fibre loss is identifiable before
symptoms develop. The condition is not always reversible when the drug is
withdrawn.
Isoniazid also causes an optic neuropathy.

https://doi.org/10.1007/978-3-319-92369-7_8
94 8 Referrals from Other Hospital Departments
Chloroquine. The Royal College of Ophthalmologists has updated screening

guidelines in 2018 for patients on chloroquine and hydroxychloroquine.
Hydroxychloroquine retinopathy is more prevalent than was thought. The risk is
highest with those on over 5 mg/kg/day for 5 years or more, those with renal impair-
ment, and those also taking tamoxifen. Hydroxychloroquine is much less toxic than
chloroquine. Early toxicity is recognized as a fine pigmentary change at the macula
which progresses to depigmentation resulting in a bull’s eye maculopathy. This loss
of RPE is not reversible and may continue to progress after the drug has been dis-
continued giving a picture resembling dry age related macular degeneration. Patients
at the start of treatment are recommended to have a baseline examination within
6 months of starting treatment, including colour fundus photography and an OCT of
the macula. They then need annual review starting after 5 years of treatment with
hydroxychloroquine or 1 year of treatment with chloroquine, with a 10-2 Humphrey
visual field and wide field autofluorescence imaging.
Tamoxifen is known to have toxic effects on the retina causing macular oedema
with crystalline intraretinal deposits and possibly an optic neuropathy. Baseline
measures are as for chloroquine and ethambutol.
Fingolimod is used for treating relapsing MS and causes uveitis and macular
oedema, which is reversible on stopping medication. The recommendation is that
patients should have an ophthalmic examination with OCT of the macula at the start
of treatment, then at 3, 6 and 12 months, then annually.
Vigabatrin is an antiepileptic drug frequently prescribed for children, which
causes asymptomatic peripheral field loss. The side effect is independent of dose or
treatment duration, and is not reversible on discontinuing treatment. Guidelines for
screening are published by the RCOphth. Formal field testing is said to be possible
from age 9, although younger children are sometimes quite cooperative. A Humphrey
120 point or Octopus 07 are suitable to reach far enough peripherally. Fields should
be charted at baseline and every 6 months for 5 years, and then annually if no defect
is found.
Desferrioxamine is used in the treatment of haemochromatosis, and can cause a
pigmentary retinopathy and optic neuropathy. Patients may be aware of deteriora-
tion in visual acuity or colour vision and night blindness. Baseline recordings prior
to starting treatment should be VA, colour vision, colour fundus photographs, visual
fields and possibly autofluorescence.
Further Reading
Hydroxychloroquine and chloroquine retinopathy: recommendations on screening. Clinical guide-

lines. RCOphth; 2018.
Mohamed Q, Healey R. Update on retinal toxicity. RCOphth Focus; 2016.
The ocular side effects of Vigabatrin (Sabril) Information and guidance for screening. RCOphth;
2008.
Chapter 9
Paediatrics
Many but not all departments will have rapid access arrangements for children. The
big difference between the access for children and adults is managing the level of
parental anxiety. For example an innocent fundus lesion in an adult found at routine
examination is not a cause for alarm but in a child, there is immediate worry for the
parents about its meaning for the child’s eyesight or health. Many children attending
at rapid access clinics turn out to have no significant abnormality, but early reassur-
ance of this is greatly appreciated by their families. A number of conditions affect-
ing children have been described in earlier chapters, but here the more common
specifically paediatric conditions are covered.
9.1 Neonates
Neonatal referrals relate to an obvious malformation such as anophthalmos or a lid

abnormality such as is seen in Goldenhar syndrome, or if the red reflex is not seen
clearly. If there is a family history of eye disease a referral is sometimes made at this
point although later would be more appropriate.
A family history of congenital cataract will have been recorded in the antenatal
notes and early recognition of cataract is important for optimum visual outcome.
The neonatologist notes a poor red reflex, asymmetry of red reflex or leukocoria.
25% of congenital cataracts are familial (autosomal dominant), but other causes to
consider are metabolic (e.g. galactosaemia), chromosomal (e.g. Down’s syndrome)
and infection (e.g. rubella). Surgery if indicated should be performed within the first
2 months of life and the infant should be referred to a paediatric unit. Uniocular
cataract may be a manifestation of a more extensive malformation of the eye. Many
of the babies referred have an insignificant cataract or a normal red reflex which was
not visualized by the neonatologist for technical reasons.
Very commonly babies and children are referred because there is asymmetry of
the red reflex in photographs. Superficial internet browsing soon throws up the

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96 9 Paediatrics
p ossibility of retinoblastoma, but the reason is almost always that the flash from the
camera has reflected back from the optic nerve rather than the retina giving the
white appearance. Usually simply looking at the photograph can make the diagno-
sis, if the child is looking away from the camera, at about 15° nasally to the source
of the flash rather than at it directly.
Other causes of leukocoria include retinoblastoma, retinopathy of prematurity
(ROP), vitreous haemorrhage, retinal detachment and, persistent hyperplastic pri-
mary vitreous. Rarer causes include tuberous sclerosis, incontinentia pigmenti, von
Hippel-Lindau disease and Toxocara canis infestation. The rapid access role is to
confirm that an abnormality is present, and refer on to a paediatric ophthalmology
service for further management. 10% of retinoblastoma patients have a family his-
tory with an autosomal dominant inheritance pattern, and present at a younger age
than the sporadic form. Presentations later in childhood may be with a poor red
reflex, a squint, ocular inflammation or orbital cellulitis. 90% of retinoblastomas are
diagnosed before age 5 and by age 1 if there is a family history. Offspring of affected
individuals are automatically enrolled in screening from the first week of life.
Siblings need to be screened for the condition.
A coloboma may be the cause of an abnormal red reflex, with a whitish reflex
from an area of chorioretinal defect and an abnormally shaped reflex if the iris is
involved. The effect on visual prognosis depends on its extent and any associated
developmental anomalies.
ROP does not present to the clinic as at risk babies are screened in the neonatal
unit and treated if necessary.
Ophthalmia neonatorum is usually caused by chlamydia, gonococcus or HIV in
the birth canal. Chlamydia is treated with oral erythromycin. Gonococcus is feared
because it can rapidly cause necrosis of corneal tissue and is currently treated with
iv cefotaxime100 mg/kg and Polyfax (bacitracin and polymyxin) ointment. HIV is
treated systemically.
Congenital infection with toxoplasmosis, rubella, herpes viruses, cytomegalovi-
rus and HIV will have been detected antenatally. The severity of damage to the eye
depends on the time of infection. They all cause cataract and chorioretinopathy.
9.2 Infants and Toddlers
9.2.1 Poor Visual Responses
Older babies may be referred because their visual responses do not appear to be
normal. Fixation develops at around 6 weeks and failure is most commonly due to
hypoxic brain injury at birth. Other manifestations of the injury will have been iden-
tified by the time the child reaches the ophthalmologist. Totally blind babies have
roving eye movements and rub their eyes. Babies with low vision do not show this
behaviour but fail to fix their gaze on an object such as their mother’s face or a toy.
When testing it is important to avoid any noise as the baby will turn its head to an
auditory as well as a visual stimulus. Blindness is likely to be Leber’s amaurosis, or
9.2 Infants and Toddlers 97
achromatopsia and apparently reduced visual responses are seen in delayed matura-
tion which shows signs of recovery at around 3 months, or cone dystrophy where the
child sees peripherally but not centrally. Fundus examination is best combined with
a cycloplegic refraction (cyclopentolate 0.5% for babies under 6 months), and the
first investigation is an ERG with a VEP. If there are any other indicators of brain
abnormality or developmental delay, the paediatrician will order an MRI under gen-
eral anaesthetic which should be combined with ophthalmic examination.
9.2.2 Developmental Glaucoma
Glaucoma presents in young children with photophobia and tearing. The globe is
enlarged (buphthalmos) and the cornea cloudy. Icare IOP measurement confirms the
diagnosis and the child should be referred urgently to the paediatric clinic.
9.2.3 Non Accidental Injury
Children may present to the hospital with unexplained injuries and the ophthal-
mologist may be asked to examine the child for evidence of shaking. Baby shaking
happens in children under 6 months and results in bilateral retinal haemorrhages
which are usually extensive and in multiple layers, i.e. preretinal, intraretinal and
subretinal, as well as intracranial haemorrhage. If a Retcam is available photographs
should be taken as evidence. These infants are cared for in conjunction with the
local safeguarding team.
9.2.4 Strawberry Naevus
Capillary haemangioma or strawberry nevi occur on the head of 1–2% of babies.

They may not be present at birth but appear in the first 6 months and extend fairly
quickly and then begin to resolve at about 1 year and disappear by 5 years. However
on the eyelid they can cause eyelid thickening inducing astigmatism or ptosis both
of which result in amblyopia. If there is a risk of amblyopia, treatment with oral
propranolol can cause significant shrinkage of the lesion. The child should be
referred to a paediatric plastic surgeon at an early opportunity.
9.2.5 Nystagmus
Congenital idiopathic nystagmus is in fact not present at birth but is the likely diag-
nosis in nystagmus noted before the age of 2 months. It is present in all positions of
gaze although varies in amplitude in different directions, and the eyes are otherwise
98 9 Paediatrics
healthy. There is often a family history. Onward referral to a paediatric clinic for eye
examination is required. Neurological causes such as space occupation have to be
excluded by MRI scan.
Nystagmus can be due to poor VA, such as in foveal hypoplasia seen in albinism
and in achromatopsia.
Photophobia is quite often reported by parents.
Photophobia in Children
Blonde complexion
Migraine
Corneal disease
Retinal disease
Intermittent exotropia
Cortical visual impairment
Migraine in children is characterised by headache which is relieved by sleep,

nausea, vomiting and photophobia. They may also be photophobic between attacks.
This is explained by the finding that migraine sufferers have a population of gan-
glion cells in the retina which are termed intrinsically photosensitive retinal gan-
glion cells (ipRGC). Corneal disease is commonly a retained foreign body, keratitis
secondary to blepharitis or a shield ulcer secondary to allergic eye disease.
Buphthalmos causes corneal oedema and can present with photophobia. Babies
with cone dystrophy function better in dim lighting by maximising the use of the
rods, and may show behaviour avoiding brightly lit environments rather than being
truly photophobic. Children with intermittent exotropia “squint”, that is they screw
up one eye in bright sunshine. The reason for this is unclear. A third of brain dam-
aged children with cortical visual impairment are apparently photophobic and are
more comfortable wearing dark glasses. Photophobia is a recognised psychiatric
symptom in adults and can sometimes also be in children, perhaps as attention seek-
ing behaviour. Blonde children and myopes are less tolerant of bright sunshine and
should wear dark glasses, as indeed should all children to protect the macula from a
lifetime of light damage as a contributing factor to age related macular degenera-
tion. The best filters for photophobia filter out blue light (480 nm) with an FL-41
lens which is pink. Dark glasses should not be worn indoors as dark adaptation
aggravates light sensitivity.
9.2.6 Strabismus
Babies often appear to squint in the early months. After 6 months they require
orthoptic and optometric investigation. Childhood squints usually appear at around
age 3 and are intermittent at the onset and may become evident after an illness.
9.3 School Age Children 99
These children can be managed by the orthoptists and optometrists with periodic
input from an ophthalmologist. However, if the child appears unwell, shows a
change in behaviour or complains of headache further investigations are necessary.
Incomitance is seen in Brown’s and Duane’s syndromes but cranial nerve lesions
should be considered. Diplopia is unusual in children and if reported may indicate a
recent onset of a squint with a neurological cause such as medulloblastoma.
9.2.7 Anisocoria
Babies often have slightly different pupil sizes, another finding which brings up
alarming facts on a parental internet search. If the pupils respond equally in the dark
and in the light, the difference is physiological. If the difference between the pupils
is greater in the light the lesion is in III nerve. A dilated pupil without abnormality
of eye movement or of the lid is not neurological and is due to perhaps inadvertent
administration of a mydriatic. III nerve palsy in children is nearly always due to a
developmental anomaly or intrauterine trauma. There is likely to be a degree of
ptosis.
A small pupil showing greater anisocoria in the dark is a Horner’s syndrome.
Cocaine used to be the standard test to prove the diagnosis but apraclonidine can be
used in adults but not in infants because it induces bradycardia. A Horner’s pupil is
hypersensitive to aproclonidine and will dilate while the fellow eye will not alter.
Horner’s syndrome can be developmental due to absence of sympathetic fibres in
which case the iris is paler than the fellow. If it is acquired, it can be due to brachial
plexus injury at birth, or rarely neuroblastoma in the cervical or upper thoracic
region. Usually no investigation is necessary.
9.3 School Age Children
9.3.1 Optometry Referrals
Older children are referred after routine visits to the optometrist with a variety of
asymptomatic fundus lesions including CHRPE, bear track pigmentation, chorioreti-
nal scars and naevi. CHRPE are present in 90% of patients with familial polyposis
coli but it is not known what the risk is of developing the colonic lesions if CHRPE
are found incidentaly. If multiple or bilateral CHRPE are present it may be worth
referring the patient for genetic testing unless there is already a known family history
in which case referral to a colonic surgeon is appropriate. Familial polyposis coli is
autosomal dominantly inherited and at risk families are usually already involved in a
screening programme. The referral to the ophthalmologist may come from a screen-
ing programme. Naevi grow and become more pigmented in childhood but photo-
graphic recording and follow up by an optometrist is the appropriate management.
100 9 Paediatrics
9.3.2 Blepharitis
Many children, particularly red heads with dry skin are troubled with recurrent lid
inflammation and chalazion. Incision of lid cysts is discouraged because it causes
scarring to the lid, damage to meiboman glands and requires general anaesthesia. As
the condition is recurrent in some children, incision and curettage is only temporarily
beneficial. Local heat and massage, with reassurance that the condition is painless
and that the child will grow out of the problem are the correct management. Demodex
mites are a cause of anterior blepharitis and can be identified on the lashes of children.
Cylindrical cuffs of dandruff on the lashes are indicative of the problem. Demodex
are very common parasites on human skin, becoming more frequent with age and
found in 100% of individuals over 70 years. 50% Tea tree oil is recommended as a lid
scrub and fucidic acid ointment at night is also helpful as it is of a consistency which
traps the mites as they emerge from the lash follicle. Vaseline has a similar action.
9.3.3 Intracranial Tumour
Medulloblastoma is the commonest paediatric brain tumour accounting for 30% of

cases. Its median age at presentation is 9 years. It may present with symptoms of
raised intracranial pressure, i.e. headache and vomiting in older children and behav-
iour change and listlessness in younger ones. The VI nerve may be affected giving
double vision or papilloedema may cause blurred vision. Sometimes the IV nerve
may be affected giving rise to a head tilt, with specifically problems going down
stairs.
9.3.4 Neurofibromatosis
Paediatricians refer children in whom a diagnosis of neurofibromatosis is being

investigated. Neurofibromatosis type I (NF type 1) is autosomal dominant, with
multiple skin lesions and café au lait spots. Lisch nodules are melanocytic hamarto-
mas on the iris and are a marker for the disease. They are yellow-brown and dome
shaped, unlike nevi which are flat and heavily pigmented. They develop with time
and are universal in NF type 1 patients by the age 20, but are rarely seen in young
children. 15–40% of NF type 1 children develop glioma in the optic tract or visual
pathway. Optic nerve glioma presents usually by the age of 7 as a painless proptosis.
The lesions are slow growing and benign, producing fusiform dilatation of the optic
nerve on imaging. They are usually not treated, but cause loss of vision with an
RAPD and optic atrophy. The other ophthalmic presentation is a lid lesion that feels
like a bag of worms, a plexiform neuroma. This can be mistaken for a chalazion by
9.3 School Age Children 101
inexperienced observers. There can also be retinal lesions and raised intraocular
pressure. Other associations are choroidal hamartoma, astrocytic hamartoma of the
optic nerve and combined hamartoma of the retina and RPE. Screening of children
with NF type 1 is recommended annually until the age of 7 with visual acuity and
fundus examination, although the yield of abnormality is extremely small and its
value questionable.
Chapter 10
Online Resources
The excitement and interest of dealing with rapid access patients is that the physi-
cian has no idea what is going to be presented to him with the next patient. However
while some things are relatively common and their management becomes second
nature, rarities will from time to time appear and access to online information means
that a run down to the hospital library is no longer necessary.
The easiest way to retrieve medical information and which does not require pass-
words, is to use Google Advanced Search. This gives access to more specific infor-
mation and up to date academic publications than does regular Google. The option
is to search using a variety of terms to narrow down the required information
quickly. Similarly Google Advanced Search Images can be accessed to show clini-
cal photographs, which are useful when researching unusual cases and are particu-
larly valuable for teaching trainee doctors and non medical staff, as well as for
explaining conditions to patients.
Medscape.com is a website and also a mobile app which accesses medical jour-
nals and news and publishes expert articles on a variety of conditions with frequent
updates, as well as publishing information on drugs and procedures. This extremely
valuable resource is free but requires a registration e-mail and password. The app
can be conveniently downloaded onto a smart phone or tablet computer.
Hospital libraries give access by registration and a password to www.evidence.
nhs.uk which is provided by NICE, the National Institute for Health and Care
Excellence, the UK body which supports evidence based medicine (Fig. 10.1). The
opening page does not require a login and is accessible to patients as well as health
professionals. A simple approach to an evidence search can be made here and scien-
tific as well as lay material is listed, which can be useful when preparing patient
information pamphlets. Articles which are particularly robust in quality guidance
are marked with The NICE accredited mark, a blue and black target symbol. On the
left of the search results are filters for refining the search.

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104 10 Online Resources
Fig. 10.1 Screenshot of NICE evidence website home page
NICE guidelines and pathways for different services as well as information on

how to set standards and use indicators are also indexed at the top of the opening
page of evidence.nhs.uk
For more detailed scientific searches, click on journals and databases. Access
requires a user name and password. The hospital premium collection (Fig. 10.2) is
generally the most useful, particularly if advanced search is selected and then a
number of key words can be searched, connected with “and”, “or” or “not”. In many
cases the full text of the paper is immediately available although sometimes only an
abstract. The most recent publications are not usually available as full text which is
why Medscape is so useful for up to date information. Hospital librarians are gener-
ally extremely helpful and offer willing guidance.
There are other resources similar to Medscape such as Up To Date and Dynamed
which use the same login as for www.evidence.nhs.uk, and EyeWiki, supported by
the American Academy of Ophthalmology for which registration is required but
which is free, and which publishes up to date review articles. The Hoskins Center
and Academy–approved Compendium of Evidence-Based Eye Care is free to use
and is an excellent resource for clinical guidelines.
10 Online Resources 105
Fig. 10.2 Screenshot of Hospital Premium Collection literature search
Cochrane reviews, available further down the opening page on the NICE evi-
dence website, are of limited value in ophthalmology. The majority of published
reviews are inconclusive because of the poor quality of clinical trials, the absence of
comparable trials, or more often because the pace of therapeutic and technological
development is such that trials are out of date by the time they reach the Cochrane
review stage.
College guidelines from the Royal College of Ophthalmology and the College of
Optometry are also useful points of reference.
Part II
Leading an Emergency
and Rapid Access Service
Chapter 11
Introduction
The NHS is the envy of the world in being a service available to all and free at the
point of delivery, but the problem it faces is how to manage capacity in the face of
an ever growing demand as the population grows, new treatments evolve, and the
health seeking behaviour of the public is encouraged. In addition to these general
pressures on the service, there is a specific source of increased volume of referrals
to ophthalmology due to changes in optometric practice. Before 2006, optometrists
referred patients to their GP who then referred on to the hospital eye service. GPs
have an acute awareness of funding of the services and did not refer all cases to the
hospital. In 2006 direct hospital referral was included in a new General Ophthalmic
Services contract with the intention of simplifying the process but the unexpected
outcome has been an avalanche of referrals to hospital eye services. Unlike GPs who
are constantly aware that the available budget is limited, there is no disincentive to
optometrists to refer, nor any incentive to manage minor abnormalities themselves.
Many of the referrals, estimated to be about 1/3 of the total received by the hospital
departments, are trivial or inconsequential, and put a massive, often inappropriate,
pressure on hospital services. Roughly coinciding with this change was the 4 hour
waiting time target (2004) for general emergency patients, meaning that patients
with less severe complaints would not be discouraged from inappropriate use of
services, knowing that they would have to wait only a maximum of 4 hours to be
seen. Prior to that, patients with non life-threatening conditions presenting to an
emergency department might have expected to wait many hours. They might there-
fore have chosen to seek advice elsewhere such as from their GP or pharmacist.
While many of these extra referrals may be non-acute, some are referred through
acute pathways.
The size of the problem is difficult to pin down because of different practices in
different areas at different times, but a good guess based on figures published
20 years and more ago is of eye complaints occurring at about 70/1000 per year.
Accepting that the threshold for hospital referral is greatly reduced, this figure could
be enhanced by conservatively 50% to say 100/1000 per year as an estimate. 30/1000
might be expected to be referred acutely. To enable accurate commissioning, it is

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110 11 Introduction
worth undertaking a short survey of local practice to ensure the correct provision of
time and manpower to run a service.
The responsibility for running a rapid access service can be divided into the
application of clinical skills and aspects of planning and organisation. Doctors are
well trained in the clinical aspects which have been covered in Part I of this book,
but the other aspects are more intuitive and are acquired with experience, observa-
tion, comparison and discussion with colleagues. The organisational aspects may
require some trial and error until a service evolves which suits the local medical
services and the community it serves. There is no one size fits all plan and the detail
of the service depends to an extent on what services are available close by and the
relationships with those other services. The objective is to run a service which inter-
links with other ophthalmic specialties, other allied ophthalmic professionals, and
other medical specialties, and particularly with the referring parties, usually general
practitioners and emergency doctors, but also in the UK, optometrists.
The review of ophthalmic emergency services in the UK undertaken by the Royal
College of Ophthalmologists and published as part of “The Way Forward” found
that large departments in metropolitan areas work best with direct access “walk in”
facilities, whereas smaller departments in less densely populated areas make better
use of resources by triaging referrals, limiting immediate access to true emergencies
while running a rapid access booked clinic in parallel. In Scotland a more uniform
model is adopted with no facility for direct access for the patient to ophthalmology
departments. The difference from elsewhere in the UK is that all optometrists offer
the same minor eye care service which is funded inclusively with eye tests, whereas
in the rest of the UK only the sight test is funded and any additional examination lies
outside the NHS contract. The Ophthalmic Services Contract (2010) covering
England and Wales, states that the optometrist will “secure the testing of the patient’s
sight to determine whether he needs to wear or use an optical appliance”, and if he
“shows on examination signs of injury, disease or abnormality in the eye or else-
where which may require(d) medical treatment; or is not likely to attain a satisfac-
tory standard of vision notwithstanding the application of corrective lenses, shall, if
appropriate, … refer the patient to an ophthalmic hospital”. “If appropriate” is not
elaborated on in the contract, and for some optometrists is interpreted as meaning
that all abnormalities need to be referred.
The increase in the acute referrals prompted the development of optometrist led
urgent services. In the UK there are less than 1000 consultant ophthalmologists and
over 10,000 optometrists. Enlisting optometrists to take on part of the work load or
to work in partnership with ophthalmologists may be part of the solution to the
problem. The Scottish service is more costly to fund but appears preferable to the
rest of the country where there are patchy minor eye care services (MECS) often
referred to as red eye schemes and primary eye care assessment and referral services
(PEARS) which include refinement of referrals for glaucoma, cataract and macular
disease, creating a variety of commissioning problems. These optometry led s ervices
tend to work well in rural areas where patients would have to travel a long way to a
hospital, and less well in urban areas where onward care at a hospital, whether
advised or not by an optometrist, is a convenient option. The result is that the total
Further Reading 111
health economy loses out, as the same patient may generate multiple charges when
a single consultation may have been adequate.
Review of the initial PEARS scheme showed that 66% of patients were dealt
with conclusively. Many acute eye conditions can be treated safely and effectively
without being seen by an ophthalmologist, but the experience of MECS services
reported by hospital ophthalmologists appears to be that the load on GPs is reduced
but not the load on hospital services. In other words many of the conditions which
could be managed by GPs are now managed by optometrists. While this may be a
better service for the patients it is not the intended outcome of setting up such ser-
vices. Communication and training opportunities between the HES and MECS, and
indeed the whole optometry community could improve the situation and may be the
way forward. A change to the optometrists’ contract is also necessary to address this
issue.
Further Reading
Buchan JC, Barnes B, Cassels-Brown A, Chang BY, Harcourt J, Pilling RF, Shickle D, Spencer
AF, SA V, MacEwen C. The urgent need to develop emergency eye care in the UK: the way
forward. Eye. 2017;31:1515–8.
Commissioning better eye care. Clinical commissioning guidance from the college of Optometrists
and The Royal College of Ophthalmologists. Urgent Eye Care; 2013.
Emergency eye care in hospital eye units and secondary care. Royal College of Ophthalmologists
Ophthalmic service guidance; 2017.
General Ophthalmic Mandatory Services Model Contract. Prepared by the Department of Health
October 2010.
Minor Eye Conditions Service (MECS) Pathway. Local Optical Committee Support Unit; 2008.
Primary Eye Care Framework for first contact care. Clinical Council for eye health commission-
ing; 2016.
Chapter 12
Organisation and Management
The clinician leading the emergency and rapid access ophthalmology service needs
to explore a number of areas whether developing a pre-existing service or setting up
from scratch. Topics to consider are population statistics and volume of work antici-
pated, including workforce planning and clinic management. Plans also need to be
developed for training and communication to the local healthcare providers and
referring agencies and the development of communication networks within the hos-
pital. In house training and updating of staff is an integral and importany aspect.
When setting up a new service or modifying an existing service, the first decision
relates to access. “The Way Forward” surveyed eye departments and found that in
departments covering a population of less than 500,000, selection of referrals was
the preferred pattern, fitting patients into existing clinics in the smallest units
(<350,000) and having booked clinics in larger units (350,000–500,000). A popula-
tion over 500,000 supported a walk in service, open in office hours in medium sized
units (500,000–760,000) and 24 hour walk in for the largest. The ever present
dilemma is that the better the access, the more people will come to use the service.
While wishing to provide the most efficient service possible, increasing capacity
and efficiency may encourage more patients to present with increasingly trivial
complaints.
12.1 Volume and Workload Planning
A walk in service requires an estimate of volume. For example a population of

600,000 based on an estimate of 30/1000 per year as emergency or urgent referrals
would generate 18,000 referrals a year, or 360 per week. Half of these might be
expected to have minor complaints and to be seen and treated by nursing staff leav-
ing 180 to be seen for more complex conditions, equating to 12 sessions of clinical
time for doctors or appropriately trained ancillary staff.

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114 12 Organisation and Management
A population of 320,000 would generate the same 30/1000 acute problems but
up to half of these might be redirected to a MECS scheme with appropriate support,
training and communication, leaving 15/1000 or 4800 to be dealt with in the hospi-
tal setting. Of these, approximately a quarter require to be seen on the day, about 25
a week, and the remainder in prebooked rapid access clinics in 5 consultant led ses-
sions per week. Those requiring to be seen as emergencies on the day of presenta-
tion are small enough in number to be efficiently absorbed into existing clinics,
spread over various specialties. For example in a unit with 5 full time consultants
each delivering 4 clinics a week, the expected emergencies would amount to 1–2 per
consultant clinic. These estimates are derived from a number of unpublished sur-
veys conducted by the author during 2015–2018.
It may be worth carrying out a local analysis of referral patterns and numbers to
make as accurate an estimate as possible of local need. For example, the referrals
received during a 1 week period were analysed in the author’s department. The
population served is 400,000, with a local MECS scheme. The week selected was
one in November in which there were no national or school holidays. Obviously a
longer period would be more indicative but collecting and analyzing the data is
laborious as it is not recorded for any management reports. During that week there
were 99 emergency and rapid access referrals of which 46% came direct from
optometrists, and 183 non acute referrals.
The acute arm of the service (excluding the macular service) accounted for 37%
of the total number (282) of referrals. 29% (11% of the total new referrals) of these
were seen as emergencies, 29% were seen in rapid access booked appointments,
25% were triaged to the routine booking pathway and the remaining 17% were sent
to the macula clinic, orthoptics and diabetic screening services.
Of the non acute referrals, 25% were considered general ophthalmology, 20%
were for glaucoma, 15% retinal, 12% paediatric and single figures for other sub
specialties. In this hospital there is a separate and direct pathway for macular dis-
ease and cataract. 35% of the non acute cases were discharged at their first visit
indicating the trivial element of the referrals not requiring anything beyond basic
examination.
By analyzing the emergency referrals, the percentage requiring to be seen on the
same day equated to approximately 3 per session and the rapid access cases approx-
imated to 30 per week. The emergencies were more likely to present on a Monday
or a Friday and for that reason more resource needed to be allocated in this service
on those days. Similarly before and after a public holiday an increase in demand can
be anticipated and planned for. In this example, the rapid access clinics are run on 3
mornings a week with 10–12 patients in each clinic, run by a single consultant.
Ideally these clinics would run daily, with any overcapacity being given over to
routine referrals and occasional follow ups. Patients requiring more than very acute
follow up are seen in appropriate subspecialty clinics.
This pattern of working is very popular with training staff as they are exposed to
a large variety of conditions, with consultant supervision for advice and discussion.
It is also an excellent training ground for nurses and optometrists who wish to take
their career a step further.
12.2 Workforce 115
12.2 Workforce
The next step is to plan the workforce. Apart from medical staff, nurses, optome-
trists and orthoptists have a role to play. Optometrists and nurses can be trained to
deal with many of the conditions, assisted with protocols for common conditions,
and with consultant support. A difficult part of workforce planning is to put in place
systems which take into consideration periods of staff leave, public holidays, and
peaks in demand at the beginning and end of the week. The roles of non medical
staff need to be defined.
An ophthalmic nurse practitioner is a senior nurse with further training in the
care of ophthalmic patients. The role of this team member or members is to super-
vise areas recognised to fall within the nursing sphere, namely to manage the
workforce, monitor adherence to clinical guidelines and local policies, participate
in education and training of the team, maintain the equipment and monitor the
quality and cleanliness of the environment. Additionally she works together with
the lead consultant and the rest of the team to develop the service and introduce
innovative practices. The lead nurse takes responsibility for monitoring patient
experience with feedback to the team. Collection of data for audit and designing
audit projects should be recognised as an important part of the role of nurses as
well as of all team members. The support of a nurse trained in emergency and
urgent ophthalmology is invaluable as a team leader and trainer. This person will
train others to act up in periods of leave. The role differs slightly in large centres
with a walk in facility where the role is largely managerial compared with smaller
centres with telephone or e-mail triage and booked appointments where the role is
a mix of managerial and clinical.
Optometrists within the team would be required to support the optical needs of
the service and also, with appropriate training and supervision, manage according
to agreed protocols a number of ophthalmic conditions, and refer on appropriately.
The extent of the role depends on the motivation of the optometrists to learn new
skills and to expand his knowledge, both achieved with encouragement from the
consultant lead. Optometry training allows optometrists to be particularly effective
in dealing with corneal and anterior segment conditions where there is limited sys-
temic significance but does not equip them for managing conditions with a signifi-
cant systemic or medical element. They also would have a teaching role for other
members of the team, and a role in conducting audits of the service.
An emergency orthoptic clinic is essential to deal with acute presentations of
diplopia, and also for a variety of other, particularly paediatric, rapid access
referrals.
Other members of the team include reception, clerical and secretarial staff who
should identify themselves with the service. Clerical staff are extremely valuable
members of the team, and until paperless records are universally the norm, rapidly
retrieving case records for patients with complex or recurring eye problems is enor-
mously helpful, and communication back to the referring individual educates and
improves the quality of referrals from the community over a period of time. All team
members should know and value the aim of the service which is to provide the
highest quality service in a timely way. This may seem obvious and trite, but
inspirational leadership is a major step towards creating a successful and motivated
department.
Negotiating an allocation of manpower for a good acute service involves demon-
strating the overall benefit, of course to patient care but also to the better running of
the department overall. A consultant led and delivered emergency and rapid access
service offers excellence to patients in having access to a senior opinion either on
the first visit or immediately thereafter, but the advantages to the other services is
also easy to demonstrate.
Firstly, by seeing a patient on the first or at worst a second visit, a senior opinion
is given and appropriate investigations commenced. Previously, emergency ophthal-
mology was largely delivered by ophthalmologists in training or by non-consultant
grade doctors, with sometimes a patient having a number of visits before a definitive
diagnosis was reached or a plan was made, or the patient eventually recovered or
defaulted.
Secondly, with a consultant delivered service, the minority of patients who need
to be referred to subspecialty clinics will arrive at those appointments with a pre-
liminary set of investigations already completed.
Thirdly, as already stated, consultant led emergency and rapid access clinics are
an excellent and popular training ground for young ophthalmologists, as well as
emergency department trainees, GPs, optometrists, orthoptists and nurses.
Lastly, when the system is fully established and working well, GPs, optometrists
and doctors from the emergency department and other hospital departments will
have an access point to the ophthalmology department for an opinion and this is
likely to result in a reduction in some of the more trivial referrals, in that they may
be discussed between colleagues without necessitating a visit to the department.
It is beneficial to designate a small management group within the service, made
up of the senior consultant, the lead nurse and a manager. The group should meet
regularly to address day to day problems and ideas which arise from time to time.
Either this group, or a separate person should take responsibility for in house train-
ing and a programme of audit. Thought should be given to induction of new staff
members with a preprepared document containing specific information about the
department, such as telephone numbers, leave arrangements and secretarial con-
tacts. The RCOphth has produced a document with induction recommendations for
trainee ophthalmologists.
12.3 Walk in or Prebooked Service?
12.3.1 Walk in Service
Nursing staff with advanced training in ophthalmology can manage patients within
their clinics but they are most usefully deployed in sorting (triaging) patients to
12.3 Walk in or Prebooked Service? 117
different practitioners according to their complaints, as well as recording histories

and initiating investigations according to protocols.
In a walk in facility, guidelines need to be in place to direct the patient to the
appropriate management resource. There would normally be three categories, triv-
ial or straight forward to the nurse (green), more complex to the medical or optom-
etry practitioner (amber), and priority for emergencies to be seen in advance of the
other categories (red). These categories are defined by a mixture of symptoms and
readily recognised diagnoses. The make up of the categories varies depending on
the training and competence of the staff involved. This is of course triage but the
term has not been used here to avoid confusion with the other system in smaller
units often identified as a “triage system”, although perhaps better termed a booked
appointment system.
12.3.2 Sorting Categories for Walk in Service
Green Amber Red

Foreign body Red eye with Severe trauma
photophobia
Red eye without photophobia Progressive visual loss Sudden loss of vision less than
24 h
Minor trauma Headache Possible post op endophthalmitis
Work up of possible swollen Orbital cellulitis Possible acute glaucoma
disc
GCA without visual symptoms TIA
12.3.3 Prebooked Service
A prebooked rapid access service works by having a trained staff member receiv-
ing referrals either by phone or by e-mail, and triaging according to a list of criteria
into those patients requiring a same day opinion, in which case they are asked to
attend at the clinic and are seen as emergencies in any available clinic, and those
that can reasonably wait up to 2 weeks, but usually seen much sooner, in a rapid
access consultant clinic. The 2 week rule allows for exceptional periods of staff
absence and is considered a maximum wait. Doctors or appropriately trained other
staff working in that session in other clinics are expected to see occasional emer-
gencies on a rotating basis, as the rapid access consultant will not always be present
and if he is will also have a fully booked clinic. The patients requiring to be seen as
an emergency fall into four groups according to their source. The triage categories
are divided into Emergency, i.e. same day, and Rapid Access, i.e. booked
appointments.
EMERGENCY Same day
1. Patient direct access History of uveitis, keratitis or ulcer

Recent eye surgery with pain or reducing vision
2. Optometrist Suspect acute ACG- pain, raised pressure, blurred vision
Retinal artery occlusion-sudden profound visual loss, one
eye or half field
Retinal tear or detachment- direct to retinal clinic
Corneal ulcer
Acute uveitis
Macular disease with acute symptoms—direct to macula
clinic
3. GP Red eye, flashes and floaters—direct to MECS
Painful red eye with photophobia with or without visual
loss
Sudden or recent onset (<2 days) painless visual
disturbance
Recent onset diplopia (<2 days or progressive)
Suspicion of GCA
Periorbital swelling
4. Emergency department Trauma
or acute medicine Sudden loss of vision
Suspected disc swelling
Rapid access appointments similarly are listed according to the source of the
referral. Some of these are clearly not urgent, but cause significant anxiety to the
patient or in the case of a child, a parent.
Rapid Access Booked Appointment Within 2 Weeks
1. Optometrist Retinal vein occlusion/retinal haemorrhages with visual symptoms

Unexplained visual deterioration
Neurological pattern visual field defect
Central serous retinopathy
Children with abnormal discs
Children with unexplained fundus abnormality
2. GP Headache with visual symptoms other than GCA
Bell’s palsy
Infants and children with anything the GP chooses to refer by this
route other than periorbital swelling which should be seen as
emergency
3. Other Requests for visual field tests
departments Any inpatient request
12.4 Training 119
To maximise throughput in a rapid access clinic, an ophthalmically trained nurse

would work according to prepared guidelines to support the consultant. This
involves history taking and ordering the appropriate tests before seeing the consul-
tant. An example of such a guideline for a patient referred by an optometrist with a
suspicion of disc oedema is given.
Optometrist Referral Blurred Disc

History—specifically was this a routine eye test or because of symptoms
If symptoms describe
If headache describe-diurnal pattern, visual symptoms, nausea, vomiting
VA, pupil response, Ishihara
G Tropicamide 1% both eyes
Enhanced depth OCT disc
Optos imaging with auto fluorescence
To doctor
12.4 Training
There is a national plan to develop training of non medical healthcare profes-

sionals in secondary ophthalmic care, the outline of which was published in 2016
as “The Common Clinical Competency Framework” (CCCF). While a national
plan may be developed, much can be achieved in house. A programme for each
group of staff can be drawn up with records of achievement of levels of compe-
tency. For example, from the simplest being use of slit lamp, checking calibra-
tion of Goldman tonometer, measurement of IOP, pachymetry, removal of foreign
body from cornea, up to more complex such as gonioscopy, fundus examination
and laser treatment. The training can be delegated depending on the experience
of the workforce, both in didactic teaching sessions and in apprentice-type
learning.
This book is laid out largely in relation to diagnosis, but teaching is best
approached from a symptomatic presentation viewpoint, as this is the basis of tri-
age. The groups of symptoms most often discussed are red eyes, painful loss of
vision, painless loss of vision and sudden loss of vision, but this can be expanded
widely to cover all the conditions discussed in Part I.
Red Eye
Subconj haemorrhage
Conjunctivitis
With pain and photophobia
AAU
Keratitis/ulcer
ACG
Scleritis
endophthalmitis
Painful Loss of Vision

AAU
Endophthalmitis
ACG
Optic neuritis
Sudden Loss of Vision

CRAO
ION
TIA
Retinal detachment
GCA
There is no national standard for training ophthalmic nurses but there are a num-
ber of courses including online and day courses advertised. Health Education
England supports funding through the apprenticeship levy of a 2 year apprentice-
ship for a BTEC diploma, with course materials supplied by the Association of
Health Professions in Ophthalmology. This may be a means or recruiting and train-
ing technical support for a department, with members termed healthcare science
associates. It is extremely rewarding personally and for the team for the clinical lead
to invite staff members as guests to local or national seminars or to BEECS (British
Emergency Eye Care Society) annual conference.
A sample of courses currently available is;
Ophthalmic and optometry CPD courses University of Manchester
Ophthalmic nursing online course Metropolitan University, Manchester
Clinical ophthalmic practice course, Moorfields Eye Hospital
University of Edinburgh MSc in primary care ophthalmology (on line course) aimed
at non-medical professionals working in ophthalmology
12.5 Equipment and Useful Reference Charts 121
12.5 Equipment and Useful Reference Charts
Each consulting room An external telephone line

requires: A high quality slit lamp
Goldman tonometer
Snellen chart
Near vision test type
Amsler chart
Ishihara plates or alternative colour vision test
Angle poise lamp
Fundus lenses 90D, 78D, superfield or equivalents
Gonioscopy lens
Direct ophthalmoscope
Binocular Indirect ophthalmoscope with 20D and 28D or 30D lens
Pachymeter
I care tonometer
Exophthalmometer
Battery operated corneal burr
Cotton buds
Small toys
Instruments: Artery forceps
Surgical scissors
Jewelers forceps
Epilating forceps
Scalpel blades 11 and 15 size and handle
Cornea scrape kits
Lacrimal cannula
Punctum dilator
Drugs: Tropicamide 1%
Local anaesthetic drops according to local usage
Local anaesthetic cream
Fluorescein drops
Cyclopentolate 0.5% and 1%
Phenylephrine 2.5 and 10%
Pilocarpine 1%, 2%
Iopidine drops
Apraclonidine drops
GTN
Acetazolamide iv prepartation
Acetazolamide tablets
Mannitol iv
Glycerol
Paracetamol, indomethacin
Gel tear preparation for use with gonioscopy lens
Chloramphenicol ointment
Mydricaine
Triamcinolone for injection
Dexamethasone for injection
Dressings: Eye pads
Eye shield
Crepe bandage
Adhesive tape
Bandage contact lenses
Reward stickers for children
And access to: Children’s vision testing
A slit lamp teaching arm and camera
Portable slit lamp
OCT
Optos or other ultra wide field scanning ophthalmoscope
Fluorescein or OCT angiography
Humphrey visual field analyser
B scan ultrasound
Electrodiagnostic testing
ECLO
Notice board Conversion for Snellen/log MAR/Cardiff card acuities
Corneal thickness conversions
12.5 Equipment and Useful Reference Charts 123
12.5.1 Visual Acuity Recording
Snellen Minimum angle of resolution (MAR) Log MAR Decimal

6/60 10 1.0 0.1
6/38 6.3 0.8 0.13
6/24 4 0.6 0.25
6/19 3.2 0.5 0.32
6/12 2 0.3 0.5
6/9.5 1.6 0.2 0.63
6/6 1.0 0.0 1.0
6/4.8 0.8 −0.1 1.25
12.5.2 V
isual Acuity Measured Using Gratings
with Preferential Looking (Infants)
Cycles/degree Snellen equivalent

1 6/180
2 6/90
3 6/60
5 6/36
7.5 6/24
10 6/18
15 6/12
30 6/6
12.5.3 Corneal Thickness Adjustments for IOP
Corneal thickness (μm) Correction (mm Hg)

445 7
455 6
465 6
475 5
485 4
495 4
505 3
515 2
525 1
535 1
545 0
555 −1
565 −1
575 −2
Corneal thickness (μm) Correction (mm Hg)

585 −3
595 −4
605 −4
615 −5
625 −6
635 −6
645 −7
12.6 P
rotocols and Guidelines/Standard Operating
Procedures (SOP)
In an aim to make sure that all patients receive the same optimum standard of care,
protocols should be prepared for the commonest conditions. This is a time consum-
ing process but worth the investment, and once done regular updates can be made.
Some protocols prepared by other departments are available on the internet and can
be downloaded and modified. The task of updating can be delegated to different
staff members, encouraging engagement with the team.
Suggested topics for protocols are: Giant cell arteritis, acute anterior uveitis,
corneal ulcer, transient ischaemic attack and amaurosis fugax, blepharitis, flashes
and floaters, acute glaucoma, retinal vein occlusion, central retinal artery occlusion,
orbital and preseptal cellulitis, Bell’s palsy, endophthalmitis, diplopia, and possible
swollen discs. Examples of such protocols are given here, and others appear in the
text in relation to the conditions covered. These can be prepared in printed form or
set up as an online resource.
Protocol for Management of Suspected Giant Cell Arteritis (GCA)

Complete symptoms check list (Form available in clinic)
If ESR >60
or CRP >40
or symptoms highly suggestive of GCA
book TAB to be done within 2 weeks
and commence prednisolone 1 mg/kg up to 60 mg/day
and omeprazole 20 mg/day
and book review appointment 5 days after TAB for removal of sutures and
results
If patient presents with visual symptoms or is significantly unwell, refer to
rheumatology as an emergency for pulsed methylprednisolone as an
inpatient
12.6 Protocols and Guidelines/Standard Operating Procedures (SOP) 125
Protocol for Management of Acute Anterior Uveitis (AAU)

Check VA and IOP every visit
If VA reduced send for OCT macula
If PS present at first visit dilate with phenylephrine 10% and cyclopento-
late1% and leave in dark with hot compress. If dilatation fails consider sub-
conj mydricaine 0.3 ml with dexametasone 0.5 ml
Prescribe G predforte hourly or 2 hourly depending on severity
And G cyclopentolate 1% od or bd depending on presence of PS
If IOP raised, prescribe acyclovir 400 mg bd and G timolol 0.25% (Unless
contraindicated)
If second attack or bilateral, unless predisposing disease already identified,
order;
FBC
HLA B27
Syphilis serology
ACE
If posterior involvement, also
Rheumatoid factor
ANA
pANCA
cANCA
Review at 1–2 weeks to check response and IOP
Adjust medication response and review in 1 month or if not improving or
complicated, refer to uveitis clinic
Protocol for Management of Corneal Ulcer

Record VA and draw ulcer with measurements on every visit
Dendritic (except contact lens wearers)
Prescribe oc acyclovir 5× daily for 10 days
Arrange nurse clinic review 10 days
CL related or bacterial
Scrape for bacteriology and acanthamoeba
Prescribe G levofloxacin hourly day and night for 3 days, then daytime
only
Prescribe G cyclopentolate 1% bd
If acanthamoeba suspected prescribe G PHMB 0.02% hourly
Review daily with measurements if improving
If not improving, or if acanthamoeba identified refer to corneal clinic
Marginal keratitis
G predsol qid with antibiotic e.g. chloramphenicol
Review 1 week in nurse clinic and record IOP
If healed stop steroid and advise management of blepharitis with hot com-
presses and lid scrubs
Offer www.eyecalm.org if computer literate. Discharge
If not healed or significantly improved refer to cornea clinic
Protocol for Management of TIA or Amaurosis Fugax

History—hypertension, diabetes, heart attack, stroke, peripheral vascular dis-
ease, smoking
VA, bp and pulse, urinalysis, IOP, ESR, CRP
Refer to TIA clinic using proforma
Discharge to GP
Phone GP if any new finding e.g. untreated hypertension
Similarly Standard operating procedures (SOP) should be drawn up for particu-

lar activities such as fluorescein angiography, laser, subconjunctival injections and
checking calibration of a Goldmann tonometer.
Standard Operating Procedure

How to calibrate a Goldmann tonometer (GAT)
Place GAT on slit lamp platform and insert prism
Set dial to 1
Insert calibration bar into side bracket of GAT, lined up with first mark on bar,
shorter portion away from examiner
Rotate dial slowly towards 2 when prism should rock slightly
Move bar to line up with end mark, short portion away from examiner
Rotate dial towards 6 when prism should rock slightly
GAT is correctly calibrated. If not, send for repair
12.9 Audit 127
12.7 Patient Information and Education
A selection of information pamphlets is useful and these should be updated regu-

larly, with a record of the updating schedule. A number are published by national
bodies such as the RNIB and the RCOphth, but locally prepared ones have the
advantage that the task of preparation can be incorporated into a teaching exercise,
and local telephone numbers and contacts can be included. Useful topics include,
for example, blepharitis management and posterior vitreous detachment.
12.8 Networking
It is very helpful to make contact with colleagues in the emergency department,

rheumatology, diabetology, paediatrics, medicine for the elderly, neurology, neuro-
surgery, histopathology and radiology to name the most frequent. Speaking to a
colleague on the telephone saves a considerable amount of time when sorting out a
problem involving more than one department.
12.9 Audit
Although this is the final part of this book, it is not an afterthought. Regular reviews
of the service looking at quality and outcomes is essential for continuous develop-
ment and for maintaining motivation and morale in a team. Particular problems may
become evident over time. For example, the London Eye Health Network audited
the number of optometry referrals per named optometrist and found that the major-
ity referred similar numbers, but a few were on a wildly different scale. The solution
to a problem such as this is the invite the outlying optometrists to the hospital for a
few sessions for mentoring and training. This has a remarkable effect on the confi-
dence of the referrer who knows who is at the other end of the pathway and may in
future make a phone call rather than a referral if in doubt.
It is useful to think about data collection for potential audits when drawing up
paper or electronic records for the service. A suggested format is shown on the next
page.
EMERGENCY AND RAPID ACCESS OPHTHALMOLOGY CLINIC
TRIAGE date Name DOB Record number source of referral
PRESENTING COMPLAINT
Duration of symptoms
Pain
Vision affected/VA
POH (if recent, rebook to same clinic if poss)
EMERGENCY Y/N RAPID ACCESS Y/N Triage nurse initials
CLINIC Date and time
History of presenting complaint
Past medical history
Past ophthalmic history
Ophthalmic family history
Drugs Allergies
R L
VA distance
Near
Ishihara
Pupils
Icare
Bp urinalysis drops administered with time
PRIORITY EXPEDITE Y/N IN TURN Y/N
Resource consultant other
Clinic nurse initials

Further Reading 129
Further Reading
Clinical management guidelines The College of Optometrists online resource

Community ophthalmology framework. Clinical council for eye health care commissioning; 2015
Emergency Eye care in hospital eye units and secondary care. Ophthalmic service guidance.
RCOphth; 2017
Eye Health Network for London: Acheiving better outcomes. NHS England; 2015
Local Eye health networks, LOC Support unit publications
PEARS Accreditation and MECS assessment, available through Wales optometry postgraduate
education centre. Guidance notes and curriculum available from the College of Optometrists
Primary care ophthalmology. Ophthalmic services guidance. RCOphth; 2013
Primary eye care framework for first contact care Clinical council for eye health commissioning;
2016
Recommended induction for ophthalmology. RCOphth; 2017
Ring R, Okoro M. A handbook of ophthalmic standards and procedures. 2nd ed. Keswick: M&K
Publishing; 2016.
The common clinical competency framework for non-medical ophthalmic healthcare profession-
als in secondary care. Prepared jointly by the Royal College of Ophthalmologists, Royal col-
lege of Nursing, British and Irish orthoptic society, the College of optometrists and association
of health professionals in ophthalmology; 2016
The nature, scope and value of ophthalmic nursing. Royal college of Nursing; 2016
Index
A Aproclonidine, 27, 82
Acanthamoeba keratitis, 43 Arterial occlusion, 17, 18, 87
Acetazolamide, 18, 25–28 Arteritic arterial occlusion, 20
Achromatopsia, 97 Atopic keratoconjunctivitis, 46
Acute angle closure glaucoma, 25–28 Azathioprine, 59
Acute anterior uveitis (AAU), 51–53
Behçet’s disease, 55
Fuch’s heterochromic cyclitis, 55 B
herpetic anterior uveitis, 54 Bacterial keratitis, 41
HLA B27 associated, 52 Behçet’s disease, 55
management protocol, 125 Bell’s palsy, 82–83
Posner-Schlossman syndrome, 55 Beta blockers, 29
sarcoidosis, 54 Binocular double vision, 59
Acute dacryocystitis, 34 Birdshot retinopathy, 56
Acute retinal necrosis, 56 Birmingham Eye Trauma Terminology
Adenoviral keratoconjunctivitis, 40 (BETT), 8
Adrenal suppression, 32 Blast injury, 7
Afferent pupil defect (APD), 81 Blebitis, 22
Alkali burns, 12 Blepharitis
Allergic eye disease, 45–46 children, 49, 100
Alpha blocker, 29 classification, 47
Altitudinal field defect, 30 rosacea associated with, 48
Amaurosis fugax, 18 symptoms, 47
management protocol, 126 treatment, 48
transient ischaemic attacks, 63–64 Booked appointment system, 117
Amiodarone, 30 Botulinus toxin, 44
Amphoterecin B, 44 Branch retinal vein occlusion (BRVO), 28, 87
Angle recession, 14 Buphthalmos, infants and toddlers, 98
Anisocoria, 82, 99
Anterior ischaemic optic neuropathy (AION),
30–34 C
Anterior segment OCT, 25, 26, 75 Candida endophthalmitis, 22
Anterior uveitis, 55 Capillary haemangioma, 84, 97
acute, 51–54, 125 Carbonic anhydridase inhibitor, 29
in children, 55 Cavernous haemangioma, 84
herpetic, 54–55 Cavernous sinus disease, 60, 61

https://doi.org/10.1007/978-3-319-92369-7
132 Index
Cavernous sinus fistula, 60 Dermoids, 84

Cavernous sinus thrombosis, 34 Descemetocoele, 41, 42
Central retinal artery occlusion, 17–20 Desferrioxamine, 94
Central retinal vein occlusion (CRVO), 28, 87 Dexamethasone, 53
Central serous retinopathy (CSR), 90 Diplopia, 59
Cerebral vein thrombosis, 67 Disciform keratitis, 38
Chemical injury, 12 Drusen characteristics, 74
Chiari malformation, 68
Chlamydia, 40, 96
Chloroquine, 94 E
Choroidal haemangioma, 91 Electrodiagnostic testing, 84
Choroidal infarction, 90 Endogenous endophthalmitis, 22
Choroidal naevus, 91–92 Endophthalmitis, 8
Choroidal neovascularisation, 13 candida, 22
Choroidal rupture, 13 endogenous, 22, 23
CHRPE, 99 exogenous, 20, 23
Ciliary block glaucoma, 25 fungal, 23
Closed injury, 8 Endophthalmitis Vitrectomy study
Cluster headache, 66 (EVS), 21
Cochrane review, 105 Endothelial keratitis, 38, 39
Coloboma, 96 Endotheliitis, 38
Colour duplex ultrasonography, 32 Epithelium, 38
Commotio retinae, 13 Ethambutol, 93
Comprehensive ophthalmology, 1, 2 Ethmoid sinusitis, 34
Cone dystrophy, 97 Exogenous endophthalmitis, 20–22
Congenital cataract, 95 Eye injury
Congenital nystagmus, 97 classification, 8
Conjunctivitis, 40 corneal flash burns, 15
Contact dermatitis, 48 cyanoacrylate glue, 15
Contact lens associated corneal disease, 42 local anaesthesia, 15–16
Contact lens keratitis, 40 terrorist bomb attacks, 7–9
Contusion, 13–14 EyeWiki, 104
Conversion disorder, 76
Corneal abrasion, 15
Corneal disease, 98 F
Corneal flash burns, 15 Fingolimod, 94
Corneal thickness adjustments, for IOP, Fistula, 60
123–124 Fuch’s heterochromic cyclitis, 55
Corneal ulcer, 37 Fungal endophthalmitis, 23
bacterial ulcer, 40–42 Fungal ulcer, 43–44
classification, 37
fungal ulcer, 43
idiopathic ulcer, 49 G
management protocol, 125, 126 Ghost cell glaucoma, 29
neurotrophic ulcer, 39 Giant cell arteritis (GCA), 20, 31–34
non infective ulcer, 44–46 management protocol, 124
Cranial nerve palsy, 60–61 Glaucoma
acute angle closure, 25
ghost cell, 29
D infants and toddlers, 97
Dacryocystitis, 34 neovascular, 28
Demodex, 47, 100 phakomorphic, 29
Demographics, 1 secondary, 28–29
Index 133
subacute angle closure, 74 K

treatment, 29 Keratoconus, 46
Globe penetration, 9
Globe rupture, 9
Glycerol, 26 L
Goldmann tonometer (GAT), 126 Lacrimal abscess, 35
Google Advanced Search, 103 Laser peripheral iridotomy, 26
Gunderson flap technique, 42 Lateral canthotomy and cantholysis, 10
Leber’s amaurosis, 96
Leukocoria, 96
H Lid laceration, 11, 12
Haemorrhage, 9, 10 Lisch nodules, 100
Headache Lyme disease, 57
classification, 65
diagnosis, 65
examination, 66 M
intermittent angle closure, 75 Macular oedema, 87, 89, 91
medication overuse, 67, 68 Malingering, 76
primary, 66–67 Mannitol, 26
secondary, 67–69 Medication overuse headache, 67, 68
Herpes simplex keratitis, 37–39 Medscape, 104
Herpes zoster ophthalmicus (HZO), 39 Medtech innovation briefing, 28
Herpetic anterior uveitis, 54 Medulloblastoma, 100
HLA B27 associated uveitis, 52 Meningioma, 84
Holmes-Adie pupil, 82 Methotrexate, 51, 59
Horner’s syndrome, 60, 82 Migraine, 63, 66, 98
Hospital ophthalmology, 1 Minor eye care services
Hospital premium collection, 104, 105 (MECS), 110, 111
Hydroxychloroquine retinopathy, 94 Monocular diplopia, 59
Hypercoagulability, 20, 88 Mooren’s ulcer, 49
Hyphaema, 14 Myasthenia gravis, 62
Mydriatics, 15
I
Icare tonometry, 9, 10 N
Idiopathic intracranial hypertension National Institute for Health and Care
(IIH), 68 Excellence (NICE), 103–105
Immunosuppression, 59 Neonatal referrals, 95–96
Inflammatory chorioretinopathy, 56–57 Neovascular glaucoma, 28
Intermediate uveitis, 55 Neovascularisation, 89
Intracranial pressure (ICP) Networking, 127
disc swelling, 69 Neurofibromatosis type I (NF type 1), 100
headache, 67 Neurofibromatosis, school age children, 100
Intracranial tumour, school age Neurological visual field defect, 76
children, 100 Neuromyelitis optica (NMO), 78
Intraocular foreign body, 7 Neurotrophic keratitis, 40
Intraocular haemorrhage, 7 Neurotrophic ulcer, 39, 44
Intravitreal antibiotics, 23 NHS contract with optometrists, 1
Isoniazid, 93 Non accidental injury, infants and toddlers, 97
Non traumatic eye emergency, 20
central retinal artery occlusion, 17
J endophthalmitis (see Endophthalmitis)
Juvenile idiopathic arthritis (JIA), 55 Nystagmus, infants and toddlers, 97
134 Index
O R
Ocular ischaemic syndrome, 28 Rapid access booked appointments, 118
Ocular Trauma Score (OTS), 8 Rapid access ophthalmology, 2, 3
Open injury, 8 Rapid access service, 110
Ophthalmia neonatorum, 96 Red eye symptoms, 119
Ophthalmology, 1, 2 Red flag headache, 67
Optic nerve glioma, 84, 100 Referrals to ophthalmology, 109
Optic neuritis, 78–80 for elderly, 93
Optic neuropathy, 63, 79, 80, 93 neonatal, 95–96
Optometry referrals, school age children, 99 school age children, 99–101
Orbital apex, 60, 61 Relative afferent pupil defect (RAPD), 81
Orbital cellulitis, 31–35 Retinal detachment, 8, 30
Orbital fracture, 11 Retinal ischaemia, 88
Organisation and management Retinal migraine, 66
audits, 127 Retinal tear, 30, 92
networking, 127 Retinal vasculitis, 56
patient information and education, 127 Retinal vein occlusion, 87–90
staff training, 119–120 Retinoblastoma, 96
standard operating procedures (SOP), 126 Retrobulbar haemorrhage, 9, 10
walk in service Rhabdomyosarcoma, 84
categories, 117 Rosacea, 48
guidelines, 117 Rubeotic glaucoma, 25
volume and workload planning,
113–114
workforce planning, 113–116 S
Sagging eye syndrome, 63
Sarcoidosis, 54
P Scleritis, 49
Painful loss of vision, 120 Secondary care, 2, 42
Papilloedema, 69–74 Service equipments, 121–124
Paraneoplastic syndrome, 92 Sildenafil, 30
Periorbital edema, 34 Skew deviation, 61
Phakolytic glaucoma, 29 Sleep apnoea, 30
Phakomorphic glaucoma, 29 Strabismus, infants and toddlers, 98–99
Photophobia, infants and toddlers, 98 Strawberry naevus, infants and toddlers, 97
Phthiriasis palpebrarum, 48 Stroke, 18, 64
Pilocarpine, 25–27, 29, 30, 82 Stromal keratitis, 38
Polymyalgia rheumatica (PMR), 31 Subacute angle closure glaucoma, 74–75
Poor visual response, infants and toddlers, 96 Subperiosteal abscess, 34
Posner-Schlossman syndrome, 55 Subretinal hyporeflective space (SHS), 72
Posterior uveitis, 56 Sudden loss of vision, 120
Posterior vitreous detachment (PVD), 30 Supranuclear ophthalmoplegia, 62
Prebooked rapid access service, 117
Prednisolone, 32
Preseptal cellulitis, 34 T
Primary care, 2, 34, 47 Tamoxifen, 94
Primary eye care assessment and referral Temporal artery biopsy (TAB), 31–33
services (PEARS), 110, 111 Tension headache, 66
Propionibacterium acnes, 20–22 Thermal burns, 13
Proptosis, 83–84 Thrombosis, 60
Prostaglandin inhibitor, 29 Thunderclap headache, 68
Pseudomonas aeruginosa, 41 Thyroid eye disease, 62–63
Pupil light reflex, 80 Toxic anterior segment syndrome, 22
Index 135
Toxic optic neuropathy, 79, 80 Vernal keratoconjunctivitis, 45

Toxoplasma choroiditis, 56 Vigabatrin, 94
Transient ischaemic attack (TIA), 63 Visual acuity
Traumatic choroidal rupture, 13 recording, 123
Trigeminal autonomic cephalgias, 66 using gratings with preferential looking
Tropicamide, 26 infants, 123
Visual field defect, 76–78
Vitrectomy, 21–23
U Vitreous biopsy, 21
Ultrasound, 51 Vitreous haemorrhage, 14, 92
Ultrawidefield retinal imaging (Optos), 30
Uveitis, 51
acute anterior (see Acute anterior W
uveitis (AAU)) Walk in service
anterior, 55 categories, 117
classification, 53 guidelines, 117
intermediate, 55 volume and workload
medical evaluation, 57–58 planning, 113
posterior, 56 White dot syndromes, 56, 57
Workforce planning, 115
V
Van Herrick’s method, 75 Y
Vasculitis, 19, 20, 91 YAG PI technique, 27

Emergency Acute and Rapid Access Ophthalmology Practical, Clinical and Managerial Aspects 2019 PDF

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Emergency, Acute

and Rapid Access

Practical, Clinical and Managerial

Emergency, Acute and Rapid

ISBN 978-3-319-92368-0 ISBN 978-3-319-92369-7 (eBook)

Library of Congress Control Number: 2018954715

© Springer Nature Switzerland AG 2019

Part I Clinical Aspects

5 Conditions Requiring Same Day Management������������������������������������ 37

7.6 Vitreous Haemorrhage���������������������������������������������������������������������� 92

Part II Leading an Emergency and Rapid Access Service

12.8 Networking ������������������������������������������������������������������������������������ 127

Josephine Duvall-Young trained in general oph-

Fig. 2.1 Icare rebound tonometer ������������������������������������������������������������������ 10

Table 2.1 Birmingham Eye Trauma Terminology (BETT)������������������������������ 8

1.1 What Is the Problem?

In recent decades, ophthalmology has become increasingly superspecialised, with

© Springer Nature Switzerland AG 2019 1

1.2 Defining the Problem

1.3 The Book

2.1 Terrorist Attack or Major Incident

© Springer Nature Switzerland AG 2019 7

Eye injuries are classified with standardised terminology according to the

Table 2.1 Birmingham Eye Trauma Terminology (BETT)

Table 2.2 Ocular Trauma Score (OTS)

2.2 Globe Penetration and Rupture

2.3 Retrobulbar Haemorrhage

While thinking about haemorrhage, retrobulbar bleeding, either caused by orbital

Fig. 2.1 Icare rebound tonometer

Emergency Lateral Canthotomy and Cantholysis

2.4 Orbital Fracture

Ophthalmologists are often asked to give an opinion on orbital fractures. Their

2.5 Lid Injury

Technique for Repair of Lid Lacerations

2.6 Chemical and Thermal Burns

2.8 Minor Injuries

2.9 Local Anaesthesia

3.1 Central Retinal Artery Occlusion

© Springer Nature Switzerland AG 2019 17

PATIENT DETAILS DATE AND TIME

PRESENTING SYMPTOMS Date and time of onset

bp pulse Regular Y/N

ABCD2 Score SCORE POINTS

Score 2 or 3, e-mail referral. Patient will be seen in (check)

Vascular Disease Investigations

hypercoagulability may be started or if being seen immediately by a physician,

Exogenous endophthalmitis, resulting from an infective organism being intro-

be overemphasised. The rate of development of the inflammatory reaction and the

Technique for Vitreous Biopsy

A delayed chronic form of postoperative endophthalmitis is recognised. This can

Differential Diagnosis of Red Eye Post Operatively

Treatment of Exogenous Endophthalmitis

Treatment of Endogenous Endophthalmitis

Treatment of Fungal Endophthalmitis

Endophthalmitis Study Group. Results of the endophthalmitis vitrectomy study. A randomized

4.1 Acute Angle Closure Glaucoma

© Springer Nature Switzerland AG 2019 25

Protocol for Management of Acute Angle Closure

Icare NICE Medtech Innovation Briefing

4.2 Secondary Glaucomas

Glaucoma Drop Treatment

4.3 Retinal Detachment

4.4 Anterior Ischaemic Optic Neuropathy

Part I Clinical Aspects

5 Conditions Requiring Same Day Management�� 37

7.6 Vitreous Haemorrhage�� 92

Part II Leading an Emergency and Rapid Access Service

12.8 Networking �� 127

Fig. 2.1 Icare rebound tonometer �� 10

Table 2.1 Birmingham Eye Trauma Terminology (BETT)�� 8

1.1 What Is the Problem?

1.2 Defining the Problem

1.3 The Book

2.1 Terrorist Attack or Major Incident

2.2 Globe Penetration and Rupture

2.3 Retrobulbar Haemorrhage

2.4 Orbital Fracture

2.5 Lid Injury

2.6 Chemical and Thermal Burns

2.8 Minor Injuries

2.9 Local Anaesthesia

3.1 Central Retinal Artery Occlusion

4.1 Acute Angle Closure Glaucoma

4.2 Secondary Glaucomas

4.3 Retinal Detachment

4.4 Anterior Ischaemic Optic Neuropathy

4.4.1 Giant Cell Arteritis

4.5 Orbital Cellulitis

5.1 Corneal Ulcers

5.1.1 Herpes Simplex Keratitis

5.1.2 Herpes Zoster Ophthalmicus (HZO)

5.1.3 Adenoviral Keratoconjunctivitis

5.1.4 Bacterial Ulcer

5.1.5 Contact Lens Associated Corneal Disease

5.1.6 Acanthamoeba Keratitis

5.1.7 Fungal Ulcers

5.1.8 Non Infective Ulcers

5.1.11 Keratitis Secondary to Blepharitis

5.1.12 Idiopathic Keratitis

5.3.1 Acute Anterior Uveitis (AAU)

5.3.2 Identifiable Associations of AAU

5.3.3 Inflammatory Chorioretinopathies

5.3.4 Lyme Disease

5.3.5 Medical Evaluation of Uveitis Patients

5.4 Recent Onset or Progressive Ophthalmoplegias

5.5 Transient Ischaemic Attack (TIA)