John F.

Salmon
MD, FRCS, FRCOphth
Consultant Ophthalmic Surgeon
Oxford Eye Hospital
Oxford Radcliffe NHS Trust
Oxford
United Kingdom

KANSKI'S
Clinical
Ophthalmology
A Systematic Approach

Tenth Edition

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 Dedication
This book is dedicated to my grandsons,
Jack and Sam Spath and Ronan Salmon

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 ix

Preface to the Tenth Edition

As has been the approach adopted in previous editions of this clas- in the inherited eye disease chapter; Dr Christine Kiire the text
sic textbook, the tenth edition of Kanski’s Clinical Ophthalmology in the diabetic retinopathy section; Dr Richard Corkin the text
is intended to be a useful and comprehensive basis for general oph- in the refractive surgery section; and Professor Gordon Dutton
thalmic and optometric practice. The book is filled with in-depth the text in the cerebral visual impairment section. I have received
information on most ophthalmic diseases and includes hundreds numerous excellent images from Aude Ambresin (medical retina),
of beautiful images and illustrations. I have included sufficient Trevor Carmichael (cornea), Bertil Damato (ocular oncology),
practical information for trainees to manage common ophthalmic Darius Hildebrand and Manoj Parulekar (paediatrics), Jonathan
conditions in the clinic and enough detail on rare conditions to Norris and Elizabeth Insull (ocular plastics), Geoff Rose and Andy
enable them to prepare for their examinations without resorting Pearson (orbits/ocular plastics), Martin Leyland (corneal surgery)
to the internet. and Pieter Pretorius (neuroradiology). I have kept many of the out-
The challenge has been to cover the entire field of ophthalmology standing pictures that Chris Barry, Simon Cheng, Mitch Ménage,
for a world-wide audience without depending on sub-specialists to Stephen Tuft and Pablo Gili have provided for previous editions.
prepare each chapter. To do this, I have maintained Jack Kanski’s Terry Tarrant’s beautiful paintings, which have been a character-
unique approach of presenting core clinical knowledge in a sys- istic feature of the book in the past, have been maintained in this
tematic and succinct form. Each chapter has been considerably edition. Images of rare conditions have been generously provided
updated and the latest evidence-based diagnostic and therapeutic by a number of colleagues throughout the United Kingdom and
approaches have been covered, including genetics and imaging elsewhere in the world and their contribution has been recognised
techniques. Many new illustrations have been added and better next to the relevant figures. I wish to thank the team at Elsevier for
examples of a range of conditions have been included. An enhanced their meticulous input in the production of the book, especially
eBook version is available for rapid access. Kayla Wolfe, Joanie Milnes, Elaine Leek and Julie Taylor.
I have been extremely fortunate to have received help from col- I have once again had the good fortune to work with Jon Brett,
leagues past and present, to whom I wish to express my grateful a world-class photographer and artist, whose creativity and genius
thanks. Brad Bowling has had a significant influence on previ- is present in many of the images included in this edition. I am
ous editions and his accuracy and meticulous attention to detail extremely grateful for his diligence, skill and support.
have been remarkably helpful. Professor Tony Murray provided
considerable advice on the text in the strabismus chapter and John F. Salmon
images of pathology that are not easily found in higher-income 2025
countries. Professor Peter Charbel Issa helped to update the text

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x

Abbreviations

AAION arteritic anterior ischaemic optic neuropathy C-MIN conjunctival melanocytic intraepithelial neoplasia
AAU acute anterior uveitis CMO cystoid macular oedema (US = CME)
AC anterior chamber CNS central nervous system
AC/A ratio accommodative convergence/accommodation ratio CNV choroidal neovascularization
ACE angiotensin-converting enzyme CNVM choroidal neovascular membrane
AD autosomal dominant COX-2 cyclo-oxygenase-2
AF autofluorescence CPEO chronic progressive external ophthalmoplegia
AGIS Advanced Glaucoma Treatment Study CRAO central retinal artery occlusion
AHP abnormal head posture CRP C-reactive protein
AI accommodative insufficiency CRVO central retinal vein occlusion
AIBSE acute idiopathic blind spot enlargement syndrome CSMO clinically significant macular oedema (US = CSME)
AIDS acquired immune deficiency syndrome CSR central serous chorioretinopathy
AIM (unilateral) acute idiopathic maculopathy CSS central suppression scotoma
AION anterior ischaemic optic neuropathy CT computed tomography
AIR autoimmune retinopathies CVI cerebral visual impairment
AKC atopic keratoconjunctivitis CXL corneal collagen cross-linking
ALT argon laser trabeculoplasty DALK deep anterior lamellar keratoplasty
AMD age-related macular degeneration DCCT Diabetes Control and Complication Trial
AMN acute macular neuroretinopathy DCR dacryocystorhinostomy
ANA antinuclear antibody DCT dynamic contour tonometry
ANCA antineutrophil cytoplasmic antibodies DMEK Descemet membrane endothelial keratoplasty
APD afferent pupillary defect DMO diabetic macular oedema (US = DME)
APMPPE acute posterior multifocal placoid pigment DR diabetic retinopathy
epitheliopathy DRCR.net Diabetic Retinopathy Clinical Research Network
AR autosomal recessive DRPPT dark room prone provocative test
ARDE age-related distance esotropia DRS Diabetic Retinopathy Study
AREDS Age-Related Eye Disease Study DSAEK Descemet stripping automated endothelial
ARN acute retinal necrosis keratoplasty
ARPE acute retinal pigment epitheliitis DVD dissociated vertical deviation
AZOOR acute zonal occult outer retinopathy ECG electrocardiogram
AZOR acute zonal outer retinopathy EDTA ethylenediaminetetraacetic acid
BADI bilateral acute depigmentation of the iris EGFR epidermal growth factor inhibitors
BCC basal cell carcinoma EKC epidemic keratoconjunctivitis
BCVA best-corrected visual acuity EMGT Early Manifest Glaucoma Trial
BIO binocular indirect ophthalmoscopy EOG electro-oculography/gram
BP blood pressure ERG electroretinography/gram
BRAO branch retinal artery occlusion ERM epiretinal membrane
BRVO branch retinal vein occlusion ESR erythrocyte sedimentation rate
BSV binocular single vision ETDRS Early Treatment Diabetic Retinopathy Study
BUT breakup time ETROP Early Treatment of Retinopathy of Prematurity
CAI carbonic anhydrase inhibitor FA fluorescein angiography (also FFA)
CCDD congenital cranial dysinnervation disorders FAF fundus autofluorescence
CCT central corneal thickness FAP familial adenomatous polyposis
C/D cup/disc FAZ foveal avascular zone
CDCR canaliculodacryocystorhinostomy FBA frosted branch angiitis
CF counts (or counting) fingers FBC full blood count
CHED congenital hereditary endothelial dystrophy FDT frequency doubling test
CHP compensatory head posture FFM fundus flavimaculatus
CHRPE congenital hypertrophy of the retinal pigment FTMH full-thickness macular hole
epithelium 5-FU 5-fluorouracil
CI convergence insufficiency GA geographic atrophy

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 Abbreviations xi

GAT Goldmann applanation tonometry MRI magnetic resonance imaging

GCA giant cell arteritis MS multiple sclerosis
GDD glaucoma drainage device NF1 neurofibromatosis type 1
GHT glaucoma hemifield test NF2 neurofibromatosis type 2
GPA guided progression analysis NPDR non-proliferative diabetic retinopathy
GPC giant papillary conjunctivitis NRR neuroretinal rim
G-TOP glaucoma tendency orientated perimetry NSAID non-steroidal anti-inflammatory drug
HAART highly active antiretroviral therapy NSR neurosensory retina
HFA Humphrey field analyser NTG normal-tension glaucoma
HIV human immunodeficiency virus NVD new vessels on the disc
HM hand movements NVE new vessels elsewhere
HRT Heidelberg retinal tomography NVG neovascular glaucoma
HSV-1 herpes simplex virus type 1 NVI new vessels iris
HSV-2 herpes simplex virus type 2 OCT optical coherence tomography/gram
HZO herpes zoster ophthalmicus OCTA optical coherence tomography angiography
ICE iridocorneal endothelial OHT ocular hypertension
ICG indocyanine green OHTS Ocular Hypertension Treatment Study
ICGA indocyanine green angiography OIS ocular ischaemia syndrome
ICROP International Classification of Retinopathy of OKN optokinetic nystagmus
Prematurity OVD ophthalmic viscosurgical devices
IFIS intraoperative floppy iris syndrome PAC primary angle closure
Ig immunoglobulin PACG primary angle-closure glaucoma
IK interstitial keratitis PACS primary angle-closure suspect
ILM internal limiting membrane PAM primary acquired melanosis
IMT idiopathic macular telangiectasia PAN polyarteritis nodosa
INO internuclear ophthalmoplegia PAS peripheral anterior synechiae
INR international normalized ratio PC posterior chamber
IOFB intraocular foreign body PCO posterior capsular opacification
IOID idiopathic orbital inflammatory disease PCR polymerase chain reaction
IOL intraocular lens PCV polypoidal choroidal vasculopathy
IOP intraocular pressure PDR proliferative diabetic retinopathy
IRMA intraretinal microvascular abnormality PDS pigment dispersion syndrome
IRVAN idiopathic retinal vasculitis, aneurysms and PDT photodynamic therapy
neuroretinitis syndrome
PED pigment epithelial detachment
ITC iridotrabecular contact
PEE punctate epithelial erosions
IU intermediate uveitis
PEK punctate epithelial keratitis
IVTS International Vitreomacular Traction Study
PEHCR peripheral exudative haemorrhagic
JIA juvenile idiopathic arthritis chorioretinopathy
KC keratoconus PH pinhole
KCS keratoconjunctivitis sicca PHM posterior hyaloid membrane
KP keratic precipitate PIC punctate inner choroidopathy
LA local anaesthetic PIOL primary intraocular lymphoma
LASEK laser (also laser-assisted) epithelial keratomileusis PION posterior ischaemic optic neuropathy
LASIK Laser (also laser-assisted) in situ keratomileusis PKP penetrating keratoplasty
LN latent nystagmus PMMA polymethyl methacrylate
LSCD limbal stem cell deficiency POAG primary open-angle glaucoma
LV loss variance POHS presumed ocular histoplasmosis syndrome
MALT mucosa-associated lymphoid tissue PP pars planitis
MCP multifocal choroiditis and panuveitis PPCD posterior polymorphous corneal dystrophy
MEWDS multiple evanescent white dot syndrome PPDR preproliferative diabetic retinopathy
MFC multifocal choroiditis and panuveitis PPM persistent placoid maculopathy
MIGS minimally invasive glaucoma surgery PPRF paramedian pontine reticular formation
MLF medial longitudinal fasciculus PPV pars plana vitrectomy
MLT micropulse laser technology PRK photorefractive keratectomy
MMC mitomycin C PRP panretinal photocoagulation
MNV macular neovascularization PS posterior synechiae
MPS mucopolysaccharidosis

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xii Abbreviations

PSD pattern standard deviation SJS Stevens–Johnson syndrome

PSS Posner–Schlossman syndrome SLK superior limbic keratoconjunctivitis
PUK peripheral ulcerative keratitis SLT selective laser trabeculoplasty
PVD posterior vitreous detachment SMILE small incision lenticule extraction
PVR proliferative vitreoretinopathy SPARCS Spaeth Richman contrast sensitivity test
PVRL primary vitreoretinal lymphoma SPK superficial punctate keratitis
PXE pseudoxanthoma elasticum SRF subretinal fluid
PXF pseudoexfoliation SS Sjögren syndrome
RA rheumatoid arthritis STIR short T1 inversion recovery
RAO retinal artery occlusion SWAP short-wave automated perimetry
RAPD relative afferent pupillary defect TAL total axial length
RD retinal detachment TB tuberculosis
RNFL retinal nerve fibre layer TEN toxic epidermal necrolysis
ROCK Rho-kinase TGF transforming growth factor
ROP retinopathy of prematurity TIA transient ischaemic attack
RP retinitis pigmentosa TM trabecular meshwork
RPC relentless placoid chorioretinitis TRD tractional retinal detachment
RPE retinal pigment epithelium TRP targeted retinal photocoagulation
RRD rhegmatogenous retinal detachment TTT transpupillary thermotherapy
RS retinoschisis UBM ultrasonic biomicroscopy
RVO retinal vein occlusion US ultrasonography
SACE serum angiotensin-converting enzyme VA visual acuity
SANS space flight-associated neuro-ocular syndrome VEGF vascular endothelial growth factor
SAP standard automated perimetry VEP visual(ly) evoked potential(s)
SCC squamous cell carcinoma VFI visual field index
SCN suprachiasmic nucleus VHL von Hippel–Lindau syndrome
SD-OCT spectral domain optical coherence tomography VKC vernal keratoconjunctivitis
SES sagging eye syndrome VKH Vogt–Koyanagi–Harada syndrome
SF short-term fluctuation VMA vitreomacular adhesion
SFU progressive subretinal fibrosis and uveitis syndrome VMT vitreomacular traction
SIC solitary idiopathic choroiditis VZV varicella zoster virus
SITA Swedish Interactive Thresholding Algorithm XL X-linked

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 Chapter

Examination Techniques 1
OPHTHALMIC HISTORY 2 High-sensitivity field modalities 17 Head-mounted binocular indirect
Sources of error 18 ophthalmoscopy 25
OPHTHALMIC EXAMINATION 2 Microperimetry 18 Fundus drawing 27
PSYCHOPHYSICAL TESTS 2 SLIT LAMP BIOMICROSCOPY OF TONOMETRY 28
Visual acuity 2 THE ANTERIOR SEGMENT 20 Goldmann tonometry 28
Contrast sensitivity 4 Direct illumination 20 Other forms of tonometry 30
Amsler grid 5 Scleral scatter 21 Ocular response analyser and
Light brightness comparison Retroillumination 21 corneal hysteresis 31
test 6 Specular reflection 21
Photostress test 6 GONIOSCOPY 31
Portable slit lamp 21
Colour vision testing 7 Introduction 31
Plus lens test 9 FUNDUS EXAMINATION 21 Indirect gonioscopy 32
Direct ophthalmoscopy 21 Direct gonioscopy 33
PERIMETRY 9 Slit lamp biomicroscopy 23 Surgical gonioscopy 33
Definitions 9 Using new technology for retinal Identification of angle
Testing algorithms 11 imaging in rural settings 23 structures 35
Testing patterns 11 Goldmann three-mirror Pathological findings 36
Analysis 14 examination 23
CENTRAL CORNEAL THICKNESS 36

1
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2 Psychophysical Tests

OPHTHALMIC HISTORY
Before examining the patient, a thorough ophthalmic history
should be taken. A detailed history will depend on the particular
visual symptoms and suspected ocular disease. However, a basic
history can be divided into the following categories of questioning:
zzMain complaint: (a) rapidity of onset, (b) circumstances sur-
rounding the onset, (c) severity, (d) duration of symptoms, (e)
frequency of symptoms.
zzPast ocular history: e.g. previous surgery, inflammation,
trauma.
zzPast medical history: e.g. diabetes and hypertension.
zzSystemic medication: e.g. corticosteroids, tamsulosin.
zzAllergies: e.g. antibiotics, topical glaucoma medications.
zzFamily history: e.g. glaucoma, macular degeneration, inherited
retinal disease.
zzOccupation and hobbies!

OPHTHALMIC EXAMINATION
Fig. 1.1 Snellen visual acuity chart.
Patients with ophthalmic disease must have their vision accurately
measured and their eyes need to be examined using specialized
techniques. Special investigations should be used to supplement
the findings of clinical examination. Electrophysical tests, fluores-
cein angiography and optical coherence tomography are discussed
in later chapters.!

PSYCHOPHYSICAL TESTS
Visual acuity
Snellen visual acuity
Distance visual acuity (VA) is directly related to the minimum angle
of separation (subtended at the nodal point of the eye) between two
objects that allow them to be perceived as distinct. In practice, it
is most commonly carried out using a Snellen chart, which utilizes
black letters or symbols (optotypes) of a range of sizes set on a white
chart (Fig. 1.1), with the subject reading the chart from a standard Fig. 1.2 Pinhole.
distance. Distance VA is usually first measured using a patient’s
refractive correction, generally their own glasses or contact lenses.
For completeness, an unaided acuity may also be recorded. The eye zzBinocular VA is usually superior to the better monocular VA
reported as having worse vision should be tested first, with the other of each eye, at least where both eyes have roughly equal vision.!
eye occluded. It is important to push the patient to read every letter
possible on the optotypes being tested. Very poor visual acuity
zzNormal monocular VA equates to 6/6 (metric notation; 20/20 zzCounting (or counts) fingers (CF) denotes that the patient is
in non-metric ‘English’ notation) on Snellen testing. Normal able to tell how many fingers the examiner is holding up at a
corrected VA in young adults is often superior to 6/6. specified distance (Fig. 1.3), usually 1 metre.
zzBest-corrected VA (BCVA) denotes the level achieved with zzHand movements (HM) is the ability to distinguish whether
optimal refractive correction. the examiner’s hand is moving when held just in front of the
zzPinhole VA: a pinhole (PH) aperture compensates for the patient.
e%ect of refractive error, and consists of an opaque occluder zzPerception of light (PL): the patient can discern only light
perforated by one or more holes of about 1 mm diameter (Fig. (e.g. pen torch), but no shapes or movement. Careful occlu-
1.2). However, PH acuity in patients with macular disease and sion of the other eye is necessary. If poor vision is due solely
posterior lens opacities may be worse than with spectacle cor- to a dense media opacity such as cataract, the patient should
rection. If the VA is less than 6/6 Snellen equivalent, testing is readily be able to determine the direction from which the light
repeated using a pinhole aperture. is being projected (Fig. 1.4).!

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Examination Techniques 3

Table 1.1 Comparison of Snellen and LogMAR Visual

Acuity Testing
Snellen LogMAR
Shorter test time Longer test time
More letters on the Equal numbers of letters on
lower lines introduces an different lines controls for
unbalanced ‘crowding’ ‘crowding’ effect
effect
Fewer larger letters reduce Equal numbers of letters
accuracy at lower levels on low and higher acuity
of VA lines increases accuracy at
lower VA
Variable readability Similar readability between
between individual letters letters
Lines not balanced with Lines balanced for
each other for consistency consistency of readability
Fig. 1.3 Testing of ‘counting fingers’ vision. of readability
6"m testing distance: longer 4"m testing distance on
testing lane (or a mirror) many charts: smaller
required testing lane (or no mirror)
required
Letter and row spacing not Letter and row spacing
systematic set to optimize contour
interaction
Lower accuracy and Higher accuracy and
consistency so relatively consistency so appropriate
unsuitable for research for research
Straightforward scoring More complex scoring
system
Easy to use Less user-friendly

zzAs letter size changes by 0.1 logMAR units per row and there
are five letters in each row, each letter can be assigned a score of
0.02. The final score can therefore take account of every letter
that has been read correctly and the test should continue until
Fig. 1.4 Notation for the projection of light test (right eye); the half of the letters on a line are read incorrectly.
patient cannot detect light directed from the superior and zzFor conversion of logMAR into metres and feet: see
temporal quadrants. Table 1.2.!

LogMAR charts
LogMAR acuity zzThe Bailey–Lovie chart (Fig. 1.5)
LogMAR charts address many of the deficiencies of the Snellen {z Used at 6 m testing distance.
chart (Table 1.1), and are the standard means of VA measurement {z Each line of the chart comprises five letters and the spacing
in research and, increasingly, in clinical practice. between each letter and each row is related to the width
zzLogMAR is an acronym for the base-10 logarithm of the mini- and the height of the letters. The letter signs are rectangular
mum angle of resolution (MAR) and refers to the ability to rather than square, as with the EDTRS chart. A 6/6 letter is
resolve the elements of an optotype. Thus, if a letter on the 6/6 5′ in height by 4′ in width.
(20/20) equivalent line subtends 5′ of arc, and each limb of the {z The distance between two adjacent letters on the same row
letter has an angular width of 1′, a MAR of 1′ is needed for is equal to the width of a letter from the same row, and
resolution. For the 6/12 (20/40) line, the MAR is 2′, and for the the distance between two adjacent rows is the same as the
6/60 (20/200) line it is 10′. height of a letter from the lower of the two rows.
zzThe logMAR score is simply the base-10 log of the MAR. {z Snellen VA values and logMAR VA are listed to the right
Because the log of the MAR value of 1′ is zero, 6/6 is equivalent and left of the rows respectively.
to logMAR 0.00. The log of the 6/60 MAR of 10′ is 1, so 6/60 zzThe ETDRS (Early Treatment Diabetic Retinopathy Study)
is equivalent to logMAR 1.00. The log of the 6/12 MAR of 2′ chart is calibrated for 4 m. This chart utilizes balanced rows
is 0.301, giving a logMAR score of 0.30. Scores better than 6/6 comprising Sloan optotypes, developed to confer equivalent
have a negative value. legibility between individual letters and rows. ETDRS letters

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4 Psychophysical Tests

Table 1.2 Conversion of Snellen Visual Acuity from

LogMAR
LogMAR Metre Foot Decimal
1.00 6/60 20/200 0.10
0.90 6/48 20/160 0.125
0.80 6/38 20/125 0.16
0.70 6/30 20/100 0.20
0.60 6/24 20/80 0.25
0.50 6/19 20/63 0.32
0.40 6/15 20/50 0.40
0.30 6/12 20/40 0.50
0.20 6/9.5 20/32 0.63
0.10 6/7.5 20/25 0.80
0.00 6/6 20/20 1.00
−0.10 6/4.8 20/16 1.25 Fig. 1.6 Near visual acuity using a magnifying lens.
−0.20 6/3.8 20/12.5 1.60
−0.30 6/3 20/10 2.00 own reading spectacles). The smallest type legible is recorded for
each eye individually and then using both eyes together (Fig. 1.6).!

Contrast sensitivity
zzPrinciples. Contrast sensitivity is a measure of the ability of the
visual system to distinguish an object against its background.
A target must be sufficiently large to be seen, but must also be
of high enough contrast with its background. A light grey letter
will be less well seen against a white background than a black
letter. Contrast sensitivity represents a di%erent aspect of visual
function to that tested by the spatial resolution tests described
above, which all use high-contrast optotypes.
{z Many conditions reduce both contrast sensitivity and VA,
but under some circumstances (e.g. amblyopia, optic neu-
ropathy, some cataracts, and higher-order aberrations),
visual function measured by contrast sensitivity can be
reduced whilst VA is preserved.
Fig. 1.5 Bailey–Lovie chart. {z Hence, if patients with good VA complain of visual symp-
toms (typically evident in low illumination), contrast
are square, based on a 5 × 5 grid, i.e. 5′ × 5′ for the 6/6 equiva- sensitivity testing may be a useful way of objectively dem-
lent letters at 6 m. onstrating a functional deficit. Despite its advantages, it
zzComputer charts are available that present the various forms has not been widely adopted in clinical practice.
of test chart on display screens, including other means of zzThe Pelli–Robson contrast sensitivity letter chart is viewed at
assessment such as contrast sensitivity (see below). 1 metre and consists of rows of letters of equal size (spatial fre-
quency of 1 cycle per degree) but with decreasing contrast of
TIP LogMAR charts are commonly used in clinical trials 0.15 log units for groups of three letters (Fig. 1.7). The patient
because they are the most accurate method of measuring reads down the rows of letters until the lowest-resolvable
group of three is reached.
visual acuity.
! zzSinusoidal (sine wave) gratings require the test subject to
view a sequence of increasingly lower contrast gratings.
Near visual acuity zzSpaeth Richman contrast sensitivity test (SPARCS) is per-
Near vision testing can be a sensitive indicator of the presence of formed on a personal computer with internet access. It can be
macular disease. A range of near vision charts (including logMAR accessed online. Each patient is supplied with an identification
and ETDRS versions) or a test-type book can be used. The book or number and instructions on how to do the test. The test takes
chart is held at a comfortable reading distance and this is measured 5–10 minutes per eye and measures both central and periph-
and noted. The patient wears any necessary distance correction eral contrast sensitivity. Since the test is based on gratings it
together with a presbyopia correction if applicable (usually their can be used on illiterate patients.!

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Examination Techniques 5

Fig. 1.7 Pelli–Robson contrast sensitivity letter chart. Fig. 1.8 Amsler grid superimposed on the macula.

Amsler grid
The Amsler grid evaluates the 20° of the visual field centred on
fixation (Fig. 1.8). It is principally useful in screening for and mon-
itoring macular disease, but will also demonstrate central visual
field defects originating elsewhere. Patients with a substantial risk
of macular neovascularization (MNV) should be provided with an
Amsler grid for regular use at home.

TIP An Amsler grid is a simple and easy method of monitoring

central visual field and is commonly abnormal in patients with
macular disease.

Charts
There are seven charts, each consisting of a 10-cm outer square
Fig. 1.9 Amsler grid chart.
(Figs 1.9 and 1.10).
zzChart 1 consists of a white grid on a black background, the
outer grid enclosing 400 smaller 5-mm squares. When viewed Technique
at about one-third of a metre, each small square subtends an The pupils should not be dilated, and in order to avoid a photo-
angle of 1°. stress e%ect, the eyes should not yet have been examined on the slit
zzChart 2 is similar to chart 1 but has diagonal lines that aid fixa- lamp. A presbyopic refractive correction should be worn if appro-
tion for patients with a central scotoma. priate. The chart should be well illuminated and held at a comfort-
zzChart 3 is identical to chart 1 but has red squares. The red- able reading distance, optimally around 33 cm.
on-black design aims to stimulate long wavelength foveal zzOne eye is covered.
cones. It is used to detect subtle colour scotomas and desatu- zzThe patient is asked to look directly at the central dot with the
ration in toxic maculopathy, optic neuropathy and chiasmal uncovered eye, to keep looking at this, and to report any dis-
lesions. tortion or waviness of the lines on the grid.
zzChart 4 consists only of random dots and is used mainly to dis- zzReminding the patient to maintain fixation on the central dot,
tinguish scotomas from metamorphopsia, as there is no form he or she is asked if there are blurred areas or blank spots any-
to be distorted. where on the grid. Patients with macular disease often report that
zzChart 5 consists of horizontal lines and is designed to detect the lines are wavy whereas those with optic neuropathy tend to
metamorphopsia along specific meridians. It is of particular remark that some of the lines are missing or faint but not distorted.
use in the evaluation of patients describing difficulty reading. zzThe patient is asked if he or she can see all four corners and all
zzChart 6 is similar to chart 5 but has a white background and four sides of the square – a missing corner or border should
the central lines are closer together, enabling more detailed raise the possibility of causes other than macular disease, such
evaluation. as glaucomatous field defects or retinitis pigmentosa.
zzChart 7 includes a fine central grid, each square subtending an zzThe patient may be provided with a recording sheet and pen
angle of a half degree, and is more sensitive.! and asked to draw any anomalies (Fig. 1.11).!

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6 Psychophysical Tests

Chart 2 Chart 3 Chart 4

Chart 5 Chart 6 Chart 7

Fig. 1.10 Amsler charts 2–7.

Light brightness comparison test

This is a test of optic nerve function, which is usually normal in
early and moderate retinal disease. It is performed as follows:
zzA light from an indirect ophthalmoscope is shone first into the
normal eye and then the eye with suspected disease.
zzThe patient is asked whether the light is symmetrically bright
in both eyes.
zzIn optic neuropathy the patient will report that the light is less
bright in the a%ected eye.
zzThe patient is asked to assign a relevant value from 1 to 5 to
the brightness of the light in the diseased eye, in comparison
to the normal eye.!

Photostress test
zzPrinciples. Photostress testing is a gross test of dark adap-
tation in which the visual pigments are bleached by light.
This causes a temporary state of retinal insensitivity perceived
by the patient as a scotoma. The recovery of vision is depen-
Fig. 1.11 Stylized Amsler recording sheet showing wavy lines dent on the ability of the photoreceptors to re-synthesize visual
indicating metamorphopsia, and a dense scotoma.
pigment. The test may be useful in detecting maculopathy when

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Examination Techniques 7

A B

Fig. 1.12 Photostress test. (A) The patient looks at a light held about 3 cm away from the eye,
for about 10 seconds; (B) the photostress recovery time is the time taken to read any three
letters of the pre-test acuity line and is normally between 15 and 30 seconds.

ophthalmoscopy is equivocal, as in mild cystoid macular oedema retinal dystrophies prior to the impairment of other visual param-
or central serous retinopathy. It may also di%erentiate visual loss eters. Colour vision depends on three populations of retinal cones,
caused by macular disease from that caused by an optic nerve lesion. each with a specific peak sensitivity: blue (tritan) at 414–424 nm,
zzTechniques green (deuteran) at 522–539 nm and red (protan) at 549–570 nm.
{z The best-corrected distance VA is determined. Normal colour perception requires all these primary colours to
{z The patient fixates on the light of a pen torch or an indirect match those within the spectrum. Any given cone pigment may
ophthalmoscope held about 3 cm away for about 10 sec- be deficient (e.g. protanomaly – red weakness) or entirely absent
onds (Fig. 1.12A). (e.g. protanopia – red blindness). Trichromats possess all three
{z The photostress recovery time (PSRT) is the time taken to types of cones (although not necessarily functioning perfectly),
read any three letters of the pre-test acuity line and is nor- while absence of one or two types of cones renders an individual
mally between 15 and 30 seconds (Fig. 1.12B). a dichromat or monochromat, respectively. Most individuals with
{z The test is performed on the other, presumably normal, eye congenital colour defects are anomalous trichromats and use
and the results are compared. abnormal proportions of the three primary colours to match those
{z The PSRT is prolonged relative to the normal eye in macu- in the light spectrum. Those with red–green deficiency caused
lar disease (sometimes 50 seconds or more) but not in an by abnormality of red-sensitive cones are protanomalous (2%
optic neuropathy.! of men), those with abnormality of green-sensitive cones (6% of
men) are deuteranomalous and those with blue–green deficiency
caused by abnormality of blue-sensitive cones are tritanomalous.
Colour vision testing Acquired macular disease tends to produce blue–yellow defects,
and optic nerve lesions, red–green defects.
Introduction
Assessment of colour vision is useful in the evaluation of optic TIP Children who are found to have a congenital colour vision
nerve disease and in determining the presence of a congenitally deficiency will find it difficult or impossible to engage in certain
anomalous colour defect. Congenital colour vision deficiency occupations and may benefit from early career counselling.
!
(CVD) is a common X-linked recessive condition that a%ects
about 8% of Northern European men and 0.5% of women. The
prevalence in Black Africans is about 4% for men and 1.5% for Colour vision tests
women. CVD is the decreased ability to see colour or di%erences in zzThe Ishihara test is designed to screen for congenital protan
colour along a red–green axis. It can impair tasks such as choosing and deuteran defects. It is simple, widely available and fre-
clothing and seeing traffic lights. Dyschromatopsia may develop in quently used to screen for a red–green colour vision deficit.

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8 Psychophysical Tests

A B

Fig. 1.13 Colour vision tests. (A) Ishihara; (B) City University; (C) Hardy–Rand–Rittler; (D)
Farnsworth–Munsell 100-hue test.

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Examination Techniques 9

The test can be used to assess optic nerve function. It consists

of a test plate followed by 16 plates, each with a matrix of dots
arranged to show a central shape or number that the subject is
asked to identify (Fig. 1.13A). A colour-deficient person will
be able to identify only some of the figures. Inability to identify
the test plate (provided VA is sufficient) indicates non-organic
visual loss.
zzThe City University test consists of 10 plates, each containing
a central colour and four peripheral colours (Fig. 1.13B) from
which the subject is asked to choose the closest match.
zzThe Hardy–Rand–Rittler test is similar to the Ishihara, but
can detect all three congenital colour defects (Fig. 1.13C).
zzThe Farnsworth–Munsell 100-hue test is a sensitive but
longer test for both congenital and acquired colour defects.
Despite the name, it consists of 85 caps of di%erent hues in
four racks (Fig. 1.13D). The subject is asked to rearrange ran-
domized caps in order of colour progression, and the findings
are recorded on a circular chart. Each of the three forms of A
dichromatism is characterized by failure in a specific meridian
of the chart (Fig. 1.14).!

Plus lens test

A temporary hypermetropic shift may occur in some conditions
due to an elevation of the sensory retina – the classic example is
central serous chorioretinopathy (CSR). A +1.00-dioptre lens will
demonstrate the phenomenon.!

PERIMETRY
Definitions
zzThe visual field can be represented as a three-dimensional
structure akin to a hill of increasing sensitivity (Fig. 1.15A).
The outer aspect extends approximately 50° superiorly, 60° B
nasally, 70° inferiorly and 90° temporally. VA is sharpest
at the very top of the hill (i.e. the fovea) and then declines
progressively towards the periphery, the nasal slope being
steeper than the temporal. The ‘bottomless pit’ of the blind
spot is located temporally between 10° and 20°, slightly below
the horizontal.
zzAn isopter is a line connecting points of the same sensitivity,
and on a two-dimensional isopter plot encloses an area within
which a stimulus of a given strength is visible. When the field
is represented as a hill, isopters resemble the contour lines on
a map (Fig. 1.15B).
zzA scotoma is an area of reduced (‘relative’) or total (‘absolute’)
loss of vision surrounded by a seeing area.
zzLuminance is the intensity or ‘brightness’ of a light stimulus,
measured in apostilbs (asb). A higher intensity stimulus has a
higher asb value; this is related inversely to sensitivity.
zz A logarithmic rather than a linear scale is used for stimulus C
intensity and sensitivity, so that for each log unit intensity
changes by a factor of 10. With a log scale, greater significance
is given to the lower end of the intensity range. The normal eye
has a very large sensitivity range, and assessment of the lower Fig. 1.14 Farnsworth–Munsell test results of colour deficiencies.
end of the scale is of critical significance so that early damage (A) Protanopia; (B) deuteranopia; (C) tritanopia.

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10 Perimetry

Degrees from fixation

Fixation 10 20 Physiological blind spot

30
40 50
60
Sensitivity

70 Isopter plot
80
90
100

100 90 80 70 90 80 70 60 50 40 30 20 10 10 20 30 40 50 60 70 80 90
Nasal Temporal Nasal Temporal

90
Inferior 100
Degrees from fixation

A B

Fig. 1.15 (A) Hill of vision; (B) isopter plot.

can be detected. With a linear scale, the lower end would be light levels the peripheral retina becomes more sensitive in pro-
reduced to a very small portion of a graphical chart axis. The portion to the central retina. The hill of vision flattens, with a
visual system itself operates on close to a logarithmic scale, central crater rather than a peak at the fovea due to the high
so using this method more closely matches the physiological concentration of cones, which have low sensitivity in scotopic
situation. conditions. Some diseases give markedly di%erent field results
zzDecibels. Simple log units are not used in clinical perimetry, at di%erent background luminance levels, e.g. in retinitis pig-
but rather ‘decibels’ (dB), where 10 dB = 1 log unit. Decibels mentosa the field is usually much worse with low background
are not true units of luminance but a representation, and vary luminance. It should be noted that it takes about 5 minutes
between visual field machines. Perimetry usually concen- to adapt from darkness to bright sunlight and 20–30 minutes
trates on the eye’s sensitivity rather than the stimulus inten- from bright sunlight to darkness. The HFA (see below) uses a
sity. Therefore, the decibel reading goes up as retinal sensitivity photopic (preferentially cone) level of background luminance
increases, which obviously corresponds to reducing intensity at 31.5 asb.
of the perceived stimulus. This makes the assessment of visual zzStatic perimetry. A method of assessing fields, usually auto-
fields more intuitive, as a higher number corresponds with mated, in which the location of a stimulus remains fixed, with
higher retinal sensitivity. If the sensitivity of a test location is intensity increased until it is seen by the subject (threshold
20 dB (= 2 log units), a point with a sensitivity of 30 dB would is reached – Fig. 1.16A) or decreased until it is no longer
be the more sensitive. The blind spot has a sensitivity of 0 dB. detected.
If, on a given machine, seeing a stimulus of 1000 asb gives a zzKinetic (dynamic) perimetry is now much less commonly
value of 10 dB, a stimulus of 100 asb will give 20 dB. performed than static perimetry. A stimulus of constant inten-
zzDifferential light sensitivity represents the degree by which sity is moved from a non-seeing area to a seeing area (Fig.
the luminance of a target must exceed background luminance 1.16B) at a standardized speed until it is perceived, and the
in order to be perceived. The visual field is therefore a three- point of perception is recorded on a chart. Points from di%er-
dimensional representation of di%erential light sensitivity at ent meridians are joined to plot an isopter for that stimulus
di%erent points. intensity. Stimuli of di%erent intensities are used to produce a
zzThreshold at a given location in the visual field is the contour map of the visual field. Kinetic perimetry can be per-
brightness of a stimulus at which it can be detected by the formed by means of a manual (Goldmann) or an automated
subject. It is defined as ‘the luminance of a given fixed- perimeter if the latter is equipped with an appropriate software
location stimulus at which it is seen on 50% of the occa- program.
sions it is presented’. In practice we usually talk about an zzManual perimetry involves presentation of a stimulus by the
eye’s sensitivity at a given point in the field rather than the perimetrist, with manual recording of the response. It was for-
stimulus intensity. The threshold sensitivity is highest at merly the standard method of field testing but has now largely
the fovea and decreases progressively towards the periph- been superseded by automated methods. It is still used occa-
ery. After the age of 20 years the sensitivity decreases by sionally, particularly in cognitively limited patients unable
about 1 dB per 10 years. to interact adequately with an automated system, and for
zz Background luminance. The retinal sensitivity at any location dynamic testing of peripheral fields.
varies depending on background luminance. Rod photorecep- zzStandard automated perimetry (SAP) is the method used
tors are more sensitive in dim light than cones, and so owing to routinely in most clinical situations. Automated perimeters
their preponderance in the peripheral retina, at lower (scotopic) in common use include the Humphrey Field Analyser (HFA),

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Examination Techniques 11

Retinal sensitivity

Retinal sensitivity
A B

Fig. 1.16 Principles of perimetry. (A) Static – stimulus intensity (red arrow) at a single location is
increased until perceived – areas of lower sensitivity perceive only stimuli of greater intensity
(longer red arrows); (B) kinetic – stimulus of constant intensity is moved from a non-seeing
area until perceived.

the Octopus, Medmont, Henson and Dicon (Figs 1.17 and Fast algorithms
1.18). These predominantly utilize static testing, though
In recent years, strategies have been introduced with shorter test-
software is available on some machines to perform dynamic
ing times, providing efficiency benefits with little or no detriment
assessment.
to testing accuracy. The HFA o%ers the SITA (Swedish Interactive
Thresholding Algorithm), which uses a database of normal and
TIP Visual field results should always be used in conjunction glaucomatous fields to estimate threshold values and takes responses
with the clinical findings.
! during the test into account to arrive at adjusted estimates through-
out the test. Full-threshold values are obtained at the start of the test
for four points. SITA-Standard and SITA-Fast (Fig. 1.20) versions
Testing algorithms are available. The Octopus Perimeter uses G-TOP (Glaucoma Ten-
dency Oriented Perimetry), which estimates thresholds based on
Threshold information gathered from more detailed assessment of adjacent
Threshold perimetry is used for detailed assessment of the hill points. TOP presents each stimulus once at each location, instead of
of vision by plotting the threshold luminance value at vari- 4–6 times per location with a standard technique.!
ous locations in the visual field and comparing the results with
age-matched ‘normal’ values. A typical automated strategy is
to present a stimulus of higher than expected intensity. If seen,
Testing patterns
the intensity is decreased in steps (e.g. 4 dB) until it is no longer zzGlaucoma
seen (‘staircasing’). The stimulus is then increased again (e.g. 2 {z Most
important defects in glaucoma occur centrally –
dB steps) until seen once more (Fig. 1.19). If the stimulus is not within a 30° radius from the fixation point – so this is the
seen initially, its intensity is increased in steps until seen. Essen- area most commonly tested.
tially, the threshold is crossed in one direction with large incre- {z 24-2 is a routinely used glaucoma-orientated pattern. ‘24’
ments, then crossed again to ‘fine-tune’ the result with smaller denotes the extent in degrees to which the field is tested
increments. Threshold testing is commonly used for monitoring on the temporal side (to 30° on the nasal side). The num-
glaucoma.! ber after the hyphen (2) describes the pattern of the points
tested. 30-2 is an alternative.
Suprathreshold {z 10-2 is used to assess a central area of radius 10°. Glauco-
Suprathreshold perimetry involves testing with stimuli of lumi- matous defects here may threaten central vision. The 10-2
nance above the expected normal threshold levels for an age- pattern facilitates more detailed monitoring of the extent
matched population to assess whether these are detected. In other of damage, especially in advanced glaucoma where there
words, testing to check that a subject can see stimuli that would be is ‘split’ fixation.
seen by a normal person of the same age. It enables testing to be zzPeripheral field. Patterns that include central and peripheral
carried out rapidly to indicate whether function is grossly normal points (e.g. FF-120) are typically limited to the assessment of
or not and is usually reserved for screening.! neurological defects.

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12 Perimetry

Fig. 1.17 Humphrey perimeter and printout.

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Examination Techniques 13

Fig. 1.18 Octopus perimeter and printout.

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14 Perimetry

threshold has already been determined. If the patient fails to

respond, a false negative is recorded. A high false-negative
score indicates inattention, tiredness or malingering, but is
occasionally an indication of disease severity rather than
unreliability. The grey scale printout in individuals with
4 dB
high false-negative responses tends to have a clover leaf
Full threshold (4-2 dB) shape (Fig. 1.22).
Seeing double crossing staircase

Non-seeing
4 dB
2 dB TIP If the reliability indices are poor, be careful when
2 dB
interpreting the results of a visual field test.
!

Sensitivity values
zzA numerical display (see Fig. 1.20B) gives the measured or esti-
mated (depending on strategy) threshold in dB at each point. In
a full-threshold strategy, where the threshold is rechecked either
Fig. 1.19 Determination of threshold. as routine or because of an unexpected (>5 dB) result, the second
result is shown in brackets next to the first.
zzBinocular field testing (e.g. Esterman strategy) is used to zzA grey scale represents the numerical display in graphical form
assess statutory driving entitlement in many jurisdictions.! (see Fig. 1.20C) and is the simplest display modality to inter-
pret: decreasing sensitivity is represented by darker tones –
the physiological blind spot is a darker area in the temporal
Analysis field typically just below the horizontal axis. Each change in
SAP provides the clinician with an array of clinically relevant infor- grey scale tone is equivalent to a 5 dB change in sensitivity at
mation via monitor display or printout. The patient’s name and age that location.
are confirmed and a check made that any appropriate refractive zzTotal deviation (see Fig. 1.20D) shows the di%erence between
error compensation was used. General information should be a test-derived threshold at a given point and the normal sen-
reviewed, such as the type of algorithm performed, the time taken sitivity at that point for the general population, correcting for
for the test and the order in which the eyes were tested. age. Negative values indicate lower than normal sensitivity,
positive values higher than normal.
Reliability indices zzPattern deviation (see Fig. 1.20E) is derived from total devia-
Reliability indices (see Fig. 1.20A) reflect the extent to which the tion values adjusted for any generalized decrease in sensitivity
patient’s results are reliable. If significantly unreliable, further in the overall field (e.g. lens opacity), and demonstrates local-
evaluation of the visual field printout is pointless. With SITA ized defects.
strategies, false negatives or false positives over about 15% should zzProbability value plots of the total and pattern deviation (see
probably be regarded as significant and with full-threshold strate- Fig. 1.20G and H) are a representation of the percentage (<5%
gies, fixation losses over 20% and false positives or negatives over to <0.5%) of the normal population in whom the measured
33%. In patients who consistently fail to achieve good reliability defect at each point would be expected. Darker symbols repre-
it may be useful to switch to a suprathreshold strategy or kinetic sent a greater likelihood that a defect is significant.!
perimetry.
zzFixation losses indicate steadiness of gaze during the test. Summary values
Methods of assessment include presentation of stimuli to the Summary values (‘global indices’ on the HFA – see Fig. 1.20F)
blind spot to ensure no response is recorded, and the use of a represent distilled statistical information, which considers age-
‘gaze monitor’. matched normal data. These values are principally used to moni-
zzFalse positives are usually assessed by decoupling a stimulus tor progression of glaucomatous damage rather than for initial
from its accompanying sound. If the sound alone is presented diagnosis.
and the patient still responds, a false positive is recorded. With zzVisual field index (VFI) in the HFA is a measure of the
a high false-positive score the grey scale printout appears patient’s overall visual field function expressed as a percentage,
abnormally pale (Fig. 1.21). In SITA testing, false positives are the normal age-adjusted value being 100%.
estimated based on the response time. zzMean deviation (MD) on the HFA (mean defect on the Octo-
zzFalse negatives are registered by presenting a stimu- pus) gives an indication of the overall sensitivity of the field. It
lus much brighter than threshold at a location where the is derived from averaging the total deviation values.

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Examination Techniques 15

Single Field Analysis Eye: Right

Name: DOB:
ID:
Central 24-2 Threshold Test

Fixation Monitor: Gaze/Blind Spot Stimulus: III, White Pupil Diameter: 4.7 mm Date: 16-08-2013
Fixation Target: Central Background: 31.5 ASB Visual Acuity: Time: 2:12 PM
Fixation Losses: 0/10 Strategy: SITA-Fast RX: +3.25 DS DC X Age: 68
False POS Errors: 0% A
False NEG Errors: 0%
Test Duration: 02:42

Fovea: OFF
26 23 28 28

30 30 30 29 29 29

29 29 31 31 29 30 31 31

29 30 32 33 33 32 29 26 33
30
29 31 33 34 33 34 33 <0 31

30 30 33 33 33 31 32 28

30 30 30 32 32 30
B C
31 30 29 31

0 -4 1 2 -2 -6 -1 0
2 1 1 1 1 1 0 -1 -1 -2 -1 -1
1 -1 0 0 -1 0 2 3 -1 -3 -2 -2 -3 -2 -1 1
GHT
2 1 1 1 1 1 -2 3 0 -1 -1 -1 -1 -2 -4 1
Within Normal Limits
2 1 2 2 1 2 2 1 0 -1 0 0 -1 0 0 -1
1 0 2 1 2 0 1 -2 -1 -2 0 -1 0 -2 -1 -4
VFI 100%
0 -1 -1 1 1 0 -2 -3 -3 -1 -1 -2
3 1 -1 1 1 -1 -3 -1 MD +0.75 dB
D E F PSD 1.25 dB

Total Deviation Pattern Deviation

< 5%
G < 2% H
< 1%
< 0.5%

© 2010 Carl Zeiss Meditec

HFA II 750-41202-5.1.2/5.1.2
Fig. 1.20 Humphrey perimeter – SITA-Fast printout (see text).

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16 Perimetry

SINGLE FIELD ANALYSIS EYE: RIGHT

NAME: ID: DOB:
CENTRAL 24-2 THRESHOLD TEST

FIXATION MONITOR: GAZE/BLINDSPOT STIMULUS: III, WHITE PUPIL DIAMETER: DATE: 14-02-2002
FIXATION TARGET: CENTRAL BACKGROUND: 31.5 ASB VISUAL ACUITY: TIME: 5:03 PM
FIXATION LOSSES: 9/14 STRATEGY: SITA-STANDARD RX: DS DC X AGE: 77
FALSE POS ERRORS: 66%
FALSE NEG ERRORS: 33%
TEST DURATION: 08:30

FOVEA: OFF
32 25 30 29

27 30 28 29 27 26

33 33 32 32 30 29 30 29

36 322 33 34 32 31 31 39 49
30 30
35 29 33 32 33 34 32 32 32

28 34 36 35 32 32 32 31

32 34 34 33 32 39

32 34 29 33

Fig. 1.21 High false-positive score (arrow) with an abnormally pale grey scale display.

SINGLE FIELD ANALYSIS EYE: RIGHT

NAME: ID: DOB:
CENTRAL 24-2 THRESHOLD TEST

FIXATION MONITOR: GAZE/BLINDSPOT STIMULUS: III, WHITE PUPIL DIAMETER: DATE:

FIXATION TARGET: CENTRAL BACKGROUND: 31.5 ASB VISUAL ACUITY: TIME: 3:33 PM
FIXATION LOSSES: 0/17 STRATEGY: SITA-STANDARD RX: +0.50 DS +1.00 DC " 30 AGE: 85
FALSE POS ERRORS: 3%
FALSE NEG ERRORS: 53%
TEST DURATION: 08:52

FOVEA: OFF
!0 !0 !0 !0

13 !0 7 9 17 !0

!0 20 23 17 11 25 12 4

!0 !0 13 18 28 24 22 13 6
30 30
0 8 15 26 28 28 22 !0 24

2 27 26 15 19 28 26 21

18 26 21 16 18 24

10 18 17 15

Fig. 1.22 High false-negative score (arrow) with a clover leaf-shaped grey scale display.

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Examination Techniques 17

zzPattern standard deviation (PSD) is a measure of focal loss

or variability within the field and considers any generalized
depression in the hill of vision. An increased PSD is there-
fore a more specific indicator of glaucomatous damage than
MD.
zzLoss variance (LV) is a summary measure on the Octopus
Perimeter similar to PSD.
zzProbability values. Abnormal summary values are followed
by a probability value, representing the percentage likelihood
that an abnormal value of this level will occur in a normal
subject. The lower the P value, the more likely the result is
abnormal.
zzThe glaucoma hemifield test (GHT) compares corresponding
areas in the superior and inferior hemifields and relates only
to glaucoma.!

Computed analysis of serial fields

Computed analysis of serial visual fields for progression is in wide-
spread use. A disadvantage is the requirement for several reliable
fields to be carried out before analysis is e%ective. The quality of
available software has been improving steadily, with integrated
programs such as GPA (Guided Progression Analysis) on the HFA
and several trend analysis options on the Octopus.!

High-sensitivity field modalities

SAP tends to detect field damage only after substantial ganglion
cell loss is established. Attempts at detecting change at an earlier
stage include the adoption of stimuli intended to target specific
ganglion cell types.
zzShort-wave automated perimetry (SWAP) uses a blue stimu-
lus on a yellow background. Sensitivity to blue light (mediated
by blue cone photoreceptors) is adversely a%ected relatively
early in glaucoma. SWAP is more sensitive to early glaucoma-
tous defects but has not been widely adopted because cataract
decreases sensitivity to blue light (the brunescent lens acts as a
yellow filter) and patients frequently dislike the lengthy test. It
is available on newer HFA models.
zzFrequency-doubling test (FDT). Large diameter axon (mag-
nocellular) ganglion cells appear to be preferentially lost in
early glaucoma. The frequency-doubling illusion is produced
when a low spatial frequency sinusoidal grating undergoes
high temporal frequency counter phase flicker (>15 Hz). The
rapid alternation in which the light bars become dark and vice
versa produces the illusion of the grating having doubled its
frequency; magnocellular ganglion cells are believed to medi-
ate the pathways used. Screening (Fig. 1.23) and extended test-
ing (Humphrey Matrix) perimeter versions are available, the
latter being suitable for detailed assessment and monitoring of
glaucoma.! Fig. 1.23 Screening frequency-doubling perimeter with display.

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18 Perimetry

zzInadequate retinal adaptation may lead to error if perimetry

Sources of error is performed soon after ophthalmoscopy.
zzInexperienced or unskilled technician. Though less impor-
tant with SAP than manual perimetry, correctly setting up the
test, explaining the procedure to and reassuring the patient TIP Perimetry is a subjective examination and is not always
and monitoring performance are fundamental to obtaining an reliable.
!
accurate field.
zzIncorrect patient details. The patient’s date of birth must be
entered correctly to facilitate appropriate normative database
matching.
Microperimetry
zzPoor patient performance. zz Microperimetry is a visual field test that measures retinal sensitiv-
zzUncorrected refractive error can cause a significant decrease ity and fixation behaviour in patients with macular disease and
in central sensitivity. If a patient with hypermetropia who usu- focal glaucoma involving the central visual field. It allows an exact
ally wears contact lenses is tested wearing spectacles, this will correlation between pathology involving the macula and the cor-
have the e%ect of magnifying and enlarging any scotomas as responding functional abnormality. Microperimetry is more sen-
compared with contact lenses. Most perimetry is performed sitive than SAP in detecting subtle abnormality of visual function.
with a stimulus at approximately reading distance, so a near zz The MAIA perimeter is a table-top instrument (Fig. 1.25). The
correction should be used for individuals who are presbyopic. test is usually undertaken after 20 minutes of adaptation in
zzSpectacle rim artefact. Spectacles can cause rim scotomas if mesopic conditions. Fundus tracking occurs utilizing a line-
small aperture lenses are used or if incorrectly dispensed (Fig. scanning laser ophthalmoscope (SLO), with super-luminescent
1.24A). Narrow-aperture trial frame lenses are unsuitable for diode illumination using a central wavelength of 850 nm.
perimetry. zzGoldmann size 3 stimuli are projected onto the central 9° of
zzMiosis decreases sensitivity in the peripheral field and the fundus. A 4-2 staircase thresholding technique is used, with
increases variability in the central field in both normal and each stimulus applied for 200 milliseconds against a white back-
glaucomatous eyes. Pupils less than 3 mm in diameter should ground. Using this instrument normal retinal sensitivity is 18 dB.
therefore be dilated prior to perimetry; a consistent mydriatic zzResults are printed, together with reliability indices (fixation
should be used for serial tests. loss), probabilities and retinal sensitivity values, which are
zzMedia opacities (usually cataract) can have a profound e%ect, colour-coded. Microperimetry is particularly useful in assess-
exaggerated by miosis. ing the e%ect of existing and future therapeutic interventions
zzPtosis, even if mild, can suppress the superior visual field (Fig. on the macula (Fig. 1.26). It can also be helpful in patients with
1.24B). Similar e%ects result from dermatochalasis, prominent early glaucoma, particularly when there is subtle change close
eyelashes and deeply set eyes. to fixation.!

30 30

A B

Fig. 1.24 Artefact. (A) Grey scale display of spectacle rim artefact; (B) grey scale display of
upper lid artefact.

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Examination Techniques 19

Fig. 1.25 MAIA perimetry and printout.

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B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
20 Slit Lamp Biomicroscopy of the Anterior Segment

Fig. 1.26 Abnormal microperimetry in a patient with geographic atrophy of the macula.

SLIT LAMP BIOMICROSCOPY OF THE Direct illumination

ANTERIOR SEGMENT Direct illumination with a di%use light is used to detect gross
The purpose of slit lamp examination of the cornea and anterior abnormalities:
segment is to determine the position, depth and size of any abnor- zzA narrow obliquely directed slit beam is used to visualize a
mality (Fig. 1.27). cross-section of the cornea.

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
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Examination Techniques 21

Direct illumination
Portable slit lamp
A hand-held slit lamp allows high-quality examination of the ante-
rior segment in individuals who cannot be examined at a regular
slit lamp and is particularly useful when performing eye examina-
tions out of a hospital setting (Fig. 1.28).!

FUNDUS EXAMINATION
Scleral scatter
Direct ophthalmoscopy
zzA hand-held slit lamp direct examination of the structures
of the fundus using an ophthalmoscope can reveal disease of
the eye itself or may reveal an abnormality indicative of dis-
ease elsewhere in the body (for example: diabetes, systematic
hypertension, raised intracranial pressure). The major advan-
Retroillumination tage of direct ophthalmoscopy is that it can be used at the bed-
side. The image obtained is significantly magnified (15 times
normal) (Fig. 1.29).
zzThe direct ophthalmoscope can be used to examine the ‘red’
reflex using retroillumination. By placing a +15 D lens in posi-
tion and looking at the fundus at a distance of 15–20 cm, lens
and vitreous opacities can be detected (Fig. 1.30).
zzThe light beam can be used to (a) illuminate the cornea, allow-
Fig. 1.27 Technique of slit lamp biomicroscopy of the anterior ing detection of a foreign body, (b) check the pupil for irregular-
segment.
ity and (c) determine the light reflexes. Some ophthalmoscopes
incorporate a cobalt blue filter, which allows corneal abrasions
zzFurther narrowing of the beam to a very thin optical section and ulcers to be seen after the insertion of fluorescein.
moved across the cornea can determine the depth of a lesion.
zz The height of the coaxial beam can be adjusted to measure the hor- TIP A direct ophthalmoscope can be used at the bedside
izontal and vertical size of a lesion or associated epithelial defect. and provides a magnified view of the fundus, but there is no
zz The use of a red-free filter makes red objects appear black, thereby stereopsis and the field of view is small.
increasing contrast when observing vascular structures. A cobalt
blue filter is normally used in conjunction with fluorescein.!
Technique
zzIt is preferable to dilate the pupil if there is no contraindica-
Scleral scatter tion. However, a good view can be obtained through an undi-
Scleral scatter involves decentering the slit beam laterally so that lated pupil by darkening the examination room and by asking
the light is incident on the limbus with the microscope focused the patient to look into the distance.
centrally. Light is transmitted within the cornea by total inter- zzWhen examining the right eye, the clinician should stand at
nal reflection. A corneal stromal lesion will become illuminated the patient’s right side, holding the ophthalmoscope in the
because of forward light scatter. This technique is especially useful right hand. When examining the left eye, the clinician should
to detect subtle stromal haze, or cellular or lipid infiltration.! stand at the left side, holding the ophthalmoscope in the left
hand and using the left eye (Fig. 1.31).
zzThe ‘O’ on the illuminated lens dial of the ophthalmoscope
Retroillumination should be selected.
Retroillumination uses reflected light from the iris or fundus after zzTo overcome corneal reflection, the small aperture can be used
pupil dilatation to illuminate the cornea. This allows the detection and the light beam directed towards the edge of the pupil, rather
of fine epithelial and endothelial changes, such as epithelial cysts, than through the centre. The linear polarizing filter may be helpful.
keratic precipitates and small blood vessels.! zzThe examiner’s free hand should rest on the patient’s forehead.
zzThe patient is slowly approached at a slight angle, from the
temporal side.
Specular reflection zzThe light beam is directed at the pupil and the optic disc should
Specular reflection shows abnormalities of the endothelium such come into view at a distance of about 3.5 cm from the eye. If the
as reduced cell density and guttata. Pseudoguttata probably rep- disc is not in sharp focus, the ophthalmoscope lenses should
resent reversible endothelial cell oedema and inflammatory cells be rotated into the aperture using the index finger until the
beneath the endothelial layer.! disc becomes clearly visible.

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
22 Fundus Examination

A B

Fig. 1.28 Portable slit lamp. (A) Technique; (B) Keeler PSL Classic instrument.

Fig. 1.29 Magnified image obtained from direct ophthalmoscope.

Fig. 1.30 Red reflex showing anterior capsular thickening. Fig. 1.31 Technique of direct ophthalmoscopy (see text).

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B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 1
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Examination Techniques 23

zzIn hyperopic individuals use a plus lens (green) and in myopic

individuals a minus lens (red).
zzExamine the disc for clarity of outline, colour, elevation and
condition of vessels. Check for spontaneous venous pulsation.
Follow the vessels as far as possible into the retinal periphery.
zzTo locate the macula, focus the light on the disc then move the
light about 2 disc diameters temporally. Alternatively, ask the
patient to look at the light.
zzThe retinal periphery can be examined by asking the patient to
look up/down and to the sides.!

Slit lamp biomicroscopy

A range of diagnostic contact and non-contact lenses are avail-
able for use with the slit lamp. Contact lenses should not be used A
if a penetrating injury is suspected or in the presence of corneal
trauma, hyphaema or corneal infection.
zzNon-contact lenses
{z 60 D. High-magnification lens optimized for viewing the
posterior pole from a working distance of 13 mm. When
estimating optic disc diameter use a correction factor of
×0.88–1.0 for the Volk lens and ×1.0 for the Nikon lens.
{z 90 D. Wide-field lens with lower magnification and shorter
(7 mm) working distance. Can be used with smaller pupils.
The correction factor is ×1.3 (Fig. 1.32).
{z 78 D. Intermediate properties; ideal for general-purpose
examination. The correction factor is ×1.1.
{z Miscellaneous. Numerous other lenses are available, o%er-
ing qualities such as a very wide field of view and extremely
small pupil capability.

Technique B
Indirect ophthalmoscopy utilizes a high-power convex lens which is Fig. 1.32 (A) Indirect slit lamp biomicroscopy; (B) fundus view.
designed to obtain a wide field of view of the fundus. The image is
vertically inverted and laterally reversed. The technique is as follows: smartphone, using a clip-on device (Fig. 1.33). This can then be
zzThe slit beam is adjusted to a width of about one-quarter of its sent electronically to a specialist centre for an opinion on the fur-
full round diameter. ther management.
zzThe illumination is set at an angle coaxial with the slit lamp zzThe Vula Mobile App, which has been fully evaluated in rural
viewing system. settings in Africa, is a successful example of this technology.!
zzThe magnification and light intensity are adjusted to the lowest
settings.
zzThe light beam should be centred to pass directly through the
Goldmann three-mirror examination
patient’s pupil. zzGoldmann three-mirror lens consists of four parts: the cen-
zzThe lens is held directly in front of the cornea just clearing the tral lens and three mirrors set at di%erent angles. Because the
lashes so that the light beam passes through its centre. curvature of the contact surface of the lens is steeper than that
zz The fundus is examined by moving the joystick and vertical adjust- of the cornea, a viscous coupling substance with the same
ment mechanism of the slit lamp whilst keeping the lens still. refractive index as the cornea is required to bridge the gap
zzMagnification is increased to show greater detail. between the cornea and the goniolens. It is important to be
zzTo view the peripheral retina the patient should be instructed familiar with each part of the lens, as follows (Fig. 1.34A):
to direct their gaze accordingly.! {z The central part provides a 30° upright view of the poste-
rior pole.
Using new technology for retinal {z The equatorial mirror (largest and oblong-shaped) enables
visualization from 30° to the equator.
imaging in rural settings {z The peripheral mirror (intermediate in size and square-
In areas of the world with few specialist eye-care practitioners, shaped) enables visualization between the equator and the
primary health-care workers can take retinal photographs with a ora serrata.

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
24 Fundus Examination

A B

Fig. 1.33 Vula Mobile clip-on device to allow smartphone images of the posterior pole
to be taken in a rural setting. (A) Technique of use; (B) inverted retinal image. (Courtesy of
W Mapham.)

A C

Fig. 1.34 (A) Goldmann three-mirror lens; (B) U-tear left of 12 o’clock and an area of lattice de-
generation right of 12 o’clock; (C) the same lesions seen with the three-mirror lens positioned
at 6 o’clock.

{z The gonioscopy mirror (smallest and dome-shaped) may zzTechnique

be used for visualizing the extreme retinal periphery and {z The pupils are dilated.
pars plana. {z The locking screw of the slit lamp is unlocked (Fig.
{z It is therefore apparent that the smaller the mirror, the 1.35A) to allow the illumination column to be tilted
more peripheral the view obtained. (Fig. 1.35B).
zzMirror positioning {z Anaesthetic drops are instilled.
{z The mirror should be positioned opposite the area of the {z Coupling fluid (high viscosity methylcellulose or equiva-
fundus to be examined. To examine the 12 o’clock position lent) is inserted into the cup of the contact lens; it should
the mirror should be positioned at 6 o’clock. be no more than half full.
{z When viewing the vertical meridian, the image is upside {z The patient is asked to look up. The inferior rim of the lens
down but not laterally reversed, in contrast to indirect is inserted into the lower fornix (Fig. 1.36A) and quickly
ophthalmoscopy. Lesions located to the left of 12 o’clock pressed against the cornea so that the coupling fluid is
in the retina will therefore also appear in the mirror on the retained (Fig. 1.36B).
left-hand side (Fig. 1.34 B and C). {z The illumination column should always be tilted except
{z When viewing the horizontal meridian, the image is later- when viewing the 12 o’clock position in the fundus (i.e.
ally reversed. with the mirror at 6 o’clock).

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
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Examination Techniques 25

A B

Fig. 1.35 Preparation of the slit lamp for fundus examination. (A) Unlocking the screw; (B) tilting the illumination column.

A B

Fig. 1.36 Insertion of contact lens. (A) Inserted into the lower fornix with the patient looking up; (B) in position for examination
of posterior pole.

{z When viewing horizontal meridians (i.e. 3 and 9 o’clock zzScleral indentation at the slit lamp can be accomplished using
positions in the fundus) the column should remain central. a three-mirror lens with a special attachment (Eisner funnel)
{z When viewing the vertical meridians (i.e. 6 and 12 o’clock or a purpose-made ora serrata contact lens that combines a
positions) the column can be positioned left or right of centre. mirror angled similarly to a gonioscopy lens with an incorpo-
{z When viewing oblique meridians (i.e. 1.30 and 7.30 o’clock) rated attachment to facilitate scleral depression (e.g. Goldman
the column is kept right of centre, and vice versa when view- One Mirror Glass 904 lens).
ing the 10.30 and 4.30 o’clock positions (Fig. 1.37). zzMiscellaneous contact lenses are divided principally into
{z When viewing di%erent positions of the peripheral retina, those conferring high magnification for an optimal posterior
the axis of the beam is rotated so that it is always at right- pole view, and those o%ering a wide field of view, allowing
angles to the mirror. visualization extending to the ora serrata under optimal condi-
{z To visualize the entire fundus the lens is rotated for 360° using tions. Small pupil capability is available, and a flange is o%ered
first the equatorial mirror and then the peripheral mirrors. on many lenses with the aim of improving stability of retention
{z To obtain a more peripheral view of the retina the lens and of lens position on the eye.!
is tilted to the opposite side asking the patient to move
the eyes to the same side. For example, to obtain a more Head-mounted binocular indirect
peripheral view of 12 o’clock (with mirrors at 6 o’clock) tilt
the lens down and ask the patient to look up.
ophthalmoscopy
{z The vitreous cavity is examined with the central lens using The term binocular indirect ophthalmoscopy (BIO) is by conven-
both a horizontal and a vertical slit beam. tion used to refer to the head-mounted technique, though strictly
{z The posterior pole is examined. it also applies to slit lamp indirect ophthalmoscopy. BIO allows

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
26 Fundus Examination

retinal visualization through a greater degree of media opacity {z 40 D (magnification ×1.5; field 65°) is used mainly to
than slit lamp biomicroscopy, and readily facilitates scleral inden- examine small children. A broad fundus scan can be
tation. Light is transmitted from the headset to the fundus through acquired rapidly and it can also be used at the slit lamp to
a condensing lens held at the focal point of the eye, providing an provide very high magnification.
inverted and laterally reversed image that is observed through a {z Panretinal 2.2 combines magnification similar to the 20 D
stereoscopic viewing system (Fig. 1.38A). lens with a field of view similar to that of the 28 D, and can
zzLenses of various powers and diameters are available for BIO be used with small pupils.
(Fig. 1.38B). A lens of lower power confers increased magni- {z Ultra-high magnification lenses for macula and optic disc
fication, but a smaller field of view. The magnification can be examination (e.g. Macula Plus 5.5) are available.
calculated by dividing the dioptric power of the examining zzTechnique
lens into 60. (For example, a 20 D lens would have a magnifica- {z The patient should be supine on a bed or reclining chair
tion of 3.) Yellow filters may improve patient comfort. rather than sitting upright (Fig. 1.39A).
{z 20 D (magnifies ×3; field about 45°) is the most commonly {z The pupils should be dilated. Reducing the ambient illumi-
used for general examination of the fundus. nation is often helpful as this improves contrast and allows
{z 28 D (magnification with the head-mounted set of ×2.27; a lower incident light intensity to be used.
field of 53°) has a shorter working distance and is useful {z The eyepieces are set at the correct interpupillary distance
when examining patients with small pupils. and the beam aligned so that it is located in the centre of
the viewing frame.
{z The patient is instructed to keep both eyes open at all
times. If necessary, the patient’s eyelids are gently separated
with the fingers.
{z The lens is taken into one hand with the flat surface facing
the patient.
{z The peripheral fundus should be examined first in order to
allow the patient to adapt to the light. The patient is asked
to move the eyes into optimal positions for examination,
e.g. looking away from the examiner to facilitate examina-
tion of the retinal periphery.
{z For the examination of small children (e.g. retinopathy
of prematurity – see also Ch. 13) a speculum may be uti-
lized to keep the eyelids apart, with an implement such as
a squint hook employed to direct the position of the eye.
zzScleral indentation
{z The main function of scleral indentation (depression) is to
improve visualization of the retina anterior to the equator;
Fig. 1.37 The illumination column is tilted and positioned it also permits kinetic evaluation (Fig. 1.40).
right of centre to view the oblique meridian at 1.30 and 7.30 {z Indentation should be attempted only after the basic tech-
o’clock. nique of BIO has been mastered. It requires practised

B
Fig. 1.38 (A) Principles of indirect ophthalmoscopy; (B) condensing lenses.

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B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 1
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Examination Techniques 27

coordination between the relative position of the indenter {z For viewing the 3 and 9 o’clock positions, indentation
and the viewing apparatus, as well as care to prevent directly on the sclera is sometimes necessary, facilitated by
patient discomfort. For example, to view the ora serrata at topical anaesthesia. Indentation can also be performed at
12 o’clock, the patient is asked to look down and the scleral the slit lamp using some fundus contact lenses.!
indenter (a cotton-tipped applicator is preferred by some
practitioners) is applied to the outside of the upper eyelid
at the margin of the tarsal plate.
Fundus drawing
{z With the indenter in place, the patient is asked to look up. When available, wide-field photographic imaging can be an excel-
At the same time the indenter is advanced into the ante- lent aid in recording the features of a retinal detachment (Fig.
rior orbit parallel with the globe, and the examiner’s eyes 1.41), but documentation generally takes the form of a manually
are aligned with the condensing lens and indenter (see Fig. drawn illustration that optimally is colour-coded (Fig. 1.42). If a
1.39B). retinal detachment is found, the boundaries are drawn by starting
{z Gentle pressure is exerted so that a mound is created. After at the optic nerve and then extending to the periphery. Detached
adequate viewing, the indenter is gently moved to an adja- retina is shaded in blue and flat retina in red. The course of reti-
cent part of the fundus. nal vessels (usually veins) is indicated with blue. Retinal breaks are
{z The indenter should be kept tangential to the globe at all drawn in red with blue outlines; the flap of a retinal tear is also
times, as perpendicular indentation will cause pain and drawn in blue. Thin retina may be represented by red hatching out-
even risk perforation if the sclera is very thin. lined in blue and lattice degeneration by blue hatching outlined

A B

Fig. 1.39 (A) Position of patient during indirect ophthalmoscopy; (B) scleral indentation.

A B

Fig. 1.40 Appearance of retinal breaks in detached retina. (A) Without scleral indentation; (B)
with indentation.

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
28 Tonometry

a
tin
re
ed

br
ch

ea
ta

ks
de

vitre
ous

ning
opa

thin
city
ex
ud
at
e
e
tic
retin
al pig lat
men
t

Fig. 1.41 Wide-field fundus photograph showing a peripheral Fig. 1.42 Colour coding for retinal illustration.
retinal dialysis.

in blue. Retinal pigment is indicated in black, retinal exudates in Corneal rigidity and capillary attraction cancel each other out
yellow and vitreous opacities in green.! when the flattened area has a diameter of 3.06 mm, as in Goldmann
tonometry (Fig. 1.43A). The Goldmann tonometer is an accurate
variable-force tonometer consisting of a double prism (Fig. 1.43B).
TONOMETRY The tonometer prism should be disinfected between patients and
replaced regularly in accordance with the manufacturer’s instruc-
Goldmann tonometry tions. Two per cent Na-hypochlorite (dilute bleach) o%ers e%ective
Principles disinfection against adenovirus and herpes simplex virus. However,
Goldmann applanation tonometry is based on the Imbert–Fick disinfectants can cause the tonometer tip to swell and crack with
principle, which states that for an ideal, dry, thin-walled sphere, time, which can lead to disinfectant entering the tonometer tip
the pressure inside the sphere (P) equals the force necessary to resulting in a corneal abrasion when applied to the cornea. Seventy
flatten its surface (F) divided by the area of flattening (A) (i.e. P = per cent isopropyl alcohol wipes do not o%er protection against
F/A). The intraocular pressure (IOP) is proportional to the pres- viral infections. Disposable tonometer prisms and caps have been
sure applied to the globe (in practice, the cornea) and the thick- introduced to address concerns of infection from reusable prisms.!
ness of the wall of the globe (i.e. the thickness of the cornea, which
is variable). The human eye, however, is not an ideal sphere – the Technique
cornea is rigid and resists flattening. Capillary attraction of the tear zz Topical anaesthetic (commonly proxymetacaine 0.5%) and a
meniscus, however, tends to pull the tonometer towards the cornea. small amount of fluorescein are instilled into the conjunctival sac.

A B

Fig. 1.43 Goldmann tonometry. (A) Physical principles; (B) tonometer.

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Examination Techniques 29

zzThe patient is positioned at the slit lamp with his or her fore- zzPressure on the globe from the examiner’s fingers, eye-
head firmly against the headrest and instructed to look straight lid squeezing or restricted extraocular muscles (e.g. thyroid
ahead (often at the examiner’s opposite ear) and to breathe myopathy) may give an anomalously high reading.
normally. zzCentral corneal thickness (CCT). Calculations of IOP by
zzWith the cobalt blue filter in place and illumination of maxi- Goldmann applanation tonometry (GAT) assume that CCT
mal intensity directed obliquely (approximately 60°) at the is 520 µm, with minimal normal variation. If the cornea is
prism, the prism is centred in front of the apex of the cornea. thinner, an underestimation of IOP is likely to result, and if
zzThe dial is preset at 1 (i.e. 10 mmHg). thicker, an overestimation. Corneas tend to be thicker than
zzThe prism is advanced until it just touches the apex of the cor- average in individuals with ocular hypertension, and thinner
nea (Fig. 1.44A). in normal-tension glaucoma (NTG). Following refractive sur-
zzViewing is switched to the ocular of the slit lamp. gery procedures, the cornea is both thinner and structurally
zzA pattern of two green semi-circular mires will be seen, one altered, leading to an underestimation of the IOP. Some meth-
above and one below the horizontal midline, which represent ods of IOP measurement (e.g. DCT – see below) may reduce
the fluorescein-stained tear film touching the upper and lower the e%ect of structural confounding variables. Other corneal
outer halves of the prism. Mire thickness should be around mechanical factors may also be important but are less well
10% of the diameter of its total arc (Fig. 1.44B). Care should defined.
be taken to horizontally and vertically centre the mires so that zzCorneal oedema may result in artificial lowering of IOP,
two centralized semi-circles are observed. hypothesized to be due to a boggy softening; the associated
zzThe dial on the tonometer is rotated to vary the applied force. increased CCT seems to be more than o%set.
The inner margins of the semi-circles align when a circular zzAstigmatism, if significant, may give distorted mires as well as
area of diameter precisely 3.06 mm is flattened. leading to mechanically induced errors. If over 3 dioptres, the
zzThe reading on the dial, multiplied by 10, gives the IOP in average reading of two can be taken with the prism rotated 90°
mmHg. for the second, or optimally the prism is rotated so that the red
line on the tonometer housing is aligned with the prescription
TIP Inadvertent finger pressure applied to the globe when of the minus axis.
undertaking applanation tonometry or squeezing of the eyelids zzIncorrect calibration of the tonometer can result in a false
reading. Calibration should be checked before each clinical
can cause an anomalously high IOP reading.
!
session using the manufacturer’s calibration arm (Fig. 1.44C).
zzWide pulse pressure. It is normal for there to be a small oscil-
Sources of error lation of IOP in concert with the rhythm of ocular perfusion.
zzInappropriate fluorescein pattern. Excessive fluorescein will If this ‘pulse pressure’ is substantial, either the midpoint or the
result in the mires being too thick, with consequent overes- highest level observed may be taken.
timation of IOP. Insufficient will make the semi-circles too zzRepeated readings over a short period will often be associ-
thin, with consequent underestimation (see Fig. 1.44B, left and ated with a slight fall in IOP due to a massaging e%ect on the
centre). eye.

B C

Fig. 1.44 Applanation tonometry. (A) Contact between the tonometer prism and the cornea;
(B) fluorescein-stained semi-circular mires – the diagram at right showing the correct end-
point using mires of appropriate thickness; (C) tonometer calibration bar in position.

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
30 Tonometry

zzOther factors, including a tight collar and breath-holding, It is hand-held and can therefore be used in bed-bound or
both of which obstruct venous return, can raise IOP. anaesthetized patients.
zzDynamic contour tonometry (DCT) (e.g. PASCAL) uses a
TIP The Goldmann tonometer loses accuracy with constant solid-state sensor and a corneal contour-matching surface,
use and should be checked for calibration error on a regular with the aim of measuring IOP relatively independently of
corneal mechanical factors such as rigidity. It is mounted on
basis.
!
a slit lamp in similar fashion to the Goldmann tonometer and
IOP is shown on a digital display. Studies comparing DCT and
GAT IOP readings with manometric intracameral IOP seem to
Other forms of tonometry confirm DCT as providing a more physiological measurement.
zzPneumotonometry (Fig. 1.45A) is based on the principle of zzElectronic indentation/applanation tonometry (e.g. Tono-
applanation, but the central part of the cornea is flattened by a Pen AVIA – Fig. 1.45C) is a hand-held electronic contact
jet of air rather than a prism. The time required to sufficiently tonometer (a modified version of the older Mackay–Marg
flatten the cornea relates directly to the level of IOP. Contact is tonometer). The probe tip contains a transducer that measures
not made with the eye and topical anaesthesia is not required, applied force. Besides portability, its main advantage is the
so it is particularly useful for screening in the community. The facility to measure IOP reasonably accurately in eyes with dis-
sudden jet of air can startle the patient. Accuracy is improved torted or oedematous corneas and through a soft contact lens.
if an average of at least three readings is taken. zzRebound tonometry (e.g. iCare – Fig. 1.45D) involves a
zzPortable applanation tonometry (Perkins) uses a Goldmann 1.8 mm plastic ball attached to a wire. Deceleration of the
prism in conjunction with a portable light source (Fig. 1.45B). probe upon contact with the cornea is proportional to IOP.

A B

C D

Fig. 1.45 Portable tonometers. (A) Keeler Pulsair pneumotonometer; (B) Perkins applanation
tonometer; (C) Medtronic Tono-Pen; (D) iCare IC100.

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 1
CHAPTER
Examination Techniques 31

Anaesthesia is not required. The instrument can be used for Applanation Signal Pressure (air pulse)
self-monitoring and for screening in the community.

Pressure / Signal Amplitude

zzHome tonometry (e.g. iCare HOME2) is available for patients “In” Signal Peak dslope1 “Out” Signal Peak

who require regular monitoring of their IOP, especially when

uslope1
information is needed on diurnal IOP fluctuation. It is fairly
easy to use and provides accurate measurements. It has a posi- Applanation
tioning system for correct alignment of the instrument and a Pressure 1
Hysteresis Applanation
magnet within the applicator which prevents the probe from Pressure 2
falling out. w2
Baseline
zzIndentation (impression) tonometry (e.g. Schiotz) is a
seldom-used portable device that measures the extent of cor- 0 10 15 20 25
Time - msec
neal indentation by a plunger of known weight.
zzImplantable tonometers are under development and if a clini-
cally workable device is realized should facilitate accurate life-
long 24-hour IOP measurement.!

Ocular response analyser and corneal

hysteresis
The ocular response analyser (e.g. Reichert ORA) utilizes air-pu%
technology to record two applanation measurements: one while
the cornea is moving inward and one when the cornea returns to
its normal position. The average of these two IOP measurements
provides a Goldmann-correlated IOP measurement. The di%er-
ence between the IOP measurements is called corneal hysteresis
(Fig. 1.46). Corneal hysteresis is not a measure of the sti%ness of
the cornea, but rather a measure of how corneal tissue absorbs and
dissipates energy during deformation and return. Corneal hyster-
esis appears to provide a measurement of IOP that is less a%ected
by factors such as previous laser refractive surgery. It also o%ers
information on which patients with glaucoma are more likely to
progress (patients with a low hysteresis value are at a greater risk of Fig. 1.46 Corneal hysteresis as measured by the Ocular
Response Analyzer (Reichert). It is defined as the difference
glaucoma progression) and it may serve as a biomarker to aid glau- between the pressure at which the cornea bends inward
coma case detection. There is also a strong relationship between during air-jet applanation and the pressure at which it bends
corneal hysteresis and the magnitude of IOP reduction with topi- out again.
cal prostaglandin therapy.!
Optical principles
GONIOSCOPY The angle of the AC cannot be visualized directly through the
intact cornea because light from angle structures undergoes ‘total
Introduction internal reflection’ at the anterior surface of the precorneal tear
film (Fig. 1.47, top). When light travels from a medium of higher
Overview to one of lower refractive index (such as cornea to air) it will
zzGonioscopy is a method of evaluating the anterior chamber be reflected at the interface between the two unless the angle of
(AC) angle, and can be used therapeutically for procedures incidence is less than a certain ‘critical angle’ dependent on their
such as laser trabeculoplasty and goniotomy. refractive index di%erence (46° for the tear film–air interface). The
zzOther means of angle assessment, such as anterior segment phenomenon is utilized in optical fibre signal transmission, where
optical coherence tomography (OCT) and high-frequency it ensures that light is retained within the core of a cable. Because
ultrasound biomicroscopy (UBM), o%er advantages in some the refractive index of a goniolens is similar to that of the cornea,
aspects of angle analysis, but current clinical opinion suggests it eliminates total internal reflection by replacing the tear film–air
they should supplement rather than replace gonioscopic evalu- interface with a tear film–goniolens interface (Fig. 1.47, bottom).
ation (see Ch. 11). Light rays can then be viewed as they exit the contact lens, directly
or indirectly (see below).!

TIP Gonioscopy is an indispensable technique that allows Disinfection

the filtration angle to be examined in patients with ocular
Lenses must be cleaned between patients to remove any particulate
hypertension or glaucoma.
! matter and then sterilized. A suggested regimen is to soak the lens

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32 Gonioscopy

Fig. 1.48 Goldmann single-mirror goniolens.

substance of refractive index similar to the cornea is

required to bridge the gap between cornea and lens.

TIP Gonioscopy should be undertaken in a room where the

ambient illumination is low, as pupil size influences the grade of
angle closure.
n=1.50

zzTechnique
n=1.37 {z It is essential that the examination takes place in a room in
which the ambient illumination is low.
{z The size and intensity of the slit beam should be reduced to
the absolute minimum compatible with an adequate view.
Fig. 1.47 Optical principles of gonioscopy; n = refractive index; Do not direct the beam through the pupil.
i = angle of incidence. {z The patient is seated at the slit lamp and advised that the
lens will touch the eye but will not usually cause discom-
fort. The forehead must be kept against the headband and
in 2% Na-hypochlorite solution for at least 5 minutes followed by both eyes should remain open.
thorough rinsing in sterile saline, then air-drying.! {z A drop of local anaesthetic is instilled.
{z A drop or two of coupling fluid (e.g. hypromellose 0.3%) is
placed on the contact surface of the lens.
Indirect gonioscopy {z The patient is asked to look upwards and the lens is inserted
Indirect goniolenses use a mirror to reflect rays from the angle rapidly so as to avoid loss of the coupling fluid. The patient
such that they exit the goniolens at much less than the critical then looks straight ahead.
angle. They provide a mirror image of the opposite angle and can {z Indirect gonioscopy gives an inverted view of the portion
be used only in conjunction with a slit lamp. of the angle opposite the mirror.
{z Once the initial examination has been performed and the
Non-indentation gonioscopy findings noted, increasing the level of illumination may
zzGoniolenses help in defining the angle structures.
{z The classic Goldmann lens consists of three mirrors, one {z When the view of the angle is obscured by a convex iris,
of which is specifically for gonioscopy. Some goniolenses it is possible to see ‘over the hill’ by asking the patient to
have one (Fig. 1.48), two or four mirrors. look in the direction of the mirror. Only slight movement
{z Lenses of similar basic structure but with modifications is permissible, otherwise the structures will be distorted
include the Magna View, Ritch trabeculoplasty and the and a closed angle may appear open.
Khaw direct view. {z Excessive pressure with a non-indentation lens narrows
{z Because the curvature of the contact surface of the lens the angle appearance (in contrast to the e%ect of pressure
is steeper than that of the cornea, a viscous coupling during indentation gonioscopy – see below). Excessive

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Examination Techniques 33

A B

Fig. 1.49 (A) Zeiss goniolens; (B) slit lamp view with lens in place on the cornea.

pressure also causes folds in the cornea that compromise

the clarity of the view.
Direct gonioscopy
{z In some eyes, suction on the cornea from the lens may Direct goniolenses work by constructing the viewing surface of the
artificially open the angle. Awareness of the need to avoid lens in a domed or slanted configuration such that exiting light
retrograde, as well as anterograde, pressure on the lens will rays strike the contact lens/air interface at a steeper than critical
tend to prevent inadvertent distortion. angle so that they will pass through to the observer. This approach
is called ‘direct’ because light rays from the angle are viewed
TIP Indentation gonioscopy is a useful technique to directly, without reflection inside the lens. They do not require
determine the degree of peripheral anterior synechiae in a a slit lamp and are used with the patient in the supine position,
typically under general anaesthesia in the evaluation and surgical
patient with appositional angle closure.
!
treatment of infantile glaucoma.
zzDirect goniolenses include the Koeppe (Fig. 1.51A and B),
Indentation (dynamic, compression) Medical Workshop, Barkan and Swan–Jacob.
gonioscopy zzTechnique
zzGoniolenses include the Zeiss (Fig. 1.49), Posner and Sussman {z Gonioscopy is performed with the patient in the supine
(no handle), all of which are four-mirror gonioprisms. position (note that this may deepen the angle) in conjunc-
{z The contact surface of the lenses has a curvature flatter tion with an operating or hand-held microscope or mag-
than that of the cornea, negating the need for a coupling nifying loupes.
substance. {z The technique cannot be used with a desktop slit lamp so
{z The lenses do not stabilize the globe and are relatively clarity, illumination and variable magnification are not
unsuitable for laser trabeculoplasty. comparable with indirect lenses.!
{z The lenses are useful for indentation gonioscopy.
zzTechnique
{z The first stages are as set out above for non-indentation
Surgical gonioscopy
gonioscopy. zzTechnique
{z Indentation is performed by gently pressing the lens pos- {z The key to successful microinvasive glaucoma surgery is
teriorly against the cornea. This forces aqueous into the adequate visualization of the filtration angle.
angle, pushing the peripheral iris posteriorly. {z This is best achieved by rotating the operating microscope
{z If the angle is closed only by apposition between the iris downwards (arrow) (Fig. 1.51C) and by tilting the patient’s
and cornea it will be forced open, allowing visualization of head away from the surgeon by 30–35° (arrow), until the
the angle recess (Fig. 1.50). coaxial light is aligned along the iris plane. Surgical instru-
{z If the angle is closed by adhesions between the peripheral ments can then approach the angle parallel to the iris plane.
iris and cornea – peripheral anterior synechiae (PAS) – it {z A useful surgical landmark is the scleral spur, which sepa-
will remain closed. rates canal-based surgery (via the trabecular meshwork,
{z Dynamic gonioscopy can be invaluable in helping to define anteriorly), from suprachoroidal-based surgery (via the
the structures in angles that are difficult to assess, such as ciliary body band, posteriorly).
in distinguishing an extensive or double highly pigmented {z Pigment within the trabecular meshwork can serve as a
Schwalbe line from the pigmented meshwork.! visual guide to the Schlemm canal. The canal can also be

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34 Gonioscopy

A B

Fig. 1.50 Indentation gonioscopy in appositional angle closure. (A) Total angle closure prior to
indentation; (B) during indentation the structures of the angle become visible (arrow).

A B

C D

Fig. 1.51 Direct gonioscopy. (A) Optical principles of direct gonioscopy; (B) Koeppe lens; (C)
operative gonioscopy: position of microscope (inset) and rotated patient’s head when under-
taking microinvasive glaucoma surgery (MIGS). Arrows indicate the direction of rotation to
obtain an optimum view of the angle; (D) Volk Surgical Gonio Lens.

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Examination Techniques 35

A B

C D

Fig. 1.52 Normal angle structures. (A) Schematic cross-sectional representation with (B) show-
ing the corneal wedge; (C) goniophotograph – a broad Schwalbe line is indicated by the
white arrow, below which is the non-pigmented meshwork, the pigmented meshwork, the
scleral spur and the ciliary body (black arrow) – the ciliary body is relatively lightly pigmented;
(D) corneal wedge on gonioscopy (arrow showing Schwalbe line). (Courtesy of W Alward,
from Color Atlas of Gonioscopy, Wolfe 1994 – figs A and B; R Tailor – fig. D.)

located by applying gentle pressure with the edge of the syndrome). It may have a double-line configuration, when
goniolens (Fig. 1.51D) on the perilimbal episcleral vessels the posterior component may be mistaken for the pigmented
nasally, allowing reflux of blood into the canal. Trypan blue meshwork (see Ch. 11).
can be used to stain the trabecular meshwork when there is zzThe corneal wedge is useful in locating an inconspicuous
limited pigmentation.! Schwalbe line. Using a narrow slit beam, two distinct linear
corneal reflections can be identified, one on the inner and one
on the outer corneal surface. The outer reflection will arc round
Identification of angle structures across the corneoscleral interface – due to the sclera being
Accurate identification of angle structures (Fig. 1.52A–C) is not opaque – to meet the inner reflection at the apex of the corneal
always straightforward, even for the experienced clinician. wedge that coincides with the Schwalbe line (Fig. 1.52D).
zzSchwalbe line. This is the most anterior structure, appearing zzThe trabeculum extends from the Schwalbe line to the scleral
whitish to variably pigmented. Anatomically it demarcates the spur, with an average width of 600 µm. In younger people it
peripheral termination of Descemet membrane and the ante- has a ground-glass translucent appearance. The anterior non-
rior limit of the trabeculum. It may be barely discernible in functional part lies adjacent to the Schwalbe line and has a
younger patients. In contrast, there may be pigment depos- whitish colour. The posterior pigmented functional part lies
its on or anterior to the Schwalbe line – a Sampaolesi line – adjacent to the scleral spur and has a greyish-blue translucent
especially in heavily pigmented angles (e.g. pseudoexfoliation appearance in the young. Trabecular pigmentation is rare prior

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36 Central Corneal Thickness

to puberty, but in older eyes involves the posterior trabeculum

to a variable extent, most marked inferiorly. Patchy trabecular
pigmentation in a suspiciously narrow angle raises the possi-
bility of intermittent iris contact.
zzThe Schlemm canal may be identified in the angle, especially
if non-pigmented, as a slightly darker line deep to the poste-
rior trabeculum. Blood can sometimes be seen in the canal,
either physiologically (sometimes due to excessive pressure on
the episcleral veins with a goniolens), or in the presence of low
intraocular or raised episcleral venous pressure.
zzThe scleral spur is the most anterior projection of the sclera
and the site of attachment of the longitudinal muscle of the
ciliary body. Gonioscopically it is situated immediately poste-
rior to the trabeculum and appears as a narrow whitish band
that yellows with age.
zzThe ciliary body stands out just behind the scleral spur as a
pink, dull brown or slate grey band. Its width depends on the Fig. 1.53 Gonioscopy showing iris processes (arrow).
position of iris insertion and it tends to be narrower in hyper-
metropic eyes and wider in myopic eyes. The angle recess rep- {z Blunt ocular trauma.
resents the posterior dipping of the iris as it inserts into the {z Anterior uveitis.
ciliary body. It may not be visible in some eyes due to a physi- {z Following acute angle-closure glaucoma.
ological anterior iris insertion, though fixed pathological angle {z Following YAG laser iridotomy.
narrowing due to PAS – adhesions between the iris and angle {z Iris or angle melanoma or naevus.
structures – should be excluded. {z Iris pigment epithelial cysts.
zzIris processes are small, usually tenuous extensions of the {z Naevus of Ota.
anterior surface of the iris that insert at the level of the scleral zzTrauma
spur and cover the ciliary body to a varying extent (Fig. 1.53). {z Angle recession.
They are present in about one-third of normal eyes and are {z Trabecular dialysis.
most prominent during childhood and in brown eyes. The {z Cyclodialysis.
processes should not be confused with PAS, which typically {z Foreign bodies.
extend more anteriorly and are more substantial. zzBlood in the Schlemm canal
zzBlood vessels. Radial vessels at the base of the angle recess are {z Physiological variant.
often seen in normal eyes. Pathological blood vessels run ran- {z Carotid–cavernous fistula and dural shunt.
domly in various directions. As a general principle, any blood {z Sturge–Weber syndrome.
vessel that crosses the scleral spur onto the trabecular mesh- {z Obstruction of the superior vena cava.!
work is abnormal. Larger circumferential vessels may also be
seen.!
CENTRAL CORNEAL THICKNESS
Central corneal thickness (CCT) can be measured using pachym-
Pathological findings etry or by Orbscan (see Fig. 11.5). The normal distribution is
zzPeripheral anterior synechiae 540±30 µm (mean ±1 standard deviation). CCT influences GAT
{z Primary angle-closure glaucoma. readings. Eyes with a thin cornea have a true IOP that is greater
{z Anterior uveitis. than the measured IOP. Conversely, eyes with a thick cornea have a
{z Iridocorneal endothelial (ICE) syndrome. true IOP that is lower than the measured IOP. There is no validated
zzNeovascularization and useful correction algorithm for these two measurements.
{z Neovascular glaucoma. Patients with NTG tend to have thin CCT measurements. It is an
{z Fuchs heterochromic cyclitis. important value when determining risk of conversion to glaucoma
{z Chronic anterior uveitis. in individuals with raised IOP (see Ch. 11). It is also an easy and
zzHyperpigmentation practical indicator of corneal endothelial function.
{z Physiological variant.
{z Pigment dispersion syndrome. TIP CCT should be measured in individuals with ocular
{z Pseudophakic pigment dispersion. hypertension, as it helps to determine the risk of conversion to
{z Pseudoexfoliation syndrome. glaucoma.

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 Chapter

Eyelids 2
INTRODUCTION 38 Madarosis 63 Surgery 79
Anatomy 38 Poliosis 63
ECTROPION 80
Terminology 39 ALLERGIC DISORDERS 64 Involutional ectropion 80
General considerations 39
Acute allergic oedema 64 Cicatricial ectropion 84
NON-NEOPLASTIC LESIONS 39 Contact dermatitis 64 Paralytic ectropion/facial nerve
Chalazion (meibomian cyst) 39 palsy 84
IDIOPATHIC DERMATITIS 64 Mechanical ectropion 85
Other eyelid cysts 41
Atopic dermatitis 64
Xanthelasma 41 ENTROPION 85
Periorificial dermatitis 65
BENIGN EPIDERMAL TUMOURS 43 Involutional entropion 85
IMMUNE-RELATED Cicatricial entropion 86
Squamous cell papilloma 43 INFLAMMATION 66
Seborrhoeic keratosis 43 MISCELLANEOUS ACQUIRED
Dermatomyositis 66
Actinic keratosis 43 DISORDERS 87
Systemic monoclonal antibody
BENIGN PIGMENTED LESIONS 43 therapy 66 Varix 87
Freckle 43 Dermatochalasis 87
BACTERIAL INFECTIONS 66
Lentigo 45 Floppy eyelid syndrome 87
External hordeolum 66
Congenital melanocytic naevus 45 Blepharochalasis 90
Impetigo 66
Acquired melanocytic naevus 45 Eyelid imbrication syndrome 90
Erysipelas 66
Upper lid retraction 90
BENIGN ADNEXAL TUMOURS 46 Necrotizing fasciitis 66
Lower lid retraction 91
Syringoma 46 VIRAL INFECTIONS 67
Pilomatricoma 46 COSMETIC EYELID AND
Molluscum contagiosum 67 PERIOCULAR SURGERY 91
MISCELLANEOUS BENIGN Mpox (Monkey pox) 69 Involutional changes 91
TUMOURS 48 Herpes zoster ophthalmicus 69 Non-surgical techniques 91
Capillary haemangioma 48 Herpes simplex 69 Surgical techniques 91
Port-wine stain 48 BLEPHARITIS 70
Pyogenic granuloma 50 CONGENITAL MALFORMATIONS 92
Chronic blepharitis 70 Epicanthic folds 92
Neurofibroma 50 Phthiriasis palpebrarum 73 Blepharophimosis, ptosis and
MALIGNANT TUMOURS 50 Tick infestation of the eyelid 73 epicanthus inversus syndrome 92
Rare predisposing conditions 50 Angular blepharitis 74 Telecanthus 94
Basal cell carcinoma 50 Childhood Epiblepharon 94
Squamous cell carcinoma 51 blepharokeratoconjunctivitis 74 Congenital entropion 94
Keratoacanthoma 54 PTOSIS 75 Coloboma 94
Sebaceous gland carcinoma 54 Classification 75 Cryptophthalmos 95
Lentigo maligna and melanoma 54 Clinical evaluation 75 Euryblepharon 95
Merkel cell carcinoma 56 Simple congenital ptosis 77 Microblepharon 95
Kaposi sarcoma 57 Marcus Gunn jaw-winking Congenital upper lid eversion 95
Treatment of malignant tumours 57 syndrome 77 Ablepharon 95
DISORDERS OF THE EYELASHES 61 Third nerve misdirection Ankyloblepharon filiforme
syndromes 79 adnatum 95
Misdirected lashes 61
Eyelash ptosis 63 Involutional ptosis 79
Trichomegaly 63 Mechanical ptosis 79
37
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38 Introduction

zzPrickle cell layer (stratum spinosum) is approximately

INTRODUCTION five cells deep. The cells are polygonal in cross-section
and have abundant eosinophilic cytoplasm. Their free bor-
Anatomy ders are united by spiny-appearing desmosomes (cellular
The skin (Fig. 2.1A) consists of the epidermis, dermis and related junctions).
structures (adnexa). zzBasal cell layer (stratum basale) comprises a single row of
columnar-shaped proliferating cells containing melanin
Epidermis derived from adjacent melanocytes.!
The epidermis comprises four layers of keratin-producing cells
(keratinocytes). It also contains melanocytes, Langerhans cells Dermis
and Merkel cells. The layers of the epidermis around the eye are The dermis is much thicker than the epidermis. It is composed
described below. Cells migrate superficially, undergoing matura- of connective tissue and contains blood vessels, lymphatics and
tion and differentiation through successive layers. nerve fibres in addition to fibroblasts, macrophages and mast cells.
zzKeratin layer (stratum corneum or horny layer) consists of flat Upward dermal extensions (papillae) interdigitate with downward
cells devoid of nuclei. epidermal projections (rete ridges). In the eyelid the dermis lies
zzGranular cell layer (stratum granulosum) typically consists of one on the orbicularis muscle. Adnexa lie deep in the dermis or within
or two layers of flattened cells containing keratohyaline granules. the tarsal plates.

A B

C D

Fig. 2.1 Eyelid skin. (A) Normal skin is composed of keratinized stratified epithelium that covers
the surface; pilosebaceous elements are conspicuous in the dermis and a few blood vessels
and sweat glands are also seen; (B) dysplasia with loss of cell polarity; (C) dyskeratosis – a
non-surface epithelial cell producing keratin; (D) parakeratosis – retention of cell nuclei into
the surface keratin layer. (Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology,
Butterworth-Heinemann, 2001.)

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Eyelids 39

zzSebaceous glands are located in the caruncle and within eye- of normally structured and recognized layers of tissue (e.g. loss
brow hairs. Tiny sebaceous glands are associated with the thin of cell polarity – Fig. 2.1B).
(vellus) hairs covering periocular skin. zzCarcinoma in situ (intraepidermal carcinoma, Bowen dis-
zzMeibomian glands are modified sebaceous glands found in ease) exhibits dysplastic changes throughout the thickness of
the tarsal plates. They empty through a single row of 20–30 the epidermis.
orifices on each lid. A gland consists of a central duct with zzHyperkeratosis. An increase in thickness of the keratin layer
multiple acini, the cells of which synthesize lipids (meibum) that appears clinically as scaling. Hyperkeratosis can be a fea-
that form the outer layer of the tear film. ture of benign or malignant epithelial tumours.
zzGlands of Zeis are modified sebaceous glands associated with zzAcanthosis. Thickening of the prickle cell layer.
lash follicles. zzDyskeratosis is keratinization other than on the epithelial
zzGlands of Moll are modified apocrine sweat glands open- surface (Fig. 2.1C).
ing either into a lash follicle or directly onto the anterior zzParakeratosis is the retention of nuclei into the keratin layer
lid margin between lashes. They are more numerous in the (Fig. 2.1D).!
lower lid.
zzEccrine sweat glands are distributed throughout eyelid skin
General considerations
and are not confined to the lid margin, in contrast to glands
of Moll. zzClassification. Epidermal, adnexal or dermal.
zzPilosebaceous units comprise hair follicles and their seba- zzDiagnosis. The clinical characteristics of benign lesions are
ceous glands (see Fig. 2.1A).! a tendency to a lack of induration and ulceration, uniform
colour, limited growth, regular outline and preservation of
normal lid margin structures. Biopsy may be required if the
Terminology appearance is suspicious.
{z Incisional biopsy involves removal of a portion of a lesion

Clinical for histopathology.

{z Excision biopsy is performed on small tumours and fulfils
zzMacule. Localized area of colour change without infiltration,
depression or elevation, less than 1 cm in diameter. both diagnostic and treatment objectives.
zzTreatment options include:
zzPapule. A solid elevation less than 1 cm in diameter.
{z Excision of the entire lesion and a small surrounding por-
zzVesicle. Circumscribed lesion containing serous fluid (less
than 0.5 cm across). tion of normal tissue.
{z Marsupialization involves the removal of the top of a
zzBulla. A large (more than 0.5 cm) serous fluid-filled lesion
(plural – bullae). cyst allowing drainage of its contents and subsequent
zzPustule. A pus-filled elevation less than 1 cm in diameter. epithelialization.
{z Ablation with laser or cryotherapy.!
zzCrust. Solidified serous or purulent exudate.
zzNodule. A palpable solid area measuring more than 1 cm.
zzCyst. A nodule consisting of an epithelial-lined cavity filled NON-NEOPLASTIC LESIONS
with fluid or semi-solid material.
zzPlaque. A solid elevation of the skin, greater than 1 cm in Chalazion (meibomian cyst)
diameter.
zzScale. Readily detached fragments of shed keratin layer.
Pathogenesis
zzPapilloma. A benign neoplastic warty or tag-like projection of A chalazion is a sterile chronic granulomatous inflammatory lesion
the skin or mucous membrane. (lipogranuloma) of the meibomian, or sometimes Zeis, glands
zzUlcer. A circumscribed area of epithelial loss. In skin an ulcer caused by retained sebaceous secretions. Histopathology shows a
extends through the epidermis into the dermis.! lipogranulomatous chronic inflammatory picture with extracel-
lular fat deposits surrounded by lipid-laden epithelioid cells, mul-
Histological tinucleated giant cells and lymphocytes (Fig. 2.2A). Blepharitis is
commonly present; rosacea can be associated with multiple and
zzTumour strictly refers only to a swelling, though is commonly
recurrent chalazia. Bortezomib, a proteasome inhibitor used in
used to denote a neoplasm.
the treatment of multiple myeloma, predisposes to the formation
zzNeoplasia. Abnormal tissue growth, either benign (localized,
of chalazia within 3 months of initiation of treatment. A recurrent
non-invasive and non-spreading) or malignant (progressive
chalazion should be biopsied to exclude masquerading malignancy.
growth with the potential for distant spread).
zzAtypia refers to an abnormal appearance of individual cells,
e.g. abnormal mitotic figures. TIP If a chalazion recurs in the same lid position in an
zzDysplasia is an alteration of the size, morphology and organi- older patient, consider undertaking a biopsy to exclude
malignancy.
zation of cellular components of a tissue. There is disturbance !

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40 Non-Neoplastic Lesions

A B

C D

E F

Fig. 2.2 Chalazion. (A) Histopathology showing a lipogranuloma; the large pale cells are
epithelioid cells and the well-demarcated empty space contained fat dissolved out during
processing; (B) uninflamed chalazion; (C) marginal chalazion with superimposed bacterial
infection; (D) conjunctival chalazion; (E) conjunctival view of chalazion clamp in place; (F)
after curettage. (Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology,
Butterworth-Heinemann 2001 – fig. A.)

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CHAPTER
Eyelids 41

Diagnosis or by incision and curettage via a horizontal incision on the

conjunctival surface or vertically through the grey line.
zzSymptoms
zzProphylaxis
{z Subacute/chronic: gradually enlarging painless rounded
{z Treatment of blepharitis, e.g. daily lid hygiene regimen.
nodule (Fig. 2.2B).
{z Systemic tetracycline may be required as prophylaxis in
{z Acute: sterile inflammation or bacterial infection with
patients with recurrent chalazia, particularly if associated
localized cellulitis. A secondarily infected meibomian
with acne rosacea.!
gland is referred to as an internal hordeolum (Fig. 2.2C).!
zzSigns
{z A nodule within the tarsal plate, sometimes with associ- Other eyelid cysts
ated inflammation (Fig. 2.2D).
{z Bulging inspissated secretions may be visible at the orifice zzCyst of Zeis is a small, non-translucent cyst on the anterior lid
of the involved gland. margin arising from obstructed sebaceous glands associated
{z There may be an associated conjunctival granuloma. with the eyelash follicle (Fig. 2.3A).
{z A lesion at the anterior lid margin may be connected to zzCyst of Moll (apocrine hidrocystoma) is a small retention cyst
a typical chalazion deeper in the lid or be due to isolated of the lid margin apocrine glands. It appears as a round, non-
involvement of a gland of Zeis.! tender, translucent fluid-filled lesion on the anterior lid margin
(Fig. 2.3B).
Treatment zzSebaceous (pilar) cyst is caused by a blocked pilosebaceous
zzOral antibiotics are required for significant bacterial infec- follicle and contains sebaceous secretions; the gland orifice will
tion, but not for sterile inflammation. o%en be visible (Fig. 2.3C). It is only rarely found on the eyelid
zzConservative. At least a third resolve spontaneously so obser- although it may occasionally occur at the inner canthus.
vation may be appropriate, especially if the lesion is showing zzComedones are plugs of keratin and sebum within the dilated
signs of improvement. orifice of hair follicles that o%en occur in patients with acne
zzHot compress application several times daily may aid resolu- vulgaris. They may be either open (blackheads), containing
tion, particularly in early lesions. a darkened plug of oxidized material (Fig. 2.3D), or closed
zzExpression. Compression between two cotton-tipped applica- (whiteheads).
tors is sometimes effective in expressing the contents of a fresh zzMilia are caused by occlusion of pilosebaceous units resulting
lesion near the lid margin. in retention of keratin. They are tiny, white, round, superficial
zzSteroid injection into or around the lesion has been reported papules that tend to occur in crops (Fig. 2.3E).
to give similar resolution rates to incision and curettage (see zzEpidermal inclusion cyst is usually caused by implantation of
below). It may be preferred for marginal lesions or lesions epidermis into the dermis following trauma or surgery. It is a
close to structures such as the lacrimal punctum because of slow-growing, round, firm, superficial or subcutaneous lesion
the risk of surgical damage. containing keratin (Fig. 2.3F).
{z Reported regimens include 0.2–2 ml of triamcinolone zzEpidermoid cyst is uncommon and usually developmental,
acetonide aqueous suspension diluted with lidocaine to occurring along embryonic lines of closure. It is similar in
a concentration of 5 mg/ml and 0.1–0.2 ml of 40 mg/ml, appearance to an epidermal inclusion cyst.
injected with a 27- or 30-gauge needle. zzDermoid cyst is usually subcutaneous or deeper and is typi-
{z The success rate following one injection is about 80%. A cally attached to the periosteum at the lateral end of the brow
second can be given 1–2 weeks later. (Fig. 2.3G). It is caused by skin sequestered during embryonic
{z Local skin depigmentation and fat atrophy are uncommon development.
complications, which are less likely to occur by utilizing a zzEccrine hidrocystoma is less common but similar in appear-
conjunctival approach. ance to a cyst of Moll except that it is usually located along the
{z Retinal vascular occlusion is a rare complication. medial or lateral aspects of the lid and is close to, but does not
zzSurgery involve, the lid margin itself (Fig. 2.3H).!
{z Following local anaesthesia infiltration, the eyelid is
everted with a specialized clamp (Fig. 2.2E), the cyst is
incised vertically through the tarsal plate and its contents
Xanthelasma
curetted (Fig. 2.2F).
{z Limited excision of solid inflammatory material (sent for Introduction
histopathology) with fine scissors may be helpful in some Xanthelasma (plural – xanthelasmata) is a common, frequently
cases, especially if there is no focus of secretions. bilateral condition typically affecting middle-aged and elderly
{z A suture should not be used. individuals. It is a subtype of xanthoma. Hyperlipidaemia is
{z Topical antibiotic is used three times daily for 5 days fol- found in about one-third of patients, in whom corneal arcus may
lowing curettage. also be present. In contrast to chalazion, fat in xanthelasmata is
zzMarginal lesions can be managed by steroid injection, by mainly intracellular, with lipid-laden histiocytes (foam cells) in
curettage of an associated deeper chalazion, by shave curettage the dermis.!

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B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
42 Non-Neoplastic Lesions

A B

C D

E F

G H

Fig. 2.3 Eyelid cysts. (A) Cyst of Zeis; (B) cyst of Moll; (C) sebaceous cyst; (D) comedones –
blackheads; (E) milia (arrows); (F) epidermal inclusion cyst; (G) dermoid cyst; (H) eccrine hidro-
cystomas. (Courtesy of A Pearson – figs D and H.)

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 2
CHAPTER
Eyelids 43

A B

Fig. 2.4 Xanthelasma. (A) Large isolated lesions; (B) multiple smaller lesions.

Diagnosis keratin-filled horns or cystic inclusions (Fig. 2.6A). Seborrhoeic

Xanthelasmata are yellowish subcutaneous plaques, usually in the keratosis (basal cell papilloma) is an extremely common slowly
medial aspects of the eyelids (Fig. 2.4A), commonly bilateral and growing lesion found on the face, trunk and extremities of elderly
are multiple (Fig. 2.4B).! individuals as a discrete light- to dark-brown plaque with a friable,
greasy, verrucous surface and a ‘stuck-on’ appearance (Fig. 2.6B).
Treatment They are frequently numerous. The differential diagnosis includes
This is principally for cosmesis. Recurrence occurs in up to 50% pigmented basal cell carcinoma, naevus and melanoma. Treatment
and is most common in patients with hypercholesterolaemia. involves shave biopsy (occasionally simple excision), electrodesic-
zzSimple excision is commonly performed where adequate cation with curettage, laser ablation, cryotherapy with liquid nitro-
excess skin is present. gen and chemical peeling.!
zzMicrodissection. Larger lesions can be raised in a flap, the
fatty deposits dissected from overlying skin under a surgical
microscope using micro scissors and the skin replaced.
Actinic keratosis
zzOther methods. Good results can be obtained using chemi- Histopathology (Fig. 2.7A) shows irregular dysplastic epidermis
cal peeling with bi- or trichloroacetic acid. Laser ablation and with hyperkeratosis, parakeratosis and occasionally cutaneous
cryotherapy have advantages but may be more prone to scar- horn formation. Actinic (solar, senile) keratosis is a common
ring, including pigmentary changes.! slowly growing lesion that rarely develops on the eyelids. It typi-
cally affects elderly, fair-skinned individuals on areas of sun-
damaged skin, such as the forehead and backs of the hands, and
BENIGN EPIDERMAL TUMOURS appears as a hyperkeratotic plaque with distinct borders and a
scaly surface that may become fissured (Fig. 2.7B). Occasionally
Squamous cell papilloma the lesion is nodular or wart-like and may give rise to a cutaneous
Histopathology in all clinical types is similar, showing finger-like horn. It has potential, though low, for transformation into squa-
projections of fibrovascular connective tissue covered by irregular mous cell carcinoma. Treatment involves biopsy followed by exci-
acanthotic and hyperkeratotic squamous epithelium (Fig. 2.5A). sion or cryotherapy.!
Squamous cell papilloma is a very common benign epithelial
tumour with a range of clinical appearances, including narrow-
based (pedunculated or ‘skin tag’ – Fig. 2.5B), pink broad-based
BENIGN PIGMENTED LESIONS
(sessile – Fig. 2.5C) and whitish thread-like (filiform) hyperkera-
totic lesions (Fig. 2.5D). The incidence increases with age. Some
Freckle
cases result from human papilloma virus infection. Treatment A freckle (ephelis, plural ephelides) is a small (generally 1–5 mm)
usually involves simple excision, but other options include cryo- brown macule due to increased melanin in keratinocytes, typically
therapy and laser or chemical ablation.! in sun-exposed skin. This is particularly common in fair-skinned
individuals and children with red hair, where the MC1R gene is
involved (Fig. 2.8A). The number of freckles varies with the level of
Seborrhoeic keratosis sun exposure and can sometimes regress completely. Histopathol-
Histopathology shows expansion of the squamous epithelium ogy shows hyperpigmentation of the basal layer of the epidermis,
of the epidermis by proliferating basal cells, sometimes with with a normal melanocyte population.!

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
44 Benign Pigmented Lesions

A B

C D

Fig. 2.5 Squamous cell papilloma. (A) Histopathology showing finger-like projections of
fibrovascular connective tissue covered by irregular acanthotic and hyperkeratotic squamous
epithelium; (B) pedunculated ‘skin tag’; (C) sessile lesion; (D) hyperkeratotic filiform lesion.

A B

Fig. 2.6 Basal cell papilloma. (A) Histopathology showing an elevated expansion of the epi-
dermis with proliferation from basal cells – horn cysts and pseudo-horn cysts are present; (B)
typical ‘stuck-on’ appearance. (Courtesy of A Pearson – fig. B.)

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 2
CHAPTER
Eyelids 45

A B

Fig. 2.7 Actinic keratosis. (A) Histopathology showing irregular dysplastic epidermis with
hyperkeratosis and parakeratosis; (B) clinical appearance. (Courtesy of J Harry and G Misson,
from Clinical Ophthalmic Pathology, Butterworth-Heinemann 2001 – fig. A.)

A B

Fig. 2.8 Benign pigmented lesions. (A) Freckle (ephelis) (arrows); (B) lentigo (arrows).

lesion covering the face (Fig. 2.9C) or an extensive area of the body
Lentigo (‘giant hairy naevus’). Large lesions have the potential for malig-
Lentigo (plural: lentigines) are harmless flat, brownish lesions with nant transformation (up to 15%). Treatment, if necessary, involves
a clearly defined scalloped edge that are larger than freckles. They complete surgical excision.!
may have a dry or slightly scaly surface. Solar lentigines occur in
middle-aged individuals and result from sun damage. They are
persistent and do not disappear in the winter (Fig. 2.8B). Histopa- Acquired melanocytic naevus
thology shows a proliferation of keratinocytes and an increase in
the number of melanocytes.! Diagnosis
The clinical appearance and potential for malignant transformation
of a naevus is determined by the histological location within the skin.
Congenital melanocytic naevus zzJunctional naevus occurs in young individuals as a uniformly
Congenital naevi are uncommon and histologically resemble brown macule or plaque (Fig. 2.10A). The naevus cells are
their acquired counterparts (see below). They are usually small located at the junction of the epidermis and dermis and have a
and of uniform colour. Rare variants include a ‘kissing’ or split low potential for malignant transformation (Fig. 2.10B).
naevus that involves the upper and lower eyelid (Fig. 2.9A) and zzCompound naevus occurs in middle age as a raised papular
may occasionally contain numerous hairs (Fig. 2.9B), and a large lesion. The shade of pigment varies from light tan to dark

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
46 Benign Adnexal Tumours

B C

Fig. 2.9 Congenital melanocytic naevus. (A) Split naevus; (B) split naevus containing hair; (C)
large facial naevus. (Courtesy of A Pearson – fig. B; RSR Naik – fig. C.)

brown but tends to be relatively uniform throughout (Fig. complete in most cases, with at least a 3 mm margin if melanoma
2.10C). The naevus cells extend from the epidermis into the is suspected.!
dermis (Fig. 2.10D). It has a low malignant potential related to
the junctional component.
zzIntradermal naevus, the most common, typically occurs in
BENIGN ADNEXAL TUMOURS
older patients. It is a papillomatous lesion, with little or no
pigmentation (Fig. 2.10E). Histologically, naevus cells are
Syringoma
confined to the dermis and have no malignant potential (Fig. Syringomas are benign proliferations arising from eccrine sweat
2.10F). glands. They are characterized by small papules that are o%en mul-
zzVariants of naevus include balloon cell naevus, halo naevus, tiple and bilateral (Fig. 2.11A).!
Spitz naevus (juvenile melanomas) and dysplastic naevus
(atypical moles). Multiple dysplastic naevi constitute the dys-
plastic naevus syndrome (atypical mole syndrome – AMS).
Pilomatricoma
Individuals with AMS are at increased risk of developing Pilomatricoma (pilomatrixoma, calcifying epithelioma of Mal-
conjunctival and uveal naevi and cutaneous, conjunctival and herbe) is derived from the germinal matrix cells of the hair bulb
uveal melanomas.! and is the commonest hair follicle proliferation seen by ophthal-
mologists. It affects children and young adults and is more com-
Treatment mon in females. Histopathology shows irregular epithelial islands
Treatment is indicated for cosmesis or if the naevus has unusual exhibiting viable basophilic cells at the periphery and degenerate
features that raise concern about malignancy. Excision should be ‘shadow’ cells centrally. Clinically it appears as a mobile purplish

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 2
CHAPTER
Eyelids 47

A B

C D

E F

Fig. 2.10 Acquired melanocytic naevus. (A) Junctional naevus; (B) histopathology showing
heavily pigmented naevus cells at the epidermal/dermal junction; (C) compound naevus;
(D) histopathology showing naevus cells both at the epidermal/dermal junction and within
the dermis; (E) intradermal naevus showing little pigmentation and eccentric lashes; (F)
histopathology showing naevus cells within the dermis separated from the epidermis by a
clear zone.

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
48 Miscellaneous Benign Tumours

A B

Fig. 2.11 Benign adnexal tumours. (A) Syringomas; (B) pilomatricoma. (Courtesy of A Pearson
– fig. A.)

A B

Fig. 2.12 Capillary haemangioma. (A) Unilateral, raised and bright red lesion; (B) mechanical
ptosis.

dermal nodule that may have a hard consistency due to calcifica- frequent and there may be orbital extension (see Ch. 4). Occasion-
tion (Fig. 2.11B). Calcification is frequently present and there is ally the lesion may involve the skin of the face and some patients
o%en a foreign body giant cell reaction. Malignant change is rare. have strawberry naevi on other parts of the body. It is important
The lesion is usually removed surgically. Other less common disor- to be aware of an association between multiple cutaneous lesions
ders of hair follicle proliferation include trichofolliculoma, tricho- and visceral haemangiomas and to consider systemic assessment
epithelioma and trichilemmoma.! in appropriate cases. Treatment is described in Chapter 4.

MISCELLANEOUS BENIGN TUMOURS TIP A capillary haemangioma can be easily and successfully
treated by regular application of a topical beta-blocker to the
Capillary haemangioma affected lesion.
!
Capillary haemangioma (strawberry naevus) is one of the most
common tumours of infancy. It is three times as common in girls as
boys. Histopathology shows proliferation of varying-sized vascular
Port-wine stain
channels in the dermis and subcutaneous tissue. It presents shortly
a%er birth as a unilateral, raised bright red lesion (Fig. 2.12A), usu- Introduction
ally in the upper lid. A deeper lesion appears purplish (Fig. 2.12B). Port-wine stain (naevus flammeus) is a congenital malformation of
The lesion blanches on pressure and may swell on crying. Ptosis is vessels within the superficial dermis, consisting histopathologically

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B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 2
CHAPTER
Eyelids 49

A B C

D E F
Fig. 2.13 Port-wine stain. (A) Histopathology showing widely dilated blood-filled spaces
separated by fibrous septa; (B) and (C) clinical appearance; (D–F) progression of port-wine
stain over time, with associated underlying soft tissue hypertrophy.

of vascular spaces of varying calibre separated by thin fibrous septa preparations such as imiquimod and rapamycin, alone or with
(Fig. 2.13A). About 10% have associated ocular or CNS involve- adjuvant laser, show promise. So% tissue debulking is used in a
ment, including Sturge–Weber (see below) and other defined small number of cases. Screening for glaucoma should begin in
syndromes.! infancy. Systemic investigation is considered in some patients, par-
ticularly those with a lesion of the lumbar area.!
Diagnosis
Port-wine stain manifests clinically as a sharply demarcated so% Sturge–Weber syndrome
pink patch that does not blanch with pressure, most frequently Sturge–Weber syndrome (encephalotrigeminal angiomatosis) is a
located on the face. It is usually unilateral and tends to be aligned congenital, sporadic phacomatosis.
with the skin area supplied by one or more divisions of the tri- zzPort-wine stain, extending over the area corresponding to
geminal nerve (Fig. 2.13B and C). Darkening to red or purple the distribution of one or more branches of the trigeminal
takes place with age and there is commonly associated so% tis- nerve.
sue hypertrophy (Fig. 2.13D–F). Bleeding may occur from focal zzLeptomeningeal haemangioma involving the ipsilateral pari-
overlying lobulations (pyogenic granulomas – see below).! etal or occipital region may cause contralateral focal or gener-
alized seizures, hemiparesis or hemianopia.
Treatment zzOcular features may include ipsilateral glaucoma, episcleral
Treatment with laser (e.g. pulsed-dye) is effective in decreas- haemangioma, iris heterochromia and diffuse choroidal haem-
ing skin discoloration, particularly if undertaken early. Topical angioma (see Ch. 20).!

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B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
50 Malignant Tumours

A B

Fig. 2.14 Other benign tumours. (A) Pyogenic granuloma of the tarsal surface of the lower lid;
(B) plexiform neurofibroma – characteristic S-shaped upper lid.

patients have a bird-like facial appearance and a significant

Pyogenic granuloma propensity to the development of basal cell carcinoma (BCC),
Pyogenic granuloma is a rapidly growing vascularized prolif- squamous cell carcinoma (SCC) and melanoma, which are
eration of granulation tissue that is usually antedated by surgery, commonly multiple. Conjunctival malignancies have also
trauma or infection, although some cases are idiopathic. Clinically been reported.
there is a painful, rapidly growing, vascular granulating polypoi- zzGorlin–Goltz syndrome (naevoid BCC syndrome) is a rare
dal lesion (Fig. 2.14A) that may bleed following relatively trivial autosomal dominant (AD) disorder characterized by exten-
trauma. Cutaneous lesions should be excised. Conjunctival pyo- sive congenital deformities of the eye, face, bone and central
genic granuloma is discussed in Chapter 6.! nervous system. Many patients develop multiple small BCC
during the second decade of life (Fig. 2.15B) and are also pre-
disposed to medulloblastoma, breast carcinoma and Hodgkin
Neurofibroma lymphoma.
Cutaneous neurofibromas are benign nerve tumours, usually zzMuir–Torre syndrome is a rare AD condition that predisposes
nodular or pedunculated, that can be found anywhere on the skin. to cutaneous and internal malignancies. Cutaneous tumours
An isolated neurofibroma is common in normal individuals, but include BCC, sebaceous gland carcinoma and keratoacan-
if multiple lesions are present, neurofibromatosis (see Ch. 19) thoma. Colorectal and genitourinary carcinomas are the most
should be excluded. Plexiform neurofibromas typically present in common systemic tumours.
childhood as a manifestation of neurofibromatosis type 1 with a zzBazex syndrome can be used to describe two distinct condi-
characteristic S-shaped deformity of the upper eyelid (Fig. 2.14B). tions: Bazex–Dupré–Christol syndrome, an X-linked domi-
Treatment of solitary lesions involves simple excision, but removal nant condition characterized by multiple BCCs, commonly
of the more diffuse plexiform lesions may be difficult.! facial including the eyelids, associated with skin changes
including follicular indentations without hairs on extensor
surfaces (follicular atrophoderma), hypohidrosis and hypo-
MALIGNANT TUMOURS trichosis and acrokeratosis paraneoplastica of Bazex, in which
The treatment of malignant eyelid tumours in general is discussed eczema-like and psoriasiform lesions are associated with an
at the end of this section. underlying malignancy of the upper respiratory or digestive
tract.
zzOther predispositions include immunosuppression, prior
Rare predisposing conditions retinoblastoma and albinism.!
Young patients who suffer from one of the following conditions
may develop eyelid malignancies.
zzXeroderma pigmentosum is characterized by skin damage
Basal cell carcinoma
on exposure to sunlight, leading to progressive cutaneous
abnormalities (Fig. 2.15A). More than 90% have ocular or Introduction
periocular involvement and 65% experience photophobia. It BCC is the most common human malignancy and typically
is inherited in an autosomal recessive (AR) fashion. Affected affects older people. The most important risk factors are fair

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B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 2
CHAPTER
Eyelids 51

A B

Fig. 2.15 Predispositions to eyelid malignancies. (A) Xeroderma pigmentosum; (B) Gorlin–
Goltz syndrome. (Courtesy of J Krachmer, M Mannis and E Holland, from Cornea, Mosby
2005 – fig. B.)

skin, inability to tan and chronic exposure to sunlight. Ninety zzSclerosing (morphoeic) BCC is less common and may be
per cent of cases occur in the head and neck and about 10% difficult to diagnose because it infiltrates laterally beneath
of these involve the eyelid. BCC is by far the most common the epidermis as an indurated plaque (Fig. 2.16G and H).
malignant eyelid tumour, accounting for 90% of all cases. It The margins of the tumour may be impossible to delineate
most frequently arises from the lower eyelid, followed in rela- clinically and the lesion tends to be much more extensive
tive frequency by the medial canthus, upper eyelid and lateral on palpation than inspection. On cursory examination a
canthus. Madarosis is commonly seen in association with the sclerosing BCC may simulate a localized area of chronic
tumour, which is slow-growing and locally invasive, but non- blepharitis.
metastasizing. Tumours located near the medial canthus are zzOther types not usually found on the lid are cystic, adenoid,
more prone to invade the orbit and sinuses, are more difficult to pigmented and multiple superficial.
manage than those arising elsewhere and carry the greatest risk
of recurrence. Tumours that recur following incomplete treat-
ment tend to be more aggressive.!
TIP Sclerosing BCC can mimic a localized area of unilateral
chronic blepharitis.
!
Histopathology
The tumour arises from the cells that form the basal layer of
the epidermis. The cells proliferate downwards (Fig. 2.16A) and
Squamous cell carcinoma
characteristically exhibit palisading at the periphery of a tumour
lobule of cells (Fig. 2.16B). Squamous differentiation with the Introduction
production of keratin results in a hyperkeratotic type of BCC. SCC is a much less common, but typically more aggressive tumour
There can also be sebaceous and adenoid differentiation while than BCC, with metastasis to regional lymph nodes in about 20%
the growth of elongated strands and islands of cells embedded of cases. Histopathology shows dysplastic changes throughout
in a dense fibrous stroma result in a sclerosing (morphoeic) type the thickness of the epidermis (Fig. 2.17A). Careful surveillance
of tumour.! of regional lymph nodes is therefore an important aspect of ini-
tial management. The tumour may also exhibit perineural spread
Clinical features to the intracranial cavity via the orbit. SCC accounts for 5–10% of
Eyelid BCC conforms to one of the morphological patterns below. eyelid malignancies and may arise de novo or from pre-existing
zzNodular BCC is a shiny, firm, pearly nodule with small over- actinic keratosis or carcinoma in situ (Bowen disease, intraepi-
lying dilated blood vessels. Initially, growth is slow and it may dermal carcinoma – Fig. 2.17B). Immunocompromised individ-
take the tumour 1–2 years to reach a diameter of 0.5 cm (Fig. uals, such as those with acquired immunodeficiency syndrome
2.16C and D). (AIDS) or following renal transplantation are at increased risk,
zzNodulo-ulcerative BCC (rodent ulcer) is centrally ulcer- as are those with a predisposing syndrome such as xeroderma
ated with pearly raised rolled edges and dilated and irregu- pigmentosum. The tumour has a predilection for the lower eyelid
lar blood vessels (telangiectasis) over its lateral margins (Fig. and the lid margin. It occurs most commonly in older individu-
2.16E). With time it may erode a large portion of the eyelid als with a fair complexion and a history of chronic sun expo-
(Fig. 2.16F). sure. The diagnosis of SCC may sometimes be difficult because

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
52 Malignant Tumours

A B

C D

E F

G H

Fig. 2.16 Basal cell carcinoma. (A) Histopathology showing downward proliferation of lobules
of basophilic (blue) cells (arrow); (B) palisading of cells at the periphery of a tumour lobule
(arrow); (C) small lid margin tumour; (D) larger nodular tumour; (E) rodent ulcer; (F) large rodent
ulcer; (G) sclerosing tumour; (H) extensive sclerosing tumour.

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 2
CHAPTER
Eyelids 53

A B

C D

E F

Fig. 2.17 Squamous cell carcinoma. (A) Histopathology of carcinoma in situ showing dysplas-
tic changes throughout the thickness of the epidermis; (B) Bowen disease; (C) histopathol-
ogy of squamous cell carcinoma showing acanthotic squamous epithelium and eosinophilic
(pink) islands of dysplastic squamous epithelium within the dermis. Note the large nucleus
with more than one large purple nucleoli (arrows); (D) nodular tumour with surface keratosis;
(E) ulcerating tumour; (F) cutaneous horn. (Courtesy of ADN Murray – fig. E.)

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
54 Malignant Tumours

certain ostensibly benign lesions such as keratoacanthoma and

cutaneous horn may reveal histological evidence of invasive SCC
Sebaceous gland carcinoma
at deeper levels of sectioning.!
Introduction
Histopathology Sebaceous gland carcinoma (SGC) is a very rare, slowly growing
The tumour arises from the squamous cell layer of the epidermis. tumour that most frequently affects the elderly, with a predispo-
It is composed of variably sized groups of atypical epithelial cells sition for females. It usually arises from the meibomian glands,
with prominent nuclei and abundant eosinophilic cytoplasm although on occasion it may arise from the glands of Zeis or else-
within the dermis (Fig. 2.17C). Well-differentiated tumours where. Histopathology demonstrates lobules of cells with pale
may show characteristic keratin ‘pearls’ and intercellular bridges foamy vacuolated lipid-containing cytoplasm and large hyper-
(desmosomes).! chromatic nuclei. Pagetoid spread refers to extension of a tumour
within the epithelium and is not uncommon. Overall mortality
Clinical features is 5–10%. Adverse prognostic features include upper lid involve-
The clinical types are variable and there are no pathognomonic ment, tumour size of 10 mm or more and duration of symptoms of
characteristics. The tumour may be indistinguishable clinically more than 6 months.!
from a BCC but surface vascularization is usually absent, growth is
more rapid and hyperkeratosis is more common. Clinical features
zzNodular SCC is characterized by a hyperkeratotic nodule that In contrast to BCC and SCC, SGC occurs more commonly on the
may develop crusting, erosions and fissures (Fig. 2.17D). upper eyelid where meibomian glands are more numerous. There
zzUlcerating SCC has a red base and sharply defined, indurated may be simultaneous involvement of the lower and upper lid on
and everted borders, but pearly margins and telangiectasia are one side (5%).
not usually present (Fig. 2.17E). zzYellowish material within the tumour is highly suggestive of
zzCutaneous horn with underlying invasive SCC (Fig. 2.17F). SGC.
zzNodular SGC presents as a discrete, hard nodule, most com-
monly within the upper tarsal plate (Fig. 2.19A) and may
TIP Ostensibly benign lesions such as keratoacanthoma and exhibit yellow discoloration due to the presence of lipid; it can
cutaneous horn may reveal histological evidence of SCC in
be mistaken for a chalazion.
deeper levels of sectioning.
! zzSpreading SGC infiltrates into the dermis and causes a diffuse
thickening of the lid margin (Fig. 2.19B) o%en with eyelash
distortion and loss and can be mistaken for blepharitis.
Keratoacanthoma
Introduction TIP SGC can be confused with chronic or recurrent localized
Keratoacanthoma is a rare, rapidly growing but subsequently
meibomian gland inflammation.
!
regressing tumour that usually occurs in fair-skinned individuals
with a history of chronic sun exposure. Immunosuppressive therapy
is also a predisposing factor. Invasion and metastasis are rare. His-
Lentigo maligna and melanoma
topathological examination reveals irregular thickened epidermis
surrounded by acanthotic squamous epithelium. A sharp transition Introduction
from the thickened involved area to normal adjacent epidermis is Melanoma rarely develops on the eyelids but is potentially lethal.
referred to as shoulder formation (Fig. 2.18A) and a keratin-filled Although pigmentation is a hallmark of skin melanomas, half of
crater may be seen.! lid melanomas are non-pigmented and this may give rise to diag-
nostic difficulty. Features suggestive of melanoma include recent
Diagnosis onset of a pigmented lesion, change in an existing pigmented
A pink dome-shaped hyperkeratotic lesion develops, o%en on the lesion, irregular margins, asymmetrical shape, colour change or
lower lid (Fig. 2.18B) and may double or treble in size within weeks presence of multiple colours and diameter greater than 6 mm.!
(Fig. 2.18C). Growth then ceases for 2–3 months, a%er which
spontaneous involution occurs, when a keratin-filled crater may Lentigo maligna
develop (Fig. 2.18D). Complete involution may take up to a year Lentigo maligna (melanoma in situ, intraepidermal melanoma,
and usually leaves an unsightly scar.! Hutchinson freckle) is an uncommon condition that develops
in sun-damaged skin in elderly individuals (Fig. 2.20A). Malig-
Treatment nant melanoma arises within lentigo maligna in 3–10% of cases.
Treatment involves complete surgical excision with a margin of at Histopathology shows intraepidermal proliferation of spindle-
least 3 mm or utilizing Mohs surgery. Radiotherapy, cryotherapy shaped atypical melanocytes replacing the basal layer of the epi-
or local chemotherapy are sometimes used.! dermis. Clinically lentigo maligna presents as a slowly expanding

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 2
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Eyelids 55

A B

C D

Fig. 2.18 Keratoacanthoma. (A) Histopathology showing irregularly thickened eosinophilic

epidermis with a keratin-containing cup; (B) small nodule on lid margin; (C) hyperkeratotic
nodule; (D) keratin-filled crater during involution.

A B

Fig. 2.19 Sebaceous gland carcinoma. (A) Nodular tumour; (B) spreading tumour.

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56 Malignant Tumours

A B

C D

Fig. 2.20 Melanoma. (A) Clinical appearance of early lentigo maligna; (B) melanoma (arrow)
arising from lentigo maligna; (C) spreading melanoma onto tarsal conjunctiva; (D) histopathol-
ogy showing melanoma cells (arrows) within the dermis. Note the large nucleus with irregular
nuclear border and large, purple nucleoli. (Courtesy of ADN Murray – fig. C.)

pigmented macule with an irregular border and variable pigmen- approaches using immunotherapy (ipilimumab, nivolumab, pem-
tation. Nodular thickening and areas of irregular pigmentation are brolizumab, talimogene laherparepvec) are showing considerable
highly suggestive of malignant transformation (Fig. 2.20B).Treat- promise. Targeted treatment for advanced melanoma exhibiting
ment is usually by complete excision with large margins. Imiqui- the BRAF gene is also available (verumurafenib, dadrafenib, tra-
mod 5% topical cream is a reasonable alternative in those with metinib). Research is under way to produce a vaccine for cutane-
extensive involvement or in those who decline surgery.! ous melanoma.!

Melanoma
Superficial spreading melanoma is characterized by a plaque
Merkel cell carcinoma
with an irregular outline and variable pigmentation (Fig. 2.20C). Merkel cells are a form of sensory receptor concerned with light
Nodular melanoma is typically a blue–black nodule surrounded touch. Merkel cell carcinoma is a rapidly growing, highly malig-
by normal skin. Histopathology shows large atypical epithelioid nant tumour that typically affects older adults. Its rarity may lead
melanocytes scattered throughout the epidermis and invading the to difficulty in diagnosis and delay in treatment and 50% of patients
dermis (Fig. 2.20D). Treatment is usually by wide excision and have metastatic spread by presentation. Factors involved in the
may include local lymph node removal. Early detection carries a development include exposure to the Merkel cell polyomavirus,
good prognosis and surgical excision is o%en curative, but long- a weakened immune system and sun exposure. Histopathology
term survival for patients with metastatic disease is poor. Radio- shows a sheet of Merkel cells. A violaceous, well-demarcated nod-
therapy and chemotherapy are of limited efficacy. However, new ule with intact overlying skin is seen, most frequently involving

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 2
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Eyelids 57

A B
Fig. 2.21 Rare malignant tumours. (A) Merkel cell carcinoma; (B) Kaposi sarcoma.

the upper eyelid (Fig. 2.21A). Treatment is by excision, o%en with undertaking any reconstruction. There are several options for the
adjuvant therapy.! coordination of histopathological diagnosis and tumour clear-
ance with excision.
zzConventional paraffin-embedded specimen. Rapid process-
Kaposi sarcoma ing can reduce the interval to confirmation of histological
Kaposi sarcoma is a vascular tumour that typically affects indi- clearance but still requires that reconstruction be performed
viduals with AIDS. Many patients have advanced systemic disease as a separate procedure. Faster confirmation can be achieved
although in some instances the tumour may be the only clinical using either frozen-section control or micrographic surgery
manifestation of human immunodeficiency virus (HIV) infection. (see next) and reconstruction can then take place on the same
Histopathology shows proliferating spindle cells, vascular chan- day.
nels and inflammatory cells within the dermis. Clinically a pink, zzStandard frozen section involves histological examination
red-violet to brown lesion (Fig. 2.21B) develops, which may be of the margins of the excised specimen at the time of surgery
mistaken for a haematoma or naevus. Treatment is by radiotherapy to ensure that they are tumour-free. If no tumour cells are
or excision and by optimal control of AIDS.! detected, the eyelid is reconstructed on the same day. However,
if residual tumour is present, further excision is performed at
the appropriate edge of the surgical site until no tumour is
Treatment of malignant tumours detected.
zzMohs micrographic surgery involves layered excision of the
Biopsy tumour; specimens are usually examined frozen. Processing
Biopsy can be either incisional, using a blade or a biopsy punch, in of each layer enables a map of the edges of the tumour to be
which only part of the lesion is removed for histological diagnosis, developed. Further tissue is taken in any area where tumour
or excisional, in which the entire lesion is removed. The latter may is still present until clearance is achieved. Although time-
consist of shave excision using a blade to remove shallow epithelial consuming, this technique maximizes the chances of total
tumours, such as a papilloma and seborrhoeic keratosis, or full- tumour excision whilst minimizing sacrifice of normal tissue.
thickness skin excision for tumours that are not confined to the This is a particularly useful technique for tumours that grow
epidermis.! diffusely and have indefinite margins with finger-like exten-
sions, such as sclerosing BCC, SCC, recurrent tumours and
Surgical excision those involving the medial or lateral canthi. The irregular con-
Surgical excision aims to remove the entire tumour with preser- tours around the eyelids and extension of tumours into orbital
vation of as much normal tissue as possible. Smaller tumours can fat can make interpretation difficult.!
be removed via an excision biopsy and the defect closed directly,
whilst awaiting histological confirmation of complete clear- Reconstruction
ance. Most small BCCs can be cured by excision of the tumour The technique of reconstruction depends on the extent of tissue
together with a 2–4 mm margin of clinically normal tissue. More removed. It is important to reconstruct both anterior and posterior
radical surgical excision is required for a large BCC and aggres- lamellae, each of which must be reconstructed with similar tissue.
sive tumours such as SCC, SGC and melanoma. It may not be Anterior lamellar defects may be closed directly or with a local flap
possible to close all defects at the time of initial removal, but or skin gra%. Options for the repair of full-thickness defects are set
it is necessary to ensure complete clearance of tumour prior to out below.

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58 Malignant Tumours

zzSmall defects involving less than one-third of the eyelid can lamella requires its own blood supply to maximize the via-
usually be closed directly, provided the surrounding tissue is bility of a free gra% component.!
sufficiently elastic to allow approximation of the cut edges (Fig.
2.22A–D). If necessary, a lateral cantholysis can be performed Laissez-faire
for increased mobilization. Full reconstruction of the defect created by tumour removal may
zzModerate size defects involving up to half of the eyelid may not always be required. In the laissez-faire approach the wound
require a flap (e.g. Tenzel semi-circular) for closure (Fig. edges are approximated as far as possible and the defect is allowed
2.23A–D). to granulate and heal by secondary intention. Even large defects
zzLarge defects involving over half of the eyelid may be closed can o%en achieve a satisfactory outcome with time.!
by one of the following techniques:
{z Posterior lamellar reconstruction may involve an upper lid Radiotherapy
free tarsal gra%, buccal mucous membrane or hard palate The recurrence rate following irradiation alone is higher than a%er
gra%, or a Hughes tarsoconjunctival flap from the upper surgery, and radiotherapy does not allow histological confirmation
lid, which is le% attached for 4–6 weeks before transection of tumour eradication. Recurrences following radiotherapy are dif-
(Fig. 2.24A–F). ficult to treat surgically because of the poor healing properties of
{z Anterior lamellar reconstruction may involve skin irradiated tissue.
advancement, a local skin flap or a free skin gra% (Fig. zzIndications
2.24D). The patient must be made aware that gra%ed skin {z Patients who are either unsuitable for, or refuse, surgery.
is unlikely to be a perfect match. At least one reconstructed {z Highly radiosensitive tumours, such as Kaposi sarcoma.

A B

C D

Fig. 2.22 Direct closure. (A) Preoperative appearance of a basal cell carcinoma, showing lid
marking; (B) first cut at right-angles to lid margin; (C) excision of pentagon and first suture at
posterior lid margin; (D) tarsal plate closure with lid margin suture in situ. (Courtesy of AG
Tyers and JRO Collin, from Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann
2001.)

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 2
CHAPTER
Eyelids 59

{z Adjunctive therapy in some cases. Cryotherapy

{z Palliative treatment. Cryotherapy may be considered for a small superficial BCC and can
zzRelative contraindications be a useful adjunct to surgery in some patients. Complications include
{z Medial canthal lesions due to the high probability of lacri- skin depigmentation, madarosis and conjunctival overgrowth.!
mal canalicular damage.
{z Upper eyelid tumours – conjunctival keratinization is Immunotherapy
common and difficult to manage. Vismodegib (administered orally) has been approved for use in
{z Aggressive tumours such as SGC are relatively radioresis- patients with extensively advanced BCC. Overall response rates
tant, but higher-dose treatment may be effective. in up to two-thirds of patients have been reported. The initial
zzComplications. Many of these can be minimized by appropri- response to treatment can predict a complete response outcome.
ate shielding. However, systemic side effects are common and are the main rea-
{z Skin damage and madarosis (eyelash loss). son for treatment failure.!
{z Nasolacrimal duct stenosis following irradiation to the
medial canthal area. Oral nicotinamide for non-melanoma skin
{z Conjunctival keratinization, dry eye, keratopathy and cancers
cataract. Nicotinamide in a dose of 500 mg once daily appears to reduce the
{z Retinopathy and optic neuropathy.! incidence of skin malignancies. Because of its favourable safety

A B

C D

Fig. 2.23 Tenzel flap. (A) Incision marks and excision of tumour; (B) flap reflected deep to
orbicularis muscle (arrowheads) and cut lower limb of lateral canthal tendon (arrow); (C)
flap edge fixed to the opposite limb of the lateral canthal tendon; eyelid defect and flap
closed; (D) 6 months after surgery. (Courtesy of AG Tyers and JRO Collin, from Colour Atlas of
Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001.)

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60 Malignant Tumours

A B

C D

E F

Fig. 2.24 Posterior lamellar reconstruction with a Hughes upper lid flap. (A) Lower lid defect;
(B) tarsoconjunctival flap cut and turned into the defect; Müller muscle separated from the
superior tarsal border; (C) tarsoconjunctival flap sutured into the defect; (D) anterior lamellar
reconstruction with full-thickness skin graft from upper lid; (E) division of conjunctival pedicle;
(F) 3 months after surgery. (Courtesy of AG Tyers and JRO Collin, from Colour Atlas of
Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001.)

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 2
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Eyelids 61

profile regular supplementary nicotinamide should be considered zzThe meibomian gland orifices are located just anterior to the
in individuals who are at high risk of further skin tumours.! mucocutaneous junction. The edge of the tarsal plate is deep to
the gland orifices; the glands themselves run vertically within
the plate.
DISORDERS OF THE EYELASHES zzThe mucocutaneous junction is where keratinized epithelium

Misdirected lashes of the skin merges with conjunctival mucous membrane.

zzConjunctiva lines the posterior margin of the lid.!
Introduction
The roots of the eyelashes (cilia) lie against the anterior surface Clinical features
of the tarsal plate. The cilia pass between the main part of the Trauma to the corneal epithelium may cause punctate epithelial
orbicularis oculi and its more superficial part (Riolan muscle), erosions, with ocular irritation o%en worsened by blinking. Cor-
exiting the skin at the anterior lid margin and curving away from neal ulceration and pannus formation may occur in severe cases.
the globe. It is particularly important to be familiar with the nor- The clinical appearance varies with the cause.
mal anatomical appearance of the lid margin in order to be able zzTrichiasis refers to misdirection of growth from individual
to identify the cause of eyelash misdirection. From anterior to follicles (Fig. 2.25A and B), rather than a more extensive
posterior: inversion of the lid or lid margin. The follicles are at anatomi-
zzEyelashes (cilia). cally normal sites. It is commonly due to inflammation such
zzThe grey line, by definition the border between the anterior as chronic blepharitis or herpes zoster ophthalmicus, but can
(lashes, skin and orbicularis) and posterior (tarsal plate and also be caused by an injury or by surgery, such as incision and
conjunctiva) lamellae. curettage of a chalazion (Fig. 2.25C).

A B

C D
Fig. 2.25 Misdirected lashes. (A) Single trichiatic lash; (B) ingrown lash; (C) group of mis-
directed lashes secondary to chronic lid inflammation; (D) acquired distichiasis. (Courtesy
of R Bates – fig. D.)

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62 Disorders of the Eyelashes

z zMarginal entropion has increasingly been recognized as

a very common cause of eyelash misdirection, second-
ary to subtle cicatricial posterior lamellar shortening
that rotates a segment of the lid margin towards the eye.
The mucocutaneous junction migrates anteriorly and the
posterior lid margin becomes rounded rather than physi-
ologically square. Typically, numerous aligned lashes are
involved.
zzCongenital distichiasis is a rare condition that occurs
when a primary epithelial germ cell destined to differ-
entiate into a meibomian gland develops instead into a
complete pilosebaceous unit. The condition is frequently
inherited in an AD manner with high penetrance but vari-
able expressivity. The majority of patients also manifest
primary lymphoedema of the legs (lymphoedema–disti-
chiasis syndrome). A partial or complete second row of
lashes is seen to emerge at or slightly behind the meibo- Fig. 2.26 Appearance following laser ablation of multiple lashes.
mian gland orifices. The aberrant lashes tend to be thin-
ner and shorter than normal cilia and are often directed zzSurgery
posteriorly. They are usually well tolerated during infancy {z Tarsal fracture (transverse tarsotomy) is performed for
and may not become symptomatic until the age of about marginal entropion. A%er placing a 4-0 traction suture,
5 years. a horizontal incision is made through the tarsal plate via
zzAcquired distichiasis is caused by metaplasia of the meibo- the conjunctiva, at least halfway down the plate, along the
mian glands into hair follicles such that a variable number affected length of the lid and extended to 2–3 mm either
of lashes grow from meibomian gland openings. The most side of the involved region. Depending on the extent of lid
important cause is intense conjunctival inflammation (e.g. involvement, either two or three double-armed absorbable
chemical injury, Stevens–Johnson syndrome, ocular cicatricial sutures are passed through the upper edge of the lower sec-
pemphigoid). In contrast to congenital distichiasis, the cilia tion of the tarsal plate to emerge just anterior to the lashes,
tend to be non-pigmented and stunted (Fig. 2.25D) and are leaving the lid margin very slightly everted (Fig. 2.27A–
usually symptomatic. D). The sutures are le% in place following the surgery.
zzEpiblepharon – see later. Occasionally short-term use of a bandage contact lens is
zzEntropion. In contrast to marginal entropion, profound inver- required to prevent corneal abrading.
sion of a substantial width of the lid is readily identified – see {z A full-thickness eyelid pentagon resection can be used for
later.! a focal group of aberrant lashes, typically a%er trauma, or
for localized marginal entropion.
Treatment {z Other options include lid splitting (see next) with follicle
zzEpilation with forceps is simple and effective but recurrence excision and anterior lamellar rotation surgery.
within a few weeks is essentially invariable. It can be used as a zzCryotherapy applied externally to the skin just inferior to the
temporizing measure or in the occasional patient who refuses base of the abnormal lashes, or – especially in distichiasis – to
or cannot tolerate surgery. the internal aspect of the anterior lamella of the lid follow-
zzElectrolysis or electrocautery (hyfrecation) are broadly similar ing splitting of the margin at the grey line (Fig. 2.28A), can
electrosurgical techniques in which, under local anaesthesia, be used for numerous lashes. A%er separation of the anterior
a fine wire is passed down the hair follicle to ablate the lash. and posterior lamellae under local anaesthesia (Fig. 2.28B),
It is generally useful for a limited number of lashes, but scar- the cryoprobe is applied to the posterior lamella (Fig. 2.28C),
ring can follow. Frequently multiple treatments are required to using a double freeze–thaw cycle at −20 °C with a plastic eye
obtain a satisfactory result. protector in place. Full-thickness sutures hold the anterior
zzLaser ablation is also useful for the treatment of a limited lamella recessed on the posterior lamella (Fig. 2.28D). Sutur-
number of aberrant eyelashes and is performed using a spot ing of the lid margin is not usually necessary following lim-
size of 50 µm, duration of 0.1–0.2 s and power of 800–1000 ited splitting. The method is effective, but carries a high rate
mW. The base of the lash is targeted and shots are applied of local adverse effects, especially loss of lashes and entro-
to create a crater that follows the axis of the follicle (Fig. pion of the upper lid if there is inadequate recession of the
2.26). Success is broadly comparable to that achieved with anterior lamella. It is less commonly performed now than in
electrosurgery. previous years.!

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 2
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Eyelids 63

A B

C D

Fig. 2.27 Tarsal fracture for repair of mild entropion of lower lid. (A) Transverse, full-thickness
incision along the middle of the tarsal plate; (B) double-armed 4-0 sutures passed through
conjunctiva and lower lid retractors. Note the posterior surface of the pretarsal muscle
(arrow); (C) sutures are passed anterior to the marginal strip of tarsus, emerging just below
the lashes; (D) everting sutures tied with slight over-correction. (Courtesy of AG Tyers and
JRO Collin, from Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001.)

Eyelash ptosis Table 2.1 Causes of Trichomegaly

Drug-induced – topical prostaglandin analogues,
Eyelash ptosis refers to a downward sagging of the upper lid lashes phenytoin and ciclosporin
(Fig. 2.29A). The condition may be idiopathic or associated with Malnutrition
floppy eyelid syndrome, dermatochalasis with anterior lamellar AIDS
slip or long-standing facial palsy.! Porphyria
Hypothyroidism
Familial
Trichomegaly Congenital: Oliver–McFarlane, Cornelia de Lange,
Goldstein–Hutt, Hermansky–Pudlak syndromes
Trichomegaly is excessive eyelash growth (Fig. 2.29B). The main
causes are listed in Table 2.1.!
Poliosis
Madarosis Poliosis is a premature localized whitening of hair, which may
Madarosis is the term used for the loss of lashes (Fig. 2.29C). The involve the lashes and eyebrows (Fig. 2.29D). The main causes are
main causes are shown in Table 2.2.! shown in Table 2.3.!

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64 Idiopathic Dermatitis

A B

C D
Fig. 2.28 Cryotherapy to the eyelid in distichiasis. (A) Incision in the grey line; (B) separation of
the anterior and posterior lamellae; (C) application of cryoprobe to the posterior lamella; (D)
full-thickness sutures hold the anterior lamella recessed on the posterior lamella. (Courtesy
of AG Tyers and JRO Collin, from Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-
Heinemann 2001.)

scaling, angular fissuring, oedema and tightness (Fig. 2.30C).

ALLERGIC DISORDERS There may be chemosis, redness and papillary conjunctivitis. Cor-
neal involvement is usually limited to punctate epithelial erosions.
Acute allergic oedema Treatment consists primarily of avoidance of allergen exposure,
Acute allergic oedema may occur rapidly in a child exposed to cat provided it can be identified. Cold compresses provide symptom-
or dog hairs (Fig. 2.30A). When it is caused by exposure to pol- atic relief. Topical steroids and oral antihistamines can be used but
len or by insect bites it manifests with the sudden onset of bilat- are rarely required.!
eral boggy periocular oedema (Fig. 2.30B), o%en accompanied by
conjunctival swelling (chemosis – see Ch. 6). Treatment is o%en
unnecessary, but systemic antihistamines are sometimes given.!
IDIOPATHIC DERMATITIS
Atopic dermatitis
Contact dermatitis Atopic dermatitis (eczema) is a very common idiopathic condition,
Contact dermatitis is an inflammatory response that usually fol- typically occurring in patients who also suffer from asthma and hay
lows exposure to a medication such as eye drops, cosmetics or fever. Eyelid involvement is relatively infrequent but when present
metals. An irritant can also cause a non-allergic toxic dermati- is invariably associated with generalized dermatitis. Thickening,
tis. The individual is sensitized on first exposure and develops an crusting and fissuring of the lids (Fig. 2.30D) is typical and staphy-
immune reaction on further exposure; the mediating reaction is lococcal blepharitis, vernal or atopic keratoconjunctivitis are also
type IV (delayed type) hypersensitivity. Signs consist of lid skin commonly present. Herpetic blepharitis and keratoconjunctivitis

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 2
CHAPTER
Eyelids 65

A B

C D

Fig. 2.29 Miscellaneous eyelash disorders. (A) Eyelash ptosis; (B) trichomegaly; (C) madarosis;
(D) poliosis.

Table 2.2 Causes of Madarosis Table 2.3 Causes of Poliosis

1. Local 1. Ocular
Chronic anterior lid margin disease Chronic anterior blepharitis
Infiltrating lid tumours Sympathetic ophthalmitis
Burns Idiopathic uveitis
Radiotherapy or cryotherapy of lid tumours 2. Systemic
2. Skin disorders Vogt–Koyanagi–Harada syndrome
Generalized alopecia Waardenburg syndrome
Psoriasis Vitiligo
3. Systemic diseases Marfan syndrome
Myxoedema Tuberous sclerosis
Systemic lupus erythematosus Systemic monoclonal antibody treatment
Acquired syphilis
Lepromatous leprosy
4. Following removal
Procedures for trichiasis
Periorificial dermatitis
Trichotillomania – psychiatric disorder of hair removal This is a common chronic inflammatory skin eruption of
unknown cause that can affect the periocular region. It
are more common and more severe in patients with atopy (eczema resembles rosacea and allergic dermatitis, but is considered
herpeticum). Treatment of the lid features is with emollients to a disease of the hair follicle, with biopsy samples showing a
hydrate the skin and the judicious use of mild topical steroid such lymphohistiocytic infiltrate. Clusters of red papules, vesicles
as hydrocortisone 1%. Uncommon ocular associations include and pustules with an erythematous base and a sharp border
keratoconus, cataract and retinal detachment (see also Ch. 6).! are seen, with dry surrounding skin. It usually affects young

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66 Bacterial Infections

A
Fig. 2.31 Acute lid swelling secondary to dermatomyositis in
a child.

Systemic monoclonal antibody therapy

See Chapter 21.!
B
BACTERIAL INFECTIONS
External hordeolum
An external hordeolum (stye) is an acute staphylococcal abscess
of a lash follicle and its associated gland of Zeis that is common
in children and young adults. A stye presents as a tender swell-
C ing in the lid margin pointing anteriorly through the skin, usually
with a lash at its apex (Fig. 2.32A). Multiple lesions may be pres-
ent and occasionally an abscess may involve the entire lid margin.
Treatment involves topical (occasionally oral) antibiotics, hot com-
presses and epilation of the associated lash.!

Impetigo
D Impetigo is a superficial skin infection caused by Staphylococ-
cus aureus or Streptococcus pyogenes, typically affecting children.
Fig. 2.30 Allergic disorders. (A) Unilateral acute allergic oede- Involvement of the eyelids is usually associated with infection of
ma in a child; (B) bilateral acute allergic oedema; (C) contact the face. Painful erythematous macules rapidly develop into thin-
dermatitis; (D) atopic dermatitis in a young adult.
walled blisters, which develop golden-yellow crusts on rupturing
(Fig. 2.32B). There may be fever, malaise and local lymphade-
to middle-aged women. Exposure to sunlight, heat and wind nopathy. Treatment is with topical and sometimes oral antibiot-
can exacerbate the inflammation. The condition is benign and ics (beta-lactamase resistant) and preventative measures to reduce
self- limiting. If topical steroids have been used, they should be transmission as the condition is highly contagious. It is particularly
slowly discontinued and replaced with a bland emollient with- dangerous to neonates, contact with whom should be avoided.!
out preservatives. Specific treatment includes topical metroni-
dazole 0.75%, topical erythromycin 1% and oral tetracycline
for 4–8 weeks.!
Erysipelas
Erysipelas (St Anthony’s fire) is an uncommon acute, potentially
severe, dermal and superficial lymphatic infection usually caused
IMMUNE-RELATED INFLAMMATION by S. pyogenes. Diabetes, obesity and alcohol abuse are predis-
posing factors. An inflamed erythematous plaque develops (Fig.
Dermatomyositis 2.32C). A well-defined raised border distinguishes erysipelas from
Dermatomyositis is a chronic inflammatory disorder of muscles other forms of cellulitis. Complications such as metastatic infec-
which can present in an acute or chronic fashion with muscle tion are rare. Treatment is with oral antibiotics, but recurrence is
weakness and a skin rash. In children, about half develop eye- common.!
lid swelling and a heliotrope rash (Fig. 2.31). In adults it may
be associated with ovarian, breast or lung cancer, as an auto-
immune response. The condition is treated with systemic
Necrotizing fasciitis
steroids or immunosuppressive medication (methotrexate, Necrotizing fasciitis is a rare but commonly very severe infection
azathioprine).! involving subcutaneous so% tissue and the skin, with associated

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 2
CHAPTER
Eyelids 67

A B

C D

Fig. 2.32 Bacterial infections. (A) External hordeolum (stye); (B) impetigo; (C) erysipelas;
(D) necrotizing fasciitis.

rapidly progressive necrosis. It is usually caused by S. pyogenes intracytoplasmic (Henderson–Patterson) inclusion bodies that
and occasionally S. aureus. The most frequent sites of involvement displace the nuclear remnant to the edge of the cell. The bodies are
are the extremities, trunk and perineum, as well as postoperative small and eosinophilic near the surface and large and basophilic
wound sites. Unless early aggressive treatment is instituted, in the deeper down (Fig. 2.33A).!
form of surgical debridement and high-dose intravenous antibi-
otics, death may result. Redness and oedema are followed by the Diagnosis
formation of large bullae and black discoloration of the skin due to Single or multiple pale, waxy, umbilicated nodules develop (Fig.
necrosis (Fig. 2.32D).! 2.33B). White cheesy material consisting of infected degenerate
cells can be expressed from the lesion. Lesions on the lid margin
(Fig. 2.33C) may shed virus into the tear film and give rise to a sec-
VIRAL INFECTIONS ondary ipsilateral chronic follicular conjunctivitis. Unless the lid
Molluscum contagiosum margin is examined carefully the causative molluscum lesion may
be overlooked. Staphylococcal nodules of the lid may look similar,
Introduction but are whiter and not umbilicated (Fig. 2.33D).!
Molluscum contagiosum is a skin infection caused by a human-
specific double-stranded DNA poxvirus that typically affects Treatment
otherwise healthy children, with a peak incidence between 2 Spontaneous resolution will usually occur within a few months,
and 4 years of age. Transmission is by contact and subsequently so treatment may not be necessary, particularly in children, unless
by autoinoculation. Multiple and occasionally confluent lesions complications such as a significant secondary conjunctivitis are
may develop in immunocompromised patients. Histopathology problematic. Options include shave excision, curettage, cauteriza-
shows a central pit and lobules of hyperplastic epidermis with tion, chemical ablation, cryotherapy and pulsed-dye laser.

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68 Viral Infections

A B

C D

E F

Fig. 2.33 Viral infections. (A) Histopathology of molluscum contagiosum, showing lobules of
hyperplastic epidermis and a pit containing intracytoplasmic inclusion bodies; (B) multiple
molluscum nodules; (C) lid margin nodule; (D) should be differentiated from Staphylococcus
pustules; (E) Mpox (monkey pox) lesion on lid margin with follicular conjunctivitis and lid swell-
ing; (F) Mpox skin pustules. (Courtesy of RSR Naik – fig. D; G Kontos – figs E and F.)

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Eyelids 69

zzThe illness is usually mild and most will recover within a few
TIP In a patient with chronic follicular conjunctivitis that
weeks without treatment. There is no cure, but the antiviral
does not respond to topical antibiotics, look for molluscum
drugs cidofovir and tecovirimat and vaccinia immune globulin
contagiosum involving the lid margin.
! may be used in severe cases. The smallpox vaccine is 85% pro-
tective in preventing infection in close contacts.!
Mpox (Monkey pox)
Mpox is caused by a zoonotic virus in the genus Orthopoxvirus. It
Herpes zoster ophthalmicus
was once considered rare in humans, but the quantity and sever- Herpes zoster ophthalmicus (HZO – Fig. 2.34A and B) is a com-
ity of outbreaks is increasing, possibly as a consequence of waning mon, generally unilateral infection caused by varicella-zoster
immunity since the stopping of routine smallpox vaccination. In virus. It is discussed in detail in Chapter 7.!
July 2022 the World Health Organization declared the outbreak a
Public Health Emergency of International Concern.
zzIt is spread from animals by handling infected meat and from
Herpes simplex
human-to-human through exposure to contaminated body
fluids and intimate contact with the pox lesions. Sexual trans- Introduction
mission occurs predominantly in a man who has sex with a Herpes simplex skin rash results from either primary infection or
man who carries the virus. The virus is spread from the onset reactivation of herpes simplex virus previously dormant in the tri-
of symptoms until all the lesions have scabbed and fallen off. geminal ganglion. Prodromal facial and lid tingling lasting about
zzEarly symptoms include headache, fever and fatigue. This is 24 hours is followed by the development of eyelid and periocular
followed a few days later by the development of vesicles on the skin vesicles (Fig. 2.35A) that break down over 48 hours. Although
face and then elsewhere on the body, particularly on the palms typically confined to a single dermatome and with individual
of the hand and soles of the feet. lesions that are o%en similar in appearance, the distribution of
zzApproximately one in five cases have ocular lesions. Pustules the herpes simplex skin rash contrasts with the sharply delineated
on the lid margin can result in follicular conjunctivitis, sec- unilateral involvement in HZO (see Fig. 2.34). There is commonly
ondary lid swelling (Fig. 2.33E) and preauricular lymph gland an associated papillary conjunctivitis, discharge and lid swelling.
enlargement. In those with conjunctivitis there is a higher Dendritic corneal ulcers can develop, especially in atopic patients,
frequency of other systemic symptoms, compared to those in whom skin involvement can be extensive and very severe
without conjunctivitis. Corneal involvement in the form of (eczema herpeticum – Fig. 2.35B).!
keratitis may range from mild to severe. Loss of vision has
been reported in about 5%. Treatment
zzThe lesions on the body resemble chickenpox (Fig. 2.33F), In many patients the inflammation will gradually settle without
except that swollen glands are found. They start as small spots treatment over about a week. If treatment is necessary, a topical
which become bumps that fill with clear fluid; then umbili- (aciclovir cream five times daily for 5 days) or oral (oral aciclovir,
cated pustules which subsequently burst and scab. famciclovir or valaciclovir) antiviral agent can be used. Antibiotics

A B

Fig. 2.34 Herpes zoster ophthalmicus. (A) Healing rash in a child; (B) left maculopapular
crusting rash with periocular oedema; the right side shows passive swelling. (Courtesy
T Carmichael – fig. B.)

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70 Blepharitis

A B

Fig. 2.35 Herpes simplex. (A) Vesicles; (B) eczema herpeticum.

(e.g. co-amoxiclav, erythromycin) may also be required in patients thought of as a more persistent and chronic inflammatory
with secondary bacterial infection. This is particularly common in condition than anterior blepharitis; there is an association
eczema herpeticum.! with acne rosacea.
zzDemodex. A reaction to the extremely common hair follicle
and sebaceous gland-dwelling mite Demodex and other micro-
BLEPHARITIS organisms may play a causative role in some patients – Demo-
Chronic blepharitis dex folliculorum longus in anterior blepharitis and Demodex
folliculorum brevis in posterior blepharitis – though the mite
Introduction can be found normally in a majority of older patients, most
Chronic blepharitis (chronic marginal blepharitis) is a common of whom do not develop symptomatic blepharitis. Collarettes,
cause of ocular discomfort and irritation. The poor correlation a combination of partially digested epithelial cells, keratin,
between symptoms and signs, the uncertain aetiology and mecha- waste and mite eggs, are considered a pathognomonic sign of
nisms of the disease process all combine to make management dif- Demodex infection. It has been proposed that circumstances
ficult. Blepharitis may be subdivided into anterior and posterior such as overpopulation or hypersensitivity (perhaps to a bacil-
forms, although there is considerable overlap and both types are lus carried symbiotically by Demodex) may lead to symptoms.
o%en present (mixed blepharitis). Demodex mites are a major cause of the animal disease mange.
zzAnterior blepharitis affects the area surrounding the bases of The characteristics of the different forms of blepharitis are set
the eyelashes and may be staphylococcal or seborrhoeic. It is out in Table 2.4.!
sometimes regarded as related more to chronic infective ele-
ments and hence more amenable to treatment and remission Diagnosis
than the posterior form. An aetiological factor in staphylococ- Involvement is usually bilateral and symmetrical, with no visual
cal blepharitis may be an abnormal cell-mediated response to disturbance.
components of the cell wall of S. aureus, which may also be zzSymptoms are caused by disruption of normal ocular surface
responsible for the red eyes and peripheral corneal infiltrates function and reduction in tear stability and are similar in all
seen in some patients. It is more common and more marked forms of blepharitis, though stinging may be more common
in patients with atopic dermatitis. Seborrhoeic blepharitis is in posterior disease. Because of poor correlation between the
strongly associated with generalized seborrhoeic dermatitis severity of symptoms and signs it can be difficult to objectively
that characteristically involves the scalp, nasolabial folds, skin assess the benefit of treatment. Burning, grittiness, mild pho-
behind the ears and the sternum. tophobia and crusting and redness of the lid margins with
zzPosterior blepharitis is caused by meibomian gland dys- remissions and exacerbations are characteristic. Symptoms are
function and alterations in meibomian gland secretions. usually worse in the mornings although in patients with asso-
Bacterial lipases may result in the formation of free fatty ciated dry eye they may increase during the day. Contact lenses
acids. This increases the melting point of the meibum, may be poorly tolerated.
preventing its expression from the glands, contributing to zzSigns – staphylococcal blepharitis
ocular surface irritation and possibly enabling growth of S. {z Hard scales and crusting are found, mainly located around
aureus. Loss of the tear film phospholipids that act as sur- the bases of the lashes and are associated with collarettes,
factants results in increased tear evaporation and osmolarity which are cylindrical collections around lash bases (Fig.
and an unstable tear film. Posterior blepharitis is commonly 2.36A).

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Eyelids 71

Table 2.4 Summary of Characteristics of Chronic Blepharitis

Feature Anterior blepharitis Posterior blepharitis
Staphylococcal Seborrhoeic
Lashes Deposit Hard Soft
Loss ++ +
Distorted or trichiasis ++ +
Lid margin Ulceration +
Notching + ++
Cyst Hordeolum ++
Meibomian ++
Conjunctiva Phlyctenule +
Tear film Foaming ++
Dry eye + + ++
Cornea Punctate erosions + + ++
Vascularization + + ++
Infiltrates + + ++
Commonly associated Atopic dermatitis Seborrhoeic dermatitis Acne rosacea
skin disease

A B

Fig. 2.36 Chronic anterior blepharitis. (A) Scales and crusting including collarettes; (B) greasy
lid margin with sticky lashes in seborrhoeic blepharitis.

{z Mild papillary conjunctivitis and chronic conjunctival {z Pouting, recession, or plugging of meibomian gland ori-
hyperaemia are common. fices (Fig. 2.37B).
{z Long-standing cases may develop scarring and notching of {z Hyperaemia and telangiectasis of the posterior lid margin.
the lid margin, madarosis, trichiasis and poliosis. {z Pressure on the lid margin results in expression of mei-
{z Associated tear film instability and dry eye syndrome are bomian fluid that may be turbid or toothpaste-like (Fig.
common. 2.37C). In severe cases the secretions become so inspis-
{z Atopic keratoconjunctivitis may be present in patients sated that expression is impossible.
with atopic dermatitis. {z Lid transillumination may show gland loss and cystic dila-
zzSigns – seborrhoeic blepharitis tation of meibomian ducts.
{z Hyperaemic and greasy anterior lid margins with so% {z The tear film is oily and foamy and o%en unstable, and
scales and adherence of lashes to each other (Fig. 2.36B). froth may accumulate on the lid margins (Fig. 2.37D) or
zzSigns – posterior blepharitis (meibomian gland disease) inner canthi.
{z Excessive and abnormal meibomian gland secretion, man- zzDemodex infestation may lead to cylindrical dandruff-like
ifesting as capping of meibomian gland orifices with oil scaling (collarettes) around the base of eyelashes, though
globules (Fig. 2.37A). this is not always present (see Fig. 2.36A). The mites can be

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B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
72 Blepharitis

A B

C D

Fig. 2.37 Chronic posterior blepharitis. (A) Capping of meibomian gland orifices by oil glob-
ules; (B) plugged meibomian gland orifices; (C) expressed toothpaste-like material; (D) froth
on the eyelid margin.

demonstrated under (16 slit lamp magnification by first man- types, particularly as they commonly co-exist, but some treat-
ually clearing around the base of an eyelash and then by gently ments are fairly specific for one or the other.
rotating the lash or moving it from side to side for 5–10 sec- zzLid hygiene can be carried out once or twice daily initially,
onds with fine forceps. If one or more mites (0.2–0.4 mm long) although compliance and technique are highly variable.
do not emerge, the lash should be gently epilated. Slide micros- {z A warm compress should first be applied for several min-
copy can be performed on the mites or lashes if necessary. utes to so%en crusts at the bases of the lashes.
zzSecondary changes include papillary conjunctivitis, inferior {z Lid cleaning is subsequently performed to mechanically
corneal punctate epithelial erosions, corneal scarring and remove crusts and other debris, scrubbing the lid margins
vascularization including Salzmann nodular degeneration with a wet cotton bud or clean, wet face-cloth. Contrary
and advancing wave-like epitheliopathy-type changes, stye to popular belief, baby shampoo should not be recom-
formation, marginal keratitis and occasionally bacterial kera- mended, as it destabilizes the tear film and is a common
titis (especially in contact lens wearers) and phlyctenular eye cause of contact dermatitis.
disease.! {z Commercially produced soap/alcohol impregnated pads
for lid scrubbing are available and are o%en highly effec-
Treatment tive, but care should be taken not to induce mechanical
There is limited evidence to support any particular treatment pro- irritation.
tocol for blepharitis. Patients should be advised that a permanent {z When substantial meibomian gland disease is present, the
cure is unlikely, but control of symptoms is usually possible. The regimen may include expression of accumulated meibum
treatment of anterior and posterior disease is similar for both by rolling the finger anteriorly over the margin.

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Eyelids 73

{z The putative action of lid hygiene against Demodex is via zzNovel therapies include topical ciclosporin, pulsed light
prevention of reproduction. application and purpose-designed devices to probe, heat and/
{z Lid hygiene can be performed less frequently as the condi- or express the meibomian glands (e.g. LipiFlow) in posterior
tion is brought under control. disease.
zzAntibiotics zzComplications are treated specifically.
{z Topical sodium fusidic acid, erythromycin, bacitracin,
azithromycin or chloramphenicol is used to treat active
folliculitis in anterior disease and is occasionally used for
TIP Demodex infection is characterized by itching and the
presence of collarettes, a combination of partially digested
an extended period. Following lid hygiene, the ointment
epithelial cells, keratin, waste and mite eggs. The infestation
should be rubbed onto the anterior lid margin with a cot-
ton bud or clean finger.
can be treated with accurately applied tea tree oil products.
!
{z Oral antibiotic regimens include doxycycline (50–100
mg twice daily for 1 week and then daily for 6–24 weeks), Phthiriasis palpebrarum
lymecycline (one 408 mg capsule daily for up to 3 months),
other tetracyclines, or azithromycin (500 mg daily for 3 The crab louse Phthirus pubis is adapted to living in pubic hair, but
days for three cycles at 1-week intervals). Antibiotics are is also commonly found in other hair-covered body areas such as
thought to reduce bacterial colonization and may also the chest, axillae and eyelids (phthiriasis palpebrarum). Symptoms
exert other effects such as a reduction in staphylococcal consist of chronic irritation and itching of the lids, but the lice are
lipase production with tetracyclines. Tetracyclines may be o%en an incidental discovery. Conjunctivitis is uncommon. The
more effective in the treatment of posterior disease and lice are readily visible anchored to lashes (Fig. 2.38A); lice have
azithromycin in anterior. Tetracyclines should not be used six legs rather than the eight possessed by ticks (see next). Ova
in children under the age of 12 years or in pregnant or and their empty shells appear as oval, brownish, opalescent pearls
breastfeeding women because they are deposited in grow- adherent to the base of the cilia (Fig. 2.38B). Treatment consists of
ing bone and teeth. Patients should also be made aware of mechanical removal of the lice and their attached lashes with fine
the possibility of increased sun sensitivity. Erythromycin forceps. If necessary, topical yellow mercuric oxide 1% or petro-
250 mg once or twice daily is an alternative. leum jelly can be applied to the lashes and lids twice a day for 10
zzPlant and fish oil supplements have been shown to be of sub- days. Delousing of the patient, family members, clothing and bed-
stantial benefit in some cases. ding is important to prevent recurrence.!
zzTopical steroid. A low potency preparation such as fluoro-
metholone 0.1% or loteprednol four times daily for 1 week is Tick infestation of the eyelid
useful in patients with substantial active inflammation, espe-
cially papillary conjunctivitis; occasionally a higher strength Ticks can attach themselves to the eyelid and should be removed at
preparation is used. the earliest opportunity in order to minimize the risk of contract-
zzTear substitutes and other dry eye treatments are typically ing a tick-borne zoonosis such as Lyme disease, Rocky Mountain
helpful for associated tear insufficiency and instability. fever, African tick bite fever (Fig. 2.39) or tularaemia. If the tick is
zzTea tree oil has been suggested as a treatment based primarily attached some distance from the eye such that spray can safely be
on its activity against Demodex infestation in vitro, although applied, an insect repellent containing pyrethrin or a pyrethroid
there remains some uncertainty to the effectiveness in vivo. The should be sprayed on the tick twice at intervals of a minute. Alter-
optimal vehicle and regimen have not been established, but natively, a scabies cream containing permethrin can be applied.
lid, eyebrow and periocular skin cleansing once daily with a These have a toxic effect that prevents the tick from injecting
50% scrub and application of 5% ointment has been described. saliva and a%er 24 hours it should drop off or can be removed with
Preservative-free, sterile, ready-to-use wipes containing 2.5% fine-tipped forceps at the slit lamp (blunt-tipped needle-holders
terpinen-4-ol are available and can be applied once or twice are an alternative in restrained small children). It is critical that
daily to the lid margins (depending on severity) for 4 weeks. the tick is detached as close to its skin attachment as possible in
People should be educated on how to accurately apply tea order to remove its head and mouthparts, following which it might
tree oil products, because adherence and method of applica- be retained in sealed packaging to permit identification if neces-
tion are likely to affect efficacy. Topical permethrin and topi- sary. In areas endemic for Lyme disease, some authorities sug-
cal (1% cream) or oral (two doses of 200 µg/kg 1 week apart) gest routine antibiotic prophylaxis with doxycycline following a
ivermectin have also been used by some practitioners. Early confirmed deer tick bite. Patients should be told to seek medical
pre-clinical results using lotilaner 0.25% ophthalmic solution advice urgently at the onset of suspicious symptoms, particularly
(TP-03) are encouraging, as it is well tolerated and effective erythema migrans, over the subsequent few weeks. Lyme dis-
in reducing collarettes and Demodex density within 4 weeks ease transmission is thought to require attachment of the tick for
of commencing treatment. High temperature cleaning of bed- at least 36 hours. African tick bite fever is a rickettsial infection,
ding, the use of tea tree shampoo and facial soap, and treating which is fairly common in sub-Saharan Africa. The onset of symp-
the patient’s partner may all help to reduce recurrences. toms occurs 4–10 days a%er the bite.!

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74 Blepharitis

A B

Fig. 2.38 Phthiriasis palpebrarum. (A) Louse anchored to lashes with ova; (B) close-up appear-
ance of a louse.

A B

Fig. 2.39 (A) African tick on lid margin; (B) 2 weeks after removal. (Courtesy of ADN Murray.)

Angular blepharitis
The infection is usually caused by Moraxella lacunata or S. aureus
although other bacteria and, rarely, herpes simplex have also been
implicated. Red, scaly, macerated and fissured skin is seen at the
lateral and/or medial canthi of one or both eyes (Fig. 2.40). Skin
chafing secondary to tear overflow, especially at the lateral can-
thus, can cause a similar clinical picture and may also predispose
to infection. Associated papillary and follicular conjunctivitis may
occur. Treatment involves topical chloramphenicol, bacitracin or
erythromycin.!

Childhood blepharokeratoconjunctivitis
Childhood blepharokeratoconjunctivitis is a poorly defined con-
dition that tends to be more severe in Asian and Middle Eastern
populations. Presentation is usually at about 6 years of age with
recurrent episodes of anterior or posterior blepharitis, sometimes Fig. 2.40 Angular blepharitis. (Courtesy of S Tuft.)
associated with recurrent styes or chalazia. Constant eye rubbing

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 2
CHAPTER
Eyelids 75

and photophobia may lead to misdiagnosis as allergic eye disease.

Conjunctival changes include diffuse hyperaemia, bulbar phlyc-
tens and follicular or papillary hyperplasia.
Corneal changes include superficial punctate keratopathy,
marginal keratitis, peripheral vascularization and axial subepithe-
lial haze. Treatment is with lid hygiene and topical antibiotic oint-
ment at bedtime. Topical low-dose steroids (prednisolone 0.1% or A
fluorometholone 0.1%) and erythromycin syrup 125 mg daily for
4–6 weeks may also be used.!

PTOSIS
Classification
Ptosis is an abnormally low position of the upper lid, which may be B
congenital or acquired.
zzNeurogenic ptosis is caused by an innervational defect such
as third nerve paresis and Horner syndrome (see Ch. 19) (Fig.
2.41A).
zzMyogenic ptosis is caused by a myopathy of the levator mus-
cle itself, or by impairment of transmission of impulses at the
neuromuscular junction (neuromyopathic). Acquired myo-
genic ptosis occurs in myasthenia gravis, myotonic dystrophy C
and progressive external ophthalmoplegia (see Ch. 19) (Fig.
2.41B).
zzAponeurotic or involutional ptosis is caused by a defect in the
levator aponeurosis (Fig. 2.41C).
zzMechanical ptosis is caused by the gravitational effect of a
mass or by scarring (Fig. 2.41D).!

Clinical evaluation D

Fig. 2.41 Causes of ptosis. (A) Neurogenic (third nerve pal-

General sy); (B) myogenic (myasthenia gravis); (C) aponeurotic (age-
related); (D) mechanical (neurofibroma).
The age at onset of ptosis and the duration will usually distinguish
congenital from acquired cases. If the history is ambiguous, old
photographs may be helpful. It is also important to enquire about zzDermatochalasis. Overhanging skin on the upper lids
symptoms of possible underlying systemic disease, such as asso- (Fig. 2.42D) may be mistaken for ptosis, but may also cause
ciated diplopia, variability of ptosis during the day and excessive mechanical ptosis.!
fatigue.!
Measurements
Pseudoptosis zzMargin–reflex distance is the distance between the upper lid
A false impression of ptosis may be caused by the following: margin and the corneal reflection of a pen torch held by the
zzLack of support of the lids by the globe may be due to an examiner on which the patient fixates (Fig. 2.43). The normal
orbital volume deficit associated with an artificial eye (Fig. measurement is 4–5 mm.
2.42A), microphthalmos, phthisis bulbi, or enophthalmos. zzPalpebral fissure height is the distance between the upper
zzContralateral lid retraction, which is detected by comparing and lower lid margins, measured in the pupillary plane (Fig.
the levels of the upper lids, remembering that the margin of 2.44). The upper lid margin normally rests about 2 mm below
the upper lid normally covers the superior 2 mm of the cornea the upper limbus and the lower 1 mm above the lower lim-
(Fig. 2.42B). bus. This measurement is shorter in males (7–10 mm) than
zzIpsilateral hypotropia causes pseudoptosis because the upper in females (8–12 mm). Unilateral ptosis can be quantified by
lid follows the globe downwards (Fig. 2.42C). It disappears comparison with the contralateral side. Ptosis may be graded
when the hypotropic eye assumes fixation on covering the as mild (up to 2 mm), moderate (3 mm) and severe (4 mm
normal eye. or more).
zzBrow ptosis due to excessive skin on the brow, or seventh nerve zzLevator function (upper lid excursion) is measured by
palsy, which is diagnosed by manually elevating the eyebrow. placing a thumb firmly against the patient’s brow to negate

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76 Ptosis

MRD

A A

B
B

C
C

D Fig. 2.43 Margin–reflex distance (MRD). (A) Normal; (B) mild

ptosis; (C) moderate ptosis; (D) severe ptosis.
Fig. 2.42 Causes of pseudoptosis. (A) Left ill-fitting artificial
eye; (B) asymmetrical lid position with contralateral lid retrac-
tion; (C) ipsilateral hypotropia which disappears on covering
the normal left eye; (D) bilateral brow ptosis with marked
dermatochalasis.

the action of the frontalis muscle, with the eyes in downgaze

(Fig. 2.45A). Alternatively, the brow can be lifted above the
orbit and held firmly against the forehead to reduce fron-
talis function. The patient then looks up as far as possible
and the amount of excursion is measured with a rule (Fig.
2.45B). Levator function is graded as normal (15 mm or
Fig. 2.44 Measurement of palpebral fissure height.
more), good (12–14 mm), fair (5–11 mm) and poor (4 mm
or less).
zzUpper lid crease is taken as the vertical distance between
the lid margin and the lid crease in downgaze. In females Associated signs
it measures about 10 mm and in males 8 mm. Absence of zzThe pupils should be examined to exclude Horner syndrome
the crease in a patient with congenital ptosis is evidence of and a subtle pupil-involving third nerve palsy – the latter is an
poor levator function, whereas a high crease suggests an unlikely acute clinical presentation (see Ch. 19).
aponeurotic defect (usually involutional). The skin crease is zzIncreased innervation may flow to the levator muscle of a
also used as a guide to the initial incision in some surgical unilateral ptosis, particularly in upgaze. Associated increased
procedures. innervation to the contralateral normal levator will result in lid
zzPretarsal show is the distance between the lid margin and the retraction. The examiner should therefore manually elevate the
skin fold with the eyes in the primary position.! lid and look for drooping of the opposite lid. If this occurs, the

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 2
CHAPTER
Eyelids 77

particularly following large levator resections or suspension

procedures.
zzThe tear film should be inspected – a poor volume or unsta-
ble film may be worsened by ptosis surgery and should be
addressed preoperatively as far as possible.!

Simple congenital ptosis

Diagnosis
Congenital ptosis probably results from a failure of neuronal
migration or development with muscular sequelae secondary to
A this. A minority of patients have a family history.
zzSigns
{z Unilateral or bilateral ptosis of variable severity (Fig. 2.46A
and B).
{z Absent upper lid crease and poor levator function (Fig.
2.46C–F).
{z In downgaze the ptotic lid is higher than the normal
because of poor relaxation of the levator muscle. This
is in contrast to acquired ptosis, in which the affected
lid is either level with or lower than the normal lid on
downgaze.
{z Following surgical correction, the lid lag in downgaze may
worsen.
zzAssociations
{z Superior rectus weakness may be present because of its
close embryological association with the levator.
{z Compensatory chin elevation in severe bilateral cases.
{z Refractive errors are common and more frequently respon-
B
sible for amblyopia than the ptosis itself.!
Fig. 2.45 Measurement of levator function. (A) Place a thumb
firmly against the brow to reduce the effect of the frontalis Treatment
muscle and ask the patient to look down; (B) the patient then
Treatment should be carried out during the preschool years once
looks up as far as possible and the amount of excursion is
measured with a ruler. accurate measurements can be obtained, but may be considered
earlier in severe cases to prevent amblyopia. Levator resection (see
below) is usually required.
patient should be warned that surgical correction may induce
a lower position in the opposite lid.
zzFatigability is tested by asking the patient to look up without TIP A weak Bell phenomenon can result in exposure
blinking for 30–60 seconds. Progressive drooping of one or
keratopathy after ptosis surgery.
!
both lids, or an inability to maintain upgaze, is suggestive of
myasthenia gravis (see Ch. 19). Myasthenic ptosis may show
an overshoot of the upper lid on saccade from downgaze to the
Marcus Gunn jaw-winking syndrome
primary position (Cogan twitch sign) and a ‘hop’ on side-gaze.
zzOcular motility defects, particularly of the superior rectus, Introduction
must be evaluated in patients with congenital ptosis. Correc- About 5% of all cases of congenital ptosis are associated with the
tion of an ipsilateral hypotropia may improve the degree of Marcus Gunn jaw-winking phenomenon. Most are unilateral.
ptosis. Deficits consistent with a subtle or partial third nerve Although the exact aetiology is unclear, it has been postulated that
paresis should be identified. a branch of the mandibular division of the fi%h cranial nerve is
zzJaw-winking can be identified by asking the patient to chew misdirected to the levator muscle.!
and move the jaws from side to side (see below).
zzBell phenomenon is tested by manually holding the lids open, Diagnosis
asking the patient to try to shut the eyes and observing upward zzSigns
and outward rotation of the globe. A weak Bell phenomenon {z Retraction of the ptotic lid in conjunction with stimu-
carries a variable risk of postoperative exposure keratopathy, lation of the ipsilateral pterygoid muscles by chewing,

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
78 Ptosis

A B

C D

E F

Fig. 2.46 Congenital ptosis. (A) Mild right ptosis; (B) good levator function; (C) severe left pto-
sis with absent skin crease; (D) poor levator function; (E) severe bilateral ptosis; (F) poor leva-
tor function.

A B

Fig. 2.47 Marcus Gunn jaw-winking syndrome. (A) Moderate left ptosis; (B) retraction of the lid
on opening the mouth.

sucking, opening the mouth (Fig. 2.47) or contralateral zzMild cases with reasonable levator function of 5 mm or better
jaw movement. and little synkinetic movement may be treated with unilateral
{z Less common stimuli to winking include jaw protrusion, levator advancement.
smiling, swallowing and clenching of teeth. zzModerate cases. Unilateral levator disinsertion can be per-
{z Jaw-winking does not improve with age, although patients formed to address the synkinetic winking component, with
may learn to mask it.! ipsilateral brow (frontalis) suspension so that lid elevation is
due solely to frontalis muscle elevation.
Treatment zzBilateral surgery. Bilateral levator disinsertion with bilateral
Surgery should be considered if jaw-winking or ptosis represents a brow suspension may be carried out to produce a symmetrical
significant functional or cosmetic problem. result.!

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 2
CHAPTER
Eyelids 79

Third nerve misdirection syndromes

Third nerve misdirection syndromes may be congenital, but more
frequently follow acquired third nerve palsy. Bizarre movements of
the upper lid accompany various eye movements (Fig. 2.48). Ptosis
may also occur following aberrant facial nerve regeneration. Treat-
ment is by levator disinsertion and brow suspension.! A

Involutional ptosis
Involutional (aponeurotic) ptosis is an age-related condition
caused by dehiscence, disinsertion or stretching of the levator apo-
neurosis, limiting the transmission of force from a normal levator
muscle to the upper lid. Due to fatigue of the Müller muscle, it
frequently worsens towards the end of the day, so that it can some- B
times be confused with ptosis secondary to myasthenia. There is a
variable, usually bilateral, ptosis with a high upper lid crease and Fig. 2.48 Third nerve redirection. (A) Moderate right ptosis;
(B) retraction of the right lid on right gaze. (Courtesy of A
good levator function. In severe cases the upper lid crease may be
Pearson.)
absent, the eyelid above the tarsal plate is very thin and the upper
sulcus deep (Fig. 2.49). Treatment options include levator resec-
tion, advancement with reinsertion or anterior levator repair.

TIP Involutional ptosis may be confused with ocular

myasthenia gravis because it frequently gets worse towards the
end of the day.
!

Mechanical ptosis Fig. 2.49 Severe bilateral involutional ptosis with absent skin
creases and deep sulci.
Mechanical ptosis is the result of impaired mobility of the upper
lid. It may be caused by dermatochalasis, large tumours such as
a neurofibroma, heavy scar tissue, severe oedema and anterior zzThe inferior tarsal muscle is analogous to Müller muscle.!
orbital lesions.!
Conjunctiva–Müller resection
This involves excision of Müller muscle and overlying conjunc-
Surgery tiva with reattachment of the resected edges (Fig. 2.52A–C). The
maximal elevation achievable is 2–3 mm, so it is used in cases of
Anatomy mild ptosis with good (at least 10 mm) levator function, which
zzThe levator aponeurosis fuses with the orbital septum about 4 includes most cases of Horner syndrome and mild congenital
mm above the superior border of the tarsal plate (Fig. 2.50A). ptosis.!
Its posterior fibres insert into the lower third of the anterior
surface of the tarsal plate. The medial and lateral horns are Levator advancement (resection)
expansions that act as check ligaments (Fig. 2.50B). Surgi- In this technique the levator complex is shortened through either
cally, the aponeurosis can be approached through the skin or an anterior – skin (Fig. 2.53A–C) – or posterior – conjuncti-
conjunctiva. val – approach. Indications include ptosis of any cause, provided
zzEyelids of Asians. The main difference in the upper lid appear- residual levator function is at least 5 mm. The extent of resection
ance is a low or absent skin crease in about 50% of Asians due is determined by the severity of the ptosis and the amount of leva-
to the low insertion of the levator aponeurosis, close to the tor function. The predictability of correcting height is the same
lashes. The septum also inserts lower on the aponeurosis. The whether an anterior or posterior approach is used. However, the
presence of a medial canthal fold can cause lash ptosis (Fig. advantage of a posterior approach is the predictability of the lid
2.51). contour.!
zzMüller muscle is inserted into the upper border of the tarsal
plate and can be approached transconjunctivally. Brow (frontalis) suspension
zzThe inferior tarsal aponeurosis consists of the capsulo- Brow (frontalis) suspension is used for severe ptosis (>4 mm) with
palpebral expansion of the inferior rectus muscle and is analo- very poor levator function (<4 mm) from a variety of causes. Typi-
gous to the levator aponeurosis. cal indications include ptosis associated with third nerve palsy,

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
80 Ectropion

Tendon of levator
palpebrae superioris muscle
Orbital septum
Orbital septum Anterior lacrimal crest

Levator
Levator muscle aponeurosis
Müller muscle

Superior tarsus

Lateral palpebral
Tarsal plate
ligament

Inferior tarsus
Inferior oblique Pretarsal orbicularis

Lower lid retractors

Orbital septum
Medial palpebral ligament
Preseptal orbicularis
A B
Fig. 2.50 Anatomy of the eyelid. (A) Cross-sectional diagram; (B) anterior view.

zz Horizontal lid laxity, demonstrated by pulling the central part of

the lid 8 mm or more from the globe, with a failure to snap back
to its normal position on release without the patient first blinking.
zzLateral canthal tendon laxity, characterized by a rounded
appearance of the lateral canthus and the ability to pull the
lower lid medially more than 2 mm.
zzMedial canthal tendon laxity, demonstrated by pulling the
lower lid laterally and observing the position of the inferior
Fig. 2.51 Appearance of eyelids in an Asian child. punctum. If the lid is normal the punctum should not be
displaced more than 1–2 mm. If laxity is mild the punctum
reaches the limbus and if severe it may reach the pupil.!
blepharophimosis syndrome and following an unsatisfactory result
from previous levator resection. The tarsal plate is suspended from Treatment
the frontalis muscle with a sling consisting of non-absorbable The approach to repair depends on apparent causation and the
material such as silicone or less commonly in recent years, autolo- predominant location of the ectropion.
gous fascia lata (Fig. 2.54A–C).! zzGeneralized ectropion is treated with repair of horizontal
lid laxity. This is achieved with a lateral tarsal strip procedure,
in which the lower canthal tendon is tightened by shortening
ECTROPION and reattachment to the lateral orbital rim (Fig. 2.56). This is
Involutional ectropion particularly helpful if the lateral canthus is rounded and lax,
with associated tear overflow. Excision of a tarsoconjunctival
Introduction pentagon is an alternative that can be placed to excise an area
Involutional (age-related) ectropion affects the lower lid of elderly of misdirected lashes or keratinized conjunctiva (Fig. 2.57).
individuals. It causes epiphora (tear overflow) and may exacerbate zzMedial ectropion, if mild, may be treated with a medial con-
ocular surface disease. The red appearance of the exposed conjunc- junctival diamond excision (medial spindle procedure). It is
tiva is cosmetically poor. In long-standing cases the tarsal conjunc- o%en combined with a tarsal strip or lateral canthal sling (see
tiva may become chronically inflamed, thickened and keratinized ‘Treatment’), or pentagon excision as significant horizontal
(Fig. 2.55A). Aetiological factors include: laxity frequently co-exists.

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 2
CHAPTER
Eyelids 81

A A

B B

C C

Fig. 2.52 Conjunctiva–Müller resection (Fasanella-Servat). Fig. 2.53 Anterior levator resection. (A) Skin incision; (B) dis-
(A) Forceps applied parallel to the superior border (arrow) of section and resection of levator aponeurosis; (C) levator
the tarsal plate. A 6-0 absorbable suture is inserted medi- reattachment to the tarsal plate. (Courtesy of AG Tyers and
ally and passed continuously through the full thickness of the JRO Collin, from Colour Atlas of Ophthalmic Plastic Surgery,
lid behind the forceps; (B) superior tarsal plate and smooth Butterworth-Heinemann 2001.)
muscle are excised; (C) the second arm of the suture runs
continuously over the cut edge. The two sutures are brought
out through a lateral skin incision and tied. (Courtesy of AG
Tyers and JRO Collin, from Colour Atlas of Ophthalmic Plastic
Surgery, Butterworth-Heinemann 2001.)

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
82 Ectropion

A A

B B

C C

Fig. 2.54 Brow suspension. (A) Site of incisions marked; (B) Fig. 2.55 Ectropion. (A) Severe long-standing involutional
threading of fascia lata strips; (C) tightening and tying of ectropion with keratinization of the marginal conjunctiva;
strips. (Courtesy of AG Tyers and JRO Collin, from Colour (B) cicatricial; (C) paralytic secondary to a facial nerve palsy.
Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann (Courtesy of C Barry – fig. A; A Pearson – fig. B; JH Norris –
2001.) fig. C.)

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 2
CHAPTER
Eyelids 83

A B

C D

E F

Fig. 2.56 Lateral tarsal strip procedure. (A) Lower limb of the lateral canthal tendon is iso-
lated; (B) the tendon is cut close to the orbital rim; (C) lid margin skin is excised, then skin
and orbicularis tissue are separated from the tarsal plate; (D) a lateral tarsal strip is creat-
ed; (E) this is sutured to periosteum anterior to the orbital rim; (F) a new canthus is formed
(arrow). (Courtesy of AG Tyers and RJO Collin, from Colour Atlas of Ophthalmic Plastic
Surgery, Butterworth-Heinemann 2001.)

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
84 Ectropion

zzMedial canthal tendon laxity, if marked, requires stabilization

prior to horizontal shortening to avoid excessive dragging of
the punctum laterally.
zzPunctal ectropion without more extensive lid involvement is
considered in Chapter 3.!

Cicatricial ectropion
Cicatricial ectropion is caused by scarring or contracture of the
skin and underlying tissues, which pulls the eyelid away from the
globe (see Fig. 2.55B). If the skin is pushed up over the orbital mar-
gin with a finger the ectropion will be relieved. Opening the mouth
tends to accentuate the eversion. Depending on the cause, both
lids may be involved, and the defect may be local (e.g. trauma) or
A general (e.g. burns, dermatitis, ichthyosis). Mild localized cases are
treated by excision of the offending scar tissue combined with a
procedure that lengthens vertical skin deficiency, such as Z-plasty.
Severe generalized cases require transposition flaps or free skin
gra%s. Sources of skin include the upper lids, posterior auricular,
preauricular and supraclavicular areas.!

Paralytic ectropion/facial nerve palsy

Introduction
Paralytic ectropion is caused by ipsilateral facial nerve palsy (see Fig.
2.55C) and is associated with retraction of the upper and lower lids and
brow ptosis. The latter may mimic narrowing of the palpebral aperture.
Complications include exposure keratopathy due to lagoph-
thalmos and watering caused by malposition of the inferior lac-
rimal punctum, failure of the lacrimal pump mechanism and an
B
increase in tear production resulting from corneal exposure.!

Treatment
zz Temporary measures may be instituted to protect the cornea in
anticipation of spontaneous recovery of facial nerve function.
{z Lubrication with high viscosity tear substitutes during the
day, with instillation of ointment and taping shut of the lids
during sleep, are usually adequate in mild cases.
{z Botulinum toxin injection into the levator to induce tem-
porary ptosis.
{z Temporary tarsorrhaphy may be necessary, particularly in
patients with a poor Bell phenomenon where the cornea
remains exposed when the patient attempts to blink. The lat-
eral aspects of the upper and lower lids are sutured together.
zzPermanent treatment should be considered when there is irre-
versible damage to the facial nerve which may occur follow-
C
ing removal of a vestibular schwannoma, or when no further
Fig. 2.57 Horizontal lid shortening to correct ectropion. (A) improvement has occurred for 6–12 months in a Bell palsy.
Marking; (B) excision of a pentagon; (C) closure. (Courtesy of {z Medial canthoplasty may be performed if the medial
AG Tyers and JRO Collin, from Colour Atlas of Ophthalmic canthal tendon is intact. The eyelids are sutured together
Plastic Surgery, Butterworth-Heinemann 2001.)
medial to the lacrimal puncta (Fig. 2.58A) so that the

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 2
CHAPTER
Eyelids 85

Fig. 2.58 (A) Medial canthoplasty as permanent treatment

of paralytic ectropion; (B) platinum weight used to correct B
lagophthalmos. (Courtesy JH Norris – fig. B.)
Fig. 2.59 (A) Involutional entropion and pseudotrichiasis;
(B) preseptal orbicularis over-riding the pretarsal orbicularis.

puncta become inverted and the fissure between the inner

canthus and puncta is shortened.
{z A lateral canthal sling or tarsal strip may be used to correct
residual ectropion and raise the lateral canthus.
ENTROPION
{z Upper eyelid lowering by levator disinsertion.
{z In patients with lagophthalmos either gold or platinum
Involutional entropion
weights can be implanted into the upper lid. Platinum Introduction
weights are preferred because they are less bulky than gold Involutional (age-related) entropion affects mainly the lower
weights, provide a better eyelid contour and are less likely lid. The constant rubbing of the lashes on the cornea in long-
to need long-term revisional surgery (Fig. 2.58B). standing entropion (pseudotrichiasis – Fig. 2.59A) may cause
{z A small lateral tarsorrhaphy is usually cosmetically irritation, corneal punctate epithelial erosions and, in severe
acceptable.! cases, pannus formation and ulceration. Aetiological factors
include:
zzHorizontal lid laxity caused by stretching of the canthal ten-
Mechanical ectropion dons and tarsal plate.
Mechanical ectropion is caused by tumours on or near the lid mar- zzVertical lid instability caused by attenuation, dehiscence or
gin that mechanically evert the lid. Treatment involves removal of disinsertion of the lower lid retractors. Weakness of the lat-
the cause if possible and correction of significant horizontal lid ter is recognized by decreased excursion of the lower lid in
laxity.! downgaze.

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
86 Entropion

zzOver- riding of the pretarsal by the preseptal orbicularis

during lid closure tends to move the lower border of the
tarsal plate anteriorly, away from the globe and the upper
border towards the globe, thus tipping the lid inwards
(Fig. 2.59B).
zzOrbital septum laxity with prolapse of orbital fat into the
lower lid.!

Treatment
Temporary protection must be as short-term as possible.
Options include lubricants, taping, soft bandage contact lenses
and orbicularis chemodenervation with botulinum toxin injec-
tion. Surgical treatment aims to correct the underlying prob-
lems as follows:
zzOver-riding and disinsertion
A
{z Transverse everting sutures prevent over-riding of the
preseptal orbicularis. They are quick and easy to insert
(Fig. 2.60A–C), providing a correction that typically lasts
several months and may be used in circumstances (e.g. a
confused patient) where a more intricate procedure is not
likely to be tolerated.
{z The Wies procedure gives a durable correction. It consists
of full-thickness horizontal lid splitting and insertion of
everting sutures (Fig. 2.61A–D). The scar creates a barrier
between the preseptal and pretarsal orbicularis and the
everting suture fairly effectively transfers the pull of the
lower lid retractors from the tarsal plate to the skin and
orbicularis.
{z Lower lid retractor reinsertion (Fig. 2.62A–D) involves
direct exposure and advancement of the retractors as
opposed to the less precise approach used in the Wies pro-
cedure. The subciliary skin incision used and its repair also B
create a barrier to over-riding of the preseptal orbicularis
muscle. It can be performed as a primary treatment but
may be reserved for recurrence.
zzHorizontal lid laxity is usually present and can be corrected
with a lateral canthal sling (tarsal strip –see Fig. 2.56) or, less
commonly, a full-thickness lateral pentagon excision (see Fig.
2.57). Tightening serves also to retain the lid in apposition
against the globe, preventing over-correction.!

Cicatricial entropion
Scarring of the palpebral conjunctiva can rotate the upper or lower
lid margin towards the globe. Causes include cicatrizing conjuncti-
vitis, trachoma, trauma and chemical injuries (see Figs 6.7F, 6.18C,
6.20D). Treatment of mild cases is by tarsal fracture (transverse
tarsotomy) with anterior rotation of the lid margin, as for marginal
entropion of the lower lid. Treatment of severe cases is difficult and
is directed at replacing deficient or keratinized conjunctiva and
replacing the scarred and contracted tarsal plate with composite
C
gra%s.
Fig. 2.60 Lid-everting sutures for entropion. (A) Three double-
armed sutures are passed as shown; (B) sutures are tied; (C)
TIP Horizontal lid laxity should be corrected with a lateral schematic. (Courtesy of AG Tyers and JRO Collin, from Colour
tarsal sling procedure. Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann
! 2001 – figs A and B.)

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 2
CHAPTER
Eyelids 87

A B

C D

Fig. 2.61 Wies procedure for entropion. (A) Full-thickness incision; (B) sutures are passed
through the conjunctiva and lower lid retractors; (C) sutures are passed anterior to the tarsal
plate to exit inferior to the lashes; (D) schematic. (Courtesy of AG Tyers and JRO Collin, from
Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001 – figs A–C.)

MISCELLANEOUS ACQUIRED DISORDERS Dermatochalasis

This is described in the discussion of pseudoptosis (above) and
Varix upper lid blepharoplasty (below).!
An eyelid varix (plural – varices) is a common lesion that may be
mistaken for a naevus or haemangioma. A varix is commonly an
isolated lesion, but may be associated with orbital involvement (see
Floppy eyelid syndrome
Ch. 4). It appears as a dark red or purple subcutaneous compress-
ible (unless thrombosed) lesion (Fig. 2.63A), which in some cases Introduction
becomes apparent only with a Valsalva manoeuvre (Fig. 2.63B and Floppy eyelid syndrome (FES) is an uncommon unilateral or bilateral
C). It is clinically and histologically similar to a lymphangioma. condition that is o%en overlooked as a cause of persistent ocular sur-
Simple excision may be performed for diagnostic or cosmetic rea- face symptoms. It typically affects obese middle-aged and older men
sons. The possibility of orbital communication should be borne in who sleep with one or both eyelids against the pillow, which results in
mind during surgery.! the lid pulling away from the globe. Consequent nocturnal exposure

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
88 Miscellaneous Acquired Disorders

A B

C D

Fig. 2.62 Lower retractor reinsertion. (A) Incision to expose the lower border of the tarsal
plate; (B) reflection of the orbital septum and fat pad to expose the lower lid retractors; (C)
tightening of retractors by plication; (D) schematic. (Courtesy of AG Tyers and JRO Collin,
from Colour Atlas of Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001 – figs A–C.)

and poor contact with the globe, o%en exacerbated by other ocular zzPapillary conjunctivitis of the superior tarsal conjunctiva
surface disease such as dry eye and blepharitis, result in chronic may be intense (Fig. 2.64D).
keratoconjunctivitis. Obstructive sleep apnoea (OSA) is strongly zzKeratopathy. Punctate keratopathy, filamentary keratitis and
associated and is linked to significant morbidity, including cardio- superior superficial vascularization may be present.
pulmonary disease and subtle but irreversible mental dysfunction.! zzOther findings may include eyelash ptosis, lacrimal
gland prolapse, ectropion and aponeurotic ptosis. Patients
Diagnosis with both FES and OSA are at greater risk of developing
zzThe upper eyelid is typically extremely lax, o%en with substan- glaucoma.
tial excess loose upper lid skin (Fig. 2.64A). The tarsal plate has zzInvestigation for OSA should be considered in most cases of
a rubbery consistency (Fig. 2.64B). The lid is very easy to evert FES, particularly if the patient reports substantial snoring and/
(Fig. 2.64C), to fold and to pull away from the eye. or excessive daytime sleepiness.!

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 2
CHAPTER
Eyelids 89

A C

Fig. 2.63 Eyelid varices. (A) Typical appearance of a commonly seen small varix; (B) larger
lesion, probably with orbital involvement, before Valsalva manoeuvre; (C) during Valsalva.
(Courtesy of G Rose – figs B and C.)

A B

C D

Fig. 2.64 Floppy eyelid syndrome. (A) Redundant upper lid skin; (B) loose and rubbery tarsal
plates; (C) very easily everted eyelid; (D) superior tarsal papillary conjunctivitis.

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
90 Miscellaneous Acquired Disorders

Treatment
zzTreatment of associated OSA is likely to be of benefit, and
overweight patients should be encouraged to lose weight.
zzMild cases may respond to lubrication together with nocturnal
eye shield wear or taping of the lids.
zzModerate–severe cases require horizontal shortening to stabi-
lize the lid and ocular surface and prevent nocturnal lagoph-
thalmos. A pentagonal excision of 10 mm or more is taken Fig. 2.65 Blepharochalasis – left aponeurotic ptosis and
from the junction of the lateral third and medial two-thirds thinned upper lid skin.
of the upper lid.!

Blepharochalasis
Blepharochalasis is an uncommon condition characterized by
recurrent episodes of painless, non-pitting oedema of both upper
lids that usually resolves spontaneously a%er a few days. Presenta-
tion is usually around puberty, episodes becoming less frequent
with time. Eyelid skin becomes stretched and atrophic, charac-
teristically said to resemble wrinkled cigarette paper. Severe cases
A
may give rise to stretching of the canthal tendons and levator apo-
neurosis resulting in ptosis (Fig. 2.65) and lacrimal gland prolapse
may occur. A hypertrophic form with orbital fat herniation and an
atrophic form with absorption of orbital fat have been described.
The differential diagnosis includes similarly episodic conditions,
particularly drug-induced urticaria and angioedema. Treatment
involves blepharoplasty for redundant upper lid skin and correc-
tion of ptosis.!
B

Eyelid imbrication syndrome Fig. 2.66 (A) Left lid retraction in thyroid eye disease; (B) fol-
lowing Müller muscle recession. (Courtesy of A Pearson.)
Eyelid imbrication syndrome is an uncommon and frequently
unrecognized disorder in which the upper lid overlaps the lower
on closure so that the lower lashes irritate the superior marginal
tarsal conjunctiva. It may be unilateral or bilateral and the major Table 2.5 Causes of Lid Retraction
symptom is ocular irritation. It can be acquired, commonly asso- 1. Thyroid eye disease (Fig. 2.67A)
ciated with FES, or – very rarely – can be congenital. Occasion- 2. Neurogenic
ally it may follow lower lid tarsal strip surgery. Associated signs Contralateral unilateral ptosis (Fig. 2.67B)
include superior tarsal papillary conjunctivitis and rose Bengal Unopposed levator action due to facial palsy
Third nerve misdirection
staining of the superior marginal conjunctiva. Definitive treat-
Marcus Gunn jaw-winking syndrome
ment consists of upper lid pentagon resection and/or lateral can- Collier sign of the dorsal midbrain (Parinaud syndrome
thal tightening.! – see Fig. 19.85D)
Infantile hydrocephalus (setting sun sign – Fig. 2.67C)
Parkinsonism (Fig. 2.67D)
Upper lid retraction Sympathomimetic drops
3. Mechanical
Upper lid retraction is suspected when the upper lid margin is Surgical over-correction of ptosis
either level with or above the superior limbus (Fig. 2.66A). Inter- Scarring of upper lid skin
mittent upper lid retraction may occur in healthy infants and 4. Congenital
disappears by 6 months. Other causes are listed in Table 2.5 and Isolated
Duane retraction syndrome
selected examples are illustrated in Fig. 2.67. Where there is no
Down syndrome
loss or tightness of the upper eyelid skin, retraction is corrected Transient ‘eye popping’ reflex in normal infants
by surgical release of the eyelid retractors, usually via a transcon- 5. Miscellaneous
junctival posterior approach. Mild retraction may be treated with Prominent globe (pseudo-lid retraction)
Müller muscle recession (Fig. 2.66B). Moderate–severe retraction Severe liver disease (Summerskill sign)
Idiopathic
may require levator aponeurosis recession.!

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CHAPTER
Eyelids 91

A B

C D

Fig. 2.67 Causes of lid retraction. (A) Thyroid eye disease; (B) unilateral myasthenic ptosis with
contralateral lid retraction; (C) ‘setting sun’ sign in infantile hydrocephalus; (D) Parkinson disease.
(Courtesy of R Bates – fig. C.)

Lower lid retraction Non-surgical techniques

Inferior scleral show may be physiological in patients with
large eyes or shallow orbits, but is commonly involutional or Botulinum toxin injection to periocular muscles
secondary to some of the conditions in Table 2.5. It may fol- Botulinum toxin injection can be used to reduce wrinkling, particularly
low lower lid blepharoplasty, when aggressive upward mas- for ‘crows’ feet’ at the lateral canthus and for glabellar frown lines and
sage of the lid for 2 or 3 months may be curative for minor ‘brow li%’ by a reduction in the action of brow depressors. Complica-
degrees. In other cases, a tarsal strip operation may raise the tions include temporary ptosis, lagophthalmos, ectropion and diplopia.!
lid slightly but when moderate elevation is required, inferior
retractor recession with a posterior lamellar spacer is likely to Tissue fillers
be necessary. More aggressive procedures have been described These are used to address age-related wrinkles and, less commonly,
for severe cases.! defects from other causes such as trauma. Complications include
hypersensitivity reactions.
zzHyaluronic acid is the most commonly used tissue filler and
COSMETIC EYELID AND PERIOCULAR can be used to temporarily fill in hollows and replace lost vol-
SURGERY ume. This is injected deep to orbicularis and the effects gener-
ally last 3–12 months depending on the agent used.
Involutional changes zzAutologous fat gives a more permanent replacement.
Involutional (age-related) changes around the eyes can lead to zzOthers include collagen, microspheres of calcium hydroxy-
functional and cosmetic concerns that may require treatment. apatite and synthetic fillers.!
zzReduction in cutaneous elasticity and thickness results in
loose, wrinkled skin. Skin resurfacing
zzWeakening of the orbital septum may lead to orbital fat Removal of the superficial layers of the skin, by chemical peels or laser,
prolapse. can lead to a reduction in wrinkling, increased evenness of pigmenta-
zzThinning and stretching of the canthal tendons, levator apo- tion, removal of blemishes and improved texture by generating new
neurosis and lower lid retractors may cause eyelid laxity and epidermis and increasing collagen production in the dermis.!
ptosis.
zzAtrophy of orbital and eyebrow fat pads can give enophthal-
mos and eyebrow sagging.
Surgical techniques
zzWeakening of the frontalis muscle and epicranial aponeuro-
sis may cause descent of the eyebrows and increasing looseness Upper eyelid blepharoplasty
of upper eyelid skin. Upper eyelid involutional changes are characterized by surplus
zzThinning and stretching of midfacial support leads to upper eyelid skin (dermatochalasis) that leads to baggy lids with
descent with formation of a tear trough depression and exac- indistinct creases and pseudo- or mechanical ptosis. It may cause a
erbation of lower eyelid changes. heavy sensation around the eyes, brow-ache and, in more advanced
zzThinning and resorption of periorbital bone exacerbates the cases, obstruction of the superior visual field (Fig. 2.68A). Upper
appearance of surplus overlying tissues.! lid blepharoplasty (Fig. 2.68B) is effective for the removal of

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92 Congenital Malformations

A B

C D

E F

Fig. 2.68 (A) Severe dermatochalasis causing reduction of upper visual field; (B) appearance
following surgery; (C) mild dermatochalasis and excess lower lid skin; (D) appearance follow-
ing upper and lower lid blepharoplasty; (E) right brow ptosis and dermatochalasis; (F) follow-
ing direct brow lift. (Courtesy of A Pearson.)

surplus skin and can be combined with reduction of the superior zzDirect brow lift. An incision is made above the eyebrow hairs
orbital fat pads. Care must be taken prior to surgery to look for and an ellipse of skin is removed (Fig. 2.68F).
ptosis of the eyelid or eyebrow and ocular surface dryness. Com- zzEndoscopic brow lift. Small incisions within the hair-line
plications include removal of excess skin leading to lagophthalmos enable endoscopic elevation of the whole forehead tissues
and corneal drying and removal of excess orbital fat leading to an and release at the eyebrow periosteum to allow li%ing of the
unattractive hollowed out upper eyelid sulcus.! eyebrows through sutures supported on frontal bone anchors
within the hair-line.!
Lower eyelid blepharoplasty
Lower lid involutional changes are characterized by excess skin
and/or prolapsed orbital fat (Fig. 2.68C) which can be addressed
CONGENITAL MALFORMATIONS
with blepharoplasty (Fig. 2.68D).
zzAnterior approach. Where there is excess skin, an anterior
Epicanthic folds
approach is used to raise a skin/muscle flap that can be li%ed Epicanthic folds are bilateral vertical folds of skin that extend from
and re-draped on the lid with the surplus removed. At the the upper or lower lids towards the medial canthi (Fig. 2.69A). They
same time the inferior orbital fat pads can be reduced by a may give rise to pseudoesotropia. The folds may involve the upper or
small incision through the septum. lower lids or both. Lower lid folds extending upwards to the medial
zz Posterior approach. Bulging of the lower eyelid fat pads without eye- canthal area (epicanthus inversus – Fig. 2.69B) are associated with
lid laxity or surplus skin is best reduced by a posterior, transconjuncti- the blepharophimosis syndrome. Treatment is by V–Y or Z-plasty.!
val approach. Complications include lower eyelid retraction, contour
abnormalities (particularly lateral drooping) and ectropion.! Blepharophimosis, ptosis and
Brow ptosis correction epicanthus inversus syndrome
Brow ptosis frequently accompanies dermatochalasis (Fig. 2.68E) Blepharophimosis, ptosis and epicanthus inversus syndrome
and may also follow facial nerve palsy or localized trauma. Li%ing (BPES) is a complex of eyelid malformations consisting of mod-
of the brow needs to precede or occasionally be combined with erate–severe symmetrical ptosis with poor levator function, tele-
upper lid blepharoplasty. canthus, epicanthus inversus, manifesting with small palpebral

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 2
CHAPTER
Eyelids 93

A B

D
Fig. 2.69 Congenital malformations. (A) Epicanthic fold; (B) epicanthus inversus; (C) dominantly inherited blepharophimosis –
the child’s grandfather and father have undergone surgery to correct the defect; (D) telecanthus.

fissures (Fig. 2.69C). Other minor facial anomalies are com- chromosome 3. Treatment initially involves correction of epican-
monly present. Inheritance is usually AD. Both BPES type I (with thus and telecanthus, followed later by bilateral frontalis suspen-
premature ovarian failure) and BPES type II (without premature sion. It is also important to treat amblyopia, which is present in
ovarian failure) are caused by mutations in the FOXL2 gene on about 50%.!

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94 Congenital Malformations

Fig. 2.71 Congenital lower lid entropion.

the medial canthi due to abnormally long medial canthal ten-

dons (Fig. 2.69D). It should not be confused with hypertelorism
in which there is wide bony separation of the orbits. Treatment
involves shortening and refixation of the medial canthal ten-
dons to the anterior lacrimal crest, or insertion of a trans-nasal
suture.!

Epiblepharon
Epiblepharon comprises an extra horizontal fold of skin stretch-
ing across the anterior lid margin and is commonly found in
individuals of Eastern Asian ethnicity. The lashes are directed
B vertically, especially in the medial part of the lid (Fig. 2.70A
and B). When the fold of skin is pulled down the lashes turn
out and the normal location of the lid becomes apparent (Fig.
2.70C). It should not be confused with the much less common
congenital entropion (see next). Treatment is not required in
the majority of individuals of European origin because spon-
taneous resolution with age is usual. Persistent cases may be
treated surgically.!

Congenital entropion
Upper lid entropion is usually secondary to the mechanical
effects of microphthalmos, which cause variable degrees of
upper lid inversion. Lower lid entropion (Fig. 2.71) is gener-
ally caused by maldevelopment of the inferior retractor apo-
neurosis. Treatment involves the excision of a strip of skin and
muscle and fixation of the skin crease to the tarsal plate (Hotz
C
procedure).!
Fig. 2.70 (A) Epiblepharon; (B) lashes pointing upwards; (C)
normal position of lashes following manual correction.
Coloboma
A congenital coloboma is an uncommon, unilateral or bilateral,
Telecanthus partial- or full-thickness eyelid defect. It occurs when eyelid devel-
Telecanthus is an uncommon condition that may occur in iso- opment is incomplete, due to either failure of migration of lid ecto-
lation or in association with blepharophimosis and some sys- derm to fuse the lid folds or to mechanical forces such as amniotic
temic syndromes. It manifests with increased distance between bands. Coloboma elsewhere in the eye (see Figs 19. 26 and 19.27),

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 2
CHAPTER
Eyelids 95

B C

Fig. 2.72 (A) Upper lid coloboma; (B) lower lid coloboma in Treacher Collins syndrome;
(C) Treacher Collins syndrome.

as well as a range of other associations, may be present. The treat- (Fig. 2.73C), which may result in lagophthalmos and exposure kera-
ment of small defects involves primary closure, while large defects topathy. Euryblepharon is present at birth and there are no other
require skin grafts and rotation flaps. associated ocular abnormalities. It is usually dominantly inherited,
zzUpper lid coloboma occurs at the junction of the middle but may occur sporadically. Systemic conditions associated with
and inner thirds (Fig. 2.72A). Relatively strong associations euryblepharon include: Kabuki syndrome, Down syndrome and
include cryptophthalmos (see below), facial abnormalities and blepharocheilodontic syndrome. Most cases do not require treat-
Goldenhar syndrome. ment, as the condition becomes less pronounced with facial growth.
zzLower lid coloboma occurs at the junction of the middle and Surgical treatment may be needed to protect the cornea.!
outer thirds (Fig. 2.72B) and is frequently associated with sys-
temic conditions.
zzTreacher Collins syndrome (mandibulofacial dysostosis) is a
Microblepharon
genetically heterogeneous condition characterized by malfor- Microblepharon is characterized by small eyelids, often associated
mation of derivatives of the first and second branchial arches, with anophthalmos.!
principally mandibular and ear anomalies. Lower eyelid colo-
boma is a feature. Ocular anomalies also described include
slanted palpebral apertures, cataract, microphthalmos and lac-
Congenital upper lid eversion
rimal atresia (Fig. 2.72C).! Congenital upper lid eversion is a rare condition more frequently
seen in infants of Afro-Caribbean origin, in Down syndrome and
in congenital ichthyosis (collodion skin disease – Fig. 2.73D). It is
Cryptophthalmos typically bilateral and symmetrical. It may resolve spontaneously
Cryptophthalmos is a rare congenital anomaly in which the eyelids with conservative treatment or require surgery.!
are absent, replaced by a continuous layer of skin.
zzComplete cryptophthalmos. A microphthalmic eye (Fig.
2.73A) is covered by a fused layer of skin with no separation
Ablepharon
between the lids. Ablepharon consists of deficiency of the anterior lamellae of
zzIncomplete cryptophthalmos is characterized by rudimen- the eyelids (Fig. 2.73E). Treatment involves reconstructive
tary lids and microphthalmos (Fig. 2.73B). skin grafting. Ablepharon-macrostomia syndrome is charac-
zzFraser syndrome is a recessively inherited condition in terized by an enlarged fish-like mouth, ear, skin and genital
which cryptophthalmos is a common finding. Other fea- anomalies.!
tures can include syndactyly, urogenital and craniofacial
anomalies.!
Ankyloblepharon filiforme adnatum
In ankyloblepharon filiforme adnatum the upper and lower eye-
Euryblepharon lids are joined by thin tags (Fig. 2.73F). Most cases are sporadic.
Euryblepharon refers to horizontal enlargement of the palpebral fis- Treatment involves transection with scissors, usually without
sure with associated lateral canthal malposition and lateral ectropion anaesthesia.

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B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
96 Congenital Malformations

A B

C D

E F

Fig. 2.73 (A) Complete cryptophthalmos; (B) incomplete cryptophthalmos; (C) euryblepharon
(arrow shows slightly everted lateral lower lid); (D) congenital upper lid eversion in a patient
with ichthyosis; (E) ablepharon; (F) ankyloblepharon filiforme adnatum (arrow shows adhe-
sion). (Courtesy of D Meyer – figs A and D; M Parulekar – figs C, E and F.)

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B A BC EC 0 D B EC C D ED A B CC A B DI ,C F B. ( B DC B C BF
 Chapter

Lacrimal Drainage System 3

INTRODUCTION 98 Secondary punctal stenosis 104 CHRONIC CANALICULITIS 109
Anatomy 98 Canalicular obstruction 105
Nasolacrimal duct obstruction 106 DACRYOCYSTITIS 110
Physiology 98
Dacryolithiasis 106 Acute dacryocystitis 110
Causes of a watering eye 99
Chronic dacryocystitis 111
Evaluation 99 CONGENITAL OBSTRUCTION 108
ACQUIRED OBSTRUCTION 103 Nasolacrimal duct obstruction 108
Conjunctivochalasis 103 Congenital dacryocoele 109
Primary punctal stenosis 103

97
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98 Introduction

INTRODUCTION Physiology
Tears secreted by the main and accessory lacrimal glands pass
Anatomy across the ocular surface. A variable amount of the aqueous com-
The lacrimal drainage system consists of the following structures ponent of the tear film is lost by evaporation, with the remainder
(Fig. 3.1): of the tears hypothesized to drain substantially as follows (Fig. 3.2):
zzThe puncta are located at the posterior edge of the lid margin, zzTears flow along the upper and lower marginal strips (Fig.
at the junction of the lash-bearing lateral five-sixths (pars cili- 3.2A), pooling in the lacus lacrimalis medial to the lower
aris) and the medial non-ciliated one-sixth (pars lacrimalis). puncta, then entering the upper and lower canaliculi by a com-
The punctum and vertical canaliculus are part of the tarsal bination of capillarity and suction.
plate. Normally the puncta face slightly posteriorly and can be zzWith each blink, the pretarsal orbicularis oculi muscle com-
inspected by everting the medial aspect of the lids. Treatment presses the ampullae, shortens and compresses the horizontal
of watering caused by punctal stenosis or malposition is rela- canaliculi and closes and moves the puncta medially, resisting
tively straightforward. reflux. Simultaneously, contraction of the lacrimal part of the
zzThe canaliculi pass vertically from the lid margin for about orbicularis oculi creates a positive pressure that forces tears
2 mm (ampullae). They then turn medially and run horizon- down the nasolacrimal duct and into the nose, mediated by
tally for about 8 mm to reach the lacrimal sac. The horizontal helically arranged connective tissue fibres around the lacrimal
canaliculus is surrounded by the palpebral part of the orbicu- sac (Fig. 3.2B).
laris oculi muscle (Horner muscle). The superior and inferior zzWhen the eyes open, the canaliculi and sac expand, creating
canaliculi usually (>90%) unite to form the common cana- negative pressure that draws tears from the canaliculi into the
liculus, which opens into the lateral wall of the lacrimal sac. sac (Fig. 3.2C).!
Uncommonly, each canaliculus opens separately into the sac.
A small flap of mucosa (Rosenmüller valve) overhangs the
junction of the common canaliculus and the lacrimal sac (the
internal punctum) and prevents reflux of tears into the cana- Canaliculus (8 mm)
liculi. Treatment of canalicular obstruction may be complex.
zzThe lacrimal sac is 10–12 mm long and lies in the lacrimal
fossa between the anterior and posterior lacrimal crests. The Lacrimal
lacrimal bone and the frontal process of the maxilla separate sac (10 mm)
the lacrimal sac from the middle meatus of the nasal cavity.
In a dacryocystorhinostomy (DCR) an anastomosis is created Nasolacrimal
Ampulla (2 mm) duct (12 mm)
between the sac and the nasal mucosa to bypass an obstruction
in the nasolacrimal duct.
Common canaliculus Valve of Hasner
zzThe nasolacrimal duct is 12–18 mm long and is the infe-
rior continuation of the lacrimal sac. It descends and angles
slightly laterally and posteriorly to open into the inferior
nasal meatus, lateral to and below the inferior turbinate. The
opening of the duct is partially covered by a mucosal fold
(valve of Hasner).! Fig. 3.1 Anatomy of the lacrimal drainage system.

A B C

Fig. 3.2 Physiology of the lacrimal drainage system. (A) Tears flow along the marginal strips,
pooling in the lacus lacrimalis; (B) contraction of the orbicularis oculi muscle with each blink
forces tears down the nasolacrimal duct; (C) when the eyes open, the canaliculus and sac
expand creating a negative pressure that draws tears from the canaliculus into the sac.

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 Lacrimal Drainage System
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3 99

{z Occasionally an eyelash may lodge in the ampulla (Fig. CHAPTER

Causes of a watering eye 3.3D).
Epiphora is the overflow of tears at the eyelid margin. There are {z A large caruncle may displace the punctum away from the
two mechanisms: globe (Fig. 3.3E).
zzHypersecretion secondary to anterior segment disease such {z In the presence of substantial lid laxity, the puncta may
as dry eye (‘paradoxical watering’) or inflammation. In these rarely over-ride each other.
cases, watering is associated with symptoms of the underlying {z A pouting punctum (Fig. 3.3F) is typical of canaliculitis.
cause and treatment is usually medical. {z The eyelid skin will often be moderately scaly and ery-
thematous in chronic epiphora.
{z Centurion syndrome is characterized by anterior malpo-
TIP It is common for a watering eye to be caused by reflex sition of the medial part of the lid, with displacement of
hypersecretion of tears secondary to a dry ocular surface.
puncta out of the lacus lacrimalis due to a prominent nasal
bridge.
zzDefective drainage due to a compromised lacrimal drainage {z Occasionally a child with congenital glaucoma can present
system. This may be caused by: with a watery eye.
{z Malposition (e.g. ectropion) of the lacrimal puncta. zzThe lacrimal sac should be palpated. Punctal reflux of muco-
{z Obstruction at any point along the drainage system, from purulent material on compression is indicative of a mucocoele
the punctal region to the valve of Hasner. (a dilated mucus-filled sac; US spelling – mucocele) with a pat-
{z Lacrimal pump failure, which may occur secondarily to ent canalicular system, but with an obstruction either at or dis-
lower lid laxity or weakness of the orbicularis muscle (e.g. tal to the lower end of the lacrimal sac. In acute dacryocystitis,
facial nerve palsy).! palpation is painful and should be avoided. Rarely, palpation of
the sac will reveal a stone or tumour.!
Evaluation Fluorescein disappearance test
The marginal tear strip of both eyes should be examined on the
History slit lamp prior to any manipulation of the eyelids or instillation of
Enquiry should be made about ocular discomfort and redness topical medication. Many patients with watering do not have obvi-
to aid in excluding hypersecretion. Drainage failure tends to be ous overflow of tears but merely show a high meniscus (marginal
exacerbated by a cold and windy environment and to be least evi- tear strip) of 0.6 mm or more (Fig. 3.4) versus 0.2–0.4 mm nor-
dent in a warm, dry room. A complaint of the tears overflowing mally. The fluorescein disappearance test is performed by instill-
onto the cheek is likely to indicate drainage failure rather than ing fluorescein 1% or 2% drops into both conjunctival fornices.
hypersecretion.! Normally, little or no dye remains after 5–10 minutes. Prolonged
retention is indicative of inadequate lacrimal drainage. This should
External examination be distinguished from the ‘fluorescein clearance test’ used to assess
Punctal abnormality is the most common cause of lacrimal drain- tear turnover in dry eye, in which retained stain is measured in the
age failure. meniscus 15 minutes after instillation of 5 µl of fluorescein.!
zzThe puncta and eyelids should be examined using a slit lamp.
It is critical that examination of the puncta is performed prior Lacrimal irrigation
to cannulation for diagnostic irrigation, which temporarily Lacrimal irrigation should be performed only after ascertaining
dilates the punctal opening and masks stenosis. punctal patency. If absent or severely stenosed, surgical enlarge-
{z There will often be obvious tear overflow from the medial, ment of the punctum may be needed before canalicular and naso-
or less commonly the lateral, canthal region. lacrimal duct patency can be confirmed. It is contraindicated in
{z Visible mucopurulent discharge is more likely to occur acute infection.
with nasolacrimal duct obstruction than a blockage more zzLocal anaesthetic is instilled into the conjunctival sac.
proximally. zzA punctum dilator is used to enlarge the punctal orifice (Fig.
{z Punctal stenosis (Fig. 3.3A). This is extremely common 3.5A), entering vertically and then tilting the instrument horizon-
and has been reported as present in up to about half of the tally whilst exerting lateral tension on the lid (Fig. 3.5B and C).
general population. Over half of patients with evident ste- zzA gently curved, blunt-tipped 26- or 27-gauge lacrimal can-
nosis are asymptomatic, in many cases due to insu%ciency nula on a 3 ml saline-filled syringe is inserted into the lower
of tear production or increased evaporation. punctum and, whilst keeping a gentle stretch laterally on the
{z Ectropion, either localized to the punctal region or involv- eyelid, advanced a few millimetres, following the contour of
ing the wider lid, is often associated with secondary steno- the canaliculus (Fig. 3.5D).
sis (Fig. 3.3B). zzA hard stop occurs if the cannula enters the lacrimal sac, com-
{z Punctal obstruction, usually partial, by a fold of redundant ing to a stop at the medial wall of the sac, through which can be
conjunctiva (conjunctivochalasis – Fig. 3.3C) is common felt the rigid lacrimal bone (Fig. 3.6A). This excludes complete
but underdiagnosed. obstruction of the canalicular system. Gentle saline irrigation

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100 Introduction

A B

C D

E F

Fig. 3.3 (A) Marked punctal stenosis; (B) mild medial ectropion; (C) conjunctivochalasis
(arrows); (D) punctal obstruction by an eyelash (arrow); (E) large caruncle; (F) pouting punctum.

is then attempted. If saline passes into the nose and throat, the nasolacrimal duct. In this situation, the lacrimal sac will
when it will be tasted by the patient, a patent lacrimal system distend slightly during irrigation and there will be reflux, usu-
is present, although there may still be stenosis; alternatively, ally through both the upper and lower puncta. The regurgitated
symptoms may be due to subtle lacrimal pump failure. Failure material may be clear, mucoid or mucopurulent, depending on
of saline to reach the throat is indicative of total obstruction of the contents of the lacrimal sac.

B( EC 2C B D , AD / ) B / 4 ,C F B 1 AD 4 B
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 Lacrimal Drainage System
CHAPTER
3 101

zzA soft stop is experienced if the cannula stops at or proximal to the cannula presses the soft tissue of the common canaliculus CHAPTER
the junction of the common canaliculus and the lacrimal sac. and the lateral wall against the medial wall of the sac and the
The sac is thus not entered – a spongy feeling is experienced as lacrimal bone behind it (Fig. 3.6B). As a crimped canaliculus
with occlusion of the cannula tip against the canalicular wall
can also give this impression, it is worthwhile slightly retract-
ing the tip, increasing the lateral tension on the lid and gently
repeating the attempt to advance the probe. In the case of lower
canalicular obstruction, a soft stop will be associated with
reflux of saline through the lower punctum. Reflux through
the upper punctum indicates patency of both upper and lower
canaliculi, but obstruction of the common canaliculus.!

Jones dye testing

Dye testing is indicated only in patients with suspected partial
obstruction of the drainage system. Epiphora is present, but there
is no punctal abnormality and the patient tastes saline in his or her
throat on irrigation.
zzThe primary test (Fig. 3.7A) differentiates partial obstruc-
tion of the lacrimal passages and lacrimal pump failure from
primary hypersecretion of tears. A drop of 2% fluorescein is
Fig. 3.4 High marginal tear strip stained with fluorescein. instilled into the conjunctival sac of one eye only. After about 5

A B

C D

Fig. 3.5 (A) Dilatation of the inferior punctum; (B) and (C) dilatation technique; (D) irrigation.

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102 Introduction

A B

Fig. 3.6 Possible results of probing. (A) Hard stop; (B) soft
stop.

Positive Negative

Fig. 3.8 Drainage test showing 2% fluorescein in the left

nostril only (arrow), confirming a patent nasolacrimal duct on
that side. (Courtesy of D Hildebrand.)

on the basis of whether the topical fluorescein instilled for

the primary test entered the lacrimal sac. Topical anaesthetic
is instilled and any residual fluorescein washed out from the
conjunctival fornix. The drainage system is then irrigated with
a cotton bud under the inferior turbinate.
{z Positive: fluorescein-stained saline recovered from the
A
nose indicates that fluorescein entered the lacrimal sac,
thus confirming functional patency of the upper lacrimal
passages. Partial obstruction of the nasolacrimal duct dis-
Positive Negative
tal to the sac is inferred (Fig. 3.8).
{z Negative: unstained saline recovered from the nose indicates
that fluorescein did not enter the lacrimal sac. This implies
upper lacrimal (punctal or canalicular) dysfunction, which
may be due to partial physical occlusion and/or pump failure.!

Contrast dacryocystography
Dacryocystography (DCG – Fig. 3.9) involves the injection of radio-
opaque contrast medium (ethiodized oil) into the canaliculi followed
B by the capture of magnified images. Indications include confirmation
of the precise site of lacrimal drainage obstruction to guide surgery
Fig. 3.7 Jones dye testing. (A) Primary; (B) secondary. and the diagnosis of diverticuli, fistulae and filling defects (e.g. stones,
minutes, a cotton-tipped bud moistened in local anaesthetic is tumours). It should not be performed in the presence of acute infec-
inserted under the inferior turbinate at the nasolacrimal duct tion. A DCG is unnecessary if the site of obstruction is obvious (e.g.
opening. The results are interpreted as follows: regurgitating mucocoele). A normal dacryocystogram in the presence
{z Positive: fluorescein recovered from the nose indicates of subjective and objective epiphora suggests failure of the lacrimal
patency of the drainage system. Watering is due to primary pump, though this is more readily demonstrated by simple irrigation.!
hypersecretion and no further tests are necessary.
{z Negative: no dye recovered from the nose indicates a partial Nuclear lacrimal scintigraphy
obstruction (site unknown) or failure of the lacrimal pump Scintigraphy (Fig. 3.10) assesses tear drainage under more physi-
mechanism. In this situation the secondary dye test is per- ological conditions than DCG, by labelling the tears with a radio-
formed immediately. There is a high false-negative rate – that active substance and tracking their progress. Although it does not
is, dye is commonly not recovered even in the presence of a provide the same detailed anatomical visualization as DCG, it may
functionally patent drainage system. Modifications involving be used to identify the location of a partial or functional block (e.g.
direct observation of the oropharynx using cobalt blue light for indicating the absence of significant tear entry to the canaliculi,
up to an hour may reduce the false-negative rate almost to zero. localizing the site of physiological obstruction to the eyelids), to
zzThe secondary (irrigation) test (Fig. 3.7B) identifies lacri- confirm functional obstruction, or sometimes to confirm the pres-
mal pump failure or the probable site of partial obstruction, ence of normal drainage such that surgery is not indicated.

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 Lacrimal Drainage System
CHAPTER
3 103

CT and MRI CHAPTER

Computed tomography (CT) and magnetic resonance imaging
(MRI) are occasionally employed in the assessment of lacrimal
obstruction, for instance in the investigation of paranasal sinus or
suspected lacrimal sac pathology.!

Internal nasal examination

Assessment of the nasal cavity, especially with endoscopy, can be
invaluable in the detection of obstructions such as nasal polyps or
a deviated septum.!

ACQUIRED OBSTRUCTION
A Conjunctivochalasis
Conjunctivochalasis is characterized by one or more folds of
redundant conjunctiva prolapsing over the lower eyelid margin
(see Fig. 3.3C). It can exacerbate the symptoms of dry eye and
commonly contributes to epiphora, of which it can be an under-
recognized cause. It is thought to be predominantly an involutional
process involving the loss of conjunctival adhesion to underlying
Tenon capsule and episclera and may be analogous to the conjunc-
tival abnormalities leading to superior limbic keratoconjunctivitis
(see Ch. 6). Chronic low-grade ocular surface inflammation (e.g.
dry eye, blepharitis) is likely to play a role. If severe, exposure of a
redundant fold can occur (Fig. 3.11).
zzObservation or lubricants alone may be appropriate in mild
cases.
zzTopical steroids or other anti-inflammatories.
zzSurgical options include securing the bulbar conjunctiva to
B the sclera with three absorbable sutures (e.g. 6-0 polyglactin)
placed 6–8 mm from the limbus, or excision of a crescent-
shaped area of excess bulbar conjunctiva, with an anterior limit
of around 6 mm from the limbus. Suturing the edges of the
excised patch together, or replacement with amniotic mem-
brane have been described.!

Primary punctal stenosis

Primary stenosis (see Fig. 3.3A) occurs in the absence of punctal
eversion. The most common causes are chronic blepharitis and
idiopathic stenosis. Other causes include herpes simplex and her-
pes zoster lid infection, local radiotherapy, cicatrizing conjunctivi-
tis, chronic topical glaucoma treatment, systemic cytotoxic drugs
such as 5-fluorouracil and rare systemic conditions such as por-
phyria cutanea tarda.
C zzDilatation of the punctum alone can be tried, but rarely
Fig. 3.9 Dacryocystography (DCG). (A) Conventional DCG gives sustained benefit. The results are significantly improved
without subtraction showing normal filling on both sides; (B) by inserting a mini-Monoka stent (FCI Ophthalmics) after
normal left filling and obstruction at the junction of the right simple dilatation. The stent is removed after one month (see
sac and nasolacrimal duct (arrow); (C) digital subtraction DCG Fig. 22.3B).
showing similar findings to (B). (Courtesy of A Pearson.) zzPunctoplasty may be needed, but it is important to remember
that surgical intervention has the potential to disrupt the nor-
TIP Scintigraphy allows tear drainage to be assessed under mal anatomical and physiological punctal tear drainage func-
physiological conditions and may be helpful to identify the tion. A number of techniques have been described, including
location of a partial or functional block.
! one-, two- (Fig. 3.12) or three-snip enlargement with removal

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104 Acquired Obstruction

A B

C D

Fig. 3.10 (A–D) Nuclear lacrimal scintigraphy showing passage of tracer via the right lacrimal
system but obstructed drainage in the left nasolacrimal duct. (Courtesy of A Pearson.)

of the posterior ampulla wall and procedures using a mechani-

cal punch (e.g. Kelly punch), laser or microsurgery; a tempo-
rary stent can be used.!

Secondary punctal stenosis

Secondary stenosis occurs after punctal eversion leads to chronic
failure of tear entry and punctoplasty is usually performed in con-
junction with correction of the eversion.
zzRetropunctal (Ziegler) cautery can be used for pure punc-
tal eversion. Burns are applied to the palpebral conjunctiva at
approximately 5 mm below the punctum. Subsequent tissue
shrinkage should invert the punctum.
zzMedial conjunctivoplasty can be used in medial ectropion of
a larger area of lid if there is no substantial horizontal laxity.
A diamond-shaped piece of tarsoconjunctiva is excised, about
Fig. 3.11 Conjunctivochalasis. Substantial exposed fold with 4 mm high and 8 mm wide, parallel with and inferolateral to
conjunctival and corneal rose Bengal staining. (Courtesy of the canaliculus and punctum, followed by approximation of the
S Tuft.) superior and inferior wound margins with sutures (Fig. 3.13).

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 Lacrimal Drainage System
CHAPTER
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CHAPTER

A B

Fig. 3.12 Two-snip punctoplasty. (A) Technique; (B) postoperative appearance (arrowheads).

Fig. 3.13 Medial conjunctivoplasty. Fig. 3.14 Silicone lacrimal stent in situ.

Incorporation of the lower lid retractors in the sutures further silicone stents. These are left in situ for 6 weeks to 6 months
aids repositioning. (Fig. 3.14).
zzLower lid tightening, usually with a tarsal strip, is used to cor- zzTotal individual canalicular obstruction
rect lower lid laxity and may be combined with medial con- {z Canalicular trephination using a purpose-made minitrephine
junctivoplasty where there is a significant medial ectropion (Sisler), followed by intubation. Trephination has also been
component.! described using an intravenous catheter with a retracted intro-
ducer needle used as a stent and then advanced to overcome
the obstruction. Balloon canaliculoplasty and endoscopic
Canalicular obstruction laser techniques are available. These less invasive options may
Causes include congenital, trauma, herpes simplex infection, drugs have lower success rates than more aggressive surgery.
and irradiation. Chronic dacryocystitis can cause a membrane to {z With 6–8 mm of patent normal canaliculus between the
form in the common canaliculus. The initial surgical approach has punctum and the obstruction, anastomosis of the patent
tended over recent years to attempt to preserve the physiological part of the canaliculus into the lacrimal sac, with intuba-
anatomy. tion, can be performed.
zzPartial obstruction of the common or individual canalic- {z Where obstruction is severe or it is not pos-
uli, or anywhere in the lacrimal drainage system, may be sible to anastomose functioning canaliculi to the
treated by simple intubation of one or both canaliculi with lacrimal sac, conventional surgery consists of

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106 Acquired Obstruction

Fig. 3.16 Dacryocystorhinostomy showing the anastomosis

between the lacrimal sac and the mucosa of the nose.

opening of the common canaliculus and consists of anas-

tomosis of the lacrimal sac to the mucosa of the middle
nasal meatus (Fig. 3.16). The procedure is usually per-
formed under hypotensive general anaesthesia. A vertical
skin incision is made 10 mm medial to the inner canthus,
the medial canthal tendon and lacrimal sac exposed and
reflected and after removal of the intervening bone the
sac is incised and attached to an opening created in the
nasal mucosa (Fig. 3.17). The success rate is over 90%, but
causes of failure include inadequate size and position of
the ostium, unrecognized common canalicular obstruc-
B tion, scarring and the ‘sump syndrome’, in which the sur-
gical opening in the lacrimal bone is too small and too
Fig. 3.15 (A) Lester Jones tube; (B) CT scan 3D reconstruction high. Complications include cutaneous scarring, injury
of tube in situ. to medial canthal structures, haemorrhage, infection and
cerebrospinal fluid rhinorrhoea if the subarachnoid space
conjunctivodacryocystorhinostomy and the insertion is inadvertently entered.
of a toughened glass (Lester Jones) tube (Fig. 3.15). This {z Endoscopic DCR encompasses several techniques. A light
may also be used when the lacrimal system is intact but pipe can be passed through the canalicular system into the
non-functioning due to failure of the physiological pump lacrimal sac to guide an endoscopic approach from within
(e.g. facial nerve palsy). The surgery is performed as for the nose, or a microendoscopic transcanalicular procedure
an external approach dacryocystorhinostomy (DCR – see can be performed using a drill or laser to establish com-
below) but the caruncle is excised and a track for the tube munication with the nasal cavity. Advantages over conven-
created between the lacus lacrimalis and the lacrimal sac. tional DCR include less marked systemic disturbance with
Patient satisfaction is variable.! minimal blood loss and a lower risk of cerebrospinal fluid
leakage, the avoidance of a skin incision and generally a
shorter operating time. Disadvantages include generally
Nasolacrimal duct obstruction a slightly lower success rate and visualization di%culties,
zzCauses meaning that additional procedures are sometimes needed.
{z Idiopathic stenosis – by far the most common. {z Other procedures, often reserved for partial nasolacrimal
{z Naso-orbital trauma, including nasal and sinus surgery. duct obstruction, include probing and intubation, stent
{z Granulomatous disease such as granulomatosis with poly- insertion and balloon dacryocystoplasty.!
angiitis and sarcoidosis.
{z Infiltration by nasopharyngeal tumours.
zzTreatment
Dacryolithiasis
{z Conventional (external approach) dacryocystorhinostomy Dacryoliths (lacrimal stones) may occur in any part of the lac-
(DCR) is indicated for obstruction distal to the medial rimal system. They are more common in males. Although the

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 Lacrimal Drainage System
CHAPTER
3 107

CHAPTER

A B

C D

E F

Fig. 3.17 Technique of dacryocystorhinostomy. (A) Vertical skin incision, made 10 mm medial
to the inner canthus, avoiding the angular vein; (B) the anterior lacrimal crest is exposed and
the periosteum is divided from the spine on the anterior lacrimal crest to the fundus of the
sac and reflected forwards. The sac is reflected laterally from the lacrimal fossa; (C) the an-
terior lacrimal crest and the bone from the lacrimal fossa are removed; (D) the sac is incised
in an ‘H-shaped’ manner to create two flaps. A vertical incision is made in the nasal mucosa
to create anterior and posterior flaps; (E) the posterior flaps are sutured; (F) the anterior flaps
are sutured.

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108 Congenital Obstruction

C B

Fig. 3.18 Epiphora. (A) Child with a watering eye; (B) probing of the nasolacrimal duct; (C) con-
genital dacryocoele. (Courtesy of Ken K Nischal – fig. B; A Pearson – fig. C.)

pathogenesis is unclear, it has been proposed that tear stagnation

TIP Because congenital nasolacrimal obstruction with epiphora
secondary to inflammatory obstruction may precipitate stone for-
undergoes spontaneous resolution in 90% of babies by their first
mation, which tends to be associated with squamous metaplasia of birthday, initial probing should be delayed until this age.
the lacrimal sac epithelium. Presentation is often in late adulthood.
Symptoms may include intermittent epiphora, recurrent attacks of zzSigns
acute dacryocystitis and lacrimal sac distension. {z Epiphora (Fig. 3.18A) and matting of eyelashes may be
The lacrimal sac is distended and relatively firm, but is not constant or intermittent and may be particularly notice-
inflamed and tender as in acute dacryocystitis. able when the child has an upper respiratory tract infec-
Mucus reflux on pressure may or may not be present. Treat- tion. Superimposed bacterial conjunctivitis may be treated
ment involves a DCR.! with a broad-spectrum topical antibiotic.
{z Gentle pressure over the lacrimal sac may cause muco-
purulent reflux.
CONGENITAL OBSTRUCTION {z Acute dacryocystitis is very rare.
{z Normal visual function should be confirmed as far as pos-
Nasolacrimal duct obstruction sible and an anterior segment examination with assess-
The lower end of the nasolacrimal duct, in the region of the valve of ment of the red reflex performed.
Hasner, is the last portion of the lacrimal drainage system to cana- {z The fluorescein disappearance test (see above) is highly
lize, with complete patency most commonly occurring soon after specific in this setting. Only a fine line of dye, at most,
birth. Epiphora occurs in about 20 % of normal babies, but sponta- should remain at 5–10 minutes under inspection with a
neous resolution occurs in approximately 90% within the first year. blue light in a darkened room.

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 Lacrimal Drainage System
CHAPTER
3 109

zzDifferential diagnosis includes other congenital causes of a CHAPTER

watering eye, such as punctal atresia, congenital glaucoma,
chronic conjunctivitis (e.g. chlamydial), keratitis and uveitis.
zzTreatment
{z Massage of the lacrimal sac has been suggested as a means
of rupturing a membranous obstruction by hydrostatic
pressure. The index finger is initially placed over the com-
mon canaliculus to block reflux and then rolled over the
sac, massaging downwards. The likelihood of success and
the optimal regimen is undetermined.
{z Probing. Passage of a fine wire via the canalicular system
and nasolacrimal duct (Fig. 3.18B) to disrupt the obstruc-
tive membrane at the valve of Hasner is usually regarded as
the definitive treatment and may be preceded and followed
by irrigation to confirm the site of obstruction and subse- A
quent patency respectively. Probing can be repeated if a first
procedure is unsuccessful. Nasal endoscopic guidance may
enhance success and should be considered at least for repeat
procedures. If symptoms are mild–moderate, probing may
be delayed until the age of 12–18, or even 24, months and
is carried out under general anaesthesia. For more marked
symptoms, early probing may be appropriate and in young
children is sometimes performed under topical anaesthesia
in an outpatient setting. Risks include the induction of cana-
licular stenosis due to probe trauma, which may be relatively
common. It should be noted that there is little evidence of
a difference in final outcome at 24 months conferred by
intervention versus non-intervention. Failure of probing
may result from abnormal anatomy, which can usually be
recognized by di%culty in passing the probe and subsequent
non-patency of the drainage system on irrigation. B
{z Options after probing failure include intubation with
silicone tubing with or without balloon dilatation of
the nasolacrimal duct, endoscopic procedures and
dacryocystorhinostomy.!

Congenital dacryocoele
A congenital dacryocoele (amniontocoele) is a collection of amni-
otic fluid or mucus in the lacrimal sac caused by an imperforate
Hasner valve. Presentation is perinatal with a bluish cystic swell-
ing at or below the medial canthus (Fig. 3.18C), accompanied by
epiphora. If an intranasal component is large, it can cause respira-
tory distress. It should not be mistaken for an encephalocoele, the
latter being characterized by a pulsatile swelling above the medial
canthal tendon. Resolution is common with only conservative
treatment, but if this fails, probing is usually adequate. C

Fig. 3.19 Chronic canaliculitis. (A) Gram-stain of Actinomyces

TIP A congenital dacryocoele should not be confused with an israelii; (B) mucopurulent discharge on pressure over an in-
encephalocoele, which is characterized by a pulsatile swelling flamed upper canaliculus; (C) sulfur concretions released by
above the medial canthal ligament.
! canaliculotomy.

(Fig. 3.19A). Occasionally scarring and canalicular obstruction

CHRONIC CANALICULITIS may result. Presentation is with unilateral epiphora associated
with chronic mucopurulent conjunctivitis refractory to conven-
Chronic canaliculitis is an uncommon condition, frequently tional treatment. There is pericanalicular redness and oedema
caused by Actinomyces israelii, anaerobic Gram-positive bacteria and mucopurulent discharge on pressure over the canaliculus

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110 Dacryocystitis

A B

C D

Fig. 3.20 (A) Acute dacryocystitis; (B) lacrimal abscess and preseptal cellulitis; (C) lacrimal
fistula with fluorescein; (D) healed lacrimal fistula (arrow).

(Fig. 3.19B). A ‘pouting’ punctum (see Fig. 3.3F) may be a diag-

nostic clue in mild cases. In contrast to dacryocystitis, there is no
DACRYOCYSTITIS
lacrimal sac involvement. Concretions (sulfur granules) are meta- Infection of the lacrimal sac is usually secondary to obstruction of
bolic products of Actinomyces and other hydrogen sulfide-utilizing the nasolacrimal duct. It may be acute or chronic and is most com-
bacteria and classically are expressed on canalicular compression monly staphylococcal or streptococcal.
or following canaliculotomy (Fig. 3.19C). A topical antibiotic such
as a fluoroquinolone four times daily for 10 days may be tried ini-
tially, but is rarely curative unless combined with canaliculotomy
Acute dacryocystitis
(a linear incision into the conjunctival side of the canaliculus) and Presentation is with the subacute onset of pain in the medial
curettage of concretions. Giant fornix syndrome (see Ch. 6), dac- canthal area, associated with epiphora. A very tender, tense red
ryolithiasis and lacrimal diverticulum may give a similar clinical swelling develops at the medial canthus (Fig. 3.20A), commonly
picture. Herpes simplex is a classic cause of acute – as opposed to progressing to abscess formation (Fig. 3.20B). There may be asso-
chronic – canaliculitis. ciated preseptal cellulitis.
zzTreatment
{z Initial treatment involves the application of warm com-
TIP Chronic canaliculitis should be suspected in a patient presses and oral antibiotics such as flucloxacillin or co-
with unilateral mucopurulent conjunctivitis that is refractory to
amoxiclav. Irrigation and probing should not be performed.
conventional treatment.
!

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 Lacrimal Drainage System
CHAPTER
3 111

CHAPTER

A B

Fig. 3.21 (A) Mucocoele; (B) expression of mucopurulent material by applying pressure to the
sac (arrow).

{z Incision and drainage may be considered if pus points and is usually evident as a painless swelling at the inner canthus (Fig.
an abscess is about to drain spontaneously. However, this 3.21A), but if an obvious swelling is absent pressure over the sac
carries the risk of a persistent sac–skin fistula (Fig. 3.20C). commonly still results in mucopurulent canalicular reflux (Fig.
{z Dacryocystorhinostomy is commonly required after the 3.21B). Treatment is with a dacryocystorhinostomy.
acute infection has been controlled and may reduce the
risk of recurrent infection and can result in closure of a
fistula (Fig. 3.20D).!
TIP To reduce the risk of endophthalmitis postpone
intraocular surgery if there are any signs of lacrimal drainage
system infection.
Chronic dacryocystitis
Presentation is with chronic epiphora, which may be associated
with a chronic or recurrent unilateral conjunctivitis. A mucocoele

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 Chapter

Orbit 4
INTRODUCTION 114 Granulomatosis with Optic nerve sheath
Anatomy 114 polyangiitis 130 meningioma 145
Clinical features 114 Plexiform neurofibroma 145
NON-NEOPLASTIC VASCULAR
Investigation 117 Isolated neurofibroma 145
ABNORMALITIES 131
THYROID EYE DISEASE 117 Cavernous sinus thrombosis 131 LYMPHOMA 147
Introduction 117 Carotid–cavernous fistula 131
RHABDOMYOSARCOMA 148
Clinical features 119 CYSTIC LESIONS 133
Investigation 121 METASTATIC TUMOURS 149
Dacryops 133
Treatment 122 Dermoid cyst 133 Adult metastatic tumours 149
Sinus mucocoele 135 Childhood metastatic tumours 150
INFECTIONS 124
Encephalocoele 135 Orbital invasion from adjacent
Chandler–Hubert classification structures 150
of orbital complications of sinus VASCULAR TUMOURS 136 Orbital invasion from eyelid,
infection 124 conjunctival and intraocular
Varices 136
Preseptal cellulitis 124 tumours 151
Lymphangioma 136
Bacterial orbital cellulitis 125
Capillary haemangioma 138 SILENT SINUS SYNDROME 152
Rhino-orbital mucormycosis 126
Cavernous haemangioma 139
NON-INFECTIVE INFLAMMATORY THE ANOPHTHALMIC SOCKET 152
LACRIMAL GLAND TUMOURS 141
DISEASE 127 Surgical procedures 152
Pleomorphic lacrimal gland
Idiopathic orbital inflammatory Rehabilitation 152
adenoma 141
disease 127
Lacrimal gland carcinoma 142 CRANIOSYNOSTOSES 155
Orbital myositis 128
Acute dacryoadenitis 130 NEURAL TUMOURS 144
Tolosa–Hunt syndrome 130 Optic nerve glioma 144

113
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114 Introduction

{z Inflammation of the superior orbital fissure and apex

INTRODUCTION (Tolosa–Hunt syndrome) may therefore result in a multi-
tude of signs including ophthalmoplegia and venous out-
Anatomy flow obstruction.
The orbit is a pear-shaped cavity, the stalk of which is the optic zzThe inferior orbital fissure lies between the greater wing
canal (Fig. 4.1). of the sphenoid and the maxilla, connecting the orbit to the
zzThe roof consists of two bones: the lesser wing of the sphenoid pterygopalatine and infratemporal fossae. Through it run the
and the orbital plate of the frontal bone. It is located subjacent maxillary nerve, the zygomatic nerve and branches of the ptery-
to the anterior cranial fossa and the frontal sinus. A defect in gopalatine ganglion, as well as the inferior ophthalmic vein.
the orbital roof may cause pulsatile proptosis due to transmis- zzThe sensory supply of the eye and orbit is shown in Fig. 4.3.!
sion of cerebrospinal fluid pulsation to the orbit.
zzThe lateral wall also consists of two bones: the greater wing of
the sphenoid and the zygomatic. The anterior half of the globe
Clinical features
is vulnerable to lateral trauma since it protrudes beyond the
lateral orbital margin. Symptoms
zzThe floor consists of three bones: the zygomatic, maxillary and Symptoms of orbital disease include eyelid and conjunctival swell-
palatine. The posteromedial portion of the maxillary bone is ing, redness, watering, pain (sometimes on, or exacerbated by,
relatively weak and may be involved in a ‘blowout’ fracture (see eye movement), increasing ocular prominence, displacement or
Ch. 22). The orbital floor also forms the roof of the maxillary a sunken impression of the eye, double vision and blurring and
sinus so that maxillary carcinoma invading the orbit may dis- sometimes a pulsing sensation or audible bruit.!
place the globe upwards.
zzThe medial wall consists of four bones: maxillary, lacrimal, Soft tissue involvement
ethmoid and sphenoid. The lamina papyracea, which forms Eyelid and periocular oedema, skin discoloration, ptosis, chemo-
part of the medial wall, is paper-thin and perforated by numer- sis (oedema of the conjunctiva, which may involve the plica and
ous foramina for nerves and blood vessels. Orbital cellulitis is caruncle) and epibulbar injection (Fig. 4.4) may be seen. Causes
therefore frequently secondary to ethmoidal sinusitis. include thyroid eye disease, orbital inflammatory diseases and
zzThe superior orbital fissure is a slit linking the cranium and the obstruction to venous drainage.!
orbit, between the greater and lesser wings of the sphenoid bone.
A number of important structures pass through it (Fig. 4.2). Proptosis
{z The superior portion contains the lacrimal, frontal and Proptosis (Fig. 4.5) describes an abnormal protrusion of an organ,
trochlear nerves and the superior ophthalmic vein. but is generally applied to the eyeball; exophthalmos refers specifi-
{z The inferior portion contains the superior and inferior cally to the eyeball only. Proptosis may be caused by retrobulbar
divisions of the oculomotor nerve, the abducens and naso- lesions or, less frequently, a shallow orbit. The intraorbital portion
ciliary nerves and sympathetic fibres from the cavernous of the optic nerve is longer (25 mm) than the distance between
plexus. the back of the globe and the optic canal (18 mm). This allows for

Optic foramen Supraorbital notch

Frontal

Trochlear notch

Lesser and greater Ethmoid

wings of sphenoid Anterior lacrimal crest
Lacrimal
Superior and inferior Palatine
orbital fissures

Zygomatic Infraorbital foramen

Infraorbital groove Zygomatic–maxillary suture

Maxillary
Fig. 4.1 Anatomy of the orbit.

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 4
CHAPTER
Orbit 115

Lacrimal branch of ophthalmic nerve [V1]

Frontal branch of ophthalmic nerve [V1]
Trochlear nerve [IV]

Optic nerve
Optic canal
Ophthalmic artery
Superior branch of
oculomotor nerve [Ill]
Nasociliary branch
of ophthalmic nerve [V1]
Abducent nerve [VI]

Common tendinous ring

Inferior branch of
oculomotor nerve [Ill] Fig. 4.4 Chemosis and injection in orbital disease.

Inferior ophthalmic vein

Superior ophthalmic vein

Lateral Medial
Fig. 4.2 Innervation of the orbit and eyeball.

Posterior ethmoidal nerve

Anterior ethmoidal nerve

lnfratrochlear nerve
Medial rectus muscle
Long ciliary nerves
Short ciliary nerves
Lacrimal gland Fig. 4.5 Proptosis.

Lateral
significant forward displacement of the globe (proptosis) without
Lacrimal nerve excessive stretching of the nerve.
(from [V1])
zzAsymmetrical proptosis is readily detected by looking down
Lateral rectus
at the patient from above and behind (Fig. 4.6A).
Ciliary ganglion zzThe direction of proptosis may indicate the likely pathology.
Abducent nerve For example, space-occupying lesions within the muscle cone
[VI] such as a cavernous haemangioma or optic nerve tumours
Inferior branch of cause axial proptosis, whereas extraconal lesions usually give
the oculomotor rise to combined proptosis and dystopia (see next).
nerve [III]
zzDystopia implies displacement of the globe in the coronal
Nasociliary plane, usually due to an extraconal orbital mass such as a lac-
nerve (from [V1]) rimal gland tumour (Fig. 4.6B). Horizontal displacement is
measured from the midline (nose) to the centre of the pupil
Superior branch while vertical dystopia is read on a vertical scale perpendicular
of the oculomotor to a horizontal rule placed over the bridge of the nose. The
nerve [III] measured eye should fixate straight ahead; if necessary, this
can be facilitated by occluding the fellow eye.
zzThe severity of proptosis can be measured with a plas-
Fig. 4.3 Sensory nerve supply of the orbit. tic rule resting on the lateral orbital margin, or with the

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116 Introduction

Measurements can be taken both relaxed and with the Valsalva

manoeuvre. Readings greater than 20 mm are indicative of
proptosis and a difference of 2–3 mm or more between the two
eyes is suspicious regardless of the absolute values. The dimen-
sions of the palpebral apertures and any lagophthalmos should
also be noted.
zzPseudoproptosis (the false impression of proptosis) may
be due to facial asymmetry, enlargement of the globe (e.g.
high myopia or buphthalmos), lid retraction or contralateral
enophthalmos.!

Enophthalmos
Enophthalmos implies recession of the globe within the orbit.
Causes include congenital and traumatic orbital wall abnormali-
A ties, atrophy of the orbital contents (e.g. radiotherapy, scleroderma,
chronic eye poking in blind infants – the ‘oculodigital’ sign) or
sclerosis (e.g. metastatic scirrhous carcinoma, sclerosing orbital
inflammatory disease). Pseudoenophthalmos may be caused by a
small or shrunken eye (microphthalmos or phthisis bulbi), by pto-
sis, or by contralateral proptosis or pseudoproptosis.!

Ophthalmoplegia
Defective ocular motility is very common in orbital disease. Causes
include an orbital mass, restrictive myopathy (e.g. thyroid eye dis-
ease – Fig. 4.7, orbital myositis, tethering of muscles or tissue a%er
orbital wall fracture), ocular motor nerve involvement associated
with lesions in the cavernous sinus, orbital fissures or posterior orbit
(e.g. carotid–cavernous fistula, Tolosa–Hunt syndrome, malignant
lacrimal gland tumours). The following tests may be used to differ-
entiate a restrictive from a neurological motility defect:
zzForced duction test. Under topical anaesthesia, the insertion
B
of the muscle in an involved eye is grasped with forceps and
the globe rotated in the direction of reduced mobility. Checked
movement of the globe indicates a restrictive problem. No
resistance will be encountered with a neurological lesion.
zzDifferential intraocular pressure (IOP) test involves less
discomfort than forced duction and an objective rather than
subjective endpoint. The IOP is measured in the primary posi-
tion of gaze and then with the patient attempting to look in the
direction of limited mobility. An increase of 6 mmHg or more
denotes resistance transmitted to the globe by muscle restric-
tion (the Braley sign).
zzSaccadic eye movements in neurological lesions are reduced
in velocity, while restrictive defects manifest normal saccadic
velocity with sudden halting of ocular movement.!

Dynamic properties
C
zzIncreasing venous pressure by dependent head position, the
Fig. 4.6 General signs of orbital disease. (A) Left proptosis Valsalva manoeuvre or jugular compression may induce or
visualized from above; (B) right inferior dystopia; (C) meas- exacerbate proptosis in patients with orbital venous anomalies
urement of proptosis with an exophthalmometer. or infants with orbital capillary haemangioma.
zzPulsation is caused either by an arteriovenous communica-
Luedde exophthalmometer using a similar principle. Com- tion or a defect in the orbital roof. In the former, pulsation
monly, a binocular exophthalmometer (e.g. Hertel Oculus) is may be associated with a bruit depending on the size of the
employed, using visualization of the corneal apices to deter- communication. In the latter the pulsation is transmitted from
mine the degree of ocular protrusion from a scale (Fig. 4.6C). the brain by the cerebrospinal fluid and there is no associated

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Orbit 117

Fig. 4.7 Restrictive myopathy, bilateral lid retraction and proptosis in thyroid eye disease –
nine positions of gaze. (Courtesy of C Barry.)

bruit. Mild pulsation is best detected on the slit lamp, particu- It is of particular value in patients with orbital trauma because
larly by applanation tonometry. it can detect small fractures, foreign bodies, blood, herniation
zzA bruit is a sign found with a larger carotid–cavernous fistula. of extraocular muscle and emphysema (see Ch. 22). It is, how-
It is best heard with the bell of the stethoscope and is lessened ever, unable to distinguish different pathological so% tissue
or abolished by gently compressing the ipsilateral carotid masses that are radiologically isodense. Confirmation of an
artery in the neck.! orbital abscess in cellulitis is a relatively common indication.
zzMagnetic resonance imaging (MRI) can demonstrate orbital
Fundus changes apex lesions and intracranial extension of orbital tumours and
zzOptic disc swelling may be the initial feature of compressive is useful for imaging orbital inflammatory disease. Serial short
optic neuropathy (Fig. 4.8A). T1 inversion recovery (STIR) scans are valuable in assessing
zzOptic atrophy (Fig. 4.8B), which may be preceded by swelling, inflammatory activity in thyroid eye disease (see Ch. 19).
is a feature of severe compressive optic neuropathy. Important zzPlain X-rays are little used except for the initial diagnosis of
causes include thyroid eye disease and optic nerve tumours. traumatic bony injury.
zzOpticociliary collaterals consist of enlarged pre-existing peri- zzUltrasonography can provide useful information, particularly
papillary capillaries that divert blood from the central retinal with high-grade apparatus and an experienced operator, but
venous circulation to the peripapillary choroidal circulation does not image the orbital apex well.
when there is obstruction of the normal drainage channels. On zzFine-needle biopsy is sometimes performed, particularly
ophthalmoscopy the vessels appear as large tortuous channels in suspected neoplastic disease. Potential problems include
most frequently sited temporally, which disappear at the disc haemorrhage and ocular penetration.
margin (Fig. 4.8C). The collaterals may be associated with any
orbital or optic nerve tumour that compresses the intraorbital TIP The most common tumour associated with opticociliary
optic nerve and impairs blood flow through the central retinal
vein. The most common tumour associated with shunts is an
shunt vessels on the disc is an optic nerve sheath meningioma.
!
optic nerve sheath meningioma but they may also occur with
optic nerve glioma, central retinal vein occlusion, idiopathic
intracranial hypertension and glaucoma.
THYROID EYE DISEASE
zzChoroidal folds (Fig. 4.8B and D) are discussed in detail in
Chapter 14. They may occur in a wide variety of orbital lesions.
Introduction
Although tending to be more common with greater amounts Thyroid eye disease (TED), also known as thyroid-associated orbi-
of proptosis and anteriorly located tumours, in some cases topathy and Graves ophthalmopathy, is a common orbital disorder
their presence can precede the onset of proptosis.! and is the most common cause of both bilateral and unilateral pro-
ptosis in an adult.

Investigation Thyrotoxicosis
zzComputed tomography (CT) is useful for depicting bony Thyrotoxicosis (hyperthyroidism) is a condition involving exces-
structures and the location and size of space-occupying lesions. sive secretion of thyroid hormones. Graves disease, the most

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118 Thyroid Eye Disease

A B

C D

Fig. 4.8 Fundus changes in orbital disease. (A) Disc swelling; (B) optic atrophy with choroidal
folds; (C) optociliary vessels in the presence of optic atrophy; (D) choroidal folds.

common form of hyperthyroidism, is an autoimmune disorder Risk factors for ophthalmopathy

in which IgG antibodies bind to receptors in the thyroid gland Once a patient has Graves disease, the major clinical risk factor for
and stimulate secretion of thyroid hormones. It is more common developing TED is smoking. The greater the number of cigarettes
in females and may be associated with other autoimmune dis- smoked per day, the greater the risk and giving up smoking seems
orders. Presentation is o%en in the fourth or fi%h decades with to reduce the risk. Women are five times more likely to be affected
symptoms including weight loss despite good appetite, increased by TED than men, but this largely reflects the increased incidence
bowel frequency, sweating, heat intolerance, nervousness, irrita- of Graves disease in women. Radioactive iodine used to treat
bility, palpitations, weakness and fatigue. There may be enlarge- hyperthyroidism can worsen TED. This condition can also, though
ment of the thyroid gland (Fig. 4.9A), tremor, palmar erythema less commonly, occur in euthyroid and hypothyroid (including
and warm and sweaty skin. Thyroid acropachy is a phenomenon treated hyperthyroid) patients. It can sometimes be the presenting
similar to clubbing of the fingers (Fig. 4.9B), occurring in 1%; manifestation of thyroid-related disease.!
pretibial myxoedema (1–5%) is indurated thickening of the skin
of the shins. Cardiac manifestations may include sinus tachycar- Pathogenesis of ophthalmopathy
dia and other arrhythmias. Other autoimmune disorders can be Thyroid ophthalmopathy involves an organ-specific autoim-
associated. Thyroid function is commonly tested initially with a mune reaction in which anti-thyrotropin-receptor antibody reacts
TSH level. If this is low, or normal but thyroid disease is still against thyroid gland cells and orbital fibroblasts. Insulin-like
suspected, a range of additional investigations can be carried growth factor 1 receptor (IGF-1R) and the thyroid-stimulating
out. Treatment options include carbimazole, propylthiouracil, hormone receptor (TSHR) are upregulated in orbital and brow
propranolol, thyroid ablation with radioactive iodine and partial fat. This leads to inflammation of extraocular muscles, interstitial
thyroidectomy.! tissues, orbital fat and lacrimal glands. A pleomorphic cellular

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 4
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Orbit 119

A B
Fig. 4.9 Systemic signs in thyrotoxicosis. (A) Goitre; (B) acropachy.

infiltration and increased secretion of glycosaminoglycans leads to {z Moderate–severe, with one of moderate–severe so% tissue
osmotic imbibition of water. The volume of the orbital contents involvement, lid retraction of 2 mm or more, diplopia and
increases and the muscles can swell up to eight times their normal proptosis of 3 mm or more.
size. There is also lipogenesis induced by fibroblasts and preadipo- {z Sight-threatening due to optic neuropathy or corneal
cytes, which causes orbital fat enlargement. There may be a sec- breakdown.!
ondary elevation of intraorbital pressure and the optic nerve may
be compressed. Subsequent degeneration of muscle fibres eventu- Soft tissue involvement
ally leads to fibrosis, which exerts a tethering effect on the involved zzSymptoms. Grittiness, red eyes, lacrimation, photophobia,
muscle, resulting in restrictive myopathy and diplopia.! puffy lids and retrobulbar discomfort.
zzSigns may include:
{z Epibulbar hyperaemia. This is a sensitive sign of inflam-
Clinical features matory activity. Intense focal hyperaemia may outline the
insertions of the horizontal recti (Fig. 4.10A).
Introduction {z Periorbital swelling is caused by oedema and infiltration
TED typically proceeds through a congestive (inflammatory) stage behind the orbital septum. This may be associated with
in which the eyes are red and painful. This tends to remit within chemosis and prolapse of retroseptal fat into the eyelids
1–3 years and only about 10% of patients develop serious long- (Fig. 4.10B).
term ocular problems. A fibrotic (quiescent) stage follows in which {z Tear insufficiency and instability is common.
the eyes are white, although a painless motility defect may be pres- {z Corneal signs are exacerbated by lid retraction (see next)
ent. Clinical features broadly can be categorized into: (1) so% tissue and can include punctate epithelial erosions, superior lim-
involvement, (2) lid retraction, (3) proptosis, (4) optic neuropathy bic keratoconjunctivitis (Fig. 4.10C and see Ch. 7) and
and (5) restrictive myopathy.! occasionally bacterial keratitis, thinning and scarring.!

European Group on Graves Orbitopathy Lid retraction

(EUGOGO) Classification (2021) Retraction of upper and lower lids occurs in about 50% of patients
zzActivity with Graves disease. Overaction of Müller muscle is postulated
{z Spontaneous retrobulbar pain. to occur as a result of sympathetic overstimulation secondary to
{z Pain on attempted upward or downward gaze. high levels of thyroid hormones. Fibrotic contracture of the levator
{z Redness of eyelids. palpebrae and inferior rectus muscles associated with adhesion to
{z Redness of conjunctiva. overlying orbital tissues is another probable mechanism, together
{z Swelling of conjunctiva or plica. with secondary overaction in response to hypo- or hypertropia
{z Swelling of eyelids. produced by fibrosis.
{z Chemosis. zzSymptoms. Patients may complain of a staring or bulging-
zzSeverity eyed appearance, difficulty closing the eyes and ocular surface
{z Mild, with only a minor impact on daily life. symptoms.

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120 Thyroid Eye Disease

C D

Fig. 4.10 Soft tissue involvement in thyroid eye disease. Fig. 4.11 Lid signs in thyroid eye disease. (A) Mild left lid re-
(A) Epibulbar hyperaemia overlying a horizontal rectus traction; (B) moderate bilateral asymmetrical lid retraction
muscle; (B) periorbital oedema, chemosis and prolapse of fat – Dalrymple sign; (C) severe bilateral lid retraction – Kocher
into the eyelids; (C) superior limbic keratoconjunctivitis. sign; (D) right lid lag on downgaze – von Graefe sign.

zzSigns Proptosis
{z The upper lid margin normally rests 2 mm below the zzSymptoms are similar to those of lid retraction.
limbus (Fig. 4.11A, right eye). Lid retraction is suspected zzSigns. Proptosis is axial, unilateral or bilateral, symmetri-
when the margin is either level with or above the superior cal (Fig. 4.12A) or asymmetrical (Fig. 4.12B) and frequently
limbus, allowing sclera to be visible (‘scleral show’; Fig. permanent. Severe proptosis may compromise lid closure and
4.11A, le% eye). along with lid retraction and tear dysfunction can lead to expo-
{z The lower eyelid margin normally rests at the inferior lim- sure keratopathy, corneal ulceration and infection (Fig. 4.12C).!
bus. Retraction is suspected when sclera shows below the
limbus. Lid retraction may occur in isolation or in associa- Restrictive myopathy
tion with proptosis, which exaggerates its severity. Between 30% and 50% of patients with TED develop ophthal-
{z The Dalrymple sign is lid retraction or lid spasm in pri- moplegia and this may be permanent. Ocular motility is restricted
mary gaze leading to a widened palpebral opening (Fig. initially by inflammatory oedema and later by fibrosis.
4.11B). zzSymptoms. Double vision and o%en discomfort in some posi-
{z The Kocher sign describes a staring and frightened appear- tions of gaze.
ance of the eyes, which is particularly marked on attentive zzSigns, in approximate order of frequency:
fixation (Fig. 4.11C). {z Elevation defect (Fig. 4.13A) caused by fibrotic contracture
{z The von Graefe sign signifies retarded descent of the upper of the inferior rectus, may mimic superior rectus palsy and
lid on downgaze (lid lag – Fig. 4.11D).! is the most common motility deficit.

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Orbit 121

A A

B B

C C

Fig. 4.12 Proptosis in thyroid eye disease. (A) Symmetrical; (B) Fig. 4.13 Restrictive thyroid myopathy. (A) Defective elevation
asymmetrical; (C) bacterial keratitis due to severe exposure. of the left eye secondary to fibrosis of the inferior rectus mus-
(Courtesy of ADN Murray – fig. C.) cle; (B) reduced abduction of the left eye secondary to fibro-
sis of the left medial rectus muscle; (C) defective depression
of the right eye secondary to fibrosis of the right superior
{z Abduction defect due to fibrosis of the medial rectus, rectus muscle.
which may simulate sixth nerve palsy (Fig. 4.13B).
{z Depression defect (Fig. 4.13C) secondary to fibrosis of the
superior rectus.
{z Colour desaturation is a sensitive feature.
{z Adduction defect caused by fibrosis of the lateral rectus.!
{z There may be diminished light brightness appreciation.
{z A relative afferent pupillary defect, if present, should give
Optic neuropathy
cause for marked concern.
Optic neuropathy is a fairly common (up to 6%) serious complica-
{z Visual field defects can be central or paracentral and may
tion caused by compression of the optic nerve or its blood supply at
be combined with nerve fibre bundle defects. These find-
the orbital apex by the congested and enlarged recti (Fig. 4.14A and
ings, in concert with elevated IOP, may be confused with
B) and swollen orbital tissue. Such compression, which may occur
primary open-angle glaucoma.
in the absence of significant proptosis, may lead to severe visual
{z The optic disc may be normal, swollen or, rarely, atrophic.!
impairment if adequate and timely treatment is not instituted.

TIP In thyroid eye disease, compression of the optic nerve Investigation

secondary to enlarged and congested rectus muscles may Investigations other than blood tests for thyroid disease are not
occur in the absence of proptosis.
necessary if the diagnosis is evident clinically, but the exclusion
of other conditions is sometimes indicated. Visual field testing is
zzSymptoms. Impairment of central vision occurs in conjunc- carried out if there is a suspicion of optic nerve compromise and
tion with other symptoms of TED. In order to detect early may be performed as part of a baseline evaluation even if there
involvement, patients should be advised to monitor their own is no apparent visual impairment. MRI, CT and ultrasonographic
visual function by alternately occluding each eye, reading imaging of the orbits are indicated in some circumstances, such as
small print and assessing the intensity of colours, for example helping to confirm an equivocal diagnosis by identification of the
on a television screen. typical pattern of extraocular muscle involvement in TED, consist-
zzSigns. A high index of suspicion should be maintained for ing of muscle belly enlargement with tendon sparing. Imaging is
optic neuropathy and it is important not to mistakenly attri- also used in the assessment of optic nerve compression and prior
bute disproportionate visual loss to minor disease. to orbital wall surgery. Visual evoked potentials are sometimes uti-
{z Visual acuity (VA) is usually reduced, but not invariably. lized in optic neuropathy.!

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122 Thyroid Eye Disease

Mild and active disease

zzLubricants for superior limbic keratoconjunctivitis, corneal
exposure and dryness.
zzTopical anti-inflammatory agents (steroids, non-steroidal
anti-inflammatory drugs (NSAIDs), ciclosporin) are advo-
cated by some authorities.
zzSelenium (in selenium deficient areas): sodium selenite 200 µg
(containing 91.2 µg selenium) daily for 6 months.
zzHead elevation with three pillows during sleep to reduce peri-
orbital oedema.
zzEyelid taping during sleep may alleviate mild exposure
keratopathy.!

A Moderate–severe active disease

zz‘Clinical activity score’. Using EUGOGO guidelines an activ-
ity score can be calculated to determine when immunosup-
pressives should be used. One point is assigned for each feature
that is present, using the activity classification (see above).
Treatment should be considered for a score of 3 or more out
of 7. During subsequent review, a point is allocated for an
increase in proptosis of 2 mm or more, a decrease in uniocular
excursion in any one direction of 8° or more, or a decrease in
Snellen acuity of one line (total 10).
zz Systemic steroids are the mainstay of treatment for moderate–
severe disease. Oral prednisolone 60–80 mg/day may be given
initially and tapered depending on response. Intravenous meth-
ylprednisolone is o%en reserved for acute compressive optic
neuropathy (see below), but tolerability is better, and outcomes
are superior, when compared with oral treatment. Intravenous
B steroids should not be given to patients with co-existing sig-
nificant hepatic dysfunction, significant cardiovascular disease,
Fig. 4.14 CT scan showing muscle enlargement in thyroid eye uncontrolled systemic hypertension or diabetes. A lower-
disease. (A) Axial view showing fusiform enlargement of the intensity regimen of intravenous corticosteroids in the absence
medial rectus muscle; (B) coronal view showing symmetrical
involvement of the medial, superior and inferior rectus of acute sight-threatening disease should be used in most cases
muscles as well as the superior oblique muscles. using the following regimen: 0.5 g once weekly for 6 weeks fol-
lowed by 0.25 g once weekly for 6 weeks. A reduction in discom-
fort, chemosis and periorbital oedema usually occurs within 24
hours, with a maximal response within 2–8 weeks. Ideally, oral
Treatment steroid therapy should be discontinued a%er several months,
zzThe choice of treatment depends on (a) clinical activity, (b) but long-term low-dose maintenance may be necessary.
severity and (c) duration of the orbitopathy. zzIntravenous corticosteroids weekly and mycophenolate
zzAnti-inflammatory/immunosuppressive treatment is sig- sodium (0.72 g/day for 24 weeks) have been shown to be of
nificantly less effective a%er 18 months of disease duration. greater benefit than intravenous corticosteroid monotherapy
Early referral to a specialized centre is fundamental for most and is the regimen of choice in specialized centres.
patients with moderate–severe active orbitopathy. Counselling zzOrbital steroid (triamcinolone acetate) injections are occa-
is needed to explain the aims and expectations, benefits and sionally used in selected cases to minimize systemic side
risks of different therapies. effects, but are typically considerably less effective than sys-
zzClassification. Treatment can be classified into that of: (a) temic treatment.
mild disease (most patients), (b) moderate–severe active dis- zzLow-dose fractionated radiotherapy is usually used as
ease and (c) treatment of post-inflammatory complications. second-line therapy. It may be used in addition to steroids or
zzCessation of smoking should be the first measure taken when steroids are contraindicated or ineffective, but because
in all cases. Thyroid dysfunction should also be managed of the delayed effect is not used as the sole treatment of acute
adequately. optic nerve compression. A positive response is usually evi-
zzIf radioiodine treatment is administered in patients with pre- dent within 6 weeks, with maximal improvement by 4 months;
existing TED, a short course of oral steroids should be given. around 40% will not respond. Adverse effects include cataract,

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CHAPTER
Orbit 123

radiation retinopathy, optic neuropathy and an increased risk

of local cancer. The threshold for its use should be higher in
younger patients and diabetics, the latter because of a possibly
increased risk of retinopathy.
zzCombined therapy with irradiation, azathioprine and low-
dose prednisolone may be more effective than steroids or
radiotherapy alone.

TIP Immunosuppression in the active inflammatory phase of

thyroid eye disease is important to reduce acute inflammation
and to prevent long-term complications.

zzOptic neuropathy, and less commonly intractable corneal

exposure, requires aggressive treatment. Pulsed intravenous
methylprednisolone is commonly used, regimens includ-
ing 0.5–1 g on three successive days with conversion to oral Fig. 4.15 Axial CT following bilateral lateral and medial wall
treatment (e.g. 40 mg/day prednisolone) or 0.5–1 g on alter- decompression. (Courtesy of A Pearson.)
nate days, 3–6 times, keeping the maximum dose below 8 g to
reduce the risk of liver compromise, followed by oral predniso-
lone. Appropriate monitoring should be instituted, including
be reached endoscopically. One-wall (deep lateral) decom-
liver function tests, as well as gastric protective treatment and
pression is effective (approximately 4–5 mm reduction in
osteoporosis prophylaxis if necessary. Orbital wall decompres-
proptosis) and may reduce the risk of postoperative diplopia.
sion (see below) and/or orbital apex decompression may be
Two-wall (balanced medial and lateral – Fig. 4.15) decom-
considered if steroids are ineffective (20% receiving intrave-
pression provides a greater effect but with a significant risk
nous treatment) or contraindicated. Orbital radiotherapy may
of inducing diplopia. Three-wall decompression includes the
also be administered, but is generally used only as an adjunct
floor with a reduction in proptosis of 6–10 mm but may lead to
to other modalities.
hypoglobus and carries a higher risk of infraorbital nerve dam-
zzSecond-line management for persistent, corticosteroid-
age and diplopia. Very severe proptosis may require removal of
resistant disease or non-response to first-line treatment
part of the orbital roof in addition (four-wall decompression).
includes the use of monoclonal antibody treatment with
zz Restrictive myopathy. Surgery is required in most cases expe-
rituximab, tocilizumab or teprotumumab. Teprotumumab is
riencing persistent diplopia in the primary or reading positions
an inhibitor of insulin-like growth factor 1 receptor (IGF-1R)
of gaze, provided the inflammatory stage has subsided and the
and is effective in reducing proptosis and the Clinical Activity
angle of deviation has been stable for at least 6–12 months. Until
Score in patients with acute TED. In addition, teprotumumab
these criteria are met, diplopia may be alleviated, if possible, with
appears to be effective in chronic non-inflammatory TED,
prisms or sometimes botulinum toxin. The goal of operative
resulting in a reduction of proptosis, diplopia and strabismus.
treatment is to achieve binocular single vision in the primary
This medication has been associated with mild to severe hear-
and reading positions. Restrictive myopathy o%en precludes
ing loss in some individuals.
binocularity in all positions of gaze, though with time the field
zzPost-inflammatory complications. Eyelid surgery should be
of binocular single vision may enlarge as a result of increasing
performed only a%er any necessary orbital and then strabis-
fusional vergence. Recession of the inferior and/or medial recti is
mus procedures have been undertaken, as orbital decompres-
the most commonly indicated surgery (a rectus muscle is never
sion may impact both ocular motility and eyelid position, and
resected, only recessed in TED), generally utilizing adjustable
extraocular muscle surgery may affect eyelid position.
sutures (see Ch. 18). The suture is adjusted later the same day
or on the first postoperative day to achieve optimal alignment.
TIP Surgical orbital decompression should be considered zz Lid retraction. Mild lid retraction frequently improves spon-
when there is sight-threatening compressive optic neuropathy taneously so does not require treatment. Control of hyperthy-
in thyroid eye disease.
roidism may also be beneficial. Botulinum toxin injection to the
levator aponeurosis and Müller muscle may be used as a tem-
zzProptosis. A%er active inflammation has remitted, the patient porary measure in patients awaiting definitive correction. Mül-
can be le% with cosmetically and functionally significant pro- lerotomy (disinsertion of Müller muscle) is effective for mild lid
ptosis, the treatment of which is essentially surgical. Surgical retraction, but more severe cases may also require recession/
decompression increases the volume of the orbit by removing disinsertion of the levator aponeurosis and the suspensory liga-
the bony walls and may be combined with removal of orbital ment of the superior conjunctival fornix. Recession of the lower
fat. Most surgery is undertaken via an external approach, lid retractors, with or without a hard palate gra%, can be used
though the medial wall and the medial part of the floor can when retraction of the lower lid is 2 mm or more (see also Ch. 2).!

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124 Infections

meningitis and cavernous sinus thrombosis. Rapid progression

INFECTIONS to orbital cellulitis may occasionally occur. Organisms typically
responsible are Staphylococcus aureus and Streptococcus pyogenes,
Chandler–Hubert classification of with causes including skin trauma such as laceration or insect
orbital complications of sinus infection bites, spread from focal ocular or periocular infection such as an
acute hordeolum, dacryocystitis, conjunctivitis or sinusitis and
The classification helps to determine management and prognosis
haematogenous spread from remote infection such as the upper
(Fig. 4.16).
zzType I: preseptal cellulitis
respiratory tract or middle ear.!
zzType II: post-septal cellulitis
zzType III: subperiosteal abscess
Diagnosis
zzType IV: orbital abscess
The condition manifests with a swollen, o%en firm, tender red eye-
zzType V: cavernous sinus thrombosis!
lid that may be very severe (Fig. 4.17A and B). However, in con-
trast to orbital cellulitis, proptosis and chemosis are absent and
VA, pupillary reactions and ocular motility are unimpaired. The
Preseptal cellulitis patient is o%en pyrexial. Imaging with MRI or CT is not indicated
unless orbital cellulitis or a lid abscess is suspected, or if there is a
failure to respond to therapy.!
Introduction
Preseptal cellulitis is an infection of the subcutaneous tissues ante- Treatment
rior to the orbital septum. It is considerably more common than Treatment is with oral antibiotics such as co-amoxiclav 250–500
orbital cellulitis and though regarded as less serious, can still be mg/125 mg 2–3 times daily or 875/125 mg twice daily, depending
associated with severe complications such as abscess formation,

I II

III IV

Fig. 4.16 Chandler–Hubert classification of orbital cellulitis secondary to sinus infection (see
text; Type V is not illustrated).

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CHAPTER
Orbit 125

A B

C D

Fig. 4.17 Orbital cellulitis. (A) Left preseptal cellulitis resulting from an infected eyelid abrasion;
(B) axial CT showing opacification anterior to the orbital septum; (C) right orbital cellulitis with
ophthalmoplegia; (D) axial CT showing both preseptal and orbital opacification.

on severity. Severe infection may require intravenous antibiotics. The trauma, including any form of ocular surgery. Blood-borne spread
patient’s tetanus status should be ascertained in cases following trauma. from infection elsewhere in the body may occur.!

TIP Visual acuity, pupillary responses, and ocular motility are

Clinical features
zzSymptoms consist of the rapid onset of pain exacerbated by
unimpaired, and proptosis is absent in preseptal cellulitis.
! eye movement, swelling of the eye, malaise and frequently
visual impairment and double vision. There is commonly a
recent history of nasal, sinus or respiratory symptoms.
Bacterial orbital cellulitis zzSigns
{z Pyrexia, o%en marked.
Introduction {z VA may be reduced and colour vision impaired, raising the
Bacterial orbital cellulitis is a serious infection of the so% tissues possibility of optic nerve compression. The presence of a
behind the orbital septum, which can be sight- and life-threatening. relative afferent pupillary defect in a previously normal eye
It can occur at any age but is more common in children. Streptococ- makes this almost certain.
cus pneumoniae, Staphylococcus aureus, Streptococcus pyogenes and {z Tender, firm, erythematous and warm eyelids, with peri-
Haemophilus influenzae are common causative organisms, with ocular and conjunctival (chemosis) oedema, conjunctival
infection originating typically from the paranasal (especially eth- injection and sometimes subconjunctival haemorrhage.
moid) sinuses. Infection can also spread from preseptal cellulitis, The signs are usually unilateral, though oedema may
dacryocystitis, midfacial skin or dental infection and can follow spread to the contralateral eyelids (Fig. 4.17C).

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126 Infections

{z Proptosis is common in established infection, but is o%en

Table 4.1 Differential Diagnosis of an Acutely Inflamed
obscured by lid swelling. It may be non-axial (dystopia), Orbit
particularly if an abscess is present.
{z Painful ophthalmoplegia.
Infection
{z Choroidal folds and optic disc swelling may be present on • Bacterial orbital cellulitis
fundus examination. • Fungal orbital infection
• Dacryocystitis
zzDifferential diagnosis. Major diagnostic alternatives are listed
• Infective dacryoadenitis
in Table 4.1.
Vascular lesions
zzComplications
{z Ocular complications include optic neuropathy, exposure • Acute orbital haemorrhage
• Cavernous sinus thrombosis
keratopathy, raised IOP, endophthalmitis and occlusion of
• Carotid–cavernous fistula
the central retinal artery or vein.
{z Subperiosteal abscess, most frequently located along the
Neoplasia
medial orbital wall. • Rapidly progressive retinoblastoma
{z Intracranial complications, which are uncommon (3–4%) • Lacrimal gland tumour
• Other neoplasm, e.g. metastatic lesion with
but extremely serious, include meningitis, brain abscess inflammation, lymphoma, Waldenström
and cavernous sinus thrombosis.! macroglobulinaemia
• Rhabdomyosarcoma, leukaemia, lymphangioma or
Investigation neuroblastoma in children
Investigations may include: Endocrine
zzAscertainment of tetanus immunization status in cases of • Thyroid eye disease of rapid onset
trauma.
Non-neoplastic inflammation
zzWhite cell count.
zzBlood cultures. • Idiopathic orbital inflammatory disease
• Tolosa–Hunt syndrome
zzCulture of nasal discharge.
• Orbital myositis
zzHigh-resolution CT of the orbit, sinuses and brain (Fig. • Acute allergic conjunctivitis with lid swelling
4.17B and D) is vital to confirm the diagnosis and exclude a • Herpes zoster ophthalmicus
subperiosteal or intracranial abscess. MRI is also sometimes • Herpes simplex skin rash
performed. • Sarcoidosis
• Vasculitides: granulomatosis with polyangiitis,
zzLumbar puncture if meningeal or cerebral signs develop.!
polyarteritis nodosa
• Scleritis, including posterior scleritis
Treatment • Ruptured dermoid cyst
zzHospital admission is mandatory, with urgent otolaryngo-
logical assessment and frequent ophthalmic review. Paediat-
ric specialist advice should be sought in the management of
a child and a low threshold should be adopted for infectious
disease specialist consultation. TIP Because orbital cellulitis often occurs as a consequence
zzDelineation of the extent of erythema on the skin using a sur- of a sinus infection, urgent radiological investigation of the
gical marker may help in judging progress. sinuses is mandatory. !
zzAntibiotics are given intravenously, with the specific drug
depending on local sensitivities. Ce%azidime is a typical
choice, supplemented by oral metronidazole to cover anaer-
Rhino-orbital mucormycosis
obes. Intravenous antibiotics should be continued until the
patient has been apyrexial for 4 days, followed by 1–3 weeks Introduction
of oral treatment. Mucormycosis is a rare aggressive and o%en fatal infection caused
zzMonitoring of optic nerve function is performed at least by fungi of the family Mucoraceae. It typically affects patients with
every 4 hours initially by testing VA, colour vision, light diabetic ketoacidosis or immunosuppression and is extremely rare
brightness appreciation and pupillary reactions. Deterioration in the immunocompetent. Infection is acquired by the inhalation
should prompt the consideration of surgical intervention. of spores, which give rise to an upper respiratory infection. Spread
zzSurgery. Drainage of an orbital abscess should be considered then occurs to the contiguous sinuses and subsequently to the
at an early stage. Drainage of infected sinuses should be con- orbit and brain. Invasion of blood vessels by the hyphae results in
sidered if there is a lack of response to antibiotics, or if there occlusive vasculitis with infarction of orbital tissues.!
is very severe sinus disease. Biopsy of inflammatory tissue
may be performed for an atypical clinical picture. Severe optic Diagnosis
nerve compression may warrant an emergency canthotomy/ zzSymptoms. Gradual onset facial and periorbital swelling, dip-
cantholysis (see Ch. 22). lopia and visual loss.

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Orbit 127

Diagnosis
zzSymptoms typically consist of acute or subacute ocular and
periocular redness, swelling and pain (Fig. 4.19A and B). Sys-
temic symptoms are common in children.
zzSigns
{z Pyrexia is present in up to 50% of children, but is rare in
adults.
{z Congestive proptosis.
{z Mild to severe ophthalmoplegia may occur.
{z Features of optic nerve dysfunction, particularly if the
inflammation involves the posterior orbit. There may be
optic disc swelling.
{z Choroidal folds, if present, may be associated with reduced
vision but optic neuropathy must always be suspected.
zzCourse. The natural history of the inflammatory process is

Fig. 4.18 Necrosis of the eyelid in rhino-orbital mucormy- very variable.

cosis. {z Spontaneous remission a%er a few weeks without sequelae.
{z Intermittent episodes of activity, usually with eventual
remission.
{z Severe prolonged inflammation eventually leading to pro-
zzSigns are similar to bacterial orbital cellulitis, but tend to be
gressive fibrosis of orbital tissues, resulting in a ‘frozen
less acute and with slower progression. Infarction superim-
orbit’ characterized by ophthalmoplegia, which may be
posed on septic necrosis is responsible for the classic black
associated with ptosis and visual impairment caused by
eschar that may develop on the palate, turbinates, nasal sep-
optic nerve involvement.
tum, skin and eyelids (Fig. 4.18).
zzInvestigation
zzComplications include retinal vascular occlusion, multiple
{z CT shows ill-defined orbital opacification and loss of defi-
cranial nerve palsies and cerebrovascular occlusion.
nition of contents (Fig. 4.19C and D).
zzDifferential diagnosis is listed in Table 4.1.
{z Biopsy is generally required in persistent cases to confirm
zzInvestigation is much the same as for bacterial orbital cellulitis.!
the diagnosis and particularly to rule out neoplasia and
Treatment systemic inflammatory conditions.
{z A wide range of other investigations may be considered to aid
zzCorrection of the underlying metabolic defect should be insti-
in the exclusion of alternative diagnoses, particularly infec-
tuted if possible.
tion, lymphoma and non-neoplastic infiltrative disorders
zzIntravenous antifungal treatment.
such as sarcoidosis and granulomatosis with polyangiitis.!
zzDaily packing and irrigation of the involved areas with anti-
fungal agent.
zzWide excision of devitalized and necrotic tissues. Exenteration Treatment
may be required in unresponsive cases in order to reduce the zzObservation, for relatively mild disease, in anticipation of
risk of death. spontaneous remission.
zzAdjunctive hyperbaric oxygen may be helpful.! zzNSAIDs alone (e.g. ibuprofen) are o%en effective and may be
tried in mild disease prior to steroid therapy. Co-prescription
NON-INFECTIVE INFLAMMATORY of a proton pump inhibitor should be considered.
zzSystemic steroids should be administered only a%er the
DISEASE diagnosis has been confirmed, as they may mask other
pathology such as infection and granulomatosis with poly-
Idiopathic orbital inflammatory disease angiitis. Oral prednisolone is initially given at a dose of
Idiopathic orbital inflammatory disease (IOID), also called non- 1.0–1.5 mg/kg daily, subsequently being tapered and dis-
specific orbital inflammation or orbital pseudotumour, is an uncom- continued over a number of weeks depending on clinical
mon disorder characterized by non-neoplastic, non-infective, response. Further treatment may be needed in the event of
space-occupying orbital infiltration with inflammatory features. recurrence.
The process may preferentially involve any or all of the orbital so% zzOrbital depot steroid injection may be useful in some cases.
tissues. Histopathological analysis reveals pleomorphic inflamma- zzRadiotherapy may be considered if there has been no improve-
tory cellular infiltration followed by reactive fibrosis. Unilateral dis- ment a%er 2 weeks of adequate steroid therapy. Even low-dose
ease is typical in adults, although in children bilateral involvement treatment (e.g. 2 Gy fractionation daily up to 20 Gy total dose)
may occur. Intracranial extension is rare. Simultaneous orbital and may produce remission, though much higher total doses may
sinus involvement is also rare and may be a distinct entity. be necessary (up to 36 Gy total dose).

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128 Non-infective Inflammatory Disease

A B

C D
Fig. 4.19 Idiopathic orbital inflammation. (A) Idiopathic scirrhous inflammation showing an ad-
duction weakness of the right eye, proptosis and ptosis; (B) left idiopathic inflammatory dis-
ease, showing lid swelling and ptosis; (C) CT axial view of left orbit showing ill-defined orbital
opacification; (D) coronal view. (Courtesy of G Rose – fig. A; R Bates – fig. B; A Pearson – figs
C and D.)

zzOther options, usually as supplementary treatments or in zzSigns are generally less obvious than IOID.
resistant cases, include cytotoxic drugs (e.g. methotrexate, aza- {z Lid oedema, ptosis and chemosis.
thioprine), calcineurin inhibitors (e.g. ciclosporin, tacrolimus) {z Pain and diplopia associated with eye movements
and biological blockers. (Fig. 4.20B).
zzSurgical resection of an inflammatory focus may be contem- {z Vascular injection over the involved muscle (Fig. 4.20C).
plated in highly resistant cases.! {z In chronic cases fibrosis of the affected muscle may occur
with permanent restrictive myopathy.
zzCourse
Orbital myositis {z Acute non-recurrent involvement that resolves spontane-
ously within 6 weeks.
Introduction {z Chronic disease characterized by either a single episode
Orbital myositis is an idiopathic, non-specific inflammation of one persisting for longer than 2 months (o%en for years) (Fig.
or more extraocular muscles and is considered a subtype of IOID. 4.20D) or recurrent attacks.
Histology shows a chronic inflammatory cellular infiltrate associ- zzInvestigation consists primarily of MRI or CT, which show
ated with the muscle fibres (Fig. 4.20A).! enlargement of the affected muscles (Fig. 4.20E and F), with or
without involvement of the tendons of insertion. This is in con-
Diagnosis trast to TED-related muscle enlargement, in which the tendon
zzSymptoms. Acute pain, exacerbated by eye movement and is always spared. Additional investigations may be required in
diplopia. Onset is usually in early adulthood. some cases.!

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Orbit 129

A B

C D

E F

Fig. 4.20 Orbital myositis. (A) Histology showing a chronic inflammatory cellular infiltrate in
relation to muscle fibres; (B) idiopathic myositis involving the right lateral rectus muscle with
diplopia on left eye movement; (C) vascular injection over the insertion of the right medial
rectus; (D) chronic myositis of superior rectus-levator complex; (E) coronal CT of (C), showing
enlargement of the right medial rectus (F) sagittal CT of (D) showing enlargement of supe-
rior rectus muscle. (Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology,
Butterworth-Heinemann 2001 – fig. A; G Rose – figs B, D, F; J Nerad, K Carter and M Alford,
from ‘Oculoplastic and Reconstructive Surgery’, in Rapid Diagnosis in Ophthalmology, Mosby
2008 – figs C and E.)

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130 Non-infective Inflammatory Disease

Treatment
Treatment is aimed at relieving discomfort and dysfunction, short-
ening the course and preventing recurrences. NSAIDs may be ade-
quate in mild disease, but systemic steroids are generally required
and usually produce dramatic improvement, although recurrence
is seen in 50%. Radiotherapy is also effective, particularly in limit-
ing recurrence.!

Acute dacryoadenitis
Acute dacryoadenitis may be idiopathic or due to viral (e.g. mumps,
Epstein–Barr, cytomegalovirus) or – rarely – bacterial infection;
the lacrimal gland is o%en involved in IOID. Chronic conditions
such as sarcoidosis, Sjögren syndrome, thyroid disease and some
chronic infections usually give a less acute onset and involvement A
can be bilateral. Presentation in acute disease is with the rapid onset
of discomfort in the region of the gland. Lacrimal secretion may be
reduced or increased and discharge may be reported. Swelling of the
lateral aspect of the eyelid overlying the palpebral lobe leads to a
characteristic S-shaped ptosis and enlargement of the orbital lobe
may give a slight downward and inward dystopia (Fig. 4.21A) and
occasionally proptosis and other signs of orbital disease. There is
tenderness over the lacrimal gland and injection of the conjunc-
tiva overlying the palpebral lobe may be seen on upper lid eversion
(Fig. 4.21B). Chemosis may be present. There may be local (e.g. pre-
auricular) lymph node enlargement. CT shows enlargement of the
gland and involvement of adjacent tissues (Fig. 4.21C) without bony
erosion; the latter suggests a tumour. Biopsy is sometimes indicated,
particularly to exclude a tumour. Treatment varies according to the
cause, but in many cases is not required.!
B
Tolosa–Hunt syndrome
Tolosa–Hunt syndrome is a rare idiopathic condition caused by non-
specific granulomatous inflammation of the cavernous sinus, superior
orbital fissure and/or orbital apex. It is a diagnosis of exclusion and
should be investigated fully. Presentation is with ipsilateral periorbital
or hemicranial pain and diplopia due to ocular motor paresis, with
pupillary and eyelid involvement in many cases. Proptosis, if present,
is usually mild. Sensory loss along the distribution of the first and sec-
ond divisions of the trigeminal nerve is common. The patient may be
pyrexial. Diagnosis is with imaging, together with other investigations
to rule out identifiable causes, including neoplasia. Treatment is with
systemic steroids and other immunosuppressants as necessary; the
clinical course is characterized by remissions and recurrences.!

Granulomatosis with polyangiitis C

Granulomatosis with polyangiitis, previously called Wegener gran- Fig. 4.21 Left acute dacryoadenitis. (A) Swelling on the
ulomatosis (see Ch. 9), is an idiopathic multisystem granuloma- lateral aspect of the eyelid and an S- shaped ptosis; (B)
injection of the palpebral portion of the lacrimal gland
tous disorder that may involve the orbit, o%en bilaterally, usually by and adjacent conjunctiva; (C) axial CT showing en-
contiguous spread from the paranasal sinuses or nasopharynx. Pri- largement of the gland and opacification of adjacent
mary orbital involvement is less common. This condition should tissues. (Courtesy of R Bates – fig. B; A Pearson – fig. C.)
be considered in any patient with bilateral orbital inflammation,
particularly if associated with sinus pathology. Antineutrophilic
cytoplasmic antibody (cANCA variant) is a useful serological

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Orbit 131

test. Other ocular features include scleritis, peripheral ulcerative or straining. Connective tissue and collagen vascular disorders
keratitis, intraocular inflammation and retinal vascular occlu- can be associated.!
sions. Treatment is with cyclophosphamide and steroids, which
are usually effective. In resistant cases ciclosporin, azathioprine, Diagnosis
antithymocyte globulin or plasmapheresis may be useful. Surgi- zzSymptoms – direct. Presentation may be days or weeks a%er
cal decompression may be required for severe orbital involvement.! head injury with a classic triad of pulsatile proptosis, conjunc-
tival chemosis and a whooshing noise in the head.
NON-NEOPLASTIC VASCULAR zzSymptoms – indirect. Gradual onset of redness of one or both
eyes is a typical presentation, caused by conjunctival vascular
ABNORMALITIES engorgement.
zzSigns – direct
Cavernous sinus thrombosis {z Immediate visual loss may be due to ocular or optic nerve
This refers to clotting within the cavernous sinus, usually result- damage at the time of head trauma.
ing from infection such as sinusitis, orbital or preseptal cellulitis {z Delayed visual loss may occur as a result of exposure
or otitis. There is a high mortality rate: 20% treated and up to keratopathy, secondary glaucoma, central retinal vein
100% untreated die. Features are of rapid onset and may include occlusion, anterior segment ischaemia or ischaemic optic
severe headache, malaise, nausea and vomiting, unilateral or o%en neuropathy.
bilateral proptosis, chemosis, congestion of the facial, conjunctival {z Signs are usually ipsilateral to the fistula but may be bilat-
and retinal veins, reduced vision and signs resulting from com- eral, or even contralateral, because of midline connections
promised function of the third to sixth cranial nerves, which run between the two cavernous sinuses.
through the cavernous sinus. Diagnosis is with imaging, especially {z Marked epibulbar vascular dilatation (Fig. 4.22A).
MRI and MRI venography. Systemic investigation for infection is {z Chemosis, commonly haemorrhagic, particularly in the
also performed, including lumbar puncture. Treatment consists of early stages (Fig. 4.22B).
intravenous antibiotics and sometimes surgical drainage.! {z Pulsatile proptosis associated with a bruit and a thrill, both
of which can be abolished by ipsilateral carotid compres-
TIP Cavernous sinus thrombosis has a high mortality rate sion in the neck.
{z Increased IOP due to elevated episcleral venous pres-
if the diagnosis is delayed and if intensive treatment is not
instigated urgently. sure and orbital congestion and sometimes angle-closure
glaucoma.
{z Anterior segment ischaemia, characterized by corneal epi-
Carotid–cavernous fistula thelial oedema, aqueous cells and flare and in severe cases
iris atrophy, cataract and rubeosis iridis.
Introduction {z Ptosis due to third nerve involvement.
A carotid–cavernous fistula involves the development of an arterio- {z Ophthalmoplegia (60–70%) due to the ocular motor nerve
venous fistula between the carotid artery and the venous cavern- damage from initial trauma, an intracavernous aneurysm
ous sinus (see Fig. 19.64) with a rise in venous pressure in the sinus or the fistula itself. The sixth cranial nerve is most fre-
and structures draining to it. Ocular manifestations occur because quently affected because of its free-floating location within
of venous and arterial stasis around the eye and orbit, increased the cavernous sinus. The third and fourth nerves, situated
episcleral venous pressure and a decrease in arterial blood flow to in the lateral wall of the sinus, are less frequently involved.
the cranial nerves within the cavernous sinus. Carotid–cavernous Engorgement and swelling of extraocular muscles may
fistulae are classified into ‘direct’ and ‘indirect’ forms. also contribute to defective ocular motility.
zz Direct fistulae are high-flow shunts in which carotid artery blood {z Fundus examination may show optic disc swelling, venous
passes directly into the cavernous sinus through a defect in the dilatation and intraretinal haemorrhages from venous sta-
wall of the intracavernous portion of the internal carotid artery sis and impaired retinal blood flow. Vitreous haemorrhage
as a result of trauma (75%), including surgery, spontaneous rup- is rare.
ture of an intracavernous carotid aneurysm or an atheroscle- zzSigns – indirect
rotic artery, the latter frequently in a middle-aged hypertensive {z Milder epibulbar vascular dilatation than with a direct fis-
woman; spontaneous fistulae usually have lower flow. tula (Fig. 4.22C).
zzIn an indirect fistula (‘dural shunt’), the intracavernous por- {z Exaggerated ocular pulsation, which is readily detected on
tion of the internal carotid artery remains intact. Arterial slit lamp applanation tonometry.
blood flows through the meningeal branches of the external {z The presence of ‘corkscrew’ epibulbar vessels (Fig. 4.22D)
or internal carotid arteries indirectly into the cavernous sinus is a common subtle later sign. These are not pathogno-
and the clinical features are subtler than in a direct fistula such monic and can be found in normal eyes.
that the condition may be overlooked. Spontaneous rupture {z Raised IOP, o%en bilateral but higher on the side of the
of an atherosclerotic artery or of a congenital malformation fistula.
is the usual cause and may be precipitated by minor trauma {z Proptosis and bruit are mild if present.

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132 Non-neoplastic Vascular Abnormalities

A B

C D

Fig. 4.22 Carotid–cavernous fistula. (A) Marked epibulbar vascular dilatation in an established
direct fistula; (B) haemorrhagic chemosis in an acute direct fistula; (C) mild epibulbar vascular
dilatation in an indirect fistula; (D) corkscrew conjunctival vessel. (Courtesy of S Chen – fig.
A; C Barry – figs C and D.)

{z Ophthalmoplegia caused by sixth nerve palsy or swelling should be sought at an early stage, even if features are mild, as some
of extraocular muscles in marked cases. fistula patterns (e.g. cortical venous drainage) carry a high risk of
{z Fundus may be normal or manifest moderate venous dila- stroke.
tation, with later tortuosity as with corkscrew conjunctival zzDirect. Most carotid–cavernous fistulae are not life-
vessels, this is not pathognomonic (see Ch. 13). threatening; the organ at major risk is the eye. Surgery is indi-
zzInvestigation. CT and MRI may demonstrate prominence cated if spontaneous closure does not occur. A post-traumatic
of the superior ophthalmic vein (Fig. 4.23A) and diffuse fistula is much less likely to close on its own than a spontane-
enlargement of extraocular muscles (Fig. 4.23B), though ous fistula because of higher blood flow. Treatment is likely to
these may be visible only with a direct fistula. Orbital Dop- consist of a transarterial approach to repair the artery (e.g. coil
pler imaging may show abnormal flow patterns, particularly – Fig. 4.23C and D, other) or occlude the involved sinus (e.g.
in the superior orbital vein. Definitive diagnosis may involve coil, balloon, other). Craniotomy for arterial repair is occa-
selective catheter digital subtraction angiography, especially sionally needed.
in mild dural fistulae, though CT and MRI angiography can zzIndirect. If required, treatment usually involves transve-
be useful.! nous occlusion of the involved sinus. Spontaneous closure or
occluding thrombosis sometimes (up to 50%) occurs. Inter-
Treatment mittent carotid compression under specialist supervision has
Ocular complications may require specific measures in addition been reported to increase the likelihood that this will take
to treatment of the fistula itself. Neurological subspecialist opinion place.!

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Orbit 133

A B

C D
Fig. 4.23 CT in direct carotid–cavernous fistula. (A) Axial CT image showing enlargement
of the right superior ophthalmic vein; (B) coronal view showing enlargement of extraocular
muscles on the right; (C) early arterial phase catheter angiogram showing filling of the cavern-
ous sinus (arrow) and superior ophthalmic vein (arrowhead); (D) following deposition of coils
in the cavernous sinus – the fistula is closed and there is no retrograde flow in the superior
ophthalmic vein. (Courtesy of J Trobe, from ‘Neuro-ophthalmology’, in Rapid Diagnosis in
Ophthalmology, Mosby 2008.)

CYSTIC LESIONS
Dacryops
A dacryops is a frequently bilateral cyst of the lacrimal gland that is
thought to develop from a dilated obstructed duct. A round cystic
lesion protrudes into the superior fornix from the palpebral lobe of
the gland (Fig. 4.24) and may present with inflammation. The pos-
sibility of a malignant tumour should always be considered. Treat-
ment involves excision or marsupialization, with histopathological
analysis.!

Dermoid cyst
Introduction
An orbital dermoid cyst is a choristoma (a mass of histologically Fig. 4.24 Dacryops.
normal tissue in an abnormal location) derived from displacement
of ectoderm to a subcutaneous location along embryonic lines of appendages such as sweat glands, sebaceous glands and hair fol-
closure. Dermoids are lined by keratinized stratified squamous licles; epidermoid cysts do not contain adnexal structures. Der-
epithelium (like skin), have a fibrous wall and contain dermal moids may be ‘superficial’ or ‘deep’, located anterior or posterior to

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134 Cystic Lesions

A B

Fig. 4.25 Orbital dermoid cyst. (A) Superficial cyst right eye in a child; (B) superonasal deep
cyst in a young adult.

A B

Fig. 4.26 Orbital dermoid cysts – imaging. (A) Axial CT image showing a well-circumscribed
heterogeneous superficial lesion; (B) deep dermoid – CT showing a well-circumscribed cystic
lesion. (Courtesy of Ken K Nischal – fig. A.)

A B

Fig. 4.27 Superficial orbital dermoid cyst. (A) Appearance at surgery; (B) excised specimen.
(Courtesy of JH Norris.)

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Orbit 135

the orbital septum respectively. Epibulbar dermoids and dermoli- keratin into the surrounding tissue typically results in severe
pomas are related lesions (see Ch. 20).! granulomatous inflammation.
zzDeep dermoid. Excision in toto is advisable because deep
Diagnosis dermoids enlarge and may leak into adjacent tissues induc-
Dermoid cysts are one of the most frequently encountered orbital ing inflammation, o%en followed by fibrosis. If incompletely
tumours in children. excised, dermoids may recur with persistent low-grade
zzSymptoms inflammation.!
{z A superficial orbital dermoid cyst presents in infancy with
a painless nodule, most commonly located in the supero-
temporal and occasionally the superonasal part of the orbit. Sinus mucocoele
{z A deep dermoid cyst presents in adolescence or adult life A mucocoele (US spelling – mucocele) develops when the drain-
with a gradually increasingly protruding eye, or acutely age of normal paranasal sinus secretions is obstructed due to
with an inflamed orbit due to rupture. infection, allergy, trauma, tumour or congenital narrowing. A
zzSigns slowly expanding cystic accumulation of mucoid secretions and
{z Superficial: a firm round smooth non-tender mass 1–2 cm epithelial debris develops and gradually erodes the bony walls of
in diameter (Fig. 4.25A), mobile under the skin but usually the sinus, causing symptoms by encroachment upon surrounding
tethered to the adjacent periosteum. The posterior mar- tissues. Orbital invasion occurs usually from a frontal or ethmoidal
gins are easily palpable, denoting a lack of deeper origin mucocoele, but rarely from those arising in the maxillary sinus.
or extension. Presentation is in adult life with proptosis or dystopia (Fig. 4.28A),
{z Deep: proptosis, dystopia or a mass lesion with indistinct diplopia or epiphora. Pain is uncommon unless secondary infec-
posterior margins. (Fig. 4.25B). tion develops (mucopyocoele). CT shows a so% tissue mass with
zzInvestigation thinning or erosion of the bony walls of the sinus (Fig. 4.28B).
{z Imaging is required in all cases to rule out dumb-bell or Treatment involves complete excision.!
subperiosteal extension.
{z Superficial: imaging shows a well-circumscribed heteroge-
neous cystic lesion (Fig. 4.26A). Encephalocoele
{z Deep: imaging shows a well-circumscribed lesion (Fig.
An encephalocoele (US spelling – encephalocele) is formed by
4.26B). Some deep dermoids, associated with bony defects, herniation of intracranial contents through a congenital defect
may extend into the inferotemporal fossa or intracranially. of the base of the skull and can be located at the front or back of
the head. A meningocoele contains only dura whilst a menin-
TIP When removing an orbital dermoid cyst take care not to goencephalocoele also contains brain tissue. Presentation is usu-
rupture the wall of the cyst, as this can cause a granulomatous ally during infancy. Anterior orbital encephalocoeles involve the
inflammation in the surrounding tissue.
! superomedial part of the orbit and displace the globe forwards
and laterally (Fig. 4.29A), whereas posterior orbital encepha-
Treatment locoeles (frequently associated with neurofibromatosis type 1)
zzSuperficial dermoid. Treatment is by excision in toto (Fig. displace the globe forwards and downwards (Fig. 4.29B). The dis-
4.27), taking care not to rupture the lesion, since leaking of placement increases on straining or crying and may be reduced

A B

Fig. 4.28 (A) Left frontal sinus mucocoele causing globe displacement; (B) coronal CT show-
ing mucocoele above the globe.

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136 Vascular Tumours

be acquired later secondary to a local high-flow vascular lesion or

trauma.!

Diagnosis
Most cases are unilateral and the most frequent site is upper nasal.
Intermittent non-pulsatile proptosis without a bruit is reported. If
A there is free communication with the normal circulation, reversible
proptosis may be precipitated or accentuated by increasing venous
pressure through coughing, straining, the Valsalva manoeuvre
(Fig. 4.30C and D), assuming a head-down position or external
compression of the jugular veins. Imaging (e.g. MRI and mag-
netic resonance venography [MRV], CT, ultrasound, venography)
shows a lobulated mass with variable contrast enhancement and
may demonstrate phleboliths (Fig. 4.30E) and sometimes orbital
B expansion (particularly in childhood) or an associated orbital wall
defect. Complications include acute orbital haemorrhage, throm-
bosis (pain, proptosis, decreased vision) and optic nerve compres-
sion. Patients with long-standing lesions may develop atrophy of
surrounding fat, giving enophthalmos with a deepened superior
sulcus (Fig. 4.30F).!

Treatment
C Small lesions generally do not require treatment. Surgical excision
is technically difficult and o%en incomplete because the lesions
Fig. 4.29 Encephalocoele. (A) Anterior superomedial enceph- are friable and bleed easily. It can be complicated by severe orbital
alocoele causing proptosis and down and out dystopia; (B)
posterior encephalocoele causing proptosis and inferior dys- haemorrhage and vascular optic nerve compromise. Specialized
topia; (C) coronal CT of posterior encephalocoele showing a techniques such as embolization and carbon dioxide laser surgery
large bony defect. (Courtesy of A Pearson – fig. C.) may be helpful adjuncts. Indications include recurrent thrombosis,
pain, severe proptosis and optic nerve compression.!

by manual pressure. Pulsating proptosis may occur due to com-

Lymphangioma
munication with the subarachnoid space but, because the com-
munication is not vascular, there is neither a thrill nor a bruit. CT Introduction
shows the bony defect responsible for the herniation (Fig. 4.29C). Lymphangioma is a rare hamartomatous vascular tumour that
The differential diagnosis of anterior encephalocoele includes tends to enlarge and infiltrate diffusely with time. Some authorities
other causes of medial canthal swelling such as dermoid cyst and believe lymphangiomas to be a variant of venous orbital anom-
amniontocoele, and of posterior encephalocoele includes other aly (varices) across a single spectrum and the term ‘combined
orbital lesions that present during early life such as capillary venous–lymphatic malformations of the orbit’ has been suggested.
haemangioma, juvenile xanthogranuloma, teratoma and Although usually isolated from the main circulation, bleeding into
microphthalmos with cyst.! the lumen may occur with subsequent formation of blood-filled
‘chocolate cysts’ that regress spontaneously with time. Presentation
is usually in early childhood. Differential diagnosis is principally
VASCULAR TUMOURS from orbital venous anomalies and haemangiomas. Intracranial
Varices vascular malformations can be present in association.!

Introduction Diagnosis
Primary orbital varices (combined venous–lymphatic malforma- zzAnterior lesions typically manifest as several so% bluish
tions of the orbit – see also next topic) consist of a plexus of thin- masses in the upper nasal quadrant (Fig. 4.31).
walled distensible low-flow vein-like vessels that are commonly, zzPosterior lesions may cause slowly progressive proptosis,
though not always, intrinsic to the normal circulation. They are or initially may lie dormant and later present with the sud-
probably hamartomatous (hamartoma – a disorganized over- den onset of painful proptosis (Fig. 4.32A and B) secondary
growth of mature tissues normally present in the involved area). to spontaneous haemorrhage, which may be associated with
Associations include varices of the eyelids (Fig. 4.30A and see Fig. optic nerve compression. Involvement of the lids, conjunctiva
2.63) and conjunctiva (Fig. 4.30B). They commonly present at any and oropharynx may be seen; intracranial lesions may also be
time from early childhood to late middle age, but occasionally can present.!

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A B

C D

E F

Fig. 4.30 (A) Substantial eyelid varices; (B) conjunctival varices; (C) orbital varices before Vals-
alva (arrow); and (D) with Valsalva showing forward movement of the eye and fullness of the
upper lid (arrow); (E) axial CT showing medial opacification and phleboliths; (F) left fat atrophy
resulting in enophthalmos and deep superior sulcus. (Courtesy of G Rose – figs C and D;
A Pearson – figs E and F.)

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138 Vascular Tumours

Fig. 4.31 Anterior orbital lymphangioma with typical bluish

discoloration.

Treatment
In many cases the visual prognosis is good without treatment.
Surgical excision is difficult because lesions are not encapsulated,
friable, bleed easily and commonly infiltrate normal orbital tis-
sues; repeated subtotal excision may be necessary. Persistent sight-
threatening chocolate cysts can be drained or removed sub-totally
by controlled vapourization using a carbon dioxide laser.!

B
Capillary haemangioma
Fig. 4.32 Posterior orbital lymphangioma. (A) Left proptosis
and inferior globe displacement with orbital haemorrhage;
Introduction (B) axial MRI showing proptosis and retrobulbar lymphan-
Capillary haemangioma is the most common tumour of the orbit gioma.
and periorbital area in childhood. Girls are affected more com-
monly than boys (3:1). It may present as a small isolated lesion of
minimal clinical significance, or as a large disfiguring mass that zzSigns. Extensive underlying orbital involvement should always
can cause visual impairment and systemic complications. An be ruled out in a seemingly purely superficial lesion.
established tumour is composed of anastomosing small vascular {z A superficial cutaneous lesion (‘strawberry naevus’) is
channels without true encapsulation. It is a hamartoma – a dis- bright red (Fig. 4.33A and see Fig. 2.12A).
organized overgrowth of mature tissues normally present in the {z Preseptal (deeper) tumours appear dark blue or purple
involved area – and believed to be due principally to endothelial through the overlying skin (Fig. 4.33A and B) and are most
cell proliferation. Large or multiple lesions may have associated frequently located superiorly.
visceral involvement, which can lead to serious complications {z A large tumour may enlarge and change in colour to a deep
such as thrombocytopenia (Kasabach–Merritt syndrome, with blue during crying or straining, but both pulsation and a
up to 50% mortality) and high-output cardiac failure and sys- bruit are absent.
temic investigation should be considered. The incidence of {z Deep orbital tumours give rise to unilateral proptosis with-
infantile haemangioma in the general population is around 5% out skin discoloration.
and a small proportion of these, especially if a large facial haem- {z Haemangiomatous involvement of the palpebral or forni-
angioma is present, will have PHACE (PHACES) syndrome, ceal conjunctiva is common (Fig. 4.33B).
which includes a range of possible systemic features including {z Additional haemangiomas on the eyelids (see Ch. 2) or
eye involvement.! elsewhere are common.
zzInvestigation. Imaging is generally performed for other than
Diagnosis very small lesions, mainly to rule out more extensive orbital
zzSymptoms. The lesion is usually noticed by the parent, usually disease. Ultrasound shows medium internal reflectivity and on
in the first few months of life. Approximately 30% are present MRI or CT the lesion appears as a so% tissue mass in the ante-
at birth. rior orbit or as an extraconal mass with finger-like posterior

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Orbit 139

expansions (Fig. 4.33C). The orbital cavity may show enlarge-

ment but there is no bony erosion.!

Treatment
The natural course is characterized by rapid growth (Fig. 4.34A
and B) 3–6 months a%er diagnosis, followed by a slower phase of
natural resolution in which 30% of lesions resolve by the age of 3
years and about 75% by the age of 7. Treatment is indicated prin-
cipally for amblyopia secondary to induced astigmatism, anisome-
tropia, occlusion or strabismus and less commonly for cosmesis,
optic nerve compression or exposure keratopathy.
zzBeta-blockers. Oral propranolol is now widely used. The pre-
scription and monitoring should generally be carried out by
a paediatrician. Topical preparations including timolol are
A equally effective, with excellent long-term results and no sys-
temic side effects (Fig. 4.34C and D).
zzSteroids
{z Injection of triamcinolone acetonide (1–2 ml total of
40 mg/ml over several injection sites) or betamethasone
(4 mg/ml) into a cutaneous or preseptal tumour is usually
effective in early lesions. Regression usually begins within
2 weeks but, if necessary, second and third injections can
be given a%er about 2 months. It is advisable not to inject
deeply into the orbit for fear of causing occlusion of the
central retinal artery due to retrograde introduction of the
suspension. Other complications include skin depigmen-
tation and necrosis, fat atrophy and systemic effects such
as adrenal suppression.
{z Topical high-potency steroids (e.g. clobetasol propionate
cream) are sometimes appropriate but are slow to exert
B their effect.
{z Systemic steroids administered daily over several weeks
may be used, particularly if there is a large orbital compo-
nent or a rapid onset of action is required.
zzLaser may be used to close blood vessels in superficial skin
lesions less than 2 mm in thickness.
zzInterferon alfa-2a and vincristine may be used for some
steroid-resistant sight-threatening lesions.
zzLocal resection with cutting cautery or carbon dioxide laser
may reduce the bulk of an anterior circumscribed tumour, but
is usually reserved for the late inactive stage unless a resistant
tumour is sight- or life-threatening.!

Cavernous haemangioma
C Introduction
Cavernous haemangioma occurs in middle-aged adults, with a
Fig. 4.33 Capillary haemangioma. (A) Large preseptal tumour female preponderance of 70%. The growth may be accelerated by
causing ptosis and purple cutaneous discoloration, there is a
pregnancy. It is the most common orbital tumour in adults and is
superficial component (strawberry naevus); (B) involvement
of forniceal conjunctiva; (C) axial enhanced CT showing a probably a vascular malformation rather than a neoplastic lesion.
homogeneous intraconal orbital soft tissue mass. (Courtesy Although it may develop anywhere in the orbit, it most frequently
of Ken K Nischal – fig. B; A Pearson – fig. C.) occurs within the lateral part of the muscle cone just behind the
globe and behaves like a low-flow arteriovenous malformation.
Histology shows endothelial-lined vascular channels of varying
size separated by fibrous septa (Fig. 4.35A).!

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140 Vascular Tumours

A B

C D

Fig. 4.34 Growth of capillary haemangioma. (A) At presentation; (B) several months later;
(C) at presentation; (D) 6 months after treatment with topical beta-blocker. (Courtesy of D
Hildebrand – figs C and D.)

A B

C D

Fig. 4.35 Cavernous haemangioma. (A) Histology showing congested variably sized
endothelial-lined vascular channels separated by fibrous septa; (B) right axial proptosis; (C)
axial CT showing a well-circumscribed retrobulbar oval lesion and proptosis; (D) the tumour
is encapsulated and relatively easy to remove. (Courtesy of A Pearson.)

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Orbit 141

Diagnosis appropriate. Symptomatic lesions require surgical excision in most

cases because they gradually enlarge. The cavernous haemangi-
zzSymptoms. Slowly progressive unilateral proptosis; bilateral
oma, unlike its capillary counterpart, is usually well-encapsulated
cases are very rare.
and relatively easy to remove (Fig. 4.35D).!
zzSigns
{z Axial proptosis (Fig. 4.35B), which may be associated with
optic disc oedema and choroidal folds. LACRIMAL GLAND TUMOURS
{z A lesion at the orbital apex may compress the optic nerve
without causing significant proptosis. Gaze-evoked tran- Pleomorphic lacrimal gland adenoma
sient blurring of vision may occur. Introduction
{z There may be impairment of extraocular muscle excursion.
Pleomorphic adenoma (benign mixed-cell tumour) is the most
zz Investigation. CT (Fig. 4.35C) and MRI show a well-
common epithelial tumour of the lacrimal gland and is derived
circumscribed oval lesion, usually within the muscle cone. There
from the ducts and secretory elements including myoepithelial
is only slow contrast enhancement. Ultrasound is also useful.!
cells. On histopathology, the inner layer of cells forms glandular
tissue that may be associated with squamous differentiation and
Treatment keratin production (Fig. 4.36A). The outer cells undergo meta-
Many cavernous haemangiomas are detected by chance on scans plastic change leading to the formation of myxoid tissue. Young to
performed for unrelated reasons and observation alone is o%en middle-aged adults are the predominantly affected group.!

A C

D E

Fig. 4.36 Pleomorphic lacrimal gland adenoma. (A) Histology showing glandular tissue and
squamous differentiation with keratin formation; (B) inferonasal dystopia due to a tumour aris-
ing from the orbital lobe; (C) eyelid swelling without dystopia; (D) eversion of the upper eyelid
reveals the tumour; (E) coronal CT of (B) showing an orbital lobe lesion. (Courtesy of J Harry
and G Misson, from Clinical Ophthalmic Pathology, Butterworth-Heinemann 2001 – fig. A;
A Pearson – figs B and E.)

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142 Lacrimal Gland Tumours

Diagnosis zzThe underlying bone is drilled for subsequent wiring (Fig.

4.37B).
zzSymptoms. Painless slowly progressive proptosis or swelling
zzThe lateral orbital wall is removed and the tumour excised,
in the superolateral eyelid, usually of more than a year’s dura-
including a margin of adjacent tissue and periorbita (Fig.
tion. Old photographs may reveal an abnormality many years
4.37C).
prior to presentation.
zzThe lateral orbital wall (Fig. 4.37D) and temporalis are repaired.
zzSigns
zzThe prognosis is excellent provided excision is complete and
{z Orbital lobe tumour presents as a smooth, firm, non-
without disruption of the capsule. Incomplete excision or pre-
tender mass in the lacrimal gland fossa with inferonasal
liminary incisional biopsy may result in seeding of the tumour
dystopia (Fig. 4.36B). Posterior extension may cause pro-
into adjacent tissues, with recurrence and occasionally malig-
ptosis, ophthalmoplegia and choroidal folds.
nant change.!
{z Palpebral lobe tumour is less common and tends to grow
anteriorly causing upper lid swelling without dystopia (Fig.
4.36C), which may be visible to inspection (Fig. 4.36D). Lacrimal gland carcinoma
zzInvestigation. CT shows a round or oval mass, with a smooth
Lacrimal gland carcinoma is a rare tumour that carries a high mor-
outline and indentation but not destruction of the lacrimal
bidity and mortality. In order of frequency the main histological
gland fossa (Fig. 4.36E). The lesion may indent the globe and
types are adenoid cystic (50%), pleomorphic adenocarcinoma,
calcification may be shown.!
mucoepidermoid and squamous cell. Histopathology shows nests
Treatment of basaloid cells with numerous mitoses (Fig. 4.38A). The peak
incidence is in middle-aged adults.
Treatment involves surgical excision. If the diagnosis is strongly
suspected, it is wise to avoid prior biopsy to prevent tumour seed- Diagnosis
ing into adjacent orbital tissue, although this may not always be
zzSymptoms. A malignant mixed-cell tumour presents in three
possible in the context of diagnostic uncertainty. Tumours of the
main clinical settings:
palpebral lobe are usually resected, along with a margin of normal
{z A%er incomplete or piecemeal excision of a benign pleo-
tissue, through an anterior (trans-septal) orbitotomy. Those of the
morphic adenoma, followed by one or more recurrences
orbital portion are excised through a lateral orbitotomy:
over a period of several years with eventual malignant
zzThe temporalis muscle is incised (Fig. 4.37A).
transformation.

A B

C D

Fig. 4.37 Lateral orbitotomy. (A) Incision of temporalis muscle; (B) drilling of underlying bone
for subsequent wiring; (C) removal of the lateral orbital wall and the tumour; (D) repair of the
lateral orbital wall.

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Orbit 143

A B

C D

Fig. 4.38 Lacrimal gland carcinoma. (A) Histology of adenoid-cystic carcinoma showing nests
of basaloid cells with solid and cribriform areas; (B) early changes showing lid swelling and
ptosis; (C) proptosis, periorbital oedema and epibulbar congestion; (D) axial CT of (C) show-
ing carcinoma surrounding the globe, extending along the lateral orbital wall and extending
through the right superior orbital fissure. (Courtesy of J Harry and G Misson, from Clinical
Ophthalmic Pathology, Butterworth-Heinemann 2001 – fig. A; G Rose – figs C and D.)

{z As a long-standing proptosis or swollen upper lid that sud- {z Posterior extension, with involvement of the superior orbital
denly starts to increase. fissure, may give rise to epibulbar congestion, proptosis,
{z Without a previous history of a pleomorphic adenoma as periorbital oedema and ophthalmoplegia (Fig. 4.38C).
a rapidly growing lacrimal gland mass, usually of several {z Hypoaesthesia in the region supplied by the lacrimal nerve.
months’ duration. {z Optic disc swelling and choroidal folds.
{z The history is shorter than that of a benign tumour. zzInvestigation
{z Pain is a frequent feature of malignancy but may also occur {z CT shows a globular lesion with irregular serrated edges,
with inflammatory lesions. o%en with contiguous erosion or invasion of bone (Fig.
4.38D). Calcification is commonly seen within the tumour.
{z Biopsy is necessary to establish the histological diagnosis.
TIP Beware of an incomplete excision of a benign pleomorphic
adenoma as this can result in malignant transformation. Subsequent management depends on the extent of tumour
invasion of adjacent structures as seen on imaging.
zzSigns {z Neurological assessment is mandatory because adenoid-

{z A mass in the lacrimal area causing swelling, ptosis and cystic carcinoma exhibits perineural spread and may
dystopia (Fig.4.38B). extend into the cavernous sinus.!

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144 Neural Tumours

Treatment associated neurofibromatosis type 1 (NF1 – see Ch. 19) and in these
patients the prognosis is superior. Malignant glioma (glioblastoma)
Treatment involves excision of the tumour and adjacent tissues.
is rare, has a very poor prognosis and usually occurs in adult males.!
Extensive tumours may require orbital exenteration or midfacial
resection, but the prognosis for life is frequently poor. Radiother- Diagnosis
apy combined with local resection may prolong life and reduce
zzSymptoms
pain. Adjuvant intra-arterial chemotherapy and/or brachytherapy
{z Slowlyprogressive visual loss followed later by proptosis,
may be utilized in some cases.!
although this sequence may occasionally be reversed.
{z Acute loss of vision due to haemorrhage into the tumour
can occur, but is uncommon.
NEURAL TUMOURS zzSigns
Optic nerve glioma {z Proptosis is o%en non-axial, with temporal or inferior dys-
topia (Fig. 4.39B).
Introduction {z The optic nerve head, initially swollen, subsequently
Optic nerve glioma is a slowly growing, pilocytic astrocytoma that becomes atrophic (Fig. 4.39C).
typically affects children (median age 6.5 years). Histopathology {z Opticociliary collaterals (see Fig. 4.8C) and other fundus
shows spindle-shaped pilocytic (hair-like) astrocytes and glial fila- signs such as central retinal vein occlusion are occasion-
ments (Fig. 4.39A). The prognosis is unpredictable; some have an ally seen.
indolent course with little growth, while others may extend intra- {z Intracranial spread to the chiasm and hypothalamus may
cranially and threaten life. Approximately 30% of patients have develop.

A B

C D

Fig. 4.39 Optic nerve glioma. (A) Histopathology – spindle-shaped pilocytic astrocytes and
glial filaments; (B) proptosis with inferior dystopia; (C) optic disc atrophy; (D) axial T2-weighted
MRI of (C) showing fusiform optic nerve enlargement. (Courtesy of Ken K Nischal – fig. B.)

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zzInvestigation Diagnosis
{z MRI effectively demonstrates the tumour and may show
zzSymptoms typically consist of gradual visual impairment in
intracranial extension if present (Fig. 4.39D).
one eye. Transient obscurations of vision may occur.
{z CT in patients with associated NF1 shows a fusiform
zzSigns. The classic (Hoyt–Spencer) triad consists of progres-
enlargement of the optic nerve with a clear-cut margin
sive visual loss, optic atrophy and opticociliary shunt ves-
produced by the intact dural sheath. In patients without
sels, although the simultaneous occurrence of all three signs
NF1 the nerve is more irregular and shows low-density
in one individual is actually uncommon. The usual sequence
areas.!
of involvement is the opposite of that seen in tumours that
develop outside the dural sheath:
Treatment {z Optic nerve dysfunction and chronic disc swelling fol-
As the tumour is intrinsic to the optic nerve, resection means that lowed by atrophy.
all vision will be lost in the operated eye. {z Opticociliary collaterals (30%), which regress as optic
zzObservation may be considered in patients with a typical pilo- atrophy supervenes.
cytic astrocytoma on imaging in whom the tumour is confined {z Restrictive motility defects, particularly in upgaze (Fig. 4.40C).
to the orbit, especially if there is good vision and no significant {z Proptosis (Fig. 4.40D).
cosmetic impairment. Serial MRI is important if this option is zzInvestigation
chosen. Spontaneous regression has been reported, usually in {z CT shows thickening and calcification of the optic nerve
NF1, but is very rare. (Fig. 4.40E).
zzSurgical excision with preservation of the globe is indicated in {z MRI is the investigation of choice (Fig. 4.40F).
those with large or growing tumours where complete resection {z Ultrasonography (especially coronal) may be useful.!
of the tumour can be achieved, particularly if vision is poor
and proptosis significant. A key goal is to prevent chiasmal Treatment
involvement and an intracranial approach may be necessary to Treatment may not be indicated in a middle-aged patient with a
achieve adequate resection. slowly growing lesion, but excision is required for an aggressive
zz Radiotherapy may be combined with chemotherapy for tumours tumour, particularly if the eye is blind or there is a risk of intracra-
with extension that precludes complete surgical excision.! nial extension. Attempts at optic nerve-sparing surgery commonly
fail but may be considered on a case basis. Fractionated stereotac-
tic radiotherapy may be appropriate as a vision-sparing approach,
Optic nerve sheath meningioma or as adjunctive treatment following surgery.!

Introduction
Optic nerve sheath meningioma is a benign tumour arising
Plexiform neurofibroma
from meningothelial cells of the arachnoid villi surrounding Plexiform neurofibroma is the most common peripheral neural
the intraorbital, or less commonly the intracanalicular, portion tumour of the orbit. It occurs almost exclusively in association
of the optic nerve. In some cases, the tumour merely encircles with NF1. Presentation is in early childhood with periorbital swell-
the optic nerve whilst in others it invades the nerve, growing ing. Classically, involvement of the eyelids causes mechanical pto-
along the fibrovascular pial septa. However, about two-thirds sis with a characteristic S-shaped deformity (see Fig. 2.14B), but
of all meningiomas affecting the optic nerve arise from exten- diffuse involvement of the orbit with disfiguring hypertrophy of
sion of primarily intracranial lesions. Primary optic nerve periocular tissues may occur. On palpation the involved tissues are
sheath meningiomas are less common than optic nerve gliomas said to resemble a ‘bag of worms’. Malignant change can occur and
and, as with other meningiomas, typically affect middle-aged should be suspected if there is rapid change; radiotherapy may pro-
women. Histopathologically, meningothelial (irregular lob- mote this. Treatment is o%en unsatisfactory and complete surgical
ules of meningothelial cells separated by fibrovascular strands removal is extremely difficult. Orbital surgery should be avoided
– Fig. 4.40A) and psammomatous (psammoma bodies among when possible, because of the intricate relationship between the
proliferating meningothelial cells – Fig. 4.40B) types are dis- tumour and important structures.!
tinguished. The prognosis for life is good in adults, although
the tumour may be more aggressive in children, in whom 25%
occur. They are more common in neurofibromatosis type 2
Isolated neurofibroma
(NF2). Isolated (localized) neurofibroma is less common than plexiform
neurofibroma; about 10% of patients have NF1. Presentation is in
the third or fourth decades with insidious mildly painful proptosis,
usually not associated with visual impairment or ocular motility
TIP In optic nerve sheath meningioma, the onset of visual loss
dysfunction. Excision is commonly straightforward because the
usually occurs before the onset of proptosis.
! tumour is well circumscribed and relatively avascular.!

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146 Neural Tumours

A B

C D

E F

Fig. 4.40 Optic nerve meningioma. (A) Meningothelial histopathology; (B) psammomatous his-
topathology; (C) defective elevation of the right eye; (D) proptosis and severe chemosis; (E)
axial CT of a small tumour showing calcification; (F) axial gadolinium-enhanced T1-weighted
MRI showing lesion associated with left optic nerve. (Courtesy of J Harry and G Misson, from
Clinical Ophthalmic Pathology, Butterworth-Heinemann 2001 – figs A and B; A Pearson – fig. E.)

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LYMPHOMA Diagnosis
The onset is characteristically insidious.
zzSymptoms. An absence of symptoms is common, but may
Introduction include discomfort, double vision, a bulging eye or a visible
Lymphomas of the ocular adnexa constitute approximately 8% of mass.
all extranodal lymphomas. The majority of orbital lymphomas are zzSigns
non-Hodgkin and most of these (80%) are of B-cell origin (Fig. {z Any part of the orbit may be affected (Fig. 4.41B). Anterior
4.41A). Those affected are typically older individuals. The condi- lesions may be palpated and generally have a rubbery con-
tion may be primary, involving one or both orbits only, or second- sistency (Fig. 4.41C).
ary if there are one or more identical foci elsewhere in the body. A {z Occasionally the lymphoma may be confined to the con-
substantial proportion of apparently primary lesions will develop junctiva or lacrimal glands, sparing the orbit.
disease elsewhere within a few years. The course is variable and rel- {z Local lymph nodes should be palpated, but systemic evalu-
atively unpredictable. In some patients, histological features raise ation by an appropriate specialist is required.
suspicion of malignancy and yet the lesion resolves spontaneously zzInvestigation
or with steroid treatment. Conversely, what appears to be reac- {z Orbital imaging, usually with MRI (Fig. 4.41D).
tive lymphoid hyperplasia may be followed by the development of {z Biopsy is usually performed to establish the diagnosis.
lymphoma. Small lesions and those involving only the conjunctiva {z Systemic investigation to establish the extent of disease.!
have the best prognosis. Conjunctival and intraocular lymphomas
are discussed in Ch. 20.!

A B

C D

Fig. 4.41 Orbital lymphoma. (A) Histology showing neoplastic lymphoid cells; (B) involvement of the superior orbit causing prop-
tosis and inferior dystopia; (C) anterior lesion; (D) axial, fat-saturated, gadolinium-enhanced T1-weighted MRI showing a tumour
surrounding the right optic nerve sheath and extending to the orbital apex. (Courtesy of J Harry and G Misson, from Clinical
Ophthalmic Pathology, Butterworth-Heinemann 2001 – fig. A; A Pearson – fig. B.)

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148 Rhabdomyosarcoma

A B

C D

Fig. 4.42 Rhabdomyosarcoma. (A) Histology showing a differentiated tumour in which the
rhabdomyoblast in the centre of the field has cross striations (Masson trichrome stain); (B)
massive superiorly located lesion; (C) antero-inferiorly located lesion; (D) axial CT showing
bony destruction and intracranial spread. (Courtesy of J Harry and G Misson, from Clinical
Ophthalmic Pathology, Butterworth-Heinemann 2001 – fig. A; G Rose – fig. B; S Chen – fig. D.)

Treatment have been identified, including variants of the RB1 gene respon-
sible for retinoblastoma. Emerging evidence supports the role of
Radiotherapy is used for localized lesions and chemotherapy for dis-
epigenetic dysregulation in rhabdomyosarcoma. Four subtypes are
seminated disease and some subtypes. Immunotherapy (e.g. ritux-
recognized:
imab) is a newer modality that may assume a dominant role in the
zzEmbryonal constitutes the majority (85%) of orbital lesions.
future. Occasionally a well-defined orbital lesion may be resected.!
Cells may show light microscopic features of striated muscle
differentiation (Fig. 4.42A). Embryonal usually carries a good
RHABDOMYOSARCOMA prognosis.
zzAlveolar makes up most of the balance of orbital RMS. Fewer
cells show skeletal muscle differentiation than embryonal and
Introduction
the prognosis is worse. Particular chromosomal translocations
Rhabdomyosarcoma (RMS) is the most common so% tissue sar-
are characteristic on cytogenetic analysis of biopsy material.
coma of childhood: 40% develop in the head and neck. It is the
zzBotyroid (4%) and pleomorphic RMS are much less common
most common primary orbital malignancy in children but is still
in the orbit.
a rare condition. Approximately 90% occur in children under 16
years and the average age of onset is 7 years. The tumour is derived
from undifferentiated mesenchymal cells that have the potential to TIP Rhabdomyosarcoma can mimic an orbital infection in a
differentiate into striated muscle. Various genetic predispositions
child.
!

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Orbit 149

Diagnosis METASTATIC TUMOURS

zzSymptoms. Rapidly progressive unilateral proptosis is usual
and may mimic an inflammatory condition such as orbital Adult metastatic tumours
cellulitis.
zzSigns
Introduction
{z The tumour is most commonly superonasal or superior Orbital metastases are an infrequent cause of proptosis and are much
(Fig. 4.42B), but may arise anywhere in the orbit, includ- less common than metastases to the choroid. If the orbit is the site of
ing inferiorly (Fig. 4.42C). It can also arise in other tissues, initial manifestation of the tumour, the ophthalmologist may be the
such as conjunctiva and uvea. first specialist to see the patient. In approximate order of frequency,
{z Swelling and redness of overlying skin develop but the skin the most common primary sites are breast (up to 70%), bronchus,
is not warm. prostate, skin (melanoma), gastrointestinal tract and kidney.!
{z Diplopia is frequent, but pain is less common.
zzInvestigation
Diagnosis
{z MRI shows a poorly defined mass. zzSigns. Associated with the range of tumours that can spread to
{z CT shows a poorly defined mass of homoge- the orbit, presentation can take a variety of forms.
{z Dystopia and proptosis (Fig. 4.43A) are the most common
neous density, often with adjacent bony destruction
(Fig. 4.42D). features.
{z Incisional biopsy is performed to confirm the diagnosis {z Infiltration of orbital tissues characterized by ptosis, dip-
and establish the histopathological subtype and cytoge- lopia, brawny indurated periorbital skin and a firm orbit,
netic characteristics. with resistance to manual retropulsion of the globe.
{z Systemic investigation for metastasis should be performed, {z Enophthalmos with scirrhous tumours.
{z Chronic inflammation.
with lung and bone being the most common sites.!
{z Primarily with cranial nerve involvement (II, III, IV, V, VI)
and only mild proptosis with orbital apex lesions.
Treatment zzInvestigation
Commonly used guidelines for staging and a corresponding treat- {z Imaging: CT (Fig. 4.43B) and MRI typically show a non-
ment protocol were produced by the Intergroup Rhabdomyosar- encapsulated mass.
coma Study Group (IRSG). Treatment encompasses a combination {z Fine needle biopsy is useful for histological confirmation.
of radiotherapy, chemotherapy and sometimes surgical debulk- If this fails, open biopsy may be required.
ing. A single oncogenic fusion gene is associated with treatment {z A search for a primary must be carried out if the patient
resistance and a 40–45% decrease in overall survival. Entinostat was not previously known to have cancer.!
with chemotherapy may improve the prognosis in selected cases
of rhabdomyosarcoma, but has not yet been approved for clinical Treatment
use. The prognosis for patients with disease confined to the orbit Treatment is aimed at preserving vision and relieving pain, because
is good.! most patients die within a year (average 4 months). Radiotherapy

A B

Fig. 4.43 Metastatic renal carcinoma. (A) Proptosis; (B) axial CT showing a non-encapsulated
retrobulbar mass.

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150 Metastatic Tumours

A B

Fig. 4.44 Neuroblastoma in a child. (A) Abdominal CT scan showing a large retroperitoneal
mass (arrows) with calcification, elevating the liver; (B) coronal MRI T2-weighted FLAIR
sequence, showing a secondary tumour in the right upper orbit. (Courtesy of E Blanco.)

is the mainstay of local treatment, but systemic therapy may also

be of benefit. Surgical excision of the focus is occasionally carried
out. Orbital exenteration is usually only performed if other modal-
ities fail to control intolerable symptoms.!

Childhood metastatic tumours

Neuroblastoma
Neuroblastoma is one of the most common childhood malignan-
cies. It arises from neural crest-derived tissue of the sympathetic
nervous system, most commonly in the abdomen (Fig. 4.44A).
Presentation is usually in early childhood. In almost half of all
cases the tumour is disseminated at diagnosis, when it carries a
very poor prognosis. Orbital metastases may be bilateral and typi-
cally present with the abrupt onset of proptosis accompanied by a
superior orbital mass and lid ecchymosis. MRI shows the orbital Fig. 4.45 Advanced maxillary carcinoma showing facial swell-
tumour (Fig. 4.44B).! ing and upward dystopia.

Myeloid sarcoma consists of unilateral or bilateral osteolytic lesions and so% tissue
involvement, typically in the superotemporal quadrant. Patients
Myeloid sarcoma (granulocytic sarcoma) is a solid tumour com-
with solitary lesions tend to have a more benign course and
posed of malignant cells of myeloid origin. Because the tumour
respond well to treatment with local curettage and intralesional
may exhibit a characteristic green colour it was formerly referred
steroid injection or radiotherapy. Systemic disease has a worse
to as chloroma. Myeloid sarcoma may occur as a manifestation
prognosis.!
of established myeloid leukaemia, or it may precede the disease.
Orbital involvement usually presents at about age 7 years with the
rapid onset of proptosis, sometimes bilateral and can be associ- Orbital invasion from adjacent
ated with ecchymosis and lid oedema. When orbital involvement
precedes systemic leukaemia, the diagnosis may be difficult.!
structures
Langerhans cell histiocytosis Sinus tumours
Langerhans cell histiocytosis is a rare group of disorders due Malignant tumours of the paranasal sinuses, although rare, may
to clonal proliferations of histiocytes. Presentation ranges from invade the orbit and carry a poor prognosis unless diagnosed early.
localized disease, usually with bone destruction (eosinophilic It is therefore important to be aware of both their otorhinolaryn-
granuloma), through multifocal bone involvement to a fulminant gological and ophthalmic features.
systemic disease. So% tissues are less commonly involved, but zzMaxillary carcinoma is by far the most common sinus tumour
cutaneous and visceral lesions may occur. Orbital involvement to invade the orbit (Fig. 4.45).

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Orbit 151

A B

Fig. 4.46 Fibrous dysplasia of the orbit. (A) Upward displacement of the right eye; (B) coronal
CT scan showing involvement of the floor and medial wall of the right orbit. (Courtesy of
A Pearson.)

A B

Fig. 4.47 (A) Orbital invasion by conjunctival melanoma; (B) orbital invasion by retinoblastoma.

{z Otorhinolaryngological manifestations include facial pain effect usually in childhood or early adulthood. Within the
and swelling, epistaxis and nasal discharge. orbital region this may cause facial asymmetry, proptosis, dys-
{z Ophthalmic features include upward dystopia, diplopia topia (Fig. 4.46) and visual loss. Most orbital disease is due to
and epiphora. the monostotic form. Polyostotic disease is associated with
zzEthmoidal carcinoma may cause lateral dystopia. endocrine disorders and cutaneous pigmentation (McCune–
zzNasopharyngeal carcinoma may spread to the orbit through Albright syndrome – 10% of cases).!
the inferior orbital fissure; proptosis is a late finding.!
Orbital invasion from eyelid,
Bony invasion
zzIntracranial meningioma arising from the sphenoid ridge
conjunctival and intraocular tumours
may invade the orbit by direct spread and cause proptosis (see zzOrbital invasion may occur from eyelid malignancies
Fig. 19.58). Occasionally tumours arising from the tubercu- such as basal cell carcinoma, squamous cell carcinoma or
lum sellae or olfactory groove may invade the orbit through sebaceous gland carcinoma, from conjunctival tumours
the superior orbital fissure or optic canal. (e.g. melanoma – Fig. 4.47A) and from intraocular
zzFibrous dysplasia is a disorder in which fibrous tissue devel- tumours such as choroidal melanoma or retinoblastoma
ops instead of normal bone, leading to weakening and a mass (Fig. 4.47B).!

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152 The Anophthalmic Socket

B C

Fig. 4.48 Silent sinus syndrome. (A) Left enophthalmos and ptosis; (B) left enophthalmos;
(C) coronal T2-weighted MRI showing opacification and volume loss of left maxillary sinus.
(Courtesy of JH Norris.)

zzAfter severe trauma where the risk of sympathetic ophthalmi-

SILENT SINUS SYNDROME tis may outweigh any prospect of visual recovery (see Ch. 22).
In this rare condition, which usually affects middle-aged people, zzBlind painful or unsightly eyes can also be managed by enu-
there is spontaneous asymptomatic collapse of the maxillary sinus cleation, although evisceration is generally considered the pro-
and orbital floor secondary to a negative pressure in the sinus. The cedure of choice.!
same problem may sometimes involve the frontal and ethmoidal
sinuses. The diagnosis is confirmed by undertaking a CT scan. Evisceration
Evisceration refers to removal of the entire contents of the globe, whilst
Signs the sclera and extraocular muscles remain intact (Fig. 4.49). The cor-
zzFacial asymmetry. nea is removed to provide access to the ocular contents. Retention of
zzEnophthalmos (Fig. 4.48A and B). the sclera and lack of disruption of the extraocular muscles provides
zzDiplopia secondary to inferior oblique muscle entrapment better motility than is achieved a%er enucleation. Evisceration pro-
may rarely occur. vides disrupted and incomplete material for histology and should not
zzMRI shows maxillary sinus outlet obstruction, sinus opacifica- be undertaken in the presence of suspected intraocular malignancy.!
tion and sinus volume loss (Fig.4.48C).!
Exenteration
Treatment Exenteration involves removal of the globe together with the so%
zzEarly surgery produces better results than delayed surgery. tissues of the orbit (Fig. 4.50). Indications include:
zzSurgical treatment involves restoring sinus function by zz Orbital malignancy, either primary or where a tumour has
improving the drainage from the sinus. Reconstruction of the invaded the orbit from the eyelids, conjunctiva, globe or adnexa,
orbital floor can be undertaken at the same time, but most when other forms of treatment have a very poor chance of suc-
authorities recommend waiting a further 6–8 months, as spon- cess. Anteriorly sited tumours may allow relative sparing of pos-
taneous remodeling of the orbital floor o%en occurs.! terior orbital tissue and posterior tumours may allow sparing of
eyelid skin to line the socket. Following exenteration, prostheses
can be attached to the surrounding skin with adhesive, mounted
THE ANOPHTHALMIC SOCKET on glasses (Fig. 4.51), or secured with osseo-integrated magnets
mounted on the orbital rim bones. The socket may be lined with
Surgical procedures skin or a split-skin gra%, or le% to heal by secondary intention.
Removal of an eye or the contents of the orbit may be indicated zzNon-malignant disease such as orbital mucormycosis is a rare
for intraocular or orbital malignancy or where the eye is blind and indication.!
painful or unsightly. A number of different surgical and rehabilita-
tion techniques are available.
Rehabilitation
Enucleation
Enucleation (removal of the globe) is indicated in the following Cosmetic shell
circumstances: A cosmetic shell is an ocular prosthesis that is used to cover a
zzPrimary intraocular malignancies where other treatment phthisical or unsightly eye. The shell can restore volume and o%en
modalities are not appropriate. The tumour is le% intact within provides a good cosmetic appearance, with reasonable motility as
the eye for histopathological examination. a result of transmitted movements from the globe.!
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CHAPTER
Orbit 153

A B

C D

E F

Fig. 4.49 Technique of evisceration. (A) Perilimbal conjunctival incision; (B) cornea removed
with scissors; (C) evisceration spoon inserted between the uveal tract and sclera; (D) relieving
incision in anterior sclera; (E) 22"mm ball is inserted with introducer; (F) anterior sclera closed
with 6-0 absorbable sutures, followed by Tenon capsule and conjunctiva closed in two lay-
ers with 7-0 absorbable sutures. (Courtesy of AG Tyers and JRO Collin, from Colour Atlas of
Ophthalmic Plastic Surgery, Butterworth-Heinemann 2001.)

Orbital implants prosthetic eye without an underlying orbital implant does not
zzOrbital volume reduction. Enucleation or evisceration leads provide a satisfactory solution, due to stretching of the lower
to a reduction in volume of the orbital contents. A large lid under its weight and to poor motility.

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154 The Anophthalmic Socket

A B

Fig. 4.50 Exenteration. (A) Including eyelid removal, 2 days postoperatively; (B) patient in (A)
6 months later. (Courtesy of S Chen.)

A B

Fig. 4.51 (A) Healed exenteration; (B) prosthesis attached to glasses. (Courtesy of A Pearson.)

A B

Fig. 4.52 (A) Right post-enucleation socket syndrome (PESS); (B) extruding ball implant.

zz An implant is usually inserted during the surgery at which the eye e.g. polyethylene, hydroxyapatite). Fibrovascular ingrowth into the
is removed, though secondary placement can be performed later or latter facilitates motility of an overlying prosthesis.
a previously inserted implant exchanged. Implant materials may be zz Post-enucleation socket syndrome (PESS) describes a combi-
solid (‘non-integrated’, e.g. silicone, acrylic) or porous (‘integrated’, nation of signs that occur as a consequence of a volume deficit of

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CHAPTER
Orbit 155

the orbital tissues. PESS is particularly common if an implant is

not used. Some months a%er enucleation, the upper lid loses its
support and drops down over the prosthesis. In time, the pros-
thesis tilts backwards and turns upwards, which puts forward
pressure on the lower eyelid shallowing the fornix and causing
drooping of the lower lid (Fig. 4.52A). The main features are:
{z Enophthalmos.
{z Upper lid ptosis.
{z Superior sulcus hollowing.
{z Sagging lower lid.
{z Surgical options include: (a) the insertion of a secondary
implant if there is an absence of an implant; (b) the inser-
tion of a silicone block into the inferior orbit if there is an
implant; or (c) the implantation of a dermis-fat gra%.
zzA peg can also be inserted into porous implants to improve A
later motility, though the peg must be covered in situ by socket
tissue and cannot attach directly to the overlying prosthesis.
The motility of unpegged implants is also usually good, par-
ticularly if donor sclera or a mesh wrap is used and the extra-
ocular muscles secured to the surface.
zzExtrusion (Fig. 4.52B) is a significant concern with all
implants. Careful placement of an implant, ensuring it is suf-
ficiently deep and is well covered with vascularized tissue, is
more important than the choice of implant material.!

Ocular prosthesis
A%er enucleation or evisceration, a conformer (Fig. 4.53A) made
of silicone or acrylic material is placed to support the conjunctival
fornices and remains in place until the socket is fitted with an arti-
ficial eye (Fig. 4.53B). Initial socket impression moulds can usu-
ally be taken at around 6–8 weeks postoperatively and a temporary B
artificial eye placed whilst waiting for manufacture of a prosthesis
shaped to fit the individual socket and matched to the fellow eye
(Fig. 4.53C and D).!

CRANIOSYNOSTOSES
The craniosynostoses are a group of rare congenital conditions in
which an abnormally shaped skull results from premature closure C
of skull sutures.
zz Crouzon syndrome features a short anteroposterior skull diam-
eter, with midfacial hypoplasia giving a prominent lower jaw
(Fig. 4.54A). Proptosis due to shallow orbits and hypertelorism
(wide orbital separation) are the most conspicuous ocular fea-
tures (Fig. 4.54B). Vision-threatening complications include
exposure keratopathy and optic atrophy, mechanisms including
chronic papilloedema and cerebral hypoperfusion secondary to D
sleep apnoea. Strabismus (‘V’ exotropia – see Fig. 4.54B), ame-
tropia and amblyopia can occur and other ocular associations Fig. 4.53 (A) Conformer in place; (B) a selection of artificial
eyes; (C) empty socket; (D) matching prosthesis in place.
have been reported. Inheritance is usually autosomal dominant (Courtesy of C Barry – fig. B; S Chen – figs C and D.)
(AD); allelic variants in the gene FGFR2 are responsible.
zzApert syndrome is the most severe of the craniosynosto- in the majority of cases the condition is sporadic and associ-
ses. Oxycephaly (conical skull), midfacial hypoplasia with a ated with older parental age. As with Crouzon syndrome, it is
beaked nose and low-set ears (Fig. 4.54C), syndactyly (Fig. frequently the result of mutations in FGFR2.
4.54D) and developmental delay (30%) may be present. Shal- zzPfeiffer syndrome features midfacial hypoplasia and down-
low orbits, proptosis and hypertelorism are generally less slanting palpebral fissures (Fig. 4.54E and F). Ocular features
pronounced than in Crouzon syndrome, but the same vision- are similar to Apert syndrome. Inheritance is AD with genetic
threatening complications occur. Inheritance can be AD, but heterogeneity.
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156 Craniosynostoses

A B

C D

E F

Fig. 4.54 Craniosynostosis. (A) Proptosis in Crouzon syndrome; (B) proptosis and hyper-
telorism – a ‘V’ exotropia is also shown; (C) Apert syndrome showing mild shallow orbits, mid-
facial hypoplasia and ‘parrot-beak’ nose and (D) syndactyly; (E) Pfeiffer syndrome showing
midfacial hypoplasia and down-slanting palpebral fissures and (F) broad big toe. (Courtesy of
Ken K Nischal – figs E and F.)

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 Chapter

Dry Eye 5
INTRODUCTION 158 INVESTIGATION 161 Anti-inflammatory agents 167
Definitions 158 Tear film break-up time 163 Contact lenses 167
Physiology 158 Optimization of environmental
TREATMENT 165 humidity 167
Classification 159
Strategy 165 Miscellaneous options 167
SJÖGREN SYNDROME 160 Tear substitutes 166
Clinical features 160 Punctal occlusion 166

157
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158 Introduction

INTRODUCTION Lipid layer

zzComposition
Definitions {z The outer lipid layer is composed of a polar phase contain-
ing phospholipids adjacent to the aqueous-mucin phase
Dry eye occurs when there is inadequate tear volume or function,
and a non-polar phase containing waxes, cholesterol esters
resulting in an unstable tear film and ocular surface disease. It is
and triglycerides.
an extremely common condition, particularly in postmenopausal {z The polar lipids are bound to lipocalins within the aqueous
women and the elderly.
layer. These are small secreted proteins that have the ability
zzDry eye disease is a multifactorial disease of the ocular surface
to bind hydrophobic molecules and may also contribute to
and tear film accompanied by increased osmolarity of the tear
tear viscosity.
film and inflammation of the ocular surface. {z Lid movement during blinking is important in releas-
zzKeratoconjunctivitis sicca (KCS) refers to any eye with some
ing lipids from glands. The thickness of the layer can be
degree of dryness.
increased by forced blinking and conversely reduced by
zzXerophthalmia describes a dry eye associated with vitamin A
infrequent blinking.
deficiency.
zzFunctions
zzXerosis refers to the extreme ocular dryness and keratiniza-
{z To prevent evaporation of the aqueous layer and maintain
tion that occurs in eyes with severe conjunctival cicatrization.
tear film thickness.
zzSjögren syndrome is an autoimmune inflammatory disease of
{z To act as a surfactant allowing spread of the tear film.
which dry eyes is a feature.! {z Deficiency results in evaporative dry eye.!

Physiology Aqueous layer

zzSecretion
Tear film constituents {z Themain lacrimal glands produce about 95% of the aque-
The tear film has three layers (Fig. 5.1): ous component of tears and the accessory lacrimal glands
zzLipid layer secreted by the meibomian glands. of Krause and Wolfring produce the remainder.
zzAqueous layer secreted by the lacrimal glands. {z Secretion of tears has basic (resting) and much greater
zzMucous layer secreted principally by conjunctival goblet cells. reflex components. The latter occurs in response to corneal
The constituents are complex, with as many as a hundred dis- and conjunctival sensory stimulation, tear break-up and
tinct proteins identified.! ocular inflammation and is mediated via the fi%h cranial
nerve. It is reduced by topical anaesthesia and falls during
Spread of the tear film sleep. Secretion can increase 500% in response to injury.
The tear film is mechanically distributed over the ocular surface zzComposition
through a neuronally controlled blinking mechanism. Three fac- {z Water, electrolytes, dissolved mucins and proteins.
tors are required for effective resurfacing of the tear film: {z Growth factors derived from the lacrimal gland, the pro-
zzNormal blink reflex. duction of which increases in response to injury.
zzContact between the external ocular surface and the eyelids. {z Pro-inflammatory interleukin cytokines that accumulate
zzNormal corneal epithelium.! during sleep when tear production is reduced.

Lipid layer
0.1 µm
Meibomian
glands
Lacrimal
gland

Aqueous layer
7.0 µm

Goblet cells
Mucous layer
0.2 µm

Fig. 5.1 The three layers of the tear film.

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 CHAPTER
Dry Eye 5 159

zzFunctions CHAPTER
{z To provide atmospheric oxygen to the corneal epithelium.
Classification
{z Antibacterial activity due to proteins such as IgA, lyso- The classification of dry eye usually applied is that of the 2007
zyme and lactoferrin. International Dry Eye Workshop (DEWS), with a basic division
{z To wash away debris and noxious stimuli and facilitate the into aqueous-deficient and evaporative types. Most individuals
transport of leukocytes a%er injury. have considerable overlap between mechanisms and it is impor-
{z To optically enhance the corneal surface by abolishing tant to be aware during patient assessment of the likely presence of
minute irregularities.! multiple contributory factors.

Mucous layer Aqueous-deficient

zzComposition zzSjögren syndrome dry eye (primary or secondary).
{z Mucins are high-molecular-weight glycoproteins that may zzNon-Sjögren syndrome dry eye.
be transmembrane or secretory in type. {z Lacrimal deficiency: primary (e.g. age-related dry eye,
{z Secretory mucins are further classified as gel-forming or congenital alacrima, familial dysautonomia) or second-
soluble. They are produced mainly by conjunctival goblet ary (e.g. inflammatory and neoplastic lacrimal gland
cells but also by the lacrimal glands. infiltration, acquired immunodeficiency syndrome
{z The superficial epithelial cells of the cornea and conjunc- (AIDS), gra%-versus-host disease, lacrimal gland or nerve
tiva produce transmembrane mucins that form their gly- ablation).
cocalyx (extracellular coating). {z Lacrimal gland duct obstruction, e.g. trachoma, cica-
{z Staining of diseased epithelium with rose Bengal indicates tricial pemphigoid, chemical injury, Stevens–Johnson
that the transmembrane and gel mucous layers are absent syndrome.
and the cell surface exposed. Damage to the epithelial cells {z Reflex hyposecretion: sensory (e.g. contact lens wear,
will prevent normal tear film adherence. diabetes, refractive surgery, neurotrophic keratitis) or
zzFunctions motor block (e.g. seventh cranial nerve damage, systemic
{z To permit wetting by converting the corneal epithelium drugs).!
from a hydrophobic to a hydrophilic surface.
{z Lubrication. Evaporative
{z Deficiency of the mucous layer may be a feature of zzIntrinsic
both aqueous deficiency and evaporative states. Gob- {z Meibomian gland deficiency, e.g. posterior blepharitis,
let cell loss occurs with cicatrizing conjunctivitis, vita- rosacea.
min A deficiency, chemical burns and toxicity from {z Disorders of lid aperture, e.g. excessive scleral show, lid
medications.! retraction, proptosis, facial nerve palsy.
{z Low blink rate, e.g. Parkinson disease, prolonged com-
Regulation of tear film components puter monitor use, reading, watching television.
zzHormonal {z Drug action, e.g. antihistamines, beta-blockers, antispas-
{z Androgens are the prime hormones responsible for regula- modics, diuretics.
tion of lipid production. zzExtrinsic
{z Oestrogens and progesterone receptors in the conjunctiva {z Vitamin A deficiency.
and the lacrimal glands are essential for the normal func- {z Topical drugs including the effect of preservatives.
tion of these tissues. {z Contact lens wear.
zzNeural via fibres adjacent to the lacrimal glands and goblet {z Ocular surface disease, such as allergic conjunctivitis.
cells that stimulate aqueous and mucus secretion.!

Mechanism of disease TIP Whether a patient has primary aqueous deficiency

or evaporative dry eye, the end result is ocular surface
The four core inter-related mechanisms thought to be respon-
sible for the manifestations of dry eye are tear instability, tear
inflammation and discomfort.
!
hyperosmolarity, inflammation and ocular surface damage.
Inflammation in the conjunctiva and accessory glands as well as
the ocular surface is present in 80% of patients with KCS and Effect of environmental factors
may be both a cause and consequence of dry eye, amplifying As well as the basic classification, DEWS draws attention to the
and perpetuating disease. The presence of inflammation is the effect of the environment on the type of dry eye with which a
rationale for specific anti-inflammatory measures such as steroid patient presents. These can be both internal factors, such as age,
therapy. There is a strong association between dry eye syndrome hormonal status and behaviour patterns, and external, such as the
and reduced levels of systemic androsterone sulphate and epian- exacerbation of evaporative factors in an atmosphere with low
drosterone sulphate.! relative humidity.!

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160 Sjögren Syndrome

SJÖGREN SYNDROME
Sjögren syndrome (SS) is an autoimmune disorder character-
ized by lymphocytic inflammation and destruction of lacrimal
and salivary glands (Fig. 5.2A) and other exocrine organs. The
classic clinical triad consists of dry eyes, dry mouth (Fig. 5.2B)
and parotid gland enlargement (Fig. 5.2C), but other features
are common and can affect all organ systems. The condition is
classified as primary when it exists in isolation and secondary
when associated with another disease, commonly rheumatoid
arthritis or systemic lupus erythematosus. Primary SS affects
females more frequently than males. Although in clinical prac-
tice the diagnosis may be made on less stringent grounds, the
American College of Rheumatology (ACR) criteria for diagno-
sis specify, in patients with a clinical picture suggestive of SS, A
the following:
zzPositivity for anti-SSA or anti-SSB antibodies, or positive
rheumatoid factor together with significantly positive anti-
nuclear antibody.
zzOcular surface staining above a certain grade.
zzFocal lymphocytic sialadenitis to a specified extent on salivary
gland biopsy (see Fig. 5.2A).
Widely used but older American-European Consensus Group
criteria are more extensive and include more clinical findings, but
give results consistent with the ACR criteria.
Treatment options for SS include a range of symptomatic
treatments for dry eye, as discussed below, dry mouth and other
manifestations, salivary stimulants (e.g. oral pilocarpine) and, in
some cases, immunosuppression and biological blockers such as
rituximab.
B

Clinical features
Symptoms
The most common ocular symptoms are feelings of dryness, gritti-
ness and burning that characteristically worsen over the course of
the day. Stringy discharge, transient blurring of vision, redness and
crusting of the lids are also common. Lack of emotional or reflex
tearing is unusual. The symptoms of KCS are frequently exacerbated
on exposure to conditions associated with increased tear evapora-
tion (e.g. air-conditioning, wind and central heating) or prolonged
reading or video display unit use, when blink frequency is reduced.

TIP The symptoms associated with keratoconjunctivitis sicca

are exacerbated by exposure to wind, air-conditioning and
central heating.
! C

Fig. 5.2 Sjögren syndrome. (A) Histology of the lacrimal gland

Signs showing lymphocytic infiltration; (B) dry fissured tongue; (C)
left parotid gland enlargement. (Courtesy of J David – fig. C.)
zzPosterior (seborrhoeic) blepharitis with meibomian gland
dysfunction is o%en present (Fig. 5.3A and B). {z Keratinization.
zzConjunctiva {z Conjunctivochalasis is a common response to, and exac-
{z Redness. erbating factor for, the chronic irritation of dry eye, such
{z Staining with rose Bengal (Fig. 5.4A) and lissamine green that a self-sustaining cycle is maintained. It also commonly
(Fig. 5.4B). occurs in other ocular surface diseases (see Ch. 6).

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 CHAPTER
Dry Eye 5 161

CHAPTER

A B

Fig. 5.3 Posterior blepharitis in dry eye. (A) Oil globules at meibomian gland orifices;
(B) inflamed meibomian gland.

A B

Fig. 5.4 Conjunctival staining in dry eye. (A) Rose Bengal; (B) lissamine green.

zzTear film zzComplications can be vision-threatening and include epithe-

{z In the normal eye, as the tear film breaks down, the mucin lial breakdown, melting (Fig. 5.7A), perforation (Fig. 5.7B)
layer becomes contaminated with lipid but is washed away. and occasionally bacterial keratitis (Fig. 5.7C).
{z In the dry eye, the lipid-contaminated mucin accumulates
in the tear film as particles and debris that move with each
blink (Fig. 5.5A). TIP A patient with symptomatic dry eye syndrome may
{z The marginal tear meniscus (strip) is a crude measure present with superficial punctate keratitis, which can be seen
of the volume of aqueous in the tear film. In the normal clinically with fluorescein staining.
!
eye the meniscus is 0.2–0.4 mm in height, but in dry eye
becomes thin (less than 0.25 mm) or absent (Fig. 5.5B).
zzCornea
zzPunctate epithelial erosions that stain well with fluorescein
INVESTIGATION
(Fig. 5.6A). The aim of investigation is to confirm and quantify a clinical diag-
zzFilaments consist of strands of mucus and debris (such as shed nosis of dry eye. Although the symptoms in a given patient do
epithelial cells) and are typically attached at one end to the not change, objective signs can fluctuate. The correlation between
corneal surface (Fig. 5.6B). The filaments stain well with rose symptoms and tests is poor. However, the reliability of tests
Bengal (Fig. 5.6C), but less so with fluorescein. improves as the severity of dry eye increases. The tests measure the
zzMucous plaques with similar constituents may occur in severe following parameters:
dry eye. They consist of semi-transparent, white-to-grey, o%en zzStability of the tear film as related to its break- up time
slightly elevated lesions of varying size (Fig. 5.6D). (BUT).

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162 Investigation

A B

Fig. 5.5 Tear film abnormalities in dry eye. (A) Mucous debris; (B) thin marginal tear
meniscus.

A B

C D

Fig. 5.6 Corneal signs in dry eye. (A) Punctate erosions stained with fluorescein; (B) corneal
filaments; (C) mild (rose Bengal stain) and (D) severe mucous plaque formation. (Courtesy of
S Tuft – fig. B; R Bates – fig. D.)

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 CHAPTER
Dry Eye 5 163

zzTear production (Schirmer, fluorescein clearance and tear CHAPTER

osmolarity).
zzOcular surface disease (corneal stains and impression
cytology).
There is no clinical test that allows the diagnosis of evaporative
dry eye to be definitively confirmed. It is therefore a presumptive
diagnosis based on the presence of associated clinical findings. It
is suggested the tests are performed in the following order because
the Schirmer strip paper can damage the ocular surface and cause
staining.

Tear film break-up time

The tear film BUT is abnormal in aqueous tear deficiency and mei-
A bomian gland disorders. It is measured as follows:
zzFluorescein 2% or an impregnated fluorescein strip moistened
with non-preserved saline is instilled into the lower fornix.
zzThe patient is asked to blink several times.
zzThe tear film is examined at the slit lamp with a broad beam
using the cobalt blue filter. A%er an interval, black spots or
lines appear in the fluorescein-stained film (Fig. 5.8A), indi-
cating the formation of dry areas.
zzThe BUT is the interval between the last blink and the appear-
ance of the first randomly distributed dry spot. A BUT of less
than 10 seconds is suspicious.
The development of dry spots always in the same location may
indicate a local corneal surface abnormality (e.g. epithelial base-
ment membrane disease) rather than an intrinsic instability of the
tear film.

B Schirmer test
The Schirmer test is a useful assessment of aqueous tear produc-
tion. The test involves measuring the amount of wetting of a spe-
cial (no. 41 Whatman) filter paper, 5 mm wide and 35 mm long.
The test can be performed with or without topical anaesthesia.
In theory, when performed with an anaesthetic (Schirmer 2)
basic secretion is measured and without anaesthetic (Schirmer
1) it measures maximum basic plus reflex secretion. In practice,
however, topical anaesthesia cannot abolish all sensory and psy-
chological stimuli for reflex secretion. The test is performed as
follows:
zzExcess tears are delicately dried. If topical anaesthesia is
applied the excess should be removed from the inferior fornix
with filter paper.
zzThe filter paper is folded 5 mm from one end and inserted at
the junction of the middle and outer third of the lower lid, tak-
C ing care not to touch the cornea or lashes (Fig. 5.8B).
zzThe patient is asked to keep the eyes gently closed.
Fig. 5.7 Severe corneal complications of dry eye. (A) Melting
zzA%er 5 minutes the filter paper is removed and the amount of
(arrow); (B) perforation with iris plugging; (C) bacterial infection.
(Courtesy of S Tuft – fig. B; T Carmichael – fig. C.) wetting from the fold measured.
zzLess than 10 mm of wetting a%er 5 minutes without anaesthesia
or less than 6 mm with anaesthesia is considered abnormal.
TIP There is a poor correlation between symptoms and Results can be variable and a single Schirmer test should not
clinical tests in patients with dry eye syndrome. However, the be used as the sole criterion for diagnosing dry eye, but repeatedly
reliability of tests improves as the severity increases.
abnormal tests are highly supportive.!

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164 Investigation

A B

C D

E F

Fig. 5.8 Diagnostic tests in dry eye. (A) Tear film break-up time – numerous dry spots are pre-
sent in a fluorescein-stained tear film; (B) Schirmer test; (C) corneal and conjunctival staining
with rose Bengal and (D) with fluorescein; (E) TearLab instrument used to measure tear film
osmolarity; (F) technique of obtaining a tear sample. (Courtesy of G Ratnarajan – figs E and F.)

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 CHAPTER
Dry Eye 5 165

Ocular surface staining zzTear meniscometry is a technique to quantify the height and CHAPTER
zzFluorescein stains corneal and conjunctival epithelium (see thus the volume of the lower lid meniscus.
Fig. 5.6A) where there is sufficient damage to allow the dye to zzImpression cytology can determine goblet cell numbers.
enter the tissues.
zzRose Bengal is a dye that has an affinity for dead or devitalized
epithelial cells that have a lost or altered mucous layer (Fig.
TIP Measurement of tear film osmolarity is a useful tool when
used to confirm the diagnosis and response to treatment in dry
5.8C). Corneal filaments and plaques (see Fig. 5.6B and D) are
also shown up more clearly by the dye and the use of a red-free
eye syndrome.
!
filter may help visualization. A 1% solution of rose Bengal or a
moistened impregnated strip can be used. The dye may cause
intense stinging that can last for up to a day, particularly in
TREATMENT
patients with severe KCS. To minimize irritation a very small Strategy
drop should be used, immediately preceded by a drop of topi-
cal anaesthetic and the excess washed out with saline. The underlying causative processes of dry eye are generally not
zzLissamine green stains in a similar fashion to rose Bengal but reversible, and management is therefore structured around the
causes less irritation and may be preferred (see Fig. 5.4B). control of symptoms and the prevention of surface damage.
zzThe pattern of staining may aid diagnosis: DEWS have produced guidelines based on earlier International
{z Interpalpebral staining of the cornea and conjunctiva (see Taskforce Guidelines for Dry Eye, in which suggested treatment
Fig. 5.4A and B) is common in aqueous tear deficiency. options depend on the level of severity of disease graded from
{z Superior conjunctival stain may indicate superior limbic 1 to 4. The DEWS guidelines can also be applied in a graded
keratoconjunctivitis. approach, proceeding to the next level if the preceding measures
{z Inferior corneal and conjunctival stain is o%en present in are inadequate.
patients with blepharitis or exposure (Fig. 5.8D).
Level 1
zzEducation and environmental/dietary modifications
TIP In dry eye syndrome lissamine green solution is {z Establishment of realistic expectations and emphasis on
preferable to 1% rose Bengal solution as it stains in a similar the importance of compliance.
fashion, but causes less irritation.
! {z Lifestyle review, including the importance of blinking
whilst reading, watching television or using a computer
Other investigations screen (which should be orientated below eye level to
The following tests are rarely performed in clinical practice. minimize palpebral aperture size) and the management of
zzFluorescein clearance test and the tear function index may contact lens wear.
be assessed by placing 5 µl of fluorescein on the ocular surface {z Environmental review, e.g. increasing humidity may be
and measuring the residual dye in a Schirmer strip placed on possible for some environments.
the lower lateral lid margin at set intervals. Delayed clearance {z Instillation aids for eye drops (manufacturer-supplied or
is observed in all dry eye states. makeshi%, such as nut-crackers to hold plastic bottles)
zzTear film osmolarity measurement techniques are available should be advocated for patients with reduced dexterity
and are emerging as an accurate means of diagnosis (Fig. (e.g. rheumatoid arthritis).
5.8E and F). The threshold value that distinguishes between {z Caution the patient that laser refractive surgery can exac-
a healthy eye and an eye with dry eye syndrome varies from erbate dry eye.
305 mOsm/l and 316 mOsm/l, depending on the degree of tear zzSystemic medication review to exclude contributory effects
film instability. A widely accepted threshold is 308 mOsm/l and eliminate offending agents. Discontinuation of toxic/pre-
and a value of 316 mOsm/l appears to discriminate between served topical medication if possible.
mild and moderate/severe dry eye. Tear osmolarity may not zzArtificial tear substitutes, including gels and ointments –
correlate with ocular symptoms, but it does correlate with see below. Some authorities advocate that use of preserved
effective treatment when evaluated in the long term. drops should fall within level 1 and categorize non-preserved
zzTear constituent measurement. Tear samples can be assayed drops as a level 2 measure. Mucolytic agents may be specifi-
for the presence of markers known to be elevated (e.g. matrix cally indicated for some patients.
metalloproteinase-9) or decreased (e.g. lactoferrin) in dry eye. zzEyelid therapy. Basic measures such as warm compresses and
zzPhenol red thread test uses a thread impregnated with a pH- lid hygiene for blepharitis. Reparative lid surgery (e.g. entro-
sensitive dye. The end of the thread is placed over the lower lid pion, ectropion, excessive lid laxity or scleral show) may be
and the length wetted (the dye changes from yellow to red in considered as an early measure. Nocturnal lagophthalmos
tears) is measured a%er 15 seconds. A value of 6 mm is abnor- can be addressed by taping the lids closed at bedtime, wearing
mal. It is comparable to the Schirmer test but takes less time swimming goggles during sleep, or in extreme cases by lateral
to perform. tarsorrhaphy.!

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166 Treatment

Level 2 zzArtificial tear inserts emplaced once or twice daily offer

extended duration treatment and are preferred by some
zzNon-preserved tear substitutes are categorized as level 2
patients.
treatment by some authorities.
zzMucolytic agents. Acetylcysteine 5% drops may be useful in
zzAnti-inflammatory agents such as topical steroids,
patients with corneal filaments and mucous plaques, which
oral omega fatty acids and other agents such as topical
acetylcysteine dissolves; it may cause stinging on instillation.
ciclosporin.
Acetylcysteine is malodorous and has a limited shelf-life. Man-
zzTetracyclines (for meibomianitis, rosacea).
ual debridement of filaments may also be useful.
zzPunctal plugs.
zzPreservatives can be a potent source of toxicity, especially a%er
zzSecretagogues, e.g. pilocarpine, cevimeline, rebamipide.
punctal occlusion. Numerous non-preserved drops are now
zzMoisture chamber spectacles and spectacle side shields.!
available, including some multi-dose products and in general
Level 3 should be used in preference to preservative-containing prepa-
rations in any more than mild disease or with instillation more
zzSerum eye drops. Autologous or umbilical cord serum.
than three or four times daily. If possible, preservative-free for-
zzContact lenses.
mulations should also be used for dry eye patients when other
zzPermanent punctal occlusion.!
topical medication is required, for example in the treatment of
Level 4 glaucoma. Newer preservatives such as Polyquad and Purite
seem to exhibit lower ocular surface toxicity than older agents
zzSystemic anti-inflammatory agents.
such as benzalkonium chloride.
zzSurgery
zzHaemoderivative treatment has been used to manage severe
{z Eyelid surgery, such as tarsorrhaphy.
ocular surface disease including gra%-versus-host-related dry
{z Salivary gland auto-transplantation.
eye disease, Sjögren syndrome, post-LASIK dry eye persistent
{z Mucous membrane or amniotic membrane transplanta-
epithelial defects and recurrent erosions (see below).
tion for corneal complications.!

TIP Excessive use of preserved lubricating eye drops can

Tear substitutes result in corneal toxicity.
!
Tear substitutes have a relatively simple formulation that cannot
approximate the complex components and structure of the nor-
mal tear film. Their delivery is also periodic rather than contin-
Punctal occlusion
uous. Almost all are based on replacement of the aqueous phase Punctal occlusion reduces drainage and thereby preserves natural
of the tear film. There are no mucus substitutes and paraffin is tears and prolongs the effect of artificial tears. It is of greatest value
only an approximation to the action of tear lipids. The optimal in patients with moderate–severe KCS who have not responded to
frequency of instillation varies with agent and with severity. frequent instillation of topical agents.
zzDrops and gels. A large range of preparations is available. One zzTemporary occlusion can be achieved by inserting collagen
agent or category of preparation has not demonstrated supe- plugs into the canaliculi. These dissolve over a number of
riority and particular agents are o%en preferred by individual weeks. The main aim is to ensure that epiphora does not occur
patients with limited rationale. following permanent occlusion.
{z Cellulose derivatives (e.g. hypromellose, methylcellulose) {z Initially the inferior puncta are occluded and the patient is
are appropriate for mild cases. reviewed a%er 1 or 2 weeks.
{z Carbomer gels adhere to the ocular surface and so are {z If the patient is now asymptomatic and without epiphora,
longer-lasting, but some patients are troubled by slight the plugs can be removed and the inferior canaliculi per-
blurring. manently occluded (see below).
{z Other agents include polyvinyl alcohol (PVA), which {z In severe KCS both the inferior and superior canaliculi can
increases the persistence of the tear film and is useful in be plugged.
mucin deficiency, sodium hyaluronate, povidone, glycer- zzReversible prolonged occlusion can be achieved with silicone
ine, propylene glycol, polysorbate and others. (Fig. 5.9) or long-acting (2–6 months) collagen plugs.
{z Diquafosol is a newer agent that works as a topical {z Problems include extrusion, granuloma formation and
secretagogue. distal migration.
zzOintments containing petrolatum (paraffin) mineral oil can {z Plugs that pass into the horizontal portion of the canalicu-
be used at bedtime to supplement daytime drops or gel instilla- lus cannot be visualized and although they can usually be
tion; daytime use is precluded by marked blurring. Some prac- flushed out with saline, if they cause epiphora this is not
titioners do not prescribe these for long-term use. always possible and surgical retrieval may be needed.
zzEyelid sprays are applied to the closed eye and typically con- zzPermanent occlusion should be undertaken only in patients
tain a liposome-based agent that may stabilize the tear film and with severe dry eye who have had a positive response to
reduce evaporation. temporary plugs without epiphora. It should be avoided in

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 CHAPTER
Dry Eye 5 167

CHAPTER

A B

Fig. 5.9 (A) Insertion of a silicone plug (arrow); (B) plug in position (arrow).

patients, especially if young, who may have reversible pathol- behind the lens. In addition, contact lenses are effective at reliev-
ogy. All four puncta should not be occluded at the same time. ing symptoms resulting from secondary corneal changes. How-
{z Permanent occlusion is performed following punctal ever, patients should be cautioned that there is an increased risk
dilatation by coagulating the proximal canaliculus with of bacterial keratitis.
cautery. Following successful occlusion, it is important to Several manufacturers have developed contact lenses specifi-
watch for signs of recanalization. cally designed to reduce dry eye discomfort during lens wear. The
{z Laser cautery seems to be less consistently effective than materials used (silicone hydrogel) retain moisture for 12–16 hours
surgical thermal coagulation. and are single-use daily disposable. Occlusive gas-permeable
scleral contact lenses provide a reservoir of saline over the cornea
and are occasionally used in a patient with an extremely dry eye
TIP Punctal occlusion with a silicone plug can be a useful and exposure. Silicone rubber lenses, which were used in the past,
therapeutic option in patients with moderate–severe are no longer available.!
keratoconjunctivitis sicca.
!
Optimization of environmental humidity
Anti-inflammatory agents zzReduction of room temperature to minimize evaporation of
zzTopical steroids, generally low-intensity preparations such tears.
as fluorometholone are effective supplementary treatment for zzRoom humidifiers may be tried but are frequently disap-
acute exacerbations. The risks of longer-term treatment must pointing because much apparatus is incapable of significantly
be balanced against the potential benefits in each case. increasing the relative humidity of an average-sized room. A
zzOmega fatty acid supplements (e.g. omega-3 fish oil, flax seed temporary local increase in humidity can be achieved with
oil) can have a dramatic effect on symptoms and may facilitate moist chamber goggles or side shields to glasses but may be
the reduction of topical medication. cosmetically unacceptable.!
zzOral tetracyclines for an extended course, o%en 3 months at
a relatively low dose, may control associated blepharitis, espe-
cially meibomianitis and reduce tear levels of inflammatory
Miscellaneous options
mediators. Doxycycline may be preferred to minocycline on zzBotulinum toxin injection to the orbicularis muscle may help
the grounds of adverse effect profile. control the blepharospasm that o%en occurs in severe dry eye.
zzTopical ciclosporin (usually 0.05%) reduces T-cell-mediated Injected at the medial canthus it can also reduce tear drainage,
inflammation of lacrimal tissue, resulting in an increase in the presumably by limiting lid movement.
number of goblet cells and reversal of squamous metaplasia of zzOral cholinergic agonists such as pilocarpine (5 mg four times
the conjunctiva.! daily) and cevimeline may reduce the symptoms of dry eye and
dry mouth in patients with Sjögren syndrome. Adverse effects
including blurred vision and sweating may be less marked with
Contact lenses cevimeline.
Although contact lens wear can exacerbate dry eye, particularly zzSubmandibular gland transplantation for extreme dry eye
due to inflammatory, sensory and evaporative effects, the symp- requires extensive surgery and may produce excessive levels of
toms can be outweighed by the reservoir effect of fluid trapped mucus in the tear film.

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168 Treatment

zzSerum/autologous blood eye drops. Autologous or umbilical zzCenegermin 0.002%, a topical recombinant human nerve
cord serum (20–100%), the blood component remaining a%er growth factor (registered for use in neurotrophic keratopathy)
clotting, has produced subjective and objective improvements may have a role to play in severe Sjögren disease. The safety
in studies in patients with dry eye and may aid the healing of and efficacy are being investigated.
persistent epithelial defects. However, their production and zzPhotobiomodulation therapy applied to the eyelids. Low
storage are associated with practical challenges. Finger prick level light, red light and intense pulsed light are relatively new
autologous blood is a low cost, readily accessible and practical interventional options that may be helpful in the treatment of
treatment that appears to be safe and effective. dry eye secondary to severe meibomian gland dysfunction.

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 Chapter

Conjunctiva 6
INTRODUCTION 170 Vernal keratoconjunctivitis 183 MISCELLANEOUS DISORDERS OF
Anatomy 170 Atopic keratoconjunctivitis 184 THE CONJUNCTIVA 196
Histology 170 Treatment of VKC and AKC 186 Superior limbic
Clinical features of conjunctival Non-allergic eosinophilic keratoconjunctivitis 196
inflammation 170 conjunctivitis 188 Ligneous conjunctivitis 197
Contact allergic Parinaud oculoglandular
BACTERIAL CONJUNCTIVITIS 173 blepharoconjunctivitis 189 syndrome 198
Acute bacterial conjunctivitis 173 Giant (mechanically induced) Factitious conjunctivitis 199
Giant fornix syndrome 174 papillary conjunctivitis 189
Adult chlamydial conjunctivitis 175 DEGENERATIONS 199
Trachoma 176 CONJUNCTIVITIS IN BLISTERING Pinguecula 199
Neonatal conjunctivitis 178 MUCOCUTANEOUS DISEASE 190 Pterygium 200
Mucous membrane Concretions 202
VIRAL CONJUNCTIVITIS 180 pemphigoid 190 Conjunctivochalasis 202
ALLERGIC CONJUNCTIVITIS 182 Stevens–Johnson syndrome/ Retention (primary epithelial
toxic epidermal necrolysis (Lyell inclusion) cyst 202
Acute allergic conjunctivitis 182
syndrome) 193
Seasonal and perennial allergic SUBCONJUNCTIVAL
Graft-versus-host disease 195
conjunctivitis 182 HAEMORRHAGE 202

169
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170 Introduction

zzConjunctiva-associated lymphoid tissue (CALT) is criti-

INTRODUCTION cal in the initiation and regulation of ocular surface immune
responses. It consists of lymphocytes within the epithelial lay-
Anatomy ers, lymphatics and associated blood vessels, with a stromal
The conjunctiva is a transparent mucous membrane that lines the component of lymphocytes and plasma cells, including fol-
inner surface of the eyelids and the anterior surface of the globe, licular aggregates.!
terminating at the corneoscleral limbus. It is richly vascular, sup-
plied by the anterior ciliary and palpebral arteries. There is a dense
lymphatic network, with drainage to the preauricular and sub- Clinical features of conjunctival
mandibular nodes corresponding to that of the eyelids. It has a key inflammation
protective role, mediating both passive and active immunity. Ana-
tomically, it is divided into the following: Symptoms
zzThe palpebral conjunctiva starts at the mucocutaneous junc- Non-specific symptoms include lacrimation, grittiness, stinging
tion of the lid margins and is firmly attached to the posterior and burning. Itching is the hallmark of allergic disease, although
tarsal plates. The tarsal blood vessels are vertically orientated. it may also occur to a lesser extent in blepharitis and in patients
zzThe forniceal conjunctiva is loose and redundant. with dry eye syndrome. The visual acuity is not usually affected.
zzThe bulbar conjunctiva covers the anterior sclera and is con- Significant pain, photophobia or a marked foreign body sensation
tinuous with the corneal epithelium at the limbus. Radial suggest corneal involvement.!
ridges at the limbus form the palisades of Vogt, the likely reser-
voir of corneal stem cells. The stroma is loosely attached to the Discharge
underlying Tenon capsule, except at the limbus, where the two zzWatery discharge is composed of a serous exudate and tears
layers fuse. The plica semilunaris (semilunar fold) is present and occurs in acute viral or acute allergic conjunctivitis.
nasally, medial to which lies a fleshy nodule (caruncle) consist- zzMucoid discharge is typical of chronic allergic conjunctivitis
ing of modified cutaneous tissue.! and dry eye.
zzMucopurulent discharge typically occurs in chlamydial or
acute bacterial infection.
Histology zzModerately purulent discharge occurs in acute bacterial
zzThe epithelium is non-keratinizing and around five cell layers conjunctivitis.
deep (Fig. 6.1). Basal cuboidal cells evolve into flattened poly- zzSevere purulent discharge is suggestive of gonococcal
hedral cells, subsequently being shed from the surface. Mucus- infection.!
secreting goblet cells are located within the epithelium, being
most dense inferonasally and in the fornices. Conjunctival reaction
zzThe stroma (substantia propria) consists of richly vascular- zzHyperaemia that is diffuse, beefy-red and more intense away
ized loose connective tissue. The accessory lacrimal glands from the limbus is usual in bacterial infection (Fig. 6.2A). This
of Krause and Wolfring are located deep within the stroma. ‘conjunctival injection’ should be distinguished from the cili-
Secretions from the accessory lacrimal glands are essential ary injection of iridocyclitis (see Ch. 12).
components of the tear film. zz Haemorrhages may occur in viral conjunctivitis, when they are
o%en multiple, small and discrete (‘petechial’) and severe bacte-
rial conjunctivitis, when they are larger and diffuse (Fig. 6.2B).
zzChemosis (conjunctival oedema) is seen as a translucent
swelling (Fig. 6.2C), which may protrude through the eyelids.
Acute chemosis usually indicates a hypersensitivity response
(e.g. pollen), but can also occur in severe infective conjunctivi-
tis. Subacute or chronic chemosis has numerous causes:
{z Local, e.g. thyroid eye disease, chronic allergic conjunctivi-
tis, ocular or eyelid surgery, trauma.
{z Increased systemic vascular permeability, e.g. allergic con-
ditions, infections including meningitis, vasculitis.
{z Increased venous pressure, e.g. superior vena cava syn-
drome, right-sided heart failure.
{z Decreased plasma oncotic pressure, e.g. nephrotic
syndrome.
zzMembranes
{z Pseudomembranes (Fig. 6.2D) consist of coagulated exu-
date adherent to the inflamed conjunctival epithelium. They
Fig. 6.1 Histology of the conjunctiva. can be peeled away leaving the underlying epithelium intact.

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 6
CHAPTER
Conjunctiva 171

A B

C D

E F

Fig. 6.2 Signs of conjunctival inflammation. (A) Hyperaemia (conjunctival injection);

(B) subconjunctival haemorrhage in viral conjunctivitis; (C) chemosis; (D) pseudomembrane;
(E) infiltration; (F) scarring. (Courtesy of P Saine – fig. A.)

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172 Introduction

{z True membranes involve the superficial layers of the con- fornices (Fig. 6.3A). Blood vessels run around or across
junctival epithelium so that attempted removal leads to rather than within the lesions.
tearing. The distinction between a true membrane and a {z Histology shows a subepithelial lymphoid germinal cen-
pseudomembrane is rarely clinically helpful and both can tre with central immature lymphocytes and mature cells
leave scarring following resolution. peripherally (Fig. 6.3B).
{z Causes include severe adenoviral conjunctivitis, gonococ- {z Causes include viral and chlamydial conjunctivitis,
cal and some other bacterial infections (Streptococcus spp., Parinaud oculoglandular syndrome and hypersensitiv-
Corynebacterium diphtheriae), ligneous conjunctivitis and ity to topical medications. Small follicles are a normal
Stevens–Johnson syndrome. finding in childhood (folliculosis), as are follicles in the
zzInfiltration represents cellular recruitment to the site of fornices and at the margin of the upper tarsal plate in
chronic inflammation and typically accompanies a papillary adults.
response. It is recognized by loss of detail of the normal tarsal zzPapillae can develop only in the palpebral conjunctiva and in
conjunctival vessels, especially on the upper lid (Fig. 6.2E). the limbal bulbar conjunctiva where it is attached to the deeper
zzSubconjunctival cicatrization (scarring) may occur in tra- fibrous layer.
choma and other severe forms of conjunctivitis (Fig. 6.2F). {z Signs. In contrast to follicles, a vascular core is present.
Severe scarring is associated with loss of goblet cells and acces- Micropapillae form a mosaic-like pattern of elevated red
sory lacrimal glands and can lead to cicatricial entropion. dots as a result of the central vascular channel, macropa-
zzFollicles pillae (<1 mm – Fig. 6.3C) and giant papillae (>1 mm)
{z Signs. Multiple, discrete, slightly elevated lesions resem- develop with prolonged inflammation. Apical infiltrate or
bling translucent grains of rice, most prominent in the staining with fluorescein or the presence of mucus can be

A B

C D

Fig. 6.3 (A) Conjunctival follicles; (B) histology of a follicle showing two subepithelial germinal
centres with immature lymphocytes centrally and mature cells peripherally; (C) conjunctival
macropapillae; (D) histology of a papilla showing folds of hyperplastic conjunctival epithelium
with a fibrovascular core and subepithelial stromal infiltration with inflammatory cells.

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present with marked activity. Limbal papillae have a gelati-

nous appearance. BACTERIAL CONJUNCTIVITIS
{z Histology shows folds of hyperplastic conjunctival epi-
thelium with a fibrovascular core and subepithelial stro-
Acute bacterial conjunctivitis
mal infiltration with inflammatory cells (Fig. 6.3D). Late Acute bacterial conjunctivitis is a common and usually self-
changes include superficial stromal hyalinization, scarring limiting condition caused by direct contact with infected secre-
and the formation of crypts containing goblet cells. tions. The most common isolates are Streptococcus pneumoniae,
{z Causes include bacterial conjunctivitis, allergic conjuncti- Staphylococcus aureus, Haemophilus influenzae and Moraxella
vitis, chronic blepharitis, contact lens wear, superior limbic catarrhalis. A minority of severe cases are caused by the sexually
keratoconjunctivitis and floppy eyelid syndrome.! transmitted organism Neisseria gonorrhoeae, which can readily
invade the intact corneal epithelium. Meningococcal (Neisseria
Lymphadenopathy meningitidis) conjunctivitis is rare and usually affects children.
The most common cause of lymphadenopathy associated with
conjunctivitis is viral infection. It may also occur in chlamydial Diagnosis
and severe bacterial conjunctivitis (especially gonococcal) and zzSymptoms
Parinaud oculoglandular syndrome. The preauricular site is typi- {z Acute onset of redness, grittiness, burning and discharge.
cally affected. {z Involvement is usually bilateral although one eye may
become affected 1–2 days before the other (Fig. 6.4A).
TIP Viral conjunctivitis tends to occur in epidemics and {z On waking, the eyelids are frequently stuck together and
may be difficult to open.
commonly causes preauricular lymphadenopathy.
!

A B

C D

Fig. 6.4 Bacterial conjunctivitis. (A) Early infection with slight lid swelling and a sticky dis-
charge; (B) diffuse tarsal and forniceal conjunctival hyperaemia (injection); (C) mucopurulent
discharge; (D) profuse purulent discharge secondary to gonococcus.

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174 Bacterial Conjunctivitis

{z Systemic symptoms may occur in patients with severe con- {z Some practitioners, particularly in the USA, believe that
junctivitis associated with gonococcus, meningococcus, chloramphenicol should not be used for routine treatment
Chlamydia and H. influenzae. In children, the possibility because of a possible link with aplastic anaemia.
of progression to systemic involvement should always be {z Gonococcal and meningococcal conjunctivitis should be
borne in mind. treated with a quinolone, gentamicin, chloramphenicol
zzSigns are variable and depend on the severity of infection. or bacitracin 1–2 hourly as well as systemic therapy (see
{z The vision is usually normal. below).
{z Eyelid oedema and erythema may occur in severe infec- zzSystemic antibiotics are required in the following
tion, particularly gonococcal. circumstances:
{z Conjunctival injection as previously described (Fig. 6.4B {z Gonococcal infection is usually treated with a third-
and see Fig. 6.2A). generation cephalosporin such as ce%riaxone; quinolones
{z The discharge can initially be watery, mimicking viral con- and some macrolides are alternatives. It is essential to
junctivitis, but rapidly becomes mucopurulent (Fig. 6.4C). seek advice from a microbiologist and/or genitourinary
{z Hyperacute purulent discharge (Fig. 6.4D) may signify specialist.
gonococcal or meningococcal conjunctivitis. {z H. influenzae infection, particularly in children, is treated
{z Superficial corneal punctate epithelial erosions are with oral amoxicillin with clavulanic acid. There is a 25%
common. risk of developing otitis and other systemic problems.
{z Peripheral corneal ulceration may occur in gonococcal {z Meningococcal conjunctivitis, particularly in children in
and meningococcal infection and may rapidly progress to whom early systemic prophylaxis may be life-saving, as up
perforation. to 30% may develop systemic disease without treatment.
{z Lymphadenopathy is usually absent except in severe gono- The advice of paediatric and infectious disease specialists
coccal and meningococcal infection. must be sought, but if in doubt treatment with intramus-
zzInvestigations are not performed routinely but may be indi- cular benzylpenicillin, ce%riaxone or cefotaxime, or oral
cated in the following situations: ciprofloxacin should not be delayed.
{z In severe cases, binocular conjunctival swabs and scrap- {z Preseptal or orbital cellulitis (see Ch. 4).
ings should be taken for urgent Gram staining, to exclude zzTopical steroids may reduce scarring in membranous and
gonococcal and meningococcal infection (Gram-negative pseudomembranous conjunctivitis.
kidney-shaped intracellular diplococci). zzIrrigation to remove excessive discharge may be useful in
{z Culture should include enriched media such as chocolate hyperpurulent cases.
agar or Thayer–Martin for N. gonorrhoeae (see Fig. 7.8C). zzContact lens wear should be discontinued until at least
{z Polymerase chain reaction (PCR) may be required for 48 hours a%er complete resolution of symptoms. Contact
less severe cases that fail to respond to treatment, par- lenses should not be worn whilst topical antibiotic treatment
ticularly to rule out the possibility of chlamydial and viral continues.
infection. zzRisk of transmission should be reduced by hand-washing
and the avoidance of towel sharing.
TIP A hyperacute purulent ocular discharge may be a sign zzReview is unnecessary for most mild/moderate adult cases,
of gonococcal or meningococcal conjunctivitis. A specimen although patients should be cautioned to seek further advice
should be taken for microscopy, culture and sensitivity (MCS) in in the event of deterioration.
zzStatutory notification of public health authorities may be
order to prescribe an appropriate systemic antibiotic.
!
required locally for some causes.!

Treatment
About 60% resolve within 5 days without treatment.
Giant fornix syndrome
zzTopical antibiotics, usually four times daily for up to a week Giant fornix syndrome is an uncommon entity causing chronic
but sometimes more intensively, are frequently administered relapsing pseudomembranous purulent conjunctivitis. It is
to speed recovery and prevent re-infection and transmission. believed to be due to retained debris in a voluminous upper fornix
There is no evidence that any particular antibiotic is more acting as a focus for persistent bacterial colonization (usually S.
effective. Ointments and gels provide a higher concentration aureus) in an elderly patient with levator disinsertion. Large pro-
for longer periods than drops, but should be avoided during tein aggregations may be visualized in the upper fornix, though
the day because blurred vision may follow. The following anti- double eversion with a retractor may be necessary to identify
biotics are available: these. Secondary corneal vascularization and lacrimal obstruc-
{z Chloramphenicol, aminoglycosides (gentamicin, neomy- tion are common. It is frequently unilateral. Treatment involves
cin, tobramycin), quinolones (ciprofloxacin, ofloxacin, repeated sweeping of the fornix with a cotton-tipped applicator
levofloxacin, lomefloxacin, gatifloxacin, moxifloxacin, and topical and systemic antibiotics; intensive topical steroid may
besifloxacin), macrolides (erythromycin, azithromycin) be helpful. Surgical reconstruction of the fornix may be necessary
polymyxin B, fusidic acid and bacitracin. in recalcitrant cases.!

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Conjunctiva 175

zzSigns
Adult chlamydial conjunctivitis {z Watery or mucopurulent discharge.
Pathogenesis {z Tender preauricular lymphadenopathy.

Chlamydia trachomatis (Fig. 6.5) is a species of Chlamydiae, a {z Large follicles are o%en most prominent in the inferior
phylum of bacteria that cannot replicate extracellularly and hence fornix (Fig. 6.6A) and may also involve the upper tarsal
depends on host cells. They exist in two principal forms: a robust conjunctiva (Fig. 6.6B).
infective extracellular ‘elementary body’ and a fragile intracellu- {z Superficial punctate keratitis is common.

lar replicating ‘reticular body’. Adult chlamydial (inclusion) con- {z Perilimbal subepithelial corneal infiltrates (Fig. 6.6C) may

junctivitis is an oculogenital infection usually caused by serovars appear a%er 2–3 weeks.
(serological variants) D–K of C. trachomatis and affects 5–20% of {z Chronic cases have less prominent follicles and commonly

sexually active young adults in Western countries. Transmission develop papillae.

is by autoinoculation from genital secretions, although eye-to-eye {z Mild conjunctival scarring and superior corneal pannus

spread probably accounts for about 10%. The incubation period is (Fig. 6.6D) are not uncommon.
approximately a week.! zzInvestigations. Tarsal conjunctival scrapings are obtained
using a spatula or the blunt side of a scalpel blade.
Urogenital infection {z Nucleic acid amplification tests such as PCR are likely to be
zzIn males, chlamydial infection is the most common cause of the investigation of choice in time but validation for ocular
non-gonococcal urethritis (NGU), also termed non-specific specimens is limited at present.
urethritis (NSU). It should be noted that the latter term is also {z Giemsa staining for basophilic intracytoplasmic bodies is

sometimes used to mean urethritis in which both gonococ- performed by applying scrapings onto a glass slide.
cal and chlamydial infection have been ruled out. Chlamydial {z Direct immunofluorescence detects free elementary bod-

urethritis is frequently asymptomatic in men. C. trachomatis ies with approximately 90% sensitivity and specificity.
may also cause epididymitis and can act as a trigger for Reiter {z Enzyme immunoassay for direct antigen detection is also

syndrome. useful.
zzIn females, chlamydial urethritis typically causes dysuria and {z McCoy cell culture is highly specific.

discharge. It may progress to pelvic inflammatory disease {z Swabs taken for bacterial culture and serology may be

(PID), carrying a risk of infertility. Approximately 5–10% of helpful in selected cases.!

women with PID develop perihepatitis (Fitz-Hugh–Curtis
syndrome).! Treatment
Empirical treatment may be given if the clinical picture is convinc-
Diagnosis ing pending investigation results.
zzSymptoms consist of the subacute onset of unilateral or bilat- zzReferral to a genitourinary specialist is mandatory in con-
eral redness, watering and discharge. Untreated, the conjuncti- firmed cases, particularly for the exclusion of other sexu-
vitis becomes chronic and though self-limiting may persist for ally transmitted infections, contact tracing and pregnancy
several months. It is important to enquire about sexual expo- testing.
sure if chlamydial conjunctivitis is suspected. zzSystemic therapy involves one of the following:
{z Azithromycin 1 g repeated a%er 1 week is generally the
treatment of choice, although a second or a third course is
C. trachomatis required in up to 30% of cases. Some guidelines advocate
only a single 1 g dose.
{z Doxycycline 100 mg twice daily for 10 days (tetracyclines
are relatively contraindicated in pregnancy/breastfeeding
TRIC (trachoma-inclusion LGV (lymphogranuloma and in children under 12 years of age).
conjunctivitis) agents venereum) agents
{z Erythromycin, amoxicillin and ciprofloxacin are
alternatives.
zzTopical antibiotics such as erythromycin or tetracycline oint-
ment are sometimes used to achieve rapid relief of ocular
L1,L2,L3 (immunotypes)
symptoms, but are insufficient alone.
zzReduction of transmission risk involves abstinence from
sexual contact until completion of treatment (1 week a%er
azithromycin), together with other precautions as for any
infectious conjunctivitis.
A,B,Ba,C serovars D,E,F,G,H,I,J,K serovars zzRe-testing for persistent infection should take place 6–12
– cause trachoma – cause genital, other systemic, weeks a%er treatment.
and ocular disease zzIt is important to be aware that symptoms commonly take
Fig. 6.5 Classification of Chlamydia trachomatis. weeks to settle and that follicles and corneal infiltrates can take

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176 Bacterial Conjunctivitis

A B

C D

Fig. 6.6 Adult chlamydial conjunctivitis. (A) Large forniceal follicles; (B) superior tarsal follicles;
(C) peripheral corneal infiltrates (arrows); (D) superior pannus.

months to resolve due to a prolonged hypersensitivity response infection in sensitizing the individual and as a consequence is
to chlamydial antigen. not helpful. The family childcare group is the most important re-
infection reservoir and young children are particularly vulnerable.
TIP In a patient with adult chlamydial conjunctivitis, other The fly is an important vector, but there may be direct transmission
from eye or nasal discharge. Trachoma is associated principally
sexually transmitted infections should be excluded.
! with infection by serovars A, B, Ba and C of Chlamydia tracho-
matis, but the serovars D–K conventionally associated with adult
Trachoma inclusion conjunctivitis and other species of the Chlamydiaceae
family such as Chlamydophila psittaci and Chlamydophila pneu-
Pathogenesis moniae have also been implicated.!
Trachoma is the world’s leading cause of preventable irreversible
blindness. It is related to poverty, overcrowding and poor hygiene, Diagnosis
the morbidity being a consequence of the establishment of re- Features of trachoma are divided into an ‘active’ inflammatory
infection cycles within communities. Whereas an isolated episode stage and a ‘cicatricial’ chronic stage, with considerable overlap.
of trachomatous conjunctivitis may be relatively innocuous, recur- A World Health Organization (WHO) grading system is in use
rent infection elicits a chronic immune response consisting of a (Table 6.1).
cell-mediated delayed hypersensitivity (Type IV) reaction to the zzActive trachoma is most common in pre-school children and
intermittent presence of chlamydial antigen and can lead to loss of is characterized by the following:
sight. Prior contact with the organism confers short-term partial {z Mixed follicular/papillary conjunctivitis (Fig. 6.7A) associ-
immunity but also leads to a heightened inflammatory reaction ated with a mucopurulent discharge. In children under the
upon re-infection. Vaccination has an effect similar to primary age of 2 years the papillary component may predominate.

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Conjunctiva 177

A B

C D

E F

Fig. 6.7 Trachoma. (A) Typical white subtarsal follicles; (B) marked pannus; (C) stellate conjunc-
tival scarring (arrow); (D) Arlt line and conjunctival follicles; (E) Herbert pit (arrow); (F) cicatricial
entropion. (Courtesy of C Barry – figs A, B, D–F.)

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178 Bacterial Conjunctivitis

zzSurgery is aimed at relieving entropion and trichiasis and

Table 6.1 WHO Grading of Trachoma
maintaining complete lid closure, principally with bilamellar
TF = trachomatous inflammation (follicular): five or more tarsal rotation.
follicles (>0.5"mm) on the superior tarsal plate
TI = trachomatous inflammation (intense): diffuse
involvement of the tarsal conjunctiva, obscuring 50% TIP Systemic tetracyclines should not be used in pregnancy
or more of the normal deep tarsal vessels; papillae are and in children under 12 years of age because of the risk of
present tooth staining.
!
TS = trachomatous conjunctival scarring: easily visible
fibrous white tarsal bands
TT = trachomatous trichiasis: at least one lash touching
the globe
Neonatal conjunctivitis
CO = corneal opacity sufficient to blur details of at least Neonatal conjunctivitis (ophthalmia neonatorum) is defined as con-
part of the pupillary margin junctival inflammation developing within the first month of life. It
is the most common infection of any kind in neonates, occurring in
up to 10%. It is identified as a specific entity distinct from conjunc-
{z Superior epithelial keratitis and pannus formation (Fig. tivitis in older infants because of its potentially serious nature (both
6.7B). ocular and systemic complications) and because it is o%en the result
zzCicatricial trachoma is prevalent in middle age. of infection transmitted from mother to infant during delivery.
{z Linear or stellate (Fig. 6.7C) conjunctival scars in mild
cases, or broad confluent scars (Arlt line – Fig. 6.7D) in Causes
severe disease. zzOrganisms acquired during vaginal delivery: C. trachomatis,
{z Although the entire conjunctiva is involved, the effects are N. gonorrhoeae (now rare in wealthier countries, but
most prominent on the upper tarsal plate. previously responsible for 25% of childhood blindness) and
{z Superior limbal follicles may resolve to leave a row of shal- herpes simplex virus (typically HSV-2). With all of these, con-
low depressions (Herbert pits – Fig. 6.7E). junctivitis is not uncommonly associated with severe ocular
{z Trichiasis, distichiasis, corneal vascularization and cicatri- or systemic complications. C. trachomatis is the most -com-
cial entropion (Fig. 6.7F). mon cause in cases involving moderate–severe conjunctival
{z Severe corneal opacification. inflammation.
{z Dry eye caused by destruction of goblet cells and the duct- zzStaphylococci are usually responsible for mild conjunctivi-
ules of the lacrimal gland. tis. Other bacterial causes include streptococci, H. influenzae
zzInvestigations are rarely used in the affected areas as in most and various Gram-negative organisms.
cases the diagnosis can be made based on the clinical features. zzTopical preparations used as prophylaxis against infection
Various field techniques (e.g. dipstick enzyme immunoassay) (see below) may themselves cause conjunctival irritation
are available and other investigations are similar to those for (chemical conjunctivitis).
adult inclusion conjunctivitis.! zzCongenital nasolacrimal obstruction. Despite poor neo-
natal tear production, a persistently mildly watery eye with
Management recurrent mild bacterial conjunctivitis may be secondary to a
The SAFE strategy for trachoma management supported by the blocked tear duct.!
WHO and other agencies encompasses Surgery for trichiasis,
Antibiotics for active disease, Facial hygiene and Environmental Diagnosis
improvement. zzTiming of onset
zzAntibiotics should be administered to those affected and to all {z Chemical irritation: first few days.
family members. A single antibiotic course is not always effec- {z Gonococcal: first week.
tive in eliminating infection in an individual and communities {z Staphylococci and other bacteria: end of the first week.
may need to receive annual treatment to suppress infection. {z Herpes simplex virus (HSV): 1–2 weeks.
{z A single dose of azithromycin (20 mg/kg up to 1 g) is the {z Chlamydia: 1–3 weeks.
treatment of choice. zzHistory
{z Erythromycin 500 mg twice daily for 14 days or doxycy- {z Instillation of a prophylactic chemical preparation.
cline 100 mg twice daily for 10 days (tetracyclines are rela- {z Parental symptoms of sexually transmitted infection (STI).
tively contraindicated in pregnancy/breastfeeding and in {z Recent conjunctivitis in close contacts.
children under 12). {z Features of systemic illness in the child: pneumonitis, rhi-
{z Topical 1% tetracycline ointment is less effective than oral nitis and otitis in chlamydial infection, skin vesicles and
treatment. features of encephalitis in HSV. Disseminated gonococcal
zzFacial cleanliness is a critical preventative measure. infection is relatively rare.
zzEnvironmental improvement, such as access to adequate {z Prior persistent watering without inflammation may indi-
water and sanitation, as well as control of flies, is important. cate a nasolacrimal duct that is not yet open.

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Conjunctiva 179

zzSigns bacterial culture and chocolate agar or Thayer–Martin (for

{z A mildly sticky eye may occur in staphylococcal infection, N. gonorrhoeae).
or with delayed nasolacrimal duct canalization (mucopu- {z Epithelial cells infected with HSV may show eosinophilic
rulent reflux on pressure over the lacrimal sac). intranuclear inclusions on Papanicolaou smear.
{z Discharge is characteristically watery in chemical and HSV {z Conjunctival scrapings or fluid from skin vesicles can be
infection, mucopurulent in chlamydial infection, purulent sent for viral culture for HSV.
(Fig. 6.8) in bacterial infection and hyperpurulent in gono- {z Specimens should be taken prior to fluorescein instillation
coccal conjunctivitis. if immunofluorescent testing is planned.!
{z Severe eyelid oedema occurs in gonococcal infection. It may
be difficult to distinguish severe conjunctivitis from prese- Treatment
ptal or orbital infection. Signs of dacryocystitis should be zzProphylaxis is routinely performed but there is no standard
excluded. protocol.
{z Eyelid and periocular vesicles may occur in HSV infection {z A single instillation of povidone-iodine 2.5% solution is
and can critically aid early diagnosis and treatment. effective against common pathogens.
{z Corneal examination is mandatory and is particularly {z Erythromycin 0.5% or tetracycline 1% ointment.
important if gonococcal infection is suspected, as ulcer- {z Silver nitrate 1% solution agglutinates gonococci and is
ation with rapid progression is common. Use of a pen torch, still utilized in areas where gonococcal infection is com-
insertion of an eyelid speculum and fluorescein drops may mon. It should be administered in conjunction with a sin-
be helpful. The latter may facilitate identification of a den- gle intramuscular dose of benzylpenicillin when maternal
dritic or geographic epithelial lesion that may be present in infection is present.
HSV infection (in contrast to the punctate epitheliopathy zzChemical conjunctivitis does not require treatment apart
seen in older children with primary herpetic conjunctivitis). from artificial tears.
{z Pseudomembranes are not uncommon in chlamydial zzMild conjunctivitis. A mildly sticky eye is extremely com-
conjunctivitis. mon in neonates. Investigation is o%en unnecessary and a
{z Congenital glaucoma may masquerade as neonatal con- low-intensity regimen with a broad-spectrum topical antibi-
junctivitis and should always be considered, particularly in otic such as chloramphenicol, erythromycin or fusidic acid
monocular cases. ointment is adequate in most cases. Further investigation and
zzInvestigations are tailored to the clinical picture: treatment can be instituted if the condition fails to settle.
{z The results of any parental prenatal testing for STI should zzModerate–severe cases should be investigated as above.
be obtained. Microscopy with Gram staining alone is highly sensitive and
{z Conjunctival scrapings are taken for nucleic acid amplifi- will o%en provide a working diagnosis.
cation (PCR), particularly for Chlamydia and HSV. {z If the diagnosis is uncertain but chlamydial infection is a rea-
{z Separate conjunctival scrapings are applied to a glass slide sonable possibility, oral erythromycin can be commenced
for Gram and Giemsa staining. Multinucleated giant cells on an empirical basis a%er samples have been collected.
may be present on Gram stain in HSV infection. {z If bacteria are evident on Gram stain, a broad-spectrum
{z Conjunctival swabs are taken with a calcium alginate topical antibiotic (e.g. chloramphenicol, erythromycin or
swab or a sterile cotton-tipped applicator, for standard bacitracin for Gram-positive organisms, neomycin, oflox-
acin or gentamicin for Gram-negatives) should be used
until sensitivities are available. Additional systemic treat-
ment should be considered in more severe cases.
zzSevere conjunctivitis, or when systemic illness is suspected,
requires hospital admission. Samples should be taken for a
range of investigations, including urgent microscopy and a
broad-spectrum topical antibiotic, such as erythromycin, com-
menced. The ocular risk is usually most acute from gonococcal
infection, so empirical topical treatment should cover this and
in most cases, consideration given to systemic treatment such
as parenteral ce%riaxone.
zzChlamydial infection is treated with oral erythromycin for
2 weeks. A longer or supplementary course may be needed.
Erythromycin or tetracycline ointment can be used in addi-
tion, but is probably unnecessary.
zzGonococcal conjunctivitis is treated systemically with a third-
generation cephalosporin and o%en with supplementary topi-
Fig. 6.8 Eyelid oedema and purulent discharge in neonatal cal treatment. Co-treatment for Chlamydia is prudent. Saline
conjunctivitis. irrigation to remove excessive discharge should be considered.

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180 Viral Conjunctivitis

zz Herpes simplex infection should always be regarded as a zzAcute haemorrhagic conjunctivitis usually occurs in tropical
systemic condition and is treated with high-dose intravenous areas. It is typically caused by enterovirus and coxsackievirus,
aciclovir under paediatric specialist care. Early diagnosis and though other microorganisms may present similarly. It has
treatment of encephalitis (PCR of cerebrospinal fluid (CSF) is a rapid onset and resolves within 1–2 weeks. Conjunctival
positive in 95%) may be life-saving or prevent serious neurolog- haemorrhage is generally marked.
ical disability. Topical aciclovir may be considered in addition. zzChronic/relapsing adenoviral conjunctivitis giving
zzMicrobiological advice should be sought in severe cases, espe- a chronic non-specific follicular/papillary clinical pic-
cially regarding local antibiotic sensitivities. ture can persist over years, but is rare and eventually
zzPaediatric specialist involvement is mandatory when systemic self-limiting.
disease may be present. zzHerpes simplex virus (HSV) can cause a follicular conjuncti-
zzGenitourinary referral for the mother and her sexual contacts vitis, particularly in primary infection. This is usually unilat-
is important when an STI is diagnosed. The neonate should be eral and there are o%en associated skin vesicles.
screened for other STIs. zzSystemic viral infections such as those common in child-
zz Notification of a case of neonatal conjunctivitis to the local pub- hood, e.g. varicella, measles and mumps, can feature an associ-
lic health authority is a statutory requirement in many countries.! ated follicular conjunctivitis. Varicella-zoster virus secondary
infection commonly causes conjunctivitis as part of ophthal-
mic shingles. Conjunctivitis secondary to HIV infection is
VIRAL CONJUNCTIVITIS recognized.
zzMolluscum contagiosum is a skin infection caused by a
Introduction human specific double-stranded DNA poxvirus that typi-
Viral conjunctivitis is a common external ocular infection, ade- cally affects otherwise healthy children, with a peak incidence
novirus (a non-enveloped double-stranded DNA virus) being between the ages of 2 and 4 years. Transmission is by contact,
the most frequent (90%) causative agent. It may be sporadic, or with subsequent autoinoculation. A chronic follicular con-
occur in epidemics in environments such as workplaces (including junctivitis can be associated and is due to skin lesion shedding
hospitals), schools and swimming pools. The spread of this highly of viral particles. Chronic unilateral ocular irritation and mild
contagious infection is facilitated by the ability of viral particles to discharge is typical. The eyelash line should be examined care-
survive on dry surfaces for weeks and by the fact that viral shed- fully in patients with chronic conjunctivitis so as not to over-
ding may occur for many days before clinical features are appar- look a molluscum lesion.
ent. Transmission is generally by contact with respiratory or ocular zzSARS-CoV-2 virus, responsible for the COVID-19 pan-
secretions, including via fomites such as contaminated towels. demic, can lead to multi-organ involvement, including the
eye. Viral RNA can be isolated in the tears of up to 25%
TIP Viral conjunctivitis is highly contagious and great care of patients with laboratory-proven moderate or severe
COVID-19 infection. Conjunctivitis is the most com-
should be taken to prevent transmission of the disease.
!
monly reported ocular manifestation and results in hyper-
aemia, chemosis and epiphora. The infectious potential and
Presentation risk of transmission from tears or ocular discharge is not
The spectrum of viral conjunctivitis varies from mild subclinical known. Other ocular consequences of the virus include reti-
disease to severe inflammation with significant morbidity. There nal microangiopathy (common) and optic neuritis (rare).
will o%en be a history of a close contact with acute conjunctivitis. COVID-19 vaccination generally does not increase the risk
zzNon-specific acute follicular conjunctivitis is the most com- of ocular adverse events, except in some patients with pre-
mon clinical form of viral conjunctivitis and is typically due to existing uveitis where reactivation of ocular inflammation
adenoviral infection by a range of serological variants. Unilat- may follow.!
eral watering, redness, irritation and/or itching and mild pho-
tophobia occur, the contralateral eye generally being affected Signs
1–2 days later, o%en less severely. The condition is usually zzEyelid oedema ranges from negligible to severe.
milder than the other clinical forms of adenoviral conjuncti- zzLymphadenopathy is common: tender preauricular.
vitis. Patients may have accompanying (usually mild) systemic zzConjunctival hyperaemia and follicles are typically promi-
symptoms, such as a sore throat or common cold. nent. Papillae may also be seen, particularly in the superior
zzPharyngoconjunctival fever (PCF) is caused mainly by tarsal conjunctiva.
adenovirus serovars 3, 4 and 7. It is spread by droplets within zzSevere inflammation may be associated with conjunctival
families with upper respiratory tract infection. Keratitis devel- haemorrhages (Fig. 6.9A) (usually petechial in adenoviral
ops in about 30% of cases but is seldom severe. Symptoms are infection), chemosis, membranes (rare) and pseudomembranes
essentially as above, though sore throat is typically prominent. (Fig. 6.9B), sometimes with conjunctival scarring a%er resolu-
zz Epidemic keratoconjunctivitis (EKC) is caused mainly by tion (Fig. 6.9C).
adenovirus serovars 8, 19 and 37 and is the most severe ocular zzKeratitis (adenoviral):
adenoviral infection. Keratitis, which may be marked, develops {z Epithelial microcysts (non-staining) are common at an
in about 80%. Photophobia may be correspondingly prominent. early stage.

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Conjunctiva 181

A B

C D

Fig. 6.9 Adenoviral keratoconjunctivitis. (A) Follicular conjunctivitis with sub-conjunctival

haemorrhage; (B) pseudomembrane; (C) residual scarring; (D) subepithelial infiltrates.
(Courtesy of S Tuft – figs B and C.)

{z Punctate epithelial keratitis (staining) may occur, usu- zzGiemsa stain shows predominantly mononuclear cells in
ally within 7–10 days of the onset of symptoms, typically adenoviral conjunctivitis and multinucleated giant cells in
resolving within 2 weeks. herpetic infection.
{z Focal white subepithelial/anterior stromal infiltrates (Fig. zzNucleic acid amplification techniques such as PCR are sensi-
6.9D) o%en develop beneath the fading epithelial lesions, tive and specific for viral DNA.
probably as an immune response to the virus. They may zzViral culture with isolation is the reference standard but is
persist or recur over months or years. expensive and fairly slow (days to weeks) and requires specific
{z Small pseudodendritic epithelial formations sometimes transport media. Sensitivity is variable but specificity is around
occur. 100%.
zzAnterior uveitis is sometimes present, but is mild. zzA ‘point-of-care’ immunochromatography test takes 10 min-
zzMolluscum contagiosum utes to detect adenoviral antigen in tears; sensitivity and speci-
{z A pale, waxy, umbilicated nodule on the lid margin (Fig. ficity are excellent.
6.10A) associated with follicular conjunctivitis (Fig. 6.10B) zzSerology for IgM or rising IgG antibody titres to adenovirus
and mild watery and mucoid discharge. has limitations and is rarely used.
{z Bulbar nodules and confluent cutaneous lesions may occur zzInvestigation for other causes such as chlamydial infection
in immunocompromised patients.! may be indicated in non-resolving cases.!

Investigation Treatment
Investigation is generally unnecessary, but should be considered if The treatment of herpetic ocular surface disease is addressed in
the diagnosis is in doubt or there is failure of resolution. Chapter 7.

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182 Allergic Conjunctivitis

A B

Fig. 6.10 (A) Molluscum eyelid lesion; (B) follicular conjunctivitis associated with a molluscum
lesion (arrow).

zzSpontaneous resolution of adenoviral infection usually {z Removal of symptomatic pseudomembranes or

occurs within 2–3 weeks, so specific treatment is typically membranes.
unnecessary. No antiviral agent with clinically useful activity {z Topical antibiotics if secondary bacterial infection is
against adenovirus has yet been produced. suspected.
zzReduction of transmission risk by meticulous hand hygiene, {z Povidone-iodine is very effective against free (although
avoiding eye rubbing and towel sharing. There should be scru- less so against intracellular) adenovirus and has been pro-
pulous disinfection of instruments and clinical surfaces a%er posed as a means of decreasing infectivity.!
examination of an infected patient (e.g. sodium hypochlorite,
povidone-iodine).
zzMolluscum contagiosum. Although lesions are self-limiting
ALLERGIC CONJUNCTIVITIS
in immunocompetent patients, removal is o%en necessary Atopy is a genetically determined predisposition to hypersensitiv-
to address secondary conjunctivitis or for cosmetic reasons. ity reactions upon exposure to specific environmental antigens.
Expression is facilitated by making a small nick in the skin at Clinical manifestations include the various forms of allergic con-
the margin of the lesion with the tip of a needle. junctivitis, as well as hay fever (seasonal allergic rhinitis), asthma
zzTopical steroids such as prednisolone 0.5% four times daily may and eczema. Allergic conjunctivitis is a type I (immediate) hyper-
be required for severe membranous or pseudomembranous sensitivity reaction, mediated by degranulation of mast cells in
adenoviral conjunctivitis. Symptomatic keratitis may require response to the action of IgE. There is evidence of an element of
weak topical steroids but these should be used with caution as type IV hypersensitivity in at least some forms.
they do not speed resolution but only suppress inflammation
and lesions commonly recur a%er premature discontinuation.
Steroids may enhance viral replication and extend the period
Acute allergic conjunctivitis
during which the patient remains infectious. Intraocular pres- Acute allergic conjunctivitis is a common condition caused by an
sure should be monitored if treatment is prolonged. acute conjunctival reaction to an environmental allergen, usually
zzOther measures pollen. It is typically seen in younger children a%er playing outside in
{z Discontinuation of contact lens wear until resolution of spring or summer. Acute itching and watering are common, but the
symptoms. hallmark is chemosis (Fig. 6.11), which is frequently dramatic and
{z Artificial tears four times daily may be useful for symp- worrying to the child and parents. Treatment is not usually required
tomatic relief. Preservative-free preparations may give and the conjunctival swelling settles within hours as the acute increase
superior comfort and if supplied in single-dose units may in vascular permeability resolves. Cool compresses can be used and a
reduce transmission risk. single drop of adrenaline 0.1% may reduce extreme chemosis.!
{z Cold (or warm) compresses for symptomatic relief.
{z Topical antihistamines and vasoconstrictors may improve Seasonal and perennial allergic
symptoms, particularly itching.
{z The place of non-steroidal anti-inflammatory drops is not
conjunctivitis
well established, but may be effective in some circum- These common subacute conditions are distinguished from each
stances such as steroid weaning. They are not thought to other by the timing of exacerbations, thought to relate principally
promote viral replication. to differing stimulating allergens in each.

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Conjunctiva 183

A B

Fig. 6.11 (A) Moderate and (B) severe chemosis in acute allergic conjunctivitis.

zzSeasonal allergic conjunctivitis (‘hay fever eyes’), worse dur- zzTopical steroids are effective but rarely necessary.
ing the spring and summer, is the more common. The most zzOral antihistamines may be indicated for severe symptoms.
frequent allergens are tree and grass pollens, although the spe- Some, such as diphenhydramine, cause significant drowsiness
cific allergen varies with geographic location. and may be useful in aiding sleep. Others, such as loratadine,
zz Perennial allergic conjunctivitis causes symptoms throughout have a far less marked sedative action.!
the year, generally worse in the autumn when exposure to house
dust mites, animal dander and fungal allergens is greatest. It is
less common and tends to be milder than the seasonal form. Vernal keratoconjunctivitis
Diagnosis Pathogenesis
zzSymptoms. Transient acute or subacute attacks of redness, Vernal keratoconjunctivitis (VKC) is a recurrent bilateral disorder
watering and itching, associated with sneezing and nasal in which both IgE- and cell-mediated immune mechanisms play
discharge. important roles. It primarily affects boys and onset is generally from
zzSigns. Normal vision. Conjunctival hyperaemia with a rela- about the age of 5 years onwards. There is remission by the late teens
tively mild papillary reaction, variable chemosis and lid in 95% of cases, although many of the remainder develop atopic
oedema. keratoconjunctivitis. VKC is rare in temperate regions, but relatively
zzInvestigations are generally not performed although con- common in warm dry climates such as the Mediterranean, sub-
junctival scraping in more active cases may demonstrate the Saharan Africa and the Middle East. In temperate regions over 90%
presence of eosinophils. Skin testing for particular allergens is of patients have other atopic conditions such as asthma and eczema
rarely required.! and two-thirds have a family history of atopy. VKC o%en occurs on
a seasonal basis, with a peak incidence over late spring and summer,
Treatment although there may be mild perennial symptoms.!
zzArtificial tears for mild symptoms.
zzMast cell stabilizers (e.g. sodium cromoglicate, nedocromil Classification
sodium, lodoxamide) must be used for a few days before exert- zzPalpebral VKC primarily involves the upper tarsal conjunc-
ing maximal effect, but are suitable (except lodoxamide) for tiva. It may be associated with significant corneal disease as a
long-term use if required. result of the close apposition between the inflamed conjunc-
zzAntihistamines (e.g. emedastine, epinastine, levocabastine, tiva and the corneal epithelium.
bepotastine) can be used for symptomatic exacerbations and zzLimbal disease typically affects Black and Asian patients.
are as effective as mast cell stabilizers. zzMixed VKC has features of both palpebral and limbal disease.!
zzDual action antihistamine and mast cell stabilizers (e.g.
azelastine, ketotifen, olopatadine) act rapidly and are o%en Diagnosis
very effective for exacerbations. The diagnosis is clinical and investigations are usually not required.
zzCombined preparation of an antihistamine and a vasocon- Eosinophils may be abundant in conjunctival scrapings.
strictor (e.g. antazoline with xylometazoline). zzSymptoms consist of intense itching, which may be associated
zzNon-steroidal anti-inflammatory preparations (e.g. diclof- with lacrimation, photophobia, a foreign body sensation, burn-
enac) can provide symptomatic relief but are rarely used. ing and thick mucoid discharge. Increased blinking is common.

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184 Allergic Conjunctivitis

zzPalpebral disease following a long history of atopic dermatitis (eczema). Asthma is

{z Early-mild disease is characterized by conjunctival hyper- also extremely common in these patients. About 5% have suffered
aemia and diffuse velvety papillary hypertrophy on the from childhood VKC. There is little or no gender preponderance.
superior tarsal plate. AKC tends to be chronic and unremitting, with a relatively low
{z Macropapillae (<1 mm) have a flat-topped polygonal expectation of eventual resolution and is associated with signifi-
appearance reminiscent of cobblestones. Focal (Fig. 6.12A) cant visual morbidity. Whereas VKC is more frequently seasonal
or diffuse (Fig. 6.12B) whitish inflammatory infiltrates and generally worse in the spring, AKC tends to be perennial and
may be seen in intense disease. is o%en worse in the winter. Patients are sensitive to a wide range
{z Progression to giant papillae (>1 mm) can occur, as adja- of airborne environmental allergens.!
cent smaller lesions amalgamate when dividing septa rup-
ture (Fig. 6.12C). Diagnosis
{z Mucus deposition between giant papillae (Fig. 6.12D). The distinction between AKC and VKC can be made clinically. Eosino-
{z Decreased disease activity is characterized by milder con- phils tend to be less common in conjunctival scrapings than with VKC.
junctival injection and decreased mucus production. zzSymptoms are similar to those of VKC, but are frequently
zzLimbal disease more severe and unremitting.
{z Gelatinous limbal conjunctival papillae that may be associ- zzEyelids
ated with transient apically located white cellular collec- {z Skin changes (Fig. 6.14A) are more prominent than in
tions (Horner–Trantas dots – Fig. 6.12E and F). VKC and are typically eczematoid: erythema, dryness,
{z In tropical regions, limbal disease may be severe (Fig. 6.12 scaling and thickening, sometimes with disruption to epi-
G and H). dermal integrity such as fissuring and scratches (excoria-
zzKeratopathy is more frequent in palpebral disease and may tion), the latter due to intense itching.
take the following forms: {z Associated chronic staphylococcal blepharitis and mada-
{z Superior punctate epithelial erosions associated with layers rosis are common.
of mucus on the superior cornea (Fig. 6.13A). {z There may be keratinization of the lid margin.
{z Epithelial macroerosions caused by a combination of epi- {z Hertoghe sign: absence of the lateral portion of the
thelial toxicity from inflammatory mediators and a direct eyebrows.
mechanical effect from papillae. {z Dennie–Morgan folds: lid skin folds caused by persistent
{z Plaques and ‘shield’ ulcers (Fig. 6.13B and C) may develop rubbing.
in palpebral or mixed disease when the exposed Bow- {z Tightening of the facial skin may cause lower lid ectropion
man membrane becomes coated with mucus and calcium and epiphora.
phosphate, leading to inadequate wetting and delayed re- {z Ptosis is not uncommon.
epithelialization. This development is serious and warrants zzConjunctival involvement is preferentially inferior palpebral,
urgent attention to prevent secondary bacterial infection. whereas in VKC it is worse superiorly.
{z Subepithelial scars that are typically grey and oval and may {z Discharge is generally more watery than the stringy
affect vision. mucoid discharge in VKC.
{z Pseudogerontoxon can develop in recurrent limbal dis- {z Hyperaemia; chemosis is not uncommon during active
ease. It is characterized by a paralimbal band of superficial inflammation.
scarring resembling arcus senilis (Fig. 6.13D), adjacent to {z Papillae are initially smaller than in VKC although larger
a previously inflamed segment of the limbus. lesions may develop later.
{z Vascularization does not tend to be prominent, though {z Diffuse conjunctival infiltration and scarring may give a
some peripheral superficial vessel ingrowth is common, whitish, featureless appearance (Fig. 6.14B).
especially superiorly. {z Cicatricial changes can lead to moderate symblepharon
{z Keratoconus and other forms of corneal ectasia are more formation, forniceal shortening (Fig. 6.14C) and keratini-
common in VKC and are thought to be at least partly due zation of the caruncle (Fig. 6.14D).
to persistent eye rubbing. {z Limbal involvement similar to that of limbal VKC can be
{z Herpes simplex keratitis is more common than average, seen, including Horner–Trantas dots.
though less so than in atopic keratoconjunctivitis. It can be zzKeratopathy
aggressive and is occasionally bilateral. {z Punctate epithelial erosions over the inferior third of the
zzEyelid disease is usually mild, in contrast to atopic cornea are common and can be marked.
keratoconjunctivitis.! {z Peripheral vascularization and stromal scarring are more
common than in VKC (Fig. 6.14E).
Atopic keratoconjunctivitis {z Persistent epithelial defects (Fig. 6.14F), sometimes with
associated focal thinning, can occasionally progress to per-
Pathogenesis foration with descemetocoele (US spelling – descemeto-
Atopic keratoconjunctivitis (AKC) is a rare bilateral disease that cele) formation.
typically develops in adulthood (peak incidence 30–50 years) {z Plaque formation may occur (see Fig. 6.13B and C).

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Conjunctiva 185

A B

C D

E F

G H

Fig. 6.12 Vernal disease. (A) Palpebral surface showing macropapillae with focal inflammatory
infiltrates (arrow); (B) macropapillae with diffuse infiltrate; (C) giant papillae; (D) intense dis-
ease with mucus; (E) sparse limbal papillae; (F) limbal papillae with Horner–Trantas dots; (G)
extensive limbal papillae; (H) severe limbal disease. (Courtesy of S Tuft – fig. F.)

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186 Allergic Conjunctivitis

A B

C D

Fig. 6.13 Keratopathy in vernal disease. (A) Superior punctate erosions and mucus stained with
rose Bengal; (B) early plaque in a corneal graft (arrow showing edge of graft); (C) plaque and
shield ulcer; (D) pseudogerontoxon (arrow) and limbal papillae. (Courtesy of S Tuft – fig. D.)

{z Predispositionto secondary bacterial and fungal infection General measures

and to aggressive herpes simplex keratitis. zzAllergen avoidance, if possible. Allergen (e.g. patch) testing is
{z Keratoconus is common (about 15%) and as with VKC, sometimes useful, but o%en gives non-specific results.
may be secondary to chronic ocular rubbing. zzCool compresses may be helpful.
zzCataract zzLid hygiene should be used for associated staphylococcal
{z Presenile shield-like anterior or posterior subcapsular cat- blepharitis. Moisturizing cream such as E45 can be applied to
aracts are common and may be exacerbated by long-term dry, fissured skin.
steroid therapy. zzBandage contact lens wear to aid healing of persistent epithe-
{z Because of the high lid margin carriage of S. aureus, cata- lial defects.!
ract surgery carries an increased risk of endophthalmitis.
{z Retinal detachment is more common than in the general Local treatment
population and is a particular risk following cataract surgery.! zzMast cell stabilizers (e.g. sodium cromoglicate, nedocromil
sodium, lodoxamide) reduce the frequency of acute exacerba-
tions and the need for steroids and so form the basis of many
Treatment of VKC and AKC regimens, but are seldom effective in isolation. Several days to
The management of VKC does not differ substantially from that of weeks of treatment are needed for a reasonable response and
AKC, although the latter is generally less responsive and requires long-term therapy may be needed (lodoxamide is not licensed
more intensive and prolonged treatment. for long-term use).

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Conjunctiva 187

A B

C D

E F

Fig. 6.14 Atopic disease. (A) Severe eyelid involvement; (B) infiltration and scarring of the
tarsal conjunctiva; (C) forniceal shortening; (D) keratinization of the caruncle; (E) intense cor-
neal vascularization; (F) persistent epithelial defect and peripheral corneal vascularization; a
penetrating keratoplasty interface can be seen. (Courtesy of S Tuft.)

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188 Allergic Conjunctivitis

zzTopical antihistamines (e.g. emedastine, epinastine, levo- The injection is given into the conjunctival surface of the
cabastine, bepotastine) when used in isolation are about as anaesthetized everted upper eyelid; 0.1 ml of betametha-
effective as mast cell stabilizers. They are suitable for acute sone sodium phosphate 4 mg/ml, dexamethasone 4 mg/ml
exacerbations but generally not for continuous long-term use or triamcinolone 40 mg/ml is given.
and courses of several preparations are licensed for use only in
courses of limited duration. A trial of several different agents TIP Topical steroids should not be used long term in a patient
may be worthwhile.
zzCombined antihistamine and vasoconstrictor (e.g. antazo-
with allergic conjunctivitis without monitoring the IOP.
!
line with xylometazoline) may offer relief in some cases.
zzCombined action antihistamine/mast cell stabilizers (e.g. Systemic treatment
azelastine, ketotifen, olopatadine) are helpful in many patients zzOral antihistamines help itching, promote sleep and reduce
and have a relatively rapid onset of action. nocturnal eye rubbing. Because other inflammatory mediators
zzNon-steroidal anti-inflammatory preparations (e.g. are involved besides histamines, effectiveness is not assured.
ketorolac, diclofenac) may improve comfort by blocking non- Some antihistamines (e.g. loratadine) cause relatively little
histamine mediators. Combining one of these with a mast cell drowsiness.
stabilizer is an effective regimen in some patients. zzAntibiotics (e.g. doxycycline 50–100 mg daily for 6 weeks,
zzTopical steroids (e.g. fluorometholone 0.1%, rimexolone 1%, azithromycin 500 mg once daily for 3 days) may be given to
prednisolone 0.5%, loteprednol etabonate 0.2% or 0.5%) are reduce blepharitis-aggravated inflammation, usually in AKC.
used for severe exacerbations of conjunctivitis and significant zzImmunosuppressive agents (e.g. steroids, ciclosporin, tacro-
keratopathy. Reducing conjunctival activity generally leads to limus, azathioprine) may be effective at relatively low doses
corneal improvement. They are usually prescribed in short but in AKC unresponsive to other measures. Short courses of
intensive (e.g. 2-hourly initially) courses, aiming for prompt high-dose steroids may be necessary to achieve rapid control
tapering. Although the risk of elevation of intraocular pres- in severe disease. Monoclonal antibodies against T cells have
sure is low, monitoring is advisable if long-term treatment is shown some promise in refractory cases.
necessary. Stronger preparations such as prednisolone 1% can zzOther treatments that may be effective in some patients
be used, but carry a higher risk of steroid-induced glaucoma. include aspirin in VKC (avoided in children and adolescents
Shield ulcers are o%en refractory to topical steroid treatment. due to Reye syndrome risk), allergen desensitization and plas-
zzSteroid ointment (e.g. hydrocortisone 0.5%) may be used to mapheresis in patients with high serum IgE levels.!
treat the eyelids in AKC, though as with eye drops, the dura-
tion of treatment should be minimized and the intraocular Surgery
pressure (IOP) monitored. zzSuperficial keratectomy may be required to remove plaques or
zzAntibiotics may be used in conjunction with steroids in severe debride shield ulcers and allow epithelialization. Medical treat-
keratopathy to prevent or treat bacterial infection. ment must be maintained until the cornea has re-epithelialized
zzAcetylcysteine is a mucolytic agent that is useful in VKC for in order to prevent recurrences. Excimer laser phototherapeu-
dissolving mucus filaments and deposits and addressing early tic keratectomy is an alternative.
plaque formation. zzSurface maintenance/restoration surgery such as amniotic
zzImmunomodulators membrane overlay gra%ing or lamellar keratoplasty, or eyelid
{z Ciclosporin (0.05–2% between two and six times daily) procedures such as botulinum toxin-induced ptosis or lateral
may be indicated if steroids are ineffective, inadequate or tarsorrhaphy, may be required for severe persistent epithelial
poorly tolerated, or as a steroid-sparing agent in patients defects or ulceration. Gluing may be appropriate for focal
with severe disease. The effects typically take some weeks to (‘punched-out’) corneal perforations.!
be exerted and relapses may occur if treatment is stopped
suddenly. Irritation and blurred vision are common.
{z Calcineurin inhibitors show increasing promise as an
Non-allergic eosinophilic conjunctivitis
alternative to steroids in the treatment of allergic eye dis- Non-allergic eosinophilic conjunctivitis (NAEC) is a recently
ease. Tacrolimus 0.03% ointment can be effective in AKC proposed chronic non-atopic condition said to occur predomi-
for severe eyelid disease. Instillation into the fornix has nantly in middle-aged women in whom dry eye is also com-
been effective in modulating conjunctival inflammation in monly present. It has been suggested that it is relatively common
refractory cases. Tacrolimus 0.1% ointment is particularly but under-diagnosed. It is thought to be of similar pathogenesis
useful when applied topically in patients with shield ulcers to non-allergic eosinophilic rhinitis. Conjunctival eosinophilia is
and corneal epitheliopathy and can be used without topical present without significant IgE levels in the serum or tear film.
steroids. Factors associated with a poor response to tacroli- Symptoms are similar to those of allergic conjunctivitis – itching,
mus include the presence of a giant papillae and palpebral redness, foreign body sensation and mild watery discharge. Treat-
abnormality. ment is with a 1–2 week course of topical steroid for exacerbations
{z Supratarsal steroid injection may be considered in followed by maintenance with topical mast cell stabilizers, non-
severe palpebral disease or for non-compliant patients. steroidal anti-inflammatory agents or antihistamines.!

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Conjunctiva 189

Fig. 6.15 Contact allergic blepharoconjunctivitis. Fig. 6.16 Ocular prosthesis causing giant papillary
conjunctivitis.

zzSigns
Contact allergic blepharoconjunctivitis {z Variable mucous discharge.
Analogous to contact dermatitis, this refers to the acute or sub- {z Substantial CL protein deposits may be present.
acute T-cell-mediated delayed hypersensitivity reaction seen most {z Excessive CL mobility due to upper lid capture.
commonly by ophthalmologists as a reaction to eye drop constitu- {z Superior tarsal hyperaemia and papillae. By definition,
ents and by optometrists as a reaction to contact lens solutions. ‘giant’ papillae are >1.0 mm in diameter, but the clinical
Mascara is a less common cause. There may be a conjunctival reac- syndrome of mechanically induced papillary conjunctivi-
tion, but signs predominantly involve the eyelid skin: erythema, tis commonly features only fine/medium papillae, particu-
thickening, induration and sometimes fissuring occur (Fig. 6.15). larly in early or mild disease.
Treatment is by discontinuation of the precipitant. A mild topical {z Focal apical ulceration and whitish scarring may develop
steroid ointment can be helpful.! on larger papillae.
{z Keratopathy is rare because of the relatively subdued secre-
tion of inflammatory cytokines.
Giant (mechanically induced) papillary {z Ptosis may occur, mainly as a result of irritative spasm and

conjunctivitis tissue laxity secondary to chronic inflammation.!

Pathogenesis Treatment
Mechanically induced papillary conjunctivitis, the severe form of Other causes of conjunctival papillae should be excluded, as well
which is known as giant papillary conjunctivitis (GPC), can occur as CL intolerance due to other causes, such as a reaction to lens
secondary to a variety of mechanical stimuli of the tarsal conjunc- cleaning solutions and dry eyes.
tiva. It is most frequently encountered with contact lens (CL) wear, zzRemoval of the stimulus
when it is termed contact lens-associated papillary conjunctivitis {z CL wear should be discontinued for several weeks and the
(CLPC). The risk is increased by the build-up of proteinaceous current lenses replaced. For mild–moderate disease, this
deposits and cellular debris on the CL surface. Ocular prostheses may be adequate for resolution, sometimes in conjunction
(Fig. 6.16), exposed sutures and scleral buckles, corneal surface with reduced wearing time. In severe CLPC a longer inter-
irregularity and filtering blebs can all be responsible. A related val without lens wear may be needed.
phenomenon is the so-called ‘mucus fishing syndrome’, when, in a {z Removal of other underlying causes, such as exposed
variety of underlying anterior segment disorders, patients develop sutures or a scleral buckle.
or exacerbate a chronic papillary reaction due to repetitive manual {z Assessment of the status and fit of an ocular prosthesis.
removal of mucus. Giant papillae can also be seen in other condi- {z Filtering bleb: partial excision, revision with non-
tions, such as VKC and AKC.! penetrating drainage surgery or glaucoma drainage device
implantation.
Diagnosis zzEnsure effective cleaning of CL or prosthesis
zzSymptoms consist of a foreign body sensation, redness, itch- {z Changing the type of CL solution, particularly discontinu-
ing, increased mucus production, blurring and loss of CL tol- ation of preservative-containing preparations.
erance. Symptoms may be worse a%er lens removal. Patients {z Switching to monthly then daily disposable CL if the con-
should be questioned about CL cleaning and maintenance. dition persists a%er renewing non-disposable lenses.

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190 Conjunctivitis in Blistering Mucocutaneous Disease

{z Rigidlenses carry a reduced risk of CLPC (5%), probably Progression of ocular disease has been divided into stages as
because they are easier to clean effectively. follows:
{z Cessation of CL wear, substituting spectacles or refractive zzFoster’s classification system
surgery, may be necessary for severe or refractory disease. {z Stage I: early stage associated with chronic conjunctivitis,
{z Regular (at least weekly) use of CL protein removal tablets. tear dysfunction and subepithelial fibrosis.
{z Prosthesis: polishing, cleaning with detergent, coating. {z Stage II: forniceal shortening, particularly inferiorly.
zzTopical {z Stage III: symblepharon formation.
{z Mast cell stabilizers should be non-preserved in patients {z Stage IV: surface keratinization and cicatricial
wearing so% contact lenses, or can be instilled when the ankyloblepharon.
lenses are not in the eye, with a delay of perhaps half an zzMondino’s classification system (based on inferior forniceal
hour a%er drop instillation prior to lens insertion. Most depth loss)
can be continued long-term if necessary. {z Stage I: up to 25%.
{z Antihistamines, non-steroidal anti-inflammatory agents {z Stage II: 25–50%.
and combined antihistamines/mast cell stabilizers may {z Stage III: 50–75%.
each be of benefit. {z Stage IV: greater than 75%.
{z Topical steroids can be used for the acute phase of resis- zzSymptoms. Insidious or relapsing–remitting non-specific
tant cases, particularly those where effective removal of the bilateral conjunctivitis; misdiagnosis (e.g. dry eye) is common.
stimulus is difficult, as in bleb-related disease.! zzConjunctiva
{z Papillary conjunctivitis, diffuse hyperaemia, oedema and
CONJUNCTIVITIS IN BLISTERING subtle fibrosis (Fig. 6.17A).
{z Fine lines of subconjunctival fibrosis and shortening of
MUCOCUTANEOUS DISEASE the inferior fornices. Symblepharon (plural: symblephara)
Mucous membrane pemphigoid formation refers to adhesion between the bulbar and pal-
pebral conjunctiva (Fig. 6.17B and C).
Introduction {z Necrosis in severe cases.
Mucous membrane pemphigoid (MMP), also known as ocular {z Flattening of the plica and keratinization of the caruncle.
cicatricial pemphigoid (OCP), comprises a group of chronic auto- {z Dry eye due to destruction of goblet cells and acces-
immune mucocutaneous blistering diseases. An unknown trigger sory lacrimal glands and occlusion of the main lacrimal
leads to a type II (cytotoxic) hypersensitivity response resulting in ductules.
antibodies binding at the basement membrane zone (BMZ), the {z Monitoring should include the measurement of forniceal
activation of complement and the recruitment of inflammatory cells, depth and noting the position of adhesions.
with localized separation of the epidermis from the dermis at the zzEyelids
BMZ and subsequent progression to scarring. Studies of HLA typ- {z Aberrant (trichiatic) lashes, chronic blepharitis and
ing have found an increased susceptibility to the disease in patients keratinization of the lid margin.
with HLA-DR4. The HLA-DQB1 allele in particular shows a strong {z Ankyloblepharon is an adhesion at the outer canthus
association with OCP and other forms of pemphigoid disease. between the upper and lower lids (Fig. 6.17D).
A wide range of epithelial tissues can be involved, including zzCornea
the skin and various mucous membranes. Particular clinical forms {z Epithelial defects (Fig. 6.18A) associated with drying and
of MMP tend to involve specific target tissues: bullous pemphigoid exposure.
(BP) shows a predilection for skin and ocular mucous membrane {z Infiltration and peripheral vascularization (Fig. 6.18B).
pemphigoid (OMMP, also known as ocular cicatricial pemphi- {z Keratinization and conjunctivalization of the corneal sur-
goid – OCP) involves the conjunctiva in the majority of cases and face (Fig. 6.18C) due to epithelial stem cell failure.
causes progressive scarring (cicatrization). The disease typically {z End-stage disease is characterized by total symblepharon
presents in old age and affects females more commonly than males and corneal opacification (Fig. 6.18D).!
by a 2:1 ratio. Other causes of cicatrizing conjunctivitis include
Stevens–Johnson syndrome, trachoma, drug-induced, trauma and Systemic features
severe or chronic conjunctivitis of many types. MMP should not zzMucosal involvement is very common and is characterized by
be confused with pemphigus, a distinct group of disorders.! subepidermal blisters, most frequently oral (Fig. 6.19A). Severe
manifestations include oesophageal and laryngeal strictures.
Ocular features zzSkin lesions are less common (25%) and present as tense blis-
zzDiagnosis is principally clinical, but biopsy of involved ters and erosions of the head and neck, groin and extremities
mucous membrane o%en shows supportive changes (linear (Fig. 6.19B).!
antibody and complement BMZ deposition).
zzMichel’s transport medium should be used when taking a Systemic treatment
specimen for direct immunofluorescence and immunoelec- Systemic treatment is the mainstay of management. Any detectable
tron microscopy. inflammatory activity should be suppressed.

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Conjunctiva 191

A B

C D

Fig. 6.17 Conjunctivitis in ocular cicatricial pemphigoid. (A) Early disease with hyperaemia and
conjunctival fibrosis (arrow); (B) symblepharon formation; (C) severe fibrosis with forniceal
shortening and symblepharon formation; (D) ankyloblepharon.

zzDapsone (diaminodiphenylsulfone) is a useful first-line treat- {z Artificialtears (preferably preservative-free) are an inte-
ment in patients with mild–moderate disease. Approximately gral part of most regimens.
70% of patients respond favourably. It is contraindicated in {z Topical steroids, ciclosporin or tacrolimus may be used as
glucose-6-phosphate dehydrogenase deficiency. Sulfasalazine an adjunct to systemic immunosuppressive treatment.
is sometimes better tolerated. {z Retinoic acid may reduce keratinization.
zzAntimetabolites (e.g. azathioprine, methotrexate, mycophen- {z Antibiotics when indicated.
olate mofetil) are alternatives for mild–moderate disease if {z Lid hygiene and low-dose oral tetracycline for blepharitis.
dapsone is contraindicated, ineffective or poorly tolerated and zzSubconjunctival mitomycin C and/or steroid injection may be
are suitable for long-term therapy. Dapsone can be used in used as a temporizing aid or if systemic immunosuppression
conjunction if necessary. Cyclophosphamide may be reserved is not possible.
for severe or refractory disease. zzContact lenses may be used with caution to protect the cornea
zzSteroids (prednisolone 1–1.5 mg/kg) are effective for rapid from aberrant lashes and from dehydration.!
disease control, but adverse effects limit long-term use. IOP
should be monitored. Reconstructive surgery
zzOther measures include intravenous immunoglobulin ther- Reconstructive surgery, preferably under systemic steroid cover,
apy and rituximab. Remission has been reported with a com- should be considered when active disease is controlled. Seem-
bination regimen.! ingly trivial surgical intervention can lead to exacerbation of
disease.
Local treatment zzAberrant eyelashes (see Ch. 2).
zzTopical zzPunctal occlusion to aid tear retention.

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192 Conjunctivitis in Blistering Mucocutaneous Disease

A B

C D

Fig. 6.18 Keratopathy in ocular cicatricial pemphigoid. (A) Epithelial defect; (B) peripheral vas-
cularization and infiltration; (C) keratinization with ankyloblepharon; (D) end-stage disease.
(Courtesy of S Tuft – figs A–C.)

A B

Fig. 6.19 Mucous membrane pemphigoid. (A) Oral blisters; (B) severe skin blistering. (Courtesy
of J David – fig. A.)

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Conjunctiva 193

zzLateral tarsorrhaphy or botulinum toxin-induced ptosis may {z Iritis

is not infrequent and panophthalmitis has been
be used to promote healing of corneal epithelial defects. reported.
zzEntropion repair: conjunctival incision is avoided if possible. zzLate signs
zzCataract surgery is commonly required. {z Conjunctival cicatrization (Fig. 6.20D) with forniceal
zzMucous membrane autogra%ing or amniotic membrane shortening and symblepharon formation.
transplantation for conjunctival resurfacing and forniceal {z Keratinization of the conjunctiva and lid margin (Fig.
restoration. 6.20E), sometimes with abrasive plaque formation.
zzLimbal stem cell transfer may be attempted for corneal {z Eyelid complications include cicatricial entropion
re-epithelialization. and ectropion, trichiasis, metaplastic lashes and
zzKeratoplasty carries a high risk of failure; lamellar gra%s may ankyloblepharon.
be effective for perforation. {z Keratopathy including scarring, vascularization and kera-
zzKeratoprosthesis may be the only option in end-stage disease.! tinization (Fig. 6.20F) as a result of the primary inflamma-
tion and/or infection, as well as cicatricial entropion and
aberrant lashes.
Stevens–Johnson syndrome/toxic {z Watery eyes due to fibrosis of the lacrimal puncta. Dry eyes
epidermal necrolysis (Lyell syndrome) may also occur as a result of fibrosis of lacrimal gland duct-
ules and conjunctival metaplasia with loss of goblet cells.!
Introduction
The terms ‘Stevens–Johnson syndrome (SJS)’ and ‘erythema multi- Systemic features
forme major’ have historically been used synonymously. However, Skin biopsy may help to establish the diagnosis but is rarely
it is now believed that erythema multiforme (without the ‘major’) necessary.
is a distinct disease, milder and recurrent, with somewhat dis- zzSymptoms. Flu-like symptoms, which can be severe, may last
similar clinical features. Toxic epidermal necrolysis (TEN – Lyell up to 14 days before the appearance of lesions. In many cases
syndrome) is a severe variant of SJS. SJS/TEN patients tend to be the patient is very ill and hospitalization is required. Symp-
young adults, though other groups may be affected. The condition toms of systemic mucosal involvement include nasal pain and
involves a cell-mediated delayed hypersensitivity reaction, usually discharge, pain on micturition, diarrhoea, cough, shortness of
related to drug exposure. A wide range of medications have been breath and pain on eating and drinking.
incriminated, including antibiotics (especially sulfonamides and zzSigns
trimethoprim), analgesics including paracetamol (acetaminophen), {z Mucosal involvement is characterized by blistering and
nevirapine (widely prescribed as a part of combination therapy for haemorrhagic crusting of the lips (Fig. 6.21A). The blisters
HIV infection), cold remedies and anticonvulsants. Infections due may also involve the tongue, oropharynx, nasal mucosa
to microorganisms such as Mycoplasma pneumoniae and HSV and occasionally the genitalia.
and some cancers have also been implicated. Because symptoms {z Small purpuric, vesicular, haemorrhagic or necrotic skin
o%en take weeks to develop, in many cases the precipitant cannot lesions involving the extremities, face and trunk (Fig.
be identified. Mortality overall is around 5% in SJS (death is com- 6.21B). These are usually transient but may be widespread.
monly due to infection), but is considerably higher in TEN. Healing usually occurs within 1–4 weeks, leaving a pig-
mented scar.
Ocular features {z Widespread sloughing of the epidermis is uncommon.
In the acute stage there are o%en practical obstacles to standard slit {z ‘Target’ lesions showing the classic three zones are now
lamp examination, as the patient may be bedridden and undergo- viewed as characteristic of erythema multiforme rather
ing barrier nursing. A portable slit lamp may be helpful. than SJS/TEN.!
zzSymptoms. Acute ocular symptoms may include redness,
mild–severe grittiness, photophobia, watering and blurring. Systemic treatment
zzAcute signs zzRemoval of the precipitant if possible, such as discontinua-
{z Haemorrhagic crusting of the lid margins (Fig. 6.20A) is tion of drugs and treatment of suspected infection.
characteristic. Skin lesions may be confluent and it is o%en zzGeneral supportive measures such as maintenance of ade-
difficult for an examiner to open the eyes without causing quate hydration, electrolyte balance and nutrition (especially
marked discomfort. protein replacement) are critical. Management in a specialist
{z Papillary conjunctivitis, which can range from mild, tran- burns unit should reduce the chance of infection when the
sient and self-limiting to severe (Fig. 6.20B). extent of skin involvement is substantial.
{z Conjunctival membranes and pseudomembranes (Fig. zzSystemic steroids remain controversial. There are reports
6.20C), severe hyperaemia, haemorrhages, blisters and of increased mortality in older papers, but later research
patchy infarction. has raised the possibility that early short-term high-dose
{z Keratopathy: a spectrum of lesions from punctate erosions intravenous treatment may improve outcomes.
to large epithelial defects, secondary bacterial keratitis and zzOther immunosuppressants including ciclosporin, azathio-
occasionally perforation. prine, cyclophosphamide and intravenous immunoglobulin

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194 Conjunctivitis in Blistering Mucocutaneous Disease

A B

C D

E F

Fig. 6.20 Ocular features of Stevens–Johnson syndrome. (A) Haemorrhagic lid crusting; (B)
acute conjunctivitis; (C) pseudomembrane; (D) conjunctival scarring; (E) keratinization with
severe lid margin involvement; (F) corneal keratinization. (Courtesy of R Bates – fig. A; S Tuft
– figs E and F.)

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