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KANSKI’S
Clinical
Ophthalmology
A Systematic Approach
John F. Salmon
MD, FRCS, FRCOphth
Consultant Ophthalmic Surgeon
Oxford Eye Hospital
Oxford
United Kingdom

KANSKI’S
Clinical
Ophthalmology
A Systematic Approach

Ninth Edition
© 2020, Elsevier Limited. All rights reserved.

First edition 1984


Second edition 1989
Third edition 1994
Fourth edition 1999
Fifth edition 2003
Sixth edition 2007
Seventh edition 2011
Eighth edition 2016
Ninth edition 2020

The right of John F. Salmon to be identified as author of this work has been asserted by him in accordance with
the Copyright, Designs and Patents Act 1988.

No part of this publication may be reproduced or transmitted in any form or by any means, electronic or
mechanical, including photocopying, recording, or any information storage and retrieval system, without
permission in writing from the publisher. Details on how to seek permission, further information about the
Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance
Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions.

This book and the individual contributions contained in it are protected under copyright by the Publisher (other
than as may be noted herein).

Notices

Practitioners and researchers must always rely on their own experience and knowledge in evaluating and
using any information, methods, compounds or experiments described herein. Because of rapid advances
in the medical sciences, in particular, independent verification of diagnoses and drug dosages should be
made. To the fullest extent of the law, no responsibility is assumed by Elsevier, authors, editors or contribu-
tors for any injury and/or damage to persons or property as a matter of products liability, negligence or
otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the
material herein.

ISBN: 978-0-7020-7711-1
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Printed in China

Last digit is the print number: 9 8 7 6 5 4 3 2 1


v

Contents

Dedication.......................................................................viii Cystic lesions.................................................................131


In Memoriam.....................................................................ix Vascular tumours...........................................................134
Preface to the Ninth Edition................................................x Lacrimal gland tumours.................................................140
Abbreviations.....................................................................xi Neural tumours..............................................................142
Lymphoma.....................................................................144
1 Examination Techniques....................... 1 Rhabdomyosarcoma......................................................147
Introduction.......................................................................2 Metastatic tumours........................................................148
Psychophysical tests..........................................................2 The anophthalmic socket...............................................150
Perimetry...........................................................................9 Craniosynostoses...........................................................153
Slit lamp biomicroscopy of the anterior segment..............20
Fundus examination.........................................................21 5 Dry Eye........................................... 155
Tonometry........................................................................27 Introduction...................................................................156
Gonioscopy......................................................................30 Sjögren syndrome..........................................................158
Central corneal thickness.................................................36 Clinical features.............................................................158
Investigation..................................................................159
2 Eyelids.............................................. 37 Treatment.......................................................................162
Introduction.....................................................................38
Non-neoplastic lesions....................................................39 6 Conjunctiva..................................... 167
Benign epidermal tumours...............................................43 Introduction...................................................................168
Benign pigmented lesions................................................44 Bacterial conjunctivitis...................................................171
Benign adnexal tumours..................................................47 Viral conjunctivitis..........................................................177
Miscellaneous benign tumours.........................................47 Allergic conjunctivitis.....................................................180
Malignant tumours...........................................................50 Conjunctivitis in blistering mucocutaneous disease........188
Disorders of the eyelashes...............................................60 Miscellaneous disorders of the conjunctiva....................194
Allergic disorders.............................................................66 Degenerations...............................................................197
Immune-related inflammation..........................................66 Subconjunctival haemorrhage........................................200
Bacterial infections..........................................................67
Viral infections.................................................................68 7 Cornea............................................ 203
Blepharitis.......................................................................70 Introduction...................................................................204
Ptosis..............................................................................74 Bacterial keratitis...........................................................209
Ectropion.........................................................................80 Fungal keratitis..............................................................216
Entropion.........................................................................85 Herpes simplex keratitis.................................................218
Miscellaneous acquired disorders....................................87 Herpes zoster ophthalmicus...........................................224
Cosmetic eyelid and periocular surgery............................91 Interstitial keratitis.........................................................229
Congenital malformations................................................93 Protozoan keratitis.........................................................232
Helminthic keratitis........................................................234
3 Lacrimal Drainage System.................. 99 Bacterial hypersensitivity-mediated
Introduction...................................................................100 corneal disease..........................................................234
Acquired obstruction......................................................105 Rosacea........................................................................236
Congenital obstruction...................................................108 Peripheral corneal ulceration/thinning...........................238
Chronic canaliculitis.......................................................109 Neurotrophic keratopathy...............................................241
Dacryocystitis................................................................110 Exposure keratopathy.....................................................242
Miscellaneous keratopathies..........................................243
4 Orbit................................................ 113 Corneal ectasia..............................................................248
Introduction...................................................................114 Corneal dystrophy..........................................................252
Thyroid eye disease........................................................118 Corneal degeneration.....................................................261
Infections.......................................................................124 Metabolic keratopathy....................................................266
Non-infective inflammatory disease...............................126 Contact lenses...............................................................268
Non-neoplastic vascular abnormalities...........................129 Congenital anomalies of the cornea and globe...............270
vi Contents

8 Corneal and Refractive Surgery........ 275 Investigation..................................................................429


Keratoplasty..................................................................276 Treatment.......................................................................432
Keratoprostheses...........................................................283 Immunomodulatory therapy for
Refractive procedures....................................................283 non-infectious uveitis.................................................433
Uveitis in spondyloarthropathies....................................435
9 Episclera and Sclera........................ 291 Fuchs uveitis syndrome..................................................437
Anatomy........................................................................292 Uveitis in juvenile idiopathic arthritis (JIA)......................439
Episcleritis.....................................................................292 Uveitis in bowel disease.................................................441
Immune-mediated scleritis.............................................293 Uveitis in renal disease..................................................442
Porphyria.......................................................................300 Intermediate uveitis.......................................................443
Infectious scleritis..........................................................300 Vogt–Koyanagi–Harada (VKH) syndrome.........................445
Scleral discolouration....................................................301 Sympathetic ophthalmitis..............................................448
Blue sclera....................................................................301 Lens-induced uveitis......................................................449
Miscellaneous conditions...............................................302 Sarcoidosis....................................................................450
10 Lens................................................ 307 Behçet disease..............................................................454
Acquired cataract..........................................................308 Parasitic uveitis..............................................................456
Management of age-related cataract..............................311 Viral uveitis....................................................................465
Congenital cataract........................................................335 Fungal uveitis.................................................................473
Ectopia lentis.................................................................338 Bacterial uveitis.............................................................477
Abnormalities of lens shape...........................................342 Miscellaneous idiopathic chorioretinopathies.................484

11 Glaucoma........................................ 345 13 Retinal Vascular Disease.................. 495


Introduction...................................................................346 Retinal circulation..........................................................496
Ocular hypertension.......................................................347 Diabetic retinopathy.......................................................496
Overview of glaucoma....................................................349 Non-diabetic retinopathy................................................513
Primary open-angle glaucoma.......................................349 Retinal venous occlusive disease...................................514
Normal-tension glaucoma..............................................367 Retinal arterial occlusive disease...................................525
Primary angle-closure glaucoma....................................370 Ocular ischaemic syndrome...........................................531
Classification of secondary glaucoma............................378 Hypertensive eye disease...............................................532
Pseudoexfoliation..........................................................379 Sickle-cell retinopathy....................................................533
Pigment dispersion syndrome and Thalassaemia retinopathy..............................................536
pigmentary glaucoma.................................................381 Retinopathy of prematurity.............................................536
Neovascular glaucoma...................................................383 Retinal artery macroaneurysm.......................................540
Inflammatory glaucoma.................................................385 Primary retinal telangiectasia.........................................543
Steroid-induced glaucoma.............................................388 Eales disease................................................................546
Lens-related glaucoma..................................................389 Radiation retinopathy.....................................................546
Traumatic glaucoma.......................................................390 Purtscher retinopathy.....................................................547
Ghost cell ‘glaucoma’.....................................................392 Valsalva retinopathy.......................................................549
Iridocorneal endothelial syndrome.................................392 Lipaemia retinalis..........................................................549
Glaucoma associated with intraocular tumours..............394 Retinopathy in blood disorders.......................................550
Glaucoma secondary to epithelial ingrowth....................394
14 Acquired Macular Disorders............. 555
Iridoschisis....................................................................395
Primary congenital glaucoma.........................................395 Introduction...................................................................556
Iridocorneal dysgenesis..................................................399 Clinical evaluation of macular disease...........................557
Glaucoma in phacomatoses...........................................403 Investigation of macular disease....................................558
Medical treatment of glaucoma......................................404 Age-related macular degeneration..................................572
Laser treatment of glaucoma.........................................407 Retinal angiomatous proliferation...................................589
Trabeculectomy..............................................................412 Polypoidal choroidal vasculopathy..................................589
Non-penetrating glaucoma surgery.................................419 Peripheral exudative haemorrhagic
chorioretinopathy.......................................................591
Minimally invasive glaucoma surgery (MIGS)..................420
Idiopathic choroidal neovascularization..........................591
Drainage shunts.............................................................421
Vitreomacular interface disorders...................................592
12 Uveitis............................................. 423 Central serous chorioretinopathy....................................598
Classification.................................................................424 Idiopathic macular telangiectasia...................................601
Clinical features.............................................................424 Cystoid macular oedema...............................................602
Contents
vii

Microcystic macular oedema..........................................604 19 Neuro-ophthalmology....................... 745


Degenerative myopia......................................................604 Neuroimaging................................................................746
Angioid streaks..............................................................607 Optic nerve....................................................................751
Choroidal folds..............................................................609 Pupils............................................................................779
Hypotony maculopathy...................................................610 Chiasm..........................................................................786
Solar retinopathy...........................................................610 Retrochiasmal pathways................................................792
Focal choroidal excavation.............................................611 Ocular motor nerves.......................................................795
Dome-shaped macula...................................................612 Supranuclear disorders of ocular motility.......................806
Low visual aids..............................................................613 Nystagmus.....................................................................809
Ocular myopathies.........................................................814
15 Hereditary Fundus Dystrophies......... 615
Miller Fisher syndrome...................................................819
Introduction...................................................................616 Neurofibromatosis.........................................................819
Investigation..................................................................616 Migraine........................................................................820
Generalized photoreceptor dystrophies...........................619 Neuralgias.....................................................................823
Macular dystrophies.......................................................631 Facial spasm.................................................................824
Generalized choroidal dystrophies..................................640 Disorders of circadian rhythm.........................................825
Hereditary vitreoretinopathies........................................641 Neuro-ophthalmology of space flight..............................825
Albinism........................................................................649
Cherry-red spot at the macula.......................................652 20 Ocular Tumours................................ 827
Benign epibulbar tumours..............................................828
16 Retinal Detachment......................... 653 Malignant and premalignant epibulbar tumours.............833
Introduction...................................................................654 Iris tumours...................................................................839
Peripheral lesions predisposing to Iris cysts........................................................................841
retinal detachment.....................................................657 Ciliary body tumours......................................................843
Posterior vitreous detachment........................................663 Tumours of the choroid..................................................846
Retinal breaks................................................................666 Neural retinal tumours...................................................860
Rhegmatogenous retinal detachment.............................668 Retinal vascular tumours................................................868
Tractional retinal detachment.........................................681 Primary intraocular lymphoma.......................................872
Exudative retinal detachment.........................................681 Tumours of the retinal pigment epithelium......................875
Pars plana vitrectomy.....................................................683 Paraneoplastic syndromes.............................................878
17 Vitreous Opacities........................... 691 21 Ophthalmic Side Effects of
Systemic Medication....................... 881
18 Strabismus...................................... 697 Eyelids...........................................................................882
Introduction...................................................................698 Cornea..........................................................................882
Amblyopia.....................................................................707 Ciliary effusion...............................................................882
Clinical evaluation..........................................................708 Lens..............................................................................883
Pseudostrabismus.........................................................726 Uveitis...........................................................................884
Heterophoria..................................................................727 Retina............................................................................884
Vergence abnormalities..................................................727 Optic nerve....................................................................889
Esotropia.......................................................................728 Visual cortex..................................................................890
Exotropia.......................................................................733
Congenital cranial dysinnervation disorders....................735
22 Trauma............................................ 891
Monocular elevation deficiency......................................737 Eyelid trauma.................................................................892
Brown syndrome............................................................737 Orbital trauma...............................................................894
Alphabet patterns..........................................................738 Trauma to the globe.......................................................898
Surgery..........................................................................739 Chemical injuries...........................................................912
Complications of strabismus surgery..............................742 Thermal burns................................................................916
Botulinum toxin chemodenervation................................743 Index.............................................................................917
Dedication
This book is dedicated to my wife Susie,
to my children Mark and Nicola,
and to my sister, Margaret, who initially awakened my interest in ophthalmology
In Memoriam
Jack J. Kanski MD, MS, FRCS, FRCOphth, Consultant Ophthalmic Surgeon (1939–2019)

Jack Kanski wrote more than 30 books, but is best known for Clinical Ophthalmology, which was first published in 1984. The
book has been studied by ophthalmology and optometry students throughout the world since that time. It has justifiably
become a classic because of its highly organized format, succinct but comprehensive text and superb clinical photographs. His
encyclopaedic knowledge of ophthalmology, meticulous attention to detail and unique ability to sort the wheat from the chaff
will be sorely missed. His legacy will live on in the minds of those who benefitted from his teaching.
x

Preface to the Ninth Edition

In presenting this new edition of Kanski’s Clinical Ophthalmology, South Africa, Tony Murray (strabismus) and Trevor Carmichael
I am reminded of a quotation from Lewis Carroll’s Alice’s Adven- (cornea), provided help with the text and images of pathology
tures in Wonderland: “What is the use of a book”, thought Alice, that are not easily found in developed countries. I received many
“without pictures or conversations?”. The ninth edition of this pictures from Jonathan Norris and Elizabeth Insull (oculoplas-
classic textbook is filled with beautiful illustrations and consider- tics), Darius Hildebrand and Manoj Parulekar (paediatrics),
able information and is intended to be a useful and comprehensive Peter Issa and Christine Kiire (medical retina), Bertil Damato
basis for general ophthalmic practice. It has been a privilege to (ocular oncology), Martin Leyland (corneal surgery), C.K. Patel
work on this remarkable book and I am grateful to Jack Kanski (vitreoretinal surgery), Patsy Terry (ultrasound) and Pieter Pre-
and the staff of Elsevier for entrusting me with the task. torius (neuroradiology). Mitch Ménage provided good pictures of
The challenge has been to cover the entire field of ophthalmol- common conditions. Aude Ambresin and Carl Herbort (Switzer-
ogy for a worldwide audience without depending on subspecialists land) supplied state-of-the-art retinal images. I have kept many
to prepare each chapter. In order to do this, I have maintained Jack of the outstanding pictures that Chris Barry and Simon Cheng
Kanski’s unique approach of presenting core clinical knowledge (Australia) provided for the eighth edition. Single examples of
in a systemic and succinct form. Brad Bowling has had a sig- rare conditions have been kindly provided by a number of col-
nificant influence on the two previous editions and his accuracy leagues throughout the United Kingdom and elsewhere and their
and meticulous attention to detail has been extremely helpful. I contribution has been recognized next to the images provided.
have reverted to the format that was used in the sixth edition by Other individuals have helped substantially with previous editions
starting with an initial chapter on examination techniques. Special of Clinical Ophthalmology, including Terry Tarrant, the artist who
investigations remain in the chapters where they are most relevant. produced the meticulous ocular paintings. I also wish to thank
Each chapter has been updated and the latest evidence-based Kim Benson, Sharon Nash, Kayla Wolfe, Julie Taylor, Anne Collett
diagnostic and therapeutic approaches have been covered, includ- and the production team at Elsevier.
ing genetics, immunotherapy and imaging techniques. Many The ninth edition of Kanski’s Clinical Ophthalmology could not
new illustrations have been added and better examples of a range have been produced in the time available without the help of my
of conditions have been used. Jack Kanski’s idea of including assistant, Carolyn Bouter, whose resilience, diligence, intelligence
important ‘tips’ has been reintroduced. I have included sufficient and skill were evident throughout the 6 months that she worked
practical information for trainees to manage common ophthalmic with me. I have also had the good fortune to work with Jonathan
conditions in the clinic and enough detail on rare conditions to Brett, a world-class photographer and artist, whose genius is
enable them to prepare for their examinations without resorting present in hundreds of the images included in this edition. My
to the internet. wife, Susie, has been extremely supportive throughout this project
I have been extremely fortunate to have received help from and her happy and helpful nature has made the task a pleasant and
colleagues past and present, to whom I wish to express my grate- enjoyable experience.
ful thanks. The photographers and research staff at the Oxford
Eye Hospital have been wonderfully supportive. Jack Kanski John F. Salmon
generously gave me his huge collection of images. My friends in 2019
xi

Abbreviations

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

5-FU 5-fluorouracil MMC mitomycin C


GA geographic atrophy MPS mucopolysaccharidosis
GAT Goldmann applanation tonometry MRI magnetic resonance imaging
GCA giant cell arteritis MS multiple sclerosis
GDD glaucoma drainage device NF1 neurofibromatosis type I
GHT glaucoma hemifield test NF2 neurofibromatosis type II
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 OHT ocular hypertension
ICE iridocorneal endothelial OHTS Ocular Hypertension Treatment Study
ICG indocyanine green OIS ocular ischaemia syndrome
ICGA indocyanine green angiography OKN optokinetic nystagmus
ICROP International Classification of Retinopathy of OVD ophthalmic viscosurgical devices
Prematurity PAC primary angle closure
IFIS intraoperative floppy iris syndrome PACG primary angle-closure glaucoma
Ig immunoglobulin PACS primary angle-closure suspect
IK interstitial keratitis PAM primary acquired melanosis
ILM internal limiting membrane PAN polyarteritis nodosa
IMT idiopathic macular telangiectasia PAS peripheral anterior synechiae
INO internuclear ophthalmoplegia PC posterior chamber
INR international normalized ratio PCO posterior capsular opacification
IOFB intraocular foreign body PCR polymerase chain reaction
IOID idiopathic orbital inflammatory disease PCV polypoidal choroidal vasculopathy
IOL intraocular lens PDR proliferative diabetic retinopathy
IOP intraocular pressure PDS pigment dispersion syndrome
IRMA intraretinal microvascular abnormality PDT photodynamic therapy
IRVAN idiopathic retinal vasculitis, aneurysms and PED pigment epithelial detachment
neuroretinitis syndrome
PEE punctate epithelial erosions
ITC iridotrabecular contact
PEK punctate epithelial keratitis
IU intermediate uveitis
PEHCR peripheral exudative haemorrhagic
IVTS International Vitreomacular Traction Study chorioretinopathy
JIA juvenile idiopathic arthritis PH pinhole
KC keratoconus PHM posterior hyaloid membrane
KCS keratoconjunctivitis sicca PIC punctate inner choroidopathy
KP keratic precipitate PIOL primary intraocular lymphoma
LA local anaesthetic PION posterior ischaemic optic neuropathy
LASEK laser (also laser-assisted) epithelial keratomileusis PKP penetrating keratoplasty
LASIK laser-assisted in situ keratomileusis PMMA polymethyl methacrylate
LN latent nystagmus POAG primary open-angle glaucoma
LSCD limbal stem cell deficiency POHS presumed ocular histoplasmosis syndrome
LV loss variance PP pars planitis
MALT mucosa-associated lymphoid tissue PPCD posterior polymorphous corneal dystrophy
MCP multifocal choroiditis and panuveitis PPDR preproliferative diabetic retinopathy
MEWDS multiple evanescent white dot syndrome PPM persistent placoid maculopathy
MFC multifocal choroiditis and panuveitis PPRF paramedian pontine reticular formation
MIGS minimally invasive glaucoma surgery PPV pars plana vitrectomy
MLF medial longitudinal fasciculus PRK photorefractive keratectomy
MLT micropulse laser technology PRP panretinal photocoagulation
Abbreviations
xiii

PS posterior synechiae SJS Stevens–Johnson syndrome


PSD pattern standard deviation SLK superior limbic keratoconjunctivitis
PSS Posner-Schlossman syndrome SLT selective laser trabeculoplasty
PUK peripheral ulcerative keratitis SMILE small incision lenticule extraction
PVD posterior vitreous detachment SPARCS Spaeth Richman contrast sensitivity test
PVR proliferative vitreoretinopathy SPK superficial punctate keratitis
PVRL primary vitreoretinal lymphoma SRF subretinal fluid
PXE pseudoxanthoma elasticum SS Sjögren syndrome
PXF pseudoexfoliation STIR short T1 inversion recovery
RA rheumatoid arthritis SWAP short-wave automated perimetry
RAO retinal artery occlusion TAL total axial length
RAPD relative afferent pupillary defect TB tuberculosis
RD retinal detachment TEN toxic epidermal necrolysis
RNFL retinal nerve fibre layer TGF transforming growth factor
ROCK Rho-kinase TIA transient ischaemic attack
ROP retinopathy of prematurity TM trabecular meshwork
RP retinitis pigmentosa TRD tractional retinal detachment
RPC relentless placoid chorioretinitis TRP targeted retinal photocoagulation
RPE retinal pigment epithelium TTT transpupillary thermotherapy
RRD rhegmatogenous retinal detachment UBM ultrasonic biomicroscopy
RS retinoschisis US ultrasonography
RVO retinal vein occlusion 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
SF short-term fluctuation VKH Vogt–Koyanagi–Harada syndrome
SFU progressive subretinal fibrosis and uveitis VMA vitreomacular adhesion
syndrome VMT vitreomacular traction
SIC solitary idiopathic choroiditis VZV varicella zoster virus
SITA Swedish Interactive Thresholding Algorithm XL X-linked
Chapter

Examination Techniques 1
INTRODUCTION 2 Sources of error 18 TONOMETRY 27
Microperimetry 18 Goldmann tonometry 27
PSYCHOPHYSICAL TESTS 2
SLIT LAMP BIOMICROSCOPY OF THE Other forms of tonometry 29
Visual acuity 2
ANTERIOR SEGMENT 20 Ocular response analyser and corneal
Near visual acuity 4 hysteresis 30
Contrast sensitivity 4 Direct illumination 20
Amsler grid 5 Scleral scatter 20 GONIOSCOPY 30
Light brightness comparison test 6 Retroillumination 20 Introduction 30
Photostress test 6 Specular reflection 21 Indirect gonioscopy 32
Colour vision testing 7 Direct gonioscopy 33
FUNDUS EXAMINATION 21
Plus lens test 9 Identification of angle structures 34
Direct ophthalmoscopy 21
Pathological findings 35
PERIMETRY 9 Slit lamp biomicroscopy 22
Definitions 9 Goldmann three-mirror examination 23 CENTRAL CORNEAL THICKNESS 36
Testing algorithms 11 Head-mounted binocular indirect
Testing patterns 14 ophthalmoscopy 25
Analysis 14 Fundus drawing 27
High-sensitivity field modalities 17
2 Psychophysical Tests

• Pinhole VA: a pinhole (PH) aperture compensates for the


INTRODUCTION effect of refractive error, and consists of an opaque occluder
Patients with ophthalmic disease must have their vision accurately perforated by one or more holes of about 1 mm diameter (Fig.
measured and their eyes need to be examined using specialized 1.2). However, PH acuity in patients with macular disease and
techniques. Special investigations should be used to supplement posterior lens opacities may be worse than with spectacle cor-
the findings of clinical examination. Electrophysical tests, fluores- rection. If the VA is less than 6/6 Snellen equivalent, testing is
cein angiography and optical coherence tomography are discussed repeated using a pinhole aperture.
in later chapters. • Binocular VA is usually superior to the better monocular VA
of each eye, at least where both eyes have roughly equal vision.

Very poor visual acuity


PSYCHOPHYSICAL TESTS • Counting (or counts) fingers (CF) denotes that the patient is
Visual acuity able to tell how many fingers the examiner is holding up at a
specified distance (Fig. 1.3), usually 1 metre.
Snellen visual acuity • Hand movements (HM) is the ability to distinguish whether
Distance visual acuity (VA) is directly related to the minimum the examiner’s hand is moving when held just in front of the
angle of separation (subtended at the nodal point of the eye) patient.
between two objects that allow them to be perceived as distinct. • Perception of light (PL): the patient can discern only light
In practice, it is most commonly carried out using a Snellen chart, (e.g. pen torch), but no shapes or movement. Careful occlu-
which utilizes black letters or symbols (optotypes) of a range of sion of the other eye is necessary. If poor vision is due solely
sizes set on a white chart (Fig. 1.1), with the subject reading the to a dense media opacity such as cataract, the patient should
chart from a standard distance. Distance VA is usually first meas-
ured using a patient’s refractive correction, generally their own
glasses or contact lenses. For completeness, an unaided acuity may
also be recorded. The eye reported as having worse vision should
be tested first, with the other eye occluded. It is important to push
the patient to read every letter possible on the optotypes being
tested.
• Normal monocular VA equates to 6/6 (metric notation; 20/20
in non-metric ‘English’ notation) on Snellen testing. Normal
corrected VA in young adults is often superior to 6/6.
• Best-corrected VA (BCVA) denotes the level achieved with
optimal refractive correction.

Fig. 1.2 Pinhole

Fig. 1.1 Snellen visual acuity chart Fig. 1.3 Testing of ‘counts fingers’ visual acuity
1
CHAPTER
Examination Techniques 3

Fig. 1.4 Notation for the projection of light test (right eye); the
patient cannot detect light directed from the superior and
temporal quadrants
Fig. 1.5 Bailey–Lovie chart

readily be able to determine the direction from which the light


is being projected (Fig. 1.4).

LogMAR acuity Table 1.1 Comparison of Snellen and logMAR Visual Acuity
Testing
LogMAR charts address many of the deficiencies of the Snellen
chart (Table 1.1), and are the standard means of VA measurement Snellen LogMAR
in research and, increasingly, in clinical practice. Shorter test time Longer test time
• LogMAR is an acronym for the base-10 logarithm of the More letters on the Equal numbers of letters
minimum angle of resolution (MAR) and refers to the ability lower lines introduces an on different lines controls
unbalanced ‘crowding’ for ‘crowding’ effect
to resolve the elements of an optotype. Thus, if a letter on the
effect
6/6 (20/20) equivalent line subtends 5′ of arc, and each limb of
Fewer larger letters Equal numbers of letters
the letter has an angular width of 1′, a MAR of 1′ is needed for reduces accuracy at lower on low and higher acuity
resolution. For the 6/12 (20/40) line, the MAR is 2′, and for the levels of VA lines increases accuracy at
6/60 (20/200) line it is 10′. lower VA
• The logMAR score is simply the base-10 log of the MAR. Variable readability Similar readability between
Because the log of the MAR value of 1′ is zero, 6/6 is equivalent between individual letters letters
to logMAR 0.00. The log of the 6/60 MAR of 10′ is 1, so 6/60 Lines not balanced with Lines balanced for
is equivalent to logMAR 1.00. The log of the 6/12 MAR of 2′ is each other for consistency consistency of readability
0.301, giving a logMAR score of 0.30. Scores better than 6/6 of readability
have a negative value. 6 m testing distance: 4 m testing distance on
longer testing lane (or a many charts: smaller
• As letter size changes by 0.1 logMAR units per row and there mirror) required testing lane (or no mirror)
are five letters in each row, each letter can be assigned a score required
of 0.02. The final score can therefore take account of every Letter and row spacing not Letter and row spacing
letter that has been read correctly and the test should continue systematic set to optimize contour
until half of the letters on a line are read incorrectly. interaction
Lower accuracy and Higher accuracy and
LogMAR charts consistency so relatively consistency so appropriate
• The Bailey–Lovie chart (Fig. 1.5). unsuitable for research for research
○ Used at 6 m testing distance. Straightforward scoring More complex scoring
○ Each line of the chart comprises five letters and the spacing system
between each letter and each row is related to the width Easy to use Less user-friendly
4 Psychophysical Tests

and the height of the letters. The letter signs are rectan- A target must be sufficiently large to be seen, but must also
gular rather than square, as with the EDTRS chart. A 6/6 be of high enough contrast with its background. A light grey
letter is 5' in height by 4' in width. letter will be less well seen against a white background than a
o The distance between two adjacent letters on the same row black letter. Contrast sensitivity represents a different aspect
is equal to the width of a letter from the same row, and of visual function to that tested by the spatial resolution tests
the distance between two adjacent rows is the same as the described above, which all use high-contrast optotypes.
height of a letter from the lower of the two rows. o Many conditions reduce both contrast sensitivity and
o Snellen VA values and logMAR VA are listed to the right VA, but under some circumstances (e.g. amblyopia, optic
and left of the rows respectively. neuropathy, some cataracts, and higher-order aberra-
• The ETDRS (Early Treatment Diabetic Retinopathy Study) tions), visual function measured by contrast sensitivity can
chart is calibrated for 4 m. This chart utilizes balanced rows be reduced whilst VA is preserved.
comprising Sloan optotypes, developed to confer equivalent o Hence, if patients with good VA complain of visual
legibility between individual letters and rows. ETDRS letters symptoms (typically evident in low illumination), contrast
are square, based on a 5 x 5 grid, i.e. 5' x 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
• Computer 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 • The Pelli-Robson contrast sensitivity letter chart is viewed
assessment such as contrast sensitivity (see below). at 1 metre and consists of ows of letters of equal size (spatial
frequency of 1 cycle per degree) but with decreasing contrast of
0.15 log units for groups oQ!,Jree letters (Fig. 1.7). The patient
TIP LogMAR charts are commonly used in clinical trials reads down the rows of letters until the lowest-resolvable
because they are the most accurate method of measuring VA. group of hree is reached.
• Sinusoidal (sine wave) gratings require the test subject to
view a seque ce of increasingly lower contrast gratings.
Near visual acuity • Spaeth 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 accessea online. Each patient is supplied with an identification
and ETD RS 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 measuredi' .,:5-10 minutes per eye and measures both central and peripheral
and noted. The patient wears any necessary distance correcfion I contrast sensitivity. Since the test is based on gratings it can be
together with a presbyopia correction if applicable (usually their used on illiterate patients.
own reading spectacles). The smallest type legible is recorde for
each eye individually and then using both eyes together (Fig. 1.6).

Contrast sensitivity
• Principles. Contrast sensitivity is a
visual system to distinguish an object against its background.
V R s K D R
N H C s 0 K
s C N 0 z V
C N H z 0 K
N 0 D

Fig. 1.6 Near visual acuity using a magnifying lens Fig. 1.7 Pelli-Robson contrast sensitivity letter chart
1
CHAPTER
Examination Techniques 5

Amsler grid • Chart 5 consists of horizontal lines and is designed to detect


metamorphopsia along specific meridians. It is of particular
The Amsler grid evaluates the 20° of the visual field centred on use in the evaluation of patients describing difficulty reading.
fixation (Fig. 1.8). It is principally useful in screening for and mon- • Chart 6 is similar to chart 5 but has a white background and
itoring macular disease, but will also demonstrate central visual the central lines are closer together, enabling more detailed
field defects originating elsewhere. Patients with a substantial risk evaluation.
of choroidal neovascularization (CNV) should be provided with • Chart 7 includes a fine central grid, each square subtending an
an Amsler grid for regular use at home. angle of a half degree, and is more sensitive.

Technique
TIP An Amsler grid is a simple and easy method of monitoring The pupils should not be dilated, and in order to avoid a photo-
central visual field and is commonly abnormal in patients with stress effect, the eyes should not yet have been examined on the
macular disease.
slit lamp. A presbyopic refractive correction should be worn if
appropriate. The chart should be well illuminated and held at a
comfortable reading distance, optimally around 33 cm.
Charts
• One eye is covered.
There are seven charts, each consisting of a 10-cm outer square • The patient is asked to look directly at the central dot with
(Figs 1.9 and 1.10). the uncovered eye, to keep looking at this, and to report any
• Chart 1 consists of a white grid on a black background, the distortion or waviness of the lines on the grid.
outer grid enclosing 400 smaller 5-mm squares. When viewed • Reminding the patient to maintain fixation on the central dot,
at about one-third of a metre, each small square subtends an he or she is asked if there are blurred areas or blank spots any-
angle of 1°. where on the grid. Patients with macular disease often report
• Chart 2 is similar to chart 1 but has diagonal lines that aid fixa- that the lines are wavy whereas those with optic neuropathy
tion for patients with a central scotoma. tend to remark that some of the lines are missing or faint but
• Chart 3 is identical to chart 1 but has red squares. The red-on- not distorted.
black design aims to stimulate long wavelength foveal cones. • The patient is asked if he or she can see all four corners and all
It is used to detect subtle colour scotomas and desaturation in four sides of the square – a missing corner or border should
toxic maculopathy, optic neuropathy and chiasmal lesions. raise the possibility of causes other than macular disease, such
• Chart 4 consists only of random dots and is used mainly to as glaucomatous field defects or retinitis pigmentosa.
distinguish scotomas from metamorphopsia, as there is no The patient may be provided with a recording sheet and pen
form to be distorted. and asked to draw any anomalies (Fig. 1.11).

Fig. 1.8 Amsler grid superimposed on the macula. The central


fixation dot of the grid does not coincide with the foveal Fig. 1.9 Amsler grid chart
anatomical centre in this image (Courtesy of A Franklin)
(Courtesy of A Franklin)
6 Psychophysical Tests

Fig. 1.10 Amsler charts 2–7


(Courtesy of A Franklin)

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:
• A light from an indirect ophthalmoscope is shone first into the
normal eye and then the eye with suspected disease.
• The patient is asked whether the light is symmetrically bright
in both eyes.
• In optic neuropathy the patient will report that the light is less
bright in the affected eye.
• The 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
• Principles. Photostress testing is a gross test of dark adapta-
tion 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 dependent
Fig. 1.11 Stylized Amsler recording sheet shows wavy lines 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
1
CHAPTER
Examination Techniques 7

when ophthalmoscopy is equivocal, as in mild cystoid macular match those within the spectrum. Any given cone pigment may
oedema or central serous retinopathy. It may also differentiate be deficient (e.g. protanomaly – red weakness) or entirely absent
visual loss caused by macular disease from that caused by an (e.g. protanopia – red blindness). Trichromats possess all three
optic nerve lesion. types of cones (although not necessarily functioning perfectly),
• Techniques while absence of one or two types of cones renders an individual
○ The best-corrected distance VA is determined. a dichromat or monochromat, respectively. Most individuals with
○ The patient fixates on the light of a pen torch or an indi- congenital colour defects are anomalous trichromats and use
rect ophthalmoscope held about 3 cm away for about 10 abnormal proportions of the three primary colours to match those
seconds (Fig. 1.12A). in the light spectrum. Those with red–green deficiency caused by
○ The photostress recovery time (PSRT) is the time taken abnormality of red-sensitive cones are protanomalous, those with
to read any three letters of the pre-test acuity line and is abnormality of green-sensitive cones are deuteranomalous and
normally between 15 and 30 seconds (Fig. 1.12B). those with blue–green deficiency caused by abnormality of blue-
○ The test is performed on the other, presumably normal, sensitive cones are tritanomalous. Acquired macular disease tends
eye and the results are compared. to produce blue–yellow defects, and optic nerve lesions red–green
○ The PSRT is prolonged relative to the normal eye in defects.
macular disease (sometimes 50 seconds or more) but not
in an optic neuropathy. Colour vision tests
• The Ishihara test is designed to screen for congenital protan
and deuteran defects. It is simple, widely available and fre-
Colour vision testing quently used to screen for a red–green colour vision deficit.
The test can be used to assess optic nerve function. It consists
Introduction of a test plate followed by 16 plates, each with a matrix of dots
Assessment of colour vision is useful in the evaluation of optic arranged to show a central shape or number that the subject
nerve disease and in determining the presence of a congenitally is asked to identify (Fig. 1.13A). A colour-deficient person will
anomalous colour defect. Dyschromatopsia may develop in retinal only be able to identify some of the figures. Inability to identify
dystrophies prior to the impairment of other visual parameters. the test plate (provided VA is sufficient) indicates non-organic
Colour vision depends on three populations of retinal cones, visual loss.
each with a specific peak sensitivity: blue (tritan) at 414–424 nm, • The City University test consists of 10 plates, each containing
green (deuteran) at 522–539 nm and red (protan) at 549–570 nm. a central colour and four peripheral colours (Fig. 1.13B) from
Normal colour perception requires all these primary colours to which the subject is asked to choose the closest match.

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
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"Oh," said Zep, feeling rather proud, "I just picked it up, I guess."
"Well," said the monarch, "there is nothing like it to my mind.
Perhaps you've read my famous poem on the subject? Have you?"
"No," said Zep, "I never heard of it."
"Humph!" said the Emperor, looking rather disappointed. "You're not
very literary, are you? However, there is no reason why you should
not hear it now. Listen."

When I was a lad, I said to myself


As I hooked the jam from the pantry shelf,
"I may grow up and I may grow old
But I hope I'll never do as I'm told.
"For all the fun I've ever had,
"Has always come from being bad."

So I started out on my wild career,


And I did so well that I'm Emperor here,
Where you're told to do this, and you simply don't—
And you're asked to do that, and you say you won't.
And my what a lot of fun I've had—
For I never mind, and I'm awful bad.

"You can see," said the Emperor, when he had finished, "what a
splendid place you have come to. And as the years pass, I hope you
may find it even more delightful."
"As the years pass," repeated Zep. "Why—why, I can't stay here for
years. What would my folks say?"
"If you ask me," put in the Poppykok, "I should say they'd say: 'thank
goodness, he's gone at last.'"
"Yes," said the Emperor, "it's only in Obstinate Town that people like
boys like you. Everywhere else they think you're a nuisance. Didn't
you know that?"
"Why—why, no," said Zep. "I—I thought everybody liked me."
"Ho, ho, ho!" roared the Poppykok, shaking with merriment.
"Hee, hee, hee!" cackled the Emperor, "my word, that's good! You
ought to send that to a comic paper. He thought everybody liked
him."
"Well," said Zep, sulkily, "they always acted as though they did. I—I
like people to like me. But as long as they don't I'll never go back."
"That's the stuff," said the Emperor. "Don't you do it. You stay here
with me and enjoy yourself. Do as you please. Be as cranky as you
like. Why, I wouldn't be surprised if you'd be a popular idol some day
if you go on the way you've begun."
So Zep settled down in Obstinate Town determined to enjoy himself
with all his might. And because he was a prince, the Emperor let him
live in the palace and eat his meals at the royal table.
However, he did not care much for the meals. You never could get
what you wanted. When you asked the royal butler for cold chicken,
he would always tell you he would rather you took cold ham. And if
you wanted stewed kidneys, the butler right away said he preferred
to give you broiled oysters. No matter what you asked for, the
stubborn old butler always insisted on giving you something else,
whether you liked it or not. And such an arrangement made Zep
awfully cross.
"I don't see why you have such a butler," he said to the Emperor.
"When I ask our butler at home for anything, he gives it to me quick.
He wouldn't dare give me anything else. If he did my father would
hang him."
"Humph!" responded the Emperor, "it seems to me your father must
be a very cruel person. The idea of hanging any one for wanting his
own way."
"But," said Zep, "it's so—so inconvenient. If they have their own way
how can you have yours?"
"Well," said the Emperor, "you can't, with a butler, unless you go to
the pantry and help yourself. And yet, why shouldn't he have his way
as well as you? Why shouldn't he?"
And the Prince did not know what to say to that. But nevertheless it
was tough to have every one else having their own way as well as
you. When you got in a trolley car and told the conductor to let you
off at a certain street, he would stop the car at another street, and
unless you were stronger than he, would put you off there no matter
how much you struggled and yelled. And one day, when the Emperor
and Zep were put off six blocks from their destination, the monarch
was dreadfully angry.
"I know I told you I thought other people ought to have their own way
the same as you and I," he said to Zep, "but when a conductor not
only puts me off his car before I want to get off, but kicks me into the
bargain, it's too much."
"That's what I think," said Zep, "and if I were you I'd issue a royal
decree saying that only the upper classes can have their own way
always, and that the lower classes can only have their own way,
when it suits the upper classes."
"A good idea," said the Emperor, "I'll do it."
And despite the fact that it made the lower classes fairly purple with
indignation, the decree was issued at once, and Zep, and the
Emperor, and the rest of the upper classes, did as they liked
whenever they wanted to, and had a fine time doing it.
"I tell you what," said the Emperor to the Prince one morning after
breakfast as he finished reading the paper, "that was a grand idea of
yours, Zep, about letting the lower classes have their own way only
when it suited us. Life has been much sweeter ever since."
"I think so, too," said Zep, "except that if nobody else could have
their own way, it would be sweeter still."
"Hum," said the monarch, "I never thought of that. And the more I
think of it, the more I think you're right. I know what I'll do. I'll issue
another decree putting all the upper classes into the lower classes,
except myself. Then I can do whatever I want, no matter what
anybody says."
"But," said Zep, "you wouldn't put me in the lower classes, would
you?"
"Why not," replied the Emperor. "Suppose I wanted my own way
about something at the same time that you wanted your own way
about it, the only way it could be managed without a fight, would be
for you to be in the lower classes where you couldn't have your own
way unless it suited me. See?"
"Yes," said Zep, sulkily, "I see, but I don't think it's fair. Why not put
yourself in the lower classes and let me stay in the upper class?"
"Impossible," said the Emperor, "for if any one ever belonged to the
upper classes an Emperor does."
"So does a prince," said Zep.
"Not necessarily," replied the monarch. "I had a dog named Prince
once, but you never heard of a dog named Emperor, did you?"
And as Zep could think of nothing to say to that, the Emperor issued
his decree, and Zep and all the rest of the upper classes were put in
the lower classes, and the monarch enjoyed himself more than ever.
But if the Emperor enjoyed himself, Zep and the rest of the upper
classes did not. For if they wanted to do something the Emperor
always wanted them to do something different. And if he did not want
that, he wanted them to do something nobody could do. And as Zep
lived in the palace he had it worse than anybody else.
He was told to hold his breath for an hour; to stand on his ear for half
an hour, and not wink for fifteen minutes. And when he did not do
what he was told because he could not, the Emperor stuck pins in
him and dared him to yell.
"See here," said Zep to the monarch, "I used to like you but I don't a
bit any more. I'm going back home right off."
"Very well," said the Emperor, "go ahead. I'm tired of you anyway.
The idea of a strong, healthy boy not being able to stand on his ear,
and making such a fuss, too, because a few pins are stuck in him.
Go on, go back home."
"But," said Zep, "how will I get there? I—I don't know the way."
"Of course you don't," replied the monarch, "nobody does. There
isn't any way."
"Isn't any way?" repeated the Prince in a tone of horror. "Why—why,
have I got to stay here with you always?"
The Emperor nodded. "Sure thing, unless a Kokkipop sends you
back. The Poppykoks bring you here and the Kokkipops send you
back. But as no one ever wants to go back it's mighty hard to find a
Kokkipop, so I guess I'll be sticking pins in you for some time yet.
Ho, ho, ho!"
Well, you can be sure when the Emperor said that and laughed
about it, too, Zep felt about as gloomy as he ever had in his life.
"Oh, dear," he said, "what on earth shall I do? If only I can get away
from this nasty old place I'll never want my own way again. I'll be a
different boy. I never—"
"Here, here," put in the Emperor, sternly, "stop that talk. You mustn't
say such things as that. No one ever talks about not wanting their
own way in Obstinate Town. It's downright treason. Do you want to
go to prison? But anyhow, I don't suppose you meant it."
"Indeed, I did," said Zep, "I meant every word I said. I'm tired of
having my own way—it's silly. Look at the mess it's got me into. I'm
going to be different—"
"Stop!" shrieked the Emperor, at the top of his lungs, "stop, I say!
You'll have a Kokkipop here in another moment, and oh, how I hate
'em. I hate 'em worse than—than spiders. And—and, my goodness
gracious sakes alive, you've brought one—you've brought one. Run,
run, or the Kokkipop will get you!"
And with that the Emperor dived under his throne, while the Prince,
looking about with a startled air, did not know whether to flee or not.
And then, as he hesitated, a very brisk old gentleman, dressed in
bright yellow, came into the room.
"Did you call?" he asked Zep.
"Call," said the boy, "why—why, no. What do you mean?"
"Did you call for a Kokkipop?" repeated the other testily. "And for
mercy's sake don't say you didn't, for I've been waiting for a call all
my life. I was a young man when I joined the Kokkipops, and in all
that time I have never been called until now. So I hope you did call.
Did you?"
"Well," said Zep, "I said I wanted to go home, if that's what you
mean."
"And you said you didn't want your own way any more, didn't you?"
inquired the Kokkipop, eagerly.
"Yes," replied the Prince, "I did. And I don't."
"He does, too," put in the Emperor, sticking his head out from under
his throne. "He doesn't mean what he says. He's just mad at me for
sticking pins in him."
"I don't believe it," said the Kokkipop, scowling at the Emperor,
"you're just trying to keep me out of a job." Then he turned to the
Prince. "You did mean what you said, didn't you?"
"I certainly did," said Zep, "and—"
"Whoopee!" yelled the Kokkipop, joyfully, "then I have got a job at
last."
Whereupon he took off his coat, rolled up his sleeves, and began to
paste magic postage stamps all over the Prince. "There," he said,
standing off to admire his work, "I guess that will take you back all
right."
"Take him back," sneered the Emperor, crawling from under his
throne, "why it'll take him twice over. You've put excess postage on
him. Shows what a Kokkipop knows about his business."
"Is that so," retorted the Kokkipop, "well, I know enough to send this
boy where you won't stick pins in him any more, and where he won't
want his own way any more." He turned to Zep. "Isn't that so?"
"Yes, indeed," said the Prince.
"Then," responded the Kokkipop, "here's to a quick and comfortable
trip. Good-by, I'll see you later."
"No—wait!" shouted the Emperor, running toward Zep, "don't go. I'll
put you in the upper classes again. I'll—"
But it was no use. Once again Zep felt himself leap into the air, and
whiz, and whiz, and whiz, even faster than he had before. And then
just as he was beginning to get used to the whizzing and rather
enjoy it, he commenced to descend in a graceful curve, and
presently landed with a bump in the gardens adjoining his father's
palace. And there, sitting on the grass, was the Kokkipop waiting for
him.
"Greeting," said the Kokkipop, "did you have a nice trip?"
"Fine," said Zep, "but of course I'm glad it's over and that I'm safe
home again. And of course I'm awfully obliged to you for getting me
out of such a scrape."
"Oh, that's all right," said the Kokkipop, as he peeled off the magic
postage stamps, "it's been a pleasure to help you. And who knows
but you may try to have your own way again and be taken back to
Obstinate Town. And if you do, don't forget I'm always glad to get a
job."
"All right," said Zep, "I won't, but I never expect to visit Obstinate
Town again if I can help it."
And sure enough Zep never did. From that moment he was a
changed boy, so much so that it really worried his father, the king.
"I don't understand it," said the King to his Prime Minister. "He does
just what I tell him and never whines; and when he takes a walk he
jumps about a foot if a leaf falls on him. I don't understand it."
But if the King did not, Zep did, and was determined no Poppykok
should get another chance at him.
TOOBAD THE TAILOR
Once there lived in the city of Vex a tailor named Toobad, which was
a very good name for him, for he really was too bad for anything, in
fact, he was downright wicked. And not only was he wicked but he
was also deceitful, because he was really not a tailor at all but an
enchanter or conjuror, and only practiced a tailor's trade to fool the
fathers, and grandfathers, and uncles, and big brothers of the little
boys of Vex, and make them pay him money. And this is the way he
did it:
He put a sign in his window and offered to make clothes for
gentlemen very, very cheap out of the very, very best materials that
would never wear out, and of course when he offered to do that all
the fathers, and grandfathers, and uncles, and big brothers went and
ordered their suits from him as quick as they could. But after the
clothes were made and the fathers, and grandfathers, and uncles,
and big brothers had put them on, then they found out, when it was
too late, what sort of a person Toobad was, for they had to keep on
paying, and paying, and paying for the clothes forever and forever. If
they did not the suits they were wearing got tighter and tighter until
their breath was almost squeezed out of them.
It was no use to try to get the clothes off because they simply would
not come off. So you can imagine how cross and miserable all the
fathers, and grandfathers, and uncles, and big brothers in Vex were.
Now there were lots of little boys in Vex, but the most interesting one
was a bright little fellow named Winn, because in his family there
happened to be a father, and a grandfather, and an uncle, and a big
brother all wearing suits made by Toobad the Tailor, whereas the
other boys only had a father, or a grandfather, or an uncle, or a big
brother. None of them had all four together, and therefore did not
have as much cause to dislike Toobad as Winn had.
Of course when Winn's father, and grandfather, and his uncle and
his big brother, had paid for their suits once and Toobad had told
them they must keep right on paying every week, they said they
would not. But after the suits had squeezed them once or twice, and
after they had tried to get the clothes off and found they could not,
they changed their minds. And every Saturday night as soon as they
got their salaries they rushed right down to Toobad's shop and paid
him, so they would have a comfortable Sunday, which did not please
Winn's mother at all because it left very little to buy food with.
"Good gracious," she used to say to Winn's father, and grandfather,
and uncle, and big brother, "if you keep on giving that tailor half your
money, I don't know how I'll get along."
"Indeed," said Winn's father, who was very fat, "and if I don't pay it I
don't know how I'll get along. I've got to breathe, haven't I?"
"Yes," said Winn's grandfather, and his uncle, and his big brother,
who were all as fat as his father, "we would much rather breathe
than eat."
"All right, then," said Winn's mother, "go ahead and breathe but don't
blame me if you starve also, for food is so high, I can buy very little
with the money you give me."
And when she said that Winn's father, and his grandfather, and his
uncle, and his big brother would groan awfully, which made Winn
and his mother as blue as indigo, for they knew if Toobad was not
paid, the clothes Winn's father, and grandfather, and uncle and big
brother wore would squeeze them tighter and tighter so they could
not work at all, and yet if he was paid there would not be enough
money left to keep the wolf from the door.
So finally Winn determined to go and see Toobad and try and coax
him not to be so hard on his folks. "Maybe if I offer to be his errand
boy," he said, "he'll agree to let us stop paying for a while until we
catch up with our grocery bills."
But when he got to the tailor's shop he had a very hard time to coax
Toobad into having an errand boy. "No, no," said the enchanter,
testily, "I don't need an errand boy, and even if I did need one I need
the money your family pays me much more."
"But think how stylish it is for a tailor to have an errand boy," said
Winn. "All fashionable tailors send clothes home to their customers.
They never ask customers to come after their clothes. I should think
you'd be ashamed not to have an errand boy."
So, finally, after talking and talking, Toobad agreed to hire Winn as
his errand boy, and instead of giving him wages to let his family stop
paying for their clothes for a few weeks.
"But remember this," said the tailor, "you are not to tell any one about
the arrangement, because if you do all my customers will want to
stop paying until they get caught up on their grocery bills."
So Winn promised to keep the matter secret and the next morning
started in on his duties.
Now it happened that one of the first persons he delivered clothes to
was a second cousin of his mother's aunt. This second cousin had
not heard of the trouble in Winn's family because Winn's father, and
grandfather, and uncle, and big brother had been afraid to tell any
one what Toobad had done to them for fear their clothes would
squeeze them worse than ever. So when Winn delivered his
mother's aunt's second cousin's clothes he did not know whether to
warn him about putting them on or not. And while he was trying to
make up his mind about it, his mother's aunt's second cousin went
into another room to get the money to pay for the clothes, and when
he came out he had the clothes on.
"Gee whiz," he said proudly, "don't they fit me grand?"
"Maybe they do," said Winn, "but I was just going to tell you not to
put them on, because now you can't get them off, and you've got to
keep on paying for them forever and forever."
"What!" yelled his mother's aunt's second cousin.
And then with another yell he began tearing at the clothes with all his
might, trying to get them off, but of course it was no use for although
he almost turned himself inside out, they stuck to him like sticking
plaster.

He began tearing at his clothes with all his might

"You're a nice one!" he shouted, shaking his finger under Winn's


nose. "You ought to be arrested. How dared you sit there and let me
put these awful things on? I just hope your father, and your
grandfather, and your uncle, and your big brother get stuck the same
way. I certainly do!"
"They are stuck," said Winn. "They've been stuck for some time.
That is why I am working for Toobad. And I'm very sorry I did not
warn you about the clothes in time."
And then he told his mother's aunt's second cousin what a fix his
folks were in and how if you did not pay for the clothes every week
they squeezed you until you did.
"Sakes alive!" groaned his mother's aunt's second cousin, "isn't it
dreadful the trouble some people have? Here I am all dressed up
fine but I can't enjoy it a bit after what you've told me."
Then he escorted Winn to the door and said he never wanted to see
his face again. "I'm sorry to have to say it," he continued, "but until
you came a little while ago my life was full of sunshine and now it is
nothing but a mud puddle. But I forgive you. Good-by!"
Well, you may be sure Winn felt terribly gloomy as he went back to
Toobad's shop. When he hired out as the tailor's errand boy in order
to help his family, he had not thought how he would be bringing
distress into other families by delivering Toobad's enchanted clothes.
But he could see now, after the scene with his mother's aunt's
second cousin, how selfish and wicked it was for him to help Toobad
get other people into trouble in order to make things easier for his
own folks. So he determined that he would give up his job right
away.
"I've decided not to be your errand boy any longer," he said to the
enchanter, as he handed him the money he had received from his
mother's aunt's second cousin. "I find you are too wicked to work
for."
"Humph!" said the tailor, "and why am I any wickeder now than I was
this morning? You were glad to work for me then."
"I know," said Winn, "but I have just seen my mother's aunt's second
cousin turn from a carefree, happy person into a miserable wretch,
and all because I delivered him one of your enchanted suits of
clothes. And I cannot help you in your crimes any longer even if my
family do suffer. Good afternoon!"
"Good afternoon nothing!" shouted Toobad. "Come back here at
once. Yesterday when I did not want an errand boy you talked me
into having one, and now that I've gotten used to having one you
want me to do without one. Well, I shan't do it. You'll work for me
whether you want to or not."
And with that he stretched out his hand toward Winn and then drew it
back and when he drew it back something mysterious drew Winn
back also, and though he tried to get to the door he could not move.
"Now," said Toobad, "will you work for me or not?"
"No," said Winn, firmly, "I will never work for you if I can help it."
"Very well, then," said the enchanter, "you shall work for me because
you cannot help it."
And with that he repeated the alphabet backward like lightning,
wiggling his fingers at the same time, and in a flash Winn was
transformed into a tailor's dummy, after which Toobad placed him on
the sidewalk outside his shop with one of the enchanted suits on him
and with a sign on his breast which read:
TAKE ME HOME FOR $3.75
so people could see what fine, cheap clothes were sold inside.
And maybe Winn did not feel bad as he stood there day after day not
even able to roll his eyes or move or speak. And on Saturday night
he felt particularly bad because his father, and his grandfather, and
his uncle, and his big brother came by the shop arm in arm, all
whistling merrily because they did not have to pay Toobad any
money that week and were going to a movie instead.
"My, my!" they exclaimed, when they saw Winn by the door, "doesn't
that image look exactly like our Winn, but of course it cannot be
because it's made of wax." And then the next moment they went on
their way as happy as larks.
"Oh, dear!" said Winn to himself, miserably, "whatever am I going to
do? How am I ever going to escape from this terrible tailor? If only I
could think of some way."
And later when Toobad had brought him indoors and shut the shop,
and gone off to bed and left him standing in a dark corner, he
thought and thought with all his might, for he felt if he did not find
some way to break the enchantment he might as well die.
And then as he was still puzzling over the problem he heard a
stealthy step, and into the room came Toobad in his nightgown,
holding a lighted candle in his hand, and Winn saw that he was
walking in his sleep. And not only was he walking in his sleep but he
was talking in his sleep also, and this is what he was saying:

Of all the gents who wear my clothes


Not one has ever guessed, sir,
That he could break the magic spell
By pulling down his vest, sir.
Oh, yes, indeed, there is no need
Why he should be distressed, sir,
If he but knows enchanted clothes
Are governed by the vest, sir.

And when Winn heard what he was saying he knew right away that if
he could only escape he could easily get his father, and grandfather,
and uncle, and big brother out of the power of Toobad the tailor, for
he only had to tell them to pull down their vests and they would be
rid forever of the hateful clothing they were wearing. But alas, it was
one thing to want to get home, and another to get there, for while he
was transformed into a tailor's dummy he was utterly helpless and
could only stand and watch Toobad as he wandered about the shop
with his eyes shut and the lighted candle in his hand.
And then all of a sudden something happened that transformed him
from a tailor's dummy into a very real boy, for Toobad, not seeing
where he was going, bumped right into him and the flame of the
candle came right against Winn's nose—only for a moment—but it
was long enough to scorch it and to make Winn yell—ouch! at the
top of his lungs, and to joggle all the enchantment out of him. And if
you did not believe an enchanted person can be cured by scorching
his nose, just get yourself enchanted and scorch your nose and see
if it does not work.
Anyway, it cured Winn, and not only that but it woke Toobad up. And
when the tailor found himself in his shop with his nightgown on, and
found Winn changed from a dummy into a regular boy again, he was
furious.
"Zounds!" he shrieked, dancing up and down, "how the—what the—
where did I come from and how did you get all right again?"
And when Winn told him he was more furious than ever. "Well," he
said, "I'll soon fix you anyway." And thereupon he began to say the
alphabet backward the same as he had done before, but by the time
he had said three letters and before the enchantment had had time
to work, Winn rushed at him and knocked the candle to the floor. And
then while the shop was in darkness he unhooked the door and ran
home as fast as he could. When he got there it was past midnight
and of course every one was asleep, but by and by his mother heard
him knocking and let him in.
And you may be sure it did not take his father, or his grandfather, or
his uncle, or his big brother long to hop out of bed where they had
been sleeping with their clothes on because they could not get them
off. And maybe they were not surprised when they learned that Winn
had really been the tailor's dummy they had seen outside the shop.
And maybe they were not delighted when they found that Winn knew
of a way for them to get rid of the enchanted clothes. And maybe
they did not pull down their vests in a hurry as soon as Winn had
finished telling them about it.
"My gracious," said Winn's grandfather, as he peeled the last of the
hated garments from him, "I feel twenty years younger. And I can
hardly wait until morning to get my hands on that villainous tailor."
"Nor I," said Winn's father.
"Me, too," said Winn's uncle.
"I daren't tell you what I'll do to him," said Winn's big brother.
And the first thing after breakfast they all went around to Toobad's
shop dressed in their old clothes, and each one of them kept his
word so well that Toobad was laid up in the hospital for a week. And
every time he got well and came out again a fresh batch of victims
was waiting to send him back again, for Winn had gone all about the
city telling everybody who had bought the enchanted clothes, how to
pull down their vests and get rid of them. And, of course, one of the
first persons he told after his immediate family was his mother's
aunt's second cousin. But as his mother's aunt's second cousin had
forgotten to put on his vest when he donned his enchanted suit, he
could not pull his vest down. And so the only thing to do was to give
him chloroform and skin the clothes off him a little strip at a time.
After which they sent him to the hospital also, where he lay in bed
right alongside of Toobad the tailor.
And perhaps that is the reason Toobad is still in the hospital, for after
Winn's mother's aunt's second cousin got well, he refused to go
home, but sat down on the hospital steps to wait for Toobad. And
neither Winn's father, nor his grandfather, nor his uncle, nor his big
brother, were able to coax him away.
But as for Winn, he did not try to coax him, indeed he soon forgot all
about his mother's aunt's second cousin, for all the persons in Vex
who had been wearing Toobad's enchanted clothes, began sending
Winn presents to show their gratitude, and when you have sixteen
gold watches, and a couple of ponies, and skates, and air guns, and
pretty much every sort of a thing that a boy likes, you cannot think of
much else.
The best you can do is just to enjoy yourself, and if you think Winn is
not doing that, take a trip to Vex some day and you will soon find out.
THE SNOOPING-BUG
Once there was a Snooping Bug that lived in a glass jar on a shelf in
the cottage of a Fairy Godmother. Now fairy godmothers are always
nice, but this Fairy Godmother was very nice, and the reason she
kept the Snooping Bug a prisoner in a jar on her shelf was because
she was afraid he would go about and get folks into trouble. And
another thing that showed she was unusually nice was that every
week-end she always invited a little prince or princess to be her
guest. And this story opens just as Prince Pranc, the only son of the
king of a nearby city, had arrived to spend several days with his Fairy
Godmother.
"Now, Pranc," said the Fairy Godmother, "I want you to have the
happiest kind of a time, and you'll have it without doubt if you don't
get into mischief."
"Oh, that's all right," replied the Prince, as he watched the Fairy
Godmother unpack his trunk, "if I get into mischief you just send me
home again."
"Yes," said the Fairy Godmother, "but suppose you are not here to
send home again; suppose you have disappeared. Don't forget this
is an enchanted house and that strange things can happen in an
enchanted house."
"Phew!" said Pranc, "I almost wish I hadn't come."
"Not at all," replied the Fairy Godmother, "there is nothing to be
alarmed about. You could sit on a keg of gunpowder and be perfectly
safe if you didn't explode the powder. But in case you should get into
trouble, put this ring on your finger and turn it around and around
when danger threatens."
"Oh, thank you," said the Prince, slipping on the ring. "I don't feel so
worried now."
Then the Fairy Godmother took him all over the cottage and showed
him the wonderful belongings she had, and last of all she took him
into her study and there Pranc saw the Snooping Bug in his jar on
the shelf.
"What's that?" he asked.
And the Fairy Godmother told him it was a Snooping Bug. "And this
one," she continued, pointing to another jar on the shelf, "has a
Sulking Bug in it; and this one—next to it, is a Crying Bug. If they got
out of the jars they'd bite you, and you'd start in to snoop, or sulk, or
cry."
"Whoever heard of such a thing," said the Prince. "It can't be."
"It can't, eh," said the Fairy Godmother. "Just put your finger on the
top of this bottle when I take the cork out."
And with that she took the magic stopper out of the Crying Bug bottle
and Pranc stuck his finger in. And then—ping—the next moment
something bit it, and the next moment he burst out crying, boo-hoo—
boo-hoo, as loud as he could. And as he was a boy who hardly ever
cried, he felt awfully ashamed of himself.
"Oh, dear," he sobbed, "I hate to cry this way, but—but—"
"Don't worry," said the Fairy Godmother, as she corked the bottle
again, "he only gave you a little bite. You'll be over it in a minute."
And presently the tears stopped rolling down Pranc's cheeks and he
was all right once more.
"My goodness," he said, as he wiped his eyes, "I wouldn't like that to
happen again."
"Then," said the Fairy Godmother, "see that you keep hands off
these bottles. As long as the bugs stay in the bottles everything will
be all right, but if they once get out they'll bite every girl and boy they
find. That is why I keep them prisoners. I don't care for snooping,
sulking or crying children, nor does any one else."
Then she told Pranc that she would have to leave him for awhile. "I
have been invited to the christening of a princess," she said.
So she put on her gossamer cloak and her diamond studded bonnet,
kissed her hand to Pranc and went off to the christening. But so
interested was Pranc in the bugs on the shelf he hardly noticed her
going, for the Sulking Bug looked so mad it almost startled him, and
the Crying Bug had cried so much his bottle was half full of tears and
he looked almost as mad as the Sulking Bug. But when it came to
the Snooping Bug, it was a very different affair altogether, for the
Snooping Bug, although he had a sly sort of expression in his big,
pop eyes, was real jolly looking as he slowly scratched his shoulder
blade with his hind leg. And when he saw the Prince looking at him,
he winked one eye and then turned a couple of somersaults, which
made the Prince laugh like anything.
"Gee whiz," he exclaimed, "I like this bug."
And in order to get a better look at the creature he reached the jar
down from the shelf and carried it over to the window, or at least he
started to, but before he got there he stumbled—bing—the jar
slipped from his hands, fell to the floor with a crash and broke into a
thousand pieces, leaving the Snooping Bug kicking in the midst of
the fragments.
The jar broke into a thousand pieces

"Oh," cried the Prince, "I must get something to put him in or he'll get
away."
"Nonsense," remarked the Snooping Bug. "I'm not going away. You
couldn't drive me away. I'm going to stay with you. But do get me out
of this mess, please."
So Pranc, not suspecting anything, stooped to pick the Snooping
Bug up and then as he did so—zip, the Bug bit his finger and in
about eight seconds he changed from a first class little boy who
always minded his own business and did not pry into things, into a

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