OPHTHALMOLOGY Mind Maps

MINDMAPS in
OPHTHALMOLOGY
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MINDMAPS in
OPHTHALMOLOGY
Abhishek Sharma
DPhil (Oxon), Fellow of Royal Australian and
New Zealand College of Ophthalmologists
Pediatric Ophthalmology Fellow
Hospital for Sick Children, Toronto
Ontario, Canada
CRC Press
Taylor & Francis Group
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Version Date: 20141202
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Contents
Preface vii
Acknowledgements ix
The Author xi
Abbreviations xiii
References xviii
1 Overview 1
2 Cataract and Refractive Surgery 5
3 Cornea and External Eye 27
4 Glaucoma 57
5 Neuro-Ophthalmology 79
6 Oncology 141
7 Orbit, Oculoplastics, and Lacrimal System 157
8 Pediatric Ophthalmology 193
9 Retina and Vitreous 245
10 Trauma 285
11 Uveitis and Inflammatory Eye Disease 301
Index 341
Preface
Mind Maps in Ophthalmology provides an overview of orthoptists, optometrists, general practitioners and other
clinical ophthalmology using the logical, stepwise for- health professionals. It should be used in conjunction
mat of mind maps. Each page summarizes a topic in the with a textbook for further elaboration on the topics.
field, with branches that organize the knowledge about Ophthalmologists will find this text a useful teaching
the topic. tool, simplifying complex topics and providing a struc-
This book is an excellent rapid-revision tool, suit- ture to teaching.
able for medical students, ophthalmology trainees,
vii
Acknowledgements
I gratefully acknowledge the creators of the mind map Hospital, Melbourne, Australia. Thank you to Lance
format, including Tony Buzan. These mind maps were Wobus and the team at Taylor & Francis. I am grate-
created during my final year of training for Fellowship ful for the support from my parents and my sister,
with the Royal Australian & New Zealand College of Anamika.
Ophthalmologists. I am thankful to my fellow trainees Thank you to my wife, Nipun, and my beautiful
in my year group at the Royal Victorian Eye and Ear daughters, Anjali and Ameya.
ix
The Author
Abhishek Sharma graduated with a Bachelor of Victoria, Australia in 2013 and successfully com-
Medicine and Bachelor of Surgery from the University pleted the Royal Australian & New Zealand College
of Tasmania in 2004. He completed a DPhil in Public of Ophthalmologists Fellowship examinations in
Health in the field of pediatric ophthalmology in 2009 2013. He is currently completing a fellowship in pedi-
from the University of Oxford, as a Rhodes Scholar. atric ophthalmology at the Hospital for Sick Children,
He completed his ophthalmology training at the Toronto, Ontario, Canada.
Royal Victorian Eye and Ear Hospital in Melbourne,
xi
Abbreviations
5FU: 5-fluorouracil BD: twice a day (Latin: bis die)

A/W: associated with BDUMP: bilateral diffuse uveal melanocytic proliferation
AACG: acute angle closure glaucoma BLRR: bilateral lateral rectus recession
AAION: arteritic anterior ischemic optic neuropathy BM: basement membrane
AAU: acute anterior uveitis BMI: body mass index
ABx: antibiotics BP: blood pressure
AC/A Ratio: (accommodative convergence)/(accommodation) BPES: blepharophimosis epicanthus inversus syndrome
ACA: anterior cerebral artery BRAO: branch retinal artery occlusion
ACC: adenoid cystic carcinoma BRVO: branch retinal vein occlusion
ACD: anterior chamber depth BSL: blood sugar level
ACE: angiotensin-converting enzyme BSS: balanced salt solution
ACG: angle closure glaucoma BSV: binocular single vision
ACh: acetylcholine BUN: blood urea nitrogen
ACIOL: anterior chamber intraocular lens C3F8 gas: octafluoropropane
ACPIOL: anterior chamber phakic intraocular lens Ca: carcinoma
AD: autosomal dominant CAR: cancer-associated retinopathy
AF: atrial fibrillation CCA: common carotid artery
AHP: abnormal head posture CCF: carotid–cavernous fistula
AIDS: acquired immunodeficiency syndrome CCT: central corneal thickness
AION: anterior ischemic optic neuropathy CDR: cup–disc ratio
aka: also known as cf.: (Latin) compare with
AKC: atopic keratoconjuctivitis CFEOM: congenital fibrosis of the extraocular muscles
ALL: acute lymphoblastic leukemia/lymphoma CFF: critical fusion frequency
AML: acute myelogenous leukemia CHED: congenital hereditary endothelial dystrophy
ANA: antinuclear antibodies CHRPE: congenital hypertrophy of the retinal pigment
ANCA: antineutrophil cytoplasmic antibody epithelium
Anti-TPO: antithyroid peroxidase CIN: conjunctival/corneal intraepithelial neoplasia
Anti-TSH: antithyroid stimulating hormone CLE: clear lens exchange
Anti-VEGF: antivascular endothelial growth factor CLL: chronic lymphocytic leukemia
APMPPE: acute posterior multifocal placoid pigment CME: cystoid macular edema
epitheliopathy CML: chronic myeloid leukemia
AR: autosomal recessive CMN: congenital motor nystagmus
ARC: abnormal retinal correspondence CMV: cytomegalovirus
ARMD/AMD: age-related macular degeneration CN: cranial nerve
ARN: acute retinal necrosis CNS: central nervous system
ARPE: acute retinal pigment epithelitis CNV/CNVM: choroidal neovascularization/choroidal neo-
ASCRS: American Society of Cataract and Refractive vascular membrane
Surgery CPEO: chronic progressive external ophthalmoplegia
AVM: arteriovenous malformation CRAO: central retinal artery occlusion
AZOOR: acute zonal occult outer retinopathy CRP: C-reactive protein
BARN: bilateral acute retinal necrosis CRVO: central retinal vein occlusion
BCC: basal cell carcinoma CSF: cerebrospinal fluid
BCG vaccine: Bacillus Calmette-Guérin vaccine CSME: clinically significant macular edema
BCL: bandage contact lens CSNB: congenital stationary night blindness
BCVA: best corrected visual acuity CSR: central serous retinopathy
xiii
xiv Mindmaps in Ophthalmology
CT: connective tissue G6PD: glucose-6-phosphate dehydrogenase

CT scan: computed tomography scan GA: general anesthetic
CTA: CT angiogram GAGs: glycosaminoglycans
CVA: cerebrovascular accident GCA: giant cell arteritis
CVF: confrontational visual field GDx: glaucoma diagnosis (nerve fiber analyzer from Laser
CWS: cotton wool spot Diagnostic Technologies Inc).
CXR: chest x-ray GIT: gastrointestinal tract
D: diopter GON: glaucomatous optic neuropathy
DALK: deep anterior lamellar keratoplasty GP: general physician/general practitioner
DCR: dacryocystorhinostomy GRT: giant retinal tear
DD: disc diameter GU: genitourinary
DDx: differential diagnoses GVHD: graft-versus-host disease
DIDMOAD: diabetes insipidus, diabetes mellitus, optic HAART: highly active antiretroviral therapy
atrophy, and deafness HIV: human immunodeficiency virus
DLK: diffuse lamellar keratitis HSV: herpes simplex virus
DM: diabetes mellitus HTN: hypertension
DNA: deoxyribonucleic acid HVF: Humphrey visual field
DSAEK: Descemet’s stripping automated endothelial HZO: herpes zoster ophthalmicus
keratoplasty IBD: inflammatory bowel disease
dsDNA: double-stranded deoxyribonucleic acid ICA: internal carotid artery
DVD: dissociated vertical deviation ICE syndrome: iridocorneal endothelial syndrome
EBMD: epithelial basement membrane dystrophy ICG: indocyanine green
EBV: Epstein–Barr virus ICL: implantable collamer lens
ECA: external carotid artery ICP: intracranial pressure
ECCE: extracapsular cataract extraction ICRB: International Classification of Retinoblastoma
EDTA: ethylenediaminetetraacetic acid IFN: interferon
EKC: epidemic keratoconjunctivitis IIH: idiopathic intracranial hypertension
ELISA: enzyme-linked immunosorbent assay IK: interstitial keratitis
EMG: electromyogram/electromyography ILM: internal limiting membrane
ENT: ear, nose & throat INO: internuclear ophthalmoplegia
EOG: electro-oculogram IO: inferior oblique
EOM: extraocular muscles IOFB: intraocular foreign body
EPG/iEPG: electrophoretogram, hyperviscosity screen IOID: idiopathic orbital inflammatory disease
EPO: erythropoietin IOL: intraocular lens
ERG: electroretinogram IOOA: inferior oblique over action
ERM: epiretinal membrane IOP: intraocular pressure
ESR: erythrocyte sedimentation rate IQ: intelligence quotient
ET: esotropia IR: inferior rectus
ETT: endotracheal tube IRMA: intraretinal microvascular abnormalities
EUA: examination under anesthesia IRVAN: idiopathic retinitis, vasculitis, aneurysms, and
EVP: episcleral venous pressure neuroretinitis
EW nucleus: Edinger–Westphal nucleus ITC: iridotrabecular contact
FAP: familial adenomatous polyposis IV: intravenous
FAZ: foveal avascular zone IVDU: intravenous drug use
FB: foreign body IVTA: intravitreal triamcinolone acetonide
FBC/FBE: full blood count/full blood examination JFT: juxtafoveal telangiectasia
FDT: frequency doubling technology JIA: juvenile idiopathic arthritis
FEF: frontal eye fields JXG: juvenile xanthogranuloma
FEVR: familial exudative vitreoretinopathy KPs: keratic precipitates
FFA: fundus fluorescein angiography LA: local anesthetic
FISH: fluorescence in-situ hybridization LASEK: laser epithelial keratomileusis
FK syndrome: Foster–Kennedy syndrome LASIK: laser-assisted in situ keratomileusis
FLAIR MRI: fluid attenuated inversion recovery magnetic LCA: Leber’s congenital amaurosis
resonance imaging LFT: liver function test
FTA-ABS: fluorescent treponemal antibody absorbed LG: lacrimal gland
FTMH: full-thickness macular hole LGV: lymphogranuloma venereum
Abbreviations xv
LL: lower lid OCD: obsessive compulsive disorder

LMN: lower motor neurons OCP: ocular cicatricial pemphigoid
LOC: loss of consciousness OCT: optical coherence tomography
LogMAR: logarithm of the minimum angle of resolution OHS: ocular histoplasmosis syndrome
(visual acuity chart) OHT: ocular hypertension
LP: lumbar puncture OIS: ocular ischemic syndrome
LPS: levator palpebrae superioris ONH: optic nerve head
LR: lateral rectus OPA1: optic atrophy 1 gene
LRI: limbal relaxing incision OSSN: ocular surface squamous neoplasia
LTS: lateral tarsal strip PAC: primary angle closure
M/C/S: microscopy/cultures/sensitivity PACG: primary angle closure glaucoma
MALT: mucosa-associated lymphoid tissue PACS: primary angle closure suspect
MAR: melanoma-associated retinopathy PAM: primary acquired melanosis
MAs: microaneurysms PAN: polyarteritis nodosa
MELAS: mitochondrial encephalomyopathy, lactic acido- PAS: peripheral anterior synechiae
sis, stroke PC: posterior capsule
Mentor B-VAT tester: Mentor Baylor Video Acuity Tester PCA: posterior cerebral artery
(for stereoacuity) PCIOL: posterior chamber intraocular lens
MEWDS: multiple evanescent white dot syndrome PCNSL: primary CNS lymphoma
MFC: multifocal choroiditis PCR: polymerase chain reaction
mfERG: multifocal electroretinography PCRV: polycythemia rubra vera (hyperviscosity cause)
MG: myasthenia gravis PD: prism diopters
MGJW: Marcus–Gunn jaw-winking PDG: pigmentary dispersion glaucoma
MGUS: monoclonal gammopathy of undetermined PDR: proliferative diabetic retinopathy
significance PDS: pigment dispersion syndrome
MK: microbial keratitis PDT: photodynamic therapy
MLF: medial longitudinal fasiculus PED: pigment epithelial detachment
MMC: mitomycin C PERT: pre-enucleation radiotherapy
MR: medial rectus PESS: postenucleation socket syndrome
MRD: margin reflex distance PET: positron emission tomography
MRI: magnetic resonance imaging PF: palpebral fissure
MRV: magnetic resonance venogram PHACE syndrome: posterior fossa brain malformations,
MS: multiple sclerosis hemangiomas, cardiovascular anomalies
mtDNA: mitochondrial DNA PHMB: polyhexamethylene biguanide
MuSK: muscle-specific kinase (myasthenia due to PHPV: persistent hyperplastic primary vitreous
autoantibodies) PI: peripheral iridotomy
NaCl: sodium chloride PIC: punctate inner choroiditis
NAD: noradrenaline PIOL: primary intraocular lymphoma
NAI: non-accidental injury PION: posterior ischemic optic neuropathy
NAION: non-arteritic ischemic optic neuropathy PK: penetrating keratoplasty
NF: neurofibromatosis PLGF: placental growth factor
NFL: nerve fiber layer PML: progressive multifocal leukoencephalopathy
NL: nasolacrimal POAG: primary open angle glaucoma
NLDO: nasolacrimal drainage obstruction POHS: presumed ocular histoplasmosis syndrome
NMJ: neuromuscular junction PORN: progressive outer retinal necrosis
NMO: neuromyelitis optica PPD: purified protein derivative (tuberculosis test)
NPDR: nonproliferative diabetic retinopathy PPMD: posterior polymorphous corneal dystrophy
NSAID: non-steroidal anti-inflammatory drug PPRF: paramedian pontine reticular formation
NTG: normotension glaucoma PRK: photorefractive keratectomy
NV: neovascularization PRP: panretinal photocoagulation
NVA: neovascularization angle PSCC: posterior subcapsular cataract
NVD: neovascularization disc PSD: pattern standard deviation
NVE: neovascularization elsewhere PSP: progressive supranuclear palsy
NVI: neovascularization iris (rubeosis iridis) PTK: phototherapeutic keratectomy
OB/GYN: obstetrics/gynecology PUD: peptic ulcer disease
OAG: open angle glaucoma PUK: peripheral ulcerative keratitis
xvi Mindmaps in Ophthalmology
PVD: posterior vitreous detachment UGH syndrome: uveitis–glaucoma–hyphema syndrome

PVR: proliferative vitreoretinopathy UL: upper lid
PXF: pseudoexfoliation syndrome URTI: upper respiratory tract infection
QID: four times a day (Latin: quater in die) USS: ultrasound scan
RA: rheumatoid arthritis UV: ultraviolet
RAPD: relative afferent pupillary defect VA: visual acuity
RBC: red blood cells VDRL/RPR: venereal disease research laboratory / rapid
RE: refractive error plasma reagin
REES: recurrent epithelial erosion syndrome VEGF: vascular endothelial growth factor
RF: rheumatoid factor VEP: visual evoked potential
riMLF: rostral interstitial nucleus of medial longitudinal VF: visual field
fasciculus VHL: von Hippel–Lindau disease
RNA: ribonucleic acid VKC: vernal keratoconjunctivitis
RNFL: retinal nerve fiber layer VKH syndrome: Vogt–Koyanagi–Harada syndrome
ROP: retinopathy of prematurity VOR: vestibulocochlear reflex
RP: retinitis pigmentosa VZV: varicella-zoster virus
RPE: retinal pigment epithelium WAGR syndrome: Wilms tumor, aniridia, genitourinary,
RRD: rhegmatogenous retinal detachment retardation
Rx: treatment WBC: white blood cells
SALT: sequential aggressive local therapy WCC: white cell count
SCC: squamous cell carcinoma WEBINO: wide-eyed bilateral internuclear
SCI: subcutaneous injection ophthalmoplegia
SF6: sulfur hexafluoride gas WHO: World Health Organization
SGC: sebaceous gland carcinoma XT: exotropia
SITA: Swedish interactive threshold algorithm
SLE: systemic lupus erythematosus
SLK: superior limbic keratoconjunctivitis Studies
SLT: selective laser trabeculoplasty ACCORD: Action to Control Cardiovascular Risk in
SNP: single nucleotide polymorphism Diabetes
SO: superior oblique ADVANCE: Action in Diabetes and Vascular Disease:
SOAL: secondary ocular adnexal lymphoma Preterax and Diamicron MR Controlled Evaluation
SPK: superficial punctate keratitis (evaluation of the effects of a fixed combination of
SR: superior rectus perindopril and indapamide on macrovascular and
SRF: subretinal fluid microvascular outcomes in patients with type 2 diabetes
SRK: Sanders, Retzlaff & Kraft IOL formula mellitus)
SS antibodies: Sjögren syndrome antibodies AGIS: Advanced Glaucoma Intervention Study
SSPE: subacute sclerosing panencephalitis virus ANCHOR: Anti-VEGF antibody for the treatment of
SSRI: selective serotonin reuptake inhibitor predominantly classic choroidal neovascularisation in
STD: sexually transmitted disease AMD
SWAP: short wavelength automated perimetry AREDS: Age-Related Eye Disease Study
SXR: skull x-ray BEAT-ROP: Bevacizumab Eliminates the Angiogenic
TASS: toxic anterior segment syndrome Threat of Retinopathy of Prematurity
TB: tuberculosis BENEFIT: Betaferon®/Betaseron® in Newly Emerging
TCA: tricyclic antidepressant Multiple Sclerosis for Initial Treatment
TDS: three times daily (Latin: ter die sumendus) BOLT: A two-year prospective randomized controlled trial
TED: thyroid eye disease of intravitreal Bevacizumab or Laser Therapy (BOLT)
TEE: transesophageal echocardiogram in the management of diabetic macular edema
TFBUT: tear film break-up time BRAVO: A Study of the Efficacy and Safety of
TIA: transient ischemic attack Ranibizumab Injection in Patients with Macular Edema
TM: trabecular meshwork Secondary to Branch Retinal Vein Occlusion
tPA: tissue plasminogen activator BVOS: Branch Vein Occlusion Study
Trab: trabeculectomy CATT: Comparison of Age-Related Macular Degeneration
TSH: thyroid stimulating hormone Treatments Trials
TVO: transient visual obscuration CHAMPS: The Controlled High Risk Avonex® Multiple
UBM: ultrasound biomicroscopy Sclerosis Trial
UEC: urea, electrolytes, creatinine CIGTS: Collaborative Initial Glaucoma Treatment Study
Abbreviations xvii
CNTGS: Collaborative Normal Tension Glaucoma Study MOTAS: Monitored Occlusion Treatment of Amblyopia
COMS: Collaborative Ocular Melanoma Study Study
CRASH: Corticosteroid Randomisation after Significant MPS: Macular Photocoagulation Study
Head Injury NASCET: North American Symptomatic Carotid
CRUISE: A Study of the Efficacy and Safety of Endarterectomy Trial
Ranibizumab Injection in Patients with Macular Edema NASCIS: National Acute Spinal Cord Injury Study
Secondary to Central Retinal Vein Occlusion OHTS: Ocular Hypertension Treatment Study
CRYO-ROP: Cryotherapy for Retinopathy of Prematurity ONTT: Optic Neuritis Treatment Trial
CVOS: Central Vein Occlusion Study PEDIG: Pediatric Eye Disease Investigator Group –
DCCT: Diabetes Control and Complications Trial Amylopia Studies
DIRECT: DIabetic REtinopathy Candesartan Trials PIER: Portland Identification and Early Referral (random-
DRCR.net: The Diabetic Retinopathy Clinical Research ized, double-masked, sham-controlled trial of ranibi-
Network zumab for neovascular ARM)
DRS: Diabetic Retinopathy Study PRONTO: Prospective Optical coherence tomography
DRVS: Diabetic Retinopathy Vitrectomy Study imaging of patients with Neovascular AMD Treated
EGPS: European Glaucoma Prevention Study with intra-Ocular ranibizumab
EMGT: Early Manifest Glaucoma Treatment Study RASS: Renin-Angiotensin System Study
ESCRS: European Society of Cataract & Refractive READ: Ranibizumab for Edema of the mAcula in Diabetes
Surgeons Study (prophylaxis of postoperative endo- study
phthalmitis after cataract surgery) RESOLVE: Safety & Efficacy of Ranibizumab in Diabetic
ETDRS: Early Treatment of Diabetic Retinopathy Study Macular Edema
ETOMS: Early Treatment of Multiple Sclerosis Study RESTORE Study: Ranibizumab monotherapy or com-
ETROP: Early Treatment for Retinopathy of Prematurity bined with laser versus laser monotherapy for diabetic
EUGOGO: European Group on Graves’ Orbitopathy macular edema
EVEREST: Efficacy and safety of verteporfin photody- RISE and RIDE Studies: Prospective, double-masked,
namic therapy in combination with ranibizumab or phase 3 trials, compared the use of monthly ranibi-
alone versus ranibizumab monotherapy in patients zumab to sham injections in patients with vision loss
with symptomatic macular polypoidal choroidal and diabetic macular edema
vasculopathy SAILOR: Safety Assessment of Intravitreal Lucentis for
EVS: Endophthalmitis Vitrectomy Study AMD
FFSS: Fluorouracil Filtering Surgery Study SCORE: Standard Care vs. Corticosteroid for Retinal Vein
FIELD: Fenofibrate Intervention and Event Lowering in Occlusion
Diabetes STOP-ROP: Supplemental Therapeutic Oxygen for
GLT: Glaucoma Laser Trial Prethreshold Retinopathy of Prematurity
IATSG: Infant Aphakia Treatment Study Group UKPDS: The United Kingdom Prospective Diabetes Study
IONTS: International Optic Nerve Trauma Study VIEW: VEGF Trap-Eye: Investigation of Efficacy and
MARINA: The Minimally Classic/Occult Trial of Anti- Safety in Wet Age-Related Macular Degeneration
VEGF Antibody Ranibizumab in the Treatment of WESDR: Wisconsin Epidemiologic Study of Diabetic
Neovascular AMD Retinopathy
References
Banta JT, Farris BK. Pseudotumor cerebri and optic Grogan PM, Gronseth GS. Practice parameter: Steroids,
nerve sheath decompression. Ophthalmology acyclovir, and surgery for Bell’s palsy (an evidence-
2000;107(10):1907–12. based review): report of the Quality Standards
Ciulla TA, Starr MB, Masket S. Bacterial endophthalmitis Subcommittee of the American Academy of Neurology.
prophylaxis for cataract surgery: an evidence-based Neurology 2001;56(7):830–6.
update. Ophthalmology 2002;109(1):13–24. NICE: Murray A, Jones L, Milne A, Fraser C, Lourenço T,
Clark WL, Kaiser PK, Flynn HW, Jr., Belfort A, Miller D, Burr J. A systematic review of the safety and efficacy
Meisler DM. Treatment strategies and visual acuity of elective photorefractive surgery for the correction of
outcomes in chronic postoperative Propionibacterium refractive error. Review Body Report submitted to the
acnes endophthalmitis. Ophthalmology Interventional Procedures Programme, NICE, 2005.
1999;106(9):1665–70. Settas G, Settas C, Minos E, Yeung I. Photorefractive
Dawson EL, Marshman WE, Lee JP. Role of botulinum keratectomy (PRK) versus laser assisted in situ
toxin A in surgically overcorrected exotropia. JAAPOS keratomileusis (LASIK) for hyperopia correction.
1999;3(5):269–71. Cochrane Database Syst Rev 2012;6:CD007112.
Ezra DG, Beaconsfield M, Sira M, et al. Long-term Palay DA, Sternberg P, Jr., Davis J, et al. Decrease in the
outcomes of surgical approaches to the treatment risk of bilateral acute retinal necrosis by acyclovir
of floppy eyelid syndrome. Ophthalmology therapy. Am J Ophthalmol 1991;112(3):250–5.
2010;117(4):839–46. Watzke RC, Burton TC, Leaverton PE. Ruby laser
Fisson S, Ouakrim H, Touitou V, et al. Cytokine profile in photocoagulation therapy of central serous retinopathy.
human eyes: contribution of a new cytokine combination I. A controlled clinical study. II. Factors affecting
for differential diagnosis between intraocular lymphoma prognosis. Trans Am Acad Ophthalmol Otolaryngol
or uveitis. PloS One 2013;8(2):e52385. 1974;78(2):OP205–11.
Gillies MC, Simpson JM, Gaston C, et al. Five-year results Woo KI, Yi K, Kim Y-D. Surgical correction for lower lid
of a randomized trial with open-label extension of epiblepharon in Asians. BJO 2000;84(12):1407–10.
triamcinolone acetonide for refractory diabetic macular Yu-Wai-Man P, Griffiths PG. Steroids for traumatic optic
edema. Ophthalmology 2009;116(11):2182–7. neuropathy. Cochrane Database Syst Rev 2007;17(4).
xviii
1 Overview
Overview
1.1 Management Principles
1.2 Ophthalmic Emergencies
1
2
What can KILL/BLIND this patient? First Step

What can be TREATED in this patient?
Medical
History Optical
MOST
Examination Assessment Systemic
Team approach
Investigation
Previous results of investigations
Important
MOST Important, Informed consent
Anatomical DO NO Harm! Driving
e.g. Front to Back, Brain, Systemic DO
Occupation
Vascular/Infection/Inflammatory/
Trauma/Autoimmune VITAMIN D & C Management in No treatment! Do nothing! Observe!
Surgical sieve Adults NO
Metabolic/Iatrogenic/Neoplasia/Degenerative DDx Approaches Other eye
Congenital Harmful expectations of the patient!
Serious/Common/Ocular causes Harm
Harmful behavior of the patient!
Compliance
Specialties in medicine
Prognosis
e.g. Guarded
Follow-up/Confocal microscopy/Foreign body
1.1 Management Provide Life Life
Principles Saving Treatment! Life saving/Lasers
HVF/Hess & map binocular single vision
Saving
OCT Sight saving/Surgery
Follow HOBART
Bloods/Biopsy & Swabs/BP/BABY!/Botox Treatment
Everybody! Investigations What is treatable?
Angiography/Angle
Age appropriate vision – check presence
Radiology
Baby in Pathology – exclude
Tonometry/Topography/Thickness (CCT)
A PRAM Refraction – cycloplegic
Electrophysiology
Amblyopia
Anesthetic Muscle – strabismus
Phthisis bulbi/Perforation/PVR/Pre-existing pathology
There's A PRICE Genetic counseling
Retinal detachment Management in
you pay for surgery
Children Reversible causes
Inflammation/IOP raise/Infection
Opinion
CME/Cataract/Cosmesis Second opinion
Endophthalmitis/ERM/Expectations/Endothelial loss Specific treatment
Complications GROSS!
Supportive measures
Band keratopathy
ERM Genetics of condition
In uveitis, BEN e.g. Retinal Reversible – cataract, refractive
Neovascularization
GO blind dystrophies
Glaucoma Specific – vitamin A
Edema
Supportive – guide dogs/Support groups
Blind => cataract
Mindmaps in Ophthalmology
Overview
ONLY EYE
Rhabdomyosarcoma
Diplopia
Neuroblastoma CN 3 Palsy – aneurysm
Neoplastic
Proptosis, opsoclonus, ecchymoses, Horner's syndrome
Brain tumor
Retinoblastoma Urgent
Leucocoria Bilateral disc swelling
Pediatrics
Dural venous sinus thrombosis
DEATH Intracranial extension, sepsis, Orbital cellulitis
cavernous sinus thrombosis Infection
Acute bilateral Pituitary apoplexy
Trapdoor ophthalmoplegia
Operate urgently Orbital floor fracture Acute myasthenia
Trauma Life-Threatening
1.2 Ophthalmic
Emergencies
Ptosis or diplopia + dyspnea/ Myasthenia
dysphagia/systemic weakness
Glaucoma
Acute angle closure glaucoma
Orbital cellulitis
Endogenous/Exogenous
Unilateral Pain + optic neuropathy
Endophthalmitis Sight-Threatening Mucormycosis
Tap/Inject +/– Vitrectomy URGENT
Malignant hypertension
Penetrating eye injury
Trauma
Retrobulbar hemorrhage
Bilateral Giant cell arteritis
Sight-Threatening
Pituitary apoplexy
3
2 Cataract and Refractive Surgery
2. Cataract and
Refractive Surgery
2.11 Crystalline Lens

2.1 Cataract Etiology Dislocated Lens
2.12 Implanted Intraocular Lens
2.2 Types
Intraocular Lens 2.13 Patient Assessment
2.3 IoL Power Calculations
2.15 Incisional Surgery
2.17 PRK
2.4 Phacodonesis Refractive 2.14 Surgical Options 2.16 Excimer Laser Surgery
Precataract Surgery
Surgery
2.5 Surgery In Special Situations
2.19 Intraocular Surgery 2.18 LASIK
2.20 Monovision
2.6 Extracapsular Cataract Extraction
2.7 Femtosecond 2.21 Multifocal IOLs

Cataract
2.8 Intraoperative Surgery
2.9 Suprachoroidal Hemorrhage Complications 2.22 Cataract Surgery After Laser Refractive Surgery
2.10 Postoperative
Genetic & metabolic

Down’s, galactosemia, Lowe’s
Intrauterine infection
Congenital Rubella
Ocular anomalies
Aniridia
Hereditary
2.1 Cataract
Etiology Age-related
Risk
Age, smoking, UV light, medications
Traumatic
Acquired
Metabolic
Diabetes mellitus
Toxic
Steroid use, chlorpromazine
Uveitis
Secondary to ocular disease
Angle closure glaucoma
Glaukomflecken
5
6
Rigid/foldable posterior chamber IOL
AC IOL
Types
Injectable IOL
Congenital cataract
Scleral-fixated IOL
Aphakia in fellow eye
Phakic IOL
Recurrent uveitis
In the bag IOL
If IOL will No IOL Implant
Posterior segment problems Most common approach
interfere
Posterior Chamber IOL If >200 degrees rim remaining
PC tear with significant vitreous loss Capsule as support
Sulcus IOL Aim optic in bag, haptics in sulcus
Severe glaucoma
Corneal endothelial dystrophy

Secondary IOL implant
2.2 Intraocular Lens
Similar to AC IOL (IOL) Types
Indications
During ECCE/phaco after PC rupture/zonulolysis
Younger patient
Indications
Glaucoma & PAS Planned ECCE
Cornea/endothelial problems White–white horizontal + 1mm =>
Scleral-Fixated IOL Anterior Size of implant

Anterior vitrectomy
IOL tilt/dislocation Chamber IOL Procedure
Miochol
Persistent iritis, hyphema
Complications Surgical peripheral iridectomy
CME endophthalmitis To avoid pupil block glaucoma
Bullous keratopathy
Suture erosion over time
Cystoid macular edema
Complications Chronic pain
Older ACIOLs
Glaucoma
Uveitis–glaucoma–hyphema syndrome
Immersion USS, laser interferometry
Axial length IOL master, LensStar
>1 mm axial length difference

Anisometropia
IOL master, LensStar

Corneal power – central
Better than topography (e.g.
Pentacam/Galilei) – for central power
Based on optics equations
Cataract and Refractive Surgery
e.g. Holladay Theoretical Corneal diameter, refraction, ACD, lens thickness
Regression analysis on refraction results Small incision phacoemulsification

Empirical
Sander–Retzlaif–Kraff SRK formula Accurate Calculation
Centered & consistently sized capsulorrhexis Effective lens position – 0.5 mm
Theoretical + empirical difference => +/–1D
Formulas
Combination
e.g. SRK-T
Use axial length, keratometry
<22.0 mm Old formula
Hoffer/SRK-T
Axial length SRK-T, SRK-II, HofferQ, Holladay1
22.0–24.5 mm Choice of formula
Axial length, keratometry, corneal
SRK-T/Holladay, Haigis 2.3 IOL Power
diameter, base refraction (prior to
>24.5 Calculation Lens calculation cataract, after refractive), AC depth,
New formula
SRK-T lens thickness, age
Holladay2 – excellent for all eyes
Haigis-L
Power = (A constant) − 2.5x (Axial length) − 0.9 (Keratometry) SRK Formula Postlaser refractive surgery
Keratometers measure central zone K

Difficulties
Anterior & posterior corneal surface altered
Least accurate
Postkeratorefractive Historical Pre & post measurements

Surgery K's & refraction
Use formula
Methods
Contact lens method
Measure true corneal curvature
Holladay 2
Formula Most accurate of above methods
Compare with ASCRS website calculation

7
8
PXF
No IOL, close, refer to vitreoretinal
Options
Trauma
Irrigating vectis to remove lens Zonular Loss & Nucleus
Hinges Backwards
Causes Marfan
NOT an option
Posterior assisted levitation Homocysteinuria
Connective tissue disorders
Ehlers–Danlos
Weill–Marchesani
Mechanism
Disperse tension across all zonules 2.4 Phacodonesis
Intact capsulorrhexis
Capsule hooks
<4 clock hours zonular dialysis Prerequisites

Cut rhexis in weak area
Capsular Tension Rings Rhexis
Can fix to sclera
Risk rhexis rupture Tear from other direction
Localized zonular weakness

Fix rhexis with forceps, pull with rhexis forceps
Indications
e.g. PXF Controversial benefit in
generalized weakness
Study: ESCRS, emmetropes, preoperative
before surgery, 5 y follow-up Glaucoma > Cataract
Trabeculectomy then cataract later
Posterior Vitreous
PVD present Detachment
1.1% risk retinal detachment Severity Glaucoma < Cataract
Cataract, manage IOP conservatively
No PVD Glaucoma
7.7% risk retinal detachment Glaucoma = Cataract
Triple procedure
Cataract + trabeculectomy
Pupils + light projection
Evidence Trabeculectomy alone more
Potential acuity meter + laser interferometry Assess VA + Fundus successful than combined
B-scan
Gross anatomy Mature Cataract
Amblyopia
Vision blue dye to stain capsule Anisometropia

Intraoperative Preoperative
Endocapsular technique Longer axial length
Biometry
Staphylomata
Patient factors High
PXF, uveitis, DM, glaucoma, pilocarpine Retrobulbar anesthesia
Myopia Risk perforation
Homatropine 2% TDS 3/7 preop Preoperative
Lower IOP
Intraoperative Harder to express nucleus in ECCE
Tropicamide 1%, phenylephrine 10% 2/24 preop Small
Pupil Deeper AC
AC BSS + 1:1000 adrenaline Risk zonule dehiscence, PC tear
2.5 Surgery in
Intracameral phenylephrine 2.5% Postoperative
Special
Retinal detachment
Viscoelastic Situations
Intraoperative
Stretch pupil with hook For 3/12 preoperatively
Sphincterotomy Control inflammation Prednisolone 40 mg po 2/52 preoperatively
3, 6, 9, 12 o'clock
Iris hooks, Malyugin ring Wean 2/52 postoperatively
Assess visual potential

FFA, PRP & macular laser CME, optic disc edema
Control diabetes Preoperative
Physician consult Preoperative Dilate pupil preoperatively
List early in morning Gonioscopy
Assess PAS
Corneal epithelium
Uveitis 360 degrees PS & pupil block glaucoma
Poor healing risk Consider aphakia
Small pupil Worsening inflammation, deposit on lens
Wound – consider suture Intraoperative

Avoid if neovascular glaucoma Small pupil management
Avoid ACIOL IOL Diabetes Weak zonules =>

Intraoperative PC tear risk
Large optics Hemorrhage
Anterior chamber Intravitreal triamcinolone
Inflammation
Cystoid macular edema 1–2/52 Inflammation, CME, choroidal effusion
Postoperative Postoperative
Glaucoma, PDR
Risk Corneal edema, raised IOP
Posterior capsular opacification
9
10
Peritomy
Superior Rectus Stay Suture
Minimum 120 degrees

Incision 12 mm to express nucleus
3-step
15 degree, penetrate with keratome

Enlarge with corneo-scleral scissors
Viscoelastic & Paracentesis Port
2.6 Extracapsular
Cataract
Extraction Can-opener
Capsulotomy
OR envelope
Horizontal capsulotomy
Loss of capsulorhexis Grasp 4 mm superior to superior
limbus & press toward eye center
Lens too hard Nucleus Prolapse 3 steps
Indications for Conversion
Machine failure Phaco => ECCE Tilts upper pole of lens forward
With forceps, push eye inferiorly

Loss of view
e.g. corneal clarity Simcoe Cortex Removal
IOL in Bag
Close Wound 10-0 nylon sutures

Patient cooperation/movement
Small pupil <4.5 mm
Contraindications Corneal scar

White/mature cataract
Safety/effectiveness/better? Suction ring may increase IOP
Future Glaucoma
Cost – patient/public
Trabeculectomy bleb, tube
Space/time
Decentered => optic tilt. Small => phimosis

2.7 Femtosecond
Cataract
Surgery 0.5mm axial plane deviation from
Capsulorrhexis intended lens position => +/–1D RE
Size, diameter, roundness

consistent over time
Gas expansion
Complications Customizable, water-tight
PC rupture, dropped nucleus Advantages
Wound
Anterior capsule tear
Limbal relaxing incisions option
Lens Less power, endothelial cell loss,

faster visual recovery
Reproducible, blade-free
New laser technology

11
12
Prevent vitreous hydration

Lower IOP by lowering bottle height Treat if >1/5 corneal area
Descemet's
Prevents hole enlarging Membrane Separation
Posterior Direct suturing
Instruments in eye Capsule Hole
Treatment
Rotate cortical shell on hole Air or gas bubble
Dispersive viscoelastic cover hole Intracameral phenylephrine 2.5%

Small Pupil
Iris hooks
Vacuum when good apposition
Malyugin ring
Lower bottle height
Heavy viscoelastic Refill with viscoelastic

Lost Bring rhexis to center
Infusion – direct fluid away from hole
Capsulorrhexis
Risk posterior extension by pulling
Remove vitreous, cortex, lens Options Start tear opposite direction
through capsule Vitrector Anterior
Convert to ECCE
Vitrectomy
Do not chase fragments posteriorly
Small central capsulorrhexis
Sweep wounds Zonular Weakness
Vitreous Hydrodissection
Good for visualizing vitreous Intracameral triamcinolone
and postoperative recovery Consider capsular tension ring
Miochol-E intracameral 2.8 Intraoperative
Check round pupil
Complications During irrigation/aspiration
Must suture wounds Release vacuum to prevent extension
10-0 Nylon 1: 1–2 clock-hours
1-piece IOL in capsule
Zonular
Check viscoelastic not blocking phaco aspiration ports Dehiscence 2: 2–6 clock hours, no vitreous
Grades 3-piece IOL in capsule
Check irrigation & fluids
Wound Burn 3: 2–6 clock hours, vitreous Acts as capsule tension ring
Minimize phaco ultrasound
Pulse/burst modes 4: > 6 clock hours Vitrectomy, 3-piece IOL in capsule
10-0 Nylon, leave 3/12 ACIOL or sulcus 3-piece IOL

Suture wound
Wound shrinks Posterior extension
ECCE or posterior approach
Spear nucleus 21 g needle
Radial Tear Hydrodelineation
Whole nucleus No hydrodissection
Commonly vitreoretinal referral Posterior Loss
Nuclear Material Minimal force
Anterior vitrectomy
Crack nucleus
Check no vitreous in AC Meridian away from tear
Sweep with canula
Nuclear fragments
Put IOL in
Anterior chamber shallowing
Signs
Loss of red reflex
Extrusion of ocular contents & iris prolapse

0.2–0.9% operations
General
Risk factors Close wound tight

Increased axial length, IOP 8-0 nylon
Acute
Posterior sclerotomies
Management 1.5 mm diameter if eye cannot be closed
2.9 Suprachoroidal
Wait for clot to liquefy
Hemorrhage 3–5 d
Subacute
Drain with posterior sclerotomies

Spontaneous nuclear expression
Expulsive With constant infusion pressure
Extrusion of ocular contents Types

33% require second operation
Non-expulsive 20% attain VA >6/12

Hemorrhage without loss of
ocular contents Outcomes
Poor VA < count fingers if spontaneous

nucleus expression
13
14
Biometry
Transcription error
Wrong formulae Preoperative

Cause
Wrong patient
Previous refractive surgery Contact lens, eye pad

Refractive Wound Leak
Intraoperative Surprise
Wrong lens Theater
Reform AC + suture
IOL Exchange by 3–4/52
Capsular bag fibrosis can occur Descemet's SF6 gas in anterior chamber
Piggyback lens Options Membrane Tear
LASIK for hyperopes Suturing
Refractive excimer
Can regress
Other eye 5–10 d postoperative
Re-measure but no consensus Endophthalmitis
Delayed presentation
Chronic Steroids conceal
Non-infectious
Vitreous tap
Anterior segment toxic damage by substance
2.10 Postoperative
Topical drops Complications Normal cataract surgery
Toxic anterior Suture Removal
Antibiotics, anesthetics, viscoelastic Remove 1/12 + chloramphenicol QID 1/52
segment syndrome
Intraocular Causes ECCE
Balanced salt solution - pH, additives Leave at least 3/12
Instruments Peak incidence 6–10/52 postoperative

Enzymes, detergents, ultrasound baths
Irvine–Gass syndrome
12–48 h postoperatively
Diabetics
No PAIN Get CME 1–2/52 postoperatively
c.f. endophthalmitis Symptoms OCT
Blurred vision
TASS Petaloid pattern
Cystoid
Irritation/injection Investigations
Macular Edema Diabetic
Clinical FFA
Corneal edema No disc leak
Diffuse, limbus-to-limbus
Irvine–Gass
Marked AC inflammation Disc leak
AC cells, hypopyon, fibrin Signs
Topical steroids + NSAIDs
Iris damage Resolve by 3–6/12
Dilated, irregular pupil Treatment Intravitreal triamcinolone
Vitreous clear
Intravitreal avastin
Major differential
Infectious endophthalmitis
Topical steroids hourly

Treatment
Close follow-up
Worsening inflammation => tap & inject ABx

Dilation or constriction
Avoid contact sports
Aphakic correction
Lens out of visual axis
Contact lens
Conservative
Timing important
Children Prevent amblyopia
Decrease aphakic glaucoma risk

Partial dislocation
Subluxation
Portion of zones intact Terminology
Cardiologist – rule out Marfan's
Complete zonular disruption High astigmatism

Dislocation Lens in visual axis
Lens falls into vitreous
Indications Contact lens
Intolerance
Anisometropia
Recurrent pupil block glaucoma

Young
Trauma Most common Management Vitreous in AC
Old Glaucoma, angle damage, iris trauma,

Pseudoexfoliation (PXF) syndrome Lens placement
endothelial damage
Congenital ectopia lentis – AD
Manage glaucoma, pupil capture
Preoperative
Primary Dilate, lie flat, then constrict
Ectopia lentis et pupillae – AR 2.11 Dislocated
Crystalline Lens Less cystoid macular edema,
Chromosomal – nil Surgical
suprachoroidal risk
Quiet eye
Marfan's – organize cardiologist
Echocardiography Better A-scan measurement
Connective Lens in AC, no zonule support,
Weill–Marchesani tissue Causes AC aspiration
no vitreous in AC
Systemic
Ehlers–Danlos Technique
Sticklers If cataract
Intracapsular cataract extraction (ICCE)
Metabolic Secondary Posterior pars plana vitrectomy approach
Homocysteinuria
Infections – nil Aphakic & contact lens
Buphthalmos Lens choice 3-piece intraocular lens (IOL) sutured

Congenital ocular
Aniridia Anterior chamber IOL
Angle supported or Iris clip
PXF
Acquired ocular
Trauma
15
16
Observe
Asymptomatic patients, no complications
Surgery Symptoms
indications
Complications
UGH, CME, corneal touch, recurrent pupil block
e.g. sulcus IOL after PC rupture All may need vitrectomy +/– pupilloplasty
Out of bag
Inadequate IOL length for Simple lens repositioning
If zonule/capsule support good
large sulcus diameter
Classification
Modified McCannel peripheral iris fixation
PXF Most common 3-piece + iris/scleral
Zonular Treatment fixation
compromise 10-0 polypropylene suture
Trauma, prior surgery, RP
In the bag
Connective tissue disorder Fix lens to Sliding Siepser knot
Surgical intraocular structure
Marfan, Weill–Marchesani, options Scleral fixation only
Ehlers–Danlos, homocysteinuria 1-piece IOL
Iris fixation => UGH risk
2.12 Dislocated
Rhexis overlap IOL optic Scleral fixation
By 1 mm Implanted 9-0 prolene on long needle
Intraocular Lens
Minimize zonule stress Remove lens
Mild to moderate zonular Anterior or posterior pars plana approach
compromise Lens exchange
Capsular tension rings
Not for PXF – generalized weakness Secondary IOL
Scleral fixation ACIOL angle or iris/sulcus IOL/sutured IOL
Prevention
Discuss aphakia & contact lens
Centralize IOL
Ciliary sulcus with optic–
capsulor–rohexis capture
Prevent iris chafing =>
IOL placement IOL dislocation into vitreous
UGH syndrome Scleral fixed lenses outside bag
Corneal endothelial trauma
Iris clip Intraoperative
Vitreous/suprachoroidal hemorrhage
ACIOL
Iris tear, pupil distortion
Complications
Iris tilt
Astigmatism
Postoperative
Uveitis, CME, corneal edema
Infection, retinal tears/detachments

Goals of treatment
Realistic, achievable? Visual acuity
Uncorrected, subjective, cycloplegic refraction
Presbyopia
Cannot guarantee glasses-free
Assess Monovision
Will not improve BCVA Trial contact lens
Expectations
Perfect vision, near & distance Size > optical zone (6–8 mm)
Pupil prior to dilation Risk postop glare, halo symptoms
Guarantee of 6/6 uncorrected VA
Unrealistic IMPORTANT! Especially if intolerant
Blepharitis & dry eye of contact lens
Very low myopes (0 to –1 D)
who do not wear glasses Examination Tear film breakup time
Motility, fields & anatomy
Epithelial basement membrane dystrophy (EBMD) PRK better

Work requirements than LASIK
e.g. Air Force, jeweler Occupation
Lens
Social Cataract => surgery better than laser
Sports => surface better than
Activities Temporary rise in IOP through procedure
lamellar procedure
Pregnancy & breast feeding IOP Postoperative steroids

Wait 3/12 postdelivery Cornea thin postop
Connective tissue disease False low IOP measurement postop
Delayed healing
Fundus
Poor healing, unstable Glaucoma, myopic changes, visual potential
Diabetic & immunocompromised Medical 2.13 Refractive
refractive error
Patient
Decrease immune response Assessment Automated refraction
Steroids, chemotherapy Systemic medications Ectatic disorders
Check irregular astigmatism
e.g. form fruste keratoconus
Delayed healing
Isoretinoin, amiodarone, hormone Corneal topography Steep >50 D

Risk buttonhole
replacement therapy Stability of refraction Curvature
History Investigation
Contact lens wear Flat <40 D
Stop 2/52 prior to assessment Risk small flaps, free caps
Residual stromal bed thickness post LASIK
Pachymetry >250 µm
Amblyopia
Avoid operation in good eye Ocular
Wavefront aberrometry
Ocular surface disease
e.g. dry eye, recurrent erosion Other
Pupillometry
Medications
Strabismus surgery Glasses – possible need

Trial contact lens preoperative
Discuss with all, especially Presbyopia Vision – risk decreased/loss/

myopes reading without glasses change in quality
Informed Consent Important aspects
Age, presbyopia &
Dominant eye for distance, monovision Presbyopia – laser will not treat
nondominant for near
Monovision Glare, haloes, dry eyes
Establish dominant eye
Monovision discussion
Contact lens trial
17
18
Astigmatic keratotomy
Transverse & arcuate keratotomy

Corneal surgery
Arcuate keratotomies & limbal

relaxing incisions (LRI) Astigmatism
Photoastigmatic refractive Laser

Glasses &
keratectomy & LASIK An option for all
Contact Lenses
Intraocular surgery
Toric IOL
Epikeratoplasty
Automated lamellar keratoplasty Corneal surgery

Photorefractive keratectomy (PRK)
Low –0.5 to –3 D
Radial intrastromal thermokeratoplasty Less risks but prolonged recovery
Hyperopia
Laser assisted in-situ keratomileusis (LASIK)
PRK & LASIK
Laser procedures Moderate –3 D to –8 D
PRK more scarring
Laser thermokeratoplasty Phakic IOL (ICL) also reasonable
High >–8 D
Intraocular surgery Myope Phakic IOL
2.14 Refractive
AC phakic IOL
Surgery Options High & prepresbyopic
ICLs
Radial keratotomy
High & presbyopic
Refractive lens exchange
Then donor Remove epithelium, Epikeratoplasty Preferred
peripheral annular keratotomy Refraction may not be stable
Young myope Overcorrect by +0.5 D
Corneal surgery
LASIK
Microkeratome slice, cornea Keratomileusis Low To +3.5 D
cap frozen & reshaped
Hypermetrope
Automated lamellar keratoplasty High >6 D

Microkeratome slice, second Refractive lens exchange
slide, then cap re-applied Myopia
Preoperatively 1/52 Doxycycline 50 mg nocte +
Photorefractive keratectomy (PRK)
Laser procedures topical steroids QID
Medications
Laser assisted in-situ keratomileusis (LASIK) Topical antibioitics, lubricants
Postoperatively
Intracorneal stromal ring
Doxycycline, steroids
Clear lens extraction (+/– IOL)
Intraocular surgery 5/52 more
Anterior chamber
Phakic IOL implantation
Posterior chamber
Not common now
Radial corneal stromal cuts

Change curvature, decrease steep
Hyperopic shift +1.0 D or more

Refractive instability
Vision 43% patients
Radial Keratotomy
Starburst effect at night
Vascularization
Disadvantage
Incision Microbial keratitis
Mucus plugs
Globe rupture
2.15 Incisional
Refractive
Surgery LRI commonly used
Up to 3D cylinder
Anterior to limbus
Peripheral cornea, steep meridian
600 µm deep
Arcuate Keratotomies & Procedure
Limbal Relaxing Incisions (LRI) Length of incision
Nomograms
Femtosecond laser
Automated option
Relaxes one meridian, steepens orthogonal meridian

Coupling effect
Spherical equivalent unchanged
19
20
Myopia
Flatten central zone
Excited dimer laser Principles
Outside of optical zone
Removal of blend zone
Photoablation by 193 nm high UV Excimer Hyperopia
Steepens central cornea as
it blends peripherally
Pulse of laser ruptures carbon polymer
Removes tissue, not cut it
High energy, low tissue penetrance Argon Fluoride Unrealistic patient expectations
Excimer Laser
Little thermal spread Benefits Unstable refraction
Inadequate central corneal thickness

Non mutagenic
DNA mutagenicity occurs around 250 nm Dry eye
Nd:YAG Cornea
Femtosecond laser (IntraLase) Ectasias & forme fruste
Photodisruption => Absolute keratoconus
For flap Contraindications
Scarring Interstitial keratitis, significant

corneal vascularization
100–120 µm flap Microkeratome LASIK
thickness 2.16 Excimer
Stromal flap Glaucoma
Flap surgery
9 mm diameter IntraLase LASIK – femtosecond Laser Surgery Ocular
of treatment laser flap, wavefront guided LASIK External eye disease
Sub-Bowman's LASIK Connective tissue disease

Systemic
Photorefractive keratectomy Immunodeficiency
PRK
Epithelium removed, laser applied Options
Herpetic eye infections
HSV, HZO
Laser assisted subepithelial
keratomileusis Dry eye
Cornea
Similar method to PRK, but LASEK Recurrent corneal erosion syndrome
Surface Relative
preserving epithelium
Contraindications Epithelial basement membrane dystrophy
Inferior to LASIK/PRK
Atopic disorders
Similar to LASIK method, but Ocular
superficial cornea only
Glaucoma
Epi-LASEK
Pregnancy
Inferior to LASIK/PRK
Decreased predictability
Larger corrections
Haze, loss of BCVA, increased regression
Sharp transition in treated/untreated

Refractive Error Haze, regression
Epithelium regrows & fills gutters

Regression
Hyperopic PRK through hyperplasia
=> Requires large ablation area,

additional pulses
Over/undercorrection
Intraoperative
2.17 Photorefractive
Decentered ablation
Keratectomy
(Surface Ablation) Infection or infiltrate
Immediate postoperative Pain +++

Remove epithelium to access stroma
Persistent epithelial defect
Photorefractive keratectomy
Regression
PRK Complications of PRK
Mechanical (beaver blade) or
chemical (alcohol) Ectasia
Glare, ghost images, haloes

Optic aberrations
Laser assisted subepithelial Types
keratectomy – ablates BM More in higher corrections
Delayed postoperative
LASEK Early weeks, disappears 6–12 mo
Epithelial flap by alcohol
Corneal haze
Less dry eyes than PRK Use MMC 0.02% for 10–30 seconds
Unsure benefit
Less than LASIK
Blunt blade & suction ring Dry eye
Epi-LASIK
PRK avoids transecting nerves
Epithelium + BM flap
Settas et al. (2012)
Final effectiveness equivalent
No statistical difference
Studies: Surface Ablation
Versus LASIK
90% within 1D of intended correction
NICE 2005
97% are 6/12 uncorrected
21
22
Treat if BCVA decreased
Folds become permanent quickly

Striae – flap folds If slippage
Fix urgently
Early Postoperative
Complications
Folds in Bowman's
Laser Assisted In Situ Keratomileusis Fine striae, no Rx required
Lost flap
High IOP created

Avoid in glaucoma patients Microkeratome
e.g. IntraLase Sterile lamellar keratitis

Femtosecond laser Flap Diffuse lamellar
Beveled flap keratitis (DLK) Inflammation + WBC accumulation
Stable, accurate, less gutter
Leave hinge so flap can be replaced Method Topical steroids +++

+/– Flap lift & irrigation
Wavefront aberrometer guided Lift flap, interface cultured &

excimer laser irrigated with antibiotics
Photoablation Infective keratitis
Applied to stromal bed 2.18 LASIK Consider flap amputation if
antibiotics don't settle
Make residual stroma >250 µm thick
Otherwise corneal ectasia
Dry eyes & decreased 50% of patients
corneal sensation
Thin flap with flat cornea <40 D
Check preop
Buttonhole flap with steep cornea >50 D Delayed Postoperative i.e. contact lens intolerance
Microkeratome Complications
Corneal perforation Intraoperative Traumatic flap dislocation
Complications Can be years postoperative
Lost flap
3% of eyes
Epithelial erosion
Epithelial ingrowth Observe if only at edge
Central islands & decentration If center – lift flap, scrape stroma & flap
Insufficient residual stromal bed

Risks
Ectasia Prelaser forme fruste KC
Pressure-induced Late onset interface opacity similar to DLK

stromal keratitis
Related to IOP
Keratoconus – moderate severity <53 D
Current Indications
Up to –6 D
INTACS, Ferrar rings

Method Shorten cornea arc length
Displace lamellar bundles in cornea
Reduction in myopia
Intrastromal Corneal Central cornea unaffected

Ring Segments
Advantages Maintains normal shape of cornea
Keratoconus
Reduce myopia, astigmatism
Long procedure
Not for hyperopia

Disadvantages
Extrusion
Implant
Infection
2.19 Intraocular
Refractive Larger range myopia/hyperopia
Alternative procedures unsuitable Surgery
Indication Crystalline lens intact
Glasses/contact lenses unacceptable Preserves accommodation
Indications
Reduces risk of retinal detachment
Presbyopia early, shallow AC => Hyperopes c.f. clear lens extraction
reasonable CLE candidates Corneal endothelial count
Clear Lens Preoperative Should be >3 mm in ACPIOLs

Myopes >-12D Exchange AC depth
8% at 7 y Retinal detachment Perform peripheral iridotomy
Anterior chamber
IOL formulas Endothelial loss, glare, haloes
Consent
Less accurate at high refractive error
Phakic IOL No problems with dilation
If high power required
Piggyback lens Iris fixated Good for aphakics & astigmatism
One in bag, other in sulcus
Method S/E – hyphaema, IOL dislocation, iritis,
endothelial loss
Implantable collamer lens

Types
Placed in sulcus vaulting of optic over crystalline lens
Posterior chamber Need 2.8 mm AC depth
(implantable High hypermetropes unsuitable
collamer lens) S/E – glare, haloes
PCIOL too small, vaulting decrease => cataract
PCIOL too large, vaulting increase =>

pigment dispersion
23
24
Minimize spectacle need postoperatively
Dominant eye for distance vision

Refractive/Cataract Emmetropia in one eye & –1.25 D in other eye
Surgery Technique Non-dominant eye for near vision
Range –0.75 D to –1.75 D
Discuss with patient

AFTER first eye done & emmetropic
Young
Encourage Myopes
Already prefer a close focal point
Patient Selection Prepresbyopic with early cataracts
Obsessive compulsive trait
2.20 Monovision Professions – doctors, engineers,

Discourage architects, teachers
Night drivers
e.g. professional truck drivers
Preoperatively
Trial contact lens
Wait 3/12
Postoperative After 3/12, put contact lens for Review in 3 d

Intolerance emmetropia in both eyes
Most prefer monovision again
PRK
If prefer binocular emmetropia Safest, low risk
Aims to reduce glasses need for distance & near
Reduce contrast sensitivity by 25%

Fundamentals Multifocal IOLs produce 2 images on retina
Visual phenomena – glare & haloes
Lose some near & distance extremes
Assess – personality, occupation, hobbies

Indications Excellent visual potential
Strong desire for glasses independence
Unrealistic goals & personality
Astigmatism
Contraindications Relative contraindication
Previous refractive surgery IOL calculation imprecise
2.21 Multifocal IOLs
Previous monofocal lens insertion in one eye

Both eyes require multifocal
Refractive zones
ReZoom
20% have visual phenomena
AMO
Diffractive
Technis
No intermediate vision
Lens Examples
ReSTOR Diffractive
Alcon
Limited intermediate vision
Pseudoaccommodative
Crystalens
Bausch & Lomb
Capsular opacification =>
movement, hinging & buckling
25
26
Risk hyperopic surprise

Emphasize inaccuracy
Anterior corneal central radius
Patient expectations
Total corneal power Lower them
Consent
Incorrect calculations
SRK-T, Hoffer-Q, Holladay-1 Issues Top-up refractive laser
Old
They ignore some measurements, e.g. ACD formulae May need
Lens exchange or piggyback lens –
Effective lens power from corneal power especially if hyperopic
Less applicable to astigmatic

2.22 Cataract Surgery
correction only patients after Laser
Refractive Surgery Nondominant eye first
Aim low myopia
Myopic Flattens cornea, increases spherical aberration

History laser
Surgery Use aspheric IOL
Contact lens Methods
Lens choice Hyperopic Steepens cornea, reduces spherical aberration

Haigis-L laser
Least chance refractive surprise
Formulae Use spherical IOL
Website www.ascrs.org
No multifocals
Degrade contrast further
3 Cornea and External Eye
Cornea &
External Eye Non-cicatrizing
3.15 Vernal Keratoconjunctivitis
3.1 Pathology 3.17 Atopic Keratoconjunctivitis
3.18 Ocular Cicatricial Pemphigoid

Immunological
3.2 Management Algorithm 3.16 Cicatrizing
Disease:
Keratoconjunctivitis 3.19 OCP – Treatment
3.3 Blepharitis
3.20 Stevens–Johnson Syndrome
Surface disease
3.5 Dry Eye Management
3.21 Peripheral Corneal Thinning
3.6 Recurrent Epithelial Erosion Syndrome

3.4 Dry Eye
3.22 Degenerations: Pterygium, Vortex, Band Keratopathy
3.7 Sjögren Syndrome

3.24 Fuch's Endothelial Dystrophy
3.23 Corneal Dystrophy
3.8 Infectious Conjunctivitis Infectious Disease

External Eye
3.25 Keratoconus & Other Ectasias
Ectasias
3.9 Herpes Simplex Virus
Cornea: Viral Infections 3.26 Chemical Injury

3.10 Herpes Zoster Ophthalmicus Anterior Segment Injury
3.27 Perforating Injury
3.11 Infectious Keratitis - Bacterial
3.28 Corneal Transplant
3.12 Infectious Keratitis – Other Cornea: Other Infections
3.13 Interstitial Keratitis
3.14 Crystalline Keratopathy
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Spindle, long branching processes

Keratocytes
Absence
Irradiation, chemical injury, acanthamoeba
50 µm, basal, wing, superficial layers
Keratocytes => myofibroblasts +
invading capillaries
Changes in thickness Stroma Scar
Fibroblasts (trauma, ulceration)
Hypertrophy/Atrophy
=> Dense collagen
Intra/intercellular
Neovascularization
Epithelium
Basal layer best indicator Edema
Stromal deposits
Dystrophy
Bullae
Lifts off Bowman's
Elastic, resistant to collagenolysis

Stromal disintegration Ulceration Forms descemetocele
Epithelium/Keratocytes
=> collagenases Endothelial dysfunction
Descemet's rupture
3.1 Cornea
Pathology Thickening Central nodules, excrescences (guttata)
Once damaged, never replaced Fuch's
Absence/Presence Peripheral
Trauma, inflammatory damage Descemet's Aging change
Sarcoid Congenital hereditary endothelial dystrophy

Endothelial dystrophy
Uveitis Multilayering Iridocorneal endothelial syndrome
Calcium salts
Hyperparathyroid Band keratopathy
Calcification Trauma
Topical medications Bowman's Breaks
Keratoconus
EDTA acid Hydrops
Pannus
Fibrovascular inflammatory infiltrate Monolayer, hexagonal arrangement
Postaphakic/pseudophakic
Small breaks Bullous keratopathy
Attenuation/absence
Keratoconus only Endothelium
Rejection
Inflammatory cells
Viral infection
Exclude infection/environment/
educate patient
Drops/Gel/Ointment
Lubricate eye Stepwise
Cornea and External Eye
Serum tears/Punctal plug
Omega-3, blepharitis
Lid disease
Ectropion, entropion, exposure
Doxycycline 100 mg bd 3/12

Management
ELLIPT Inflammation decrease Topical steroids
Immunosuppression
Provide scaffold
Promote healing BCL, amniotic membrane
Electrolysis Electrolysis/Epilation/Laser/Lash
Trichiasis excision/Cryotherapy/Tarsal rotation
3.2 Management
Algorithm Treat complications Limbal stem cell transfer
Infection
Lagophthalmos
Assess LAB Anesthesia
Bell's phenomenon
Surface ocular disease

Complications Lubricate, lid disease, limbal stem cell failure
LAB SSS
Symblepharon
Lysis, forniceal reconstruction
Penetrating keratoplasty
Scarring corneal opacity
Keratoprosthesis
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Lipogranulomatous inflammation
Pathology
Definition Meibomian or Zeis glands
Chronic eyelid inflammation
Erythematous overlying skin
Hard fibrinous scales, matted crust Clinical
Base of lashes
Staphylococcal
Most sterile
Lead to phlycten & marginal keratitis Antibiotics limited benefit
Hyperemic greasy lid margins Causes Chalazion Hot compress & massage
Soft scales, anywhere on lashes Seborrhoeic Management Incision & drainage
Seborrhea scalp, nasolabial folds, Generalized Indications
behind ears, sternum Anterior Intrachalazion steroid Lid margins, close to puncta
Warm compresses/commercial Lid hygiene
products (Lid Care, Sterilid) Side effect
Treatment
Recurrent – suspect sebaceous
Topical antibioitics gland carcinoma Depigmentation in darker people
In acute folliculitis
Immune reaction to staphylococcal blepharitis Inflammatory &

Phlycten/marginal keratitis infectious nodules
Topical steroids
Treatment Sebaceous gland infection & inspissation
3.3 Blepharitis External hordeola/Stye
After sure not infectious Anterior eyelid, Zeis glands or lash follicles
Meibomian gland dysfunction Types
Internal hordeola
Inspissation of glands Clinical Posterior eyelid, Meibomian glands
Pouting, recession or plugging Posterior Hordeolum
Painful, tender, red,
Oily frothy tear film
Clinical nodular eyelid margin
Contraindicated
Consider tetracyclines earlier Self-limiting
Treatment
Children <12 y & pregnant women Warm compresses & massage
Delayed hypersensitivity reaction Treatment
Topical antibioitics
Chronic acneifom disorder If infectious blepharoconjunctivitis
Eyes & skin
General
Unknown cause Epithelial cell strands
Face, neck, shoulders Cutaneous sebaceous Associated with Clinical Attached to cornea over core of mucus
gland dysfunction
Fine or coarse
Chronic blepharitis
Excessive sebum secretion Dry eye
Recurrent chalazia
Acne Rosacea Filamentary Causes Neurotrophic keratopathy
Chronic conjunctivitis, marginal keratitis, Ocular Keratopathy
episcleritis, iridocyclitis Superficial limbal keratitis, recurrent epithelial erosion syndrome
Clinical Treat underlying condition
Mid-facial erythema Malar rash
Telangiectasia Debridement
Facial lesions
Recurrent papules & pustules Management Lubrication + acetylcysteine 10% QID
Rhinophyma Topical steroids
Thickening of skin over nose
Severe
Doxycycline Systemic tetracyclines Bandage contact lens
Management
100 mg bd 2/52, 50 mg bd 2/52, 25 mg bd 2/12
Contraindicated pregnant women & age <12 y

Primary
Systemic autoimmune disease
Sjögren’s
Secondary
RA, SLE, scleroderma
Aqueous tear deficiency Primary Age atrophy lacrimal gland/keratoconjunctivitis sicca

Lacrimal gland inflammation – TED, sarcoid, GVHD

Cause
Non-Sjögren’s
Secondary Lacrimal gland obstruction – trachoma, OCP, toxic
Medications
Meibomian gland dysfunction
Lagophthalmos Familial dysautonomia
Evaporative
Decreased blink rate
Contact lenses
Environment
Exacerbating factors/ Air conditioning, computer
3.4 Dry Eye Educate/
Medications
Exclude infection
Lubrication Lubricants, punctal plugs,

autologous serum
Lid disease
Omega-3 oral, blepharitis, allergy, ectropion
Steroid
Treatment Summary ELLIPT Inflammation Cyclosporin 0.05% bd

(Same for Cicatrizing Conjunctivitis) Acetylcystine 10%
Filamentary keratopathy
Doxycycline 100 mg 3/12 minimum

Promote healing Decrease metalloproteinase
Erythromycin low dose

Pregnant/Children
Symblepharon, corneal scarring, infection

Treat complications
Limbal stem cell failure => graft

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Hot compresses & massage

Drops
+/– Preservative
Tear drops Gel
Ointment
Medical Cyclosporine 0.05% bd Chronic ocular inflammation

=> aqueous tear deficiency
Acetylcysteine (mucomyst) 10%

Treat For filaments
Topical serum drops 20% 4–6x/d
Moisture chamber
Plastic film sealed at orbital rim
Punctal plugs
3.5 Dry Eye Debride filaments
Management
Surgical Punctal cautery
Realistic expectations
Tarsorrhaphy
Avoid toxic/environmental factors
Educate Botox
Blinking If temporary condition, e.g. Bell's
Avoid laser surgery

Blepharitis
Eye drop aids Ectropion
e.g. for rheumatoid arthritis patients Lid malposition or function
Floppy eyelid syndrome
Manage
Contributing Disorders Connective tissue disease
Rheumatoid arthritis, Sjögren’s
Diabetes
Iatrogenic
Medications
Initial sharp abrading injury
EBMD
Underlying basement Ad condition, most common
2 Mechanisms
Pathogenesis membrane dystrophy
Reis–Buckler, Meesmann
Stromal dystrophies
Lattice, granular, macular
Days to years later

Symptoms recur spontaneously
Debride loose epithelium
Antibiotic ointments & cycloplegia
Acute phase Lubricate Non-preserved lubricants
Ointment at night
Bandage contact lens

3.6 Recurrent Epithelial
Manage with lubricants + lid care
Erosion Syndrome
External eye disease Exclude nocturnal lagophthalmos
Omega-3 supplement oral

Sudden onset pain
Night/First awakening
80% effective
Minor episodes resolve rapidly Symptoms Anterior stromal micropuncture 27–30 G needle punctures Bowman's
Clinical Management
Redness, photophobia, tearing, Small scar postprocedure
eyelid swelling Caution in visual axis
80–90% effective
Examine other eye Excimer laser phototherapeutic
Basement membrane dystrophy Chronic disease keratectomy 193 nm UV light
Large shallow zone – 12 µm of ablation
Alternative to PTK
Diamond burr polishing Bowman's
Transient osmotic gradient,

Hypertonic saline solution 5% NaCl
draws fluid from epithelium
Controversial
Increases adhesion
Bandage contact lens + 2/12 Painful for patients

chloramphenicol + oral doxycycline
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Sarcoid, ocular cicatricial

Inflammation
pemphigoid (OCP),
blepharitis, conjunctivitis
Middle-aged women
Toxicity
Burns, drugs
Lymphocytic infiltration of Differential Diagnosis
exocrine glands
Neurological
Autoimmune VII nerve function, lacrimal gland function
T-cell mediated Background
Vitamin A deficiency
Resultant aqueous tear deficiency
Other Lid scarring
Primary
Systemic autoimmune disease
Primary vs. secondary Neurotrophic cornea
Secondary 3.7 Sjögren’s

RA, SLE, scleroderma Syndrome Schirmer's test
Local <5 mm in 5 min
Dry eye and mouth (xerostomia) Salivary gland biopsy
Medications Sjögren’s
Antihypertensives, anticholinergics Investigations SS Antibodies – SSA & SSB
Rheumatoid arthritis Rheumatoid factor
History
Systemic lupus erythematosus Systemic Antinuclear antibodies
Scleroderma Associated connective Associated
tissue disease Assessment Thyroid function tests
Vasculitis Plasma electrophoresis
Primary biliary cirrhosis
Evaporative tear dysfunction Dry eye treatment algorithm
Lid position Examination Cyclosporin A 0.05% or 0.1% bd T lymphocyte activation => decreased
Treatment lacrimal epithelial cell apoptosis
Parotid enlargement
Mucomyst 10%
Filamentary keratopathy
Rheumatologist
Follicular conjunctivitis Explosive exudative purulent, chemosis
Features
Pharyngoconjunctival fever
Periauricular lymphadenopathy Clinical Corneal infiltrate & ulceration
Keratitis occurs in 15–40%
Pseudomembranes, subepithelial Epidemic keratoconjunctivitis Investigation Gram stain
corneal infiltrates, symblepharon Culture, sensitivities
Cool compress, lubricants Adenoviral Gonococcal Ceftriaxone 1 g

Peel membranes Allergic to penicillin

Fluoroquinolones 500 mg po 5/7
If EKC symptoms
Management Treatment Irrigation, topical ofloxacin
Risk – prolong viral shedding, Topical steroids
worsen HSV Treat chlamydia
Azithromycin 1 g po stat
Prevent transmission GP
Partners, contact tracing
Sexually transmitted disease Adults

S. pneumoniae, S. aureus, S. epidermidis
History vaginitis, cervicitis, urethritis Cause
LGV: L1,2,3 Clinical Children
Peripheral corneal infiltrates Non–Gonococcal H. influenzae
Bacterial
Periauricular lymphadenopathy 3.8 Infectious No treatment
Chlamydia D–K Conjunctivitis 60% resolve 5 d
PCR
Investigation Management Empiric antibiotics
Monoclonal fluorescent antibody microscopy
H. influenzae Oral amoxicillin & clavulanate
Test (other STDs + GU), Treat, Trace
(other STDs), Two months follow-up Risk otitis media, pneumonia,
Treatment meningitis
TTTT
Genitourinary investigations first Molluscum Dome-shaped multiple umbilicated nodules
Contagiosum
Azithromycin 1 g single dose Eyelid/margin
Follicles, inflammation, scarring, FISTO

Clinical Curette lesions
trichiasis, opacity
Tarsal rotation surgery Chronic purulent conjunctivitis

Surgery
S. aureus, pseudomonas aeruginosa
Removal of aberrant lashes
Corneal vascularization
Topical/oral Chlamydia A–C Giant Fornix Associated with
Management Trachoma Syndrome Floppy eyelid syndrome
Tetracycline 1.5 g 3/52 Antibiotics Obesity, sleep apnea
SAFE
Azithromycin 1 g single dose Shield, physician review for sleep apnea
Face washing Treatment
Oral antibiotics, topical antibiotics,
topical steroids
Environment
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Unilateral belpharoconjunctivitis
Vesicles skin/lid margin Clinical

Primary
Unilateral 97%
Management Topical antiviral

Self limiting, acyclovir ointment 5x/d 2/52 400 mg oral 5x/d
Not same dose as VZV
Blepharoconjunctivitis
Self-limiting, shorten with antivirals
Necrotizing stromal disease
Dendritic ulcer, punctate keratitis Systemic acyclovir
Iridocyclitis
Geographic epithelial ulcer – if ulcer coalesce Clinical Treatment Indications
Topical toxicity
Ciliary flush, stromal edema
Prevention
Investigations
Swab PCR Epithelial keratitis Topical steroids
Stromal keratitis, uveitis
Debridement
3.9 Herpes Doxycycline
Acyclovir ointment – Stop corneal thinning
shortens clinical course Management Simplex
If on systemic Penetrating keratoplasty
steroids => treat with
Virus
Steroids contraindicated Surgery With oral acyclovir cover
systemic antiviral
Tectonic penetrating keratoplasty
Uni/multifocal stromal haze, Recurrent Impending perforation
Interstitial
minimal edema, no epithelial ulcer
Primary endothelilitis
Decrease inflammation
Disciform Stromal keratitis Topical steroids +
Round stromal/epithelial edema a/w keratic trifluridine treats Shorten duration
precipitates under edema – Wessley ring
Vision same at 6/12
Suppurative corneal inflammation Stromal keratitis
Necrotizing
Oral acyclovir + topical Non-necrotizing
Similar to Anterior steroids + trifluridine No benefit
bacterial/fungal keratitis chamber activity
Necrotizing Some benefit,
Elevated IOP Clinical Herpetic Eye small sample
Iridocyclitis Disease Study
Iris transillumination defect, corneal Too few patients
sensation decreased Iritis
Oral acyclovir 400 mg 5x/d
Trending toward benefit
Examine posterior segment
Acute retinal necrosis Oral acyclovir does not
decrease stromal keratitis risk
Epitheliopathy Epithelial keratitis

Prolonged topical antiviral Best predictor of
stromal keratitis risk
Neurotrophic keratopathy Previous history of stromal keratitis
Round/oval Complications
Oral acyclovir
Recurrent disease 50% less with prophylaxis
Bullous keratopathy prophylaxis helpful
Prolonged disciform keratitis HSV recurrence
Protective effect stops if drug stopped
Scarring & astigmatism None found
Triggers
Healthy - Age 60-90
HZO Features
Malignancy
Age <40 y
Investigation Look for immunocompromised status
Disease
Immunosuppressed
Surgery/Trauma Age >40 y Nil, unless symptoms of
immuno‐compromised
Immunosuppressive therapy
Acyclovir 800 mg 5x/d OR valaciclovir 2 g TDS
Contagious, self-limiting
Oral antiviral Reduce duration & neuralgia
Uncommon, follow-up 1–7/7 if Start within 72 h

ocular involvement Primary – Varicella
Zoster
Chloramphenicol ointment
Dermatoblepharitis, follicular Dermatitis Prevent secondary bacterial infection
Ocular
conjunctivitis, keratitis, vesicles Treatment
Topical steroids
Stromal keratitis Inflammation Stromal keratitis, disciform, uveitis
Treat as per HZO GP

Vesicular dermatitis Amitriptyline
Postherpetic neuralgia
25 mg daily dose
Single dermatome Skin Gabapentin
Pain & dysesthesia 3.10 Herpes Zoster
Avoid graft Risk neurotrophic ulcer
Ophthalmicus Unless only eye/perforation
Eyelid vesicles
Secondary bacterial infection
Review 6 wk
Punctate & dendritic epithelial keratitis
Follow-up No ocular involvement Delayed uveitis
Nummular
Subepithelial deposits, halo stromal haze
Elevated IOP
Stromal
5% cases, similar to HSV Keratitis
Ocular involvement
Disciform As per pressure, cornea & uveitis
Less common than HSV
Corneal anesthesia secondary Re-culture, antibiotics
neurotrophic keratopathy Clinical Exclude infection/epithelial defect
Ocular
Anterior uveitis Systemic aciclovir, stop topical
Lid disease
Iris – sectoral transillumination
defects due to vasculitis Lubricants
Intensive, preservative free
Neuropathic
Focal choroiditis Keratopathy Inflammation Prednisolone preservative free OD–BD
Mild topical steroids
Occlusive retinal vasculitis
Promote healing
Retinal detachment Posterior Doxycycline
Optic neuropathy
Contact lens/Botox/Tarsorrhaphy/Gunderson's flap
ARN – uncommon with HZO
Treat complications Perforation – glue/tectonic graft
1/3 of patients
Cranial nerve 3 most affected Cranial nerve palsy No role for debriding rolled edge further
HZO optic neuropathy
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Central ulcers
Vision-threatening Unresponsive to initial treatment
Lid disease
Blepharitis, trichiasis, lagophthalmos L Significant corneal thinning
Chronic corneal disease, exposure/ Ocular surface disease Analgesia/cycloplegia

dry eye, bullous keratopathy O
Don't change for 48 h
LASIK, sutures, long-term steroid Surgery Fluoroquinolone 30–60 min Does not cover Streptococcus
S
PRP, buckle Prolonged use – corneal thinning
Trauma
If no response to ofloxacin Loading dose –
Organic matter, fungal T
5x every 5 min
Fortified topicals
Contact lens wear Antibiotics Gentamicin/cefazolin
30–60 min afterwards
Familial dysautonomia (Riley–Day
syndrome), Mobius syndrome Indications
Perforation or approaching limbus
Congenital Contact
Familial hypoesthesia, insensitivity Oral antibiotics
Corneal thinning
to pain, anhidrosis Doxycycline 100 mg bd
Risk Factors Bacterial
Compliance LOST Contact LENS Ciprofloxacin 500–750 mg bd
Chemical injury, OCP, aniridia, atopic Antimetalloproteinase
keratoconjunctivitis, Steven Johnson
syndrome, peripheral ulcerative keratitis L Culture positive, not fungus/acanthamoeba
Limbal stem cell failure Indications
Frequent follow-up possible
E 3.11 Infectious Keratitis

After 48 h for Gram +
Exposure keratopathy – Bacterial
Amethocaine Steroids
Commence After 72 h for Gram –
Corneal hypoesthesia
Neurotrophic
Herpes Check in 24–48 h
N
Trigeminal nerve Especially Pseudomonas aeruginosa
Neuralgia, ablation, Can cause relapse
acoustic neuroma
Scarring/stromal keratitis
Low yield
Diabetes S Re-scrape
Not improving at 48 h 30% of MK have >1 bacteria
Systemic
Vitamin A deficiency +/– Epithelial biopsy
Ulceration Cyanoacrylate glue & bandage contact lens

Descematocele, perforation
Prior to graft
Scleritis Tectonic graft Topical antibiotics 24 h
Especially Pseudomonas Complications Perforation
Graft will fail
Scar Need PK for clarity in future
Interstitial keratitis Consider tarsorrhaphy/punctal plugs

Lipid keratopathy
Posterior synechiae
Early diagnosis
Analgesia/Cycloplegia/NO STEROIDS Most important prognostic factor
Confocal microscopy
Debridement
Helps drop penetration
Analgesia/Cycloplegia
Polyhexamethylene biguanide
NO STEROIDS
(PHMB) 0.02% hourly

General
Treatment often for months + transplantation Acanthamoeba Antimicrobial
Brolene 8x/d
Urgent penetrating keratoplasty Approaching limbus/sclera

– bad prognostic sign Can use chlorhexidene
NO STEROIDS
Epithelial debridement PK for severe cases

Enhance medication penetration
3.12 Infectious
Keratitis – Surgery
Hourly day & night Other High risk recurrence Penetrating keratoplasty after full
amebicidal therapy
Potent, wide-spectrum, newer
Voriconazole 1%
Candida, Aspergillus, Histoplasma,
Scedosporium, Fusarium Fungal
Microsporidial HAART + topical fumagillin
Natamycin 5%
Filamentous fungal keratitis
Antifungals
Yeast keratitis & Aspergillus Amphotericin B 0.15%–0.3% Mercuric oxide 1% bd for 10/7
(filamentous) Parasitic – Phthiriasis
6/52 minimum, with Voriconazole 400mg bd Wash sheets/clothes/family

liver function tests
Ketoconazole oral (filamentous) Aggressive infection
Fluconazole oral (yeast)
Fluconazole subconjunctival
Ofloxocin hourly
Co-infection common Antibiotic
Superficial keratectomy
Surgery
Excisional penetrating keratoplasty
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Bilateral, 1st–2nd decade

Congenital
Presents as late inactive chronic
Syphilis
Unilateral 60%, sectoral
Acquired
Uveitis/Retinitis more common
Common
HSV/VZV Wesley ring
Unilateral – if bilateral => syphilis
Causes Measles + vitamin A deficiency

Other infectious
TB, leprosy, Lyme
Bilateral IK
Cogan's syndrome Vertigo, tinnitus, hearing loss

Vasculitis
Corneal stromal blood vessels + edema
Associated with polyarteritis nodosa
3.13 Interstitial
No epithelial ulcer Keratitis Non-infectious Polyarteritis nodosa, Wegener's granulomatosis
Acute
Vascularization => salmon patch Rheumatoid arthritis
Collagen vascular disease
+/– Anterior chamber cells, fine keratic Clinical Relapsing polychondritis

precipitates, conjunctival injection
Sarcoidosis
Deep corneal haze
Stromal ghost vessel – no blood Chronic

Acute
IV penicillin + topical steroids
Stromal thinning
Syphilis
Inactive
Treatment As required
Other
As per cause
Corneal grafts
Infectious
Features Chronic steroids
Schnyder
Streptococcus viridans
Crystal Cadel's Bike IS BMC

Bietti's Infectious
Scrape
Multiple myeloma
Stop steroids
Management
Cystinosis Fortified antibiotics
Topical penicillin
Stromal cysts Not fluoroquinolone – streptococci resistant

Features Multiple Myeloma
Immunoglobulin G–K is most common
Autosomal dominant
Liver function tests
Management 3.14 Crystalline Central subepithelial crystals, 50% patients
Serum electrophoresis Features
Keratopathy
Associated with hyperlipidemia
Dystrophy of Schnyder
Decreased corneal sensation
Autosomal recessive
Fasting lipid profile
Crystals in corneal stroma Features
Management Phototherapeutic keratectomy
Crystals in conjunctiva, trabecular meshwork
Penetrating keratoplasty for severe
Dwarfism Cystinosis
Associated with
Renal dysfunction Autosomal recessive
Topical cystamine Features Yellow-white spots at corneal limbus

Reduce density of deposits & pain Bietti Corneoretinal
Management Dystrophy
Retinal crystals
Penetrating keratoplasty for severe
Recurs
No treatment
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Symptoms
Itch, blepharospasm, photophobia, blur
Palpebral
Papillae hypertrophy
Horner's Trantas dots

Limbal Degenerated eosinophil clumps
Thick gelatinous appearance

Clinical
Punctate keratopathy
Pannus
Sterile Superior Shallow

Corneal Shield ulcers SSSS
Secondary plaque formation
In recurrent limbal disease

Pseudogerontoxon
Appearance of localized area of arcus senilis
Mast cell stabilizers

Olopatadine
3.15 Vernal
Keratoconjunctivitis Topical Non-particulate steroids Pain/Keratopathy
Maxidex
Vernal = springtime
Immunomodulatory agents
Type I & IV Hypersensitivity Cyclosporin A 1%
General
95% remit by late teens Injection
Supratarsal corticosteroid
Young male Superficial keratectomy

Management Debride plaque
Excimer phototherapeutic keratectomy
Surgical
Giant papillae debulking
Amniotic membrane graft
Topical steroids
Topical antibiotic
Shield ulcer
Cycloplegia
Antiallergy
Lid tightening
Stevens–Johnson syndrome
Madarosis
Features Chemical
Conjunctival scarring Injury
Forniceal shortening Mechanical/thermal
Symblepharon, ankylblepharon
Adenoviral
Infective
Non-Progressive – S III
Neisseria, Chlamydia (trachoma), streptococci A
Exclude infection Surgery
Environment E Radiation
Iatrogenic
Educate patient Topical medications
Brimonidine, pilocarpine especially
3.16 Cicatrizing
Lubrication Conjunctivitis
L Systemic medications
Lubricants, autologous serum, punctal plugs
Lid disease
Blepharitis, allergy, ectropion L
GVHD
Graft versus host disease
Topical steroids History of transplant
Inflammation decrease
I Management AKC
Immunosuppression Atopic keratoconjunctivitis
Lichen planus
Promote healing
Doxycycline decrease MMP P
Progressive Disease
GALLOPs Linear IgA IgA adherence to basement
membrane, not IgG
Symblepharon
Lysis, reconstruct
OCP
Amniotic membrane Ocular cicatricial pemphigoid
Limbal stem cell failure
Treat cause & complications
T Paraneoplastic pemphigoid
Limbal stem cell graft
Cornea
Infection
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NOT seasonal – symptoms all year
Rare, bilateral
General
Blepharitis
Cicatricial changes
Mast cell stabilizers ALL YEAR
Adults Olopatadine
Topical Non-particulate steroids Pain/Keratopathy

Maxidex
Immunomodulatory agents
Symptoms Cyclosporin A 1%
Itch, blepharospasm, photophobia, blur
Shield ulcer
Lid tightening Management Lubricants +/– contact lens
Madarosis Injection
Cicatricial changes Supratarsal corticosteroid
Conjunctival scarring
Forniceal shortening Antihistamines
Symblepharon
3.17 Atopic Systemic Antibiotics for blepharitis
Doxycycline 50–100 mg daily for 6/52
Red, thick, macerated Keratoconjunctivitis
Eyelid Cyclosporin oral
Tightening of skin, chronic blepharitis Severe cases
Cataract surgery
Papillary conjunctivitis
High risk endophthalmitis
Clinical
Tarsal conjunctiva Conjunctival
Scarring & infiltration
Forniceal shortening
Punctate keratopathy
Persistent epithelial defects

Corneal
Plaques
Pannus
Keratoconus
Anterior subcapsular cataracts Associations
Secondary bacterial/HSV infections

Conjunctivitis
Trichiasis/Distichiasis
Early
Bilateral Bullae Blistering Scarring
Shallowing Symblepharon BBBSSS TricK Pro Plica semilunaris & caruncle loss
Trichiasis Keratinization Progressive
Depth <8 mm
Autoimmune mucocutaneous Symblepharon & ankyloblepharon

blistering disease General
Entropion
Age >60 y, female, HLA-DR4 Late
SPK, vascularization, scarring
Signs
IgG & IgA bind to basement membrane Corneal disease
Type 2 hypersensitivity
Complementary/Inflammation
Bullae/Scarring Ocular motility restricted
Opacification
Advanced
3.18 Ocular Cicatricial Cornea Microbial keratitis
Measure fornix depth & adhesions Pemphigoid
Monitor Disease Perforation
Check inflammation & injection
Cataract from treatment

DOCUMENT PROGRESSION and TREAT Complications
Elevated episcleral venous pressure
Glaucoma
Conjunctival fibrosis
Ocular
Conjunctivitis, epiphora
Active inflamed area, ring LAB
Mouth ulcers
Immunohistochemistry
Symptoms Fresh sample
C3, IgG/M/A in BMZ
Esophageal strictures, scarring dysphagia Conjunctival biopsy
Other Investigations
Bullae
Larynx – emergency
Formalin sample
Inflammatory changes around BM
Skin
Biopsy negative OCP

Common
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MAINSTAY OF TREATMENT
1–1.5 mg/kg/d
Remit active disease, then tailor Systemic therapy
General Steroids
Acute
Rheumatologist/Immunologist Also peri- & postoperatively
Refer
50 mg => 100 mg daily
OB/GYN, Gastroenterologist
Dapsone Contraindicated in G6PD deficiency
Mild–moderate
Lubricants Hemolytic anemia

Side effects
+/– Topical steroids Systemic FBE monitoring
All patients Methotrexate
Epilation aberrant lashes Severe
Every visit
Azathioprine/mycophenolate mofetil
Punctal plugs 3.19 Ocular Cicatricial

If not scarred already Cyclophosphamide
Pemphigoid Risk bladder cancer after 12 mo
Oral steroids Treatment
Pre- and postop to control inflammation Severe, rapidly
Rituximab
progressive
Electrolysis
Immunoglobulins
Cryotherapy Lash removal

Plasmaphoresis
Risk increase symblepharon Local
Surgical
Tarsorrhaphy No trabeculectomies
For recurrent epithelial defects
Glaucoma
Entropion repair No proinflammatory medications
Avoid conjunctival incision
Complications
Consider cyclodiode
Limbal stem cell transplant
Clear corneal incision
Buccal membrane grafts
Cataract
Deepen fornices
Steroids perioperatively
Mitomycin 0.04%
If disease localized to small area
NEVER do corneal graft

Cornea
Keratoprosthesis for keratinized surface
Osteo-odonto-keratoprosthesis
Antibioitics
Sulfonamides
Anticonvulsants
Phenytoin, carbamazepine, barbiturates
Oxicam NSAIDs
Systemic skin/mucous Allopurinol

membranes blistering disease Causes
Traditional Chinese medications
10–30 yo, M>F, HLA-B12
HAART
General Highly active antiretroviral therapy
Acute onset
HSV, influenza, mumps, EBV

Type 3 hypersensitivity
Infectious Histoplasmosis
Mortality SJS 5%
Life-threatening
Cat-scratch fever
Mortality TEN 30–50%
3.20 Stevens–Johnson
Syndrome
Daily review
Spectrum of same disease Toxic Epidermal Necrolysis
Lysis conjunctival adhesions

Glass rod
Lubrication
Acute
Desquamating pseudomembranous conjunctivitis
Prevent exposure
Scarring of upper tarsal plate
Topical steroids
Features Local
Treatment Topical antibiotics
Symblepharon Late
+/– Contact lenses
For epithelial defects
Cornea keratization
Corneal graft
Trichiasis
Intensivists
Steroids
Systemic
Immunosuppression IV immunoglobulin
IVIG within 4 d of symptoms
Anti-TNF – infliximab
47
48
Metalloproteinase inhibitor
Doxycycline
Consider in all Unknown cause, 75% male
General
Age 20–40
Systems review for connective tissue disorders
Slit lamp exam General
CWS Bilateral
CT disease Posterior segment Workup Painless, non-inflammatory
Posterior scleritis
Serology/Bloods Epithelium intact
NO ULCER
PUK associated with life-threatening
Refer to rheumatologist
systemic vasculitis
Superior circumferential spread
Terrien Marginal Thinning
Occlusive vasculitis limbal vessels Degeneration Clinical
Yellow-white stromal opacities
Immune complex deposition at limbus Separate from limbus
PMN recruited Steep central, slope peripheral
Proteolytic enzyme release 1. Rheumatoid arthritis General Vascular pannus with lipid, ectasia & melting
2. Wegener's granulomatosis Cause
3.21 Peripheral
3. Relapsing poly, SLE, PAN, UC, rosacea Astigmatism
Corneal Gas permeable scleral contact lens
Serious morbidity/mortality associated with above Treatment
Thinning
Perforation
Painful, unilateral Corneal graft
Crescent shaped & stromal infiltration at limbus
Unkown cause
Scleral involvement Clinical
General
Bilateral, rapidly progressive,
Circumferential spread diagnosis of exclusion
Risk perforation
PUK Chronic, progressive, painful
Mainstay, prevents death
Unilateral 70%
Steroids
Cyclophosphamide SYSTEMIC Epithelial defect always present
Wegener's Mooren Ulcer Clinical
Methotrexate Immunosuppression Ulcer Starts periphery, circumferentially
Cyclosporin Then centripetally
Treat Sjögren’s & epithelial healing
Lubrication & tarsorrhaphy Treatment Overhanging central edge
Reduce stromal melt Inflamed eye
Oral doxycycline
Perforation
Corneal glue/tectonic graft No scleral involvement
PK or DALK during active inflammation Life-threatening systemic causes (PUK)

Exclude
Topical corticosteroids Treatment Bloods
Delay epithelialization Avoid RF, cANCA, pANCA, dsDNA, ANA
Conjunctival grafts As for PUK
Inflammatory vessels brought to cornea Systemic immunosuppression Cyclosporin,
cyclophosphamide
Pellucid Once inflammation settles
Inferior, bilateral thinning, quiet eye
Reconstructive PUK/Mooren's
Painless corneal thinning Surgery Immunosuppression to prevent recurrence
Others
Furrow degeneration
Non-progressive, Peripheral to arcus senilis Terrien's Crescent shaped lamellar or full
no treatment required
Exposure keratopathy thickness corneoscleral
Neurotrophic keratopathy
Involves Bowman's layer
Uveitis
Chronic ocular disease

Severe keratitis, corneal edema, glaucoma
Idiopathic Phthisis bulbi

Hypercalcemia Hyperparathyroid, vitamin D

Causes toxicity, sarcoid
Elastotic degeneration
Elevated, with normal calcium
Pathology Phosphorous
Sun/ultraviolet
In renal failure
Atrophy, hyperplasia, metaplasia, dysplasia Silicon oil
Visual axis affected Calcific Band

Aphakic eye
Keratopathy
Astigmatism
Ocular examination
Inflammation Calcium, albumin, magnesium phosphate
Control with steroids before surgery
Indications Serum electrolytes
Ocular motility restricted Blood urea, nitrogen, creatinine
for surgery
3.22 Degenerations –
Pterygium, Vortex, Uric acid
Unilateral, papillomatous or leukoplakic
Neoplastic features Band Keratopathy Lubricants
Mild
Vascularized Management
Not wing shape, or 3/9 o'clock Pterygium Alcohol + EDTA - chelates calcium,
Cosmesis removes from stroma
Calcium removal
Bare sclera closure
60–70% recur 3% EDTA
Severe
5–15% recur
6/52–3/12 topical steroids Phototherapeutic keratectomy
Not in glaucoma Conjunctival autograft Lamellar keratoplasty

patients
Procedure of choice
Need superior
Not an indication to stop medication
conjunctiva Surgery
10–15% recur Fabry disease
Risk steroid Radiotherapy
response Vortex
10% have necrotizing scleritis Choroquine/hydroxychloroquine
Keratopathy
Within one year commonly Amiodarone
FCANT
Consider mitomycin Recurrent pterygia NSAIDs – ketorolac, indomethacin
Consider medial rectus hooking Tamoxifen

49
50
Map-dot-fingerprint
Epithelial basement membrane Cogan microcystic epithelial

aka
Marilyn Monroe Always
Macular Mucopolysaccharide Alcian blue Anterior basement membrane
Meesmann
Gets Her Man in Mnemonic
Mutation in keratin genes
Granular Hyaline Masson stain Epithelial/
Subepithelial Epithelial recurrent erosion
L A City
Lattice Amyloid Congo red
Subepithelial mucinous
Progressive Lisch epithelial

3.23 Corneal
Dystrophy (IC3D
Bilateral Classification) Gelatinous drop-like
Non-inflammatory opacification
Reis–Bucklers
Reis–Buckler/Thiel–Behnke Bowman’s Layer

Thiel–Behnke
Granular 5q31 (TGFBI)
Grayson–Wilbrandt
Lattice Features
Genetic
Avellino Macular
16q22
Classic type
Macular
Lattice
Gelsolin type
PPD
20p11
CHED Classic
Present 10–40 yo Granular Granular–lattice
Visual loss Reis–Bucklers

Stromal
TGFBI
Recurrent corneal erosion syndrome
Schnyder
Fuchs endothelial
Congenital stromal
Posterior polymorphous Fleck

Descemet’s Membrane
Congenital hereditary Posterior amorphous
Type 1 & Endothelial
endothelial dystrophy
Type 2 Central cloudy dystrophy of Francois
X-linked endothelial
Pre-Descemet
Soft shell
GAGs excrescences deposit on Descemet's
Balanced salt solution plus
Endothelial decompensation
Guttata Glutathione may protect
Corneal edema endothelial cells
Age >50 y, AD, Bullae affects vision Cataract surgery
Features Mild Fuchs + moderate cataract Endocapsuar phaco
Symptoms worse in a.m.

Chopping
Surgery
Decreased surface evaporation during sleep
High frequency topical steroids
Pseudophakic bullous keratopathy
Anti-IOP meds
Alternative causes
Aphakic bullous keratopathy Moderate Fuchs + moderate cataract
3.24 Fuch's Cataract surgery + DSAEK
Symptoms Endothelial
Cataract surgery + DSAEK
Cornea not clearing by midday Dystrophy Topical steroids will form cataract early
Advanced Fuchs + mild cataract Cataract once all sutures out
Appearance
Corneal haze Penetrating keratoplasty
Correct refractive error with cataract surgery
Severity
Pachymetry
Central corneal thickness >650 µm Cataract surgery + DSAEK
Polymegathism & pleomorphism Advanced Fuchs + advanced cataract

PK + ECCE/Phaco + IOL
Chickenwire appearance Specular microscopy Triple procedure
In very poor view
Endothelial cell count <700 =>
Descemet's stripping automated endothelial keratoplasty
Corneal surgery
Relieve edema & pain
No sutures, faster recovery
Lubricants
Advantage Increased tectonic sutures
Ruptured bullae => antibiotics Acute
Decreased glaucoma, suprachoroidal hemorrhage, refractive change
DSAEK
Management
Interface haze, stromal scar, poorer VA
Sodium chloride 5%
Disadvantage Endothelial loss earlier
Debatable
Decompensation degree Cannot fix astigmatism

Surgery factors
Lens status Long-term
Uncommon in phakic patients
Phakic, pseudophakic, cataract
Damage to endothelium in future cataract surgery
Visual potential of eye
51
52
>40 µm abnormal
Raised central area on posterior float
Bilateral, asymmetrical, para/axial thinning If only this sign => FORME FRUSTE KC
>55 = moderate
Early adolescence to mid-20s General Central corneal power >47 D
AKC, VKC, retinitis pigmentosa
>65 => surgery
Ocular
Leber's congenital amaurosis Diagnosis Inferior steepening >1.2 D
Associated with Topography
Down syndrome Thinning of cornea
Systemic
Marfan syndrome THIN = STEEP
Apex of cone at thinnest point of cornea
c.f. pellucid
Stretching, thinning stroma Group of disorders
Altered shape Irregular astigmatism
Non-orthogonal by >20 degrees
Bilateral, present at birth Keratoconus 3.25 Keratoconus &
Associated with Ehlers–Danlos
Other Ectasias Hypertonic 5% saline QID
Global thinning Topical steroids

Perforates with minimal trauma Keratoglobus Especially limbal vascularization
Hydrops Lubricant
Lamellar graft/full thickness Wait 3/12 before surgery, VA may improve
50% require transplant
Bilateral inferior corneal thinning Primary Keratectasia Earlier if perforation
Kissing birds/crab's claw
Topography Atopy, Down's
Steepest part above thinnest part Look & treat systemic
Pellucid marginal
degeneration Initial Stop eye rubbing
As per keratoconus Glasses + hard contact lenses
Larger grafts – more rejection Management Monitor 4/12 for progression Riboflavin + UVA light
Lamellar tectonic grafts/
Lamellar tuck-in grafts Need CCT > 400 µm
Trauma/Infective/Inflammatory Progressive disease
Secondary Collagen cross-linking Reduce astigmatism, halt
e.g. keratoglobus secondary to congenital glaucoma progression for >3 y
Glaucoma Management
Controversial Intolerant of contact lenses

Fleischer ring
Iron deposits at base of cone
Intracorneal ring segments Young, VA 6/18–6/36
Unilateral disease
Vogt's striae
Fine parallel vertical lines of stress
2–3 D flattening
Munson's sign
Protrusion of lower eyelid on downgaze e.g. deep anterior lamellar keratoplasty if no scar
Oil droplet Lamellar Tectonically stronger, less rejection, no

On direct ophthalmoscope Clinical eye penetration
Graft
Surgery
Scissoring reflex VA outcome less than PK
Retinoscopy If scar
Full thickness penetrating keratoplasty
Breaks in Descemet’s membrane => subsequent scarring
Intolerance
50% require graft
Hydrops Contact lens Poor vision
Wait 3 mo at least
50% resolve, improve Unstable fit
Vision may improve Graft indications
Need larger graft =>
Progressive thinning higher failure rate
Cornea flattening due to scarring approaching limbus
Prior to examination
AT LEAST 2 L or 1/2 h
Immediate copious irrigation Until pH normalizes
Emergency
Sweep conjunctival fornices
Analgesia
History Particulate material

Chemical
Grade burn
+ Retinopathy
Raise pH of tissue
Low – perforation
Saponification of cell membrane fatty acids Check IOP
General High – inflammation
Cell disruption Oral diamox (no topicals)
Destroys epithelium, stroma, Alkali Acid vs. Alkali Manual debridement
limbal ischemia
Retinopathy, retinal necrosis Exclude infection
Severe Daily review Exclude melting
Phthisis
Symblepharon
Less penetration due to Denature & precipitate proteins Acid Divide daily
precipitate barrier Topical antibioitics
Corneal epithelial defect Grade 1 & 2 Lubricants – non-preserved

2 hourly
Grade 1
No limbal ischemia Cycloplegia
Homatropine/Atropine
Hazy cornea but iris details visible
Grade 2 Management As per Grade 1 & 2 2 hourly, non-preserved or Maxidex
<1/3 limbal ischemia
Roper Hall
(Modified Hughes) No prednefrin forte – has phenylephrine
Total loss corneal epithelium Topical steroids
Classification Stop after 2/52 to prevent stromal melt
Stromal haze obscuring iris details Grade 3
Grade 3 & 4 Topical sodium ascorbate 10% 2 hourly
1/3–1/2 limbal ischemia Wound healing
Opaque cornea 3.26 Chemical Topical sodium citrate 10% 2 hourly
Grade 4 Inhibit neutrophil activity
>1/2 limbal ischemia Injury Collagenase inhibitors
Oral ascorbate (vitamin C) 500 mg–2 g po QID
Corneal abrasion & perforation Doxycycline 100 mg po bd
Infection Acute Cyanoacrylate glue

Perforation
Glaucoma Patch corneal graft
Trichiasis, dystychiasis, entropion
Cicatricial conjunctivitis, dry eyes Bandage contact lens + preservative free antibioitics
Ocular surface
Ankyloblepharon, symblepharon Temporary lateral tarsorrhaphy
Complications Non-healing at 2/52
Persistent epithelial defect Amniotic membrane grafting
Complications Autologous
Limbal stem cell transplantation
Cornea Chronic Allogenic
Conjunctivization of cornea
Stromal scar Avoid 12–18 mo
Glaucoma Treat limbal stem cell failure first
Trabecular damage iritis Intraocular Long-term
Guarded prognosis – limbal ischemia,
Cataract Keratoprosthesis cicatricial changes, aqueous deficiency
53
54
History
Assess severity & extent
Exclude IOFB
Acute
Remove
Endophthalmitis
Principles of IOFB present
Management Toxic, organic, vitreous hemorrhage,
Delayed presentation, remove if:
retinal damage
Iron/Copper/Organic – urgent removal
Exclude infection
Topical/systemic/intravitreal antibioitics
Restore globe integrity then vision
3.27 Perforating Manage secondary complications

Exposed uvea, vitreous, retina e.g. glaucoma, cataract, retinal detachment
Injury
Positive Seidel test Diagnostic
Shield
IOFB visualized/On x-ray/Ultrasound
Avoid topical
Deep eyelid laceration IV antibiotics – cefazolin 1g TDS
Immediate IV fluoroquinolones – soil contaminated FB
Orbital chemosis Management
External
Motility limitation Nil by mouth
Analgesia/antiemetics
Conjunctival laceration/hemorrhage
Shallow AC/Hypotony Tetanus

Anterior chamber Signs of IOFB
Macrohyphema (Intraocular
Suggestive Foreign Body)
Transillumination General anesthetic
Focal iris–corneal adhesion Iris Corneal/Limbal – 10-0 Nylon

Primary Wound closure
Peaked/irregular pupil Intraocular Surgical Repair Sclera – 9-0 Nylon
Capsule defect
Lens Intravitreal vancomycin/
Opacity Exposed vitreous
ceftazidime
Retina
Tear/Hemorrhage
Prevent infection
As potentially high-speed Do CT scan IV antibioitics 3–5 d, topical 7 d
Postoperative
Orbit AND brain Management Suppress inflammation
Prednefrin forte QID
Topical cycloplegia
For stromal opacities Average = 9 y
Graft Survival
Anterior lamellar Keratoconus/Fuchs = 17–19 y
Closed eye, maintain host endothelium keratoplasty – e.g.
deep anterior lamellar Infection/Tectonic = 4 y
Tectonic strength
keratoplasty
Viscoelastic
Interface haze, epithelial ingrowth, suture problems like PK
Suprachoroidal hemorrhage
For endothelial dysfunction
Steroid PK changes angle anatomy
High
Descemet's stripping
Closed eye, no suture Glaucoma Thicker cornea – IOP measurement less accurate
=> vision better earlier automated endothelial
keratoplasty IOP High IOP => graft failure rate higher
Tectonic strength
Wound leak/dehiscence
? More endothelial loss, need pseudophakic Types Seidel +ve, AC flat
Early Low
Flat chamber/iris incarceration
For pancorneal dysfunction/scar
? Better VA at end Failure – primary graft Cornea never clear after graft
Penetrating keratoplasty –
full thickness
3.28 Corneal Re-operate 7/52 at-least
Open eye, low tactile strength Rejection
Transplant
Sutures 12–18 mo Epithelial defect persistent
Endophthalmitis Tight/loose
4–5D astigmatism, prolonged recovery Complications
Early "I'm free" Later
Abscess/non-infectious infiltrate
"I'm scared to fail"
Early (1–12 mo),
Epithelial Suture related problems Vascularization
donor epithelium
Mucus on sutures
Infiltrates – Krachmer spots
Subepithelial Microbial keratitis/infectious
Mild AC reaction
crystalline keratopathy
Cataract
Most common, occurs in 8–37%
Late Hard contact lens
Symptoms
Photophobia, halo, red
Suture removal
Graft Rejection Astigmatism
Clump KPs Astigmatic keratotomy
Linear = Khodadoust line Signs
Endothelial PRK
AC reaction Rejection Re-graft
Non-function endothelium
Corneal edema
Disease recurrence
Topical corticosteroid
Every 15/60–1/24, day & night Failure – non-immune endothelial
Treatment
Recurrent, severe, Consider systemic steroids 1 mg/kg Unreliable IOP, disc, OCT, HVF
recalcitrant
Glaucoma Difficult Thick cornea, distorted view
Because
Rejection has no hypopyon Inflammation + steroids postoperatively +/– Peripheral anterior
Rejection vs. infection
synechiae formation
Rejection – KPs/corneal edema on graft tissue only Angle distortion
Pre-existing glaucoma
55
4 Glaucoma
Glaucoma
4.1 Glaucomatous Optic Neuropathy – Risk Factors 4.8 POAG Suspect & Normotension Glaucoma
Open Angle Glaucoma 4.9 Pseudoexfoliation Syndrome
4.2 Open Angle Glaucoma Classification 4.10 Pigment Dispersion Syndrome
4.3 Closed Angle Glaucoma 4.11 Uveitic Glaucoma
12. Lens Glaucoma
4.4 Disc Assessment – 6 Rs Open & Closed

Examination
4.5 Gonioscopy
4.13 Angle Closure
4.6 Visual Fields & Disc Imaging
4.14 Acute Angle Closure
Angle Closure Glaucoma
4.15 Primary Angle Closure
4.7 Target IOP
4.16 Plateau Iris
4.17 Secondary ACG – No Pupil Block
4.20 Early Postoperative

4.19 Glaucoma
Trabeculectomy Complications
4.18 Treatment Operations
4.21 Immunosuppressants
4.22 Glaucoma Studies
57
58
Raised IOP
Fluctuates – higher in morning
Age
Primary open
angle glaucoma Family history
Race
African origin 3–4 x > Caucasian
Decreased central corneal thickness
Age
Decreased AC depth & fluid
Family history
Primary angle
closure glaucoma Female 3–4 x > male
Risk Factors
Optic neuropathy & associated visual field loss Definition Hyperopia
Race Asian
IOP primary risk factor
V Migraine, Raynaud’s
Vasospasm
4.1 Glaucomatous Optic A

Autoimmune
Neuropathy – Risk Factors Normotension
No ocular/systemic disorder
Primary glaucoma N
Nocturnal hypotension
Often bilateral
Ocular/systemic disorder Cause C

Cardiovascular
Classification
Often unilateral Secondary
O
Obstructive sleep apnea
Increase outflow resistance
Angle Notching-excavation
Open angle glaucoma
(trabecular meshwork)
Not required Vertical rim thinning
Closed angle glaucoma
RNFL hemorrhage
CNS Visual acuity decreased

Imaging
Colour vision
Affected late in glaucoma
Required Pallor of rim
Non-vertical rim thinning
Atypical visual field

Neovascular glaucoma
Pretrabecular –
Glaucoma
ICE – iridocorneal endothelial syndrome

membrane overgrowth
Inflammatory membrane
Fuch's heterochromic iridocyclitis
PXF – pseudoexfoliation
PDG – pigmentary dispersion glaucoma
Trabecular – Uveitic inflammatory cells

drainage
Lens particle glaucoma
Liquefaction lens cortex
Lens-induced
Phacolytic
Liquefied lens => macrophage => block
Ghost cell glaucoma
Hyphema & angle recession Angle recession proxy for angle

trauma & flbrosis
Raised IOP Glaucoma Suspect Lens Phacoanaphylactic

Secondary Eosinophil hypersensitivity to lens antigen
OR suspicious optic disc/visual field Surgical trauma

Trabecular – ACIOL, silicon oil
structure Steroids
4.2 Open Angle HSV, VZV

Glaucoma Trabeculitis
Posner–Schlossman syndrome
Neurofibromatosis
Other Axenfeld–Rieger
Normal tension glaucoma Primary Dysgenesis
Aniridia
Raised IOP glaucoma Carotid-dural flstula
AV malformations Orbital varix
Raised episcleral Sturge–Weber syndrome

Post-trabecular venous pressure
Normal = 8–10 mmHg
Tetrobulbar tumor
Venous obstruction
Thyroid eye disease
Superior vena cava syndrome

59
60 Mindmaps in Ophthalmology
Phacomorphic
Lens Ectopia lentis
With pupil block Aphakic/Pseudophakic Vitreous blocking pupil/
iridectomy
Seclusio pupillae
Iridocorneal endothelial syndrome

Pull
Secondary Inflammation => peripheral anterior
Angle Closure synechiae (PAS)
Choroidal hemorrhage/effusion
Posterior segment Tumor
Without pupil block
Surgery – gas/oil
Push Malignant glaucoma Ciliary body configuration => aqueous
4.3 Closed Angle misdirection anteriorly
Glaucoma Topiramate
Drugs
Doxycycline
PAC suspect
Primary Angle ITC (iridotrabecular contact) 180 degrees only
Closure (PAC) ITC (180 degrees) + raised IOP/
PAC
PAS/Typical symptoms
PAC glaucoma
ITC (180 degrees) + Disc/Field changes
Plateau iris
Continued ITC after iridotomy
Ring – Scleral Size/orientation/tilt of disc

Thinning, notches, color
Rim – Neuroretinal Inferior > Superior > Nasal > Temporal
Normal thickness
ISNT Rule
Color Rim color preserved in glaucoma
c.f. Non-arteritic ischemic optic
neuropathy => pale rim
Document
Ratio Cup:Disc
5% population >0.6
<1% population asymmetric CDR >0.2

4.4 Disc
Assessment 6 Rs Glaucoma until proven otherwise
Rim Hemorrhage Drance hemorrhage
Indicates progression
6 mo to resolve
Retinal Nerve Fiber Layer
Correlated with rim
Region of Peripapillary Atrophy Beta variety

Sclera visible
Not alpha variety

Iris directly touching trabecular meshwork
Glaucoma
Iris
Irregular pigment deposition over angle

Ciliary body e.g. pseudoexfoliation
Iridotrabecular syndrome
Contact
Scleral spur
Anatomy: Iris insertion anterior to scleral spur
I can see the stuff
Trabecular meshwork Peripheral anterior synechiae
Schwalbe's line
4.5 Gonioscopy
Iridotrabecular contact
Yes – grade & record

Pigmentation Exfoliation syndrome Line of pigment deposit
Open angle Sampolesi's line
anterior to Schwalbe's line
Yes – grade & record Scleral

PAC – synechiae Spur Visible?
Pigment dispersion syndrome
No – indented
Yes – grade & record gonio – any peripheral
anterior synechiae?
PAC – appositional Blood in
No – IOP raised? Raised episcleral venous pressure
Schlemm's Canal
e.g. Sturge–Weber, carotid–
No – grade & record cavernous fistula
PAC suspect
Radial iris vessels
Normal
Wide ciliary body band
Portions of arterial circle of ciliary body
Prominent scleral spur

Blood
Trunk-like vessels Vessels Trauma – Angle
Cross-ciliary body & scleral spur Recession
Torn iris processes
Fine branching over trabecular meshwork Abnormal

Variation in angle depth in
different quadrants
PAS formation
Contraction of myofibroblasts with new vessels

61
62
Progression in appearance Disc Photos
Generalized depression
Full-threshold
Paracentral scotoma
Humphrey's Loss within 10 degrees of fixation
SITA - Swedish Interactive
Threshold Algorithm Arcuatescotoma
Glaucomatous
Field Nasal step
Modalities Relative depression of one hemifield
SWAP – short wavelength
automated perimetry Pattern of Altitudinal defect
VF Loss
FDT – frequency doubling technology Temporal wedge
CFF - critical fusion frequency Temporal > nasal field defect

4.6 Visual Fields & Vertical meridian
Non-Glaucomatous –
Disc Imaging Investigate CNS Hemi/quadrantanopia
Not validated in studies
Central/paracentral scotoma
Automatic detection optic Strength Optical VF defect with normal nerve

nerve borders Coherence
Tomography
Outside normal limits
e.g. with PPA Optic disc margin Weakness Glaucoma Hemifield test At least 2 consecutive occasions
inaccurate
Depressed at P<0.5%
Humphrey's Pattern deviation
Cluster >=3 non-edge points
Glaucoma Criteria At least 1 at P<1%
RNFL thickness GDx (Anderson's Criteria)
Pattern standard deviation

<5% of normals on 2 consecutive occasions
Cup
Deepening of existing scotoma
Neuroretinal rim Confocal laser
Humphrey's Enlargement of existing scotoma
RNFL Progression Criteria
Heidelberg
Retinal Development of new scotoma
Strength Tomogram
Normative data & algorithm
Repeatable
User places contour line
Weakness
Overestimate rim
Aim
Monitor, no treatment
Older age
Glaucoma
Low Risk
OHT
Ocular hypertension
PACS
Primary angle closure suspect
Features
Pigment dispersion syndrome
with normal IOP
Suspicious disc
Family history of glaucoma
4.7 Target IOP 30% reduction

Glaucoma
Slow or halt disease progression Or population mean – whichever lower
Aim Monitor or treat

Glaucoma/nonglaucomatous neuropathy Glaucoma Patient preference & risk factors
suspect
Open/Closed 20% reduction or 1 SD above
population mean
Whichever lower
Primary/Secondary Type
Stage of disease Moderate Mild GON, early VF loss, higher IOP

Rationale
Risk
Moderate GON, low IOP
Optic disc Glaucoma
PAC, high IOP, PAS
Severity
Visual field
Determined Young
Anderson's criteria from “TSPP”
Rate Features GON in fellow eye
IOP Glaucoma OHT + risk factors
Central corneal thickness Progression
suspect
PXF
Cup–disc ratio
Disc hemorrhages
Pattern standard deviation
Glaucoma mutations
>40% reduction
Fellow eye Aim
Or 1–2 SD below population mean
Patient factors e.g. 9–12 mmHg
Life expectancy
Young, advanced, high IOP
High Risk
Comorbidities Fast progression
Features Visual field Bilateral loss

Advanced loss
Secondary glaucoma
e.g. PXF, PDS
63
64
POAG with IOP <21 mmHg IOP & glaucomatous optic neuropathy
Normotension
24% of POAG IOP <24 & no GON
(Baltimore Eye Study) No treatment
Treatment of
Definition Assess risk factors
Suspect
Optic nerve or NFL suggestive of glaucoma +/– Treat
IOP 24–30 & no GON
Consider 1 eye trial
VF abnormality consistent with glaucoma
One of
Suspect IOP >30 No progression
Elevated IOP >22 mmHg 4.8 POAG Treat Consider stopping medication
Suspect &
If more than one => POAG Normotension
Glaucoma Ocular Hypertension Treatment Study
Migraine, Raynaud's Vasospasm
phenomenon history Treatment vs IOP 24–32
no treatment in suspects
Hypotension Aim Target IOP <20%
Systemic or nocturnal
Etiology 5 y follow-up
Microischemic disease, obstructive
sleep apnea, autoimmune disease High IOP
Age
Steroids, trauma, GCA history
Large CDR
Normotension OHTS Risk factors found
Water phasing, 24 h BP Investigations
Reduced CCT
monitor, neuroimaging Independent risk factor
Increased PSD
CNTGS 40% untreated => no Observe
progression at 5 y Not a risk factor
Family history & myopia
Treatment
Aim >30% reduction Treatment
35% people with IOP>26 & CCT<555 develop POAG
GP Important clinical
BP, cholesterol Treat implications CCT
Every <40 µm from median 555 µm Increase
conversion by 40%
Disc hemorrhage present
Amount of IOP reduction
Every <1 mmHg Reduce risk
by 10%
Glaucoma
Pigment line anterior Sampaolesi's line

to Schwalbe's line
Angle
Deeply pigmented
Clinical
Open angle
Peripupillary atrophy, transillumination defects
Fibrillary material blocking TM
Glaucoma
Iris
Poor pupil dilation
EMGT study
10–fold risk developing glaucoma
Irido & phacodonesis
4.9 Pseudoexfoliation Medical

Topical IOP medications +/– pilocarpine
Syndrome
Good response
to SLT Angles pigmented
Laser
Systemic condition
Insufficient data as yet
Floppy iris & poor dilation

Lens capsule, zonules, ciliary Deposit of material Pathology Treatment
processes, iris stroma, angle
Cataract Weak zonules
surgery
Associated with Postoperative inflammation & IOP spike
Homocysteinurea Surgical
IOL subluxation
Aim larger rhexis
Trabeculectomy
Tube surgery
65
66
Up to 50 mmHg
IOP
Exercise/pupil dilation
Vertical deposition on
corneal endothelium
Krukenberg spindle
Cornea Flow from PC to AC via pupil
TM
Uniform pigment
Young Caucasian myopic males General

Clinical Angle Queer iris insertion
Bowing backwards
15% risk developing OAG
Wide open angle, prominent iris processes
c.f. steep in plateau iris
Iris
Midperipheral transillumination defect
4.10 Pigment
Dispersion Syndrome Pigment on lens periphery
Lens Pigment on posterior capsule

Scheie's or
"Queer" – "Reverse pupil block" Zentamayer's line
Weigert's ligament Anterior vitreous attachment to
Posterior iris bowing posterior capsule
Iris Insertion Into

Pigment release Ciliary Body
IOP medications
Zonule abrade
peripheral iris Medical +/– Pilocarpine
TM blockage Straightens curvature of iris
IOP spike
Selective laser trabeculoplasty
Treatment Works well
Laser
Controversial, alters reverse pupil block

Peripheral YAG-laser iridotomy
One eye at a time

Surgery
Glaucoma
Young, middle-aged, male >female
White eye
Mild AC reaction
Photophobia ++
Features
High IOP – 40–50

Corneal edema
Posner-Schlossman
CMV etiology possible
Investigations
AC tap + PCR
Topical steroids
IOP medications
Treatment
Topical +/– oral
Chronic
Consider val/ganciclovir
HSV/VZV
4.11 Uveitic
Glaucoma
Toxoplasmosis, toxocara
Idiopathic, unilateral, middle-aged, male = female
Fuch's heterochromic iridocyclitis Acute

Iris heterochromia
Hypochromia
Posner–Schlossman
Mild AC reaction + stellate KPs
Endogenous endophthalmitis Cause
PSCC
Features Posterior subcapsular cataract
PAS formation Paracentesis in

Fuch's Heterochromic
Any uveitis Fine vessels crossing TM cataract surgery
Chronic Iridocyclitis Amsler sign
Progressive angle closure
AC bleed
Secondary OAG
Topical steroids
Acute
Hourly at start No steroids
Treatment Worsens PSCC, no effect on
Treatment inflammation
IOP medications
Glaucoma
Topical +/– oral
Difficult
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68
Mature/hypermature lens + intact capsule
Pathology Lens protein leakage
Protein/macrophages/debris
Clog TM
Elderly, poor vision, sudden pain

Phacolytic
White intumescent lens =>
Clinical Material in AC
Associated high IOP & uveitis signs Cells, flare, KPs,

corneal edema
IOP – topical, oral, IV mannitol as required
Treatment Steroids
Open
Cataract surgery
In eye not predisposed 24–48 hours if visual potential
to closure
Pupil block Marked lens swelling
– intumescence
Exclude endophthalmitis if hypopyon
c.f. hyperopes/Asians
4.12 Lens Glaucoma –
Open & Closed Lens particle Pathology
Disparity in AC depth between eyes glaucoma Trauma/Surgery => lens material blocks TM
Phacomorphic
Gonioscopy Phacomorphic vs PACG
White, fluffy cortical material in AC + high IOP
Significant cataract
Clinical
Laser iridotomy AC cells, flare, corneal edema
Treatment
Eosinophil reaction, not
Cataract extraction once quiet
true anaphylaxis
Closed
Pupil block +/–vitreous Anterior lens Secondary ACG Pathology
displacement with Pupil Block
Sensitized to own lens protein Autoimmune
Iris bombe, AC shallowing granulomatous
Phacoanaphylactic reaction
Ectopia lentis
Causes
Marfan syndrome, trauma, Ehlers–Danlos
Granulomatous uveitis
Clinical
Lensectomy + anterior vitrectomy – Treatment
prevent vitreous block IOP usually normal
Aphakia or Lens dislocation or subluxation

Vitreous blocking pupil/iridectomy pseudophakic angle
closure glaucoma
Glaucoma
Iris pushed forward

Tend to have pupil block
2 Mechanisms
ICE, rubeosis, uveitis with PAS,
choroidal effusions
Iris pulled forward
No pupil block
4.13 Angle Closure

Glaucoma
Most frequent cause
Apposition of peripheral iris to TM
Peripheral iris bows forward
Pressure gradient
Resultant reduced drainage through angle TM blocked

Flow from PC to AC impeded
No underlying pathology Definition Pupil Block
Primary Relative block Iris contact with IOL, capsule,
Anatomic predisposition vitreous, oil etc.
Underlying pathology
Patent PI & ongoing NOT pupil block
Secondary
Initiates closure Definitive treatment = glaucoma =>
peripheral iridotomy
Look for other cause
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70
Topical IOP + steroids
IV or oral acetazolamide
Medical
1–2 g/kg IV over 45 min
IV Mannitol
General
e.g. 500 mL of 20% mannitol
Peripheral iridotomy
Pupil block mechanism
Laser
Argon peripheral iridoplasty
Both for AACG &
plateau pris
An acute process
Bombe iris Laser YAG PI
Primary or secondary General No improvement => consider plateau iris
Phacomorphic
TM blockage by iris => acute IOP rise Pilocarpine & laser YAG PI
Pupil block
No pilocarpine
Pain, headache, nausea & vomiting Specific
Symptoms
Blurred vision, haloes Consider mydriatic
Treatment Aphakic/pseudophakic IOL/vitreous causing pupil block
High IOP
Clinical Lens repositioning
Shallow AC Surgery
Vitrectomy
No pilocarpine
Mid-dilated sluggish pupil Signs Seclusio pupillae
Laser YAG PI
Corneal edema 4.14 Acute Angle
Closure Non-pupil block
Ciliary flush, mild AC flare & cells Secondary causes, no bombe
Definitive diagnosis Gonioscopy Intracameral bevacizumab + PRP
Uveitis Steroids
Work out appositional or PAS Secondary
cause
Phacomorphic glaucoma
Cataract surgery when eye quiet
40–80% develop AACG in fellow
eye in 5–10 y Plateau iris
Bilateral disease Follow-up Argon peripheral iridoplasty
=> Treat other eye – miotics until laser PI Medications
Cease topiramate, sulfonamide
Examine angle
Chronic
Confirm opening of part of angle Goniosynechiolysis if <6/12 duration
Other Trabeculectomy – failure in hot eye
Controversial
AC paracentesis – shallow AC
Glaucoma
ITC (iridotrabecular contact) only

Normal HVF, IOP, No PAS, no symptoms
1. PAC
Suspect i.e. posterior TM visible <90 degrees
Grade II for <90 degrees
Occludable angles
Scleral spur visible on indentation only
Leading cause of glaucoma Arbitrary definition
90% of cases
ITC + IOP/PAS/typical symptoms
Pressure differential => iris bows anteriorly Relative General
pupil block 2. PAC 2 quadrants no scleral spur
Iris blocks TM
Iris blocks TM => ITC
Angle closure No GON
Prolonged apposition ITC => PAS formation
Dynamic gonioscopy
4.15 Primary PAS
Appositional OR PAS Angle
Gonioscopy
Closure Creeping angle closure
Differentiates suspect vs actual closure Appositional progresses to synechial
Risk Factors 3. PAC ITC + Disc/Field changes

Middle-aged Asian female with glasses Glaucoma
(hypermetropic) with blind mother
If cataract Only treats pupil block

Cataract surgery
Treatment PAC
If clear lens Pupil block significant
Laser YAG PI
PACG
PI may not prevent progression Peripheral Pupil block significant
Iridectomy
Follow-up Indications PACS (absolute) + PAC/G in other eye
Need repeated dilated fundus exam
PACS (relative) Poor access to ophthalmic care
Family history of PACG

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72
Pushes peripheral iris forward

Anterior ciliary processes =>
Critically narrow AC recess

Central
Flat iris plane, normal AC depth
Indent, check no PAS Iris
Cannot diagnose by General
gonioscopy Sharp convex iris
Peripheral
Clinical Steep angle of insertion
Does not work =>

Ultrasound biomicroscope
Do PI =>
Investigations
High IOP/angle opposed => Anterior segment OCT
Plateau iris Young myopes
Anatomy predisposes
4.16 Plateau Iris
Contracts peripheral iris
Mild pupil block => Plateau iris
acute angle closure configuration Patent PI present
=> Likely type of PAC
Peripheral argon Raised IOP
Treatment iridoplasty Dilation
Laser YAG PI Treatment
Types Angle closure
Indications
PAS developing
Peripheral iris closes angle
Anteriorly placed ciliary body Plateau iris appearance +

Plateau iris
glaucomatous optic neuropathy
Without pupil block syndrome
Dilation can cause AACG

Diagnose after Laser YAG PI
Proliferative diabetic retinopathy
Causes Central retinal vein occlusion

Glaucoma
Carotid occlusive disease

Common
Early
Topiramate, doxycycline NV blocks TM
Neovascular
Zonule relaxation Pathogenesis
Glaucoma
Ciliochoroidal effusion Contraction of fibrovascular neovascularization
Anterior displacement iris–lens complex Late
Mechanism PAS
Secondary ACG & high myopia Drugs Underlying cause
PRP, cryotherapy if unclear media
No carbonic anhydrase inhibitors
Visual potential Intracameral bevacizumab
Worsen ciliary body effusion Treatment
Cease medication
Treatment Filtration surgery – tubes
IV mannitol & atropine Topical steroids + IOP medications

Rotates ciliary body posteriorly
No visual potential
4.17 Secondary Cyclodiode Risk phthisis bulbi & sympathetic
Unilateral, F>M, 20–50 ophthalmia
Angle Closure –
Growth over TM No Pupil Block
Secondary ACG Pathogenesis Aqueous misdirection – anterior
Iris pulled anteriorly Anterior configuration of ciliary body
Abnormal endothelium Pathogenesis
Shallow central & peripheral AC
Corneal edema
Often postsurgery/laser
Contracting endothelial Cogan–Reese
membrane Trial PI
Iris naevus
Malignant Tighten iris–lens diaphragm
Beaten bronze endothelium Atropine & phenylephrine
Features "ICE" Glaucoma
Iridocorneal PI ineffective
Chandler's syndrome Induce ciliary body rotation
Endothelial
Microcystic corneal edema, IOP normal IOP medications
Syndrome
Treatment
Essential iris atrophy
Atrophy, corectopia,
pseudopolycoria, ectropion uvae, PAS Aphakic/pseudophakic YAG laser to posterior
hyaloid face
Surgical intervention Phakic
Unilateral angle closure glaucoma
YAG through existing PI
Diagnosis
Specular microscopy Vitrectomy
Asymmetric loss, dark–light
reversal of epithelial cells
Choroidal hemorrhage
Anterior displacement of ciliary body
IOP medications Tumor
Posterior
Segment
Hypertonic saline for corneal edema Laser surgery, buckle, central
Choroidal swelling
Treatment retinal vein occlusion
+/– YAG sclerostomy to open membrane
Surgery
Filtering surgery
Gas, oil
Tube – place anteriorly
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74
Establish & treat cause
Reduce IOP Prevent functional vision loss Aim of Treatment

Filtration surgery Penetrating or nonpenetrating
Treat fellow eye trabeculectomy
Open
25–30% IOP reduction Glaucoma drainage device

Prostaglandin analogues e.g. Baerveldt, Molteno tubes
Proinflammatory – avoid Side effects Iridectomy
in uveitis/CME
Trabeculectomy or glaucoma
Avoid in asthma/lung disease/ Beta-blockers
drainage device
young children Reduce IOP
Surgical
Topical Closed Laser peripheral iridoplasty
Carbonic anhydrase inhibitors
Oral acetazolamide Goniosynechiolysis
Alpha-agonists Medical
Brimonidine safe in pregnancy (Class B) +/– Lens surgery to widen AC
Angle closure mechanisms Controversial
Pilocarpine Goniotomy
Constrict pupils Angle surgery
Side effects
Pediatric Trabeculotomy
Cataracts, iris cysts, retinal detachment Inflammatory component
Topical steroids Trabeculectomy
IOP > target Outflow surgery
Glaucoma drainage device
Disc/field progression despite target IOP Failure 4.18 Treatment
Need >3 bottles
Young, Afro-Caribbean, high IOP,
Pupil block mechanism Nd:YAG peripheral failed surgery
Rubeosis iridectomy
Contraindications Indications Neovascular glaucoma
Especially pigmented POAG & secondary OAG Selective laser

Uveitis
angles => PDS, PXF trabeculoplasty
Contraindications Pediatric
Uveitis, juvenile, poor TM view Antimetabolite Controversial
at Time of Surgery Absolute
Primary angle closure Pregnancy
Indications Contraindications
Angle occludable post PI Laser
Plateau iris syndrome Argon iridoplasty
Stem cell deficiency
Acute angle closure
Break attack in secondary forms Diabetic, high myope
End-stage glaucoma
Pain relief from high IOP
Contact lens wear
Hypotony – unreliable control Relative
Scleral thinning, rupture Cyclodiode Poor healing, thin conjunctiva, elderly
Risks
Aqueous misdirection, inflammation,
retinal detachment
Sympathetic ophthalmia – reported case

Fornix based
Flap
Glaucoma
Blebitis/Endophthalmitis
Large
Direct fluid posteriorly
Trabeculectomy Antimetabolite therapy
Mitomycin C application
Strategy
IOP high 5-fluorouracil
Failure Needling
Tight sutures + releasables
Trabeculectomy Late Repeat trabeculectomy/drainage device

+/– AC maintainer
Complications
Maculopathy
For 1 y postop Hypotony Choroidal folds
Start 2 hourly Suprachoroidal hemorrhage

Topical steroids IOP low
Bleb looking quiet
Asymptomatic
Decrease if 4.19 Glaucoma Flat AC
Over filtering Operations
Surface
Needling No contact lens wear
Based on bleb morphology, not on IOP

Failure
Bleb IOP control
Corkscrew vessels, vascular, Management
encysting, AC activity Hypotony
5-fluorouracil
Corneal decompensation
Epitheliopathy
Glaucoma Drainage PAS
Contraindications
Device Complications
Risk of hypotony
Cataract
Uveitis,
Argon suturelysis
pediatric
Tube occlusion
glaucomas
Blood, fibrin, membrane
Bleb revision
Globe malposition, motility

Subtopic disturbance
Endophthalmitis – rare
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76
Watch
Mild
How flat Iridocorneal touch

May need theater within 48 hours
Lenticulocorneal touch
Urgent theater
Peripheral iridectomy – not patent

Laser YAG PI
Cycloplegia
High IOP Aqueous misdirection Aphakic/Pseudophakic

PI – patent YAG vitreous face
Flat AC Phakic
Pars plana vitrectomy
Suprachoroidal hemorrhage – observe
Large – surgical closure

Seidel positive Wound leak
Small – observe/pad/aqueous suppressants
Overfiltration – bleb raised

Pad/Observe/Palmberg
4.20 Early Postoperative Low IOP
Trabeculectomy Ciliary body shutdown –bleb flat
Seidel negative Cycloplegia + Increase steroids
Complications
Serous choroidal detachment
Cyclodialysis cleft
Cycloplegia + surgical clsoure
Occlusion sclerostomy – Iris

Theater
gonioscopy
High IOP
History, vitreous, viscoelastic
Deep AC Tight flap Observe
Burp
Siedel positive
Wound leak
Low IOP AC formed/contact lens/pad
Siedel negative
Overfiltration
Observe/decrease steroids/aqueous suppressants
Antitumor drug
Glaucoma
Inhibit DNA synthesis, RNA transcription & protein synthesis

Alkylating agent
Inhibits fibroblast proliferation
Glaucoma – prevent scarring & closure of bleb

0.2–0.5 mg/mL for 2–5 min
Conjunctival intraepithelial neoplasia
Indications 0.02–0.04% QID 1–2/52
Pterygium postoperatively
Mitomycin C Similar low recurrence with conjunctival autograft though
Photorefractive keratectomy Reduce haze
Persistent wound leak, hypotony

Bleb
Complications Localized scleral melt
Bullous keratopathy
Corneal edema
Side effects
Corneal/scleral perforation
4.21 Immunosuppressants Corectopia, iritis, cataract, pain
in Glaucoma Surgery
Blocks thymidylate synthase production
Interrupts DNA/RNA synthesis
Antimetabolite Cellular thymine deficiency =>
Cell death
Cell cycle-specific
So need repeated dose
To inhibit inflammatory cell proliferation

Glaucoma – Postoperative subconjunctival injections
5-Fluorouracil filtering surgery 5 mg injection
Indications Antiviral Intraoperative topical application At trabeculectomy site

25–50 mg/mL for 2–5 min
Conjunctival intraepithelial neoplasia
Prevent recurrent proliferative vitreoretinopathy
Intravitreal
Experimental
Corneal ulceration
Goes into tear film
Side effects
Lacrimation, photophobia, lacrimal duct stenosis
Myelosuppression, dermatitis, diarrhea, cardiac toxicity

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78
Advanced Glaucoma Intervention Study
ALT => Trab => Trab

Treatment
vs Trab => ALT => Trab
IOP average <14 mmHg over 6 y

Less progression
Ocular Hypertension Treatment Study Advanced OAG AGIS
IOP net <18 mmHg over 6 y
Findings NO progression
Treat
IOP >26, CCT <550
IOP fluctuation more important
OHTS than absolute value
CCT important
<40 µm from 555 µm => 40% conversion
Clinical implications
Current clinical practice Trabeculectomy after laser usually
Ocular
IOP reduction Hyptertension Problems
Decrease 1 mm Hg => reduce risk 10% Black population
Glaucoma Laser Trial
European Glaucoma Prevention Study GLT

First line therapy
EGPS
Findings ALT = medical therapy
Findings
Dorzolamide ineffective in POAG prevention 4.22 Glaucoma
Studies Collaborative Initial Glaucoma Treatment Study
CIGTS
Early Manifest Glaucoma Treatment Study Medical Rx = Surgical Rx
Findings => Use Medical Rx first
EMGTS
Treatment slows progression 5FU following trabeculectomy
Findings Fluorouracil Filtering Surgery Study
Visual fields measure progression FFSS BD 1/52, then daily 1/52!
Early to
Moderate OAG Therapeutics Findings
5 y success rate – 5FU 48% vs control 21%
Collaborative Normal Tension Glaucoma Study
Latanoprost = 360 degrees SLT
SLT vs
Latanoprost
CNTGS Latanoprost better than 90 & 180 degrees SLT
IOP lowering stops progression NTG
Findings
Medication, laser, trabeculectomy SLT side effects more
IOP rise, pain, uveitis
Migraine, disc hemorrhage

Vasogenic factors important 3 y – Trab failure 30% vs tube failure 15%
Tube vs
Female, nocturnal hypotension Trab Study IOP lowering, complications, cataracts
Tube = Trab
Problems
Change in trabeculectomy
technique – fornix flap
5 Neuro-Ophthalmology
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80
Neuro-
Ophthalmology
5.1 Optic Nerve Dysfunction

Basics
5.34 Abnormal Eye
5.2 Ophthalmic Emergencies Movements & No Visual Symptoms 5.35 Chronic Progressive
External Ophthalmoplegia
5.3 Typical Optic Neuropathy
Optic Neuropathy
5.4 Atypical Optic Neuropathy 5.36 Anisocoria 5.37 Worse in Dark: Constricted = Abnormal
5.5 Optic Neuropathy & No Swelling 5.38 Worse in Light: Dilated = Abnormal
5.6 Etiology
5.39 Ptosis
5.9 GCA Management 5.8 Anterior Ischemic Optic
Neuropathy – Arteritic 5.41 Bell's Palsy
5.10 Anterior Ischemic Optic Neuropathy – Non-Arteritic 5.40 CN 7 Disorders 5.42 Underactivity – Treatment
Unilateral Neuropathy
5.11 Unilateral Optic Neuritis – Other Causes 5.43 Overactivity
5.7 Disc Swelling
5.12 Leber's Hereditary Optic Neuropathy
5.13 Papilloedema Head & Facial Pain 5.44 Migraines, Headaches & Facial Pain
5.14 Idiopathic Intracranial Bilateral Neuropathy & No
Hypertension Neuropathy
5.45 Intracranial or Systemic Pathology
5.15 Other Causes & Pseudoswelling
5.16 Chiasmal – Retrochiasmal Lesions
5.46 Functional Visual Disorders
5.48 Management
5.17 Questions to Ask Transient Visual Loss
5.47 Demyelinating Optic Neuritis
5.49 Internuclear Ophthalmoplegia
5.18 Etiology & Neuromyelitis Optica
5.19 Assessment
5.50 Myasthenia Gravis
5.20 Differential Diagnoses Systemic Diseases 5.51 Treatment
5.21 Neural & Central 5.53 Activity & Severity

5.22 Supranuclear
Diplopia 5.54 Acute Management
5.23 3rd Nerve Palsy 5.52 Thyroid Eye Disease
5.24 Variations 5.55 Chronic Management
Peripheral
5.25 4th Nerve Palsy 5.57 Cerebrovascular Disorders
5.26 Treatment 5.56 Orbital Decompression
5.27 6th Nerve Palsy
5.28 Muscle, Orbit & Other 5.58 Brain Lesions
5.29 Multiple Cranial
Nerve Palsies
5.31 Nystagmus Assessment
5.30 Abnormal Movements 5.59 Phakomatoses
5.32 Nystagmus – Congenital of Visual World
5.33 Nystagmus – Acquired – MRI All 5.60 Pregnancy & The Eye
Red target
Color Testing
Isihara
May be worse than visual acuity in optic neuritis

Neuro-Ophthalmology
Optic nerve disease

Relative afferent Widespread ganglion cell damage
pupil defect
Pain/Numbness/Tingling Retinal disease

Head/Face/Neck Limited ganglion cell damage
Less likely RAPD
Basic Neurological
Weakness/Numbness History Relevant to Pupils If dilated
Face/Arms/Legs Ophthalmology Sensitivity to light
Hearing/Dysarthria/Dysphagia/Dyspnea No anisocoria from optic

nerve disease
Best Corrected
With distance glasses/pinhole Visual Acuity Retinal lesions
5.1 Optic Nerve Subtopic
Dysfunction
Color
Clarity of funds view Pallor
Focus on examiner's nose
Vasculature
Central
Any part of face missing Thin or absent in atrophy
Disc
Confrontational Disc
Fingers in mid-quadrant Visual Fields Examination
Peripapillary NFL
Peripheral
Count fingers or target Margin blur
Formal perimetry Elevated, variable filling of cup

Appearance
Blood vessels obscured
Peripapillary NFL edema
Gray thickening
Disc edema
Disc capillary network
Hyperemia & dilation
Vein
Dilation & tortuosity
Peripapillary hemorrhages & exudates

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ONLY EYE
Rhabdomyosarcoma
Neuroblastoma Diplopia
Neoplastic CN 3 palsy – aneurysm
Proptosis, opsoclonus, ecchymoses, Horner's syndrome
Retinoblastoma Brain tumor

Leucocoria Urgent
Pediatrics Bilateral disc swelling

DEATH Intracranial extension, sepsis, Orbital cellulitis
cavernous sinus thrombosis Infection
5.2 Ophthalmic Pituitary apoplexy
Emergencies Acute bilateral ophthalmoplegia
Trapdoor
Operate urgently Orbital floor fracture
Acute myasthenia
Trauma Life-
Threatening
Ptosis or diplopia + dyspnea/ Myasthenia

Glaucoma dysphagia/systemic weakness
Acute angle closure glaucoma
Orbital cellulitis
Endophthalmitis Unilateral Pain + optic neuropathy
Tap/Inject +/– vitrectomy URGENT Sight-Threatening
Mucormycosis
Penetrating eye injury Malignant hypertension

Trauma
Retrobulbar hemorrhage
Giant cell arteritis

Bilateral
Sight-Threatening
Pituitary apoplexy
15–45, one eye only
Acute onset VA blur, worsens over hours to days

Positive
Pain on eye movement 90% patients

Longer-Term History
Check no atypical features Both AION & optic neuritis
Follow-Up
requiring investigation No cancer, vasculitis, autoimmune disease
Negative
No diplopia, no systemic neurological symptoms
Neuro-Ophthalmology
Bilateral if arthritic AION >40 y, one eye only RAPD

Seconds to minutes Nil, mild, moderate swelling
Positive Optic nerve head
Sudden VA/field loss 2/3 normal
Same or worsening slowly over 1/52
Positive Typical Optic Other eye normal
Neuritis Examination
Vasculopathic risk factors
HTN, DM, smoking History
No disc pallor, hard exudates, CWS
Negative
No cancer, vasculitis, autoimmune disease No hemorrhages, iritis, vitritis
Negative No intraocular diseases
No eye/orbital pain, no diplopia, no
systemic neurological signs
Affected eye
Any visual field defect
5.3 Typical Optic Perimetry
Mild to severe swelling Neuropathy Unaffected eye
RAPD Asymptomatic field defect
Hyperemic or pale swelling Positive
Stops worsening
Disc margin hemorrhages Optic nerve head Within 2/52
Vision
Follow-up
Starts improving
CWS Within 4/52
Suggests arteritic
Examination Anterior No complaint of progressive Unless advanced cup/notch
History
Ischemic Optic worsening of vision
No proptosis, no ptosis, normal eye movements
Neuropathy (AION)
Increased cup–disk ratio
Normal corneal & facial sensation Field loss
Positive
Negative IOP normal/raised
No hard exudates, iritis, vitritis,
other intraocular disease
Glaucomatous Intact neuroretinal rim not pale
Examination
Optic
Normal exam or pallor if previous AION VA, color normal
Other eye Neuropathy Negative
“Crowded” optic nerve head
No proptosis, no ptosis, normal eye
Affected eye movements, normal corneal & facial sensation
Any visual field defect
Perimetry
Altitudinal defects common
Unaffected eye Disc matches fields
Normal visual field Field loss
Perimetry Respects horizontal midline
No progression after 1/52 Follow-up
Visual acuity & field Normal disc
Normal field
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84
All patients Especially if acute optic nerve

swelling not observed
MRI Optic
Nerve + Brain,
with Contrast
MRV if bilateral
BBLL – Bartonella, B12 GCA, syphilis, SLE, sarcoid bloods disc swelling Exclude dural sinus
LHON, Lyme thrombosis
BP
If MRI negative/contraindicated
GaSSS BOIL
Ocular
Color vision, HVF Acute optic neuropathy
Imaging Unilateral or bilateral disc swelling

CXR, MRI with contrast
Chronic optic
Indications neuropathy Headaches, meningitic symptoms,
Lumbar puncture
unexplained neurologic symptoms
5.4 Atypical
Optic MRI
Cancer, vasculitis, autoimmune disease Neuropathy Chronic meningitis signs
History
Infectious Progressive visual loss & other

Basic Assessment
investigations normal
BLOOD PRESSURE!
Lumbar
Blood pressure, temperature
Puncture
Examination
Opening pressure
Urine analysis
Biochemistry
Protein, glucose
FBC, UEC, LFTs, BSL, ESR, CRP
Investigations Microscopy, cell count, culture
ACE, ANA, syphilis serology Microbiology
Cryptococcal antigen
Blood Tests
Folate, Vitamin B12 Bilateral symmetric optic
neuropathy Cytology, flow cytometry,
oligoclonal bands
Other
LHON, Lyme, Bartonella No infection, tumor, medical
contraindications
Indications
Chest X-Ray 3 Days 1g IV
Acute/chronic optic neuropathy
Methylprednisolone Trial
Management If improvement, continue oral

After 3 d
Taper on clinical response
T
Trauma
Radiation/Surgery
I Iatrogenic
Neuro-Ophthalmology
LHON
LHON & AD + AR optic atrophy

L Retinal Dystrophy
DIDMOAD
Retinal
Retinitis pigmentosa
Alcohol, tobacco
T Toxic Medications
Ethambutol, amiodarone, vigabatrin
Poison
Lead, cyanide
HTN, TED, DM – papillitis

Systemic
5.5 Optic Neuropathy &
Mass effect
No Swelling Tumor, hemorrhage, trauma
"A Pale Disc TILTS"
ICP raised CSF drainage/production
Dural sinus thrombosis
Brain IIH
Inflammation
Optic neuritis, neuromyelitis optica
S = Any Cause of Chronic Swelling
H
Hereditary optic neuropathy
I
Ischemic – AION, B/CRVO
P Pseudoswelling – Drusen,
hyperopia, MNFL, tilted disc
I Infective – syphilis, TB, toxoplasmosis

Eye HIP IIN
I
Inflammatory – scleritis, posterior uveitis
Neoplastic – optic nerve

N Meningioma, glioma
Lymphoma, leukemia
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Compression, contusion, laceration

RAPD, central VA loss Young, female, eye movement e.g. retrobulbar hemorrhage
pain & vision loss
MRI all
Direct Urgent decompression
Retrobulbar
Demyelination
Optic Neuritis Urgent neuroimaging
Postviral Treatment
Causes Optic nerve sheath fenestration for
Vasculitis sheath hematoma
SLE
Consider
Granulomatous Endoscopic optic canal/apex Caution: cavernous sinus
Syphilis, sarcoidosis decompression
Only if progressive neuropathy
Autosomal Shear forces, interrupt vasculature
Bilateral symmetrical Dominant
Central/centrocecal scotomas Optic Atrophy
Traumatic
Pedigree + OPA1 mutation testing IONTS 1999
Untreated = steroids = decompression + steroids
Ethambutol, isoniazid
Anti-TB
National Acute Spinal Cord Injury Study
Chloramphenicol
Medications NASCIS 1997 Steroid Rx within 8 h of spinal cord
Indirect Studies are
Hydroxychloroquine injury => benefit
Etiology inconclusive
Antineoplastic drugs Toxic Issues – optic nerve is sensory & white matter
Vincristine, cisplatin
Substance ingestion IV methylprednisolone within 8 h of
Lead, methanol, tobacco & alcohol traumatic brain injury
CRASH 2005
Thiamine 100 mg bd Steroids group at 6/12 => increased risk of death
Treatment 5.6 Optic
Folate, multivitamin, B12 injections High rate spontaneous recovery
Neuropathy Yu-Wa-Man &
& No Disc Griffiths (2007) <8 h postinjury
Pernicious anemia, malnutrition
Swelling – Etiology Steroids on individual basis
Vitamin B12
Symmetrical visual loss >8 h postinjury
Do test in all NO STEROIDS
Vitamin B1 (thiamine) Etiology Severe painless vision loss
Nutritional Presentation
Folate deficiency Consider cortical blindness
Malnutrition, chronic alcoholism If no RAPD + brisk pupils
Posterior
Vitamin A & E Ischemic Cardiac, major thoracic, abdominal surgery
Rare
Decreased blood delivery
Tumor exclusion Etiology Postsurgical
Diffuse or temporal disc pallor Immediate – 4–7/7 d postanesthesia
Still do MRI
GCA + other vasculitides
Vascular endothelial cell damage 1–3 y Resistance/obstruction to blood flow SLE, PAN, HZO
Vision loss Etiology Atherosclerosis
Type of PION Radiation
Postsurgical
Investigations BP, FBC, check hypovolemia
Anterior or posterior disc changes Hereditary
MRI + carotid Dopplers
Urgent MRI
Exclude recurrence of tumor
Artertic
Anterior ischemic optic neuropathy
Non-arteritic
Demyelinating
Inflammatory optic neuritis Sarcoid
Other
Infectious
Neuro-Ophthalmology
Bartonella, HZO, Toxoplasma

Optic
Hyperemia neuropathy Neoplastic
Meningioma, glioma, orbital tumor
Microvascular abnormalities Compressive

Thyroid eye disease, mucocele
Non-neoplastic
True swelling Unilateral Aneurysms
Blurring of disc margin
Infiltrative
Hemorrhage, CWS, exudate Leukemia, lymphoma, solid tumors
Leber vs hereditary optic neuropathy

Features
Absent cup, small disc Becomes bilateral
Uveitis
Venous pulsation
Early papilloedema
Pseudoswelling No optic
Anomalous branching
neuropathy Hypotony
Irregular margin Incipient CRVO/AION

5.7 Disc Swelling Diabetic papillopathy
Drusen
Optic nerve meningioma (early)
Glaucoma
Chronic papilloedema
Unilateral papilloedema & contralateral optic atrophy
Other
Chronic malignant hypertension
Intracranial tumor Optic Sarcoid
Frontal olfactory groove meningioma Foster-Kennedy
syndrome neuropathy
Syphilis
Inflammatory & infectious
Previous unilateral Vogt–Koyanagi–Harada syndrome
Undergoes atrophy
optic neuropathy Pseudo FK Sympathetic ophthalmia
syndrome Bilateral
Graves’ disease
Subsequent acute event other eye
True papilloedema
Idiopathic intracranial hypertension (IIH)
No optic
neuropathy Malignant hypertension Drusen
Psuedoswelling Hypermetropia
Astrocytic hamartoma
87
88
Male = age/2
Normal
Giant cell arteritis, temporal arteritis aka
Female = (age+10)/2
ESR
>70
Abnormal
10% of AION More specific

CRP Less affected by age & anemia
Age >50 y, mean age 70 y, F>M
Investigations High ESR + CRP

Short posterior ciliary arteries 99% sensitivity, 97% specificity
Inflammatory & thrombotic occlusion
Platelets
Active disease
80–90% as AION General
Thrombocytosis (increased platelets)
10% as CRAO Suspect AAION, symptoms, high ESR

Temporal artery biopsy
60% as transient visual obscuration Presentation
False negative in 5–10%
Skip lesions
10% as PION
5.8 Anterior
FFA
Ischemic Optic
2–15% as diplopia or cranial nerve palsy 1–12 Disc & choroidal delay
Neuropathy –
Arteritic
Jaw claudication
CWS Most specific
AION + cilioretinal occlusion =
GCA until proven otherwise
Neck pain
Arteriolar occlusion
Headache, temporal tenderness

Optic disc edema + pallor Systemic
Ocular
Chalky white swelling
Signs Symptoms Malaise, anorexia, weight loss,
Uveitis, corneal edema, low IOP, Ischemia signs fever, joint & muscle pain
dilated retinal veins
Polymyalgia rheumatica
Motility deficit
Transient visual obscuration or AION

Systemic Ocular
Tender/nonpulsatile temporal artery Visual loss
Severe <6/60 in 60%
ESR repeat
General symptoms ongoing

Increase dose
2 wk
Visual symptoms ongoing
Re-admit, more IV methylprednisolone
Neuro-Ophthalmology
Compliance
Minimum 18–24 mo
Ongoing
Regime Further 12 mo follow-up
When off steroids
Some continue small dose long-term

e.g. 3 mg/daily
Prophylactic 1 mg/kg oral Initial 1mg/kg
If suspicious 6–10/52
Get to 20 mg
Taper
Still symptomatic => 5.9 Ischemic Optic
double dose IV methylprednisolone Neuropathy – 6/12
1 g/ d 3–5 d Steroids Get to 10 mg
GCA Management
No improvement =>
question diagnosis Slow wean monthly after this
Then oral prednisolone 1 mg/kg

1/3 in 1 d
Initial
Osteoporosis prophylaxis Contralateral vision loss 1/3 in 3 d
EARLY TREATMENT CRITICAL
Alendronate, vitamin D (calcitriol), calcium tablets
1/3 in 1 mo
Gastric ulcer
Omeprazole
Within 1 wk of steroids Natural History Scalp ulceration

Temporal
High index suspicion & artery biopsy
negative first biopsy Raynaud's phenomenon
Contralateral biopsy
3–14% positive rate Systemic complications Transient ischemic attack or stroke
Visceral ischemic infarctions

Myocardial, aortic
aneurysms, bowel
89
90
Crowded disc & disc drusen NAAION rare in normal

cup–disc ratio patients
Atherosclerosis
Short posterior ciliary arteries
90% of AION Risk Factors Disc at risk HTN, smoking,
hyperlipidemia, diabetes
Nocturnal hypotension
Age >50 y General Low vision when awake
"Crowded" disc – at risk Possible

Optic disc microcirculation Sildenafil
compromised
5.10 Anterior
Check contralateral disc also crowded Carotid occlusive disease
Ischemic Optic Unrelated
Neuropathy – Prothrombotic disorders
Non-Arteritic
Autoimmune disease Compressive, ESR & CRP
Investigate if Embolic sources
infiltrative, vasculitic
No investigation needed
History of cancer Investigations Typical NAION Prothrombotic sources
Age <40 y, pain with eye movements

Age <40 y, SLE
No diplopia, orbital pain, systemic Atypical NAION

Demyelinating
neurologic disease
Cardiovascular risk factors

Central visual deficit GP
Management
Inflammatory Diagnosis of Commence aspirin, cease sildenafil
No uveitis, hard exudates,
Exclusion
intraocular disease
Review 2–4/52, & 3–4/12
No proptosis, ptosis Confirm stable findings
Corneal & facial sensation Infectious &

compressive Involved eye
40% mild improvement over 6/12
Eye movements normal
Pseudo Foster–Kennedy syndrome
Exclude orbital apex syndrome
Follow-Up Previously affected disc – pale

25% risk developing NAAION
Contralateral disc crowded Contralateral eye
Currently affected disc – swollen
Vision stable or worsens at 1/52
True FK syndrome due to

intracranial mass
Risk of acute myocardial infarction & stroke
Demyelinating
See separate section
Mimics optic neuritis

But no improvement 4/52
Leukemia
Systemic
Causes Meninges, brain, lungs, joints, skin, kidneys
Lymphoma
Infiltrative Clinical Disc
Breast, lung, bowel
Neuro-Ophthalmology
Direct or diffuse Normal/Pale/Swollen

carcinomatous meningitis Solid tumors Sarcoid
Inflammatory Uveitis, iris nodules, vascular

Malignancy history, slowly progressive sheathing, granulomas
Clinical
Disc normal, swollen or pale Investigations CXR, ACE, Gallium scan, PET scan,
tissue biopsy
Sphenoid wing meningioma, Intracranial
pituitary region tumor Steroids
Responds by 1/52
Optic nerve sheath meningioma Other Similar to sarcoid

Neoplastic SLE, IBD, Reiter's
Optic nerve glioma Intraorbital
5.11 Unilateral Optic Bartonella – cat-scratch
Orbital tumor – primary or metastases Neuritis – Other Causes Bacteria
Causes TB, syphilis, Lyme disease
Viral HZO
Thyroid eye disease
Measles, mumps, chickenpox
Causes
Mucocele Fungi
Ethmoid or sphenoid Compressive Mucor, cryptococcal meningitis
Non–neoplastic
Rickettsia
Aneurysms
ICA, ACA, ophthalmic artery Parasites
Toxoplasma, Toxocara
Infectious
Slowly progressive
Field or vision loss incidental Secondary Sphenoid sinusitis, orbital cellulitis,
Two presentation types meningitis, mucoceles
Pituitary apoplexy, vascular Acute presentation
Disc
compression => infarction Normal/Swollen/Pale
Macular star
Low or normal VA, RAPD Hard exudates at macula
Eyelid edema, retraction, proptosis, ptosis Signs Signs

60% due to Bartonella
Orbital disease signs Macular star + disc swelling =
neuroretinitis Does not occur with
EOM restriction
demyelinating disease
Orbital apex syndrome, cavernous sinus disease Vitritis or vasculitis Can occur with any
ONH swelling Papilloedema,
malignant HTN
91
92
Investigation Pedigree
Male, age 10–30 Genetics
Blood test for mtDNA mutation
Mitochondrial maternal
Gene defect 11778 in inheritance
maternal DNA
Cardiology
Cardiac conduction abnormality
Treatment
Coenzyme Q10
Painless monocular severe visual loss 5.12 Leber's

<6/60 Hereditary
Acute Optic Neuropathy
RAPD, central field loss
50% bilateral sequential vision loss

Weeks/Months
Clinical Prognosis
50% simultaneous vision loss
Normal
Hyperemia & elevation of disc
Disc
Non-11778 mutations
May recover
Peripapillary telangiectasia Classic findings
Tortuosity of medium sized vessels

Headache
Worse morning, coughing, straining
Check blood pressure Important Pulsatile tinnitus
Symptoms Transient visual obscuration
Bilateral swollen discs look Transient vascular insufficiency of swollen disc
Scan all patients
same as papilloedema
CN 6 palsy
Diplopia
Optic nerve orthograde axoplasmic Nausea & vomiting
Neuro-Ophthalmology
flow interrupted
Transmitted to
subarachnoid space Visual symptoms absent
Raised intracranial Hyperemia, mild elevation

Axoplasm buildup pressure Early Optic discs
Swelling Margins indistinct
Dead discs => Pathology Loss os spontaneous venous pulsation
Do not swell Presence of SVP excludes raised ICP
Optic nerve edema
Indistinguishable from other causes of edema VA normal or reduced, TVO
Acute
Types Clinical Severe hyperemia, moderate elevation
Optic nerve function often normal Optic discs
Margins indistinct, small surface vessels obscuration
Disc Established
Pale, not hyperemic Chronic Venous engorgement, peripapillary flame hemorrhages
Optic nerve function abnormal Signs CWS, hard exudates at macula
Intracranial mass Constriction – peripheral then central

Visual field
Trauma Increased brain volume 5.13 Monitor & remove cause
Cerebral edema Papilloedema
Metabolic disorders Chronic Elevated
Champagne cork appearance
Obstructive Optic discs
Dilated ventricles on MRI Optociliary shunts
Hydrocephalus
Decreased absorption by villi
Communicating No hemorrhages/CWS
Normal ventricles on MRI
VA impaired
Cerebral edema Causes Late Gray, elevated
Meningitis Optic discs

Secondary obstructive or Loss of cup
communicating hydrocephalus
Decreased CSF Clot obstructs CSF drainage Superior sagittal or
Increased volume drainage Pathology transverse sinuses
Secondary communicating Anticardiolipin/lupus anticoagulant syndrome
hydrocephalus Subarachnoid hemorrhage
Causes
Preretinal + vitreous Terson's syndrome Dural Venous Infection Adjacent to sinus
hemorrhage Thrombosis
Dural venous thrombosis Dehydration, pregnancy, cancer
Decreased CSF resorption
Investigations
Extracranial venous outflow Urgent MRI plus MRV
obstruction
Heparin
e.g. superior vena cava obstruction Treatment
Idiopathic intracranial hypertension CSF shunt
Save vision & prevent stroke
93
94
Stop medications
Even 6% better than acetazolamide
TVO, pulsatile tinnitus, headache

As per papilloedema GP, Dietician
Symptoms
Optic nerve function normal early
Weight loss
Gastric banding
90% have bilateral
Increase to 1.5–2 g/d

Based on side effect tolerance
Female, obesity, 30
Tetracyclines Contraindicated 1st trimester
Risk Factors Medical Pregnancy
Acetazolamide
(Vitamin) A compounds Treatment 500 mg bd Safe >20/52
Isoretinoin
Cyclosporin e.g. frusemide, amiloride
Medications – TACOS Diuretics
Alternatives Monitor electrolytes
Oral contraceptive pill Monitor electrolyes
Topiramate
Steroids withdrawal
High ICP & visual loss

Nalidixic acid 5.14 Papilloedema –
Idiopathic Increases weight
Tumor IV corticosteroids
Intracranial Withdrawal can worsen
Not for long term
Hypertension
Hydrocephalus
MRI Side effects
Meningeal lesion Ventriculo-peritoneal shunt/lumbar

Repeatable
Cerebral venous occlusion Shunt
MRV Headache not controlled by medical treatment

Investigations
Dural sinus venous thrombosis
Vision threatened, headache NOT severe
Only after excluding mass lesion/ Surgical
obstructed hydrocephalus Treatment Improved VA or stabilized
Check ICP Lumbar 94% (Banta & Farris [2000])
Optic nerve sheath
puncture fenestration
Opening pressure >20 cm H20
2.5% risk severe irreversible vision loss
CSF constituency May need to repeat

Oligoclonal bands
Microscopy/Culture /Sensitivities Pregnant

Indications
Presurgical treatment
Alternative
Optic nerve function 4–5/62 Serial lumbar punctures
"Glaucoma of the brain" Follow-up Treatment
Infection risk, short-term benefit
Regular visual fields Disadvantage
Difficult in obese persons
Enlarged blind spot, arcuate defects
Peripheral depression
Autofluoresce
Clinical
Neuro-Ophthalmology
Small, elevated, indistinct margins

CWS, hemorrhages, exudates Discs
Buried
Drusen
Elschnig's spots
Extruded
Choroidal infarcts Fundus changes Yellow refractive bodies
Siegrist lines Optic Disc Progressive VF loss

Line of pigment, Malignant
Drusen Treat as glaucoma
Fibrinoid choroidal vessels Hypertension
necrosis Treatment
Tell patient
Avoids future confusion
Cushing's reflex Raised ICP investigation
Raised ICP => required
secondary raised Can cause NAION
Optic disc crowding
5.15 Disc Angiod streaks & pseudoexanthoma elasticum

Swelling – Other Causes Associations
& Pseudoswelling Retinitis pigmentosa
Originate at disc margin

Extend to peripapillary region Alagille syndrome
AD inheritance; liver, renal, cardiac disease
Astrocytic
Hamartoma
Inner retinal layer origin
Retinal vessels obscured
No autofluorescence Hypermetropia
Optic nerve hypoplasia

Small disc, double ring around disc
Congenital Anomaly =
Not Progressive
Congenital tilted disc
Maldevelopment of inferonasal crescent
“Morning Glory” disc anomaly

95
96
Respect midline
Homonymous visual field defect
Scan all
Stroke 90%, but tumor presents same way
Optic tract to lateral geniculate nucleus

Masses, aneurysm
Inferior retinal fibers => Meyer's loop

Retrochiasmal Temporal lobe Contralateral superior homonymous quadrantanopia
Superior retinal fibers Contralateral inferior homonymous

Parietal lobe quadrantanopia
Middle + posterior cerebral artery

Supply macula region
Occipital lobe
Macula sparing homonymous hemianopia
PCA supplied primary visual cortex
Nerve affected
Diminished VA one eye
5.16 Chiasmal & Anterior chiasm (junctional syndrome)
Retrochiasmal
Temporal defect opposite eye
Lesions Bitemporal visual Decussating fibers
field loss patterns
Mid chiasm No central vision loss
Bitemporal hemianopia
Bitemporal hemianopic scotomata

Posterior chiasm
Macular crossing fibers
Respect midline
Hormonal imbalance
Chiasmal Pituitary adenoma Bromocriptine therapy

Prolactinoma
Causes Trans-sphenoidal excision/radiotherapy

Craniopharyngioma
Suprasellar or parasellar
meningioma
MRI + formal perimetry

Headache, diplopia
Hemorrhage into pituitary tumor
Pituitary apoplexy
Cavernous sinus involved
Emergency
Monocular
Ipsilateral carotid, optic nerve, retinal circulation
Monocular or Binocular
Occipital lobe circulation

Binocular
Neuronal depression of visual cortex
Neuro-Ophthalmology
e.g. migraine
Migraine, vasospasm
<50 y
Age Pregnant
Eclampsia => CVA
>50 y
GCA, CVA
Drusen, papilloedema
Seconds
GCA
Duration
Seconds to hours Migraine
+ Visual phenomena
1–10 min
5.17 Transient Visual Loss – Cerebrovascular disease 70–99% (Dutch Transient Monocular
Questions to Ask Blindness Study)
Altitudinal shade onset & Carotid artery stenosis

Pattern of Loss & Recovery disappearance
Visual phenomena
Migraine
Headache/Visual phenomena
Migraine
Persistent headaches
Raised ICP
Associated Symptoms & Signs GCA symptoms
Stroke neurological symptoms
Skin, joint changes

Collagen vascular disease
Smoker, systemic hypotension, oral contraceptive

97
98
Ocular & surface

Cornea, retina, glaucoma Tumor
Giant cell arteritis Seizures
Monocular
Arteriovenous malformation
Expanding scintillating scotoma
Ocular ischemic syndrome
Diagnosis of exclusion Visual phase

Retinal migraine Fortification spectra
Zigzags
Recurrent same episodes, family history
Hemicranial, 1–24 h
Transient monocular vision loss Headache phase

Symptoms a/w Photophobia, phonophobia, nausea
Attributed to ischemia/vascular Binocular – Resolution phase
insufficiency migraine Resolve completely
ICA atherosclerosis Amaurosis fugax Visual loss, no headache

Binocular Acephalgic
ICA dissecting aneurysm
Causes
GCA Full neuroophthalmic exam
Examination
Heart valve, patent Cardiac disease Cholesterol Investigate all with new/atypical headaches
foramen ovale
Types 5.18 Transient Stop smoking
Platelet–fibrin
Visual Loss – Etiology Migraine + smoking => stroke risk
Calcium
Brainstem, cerebellum, posterior
GCA cortex via posterior cerebral artery
ESR, CRP
Carotid dissection
Arterial disease
Urgent MRI+MRA, or CTA Monocular – Stenosis, embolic, GCA
Investigations
ECG Embolic
50–80% have stenosis
Carotid Doppler USS
Suddenly standing up
Cardiovascular risk factors Causes Postural hypotension
"Blacking out"
Vertebrobasilar
North American Symptomatic insufficiency Cardiac thromboembolism
Carotid Endarterectomy Trial Heart disease
Arrhythmia
70–99% CCA stenosis
Age >75 y, male, Hx of TIA/CVA, Very sudden, no visual phenomena

intermittent claudication Surgery beneficial
Drop attacks
80–94% ICA stenosis NASCET Symptoms Sudden LOC
Treatment
Surgery not beneficial Walking, talking, swallowing
50–69% CCA stenosis
Brainstem/Cerebellar signs
Vertigo, disorientation
1st line – aspirin
Start platelet inhibitor
2nd line – clopidogrel Medical therapy Management
Cardiologist/Neurologist
Anticoagulation – embolic
source, AF present
Binocular/monocular
Sudden
e.g. ischemic
1. What does Gradual

it sound like? e.g. compressive, tumor
History
Neuro-Ophthalmology
Variable
Supranuclear = brainstem,
e.g. myasthenia
cerebellum, thalamic & above
Brain & Transient
brainstem e.g. GCA
Motor
Associated symptoms
e.g. Bitemporal
field loss – Sensory (less common)
Chain of Diagnose it
hemifield slide Command Primary gaze, smooth pursuit, saccades
CN 3, 4, 6 Nerve 2. What does The company it keeps

it look like?
Neuromuscular P – Pupils
Extraocular muscle Localize

E – Eyelids
it – PEPSI
5.19 Diplopia –
Orbit
Assessment P – Power
S – Sensation (cornea, skin)

Orbital apex, cavernous sinus,
Check CN 1–8 function
systemic disease All Diplopia I – Interpalpebral aperture & orbit
Microvascular vs aneurysmal Ischemic CN 3, 4, 6

Pain does not distinguish
Congenital 4th nerve palsy

Partial 3rd nerve palsy Safe diseases
Diplopia secondary to childhood strabismus
Great 3. Why SHOULDN'T I
Myasthenia
Mimickers investigate this?
Decompensated esophoria/esophoria
Thyroid eye disease
Primary convergence insufficiency
MRI/MRA Brain/Orbits with contrast

Investigations
Bloods – GCA, myasthenia, thyroid
Treat cause
4. Treatment
Restore binocular vision/occlusion

99
100
Miller–Fisher
Neural
Demyelinating/multiple sclerosis
Refractive Monocular
Acute disseminated encephalomyelitis
Retinal
Internuclear ophthalmoplegia
Central Nerve Lesion
Skew deviation
Brown's syndrome
Parinaud's dorsal midbrain syndrome
Thyroid orbitopathy
Supranuclear
Ophthalmoplegia Vertical saccadic palsy
Orbital myositis
Restrictive myopathy Horizontal saccadic palsy
Anesthesia – retro/peribulbar
Myotoxicity Progressive supranuclear palsy
Orbital trauma & fractures 5.20 Diplopia –
Differential
Congenital fibrosis extraocular muscles Diagnosis Third nerve palsy
Peripheral
Brain, nerve or NMJ disease
Fourth nerve palsy
Trauma
Sixth nerve palsy
Orbital myositis
Pareitic myopathy Muscle or Orbit
Genetic myopathies Neuromuscular Junction
CPEO due to mitochondrial disease
Myasthenia
Myotonic dystrophy/
oculopharyngeal dystrophy
Loss of motor control
CCF Secondary to primary Decompensated childhood strabismus
intracranial disease Loss of suppression
Cavernous sinus
thrombosis
Other Decompensated phoria
Diagnosis of exclusion
GCA Acute orbitopathy
Convergence insufficiency
Convergence spasm
Lymphoma
Divergence paralysis
Orbital pseudotumor
Lesion Medial longitudinal fasciculus
(horizontal gaze center)
Failure of adduction & nystagmus

of abducting eye
Neuro-Ophthalmology
Internuclear Feature Adduction intact on convergence

Ophthalmoplegia
Bilateral
Large angle exotropia
Young
Demyelination
Cause
Old
Stroke
Brainstem lesion causing Not nuclear or fasicular

Skew Deviation oblique/vertical deviation
Other brainstem signs

Feature Vertigo, oscillopsia, limb neurology
5.21 Diplopia –
Neural & Central
Postinfectious demyelinating Guillain–Barré – ascending limb weakness
polyneuropathy
Miller–Fisher
Ataxia
Triad Areflexia
Ophthalmoplegia
+/– Optic nerve papilloedema, pupil, ptosis signs
Multiple Sclerosis See separate section
Acute Disseminated Optic neuritis

Encephalomyelitis
101
102
Saccades > smooth pursuit > vestibuloocular reflex affected
Form of vestibulocochlear reflex (VOR)
General Head nod Eyes move in opposite direction of

Doll's eye movements head movement
Oculocephalic reflex
Idiopathic => fatal within 10 y
Vestibular pathways => saccades Not through frontal eye fields =>
ocular motor nuclei
Loss downward saccades
=> Lesion above ocular motor nuclei

"Dirty tie syndrome" Preserved pursuit
& doll's eyes Interrupt posterior Coordinate upward eye movements
Cannot see food => spill on tie commissure
Feature Lesion
Progressive +/– Pupil/convergence pathways
Later => upward & horizontal saccades Supranuclear Palsy
Then smooth pursuit & vergence Limitation upward gaze
Finally VOR lost Upgaze supranuclear palsy Poor/absent upward saccades &
smooth pursuit
Other 5.22 Diplopia – Vertical VOR & Bell's phenomenon intact

symptoms Features - UCLA
Supranuclear
Dementia, neck rigidity, speech problems Convergence retraction nystagmus
On upgaze, painful
Lid retraction
Parinaud's Dorsal Abnormal pupils

Midbrain Syndrome Bilateral large, with light/near dissociation
Paramedian pontine reticular Lesion Horizontal
formation (PPRF) Saccadic Palsy Tumors
Pinealoma
Stroke, multiple sclerosis, tumor Cause

Hydrocephalus Dilation of cerebral aqueduct in midbrain
Stroke or multiple sclerosis
MRI midbrain & pineal region
Vertical Lesion Midbrain => rostral interstitial

Saccadic Palsy nucleus of MLF
Stroke, multiple sclerosis, tumor

ESR, CRP
Exclude GCA
Restricted motility Eye down & out

Complete Essentially image all Exclude posterior communicating
Dilated pupil artery aneurysm
Poor response to light
Presentation Pupil involving

EOM some limitation Investigations
MRA or CTA
Neuro-Ophthalmology
Incomplete pupil sparing

Sparing or involving Partial Indications Failure to resolve in 3/12
Pupil
Complete sparing but one of
Evolving
If getting worse Patient <50 y
Atherosclerotic risk factors Additional cranial nerve abnormalities

Ptosis
Muscles Endovascular coiling
Loss elevation, adduction, depression Pupil Aneurysm
involving = BAD
5.23 Diplopia – Neurovascular clipping
GCA
Urgent CTA or MRA 3rd Nerve
Presume secondary to aneurysm Cause Steroids
Palsy
Junction of P. comm. & ICA Atherosclerosis
Aspirin, cardiovascular risk factors
Watch daily for 1/52 => check progression
Incomplete sparing = PROBABLY BAD Spontaneous recovery “Masterly inactivity”
Wait 12 mo
Image
Pupil
Classification
sparing Consistent measurements
Pupil normal “Established” CN 3 palsy
Lid & muscle Relief of diplopia
Ptosis & no movement Complete sparing Ptosis/Occlusion can help
Difficult surgery
Microvascular ischemia Unsatisfactory, unpredictable outcomes
Treatment MR functional
Diagnosis
Rarely GCA, cavernous sinus syndrome MR resection/LR recession
Vertical deviation
Commonly benign Isolated pupil Vertical resect/recess
abnormality Palsy
Partial Weaken overacting muscle
4 Ts – Tropicamide, Third nerve, Tonic pupil, Trauma Overaction of other eye on Hess
Recession or posterior fixation suture
LPS function present
LPS resection
Look down & nasal Ptosis
Eye should intort even if no adduction Normal CN 4
No LPS function
Examination Levator sling
Normal CN 6
Abduction Aim BSV in primary position
Fix globe to orbit – stay sutures
Complete
Normal CN 2, 5, 7 SO transposition
Relieve unopposed torsion
LR to lateral orbital wall/nasal transposition

103
104
Superior rectus & levator only
Superior
Can be congenital
Autosomal recessive
Divisional Palsy Inferior

Medial & inferior rectus, inferior oblique & pupil
Anterior cavernous sinus lesion or posterior orbit
MRI
Test other cranial nerves
5.24 Diplopia –
3rd Nerve Palsy –
Variations No trauma or aneurysm
Superior division palsies Expanding parasellar lesion
Autosomal recessive Congenital
History Imaging
Microvascular
No aberrant regeneration occurs
Transient Viral or vaccination
ophthalmoplegia can occur Children
Young Patients Aberrant Eyelid–gaze dyskinesis

Regeneration
Aneurysms rare Downgaze or adduction => Elevation of eyelid
Image (Pseudo Van-Graefe sign)
Pupil-involving
Check structural etiology Classic findings
Pupil–gaze dyskinesis
Downgaze or adduction =>
Ophthalmoplegic migraine = diagnosis of exclusion Miosis occurs

Muscle–gaze
Attempted elevation/depression =>
Adduction occurs
TRAUMA Common after trauma
Subarachnoid space, cavernous sinus Head tilted downward
Causes Bilateral Hypertropia reverses

Asymptomatic until 4th–6th decade
Neuro-Ophthalmology
Congenital Eye movements Bilateral positive Bielschowsky

Vertical fusional amplitude decreases
Age >50 y => microvascular V-pattern
Symptoms Bilateral IO overaction
Double Maddox rod
5.25 Diplopia – 4th

Excyclotorsion <10 PD = unilateral
Diplopia Nerve Palsy Special tests
Especially downgaze Symptoms
>10 PD = bilateral
Subjective torsion
Fundus torsion
Abnormal head posture

Clinical
SAME SIDE TURN
Check bilateral
WORSE ON
SAME SIDE TILT Signs
Hypertropia Head tilt
IO overaction/SO underaction
Symptoms No subjective torsion
Park's 3 step test
No pain, GCA, or neurological symptoms

Congenital
ESR, CRP
Age >50 y => GCA Same as acquired
Vasculopathic risk factors No excyclotorsion

Examination
Investigation
Does not resolve by 3/12 Vertical fusion – wider range 5 PD
Neuroimaging base of skull Age <50 y Neuroimaging

Facial asymmetry
No risk factors, no trauma

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106
IR myomectomy or recession Recession allows

re-operation
<15 PD => 1 muscle 10–20 PD
Inferior oblique Parkes point – 3mm/3mm

overaction IO weakening IR insertion point 10–15 PD
+/– IR/SR recession
Anterior transposition
Unilateral (at IR insertion) – 15–20 PD
Anterior transposition + 2 mm – 20–25 PD
Vertical deviation, Opposite side IR weakening

no IO overaction
5.26 Diplopia – +/– Ipsilateral SR weakening

4th Nerve Palsy
Treatment
Torsion
Diplopia in what range Harada-Ito/SO tuck
Torsion
Congenital
Wait 6 Months
Ability to correct with vertical prisms Treat height only
Base treatment
on symptoms
Spread of comitance Split SO tendon
Torsion – Harada–Ito
Bilateral
Abnormal head posture procedure Anterior fibers advanced + moved laterally unto LR
Acquired
Increases intorsion
Then height 2/52 later

Contralateral IR recession
Vasculopathic risk factor control
Occlusion
Supranuclear Fresnel prism

FEF or PPRF Diplopia
<20 PD deviation
Neuro-Ophthalmology
Nuclear Botox ipsilateral MR

Ipsilateral gaze palsy
Causes
Acoustic neuroma, meningioma
Cerebellopontine angle
No recovery 6/12
CN 5, 6, 7 & cerebellar signs
Involved eye
Fascicular/subarachnoid space Recess MR or resect LR
Along clivus, entering cavernous sinus Partial palsy +/– Contralateral MR recess
Ischemic neuropathy Treatment
Most common Jensen
Peripheral
IR/SR & LR split and muscle to muscle sutured
GCA, intracavernous, intraorbital
Surgery IR/SR split & dis-inserted, attached to LR

Horizontal diplopia Clinical
Abduction deficit Hummelsheim
Total IR/SR attached to anterior &
Esodeviation worsens with distance posterior to LR insertion
Complete palsy –
transposition surgery
Foster modification
Intracranial mass (posterior fossa), raised ICP 5.27 Diplopia – 6th
MRI Nerve Palsy 50 degree range of single vision in 70% patients
Patient <50 y
Demyelination (FLAIR MRI)
+/– MR recession
Foster leaves MR – preserve adduction
No neuroimaging
No resolution after Isolated CN 6 in vasculopath

Contralateral MR
3/12 => MRI Faden surgery
Investigation
ESR, CRP Slow saccades
GCA
Patient >50 y +/– Botox
Severe pain, neurological sign,

history of cancer
MRI
Papilloedema
Bilateral CN 6 palsy
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108
Esotropia (ET) at near, not at distance
Divergence Paralysis
Pontine/skull base tumor Progressive CN 6 palsy
Beware! EOM tight – enlarged, fibrosed, or entrapped
Myasthenia
Signs
Forced duction test
Horizontal diplopia
Near only Investigations
Restrictive CT orbit
Myopathy
Brainstem disorders
Dorsal midbrain syndrome, Parkinson's,
Treatment
progressive supranuclear palsy Causes Strabismus surgery Recess, never resect
Partial CN 3
Insufficiency
Myasthenia
5.28 Diplopia –
No hypertropia in primary or adduction c.f. CN 6
Muscle, Orbit
Brown syndrome
Correct hypermetropia for asthenia symptoms & Other
CT scan – SO muscle belly thickness
Primary
Pencil 'push-ups' Treatment
Convergence
Bilateral ptosis, reduction
Avoid surgery Paretic movements, orbicularis
Uncorrected hypermetropia Myopathy Chronic progressive Range does not improve
Spasm external ophthalmoplegia with doll's eye reflex
Excess convergence Slow saccades
Acute unilateral/bilateral injection,
Intermittent history, no other history
proptosis, diplopia
Presentation
No limitation in any direction Decompensated Phoria
Multiple cranial nerve palsies
Clinical Acute
Normal saccades
Orbitopathy
Comitant Investigations CT orbit, sinus & cavernous sinus

with contrast
Congenital CN 4 palsy,
intermittent exotropia (XT)
Treatment
Loss of motor control
Cause As per condition
Decompensated
Tired, alcohol
Childhood Strabismus
Subtopic
Acoustic neuroma
Nasopharyngeal cancer
CN 5–8 – Cerebello-
Pontine Angle Pontine glioma, meningioma,
Carotid–cavernous fistula
trigeminal neuroma
Proptosis, frozen globes, vision loss
Neuro-Ophthalmology
Base of skull fracture

Face, mouth,
throat, sinus, orbit Cavernous sinus thrombosis
Secondary to infection Multiple sclerosis

IV antibiotics Causes
Hemorrhage or infarction Stroke
Acute
CN 5, 8–10 – Lateral
Systemic steroids, neurosurgery referral Pituitary apoplexy Medullary Syndrome
CN 5 & spinothalamic tract
In pregnancy = Sheehan syndrome Hemihypoalgesia

8 – nystagmus
Immunocompromised
Mucormycosis, zygomycosis Symptoms 9 & 10 – dysarthria & dysphagia
GCA, VZV Sympathetic – Horner's
Meningioma 5.29 Multiple Cranial Cerebellum

Nerve Palsies Nystagmus, other cerebellar signs
Pituitary adenoma Primary
Craniopharyngioma Ataxia
Nasopharyngeal cancer CN 3–6 – Cavernous TRIAD Atrexia

Local Tumors within sinus Sinus Syndrome Miller–Fisher &
Bilateral Multiple Gullian–Barré variant Ophthalmoplegia
SCC with perineural spread
Cranial Nerve Palsies
Distant metastases Ophthalmoplegia
Ocular Ptosis
Cavernous sinus ICA aneurysm symptoms
Optic neuritis &
Sterile granulomatous
papilloedema
inflammation Subacute Wernicke's encephalopathy
Severe boring pain Tolosa–Hunt syndrome Pupil abnormality
Often lymphoma
Regress with steroids

Other Brainstem
TB, sarcoid, Wegener's, mucocele, Syndromes e.g. Benedikt
carcinomatous meningitis
109
110
Illusory to-and-fro movement of environment
If head movement worsens

Vestibular origin
Drugs
Alochol, antiepileptics, barbitals, many others
Fixation & voluntary nystagmus
Oscillopsia Vestibulocochlear/pathway lesion
Head trauma, seizures

Causes
MLF involved – e.g. internuclear ophthalmoplegia
Intermittent XT
5.30 Abnormal Opsoclonus & ocular flutter

Movements
of Visual World Vertebral artery dissection
New-onset inner ear or brain pathology

With oscillopsia
Nystagmus URGENT MRI
Without oscillopsia
Likely nystagmus present since childhood
Horizontal
Vertical
Saccadic Intrusions Direction
Rotatory
No slow phase
Neuro-Ophthalmology
Oblique
Saccadic movements only Circular
Children
Opsoclonus/Flutter
Neuroblastoma, encephalitis Pendular
Equal velocity each direction
Breast, lung, ovarian cancer Waveform
Adults Fast phase – direction named after this

MS, toxins, medications
Jerk Slow phase – actual pathological component
5.31 Nystagmus Null point = gaze location

Assessment – where minimal nystagmus
Located in gaze opposite fast phase
DWARFF component (Alexander's Law)
Large
Optic nerve hypoplasia Fundus Amplitude
Small
Effect of gaze position

Ocular Motility
Esotropia in congenital motor
nystagmus (CMN) Rest Primary position
Gaze evoked
Asymmetry Pupils
Iris abnormalities Frequency Fast
Slow
Good => CMN
Visual Acuity
Family History
Bad => retinal/optic nerve
abnormalities
Especially CMN
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112
Early onset
Age 2 mo
Initially may be pendular
Occasional vertical/rotatory Conjugate, horizontal jerk Congenital motor

nystagmus
Image if vertical/rotatory though aka infantile Extremes of horizontal gaze
Better with convergence, worse with fixation
Motor
Fast phase in direction of gaze
Null zone Nystagmus
Direction of gaze where decreased nystagmus
Physiologic Horizontal jerk Rarely lasts 3 beats
VA good, strabismus common
Beats away from covered eye One eye covered Low amplitude
Fast phase to fixing eye
Check with both eyes open also Latent Symmetric in eye & gaze
Visual acuity nystagmus
Otherwise latent nystagmus may
No other neurology
give false low vision
DVD Parkinson's
Associated Saccadic
Asymmetry of nasotemporal pursuit Intrusions
Cerebellar degenerations
Afferent visual pathway abnormality
PSP
Pendular most common Features 5.32 Nystagmus –

In adults, pendular = brainstem Multiple sclerosis
infarction or MS Congenital
Roving if VA <6/60
Periodic Alternating Albinism

Amaurosis/Achromatopsia/
Six As Nystagmus
Albinism/Aniridia/Atrophy
(optic)/Amblyopia Acquired => brainstem lesion
Media opacity
Leber's congenital amaurosis
Primary sensory retinal Sensory Prisms
Retinal dystrophy Defect e.g. if R head turn (left side gaze)
Nystagmus Kestenbaum–
Norrie disease Anderson move Treat as L XT for left eye – L LR
Vitreoretinal abnormality Treatment for All horizontal recti recess, MR resect
Familial exudative vitreoretinopathy (FEVR)
Surgery
Associated with albinism
And R ET for right eye – R MR recess, LR resect
Associated with aniridia Foveal hypoplasia
Bilateral recession/resection procedures
Isolated
Hypoplasia Oral medications, botulinum toxin

Coloboma Optic nerve disorder
Atrophy
Torticollis
Seesaw Dysconjugate TONOPEN Nodding

nystagmus
Seesaw = Ventral Midbrain
Convergence retraction (Parinaud's)
Pendular nystagmus
Latent nystagmus Cannot rule out intracranial tumors => MRI

Neuro-Ophthalmology
Lists
Spasmus nutans
Unilateral
SO myokymia nystagmus
Spasmus Nutans Craniopharyngioma
Internuclear ophthalmoplegia (INO)
Glioma of anterior visual pathway 5.33 Nystagmus –

Acquired –
Nausea MRI All
Vertigo Associated with
Part of Parinaud's dorsal Pineal region tumor

Sense of rotation midbrain syndrome
Vestibular
Inner ear disease
U – Upgaze palsy
Convergence Retraction
CN 7 disease – including nucleus in brainstem Causes Nystagmus = Dorsal Midbrain
C – Convergence retraction nystagmus
+ Interstitial keratitis => Cogan syndrome UCLA
L – Lid retraction
Arnold–Chiari malformation
A – Abnormal pupils – dilated,
light/near dissociation
Multiple sclerosis Cervicomedullar
junction lesion
Downbeat Nystagmus Alcohol intoxication
Infarction Gaze-Evoked Jerk Nystagmus
Wernicke's Cerebellar/brainstem disease
Lithium toxicity/Vitamin deficiency
Periodic Alternating Cerebellar lesions

Brainstem (pontine tegmentum) or Present in primary position Nystagmus
cerebellar vermis lesion Upbeat Nystagmus
Present in upgaze only

Drug effect
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114
Limitation symmetrical, common

Age-related
Neither eye can look in direction
Gaze palsy Horizontal CN 6 nuclear + MLF lesion

Voluntary
Bilateral riMLF in dorsal midbrain
Vertical
Doll head movements preserved
Extraocular muscles – CPEO
5.34 Abnormal Eye Neuromuscular junctions – myasthenia

Movements
Causes
& No Visual Diffuse ophthalmoplegia Miller–Fisher, carcinomatous meningitis
Cranial nerve
Symptoms bilateral disease
Pituitary apoplexy
Brain disease
Progressive supranuclear palsy
Chronic progressive external
Mitochondrial See next
ophthalmoplegia
Myopathies
Skeletal muscle impaired

relaxation after contraction
Presentation
Myotonic Teenage to young adults
dystrophy
Systemic
Frontal balding, temporalis wasting,
mental retardation, inability to relax grip
Dystrophic Myopathies EOM ophthalmoplegia

Ocular
Bilateral ptosis, polychromatic
"Christmas tree" cataract
AD/AR/Sporadic
Young adults to middle-aged adults
Oculopharyngeal Presentation
dystrophy
Dysphagia => ptosis => external
Symptoms ophthalmoplegia
Orbicularis weakness
Maternal inheritance
CPEO
Kearns–Sayre TRIAD Pigmentary retinopathy
Cardiac conduction block Refer cardiology –

Associations
Neuro-Ophthalmology
sudden death cause
Other signs Hearing loss, mental retardation,

cerebellar signs
Mitochondrial myopathy
Affects high Mitochondrial dysfunction => Encephalopathy

MELAS
metabolism tissue General
CNS, heart, EOM Lactic acidosis
Inherited
Stroke
Autosomal genes affect AR, AD, mitochondrial, sporadic
Hemianopia & cortical blindness
mitochondrial DNA & metabolism
No cure
5.35 Chronic
Progressive External Exposure keratopathy
Ophthalmoplegia
Medical & optical Coenzyme Q10 Improves exercise tolerance &
Ptosis + progressive symmetric cardiac function
external ophthalmoplegia
Prisms
No diplopia, anisocoria Diplopia or convergence insufficiency
Clinical
Strabismus
Treatment Surgery
No doll's eye movement
Disease involves muscle itself Ptosis
Check Bell's reflex – risk
exposure keratopathy
Downgaze Weak orbicularis + weak SR
Genetic counseling
Spared till late
Specialists
Cardiology in Kearns–Sayre
Biopsy
Short ragged-red muscle fibers Investigations
Abetalipoproteinemia
Pigmentary retinopathy +
Mitochondrial DNA testing neurological signs
Differential Refsum disease
RP, polyneuropathy, ataxia,
Diagnosis
hearing loss
Parinaud's dorsal midbrain syndrome

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116
Failure to dilate
Smaller pupil = abnormal Physiologic

Worse in dark
Pancoast tumor – Horner's syndrome
4 Ps
Penis pain (syphilis) – Argyll–Robertson pupil

Differential
Diagnosis Pilocarpine – pharmacological
Failure to constrict
Larger pupil = abnormal

Worse in light
Trauma – Iris sphincter damage
4 Ts
Tropicamide – pharmacologic blockade
Migraine 3rd nerve palsy
Tonic pupil
Accommodate but do not react
5.36 Anisocoria
Miosed pupil
Argyll–Robertson
Syphilis, DM, MS, alcoholism
Aberrant CN 3 regeneration
Dilated pupil
Adie tonic pupil
Light/Near Slow uneven light reaction
Dissociation
Bilateral optic neuropathy/
severe retinopathy Mid-dilated pupils
Poor light reaction, normal
convergence
Parinaud’s dorsal
midbrain syndrome Eyelid retraction, supranuclear
upgaze paralysis
Convergence retraction nystagmus
MRI
Pinealoma
20% population
2 mm or less
Not necessary if ptosis &
unequivocal worsening anisocoria in dark
Anisocoria same in light/dark & Critical Physiologic
normal light reaction
Blocks NAD uptake =>
Test Will dilate normal pupil
Both pupils dilate 10% cocaine blocks NAD
uptake at NMJ Cocaine 4–10%
CONFIRMED by CONTINUED
CONSTRICTION despite COCAINE
Neuro-Ophthalmology
Descending hypothalamic pathway
Brainstem stroke, tumor, spinal 1st order Stimulates NAD release from presynaptic NMJ
cord tumor, cervical spondylosis
Normal pupil
Ascending sympathetic trunk to Horner's Dilates
superior cervical ganglion Pharmacological Hydroxy-
Testing amphetamine 1% Preganglionic Horner's
Apical lung tumor = Pancoast Dilates
Tumor 2nd order
Thyroid adenoma, metastases No dilation as 3rd order neuron dead
Vascular – ICA dissection Causes Postganglionic Horner's
No NAD to release
Iatrogenic – previous lung/neck surgery
5.37 Anisocoria
Superior cervical ganglion => destination Worse in Dark: Postganglionic Horner's
Dilates – denervation sensitivity
ICA dissection Constricted = Phenylephrine 1%
3rd order –
Cavernous sinus tumor or postganglionic Abnormal Preganglionic
inflammation (Tolosa–Hunt) No dilation
Apraclonidine 0.5–1%
Iatrogenic Dilates Horner's
Denervation sensitivity
Prolactinoma, migraine
Muller's muscle Horner's
Light–near dissociation
Ptosis Syndrome
Small, irregular pupils
Lower lid higher Anhydrosis Does not equal tonic pupil or syphillis
Miosis Signs Lesion

Pretectal interneurons to EW nucleus
Pupil react to light Enophthalmos Dorsal midbrain
Accommodative reflex neurons spared
Lower lid elevation Syphilis
Argyll–Robertson Causes
Abnormal IOP Clinical Horny Pupil Can cause AR or tonic pupil
Lower IOP PAMELA !
Heterochromia Aberrant CN 3 regeneration
Congenital Horner's
DM, MS, alocholism
Facial or neck pain
Carotid dissection Clues Bilateral
Anhidrosis
Preganglionic lesion Miosis
Multiple nerve palsies Clinical
Cavernous sinus disease Light near dissociation
Orbital mass, cavernous sinus mass Speed of constriction & dilation is normal
Urgent CT & CTA Investigation Test
Urgent anticoagulation to
Orbit/Brain/Neck Cocaine 4–10%
prevent thrombosis
Carotid dissection Affected pupil does not dilate
Same day MRI/A
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118
Ciliary ganglion
Damage to
Short ciliary nerves
95% fibers for accommodation

Ciliary ganglion
Pathology
5% for light response
=> Any damage affects light response
Denervation supersensitivity
Iris sphincter to ACh
F:M 5:1, age 20–40

Primary = Holmes–Adie pupil
Areflexia, peripheral neuropathy
Causes Syphilis Holmes–Adie syndrome
Secondary Trauma, surgery, HZO
Sphincter damage, irregular pupil Tonic Pupil Lyme disease, HSV

Trauma
Sluggish reaction to light

Dilated pupil
Sluggish reaction to near
Clinical But better than light = light/near dissociation

IMPORTANT: slow re-dilation after constriction
5.38 Anisocoria
Worse in Light: Sector iris paralysis
Dilated = Abnormal
Cannot be overcome by Vermiform iris movements
pharmacologic agents
Pharmacologic
Blockade Supersensitivity => constricts
Pilocarpine 1% Test
At 45 min if not fully May be false-negative in acute Adie's
Pilocarpine 0.1%
constricted =>
Pharmacologic If negative – pilo 1%
blockade
If negative => pharmacologic blockade
Natural history
Constricted pupil – little old Adie
Pupil not widely dilated, still

reacts to light
3rd Nerve Palsy
Eyelid droop, diplopia

Isolated anisocoria
Lid & motility examination
Neuro-Ophthalmology 119
3rd nerve palsy

Yes
Anisocoria Horner's syndrome
Myasthenia
No
5.39 Ptosis Involutional (levator aponeurotic defect)
See Chapter 7 Oculoplastics Superior eyelid/orbital mass
CVA motor cortex, internal capsule

Supranuclear lesion
Contralateral weakness lower 2/3 face
Mild orbicularis weakness
Pontine infarction, demyelination,
in filtration by tumor
Nuclear (pons) lesion Sympathetic (Horner's) + CN 7

Associated symptoms
Contralateral hemiparesis
CN 5, 6, 7 & 8 affected
Underactivity CN 7 motor, sensory, autonomic functions
Cerebellopontine angle lesion
Unilateral LMN – no sparing upper 1/3
Acoustic neuroma, meningioma
Ramsay–Hunt syndrome, Zoster oticus
Geniculate ganglionitis Unilateral LMN – no sparing upper 1/3 Motor, sensory,

autonomic functions
5.40 CN 7 Vesicles in auditory canal
Disorders
Treatment
Acyclovir 800 mg 5x/d + ENT referral
Peripheral nerve lesion
Battle sign
Ecchymoses over mastoid
Overactivity Essential blepharospasm Temporal bone fracture
CSF otorrhea, hearing loss
Hemifacial spasm Sarcoid infiltration of parotid
Heerfordt's syndrome
Parotid tumor Gland enlargement, uveitis, CN 7 palsy
Specific conditions Bell's palsy
120
Ectropion, lagophthalmos
Epiphora
CN 7
Brow ptosis (secondary
dermatochalasis)
Corneal sensation
CN 5
Exposure & decreased sensation => blinding risk
CN 6
Most common facial neuropathy General Examination Diplopia, gaze palsy
Autoimmune or viral CN 8
Hearing loss, tinnitus
MRI – If Not Resolved by 3 Months Cerebellar signs
External auditory canal

ENT for all new CN 7 weakness
5.41 CN 7 – Bell's Anatomical
Parotid gland
structures
Palsy
Lymph nodes
Sudden onset paresis
Scars
Pain precede or concurrent
60%
Spontaneous
Examine auditory canal Diagnosis of Exclusion
recovery
Exclude Ramsay–Hunt
Start recovery by 2–3 wk
Complete by 2–3 mo
Partial recovery
Weakness progresses Natural History 20–25%
over 3 wk
Exclude neoplastic
etiology No recovery
GCA
15–20%
History
Old age
Malignancy
Poor prognostic signs Complete facial palsy
Hearing loss & tinnitus
Lacrimation impaired
Crocodile tears
Lacrimation when eating
Aberrant
Regeneration Simultaneous movement of
different CN 8 muscles Corner of mouth when eyes close
"Probably effective"
7–10 d oral if within
72 h of onset
Corticosteroids Grogan & Gronseth (2001)
Medical Therapy
1 mg/kg for 6 d, then taper over 4 d

Antivirals
Neuro-Ophthalmology
800 mg 5x/d HSV dose + steroids

Potential benefit
Brow Ptosis
Brow lift
=> Ptosis
Clinic trial sitting up – 0.6–1 g
Suture onto tarsus

Gold weight
5.42 CN 7 –
Underactivity – Upper lid retraction Decrease lagophthalmos
Aim
Anesthetic cornea Treatment
Without ptosis in primary
Botox tarsorrhaphy
Steroids
Exposure Paralytic ectropion
Keratopathy
Antivirals
Lateral tarsal strip +/– tarsorrhaphies
Lubrication Acute
Neurotrophic cornea
Perforation, infection risk
Tarsorrhaphy High risk cases
A SALTy BREW
Dense palsy
Brow ptosis Poor prognosis
for recovery Parotid tumor resection
Retraction – upper lid
Failure to resolve Ramsay–Hunt
Chronic
Ectropion – lower lid Cause

Overproduction/lacrimal block/pump paralysis
Pump failure
Lubricants +++
Watery eye
Aberrant regeneration Epiphora Tape eyelid at night
Treatment
Correct lid position
Exclude obstruction
Lester–Jones tube if lacrimal pump does not work
Botox lacrimal gland

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122
MRI Brain & To exclude tumor

Cerebellopontine Angle Unilateral, facial muscles also involved
Atypical blepharospasm
Present during sleep
Uncontrolled blinking/twitching/eyelid closure
Idiopathic
General
Bilateral – initially may be unilateral
5.43 CN 7 –
Overactivity Disappears during sleep
Reflex
Hemifacial spasm Unilateral contractors Present during sleep

Essential entire side of face
Blepharospasm
Differential Eyelid myokymia Eyelid twitch secondary to stress,
diagnosis caffeine, ocular irritation
Tourette syndrome
Trigeminal neuralgia
Acute pain episodes
Tardive dyskinesia
Antipsychotics, Parkinson's disease
Treatment
Botox type A into orbicularis
Unilateral spasm
Facial musculature – seconds to minutes
General
Hemifacial Spasm
Secondary Tumor
Compression
Vessel at cerebellopontine angle
Botox type A into facial muscles

Repeat 2/12
Migraine history, family history
History
Exacerbating factors Menstruation, pregnancy, stress,
foods, lack of sleep
Zigzag, shimmering, colorful

Aura Peripheral => central => breaks up
Tumor, aneurysm
Neuro-Ophthalmology
Classic migraine 30% – aura

Compression of CN 5 Visual field defects – anopia
Ectatic arterial loop
Persistent neurological deficity
Todd's paresis
Constant, ache, electric shock Types
Unilateral V2 & V3 pain Common migraine 65% – no aura
Hours to days
Chewing, brushing
Facial
Trigeminal Usually <60 min Scintillating scotomata,
Facial/corneal sensation decreased Clinical
Compressive neuralgia visual field constriction
Migraine aura without headache 5%
tumor signs Visual migraine
Ptosis, anisocoria
CN 6 / 7 / 8 palsy – Transient homonymous hemianopia

cerebellopontine angle lesion Migraine New onset >50 yo
History
Medical Immunosuppression, cancer history
TCAs, carbamazepine
Treatment One side always
Criteria to
Surgical investigate/refer
Neurosurgical decompression
Headache precedes aura
5.44 Migraines,
Headaches & Migraine Neurologic deficit persists after aura resolves
Severe, ipsilateral orbital/ Facial Pain
Atypical aura
supraorbital/temporal pain Age 20–40, M>F >1/d, lack of expansion/change in aura
15 min – 3 h/d, 1–8 x d Change in migraine pattern
NSAIDs
Restless c.f. migraine patients who stay still
Serotenergic agents
Acute Contraindicated in coronary artery disease
Clusters for weeks & remissions Clinical
for months–years Cluster
Headaches Topiramate
Treatment Antiepileptic medication = GABA agonist
Horner's syndrome
Avoid triggers
Conjunvtival injection, lacrimation, Ocular Prophylactic
eyelid edema Beta blockers, TCAs, SSRIs, sodium
valproate, topiramate
High flow oxygen

Acute
Subcutaneous sumatriptan Treatment
Prophylactic
Up to 960 mg/d
Verapamil
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124
Sudden hemorrhagic infarction

a/w Tumor
Equivalent to cavernous sinus syndrome
Clinical Sudden severe headache, decreased consciousness

Pituitary Apoplexy
Monocular/binocular vision loss
Ptosis & ophthalmoplegia

Diplopia
Imaging
5.45 Head & Facial
Management Endocrine assessment + steroid replacement
Pain – Intracranial or
Systemic Pathology
Neurosurgical decompression
Spontaneous
Causes a/w Marfan's syndrome
Secondary
Head/neck trauma
ICA Dissection
Pain + Horner's syndrome

Clinical
Visual loss – ischemic optic neuropathy
Oculomotor cranial neuropathies
Treatment
Anticoagulation – heparin then warfarin 6/12
GCA
See separate
Raised ICP
See separate
Checks cooperation
Non-visual tasks
Finger touching, writing
Pupillary reaction
Bilateral – testing Optokinetic nystagmus drum Eyes move but patient "sees
nothing" => functional
Neuro-Ophthalmology
Mirror test Move mirror side to side
Check for eye movements

Shock value
Blindness All above tests
Afferent pupillary defect

Visual complaints with no Induces inward shift of eye
physiologic/organic basis Definition Base-out prism test
Monocular – testing Movement indicates vision
"There is no organic cause for Medical notes Vertical prism dissociation test
patient's symptoms"
Stereopsis test
Titmus test
Afferent Visual
Elicit normal vision Fogging – trial frame on patient
Pathway
Assessment Confusion test
Elicit inconsistent responses Red–green test – red lens on non-seeing eye
Fits faker profile Monocular

Confusion tests – fogging, red–green duochrome
5.46 Functional
Malingerer Visual Disorders Bottom-up acuity
6/3 first, then larger letters at 6/6
Binocular
Somatizer Trial frames
Adult Visual aids
Hypochondriac People
Tell patient it has magnifying properties
Hysteric (conversion)
Reduced vision Alternative chart
Use if monocular defect that respects midline
Children Automated perimetry
Pressure to perform, sibling rivalry, abuse
Test individual eyes then binocular
Ocular motility & alignment Visual field defect Defect present in binocular => functional
Other Categories Non-field testing

Pupils & accommodation Map out defect, then test saccades
of Involvement
Confrontation testing
Eyelid position & function
Goldmann perimetry
Spiraling inward in functional
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126
Age 15–45, one eye only

1/4 MS patients
Acute onset VA blur, worsens over hours to days Optic neuritis 1st presentation
Positive
Pain on eye movement 1 or more MRI white matter lesions
90% of patients History
Higher risk of MS Previous optic neuritis/
No cancer, vasculitis, autoimmune disease Prognosis neurological symptoms
Negative
No diplopia, no systemic neurological symptoms Family history
Male No light perception

RAPD Lower risk of MS
Nil, mild, moderate swelling Atypical findings No pain
Optic nerve head Positive
2/3 normal Peripapillary hemorrhages,
Other eye normal MRI normal retinal exudates
Examination
Nonhereditary
Typical Optic
No disc pallor, hard exudates, CWS Neuritis
Pathology Demyelination & inflammation
No hemorrhages, iritis, vitritis Negative
Autoimmune – CNS Oligodendrocytes
No intraocular diseases “white matter” destroyed
5.47 Demyelinating Sensory/motor disturbance

Affected eye Optic Neuritis
Any visual field defect Perimetry Ataxia, dysarthria, dysphagia, visual disturbances
Symptoms
Unaffected eye Fatigue, chronic pain
Asymptomatic field defect
Stops worsening Bladder/bowel incontinence

Within 2/52 Follow-up
Vision Especially in
Starts improving >2 lesions on T2-weighted MRI periventricular region
Within 4/52
Multiple Either new T2 lesions
Sclerosis Presence of >1 Gd-enhancing
Diagnostic criteria lesions at baseline
Or new Gd enhancement on
3 mm lesions NOT required for DIAGNOSIS follow-up scan
T2 = old lesions Most people develop first 5 y

Periventricular, ovoid, radiating
Required for PROGNOSIS
toward ventricles
& INTERFERON Rx MRI Suggestive of demyelination attack
T1 with contrast = new lesions
Investigations Clinically isolated syndrome Does not fulfill MS criteria
Advise MRI for all
May benefit from immunomodulators
Relapsing–remitting 80% 30–70% develop MS
Course
MRI abnormal => LP does not help Lumbar puncture Secondary progressive
Oligoclonal bands Initially relapsing–remitting
MRI normal => LP => If abnormal Primary progressive
Visual evoked potentials
Higher chance MS Progressive relapsing
Nerve damage gradually worsens
Decreases 2 y recurrence
IV steroids
Exacerbations
Does not alter prognosis
Reduces attacks by 30%

Benefits Slows cognitive decline
Lessens disease activity on Gadolinium-enhanced MRI

Neuro-Ophthalmology
Disadvantages
Flu-like symptoms, depression, cost
IFN-beta1a IV steroids & taper as per ONTT

Optic neuritis treatment trial (Avonex) First episode
Weekly IFN-B-1a
Study – CHAMPS
22% with 0 lesions
At 10 y Treatment
Rates of conversion to
56% with one or more lesion clinically definite MS
37% vs 28% placebo
25% with 0 lesions MRI prognostic indicator
At 15 y Study – ETOMS Works without steroids
Interferon alone
72% with one or more lesions
Number of lesions Study – BENEFIT Interferon => Decreased

Does not increase risk significantly 5.48 Demyelinating clinically definite MS rate
Optic Neuritis 28% vs 45% placebo
Recovery >6/12 – Management
91% Fingolimod (Gilenya)
Daily oral tablet
After 10 y VA >6/6 Controversial – chronic venous insufficiency

74% Other
Visual acuity
ONTT Glatiramer acetate SCI daily
Brightness, stereopsis, VF & color Residual deficits
vision abnormalities
Natalizumab monthly IV Progressive multifocal
leukoencephalopathy (JC virus) + death
25% recurrence
No long-term benefit
VA & contrast at 6–12/12
Speed recovery by 1–2/52

IV methylprednisolone
Protects recurrence for 2 y 250 mg 6/24 for 3/7 Steroid
treatment Follow-up
Regime Confirm diagnosis
No benefit by 3 y 2/52 & 4/52
Followed by 1 mg/kg/d for 11 d
Oral corticosteroids
No benefit, increased recurrence rate
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NMO = Devic's Important to diagnose if recurrent optic neuritis

disease CNS lesions become permanent
MS Rx beta-interferon is harmful in NMO
Decrease neurological function
MS
Demyelinating CNS
Selective to optic neuritis & spinal cord
Inflammatory disease
Aquaporin receptors present more
at ON & spinal cord
Older – mean age 39 y
MS vs NMO
Multiple sclerosis NMO Sex – female 85% – same as MS
Relapse in pregnancy
Brainstem stroke MLF lesion Asian predisposition
Associated with
Brainstem mass lesion Myasthenia, SLE, anti-phospholipid syndrome
Neuromyelitis Relapsing disorder
Optica (NMO)
Affected side
Optic neuritis More than 1/2 relapse within 6/12
ADDUCTION paralysis
Myelitis
Spinal cord
Contralateral eye Clinical
Horizontal jerk NYSTAGMUS diagnosis Contiguous spinal cord lesion >3 vertebrae
Brain lesion not meeting Atypical brain lesions Large confluent

CONVERGENCE adduction intact Clinical 5.49 Demyelinating Supportive MS criteria lesions >3 cm
criteria Diencephalic lesions
Optic Neuritis –
Bilateral
INO & NMO Preventricular ovoid lesions
Wide-Eyed Bilateral INO WEBINO Serology positive
with exotropia NMO-IgG aquaporin-4 antibody
Sensitivity 50–80%
Serology
NMO-IgG Aquaporin-4 Antibody Specificity 90–100%
INO one side + conjugate Investigations Predicts relapse & conversion to NMO
horizontal gaze palsy
Internuclear >50 WCC – especially during attacks
Ophthalmoplegia (INO) Lumbar puncture
PPRF & both MLF damage Negative oligoclonal bands
Brainstem stroke RNFL thickness
OCT
One-and-a-half NMO becomes thinner than MS
syndrome Steroids
Ipsilateral esotropia Acute
For acute attack, as per ONTT
CN 6 nucleus
+ Plasma exchange
Signs Azathioprine
Treatment
Long-term Disease modifying therapy
Only horizontal eye movement is Rituximab
reduce relapses
abduction contralateral eye
Mycophenolate mofetil,
More severe long-term prognosis mitoxantrone,
IV Ig, cyclophosphamide
Sleep-rest
Patient rests 30 min
Myasthenic crisis Life Threatening
Cold => less ACh breakdown
Exclude CN 3 palsy – check pupils
Apply 2 min
Ice pack Improvement 2 mm or more
Immunological weakness disorder
Useful for ptosis only
Pathology
Damaged by antibodies Sensitivity/specificity similar to Tensilon
Neuro-Ophthalmology
Acetylcholine (Ach) receptor at

NMJ of striated muscle
Short-acting ACh-esterase inhibitor
Prevents striated muscle function General
IV 2 mg test – 60 sec
20% spontaneous remission Improvement => positive test
Protocol 4 mg then 4 mg again
1–2 y 66% of ocular MG progress to Natural history Endpoint
Ptosis => elevation
generalized MG
Edrophonium Bradycardia, bronchospasm, respiratory arrest
Diagnosis chloride
Serious Monitor BP, oxygenation, pulse,
(Tensilon)
Side effects resuscitation equipment
Fluctuant & fatiguable Atropine 0.5–1 mg IM if bradycardia
Worse in evening, exertion, upgaze
SLUDGE Salivation, Lacrimation, Urination,
Hering's law – more ptotic lid lifted => Ptosis GI motility, Emesis
Opposite lid falls Heart disease

Lid hopping 5.50 Myasthenia Contraindicated
Gravis Respiratory disease
Mimic any cause of strabismus
Ocular motility/diplopia Urinary retention/prostatism
Changing pattern => myasthenia
Long-acting ACh-esterase inhibitor
Cogan's twitch
IM, 45–60 min
Over elevation of eyelid on saccade Ocular 50% Children/adults
Clinical Neostigmine
from down to upgaze
Indication Poor vein access
Diplopia, without ptosis

No pupil abnormality
Pseudo-INO
Cholinergic side effects
Give mixed with atropine
No sensory deficit
Relevant negatives D – Drugs
Aminoglycosides, penicillamine (immunosuppression for RA)
Orbicularis affected in MG
E – Eaton-Lambert
c.f. CN 3 or Horner's a/w Lung Ca
Muscle strength increases after exercise
Differential
Dysarthria, dysphagia, dyspnea Diagnosis – CPEO
DEMON M – Mitochondrial Pigmentary retinopathy, CPEO
Bulbar 20% Kearns–Sayre
Weakness – muscle mastication, extensors neck/trunk/limbs Mitochondrial
O – Oculopharyngeal dystrophy
Dysphagia, ptosis
N – Neuromyotonic dystrophy
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Consider no treatment
Neurologist Mild, purely ocular
Swallowing, Breathing, Speaking
Plasmapheresis Trouble with SBS General Acetylcholinesterase inhibitor

Urgent hospitalization
+/– Ventilatory support 30 mg bd => 60 mg tds
Pyridostigmine (Mestinon)
Medical SLUDGE side effects
Aminoglycosides/Neuromuscular blocker/Ciprofloxacin
Avoid ABCDPP drugs
Respiratory depressants/Procainamide/Penicillamine Generalized MG, not purely ocular
5–100 mg, low initiation dose Risk myasthenic

Malignant thymomas
C – CT scan respiratory crisis
10% MG patients => scan all Corticosteroids
Increase then wean after 1–3/12
5.51 Myasthenia
ACh-R Ab
Gravis – Immunosuppressants
Acetylcholine receptor binding antibodies
Treatment Azathioprine 1–2 mg/kg/d
Thyroid
TSH, thyroid antibodies
If ACh-R Ab negative Thymectomy

MuSK A – Antibodies
Surgical Generalized MG with thymic enlargement
Antimuscle specific kinase
Investigations
Ocular myasthenia CASE Ptosis & strabismus surgery – rarely useful
50% of patients
Positive in
Generalized myasthenia
90% of patients 4–6/12 for 2 y
If stable => 6–12 monthly
S – Serology Isolated ocular
Thyroid, SLE coexistence Follow-up
No progression to systemic by 2 y
Repetitive stimulation at 3 mHz => unlikely to progress
E – Electromyographic
Variability, diminishing Single-fiber EMG
response, jitter
Vision & pain
Thyroid, autoimmune disease
Antibodies to TSH receptors & Autoimmune inflammatory disorder Medical
orbital fibroblasts History Steroids contraindication
e.g. DM, HTN, PUD
Lymphocyte infiltrate => water influx Social
Pathology Smoker
Soft tissue/EOM swelling
Adipogenesis Medications
Neuro-Ophthalmology
Radioactive I => can make TED worse

Chronic inflammation => fibrosis
Low TSH
Hyperthyroidism
90% TED are Graves Raised free T4 & T3
Abnormal in 90% TED
20% of Graves Hypothyroidism Associated with General
patients have TED Thyroid function Raised TSI – thyroid stimulating Igs
Hashimoto thyroiditis tests
Anti-TSH
Euthyroid Graves disease 95% Graves, > 50% TED
Other
Epidemiology Anti-TPO
Visual fields 80% Graves, 90% Hashimoto
Middle aged, F>M 6:1
5.52 Thyroid Vision Color vision Antithyroglobulin
Course of TED independent of Eye Disease
systemic disease 25% Graves, 55% Hashimoto
(TED) Electrophysiology
General Hess chart

Body habitus, neck scars, facial asymmetry Motility
BSV
Position (head)/Ptosis
(or retraction)/Proptosis Investigations 90% Graves have CT findings
6 Ps
Periorbital (inflammation)/Pupils/Prisms Avoid contrast in acute phase
Eyelids exam
Proptosis
Line through lateral orbital rims
Motility exam Examination
Enlarged muscle
VA, CVF, pupils, Isihara, disc exam
Optic nerve function CT orbits with Diagnosis Tendon sparing
EOM
contrast Inferior, Medial, Superior,
Optic neuropathy signs
diagnosis & Order – I'M SLO
Lateral, Oblique
severity
Compressive or glaucomatous
Exposure keratopathy Lacrimal gland/orbital fat enlargement
Anterior Apex – coronal section
Severity Check optic nerve compression
IOP – primary & upgaze
Slit lamp
Bones/sinus – history of decompression
Posterior
Disc pallor, shunts, choroidal folds, venous disease IOID – tendons not spared
DDx
Orbital masses
Carotid–cavernous fistula – with contrast

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N 1. Retrobulbar pain
No signs
DOLOR – Pain
Retraction, stare O 2. Gaze-evoked pain
Only upper lid signs
+/– Lid lag, lagophthalmos 3. Eyelid
Initial activity
S 7 points RUBOR – erythema 4. Conjunctival
Soft tissue involvement
NO SPECS 5. Caruncle & plica
P
Proptosis/pressure Clinical Activity 6. Eyelid
Score EUGOGO
E TUMOR – swelling
Extraocular muscle involvement 7. Conjunctival chemosis
C M – Motility
Corneal involvement Decrease uniocular excursion >8 degrees
Follow-up
V – Visual acuity
Sight loss due to optic nerve S 3 more points Decrease by 1 Snellen line
involvement 5.53 TED –
P – Proptosis
Activity & >1 mm increase proptosis
Severity >2/7
V
Active TED
Vision Score
I >3/10
Inflammation Will respond to immunosuppression
Canadian VISA
S Grading Exposure keratopathy
Strabismus Severe sight-threatening
Optic nerve compression
A
Appearance (exposure) Lid retraction >1 mm
Initial worsening then partial resolution Exophthalmos >2 mm

Moderate – severe
Severity
Diplopia
EUGOGO
Dynamic
Severity Active signs
phase
GO Static Rundle Curve Active TED
phase Immunosuppression
Runde’s
curve Treatment
Inactive TED
Surgery
Mild
Rarely require steroids
Time Risk factors
Age, male, Caucasian, age of onset, smoker
IV methylprednisolone More effective, more side effects
Systemic 1 g/d pulses, <8 g
Stop smoking
Carbimazole, steroids 500 mg/d, 2x/wk, over 8 wk
propylthiouracil, Antithyroid drugs
propanolol, steroids Oral prednisolone 1mg/kg Taper over 3/12
Endocrinologist –
Restore
Radioactive iodine131 SE
Euthyroidism Diplopia in inflammatory phase
Worsens TED
Indications Compressive optic
If poor steroid response/
Neuro-Ophthalmology
Subtotal thyroidectomy
neuropathy unsuitable to decompression
However treatment does not affect TED
Active Inflammation + 10–20 Gy over 2–20 wk
C Moderate–Severe
Features Onset 1–3 mo
Compressive optic neuropathy Progressive Signs Radiotherapy
General
SEA CAGE May worsen TED => give with steroids
Active inflammation + A CODE
Moderate/Severe/Progressive signs
Acute – A CAGE No benefit shown
G Gorman, Prummel, Bradley studies
Glaucoma
Evidence Will not work if steroids don't work
E
Exposure keratopathy
Steroids + radiotherapy more
O effective than either alone
Orbit 5.54 TED – Acute
Chronic – CODE
Management Azathioprine, cyclosporin
D Immunosuppression
Diplopia
E
Eyelids Manage corneal ulceration/perforation
Admit
Lubricants
48 h to take effect
IV methylprednisolone Moisture chamber
Cumulative dose < 8 g 1g/d for 3/7
Exposure
Systemic Tarsorrhaphy
– liver damage Keratopathy
steroids Suture or Botox
Active phase
Rarely Systemic steroids/Radiotherapy/
Then prednisolone oral 1 mg/kg/d Orbital decompression
Rarely
Steroid unresponsive 1–2/52/ Compressive Orbital decompression
Significant side effects Optic Inactive phase IR recession
Neuropathy
Lid retraction surgery
2-wall
Medial + lateral walls Topical/oral antihypertensives
3–6 mm benefit
Orbital
Medial + lateral + floor decompression IOP control Avoid trabeculectomy
6–10 mm 3-wall benefit: 2 mm/wall Risk of bleb exposure
Glaucoma
Tube/posterior bleb
+/– Fat decompression rarely
4-wall Orbital decompression
10–16 mm Orbital congestion Useful if IOP >21 mmHg
+/– Endoscopic Strabismus surgery
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Primary gaze
Identify muscles involved
HESS/BSV chart
Maximize field of binocular single vision

Aim
Align eyes for cosmesis
Prisms
Not in fibrosed state
Options
Botox
Stable TED >6/12

No optic neuropathy
1. Orbit Surgery indications Stable systemic thyroid state >6/12

Surgery Order 2. Diplopia
Chronic – ODE Not requiring orbital surgery
2. Diplopia
Never resect
3. Eyelids General Adjustable sutures
Multiple operations may be required

5.55 TED –
Chronic IR restriction <30 PD
2-wall, with steroid cover Orbital decompression +/– Management Ipsilateral IR recession
Surgery
endoscopic IR restriction >30 PD
Bilateral IR recession
Infection, CSF leak, meningitis Esotropia
MR recession
Orbital/Intracranial hemorrhage 1. Orbit –
Proptosis IR restriction + esotropia
IR + MR recession
Loss of vision Acute
Stable TED >6/12
Infraorbital hypoasthesia No optic neuropathy
Surgery indications
Damage to eyelids, muscles, eye Complications Stable systemic thyroid state >6/12
Severe
3. Eyelids Levator recession/disinsertion
Hypo-ophthalmos – floor Upper lid retraction
Mild
Chronic Mullerectomy
Diplopia/eyelid changes
Lower lid retraction >2 mm LL retractor recession/
spacer graft
Belpharoplasty/browplasty Ear cartilage, hard palate

Compressive optic neuropathy
Neuro-Ophthalmology
Severe exposure keratopathy

Indications
Progressive glaucoma
+ Failed/contraindicated steroids/radiotherapy
Cosmesis
Infection
5.56 TED – Wound, CSF => meningitis
Orbital Decompression
Hemorrhage
Orbital, intracranial
Acute
Loss of vision
Nerve
Infraorbital hypoasthesia
Complications
Damage to lids/EOM/eye
Hypo-ophthalmos (floor)
Diplopia
Chronic
Ptosis/Retraction
Lid
Ectropion/Entropion
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Headaches & papilloedema

Superior sagittal
sinus thrombosis
Cerebral Venous & Dural
MRI in men/slim women if Venous Thrombosis
considering IIH
Thromboembolic
Congenital, familial, age <30 y Carotid System CRAO/BRAO

Disease Vasculitis
Seizures, headaches, other

neurological symptoms Arteriovenous Other Hypercoagulability, vasospasm
Malformations
(migraine), carotid artery dissection
90% supratentorial
Ataxia, imbalance, staggering
Carotid-cavernous fistula
5.57 Cerebrovascular Neurological
Post-trauma Disorders Vertigo
symptoms
Headache Vertebrobasilar
Disease Hemiparesis, hemiplegia, hemisensory
Ipsilateral ophthalmic signs Carotid artery
Contralateral neurologic deficits Bilateral blurring
Vision
Headache Homonymous visual field changes
& no neurological features
Basilar artery Posterior circulation
Brainstem & cerebellar signs involvement
Arterial Dissection
Cranial nerve palsies
CN 3 palsy with pupil
Cerebral Aneurysms involvement + headache
MRI
Surgery Age <50 y

Extracranial proximal ICA
Anticoagulants
Treatment
Cannot approach surgically

Vertebrobasilar
Can do bypass procedures

Mass effect Glioblastoma multiforme – Grade IV
Seizures, dementia, focal lesions Presentation astrocytoma
Most common
Majority supratentorial GBM
Well circumscribed, General Cerebral hemispheres Can cross corpus callosum =>
at gray–white junction Adult tumors butterfly glioma
>50% metastases
Poor prognosis
Childhood tumors <1 y
Majority infratentorial 2nd most common
Neuro-Ophthalmology
Arise from arachnoid cells

Neoplastic Meningioma external to brain Convexities of hemispheres,
Metastases
parasagittal region
Ring-enhancing lesion Adults Resectable
Abscesses Primary
3rd most common
Tumors
Infective
Toxoplasmosis
Schwann cell origin Often localized to CN 8 =>
Schwannoma acoustic schwannoma
AIDS lymphoma
Resectable Usually at cerebellopontine angle
Tumor
Lymphoma
Differential Oligodendroglioma
Meningioma Diagnosis Rare, slow growing, frontal lobes common
Uniformly enhancing lesion
Hypo-/Hyperpituitarism sequelae
Metastases
Commonly prolactinoma
Usually ring enhancing Pituitary adenoma Bitemporal hemianopia
Heterogenously enhancing lesion
Glioblastoma multiforme 5. 58 Brain Surgery
Good prognosis
Lesions
1% of population
Endocrine control
M:F 2:1
Secondary hydrocephalus Well circumscribed

Low-grade astrocytoma
20% of patients Pilocytic
Arachnoid cyst Often posterior fossa
Non-enhancing, CSF intensity Benign, good prognosis
Asymptomatic, no treatment commonly Highly malignant Primitive neuroectodermal tumor

Epidermoid cyst Non-
cerebellar tumor
neoplastic Radiological Medulloblastoma
Can compress
Chronic subdural hematoma Cyst Differential
4th ventricle
Large cisterna magna Diagnosis Hydrocephalus
Mega cisterna magna Children Commonly in 4th ventricle
Primary Ependymoma Hydrocephalus
Neurenteric, neuroglial, Tumors
porencephalic Poor prognosis
Pork tapeworm
Taenia solium Cerebellar
Neurocysticercosis Other cysts
CSF serology Hemangioblastoma Can produce EPO => polycythemia
Hydatid cysts von Hippel–Lindau syndrome

Parasite larval stage Associated retinal angiomas
Echinococcus granulosus Pilocytic astrocytoma Benign childhood tumor
Neoplastic Craniopharyngioma DDx pituitary adenoma
Hemangioblastoma Most common childhood
supratentorial tumor
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138
Both autosomal dominant, variable expressivity

Neurological, ocular & cutaneous features
Definition Chromosome 17q11, 1:4,000
Develop tumors
Hamartomatous type Café au lait spots >5
Vascular abnormalities Axillary, inguinal freckling
Rare, sporadic
Sturge–Weber/Wyburn–Mason Neurofibroma Solitary >2 or plexiform
Tumors present from birth
Eye – Lisch nodules >2 Present 10% per year of life
Chromosome 3p, VHL gene mutation, AD
Type 1 Any 2 of
Skeletal Bowing legs, dysplasia of greater wing sphenoid
Ocular CAFESPOT
Retinal capillary hemangioma
von Hippel–
Hemangioblastoma Lindau Pedigree – positive family history
1st degree relative
Neurofibromatosis
Cerebellum, spinal cord, brainstem OT – Optic nerve tumor
Renal cell carcinoma
Systemic Glioma
Pheochromocytoma Choroidal nevi
Other
Islet cell carcinoma Retinal astrocytoma
Epididymal cysts/adenomas Chromosome 22q12, 1:40,000

5.59 Phakomatoses
Visceral cysts PSCC
Ocular
X-Linked dominant Type 2 Combined hamartoma of RPE & retina
Xq28 NEMO gene
Gliosis Acoustic neuroma
Neovascularization & tractional retinal detachment Ocular
Incontinentia Meningioma
Foveal hypoplasia pigmenti
Systemic Glioma
Skin
Pigment whorls Schwannoma
Systemic First-degree relative with NF-2

Seizures Sporadic inheritance
CNS
Cerebral atrophy Episcleral/ciliary body/iris
Hemangiomas
Ocular
Dwarfism, skull deformity Sturge–Weber Choroidal hemangioma (diffuse) Venous congestion, raised EVP
1:6000, Chromsome 9q or 16p, AD TM dysgenesis

30% of patients due to
Glaucoma
Retinal astroycytoma Ciliary body angioma
Ocular Lifelong monitoring
TSC1/2 mutation – hamartin & tuberin Glaucoma
Adenoma sebaceum Tuberous aka port-wine stain, cavernous hemangioma

Sclerosis Nevus flammeus of face
Ash-leaf spots, shagreen patches, subungual fibromas Systemic
CN 51 & 52, with hypertrophy of face
Cerebral astrocytomas Systemic CNS hemangioma
Epilepsy, low IQ
Wyburn– Retinal AVM Epilepsy, hemiparesis, hemianopia
Visceral hamartomas Mason Ocular
Orbital/periorbital AVM
Cardiac rhabdomyoma, renal angiomyolipoma Visceral cysts
Systemic
Pulmonary lymphangioleiomyomatosis Cerebral/brainstem AVM
CSR
HTN retinopathy
Retina
Retinal detachment
Neuro-Ophthalmology
IOP
Decreased 2nd half & postpartum Artery/Vein occlusions
Change in refractive error Optic nerve

Cornea Ischemic optic neuropathy
Pre/Eclampsia Risk
Physiological
Increase thickness
Changes
? Cause decreased IOP Disseminated intravascular
coagulation
Visual fields Coagulation Choroidal infarcts
Bitemporal contraction
Cerebral venous thrombosis
Accommodation
Transient loss
5.60 Pregnancy
& the Eye
Cortical blindness
Secondary to occipital lobe edema
Diabetes
Graves disease
Uveitis Conditions Improved
Due to immunosuppression over Pregnancy
Uveal melanoma
Systemic Multiple sclerosis

Conditions Worsened Optic neuritis
Arterial
2nd trimester to 1st week postpartum
Vessel disease
Venous
First 6 wk postpartum
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6 Oncology
Oncology
6.1 Eyelid Tumors

6.3 Anterior Non-Pigmented Lesions
6.2 Conjunctival Tumors
Anterior 6.4 Conjunctival Melanoma
6.5 Iris Tumors
6.6 Ciliary Body - Melanoma + Other Tumors
6.7 Choroidal Tumors

Choroid 6.8 Choroidal Melanoma
6.9 Choroidal Melanoma – Treatment
Posterior
6.10 Retinal + Retinal Pigment Epithelium Tumors
6.11 Primary Intraocular Lymphoma (PIOL)
6.12 Retinal Hemangioma
6.13 Paraneoplastic Syndromes
6.14 Orbital Tumors – Benign vs Malignant
6.15 Heme-Oncology
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Nodular/Solid/Cystic
Basal cell carcinoma Sclerosing/Scirrhous
Epidermis Adenoid
Malignant Differentiated
Sudoriferous Squamous cell carcinoma
Double cell wall layer Undifferentiated
Cysts Nodular
Epidermoid Sebaceous gland carcinoma
Epithelial inclusion Adnexal
Dermoid Diffuse intraepithelial spread
Xanthelasmas
Metastasis
Junctional Benign
Compound Nevi Cutaneous melanoma
Intradermal Kaposi sarcoma
Basal cell papilloma/seborrhoeic keratosis Merkel cell tumor

6.1 Eyelid Tumors
Squamous cell papilloma Benign epidermal Malignant (Rare) Lymphoma
Papillary or solid
Keratoacanthoma
Lacrimal sac Squamous cell carcinoma
Transitional cell carcinoma
Lymphomas
Premalignant
Metastatic placental tissue
Actinic/solar/senile keratosis Skin
Capillary hemangioma Glaucoma, thrombocytopenia
Retinal
Hamartoma Cavernous hemangioma
Soft Tissue
Lymphangioma
Neurofibroma
Choristoma
Oncology
Epithelial inclusion cysts

Lymphatic cyst/Lymphagiectasias
Hamartoma
Angiomas
Choristoma
Dermoid
Benign
Junctional
Nevi
Intrastromal
Compound
Amyloid
Pedunculated
Papillomas
Conjunctival/Corneal
Sessile
HIV patients intraepithelial neoplasia
Kaposi Sarcoma
Treatment Leukoplakic
If cosmetic, bleeding or infected
CIN Types Papilliform
Epithelial based
6.2 Conjunctival
Gelatinous
Tumors
Squamous cell carcinoma
Pre- & Malignant
Primary acquired melanosis
Benign reactive
lymphoid hyperplasia PAM
Mixed cells Mucosa-associated Lymphoproliferative Melanocytic neoplasia Benign
lymphoid tumors Mild
Malignant lymphoma Severe
Monoclonal
Melanoma
Vascular pannus
Angle invasion
Sclera invasion
VAST PECS – Higher Risk Thickness
Pigmentation
Extension to cornea
Cosmesis
Size
143
Smoking
144
UV
Risk factors Petroleum product exposure
HPV 16 & 18
Xeroderma pigmentosa Leukoplakic
Human papilloma virus
Cause 3 forms (LPG) Papilliform
UV, mechanical irritation Epithelial Gelatinous
Conjunctival intraepithelial
neoplasia (CIN) Fixed/tethered to sclera
Pedunculated
Fleshy, exophytic, fibrovascular core High SCC risk
Types Feeder vessels
Higher chance neoplasia Sessile Fimbriated margins, granular sheet like
Corneal intraepithelial neoplasia (CIN) Features
Flat base, red dots Rose-Bengal or Lissamine green stain
Leukoplaia Bigger margins 4 mm
Treat as CIN but
Papilloma Squamous cell carcinoma (SCC)
Symblepharon Always drops postoperatively
Dysplasia signs Poorer prognosis
Invasion Drops/XRT primary treatment
>180–270 deg
Inflammation Size & extent
Surgery => limbal stem cell failure
Examination Angle, lymph nodes, perineural sensation
Topical interferon
Medical Photography
Mitomycin C Tells dysplasia, but cannot between CIN & SCC
Treatment Impression cytology
Only if suspect malignancy 6.3 Anterior Excisional biopsy
Surgical No-touch technique
No-touch technique Non-Pigmented
Cornea
Lesions Dilate, alcohol, do not breach Bowman's
Treat body Limbal/Conjunctival
Surgery 4 mm margins
Most common: reactive lymphoid
hyperplasia, most B-cell +/– Scleral bed resection If fixation/tethering
Management of Otherwise cryotherapy sufficient

Field sterilization, cryotherapy
Salmon patch Treat roots
Ocular Surface New instruments, closure by graft or secondary intention
Small Squamous
Excisional Neoplasia Biopsy results Negative margins 5% recurrence
Large/Diffuse (OSSN) (10 y) 30% if no cryo used
Incisional biopsy Positive margins
Investigation 50% recurrence
Immunohistochemistry Good clearance
Lymphoproliferative No drops
Flow cytometry Biopsy Excise again
Inadequate side excision
Management Or drops
Gene rearrangemnet studies Postoperatively
Inadequate deep Scleral lamellar resection
2/3 isolated (MALToma) excision Plaque or external beam radiotherapy
1/3 – systemic disease Refer to oncologist
Consider enucleation
Systemic treatment Treatment Interferon QID + retinoic acid every 2nd day, 9 mo
Medications
External beam radiotherapy 2/52 On, 2/52 off
Local Mitomycin C 0.04% QID - 4/4/4
Often 4 cycles
Observation post excisional biopsy
Lifelong 4 wk cycle/4 cycles/0.04%
Metastasize
Follow-up Recurrence Exenteration, radiation, palliation
If suspect recurrence
Impression cytology
Preinvasive intraepidermal lesion of sun-exposed areas
Features
Can progress to melanoma
Nodularity
Oncology
Enlargement Suspect malignancy

Vascularity No-touch technique
Wide margins 4 mm
Close observation for malignant changes
Excisional biopsy
Full surgical excisional biopsy Primary Acquired
Map biopsy if suspect Melanosis (PAM) Limbal/Conjunctival
+ Double freeze thaw cryotherapy 4 mm margins
Treat body
PAM without atypia As for OSSN
+/– Scleral bed If fixation/tethering
Review 6/12 Management
resection
PAM with atypia Otherwise cryotherapy sufficient
MMC 0.02% QID Biopsy results
Treatment
Inadequate side excision
Field sterilization, cryotherapy
MMC 0.02% QID or Reoperate
Treat roots
Scleral resection +/– plaque, New instruments, closure by graft
Deep invasion or secondary intention
0.04% MMC
Features
Growth, nodularity, fixed to globe, dilated feeder vessels Regional lymph nodes
If spread Radical neck dissection
Palpebral conjunctiva & fornices spread Distal or multiple lymph nodes
Spread 6.4 Conjunctival
Preauricular & anterior cervical lymph nodes Palliation
Melanoma Yearly 4 quadrant biopsies
Delayed diagnosis
History Re-excise but no MMC
Recurrence
Re-excise & radiotherapy
Size Follow-up
Clinical Orbital
Recurrence No MMC
Site/Multifocal Examination
Forniceal/caruncle location, multiple
Palliation
Metastatic
Invasion/Metastases
Underlying sclera, lymph nodes Exenteration does not improve survival
Poor prognosis
Pagetoid spread pattern 5-y survival

84%
Cell type Prognosis
Epitheliod Pathology 10-y survival
71%
Grade
Poorly differentiated
Positive margins 5-y recurrence
39%
Small
Excisional
Biopsy
Large
Incisional Investigations
Underlying ciliary body melanoma
CT Chest/Abdomen/Pelvis
Sentinel lymph node
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3% of uveal melanomas
>3 mm diameter, >1 mm height

Pigmented/nonpigmented nodule
Between two pigment epithelium layers
Pupil
Epithelial
Obstruct vision
Argon laser photocoagulation Distortion surrounding structures Ectropion uveae
Primary Cataract
First years of life Features
Stromal
Most need treatment Slow
Needle aspiration or excision Growth Glaucoma
Ring spread
Postpenetrating trauma or surgery Iris Cysts Outflow invasion
Conjunctival or corneal origin Implantation Feeder vessel
Solid or cystic cysts Spontaneous hyphema
Corneal edema Secondary Tapioca appearance
Secondary features
Anterior uveitis Posterior extent
Melanoma Transpupillary/scleral transillumination
Glaucoma
Investigations
Ultrasound biomicroscopy (UBM)
Prolonged long-acting miotics
Photography
Non-Langerhans histiocytes 6.5 Iris Tumors Slit lamp, gonioscopy, UBM

Idiopathic granulomatous Observation
Multinucleate Touton giant cells Lifelong follow-up
Iridectomy
Yellowish papules Juvenile Small tumors
Cutaneous
Xanthogranuloma
Spontaneous regression Tumours invading angle
Presentation Iridocyclectomy
Treatment Do not do filtration surgery
Yellow lesions Procedures
Iris Risk spread
Spontaneous hyphema & glaucoma External beam
Radiotherapy
Treatment Plaque (brachytherapy) – least invasive, common
Topical steroids Enucleation
Freckle Prognosis 5% Develop metastases within

Does not distort iris architecture 10 y of treatment
Distorts architecture
Other Pigmented
Does not involve angle
Nevus
Does not grow
Fast growing & multiple
Flat, <3 mm usually, not vascular Metastasis
Pseudohypopyon
Oncology
Uveal effusion syndrome

Teratoid/Nonteratoid
Embryonal tumor, inner layer of optic cup
Benign/Malignant
Differential Diagnoses Medulloepitheliomas 1st decade
5% uveal melanomas Clinical Visual loss, pain, leucocoria
Enucleation
Sentinel vessels
Dilated in same quadrant as tumor Metastases
Astigmatism
Pressure on lens Clinical
Subluxation Congenital heterochromia
Cataract formation Congenital Horner's syndrome
Heterochromia after 1 y
Anterior extension
Dark epibulbar mass 6.6 Ciliary Body Fuch's heterochromic iridocyclitis
Other Diffuse
Posterior extension Melanoma + Iris Lesions Iris nevus syndrome in ICE
Exudative retinal detachment Other Tumors Hemosiderosis or siderosis
Blood or metallic foreign body breakdown
Iris heterochromia
3-mirror exam Hypertelorism
Investigations Waardenburg syndrome
Ultrasonography Hearing impairment
Biopsy White forelock of hair
Iridocyclectomy
Small/medium tumors, <1/3 of angle
Treatment Radiotherapy
Brachytherapy or proton beam
Large tumors
Enucleation
Schlemm canal invasion => secondary glaucoma
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Vascular hamartoma
Age 40–50 y
Hypermetropia from elevated retina
5–10% Caucasians Oval, indistinct margins
No follow up Fibrous metaplasia
Typical Circumscribed Complications Retinal edema
Malignant transformation low
Nevus
Exudation + RPE degeneration
Mnemonic: To Find Small Ocular Two types
Melanoma Use Helpful Hints Daily Ultrasound
(See Melanoma mindmap next) Treatment Solid, no orbital shadowing
Suspicious FFA Rapid hyper fluorescence, late leakage

Ultrasound
Baseline investigations
Fundus photos >1/2 choroid
Documented growth Diffuse Fundus dark red "tomato ketchup"
Treat as melanoma Hemangioma
Associated with retinal detachment
Multifocal in 30%, bilateral in 30%
Diffuse always associated with Sturge–Weber
Hemorrhagic Ipsilateral port wine stain
Less well circumscribed Features Seizure
6.7 Choroidal Leptomeningeal disease
Exudative retinal detachment Sturge–Weber Mental retardation
Tumors
Ultrasound Abnormal angle
Hyperechoic (not hollow) Glaucoma
Raised episcleral venous pressure
Chest most common – males & females Metastases
Iris heterochromia
In 10% eye will be first sign Breast 80%
Female Source
Amelanotic choroidal melanoma
Lung 10%, unknown 10%, other <1%
Choroidal metastasis
Lung 40–60% Differential diagnosis
In 50% eye will be first sign Male
RPE detachment
Unknown 25%
Posterior scleritis
3–5 mo bronchial
Survival
8–15 mo breast
Associated with oculodermal
melanocytosis (nevus of Ota)
Slow ossifying tumor aka Osseous choristoma
Melanocytoma Rare heavily pigmented tumor,
Chroidal Osteoma optic nerve head
Photodynamic therapy for
secondary CNVM (choroidal Treatment Treatment if malignant
neovascular membrane)
transformation – melanoma
Melanoma
See separate map

Diagnosis & monitoring
Ultrasound Highly reflective anterior border

Choroidal excavation
Acoustic hollowness
Orbital shadowing
Oncology
Ocular
Fluorescein fundus Tumor & retinal vessels
Dual circulation in collar-stud melanoma
angiogram
6 million/y Subtopic
Epidemiology Investigations
MRI With contrast & fat suppression
Oculodermal melanocytosis – 1:400
Will detect 60% of metastases if done annually, 90% if every 6/12
Photopsia LFTs and liver ultrasound
High false negative though – angiomas, cysts
Serous retinal detachment
Systemic CT Chest/Abdomen/Pelvis
Decreased visual acuity Symptoms
Vitreous hemorrhage
PET scan
Posterior tumor
Oncology referral
Field defect, floaters, pain
DIFFUSE worse vs nodular
Each risk factor confers 10% risk evidence of INVASION Extrascleral/Vortex vein
Thickness >2 mm
EPITHELOID TYPE
Best to worse: A best > B > mixed > epitheloid
Fluid
To Find Small Ocular Nucleolar size variation
Symptoms CYTOLOGY
Melanomas 6.8 Choroidal Clinical - DIET CLASs
Orange pigment Use Helpful Hints Daily Mitotic activity poor indicator
Melanoma
LOCATION
Margin of disc Anterior (ciliary body) worse
Ultrasound hollow
AGE
Older worse
Halo absent
SIZE of tumor
Drusen absent Signs >5 mm = worse
Horizontal growth IMMUNOHISTOCHEMISTRY Melan A, Anti-s100, HMB45

Exudative retinal detachment
Worse Prognosis Nevi same though
Penetration of Bruch's and RPE Collar-stud
Monosomy chromosome 3
Scleral channels Growth Worse prognosis Multiplication chromosome 8q
Invasive CYTOGENETICS
Vortex veins & nerves patterns Chromosome 1p-
Better prognosis
Laboratory – Chromosome 6 aberration
Choroidal folds ICy PIMP Nucleolar area, DNA synthesis
Hemorrhage cell PROLIFERATION INDICES
Ki-67, Mib-1
Others
Rubeosis Proliferating cell nuclear antigen
Secondary glaucoma MICROCIRCULATION Network pattern/closed loop with 3 loops
Cataract Worse prognosis
Normal DNA
PLOIDY analysis Euploid/diploid/tetraploid
Abnormal DNA multiples 'aneuploid'
Malignant DNA
Unsure use in prognosis as yet
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Uncertain diagnosis
No treatment Serial follow-up for growth
Limited life expectancy

Less spread
Prevent death Ruthenium
Isotopes <5 mm thick only
Prevent pain Iodine
Treatment Aims Brachytherapy Up to 10 mm thick tumor
Central first
Preserve vision (plaque Regression 1–2 mo
Peripheral next radiotherapy)
Radiation atrophy 2–6 mo
Cosmesis Common Radiation optic neuropathy/maculopathy
Complications Cataract
Central/marginal tumor recurrence
Retinopathy
Collaborative Ocular Melanoma Study Size >16 mm diameter
Management
Height >10 mm, Diameter >16 mm Spread
Enucleation Optic nerve or ciliary body/angle
(>2 clock hours)
Indications
PERT (pre-enucleation radiotherapy) Complications
Enucleate vs PERT
then enucleate Large tumor Glaucoma from angle invasion
PERT no effect on survival Patient
6.9 Choroidal
Survival at 5 y = 60% (both) Fear, follow-up risk
Melanoma Proton beam radiotherapy
Height 2.5–10 mm, diameter 5–16 mm Treatment
810 nm infrared diode laser
Transpupillary
No significant difference in survival thermotherapy <3 mm thick, <1/3 nerve head involved
Medium Uncommon
Brachytherapy patients require Enucleate vs iodine tumor Retinal traction & retinal detachment
regular reassessment COMS Study
125 brachytherapy Vascular occlusion
Complications
Survival at 5 y = 80% (both) Iris burns, lens opacities
Recurrence
Height 1.5–2.5 mm, diameter <5 mm Stereotactic radiotherapy
For extrascleral spread
Survival at 5 y = 99%
Small tumor Exenteration
Observation
95% to liver
Only 10% cutaneous melanoma metastasize to liver
31% grew to medium/large at 5 y
Survival
Larger/thick base, lipofuscin, Risk factors for growth 6–9 mo, 20% survival at 1 y (COMS #15)
no drusen Old age
Screening Ultrasound liver Rapid growth
Metastatic Bad prognostic signs
tests
Note: Treat based on high-risk features Large size
Cytogenetics
FISH/SNP techniques
Interferon & IL2

Chemotherapy
Treatment Hepatic resection and/or chemotherapy
Oncology
Congenital hypertrophy of the retinal pigment epithelium
Flat, dark, discrete margins, equatorial

Isolated or multifocal
Primary CNS lymphoma Multifocal – bear track pigmentation
Primary intraocular lymphoma Intraocular Lymphoma
AD, 80% of FAP have CHRPE

Metastatic systemic lymphoma CHRPE Familial adenomatous polyposis
>3 CHRPE, >1 quadrant => consider FAP
6.10 Retinal + RPE FAP + osteomas of skull/mandible/longbones

CNS medulloblastomas Tumors Gardner syndrome
Associated with von-Hippel Capillary Cutaneous soft tissue tumors
Pheochromocytoma Lindau syndrome
Epidermoid cysts, lipomas,
fibromas
Renal cell cancer Hemangioma
Turcot syndrome
FAP + tumors of CNS (glioma)
Cavernous
Racemose
Retinoblastoma
Others
Astrocytoma
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Predominantly non-Hodgkin, diffuse large B-cell lymphoma
Part of primary CNS lymphoma (PCNSL) First line

Features
20% of patients with PCNSL have PIOL 400 µg/0.1 mL, 6 injections average
Intravitreal
PIOL develop PCNSL within 2–3 y methotrexate 1–2 times for 1 mo
Poor prognosis Then monthly for 10 mo

Primary retinal
Commonly disseminates to CNS Intravitreal rituximab – combination
Good prognosis Side effect – corneal epitheliopathy

Types
Primary uveal
MALT type B-cell lymphoma
Advanced PIOL
Secondary intraocular lymphoma Treatment
Arises in uvea Radiotherapy Side effects
Cataract, epithelial changes,
risk blindness
Floaters, blurred vision, red eye & photophobia Recurrence common
Presentation
Bilateral 80%
Ocular Features
Systemic Primary CNS lymphoma
Mild anterior uveitis, vitritis
chemotherapy No radiotherapy
Multifocal large yellowish sub-RPE infiltrates Methotrexate mainstay

Signs
RPE detachment
Coalescent subretinal infiltrates
Leopard skin pigmentation 6.11 Primary Intraocular
Absence of cystoid macular edema Whole brain irradiation
Lymphoma (PIOL) Other options
Immunotherapy – rituximab
Intracranial mass
Headache, nausea, seizures Combination treatment
Presentation
Leptomeningeal disease
Neuropathy
Signs MRI brain with contrast Vitrectomy
Vitreous biopsy
Abnormal neurological exam Investigations
Lumbar puncture No preoperative steroids
Comprehensive medical/surgical history Neurological Features

Negative MRI/LP Cell cytology
Ophthalmologist
Physical examination Flow cytology
FBE with differential Analysis Immunohistochemistry
CT scan Chest/ MRI brain with contrast Gene rearrangement studies

Abdomen/Pelvis Neurologist
Lumbar puncture
Associations
Neurofibromatosis 1
Oncology
50% associated with Solitary lesions

von-Hippel Lindau
Retina or optic nerve head
Associations
Almost all have Multiple lesions Features Rare, congenital, unilateral, vascular hamartoma
von-Hippel Lindau
Sporadic
Capillary-like vascular channels Histology
between large foamy cells Aneurysms – "bunch of grapes"
Signs
Within capillary bed between arteriole & venule Fluid levels
Cavernous
Feeder artery & draining vein
Hemorrhage
Dilatation & tortuosity Complications
Exudate ERM
Endophytic
Bleeding & leakage
6.12 Retinal Avoid photocoagulation
Complications Types
Tractional retinal detachment Hemangioma Hemorrhage or tumor enlargement
Fibrotic Vitrectomy
Vitreous hemorrhage Treatment
bands
For vitreous hemorrhage
Rubeosis
MRI for patient/family Local excision
Sessile juxtapapillary lesion Intracranial
Capillary lesions Proton beam
Exophytic
radiotherapy
Edema, exudation, hemorrhage
Associations Wyburn–Mason Ipsilateral AV malformations
syndrome
CNS hemangioma (VHL) Investigation
MRI brain Midbrain, basofrontal & posterior fossa
AV malformation
Racemose
Incidental finding
No treatment
Asymptomatic without exudation
Isolated
Laser Treatment
Feeder vessels, then tumor No treatment required
Cryotherapy Treatment
Larger, or exudative detachment
Brachytherapy
If too large for cryotherapy
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Melanoma-associated retinopathy
Differs from CAR

Visual symptoms after diagnosis of
cutaneous melanoma
MAR Sudden central vision loss

Signs
CAR signs
Cancer-associated retinopathy
Investigations
Associated with small cell lung cancer ERG
Subacute bilateral visual loss over 6–18 mo Visual prognosis good

6.13 Paraneoplastic
Syndromes
Symptoms
Shimmering/flickering lights
Symptoms precede diagnosis by months in 50% patients

Bilateral diffuse uveal melanocytic proliferation
CAR
Arteriolar attenuation Proliferation of benign melanocytes in outer choroid

Signs
Optic disc pallor BDUMP Multiple nevus-like choroidal lesions

Signs
Electroretinogram (ERG)
Investigations Exudative retinal detachment
Severely attenuated photopic & scotopic
Rare
Prognosis
Poor for vision & life
Yellow = Common
Oncology
Gray = Rare Paranasal sinuses Breast

Embryonic tissue
Orbital teratoma
Lung
Metastasis Blood borne
Cavernous hemangioma
Gastrointestinal tract
Adults
Lymphangioma Neuroblastoma
Vascular Kidney
Children
Capillary hemangioma
Embryonic tissue
Malignant orbit teratoma
Glioma
Vascular
Central (optic nerve) Angiosarcoma
Meningioma
Nerves Malignant Schwannoma
Schwannoma
Nerves
Plexiform Peripheral 6.14 Orbital Tumors – Neurofibrosarcoma
Neurofibroma Benign vs Malignant
Solitary neurofibroma Fibrous connective tissue
Fibrous connective tissue Fibrosarcoma
Fibroma Fat
Includes solitary fibrous tumor & Liposarcoma
ossifying fibroma
Benign Malignant
Fat Rhabdomyosarcoma
Lipoma Muscle
Leiomyosarcoma
Rhabdomyoma
Bone
Muscle Osteosarcoma
Leiomyoma
Malignant lymphoma – B- & T-cell
Osteoma
Bone Lymphoid tissue
Fibrous dysplasia Lymphocytic leukemia
Benign reactive lymphoid hyperplasia Langerhans cell histiocytosis

Lymphoid tissue Malignant
Atypical lymphoid hyperplasia
Adenoid cystic carcinoma
Langerhans cell histiocytosis Lacrimal gland Adenocarcinoma
Benign
Lymphoma
Lacrimal gland
Pleomorphic adenoma
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Malignant tumors arising in

lymph nodes/lymphoid tissue
Definition
Unregulated leukocyte growth in bone marrow
Reed–Sternberg cells Discrete tumor masses
Blood: anemia (RBCs), infections (WBCs), hemorrhage (platelets) Definition
Definition Localized, single group of nodes
Cells in liver, spleen, lymph nodes possible 50% associated with EBV
Nodular sclerosing
Hodgkin's 65–75%, excellent prognosis,
Children, lymphoblasts, bone young, female >male
marrow or mediastinal mass Mixed cellularity
Acute lymphoblastic leukemia/
Responsive to therapy, lymphoma (ALL) 25%, Intermediate prognosis
Lymphocyte predominant
better prognosis
6%, Excellent prognosis, <35 yo males
Lymphocyte depleted
Small lymphocytic lymphoma/ Lymphoid
Adults > 60 y, Chronic lymphocytic leukemia (CLL) Lymphoma Associated with HIV/immunosuppression
Rare, poor prognosis,
often asymptomatic older males Multiple peripheral nodes
Hairy cell leukemia Definition
Most involve B-cells
Elderly, mature B-cell
Young adults,
Acute myelogenous leukemia (AML) Burkitt's EBV association
Adults, myeloblasts lymphoma Older adults, but
Non-
circulating Hodgkin's Jaw lesions 20% children
Myeloid Diffuse large B-cell
Mature B-cells Most common NHL
Raised neutrophils,
metamyelocytes, basophils
Mantle cell Mature T-cell
Chronic myelogenous origin in 20%
leukemia (CML)
6.15 Heme-Oncology
Splenomegaly Follicular
Older males, poor prognosis
Adults, hard to cure,
May transform to AML or ALL
indolent course
Adults
ALL Leukemia Adult T-cell
Mature T-cells Cutaneous lesions,
Blasts predominate Acute aggressive
AML
Monoclonal plasma cell
Children or elderly Features Mycosis fungoides/ Adults
Alternate IgG (55%) or IgA (25%) Sezary syndrome
Short, aggressive course organization Cutaneous lesions,
CLL Definition indolent
Arises in bone marrow
More mature cells CML Chronic
Most common primary tumor within bone age >50 y
Midlife age range Features Multiple Hypercalcemia
Myeloma Punched-out lytic bone lesions
Longer, less aggressive course CRAB Renal insufficiency
Anemia
If asymptomatic Monoclonal gammopathy of MGUS
undetermined significance Bone/back pain
7 Orbit, Oculoplastics, and Lacrimal System
Orbit,
Oculoplastics, &
Lacrimal System
7.1. Eyelash Disorders 7.18. Preseptal
7.3. Entropion/Ectropion Orbital cellulitis 7.19. Orbital

7.2. Lid Position
Disorders 7.20. Other Cellulitis & Mucormycosis
7.4. Ptosis Etiology
7.5. Ptosis Management 7.21. Orbital Inflammation

7.22. Vasculitis
7.6. Eyelid Lesions

Eyelids 7.24. Childhood
7.7. Benign Lesions
7.23. Vascular Lesions 7.25. Adult
7.8. Premalignant Lesions
Neoplasms 7.26. Carotid–Cavernous Fistula
7.9. Malignant Epithelial Lesions Orbit
7.10. Malignant Lesions – 7.27. Cystic Lesions

7.28. Dermoid
Melanoma & Sebaceous Gland
7.11. Treatment 7.30. Adult
7.31. Optic Nerve Tumors

7.29. Orbital Tumors
7.12. Epiphora – 3 Causes
7.32. Metastatic Tumors
7.13. Dacryocystorhinostomy (DCR) 7.33. Other Tumors

7.14. Canalicular
Stents Lacrimal 7.34. Seven Ages of the Orbit
7.15. Infections
System
7.35. Orbital implants
7.16. Lacrimal Gland Lesions

7.17. Malignant Lesions
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Posterior misdirection, normal origin

Whitening
Chronic blepharitis
Blepharitis
HZO
Cause Infection
VKH, sympathetic ophthalmia Inflammatory Poliosis Trachoma
Vitiligo Cause
Trauma
Tuberous sclerosis Chemical/Nonchemical
Marfan syndrome Electrolysis

Epilation
Increased number Laser
Electrolysis
Latanoprost Lash excision
Drugs Cryotherapy
Phenytoin
Tarsal rotation
Cyclosporin
Trichomegaly
Cause Underlying disease If entropion present
Malnutrition Trichiasis
HIV Epilation
Hypothyroidisim Electrolysis
60% recurrence rate
Management
Oculocutaneous albinism 7.1 Eyelash electrolysis Double freeze thaw -20 degrees
Disorders Cryotherapy 50% success, 70% with repeated
Desire to pull out lashes Apply ~30 seconds to lashes/conjunctiva
Impulse control disorder
Teenage girls Argon laser photocoagulation
Trichotillomania
Habit reversal therapy Heal by primary closure
Management Anterior lamellar short strip
SSRIs for OCD Heals well
Horizontal fracture of tarsus & rotation
Decreased number If entropion Trabut procedure – split at 3 mm from
Tarsal rotation present margin, everting sutures
Infiltrating lid tumor
Bilamellar tarsal WHO recommended
Burns rotation
Radiotherapy Local 77% success
Blepharitis
Madarosis Abnormal origin, generally posterior
Behind meibomian gland orifices
Iatrogenic Cause
Congenital Lymphedema
Skin disorder Lymphedema–distichiasis syndrome
Alopecia, psoriasis Cause
Distichiasis
Acquired
SLE Cicatrizing conjunctivitis
Syphilis, leprosy Systemic
Lamellar eye division
Management
Hypothyroidisim
Cryotherapy
Middle aged overweight male
Manage condition Visible superior limbus and
sclera in primary gaze Demographic Obstructive sleep apnea & snoring
General
Sleeping face down
Congenital Conjunctiva abrades pillow
Duane's, Downs
Associated with keratoconus
CN7 Palsy
Evert upper lids easily
CN3 aberrant regeneration Micropapillary & filamentary
Superior tarsal conjunctivitis
Parinaud's dorsal Neurogenic Clinical conjunctivitis
midbrain syndrome
Worse in morning
Eyelid Retraction Punctate keratopathy
Orbit, Oculoplastics, and Lacrimal System
Sympathomimetic eyedrops Floppy Eyelid Syndrome
Causes Respiratory physician referral

Thyroid eye disease
Myogenic General OSA testing
Myasthenia Weight loss
7.2 Lid Position Avoid face down sleeping

Cicatricial
Disorders
Management Lubricants
Postoperative – bleb, buckle Medical
Mechanical Tape eyelids shut at night
Myopia, buphthalmos
LTS better than
Orbital mass => proptosis wedge resection
(Ezra et al. [2010])
Trauma Horizontal
Surgical lid shortening Poor results if systemic
not controlled
Upper lid overlaps lower lid
Eyelid
Lubrication Imbrication Syndrome
Management
Ectropion/Entropion See next
Horizontal lid tightening
Puncta displaced anteriorly,

Ptosis See next
out of lacuna lacrimalis Anterior malposition Centurion Syndrome
of medial lid
Due to prominent nasal bridge
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160
Tarsal
plate Acute eversion
Palpation of tarsal plate Congenital
Atrophy
Retrobulbar Congenital ectropion
Relative enophthalmos fat
Ipsilateral facial palsy
Downgaze to see Lower lid CN 7 palsy
position of lower lid retractors Weakness Paralytic
Assessment Temporary – lubrication, Botox, temporary tarsorrhaphy
Preseptal to pretarsal AWOL Treatment
orbicularis oculi Permanent – gold weights, permanent lateral +/– medial tarsorrhaphy
Test by eyelid closure Overriding
Age related, diagnosis of exclusion
Pull lid away from globe,
Horizontal Lid laxity Horizontal lid laxity
watch 'snap back'
Distract lower lid >8 mm, failure to snap back
Hotz
Tarsal fixation of pretarsal skin & orbicularis procedure Congenital Mild >2 mm
Medial canthal
Clinical tendon laxity Moderate – punctum to limbus
Dehiscence of
lower lid retractors Horizontal
lid laxity Severe – punctum to pupil
Decreased lower Ectropion Involutional
lid excursion Age related Lateral canthal tendon laxity
Vertical lid
Lack of fornix degeneration Generalized
Downgaze instability
deepening 7.3 Lid Position Horizontal lid shortening – lateral tarsal strip (LTS)/wedge
Overriding pretarsal orbicular Disorders – Small – retropunctal diamond
is during lid closure Entropion/ Treatment
Medium – retropunctal diamond+ LTS/
Transverse lid everting sutures Temporary Ectropion Medial wedge for horizontal lid laxity
Transverse lid split No horizontal Involutional Large – above + inferior retractor plication/plastics for mid face lift
Weis
& everting sutures lid laxity
procedure Entropion Anterior eyelid Orbicularis & skin
Weis procedure +
Quickert's lamella shortening
horizontal lid shortening procedure Horizontal Treatment Cause
lid laxity Relative to posterior lamella (tarsal plate, conjunctiva)
Wedge resection
Lateral tarsal strip Worsens on opening mouth & looking up
Inferior retractor Recurrence after Underlying cause

plication Weis/Quickert's Cicatricial
Jones Remove scar tissue
procedure Cicatrizing conjunctivitis
Lid has been stretched
Trachoma Causes Treatment Horizontal lid laxity
Chemical injuries & trauma => LTS to tighten lid
Electrolysis Cicatricial Lengthen
Anterior rotation of lid margin anterior Full thickness skin graft (subciliary incision)
Cryotherapy
lamella
Lash excision Trichiasis Treatment Donor sites
Upper eyelid, preauricular, supraclavicular
Rotation of terminal tarsoconjunctiva &
posterior lamellar graft/advancement Mechanical Tumors Remove & correct residual malposition
Tape lids
Eyelid everting sutures Conservative Spastic
Botox
Ipsilateral hypotropia in upgaze
Gaze deficits
Superior division may be isolated
CN 3 palsy
Tumor
Aberrant regeneration Compressive cause
Aneurysm
Ptosis/miosis/anhydrosis
Neurogenic Anisocoria worse in dark

Unilateral
Unilateral vs Bilateral Horner's Dilation lag on affected side
Neurogenic, traumatic, mechanical syndrome
Bilateral Cervical CT/CTA
Neuromuscular, aponeurotic, myogenic Investigation MRI Head neck & upper thorax
Dystrophic levator development MRA Extracranial ICA
Without innovational abnormalities Miller–Fisher (bulbar variant of Guillian–Barré)
Tumors Congenital
Hemangiomas, neurofibromas
Chalazion
Ametropia
Management Mechanical Orbital malignancy
Amblyopia
Cicatricial/Scarring/Edema
UL should cover
Decreased excursion
1–2 mm of Acquired Myasthenia gravis
in downgaze Neuromuscular No anisocoria
upper limbus
Botulism
Dorsal midbrain syndrome Chronic progressive external Ptosis
Progressive supranuclear palsy Neurogenic ophthalmoplegia
External ophthalmoplegia + slow saccades
Sympathomimetic eye drops
Contralateral 7.4 Ptosis – Etiology Isolated
CPEO
Thyroid orbitopathy eyelid retraction Oculopharyngeal dystrophy
Myasthenia Myogenic Myogenic Classification
Pigmentary retinopathy, hear
Kearns–Sayre
Postop conduction defects
syndrome
High axial myopia Ragged-red muscle fibers
Pseudoptosis Steroid eye drops
Buphthalmos Mechanical
Myotonic dystrophy
Proptosis 2nd to mass
High skin crease >7 mm from margin
Dermatochalasis Disinsertion/
Skin Stretching/ Thin upper lid
Brow ptosis Aponeurotic Most common
Dehiscence
Normal/Increased levator function
Hypoglobus
Or contralateral No anisocoria or fatigue
Enophthalmos Position Eyelid laceration
exophthalmos
Traumatic
Hypotropia Globe Postsurgical foreign body
Phthisis bulbi
Size
Microphthalmos
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Horner's
GCA
Aneurysm Avoid teasing
Third nerve palsy Exclude these
Diabetes School age 5–7
four conditions Fascia lata sufficient
Myasthenia gravis Timing Earlier Amblyopia, astigmatism
IMPORTANT
Superior eyelid or orbit malignancy
If parents happy
Yes Later
CN 3 or Horner's Local anesthetic – reliable outcomes
Anisocoria Pediatric
Myasthenia, congenital ptosis, senile ptosis No Treatment Jaw-winking Disinsert levator + brow suspension
Procedure
Pupil size & reaction Ptosis Based on levator function
Extraocular movements Mandatory
Lagophthalmos, lid lag on downgaze
Exposure keratopathy
Complication
MRD1, PF, inferior scleral show Cosmesis
Lids Asymmetry, contour, scarring & lid malposition
Normal PF = 9
7.5 Ptosis –
Levator function Frontalis sling
Management <4 mm Brow suspension
Fox pentagon
In downgaze Upper lid
crease Measure Sutures through
Females = 10 mm, males = 8 mm
Adult Based on levator muscle belly
5–10 mm Levator resection
High or absent => aponeurotic defect Treatment function
Pretarsal For children classically
Lid margin to skin fold show Measure
Levator aponeurotic advancement
In primary position
>10 mm Sutures through aponeurosis
Exophthalmometry
Orbit exam Fasanella–Servat tarsoconjunctival
Aberrant regeneration Pediatric
mullerectomy
Palpate superior orbit Examination
Eyelid to frontalis
Corneal sensation & Bell's reflex
Ocular exam muscle
Dilated fundus exam – RP in Kearns–Sayre Using subcutaneous sling
Non-autologus Integratabale
Other lid drops => increased levator tone Mersilene, Gore-Tex
Manual lift ptotic lid Fox pentagon material
Affects surgery Brow Non-integratable
Prolene, silicon rod
If do one side, Suspension Crawford frontalis
Fatiguability Always test Autologus fascia
other side will drop sling Age >5 y to have enough fascia
Jaw-winking phenomenon
Bell phenomenon Severe ptosis (>4 mm)/poor levator function <4 mm
Myasthenia Marcus–Gunn jaw-winking
Ice, Tensilon Indications
Horner's Special tests Ptosis with CN3 palsy/aberrant regeneration
CTA/MRA if suspect aneurysm
Imaging Blepharophimosis
CT/MRI orbits if suspect malignancy
Madarosis
Cicatrization
Lid SGC signs
Thickening
Loss meibomian gland orifices
Canthal lesion, tethering
Chalazion Palpable mass, ophthalmoplegia/proptosis

Invasion into orbit
Red Flags – Poor
Moll/Zeiss/Eccerine/Milia Proptosis, abnormal
Prognosis LIPS
Cyst
Xanthelasma Abnormal 2, 5, 6 symptoms

Perineural invasion
Epidermoid
Keratoacanthoma (fast growth)
Premalignant
Basal cell papilloma (aka seborrheic keratoses)
Actinic/solar keratosis
Squamous cell papilloma 7.6 Eyelid Lesions
Benign Lymph nodes
Nevi
Systemic – metastases Preauricular, mandibular, cervical lens
Pyogenic granuloma Liver
Differential
SGC, multiple myeloma, Merkel cell
Diagnosis
Keratoacanthoma
Premalignant
Actinic/Solar keratosis
Diagnosis
Histologically
Basal cell carcinoma (BCC)
Screen
Squamous cell carcinoma (SCC) Surgical Principles Orbit invasion, metastases
Sebaceous gland carcinoma (SGC) Excision

Adequate clearance
Melanoma Malignant
Reconstruct
Multiple myeloma Structure & function
Kaposi sarcoma
Merkel cell
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Junctional
Incompletely differentiated
Compound melanocytes
Elevated
Nevi
Dermal Seborrhoic keratosis
No pigment (basal cell papilloma) Sessile or pedunculated
No treatment/excision Epithelial
Superficial shave excision
hyperplasia
Basal layer epidermis Squamous cell papilloma
More melanin granules (fibroepithelial polyp)
hyperpigmentation Freckle (ephelides)
Same number melanocytes Pedunculated, skin tag; excision
Eyelid Hair follicle origin,
Epidermal inclusion cysts
Solar lentigo keratin. excise/marsupialize
Sun exposure pigmented macules – no treatment Benign
epithelial cysts Pilosebaceous block => keratin
Dermal melanocytes proliferation Milia
Blue nevus retention. Resolves
Low malignant, but excise
Melanocytic Molluscom contagiosum
Increase number/size/pigmentation of melanocytes Lipogranulomatous,
lid care, incision/curettage
V1 & V2 dermatome commonly General
Chalazia, Recurrent -
1:400 malignant melanoma hordeola Biopsy Sebaceous gland
But no treatment
carcinoma
Iris mamillations, darker fundus, Episclera/ Oil gland
RPE degeneration + drusen uvea, Dermal (Meibomian/Zeis/ Yellow papules,
Ocular 7.7 Benign Lesions Sebaceous hyperplasia
no skin melanocytosis sebaceous) center umbilication
Melanocytoma of optic melanocytosis
nerve head Uveal Anterior lid margin
Cyst of Zeiss non-translucent cyst
melanoma
Lesion cannot risk
be moved Sebaceous adenoma
Adnexal Multiple pale nodule, 1–2 mm

5% bilateral
Syringomas lower lid, young females
Oculodermal melanocytosis Eccrine
2/3 aka nevus of Ota sweat gland Deep in dermis => complete excision
Skin & eye
Eccrinespiradenoma
V1 & V2 dermatome Congenital Rare
Eccrineacrospiroma
Blood in Schlemm's canal subcutaneous
Glaucoma in 30% Port-wine stain Cyst of Moll
Apocrine Translucent, multiple,
Cavernous hemangiomas aka apocrine
sweat gland marsupialize/excise
5% Sturge–Weber syndrome hidrocystoma
Others
Pyogenic granuloma
Hair follicle Trichoepitheliomas
Small papules eyelid/forehead
Xanthelasma
Hair bulb cells, young female Pilomatrixoma

Deep dermal nodule, calcifies
Most common, Caucasian, sun exposure
Round, scaly, keratotic plaques
Clinical Increase in response to sunlight

Actinic keratosis State of flux
aka solar keratosis Decrease with less sunlight
0.24% per year

Malignant potential
Multiple lesions => 12–16% develop SCC
SCC in situ of skin

Premalignant
Epidermal Elevated, non-healing erythematous
Bowen disease Clinical

Scaling, crusting, pigmented
keratotic plaques
Autosomal recessive disease Malignant potential

Xeroderma 7.8 Premalignant 5% become invasive
Failure of DNA
pigmentosum Lesions Low grade SCC
repair mechanisms
Sunlight => skin damage => BCC, Flesh-colored papule

SCC, melanoma Keratoacanthoma Clinical Becomes dome- Center keratin-filled crater
shaped nodule
Autosomal dominant Predisposing
Elevated rolled margins
Lesions
Congenital deformities eye/face/bone/CNS Treatment
Gorlin–Goltz Incisional/complete excision
Multiple BCC 2nd decade syndrome
Medulloblastoma Melanoma in situ

Caucasians, malar region
Breast carcinoma Other malignancies
Premalignant
Melanocytic Clinical Flat, irregular shape, uneven pigmentation
Hodgkin lymphoma
Lentigo maligna aka Slowly enlarging
Hutchinson melanotic freckle
Malignant potential
30–50% progress to melanoma
Treatment
Excision, wide margins
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Lower lid margin 50–60%
90–95% Medial canthus 25–30%

eyelid cancer
General Upper lid 15%
Lateral canthus 5%
Risk factors
Fair skin, age, sun exposure, smoking
Less common, more aggressive
General Firm, raised, pearly
Risk factors Nodular
Telangiectasia, central ulceration
Increased in immunocompromised Clinical
Morpheaform/ Firm, elevated, indeterminate margins
Basal Cell fibrosing
Carcinoma More aggressive
Nodular
Medial canthal/vertical mid face
Hyperkeratotic nodule more likely infiltrative
Assess
Red base, elevated borders extent CT scan
Ulcerating Especially if medial
No pearly margins 7.9 Malignant
or telangiectasia Cutaneous horn Epithelial Lesions Lacrimal drainage
Management Avoid reconstruction of outflow for 5 y
Lymphatic/hematogenous/
3 mm margins
direct extension – especially nerves
Frozen section
Clinical Surgery Intraoperative
Regional lymph nodes 20%
Metastasis margin control Rapid paraffin & pad area overnight
Squamous Cell
Lymph nodes
Carcinoma Moh's surgery – especially for fibrosing BCC
CHECK
Sensation
Deeper extension
Surgical excision
Same as BCC
Management
Deep extension, 2nd line Radiation
therapy
Exenteration
Local/lymph node spread

Highly malignant General
5% cutaneous cancers but <1% eyelid cancers
Meibomian gland origin General
Also Zeiss & sebaceous Invasive vertical growth 10–20% of
glands in caruncle lentigo maligna patients
Lentigo maligna
Females, upper eyelid, multicentric Nodular
90% of head & neck melanomas
Oil Red O – fresh frozen section FRESH
(unfixed) Macro 4 Types 10% cutaneous melanomas, rare eyelid
Toluidine blue – plastic section Nodular
Likely deep extension by presentation
Discrete, hard, immobile
yellow nodule
Superficial spreading
Well-differentiated Nodular
Histology Plaque, irregular outline, variable pigmentation
Poorly differentiated Acrolentiginous
Lipid spaces rare Wide surgical excision, histology – clear margins

Micro
May extend palpebral/ Primary excision, urgent paraffin section
Diffuse 2 stages
bulbar conjunctiva Sebaceous Surgery
intraepithelial Repair +/– further excision 1–2/7 later
(Pagetoid) Gland Melanoma
Foamy cytoplasm
Regional lymph node biopsy
Human milk fat globule Evidence vascular/lymphatic involvement
Chronic Imiquimod cream
With loss of lashes blepharoconjunctivitis Topical
Clinical 7.10 Malignant
Madarosis Face, not eyelids, controversial
Recurrent chalazion Lesions – Melanoma &
At clinical trial stage, but good potential if have gene
Sebaceous Gland Targeted
Metastasis Treatment
Lymph nodes, viscera drugs BRAF gene target – e.g. vemurafenib
Wide excision +/– exenteration Other C-KIT gene – e.g. imatinib
Bulbar & palpebral options
Map Radiotherapy
conjunctiva Metastatic melanoma, does not prolong survival
biopsy Surgery
All cases – skip lesions
Management Immunotherapy – Maybe small benefit in some
Inform pathologist interleukin-2
Live longer, but severe side effects
Fresh specimen
Radiotherapy Can shrink temporarily for 3–6/12
Relatively resistant Chemotherapy
Decarbazine, temozolomide
Regional lymph nodes
Follow-up Thin
< 0.75 mm
Oncologist referral Survival 5 y – 98%
Chronic, red dermal mass Kaposi Sarcoma Thick 4 mm
Associated with AIDS 5 y – <50%

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<1/3
Direct closure +/– cantholysis
Tenzel Semicircular flap

Routine histology Semicircular
flap flap Large defects of anterior and posterior lamellae
<1/2
Lower lid defect up to 66% of length
Quick Need posterior graft/flap

Full thickness skin graft
Frozen & anterior reconstruction
Good for SCC & BCC Upper lid, preauricular, subclavicular
section
Vertical defect Posterior lamellar reconstruction
Not for melanoma or sebaceous 5–10 mm Tarsoconjunctival graft
+ Advancement flap from cheek
Melanoma/sebaceous >1/2
Urgent paraffin Vertical defect
gland carcinomas Tarsoconjunctival graft
section 10–15 mm
"Slow Moh's" + Advancement flap from cheek
Turnaround 24–48 hours
Histopathology Horizontal Vertical defect Tarsoconjunctival graft
Options Extent of Full >15 mm
Maximal tissue
Thickness Defect + Cheek rotation flap for anterior lamella
preservation
Mustarde – wary of CN7 branches
100% of margin Gold standard
Moh's Glabellar flap
examined
micrographic Medial canthus & medial part of upper eyelid
Ideal for surgery Medial lesion
periocular
Suitable for SCC/BCC +/– Canalicular stents
lesions Upper lid defect
7.11 Eyelid Neoplasm
Unsuitable for – Treatment Cutler–Beard flap
SGC, melanoma Tarsoconjunctival graft
Central lesion
Lower lid defect
Difficult if orbital Surgery is primary method
fat involved Hughes flap
Most common
Cryotherapy Advancement flap
Risk incomplete removal Mini-Mustarde flap/Tenzel flap
Only for BCC/SCC Rhomboid flap Risk facial nerve damage

Refused Non-surgical Lateral lesion Rotation flap
When surgery Radiotherapy Options
Anticoagulants
contraindicated Transposition flap
Poor general health
Lateral periosteal flaps
Palliation, Kaposi Helpful if UL/LL tarsal loss
sarcoma, lymphomas Mustarde flap
Large lesions
Imiquimod
Topical chemotherapeutic agent
5-FU
Symptoms
Eye history
Medications, surgery, dacryocystitis
Trauma, environment History Reflex from CN5 and parasympathetics
1. Ocular Surface
Medical & surgical history Irritation Dry eye, blepharitis
Inflammatory disease, radiotherapy, dacryocystitis
(Hyperstimulation) Atopic disease
Cornea/conjunctiva epithelium evaluation
Examples Thygeson's
Tear meniscus 1. Dry eye
Chronic cause of irritation
TFBUT, Schirmer's e.g. Trichiasis
2. Pump Floppy eyelid syndrome
Lid position, laxity
Jones 1 – Dye in eye, cotton failure Assessment
Examine blink 2. Lacrimal Pump
bud in nasal cavity Examination Entropion
Fluorescein dye Failure Lid malposition
(Mechanical) Ectropion
Jones 2 – Dye syringed into disappearance test 3. Punctal &
duct, bud in nasal cavity + Aberrant regeneration =>
Puncta canaliculi Facial nerve palsy
Size, stenosis/occlusion & position crocodile tears

Reflux of mucoid material Primary
Probe & syringe
Nasal endoscopy Secondary e.g. Repeated 5-FU injections in
Punctal stenosis glaucoma patients
Patency of ostium
Macro-dacryocystogram DCG Tests
3-snip procedure
CT scan 7.12 Epiphora Stones
Especially suspect lesion – 3 Causes
Inflammation
Canaliculitis
Dacryocystorhinostomy
Persistent epiphora, no canalicular function (DCR) 2/3 – obstruction 2nd to chronic dacryocystitis
See next at junction Exploratory DCR, separate adhesions
Loss of function – lacrimal pump failure 3. Blockage Canalicular with sac
of Drainage stenosis Intubate
Short patent canaliculi < 7 mm Junction between patent
Indications (Plumbing) & obstructed
Congenital absence of canaliculi
Treat with
Damage to canaliculi – trauma, HSV, trachoma canaliculo Resect obstructed part
1/3 – fibrosis prior
Lester–Jones (lateral) to sac DCR Anastomize patent part to nose
Internal opening of common canaliculus
tube
Assessment Need >7 mm patent
Fine probe retrograde up Treatment of
Nasolacrimal Idiopathic canaliculus from puncta
Retrograde cDCR preferable if probe passes Duct Blockage Most common
duct
Same as DCR Previous trauma
Secondary
Replace Loss of glass bypass tube Nasal/sinus surgery
Complications
immediately, Sinusitis
or tract closes Tube buried in wound, obstructs
Re-canalization Nasal Perennial rhinitis
Granuloma over tube opening
of nasolacrimal
Excise duct Abnormal anatomy
Procedure being developed
Re-bore lumen through obstructed duct
Modified monopolar cautery or Nd:YAG laser
Then stent
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Severe symptomatic epiphora Blocked NL

drainage system
Blockage distal to internal opening
of common canaliculus
Acute dacryocystitis Indications
After acute episode settled
Chronic dacryocystitis or mucocele Absolute
Causing discharge Definitive canalicular disease/stenosis
Do prior to cataract surgery Intubation
Otherwise increased risk endophthalmitis Previous dacryocystitis
Indications Risk of scarring
GA/LA Poor flap creation
Relative
Revision surgery
Cocaine 4% nasal pack
Excessive bleeding
Nasal mucosa
Cophenylcaine intranasal Inflammatory disease
Anesthetic
Topical benoxinate 0.4% Conjunctiva Hemorrhage
Complications Avoid nose blowing 2/52
LA
Nasociliary branches =>
infratrochlear, anterior ethmoidal Recurrence of epiphora
Nerves
Infraorbital Skin over sac
Scar
Bupivacaine/lignocaine with adrenaline
7.13 Dacryocystorhinostomy Ocular surface irritation
10 mm medial to medial canthus, Dry eye, blepharitis, atopic disease
15 mm straight vertical incision
(DCR) Wrong initial
diagnosis Lacrimal pump failure
Lid position, CN 7 palsy
Avoid angular vein Skin incision
Blockage of drainage – nasal Sinusitis, perennial rhinitis
Blunt dissection of orbicularis,
divide medial canthal tendon Previous trauma – altered anatomy
Postop 50% at osmium site
Periosteal elevator Post-DCR
anatomical block 50% at common canaliculus
(e.g. Freer, Rollett's Rongine) Epiphora
Incise & elevate
Periosteum
Medial to anterior lacrimal crest Stent-related complication
Granuloma inside drainage system
Periosteum & lacrimal sac
Expose lacrimal fossa Reflect SUMP syndrome Lacrimal sac incompletely open inferiorly
Technique Only drains once
Right angled periosteal elevator (e.g. Expressible mucopurulent discharge certain level
Traquair's) at anterior lacrimal crest
Create 10 mm hole Osteotomy Postop infection
Enlarge with bone punch (e.g. Kerrison's) Operation related Endoscopic less successful than external DCR
Internal DCR
Canaliculi to lacrimal sac
Bowman probe
Check common canalicular obstruction Hypersensitivity to stent
Lacrimal sac + nasal mucosa incised

Intubation Fistula
Loops inside nose
Flap closure
Posterior then anterior, 6–0 Vicryl
Closure
Re-attach medial canthal tendon
Permanent pyrex glass
All can be used for DCR
Bypass the canaliculi and create new passage

Lester–Jones Canalicular disease

Insert at medial canthus
Cheapest
DCR Tube
Tube, thread onto 000 lacrimal probe
Usually stays in permanently
Long-term follow-up, to keep it patent
7.14 Canalicular
Stents
Olive tip
Easiest to introduce to canaliculi & retrieve from nose
Single canalicular stents, remove from canaliculi end using collarette Crawford
Has lumen Monoka Hook from inside nasal cavity, crochet hook
Canalicular damage & stenosis, congenital NLDO
Mini – physically thread O'Donoghue Has introducer

Types
Grande – has metal introducer
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Commonly due to NLDO

Localized swelling lacrimal duct
Mucocele without inflammation
General
Can express mucus from puncta
Dacryocele
Congenital malformation lacrimal duct
Rhabdomyosarcoma (pediatrics) Inflammation
Below medial canthal tendon
Neoplasm
Lacrimal gland neoplasia Mucoid/purulent discharge
Clinical
Differential +/– Cellulitis/abscess
Infection
Cellulitis/preseptal cellulitis diagnosis
Dacryocystitis Exclude orbital cellulitis
Chalazion, dermoid cyst, lymphoma (Lacrimal Sac) EOM, vision, proptosis
Sarcoid Inflammatory CT
Investigation Severe/Unresponsive cases
Idiopathic orbital inflammatory disease Gram stain + culture of discharge
Avoid probing & irrigation

Idiopathic Warm compresses + analgesia
Inflammatory Initial Incision & drainage + culture
Sarcoid Management Admit + IV antibiotics
Severe/cellulitis
Viral Etiology 7.15 Lacrimal System Oral antibiotics
Mumps, EBV, HZO, influenza
Infection – Infections Ideally within a month
DCR
Bacterial Definitive Defer until acute infection resolves
Rare, S. aureus, N. gonorrheae, streptococci
Differential diagnosis
Dacryoadenitis Chalazion, dacryocystitis
Ptosis upper eyelid
S-shaped erythema (Lacrimal Gland)
Tender over lacrimal gland fossa Viral, chlamydial, mycotic organisms
Candida, HSV/VZV
Infective
Infection, tumor, motility, optic Examine intraorbital spread Clinical Etiology Actinomyces israelii – gram-positive rod
nerve function Bacteria
Nocardia
Retained punctal plug
Parotid gland
Sarcoid, TB, syphilis, lymphoma
Canaliculitis Punctal erythema +/– conjunctivitis
CT with contrast Clinical

Mucopurulent discharge on pressure
FBE & blood cultures if febrile Investigations
Patent on lacrimal syringing, failed DCR
Swab
Warm compress/manual expression
Supportive Flush duct with cephazolin
Mild–moderate – amoxycillin + clavulanate Trimethoprim/polymyxin B QID +
Antibiotics Treatment Antibiotics
Treatment doxycycline 100 mg oral
Severe – hospital + IV cefazolin
Abscess Canaliculotomy with marsupialization +
Incision & drainage curettage of concretions
Pleomorphic adenoma
Pleomorphic adenocarcinoma
Neoplastic Adenoid cystic carcinoma
Most common epithelial lacrimal gland tumor
Lymphoma
Age 20–50
Metastases
Pseudocapsule
General
Solid pale gray bosselated surface Sarcoid
Histology Superotemporal Bilateral, other sites
Myoepithelial Mass
Two components Wegener's granulomatosis
Forms stroma Inflammatory
Epithelial Thyroid eye disease
Painless proptosis/swelling – Idiopathic orbital inflammatory disease

slow (1 y) progressive Painless, slow growth

Smooth, firm, nontender mass 7.16 Lacrimal Congenital Dermoid Molds around bone
Orbital lobe
Gland Lesions
Hyperdense capsule
Inferonasal dystopia Origin Clinical
Palpebral lobe Infective
Upper lid swelling Dacryoadenitis
Pleomorphic
Proptosis Posterior Adenoma
extension A – Adapt around globe contour
Ophthalmoplegia
Choroidal folds c.f. displace & indent
B – Bonere molding
Smooth oval mass
+/– Calcification c.f. destruction
C – Circumscribed
Lacrimal gland CT scan Imaging – Benign
ABCDEF c.f. poor demarcation
Indentation, no destruction versus Malignant
Bone Investigations D – Diffuse expansion
No destruction c.f. isolated mass
If suspect,
avoid biopsy => E – Elongated
Biopsy = pleomorphic
seeding risk c.f. globular
Excise biopsy tract
F – Fossa enlargement
Excisional biopsy – take out in toto
c.f. destruction
Malignant potential Surgery
Approach
Lateral orbitotomy Treatment
Uncommon
Dry eye
Palpebral lobe often unaffected
Leave in situ
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Children 5–12
Demographics Poor prognosis
Adults 20–40
General
Solid cords + cysts => Swiss cheese
Perineural invasion
Histology
Intracranial extension
Difficult to clear margins
Faster
<12 mo
ACC vs pleomorphic
Painful proptosis Due to perineural invasion
& bone destruction
Features
Slowly progressive Inferonasal dystopia
Lacrimal mass Epibulbar congestion
effect Proptosis
Irregular lesion Posterior extension
Lymphoma Clinical
Periorbital edema
Conforms to globe & lacrimal fossa
CT scan Examination
Homogenous Ophthalmoplegia
Lacrimal nerve distribution
No calcification Hypoanesthesia
Adenoid Cystic Fundus
Disc swelling + choroidal folds
Carcinoma
Rapid proptosis
Orbital fibrous Neuro examination
Features Perineural spread => cavernous sinus
tissue contraction Scirrhous carcinoma Enophthalmos
of stomach/breast Metastases Globular lesion, irregular serrated edges
Lung, prostate, GI, kidney CT scan Invasion/erosion of bone

Source Calcium often seen
Skin – melanoma 7.17 Lacrimal Gland – Investigations
Malignant Lesions Incisional biopsy
Difficult to clear margins
Biopsy
If think pleomorphic
Complete excisional biopsy
Histology like benign Incomplete excision Malignant Pleomorphic
of adenoma Adenocarcinoma Local resection, enbloc removal,
Areas of malignancy Extensive surgical resection exenteration, radical exenteration
Poor prognosis
Same cells as adenoid cystic carcinoma Treatment
Radiotherapy
Adenocarcinoma Palliation + reduce pain
Solid pattern, less differentiation Chemotherapy

Preop & postop
Squamous Cell
Rare Carcinoma Prognosis
Survival rate 20% at 10 y
Discharge M/C/S
Children
Intervention vs radiation
Investigations
Sinusitis
CT indications
Subperiosteal lesion requiring

Polymicrobial
Adults decompression
S. aureus, S. pyogenes
Amoxycillin + clavulanate or
Single organism
Children Etiology Trimethoprim + sulfamethoxazole
Mild
S. pneumoniae, Hib
Nasal decongestant
Pseudomonas
Immunocompromised Treatment IV ceftriaxone or moxifloxacin
Fungi, Mucor
Severe Optic nerve function & proptosis
2-hourly
90% due to adjacent 7.18 Preseptal Consider repeat CT

Cellulitis
Indirect venous spread Sinusitis Septum underdeveloped
2 methods Children < 2 yo High risk orbital cellulitis
Direct extension through lamina papyreca
Skin infection 3 Causes Drains to cavernous sinus

Medial
Direct inoculation
periorbitia Cavernous sinus thrombosis
Trauma Risk
Cerebral abscess & infection risk; death
Otitis media
Source Gaze limitation, proptosis
Bacteremia Ocular examination
Pneumonia Danger Optic nerve function
Assessment
Conjunctiva & sclera injection
for above
Orbital apex syndrome, cavernous
Sensation V1 & V2
sinus thrombosis
Lymph nodes
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Sinusitis, subperiosteal & orbital abscess

CT with contrast
Re-image 24–48 h if no improvement
90% bacterial sinusitis Investigations Full blood count, blood cultures
Adults/children/immunocompromised Swab microbial/culture/sensitivities

As per preseptal Etiology
CNS involvement signs
Lumbar puncture
Emergency Nonimmunized children Child <2 yo
– H. influenzae
Whooping cough + meningitis risk
Intranasal steroids/decongestant
Urgent pediatrician Sinusitis Pain/congestion benefit
ENT Functional endoscopic sinus surgery

Fever
Proptosis, chemosis, injection Steroids – oral/IV

If not immunocompromised
Extraocular muscle restriction Ocular 7.19 Orbital Twice daily review
Cellulitis Admit
Optic nerve function
4-hourly optic nerve function
Visual acuity, pupils, color, saturation, visual fields, discs
Vital signs, mental status Cefotaxime 2g IV 8-hourly OR

Cerebral
Neurological exam, neck stiffness Ceftriaxone 2g IV daily + Di/
Flucloxacillin 2g IV 6-hourly
Decreased VA Clinical
Treatment IV antibiotics
Orbital apex
CN 2, 3, 4, 6 syndrome Then amoxicillin+clavulanate 12-hourly for 10 d
Multiple cranial Posterior extension 875+125 mg orally
CN 5.1 nerve palsies
Delayed
Sympathetic Consider vancomycin if MRSA
treatment
Bilateral multiple cranial nerve palsies Cavernous sinus Unresponsive to antibiotics
thrombosis
Rapid proptosis, mental status changes Optic nerve compromise
Intracranial Urgent surgery
Meningitis, brain abscess
extension Orbital/subperiosteal abscess
Death
Significant sinus disease
Canthotomy + cantholysis
Tight orbit + optic neuropathy or high IOP
Diabetic
Ketoacidosis
Immunocompromised Post-transplantation
Hematological cancers
Important
Iron overload, burns patients
Differential diagnosis
Scleritis, orbital myositis
Mucor, rhizopus, zygomycetes Filamentous fungi

Fungus
Cause Branching hyphae
Nasal cavity/sinus into orbit
Facial/periorbital swelling
Symptoms
Orbital apex syndromes

Diplopia, decreased vision, ptosis
Black eschar
Ischemic infarction + septic necrosis
Group A strep, S. aureus
Cause Signs Proptosis + soft globe
Immunocompromised Globe soft due to ischemia
Diabetes, alcoholism Orbital apex or cavernous sinus signs
Spread
More severe pain Necrotizing
Mucormycosis Clinical Motility, vision, corneal sensation
Fasciitis Sinus, orbit, brain
Bullous formation + necrosis Symptoms
Sinusitis, orbital/intracranial extension
CT scan
Secondary septicemia
Bony erosion
Investigation Cavernous sinus thrombosis, ICA thromboses
Treatment 7.20 Other Cellulitis &
Debridement + IV antibioitics Mucormycosis MRI
Superior to CT – assess need for surgery
Acute fulminating sinus infection
Biopsy
3 types Swabs unreliable
Aspergillosis
Chronic indolent sinus infection
Immediate hospitalization
Life threatening
Allergic aspergillosis
Multidisciplinary
ID, neurosurgery, ENT, endocrinology
Chronic inflammation Orbital
Obtain biopsy
Cold abscess Tuberculosis
Treatment 1-1.5mg/kg/d – total 2.5–3 g
IV amphotericin Monitor renal function
Impaired => use lipid formulation
Lipid formulation 5–15 mg/kg/d
+/– Exenteration
Wide surgical debridement
+/– Repeated surgery
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See Neuro-Ophthalmology chapter

General 3rd–6th decade
Associated with
Smoking
Thyroid Eye Bilateral but asymmetrical
Clinical
Disease Mild pain
Thyroid function tests

Consider an infection first
Orbital cellulitis Investigations EOM thickened, tendon spared
Trial antibiotics first CT scan
No fat stranding
Wegener's granulomatosis Steroids
Treatment Mild–moderate response
Polyarteritis nodosa Differential
Diagnosis
Metastases
3rd–6th decade
Lymphangioma with acute hemorrhage
Non-infectious, non-neoplastic,
General Must exclude these first
IgG4 positive plasma cells + high serum IgG4 Recently recognized space-occupying lesion
IgG4
Systemic disease Subset of orbital myositis
e.g. pancreatitis
Lacrimal gland, periocular tissues Sudden painful red eye
Symptoms
7.21 Orbital Unilateral
Rare, diagnosis of exclusion
Inflammation
Cavernous sinus Painful eye movements c.f. TED
Orbital apex Granulomatous inflammation
Etiology Signs Erythema, chemosis & injection
Superior orbital fissure Congestive proptosis & ophthalmoplegia
Diplopia + hemifacial pain
Optic nerve involvement
Tolosa–Hunt
Proptosis – mild Syndrome Idiopathic Orbital EOM and tendon thickened
Clinical
Motor + pupil Inflammation Disease
Fat stranding
Cranial nerve palsies CT scan
Sensory – 5.1 & 5.2 Scleral thickening
MRI Orbital fat or lacrimal gland
Ipsilateral enlargement cavernous sinus Investigation
Investigations Infective & neoplastic
Steroids Exclude Trial antibiotics
Very sensitive Treatment Biopsy
Pleomorphic cellular infiltrate
Long-term
May be lymphoma Follow-up ESR, FBC, UEC, ACE
Bloods
cANCA, pANCA
Mild Observation
Oral NSAIDs
Treatment Once confirm non-infective
Corticosteroids Start high 60–80 mg/d, slow wean over months
Dramatic response, over 1–2 d

Idiopathic small vessel multisystem
General Necrotizing granulomatous vasculitis
Nasopharynx/paranasal sinuses =>

Orbit
Proptosis, ophthalmoplegia
Dacryoadenitis, NLDO
Ocular
Clinical Peripheral ulcerative keratitis
Necrotizing scleritis, occlusive retinal periarteritis

Wegener's
Granulomatosis
Non-ocular
ENT/Respiratory/Renal/Dermatological
cANCA
90% sensitivity, high specificity
Investigations
Tissue biopsy
General
Small & medium muscular arteries 7.22 Vasculitis Steroids
Ocular Treatment + Cyclophosphamide

PUK, scleritis Risks: myelodysplasia, cystitis, bladder Ca
Rheumatology + ENT
Fever, weight-loss, malaise Clinical
Non-ocular Polyarteritis
Renal/Musculoskeletal/Skin/
Nodosa General
Cardiac/CNS/GI Small vessel vasculitis involving cartilage
Investigations Ocular
pANCA, biopsy Scleritis & anterior uveitis
Steroids + cyclophosphamide Swelling of pinnae
Relapsing Clinical
Treatment Polychondritis
Poor prognosis Non-ocular
Tracheobronchial cartilage, nasal
bridge collapse
5-y survival 10–20%
NSAIDs
Treatment
Steroids + immunosuppression
179
Adult Varices
Children Capillary hemangioma

7.23 Vascular
Orbital Lesions Orbital lymphangioma
Direct CCF
Carotid–Cavernous Fistula
Indirect CCF
Pathology
Vascular hamartoma
General
Slow growth first year of life
Dermis only
Superficial
Single or multiple
Types Posterior to orbital septum

Deep
Non-axial proptosis
Increases with Valsalva/crying
Combined
Intra/Extraconal mass
CT scan
Capillary Poorly defined margins
Hemangioma Investigations
Multiple feeder arteries & draining veins
Angiography
Hemodynamically rapid
Pathology
Not tumor, benign vascular hamartoma Astigmatism
Indications
Transilluminated cystic lesion Visual axis obscured
Superficial 7.24 Childhood
Beneath skin of eyelid
Vascular Lesions Propanolol
Non-axial proptosis Lancet
No increase with Valsalva Types
Deep Treatment Steroids
Spontaneous hemorrhage
Sudden enlargement Laser
Orbital Superficial hemangiomas
Low density cyst-like Lymphangioma
CT scan Surgery
Enlargement of bony orbit Investigations
Intravenous vincristine
Angiography
No arterial/venous connection
Surgical excision of chocolate cysts

Hemorrhage Treatment
Difficult surgery
Recurrence
181
182
Pathology
Benign encapsulated tumor
General
Late middle age
Intermittent proptosis & pain with exertion

Deep
Valsalva
Increase size
Benign encapsulated tumor Types
Pathology Varices Superficial
Lid/Conjunctiva swelling
Lateral part of muscle cone, posterior to orbit
Combined
Age 20–30, female Abnormally dilated irregular veins
CT scan
Progressive slow growth throughout life Investigations Multilobular lesions
General 7.25 Adult
Associated with Sturge–Weber Vascular Lesions Angiography
Venous connection may be present
Axial proptosis
Intraconal tumor Management Surgical removal
Friable, hemorrhage, incomplete excision
Most common intraconal tumor Clinical
Cavernous
Choroidal/Retinal Hemangioma
Well-encapsulated & intraconal
No bony erosion CT scan
Enhances with contrast Investigations
No feeder artery/No draining vein
Angiography
Stains late
Low flow lesion
Intraconal
Surgical removal Management
Choroidal
PDT
Carotid artery to cavernous sinus
High-flow shunts
General Defect intracavernous carotid artery
Head injury
Any time afterward
Triad – Pulsatile proptosis

History
“PricewaterhouseCoopers” Whooshing noise in head
Chemosis
"Corkscrew vessels"
Severe epibulbar injection
Pulsatile proptosis
+ Bruit/Thrill
Chemosis – hemorrhagic
Raised episcleral vein pressure
Raised IOP
Ophthalmoplegia
Trauma => 60–70%
Signs Corneal epithelial edema
Ischemia Aqueous cells & flare
Anterior segment
Iris atrophy, rubeosis irides
Cataract
Direct Optic disc swelling
ECA/ICA meningeal branches to Posterior segment Choroidal folds

Low-flow shunts
cavernous sinus General
Venous dilatation & venous
occlusive disease
Secondary to intracranial vascular thrombosis Congenital 7.26 Carotid–Cavernous CT/CTA or MRI/MRA with contrast
malformation Investigations Dilated superior ophthalmic vein
Causes Fistula
Angiography
Spontaneous rupture
Hypertension, middle aged, female Tinnitus
Patient
Mild epibulbar injection Cosmetic
Indirect
Pulsating mires Raised IOP (Dural Shunt) Treatment IOP, vision
Slow signs Examination
indications Cranial nerve palsy
Mild proptosis
Ophthalmoplegia Angiographic
Investigation Especially in cortical venous reflux
IOP
Pressure control Risks
Blood pressure Chronic pain, loss of vision
Treatment
Await spontaneous closure Interventional radiology
Definitive
Interventional radiology Beta-blocker
Treatment Raised EVP => decrease production
IOP control
Carbonic anhydrase inhibitor
Lubricants
Corneal exposure
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184
Dacryops Lacrimal gland ductal cyst

Most common cystic orbit lesion
Dermoid cyst
Cystic choristoma
Limbal dermoid Dermoid Cyst Herniation of intracranial contents
Non-cystic choristoma
Etiology Skull base congenital defect
Dermolipoma
Non-cystic hamartoma
Meningocele
Dura only
Types
Obstruction of paranasal sinus Pathology Meningoencephalocele
secretion drainage Dura + brain tissue
7.27 Cystic
Congenital Lesions Superomedial in orbit
Anterior
Narrowing
Etiology Location Forward & lateral globe displacement
Acquired
Infection, allergy, tumor, trauma Posterior
Encephalocele Anterior & inferior globe displacement
Proptosis, dystopia, diplopia Sinus Mucocele Infancy
Epiphora Clinical Increase in size

Upon straining
Painless
Pulsating Communication with subarachnoid space
Soft tissue mass
CT scan Clinical No bruit/thrill
Thinning of bony walls of sinus Hypertelorism, broad nasal bridge
Bony abnormalities
Surgical excision Cleft palate
+ Surgically re-establish normal sinus drainage Associations Ocular
Microphthalmos
Neurofibromatosis Type I
Posterior encephalocele
Solid, choristoma
Forniceal can extend posteriorly

White dome shaped on limbus
Scan
Clinical Inferotemporal 3/4 cases
Hair follicles
Duane's syndrome
Cystic, choristoma
Associated with Iris/Upper eyelid colobomas
Fetal dermal elements Limbal Dermoid Goldenhar
Benign developmental
Trapped in fetal bony suture lines Preauricular skin tags
Smooth, painless
Facial hypoplasia
Superficial
Mobile, posterior margin palpable Types Gonioscopy
Check deep extension
Deep Clinical 7.28 Dermoid
Proptosis, dystopia, mass effect Treatment Astigmatism/Visual axis affected
Vision, refraction, motility
Superonasal Excision
Differential diagnosis – encephalocele – CT scan Location Excision
Superotemporal +/– Graft if deep
Dermoid Cysts
Not mobile
Posterior extension Indications
Superonasal Solid, hamartoma
Dumbbell shape CT scan Adipose + dense connective tissue

Clinical
Dermolipoma Temporal fornix
Lucent capsule, homogenous fat intensity Findings
Re-modeling & defects of underlying bone Scarring, ptosis, dry eye

Risks
Not destructive Rarely require surgery
Ocular motility problems
Surgical excision
Eyelid crease incision
Management
Intense inflammatory reaction + fibrosis
Avoid cyst rupture
Excise before school
185
186
Yellow = Common
Gray = Rare
Embryonic tissue
Orbital teratoma
Paranasal sinuses
Breast
Adults Lung
Metastasis Blood borne
Lymphangioma GI
Vascular Kidney
Children
Capillary hemangioma Neuroblastoma
Embryonic tissue
Glioma Malignant orbit teratoma
Central (optic nerve)
Meningioma Nerves Vascular
Schwannoma Angiosarcoma
7.29 Orbital Tumors
Plexiform Peripheral Benign vs Malignant Malignant Schwannoma
Neurofibroma Nerves
Solitary neurofibroma Neurofibrosarcoma
Fibroma Fibrous connective tissue
Includes solitary fibrous tumor & Fibrous connective tissue Benign Malignant
Fibrosarcoma
ossifying fibroma
Fat
Fat Liposarcoma
Lipoma
Rhabdomyosarcoma
Muscle
Rhabdomyoma Leiomyosarcoma
Muscle
Leiomyoma
Bone
Osteoma Osteosarcoma
Bone
Malignant lymphoma – B- & T-cell
Fibrous dysplasia Lymphoid tissue
Lymphocytic leukemia
Benign reactive lymphoid hyperplasia
Lymphoid tissue Langerhans cell histiocytosis
Atypical lymphoid hyperplasia Malignant
Langerhans cell histiocytosis Adenoid cystic carcinoma

Benign
Lacrimal gland Adenocarcinoma
Lacrimal gland
Pleomorphic adenoma Lymphoma
Orbit, Oculoplastics, and Lacrimal System 187
Intraconal, Well Circumscribed Cavernous hemangioma

Neurofibroma
Intraconal, Poorly Circumscribed Lymphoma

Lymphangioma (generally in children)
AV malformations
7.30 Adult
Orbital Tumors Subperiosteal abscess
Mucocele
Extraconal
Lymphoid tumors
Meningioma
Greater wing of sphenoid
188
70% 1st decade, 90% by 2nd decade
General Slow growing astrocytoma
Associations
Neurofibromatosis 1 in 30%
Vision loss + proptosis
Edema => atrophy

Clinical Optic nerve
Optociliary shunts/collaterals
Chiasm
Intracranial spread
Hypothalamus
Middle-aged females Fusiform enlargement

CT scan Clear margin
General Intact dural sheath
Meningothelial
Two types Investigations Less defined in non-NF1 patients
Psammomatous Glioma
Vision loss Arises from nerve substance
MRI
Optic atrophy Triad Isointense to brain white matter
Fusiform shape
Clinical
Optociliary shunts
Regular MRI + visual function
Proptosis 7.31 Optic Nerve Observation Acuity & fields
Tumors Growth/decrease vision => treat
Thickening & calcification
Optic nerve CT scan Chemotherapy
Tram-tracking Treatment Especially if chiasm involved
Regular MRI + visual function Avoid in NF1

Observation Sheath Meningioma Radiotherapy Risk creating secondaries
Acuity & fields
Growth/decrease vision => treat
Surgery will blind patient
Vision loss Surgical excision
Large tumors/proptosis/growth toward chiasm
Large tumors & proptosis/growth Surgery
toward chiasm Treatment
Postoperative Adult, rare, poor prognosis
Malignant gliomas
Radiotherapy if incomplete excision Prognosis Death within 1 y
Chemotherapy Children/NF1
Uncommon Slow growing, intracranial growth life-threatening
Radiotherapy
Growing, vision loss
Children
Aggressive, can be fatal
Prognosis
Adults
Better prognosis
Breast
Lung
Prostate
Origin Skin
Melanoma
GI tract
Kidney
Dystopia & proptosis

Adults
Enophthalmos in scirrhous breast cancer
Ptosis, diplopia
Nystagmus Presentation Infiltration of orbital tissue Indurated periorbital skin
Known history in 80–90% Firm orbit

Neuroblastoma 7.32 Metastatic
Unwell child Tumors Chronic orbital inflammation
Orbital apex syndrome

Peripheral leukemic deposit CN 2, 3, 4, 5.1, 6
Chloroma
Preserve vision
Investigations
FBE with film Palliative
Children Treatment Relieve pain
aka eosinophilic granuloma Radiotherapy
Solitary > multifocal
Rapid onset Langerhans cell
granulocytosis
Bony destruction
Curette Rare; poor prognosis
Treatment Maxillary carcinoma
+/– Systemic chemotherapy Sinus Tumors Most common to invade orbit
Types
Ethmoidal carcinoma
Nasopharyngeal carcinoma
189
190
Orbital inflammatory Benign

disease
Hypocellular lymphoid lesion
Benign reactive 20% malignancy risk

aka Plaque meningioma lymphoid hyperplasia
Wrap around bone General
T-lymphocytes hypercellular lesion
Age 20–40, women Atypical lymphoid
hyperplasia 30% malignancy risk
Immature cells hypercellular lesion
Immature B-lymphocytes
Hyperostosis Grades
Low grade B-cell lymphoma
Arise intracranially, thicken bone Most common
MALTomas
Possible association
Association Malignant C. psittaci infection
Neurofibromatosis Type 2 lymphoma
Diffuse large B-cell Second most common: intraorbital lymphoma
Optic nerve compression Features Most common: intraocular lymphoma

Lateral orbital Sphenoid Wing
Lymphoma Follicular lymphoma
wall Meningioma
Cranial nerve palsies
Location
Secondary ocular adnexal Patients with systemic lymphoma
Proptosis, dystopia
lymphoma (SOAL)
Anterior cranial fossa Fresh

Flow cytometry, gene rearrangement studies
Temporal fossa 7.33 Other Biopsy
Fixed
Orbital Tumors
Palpable mass temporally Immunohistochemistry
Observation Investigation
Neurosurgeons FBE with film

Surgery
Debulking Treatment Oncologist PET scan
Radiotherapy MRI or CT Chest/Abdomen/Pelvis
Suspect secondary (SOAL)
Associated with NF-1 Plexiform Neurofibroma

Low dose radioimmunotherapy
Antibodies with radioactive conjugates
Local disease
Treatment + Monoclonal antibodies
Rituximab
See Pediatrics
Rhabdomyosarcoma
Rituximab
Systemic disease
+ Conventional chemotherapy
Fibrous dysplasia
Meningioma
Fourth Age:
13–19 y – Relatively Trauma
Safe Age
First Age:
Teratomas Birth Tumors – Very Bacterial orbital cellulitis
Bad Age
Rhabdomyosarcoma Growth spurts of neurofibromas
Uncommon
Retinoblastoma Thyroid eye disease

Metastatic neuroblastoma Second Age: Mucocele of sinus

0–5 y – Fifth Age:
Moderately Bad 20–40 y – Relatively Sphenoid wing meningioma
Choristomas
Dermoid cysts, limbal dermoids Safe Age
Lacrimal gland tumors
Hamartomas
Capillary hemangiomas Inflammatory diseases
7.34 Seven Ages of
the Orbit
Rhabdomyosarcoma
Langerhans cell histiocytosis Rare in children

Sixth Age: Cavernous hemangiomas
Chloroma Third Age: 40–65 y – Relatively Most common intraconal mass in adults
Leukemia deposit, green on excision 5–12 y – Very Safe Age
Bad Age Lymphoma
Lymphangioma
Idiopathic orbital inflammatory disease
Glioma and optic nerve sheath meningioma
Lacrimal adenoid cystic carcinoma

Orbital metastases
Seventh Age:
Lymphoma
65+ y – Extremely
Bad Age
Spread of BCC and SCC from
skin, sinuses, and adnexa
191
Size
192
e.g. anophthalmos, nanophthalmos

Infection
Cellulitis
Forniceal loss
Socket
Age 0–3 Cicatricial diseases
Orbit contraction Trauma, OCP, radiation, burns
Advantage Dermis fat graft Postenucleation socket syndrome

No extrusion Autogenous See separate
Graft absorption variable Allow socket to heal +/–

Disadvantage dermis fat graft
Up to 30% Pyogenic granuloma
Inflammation
Tissue expander Secondary implant later
Intraconal with subcutaneous tube Epithelial inclusion cysts
Rough surface => tissue reaction

Infection Acute
Hydroxyapatite or Swab + treat infection/cellulitis
Blood vessels & fibroblasts
Medpor
growth into surface Excise & cauterize granuloma
Early postoperatively
Socket Extrusion/ Re-suture wound
Advantage – can be pegged Scleral patch grafts No tension on wounds
Improve motility Integratable Types Complications Exposed
Disadvantage or autogenous tissue grafts
Promote conjunctival healing
High rates extrusion Implant
Remove implant & Wait 2–3/12 before another implant
Acrylic ball Non-autogenous donor sclera
Spherical – greatest volume for surface area Nonporous
Severe Reduced infection risk
Advantage Non-integratable Exenteration
Lower extrusion than integratable Dead space
7.35 Orbital Accumulate material behind implant
Lower infection Disadvantage
Less motility Implants Epithelial ingrowth behind implant
Donor sclera Repeated extrusion
Implant cover Infection
Autogenous fascia lata Giant papillary conjunctivitis
Largest possible size Conjunctiva
Wound surface breakdown
So prosthesis can be small, light Implant
Lid Incomplete closure, poor prosthesis fit
Largest possible size Prosthesis
Leave 2 mm lagophthalmos Conformer Pyogenic granuloma
Prevents fornix shortening Entropion/Trichiasis
Technique
Tarsorrhaphy Poor lacrimal drainage system
Tips
Temporary – 1/52
Pressure bandage Old, poorly fitting prosthesis
For 1/52
Enophthalmos, deep upper lid sulcus
Avoid cutting – opens meninges
Optic nerve Gaze upward
Risk intracranial spread of infection Evisceration Features
Ptosis
Closure WITHOUT TENSION
Sclera Lax lower lid, shallow inferior fornix
Avoid braided nonabsorbable – infection risk Post-Enucleation Secondary implant, or larger implant
Uvea Socket Syndrome Orbital volume replacement Orbital floor implant
Remove all to prevent sympathetic ophthalmia
Dermis fat graft to upper sulcus
Management
Lower lid tightening
Lateral tarsal strip
Ptosis correction
Fornix deepening sutures

8 Pediatric Ophthalmology
Pediatric
Ophthalmology
8.1 Management Principles
8.2 Visual Acuity
8.3 Strabismus Visual Assessment 8.28 Optic Disc Abnormality

8.29 Morning Glory & Others
8.4 Binocular Vision
8.5 Classification 8.30 Pediatric Uveitis
Amblyopia
8.6 Management
8.31 Hereditary Vitreoretinal Dystrophies
8.7 Occlusion Therapy – Evidence
8.9 Esotropia Treatment 8.8 Esotropia

8.32 Retinopathy of Prematurity
8.10 Exotropia 8.33 Screening & Treatment
8.11 Exotropia Treatment
8.12 Pattern & Vertical Strabismus 8.34 Dangerous

Strabismus 8.36 Clinical
Tumors
8.13 Special Strabismus 8.35 Benign
8.37 International Classification (ICRB)
8.14 Consecutive Exotropia Retinoblastoma
8.38 Treatment
8.15 Squint Surgery & Botulinum Toxin
8.39 Genetics
8.16 Squint Surgery Complications 8.40 Pediatric Glaucoma
8.41 Management
8.17 Assessment – DWARFF
Nystagmus 8.43 Assessment
8.42 Pediatric Cataracts
8.18 Congenital
8.44 Management
8.19 Acquired – MRI All
8.45 Syndromes
Lacrimal Drainage System
8.46 Trisomy 21 – Down Syndrome
18.20 Obstruction
8.47 Connective Tissue Diseases
8.21 Congenital Eyelid Anomalies
8.22 Congenital 8.48 Mucopolysaccharidoses

Infections – TORCHES
Infections & Allergies
8.23 Ophthalmia
Neonatorum 8.49 Leucocoria & Dragged Disc Differential Diagnoses
8.24 Orbital Cellulitis 8.25 Anterior Segment

8.50 Non-Accidental Injury
8.26 Iris Abnormalities – Aniridia
8.27 Blindness at Birth
193
194
Age appropriate vision – check presence
Pathology – exclude
Order of Management Refraction – cycloplegic
Baby in A PRAM
Amblyopia – manage
Muscle – manage alignment & strabismus
8.1 Management
Principles Genetic counselling
Reversible causes
Opinion
Second opinion
Issues in Management Specific treatment
GROSS!
Genetics of condition
Supportive measures
Reversible – cataract, refractive

e.g. retinal dystrophies
Specific – vitamin A
Supportive – guide dogs/support groups

Fix, follow, and Test each eye's ability to fix and follow
objections to cover
Asymmetrical objection to eye cover
General habitus and interaction May indicate amblyopia
History/Observe/ Central
Facial appearance – asymmetry, Systems Neonates, Corneal light reflex near center and symmetrical for both eyes
Pediatric Ophthalmology
lesions, malformations Young Infants

Under monocular conditions
and Nonverbal
Children Steady
Head posture
CSM test Steadiness of fixation
As target motionless & then moved
Maintained
Near
In child – better assesses amblyopia Ability to maintain alignment Alternating freely
Visual acuity with first eye, then with the other,
as the opposite eye is covered Holds through blink
Distance
Maintenance of fixation indicating amblyopia Holds well
8.2 Visual Acuity Holds briefly
Cover/Prism cover test
Not holding
Visual evoked potentials
Versions Test worse eye first
Motility exam
Hundreds and thousands
Ductions
Order of Preferential Present both hands, one with, other without
Accommodation Assessment looking tests
Simultaneous perception Infants and Teller acuity cards Assess resolution acuity, not Overestimate VA
Toddlers recognition acuity
Binocularity
Binocular single vision
Fusion vs suppression Familiar pictures with variable outline width
Cardiff acuity
Stereopsis cards
Pupil exam Gray background
Distance & near
Cycloplegic Kay pictures Measures resolution, detection & recognition
Check ciliary body function – patient refraction
accommodating or not Dry refraction Age 2–5 y
Single Sheridan–Gardner
Age >3 y
e.g. post-trauma mydriasis – Full ocular exam Both underestimate No "crowding effect"
?accommodation present (optical media, fundus) amblyopia
Can use crowded SSG chart
School Snellen-based
Children
Consistent crowding at all levels
LogMAR
195
196
1 mm = 7 degrees = 15 PD Gross heterotropias Heterophorias, dissociates tw

eyes for near fixation
Pupil margin
2 mm = 15 degrees = 30 PD R eye sees white vertical,
Look at Hirschberg's
Mid-iris symmetry test red horizontal arrow
4 mm = 30 degrees = 60 PD Maddox
of light reflex
wing L eye sees vertical & horizontal
Limbus row of numbers
45 degrees = 90 PD 1. Light
Reflection Tests
Krimsky's test Patient – which number
Prism in front of fixating eye until light reflex symmetrical arrow points at
Direct ophthalmoscope, symmetry of red reflex Heterophorias, dissociates two

Bruckner's test eyes for distance fixation
3. Dissimilar
Brighter reflex from deviated eye Image Tests
(in place of Uses
Cylinder red rods, white
Cover – detects heterotropias, cover tests) refraction
spot of light into red line
look at uncovered deviated eye perpendicular to rod axis
Cover &
uncover test Maddox
Uncover – detects heterophorias, rod Rod in front of Red line temporal to
look at uncovered eye for deviation/refixation deviated eye light => esophoria
Prisms – to measure Red line nasal to

Detects heterophorias Alternate light => exophoria
degree of squint
cover test
Look at uncovered eye for movement 8.3 Strabismus
Hess test – dissociates two
Heterotropias Simultaneous eyes with red & green filter
prism cover test
Lee's screen – dissociates
Cover one eye, prism over other
two eyes with mirror
until no movement
Smaller field Abnormal eye with
Total deviation (heterotropias & phorias) limited movement
Alternate prism
cover test 2. Cover Tests
Prism over deviated eye, alternate cover each eye until no movement Distance/Near Normal eye, outward
displacement = overaction
Larger field
= Accommodative convergence/accommodation in that direction
AC/A ratio
Hess test/
(Deviation without lens – deviation No deviation or
Gradient method Lee's screen Equal size field
with lens)/(lens in diopters) equal deviation
Assess in all XT – prior to surgery Results
Mechanical restriction
Breaks down tenacious 45–60 min occlusion in opposing directions
Narrow field
proximal convergence Occlusion of (blow out fractures)
Marlowe
Do prism base cover test immediately postocclusion Sloping field
=> A or V patterns
Allows AC/A ratio assessment – maximum deviation
Is there fusion
or suppression? Eye movement in direction of apex
Microtropia check
Look for corrective movement of eye
Cortical Sensory fusion
unification Simultaneous 4 PD base out => Central suppression/abnormal
perception Definition: prism test retinal correspondence
Vergence Motor fusion Three No movement
movements Components No corrective movement in eye with microtropia
Binocular
Fusion single vision
Dissociates two eyes for distance fixation
Suppression
Stereopsis Worth's four 4 dots – red, white, two green
dot test
Phoria/intermittent tropia Cover test R – Red lens, L – green lens
implies fusion present Glasses with fine striations, 45
2. Binocular deg to each other 4 lights = normal fusion
Single Vision
Check if disparity created Tests Point of light into line perpendicular 4 lights in manifest squint =
evokes fusion response striations (like Maddox rod) anomalous retinal correspondence
Results
When break & Principle based on interference/diffraction
2 lights = left suppression
regain single vision (c.f. refraction in Maddox)
Fusional Prisms 8.4 Binocular 3 lights = right suppression
Break point Bagolini Patient sees point of light
amplitude
Vision striated
Regain point glasses 5 lights = diplopia (uncompensated ET/XT)
Lines cross at center = normal fusion
Look at eye
Eye moves in to Base out over
under prism Results Lines cross at center in patient with squint = ARC
take fixation one eye 1. Motor
20D prism
Fusion
(infants/
One line missing = left or right suppression
Opposite eye moves toddlers)
out initially, then in 600–15 s, 3 clear plastic plates
Lines do not cross center = diplopia
– 4 squares, hidden circle in one
Horizontal fusional convergence Frisby plates
stronger than divergence/vertical fusion
Steropsis Pick square with circle in it
tests
See
Equal vision both eyes – alternate True 3D tests 3000–40 s, Fly/Circle/
below Titmus test
fixation spontaneously Animal, need polaroid glasses
1900–15 s, 7 plates (squares,

If one eye doesn't => amblyopia 3. Stereopsis dots), need red green glasses
Tests
TNO random dot
Assess motor fusion Dissociated 2D tests No monocular clues – better than Titmus
Dissociates two eyes for both near Lang test 1200–200 s, plates (moon, sun)
fixation & distance fixation
Synoptophore Mentor B-VAT No glasses, no monocular clues
Two cylindrical tubes with pictures
at each end of tube
Microtropes always fail stereopsis tests Distance stereopsis test
197
198
Decreased VA >= 2 lines on Snellen
Normal ocular exam, no afferent pupillary defect

Likely in constant tropias
Crowding phenomenon Strabismic
VA better for single
optotypes than multiple Uncommon in intermittent XT
Eccentric fixation Clinical Features
Decreased contrast sensitivity &

decreased brightness perception
Monocular suppression of amblyopic eye
Decreased perception and reaction times Bilateral hyperopia (>4D) – more common
8.5 Amblyopia
Classification
Bilateral myopia (>6D)
Ametropic
Bilateral amblyopia – unlikely

myopia due to focus of near objects
Ptosis
Refractive Errors
Unequal RE between two eyes
Corneal opacities
Stimulus Deprivation Anisometropic Anisohyperopia – difference in hyperopia >1.5D

Congenital cataracts
Anisomyopia – difference in myopia >3D
Other media opacities Meridonal
Uncorrected astigmatism of >1.5D
Iatrogenic – occlusion
Cataract
1. Exclude Organic Causes of Poor Vision

Refractive errors
Tumors
Refractive correction
2. Remove Obstacles to Clear Vision
Cataract surgery
3. Occlusion Therapy (gold standard)
See next
8.6 Amblyopia
Management
Reserved for patching failure
1% atropine in good eye to blur eye for near vision

4. Penalization
Pharmacologic Atropine for VA >6/36 only (mild–moderate amblyopia)
Levodopa/Carbidopa – currently being tested
Degrade image in better eye

Optical
CAM visual stimulator
Undercorrecting refractive error in better eye
5. Others
Pleoptics
(Unproven alternatives to patching)
Education
6. Prevention
Screening
Red reflex at birth

199
200
6 h = full-time patching = weekend

Severe amblyopia (6/30–6/120)
atropine (with >1 h near activity)
3–7 yo
Moderate amblyopia (6/12–6/24)
2 hr patching = 6 hr = daily = weekend

atropine (with >1 hr near activity)
7–17 yo 75% improve 2 lines

Glasses correction alone can improve BCVA
25% resolve
Age, cause, severity determine amount
7–12 yo Can improve BCVA even if previously treated

Risk – can break down fusion, Start patching when detect amblyopia
Glasses + patching + atropine
manifest strabismus 53% improved vs 27% controls (got glasses only)
Adhesive patches
PEDIG
Patching useful if no previous patching or atropine
Practicalities Amblyopia
Full-time occlusion should not be >1wk/year of age Studies 13–17 yo
Of 25% that improved, 20% regressed
Continue until VA stable for 3–6 mo
3–10 yo Glasses correction enough
Bilateral amblyopia
Maintenance patching until age 9 may be required
Can occur a year after ceasing treatment

4 reviews per year suffcient 8.7 Amblyopia –
Occlusion Recurrence
42% had decreased VA after stopping 6h/d patching
Therapy – Evidence
Consider failure
Reduce to 14% with tapering rather than stopping
Wrong diagnosis
Not necessary
Noncompliance No Response Near work
after 3 Months
Uncorrected refractive error Probably useful – want them doing activities
Failure to prescribe suffcient treatment Bangerter filters

Neutral density filters = patching
Irreversible amblyopia Atropine Light sensitivity 18%
Only 26% side effects
Lid irritation 4%
UK 2004, 94 patients, anisometropic/strabismic amblyopia
Headache 2%
Age <4 y: <3 h/d effective
Facial flushing 1%
MOTAS Age >4 y: 3–6 h/d much better than <3 h/d
Studies
Age 6–12 y: need 6 h/d
More better, but less ok; full-time – risky, less compliance

Acute/Diplopia/Age >6 y
Do imaging
Establish comitant
and full abduction Onset b/w 6 mo & 7 y
Features
Following occlusion or loss of vision
in one eye +/– hypermetropia Usually intermittent,
Uncorrected hyperopia –
becoming constant
average amount is +4D
Increased tone of MR with Myopia
Focal Accommodative Frequently associated
close reading
neurology Refractive convergence with amblyopia
Associated with Acute Accommodative

Nystagmus Esotropia Normal AC/A Decompensation of fully
Space-occupying lesions
accommodative ET
CN 6 palsy
+/– Insuffcient fusional
Causes
Hydrocephalus divergence amplitude ET that subsequently developed
Arnold–Chiari malformation accommodative component
Partially
Acute comitant esotropia,
accommodative
but not accommodative Idiopathic
Normal AC/A
Cataract
Comitant – Sensory High accommodative
Corneal scarring Deprivation convergence/accommodative
Non- Convergence
refractive excess
Optic atrophy Esotropia is greater at near, reduced
Esodeviation varies in different fields of gaze by +3D bifocal lenses
Anisometropia, Comitant -
astigmatism Acute esotropia Acquired
Amblyopia but no Very small angle manifest ET – 8 PD or less
Rule out neurological problem
obvious strabismus
Tumor => CN 6 palsy Neurologic Central suppression scotoma of deviating eye
(+/– nystagmus) Microtropia Anomalous retinal
Hydrocephalus monofixation correspondence With reduced
Documented presence of
8.8 Esotropia syndrome stereopsis
ET by age 6 mo
Raised ICP => CN 6 palsy
Anisometropia
Symptoms
Medial rectus rare Equal visual acuity
Thyroid eye disease
restriction Check with 4 PD
base out test Features Large deviation
Medial wall fracture & entrapment Incomitant >30 PD
Acquired childhood – 1/3 Isolated CN 6 palsy DVD

intracranial lesions Infantile
(<6 mo) Associations IO over-action
Mobius syndrome
Bilateral CN 6 & 7 palsy Lateral rectus Non- Latent nystagmus
weakness Accommodative
Slipped/detached LR – trauma/surgery Asymmetric optokinetic nystagmus
Develops after Exclude by demonstrating full abduction
Epicanthic folds 6 mo age
Specific Duane's syndrome Type 1
Narrow interpupillary distance Near = distance Differential
deviation diagnoses Mobius syndrome
Negative angle kappa Pseudoesotropia Basic
(>6 mo) Exclude CNS CN 6 palsy
Face – asymmetry lesions
Secondary – sensory
Globe – proptosis, enophthalmos General
Intermittent or constant ET for

Pupils – miosis, mydriasis, heterochromia distance, ortho for near
Distance esotropia
Divergence insufficiency
Normal bilateral abduction &
201
absence of neurological disease

202
Refractive error
Patching – careful – can dissociate

Microtropia into large deviation
Full correction of hyperopia
Treat amblyopia Refractive They need ARC to keep eyes straight
Don't operate Most patients – stable & symptom free
Bifocals – executive, power +3D

Refraction After refraction
Long-acting cholinesterase inhibitors

Non-refractive
Age <12 mo – 4–6/12 good for stereopsis
(High AC/A)
Surgery – adolescents – aim straight in Acquired Acquired Infantile
distance Accommodative Non-accommodative Aim – orthophoric in glasses
Observation
Bilateral medial
Surgery rectus recession Or less frequently unilateral MR
Full refractive correction recession/LR resection
Partially
Surgery – operate on angle after maximum
accommodative 1/3 require multiple surgeries
correction child can tolerate
Many result with mono fixation

Aim – straight in glasses in distance syndrome – successful surgical result
8.9 Esotropia
Treatment
Amblyopia & surgery early to help
with binocular vision
Spontaneous resolution in Basic
Sixth nerve 10% re-operation rate
1/2 with traumatic palsies
Trial with Fresnel prisms
Forced duction test preop
Check for restriction Incomitant Prisms until spontaneous resolution
Iatrogenic (postop) Distance esotropia
Surgery in persistent cases
Rule out neurological cause
Acute esotropia Correct Rule out neurological cause
Surgery obstacles
Acute esotropia
Surgery
Sensory 2% re-op rate
Strabismus surgery
only in abnormal eye
Wide interpupillary distance
Specific
Positive angle kappa
Pseudoexotropia
Face – asymmetry, craniofacial
abnormalities – e.g. Crouzons
Exotropia (XT) with
e.g. nystagmus, pale disc, another sign
XT ~ same in distance as near General Globe – proptosis, enophthalmos
restrictive disease
Consider Basic
Midbrain Lesion 37% of intermittent XT (Kushner) Pupils – miosis, mydriasis,
New XT age >6 y
Check refraction, fundus heterochromia
Greater at distance than near by >10 PD

Convergence insuffciency age <10 y
60% have high AC/A
Age <6/12 mo If high => keeps eye converged in near

High AC/A ratio
Look for sensory XT signs 8.10 Exotropia +3D test makes near = distance deviation
Large & constant angle Distance XT – High AC/A and no change with occlusion
Infantile divergence => high AC/A cause
excess
Dissociated vertical deviation (DVD) & Simulated
inferior oblique (IO) overaction High AC/A and change with occlusion
=> high AC/A + TPF
Normal refraction Tenacious proximal fusion Normal AC/A and near to distance closer
(occlusion of Marlowe Test) with occlusion => TPF
Anisometropia
Constant Intermittent i.e. near deviation increases when
Near remains < distance after dissipate TPF with occlusion
Media opacities True Marlowe Test & normal AC/A
Normal AC/A and no change with

Optic atrophy or hypoplasia Sensory occlusion => true divergence XT
Greater at near than distance by >10 PD
Macular lesions
Low AC/A – 11% of intermittent XT
Amblyopia
Near XT –
convergence Fusional convergence insuffciency <1%
XT follows previous surgery for esotropia insuffciency
Consecutive Rarer
Slipped Early postop Medial rectus Pseudoconvergence
muscle underaction insufficiency <1% Distance deviation increases post
Late occlusion of Marlowe
Stretch
tendon scar
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204
Myopia – correct – over-minus

Refractive – over-minus
Amblyopia Hyperopia – don't correct

Minus glasses -2D – for angles mild–mod – worsen deviation
<20 PD not long-term
Over-minus
Especially useful 4–6 h daily
XT rare
in high AC/A
4/12 trial
XT <5x for distance Part-time occlusion – anti-
suppression treatment Deviating eye, or alternate eyes
History Non-
XT >5x for distance surgical Prisms – base-in Pencil push-ups, stereograms
Temporary
XT >5x for distance Useful in convergence Temporary
Near deviation => & near Orthoptic convergence exercises
insufficiency benefit
less control
Prognosis
1/2–2/3 improve with nonsurgical methods
Assess
After dissociation – Cover Test Intermittent When intermittent becomes
XT Control 60–80% constant XT
Re-fuses rapidly without Good control
blinking/re-fixation Angle >15 PD
Surgical
Re-fuses with Fair control Fusion timing Losing stereopsis
blink/re-fixation
Intermittent
Exotropia Age <4 y – increase fusion, decrease re-op
Breaks Poor control
spontaneously
Age >4 y – decrease amblyopia from postop ETs
Operate On maximal
No control
Constant XT deviation Occlusion of
XT size Marlowe preoperatively
Basic
Greater size => less control Higher risk consecutive
Bilateral LR
8.11 Exotropia ET or microtropia
Recession (BLRR)
Treatment Surgery can break
down fusion Warn bifocals for
Ocular/Neurological Simulated –
consecutive ET
pathology Surgical high AC/A
Delay, older age (10) better, can grow out of high AC/A
Infantile Distance XT
Early surgery BLRR – no MR resection, do no want further tightening of MR
Otherwise higher (like infantile esotropia) Simulated –
amblyopia rates high TPF Lower chance of consecutive ET
Constant
Exotropia Recess/Resect or BLRR
Sensory True
BLRR
Treat cause
Near XT
Bilateral MR resection
Consecutive
Type of preceding cause & surgery <30 PD – BLRR
Adult Technique Prism subjective test
options >30 PD – Recess/Resect
Botox 42% long-term success
(Dawson et al. [1999])
1/4 of horizontal strabismus cases
Features
Primary – not a/w SO paralysis
SO overaction – A = 10 PD, V = 15 PD or greater difference
Secondary – with antagonist SO palsy/paresis most common between up & downgaze
Develops in 2/3 of congenital ESO, A LR underaction

also in acquired ET/XT IR underaction
Overaction Causes
In adduction and lateral upgaze – eye elevated ESO – bilateral CN 6 palsy
A&V Correct underlying
In IOOA – higher & lower eyes Pattern V EXO – IO Overaction horizontal strabismus
reverse in opposite lateral gaze due to CN 4 palsy
Oblique muscle Weaken IO
Elevation in adduction Brown's syndrome dysfunction
only, not in primary C.f. DVD Tuck SO tendons
Craniofacial anomalies –
V pattern in IOOA, none in DVD e.g. Crouzon syndrome
Inferior Treatment While doing horizontal, Medial rectus to Apex of pattern
Oblique can do MALE
Weaken IO – insignificant effect
Lateral rectus to Empty space
on primary position 50–90% of
congenital ET Presence of DVD – excludes late acquired
Treatment ET/partially accommodative ET
Treat associated horizontal
deviation at same time Paralysis Usually due to obliques
Rare
8.12 Pattern &
Vertical
Features Eye – up, out and extorsion – on occlusion
Strabismus On removal, opposite
eye does no go down
Most common cause of torticollis in children
Primary, associated paralysis
of IR or IO uncommon Does not go away as get older
Dissociated
Lower eye – has overacting SO Vertical deviation in Vertical When spontaneous/frequent/cosmetic issue No IOOA – superior rectus recession
primary position Deviation or Faden Fixation suture
Overaction
Overdepression in adduction Types
Surgery IOOA – IO
Treatment recession weakening
Superior Postop hypotropia risk in unilateral DVD
Hyper/hypotropia Oblique
Bilateral SO tendon To minimize, recess both eyes
or A pattern
weakening Change fixation preference with patching
Treatment
Affects primary position – seldom done Direct = rim/floor, Blowout = floor only
Ecchymosis/Parasthesia/Parasthesia Enophthalmos
Congenital or acquired, nerve or muscle
Hypotropia in primary position

Head tilt toward shoulder opposite to weak SO side Features
Orbital Floor IR can become
Limited elevation/depression => diplopia
Antagonist IO weakening Fracture ischemic
Unilateral Paralysis Urgent Prolapsed IR trapped beneath trapdoor fracture
Severe – add surgery Pain on upgaze
contralateral IR recession
Treatment Oculocardiac reflex
Treatment If no IR entrapment
Bilateral
Observe
As unilateral, but both sides
If IR weakness does not reverse in 6/12 => surgery
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Absence CN 6 nuclei – aberrant innervation of LR by CN 3 Poor elevation in adduction – IMPORTANT

4th week gestation
Cause Superior oblique Classic Less poor in midline
Associated with Goldenhar, Wildervanck tendon sheath
syndrome (deafness, spine abnormality) Normal elevation in abduction
syndrome
Globe retraction on
85% Unilateral, females, left eye 60% No SO overaction
attempted adduction
Triads Vertical gaze V Pattern
Upshoot or downshoot c.f. CN 6 palsy
in attempted adduction Hypotropia in primary position
Do if primary position deviation/abnormal Features
Duane Positive forced duction testing
head posture/globe retraction/large Additional
upshoots/downshoots Syndrome Downshoot in adduction
50–80%. Poor abDuction Surgery
primary position ET Never resect – worsen retraction Congenital Widening of palpebral fissure on adduction
Type 1 –1 'D'
Recession MR Negative forced duction testing, A
IO Palsy
involved side Poor aDDuction Differential
pattern, SO overaction
primary position XT diagnoses
Brown Syndrome
Poor abDuction & aDDuction Type 2 – 2 'D's SO overaction/IR fibrosis/Contralateral DVD
"SO Brown,
primary ET/XT/no deviation Recession of LR involved side
Add Down!"
Double elevator palsy
Type 3 – 3 'D's
If severe – MR & LR recession
Internuclear ophthalmoplegia Observation – as child gets taller, less need to look up
See Neuro-ophthalmology
Surgery – primary position/hypotropia/
Treatment head posture/cosmetic
Bilateral 6 + bilateral 7 (asymmetrical) palsies
Mobius syndrome Spacer under tendon sheath
Ptosis + slowly progresses to
Associated with CN 9 & 12 palsies Surgery
total paralysis eyelids/EOM 8.13 Special Simple
Tenotomy
+ Pigmentary retinopathy + CPEO
Strabismus Trauma – accidental/surgery Augmented
cardiomyopathy =>
Kearns–Sayre syndrome Myasthenia gravis Systemic inflammatory, e.g. RA
Uncommon in children Causes
Sinusitis
Autosomal dominant Marfan syndrome, acromegaly
Congenital fibrosis
Spectrum – Isolated fibrosis to Features
Strabismus fixus – horizontal recti of EOM (CFEOM) As per congenital, a/w PAIN + diplopia commonly
all EOM – restrictive external
ophthalmoplegia + ptosis Oral NSAIDs
Vertical retraction – involves SR Others Acquired
Asthenopa => reading diffculty SO tendon steroid injection

Convergence Treatment Antibiotics for sinusitis
No manifest squint (c.f. convergence insuffciency XT) insuffciency
Avoid surgery, generally resolve
Damage to vertical
Treatment
gaze center Inferior rectus Miosis
restriction Pencil push-ups
Dysinnervation Congenital Features Partial ptosis
syndrome/CFEOM Type 1 Supranuclear Causes Horner's Syndrome
Heterochromia
causes
50% have ptosis Monocular elevation deficiency
Forced duction test (double elevator palsy) If patient well, no disc changes,
Tight IR Cranial CN 3
fontanelles soft, no other neurological
Nerve Palsy features => no imaging necessary
Treatment CN 4
See Chapter 5 Investigations
Large IR recession +/– vertical transposition CN 6 History – trauma/forceps delivery
Exclude pathology
CN3, sensory cause
Ductions, versions
Measure deviation
Prism cover test
Hess chart
Forced duction test

Assess restriction Slipped over recessed MR vs tight LR
Checks whether muscle attached

Force generation test
Assessment
Check power of muscle
30% consecutive Complete vs partial
XT over 25 y
Perfect congenital
ET surgery Diplopia
Incidence One of three outcomes
ET correction "does not
grow" with patient Postoperative Suppression
diplopia Prisms
Binocular fusion
LR botulinum
8.14 Consecutive
Exotropia Synoptophore
Worth 4 dot test
Stereopsis
Underdiagnosed hypermetropia
If present => more likely to fuse postoperatively
Surgical error
Wrong recession/Resection Etiology Non-surgical Trial LR Restrictive LR (on Forced Duction Test)
botulinum/prism Recess LR
Slipped knot
Early XT
Treatment Advance MR
Poor/Aberrant early healing
No restriction
Late XT Recess LR XT can recur
Stretched scar
Surgical
Finding MR difficult
Difficult surgery Fat may be present
No surgical tables
~70% do very well
Outcome Adjustable sutures necessary
~10% poor outcome
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Rectus
Adults
1 Recession
Primary gaze abnormality
Vertical squints
AHP Indications for
Relieve abnormal head posture Indications Thyroid eye disease
Surgery: 1-ABCD
BSV 1ABCD Blow-out fractures
Increase field of binocular single vision
CN 6 palsy
Cosmesis
Improve appearance Adjustable Re-operations
Surgery
Diplopia Children
Improve binocular diplopia
8.15 Squint Contraindications Patient uncooperative

Realistic expectations, future follow-up
Surgery &
Oblique dysfunctions & DVD
Treat first Botulinum
Toxin Pattern ET
Amblyopia Concomitant nystagmus
Will not be fixed by squint surgery
Congenital XT
Red Flags – Be
So not an indication Congenital eso/exo better for early surgery
Wary of Outcomes Incomitant deviations
for surgery Timing
Treat later if parent/patient ok Childhood
Acute onset ET
Restrictive
Consistent prism cover test
Restrictive vs palsy Duction = version
Palsy
Prism subjective test Duction > version
Diplopia when eyes made straight
Undercorrect
EMG guided
No => surgery Principles Muscle length-extension curve changes
Diplopia when eyes
Prism subjective test
made straight
Yes => Botox pretreatment Adult Method Synapse damage, Sarcolemma density changes
2–3/12 synapse regrows
Botox pretreatment => Botox effect persists >3/12
Temporary straighten, test if patient can fuse
General with ETT Small angle ET <15 PD, consecutive ET

Anesthesia Treatment
Bilateral topical with IV sedation Augment in large angle congenital ET
Botulinum
Toxin
Weakening – recession & disinsertion
Longstanding strabismus
(for IO overaction commonly)
Resection Testing Potential diplopia/ Even without full paralysis, have range of
binocularity orthotropia to test binocularity
Advancement Techniques
Strengthening
TED, CN4 palsy – IO
Tucking – enhance SO Not useful
function in congenital Never resect
restricted muscles Duane's – equivocal
4 palsy
Malignant hyperthermia
Myocardium – oculocardiac reflex
Partial + full thickness muscle bites
Muscle slip/loss Use operating microscope

Suture muscle to sclera

Maximize exposure, hemostasis, no blind hooking
Surgery – 2–5 d postop
Intraoperative Follow sclera, confirm slippage
Cannot find => transposition of adjacent muscle
Make hole in sclera – perforation

Retinal detachment, endophthalmitis
An infection
Complications
MAD Alignment
See overcorrections below
Anterior chamber activity/Corneal edema

Immediate
postoperative Anterior segment ischemia Rebeosis/Neovascular glaucoma
8.16 Squint Surgery
Plus lenses => to relax convergence
Complications Topical steroids/Cycloplegia/Analgesia
Try alternate patching Aim up to 15D
overcorrection Adherence syndrome
Fat e.g. with IO recession
of XT, if >15D:
Still present after 2 mo => Allergic reaction
re-operate on virgin muscles
Overcorrections Diplopia
Amblyopia
Dellen & conjunctival
Push minus (up to 3D) With cysts
overcorrection Palpebral fissures –
Late e.g. with IR recession
Patch alternately of ET Disfigurement
postoperative
If these fail => re-operate Lateralization of caruncle
Infection 1:10,000 Develop an exotropia

Serious Consecutive XT
complications Blinding 1:30,000
Anesthetic 1:100,000
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210
Horizontal
Vertical
Direction Rotatory
Saccadic Intrusions
Oblique
No slow phase
Circular
Saccadic movements only
Opsoclonus/Flutter Pendular
Waveform
Children Equal velocity each direction
Neuroblastoma, encephalitis
Fast phase – direction named after this
Breast, lung, ovarian cancer
Adults 8.17 Nystagmus Slow phase – actual pathological component
Assessment – Jerk
MS, toxins, medications DWARFF Null point = gaze location where
minimal nystagmus
Fundus
Optic nerve hypoplasia
Located in gaze opposite fast phase
component (Alexander's Law)
Large
Esotropia in congenital motor Ocular Motility Amplitude
nystagmus (CMN) Small
Effect of gaze position

Asymmetry
Pupils
Primary position
Iris abnormalities Rest
Gaze evoked
Good => CMN

Visual Acuity
Bad => retinal/optic nerve Fast
Frequency
abnormalities
Slow
Family History
Especially CMN
Early
Age 2 onset
Initially may be pendular
mo Extremes of horizontal gaze
Occasional vertical/rotatory Conjugate, horizontal jerk Congenital motor
nystagmus aka infantile
Fast phase in direction of gaze
Better with convergence,
Image if vertical/rotatory though
worse with fixation Rarely lasts 3 beats
Physiologic Horizontal jerk
Direction of gaze where Null zone Low amplitude
decreased nystagmus
Motor Symmetric in eye & gaze
VA good, strabismus common One eye Nystagmus
covered
No other neurology
Fast phase to fixing eye
Beats away from covered eye
Check with both eyes open also Visual Latent

acuity nystagmus
Otherwise latent nystagmus
may give false low vision Parkinson's
DVD Cerebellar degenerations

Associated Saccadic Intrusions
Asymmetry of nasotemporal pursuit PSP
Afferent visual pathway abnormality Multiple sclerosis

8.18 Nystagmus
Pendular most common Features – Congenital
In adults, pendular = Roving if VA <6/60

brainstem
infarction or MS Amaurosis/Achromatopsia/Albinism/ Periodic Alternating
Aniridia/Atrophy (optic)/Amblyopia Six As Albinism
Nystagmus
Sensory Defect
Nystagmus Acquired => brainstem lesion
Media opacity
Prisms
Primary
Leber's congenital amaurosis sensory retinal e.g. if R head turn (left side gaze)
Norrie disease Retinal dystrophy Vitreoretinal Kestenbaum– Treat as L XT for left eye – L LR
abnormality Anderson move recess, MR resect
Familial exudative Treatment horizontal recti
vitreoretinopathy (FEVR) Associated with albinism For All And R ET for right eye – R MR
recess, LR resect
Associated with aniridia Foveal hypoplasia Surgery
Hypoplasia
Bilateral recession/resection procedures
Coloboma Isolated Optic nerve
disorder
Atrophy Oral medications, botulinum toxin
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212
Seesaw Dysconjugate
nystagmus
Convergence retraction (Parinaud's) Torticollis
TONOPEN Nodding
Latent nystagmus
Lists Spasmus Nutans Pendular nystagmus

Spasmus nutans
Unilateral
nystagmus Cannot rule out intracranial
SO myokymia
tumors => MRI
Internuclear ophthalmoplegia (INO)
Glioma of anterior visual pathway Seesaw = Ventral

Midbrain
Craniopharyngioma
Nausea
8.19 Nystagmus –
Vertigo Associated with Acquired – Part of Parinaud's dorsal
midbrain syndrome
MRI All
Sense of rotation Pineal region tumor
Vestibular
U – Upgaze palsy
Inner ear disease
Convergence Retraction
C – Convergence retraction nystagmus
Causes Nystagmus = Dorsal Midbrain
CN 7 Disease – including nucleus in brainstem
UCLA L – Lid retraction

+ Interstitial keratitis => Cogan syndrome
A – Abnormal pupils – dilated, light–

Arnold–Chiari malformation near dissociation
Multiple sclerosis
Downbeat
Nystagmus Gaze-Evoked Alcohol intoxication
Infarction Cervicomedullar Jerk Nystagmus
junction lesion
Wernicke's Cerebellar/brainstem disease
Lithium toxicity/Vitamin deficiency

Periodic Alternating
Nystagmus
Brainstem (pontine tegmentum) or Present in primary position Upbeat Cerebellar lesions
cerebellar vermis lesion Nystagmus
Present in upgaze only

Drug effect
Empties sac => decreased
bacterial growth
Digital
massage
Hydrostatic pressure =>
occasionally opens duct
Spontaneously 50–90% first 6/12

Non-surgical resolve
5% full-term newborns 70% by age 1 y
Obstruction
below lacrimal sac
Thin mucosal membrane
at lower end of NLD Down
Higher rate canalicular stenosis
syndrome
Epiphora Not spontaneously resolve
Symptoms
Mucopurulent discharge Examination Management
Fluorescein into eye
Evaluate in 1/52
Retention 5–10 min Examination
Probing Successful in 90% age up to 15 mo

No dye in nose/pharynx 8.20 Lacrimal
Drainage Obstruction Fails in 1/3 >24 mo age
Below & nasal to medial canthus

When firm resistance at distal
Infracture of inferior turbinate
Anatomy end of bony canal
Above medial c.f. dermoid cysts/encephaloceles
canthal ligament
Surgical Silicon intubation after >1 probing failed
Digital massage
Congenital Intubation
Decompress Upper & lower puncta intubated
Dacryocele
Systemic antibiotics for infection
Management Left in situ 3–6 mo
Alleviate obstruction at Probe past common canaliculus

valve of Rosenmuller Balloon dacryoplasty
e.g. Lacricath balloon
Dacryocystorhinostomy
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214
Astigmatic errors BPES Differential Ectropion –

Amblyopia consider LTS
diagnoses Entropion/
Marcus–Gunn
Absent upper Ectropion
jaw-winking Entropion – exclude
Poor levator lid crease
function epiblepharon, rare Horizontal fold of skin
Acquired
Examination Congenital anomaly
Poor relaxation Ptotic lid higher in downgaze Upper/Lower eyelid
of levator
Resolve spontaneously Lashes turned into cornea
Pupil axis & amblyopia => treat <3 y
Epiblepharon
Glasses – astigmatism Everting sutures
Autologouslata fascia Wait till 6 yo (school) or older Treatment

Surgery Hotz procedure
Frontalis suspension Leaves lower lid scar
<5 mm Simple
Silicon sling Levator function Congenital
Ptosis Woo et al. (2000) – Asian eye method
5 mm–10 mm
Levator resection
>10 mm Lagophthalmos, lid Prominent, crescent shape skin
Fasanella–Servat tarso-conjunctiva lag on downgaze
mullerectomy Between eyelids & inner canthus
Supraciliaris
Exposure keratopathy Complication
Epicanthus
Palpebralis
Cosmesis Types Caucasian
Asymmetry, contour, scarring & lid malposition Tarsalis
East-Asian
Dysinnervation 8.21 Congenital
Trigemino-oculomotor synkinesis Inversus
Eyelid Common in blepharophimosis
Ptosis Features Anomalies
Blepharophimosis epicanthus inversus syndrome
Medial pterygoid – closes jaw Elevation of ptotic eyelid
with jaw movement
Lateral pterygoid – opens jaw 50% sporadic, or AD disorder
FOXL2 gene
Blepharophimosis
Horizontal/vertical palpebral fissure shortening
Ptosis Features
Visual axis, amblyopia Principles Epicanthus inversus
Jaw-winking Marcus–Gunn BPES Increase distance between inner canthi
Jaw-Winking
Ptosis Telecanthus
Wait till 6 yo (school)
Normal interpupillary distance
<5 mm – frontalis suspension Ptosis surgery
Levator function Surgery Ptosis
No eyelid fold, poor levator function
>5 mm – levator resection
Conservative
Disinsert levator muscle Management
Jaw-winking
Then do frontalis sling Treat epicanthus before ptosis
Duane's syndrome
Associations Ptosis Brow suspension
Premature ovarian failure
Toxoplasma gondii –
obligate intracellular parasite Transmission – infected birth canal
Cats definitive host 1/3: CNS, ocular, skin & oral cavity only
ELISA – high false positive, General General 2/3: disseminated disease

clinical diagnosis (including liver, adrenals, lungs)
Toxoplasmosis Herpes Viruses
Hydrocephalus
Non-ocular
Intracranficational Non- Surface disease –
Microcephaly,
calci ocular conjunctivitis, keratitis

intracranial calcification
Transplacental Retinochoroiditis –
Hepatosplenomegaly
transmission can be severe Tests – vesicular fluid, ocular
Retinitis +/–
8.22 Congenital swab – culture/PCR
Ocular
choroiditis, uveitis Cardiac
Infections – Cataracts
TORCHES IV acyclovir regardless
Thick cream colored, Otologic of ocular findings
overlying vitritis General Management
Ocular
Sensorineural
Pediatrician – if suspect
Reactivation near deafness Treponema pallidum
CNS/disseminated
previous scars
Mental Non-ocular Fetal infection common in
(COMB) primary/secondary – 50% offspring
If threatening vision Encephalitis Rubella
Management
Antimicrobials – Anemia Unexplained premature birth
Blood
Clindamycin/ Consider diagnosis
Nuclear Rhinitis, intractable rash, jaundice,
Trimethoprim + Thrombocytopenia
cataract, hepatosplenomegaly
sulfamethoxazole
glaucoma General
Hepatitis
Oral steroids while Age <2 y Mucocutaneous rash, periostitis,
Congenital infections
on antibiotics Microphthalmos Ocular osteochondritis
in 1% of infants
Non-ocular
Retinitis Syphilis Saddle nose, frontal bossing,
Transplacental OR through mother saber shins, hard palate perforation
Age >2 y
secretions to newborn Salt 'n' pepper
retinopathy Hutchinson's teeth
VDRL RPR, FTA-ABS
PCR on body secretions Scoliosis
General Age <2 y
Intrauterine growth retardation Salt 'n' pepper retinopathy
Ocular Chorioretinitis + retinal vascultis
Microcephaly, hydrocephalus, Non-ocular
Interstitial keratitis Pseudoretinitis pigmentosa
intracranial calcification
Age >2 y
Hepatosplenomegaly, thrombocytopenia Optic atrophy 10% of patients
Cytomegalovirus
Bilateral focal Retinochoroiditis Penicillin G
Direct action of infecting
Management agent damages tissue
Diffuse retinal necrosis Optic nerve anomalies, Repeat serology
microphthalmos until nonreactive
Ocular Teratogenic effect
Mechanism
Ganciclovir/Valganciclovir Uveitis, cataract
Delayed reactivation
/Foscarnet
Management of agent after birth
Antivirals
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Most serious
Direct contact during passage
through birth canal
Onset 3/7 to 3/52
Pathogens can ascend through
cervix to uterus Chemosis
Etiology Copious discharge Sepsis

Ascertain membrane
rupture or not Features
+/– Corneal ulceration, perforation
Meningitis
Urgent gram stain for Conjunctivitis in
Gram –ve diplococci first month of life Systemic
Arthritis
If positive => Swab Chemical
<2 d Diagnosis Gram stain – Gram negative
IV ceftriaxone
Definition diplococcic & culture
Gonococcal Neisseria Hospitalize/Pediatricians
Culture (N. gono) & PCR 1–3 d gonorrhoeae
(C. trachomatis, HSV) Timing Life-threatening Musculoskeletal &
Other bacteria neurological assessment
2–5 d of onset
HSV
1–14 d Ceftriaxone IV CSF, blood specimens
8.23 Ophthalmia
Chlamydia Neonatorum Normal saline irrigation
4–28 d
conjunctiva & fornices, QID
Erythromycin
ointment QID Treatment Treat Chlamydia –
Empirical Eye
Treatment azithromycin single dose Most common
Erythromycin
50 mg/kg/d elixir
for 2–3 wk Daily review – corneal
Onset days to 1/52
thinning, perforation
Less common Mild swelling, hyperemia
Parents
Test & treat
+/– Herpetic vesicles on eyelid margin
Moderate discharge
Features
Onset 2/52 Pseudomembranes
Retinochoroiditis Diagnosis
Dilate ELISA, PCR
Cataract
CNS signs Features
Herpes
Simplex Virus But treat because risk
Disseminated disease signs of pneumonia, otitis
Liver, adrenals, lung
Chlamydia Self limiting
trachomatis
Treatment Involve pediatricians
IV acyclovir in neonates
Regardless of ocular signs
Erythromycin 50 mg/kg/day 21 d
Treatment Medications
Azithromycin single dose, oral
20 mg/kg to 1 g max
Parents
Test & treat
Systemic – fever, headache, lethargy
If <1 yo – treat as orbital cellulitis
Proptosis
Post-traumatic
Features Painful/Restrictive ophthalmoplegia
Conjunctivitis
S. pneumoniae Causes Optic nerve dysfunction
S. aureus Secondary to URTI/sinus infection 5.2 nerve impairment

Preseptal Cavernous sinus thrombosis
H. influenza Bilateral involvement
Systemic illness
Orbit involvement
<1 yo – orbital septum not fully developed Sinusitis
Admit if
Treat as orbital cellulitis
Pediatrician – risk of whooping
cough in unimmunized patient CT Subperiosteal abscess
Investigation
Cavernous sinus thrombosis
Extension of preseptal - especially <1 yo

Microbiology/Culture/Sensitivities
8.24 Orbital Cellulitis
Rhabdomyosarcoma can present like cellulitis Admit
Sinus – ethmoidal Pediatrics – blood cultures, CXR, CSF

Causes
Local spread – dacrocystitis
Antibiotics – IV cefotaxime & flucloxacillin
Treatment
Hematogenous
ENT drainage subperiosteal abscess
Post-traumatic Other
Surgery
Orbit decompression – if not improved in 48 h
Postsurgical
Orbital apex syndrome

Neonates – S. aureous, Gram –ve rods
Complications Cavernous sinus thrombosis Subdural/Brain abscess
<9 y – single aerobic pathogen
Organisms Meningitis
Intracranial extension
>9 y – multiple pathogens
S. aureus, S. pyogenes, S. pneumo, anerobes
Periosteal abscess
H. influenzae if not vaccinated
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Anterior insertion
of Schwalbe's line
Posterior
embryotoxon
5% of population
Sclerocornea
Axenfeld anomaly
Forceps Posterior embryotoxon with
Tears in Descemets
attached iris strands
membraneUlcers Rieger
Glaucoma anomaly
HSV
Axenfeld + iris hypoplasia
Rubella Dysgenesis
Mucopolysaccharidoses
Metabolic Axenfeld–Rieger Axenfeld + Rieger +
Syndrome
Mucolipidoses Dental
Axenfeld–Rieger
No lens involvement, Type I syndrome
unilateral Craniofacial
Type II Skeletal deformities

Lens adherent to central Peter's anomaly
8.25 Anterior Segment
corneal defect, bilateral
50% develop glaucoma
Associated with Axenfeld–Rieger

& congenital rubella STUMPED Congenital
Corneal Opacities Megalocornea
Horizontal diameter >13 mm
PPMD Keratoglobus
Endothelial dystrophy Congenital Corneal
Abnormalities Deep Acs, DM breaks, hydrops
CHED
Keratoconus
Choristoma
fibrofatty tissue Microcornea
Subtopic
Dermoid
Covered by keratinized
epithelium
Associated
Goldenhar's
Nystagmus
75% patients
Birth
Absent irides
Glaucoma Late childhood or adolescence
Aniridia
Synechial angle closure Nephroblastoma
Gonioscopy
Peripheral anterior synechiae Poor prognosis
Features Diagnosed age <5 in 80%
Lids
Meibomian gland dysfunction

Wilms' tumor gene WT1 adjacent to PAX6
Cornea
Limbal stem cell deficiency Clinical FISH & chromosome analysis
Features Gene test
Loss umbo reflex
Foveal hypoplasia If positive => oncologist + repeated USS
Aberrant vessel through macula Wilms' Tumor
Abdominal USS every 3 mo until 5 yo
Nuclear sclerotic
Zonular weakness USS Every 6 mo until 10 yo

Cataracts
Esotropia Annually till 16 yo
Roving nystagmus
8.26 Iris Treat amblyopia/refractive errors
Abnormalities –
PAX6 gene on 11p13 Aniridia Cosmetic contact lens
Photophobia
Autosomal dominant
Morcher iris diaphragm
66% of cases
AN-1 Poor vision Magnifying lens
Complete penetrance, variable expressivity Visual aids
Ophthalmic CL, glasses
No systemic implications
Sporadic Limbal stem cell transplant
Genetics Management Usually needs trabeculectomy +

33% of cases Glaucoma
mitomycin C or tube
30% risk of Wilms' tumor before age 5
AN-2
Wilms' tumor Wilms' tumor 75%
Aniridia Genitourinary
WAGR syndrome Bicornate uterus, cryptorchidism
Genitourinary Systemic abnormalities
Retardation Streak gonad, hypospadias

Mental retardation
AN-3
Mental handicap Pediatrician
Gillespie syndrome
Cerebellar ataxia
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Unilateral
Usually no systemic problems
Optic nerve
hypoplasia
Midline abnormalities brain, thyroid
Bilateral
Septo-optic dysplasia
(de Morsier syndrome) Diabetes insipidus, growth retardation, seizures
Need MRI brain pituitary–hypothalamus
Idiopathic
Foveal
hypoplasia Congenital albinism
Macula Juvenile Aniridia

Normal looking eyes + disease retinoschisis
nystagmus
Infectious Toxoplasmosis
disease
CMV retinitis
Bilateral cataracts
Bilateral glaucoma 8.27 Blindness at Birth

ERG is diagnostic
Leber congenital (markedly abnormal or almost flat)
amaurosis
Bilateral retinoblastoma
Decreased rod & cone function
ROP Gross Ocular
Abnormality Photoreceptor CSNB (congenital Absent bipolar cell function
dystrophies stationary night blindness)
Aniridia + other anterior Decreased 'b wave'
segment dysgenesis VA may be close to normal
syndromes
Often paradoxical pupil responses
Shaken baby syndrome
Decreased cone function
ERG is markedly reduced

Severe ametropia Achromatopsia
Absence of response to flicker light
Cortical blindness
stimulus (25 Hz) is diagnostic
Normal looking eyes +
no nystagmus Ocular motor apraxia
VA usually normal by 6 mo
Delayed visual maturation
Looking past target
Features
Unilateral/bilateral, sporadic or autosomal dominant
Isolated or disorder related – midline structures Diminished Nystagmus, photophobia

nerve fibers
Alcohol, LSD, steroids, diuretics, cold Mother's gestation
White bowl-shaped excavation
remedies, anticonvulsants
Disc Inferior neuroretinal rim thin/absent
Roving eye movements, sluggish pupil response Retinal vasculature normal

Hypoplasia
Visual acuity variable

Squint, visual field defect Fluorescein angiogram
Hypofluorescence
Small gray disc
Surrounding yellow halo hypopigmentation Perimetry
Clinical
Superior defect
Chorioretinal atrophy Clinical
Foveal distance to disc Coloboma
– double-ring sign >3 disc diameters Serous retinal detachment
Vessels normal/tortuous Complications Progressive enlargement

aka Septo-optic
dysplasia 10% cases Peripapillary choroidal
Systemic neovascular membrane (CNVM)
Absent septum pellucidum, Morsier syndrome
corpus callosum agenesis 8.28 Optic Disc Microphthalmos
Ocular
0.3% of population Hyaline-like Abnormality association
Hypopituitarism calcific material Colobomas
Bilateral Iris, fundus
Drusen has Coloboma/Heart

spontaneous venous Mimic papilloedema defects/choanal Atresia
pulsation (80%) CHARGE
Systemic
No hyperemia, no association Goldenhar, Aicardi Retarded growth/Genital &
physiological cup Buried drusen Ear abnormalities
Chromosomal
Early branching Anomalous anomalies
Exposed
vascular pattern drusen Disc Drusen Patau syndrome (trisomy 13)
VA normal
If no complications
Retinitis pigmentosa Associations Clinical Clinical
Visual field defect
Angiod streaks Juxtapapillary CNVM
Pit Mimic glaucoma Observe 3 monthly
Alagille syndrome Disc neovascularization
Complications 1/4 resolve spontaneous
Initial schisis-
CRVO/CRAO like separation
Complications Temporal
VF loss Serous macular aspect of disc
Laser
detachment
FFA photocoagulation
No late leakage c.f. Autofluoresce Success
papilloedema 45% of eyes
rate 25%
US/CT Investigation Treatment
Calcific deposits Serous detachment Vitrectomy Good
success rate
OCT
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Unilateral – sporadic/bilateral – hereditary
Unilateral vascular loop

VA variable
Extending from disc into vitreous
Large disc, funnel-shaped excavation

Obstruction of retinal artery
supplying loop 10% Surrounding chorioretinal disturbance
Prepapillar loop
Signs White tuft glial tissue
Complications
Vitreous hemorrhage Persistent hyaloid remnants
Morning Glory
Anomaly
Clinical Radial pattern

Unilateral, avascular remnants of Bergmeister papilla Blood vessels
hyaloid system Increased number, hard to distinguish artery/vein
8.29 Optic Disc Serous retinal detachment

Usually bilateral, oblique entry of
nerve into globe Abnormalities – Complications 30% cases
Morning Glory &
CNVM
Others Adjacent to lesion
Visual field defect
Mid-facial anomalies
Inferonasal most common
Other Hypertelorism, notch in lip
RNFL elevated Clinical Tilted disc Frontonasal dysplasia Basal encephalocele
Associated with myopic astigmatic Absent corpus callosum, pituitary deficiency

refractive error
Systemic associations Neurofibromatosis type 2

CNVM
Complications
Serous macular detachment Posterior fossa brain malformations
PHACE syndrome
Megalopapilla, peripapillarys taphyloma, disc hypoplasia, Hemangiomas, cardiovascular anomalies
aplasia, pigmentation, papillorenal syndrome
Topical steroids
Arthritis >6/52 before age 16 S/E: glaucoma, cataract

20% develop uveitis
Short-term control
Pauciarticular, ANA+, female, age <2 y Higher risk Oral steroids
uveitis Long-term complications – long bone growth impairment
70% bilateral, 25% sight-threatening Juvenile
chronic arthritis
Uveitis asymptomatic & chronic Local steroids Orbital floor
Periocular
Posterior synechiae
Medical Sub-tenons
Band keratopathy
Findings
Cataract Anterior 1st line: weekly methotrexate
Systemic
Cyclosporine limited role with MTX
Glaucoma immunosuppresive
therapy
2nd line: azathioprine, S/E: bone marrow suppression
Treatment
Sarcoidosis mycophenolate mofetil
Older children, all segments of eye Infection
3rd line: cyclophosphamide Infertility
Tubulointerstitial nephritis &
uveitis syndrome 8.30 Pediatric Band keratopathy Secondary malignancy
Hypotony
Bilateral uveitis a/w renal disease
Uveitis
Aphakic glaucoma
Sarcoidosis
Complications Synechiae
Intermediate
Lyme disease Cystoid macular IOL contraindicated
Toxoplasmosis Surgical edema & retinal in active uveitis
Tuberculosis detachment
Most common cause
of posterior uveitis in children Cataract Contact lenses contraindicated
Oral steroids
with topic steroids
pre- and postop
Unilateral, treat with steroids
Aphakic glasses
Posterio pole granuloma Toxocariasis Correction
best choice
Features Posterior
Peripheral granuloma Glaucoma drainage device
with macular tract Glaucoma
VKH
Rare in children Trabeculectomy + mitomycin C
Endophthalmitis
Diffuse unilateral Every 3/12 for 1 y
subacute neuroretinitis
Due to nematode High risk Age <6 y, pauciarticular, ANA+ Every 6/12 for 5 y
Familial Others
Autosomal
Retinoblastoma dominant juvenile systemic Every 12/12 afterwards
granulomatosis
Lymphoma JIA Follow-Up
a/w arthritis & Polyarticular, ANA+ Every 6/12 for 5 y
vasculitic rash Masquerade Medium risk
Leukemia pauciarticular, ANA–
syndromes
Every 12/12 afterwards
Melanoma Low risk Onset age >11 y,
HLA-B27+
Every 12/12
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Vitreous syneresis Autosomal dominant hereditary Autosomal recessive, rods & cones
& myopia arthro–ophthalmopathy Oculodigital
Poor vision 6/60 to light perception reflex
Sensorineural deafness
Associated with Pierre-Robin Fundus – marbleized
Epiphyseal dysplasia, fundus to RP
arthropathy Collagen II connective tissue Leber's congenital Features
& hypermobile joints amaurosis (LCA) Sluggish pupil
Radial lattice- ERG – response
Diagnosis
Facial abnormalities – cleft like degeneration flat
palate, flat nasal bridge Hyperopic
Refsum – phytanic refraction
Differential acid storage
RPE hyperplasia, vascular diagnoses
sheathing, sclerosis Stickler
syndrome Bassen–Kornzweig –
Visual acuity ~6/60 abnormal lipids
Retinal detachment – Congenital
30% in first decade stationary night Diagnosis
Most common ERG Scotopic B wave reduced,
blindness
cause in children abnormal dark adaptation
Presenile cataract, Complications Retinal
Fundus Normal
ectopia lentis 10% AD, AR, X-linked
AD or X-linked recessive Abnormal Oguchi disease,
fundus albipunctatus
Similar to ROP – Familial exudative
but not premature vitreoretinopathy (FEVR) 8.31 Hereditary
Vitreoretinal Maternally inherited Mitochondrial mutation
Failure of vascularization, diabetes & deafness
temporal retina Dystrophies Maculopathy + renal failure
Age 5–10 with reading
Subsequent retinal Features difficulty + maculopathy Achromatopsia or
rod monochromatism Similar to LCA, AR
neovascularization
Vitreoretinal
Foveal – bilateral, Others Blue-cone Photophobia + nystagmus
Late childhood asymmetric, monochromatism
tiny cystoid spaces X-linked, less severe
Congenital than achromatopsia
Vitreous degeneration X-linked Isolated
Peripheral – retinoschisis Foveal hypoplasia
inferotemporal Types Aniridia
Peripheral vascular retina, ILM/NFL layers X-linked dominant, lethal in males
tortuosity & telengectasias
Associated with
Tests Aicardi Round widespread depigmented lesions
Fibrovascular proliferation Signs OCT, ERG syndrome
Colobomas Albinism
& traction => detachment Complications
Vitreous hemorrhage, Corpus callosuma genesis
Temporal retinal detachment X-linked –
dragging of disc Poor
male only
prognosis Juvenile macular degeneration, autosomal recessive
Stargardt
Blue light Norrie disease disease
Exudative retinal detachments Bilateral, symmetric, VA 6/60
sensitivity Enhanced S-cone
syndrome
Macular Age 8–15 y
Bilateral congenital blindness
Best vitelliform Autosomaldominant
Nyctalopia, pigmentary Hearing impairment Features dystrophy
retinopathy, macular schisis Favre–Goldman Autosomal
Age 4–10 y
Mental retardation syndrome dominant
Familial
Dark adapted ERG – drusen
no response Age >40 y
Circle centered
on disc
Radius twice distance

Proliferative vascular retinal Zone 1
from disc to fovea
condition in premature babies
Stage 1
Demarcation line
Zone 2 Zone 1 to nasal ora serrata,
Location near temporal ora
Scarring, retinal Potentially blinding Stage 2

detachment Ridge (height)
Zone 3 Remaining
<1000 g crescent, zone Stage 3 Extraretinal fibrovascular
53–89% proliferation
2 to temporal ora
<1250 g Extent
6% reached Epidemiology Tractional subtotal
43–66% Weight Number of retinal detachment
threshold Active stages clock hours
DRFTD Stage 4
<1500 g Classification
35–60% 4b = fovea off
DRFTD+
Vein dilation, vessel tortuous Stage 5
8–10% weighing <1500 g in posterior pole Total retinal detachment
develop Stage 3 % requiring
treatment Present in 2 quadrants
Plus
2% required treatment
disease +
Vitreous haze
Associated
with Iris vascular engorgement
8.32 Retinopathy of
Prematurity Poor pupillary reaction
Myopia
Macular dragging
20% of activeROP will regress without treatment Strabismus
<1500 g Amblyopia
Weight Stage 1
Myopia, pigmentary changes
<2000 g if unstable clinical course
Risk Factors
Gestational age Temporal vitreoretinal fibrosis,
< 30 wk Stage 2 dragged disc
Cicatricial
Amount of time on oxygen (Regressed) Stage 3
Stages Peripheral fibrosis & falciform retinal fold
Unwell child/Unstable clinical course
Stage 4
Partial retinal detachment
Stage 5 Total retinal detachment,

secondary glaucoma
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Prevention
Weekly, even Weight <1000 g Screen all patients PaO2 <80 mmHg
without ROP with risk factors
Gestational age Most common
31 wk OR Examination Cryotherapy method
protocol
4 wk after birth Less inflammatory
than cryo
Treatment Indirect laser
Follow-up photocoagulation Confluent ablation Stage 3 in Zone 1 or 2
to avascular retina
Threshold
Every 2/52 until vascularization complete Vitamin E 5 contiguous or 8
Controversial, disease
interrupted clock hours
antioxidant
Threshold (CRYO-ROP) OR Treat
Prethreshold (ETROP) Plus disease
Scleral buckle
/vitrectomy
Stage 4 or more
Zone 1 – any ROP 50% vs 25% treated –
Antivascular endothelial At 12 mo unfavorable
Zone 2 – stage 3 growth factor structural outcomes
Ongoing
1 wk or less studies
Active ROP
(ETROP observe arm) At 15 y 65% vs 45% treated
CRYO-ROP 1988
VA <6/60
Results
8.33 ROP – Early
Immature vascularization Screening treatment At 15 y 7.7% vs 4.5% treated retinal
Zone 1 detachment risk still present
Regressing ROP
& Treatment
Threshold TREAT
Zone 2 1–2 wks (CRYO-ROP) Zone 1 – Plus (any stage)
Stage 2
OR Stage 3
Zone 3 Pre-threshold
Any stage – 2 wk TREAT Zone 2 – Plus AND Stage 2
Regressed Screening
Stage 1 or 2 Mnemonic: 1 + 3 = 2 + 2
12 mo Studies
Regressed ROP Zone 1 – Stage 1 or
3 mo, 12 mo Stage 3 Follow-up category 2 ROP without plus
ETROP 2003
Observe
Annually until age 7 y Zone 2 – Stage 3
ROP without plus
3, 6, 12 mo Treated
eyes
Results 15.6% conventional vs
Annually until age 7 y (amblyopia cutoff)
At 9 mo 9.1% ETROP unfavorable
No ROP => repeat outcomes
Still no ROP => Increase oxygen in
exam in FOUR weeks pre-existing ROP
THREE months STOP-ROP 2003
ROP in Zone III => repeat No improvement
exam in TWO weeks Simplified Zone 1, Stage 3+ ROP in outcomes
4 - 3 - 2 - 1 Rule
Pre-threshold ROP (Zone 1 or 2) => BEAT-ROP 2011 Bevacizumab
repeat in ONE week vs laser Benefit in zone 1 but not in zone 2
Threshold ROP => TREAT!

Small study – safety unanswered
Most common pediatric
orbital malignancy
Age 1–4 y Features
Average age 5 yo
Features
Adrenal gland or
sympathetic ganglion Symptoms over
chain origin 1–2 mo
Horner's H Proptosis, globe

Well defined, no
syndrome displacement CT/MRI
bony destruction
Opsoclonus, ocular motility Signs Blepharoptosis,

disturbance, nystagmus O Signs – HOPE Biopsy Core, excisional, not
periorbital edema
for the best! fine needle aspirate
P Palpable mass Mimics orbital cellulitis

Proptosis
CT/MRI – Neuroblastoma Malignant Orbital -
Chest/Abdomen Pain Chest/Bone x-rays
Rhabdomyosarcoma
Eyelid E
Bone scan ecchymoses Investigations Bone marrow
Systemic aspirate trephine
Investigations spread
Urine 24-h
catecholamines Lumbar puncture
Similar presentation to
Orbit infiltration of malignant rhabdomyosarcoma, Excision LFTs
myeloid cells, bone predilection faster onset 1–2 wk
Adjuvent Radiation
aka Leukemic deposit/ Treatment therapy
myeloid sarcoma
8.34 Pediatric Tumors -
Dangerous Chemotherapy
Localized form of CML
Green colored – 5y Exenteration
myeloperoxidase survival
Metastatic 74–95%
Chloroma
Needs bone marrow biopsy
+ immunohistochemistry Dermoid or epidermoid cyst
Well defined, molds to bone (dumbbell shaped)
90% onset of leukemia within Investigation
Capillary hemangioma
12 mo, nearly all 1–2 mo Irregular, contrast enhancing
Prognosis Irregular, well defined, molds to
Poor ~50% Rhabdomyosarcoma
bone, no bone destruction
Metastatic neuroblastoma
Ewing sarcoma Poorly defined, bony destruction
Lymphangioma
Leukemia Orbital Lesion Non-encapsulated mass, irregular, +/– cysts
Features
Others Optic nerve glioma
Fusiform enlargement optic nerve
Lymphoma
Langerhans cell histiocystosis
Histiocytosis X Acute onset, bony destruction
Chloroma
Other
Neurofibroma
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Hamartoma, capillary
Plexiform endothelial cell proliferation
Primitive dermal elements
neurofibroma trapped in fetal bony suture lines
Active growth age <2 y Painless, mobile,
Lymphatic smooth
Features
malformation Differential Investigation – MRI Capillary
diagnoses hemangiomas Dermoid Non-tender mass
Diffierential diagnosis –
Rhabdomyosarcoma Ectopic Tissue cysts
encephaloceles, especially superonasal
(Choristomas)
Astigmatism Superonasal or
Indications Posterior extent superotemporal
Visual axis obscured
Propanolol CT Scan Dumbbell shape,
lucent capsule
Lancet
Steroids Homogenous fat
intensity
Superficial Laser Treatment
hemangiomas
Molding around
Surgery bone
Intravenous Treatment Surgical excision
Cavernous a/w Sturge–Weber vincristine
hemangiomas syndrome Avoid cyst rupture –
Solid choristoma granulomatous reaction
of orbit Port-wine stains White dome straddling
8.35 Pediatric limbus
Glaucoma Features
Tumors –
Not tumor, benign Benign Inferotemporal in 3/4
Chocolate Limbal Preauricular skin tags cases, a/w hair follicles
vascular malformation Malformations
cysts dermoids Associated Facial hypoplasia
Spontaneous with
MRI – cystic Lymphangiomas Goldenhar
loculated orbital hemorrhage Duane's syndrome
Benign Iris & upper lid
Chemotherapy – Associated with
neurofibromatosis 1 Eyelid & colobomas
carboplatin, vincristine Vision, refraction, motility
Epibulbar Treatment
Monitor optic Optic nerve glioma Gonioscopy – check
Radiotherapy
nerve function for deep extension
Similar to dermoid
Surgery
Treatment Neural plus fatty tissue
origin Surgery – astigmatism,
Debulk if chasm
Optic corneal haziness
involved Rare, more progressive Dermolipomas Temporal fornix
nerve sheath
in children meningioma +/– Graft in
Rarely require excision case deep Spontaneous
Destructive Juvenile
inflammatory lesions Tram tracking hyphema
xanthogranuloma
CT Intraocular
CT Nerve runs through Yellow papules
Bony erosion Associated with familial
tumor center Isolated or multifocal on iris
adenomatous polyposis (FAP)
Congenital
Esoniophilic granuloma Langerhans cell histiocytosis hypertrophy Gardner syndrome Spontaneously
>3 CHRPE, >1 quadrant => regress
of RPE
Local Turcot syndrome consider FAP
Curettage
Treatment Osteomas skull/
FAP + CNS tumors –
Systemic mandible/long bones
Chemotherapy especially glioma
Cutaneous soft
tissue tumors
Masquerade uveitis
Hyphema
Anterior segment
Most common malignant orbital tumor Iris neovascularization + glaucoma

Lens not involved – c.f. PHPV

Leucocoria 60%
White pupil reflex
Strabismus 25% Examination

Always check fundus in strabismus Presentation
Exophytic
Orbital inflammation Into vitreous, cottage cheese
Tumor growth
Endophytic
Yellow in subretinal space
Sellar/parasellar, pienoblastoma, Trilateral Rb Fundus
ectopic intracranial Rb Size
8.36 Retinoblastoma Rb extension
– Clinical Disc swelling
Parasellar tumor
Incidence
1/14,000 to 1/20,000 Epidemiology
Ultrasound/CT
Familial Calcification
Generally <1 yo
Age Office
Sporadic Full blood examination
Between 1–3 yo
Investigations Bilateral full indented exam

Family
History Repeat ultrasound
Visual behavior Under GA
Extraocular spread
Comorbidities
MRI Sellar/parasellar space tumor
Secondary tumors
Bone marrow biopsy

Metastasis
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A: Small <3 mm
Local treatment of course

Cryotherapy/Laser
>3 mm
Criteria Within 3 mm of fovea/1.5 mm of disc
With subretinal fluid in <3 mm

B: Big
B – Brachytherapy
Treatment
C – Chemotherapy
B – Brachytherapy
C: Contained Seeding Within 3 mm
C – Chemotherapy
Treatment
Unilateral – Enucleate
Older staging system for preservation of eyeball
8.37 Retinoblastoma
4 DD Group I International
Solitary or multiple
Classification (ICRB)
Group II
4–10 DD C – Chemotherapy
Solitary or multiple
>10 DD >3 mm D – between C and E
D: Diffuse Seeding
Group III Reese–Ellsworth
Staging System E – Enucleation
Anterior to equator Treatment
>10 DD Unilateral – Enucleate

Group IV
Anterior to ora serrata
>50% globe
Massive seeding >1/2 retina
Group V
Vitreous seeding Criteria Opaque media from hemorrhage
E: Extensive Postlaminar optic nerve head

involvement
Choroid, AC, sclera, orbit involvement

Treatment
E – Enucleation
Enucleate + Tissue histology/
Staging/Genetics
Life Extensive – ICRB Group E
Intraretinal melphalan
Limb – organ Aims
Function – eyesight Unilateral

ICRB Group C, D
Counseling & risk assessment Enucleation Avoids chemotherapy,

allows tissue genetics
Mild
Enucleation Rare to present
Enucleate all non-seeing eyes Algorithm as mild
where differential is Rb
Sequential aggressive
Options
Chemotherapy + local therapy
Systemic chemotherapy
SALT (sequential
aggressive local therapy) Chemotherapy
4–6 cycles CEV = carboplatin, Cryotherapy
etoposide, vincristine
Radiation (rarely)
+ SALT Laser photocoagulation
Intraretinal – melphalan
8.38 Retinoblastoma
Plaque brachytherapy
Patient/siblings screen – Treatment
every 4/12 until age 4 y Bilateral
Extension beam
Sub-tenon radiotherapy
Hereditary topotecan For ICRB Group A, B, C,
Every 6 mo until age 6 y
Monitoring not for D or E
Annually >8 yo
Often enucleate worse eye >1 mm past lamina cribrosa =>
Treated or for definitive genetics treat as disseminated
Monthly examinations under only eye
anesthesia initially Surgery
Bilateral if poor response Optic Chemotherapy
to treatment nerve >3 mm past
Spread choroid
Visual potential Chemotherapy
Only eye => Orbit

Factors – => CNS Poor
To Save an Eye Pinealoblastoma prognosis
E – adverse features –
ICRB Groups A, B, C, D Our third eye –
unsalvageable
Melanoma, similar tissue
Distant
sarcoma metastasis
Sustainable follow-up Rb mutation
Secondary Chemotherapy
Other solid
Tumors tumors
Breast/Bowel/Cervix
Secondary to
radiation
Osteogenic
sarcoma
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232
Tumor tissue Investigate proband child RB1 gene –13q14

Identify sporadic or germline Tumor suppressor gene
Blood
Gene
Compare parents, blood
Seek proband's blood mutation Autosomal dominant
behavior At inheritance level
Determine risk (i.e. pedigree)
Based on germline or sporadic
Pathway Recessive at
cellular level Lose both copies for
Assess blood for known mutations
cancer to occur
If no tissue diagnosis
Or use general risk figures 5 mutations occur in gene
in retina development
Knudson 2
Future pregnancies hit hypothesis So if germ line mutation
Issues present
Implication for child/siblings Earlier, bilateral
Offspring disease
45% Availability of testing Empirical 8.39 Retinoblastoma
Bilateral risk table
– Genetics Parent with
Sibling mutation
5% Germ line Parental
Has Rb mutation mosaicism
45% affected because Familial New germ line
Offspring (germline)
AD inheritance, 90% (bilateral) 40% tumor
7. 5%
Unilateral penetrance Risks New mutation
Sibling 100% germinal in embryo
1%
7–15% children affected Has
unilateral Lose both copies in one cell
If child affected => germ line
Parent
mutation => 45% subsequent children Sporadic
(unilateral) 60% Not passed on to offspring
Risk = population risk <1% No Rb, not a carrier
Inheritance
Get tissue specimen,
First child has Rb => low risk, Genetic Germinal 15%, somatic 85% identify gene mutation
but higher than population Counseling
Mosaicism, Positive Sample child's peripheral

polygenetics blood for same mutation
Family history
Preimplantation check Negative

Examine for aborted Rb
Chorionic villus sampling 10–12/52
Peripheral blood
Pregnant & Investigations gene testing If negative, may have
Amniocentesis 13–14/52 hereditary RB new unknown mutation
Baby – peripheral blood

Proband child Compare to peripheral blood
Mixed blood – mother's No cord blood
mutation may be present Tissue
specimen Same defect => inherited
OCT to find early If positive – induce
at 36 wk
Can test siblings/parents
Laser/cryotherapy – preserve fovea
1:30,000, 2–15% go blind Primary congenital
At birth, worse prognosis
Bilateral 2/3 of patients Infantile
Primary
childhood
Disease onset 80% within 1st year 6–12 mo, can spontaneously resolve
Features
60% final VA 6/12, 40% worse Classification Age 2–16 y
Juvenile open angle
Majority sporadic: GLC3A, GLC3B
Investigate/treat like adult

Anomalies, systemic disease
Genetics
> Age 3–4 y => juvenile POAG => Secondary
GLC1A myocilin gene, treat like adult Uveitis, steroid responder,
NF1, aphakia 75% develop glaucoma
Aniridia
PAS, preadolescents
Ocular
IOP > 21 mmHg anomalies Axenfeld–Rieger Pediatrician
8.40 Pediatric Regular screening until
Optic disc cupping Glaucoma Peter's anomaly 50% develop glaucoma
Haab striae age 6 for Wilms tumor
Corneal findings 50% develop glaucoma

Edema + enlargement
>12 mm
Secondary to
Denition 2 or venous congestion
Sturge–Weber
Untreated => buphthalmos – more required
Primary Congenital syndrome
cornea diameter 16–17 mm
"Corneal Disease" Systemic Bimodal incidence
Progressive myopia Age 2 & 6 y
Axial length disease
NF1 – NF at angle
Secondary Surgery risk Intraoperative expulsive
Reproducible visual field defect Glaucoma Lowe syndrome
Older age group hemorrhage, choroidal effusion
Cataract, glaucoma, 15–50% develop

Iris hypoplasia
renal; XL recessive
Other signs
Gonioscopy – anterior iris insertion Aphakic 25% 5 y post-
cataract surgery
Early IOL may reduce

incidence
Lens disorders
Most common secondary,
Photophobia Posterior segment mechanism unknown
Mechanical abnormalities
Epiphora Presenting Observe
symptoms PEBBle PHPV, ROP,
Treatment
Blepharospasm Uveitis & FEVR Topical therapy
Other steroids
Buphthalmos >12 mm Medications – Surgery - trabeculectomy/
Trauma e.g. topirimate seton + antimetabolite
233
234
Preferred primary intervention
Angle surgery Goniotomy – success with one 56%, two 74%

Clear cornea – IOP drops topical
Trabeculotomy – if poor angle view

Refract – high IOP => long axial Primary
Surgical
length => myopia
Progressive myopia = ? high IOP Trabeculectomy + 5-FU or MMC
Amblyopia/strabismus
Tube surgery
If above fails
Cyclodestructive – controversial
8.41 Pediatric
Glaucoma
Congenital – Management
Treat medically while awaiting surgery Glaucomatous optic neuropathy
Prostaglandin analogs Myopia
Poor vision due to Haab's striae/corneal scarring

Beta-blockers
Airways disease & very young patients – bradycardia Cornea
Band keratopathy
Medical Prognosis
Topical carbonic anhydrase inhibitor + oral (5–10 mg/kg QID) Strabismic amblyopia
Brimonidine contraindicated – cardiovascular

collapse, cerebral depression No contact sports
Lifelong monitoring
Alpha-agonist
Treatment compliance & compliance
Apraclonidine – tachyphylaxis, allergy risk
TORCHES
Toxoplasmosis, rubella, CMV, herpes, syphilis
Chromosomal
Down's
Marfan syndrome
Deficiency of enzyme
Galactosemia Type 1 – oil drop cataract + failure to thrive
Type 2 – lamellar cataract + healthy
Incidence Systemic Hyperferritinemia

1:5,000 disease
Metabolic X-linked recessive
16% sporadic disorder – Down's, Lowe
rubella, persistent hyperplastic Congenital glaucoma + mental retardation
primary vitreous
80% sporadic X-linked or autosomal recessive,
84% no association Congenital Secondary basement membrane disease
Anterior subcapsular cataract

Autosomal dominant
Alport
20% familial Anterior lenticonus, PPMD, Fleck retinopathy
Associated with aniridia,
hyperferritinemia (dominant)
Deafness, renal failure
Phacomatoses
8.42 Pediatric
Cataracts Neurofibromatosis 1
Aniridia
Anterior segment Peters

Ocular congenital dysgenesis
Primary Axenfeld–Rieger
Persistent hyperplastic
primary vitreous
Idiopathic
Most common Trauma
Ocular
acquired Steroids
Radiation
235
236
B-scan mandatory if no view

Exclude PHPV
Familial
History of cataracts FBC
Polycythemia, hyperferritenemia
History
Maternal
Infections Renal function tests, serum calcium – hypoparathyroidism
Child Serology – TORCH infections –

Failure to thrive, visual behavior Bloods toxoplasmosis, syphilis (VDRL)
Often from history
Ix not necessary
RBC GPUT & galactokinase activity – galactosemia
Arterial blood gas – Lowe's
Microphthalmos & rubella Investigation

Maternal & familial
Reducing substance – galactosemia
Syndromes
Persistent hyperplastic primar Determine cause Amino acid – Lowe's
vitreous (PHPV) Ocular
Urine Sediments – Fabry's

8.43 Pediatric Cataracts
Anterior segment dysgenesis
– Assessment
Copper – Wilson's
Visual behavior, acuity, preferential looking Blood – Alport's
Red reflex in undilated pupil Determine Examination Skull x-ray Toxoplasmosis,

visual significance Calcifications hypoparathyroidism
Central, >3 mm
Unilateral Karyotyping – chromosomal disorders
Laterality
Bilateral Fibroblasts – low mannosidase level
Other
Conjunctival biopsy – birefringent
cell inclusions – Fabry's
Posterior
Retinoblastoma, ciliary body tumor, segment Multiple
retinal detachment procedures
Exclude other Audiological evaluation
pathology Treatment Electrophysiology
Consent Specific
TORCH (rubella) complications Determine sensory deprivation –
Glaucoma Screening unilateral especially
Metabolic (Lowe) Compliance &
follow-up
Availability of services
Frequency of visits
Infant Aphakia Treatment Study Group
If small unilateral Consider pupil dilation alone
cataract IATSG 2010 114 infants, congenital unilateral,
operated 1–6 mo
Better visual rehabilitation Early
Surgery
Aphakia versus primary IOL implantation
Risk aphakic glaucoma
No difference acuity
Bilateral
At 1 y 4x/y
Committed to surgery IOL group more reoperations
(60% vs 10% aphakic) Follow-up
Once older – lifelong
Refraction Prevent near

Age >6/52 Timing Aim -3D
<6/52 Increased risk of glaucoma amblyopia
Complete unilateral Patch 50% waking hours

Best before 4 mo Amblyopia
Urgent
With contact lens on
Unilateral of recent onset 8.44 Pediatric
e.g. trauma
Cataracts – Management Glaucoma Age <6/52 especially,
Complete bilateral cataracts 50% eventually
Semi-elective
Unilateral cataract, uncertain age Secondary intraocular lens
Elective Age >10 y

PostOp
Long duration unilateral cataract Lens Contact lens
Likely amblyopic
Oral steroids Nd:YAG laser capsulotomy May require
multiple
5 d preop & postop
Operation Strabismus, retinal detachment
Topical steroids If nystagmus, esotropia
Hourly postoperative preoperatively
Poor Parental support Long-term treatment
Suture wounds prognosis & follow-up Support
Dissolves, prevent 10-0 Vicryl Glaucoma group
repeat EUA postoperatively
Controversial before <2 y, Never <2 y Outcomes >6/12

Unilateral cataract 10% If no nystagmus,
ongoing research
visual acuity - Melbourne ET, or glaucoma
Often 2–5 y experience >6/60
Intraocular
Aim hypermetropic Lens Choice 60%
Always >5 y Bilateral cataracts <6/60

Aim emmetropic visual acuity 40%
>6/12
Contact lens Lose eye 90%
Can adjust power anytime
2%
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238
Epibulbar & limbal dermoids

How many sutures involved?
Duane syndrome Ocular
Examination Syndromic or not?
Upper eyelid coloboma
Decreased jaw & Goldenhar
cheek growth Disc swelling Nerve compression requires cranial
Hemifacial microsomia cavity expansion
Ear abnormalities Non-ocular
Unilateral or bilateral
Autosomal dominant, early fusion of Calvarial suture
General
Fibroblast growth factor 2 (FGFR2) gene
Craniosynostosis
Hypertelorism & proptosis
Autosomal dominant, variable penetrance

Ocular Globe luxation, shallow orbits
Mandibulofacial Crouzon
Zygoma & malar eminence dysostosis Refractive error
underdeveloped
Treacher– 8.45 Syndromes
Collins Strabismus – abnormal muscle insertion
Colobomas – outer 1/3 lower lid
Ocular
Meibomian gland absence
No hand/foot abnormalities
Hypoplastic mandible – micrognathia
Non-ocular Non-ocular
Branchial Arch Broad retruded forehead
Macrostomia
Nonsynostotic
Midface retrusion
AD, FGFR2 gene
Apert
Look like Crouzon + syndactyly
Micrognathia, cleft palate
Microphthalmos Coronal synostosis, high prominent forehead

1:100,000 births
Pierre–Robin Pfeiffer
Congenital cataract & myopia sequence Maxillary hypoplasia
Ocular 48% optic nerve Eyes bulging appearance – small
Stickler syndrome association hypoplasia underlying cheek bones
Fetal Alcohol
Retinal detachment Asymmetric ptosis,
telecanthus, strabismus
Cranial
1:700 births
Flat occiput, low-set ears Most common
chromosome abnormality Presence of a third copy of
Facial chromosome 21
Flattened features
Genetics 47, XX (or XY), +21
Atrioventricular & ventricular Cardiac

septal defect
Non-disjunction
Intellectual deficiency, Mental translocation 14/22
presenile dementia Trisomy 21
Product of q22 band
of chromosome 21
95% meiotic
8.46 Trisomy 21 –
non-disjunction
Down Syndrome Up-slanting, mongoloid palpebral fissures
Mutation
5% translocation –
90%
long arm of 21 to 14/22
Epicanthic folds
Lids
Systemic Hypertelorism
Features Lacrimal
Neurohypotonia Nasolacrimal Blepharophimosis & ptosis
Ocular duct obstruction
Short stature Features Blepharitis
Keratoconus
Cornea Myopia >5D in 25%
Simean crease Refractive errors 85%
Other Astigmatism >3D in 25% Brushfield spots Junction inner &

Leukemia risk Iris outer half iris
increased 20–50x
Cataracts Increased number 80%
of vessels Light colored iris
Atlanto-occiput joint lax Stromal hypoplasia
Aberrant vessels
Hyperextension crossing disc
with intubation Optic disc
Optic atrophy Nystagmus

Extraocular
Horizontal
muscles
Strabismus
Esodeviations
239
240
Autosomal dominant
X-linked disease
Systemic Long limbs, arachnodactyly, high arch palate
Alports
Deafness
Aortic regurgitation
Systemic
Nephritis
Marfan Superotemporal ectopia lentis –
Syndrome intact zonules
Retina
Flecks
Myopia
Anterior Lens Ocular Common
lenticonus Autosomal recessive Retinal detachment/lattice
Short stature Keratoconus

Cornea Ocular
Systemic Weill–Marchesani
Bradydactyly
Posterior polymorphous "Reverse Marfan's" Microspherophakia
dystrophy Low IQ Uncommon
Autosomal Long limbs,
Cornea plana dominant
Subluxed lens => inferior Marfanoid arachnodactyly,
high arch palate
Microspherophakia 8.47 Connective
Ocular
Optic atrophy Tissue Diseases
Systemic
Anomalous angles Pierre–Robin
Micrognathia
Autosomal recessive Glaucoma
Midface hypoplasia
Brittle bones Sticklers

Mitral valve prolapse
Muscle wasting Deafness
Osteogenesis
Systemic Imperfecta
Joint laxity Myopia
Small stature Autosomal Ectopia lentis

recessive
Hyper-reflex joints,
Ocular Empty vitreous
Blue sclera hyper-elastic skin
Ocular Systemic
Keratoconus Giant retinal detachment
GI bleed Autosomal Aortic dissection
recessive Radial lattice
Loose "chicken" skin
Neck, axilla Systemic Ocular fragility
Beware sports
Mitral valve prolapse Ehlers–Danlos
Angiod streaks
Psuedoxanthoma Common
Angiod streaks
Elasticum
CNVM Myopia & keratoconus
Ocular
Peau d'orange
Retinal detachment
Blue sclera Ocular
Blue sclera
Optic disc drusen Uncommon
Ectopialentis
Enzymes missing => Lysosomal storage
disorders
GAGs build up in tissues
Face
Hurler
Coarse features, large head, short neck
General
Scheie
Types I to IX Short, joint stiffness
Hunter
Skeletal Claw hands
Systemic
Sanfilippo
Spinal deformity, hip dysplasia
ENT
Hypertelorism, proptosis, ptosis, squint, refractive error Otitis media
Ground glass GAG deposition, 8.48 Mucopolysaccharidoses Cardiovascular/GI/CNS

worse peripherally
Corneal clouding Urinary metabolic screen
GAG excretion
Rx => photochromatic glasses,
Investigations
Confirm by lysosomal
GAGs at angle enzyme assay
Open/Narrow angles
Glaucoma
Ocular Leucocytes/Plasma cells/
Thick corneas, hard to see discs =>
4 important ones Fibroblasts
management issue
GAG deposition
Frank pigmentary retinopathy Early treatment improves/delays disease

Rod/Cone degeneration Retinopathy
Systemic Treatment Bone marrow transplant
ERG Especially <2 y =>
Negative B wave decreases deposition
Primary – GAG deposition at disc

Optic neuropathy Enzyme replacement
Secondary to glaucoma/retinopathy Weekly IV infusion
241
C Cataract
Coats disease
A
Astrocytic hamartoma
Retinoblastoma
Leucocoria – CARPET R
Retinopathy of prematurity
P
Persistent hyperplastic primary vitreous
E
Endophthalmitis
T Toxocara retinitis
8.49 Leucocoria & Dragged Disc
Differential Diagnoses
Proliferative Retinopathy of prematurity
vitreoretinopathy
Congenital Familial exudative vitreoretinopathy
Incontinentia pigmenti
Infective
Dragged Disc Toxocara
Inflammatory
Pars planitis
Neoplastic
Combined hamartoma RPE & retina
Trauma
Globe rupture
Eyelids
Laceration
Cornea
Laceration, keratoconjunctivitis, foreign body, scar
AC
Shallow, hyphema
No pathognomonic signs
Does examination fit history? Principles Iris Iridodialysis/Iridocyclodialysis
Various stages of evolution Lens
Multiple injuries over time Cataract, phacodonesis, subluxed lens
Ophthalmic Increased red cell, phacoanaphylactic, iridodialysis

Document all details Features
IOP Pupil block, ghost cell, angle recession
Dilated exam
Ruptured globe
Document adnexal trauma Assessment
Vitreous hemorrhage
Retinal drawing, description, photography Preretinal
Second opinion Extensive multilayered retinal hemorrhages Nerve fiber layer
Pediatric assessment 8.50 Non-Accidental Deep retinal/subretinal
Injury Retina
Retinal dialysis, tear, detachment
Expert witness Choroidal rupture, CNVM
Describe observations Traumatic optic neuropathy, ON avulsion
Role in Court
Provide opinion as to cause Peripapillary scleral hemorrhage, optic nerve swelling
Court makes determination regarding causation/guilt
Non-accidental injury
Skeletal Subarachnoid hemorrhage
Terson's
Skin & soft tissue Non-Ophthalmic Features Hemorrhage centered around optic disc
Valsalva, Purtscher's
Neurological
Retinal Hemorrhages
Birth
Differential Diagnoses Trauma Within 1/12 of birth
Short fall
Anemia, seizure, cardiopulmonary resuscitation
Bleeding diathesis
Sepsis
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9 Retina and Vitreous
Retina &
Vitreous
9.1 Retinal Vascular Disease Features

9.24 Peripheral Retinal Neovascularization
9.2 Fluorescein Angiogram

9.25 Vitreous Hemorrhage
9.3 Central Serous 9.27 Retinitis Pigmentosa

Chorioretinopathy – General
9.4 General 9.28 Retinitis Pigmentosa

Age-Related Macular Photoreceptor Management
9.5 Fluorescein Angiogram Degeneration
9.26 Fundus Dystrophy 9.29 Others
9.6 Treatment
Macula 9.30 Retinal Pigment Epithelium Dystrophy
9.7 Differential Diagnoses 9.31 Choroidal Dystrophy
9.8 Myopia, Angioid Choroidal Neovascular 9.32 Ocular Albinism
Streaks, Ocular Membrane
Histoplasmosis 9.33 Electrophysiology
Syndrome
9.9 Idiopathic Polypoidal Choroidal Vasculopathy

9.35 Classification
9.10 Macular Hole 9.34 Retinal Tear
Peripheral Abnormalities 9.36 Management
9.11 Hypertension Retinal
9.12 Diabetic Retinopathy Detachment 9.37 Complications
9.13 Management Diabetes
Vascular 9.38 Retinal Surgery –
9.14 Studies Disease Tamponading Agents
9.16 Branch Retinal Vein Occlusion 9.39 Serous
9.15 Vein Occlusion
9.17 Central Retinal Vein Occlusion Detachment
9.18 Retinal Artery Occlusion

Artery Occlusion
9.19 Ocular Ischemic Syndrome
9.20 Macroaneurysms
9.21 Juxtafoveal Retinal Telangiectasia

Telangiectasias
9.22 Coats' disease
9.23 Retinopathy – Other
245
Retinal Hemorrhage Flame Vein occlusions

RBCs in NFL
Diabetes
Dot Ocular ischemic syndrome
RBCs within outer retina
Arterial embolism
Blot
RBCs in subretinal space Blood dyscrasias
Circular red area, central white spot

Roth's Spot
Hemorrhage in all retinal layers Subacute bacterial endocarditis
adjacent to occluded arteriole Leukemia
Other microembolism cause
Inflammatory/neoplastic cell foci
Microinfarcts Swollen NFL DM/HTN/HIV/SLE/

CWS CRVO/Septic embolism/
Focal NFL infarct affecting axon flow Blood dyscrasias
Exudates Edema early
Yellow deposits, well circumscribed

Ischemic damage to endothelial cells
DM/HTN/Radiation/Coats' disease
9.1 Retinal Vascular Lipoprotein leak in outer
Disease Features plexiform layer with infiltrating
macrophages
Microaneurysm Small red dots within retina

Loss of capillary endothelial cells or pericytes CRVO (peripheral)
DM (central)
Small bulge in capillary wall, fills with fibrosis
Arterioles, venules, capillaries,

fibroblasts proliferate
Retinal Neovascularization CRVO
Retina/vitreous scaflold
DM
Ischemic retina secretes VEGF
Fibrovascular DM/CRVO – traction, retinal detachment
Laser Fusion of retina to RPE layer

Detects macular ischemia
Detects neovascularization Function

Melanin
Microaneurysms
Hemoglobin
Retina and Vitreous
Baseline & medicolegal Pigment

Xanthophyll
Lipofuscin
Retinal Hard
Cystoid edema
Pooling (in a space) Transmission decrease Exudates
(blocked) Soft
Sensory retinal detachment
Subretinal Edema and transudate
RPE detachment
Best's disease
Non-cystoid edema
Hypofluorescence Other abnormal materials Foreign body
Perivascular staining Retinal Leak
Fundus flavimaculatus
Drusen Artery
Staining (in a tissue)
Scars Retinal
Subretinal Vein
Sclera 9.2 Fluorescein Capillary bed
Angiogram Filling defect
Lamina cribrosa Choroideremia
(delay & occlusion) Dystrophies
Atrophy Choroidal atrophy
Pigment epithelial window defect Loss of tissue
Transmission increase Subretinal
Drusen Myopia
Hyperfluorescence
Degeneration
Central areolar atrophy
Tortuosity & dilation
Non-perfusion
Neovascularization
Microaneurysms Aneurysms Retinal
Macroaneurysms Ability of substance to emit Blue light stimulation
Telangiectasias
yellow–green light
Shunts and collaterals In absence of fluorescein
Abnormal Auto fluorescence Black vessels & disc
Other
Neovascularization vessels
Subretinal Absorb light & emit back
Vessels in scar
Angioma Relies on RPE/Lipofuscin Macula has luteal pigment
Retinal
Retinoblastoma Masks autofluorescence
e.g. drusen
Tumors
Hemangioma
Red free
Melanoma Subretinal
Black vessels & visible disc
Metastases
247
248
Stop steroids
No treatment 90% resolve in 4 mo
Serous Detachment of Sensory Retina 50% experience recurrence
> 4 mo
Recurrent
M – M>F 10:1, 25–35 yo Criteria
Chronic signs – cystic or RPE
A – type A personality Risk Factors – M*A*S*H Occupational needs
Argon laser
S – Steroids At sites seen on FFA
H – Hypermetropia 100 mW 100 µm 20 ms, gray retina
Treatment Faster resolution, no change in final VA

Micropsia, metamorphopsia, scotoma, decreased color vision
Does not prevent recurrence (Watzke [1974])
Clinical
Well circumscribed, oval If close to fovea
Photodynamic therapy
Adjacent RPE atrophic areas Reasonable success rate – anecdotal
9.3 Central
Serous Decrease production
Smokestack
Chorioretinopathy Related to steroids Decrease binding
Hyper fluorescence spreads vertical then horizontal
Inkblot Oral medications – unproven Increase metabolism
Expansile dot pattern FFA
Spironolactone 25 mg po bd
Diffuse pattern
Gravity dependent Rifampcin – liver cytochrome P450
Gravitational patchy fluorescence Increase metabolism
Investigations Anti-VEGF
Dome shaped elevation of neurosensory retina
OCT Case-series show some benefit
+/– RPE detachment
24 h urine free cortisol

Home
As for Cushing's
1mg dexamethasone suppression test syndrome Endogenous
steroids
Hospital
Pituitary adenoma, adrenal gland tumors

RPE pigmentary changes
Small <63 µm
No increased risk of ARMD
Intermediate And no pigmentary change => early ARMD

Retina and Vitreous
Early – pre-ARMD - >63–<125 µm

Drusen With pigmentary change => intermediate ARMD
Dry 85%
Large >125 µm or width of retinal vein
Degenerative disorder of the macula higher risk of CNVM at disc margin –
Age >50 y, 20% in age >85 y Definition Intermediate ARMD
Geographic atrophy
Late
Characterized by typical "dry" and "wet" Drusenoid pigment epithelial detachment (PED)
macula features
Classic
Vision loss – central & paracentral Symptoms
Choroidal neovascular membrane Occult
Metamorphopsia (CNVM) Massive exudation (intra/subretinal)
Features Subretinal hemorrhage
Genetic
9.4 Age-Related Vitreous hemorrhage
Smoking Macular
Factors Serous
Hypertension & cardiovascular disease
Degeneration –
General Pigment epithelial detachment Fibrovascular Choroidal neovascularization (CNVM)
UV exposure
+ pigment epithelial detachment (PED)
Category 1 – no changes Drusenoid
0.5%
Risk RPE tear
Category 2 – small drusen, no GA or CNVM Wet (Exudative)
1.3% 15% - Late Disciform scar
Category 3 – extensive intermediate 5-year risk progression Retinal neovascularization; not choroidal
drusen & 1 large drusen, or to late AMD
20% 1 – intraretinal neovascularization
noncentral GA in one or both eyes
Stages 2 – subretinal neovascularization
Category 4 – advanced ARMD or 3 – choroidal neovascularization
45%
vision loss due to ARMD in one eye with retinal/choroidal anastamosis
Retinal angiomatous
proliferation Subsequent dilatation of vessels
"Angiomatous"
Parafoveal intraretinal hemorrhage
Clinical
100% develop in other eye by 3 y
Anti-VEGF
Treatment Longer treatment course than CNVM
249
Autofluorescence on red-free
Drusen
Staining
Hypo- or hyperfluorescent
PED Pooling
Window defects
RPE tear Hypofluorescence (masking)
Extrafoveal (>200 µm from FAZ)
Well defined CNV early in FFA Juxtafoveal (<200 µm from FAZ)
9.5 ARMD Classic
Fluorescein Subfoveal (encroaching into center of FAZ)
Angiogram Fibrovascular PED
Fills slower than classic CNV
Occult
Late leakage, undetermined source
CNVM
Leaking
Features of both Hemorrhage
Staining
Predominantly classic = >50% lesion classic CNV
Types Minimally classic = 1– 49%
Occult with no classic = 0% classic
Geographic & RPE Atrophy

Window defects
Safety Assessment of Intravitreal Lucentis for AMD
6. SAILOR 2008 2400 patients
0.3 mg vs 0.5 mg ranibizumab
3640 patients Vitamin A, C, E, beta-carotene, copper,
zinc supplements to prevent ARMD progression Rates of stroke/cardiovascular/ocular side effects same
Category 1 (no changes) &
No benefit Category 2 (small drusen, no GA
Retina and Vitreous
Comparison of ARMD Treatments Trial Monthly

[geographic atrophy] or CNVM)
500 mg vitamin C, 400 IU vitamin E, 15 mg beta- Cat 3 (med–large drusen or non- 1. Dry 0.5 mg ranibizumab vs 1.25 mg bevacizumab
Active CNVM signs only
carotene, 80 mg zinc, and 2 mg copper central GA in one eye) & AREDS 2001
Decrease progression by 25% Cat 4 (advanced AMD or vision loss VA – monthly bevacizumab = monthly ranibizumab
in AMD in one eye)
VA – as needed bevacizumab = as needed ranibizumab
Beta-carotene increases risk of 7. CATT 2012
lung Ca in smokers VA similar to MARINA, ANCHOR
Stop smoking Outcomes
VA not <15 letters from baseline ~95% for all
Predominantly classic CNV
716 patients 2. ANCHOR 2009 Central retinal thickness decrease Best in monthly ranibizumab
Maintain VA ranibizumab 95% vs PDT 60%
PDT vs monthly ranibizumab (0.3, 0.5 mg) Bevacizumab = ranibizumab
Improve VA ranibizumab 35–40% vs PDT 5% 9.6 ARMD Treatment
Ranibizumab improves VA in classic CNV Aflibercept (EYLEA) 3x monthly then 8/52 vs
Wet & Dry ranibizumab 0.5 mg monthly
Occult + minimally classic CNV
423 patients 13 vs 8 injections over 2 years
Maintain VA ranibizumab 95% vs sham 60% 8. VIEW 2012
Sham vs monthly ranibizumab (0.3, 0.5 mg) 3. MARINA 2006
Key difference Similar efficacy
Improve VA ranibizumab 25%–30% vs sham 5%
Ranibizumab improves VA in Macular Extrafoveal classic CNVM
occult + minimally classic CNV AMD, OHS, idiopathic CNVM
photocoagulation study
Extrafoveal
Subfoveal CNV 300–2500 µm, VA > 6/30, > 50 yo
184 patients 9. MPS 1982
4. PIER 2008 Severe visual loss > 6 lines
3 years
3x monthly then 3 monthly ranibizumab vs sham Results 62% untreated vs 47% treated
Good response 3/12, then plateaus
Ranibizumab monthly better than 3 monthly Scar extension
Myopic, juxtafoveal
We have anti-VEGF now
Any CNV, 40 patients
Targets
Decrease ranibizumab frequency (PRN) after 3x monthly vs sham Endothelial cells, monocytes, stem cells, neurons
Symptoms Promotes physiological angiogenesis

Loss 5 letters + OCT fluid (intra/subretinal) VEGF-A
VEGF
Antibody – ranibizumab, bevacizumab
New macular hemorrhage
Placental growth
5. PRONTO 2009 Promotes pathological angiogenesis Ischemia, inflammation,
FFA 5 criteria for PRN injection factor PLGF
New classic CNV wound healing, cancer
OCT alone Antibody – aflibercept
Gain 100 µm
Wait 3/12 after
Persistent fluid on OCT 1/12 after previous injection cerebrovascular event
Reduces injections Ranibizumab PRN based
Mean 5.6 injections/12 mo on criteria
Similar VA results as MARINA & ANCHOR

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Macular degeneration/Myopia/Macular Telengectasia/MEWDS/MFC

Scar – iatrogenic
Histoplasmosis
Any Fundus Scarring 4M SHATIN
Process Can Lead to CNVM Angioid streaks
Toxoplasmosis scar
Inflammation – uveitis
Non-CNVM – see below – CAMP RAP
Chronic central Younger, no subretinal hemorrhage, no drusen

serous retinopathy
Adult vitelliform foveomacular dystrophy

Subretinal Fluid +/– Hemorrhage –
Macroaneurysm retinal artery
& not CNVM CAMP RAP
9.7 Choroidal Polypoidal choroidal Multiple recurrent serosanguinous RPE detachment
Neovascular vasculopathy
Membrane (CNVM)
Retinal angiomatous proliferation
Differential Diagnoses
ARMD
Pathological myopia
Degenerative Optic disc drusen
Angiod streaks
Idiopathic polypoidal choroidal vasculopathy
Macular CNVM Presumed ocular histoplasmosis syndrome

Inflammatory
VKH
Posterior uveitis
Toxoplasmosis
Trauma Choroidal rupture
Laser photocoagulation
Tumor Choroidal nevus

Choroidal hemangioma
Retina and Vitreous
Fungus histoplasma capsulatum >-8.0D & axial length >32.5 mm

Definition
Inhaled => bloodstream Ocular histoplasmosis Secondary ocular changes
syndrome
Focal degeneration Tilted optic discs
Endemic in US Visual field defects
Disc
Punched out chorioretinal lesions – deep to retina Peripapillary atrophy
Central
Macular CNV Triad Staphylomas Tigroid tessellated fundus
Features
Features OHS
Peripapillary atrophy/scarring Focal chorioretinal atrophy
No vitritis Periphery
Lattice degeneration
Bilateral in 60%
Lacquer cracks
Photocoagulation to extrafoveal lesions CNVM
Pathologic Myopia 5–10% with axial length >26.5mm
Observe foveal lesions Treatment
Laser potentially more damaging Non CNVM related
Complications Subretinal "coin" hemorrhage
Linear gray/dark red lesions Fuch's spot
Rhegmatogenous retinal
Peau d'orange Signs detachment, macular hole Raised pigmented lesion
Mottled pigmentation 9.8 CNVM – Myopia/Angioid
Optic disc drusen Streaks/OHS Foveal retinoschisis
Hyperfluorescence FFA
RPE window defects
CNVM Extramacular
Laser/Anti-VEGF Argon laser, risk scar extension
Choroidal rupture Prognosis Treatment
No contact sports Angioid Streaks Bevacizumab
CNVM Beware child-bearing age females
Foveal involvement of streak
Most common
Retinal detachment
85% have streaks Pseudoexanthoma elasticum Surgery
Protection during sports
AD collagen disorder Ehlers–Danlos
High myopia >12D
Metabolic bone disease Associations Cataract/clear lens surgery
Paget disease 8% have retinal detachment in 7 y
2% have streaks
Sickle cell disease

Hemoglobulinopathies
Thalassemia
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Indocyanine
Choroidal aneurysms green angiogram Polypoidal & aneurysmal dilatation
Features Investigations
Terminal branching sub-RPE vessels
Pigmented patients Slow fill, intense leak
Increased incidence in Asian patients
9.9 Idiopathic Polypoidal Laser

Unilateral NOT subfoveal lesions
Choroidal Vasculopathy
Peripapillary
PDT + ranibizumab vs PDT
PDT = photodynamic therapy
Lack of drusen vs ranibizumab
Signs Treatment 80% vs 70% vs 30% regression
Exudation
Multiple, PEDs, serous detachments PDT with verteporfin
EVEREST 2011 Subfoveal, reduce size; recurrence 10%/y
Subretinal hemorrhage
Stabilize VA, reduce exudate
Not intraretinal
Anti-VEGF No polyp regression
Think IPCV if anti-VEGF for “ARMD” not responding

Retina and Vitreous
High myopia
True hole
Blunt ocular trauma
ERM with pseudo-hole

Watzke–Allen negative
Differential
Hole not full thickness, no Lamellar hole Diagnoses Slit lamp Watzke–Allen test
treatment required Pseudo-hole
Micro-hole Laser aiming beam
Investigations
White dot fovea OCT – to stage
FFA – hyperfluorescent spot

6th–8th decade of life
Decreased central vision

Epidemiology FTMH Stage 2 or more
Commonly secondary to PVD traction
VA <6/9
Indications
10% risk developing in other eye if no PVD Hole less than 1 y duration
Impending – loss of foveal depression with small yellow spot 1a 9.10 Macular Hole Higher baseline => higher end visual acuity
Stage 1
Occult – yellow ring due to break in outer foveal layer 1b
Vitrectomy + relieve traction
Full thickness defect <400 µm diameter Treatment ILM peel
Stage 2 Technique
Hole includes inner foveal layer Gas tamponade
Full thickness defect >400 µm 2 d face down posturing
Vitreous still attached at disc, not macula Stage 3 Staging

Closure 90–95%
VA <60
Success rate Repeat surgery after 2/52
Full thickness defect >400 µm
Myopes – 25% closure + macula buckling
PVD
Stage 4
Cataract
Surrounded by cuff of SRF Complications
Yellow deposits in center of crater Retinal detachment
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Arteriosclerosis
Focal/Generalized
Flame retinal hemorrhages
Vascular leakage CWS

Chronic edema & accumulation
Macular star
of hard exudates around fovea
Blood Pressure-Related Retinal Changes Fundus Features Optic disc swelling
Rarer
Grade 0 Elschnig spots
No changes Black spots, yellow halo – choroidal infarct
Choroidopathy Siegrist streaks Flecks linearly along choroidal
Grade 1
Arteriolar narrowing vessel – fibrinoid necrosis
Grade 1 – barely detectable 9.11 Hypertension
Exudative retinal detachment
Grade 2 – obvious narrowing Especially in eclampsia
Systolic >180 mmHg,
Classification diastolic >120 mmHg
Grade 3 – copper wiring, AV nipping Arteriolar narrowing, focal Grade 2
irregularities
Grade 4 – Grade 3 + silver wiring Hypertensive retinal changes
ECG, FBC, UEC, BUN, creatinine,
Grade 3 Malignant Hypertension Primary glucose, coagulation profile,
Grade 2 + retinal hemorrhages/exudates urinalysis, CXR
Grade 4
Secondary Cardiac enzymes, TSH, urinary
Grade 3 + disc swelling Investigations catecholamines, CT, TEE
Cushing's reflex – raised ICP => raised BP
Brain tumor
Pericyte loss, BM thickening
Vascular endothelial
Platelet adhesiveness increased damage
CWS, venous beading RBC aggregation increased

Block
Pathogenesis
Intraretinal hemorrhages
Bleed Pericyte death = 4 outcomes
Microaneurysm leading to Ischemia
Blowout Macular Ischemia Assess with FFA
Retina and Vitreous
Edema Abnormal serum lipids Thickening relative to fovea

Leak
Exudates
Does not extend beyond ILM Features
CSME
Macular ischemia – intraretinal capillary closure Features
Decreases VA Clinically significant macular edema
Macular edema – increased retinal vascular permeability Macular Edema
Retinal edema
MILD Within 500 µm of fovea
Microaneurysms only
CSME – EDTRS
Within 500 µm of fovea
At least 1 quadrant Hard exudates
+ thickened adjacent retina
Thickening
>1 disc area, within 1DD of fovea
Hemorrhages
+ MA's Ischemia induced neovascularization
<ETDRS standard
photograph 2a MODERATE
<1/3 DD NVD
9.12 Diabetic Non-high-risk
Retinopathy
NVD <1/3 disc area + vitreous hemorrhage
High-risk (DRS)
+ Beading or CWS1 quadrant only NVD >1/3 disc area +/– vitreous hemorrhage
Proliferative
4:2:1 Rule Moderate NVE (1/2 disc area) + vitreous hemorrhage
4 quadrants – diffuse intraretinal hemorrhages & MAs
Retrohyaloid/vitreous hemorrhage
2 quadrants Nonproliferative
Venous – ETDRS Tractional retinal detachment
beading Advanced
>ETDRS standard Tractional retinoschisis
photograph 6a
Rubeosis iridis
Iris, angle
SEVERE
Intraretinal microvascular abnormalities 1y
Absent/Mild 1%
1 quadrant 6–9 monthly
Moderate 4%
IRMA Follow-up + 4–6 monthly
Risk of High-Risk Severe 8%
>ETDRS standard
photograph 8a PDR in 1 Year 3–4 monthly
Very severe 17%
2 monthly
Non-high risk PDR 50%
PRP within 1/52
Progression 1 y High risk PDR
15% to high-risk PDR Coexisting CSME
Macular laser first
2 or more severe characteristics VERY SEVERE
Progression 1 y
45% to high-risk PDR
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Initial worsening
258
Decreased risk developing new DR Intensive glycemic control Glycemic control OCT
Decreased by 76% Intraretinal edema
HbA1c 7% – DCCT Pretreatment No laser if ischemic – as no VEGF
Decreased progression of existing DR FFA being released
Decreased by 54% Ischemic maculopathy
Hypertension
UKPDS – a/w risk of progression Systemic
Monitor each trimester Pregnancy Argon focal laser
Focal leakage/Microaneurysms
a/w worsening retinopathy
Field study Fenofibrates Diffuse leakage/Capillary non-

Independent of cholesterol level perfusion adjacent to macula
Other Argon grid laser
Anemia, renal disease, asymmetric carotid disease Treatment Thickening 500 µm away from fovea/disc
Decrease viable retina => decrease ischemia => VEGF Scatter panretinal 50–100 shots/50–100 µm/0.05 s –
photocoagulation – PRP ETDRS 0.1 s/50–200mW/1 burn width apart
Regresses neovascular tissue => prevents NV in future
Blanch, light gray burn
PRP reduces visual loss compared to no treatment Otherwise turn power up
Most benefit in high risk PDR
Treat CSME before cataract surgery/PRP
1200 or more shots/500 µm/0.1 s/200 mW/1/2 burn width apart Treatment – DRS
Laser – CSME Indications
Treat regardless of VA
2 sessions, inferior retina first ETDRS
Vitreous hemorrhage hides inferior retina Treatment reduces moderate visual loss by 50%
High-risk PDR – absolute
Risk lipid dump
Early PDR – likely Laser - DR Dense exudative
If good control, 2/12 follow-up Indications maculopathy Anti-VEGF/steroid first
Severe/Moderate NPDR – relative Systemic – lipids – fenofibrates
Lives far away/noncompliant Contraindications
9.13 Diabetic Enlarged FAZ
Decreased vision, night, color, peripheral
Retinopathy Central macular ischemia
Worsen macular edema Side effects
Apply focal laser first Management
Retinal hemorrhage areas Laser will burn retina
Choroidal effusions/CNVM/burns – cornea/lens
Treat MAs
Helps dry edema, prevent bleeding Paracentral scotoma
Anti-VEGF
Transient increase in edema/decrease VA
Only use if planning vitrectomy Side effects
Otherwise NV may induce tractional detachment
CNVM, subretinal fibrosis, laser scar expansion
Better equipment, endo-laser Inadvertent foveal burn
Iris new vessels Can wait – up to 3/12

Anti-VEGF – CSME Early results Suggest ranibizumab +/– laser better
Ischemic CRVO
Afferent pupillary defect Follow-up monthly to check Previous PRP than laser alone
Current practice
Retinal detachment
B-scan CSME not responding to laser
Reasons for early surgery Vitrectomy for Intravitreal Refractory CSME
Traction, decrease PVR, social Vitreous Triamcinolone Pseudophakics
Vitrectomy early – CSME
No previous PRP Hemorrhage
Within few weeks Anti-VEGF compliance/social issues
Iris neovascularization + hemorrhage + planning surgery within 1/52 Cataracts
Side effects
Including for diabetic delamination surgery Facilitate surgery – less bleeding
Glaucoma
Anti-VEGF
Retinal neovascularization shrinks => preoperatively
Fibrovascular contraction => Future Wide-field angiogram Peripheral ischemia demonstrated
Risk of "avastin crunch"
Tractional retinal detachment early => earlier treatment
So operate beforehand Develops over 1/52

Created CSME criteria Moderate visual loss = 15 letters
Wisconsin Epidemiologic Study of DR (doubling visual angle)
Duration of DM Focal/Grid laser Reduced risk of moderate VA loss by 50%

ETDRS
A/w increased prevalence of DR – Type 1 & 2 DM WESDR
Epidemiology Laser
99% Type 1 have DR Gain 15 letters VA gain 40% vs 25%
After 20 y
60% Type 2 have DR Focal first then PRP
PRP exacerbates macular edema
Retina and Vitreous
DRCR. net Macular laser > IVTA

Decreases new 25% & progression 50% of DR Laser alone 30% gain >10 letters over 2 y
HbA1c 7% DCCT (T1DM)
UKPDS (T2DM) IVTA complications – glaucoma, cataract
Initial worsening Macular laser > IVTA
Glycemic Refractory macular edema
control DRCR. net
HbA1c <6.5% Pseudophakics – less efficacious than
No effect on new or progression DR ADVANCE IVTA + prompt laser vs ranibizumab
ranibizumab (by 2 letters)
IVTA + deferred laser
ACCORD
Aggressive glycemic control => increased mortality Refractory CSME 5 y results
Gilles et al.
10% increase early DR 10 mmHg increase SBP (2009) 42% vs 32% gain 5 letters
WESDR IVTA 4mg vs placebo
15% increase PDR IVTA glaucoma 44%, cataract 54% within 5 y
c.f. ARMD, in DM
Reduced progression of DR 1/3 Tight BP control in Important for consent Once edema resolves, can stop anti-VEGF
CSME
T2DM 140/80
Decreased visual loss by 1/2 Prevention
UKPDS
Blood Ranibizumab gain 9 letters
Need for laser decreased by 1/3 pressure DRCR. net Ranibizumab + prompt/deferred
laser > laser alone
Decreases development in T1DM by 18–35% Candesartan No difference between prompt/deferred laser
DIRECT 9.14 Diabetic
Decreases progression in T2DM by 34%
Retinopathy RESOLVE Improves VA & edema
Ranibizumab > placebo
Enalapril Studies Rescue laser for both
Reduces DR in T1DM by 65%
RASS
Reduces DR in T1DM by 70% READ 1+2 Improves VA & edema
Losartan
Ranibizumab > laser
Anti-VEGF 2 y results
Fenofibrate in Lipid
Decreased need for PRP/macular laser by 31% FIELD
T2DM DR therapy Improves VA & edema
Bevacizumab > laser
Not associated with lipid profile BOLT
Median 9 injections over 12 mo
Panretinal photocoagulation Improves VA & edema
Bevacizumab > laser
PRP reduced severe visual loss by 50% over 5 years High-risk PDR Median 9 injections over 12 mo
DRS Proliferative DR
Severe visual loss = 5/200 Ranibizumab > ranibizumab + laser > laser alone
RESTORE
>15 letters 22%, 22% & 8% respectively
Non-high risk PDR
Lower risk progression ETDRS Laser crossover to ranibizumab
RESTORE 3 Does not catch up to ranibizumab alone
Vitrectomy 1–6 mo vs 12 mo
VA 6/12 36% vs 12%

T1DM DRVS Vitreous Refractory CSME – 40% >15 letter gain
Reason why T1DM = ? risk tractional retinal detachment Ranibizumab in refractory CSME postlaser
Vitrectomy Hemorrhage RIDE & RISE studies
Monthly x3 then prn
VA 6/12 25% vs 15%
T1DM & T2DM together
? Less benefit in T2DM due to macular ischemia
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Beçhet's
Sarcoidosis
Inflammatory disease associated Syphilis

with occlusive periphlebitis Wegener's
Hypercoagulability Goodpasture syndrome
Stasis Virchow's triad
Hemodynamic changes Polyarteritis nodosa
Turbulence
Endothelial/wall injury/dysfunction
Lupus anticoagulant
Acquired
Underperfusion Pathology Causes hyperviscosity Polycythemia
Venous stasis Arteriolar occlusive disease Multiple myeloma
Thrombus forms Waldenstrom macro

Hypercoagulable
Adjacent arterial wall => compression venous wall states
Branch retinal vein occlusion Factov V Leiden mutation
At AV crossing Inherited Protein C & S deficiencies
thrombophilia
9.15 Retinal Vein a-thrombin III deficient
Age >50 y
Occlusion Prothrombin mutation
Obesity, smoking
Homocysteinemia
Hypertension Ocular
Cardiovascular IOP
Lipids
Cardiovascular
Diabetes mellitus
BP, cholesterol, BSL, BMI
For central retinal vein occlusion only Risk Factors
Disc drusen
Ocular ACE, HLAB51, syphilis, RF, ANA, pcANCA, dsDNA
Raised IOP Vasculitis
ESR
Deep vein thrombosis, pulmonary embolism Inflammatory conditions
If age <50 y
Miscarriage history GCA does not cause BR/CRVO
Previous BRVO/CRVO INR, APTT, fasting homocysteine level

Thrombophilia screen
8–10% risk to other eye
Antithrombin III, protein C & protein S
FBC
Hyperviscosity screen Hb, platelets, plasma cells
EPG/iEPG
Physician referral
Hemorrhage
IOP treatment
Retinal edema Wait 3/12 from BRVO onset

Retina and Vitreous
CWS(NFL infarcts) If intact perifoveal perfusion (on FFA)

Signs Treatment CMO
PRP
Dilated, tortuous vein 100–200 µm/10 ms/100 mW – over edema
Avoid hemorrhages, maximal to edge of FAZ

Commonly at AV crossing
Neovascularization
CMO
PRP
Complications
Neovascularization
FFA to look for ischemia
VA >6/12
When macular edema & VA<6/12, intact foveal vascularity None => no treatment & follow-up
9.16 BRVO 3/12
Grid macular argon laser
Increase VA by 2 lines
BVOS 1984 VA <6/12
65% vs 37% untreated Follow-up FFA for macular ischemia/shunts
Sector PRP
Once have neovascularization
If ischemia (>5DD of non-
No difference in vision at 1 y perfusion) or poor VA
3 monthly
IVTA = laser After laser/anti-VEGF
IVTA Increased complications
SCORE report #6 2009
Cataracts, IOP
Studies
Laser remains main treatment VA > 6/12 at 1 year
50–60%
397 patients
No laser
50%
BRAVO 2009
Collateral vessels
6 mo – 16 letter gain c.f. 7 in sham Ranibizumab 0.3mg/0.5mg/ Prognosis
Take 2–8/12 to develop
55%/61% vs 30% sham sham injection
Retina
25%
BRVO with no pre-existing PVD
Neovascularization
Theory – less vitreous better retinal oxygenation Vitrectomy
Rubeosis iridis
No randomized controlled trials 1%
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Underlying medical condition + IOP (iatanoprost)
262
Macula similar to BRVO Hemiretinal

VO No grid laser
For CME
Rubeosis risk similar to CRVO Treatment IVTA 1mg Improve VA by 15 letters at 12/12
For CME
CRVO Anti-VEGF Ranibizumab 0.3 mg
DR/OIS/HTN Differential
PRP
Diagnoses Forrubeosis
Purtscher
Hyperviscosity Non-ischemic
Other 1/3 ischemic by 1 y
Radiation retinopathy No benefit from prophylactic PRP
CVOS 1995 Ischemic
=> Venous pressure not high If spontaneous 1/3 develop neovascularization
venous pulsation PRP them!
=> Not CRVO
Macular edema
No benefit from grid laser
RAPD – Absent/Mild
VA mild–moderately impaired 271 patients

1 mg IVTA improved VA by >15 letters at 12 mo
Dilation/Tortuosity of CRV 9.17 CRVO
Features 27% vs 7% untreated
SCORE report
Dot/Flame hemorrhages 4 quadrants Injections 2.2/y/time between injections 4/12
Non-ischemic #5 2009
Macular edema Studies – 1/4 IVTA patients had no response
Non-Ischemic Cataracts
Disc swelling CME Side effects
Raised IOP
Neovascularization rare 4 mg > 1 mg = observation
Prognosis
Chronic CME => poor VA Ischemic CRVO excluded
Signs 392 patients
FFA No laser or IVTA
>10 disc areas capillary nonperfusion
CRUISE 2009 6 mo – 15 letter gain c.f. 1 in sham
RAPD – marked Ranibizumab 45% vs 15% sham
0.3/0.5 mg vs sham 7 letter gain
VA severely impaired At 6/12 – sham group crossed
CWS +++ over to ranibizumab Foveal thickness same
Features
Ischemic
Dilation +++
2–4 mo (100 d glaucoma)
Dot/Flame hemorrhages 4 quadrants Monthly for 6/12
Initial Rubeosis irides Gonioscopy each visit
Macular edema
VA >6/120 10% Follow-up 1–2/12 for 1 y

Prognosis VA <6/12 / ischemic CRVO
Rubeosis iridis 60% Later Then as per complications
VA >6/12, non-ischemic
Gradual taper then annual
Central retinal artery occlusion
Sudden severe painless loss of vision

Cholesterol – Hollenhorst plaque Preceding CRAO
From carotid artery Clinical Amaurosis fugax
Large vessel atherosclerosis Carotid emboli that dissolves
Platelet-fibrin RAPD
Retina and Vitreous
Carotid emboli
TIA/amaurosis fugax
Opaque, edematous retina + cherry red spot
Cardiac valves, atheromatous plaques
Calcific
Most dangerous 1–2% of CRAO
Thrombus GCA
Myocardial infarct, mitral valve stenosis
Causes Atherosclerosis-related thrombosis
Cardiac
Calcific
Aortic valve emboli
Embolus
Bacterial Stroke/Contralateral eye affected
Endocarditis Risks
Myxomatous If elevated
Atrial myxoma – rare Causes ESR/CRP unless embolus present Steroids/Temporal artery biopsy
Giant cell arteritis CRAO
Vasculitis Investigations BP, ECG, BSL, lipids
Commonly AION, but can cause B/CRAO
SLE, PAN MRI + MRA (or CTA) if suspect carotid artery dissection
Cardiovascular
Protein C/S deficiencies Carotid USS & Echocardiocraphy/Angiography
Coagulation 9.18 Retinal
Antithrombin-III deficiency,
disorders Artery
antiphospholipid syndrome Systemic hypotension (older patients), vasospasm
Occlusion (younger patients), papilloedema
Hyperhomocysteinemia
Retrobulbar hemorrhage GCA prophylaxis

Prednisolone 1 mg/kg
Raised IOP
Orbital tumor, orbital inflammatory disease Digital – finger applied to globe
Ocular
Apply ocular pressure
Retinal surgery, neurosurgery Dislodge thrombus Gonioscopy lens
Trauma
Anterior chamber paracentesis
Management 30G needle, inferior 1/3 AC,
Branch retinal artery occlusion
Clinical remove 0.05 mL
If can see embolus => not GCA, Field defect
no bloods necessary Aspirin
Urgent ESR/CRP In 18% by 4–5/52
Iris neovascularization PRP
Investigations
ECG, BP, BSL, lipids, carotid Doppler BRAO
Cardiovascular Review monthly for 6/12, then as per complications
Digital Apply ocular pressure
Dislodge thrombus GP/Cardiologist for risk factors
Gonioscopy lens Management
Review 3-6 monthly initially
Neovascularization rare
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Carotid >90% blockage

Atherosclerosis
Causes
Ocular + chronic carotid artery obstruction symptoms Delayed arm–eye time
GCA FFA Delayed AV transit time
Late
Decrease VA over weeks/months Disc and perivascular leaking
Investigations
Symptoms
Prolonged recovery after bright light Doppler
Pain localized to affected eye Carotid imaging MR angiogram
Angiography
Diffuse episcleral injection 9.19 Artery Occlusion –
Ocular Ischemic
Corneal edema Syndrome Intracameral bevacizumab
Rubeosis in 2/3 patients Anterior segment Paracentesis first to prevent IOP rise
Aqueous flare
Ischemic pseudoiritis Anterior
IOP meds, cyclodiode
Iris atrophy
PRP if proliferative Often unsuccessful since
Mid-dilated poorly reacting pupil Management Posterior segment
Cataract choroidal ischemia present
Venous dilataiton
Not tortuous Signs If obstruction 70–99%
Retinopathy signs NASCET
Arteriolar narrowing Poor VA results
Carotid endarterectomy
Mid-peripheral hemorrhages, microaneurysms
Fundus If 0–70%, 100%
Proliferative retinopathy Antiplatelets
NVD/NVE Natural History Rubeosis
Spontaneous arterial pulsation at disc => 90% blind by 1 y
=> Decreased arterial closing pressure
Ophthalmodynamometry
Low pressure c.f. CRVO (normal to high)
Retina and Vitreous
Retinal edema & hard exudate

Chronic leakage
Decreased vision
Course Rupture
Hemorrhage
Acquired retinal vascular abnormality
General
Spontaneous thrombosis & involution
Systemic hypertension
Common
In 2/3 of cases
Good VA & macula not threatened

Observation
9.20 Macroaneurysms Hemorrhage becomes thrombosed
Indication Recurrent bleeds or exudate

Insidious decreased central vision threaten macula
Leakage involving macula
Edema, hard exudate, fovea involved

Especially at bifurcation Laser
Clinical Treatment
Saccular arteriolar dilatation Argon photocoagulation Long duration 0.5 s, low intensity 200 mW
Often multiple
Avoid arteriole that supply central macula
Retinal hemorrhage
Review 1/12 postlaser
Surgery Tissue plasminogen activator + gas

if submacular hemorrhage
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6th decade, slow vision loss, M = F

Clinical
Vessels not visible on fundoscopy
Need FFA
Symmetrical, 1DD or less area, around fovea
Right angled vessels around FAZ
Capillary bed White refractive crystals temporal to fovea

Rare idiopathic retinal
Dilatation & tortuosity of blood vessels vascular abnormalities No hard exudates/No CME
Telangiectasia
Multiple aneurysms, vascular leakage, hard exudates Acquired Stellate plaques, gray discoloration temporal to fovea
Congenital or acquired Subretinal CNVM may develop
Types OCT
Intraretinal cysts in fovea
Congenital or acquired Bilateral
"Type 2 = 2 Eyes,
Compared with Coats' disease no CME" Capillary dilatation outside FAZ & leakage
Coats’ is male, unilateral, periphery FFA
General
Unilateral Central autofluorescence
Lipofuscin not blocked by
Familial
Bilateral Three groups Presents earlier xanthophyll in Muller cells
occult
Occlusive
9.21 Juxtafoveal No refractile deposits
Retinal
Telangiectasia Guarded prognosis
Aneurysmal
Anti-VEGF
Clinical Preproliferation Does not prevent recurrence
Middle aged male, vision blur
Treatment Laser
Vessels visible on fundoscopy No benefit
Proliferative – CNVM Anti-VEGF x3, then observe 3 monthly

Telangiectasia 1.5DD temporal to fovea Unilateral Photodynamic therapy
"Type 1 = 1 Eye, CME"
Leakage, CME, hard exudates
Rarest, most severe, 6th decade, central vision loss
Congenital
Prevent CME, resolve exudate Laser photocoagulation Aneurysmal dilatation of terminal capillaries
Clinical
to leakage areas Signs
Keep >500 µm away from fovea Occlusive
Progressive occlusion parafoveal capillaries
"Type 3 = A/W
2 types Tumors"
Intravitreal steroids if lesion close to fovea Widening FAZ, NO leakage
Polycythemia, multiple myeloma, CLL

Confined to one clock hour, Associations
edge of FAZ, no leakage Type B – neurological disease
Acquired
No treatment indicated No effective treatment
Retina and Vitreous
75% male, unilateral

General
Idiopathic, non-heriditary
Observe
Treatment Mild, vision not threatened
1st decade, unilateral visual loss Laser

If progressive exudate documented
Leucocoria Symptoms 9.22 Telangiectasia
Coats' Disease
Strabismus Poor
In young with extensive exudate
Intraretinal/Subretinal exudate
Prognosis
Exudative retinal detachment Signs Better
Inferotemporal quadrants If older, less exudate
Between ora & equator Leber's Miliary Aneurysm
Clinical
Older, milder spectrum of Coats'
Rubeosis + glaucoma
Uveitis
Complications
Cataract
Phthisis
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Causes
268
Multiple myeloma, PCRV Hyperviscosity

6/12–3 y postradiation
Clinical
Retinal hemorrhages, venous dilatation Clinical Endothelial cells mitosis damaged
Radiation
Microvascular damage Diabetic retinopathy signs
Occlusion & ischemia Causes
Laser
Severe head/chest trauma Treatment
IVTA for CME
Acute pancreatitis, connective tissue Psuedo-Purtscher
disease, lymphomas, TTP Abnormal hemoglobin => abnormal blood cell
Pathology
Sudden visual loss Purtscher Less deformable => occludes small vessels
Resemble CWS
Clinical Blockage + neovascularization
Superficial peripapillary hemorrhages Multiple white patches Sickle Cell Black population
Disease
Uni- or bilateral Peripheral retinal ischemia => neovascularization
Single arteriole
Macular/optic nerve damage Clinical Sea fan appearance
Guarded Neovascularization
50% have visual impairment Prognosis Single drainage vein
9.23 Retinopathy – Retinal detachments
Other
Venous dilation + small hemorrhages
Once toxicity develops => cessation does not regress disease
=> severe dilation + large hemorrhage
Clinical High-Altitude
Cumulative dose >300 g
=> vitreous hemorrhage => venous engorgement Chloroquine
Stop medication
=> disc edema Superceded, should not be used
Current use SLE, RA, inflammatory/dermatologic disease
Rise thoracic pressure =>
Forcible exhalation against closed glottis
rise venous pressure Dose
Pathology >6.5 mg/kg
Uni/bilateral premacular hemorrhage Valsalva Factor
Perifoveal capillaries rupture Cumulative dose
Under ILM Toxic >1000g
Hydroxychloroquine
Resolves spontaneously (Plaquenil) Bull's eye maculopathy
Clinical
Vortex keratopathy
AD drusen
Visual acuity, 10–2 visual field, fundus photo
Annual review
Bietti crystalline dystrophy If problem noted
Cystinosis, oxalosis OCT, mfERG, fundus autofluorescence
Crystalline
Drugs Causes Stop medication, find alternative
Tamoxifen, talc, indomethacin, hydroxy/chloroquine "ABCD iMac"
Treatment Choroquine
Ichthyosis Not reversible, highly toxic
JFT (MacTel) Hydroxycholorquine

Early reversibility
Retinopathy of prematurity
Retina and Vitreous
Vessel Development Familial exudative vitreoretinopathy
Norrie's
Other
Incontinentia pigmenti
Neovascularization due to ischemia Coats' disease
Features
+/– Vitreous hemorrhage
Diabetes
+/– Retinal detachment
CRVO
Cardiovascular
9.24 Peripheral Retinal HTN
Neovascularization
Anemia
Sarcoid
Vessel Blockage
Sickle cell disease
Behçet's
Macroglobulinemia Blood Quality Wegener's

Vasculitis
(Walden–Strom syndrome)
SLE
Werlhof disease
Hemophilia & thrombocytopenic purpura
Eales disease
Leukemia
Infective
Syphilis
Vitreoretinal Retinal detachment
Trauma
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20% with intracranial bleeds have intraocular hemorrhage
Subarachnoid/Subdural hemorrhage
Cause Trauma
General
Spontaneous
Sudden rise ICP with bleed
Pathology
Obstruction of vein circulation
Often bilateral
Intraretinal
Clinical Hemorrhages Preretinal
Tear until proven otherwise Terson Vitreous
Unless diabetic Examination Syndrome
Vision-obscuring Headache, decreased vision
Weekly review with B-scan hemorrhage
Options Persistent hemorrhage
If no view in 1/52 => Complication ERM
early vitrectomy
Traction/rhegmatous retinal detachment
9.25 Vitreous
Diabetic retinopathy Prognosis
Hemorrhage Good spontaneous recovery
BRVO Neovascularization
Purtscher's retinopathy
ARMD
Shaken baby syndrome
Macroaneurysm Causes Differential diagnoses
Valsalva retinopathy
PVD
Vitreoretinal Blood dyscrasias
Tears
Retinal break/detachment >3/12
Others Only eye/poor vision in other eye

Eales disease, tumor, Terson syndrome
Neovascularization with NVI
Vitrectomy T1DM with proliferative DR
Indications Unknown cause => early vitrectomy

Retinal tear/detachment
2/3 have tears/detachment
Associated submacular tPA + gas to displace hemorrhage
hemorrhage in AMD
Ghost cell glaucoma

Choroideremia – XL
Congenital stationary night blindness Choroid
Gyrate atrophy – AR
Achromatopsia Stationary
Central areolar dystrophy – AD Kearns–Sayre
Blue cone monochromatism Progression
Color blindness Usher

11q13 MYO7A protein
Retina and Vitreous
Protanopia, deutranopia, tritanopia Systemic associations

Progressive Refsum
Most others Rod–cone retinitis
Photoreceptor Abetalipoproteinemia hereditary
pigmentosa
RPE Diffuse vs isolated Bardet–Biedl
Choroidal Anatomical Renal
Classification REMember
Vitreous Ear – hearing problems
Entire retina Mental problems

Widespread vs focal
Rod–cone
Macula Leber's congenital amaurosis
Congenital
Infantile Age of onset Drugs – chloroquine, chlorpromazine
Photoreceptor
Advanced Stargardt's
Childhood Adults
Bulls-eye
Refsum maculopathy DDx Cone–rod dystrophies
Phytanic acid-free diet Cone–rod
Cone–rod dystrophies
Abetalipoprotenemia Treatable 9.26 Fundus Dystrophy Children
Bardet–Biedl
Fat-soluble vitamins ADEK, low fat intake
Gyrate atrophy Leber's congenital amaurosis
Vitamin B6 X-linked RP
Rods
Genetic counseling Congenital stationary night blindness
Reversible causes
X-linked
Blue cone monochromatism
Protanopia
Opinion GROSS approach Cones
Second opinion Color blindness Xq22-28 Deutranopia
Specific treatment Tritanopia
e.g. vitamin A
Supportive groups 11q13 Bestrophin protein
Best's vitelliform macular dystrophy
Sticklers 6p21–22 RDS
Stargardt's/fundus flavimaculatus
Familial exudative Vitreoretinal Interface
Retinal Detachment – Sticklers/FEVR 1p21-22 ABCA4 Rod ABC transporter
vitreoretinopathy RPE Familial dominantdrusen
Retinoschisis – X-Linked/Norrie/
X-linked retinoschisis Favre-Goldman Pattern dystrophies
Xp22.2 RSI protein
Norrie disease Sorsby pseudoinflammatory
macular dystrophy
Favre–Goldman syndrome
North Carolina
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Photoreceptors
A-wave
Waves Bipolar cells
B-wave
Rod–cone dystrophy General
Latency & amplitude of waves
Features
Bilateral General
Loss of peripheral vision Scotopic rod responses reduced

Early
Incidence Reduced a & b waves
1:5,000 Amplitude reduced, prolonged in time
Full-field Late
Photopic responses reduced
Peripheral vision deterioration
Symptoms Advanced
Nyctalopia ERG extinguished
3rd decade, while driving Cone dystrophies Severe, selective cone loss
Arteriolar attenuation Electroretinogram Absent rod function
(ERG) Comparison Fundus albipunctatus
Bone spicule formation Classic ABCD After 3 h, ERG may be normal
CSNB
Color of disc Negative waveform ERG
Waxy pallor
Full field Ganzfeld bright flash
Cone function remnants
Vitreous cells
Clinical Scotopic rod response
CME Extinguished
Rx with diamox Rods affected more than cones
Signs Scotopic maximal ERG
9.27 Photoreceptor Cone function remnants
Posterior subcapsular cataract Other ERG
Dystrophy Photopic ERG
Rx with surgery Other Cone function remnants
ERM Retinitis
RPE atrophy => unmasks Tessellated fundus Pigmentosa Photopic 30 Hz flicker
– General May have cone response
choroidal vessels
Optic disc drusen Oscillatory potential
Not recordable in RP
Myopia
Bipolar cells
Autosomal dominant Electro-oculogram (EOG)
40%, best prognosis Subnormal, absence of light rise
Other Central island of vision
Autosomal recessive
Investigations Visual field Annular midperipheral scotoma
20%, intermediate prognosis Inheritance Island may disappear
10%, severe Do ERG on mother

RP male with unknown
Male – unaffected X-linked inheritance Abnormal peripheral pigmentation
Affected male's progeny If X-linked disease
Female – carriers Abnormal dark adapted ERG
20% sporadic
Central vision loss
Poor
Prognosis Age 50 y, VA<6/60
25% have good VA

Restricted fields but can read
VA, HVF, OCT (CME), cataract
Follow-up 6–12 monthly
Retina and Vitreous
Ammetropia/Myopia correction
Reversible
causes Cataract surgery
Macular sparing
Retinitis puncta CME
Small white dots at equator Treatment Acetazolamide 500 mg/d
albescens
Vascular "Atypical" RP
attenuation Vitamin A Non-pregnant >21 yo
Sector of RP Medical
Controversial
"Pseudo" RP May slow ERG deterioration
Usually unilateral, asymmetrical and atypical Genetic counseling
9.28 Photoreceptor
Dystrophy Retinitis Low vision services
Quinine/hydroxychloroquine Pigmentosa – Management
Drugs
Phenothiazine Kearns–Sayre
Syphilis Usher
Rubella Infective KURABy Refsum

Differential
Measles Diagnoses Hereditary abetalipoproteinemia
Systemic
Chronic CSR Bassen–Kornzweig syndrome
Bardet–Biedl syndrome
Laser PRP scars DISCO
Retinal detachment Scarring

RENAL problems
Trauma
REMember to look for EAR – hearing problems
Uveitis
Vogt–Koyanagi–Harada syndrome MENTAL problems
Cancer-related retinopathy
CRAO Occlusion (vascular)

Ophthalmic artery occlusion
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General
274
Worse prognosis than cone–rod
General Autosomal recessive, rod & cone

Blindness at birth
Features Vision <6/60 to light perception

Nystagmus in first 2–3/12 of life
Hyperopic refraction
Sluggish pupil response
Diagnosis Oculodigital reflex
Group of disorders Marbelized
X-linked RP Fundus
General Rod–Cone
X-linked (with myopia) Leber's congenital
Flat ERG RP
amaurosis
AD/AR (no myopia)
Mimicking treatable Refsum – restrict phytanic acid
Stationary (not progressive) conditions
Treatment Bassen–Kornzweig – Rx with Vit A & E
Nyctalopia Gene therapy
RPE65 subretinal injection
VA <6/60, strabismus, nystagmus
Clinical Mental retardation
Normal
Congenital stationary Deafness
Oguchi disease Fundus Rods Systemic
night blindness 9.29 Photoreceptor Epilepsy, CNS
Fundu salbipunctatus Dystrophy – Others
Renal abnormalities
Reduced scotopic b-wave Skeletal abnormalities
ERG
Abnormal dark adaptation Most sporadic, or autosomal dominant
Blue cones encoded on X-Linked General Rods often preserved => vision stabilizes
autosomal chromsomes Red/green cones encoded
on X chromosome 1st–2nd decades gradual bilateral
So red/green loss more common central & color decline
Good night vision Bull's-eye maculopathy

Red/green cones lost Blue cone
Fovea normal/granular, Progressive RPE atrophy
monochromatism
Small amplitude nystagmus Poor day vision Clinical macula sheen
Eventual geographic atrophy
Decreased VA ~6/18 Cones
Cone–Rod Bone spicules, arteriolar attenuation, temporal disc pallor
Non-progressive GreeDy ReP needs their BuTt kicked!
Color Reduced photopic & flicker fusion
Deutranopia blindness ERG
Green Rod response preserved
Protanopia Investigations EOG
Red Normal/subnormal
Tritanopia Severe deuteron-tritan defect
Blue, rare & not X-linked Color vision
Out of proportion to VA
Rod monochromatism
Rod or blue cone
Achromatopsia Similar to LCA
Nystagmus + photophobia
Symmetrical bilateral macular deposits AD, bilateral lipofuscin deposition at macula
4th–6th decade General OCT shows lesions within RPE
Yellow subfoveal deposits
OK until 5th decade
Pigmented + hypopigmented halo Signs Visual acuity CNVM, geographic atrophy
Adult-onset Normal fundus
Autofluorescence foveomacular Stage 0 Previtelliform
vitelliform Subnormal EOG (Arden ratio <1.50)
Central hypofluorescence, ring FFA
hyperfluorescence Stage 1
Retina and Vitreous
Investigations Pigment mottling at macula

No leak
Vitelliform Sunny-side young egg yolk
Round, elevated central Best's Vitelliform Stage 2
OCT Pattern Dystrophies Clinical Vision ok
lesion outer retina
PV P V P V Lesion starts being absorbed
Prognosis Pseudohypopyon
Stage 3
Some may have FTMH Vision ok
2nd–3rd decade of life Butterfly-shaped
Good prognosis macular Vitelliruptive stage Egg yolk breaks
Stage 4
Yellow pigment macula Vision drops
Triradiate arrangement Stage 5
4th decade Macular scarring, CNVM, geographic atrophy
Multifocal yellow lesions Simulates fundus flavi Multifocal EOG Bipolar cells measured
Investigations
Hyperfluorescence of flecks Arden ratio <1.5, around 1.1
FFA
Choroid not dark AR, ABCA4 gene on 1p21-22
9.30 RPE
General
Dystrophy 1st–2nd decades
aka Doyne honeycomb choroiditis Gradual vision impairment
AD, full penetrance General Non-specific mottling
Similar appearance to fundus flavi Clinical Oval "snail slime"/"beaten bronze" fovea
Surrounding yellow flecks
3rd decade Geographic atrophy
Mild Familial Dominant Stargardt's
Small hard drusen, macula Drusen Bull's-eye maculopathy
FFA Silent choroid = Stargardt's
Moderate
Large soft drusen, posterior pole/peripapillary Signs Investigations
MfERG
Advanced Diminished responses
CNVM, GA
VA 6/12 => 6/60
Spoke-like basal laminar drusen Malattia
leventinese Treatment
Centered around fovea Avoid vitamin A overdose
Symptoms 40–50s due to CNVM/GA
Pseudoinflammatory, AR, ABCA4 gene on 1p21-22
macular, AD TIMP gene General
Age 40–50 y, Stargardt spectrum
Subretinal scarring
Bilateral subfoveal CNVM Sorsby
4th decade Fundus Bilateral ill-defined yellow-white
GA Flavimaculatus deep retinal lesions
Other Clinical
Yellow drusen deposits along arcade
Nasal to disc Round, linear, semilunar
Flecks
Rare, nonprogressive AD Pisciform (fish tail-like)
North Carolina
Confluent macular deposits Investigations
Drusen Fundus autofluorescence
Coloboma – atrophic macular lesions
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General
Autosomal recessive, ornithine
aminotransferase deficiency
X-linked recessive
Nyctalopia & VF constriction & axial myopia
Defect between RPE & PR
General PSCC
Like RP but lose more layers Ocular
CME
Choriocapillaris affected = "anemia"
Clinical
Signs Circular patches atrophy mid-periphery
Choroideremia
Nyctalopia & VF constriction Gyrate Atrophy
Fundus Peripheral & central spread
RPE & choroid atrophy mid-periphery
Signs Foveal sparing
Clinical
Central spread, macular sparing till late
Legally blind by 50 yo
Female carrier 9.31 Choroidal
Mild patchy pigmentary atrophy of periphery Dystrophy Reduce ornithine levels (plasma, urinary)
Treatment Vitamin B6
Responder vs no responder
Autosomal dominant
Dietary restriction of ornitihine
General
3rd–4th decade gradual impairment central vision
Non-specific foveal granularity

Central Areolar
Circumscribed RPE atrophy Clinical
Macula depigmentation
Loss of macula choriocapillaris
Late Onset Retinal Old age presentation

Poor prognosis Degeneration (LORD)
Peripheral pigmentary degeneration
Severe vision loss by 6th–7th decade
Deficiency of tyrosinase Pathology 10% of all albinisim
Tyrosine to dopaquinone affected X-linked OA1 gene
Melanosomes decreased
Ocular 10%
Nystagmus
aka Nettleship–Falls albinism
Reduced vision Tyrosinase negative Ch11q
4 out of 5
Autosomal recessive
Iris transillumination
Retina and Vitreous
features
Macular hypoplasia Classification White cells
Chediak–Higashi
OCA 1 gene Tyrosinase => Recurrent pyogenic infections
Fundal hypopigmentation Oculocutaneous positive Ch15q
90% Platelet
Photophobia Hermansky–Pudlak
Other features => Bruising
Ametropia, strabismus, amblyopia Ocular
Puerto Rican ancestry
Asymptomatic OCA 2 – P gene
If + ve => oculocutaneous albinism
Partial iris transillumination 9.32 Ocular OCA 3, OCA 4 – rare
Female Albinism
Macular stippling Features
Look at mother Perform if diagnosis uncertain
Mottled area of hypopigmentation
Complete albinos Visual evoked potential from 3/12 Monocular flash in RE => larger wave in L occipital lobe
"Full crossing"
Refractive error, strabismus Investigations
Pathognomic
Nystagmus Hair bulb/skin biopsy
Pendular, horizontal Tyrosinase test
Iris Clinical Full blood examination/
Diaphonous, translucent Check for Chediak–Higashi/Hermansky–Pudlak
coagulation studies
Lacks pigment, large choroidal vessels
Tyrosinase
VA <6/60 negative Ammetropia
Vessel through center of macula Foveal
Photophobia
hypoplasia Ocular Tinted lens
No umbo Fundus Amblyopia
Vessel cross horizontal raphe
Strabismus/Nystagmus
Lack perimacular vascular arcades Surgery
Treatment
Skin cancers
More decussated fibers UV protection
Optic General
Majority go to chiasm Genetic counseling
contralateral hemisphere
Vision
VEP to detect Support
Education
VA 6/24 – 6/60
Foveal hypoplasia, refractive error, strabismus Geneticist/Dermatologist/Hematologist
Variable amounts of melanin Tyrosinase
Pale at birth, darken with age positive
Iris
Blue/dark brown with translucency
Fundus
Variable depigmentation
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EOG light peak Ganzfield stimulus – a bowl,
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RPE cells Dim scotopic

flash stimulate all parts of retina at
ERG A-wave same time
Photoreceptors Dark adapted 30 min, dilated
Tests
ERG B-wave
Amacrine, indirectly Muller cell function
Bright scotopic
Oscillatory potentials flash Rods + cones, but since
Ganglion cells many more rods
Pattern ERG
Nerve fiber layer Photopic flash
Still many rods involved
Check rod then cone function Light adapt now
ERG Pure cone response
Pattern ERG for macula Approach Flicker 30 Hz Critical fusion frequency
for rods 18, for cones 50
Multifocal ERG for fovea
140 million
Then go through dystrophies rods, 6 million
Vitreoretinal/Photoreceptors/RPE/Choroid cones, but
ERG Rod-Mediated 1.2 million
Macular Vast majority are macular
Quantity of functioning retina Responses ganglion
function
A-wave Amplitude Pattern ERG => Ganglion cell layer function proxy for
Baseline to trough, outer retina
macular function
B-wave
A-wave trough to B-wave peak, inner retina P50 – ganglion cell layer
Latency (implicit time)
Quality of functioning retina 9.33 Electrophysiology
N95 – optic nerve function Expect it to be more negative
Likely genetic than baseline
Symmetry between both eyes Ratio – N95:P50 > 1
Rod–cone, cone–rod, choroideremia Symmetry vs
asymmetry
Unilateral disease – any retinal disease
Response from central
Acquired usually, even if systemic Asymmetry between eyes retina Pattern, center 20 degrees,
can be asymmetric Key Features Multifocal ERG focused on fovea
of Flash ERG 61 hexagons
e.g. AZOOR, medications
Global vs macular
e.g. macular dystrophies – Stargardts
Electro-oculogram
Stationary vs progressive
EOG 12 min in dark, then light; moving eyes left right
e.g. only cones affected vs rods Disproportionate Light peak twice amplitude of dark
=> e.g. achromatopsia Arden ratio
Inherited At least 1.8–2
CSNB, retinoschisis, Goldman–Favre
Visual evoked potential e.g. autosomal dominant
Melanoma/Carcinoma associated retinopathy VEP
Negative waveform – optic atrophy
Autoimmune retinopathy inner retinal Reduced => decreased optic nerve function ERG would look normal
dysfunction
Toxicity
e.g. Quinine Acquired
Inflammation
Birdshot chorioretinopathy
Vascular
CRAO, ischemic CRVO
Pathology
Vitreous fluid between RPE/retina
Centripetal force
Retinal tears/RRD Dynamic Vitreoretinal
traction
Independent of eye movements
Static Horseshoe
Tractional RD/Proliferative VR
> 90 degrees
Retina and Vitreous
Giant retinal tear

Separation cortical vitreous from PreBreak ircumferentially
ILM posterior to vitreous base Operculated hole Traction separates
Definition
retina completely
Vitreous base Tear
2 mm anterior/4 mm posterior of ora Macular hole
Classification
Syneresis => fluid past posterior hyaloid Circumferential linear breaks
Process Posterior vitreous
Vitreous remains attached at base detachment (PVD) Not PVD Dialyses Trauma most common cause
Remaining adhesions => TRACTION associated c.f. GRT – dialyses has no retina
PVD + vitreous hemorrhage anterior to break
60–90% have tear Clinical
Atrophic hole
Axial myopia No VR traction or RD
Aphakia
9.34 Retinal
PVD Tear Equatorial breaks
Inflammatory disease
Trauma Ciliary epithelium + ora serrata
+ postora retina
Myopia Traumatic
Tears Dialysis Anterior vitreous detachment
Previous rhegmatogenous retinal detachment + dialysis = pathognomic
Discontinuity of ILM Macular hole
Histology
Vitreous condensation & adherence at margin Lattice Delayed 12% immediately
degeneration Detachment presentation
1% have retinal detachment rare 50% @ 8 mo
Prophylaxis Young patients
Only high myopia, retinal detachment Risk Factors 89% @ 24 mo
fellow eye, tear, aphakia Tear
With pressure Chorioretinal scar

Translucent gray on indentation around break
White Goal Prevent vitreous enter subretinal space
Same without indentation
Without pressure Atrophic
Risk giant retinal tears
Low Operculated
Previous cataract surgery
Treatment Macular holes
Retinoschisis
Risk High
Vitreoretinal tufts Acute symptomatic
Meridional folds U-shaped tears
RPE hyperplasia/hypertrophy Treat Any traction
Cobblestone degeneration Non-factors Laser beyond subretinal fluid
Peripheral cystoid degeneration
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Rhegmatogenous = arising from break or tear

Retinal breaks
> 40 yo 7%
Prevalence of RRD
1:10,000
11:100,000
1/3 myopic
Layer – OPL Neurosensory Lifetime risk 1%
Between Epidemiology
layer splits into two 1/3 postcataract
PR & bipolar Age 60 y – 6% lifetime risk
Layer – NFL Pathology Incidence surgery
of RRD Age 70 y – 0.6% lifetime risk
Absolute scotoma in area
Complicated surgery – higher risk
Inferotemporal, bilateral (Retinoschisis)
Hypermetrope more common Rhegmatogenous Family hx, trauma, lattice, retinoschisis,
Clinical Other retinal tufts, macular holes
Anterior to equator, smooth dome shaped
No evidence for YAG capsulotomy
Small risk retinal detachment
Flashing lights
No treatment required Photopsia
9.35 Retinal Detachment Floaters
4 Fs
Classification Symptoms
Proliferative retinopathies (ROP, DM) Fields
Scotoma, shadow
Toxocariasis Failing vision
Primary cause
Penetrating trauma Break in 95%
Superotemporal most common
"Tobacco dust" pigment cells in vitreous Tractional Tobacco dust in 95%
Sickle cell disease Signs
Break Frequent PVD
No primary break
Demarcation line
Taut retina, peaks at traction points
Blood retinal barrier
Treatment compromised
Pathology
Delamination Serous fluid leak into subretinal space
Disconnect vascular pegs
connecting membranes
Malignant hypertension, eclampsia, renal failure
Primary cause
Exudative/Serous Consider neoplasia until proven otherwise
No break
Signs Smoothly elevated, no tobacco dust
Multifocal areas
Breaks superior 2/3 fundus
Tear
Single or small cluster
Ocular Associated PVD
Indications Clear media
Other For laser
No glaucoma risk Gas injected
Retina and Vitreous
FIND all breaks Patient must posture postprocedure

Non-ocular
Laser Pneumatic No access to theater
Retina & RPE into contact Principles – FAR Retinopexy Single operation
Cryotherapy ATTACH all breaks 75%
Diathermy Success Final operation Pneumatic + surgery

RELIEVE vitreoretinal TRACTION 96%
More breaks/PVR
<10%
Position patient to put pressure on detachment Preoperatively Untreated tears
Large superior tear Subretinal gas
Urgent General
Syneretic vitreous & macula still attached Complications Lens contact
3 mo Demarcation line High IOP Diamox overnight

Chronic retinal detachment
Retinal cysts Vitreous hemorrhage
12 mo
9.36 Retinal Detachment PVD present
Age, patient preference, 1st vs 2nd eye Management
Cannot buckle
Assessment GRT, posterior breaks, PVR
RAPD, rubeosis, and B-scan for
detachment if poor view Indications Breaks not visible
Vitreous hemorrhage, cataract, IOL edge effects
Lens status Considerations
Surgical view Traction threatening macula
Clarity of media Doubles volume, USE 20–30%
Penetrating trauma
Location, size, number of breaks SF6 Lasts 2/52
Retina status
Extent of proliferative vitreoretinopathy (PVR) Expanding gases Quadruples volume, 12–16%
Breaks large, easily visible, good locations – pneumatic/buckle Vitrectomy C3F8
Lasts 6–8/52
Retinal dialyses High specific gravity
Absolute Heavy liquids
Young myope, no PVD Tamponading
Vitreous adherent => Leave 1/52 only
Indications agents Risk ERM/traction
risk iatrogenic tears Breaks in a line
Relative Scleral Buckle Low specific gravity
Younger, phakic Floats above water
Inferior tear Inferior PI prevents pupil block
Silicone oils
Failure of SRF drainage PVR, postdiabetic
delamination, trauma
Hemorrhage Complications
Choroidal, subretinal, vitreous Indications Inflammatory retinitis – ARN, CMV
Retinal incarceration in sclerotomy
Recover VA >6/12 Breaks 4 quadrants
Macula attached 87%
Prognosis Mechanical intraoperative reasons
<1/52
75% > 6/18 Social – only eye, air travel
Macula detached
>1/52
50% >6/18
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Iatrogenic breaks
Intraoperative
Retina/lens touch
Raised IOP
Early Postop
Inflammation excessive
RPE & glial cells metaplasia
Membrane formation
Endophthalmitis
Pathology
Membrane contraction
9.37 Retinal Detachment Traction
Complications A – vitreous haze, tobacco dust
Proliferative
vitreoretinopathy Grades Rolled edge retinal break
B – wrinkling inner retina, retinal stiffness
Blood vessel tortuousity
Late Postop
C – full thickness retinal star folds
Heavy vitreous condensation
Re-attach retina early
Treatment Close breaks, heavy liquids
Relieve traction with retinotomy

Recurrent retinal detachment
Cataract
Does not expand
Air
Intraoperative retinal flattening Dissolves 2/7

Purpose
Retina and Vitreous
100% concentration doubles in volume Use 0.6 mL 100% => 1.2 mL

Tamponade of retinal breaks postoperatively after expansion
Isovolumetric amount
SF6 20–30% concentration does not expand
Expands over 24 h
Low specific gravity => floats on vitreous Lasts 2/52

Expanding Gas
Prolonged tamponade required 100% concentration quadruples in volume Use 0.3 mL 100% => 1.2 mL
after expansion
PVR Isovolumetric amount

9.38 Retinal C3F8 12–16% concentration does not expand
Trauma High risk
re-detachment Surgery – Expands over 72 h
Inflammatory retinitis Tamponading
CMV, ARN Agents Lasts 6–8/52
Postdiabetic Indication
delamination No flying
Prevent recurrent >500 m height
hemorrhage, allowvisual access
Mechanical Until gas cleared
Risks No diving
Required heavy liquid during operation Silicon Oil
No nitrous oxide anesthetics
Air travel required
Social
One eyed
Can see through oil, cannot through gas
1000 Centistoke e.g. Densiron
Types
5000 Centistoke High specific gravity => sinks to bottom
Heavy Liquids Good for inferior pathology
Can stay intraocular longer
Superior PI to prevent pupil block glaucoma
Inferior PI to prevent pupil block glaucoma
Aqueous uses inferior PI to flow
Remove 2–3/12 Remove 1–2/52
Risk ERM & traction formation
Vision
+6 D Hyperopia
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Consider Neoplasia Until Proven Otherwise
Posterior scleritis
Unilateral
Inflammatory Signs Sympathetic ophthalmia
Bilateral Vogt–Koyanagi–Harada syndrome
Uveal effusion syndrome

9.39 Serous Retinal
Detachment Central serous retinopathy
Unilateral
Choroidal melanoma
Optic nerve pit with serous

No Inflammatory Signs macular detachment
Exudative ARMD
Bilateral Choroidal metastasis or lymphoma

Malignant hypertension & eclampsia
Nanophthalmos
10 Trauma
Trauma
10.1 Terminology
10.9 Lacrimal Drainage System Trauma
10.2 Risk Factors for Poor Outcome Globe Trauma
10.3 Investigations & Initial Management 10.10 Light Toxicity
10.4. Complications Electromagnetic 10.11 Solar Retinopathy
Radiation
10.12 Radiation Retinopathy
10.5 Globe Lacerations
10.13 Phototoxicity from Ophthalmic Instruments
10.6 Intraocular Foreign Body 10.14 Orbital Fracture Assessment & Management
Fractures 10.15 Orbital Fracture Classification
10.16 Orbital Floor Surgery

10.7 Blunt Trauma
10.17 Post-Traumatic Vision Loss with Clear Media

10.8 Eyelid Trauma
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Contusion
Closed Globe Injury No full thickness wound to eyeball Lamellar laceration
Superficial foreign body
Full thickness wound to eyeball
Open Globe Injury Intact

Choroid, retina Prolapsed
Damaged
Blunt trauma transiently increasing IOP

Opening scleral wall
Rupture
Wound not necessarily at impact site
10.1 Globe Trauma
– Terminology Frequent tissue herniation & loss
Laceration Wound is outside-to-in mechanism at impact site
Penetrating Injury Single entrance wound
Perforating Injury 2 wounds

Entrance & exit by same object
Intraocular foreign body

IOFB
Retained due to penetrating injuries
Blunt
Trauma
Gunshot
Large
Wound
Posterior scleral
Vision Poor initial vision
RAPD
10.2 Globe Trauma – Structure Damage Lenticular involvement

Risk Factors for Dense vitreous hemorrhage
Poor Outcome
VA perception of light or worse
Hemorrhagic chemosis
Signs of Anterior Hypotony
Scleral Rupture
Pupil peaking
Lens – dislocation, subluxation
Vitreous traction, hemorrhage

Posterior
Choroidal detachment
Admit
Nil by mouth
Trauma
Practical
Shield
Investigations
CT, B-scan
Tetanus prophylaxis
Topical
Medical Antibiotics Systemic IV ceftazidime 1g bd

Immediate
Management Oral ciprofloxacin 750 mg bd
Cycloplegia
Donor cornea/sclera Primary closure

Not usually for enucleation
Consent Second procedure once patient
10.3 Globe Trauma – understands poor prognosis
Investigations &
Initial Management OR booking
General anesthetic
CT orbit/brain Risk of expulsive
Difficult to see wood/glass/plastic Surgical
Anesthesia Avoid suxamethonium
Plain x-ray May cause EOM contraction
Prep & drape & swab if infection
B-scan
Investigations Infection
Swabs, AC & vitreous tap
MRI – contraindicated if metallic
Preoperatively
Axial length, keratometry & lens calculation Antibiotics, cycloplegia
From fellow eye Topical
Steroids
Medications Taper 4–6/52
Photos
IV then oral antibiotics
Systemic
Analgesia
Postoperative Secondary repair

Surgery
Vitreoretinal surgeon
Amblyopia therapy
Suture removal 6–8/52 selective removal

Follow-up depending on keratometry
Sympathetic ophthalmia
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Incidence
2–7% penetrating injuries
IOFB
Type of IOFB
Risk factors
Capsular rupture
Time to closure
Post-Traumatic
Activation of inflammatory system Compression injury chest, Endophthalmitis
head, lower extremities Gram negative
Ceftazidime intravitreal
Occlusion of small arterioles
Mechanism 25% of penetrating injuries endophthalmitis
Organisms
No direct ocular injury Soil contaminants
Bacillus cereus
Purtscher Rapid visual loss
Multiple CWS Retinopathy
Intravitreal vancomycin & clindamycin
Peripapillary hemorrhages Signs
Prophylaxis
Bilateral Ciprofloxacin 750 mg po bd
10.4 Globe
No treatment Trauma –
Normal VA in 50% Band keratopathy
Complications
ERM
Incidence Chronic Neovascularization
1:500 penetrating injuries
Inflammation BEN GO blind
Glaucoma
Sympathetic
Only if globe cannot be repaired Primary Ophthalmia Macular edema
No visual potential Enucleation Cataract

Secondary
Enucleate by 2/52 to reduce
sympathetic ophthalmia risk Obvious capsular rupture + flocculent material
Retinal Cataract extraction if
+/– Proliferative vitreoretinopathy No fundus view
Detachment
Phacoanaphylaxis
Lens Trauma Less inflammation
Benefit in delaying May not progress
Better measurements
Potential instability
Surgery
Phacoemulsification => +/– convert to ACIOL/stabilized PCIOL
Trauma
Wound closure
Close the globe Reposition/excise exposed uvea

Initial
Management Plan Vitreous excised from wound
AC reformed
4–14 d postoperatively
Vitrectomy
Subsequent
Decrease tractional retinal detachment
Evaluate extent
Conjunctival peritomy
Retina/choroid/ciliary body
Reposited unless necrotic
Prolapsed tissue Vitreous
Stroll vitrectomy
Iris
Excised if >12 h/necrotic/contaminated
Corneoscleral Wound Reform AC
Viscoelastic
Suture anterior to posterior
Sclera
8-0 Nylon partial thickness
Suture wounds
10.5 Globe Lacerations 10-0 Nylon, interrupted, 75% partial thickness, buried
Cornea Large compressive sutures periphery
Decrease astigmatism by
Small short sutures centrally
Leak check
Reinflate AC & 2% fluorescein
360 degree peritomy

Evaluate extent
If beyond muscle insertion
Disinsert muscle & suture
Reposition viable tissue
Prolapsed tissue
Posterior Full Thickness Remove necrotic tissue
Wound/Rupture
Repair globe integrity
Culture/Prophylactically treat for infection
+/– Encircling band for retinal support
Vitreoretinal input
Second surgery 10–14/7 postinjury
Safer to induce PVD
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Magnetic
Iron, steel – siderosis
Severe inflammatory reaction
Copper – chalcosis
Non-magnetic
Wood, vegetable matter
High Foreign Body Magnetic
Delay exam until surgery Nickel
Extrusion risk Mild inflammatory reaction
Low Non-magnetic
+/– Gonioscopy if no wound Aluminium, mercury, zinc
Slit lamp exam Assessment
leak & globe intact Gentle Carbon, gold, silver, coal
B-scan Inert – if clean entry
If no extrusion risk & suspect posterior rupture Stone, glass, plastic
CT first, never MRI

Oxidation Cell damage, loss of function
Iron deposits
Toxic to photoreceptors & RPE cells
If inert
Observe Symptoms
Delayed infection, inflammation, Periodic examination 10.6 Intraocular Nyctalopia, constricted fields, decreased VA
migration of FB
Foreign Body Corneal stromal staining
Risk intracranial bleed, Removal of foreign Heterochromia, mydriasis
Only if know depth & direction
CSF leakage body at slit lamp
Iron in trabecular meshwork
Siderosis Signs
Infection/Inflammation Secondary OAG
Lens deposits
Organic, wooden, copper, iron Surgical indications Cataract
Retinal pigmentation
Optic nerve compression signs Management
Optic atrophy & discoloration
Pars plana vitrectomy
Early – decreased A-wave
Pars plana magnet extraction
Small metallic FB ERG Later – decreased B-wave
Larger, non-metallic FB
Forceps extraction End – extinguished ERG
Vitreous Surgery
Affinity for limiting membranes
Scleral trap door incision Copper deposits
FB embedded in retina Severe inflammation
Or pars plana vitrectomy Poor VA

+ retinotomy & forceps Descemet's membrane – Kayser–Fleischer ring
Chalcosis Iris green discoloration

+/– Lensectomy Signs Sunflower cataract
For better view Vitreous opacities
Metallic flecks on retinal vessels/macula

Trauma
Limbus
Common sites
Parallel to/under EOM insertion
Sclera thinnest
Decreased ocular ductions

Scleral Rupture Conjunctival chemosis, hemorrhage
Vitreous Signs Deepened AC
Determine cause
Tear, blood vessel, choroidal detachment Hemorrhage
Severe vitreous hemorrhage
Cannot visualize => B-scan
"Sclaw" = tear, pull, or scratch
Outer retinal layer damage by shock waves High velocity missile passing through orbit
Chorioretinitis Mechanism
Posterior pole = Berlin edema Commotio Sclopetaria No direct globe contact
Retinae Shock waves
Features
Sheen-like retinal whitening
Choroidal & retinal rupture
No treatment, good prognosis Bare sclera on fundus exam
Signs 3 layer hemorrhage
10.7 Blunt Sub-, intra- & preretinal
Eye compressed Trauma "Claw-like" break in Bruch's
antero-posterior
direction Ruptured globe
Choroid stretched horizontal axis Mechanism Differential diagnoses White/yellow crescent
Choroidal rupture
Bruch's membrane tear => No retinal rupture
Choroidal rupture
Choroidal Treat retinal dialysis or detachment
Rupture Treatment Prognosis
Rupture at posterior pole => Permanent visual loss
dependent on size
Pale crescent area in retina Clinical Total visual loss
Single/multiple Avulsion of
Concentric to optic disc Optic Disc + Posterior hemorrhage
Pit-like depression of nerve head
+ Contusion necrosis
Review 1–2/52 until choroid visible
CNVM Hemorrhage seen acutely
Amsler grid to patient Complication
Other Angle recession
Post-traumatic macular hole

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Check deep foreign body

Orbital fat in wound => Irrigation ++
orbital septum violated
Fat prolapse in upper eyelid
Need levator exploration
Orbital cellulitis prophylaxis
No margin Conservative debridement
involvement
Technique Small caliber sutures
6-0 to 7-0
Laceration Evert wound edge
Early suture removal
Orbicularis & eyelid skin meticulous closure
Do not suture septum
Septum tethered to fascial layers
Suture => risk secondary lid position disorders
10.8 Eyelid Trauma Gray line, meibomian gland, eyelash line
Margin Margin alignment
Surgery 2–3 sutures, moderate eversion
involvement
Direct tarsal approximation
Suture should not extend on conjunctiva surface
Skin
Use to tie down margin suture tails
Mechanism
Horizontal traction
Canthal Tissue Trauma
Examine lacrimal drainage apparatus
Pull lid with forceps
Medial canthus
Examine posterior portion of tendon attachment to lacrimal crest
Direct laceration
e.g. stab wound, dog bite
Mechanism Traction
e.g. sudden lateral displacement
Blunt trauma with eyelid laceration =>

Suspect medial injury - probe & irrigate
50% asymptomatic
One functioning canaliculus 10% constant epiphora
40% symptomatic epiphora + ocular irritation
Primary repair Success better than secondary reconstruction

Canaliculi
Aim <48/24 h repair
Identify severed ends
Irrigate canaliculus Air, fluorescein, yellow viscoelastic
Prevent postoperative canalicular strictures

Management Stent injured canaliculus e.g. Mini-monoka
Place stent on traction
Draw together severed ends
10.9 Lacrimal Drainage Repair medial Leave in situ >3/12
System Trauma canthal tendon
Cheese-wiring
Complications Ocular irritation, infection, inflammation
Pyogenic granuloma
Remove stent
Direct laceration
Mechanism
Lacrimal Sac & Fracture of surrounding bones
Nasolacrimal Duct Fracture reduction
Soft tissue repair
Management
Silicone intubation
Entire lacrimal drainage system
Trauma
Physiology
Blink, squint, aversion reflexes
Mechanical
Eyelids, brow
Absorbs UV B & C, some infrared

Cornea Reflects ~60% tangential
incidental light
Protective Miosis
Mechanisms Pupil
Pigmentation
Absorbs UV A & visible blue

Lens
Absorbs more with age
Xanthophyll absorbs UV B & blue

Choroidal circulation
10.10 Light Retina Thermal heat sink – temperature control
Toxicity Intracellular molecular detoxification
Irradiance
RPE mediated photoreceptor renewal
Duration of exposure
Toxicity Factors
Wavelength of incident light High irradiance, short duration
Mechanical Disintegrated target tissue => plasma forms

Absorption by target tissue Photodisruption
e.g. ND:YAG laser
Critical temperature rise in tissue
Protein denaturation, enzyme inactivation

Tissue Effects
Thermal Photocoagulation
Coagulation, necrosis, hemostasis
e.g. panretinal photocoagulation
Low irradiance, short wavelength

Photochemical
Cellular damage at low temperature
Photon absorption => chemical reactions
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Retinal injury aka Fovea–Macula

Due to direct/indirect solar viewing Retinitis
RPE mottling
Photochemical reaction Yellow–white spot in fovea
+/– Lamellar hole
Signs
Environmental Days later – replaced by red dot
Duration of exposure, season, ozone
RPE detachment, necrosis, pigmentation
Histology
Dilation
Abnormal photoreceptors
Clarity of media Ocular
Emmetropia/hypertropia focus better Risk Factors

10.11 Solar
Retinopathy
Increased body temperature
Investigation OCT scan
Amount of pigmentation Systemic
Drugs
+/– Residual metamorphopsia
e.g. tetracyclines
Prognosis Resolves within 3–6/12
+/– Paracentral scotomata

VA 6/7.5–6/36 or worse
1–4/24 h
Symptoms
After image, paracentral scotoma, metamorphopsia

Trauma
~18 mo postexposure
General Optic atrophy
~ >30–35 Gray's exposure

Complications CRAO, CRVO
Choroidal neovascularization membrane
Rubeotic glaucoma
Symptoms 10.12 Radiation

Decreased VA Retinopathy
CWS Prognosis Depends on macular involvement
Hemorrhages
Micro-aneurysms Similar to diabetic retinopathy
Retinal vascular disease Signs
Perivascular sheathing (like diabetic retinopathy) Clinical Treatment Macular edema
Focal laser
Telangiectasias
Ischemia, CNVM
Macular & disc edema
PRP laser
Capillary nonperfusion
Fundus fluorescein angiogram
Retinal ischemia
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Photochemical +/– thermal enhancements

24–48 h Yellow edematous lesion
3–7% of cataract patients General Match shape of light source
Vision returns to normal Fades over weeks

Signs RPE clumping + atrophy
Complications
Intensity of light CME, CNVM
Duration Histology
Surgery 10.13 Phototoxicity from RPE & photoreceptor tight junction damage
Lack of filters
Ophthalmic Instruments
Topical anesthetic
"Look at light"
Increased chorioretinal pigmentation Risk Factors No Treatment

Heat sink Dilation Eye
Maculopathy
Patient
Raised body temperature
Blood oxygen levels Systemic

Free radicals
Diabetes
Trauma
Identify fractures
Bony windows
CT orbit 2 mm coronal slices Prolapsed EOM, orbital fat
Investigation Hemorrhage
If loss of consciousness => CT brain
Mechanism If intraocular foreign body => x-ray, CT scan

MRI if not metallic
Diplopia
History No nose blowing
Numbness
Ice packs to orbit for 24/24
Epistaxis General
Orthoptic follow-up
Visual symptoms & pain
Warning signs
10.14 Orbital Retinal precautions & orbital cellulitis precautions
Fracture – Assessment &
Management Especially if diabetic, sinusitis,
immunosuppressed
Antibiotic
prophylaxis
Treatment Amoxycillin + clavulanic acid
Bony tenderness Decrease edema &

+/– Rim discontinuity Prednisolone
long-term diplopia
1mg/kg for 7/7
Ecchymosis Medications Oral steroids
Decrease inferior rectus
Edema Periorbital fibrosis/contracture
Examination
Surgical emphysema
Nasal decongestant
Ocular motility e.g. Pseudoephedrine bd 3/7
Hyphema
Position
Globe Anterior segment Angle recession
Subconjunctival hemorrhage
Exclude
Corneoscleral laceration
Retinal tear, dialysis

Posterior segment
Vitreous hemorrhage
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Blunt or missile injury

Tripod fracture/malar fracture
Frontal sinus not Maxillary sinus
pneumatized Young General Anterior/posterolateral walls + floor orbit
children Zygomatic-maxillary
Prone to non- Zygomatic arch/zygomaticofrontal suture
complex fractures
displaced fracture 3 Parts Lateral orbital rim/wall
Soft tissue signs +/– Fourth suture
Superior subconjunctival hemorrhage Between sphenoid & zygomatic bones
Zygomatic Cosmetic deformity
Supratrochlear distribution hypoesthesia
Clinical (+ Tripod)
Clinical
Inferior axial globe displacement Coronoid process of mandible impinged
Orbital Roof Pain opening mouth
Intracranial injuries, CSF leak Mild displacement
Complications No treatment
Management
EOM entrapment – rare Open reduction & fixation
Separate teeth from upper face
Horizontal maxillary #
Type 1
Neurosurgical approach + Alveolar ridge/lateral nose/inferior wall maxillary sinus
orbital surgeon Neurosurgical Treatment
indications Base – teeth
10.15 Orbital Apex – nasofrontal suture
General Fracture Pyramidal #
Direct & indirect (blowout) fractures Type 2 Posterior alveolar ridge/lateral walls maxillary sinus/
Classification inferior orbital rim/nasal bones
Ecchymosis Le Fort
+ Edema eyelids Transverse # Craniofacial disjunction
Fractures Type 3
Nasofrontal suture/maxillofrontal suture/orbital wall/zygomatic arch
Limitation vertical movement of globe
Diplopia Orbital Pterygoid plate fractures
Pain inferior orbit on vertical movement Clinical All have
Floor
If horizontal affected Lacrimal bone =>
Frontal process of maxilla => Ethmoid bones
Nerve damage Enophthalmos & ptosis
Depressed bridge of nose
Hypoesthesia Direct trauma
Telecanthus
Infraorbital nerve distribution Increased distance from midline to medial canthus
Direct
Emphysema Cerebral & ocular damage
Orbit & eyelids Medial
Orbital Complications Anterior ethmoidal artery severance
Severe epistaxis
Orbital hematoma
CT scan, clinical photography
Investigations
Forced duction testing Extension of blowout fractures inferior orbital wall
Emphysema of eyelids/orbit
Indirect (blowout)
Associated with other facial fractures If medial rectus entrapped
Optic nerve damage Orbital Apex Surgery Explore from floor to medial wall
Approach
CSF leaks Medial orbitotomy through skin/caruncle
Clinical
Trauma
No surgery 1. Majority
Minimal diplopia/restriction
Orbital floor repair not enough

Diplopia
Strabismus
4. Delayed More useful than floor repair
surgery
Herniated muscle on CT + oculocardiac syndrome Presentation
(e.g. 6/12)
"Missing" muscle on CT or trapdoor fracture
Risk muscle strangulation Subperiosteal implant
Enophthalmos Merselene
2. Indications for
Minimal signs, no diplopia as eye closed
Repair (Within
24–72 h) Optic nerve damage
Inferior rectus in
trapdoor fracture 'White-eyed' blowout Globe
Plasticity of bone snaps
fracture in children Diplopia
back into place
Lower lid retraction
High rates oculocardiac reflex
Anterior structures Lacrimal pump damage
Prevent fibrosis, relieve Urgent surgery 10.16 Orbital
oculocardiac reflex Floor Surgery Lymphoedema
Enopthalmos
Observe 7–10 d Cosmetic Under/overcorrection
Swelling & hemorrhage subsides Surgical
Complications
Implant extrusion
Limitation vertical gaze within 30
decrees of primary position Infraorbital hypoesthesia
Diplopia Surgical
Infection
Positive duction test + CT evidence
3. Indications for
Delayed Repair Hemorrhage
Enophthalmos (e.g. 2/52)
>2 mm & cosmetic problem
Indications
Pupil check
1/2 of floor Check afferent and pupil dilation
Associated with medial wall # Intraoperative
Large medial wall fractures Large fractures Forced duction test

Assess muscle free prior to closure
Tripod fractures
Progressive infraorbital hypoesthesia
Prefibrosis of tissue planes

Repair within 2/52 of trauma
Infraciliary or lower eyelid crease incision
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Direct injury
Optic nerve from penetrating injury
Three Mechanisms Disruption of blood supply

Compartment syndrome
Force transmitted to optic nerve

Indirect injury
Apex & canal
Afferent pupil response

Diagnostic Steps
Not IOP
Orbital pressure
Proptosis
Resistance to retropulsion
10.17 Post-Traumatic
Features Diffuse subconjunctival hemorrhage
Vision Loss Tight orbit
with Clear Media Tight eyelids
Inability to distract eyelids from globe

Retrobulbar Hemorrhage &
Compartment Syndrome Urgent lateral canthotomy
Elevated orbital pressure
+ Inferior cantholysis
Management Relieves IOP also
Reticular pattern intraconal orbital fat

CT scan after relieving pressure
Optic nerve full stretch
Teardrop
Distorts globe into teardrop shape
Traumatic Optic Neuropathy See Neuro-ophthalmology

11 Uveitis and Inflammatory Eye Disease
Uveitis and Inflammatory Eye Disease
Granulomatous 11.20 ARN & PORN

11.1 Classification infective 11.21 Syphilis
11.22 Tuberculosis
11.3 Treatment
11.2 Management
Granulomatous 11.23 Sarcoid
11.4 Immunosuppressives
inflammatory 11.24 Vogt–Koyanagi–Harada Disease
11.19 Panuveitis
11.5 Masquerade Syndromes 11.25 Sympathetic Ophthalmia
11.7 HLA-B27 Non-granulomatous
Spondyloarthropathies infective Endophthalmitis – see below
– PAIR 11.6. Acute Anterior Uveitis
11.26 Toxoplasmosis
11.8 Herpetic Uveitic Disease 11.27 Toxocara
Non-granulomatous 11.28 Behçet's Disease
11.10 Juvenile Idiopathic Arthritis 11.9 Chronic inflammatory
Uveitis Lymphoma
11.11 Fuch's Heterochromic
11.29 Retinal Vasculitis
Iridocyclitis
11.12 Iris Heterochromia 11.31 Acute Postoperative

11.13 Intermediate Uveitis
11.30 Endophthalmitis 11.32 Chronic Postoperative
11.15. Systemic Vasculitides 11.33 Other Causes
11.16 Bacterial 11.34 Episcleritis

11.14 Posterior Uveitis
11.17 Viral Infective
11.18 Fungal 11.35 Scleritis 11.36 Wegener's Granulomatosis
& Relapsing Polychondritis
11.37 White Dot Syndromes – Retinochoroidopathies
11.38 White Dot Syndromes
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Anterior Keratouveitis
Iritis & iridocyclitis
Intermediate Vitreous
Pars planitis, posterior cyclitis, hyalitis (vitritis)
Fundus posterior to vitreous base
Posterior Choroiditis
Focal, multifocal, diffuse
Chorioretinitis
11.1 Uveitis Retinitis
Classification
Neuroretinitis
Panuveitis (Diffuse uveitis)
Endophthalmitis Intraocular tissues, no sclera
Panophthalmitis Entire globe, often orbital extension

P – Polyarteritis nodosa
W – Wegener's
V – Vasculitides PWRRS
R – Relapsing polychondritis
R – Rheumatoid arthritis
S – Systemic lupus erythrematous
S – Syphilis
T – Tuberculosis
STATE
I – Infective A – ARN – HSV/VZV/AIDS – HIV
T – Toxoplasmosis
E – Endophthalmitis
B –Behçet's
S – Sarcoidosis
I – Inflammatory BSVSMS
V – Vogt–Kayanagi–Harada syndrome
S – Sympathetic ophthalmia
M – Multiple sclerosis
S – SINS Surgical-induced necrotizing scleritis
P – Psoriatic arthritis
PAIR A – Ankylosing spondylitis
A – Arthropathies
2. Treat Cause – 1. Establish I – Inflammatory bowel disease
The Mission is Diagnosis VIISA M
Local R – Reiter's reactive arthritis
Remission
Systemic Non-neoplastic Ocular ischemic syndrome
M – Masquerade Chronic retinal detachment
11.2 Uveitis Lymphoma
Neoplastic
B – Band keratopathy Management Leukemia, retinoblastoma
E – ERM Idiopathic
BEN GO Anterior
N – Neovascularization uveitis HLAB27
blind 3. Manage PAIR
Retinal & choroidal JIA
G – Glaucoma Complications
O – Edema - CME Sarcoid, syphilis
blind – cataract V – Viral

VB
B – Bartonella
Neuroretinitis
Infective STATE
Malignant HTN, Leber's, IRVAN
HSV
Toxoplasmosis
Posner–Schlossman
Hypertensive Fuchs heterochromic iridocyclitis
uveitis
Phacolytic glaucoma, endogenous endophthalmitis
Other Any granulomatous uveitis
Involves TM
Masquerade syndromes
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Ocular side effects

Cataract, glaucoma
Mainstay of
treatment Peptic ulcer disease
Systemic side effects Raised BSL, osteoporosis, obesity
Cushings, mood instability, IIH

Local side effects
Prevent/break posterior synechiae Topical
Cycloplegia Cataract, glaucoma
Decrease pain from ciliary spasm
Orbital floor, roof, subtenon triamcinolone
Posterior effect required
Postoperative inflammation & CME Indications
Poor response or compliance
Chronic iridocyclitis Steroids Periocular Infectious uveitis

e.g. JIA Useful Contraindications Necrotizing scleritis
NSAIDs Risk perforation
Endogenous anterior uveitis Glaucoma patients
Less useful
11.3 Uveitis Ptosis, globe perforation, infection,
May take weeks to be active Side effects
General Treatment retrobulbar hemorrhage
Continue steroids
until active Uveitic CME
Indications
Vision-threatening intraocular inflammation Chronic posterior uveitis treatment
Immunomodulators Intraocular
Indications Retinal tear/detachment
Reversibility of disease process Side effects
Immunosuppressive Endophthalmitis, sterile endophthalmitis
Poor response medications
Oral or IV
Contraindicated – systemic Steroids
Systemic Vision threatening chronic uveitis
side effects or Indications
disease, e.g. DM, PUD Topical steroids are insufficient
e.g. VKH, Behçet syndrome,

sympathetic ophthalmia
CNVM Photodynamic therapy
Works well for inflammatory causes
Informed consent
Infection Treatment
Absent
Hematologic contraindications considerations
Close
Follow-up
Objective evaluation
of disease process
Do Full Blood Examination,
Liver Function Tests and Electrolytes
Prior to and during Folate analog, inhibits dihydrofolate reductase
Mode of action
Alters pyrimidine synthesis Inhibits DNA synthesis &
Indication mRNA synthesis
Methotrexate Sarcoid, JIA
Dose 7.5–25 mg weekly, with folate

Aggressive systemic disease, uveitis 6–8/52 for full effect
GI upset, hepatotoxicity
Side effects
Alefacept, efalizumab, anakinra
Mode of action DNA synthesis
Receptor antagonists Alters purine metabolism
Mode of action
Prevent cytokine action
Chronic disease, older patients
Indication
Azathioprine
Anti- Multifocal choroiditis, intermediate uveitis
Etanercept, infliximab, adalimumab (Immuran)
Metabolites Dose
Cytokine inhibitors
Neutralize cytokine TNF-alpha 100–250 mg daily oral
Mode of action Biologics Side effects
GI upset, hepatotoxicity
Rituximab, daclizumab
Mode of action Inhibits purine synthesis
Cell-specific antibodies
Bind glycoproteins Mode of action
Destroy whole lines Used when failed azathioprine
Interferon alpha-2a Indication
Not good for children
Other
IV immunoglobulin Mycophenolate Expensive – 15x > azathioprine
mofetil (Cellcept)
DNA cycle affected Dose Divided dose of 250–500 mg
Purine alkylating agent 11.4 Immunosuppressives 1–3 g daily PO
Lymphocyte cell Mode of action
<20% GI symptoms
division decreased Side effects Safest
Hepatic metabolism,
renal excretion Liver function, bone marrow suppression
Alkylating Less effective T-cells

Wegener's & PAN Inhibit IL-2 release
First line Agent Mode of action
Severe OCP Indication Hepatic metabolism, biliary excretion
Severe uveitis
Cyclophosphamide Behçet's disease
1–2 mg/kg/d oral (& chorambucil) Indication
Dose Vernal keratoconjunctivitis
Cyclosporin Topical CSA 1%
Monitor WCC => >3 Inhibitor of
(Neoral) Young patients
Cell Signaling
Dose
Sterility 2.5–5 mg/kg/d
Hemorrhagic cystitis Side effects Man with hairy teeth,
Bone marrow suppression BP has shot his kidneys, can't feel pain
Side effects Nephrotoxcity, hirsuitism,
Increased risk malignancy
Tacrolimus, gingival hyperplasia, hepatotoxicity
sirolimus
Parasthesias, seizures
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Lymphoma
Primary or metastatic melanoma

Conditions
Metastases
Retinoblastoma
Consider when Antibiotics

Present as anterior/posterior uveitis fails to respond to
Ophthalmic Anti-inflammatory
disorders
But not primarily inflammatory Systemic overview
Physician Neuroimaging
Definition Neoplastic
A differential diagnosis for uveitis
Lumbar puncture
Young – <18 y
Consider in Investigations Aqueous tap
Old – >50 y Ophthalmologist
11.5 Masquerade Vitreous biopsy
Syndromes
IOFB – Intraocular foreign body Cytokine analysis
Pathologist
IL10/IL6 vs IL10/IFN gamma ratios
JXG – juvenile xanthogranuloma Anterior
Fisson et al. (2013)
Intravitreal/intrathecal methotrexate
Retinal detachment
Treatment +/– Systemic steroids
Multiple sclerosis +/– Radiotherapy

Posterior
Spondyloarthropathies HLA-B27
PAIR diseases
Adolescent girls
Tubulointerstitial
Pain
Symptom nephritis & uveitis Idiopathic, constitutional symptoms
Renal disease
Redness Triad
Uveitis Anterior, bilateral, non-granulomatous
Photophobia Good steroid response

Whipple Intestinal lipodystrophy
disease Tropheryma whippeli Gm +ve infection
Bowel disease
Using 1x1 mm high Middle-aged men
magnification & illumination slit
Behçet syndrome
0 = <1 cell
Herpetic eye disease
0.5+ = 1–5 cells

Young–middle-aged males
1+ = 6–15 cells
Cells aka Glaucomatocyclitic crisis
2+ = 16–25 cells Discomfort, blurred vision, haloes
Mild unilateral iritis
3+ = 26–50 cells Fine white keratin precipitates
Secondary Posner– Corneal edema
4+ = >50 cells 11.6 Acute
Uveitis Hypertensive Schlossman
Anterior uveitis High IOP – 30–40 Minimal pain
0 = none Uveitis
Photophobia ++
1 = faint AC paracentesis for CMV PCR
2 = moderate, iris/lens details clear Fibrin
Topical steroids, cycloplegia, glaucoma therapy
3 = marked, iris/lens haze Signs
CMV => valganciclovir
4 = intense, fibrin/plastic aqueous If severe, progressive or bilateral glaucoma
Toxoplasmosis
Endophthalmitis/Microbial keratitis Fuch's heterochromic iridocyclitis
HLA-B27 diseases Phacoanaphylaxis

True uveitis
Behçet's Lens &
Without fibrin, is mobile
Hypopyon cataract related Phacolytic & lens particle glaucoma
Sarcoid Not true uveitis
Masquerade IOL related

ACIOL => uveitis glaucoma hyphema syndrome
Iatrogenic
Rifabutin Bisphosphonates
Drugs
Rifabutin
Young age of onset
Masquerade syndromes
When to
Systemic features
Investigate
Bilateral/Recurrent/Severe
Posterior uveitis
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308
Other ocular
features
Psoriatic
Arthritis Conjunctivitis
Uveitis in ~7% Marginal corneal infiltrates
Secondary Sjögren syndrome
95% are HLA-B27 positive
Lower back pain & stiffness after inactivity

Symptoms
Ankylosing Pulmonary apical fibrosis, aortitis
Spondylitis (5%) a/w aortic valve insufficiency
Sacroiliac x-rays Sclerosis & narrowing of joint space

Investigations
Ligamentous ossification
11.7 HLA-B27 NSAIDs treatment

Refer to physician
Spondyloarthropathies –
PAIR
HLA-B27 genotype located on 6p Inflammatory Ulcerative colitis
Uveitis 5–12%
Bowel Disease
Aqueous fibrinous exudate typical 1.4–8% of population, but Crohn's disease

~50% of iritis patients Uveitis 2.4%
General
P – Psoriatic arthritis aka Reiter's syndrome
CAN'T SEE, CAN'T PEE, CAN'T CLIMB UP A TREE
A – Ankylosing spondylitis
PAIR Young adult males 90% Preceding diarrhea/dysentry
I – Inflammatory bowel disease
R – Reactive arthritis (Reiter's) N – Nonspecific urethritis
Reactive Polyarthritis
Reiter was A – Arthritis Knees, ankles, feet, wrists
Arthritis
a NAZI Sacroilitis in 70% patients
Z – Zones of inflammation Circinate balanitis, keratoderma

blennorhagicum
I – Iritis
Treatment If urethritis present

Chlamydia – patient & sexual partners
Uveitis and Inflammatory Eye Disease 309
40% develop iritis

General
Acute mild nongranulomatous self-limited bilateral iritis/iridocyclitis
Nose tip involved Nasociliary nerve

Hutchinson sign Increase likelihood eye involved
Corneal dendrites, scarring denervation =>
Likely herpetic
Trabeculitis
50–60 mmHg
Varicella IOP
Zoster c.f. most inflammatory conditions => lower IOP
Clinical
Iris atrophy Sectoral/patchy
Acute retinal necrosis & progressive
Viral retinitis ARN and PORN outer retinal necrosis
Anterior segment ischemia, retinal artery occlusion

Vasculitis
Scleritis
11.8 Herpetic Multiple CN palsies
CN 3 most common, CN 4 least common
Uveitic Disease Neurotrophic cornea
Herpes
Uveitis, chronic
Simplex
Iris atrophy (less than zoster)
ARN (not PORN)
Dilate Posterior ARN

Within 72 h of VZV rash
Acute Oral antivirals
Topical steroids
Management Treatment
Topical antihypertensives
HSV – delay steroids until no epithelial disease
IOP & uveitis checks

Long-term 6/52 from onset of rash
Treat neurotrophic cornea

Inflammation >3/12
Anterior/posterior, non-granulomatous
Juvenile idiopathic arthritis
Non-granulomatous
Fuch's heterochromic iridocyclitis
Syphilis
Classification Infective Tuberculosis

Leprosy
Toxoplasmosis
Granulomatous
Sarcoidosis
Vogt–Koyanagi–Harada syndrome
Non-infective
11.9 Chronic
Uveitis
External Phacolytic glaucoma
Eye white/pink
Band keratopathy
Cornea
Keratic precipitates
Mutton fat, resolved mutton fat, or non-granulomatous
Anterior chamber
Aqueous cells, flare
KOEPPE
At "Korner" of iris
Signs
Iris nodules BUSACCA
Mid iris
BERLIN
Iris angle
Lens
Posterior subcapsular cataract
Vitreous spillover
Fundus
CME, ERM, neovascularization
Children <5 y
Still disease
No correlation between joint & ocular inflammation Systemic Rash, fever, lymphadenopathy, hepatosplenomegaly
Uveitis rare
Mild–moderate pain, photophobia, blurring
Often asymptomatic Three Types
Clinical Polyarticular onset
White, uninflamed >4 joints within first 6/52 of disease
Fine keratin precipitates, band keratopathy, flare, cells <5 joints

Eye Pauciarticular onset
11.10 Juvenile Uveitis commoner
Posterior synechiae, cataract Idiopathic

Arthritis
Topical steroids
Methotrexate
Age onset <2 y Treatment High complication risk
Cataracts
Female Uveitis
Risk Factors Control inflammation 3/12 preoperatively
Pauciarticular <5 joints Glaucoma
ANA+ As it is asymptomatic
initially
Prognosis Significant ocular damage
=> Screen based on risk factors
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Cataract => decreased VA
Symptoms Eye color
Vitreous floaters
White eye
Heterochromia iridis
Diffuse iris stromal atrophy
Lighter eye
Clinical
White stellate KPs
Entire endothelium
Signs
Unilateral Inflammation
95% General Hypertensive uveitis
Associated with rubella virus Iris nodules

Koeppe, Busacca
11.11 Fuchs Secondary BEN GO blind

But no CME
Heterochromic
Iridocyclitis Gonioscopy
Fine radial vessels in angle
Keratic precipitate diffuse distribution

Good prognosis
No posterior synechiae
Lessen inflammation
Steroids
Classic Findings Does not resolve fully
White eye
Heterochromia Control inflammation

1/52 steroids preoperatively
No cystoid macular edema Filiform hemorrhages on AC paracentesis

Treatment Amsler sign
Cataract 180 degrees to entry point
Scleral tunnel surgery
Poor dilation
Vitreous floaters become more evident

Future vitrectomy
Topical treatment usually sufficient
Glaucoma
Congenital Horner's
Hypochromia
Waardenburg syndrome
Congenital
Oculodermal/ocular melanosis
Heterochromia
Sector iris pigment epithelium hamartoma
11.12 Iris
Heterochromia
Uveitis
Fuch's, Posner–Schlossman, HSV/VZV, leprosy
Hypochromia Glaucoma PXF, pigment dispersion syndrome,

Post angle closure glaucoma
Acquired Juvenile xanthogranuloma
Uveitis – Fuch's
Heterochromia Pigment dispersion glaucoma
Iridocorneal endothelial syndrome

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Vitreous Main Site of Inflammation
70% idiopathic
Multiple sclerosis
Causes
Inflammatory
Sarcoidosis
Infections
Syphilis, TB, Lyme disease
Anterior uveitis
Vitritis Peripheral periphlebitis &

11.13 Intermediate
perivascular sheathing
Uveitis
Posterior Especially multiple sclerosis
segment
Gray–white fibrovascular plaque
Snowbanking
Signs
Fuch's heterochromic Neovascularization can occur in snowbank
Severe vitreous involvement
iridocyclitis
CME 30% of patients
Unilateral anterior segment disease though Complications BEN GO blind
ERM, glaucoma, cataract
No snowballs or CME Primary intraocular Differential
lymphoma Diagnoses
Snowbanking => must treat
Neovascularization risk
Peripheral Toxocara
granuloma 1st
Unilateral Topical steroids
Orbital floor methylprednisolone + dexamethasone
Other
Amyloidosis, Whipple disease
Steroids 2nd OR subtenon triamcinolone
Treatment
Repeat injections 6–8/52
3rd
If fails 3 injections => systemic steroids
Retisert
Intravitreal steroid implant
Other
Areas of snowbanking
Trans-scleral cryotherapy
After failed steroids
Retinochoroidopathies
aka White dot syndromes
Inflammation of
Systemic lupus erythematosus Retina or Choroid +/– Overlying vitreous
Inflammatory
Polyarteritis nodosa
Wegener granulomatosis
PAIN ++ => endophthalmitis/posterior scleritis
Bartonella – cat-scratch disease
Symptoms Decreased visual acuity
Lyme disease
Bacterial Clinical Floaters, metamorphopsia, scotomata
Leptospirosis Others
Leprosy Retina primary
HIV/AIDS Diffuse, poorly demarcated

Retinochoroiditis
CMV 11.14 Posterior Focal or multifocal
Viral Retinitis
Uveitis
Rubella Discrete or diffuse
Subacute sclerosing Measles
panencephalitis virus (SSPE) Choroid primary
Infective
Chorioretinitis Lobules of choroidal blood
Presumed ocular histoplasmosis syndrome Well demarcated
Causes
Pneumocystis
Candidiasis Fungal
Cryptococcus
Methylprednisolone +
Aspergillosis dexamethasone + lignocaine
Orbital floor Transcutaneous, lateral

Protozoan Local steroids
Toxoplasmosis Treat cause limbus in primary gaze
Posterior subtenons
Helminth (worm) Treatment triamcinolone
Toxocara
Large cell lymphoma
Malignant melanoma
Masquerade
Retinitis pigmentosa BEN GO blind
syndrome Treat complications
Intraocular foreign body Other
Retinoblastoma
Leukemia
Ocular ischemia
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316
4th–5th decade, small hepatitis B link
Multisystem, Immune complex deposition

General necrotizing vasculitis
Small & medium muscular arteries
Serious, may be fatal
Fever, weight loss, malaise 50%

Renal failure & hypertension
Lungs/Kidneys 60%
Idiopathic, multisystem small vessel Skin
predominantly
necrotizing granulomatous vasculitis Erythema nodosum, Raynaud's phenomenon
General Systemic
Polyarteritis Cardiac 43%
Mean age 40s
AMI, CCF, pericarditis
Nodosa
CNS
CVA, seizure
ENT 95% patients
Sinus pain, purulent/bloody discharge Abdominal pain, bowel infarction & perforation
GI
Multifocal nodular lesions, Respiratory signs 45%
Ocular Retinal microinfarcts + choroidal infarcts
infiltrates, cavitation
Peripheral ulcerative keratitis, scleritis
Necrotizing glomerulonephritis Systemic
Renal 18%
11.15 Posterior p-ANCA
=> Renal failure Diagnosis Sensitivity 15%
Uveitis – Systemic
Papules, subcutaneous nodules Vasculitides Biopsy Characteristic vasculitis
Skin 46%
Proptosis, orbital Treatment
Corticosteroids + cyclophosphamide
congestion Contiguous spread
Mass in orbit from paranasal Poor 10–20%
Ophthalmoplegia Prognosis 5y
sinuses or nasopharynx Wegener's Autoimmune – ANA &
Dacryoadenitis & NLDO ANCA-Associated Collagen vascular disorder immune complexes
Ocular Granulomatous General
Anterior necrotizing scleritis
Vasculitis 90% are female, child-bearing age
Keratitis, episcleral vasculitis
Malaise, fever, arthritis, rash, pleurisy, oral ulcers
Retina
Occlusive periarteritis Systemic Renal disease & hypertension
Active disease
90% sensitive c-ANCA Cerebral vasculitis
Neurological complications
Inactive disease Diagnosis Systemic Lupus
60% sensitive Erythematosus
CWS
Pulmonary/Upper airway tissue Retina Ischemia => NV =>
Biopsy Arteriolitis & vascular occlusion
Gold standard vitreous hemorrhage
Rheumatologist + ENT Ocular
Choroid Multifocal choroiditis, choroidal infarction
High dose steroids Treatment Exudation, CNVM
+ Immunosuppression PUK, scleritis
Cyclophosphamide 2 mg/kg/day
No treatment Diagnosis
Fatal ANA, ds-ANA
Prognosis Corticosteroids + immunosuppression
Remission in 75% Physician
50% relapse With treatment Treatment
Renal monitoring
2/52 later
Cat scratch
Pathology Lymphadenopathy, fever, malaise
VA impaired
Neuroretinitis Papillitis
+/– Peripapillary hemorrhages/venous engorgement
Clinical Macular star of hard exudates

Bartonella henselae + Macular edema
Cat-Scratch Disease
Conjunctival inflammation
Parinaud oculoglandular syndrome 7%
Preauricular adenopathy
Doxycycline 100 mg bd
Treatment
Or ciprofloxacin 750 mg bd
Ticks => humans

Pathology
11.16 Posterior Rodents, deer, birds, cats, dogs
Uveitis – Bacterial Animal reservoir
Bull's-eye skin lesions

Enlarge in all directions – erythema migrans
Systemic
Low-grade inflammation Facial palsies
Mycobacterium
leprae Clinical Polyarticular migratory arthritis
Iris pearls from dead bacteria Leprosy
Borrelia burgdorferi Inflammation of all ocular structures
Pathognomonic
Lyme Disease Ocular Separate or together
Miotic pupil, atrophic iris
Chronic iridocyclitis & vitritis
History of tick bites

Investigations
Western blot
Serum antibody titers
Doxycycline 100 mg bd 3/52

Treatment
Children/Pregnant women
Amoxicillin, clarithromycin
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318
Congenital CMV
Multifocal areas of brushfire retinitis Indicated declining CD4+ count
HIV microangiopathy
Associated with immunocompromised status CWS, retinal hemorrhages
Primary
Peripheral granular opacification
Indolent Anterior uveitis & vitritis
No retinitis, slow progression HIV retinitis
Gray-white-yellow lesions on fundus
Confluent necrotic areas & hemorrhage
CMV
Spreads along vascular arcades Fulminating Most common
CMV retinitis
brushfire retinitis
Viral HZO, PORN
Clumping at active parts CMV HIV/AIDS
Acquired
Trail of RPE atrophy Molloscum contagiosum
CMV
Secondary
opportunistic
Necrotic retinitis => Cryptococcosis
infections Fungi
Retinal detachment
Candidiasis
HAART – highly active anti-retroviral therapy
Systemic Protozoan
IV ganciclovir Toxoplasma
Bone marrow suppression
Management 11.17 Posterior
Then oral valganciclovir Kaposi sarcoma
Uveitis – Viral
Ganciclovir implant – last 8 mo
Cataract Intravitreal
Single-strand RNA virus
Rubella/German measles
Contagious RNA virus Retinitis
Congenital
1st trimester of pregnancy
Congenital
Similar to rubella Cataract, glaucoma
Macular edema
Rubella Strabismus, extreme refractive error
Neuroretinitis & macular star Acquired Ocular

Measles Anterior inflammatory signs, iris atrophy
Attenuated vessels Vitreous haze

Posterior
Maculopathy & encephalitis Salt & pepper fundus – does not affect vision
SSPE – subacute sclerosing
Progressively fatal panencephalitis virus Systemic
Deafness, cardiac defects, mental retardation
Poor visual prognosis
Immunocompromised, indwelling
catheters, chronic antibiotic use
Risk factors
Candida albicans & other spp.
Abdominal surgery
Immunosuppression
Decreased VA, floaters
String of pearls
Clinical Mild–moderate intraocular inflammation
Candidiasis Yellow–white choroidal lesions

Single/multiple
Systemic infection workup
Investigations
Pneumocystis carinii Tap & inject +/– vitrectomy
Associated with AIDS

Infectious disease physician IV fluconazole
Vision good
Pneumocystis Poor penetration by amphotericin B
Choroidal Treatment
Posterior pole
pneumocystosis
Flat yellow round lesions Pars plana vitrectomy
Intraocular cultures
No vitritis Intravitreal amphotericin B
11.18 Posterior Aspergillus spp. Immunocompromised (e.g. post-

Presumed ocular histoplasmosis syndrome Uveitis – Fungal transplant) & IVDU
Inhaled Acute pain & vision loss

Histoplasma capsulatum
Immune mediated response, bilateral Aspergillosis Clinical Severe inflammation
Larger than Candida
POHS Chorioretinal lesions
Histoplasmosis Rapidly progressive
Round yellow–white/pigmented lesions
spots
Mid-periphery Presentation Diagnostic culture
Peripapillary atrophy Pars plana vitrectomy
Treatment
Intravitreal amphotericin B
Exudative maculopathy
Treatment of CNVM Cryptococcus Yeast in pigeon droppings

PDT – Photodynamic therapy neoformans
Immunocompromised/AIDS patients
Papilloedema, ophthalmoplegia, optic neuropathy

Meningitis
Cryptococcus CN 6 palsy
Clinical
Iris inflammation, keratitis
Multifocal choroiditis
Intravitreal amphotericin
Treatment
Oral fluconazole
319
Severe Diffuse Inflammation Anterior & posterior segments

Often bilateral
Infective
Syphilis, TB, ARN (& PORN)
Granulomatous
Inflammatory
Sarcoid, VKH, sympathetic
Causes
Infective Toxoplasmosis, endophthalmitis, Toxocara
Non-granulomatous Inflammatory
Behçet's
11.19 Panuveitis Masquerade
Lymphoma
Infective STATE Syphilis, TB, ARN, Toxoplasmosis,

Endophthalmitis (Endo/exogenous)
Inflammatory BSVSMS
Bechet's, Sarcoid, VKH, Sympathetic, MS
Surgical Sieve
Masquerade
Lymphoma
Phacoanaphylactic uveitis
Other
Leprosy, Lyme, Candida
Emergency
Retinal necrosis progresses fast
Do at time of tap
Intravitreal foscarnet 2.4 mg/0.1 mL
1. Local antivirals
Decreases retinal detachment rates
Then 3/12 acyclovir/valaciclovir oral

IV acyclovir 10 mg/kg tds for 2/52
Renal function
Treats infected eye
2. Systemic
Benefits
antivirals Decreases rate of bilateral ARN by 50%
Fulminant, necrotizing Acute retinal necrosis
viral retinal infection Palay et al. (1991)
GI upset, rash, CNS toxicity
Side effects
Bilateral Renal failure
Treatment
33% develop BARN General Contraindication

HSV, VZV, EBV, CMV 3. Prophylactic Once inflammation controlled
Associated with barrier laser
Patient not immunosuppressed At border of damaged/healthy retina
Decreases retinal detachment rates
1 mg/kg/d oral + topical +
Vision loss, pain
11.20 Granulomatous homatropine
Panuveitis – 4. Steroids Debated
Anterior granulomatous uveitis & vitritis ARN & PORN May introduce 48 h postantiviral
Retinal vasculitis Debated For occlusive arteritis

Occlusive peripheral periarteritis 5. Antithrombosis Aspirin 100 mg daily
4 Signs Clinical
Relieve traction, prevent detachment
Multifocal, deep yellow–white Retinitis Debated
6. Retinal surgery
peripheral infiltrates If detachment
Use silicon oil
Rapid circumferential spread
Poor
Infiltrates retina in periphery Prognosis Most VA <6/60
+/– Scleritis, optic nerve involvement
Patient must monitor other side
Outer retina =>

Progressive outer Necrotizing VZV retinitis
PCR retinal necrosis Rapidly full thickness retinal necrosis
HSV, VZV, EBV, CMV AC & vitreous tap
Immunocompromised patients
HIV, syphilis serology Investigations
Renal function with Advanced AIDS
acyclovir Posterior pole
Blood electrolytes Involved early
Build up => CNS toxicity PORN
Clinical Vitreous inflammatory cells
Absent
Vasculitis
Minimal
Early macula involvement

Prognosis Poor
Rapid progression
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Tests current infection

VDRL & RPR Monitor response to treatment
Can take CSF sample
Positive serology
More sensitive & specific
Diagnosis FTA-ABS Once positive – does not normalize
Cannot use to monitor treatment
CSF sample invalid
Lumbar puncture
Active uveitis, neurological/neuro-ophthalmic signs
Consider HIV & Chlamydia testing

Treponema pallidum
5–10% secondary syphilis develop uveitis General IV penicillin G 2–5 million units QID 2/52
Great masquerader Neurosyphilis Then IM penicillin 2.4 million units weekly for 3/52
Consider in all uveitis Penicillin allergy
IV ceftriaxone, tetracycline
11.21 Granulomatous IM penicillin
Panuveitis – Ocular syphilis, normal CSF
Age 5–25 y Retinal & optic nerve involvement
Syphilis
Bilateral interstitial keratitis Debated as “neurosyphilis”
3/12 VDRL titers
Treatment Follow-up
Blood vessels invade sclera 6/12 lumbar punctures
Secondary cataracts Clinical
Salt & pepper fundus Congenital Single dose 1 g azithromycin oral
Chorioretinitis, iridocyclitis Chlamydia
Ulcerated, painless ulcer + regional lymphadenopathy

K – interstitial KERATITIS Chancre
Primary
Hutchinson's triad – KID Can be on eyelid/conjunctiva
I – widely spaced INCISORS
6/52–6/12 postprimary infection
D – DEAFNESS
Rash palms & soles, fever, weight loss, arthralgia, meningitis
Systemic
Secondary
Acquired
Iritis symptoms
Ocular
Iridocyclitis, chorioretinitis, periphlebitis, neuroretinitis
Imitator
Patchy iris hyperemia, fleshy pink nodules

Gumma of iris
Ocular
Tertiary Near iris sphincter
Argyll–Robertson pupil
Constricted
Primary When eye primary portal of entry
ocular TB
Conjunctival, corneal, scleral disease
Hematogenous or contiguous spread

Uveitis
Granulomatous uveitis
With posterior disease
Scleritis, phylctenulosis, interstitial keratitis

Anterior
Corneal infiltrates, nodules – iris, anterior
Multiple, discrete, yellow lesions,

Disseminated 5–100 tubercles
choroiditis
High affinity for most common
+/– Disc edema, NFL hemorrhage, vitritis,
oxygenated tissues Mycobacterium anterior acute uveitis

Lung & choroid tuberculosis
General Secondary Single, focal, large choroidal mass
Posterior
ocular TB
Health care, immigrants,
Tuberculoma +/– Neurosensory retinal detachment
immunosuppressed Clinical
At-risk populations Macula star
Indigenous
Associated with disseminated disease
Multifocal choroiditis
Serpiginous-like choroiditis
Uveitis, poor steroid response,
Subretinal abscess, CNV, optic neuritis, panophthalmitis
positive contact history, skin test
Presumed Peripheral retinal
perivasculitis Unilateral retinal/vitreous hemorrhage
11.22 Granulomatous
Chest x-ray, blood cultures, AC tap
Panuveitis – Subsequent involvement other eye
In immunocompetent Eales TB & disease
Tuberculosis
people Venous occlusion, peripheral non-perfusion
Distinguish latent Neovascularization
Immunosuppressed will Periphlebitis
from active TB
not mount skin response Mantoux test Tractional retinal detachment
Diagnosis
Positive if previous BCG vaccine Hyperfluorescence with late leakage
Previous BCG vaccine FFA
Cicatricial lesions
Can test in Early blocked fluorescence, late staining
Latent TB
Quantiferon gold
Immunosuppressed Early & later hypofluorescence
ICG
Choroidal lesions
Cannot distinguish latent from active
Rifampicin
Isoniazid
Treatment RIPE
Pyrazinamide
Ethambutol
Avoid steroids
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Small choroidal lesion

TB, multifocal choroiditis, birdshot
Multisystem noncaseating epithelioid granulomatous disease
Differential Diagnoses Large choroidal infiltrates Metastatic tumor, PIOL,
Idiopathic
General serpiginous,VKH
African-American > Caucasian
Periphlebitis
Commonly age 20–50 y TB, Behçet's, CMV, Eale disease
Erythema nodosum
Skin
Serum ACE (angiotensin-converting enzyme)
Bloods
Constitutional symptoms Serum & urinary calcium
Myocardium, liver, neurosarcoidosis (CN 7 commonly)
Systemic Chest x-ray
Fever Hilar lymphadenopathy
Parotid enlargement
Heerfordt syndrome Diagnosis
CN 7 palsy – lower motor neuron CT chest or MRI nodules
Anterior uveitis
Panda sign Parotid, submandibular, lacrimal
gland involvement
Orbital & eyelid granulomas Imaging
11.23 Granulomatous Whole body
Palpebral & bulbar conjunctival nodules Panuveitis – Gallium scan Perihilar & peritracheal
Cutaneous Lambda sign
Sarcoid rash near eyelid margin Sarcoid lymph nodes
Lupus pernio
Red-purple indurated plaques Bronchoalveolar lavage, transbronchial lung biopsy
Mutton fat KPs Biopsy Accessible lesions
Lacrimal gland, skin, conjunctiva
Koeppe & Busacca iris nodules
Anterior segment Less accessible lesions
Nummular corneal infiltrates Lung, lymph node
Posterior & anterior synechiae Vitrectomy
Ocular To exclude masquerade
Vitreous
Snowballs
Periphlebitis – perivenous sheathing
Candle wax drippings in severe cases Retina
Posterior Steroids
Granulomas segment Topical, periocular, systemic
Management
Choroid Cycloplegia
Comfort, prevent synechiae
Sarcoid spots – multiple punched out
yellow lesions inferiorly Systemic immunosuppression
Methotrexate, azathioprine
Focal granulomas
CNS involvement Optic nerve

Or secondary to retina/vitreous Disc edema
involvement
CNS
Encephalopathy, meningeal irritation, CSF pleocytosis
Rare, systemic granulomatous disease
Bilateral panophthalmitis Systemic Triads Auditory
Vertigo, tinnitus, deafness
Pathology
Uveal melanin-associated protein autoimmune reaction
Skin Alopecia, poliosis, vitiligo
Dark-skinned individuals
More commonly affected
Must exclude sympathetic ophthalmia

Check trauma history

Flu-like symptoms
Prodromal
CNS symptoms 11.24 Granulomatous Fundus fluorescein angiogram
Fever, headache, neck stiffness, LOC, seizures Panuveitis – Multifocal subretinal leakage, early phase
1–2/7 postprodromal phase
Vogt–Koyanagi–
Harada Disease Choroidal thickening, serous retinal
B-scan
Photophobia, redness, low VA, ocular pain
Acute – uveitic phase detachment, vitreous opacities
Non then granulomatous KPs Lymphocytosis

Bilateral AC reaction Cerebrospinal fluid
+/– Vitreous reaction 80% by 1/52, 97% by 3/52
Investigations
Detachment of retina HLA-DR4
Multifocal choroiditis, exudative detachment Clinical Strong association
Disc swelling and hyperemia Triad of Ds

OCT scan
Subretinal fluid
Dalen–Fuchs nodules
Peripheral discrete nodules
Peripapillary atrophy Local, periocular
Pigment epithelial atrophy Steroids

Triad of Ps Systemic Prednisolone 50mg 6/12 before
Sunset glow fundus – retinal detachment resolution Treatment switching to steroid-sparing
Steroids more
Poliosis & skin changes effective
Immunosuppression
Recurrent granulomatous uveitis phase Cyclosporine
Uveitis complications Bevacizumab

Cataract, glaucoma, CNVM Secondary CNVM
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Ocular appearance identical to VKH
Bilateral
Worse in exciting eye initially
Non-necrotizing
Chronic, recurrent
Similar to VKH Thickening of the iris from

Autoimmune reaction to uveal antigen released
lymphocytic infiltration
50% – 90% within 1 y latent period Surgery/trauma Mutton–fat keratin precipitates
Anterior
Can occur to one eye Posterior synechiae
Then uveitis to uninvolved Pathology Clinical
anytime
other "sympathizing" eye Granulomatous Raised – due to trabeculitis
uveitis IOP
Trauma Hypotony – due to ciliary body shutdown
0.1 to 0.5%
Incidence
Vitrectomy Surgery 11.25 Granulomatous Vitritis
0.1% Panuveitis – Dalen–Fuchs nodules
Sympathetic Posterior
Ophthalmia Peripapillary choroidal lesions
VKH Exudative retinal detachment
No trauma, systemic signs
Sarcoid Band keratopathy, ERM,

Differential
Diagnoses BEN GO neovascularization
Syphilis
blind Glaucoma, cystoid macular
Complications edema
Phacoanaphylactic endophthalmitis
Unilateral
Cataract
Local, periocular
Steroids
Management
Systemic 60–80 mg oral
3–6/12
Immunosuppression
Antimetabolites, cyclosporine
Small or peripheral lesions
No treatment if =>
Observe 3/52 to 6/12 until
symptoms resolve
Indications Lesion involves disc, macula, papillomacular bundle

to treat
Severe vitritis
Risk vitreous fibrosis & tractional retinal detachment
Obligate intracellular parasitic protozoon
Immunocompromised patients
Toxoplasma
Human food sources Cats – intestinal parasite => gondii
oocysts in feces Sulfamethoxazole 800 mg + One tablet, twice daily 4/52

trimethoprim 160 mg
Infection from undercooked meat with tissue cysts General

If sulfa allergy
Antibiotics
Risk acquiring Toxoplasma
1–2% Or clindamycin 300 mg Risk pseduomembranous colitis
Treatment QID oral
40% Pregnancy Off-label use
Passing onto
fetus though Intravitreal clindamycin
CNS, ocular & systemic complications If patient pregnant
Treating vitritis
11.26 Non- Start after antibiotics
Most common necrotizing retinochoroiditis & posterior uveitis cause granulomatous Steroids
Panuveitis – Prednisolone 50 mg oral
Unilateral Wean over 6/52
Symptoms Toxoplasmosis
Floaters, visual loss, photophobia CNS imaging
High association of CNS disease
Anterior uveitis spillover

Immuno- Vitritis less prominent
=> May need vitrectomy for PCR for diagnosis
compromised
Solitary inflammatory focus
Exudative focal retinitis Pathognomonic Clinical
Treat since higher risk of transmission Atovaquone – less
Near old pigmented scar Signs immunosuppression
Multiple foci uncommon

Consider maintenance therapy
Poor fundus visibility, lesion visible Bactrim one tablet daily or BD
Vitritis
Headlight in fog appearance
Clinical diagnosis
Investigations
Serum ELISA antibodies may be used
327
328
Ingestion of egg
Mature into larvae in intestine

Nematode protozoon
General Hematogenous dissemination of larvae into choroid & retina
Toxocara canis
Vitreoretinal immune reaction to larvae
Intestinal parasite in dogs
Young adult or child
Children – leucocoria
Ocular Signs Severe uveitis

Pars plana snowbank
Posterior segment granuloma
Tractional membranes may form
11.27 Nongranulomatous Nematode death =>
Panuveitis – Toxocara Worsens inflammation
Diagnosis Clinical
ELISA titer
90% sensitivity & specificity
Steroids Periocular + s ystemic

Treatment
Antihelminths
Not effective
Vitreoretinal surgery
Relieve tractional retinal detachment
Location
Prognosis
Factors
Resulting scar
Macular traction
Acute iritis with hypopyon
Idiopathic generalized occlusive vasculitis, a/w HLA-B51
General Triad Aphthous stomatitis
Male = female, young Painful, present in 98–100%
But males have worse disease
Genital ulceration
Silk route people Diagnosis
Genital ulceration
Recurrent AAU +/– hypopyon – and white eye Recurrent oral Eye lesions
ulceration + 2 of Sterile needle puncture,
Bilateral Criteria Skin lesions
Anterior develops pustule
Hypopyon Pathergy test
No fibrin => shifts on head movement 80% sensitivity/specificity

Ocular Features
Fulminant uveitis
Bilateral
blindness 3 y Retinal vascular occlusion
Occlusive arteritis & periphlebitis Natural history Occipital lobe
Primary retinal vasculitis
Vascular leakage 11.28 Behçet's Disease Neuro-Behçet's => infarct
Macular, retinal, disc edema Optic

Death neuropathy
Posterior CNS involvement
White, superficial lesions
Necrotic retinal infiltrates Rheumatologist review for ALL
Acute phase, resolve without scarring
Treatment
Oral prednisolone
Ischemic optic neuropathy 1–2 mg/kg + gastric/bone protection
Disc edema, vascular attenuation Effective, rapid acting

Burnt out Cyclosporine
Risk nephrotoxicity
Possible peripheral laser for ischemic neuropathy
Azathioprine
Effective but slower
Early immunosuppression
Severe disease only
Erythema nodosum Cyclophosphamide
Legs, ankles, elsewhere Skin Risk sterility
Non–destructive arthritis/arthralgia
Wrists & ankles Musculoskeletal Chlorambucil, infliximab
Systemic Features
GI
Ulcerative hemorrhages
Life-threatening CNS
Neuro-Behçet's
Strokes, palsies, confusional states
329
330
M>F, 30–50 y
Primary idiopathic
vasculitis Common in young Asian males
Rare in Caucasians
Unilateral => bilateral
Associated with TB
No anterior segment inflammation

Perivascular infiltrates
Obliterative periphlebitis
Eales Disease involving periphery Sheathing of retinal vessels
Clinical
Hemorrhage and edema
Junction of perfused/
nonperfused retina
Neovascularization
=> Vitreous hemorrhage
Treatment
PRP or feeder vessel photocoagulation
Behçet's Prognosis
11.29 Retinal Good visual prognosis
Collagen vascular disorders Vasculitis
Inflammatory
SLE, Wegener's,
polyarteritis nodosa Multiple sclerosis
GCA
Birdshot Retinal Vasculitis
Causes Non–occlusive ARN
Syphilis, Toxoplasmosis, ARN causes,
STATE Birdshot
Tuberculosis, Endophthalmitis Infective Arteritis
Behçet's
Lyme, cat-scratch disease
Secondary Occlusive CMV retinitis
Vasculitides
Eales Collagen vascular disorders
Idiopathic Causes PAN, SLE, Wegener's
Occlusive disease
Sarcoidosis
TB, Lyme
Periphlebitis Non–occlusive
(veins) Birdshot
Eales disease
Occlusive Syphilis, CMV retinitis, TB
Behçet's
Vitreous cavity & anterior chamber
Intraocular
+/– Retina/Choroid inflammation
Definition 1/1000 cataract
Infectious or sterile Surgical

Acute postoperative 1/2000 vitrectomy
incision
1/2500 intravitreal injection

Ocular emergency
Chronic postoperative uveitis
Exogenous
Post-traumatic 2.4–8%, higher if
Traumatic laceration
retained foreign body

Infectious Filtering blebs
Diagnosis until proven otherwise 0.2–9.6%
Infectious
Pain, low vision, AC fibrin, vitritis Intravitreal injection

0.8%
VIEW Study 2011
Retained lens material Bacterial
Severe granulomatous reaction
Endogenous
11.30 Endophthalmitis Candida
Severe sterile
IVDU, immunocomprimised
Aseptic
Not painful, or significant vision loss
Toxic Anterior Segment Syndrome

Differential
Following uneventful surgery Diagnoses
Acute sterile inflammation
Rapidly develops 12–24 h TASS
AC cell + flare +/– fibrin +/– hypopyon

White eye
Limbus–limbus corneal edema Pain
Admit Signs & Symptoms Vision

Decreased
Treat as endophthalmitis
Monitor response If any doubt AC reaction + hypopyon
2x/daily initially
Vitritis
TASS responds Frequent steroids Poor red reflex
quickly
331
Vitreous tap 0.2 mL, 27 or 25G needle
332
Tap
S. epidermidis 70%
AC tap 0.05–0.1 mL, 30G needle
Organisms
Common Vancomycin 1.0 mg/0.1 mL
S. aureus, Strep species (not pneumococcus)
Inject
Ceftazidime 2.25 mg/0.1 mL
Gram negative
Less common Admit Dexamethasone 4 mg/0.1 mL
Pseudomonas, proteus, E.coli, H. influenzae Limit enzymatic retinal destruction by PMNs
Medical Steroids 1–2 hourly

Atropine 1% TDS
Topical
Age, concurrent operations Treatment Chloramphenicol QID
PC tear & vitreous loss Risk factors Or ofloxacin 1/24
If focus of infection – bleb, suture, wound leak
Lid & lacrimal disease
No systemic antibiotics required
Treat before operation EVS 1995
Re-inject if no improvement Metrics – pain, inflammation,

No improvement 48 h
hypopyon smaller
Develops 1–14 d postoperative Initial VA light perception or worse
Clinical Vitrectomy
11.31 Exogenous fungal endophthalmitis
Present 7–14 d – postoperative
Mild Endophthalmitis - Surgical
Acute Postoperative 1. Capsulectomy & pars plana vitrectomy
VA >6/60 (S. epidermidis) Propionibacterium acnes
2. Removal of lens if no improvement
Endophthalmitis Vitrectomy Study

Presents 1–4 d postoperative
Often Strep species Severe Systemic antibiotics no use

VA <6/60 Conclusions
Or Gram negative – Serratia, Initial VA light perception or worse Early vitrectomy reduced
EVS 1995
Pseudomonas, Proteus severe vision loss by 50%
At 3/12
Progressive vitritis 41% VA >6/12
VA prognosis
Diagnosis At 9–12/12
If doubt => treat as infective
53% VA >6/12
Confirms vitritis
Studies Ciulla et al.
TASS has no vitritis B-scan if poor posterior view Betadine
(2002)
Decreases endophthalmitis rates
Baseline for success of therapy
ESCRS Endophthalmitis Study
Clear corneal incisions
5x increased risk c.f. scleral tunnel
Intracameral cefuroxime 1 mg/0.1 mL, 5x increased risk if did not use this
ESCRS 2006 normal saline intracameral
Findings
Surgical complication (e.g. PC tear)
5x increased risk
Silicone lenses
4x increased risk
>4/52 Postcataract surgery
Proprionibacterium acnes common General Clark et al. (1999)

Gram positive, sensitive to vancomycin
1st line for central,
Fungi – Candida small PCO
Recurrence rate 14%
Intravitreal amphotericin at time of vitrectomy Vitrectomy + partial
capsulectomy + intravitreal
vancomycin c.f. 50% for vitrectomy alone
Recurrent cyclical granulomatous Treatment c.f. 100% for tap & inject alone
AC activity Recurrence rate 0%

Transient steroid response
Presentation
Vitrectomy + total Recurrence after partial capsulectomy
+/– Hypopyon capsulectomy Indications
+ intravitreal injections +
Capsular plaque extensive or peripheral
removal of IOL
Posterior capsular opacification
Disadvantage
11.32 Endophthalmitis – ACIOL or iris fixed lens required
Chronic
Postoperative
Poor steroid compliance
General Retained lens material
AC + vitreous tap/vitreous inject Pre-existing uveitis
Anaerobic cultures for 14 DAYS Differential UGH syndrome

Investigation
Notify lab or they will destroy it Diagnoses – Atypical
PostOp Uveitis Retinal detachment
Sensitive
PCR
Epithelial downgrowth
Expensive, high false-positives from contamination
Low grade endophthalmitis

333
Staph species (coagulase negative,
334
epidermis), Strep spp.

Intravitreal ceftazidime
Ocular – proptosis, corneal ring abscess
Fulminant endophthalmitis
Organisms Bacillus cereus Systemic – fever, leucocytosis
Intravitreal vancomycin + clindamycin

Fungal
Admit to hospital
Strep spp., S. epidermidis, H. Organisms Cefazolin, vancomycin
influenzae, Enterococcus species Post-Traumatic
Topical fortified antibiotics
Treatment Systemic antibioitics

Ciprofloxacin 750 mg po bd
Immediate postoperative Vitrectomy + antibiotics
S. epidermidis Early
Filtering Steroids
Avoid until fungal organisms ruled out
Blebitis Blebs
Intense AC reaction, minimal vitreous reaction
Types 10% get VA >6/60
EVS 1995
Poor prognosis
Endophthalmitis secondary to blebitis Risk factors
Marked vitritis Dirty injury, breach of capsule, retained IOFB, delay in repair
47% get VA >6/120
EVS 1995 Poor prognosis Endocarditis – Strep. spp
Gram positive Cutaneous infections – S. aureus
IVDU, immunocompromised,
General 11.33 Endophthalmitis – IVDU – Bacillus spp.
long-term catheter Organisms
Other Causes N. meningitidis
Discrete, multifocal
Choroidal or deep Gram negative
yellow–white H. influenzae
retinal lesions
Clinical Enteric organisms – E. coli, Klebsiella
Become diffuse lesions
Candida Retinitis/ Internal environment
+/– Vitreous haze, cells Uveitis/Endophthalmitis Source
Hematogenous Bilateral in 10%
Admit + involve physician Patients
Immunocompromised/sick people
Blood, urine, Search for fungus Treatment Two groups of people e.g. indwelling IVC, septic
Endogenous
catheter tip IVDU – intravenous drug users
Proptosis, chemosis, corneal edema

Fluconazole IV/oral Anterior
Clinical Anterior fibrinous exudate +/– hypopyon
Vitreous haze, white–yellow retinal infiltrates
Posterior
May be single choroidal infiltrate
Tap
Anterior chamber and vitreous
Oral fluconazole
IVDU patient – Candida
Amphotericin B 5 +g/0.1 mL intravitreal
Treatment
Inject Intravitreal vancomycin + cephazolin + amphotericin B
Non–IVDU Consider systemic antibacterials
Consider systemic voriconazole
Aspergillus, Scedosporium, Candida
Sectoral
Episcleral Simple 75% Diffuse

Inflammation Types Both resolve in 2 wk
Oval mobile nodule & little surrounding congestion

Nodular 25%
Resolve 5–6 wk
Self-limiting, benign episcleral inflammation

Abrupt onset
Within 1 h
Symptoms
Clinical Mild ocular discomfort No radiation
Superficial episcleral blood vessels
Injected, dilated
Signs Bilateral 1/3 patients
11.34 Episcleritis
No globe tenderness
10% phenylephrine => eye becomes white

Idiopathic, clinical diagnosis
Mobile inflamed vessels – with cotton bud
Investigations
Atopy, rosacea, gout, HSV/VZV, TED
Systemic associations in 1/3 Rheumatological – RA most common
Drugs – bisphosphonates
Underlying cause
Cool compress, lubricants
Treatment
Indomethacin or ibuprofen
Oral NSAIDs Gastric protection – omeprazole
Topical steroids
Rebound effect though
335
336
Investigate if unknown cause

Systemic disease in 40–57% Posterior scleritis Scleral & choroidal thickening
B-scan
Edema in Tenon's space = "T Sign"
PWRRS
PAN, Wegener's, Relapsing polychondritis, RA, SLE Inflammatory Etiology CXR +/– Quantiferon Gold
IBD Must Rule Out TB
INFECTIOUS Syphilis serology
Toxoplasmosis, herpes zoster, Lyme disease Investigations
Infectious
Prior ocular trauma, surgery Rheumatoid factor Wegener's – specificity 85–95%
c-ANCA
Masquerade – neoplasia Other ANCA PAN – specificity only 15%
p-ANCA
Drugs – bisphosphonates
HLAB27, Lyme, ANA (SLE)
Biopsy
If not resolving
Common, mildest
D – diffuse Treat aggressively
Immobile nodule in interpalpebral zone Scleritis – sign of systemic disease progressing
Tender, elevated red N – nodular
Destructive +/– Topical steroids
White avascular areas, surrounding scleral inflammation Inflammation Anterior Types Mild–moderate diffuse/nodular disease
1. Oral NSAIDs
Sclera thin – underlying uvea brown N - Necrotizing No systemic disease
Scleromalacia perforans Small benefit in severe/necrotizing scleritis
No inflammation
Rare bilateral condition
+/– Initial IV methylprednisolone
Elderly women with longstanding RA Posterior 11.35 Scleritis pulse
Inflammation posterior to ora serrata 1.5 mg/kg/d prednisolone
Usually treat for 6–12/12
Ocular history
Radiotherapy, mitomycin 2. Oral steroids
Indications Severe disease, systemic association
Insidious onset 5–10 d Failed NSAIDs
Radiating to forehead, jaw, temple, sinuses
Symptoms Treatment Gastric & bone protection
+/– Prevent sleeping Moderate–severe pain
Eye tender ++
Rheumatologist
Anterior – sometimes affected Failed steroids
Vision
Indications Too high steroid dose required
Posterior – always affected Decreased vision Clinical
Adjacent structures inflamed Best, 15–25mg po/wk

Keratitis, uveitis, glaucoma, cataract, 1. Methotrexate
fundus abnormality 3. Immunosuppression + Folic acid & monitor LFTs, FBC
Bluish-tinged sclera
Inflammation – does not blanch 2. Azathioprine OR mycophenolate mofetil
Signs
with 2.5%/10% phenylephrine
3. Cyclophosphamide Underlying vasculitis –
Exudative retinal detachment Wegener's, PAN
4. TNF-alpha
Choroidal folds or detachment Last resort, increase cancer risk
Posterior scleritis
Disc, macular edema, subretinal mass
Restricted motility & proptosis
Periocular Risk exacerbating scleral melt/perforation
Controversial
corticosteroids Avoid in necrotizing disease
Subconjunctival/orbital floor Methylprednisolone 0.5 mL,

40 mg/mL inferotemporal
aka Wegener's (associated with NAZI regime)
Idiopathic multisystem disorder
General Small vessel vasculitis Respiratory tract + kidneys
5th decade, pulmonary symptoms, hematuria

Nasal or oral inflammation
Abnormal CXR
2 of
Criteria Abnormal urinary sediment
ANCA-Positive
Granulomatosis, Granulomas on biopsy

Granulomatosis
ANCA +ve
with Polyangiitis
Necrotizing granulomatous
reaction
Respiratory tract Nasal septum perforation
Involving cartilage
"Chondritis" Small vessel vasculitis Infiltrates, cavitation
Rare, idiopathic Systemic
11.36 Scleritis – Wegener's
Necrotizing glomerulonephritis =>
Ocular Granulomatosis & Renal failure
Scleritis, anterior uveitis
Relapsing Polychondritis Clinical Focal vasculitis – heart, spleen, adrenals
Pinnae Orbit
Recurrent swelling Orbital inflammatory disease, NLDO, dacryocystitis
Clinical
Tracheobronchial
Hoarse voice, cartilage Systemic Necrotizing scleritis
cough, stridor
Relapsing Ocular Peripheral ulcerative keratitis
Collapse of nasal Polychondritis
Saddle-shaped bridge Occlusive retinal periarteritis
nasal deformity
Investigation
Biopsy when ear actively inflamed c-ANCA
Investigations 90% sensitivity
Mild
Oral steroids
NSAIDs
Treatment Treatment
Major organ + Cyclophosphamide
High dose steroids
involvement
+ Immunosuppression
337
aka Vitiliginous
338
AM PM Bird & Serpent AA chorioretinitis A/w HLA-A29 + nyctalopia, color disturbance

Involve retina & choroid
Group of idiopathic multifocal inflammation Multifocal, indistinct cream lesions, 1/2 DD
"White dots in fundus" Clinical Do not pigment, radiate from disc Shotgun spray around disc
Generally bilateral Birdshot
Except MEWDS + AZOOR retinochoroidopathy Late optic atrophy, retinal vascular attenuation
General Bird & Serpent FFA
Early hypo, late hyper fluorescence Retinal vascular leakage, pronounced CME
FFA Middle-aged
+/– CME/CNVM pattern
Treat With Exacerbations + remissions 20% self-limited
Differential diagnoses Immunosuppression Complications
Infectious, inflammatory, masquerade CME, ERM, CNVM, optic atrophy 40% >6/60 after 10 y
Chronic Progression +
Uveitis Complications End-stage MFC, sarcoid, lymphoma
Differential diagnoses
Acute Posterior Multifocal Placoid Pigment Epitheliopathy Mild vitritis
Vitritis, disc edema Gray–white–yellow lesions at RPE

Clinical
Serpiginous Start at optic disc =>
Multiple cream color, plaque-like lesions – at RPE level choroidopathy spread in serpentine manner Toward macula/peripheral fundus
Fade over 2–6/52 – leave scars Active at edge of lesion – white, fluffy
Clnical
20% left with visual dysfunction Initial blockage
APMMPE FFA
Long-term
Cerebral vasculitis risk Leakage at active edge
Subtopic
Requires treatment
11.37 White Dot
Acute Retinal Pigment Epithelitis
Syndromes –
Early hypofluorescence due to choroid nonperfusion Unilateral Small hyperpigmented lesion – RPE level
Retinochoroidopathies
FFA ARPE Clinical 2–4 clusters, 2–6 dots in posterior pole
Late hyper staining
FFA Honeycomb pattern
DDx does not include
(APMMPE larger) AM AA Young, Healthy F>M Electrophysiology EOG subnormal & out of proportion with retina
MEWDS, PIC, MFC, birdshot Young Female, Electrophysiology Testing
Viral Prodrome No Treatment +/– Steroids Acute Zonal Occult Outer Retinopathy Unilateral
Multiple Evanescent White Dot Syndrome No Treatment Good Prognosis Photopsia + temporal visual field loss
Good Visual
AZOOR – NO Early – fundus normal + large
Unilateral 80% Prognosis
DOTS! peripheral scotoma
Clinical
Later – minimal retinal findings Mild vitritis 50%
Photopsia – central constant whirl shimmer RPE clumping & arteriolar narrowing
MEWDS
Multiple small white retinal ERG – decreased amplitude of A- & B- waves
Resolve 1–2/12 Clinical Electrophysiology
dots – posterior pole EOG – bipolar cells – absence/severe reduction
Granular macula in acute phase Punctate Inner Choroiditis

Myopic women
Lesions transient – evanescent – disappear
PIC Clinical Vitreous clear
PM Young Female Punctate yellow inner choroidal lesions – posterior pole
FFA Rx With Steroids/ FFA
Wreath sign, many more dots seen than funduscopy Immunosuppression Block early & stain late
Complications – CNVM,
CME, Subretinal Fibrosis MultiFocal Choroiditis (& Panuveitis)
Bilateral vitritis & multifocal choroiditis
OCT
Disturbance outer photoreceptor layer MFC Clinical AC activity, CME
Small yellow–white choroidal lesions
Pigment over time
Disc edema
Check Infective/Inflammatory causes
APMPPE Birdshot
MEWDS Serpiginous
11.38 White Dot

Syndromes
AZOOR –
No dots
PIC
ARPE
MFC

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MINDMAPS in

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International Standard Book Number-13: 978-1-4822-3065-9 (eBook - PDF)

2 Cataract and Refractive Surgery 5

3 Cornea and External Eye 27

7 Orbit, Oculoplastics, and Lacrimal System 157

8 Pediatric Ophthalmology 193

9 Retina and Vitreous 245

11 Uveitis and Inflammatory Eye Disease 301

5FU: 5-fluorouracil BD: twice a day (Latin: bis die)

CT: connective tissue G6PD: glucose-6-phosphate dehydrogenase

LL: lower lid OCD: obsessive compulsive disorder

PVD: posterior vitreous detachment UGH syndrome: uveitis–glaucoma–hyphema syndrome

1.1 Management Principles

1.2 Ophthalmic Emergencies

What can KILL/BLIND this patient? First Step

2.11 Crystalline Lens

2.7 Femtosecond 2.21 Multifocal IOLs

Genetic & metabolic

Rigid/foldable posterior chamber IOL

Corneal endothelial dystrophy

Cornea/endothelial problems White–white horizontal + 1mm =>

Scleral-Fixated IOL Anterior Size of implant

>1 mm axial length difference

IOL master, LensStar

e.g. Holladay Theoretical Corneal diameter, refraction, ACD, lens thickness

Regression analysis on refraction results Small incision phacoemulsification

Holladay2 – excellent for all eyes

Keratometers measure central zone K

Postkeratorefractive Historical Pre & post measurements

Compare with ASCRS website calculation

<4 clock hours zonular dialysis Prerequisites

Localized zonular weakness

Vision blue dye to stain capsule Anisometropia

Assess visual potential

Wound – consider suture Intraoperative

Avoid ACIOL IOL Diabetes Weak zonules =>

Superior Rectus Stay Suture

Minimum 120 degrees

15 degree, penetrate with keratome

Viscoelastic & Paracentesis Port

With forceps, push eye inferiorly

Close Wound 10-0 nylon sutures

Small pupil <4.5 mm

Contraindications Corneal scar

Decentered => optic tilt. Small => phimosis

Size, diameter, roundness

Lens Less power, endothelial cell loss,

New laser technology

Prevent vitreous hydration

Dispersive viscoelastic cover hole Intracameral phenylephrine 2.5%

Heavy viscoelastic Refill with viscoelastic

10-0 Nylon, leave 3/12 ACIOL or sulcus 3-piece IOL

Extrusion of ocular contents & iris prolapse

Risk factors Close wound tight

Drain with posterior sclerotomies

Extrusion of ocular contents Types

Non-expulsive 20% attain VA >6/12

Poor VA < count fingers if spontaneous

Wrong formulae Preoperative

Previous refractive surgery Contact lens, eye pad

Instruments Peak incidence 6–10/52 postoperative

Topical steroids hourly