Notes To Remember
For Clinical Ophthalmology MCQ
Exams
Dr Abdullah Fouad
Index
1) General Medicine related to Ophthalmology……………. 3
2) Community and Public Health…………….…………….……… 5
3) Ophthalmic Pathology and Tumours…………….………….. 7
4) Neuro Ophthalmology…………….…………….…………….……. 12
5) Pediatric Ophthalmology and Strabismus………………… 27
6) Orbit, Eyelid and Lacrimal diseases…………….……………. 30
7) Trauma, External Eye diseases and Cornea………………. 41
8) Ocular Inflammation and Uveitis…………….…………….….. 52
9) Glaucoma…………….…………….…………….…………….…………. 58
10) Lens and Cataract…………….…………….…………….…………. 70
11) Retina and vitreous…………….…………….…………….……….. 79
Abdullah Fouad Notes
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1) General Medicine Related To Ophthalmology
From Exams
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Ophthalmologic signs of TTP may be: (Eyewiki)
A) Retinal: hemorrhage, arterial or venous occlusion, or serous retinal detachment.
B) Cranial nerves: anisocoria or ocular misalignment.
C) Papilledema (optic disc edema due to increased intracranial pressure)
D) Others hypertensive choroidopathy, retinopathy, and optic neuropathy have also been reported.
Ocular Disorders associated with Thrombocytopenia : (Kanski)
1- Capillary Hemangioma (Kasabach–Merritt syndrome)
2- Albinism (Hermansky–Pudlak syndrome)
3- Drug induced (Pyrimethamine > as a treatment of Toxoplasmosis)
Regarding ocular involvement in Graft Versus Host disease (GVHD), a mean Schirmer’s test in both eyes
of less than 5mm is characteristic
Rifampicin >> Pink-tinged tears
Quinilones >> inhibition of nucleic acid synthesis
Anaphylactic Shock >>IgE
Burkitt’s lymphoma >> Epstein-Barr virus
Metabolic Syndrome >> 3 or more of (Large waist – High Triglyceride – Reduced HDL – increased BP –
increased Bood Sugar)
In the inflammation cascade, cell membrane lipids are converted to arachidonic acid by phospholipase
A2, and prostaglandins are then formed by cyclooxygenases (COX).
Corticosteroids reduce inflammation by → increase the synthesis of lipocor ns → block phospholipase
A2 → prevents the conversion of phospholipids to arachidonic acid
Tumor Necrosis Factor (TNF) → Infliximab, Adalimumab, Etanercept.
Rituximab→ (It’s not TNF) is a monoclonal antibody directed against CD20 positive cells mainly B
lymphocytes useful in treatment of Behcet disease and Wegener granulomatosis.
Abdullah Fouad Notes
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Antiphospholipid syndrome (APS) → elevated levels of an bodies directed against membrane anionic
phospholipids (i.e., anticardiolipin antibody, antiphosphatidylserine) or their associated plasma
proteins, predominantly beta-2 glycoprotein I (Apo lipoprotein H); or evidence of a circulating
anticoagulant.
The best screening test for SLE → The antinuclear antibody (ANA) test “most sensitive” (Anti-native (N)DNA and anti-Sm (Smith antigen) tests are highly specific for SLE and have strong confirmatory powers)
Corticosteroids in uveitis → reduction of inflammatory infiltration of the retina, choroid, or optic
nerve.
Lymphocytes → mostly Short-lived with an average life span of a week to a few months, but a few live
for years, providing a pool of long-lived T and B cells.
Cyclosporine → is isolated from the fungus Tolypocladium inflatum.
Interleulcin-9 → is a cytokine secreted by CD4+ T-helper cells that acts as a regulator of a variety of
hematopoietic cells. This cytokine stimulates cell proliferation and prevents apoptosis.
Dr Abdelrahman → malignant hyperthermia is genetic, caused by succinyl choline or inhalational
anesthesia "Halothane" and treated by Dantrolin. Muscle biopsy can diagnose but after hyperthermia
happens. succinyl choline can cause also apnea.
Hypersensitivity Reactions: (My Own Mnemonic → High City Against Media)
Type I > Histamine (IgE, immediate) 'Spring Catarrh, Ligneous conjunctivitis, Anaphylactic shock'
Type II > Cytotoxic 'Thyroid eye disease, OCP'
Type III > Ag-Ab complex 'Marginal Keratitis, Vasculitis'
Type IV > Cell-mediated or delayed hypersensitivity 'Granuloma, Tuberculin skin test (TST), Trachoma,
SJS, Graft rejection'
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From MCQ Books
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Up to 25% of individuals with HLA-B27 develop sacroiliac disease.
Bendroflumethiazide is a thiazide diuretic which can be associated with increased loss of postassium
leading to hypokalaemia.
Amiloride and spironolactone are potassium-sparing diuretics, so are not likely to cause hypokalaemia.
As an ACE inhibitor, ramipril causes hyperkalaemia not hypokalaemia.
Wilson's disease → AR, paradoxically low serum copper, high urinary copper.
RCC (Hypernephroma) > Megalocornea
Wilms tumour > Aniridia
Pheocromocytoma > Von-Hipple-Lindau
Von-Hipple-Lindau > Ch.3
All phakomatosis AD, except Sturge weber > sporadic
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Abdullah Fouad Notes
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2) Community and Public Health
From Exams
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Cesarean section leads to a decrease of maternal to fetal transmission of HIV
Breast Milk is an important source of Vitamin A
AMD>> have increased prevalence in long term tobacco smokers **
Immunization programs can reduce the incidence of childhood blindness **
An ideal screening test for annual glaucoma or diabetic retinopathy screening would have (optimal
positive predictive value at the expense of negative predictive value)
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Musca sorbens (a fly) is a transmission vector of Trachoma
Cross-sectional study (also known as a cross-sectional analysis, transverse study, prevalence study) is a
type of observational study that analyzes data from a population, or a representative subset, at a
specific point in time.
QuantiFERON-TB GoldTest(QFT) is a modern simple blood test, an alternative to the tuberculin skin test
(TST, PPD or Mantoux) >> QFT is an interferon-gamma protein (IFN-γ)
Carotic Edndarterectomy (CEA) has pre-operative morbidity and mortality rate of less than 5%.
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Abdullah Fouad Notes
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Regarding the global burden of uncorrected referactive errors → it accounts for 7 million cases of
Blindness.
Receiver Operating Characteristic curve (ROC curve) → it is a plot of the true positive rate “TPR”
(sensitivity) on “X” axis against the false positive rate “FPR” (1-specificity) on “Y” axis for the different
possible cut points of a diagnostic test.
Cross-sectional study → observation of all or a subset of a population, at a defined time.
Randomized Controlled Trial → a controlled clinical trial that randomly (by chance) assigns participants
to two or more groups. There are various methods to randomize study participants to their groups.
Example: Meditation or exercise for preventing acute respiratory infection: a randomized controlled
trial.
Cohort Study (Prospective Observational Study) → a clinical research study in which people who
presently have a certain condition or receive a particular treatment are followed over time and
compared with another group of people who are not affected by the condition. Example: Smokeless
tobacco cessation in South Asian communities: a multi-centre prospective cohort study.
Case-control Studies → begin with the outcomes and do not follow people over time. Researchers
choose people with a particular result (the cases) and interview the groups or check their records to
ascertain what different experiences they had. They compare the odds of having an experience with the
outcome to the odds of having an experience without the outcome. Example: Non-use of bicycle
helmets and risk of fatal head injury: a proportional mortality, case-control study.
In 1968, Wilson and Jungner published 10 “principles” for evaluating screening programs, criteria widely
used since then:
1- The condition sought should be an important health problem. (Came in 2013,2012 exams)
2- There should be an accepted treatment for patients with recognized disease.
3- Facilities for diagnosis and treatment should be available.
4- There should be a recognizable latent or early symptomatic stage.
5- There should be a suitable test or examination.
Abdullah Fouad Notes
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6- The test should be acceptable to the population.
7- The natural history of the condition, including development from latent to declared disease, should
be adequately understood.
8- There should be an agreed policy on whom to treat as patients.
9- The cost of case finding (including diagnosis) should be economically balanced in relation to
possible expenditure on medical care as a whole.
10- Case finding should be a continuing process and not a “once and for all” project.
The WHO Global Action Plan 2014_2019 is underpinned by 5 principles and approaches:
1- Universal access and equity.
2- Human rights.
3- Evidence-based practice.
4- A life course approach.
5- Empowerment of people with visual impairment.
The World Medical Association (WMA) has developed the Declaration of Helsinki as a statement of
ethical principles for medical research involving human subjects, including research on identifiable
human material and data.
3) Ophthalmic Pathology and Tumours
From Exams
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In Von Hippel-Lindau lesions (retinal angiomatosis), Multiple angiomas may be found in the same eye
(80% of cases are familial)
Regarding Malignant Melanoma of Choroid>>Histological cell type is the most significant prognostic
indicator
Regarding Malignant melanoma of the Uvea >>Tomours involving the CB carry a worse prognosis
Ataxia telangectesia (Louis-Bar syndrome)>> increased incidence of cancer
Regarding Optic Atrophy >> loss of ganglion cell axons
Scattered, well demarcated tumours of the retina composed of glial cells >> Tuberous Sclerosis
BCC of the eye lid >> Mohs’ Surgery carries a better than 95% better cure rate for BCCs
Regarding Malignant Melanoma of the uvea :
- Spindle cell melanoma → best prognosis
- Epithelioid melanoma → worst
- Mixed-cell type (mixture of spindle and epithelioid cells) → intermediate prognosis
Regarding Uveal Melanoma → Monosomy of chromosome 3 carries poor prognosis, especially with
gains in chromosome 8. Chromosome 3 loss associated with a reduction of the 5-year survival
probability from approximately 100% to 50%.
Choroidal Melanoma → Presenta on peaks at around the age of 60 years (Range 45-80 years).
Mortality is up to 50% at 10 years. Every millimeter of increasing thickness promotes and increasing 5%
risk for metastasis by 10 years follow up.
The American Cancer Society reports: (Regarding Choroidal Melanoma)
Abdullah Fouad Notes
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Survival Rate
5 Years Survival 10 Years Survival
Stage 3 (Spread to LN)
40-78%
24-68%
Stage 4 (Spread to other parts of the body) 15-20%
10-15%
Choroidal Melanoma → once hepatic metastases are established, survival is usually less than 12 months.
In Uveal Melanoma, involvement of vortex veins is of prognostic significance → A higher propor on of
patients with vortex vein invasion progress to develop liver metastasis compared with the general uveal
melanoma population.
Among Uveal melanomas → melanoma of the iris has the best prognosis, the ciliary body and anterior
choroidal melanomas have the worst prognosis of all intraocular melanomas.
Epithelioid cells have distinct cell borders, in contrast to Spindle A and B cells.
A medulloepithelioma (diktyoma) originates from the nonpigmented epithelium of the ciliary body
and most often presents as an iris mass during the 1st decade of life.
Although distant metastasis is rare, local invasion can lead to death. Teratoid elements are often
present. Enucleation is usually required and is curative in most cases.
Nevus of Ota → can present acutely with an intracranial melanoma as an extension of a primary uveal
melanoma.
Choroidal haemangioma → Characteristic very high internal reflectivity on A-scan ultrasonogram.
Choroidal melanoma → Characteristic low internal reflectivity on A-scan ultrasonogram.
The most common primary sites for cancer in women → breast, colorectal and lung cancers.
The most common cause of visual loss in patients with a choroidal osteoma → CNV.
NF1 → Op c pathway gliomas (OPGs). AD Ch.17, 50% have new mutation.
NF2 → BL acous c neuroma.
Every patient with NF-1 receives at least an annual neuro-ophthalmologic examination, and unexplained
acuity, color,or field loss; afferent pupillary defect; proptosis; or opticatrophy would also mandate
neuroimaging.
Subtenon carboplatin for treatment of retinoblastoma → Ischemic op c neuropathy and atrophy.
Extensive intraocular calcification in a tumour mass in a degenerate and disorganized retina → Occlusion
of central retinal vessels is a probable cause of necrosis. (answer sheet 2017)
In rare instances, retinoblastoma can undergo complete and spontaneous necrosis “Phthisis” (although
this is seldom recognized with active disease). The vitreous cavities of these phthisical eyes are filled
with islands of calcified cells.
Homer Wright rosett “typically seen in RB, neuroblastomas, medulloblastomas, and primitive
neuroectodermal tumors (PNETs)” → consists of a halo of tumor cells surrounding a central region
Abdullah Fouad Notes
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containing neuropil (hence its association with tumors of neuronal origin)
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Flexner-Wintersteiner Rosette “characteristic for RB” → consists of tumor cells surrounding a central
lumen that contains cytoplasmic extensions from the tumor cells.
cells
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Pseudorosettes “CNS ependymomas “→ Collections of tumor cells around blood vessels (It’s called a
pseudorosette because the central structure isn’t part of the tumor)
Abdullah Fouad Notes
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True Ependymal Rosette “only
only present in a small percentage of well-differentiated
well differentiated ependymoma) →
tumor cells surrounding an empty lumen.
lumen
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A bilateral retinoblastoma survivor has a 45% chance of having an affected child
A unilateral retinoblastoma survivor has a 7%–15%
7%
chance of having an affected child.
Unaffected parents of a child with bilateral retinoblastoma have less than a 5% risk of having another
affected child.
Isolated astrocytomas are usually not associated with systemic disease,
disease, but most multiple/ bilateral
tumours are seen in the context of tuberous sclerosis.
sclerosis An association with NF1 is also suggested
suggested.
In the presence of a positive family history, VHL disease can be diagnosed clinically in a patient with at
least one typical VHL tumour.
In patients with a negative family history of VHL-associated
VHL associated tumours, a diagnosis of VHL disease can also
be made when they exhibit two or more haemangioblastomas, or a single haemangioblastoma in
association with another typical manifestation.
manifestation
Subependymal nodules → observed in 80% of Tuberous Sclerosis (TSC) patients and often prenatally
detected or at birth.
Shagreen patches → observed in 50% of Tuberous Sclerosis (TSC) patients and typicall
typically have their onset
st
in the 1 decade of life.
The classic triad of TSC → epilepsy, mental retardation and adenoma sebaceum (zits, fits and twits).
Regarding VHL→ An inherited mutation of the VHL gene (AD) is responsible for 80 percent of cases, 20%
of cases of disease are found in individuals without a family history, known as de novo mutations.
Renal cell carcinoma is the leading cause of death in VHL disease. CNS haemangioblastomas are the 2nd.
Renal cell carcinoma is the presenting finding in 10 % of patients with VHL disease.
Pheochromocytoma was the first manifestation in 50% of patients.
patients (in study)
Cerebellar or spinal (CNS) hemangioblastoma occur in 70 % of patients with VHL disease
disease.
The prevalence of retinal tumours in VHL is approximately 60%.
Von Hippel-Lindau disease manifests in the 3rd to 4th decades of life,, depending on the location of the
tumors. The mean age of onset of retinal hemangioblastoma, one of the first manifestations, is around
26 years. However, 5% of patients with retinal hemangioblastoma can present under the age of 10.
Regarding Retinal hemangioblastoma → the median age at presentation for VHL disease is usually lower
than the sporadic form: 17.6 years and 30.8 years, respectively.
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Abdullah Fouad Notes
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A retinal racemose haemangioma (shown in the image above) is associated with Wyburn Mason
syndrome,, which is a sporadic condition with ipsilateral AV malformations involving the midbrain,
basofrontal region and posterior fossa.
Primary vitreoretinal lymphoma (PVRL) is the name given to primary central nervous system lymphoma
(PCNSL) when its initial or primary manifestation is in the eyes. (it is known as a masquerade syndrome
because it frequently simulates the signs and symptoms of uveitis)
- 98% of PCNSL are non-Hodgkin B--lymphocytic lymphomas and only 2% are T ly mphocytic lymphomas.
- 75% of patients presented with either anterior or posterior uveitis.
- The pathognomonic feature of PCNSL→
PCNSL sub RPE infiltrates (only 20%) “Creamy yellow subretinal
infiltrates with overlying RPE detachments”.
detachments” When they resolve after being present for some time
they typically leave an area of geographic atrophy. (the Figure below)
- At times, the KPs associated with PCNSL will have a branching, dendritiform appearance.
appearance.
- CSF seeding of lymphoma cells occurs in 42% of patients with PCNSL.
- Behavioral changes appear to be the most frequent sign because of the periventricular location of
many of the CNS lesions. (AAO)
- Lesions predominantly affect oculomotor and facial nerves and aree most frequently caused by the
meningeal spread of neoplastic cells. (AAO)
- Steroid TTT may be initially successful.
Abdullah Fouad Notes
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The Below figure is congenital hypertrophy of the RPE (CHRPE), the RPE cells are larger than normal and
contain more densely packed melanin granules.
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The figures below are → Bilateral diffuse uveal melanocytic proliferation (BDUMP), a very rare
paraneoplastic syndrome occurring usually in patients with systemic, often occult, malignancy. It is
characterized by the proliferation of benign melanocytes in the outer choroid often manifesting with
multiple naevus-like choroidal lesions. (Kanski)
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4) Neuro Ophthalmology
From Exams
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Oculomotor apraxia is an inability to initiate voluntary horizontal saccades.
* characterized by striking compensatory head movements.
* Congenital OMA usually improves with age.
* Pursuits remain relatively unaffected in oculomotor apraxia.
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* Acquired lesions (e.g. resulting from bilateral frontal or parietal lobe strokes) usually produce defects
in initiation of bidirectional saccades; patients may blink to break fixation and then turn their head to
fixate on something else.
* The location of the lesion that causes congenital ocular motor apraxia is not known but probably is
above the brainstem centers that drive volitional saccades.
Cerebral Venous Thrombosis (CVT) may have led to sensorineural hearing loss by inducing increased
intracranial pressure. Increased pressure in the subarachnoid space may be conveyed to the perilymph
and endolymphatic system via the cochlear aqueduct, causing increased fluid pressure in the internal
ear which eventually causes cochlear dysfunction. Cranial nerve VIII function may also be disturbed by
the increased intracranial pressure (9). Thrombosis of the transverse sinus can increase cochlear
pressure and give rise to anoxic changes as a result of impaired drainage or thrombosis extending to the
cochlear or labyrinthine veins. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5866491/
How to differentiate between carotid-cavernous sinus fistula (direct) and dural cavernous sinus fistula
(indirect) >> Audible bruit
Glaucomatous optic neuropathy (2ry OAG) can occur in 20% of patients with untreated CCFs.
2ry CAG can be caused by CCF (partial thrombosis of the ipsilateral superior ophthalmic vein and
cavernous sinus → Choroidal effusion → ACG)
High- flow, direct fistulas most commonly occur after severe head trauma and produce a cranial bruit,
whereas low- flow, indirect fistulas most often occur spontaneously, particularly in older women.
4th nerve palsy ipsilateral to Horner syndrome locates the lesion in the cavernous sinus, while
contralateral 4th nerve palsy locates the lesion in the midbrain.
See-saw nystagmus >> tumor affecting optic chiasma ‘’visual field defect’’ ***
Types of nystagmus that associated with a visual field defect > See-saw and Pseudo-spasmus nutans (a
monocular shimmering nystagmoid oscillation)
Downbeat nystagmus >> Chiari $ - Lithium – Idiopathic
Alexander's law: Increased amplitude in the gaze that have the same direction of the nystagmus
(e.g. Upbeat nystagmus have the largest amplitude in up-gaze)
Hemifacial spasm is typically caused by irritation of the facial nerve as it exits the brain stem (in the
cerebellopontine angle).
Infantile nystagmus syndrome (INS) or Congenital nystagmus (CN) is associated with conditions that
cause bilateral, pregeniculate vision loss prior to 2 years of age.
 Bilateral dense Congenital Cataract
 Retinal Dystrophies (Acrompatopsia, Leber congenital amaurosis)
 Foveal Hypoplasia
 Optic Neuropathy (Optic nerve hypoplasia)
Fusional maldevelopment nystagmus (FMN) or latent nystagmus (LN) → associated with Infantile
Esotropia
Ischemic and demyelinating INO typically recover. (eyewiki)
The 2 most common causes of INO are demyelination and stroke. In adolescents and younger adults,
INO is typically caused by demyelination. In older adults, microvascular disease is the most common
cause.
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Most supranuclear disorders affect both eyes equally and therefore do not cause diplopia EXCEPT:
(AAO)
✓ Convergence insufficiency or spasm
✓ Divergence insufficiency
✓ Ocular tilt reaction
✓ Skew deviation
✓ Thalamic esodeviation
• The vertical misalignment can be comitant or incomitant
• Skew deviation typically occurs with intorsion of the hypertropic eye (in contrast, 4th cranial nerve
palsy causes extorsion of the hypertropic eye) (AAO)
• The vertical deviation in skew deviation typically improves when the patient lies down, whereas it
doesn‘t change in 4th nerve palsy.
• An alternating skew deviation manifests as hypertropia on abduction
Dominant optic atrophy (Kjer type optic atrophy, optic atrophy type 1) >> frequently due to a mutation
in the OPA1 gene on chromosome 3.
Central Tegmental Tract lesion > Oculopalatal Nystagmus
Homonymous paracentral scotoma is a common presentation of Migraine.
Acute Demylinating encephalomyelitis > pre-pubertal Childhood, while MS > young adults.
Glaucomatous optic neuropathy can occur in 20% of patients with untreated CCFs.
The spontaneous closure rate of dural arterio- venous fistulas has been reported to be as high as 75%.
Closure has been reported after air travel or after angiography or manual compression of the carotid
artery.
Ocular finings of cerebral palsy → Prosopagnosia (problems recognizing faces), segmental (temporal )
optic disc pallor and generalized pallor, large cup and hypoplasia.
- VEPs and BAEPs. (NCBI) Normal ERG (answer sheet 2017).
- Congenital esotropia
A visual hallucination associated with macular degeneration is known as Charles Bonnet syndrome and
is relatively common in patients with severe visual loss.
In TED → Visual field defects may be reversed by systemic steroids (answer Sheet)
Vigabatrin “Sabril, Sabrilex” (antiepileptic drugs) → inhibits GABA transaminase irreversibly →
increases concentration of GABA (major inhibitory neuro-transmitter in brain).
→ Causes visual field constriction, usually asymptomatic unless the defect encroaches central area.
Optocociliary shunt Causes (Chronic venous obstruction):
- Longstanding OAG
- CRVO
- Meningioma and glioma
- Chronic Papilledema
idiopathic intracranial hypertension → Lumbar puncture Opening pressure >25cm water (normally 1018cm water)
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Morning Glory usually associated with midline defects like cleft palate and corpus callosum agenesis
agenesis.
Serous retinal detachment occurs in 30%.
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Myotonic Dystrophy is associated with light near dissociation.
Retinal migraine (otherwise known as ophthalmic migraines, anterior visual pathway migraines, Retinal
vasospasm or ocular migraines) causes monocular visual loss for 10–20
10 20 minutes which can be associated
with diffuse or unilateral headache..
Neurofibromatosis (NF) with sphenoid wing dysplasia is a rare clinical entity which presented with
pulsatile progressive proptosis.
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The following findings may suggest the need for additional evaluation of those patients presumed to
have migraine:
1-Headache
ache or aura always occurring on the same side.
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2- Headache preceding the aura.
3- Neurologic deficit, including visual field defect, persisting after aura resolves.
4- Atypical aura.
The classic (Hoyt–Spencer) diagnostic triad of Optic nerve sheath meningioma (ONSM):
1- Painless, slowly progressive monocular vision loss.
2- Optic atrophy.
3. Optociliary shunt vessels.
Minimal to mild proptosis can also occur with ONSM.
The most common cause of acquired 4th nerve palsy in adults is → trauma
The most commonly damaged cranial nerve from head trauma → The 4th nerve.
The most commonly damaged cranial nerve from increased ICT → The 6th nerve (False localizing sign).
Structural crowding of the optic disc is one of the predisposing factors of NAION.
Prognosis
Pain
Fellow
eye
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NAION
50% achieve 6/9 or better, though 25% will only
reach 6/60 or worse
painless
10% after 2 years,
15% after 5 years.
AAION
40% are associated with permanent
visual loss
periocular pain is common
25% in 6 days
95% of untreated patients.
Regarding GCA → Multinucleated Giant cells are not necessary on the pathologic specimen to confirm
the diagnosis; however, when they are present, they are most often situated near the fragmented
internal elastic membrane. (eyewiki)
- ESR normal in 20%
- The CRP has a higher sensitivity than ESR; when used together the sensitivity increases to 97-99%.
- Histopathological findings persist for at least 2-6 weeks after commencing steroids.
Fundoscopic clues to a diagnosis of AAION over NAION include the following:
1- Chalky-white ONH edema (in NAION, the ONH is often hyperaemic).
2- Cotton-wool spots away from the ONH, which indicate concurrent retinal ischemia (cotton-wool spots
on or adjacent to the ONH can be present in NAION).
3- delayed optic nerve and choroidal filling while in NAION delayed optic nerve filling but normal
choroidal filling (normally, the choroid fills completely within 3–5 seconds, before the retinal arteries
do).
4- Normal or large cup in the fellow eye (in NAION, a small cup–disc ratio is common).
Occurrence of NAION in the second eye produces the clinical appearance of pseudo Foster Kennedy
syndrome, in which the previously affected ONH is atrophic and the currently involved ONH is
edematous.
Aberrant regeneration is common after trauma or compression by an aneurysm or tumor but does not
occur with microvascular ischemia.
Regarding Aberrant regeneration → Any combina on of synkine c movement of the muscles innervated
by the third nerve may be present (e.g., elevation of the upper eyelid on downward gaze or adduction;
adduction of the eye on attempted vertical gaze; limited elevation and depression of the eye with
retraction of the globe on attempted vertical gaze; or pupillary constriction with extraocular muscle
movement). (Eyewiki). However Classic findings include eyelid retraction with adduction or pupillary
miosis with elevation, adduction, or depression. (AAO)
- “Pseudo Von-Graefe Sign:” elevation of upper eyelid on downward gaze or adduction
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- “Inverse duane”: elevation of the upper eyelid on attempted adduction
- “Pseudo-Argyll Robertson pupil”: greater constriction of pupil to convergence than to light and gaze
evoked pupillary constriction.
Marcus Gunn jaw-winking syndrome → Connection between (5th and 3rd) mandibular division of the
Trigeminal nerve “External Pterygoid muscle” and superior division of oculomotor nerve “levator
muscle”.
In true papilledema, swelling of the peripapillary nerve fiber layer causes an obscured view of underlying
retinal vessels. The appearance of vessels in the peripapillary nerve fiber layer is the most important
distinguishing feature between pseudopapilledema and papilledema.
The presence of SVP in optic disc pseudo-swelling > Can differentiate it from true swelling (Papilledema)
Absence of SVP > Papilledema or (10-20% of normal population)
The risk of developing MS following an acute episode of optic neuritis after 15 years:
(Optic Nerve Treatment Trial “ONTT”)
- Overall → 50%
- Without MRI lesion → 25%
- With MRI lesion → 70% (If 3 or more lesions and each lesion is 3cm >> risk increase up to 12 times)
A substantially lower risk of developing MS when there are no MRI lesions is conferred by the following
factors: (Optic Nerve Treatment Trial “ONTT”)
○ Male gender.
○ Absence of a viral syndrome preceding the optic neuritis.
○ Op c disc swelling, disc/peripapillary haemorrhages or macular exudates.
○ Vision reduced to no light percep on.
○ Absence of periocular pain
Antibodies to Myelin oligodendrocyte glycoprotein (MOG) are associated with a phenotype involving
optic neuritis.
Course of Demylinating Optic Neuritis → Vision worsens over several days to 3 weeks and then begins
to improve. Initial recovery is fairly rapid and then slower over 6–12 months.
in the vast majority of cases optic neuritis shows spontaneous improvement with in 1 month (AAO)
Congenital fibrosis of the EOM (CFEOM) → a rare congenital syndrome due to abnormal development
of the oculomotor nuclei.
- Classic CFeOM (CFeOM1) is AD Chr 12q. there is bilateral restrictive ophthalmoplegia and ptosis, with
an inability to elevate the globes above the midline.
- CFeOM2 is AR Chr 11q. there is bilateral ptosis, wide angle exotropia, and severe limitation of
horizontal and vertical movements.
Facial myokymia (involuntary, spontaneous, localised quivering of facial muscles) is caused by disease in
the pons involving the facial nucleus or fascicle. The most common causes include MS in adults and
pontine glioma in children.
Viagra → increase the risk for NAION (Unilateral, central or paracentral) more than a direct toxicity
(Bilateral, altitudinal) to the optic nerve.
Regarding toxic and nutritional optic neuropathy → Dyschromatopsia, a change in color vision, is often
the first symptom. Bilateral, symmetric, painless, gradual, and progressive vision loss.
Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH) → inferonasal defect,
Arcuate defect, or severe visual field constriction (generalized peripheral depression). (Eyewiki). As
with acute papilledema, early IIH shows normal visual acuity and enlarged blind spots on perimetry
testing. (AAO)
However in study puplished in American Journal of ophthalmology → The most common were blind
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spot enlargement (all 12 cases), isopter constriction (nine cases), and loss on the nasal side of the visual
field (seven cases), especially in the inferonasal quadrant. Four patients had diminished visual acuities.
Optic Disc Drusen (ODD):
- Often BL.
- Initially buried, then visible.
- VA rarely decline.
- Visual field → normal, enlarged blind spot, or arcuate NFL defects.
- Different from disc edema, being Lumpy, Pumpy, Yellowish, Obvious Bl. Vs, FFA : Autoflourescence and
no leakage, B. Scan > Diagnose.
CHARGE syndrome (coloboma, heart defects, choanal atresia, mental retardation, genitourinary abnormalities, and
ear abnormalities) and the papillorenal syndrome (renal anomalies and optic nerve defects).
Coloboma of the iris → inferonasal, the pupil is shaped like a lightbulb, keyhole, or inverted teardrop.
Congenital Horner syndrome is usually caused by birth trauma to the brachial plexus. Nontraumatic
Horner syndrome in infants and children raises the possibility of neuroblastoma arising in the
sympathetic chain of the chest. In these circumstances, MRI of the head, neck, and chest is indicated.
Imaging in Horner cases is not indicated except in acute acquired cases or cases of increasing
hetetrochromia, in contrast to congenital or longstandig cases with over a year with no other localizing
lesions.
In progressive supranuclear palsy:
- downward gaze is generally affected, becoming smaller and slower. This is in distinction to the dorsal
midbrain syndrome, in which upward saccades are generally affected.
- Saccades are affected more than pursuits.
- Affects the Voluntary movements, but Spare the involuntary movements (e.g. intact Bell's
phenomenon)
The most common cause of an acquired 6th nerve dysfunction in children is a neoplasm. This is
followed by trauma, elevated intracranial pressure, inflammatory causes and post-viral etiologies.
(eyewiki).
Sixth nerve palsy in a child is a common post-viral syndrome and typically resolves entirely of its own
accord. Nonetheless, neuroimaging should be arranged to exclude intracranial pathology. The second
most common aetiology of childhood abducens palsy is increased intracranial pressure (either a CNS
tumour or idiopathic intracranial hypertension) (Eyedocs)
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4th N palsy → Compensatory Head position ( Contralateral tilt, Contralateral turn, Chin depression)
‘Contralateral T’
4th N palsy → Increased Hypertropia with ( Ipsilateral tilt, Ipsilateral turn) ‘Ipsilateral T’
Parks three-step test Made easy (Dr Gamal Sobhy)
Lt Hypertropia → Palsy of Lt depressors (Lt SO or Lt IR) or Rt elevators (Rt IO or Rt SR)
If hypertropia increase with
- Nasal eye movement > Palsy of superiors (Lt SO or Rt SR) > then hypertropia increase with
** Ipsilateral head tilt > Oblique (Lt SO)
** Contralateral head tilt > Rectus (Rt SR)
- Temporal eye movement > Palsy of inferiors (Lt IR or Rt IO) > then hypertropia increase with
** Ipsilateral head tilt > Oblique (Rt IO)
** Contralateral head tilt > Rectus (Lt IR)
CPEO → o en the only feature of mitochondrial disease (mitochondrial cytopathy)
Most unilateral migraine headaches → at some point change sides of the head.
- Headaches always on the same side of the head → may have another cause of headache (e.g.,
intracranial structural lesions).
- If headache precedes visual symptoms → is more common with arteriovenous malformations, mass
lesions with cerebral edema, or seizure foci. (Wills Eye Manual)
Ophthalmoplegic migraine → Onset in childhood. May develop 3rd nerve palsy.
Cluster Headache → Typically unilateral, very painful (stabbing), periorbital, frontal, or temporal
headache associated with ipsilateral tearing, rhinorrhea, sweating, nasal stuffiness, and/or a droopy
eyelid. Usually lasts for minutes to hours. typically recurs once or twice daily for several weeks, followed
by a headache-free interval of months to years. The cycle may repeat.
- Obtain an MRI of the brain when the history is atypical or a neurologic abnormality is present.
- Effective prophylactic agent during cluster periods: Ergotamine 1 to 2 mg p.o. q.d. (Wills Eye Manual)
Trigeminal Neuralgia → The pain is paroxysmal and sharp, usually occurring in mul ple bursts in rapid
succession lasting a few seconds.
- Attacks can be triggered either by cutaneous stimulation such as shaving or by motor activity such as
chewing. Facial sensation is normal.
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- Chewing, tooth brushing, or a cold wind may precipitate paroxysmal burning or electric shock–like jabs,
which last seconds to minutes. (AAO)
- Treatment → an epilep c drugs
- Autonomic features are sparse or absent
The most common side effect of botulinum toxin as a treatment of blepharospasm → ptosis.
In preterm infants, optic disc cupping resembling that seen in glaucoma can occur as a result of
transsynaptic degeneration, most commonly secondary to periventricular leukomalacia (necrosis of
white matter near the lateral ventricles).
Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of children and young
adults that affects the central nervous system (CNS). It is a slow, but persistent, viral infection caused by
defective measles virus.
Miller-Fisher syndrome (MFS), which is observed in about 5% of all cases of GBS, classically presents as a
triad of ataxia, areflexia, and ophthalmoplegia. TTT: IV immunoglobulin (IVIg) or plasmapheresis.
- Acute onset of external ophthalmoplegia is a cardinal feature.
- Anti-GQ1b antibodies are prominent in MFS, and have a relatively high specificity and sensitivity for
the disease.
- MFS causes descending paralysis, while GBS ascending paralysis.
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From MCQ Books
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Fingolimod (Gilenya) is a new oral agent used to treat multiple sclerosis. It has been associated with
macular edema in 0.4% of patients using the medication.
LHON →Hyperemic op c nerve with telangiecta c capillaries.
The sixth nerve nucleus contains both sixth nerve axons and interneurons destined for the contralateral
medial rectus subnucleus via the medial longitudinal fasciculus. Thus, a lesion of the sixth nerve nucleus
would produce ipsilateral gaze palsy.
All EOM are supplied by the ipsilateral Nucleus except the superiors
SO&SR → Contralateral - Both LPS → by a fused central nucleus.
Normal vertical fusion amplitudes are 3 to 5 PD. In congenital IV palsy, patient can develop amplitudes
of 10 to 25 PD, and large vertical fusion amplitudes are an indication of a long-standing vertical deviation
usually since childhood. If the cover–uncover test shows no deviation but the alternate cover test
discloses a deviation, this means a phoria, and indicates fusion amplitude (the PD needed to neutralize
the phoria with alternate cover test).
Ipsilateral pursuit may be affected by parietal lesions (Poor OKN with the stripes moving to the same
side of the lesion).
Each frontal lobe initiates contralateral saccades.
Visual hallucinations →Unformed with occipital lobe damage, formed with temporal lobe lesions.
Some notes about Phakomatosis
 Seizures → Bourneville syndrome
 Glaucoma→ Sturge–Weber syndrome
 Retinal detachment → von Hippel–Lindau syndrome (serous exudation)
 Astrocytic hamartomas → Tuberous sclerosis (Bourneville syndrome) or NF-1 (Von Recklinghausen
disease).
 Pheochromocytomas → von Hippel–Lindau syndrome
 Thymic aplasia → Louis–Bar syndrome
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Adrenaline 1:1000 (or phenylephrine 1%) → in Contrast to hydroxyamphetamine, if dilata on → 3 order
neuron Horner’s (Post-ganglionic)
Palinopsia refers to persistence of images after their removal.
Unilateral retrogeniculate lesions do not lead to cortical blindness, Must be Bilateral.
Patients with papillophlebitis have normal or near normal visual acuity.
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Uhthoff symptom occurs with optic neuritis and is a decrease in vision with an increase in body
temperature.
Lhermitte sign is the electric shock sensation with neck flexion and is found in patients with multiple
sclerosis.
prosopagnosia, the inability to distinguish faces, caused by bilateral medial inferior occipitotemporal
lesion
Riddoch phenomenon occurs in patients with ‘cortical blindness’ who are able to perceive objects in
motion, but cannot see stationary objects.
Papilledema, although commonly present in 90% of patients with pseudotumor cerebri (idiopathic
intracranial hypertension), is not necessary for the diagnosis.
DD of BL motility deficit + pupil involvement includes Guillain–Barré syndrome and botulism. In Guillain–
Barré syndrome Lumbar puncture may reveal elevated protein, and anti-GQ1b antibodies are often
present.
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DD of disorders with blind spots + positive photopsias includes AZOOR (Acute Zonal Occult Outer
Retinopathy), autoimmune retinopathy such as cancer-associated retinopathy and melanoma associated
retinopathy, as well as some of the white dot retinal syndromes. ERG is needed to confirm the diagnosis.
Optic disc drusen may be caused by:
 idiopathic (often familial in an AD pattern)
 pseudoxanthoma elasticum
 angioid streaks
 retinitis pigmintosa
 Alagille syndrome
Bilateral sixth nerve palsy → stretched against the cilvus (increased intracranial pressure)
Frey's syndrome → aberrant regenera on of the 9th cranial nerve that may occur a er trauma or
tumor, with synkinesis between salivation fibers and sympathetic fibers. This causes flushing and
sweating when eating.
Migraine prophylaxis → Beta-blockers.
Acute migraine attack → Sumatriptan.
Optic disc hypoplasia can be associated with:
 septo-optic dysplasia (de Morsier's syndrome)
 aniridia
 fetal alcohol syndrome
 maternal exposure to: LSD, quinine, and phenytoin
Non-dominant parietal lobe lesions cause:
 hemispatial neglect
 difficulty with spatial orientation
 dressing apraxia
 object agnosia (or astereoagnosia: unable to name object in hand with eyes closed)
Dominant parietal lobe lesions cause: (Gerstmann syndrome the 1st 4 constitute)
 dyscalcula
 dysgraphia
 left-right disorientation
 finger agnosia
 alexia
 speech disturbance
Retrochiasmal visual disorders:
 alexia with agraphia: dominant parietal lobe (angular gyrus)
 alexia without agraphia: dominant occipital lobe and corpus callosum
 hemi-alexia: corpus callosum
 prosopagnosia: bilateral inferior medial occipito-temporal junction
 formed hallucinations: temporal lobe
 unformed hallucinations: occipital lobe
 Balint syndrome (bilateral superior parieto-occipital lesions):
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- inability to perceive the visual field as a whole (simultanagnosia)
- difficulty in fixating the eyes (oculomotor apraxia)
- inability to move the hand to a specific object by using vision (optic ataxia)
Synoptophore → a test for subjec ve angle of devia on
All MPS may cause Keratopathy (comprises punctate corneal opacification and diffuse stromal haze)
except Hunter and Sanfilippo.
Pulfrich phenomenon reflects delayed conduction in the demyelinated nerve and occurs where
oscillating objects viewed binocularly and moving in one plane (e.g. pendulum) appear to have threedimensional movement.
Hallucinations and deja vu experiences → temporal lobe lesion
Ant. communicating artery → compress the upper por on of chiasma > bitemporal lower
quandrantanopia.
LHON :
• 10% of female carriers are affected
• 50% of male carriers are affected
10% of patients with MG have thymomas visible on CT scanning.
Surprisingly → 80% of MG pa ents without thymoma had remission of disease a er thymectomy. This
compares to only 10% of patients with thymoma having remission of MG after thymectomy. (Eyedocs !!)
Dysthyroidism is more common in patients with myasthenia gravis.
5% of MG patients develop Graves disease
75% of all myasthenics will have eye findings at presentation, but only 33% to 50% will have ocular
myasthenia only at presentation.
Up to 90% of MG patients will develop ocular symptoms at some point during the course of the disease.
ACh receptor binding antibodies are found in 90% of patients with generalized myasthenia and in
approximately 50% of patients with ocular myasthenia.
When a patient has isolated ocular myasthenia gravis for more than 2 years, there is a less than 20%
chance that he or she will develop systemic disease.
Optic nerve meningioma → more likely to be aggressively malignant in children.
Optic nerve glioma → malignancy are rare (more likely in adults than in children)
Von Hippel Lindau → retinal capillary haemangioma. (Happeel Retains a Cap)
Sturge-Weber syndrome → choroidal cavernous haemangioma. (Strange Car in the cave)
Cogan's anterior INO → impaired convergence (Kanski and Oxford > intact if anterior)
The order of signal intensity in MRI is as follows:
 T1 weighted: fat > white matter > grey matter > CSF
 FLAIR: fat > grey matter > white matter > CSF
 T2 weighted: CSF > grey matter > white matter > fat
 STIR: CSF = grey matter > white matter > fat
Meningothelial meningioma is the most common histopathologic type of meningioma seen within the
orbit.
The Kestenbaum procedure is a bilateral resection/recession that surgically moves the eyes toward the
direction of the head turn to dampen the nystagmus.
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The Kestenbaum index is the number of capillaries counted on the optic disc, which normally is around
10. Less than 6 capillaries indicates optic atrophy; more than 12 suggests disc hyperaemia. In nerve
transaction, the severed end produces bulbous axonal swellings (Cajal end bulbs).
A contralateral RAPD would be present if the optic tract was damaged because relatively more nasal
fibers from the contralateral eye cross in the chiasm. Approximately 52% of the optic nerve axons cross
in the chiasm, whereas 48% remain ipsilateral.
The presence of an RAPD without any visual loss suggests damage to the contralateral brainstem.
(Damage to the pupillomotor fibers after they separate from the visual fibers in the midbrain can result
in a small contralateral RAPD without visual loss because of the asymmetric decussation in the chiasm.)
The only retro-chiasmal lesion of the visual pathway to produce a monocular field defect is a lesion of
the anterior-most occipital lobe → Contralateral far temporal field defect ‘temporal crescent’ (from 60
to 90 degree, which is perceived only by the nasal-most retinal periphery of the ipsilateral eye)
Nerve fibres in the optic tract are rotated 90 degrees with inferior fibres located laterally.
Essential blepharospasm → basal ganglia dysfunc on.
Which contralateral EOM is LEAST affected as a result of a compressive third nerve palsy → inferior
rectus
Uniocular Pie in the Sky → contralateral anterior chiasmal lesion.
Communicating hydrocephalus (non-obstructive) is caused by impaired CSF reabsorption in the absence
of any CSF flow obstruction between the ventricles and subarachnoid space (functional impairment of
the arachnoid granulations ‘the site of CSF re absorption’ caused by subarachnoid haemorrhage,
meningitis and congenital absence of arachnoid villi).
Non communicating (obstructive) is caused by CSF flow obstruction due to external compression or
intra-ventricular mass lesions (e.g. Colloid cyst).
The commonest defect of colour vision is deuteranomaly which occurs in 5% of men and 0.3% of
women. (X-linked)
Visioscopy → is a test for eccentric fixa on.
The most common cranial mononeuropathy in (MS) is optic neuritis, while The most frequently affected
motor nerve is the sixth nerve.
Red-green defects: acquired optic nerve disease (except glaucoma and autosomal dominant optic
atrophy)
Blue-yellow defects: acquired retinal disease (except cone dystrophy and Stargardt's)
A binasal hemianopsia is almost never due to chiasmal compression and is usually the result of
glaucoma.
A binasal field defect may be due to:
 Retina
o atypical RP
o schisis
o vascular occlusion (2 vessels)
o juxtapapillary retinochoroiditis
o myopia with peripapillary atrophy
 ON Head
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o glaucoma
o disc drusen
o chronic papilloedema
 Chiasm (rare)
o tumor
o aneurysm
Headache + transient visual obscuration + papilledema → Management in this sequence;
1- Measure Blood pressure to exclude Malignant Htn first
2- Neuroimaging to exclude brain lesion causing intracranial Htn
3- Lumbar Puncture for Idipathic intracranial Htn
Maroteaux-Lamy and Morquio → accumula on of dermatan sulphate, causes corneal clouding and
optic atrophy.
Acromegaly is associated with: Acrom
 A >> Angoid streaks
 C >> Chiasma
 R >> Retinopathy (DM,Htn)
 O >> OA
 M >> enlarged EOM
Anhidrosis may or may not be present in Horner’s $ and can assist localisation:
 First-order lesions (CNS) → ipsilateral anhidrosis of the en re body
 Second-order lesions (Pancoast's tumor, neck trauma) → ipsilateral facial anhidrosis
 Third-order lesions → anhidrosis only around the affected eye or none at all.
Leber's congenital amaurosis is autosomal recessive.
Leber's optic atrophy is mitochondrially inherited.
5) Pediatric Ophthalmology and Strabismus
From Exams
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Regarding Neuroblastoma:
- Children presenting with opsoclonus have a good prognosis
- The adrenal gland is the most common site of origin
Rate Toxoplasmosis fetal transmission (in contrast to severity)
- less than 6% in the 1st trimester.
- 60-81% in the 3rd trimester.
Signs of Orbital cellulitis + Black discoloration of the nasopharynx >> Rhino-Orbital Mucormycosis
Periventricular leukomalacia >> commonly associated with Optic disc cupping **
Plus disease in ROP >>Significant Venous engorgement and Arterial tortuosity
The 4Δ base-out prism test is the least reliable method of documenting a central suppression
scotoma:
- Patients with bifixation may recognize diplopia when the prism is placed before an eye but make no
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convergence movement to correct for it.
- Patients with monofixation may switch fixation each time the prism is placed and show no version
movement, regardless of which eye is tested.
A patient with monofixation syndrome may have no manifest deviation but usually has a small (≤8
prism diopters [Δ]) heterotropia; the heterotropia is most commonly an esotropia but is sometimes an
exotropia or hypertropia. Stereoacuity is present but reduced. Amblyopia is a common finding.
Alternate prism cover test measurement will EXCEED simultaneous prism cover test.
Any amount of gross stereopsis confirms the presence of peripheral fusion. Most patients with
monofixation syndrome demonstrate stereopsis of 200–3000 seconds of arc.
If associated amblyopia is clinically significant, occlusion therapy is indicated. Monofixation may
decompensate to a larger heterotropia in adulthood, resulting in diplopia. Strabismus surgery may be
required to restore fusion.
The AC:A ratio is determined based on the distance and near measurements, whereby if the near
esotropia is greater than 10 prism diopters, the AC:A ratio is considered high.
Latent nystagmus (LN) is seen only when one eye is covered and the fast phase beats towards the side
of the fixing eye. This means that the direction of the fast phase reverses according to which eye is
covered.
Manifest latent nystagmus (MLN) is the same except that nystagmus is present with both eyes open,
but the amplitude increases when one is covered.
Cyclic esotropia is a very rare condition characterized by alternating manifest esotropia with
suppression and BSV (Stereopsis is excellent on days when eyes are straight), each typically lasting 24
hours. The condition may persist for months or years and the patient may eventually develop a constant
esotropia requiring surgery (Surgical correction of the full angle of deviation)
The best long term cosmetic result following surgery for an intermittent exotropia is → 10 prism
(Dioptre) overcorrection (induced esotropia).
A small-angle esotropia in the immediate postoperative period tends to resolve and is desirable because
of its association with a reduced risk of recurrent exotropia. Patients may experience diplopia while
esotropic, and they should be advised of this possibility preoperatively. AAO
Infantile (congenital) esotropia → accompanied by DVD (50-90%), IOO (70%), latent nystagmus (40%),
and optokinetic asymmetry “reduced towards temporal = T to N > N to T”. (AAO)
Ideally, the eyes should be surgically aligned by the age of 12 months, and at the very latest by the age
of 2 year. (Kanski) Surgery can be performed in healthy children as early as age 4 months. (AAO)
A ‘V’ pattern is said to be significant when the difference between upgaze and downgaze is ≥15 Δ.
Esotropia + BL IOO (V-Pattern ) → BMRR + IO weakening procedure
V-Pattern Casued by IOO → IO weakening procedure
If V-pattern without IOO:
- Eso → BMRR + downward transposi on of MR tendon
- Exo → BLRR + upward transposi on of LR tendon
The sequelae of a paretic squint are:
 Primary underaction (e.g. left superior oblique palsy)
 Overaction of yoke muscle by Hering's law (e.g. right inferior rectus)
Abdullah Fouad Notes
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 Secondary contracture of unopposed antagonist (e.g. left inferior oblique)
 Secondary inhibitional palsy of the Yoke muscle's antagonist by Sherrington's law (e.g. right superior
rectus)
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Faden (posterior fixation suture) → weakening the muscle without affecting it in the 1ry position.
Inferior rectus muscle paresis with diplopia in downgaze following blowout fracture of the orbit or
operative trauma → can be treated by Contralateral inferior rectus recession with a posterior fixation
suture.
Pulling the inferior rectus muscle forward, as in a resection → advances the lower eyelid upward (Lower
eyelid elevation); recessing this muscle pulls the lower eyelid down, exposing bare sclera below the
lower limbus. Surgery on the superior rectus muscle is somewhat less likely to affect upper eyelid
position. Release of the lower eyelid retractors or advancement of the capsulopalpebral head is helpful
to prevent lower eyelid retraction after inferior rectus muscle recession.
The most appropriate type of needle for suturing the sclera in strabismus surgery → Micro-spatulated
(risk of perforation is lower)
(The two basic types of needles are vertical cutting (See Figure 11A) and side cutting or spatulated (See Figure 11B). The
preferred side cutting or spatulated needle has a flat top and bottom, thereby reducing the chance of inadvertent perforation
deep into the globe or the roof of the scleral tunnel above. The vertical cutting needle, with the sharp pointed up or down, can
either penetrate too deep or cut the roof of the scleral pass above)
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The risk of occurrence of the oculocardiac reflex may be decreased during strabismus surgery by IV
atropine or glycopyrolate (muscarinic anticholinergic).
Duane surgery → Unilateral or bilateral muscle recession or transposi on of the ver cal rec are the
procedures of choice. The lateral rectus of the involved side should not be resected, as this increases
retraction.
Chronic oculomotor (3rd) nerve palsy → contralateral lid retrac on (Hering’s law)
MG → some mes transient retraction of the upper eyelids which usually followed prolonged upgaze
and which persisted for many seconds. This could result from post-tetanic facilitation, myotonia, or a
transient contracture.
Congenital fibrosis of the extraocular muscles (CFEOM) → Deficits of ver cal eye movements, especially
upgaze, are a hallmark of the condition, and patients’ eyes are often stuck in infraduction, make eyes at
risk for corneal exposure, particularly after ptosis surgery. (AAO)
Möbius syndrome → although its associated with lagophthalmos, cornea is intact since usually vertical
movements are preserved, preventing it from exposure.
6) Orbit, Eyelid and Lacrimal diseases
From Exams
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Distichiasis mainly affects the lower lid.
- The majority of patients also manifest primary lymphoedema of the legs (lymphoedema– Distichiasis
syndrome).
- Cryotherapy → effective but carries a high rate of local adverse effects, and is less commonly
performed than previously
- Electrolysis → frequently multiple treatments are required to obtain a satisfactory result.
Regarding Non-Hodgkin Lymphoma of the orbit and ocular adnexa >>They are usually B-Cell lymphoma
Upper lid retraction caused by thyroid eye disease can be treated with recession of levator aponeurosis
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Necrotizing fasciitis → a rapidly progressive disease of the subcutaneous tissue that carries a high
mortality (30%) within hours to days without prompt medical and surgical interventions, usually due
to toxic shock syndrome, but this occurs less commonly in the periocular region. It is usually caused by
Group A beta-Hemolytic streptococci (Streptococcus pyogenes), and occasionally by S. aures.
Clindamycin is of particular value as it is uniquely effective against the toxins produced by group A
streptococcus.
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MC orbital tumour in adults → Cavernous Haemangioma
MC Malignant orbital tumour in adults → Lymphoma
MC Orbital Metastasis in adults → Metastases from breast, lung then prostate
MC intraocular tumour in adults → Uveal (MC Choroidal) melanoma
MC Intraocular Metastasis in adults → Metastases from Ca lung and breast
MC orbital tumour in children → Dermoid cyst, then Capillary Haemangioma
MC Malignant orbital tumour in children → Rhabdomyosarcoma
MC Orbital Metastasis in children → Neuroblastoma
MC Intraocular tumour in children → Retinoblastoma
Epicanthus palpebralis. Skin folds are symmetrically distributed between the upper and lower eyelids.
The most common among Caucasians
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Epiblepharon
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Ankyloblepharon
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Blepharochalasis is a disease of young age, dermatochalasis is a disease of old age.
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Blepharochalasis → recurrent episodes of painless, non-pitting oedema of both upper lids which usually
resolves spontaneously after a few days.
Blepharochalasis → Although the average duration of attack is only two days, multiple attacks
eventually lead to atrophic, wrinkled, and discolored periorbital skin. ptosis secondary to dehiscence
or attenuation of the levator aponeurosis
Blepharoptosis = Ptosis.
Necrobiotic xanthogranuloma (NXG) → a potentially destructive local course. Associated with paraproteinemia and multiple myeloma (MM). (AAO)
Nevi are the third most common benign lesions encountered in the periocular region (after papillomas
and epidermal inclusion cysts). They arise from nevus cells, which are incompletely differentiated
melanocytes in the epidermis and dermis and in the junction zone between these 2 layers.
Ephelides, or freckle is a small, brown spot on the skin, the malar areas, eyelids, or the conjunctiva.
Ephelides arise from hyperpigmentation of the basal layer of the epidermis. The number of epidermal
melanocytes is not increased, but they extrude more than the usual amount of pigment into the
epidermal basal cell layer.
Junctional nevi are flat or slighly elevated and are comprised of nests of nevus cells in the basal
epithelial layer. They have the greatest potential for malignant transformation to melanoma by
comparison to intradermal nevi and compound nevi.
Other types of pigmented lesions:
- Nevus of Ota: diffuse congenital deep dermal nevus of the periocular skin
- Blue nevus: deeply located dermal nevus present at birth with very little elevation.
- Spindle cell nevus: compound nevus of childhood with bizarre cellular components but no malignant
potential.
Congenital nevi have a 5-10% lifetime risk of transformation to melanoma.
Xeroderma pigmentosum is characterized by skin damage on exposure to sunlight, leading to
progressive cutaneous abnormalities. It is inherited in an autosomal recessive (AR) fashion. Affected
patients have a bird-like facies and a great propensity to the development of basal cell carcinoma
(BCC), squamous cell carcinoma (SCC). It is due to a decreased ability to repair DNA damage such as
that caused by ultraviolet (UV) light.
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BCC → on the lower eyelid margin (50%–60%) and near the medial canthus (25%–30%). Less
commonly, they may occur on the upper eyelid (15%) and lateral canthus (5%). AAO
BCC is the most common human malignancy and typically affects older age groups. The most important
risk factors are fair skin. Kanski
The most common types of BCC in order is:
1) Nodular
2) Superficial spreading
3) Infiltrative
BCC →The pigmented type most commonly affects people of pigmented races.
Gorlin and Goltz's eponymous syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS)
→ AD disorder distinguished by multisystemic developmental abnormalities 2ry to mutations in the
patched-1 (PTCH1) gene.
Orbital invasion of BCC → Orbital exenteration
Tumours of the medial canthus tend to be more deeply invasive, and the involved structures (e.g.
nasolacrimal system) are more difficult to be freed of tumour.
The hedgehog signalling pathway (HH pathway) influences the differentiation of various tissues during
fetal development. In adults, it continues to function in the regulation of cell growth and
differentiation.Malfunctioning of this pathway is associated with human malignancy, including BCC.
Vismodegib (trade name Erivedge™) is a hedgehog pathway inhibitor that was approved in 2012 for
the treatment of advanced and metastatic BCC.
In 2015, sonidegib was also approved for the treatment for advanced basal cell carcinoma.
Dyskeratosis → keratinization other than on the epithelial surface.
Hyperkeratosis → Thikening of the keratin layer that appears clinically as scaling or white, flaky lesion
(leukoplakia). It can be a feature of benign or malignant epithelial tumours.
Parakeratosis → thickening of the keratin layer with retention of nuclei; indicates shortened epidermal
regeneration time; granular layer is thin or absent.
Acanthosis → thickening of the sqamous cell layer due to proliferation of prickle cells.
In full thickness eye lid defect:
- Small (<1/3 eyelid) → Direct closure “+/- Canthotomy and Cantholysis”
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- Moderate (33-50% eyelid) → Canthotomy + Tenzel semicircular rotational flap (C-shaped myo
cutaneous flap)
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- Large Defect (>50% eyelid) → Either Eyelid sharing “Cutler-Beard (Bridge) procedure” for upper lid
and “Hughes” procedure for lower lid or Mustarde cheek rotational flap for very large defect.
Immunocompromised patients are at increased risk of SSC.
Sebaceous gland carcinoma (SGC) is a highly malignant and potentially lethal tumor, the tumor within
the tarsal plate tends to progress to an intraepidermal growth phase, which may extend over the
palpebral and bulbar conjunctiva.
- A fine papillary elevation of the tarsal conjunctiva (not extra epidermal) may indicate pagetoid spread
of tumor cells.
- Wide surgical excision is mandatory for adequate treatment of sebaceous adenocarcinoma.
- Orbital exenteration may be considered for recurrent or large tumors invading through the orbital
septum.
- Sentinel lymph node (SLN) biopsy is considered for patients with eyelid sebaceous cell carcinoma
with high-risk features (recurrent lesions or extensive involvement of the eyelid or orbit)
- Sebaceous carcinomas are relatively radioresistant.
Kaposi sarcoma is highly radiosensitive tumours.
Management of trachomatous trichiasis in endemic regions → Bilamellar tarsal rotation with either
absorbable or a nonabsorbable suture.
Treatments for trichiasis and recurrence: (NCBI)
- Cryotherapy → 90% success and the least recurrence, but significant risk of complications
- Argon laser → 67.9% successful with no evidence of recurrence
- Electrolysis → me consuming, suitable only for isolated eye lashes with a high recurrence rate.
- Epilation → the simplest method, not suitable for a large number of lashes and the lashes tend to
regrow within few weeks.
Collarettes of staphylococcal blepharitis → fibrinous crust.
A frequent complaint of patients with aponeurotic ptosis is worsening of the ptosis when they look
down because the eyelid has no opposition on downgaze.
Ptosis → restriction of both the upper and lower visual fields.
Dermatochalasis → upper field but not lower field defect.
Muller muscle-conjunctival resection is indicated for small degrees of involutional ptosis.
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Bell’s palsy → with no treatment 85% show first signs of recovery within 3 weeks of the onset.
They’ve found evidence that suggests HSV, HZV, EBV, adenovirus, rubella, mumps virus, influenza B and
coxsackievirus. The paralysis may be on partial half of face or completely according severity.
The fluorescein disappearance test is performed by instilling fluorescein 1 or 2% drops into both
conjunctival fornices; normally, little or no dye remains after 5–10 minutes (based on the amount of
dye still present in the conjunctival meniscus). Prolonged retention is indicative of inadequate lacrimal
drainage.
Infection of the lacrimal sac is usually secondary to obstruction of the nasolacrimal duct.
Diffuse acquired bilateral canalicular scarring in an adult:
- Systemic Chemotherapy (Answer sheet 2018)
- Ocular cicatricial pemphigoid (OCP)
The viruses of primary herpes simplex, herpes zoster, chicken pox, and vaccinia may produce
inflammatory changes in the epithelial lining of the lacrimal canaliculi, resulting in varying degrees of
obstruction. Early recognition and intubation can often prevent permanent canalicular obstruction, but
late cases may require the Jones tube procedure, Conjunctivo-DCR.
In general, HPV is thought to be responsible for more than 90% of anal and cervical cancers, about 70%
of vaginal and vulvar cancers, and more than 60% of penile cancers
The most likely source of significant epistaxis following DCR → Anterior ethmoidal artery.
Regarding congenital obstruction of the NLD:
- 20 % of infants, 1/3 is have BL involvement. Most of them resolve spontaneously within Ws or Ms.
- Persist in 2%–6% of full-term infants at 3–4 weeks of age.
- 90 % of cases improve with 1st probing and more than half of reminder by 2nd one (kanski).
- Congenital dacryocystocele is initially sterile (usually the Skin is not inflamed and hot).
- The optimal age for probing congenital NLD obstruction → 6-13 months.
The most common cause of hypothyroidism in developed countries → Hashimoto thyroiditis.
The most common cause of hypothyroidism worldwide → inadequate dietary intake of iodine.
Graves autoimmune disease → male-to-female ratio is 1:5-10 (Medscape) (1:10 answer sheet 2017).
Grave's Orbitopathy → The extra-ocular muscles are infiltrated with lymphocytes, plasma cells and
mast cells. The orbital tissues are increased in volume, mostly by an increase of Extracellular matrix.
Orbital decompression for thyroid Orbitopathy was shown to improve diplopia in 28.1% of patients;
however, the surgery can induce diplopia in 29.7% of patients. Opening the periorbita is associated
with increased new-onset diplopia. (NCBI) worsening of pre-existing diplopia (answer sheet 2018).
Thyroid optic neuropathy may occur in the absence of signifcant proptosis.
Immunoglobulins that activate insulin-like growth factor I (IGF-I) receptor (IGF-IR) signaling have been
detected in patients with Graves’ disease. Insulin like growth factor receptor (IGF-1R) is expressed at
higher levels in the orbital connective tissue.
In a study of visual field changes in thyroid eye disease-compressive optic neuropathy (TED-CON) →
Fifty-three of 96 visual fields (55%) showed an inferior defect.
(https://www.ncbi.nlm.nih.gov/m/pubmed/28453366/)
Dyschromatopsia may be an early sign of thyroid optic neuropathy, visual field testing shows central or
diffuse depression .ONH is commonly normal but may be mildly edematous. (AAO)
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The cytoplasmic pattern, or c-ANCA, is both sensitive and specific for Wegener granulomatosis and is
present in up to 95% of patients; proteinase 3 is the most common target antigen.
Dermoid cyst → should be surgically removed at ages 1–2 as they may enlarge and lead to
deformation in orbital bony structures and erosion. It may rupture spontaneously.
Dermoids may be superficial or deep, located anterior or posterior to the orbital septum respectively.
Hypoglycaemia can be an adverse effect of treatment with propranolol.
Capillary haemangioma → Natural history shows rapid progression in the 1st 3 months of life followed
by resolution in 30% by age of 3 years and 75% by the age of 7 years. (Remember 73 war)
Histology shows benign proliferation of vascular endothelial cells. Approximately 1 in 10 children
(10%) are affected by capillary hemangiomas of varying severity. Females: Males = 3: 1.
Injection of triamcinolone acetonide (1–2 ml total of 40 mg/ml over several injection sites) or
betamethasone (4 mg/ml) into a cutaneous or preseptal tumour is usually effective in early lesions.
Regression usually begins within 2 weeks but, if necessary, second and third injections can be given
after about 2 months
Pyogenic granulomas are thought to be a form of capillary haemangioma (lobular capillary
hemangioma ).
There are rare reports of spontaneous regression of optic nerve and visual pathway gliomas.
High grade tumors (grade IV astrocytomas / Glioblastoma Multiforme) rarly involve optic nerve .(AAO)
Nystagmus may be the presenting feature of optic nerve glioma. (answer sheet 2019)
Optic nerve gliomas peak incidence in childhood (age 2-6 years), presenting in adulthood tend to be
particularly malignant; this applies to isolated tumours unassociated with neurofibromatosis. Gliomas
related to neurofibromatosis generally have a better prognosis in both adults and children. Note:
meningiomas are opposite to gliomas in that meningiomas presenting in childhood tend to be more
aggressive. Eye doc
To distinguish Orbital apex syndrome from superior orbital fissure → the main differentiator is optic
nerve involvement, since both syndromes involve CN III, IV, VI, and the ophthalmic division of CN V
(CNV-1). Superior orbital fissure syndrome accompanied by an ipsilateral posterior ischemic optic
neuropathy from dissection and an occlusion of the internal carotid artery can be mistakenly diagnosed
as orbital apex syndrome.
Spindle cells in Antoni A and B tissue patterns is charachterestic of Schwannoma which is found in NF2.
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EBV is associated with Burkitt’s lymphoma, nasopharyngeal carcinoma, and some forms of Hodgkin’s
disease. EBV can readily infect and alter the genetic code of human B cells, and may predispose
immunosuppressed patients to malignant tumors.
Regarding Ataxia-Telangiectasia “AT” (Louis-Bar Syndrome) the increased susceptibility to various
malignancies, particularly lymphomas and leukemias, contributes to early mortality in one third to
one-half of cases.
Unpredictable fat resorption is a serious drawback to the autologus dermis-fat graft (ADG) for volumedeficient sockets after enucleation in adults.
OTX2 mutations result in anophthalmia/microphthalmia (A/M) often associated with systemic
anomalies.
During the first few months of life left orbital volume is on average 15 cm3 in male and 13 cm3 in
female infants; these volumes increase to 26 cm3 and 24 cm3, respectively, by the time the child
reaches 15 years of age.
Inflatable orbit expanders may reduce requirement for repeated general anesthesia exposure.
If familial ,the typical inheritance pattern of Apert , Crouzan , Pfeiffer, and Treacher Collins cranio facial
syndrome is → AD.
Malposition of the rectus muscle pulleys can lead to anomalous movements that can simulate
oblique muscle overactions. This can be seen in craniofacial syndromes. For example, an inferiorly
displaced lateral rectus muscle pulley can cause depression in abduction or, if this is the fixating eye,
OEAd of the contralateral eye and a V-pattern deviation that simulate inferior oblique muscle
overaction. Conversely, a superiorly displaced lateral rectus muscle can produce ODAd—simulating a
superior oblique overaction—along with an A-pattern deviation. Lateral and medial malpositioning of
vertical rectus muscles can also create pseudo-overactions of oblique muscles. (AAO)
The most common ophthalmic manifestations of Crouzon syndrome are proptosis secondary to
shallow orbits in about 100% of cases, exotropia, exposure keratopathy, hypertelorism, and optic
atrophy secondary to chronic papilledema. Other ophthalmic manifestations may include globe
subluxation, ametropia, amblyopia and nystagmus. Congenital glaucoma secondary to closed angles
and FGFR2-related anterior segment dysgenesis has been reported. Many of the craniosynostosis
syndromes are caused by mutations in the Fibroblast growth factor receptor (FGFR1, FGFR2, and
FGFR3 genes).
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Le Fort I is a low transverse maxillary fracture above the teeth with no orbital involvement.
Le Fort II fractures involve the nasal, lacrimal, and maxillary bones as well as the medial orbital floor.
Le Fort III fractures cause craniofacial disjunction in which the entire facial skeleton is completely
detached from the base of the skull and suspended only by soft tissues. The orbital floor and medial
and lateral orbital walls are involved.
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Some wooden orbital foreign bodies may be missed on CT and are seen better on MRI.
Regarding orbital fracture repair → transcaruncular approach to the medial wall will avoid the
complication of postoperative lower lid retraction.
Calvarial (the top part of the skull) graft represents a notable exception to the usual rule of late
remodelling resorption seen in other corticocancellous bone grafts. This is due to diploic vascular
system of this bone.
The incidence of traumatic optic neuropathy (TON) in the setting of a closed traumatic head injury in
various studies ranges from 0.5-5%. (mesdscape)
Spontaneous visual improvement occurs in up to about half of indirect injury patients.
Waardenburge syndrome (WS) → AD, patchy areas of depigmentation of the hair and skin in piebaldlike distribution, heterochromia, broad nasal root, congenital deafness (20%). An estimated 2 to 35%
of all congenital deafness results from WS.
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7) Trauma, External Eye diseases and Cornea
From Exams
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Superior limbic keratoconjunctivitis (SLK) is associated with abnormal thyroid function (usually
hyperthyroidism)
Natamycin "Polyene" → Filamentous fungi (Fusarium and Aspergillus).
Amphotericin → Aspergillus and Candida.
Miconazole → Paecilomyces lilacinus.
Flucytosine → an adjunc ve agent for treatment of Candida spp.
Fusarium Keratitis → Responds well to Imidazole antibiotics e.g. Fluconazol (answer sheet 2019&2017)
Histoplasmosis may be a cause of fungal keratitis (Case reports in india).
https://www.sciencedirect.com/science/article/pii/S2452403416300115
Miconazole is effective against yeast and filamentous.
http://www.scielo.br/scielo.php?pid=S0034-72802013000200014&script=sci_arttext&tlng=en
Terrien Marginal Degeneration >> more in Men (75%). (Kanski)
Typically affects 20-40 years patients (AAO). Onset after the 4th decade. (Answer sheet!!!).
Typically begins superiorly. (AAO)
Skin Scarring is a characteristic clinical feature to distinguish HZ Keratitis from HS Keratitis
Rheumatoid Arthritis causes dry eye syndrome by altering the functions of the lacrimal glands.
Trachoma is more common in females
In Salzmann nodular degeneration >> hyaline material deposits in Bowman layer
SJS >>Type IV immune-complex reaction
Classically, the most common cause of neonatal conjunctivitis is due to post-delivery use of ophthalmic
silver nitrate used in the prophylaxis of ocular gonococcal infections
Chlamydial conjunctivitis (C. trachomatis serotypes D-K) is the most common infective cause of
neonatal conjunctivitis.
Neonate tears also lack immunoglobulin IgA
Neonatal Conjunctivitis:
- Chemical conjunctivitis (Typically presents within first 24 hours following birth)
- Neisseria gonorrhea (3-5 days after birth)
- C. trachomatis (5-14 days)
- HSV (1-2 weeks)
Collarettes of staphylococcal blepharitis > fibrinous scales.
Moraxella lacunata > Angular Blepharitis
Pseudo-epihheliomatous hyperplasia > is found in Keratoconus and Keratocanthoma
Goldenhar syndrome (First branchial arch abnormalities) > triad of epibulbar choristomas (Dermoid),
preauricular skin appendages, and pretragal blind ending fistulas in association with mandibular facial
dysplasia.
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Dermoids or dermolipomas are more likely to be associated with Goldenhar’s Syndrome if they are
multiple or bilateral.
Dermoid > associated with Hemifacial atrophy.
Complex choristomas combine features of multiple types of choristomas, for example, dermoid or
lipodermoid plus osseous choristoma.
Epibulbar dermoids are classified into three grades:
- Grade I are lesions with a superficial tumor measuring <5 mm.
- Grade II are of larger size and extend into the corneal stroma down to Descemet's membrane.
- Grade III involves the whole cornea and structures of the anterior chamber.
Ocular surface squamous neoplasia (OSSN) involving the cornea is considered invasive squamous cell
carcinoma when it penetrates Bowman membrane (which is thought to be a barrier for tumor
invasion).
The risk of conjunctival melanoma is greatest with Primary acquired melanosis (PAM). 75% of
conjunctival melanomas arise in association with PAM.
PAM with atypia will have a 50% chance of progressing to a melanoma.
PAM is usually unilateral or asymmetric if bilateral, and is most often seen in light-skinned individuals.
A pterygium larger than 3 mm may induce some astigmatism, and intervention may be warranted in
such a case. If larger than 3.5 mm (more than halfway to the center of the pupil in a typical cornea) are
likely to be associated with more than 1 D of astigmatism
The Tear breakup time (TBUT) is recorded as the number of seconds that elapse between the last blink
and the appearance of the first dry spot in the tear film, as seen in this progression of these slit lamps
photos over time. A TBUT under 10 seconds is considered abnormal.
Schrimer test
- Without anesthetic (schrimer 1) measure basic and reflex secretion > less than 10 mm of wetting after
5 minutes is considered abnormal
- With anesthetic (schrimer 2) measure basic secretion > less than 6 mm after 5 minutes is abnormal
Sjögren's syndrome > can occur at any age, but is most common in older women.
Episcleritis > Topical or oral NSAID
Scleritis:
- Mild to moderate > Oral NSAID
- Sever or Necrotizing or not responding to NSAID > Oral Corticosteroid
Oculodermal melanosis (Nevus of Ota), also known as oculodermal melanocytosis, oculomucodermal
melanocytosis, or congenital melanosis bulbi. > Involve conjunctiva and Sclera. It is caused by failure of
migration of melanocytes from neural crest cells to their normal location within the basal layer of the
epidermis.
Ocular Melanocytosis > Sclera and episcleral. (Congenital). The overlying conjunctiva is mobile over the
episcleral pigmentation but the pigmentation itself is intrinsic and cannot be moved over the globe.
Intracranial and uveal melanomas can occur in aevus of Ota (oculodermal melanosis).
Dexamethasone is a more potent corticosteroid (six times that of prednisolone). However, since
dexamethasone is made available to the ophthalmologist in a 0.1 % formulation, while prednisolone is
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available as a 1.0 % formulation, the tenfold difference in concentration seems to give prednisolone a
slight edge.
Among the corticosteroid formulations studied to date, prednisolone acetate 1.0 % ophthalmic
suspension is the most effective topical anti-inflammatory agent when the epithelium of the inflamed
cornea is intact. It must be re-emphasized that because of differences in the aqueous solubility of these
compounds and because of the nature of the lipophilic barrier of the corneal epithelium, the relative
order of corneal anti inflammatory potency among these drugs may differ when the epithelium is not
intact.
Both (Dexamethasone alcohol 0.1% and prednisolone acetate 1%) are more potent than Both
(Dexamethasone phosphate 0.1% and prednisolone phosphate 1%).
Dexamethasone alcohol 0.1% and prednisolone acetate 1% are potent corticosteroids, but the latter
achieves the highest aqueous concentration within 2 h and maintains higher levels for 24 h.
Golden Roles: (my own summary)
- Dexamethasone is 6 times more potent than Prednisolone.
- Prednisolone 1% is more potent than Dexamethasone 0.1%.
- Acetate or Alcohol preparations are more potent than phosphate.
In normal cornea, MHC class-2 expression in Langerhans cells.
The rate of corneal perforation in pseudomonas ulcers by approximately 50%.
Numerous efforts have been made to understand the molecular processes that result in the devastating
corneal damage that can occur in Pseudomonas keratitis. There are host factors associated with the
inflammatory response that can mediate tissue damage. The infiltration of the corneal stroma by
polymorphonuclear neutrophils (PMN) provides a source of damaging enzymes and reactive oxygen
species including peroxides, superoxide, hydroxyl radical, and singlet oxygen that damage normal tissue
[6]. The activation of the host matrix metalloproteinases (MMP) also contributes to the destruction of
corneal tissue [7]. These host factors are probably synergistic with the multiple bacterial products that
also attack the host.
https://www.google.com/url?sa=t&rct=j&q=&esrc=s&source=web&cd=3&ved=2ahUKEwjN8_K6rMvmA
hVp7XMBHfeVAB8QFjACegQIAxAC&url=https%3A%2F%2Fwww.mdpi.com%2F20762607%2F7%2F9%2F281%2Fpdf&usg=AOvVaw304OMw44b0oMCFclBNojZy
Anaerobic bacteria > Thioglycolate broth Media
Contact lenses > Hypoxia > Corneal NVs
The proliferation of organisms in bacterial keratitis occurs after the bacteria have invaded the underlying
stroma. (Interruption of an intact corneal epithelium and/or abnormal tear film permits entrance of
microorganisms into the corneal stroma, where they may proliferate and cause ulceration .Medscape)
Regarding the mycotic ulcer treatment trial (MUTT)
- one drop hourly while awake for first week followed by 2 hourly for next 2 weeks.
- Topical Natamycin 5% ‘compared to Voriconazole’ treatment resulted in better visual acuities at 3
months.
- The difference between the outcomes in the 2 treatment groups was accounted for by the Fusarium
subgroup. The difference in outcome in other species was not significant.
- Voraconazole treatment resulted in more corneal perforations.
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Several species of Acanthamoeba, including A. culbertsoni, A. polyphaga, A. castellanii, A. astronyxis, A.
hatchetti, A. rhysodes, A. divionensis, A. lugdunensis, and A. lenticulata are implicated in human disease.
Propamidine = Diamidines (Acanthamoeba TTT)
1ry HSV infection > Within hours, it enters the sensory nerve endings and travels to the sensory
ganglion.
25% of unilateral, non-epidemic follicular conjunctivitis are culture -positive for HSV.
There are 11 antiviral agents with proven efficacy against HSV. 3 are no longer manufactured
(idoxuridine, vidarabine, and brivudine), and the use of three others (valganciclovir, foscarnet, and
cidofovir) is limited by a poor safety profile. There are two topical (trifluridine and ganciclovir) and three
systemic (acyclovir, famciclovir, and valacyclovir) antiviral agents available and actively used for the
treatment of HSV epithelial keratitis in the United States.
HSV > the leading cause of infectious blindness in the developed world.
Profound loss of corneal sensation can result from a single episode of HZO, and these ulcers are more
likely to perforate. (AAO)
N.B. this may cause neurotrophic keratitis > wide central epithelial defect > lubricating ointment qid.
HZO Treatment options: AAO
- Oral acyclovir 800 mg po five times daily for 7 to 10 days is the standard treatment.
- Oral famciclovir 500 mg po tid for 7 to 10 days.
- Oral valacyclovir 1000mg po tid for 7 to 10 days.
Varicella-zoster immune globulin is used to prevent exposure of varicella virus in high risk individuals,
such as adults and children with weakened immune system.
Corneal scarring caused by herpes simplex keratitis (HSK) is the leading infectious cause of penetrating
corneal graft in high-income countries.
Prophylactic oral antiviral therapy following PK for HSV > systemic antiviral agent (eg, acyclovir 400 mg
bid) for at least 6-12 months.
In the majority of developed countries, the main indications for keratoplasty are noninfectious
disorders such as keratoconus and bullous keratopathy.
The diagnosis of Epstein-Barr (EB) virus infection, If in a case of “atypical herpes keratitis” is made on
the basis of;
1- No antibodies against ‘’herpes simplex virus (HSV), varicella zoster virus (VZV), and cytomegaly
virus (CMV)’’
2- Only EBV specific antibodies are found
3- Positive PCR of EBV DNA in the disease area.
Epistein Barr virus antibodies are found in 90-95% of all adult. (AAO)
The earliest manifestation of Vitamin A deficiency is night blindness, or nyctalopia.
Xerophthalmia, caused by vitamin A deficiency, is still the leading cause of childhood blindness. (WHO)
WHO recommends treating onchocerciasis with ivermectin at least once yearly for about 10 to 15
years.
Keratoconus is more frequent in India (2300 per 100,000 in Central India)
Injection of anterior chamber air or gas may accelerate recovery in Acute hydrops (Keratoconus)
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Best corrected visual acuity (BCVA) improves and Uncorrected Visual Acuity (UCVA) may reduce after
Phototherapeutic keratectomy (PTK), because of the induced refractive error.
PMD > men and women are affected equally, Presenting age is 20-40.
- Crescentic (Arcuate) 1–2 mm band of inferior corneal thinning extending from 4 to 8 o’clock
- Protrusion of the cornea occurs above the Crescentic (Arcuate) band of thinning.
Regarding trials comparing treatment of moderate to high myopia with phackic intraocular implants
(IOLs) versus excimer laser:
- Contrast sensitivity was better with phakic IOL compared with excimer.
- The PIOL group was less likely to lose one or more lines of BSCVA than the LASER group after 12 Ms.
- On the whole, there is no significant difference in efficacy and complications between the two kinds of
surgeries.
Granular corneal dystrophy is caused by a mutation in the Transforming Growth Factor Beta (TGF-β)
gene, located on chromosome 5q31.
superficial variant of granular corneal dystrophy → mutation of the BIGH3 gene.
This mutation causes a severe form of superficial granular corneal dystrophy by producing abnormal
keratoepithelin between the epithelium and the Bowman layer and thus clinical similarities to ReisBücklers corneal dystrophy. JAMA NETWORK
As regard Cystinosis, Topical cysteamine → may be beneficial to reverse corneal crystal forma on.
Oral cysteamine → may be beneficial for re nopathy and op c neuropathy.
In PKP → Con nuous (Running) suture pa erns are not recommended for pediatric cases.
- The single interrupted suture technique is the ideal technique in pediatric cases, because it provides
for better wound apposition and a more stable wound as sutures began to loosen over time.
5 year survival rate for penetrating keratoplasty for keratoconus is approximately 90%. And less than
50% when the indication is corneal edema due to endothelial failure from intraocular surgery, and even
lower when the eye is aphakic.
Overall risk of failure for re-grafts is about 50% at five years.
10% to 16% of all corneal transplants around the world are regrafts.
90% of corneal grafts are clear greater than 1 year postoperatively.
65% of corneal grafts performed for HSV Keratitis remains clear greater than 1 year postoperatively.
Overall the incidence of an endothelial graft rejection episode is 20% to 25% post-PK according to most
studies. (Khodadoust line) (Eyedocs)
Abdullah Fouad Notes
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DMEK has a better post-operative visual acuity and quicker healing than DSAEK.
In DSAEK detachment, management → Fill air (rebubbling the gra ).
Glaucoma following penetrating keratoplasty (PKP) is one of the most common causes for irreversible
visual loss and the second leading cause for graft failure after rejection.
DSAEK → Hyperopic shift of 1-1.5D.
Siderosis → Heterochromia, ERG: Attenuation of B-wave over time.
The incidence of sympathetic ophthalmitis → 0.2–0.5% after injury and 0.01% following intraocular
surgery.
In Chemical injury → topical anesthesia should be instilled prior to irrigation.
Regarding RA → Immune complex deposition occur in synovial membrane.
Vitamin C deficiency is common, even in industrialized countries.
Vitamin C allows hydroxylation and crosslinking of pro-collagen catalyzed by lysyl hydroxylase. Lack of
vitamin C decreases transcription of pro-collagen. Additionally, a lack of scorbic acid leads to epigenetic
DNA hypermethylation and inhibits the transcription of various types of collagen found in skin, blood
vessels, and tissue.
Ocular GVHD is associated with meibomian gland obstruction, anterior and posterior blepharitis. There
is often associated scaring of the lacrimal gland leading to decreased tear production. Conjunctival
hyperemia with pseudomembrane and membrane formation can also be seeing. A history of allogenic
bone marrow transplant is present in all patients with this disease. (EyeWiki)
Areas where trachoma is endemic, active (inflammatory) trachoma is common among preschool-aged
children, with prevalence rates which can be as high as 60–90%.
Serotypes of C trachomatis:
o Trachoma: serotypes A–C
o Adult and neonatal inclusion conjunctivitis: serotypes D–K
o Lymphogranuloma venereum: serotypes L1, L2, and L3
Immunological tests are more sensitive than Giemsa stain for diagnosis of chlamydial conjunctivitis.
Intra-nuclear inclusions → HSV (Lipschutz bodies or Cowdry type A inclusions) (Papanicolaou stain - This
shows intranuclear eosinophilic inclusion bodies)
Intra-cytoplasmic inclusions → Chlamydia (Giemsa staining).
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Giemsa staining in HSV → shows multinucleated giant cells.
General improvement of personal and community hygiene are almost universally associated with a
reduction in the prevalence and eventually disappearance of trachoma including; improved water
supplies, household sanitation particularly safe disposal of human feces. The flies that transmit
trachoma preferentially lay their eggs on human feces lying exposed on the soil.
Pneumonia has been reported in 10-20% of infants with chlamydial conjunctivitis (Common).
Recent studies showed that significantly fewer chlamydial infections occurred with povidone-iodine
than with silver nitrate or erythromycin. (EyeWiki)
Ocular prophylaxis is not effective in preventing chlamydial conjunctivitis. More effective means of
preventing ophthalmia neonatorum include screening all pregnant women for gonorrhea and chlamydia
infection, and treatment and follow-up of those found to be infected. (NCBI)
Giant papillary conjunctivitis → the risk is increased by the build-up of proteinaceous deposits and
cellular debris on the contact lens surface.
Ocular cicatricial pemphigoid (OCP) is one of the subsets of mucous membrane pemphigoid (MMP) →
linear deposition of immunoglobulin and complement at the BM zone of mucosal surfaces.
Ligneous conjunctivitis → this is a rare idiopathic chronic conjunctivitis of children (especially girls),
characterized by recurrent pseudomembranes or membranes of the ‘wood- like’ tarsal conjunctiva and
often of other mucous membranes (e.g. oropharynx, trachea, etc.). Histologically, these comprise fibrin,
albumin, IgG, and T and B cells. Treat with topical ciclosporin.
Parinaud's oculoglandular syndrome is the combination of granulomatous, follicular conjunctivitis in
one eye and swollen ipsilateral pre-auricular lymph nodes. Most cases are caused by cat-scratch
disease, although it is an unusual feature of this condition. Occasionally, it may be caused by other
infections, including Rickettsiae, Treponema pallidum, and mycobacterial species. (Eye doc)
Nephrotoxicity and ototoxicity are the most frequently reported adverse or toxic reactions associated
with systemic aminoglycoside therapy.
Regarding the steroid for corneal ulcer trial (SCUT):
- Severe corneal ulcers are more likely to benefit from steroids.
- No overall difference in 3-month BSCVA
- Steroid is not a major concern for the risk of perforation or the need for a therapeutic PKP.
- Steroid not associated with an increase in IOP. In fact, more patients had elevated IOP in the placebo
arm than in the corticosteroid arm
Fluoroquinolones and quinolones → Interfere with bacterial DNA replication, transcription and repair
through inhibition of bacterial DNA gyrase (topoisomerase II and IV).
G- activity is correlated with inhibition of II.
G+ activity is correlated with inhibition of IV.
Chocolate agar is used for growing fastidious respiratory bacteria, such as Haemophilus influenzae and
Neisseria meningitidis.
Early weaning can also increase the risk of vitamin A deficiency.
Megalocornea “anterior megalophthalmos” (> 12.5mm) → XLR (rarely can be AD,AR).
Regarding eye trauma → The existence of hyphema or iris prolapse was associated with lower rates of
endophthalmitis .
Abdullah Fouad Notes
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Relapsing polychondritis is inflammation of cartilage most commonly Auricular chondritis and nasal
chondritis. Cardiovascular lesions include aortic insufficiency and vasculitis , Skin lesions due to
cutaneous vasculitis. Ocular condition occurs in 50% of patients including conjunctivits, scleritis,uveitis
and retinal vasculitis.
The cyst of Acanthamoeba has a double wall, that is, endocyst and exocyst.
There are many connective tissue disorders that are associated with scleral disease. Rheumatoid
arthritis is the most common.
Scleritis may occur in isolation (43%) or in association with several types of systemic disorders (57%).
Chronic ocular inflammation is the most common cause of band keratopathy, particularly juvenile
idiopathic arthritis (JIA).
The causes of band keratopathy include:
 age
 ocular disease
o chronic uveitis (especially JIA)
o phthisis bulbi
o silicone oil
o alkali injury
 systemic causes
o hypercalcaemia
o hyperphosphataemia
o hyperuricaemia
 hereditary
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From MCQ Books
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Medias
* Fungi → Sabouraud dextrose agar without cycloheximide
Abdullah Fouad Notes
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* Atypical mycobacteria → blood agar
*Haemophilus → Chocolate agar (requires hemin and NAD)
*Moraxella → blood agar in 5% to 10% carbon dioxide at 37°C (Loeffler serum medium is used to
speciate Moraxella based on growth characteristics)
* Anaerobic bacteria > Thioglycolate broth
Enlarged Corneal nerves may be found in Refsum disease, ichthyosis, congenital glaucoma, and MEN
type IIb and other diseases.
Demodex folliculorumis a normal commensal in adults living in meibomian glands.
Physostigmine is effective as a respiratory poison against the lice of Phthirus pubis but has many ocular
side effects, limiting its effectiveness.
Dacryocystitis → Streptococci and staphylococci in adults (Haemophilus in children).
Canaliculitis → Actinomyces Israeli and Nocardia.
Racial or Ocular melanosis → in pigmented individuals, BL, no malignant poten al.
Conjunctival nevus → enlarge during adolescence or with pregnancy.
Thinning that is more apparent than real is a characteristic of furrow degeneration.
Terrien marginal degeneration → Epithelium remains intact, Against-the-rule astigmatism.
PMD > irregular, against-the-rule astigmatism.
Pterygium causes flattening (not steepening) in the involved meridian.
Keratoglobus is typically associated with Ehlers-Danlos syndrome.
Mooren ulcer type 1 (Milder)
Old adult
Unilateral
mild pain
More responsive to therapy such as topical steroids
Mooren ulcer type 2 (More sever)
Younger black males
Bilateral
Rapidly progressive
Respond poorly to therapy, and systemic
immunosuppressives are often necessary
Corneal perforation is rare
plasma cells are found in the adjacent conjunctiva
Mooren ulcer in old age
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The intial therapy of Corneal Hydrops in Keratoconus >> hypertonic saline drops, Cycloplegic and topical
steroids. May resolve enough that no surgery is needed.
Abdullah Fouad Notes
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Congenital hereditary endothelial dystrophy
AR Type
AD type
Non-progressive
Slowly progressive
Nystagmus
No Nystagmus
Corneal edema at birth
Corneal decompensation at 1-2 year old
No known associated ocular or systemic diseases
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Fuch's endothelial dystrophy is the most common posterior corneal dystrophy (and the most overall).
Hassall-Henle bodies → histologically similar to gu ata except they occur in the periphery.
The most common stromal dystrophy is lattice, followed by granular.
Macular corneal dystrophy, the least common of the classic stromal dystrophies.
Map-dot-fingerprint (Cogan) dystrophy is the most common anterior dystrophy. The most common
dystrophy associated with RCE is Cogan's (map-dot-fingerprint) dystrophy.
Of the 3 common stromal dystrophies (lattice, macular and granular), lattice is the most commonly
linked to RCE. (In Kanski RCE is very rare in Lattice AND Very common in Macular)
Reis–Buckler dystrophy, Avellino (granular 2 + lattice) dystrophy → chromosome 5q31.
Bacteria that are able to penetrate a healthy corneal epithelium;
eria Family → Neisseria, Diphtheria, Listeria and H. Influenza
Iris atrophy is more common with HZV than HSV.
(in HZV: Sectorial atrophy, in HSV: moth-eaten peri-pupillary defects)
HSV →Dentric ulcer with characteristic terminal buds (its bed Stained well with Fl. And The virus-laden
cells at the margin of the ulcer stain with rose Bengal)
HZV → Dentric epithelial lesions (not ulcer) that are smaller and finer than herpes simplex dendrites,
and have tapered ends without terminal bulbs (Stained better with rose Bengal than with Fl.).
(The active viral replication causes sloughing of epithelial cells in HSV, whereas in HZV, the epithelial
cells are swollen and heaped up ‫)ﯾﺘﻜﻮم‬.
Hyperventilation, rather than hypoventilation, can be used during cases under general anesthesia to
decrease vitreous pressure.
Peters anomaly is the most frequent indication for penetrating keratoplasty in children.
In Radial Keratotomy →the dioptric effect per incision decrease significantly a er 8 of incisions.
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Arcuate incisions (for Astigmatism TTT) greater than 90 degrees are relatively contraindicated.
Corneal deposits:
 Ferry's line: edge of filtering bleb
 Stockers line: in front of pterygium
 Hudson-Stahli's line: junction of upper 2/3rds and lower 1/3rd of otherwise normal cornea
 Fleischer's ring: base of keratoconus cone in Epithelium
 Kayser-Fleischer ring: copper deposition in Descemet's membrane in Wilson's disease
Thygeson's Keratitis → associated with HLA-DR3 (Thygeson → Three)
Topical treatment of Acanthamoeba including: (+ Oral ketoconazole)
Chlorhexidine
Neomycin
Miconazole
Propamidine (Diamidines)
Polyhexamethylene biguanide
Hypersensitivity marginal Keratitis → Staphylococcus aureus
Type 1 Vogt’s limbal girdle (band keratopathy) contains sub-epithelial deposits of calcium, which stain
black with Von Kossa, or red with Alizurin.
Arcus Senilis → in the se ng of high grade carotid stenosis, the ipsilateral eye is protected from lipid
deposition. (if unilateral > contralateral carotid insufficiency)
Urrets-Zavalia syndrome → persistent post-operative mydriasis after PK. The cause is uncertain, but it
is thought to be due to peri operative iris ischaemia.
Pharyngo-conjunctival fever (adenovirus type 3, 4, 7) generally spares the cornea, although
subepithelial infiltrates or superficial punctate keratitis may rarely be seen.
Epidemic keratoconjunctivitis (adenovirus type 8, 19, 37) has a greater likelihood of corneal
involvement.
Infectious crystalline keratopathy on a PK graft (The image below) → a white branching lesion on the
corneal graft. Caused by an indolent infection by Streptococcus viridans. It is predisposed in patients
taking long-term steroid drops.
Micropapillae are more common in contact lens wearers than the classic giant papillae
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Giant papillae are over 1 mm in diameter.
Acute haemorrhagic conjunctivitis is classically caused by picorna viruses including the Coxsackie group
A24 and enterovirus E70. Adenoviral conjunctivitis can also cause haemorrhage.
Keratoconus is the most common indication for DALK in adults.
Fuch's dystrophy is the most common indication for DSEK in the UK.
Conjunctival papillae are a normal appearance in young people especially at superior edge of superior
tarsus
Potent steroids may precipitate melting in eyes with rosacea keratitis.
Topical Metronidazol has a grade A evidence rating for the treatment of mild to moderate
inflammatory Rosacea, with a small study showing an improvement in Rosacea-related eye healthcare.
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8) Ocular Inflammation and Uveitis
From Exams
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Lyme disease → Borrelia burgdorferi & False +ve result for syphilis (Both are spirochetes)
Neurological involvement is common in Lyme disease (40%)
Cat-Scratch disease → Bartonella henselae
Juvenile xanthogranuloma (JXG) → (Early Childhood –Multiple skin lesions -Heterchromia- HyphemaTouton Giant Cell) **
Syphilis may cause Ischemic Optic Neuropathy
Sub-tenon injection of Corticosteroid is contraindicated in Toxoplasmosis associated with active
Chorioretinitis **
Immune recovery Uveitis → induced by cidofovir in patients taking HAART therapy
Reactive arthritis (ReA, also known as Reiter syndrome) is characterized by a triad of non-specific
urethritis, conjunctivitis and arthritis. (75% HLA-B27) (20% AU)
The likelihood to develop reactive arthritis:
- Following a sexually-acquired infection → Men > females 9 times
- Following a gastrointestinal infection → Men = Females.
In humans the MHC (HLA) is found on chromosome 6.
HLA haplotypes that most commonly associated with acute anterior uveitis → HLA-B27
The MOST common cause of uveitis in patients under 20 years of age → Idiopathic anterior uvei s.
The most likely to be normal in Ankylosing spondylitis → Thoracic spine.
Psoriasis → AAU 7% - HLA-B27
Risk factors for Chronic Non-Granulomatous Anterior Uveitis in JIA:
1- ANA +ve
2- Oligoarticular
3- Female
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4- RF –ve
5- Age below 4 years
The incidence of blindness in medically managed JIA is 6-25%.
The first choice immunomodulatory agent to reduce the effective dose of systemic corticosteroids in
juvenile rheumatoid arthritis (JRA) → Methotrexate
Antimetabolites such as methotrexate and azathioprine → interfering with DNA synthesis.
Adalimumab, certolizumab pegol, etanercept, golimumab and infliximab → their primary target is TNFalpha & Optic Neuritis is an adverse effect.
Approximately 50-75% of patients with spondylitis in association with inflammatory bowel disease
have HLAB27. In contrast, patients who develop sclerouveitis in the presence of inflammatory bowel
disease tend to be HLA B27 negative, and these patients do not develop sacroiliitis.
In patients with pars planitis, the following findings increase the risk of developing MS:
- Periphlebitis.
- Vascular sheathing.
Pars planitis = (Idiopathic IU + ‘Snowbank’ or 'snowball') → ‘Snowbank’ or 'snowball' forma on is
essential to the diagnosis of PP.
In PP → CMO occurs in up to half of patients and is the major cause of impaired visual acuity.
In the prodromal stage of VKH → symptoms will resemble a viral flu like illness.
IOP elevation may occur in more than 50% of patients with intravitreal steroid injections,
- Up to 25% may require topical medications to control IOP.
- 1-2% may need filtering surgery.
Difluprednate, a fluorinated corticosteroid is highly potent and has the same efficacy as the every 2 hour
dosage of prednisolone when give just 4 times daily.
Periocular corticosteroid injection have potential raise IOP precipitously or for long time. The peak
action of Periocular steroid is about 4 weeks with maximum duration of action of around 3 months.
Class II HLA → (DP, DM, DO, DQ, and DR) e.g. VKH “DR1,DR2”
A tuberculin skin test (e.g. standard Mantoux “purified protein derivative test”) is negative in most
sarcoid patients; a strongly positive reaction to one tuberculin unit makes a diagnosis of sarcoidosis
highly unlikely.
Sarcoidosis → All age groups, non-caseating granulomas (aggregates of epithelioid histiocytes),
Schaumann bodies and asteroid bodies.
In Behcet → Major morbidity and mortality are the result of vascular occlusions (e.g. hepatic portal vein,
cerebral venous sinus) and CNS involvement. (Vascular > Neurological)
- The disease and visual prognosis are more severe in males.
- About 25% of BD patients → severe vision loss (a visual acuity less than 20/200).
The most Common Uveitis → Anterior (1/3). Most of these cases are considered idiopathic. However
HLA B27 positivity is associated with the disease in up to 1/3 of patients
Periocular steroid injection (sub-tenon) should not be used in cases of infectious uveitis such as
toxoplasmosis.
In congenital Toxoplasmosis → Retinochoroiditis may occur in over 75%, leaving scars that are
commonly a later incidental finding.
Abdullah Fouad Notes
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Toxoplasma Ig (G → Gadeem “old,chronic”, M → Modern “Recent,acute”)
- IgG → indicate exposure to the organism at some point in the past (appears after 1-3 weeks after
infection, remain detectable for life at variable levels, and cross the placenta.), but its +ve at least in 1/3
of individuals in most communities. (may be –ve in immunocompromised)
-IgM → means that infec on has been acquired only within the last year, persistence or reactivation
following earlier infection. Therefore it helps to distinguish between acute (newly acquired) and chronic
infection. And do not cross the placenta.
- Measurement of IgA antibody titers may also be useful in a diagnosis of congenital toxoplasmosis in a
fetus or newborn; during this period, IgM production is often weak and the presence of IgG antibodies
may indicate passive transfer of maternal antibodies in utero. IgA antibodies, however, usually
disappear by 7 months.
Rate of fetal Toxoplasma transmission (in contrast to severity)
- less than 6% in the 1st trimester.
- 60- 81% in the 3rd trimester.
In most societies, prenatal fetal testing of toxoplasmosis incurs more fetal losses than cases of the
disease prevented.
Visceral toxocaraiasis (VLM) and ocular toxocariasis for unknown reasons seldom occur in the same
patient.
In auto-immune retinopathy (AIR) → (ERG) changes are seen earlier than (OCT) changes.
Viral Posterior Uveitis
CMV
Immunity
- Compromised
Laterality
- UL, then BL
Causative agent - CMV
Retinitis
Vitritis
Treatment
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PORN
- Compromised
- UL or BL
- HZV mainly
- And HSV
- Peripheral
- Starts in Macula
- Patchy
- Multifocal
- Mild
- Minimal
- HAART
- HAART
- Oral Valgancyclovir - Intravitreal and IV
- IV Gancyclovir,
Ganciclovir,
Foscarnet, Cidfovir
Foscarnet
- Ganciclovir SR
intravitreal implant
- +/- Steroid for IRU
ARN
- Healthy, but may also in compromised
- Usually UL
- HSV (Young)
- HZV (Old)
- Peripheral - Multifocal
- Posterior pole is usually spared
- Prominent
- IV Acyclovir, then oral
- Oral Valaciclovir or Famciclovir
- Intravitreal Ganciclovir or Foscarnet
- +/- Steroid “after 24hs of antiviral”
PORN is often resistant to systemic treatment with intravenous acyclovir alone.
CMV is the most common congenital infection of humans
CMV Retinitis → Risk factors: HIV, CD4 count below 50, or severe systemic immunosuppression. (oral
valganciclovir)
Intranuclear “owl’s eye” inclusions and intracytoplasmic inclusions are present in CMV infected cells
(H&E stain).
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Cidofovir is a strong antiviral agent which is effective against HS, HZ, CMV, EBV, and HIV. It prevents
DNA synthesis by viral DNA polymerase. In 37% of patients treated anterior uveitis happens.
Foscarnet is an antiviral medication used to treat (CMV) retinitis in people with AIDS. Associated with
Renal toxicity.
Gancyclovir → marrow depression
Foscarnet:
* Nephotoxicity
* Convulsions
* Electrolyte dist.
Cidofovir:
* Nephrotoxicity
* Neutropenia
* Uveitis with hypopyon
The most common manifestations of congenital (CMV) infection are hearing loss and developmental
delay.
CMV infection in healthy adults is usually asymptomatic or causes a mild mononucleosis-like syndrome
“flue-like”: Fatigue, Low-grade fever (can last days or weeks), Chills and/or sweats, Muscle aches,
Decreased appetite, enlarged lymph nodes, Sore throat, Headache.
Immune recovery uveitis (IRU) “Immune reconstitution inflammatory syndrome “→ due to a
rejuvenated immune response against residual viral antigen following immune reconstitution with
HAART. In addition to improved immunity itself, risk factors include:
1) a low CD4 T count at the time of initiation of HAART
2) involvement of a larger proportion of retina.
3) Treatment with cidofovir
The most common infectious organisms causing AIDS related diarrhea include → cytomegalovirus
(CMV); the parasites Cryptosporidium, microsporidia and Giardia lamblia; and the bacterium
Mycobacterium avium-intracellulare (MAC).
Regarding Cystocercosis → Inflammation develops in response to antigens released by dead organisms.
Ivermectin → kills microfilariae of Onchocerciasis (but not adult worms) and is given at least annually
for many years.
Regarding Onchocerciasis “African River Blindness” → Degenerating microfilariae (Not live microfilaria)
excite an intense inflammatory reaction accounting for most of the clinical manifestations of the
disease.
Humans are the only host for Onchocerca volvulus. The vector is the Simulium blackfly, which breeds in
fast-flowing water.
Abdullah Fouad Notes
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To confess that the patient has antibodies to HIV → ELISA must be repeated twice and confirmed by
Western blot analysis or immunofluorescence assay
Caesarian section decreases maternal-to-fetal transmission of HIV.
Women who take HIV medicines during pregnancy and childbirth and whose babies receive HIV
medicines for 4 to 6 weeks after birth have a low risk of transmitting HIV.
Ebola disease: (in Africa, primarily Liberia, Sierra Leone, and Guinea)
- Caused by infection with a virus of the family Filoviridae, genus Ebolavirus.
- Anterior or Panuveitis > Sudden loss of vision
- AC tap → Live virus
POHS:
- Classic triad: (i) multiple white atrophic chorioretinal ‘histo’ spots about 200 µm in diameter (ii)
peripapillary atrophy (iii) vitritis is absent. Linear midperipheral scars also occur (5%).
- Usually asymptomatic unless macular CNV.
- Associated with HLA-B7 and DRw2.
- Skin antigen testing was of limited utility and may have worsened POHS in some cases.
- Without treatment 60% of eyes with CNV have a final visual acuity of less than 6/60 (Intravitreal
“VEGF”)
Pneumocystis jiroveci pneumonia (PJP), formerly known as Pneumocystis carinii pneumonia (PCP):
- The most common opportunistic infection in persons with HIV infection.
- Trimethoprim-sulfamethoxazole (TMP-SMX) (Cotrimoxazol) has been shown to be as effective as
intravenous pentamidine. (does not respond to antifungal treatment).
- Choroiditis without vitritis.
Abdullah Fouad Notes
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Cotton-ball colonies “Fluffy white puffball” → Endogenous fungal endophthalmi s.
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Posterior uveitis is the most common presentation of intraocular TB (involvement of retina alone is
uncommon).
TB drug therapy can cause ocular and systemic side effects: (particularly hepatic and neurologic ones)
- Isoniazide and Ethambutol → Optic neuritis in few treated patients.
- Rifampicin → blepharo-conjunctivitis and pink tinged tears.
Drug resistant tuberculosis (DR-TB) is strongly associated with HIV infection.
The tuberculin skin test; PPD (Protein purified derivative) or Mantoux tuberculin skin test (TST) →
measures Cell mediated delayed hypersensitivity (type 4) to tuberculoprotein.
Ocular tuberculosis is usually not associated with clinical evidence of pulmonary tuberculosis, as up to
60% of extrapulmonary tuberculosis patients may not have pulmonary disease.
IV penicillin G is the recommended treatment for all forms of neurosyphilis and for syphilitic eye disease.
All forms of natural and semisynthetic penicillins can cause allergy, but it is more commonly seen after
parenteral than oral administration. Penicillin G is the most common drug implicated in drug allergy.
Incidence is probably highest with procaine penicillin, as procaine itself is allergenic.
In neurosyphilis, the serum VDRL test may be negative and cerebrospinal fluid VDRL may be positive.
Eye complications are more commonly found in patients with long standing multibacillary
“Lepromatous” than paucibacillary “Tuberculoid” leprosy.
Nearly 25% of patients worldwide with chronic ocular Behcet disease have visual acuity less than
20/200. AAO UVEITIS
Uncommon complications of Toxoplasma retinitis → Periarteritis resulting in BRAO. Kanski
Acute zonal occult outer retinopathy (AZOOR) is more common in females (in one or both eyes of young
or middle-aged).
Leukotriene B4 (LTB4) is a leukotriene involved in inflammation. It is produced from leukocytes in
response to inflammatory mediators and is able to induce the adhesion and activation of leukocytes on
the endothelium, allowing them to bind to and cross it into the tissue.[1] In neutrophils, it is also a
potent chemo-attractant, and is able to induce the formation of reactive oxygen species and the
release of lysosomal enzymes by these cells.[1] It is synthesized by leukotriene-A4 hydrolase from
leukotriene A4.
Cysticercosis is an infection caused by the eggs of Taenia solium, or pork tapeworm. It is the most
common parasitic infestation affecting the central nervous system (CNS), and approximately 90% of
patients with cysticercosis have CNS involvement. When the CNS is involved in cysticercosis, it is called
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Abdullah Fouad Notes
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neurocysticercosis (NCC).
CT: multiple calcified lesions.
CT: numerous lesions.
MRI: multiple, large, cysticercus
cysts in the basal cisternal spaces.
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9) Glaucoma
From Exams
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Regarding Hyphema >> the risk of Rebeelding is greatest 3-5 days after the injury
POAG >> the prevalence is 3-8 times higher at 80 than at 40 years old
FH risk factor of POAG > risk to siblings “brother or sister” is 4 times and to offspring 2 times the normal
population risk.
In POAG > the vertical cup-to-disc diameter ratio increases faster than the horizontal one, leading to an
increase of the quotient of horizontal-to-vertical cup to-disc ratios to values lower than 1.0.
NTG >> more common in women
The rise of IOP takes 2-4 weeks or up to 6 weeks after starting ocular steroid.
Systemic hypertension is associated with a lower risk of the presence of glaucoma because higher
blood pressure is associated with improved optic nerve perfusion, So over treatment of systemic
hypertension may be a factor to glaucoma progression in some cases and should be avoided.
Ocular perfusion pressure is the difference between the arterial BP and the intraocular pressure (IOP),
and has been shown in population studies to be linked to increased risk for the development and
progression of glaucoma.
Disc hemorrhage most commonly inferotemporally , their presence is a risk factor for the development
and progression of glaucoma.
Increase in corneal thickness of 10 microns results in increase IOP by 1mm Hg.
Abdullah Fouad Notes
Page 58
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Depressed sensitivity in the superior nasal quadrant is the most prominent in the typical arcuate
scotoma found in patient with OAG
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Paracentral, small, relatively steep depressions constitute approximately 70% of all early glaucomatous
field defects.
The earliest defect due to glaucoma is generalized decreased sensitivity.
Typically the central island of vision and the infertemporal visual field are retained until late in the
course of glaucomatous optic nerve damage.
As nasal fibers and the maculopapular bundle are typically spared until late in the disease process, it is
common to have a central or temporal island of vision remaining in eyes with advanced glaucoma.
Mitomycin C inhibits Fibroblast proliferation.
Kinetic perimetry is more efficient at holding the tested person's attention than static.
Manual perimetry is more efficient at holding the tested person's attention than Automated.
Adverse effects of PG analogues include conjunctival hyperemia (as a result of vasodilation ) more with
bimatoprost and travoprost.
Thymoxamine is alpha receptor blockers that revese pharmachological dilation.
The basic protocol for Argon laser trabeculoplasty:
1- Spot size of 50 micron.
2- Power of 300-1000mwatt.
3- Spot duration of 0.1 second.
4- Laser spot placed at the junction of the anterior non-pigmented and the posterior pigmented edge
of trabecular meshwork.
A cyclodialysis cleft is a separation of the ciliary body from the scleral spur, creating a direct connection
between the anterior chamber and the suprachoroidal space. Many will spontaneously close, but those
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Abdullah Fouad Notes
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that do not can cause chronic hypotony, resulting in hypotony maculopathy, optic disc edema, and
decreased visual acuity. Treatment begins with conservative medical therapy, but when this fails, a wide
range of laser and surgical procedures have been reported to be effective. (eyewiki)
ARG → Tear between longitudinal and circular muscles of CB
Gonioscopy of ARG → Initially irregular widening of the ciliary body face.
Look at (P.314 Kanaski) for normal Gonioscopy, then look for these photos of ARG
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Glaucoma Drainage Device common long adverse effect:
- Encapsulated bleb (n = 27, 24.5%)
- Corneal decompensation (n = 20, 19%) (came in 2019, 2016 exams)
- Retinal detachment (n = 7, 6.4%)
- GDD or tube dislocation (n = 7, 6.4%)
- Tube erosion (n = 7, 6.4%)
- Tube-endothelial touch or blockage (n = 5, 4.5%)
- Chronic hypotony (n = 5, 4.5%)
- Phthisis bulbi (n = 4, 3.6%).
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6327392/
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Beta blockers are competitive antagonist of the β-adrenergic receptors. They inhibit the activation of
these receptors in the ciliary processes by blocking the binding of endogenous adrenergic
neurotransmitters.
A cyclodialysis is a separation of the ciliary body from the scleral spur
The Majority of Resistance of TM at the juxtacanaclicular (Cribriform) CT meshwork.
End stage Glaucoma → the last VF to be affected beside Central island is Inferotemporal
PG > Risk of reactivation of Herpetic Keratitis.
In Cyclodiode ablation → the power is adjusted over sequen al shots un l a ‘popping’ sound is heard
and then reduced to just below that level.
Intracameral Carbachol induces more prolonged ocular hypotensive effect than Acetyl Choline.
A large number of surgical complications were observed in the TVT(tube versus trabeculectomy) Study,
but most were transient and self-limited. The incidence of early postoperative complications was higher
following trabeculectomy with MMC than tube shunt surgery. The rates of late postoperative
complications, reapportion for complications, and cataract extraction were similar with both surgical
Abdullah Fouad Notes
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procedures after 5 years of follow-up.
https://www.sciencedirect.com/science/article/pii/S0002939411008452
Retinal ganglion cell death in glaucoma occurs predominantly through apoptosis (programmed cell
death) rather than necrosis.
The screening for glaucoma based solely on IOP greater than 21 mmHg misses up to half of people with
glaucoma and optic nerve damage in screened population.
Alcohol consumption decrease IOP.
Early Manifest Glaucoma Trial (EMGT) (6 years):
- In the treated group, IOP was lowered by an average of 25 %.
- Progression risk was halved by treatment.
- Risk of progression decreased 10% with each 1mmHg IOP reduction from baseline to the first F/U visit.
- Only exfoliation glaucoma > IOP increased by almost 1 mm Hg annually without treatment.
NTG > abnormal ocular blood flow.
Only Sporadic Aniridia (WARG) is associated Wilms Tumor. In contrast, Familial Aniridia is not
associated with Wilms.
AD Anidridia > 2/3 of cases and has no systemic associations (PAX6 mutation)
AR Aniridia (Gillespie syndrome) > 1% of cases. Not caused by PAX6. Cerebellar ataxia and mental
handicap are features.
Aniridia > Glaucoma (75%), usually ACG.
Lens extraction does not alleviate the pseudoexfoliation syndrome.
Pseudoexfoliation often presents unilaterally and the uninvolved eye manifests signs of the disease at a
later time. Mutations in single gene LOXL1 seem to be present nearly in all cases. The risk of progression
to glaucoma also varies widely and can be as high as 40% of patients over 10 year period.
NVs of the angle without associated fibrous membrane or PAS > Fuchs heterochromic iridocyclitis.
ERG in Ischemic CRVO > decrease in b/a wave amplitude ratio (sometimes been used to assess
neovascular risk)
Glaucoma in association with retinoblastoma most commonly is caused by iris NVs followed by
displacement of the iris-lens diaphragm.
PSS > In the past, iris atrophy or heterochromia has been noted in some cases, but it is not currently
considered a characteristic finding for PSS.
The lack of KPs helps distinguish phacolytic from phacoantigenic “phacoanaphylactic” glaucoma.
A newborn's cornea is typically 9.5-10.5 mm in diameter and increases to 10.0-11.5 mm by age 1. Any
diameter above 12.5 mm suggests an abnormality, especially if there is asymmetry between the two
eyes.
Hyphema Grades:
Grade I → <1/3 AC
Grade II → 1/3 - 1/2 AC
Grade III → >1/2 AC
Grade IV → Total AC
Hyphema and high IOP: (AAO)
- Grade I to II hyphemas → 13.5% has an IOP increase
Abdullah Fouad Notes
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- Grade III hyphemas → 27% has an IOP increase.
- Grade IV hyphemas → 52% experiencing an IOP increase.
- Cases with rebleeding → had a more than 50% chance of having elevated IOP.
Marijuana → lowers IOP for 3 hours after it is taken. It shows drug tolerance and tachyphylaxis.
EyeWiki
Study suggests that peripapillary angio flow density measurements (using OCTA) can identify decreased
peripapillary perfusion early in the glaucomatous RNFL thinning process, prior to the development of
clinically significant RNFL damage and visual field deterioration.
Regarding express shunt:
- Direct shunt from anterior chamber to subconjunctival space.
- It is inserted under scleral flap to control hypotony.
- 3 mm long with an external diameter of approximately 400 microns. It is a non-valved, MRI
compatible, stainless steel device with a 50 micron lumen.
- It has an external disc at one end and a spur-like extension on the other to prevent extrusion.
- have a lower rate of hypotony than trabeculectomy.
Abdullah Fouad Notes
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About one half of PACG are Asian, It increases after 40 years and rare in person younger than 40 years.
PACG occurs commonly in patients with hypermetropia. It is 2-4 times more commonly in women than
men.
Up to 50% of eyes develop NVG following ischemic CRVO . AAO
Among eyes with severely ischemic CRVO, the incidence of anterior segment neovascularization, iris and
angle, is high (up to 60%). "The Central Vein Occlusion Study"
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From MCQ books
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Topical or systemic corticosteroids are not a risk factor for POAG; rather, it is a risk factor for steroid
induced Secondary OAG.
Applanation tonometry→ Use of excessive fluorescein causes thick mires and an overestimation of IOP.
Applanation tonometry→ if corneal as gma sm is greater than 3D, IOP is underestimated for with therule astigmatism and overestimated for against-the-rule astigmatism.
A pupillary diameter of less than 3 mm can cause general depression of the Visual field.
On the Goldmann perimeter, The III4e test object will have twice the diameter (size) and four times the
area of the II4e test object.
A fixation loss occurs when the patient responds as if seeing a light when a target is displayed in his blind
spot.
Intermittently, the perimeter will pause and the motorized light will change position, but no stimulus
will be presented. If the patient presses the button, a false-positive response is recorded.
Topical timolol has been shown to decrease HDL.
Allergic Conjunctivitis with Glaucoma drugs → Bromonidine 20%, Dorzolamide 1-2%. PG analogs more
commonly produce conjunctival hyperemia than true allergic conjunctivitis.
CAI may cause idiosyncratic and transient acute myopia.
Using CAI → aqueous produc on in the eye is not significantly reduced until more than 90% of the
carbonic anhydrase activity is inhibited.
The most common cause of bleb failure → Episcleral fibrosis.
Apraclonidine side effects → dry mouth, eyelid retrac on, mydriasis, and conjunc val blanching.
Colchicine affects Collagen Cross-linking and thereby decreases sCar formation.
BAPN (β-aminoproprionitrile), an inhibitor of lysyl oxidase, blocks collagen crosslinking.
5-FU inhibits fibroblast proliferation by acting selectively on the S phase of the cell cycle.
5-FU stimulates thymidylate synthetase.
Mitomycin C is an alkylating agent that decreases DNA synthesis by causing DNA crosslinking.
Mitomycin C is a potent antifibrotic that inhibits fibroblast proliferation. It is injected Subconjunctival.
Because of its toxicity and mutagenic intracameral injection must be avoided.
Abdullah Fouad Notes
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Although Mitomycin C is more potent than 5-FU, it is associated with a lower incidence of hypotony and
corneal-surface disorders than 5- fluorouracil.
Corneal epithelial defects → more with Mitomycin C than 5-FU.
5- fluorouracil → improve intraocular pressure control in patients who have undergone previous
cataract or glaucoma filtering surgery.
In acute ACG, hydropic degeneration and impaired axoplasmic flow cause swelling and hyperemia of the
optic nerve.
Schwartz $ is the name given to OAG after rhegmatogenous RD.
A large inferior peripheral iridectomy is mandatory when silicone oil is placed into an eye. The oil is
lighter than water, and to prevent pupillary block glaucoma, an inferior peripheral iridectomy is
performed.
Unlike latanoprost and travoprost, which lower IOP by increasing uveoscleral outflow, bimatoprost
decreases IOP by increasing uveoscleral and trabecular outflow. (Bi = 2)
Unoprostone appears to lower IOP by increasing trabecular outflow alone. (uno = 1)
Latanoprost and travoprost are prodrugs that penetrate the K and become biologically active after being
hydrolyzed by corneal esterase. Neither bimatoprost nor unoprostone appears to be a prodrug.
The candy stripe is a tip-off to ghost cell glaucoma (occurs 1-3 months after VH).
Two conditions are necessary for the development of this unique form of glaucoma: vitreous
hemorrhage (red blood cells degenerate and become rigid in the vitreous) and a break in the anterior
hyaloid face (to allow the cells to enter the anterior chamber). The crenated khaki-colored ghost cells
layer out in the anterior chamber and can be distinguished from fresher red cells, creating the effect of a
candy stripe. (N.B. Candy-cane hypopyon caused by HSV “Hypopyon + hyphema”)
Haemolytic glaucoma → haemoglobin-laden macrophages block the trabecular meshwork (may occur
within days of hemorrhage)
Isolated depressed quadrantic defects in perimetry result if the patient misses the early portion of the
test in which the machine attempts to determine the threshold for each quadrant.
Abdullah Fouad Notes
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Overall, the most common cause of bleb-associated endophthalmitis is Streptococcus species, followed
by Staphylococcus species and Haemophilus influenzae (Kenneth Wright, Massachusetts).
(Kanski, The most frequent are Haemophilus influenzae, Streptococcus spp., and Staphylococcus spp.)
The microbes most commonly associated with early bleb associated endophthalmitis (BAE) (<6wks)
include Staphylococcus epidermidis. Late onset(>6wks) BAE is associated with greater percentage of
Streptoccous species 31%, and Gram-negatives .such as Haemophilus influenzae 23%, Enterococcus
7%, Pseudomonas 7%, and fewer Staphylococcus species7%-22%
https://eyewiki.aao.org/Bleb_Associated_Infections
Iridoschisis is usually most prominent in the inferior iris.
Static perimetry is the most useful for quantifying and tracking visual field changes in a patient with
established glaucoma.
Direct Gonioscopy → Koeppe, Richardson, Barkan, Wurst and Swan-Jacob.
Indirect Gonioscopy → Goldmann, Zeiss, Posner and Sussman.
Conjunctival melanosis was classically associated with topical epinephrine.
Most cases of primary congenital glaucoma are sporadic.
Haab's striae = Horizontal = Healed breaks in DM, infantile glaucoma.
Vogt's striae = Vertical = Vagina, birth trauma.
Melanomalytic glaucoma results from blockage of the trabecular meshwork by macrophages that have
engulfed material released from an intra-ocular melanoma tumor.
The most common cause of secondary glaucoma due to intra-ocular melanoma (Choroidal melanoma or
retinoblastoma) is neovascularisation.
In ciliary body melanoma, anterior displacement of the iris-lens diaphragm resulting in angle-closure is
the most common mechanism. (in Massachusetts, direct invasion of the anterior chamber angle)
Iris melanomas raise IOP by mechanical obstruction of the meshwork, either by direct angle invasion, or
by Melanomalytic.
Indirect-acting parasympathomimetic agents tend to have the most pronounced systemic and ocular
side effects (e.g. Cataract) compared to direct-acting agents.
Topiramate is associated with acute 2ry ACG. Most cases are bilateral, and can lead to blindness if not
treated or recognised. All ocular side-effects are reversible if recognised early and the drug is
discontinued. Ocular side-effects of topiramate:
 blurred vision
 myopia
 suprachoroidal effusion
 angle-closure glaucoma
 scleritis
ICE syndrome has a correlation with HSV.
Optic disc haemorrhages occur most commonly infero-temporally.
Anderson's minimal criteria for glaucomatous damage include:
 Glaucoma hemifield test outside normal limits on at least two consecutive occasions
 A cluster of three or more non-edge points, all depressed on pattern standard deviation at the level of
Abdullah Fouad Notes
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P less than 5% one of which is at P less than 1%, on two consecutive occasions
 CPSD that occurs in less than 5% of normal individuals on two consecutive occasions
Compared with plasma, aqueous is slightly hypertonic and acidic. Aqueous has a marked excess of
ascorbate (15 times greater than that of arterial plasma) and a marked deficit of protein (0.2% in
aqueous as compared to 7% in plasma).
The most common side effect of CAI is Paraesthesia.
A shallow anterior chamber with high IOP post-trabeculectomy can be the result of:
 Pupillary block: this is due to a non-patent peripheral iridotomy with iris bombe. The bleb is flat and
Siedl's is negative
 Aqueous misdirection: this is due to aqueous being directed posteriorly into the vitreous. The AC is
shallow, the IOP high, the bleb flat and Seidel negative. However, the iridotomy in this case is patent
with no iris bombe.
 Suprachoroidal hemorrhage: Chroidal detachement by fundus + U/S
A shallow anterior chamber with low IOP post-trabeculectomy can be the result of:
 Scleral flap leak: the bleb is well formed and Seidel test negative
 Conjunctival bleb leak: the bleb is flat and Seidel test positive
 Ciliary body shutdown: the bleb is flat and Seidel test is negative
Oral nicotinic acid “Niacin = Vit. B3) → Causes CME without leakage, in contrast to topical Epinephrine,
topical latanoprost and oral venlafaxine “SSNRIs Antidepressant” can all cause CME with leakage on
fluorescein angiogram.
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Abdullah Fouad Notes
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Management of an over-draining bleb > A Plamberg (compression mattress) sutures.
Glaucoma is much more common in Sturge-Weber patients with facial haemangioma involving the
upper eye lid.
Carbonic anhydrase inhibitors can exacerbate corneal oedema due to their effect on the endothelial
pump mechanism.
Alpha agonists such as brimonidine can precipitate a hypertensive crisis with TCA (e.g. amitryptyline that
used also for trigeminal neuralgia).
The IOP lowering effect of pilocarpine is additive (complimentary to) beta-blockers, but not with
prostaglandin analogues.
Pilocarpine gel at night is known to induce diffuse corneal haze.
The rate of production of aqueous humour is approximately 2 and 4 microlitres per minute.
In sub-acute (intermittent) angle closure → White eye & A acks are usually broken a er 1-2 hours by
physiological miosis (e.g. bright light or sleep).
Apraclonidine (alpha-2 agonist ) → cause a mid-miosis in normal eyes and mydriasis of Horner's
syndrome eye.
Abdullah Fouad Notes
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The distribution of IOP in the population → Gaussian with a skew to the right.
Glaucoma → linked by the presence of endothelial leukocyte adhesion molecule 1 (ELAM-1)
PSS is characterized by an open irido-corneal angle.
(This differentiates the acute spike in IOP of PSS from ACG and/or inflammatory glaucoma)
Topical CAI commonly causes a transient bitter taste.
The standard reference plane taken in Heidelberg retinal topography (HRT) of the optic disc → 50 um
below the optic nerve head boundary line at the temporal disc edge along the horizontal
midline(because it corresponds to the centre of the PMB of the RNFL).
The ICE syndrome consists of the following three overlapping disorders: (Typically Unilateral)
Progressive iris atrophy: pseudopolycoria, corectopia, iris atrophy.
Cogan-Reese syndrome: diffuse iris naevus or nodules. Iris changes also prominent in 50% of cases.
Chandler syndrome: hammered-silver corneal endothelial changes. Iris changes present in
40%.Glaucoma less prominent than the other 2 forms.
Plateau iris configuration (PIC) → Pilocarpine and peripheral iridotomy.
Plateau iris syndrome (PIS) → Peripheral iridoplasty.
IOP increases more with → dexamethasone than betamethasone than fluorometholone.
30% of Sturge-Weber syndrome patients develop glaucoma. Most (60%) occur in patients under the age
of 2 years.
In Sturge-Weber syndrome, isolated trabeculodysgenesis is important in the pathophysiology of earlyonset glaucoma <1year old. Meanwhile, raised episcleral venous pressure is important in late-onset
glaucoma >1year old.
Up to 50% of optic nerve axons can be lost before any change is detected on the Goldmann perimetry.
Koeppe Gonioscopy is considered best for evaluating a patient with ARG.
B-Blockers → Depression.
Grading of glaucomatous damage:
Mild (Grade 1)
Moderate (Grade 2)
Severe (Grade 3)
End-Stage (Grade 4)
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Fundus
Minimal cupping
Thinning of neuroretinal rim
Marked cupping
Gross disc cupping
Scotoma
Nasal step or paracentral scotoma
Arcuate scotoma
Extensive VF loss including the central 5 degrees
Small residual field
MD
< -6 dB
< -12 dB
> -12 dB
Echothiophate → is advisable to discon nue the drug several weeks before surgery:
1- Causes a disruption of the blood-aqueous barrier, which may cause increased inflammation after
intraocular surgery.
2- Indirect sympathomimetic agents also block other cholinesterases, including plasma
pseudocholinesterase, which deactivates succinylcholine. Patients may be paralyzed for extended
periods of time after anaesthesia with succinylcholine and need to be warned about this.
Brimonidine is the safest anti-Glaucoma drug in early pregnancy (However, their use in late pregnancy
and during breast-feeding should be avoided because they can cross the blood-brain barrier causing CNS
depression and apnoea in the neonate)
Sub-conjunctival injections of 5- FU in the post- trabeculectomy are administered 180 degrees from the
bleb to minimize intraocular diffusion and the risks of bleb leakage.
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Brimonidine → It is contraindicated in pa ents taking monoamine oxidase inhibitors since it can
potentiate a hypertensive crisis. Its IOP lowering effects can be blunted by tricyclic antidepressants.
That TM cells have phagocytic capacity in addition to maintaining the health and hydration of the
connective tissues.
IOP is lower when a person sleeps inclined head up, compared to lying flat.
Peak pressures are reached during sleep, in the early-morning hours.
Scleral buckles that are too tight can cause CB effusions, forward rotation of the CB and secondary ACG.
Another mechanism of angle closure following buckling surgery is NVG caused by buckle-induced ocular
ischemia.
Nanophthalmic eyes are at risk of uveal-effusion syndrome due to thick sclera impeding vortex vein
drainage. This can cause CB effusions, which can lead to a secondary ACG.
Carteolol has intrinsic sympathomimetic activity, thus inducing less bradychardia and bronchospasm
than timolol.
The widest and the easiest portion of the irido-corneal angle for distinguishing landmarks on Gonioscopy
→ The inferior angle.
Apraclonidine → tachyphylaxis. (acute, sudden decrease in response to a drug after its administration;
i.e. a rapid and short-term onset of drug tolerance)
PXF → may be associated with increased risk of cardiovascular disease.
Gonioscopy in primary congenital glaucoma → absence of the ciliary body band.
Split fixation is the presence of visual field loss that comes close to fixation.
A typical pattern of glaucomatous progression is: (i) loss near fixation (paracentral scotoma) to (ii) split
fixation to (iii) loss of fixation.
Ketamine (at high doses) and Succinylcholine → increase in IOP. Other anaesthe c agents diminish
intraocular pressure.
General anesthetic agents and sedatives can profoundly lower IOP. Exceptions include chloral hydrate,
which does not affect IOP, and ketamine, which may increase IOP.
The initial success rate of ALT is about 75% in POAG.
The failure rate of ALT is about 50-65% at 5 years.
The initial reduction in IOP from ALT is 30%.
The Perkins tonometer and Tonopen → can be used in the erect or supine posi on.
Digital pressure on a Goldmann lens (which is large) tends to shallow the angle, while pressure on a
smaller Zeiss lens tends to open the angle.
Paracentral scotomas in glaucoma → most commonly supero-nasally.
The alpha-2 agonists (apraclonidine and brimonidine) should be avoided in children because they have
the potential to cross the blood-brain barrier.
The most common late complication of glaucoma drainage implants → bleb encapsula on (10%).
Dipivefrin (epinephrine) → cause conjunc val injec on and CMO, par cularly in aphakic patients.
5% of normal individuals will have C/D ratios >0.6.
Less than 1% of normal individuals have an C/D ratio asymmetry of >0.2.
Disc hemorrhages may occur in as many as 1/3 of glaucoma patients at some time during the course of
their disease.
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Frequency-doubling technology (FDT) is believed to activate M cells (magnocellular ganglion cells).
Normally the superior field is depressed 1 to 2 dB compared with the inferior field.
The lack of keratic precipitates helps distinguish phacolytic glaucoma from phacoantigenic glaucoma.
Adrenergic agonists with significant α1-agonist effects (apraclonidine, dipivefrin, epinephrine) should
also be avoided in sickle cell disease because of concerns regarding anterior segment vasoconstriction.
An untreated fellow eye in a patient who has had an acute angle-closure attack has a 40% to 80%
chance of developing an acute attack of angle closure over the next 5 to 10 years.
Fibrous ingrowth is more prevalent than epithelial downgrowth.
Argon laser produces characteristic white burns on the epithelial membrane on the iris surface that can
help confirm the diagnosis of epithelial downgrowth as well as demarcate the extent of involvement.
Epithelial downgrowth takes weeks to develop.
The proper pediatric dose of acetazolamide (Diamox) is 15 mg/kg/day in three or four divided doses.
15% of patients with Fuchs heterochromic iridocyclitis will have secondary open-angle glaucoma.
(in Kanski up to 60%!)
10) Lens and Cataract
From Exams and MCQ Books
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Specular Microscopy is the best means to predict how the cornea will respond after cataract surgery.
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WHO >>The major causes of visual impairment are uncorrected refractive errors (43%) and cataract
(33%); the first cause of blindness is cataract (51%).
Posterior lenticonus is due to a type IV collagen disorder (Answer Sheet 2019)
NVI may occur after intra capsular cataract surgery
YAG laser capsulotomy increase the risk of developing RD 4 times.
Vitreous loss during cataract surgery is more common in myope.
Alport syndrome → Anterior Lenticonus, Anterior polar cataract (at birth) and Cortical Cataract,
posterior polymorphous corneal dystrophy and Megalocornea, XLR 80% and XLD 5%, COL4A5 gene
(deafness, glomerulonephritis) and retinal flecks, Hematuria.
Posterior lenticonus → more common than anterior, usually unilateral, and usually associated with
microphthalmos
Lowe's syndrome → Posterior Lenticonus, XLR, albuminuria and Fanconi syndrome of proximal renal
tubules.
Myotonic dystrophy → “Christmas tree” cataract (Polychromatic lenticular deposits by 3rd decade and
PSC by 5th decade. Associated systemic findings with myotonic dystrophy include temporal bossing,
muscular atrophy and weakness, mental retardation, and abnormalities in cardiac conduction.
Weill–Marchesani syndrome (The reverse of Marfan $) → AR or AD, short of stature and has short
stubby fingers with broad hands and tight joints and downward lens subluxation. Given the spherical
shape of the lens (high Myopic), patients are prone to angle-closure glaucoma, which is aggravated by
miosis. Cycloplegia moves the diaphragm backward and flattens the lens. It is the medical treatment of
choice.
The combination of ectopia lentis and mental retardation may be seen in:
 Weill-Marchesani syndrome
 hyperlysinaemia
 homocystinuria
Marfan → AD, BL Symmetrical superotemporal subluxation and zonules are present with intact
accommodation.
Homocystinuria → AR, BL Symmetrical inferonasal subluxation and zonules are absent with
accommodation lost, TTT with Vit.B6.
Echothiophate (Miotic) → cataract formation
PHPV → is a congenital, nonhereditary. (Typically unilateral)
PSC→ Wedl or bladder cells.
Cortical cataract → morgagnian globules.
Elschnig pearls → proliferating remnant lens epithelial cells.
LHON → TTT with Coenzyme Q
Penylketonuria → TTT with A low pheylalanine
Regarding aspiration systems in Phaco machines: (Vacuum response time)
* The Diaphragm pump → a slower build of vacuum.
* The Peristaltic pump → a rela vely rapid in vacuum. (AAO)
* The Venturi pump → the most rapid increase in vacuum.
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In peristaltic systems, occlusion of the phaco tip is required to produce vacuum. By contrast, in Venturi
systems the pump generates vacuum by creating negative pressure in a vessel by gas flowing across its
entrance. This means there is no difference between vacuum and aspiration flow rate in Venturi systems
- depressing the pedal increases vacuum, which is not dependent on occlusion.
In dual linear systems, vacuum and phaco power are controlled on different axes of the foot pedal.
Concerning cataract surgery in patients with nanophthalmos → Anterior sclerotomies may be indicated
at the time of surgery, when the anterior chamber is shallow preoperatively and the choroid is
thickened.
IOL implantation is contraindicated in patients with juvenile rheumatoid arthritis–associated iridocyclitis.
In scleral-sutured posterior chamber IOL → The needle should be passed approximately 0.75mm
posterior to the limbus, an oblique needle insertion at the 2 o’clock and 8 o’clock or 10 o’clock and 4
o’clock positions are preferable to decrease the chance of damaging the long ciliary arteries and nerves,
which tend to run in the vertical meridian.
Phenothiazines → pigmented deposits in the anterior lens epithelium in an axial configuration.
Miotics → small vacuoles within and posterior to the anterior lens capsule and epithelium that can
progress to posterior cortical and nuclear lens changes.
Statins → reduce the risk of nuclear cataracts by 50% over 5 years.
Amiodarone → in addition to cataracts (AC) can cause deposits in the cornea and optic neuropathy.
Galactosaemia type 1→ Oil droplet cataract.
(Deficiency of galactose-1- phosphate uridyl transferase).
Galactosaemia type 2 and 3 → lamellar cataract.
Type 2 (deficiency of galactokinase), while Type 3 (deficiency of UDP galactose epimerase).
The key feature clinically differentiating true galactosemia (galactose-1-phosphate uridyltransferase
deficiency) from galactokinase deficiency is the presence of hepatosplenomegaly, mental retardation,
and other systemic manifestations.
TASS presents within 12 to 24 hours, whereas acute infectious
endophthalmitis typically develops 2 to 7 days after surgery.
The Endophthalmitis Vitrectomy Study (EVS) demonstrated that
→ for with HM or better vision, vitrectomy and vitreous tap/inject were equally beneficial.
→ for pa ents with LP or worse vision, vitrectomy was found to be be er than vitreous tap/inject.
Delayed-onset postoperative endophthalmitis → The recommended ini al therapy includes a pars plana
vitrectomy with posterior capsulectomy and intravitreal injection of vancomycin with or without explant
of the IOL.
Gentamicin is occasionally used intra-vitreally in endophthalmitis for Gram-negative cover in
combination with cefuroxime. However gentamicin is retino-toxic and alternative regimes include:
 vancomycin and Ceftazidime
 vancomycin and Amikacin
Amikacin intra-vitreally → Macular infarction.
PCO formation is influenced by lens material (Hydrophilic "hydrogel" > PMMA > silicone > acrylic) and
edge design (round > square). Pooled data has found the overall PCO rate at 5 years to be 28%.
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The refractive indices of popular lens materials are:
 Hydrophobic acrylic =1.54
 PMMA = 1.49
 Hydrophilic acrylic = 1.47
 Silicone = 1.41- 1.46
Hydrophilic acrylic IOLs are the most susceptible to calcification.
A hydrophilic acrylic IOL is susceptible to opacification with use of intraocular gases during retinal
surgery or endothelial keratoplasty.
Hydrophobic acrylic IOLs have been associated with glistenings in the optic. These glistenings are water
(from the aqueous) that becomes trapped as droplets in a hydrophobic environment.
Hydrophobic acrylic IOLs optic material have shown stronger attachment to anterior capsule and by this
way it prevents epithelial cells proliferation and migration on the optic surfaces (anterior capsular
phimosis).
Hydrogel (hydrophilic) IOLs are associated with a reduced inflammatory cell reaction.
Silicone plate haptic lens → has the greatest risk of dislocation into the vitreous after a Nd: YAG
capsulotomy.
The most common source of organisms leading to post-operative endophthalmitis is → the pa ent's lids
and Conjunctiva.
The primary abnormality seen in ptosis which develops after cataract surgery is in the levator
aponeurosis (This may be due to the effect of anaesthetic injections, lid specula, and bridle sutures).
Alfuzosin, doxazosin, terazosin, prazosin and tamsulosin (Alpha-1 antagonists) → may cause floppy iris
syndrome.
Cohesive viscoelastics offer a better view. Dispersives tend to trap air bubbles which can be a
nuisance for the view during surgery.
The Zeiss IOLMaster is a non-contact method that uses two coaxial laser beams that are partially
coherent. The beams produce an interference pattern that is used to interpret measurements, thus the
technology is referred to as partial coherence interferometry. It is highly reproducible and less operatordependent than ultrasound biometry. The machine can measure keratometry, axial length, anterior
chamber depth and corneal white-to-white diameter.
ICCE is contraindicated in paediatric eyes because they have more prominent hyaloideocapsular
attachments, leading to high chance of inducing retinal tears from traction.
In A-scan biometry, a one diopter IOL power error could result from an axial length error of 0.4mm.
A younger patient's lens epithelial cells are more actively growing than an older patient's and are thus
more susceptible to radiation damage.
Regarding refractive surprise of -1.50D after routine Phaco and PCIOL;
* Posterior dislocation of an IOL and cystoid macular oedema would both tend to produce hyperopic
(not myopic) shifts.
* In routine cataract surgery with in-the-bag IOL placement and intact zonules, anterior dislocation of
the IOL to produce 1.50D of myopic shift is unlikely.
* Retained viscoelastic between the lens and the posterior capsule (capsular bag distension syndrome) is
the most probable cause of inducing a myopic shift.
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Syndromes featuring congenital cataract + facial abnormalities?
1. Crouzon syndrome → craniosynostosis, small orbits, proptosis, and cranial changes that affect facial
structure.
2. Hallermann-Streiff syndrome → mandibular hypoplasia with a "bird face."
3. Stickler's syndrome → maxillary and mandibular hypoplasia, epicanthus, a long philtrum, and the
Pierre-Robin's anomaly.
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Causes of microspherophakia include:
 Sporadic
 Weill-Marchesani syndrome (the most common hereditary cause)
 Alport's syndrome
 Marfan's syndrome
 Peter's anomaly
 Congenital rubella
Note: Lowe's syndrome is associated with microphakia
Unilateral congenital cataracts are usually sporadic without a family history or systemic disease.
Affected infants are usually full-term and healthy.
As a general rule of thumb, a toric IOL loses one third of its power for every 10 degree rotation off
alignment. By 30 degrees, the toric IOL has lost most of its power (equivalent to a spherical lens).
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A 30 degree rotation of the lens keeps the same magnitude of cylinder but flips the axis to a new
meridian.
Peribulbar anaesthetic requires greater volume than retrobulbar anaesthetic and therefore causes a
higher intra-orbital pressure. It also results in less akinesia compared to retrobulbar and causes more
chemosis. However, the advantage of peribulbar is that there is a lower risk of optic nerve damage,
globe perforation and systemic neurological effects of anaesthesia.
Intrauterine infections that cause cataract include: (TORCH)
 toxoplasmosis
 other: HIV
 rubella
 CMV
 Herpes: simplex and zoster (extremely unusual)
Tuberculosis does not cause congenital cataract.
Drugs causing cataract include:
 chlorpromazine (a phenothiazide): fine yellow-brown granules on anterior lens capsule
 gold: innocuous, anterior capsular deposits in 50% of patients
 busulphan: a cancer drug
 allopurinol
 corticosteroids
 Amiodarone (AC)
 Echothiophate
 Chloroquine (however, there is no evidence for an association between hydroxychloroquine and
cataract).
Both amiodarone and phenothiazine cause stellate cataracts in the anterior lens capsule.
Uveitis-glaucoma-hyphaema (UGH) syndrome is most commonly caused by the placement of an illfitting or mal-positioned anterior chamber IOL. This results in iris and angle trauma, with chronic
inflammation, secondary iris neovasculariation, recurrent hyphaemas and glaucoma. The syndrome can,
however, occur with posterior chamber lenses and suture-fixated lenses.
Sulcus IOL’s often cause pigment chaffing from the iris, which may lead to raised IOP. UGH is
uncommon with modern IOLs, even those in the sulcus.
Nanophthalmic eyes have a higher complication rate (Potential complications include);
 retinal detachment
 choroidal effusion
 post-op angle closure glaucoma
 flat anterior chamber
 cystoid macular oedema
 corneal decompensation
 malignant glaucoma
Phaco with IOL is generally the procedure of choice, using the smallest incision possible. In patients with
glaucoma, cataract extraction may be combined with trabeculectomy. When the anterior chamber is
Abdullah Fouad Notes
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shallow pre-operatively and the choroid is thickened, anterior sclerotomies are indicated at the time of
surgery to reduce the chances of uveal effusion.
The Royal College of Ophthalmologists guideline for cataract surgery (2010) recommends that:
 The main corneal section is sutured in children to prevent wound leak and astigmatism.
 Intravenous access is recommended for peribulbar and retrobulbar anaesthetic.
 If the departmental endophthalmitis rate is higher than that of the published Bolton study (0.055%),
then intracameral cefuroxime should be considered
 While most modern formulae work well in the normal axial length range, for eyes <22mm "the Haigis
and Hoffer Q may be slightly better."
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Hereditary fundus dystrophies such as RP, Leber's congenital amaurosis, gyrate atrophy and Sickler's
syndrome may be associated with PSC.
Lamellar cataract may be inherited or caused by:
 rubella
 diabetes
 galactosaemia
 hypocalcaemia
Anterior chamber IOL's can cause corneal decompensation from loss of endothelial cells, especially if the
ACIOL is mobile and ill-fitted.
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Corneal over-tightened sutures cause steepening of the same meridian of the wound.
When using standard IOL calculations in patients who have undergone myopic LASIK, hyperopia after
cataract surgery is common, because the standard calculations lead to overestimation of central corneal
power. The opposite occurs after hyperopic LASIK. To overcome this, special post-LASIK formulas such as
the Haigis-L are advised.
In cortical cataracts, there is hydropic swelling of lens fibres. Eosinophilic globular material may be
found between lens fibres called morgagnian globules.
In nuclear cataract there is homogenous loss of cellular laminations.
In posterior subcapsular cataract there is posterior migration of lens epithelial cells, which swell to
become Wedl or bladder cells.
The most common type of congenital cataract > Lamellar.
There are five A-scan peaks in a phakic eye:
1- corneal surface 2- lens anterior surface 3- lens posterior surface 4- ILM of the retina 5- sclera
Congenital cataracts and positive findings on urinalysis:
 Galactosaemia: reducing substance in urine
 Lowe's: aminoaciduria
 Fabry's: sediments
 Wilson's: copper
 Alport's: blood (glomerulonephritis)
Neurofibromatosis type 2, not type 1, may be associated with Cataract (PSC).
Viscoelastics (including sodium hyaluronate, chondroitin sulfate and hydroxymethylcellulose) all
produce pressure spikes at a mean of 4 hours (+/- 1 hour) after surgery
Cataract in systemic disease
- Wilson disease → Anterior capsular ‘sunflower’ cataract
- Diabetes mellitus → snowflake cortical
- Myotonic dystrophy → Christmas tree cataract
- Atopic dermatitis → Shield-like dense anterior subcapsular plaque that wrinkles the anterior capsule
(PSC - may also occur).
- Neurofibromatosis type 2 → posterior subcapsular or capsular, cortical or mixed.
- Galactosaemia type 1→ Oil droplet cataract. (Deficiency of GPUT).
- Galactosaemia type 2 and 3 → lamellar cataract.
Type 2 (deficiency of galactokinase), while Type 3 (deficiency of UDP galactose epimerase).
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(Opacity develops withins the first few days or weeks of life "not congenital")
(Summarized from Kanski 8)
Cataract Backlog → the es mated number of persons/eyes with operable cataract in the population.
The term ‘operable’ cataract is used to define a cataract where the patient and the surgeon agree to
proceed with cataract surgery.
The cataract surgical coverage (CSC) → the propor on of pa ents (or eyes) with “operable” cataract,
who have already received surgery (the indicator used to estimate the extent to which a population’s
cataract surgical needs have been met. CSC is expressed as the proportion of visually impaired
individuals with cataract who require surgery who actually receive it.)
To define and demonstrate effective cataract surgical coverage (eCSC), a candidate UHC indicator that
combines a coverage measure (cataract surgical coverage, CSC) with quality (post-operative visual
outcome).
Cataract Surgical Rate (CSR) → The number of cataract operations performed per year, per million
population.
The main aims of RAAB (Rapid Assessment of avoidable blindness) are:
- to estimate the prevalence and causes of avoidable blindness and visual impairment in people aged 50
and above
- to assess cataract surgical coverage (CSC)
- to identify the main barriers to the uptake of cataract surgery
- to measure outcome after cataract surgery.
Other age-related lens changes include decreased concentrations of glutathione and K and increased
concentrations of Na and Ca in the lens cell cytoplasm. (AAO)
Alkali compounds penetrate the eye readily, causing an increase in aqueous pH and a decrease in the
level of aqueous glucose and ascorbate (Cataract formation)
Sunlight is associated with an increased risk of cortical cataracts. (AAO)
Nuclear cataracts are not causally associated with exposure to solar UVB.
https://bjo.bmj.com/content/bjophthalmol/78/6/478.full.pdf
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Retrobulbar bupivacaine (0.75%) administration has been implicated as a causative factor in respiratory
distress, cardiovascular dysfunction, and various central nervous system disturbances.
Intermittent touch syndrome → Intermi ent touch of a pseudophakos to the corneal endothelium is
heralded by three signs:
1- Ciliary flush
2- Local corneal dystrophy
3- Cystoid macular edema.
Post-traumatic endophthalmitis has worst visual prognosis than other categories. Eyewiki.org
The incidence of clinically relevant CMO post-cataract surgery is about 1% across studies,
although angiographic and OCT evidence of CMO may be found in up to 10% of patients.
(The peak incidence of both occurs 6–10 weeks after surgery)
Spontaneous resolution occurs in approximately 95% of uncomplicated cases, usually within 3–12
months.
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Uncomplicated cataract surgery in Glaucoma Pt who is on PG, then CME occurs → topical NSAID,
steroid, and discontinue PG (give another gl. TTT)
PCO, glaucoma (ACG caused by excessive inflammation), and RD (lifetime risk about 3%) are more
common postoperative complications in children undergoing cataract extraction. The incidence of CME
is lower in children than in adults.
Lamellar or zonular cataracts (most common congenital type), bilateral and symmetrical, AD inheritance
and in some cases due to transient toxic effect in the embryonic life.
Anterior polar cataract and nuclear cataract are usually static, although they may rarely progress.
Cataracts that typically progress include posterior lenticonus, persistent hyperplastic primary vitreous,
lamellar, sutural, and anterior or posterior subcapsular. They usually have a better prognosis because
they only usually begin to obstruct the vision after the critical period of visual development has passed.
Photostress recovery test: determine best-corrected vision, shine bright light into eye for 10 seconds,
record time for vision to recover within 1 line of best-corrected vision; test each eye separately; invalid
for eyes with vision worse than 20/80 (6/24)
 Optic nerve disease: normal recovery time (< 60 seconds)
 Macular disease: prolonged time (> 90 seconds)
(Friedman Review of Ophthalmology 3rd Edition)
11) Retina and vitreous
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From Exams
The intravitreal antibiotic regimen that was used in endophthalmitis vitrectomy study >> Vancomycin
and Amikacin
Autofluorescence can be detected on standard fundus photography >> with the excitation and barrier
filters both in place (both filters on)
Obesity and a poor diet predispose to AMD
Regarding wet AMD → AVEGF may slow visual loss in up to 90% of affected people
Dry AMD → comprising around 90% of diagnosed disease.
According to Age-Related Eye Disease Study 2 (ARED2) Beta-carotene (anti-oxidant) almost certainly
increases the incidence of lung cancer in current and former smokers.
Regarding ARMD → a gene c component can be iden fied in up to 50-75% of cases. ‘’Tyr402His at
10q26 (Complement factor H locus), and Ala69Ser (LOC387715)’’
Incontinentia pigmenti >> loss of pericytes and BM thickening.
Age at onset significantly modifies the long-term risk of proliferative retinopathy in Type 1 Diabetes:
- The highest risk → 5–14 years
- The lowest risk → 15–40 years.
- Onset at age <5 years may have a protective effect on the development of retinopathy
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The Diabetic Control and Complications Trial (DCCT) is a randomized, controlled clinical trial designed
to assess the relationship between glycemic control and the development, progression, or amelioration
of early vascular complications in persons with insulin-dependent diabetes mellitus (IDDM).
- Intensive blood glucose control reduced the progression of diabetic retinopathy by 76%.
- slowed the progression of retinopathy by 54%
- reduced the development of proliferative or severe non proliferative retinopathy by 47%
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DRVS (Diabetic Retinopathy Vitrectomy Study) results:
- Eyes of patients with type 1 diabetes mellitus and severe vitreous hemorrhage clearly demonstrated a
benefit from earlier vitrectomy
- Eyes of patients with type 2 or mixed diabetes don’t.
Ranibizumab (lucentis) and Bevacizumab (Avastin) → work by Binding directly to VEGF.
In BRVO → the supero-temporal branch vein is most commonly affected.
In BRVO → Iris NVs 2-3%, Retinal NVs 8% by three years; the risk is much higher in eyes with more than
about 5 disc areas of non-perfusion on FA. (Kanski)
Regarding sickle cell anaemia → AR, under the age of 5 years, as many as 15% of children develop
sepsis or meningitis (Children in this age group develop pneumococcal sepsis with 400 times the
frequency of the general population)
CSR typically affects one eye of a young or middle-aged Caucasian man; women with CSR tend to be
older. Pregnancy is a common risk factor.
H. pylori has been linked to CSCR.
Regarding photodynamic therapy (PDT) → photosensitivity of the skin, attributed to the administration
of verteporfin.
Locations of retinoid binding proteins, retinoid receptors and biosynthetic enzymes:
(https://www.ncbi.nlm.nih.gov/pubmed/10800207)
- Cellular (retinaldehyde = retinal) binding protein (CRALBP) → in Müller cells, pigmented epithelium,
choroid, and fibrous sclera (came in 2008 exam)
- Cellular retinol binding protein (CRBP) → in the pigmented epithelium
- Cellular retinoic acid binding protein (CRABP) → in amacrine and bipolar cells in the retina
- Retinaldehyde dehydrogenase (RALDH) → in retinal amacrine cells, pigmented epithelium, and choroid
- Retinoic acid receptors (RAR) → in amacrine cells, choroid, and chondrocytes and fibroblasts in the
sclera
- Retinoic acid receptors RXR → in amacrine and ganglion cells, bipolar cell nuclei, choroid, and
chondrocytes.
Regarding RP → Pa ents with the same gene defect can have variable severity of disease at a given age
and variable retinal appearances.
Retinal densitometry → is used to measure the amount of bleached rhodopsin in the outer segments of
the rod receptors.
BEST Vitelliform Macular Dystrophy → EOG Arden index less than 1.5
Choroideremia (CHM) → is an X-linked monogenic disease caused by various mutations in the CHM
gene that result in the loss of function of Rab escort protein (REP-1) and cause slow degeneration of the
retinal pigment epithelium (RPE), choroid and photoreceptors.
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Treatment of symptomatic horseshoe tears reduces the risk of RD to 5%
15% of patients with symptomatic PVD have a retinal tear, as opposed to 50%-70% of patients with PVD
+ vitreous haemorrhage.
PVD affects most eyes by the eighth decade of life (in 80 years old is greater than 60 %). Age at onset is
generally in sixth to seventh decade.
PVD occurs earlier in myopic eyes, in eyes with inflammatory disease ‘uveitis’ and following blunt
trauma or cataract surgery (especially when there is surgical vitreous loss).
PVD with pigmented cells (shafer sign or tobacco dust) in the vitreous is suggestive of Retinal tear.
Regarding Lattice degeneration:
→ Peak incidence is in the 2nd and 3rd decades.
→ Bilateral in 30-50% of cases
→ Parallel to the ora serrata in a circumferen ally oriented pa ern.
Asymptomatic retinal holes are present in over 4-5% of the population.
In patients with retinal detachment, the fellow-eye risk of detachment is approximately 10% for phakic
retinal detachment and as high as 20%–36% for aphakic or pseudophakic detachment.
Approximately 40-50% of all patients with detachments have myopia, 30-40% have undergone cataract
removal, and 10-20% have encountered direct ocular trauma.
Silicone oil also has lower solubility for various inflammatory and vasogenic mediators.
The risk of RD following ECCE → 11 mes higher in the 1st year, 4 times in year 5-20 (The cumulative risk
of retinal detachment steadily increased to 1.79% at 20 years)
Treatment of retinal holes without detachment is nearly 100% successful.
Regarding degenerative retinoschisis → affects about 5-7% of the adult population, most common in
inferotemporal quadrants, can be monitored by an annual visual field test.
In auto-immune retinopathy (AIR) → (ERG) changes are seen earlier than (OCT) changes.
Hydroxychloroquine retinopathy is most influenced by daily dose, length of use, and cumulative dose
over time. Risk for toxicity is least with less than 5 mg/kg/day for hydroxychloroquine and 2.3 mg/kg/day
for chloroquine.( Cumulative use in excess of 250 grams increases the risk for toxic retinopathy)
(Patients are at low risk during the first 5 years of treatment)
Susac syndrome → This condition may also be referred as;
- SICRET : small infarctions of cochlear, retinal, and encephalic tissue.
- RED-M microangiopathy with retinopathy, encephalopathy, and deafness.
- Retinocochleocerebral vasculopathy.
concomitant use of ACE inhibitors and antidiabetic medicines (insulin, oral hypoglycaemic agents) may
cause an increased blood glucose lowering effect with the risk of hypoglycaemia, especially in the first
weeks of combined treatment and in patients with renal impairment.
People with Type 1 diabetes should have annual examinations for diabetic retinopathy beginning five
years after the onset of their disease, while those with Type 2 diabetes should have a prompt
examination at the time of diagnosis, then at least yearly examinations thereafter.
https://www.aao.org/clinical-statement/screening-diabetic-retinopathy
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High density lipoprotein (HDL)
At risk
Desirable
Men
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Less than 40 mg/dL (1.0 mmol/L) 60 mg/dL (1.6 mmol/L) or above
Women Less than 50 mg/dL (1.3 mmol/L) 60 mg/dL (1.6 mmol/L) or above
Prothrombin gene mutation causes the body to produce excess amounts of prothrombin → a risk factor
for thrombosis.
Calcium Channel Blockers (CCBs) block the entry of calcium into vascular smooth muscle cells, resulting
in reduced myocardial contractility and decreased systemic vascular resistance.
Methyldopa is a drug of first choice for control of mild to moderate hypertension in pregnancy.
There are some blood pressure medications that you should avoid during your pregnancy. These
include: ACE inhibitors (Lisinopril).
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From MCQ Books
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The majority of CRVOs are associated with hypertension. However, in the younger population, a
secondary systemic etiology may be the cause such as protein C/S deficiency, or elevated serum
homocysteine, or a hyperviscosity syndrome such as multiple myeloma or Waldenstrom’s (abnormal
serum electrophoresis).
The following Figure shows the appearance of multiple peripapillary cotton-wool spots in an
asymptomatic patient that are characteristic of interferon retinopathy. DD → DM,HTN,HIV
Abdullah Fouad Notes
Page 82
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The reported incidence of endophthalmitis following intravitreal injection of anti-VEGF agents →
between 1 in 2,000 and 1 in 3,000.
The incidence of endophthalmitis after intravitreal triamcinolone → between 1-2%. However, this also
includes cases of pseudo-endophthalmitis (sterile endophthalmitis), which account for the majority.
Excluding pseudo-endophthalmitis, the prevalence is approximately 0.1- 0.6%.
According to the ANCHOR clinical trial regarding the use of intravitreal ranibizumab for (Wet ARMD),
approximately 30% of patients gain at least three lines of vision after 2 years of therapy, and 90% of
patients lost less than three lines of vision.
Coats disease → unilateral, boys, Bimodal age distribution (Juvenile <10ys & adult >16ys
‘’↑Cholesterol’’)
Renal cell carcinomas may be found in up to 25% of patients with von Hippel Lindau disease (retinal
hemangioblastomas).
The most common ocular manifestation of congenital CMV infection → retinochoroiditis.
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CVM Retinitis → Spot diagnosis (Rhegmatogenous RD may be associated 25 %)
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Abdullah Fouad Notes
Page 83
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To differentiate melanomas from choroidal nevi
“To Find Small Ocular Melanoma.”
T = thickness >2 mm, F = fluid, S = symptoms, O = orange pigmentation (lipofuscin), M = margin-touching
optic disk.
MPS that is associated with RPE degenerations (Pigmentary retinopathy)
Huria Hunted Sandfly in the Sky (My Own Mnemonic)
Hurler, Hunter, Sanfilippo, and Scheie.
All MPS associated with corneal clouding except → Hunter, Sanfilippo
Maroteaux-Lamy and Morquio → accumula on of dermatan sulphate, causes corneal clouding and op c
atrophy.
All MPS has normal IQ except → Hurler, Sanfilippo
Retinal crystals → may be seen in patients using tamoxifen, canthaxanthine, methoxyflurane, or talc
crystals.
A copper IOFB → can cause Reversible flat ERG.
Commotio retinae: an actual disruption or destruction of retinal photoreceptor elements and
photoreceptor cells in the outer layers of the retina. It does not represent retinal edema.
In order of frequency, the likelihood of traumatic retinal tears after blunt ocular injury is as follows:
1- Inferotemporal dialysis
2- Superonasal dialysis
3- Giant retinal tears
4- Flap tears
5- Tears around lattice
Terson Syndrome → (Re nal and/or VHs) + (subarachnoid or subdural hemorrhages).
About 20% of patients with spontaneous or traumatic subarachnoid hemorrhages will present with
intraocular hemorrhages.
Punctate Inner Choroidopathy (PIC) is an entity that is slightly more common in myopic women in the
3rd and 4th decades of life. It may be associated with recurrent CNVMs.
Macular pucker → may be associated with Retinal breaks.
Abdullah Fouad Notes
Page 84
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Roth spot
(APMPPE) is associated with → Viral prodrome. FA: Early hypo, late hyper. ICGA: hypo
Incontinentia pigmenti is associated with → ROP-like proliferative retinopathy (vitreoretinal dysplasia
with proliferative retinopathy, retrolental masses, leukocoria).
A large angle kappa occurs when there is temporal displacement of the macula, as can occur with:
- ROP
- FEVR
- combined hamartoma of RPE and retina
- persistent posterior fetal vasculature.
High myopia is likely to cause a negative angle kappa, which leads to pseudo-esotropia. A large angle
kappa causes a pseudo-exotropia.
Spontaneous retinal dialyses, often in patients with a family history of retinal dialysis and/or RD.
Spontaneous retinal dialyses > Inferotemporal
Traumatic retinal dialyses > Superonasal
Dialysis May Cause RD > Slowly progressive
Usher’s syndrome is the most common cause of combined deafness and blindness.
Abdullah Fouad Notes
Page 85
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The incidence of cobblestone degeneration in the adult population is approximately → 25%
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EOG reflects the activity of the RPE and the photoreceptors. This means that an eye blinded by disease
proximal to the photoreceptors will have a normal EOG.
The EOG is particularly useful in Best disease (Best vitelliform dystrophy) where the light rise is
consistently abnormal while the ERG is usually unaffected.
The Arden ratio of EOG, the ratio of the Light peak (Lp) to dark trough (Dt) is used to determine the
normalcy of the results. An Arden ratio of 1.80 or greater is normal, 1.65 to 1.80 is subnormal, and <
1.65 is significantly subnormal.
Regarding ERG → Rods cannot respond to stimuli flickering above 20 Hz, meaning the 30 Hz flicker
isolates the cone response. Cone responses can be elicited in normal eyes up to 50 Hz, after which point
individual responses are no longer recordable (‘critical flicker fusion’). (20+30=50)
Causes of a negative ERG (Selective Absence of b-wave or enverted ERG):
 Congenital stationary night blindness
 X-linked retinoshisis
 CRAO
Enophthalmos as a result of resorption of orbital fat → Leber's congenital amaurosis (Oculodigital
syndrome may occur, in which constant rubbing of the eyes by the child causes enophthalmos as a
result of resorption of orbital fat).
The duration of intra-ocular gases are as follows:
 air: 5 days
 SF6: 2 weeks
 C3F8: 2 months
SF6 has the highest expansile rate for any intraocular gas, which can cause dramatic spikes in IOP in the
post-operative period.
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Abdullah Fouad Notes
Page 86
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C3F8 expands 4 times its volume at 100% concentration and lasts 8 weeks. The non-expansile
concentration used in vitrectomy is between 12-16% with air.
SF6 doubles its volume in 2 days and lasts for 2 weeks. Its non-expansile concentration is between 2030% with air.
70% of retinal tears occur between 10 o'clock and 2 o'clock.
Cancer associated retinopathy (CAR) is most commonly associated with small cell lung cancer, followed
by gynaecological and breast cancer.
An absolute scotoma suggests retinoschisis while a relative scotoma suggests RD.
Refraction
Location
Scotoma
Pigments
Surface
Shifting Fluid
Reaction to PRP
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Retinoschisis
myope
supero-temporal
relative
present
corrugated
may be present
no reaction
RD
hypermetrope
infero-temporal
absolute
absent
smooth
absent
reaction
Idiopathic Epiretinal membranes are bilateral in 10-20% of patients.
The Mizuo-Nakamura phenomenon (golden yellow colour in the light-adapted state, which becomes
normal after prolonged dark adaptation) has been described in;
 X-linked cone dystrophy.
 Oguchi disease (CSNB)
Sudden onset flashes and floaters in the presence of tobacco dust is a retinal tear until proven
otherwise. RD may or may not develop and would be less likely in the absence of peripheral field loss.
Key features of Bardet-Biedl syndrome are:
 retinitis pigmentosa
 obesity
 hypogonadism
 polydactyly
 short stature
 mental retardation
Note: Bardet-Biedl is closely related to Lawrence-Moon syndrome and the two are considered part of
the same spectrum: thus Bardet-Biedl-Lawrence-Moon.
The retinal circulation has a low flow rate (25mm/s) and a high oxygen exchange. By contrast, the
choroidal circulation has a high flow-rate and a low oxygen exchange.
If choroidal folds → do B-scan ultrasound
Susac syndrome (retinocochleocerebral vasculopathy) → triad of encephalopathy, hearing loss and
embolic branch RAO.
Radiation retinopathy may occur with doses of approximately 3,000 rad (30 Gy) or more.
The biggest risk factor of RD is; (in order)
1- RD in the fellow eye
Abdullah Fouad Notes
Page 87
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2- Myopia
3- FH of RD
4- Lattice degeneration.
A capillary haemangioma fills in the early arterial phase, not arteriovenous phase.
JXLR → Foveal schisis
Foveal hypoplasia associated with:
 Albinism
 Aniridia
 PHPV
 ON hypoplasia
CMO → epinephrine, latanoprost and nico nic acid are recognized causes.
Rods are approximately 1,000 times more sensitive than cones during dark adaptation.
PVD is protective against the progression of diabetic retinopathy including the development of NVD,
NVE and diabetic maculopathy.
Conditions that produce CMO without leakage include:
 Golmann-Favre
 juvenile x-linked retinoschisis
 retinitis pigmentosa
 nicotinic acid maculopathy
Asteroid hyalosis (Calcium phosphate) → has been associated with DM, doesn’t sediment inferior.
Synchysis scintillans (Cholesterolosis bensom) → sediment inferior
MEWDS typically presents with shimmering photopsias and paracentral scotomas.
The peak incidence of Irvine-Gass syndrome is at 6-10 weeks postoperatively.
RB cells derive from neuro-epithelial cells and have the potential to differentiate into photoreceptors or
Muller cells.
In order of increasing maturity:
 Homer-Wright rosettes (the most immature)
 Neuroblasts
 Flexner Wintersteiner rosettes
 Early retinal cell
 Fleurettes
 Immature photoreceptor cells.
(Although the type of cells is not a prognostic indicator).
Some popular genetic correlations are listed below:
Chromosome
13q14
1p
19
5q
Condition
retinoblastoma
Stargardt's
myotonic dystrophy
granular, Avellino, Reis-Buckler and
Lattice I dystrophies
Abdullah Fouad Notes
Page 88
15
11p
20
HLAB51
HLA A29
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Marfans
aniridia
Alagille syndrome
Behcet's disease
Birdshot chorioretinopathy
Mulberry-like lesion in each fundus → Tuberous sclerosis.
The prevalence of bilaterality of idiopathic macular holes, including earlier stages before a full
thickness dehiscence develops, approaching 20% to 25%. Because a large proportion of early holes
spontaneously resolve, only a small fraction (5%) develop bilateral full thickness defects.
Proliferative vitreoretinopathy (PVR) is the most common cause of late failure after sclera buckling.
The most common cause of early failure following retinal detachment surgery is missed breaks, while
PVR is the commonest cause of late failure.
The most common cause of recurrent retinal detachment following scleral buckle repair is PVR.
Ishihara plates detect only red-green defects.
The best laser for retinal photocoagulation in the setting of dense cataract or vitreous haemorrhage →
krypton red (poorly absorbed by haemoglobin or red blood cells, Red light penetrates cataract and
vitreous hemorrhage better than lights of other wavelengths.). In addition, because it is poorly absorbed
by xanthophyll, it is useful for macular laser treatment.
Pseudo-autoflourescence occurs due to overlap in the spectral transmission of the excitation and barrier
filters, meaning that some blue reflected light does pass through the barrier (yellow-green filter) and
appear as fluorescence.
The ICG dye absorbs light of wavelength 805nm and reflects light of wavelength 835nm, both being in
the infrared spectrum.
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Abdullah Fouad Notes
Page 89