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for Medical Students
A Syllabus for the Clerkship in Ophthalmology

Cynthia S. Chiu, MD
Assistant Professor of Ophthalmology
Weill School of Medicine, Cornell University
New York Presbyterian Hospital
with contributions by: D. Jackson Coleman, MD, Kip L. Dolphin, MD, and Thomas C. Lee, MD

Section 1: Ophthalmic Anatomy

The eye is an extension of the central nervous
system. In fact, the retina is the only part of the
brain you can visualize without the aid of neuroimaging. The eye is designed much like a
camera, and the tissues along its axis are
translucent to allow the penetration of light.
The anatomy of the globe can be divided into 3
layers: exterior, uvea, and interior, with associated
attachments. The exterior layer consists of
tissues that form the wall and the structure of the
eye, and includes the cornea, conjunctiva, and
sclera. The uveal layer is comprised of all the
vascular structures, including the iris, ciliary body,
choroid, and blood vessels. The interior layer
contains the aqueous humor, the crystalline lens,
the vitreous humor, and the neurosensory retina.
Attached to the globe are the optic nerve, the
extraocular muscles, and the cranial nerves.
The retina is an intricate network of neurons
which, analogous to the film in a camera, absorbs
the visual image. Histologically, the retina is
organized into 8 layers; counter-intuitively light
activates the outermost layer (closest to sclera)
first, and then neurotransmission proceeds inward
(toward the vitreous) until the axons of the
ganglion cells coalesce into the optic nerve.
Following the passage of light, the layers are: rodcone outer segments, the outer nuclear layer (the
cell bodies of the photoreceptors), the outer
plexiform layer, the inner nuclear layer (the cell
bodies of interneurons such as bipolar, amacrine,
and horizontal cells), the inner plexiform layer, the
ganglion cell layer, and the nerve fiber layer (the
axons of the ganglion cells). Outside of the
photoreceptor outer segments, the 8 layer is the
retinal pigment epithelium, which maintains the
health of the outer segments, helps recycle
rhodopsin, and comprises the retinal blood-brain
The orbit is a complicated 3-dimensional space
made up of 7 bones: ethmoid, frontal, lacrimal,
maxillary, palatine, sphenoid, and zygomatic. The
important apertures of the orbit include the optic
canal (for passage of the optic nerve and
ophthalmic artery), the superior orbital fissure (for
passage of CN III, IV, V, VI, sympathetic nerves,
and the superior ophthalmic vein), and the inferior
orbital fissure (for passage of the inferior
ophthalmic vein and CN V). The supraorbital and
infraorbital notch/foramens allows passage of CN
V1 and V2 to innervate the frontal and maxillary
regions, respectively.

Six extra-ocular muscles are attached to the

globe, four recti (superior, inferior, medial, and
lateral), and 2 obliques (superior and inferior).
The eye and orbit are served by 6 cranial nerves.
CN II is the optic nerve, transporting visual
information to the brain. CN III provides motor
input to the superior, medial, and inferior recti, the
inferior oblique, and the eyelid retractors. CN III
also provides parasympathetic innervation, via the
ciliary ganglion, located just posterior to the globe.
CN IV innervates the superior oblique. CN V
provides sensory input to the eye and orbit. CN
VI innervates the lateral rectus. CN VII provides
motor input to the orbicularis oculi (eyelid
The lacrimal gland sits in the superotemporal
quadrant of the orbit. In contrast, the nasolacrimal
sac sits in a fossa along the inferonasal orbital
rim. The nasolacrimal system begins with upper
and lower canaliculi located along the nasal
aspect of the eyelids. These canaliculi drain tears
from the conjunctival fornices into the lacrimal
sac. The sac is then drained by the nasolacrimal
duct into the inferior meatus of the nose.

The visual pathway extends into the cranial cavity

via the optic nerve. The optic nerves cross at the
chiasm, where the nasal fibers (which actually
serve the temporal visual field) decussate. From
that point onward, each hemisphere contains
neural signals from both eyes for the contralateral
visual field. Posterior to the chiasm, the fibers
form the optic tracts, then the optic radiations
(located in the temporal and parietal lobes), and
finally the visual cortex along the calcarine fissue
of the occipital lobe.


Section 2: Trauma and Ophthalmic Emergencies

Subconjunctival Hemorrhage
Most simply put, a subconjunctival hemorrhage is a bruise
under the conjunctiva. This occurs when a conjunctival
capillary breaks and leaks blood into the subconjunctival
space. Without a history of trauma, this commonly occurs
with valsalva (cough, sneeze, screaming, etc.), eye
rubbing, or may be spontaneous with a history of the use
of aspirin, NSAIDs, or anti-coagulants. However, in the
setting of trauma, if the hemorrhage is dense and opaque,
a scleral rupture can be hidden beneath it, and the patient
must be ruled-out for ruptured globe. Subconjunctival
hemorrhages resolve spontaneously over 1-2 weeks, and
unnecessary anti-coagulants should be held until
Corneal Abrasion
A corneal abrasion is a break in the corneal epithelium.
This can occur with both blunt (finger-poke) and shearing
trauma (paper edge), or even prolonged exposure and
drying (poor lid taping during general anesthesia). The
corneal surface is the most densely innervated sensory
surface of the body, and abrasions are exquisitely painful.
Luckily, the corneal epithelium regenerates quickly, and
with proper lubrication (antibiotic ointment) and analgesia,
abrasions will heal spontaneously within days. Linear
abrasions such as in this photo imply a foreign body under
the eyelid, and these must be removed.
Corneal Foreign Body
Metal, rust, and other particulate foreign bodies can
become imbedded in the corneal stroma. These injuries
often occur on-the-job, with metal work or explosions.
Metal and rust are particularly toxic to the cornea and
must be removed, usually with a tuberculin syringe needle
or a drilling burr at the slit lamp. Care must be taken to
remove as much foreign material as possible, while
leaving enough corneal stroma to avoid perforation. Once
a foreign body is found on the globe, the eye must be
dilated to ensure there is no globe perforation or
intraocular foreign bodies. To speed healing and prevent
a secondary corneal ulcer, patients are treated with
antibiotic ointment and frequent follow-up.
A hyphema is a layered blood clot within the anterior
chamber. During blunt trauma, compressive forces cause
fragile capillaries within the iris and ciliary body to break,
and with sufficient bleeding, a clot will form. A hyphema is
an indication that significant injury has occurred to the
globe. Most hyphemas resolve spontaneously with topical
steroids, cycloplegics (dilating drops), and activity
restriction. However, these patients are prone to
rebleeding, acute and chronic glaucoma, and permanent
damage to the iris and angle. Very large clots filling the
anterior chamber are called 8-ball hyphemas (after the
black 8-ball in billiards) and may need surgical evacuation.

Acute Angle-Closure Glaucoma

Angle-closure glaucoma usually occurs in middle-aged
hyperopic (far-sighted) patients with cataracts. The
combination of short globe length (common in hyperopes)
and a large crystalline lens (cataract) can cause the pupil
to become obstructed against the lens in a mid-dilated
position. Aqueous humor is unable to circulate to the
anterior chamber and pushes the peripheral iris forward,
closing the angle, where fluid normally drains from the
eye. Aqueous continues to accumulate behind the lens
and iris, and the intraocular pressure increases
dramatically, causing pain and loss of vision. Temporary
therapy consists of pressure-lowering medications (topical
and systemic), but definitive treatment is a laser iridotomy
(hole in the iris) to create an opening by which aqueous
can bypass the blockage to reach the drainage canals in
the angle.
Ruptured Globe
In addition to penetrating injuries, the globe can rupture if
sufficient blunt compressive force is applied. The
structurally weakest areas of the globe are just posterior to
the attachments of the extraocular muscles and at the
limbus. In addition, prior surgical wounds, like those made
for cataract surgery, are never as strong as native tissue
and may open preferentially. Patients with suspected
globe rupture should undergo thin-cut orbital CT, to
evaluate the extent of injury. MRI is contraindicated due
to the risk of metal foreign body. Treatment for ruptured
globes is surgical exploration and repair. Systemic
antibiotics and tetanus prophylaxis are also given.
Retinal Detachment
Patients with retinal detachment complain of vision loss,
photopsias (flashing lights), and floaters. Though
detachments can occur in the setting of trauma, they also
occur spontaneously, especially in high myopia (nearsightedness) and with pre-existing retinal holes or tears.
Retina that is detached for a prolonged period of time will
atrophy and cause permanent visual loss, therefore
surgical repair should be expedited. Depending on the
size of the detachment, various surgical procedures can
be used for repair: cryo (freezing), pneumo (injection of
gas bubble), scleral buckle (silicone ring around the
equator of the globe), and vitrectomy.
Shaken Baby Syndrome
Several factors lead to retinal injury in shaken baby
syndrome. First of all, babies have large heads relative to
their bodies, and weak neck support. Shaking results in a
coup-contrecoup injury of the brain parenchyma, leading
to contusions and intracranial hemorrhages. Increased
intracranial pressure is translated down the optic nerve via
the meninges. Squeezing the thorax causes increased
venous pressure in the head. Both mechanisms increase
pressure on the optic nerve and cause retinal vascular
engorgement, leading to vitreous, retinal, and intraretinal
hemorrhages. If the baby survives, treatment of the eye is
mainly supportive, awaiting spontaneous reabsorption of
the blood.

Infection within the globe can result from ocular injury
(penetrating trauma or intraocular surgery) or
hematogenous spread (sepsis). The common culprits are
bacteria (staph, strep, Bacillus, and P.acnes) as well as
ubiquitous fungi (yeasts from indwelling catheters). The
vitreous is a nutritive jelly, much like agar, and bacterial
seeding of the vitreous can quickly turn the eye into a selfcontained abscess. Patients report loss of vision, pain,
and redness. Treatment consists of vitreous culture and
injection of broad spectrum intraocular antibiotics. Severe
loss of vision may necessitate surgical removal of the
vitreous abscess. Recovery is variable and depends on
the virulence of the organism.
Eyelid and Corneal Burns
Both thermal and chemical burns cause devastating injury
to the eyes. Early after injury, the eyelid skin can slough
and the tissue becomes edematous from fluid thirdspacing. As it heals, the skin contracts, and lid malpositioning causes corneal exposure and thinning.
Corneal and conjunctival defects also occur, and if limbal
stem cells are damaged, these evolve into non-healing
ulcers. Injury to the blood supply can cause ocular
ischemia. Of chemical burns, alkaline agents cause more
widespread damage due to deeper tissue penetration.
Patients with chemical burns must undergo irrigation until
the pH is neutralized. Aggressive lubrication and antibiotic
prophylaxis are standard treatment. With severe
inflammation, topical steroids can be used to minimize
long-term scarring and contraction.
Orbital Cellulitis
Infection of the orbit and periorbital tissues may extend
from adjacent sinusitis or be inoculated by skin trauma.
The pathogens most commonly implicated are staph,
strep, and H.flu. It is important to differentiate between
orbital and preseptal cellulitis; the former threatens the
orbital apex, the cavernous sinus, and the CNS; the latter
is limited to the skin. The orbital septum, a fibrous
membrane which extends from periosteum to the eyelids,
is the barrier which separates skin from deeper orbital
contents. Proptosis, limited motility, and decreased vision
are hallmarks of orbital involvement. Systemic antibiotics
are used to treat both, but orbital infection requires longterm therapy.
Orbital Fractures
Compressive forces during trauma can cause orbital blowout fractures. The bones which are thinnest and most
prone to fracture are the postero-medial floor (the
maxillary bone) and the medial wall (the lamina
papyrecea). Large fractures of the orbital floor will permit
the globe and orbital contents to sink inferiorly, resulting in
enophthalmos and diplopia. Small fractures may entrap
extraocular muscles, causing motility restriction, muscle
atrophy, and nausea/bradycardia (pulling on extraocular
muscles causes a vasovagal response). Fractures which
are functionally or cosmetically significant are repaired
using metal implants with antibiotic prophylaxis.

Orbital Compartment Syndrome

The orbit is a fixed space, and the movement of the globe
is limited posteriorly by the bony anatomy and anteriorly
by the canthal tendons (which position the eyelids against
the globe). Any mass (hemorrhage, abscess, tumor)
which occupies space within the orbit will displace the
globe forward, and a rapidly expanding mass will cause an
orbital compartment syndrome. As pressure builds in a
tense orbit, it can exceed the perfusion pressure of the
globe and optic nerve, causing vision loss. Intraocular
pressure also increases, causing acute glaucoma. A
lateral canthotomy and cantholysis (to cut the tendons
holding the globe back) will release the pressure and
restore vision.

Section 3: Primary Care Ophthalmology

Refractive Error
In healthy eyes, good distance vision depends on a
combination of factors: the power of the cornea and lens,
which are responsible for bending (refracting) light, must
be paired with a globe of the right length for an image to
be focused on the retina.
If the power of the cornea and lens are fixed, the image
will be focused to a discrete point. If the length of the eye
is such that the retina coincides with this focal point, a
sharp image will be perceived. This perfect system is
called emmetropia.
However, if the globe is too long and the focal point lies
anterior to the retina, the image will defocus and blur by
the time it falls on the retina. This is called myopia, or
near-sightedness. Myopic patients can see better up
close because as they bring the object of regard closer to
the eye, the focal point of the image moves posteriorly to
the retina until the image is clear. Luckily adding lenses in
front of the eye, in the form of glasses or contacts, can
correct for refractive error.
If the globe is too short and the focal point lies posterior to
the retina, the image will also be blurred when it reaches
the retina. This is called hyperopia, or far-sightedness.
Young hyperopic patients can move the focal point
forward by increasing the power of their native lenses (by
stimulating cholinergic input to the ciliary muscles) in a
process called accommodation.
Astigmatism occurs when the shapes of cornea and lens
are not perfectly spherical, that is, when the curvature is
greater in one axis compared to another. This causes
light to bend differently along different planes and there
can be two separate focal points. Just as before, when
the focal points do not both coincide with the retina, a
blurred image results.
The closer a patient brings the object to the eye, the more
posterior the image will be focused (see above under
myopia). An object which is too close has an image that
falls behind the retina. The process of accommodation
increases the power of the lens to decrease the focal
distance and pull the image forward until it is sharply
focused on the retina. However, the human lens
continues to grow with age and over time it becomes less
flexible. Past the age of 40, the process of
accommodation weakens significantly and patients have
difficulty seeing at near. This is called presbyopia. At this
stage patients need reading glasses, and if they also wear
a distance correction, they require bifocals.

Pink eye is the lay term for conjunctivitis, or inflammation
of the conjunctiva which is usually the transparent lining of
the globe. With inflammation, the conjunctiva becomes
edematous and the blood vessels become dilated. The
most common causes of conjunctivitis are viral, bacterial,
and allergic. The same viruses that cause the common
cold (adenovirus, enterovirus, etc) can also infect the eye,
causing teary/mucous discharge and irritation. Bacterial
infections are most often caused by strep, staph, and
H.flu, but may also be caused by gonococcus and
Chlamydia. The discharge is much thicker, more copious
and purulent. Patients with allergic disease complain of
itching and teary discharge. In all cases a history is
important for diagnosis. Bacterial infection is treated with
topical antibiotics (or systemic if gonococcal or
chlamydial). Allergic disease is treated with topical and
systemic antihistamines and mast cell stabilizers. There is
no treatment for viral conjunctivitis, but patients are given
contact precautions as it is highly contagious.
Inflammation of the eyelids leads to chronic irritation,
foreign body sensation, and dryness. There are
specialized oil-secreting glands at the eyelid margins
which can become congested with lipid and infected with
skin flora. Shed skin cells, oil, and bacteria can
accumulate at the base of the lashes. Treatment for
blepharitis includes warm compresses, eyelid scrubs, and
artificial tears. Severe cases associated with rosacea
require systemic tetracyclines.
A stye, or hordeolum, is a small abscess that develops
within an eyelid oil gland. If it persists, a localized
granuloma, or chalazion, develops. Medical treatment
consists of warm compresses and antibiotic ointment, to
try to elicit the pus to the surface and induce spontaneous
drainage. If the chalazion fails to open on its own, surgical
curettage is necessary. Chalazia can be prevented if the
underlying condition, usually blepharitis, is well-controlled,
and therefore daily compresses and eyelid hygiene are
Contact Dermatitis
An allergic dermatitis can develop from foreign soaps,
lotions, or makeup applied around the eye. The skin
becomes inflamed, with tissue edema, erythema, and
discomfort. The skin often has a corrugated appearance.
Differentiating from a preseptal cellulitis requires a careful
history, but often contact dermatitis extends over a wider
area (wherever the inciting substance was applied) and
the redness is more prominent. The offending agent
should be discontinued immediately. Often this alone is
enough. With more severe cases, a mild ophthalmic
steroid cream can be used to quiet the inflammation.

Cataracts can be congenital or acquired. Simply put, a
cataract is the clouding of the crystalline lens, which
results in scattering, or diffraction, rather than focusing, or
refraction, of light. Congenital cataracts are caused by
insults to the fetus in utero. For senile cataracts, lens
fibers continue to add to the diameter of the lens over
time, much like the trunk of a tree. In addition, as patients
age, lens proteins become less soluble and precipitate,
causing the lens to change color and opacify. Symptoms
caused by cataract include blurred vision, difficulty with
reading, and glare. Treatment is by surgical removal, by a
process called phacoemulsification (highly concentrated
ultrasound with vacuum) and, in adults, replacement with
an artificial intraocular lens.
Although the mechanism is not well understood, glaucoma
is a disease caused by elevated intraocular pressure or
vascular instability which causes damage to the optic
nerve. Acute glaucoma causes pain and rapid visual loss.
However, chronic glaucoma is often painless and patients
can lose a considerable number of optic nerve fibers, and
subsequently much peripheral vision, before they present.
The hallmark of glaucoma is a cupped nerve, where
nerve tissue is lost and consequently a large central crater
is formed (normally the central depression is less than 3040%). Visual field testing reveals loss of peripheral, and
sometimes central, vision. The goal of treatment is to
reduce the intraocular pressure, and this can be
accomplished with topical medications (aqueous
suppressants and outflow stimulants), laser treatment, and
filtering surgery.
Macular degeneration
Age-related macular degeneration (ARMD) is an idiopathic
disease that causes central visual loss in elderly patients.
In the dry form, a yellow substance called lipofuscin
deposits under the RPE in discrete lesions called drusen.
Drusen cause dysfunction and atrophy of the RPE. Since
the RPE is vital to the normal functioning of the retinal
photoreceptors and the blood-retinal barrier, visual loss
ensues. Dry ARMD is a slowly progressive disease with
no known cure. Recent studies have shown a diet rich in
anti-oxidant vitamins and smoking cessation can slow the
course of disease.
If there is enough damage to the RPE and the underlying
Bruchs membrane, choroidal vessels will break through
and gain access to the retina and subretinal space. This
choroidal neovascularization is comprised of abnormally
leaky capillaries which are fragile and prone to bleeding.
If this occurs, the disease transitions to the wet form of
macular degeneration. Macular hemorrhages can cause
instant and grave visual loss. Photodynamic therapy
(laser treatment combined with a drug which targets
abnormal choroidal vessels) can retard the progression of
disease but may not restore vision. Several new drugs
targeting stimulants to new vessel growth, namely VEGF,
are showing promising results.

Migraines are thought to originate from vasospasm of the
cerebral arteries. In addition to debilitating headache,
nausea, photophobia, and phonophobia, migraines can be
accompanied by visual or other sensory auras. It is
important to recognize that the symptoms arise from the
visual cortex in the occipital lobe and not from the eyes.
Visual auras usually begin as scintillating scotomas,
shimmering curtains or colored geometric patterns. As the
migraine progresses, the positive visual phenomena can
expand to cover the visual field. In some patients,
negative phenomena can cause a hemianopia (or
blackout of half the visual field)! Treatment is aimed at
recognition of symptoms and serotonin agonists in
conjunction with neurological consultation.

In normal alignment of the eyes, the corneal light reflex is
centered in both pupils. Good alignment is necessary for
normal development of visual acuity and stereopsis, or
depth perception. Any misalignment results in competition
between the fovea of the two eyes (since the brain cannot
reconcile two different images both supposedly at the
center of vision). This causes one eye to develop a
central scotoma, or blind spot. Thus strabismus during
childhood results in amblyopia, or poor visual
development of one eye. Strabismus in adulthood (due to
trauma, thyroid disease, or sensory deprivation) can
cause diplopia and cosmetic concerns. In children,
patching for amblyopia (to strengthen the weaker eye)
should accompany surgical realignment. Adults who do
not opt for surgery can try prism glasses and patching.

Section 4: Ophthalmology Associated with Systemic Disease

Thyroid Ophthalmopathy
Autoimmune thyroid disease is a systemic disorder that
spans the spectrum from hyper- to hypo-thyroid. Orbital
disease does not necessarily parallel the exocrine
abnormalities; in fact, orbital disease can precede
hormonal changes by months, or lag behind by years!
The cardinal features of thyroid ophthalmopathy include:
exophthalmos, eyelid retraction (which causes corneal
exposure and injury) and abnormally thickened
extraocular muscles (which cause strabismus, limited
motility, and diplopia). Rarely visual loss can occur due to
optic nerve compression by hypertrophied extraocular
muscles. Primary treatment is to stabilize the exocrine
abnormality. Treatment of eye disease includes
lubrication, orbital decompression (inducing orbital
fractures to reduce the proptosis), and strabismus and
eyelid surgery.
Stevens-Johnson Syndrome
Mucosal sloughing and scar formation are the hallmarks of
Stevens-Johnson Syndrome, which can be caused by
reaction to systemic medications or viral infections. The
initial phase of injury is much like a burn: tissue necrosis
and third-spacing of fluid. Later stages are cicatricial
(meaning scar-forming) and aberrant bands of
conjunctiva, or synechiae, and corneal neovascularization
can cause permanent visual loss. Treatment is supportive
with aggressive lubrication and antibiotic prophylaxis.
Topical steroids may quiet the inflammation and minimize
long-term scarring. Corneal transplant or keratoprosthesis
(artificial cornea) are options to restore vision.
Herpes Simplex
Over ninety percent of Americans are sero-positive for
herpes simplex virus (HSV). After initial infection, the
virus stays dormant in the sensory ganglia and can
reactivate and travel down the sensory nerves to erupt as
a vesicular rash. The virus can infect the eye directly from
branches of the trigeminal nerve, or by contact from
adjacent infected areas. In addition to a vesicular eyelid
dermatitis, HSV can cause a viral conjunctivitis, corneal
ulcers, uveitis (intraocular inflammation), and retinal
necrosis. HSV corneal ulcers have a characteristic
branching appearance like the dendrites of a neuron.
When deeper corneal tissues are affected, dense scarring
and neovascularization can develop, causing loss of
Treatment of HSV ocular infection is by antiviral antibiotics
(acyclovir) and topical steroids for immunosuppression.
The inflammatory response to virus often causes more
damage than the infection alone. Patients with recurrent
outbreaks are given low dose acyclovir for long-term
prophylaxis. Visual loss due to corneal scarring can be
treated with corneal transplant.

Herpes Zoster
After chickenpox infection, the herpes zoster virus (HZV)
also remains dormant in the sensory ganglia.
Reactivation of HZV manifests as shingles, a painful
vesicular rash which, unlike HSV, strictly respects the
dermatomes. Virus latent in the trigeminal ganglion can
erupt in the V1 or V2 dermatomes, and can infect the eye.
Like HSV, HZV can cause conjunctivitis, corneal ulcers,
uveitis, and retinal necrosis. When the eye is involved,
topical and oral acyclovir limit viral replication while topical
steroids are used to treat the inflammation. Though HZV
does not recur in the eye, corneal scarring can cause
permanent visual loss. If this occurs, corneal transplant is
an option. Post-herpetic neuralgia is a chronic and
debilitating condition that is induced by sensory nerve
damage during the initial shingles outbreak, and can be
treated with neurontin or tricyclic antidepressants.
Inflammation within the eye has many etiologies. Any
cause of systemic inflammation (autoimmune disease,
infection, neoplasm) can also cause ocular disease.
Uveitis is characterized by pain, visual loss, glaucoma,
tissue necrosis, and scarring. Patients should undergo a
diagnostic work-up to evaluate for autoimmune and
infectious etiologies.
Uveitis can affect many different ocular tissues. Scleritis
is focal inflammation of the ocular wall, causing redness
and tenderness to touch. Iritis/iridocyclitis is inflammation
within the anterior chamber (white blood cells leaking from
the iris and ciliary body), and is commonly seen in juvenile
rheumatoid arthritis and HLA-B27 related diseases
(ankylosing spondylitis, Reiters, etc). Patients can
present with loss of vision, photophobia, and acute
glaucoma. With severe iritis, white blood cells can layer
into a precipitate called a hypopyon.
Posteriorly, retinitis, retinal vasculitis, and choroiditis can
occur in patients with systemic lupus erythematosus,
sarcoid, and various infectious organisms such as
tuberculosis, syphilis, and cytomegalovirus. Scarring and
necrosis here not only lead to visual loss but also to
secondary complications such as retinal detachment.
Optic neuritis, or inflammation along the optic nerve, is
often associated with multiple sclerosis. When the orbit,
extraocular muscles, and lacrimal gland are involved, the
term inflammatory orbital pseudotumor is used (to be
distinguished from pseudotumor cerebri).
Treatment of uveitis can be difficult with topical steroids
alone if the patient has concurrent systemic disease.
Though topical treatment is the first line of therapy for
intraocular disease, addition of systemic
immunosuppressives such as methotrexate, Cytoxan,
Imuran, and Embrel are often needed to keep the
inflammation under control. Patients are frequently comanaged with rheumatologists.

Ocular Side Effects from Systemic Medications

Numerous systemic medications can induce ocular
changes and toxicity. For example, tetracyclines and oral
contraceptives can cause idiopathic intracranial
hypertension (pseudotumor cerebri) which can cause
papilledema and optic neuropathy. Plaquenil can induce
an irreversible maculopathy; amiodarone can leave
vortex-shaped corneal opacities. Topamax can cause a
uveal effusion syndrome leading to acute angle closure
glaucoma. Rifabutin can cause severe uveitis and a
hypopyon. Cessation of the inciting drug can reverse the
ocular injury in many, but not all, cases. In the photo,
systemic steroid use and radiation to the head and neck
can both induce cataract formation, but these cataracts
require surgery to restore vision.
Diabetic Retinopathy
Diabetes induces systemic microvascular ischemia, and
the end-organs most severely involved are the eyes,
kidneys, and peripheral nerves. Both juvenile- and adultonset diabetics get ocular complications; the longer a
patient has had diabetes, the greater the chances of
vision-threatening retinopathy.
Poor glucose control leads to cytostructural changes in the
retinal vasculature. In the non-proliferative form of
retinopathy, small vessel ischemia and infarcts lead to
hemorrhages, cotton-wool spots (infarcts of the nerve fiber
layer), and microaneurysm formation. These abnormal
blood vessels also leak fluid, causing distortion of the
retinal architecture and vision loss. More severe diabetes
and chronic ischemia induce the proliferative form of
retinopathy, with the development of retinal, optic disc,
and anterior segment neovascularization. Abnormal
retinal vessels extend up into the vitreous cavity and can
cause large hemorrhages as well as tractional retinal
detachments. Abnormal iris and angle vessels cause
neovascular angle-closure glaucoma.
Patients with both diabetes and hypertension get
accelerated disease. Primary treatment is with strict
glucose and blood pressure control. Once patients
present with visual loss or neovascularization, laser
photocoagulation of the retina can treat or slow the
disease. Vitrectomy and retinal detachment repair are
surgical options. New drugs targeted at the stimulants for
neovascularization, namely VEGF, are currently being
introduced and are showing promising results.
Hypertensive Retinopathy
Hypertension also causes systemic ischemia and ocular
complications. Signs of chronic hypertension include
attenuated and tortuous retinal vessels, hemorrhages, and
cotton-wool spots. Retinal macroaneurysms can develop,
which then leak fluid or rupture and bleed, causing acute
vision loss. Severe malignant hypertension can cause
papilledema and retinal and choroidal ischemia, which can
occur in other acute hypertensive states such as preeclampsia. Treatment is with acute and long-term blood
pressure control.

Retinal Artery Occlusions

Acute painless visual loss is usually an indication of a
vascular catastrophe. Occlusion of the retinal arteries,
whether by embolus, atherosclerotic disease, or vascultis,
is analogous to a stroke in the eye. Like the brain there
is loss of function and tissue edema. The recovery of
vision is variable, but due to the intense metabolic
demands of the retina, more often than not the damage is
irreparable. Occlusion of the central retinal artery causes
severe and widespread visual loss. Involvement of a
branch retinal artery can cause central visual loss if it is
located in the macula (central retina) or a scotoma (blind
spot) if located more peripherally. Emboli that can be
visualized within the vessel lumen are called Hollenhorst
plaques. Patients with such plaques must undergo
embolic work-ups including carotid dopplers and cardiac
echograms. Treatment is by modification of risk factors.
Giant Cell Arteritis
One cause of retinal artery occlusion in elderly patients is
giant cell, or temporal, arteritis (GCA). GCA is a systemic
vasculitis that can lead to blindness, stroke, and
myocardial infarction. Symptoms worrisome for GCA
include sudden visual loss, temporal headache, scalp
tenderness, pain with chewing, proximal limb myalgias,
loss of appetite, and weight loss. Elevated sedimentation
rate and C-reactive protein help make the diagnosis.
Patients are treated with high dose systemic steroids with
a slow taper. Temporal artery biopsy, performed by the
ophthalmologist, provides the histological diagnosis for
Retinal Vein Occlusion
Another vascular event marked by acute painless visual
loss is the occlusion of a retinal vein. On fundus exam,
large flame-shaped hemorrhages and tortuous vessels are
pathognomonic. Similar to arterial occlusions, ischemia
and tissue edema set in. However, the causes of vein
occlusions are quite different. Diabetes, hypertension,
and hypercoagulable states (such as polycythemia vera,
anticardiolipin antibodies, pregnancy, etc) are the most
common etiologies. In addition, vein occlusions may be
caused by intrinsic ocular factors, such as glaucoma and
venous compression by overlying arteries (in the setting of
hypertension). Widespread ischemia, as with a central
vein occlusion, can cause retinal and anterior segment
neovascularization, which is treated with laser. Otherwise,
treatment is by modification of risk factors.
Sickle Cell Retinopathy
In low oxygen environments, the erythrocytes of patients
with sickle cell change morphology (sickle) and cause
systemic vascular occlusions. In the retina, these infarcts
cause bleeding and retinal neovascularization (in
response to the ischemia) in a characteristic pattern called
the sea fan. Like other proliferative retinopathies, sea
fans can cause vitreous hemorrhages and tractional
retinal detachments. Treatment is by laser
photocoagulation and by stabilizing the patients overall

Retinopathy of Prematurity
Premature babies have underdeveloped lungs at birth and
are given supplemental oxygen therapy. However, their
retinas are also immature, and excess oxygen causes the
retinal vascular pattern to change. Neovascularization
occurs, with scaffolding of fibrovascular sheets into the
vitreous. Left untreated, these fibrovascular membranes
contract and cause retinal detachment. Premature babies
under 2000 grams must be monitored for the development
of retinopathy of prematurity (ROP). Once ROP begins,
treatment with laser or cryo (freezing) to the peripheral
retina is performed to induce regression of abnormal
vessels. These children must be monitored long-term for
the development of high myopia and strabismus that
accompany ROP.

The most common malignant intraocular tumor of
childhood is retinoblastoma. The retinoblastoma gene
normally functions as a tumor suppressor. When one
allele is knocked out, either by a germline or somatic
mutation, damage to the second allele will result in the
formation of a white calcific tumor. Both eyes can be
affected, as can the pineal gland, which also contains
photoreceptor elements.
Affected children most commonly present with leukocoria
(white pupil) and strabismus. Small tumors confined to
the retina can be treated with laser and cryo. Large
tumors which threaten to metastasize can be treated with
chemo- and radio-therapy, or by enucleation (removal of
the eye). Siblings and parents should also be examined.
Unfortunately, patients with retinoblastoma often develop
other malignancies such as osteogenic sarcomas,
especially in the areas previously treated with radiation.

The strict definition of papilledema is elevated intracranial
pressure causing bilateral optic nerve edema. The causes
of papilledema include hydrocephalus, infection
(meningitis, encephalitis), space-occupying mass, and
idiopathic intracranial hypertension (pseudotumor cerebri).
Though visual acuity may not be affected initially, over
time papilledema can cause death of optic nerve fibers
and optic neuropathy and vision loss will result.
Treatment focuses on the inciting etiology. In cases of
idiopathic intracranial hypertension, intractable
papilledema can be treated by optic nerve fenestration, a
surgical procedure to create a window in the meninges
that line the optic nerve.

Optic Neuritis
Optic neuritis is the inflammation of the optic nerve. It can
be caused by many autoimmune, toxic, and infectious
processes, but it is commonly associated with multiple
sclerosis (MS). MS is an autoimmune disorder that
targets myelin, and is characterized by waxing and waning
neurological deficits and white matter lesions on MRI.
Optic neuritis presents with vision loss and pain with eye
movements. Often optic disc edema can be seen in the
affected eye. The natural course of disease is
spontaneous improvement, however patients with severe
vision loss can be treated with high-dose intravenous
steroids to accelerate recovery.
Cerebral infarct along the visual pathways will cause
various patterns of visual loss, depending on the location
of injury. All lesions posterior to the optic chiasm will
cause bilateral visual field defects; the more posterior the
lesion, the more congruous the field defects will appear.
To determine the location of the stroke, the rule is as
follows: defects correspond to lesions in the opposite
hemisphere, and superior defects point to inferior lesions
(and vice versa). Optic tract lesions (C) can produce
homonymous hemianopias. Since fibers which serve the
superior visual field travel inferiorly to the temporal lobes
(D), lesions here create superior quadrantanopias or
segmental defects. Thus parietal lobe lesions produce
inferior defects. Occipital lobe lesions (E) can involve or
spare the center of vision, depending on the area
involved. Though little can be done to recover loss field,
visual rehabilitation and training can help patients cope
with their limited peripheral vision.
16. American Academy of Ophthalmology, Basic and Clinical Science Course 2001, Section 12 Retina.

Section 5: Ophthalmic Surgeries

Cataract Surgery
Most cataracts in the United States are removed with
small incision, sutureless surgery. Patients are kept
awake and receive local anesthesia to the eye, in the form
of topical lidocaine jelly or an orbital injection. Two small
wounds are created in the peripheral cornea. After the
anterior lens capsule is removed, the cataract itself is
emulsified and vacuumed out using an ultrasonic probe
approximately the size of a large pen. Once the cataract
is removed, an artificial lens is folded and injected into the
same lens capsular bag. The artificial lens is specially
designed with a central optic (the actual lens) and two
haptics (arms) which unfold and hold the lens in place.
Post-operatively the eye is treated with topical antibiotics
and steroids and protected while the sutureless wounds
heal. Most patients have significant visual improvement
by the first post-operative day.

Glaucoma Surgery
Filtration surgery is performed when medications have
failed to reduce the intraocular pressure to a level that is
safe for the optic nerve. The goal of glaucoma surgery is
to create an alternative path by which intraocular fluid can
drain from the eye and be reabsorbed by the venous
circulation. The traditional trabeculectomy involves
creating a partial thickness flap in the sclera, which covers
a hole connecting the anterior chamber to the outside
world. The flap helps keep the efflux of fluid partially
controlled, so that hypotony does not develop. Aqueous
leaves the eye through the hole (sclerotomy), travels
under the flap and under the conjunctiva, to be
reabsorbed posteriorly by the ophthalmic veins. When the
scleral tissue is not healthy, artificial valves can be
implanted to act as a conduit for aqueous removal.
Strabismus Surgery
Realignment of the eyes can be done by mechanically
repositioning the extraocular muscles. Weak muscles are
shortened or moved forward to increase their actions.
Overactive muscles are shifted more posteriorly to cause
relaxation and weakening. Strabismus surgery is usually
performed on two muscles at a time, either corresponding
muscles on both eyes (both strengthened or weakened) or
antagonist muscles on the same eye (one strengthened
while the other weakened). In adult patients with complex
strabismus (such as thyroid disease), temporary knots can
be tied in the operating room, to be more finely adjusted in
the recovery room, with the patient awake and able to

Vitrectomy and Retina Surgery

Access to the posterior segment of the globe is by
endoscopic surgery. A magnifying lens is placed on top of
the cornea to provide view of the vitreous and retina.
Small ports in the sclera are created, through which fine
instruments and a light source are inserted. The vitreous
is a thick tenacious jelly which must be cut and evacuated
before the retina can be approached. Once the retina is
repaired, the vitreous cavity can be filled with a variety of
different liquids and gases to help position the retina for
healing. Surgery to the optic disc can also be performed
via this endoscopic approach, though such procedures are
still mostly experimental.

Chronic naso-lacrimal duct obstruction leads to excess
tearing and the potential for recurrent infection. To bypass
the blockage, a small opening in the nasal bone is made
adjacent to the lacrimal sac, and the mucosa of the sac
and the nasal cavity are joined. Then, silicone tubes are
passed through the lacrimal canaliculi, into the new
opening, and then retrieved from the nose and tied.
These tubes stay in place for several weeks while the new
passageway heals, to help maintain patency of the
openings, before they are removed.

Temporal Artery Biopsy

To make the diagnosis of giant cell arteritis and justify
long-term steroid treatment, ophthalmologists are often
asked to perform temporal artery biopsies. The temporal
artery is identified either by palpation or by Doppler
ultrasonography. The skin and subcutaneous fascia are
dissected until the artery is identified. The artery is then
carefully isolated and ligated before excising a specimen
for pathology. Histologically, hallmarks for vasculitis
include granulomatous inflammation, obliteration of the
vessel lumen, and disruption of the elastic lamina.

Laser Surgery
There are numerous applications for laser
photocoagulation in ophthalmology. In refractive surgery,
laser energy is used to remodel the cornea and to change
its refractive power, to give patients clear vision without
the use of spectacles or contact lenses. In the anterior
segment, laser is used to perforate the iris (in acute angle
closure glaucoma), to remodel the drainage structures of
the eye (in chronic glaucoma), to open a clear window in
lens capsule opacities (behind an artificial lens), and to cut
buried sutures. In the posterior segment, laser is used to
treat proliferative retinopathies, including diabetic disease,
sickle cell disease, and ROP. By coagulating selected
sections of retina, there is decreased retinal metabolic
demand, and decreased production of the chemical
stimulants to neovascularization (such as VEGF). A new
form of laser is now the standard of care in macular
degeneration. Photodynamic therapy is the combination
of an intravenous drug which specifically targets abnormal
new vessels, followed by selective activation of the drug in
the eye through laser light. In orbital surgery, laser is also
used during dacryocystorhinostomy and in eyelid skin


Section 6: Equipment and Diagnostic Studies Used in Ophthalmology

The Slit Lamp
The translucency of ocular tissues makes the eye an ideal
organ to examine by visualization. The slit lamp
microscope is specially designed to allow for a magnified
and stereoscopic view. Anterior segment structures (the
eyelids, cornea, conjunctiva, iris, anterior chamber, and
lens) can be seen directly. Combining the slit lamp with a
hand-held lens allows visualization of the posterior
segment (the optic nerve, posterior vitreous, and central
retina. Contact lenses can be applied to examine the
angle and the peripheral retina. An applanation tonometer
attached to the slit lamp is used to check intraocular
pressure. Procedures such as corneal foreign body
removal and suture placement can be performed with the
patient positioned in the slit lamp. Hospitalized patients
are often supine and unable to sit at the slit lamp. In these
situations, a penlight or portable slit lamp exam can
suffice, but the exam is significantly less detailed.
The Direct and Pan-Optic Ophthalmoscopes
Most ophthalmologists prefer to examine patients with the
pupils pharmacologically dilated. However, when the pupil
is small, a direct or pan-optic ophthalmoscope can be
used to view the optic nerve and retina. Even with the
pupils dilated, the view of the optic disc is greatly
magnified with the direct scope, so this is preferred by
many neuro-ophthalmologists. These instruments are
small, battery-charged, and portable, making them ideal
for use at the bedside.
The pan-optic is a recent innovation on the direct
ophthalmoscope to give a wider field of view. However
both scopes are used in the same way. With the scope
positioned in front of the patients pupil, the physician dials
in power until the retinal vessels are in focus. Then with
small adjustments to the viewing angle, the vessels can
be traced proximally to the optic nerve or more distally to
examine the peripheral retina. Hemorrhages and retinal
emboli can be visualized in this way. The major
disadvantages to the direct scope are the necessity for
close proximity to the patient and the small field of view.
The Indirect Ophthalmoscope
For a stereoscopic view of the retina, the indirect
ophthalmoscope can be used. A hand-held lens is
positioned a few centimeters above the cornea, and
through a dilated pupil, a three-dimensional, wide-field
image of the retina can be seen. With additional
techniques such as scleral depression (indenting the wall
of the eye towards the vitreous), the far retinal periphery
can be examined. Laser photocoagulation can be
combined with indirect ophthalmoscopy to treat peripheral
lesions not accessible to traditional slit lamp lasers.

The Goldmann Applanation Tonometer

The gold standard for measuring intraocular pressure is
the Goldmann tonometer. It was designed to balance the
force exerted outward by the cornea (the wall of the ocular
water balloon) and the force inward by the applanation
tip mounted on an adjustable pressure gauge. A yellow
dye called fluorescein is applied to the cornea and, with
the cobalt blue light from the slit lamp, the cornea makes a
green meniscus against the applanation tip. The size of
the meniscus varies depending on the force applied by the
tip, and the gauge is adjusted until the two forces are
equalized. The reading at the point of equalization is the
intraocular pressure.
The Tono-Pen
The Tono-Pen is another contact method by which the
intraocular pressure is measured. It is portable for use in
supine patients, and will still function even for eyes with
corneal surface diseases where the Goldmann applanator
cannot be used. It is also a better choice for children,
since it requires less cooperation. A rubber sleeve is
placed over the metal tip for protection. Once the pen is
calibrated, the tip is gently tapped against the central
cornea until the pressure is displayed digitally on the
The Goldmann Perimeter
Visual fields can provide important diagnostic information
for patients with glaucoma and neuro-ophthalmic
disorders. The Goldmann Perimeter is a manual,
operator-driven visual field machine. The patient is
instructed to look at a central fixation point and lights of
different sizes and intensities are presented to his
peripheral vision. The targets are first positioned in nonseeing areas and are moved toward central fixation.
When the patient first notices the target, he sounds the
buzzer, and that point is marked on the field map by the
operator. Point by point the visual field is plotted out. By
connecting the points for a set size/intensity of light, a
contour map of visual field is made. Targets which are
larger and brighter are better visualized in the periphery.
As the size and intensity of the target decrease, the field
constricts. Glaucomatous defects and hemianopias are
easy to demonstrate, as shown by the example.

The Humphrey Visual Field Analyzer

The Humphrey is a second visual field machine which is
automatic and operator-independent. The patient
interacts with a computer, and based on complex
algorithms, he is tested for selected patterns of visual field
loss. Like the Goldmann, the targets vary in intensity, but
unlike the Goldmann, they are all the same size. The
bowl of the Humphrey has a fixed array of targets, which
activate in a seemingly random sequence. Once a defect
is detected, the Humphrey is programmed to carefully test
the points surrounding the defect to elicit any possible
patterns consistent with optic nerve or CNS disease. In
addition, the Humphrey is better able to test the center of
fixation and has special programs to detect central
scotomas (blind spots). Unlike the contour map generated
with the Goldmann perimeter, the printout from the
Humphrey shows field loss as decreased sensitivity on a
numerical scale which is then translated graphically into

The Fluorescein Angiogram

Retinal disease is often vascular in etiology. Diabetic
retinopathy with macular edema and neovascularization,
macular degeneration with choroidal neovascular
membranes, and central retinal artery occlusions are
prime examples. In fluorescein angiography, fluorescein
dye is injected intravenously and circulates to the retina
where it gets activated by blue light mounted on a camera.
Photos are taken as retinal and choroidal vessels fill.
Thus retinal perfusion can be traced chronologically, and
any abnormal vessels prone to leakage can be identified.
In addition, emission of fluorescent light from the dye can
identify retinal lesions that cannot be visualized directly
due to blood or other media opacity. Even after most of
the fluorescein has left the retinal circulation, the staining
pattern which remains can also be diagnostic for many
retinal diseases.