OPHTHALMOLOGY

BOOKLET
for
USMLE
Amaurosis fugax
Amaurosis fugax is characterized by visual loss that us usually monocular and transient. It is
sometimes described as ‘like a curtain falling down.’ Ophthalmoscopy reveals zones of
whitened, edematous retina following the distribution of the retinal arterioles. The condition is
usually seen in patients with atherosclerosis, cardiovascular disease or hypertension. It is
caused by retinal emboli from the ipsilateral carotid artery. Rarely, cholesterol emboli or
plaques can be visualized (Hollenhurst plaque). Non-invasive evaluation of the carotids is useful
in providing information regarding the degree of stenosis. Treatment of the atherosclerosis is
important to reduce the risk of stroke.

Central retinal artery occlusion (CRAO)

It is characterized by a sudden painless loss of vision in one eye. Fundus exam reveals pallor of
the optic disc, cherry red fovea and boxcar segmentation of blood in both retinal arteries and
veins. Risk factors include carotid artery disease, endocarditis, cardiac valvular disease, long
bone fracture, hypercoagulable conditions, vasculitis, atrial myxoma, etc. It occurs when a
severe, abrupt diminution of blood flow through the central retinal artery causes ischemia to
the inner retina. Embolism of the retinal artery is the most common cause of ocular stroke.
Emboli may travel to the distal branches of the retinal artery, causing loss of only a section of
the visual field. It is commonly associated with amaurosis fugax before the occlusion. Visual
acuity is typically 20/800 (6/240) or worse.

Remember that the ophthalmic artery is the first intracranial branch of the internal carotid
artery. It supplies blood to the eye via the:
1) central retinal artery, which is an end artery that supplies the inner retina
2) ciliary branches, which supply the choroids and the anterior portion of the globe

CRAO is an ophthalmic emergency. A delay in treatment may result in permanent loss of vision.
Immediate intervention includes ocular massage, which dislodges the embolus to a point
further down the arterial circulation and improves retinal perfusion. Medical management and
anterior chamber paracentesis to lower the intraocular pressure may be used, but ocular
massage has the most rapid action. Carbogen therapy (5% CO2 and 95% O2) or hyperbaric
oxygen (HBO) therapy have been shown to be beneficial if given early.

Note: Thrombolytics may be useful if initiated within 4-6 hours of visual loss but they are
administered intraarterially. Their efficacy has not been systematically studied.
Central retinal vein occlusion (CRVO)
It presents with sudden, painless, unilateral loss of vision – though typically not as sudden as
vision loss seen in patients with CRAO. CRVO is associated with coagulopathy, hyperviscosity,
chronic glaucoma, and atherosclerotic risk factors (age, diabetes, and hypertension). Fundus
exam is described as “blood and thunder” and shows optic disc swelling, venous dilatation,
tortuosity, retinal hemorrhages and cotton wool spots. No treatment is particularly effective,
but some patients have partial recovery of vision within the first three months.

Vitreous hemorrhage
It presents with a sudden loss of vision and floaters in the visual field. Diabetic retinopathy is
the most common cause. An important clue to the diagnosis is a fundus that is hard to visualize
with obscure details. For patients with underlying medical conditions, conservative
management (i.e. upright position during sleep, which enhances the settling of the
hemorrhage) is recommended.

Hypertensive retinopathy
Patients with hypertensive retinopathy usually do not show any symptoms associated with
visual impairment. There is initially focal spasm of arterioles, followed by progressive sclerosis
and narrowing. Fundoscopy findings may reveal AV nicking, copper wiring or silver wiring,
exudates and hemorrhages (specific finding depends on the grade of retinopathy).

Glaucoma

Open Angle glaucoma is usually asymptomatic in the earlier stages. It is more common in
African Americans, and has an increased prevalence in those with a family history of glaucoma
and diabetes. There is a gradual loss of peripheral vision over a period of years, and eventual
tunnel vision. On examination, the intraocular pressure is high. There may be cupping of the
optic disc with loss of peripheral vision. Annual examination in high-risk populations is of great
benefit in prevention of the condition. Beta-blockers such as timolol eye drops are effective in
the initial management of the patient. Laser trabeculoplasty is used as an adjunctive measure.
If there is continuous increase in intraocular pressure, surgical trabeculectomy is done.

Angle closure glaucoma is characterized by a sudden onset of symptoms such as blurred bision,
sever eye pain, nausea and vomiting. Examination reveals a red eye with a hazy/steamy cornea
and a fixed, dilated pupil that is not responsive to light. It occurs with the closure of a pre-
existing narrow anterior chamber angle. It predominately occurs in people aged 55-70 years. It
usually occurs following papillary dilation, which may occur in darkened movie theaters, during
times of stress or due to drug intake. The anterior chamber is shallow with inflammatory
changes. Tonometry reveals increased IOP. Intravenous acetazolamide (with subsequent oral
administration) may lower the IOP. Increased IOP can also be reduced with mannitol,
acetazolamide, timolol or pilocarpine. Narcotics are used to control pain. Atropine should be
avoided since it can dilate the pupil and worsen the glaucoma. Permanent cure is offered with
laser peripheral iridotomy.

Diabetic retinopathy
It is the leading cause of acquired blindness among US adults. It occurs in both insulin
dependent and non-insulin dependent diabetes mellitus. It can be classified into:
1. Non-proliferative retinopathy – characterized by dilation of veins, microaneurysms, retinal
hemorrhages, edema, and hard exudates.
2. Pre-proliferative retinopathy – with cotton wool spots
3. Proliferative retinopathy is characterized by neovascularization.
Most patients with diabetic retinopathy are asymptomatic until very late in the disease, at
which point they may present with poor night vision, a curtain falling with a vitreous bleed or
floaters during the resolution of the vitreous bleeds. Visual impairment occurs with the
development of macular edema. Argon laser photocoagulation is the suggested treatment for
prevention of complications.

Cataract
It is a vision impairing disease characterized by progressive thickening of the lens. Oxidative
change of the lens occurs with aging and leads to cataract formation. Patients usually complain
of blurred vision, problems with nighttime driving and glare. Definitive treatment is lens
extraction. The three most commonly used procedures are phacoemulsification, extracapsular
cataract extraction (ECCE) and intracapsular cataract extraction (ICCE).

Herpes zoster ophthalmicus

It is an infection caused by VZV. Most episodes occur in the elderly or in the immune-
suppressed. VZV remains latent in the trigeminal ganglion. During periods of
immunosuppression, the virus travels via the ophthalmic branch to the forehead and the eye.
Symptoms become manifest thereafter with fever, malaise and burning, itching sensation in the
periorbital region. Examination reveals a vesicular rash in the distribution of the cutaneous
branch of the first division of the trigeminal nerve. Conjunctivitis and dendriform corneal ulcers
characterize the eye movement. Treatment started within 72 hours after eruption with high
dose acyclovir reduces the development of complications.
Herpes simplex keratitis
This presents with pain, photophobia and decreased vision. Dendritic ulcer is the most common
presentation. There may also be minute clear vesicles in the corneal epithelium. The typical
vascular rash of herpes zoster and systemic symptoms are not seen. It is the most frequent
cause of corneal blindness in the US. It mostly occurs in adults. A history of previous episodes
may be present. Usually, recurrences are precipitated by excessive sun exposure, outdoor
occupation, fever or immunodeficiency. It is diagnosed clinically, although epithelial scrapings
will show multi-nucleated giant cells. Antiviral therapy (oral or topical) is effective in the
treatment of Herpes simplex keratitis.

HSV Retinitis
Note: Retinitis can occur as a complication of opportunistic infections in AIDS patients. Both
VZV and HSV can cause severe, devastating intraocular inflammation. Most cases of retinal
disease are believed to be a reactivation of a previously acquired infection.

In an immunocompromised individual, HSV retinitis may be characterized by a rapidly

progressing bilateral necrotizing retinitis (referred to as “acute retinal necrosis syndrome”). The
initial symptoms are keratitis and conjunctivitis with eye pain, followed by rapidly progressive
visual loss. Fundoscopy reveals widespread, pale, peripheral lesions and central necrosis of the
retina.

Fungal keratitis
It occurs after corneal injury in agricultural workers or immunocompromised patients. The
cornea shows multiple stromal abscesses.

Dacryocystitis
It is an infection of the lacrimal sac due to obstruction of the nasolacrimal duct. It usually occurs
in infants and adults over the age of 40. It is characterized by pain, swelling, tenderness and
redness in the tear sac area/medial canthal region. Sometimes a purulent discharge is noted
from the punctum. A few patients present with fever, prostration and an elevated leukocyte
count. Staphylococcus aureus and beta-hemolytic Streptococcus are the usual infecting
organisms. It usually responds to systemic antibiotic therapy.

Note: it can occur in infants days to weeks after birth.

Bacterial keratitis
It is usually seen in contact lens wearers, and following corneal trauma. The cornea appears
hazy with a central ulcer and adjacent stromal abscess. Hypopyon may be present.

Trigeminal Neuralgia
The diagnosis is made clinically – it is characterized by a stabbing or shock-like pain over the
distribution of the maxillary or mandibular division of the trigeminal nerve. The pain may be
triggered by light touch or vibration. Rash is not seen.

ARMD
It is the leading cause of blindness in industrialized countries. Macular degeneration affects the
central vision. It is characterized by distorted vision and central scotoma. Peripheral fields and
navigational vision are always maintained, but may become impaired by the development of
cataracts. The condition results from degeneration and atrophy of the outer retina, retinal
pigment epithelium, Bruch’s membrane and choriocapillaries.
One of the earliest findings in macular degeneration is distortion of straight lines such that they
appear wavy (visualization of straight lines is a task that requires fine visual acuity, which is
controlled primarily by the centrally located macula as opposed to the more peripheral retina).
The Amsler grid test is used to screen for patients with macular degeneration. The primary risk
factor for macular degeneration is age, although cigarette smoking can increase the risk.
Ophthalmoscopy findings vary according to the type (atrophic vs. exudative). The atrophic form
is characterized by multiple sores in the macular region, while the exudative form is
characterized by new blood vessels that may leak, bleed and scar the retina.

Retinal Detachment
It refers to the separation of the layers of the retina. It usually occurs in people aged 40-70
years. Patients complain of photopsia (flashes of light) and floaters (spots in the visual field).
The most classic description is that of a curtain coming down over the eye. Usually, the inciting
event occurs months before the RD. Myopia or trauma can cause retinal breaks, through which
fluid seeps in and separates the retinal layers. RD occurs unilaterally. It presents as blurred
vision that progressively worsens. Ophthalmoscopy reveals the retina hanging in the
vitreous/gray, elevated retina. Laser therapy and cryotherapy are done to create permanent
adhesions between the neurosensory retina, retina pigment epithelium and choroid.

Optic Neuritis
Optic neuritis typically presents in people aged 20-45 years. Females are more commonly
affected. Patients experience rapid impairment of vision in one eye (or rarely both) and pain on
eye movement. There are marked changes in color perception. Afferent papillary defect and
field loss occur, usually with central scotoma. Funduscopy may reveal a swollen optic disc. Optic
neuritis is common in patients with multiple sclerosis; such patients will usually give a history of
similar occurrences in the past.

Note: always suspect MS in a female with multiple neurologic presentations that are
interspaced between time periods. Optic neuritis can result in blurring of vision, and is often
associated with retrobulbar pain.

Orbital cellulitis
It refers to infection posterior to the orbital septum. These infections are unilateral and more
common in children. It presents with an abrupt onset of fever, proptosis, restriction of
extraocular movements and swollen, red eyelids.

Anterior Uveitis
The eye is usually painful and red, with blurring of vision. Typically, the eye has perilimbal
injection. Examination reveals keratitic precipitates (“Mutton fat”) and iris nodules. Corneal
stromal edema may be present. The anterior chamber shows flare and cells on slit lamp
examination. The pupil is constricted with poor light response. It is associated with HLA B27-
related conditions.

Episcleritis
It is an inflammation of the episcleral tissue between the conjunctiva and sclera. There is an
acute onset of mild to moderate discomfort, photophobia and watery discharge. Examination
reveals diffuse or localized bulbar conjunctival injection, and episcleritic nodules may be
present.

Conjunctivitis
It is characterized by very mild pain. The cornea is clear. Pupillary size and response to light is
normal. Visual acuity is not affected.

Allergic conjunctivitis
It is an acute hypersensitivity reaction that is caused by environmental exposure to allergens.
Intense itching, hyperemia, tearing and conjunctival & eyelid edema are characteristic. Some
patients complain of photophobia and a burning sensation. There is usually a family or personal
history of asthma, seasonal rhinitis, atopic dermatitis, food allergies and urticaria. The condition
usually subsides in 24 hours, even without treatment. Reducing exposure to allergens can
prevent conjunctivitis. Patients should be advised to never rub their eyes and to use topical
antihistamines, artificial tears and cool compresses.
Toxic conjunctivitis
It occurs due to direct damage to ocular tissues from drugs. It happens with prolonged use of
the offending agent. A history of using aminoglycoside antibiotics, glaucoma drops, artificial
tears or contact lens solution can be elicited in patients.

Atopic Keratoconjunctivitis
It is a severe form of ocular allergy. The most common symptom is itching, tearing, thick mucus
discharge, photophobia and blurred vision. Usually, visual disturbances do not occur with
allergic conjunctivitis.

Blepharitis
Blepharitis is a chronic inflammatory condition involving the lid margins bilaterally. Crusty
discharge is seen clinging to the lashes in anterior blepharitis, whereas hyperemic lid margins
with talengectasias are seen in posterior blepharitis.

Corneal Abrasion
It presents with severe pain and photophobia. There is usually a history of trauma to the eye.
Slit lamp examination with flourescein will reveal the corneal abrasion.

Subconjunctival Hemorrhage
It is a completely benign condition which may be due to simple trauma from rubbing the eyes
vigorously, violent coughing spells, hypertensive episodes or coagulopathy. In the majority of
cases, the condition is from minor bruising and does not require any workup at all. The
hemorrhage usually disappears in 24-48 hours. Simple observation is the best treatment. The
occasional patient with subconjunctival hemorrhage may have an elevated blood pressure. In
such rare cases, lowering of the blood pressure may be useful.

Hordeolum
It refers to an abscess located over the upper or lower eyelid. It is usually caused by
Staphylococcus aureus. It appears as a localized, red, tender swelling over the eyelid. It is
treated with warm compresses. Incision and drainage is performed if resolution does not begin
in the next 48 hours.

Chalazion
It presents as lid discomfort. It is a chronic, granulomatous inflammation of the meibomian
gland. It appears as a hard, painless lid nodule.
Postoperative Endophthalmitis
It is the most common form of endophthalmitis. It usually occurs within 6 weeks of surgery. It is
an infection within the eye, particularly the vitreous. Patients usually present with pain and
decreased visual acuity. Examination reveals swollen eyelids and conjunctiva, hypopyon,
corneal edema and infection. The vitreous can be sent for Gram stain and culture. Based on the
severity, the intravitreal antibiotic injection or vitrectomy is done.

Corneal ulceration
It presents as a foreign body sensation, blurred vision, photophobia and pain. A history of
contact lens use, recent trauma or ocular disease may be present. The eye is erythematous, and
ciliary injection is present. Purulent exudates are seen in the conjunctival sac and on the ulcer
surface.

Cavernous sinus thrombosis

Cavernous sinus thrombosis is characterized by proptosis, ophthalmoplegia, chemosis and
visual loss. It occurs due to hematogenous spread from an infected, inflamed sinus.

CMV Retinitis
It is the most serious ocular complication of HIV-positive patients. It occurs in AIDS patients
with a CD4 count less than 50 cells/μL. It is typically painless and does not cause initial
conjunctivitis or keratitis. It presents as yellowish-white fluffy/granular patches of retinal
opacification and retinal hemorrhages. In the initial stage, the eye is usually white and quiet.
The treatment is ganciclovir or foscarnet.

Ocular Toxoplasmosis
In the immunocompromised host, it is characterized by typical sever necrotizing
retinochoroiditis. More than half of patients with ocular manifestations have encephalitis. The
necrosis involves the inner layers of the retina, which appears as white, fluffy lesions
surrounded by retinal edema and vitritis.

HIV retinopathy
It presents as benign, cotton wool spots in the retina which remit spontaneously.

Candida Endophthalmitis
Candida can cause endophthalmitis, especially with disseminated candidiasis infection. Patients
with Candida fungemia who appear sick should be evaluated by an ophthalmologist for possible
endophthalmitis.
Medications:
Mannitol: First line treatment for acute angle closure glaucoma. It is an osmotic diuretic and
works immediately.
Acetazolamide: CAI that rapidly reduces the production of aqueous humor. It is applied
topically.
Pilocarpine: It rapidly reduces IOP by opening the canals of Schlemm and allowing for drainage
of the aqueous humor. It is administered topically.
Timolol: It is a Beta blocker that decreases the intraocular pressure by decreasing production of
the aqueous humor. It is administered topically.

Non-Ketotic Hyperosmolar Syndrome (NKHS)

Type II diabetics are prone to develop NKHS in association with physiologic stressors like
infection. Such stress causes elevations in the serum cortisol and catecholamine levels,
substances that serve as insulin counterregulatory hormones. Sever hyperglycemia (>600) in
the absence of significant ketosis can result. The resultant osmotic dieresis can cause
dehydration and serum hyperosmolarity (>320 mOsm/L).
Altered consciousness ranging from confusion to coma is the main symptom of the metabolic
disturbances in NHKS. Acute hyperglycemia can also cause blurred vision due to myopic
increase in lens thickness and intraocular hypotension secondary to hyperosmolarity.

Note: cataracts can be a manifestation of chronic diabetes, especially in patients with

suboptimal glycemic control. In these patients, cataracts are thought to result from the gradual
accumulation of intralenticular sorbitol. This process would take longer than a week to develop.

Choroid Rupture
It occurs due to blunt ocular trauma. Examination reveals central scotoma, retinal edema,
hemorrhagic detachment of the macula, subretinal hemorrhage and crescent-shaped streak
concentric to the optic nerve. The usual complaint is blurred vision following blunt trauma.

Intraocular Foreign body

Be overly suspicious for an intraocular foreign body in patients with high velocity injuries
(drilling, grinding…). If the initial pen light examination does not reveal any conjunctival and
corneal abrasions or foreign bodies, proceed with Fluorescein examination.
Sympathetic Ophthalmia
It is known as “spared eye injury” and is characterized by an immune mediated inflammation of
one eye (the sympathetic eye), after penetrating injury to the other eye. The typical
manifestation is anterior uveitis, but panuveitis, papillary edema, and blindness may develop.
The pathophysiological mechanism is believed to be the uncovering of hidden antigens. Some
antigens contained within the eye are protected from immunologic recognitions by natural
barriers. Breaking these barriers results in the uncovering of hidden antigens. An immune
response against these antigens can involve autoantibodies as well as a cell-mediated reaction.

Optic Glioma
Intensive freckling and café-au-lait spots are suggestive of neurofibromatosis, type 1. Optic
glioma is a well known complication of NF-1. It occurs in 15% of patients, mostly in children
younger than 6 years of age. A history of slowly progressive unilateral visual loss and
dyschromotopsia are characteristic. Exophthalmos is sometimes present on PE. The optic disc
may be normal, swollen, or atrophic.

Ocular manifestations of certain conditions

Retinal hemartoma is typical for tuberous sclerosis
Pituitary adenoma results in bilateral hemianopsia
Pigment retinitis leads to bilateral visual loss

Presbyopia
It is a common age related disorder that results from loss of elasticity in the lens. This decrease
in elasticity prohibits accommodation of the lens, which is required in order to focus on near
objects. The tendency of patients to hold reading material at a further distance is classis for
presbyopia. Patients often have no history of eye problems. Most patients will report the onset
of presbyopia while they are in their forties, and symptoms typically peak at some point in their
sixties. The poor near vision associated with presbyopia can easily be improved with reading
glasses.

Papilledema
When intracranial pressure is increased, the pressure is transmitted to the optic nerve sheath
resulting in swelling of the optic nerve head, which can be visualized as papilledema on
ophthalmologic examination. Papilledema can cause visual symptoms such as momentary loss
of vision that varies according to changes in head positioning. Normal individuals have a blind
spot in their visual fields at the location of the optic nerve head, but this blind spot increases in
patients with papilledema. Headaches due to increased ICP are often worse in the morning.
Trachoma
It is caused by Chlamydia trachomatis serotype A-C. It is a major cause of blindness worldwide.
It usually presents 5-15 days after birth. The active phase of the disease is caused by follicular
conjunctivitis and pannus (neovascularization) formation in the cornea. Concurrent infection
occurs in the nasopharynx, leading to nasal discharge. The diagnosis can be made by Giemsa
stain examination of the conjunctival scrapings. Topical tetracycline or oral azithromycin should
be started immediately. Repeated infections can lead to scarring of the cornea.

Gonococcal Conjunctivitis
It is acquired through contact with infected genital secretions, and occurs 2-3 days after birth. It
presents as copious, purulent eye discharge with swollen eyelids and chemosis. It can be quite
severe, leading to corneal ulceration and blindness if left without treatment. In neonates with
suspected gonococcal conjunctivitis, a gram stain of the conjunctival exudates should be done
to look for intracellular gram negative diplococcic, and culture should be performed on Thayer-
Martin media.

Chemical conjunctivitis
Is occasionally seen in neonates who are given prophylactic silver nitrate eye drops, but it
would not be suspected to produce purulent discharge. It occurs in the first 24 hours after
birth.