Ophthalmologic Emergencies

Emerg Med Clin N Am
26 (2008) xiii–xiv
Foreword
Amal Mattu, MD
Consulting Editor
Noted English writer Max Beerbohm was quoted as saying ‘‘. . .the eyes
are the window of the soul.’’ Beerbohm may have been discussing human
nature, but the eyes are no less important in medicine. The eyes provide
important information regarding a patient’s neurologic status, including
the cranial nerves, cortical and brainstem function, toxic and metabolic dis-
orders, atherosclerotic disease, diabetes, connective tissue disease, thyroid
disease, and a myriad of other systemic ailments in addition to intrinsic oph-
thalmologic disorders. Though the eyes may be important to the physician,
they are even more important to the patient; none of the other four major
senses (touch, taste, hearing, smell) are as important as vision. The ability
for a person to function well in our society is influenced tremendously by
the ability to see. Vision is so important of a sensation, in fact, that an entire
specialty is devoted to the maintenance of this sensation.
Therefore, it is vital for emergency physicians to have an excellent under-
standing of ophthalmologic emergencies. Emergency physicians must have
a sound knowledge of the anatomy and physiology of the eye, be well-versed
in proper examination techniques, and be able to recognize intrinsic and
extra-ocular manifestations of disease. Emergency physicians also should
have proficiency in proper management of these conditions to ensure pres-
ervation of vision in high-risk conditions. Acute visual complaints often re-
ceive less attention than other perilous conditions, such as chest pain in
a busy emergency department (ED), but the consequences of misdiagnosis
are equally disastrous for the patient and in terms of medicolegal risk.
0733-8627/08/$ - see front matter Ó 2008 Elsevier Inc. All rights reserved.
doi:10.1016/j.emc.2007.12.003 emed.theclinics.com
xiv FOREWORD
In this issue of Emergency Medicine Clinics of North America, guest

editors Drs. Kahn and Magauran have assembled an outstanding group
of authors to educate us on this vital aspect of our specialty. Important
articles are devoted to proper examination techniques and procedures,
and subsequent articles are devoted to the myriad of ophthalmologic condi-
tionsdmedical, toxic, and traumaticdthat are encountered in ED patients.
The articles are organized primarily in a complaint-based format, rather
than a disease-based format, which simulates the real-world experience of
patient presentations in the ED. Articles also are devoted to ophthalmologic
manifestations of systemic disease, neuro-ophthalmologic conditions, and
pediatric issues. This issue also includes an immensely practical article that
focuses on conditions that require emergency ophthalmologic consultation,
an article that is worth posting in every ED and urgent care center.
I congratulate the editors and authors on producing an outstanding
addition to the Clinics series. This issue should be considered required read-
ing not only for practicing emergency physicians, but also for emergency
medicine trainees and for any other health care providers who manage
patients who have acute ophthalmologic complaints in their daily practice.
This issue certainly provides us all with a ‘‘window’’ into better patient care
and decreased medicolegal risk.
Amal Mattu, MD
Program Director
Emergency Medicine Residency
and
Associate Professor
Department of Emergency Medicine
University of Maryland School of Medicine
110 S. Paca Street, 6th Floor, Suite 200
Baltimore, MD 21201, USA
E-mail address: [email protected]
Emerg Med Clin N Am
26 (2008) xi
Erratum
An Emergency Medicine Approach
to Neonatal Hyperbilirubinemia
James E. Colletti, MD, FAAEM, FAAPa,
Samip Kothori, MDb, Danielle M. Jackson, MDc,
Kevin P. Kilgore, MDc, Kelly Barringer, MDc
a
Department of Emergency Medicine, Mayo Clinic College of Medicine,
200 First St. SW, Rochester, MN 55905, USA
b
Department of Pediatrics, University of Arizona, 150 N. Campbell Avenue,
Tucson, AZ 85724, USA
c
Department of Emergency Medicine, Regions Hospital, 640 Jackson Street,
Mail Stop 11102F, St. Paul, MN 55101, USA
The above article, which appeared in the November 2007 issue (‘‘Pediatric
Emergencies in the First Year of Life’’), contained a misspelling of the second
author’s name. The correct spelling is Samip Kothari, MD.
Emerg Med Clin N Am
26 (2008) xv–xvi
Preface
Joseph H. Kahn, MD, FACEP Brendan Magauran, MD, MBA

Guest Editors
With more than 2 million visits annually to United States emergency de-
partments, patients who have complaints involving the eye and its surround-
ing tissues are encountered frequently by the emergency physician. Eye
complaints represent the full range of disease, from trivial (uncomplicated
subconjunctival hemorrhage) to life-threatening (intracranial aneurysm).
This issue attempts to provide a framework to approach patients who have
eye complaints.
The issue begins with ‘‘Physical Exam of the Eye,’’ which reviews the anat-
omy of the eye, including its innervation and blood supply and suggests how to
perform a rapid, yet thorough, physical exam of the eye. There is an article
entitled ‘‘Ophthalmologic Procedures’’ that reviews commonly performed
ophthalmologic procedures, such as measurement of visual acuity, tonometry,
and slit lamp evaluation, and infrequently used procedures, such as
paracentesis and lateral canthotomy, with a discussion of ocular ultrasound.
The subsequent articles approach the patient who has eye complaints in one of
two ways: a system-based approach and a problem-based approach.
The system-based articles include ‘‘Ocular Infection and Inflammation,’’
which discusses topics from conjunctivitis to scleritis and orbital cellulitis.
The next system-based article is entitiled ‘‘Trauma to the Globe and Orbit,’’
and is a comprehensive article that covers topics ranging from corneal
abrasion to hyphema, ruptured globe, and retro-orbital hemorrhage.
Another system-based article is ‘‘Neuro-Ophthalmology,’’ which presents
a detailed discussion of the unique ocular findings seen in neurologic
xvi PREFACE
diseases, including afferent papillary defect, Argyll Robertson pupil, and

Adie’s tonic pupil. The final system-based article is ‘‘Ophthalmologic Com-
plications of Systemic Disease,’’ which covers topics ranging from diabetes
to acquired immunodeficiency syndrome.
The first of the problem-based articles is ‘‘The Red Eye,’’ and it discusses
the differential diagnosis and treatment of this very common emergency
department presentation, which ranges from conjunctivitis to acute angle
closure glaucoma. Another problem-based article is ‘‘Acute Visual Loss,’’
and it discusses central retinal artery occlusion, retinal detachment, and
other causes of this condition that can be alarming for the physician and
the patient. The next problem-based article is ‘‘Eye Exposures,’’ which
attempts to guide the emergency physician in the management of conditions
ranging from alkali exposures to biologic exposures to health care workers.
The final problem-based article is entitled ‘‘The Painful Eye,’’ which dis-
cusses topics including uveitis and optic neuritis. There is a separate article
entitled ‘‘Pediatric Ophthalmology in the Emergency Department’’ that pro-
vides guidelines for the evaluation and management of ocular complaints in
children of various age groups. The final article, entitled ‘‘Conditions Re-
quiring Emergency Ophthalmologic Consultation,’’ cuts through the myriad
of ophthalmologic complaints that patients present with and presents those
diagnoses, such as endophthalmitis and corneal ulceration, that require im-
mediate attention by an ophthalmologist.
We thank all of the authors who so carefully researched and wrote the
articles in this edition of Emergency Medicine Clinics of North America.
We also thank our families for their support as we revised and assembled
this issue. We thank Patrick Manley and the staff at Elsevier for their
guidance and patience. We especially thank those of you who read this
edition; we sincerely hope that you find it worthwhile.
Joseph H. Kahn, MD, FACEP

Brendan Magauran, MD, MBA
Department of Emergency Medicine
Boston Medical Center
1 Boston Medical Center Place
Boston, MA 02118, USA
E-mail addresses: [email protected]; [email protected]
Emerg Med Clin N Am
26 (2008) 1–16
The Physical Examination of the Eye

Derek A. Robinett, MDa,b,
Joseph H. Kahn, MD, FACEPa,b,*
a
Boston University School of Medicine, 80 East Concord Street, Boston, MA 02118, USA
b
Department of Emergency Medicine, Boston Medical Center,
1 Boston Medical Center Place, Boston, MA 02118, USA
There are more than 2 million visits annually to United States emergency
departments for complaints involving the eye and its surrounding tissues [1].
Approximately one half of these visits are related to trauma to the eye and its
adnexal tissues, 3% of which require admission to the hospital [1,2]. Six per-
cent of eye injuries presenting to emergency departments annually are related
to the use of drugs and/or alcohol [1]. Most of these patients are treated ini-
tially by emergency physicians, with or without ophthalmology consultation.
Performing a complete, accurate, and efficient physical examination of the
eye allows the emergency physician to treat most eye complaints and to com-
municate effectively with the ophthalmologist on call. The physical examina-
tion of the eye requires a Snelling Chart (or substitute), a light source,
ophthalmoscope, slit lamp, a method for determining intraocular pressure,
proparicaine ophthalmic solution, and fluorescein stain [3].
Knowledge of the anatomy of the eye is essential in emergency medicine.
To properly diagnose ocular problems, one should have full knowledge of
the eye, orbit, cranial nerves, musculature for the control of eye movements,
and blood supply of the eye.
The orbit
The orbit is composed of four walls. The roof of the orbit is comprised of
the frontal bone and the lesser wing of the sphenoid bone. The lateral wall is
made of the zygomatic bone and the greater wing of the sphenoid bone. The
lateral wall and roof are separated by the superior orbital fissure. The floor
of the orbit is composed primarily of the maxilla. However, portions of the
zygomatic and palatine bones contribute to the floor. The medial wall is
formed by portions of multiple bones including the ethmoid, lacrimal,
* Corresponding author.
E-mail address: [email protected] (J.H. Kahn).
2 ROBINETT & KAHN
sphenoid, frontal, and maxilla. The anterior portion of the orbit is narrower
than the area behind the rim, which adds protection [4–7].
The orbit lies in close proximity to the paranasal sinuses (Fig. 1), allowing
sinus infections to spread to the periorbital tissues (preseptal cellulitis) and
into the orbit itself (orbital cellulitis) [8]. These conditions will be discussed
in detail in an article by Mueller and McStay elsewhere in this issue.
The external eye

The external anatomy of the eye is made up of the eyebrows, the eyelids,
and the lacrimal apparatus. The eyebrows consist of thick skin and hair with
muscle fibers underneath. The eyelids function to protect the eyeball by clos-
ing. They are made of thin skin covering the orbicularis oculi muscle. This
muscle closes the lids. Beneath the muscle is areolar tissue followed by a fi-
brous connective tissue layer called the ‘‘tarsal plate.’’ The lids are covered
on their posterior surface by mucosa called the ‘‘palpebral conjunctiva.’’ Be-
tween the orbit and tarsal plate lies fascia that makes up the orbital septum.
The lacrimal apparatus is composed of the lacrimal gland, lacrimal sac,
canaliculi, and the nasolacrimal duct. Many accessory lacrimal glands
also exist. The lacrimal gland produces tears that coat the anterior portion
of the eye. The tears are drained through the canaliculi to the lacrimal sac.
The lacrimal sac drains into the nasal cavity via the nasolacrimal duct [5,6].
During examination of the external structures surrounding the eye, many
diseases and injuries should be looked for specific to the structures being exam-
ined. Examination of the lids may reveal chalazion, hordeolum, or lid cellulitis,
all of which will be discussed by Mueller and McStay in an article found else-
where in this issue. Abnormalities of the orbicularis oculi muscle may be seen in
seventh cranial nerve palsies. Lid lag (ptosis) may be seen in third cranial nerve
palsies and in Horner’s syndrome. These entities will be discussed by Duong,
Leo, and Mitchell in an article found elsewhere in this issue. Conjunctivitis
Fig. 1. (A) Axial cross sections of the maxillary sinus and orbit. The sinus is completely
occluded with, in this case, pus. (B) Dorsally, of the eyeball, an expansile process with visible
gas inclusions is visible. Gas-producing microorganisms are the likely source of this hypodense
area. (From Blake FAS, Siegert J, Wedl J, et al. The acute orbit: etiology, diagnosis, and ther-
apy. J Oral Maxillofacial Surg 2006;64:91; with permission.)
THE PHYSICAL EXAMINATION OF THE EYE 3
may involve the inner lining of the eyelids (palpebral conjunctiva). The palpe-
bral conjunctiva lining the upper lid is a common site for occult ocular foreign
bodies. All elements of the tear-producing and collecting system are subject to
infection (dacrocystitis) or obstruction and require special attention in the set-
ting of trauma, because lacerations to these structures may require repair by an
ophthalmologist. These injuries will be discussed in an article by Bord and Lin-
den elsewhere in this issue.
The eyeball
A review of daytime presentations to an ophthalmologic emergency de-
partment in Sydney, Australia found the following five most common diag-
noses: conjunctivitis, keratitis, cataract, corneal abrasion, and iridocyclitis [9].
The conjunctiva
A thin mucous membrane covers the posterior lids and anterior sclera.
This is the conjunctiva. It is continuous with the skin at the margin of the
lid. In the United States, more than 500,000 patients present to the emer-
gency department annually for inflammation of the conjunctiva [1]. Con-
junctivitis will be discussed in detail in the article ‘‘Ocular Infection and
Inflammation’’ by Mueller and McStay and ‘‘The Red Eye’’ by Mahmood
and Narang elsewhere in this issue.
Tenon’s capsule
Tenon’s Capsule (also called the fascia Bulbi) is a fibrous globe that en-
velops the eyeball from the optic nerve to the limbus. Tubular reflections of
the capsule enclose each extraocular muscle at its site of attachment and fuse
with the fascia of these muscles and attach to adjacent orbital structures.
These reflections limit the movement of these muscles [5,6].
The sclera and episclera
The fibrous coating of the eye is the sclera. This protective coating is
made almost entirely of collagen. It is white in color and very dense. Ante-
riorly, it is continuous with the cornea. The episclera is a thin layer of elastic
tissue that covers the anterior sclera [4,6].
Inflammation of these layers may be associated with connective tissue
disorders. Scleritis is usually painful, whereas episcleritis is not. The redness
associated with episcleritis blanches with instillation of phenylephrine drops,
whereas the redness associated with scleritis does not. See the article by Muel-
ler and McStay for a detailed discussion of these diseases.
The cornea
The transparent structure covering the anterior of the eye is the cornea. It
is composed of five separate layers: the epithelium, Bowman’s layer, the
4 ROBINETT & KAHN
stroma, Descemet’s layer, and the endothelium. The epithelium is highly

prone to damage [6].
Corneal abrasions and foreign bodies comprise the majority of corneal
abnormalities presenting to emergency departments. Other entities include
keratitis, corneal ulcers, corneal perforation, and corneal exposures. Propar-
icaine to make the patient comfortable and fluorescein instillation to visual-
ize corneal defects, in conjunction with slit lamp evaluation, are essential to
evaluate the cornea. Corneal abnormalities are discussed in the articles ‘‘The
Painful Eye’’ by Dargin and Lowenstein and ‘‘Ocular Infection and Inflam-
mation’’ by Mueller and McStay in this issue.
The anterior chamber

Between the cornea and iris is the anterior chamber. The anterior cham-
ber is filled with aqueous humor. The aqueous is formed by the ciliary body.
It is drained from the anterior chamber by the trabecular meshwork. The
trabecular meshwork is a series of collagen and elastic tissue with trabecular
cells that form a filter. The ciliary muscle can contract and increase the size
of the filter pores. This enhances the drainage of the aqueous. The aqueous
is absorbed at a venous channel (the canal of Schlemm) where the iris and
cornea join. This is the angle of the anterior chamber [4–7].
People with a narrow anterior chamber angle are at risk for acute angle
closure glaucoma (Figs. 2 and 3). This vision-threatening, sometimes elusive
diagnosis will be discussed in the article by Mahmood and Narang in this
issue.
The uvea
The uvea consists of the iris and the ciliary body (anterior uvea) and the
choroid (posterior uvea) [6].
Fig. 2. Anterior-segment optical coherence tomography using 1.3-mm wavelength light provides
better detail of anterior chamber angle anatomy. (From Radhakrishnan S, Huang D, Smith SD.
Optical coherence tomography testing. Ophthalmol Clin North Am 2005;18:376; with permission.)
Fig. 3. Anterior segment optical coherence tomography image shows anterior chamber angle
anatomy. (From Radhakrishnan S, Huang D, Smith SD. Optical coherence tomography testing.
Ophthalmol Clin North Am 2005;18:376; with permission.)
The iris
The amount of light entering the eye is controlled by the iris. It is an ex-
tension of the ciliary body. It is a flat surface with a central, round opening
(the pupil). The posterior surface of the iris is heavily pigmented. The
stroma of the iris contains the sphincter and dilator muscles. The size of
the pupil is controlled by parasympathetic innervation (contraction) and
sympathetic innervation (dilation) [4–7].
The iris is the site of abnormalities of both traumatic and atraumatic eti-
ologies. Iritis (anterior uveitis) may be infectious, rheumatologic, or idio-
pathic. Trauma may cause traumatic iritis, traumatic midriasis, or tears of
the iris. Anterior uveitis will be discussed in the articles entitled ‘‘The Red
Eye’’ by Mahmood and Narang and ‘‘The Painful Eye’’ by Dargin and
Lowenstein in this issue.
The ciliary body
The ciliary body is composed of a series of veins and capillaries. The ep-
ithelium covering this body produces the aqueous. The ciliary muscle is
made up of a series of muscle fibers arranged in a longitudinal, circular,
and radial direction. These fibers can contract to change the shape of the
lens and provide for different focal lengths [6].
The choroid
The choroid lies between the retina and sclera. It is comprised of a series
of blood vessels that provide flow to the outer portion of the retina. There
are three layers of these blood vessels [6].
Posterior uveitis, or choroiditis, is a vision-threatening emergency requir-
ing urgent treatment. It is discussed in detail in the article by Mueller and
McStay elsewher in this issue.
The lens
The lens is approximately 4 mm thick and 9 mm in length. It is convex,
colorless, and transparent. It is held behind the iris by the zonule. The
6 ROBINETT & KAHN
zonule connects to the ciliary body. As one ages, the lens becomes larger and
less flexible. The lens contains no nerves or blood vessels [6].
The lens may be dislocated in the setting of trauma. Also, trauma may cause
cataract formation. See the article by Bord and Linden elsewhere in this issue.
The retina
The specialized neural tissue lining the posterior, inner part of the globe is
the retina. It is semitransparent and multilayered. It is composed of ten
layers containing nervous tissue, pigmented epithelial cells, and photorecep-
tors. The retina is easily detached from its epithelium, except at the optic
disk [4–6].
Retinal detachment, a vision-threatening ophthalmologic surgical emer-
gency, can be recognized by historical features (visual loss, floaters, flashing
lights) and on physical examination (elevated portion of retina on fundo-
scopic examination). This will be discussed in detail in the by Vortmann
and Schneider found elsewhere in this issue.
The vitreous
Making up two thirds of the volume and weight of the eye, the vitreous
fills the space between the lens and retina. It is clear and gelatinous. It is
composed of collagen and hyaluronic acid but is 99% water [4–6].
Vitreous floaters are defined as dots or spots floating in the field of vision
that change position when the patient shifts his or her gaze to a different di-
rection [10]. They may be from blood or debris in the vitreous fluid. They
may be caused by retinal detachment or hemorrhage.
The extraocular muscles

The extraocular muscles control the movements of the eye. There are six
extraocular muscles.
The rectus muscles

The eye has four rectus muscles that are named by their insertion: medial,
lateral, superior, and inferior. They originate from a common site, a ring
shaped tendon called the annulus of Zinn. These muscles adduct, abduct,
elevate, and depress the eye [4–6].
The oblique muscles

There are two oblique muscles of the eye. They provide some upward and
downward movement of the eye but primarily provide rotational movement.
These muscles are the superior oblique and the inferior oblique [4–6].
Disorders of upward gaze in a traumatized eye may be caused by orbital
floor fracture with inferior rectus entrapment. This will be discussed in detail
in the article by Bord and Linden elsewhere in this issue. Inability to move
the eye, associated with proptosis, may be caused by orbital cellulitis or or-
bital hemorrhage.
Innervation of the eye

The eye is innervated by the cranial nerves listed below.
The optic nerve (II)

The optic nerve is composed mainly of visual fibers. It arises from the ret-
ina and consists of approximately one million axons. After leaving the
globe, the nerve becomes myelinated. Each nerve is then enwrapped in
a sheath of fibrous tissue that is contiguous with the meninges. It passes
through the orbit and joins the nerve from the other eye at the optic chiasm.
At the chiasm, nasal fibers from each optic nerve cross to the opposite tract.
These axons then course to the lateral geniculate nucleus. After a synapse at
the lateral geniculate nucleus, the nerves end in the primary visual cortex of
the occipital lobes [4–6].
The oculomotor nerve (III)

The oculomotor nerve originates from the brainstem. It passes through
the cavernous sinus and splits into inferior and superior branches. The su-
perior branch innervates the superior rectus muscle. The inferior branch in-
nervates the inferior and medial rectus muscles and the inferior oblique
muscle. The inferior branch also provides parasympathetic innervation to
the ciliary body [4–6].
The trochlear nerve (IV)

The trochlear nerve begins on the dorsal surface of the brain stem. It
travels along the lateral wall of the cavernous sinus. It innervates the supe-
rior oblique muscle [4–6].
The trigeminal nerve (V)

The trigeminal nerve begins in the pons and forms the trigeminal ganglion.
It then splits into three divisions. The first division is the ophthalmic division
(V1). The ophthalmic division passes through the cavernous sinus and divides
to provide sensation to the brow, forehead, cornea, iris, and ciliary body. It
also provides sensation to the conjunctiva, eyelids, and the tip of the nose.
The second division is the maxillary division (V2). It passes through the in-
fraorbital canal and forms the infraorbital nerve. This provides sensation
to the lower lid and cheek. The third division is the mandibular division
(V3). It provides sensation over the lower portion of the face and mouth. It
also provides innervation to eight of the muscles of the face and jaw [4–6].
8 ROBINETT & KAHN
The abducens nerve (VI)

Originating between the medulla and pons, the abducens nerve passes
within the cavernous sinus. It innervates the lateral rectus muscle [4–6].
Multiple disorders involve the innervation of the eyes. Optic neuritis may
herald the onset of multiple sclerosis. It causes decreased visual acuity, an
afferent papillary defect, and pain on eye movement. Fundoscopic examina-
tion may be normal (if the inflammation is retrobulbar) or may reveal edema
of the optic disc (if the inflammation is anterior) [11].
Unilateral or bilateral abducens nerve palsy (inability to abduct one or
both eyes) may be an early sign of increased intracranial pressure [12]. A
posterior communicating artery aneurysm may compress the ophthalmic
nerve, causing lid lag and ophthalmoplegia [12]. These entities will be dis-
cussed in detail in the article by Duong, Leo, and Mitchell found elsewehere
in this issue. Furthermore, trauma can cause an orbital hemorrhage, which
may lead to increased orbital pressure and ischemia of the optic nerve.
The blood supply of the eye

The first branch of the intracranial section of the internal carotid artery is
the ophthalmic artery. The ophthalmic artery and its subsequent branches
supply the main blood source to the eye and its structures. After the ophthal-
mic artery enters the orbit, the first branch is the central retinal artery. As the
artery continues to course within the orbit, multiple other branches supply
the structures of the eye until the most superficial branches reach the eyelids.
Here they form arcades that anastomose with the external carotid artery.
The main venous drainage of the eye occurs via the superior and inferior
ophthalmic veins. These two veins receive the drainage from the eye includ-
ing the central retinal vein. They then empty into the cavernous sinus. The
venous supply of the periorbital skin is contiguous with the deeper venous
structures. Superficial skin infections can therefore spread via the venous
system into the cavernous sinus and deeper structures [4–6].
The blood supply to the eye can be threatened in several ways. Emboliza-
tion of plaque or clot to the central retinal artery can cause central retinal ar-
tery occlusion and acute painless monocular visual loss. Central retinal vein
occlusion also causes visual loss over a longer time period than central retinal
artery occlusion. Inflammation of the ophthalmic artery can result from tem-
poral arteritis, causing painless visual loss, which may be unilateral or bilateral
[11]. These disorders will be discussed in the article by Vortmann and
Schneider in this issue. Also, trauma can disrupt the blood supply to the eye.
Physical examination of the eye

In performing the physical examination of the eye, one should be able to
evaluate both anatomy and function of each eye. The evaluation of the anat-
omy should focus on whether the problem arises from the globe, the orbit,
or the external structures. The evaluation of the function of the eye should
include vision, alignment, and movements. Generally, the physical examina-
tion of the eye should begin with a measurement of visual acuity (an excep-
tion to this is when ocular exposure to toxic substances requires irrigation
before visual acuity measurement [13]din this case, a quick measurement
of ocular pH can precede irrigation if it can be achieved in a few seconds
and does not delay irrigation), then the examiner should start peripherally
with the periorbital area and work centrally to examine the orbits, lids, the
extraocular muscles, the conjunctiva, sclera and episclera, the uvea, the intra-
ocular pressure, the cornea, the pupils, the anterior chamber, and the fundus.
Vision
The standard way to test vision is by reading a Snellen chart at 20 feet
(Fig. 4). Each eye should be tested separately. The vision is then assigned
a two-digit score (for example, 20/50) for each eye. The first number repre-
sents the distance from the chart, and the second digit represents the small-
est line readable by the patient. Recording the vision separately for each eye,
even if the vision is the same in each eye, is essential (for example, visual acu-
ity: right eye 20/50, left eye 20/50). If a patient can read three letters on the
next line, this can be recorded as, for example, 20/50 þ3. If a patient gets 2
letters wrong on the last line, this can be recorded as, for example, 20/50 2
Fig. 4. Standard Snellen chart. (From Kniestedt C, Stamper RL. Visual acuity and its measure-
ment. Ophthalmol Clin North Am 2003;16:159; with permission.)
10 ROBINETT & KAHN
[12]. Patients who cannot read English can generally be tested with a tum-
bling E chart, in which the patient identifies the orientation of the letter E
(facing to right, left, up, down) [14]. Testing vision in children will be dis-
cussed in detail in the article by Prentiss and Dorfman in this issue.
If the subject wears glasses and they are unavailable, vision can be tested
through a pinhole. By reading the Snellen chart through multiple pinholes,
the image is transmitted centrally through the lens onto the retina, excluding
peripheral light. This allows for a clearer image to be formed and substitutes
for eyeglasses [6].
If the patient cannot read the chart, he or she should be moved closer to the
chart until he or she is 5 feet away. If he or she is still unable to read the chart,
the examiner should test vision by having the patient count fingers at 2 feet. If
the patient cannot count fingers accurately, the next step is to test whether he
or she can detect hand motion. If the patient is unable to detect hand motion,
the next step is to determine if he or she has light perception. The eye not being
tested must be completely covered to test for light perception. If the patient
can perceive light, the examiner can check to see if the patient can determine
which direction the light is coming from (light perception with projection) or
not (light perception without projection) [12]. If the patient is unable to detect
light in an eye, that eye is considered totally blind (no light perception) [6].
Near vision may be tested at the bedside with a near-vision card, held 14
inches from the patient’s face [12,15]. Poor performance on near-vision test-
ing can be caused by acute visual impairment but may also be caused by
presbyopia [16], which is age-related chronic impairment of near vision [12].
External examination
To begin the physical examination of the eye, one should start with the
external structures. The eyelids and surrounding areas should be examined
for swelling, erythema, warmth, skin growths, and tenderness. Trauma or
infection (periorbital cellulites or zoster) may become apparent on evalua-
tion of the peri-orbital region.
Proptosis can easily be identified with gross inspection of the orbits. Uni-
lateral proptosis may be caused by orbital cellulitis, orbital hemorrhage,
cavernous sinus thrombosis, or tumors. Bilateral exophthalmos is most
commonly caused by hyperthyroidism [12]. Proptosis can be quantified by
the ophthalmologist using an exophthalmometer [14]. Enophthalmos may
be caused by an orbital blow-out fracture. The bony structures should be
palpated to ascertain tenderness [6].
Lid evaluation may find ptosis, which may be congenital or acquired.
Newly acquired ptosis may represent Horner’s syndrome, third nerve palsy,
botulism, or myasthenia gravis [10]. Inability to close the lids may result
from weakness of the orbicularis oculi muscle, as in seventh cranial nerve
palsy. It is important to flip the upper lid if you are searching for a foreign
body. Careful evaluation of the lids may reveal infections of the lid (lid
cellulitis) or dacrocystitis, which is an infection of the tear collection system

(puncta, canaliculi, nasolacrimal duct). Other lid findings include hordeo-
lum and chalazion. A hordeolum is an acute inflammation of a meibomian
gland, and a chalazion is a chronic obstruction and inflammation of a mei-
bomian gland [10,11].
Extraocular movements
When testing extraocular movement, special note should be made of the
range, symmetry, smoothness, and speed of the eye movements. Nystagmus
can also be observed. To test these movements, the patient is asked to fixate
on a target with both eyes. The examiner then moves the target in four di-
rections [6]. Disorders of ocular motility may be caused by cranial nerve dys-
function, extraocular muscle entrapment, or increased pressure within the
orbit (orbital cellulitis or hemorrhage).
Examination of the conjunctiva

It is important to evaluate the conjunctiva lining the lids (palpebral con-
junctiva) and the conjunctiva on the surface of the eye (bulbar conjunctiva).
Conjunctival injection (prominence of vessels) may be diffuse or perilimbal
(radiating outward from the limbus, which is where the cornea meets the
sclera). Diffuse conjunctival injection usually results from inflammation or
infection within the conjunctiva itself (conjunctivitis), whereas perilimbal in-
jection may be the result of inflammation or infection within the uvea or
anterior chamber. Chemosis refers to edema of the conjunctiva [11].
Conjunctivitis will be discussed in detail in the article by Mahmood and
Narang found elsewhere in this issue.
Examination of the conjunctiva may also reveal a subconjunctival hemor-
rhage, which may be spontaneous or traumatic. Although these usually are
benign and self-limited, severe subconjunctival hemorrhages (360 of bulbar
conjunctiva) may be secondary to ruptured globe or coagulopathy [10].
Examination of the sclera and episclera

Inflammation of the episclera and sclera may be difficult to distinguish
from conjunctivitis. Episcleritis is usually painless and causes injection of
a sector of episcleral vessels. These vessels will blanche with application of
phenylephrine. Vision usually is preserved. It is self-limited and may be as-
sociated with collagen vascular diseases [11].
Scleritis causes injection of scleral vessels with a characteristic violaceous
color. It is painful and often causes decreased vision. The injection will not clear
with topical phenylephrine. It is associated with collagen vascular diseases and
certain infections (zoster, tuberculosis, syphilis). Treatment depends on sever-
ity but often includes systemic steroids [11]. See the article by Mahmood and
Narang in this issue for detailed discussion of scleritis and episcleritis.
12 ROBINETT & KAHN
Examination of the uvea

Inflammation of the anterior uvea (anterior uveitis, iritis, iridocyclitis) can
be detected by the associated photophobia and unilateral constricted pupil
that results from ciliary spasm as well as cells and flare in the anterior cham-
ber. Causes include trauma, viral, Reiter’s Syndrome, sarcoidosis, juvenile
rheumatoid arthritis, and retinoblastoma, and it often is idiopathic [11].
The iris should also be examined for traumatic tears at the papillary margin
and for iridodialysis (separation of the iris from the ciliary body) [11].
Inflammation of the posterior uvea can be seen as exudates around reti-
nal vessels and hazy vitreous humor. It can be caused by cytomegalovirus,
toxoplasmosis, sarcoidosis, syphilis, herpes, and other causes [10]. Anterior
and posterior uveitis will be discussed in the article by Mahmood and
Narang in this issue.
Intraocular pressure
The intraocular pressure (IOP) should be measured if glaucoma is sus-
pected. There is a detailed description of various tonometers in the article
by Babineau and Sanchez elsewhere in this issue. High pressures may result
from acute angle–closure glaucoma, open-angle glaucoma, hyphema, ca-
rotid-cavernous fistula, retro-bulbar hemorrhage, and other causes [10].
Low IOP may be seen in ruptured globe, retinal detachment, ocular ische-
mia, glaucoma medications, and other conditions.
Examination of the cornea

Corneal evaluation is accomplished with the aid of proparicaine, fluores-
cein, and the slit lamp. Defects in the corneal surface will appear bright yel-
low-green under the cobalt blue light on the slit lamp when stained with
fluorescein [17]. It is an essential part of the emergency eye examination
to carefully evaluate the cornea with the slit lamp for foreign bodies, abra-
sions, and ulcerations [17]. Many causes of infection and inflammation of
the cornea (keratitis) have specific patterns of fluorescein uptake on slit
lamp examination (herpes simplex, herpes zoster, adenovirus, ultraviolet
keratitis) [11]. Corneal edema, seen in acute angle closure glaucoma, will ap-
pear to the examiner as a ‘‘steamy’’ cornea, and the patient will see ‘‘halos’’
around lights. Keratitis will be discussed in detail in the article by Dargin
and Lowenstein in this issue.
Examination of the pupils

On gross examination, the pupils are symmetric and circular. As many as
20% of normal individuals may have anisocoria (unequal pupils) of less
than 1 mm [18,19]. Anisocoria also may be caused by uveitis, trauma, uncal
herniation, oculomotor nerve palsy, or Horner’s Syndrome and iatrogeni-
cally by dilating or constricting drops or nebulized ipratropium [20]. The
examiner should test the pupils’ response to direct light. As the pupil reacts
to direct light, a consensual response (constriction of the opposite pupil) will
also occur. After the examiner notes the degree and speed of response of
both the direct and consensual response in each eye, one should swing the
light between the two eyes (swinging flashlight test) [15]. Each pupil should
constrict with both direct and consensual light. If the pupil dilates as the
light is swung to it from the opposite pupil, this means that the pupil con-
stricts more vigorously to consensual light than direct light, the hallmark
of an afferent papillary defect. This is referred to as a ‘‘Marcus Gunn pupil’’
(Fig. 5) [4–6]. The Marcus Gunn pupil is also discussed in the article by
Duong, Leo, and Mitchell in this issue.
Examination of the anterior chamber

Hyphema (blood in the anterior chamber) and hypopyon (layer of white
blood cells [WBCs] in the anterior chamber) often can be seen without mag-
nification, but a slit lamp evaluation is essential to adequately evaluate the
anterior chamber. With a slit lamp, inflammation of the anterior chamber
will be seen as cells (WBCs floating in the aqueous) and flare (increased pro-
tein causing the aqueous to appear cloudy) [10]. Cells and flare in the ante-
rior chamber can be seen in uveitis, scleritis, and trauma [11]. The use of the
slit lamp is discussed in detail in the article by Babineau and Sanchez in this
issue (Fig. 6).
The depth of the anterior chamber can be approximated using a penlight.
The penlight is shone tangentially across the cornea from the temporal side.
If the entire cornea is illuminated, this implies a deep anterior chamber with
Fig. 5. Testing for relative afferent papillary difference. (A) The right optic nerve is damaged.
The light is shone in the left eye; both pupils constrict (the direct and consensual light reflex). (B)
The light is moved to the right eye, and there is no afferent limb to the reflex, thus both pupils
dilate. (From James B, Benjamin L. Ophthalmology: investigation and examination techniques.
China: Butterworth Heinemann (an imprint of Elsevier); 2007; with permission.)
14 ROBINETT & KAHN
Fig. 6. An overall view of the slit lamp. (From James B, Benjamin L. Ophthalmology: investi-
gation and examination techniques. China: Butterworth Heinemann (an imprint of Elsevier);
2007; with permission.)
a wide open angle. If the nasal portion of the cornea is in shadow, this
implies a shallow anterior chamber with a narrow angle [21].
Ophthalmoscopy
The use of a hand-held ophthalmoscope enables the examiner to visualize
the fundus. In a darkened room, the patient fixes his vision across the room,
and the examiner then brings the ophthalmoscope as close as possible to ex-
amine each eye. The dial on the ophthalmoscope can then be adjusted to fo-
cus the image. This allows for examination of the structures in the retina.
The examiner notes the optic disk, the vasculature, and the macula
(Fig. 7). Dilation of the pupil can assist in the visualization of the retina. Ex-
amination of the periphery of the retina requires the use of an indirect oph-
thalmoscope and usually is performed by an ophthalmologist [4–6].
Summary
This article has provided a review of the anatomy of the eye and its sur-
rounding tissues. A working knowledge of the functional anatomy of the eye
will aid the emergency physician in performing a thorough yet efficient
Fig. 7. Examples of optic disc abnormalities. (A) Normal disc: Note the distribution of the
neuroretinal rim around the disc. (B) A swollen disc: The margin is not clearly demarcated;
the patient had papilloedema. (C) The cup is enlarged, the neuroretinal rim thinned, and the
inferior-superior-nasal-temporal pattern lost. The patient has glaucoma. (D) New vessels are
growing at the disc: this patient had diabetes. (E) The disc has an irregular lumpy appearance
and optic disc drusen. (F) Myelinated nerve fibers at the disc margin. (From James B, Benjamin
L. Ophthalmology: investigation and examination techniques. China: Butterworth Heinemann
(an imprint of Elsevier); 2007; with permission.)
physical examination of the eye. A goal-directed physical examination of the

eye will allow the emergency physician to attempt to identify (or exclude)
vision-threatening disease processes and facilitate communication with the
ophthalmologist.
16 ROBINETT & KAHN
References
[1] Nash EA, Margo CE. Patterns of emergency department visits for disorders of the eye and
ocular adnexa. Arch Ophthalmol 1998;116:1222–6.
[2] Nawar EW, Niska RW, Xu J. National hospital ambulatory medical care survey: 2005 emer-
gency department summary. Advance Data from Vital and Health Statistics 2007;386:1–32.
[3] Khaw PT, Shaw P, Elkington AR. Injury to the eye. BMJ 2004;328:36–8.
[4] Lang G. Ophthalmology: a pocket textbook atlas. 2nd edition. New York: Thieme Medical
Publishers; 2007.
[5] Goodman R. Ophto notes: the essential guide. 1st edition. New York: Thieme Medical Pub-
lishers; 2003.
[6] Riordan-Eva P, Whitcher J. Vaughan & Ashbury’s general ophthalmology. 16th edition.
New York: The McGraw-Hill Companies; 2004.
[7] Schlote T, Rohrbach J, Grueb M, et al. Pocket atlas of ophthalmology. 1st edition. New
York: Thieme Medical Publishers; 2007.
[8] Blake FAS, Siegert J, Wedl J, et al. The acute orbit: etiology, diagnosis, and therapy. J Oral
Maxillofac Surgery 2006;64:87–93.
[9] Kumar NL, Black D, McClellan K. Daytime presentations to a metropolitan ophthalmic
emergency department. Clin Experiment Ophthalmol 2005;33:586–92.
[10] Kunimoto DY, Kanitkar KD, Makar MS, editors. The Wills eye manual: office and emer-
gency room diagnosis and treatment of eye disease. 4th edition. New York: Lippincott
Williams & Wilkins; 2004.
[11] Kaiser PK, Friedman NJ, Pineda R II. The Massachusetts eye and ear infirmary illustrated
manual of ophthalmology. 2nd edition. China: Saunders; 2004.
[12] Leitman MW. Manual for eye examination and diagnosis. 7th edition. Massachusetts:
Blackwell; 2007.
[13] Knoop K, Trott A. Ophthalmologic procedures in the emergency departmentdPart I: im-
mediate sight-saving procedures. Acad Emerg Med 1994;1:408–12.
[14] James B, Benjamin L. Ophthalmology: investigation and examination techniques. China:
Butterworth Heinemann; 2007.
[15] Chern KC. Emergency ophthalmology: a rapid treatment guide. Singapore: McGraw-Hill;
2002.
[16] Knoop K, Trott A. Ophthalmologic procedures in the emergency departmentdPart II: rou-
tine evaluation procedures. Acad Emerg Med 1995;2:144–50.
[17] Knoop K, Trott A. Ophthalmologic procedures in the emergency departmentdPart III: slit
lamp use and foreign bodies. Acad Emerg Med 1995;2:224–30.
[18] Adams RD, Victor M, Ropper AH. Adams and Victor’s Principles of Neurology. 7th edition.
New York: McGraw-Hill; 2001.
[19] Trobe J. Anisocoria. The eyes have it. Available at: http://www.kellogg.umich.edu/
theeyeshaveit/symptoms/anisocoria.html. Accessed October 8, 2007.
[20] Iosson N. Nebulizer-associated anisocoria. N Engl J Med 2006;354:e8.
[21] Sparks BI. Tangential penlight angle estimation. J Am Optom Assoc 1997;68:432–4.
Emerg Med Clin N Am
26 (2008) 17–34
Ophthalmologic Procedures
in the Emergency Department
Matthew R. Babineau, MD*,
Leon D. Sanchez, MD, MPH
Department of Emergency Medicine, Beth Israel Deaconess Medical Center, One Deaconess
Road, West Campus Clinical Center, 2nd Floor, Boston, MA 02215, USA
Ophthalmologic emergencies account for up to 3% of visits to emergency

departments in the United States [1]. Although isolated ocular complaints
are rarely life-threatening, they can lead to significant short- and long-
term morbidity, including permanent visual loss. The role of the emergency
physician in management of ocular emergencies is similar to that for other
chief complaints: (1) recognize and diagnose emergency conditions, (2)
provide appropriate initial therapy, and (3) ensure correct disposition.
This article reviews several of the essential ophthalmologic procedures
that are within the scope of emergency medical practice.
Visual acuity testing

A simple but vital part of the ophthalmologic examination is a test of
visual acuity. This is essential for all patients who have ocular or visual com-
plaints. The affected and non-affected eyes should be tested individually,
and then together, using a Snellen chart or equivalent. If the patient wears
corrective lenses during the examination (or is not wearing lenses that are
usually used), this should be noted. A critical part of the visual acuity exam-
ination is that decreased visual acuity should be rechecked using a pinhole
card. A pinhole corrects for most refractive errors, by ensuring that only
light striking the lens perpendicularly reaches the retina. Initially abnormal
visual acuity that corrects with a pinhole indicates a problem with the lens,
and is less concerning to an emergency physician. If this does not correct the
visual problems, it indicates pathology that is more likely located within the
retina or central nervous system.
E-mail address: [email protected] (M.R. Babineau).
18 BABINEAU & SANCHEZ
Slit lamp examination

Along with visual acuity, pupillary movements, extra-ocular muscle
movements, and fundoscopy (all reviewed elsewhere in this issue), the slit
lamp examination is an essential aspect of a complete ophthalmologic
examination [2]. The slit lamp is an illuminated biomicroscope that allows
a magnified, stereoscopic view of the eye and surrounding structures [2].
It is especially useful for evaluation of the anterior segment of the eye,
and should be a standard part of the examination in all ophthalmologic
complaints [3].
Despite some manufacturer-specific differences in slit lamps, some gen-
eral principles apply to the use of this device. Each unit has an illumination
system and an observation system. The illumination system is a light source
that projects through the slit aperture and onto the patient’s eyes. This part
should be mobile to allow different angles of light projection. Most systems
have a rheostat mechanism that allows the examiner to change the shape
and size of the slit beam, as well as a means to change the filter on the
beam (neutral, cobalt blue, and red-free) [4]. The observation system is a bin-
ocular microscope, which typically allows magnification from 6X to 40X.
The observation system is mounted on a chassis, which can be moved
horizontally with a joystick device. Often the handle of the joystick can
be rotated to allow for vertical movement of the observation system.
To perform the examination, the patient is asked to place his chin on the
chin rest, with the forehead resting on the forehead bar. Efforts should be
maintained to ensure that the patient is in a comfortable position, because
this will lead to a more efficient examination [5]. The chin rest is adjusted
so that the patient’s eyes rest at the level of the black marker on the vertical
pole next to the patients face [3]. The height of the slit lamp is adjusted so
that the slit beam is centered on the patient’s eyes. The patient should be
asked to fix his gaze on a specific location (such as the examiner’s ear) [5].
Initially, the examiner should adjust the diopter rings on the ocular piece
to account for his or her individual refractive error and accommodation [6].
This will also account for any misalignment in the instrument [6]. This is
done by using the focusing rod on the instrument [7]. The slit beam should
be set on a low-voltage, wide beam, and should be at 45 from the patient-
clinician axis [4]. The microscope should be set at its lowest magnification,
and the joystick is used to move the device on the chassis until the eye is
in focus. Once the eye is in focus, the clinician begins a systematic examina-
tion of individual structures. Although any system may work, the authors
recommend using the mnemonic L-L-L-L-L-C-C-C (Table 1). If any abnor-
mality is detected, the beam should be narrowed to help assess the depth,
extent, and level of the abnormality. At this time, the magnification can
also be increased for further evaluation.
Assessment of the anterior chamber (the region between the cornea and
iris) is perhaps the most difficult part of the examination. The purpose of the
OPHTHALMOLOGIC PROCEDURES IN THE EMERGENCY ROOM 19
Table 1
Mnemonic for systematically assessing all portions of the eye, eyelid and adnexae during routine
slit lamp examination
Stands for Example of pathology
L Lids (eyelids) Lacerations, edema, meibomian cyst
L Lashes (eyelashes) Discharge, inverted eye lash
L Lacrima Discharge, plugging, cannalicular lacerations
L Lens (and iris) Opacification/cataract, dislodgement, pupil shape and
function, synechiae, iridodialysis, blood vessels
L Limbus Injection
C Conjunctiva (and sclera) Hyperemia, follicles, chemosis, foreign body
C Cornea (and iris) Edema, foreign body, abrasions, ulcers, opacification,
keratitic precipitates
C Chamber (anterior) Cells/flare, hyphema/hypopion, shallow chamber
anterior chamber examination is mainly to assess the depth of the chamber

(narrow in acute closed angle glaucoma), and to assess for the presence of
‘‘cells and flare’’ (present in inflammatory conditions such as uveitis).
To assess anterior chamber angle depth, use low magnification and a nar-
row slit beam at 60 from the patient-clinician axis [8]. The beam is centered
as close to the limbus as possible, and normal to the cornea [4]. The depth is
assessed by comparing the width of the cornea as seen by the light beam
with the depth of the aqueous, which is represented by the darker section
between the iris and the posterior aspect of the cornea [4]. An aqueous-to-
cornea ratio of smaller than 1:4 (eg, a thin dark section compared with
the corneal thickness) indicates a dangerously narrow angle, whereas a ratio
of greater than 1:2 indicates an open angle [4]. To assess for cells and flare,
the slit beam should be between 45 and 60 , and focused onto the front of
the cornea. The examiner should assess the space between the focused beam
of light (on the cornea), and the more diffuse light (on the lens), using the
pupil as a dark background. In normal patients, this area should be
completely clear. ‘‘Flare’’ appears as scattered light, and is caused by the
presence of inflammatory proteins in the aqueous humor; cells appear as
small, floating particulate matter within the aqueous (Fig. 1) [3].
Flourescein examination
The flourescein examination is another important aspect of a complete
ophthalmologic examination. It is used to detect defects in the corneal
epithelium [3,9]. It should be done in all evaluations of ocular trauma,
foreign body, or suspected infection (including conjunctivitis, because symp-
toms of herpetic keratitis can occasionally mimic conjunctivitis) [10]. The
only absolute contraindication to the procedure is a flourescein allergy.
Sodium flourescein is a water-soluble dye that flouresces in an alkaline
environment (such as that encountered below the corneal epithileum), and
Fig. 1. Positioning for examination of the anterior chamber. Note the following: forehead is
resting on the forehead bar; the patient’s eyes rest at the level of the black marker on the vertical
pole next to the patients face; two strips of light are visible, one at the cornea, the second at the
iris with a clear anterior chamber.
not in the acidic environment (such as the tear film) [10]. Therefore, when
instilled into the eye, defects in the corneal epithelium will show up as bright
green when viewed under a blue light (on the slit lamp, or with a Wood’s
lamp). Deep corneal defects may allow penetration of flourescein into the
anterior chamber. Although this is not a contraindication to the examina-
tion (the substance is nontoxic), it may cause a ‘‘flare’’ appearance on exam-
ination of the anterior chamber [10].
To perform the flourescein examination, a blue light source is first passed
over the eye. Certain infectious organisms (eg, some species of Pseudomonas
bacteria) may fluoresce under a blue light even without flourescein. Then an
individually wrapped flouroscein paper is moistened with sterile water or the
patient’s own tear film. Many practitioners use topical anesthetic to wet the
strip. This is not recommended by some authors, because the anesthetic so-
lution may cause some patients to develop a superficial punctate keratitis,
thereby confounding the examination [10]; however, in patients who have
significant pain or blepharospasm and are refusing to open the eye, the
use of topical anesthetic is advised [10]. The strip is then touched to the
tear well, and removed. The patient is asked to blink several times to distrib-
ute the dye in a thin film over the entire surface of the eye. Too much dye
can impair the examination by causing the eye to become flooded with flour-
escein. The eye is then inspected with a blue light source, and areas of green
fluorescence, indicating corneal disruption, are noted [9].
A variation of this standard flourescein examination, the Seidel test, is
used to test for a globe injury or full-thickness corneal disruption. To
perform the test, a large amount of flourescein is instilled into the eye
(preferably over the area of suspected perforation), which makes the eye
appear orange. The globe is then examined for a dark stream interrupting
the flourescein, indicating leakage of aqueous humor, and suggesting the
diagnosis of globe rupture [10,11].
Note that in the past, bottles containing flourescein dye were used
directly to instill the solution into the eye, but this practice is now discour-
aged because of the possibility of these solutions becoming contaminated
with bacterial flora, particularly Pseudomonas [9,10].
Tonometry
Tonometry is the determination of intraocular pressure (IOP). Elevated
intraocular pressures are associated with vision loss. The main indications
of the procedure are to confirm a clinical diagnosis of acute angle closure
glaucoma and to determine a baseline IOP after a blunt eye injury [10]. It
can also be used to determine baseline IOPs in patients who have conditions
such as iritis, or risk factors for open angle glaucoma [10].
With any method of tonometry, an intraocular pressure of 12 to 20
mmHg is considered normal. With an intraocular pressure of greater than
30 mmHg, especially in the context of abnormal optic disks or with visual
field defects, therapy for glaucoma should be promptly initiated, and oph-
thalmologic consultation is warranted. IOPs between 20 and 30 mmHg
require urgent follow-up with ophthalmology [9].
Tonometry should be avoided in suspected penetrating eye injuries,
because it can worsen the defect and lead to extrusion of intraocular contents.
Other relative contraindications to tonometry include eye infection (unless
using a sterile cover), patients who have corneal defects, or patients who
cannot remain relaxed and may induce corneal injury with sudden, rapid
movements [9,10]. Corneal abrasions are rarely caused by tonometry [9].
There are several distinct methods of tonometry. The simplest is direct
palpation of the globe. In this method, patients are instructed to look down-
ward, but keep the eyes open. The clinician’s index finger is used to gently
palpate the globe through the eyelid, with just enough pressure applied to
indent the globe slightly. This procedure is repeated on the unaffected eye
as well, and the examiner qualitatively compares the amount of pressure
required to indent each eye [10]. One study, comparing digital palpation
with Goldmann tonometry, found digital palpation to be insensitive,
accurately identifying only five of seven eyes that had an IOP greater than
30 mmHg [12]. In clinical practice, it may be more useful as a quick screen-
ing tool to test for asymmetric intraocular pressures between the two eyes.
Impression tonometry is the most common method used in most emer-
gency departments. Impression tonometry measures intraocular pressure
by measuring the amount of corneal indentation produced by a certain
amount of force [13]. The two types of impression tonometry typically
used by emergency physicians are Schiotz tonometry and a portable hand-
held instrument that provides readings that correlate closely with Goldmann
tonometry.
With Schiotz tonometry, the first step is to ensure that the tonometer is
functioning properly. Most Schiotz tonometers come with a metal test
button in the kit. The tonometer can be lowered onto the test button to ver-
ify that the plunger is freely mobile with various amounts of pressure. If not,
it can lead to falsely low readings, and should be cleaned with an alcohol
solution [9]. To begin, the patient is placed in a supine position and asked
to gaze straight ahead at a distant object. Topical anesthetic is instilled
into the eyes. The clinician then separates the eyelids, taking care to avoid
exerting manual pressure on the globe while doing so (this could lead to
a falsely elevated reading). The tonometer is held in the patient’s line of
sight, and the patient is instructed that the procedure will not be painful
(anxiety can cause tension in extra-ocular muscles and elevate the IOP)
[10]. The tonometer foot is lowered onto the cornea, held loosely between
the examiner’s index finger and thumb. With the tonometer vertical, the
clinician reads the number from the scale on the top of the tonometer. If
the reading is low (!4 or !5), additional weight should be added to the
scale and the above procedure repeated [9]. The reading is compared with
a conversion table (based on the scale weight), thus yielding an intraocular
pressure reading. This is compared between both eyes.
The other type of impression tonometry frequently used in the emergency
department is the handheld digital device, which is used is used in a similar
fashion to the Schiotz tonometer. Although somewhat less accurate, espe-
cially at high IOPs, its measurements have good interexaminer reliability,
and it can be used with the patient in any position [10]. The pen must be cali-
brated at least once per day (see package insert for instructions on calibra-
tion). To use the device, the eye is instilled with topical anesthetic, and the
patient is asked to relax and maintain his gaze fixed on a distant object. A
fresh latex cover is placed over the end of the pen. The device is held in the
same manner as a pen, and touched lightly four times to the cornea at a per-
pendicular angle. With each touch, the probe records an IOP. After the
fourth touch, a final beep sounds and the liquid crystal display (LCD) screen
displays the average IOP measurement in mmHg, along with a series of
dashes. The dashes correspond to the statistical variance of the four
measurements (with more dashes indicated a higher variance between the
individual measurements). A single dash indicates that all measurements
were within 20% of each other. If four dashes appear, it indicates that
too few valid readings were obtained, and the process should be repeated
[10]. The probe may need to be recalibrated if this problem continues.
Applanation tonometry is a very accurate method of tonometry that is
less frequently used in the emergency department setting. This device is typ-
ically found attached to the slit lamp, and is performed as a part of the slit
lamp examination. The patient is arranged in the slit lamp apparatus as
described previously, and topical anesthesia and flourescein dye are instilled.
The cobalt blue filter is selected and the aperture set so the beam is fully
open (as would be done for an evaluation of corneal abrasion) [10]. The
illumination apparatus is angled at 45 to 60 , and directed such that it
shines through the applanation prism. The pressure knob of the tonometer
is turned to its lowest setting (10 mmHg), so that when corneal contact is
placed, there will be only light pressure. The instrument is brought toward
the patient’s eye with the joystick control. Once contact is made with the
cornea, a diffuse bluish glow will be visible throughout the limbus. When
the operator looks through the ocular pieces, he will see two blue semicircles
bordered by an arc of green light [10]. The semicircles may pulse in syn-
chrony with the patient’s heartbeat. The pressure knob is adjusted such
that the ends of the semicircles are in contact and aligned. If they overlap
it indicates the tonometer pressure is too high. If they are not in contact,
it indicates that the pressure is too low (Fig. 2) [10].
There are several sources of error with applanation tonometry. Accom-
modation, Valsalva maneuver, and vertical gaze can cause large errors in
readings [14]. Furthermore, repeated measurements can cause a false under-
estimation of intraocular pressure. Other sources of error are similar to
those in the above-described methods, with the important exception that
ocular rigidity does not affect the measurements [10].
Other methods of tonometry, such as the McKay-Marg method and
pneumatic tonometry, are less frequently used, and discussion of these
methods can be found elsewhere in this issue.
Lid eversion
Lid eversion is a simple but essential task for the emergency practitioner
to be familiar with. It is useful primarily for inspecting the tarsal conjunctiva
and fornices, as well as to localize a foreign body located in one of these
areas.
To evert the upper lid, the patient is instructed to look downwards. The
clinician grasps the eyelids at the lid margin with the thumb and index
finger. The upper lid is gently pulled downward and outward, and a cot-
ton-tipped applicator is used to press on the superior aspect of the tarsal
plate, over the lid. The examiner then turns this lid rapidly outward and
upward over the cotton swab. The lashes can be held against the superior
orbital rim to keep the lid everted while the adnexal structures are inspected
[10]. To return the lid to normal position, the examiner releases his grip on
the lid margin, and the patient is simply instructed to look upward [9].
A B C
Fig. 2. Schematic representation of visualized semicircles while using applanation tonometer.

(A) Alignment indicating correct pressure reading. (B) Pressure reading is too high. (C) Pressure
reading is too low.
The lower eyelid is smaller, and lacks a tarsal plate, and therefore is not
suitable to eversion; however, full inspection of the fornices and deep
adnexae can be readily accomplished via directly pulling at the base of the
eyelid.
Foreign body removal

Ocular foreign bodies represent a high-risk chief complaint because they
can be vision-threatening in both the short and long term. The two tasks
that face the emergency clinician in this situation are: (1) to determine if
the foreign body is superficial or intraocular, and (2) to remove superficial
foreign bodies.
Intraocular foreign bodies (IOFBs) are diagnosed through a variety of mo-
dalities. First, gross inspection is performed, looking for features that suggest
perforation, such as prolapse of intraocular contents, changes in pupillary
anatomy, or conjunctival edema [15]. Lid eversion is performed to assess
for forniceal foreign bodies. The slit lamp examination may reveal a foreign
body in the anterior chamber (w15% of IOFBs) [15], and the entire surface
of the eye should be inspected, with and without flourescein [10]. Posterior for-
eign bodies are more difficult for the emergency physician to assess by direct
visualization; however, this may be the only opportunity to do so before the
foreign body is obscured by vitreal hemorrhage or cataract formation [15].
The practice of pupillary dilation is currently controversial; although it is
helpful in determining location of IOFBs, it may compound problems (ie, if
it pulls out iris that is plugging a corneal laceration) [15].
If an IOFB is suspected based on the examination, ophthalmology should
be consulted immediately. The priority of the emergency provider is then to
prevent further globe injury. The patient should be kept quiet, with the head
of the bed elevated, and a shield should be placed over the eye (taking care
to make sure pressure points are directed onto the orbits, and not the globe).
Tonometry or other procedures that place pressure on the eye should be
avoided [10]. In these patients, systemic antibiotics should be given, as
should antiemetics (to prevent increased intraocular pressures from
vomiting) [15].
When the initial ophthalmologic examination is complete, further imag-
ing is often necessary. It is important that the emergency provider does not
stop the evaluation after a corneal foreign body or single IOFB is discov-
ered, or he may overlook the presence of multiple foreign bodies. Histori-
cally, plain radiographs (anteroposterior and lateral orbital views) have
been used to look for radiopaque foreign bodies, with precise localization
performed by using various radiographic markers (ie, Comberg’s method
or Sweet’s method) [15]. Both of these have been shown to have significant
inaccuracy, and to miss radiolucent or small foreign bodies. Ultrasonogra-
phy (US) is an increasingly popular modality, because of its immediate
availability for emergency physicians familiar with the practice (see below
for a complete discussion of this topic). At present, CT scans are considered

the gold standard, and have the highest accuracy in diagnosis and localiza-
tion [16]. The physician should request 3 mm sections through the orbit,
unless a foreign body was seen on plain radiography, in which case 6 mm
sections are acceptable [17]. MRI is not considered a first-line diagnostic
modality, because it is contraindicated in the case of known or suspected
metallic IOFB (70%–90% of cases) [15], and is typically more difficult to
obtain than the other studies mentioned above.
If a foreign body has been found to penetrate the globe through using any
of these modalities, emergent ophthalmologic consultation is indicated. The
emergency physician should take care to note visual acuity, lens damage,
presence of vitreal hemorrhage, type of IOFB, and entry site, because these
are shown to be the most important indicators of final visual outcome [18].
If an uncomplicated (ie, not intraocular, multiple, or deeply embedded)
ocular foreign body is discovered, the emergency physician should attempt
to remove it. The exception is when the patient is uncooperative, in which
case ophthalmology should be consulted to remove it [10].
To remove the foreign body, the eye is anesthetized with topical anes-
thetic, and a slit lamp examination is performed. The patient is asked to
move the eye to a position such that the foreign body is rotated to the
highest point of the cornea [9]. Successful removal can often be achieved
by gentle jet stream irrigation [10], which may be especially useful if there
is more than one foreign body [11]. Another method is to use a cotton-
tipped applicator that has been moistened with topical anesthetic [15].
This can be rolled over the foreign body to gently lift the object off of the
ocular surface. If these less invasive methods are unsuccessful, needle
removal is often required. A 25-gauge needle is placed on a tuberculin
syringe. The foreign body is approached tangentially to the globe (viewed
through the slit lamp), and the beveled edge is used to gently scrape it off
of the cornea [15]. Although this procedure may inspire some degree of anx-
iety in both the clinician and the patient, the corneal epithelium is resilient
and should not be perforated with careful manipulation. Many practitioners
now recommend avoidance of the ‘‘spud’’ device, because there may be
a risk of increased corneal scarring [9,15].
Multiple corneal foreign bodies can be removed with the same methods,
though may be more amenable to jet stream irrigation [11]. If there are dif-
fusely located foreign bodies (ie, from an explosion), however, the patient
should be referred to ophthalmology, where most of the corneal epithelium
will be removed to decrease the scarring. Another special situation involves
rust rings. These can often be removed with a needle (as described above) or
with a pressure sensitive drill, but are not a true emergency [10]. Many will
wash out spontaneously, and the others can be referred nonemergently to
ophthalmology for removal [15]. During the next 24 to 48 hours after forma-
tion, the iron will often necrose the surrounding epithelial cells and allow the
ring to then be removed intact [10]. Delayed removal may be especially
indicated in the case of a rust ring that is deep or located in the center of the
visual field, because this may allow time for the rust to migrate to the
corneal surface, thus facilitating extraction [11].
Once the foreign body has been removed, the eye should be irrigated
profusely. Studies have shown a lack of consensus among providers regard-
ing further management of these injuries [19]. Topical nonsteroidal anti-
inflammatory drugs (NSAIDs) have been shown to provide effective analgesia
in several randomized clinical trials [20,21], and should be prescribed, with
or without systemic analgesics (NSAID or opioid). Some authors rec-
ommend using a cycloplegic for pain caused by ciliary spasm, although this
practice is not routine [11,15,19,22]. Artificial tears may help with superficial
irritation [11]. Topical antibiotics and a tetanus booster may be given,
although both of these practices are controversial with superficial injuries
[9,10,23]. One prospective study where removed corneal foreign bodies
were cultured showed that 32.7% grew positive cultures, mostly staphylo-
coccal and streptococcal species [24]. Pseudomonas species are more
common in patients who wear contact lenses [25], therefore mandating treat-
ment with a quinolone antibiotic. Patching is not generally recommended,
because studies have shown no significant improvement in patient comfort
or healing rate [26,27]. Topical steroids should be avoided, because they
may promote fungal ulceration. Topical anesthetics should also be avoided,
because these agents may hide pain associated with retained foreign body
or corneal ulceration [28].
Patients who have only superficial injuries do not need ophthalmology
follow-up unless symptoms persist [10]. Patients who have large or central
defects, purulent discharge, or anterior chamber reactions, but without
globe penetration, can be discharged with 24-hour follow-up with ophthal-
mology for repeat examination and evaluation of infection [9,11].
Contact lens removal

The removal of contact lenses is a simple procedure that emergency phy-
sicians should be comfortable with. The patient should be asked whether he
wears ‘‘soft’’ or ‘‘hard’’ lenses, because this may affect the method of re-
moval. The first task is to localize the contact lens. Often the patient may
not be able to sense the lens if it is in a conjunctival fornix. The patient is
examined in the standard fashion. The upper lid is everted. If the patient
has significant pain or blepharospasm, the eye should be topically anesthe-
tized. Note that flourescein dye can be used to help localize the lens,
although it will permanently stain a soft lens.
When the lens is encountered, gentle pressure should be applied with
a cotton-tipped applicator or a gloved finger [29]. If it is loose, it may be
slid over the cornea, and the patient can remove it as usual [29]. A soft
lens can be safely removed from any part of the eye. If the lens is somewhat
adherent, gentle irrigation with a plastic catheter may moisten it and allow it
to be moved to a position where it can be easily removed from the eye [30]. If
it continues to be adherent, the practitioner should attempt to remove the
lens directly by gently compressing it between a gloved thumb and forefinger
to break the suction with the globe, then remove it.
If these steps fail, there are devices available that can help in removal. For
soft lenses, rubber-tipped forceps can be used to squeeze the lens and allow
removal [29]. For hard lenses, suction-cup removers can be used. The suc-
tion cup should be moistened with saline, and then applied gently directly
to the front of the lens [30]. Once a seal is formed, the lens should be pulled
off perpendicularly to the globe.
After removal, a flourescein examination should be performed to evalu-
ate for the presence of corneal abrasions. These should be treated as any
other corneal abrasion, with the caveat that if topical antibiotics are
prescribed, it should be a quinolone (rather than a macrolide) to cover
Pseudomonas and other organisms more common in contact-lens wearers
[25,30]. The patient should be instructed not to wear the lens for at least
24 hours, or until free of any symptoms [29].
Eye irrigation
The foundation of emergency treatment for chemical burns is immediate
and copious irrigation, because this has been shown to have the greatest
effect on visual prognosis [31].
There are two broad types of chemical burn: acid and alkali. Acid burns
produce a coagulative necrosis, and therefore, after the initial injury, the
deeper structures may be somewhat protected from further injury (with
the notable exception of hydrofluoric acid burns). Alkali burns produce
a liquefactive necrosis and penetrate more rapidly into the anterior chamber
(within minutes) [32].
Despite these differences, the treatment of ocular chemical burns is the
same. When a patient presents with an ocular burn, irrigation should begin
immediately, even in triage [33], especially because in practice, immediate,
on-site irrigation occurs only in 40% to 50% of patients [34]. Physical
examination should initially be limited to, at most, a very rapid visual acuity
measurement and the determination of pH [3].
Measurement of ocular surface pH is a simple task. One simply touches
the end of a piece of pH litmus paper to the tear well and compares the
resulting color to the color scale located on the side of the container that
holds the strips. The shelf life of the strips is roughly 5 years according to
the manufacturer. There are several methods of irrigating the eye. Eye
showers are available at many workplaces, but the actual amount of irrigant
they deliver to the corneal surface and conjunctival fornices may be limited
by blepharospasm caused by pain. In the emergency department, the ideal
device to use is a Morgan lens. The Morgan lens is a molded lens that is
applied directly to the eye surface, and can be directly attached to a length
of intravenous (IV) tubing to allow continuous irrigation of the eye. To

insert the lens, the patient should be instructed to look down, as the upper
lid is grasped and the superior end of the lens is placed under the lid. This
procedure is repeated with the lower lid. At this point, the lens is connected
to the tubing from the irrigant, and the solution is allowed to bathe the eye.
If the Morgan lens is not available, the eye can be directly irrigated with the
cut end of IV tubing. This often requires an assistant to manually retract the
eyelids throughout the procedure, however; otherwise, blepharospasm is
likely to limit the amount of irrigant that reaches the corneal surface [15].
The irrigant is instilled until the pH of the eye returns to normal (pH 6.8–
7.4) [9]. After irrigation, the practitioner should wait for 5 minutes before
checking the pH, because earlier measurements may give a falsely reassuring
pH by measuring the pH of the irrigant solution [11]. After achieving a nor-
mal pH, the eye should be continually irrigated for at least 30 more minutes
[34]. Some authors recommend episodic irrigation for up to the following
24 hours [34].
There may be controversy regarding the specific type of irrigant to be
used. Whereas different features make some solutions preferable to others,
the most important factor is rapid institution of therapy, and it is essential
to not delay irrigation while setting up the equipment or finding the best
buffer solution [9]. Irrigation works by mechanical and chemical means. Me-
chanically, a volume of liquid coursing over the eye serves to remove any
excess particles or drops of the offending agent. In this way, even tap water
or other nonsterile fluid is a suitable irrigant if used immediately [11,34].
The preferred buffer solutions are amphoteric (universal) buffers that
may help to limit further damage to the eye caused by exothermic chemical
reactions between the irrigant and the offending agent [9]. Ethylenediamine-
tetraacetic acid (EDTA) used to be the primary amphoteric buffer, but has
now been largely replaced by diphoterine [34,35]. Notably, acidic solutions
should not be used to neutralize an alkaline burn, and alkaline solutions
should not be used to neutralize an acidic burn [11]. Another consideration
in use of buffers is the osmolarity. Because the cornea has a higher osmolar-
ity (420 mOsm/L) than other areas in the human body, use of a hypotonic
solution can lead to increased corneal water uptake, and therefore, exacer-
bate corneal edema from a burn [34,36]. This will also allow deeper corneal
penetration of corrosive materials [34]. Therefore, osmotic solutionsdnor-
mal saline (NS), lactated Ringer’s solution (LR), or balanced salt solution
(BSS)dare often preferred to nonosmotic solutions such as tap water.
The pH of the solution is also a factor in the choice of irrigant. Normal
saline has a pH of 4.5 to 7.0, lactated Ringer’s is 6.2 to 7.5, and balanced
salt solution is 7.1 to 7.4 [37]. For all of these reasons, many authors prefer
BSS or LR as an irrigant, although isotonic NS is considered to be an
appropriate substitute if others are unavailable [32], and even tap water
irrigation has been shown to decrease the severity of ocular burns [36,38].
Each of these solutions has been shown to have equal tolerability to patients
[39], although warmed solutions have been shown to be better tolerated than
room temperature [40].
Following adequate irrigation, it is important to do a complete ophthal-
mologic examination to rule out any retained foreign bodies, corneal
injuries, or other injuries that need attention [9,11]. Some authors recom-
mend sweeping the fornices with a moistened cotton-tipped applicator to
remove any remaining particles of caustic material or necrotic conjunctiva.
All patients, even those who have minor burns, should receive a tetanus
shot, topical antibiotics, and oral analgesics, and be instructed to follow
up with ophthalmology within 24 hours. Cycloplegics and pressure patches
are controversial. For more severe burns, and especially those caused from
alkali exposure, hospitalization may be warranted for intravenous antibi-
otics and further ophthalmologic care. In these cases, ophthalmologic
consultation is required before institution of therapy such as cycloplegics,
topical steroids, and further therapy. Prognosis depends on the depth of
the injury, with opacification of the cornea and greater than 50% area of
conjunctival ischemia being predictive of a poor outcome (Table 2).
Paracentesis
Anterior chamber paracentesis is a rare procedure in the emergency
department; however, it is one that emergency providers should feel com-
fortable performing. The primary indication in the emergency setting is in
the treatment of central retinal artery occlusion. In this disease, there is
acute embolization to the central retinal artery, which is manifested by
a painless loss of vision in the affected eye, and appears as a pale optic
disc or a cherry red spot on fundoscopy. Immediate treatment is indicated
if the patient presents within 24 to 48 hours of symptom onset, because
once the retina has infarcted, vision loss is permanent [13]. Even with appro-
priate treatment, successful return of full visual acuity is rare [13].
Initial treatment is directed at improving retinal blood flow and trying to
dislodge the clot into a more distal branch of the retinal artery. Common ma-
neuvers include supine positioning (which improves retinal blood flow),
Table 2
Classification and prognosis of chemical ocular burns
Grade Corneal findings Conjunctival ischemia Prognosis
I Only epithelial loss. None Very good
II Some edema and haze. !1/3 of limbus Some permanent scarring
may occur.
III Significant haziness !1/2 limbus Variable, usually some
visual impairment
IV Opaque O1/2 limbus Poor
Data from Cheh AI, Reensta-Buras WR. Ocular. http://www.emedicine.com/emerg/topic736.
htm. Accessed August 3, 2007.
digital orbital massage (to diminish intraocular pressure and possibly dislodge
the clot), and intravenous acetozolamide or topical B-blockers to further de-
crease intraocular pressure [41]. Carbogen therapy involves inhalation of
a 5% CO2/95% O2 mixture of gas. The carbon dioxide may dilate retinal ar-
terioles, thereby enhancing the delivery of oxygen-rich blood to the ischemic
retina [42]. This should be done for 10 minutes every 2 hours, and continued
for 48 hours. Hyperbaric oxygen therapy may provide similar benefits, but
must be initiated soon after symptom onset (within 12 hours, and preferably
within 2 hours) [42]. If these techniques fail to restore vision or are unavail-
able, however, anterior chamber paracentesis should be attempted.
To perform the procedure, the eye is topically anesthetized. The provider
prepares a 30-gauge needle on a tuberculin syringe. The patient is positioned
in the slit lamp apparatus as described above. The anterior chamber is care-
fully entered at the limbus, with the bevel facing the examiner. The clinician
then removes 0.1 to 0.2 cc of aqueous, until the chamber becomes slightly
shallower. The needle is withdrawn and the patient should then be given
a topical antibiotic [41].
Lateral canthotomy
Lateral canthotomy is another rarely indicated but potentially vision-
sparing procedure that emergency practitioners must be familiar with. The
purpose of the procedure is to emergently relieve retro-orbital pressure after
trauma. With significant retrobulbar hemorrhage, the optic nerve can
become ischemic, leading to permanent visual loss within 90 to 120 minutes
of ischemic time [43]. Although retrobulbar hemorrhage is now often diag-
nosed based on CT scan findings in the trauma victim, signs of retrobulbar
hemorrhage include acute loss of visual acuity, elevated IOP (O40 mm Hg),
and proptosis [43]. There are other more subtle findings suggestive of the
diagnosis, including afferent pupillary defect, optic nerve pallor, and severe
eye pain [43], but these findings alone should not merit canthotomy without
confirmatory findings.
To perform the procedure, the patient is placed in the supine position,
and 1 to 2 cc of 1% lidocaine with epinephrine is injected into the lateral
canthus. The eye is then irrigated with normal saline to ensure a clean,
noncontaminated working surface. A straight Kelly clamp is used to crimp
the skin at the lateral canthus, then promptly removed. This serves two func-
tions: it helps with hemostasis, and it marks the location for the canthot-
omy. At this point, an assistant may be useful to laterally retract the lid,
thereby reducing the risk of inadvertent globe injury. Small scissors are
used to make a 1 to 2 cm lateral incision along the line formed by the
crimped Kelly clamp. The lower eyelid is then retracted to exposure the
lateral canthus tendon, which can then be cut with the scissors. A small
amount of blood should be expressed at this time. The intraocular pressure
should be less than 40 mm Hg if the procedure is successful, and the patient
should have improved visual acuity. If the pressure continues to remain

elevated, the superior canthus can be cut in the same way [43].
Ocular ultrasonography
Bedside US, performed by emergency physicians, is a procedure that is
gaining popularity in the emergency department. It can be a rapid, safe,
and noninvasive method to make several diagnoses, and may be especially
useful in the trauma patient where periorbital swelling limits direct visuali-
zation of the eye and surrounding structures [1]. It is also useful in nontrau-
matic eye complaints, because patients who have new-onset visual loss can
be rapidly evaluated for retinal detachment or central retinal artery or vein
occlusion (Table 3) [1].
To perform ocular US, a linear 10-mHz transducer (also known as
a ‘‘vascular’’ probe) is used. The power output and gain settings should
be minimized, because this decreases artifact from eyelid echoes and allows
better visualization of ocular structures [44]. The patient is asked to close his
eyes, and ultrasound gel is applied to the closed eye. The clinician should
take care to avoid putting excessive pressure on the globe while performing
the scan, and if there is a possibility of globe rupture, the scan should be per-
formed without the transducer touching the eyelid (instead using a larger
amount of ultrasound gel) [44]. The scan is done in both sagittal and trans-
verse planes, as well as with two-dimensional Doppler imagining if there is
suspicion of central retinal artery or vein occlusion [1,44]. The imaging can
typically done by an experienced sonographer within 60 to 90 seconds [1].
There are few prospective studies regarding the role of ocular US in the
emergency department. In one study by Blaivas and colleagues [1], 61 con-
secutive patients who had ocular complaints were enrolled, all of whom had
bedside ocular US followed by a ‘‘confirmatory’’ study (either CT of orbits
or formal ophthalmologic examination). The scans were done by experi-
enced attending physicians or residents who had undergone a 1-hour lecture
Table 3
Ocular pathology visible with ultrasonography, and sonographic findings
Pathology Ultrasonographic appearance
Retinal detachment Band of echogenic material within vitreous body
Vitreous detachment Collection of echogenic material between
vitreous body and retina
Vitreous hemorrhage Echogenic material within vitreous body
Retrobulbar hemorrhage Hypoechoic lucency deep to retina
Lens dislocation Lens in abnormal position within globe
Globe rupture Difference in globe size between eyes; scleral folds
Intraocular foreign body Echogenic foreign body visualized within globe
Central retinal artery/vein occlusion Absence of Doppler flow at posterior retina
Data from Legome E, Pancu D. Future applications for emergency ultrasound. Emerg Med
Clin N Am 2004;22:817–27.
and a 1-hour hands-on teaching experience. Of these 61 patients, the correct

diagnosis was made by bedside US in 60, yielding test characteristics of:
sensitivity 100% (95% CI 94–100), specificity 97% (95% CI 88–99), positive
predictive value (PPV) 96% (95% CI 88–99), negative predictive value
(NPV) 100% (95% CI 94–100) [1]. There have been no studies that look
at how bedside ocular US changes decision-making regarding treatment,
further diagnostic studies, or disposition [1,44].
Another novel use of bedside ocular US is in the evaluation of the optic
nerve sheath diameter (ONSD) in a patient who has possible increased intra-
cranial pressure (ICP). The scan is performed as above, and the ONSD is
measured in each eye at a distance of 3 mm behind the globe. The ONSD
measurement is then an average of both eyes [45]. An increased ONSD
(O5.0 mm in adults, O4.5 mm in children age 1–15years, or O4.0 mm in
children less than 1 year old) is considered abnormal and indicative of
elevated intracranial pressure [45–47]. One study [46] has shown abnormal
ONSD to have a 100% sensitivity (95% CI 68–100) for CT findings consis-
tent with increased ICP, and a specificity of 63% (95% CI 50–76). Further-
more, this had a sensitivity of 84% and specificity of 73% for any traumatic
intracranial injury [46].
Summary
Ophthalmologic complaints are a small but important proportion of
emergency department visits. Familiarity with many important ophthalmo-
logic procedures is an essential part of any emergency clinician’s armamen-
tarium, and may help to decrease morbidity associated with ophthalmologic
emergencies.
References
[1] Blaivas M, Theodoro D, Sierzenski PR. A study of bedside ocular ultrasonography in the
emergency department. Acad Emerg Med 2002;9(8):791–9.
[2] Kercheval DB, Terry JE. Essentials of slit lamp biomicroscopy. J Am Optom Assoc 1997;
48(11):1383–9.
[3] Broocker G. Chapter 15: the ophthalmic examination. In: Wolfson AB, Hendey GW,
Hendry PL, et al, editors. Harwood-Nuss’ clinical practice of emergency medicine. 4th
edition. Philadelphia: Lippincott, Williams and Wilkins; 2005. p. 112–7.
[4] Available at: http://www.academy.org.uk/lectures/eperjesi5.htm. Accessed August 2, 2007.
[5] Nemeth SC. Basic slit lamp techniques. J Ophthalmic Nurs Technol 1996;15(4):134–41.
[6] Blumenthal EZ, Serpetopolous CN. On focusing the slit lamp: part I. an inaccurate ocular
settingdwhat is there to lose? Surv Ophthalmol 1998;42(4):351–4.
[7] Blumenthal EZ, Serpetopolous CN. On focusing the slit lamp: part II. ‘‘The fading-slit
test’’dverifying the ocular setting. Surv Ophthalmol 1998;42(4):355–7.
[8] Osuobeni EP, Oduwaiye KA. The effect of illumination-microscope angle on slit lamp
estimate of the anterior chamber depth. Optom Vis Sci 2003;80(3):237–44.
[9] Dennis WR Jr, Dennis AM. Chapter 31: Eye emergencies. In: Stone CK, Humphries EH,
editors. Current emergency diagnosis and treatment. 5th editon. Lange Medical Books/
McGraw-Hill; 2004.
[10] Knoop KJ, Dennis WR, Hedges JR. Opthalmologic procedures. In: Roberts MD, Hedges
JR, editors. Clinical procedures in emergency medicine. 4th edition.
[11] Cullom Jr RD, Chang B, editors. The Wills eye manual: office and emergency room diagnosis
and treatment of eye disease. 2nd edition. 1994.
[12] Baum J, Chaturvedi N, Netland PA, et al. Assessment of intraocular pressure by palpation.
Am J Ophthalmol 1995;119(5):650–1.
[13] Beers MH, Porter RS, Jones, TV, et al, editors. The Merck manual of diagnosis and
therapydchapter 98: Approach to the ophthalmologic patient. 18th edition. 2006.
[14] Whitacre MM, Stein R. Sources of error with use of Goldmann-type tonometers. Surv
Ophthalmol 1993;38(1):1–30.
[15] Schwartz GR, et al. Ocular foreign body removal. In: Principles & practice of emergency
medicine. 4th edition. Copyright (c) 1999 Lippincott Williams & Wilkins.
[16] Coleman DJ, Lucas BC, Rondeau MJ, et al. Management of intraocular foreign bodies.
Ophthalmology 1987;94:1647–53.
[17] Etherington RJ, Hourihan MD. Localisation of intraocular and intraorbital foreign bodies
using computerized tomography. Clin Radiol 1989;40:610–4.
[18] De Juan E Jr, Sternberg P Jr, Michels RG. Penetrating ocular injuries: types of injuries and
visual results. Ophthalmology 1983;90:1318–22.
[19] Calder L, Baladubramanian S, Stiell I. Lack of consensus on corneal abrasion management:
results of a national survey. CJEM 2004;6(6):402–7.
[20] Donnenfeld ED, Selkin BA, Perry HD, et al. Controlled evaluation of a bandage contact
lens and a topical non-steroidal anti-inflammatory drug in treating corneal abrasions.
[21] Weaver CS, Terrell KM. Evidence-based emergency medicine. Update: do ophthalmic non-
steroidal anti-inflammatory drugs reduce the pain associated with simple corneal abrasion
without delaying healing? Ann Emerg Med 2003;41(1):134–40.
[22] Elkington AR, Khaw PT. ABC of eyes. Injuries to the eye. BMJ 1988;297(6641):122–5.
[23] Mukherjee P, Sivakumar A. Tetanus prophylaxis in superficial corneal abrasions. Emerg
Med J 2003;20(1):62–4.
[24] Macedo Filho ET, Lago A, Duarte K, et al. Superficial corneal foreign body: laboratory and
epidemiologic aspects. Arq Bras Oftalmol 2005;68(6):821–3.
[25] Fleiszig SM, Efron N, Pier GB. Extended contact lens wear enhances Pseudomonas aerugi-
nosa adherence to human corneal epithelium. Invest Ophthalmol Vis Sci 1992;33(1):2908–16.
[26] Arbour JD, Brunette I, Boisjoly HM, et al. Should we patch corneal erosions? Arch Ophthal-
mol 1997;115:313–7.
[27] Wilson SA, Last A. Management of corneal abrasions. Am Fam Physician 2004;70(1):123–8.
[28] Newell SW. Management of corneal foreign bodies. Am Fam Physician 1985;31(2):149–56.
[29] Buttaravoli P, Stair T. Common simple emergencies. 2.12–Removal of dislocated contact
lens. Available at: http://www.ncemi.org/cse/cse0212.htm. Accessed August 5, 2007.
[30] Ramponi DR. Eye on contact lens removal. Available at: http://findarticles.com/p/articles/
mi_qa3689/is_200108/ai_n8985396. Accessed August 5, 2007.
[31] Kucklkorn R, Kottel A, Schrage N, et al. Poor prognosis of severe chemical and thermal eye
burns: the need for adequate emergency care and primary prevention. Int Arch Occup
Environ Health 1995;67(4):281–4.
[32] Cheh AI, Reensta-Buras WR, and Rosen C. Burns, ocular. Available at: http://www.
emedicine.com/emerg/topic736.htm.
[33] Garcia GE. Management of ocular emergencies and urgent eye problems. Am Fam
Physician 1996;53(2):565–74.
[34] Schrage, et al. Eye burns. Burn 2000;26:699.
[35] Langefeld S, Press UP, Frentz M, et al. Use of lavage fluid containing diphoterine for irriga-
tion of eyes in first aid emergency treatment. Ophthalmologe 2003;100(9):727–31 [Article in
German].
[36] Kompa S, Redbrake C, Hilgers C, et al. Effect of different irrigating solutions on aqueous
humour pH changes, intraocular pressure and histological findings after induced alkali
burns. Acta Ophthalmol Scand 2005;83(4):467–70.
[37] Saidinejad M, Burns MM. Ocular irrigant alternatives in pediatric emergency medicine.
Pediatr Emerg Care 2005;21(1):23–6.
[38] Ikeda N, Hayasaka S, Hayasaka Y, et al. Alkali burns of the eye: effect of immediate copious
irrigation with tap water on their severity. Ophthalmologica 2006;220(4):225–8.
[39] Jones JB, Schoenleber DB, Gillen JP. The tolerability of lactated Ringer’s solution and BSS
plus for ocular irrigation with and without the Morgan therapeutic lens. Acad Emerg Med
1998;5(12):1150–6.
[40] Ernst AA, Thomson T, Haynes M, et al. Warmed versus room temperature saline solution
for ocular irrigation: a randomized clinical trial. Ann Emerg Med 1998 Dec;32(6):676–9.
[41] Haddad C. Central retinal artery occlusion. Available at: http://www.eyeweb.org/CRAO.
htm. Accessed August 5, 2007.
[42] Graham RH, Huang E, Kim DB, et al. Central retinal artery occlusion. Available at: http://
www.emedicine.com/OPH/topic387.htm.
[43] Peak DA. Acute orbital compartment syndrome. Available at: http://www.emedicine.com/
emerg/topic881.htm.
[44] Legome E, Pancu D. Future applications for emergency ultrasound. Emerg Med Clin North
Am 2004;22:817–27.
[45] Blaivas M, Theodoro D, Sierzenski PR. Elevated intracranial pressure detected by emer-
gency ultrasonography of the optic nerve sheath. Acad Emerg Med 2003;10(4):376–81.
[46] Tayal VS, Neulander M, Norton HJ, et al. Emergency department sonographic measure-
ment of optice nerve sheath diameter to detect findings of increased intracranial pressure
in adult head injury patients. Ann Emerg Med 2007;49(4):508–14.
[47] Tsung JW, Blaivas M, Cooper A, et al. A rapid noninvasive method of detecting elevated
intracranial pressure using bedside ocular ultrasound: application to 3 cases of head trauma
in the pediatric emergency department. Pediatr Emerg Care 2005;21(2):94–8.
Emerg Med Clin N Am
26 (2008) 35–55
Diagnosis and Management

of the Acute Red Eye
Ahmed R. Mahmood, MD, Aneesh T. Narang, MD*
Department of Emergency Medicine, Boston Medical Center, Dowling 1 South,
The red eye is a clinical problem encountered on a daily basis in most

emergency departments. Fortunately, most causes are relatively benign
and self-limiting; however, many conditions associated with high morbidity
and that are potentially vision threatening may manifest as a red eye. The
history should address the following essential components: the presence or
absence of pain, foreign body sensation, and itching; the presence and
type of discharge; photophobia; onset; visual disturbances; recent illnesses
and trauma; and ophthalmologic history. The examination must include
visual acuity, pupil shape and reactivity, a comparison between the pupils,
the gross appearance of the sclera and conjunctiva, extraocular muscle func-
tion, and palpation for preauricular nodes. Often, evaluation of the affected
eye requires measurement of intraocular pressure, fluorescein staining and
a cobalt blue light, and a slit lamp evaluation [1].
Emergency physicians should be adept at recognizing high-risk features
from the history and examination that would require urgent ophthalmologic
referral and treatment. The differential diagnosis of the red eye is extensive.
Some of the more common causes, including viral, allergic, and bacterial
conjunctivitis, subconjunctival hemorrhage, episcleritis, scleritis, anterior
uveitis, and acute angle-closure glaucoma (AACG), are discussed herein.
Characteristic features of the history and examination as well as manage-
ment and indications for ophthalmology consultation for each of these
entities are described in detail.
Conjunctivitis
The most common cause of red eye is conjunctivitis. The term conjuncti-
vitis refers to inflammation of the conjunctiva, a membrane that lines the
E-mail address: [email protected] (A.T. Narang).
36 MAHMOOD & NARANG
outer aspect of the globe (bulbar conjunctiva) and reflects back on itself to
line the inner lids (the palpebral conjunctiva) [2]. Conjunctivitis is usually
separated into broad categories based on the etiologic agent and time course
of illness. The most common causes of acute conjunctivitis (less than 4
weeks) are allergic, viral, and bacterial. Common and distinguishing features
of the various types of acute conjunctivitis are reviewed herein.
Allergic conjunctivitis, also known as hay fever conjunctivitis or seasonal
allergic rhinoconjunctivitis, is the most common type of ocular allergy. This
IgE-mediated reaction is usually, but not always, seasonal and may be seen
with sensitivity to allergens such as dust or animal dander. The patient will
almost always present with itching and may or may not have associated
watery eyes and rhinorrhea. If there is no component of itching, allergic con-
junctivitis is less likely, and another diagnosis should be sought. The family
history often includes other forms of atopy such as asthma, eczema, or
allergic rhinitis. On examination, the clinician will notice a global bilateral
injection pattern that is equal in both eyes [1]. If there is a discharge, it
may be clear and watery, such as in tears, or mucoid. Mild eyelid swelling
may complete the clinical presentation. Similar presentations can be caused
by dry eyes, contact lenses, and over-the-counter eye products, and these
causes should be considered in the differential diagnosis [2].
As with the treatment of other forms of allergy, avoidance of triggers is
paramount. The patient may use cold compresses, over-the-counter vaso-
constrictors, or ocular non-steroidal anti-inflammatory agents (NSAIDs)
to help reduce discomfort, redness, and swelling. Oral antihistamines can
often help relieve many of the patient’s symptoms. More specific therapy
includes histamine-blocking drops such as olopatadine, pemirolast, or keto-
tifen [1]. Topical mast cell stabilizers such as cromolyn sodium or lodoxa-
mide can also be beneficial. Administering corticosteroids should only be
done in consultation with an ophthalmologist [2].
Conjunctivitis can have a variety of infectious etiologies. Viral infections
are among the most common forms of infectious conjunctivitis, with many
types implicated, including adenovirus, herpes, mumps, and rubella. Be-
cause the last two causes are rare, the more common presentations of ade-
novirus and herpes virus are discussed herein. Adenovirus is the most likely
etiologic agent of any viral conjunctivitis, with most of the common sero-
types causing a mild follicular conjunctivitis. Common modes of transmis-
sion for this viral-borne illness are the fingers, medical instruments, and
swimming pool water. As such, it is responsible for community-wide epi-
demics and is commonly found in schools, workplaces, and doctors’ offices
[2]. The patient will usually complain of irritation beginning in one eye and
spreading to the other a few days later. This spread is not uncommon as the
infection is transmitted via hand–eye contact. Some patients may have an
associated upper respiratory tract infection. Common but nonspecific find-
ings include preauricular lymphadenopathy, global conjunctival injection,
watery discharge, and a follicular reaction of the inferior tarsal conjunctiva
DIAGNOSIS AND MANAGEMENT OF THE ACUTE RED EYE 37
(Fig. 1). Follicles are tiny, avascular, round, white or gray patches on the
palpebral conjunctiva. They differ from papules, which are larger, include
a tuft of blood vessels, and resemble cobblestones [3]. Pain and photophobia
are not typically associated with most instances of adenoviral conjunctivitis.
Likewise, blurred vision that does not clear on blinking may be an indica-
tion that another diagnosis should be considered [2].
Treatment for most cases of viral conjunctivitis includes supportive care
such as artificial tears and cold compresses. Topical decongestants and top-
ical steroids (in consultation with an ophthalmologist) may be prescribed if
the ocular edema is severe. Because viral conjunctivitis is usually a benign
and self-limiting condition, there is a low likelihood of secondary bacterial
infection. Topical antibiotics such as erythromycin are not necessary; how-
ever, it is not inappropriate to prescribe antibiotics if the diagnosis is diffi-
cult to discern from bacterial conjunctivitis. Fortunately, there is little
harm in using topical antibiotics for viral conjunctivitis [4]. Adenovirus
has been found to have a 95% replication rate at 10 days, which drops to
5% at 16 days. The patient should be instructed to practice frequent hand
washing for 2 weeks and should be reminded that personal items that
may come in contact with the eyes, such as towels, should not be shared
[1]. In most cases, viral conjunctivitis can be managed on an outpatient basis
with elective referral to an ophthalmologist if there is no improvement
within 7 to 10 days [5].
Depending on the serotype of adenovirus, the clinical presentation may
include more than just a mild follicular conjunctivitis. Of the 47 adenoviral
serotypes, many have a predilection for other mucosal surfaces in addition
to the bulbar and palpebral conjunctiva. These serotypes can cause a clini-
cally significant infection in the respiratory, genitourinary, or gastrointesti-
nal tracts as well; hence, adenoviral conjunctivitis may be isolated or
a feature of a systemic viral syndrome. Two of the more common syndromes
Fig. 1. Viral conjunctivitis. Note the global injection pattern. (From Boruchoff SA. Anterior
segment disease: a diagnostic color atlas. Boston: Butterworth-Heinemann; 2001. p. 120; with
permission.)
38 MAHMOOD & NARANG
are pharyngoconjunctival fever and epidemic keratoconjunctivitis. Pharyng-

oconjunctival fever presents with an abrupt onset of high fever, pharyngitis,
and bilateral follicular conjunctivitis. It is more common in children and can
occur sporadically and in clusters. Schools during the winter and camps dur-
ing the summer are common settings for this type of infection. Treatment is
the same as for mild follicular conjunctivitis, with referral to ophthalmology
if symptoms are unremitting after 1 week. Epidemic keratoconjunctivitis is
frequently caused by serotypes 8, 19, and 37 and is associated with ocular
pain and decreased visual acuity from corneal subepithelial infiltrates
(Fig. 2). The infiltrates appear as 1- to 2-mm, grayish-white, ‘‘crumb-like’’
defects numbering up to 30 throughout the central and peripheral cornea.
Visual acuity may drop by several lines on the Snellen chart. Edema, small
petechial hemorrhages, and the formation of inflammatory pseudomem-
branes are other distinctive features of epidemic keratoconjunctivitis [3].
Treatment should consist of local care as outlined previously. Patients
should receive follow-up with an ophthalmologist in 1 week to monitor
for the development of keratitis, a complication of keratoconjunctivitis [6].
Herpes simplex virus (HSV) conjunctivitis is an example of another infec-
tion that may present as a conjunctivitis alone or as a more pervasive infec-
tion involving the cornea, eyelid, and skin. HSV conjunctivitis occurs at
a higher rate in HIV-infected patients. This infection will usually present
unilaterally and has many of the features of an adenovirus conjunctivitis,
including a watery discharge and palpable preauricular nodes. Pain, burn-
ing, and a foreign body sensation associated with HSV conjunctivitis help
distinguish it from most other forms of viral conjunctivitis. Other distin-
guishing features include episodic copious tearing and mildly decreased
vision [3]. The first-line treatment for patients who have HSV conjunctivitis
alone, without skin or corneal involvement, is cool compresses and topical
antiviral medication for 10 to 14 days. Recommended medications are tri-
fluridine 1% drops, five times per day, or vidarabine 3% ointment, five times
Fig. 2. Epidemic keratoconjunctivitis. (From Boruchoff SA. Anterior segment disease: a diag-
nostic color atlas. Boston: Butterworth-Heinemann; 2001. p. 132; with permission.)
per day. The patient should receive follow-up in 2 to 5 days to monitor for
corneal involvement [7].
If HSV conjunctivitis also involves the skin (eg, HSV dermatitis of the
eyelids) or is associated with photophobia or decreased vision, the clinician
must be more aggressive with work-up and treatment. In these circum-
stances, corneal staining should be performed. On slit lamp examination,
one may see pinpoint or dendritic lesions on the cornea using fluorescein
staining and a cobalt blue light. The lesions may be confluent and geo-
graphic, or atypical. Classic skin findings include grouped pustules or vesi-
cles on an erythematous base which progress to crusting. The patient may
have a history of similar bouts of conjunctivitis, usually unilateral, suggest-
ing remote HSV outbreaks. Stress, fever, trauma, or UV light can all trigger
reactivation. In severe cases, uveitis, iritis, and increased intraocular pres-
sure may be seen. Treatment consists of topical trifluridine 1% drops admin-
istered nine times per day and oral acyclovir, 400 mg orally five times daily
for 7 to 10 days. If there is flare in the anterior chamber on slit lamp exam-
ination, one should consider the addition of a cycloplegic agent such as
scopolamine 0.25% three times daily. The patient should undergo an oph-
thalmologic follow-up in 2 days to evaluate for response to treatment [7].
Steroids should not be prescribed for patients with HSV conjunctivitis be-
cause the risk for secondary infection and other complications from
uncontrolled viral proliferation is increased [2].
Herpes zoster (HZV) ophthalmicus occurs when the varicella-zoster virus
is reactivated in the ophthalmic division of the trigeminal nerve. This entity
represents approximately 10% to 25% of all zoster cases. Although most
cases of HZV ophthalmicus involve the skin only, serious ocular involve-
ment can occur if the infection is reactivated in the nasociliary branch of
the ophthalmic nerve (Fig. 3A, C). Herpes pustules at the tip of the nose
(Hutchinson’s sign) are thought to be a classic predictor of ocular involve-
ment. Although patients with a positive Hutchinson’s sign have twice the
incidence of ocular involvement, one third of patients without the sign
can experience ocular manifestations. A common complication of HZV
infection is an injected and edematous conjunctiva, often with petechial
hemorrhages. This conjunctivitis will usually resolve in 1 week unless sec-
ondary bacterial infection occurs. The use of topical antibiotics may help
to prevent secondary infection, whereas cool compresses and lubrication
drops can be used for comfort. In corneal involvement, HZV dendrites
appear as a branching or ‘‘medusa-like’’ pattern with tapered ends in con-
trast to HSV dendrites, which often have terminal bulbs. This pattern can
be viewed by Wood’s lamp or slit lamp examination after fluorescein stain-
ing. These patients need preservative-free artificial tears every 1 to 2 hours
and an ocular lubricant ointment nightly. An ophthalmologist should be
consulted regarding systemic or topical antiviral agents; topical steroids
are occasionally indicated depending on the ocular manifestations of HZV
and should be prescribed only in consultation with ophthalmology [8].
40 MAHMOOD & NARANG
Fig. 3. (A) Herpes zoster in the distribution of the first division of the trigeminal nerve. Note
the presence of Hutchinson’s sign, a vesicle on the tip of the nose (black arrow). (B) Herpes
zoster keratoconjunctivitis. (C) HZV dendrites have a ‘‘medusa-like’’ pattern. (From Boruchoff
SA. Anterior segment disease: a diagnostic color atlas. Boston: Butterworth-Heinemann; 2001.
p. 128–9; with permission.)
If the patient with HZV activation should have any ocular involvement,
ophthalmologic follow-up should occur within 24 hours [8].
Bacterial conjunctivitis is a condition usually caused by gram-positive
organisms, the most common being Streptococcus pneumoniae and Staphy-
lococcus aureus, and gram-negative organisms such as Haemophilus influen-
zae [1]. The first two infections occur more often in children; the last afflicts
mostly adults [2]. Bacterial conjunctivitis has a more abrupt onset than viral
conjunctivitis and is also associated with tearing and ocular irritation. In
bacterial conjunctivitis, the infection usually spreads to the contralateral
eye within 48 hours [1]. The patient may complain of morning crusting
and difficulty opening the eyelids [2]. This symptom results from a mucopur-
ulent yellow-colored discharge that causes matting of the lids and lashes
(Fig. 4). On examination, the red eye injection pattern of bacterial conjunc-
tivitis is diffuse but often more pronounced at the fornices [1].
The definitive treatment for bacterial conjunctivitis is topical ophthalmic
broad-spectrum antibiotics. Although it is usually a self-limiting disease,
treatment shortens the course, reduces person-to-person spread, and lowers
the risk of sight-threatening complications such as ulceration. Erythromycin
and bacitracin/polymyxin B provide excellent broad-spectrum coverage
against most pathogens found in adult and pediatric cases. Aminoglycosides
Fig. 4. Bacterial conjunctivitis. Note matting of the eyelashes caused by the mucoid and thick
discharge. (From Boruchoff SA. Anterior segment disease: a diagnostic color atlas. Boston:
Butterworth-Heinemann; 2001. p. 135; with permission.)
should not be used because they have relatively poor coverage of gram-
positive organisms such as staphylococcus and streptococcus species. Oint-
ment, which is less irritating, works best for children who also benefit from
less frequent application and can tolerate the associated blurred vision well.
Drops are recommended for adolescents and adults because they are easier
to apply [2]. Most immunocompetent patients with uncomplicated cases of
bacterial conjunctivitis should be seen in 3 to 4 days if there is no improve-
ment in symptoms [9]. Bacterial conjunctivitis in young children or the
debilitated should be managed conservatively and may need closer follow-
up. It is wise to obtain cultures in these populations [2].
The contact lens user who has pain or redness should remove the lens
immediately. After a work-up, if one suspects an infectious complication
of contact lens use, the patient should discontinue lens wear. Smears and
cultures, which are usually performed with ophthalmology consultation,
should be obtained in patients who have an infectious corneal ulcer greater
than 1 mm or when an unusual organism is suspected. Intensive antibiotic
therapy should be initiated using topical fluoroquinolone, six to eight times
per day, and a cycloplegic agent. The patient requires follow-up in 1 day [7].
Hyperacute conjunctivitis is a conjunctivitis caused by Neisseria gonor-
rhoeae and occurs most commonly in sexually active persons. Infection
with N meningitidis is also known to cause hyperacute conjunctivitis but
occurs less frequently and can only be differentiated from infection with
N gonorrhoeae through laboratory testing. N gonorrhoeae is usually spread
from genital-hand-eye contact in the young sexually active population, but
neonates can acquire it from the birth canal. The infection will manifest in
neonates 3 to 5 days postpartum with bilateral discharge [2].
Ocular N gonorrhoeae infection is abrupt in onset and produces copious
amounts of purulent discharge that reforms quickly after wiping away.
Marked conjunctival injection, conjunctival chemosis, lid swelling, globe
42 MAHMOOD & NARANG
tenderness through closed lids, and preauricular lymphadenopathy may all

be found on physical examination (Fig. 5). Work-up should include immedi-
ate staining for gram-negative diplococci and special cultures for Neisseria sp.
The infection may or may not be associated with a urethral discharge. In
infants born to infected mothers, the infection may be localized to other or-
gans (arthritis, meningitis, pneumonia) or may be disseminated (sepsis) [2].
Treatment for hyperacute conjunctivitis need not be complicated. The eye
should be irrigated with saline solution. Selection of topical antibiotics is the
same as for bacterial conjunctivitis. It is recommended that systemic antibi-
otics directed against N gonorrhoeae be initiated, because a large number of
patients with N gonorrhoeae conjunctivitis also have concurrent venereal dis-
ease. Urgent referral is critical in N gonorrhoeae infection. In contrast to bac-
terial conjunctivitis, hyperacute conjunctivitis can have sight-threatening
outcomes secondary to ulceration and perforation [2].
Ocular chlamydial infection leads to two forms of conjunctivitis depend-
ing on the serotype of the organism. Serotypes A through C cause trachoma,
a chronic keratoconjunctivitis that is the most common form of preventable
blindness in the world. Inclusion conjunctivitis is caused by serotypes D
through K. Inclusion conjunctivitis is a common, primarily sexually trans-
mitted disease that affects both newborns and adults. The incidence of inclu-
sion conjunctivitis is higher than that of ocular N gonorrhoeae infection in
newborns. Newborns acquire the infection in the birth canal and cervix
and present with tearing, conjunctival inflammation, and eyelid swelling
with moderate discharge starting from 5 to 12 days after birth. In adults,
inclusion conjunctivitis is transmitted via genital secretions and may be
a result of autoinoculation. The infection can be subacute or even chronic
and is most common in young, sexually active persons aged 18 to 30 years.
The adult patient will present with unilateral or bilateral redness, foreign
body sensation, mucopurulent discharge, and preauricular adenopathy.
Because as many as one half of affected adults will also have concurrent,
Fig. 5. Hyperacute conjunctivitis caused by Neisseria sp. Note the copious amounts of purulent
discharge. (From Boruchoff SA. Anterior segment disease: a diagnostic color atlas. Boston:
possibly asymptomatic, cervical/urethral chlamydial infection, laboratory

studies should be performed when inclusion conjunctivitis is suspected.
A work-up for other sexually transmitted diseases such as syphilis and gon-
orrhea should also be considered because co-infection rates can be high [2].
In the adult, one should treat the sexual disease by prescribing azithromy-
cin, 1 g orally for one dose, doxycycline, 100 mg orally twice daily, or eryth-
romycin, 500 mg orally four times daily for 7 days for the patient and sexual
partners. Topical erythromycin, tetracycline, or sulfacetamide ointment
twice to three times daily for 2 to 3 weeks will help treat the ocular infection.
Follow-up should be arranged with ophthalmology in 1 week [7]. In neona-
tal inclusion conjunctivitis, systemic antibiotics are commonly used in addi-
tion to topical antibiotics because this condition can be associated with otitis
media and respiratory and gastrointestinal tract infections. Consultation
with ophthalmology is essential because special cultures and stains may be
required to direct treatment [2]. If no information regarding a specific organ-
ism is available during the initial visit, empiric therapy with erythromycin
ointment four times daily and with erythromycin elixir, 50 mg/kg/d divided
four times per day, may be initiated [7].
Chronic forms of conjunctivitis have many causes, such as contact lens
use, prescription and over-the-counter eye drops, chlamydia, or molluscum
contagiosum, as well as other less common causes. A history of collagen vas-
cular disease or diuretic or antidepressant use raises the possibility of dry
eyes as a cause of chronic conjunctivitis. Chronic unilateral conjunctivitis
is a separate entity and has more causes. It presents a diagnostic dilemma
even to the specialist. Possible diagnoses may include keratitis, nasolacrimal
duct obstruction, occult foreign body, or ocular neoplasm. Referral is indi-
cated in the case of chronic unilateral conjunctivitis [2].
Subconjunctival hemorrhage
Subconjunctival hemorrhage is caused by bleeding of the conjunctival or
episcleral vessels deep to the conjunctiva into the subconjunctival space [10].
Subconjunctival hemorrhage can be spontaneous or related to trauma or
systemic illness. When it is spontaneous, it is usually secondary to decreased
lubrication of the eye [11].
The patient will usually present with a painless red eye that has no effect
on his or her vision. It often causes alarm when the patient first notices it.
There is no discharge associated with subconjunctival hemorrhage. The
patient may recall a history of mild trauma or valsalva (such as coughing
or vomiting). A history of anticoagulation therapy may also be elicited.
The patient should be asked about a history of hypertension, diabetes, or
any bleeding disorder because subconjunctival hemorrhage may be a pre-
senting sign of any of these conditions [5].
On examination, subconjunctival hemorrhage appears as fresh red blood
on a white sclera with clear borders and masks the conjunctival vessels
44 MAHMOOD & NARANG
(Fig. 6). When evaluating a subconjunctival hemorrhage, one should con-

sider staining the eye to rule out corneal injury if the history suggests it or
if the patient has any type of pain associated with the subconjunctival hem-
orrhage [1]. If the subconjunctival hemorrhage is large and the injury
occurred in the setting of trauma, there may be penetrating injury to the
globe (globe rupture) that is obscured by the hemorrhage, requiring emer-
gency ophthalmology consultation [7]. For minor subconjunctival hemor-
rhages, patient education and reassurance are the mainstays of treatment,
and these patients do not need ophthalmology referral. Prescribing warm
compresses and lubrication drops may help reduce recovery by 1 to 3
days. A high-profile subconjunctival hemorrhage will take approximately
10 to 14 days to resolve [1].
Episcleritis
The episclera is a thin membrane that covers the sclera and lies beneath
the conjunctiva. Episcleritis is generally a benign inflammatory condition
that involves only the superficial episcleral tissue and not the deep episcleral
tissue that overlies the sclera. Most cases are idiopathic in nature and are
commonly seen in young adults [12,13]. Episcleritis can be associated with
systemic diseases such as rheumatoid arthritis, polyarteritis nodosa, sys-
temic lupus erythematosus (SLE), inflammatory bowel disease, sarcoidosis,
Wegener’s granulomatosis, gout, HZV, or syphilis [13].
Episcleritis is usually characterized by a rapid onset of redness that may
be associated with a feeling of grittiness and a dull headache. Vision is
unaffected, and discharge, if present, is usually watery. On examination,
Fig. 6. Subconjunctival hemorrhage. The extravasated blood has completely obscured the
sclera underneath. Note the slightly raised appearance of the affected area. (From Boruchoff
SA. Anterior segment disease: a diagnostic color atlas. Boston: Butterworth-Heinemann;
2001. p. 147; with permission.)
focal areas of redness are usually observed rather than a diffuse process.
White sclera may be seen between radially coursing dilated episcleral vessels
[5]. The conjunctival and superficial episcleral vessels can be seen displaced
outward from the sclera while the underlying deep episcleral plexus is not
involved and lies flat against normal scleral tissue. This appearance is best
observed using red-free light on slit lamp examination. Nodular episcleritis
occurs with the presence of a tender scleral nodule and generally is more
uncomfortable than simple episcleritis with a more prolonged course
(Fig. 7). The engorgement of the superficial episcleral plexus gives the eye
a distinct red or salmon hue, and there may be tenderness on palpation [12].
This condition is usually self-limiting and will resolve within 2 to 3 weeks
even without intervention. Treatment may involve oral NSAIDs and should
be referred electively to an ophthalmologist for outpatient evaluation. Top-
ical anti-inflammatory agents and lubricants may also be helpful, but
steroids should only be prescribed after consultation with an ophthalmolo-
gist [5,13].
Scleritis
Scleritis is defined as an inflammation of the sclera that may involve the
cornea, adjacent episclera, and underlying uvea. The maximal involvement
in scleritis is the deep episcleral plexus, which is displaced outward by the
edematous swollen sclera. The classification of scleritis can be divided into
anterior and posterior. Anterior scleritis is further subdivided into diffuse,
nodular, or necrotizing. Diffuse anterior scleritis is characterized by exten-
sive scleral edema and congestion of the scleral vessels with poorly defined
margins. Nodular anterior scleritis is characterized by focal, often multiple
well-defined nodules of scleral edema and congestion of scleral vessels dis-
tinct from its overlying inflamed episclera. Necrotizing anterior scleritis is
Fig. 7. Nodular episcleritis. Note the focal area of redness and dilated blood vessels. (From
Boruchoff SA. Anterior segment disease: a diagnostic color atlas. Boston: Butterworth-
Heinemann; 2001. p. 183; with permission.)
46 MAHMOOD & NARANG
characterized by an avascular area of scleral necrosis and often by signifi-

cant inflammation of the surrounding sclera. The sclera surrounding the
necrosis may also be devoid of any prominent inflammation, which is
seen in scleromalacia perforans, often associated with rheumatoid arthritis.
Posterior scleritis is characterized by involvement of the sclera posterior to
the insertion of the rectus muscles and may occur in association with ante-
rior scleritis or in isolation [12].
Although most cases of scleritis are immune mediated, the disease can
also be triggered by infection, surgery, malignancy, or drugs. Approximately
39% to 50% of the cases are associated with a systemic disorder. The result-
ing scleral inflammation may be in part or completely secondary to the
immune-mediated response. A large number of connective tissue disorders
are associated with scleral disease; the most common is rheumatoid arthritis.
Wegener’s granulomatosis is the most common vasculitis associated with
this condition. Other causes include relapsing polychondritis, SLE, polyar-
teritis nodosa, Reiter’s syndrome, inflammatory bowel disease, and progres-
sive systemic sclerosis. Approximately 5% to 10% of anterior scleritis is
infectious owing to viral, bacterial, fungal, or parasitic agents. Infectious
scleritis is more likely in the presence of infectious keratitis. Infectious scler-
itis should be suspected in patients with a history of accidental trauma,
ocular surgical procedures, or recurrent attacks of HSV or varicella-zoster
virus. Herpes zoster ophthalmicus was found in one review to be the most
common infectious cause of scleritis. Other common causes are syphilis, mil-
iary tuberculosis, HSV, Pseudomonas aeruginosa, Epstein-Barr virus, and
Coxsackie B5. A history of surgery has been shown to be a risk factor for
bacterial causes of scleritis. For instance, a patient with pterygium removal
may present months to years after surgery with bacterial scleritis, most often
owing to Pseudomonas aeruginosa. When a bacterial scleritis leads to a pyo-
genic infection, it can be a therapeutic challenge due to the avascularity of
the sclera. Fungal infections may remain undiagnosed for a long time before
the exact cause is found [12].
Patients who present with scleritis either already have a known underly-
ing systemic disorder such as rheumatoid arthritis or present de novo. Scler-
itis may also be the sole initial presenting feature of a systemic disease, most
commonly rheumatoid arthritis [14]. Scleritis usually occurs in white, mid-
dle-aged women with a mean age of onset of 49 years, and female patients
account for 71% of all cases. The characteristic feature of scleritis is severe
pain that may involve the eye and orbit and radiate to the ear, scalp, face,
and jaw. It is usually dull, boring, and can be so severe that it often awakes
the patient from sleep or causes significant impairment in their daily activ-
ities. It may often be confused with other causes of headache, such as giant
cell arteritis, migraines, cerebral aneurysm, tic douloureux, and tumor [12].
It can also be associated with photophobia, tearing, and with pain on
accommodation [14]. Mild analgesics will often not relieve the pain,
although some patients may present with little or no discomfort due to
the use of NSAIDs before presentation. Patients who have posterior scleritis
may present with reduced vision with or without pain [12].
Many features on the physical examination will aid in making the diag-
nosis. The redness may be focal or diffuse, unilateral or bilateral, and pres-
ent with tenderness to palpation of the globe. Nodular anterior scleritis is
characterized by a localized area of scleral edema in which distinct nodules
reside. Nodules may be single or multiple and are tender to palpation. In
scleritis, the vascular engorgement of the deep episcleral plexus gives the
eye a characteristic bluish-violet discoloration, and the deep scleral vessels
will not blanch in response to vasoconstrictor agents such as 2.5% to
10% phenylephrine or 1:1000 epinephrine (Fig. 8). This characteristic is in
contrast to the blanching seen with conjunctival and superficial vessels in
conjunctivitis and episcleritis [12]. The hallmark of scleritis is the presence
of scleral edema and congestion of the scleral plexus, which may involve
the superficial and deep vascular coats. In scleritis, the vascular architecture
of the superficial episcleral plexus is disrupted by the presence of irregularly
oriented vessels. In episcleritis, only the superficial episcleral plexus is con-
gested, and its radial configuration is preserved. Avascular areas strongly
imply the diagnosis of necrotizing scleritis. Slit lamp examination using
red-free light is helpful in characterizing the pattern and depth of episcleral
vascular engorgement. The sclera will often be edematous and may cause the
slit lamp light beam to be displaced forward in the area of maximal swelling.
Anterior uveitis, peripheral choroiditis with or without retinal detachment,
uveal effusion, or disk edema can all be seen with posterior extension of
scleritis and are not seen with episcleritis [14]. In isolated posterior scleritis,
the eye may be white, but sometimes inflamed posterior sclera can be visu-
alized in the extremes of gaze. Because there may be a paucity of physical
examination findings, imaging is often necessary to make the diagnosis. Pos-
terior scleritis can be delineated using B-scan ultrasonography and high-
definition orbital MRI [12].
Fig. 8. Dilation of the deep episcleral vessels and a bluish discoloration of the eye in a patient
with scleritis. (From Boruchoff SA. Anterior segment disease: a diagnostic color atlas. Boston:
48 MAHMOOD & NARANG
In acute presentations of scleritis, blood work may include a complete

blood count, electrolytes, tests of renal function, assays for acute phase
reactants such as C-reactive protein, and evaluation of the erythrocyte sed-
imentation rate, and serologic testing to rule out and determine the degree of
associated systemic conditions may be performed [12,14]. In cases of sus-
pected infectious scleritis, serologies and conjunctival scrapings for smears
and cultures are often obtained. If these tests are negative after 48 hours
and the scleritis continues to progress, scleral or corneoscleral biopsy is
essential. Tests may include rapid plasma reagin and microhemagglutina-
tion-Treponema pallidum in cases of suspected syphilis. A positive
c-ANCA in a patient with scleritis is specific for Wegener’s granulomatosis.
Assays for rheumatoid factor for rheumatoid arthritis and for anti-nuclear
antibody suggestive of SLE, rheumatoid arthritis, polymyositis, progressive
systemic sclerosis, or mixed connective tissue diseases should also be per-
formed. Ultrasonography, an orbital CT scan, fluorescein angiography,
and high-definition orbital MRI may be required to confirm the diagnosis
and to assess the extent of disease [14]. Although these tests are generally
not done in the emergency room setting, the consulting ophthalmologist
and primary care physician should be involved in expediting the work-up
and delineating the etiology.
Making this diagnosis early is important owing to associated serious
complications. In a study of 358 patients with scleritis, decreased vision
occurred in 37%, anterior uveitis was present in 42%, peripheral ulcerative
keratitis in 14%, glaucoma in 13%, and cataracts in 17%. Elevated intraoc-
ular pressure can develop in any patient with any type of scleritis, with the
rate of glaucoma ranging from 9% to 22% in anterior scleritis. Greater
visual loss is generally seen in patients with systemic disease, especially in
rheumatoid arthritis, and is found to be independent of corneal complica-
tions and cataracts. Necrotizing scleritis also results in greater visual loss
than in the non-necrotizing form [14].
The goal of treatment is to remove or treat the underlying cause but,
most importantly, to control the inflammatory process and therefore reduce
damage to the eye. Patients with posterior or necrotizing scleritis need more
intensive and urgent therapy than patients with anterior non-necrotizing dis-
ease. Any scleritis associated with an underlying systemic disease usually
requires more aggressive immunosuppressive therapy. Approximately one
third of patients with anterior scleritis will respond to NSAIDs, one third
to systemic corticosteroids, and the other third to more aggressive systemic
immunosuppression [14]. The diagnosis of scleritis calls for a prompt refer-
ral to an ophthalmologist and initiation of an oral NSAID initially. Further
management including topical or oral corticosteroids, other immunosup-
pressive agents, and possible surgical intervention should be done in con-
junction with the primary care physician and ophthalmologist [12]. When
timely outpatient evaluation is not possible or treatment is more urgent,
admission may be required.
Uveitis
Uveitis can be defined as an inflammation of the iris (iritis), ciliary body
(cyclitis), and choroid (choroiditis). Anterior uveitis, often called iridocyclitis,
involves inflammation of the anterior portion of the uveal tract and usually
affects young or middle-aged persons. Posterior uveitis includes vitritis, cho-
roiditis, retinitis, chorioretinitis, or retinochoroiditis. Panuveitis or diffuse
uveitis are the terms used to characterize both anterior and posterior involve-
ment. There are multiple causes of uveitis, but uveitis can generally be char-
acterized as inflammatory, traumatic, or infectious. Uveitis has been shown
to be associated with the histocompatability antigen HLA B-27. In the United
States and England, 30% to 70% of patients with anterior uveitis have this
antigen. About half of these patients have an associated systemic disease
such as ankylosing spondylitis, psoriatic arthritis, reactive arthritis, or inflam-
matory bowel disease. Many other systemic diseases may present with uveitis,
including sarcoidosis, juvenile idiopathic arthritis, Behçet disease, Kawasaki
disease, multiple sclerosis, and Wegener’s granulomatosis [15].
Although infection is an uncommon cause of uveitis, it is important to
rule it out before instituting immunosuppressive therapy. Parasitic (eg, toxo-
plasmosis), viral (cytomegalovirus, herpes viruses), bacterial (tuberculosis,
Lyme disease), and treponemal (syphilis) agents could be responsible. Toxo-
plasmosis is a common cause of retinochoroiditis in normal and immuno-
suppressed patients and is responsible for as many as 25% of occurrences
of posterior uveitis in the United States. Cytomegalovirus can cause poste-
rior uveitis in immunocompromised individuals, particular in AIDS
patients, but its incidence has decreased in the United States since antiretro-
viral therapy has become available. Tuberculosis was once the most com-
mon cause of choroiditis but is now found in less than 1% of all cases in
the United States. Syphilis can cause both anterior and posterior uveitis,
usually during the secondary or tertiary stages of infection. Traumatic iritis
is commonly seen in the emergency department and is usually secondary to
a direct blow from a blunt object [15].
Anterior uveitis often presents suddenly with a red and painful eye.
Photophobia and conjunctival injection are often seen; sometimes blurred
vision is present [1]. Patients generally complain of a deep aching pain
that may radiate to the periorbital or temple area. It is worse with eye move-
ment and during accommodation. On examination, hyperemia is prominent
adjacent to the limbus (perilimbal or circumcorneal injection), in contrast to
that seen in conjunctivitis in which inflammation tends be more prominent
at a distance from the limbus (Fig. 9). Tearing may be present, but no
purulent discharge is seen [16]. Generally, the pupil is constricted, may be
irregular, and is sluggish in response to light in comparison with the unin-
volved eye. On pupillary examination, the patient may have direct photo-
phobia when light is directed into the affected eye, as well as consensual
photophobia when light is shone on the unaffected eye. Consensual
50 MAHMOOD & NARANG
Fig. 9. Anterior uveitis. Note the perilimbal injection and the irregularly shaped pupil.
(Courtesy of the University of Michigan Kellogg Eye Center, Ann Arbor, Michigan; with
permission.)
photophobia can be a helpful distinguishing feature of anterior uveitis be-

cause it is not seen in other superficial causes of photophobia [1].
Posterior uveitis can cause ‘‘floaters’’ and visual changes but generally
does not cause redness or significant pain. When the retina is involved,
patients may complain of blind spots or flashing lights [15].
The diagnosis of uveitis is often confirmed by the presence of inflammatory
cells and a proteinaceous flare in the anterior or posterior chambers of the eye
on slit lamp examination. If inflammation is severe, leukocytes can settle in
the anterior chamber and form a hypopyon, a white or yellowish accumula-
tion of purulent material. This accumulation can sometimes be visible without
the aid of magnification [5]. On slit lamp examination, deposits of white blood
cells on the endothelium (keratitic precipitates) are often visualized, which is
a hallmark of iritis. Small-to-medium keratitic precipitates are classified as
nongranulomatous, whereas granulomatous (eg, sarcoid) keratitic precipi-
tates are large [16]. Infectious uveitis can be difficult to diagnose and usually
requires specific findings on a detailed ophthalmologic examination, specific
serology testing, or, after uveitis, does not respond to anti-inflammatory ther-
apy [15]. The diagnostic evaluation in patients suspected to have a systemic
condition requires further testing. Patients with pulmonary complaints
should undergo chest radiography or CT or both to assess for possible sar-
coidosis. Radiographic images of the sacroiliac joints and HLA-B27 typing
are helpful when considering a spondyloarthropathy as a diagnosis. Colono-
scopy should be done in patients with gastrointestinal symptoms or heme-
positive stools to assess for inflammatory bowel disease [15]. These tests rarely
need to be done in the emergency room or inpatient setting as long as an
expedited outpatient work-up can be performed.
Uveitis is best managed in collaboration with an ophthalmologist.
Initiation of therapy in a timely fashion is critical because of the severe
sight-threatening complications associated with uveitis, including cataracts,
glaucoma, and retinal detachment. Corticosteroids are the mainstay of
therapy in treating noninfectious causes [15]. Topical corticosteroids (eg,

prednisolone acetate 1%) are used in anterior uveitis but are not helpful
in posterior uveitis owing to poor penetration. This treatment may worsen
this condition if there is a concern of an infectious etiology; therefore, it
should only be prescribed after appropriate consultation with an ophthal-
mologist. Although cataracts can occur secondary to the inflammatory pro-
cess, they are also a recognized complication of corticosteroids [15].
Mydriatic and cycloplegic agents are also often prescribed. Mydriatics (eg,
phenylephrine HCl, hydroxyamphetamine HBr) are sympathomimetic
agents used to prevent the formation of synechiae by pupillary dilation.
A synechia is an adhesion where the iris adheres to either the cornea or
lens. Although synechiae rarely cause visual problems, they can lead to sec-
ondary glaucoma. Parasympatholytic agents (eg, atropine sulfate, homatro-
pine, cyclopentolate) produce both mydriasis and cycloplegia which block
nerve impulses to the pupillary sphincter and ciliary muscle, reducing pain
and photophobia [16]. Systemic corticosteroid therapy is usually reserved
for bilateral disease that is refractory to local medication or for patients
with significant ocular disability or retinitis. Other immunosuppressive med-
ications may be used in steroid-dependent or refractory uveitis. In general,
management of uncomplicated noninfectious uveitis can be done on an out-
patient basis, but follow-up with an ophthalmologist should be arranged
within 24 hours. Refractory cases or those secondary to an infectious cause
often require admission and further diagnostic tests. Patients with suspected
posterior uveitis who present with floaters or visual changes need urgent
ophthalmology consultation [15].
Acute angle-closure glaucoma

AACG is a condition that develops when the peripheral tissue of the iris
blocks the outflow of fluid from the anterior chamber, resulting in elevated
intraocular pressure [1]. Mydriasis, such as in the low-light evening hours,
can often worsen the condition as the accordion-like folds of the iris gather
together into folds that cause obstruction. Mydriatic medications, systemic
anticholinergics (eg, antihistamines or antipsychotics), and accommodation
(ie, reading) can also cause pupillary block [7]. The patient with AACG will
usually present with severe ocular pain, redness, a decrease in vision, and
a pupil in mid-dilation [1]. Blurred vision, seeing halos around lights, and
headache with nausea and vomiting may also be a part of the clinical
presentation. Acute attacks may be self-limited and resolve spontaneously
or may occur repeatedly. Patients who are elderly or far-sighted are at the
highest risk for AACG because of enlargement of their lenses [7]. Because
there are several other causes of a non-traumatic acute increase in intraoc-
ular pressure, such as glaucomatocyclitic crisis, inflammatory open-angle
glaucoma, pigmentary glaucoma, and mechanical and postsurgical closure,
the work-up should include a thorough history (family history, retinal
52 MAHMOOD & NARANG
problems, recent laser treatment or surgery, medications), a slit lamp exam-

ination, and measurement of intraocular pressure [7].
On physical examination, AACG will usually present with a global injec-
tion pattern. There may be tearing but no other discharge. Corneal edema
may make the cornea appear ‘‘steamy’’ or ‘‘hazy.’’ On slit lamp examina-
tion, there may be keratitic precipitates, anterior chamber cells and flare,
posterior synechiae, and a shallow anterior chamber. A fundoscopic exam-
ination that reveals optic nerve cupping indicates the need for urgent treat-
ment. Perhaps the most important feature is that, on penlight examination,
the pupil will be mid-dilated and nonreactive (Fig. 10A, B). If the pupil
reacts, the diagnosis should be reconsidered [1]. Normal intraocular pressure
is less than 21 mm Hg. Patients with glaucoma have an intraocular pressure
greater than that, and those with an intraocular pressure greater than 30 mm Hg
require prompt treatment. Most emergency departments are fitted with at
least one instrument to allow measurement of intraocular pressure. The
emergency physician should be familiar with the tools available in his or
her workplace. The most common instruments used in the emergency
department for measuring intraocular pressure are the tonopen and Schiotz
tonometer. Goldmann tonometry (applanation) may also be used. Other
instruments include the MacKay-Marg tonometer. If infection is suspected,
the clinician should be certain to use a sterile tip when using a contact
tonometer. A simple and direct method to measure intraocular pressure
is to gently press the globe through the eyelid while the patient is instructed
to look down; the experienced practitioner may note differences in the
‘‘hardness’’ of one globe when compared with the other. Unfortunately,
this method requires experience to gain accuracy, and most emergency phy-
sicians should use this only as a rough estimate [17].
Treatment for AACG mandates prompt and rapid transfer to an eye
specialist. In the emergency department, 1 drop of 0.5% timolol can be admin-
istered, followed by another drop 5 minutes later. Topical steroids, such as 1%
Fig. 10. (A, B) Acute angle-closure glaucoma. These close-ups reveal a global injection, a
‘‘hazy’’ appearance to the pupil, and a loss of iris structure. (Panel A is from Dayan M, Turner B,
McGhee C. Lesson of the week: acute angle closure glaucoma masquerading as systemic illness.
BMJ 1996;313:413–5; with permission; and Panel B is from Thiel R. Atlas of diseases of the eye,
vol. I. New York: Elsevier; 1963. p. 255; with permission.)
prednisolone acetate, should be given every 15 to 30 minutes for four doses and
then hourly [7]. Also, 1% topical apraclonidine or 0.15% or 0.2% topical bri-
monidine should be given for one dose [7]. Acetazolamide, a carbonic anhy-
drase inhibitor, can be given at a dose of 500 mg orally to help decrease the
production of fluid. In cases of phakic pupillary block (in which there is a na-
tive, natural lens), pilocarpine, 1% to 2%, can be administered every 15 min-
utes for 2 minutes [7]. If vision has been compromised to detection of hand
motion or worse, all topical medications not contraindicated, intravenous ac-
etazolamide, and intravenous hyperosmotic fluid (such as mannitol) should be
administered. Systemic symptoms such as pain and vomiting should be treated
as appropriate with intravenous or oral medications. Definitive treatment in-
cludes peripheral iridotomy performed by either laser or incision. The fellow
eye should receive a prophylactic iridotomy based on anatomy, because ap-
proximately one half of fellow eyes in patients with acute angle-closure will
sustain acute attacks within 5 years [18]. The specialist may choose to delay de-
finitive treatment until the inflammation has subsided.
Failure to recognize and treat AACG can result in excessive intraocular
pressures that can damage the optic nerve and lead to visual loss. When
evaluating a patient with migraine headache, it is prudent to document an
eye examination and pupil reactivity because AACG can mimic migraine
headache. One article highlights three cases in which the diagnosis of
AACG was delayed or missed due to several factors that hindered appropri-
ate and thorough evaluation. When patients are elderly, have disabilities
such as dementia, deafness, or limited mobility, or have concurrent psychi-
atric or medical conditions, a thorough and directed history and physical (ie,
using a slit lamp) may be problematic and challenging. In these cases, a high
index of suspicion and a diligent work-up must be pursued when the presen-
tation includes red eye, blurred vision, or headache [19].
Summary
The acutely red eye is a common complaint in the emergency department.
Although most causes are benign and self-limiting, appropriate work-up
and treatment can identify serious conditions and prevent significant mor-
bidity such as blindness. As outlined herein, the emergency physician should
obtain a relevant history and perform a thorough examination using the slit
lamp and measurement of intraocular pressures when appropriate.
In general, patients who present with severe ocular pain, acute visual
changes, corneal opacification, hypopyon, or blurred disk margins in the
setting of a red eye warrant an aggressive search for serious causes and ur-
gent ophthalmology referral. Topical analgesics should never be prescribed
[5], and topical steroids should be initiated only after ophthalmology consul-
tation. If the clinical presentation is concerning for scleritis and AACG, ur-
gent consultation with the specialist for confirmation of diagnosis and initial
management is recommended. Gonococcal conjunctivitis and posterior
Table 1
Common clinical findings in the acute red eye
Foreign Threat
body to Timing of
Diagnoses Pain hyperemia sensation Discharge Itching Photophobia Onset Pupil Cornea Vision vision consultation
Episcleritis No Focal No Tearing No No Rapid Not Clear Not No Electively
affected affected
Scleritis Yes Focal or No No No Yes Progressive Not Occasional Not Yes Urgent
diffuse affected peripheral affected
opacity
Uveitis Yes Diffuse, No Watery, No Yes Sudden Constricted, May be Blurred Yes Within
perilimbal minimal sluggish hazy 24 hours
to light
Allergic No Diffuse, Yes Watery to Yes No Progressive Not Clear Not No Electively
conjunctivitis toward mucoid affected affected
fornices
Viral Noa Diffuse, Yes Watery Mild Noa Sudden Not Clear Not Noa Electively
conjunctivitis toward and clear with rapid affected affected
fornices progression
Bacterial No Diffuse, Yes Mucopurulent Mild No Sudden Not Clear Not No Usually
conjunctivitis toward affected affected elective
fornices
Subconjunctival No Focal or No No No No Acute Not Clear Not No Usually
hemorrhage diffuse affected affected elective
Acute Yes Diffuse, No Tearing No Yes Sudden, Mid- Hazy Blurred, Yes Urgent
angle-closure perilimbal usually in dilated, halos
glaucoma evening nonreactive around
lights
a
Can occur in patients with herpes zoster ophthalmicus.
uveitis associated with floaters also represent serious, sight-threatening con-

ditions and similarly require urgent ophthalmology evaluation. Expeditious
follow-up with the specialist will help ensure that the patient has as little per-
manent damage to their vision as possible. With this is mind, one should
remember that most causes of red eye in the emergency department are
self-limiting and can be treated supportively. In general, patients with
benign etiologies may receive follow-up from their primary care physician
or the ophthalmologist electively to monitor for treatment effectiveness
and for complications. The key clinical features and the urgency of consul-
tation for the various diseases are outlined in Table 1.
References
[1] Roscoe M, Landis T. How to diagnose the acute red eye with confidence. JAAPA 2006
2003;19(3)24–30.
[2] Morrow GL, Abbott RL. Conjunctivitis. Am Fam Physician 1998;57(4):735–46.
[3] Weber CM, Eichenbaum JW. Acute red eye: differentiating viral conjunctivitis from other,
less common causes. Postgrad Med 1997;101(5):185–9.
[4] Hara JH. The red eye: diagnosis and treatment. Am Fam Physician 1996;54(8):2423–30.
[5] Leibowitz HM. The red eye. N Engl J Med 2000;343:345–51.
[6] Wirbelauer C. Management of the red eye for the primary care physician. Am J Med 2006;
119:302–6.
[7] Kunimoto DY, Kanitkar KD, Makar MS, editors. The Wills eye manual: office and emer-
gency room diagnosis and treatment of eye disease. 4th edition. Philadelphia: Lippincott
Williams & Wilkins; 2004. p. 61, 71, 91, 94, 98, 155, 179–81.
[8] Shaikh S, Ta CN. Evaluation and management of herpes zoster ophthalmicus. Am Fam
Physician 2002;66(9):1723–30.
[9] Prepared by the American Academy of Ophthalmology Cornea/External Disease Panel Cor-
nea/External Disease Panel Members. American Academy of Ophthalmology. Conjunctivi-
tis, preferred practice pattern. San Francisco (CA): American Academy of Ophthalmology;
2003. Available at: www.aao.org/ppp. Accessed August 11, 2007.
[10] Roy FH. The red eye. Ann Ophthalmol 2006;38(1):35–8.
[11] Walling AD. Tips from other journals: when is red eye not just conjunctivitis? Am Fam
Physician 2002;66(12):2299–300.
[12] Okhravi N, Odufuwa B, McCluskey P, et al. Scleritis. Surv Ophthalmol 2005;50(4):351–63.
[13] Sowka JW, Gurwood AS, Kabat AG. Episcleritis. Handbook of ocular disease management
web site 2000. Available at: http://www.revoptom.com/HANDBOOK/sect2f.htm. Accessed
August 4, 2007.
[14] Albini TA, Rao NA, Smith RE. The diagnosis and management of anterior scleritis. Int
Ophthalmol Clin 2005;45(2):191–204.
[15] Hajj-Ali RA, Lowder C, Mandell BF. Uveitis in the internist’s office: are a patient’s eye
symptoms serious? Cleve Clin J Med 2005;72(4):329–39.
[16] Nishimoto JY. Iritis. How to recognize and manage a potentially sight-threatening disease.
Postgrad Med 1996;99(2):255–62.
[17] Knoop KJ, Dennis WR. Ophthalmologic procedures. In: Roberts JR, Hedges JR, editors.
Clinical procedures in emergency medicine. 4th edition. Philadelphia: Saunders; 2004.
p. 1241–79.
[18] American Academy of Ophthalmology. Primary angle closure, preferred practice pattern
web site 2005. Available at: www.aao.org/ppp. Accessed August 5, 2007.
[19] Gordon-Bennett P, Ung T, Stephenson C, et al. Misdiagnosis of angle closure glaucoma.
BMJ 2006;333(7579):1157–8.
Emerg Med Clin N Am
26 (2008) 57–72
Ocular Infection and Inflammation

Jorma B. Mueller, MDa,
Christopher M. McStay, MDb,*
a
Emergency Medicine Residency, New York University/Bellevue Hospital Center,
462 First Avenue, New York, NY 10016, USA
b
Department of Emergency Medicine, New York University/Bellevue Hospital Center,
462 First Avenue, New York, NY 10016, USA
Managing the inflamed or infected eye in the emergency setting presents

a diagnostic and therapeutic challenge to the emergency physician; the
causes and prognoses range from benign, self-limited illness to organ-threat-
ening pathology. A careful history, with attention to comorbid illnesses and
time course, is paramount, as is knowledge of the complete ophthalmologic
examination. Much of the organ morbidity is ameliorated with prompt ther-
apy in the emergency department (ED) and by initiating ophthalmologic
consultation.
Conjunctivitis
Inflammation of the bulbar (covering the globe of the eye) and tarsal (lining
the orbit) conjunctiva can be caused by viral or bacterial infections, trauma,
toxic exposure, or autoimmune disease. Patients will complain of redness,
pruritis, and a foreign body sensation that is often associated with discharge.
Awakening in the morning with eye crusting is a common complaint. Photo-
phobia and visual loss should not be present. Physical examination will reveal
injection of the conjunctiva associated with lid edema. Discharge may be scant
or may range from stringy and fibrinous to copious and purulent. If bacterial
conjunctivitis is expected, gram stain and culture of the discharge is often
revealing and can guide initiation of therapy. Most conjunctivitis seen in
the ED is of viral origin and is self limited. However, the emergency practi-
tioner should be familiar with all causes because the clinical course of conjunc-
tivitis can lead to significant morbidity, depending on the cause.
E-mail address: [email protected] (C.M. McStay).
58 MUELLER & MCSTAY
Viral conjunctivitis
Most cases of viral conjunctivitis are caused by adenoviridae, but entero-
viruses and coxsackievirus may also be implicated. Conjunctivitis of viral
origin is associated with significant redness and pruritis but should have
less discharge than bacterial conjunctivitis. Chemosis and soft tissue swelling
may be dramatic. The presence of preauricular lymphadenopathy, conjunc-
tivitis, and a viral prodrome are classic and frequently present. Disease often
becomes bilateral in the first 24 to 48 hours [1,2].
Viral conjunctivitis is contagious for almost 2 weeks; patients, family, and
caretakers should be educated in appropriate infection-preventing measures,
including hand washing. Treatment is symptomatic, with artificial tears,
cold compresses, and vasoconstrictor-antihistamine combinations for severe
pruritis.
Fever, lymphadenopathy, and conjunctivitis with concomitant pharyngi-
tis constitute pharyngoconjunctival fever, caused by certain strains of adeno-
viridae. It can have a prolonged course (up to 2 weeks) and resolves
spontaneously. Preceding upper respiratory infection supports this diagnosis.
Epidemic keratoconjunctivitis is also caused by strains of adenoviridae
(usually adenovirus type 8). It generally presents with thicker discharge and
punctuate keratitis (corneal inflammation seen on fluorescein staining), and
may result in conjunctival membrane formation with scarring. It has an
even longer clinical course (up to 3 weeks) and corneal opacities may persist
for months afterwards, affecting vision but ultimately resolving [1,2]. Consul-
tation with an ophthalmologist is recommended to decide whether steroids are
indicated based on the presence and extent of the corneal involvement (Fig. 1).
Bacterial conjunctivitis
The pathogens most commonly associated with bacterial conjunctivitis
include staphylococci, streptococci, N gonorrohae, and C trachomatis. In
Fig. 1. Conjunctivitis in Kawasaki disease. (Courtesy of Christopher McStay, MD, New York,
NY.)
OCULAR INFECTION AND INFLAMMATION 59
contrast to viral conjunctivitis, discharge may be purulent, and crusting

marked, typically with less pruritis. Patients who have bacterial pathogens
may be more likely to report eye crusting that impedes opening each morn-
ing [3]. The process is more likely to be unilateral, but bilateral involvement
does not rule out a bacterial cause. Acuity should be preserved and photo-
phobia absent; presence of either photophobia or decreased visual acuity
should raise concern for corneal involvement. In this setting, the practitioner
should look for signs of keratoconjunctivitis on slit lamp examination. Cor-
neal involvement portends a worse clinical course and is an indication for
ophthalmologic consultation within the ED. Corneal opacification or hypo-
pyon may indicate a keratitis, which could be vision threatening.
If a bacterial pathogen is suspected, treatment with topical drops (eg, qui-
nolone, trimethoprim-polymyxin) should be initiated for 7 to 10 days.
Erythromycin ointment may also be used and is preferred in pediatrics for
easier compliance. In contact lens wearers, pseudomonal coverage is essen-
tial and a quinolone is first line. Most cases of bacterial conjunctivitis
resolve spontaneously in a week to 10 days, but antibiotics limit the duration
and severity of disease [4]. Delaying antibiotic therapy (by 1–3 days) while
awaiting gram stain and culture results appears to cause little harm and
may reduce inappropriate antibiotic usage [5]. Ophthalmologic or ED fol-
low-up can be given, with antibiotic choice tailored to culture results. Sup-
portive care, including warm compresses and eye irrigation, are also
indicated and are of equal importance. Eye patching should be avoided.
A fulminant course of conjunctivitis in a young, sexually active patient or
a neonate (24–48 hours postpartum) should raise the possibility of N gonor-
rohae as the causative organism (Fig. 2). Discharge is often thick, yellowish
green, and associated with significant underlying chemosis. Concomitant
and symptomatic genitourinary symptoms may be present, but their absence
does not rule out the diagnosis. Untreated, corneal involvement leading to
rupture and endophthalmitis can ultimately occur. The decision as to
Fig. 2. Fulminant gonococcal conjunctivitis. (From Goldman L, Ausiello D, editors. Cecil textbook
of medicine. 22nd edition. Philadelphia: Saunders (an imprint of Elsevier); 2004; with permission.)
60 MUELLER & MCSTAY
whether to treat for gonococcal conjunctivitis is based on the history, fulmi-

nant course, and gram stain (chocolate agar cultures should also be sent).
The practitioner should ask about sexual practices, partners, and symptoms
[6]. Treatment is typically more aggressive, and hospital admission for intra-
venous and topical antibiotics may be warranted. For milder cases, one
gram of ceftriaxone intramuscularly and appropriate coverage for concom-
itant Chlamydia infections (doxycycline or azithromycin) is indicated, in
addition to ophthalmic antibiotics (bacitracin, erythromycin ointment). The
high risk of corneal involvement warrants daily ophthalmologic follow-up.
Sexual partners should seek appropriate medical care.
Neonatal conjunctivitis
In the neonatal period, conjunctivitis can occur and the causes are closely
linked with postpartum age at presentation. Conjunctivitis in the first 36
hours postpartum is often chemically induced, caused by silver nitrate
used to prevent maternal transmission of gonococcus, Chlamydia, and other
bacteria. Almost all infants treated with silver nitrate will develop a transient
conjunctivitis that resolves in 1 to 2 days. Silver nitrate drops have largely
been replaced by topical erythromycin in the United States. Neonatal symp-
toms occurring 24 to 48 hours postpartum are often due to N gonorrhoeae
and should be treated with intravenous penicillin, or intramuscular ceftriax-
one or cefotaxime, in addition to saline washes. Topical antibiotics are also
indicated; penicillin or erythromycin ointment should be used every 2 hours
while discharge persists. Blood cultures and cerebrospinal fluid gram stain
and culture are also recommended but are controversial. These patients
should also be treated for concomitant Chlamydia infection [7]. Other bac-
terial causes often occur on days 2 through 5 postpartum and treatment
should be guided by gram stain (erythromycin ointment for gram-positive
organisms, gentamicin or tobramycin drops for gram-negative organisms).
Chlamydial conjunctivitis often occurs on days 5 through 12 postpartum
and should be treated with oral erythromycin for 2 weeks.
Episcleritis
Inflammation of the episclera and sclera may be difficult to distinguish.
Because their causes and prognoses differ considerably, it is important to
differentiate between the two. Both have significant association with sys-
temic disease, so primary care or ophthalmologic follow-up is indicated,
based on patient comorbidities and severity of symptoms.
Inflammation of the episclera, generally a benign, self-limited condition,
presents as acute redness, tearing, pruritis, and foreign body sensation
(‘‘grittiness’’), with minimal-to-moderate pain in one or both eyes. It typi-
cally occurs in young adults and may be recurrent. Examination will reveal
dilated episcleral vessels and an overall injected appearance. The differential
diagnosis also includes conjunctivitis, scleritis, keratitis, and glaucoma.

A slit lamp examination should also be performed to rule out other common
causes of ocular inflammation. A true change in acuity should prompt the
clinician to consider other causes.
To differentiate episcleritis from conjunctivitis, topical anesthetic should
be applied and, under slit lamp, the ocular surface probed with a cotton-tip-
ped applicator. Conjunctival vessels will be highly mobile, whereas episcleral
vessels should remain relatively fixed. In contrast to conjunctivitis, the find-
ings are generally limited to one segment of the bulbar surface. Conjunctival
involvement may also occur in episcleritis but should be focal [8,9]. The epis-
cleral vessels should also blanch with application of a topical vasoconstrictor,
which will help the clinician differentiate between episcleritis and scleritis.
Distribution of the findings may be simple or nodular. In simple disease,
sectoral involvement of the episclera is generally seen, with only part of the
episclera involved. Nodular densities with surrounding injection of the
sclera vessels appear in nodular episcleritis.
Episcleritis is of idiopathic origin in two thirds of cases and generally im-
proves after a week or so. Supportive treatment includes the use of artificial
tears. The use of topical corticosteroids and nonsteroidal anti-inflammatory
drugs has been suggested but both are controversial. A recent study found
the duration and severity of symptoms the same with topical ketorolac
and artificial tears. Patients treated with ketorolac were more likely to com-
plain of stinging [10]. If treatment has failed by 2 weeks, ophthalmologic re-
ferral is indicated. In one third of cases, systemic disease is present.
Evaluation for connective tissue, autoimmune vascular, or rheumatoid
(more common in nodular subtypes) disease may be indicated. Rosacea
and atopy are also commonly associated with episcleritis.
Inflammation of the sclera itself has a more insidious onset, more pro-
nounced pain, and a greater number of complications and systemic asso-
ciations (Fig. 3). It is most often idiopathic in origin, but patients may
Fig. 3. Scleritis. (From Sen HN, Suhler EB, Al-Khatib SQ, et al. Mycophenolate mofetil for the
treatment of scleritis. Ophthalmol 2003;110(9):1750–5; with permission.)
62 MUELLER & MCSTAY
give a history of recent eye surgery, infection, new malignancy, or new

drug therapy. Patients may describe the onset of progressive pain and vi-
sual deterioration over the course of days to weeks. Pain may radiate from
the eye to the face and may be intense and boring in nature. Photophobia,
tearing, and pain with ocular motion may be present. Patients may notice
marked scleral swelling and globe tenderness if the anterior sclera is in-
volved. On examination, swelling may be focal and limited to one part
of the globe or may involve the entire visible sclera. The overlying epis-
cleral and conjunctival vessels may be dilated and inflamed, making the
diagnosis difficult. Engorgement of vessels with a surrounding violaceous
hue is indicative of scleritis. Topical vasoconstrictors will blanch the con-
junctival and episcleral vessels, but the sclera itself should remain swollen
and inflamed. Findings adjacent to the cornea may also show a keratitis.
Areas of necrosis should raise the possibility of an infectious cause of
scleritis, including tuberculosis. Necrotizing scleritis will also reveal areas
of capillary nonperfusion and represents an immediate threat to globein-
tegrity [8,9].
In patients who do not have involvement of the anterior sclera, posterior
scleritis is easily missed. Patients may have similar presenting symptoms and
may also have proptosis. Alternatively, patients may present with minimal
pain, complaining only of visual loss in a rare variant of necrotizing poste-
rior scleritis. Concomitant, secondary uveitis, either anterior or posterior, is
not uncommon and the ED practitioner can easily be fooled into thinking
this is the primary diagnosis. Retinal detachment or other retinal pathology
may also accompany posterior scleritis, but the painful nature of the condi-
tion should initiate a more thorough workup. Diagnosis by ultrasound is
preferred. CT with intravenous contrast may be helpful in these cases and
can show a thickened scleral component. MRI is also useful but is not rou-
tinely available in the ED [8,9,11].
One half of all cases of scleritis are associated with systemic disease. The
most commonly associated disease is rheumatoid arthritis. Other associa-
tions include Wegener’s granulomatosis, relapsing polychondritis, systemic
lupus erythematosus, and polyarteritis nodosa. Workup for these conditions
can begin in the ED or with the patient’s primary care provider. All patients
should have screening tests for syphilis. Tuberculosis and Hansen’s disease
can also cause a granulomatous scleritis.
Complications of scleritis include necrosis, scleral thinning, and visual
loss. Treatment with oral indomethacin is the standard of care in patients
who have active anterior scleritis. Necrotizing or posterior scleritis should
be treated in concert with ophthalmologic consultation; treatment should in-
clude systemic and intraocular steroid injection. If disease is mild, with no
tenting of the sclera, and with no secondary iritis or uveitis, treatment can
be deferred, with close ophthalmologic follow-up. If an infectious cause is
suspected (postsurgical patients, necrosis, immunocompromised hosts),
treatment decisions should be made with an ophthalmologic consultant.
Antibiotic penetration into the sclera is generally poor, owing to its avascu-
lar nature [8,9].
Keratitis
Inflammation of the cornea with or without violation of its epithelium
constitutes keratitis. Patients will present with an acutely red, painful eye
and often complain of foreign body sensation, photophobia, tearing, and vi-
sion change. Infectious causes may be associated with secondary lid edema,
conjunctival reaction, hypopyon, and anterior chamber reaction. Photopho-
bia is due to ciliary spasm. Keratitis is most often viral or bacterial in origin,
but exposure to intense ultraviolet light, chemicals, and contact lenses may
be implicated. Contact lens use itself imparts a 10-fold risk of developing an
infectious keratitis. Corneal abrasions may accompany (or mimic) a keratitis
because of excessive rubbing or scratching of the affected eye. Prompt diag-
nosis, treatment, and identification of cause are paramount to prevent vision
loss due to ulceration, necrosis, and scarring [12,13].
Patients should receive a full ophthalmologic examination. The presence
of corneal opacification, ulceration, hypopyon, or other irregularities
strengthen the diagnosis. Fluorescein staining is essential to evaluate for
epithelial disruption, and its pattern can also help reveal the cause.
Viral keratitis
The herpesviridae (herpes simplex virus [HSV]-1, HSV-2, varicella zoster
virus [VZV], Epstein-Barr virus [EBV], cytomegalovirus [CMV]) are the
viral agents most commonly associated with keratitis. Their clinical presen-
tations and treatments have considerable overlap.
HSV can present with ocular involvement in primary or recurrent infec-
tions. Primary infections often occur in neonates (by way of maternal deliv-
ery with active lesions) or at 6 months of age (by way of saliva), when
maternal antibodies no longer protect the infant from HSV transmission.
A follicular conjunctivitis with the presence of lid or periorbital vesicles
should alert the clinician to HSV infection. Slit lamp examination may
show staining of the cornea in a dendritic pattern. Ophthalmologic antivi-
rals and systemic antivirals (acyclovir) should be initiated promptly. Corneal
involvement may lag behind and persist after conjunctival and dermatologic
involvement.
Most HSV pathology is caused by recurrent disease and may present
without dermatologic findings. Patients may complain simply of eye pain,
redness, and change in vision. Slit lamp examination should reveal a corneal
ulcer with fluorescein uptake in a characteristic dendritic branching pattern
that extends outward. Sometimes, the ulcer alone may stain with fluorescein,
without staining of the advancing dendritic cells. In these cases, ophthalmo-
logic consultation for rose bengal or other dye staining will reveal the
64 MUELLER & MCSTAY
pattern. Advanced or recurrent disease may lead to stromal (subepithelial

corneal matrix) involvement with opacification, thinning, and edema present
on slit lamp examination [14].
The cornea should be debrided of infected cells using a cotton applicator,
starting with the ulcer. This procedure can be done by the ED practitioner
or, preferably, by the ophthalmologic consultant. Pharmacologic treatment
of HSV keratitis consists of ophthalmic antiviral drops (acyclovir or idoxur-
idine) or systemic antiviral medication (oral acyclovir). ED ophthalmologic
consultation is indicated and close follow-up or admission is appropriate.
Topical drops may be associated with corneal toxicity and could prolong
healing, so some clinicians prefer oral agents. Some research suggests that
topical ganciclovir gel may also be effective. Daily ophthalmologic follow-
up or admission is indicated (Fig. 4) [14,15].
Reactivation of herpes zoster virus in the trigeminal distribution can
cause significant ocular disease, including a keratitis. Zoster is often pre-
ceded by a viral prodrome of malaise, fever, headache, and neuralgia. Ocu-
lar and periorbital disease can then follow, with the conjunctiva, lid, and
periorbital skin showing crops of vesicular lesions. The rash is typically uni-
lateral, does not cross the midline, and involves only the upper portion of
the lid. The presence of vesicular lesions on the tip of the nose (nasociliary
branch of the ophthalmic division of trigeminal V1), or Hutchinson’s sign,
should prompt the clinician to evaluate the cornea for involvement. Disease
can be marked and conjunctival vesicles with scarring can occur [16].
Corneal involvement usually occurs after other manifestations and may
present after other symptoms have resolved, or separately from them. Fluo-
rescein staining will reveal dendritic branching that is thicker and more
rope-like than the lace-like pattern of HSV keratitis. Terminal bulbs, present
in HSV keratitis, should be absent in VZV. Fluorescein uptake is much less
pronounced on the VZV dendrites, so inspection under slit lamp must be
Fig. 4. HSV keratitis with characteristic dendritic pattern of staining. (From Goldman L,
Ausiello D, editors. Cecil textbook of medicine. 22nd edition. Philadelphia: Saunders (an im-
print of Elsevier); 2004; with permission.)
careful so as to avoid missing the lesions. Complications include uveitis and

keratitis (even bacterial coinfection). Ophthalmologic consultation is indi-
cated to start oral versus topical antivirals. Steroids are controversial and
should not be started by the emergency practitioner [15,16].
EBV and CMV can also cause a keratitis, but this is much more likely in
the immunocompromised host. A dendritic corneal branching pattern is of-
ten seen with EBV and the conjunctiva and soft tissue may be affected as in
HSV infection. Diagnosis is made by way of polymerase chain reaction of
infected cells. Supportive care is indicated; the use of systemic antivirals is
controversial. In the ED, where it will be difficult to distinguish cause, start-
ing the patient on oral acyclovir may be appropriate. CMV keratitis has
been reported but is uncommon. Its corneal findings are almost identical
to VZV [17].
Bacterial keratitis
The propensity to cause visual loss makes bacterial keratitis an ophthal-
mologic emergency. Its incidence is increasing in the setting of contact lens
use. Most cases are caused by staphylococcal species, but in contact lens
wearers, pseudomonas species may predominate. Both organisms have in-
creased because of contact lens use; in the non–contact lens wearer, strepto-
coccal species are common. In immunocompromised patients, Moraxella
catarrhalis should be considered. Patients will complain of pain, tearing,
and decreased vision. N gonorrohae and C trachomatis should be considered
in the sexually active patient, particularly if conjunctivitis is present. Depend-
ing on the causative organism and the condition of the underlying cornea,
physical examination may reveal a corneal ulcer, with or without surrounding
stromal involvement. The overlying epithelium may be absent. The cornea
will often be opacified and edematous, and hypopyon may be present. The
surrounding conjunctiva may show injection with vessel dilatation [18].
Topical aminoglycosides, quinolones, or cephalosporins should be used,
based on history and gram stain. If gram stain is unavailable or the causative
organism is unlikely to be discovered in the ED, broad-spectrum coverage
using two antibiotics should be initiated with rapid alternating of agent
(eg, two drops aminoglycoside followed by two drops of cephalosporin every
5 minutes for the first hour followed by applications every 15 minutes to 1
hour around the clock). Alternatively, quinolone monotherapy may be effec-
tive. ED ophthalmologic consultation should be obtained and the patient
admitted [18].
Keratitis due to light exposure

Prolonged exposure to ultraviolet light, or brief exposure to intense ultra-
violet flashes, can produce a photokeratitis or photokeratoconjunctivitis of
noninfectious origin. Patients may complain of eye pain, change in vision,
66 MUELLER & MCSTAY
and redness. The staining pattern is generally punctuated with some degree of
corneal opacification. Ancillary signs of infection (purulent discharge, cellu-
litic changes) are generally absent. In punctuate keratitis due to welding expo-
sure, symptoms begin hours after exposure. In patients who have had
prolonged ultraviolet exposure (ie, snow blindness), time to onset may be vari-
able [19]. Topical antibiotics are indicated to prevent bacterial superinfection.
Uveitis
Understanding the anatomy of the uvea is essential in diagnosing and
treating the different forms of uveitis. The uvea is the middle eye. Anteriorly,
this includes the iris and ciliary body, with the posterior reflection of these
structures known as the choroid. Inflammation of the anterior structures
presents with anterior chamber reaction and is easily seen and diagnosed in
the ED. Inflammation of the anterior chamber (anterior uveitis) is a much
more common presentation in the ED than middle or posterior involvement.
Posterior involvement is synonymous with retinitis. Inflammation of the
uveal structures can be a primary, idiopathic process, or can occur secondary
to other ophthalmologic infections or inflammatory conditions.
Anterior uveitis
Patients who have anterior uveitis (commonly called iritis) can present
with a remarkable range of complaints, from mild visual disturbance to
severe pain and loss of vision. Approximately 50% of cases are considered
idiopathic and the next most common cause is thought to be HLA-B27
associated. Patients typically present with eye pain, redness, decreased
vision, photophobia, and headache. The symptoms may mimic glaucoma,
which must be ruled out first by measuring intraocular pressure. When pres-
ent, scleral injection and anterior chamber cells and flare make the diagnosis
clear. The limbus is characteristically injected (known as ciliary flush) and
the pupil is often constricted. In idiopathic anterior uveitis, small keratic
precipitates may be present in the anterior chamber. Larger, granulomatous
or clumpy precipitates should lead the physician to consider specific causes,
including syphilis, toxoplasmosis, and tuberculosis. Hypopyon, when pres-
ent, should prompt the clinician to think of HLA-B27–associated disease,
and referral to a rheumatologist is appropriate [20].
In patients who have HIV, the antibiotic rifabutin and the antiviral cido-
fovir should be considered as possible causes. Both have been associated
with anterior uveitis, and withdrawal of the drug, or reduction of the
dose, is often enough to stop or reduce symptoms [21]. Anterior chamber
reaction can also be secondary to many of the other conditions discussed
in this article, including scleritis, episcleritis, and posterior segment disease.
Autoimmune diseases, such as sarcoidosis, are also associated with anterior
chamber reactions (Fig. 5).
Fig. 5. Acute anterior uveitis with hypopyon (arrow). (From Munoz-Fernandez S, Martin-Mola
E. Uveitis. Best Pract Res Cl Rh 2006;20(3):487–505; with permission.)
The ED workup will be dictated by the patient presentation. Individuals

with only mild complaints without visual disturbance could be referred for
ophthalmology follow-up. Those who have acute onset and severe symp-
toms may require more thorough testing in the ED, including chest radiog-
raphy, angiotensin-converting enzyme, and syphilis and tuberculosis testing.
The common treatment for uncomplicated anterior uveitis is topical cor-
ticosteroid drops, which could potentially be started from the ED in con-
junction with ophthalmology consultation. The addition of scopolamine
or cyclopentolate drops may help decrease the pain associated with ciliary
spasm. Disease that does not improve, or worsens, in the setting of steroid
treatment should alert the practitioner to an infectious cause, particularly
tuberculosis.
Intermediate uveitis
Patients who have blurry vision or floaters, mild pain, and redness but
without elevated intraocular pressure or anterior chamber findings may
have intermediate uveitis. Intermediate uveitis comprises inflammation of
the anterior vitreous humor, the peripheral retina, or the pars plana ciliaris
(the posterior aspect of the ciliary body). Examination will reveal anterior
vitreous floaters and inflammatory cells, absence of anterior chamber cells,
and snowbank-like exudates heaped at the inferior pars plana (in patients
who have pars planitis). The diagnosis of pars planitis may be difficult to
make because most ED practitioners are not familiar with scleral depres-
sion, which may be required to see the heaped exudates. Concomitant mac-
ular edema, cataract formation, or retinal detachment are the most common
complications, in descending frequency. Disease is often bilateral and pres-
ents in patients in the first half of life; presentation in the elderly should raise
the specter of human T-cell lymphoma virus–associated disease.
68 MUELLER & MCSTAY
The association of uveitis with systemic disease should prompt investiga-

tion based on patient age and comorbidities. All patients who have not been
evaluated for sarcoidosis should receive a chest radiograph as a screening
test. Screening for syphilis is recommended. Its association with multiple
sclerosis and Lyme disease has been documented. Still, most cases are idio-
pathic in origin.
Treatment should be initiated in concert with ophthalmologic consulta-
tion, and includes intraocular and systemic corticosteroids. Further ophthal-
mologic treatment is beyond the scope of the emergency physician.
Posterior uveitis and retinitis

Posterior uveitis and retinitis can be thought of interchangeably. The ED
practitioner should be aware of the causes of acute retinitis. For the pur-
poses of this article, retinitis pigmentosa and other slowly progressive causes
of retinal deterioration are not discussed.
In the immunocompromised host (eg, patients who have AIDS, or who
are on chemotherapy or receiving immunosuppressive treatment), progres-
sive, painless vision loss should alert the practitioner to the possibility of
CMV-induced retinitis. Patients may also complain of floaters and light
flashes. CMV retinitis is a marker of profound immune suppression and
rarely presents in AIDS patients unless the CD4 count is very low, but oc-
casionally is the presenting symptoms of undiagnosed HIV infection [22].
Examination will generally reveal a necrotic retinitis with whitening. Hem-
orrhages may be present at the advancing edge of the lesion. Other causative
organisms should be considered in the immunocompromised patient,
including tuberculosis, toxoplasmosis, syphilis, and other herpesviridae.
In the immunocompromised patient who has suspected CMV retinitis, acute
or subacute visual loss does not preclude the diagnosis because retinal de-
tachment is a well-described complication. Diagnosis of CMV retinitis war-
rants hospital admission and initiation of treatment with ganciclovir.
HSV and VZV can also cause a retinitis. Their pattern of retinal involve-
ment and clinical presentation vary, depending on the host’s immune status.
In the immunocompromised host, HSV and VZV cause a rapidly progres-
sive retinal necrosis without evidence of vasculitis and with minimal vitreous
inflammatory changes. The pattern of retinal necrosis may be patchy and it
may begin at the periphery, making diagnosis difficult for the ED practi-
tioner. These viruses can also cause an acute retinal necrosis in the immuno-
competent patient, with a marked vitritis and with a retinal vasculitic
appearance. Eventually, retinal detachment occurs. Treatment for both is
with intravenous acyclovir [23].
Hordeolum and chalazion

Patients who present complaining of acute onset of pain, focal swelling,
and lid edema should be evaluated for hordeolum. A hordeolum represents
purulent infection of a cilium and adjacent gland with local abscess forma-
tion. They can be either present at the lid margin or contained in the lid it-
self. The resulting abscess can point toward either the mucosa (internal) or
the skin (external surface). They will generally swell and then ultimately re-
solve on their own within a week. Staphylococcal species are the most com-
mon causative organism. Cellulitis may accompany the hordeolum.
Treatment is conservative, with warm compresses several times a day for
10 minutes. If this fails, an incision and drainage may be performed.
Some sources cite the need for topical antibiotics to prevent local complica-
tions, but their use is controversial.
Chalazions are granulomatous inflammatory lesions present on the lid
that occur from obstruction of a sebaceous gland (Fig. 6) [24]. They may
arise acutely or persist and recur after initial formation. Clinically, they
are often indistinguishable from hordeolum and there is little reason to sep-
arate them. Indeed, a hordeolum may occur acutely in the setting of a cha-
lazion. They may resolve spontaneously, similar to a hordeolum, with
a more sebaceous, waxy drainage. Recurrent chalazions need ophthalmo-
logic referral for biopsy and evaluation for malignant origin [24].
Dacryocystitis
Acute infection of the lacrimal sac will present as pain, swelling, and er-
ythema overlying the sac. Dacryocystitis is the result of obstruction of the
nasolacrimal duct and resultant purulent infection. Staphylococcal species
are the most common causative organisms. It is managed conservatively
initially with warm compresses and massage of the infected sac to encour-
age drainage of purulent material through the puncta. Oral and topical
antistaphylococcal antibiotics are also indicated. Prompt ophthalmologic
Fig. 6. Bilateral chalazion. (From Goldman L, Ausiello D, editors. Cecil textbook of medicine.
22nd edition. Philadelphia: Saunders (an imprint of Elsevier); 2004; with permission.)
70 MUELLER & MCSTAY
follow-up is indicated to evaluate for response to therapy. Patients who

have signs of systemic illness should be hospitalized (Fig. 7).
In cases of impending rupture, incision and drainage are indicated,
which, in the ED, should be done by an ophthalmologist, if possible. Aspi-
ration with a high-gauge needle before incision and drainage should be per-
formed and sent for culture. Incision and drainage is then performed in
a vertical fashion, and purulent contents curetted. Prompt ophthalmologic
referral is then indicated for follow-up and potential surgical intervention
[24]. Acute dacryocystitis may progress to periorbital or orbital cellulites,
depending on direction of spread and host factors. These patients should
be managed as outlined in the next section.
Periorbital and orbital cellulitis

Infection of the periorbital and orbital soft tissues may represent exten-
sion of a primary infection, or may accompany other ocular pathology. Su-
perficial anterior infection, with involvement limited by the ocular fascia,
presents as upper or lower lid edema, pain, and warmth. Patients may
have systemic symptoms (eg, fever), but change in vision should be absent
and the pupil examination and intraocular pressures normal. Ocular range
of motion should be essentially intact, with little pain. Treatment is with
antistaphylococcal antibiotics, and selected cases may be managed in the
outpatient setting. Amoxicillin-clavulanate or a suitable cephalosporin
may be used (eg, cefpodoxime) [24,25].
Involvement of the orbital soft tissues is much more serious, with signif-
icant chance of permanent ocular damage and potentially fatal infectious
consequences. Patients will present with marked swelling, pain, and edema,
Fig. 7. Acute dacryocystitis. (From Goldman L, Ausiello D, editors. Cecil textbook of medi-
cine. 22nd edition. Philadelphia: Saunders (an imprint of Elsevier); 2004; with permission.)
but symptoms will be more pronounced. Proptosis may be present, and the
eye may exhibit an afferent papillary defect. Extraocular motion may be ei-
ther limited or associated with pain. Intraocular pressure may be elevated be-
cause of external compression. CT is indicated to aid in diagnosis and to
evaluate for abscess or soft tissue extension. Periorbital swelling should be
evident on CT, and contrast enhancement may aid in abscess identification.
Most cases of orbital cellulitis represent an extension of sinusitis. Staph-
ylococcal species are the most common pathogen, but other gram-positive
organisms should be considered when starting antibiotics. Pseudomonas
should also be considered in recently hospitalized, instrumented, or immu-
nocompromised patients. The remaining cases are caused by primary infec-
tion of the lid or face, or by a foreign body. A recent case series in the
pediatric population showed increasing presence of methicillin-resistant
Staphylococcus aureus in orbital cellulitis, so vancomycin should be consid-
ered [24–26].
Ophthalmologic consultation and admission is indicated for cases of or-
bital cellulitis. The presence of proptosis, a dilated pupil, or vision loss por-
tends a worse clinical course. Sinus drainage is often performed. Other
complications, including cavernous sinus thrombosis and meningitis, have
been described.
References
[1] Rubenstein JB, Jick SL. Cornea and external disease. In: Yanoff M, Duker JS, editors.
Yanoff opthalmology. 2nd edition. Philadelphia: Mosby; 2004. p. 398–404.
[2] Kaufman HE. Treatment of viral diseases of the cornea and external eye. Prog Retin Eye Res
2000;19(1):69–85.
[3] Rietveld RP, ter Riet G, Bindels PJ, et al. Predicting bacterial cause in infectious conjuncti-
vitis: cohort study on informativeness of combinations of signs and symptoms. BMJ 2004;
329:206–10.
[4] Sheikh A, Hurwitz B. Antibiotics versus placebo for acute bacterial conjunctivitis. Cochrane
Database Syst Rev; 2006.
[5] Everitt HA, Little PS, Smith PW. A randomised controlled trial of management strategies
for acute infective conjunctivitis in general practice. BMJ 2006;333:321–4.
[6] Wan WL, Farkas GC, May WN, et al. The clinical characteristics and course of adult gon-
ococcal conjunctivitis. Am J Ophthalmol 1986;102:575–83.
[7] Woods CR. Gonococcal infections in neonates and young children. Semin Pediatr Infect Dis
2005;16(4):258–70.
[8] Jabs DA, Mudun A, Dunn JP, et al. Episcleritis and scleritis: clinical features and treatment
results. Am J Ophthalmol 2000;130:469–76.
[9] Okhravi N, Odufuwa B, McCluskey P, et al. Scleritis. Surv Ophthalmol 2005;50:351–63.
[10] Williams CP, Browning AC, Sleep TJ, et al. A randomised, double-blind trial of topical
ketorolac vs artificial tears for the treatment of episcleritis. Eye 2005;19:739–42.
[11] McCluskey PJ, Watson PG, Lightman S, et al. Posterior scleritis: clinical features, systemic
associations, and outcome in a large series of patients. Ophthalmology 1999;106:2380–6.
[12] Keay L, Edwards K, Naduvilath T, et al. Microbial keratitis: predisposing factors and mor-
bidity. Ophthalmology 2006;113:109–16.
[13] Titiyal JS, Negi S, Anand A, et al. Risk factors for perforation in microbial corneal ulcers in
north India. Br J Ophthalmol 2006;90:686–9.
72 MUELLER & MCSTAY
[14] Kaye S, Choudhary A. Herpes simplex keratitis. Prog Retin Eye Res 2006;25:355–80.
[15] Tabbara KF. Treatment of herpetic keratitis. Ophthalmology 2005;112:1640–1.
Physician 2002;66(9):1723–30.
[17] McLeod SD. Infectious keratitis. In: Yanoff M, Duker JS, editors. Yanoff ophthalmology.
2nd edition. Philadelphia: Mosby; 2004. p. 466–91.
[18] Thomas PA, Geraldine P. Infectious keratitis. Curr Opin Infec Dis 2007;20(2):129–41.
[19] Cullen AP. Photokeratitis and other phototoxic effects on the cornea and conjunctiva. Int
J Toxicol 2002;21(6):455–64.
[20] Gutteridge IF, Hall AJ. Acute anterior uveitis in primary care. Clinical Exp Optom
2007;90(2), 70–82.
[21] Bhagat N, Read RW, Rao NA, et al. Rifabutin-associated hypopyon uveitis in human
immunodeficiency virus–negative immunocompetent individuals. Ophthalmology 2001;
108:750–2.
[22] Pertel P, Hirschtick R, Phair J, et al. Risk of developing cytomegalovirus retinitis in persons
infected with the human immunodeficiency virus. J Acquir Immune Defic Syndr 1992;5:
1069–74.
[23] Ganatra JB, Chandler D, Santos C, et al. Viral causes of acute retinal necrosis syndrome.
Am J Ophthalmol 2000;129:166–72.
[24] Wald ER. Periorbital and orbital infections. Pediatr Rev 2004;25(9):312–20.
[25] Starkey CR, Steele RW. Medical management of orbital cellulitis. Pediatr Infect Dis J 2001;
20:1002–5.
[26] McKinley SH, Yen MT, Miller AM, et al. Microbiology of pediatric orbital cellulitis.
Am J Ophthalmol; 2007;144(4):497–501.
Emerg Med Clin N Am
26 (2008) 73–96
Acute Monocular Visual Loss

Michael Vortmann, MD, Jeffrey I. Schneider, MD*
Department of Emergency Medicine, Boston University School of Medicine, Boston Medical
Center, Dowling 1 South, 1 Boston Medical Center Place, Boston, MA 02118, USA
Temporal arteritis
Temporal arteritis is a medium- and large-vessel vasculitis that affects the
extracranial branches of the carotid artery. Its presentation can vary from
that of a chronic headache to sudden monocular loss of vision. Confirma-
tion of the diagnosis requires a temporal artery biopsy and the cornerstone
of treatment is the administration of high-dose steroids. Sudden loss of vi-
sion attributable to temporal arteritis constitutes an ophthalmologic emer-
gency; prompt recognition of the disorder and institution of therapy can
prevent further vision loss in the affected eye or new visual deficits in the
contralateral eye.
Epidemiology
Temporal arteritis is the most common primary vasculitis among the el-
derly with an annual incidence of approximately 18 per 100,000 in people
older than 50 years. Peak incidence occurs between 70 and 80 years of age
with a 2:1 female to male ratio. Of those affected, 88% are white [1]. Certain
human leukocyte antigen (HLA) types have been found to entail an in-
creased risk for developing temporal arteritis [2]. Although discussion of
the relationship between temporal arteritis and polymyalgia rheumatica is
beyond the scope of this article, individuals who have these same HLA types
are at an increased risk for developing both of these disorders [2,3].
Etiology
Although the exact inciting event of temporal arteritis is poorly under-
stood, and no causal relationship has been established, there is some evi-
dence that infection may play a role, specifically parvovirus B19,
E-mail address: [email protected] (J.I. Schneider).
74 VORTMANN & SCHNEIDER
Mycoplasma pneumoniae, and Chlamydia pneumoniae [4,5]. CD4 cells, re-

sponding to an unknown antigen, migrate through all three layers of the af-
fected artery and initiate an inflammatory cascade. This inflammation
causes a reactive proliferation of the intimal layer of the artery, with result-
ing narrowing of the arterial lumen and ischemia distal to the lesion [6]. The
occlusion of end-arteries to the eye, scalp, tongue, and muscles of mastica-
tion causes the blindness, jaw and tongue claudication, and scalp ischemia
associated with the disease. The inflammation also produces cytokines,
which are believed to be responsible for the frequent low-grade fever and
constitutional symptoms, such as anorexia, malaise, and weight loss, that
are often associated with the disease [7]. Thrombotic occlusion of the ar-
teries, however, does not seem to play a role in the pathogenesis. Granulo-
matous inflammation forms giant cells in the classic pathologic lesion and
gives the disease its alternate name, giant cell arteritis [6].
Clinical features
The most common clinical manifestation of temporal arteritis is head-
ache, seen in two thirds of patients and generally located in the area of
the temporal or occipital arteries [8,9]. Systemic symptoms, such as low
grade fever, chills, malaise, anorexia, and weight loss, are present in one
half of patients [8,10]. Other associated symptoms include jaw claudication,
tongue pain, and mandibular pain [10]. Visual complaints are also common
with partial or complete vision loss in one or both eyes occurring in up to
20% of patients as a presenting symptom of disease [9–12]. Amaurosis fugax
precedes vision loss in 44% of affected individuals; diplopia and visual hal-
lucinations are less common, but may also precede vision loss [11–13].
Thirty percent of patients have neurologic manifestations that may include
mononeuropathies, peripheral polyneuropathies, and occasionally TIAs or
strokes [14,15].
Physical examination should include temporal and occipital artery palpa-
tion searching for nodular or firm, tender arteries with overlying erythema.
Joint examination may reveal polyarticular pain with movement or synovitis,
especially of the shoulders and hip. This finding may suggest polymyalgia
rheumatica, which is associated with temporal arteritis [16]. The eye examina-
tion should include visual acuity, assessment of pupils for an afferent pupillary
defect, and a funduscopic examination to evaluate the retina and optic disks
[17]. Typical funduscopic findings in those who have visual loss from temporal
arteritis include pallor and edema of the optic disk with scattered cotton-wool
patches and small hemorrhages (Fig. 1) [18]. Laboratory evaluation may re-
veal elevated C-reactive protein and erythrocyte sedimentation rate (ESR),
a normocytic anemia, and a reactive thrombocytopenia. One may also see
mild elevations in the liver enzymes [16].
A 2002 meta-analysis [19] that included 41 studies and more than 2600
patients evaluated the accuracy of history, physical examination, and ESR
ACUTE MONOCULAR VISUAL LOSS 75
Fig. 1. Giant cell arteritis. (From Kaiser PK, Friedman NJ, Pineda R, II. The Massachusetts
Eye and Ear Infirmary illustrated manual of ophthalmology. 2nd edition. China: Saunders;
2004; with permission.)
in predicting a diagnosis of temporal arteritis. The only two historical fea-

tures found to appreciably increase the likelihood of temporal arteritis
were jaw claudication and diplopia. Other visual symptoms, including mon-
ocular vision loss, were not found to be helpful in distinguishing temporal
arteritis from other causes of sudden vision loss.
On physical examination, the presence of any abnormality of the tempo-
ral artery (beading, prominence, tenderness) was most predictive of tempo-
ral arteritis. Conversely, the absence of any of these findings reduced the
likelihood of the diagnosis substantially. Evidence of optic atrophy or any
funduscopic abnormality was not helpful in establishing or eliminating the
diagnosis, because the end result of retinal ischemia is common to many
causes of sudden vision loss [19].
A normal ESR, defined as age in years divided by 2 for men, and age in
years plus 10 divided by 2 for women, conferred a negative likelihood ration
of 0.2. Any ESR of less than 50 conferred a reduced probability of having
the disease. An ESR greater than 100, although increasing the likelihood
of temporal arteritis, was less predictive than the previously described his-
torical features and physical examination findings. Similarly, the mean
ESR for patients who had biopsy-negative and biopsy-positive temporal ar-
teries was not statistically different. CRP was not evaluated in the meta-
analysis. Other laboratory abnormalities, such as anemia, were not helpful
in distinguishing those who had temporal arteritis [19].
Diagnosis and treatment

Suspicion of temporal arteritis should trigger rheumatology and ophthal-
mology consultation, especially in the setting of acute vision loss. The crite-
ria for diagnosis of temporal arteritis as defined by the American College of
Rheumatology include: age greater than 50 years, new headache, temporal
artery abnormality, ESR greater than 50, and abnormal findings on a tempo-
ral artery biopsy. The presence of three or more criteria conferred
a sensitivity of 93.5% and a specificity of 91.2% for the diagnosis [20]. Char-
acteristic giant cell granulomatous inflammation on temporal artery biopsy
is considered the gold standard to confirm the diagnosis, however. Doppler
ultrasound of the temporal arteries has been studied as a noninvasive ad-
junct to biopsy [21]. A sensitivity of 95% has been reported in biopsy-con-
firmed temporal arteritis when abnormalities in the structure of the arterial
wall were discovered on ultrasound. The specificity is low, however, and
a negative result cannot exclude the need for biopsy [22].
High-dose methylprednisolone is the first-line therapy in patients who
have temporal arteritis who present with ocular manifestations. Three
days of intravenous steroids followed by 2 years of oral prednisone begin-
ning at 40 to 60 mg/d is a common suggested treatment course [16]. Patients
who do not have ocular manifestations are started on oral prednisone with-
out the methylprednisolone burst. Prednisone is gradually tapered in con-
junction with monitoring ESR and CRP levels for change. Any increase
in either halts any further reduction in prednisone level [18,23]. CRP has
been shown to be more sensitive than ESR at diagnosis and during monitor-
ing of relapse [24]. Various dosing protocols exist, but none have been pro-
spectively validated.
Unfortunately, the visual loss associated with temporal arteritis is often
permanent. In one retrospective study in patients who had biopsy-proven
disease, central vision improvement after the initiation of steroid therapy
was minimal, and was present in only 4% of patients [23]. Corticosteroids,
however, do seem to prevent further vision loss; only 13% of those who had
vision loss before diagnosis developed further vision loss after initiation of
steroid therapy. Similarly, of those who had no visual complaints before ste-
roid treatment, only 1% subsequently developed vision loss [11].
Optic neuritis
Optic neuritis is an acute demyelinating disorder of the optic nerve that
typically presents as painful, monocular vision loss. Although most affected
patients regain vision even without treatment, a substantial number subse-
quently develop multiple sclerosis (MS). Consequently, emergency physi-
cians often enlist the help of ophthalmologists and neurologists in the
management of these patients.
Epidemiology
Optic neuritis has an incidence of 6.4 per 100,000 with two thirds of cases
occurring in women, generally between 20 and 40 years of age [25]. It is most
common in northern latitudes (the United States and Northern Europe) and
is more often diagnosed in white Americans than African Americans [25,26].
It is the presenting symptom in 15% to 20% of patients subsequently diag-
nosed with MS and occurs in one half of MS patients during the course of
their illness [25,27,28]. In addition, 31% of patients who have optic neuritis
have a recurrence within 10 years of their initial presentation [29].
Etiology
The inflammatory demyelination of the optic nerve is believed to be an au-
toimmune phenomenon characterized by systemic T-cell activation present at
the onset of visual symptoms [30]. Although the specific mechanism is un-
known, inflammatory cytokines are believed to play a role and B-cell activa-
tion against myelin basic protein may be seen in the cerebrospinal fluid of
affected individuals [31]. The inflammatory response against the optic nerve
results in edema and breakdown of the myelin sheaths and perivascular cuff-
ing of the retinal vasculature [32]. Genetic susceptibility for optic neuritis is
suspected based on higher incidences among certain HLA types [33].
Clinical features and diagnosis

Optic neuritis is a clinical diagnosis based on history and physical exam-
ination findings. The Optic Neuritis Treatment Trial [34] surveyed 448 pa-
tients who had optic neuritis about their visual symptoms and performed
detailed visual assessments. Eye pain accompanied vision loss in 92% of pa-
tients. Vision loss was typically monocular and progressed rapidly over a pe-
riod of hours to days. Even patients who had 20/20 vision at presentation
had defects in their ability to perceive color and contrast; patients often de-
scribed their vision as ‘‘blurry’’ or felt the color has been ‘‘washed out’’ [35].
Although physical examination findings may vary in optic neuritis, pain
with eye movements and an afferent pupillary defect are almost universally
present [34]. Even in those patients who have normal visual acuity, mild op-
tic nerve dysfunction causes an asymmetry in the pupillary reflex that can be
elicited by the swinging flashlight test [25]. Visual acuity in the affected eye
can range from 20/20 to no light perception. Although central scotoma is
the classic visual deficit, a wide variety of visual field cuts may occur [34].
Two thirds of patients have a normal funduscopic examination with retro-
bulbar optic neuritis. One third of patients have optic disk swelling, blurring
of disk margins, and swollen veins caused by optic nerve inflammation as it
terminates in the retina (Fig. 2) [34].
Although the diagnosis of optic neuritis is often made on clinical
grounds, gadolinium-enhanced MRI can help confirm the diagnosis and
risk-stratify patients who are likely to develop MS. Optic nerve inflamma-
tion can be demonstrated in 95% of patients who have optic neuritis on
gadolinium-enhanced MRI [36–38]. The imaging may also demonstrate
oval-shaped lesions in the periventricular white matter that suggest MS
[39]. In one study, the risk for MS 10 years after the first episode of optic
neuritis was 56% versus 22% in those who did not have lesions [40]. This
information may be useful for consulting ophthalmologists and neurologists
and may guide the initial therapy of affected patients.
Fig. 2. Optic neuritis. (From Kanski JJ. Clinical Diagnosis in Ophthalmology. China: Mosby
Elsevier; 2006; with permission.)
Treatment
Visual acuity in optic neuritis generally improves without treatment over
the course of several weeks [29]. Treatment is focused on hastening the return
of vision, preventing recurrences, and reducing the incidence of MS. In a study
that randomized affected patients to either high-dose intravenous methyl-
prednisolone or oral prednisone (or placebo), those who were given the meth-
ylprednisolone demonstrated a more rapid return to normal vision [41] and
a lower risk for recurrent optic neuritis. The differences in visual acuity
were not significant at 2 years follow-up, however. Oral prednisone was asso-
ciated with an increased risk for recurrent optic neuritis compared with pla-
cebo. Methylprednisolone delayed the onset of MS compared with placebo at
2 years, but this advantage did not persist beyond this time frame [41]. Ac-
cording to the American Academy of Neurology, although corticosteroids
may hasten the return of vision after initial presentation, there is no compel-
ling evidence for long-term benefit for patients who have optic neuritis [42].
Several randomized trials have demonstrated that interferon beta 1a and
interferon beta 1b may reduce the development of MS in patients who have
optic neuritis [25,39,43,44]. Although this is typically started at the onset of
symptoms, the initiation of this therapy is probably beyond the scope of
emergency physicians and should only be done in conjunction with an in-
volved neurology consultant.
Central retinal artery occlusion

Central retinal artery occlusion (CRAO) generally causes abrupt, painless
loss of vision that can be permanent unless blood flow to the retina is
restored before the onset of irreversible ischemic damage. The retina is

perfused by the retinal artery, a branch of the ophthalmic artery that arises
from the internal carotid artery. The ophthalmic artery enters the orbit
along with the optic nerve at which point the retinal artery branches off,
enters the cerebrospinal space, and travels within the optic nerve where it
provides blood flow to the central retina and optic nerve. In some
patients, additional perfusion of the central retina (including the macula)
is provided by the cilioretinal artery, an anatomic variant that allows for
the preservation of central vision in some who have CRAO.
Epidemiology
Although the true incidence of CRAO is unknown, most estimates are
that it occurs in between 1 to 10 in 100,000 individuals [45]. Risk factors
for the development of CRAO are similar to those of other cardiovascular
diseases, namely increasing age, hypertension, hypercholesterolemia, diabe-
tes, elevated homocysteine levels, and tobacco use. Some studies have de-
scribed an increased incidence in males, whereas others have failed to
demonstrate a difference in disease prevalence in one sex over the other
[46–48].
Etiology
CRAO and other strokelike syndromes share a final common pathway:
interruption of blood flow to distal tissues. Although several causes of
CRAO are described later, the most common are atherosclerotic disease
of the ipsilateral carotid artery [49] and propagation of cardiogenic emboli
to the retinal artery [50]. Several studies have suggested factors such as
patient age and ethnicity may play a role in the distribution of these causes.
For example, whites are more likely to have carotid disease [49], whereas
those less than 40 years of age are more likely to have a cardiogenic
embolus as the source of their symptoms [50], and patients older than
age 70 are more likely to have temporal arteritis as the basis of their
CRAO [49].
Carotid artery atherosclerosis

Atherosclerotic disease of the carotid artery is believed to be the most
common source of emboli resulting in the disruption of retinal artery blood
flow. Although the data are largely from small case series, the prevalence of
carotid artery disease in those who have CRAO is generally believed to be
10% to 25%, although there is literature suggesting rates as high as 70%
[49,51–55]. Additionally, CRAO in the setting of carotid disease may por-
tend an increased risk for stroke; for this reason, carotid endarterectomy
is often recommended in this setting [55].
Cardiogenic embolism
Between 2% and 20% of patients who have CRAO have a cardiac source
of embolus [54,56]. These patients tend to be younger in age, and there may
be aspects of their history that should alert the clinician to consider this as
a possible cause, including: a history of congenital heart disease, rheumatic
heart disease, myocardial infarction, endocarditis, or the presence of a car-
diac tumor or murmur [57]. Chronic anticoagulation may be warranted for
patients who have a cardiogenic source of embolus.
Other causes
Although carotid artery atherosclerosis and cardiogenic embolism
represent the cause of most cases of CRAO, there are several other causes
reported in the literature. These can be divided broadly into other vascular
disease processes (carotid artery dissection [58], radiation injury of the reti-
nal artery [59], Moyamoya disease [60], arterial vasospasm, and migraine)
[61], hematologic disorders (sickle cell disease) [62], disorders resulting in
hypercoagulable states (antiphospholipid syndrome, factor V Leiden muta-
tion [63], protein S deficiency [64], and protein C deficiency) [65], and auto-
immune/inflammatory conditions (giant cell arteritis [66], lupus [67],
polyarteritis nodosa [68], and Wegener granulomatosis) [69]. There are
case reports of all of these causes of CRAO; no large cohort studies have
been published.
Clinical features
The visual loss associated with central retinal artery occlusion is generally
painless, monocular, and dramatic, often leaving the affected individual with
only a small area of unaffected vision. The symptoms may be less severe,
however, if the retina is also perfused by a ciolioretinal artery (15% of pa-
tients in one series) [70]. Similarly, the blindness may be transient, or the pa-
tient may have ‘‘stuttering’’ symptoms if an embolus moves along the
vascular tree and eventually dissolves before causing complete vessel occlu-
sion [71].
On funduscopic examination, the ischemic retina initially appears white
and the macula classically is described as having a ‘‘cherry red spot’’ where
the retinal epithelium is thinner and the retinal pigment epithelium and cho-
roidal vasculature appear more prominent and can be seen more easily
(Fig. 3). In those who have a cilioretinal artery, there may be an area of nor-
mal-appearing retina surrounded by an ischemic, pale retina. The actual em-
bolus may be visible in as many as 40% of patients who have CRAO [72].
The clinician may see shiny, iridescent cholesterol plaques, grayish platelet
deposits, or bright white calcium fragments. There is generally a complete
or relative afferent papillary defect. Finally, funduscopic examination may
reveal a characteristic ‘‘boxcarring’’ in the retinal veins and arteries as serum
separates from the Rouleau stacking of red blood cells.
Fig. 3. Central retinal artery occlusion. (From Wipf JE, Paauw DS. Ophthalmologic emergen-
cies in the patient with diabetes. Endocrinol Metab Clin N Am 2000;29(4):813–29; with
permission.)

The diagnosis of central retinal artery occlusion is made on clinical
grounds in combination with characteristic eye ground findings. It is
a true ocular emergency; retinal ischemia lasting longer than 240 minutes
may lead to massive irreversible vision loss [73], whereas restoration of
blood flow within 100 minutes may preserve a patient’s vision [74]. Although
many of those who have occlusion of a branch retinal artery may regain nor-
mal vision, spontaneous visual recovery in those who have CRAO is rare
and the literature of this population is largely limited to case reports. In
one case series, only 35% of patients recovered vision that was better
than 20/100 [75], whereas in another series of 73 CRAOs, there were only
four instances in which final visual acuity was better than counting fingers
[70]. Visual acuity at presentation is believed to be predictive of eventual
acuity after the acute CRAO.
Although there is literature documenting and describing the debilitating
effects of CRAO, there is a relative paucity of literature examining and com-
paring the effectiveness of several different treatments. Given the poor out-
comes of those afflicted with CRAO, however, several potential therapies
have been advocated. Although some of the more conservative treatments
are within the practice scope of emergency medicine, patients who have sus-
pected CRAO should receive emergent ophthalmologic consultation and
evaluation.
Although many of the therapies vary in their invasiveness and risk, all
share a common goal of restoring blood flow to an ischemic retina. As men-
tioned previously, spontaneous resolution (ie, the embolus dissolving or
moving on from the ophthalmic circulation) is relatively uncommon, occur-
ring in 1% to 8% of cases [45]. Initial therapies involved attempts by
patients or clinicians to manually dislodge the embolus by massaging the

eyeball over a closed lid. It is believed that this may also augment aqueous
outflow and retinal perfusion may increase with relief of the digital pressure
[76,77].
Other therapies have aimed to increase ocular perfusion pressure by de-
creasing intraocular pressure, which may be accomplished by performing
anterior chamber paracentesis [78]; giving intravenous diuretics, such as ac-
etazolamide [79] or mannitol [45]; using enhanced external counterpulsation
(a procedure in which air-filled cuffs are applied to the vascular bed of the
lower extremities during diastole) [80]; or performing a trabeculectomy
[81], in which a fistula is created between the anterior chamber and the sub-
conjunctival space.
Some have suggested treating affected patients with vasodilators with the
hope of increasing retinal blood flow or perhaps even dislodging the culprit
embolus. The use of agents such as nitrates [45], rebreathing carbon dioxide
[82], and inhaling carbogen (95% oxygen and 5% carbon dioxide) [78] have
been met with mixed results because systemic vasodilatation may result,
causing a reduction in systemic blood pressure and a subsequent decrease
in retinal perfusion. Others have advocated for alternative forms of medical
therapy, including various platelet inhibitors, anticoagulants, aqueous for-
mation inhibitors, hyperosmotics, and corticosteroids.
A more aggressive approach involves the use of lytic agents in the
treatment of CRAO. Using principles adapted from the use of thrombo-
lytics in acute myocardial infarction and ischemic stroke, some have
advocated for its use in CRAO. Any benefits, however, must be weighed
against the risk for life-threatening complications (ie, hemorrhage,
especially intracranial) in what is a debilitating, but not necessarily life-
threatening, disease. Several reports involving various agents, including
urokinase and recombinant tissue plasminogen activator (rt-PA), given
both systemically and locally are reported in the literature. A 2000
meta-analysis provides the best summary of these data (although it
examines only 16 studies, all of which were retrospective and nonrandom-
ized and included a total of only 100 patients). The authors concluded
that local intra-arterial fibrinolysis offered a ‘‘marginally better visual out-
come than conservative forms of management’’ [83]. Similarly, a 2005
Cochrane review suggested that ‘‘there is currently not enough evidence to
decide which, if any, interventions for acute nonarteritic central retinal ar-
tery occlusion would result in any beneficial or harmful effect. Well-designed
randomized controlled trials are needed to establish the most effective
treatment’’ [84].
In summary, although a wide variety of medical and surgical treatments
for CRAO have been used, none have been prospectively shown to have
more than a limited or marginal benefit. There are few, if any, randomized
controlled trials of these therapies, and there has been little advancement in
the treatment of this disorder in the last 20 years.
Central retinal vein occlusion

There is much overlap in the epidemiology, causes, clinical features, and
diagnosis and treatment between central retinal artery and vein occlusion.
Below, critical similarities and differences are highlighted.
Epidemiology
Retinal vein occlusion (RVO) is the second most common retinal vascu-
lar disorder behind diabetic retinopathy [85]. Prevalence reports vary,
largely depending on whether the data are taken from a hospital- or popu-
lation-based sample. The prevalence of RVO in hospital-based studies
generally ranges from 0.3% to 1.6% [86,87], whereas a large Israeli popula-
tion-based study reported a 4-year incidence of retinal vein occlusion of 2.14
cases per 1000 of general population older than 40 years and 5.36 cases per
1000 of general population older than 64 years [88]. An Australian popula-
tion-based study reported a 1.6% 10-year incidence of RVO in those older
than 60 years [89].
Etiology
Since the initial description of RVO in the medical literature in 1854, sev-
eral different classification systems have been used. Typically, RVO is first
divided into that affecting a central vein (CRVO) and that affecting a branch
vein (BRVO). Central vein occlusion is further divided into ischemic and
nonischemic types.
BRVO is believed to result in large part from factors related to the ana-
tomic relationship between the retinal vein and artery as they pass through
the region of the lamina cribrosa. In this area, the structures share an adven-
titial sheath and are in close proximity to each other. This factor, in combi-
nation with degenerative changes in the wall of the vein and artery, leads to
a narrowing of the vein lumen and subsequently to stasis and thrombosis.
Ischemic RVO can also occur in any other area of the retina where the ar-
teries and veins cross [90]. Many of the risk factors for the development of
BRVO are similar to those of CRAO, and include hypertension [87,91,92],
diabetes [87,91,93], cerebrovascular disease [91,92], cardiovascular disease
[94], increased body mass index [94], dyslipidemia [94], thyroid disease
[91], peptic ulcer disease [91], glaucoma [95], and hyperhomocysteinuria
[96]. On ophthalmologic examination, those who have focal arteriolar nar-
rowing or arteriovenous nicking are also more likely to develop RVO [89].
CRVO, however, is believed to occur because of clots present in the main
draining vein of the retina. Depending on clot burden, collateral circulation,
and perfusion of the retina, it is divided into ischemic and nonischemic
types. In nonischemic CRVO, the vascular lesion is generally more proxi-
mal, allowing for collateral circulation to provide some blood flow to the
retina. Consequently, visual loss is by and large less severe and debilitating.
In addition, in those eyes that have nonischemic disease, examination may

reveal stasis of blood within the veins as opposed to frank thrombosis [90].
Clinical features
In those who have ischemic RVO, the visual loss is often dramatic and is
frequently noted on awakening one morning. The persistent blurring may be
preceded by episodes of amaurosis fugax in some patients. Conversely, non-
ischemic RVO may be relatively asymptomatic and may only be noted on
routine ophthalmologic examination. Visual symptoms, when present,
may be limited to a vague blurring of central vision with sparing of the pe-
ripheral vision and are often worse in the morning and improve gradually
over the course of the day [90]. In one study, the final visual acuity was
20/400 or worse in 87% of those who had ischemic RVO, whereas it was
20/30 or better in 57% of those who had nonischemic disease [97]. As
with CRAO, long-term visual acuity in those who have ischemic RVO is
largely determined by visual acuity at presentation [98].

Although the formal diagnosis of RVO and the differentiation between
the ischemic and nonischemic varieties is beyond the clinical scope of
most emergency medicine physicians, one should be able to appreciate sev-
eral of the clinical aspects of RVO mentioned earlier, along with some typ-
ical findings on funduscopic examination.
The funduscopic examination classically reveals retinal hemorrhages
(which may be mild or severe), dilated and tortuous retinal veins, edema
of the optic disc, and small areas of yellow-white discoloration of the retina
(‘‘cotton wool spots’’), which are indicative of local retinal ischemia (Fig. 4).
When retinal hemorrhages are severe, and cover much of the fundus, the
Fig. 4. Branch retinal vein occlusion Arrows represent sites of branch retinal vein occlusion.
(From Kaiser PK, Friedman NJ, Pineda R, II. The Massachusetts Eye and Ear Infirmary
illustrated manual of ophthalmology. 2nd edition. China: Saunders; 2004; with permission.)
clinician may see the classic ‘‘blood and thunder’’ appearance of the retina
(Fig. 5).
RVO is generally a self-limited disease (the retinopathy may resolve over
weeks to years), but the complications of the disorder can be severe. Al-
though there is little that an emergency medicine physician can do acutely
for affected patients, referral to a specialist is of paramount importance.
In addition to making a formal diagnosis, ophthalmologists should differen-
tiate between ischemic and nonischemic disease, a distinction that has im-
portant ramifications in the complications that may be anticipated.
Specifically, those who have ischemic disease are at a higher risk for devel-
oping ocular neovascularization, which may result in severe vision loss. A
detailed discussion of this complication is beyond the scope of this article.
As with CRAO, various therapeutic strategies have been used with vary-
ing levels of success. Interventions, such as anticoagulation, lytic agents, he-
modilution, hyperbaric oxygen, surgical decompression, and systemic and
intravitreal steroids, are all reported in the literature. A recent meta-analysis
reported that although hemodilution seemed to be of some benefit in some
trials, and panretinal photocoagulation may improve visual acuity in those
who have neovascularization, there is ‘‘limited level I evidence for any inter-
vention to improve visual acuity in patients with RVO’’ [99]. Most studies
are case reports, retrospective, do not have a control group, or involve
very small numbers of patients.
Retinal detachment
Epidemiology
Retinal detachment is a relatively uncommon affliction of the eye, affect-
ing approximately 1 to 2 in 10,000 people per year [100,101], or about 1 in
Fig. 5. Central retinal vein occlusion. (From Kaiser PK, Friedman NJ, Pineda R, II. The
Massachusetts Eye and Ear Infirmary illustrated manual of ophthalmology. 2nd edition. China:
Saunders; 2004; with permission.)
300 people over the course of their lifetime [102]. Risk factors for the devel-
opment of retinal detachment include increasing age [103], previous cataract
surgery [104], focal retinal atrophy, myopia [105], trauma [106], diabetic ret-
inopathy, family history of retinal detachment, uveitis, and prematurity
[107].
Etiology
Although the pathogenesis of retinal detachment can be divided into
three subtypes, all share a common final pathway: separation of the retina
from the underlying retinal epithelium. In the first type, exudative retinal de-
tachment, there is accumulation of serous or hemorrhagic fluid in the sub-
retinal space, generally as a result of systemic conditions, such as severe
acute hypertension, sarcoid, or cancer. In the second type, tractional retinal
detachment, previous trauma from infection, surgery, inflammation, or
hemorrhage results in fibrotic tissue that can provide traction on the retina
resulting in detachment [107–109].
The third type, rhegmatogenous detachment, is the most commonly en-
countered. In this form of retinal detachment, age-related changes in the vit-
reous humor cause it to liquefy and shrink away from the back of the eye
(termed posterior vitreous detachment). In turn, the vitreous may pull the
retina from its underlying epithelial layer. Although patients who have pos-
terior vitreous detachment often only experience bothersome floaters in their
peripheral vision, more dramatic vision loss can occur if a retinal tear or flap
occurs. As the vitreous pulls away from the retina, the vitreous is able to en-
ter the subretinal space and create a plane of dissection. As more vitreous
enters this space, more retina is separated from its underlying epithelial
layer. As the detachment grows, so does the patient’s visual field deficit.
Most rhegmatogenous detachments, if left untreated, expand to eventually
include the macula, impacting central visual acuity [107–109].
The aforementioned risk factors for the development of retinal detach-
ment all result in either changes in the makeup of the vitreous or in alter-
ations in the normal anatomy or shape of the eyeball, resulting in retinal
traction and forces that tend to pull the retina from the epithelium layer
it normally covers.
Clinical features
The hallmark complaint of those who have vitreous detachment is the
presence of floaters. Characteristically described as dots, cobwebs, lines,
or strings in the visual field, the floaters of vitreous detachment (especially
with an associated retinal tear) tend to occur abruptly. As the vitreous de-
tachment progresses to retinal detachment, patients may describe visual field
loss. This loss generally begins at the periphery (where the retina is the thin-
nest), and over hours to weeks may spread toward the central visual field
axis. Other ocular complaints such as pain, tearing, redness, and drainage
are typically associated with conditions other than retinal detachment.

Funduscopic examination with a direct ophthalmoscope is generally not
sufficient to rule out the diagnosis of retinal detachment. The narrow field of
view provided by the instrument does not allow one to see the peripheral as-
pects of the retina where tears are more frequently seen. Although the clini-
cian may see an abnormal red reflex or occasionally the classic white
billowing retinal separation, and indirect examination in a dilated eye by
an ophthalmologist is often necessary (Fig. 6) [110].
A central tenet in the treatment of retinal detachment is its initial preven-
tion. Risk factor modification and education of susceptible patients is cru-
cial. For example, those who have severe myopia should be encouraged to
wear protective lenses when playing sports, and those who undergo cataract
surgery should receive specific instructions about worrisome symptoms and
reasons to return to care. Similarly, those who have known vitreous detach-
ment or are at risk for developing the finding should be followed closely.
Fortunately, only 1% to 2% of such patients have a retinal break. If vitre-
ous hemorrhage is present, however, this risk increases to 70% [111]. If iden-
tified, retinal tears can be treated with cryotherapy or laser therapy, which
serve to create a scar at the site of the tear and prevent fluid from entering
the subretinal space. Success rates (ie, the prevention of retinal detachment)
are greater than 95% with this technique [112].
Surgical repair of retinal detachments, typically done by a retinal special-
ist, also has high success rates. More invasive therapies, such as scleral buck-
ling and posterior vitrectomy, post success rates of nearly 90% [113,114],
whereas less invasive therapies, such as pneumatic retinopexy, may be per-
formed in an office setting in select cases [115]. Retinal detachment surgery
Fig. 6. Retinal detachment. (From Kanski JJ. Clinical diagnosis in ophthalmology. China:
Mosby Elsevier; 2006; with permission.)
generally fails because of the growth of scar tissue on the retina in the weeks
following repair [116]. This phenomenon, termed proliferative vitreoretinop-
athy, is also amenable to surgery, although success rates are not as high and
resulting visual acuity is often poor [117].
If repair is technically successful, visual acuity is often restored to prede-
tachment levels. Critical in determining eventual visual outcome is the pres-
ence or absence of macular involvement in the tear, and if it is involved, the
duration and extent of its involvement. For this reason, those who have pre-
served central acuity should be referred for immediate surgery [118,119].
Retinal vasculitis
Retinal vasculitis is characterized by inflammation of the blood vessels
of the retina. It may be primarily ocular and limited to the orbit or may
be associated with various autoimmune and infectious systemic diseases.
Retinal vasculitis typically presents as a painless decrease in vision and is
diagnosed clinically by funduscopic examination; ancillary fluorescein angi-
ography and laboratory testing may be used to define the degree of in-
volvement and diagnose associated conditions. Treatments include local
and systemic immunosuppressive regimens and treatment of the underlying
disorder.
Epidemiology and etiology

Retinal vasculitis may be caused by infectious or systemic inflammatory
conditions or may be restricted solely to the retina. Retinal vasculitides as-
sociated with primary ocular disorders include idiopathic retinal vasculitis,
birdshot retinochoroidopathy, Eales disease, and pars plantaris syndrome
[120].
Autoimmune causes of retinal vasculitis include systemic lupus erythema-
tosus (SLE), Behçet disease, sarcoidosis, Wegener granulomatosis, polyar-
teritis nodosa (PAN), HLA-B27 associated conditions, and inflammatory
bowel disease (IBD). Various inflammatory ocular conditions may occur
as part of these diseases. In SLE, retinal involvement, including vasculitis,
is seen in 3% to 30% of patients [121,122]. Severe decreases in vision corre-
late with decreased survival and are likely related to uncontrolled systemic
inflammation [121,123–125]. Similarly, 80% of those who have Behçet dis-
ease have ocular involvement [126]. Those who have skin lesions and arthri-
tis have an increased risk for visual loss, generally resulting from recurrent
vasoocclusive disease from retinal vein vasculitis [127]. Of those patients
who have sarcoidosis, 20% to 25% have ocular disease [128,129], and 7%
of those have posterior segment involvement (a subset of which includes ret-
inal vasculitis) [129]. In Wegener granulomatosis ocular involvement occurs
in 30% to 60% with significant vision loss in 8%. Retinal vasculitis is rare,
however [130,131]. There are ocular findings in 10% to 20% of patients who
have PAN. Anterior segment findings are most common but retinal veins
may also be affected [132]. In HLA-B27 associated conditions, anterior uve-
itis is most common; posterior segment disease occurs in 4% to 17%. Of
those who have posterior segment disease, 24% have retinal vasculitis
[133–135]. Retinal vasculitis is uncommon in IBD and has only been ob-
served in case reports with Crohn disease [136].
Infectious causes of retinal vasculitis include toxoplasmosis, syphilis, tu-
berculosis, and several viruses, including varicella (VZV), herpes simplex
(HSV), and cytomegalovirus (CMV). Toxoplasmosis is responsible for
25% of cases of posterior uveitis in the United States. The resulting vitreous
inflammation often induces localized retinal vasculitis [137]. Ocular tubercu-
losis is an uncommon manifestation of extrapulmonary TB seen in approx-
imately 1% of patients [138,139]. Similarly, retinal vasculitis in syphilis is
rare, but may be seen in the secondary or tertiary stages [140]. HSV and
VZV retinal vasculitis are seen in immunocompromised chemotherapy pa-
tients, and those who are congenitally infected [141]. CMV retinal vasculitis
is seen in those who have HIV and low CD4 counts.
Clinical characteristics
Those who have retinal vasculitis typically present with a painless de-
crease in vision but may also report blind spots or floaters. More subtle
symptoms, such as changes in color vision and changes in the perceived
shape of an object, may occur with involvement of the macula [141].
Slit lamp examination reveals cells in the vitreous body [141]. Fundu-
scopic examination may reveal characteristic ‘‘vascular sheathing,’’ the ac-
cumulation of inflammatory cells on vessel walls manifested as an area of
retinal pallor paralleling the vessel lumen. This finding is more common
in peripheral vessels, and there may be segments of unaffected arterioles.
The increased vessel permeability that leads to vascular sheathing predis-
poses patients to vitreous hemorrhage and macular edema that may also
be seen [120].

Retinal vasculitis is diagnosed clinically based on a dilated funduscopic
examination in conjunction with consultation from an ophthalmologist. A
history and physical should be performed with specific attention to associ-
ated findings seen in the various systemic inflammatory and infectious con-
ditions described above and should direct laboratory and radiographic
testing. A detailed review of the clinical presentation of the various disorders
is beyond the scope of this article.
Regardless of the cause of the retinal vasculitis, fluorescein angiography
(injection of fluorescein in the retinal artery percutaneously to delineate the
retinal vasculature) is a commonly used ancillary test. It can help define the
extent of disease and aid in narrowing the differential diagnosis of

causative disorders. Angiography typically demonstrates perivascular stain-
ing that results from increased vascular permeability and is the visual cor-
relate of vascular sheathing on funduscopic examination. Areas of
nonperfused retina may also be detected as dark patches on the angiogram
[142].
In addition to angiography, focused laboratory and radiographic assess-
ment is still indicated even in the absence of historical or examination find-
ings that suggest an associated condition. According to a study performed
by the National Eye Institute, only 1% of patients initially diagnosed with
idiopathic retinal vasculitis developed subsequent systemic disease after 4
years of follow-up. The authors recommend a CBC, urinalysis, ESR, syph-
ilis and HIV serologies, and a chest radiograph in the absence of indications
for additional testing, but caution against low-yield indiscriminate labora-
tory testing [120].
Treatment of retinal vasculitis generally includes an immunosuppressive
regimen that may be topical, intraorbital, or systemic. Systemic corticoste-
roids are often the cornerstone of treatment, but regimens may also include
methotrexate, azathioprine, cyclosporine, and other immunosuppressive
agents, depending on the cause of the vasculitis. Important exceptions are
retinal vasculitides associated with infectious causes, wherein treatment is
based on correcting the underlying infection. Corticosteroids are used ad-
junctively in CMV, VZV, HSV, and toxoplasmosis to reduce inflammation.
[140]. The decision to initiate treatment of retinal vasculitis should be made
in conjunction with ophthalmology consultation, in addition to infectious
disease and rheumatology consultation if appropriate.
Summary
In summary, there are several causes of acute monocular vision loss,
some of which are discussed here, that require prompt recognition by the
emergency physician. Although the initial diagnosis is made by the emer-
gency department clinician, a prompt, and at times emergent, referral to
an ophthalmologist may be necessary.
References
[1] Salvarani C, Gabriel SE, O’Fallon WM, et al. The incidence of giant cell arteritis in Olmsted
County, Minnesota: apparent fluctuation in a cyclic pattern. Ann Intern Med 1995;123(3):
192–4.
[2] Weyand CM, Hunder NN, Hicok KC, et al. HLA-DRB1 alleles in polymyalgia
rheumatica, giant cell arteritis, and rheumatoid arthritis. Arthritis Rheum 1994;37(4):
514–20.
[3] Haworth S, Ridgeway J, Stewart I, et al. Polymyalgia rheumatica is associated with both
HLA-DRB1*0401 and DRB1*0404. Br J Rheumatol 1996;35(7):632–5.
[4] Elling P, Olsson AT, Elling H. Synchronous variations of the incidence of temporal arteritis
and polymyalgia rheumatica in different regions of Denmark: association with epidemics of
Mycoplasma pneumoniae infection. J Rheumatol 1996;23(1):112–9.
[5] Gabriel SE, Espy M, Erdman DD, et al. The role of parvovirus B19 in the pathogenesis of
giant cell arteritis: a preliminary evaluation. Arthritis Rheum 1999;42(6):1255–8.
[6] Weyand C, Goronzy JJ. Medium and large vessel vasculitis. N Engl J Med 2003;349(2):
160–9.
[7] Weyand CM, Tetzlaff N, Bjornsson J, et al. Disease patterns and tissue cytokine profiles in
giant cell arteritis. Arthritis Rheum 1997;40(1):19–26.
[8] Salvarani C, Macchioni PL, Tartoni PL, et al. Polymyalgia rheumatica and giant cell arter-
itis: a 5 year epidemiologic and clinical study in Reggio Emilia, Italy. Clin Exp Rheumatol
1987;5(3):205–15.
[9] Huston KA, Junder GG, Lie JT, et al. Temporal arteritis: a 25 year epidemiologic, clinical,
and pathologic study. Ann Intern Med 1978;88(2):162–7.
[10] Calamia KT, Hunder GG. Clinical manifestations of giant cell (temporal) arteritis. Clin
Rheum Dis 1980;6:389–403.
[11] Aiello PD, Trautmann JC, McPhee TG, et al. Visual prognosis in giant cell arteritis. Oph-
thalmology 1993;100(4):550–5.
[12] Gonzales-Gay MA, Blanco R, Rodriguez-Valverde V, et al. Permanent visual loss and ce-
rebrovascular accidents in giant cell arteritis: predictors and response to treatment. Arthri-
tis Rheum 1998;41(8):1497–504.
[13] Nesher G, Nesher R, Rozenman Y, et al. Visual hallucinations in giant cell arteritis: asso-
ciation with visual loss. J Rheumatol 2001;28(9):2046–8.
[14] Caselli RJ, Junder GG, Whisnant JP. Neurologic disease in biopsy-proven giant cell (tem-
poral) arteritis. Neurology 1988;38(3):352–9.
[15] Caselli RJ, Daube JR, Junder GG, et al. Peripheral neuropathic syndromes in giant cell
(temporal) arteritis. Neurology 1988;38(5):685–9.
[16] Unwin B, Williams C, Gilliland W. Polymyalgia rheumatica and giant cell arteritis. Am
Fam Physician 2006;74(9):1547–54.
[17] Spiera R, Spiera H. Inflammatory disease in older adults. Cranial arteritis. Geriatrics 2004;
59(12):25–9.
[18] Salvarani C, Cantini F, Boiardi L, et al. Polymyalgia rheumatica and giant cell arteritis.
N Engl J Med 2002;347(4):261–71.
[19] Smetana GW, Shmerling RH. Does this patient have temporal arteritis? JAMA 2002;
287(1):92–101.
[20] Hunder GG, Bloch DA, Michel BA, et al. The American College of Rheumatology 1990
criteria for the classification of giant cell arteritis. Arthritis Rheum 1990;33(8):1122–8.
[21] Schmidt W. Doppler sonography in rheumatology. Best Pract Res Clin Rheumatol 2004;
18(6):827–46.
[22] Reinhard M, Schmidt D, Hetzel A. Color-coded sonography in suspected temporal arter-
itis-experiences after 83 cases. Rheumatol Int 2004;24(6):340–6.
[23] Hayreh SS, Zimmerman B, Kardon R. Visual improvement with corticosteroid therapy in
giant cell arteritis. Report of a large study and review of literature. Acta Ophthalmol Scand
2002;80(4):355–67.
[24] Hayreh SS, Podhajsky PA, Rama R, et al. Giant cell arteritis: validity and reliability of var-
ious diagnostic criteria. Am J Ophthalmol 1997;123(3):285–96.
[25] Balcer LJ. Clinical practice. Optic neuritis. N Engl J Med 2006;354(12):1273–80.
[26] Phillips PH, Newman NJ, Lynn MJ. Optic neuritis in African Americans. Arch Neurol
1998;55(2):186–92.
[27] Arnold AC. Evolving management of optic neuritis and multiple sclerosis. Am J Ophthal-
mol 2005;139:1101–8.
[28] Frohman EM, Frohman TC, Zee DS, et al. The neuro-ophthalmology of multiple sclerosis.
Lancet Neurol 2005;4(2):111–21.
[29] Beck RW, Gal RL, Bhatti MT, et al. Visual function more than 10 years after optic neuritis:
experience of the Optic Neuritis Treatment Trial. Am J Ophthalmol 2004;137(1):77–83. [Er-
ratum in: Am J Ophthalmol 2004 Apr;137(4):following 793. Am J Ophthalmol 2004
Aug;138(2):following 321].
[30] Roed H, Frederiksen J, Langkilde A, et al. Systemic T-cell activation in acute clinically iso-
lated optic neuritis. J Neuroimmunol 2005;162(1–2):165–72.
[31] Soderstrom M, Link H, Xu Z, et al. Optic neuritis and multiple sclerosis: anti-MBP and
anti-MBP peptide antibody-secreting cells are accumulated in CSF. Neurology 1993;
43(6):1215–22.
[32] Lightman S, McDonald WI, Bird AC, et al. Retinal venous sheathing in optic neuritis. Its
significance for the pathogenesis of multiple sclerosis. Brain 1987;110(Pt 2):405–14.
[33] Frederiksen JL, Madsen HO, Ryder LP, et al. HLA typing in acute optic neuritis. Relation
to multiple sclerosis and magnetic resonance imaging findings. Arch Neurol 1997;54(1):
76–80.
[34] Optic Neuritis Study Group. The clinical profile of acute optic neuritis: experience of the
Optic Neuritis Treatment Trial. Arch Ophthalmol 1991;109(12):1673–8.
[35] Cole SR, Beck RW, Moke PS, et al. The national eye institute visual function questionnaire:
experience of the ONTT. Invest Ophthalmol Vis Sci 2000;41(5):1017–21.
[36] Rizzo JF III, Andreoli CM, Rabinov JD. Use of magnetic resonance imaging to differen-
tiate optic neuritis and nonarteritic anterior ischemic optic neuropathy. Ophthalmology
2002;109(9):1679–84.
[37] Rocca MA, Hickman SJ, Bo L, et al. Imaging the optic nerve in multiple sclerosis. Mult
Scler 2005;11(5):537–41.
[38] Kupersmith MJ, Alban T, Zeiffer B, et al. Contrast-enhanced MRI in acute optic neuritis:
relationship to visual performance. Brain 2002;125(Pt 4):812–22.
[39] CHAMPS Study Group. Interferon beta-1a for optic neuritis patients at high risk for mul-
tiple sclerosis. Am J Ophthalmol 2001;132(4):463–71.
[40] Beck RW, Trobe JD, Moke PS, et al. High- and low-risk profiles for the development of
multiple sclerosis within 10 years after optic neuritis: experience of the Optic Neuritis Treat-
ment Trial. Arch Ophthalmol 2003;121(7):944–9.
[41] Beck RW, Cleary PA, Anderson MM Jr, et al. A randomized, controlled trial of
corticosteroids in the treatment of acute optic neuritis. N Engl J Med 1992;326(9):
581–8.
[42] Kaufman DI, Trobe JD, Eggenberger ER, et al. Practice parameter: the role of corticoste-
roids in the management of acute monosymptomatic optic neuritis: report of the Quality
Standards Subcommittee of the American Academy of Neurology. Neurology 2000;
54(11):2039–44.
[43] Jacobs LD, Beck RW, Simon JH, et al. Intramuscular interferon beta-1a therapy initiated
during a first demyelinating event in multiple sclerosis. N Engl J Med 2000;343(13):
898–904.
[44] Comi G, Filippi M, Barkhof F, et al. Effect of early interferon treatment on conversion to
definite multiple sclerosis: a randomised study. Lancet 2001;357(9268):1576–82.
[45] Rumelt S, Dorenboim Y, Rehany U. Aggressive systematic treatment for central retinal ar-
tery occlusion. Am J Ophthalmol 1999;128:733–8.
[46] Cugati S, Wang JJ, Rochtchina E. Ten-year incidence of retinal emboli in an older popula-
tion. Stroke 2006;37:908–10.
[47] Wong TY, Klein R. Retinal arteriolar emboli: epidemiology and risk of stroke. Curr Opin
Ophthalmol 2002;13(3):142–6.
[48] Cahill MT, Stinnett SS, Fekrat S. Meta-analysis of plasma homocysteine, serum folate, se-
rum B12, and thermolabile MTHFR genotype as risk factors for retinal vascular occlusive
disease. Am J Ophthalmol 2003;136(6):1136–50.
[49] Ahuja RM, Chaturvedi S, Eliott D, et al. Mechanisms of retinal artery occlusive disease in
African American and Caucasian patient. Stroke 1999;30:1506–9.
[50] Greven CM, Slusher MM, Weaver RG. Retinal arterial occlusions in young adults. Am J
Ophthalmol 1995;120(6):776–83.
[51] Sharma S, Brown GC, Pater JL, et al. Does a visible retinal embolus increase the likelihood
of hemodynamically significant carotid artery stenosis in patients with acute retinal artery
occlusion? Arch Ophthalmol 1998;116(12):1602–6.
[52] Sheng FC, Quinones-Baldrich W, Machleder HI, et al. Relationship of extracranial ca-
rotid occlusive disease and central retinal artery occlusion. Am J Surg 1986;152(2):
175–8.
[53] Merchut MP, Gupta SR, Naheedy MH. The relation of retinal artery occlusion and carotid
artery stenosis. Stroke 1988;19(10):1239–42.
[54] Babikian V, Wijman CA, Koleini B, et al. Retinal ischemia and embolism. Etiologies and
outcomes based on a prospective study. Cerebrovasc Dis 2001;12(2):108–13.
[55] Douglas DJ, Schuler JJ, Buchbinder D, et al. The association of central retinal artery occlu-
sion and extracranial carotid artery disease. Ann Surg 1988;208(1):85–90.
[56] Appen RE, Wray SH, Cogan DG. Central retinal artery occlusion. Am J Ophthalmol 1975;
79(3):374–81.
[57] Sharma S, Naqvi A, Sharma SM, et al. Transthoraic echocardiographic findings in patients
with acute retinal arterial obstruction. A retrospective review. Arch Ophthalmol 1996;
114(10):1189–92.
[58] Mokhtari F, Massin P, Paques M, et al. Central retinal artery occlusion associated with
head or neck pain revealing spontaneous internal carotid artery dissection. Am J Ophthal-
mol 2000;129(1):108–9.
[59] Evans LS, Van de Graaf WB, Baker WH, et al. Central retinal artery occlusion after neck
irradiation. Am J Ophthalmol 1992;114:224–5.
[60] Chace R, Hedges TR. Retinal artery occlusion due to Moyamoya disease. J Clin Neuroop-
thalmol 1984;4(1):31–4.
[61] Katz B. Migrainous central retinal artery occlusion. J Clin Neuroophthalmol 1986;6(2):
69–75.
[62] Mansi IA, Alkhunaizi AM, Al-Khatti AA. Bilateral central retinal artery occlusion second-
ary to sickle cell disease. Am J Hematol 2000;64:79–80.
[63] Rumelt S, Rehany U. Central retinal artery occlusion associated with primary antiphos-
pholipid syndrome. Eye 1999;13(part 5):699–700.
[64] Greven CM, Weaver RG, Owen J, et al. Protein S deficiency and bilateral branch retinal
artery occlusion. Ophthalmology 1991;98(1):33–4.
[65] Nelson ME, Talbot JF, Preston FE. Recurrent multiple-branch retinal arteriolar occlusions
in a patient with protein C deficiency. Graefes Arch Clin Exp Ophthalmol 1989;227(5):
443–7.
[66] Mohan K, Gupta A, Jain IS, et al. Bilateral central retinal artery occlusion in occult tem-
poral arteritis. J Clin Neuroophthalmol 1989;9(4):270–2.
[67] Read RW, Chong LP, Rao NA. Occlusive retinal vasculitis associated with systemic lupus
erythematosus. Arch Ophthalmol 2000;188:588–9.
[68] Soloman SM, Soloman JH. Bilateral central artery occlusions in polyarteritis nodosa. Ann
Ophthalmol 1978;10(5):567–9.
[69] Mirza S, Raghu Ram AR, Bowling BS. Central retinal artery occlusion and bilateral cho-
roidal infarcts in Wegener’s granulomatosis. Eye 1999;13(pt 3a):374–6.
[70] Brown GC, Shields JA. Cilioretinal arteries and retinal artery occlusion. Arch Ophthalmol
1979;97(1):84–92.
[71] Werner MS, Latchaw R, Baker L, et al. Relapsing and remitting central retinal artery oc-
clusion. Am J Ophthalmol 1994;188:393–5.
[72] Savino PJ, Glaser JS, Cassady J. Retinal stroke. Is the patient at risk? Arch Ophthalmol
1977;95(7):1185–9.
[73] Hayreh SS, Zimmerman MB, Kimura A, et al. Central retinal artery occlusion. Retinal sur-
vival time. Exp Eye Res 2004;78(3):723–36.
[74] Hayreh SS, Weingeist TA. Experimental occlusion of the central artery of the retina. Ret-
inal tolerance time to acute ischaemia. Br J Ophthalmol 1980;64(11):818–25.
[75] Stone R, Zink H, Klingele T, et al. Visual recovery after central retinal artery occlusion: two
cases. Ann Ophthalmol 1977;9(4):445–50.
[76] Ffytche TJ. A rationalization of treatment of central retinal artery occlusion. Trans Oph-
thalmol Soc U K 1974;94(2):468–79.
[77] Nielsen NV. Treatment of acute occlusion of the retinal arteries. Acta Ophthalmol 1979;
57(6):1078–113.
[78] Atebara NH, Brown GC, Cater J. Efficacy of anterior chamber paracentesis and carbogen
in treating acute nonarteritic central retinal artery occlusion. Ophthalmology 1995;201(12):
2029–34.
[79] Rassam SM, Patel V, Kohner EM. The efficacy of acetazolamide on the retinal circulation.
Eye 1993;7(part 5):697–702.
[80] Werner D, Michalk F, harazny J, et al. Accelerated reperfusion of poorly perfused retinal
areas in central retinal artery occlusion and branch retinal artery occlusion after a short
treatment with enhanced external counterpulsation. Retina 2004;24:541–7.
[81] Harvey PA, Winder S, Talbot JF. Trabeculectomy for central retinal artery occlusion. Eye
2000;14(part 2):256–7.
[82] Harino S, Grunwald JE, Petrig BJ, et al. Rebreathing into a bag increases human retinal
macular blood velocity. Br J Ophthalmol 1995;79(4):380–3.
[83] Beatty S, Au Eong KG. Local intra-arterial fibrinolysis for acute occlusion of the central
retinal artery: a meta-analysis of the published data. Br J Ophthalmol 2000;84:914–6.
[84] Fraser S, Siriwardena D. Interventions for acute non-arteritic central retinal artery
occlusion. Cochrane Database of Systematic Reviews 2002;1:CD001989.
[85] Bananrjee S. A review of developments in the management of retinal diseases. J R Soc Med
2006;99:125–7.
[86] Wong TY, Larsen EK, Klein R, et al. Cardiovascular risk factors for retinal vein occlusion
and arteriolar emboli: the atherosclerosis risk in communities & cardiovascular health stud-
ies. Ophthalmology 2005;112:540–7.
[87] Mitchell P, Smith W, Chang A. Prevalence and associations of retinal vein occlusion in Aus-
tralia: the Blue Mountains Eye Study. Arch Ophthalmol 1996;114:1243–7.
[88] David R, Zangwill L, Badarna M. Epidemiology of retinal vein occlusion and its associa-
tion with glaucoma and increased intraocular pressure. Ophthalmologica 1988;197(2):
69–74.
[89] Cugati S, Wang JJ, Rochtchina E, et al. Ten year incidence of retinal vein occlusion in an
older population. The Blue Mountain Eye Study. Arch Ophthalmol 2006;124:726–32.
[90] Hayreh SS. Retinal vein occlusion. Indian J Ophthalmol 1994;42:109–32.
[91] Hayreh SS, Zimmerman B, McCarthy MJ, et al. Systemic diseases associated with various
types of retinal vein occlusion. Am J Ophthalmol 2001;131:61–77.
[92] Elman MJ, Bhatt AK, Quinlan PM, et al. The risk for systemic vascular diseases and mor-
tality in patients with central retinal vein occlusion. Ophthalmology 1990;97:1543–8.
[93] Eye Disease Case-Control Study Group. Risk factors for central retinal vein occlusion.
Arch Ophthalmol 1996;114:545–54.
[94] Eye Disease Case-Control Study Group. Risk factors for branch retinal vein occlusion. Am
J Ophthalmol 1993;116:286–96.
[95] Hayreh SS, Zimmerman MB, Beri M, et al. Intraocular pressure abnormalities associ-
ated with central and hemicentral retinal vein occlusion. Ophthalmology 2004;111:
133–41.
[96] Janssen MC, den Heijer M, Cruysberg JR, et al. Retinal vein occlusion: a form of venous
thrombosis or a complication of atherosclerosis? A meta-analysis of thrombophilic factors.
Thromb Haemost 2005;93:1021–6.
[97] Zegarra H, Gutman FA, Conforto J. The natural course of central retinal vein occlusion.
[98] Shahid H, Hossain P, Amoaku WM. The management of retinal vein occlusion: is interven-
tional ophthalmology the way forward? Br J Ophthalmol 2006;90:627–39.
[99] Mohamed Q, McIntosh RL, Saw SM, et al. Interventions for central retinal vein occlusion:
an evidence-based systematic review. Ophthalmology 2007;114(3):507–19, 524.
[100] Algvere PV, Jahnberg P, Textorius O. The Swedish retinal detachment register. I. A data-
base for epidemiological and clinical studies. Graefes Arch Clin Exp Ophthalmol 1999;237:
137–44.
[101] Rowe JA, Erie JC, Baratz KH, et al. Retinal detachment in Olmsted County, Minnesota,
1976 through 1995. Ophthalmology 1999;106:154–9.
[102] Haimann MH, Burton TC, Brown CK. Epidemiology of retinal detachment. Arch Oph-
thalmol 1982;100:289–92.
[103] Flood MT, Balazs EA. Hyaluronic acid content in the developing and aging human liquid
and gel vitreous. Invest Ophthalmol Vis Sci 1977;16(Suppl):67.
[104] Javitt JC, Street DA, Tielsch JM, et al. National outcomes of cataract extraction. Retinal
detachment and endophthalmitis after outpatient cataract surgery. Cataract Patient Out-
comes Research Team. Ophthalmology 1994;101:100–5.
[105] Schepens CL, Marden D. Data on the natural history of retinal detachment. Further char-
acterization of certain unilateral nontraumatic cases. Am J Ophthalmol 1966;61:213–26.
[106] Delori F, Pomerantzeff O, Cox MS. Deformation of the globe under high-speed impact: its
relation to contusion injuries. Invest Ophthalmol 1969;8:290–301.
[107] Gariano RF, Kim CH. Evaluation and management of suspected retinal detachment. Am
Fam Physician 2004;69:1691–8.
[108] Ghazi NG, Green WR. Pathology and pathogenesis of retinal detachment. Eye 2002;16:
411–21.
[109] Zayit-Soudry S, Moroz I, Loewenstein A. Retinal pigment epithelial detachment. Surv
Ophthalmol 2007;52:227–43.
[110] Shingleton BJ, O’Donoghue MW. Blurred vision. N Engl J Med 2000;343(8):556–62.
[111] Benson WE. Retinal detachment: diagnosis and management. 2nd edition. Philadelphia:
Lippincott; 1988. p. 4.
[112] Smiddy WE, Flynn HW Jr, Nicholson DH, et al. Results and complications in treated ret-
inal breaks. Am J Ophthalmol 1991;112:623–31.
[113] Campo RV, Sipperley JO, Sneed SR, et al. Pars plana vitrectomy without scleral buckle for
pseudophakic retinal detachments. Ophthalmology 1999;106:1811–5.
[114] Saw SM, Gazzard G, Wagle AM, et al. An evidence-based analysis of surgical interventions
for uncomplicated rhegmatogenous retinal detachment. Acta Ophthalmol Scand 2006;84:
606–12.
[115] Tornambe PE, Hilton GF. Pneumatic retinopexy. A multicenter randomized controlled
clinical trial comparing pneumatic retinopexy with scleral buckling. The Retinal Detach-
ment Study Group. Ophthalmology 1989;96:772–83.
[116] Machemer R, Aaberg TM, Freeman HM, et al. An updated classification of retinal detach-
ment with proliferative vitreoretinopathy. Am J Ophthalmol 1991;112:159–65.
[117] Vitrectomy with silicone oil or perfluoropropane gas in eyes with severe proliferative vitre-
oretinopathy: results of a randomized clinical trial. Silicone Study Report 2. Arch Ophthal-
mol 1992;110:780–92.
[118] Hassan TS, Sarrafizadeh R, Ruby AJ, et al. The effect of duration of macular detachment
on results after the scleral buckle repair of primary, macula-off retinal detachments. Oph-
thalmology 2002;109:146–52.
[119] Das T. Guidelines for the management of rhegmatogenous retinal detachment. Indian J
Ophthalmol 1993;41:37–40.
[120] George R, Walton R, Whitcup S, et al. Primary retinal vasculitis. Systemic associations and
diagnostic evaluation. Ophthalmology 1996;103(3):384–9.
[121] Gold D, Morris D, Henkind P. Ocular findings in systemic lupus erythematosus. Br J Oph-
thalmol 1972;56(11):800–4.
[122] Dunn JP, Noorily SW, Petri M, et al. Antiphospholipid antibodies and retinal vascular dis-
ease. Lupus 1996;5(4):313–22.
[123] Lanham JG, Barrie T, Kohner EM, et al. SLE retinopathy: evaluation by fluorescein angi-
ography. Ann Rheum Dis 1982;41(5):473–8.
[124] Stafford-Brady FJ, Urowitz M, Gladman D, et al. Lupus retinopathy: patterns, associa-
tions, and prognosis. Arthritis Rheum 1988;31(9):1105–10.
[125] Hall S, Buettner H, Luthra HS. Occlusive retinal vascular disease in systemic lupus eryth-
ematosus. J Rheumatol 1984;11(6):846–50.
[126] Mishima S, Masuda K, Izawa Y, et al. Behcet’s disease in Japan: ophthalmologic aspects.
Trans Am Ophthalmol 1979;77:225–79.
[127] Sakamoto M, Akazawa K, Nishioka Y, et al. Prognostic factors of vision in patients with
Behcet disease. Ophthalmology 1995;102(2):317–21.
[128] Siltzbach LE, James DG, Neville E, et al. Course and prognosis of sarcoidosis around the
world. Am J Med 1974;57(6):847–52.
[129] Jabs D, Johns C. Ocular involvement in chronic sarcoidosis. Am J Ophthalmol 1986;102(3):
297–301.
[130] Hoffman G, Kerr G, Leavitt R, et al. Wegener’s granulomatosis: an analysis of 158 patients.
Ann Intern Med 1992;116(6):488–98.
[131] Spalton D, Graham E, Page N, et al. Ocular changes in limited forms of Wegener’s gran-
ulomatosis. Br J Ophthalmol 1981;65(8):553–63.
[132] Morgan C, Foster C, D’Amico D, et al. Retinal vasculitis in polyarteritis nodosa. Retina
1986;6(4):205–9.
[133] Rodriguez A, Akova Y, Pedroza-Seres M, et al. Posterior segment ocular manifestations in
patients with HLA-B27 associated uveitis. Ophthalmology 1994;101(7):1267–74.
[134] Rothova A, Buitenhuis H, Meenken C, et al. Uveitis and systemic disease. Br J Ophthalmol
1992;76(3):137–41.
[135] Mapstone R, Woodrow J. HLA-27 and acute anterior uveitis. Br J Ophthalmol 1975;59(5):
270–5.
[136] Ruby A, Jampol L. Crohn’s disease and retinal vascular disease. Am J Ophthalmol 1990;
110(4):349–53.
[137] Henderly D, Gentsler A, Smith R, et al. Changing pattern of uveitis. Am J Ophthalmol
1987;103(2):131–6.
[138] Dutt A, Moers D, Stead WW. Short-course chemotherapy for extrapulmonary tuberculo-
sis: nine years’ experience. Ann Intern Med 1986;104(1):7–12.
[139] Donahue H. Ophthalmic experience in a tuberculosis sanatorium. Am J Ophthalmol 1967;
64(4):742–8.
[140] Park SS, Friedman A. Infectious causes of posterior uveitis. In: Alberts D, Jakobiec F,
editors. Principles and practice of ophthalmology. Philadelphia: WB Saunders; 2000.
p. 1236–49.
[141] Lang GK. Retinal inflammatory disease. In: Lang GK, editor. Ophthalmology. New York:
Thieme; 2000. p. 346–53.
[142] Alexander A, Sherman J, Horn D. Fundus fluorescein angiography: a summary of theoret-
ical concepts and clinical applications. J Am Optom Assoc 1979;50(1):53–63.
Emerg Med Clin N Am
26 (2008) 97–123
Trauma to the Globe and Orbit

Sharon P. Bord, MD*, Judith Linden, MD
Department of Emergency Medicine, Boston University Medical Center,
One Boston Medical Center Place, Dowling 1 South, Boston, MA 02118, USA
Trauma to the eye represents approximately 3% of all emergency depart-

ment visits in the United States. Rapid assessment and examination follow-
ing trauma to the eye is crucial. A thorough knowledge of potential injuries
is imperative to ensure rapid diagnosis, to prevent further damage to the eye,
and to preserve visual capacity. Although the eye represents only 0.3% of
the total surface area on the human body, loss of vision in one or both
eyes has been classified as a 24% or 85% whole-person impairment or dis-
ability, respectively [1].
History and physical examination

The general principles of the routine ocular examination also pertain to
an examination in the setting of trauma, but certain aspects of the examina-
tion deserve special attention. Triage and registration personnel should be
instructed regarding the urgency of eye injuries and of the need for simulta-
neous treatment and triage. One always should ‘‘take a step back’’ when
considering eye injuries and assess the entire patient. Life- or limb-threaten-
ing injury should be addressed initially. In addition, concomitant injury to
the brain, spinal cord, or facial bones is common and should be ruled out
in the appropriate setting. Historical details should include the timing,
mechanism, and location of injury. If there is penetrating trauma, one
should learn the energy and type of material involved; organic matter has
a higher rate of infection, and metal sometimes can cause a reaction in
the vitreous. In pediatric eye trauma, obtaining a history can be difficult; re-
luctance of the caregiver to provide basic historical information should raise
concern for abuse [2]. Visual symptoms such as change in vision, floaters,
flashing lights, pain, discharge, or diplopia should be noted. Prior ocular
E-mail address: [email protected] (S.P. Bord).
98 BORD & LINDEN
history such as previous visual impairment, tetanus status, and surgical his-
tory should be recorded. In addition one should ascertain if protective eye-
wear was used.
Physical examination should be performed in a systematic manner to
avoid missed injury. The examination can be accomplished in an external
to internal fashion. Throughout the examination, care should be taken to
avoid placing pressure on the globe, because pressure can cause herniation
of intraocular contents if the globe is ruptured. Initially, one should examine
the head, scalp, face, and periorbital tissues to assess for lacerations, lid
edema, foreign bodies, or sensory deficits (such as infraorbital hypoesthesia
in the setting of orbital blowout fractures). Tenderness to palpation around
the orbital rim or step-offs should be noted as well. One should look for ex-
ophthalmos, enophthalmos, or deformity of the external eye structures. Vi-
sual acuity should be tested before manipulation of the eye. Both near and
far vision of each eye should be tested separately, with the other eye oc-
cluded if possible. Visual acuity should be measured with the patient wear-
ing spectacle correction or using a pinhole occluder if spectacles are not
available. A Snellen eye chart or standardized near reading card should
be used. Any written material or an intravenous bottle may be used. If
the patient is unable to read, one can assess vision by having the patient
count the examiner’s displayed fingers, detecting hand motion, and indicat-
ing light perception or lack of light perception. The conjunctivae should be
examined for blood, chemosis (swelling), foreign bodies, and exposed tissue.
Examination of the cornea should include fluorescein staining under cobalt
blue light to look for irregularities and foreign bodies. Next, the internal
structures, including the iris and pupil, should be inspected, noting size,
shape, symmetry, and reaction to light. In addition to direct pupillary re-
sponse, one should assess consensual response using the ‘‘swinging flash-
light’’ test. When an afferent pupillary defect is present, both pupils will
dilate when the affected eye is exposed to the light source. This defect should
be differentiated from pharmacologic or traumatic mydriasis, which is more
specifically poor pupillary constriction and is more evident in bright settings.
In mydriasis the pupil of the nonaffected eye constricts briskly. Intraocular
pressure measurements help differentiate glaucomatous etiologies from
other causes of a red painful eye with decreased visual acuity. Elevated in-
traocular pressure measurements also may indicate the need for emergent
procedures such as canthotomies. Devices that can measure intraocular pres-
sure are adequate; even palpation on an anesthetized cornea can give useful
diagnostic information. Fundoscopy should be performed, first noting the
presence of a red reflex. Decreased intensity of the red reflex may indicate
the presence of a cataract, vitreous hemorrhage, or a large retinal detach-
ment. Slit-lamp examination should be performed when possible to assess
for injury to the anterior chamber, cornea, iris, and lens. If a slit-lamp exam-
ination cannot be performed, examination with a penlight may be used to
look for visible hyphema, obvious laceration, or a shrunken-appearing globe.
TRAUMA TO THE GLOBE AND ORBIT 99
Imaging techniques
Plain films
Plain film radiographs of the orbits and sinuses are rarely used for diagnosis
in orbital trauma. When performed, various views can provide information
regarding the orbits and sinuses, such as the presence of an orbital wall or fa-
cial bone fracture or opacification of the sinuses. The conventional Caldwell’s
and Waters’ views have moderate sensitivity in detecting orbital fractures:
73% to 78% for fractures of the orbital floor, 71% for fractures of the medial
orbital wall, and 64% for fractures of the ethmoid-maxillary plate [3].
CT scan
The CT scan is considered the reference standard imaging modality in the
diagnosis of mid-face fracture and orbital trauma. Orbital fractures are
commonly missed in patients who have concomitant head trauma, making
it imperative that one maintain a high suspicion for orbital injury [4]. The
advent of thin-slice helical CT with coronal reconstructions has demon-
strated improved image quality and reduced radiation to the lens [5]. The
sensitivity of CT scan for orbital fractures ranges from 79% to 96%, with
a lower sensitivity when evaluating the infraorbital rim [6]. Vegetable or
organic foreign bodies, which increase the risk of endophthalmitis, may
not be visualized on CT scan.
Ultrasound
Ocular ultrasound can be a useful tool for the emergency physician when
evaluating trauma to the eye. Trauma frequently results in considerable
soft tissue swelling, making it difficult to retract the lids and examine the
eye fully. Ultrasound can evaluate noninvasively for lens dislocation, globe
rupture, retrobulbar hemorrhage, intraocular foreign body, and retinal de-
tachment (Fig. 1). Visualization of periorbital gas on ultrasound may prompt
the physician to evaluate further for orbital fracture if not previously consid-
ered [7]. One study reported that ocular ultrasound performed by emergency
physicians had a sensitivity of 100% and a specificity of 97.2% for identifying
ocular pathology [8]. Care should be taken to place minimal pressure on the
lid when performing the study, especially if globe rupture has not yet been
ruled out. Ultrasound is contraindicated if there is high suspicion of rupture.
MRI
MRI is of limited usefulness in the acute stages of ocular trauma and
should not be performed if there is any concern that a metallic intraocular
foreign body is present. If there is concern regarding an organic foreign
body, MRI may help further differentiate this foreign body from soft tissues
when compared with a CT scan.
100 BORD & LINDEN
Fig. 1. Ultrasound demonstrating intraocular foreign body. (Courtesy of Andreas Dewitz, MD,
Boston, MA.)
Blunt trauma to the orbit

Periorbital tissues
Contusion
Periorbital contusion and swelling may be the most prominent initial fea-
tures in patients presenting to the hospital following trauma to the orbit.
The appearance of the ecchymosis and swelling can be dramatic and
make examination of the orbit challenging (Fig. 2). The emergency physi-
cian always must attempt to examine the structures underlying the swollen
Fig. 2. Contusion of the eye and adnexa. (From Boruchoff SA. Anterior segment disease: a
diagnostic color atlas. Philadelphia: Elsiever; 2001. p. 204; with permission.)
eyelids. Examination of the underlying tissue may be aided with a Desmarres

retractor, which will help avoid global pressure and damage to underlying
structures. It is important to keep in mind that periorbital contusion may
indicate related significant injury (eg, bilateral raccoon eyes may indicate
basilar skull fracture). A study of 600 patients who had sustained significant
head trauma found that 58.3% of patients who had isolated blepharohema-
toma on examination were found to have an orbital fracture on CT scan [9].
Treatment includes head elevation, cold compresses, and reassurance. Com-
plete resolution typically takes 2 to 3 weeks.
Orbital fractures
Orbital wall fractures were first termed ‘‘blowout’ fractures’’ in 1957 by
Converse and Smith. Orbital blowout fractures are those that occur within
the bony orbit, usually along the medial walls and/or the floor, but the or-
bital rims are intact. There are three proposed theories regarding the mech-
anism of a blowout fracture. The first is the hydraulic theory, which
postulates that when orbital pressure is increased, the globe decompresses
through the weakest part of the orbit. The globe-to-wall contact theory
was postulated in 1943 by Raymond Pfeiffer. This theory states that when
the globe becomes displaced posteriorly in trauma, it strikes the wall, caus-
ing a fracture. The third theory, proposed by Le Fort and Lagrange in 1917,
is that of buckling: the posterior movement of the orbital rim causes fracture
along the medial wall or floor of the orbit [10].
Blowout fractures account for approximately 11% of fractures involving
the orbit [11]. Following a blowout fracture, contents may herniate into the
maxillary sinus (with orbital floor fractures), or into the ethmoid sinus (with
medial wall fracture). A blowout fracture should be suspected when a patient
presents with trauma to the globe and soft tissue swelling. Patients may
complain of swelling following nose blowing, diplopia, or epistaxis. On ex-
amination one may find periorbital ecchymosis, subcutaneous emphysema,
restricted extraocular movements, enophthalmos or exophthalmos, ptosis,
or anesthesia in the distribution of the infraorbital nerve. The most common
limitation in extraocular movements is restriction of upward gaze caused by
the entrapment of the inferior rectus muscle. Muscle contusion or cranial
nerve disruptions also may be the cause of abnormal extraocular motility,
however (Fig. 3). Decreased sensation thought to be related to infraorbital
nerve injury can be verified further by testing sensation on the ipsilateral up-
per gum. It is of utmost importance to remember to inspect the entire globe,
because associated injury may occur in 10% to 25% of patients who have
orbital floor fractures [11].
The diagnosis of orbital fractures is made most often using CT scan. Al-
though rarely performed, plain-film radiographs of the orbits and sinuses
may demonstrate the classic teardrop sign, which may signify orbital con-
tents herniating into the maxillary sinus. In addition one can see air fluid
levels or opacification of the sinuses that may indicate a blowout fracture.
102 BORD & LINDEN
Fig. 3. Decreased extraocular movement. (Courtesy of D. Wagner, MD, Washington, DC.)
CT scan of the orbits is extremely sensitive for identifying fractures. In ad-

dition ultrasound is a promising tool that can be used to identify orbital
fractures. Periorbital gas seen on ultrasound may indicate an underlying or-
bital fracture [7].
Orbital fractures are not considered an ophthalmologic emergency unless
there is visual impairment or globe injury. Surgical repair is indicated for pa-
tients who have persistent diplopia or cosmetic concerns (enophthalmos)
and in general is not performed until swelling subsides 7 to 10 days follow-
ing injury [12].
Patients should be discharged with instructions to use ice compresses and
should be cautioned to avoid nose blowing and Valsalva maneuvers and to
sneeze with their mouths open. The routine use of prophylactic antibiotics
following orbital fracture is controversial and may not be indicated in all
cases [13]. Orbital cellulitis is a rare complication following orbital fracture.
Risk factors for developing orbital cellulitis include fracture adjacent to an
infected sinus and nose blowing. Prophylactic antibiotics are recommended
if adjacent sinusitis is present [14].
Patients should be instructed to return if they experience intense eye pain,
changes in vision, proptosis, or a tense globe. These complaints should raise
concern for compressive orbital emphysema or retrobulbar hemorrhage.
Retrobulbar hemorrhage
Traumatic hemorrhage into the retrobulbar space may result in acute vi-
sual loss [15]. Hemorrhage into the potential space surrounding the globe
may occur following blunt trauma because of injury to the orbital vessels.
It is important to remember that the orbit is an enclosed space bound later-
ally and posteriorly by bony walls, superiorly and inferiorly by the orbital
septa, and anteriorly by the globe and inelastic orbital septum. In a small
series of patients nondisplaced fractures of the orbital walls were found to
be associated with retrobulbar hematoma [16]. This condition is rare follow-
ing displaced fractures, however, because the blood will decompress into the
sinuses [17]. Hemorrhage can lead to an acute increase in intraorbital pres-
sure, which then is transmitted to the optic nerve and globe, resulting in cen-
tral retinal artery occlusion and optic nerve ischemia. Clinical signs and
symptoms include proptosis, limitation of extraocular movements, visual
loss, afferent pupillary defect, and increased intraocular pressure. Diagnosis
can be confirmed by CT scan, but treatment never should be delayed while
waiting for imaging.
Early recognition and decompression is key to preserving vision and
warrants emergent ophthalmologic consultation. Treatment of increased intra-
ocular pressure can be attempted with topical beta-blockers or intravenous
mannitol or carbonic anhydrase inhibitor. Lateral canthotomy can be a
vision-saving procedure and is indicated in patients who have a history of
trauma and marked periorbital edema with visual loss, severe proptosis, diffuse
subconjunctival hemorrhage, or an afferent papillary defect. Lateral canthot-
omy is performed by applying a small, straight clamp to the lateral canthus,
aiming inferiorly and laterally toward the conjunctival sac after local anesthesia
administration (lidocaine 1% with epinephrine). The clamp is left in place for
15 seconds to 2 minutes. After removal of the clamp there is an impression in
the tissue. A 1-cm incision is made along this impression using iris scissors. If
pressures remain elevated, the lateral canthal tendon should be transected as
well by aiming scissors inferolaterally. Transecting the inferior lateral canthal
tendon should result in the lower lid pulling easily away from the lid margin
(Fig. 4). Despite the decompression of high intraorbital pressure, usually
only a small amount of blood is expressed with the release of the hematoma.
Fig. 4. Orbit following lateral canthotomy. Note the lower lid pulling easily away from lid mar-
gin. (Courtesy of John Lee, MD, Boston, MA.)
104 BORD & LINDEN
Anterior chamber
Traumatic hyphema
Traumatic hyphema is caused by disruption of blood vessels in the iris or
ciliary body causing blood to extravasate into the anterior chamber (Fig. 5).
Hyphemas are classified from grade 0 to 4, based on the percentage of the
anterior chamber that is filled with blood. Microhyphemas are grade
0 and represent circulating red blood cells that can be detected only by
slit-lamp examination. The grading system then progresses from grades 1
(less than one fourth to one third of the anterior chamber) through 4 (total
anterior chamber filled with blood). An ‘‘eight-ball hyphema’’ refers to an
anterior chamber that is entirely filled with a black-appearing clot. Hy-
phema may occur after blunt or penetrating trauma, and more than 50%
have been documented as being sports related [1].
In traumatic hyphema, it is important to obtain a complete past medical
history specifically addressing bleeding disorders such as hemophilias and
Von Willebrand’s disease and hemoglobinopathies such as sickle cell anemia
or sickle trait. Patients also should be questioned regarding the use of med-
ications that may affect coagulation, including warfarin and aspirin. The
symptoms of hyphema include pain, photophobia, and blurring of vision.
Lethargy or somnolence can be associated with isolated traumatic hyphe-
mas but always should raise concern for a concomitant head injury. On ex-
amination one frequently can see a hyphema on gross inspection with the
patient sitting upright (causing the blood to layer in the aqueous fluid). In
addition slit-lamp examination should be performed to assess for associated
injury and further quantify the hyphema. Traumatic miosis or mydriasis
may be present as well and should be differentiated from an afferent papil-
lary defect. This distinction can be done by the swinging flashlight test, as
described elsewhere in this issue. An afferent papillary defect will cause par-
adoxical dilatation in the affected eye and the unaffected eye, whereas trau-
matic mydriasis will result in constriction of both pupils (although limited in
the affected eye). An afferent pupillary defect should raise concern for an op-
tic nerve or posterior pole injury.
Fig. 5. Hyphema. (Courtesy of D. Wagner, MD, Washington, DC.)

The most common complication of hyphema is rebleeding, which occurs

2 to 5 days following injury when the initial clot retracts and loosens. Re-
bleeding occurs in approximately 22% of patients [1]. Rebleeding is more
common in those who have visual acuities of 20/200 on presentation, initial
hyphema of more than one third of the anterior chamber, more than 1 day’s
delay in obtaining medical attention, and elevated intraocular pressure at
initial presentation [1,18]. Other complications include corneal blood stain-
ing (in approximately 5% of patients), elevated intraocular pressure, and
synechiae formation between the iris and the cornea or the lens posteriorly.
Elevated intraocular pressure facilitates corneal blood staining. As the pres-
sure in the anterior chamber increases, the ability of the corneal endothelial
cells to transport fluid out of the stroma is impaired. The cornea swells, and
the endothelium becomes leaky, forming gaps large enough for the hemo-
globin to enter the corneal stroma. Patients who have sickle cell anemia
or who are trait positive have a higher rate of complications associated
with hyphema. The acidic and hypoxic nature of the anterior chamber leads
to sickling of the red blood cells, which can cause increased intraocular pres-
sure and decreased aqueous humor output. Patients who have a history of
sickle cell trait have rates of rebleeding up to 64% [1].
Treatment for hyphema should be directed at minimizing secondary hem-
orrhage and reducing the incidence of secondary glaucoma. Recent literature
indicates that hospitalization versus outpatient management, moderate am-
bulation versus strict bed rest, or treatment with unilateral or bilateral eye
patches has no significant effect on the incidence of secondary hemorrhage
or final visual outcome [1]. In addition, outpatient treatment has been shown
to be cost effective [19]. Hospitalization has been recommended for patients
who have rebleeding, elevated intraocular pressure, positive sickle cell trait
or anemia, hyphemas greater than 50%, or decreased vision, for noncompli-
ant patients, and in suspected child abuse. The disposition of these patients
always should be discussed with an ophthalmologist. Patients who are dis-
charged from the hospital must undergo daily examinations by an ophthal-
mologist to assess for increased size of hyphema or other complications.
Supportive therapy includes elevation of the head to 30 while at rest. In
addition, eye patching with a metal shield provides protection from further
ocular injury and often is recommended until the hyphema resolves. The
shield must have holes or be made of clear plastic so that patients can mon-
itor their vision, because decreased vision is the earliest symptom of rebleed-
ing. Topical application of cycloplegics has not been shown to have
significant therapeutic effects but can increase patient comfort and facilitate
the examination of the posterior segment. Topical corticosteroids have been
shown to reduce intraocular inflammation and decrease the incidence of sec-
ondary hemorrhage [20]. Topical and systemic antifibrinolytics, such as ami-
nocaproic acid (ACA) and tranexamic acid (not available in United States),
have been shown to decrease secondary hemorrhage significantly [18]. Ami-
nocaproic acid is an antifibrinolytic agent that prevents the conversion of
106 BORD & LINDEN
plasminogen to plasmin and therefore delays clot dissolution and theoreti-

cally decreases the risk of rebleeding. Studies have shown that topical
ACA is as effective as systemic ACA and avoids the side effects typically as-
sociated with administration such as nausea, vomiting, and hypotension [1].
These agents should be avoided in pregnant patients, patients who have re-
nal or hepatic dysfunction, or those at risk for thromboembolic disease. In-
tracameral tissue plasminogen activator currently is under investigation and
is reserved for large clots of prolonged duration or malignant elevation of
intraocular pressure. In addition, elevated intraocular pressure (O 24 mm
Hg) can be treated with topical beta-blockers and carbonic anhydrase inhib-
itors such as acetazolomide. Acetazolomide lowers pH, causing increased
sickling, and should be avoided in pediatric patients or those who have
sickle cell trait or anemia. Methazolomide is the preferred agent in this sit-
uation [21]. Mannitol may be administered for severely elevated intraocular
pressure (O 35 mm Hg). Nonsteroidal anti-inflammatory medications and
aspirin should be avoided because of the increased risk of rebleeding. Sur-
gery usually is reserved for delayed complications such as corneal staining
and persistently elevated intraocular pressures but often is performed early
in patients who have sickle cell anemia or trait.
Subconjunctival hemorrhage
Subconjunctival hemorrhage is caused by the rupture of small subcon-
junctival blood vessels. Although patients may report a prior sneezing or
coughing episode or a Valsalva maneuver, some patients may have no rec-
ollection of the preceding events. Patients often seek care because of the dra-
matic appearance. On examination a painless, smooth, bright-red area is
noted over the bulbar conjunctiva and is sharply demarcated at the limbus.
Visual acuity should be normal. If there is preceding blunt or penetrating
trauma, underlying scleral penetration should be considered. Pain on extra-
ocular movements and bloody chemosis also should prompt suspicion for
injury to the globe. Subconjunctival hemorrhage should always be distin-
guished from chemosis: a hemorrhage is flat and smooth, whereas chemosis
presents as erythema with edema, and the area is raised. Bilateral or recur-
rent subconjunctival hemorrhage may require a work-up for bleeding diath-
esis. Treatment of subconjunctival hemorrhage consists of reassurance and
local cold compresses for 24 hours. Subconjunctival hemorrhages heal spon-
taneously in 2 to 4 weeks.
Injury to the iris and ciliary body

Traumatic iridocyclitis (uveitis)
Blunt injury to the globe may contuse and inflame the iris and ciliary
body, resulting in ciliary spasm. Patients may complain of photophobia,
blurred vision, and eye pain. Examination reveals conjunctival injection,
specifically ciliary flush, cells, and flare in the anterior chamber and a small,
poorly dilating pupil. Cells and flare are best seen in the slit lamp with high
magnification and very bright light. This condition is self limited; symptom-
atic treatment consists of paralyzing the iris and ciliary body with long-act-
ing cycloplegic agents such as homatropine 5% for a period of 7 to 10 days.
If there is no improvement after 5 to 7 days, prednisolone acetate 1% may
be used to decrease inflammation in consultation with an ophthalmologist.
Topical steroids should be withheld in patients who have a corneal epithelial
defect. Resolution typically occurs within 1 week.
Traumatic mydriasis and miosis

Blunt injury to the orbit may damage the iris sphincter. Bruising and ir-
ritation of the sphincter leads to constriction of the pupil (miosis). Small
tears to the sphincter muscle may result in dilatation or mydriasis. In the set-
ting of significant head trauma and altered mental status, one must rule out
a cranial nerve palsy and brain herniation. Treatment is supportive, and the
condition often resolves spontaneously.
Iridodialysis
Traumatic iridodialysis is a tearing of the iris root from the ciliary body
leading to formation of a ‘‘secondary pupil’’ (Fig. 6). This injury may also
cause a hyphema. Large tears can be a cause of monocular diplopia. Urgent
ophthalmologic consultation is warranted when iridodialysis causes a hy-
phema or decreased visual acuity. Surgical repair may be indicated for per-
sistent monocular diplopia.
Acute glaucoma
Acute glaucoma can occur following trauma. The underlying pathophys-
iology is narrowing of the anterior chamber or disruption of outflow of
Fig. 6. Iridodialysis. (Courtesy of D. Wagner, MD, Washington, DC.)

108 BORD & LINDEN
aqueous humor, either secondary to red blood cells (hyphema), formation of

a trabecular meshwork scar, or lens dislocation. Posttraumatic glaucoma is
associated with advanced patient age, lens injury, poor baseline visual acu-
ity, and inflammation of the anterior chamber [22]. Patients who have these
characteristics should be followed closely by an ophthalmologist to assess
for development of glaucoma. Acute rises in intraocular pressure should
be treated aggressively with miotics (contraindicated in eyes following cata-
ract surgery or lens extraction), topical drops including beta-blockers, alpha
agonists, prostaglandin analogues, acetazolomide, or mannitol.
Injury to the lens

Subluxation and dislocation
Blunt trauma to the eye results in a sudden compressive deformation of
the globe, displacing the cornea and anterior sclera posteriorly with a com-
pensatory expansion of the globe in the equatorial direction. This trauma
can result in damage to the lens zonule fibers, which are responsible for
holding the lens in place, causing lens dislocation or subluxation. Following
complete disruption of the lens zonules, the lens may dislocate posteriorly
or, less commonly, into the anterior chamber (Fig. 7). Diagnosis of this in-
jury is based on history and physical examination. Symptoms include blur-
ring of the vision or monocular diplopia and distortion when the lens
remains partially in the visual axis. The lens may be visualized when dis-
placed into the anterior chamber or may be viewed after pupillary dilatation
when dislocated posteriorly. Iridodonesis, a tremor of the iris after rapid eye
movements, may be a helpful finding associated with posterior dislocations
[23]. An anteriorly dislocated lens may cause acute angle closure glaucoma,
which may be a vision-threatening complication. Predisposing factors for
lens dislocation include Marfan’s syndrome, homocystinuria, and sphero-
phakia [23]. Lens subluxations and dislocations should be referred to an
Fig. 7. Traumatic lens dislocation. (From Harlan JB, Pieramici DJ. Evaluation of patients with
ocular trauma. Ophthalmol Clin North Am 2002;15:159; with permission.)
ophthalmologist for surgical repair. Repair should be performed on an

emergent basis if the lens is obstructing the flow of aqueous humor, leading
to elevated intraocular pressure.
Cataract formation
The stroma of the lens normally are sequestered in a relatively dehy-
drated environment by the lens capsule. If the lens capsule is disrupted by
either blunt or penetrating trauma, the stroma may absorb fluid, swell,
and become cloudy (Fig. 8), obstructing the outflow of aqueous humor
and leading to acute glaucoma. Traumatic cataracts may occur acutely or
develop over weeks to months. Bilateral cataracts may develop after light-
ning strike or electrical injury [24]. There is no effective prevention, and de-
finitive treatment requires lens replacement.
Globe injury
Globe rupture
Globe rupture always should be considered when evaluating a patient
who has sustained blunt trauma or a penetrating injury, because it is a major
cause of monocular blindness. More than 90% of these injuries are prevent-
able, with trauma from violent behavior accounting for a large portion [25].
Ruptures are most common at the insertions of the intraocular muscles or at
the limbus, where the sclera is thinnest. Diagnosis of a ruptured globe can be
obvious if intraocular contents are visualized, but occult rupture can be dif-
ficult to diagnose. Critical signs and symptoms include decreased visual acu-
ity, severe bullous subconjunctival hemorrhage (involving 360 of the bulbar
conjunctiva), a deep or shallow anterior chamber, and limitation of extraoc-
ular motility. Other signs include low intraocular pressure (although pres-
sure may be normal or increased), an irregularly shaped pupil (peaked
toward the wound), iridodialysis, exposed uveal tissue (which appears
brownish-red), or vitreous hemorrhage (Fig. 9).
Fig. 8. Traumatic cataract and iridodialysis. (From Harlan JB, Pieramici DJ. Evaluation of
patients with ocular trauma. Ophthalmol Clin North Am 2002;15:159; with permission.)
110 BORD & LINDEN
Fig. 9. Ruptured globe. (From Kanski JJ. Clinical diagnosis in ophthalmology. Philadelphia:
Mosby Elsevier; 2006.)
Once the diagnosis of globe rupture is made or suspected, further manip-

ulation of the globe should be deferred. Treatment includes emergent oph-
thalmologic consultation, placement of a protective eye shield, antiemetics
(to avoid further increasing intraocular pressure during a Valsalva maneu-
ver), analgesics, and systemic antibiotics to prevent endophthalmitis. Any
maneuver that may increase intraocular pressure should be avoided. A
CT scan should be obtained to evaluate for the presence of orbital and in-
traocular foreign bodies. CT scan evaluation alone is only 75% sensitive
for diagnosis of globe rupture and therefore should be used as a complement
to physical findings [26]. Ultrasonography and indirect ophthalmoscopy
may be helpful in further evaluation but should be deferred to the ophthal-
mologist. The administration of succinylcholine for airway management is
controversial. There have been anecdotal reports of expulsion of intraocular
material with succinylcholine use, but more recent case studies do not sup-
port this finding [27]. The current recommendation for patients who require
rapid airway management with concomitant penetrating ocular injury is
rapid-sequence intubation with succinylcholine following pretreatment
with a nondepolarizing and sedating agent.
Globe luxation
Luxation of the globe is a rare condition that results from an extreme
form of trauma. Many years ago it was thought to be caused by gouging
during fights and historically has been a complication of forceps delivery
in obstructed labor [28]. Now it is seen more commonly after great forces
are applied to the head in an anteroposterior direction (eg, in a motor vehi-
cle crash), but there have been case reports documented in which luxation
was caused by penetrating trauma. The most commonly injured and avulsed
extraocular muscle is the medial rectus. The status of the optic nerve is clin-
ically important. Injury to the optic nerve may occur from the forward pro-
pulsion of the globe with traction on the nerve or may be related to a sudden
rise in intraocular pressure.
Acute care consists mainly of protecting the eye from further damage.
This protection can be accomplished with a cup or other hard protective de-
vice. In addition, early reduction with topical anesthesia can be attempted,
because it will minimize the amount of traction placed on the optic nerve.
Globe reduction may not be possible in the emergency department because
of the swelling of the eyelids and surrounding tissues, necessitating intrao-
perative reduction under general anesthesia [28]. Spontaneous and volun-
tary globe luxation has been reported during the insertion of contact
lenses and may be more common in diseases that cause proptosis such as
Grave’s disease and orbital tumors. Calming the patient and having the pa-
tient relax the eyelids will make it easier for spontaneous repositioning to
occur.
Posterior segment
Vitreous hemorrhage
Vitreous hemorrhage occurs when blood enters the normally avascular
vitreous space, which is filled with a clear gelatinous material. This space
is bordered anteriorly by the lens, posteriorly and laterally by the retina,
and laterally by the ciliary body. Although most causes of vitreous hemor-
rhage are nontraumatic (diabetic retinopathy, sickle cell disease, posterior
vitreous detachment, retinal vein occlusion, leukemia), trauma accounts
for 12% to 31% (depending on study population) and is the most common
cause of vitreous hemorrhage in younger patients [29,30]. Vitreous hemor-
rhages can be associated with retinal tears, avulsed retinal veins, or sub-
arachnoid hemorrhage. Vitreous hemorrhages in an infant should prompt
consideration of shaken baby syndrome, admission, and an in-depth search
for other traumatic injuries. Vitreous hemorrhages may be associated with
traumatic and atraumatic subarachnoid hemorrhage (Terson’s syndrome).
The theory is that increasing intracranial pressure results in increased retinal
vein pressures, causing rupture and vitreous hemorrhage [31]. Patients who
have Terson’s syndrome have a poorer prognosis than patients who have
subarachnoid hemorrhage without vitreous hemorrhage.
Symptoms of vitreous hemorrhage include floaters (small hemorrhage),
cobwebs, shadows, a smoky haze, or loss of vision (larger hemorrhage).
Physical examination may reveal a decreased or absent light reflex, loss of
fundus detail, or floating debris but may be normal in the undilated eye.
An indirect fundoscopic examination should be performed if the direct ex-
amination is normal. In the preretinal hemorrhages of shaken baby syn-
drome, layering of blood with a meniscus may be seen on the retina.
Traumatic vitreous hemorrhage mandates emergent ophthalmologic con-
sultation, because 11% to 44% of vitreous hemorrhages are associated with
retinal tears [32]. Ultrasound may be helpful if the fundus is obscured. Dis-
charge instructions include limited physical activity and head elevation when
sleeping. Nonsteroidal anti-inflammatory drugs and aspirin should be
112 BORD & LINDEN
avoided, unless the benefit outweighs the risk (ie, in patients who have un-
stable angina or other clinical indications). Definitive treatment depends on
the underlying cause and may include laser, cryotherapy, or scleral buckling
in cases of retinal detachment. Most hemorrhages resolve spontaneously
within a few weeks to months.
Chorioretinal injury
Trauma is the most common cause of retinal detachment in children and
is responsible for about 10% of detachments in the general population [33].
Symptoms of retinal tears or detachments include floaters from bleeding and
flashing lights from stimulation of retina neurons. Isolated tears and detach-
ments do not cause pain. Visual field defects or decreased visual acuity may
be present as well. Retinal detachment generally progresses slowly following
injury, occurring weeks to months later. Fundoscopic examination may re-
veal a hazy, gray membrane of the retina billowing forward (Fig. 10), but
small peripheral tears may not be visualized on direct ophthalmoscopy. In-
direct ophthalmoscopy should be performed on every patient in whom the
diagnosis of retinal detachment or tear is being considered. Approximately
one third of retinal detachments are not diagnosed until at least 6 weeks
after trauma [34].
Management of retinal tears or detachment begins with urgent ophthal-
mologic consultation. Treatment consists of either head elevation or supine
positioning, depending on whether the tear is superior or inferior. Surgical
correction consists of scleral buckling, pars plana vitrectomy, or photocoag-
ulation. The timing of surgical correction is based on the size of the retinal
detachment and the degree of macular involvement.
Fig. 10. Retinal detachment. (From Kanski JJ. Clinical diagnosis in ophthalmology. Philadel-
phia: Mosby Elsevier; 2006.)
Commotio retina
Commotio retina, also known as ‘‘Berlin’s edema,’’ may occur after re-
cent blunt ocular trauma. Studies have demonstrated this injury to be pres-
ent in 9% to 14% of blowout fractures [11]. The term ‘‘commotio retina’’
literally means retinal contusion in Latin and is caused by disruption of
the retinal photoreceptors. The term ‘‘Berlin’s edema’’ is actually a misno-
mer, because no edema is present (Fig. 11). Patients may complain of de-
creased visual acuity or may be asymptomatic if the macula is not
involved. Examination reveals a confluent area of retinal whitening. Blood
vessels can be seen distinctly below the whitened area. Commotio retina
can occur anywhere on the retina, but it usually is maximal in the area op-
posite the traumatic blow. There is no specific treatment for this condition,
because it usually resolves spontaneously in approximately 2 weeks. Serial
examination is necessary to ensure that retinal tear or detachment has not
occurred.
Penetrating ocular injury

Periorbital tissues
Conjunctival lacerations
Lacerations of the bulbar conjunctiva are commonly associated with in-
traocular foreign bodies or underlying sclera perforation, so a ruptured
globe must be ruled out. Conjunctival lacerations may be seen as a conjunc-
tival defect, exposure of Tenon’s capsule, or orbital fat. Slit-lamp examina-
tion can help differentiate superficial from deep lacerations. Small,
superficial lacerations (! 1 cm) require no suturing and generally heal rap-
idly. Lacerations that are greater than 1 cm may be repaired by an
Fig. 11. Commotio retina is visualized as a patchy, gray-white opacification of the retina.
(Courtesy of D. Wagner, MD, Washington, DC.)
114 BORD & LINDEN
ophthalmologist using 6-0 to 8-0 absorbable suture. Treatment with prophy-

lactic antibiotic ointment or drops is recommended for all patients who have
conjunctival lacerations [35].
Laceration of the eyelid

Lacerations of the eyelid should prompt concern for a penetrating globe
injury or foreign body. Emergency physicians can manage simple horizontal
or oblique partial-thickness lid lacerations. Closure should be performed
with interrupted 6-0 or 7-0 nylon sutures. Sutures should be removed in 3
to 5 days. Lacerations that require immediate referral to an ophthalmologist
for repair include those that involve the lid margins, the canalicular system,
or the levator or canthal tendons, loss of tissue, or lacerations through the
orbital septum. A laceration through the orbital septum should be suspected
if orbital fat protrudes through the wound [35].
Globe injury
Corneoscleral laceration and puncture wounds
Signs of corneal perforation include loss of anterior chamber depth,
blood in the anterior chamber, and a teardrop-shaped pupil caused by iris
prolapse through the corneal laceration. Small corneal lacerations may be
difficult to diagnose. If corneal laceration is suspected, one must inspect
the entire cornea while taking care to not put excessive pressure on the
globe. Corneal lacerations occur most often on the inferior aspect of the
globe because of Bell’s phenomenon, the reflex upward rotation of the globe
during blinking in response to potential foreign body penetration. Suspicion
that aqueous humor is leaking from the wound can be confirmed by Seidel’s
test, which is performed by applying fluorescein dye over the area of concern
and noting a stream of yellow dye on slit-lamp examination (Fig. 12).
Fig. 12. Corneal laceration following repair. (Courtesy of John Lee, MD, Boston, MA.)
Once a laceration is suspected, a protective cover should be placed on the

eye, and prophylactic antibiotics should be administered. Ophthalmologic
consultation should be obtained when there is concern for a full-thickness
laceration. Partial-thickness lacerations that are not widened can be treated
with cycloplegics, topical antibiotics, and a pressure patch. Lacerations that
require repair are performed in the operating room.
Intraocular foreign body

An intraocular foreign body is present in 18% to 41% of open globe in-
juries. The diagnosis should be suspected based on history [36]. Hammering
(especially of metal on metal) is the most common mechanism, responsible
for 60% to 80% of cases (Figs. 13 and 14). In developed countries this type
of injury now occurs more commonly in the home than in the workplace. It
is crucial to identify the material of the foreign body because it will influence
treatment decisions. Specifically, iron-containing foreign bodies can cause
siderosis, brownish discoloration of the iris, and yellow cataracts, and cop-
per may lead to cheilosis, a rapidly developing sterile endophthalmitis.
Lead-containing products may cause lead poisoning if left in place.
An intraocular foreign body should be considered strongly in penetrating
globe injuries. Visual acuity generally is decreased, but normal visual acuity
does not rule out the presence of a foreign body. Additional warning signs
on examination consist of localized corneal edema, hemorrhage over the
sclera, a nonsurgical hole in the iris, or a cloudy lens. CT has largely re-
placed plain-film radiography in the diagnosis of an intraocular foreign
Fig. 13. Intraocular foreign body located near the retina. (Courtesy of D. Wagner, MD,
Washington, DC.)
116 BORD & LINDEN
Fig. 14. Large plastic foreign body from fireworks injury. (From Mester V, Kuhn F. Intraocu-
lar foreign bodies. Ophthalmol Clin North Am 2002:15;239; with permission.)
body and has a sensitivity ranging from 65% (for a foreign body ! 0.06
mm3) to 100% (for foreign bodies O 0.06 mm3) [36]. Other modalities for
diagnosis include ultrasound (relatively contraindicated in suspected globe
penetration), in which sensitivity varies greatly based on clinician experience
[36], and MRI, which is limited because of concern for secondary intraocu-
lar damage if the foreign body is metallic. Treatment consists of intravenous
antibiotics to prevent endophthalmitis. Vitrectomy should be performed
without delay on patients who have signs of endophthalmitis and for foreign
bodies that consist of vegetable matter or copper or those that occur in the
rural setting and may be contaminated with soil. In a series of patients from
the military population, rates of endophthalmitis did not increase if foreign
body removal was delayed [37]. Preventive measures include encouraging
proper eye protection for all patients.
Orbital foreign body

Patient history often is the first clue to the diagnosis of an orbital foreign
body and also can indicate the most likely material (Fig. 15). As with intra-
ocular foreign bodies, it is important to discern if the foreign body is metal-
lic or organic in nature. The location of the foreign body can be confirmed
with an orbital CT scan (best for metallic foreign bodies) or by MRI and
orbital ultrasound, which may help better locate organic material. Foreign
bodies that can be left in the orbit include BBs, metallic shrapnel fragments,
and bits of metal that are not copper. Small metallic fragments generally are
well tolerated in the orbit, and leaving them in place may cause less harm
than a deep exploration. Organic foreign bodies and objects that are pro-
truding through the skin or penetrating the brain or the sinuses should be
removed in the operating room. Inorganic foreign bodies that require spe-
cial consideration and possible removal include objects containing copper,
lead, and iron [38]. Incomplete extraction has been documented when at-
tempted in the emergency department. It is important to assure complete
Fig. 15. External photograph demonstrating obvious ocular/orbital foreign body. (From
Harlan JB, Pieramici DJ. Evaluation of patients with ocular trauma. Ophthalmol Clin North
Am 2002:15;158; with permission.)
removal, especially of organic material, because it is associated with a high

risk of infection [39]. It is common for metallic fragments to migrate with
time in the orbit. Patients who present with orbital pain after injury by
shrapnel injury or another foreign body may require removal even years af-
ter the injury.
Delayed complications
Endophthalmitis
Posttraumatic endophthalmitis, or inflammation of the deep structures of
the eye, is a devastating complication of open globe injury, occurring in 0%
to 13% of patients [40]. Infection may occur in hours to months following
ocular trauma. Symptoms consist of pain, photophobia, and visual loss
and often are hard to distinguish from other posttraumatic infections.
Pain or visual loss out of proportion to the clinical condition should prompt
consideration of endophthalmitis. Examination may also reveal nonspecific
signs including hypopyon, conjunctival erythema and edema, and lid swell-
ing. The pathogens causing endophthalmitis, in order of prevalence, include
Staphylococcus epidermidis, Bacillus spp, and Streptococcus (which is partic-
ularly prevalent in the pediatric population) [41]. Bacillus causes up to 46%
of endophthalmitis cases seen in a rural population because of contaminated
soil or farming equipment [41]. Systemic and topical antibiotics against the
common causative pathogens are the standard of care for prevention of
posttraumatic endophthalmitis, although there have been no randomized,
clinical trials. Prophylactic intravitreal antibiotics should be administered
only by a trained ophthalmologist. Risk factors for developing posttrau-
matic endophthalmitis were thought to include delayed primary repair, de-
lay in initiating therapy, breach in the lens capsule, and retained intraocular
foreign body [40]. Experience with penetrating eye injury during Operation
Iraqi Freedom, however, found that delayed removal of intraocular foreign
118 BORD & LINDEN
bodies did not increase the rate of endophthalmitis if systemic and topical
antibiotics were administered [42]. Definitive treatment consists of vitrec-
tomy in addition to antibiotics. Topical or systemic corticosteroid therapy
is controversial. Early recognition and prompt treatment of Bacillus infec-
tion increases the chances of improved visual outcome.
Sympathetic ophthalmia
Sympathetic ophthalmia is an inflammation that occurs in the uninjured
(sympathizing) eye weeks to decades following the initial insult to the in-
jured eye. It also may occur following uncomplicated intraocular surgery.
It is thought to be caused by an autoimmune response to the release of
the uveal contents into the vitreous humor of the injured eye. This release
of tissue leads to autoimmune destruction in the uninjured eye, potentially
causing severe bilateral vision loss. Sympathetic ophthalmia is rare, occur-
ring in 0.2% to 1% of patients following trauma and in 0.001% of patients
following surgical procedures. Symptoms are similar to a nongranulomatous
uveitis with blurry vision, photophobia, decreased visual acuity, and tearing.
Examination may reveal keratic precipitates, papillitis, and subretinal exu-
dates. These exudates may coalesce at the posterior pole, producing an
area of exudative retinal detachment and, over time, scarring of the poste-
rior pole. Treatment consists of high-dose oral corticosteroids. If the inflam-
mation cannot be controlled, other immunosuppressive agents, including
cyclosporine, chlorambucil, or azathioprine, may be used. Sympathetic oph-
thalmia can be prevented if the hopelessly injured eye with no visual poten-
tial is enucleated within 14 days of trauma. The role of enucleation following
development of sympathetic ophthalmia is controversial and should be
based on the visual acuity of the injured eye. It remains unclear if enucle-
ation of the injured eye improves prognosis of the sympathizing eye. If
the eye retains some visual function, it probably should not be removed.
Burns
Acid and alkali exposure
Chemical burns are worrisome because of their ability to affect multiple
ocular structures profoundly and potentially cause blindness. Alkali-con-
taining agents, such as oven and drain cleaners (potassium hydroxide),
lime in plaster (calcium hydroxide), fertilizers and sparklers (ammonium hy-
droxide), and high-concentration bleach (sodium hypochlorite), are particu-
larly damaging because they are both lipophilic and hydrophilic and can
penetrate cell membranes rapidly (Fig. 16). Deployment of airbags causes
release of sodium hydroxide, which can lead to an alkaline chemical keratitis
[34]. Ocular damage results from saponification of cell membranes and cell
death along with destruction of the extracellular matrix. Acidic agents, such
as hydrofluoric acid (used in industrial etching and alkylation of high-octane
gasoline), battery acid (sulfuric acid), and hydrochloric acid, generally cause
Fig. 16. Eye following alkaline exposure. (Courtesy of John Lee, MD, Boston, MA.)
less damage than alkaline agents because many corneal proteins bind acid
and act as a buffer. The coagulated tissue functions as a barrier and prevents
further penetration of the acid.
The Roper-Hall classification of chemical burns can be used; for simplic-
ity, however, burns can be classified as mild-to-moderate burns and severe
burns. The most important differentiating feature is limbal and corneal is-
chemia. On examination of mild-to-moderate burns one can see focal areas
of conjunctival chemosis and hyperemia as well as mild eyelid edema and
anterior chamber reaction. First- and second-degree burns may be noted
on the periocular skin. Severe burns are characterized by conjunctival
blanching and pallor [43]. Corneal edema or opacification can make it dif-
ficult to evaluate the anterior chamber. Early sequelae of chemical burns oc-
curring 1 to 3 days after exposure include elevated intraocular pressure
caused by damage of the trabecular meshwork and corneal edema. Long-
term complications include perforation, scarring, and neovascularization
of the cornea, adhesions of the lids to the globe (symblepharon), glaucoma,
cataract, and retinal damage.
Treatment should be initiated immediately after exposure by irrigating
the eyes with copious amounts of fluid, preferably saline or lactated Ringer’s
solution, for at least 30 minutes. A Morgan lens may be used if there is no
particulate material. (See the article entitled ‘‘Eye Exposures’’ in this issue
for a discussion of eye irrigation techniques, including the Morgan lens).
Five to 10 minutes after completing irrigation, litmus paper should be
placed in the inferior cul de sac. Irrigation is continued until a neutral
pH, 7.0 to 7.4, is reached. pH is measured before adding topical anesthetic
to the eye, because most anesthetics have an acidic pH and may lower the
measured pH artificially. Foreign body removal, by sweeping the fornices
with a cotton swab, should be performed as well, with special attention to
the conjunctival fornices, because material that is crystallized may cause
a persistently elevated pH. Additional treatment consists of cycloplegia
120 BORD & LINDEN
(using scopolamine 0.25%), topical antibiotic ointment, and topical steroids

if significant inflammation of the cornea or anterior chamber is present.
Phenylephrine should be avoided because it causes vasoconstriction. If ele-
vated intraocular pressure is noted, anti-glaucoma medications such as acet-
azolamide should be initiated. Oral vitamin C (ascorbic acid) stimulates
collagen production and therefore has a theoretical benefit [43]. These pa-
tients require close follow-up, and some may require hospital admission
for further irrigation or monitoring.
Miscellaneous irritants, solvents, and detergents

Unknown exposures should be treated initially as though they were an
acid or alkali exposure. The eye should be irrigated immediately. Detergents
generally cause only a mild conjunctival irritation, but more irritating sub-
stances can cause denudation of the cornea and inflammation of the anterior
chamber. After the eye is thoroughly irrigated, it should be treated like a cor-
neal abrasion with topical erythromycin ointment. Exposure to aerosol
products may lead to intraocular foreign body from the propellant. Com-
pounds that are found in personal protective devices such as mace and pep-
per spray are treated in the same fashion as other chemical injuries.
Special consideration should be given to cyanoacrylate adhesive (‘‘super-
glue’’) exposure. These glues are rapid setting and harden quickly on contact
with moisture. If the eyelids are glued together, an attempt should be made
to separate them with gentle traction. Lids that are sealed shut in normal
anatomic position that cannot be separated with gentle traction can be
left alone, allowing time for the super glue to dissolve over the next few
days. Misdirected lashes and hardened glue may cause corneal defects and
irritation. These defects should be treated like corneal abrasions. Attempts
to dissolve the adhesive with other agents should be avoided. Ophthalmo-
logic consultation should be obtained for these exposures [43].
Thermal burns
Thermal burns are more frequently seen on the eyelids than on the globe
because of reflex blinking and Bell’s phenomenon. Superficial burns may be
treated with irrigation and topical antibiotic ophthalmic ointment. Second-
or third-degree burns of the eyelid warrant ophthalmologic consultation.
Although patients frequently cover the face, hot liquid splashes and ciga-
rettes ashes may cause a superficial corneal epithelial injury and are treated
like a corneal abrasion. There have also been documented cases of air bag–
related thermal burns caused by failure of the inflation mechanism [34].
UV keratitis
Exposure to UV light can occur from tanning booth exposure, from high-
altitude environments, or from welding. The UV light causes direct corneal
epithelial damage. Patients give a history of exposure approximately 6 to 10
hours before developing symptoms. Symptoms include moderate-to-severe
ocular pain, foreign body sensation, red eye, tearing, photophobia, and
blurred vision. Examination reveals decreased visual acuity and diffuse
punctate lesions on fluorescein staining, with a sharp demarcation at the
lower lid where the eye was protected. Treatment consists of cycloplegic
drops, topical antibiotic ointment, and oral analgesics.
Prevention
Wearing protective eyewear often can prevent injuries to the globe. A
study of patients in the US military who had sustained eye injury found
that only a small percentage of them were wearing protective glasses at
the time of injury [37,42]. In addition, many sports-related eye injuries could
be prevented by wearing the recommended protective equipment. Air bag
deployment, although associated with a 20% reduction in the incidence of
fatal and severe injuries after frontal and near-frontal automobile collisions,
increases the risk of orbital fractures or other ocular trauma [44].
Acknowledgment
The authors thank Dr. John Lee for his expert manuscript review and
comments.
References
[1] Brandt MT, Haug R. Traumatic hyphema: a comprehensive review. J Oral Maxillofac Surg
2001;56:1462–70.
[2] Harlan JB, Pieramici DJ. Evaluation of patients with ocular trauma. Ophthalmol Clin North
Am 2002;15:153–61.
[3] Iinuma T, Hirota Y, Ishio K. Orbital wall fractures. Conventional views and CT. Rhinology
1994;32(2):81–3.
[4] Holmgren E, Dierks E, Homer L, et al. Facial computed tomography use in trauma patients
who require a head computed tomogram. J Oral Maxillofac Surg 2004;62(8):913–8.
[5] Lakits A, Prokesch R, Scholda C. Orbital helical computed tomography in the diagnosis and
management of eye trauma. Ophthalmology 1999;106:2330–5.
[6] Jank S, Deibl M, Strobl H, et al. Intrarater reliability in the ultrasound diagnosis of medial
and lateral orbital wall fractures with a curved array transducer. J Oral Maxillofac Surg
2006;64(1):68–73.
[7] McIlrath ST, Blaivas M, Lyon M. Diagnosis of periorbital gas in ocular ultrasound after fa-
cial trauma. Am J Emerg Med 2005;23:517–20.
[8] Blaivas M, Theodoro D, Sierzenski P. A study of bedside ultrasonography in the emergency
department. Acad Emerg Med 2002;9(8):791–9.
[9] Exadaktylos A, Sclabas GM, Smolka K, et al. The value of computed tomographic scanning
in the diagnosis and management of orbital fractures associated with head trauma: a prospec-
tive, consecutive study at a level I trauma center. J Trauma 2005;58(2):336–41.
[10] Kreidl K, Kim D, Mansour S. Prevalence of significant intraocular sequelae in blunt ocular
trauma. Am J Emerg Med 2003;21(7):525–8.
[11] He D, Blomquist P, Ellis E. Association between ocular injuries and internal orbital frac-
tures. J Oral Maxillofac Surg 2007;65(4):713–20.
122 BORD & LINDEN
[12] Burnstine AU. Clinical recommendations for repair of orbital facial fractures. Curr Opin
Ophthalmol 2003;14(5):236–40.
[13] Martin B, Ghosh A. Antibiotics in orbital floor fractures. J Emerg Med 2003;20(1):66.
[14] Simon GJB, Bush S, Selva D, et al. Orbital cellulitis: a rare complication after orbital blow-
out fracture. Ophthalmology 2005;112(11):2030–4.
[15] Vasallo S, Hartstein M, Howard D, et al. Traumatic retrobulbar hemorrhage: emergent de-
compression by lateral canthotomy and cantholysis. J Emerg Med 2001;22(3):251–6.
[16] Gerbino G, Ramieri GA, Nasi A. Diagnosis and treatment of retrobulbar haematomas fol-
lowing blunt orbital trauma: a description of eight cases. Int J Oral Maxillofac Surg 2005;
34(2):127–31.
[17] Popat H, Doyle PT, Davies SJ. Blindness following retrobulbar haemorrhagedit can be pre-
vented. Br J Oral Maxillofac Surg 2007;45(2):163–4.
[18] Rahmani B, Jahadi H, Rajaeefard A. An analysis of risk for secondary hemorrhage in trau-
matic hyphema. Ophthalmology 1999;106:380–5.
[19] Shiuey Y, Lucarelli MJ. Traumatic hyphema: outcomes of patient management. Ophthal-
mology 1998;105:851–5.
[20] Crouch ER Jr, Crouch ER. Management of traumatic hyphema: therapeutic options. J Pe-
diatr Ophthalmol Strabismus 1999;36:238–50.
[21] Recchia F, Saluja R, Hammel K. Outpatient management of traumatic microhyphema.
Ophthalmology 2002;109(8):1465–70.
[22] Girkin C, McGwin G, Morris R, et al. Glaucoma following penetrating ocular trauma: a co-
hort study of the United States eye injury registry. Am J Ophthalmol 2005;139(1):100–5.
[23] Netland K, Martinez J, LaCour O, et al. Traumatic anterior lens dislocation: a case report.
J Emerg Med 1998;17(4):637–9.
[24] Lee MS, Gunton KB, Fischer D, et al. Ocular manifestations of remote lightning strike. Ret-
ina 2002;22(6):808–10.
[25] Rahman I, Maino A, Devadson D, et al. Open globe injuries: factors predictive of poor out-
come. Eye 2006;20:1336–41.
[26] Joseph D, Pieramici D, Beauchamp N. Computed tomography in the diagnosis and progno-
sis of open-globe injuries. Ophthalmology 2000;107:1899–906.
[27] Vachon C, Warner D, Bacon D. Succinylcholine and the open globe: tracing the teaching.
Anesthesiology 2003;99(1):220–3.
[28] Bajaj M, Pushker N, Naniwal S, et al. Traumatic luxation of the globe with optic nerve avul-
sion. Clin Experiment Ophthalmol 2003;31:362–3.
[29] Rabinowitz R, Yagev R, Shoham A, et al. Comparison between clinical and ultrasound find-
ings in patients with vitreous hemorrhage. Eye 2004;18(3):253–6.
[30] Dana MR, Werner MS, Viana MA, et al. Vitreous hemorrhage. Ophthalmology 1993;
100(9):1380–2.
[31] McCaron MO, Alberts MJ, McCaron P. A systematic review of Terson’s syndrome: fre-
quency and prognosis after subarachnoid hemorrhage. J Neurol Neurosurg Psychiatry
2004;75(3):491–3.
[32] Spraul CW, Grossniklaus HE. Vitreous hemorrhage. Surv Ophthalmol 1997;42(1):3–39.
[33] Pieramici DJ. Vitreoretinal trauma. Ophthalmol Clin North Am 2002;15:225–35.
[34] Duma S, Kress T, Porta D, et al. Airbag-induced eye injuries: a report of 25 cases. J Trauma
1996;41(1):114–9.
[35] Brunette DD. Ophthalmology. In: Marx JA, Hockberger RS, Walls R, editors. Rosen’s
emergency medicine: concepts and clinical practice, vol 2. 6th editionPhiladelphia: Mosby;
2006. p. 1044–52.
[36] Mester V, Kuhn F. Intraocular foreign bodies. Ophthalmol Clin North Am 2002;15:235–42.
[37] Thach A, Ward T, Dick J, et al. Intraocular foreign bodies during Operation Iraqi Freedom.
Ophthalmology 2005;112(10):1829–33.
[38] Fulcher T, McNab A, Sullivan T. Clinical features and management of intraorbital foreign
bodies. Ophthalmology 2002;109(3):494–500.
[39] Long J, Tann T. Orbital trauma. Ophthalmol Clin North Am 2002;15:249–53.

[40] Essex R, Yi Q, Charles P, et al. Post-traumatic endophthalmitis. Ophthalmology 2004;
111(11):2015–22.
[41] Danis RP. Endophthalmitis. Ophthalmol Clin North Am 2002;15:243–8.
[42] Colyer M, Weber E, Weichel E, et al. Delayed intraocular foreign body removal without en-
dophthalmitis during operations Iraqi Freedom and Enduring Freedom. Ophthalmology
2007;114(8):1439–47.
[43] The Wills eye manual. In: Rhee D, Pyfer M, editors. Philadelphia: Lippincott, Williams &
Wilkins; 1999. p. 19–50.
[44] Francis D, Kaufman R, Bevan Y, et al. Air bag induced orbital blowout fractures. Laryngo-
scope 2006;116(11):1966–72.
Emerg Med Clin N Am
26 (2008) 125–136
Chemical, Thermal, and Biological

Ocular Exposures
Jordan Spector, MDa,b,
William G. Fernandez, MD, MPHa,*
a
Boston Medical Center, Department of Emergency Medicine, Boston University School
of Medicine, One BMC Place, Dowling 1 South, Boston, MA 02118, USA
b
Department of Emergency Medicine, Albert Einstein Medical Center, 5501 Old York Road,
Philadelphia, PA, USA
Chemical or radiant energy injuries to the eyes are considered ocular

burns. The majority of these injuries are occupation-related [1]. Chemical
burns are by far more common and represent a true emergency. Thermal
and UV injuries are associated with severe pain, but often result in less
long-term sequelae than chemical injuries do. The term ‘‘biologic exposure’’
refers to an exposure to human blood or other body fluid. This article
describes patterns of these injuries and exposures, with particular emphasis
on emergent management and including acute diagnostic and treatment
considerations.
Chemical burns
Chemical burns to the eye are common, particularly in industrial settings,
and constitute an ocular emergency. In fact, chemical burns were the second
leading cause of work-related eye injury treated in United States emergency
departments in 1999 [2]. A burn may occur with exposure of the eye to any
chemical, solid, liquid, or aerosol. Household cleaning supplies and
cosmetics are common offenders. The potent alkaline or acidic substances
contained within these products cause the burn injury. Accidents involving
industrial materials in the workplace are a frequent cause of eye burns.
Chemical exposures to the eye can result in significant damage to the
ocular surface epithelium, the cornea, and the anterior segment. Permanent
unilateral or bilateral visual impairment may result. Alkaline substances can
E-mail address: [email protected] (W.G. Fernandez).
126 SPECTOR & FERNANDEZ
be particularly injurious. An alkali causes liquefactive necrosis of the surface

epithelium, leading to rapid penetration of the substance to the deeper layers
of the eye. There can be irreversible damage to the corneal stroma and
endothelium, as well as to anterior segment structures. Most acidic sub-
stances cause coagulation necrosis when exposed to the cornea. The reaction
precipitates surface proteins, which serve as a barrier to deeper damage, and
acidic injury tends to be superficial [3].
Pathophysiology
The external portion of the eye is covered by the cornea centrally and the
conjunctiva peripherally. The cornea is a multilayered structure atop a base-
ment membrane, all covered by a thin film of tears. Both the cornea and the
conjunctiva produce a nonkeratinized epithelium, and both have the ability
to rapidly regenerate and renew surface epithelium during injury repair [3].
Within minutes of a small injury to the corneal epithelium, the regenerative
process begins. However, after a larger injury, healing may take 4 to 5 hours
to begin. Injuries that destroy the basement membrane may require up to
6 weeks to complete healing. After significant injuries, full restoration of
the cornea may never occur [4].
Alkali injury
The severity of chemical burn injury is directly proportional to the sur-
face area of contact on the cornea and the depth of penetration of the
substance. Alkalis penetrate into the eye more readily than do acids. The
hydroxyl ion of a base causes saponification of the fatty acids within
the cornea, resulting in epithelial cell disruption and death. The associated
cation then penetrates toward deeper structures. Depending on the degree
of penetration, a number of structures may suffer injury, including the cor-
neal and conjunctival epithelium, the basement membrane, stromal kerato-
cytes, the lens, stromal nerve endings, the episclera, the iris, and the ciliary
body (Figs. 1 and 2) [3].
A number of alkaline substances cause ocular injury. Some of the more
common agents are listed in Table 1. The most serious alkali injuries are as-
sociated with exposures to ammonia (anterior segment injury in !1 minute)
and to lye (deep injury within 3–5 minutes). Magnesium hydroxide, which is
present in fireworks, may produce a severe injury because of the coexistent
thermal burn [3]. Eye injuries secondary to methamphetamine production
have been described as well [5].
On presentation, a patient with an alkaline eye burn demonstrates
conjunctival hyperemia, chemosis, and corneal clouding. The stroma may
have mild edema, and the anterior chamber may develop cells and flare.
Severe alkali burns are characterized by corneal opacification and limbal
ischemia [6].
CHEMICAL, THERMAL, AND BIOLOGICAL OCULAR EXPOSURES 127
Fig. 1. Alkali burn demonstrating corneal burns and conjunctival injection on the day of the
accident. (From Kaiser PK, Friedman NJ, Pineda II R. The Massachusetts Eye and Ear
Infirmary illustrated manual of ophthalmology, second edition. China: Saunders; 2004.
p. 122, 123 ; with permission.)
Acid injury
Acids cause less severe, more focal tissue injury. The corneal epithelium
offers moderate protection against weaker acids. The hydrogen ion alters
surface pH, while the associated anion reacts with epithelial and superficial
stromal cells to precipitate and denature surface proteins [3]. The coagulated
proteins function as a superficial barrier and prevent intraocular injury.
Stronger acids may penetrate and produce an injury pattern comparable
to that of an alkali burn, as deep tissue damage occurs when the exposed
eye reaches a pH of 2.5 or less [6].
Some common acidic agents that cause ocular injury are listed in Table 2.
Sulfuric acid is the most common cause of acid injury. It seldom produces
Fig. 2. Complete corneal tissue destruction 7 days after alkali burn. (From Kaiser PK, Fried-
man NJ, Pineda II R. The Massachusetts Eye and Ear Infirmary illustrated manual of ophthal-
mology, second edition. China: Saunders; 2004. p. 122, 123; with permission.)
Table 1
Common products containing alkali
Product Chemical
Lime Calcium carbonate or magnesium carbonate
Plaster and mortar Calcium hydroxide
Oven and drain cleaner (lye) Sodium hydroxide or potassium hydroxide
Fireworks and sparklers Magnesium hydroxide
Ammonia (in cleaning agents and fertilizers) Ammonium hydroxide
Dishwasher detergent Sodium tripolyphosphate
Data from Xiang H, Stallones L, Chen G, et al. Work-related eye injuries treated in hospital
emergency departments in the US. Am J Ind Med 2005;48(1):57–62; and Farjo AA, Soong HK.
Corneal epithelium. In: Yanoff M, Duker JS, editors. Ophthalmology. 2nd edition. St. Louis
(MO): Mosby; 2004.
serious injury unless there is additional damage from thermal injury or high-
velocity penetration of a foreign body into the eye, as may occur in the
explosion of an automobile battery [3]. Acids containing heavy metals may
penetrate and produce injury patterns similar to those seen in alkali burns [6].
Hydrofluoric acid (HF) exposure can cause the most potentially serious
ocular acid injury and deserves special consideration. HF is a low molecu-
lar-weight acid found in many industrial and commercial products. It can
exist in gaseous or aqueous form. After exposure, HF penetrates deeply in
tissues and causes liquefaction necrosis, much like an alkali. HF contamina-
tion may cause significant injury to multiple organ systems. In the eye, HF
can rapidly penetrate to anterior chamber structures and cause devastating
ocular injury [7,8].
In burns caused by strong acids, the cornea and conjunctiva rapidly
become white and opaque. The epithelium may slough, leaving a relatively
clear stroma. This may initially mask the severity of the injury. The cornea
eventually becomes opacified. Very severe acid burns also cause complete
corneal anesthesia, limbal pallor, and uveitis [6].
Table 2
Common products containing acid
Product Chemical
Toilet cleaner Sulfuric acid (80%)
Battery fluid Sulfuric acid (30%)
Pool cleaners Sodium hypochlorite or
calcium hypochlorite (70%)
Bleaches Sodium hypochlorite (3%)
Vinegar or essence of vinegar Acetic acid
Glass polishers, silicone production agents, Hydrofluoric acid
rust removal agents
Food- and leather-processing compounds Hydrochloric acid
Data from Xiang H, Stallones L, Chen G, et al. Work-related eye injuries treated in hospital
emergency departments in the US. Am J Ind Med 2005;48(1):57–62; and Farjo AA, Soong HK.
Corneal epithelium. In: Yanoff M, Duker JS, editors. Ophthalmology. 2nd edition. St. Louis
(MO): Mosby; 2004.
Cyanoacrylate exposure
Cyanoacrylate is an adhesive that results in strong polymer bonds with
a variety of materials and is used in both industrial and domestic settings
[9]. It is a substance considered to have relatively little toxicity. It has also
been used by ophthalmologists during surgical procedures to seal globe
injuries without incident [10]. However, the emergency department clinician
may encounter a patient who inadvertently instilled cyanoacrylate-contain-
ing adhesives into the eye. Alternatively, the clinician may accidentally spill
skin adhesives into a patient’s eye when repairing lacerations on the fore-
head or face. Such occurrence can result in adherence of the upper and lower
eyelids. This may lead to local dermatitis of the eyelids, as well as to eyelash
loss [10]. Occasionally, cyanoacrylate may collect over the cornea and irri-
tate the eyes, resulting in mild to moderate corneal abrasions [11]. In such
exposures, treatment as per the management for corneal abrasions in indi-
cated. Attempting to pry the eyelids open should be done with caution, as
this may result in further injury [12]. In the vast majority of cases, the
adhesive separates from the eyelids within 1 week. As needed, irrigation
with normal saline may remove particulate debris in and about the eye.
Otherwise, no other specific interventions are indicated, and expectant
management is appropriate [9–12].
Treatment
For the emergency physician, the management of chemical burns is
contingent upon the time elapsed since injury. Eye injury due to chemical
exposure occurs in phases. The immediate or acute phase occurs at the
time of the injury and results in corneal and conjunctival epithelium necrosis
and chemical invasion into the anterior chamber, the ciliary body, and the
iris. The later phases of eye burns (intermediate and chronic) occur over
the subsequent days to weeks and require management by ophthalmologic
and plastic surgery specialists.
The goal of treatment after a chemical burn is to restore physiologic pH
of the eye as rapidly as possible. If the external pH is restored to normal, the
aqueous pH within the eye returns to normal within 30 minutes [3]. The
most important initial intervention for all chemical exposures is irrigation.
Delays in the initiation of irrigation by as little as 20 seconds have been dem-
onstrated in animal models to result in a higher maximal pH and higher risk
for more severe injury after alkaline exposure [13]. If possible, irrigation
should begin immediately after injury, even before a patient presents to
an emergency department. A number of studies have looked for the best
agent for irrigation, and certain solutions containing buffering agents have
been proposed as ideal [14]. However, even tap water is an appropriate irri-
gating solution [15,16], and any nontoxic solution, such as normal saline or
lactated Ringer’s solution, is effective. If the nature of the chemical injury is
unknown, one may apply a piece of pH paper to the inferior fornix to

determine if the offending substance was basic or acidic, but this should
not delay the start of irrigation.
To permit appropriate irrigation of the chemically exposed and inflamed
eye, topical anesthesia with 0.5% proparacaine may be necessary. Lid
retractors or a lid speculum may be helpful. Prolonged irrigation can be
accomplished with the use of a polymethylmethacrylate scleral lens with
an attached perfusion tube. If such a lens is not available, an angiocath
may be inserted percutaneously across the upper lid, though this should
be reserved for rare instances and after discussion with consultants [6].
Chemical burns can be quite painful. Topical anesthesia may help. Addi-
tionally, the clinician should be prepared to provide the patient with sys-
temic analgesics. For rapid pain relief and for ease of administration
during irrigation, parenteral nonsteroidal anti-inflammatories or narcotic
analgesics should be strongly considered.
Classically, ophthalmologists have suggested irrigation for 30 minutes or
until 2 L of irrigant has been applied [6]. However, most now advocate contin-
ued irrigation until the eye is restored to normal pH (checked at 30-minute
intervals). In animal models, greater than 2 hours of irrigation were necessary
to achieve normal pH in the aqueous humor after a severe alkali burn [17].
Many ophthalmologists recommend reassessing pH approximately 30
minutes after the eye is restored to normal pH, especially after alkali burns,
as particulate matter lodged in the conjunctiva can dissolve slowly and cause
persistently increased pH, requiring further irrigation [6].
Emergent referral to an ophthalmologist is indicated in all but the most
minor of injuries. Considering that injury severity can be difficult to ascer-
tain upon emergent presentation, urgent follow-up is recommended for all
chemical burns that cannot be evaluated by the ophthalmologist in the
emergency department.
In a patient that reports an eye splash of rust remover, leather-tanning
fluid, high-octane gasoline or glass or enamel etching materials, the clinician
should be suspicious for an HF exposure. This is significant because, even
though irrigation with water or saline should be instituted immediately,
some reports suggest prolonged or repetitive irrigation can increase corneal
ulceration and worsen outcome following HF exposure [8,18]. As topical
calcium gels are indicated for dermal exposures to HF, some have suggested
that application of 1% calcium gluconate drops to affected eyes may be
protective after an HF burn [18,19]. However calcium salts can be quite
irritating to the eye, and most would advise use of calcium gluconate drops
only after discussion with an ophthalmologist [7,8].
Once irrigation is complete, a thorough evaluation for any particulate
matter should be performed. This must include eversion of both upper
and lower lids, and retraction of redundant conjunctival tissue. Topical
anesthetic may be helpful. Any particles can be removed with a moistened
cotton-tip applicator. Larger particles can be removed with smooth forceps.
As with all patients who present to an emergency department with eye

complaints, an assessment of visual acuity must be performed. For patients
who do not arrive in the emergency department with their corrective lenses,
pinhole refraction can aid in the evaluation. A comprehensive slit-lamp
examination is indicated for all patients. All but the mildest of burns
develop a significant uveitis. In such cases, a clinician should appreciate cells
and flare in the anterior chamber. For pain relief and prevention of syne-
chiae (adhesion of the iris to the cornea or lens), cycloplegic eye drops
(eg, cyclopentolate 0.5%) are indicated. Phenylephrine is contraindicated
because it can exacerbate ischemic injury to deeper structures. Intraocular
pressure should be assessed after a burn and, if elevated, systemic treatment
with carbonic anhydrase inhibitors is indicated [6].
As with all patients presenting with superficial injury to the eye, a floures-
cein exam is indicated. Flourescein uptake occurs in all areas where the
chemical exposure produced injury. An ophthalmologist will follow the
observed injury pattern over time.
The goal of treatment is to promote epithelial cell growth as rapidly as
possible. Topical antibiotic ointment, such as erythromycin or tetracycline,
should be applied. Some investigators suggest topical corticosteroid to
reduce the inflammatory response to chemical injury (ie, prednisolone or
dexamethasone) [6]. However, this should be used in close collaboration
with an ophthalmologist [20]. Although a semipressure patch can be applied
to relieve pain [4,6], this has been shown to provide no benefit in the emer-
gent treatment of traumatic corneal abrasions [21–23]. Rapid follow-up with
an ophthalmologic specialist to manage care is vital.
Thermal injuries
Thermal injuries to the eye generally occur as a result of exposure to scald-
ing liquid, direct flame, or such items as cigarettes and curling irons.
Although a direct thermal injury to the eye is a rare event, burns to the
surrounding adnexal structures (ie, eyebrow, eyelid, eyelashes) are not
uncommon [6,20]. Due to the rapidity of the lid reflex, the eyelid provides
protection for the eye itself, and long-term visual acuity is preserved. How-
ever, the eyelid takes on the majority of insult in a thermal injury, which leads
to an inordinately high rate of postinjury lid contractures [24]. Much like
chemical burns, the severity of thermal eye burns is related to the duration
of exposure and the nature of the causative agent. Compared to water-based
liquids, hot oils and greases are more adherent and subsequently result in
deeper thermal injury [25,26]. The grading system for thermal burns to the
eyelid is similar to that for burns in other areas of skin [27]. First-degree or
superficial partial-thickness burns to the eyelid affect only the epidermal layer
of skin. This type of burn pattern is characterized by pain, erythema, and
edema. Second-degree or deep partial-thickness burns affect both the epider-
mal and dermal layers of skin, and also exhibit pain and erythema. However,
second-degree burns blister. Third-degree or full-thickness burns of the eye-

lid affect the entire dermal layer and may extend into the subcutaneous tissue.
Patients present with a black or white eschar over the adnexal structures of
the eye, which may lead to contractures. Full-thickness burns damage nerve
endings and are less painful or even painless.
Radiation injuries
UV radiation (wavelengths 295–400 nm) is primarily absorbed by the
corneal surface [28,29]. Prolonged unprotected exposure to sunlight, partic-
ularly at high elevation or with highly reflective surfaces, result in damage to
the corneal surface (eg, snow blindness) [6,30]. UV damage to the corneal
epithelium results in an inflammatory keratitis known as superficial punctu-
ate keratitis. The pinpoint corneal defects characteristic of superficial punc-
tuate keratitis can readily be found on flourescein staining. Additionally,
there is often a varying degree of conjunctival injection and eyelid edema.
The onset of pain and decreased visual acuity is delayed and occurs
approximately 6 to 12 hours after prolonged UV light exposure. Overall,
the epithelial layer heals within 72 hours of the injury, and the long-term
prognosis for UV burns is excellent.
Artificial sources of UV radiation include welding arcs, sun-tanning beds,
electric sparks, and halogen desk lamps. In contrast, prolonged exposure to
infrared radiation (wavelengths of O700 nm) damages the anterior lens and
may lead to cataract formation (eg, glassblowers cataracts) [6,30]. This oc-
cupational injury is rarely seen now that appropriate eye protection is used.
Prolonged exposure to visible solar light (wavelengths 400–700 nm) may
rarely result in retinal injury and subsequent visual loss [6,30]. This delayed
photochemical retinal injury most commonly occurs among beachgoers and
persons engaged in maritime industries who are exposed for long periods in
the sun without eye protection. Patients often present 1 to 2 days after
exposure with complaints of pain and visual change. On examination, there
are often minimal findings apart from a reduction in visual acuity. Although
no immediate management is necessary, outpatient ophthalmology follow-
up is necessary, as the visual loss may take several months to improve.
Treatment
The treatment for thermal injuries is comparable to treatment for chem-
ical burns. For all thermal injuries of the eye, the first priority is to remove
the individual from the source and to cool the tissues as rapidly as possible.
The eye should be irrigated with copious normal saline, lactated Ringer’s
solution, or tap water. Ideally, this should be instituted at the scene (eg,
coworkers at the worksite) or before emergency department evaluation
(eg, by pre–hospital-care providers), and should be continued in the emer-
gency department. The removal of adherent debris or retained contact lenses
is facilitated via copious irrigation. The initial use of topical anesthetic (eg,
proparacaine 0.5%) is suggested in the emergency department, but long-
term application of these agents delays epithelial healing. Cold compresses
should be applied to the affected areas to decrease thermal injury and to
relieve discomfort. Both thermal and UV injuries are quite painful, so
oral and parenteral nonsteroidal and narcotic analgesics should be pre-
scribed. Topical analgesics have not been shown to be superior to oral
agents [31,32]. Once pain control is addressed, visual acuity testing should
be performed to identify any baseline visual deficits. Pinhole refraction
may optimize visual acuity assessment among selected patients who arrive
in the emergency department without their corrective lenses. While evaluat-
ing the eye, the eyelids should be everted so that a complete inspection of the
stromal structures can be performed. Frequent application of artificial tears
or ointment should be instituted in the event that tear production has been
affected. This also helps to prevent long-term adhesion formation between
the eyelid and the globe itself. The patient’s tetanus status should be
updated. Systemic antibiotics are unnecessary unless the source of infection
has been identified.
In addition to the above actions, the specific treatment for first- and sec-
ond-degree thermal eye burns includes an antibiotic ointment (erythromycin
0.5%) or eye drops. Cycloplegic agents (eg, cyclopentolate 0.5%) are com-
monly administered. However, a recent systematic review found no compel-
ling evidence to support this practice [33]. For third-degree burns,
a nonocclusive dressing should be applied to prevent infection, and urgent
ophthalmologic consultation is warranted.
The traditional therapy for UV keratitis is cycloplegic drops (eg, cylco-
pentolate 0.5%) to reduce reflex ciliary muscle spasm and to potentially
reduce pain. However, as noted above, no studies have proven the efficacy
of this treatment [33]. Topical antibiotic drops or ointment (eg, erythromy-
cin 0.5%) should be prescribed. Although some ophthalmologists continue
to recommend eye patching, this is viewed as controversial and should be
done with caution [21–23].
Biologic exposures
In practical terms, a ‘‘biologic’’ exposure refers to contact with human
blood or body fluids (eg, saliva, semen, urine). The key issue that the emer-
gency physician must confront is that such an exposure may result in an infec-
tion with blood-borne pathogen. The most serious of these pathogens include
hepatitis B virus (HBV), hepatitis C virus, and HIV [34,35]. The infectious risk
increases if the biologic exposure (ie, blood, body fluid) comes into direct
contact with the exposed person’s bloodstream (eg, needle-stick incident).
However, exposure to the mucous membranes of the eye can potentially result
in infection. The risk of infection via exposure through mucous membranes or
injured skin is considerably less than that via a percutaneous needle-stick
event. Although the true risk is lower with mucous membrane exposures, the
risk is not zero [34]. The risk of infection is related to the amount (ie, volume)
and concentration (ie, viral load) of the biologic exposure.
Treatment
The most important action that the emergency physician can make in the
treatment of biologic exposures to the eye is to irrigate the eye with water or
normal saline. For those who wear contact lenses, recommendations call for
the lenses to be kept on during irrigation because the lens itself serves as
a protective barrier for the eye. The use of soap or other agents may irritate
and compromise the mucosal barrier of the eye. Lenses may be removed
after irrigation and either discarded or cleaned as per the usual manner.
Soap and water may be used, however, for biologic exposures to nonintact
skin of the orbital adnexal structures [34].
Of the three viral agents discussed, the risk of hepatitis B infection is
greatest. Fortunately, in the health care environment, most hospital person-
nel are vaccinated against HBV. Among unvaccinated persons, the emer-
gency department clinician should consider initiating treatment with HBV
vaccination in ocular exposures with blood from source individuals where
the HBV status is unknown. The first dose should be given at the time of
exposure; a follow-up vaccination is given at 1 month and 6 months
postexposure. If the blood is known to be from an HBV-positive source,
then hepatitis-B immune globulin, which contains antibodies to HBV, is
recommended [34,35].
The Centers for Disease Control and Prevention recommends postexpo-
sure prophylaxis to reduce HIV infection if the exposure is discovered within
72 hours and one or more of the following bodily fluids was involved: blood,
semen, vaginal secretions, rectal secretions, breast milk, or any body fluid
that is visibly contaminated with blood [35]. However, given that mucous
membranes of the eye are an extremely unlikely route of HIV transmission,
it is recommended that each situation be considered on an individual basis
[34,35].
Unfortunately, for biologic exposure to hepatitis C virus, there is no
known effective preventive measure apart from copious irrigation with
water or saline.
In cases of occupational biologic exposures to the eye, health care workers
may be referred to the hospital’s occupational health clinic for same-day or
next-day baseline serum testing, as well as follow care. All others should be
referred to a primary care provider for further management.
Disposition
Most first-degree thermal burns and radiation injuries may be discharged
from the emergency department with outpatient ophthalmology follow-up
in 24 to 48 hours. Second-degree burns of the eye merit at least a phone call

to the on-call ophthalmologist, or referral for urgent ophthalmology consul-
tation. Third-degree eye burns require ophthalmologic consultation and
either hospital admission or early burn-center transfer. All those with
biologic exposures should be referred for baseline and follow-up testing
and management.
References
[1] Schrage NF, Langefeld S, Zschocke J, et al. Eye burns: an emergency and continuing
problem. Burns 2000;26(8):689–99.
[2] Xiang H, Stallones L, Chen G, et al. Work-related eye injuries treated in hospital emergency
departments in the US. Am J Ind Med 2005;48(1):57–62.
[3] Wagoner MD. Chemical injuries of the eye: current concepts in pathophysiology and
therapy. Surv Ophthalmol 1997;41(4):275–313.
[4] Farjo AA, Soong HK. Corneal epithelium. In: Yanoff M, Duker JS, editors. Ophthalmol-
ogy. 2nd edition. St. Louis (MO): Mosby; 2004. p. 413–20.
[5] Charukamnoetkanok P, Wagoner M. Facial and ocular injuries associated with metham-
phetamine production accidents. Am J Ophthalmol 2004;138(5):875–6.
[6] Belin MW, Catalano RA, Scott JL. Burns of the eye. In: Catalano RA, Belin MW, editors.
Ocular emergencies. Philadelphia: WB Saunders; 1992. p. 179–96.
[7] Salzman M, O’Malley RN. Updates on the evaluation and management of caustic
exposures. Emerg Med Clin North Am 2007;25(2):459–76.
[8] Su M. Hydrofluoric acid and fluorides. In: Flomenbaum NE, Goldfrank LR, Hoffman RS,
et al, editors. Goldfrank’s toxicologic emergencies. 8th edition. New York: McGraw-Hill
Medical Publishing Division; 2002. p. 1417–23.
[9] Fisher AA. Reactions to cyanoacrylate adhesives: instant glue. Cutis 1985;35:18–24.
[10] Patel KC, Hussain U, Zia R. Cyanoacrylate injuries in the eye: a review of management.
2007. Available at: http://www.karnesh.com/downloads/documents/articles/cyanoacrylate.
pdf. Accessed October 8, 2007.
[11] Derespinis PA. Cyanoacrylate nail glue mistaken for eye drops. JAMA 1990;263(17):2301.
[12] McClean CJ. Ocular superglue injury. J Accid Emerg Med 1997;14(1):40–1.
[13] Rihawi S, Frentz M, Becker J, et al. The consequences of delayed intervention when treating
chemical eye burns. Graefes Arch Clin Exp Ophthalmol 2007;245(10):1507–13.
[14] Rihawi S, Frentz M, Schrage NF. Emergency treatment of eye burns: Which rinsing solution
should we choose. Graefes Arch Clin Exp Ophthalmol 2006;244(7):845–54.
[15] Ikeda N, Hayasaka S, Hayasaka Y, et al. Alkali burns of the eye: effect of immediate copious
irrigation with tap water on their severity. Ophthalmologica 2006;220(4):225–8.
[16] Hall AH, Maibach HI. Water decontamination of chemical skin/eye splashes: a critical
review. Cutan Ocul Toxicol 2006;25(2):67–83.
[17] Grant WM, Schuman JS. Treatment of chemical burns of the eye. In: Grant WM, Schulman
JS, editors. Toxicology of the eye. 4th edition. Springfield (MA): Thomas Books; 1993.
[18] Bertolini JC. Hydrofluoric acid: a review of toxicity. J Emerg Med 1992;10(2):163–8.
[19] Bentur Y, Tannenbaum S, Yaffe Y, et al. The role of calcium gluconate in the treatment of
hydrofluoric acid eye burn. Ann Emerg Med 1993;22(9):1488–90.
[20] Chen AI, Reenstra-Buras WR, Rosen C, et al. Burns, occular. Emedicine 2006. Available at:
http://www.emedicine.com/emerg/topic736.htm. Accessed August 9, 2007.
[21] Kaiser PK. A comparison of pressure patching versus no patching for corneal abrasions
due to trauma or foreign body removal. Corneal Abrasion Patching Study Group.
Ophthalmology 1995;102(12):1936–42.
[22] Flynn CA, D’Amico F, Smith G. Should we patch corneal abrasions? A meta-analysis.
J Fam Pract 1998;47(4):264–70.
[23] Le Sage N, Verreault R, Rochette L. Efficacy of eye patching for traumatic corneal
abrasions: a controlled clinical trial. Ann Emerg Med 2001;38(2):129–34.
[24] Stern JD, Goldfarb IW, Slater H. Ophthalmological complications as a manifestation of
burn injury. Burns 1996;22(2):135–6.
[25] Schubert W, Ahrenholz DH, Solem LD. Burns from hot oil and grease: a public health haz-
ard. J Burn Care Rehabil 1990;11(6):558–62.
[26] Allen SR, Kagan RJ. Grease fryers: a significant danger to children. J Burn Care Rehabil
2004;25(5):456–60.
[27] Pham TN, Gibran NS. Thermal and electrical injuries. Surg Clin North Am 2007;87(1):
185–206.
[28] Taylor HR. The biological effects of UV-B on the eye. Photochem Photobiol 1989;50(4):
489–92.
[29] Roberts JE. Ocular phototoxicity. J Photochem Photobiol B 2001;64(2–3):136–43.
[30] Brozen R, Fromm C. Ultraviolet keratitis. Emedicine 2006. Available at: http://www.
emedicine.com/EMERG/topic759.htm. Accessed August 9, 2007.
[31] Szucs PA, Nashed AH, Allegra JR, et al. Safety and efficacy of diclofenac ophthalmic
solution in the treatment of corneal abrasions. Ann Emerg Med 2000;35(2):131–7.
[32] Weaver CS, Terrell KM. Evidence-based emergency medicine. Update: Do ophthalmic non-
steroidal anti-inflammatory drugs reduce the pain associated with simple corneal abrasions
without delayed healing? Ann Emerg Med 2003;41(1):134–40.
[33] Carley F, Carley S. Towards evidence based emergency medicine: best BETs from the Man-
chester Royal Infirmary. Mydriatics in corneal abrasion. Emerg Med J 2001;18(4):273.
[34] Zaleznik D. Patient information: blood and body fluid exposure. 2007. Available at:
http://patients.uptodate.com/topic.asp?file¼inf_immu/8025. Accessed October 18, 2007.
[35] CDC. Exposure to blood: what healthcare personnel need to know. 2003. Available at:
http://www.cdc.gov/ncidod/dhqp/pdf/bbp/Exp_to_Blood.pdf. Accessed October 18, 2007.
Emerg Med Clin N Am
26 (2008) 137–180
Neuro-Ophthalmology
David K. Duong, MD, MSa, Megan M. Leo, MDa,
Elizabeth L. Mitchell, MDb,*
a
Emergency Medicine Residency, Boston Medical Center, 1 Boston
Medical Center Place, Boston, MA 02118, USA
b
Department of Emergency Medicine, Boston University School of Medicine, Boston
Medical Center, 1 Boston Medical Center Place, Dowling 1, Boston, MA 02118, USA
Neuroanatomy and neuro-ophthalmologic examination

Neuro-ophthalmologic disorders arise from all areas of the neuro-
ophthalmologic tract. They may be expressed simply as loss of vision or
double vision, or as complex syndromes or systemic illnesses, depending
on the location and type of lesion. Problems may occur anywhere along
the visual pathway, including the brainstem, cavernous sinus, subarachnoid
space, and orbital apex, and may affect adjacent structures also. A firm
understanding of the neuroanatomy and neurophysiology of the eye is
essential to correct diagnosis [1–3].
The visual pathway

Functionality of the visual pathway requires the afferent innervation of the
optic nerve, cranial nerve (CN) II. A lesion can occur anywhere along the
pathway and is manifested according to distinct location and fiber involve-
ment. An adequate understanding of the visual pathway from the retina,
through the optic nerve and chiasm, with projection dorsally to the occipital
lobe, allows for localization of the insult (Fig. 1). The fibers from the tempo-
ral retina (capturing the images from the nasal visual field) and nasal retina
(capturing images from the temporal visual field) converge at the optic disc
and proceed dorsally to form the optic nerve. The ophthalmic artery supplies
the retina and optic nerve. It is the first intracranial division off the internal
carotid artery. The optic nerve is subject to ischemic, compressive, infiltra-
tive, or inflammatory damage because of its vulnerability to increased
E-mail address: [email protected] (E.L. Mitchell).
138 DUONG et al
Fig. 1. Lesions of the afferent visual pathway and their corresponding visual field defects. The
term hemianopsia (or hemianopia) refers to a visual field defect that respects the vertical merid-
ian. Homonymous indicates that the defect involves the same side of the visual field in both
eyes. An incongruous defect is that for which the extent of visual field loss is asymmetric
between the two eyes. (Adapted from Mason C, Kandel ER. Central visual pathways. In:
Kandel ER, Schwartz JH, Jessell T, editors. Principles of neural science. 3rd edition. Norwalk
(CT): Appleton & Lange; 1991. p. 437; with permission.)
intraocular pressure or papilledema. An optic nerve deficit may result in

unilateral, complete vision loss, without macular sparing.
As the fibers approach the optic chiasm intracranially, the temporal fibers
remain ipsilateral and the nasal fibers cross within the chiasm to join the
contralateral optic tracts. The optic chiasm is located directly above the
pituitary fossa, anterior to the pituitary stalk, and inferior to the hypothal-
amus and third ventricle. Infarction of the optic chiasm is extremely rare
because of its collateral blood supply. The superior hypophyseal arteries
perfuse the chiasm inferiorly, which are fed by the internal carotid, posterior
communicating, and posterior cerebral arteries. Superiorly the optic chiasm
is perfused by the branches of the anterior cerebral arteries. A lesion in the
chiasm classically presents as bitemporal hemianopsia, although this can
vary depending on the specific area of the chiasm that is affected (see Fig. 1).
Posterior to the chiasm, the optic fibers course superiorly and around the
infundibulum, below the third ventricle. They contain fibers from the ipsilat-
eral temporal retina and the contralateral nasal retina. Most of the blood
supply is from the thalamic perforators of the posterior cerebral artery
and branches of the anterior choroidal artery. Most of the fibers synapse
NEURO-OPHTHALMOLOGY 139
in the lateral geniculate nucleus (LGN), whereas those axons that are
responsible for the pupillary light reflex branch off before the LGN and
synapse in the pretectal nuclei of the midbrain.
The optic radiations that emerge from the LGN are called the geniculo-
calcarine fibers. These separate into superior and inferior bundles, contain-
ing the afferent signal from the contralateral inferior visual fields and the
contralateral superior visual fields, respectively. The superior bundles that
pass through the parietal lobe project back to the upper bank of the calcar-
ine cortex of the occipital lobe, thus receiving the image from the contralat-
eral inferior visual field. The inferior bundles pass through the temporal lobe
to form the Meyer loop and project back to the lower bank of the calcarine
cortex of the occipital lobe, thus receiving the image from the contralateral
superior visual field. The optic radiations receive blood supply from the
middle cerebral arteries and anterior choroidal arteries. The macular area
of the visual cortex, which represents the central 30 of vision, is processed
by one half of the visual cortex. The fovea, or very center of the visual field,
is represented within the tips of the occipital poles and has a dual blood sup-
ply, including branches of the posterior and middle cerebral arteries, provid-
ing the basis for macular sparing in the setting of occipital lobe infarct.
Efferent and afferent pupillary innervation

The efferent innervation of the pupil, which is responsible for pupillary
constriction and dilation, involves parasympathetic and sympathetic fibers.
Afferent pupillary innervation involves the retina, optic nerve, chiasm, optic
tract and optic radiations.
The parasympathetic preganglionic fibers originate in the Edinger-
Westphal subnucleus, adjacent to the oculomotor nucleus in the periaqueduc-
tal midbrain. The fibers travel on the superficial aspect of the oculomotor
nerve (CN III) until it synapses in the ciliary ganglion in the orbit. The super-
ficial location of the parasympathetic fibers explains why a blown pupil is an
early sign of a compression lesion, including aneurysm or uncal herniation.
From the ciliary ganglion, the postganglionic nerve fibers travel to the ciliary
muscle and the iris sphincter and provide the tone for pupillary constriction.
The pupillary light reflex pathway, allowing the pupil to constrict when
exposed to light, consists of four neurons (Fig. 2). The image obtained
from the retinal ganglionic cells travels through the optic nerve and follows
the nasal retinal fibers across the chiasm, where they synapse in both of the
pretectal nuclei of the dorsal midbrain. Each pretectal nucleus sends axons
bilaterally to the two Edinger-Westphal nuclei, which contain the parasympa-
thetic preganglionic fibers. These fibers travel with the third nerve to the
ciliary ganglion within the orbit, which then sends parasympathetic postgan-
glionic fibers to the pupillary sphincter muscle and ciliary muscle for lens
accommodation. The projections to bilateral pretectal and Edinger-Westphal
nuclei explain consensual pupillary constriction to light. Near inputs, such as
140 DUONG et al
Fig. 2. The pupillary light reflex pathway. (Adapted from Walsh FB, Hoyt WF. The autonomic
nervous system. In: Clinical neuro-ophthalmology. 3rd edition. Baltimore (MD): Williams &
Wilkins; 1969. p. 473; with permission.)
an object placed directly in front of a patient being examined, project directly

to both Edinger-Westphal nuclei, bypassing the pretectal area, which can
serve to distinguish some forms of light-near dissociation in which the dorsal
midbrain is involved. Adie syndrome, or Adie tonic pupil, is an example of
light-near dissociation.
The sympathetic pathway originates in the posterolateral hypothalamus
and descends along the lateral brainstem to synapse at the ciliospinal center
of Budge-Waller, at the level of C8-T2 (Fig. 3). The preganglionic fibers exit
the spinal cord with the ventral rootlets at this level and travel across the
apex of the lung before ascending the neck to synapse in the superior cervi-
cal ganglion. The postsympathetic fibers form a plexus around the cervical
internal carotid artery and reenter the skull. The oculosympathetic fibers
leave the carotid artery in the cavernous sinus and enter the orbit with the
ophthalmic division of the trigeminal nerve. The sympathetic fibers continue
with the long ciliary nerves to the radial iris muscle, causing pupillary dila-
tation. There are also fibers that innervate the smooth muscle in the upper
and lower eyelids (Mueller muscle) and the blood vessels in the skin
and conjunctiva. Classic symptoms of sympathetic dysfunction, Horner
Fig. 3. The autonomic innervation of the pupil. The two-neuron parasympathetic pathway
begins in the Edinger-Westphal nucleus of the midbrain and synapses in the ciliary ganglion
in the orbit. Short ciliary nerves innervate the iris sphincter. The three-neuron sympathetic path-
way begins in the hypothalamus, synapses in the lower cervical cord, ascends the neck on the
carotid artery, and synapses again in the superior cervical ganglion to innervate the pupillodi-
lator. (From Selhorst J. The pupil and its disorders. Neurol Clin 1983;1:861; with permission.)
syndrome, occur ipsilateral to the side of the lesion and include mild ptosis,
papillary miosis, and inconsistently anhidrosis (Fig. 4). There is a small area
of sweat glands above the eyebrows innervated by branches directly from
the superior cervical ganglion, which explains why some third-order neuron
Horner syndromes do not involve anhidrosis.
The cranial nerves

The five cranial nerves that innervate the eye are the optic nerve (CN II),
the oculomotor nerve (CN III), the trochlear nerve (CN IV), the abducens
nerve (CN VI), and the first division of the trigeminal nerve (CN V1). A
review of the course of these cranial nerves is important for understanding
ocular pathology.
142 DUONG et al
Fig. 4. A patient who had a left Horner syndrome secondary to an apical lung tumor. (From
Balcer LJ, Galetta SL. Pancoast’s syndrome. N Engl J Med 1997;337:359; with permission.
Copyright Ó 1997, Massachusetts Medical Society.)
Ocular motility
Eye movement is controlled by a group of muscles innervated by the
oculomotor nerve (CN III), the trochlear nerve (CN IV), and the abducens
nerve (CN VI). The coordinated action of these muscles is accomplished by
supranuclear control and is what allows for conjugate gaze of both eyes (Fig. 5).
Cranial nerve III. The oculomotor nerve (CN III) innervates the superior
rectus muscle, inferior rectus muscle, medial rectus muscle, inferior oblique,
levator palpebrae, and ciliary ganglion, providing motor and parasympa-
thetic innervation to the eye. Partial dysfunction is common but a classic dys-
function of the entire nerve results in ptosis (attributable to loss of levator
palpebrae function), papillary dilation (with loss of parasympathetic tone
of ciliary ganglion), and the eye deviated down and out (attributable to the
sole action of superior oblique and lateral rectus muscles) (Fig. 6). The origin
Fig. 5. The extraocular muscles. The medial rectus, superior rectus, inferior rectus, and inferior
oblique are innervated by the third nerve (III); the superior oblique is innervated by the fourth
nerve (IV); the lateral rectus is innervated by the sixth nerve (VI). (From Patten J. Vision, the
visual fields, and the olfactory nerve. In: Patten J, editor. Neurological differential diagnosis.
2nd edition. New York: Springer-Verlag; 1996; with permission.)
Fig. 6. Complete right third nerve palsy resulting in hypotropia, exotropia, and pupillary
mydriasis. The ptotic eyelid is elevated manually. (From Bennett JL, Pelak VS. Palsies of the
third, fourth, and sixth cranial nerves. Ophthalmol Clin North Am 2001;14(1):169–85; with
permission.)
of the oculomotor nerve is in the periaqueductal midbrain, from a paired nu-

clear complex (Fig. 7). Within the complex are several paired subnuclei that
send projections ipsilaterally to its target organ. The two exceptions to this
are the axons originating from the unpaired midline central caudal nucleus,
which innervate the levator palpebrae muscles, and the axons innervating
the superior rectus muscles, which travel from the contralateral subnucleus.
The parasympathetic preganglionic axons originate from the Edinger-
Westphal nuclei, then travel to innervate the ciliary ganglion.
The fascicles that form from the oculomotor nuclei project anteriorly
through the midbrain, passing through the red nucleus, then run adjacent
to the substantia nigra and cerebral peduncle. The oculomotor nerve
emerges from the ventral surface of the midbrain, where it passes between
the superior cerebellar artery and the posterior cerebral artery. It is at this
point that it is vulnerable to aneurysmal compression. Within the subarach-
noid space, the nerve travels adjacent to the posterior communicating artery
and the tip of the basilar artery, making a CN III deficit one of the ominous
signs of aneurysm in this area. The oculomotor nerve continues to track
anteriorly and enters the cavernous sinus and runs along the wall where it
lies superior to CN IV. As the nerve exits the cavernous sinus, it passes
through the superior orbital fissure and divides into superior and inferior
branches. The superior branch provides motor function to the superior
rectus and levator palpebrae, whereas the inferior branch provides motor
function to the inferior rectus, medial rectus, inferior oblique, and the para-
sympathetic fibers for the ciliary ganglion.
Cranial nerve IV. The trochlear nerve (CN IV) innervates the superior
oblique muscle (see Fig. 5). The dysfunction of this muscle is the most
common cause of vertical strabismus. The superior oblique muscle depresses
the eye in adduction and intorts the eye in abduction, making the deficit
more apparent and exaggerating symptoms when the gaze is deviated down-
ward and inward toward the affected side (Fig. 8). The nucleus is dorsal to
the medial longitudinal fasciculus and inferior to the oculomotor nuclear
144 DUONG et al
Fig. 7. Anatomy of the oculomotor nerve. The dotted lines depict the crossed fibers emanating
from the superior rectus subnucleus. ICA, internal carotid artery; III, oculomotor nerve; IV, troch-
lear nerve; ON, optic nerve; PCA, posterior cerebral artery; SCA, superior cerebellar artery; VI,
abducens nerve; V1, trigeminal nerve, ophthalmic division; V2, trigeminal nerve, maxillary division.
(From Bennett JL, Pelak VS. Palsies of the third, fourth, and sixth cranial nerves. Ophthalmol Clin
North Am 2001;14(1):169–85; with permission.)
Fig. 8. Left fourth nerve palsy in a patient who had head trauma. There is a significant left
hypertropia in down and right gaze. (From Bennett JL, Pelak VS. Palsies of the third, fourth,
and sixth cranial nerves. Ophthalmol Clin North Am 2001;14(1):169–85; with permission.)
complex within the midbrain (Fig. 9). As the fascicles leave the nucleus, they
course posteriorly and inferiorly, where they decussate in the superior med-
ullary velum of the dorsal midbrain. It emerges at this point at the level of
the inferior colliculus and runs within the subarachnoid space anteriorly, be-
tween the lateral midbrain and the tentorium cerebelli, between the superior
cerebellar and posterior cerebral arteries. The trochlear nerve has the longest
course outside of the midbrain. It courses around the cerebral peduncle as it
enters the cavernous sinus, which makes it the most vulnerable cranial nerve
to injury. Within the cavernous sinus, the trochlear nerve holds a position
that is inferior to the oculomotor nerve and superior to the first division
of the trigeminal nerve. The trochlear nerve then passes through the superior
orbital fissure to enter the orbit and innervate the superior oblique muscle.
Fig. 9. Anatomy of the trochlear nerve. The speckled area demarcates the descending pregan-
glionic sympathetic fibers. ICA, internal carotid artery; III, oculomotor nerve; IV, trochlear
nerve; MLF, medial longitudinal fasciculus; PCA, posterior cerebral artery; SCA, superior
cerebellar artery; VI, abducens nerve; V1, trigeminal nerve, ophthalmic division; V2, trigeminal
nerve, maxillary division. (From Bennett JL, Pelak VS. Palsies of the third, fourth, and sixth
cranial nerves. Ophthalmol Clin North Am 2001;14(1):169–85; with permission.)
146 DUONG et al
Cranial nerve VI. The abducens nerve (CN VI) innervates the lateral rectus
muscle (see Fig. 5). Paresis of this muscle results in binocular, horizontal
diplopia that is worse in the direction of the affected side. Examination
reveals an inability to abduct the affected eye on lateral gaze (Fig. 10).
The abducens nerve fascicles originate in the dorsal pons, on the floor of
the fourth ventricle, adjacent to the medial longitudinal fasciculus (MLF),
paramedian pontine reticular formation (PPRF), and the fascicles of the
facial nerve (CN VII). These fascicles are grouped to innervate the ipsilateral
lateral rectus muscle and another group to innervate the contralateral
medial rectus subnucleus by way of the MLF. The fascicles that course to
the lateral rectus muscle project ventrally from the abducens nucleus and
course through the tegmentum of the pons, where it emerges caudally.
The nerve courses superiorly along the clivus to the level of the petroclinoid
(Gruber) ligament, where it leaves the subarachnoid space and enters the
Dorello canal and into the cavernous sinus. Within the cavernous sinus,
the abducens nerve courses freely along with the carotid artery ventrally
until it leaves the cavernous sinus and enters the orbit through the superior
orbital fissure (Fig. 11). The abducens nerve is often the first deficit to be
seen with cavernous sinus pathology because of its vulnerable position
within the space.
Corneal reflex. The corneal reflex is accomplished by the afferent action of

the ophthalmic branch of the trigeminal nerve (CN V1) and the efferent
action of the facial nerve (CN VII). When the cornea is touched, an impulse
is sent by way of CN V1 to the chief sensory nucleus of CN V, which is located
in the rostral pons. Axons then project bilaterally to motor nucleus of CN
VII, leading to coordinated eye closure with touch stimulation of CN V1.
Conjugate eye movements. The coordination of eye movements in a deter-

mined direction is orchestrated by a supranuclear and internuclear pathway
between the frontal eye fields and the contralateral side of the body. The
Fig. 10. Left abducens palsy caused by vasculopathic injury. There is a large angle esotropia in
left lateral gaze. (Data from Bennett JL, Pelak VS. Palsies of the third, fourth, and sixth cranial
nerves. Ophthalmol Clin North Am 2001;14(1):169–85.)
Fig. 11. Anatomy of the abducens nerve. The speckled area demarcates the paramedian
pontine reticular formation. ICA, internal carotid artery; III, oculomotor nerve; IV, trochlear
nerve; MLF, medial longitudinal fasciculus; VI, abducens nerve; V1, trigeminal nerve, ophthal-
mic division; V2, trigeminal nerve, maxillary division. (Data from Bennett JL, Pelak VS. Palsies
of the third, fourth, and sixth cranial nerves. Ophthalmol Clin North Am 2001;14(1):169–85.)
frontal eye fields send a signal to the superior colliculus, the contralateral
PPRF, and the rostral interstitial nucleus of the MLF (Fig. 12). Axons
from the cell bodies in the PPRF project to the ipsilateral sixth nerve nucleus
and synapse. Axons of the abducens motor nucleus travel to the ipsilateral
lateral rectus muscle and axons from the abducens internuclear neurons
cross over and ascend the contralateral MLF to the medial rectus subnu-
cleus of the third cranial nerve. This internuclear connection between the
PPRF of CN VI and the contralateral third nerve nucleus by way of the
MLF allows for horizontal conjugate gaze. Distinction between sixth nerve
palsies and lesions of the PPRF, or internuclear ophthalmoplegia (INO),
can be distinguished by performing the oculocephalic (doll’s eyes) maneuver
or caloric testing. One of these maneuvers will overcome the ipsilateral
horizontal gaze palsy if the sixth nerve is intact, whereas a PPRF lesion
prevents the voluntary movement of the eye past midline.
The vestibulo-ocular system allows for stabilization of conjugate vertical
and horizontal gaze. Inputs from each vestibular nucleus produce conjugate
148 DUONG et al
horizontal gaze by sending signals to the contralateral sixth nerve nucleus,

serving the lateral rectus muscle. Interneurons then cross back by way of
the MLF ipsilateral to the vestibular nucleus to allow for horizontal gaze
toward the contralateral size of the body (Fig. 13). Vertical gaze holding
occurs through the contralateral fourth nerve nucleus, third nerve nucleus,
INO, and MLF. Vertical alignment is maintained by coordination through
the fourth nerve nucleus (contralateral superior oblique muscle) and the
third nerve subnuclei (contralateral superior rectus muscle, ipsilateral infe-
rior oblique muscle, and inferior rectus muscle).
Neuro-ophthalmologic examination
Visual acuity
Visual acuity is a vital part of the neuro-ophthalmologic examination.
Any change in visual acuity should be noted. Please refer to the article by
Robinett and Kahn elsewhere in this issue.
Visual fields
Testing the visual field is essential for the neuro-ophthalmology evalua-
tion. The visual field of each eye should be assessed individually. The patient
begins by covering one eye and fixating on the nose of the examiner. The
patient should note any obvious differences in upper and lower face. Hemi-
anopic defects can be detected in this manner. If the nose is not clear to the
patient, he or she likely has a central scotoma. The patient should then
count fingers in the four quadrants. Simultaneous presentations of fingers
in two separate quadrants can increase the sensitivity of finding a visual field
defect. If a defect is found, the hand should be moved from the defected field
to the normal one to establish the boundaries of the defect. Recordings of
the visual fields are taken from the patient’s perspective. If the cap held
off midline is a clearer red, a central scotoma is suggested. By mapping
out the visual field defect, the location of the lesion can be isolated using
the anatomy of the neuro-ophthalmic pathways (see Fig. 1).
Visual field defects may be associated with higher cortical functioning, such
as neglect (parietal lesion) or visual agnosia. Neglect is present if a patient can
perceive a single stimulus (such as a finger), but on introduction of a second
stimulus, one stimulus is neglected. An example of visual agnosia is prosopag-
nosia, which is a condition in which the patient cannot recognize familiar
faces. These defects should be distinguished from pure visual field defects.
:
Fig. 12. Summary of eye movement control. The center shows the supranuclear connections
from the frontal eye fields (FEF) and the parieto-occipital-temporal junction region (POT) to
the superior colliculus (SC), rostral interstitial nucleus of the medial longitudinal fasciculus
(riMLF), and the paramedian pontine reticular formation (PPRF). The FEF and SC are
involved in the production of saccades, whereas the POT is believed to be important in the
production of pursuit. The left shows the brainstem pathways for horizontal gaze. Axons
from the cell bodies located in the PPRF travel to the ipsilateral sixth nerve (abducens) nucleus
(VI) where they synapse with abducens motoneurons whose axons travel to the ipsilateral
lateral rectus muscle (LR) and with abducens internuclear neurons whose axons cross the
midline and travel in the medial longitudinal fasciculus (MLF) to the portions of the third nerve
(oculomotor) nucleus (III) concerned with medial rectus (MR) function (in the contralateral
eye). The right shows the brainstem pathways for vertical gaze. Important structures include
the riMLF, PPRF, the interstitial nucleus of Cajal (INC), and the posterior commissure
(PC). Note that axons from cell bodies located in the vestibular nuclei (VN) travel directly to
the sixth nerve nuclei and, mostly by way of the MLF, to the third (III) and fourth (IV) nerve
nuclei. (From Miller NR. Neural control of eye movements. In: Miller RN, editor. Walsh and
Hoyt’s Clinical Neuro-Ophthalmology. 4th edition. Baltimore (MD): Williams & Wilkins; 1985.
p. 627; with permission.)
150 DUONG et al
Fig. 13. The main excitatory vestibulo-ocular connections from the vertical semicircular canals.
Dashed line, midline of the brainstem; arrows, directions of eye movement when individual
extraocular muscles are stimulated; solid circles, receivers of the anterior canal projection;
open circles, receivers of the posterior canal projection. Lesions occurring within these vesti-
bulo-ocular pathways result in skew deviation. III, third nerve (oculomotor) subnuclei; IV,
fourth nerve (trochlear) nucleus; IO, inferior oblique muscle; IR, inferior rectus muscle; SCC,
semicircular canals; SO, superior oblique muscle; SR, superior rectus muscle; VN, vestibular
nuclei. (Adapted from Zee DS. The organization of the brainstem ocular motor subnuclei.
Ann Neurol 1978;14:384; with permission. Copyright Ó 1978. Reprinted with permission of
John Wiley & Sons, Inc.)
Funduscopic examination
Evaluation of the visual pathway is not complete without a funduscopic
examination. A swollen and elevated optic disc may be present in a patient
who has papilledema. In other conditions, the optic disc may be pale and
atrophic. The presence of spontaneous venous pulsations should be noted,
which suggests that the intracranial pressure is not elevated. Getting a gen-
eral sense of the condition of the retina can help guide a differential diagno-
sis for visual complaints (see the article by Robinette and Kahn elsewhere in
this issue for a more complete discussion of the funduscopic examination).
Testing efferent and afferent pupillary innervation

Pupillary size is determined by the efferent dilating tone of the sympathetic
nervous system and the efferent constricting tone of the parasympathetic
nervous system. The pupils should be assessed for size and reactivity to light
with the patient’s vision fixated on an object in the distance. The pupils
should be round and equal in diameter, although 1 mm of inequality may
be a normal variation. Direct response to light is assessed one eye at a time
by bringing the light source from below to avoid triggering near reaction.
Near response is tested by having the patient look at an object, such as
a pen, starting at 14 in away. The examiner should note the pupillary change
as the object is drawn closer. Pupillary constriction indicates a response to
convergence. An absent response to light with normal response to conver-
gence suggests the presence of light-near dissociation. Argyll Robertson
pupil is an example of this and is often seen in patients who have untreated
syphilis or encephalitis that has damaged the pretectal area of the dorsal
midbrain. These pupils also dilate with anticholinergic eye drops but do
not constrict with light because of the damaged pretectal area.
Afferent pupillary defects (APD) are examined using the swinging flash-
light test. Marcus Gunn pupil is an example of an APD in which the pupils
do not react equally to light, indicating a disturbance in the anterior afferent
visual pathway, including retina, optic nerve, chiasm, or optic tract. Ask the
patient to fixate on an object at a distance. A light is directed in the pupil for 1
to 2 seconds and the light is moved rapidly across the bridge of the nose to the
other eye and moved back in a rhythmic fashion to equally stimulate both
eyes (Fig. 14). The pupillary size should be compared bilaterally. In the pres-
ence of an afferent pupillary defect, there is a paradoxic dilation in the
affected eye as light is directed into it, despite a normal consensual
response (Fig. 15). Afferent pupillary defects can also be uncovered indirectly
in the setting of a third nerve palsy with a dilated pupil by shining a light in the
affected eye and observing the response in the opposite eye. Adequate con-
striction in the opposite eye indicates an intact optic nerve in the interrogated
eye, despite its lack of direct response. Another example of a tonically dilated
pupil with intact optic nerve is Adie tonic pupil, a lesion of the ciliary gan-
glion, which holds the cell bodies for the postsynaptic parasympathetic fibers
that allow pupillary constriction. The lesion is often unilateral and in the
setting of a traumatic or postviral injury. At about 8 weeks past the initial
insult, the accommodating fibers can regenerate and the pupil begins to act
like a light-near dissociation with improvement in the patient’s near vision.
The corneal reflex can be tested by touching the cornea lightly with the
edge of a sterile piece of gauze. A lack of response indicates a deficit in
the afferent fibers of CN V1 or the efferent fibers of CN VII. Testing both
corneas is important to reveal direct and consensual reflexes.
Testing ocular motility

The motility examination assesses the integrity of the supranuclear path-
ways, ocular motor nuclei and nerves, and the muscles they innervate. The
examination should begin with eyelid examination, observing for asymmetry
and lid retraction, which could suggest CN III palsy or thyroid disease,
152 DUONG et al
Fig. 14. The swinging flashlight test is used to test the integrity of the afferent visual pathway.
In this example, the left optic nerve is not functioning properly, and there is a paradoxic dilation
of the left pupil as the light is directed into it (left relative afferent pupillary defect). (From
Wilhelm H. Neuro-ophthalmology of pupilary function–practical guidelines. J Neurol
1998;245:573–83; with permission.)
respectively. Myasthenia gravis is suggested when fatigable ptosis or Cogan

lid twitch sign (transient improvement of lid function after sustained down
gaze) is present. Ocular deviation is assessed from the neutral position and
is classified as esotropia with inward displacement, exotropia with outward
displacement, or hypertropia with vertical displacement. Corneal light reflex,
in which a penlight is held directly in front of the patient, can be used to
compare position as the light reflects off the cornea. Deviation is suggestive
of strabismus, extraocular muscle dysfunction, or oculomotor nerve
dysfunction.
Diplopia is often binocular, although monocular diplopia can be caused
by early cataract formation or astigmatism and can be relieved by viewing
through a pinhole device. Palsies of the third, fourth, or sixth cranial nerves
or the muscles they innervate result in binocular diplopia, typical of paretic
strabismus. In assessing a patient who has complaints of diplopia, it is
important to establish whether the double vision is vertical, horizontal, or
oblique, and determine in which direction the two images are most widely
separated. The patient is then taken through the six cardinal fields of gaze
to localize any involved nerve or muscle. This examination is usually done
by asking the patient to follow an object, usually the examiner’s finger, to
the patient’s upper right, upper left, lower left, lower right, right, and left,
Fig. 15. The alternating light test in a patient who had a right optic neuropathy. (A) Patient is
fixating a distant target while in dim room light. Light stimulation of the good left eye produces
brisk, strong pupilloconstriction in both eyes. Clinically, the observer watches only the reaction
of the illuminated pupil. (B and C) Light stimulation of the right eye produces lesser pupillo-
constriction compared with light stimulation of the good left eye. (D) Rapid alternation of
the light stimulus back to the bad right eye results in pupillodilation because that eye ‘‘sees’’
a relative decrement in light intensity. There is thus a relative afferent pupillary defect in the
right eye. (From Kawasaki A, Kardon R. Disorders of the pupil. Ophthalmol Clin North
Am 2001;14(1):149–68; with permission.)
returning to midline between each field. Any paralysis or weakness of the

extraocular muscles can be isolated in this manner. Nystagmus is also
observed if present in any of these six cardinal positions. Horizontal nystag-
mus usually indicates a lesion in the cerebellar or vestibular pathways,
whereas downbeat nystagmus localizes to the cervicomedullary junction.
Saccades or fast eye movements are assessed by having the patient fixate
on two targets, usually the examiner’s nose and an eccentrically placed
finger. Have the patient follow a swinging reflex hammer from side to side
to test pursuit. Pursuit can also be assessed vertically by moving the reflex
hammer up and down in a constant manner. Lastly, the vestibular ocular
reflex (VOR) can be tested by having the patient fixate on the examiner’s
finger while rotating the head from side to side. A defective VOR results
in catch-up saccadic eye movements.
154 DUONG et al
Finally, a complete neurologic examination should be done on any

patient in whom a neuro-ophthalmologic problem is suspected. Most lesions
involving the optic tract, cranial nerves, or occipital lobe affect adjacent
structures. These are manifest in physical examination findings consistent
with pathology in those areas.
Pupillary disorders
Neurologic dysfunction of the pupils may be classified as an afferent or
efferent defect based on the neuroanatomic site of the lesion. An afferent
pupillary lesion refers to the retina, the optic nerve, chiasm, and tract,
and the optic radiations. An efferent pupillary lesion refers to lesions of
parasympathetic and sympathetic innervation, controlling constriction and
dilation, respectively (see Fig. 3). Disorders of the pupils may also be defined
by a clinical presentation, such as asymmetry of pupils, bilaterally abnormal
pupil size, abnormal light reactivity, or deficits with other associated neuro-
logic abnormalities. For the purposes of this article, we describe pupillary
disorders by the defect and clinical presentation.
Pupil size and reactivity

Anisocoria
Anisocoria is defined as asymmetry of pupillary size. Physiologic, or
simple, anisocoria occurs in about 20% of people [4,5]. The asymmetry is
more prominent in dim light and rarely more than 1 mm. On examination,
there is symmetric, rapid papillary constriction and dilatation. There has
been no clear physiologic explanation, but it is believed to be attributable
to an asymmetric supranuclear inhibition of the Edinger-Westphal parasym-
pathetic nuclei in the pupilloconstrictor pathway [5].
Horner syndrome
Horner syndrome is classically described as ptosis, miosis, and anhidro-
sis. Ptosis and anhidrosis may be present to varying degrees, however,
and may be clinically subtle (Fig. 16). When anisocoria is present and there
is sluggish dilation of the miotic pupil, one should suspect Horner syn-
drome. The pupillary lag in dilation takes about 15 to 20 seconds, with the
most prominent asymmetry at 4 to 5 seconds [5,6]. Normally, or in the case
of physiologic anisocoria, there is no such lag and the pupils dilate with equal
speed. Pupillary lag is present in about 50% of those who have Horner
syndrome [5]. Ptosis is attributable to denervation of the tarsal muscles. The
degree of ptosis is usually mild, partly because of compensation of the levator
palpebrae and frontalis muscles. Anhidrosis is not always present, although its
presence can be helpful diagnostically and usually implies an acute cause,
because there is reinnervation of sweat glands in chronic cases. If Horner
syndrome is present, localization of the lesion is the next step because the
Fig. 16. (A) Right upper lid ptosis and ipsilateral miosis in a patient who had pseudo-Horner
syndrome (levator dehiscence and simple anisocoria). (B) Right upper and lower lid ptosis and
ipsilateral miosis in a patient who had true Horner syndrome (oculosympathetic defect). (From
Kawasaki A, Kardon R. Disorders of the pupil. Ophthalmol Clin North Am 2001;14(1):149–68;
with permission.)
differential is broad and ranges from benign to serious (Box 1). There should
be a search for associated signs and symptoms to help localize the lesion. For
instance, if anhidrosis is present over the entire body, this indicates a central
lesion. If anhidrosis affects the face, neck, and arm only, there is a cervical
lesion. Consultation with neurology or ophthalmology for pharmacologic
tests (cocaine or hydroxyamphetamine provocation) may be necessary. For
further localization, imaging is usually indicated.
Tonic (Adie) pupil

Another cause of a unilateral sluggishly reactive pupil is Adie, or tonic,
pupil. Adie pupil is caused by degeneration of the ciliary ganglion, followed
by aberrant reinnervation of the pupilloconstrictor muscles. On slit-lamp
examination, segmental pupillary sphincter palsies may be appreciated.
Most cases are idiopathic, with a minority of cases caused by ocular trauma
or surgery, infections such as zoster, neoplasm, or ischemia from temporal
arteritis. In general, a finding of a tonic pupil requires no further evaluation.
In the elderly, however, giant cell arteritis should be considered because
prompt therapy can slow visual loss, reduce the risk for contralateral eye
involvement, and may restore vision [7]. Although corticosteroids have
been the mainstay of treatment, a small, randomized, double-blinded trial
showed that combination oral methotrexate and oral prednisone signifi-
cantly reduces disease relapse compared with prednisone alone (Fig. 17) [8].
Pharmacotherapy and pupils

The sympathetic and parasympathetic innervation of the pupil (see
Fig. 3) makes the response to certain pharmacologic agents predictable.
156 DUONG et al
Box 1. Etiologies of Horner’s Syndrome

Central
Hypothalamus/brainstem
Ischemia
Hemorrhage
Demyelination
Cervical Cord
Trauma
Tumor
Syrinx
Arteriovenous malformation
Preganglionic
Cervicothoracic Cord
Trauma
Intramedullary paravertebral tumor
Syrinx
Cord arteriovenous malformation
Cervical spondylosis
Epidural anesthesia
Lower brachial plexus
Birth trauma
Acquired trauma
Pulmonary apex/mediastinum
Vascular anomalies of ascending aorta or subclavian artery
Apical lung tumor (Pancoast)
Mediastinal tumors
Cervical rib
Iatrogenic (eg, chest tube, cardiothoracic surgery)
Infection (eg, apical tuberculosis)
Anterior neck
Iatrogenic (eg, neck surgery, internal jugular/subclavian
catheter)
Trauma
Tumor (eg, thyroid, lymphoma)
Postganglionic
Superior cervical ganglion
Trauma
Jugular venous ectasia
Iatrogenic (eg, ganglionectomy, tonsillectomy)
Internal carotid artery
Dissection
Trauma
Thrombosis
Tumor
Migraine/cluster
Skull base/carotid canal
Trauma
Tumor (eg, nasopharyngeal carcinoma)
Cavernous sinus
Tumor (eg, meningioma, pituitary adenoma)
Inflammation
Carotid aneurysm
Carotid-cavernous fistula
Thrombosis
Data from Kawasaki A, Kardon R. Disorders of the pupil. Ophthalmol Clin North
Am 2001;14(1):158.
Pharmacologic dilation can result with sympathomimetics and with para-

sympatholytics. Mydriasis by way of sympathomimetics occurs through
alpha-adrenergic stimulation with agents, such as phenylephrine, ephedrine,
and naphazoline, found in over-the-counter and prescription eye drops. Par-
asympatholytics, mainly anticholinergic agents, such as scopolamine, tropi-
camide, cyclopentolate, and bronchodilating inhalers, also cause
pharmacologic mydriasis. These give anisocoria that is most pronounced
in bright light in the case of ophthalmic application. There may still be
Fig. 17. A slit-lamp view of a 33-year-old man who had Adie tonic pupil. The white arrows are
pointing to sectors of the sphincter that are contracting well, and the dark arrows indicate areas
in which the iris sphincter is weak. Notice that the curvature of the pupillary margin is usually
tighter in the sections that are functional and flatter in the palsied parts. (From Kardon RH,
Thompson HS. The pupil. In: Rosen ES, Eustace P, Thompson HS, et al, editors. Neuro-
ophthalmology. London: Mosby International Limited; 1998. p. 13.1–9; with permission.)
158 DUONG et al
constriction to bright light with these agents. Pharmacologic constriction

mainly occurs through cholinergic drugs, such as pilocarpine. Glaucoma
ophthalmic drops, such as brimonidine, also cause pupillary constriction.
There is more pronounced anisocoria in dim light in these instances.
Relative afferent pupillary defect

Any unilateral or asymmetric decreased afference of the optic nerve may
manifest as a relative afferent pupillary defect (RAPD). An RAPD should
not be assumed to be caused by a media disturbance (ie, cataracts) because
light flashed directly into the pupil is still scattered onto the macula [6]. One
exception may be a dense intraocular hemorrhage, which is generally not
diagnostically elusive. Optic nerve disease also presents as a deficit in visual
sensory function and possibly a change in the appearance of the optic disc.
Optic neuropathies are caused by a myriad of processes and differentiating
these causes is difficult (Table 1). The history may be the most helpful tool in
narrowing the differential.
Preservation of visual acuity or preventing further visual loss is the goal of
management. Determining which patients need emergent intervention may
require further testing and consultation with an ophthalmologist, depending
on the history and physical examination findings.
Traumatic optic neuropathy

Patients who present with an RAPD after trauma must be presumed to
have optic nerve injury. Penetrating trauma requires prompt ophthalmo-
logic evaluation and consultation for possible surgical intervention and
Table 1
Conditions producing relative afferent pupillary defect
Condition Site
Intraocular hemorrhage Anterior chamber or vitreous (dense or diffuse)
Intraocular hemorrhage Preretinal
Central serous retinopathy Retina (fovea)
Cystoid macular edema Retina (fovea)
Central or branch retinal vein occlusion Inner retina
Central or branch retinal artery occlusion Inner retina
Retinal detachment Outer retina
Anterior ischemic optic neuropathy Optic nerve head
Optic neuritisdacute Optic nerve
Optic neuritisdrecovered Optic nerve
Compressive optic neuropathy Optic nerve
Chiasmal compression Optic chiasm
Optic tract lesion Optic tract
Postgeniculate damage Visual radiations, visual cortex
Midbrain tectal damage Olivary pretectal area of pupil, midbrain
Data from Kawasaki A, Kardon RH. Disorders of the pupil. Ophthalmol Clin North Am
2001;14(1):166.
a search for foreign bodies. In addition to careful examination, these

patients should undergo CT scanning and tetanus prophylaxis, and be
placed on systemic antibiotics [9,10]. Indirect trauma, from concussive
forces, can result in shearing forces that result in mechanical and ischemic
damage to the optic nerve. This type of traumatic optic nerve injury is
much more common. The treatment of this type of injury is less well studied.
In the International Optic Nerve Trauma Study, there was no clear benefit
on visual acuity for treatment with corticosteroids, decompression surgery,
or observation alone [11]. This conclusion should be taken in the context of
the study design, however. This study was non-randomized, uncontrolled,
unblinded, and retrospective, making the conclusions weakly generalizable.
Optic neuritis
Optic neuritis refers to inflammatory optic neuropathies, but more specif-
ically to those neuropathies in which demyelination is predominant (idio-
pathic or secondary to multiple sclerosis [MS]). Optic neuritis is mainly
a clinical diagnosis. Patients generally present with monocular visual deficits
associated with periorbital or ocular pain. The pain may precede or present
concurrently with the vision loss. Eye pain is present in 92% of patients
and is almost universally worsened by eye movement [12–14]. There may
also be decreased color perception or central scotoma. The optic disc has
a normal presentation in about 70% of patients initially, because the inflam-
matory process is usually posterior to the optic disc [4,13]. Optic disc swelling
and, less commonly, flame-shaped hemorrhages at or near the disc may be
present, however. RAPD is usually seen, but may be subtle, requiring
advanced ophthalmologic evaluation. MRI imaging of the brain is helpful
diagnostically and prognostically but is not always needed emergently. In
those who have monocular optic neuritis, there is a 50% incidence of MS if
the initial MRI shows at least three typical white matter lesions. Conversely,
the 5-year incidence of MS drops to 15% if the MRI is normal [14]. Consul-
tation with an ophthalmologist is important. If the examination shows a mac-
ular star figure, this represents an infectious cause. In general, further
investigation with chest radiograph, anti-nuclear antibody, or testing for
syphilis are generally noncontributory in the emergency department setting
and not recommended for evaluation in the patient who has typical optic neu-
ritis [12,14]. Emergency physicians should be aware of more recent treatment
recommendations based on the Optic Neuritis Treat Trial data [14–16]. Oral
prednisone was associated with an increased incidence of recurrent optic neu-
ritis in a 5-year follow-up period and was no better than placebo in improve-
ment of visual outcomes. Intravenous methylprednisolone (over 3 days
followed by a prednisone taper) was associated with faster recovery in visual
function if given within 8 days of symptom onset. The difference in benefit
seems to wash away after about 6 months. There is no current convincing
data on the use of immunoglobulin or plasma exchange therapy in optic
neuritis [13].
160 DUONG et al
Oculomotor nerve palsy

Pupillary disorders may occur in the context of a third nerve lesion as
discussed in the next section.
Extraocular movement disorders

Cranial nerve palsies and binocular diplopia
There are three cranial nerves involved in controlling the six extraocular
muscles, the oculomotor nerve (III), the trochlear nerve (IV), and the abdu-
cens nerve (VI). The eyes have equal and opposed actions of the muscles of
extraocular movement, which are in turn coordinated by supranuclear con-
trol to produce conjugate and divergent gaze. These supranuclear controls
consist of brainstem gaze centers and cortical input. When there is a disrup-
tion of extraocular muscle function, the eye is unable to move in the direc-
tion of the action of the affected muscle (ie, ophthalmoplegia). Palsies of the
extraocular muscles commonly present as binocular diplopia because the
images fall on a different region of each retina. It is important to identify
associated signs and symptoms and establish in which gazes double vision
occurs to determine which cranial nerves are affected. During the examina-
tion, all six cardinal positions of gaze should be tested, not only for muscle
palsies but also to see if diplopia is elicited. The examiner may use a light
source to see if the light falls on the same spot on both corneas in each po-
sition of gaze, which determines which cranial nerves are affected. The most
common palsy is a sixth nerve palsy, followed by third nerve and fourth
nerve palsies. Involvement of multiple cranial nerves is the least common
presentation [17,18].
In this section, we divide the causes of binocular diplopia by each cranial
nerve, and then neuroanatomically for each cranial nerve. When presented
with diplopia, the emergency physician should determine which cranial
nerves are affected. This determination may help in formulating a differential
diagnosis and the urgency of such a presentation.
Cranial nerve III

The oculomotor nerve innervates the medial, superior, and inferior rectus,
the inferior oblique, the levator palpebrae, and the parasympathetic pupillary
constrictors. There is ptosis, pupillary dilation, and lateral deviation from
unopposed action of the lateral rectus in a complete CN III lesion (see
Fig. 6). The patient reports diplopia in all directions of gaze except on lateral
gaze to the affected side. This discussion focuses on lesions according to their
location: brainstem (nuclear and fascicular), subarachnoid, cavernous sinus,
orbital apex, and neuromuscular lesions. Despite the wide differential, urgent
evaluation is generally warranted for a patient presenting with new third
nerve palsy, because this may herald serious diseases, such as brainstem
infarction, aneurysm, and neoplasm. Vascular causes are the most common
known causes of oculomotor nerve palsy, whereas aneurysms are the second
most common. Usually, the cause is never determined (Table 2) [17].
Brain stem lesions. Brainstem oculomotor lesions classically present with

bilateral ptosis and contralateral superior rectus and ipsilateral oculomotor
paresis. This presentation occurs because the levator palpebrae are inner-
vated by an unpaired central nucleus and the superior recti are innervated
by contralateral subnuclei. The most common cause is ischemia [19]. The
patient should thus undergo a stroke evaluation with appropriate accompa-
nying management. Besides mass lesions, compression, and inflammation,
other causes for higher-order third nerve palsies are Wernicke encephalop-
athy and progressive supranuclear palsy. In Wernicke encephalopathy, the
third nerve ophthalmoplegia is usually bilateral and is associated with a his-
tory of chronic alcohol use or malnutrition, ataxia, nystagmus, and altered
mentation. There may be ophthalmoplegia because of involvement of CN
Table 2
Lesions of the oculomotor nerve
Anatomic location Cause Associated symptoms
Nuclear Infarction, mass, Bilateral ptosis and paresis of the
infection, inflammation, contralateral superior rectus; lid
compression function may be spared
Wernicke-Korsakoff Ataxia, abducens palsy, nystagmus,
syndrome altered mentation
Fascicular Infarction, mass, Contralateral hemiparesis or tremor; pupil
infection, inflammation, may be spared
compression
Demyelination
Subarachnoid Aneurysm Headache, stiff neck, pupil-involved
space Vasculopathic Pupil generally spared
Meningitis Headache, CN involvement,
meningismus, fever
Miller-Fisher syndrome Areflexia, ataxia, previous viral illness
Migraine Headache, family history
Uncal herniation Early pupil involvement, altered
mentation, ipsilateral hemiparesis
Cavernous sinus Neoplasm Cavernous sinus syndrome, pain, sensory
changes, sympathetic involvement
Fistula Exophthalmos, bruit, chemosis
Thrombosis Previous infection/trauma, pain,
exophthalmos, chemosis
Superior orbital Neoplasm Superior division involvement
fissure
Neuromuscular Myasthenia gravis No pupil involvement, frequent
junction fluctuation, ptosis, ophthalmoparesis,
orbicularis oculi weakness, dysarthria
Data from Bennett JL, Pelak VS. Palsies of the third, fourth, and sixth cranial nerves.
Ophthalmol Clin North Am 2001;14(1):173.
162 DUONG et al
IV and VI also. Progressive supranuclear palsy is an idiopathic degenerative

disease that mainly affects the subcortical gray matter regions of the brain in
older men. The ocular manifestations are ophthalmoplegia in all directions,
although initially more severe with voluntary vertical gaze. This disease is
also associated with dementia, decreased facial expression, pseudobulbar
palsy, and axial dystonia. These patients should have routine follow-up
with a neurologist. Although prognosis is poor, quality of life may be im-
proved with multidisciplinary symptom management [20].
Subarachnoid lesions. The subarachnoid segment of CN III is that section

after the nerve leaves the midbrain and before it enters the cavernous sinus
(Fig. 18). Lesions generally present as unilateral ptosis and third nerve
paresis, with or without pupillary dilation. A search for associated signs
and symptoms is helpful because there are many causes of lesions of this
portion of the oculomotor nerve, including meningitis, migraines, Miller-
Fisher variant of Guillian-Barre, vasculopathy, uncal herniation, and aneu-
rysm. The nerve travels near the junction of the internal carotid and posterior
communicating artery and between the posterior cerebral and the superior
cerebellar arteries, and is thus susceptible to compression from aneurysms.
In fact, about 30% of oculomotor palsies are from aneurysms, most
Fig. 18. Subarachnoid and intracavernous courses of the third, fourth, and sixth cranial nerves.
(From Porter JD, Baker RS. Anatomy and embryology of the ocular motor system. In: Miller
NR, Newman NJ, editors. Walsh and Hoyt’s clinical neuro-ophthalmology. 5th edition.
Baltimore (MD): Williams & Wilkins; 1998. p. 1066; with permission.)
commonly from the posterior communicating artery [21]. A third nerve palsy
may also result from aneurysms of the basilar, superior cerebellar, and poste-
rior cerebral arteries. The aneurysm causes oculomotor paresis by compres-
sion of the nerve. Parasympathetic fibers travel in the periphery of the
subarachnoid portion of the nerve, with the motor fibers centrally located.
Oculomotor involvement from an aneurysm commonly causes pupillary dila-
tion. Aneurysm is extremely unusual if there is not pupillary involvement in
the presence of a complete palsy [21]. CT angiography is the study of choice
for noninvasive diagnosis of unruptured aneurysms, with sensitivities ranging
from 85% to 90% for aneurysms larger than 5 mm and 79% for aneurysms
smaller than 5 mm [22]. MR angiography is an alternative, able to detect
aneurysms as small as 3 mm. It is not as sensitive, however, especially for
aneurysms smaller than 5 mm [19,21,22]. CT scan with or without lumbar
puncture should be undertaken if aneurysmal rupture with subarachnoid
bleed is suspected. Definitive treatment includes surgical clipping or endovas-
cular coil placement.
Migraine headache variants, such as cranial neuralgias and ophthalmo-
plegic migraine, present with ocular and pupillary dysfunction. This diagno-
sis usually requires the exclusion of other, more serious disease processes.
Presentations supportive of a migraine diagnosis are a previous history of
migraines, especially with childhood onset, aura, typical positive and nega-
tive visual phenomena (scotoma, transient visual distortions), and migraine-
like headache. The International Headache Society requires at least two
attacks of a migrainelike headache accompanied or followed by oculomotor
paresis within 4 hours, not attributed to other disorders, for diagnosis of
ophthalmoplegic migraine [23]. This condition is rare and usually presents
in the pediatric population [24]. The ophthalmoplegia is usually transient
but rarely may become permanent. Consultation with a neurologist is
important because acute treatment with corticosteroids has been shown to
provide relief and may prevent a permanent deficit [23,25].
Cavernous sinus lesions. Diplopia may be the presenting symptom of a cav-

ernous sinus fistula, seen in about 60% to 70% of cases [21]. These fistulas
are abnormal communications between the cavernous sinus and the carotid
artery, usually the result of trauma, but also occur as a complication of
surgery or after rupture of a cavernous carotid artery aneurysm. They typi-
cally present days to weeks after traumatic head injury with conjunctival
chemosis, proptosis, and a cranial bruit, either subjective or objective. Con-
junctival chemosis occurs from the arterialization of the conjunctival veins.
Proptosis occurs from the arterialization of the orbital veins and edema of
the orbital tissue. Pulsating exophthalmos may be visually or palpably appre-
ciated on examination, and occurs in about 95% of cases [21]. Diplopia occurs
through compression or ischemia of the ocular nerves by the fistula itself.
Most commonly CN VI is involved, although ophthalmoplegia may be attrib-
utable to any combination of ocular nerve involvement. Definitive diagnosis
164 DUONG et al
requires arteriography with selective injection of the internal and external

carotid arteries, although CT and MR imaging may be suggestive of a fistula
and can help exclude other disease processes. Visual loss may be delayed or
immediate and is the main morbidity associated with this condition, occurring
in up to 90% of cases. Other complications include intracranial or subarach-
noid hemorrhage, venous infarction, and epistaxis, although life-threatening
sequelae are rare [21]. Addressing these high-flow carotid-cavernous fistulas
usually requires urgent treatment.
Superior orbital fissure lesions. The most common cause of a third nerve
palsy from a superior orbital fissure lesion is a neoplasm. When vision is
affected, the lesion is almost always in the orbital apex [19]. Other associated
signs include conjunctival injection and chemosis, proptosis, and lid swell-
ing. CN IV, CN VI, and also CN V1 may be involved because of their prox-
imity to one another. MR imaging is the modality of choice, keeping in
mind that the most common cause is a neoplasm. CT may have a role,
but early in the growth of a tumor the neoplasm may be isodense with nor-
mal brain tissue.
Neuromuscular disease. Neuromuscular disease, such as myasthenic syn-

dromes, may mimic isolated nerve palsies. There is a separate discussion
later in this article.
Cranial nerve IV
Trochlear nerve palsy presents with paresis of the superior oblique
muscle. The function of this muscle is tested in isolation with downward
gaze with the eye adducted, which is the position of gaze that elicits the
most pronounced diplopia. The involved eye is elevated in neutral position
and the degree of elevation is increased with adduction and decreased with
abduction (see Fig. 8).
A lesion causing a fourth nerve palsy can also occur anywhere along the
path of the nerve, from the nucleus to the superior oblique muscle. Deter-
mining where the lesion lies may be difficult, requiring a search for associ-
ated, or absent, symptoms and signs along with neurologic consultation
and imaging. For instance, association with an oculomotor palsy strongly
suggests a cavernous sinus lesion. Most fourth nerve palsies have no known
cause, but of the known causes, most are attributable to head trauma.
Vasculopathies are the next most common cause (Table 3) [17,26].
Brain stem lesions. Nuclear and fascicular lesions of the fourth cranial nerve
present as a contralateral superior oblique palsy because of the crossing of
the fascicles in the brainstem. They may be caused by ischemia, trauma,
inflammation, a mass, or in the case of a fascicular lesion, MS. There are
usually other associated neurologic findings attributable to involvement of
nearby structures. The periaqueductal gray matter carries sympathetic
Table 3
Lesions of the trochlear nerve
Nuclear Infarction Internuclear ophthalmoplegia, Horner
syndrome, relative afferent pupillary defect
Trauma, tumor, As above
infection,
inflammation
Fascicular Infarction As above
Trauma, tumor, As above
infection, inflammation
Demyelination Isolated or with midbrain signs above
Subarachnoid Trauma, hydrocephalus May be bilateral
space Vasculopathic Usually isolated
Mass lesion Contralateral hemiparesis or ipsilateral ataxia
Cavernous sinus As with third nerve palsy Cavernous sinus syndrome
Herpes zoster ophthalmic Rash, trigeminal sensory loss V1 or V2
distribution
Orbit Inflammation, trauma, Oculomotor, abducens, optic nerve
tumor dysfunction, proptosis
fibers. There may thus be a preganglionic Horner syndrome. The medial

longitudinal fasciculus is just anterior to the nucleus and a lesion affecting
this causes an internuclear ophthalmoplegia. Evaluation for a nuclear troch-
lear nerve lesion should include MR imaging and a stroke workup [5].
Subarachnoid lesions. The trochlear nerve has a relatively long subarachnoid

course after it leaves the brainstem posteriorly, which makes it susceptible to
trauma, the most common cause of a fourth nerve palsy [17,26]. The trauma
may cause unilateral or bilateral isolated trochlear nerve palsy. Other causes
of fourth nerve palsies in the subarachnoid space are meningitis, aneurysm of
the superior cerebellar artery, schwannoma, and small vessel infarction
[17,19,26]. Work-up includes MR imaging to rule out a mass lesion, detect
the extent of trauma, and direct any possible neurosurgical intervention.
Distal nerve lesions. Just as with third nerve palsies, orbital fissure lesions
and neuromuscular lesions may produce a fourth nerve palsy. A nonneuro-
logic entity that may mimic isolated trochlear nerve palsy is Brown superior
oblique tendon syndrome, likely caused by a tenosynovitis or scarring of
the superior oblique tendon. The superior oblique tendon is prohibited
from free movement through the trochlear pulley. There is inhibition with
a forced duction test where the entrapped muscles resist forced movement,
indicating mechanical trapping of the superior oblique muscle. Referral to
an ophthalmologist is required because surgery may be the best option for
the patient.
166 DUONG et al
Cranial nerve VI
Of the three cranial nerves involved in extraocular movement, the abdu-
cens nerve is the most commonly affected [17]. A unilateral sixth nerve lesion
results in diplopia on lateral gaze, attributable to paresis of the lateral rectus
muscle. In neutral position, the affected eye is adducted and fails to abduct
with examination (see Fig. 10). Similar to the third and fourth cranial nerve,
lesions of the sixth cranial nerve can be subdivided anatomically with some
distinguishing features associated with each lesion. The most commonly
determined cause for a sixth nerve palsy is a neoplasm, most often metastatic
disease or a meningioma, followed by head trauma and vascular disease
(Table 4) [17].
Brainstem lesion. A nuclear sixth nerve lesion produces conjugate, horizon-

tal gaze palsy toward the side of the lesion. Nuclear lesions of the abducens
nucleus rarely present with an isolated ophthalmoplegia, however. These
lesions are associated with damage to the surrounding brainstem structures,
such as the facial nerve nucleus, the pontine lateral gaze centers, and the
reticular activating system. There may thus be facial weakness, impaired
conjugate gaze, and depressed level of consciousness, respectively. The sixth
cranial nerve has two projections, one to the ipsilateral lateral rectus and the
other to the contralateral CN III nucleus by way of the MLF (see Fig. 12). A
pontine lesion involving the adjacent MLF and the pontine lateral gaze
centers produces a one-and-a-half syndrome, seen as a complete gaze palsy
Table 4
Lesions of the abducens nerve
Nuclear Infarction Ipsilateral facial paralysis, INO
Infiltration, trauma,
inflammation
Wernicke-Korsakoff Ataxia, nystagmus, altered mentation
Fascicular Infarction, tumor, Ipsilateral facial nerve paralysis and
inflammation, MS contralateral hemiplegia
Anterior inferior cerebellar Ipsilateral facial paralysis, loss of taste,
artery infarction ipsilateral Horner, ipsilateral trigeminal
dysfunction, and ipsilateral deafness
Subarachnoid Mass Contralateral hemiparesis
Ischemia Usually isolated
Trauma Papilledema, headache
Intracranial hypertension Headache
or hypotension
Petrous apex Mastoiditis, skull fracture, Ipsilateral facial paralysis, severe facial
lateral sinus thrombosis, pain
neoplasms, tumor
Cavernous sinus As with third nerve palsy Cavernous sinus syndrome
in one direction and a unilateral (half) gaze palsy in the other direction. The
lesion to the MLF causes an internuclear ophthalmoplegia and the lesion to
the pontine lateral gaze center causes a conjugate gaze palsy to the side of
the lesion. Attempted gaze away from the lesion activates the unaffected
contralateral lateral gaze center and abducens nucleus but with paresis of
ipsilateral adduction. On gaze toward the lesion, neither eye can effectively
move laterally, because the pontine lateral gaze center cannot be activated.
The most common causes of a one-and-a-half syndrome include pontine
infarct, pontine hemorrhage, and multiple sclerosis. Wernicke encephalopa-
thy may present with a nuclear sixth nerve palsy. If a nuclear abducens nerve
lesion is suspected, the patient should undergo MR imaging and a stroke
workup, because infarction may be the cause of such a lesion.
Subarachnoid lesions. The sixth cranial nerve has a unique course in the
subarachnoid space. It emerges from the brainstem just between the pons
and the medulla and courses just lateral to the basilar artery and just poste-
rior to the clivus in the prepontine cistern before entering the cavernous
sinus. This course makes it susceptible to injury from trauma and changes
in intracranial pressure (ICP). The nerve may be compressed between the
pons and the basilar artery or the clivus. In addition to MR imaging, the
patient may require evaluation for elevated or diminished ICP.
Aneurysms of the basilar artery, and less commonly of the anterior and
posterior inferior cerebellar arteries, and the vertebral arteries can produce
a sixth nerve palsy. Sixth nerve palsy from an aneurysm is less common than
a third nerve palsy [21]. Diagnosis and management have been mentioned
previously.
Cavernous sinus lesions. Ophthalmoplegia may be the presenting symptom

of a cavernous sinus thrombosis, usually affecting all the extraocular
muscles. These occur from trauma, but more commonly from infection.
CN VI is usually affected first, however, because it lies freely within the sinus
and not within the lateral walls of the sinus as CN III and CN IV do [19,27].
Besides headache, which is present in about 90% of cases, the most common
accompanying signs are fever, ptosis, proptosis, and chemosis, occurring in
80% to 100% of infectious cases [19,27,28]. Periorbital swelling and papil-
ledema only occur in 50% to 80% of cases [27]. MRI with MR angiography
is the most sensitive noninvasive modality, approaching 100% sensitivity for
all types of sinus thromboses in small studies [28,29]. Another promising
diagnostic option is CT venography, but more study is required [27]. The
gold standard is angiography, although its diagnostic use has dramatically
declined. Therapeutically, cavernous sinus thromboses may be treated
with anticoagulation and antibiotics, in addition to supportive measures
and management of elevated intracranial pressures. There are no rigorous,
large trials on the use of anticoagulation in venous sinus thrombosis. A
recent prospective observational study showed that more than 80% of the
168 DUONG et al
624 patients received anticoagulation with a good safety profile [30]. The
outcomes were fairly encouraging in this subgroup, with only 5% rated as
severely handicapped and 8% having died. In cases of septic cavernous sinus
thrombosis, broad-spectrum antibiotics should be given. The most common
offending organism is Staphylococcus aureus, and less commonly streptococ-
cus, gram-negative bacilli, and anaerobes [27]. Surgical drainage of the sinus
thrombosis is rarely performed, although decompression of a hemorrhagic
infarction may be indicated.
Nystagmus
Nystagmus is rhythmic oscillation of the eyes. To detect nystagmus, the
eyes should be observed in primary position and each of the six positions of
gaze. Nystagmus is described by the position in which it occurs, precipitat-
ing factors, such as head position, and the direction of the fast phase. It is
useful to classify nystagmus as physiologic or pathologic, and subdivide
the pathologic causes as peripheral or central.
Physiologic nystagmus occurs normally at the extremes of gaze. In this
instance a clear image is preserved as reflexes reset the position of the
eyes and prevent the image from slipping on the retina [31]. These reflexes
are collectively known as the slow eye movement system. Their function is
to maintain clear vision with a quick corrective saccade, resulting in nystag-
mus. Pathologic nystagmus is usually accompanied by vertigo, oscillopsia,
nausea, gait disturbance, and other symptoms depending on the cause.
The patient may complain of oscillopsia, a sensation of oscillation of the
environment in the direction of the fast phase.
Peripheral nystagmus
In general, pathologic peripheral vestibular causes of nystagmus and
vertigo are less worrisome and less emergent than central causes. The nystag-
mus from vestibular causes usually has a combined horizontal and torsional
component, which can be inhibited by visual fixation onto an object. The
horizontal component does not change direction with gaze (unidirectional).
Nausea, vomiting, auditory symptoms, and vertigo are significant compo-
nents of the presentation. Vertical nystagmus may be present in addition to
horizontal nystagmus, but is far less common in peripheral causes. Vertical
nystagmus has been shown to be about 80% sensitive for central causes of
vertigo in a small prospective trial (Table 5) [32].
A common emergency department presentation of horizontal nystagmus is
benign paroxysmal positional vertigo (BPPV). BPPV presents with a com-
bined horizontal and torsional nystagmus and vertigo on provocation with
the Dix-Hallpike maneuver. The nystagmus lasts for less than 1 minute, and
fatigues on repeated testing. It has a positive predictive value of 83% and
negative predictive value of 52% for BPPV. If the induced nystagmus is
vertical, and not preceded by a latency, or does not fatigue with repeated
Table 5
Peripheral versus central nystagmus
Feature Peripheral Central
Direction of nystagmus Horizontal/torsional Pure vertical, pure torsional, or mixed
Visual fixation Inhibits nystagmus No inhibition
Severity of vertigo symptoms May be severe Usually mild
Associated eye movements None May have pursuit or saccadic defects
Other findings Hearing loss May have other CN or long tract signs
Data from Moster, ML. Nystagmus. Ophthalmol Clin North Am 2001;14(1):205.
provocation, then a central cause cannot be excluded [33]. If BPPV is diag-

nosed, the treatment of choice is canalith repositioning, such as the Epley ma-
neuver. In a meta-analysis of nine studies, canalith repositioning was 4.6 times
more likely to have symptom resolution at the time of first follow-up, com-
pared with those who did not have repositioning [34]. Although commonly
used, vestibular suppressant (anticholinergic and benzodiazepine) pharmaco-
therapy has not been well studied or shown to improve symptoms in those who
have BPPV. Its use is discouraged because it does not seem to reduce the
frequency of attacks and may even worsen a patient’s imbalance [35].
Drug intoxication from alcohol, anticonvulsants, and sedative-hypnotics
is the most common cause of nystagmus. The nystagmus is usually in the
horizontal plane, but can occasionally appear in the vertical plane. Antiep-
ileptic medications, including the newer agents, may cause a gaze-evoked
nystagmus and even diplopia. Other types of nystagmus are rare with anti-
convulsants, so other causes for the nystagmus should be sought if they are
not gaze-evoked [36]. Other peripheral causes of nystagmus and vertigo
include BPPV, Ménière disease, labyrinthitis, perilymphatic fistula, and
Ramsey-Hunt syndrome.
Central nystagmus
In contrast to peripheral causes of nystagmus, central causes (from the
brainstem or cerebellum) generally produce a purely vertical, horizontal, or
torsional nystagmus that is not inhibited by visual fixation, and the fast phase
may change direction with gaze (bidirectional). Vertical nystagmus should not
be considered as arising from a vestibular cause. Downbeat nystagmus, which
is always of central origin, is characteristic of lesions in the medullary–cervical
region, Chiari malformation, and demyelinative plaques. Upbeat nystagmus
has less localizing value [31]. Rarely are there auditory symptoms with central
nystagmus [37]. Instead, there are other neurologic symptoms, including
dysarthria, weakness, and diplopia. These differences between peripheral and
central nystagmus are only guidelines and not absolute rules. Brainstem and
cerebellar lesions causing central nystagmus include cerebellopontine angle tu-
mors, cerebellar disease, cerebrovascular disease, seizures, migraine, and MS.
Cerebellopontine angle tumors, including vestibular schwannomas, epen-
dymomas, brainstem gliomas, medulloblastomas, and neurofibromatosis
170 DUONG et al
can cause vertigo and nystagmus. The nystagmus is usually coarse and bidi-
rectional. Hearing loss of insidious onset is usually the initial symptom, and
then gait ataxia, facial pain, tinnitus, a sensation of fullness in the ear, or
facial weakness may ensue.
Cerebellar mass lesions or ischemia may cause nystagmus that is gaze-
evoked, bidirectional, downbeat, or even pendular [38]. Pendular nystagmus
is nystagmus with no fast component. Rather, the eyes move back and forth
with slow velocity; this is also seen in MS. Cerebellar lesions usually have
associated dysfunction in coordination, equilibrium, and gait.
Arnold-Chiari malformations are congenital abnormalities at the base of
the brain that cause extensions of a tongue of cerebellar tissue into the
cervical canal with displacement of the medulla into the cervical canal, along
with the inferior part of the fourth ventricle. The herniated tissue blocks the
circulation of cerebrospinal fluid in the brain and can lead to the formation
of a cavity (syrinx) within the spinal cord. Without the meningomyelocele, it
is classified as a type I malformation, and with a meningomyelocele, a type
II malformation. The nystagmus is generally downbeating. Other associated
symptoms are occipital headache (from increased intracranial pressure),
ataxia, quadriparesis, and sensory loss (from cervical syringomyelia) [37].
MS may cause various types of nystagmus [13,39]. The most common is
the nystagmus exhibited by INO, discussed in more detail later. The nystag-
mus in INO is seen during abduction of the unaffected eye, and the affected
eye is unable to adduct. Demyelinating lesions of the cerebellum may cause
a downbeat nystagmus, whereas bilateral internuclear ophthalmoplegia can
cause an upbeat nystagmus. Multiple sclerosis is the most common cause of
pendular nystagmus, in any direction [13,31].
The extraocular movement disorders of Alzheimer disease and Parkinson
disease are usually delays of saccades and impairment of smooth pursuit,
but not nystagmus [40].
Myasthenia gravis
Half of all patients who have myasthenia gravis (MG) present with pure
ocular findings. Of these, 70% eventually develop generalized myasthenia
[41]. Ptosis and diplopia are the presenting complaint in 75% of patients
and develop eventually in 90% of patients who have MG [41–43].
The clinical hallmarks of myasthenia gravis are variable muscular weak-
ness and fatigability, with amelioration after rest. Normally, there are more
than enough acetylcholine receptors to ensure a muscle action potential
after repeated ligand and receptor interactions. In MG, however, the num-
ber of available receptors quickly diminishes with each muscle contraction,
resulting in variability and fatigability. Variability is caused by the decreased
probability that acetylcholine will trigger an action potential given the
reduced number of acetylcholine receptors. Fatigability results from deple-
tion of acetylcholine after repeated action potentials.
Propensity for the muscles of extraocular movement is not well under-

stood [41]. Ptosis is a common manifestation, which may be unilateral or
asymmetric, and is exacerbated with prolonged upward gaze. Diplopia re-
sults from weakness of one or more of the extraocular muscles. There
may be strabismus evident on examination, or loss of conjugate gaze on pro-
longed eccentric gaze. There are no sensory changes, loss of muscle bulk, or
reflex changes. In general, the pupils are not affected, although there is dis-
agreement if this is universal [41,42]. On examination there may be weakness
of the orbicularis oculi muscles. The examiner may find weakness in forced
eyelid closure, whereas normally it is very difficult to retract the eyelids
against forced eye closure.
The differential may be fairly limited in more advanced disease or with
generalized myasthenia, but can be broader in the earlier stages because there
are fewer stigmata of MG. MG can mimic a single nerve palsy and other pe-
ripheral or central neuropathic diseases described previously. There are also
other neuromuscular junction diseases, although they are less common. Lam-
bert-Eaton myasthenic syndrome, like MG, is an autoimmune disease affect-
ing the neuromuscular junction. It affects the presynaptic nerve terminal,
however. With repeated stimulation, the nerve terminal eventually releases
adequate acetylcholine. This process is clinically evident with improvement
of power as a contraction is maintained, most prominently affecting the prox-
imal muscles. Lambert-Eaton rarely affects the extraocular muscles [4,41].
Treatment of Lambert-Eaton syndrome is similar to treatment of MG as
described later. Botulism may cause a myasthenic-like syndrome. The
Clostridium botulinum toxin prevents the release of acetylcholine at the neuro-
muscular junction and autonomic synapses. Weakness begins 2 to 36 hours
after toxin ingestion and fulminating weakness begins 12 to 72 hours after
ingestion with a distinctive pattern of development [4,44]. There is descending
flaccid paralysis, with virtually all cases starting with cranial nerve palsies,
including extraocular nerve palsies. Unlike MG, there are also autonomic
symptoms in botulism, such as dry mouth, ileus, postural hypotension, and
blurring of vision. There may be similar symptoms in clusters of people,
strongly indicating botulism. It is also important to consider botulism in intra-
venous drug users who have cranial nerve palsies, including diplopia. Treat-
ment includes hospitalization because respiratory compromise may develop
quickly. Elective intubation should be considered because there seems to be
a mortality benefit over those who suffer a respiratory arrest before intubation
[44]. Antitoxin should be administered, although this only binds circulating
toxins and does not reverse established paralysis. All cases are public health
emergencies and must be reported to public health officials. Organophosphate
poisoning may also present with a combination of neuromuscular and auto-
nomic symptoms and signs, classically as a cholinergic crisis. Treatment is
supportive, along with atropine or pralidoxime. Finally, medications may
produce myasthenic syndromes or exacerbate the symptoms of those who
have MG (Table 6).
172 DUONG et al
Table 6
Drugs that exacerbate myasthenia gravis
Antibiotics Aminoglycosides
Polymyxin
Clindamycin
Lincomycin
Tetracycline
Ciprofloxacin
Chloroquine
Pyrantel
Antiarrhythmics Quinidine
Procainamide
Lidocaine
Propafenone
Beta blockers
Calcium channel blockers
Anticonvulsants Phenytoin
Trimethadione
Psychiatric drugs Lithium
Chlorpromazine
Phenelzine
Other Cocaine
Steroids
Magnesium
Iodinated contrast
Data from Barton JJS, Fouladvand M. Ocular aspects of myasthenia gravis. Semin Neurol
2000;20(1):17.
Diagnosis of MG and differentiation from other neuromuscular disorders

is generally confirmed with clinical response to acetylcholinesterase inhibi-
tors, EMG, and antibody/toxin assays. Treatment of ocular MG is generally
not emergent, but timely referral or consultation with a neurologist is
important. These patients may be offered symptomatic treatment, immuno-
suppressive therapies, and thymectomy if there is a demonstrated thymoma.
Those in a myasthenic crisis (ie, weakness of respiration requiring mechan-
ical ventilation) temporarily benefit from plasmapheresis or intravenous
immunoglobulins [4,43].
Multiple sclerosis
In addition to optic neuropathy, MS may cause ocular motility dysfunc-
tion, mimicking cranial nerve lesions at any neuroanatomic level. It is clini-
cally defined by neurologic disturbances separated by time and space. In
other words, deficits involve different areas of the central nervous system at
different times. After the initial symptoms resolve in days to weeks, there
may be an interval of months to years before other neurologic deficits appear.
The most common oculomotor nerve palsy in multiple sclerosis is CN VI
palsy, which may be the initial presentation of MS [39]. After trauma and
mass lesions, it is one of the most common causes of an abducens palsy.

Trochlear nerve palsies are rare in MS because of the limited myelination
of the trochlear nerve [39].
INO is a distinctive feature of MS, caused by a demyelinating lesion of the
MLF (see Fig. 12). The MLF carries ascending projections from the CN VI
nucleus to the contralateral CN III nucleus to coordinate horizontal gaze. It is
characterized by lost or limited adduction of one eye and a horizontal nystag-
mus, during abduction, of the contralateral eye. Many cases of INO are subtle
because the deficit is partial and no diplopia with lateral gaze is reported [39].
It is present in 17% to 41% of patients who have MS [13]. Other common
causes of INO include vascular disease and infection, whereas less common
causes include trauma, tumor, hemorrhage, and vasculitis [13]. Isolated
INO without other accompanying symptoms or signs is uncommon, but
work-up should still include neurologic consultation and MRI.
Stroke syndromes and gaze palsies

Stroke syndromes and the visual system
Stroke should be considered in any evaluation of visual or ocular distur-
bance. Visual field defects are a common consequence of a large vessel cerebro-
vascular accident (CVA). The type of visual field defect depends on the
location of the lesion, which can range anywhere from the retina to the occip-
ital lobe (Fig. 19). These lesions lead to predictable patterns of visual field
deficits. The simplest and most appropriate emergency method of testing
visual fields is the confrontation technique described previously. Oculomotor
dysfunction in the setting of CVA is more indicative of brainstem and poste-
rior circulation ischemia. Ischemia of the brain has been mentioned in virtu-
ally all sections thus far, but we dedicate a section to the effects of stroke on
the visual and oculomotor system, organized by the affected artery. A full
discussion of stroke and its diagnosis and management is outside the scope
of this article.
Anterior cerebral artery

The anterior cerebral artery supplies the parasagittal cerebral cortex. A
CVA involving the anterior cerebral artery does not typically result in a visual
or oculomotor defect.
Internal carotid artery

The internal carotid artery (ICA) feeds into the circle of Willis, and
because of collateral circulation, no part of the brain is completely depen-
dent on it. In fact, 30% to 40% of occlusions are asymptomatic [37]. The
ICA gives rise to two branches of interest to the visual system: the ophthal-
mic artery and the anterior choroidal artery. A lesion of these branches may
cause monocular blindness and homonymous hemianopia, respectively. An
174 DUONG et al
Fig. 19. (A) The regional anatomy of the base of the skull, cranial nerves, and circle of Willis.
(B) The section through the brain showing the relationship between arteries of the circle of
Willis and adjacent component parts of the visual pathways. (From Walsh FB. Visual field
defects due to aneurysms at the circle of Willis. Arch Ophthalmol 1964:71:49; with permission.)
ICA lesion must be considered in these presentations. Vascular studies of

the carotid system should be performed as part of the stroke work-up to
determine if carotid endarterectomy is indicated.
The ophthalmic artery supplies the retina. Ischemia from ICA occlusion
may initially manifest as transient monocular blindness attributable to retinal
artery ischemia, whereas infarction leads to more persistent monocular blind-

ness. Because the ICA is connected to the middle and anterior cerebral
arteries by the circle of Willis, other symptoms, such as hemiplegia and hemi-
sensory deficits, may accompany the ocular blindness.
The anterior choroidal artery comes off the ICA near the posterior
communicating artery. It supplies parts of the globus pallidus, internal
capsule, and various contiguous structures, including the optic tract. In
addition to the motor and sensory deficits in an anterior choroidal artery
infarct, there is also a homonymous hemianopia contralateral to the side
of the lesion. In general, there is preservation of language and cognition.
Anterior choroidal artery strokes have not been well studied or documented
and there is debate as to whether there is a distinct syndrome [37].
Middle cerebral artery

The middle cerebral artery (MCA) supplies the lateral convexity of the
cerebral hemisphere, including the frontal eye field, the lateral geniculate
nucleus, and the optic radiations, the region of the cortex related to mac-
ular vision. Deep branches supply the basal ganglia and the internal cap-
sule. It is the most commonly involved vessel in strokes. MCA strokes are
anatomically separated into a superior division and an inferior division de-
pending on which branch is affected after the MCA bifurcates, although
a more proximal lesion can occur that includes the superior and inferior
divisions.
The superior division of the MCA does not supply the optic tract or the
radiations, but it does supply the frontal eye field and the cortical region
responsible for voluntary lateral conjugate gaze. Superior division strokes
thus do not result in a visual field defect, but may cause a conjugate gaze
deviation [4,37]. This phenomenon is discussed further in the section on
gaze palsies. Inferior division strokes may cause a homonymous quadrantano-
pia or hemianopia depending on how extensively the optic radiation is
affected.
Posterior cerebral artery

The posterior cerebral artery (PCA) supplies the occipital visual cerebral
cortex, the medial temporal lobes, thalamus, and rostral midbrain. Ischemia
in the PCA distribution is most commonly attributable to embolic PCA
infarcts that affect the visual cortex causing a homonymous hemianopia
because of unilateral ischemia of the occipital lobe. There may be macular
sparing (central visual field) because of the collateral blood supply to the
section of cortex involved in macular vision from the MCA.
Involvement of the extraocular muscles occurs through ischemia of
cranial nerve nuclei, resulting in nerve palsies, vertical gaze palsies, and
internuclear ophthalmoplegia. When deep branches of the posterior cerebral
artery in the midbrain are affected, there can be involvement of the third
cranial nerve nucleus, causing an oculomotor palsy. These arterial branches
176 DUONG et al
also supply other nearby structures and, in addition to the third nerve palsy,
can cause an accompanying contralateral hemiplegia (Weber syndrome) if
the corticospinal tracts are involved, or a contralateral ataxia and tremor
(Claude syndrome), if the red nucleus is involved [37].
Cortical blindness may result from bilateral occlusion of the PCA, which
is a rare event, and more commonly cortical blindness is the result of trauma
to the occipital lobe, or global ischemia, such as in cardiac arrest. The hall-
mark of cortical blindness or cortical visual impairment is normally func-
tioning pupils and a normal funduscopic examination with no perceptible
vision. The occipital lobe is particularly vulnerable to insult from global
ischemia because of its location in the border zone between the middle
and posterior cerebral arteries. Cortical blindness from global ischemia is
usually transient, but may be permanent [4].
Basilar artery
The basilar artery supplies deep structures of the cerebrum and the entire
brainstem and cerebellum. Occlusion of the basilar artery is usually incom-
patible with life [4]. If the proximal basilar artery is affected there may be
unilateral or bilateral abducens nerve palsy, usually with associated neuro-
logic findings, such as paresis.
‘‘Top of the basilar’’ syndrome occurs when there is an embolism small
enough to lodge at the basilar artery bifurcation into the posterior cerebral
arteries. Unilateral or bilateral third nerve palsies are characteristic in this
syndrome. The basilar artery supplies a large number of structures and is
associated with numerous other defects, however. There is generally coma
because of involvement of the reticular activating system and multiple
motor-sensory deficits.
Vertebal arteries
The vertebral arteries supply the medulla and their branches supply the
cerebellum. Occlusion of the vertebral artery and its branch, the posterior
inferior cerebellar artery, may cause lateral medullary (Wallenberg) syndrome
because of infarction of a lateral wedge of the medulla. This is the most
common ischemic lesion involving the vertebral arteries. The complete
syndrome involves the vestibular nuclei (vertigo, nystagmus, vomiting);
spinothalamic tract (impairment of pain and thermal sense over half the
body); descending sympathetic tract (ipsilateral Horner syndrome); the ninth
and tenth cranial nerves (hoarseness, dysphagia, diminished gag reflex);
otolithic nucleus (vertical diplopia and illusion of tilting of vision); olivocere-
bellar or spinocerebellar tracts (ipsilateral ataxia of limbs, falling or toppling
to the ipsilateral side, or lateropulsion); the fifth cranial nerve (pain, burning,
and impaired sensation over half of the face); and nucleus and tractus solitar-
ius (loss of taste) [37]. The most common findings are vertigo, nystagmus,
lateropulsion, and limb ataxia. This syndrome does not characteristically
involve the motor system.
Gaze palsies/conjugate gaze deviation

The decision to move one’s eyes laterally begins in the cerebral hemisphere.
Cortical fibers then travel down to the CN VI nucleus and the contralateral
CN III nucleus through the MLF as described previously for conjugate
horizontal gaze (see Fig. 12). A gaze palsy, or impairment of conjugate eye
movement, mainly results from lesions above the level of the cranial nerve
nuclei. In this case, an impairment of one hemisphere results in an imbalance
in neural tone between the two hemispheres. The result is a conjugate gaze
deviation. In this section we discuss gaze palsies and their causes by their
location in the cerebral cortex, midbrain, or pons.
Hemispheric lesions
The most common cause of gaze palsy is a hemispheric lesion. In destruc-
tive lesions of the frontal eye field (Brodmann area 8) of the cerebral cortex,
there is gaze palsy toward the side of the lesion in the area of the anterior
circulation. Hemispheric ischemia or infarction can result in a conjugate
gaze deviation. Seizure activity to the frontal eye field causes a gaze deviation
away from the affected hemisphere because of uninhibited neural stimulation.
The incidence of gaze palsy in stroke has been documented to be approx-
imately 20% to 32% and its presence has been associated with poorer func-
tional outcomes [45]. In an acute stroke or transient ischemic attack, the
eye is deviated away from the side of the hemiparesis and toward the side
of the lesion. At times this physical examination finding is subtle if the
patient’s gaze is fixated during examination. The eyes are still yoked, so there
is usually no diplopia. Visualization of the conjugate eye deviation on CT
scanning has been offered as a sign supporting acute stroke. Patients usually
close their eyes, removing gaze fixation and revealing underlying tonic eye
deviation [46,47]. This phenomenon has not been prospectively studied,
however.
Midbrain lesions
The dorsal midbrain has centers responsible for voluntary upward gaze.
This area may be damaged by trauma, hydrocephalus, compressive masses
such as pineal tumors, demyelinating disorders, infection, ischemia, or hem-
orrhage, resulting in features of Parinaud syndrome [46,48]. The syndrome is
characterized by up-gaze paresis. There is preservation of vertical eye move-
ments with the doll’s eye maneuver and with voluntary downward gaze, and
nystagmus mainly on downward gaze, eyelid retraction, midposition pupils,
and loss of accommodation. These patients require MRI for further delinea-
tion of the cause and confirmation of a midbrain lesion. Although most case
reports describe clinically stable patients [46,48], patients who are suspected
to have Parinaud syndrome should have prompt neurologic consultation
and admission given the potentially life-threatening causes and location of
the lesion.
178 DUONG et al
Pontine lesions
The paramedian pontine reticular formation, mentioned previously, is
involved in conjugate horizontal gaze. Unlike lesions of the cerebral hemi-
sphere, the gaze deviation is away from the side of the lesion and toward
the side of the hemiparesis, as in the case of unilateral ischemia to the
pons, because of the level of the lesion in relation to the decussation of
the motor pathways. Also in contrast to hemispheric or midbrain lesions,
gaze paresis from pontine lesions is more resistant to attempts to move by
doll’s eye maneuver or caloric stimulation [4]. It is rare to have isolated
gaze palsy from a pontine lesion, except in MS. Evaluation is similar to
other gaze palsies and usually requires neurologic consultation, neuroimag-
ing, and treatment of the underlying cause.
Summary
Neuro-ophthalmology is a complex, broad-ranging area of ophthalmo-
logy. Appropriate work-ups, correct diagnosis, treatment, and referral all
depend on a thorough understanding of the neuroanatomy and
neuro-ophthalmologic examination. Recognizing subtle physical findings,
such as cranial nerve abnormalities or visual field cuts, may be the key to
finding significant pathology in emergency department patients.
References
[1] Balcer LJ. Anatomic review and topographic diagnosis. Ophthalmol Clin North Am 2001;
14(1):1–21.
[2] Liu GT, Galetta SL. The neuro-ophthalmologic examination (including coma). Ophthalmol
Clin North Am 2001;14(1):23–39.
[3] Bradford CA, et al, editors. Basic ophthalmology for medical students and primary care
residents. 7th edition. American Academy of Ophthalmology. Library of Congress Catalog-
ing-in-Publication Data; 1999.
[4] Simon RP, Aminoff MJ, Greenberg DA. Clinical neurology. 4th edition. Appleton and
Lange; 1999.
[5] Kawasaki A, Kardon R. Disorders of the pupil. Ophthalmol Clin North Am 2001;14(1):
149–68.
[6] Wilhelm H. Neuro-ophthalmology of pupillary functiondpractical guidelines. J Neurol
1998;245:573–83.
[7] Balcer LJ. Evidence-based neuro-ophthalmology: advances in treatment. Curr Opin
Ophthalmol 2001;12:387–92.
[8] Jover JA, Hernandez-Garcia C, Morado IC, et al. Combined treatment of giant-cell arteritis
with methotrexate and prednisone: a randomized, double-blind, placebo-controlled trial.
Ann Intern Med 2001;134:106–14.
[9] Kaiser PK, Friedman NJ. The Massachusetts eye and ear infirmary illustrated manual of
ophthalmology. 2nd edition. Elsevier Sciences; 2004.
[10] Van Stavern GP, Newman NJ. Optic neuropathies: an overview. Ophthalmol Clin North
Am 2001;14(1):61–72.
[11] Levin LA, Beck RW, Joseph MP, et al. The treatment of traumatic optic neuropathy: the
International Optic Nerve Trauma Study. Ophthalmology 1999;106(7):1268–77.
[12] Eggenberger ER. Inflammatory optic neuropathies. Ophthalmol Clin North Am 2001;14(1):
73–82.
[13] Chen L, Gordon LK. Ocular manifestations of multiple sclerosis. Curr Opin Ophthalmol
2005;16:315–20.
[14] Optic Neuritis Study Group. The clinical profile of optic neuritis: experience of the Optic
Neuritis Treatment Trial. Arch Ophthalmol 1991;109:1673–8.
[15] Optic Neuritis Study Group. Visual function more than 10 years after optic neuritis: experi-
ence of the Optic Neuritis Treatment Trial. Am J Ophthalmol 2004;137(1):77–83.
[16] Beck RW, Cleary PA, Anderson MM, et al. A randomized, controlled trial of corticosteroids
in the treatment of acute optic neuritis. The Optic Neuritis Study Group. N Engl J Med 1992;
326(9):581–8.
[17] Richards BW, Jones FR, Younge BR. Causes and prognosis in 43,278 cases of paralysis of
the oculomotor, trochlear, and abducens cranial nerves. Am J Ophthalmol 1992;113(5):
489–96.
[18] Sobel J. Botulism. Clin Infect Dis 2005;41:1167–73.
[19] Bennett JL, Pelak VS. Palsies of the third, fourth, and sixth cranial nerves. 2001;14(1):
169–85.
[20] Warren NM, Burn DJ. Progressive supranuclear palsy. Pract Neurol 2007;7(1):16–23.
[21] Biousse V, Newman NJ. Intracranial vascular abnormalities. 2001;14(1):243–65.
[22] Vaphiades MS. Imaging the neurovisual system. Ophthalmol Clin North Am 2004;465–80.
[23] Friedman DI. The eye and headache. Ophthalmol Clin North Am 2004;357–69.
[24] Manzouri B, Sainani A, Plant GT, et al. The aetiology and management of long-lasting sixth
nerve palsy in ophthalmoplegic migraine. Cephalalgia 2007;27(3):275–8.
[25] Crevits L, Verschelde H, Casselman J. Ophthalmoplegic migraine: an unresolved problem.
Cephalalgia 2006;26(10):1255–9.
[26] Keane JR. Fourth nerve palsy: historical review and study of 215 inpatients. Neurology
1993;43(12):2439–43.
[27] John R, Ebright MD, Mitchell T, et al. Septic thrombosis of the cavernous sinuses. Arch
Intern Med 2001;161(22).
[28] Stam J. Thrombosis of the cerebral veins and sinuses. N Engl J Med 2005;352:1791.
[29] Lafitte F, Boukobza M, Guichard JP, et al. MRI and MRA for diagnosis and follow-up of
cerebral venous thrombosis (CVT). Clin Radiol 1997;52:672–9.
[30] Ferro JM, Canhao P, Stam J, et al. Prognosis of cerebral vein and dural sinus thrombosis:
results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT).
Stroke 2004;35:664–70.
[31] Moster ML. Nystagmus. Ophthalmol Clin North Am 2001;14(1):205–15.
[32] Kroenke K, Lucas CA, Rosenberg ML, et al. Causes of persistent dizziness. A prospective
study of 100 patients in ambulatory care. Ann Intern Med 1992;117(83)):163–77.
[33] Labuguen RH. Initial evaluation of vertigo. Am Fam Physician 2006;(2):244–54.
[34] Woodworth BA, Gillespie MB, Lambert PR. The canalith repositioning procedure for
benign positional vertigo: A meta-analysis. Laryngoscope 2004;114:1143–6.
[35] Koeliker P, Summers RL, Hawkins B. Benign paroxysmal positional vertigo: diagnosis
and treatment in the emergency departmentdA review of the literature and discussion of
canalith-repositioning maneuvers. Ann Emerg Med 2001;(4):392–8.
[36] Hadjikoutis S, Morgan JE, Wild JM, et al. Ocular complications of neurologic therapy.
Eur J Neurol 2005;12:499–507.
[37] Ropper AH, Brown RH. Adams and Victor’s principles of neurology. 8th edition. McGraw-
Hill; 2005.
[38] Abadi RV. Mechanisms underlying nystagmus. J R Soc Med 2002;95:231–4.
[39] Jacobs DA, Galetta SL. Multiple sclerosis and the visual system. Ophthalmol Clin North
Am 2004;17:265–73.
[40] Pelak VS, Hall DA. Neuro-ophthalmic manifestations of neurodegenerative disease. Oph-
thalmol Clin North Am 2004;17:311–20.
180 DUONG et al
[41] Barton JJS, Fouladvand M. Ocular aspects of myasthenia gravis. Semin Neurol 2000;20(1):
7–20.
[42] Elrod RD, Weinberg DA. Ocular myasthenia gravis. Ophthalmol Clin North Am 2004;17:
275–309.
[43] Drachman DB. Myasthenia gravis. N Engl J Med 1994;330(25):1797–810.
[44] Cherington M. Botulism: update and review. Semin Neurol 2004;24(2):155–63.
[45] Singer OC, Humpich MC, Laufs H, et al. Conjugate eye deviation in acute stroke: incidence,
hemispheric asymmetry, and lesion pattern. Stroke 2006;37(11):2726–32.
[46] Simon JE, Morgan SC, Pexman JH, et al. CT assessment of conjugate eye deviation in acute
stroke. Neurology 2003;60:135–7.
[47] Bhola R, Olson RJ. Dorsal midbrain syndrome with bilateral superior oblique palsy follow-
ing brainstem hemorrhage. Arch Ophthalmol 2006;124(12):1786–8.
[48] Baloh RW, Furman JM, Yee RD. Dorsal midbrain syndrome: clinical and oculographic
findings. Neurology 1985;35:54–60.
Emerg Med Clin N Am
26 (2008) 181–198
Pediatric Ophthalmology
in the Emergency Department
Kimball A. Prentiss, MD, David H. Dorfman, MD*
Boston University School of Medicine, Boston Medical Center,
Examining the young child with a visual or ocular complaint can be

a daunting challenge. Understanding the basic concepts of visual and behav-
ioral development will facilitate the examination of the child who presents to
the emergency department with eye complaints. Ocular complaints may in-
clude pain and visual impairment which may lead to anxiety and interfere
with the examination of the child. Keeping the child calm and taking the
time to engage the child in a manner he or she is comfortable with will allow
a more accurate examination.
Visual development
Vision development is a complex system that requires the development of
neuro-ocular pathways and depends on proper visual stimulation of both
eyes. The first 3 to 4 months of life are most critical for this development.
If significant disruption of a child’s vision occurs during this period and is
not quickly corrected, lifelong visual deficit is the likely result despite later
treatment. The rate of vision development remains steep until about 2 years
of life, at which time three-dimensional binocular depth perception de-
velops. It is not until 9 years of age that the brain’s development of vision
is complete.
Full-term newborns do not generally respond well to visual targets. Vi-
sual acuity at birth is approximately 20/400. In newborns, the presence of
vision may be demonstrated by pupil responses or by aversive behavior to
bright lights. Eye position at birth varies greatly. Outward deviation may
be normal eye alignment in the newborn period. After birth, the eyes tend
E-mail address: [email protected] (D.H. Dorfman).
182 PRENTISS & DORFMAN
to move to a more convergent position and should be well aligned and stable
by 4 months of age [1]. The pupils of newborns are often constricted.
Although fixation is generally present at birth in the full-term newborn,
the ability to follow targets is not developed until about 3 months of age.
Accommodation, the ability to focus, develops by 4 months [2,3]. Vision
improves dramatically during infancy. By 1 year of age, children’s vision
is 20/50 and by 2 years of age 20/20.
The eye examination in a child

Assessing vision in a child can be difficult but should be evaluated in
every child with an eye complaint. To accomplish this it is necessary to ad-
just the examination to the age and cognitive ability of the child. There are
many aspects of the eye examination, but in the emergency department, the
practitioner may focus on the skin and the surrounding tissues, light re-
sponses, fixation responses, and visual acuity. The discussion herein focuses
on those parts of the eye examination which differ in children and adults and
describes the examination appropriate for children of different ages [4].
Testing of visual acuity varies markedly depending on the age, verbal
skills, and cooperation of the child. Eye alignment is an important part of
the evaluation in children. In infants, misalignment or strabismus can lead
to severe visual deficits. Misalignment may also be associated with a range
of acute processes including orbital cellulitis.
Examination of the newborn and young infant

The first part of any eye examination is to observe the child. In children
of all ages, it is best to leave the most invasive part of the examination that
will cause the most distress to the child until the end. One should evaluate
the lids and the periorbital area for swelling, redness, drainage. One should
observe how the child moves their eyes and note the color of the conjunctiva
and sclera. The macula in young infants is not fully developed; therefore, the
eyes do not fixate well centrally and do not follow objects until about 3 to
4 months of age. To examine infants in this age group some recommend
having the parent hold the child in the feeding position and then move
the child’s head from side to side. The baby should follow this movement
with his or her eyes or head and should also blink when a light is shone
into their eyes. Another method of evaluating an infant for fixing is to cradle
him or her in one arm upright and facing the examiner while gently rocking
the infant side to side. If an infant has the eyes closed, he or she will gener-
ally open them when rocked in this manner, allowing for examination.
Of note, young infants may have intermittent downward deviation of the
eyes. This finding usually lasts only a few weeks. If this sunsetting is con-
stant or associated with poor feeding, lethargy, a large head size or bulging
fontanelle, or occurs in a child in whom it had not been present, it may be
PEDIATRIC OPHTHALMOLOGY 183
caused by increased intracranial pressure. Downward eye deviation that per-

sists pasts a few weeks may be a sign of neurologic conditions, and the child
should be evaluated by neurology and ophthalmology.
The retina is indirectly evaluated by examining the red reflex. Fundoscopic
examination of a young child who is awake and has not had his or her pupils
dilated can be extremely difficult. The red reflex allows the examiner to assess
light that enters the child’s eye and is reflected off the retina. The examiner
should dim the lights in the room and calm the baby by giving the child a pac-
ifier or bottle or by gentle side-to-side rocking. With the child’s eyes open, the
direct ophthalmoscope can be used to look at the red reflex. The key to eval-
uating the red reflex is symmetry and uniformity in the child’s eyes. In light
skinned infants, the red reflex appears orange-red. In dark skinned infants,
the reflex looks dull orange or whitish orange. This finding should not be con-
fused with leukokoria (Fig. 1), which is a whitish appearance of the pupil
that, if present, is not generally found in both eyes. Leukokoria indicates
a problem with reflection of light from the retina and may be caused by an
array of pathologic entities including cataracts and tumor.
Older infants and preverbal children

One should start with the least invasive, least painful parts of the exam-
ination in older infants and preverbal children. Some young children are
afraid of physicians because stranger anxiety is most pronounced around
10 months of age. One should begin the examination by observing the child
from a distance to determine whether there is any swelling or redness to the
eyes and surrounding tissues and how the child uses and moves their eyes.
Children in this age group usually fix on and follow a toy or other object
of interest. With some children, it may be necessary to seat the child in
the guardian’s lap and have him or her hold the child’s head still while
the examiner moves the object from side to side and up and down.
Fig. 1. Right eye leukokoria from a traumatic cataract. (From Levine LM. Pediatric ocular
trauma and shaken baby. Pediatr Clin North Am 2003;50:145; with permission).
In preverbal children visual acuity can be difficult to assess. A child who

consistently protests to having one eye covered as opposed to the other
likely has better visual acuity in the favored eye. To check for such a prefer-
ence, the examiner should cover one eye of the child and observe whether
the child fixes and follows with the uncovered eye. If the child objects to
having the eyes covered by the physician, sometimes enlisting the aid of
a guardian to hold a hand in front of the child’s eye can be helpful. Again,
the child who consistently favors viewing with a particular eye likely has bet-
ter vision on that side, and more detailed testing is indicated.
A similar technique can be used to evaluate for eye movement and strabis-
mus (Fig. 2). Using a thumb to cover one eye of the child (a patch or parent’s
hand can be used as well), the examiner holds a toy or penlight and checks that
the child fixes on the object with the uncovered eye and follows the object as it
is moved. The examiner then moves the thumb to the other eye to check for
strabismus. If the child has been focused on the light or object, in the absence
of strabismus, the newly uncovered eye should not move. Strabismus should
also be suspected if the light reflex does not fall on the center of both pupils. If
one is concerned about the presence of esotropia (eye turning in), a light or
lighted toy can be held in front of the child and then brought closer. In
some cases esotropia is only revealed with focusing on near objects.
A third test is needed to check for intermittent exotropia (eye turning
out). Intermittent exotropia often occurs only with viewing objects from
a distance. This condition is brought out by presenting the child with
a toy or object at a distance and looking at the corneal light reflex. This re-
flex can be difficult to assess; if a history of an intermittent out turning of an
eye is obtained, the child should be referred to a pediatric ophthalmologist.
In practice, certain parts of the examination may be reasonably omitted
in the emergency department setting, because a young child who has eye
Fig. 2. Fixation examination in children 4 months and older. Use of a toy will often help with
the examination. Use the thumb to cover each eye in turn to check for fixation. Move the thumb
from one eye to the other to check for strabismus (cover testing). (From Drack AV. Pediatric
ophthalmology. In: Palay DA, Krachmer JH, editors. Primary care ophthalmology. 2nd edi-
tion. Philadelphia: Mosby; 2005. p. 234; with permission).
pain or discomfort may not be cooperative with some or all of the examina-
tion. As long as the child is opening both eyes for any length of time, fixa-
tion and corneal light reflex can be assessed.
If the child has pain and the history suggests a corneal abrasion or
foreign body (and not a ruptured globe), instilling a topical anesthetic
may decrease eye pain and allow for an easier examination. The intervention
may also be diagnostic as the relief of symptoms isolates the pathology to
disruption of the conjunctiva or corneal surface.
In children with a large amount of swelling around the eye, it may be nec-
essary to retract the eyelids to perform the examination. If the history sug-
gests significant trauma and globe rupture is a possibility, it is important to
avoid putting pressure directly on the eye by placing the examiner’s thumbs
on the infraorbital and supraorbital rims and separating the lids. In some
instances, lid retractors may be needed to examine the eye. If only one re-
tractor is being used, it is most helpful to apply it to the upper lid. Cotton
swabs can also be used to open the eyes. In this method, one swab is placed
on the upper eyelid and one on the lower. The swabs are then rotated to-
ward the eyeball, the upper swab rotated down, and the lower swab rotated
up. Simultaneous with rotation, the swabs are moved toward the orbital
margins [5]. This method should not be used if trauma is suspected because
it places pressure on the eyeball.
Verbal children
The examination becomes much easier in children who can talk and allow
for objective testing of visual acuity. In young children who do not yet know
numbers or letters, the Allen card or other calibrated picture tests may be
used (Fig. 3). Before testing, one should have the child identify the objects
up close. The Tumbling E chart is also commonly used. With this test,
one should make the instructions clear to the child. Some recommend telling
the child that the E is a table, sometimes right side up, sometimes on its side,
and sometimes upside down [4]. The examiner should have the child point
the direction legs of the table are pointing. As in adults, each eye should
be tested individually; however, in children, special attention should be
paid to whether they are using the covered eye to see. Children tend to
look around the hand-held eye covers, and it may be necessary to patch
the child. Vision should be 20/50 or better at a distance in children aged
less than 5 years but 20/30 or better at near distance in all ages. In the ab-
sence of nystagmus, there should be no significant improvement in acuity
viewing with both eyes open. One should remember children have short at-
tention spans. There is no need to start on the line with the largest figures or
to have the child read every figure in a given line.
With patience, an understanding of children’s development, and a few
techniques, it is possible to perform an eye examination on children in the
emergency department. The following sections discuss an array of disease
Fig. 3. Allen chart (A) and Osterberg chart (B) used to assess vision in verbal children who do
not know the alphabet. (From Kniestedt C, Stamper RL. Assessing visual function in clinical
practice. Ophthalmol Clin North Am 2003;16:166; with permission).
processes that are particular to, or more common in, the pediatric age
group.
Conjunctivitis
Ophthalmia neonatorum (neonatal conjunctivitis)
Ophthalmia neonatorum is defined as conjunctivitis within the first
month of life. There are three main types of neonatal conjunctivitis: chem-
ical, bacterial, and viral. Although these entities may present with similar
symptoms, the timing of the development of symptoms can often be a useful
diagnostic clue. Chemical conjunctivitis secondary to perinatal ocular pro-
phylaxis generally presents within the first 24 to 48 hours of life [6]. Eryth-
romycin ointment is the agent most commonly used today and only rarely
causes chemical conjunctivitis. Silver nitrate was used in the past and has
been more frequently associated with chemical conjunctivitis. Infants with
chemical conjunctivitis typically present with bilaterally inflamed lids and
watery discharge. Gram stain reveals white cells without bacteria. Treatment
initially is supportive and involves the discontinuation of any ophthalmic
medications and observation, with an expected resolution of symptoms
within 48 hours. If no improvement is seen, a culture should be obtained
and topical antibiotic therapy initiated, with care to avoid whatever agent
was used for initial prophylactic therapy.
The epidemiology of neonatal infections is related to the transmission of
organisms at the time of delivery; therefore, pathogens found in the genital
tract and enteric system should be suspected. Chlamydia trachomatis is more
commonly acquired from the birth canal than are Neisseria gonorrhoeae and
herpes viruses (herpes simplex virus [HSV]) [7]. In addition, gram-negative
enteric organisms and several staphylococcus and streptococcus species
may also be acquired peri- and postnatally. Gonorrheal infections typically
occur 2 to 5 days after birth but can be delayed if neonatal prophylactic
therapy provides partial suppression. Chlamydial infections present slightly
later, often between 5 and 14 days of life [6].
Physical examination findings can be helpful with diagnosis, but there is
tremendous overlap of symptoms from different pathogens. Accurate diag-
nosis on the basis of physical examination alone is challenging and often re-
quires supplementary laboratory data. Gonorrheal infections are classically
characterized by a hyperacute mucopurulent discharge with lid edema, bul-
bar conjunctivitis, and chemosis. Chlamydial infections can also present
with copious discharge but more commonly are characterized by palpebral
conjunctival injection and inflammation with less associated lid edema and
thick discharge [6,8]. A statim Gram stain and culture, including chocolate
agar, should be obtained to aid in the diagnosis but should not delay the ini-
tiation of therapy when a high clinical suspicion for disease is present. In ad-
dition to Gram stain and culture, Giemsa stain, direct fluorescent antibody,
ELISA, and polymerase chain reaction can be used to diagnose chlamydial
infections, and laboratory investigation should be guided based on method
availability [9]. Intracellular gram-negative diplococci are consistent with
gonorrheal infection and constitute an ocular emergency because this organ-
ism can penetrate through and ulcerate the cornea, rapidly causing blindness
[8]. An ophthalmology consult should be obtained immediately without de-
lay in therapy. Current recommendations for treatment are a single dose of
intravenous or intramuscular ceftriaxone with admission and hourly saline
eye lavage. The infant should simultaneously be covered for chlamydial dis-
ease until cultures are negative using oral erythromycin therapy to treat oph-
thalmic disease and prevent the late onset of chlamydial pneumonitis [7,8].
Staphylococcus aureus, Streptococcus epidermis, Haemophilus influenzae,
Escherichia coli, and Pseudomonas are other causes of neonatal conjunctivi-
tis and typically present from 5 to 7 days of life. Clinical findings are often
indistinguishable from that of other pathogens. Diagnosis is by Gram stain
and culture, and polymyxin/bacitracin/neomycin topical ointment is gener-
ally accepted as standard treatment. Diagnosis of typeable Haemophilus
influenzae conjunctivitis is an exception and should be treated with systemic

antibiotics, with consideration given to a full septic evaluation before paren-
teral antibiotic administration.
Neonatal conjunctivitis caused by HSV, typically, although not exclu-
sively, HSV-2, may also be acquired through the birth canal, and ocular
manifestations may be the only presenting symptoms of neonatal herpetic
infections [6]. Clinical suspicion should be elevated with a maternal history
of infection, vesicular blepharitis, or the presence of ocular dendritic ulcers
with fluorescein staining. Diagnosis is made by immunofluorescence, smear,
or culture. Treatment involves both topical and systemic parenteral acyclo-
vir and the avoidance of steroids. Full septic evaluation should be per-
formed in the neonate with HSV infection [10].
Childhood conjunctivitis
Acute conjunctivitis is the most common eye disorder in young children
and is the most frequent ophthalmologic complaint seen in the pediatric
emergency department [11]. To date, there are no evidence-based guidelines
for the diagnosis and empirical treatment of conjunctivitis [12]. Bacterial
infections are predominant and are chiefly caused by one of three pathogensd
non-typeable Haemophilus influenzae, Streptococcus pneumoniae, and Staph-
ylococcus aureus [7,11]. The clinical course of bacterial conjunctivitis
generally has an abrupt uniocular onset, with spread to the opposite eye
within 48 hours [13]. Tearing and irritation are the initial symptoms, followed
by mucopurulent discharge, typically with a history of crusting or gluing of
the eyelashes. Diffuse erythema of the bulbar and palpebral conjunctivae is
generally present, whereas preauricular lymphadenopathy is not [14].
Laboratory studies to determine the causative organism are usually re-
served for severe cases and those unresponsive to initial treatment. Em-
piric treatment is commonplace, particularly when a history of sticky
eyelids is obtained in conjunction with a physical finding of purulent dis-
charge [12]. Treatment typically involves erythromycin ointment, bacitra-
cin-polymyxin B ointment, or topical fluoroquinolones [7]. Several
clinical associations can also help guide diagnosis and subsequent treat-
ment. Conjunctivitis-otitis syndrome is common, occurring about 25%
of the time, and is most often associated with non-typeable Haemophilus
influenzae infections [11,15]. In this scenario, monotherapy with systemic
antibiotics is indicated, and a topical agent is not needed [16,17]. Several
studies suggest that if Haemophilus influenzae is recovered from a culture,
or if the patient has a history of recurrent otitis media, systemic treatment
should be initiated even in the absence of acute otitis media in the hope of
preventing its development [17].
Another common cause of pediatric conjunctivitis is viral illness. The
overall frequency of pediatric viral illness is extremely high, but the presence
of conjunctivitis in systemic pediatric viral disease varies. The most common
type of viral conjunctivitis in children is adenoviral conjunctivitis, which can

present as an isolated condition or as part of a viral syndrome [7]. Adeno-
virus can cause a nonspecific acute conjunctivitis characterized by red pro-
fusely watery eyes, or more severely, epidemic keratoconjunctivitis if corneal
involvement is present. Pharyngoconjunctival fever caused by adenovirus is
common in children and presents with the triad of pharyngitis, fever, and
conjunctivitis, as the name implies. The typical course lasts 2 weeks and of-
ten begins with unilateral involvement, becoming bilateral within several
days, with preauricular lymphadenopathy [18]. Although the typical course
of pharyngoconjunctival fever is self-limited with an excellent prognosis, the
same adenovirus types can also cause the rarer but more serious dissemi-
nated adenoviral disease which results in multisystem organ failure and
death [18,19]. Upper respiratory tract infections caused by rhinovirus, en-
terovirus, and influenza virus are accompanied by a self-limited conjunctivi-
tis less than 50% of the time, and less than one third of respiratory syncytial
virus infections are accompanied by conjunctivitis. Conjunctivitis is also
commonly associated with measles, although this pathogen is now rare in
the United States [19].
The diagnosis of adenoviral conjunctivitis remains primarily clinical.
Conjunctival hemorrhage can occur with adenoviral infection, as can punc-
tate corneal epithelial defects; therefore, the slit lamp examination is an im-
portant part of the diagnostic evaluation, although it is often difficult to
perform on a young patient. An ideal laboratory study does not yet exist.
Viral cultures are epidemiologically useful, but delayed results have little
use in the emergency department setting. Enzyme immunoassay and poly-
merase chain reaction tests are rapid, but the sensitivity varies considerably.
Treatment options are also limited and are largely supportive because there
is no proven effective treatment for adenoviral conjunctivitis [20,21]; how-
ever, topical antibiotics are often prescribed to prevent bacterial superinfec-
tion. Corticosteroids should be avoided in treating most cases of pediatric
adenoviral conjunctivitis and should only be administered under the care
of an ophthalmologist. In fact, the prescription of ophthalmic steroids in
general in the emergency department should be limited, because steroids
can be devastating in the presence of herpetic infections, which must always
be considered and effectively ruled out.
Herpetic ocular infections outside of the neonatal period are typically
from HSV-1 [6]. Herpetic keratitis with its classic dendritic pattern with
fluorescein staining may be present, is most often unilateral, and is some-
times associated with vesicles in the distribution of the ophthalmic branch
of the trigeminal nerve, involving the forehead, periorbital area, and tip
of the nose [22]. More commonly, the clinical presentation of HSV conjunc-
tivitis is nonspecific, although always painful, and very similar to other eti-
ologies of conjunctivitis previously discussed. Treatment of HSV ocular
infection, most often with a topical antiviral agent, should involve an
ophthalmologist.
Orbital and periorbital cellulitis

Orbital and ocular adnexal infections are more common in children than
adults and must be accurately distinguished from periorbital infections, be-
cause the pathogenesis, treatment, and potential severity of sequelae vary
considerably. Knowledge of the region’s anatomy helps one to clinically dis-
tinguish orbital from periorbital infections and aids in understanding the
pathophysiology and the potential for spread of infection of each of these
two entities. Orbital infections are defined by their location relative to the
orbital septum, which is a thin membrane that extends from the periosteum
and reflects into the upper and lower eyelids [23]. The septum separates the
periorbital soft tissues (preseptal region) from the orbital space (septal) and
provides a barrier to the spread of infection between the two regions. Pre-
septal processes do not directly progress into the septal space, nor do septal
infections directly spread into the preseptal space [23,24]; however, infection
can also travel through the valveless venous drainage system of the midfacial
region involving the eye cavity and the ethmoid and maxillary sinuses,
thereby allowing for the indirect spread of infection in an anterograde
and retrograde fashion [25].
Another important anatomic consideration is the relationship between
the sinus cavities and the orbit. The eye is surrounded by paranasal sinuses
on three of its four walls. The floor of the frontal sinus is the roof of the or-
bit, and the roof of the maxillary sinus is the floor of the orbit. The medial
border of the eye is formed primarily from the extremely thin lamina papy-
racea of the ethmoid bone. Infection can spread from the paranasal sinuses
to the bone, forming osteitis or subperiosteal abscesses, and into the orbital
space, producing an orbital abscess or orbital cellulitis (Fig. 4) [24,26]. These
anatomic considerations help to explain the typical pathogens found in or-
bital cellulitis. The periorbital area is protected from the paranasal sinuses
by the orbital septum; therefore, it is far less susceptible to infection by sinus
pathogens. Infections in the periorbital area are usually secondary to skin
pathogens and are often associated with soft tissue injuries such as insect
Fig. 4. Orbital cellulitis. (From Greenberg MF, Pollard ZF. The red eye in childhood. Pediatr
Clin North Am 2003;50:106; with permission).
bites or the spread of local infection (impetigo, hordeolum, chalazion, da-

cryocystitis) [27].
Periorbital cellulitis is much more common than orbital cellulitis [25]. It
presents clinically with erythema, induration, tenderness, or warmth of the
periorbital tissues. Signs of systemic illness are often absent, although fever
may be present, particularly if bacteremia served as the origin of the cellu-
litis. Extraocular motion is not affected and should be full. In fact, decreased
movement of the eye is one of the cardinal features of orbital cellulitis, along
with proptosis, decreased visual acuity, chemosis, and papilledema [8,23].
Orbital cellulitis is also associated with erythema, pain, and swollen eyelids,
but the eyelid swelling of orbital cellulitis can be differentiated from that of
periorbital cellulitis in that it will not extend beyond the superior orbital rim
onto the brow [27]. This limitation of upper eyelid swelling is due to the ex-
tension of the orbital septum onto the periosteum of the inferior margin of
the superior orbital rim, which effectively provides a structural barrier lim-
iting the degree of upper eyelid swelling in orbital cellulitis.
Distinguishing between these two clinical entities is paramount. If one is
unable to do so clinically, a CT scan should be obtained, as should ophthal-
mology consultation [25]. If CT scanning demonstrates sinus disease as
a likely etiology of orbital cellulitis, otorhinolaryngology should also be
consulted because surgical drainage may be necessary. Any child with or-
bital cellulitis must be admitted for parenteral antibiotics and close observa-
tion among a multidisciplinary team, with or without surgical intervention.
The antibiotic choice should be aimed at the most likely pathogens, typi-
cally, respiratory pathogens and anaerobes originating from the paranasal
sinuses. Ampicillin/sulbactam or cefuroxime with clindamycin or metroni-
dazole are reasonable choices because they appropriately target the most
common organisms, such as Streptococcus pneumoniae, non-typeable Hae-
mophilus influenzae, group A streptococcus, Staphylococcus aureus, and
anaerobic organisms [23].
For children with periorbital cellulitis, skin trauma is the most likely eti-
ology. Antibiotics targeted at gram-positive organisms should be adminis-
tered, because staphylococcus and streptococcus species are the most
likely cause of post-traumatic periorbital cellulitis [23]. These patients
should be followed up closely for any progression of symptoms. Primary
bacteremia is another etiology of periorbital cellulitis but is rare due to ef-
fective vaccination against Streptococcus pneumoniae and Haemophilus influ-
enzae [27]. If bacteremia is suspected as a source for infection, particularly in
a child aged less than 3 months or in an unvaccinated or newly immigrated
patient, Streptococcus pneumoniae and Haemophilus influenzae should be
suspected. Any child aged less than 2 years or who has signs of systemic
illness should be admitted for parenteral antibiotics and close observation.
A full septic evaluation, including lumbar puncture, should be strongly
considered before antibiotic administration in any toxic appearing child,
or in the presence of any signs or symptoms suggestive of meningitis.
Another special consideration involves immunocompromised children,

including children with diabetes. These children should immediately be re-
ferred to an ophthalmologist for evaluation because mucormycosis presents
with eyelid erythema and is a diagnosis that generally requires surgical de-
bridement [25].
Lacrimal system infections

Infections of the lacrimal system are named according to the location of
infection. Infection of the nasal lacrimal duct, located between the medial
canthus of the eye and the nasal bridge, is known as dacryocystitis and
can occur in the setting of acute or chronic obstruction of the duct
(Fig. 5). Often, a history of watery or even mucopurulent discharge from
the eyes can be elicited, followed by the development of erythema, swelling,
and tenderness over the lacrimal sac. The major complication of dacryocys-
titis is periorbital cellulitis [26] and, less commonly, orbital cellulitis [27] or
cavernous sinus thrombosis [23]; meningitis, brain abscesses, and sepsis can
also occur, although rarely. Diagnosis must be prompt, and treatment
should include oral antibiotics. The most common pathogens in children
with acute dacryocystitis are Staphylococcus epidermidis and Staphylococcus
aureus [23]. Any child with dacryocystitis who appears ill or toxic should be
admitted for parenteral antibiotic therapy [27].
Another infection of the lacrimal system is dacryoadenitis. This infection
of the lacrimal gland is located in the supratemporal orbit. The gland is
composed of two lobes. The palpebral lobe is easily visualized with eversion
of the superior lid, but the orbital lobe cannot be directly visualized on
physical examination. Dacryoadenitis may present as an acute or chronic
problem. Acute disease is characterized by the abrupt onset of pain,
Fig. 5. Acute dacryocystitis. Maximal swelling nasally below the medial canthal ligament.
(From Greenberg MF, Pollard ZF. The red eye in childhood. Pediatr Clin North Am
2003;50:108; with permission).
swelling, and erythema of the supraorbital region, often associated with

chemosis, conjunctivitis, and mucopurulent discharge, and sometimes asso-
ciated with limited ocular mobility, proptosis, fever, and malaise. More
chronic infections typically present with swelling of the superior lid. Mild
ptosis may be present secondary to the swelling, but pain, erythema, and
fever are not present.
The treatment of dacryoadenitis is dependent on the acuity of the presen-
tation and the most likely etiology. Imaging is often not necessary, although
CT scanning can help to make the distinction between orbital cellulitis and
dacryoadenitis if the orbital lobe of the gland is involved and clinical distinc-
tion is difficult. Acute dacryoadenitis is most commonly associated with vi-
ral infections, and treatment is supportive. Bacterial pathogens should be
suspected if the discharge is mucopurulent. Cultures should be obtained
while initiating treatment to cover the most common pathogens until culture
data become available. A first-generation cephalosporin is generally recom-
mended; however, an increasing prevalence of ocular methicillin-resistant
Staphylococcus aureus (MRSA) has recently been reported. Choosing an
oral antimicrobial agent to best fit the MRSA susceptibility profile within
your institution is prudent [28,29].
Congenital
Nasal lacrimal duct obstruction
The most common congenital ophthalmologic finding in newborns is na-
sal lacrimal duct obstruction. Tears are produced in the lacrimal gland
which rests within the temporal portion of the superior lid. They then circu-
late over the eye toward the punctum located in the nasal corner of the eye
where the two lid margins unite. Typically, tears drain through the punctum
and canalicular system into the nasolacrimal sac and then into the duct
which drains intranasally through the valve of Hasner. When the drainage
path is obstructed, most commonly at the level of the valve of Hasner, pa-
tients present with watery discharge from the eye, often bilaterally [21]. On
further inspection, the tear lake in the inferior portion of the lid is often el-
evated. If bacterial superinfection exists, a chronic mucopurulent discharge
is present, with parents commonly reporting lid adherence. If this adherence
persists, symptoms may progress to include conjunctival injection with
thickening of the periorbital skin. The examination of any newborn with
these complaints should involve carefully applied pressure with a cotton
tip to the region of the nasolacrimal sac. If nasal lacrimal duct obstruction
is present, reflux of mucopurulent material from the punctum may occur.
Careful examination of the skin that overlies the drainage system is also im-
portant, because identification of a bluish hued palpable mass is indicative
of a mucocele, specifically, a cyst of the nasal lacrimal duct also known as
a dacryocele [21]. Simple nasal lacrimal duct obstruction should not be
associated with any photophobia, ocular cloudiness, or abnormal appear-

ance of the red reflex. If any of these findings are present, the diagnosis of
nasal lacrimal duct obstruction should be questioned, and congenital glau-
coma or cataracts should be considered.
Treatment of nasal lacrimal duct obstruction in the newborn is simply
supportive if no superinfection or dacryocele is suspected. Parents should
be instructed to apply gentle massage over the nasal lacrimal duct with rapid
downward motions three to four times daily to facilitate opening of the
valve of Hasner. After the age of 6 months, the patient should be referred
to an ophthalmologist, because obstructions rarely resolve on their own be-
yond the first several months of life and often require surgical probing [21].
If suspicion of a dacryocele exists, the patient should rapidly be referred to
a pediatric ophthalmologist and otolaryngologist because obstructive intra-
nasal cysts are often associated and require rapid intervention. The presence
of mucopurulent discharge warrants the administration of topical antibi-
otics for 1 to 2 weeks in conjunction with daily massage as described previ-
ously. If this regimen does not clear the discharge, the patient should be
referred to an ophthalmologist for further evaluation, independent of age.
Continued infection in the lacrimal sac is associated with preseptal cellulitis,
a more serious condition that often requires hospitalization in this age
group.
Congenital cataracts
A cataract is an opacity of the lens of the eye requiring prompt diagnosis
and treatment to prevent partial or complete blindness. Congenital cataracts
can be present at birth and associated with certain congenital infections such
as rubella, toxoplasmosis, HSV, or cytomegalovirus [30]. They can also de-
velop in the first several months of life secondary to several metabolic con-
ditions, such as galactosemia or peroxisomal disorders, or in genetic
conditions such as trisomy 21 or Turner syndrome [21].
The clinical presentation of infants with cataracts is dependent on the
density of the opacification and the presence in one or both eyes. Leukoko-
ria is caused when the cataract is dense enough to prevent a significant
amount of light from penetrating through the cornea to the retina (see
Fig. 1). The red reflex is abnormal and may even be absent if the cataract
is severe. Nystagmus or strabismus may also be noted if the cataract de-
velops within the first several months of life. Vision may be mildly to se-
verely decreased. In severe cases in which vision is absent, the infant may
not even spontaneously open his or her eyes. In moderate cases, the infant
may be noted to squint in bright sunlight in an effort to reduce the glare
resulting from the reduced ability of the pupil to constrict [21].
Treatment of congenital cataracts should be initiated emergently through
a pediatric ophthalmologist, because the first several months of life are crit-
ical to the development of the visual axis.
Congenital glaucoma
Pediatric glaucoma is divided into primary and secondary types depending
on the presence of isolated angle malformations (primary) versus other under-
lying ocular abnormalities (secondary) [30]. Both types may be present at birth
(congenital) or develop at any age (infantile or juvenile). The common finding
with any form of glaucoma is increased intraocular pressure, which, if left
undiagnosed and untreated, can lead to optic nerve damage and vision loss.
Additional damage, such as large refractive errors, astigmatisms, strabismus,
and amblyopia, may occur as a result of congenital or infantile glaucoma, be-
cause the visual system is undergoing crucial stages of development during in-
fancy, and any disruption to the visual axis may have multiple sequelae [30].
Forty percent of cases are present at birth and 85% by age 1 year;
however, the age of diagnosis varies from birth to late childhood. The
most common finding in patients who have congenital glaucoma is excessive
tearing, also known as epiphora, as well as photophobia and some degree of
blepharospasm [30]. Corneal enlargement or asymmetry (when disease is
unilateral) is often present, and a corneal diameter of greater than 12 mm
in an infant younger than 1 year of age should prompt urgent referral to
a pediatric ophthalmologist [30]. Other findings include corneal clouding,
conjunctival injection, corneal edema, ocular enlargement, and ocular nerve
cupping observed on fundoscopic examination [21].
Treatment of glaucoma in infants and children is almost always primarily
surgical, complemented by medical therapy with topical or oral pressure-
lowering agents. Prognosis is generally better the later the onset of symp-
toms, because the structural anomaly is typically less severe [30].
Misalignment
Ocular misalignment, generally referred to as strabismus, is not uncom-
mon in newborns and young children and may be of enough concern to
the parents to prompt an emergency room visit. It is important to distin-
guish normal misalignment from more worrisome clinical presentations.
Newborns commonly have an ocular instability that is characterized by vari-
able, intermittent ocular misalignment throughout their first several months
of life. This misalignment is most commonly secondary to immaturity of the
extraocular muscles and self-resolves by 3 to 4 months of life [21]. If the de-
viation is constant, or if it is bilateral, the patient should be referred to a pe-
diatric ophthalmologist for further investigation, because these patterns
may be more consistent with significant pathology such as primary neuro-
logic or oncologic processes.
Patients with congenital strabismus typically have normal eye movements
for the first several months of life and then develop the tendency for one or
both eyes to deviate [21]. If this deviation is present without interruption of
the visual axis, it is referred to as a ‘‘manifest strabismus.’’ More specifically,
it is termed esotropia if there is inward deviation of the eye or exotropia if the

deviation is outward. The examination techniques for the evaluation of stra-
bismus described earlier in this article, specifically the ‘‘cover uncover test,’’
may elicit a latent strabismus also known as a ‘‘phoria’’ that is only present
when fixation is interrupted by covering one eye [31]. Children who have ei-
ther manifest or latent strabismus should be evaluated by an ophthalmolo-
gist because these conditions can lead to amblyopia, although much less
commonly with phorias than tropias [31].
The emergency room physician should always rule out a sixth nerve palsy
that could mimic congenital esotropia, particularly if accompanied by other
signs of increased intracranial pressure such as nausea, vomiting, lethargy,
and sunsetting of the eyes [8]. Similarly, third nerve palsies should be con-
sidered when evaluating a child with an exotropia [3,16]. In general, emer-
gent presentations of cranial nerve palsies or mechanical restriction due to
orbital fractures, cellulitis, masses, or other intracranial processes can effec-
tively be ruled out by full extraocular muscle movements [8].
Oncology
Retinoblastoma is the most common primary intraocular malignancy of
childhood and frequently presents with leukokoria, often detected by a parent
who may seek medical evaluation in the emergency department. The white
pupil is actually the tumor itself visualized through the pupil and vitreous
[21]. The tumor may be unilateral, typically associated with a spontaneous
mutation, or bilateral, almost always heritable. These children may also pres-
ent with a unilateral fixed and dilated pupil, visual changes, a red and painful
eye, proptosis, or different colored irises, also known as heterochromia iridis
[21]. Any child with a white pupil or any other findings suspicious for retino-
blastoma should be immediately referred to an ophthalmologist for a com-
plete ocular examination, typically performed under anesthesia.
Other tumors that may present as orbital masses with proptosis include
rhabdomyosarcoma, Langerhan’s cell histiocytosis, acute myeloid leukemia,
metastatic Ewing’s sarcoma, Burkitt’s lymphoma, or neuroblastoma [26].
Neuroblastoma can also present with the rare ocular finding of opsoclo-
nus/myoclonus. This condition is often referred to as ‘‘dancing eyes,’’ de-
scribing the simultaneous presence of rapid irregular eye movements and
involuntary twitching of the eyelids, and is believed to be secondary to an
autoimmune reaction. When present, opsoclonus/myoclonus should prompt
an immediate evaluation for neuroblastoma, because this is the most com-
monly associated pediatric tumor.
References
[1] Weinacht S, Kind C, Mounting JS, et al. Visual development in preterm and full-term in-
fants: a prospective masked study. Invest Ophthalmol Vis Sci 1999;40(2):346–53.
[2] Curry DC, Manny RE. The development of accommodation. Vision Res 1997;37(11):
1525–33.
[3] Hainline L, Riddell P, Grose-Fifer J, et al. Development of accommodation and convergence
in infancy. Behav Brain Res 1992;49(1):33–50.
[4] Levin AV. Eye emergencies: acute management in the pediatric ambulatory setting. Pediatr
Emerg Care 1991;7(6):367–77.
[5] Drack AV. Pediatric ophthalmology. In: Palay DA, Krachmer JH, editors. Primary care
ophthalmology. 2nd edition. Philadelphia: Elsevier Mosby; 2005. p. 229–73.
[6] Erogul M, Shah B. Ophthalmology. In: Shah B, editor. Atlas of pediatric emergency medi-
cine. New York: McGraw-Hill; 2006. p. 361–84.
[7] Morrow G, Abbott R. Conjunctivitis. Am Fam Physician 1998;57(4):735–48.
[8] Levin A. Ophthalmic emergencies. In: Fleisher G, Ludwig S, Henretig F, editors. Textbook
of pediatric emergency medicine. Philadelphia: Lippincott Williams and Wilkins; 2006.
p. 1653–62.
[9] American Academy of Pediatrics. Chlamydia trachomatis. In: Pickering LK, Baker CJ,
Long SS, et al, editors. Red book: 2006 report of the committee on infectious diseases.
27th edition. Elk Grove Village (IL): American Academy of Pediatrics; 2006. p. 254–5.
[10] American Academy of Pediatrics. Herpes simplex. In: Pickering LK, Baker CJ, Long SS,
et al, editors. Red book: 2006 report of the committee on infectious diseases. 27th edition.
Elk Grove Village (IL): American Academy of Pediatrics; 2006. p. 364–5.
[11] Buznach N, Dagan R, Greenburg D. Clinical and bacterial characteristics of acute bacterial
conjunctivitis in children in the antibiotic resistance era. Pediatr Infect Dis J 2005;24(9):
823–8.
[12] Patel P, Diaz M, Bennett J, et al. Clinical features of bacterial conjunctivitis in children. Acad
Emerg Med 2007;14(1):1–5.
[13] Leibowitz H. The red eye. N Engl J Med 2000;343(5):345–51.
[14] Datner E, Tilman B. Pediatric ophthalmology. Emerg Med Clin North Am 1995;13(3):
669–79.
[15] Bingen E, Cohen R, Jourenkova N, et al. Epidemiologic study of conjunctivitis-otitis syn-
drome. Pediatr Infect Dis J 2005;24(8):731–2.
[16] Fischer P, Miles V, Stampfi D, et al. Route of antibiotic administration for conjunctivitis.
Pediatr Infect Dis J 2002;21(10):989–90.
[17] Wald E. Conjunctivitis in infants and children. Pediatr Infect Dis J 1997;16(2):S17–20.
[18] Scott I. Pharyngoconjunctival fever. eMedicine. Available at: http://www.emedicine.com/
oph/topic501.htm. Updated February 27, 2007. Accessed February 27, 2007.
[19] R. Hered. Pediatric viral conjunctivitis. Northeast Florida Medical Journal 2002. Available
at: http://www.dcmsonline.org/jaz-medicine/2002journals/augsept2002/conjunctivitis.htm.
Accessed July 25, 2007.
[20] American Academy of Pediatrics. Adenovirus infections. In: Pickering LK, Baker CJ,
Long SS, et al, editors. Red book: 2006 report of the committee on infectious diseases.
27th edition. Elk Grove Village (IL): American Academy of Pediatrics; 2006. p. 202–4.
[21] Drack AV. Pediatric ophthalmology. In: Palay D, Krachmer J, editors. Primary care
ophthalmology. 2nd edition. New York: Elsevier/Mosby; 2005. p. 238–64.
[22] Baskin M. Ophthalmic and otolaryngologic emergencies. In: Fleisher G, Ludwig S, Baskin
M, editors. Atlas of pediatric emergency medicine. Philadelphia: Lippincott Williams and
Wilkins; 2004. p. 267–72.
[23] Wald E. Periorbital and orbital infections. Pediatr Rev 2004;25(9):312–9.
[24] Givner L. Periorbital versus orbital cellulitis. Pediatr Infect Dis J 2002;21(12):1157–8.
[25] Jain A, Rubin P. Orbital cellulitis in children. Int Ophthalmol Clin 2001;41:71–86.
[26] Greenburg M, Pollard Z. The red eye in childhood. Pediatr Clin North Am 2003;50:
105–24.
[27] Nield L, Kamat D. A 9-year-old girl who has fever, headache, and right eye pain. Pediatr Rev
2005;26(9):337–40.
[28] Asbell P, Sahm DF, Draghi DC, et al. Increasing prevalence of ocular methicillin-resistant
Staphylococcus aureus [poster 62]. In: Programs and abstracts of the 2006 joint meeting
of the American Academy of Ophthalmology and Asia Pacific Academy of Ophthalmology.
Las Vegas (NV), 2006. Available at: http//:www.osnsupersite.com/view.asp?rID¼19307.
Accessed August 28, 2007.
[29] Johnson K. Overview of TORCH infections. UpToDate.com. Available at: http://www.
utdol.com/utd/content/topic.do?topicKey¼pedi_id/25219. Updated April 2007. Accessed
July 16, 2007.
[30] Olitsky S, Reynolds J. Overview of glaucoma in infants and children. UpToDate.com. Avail-
able at: http://www.utdol.com/utd/content/topic.do?topicKey¼pedi_opth/8856. Updated
December 2006. Accessed April 3, 2007.
[31] Coats D, Paysse E. Evaluation and management of strabismus in children. UpToDate.
com. Available at: http://www.utdol.com/utd/content/topic.do?topicKey¼pedi_opth/7374.
Updated December 2006. Accessed April 3, 2007.
Emerg Med Clin N Am
26 (2008) 199–216
The Painful Eye

James M. Dargin, MDa, Robert A. Lowenstein, MDa,b,*
a
Department of Emergency Medicine, Boston University School of Medicine,
Boston Medical Center, 1 Boston Medical Center Place, Boston, MA 02118, USA
b
Department of Emergency Medicine, Quincy Medical Center, 114 Whitwell Street,
Quincy, MA 02169, USA
Acute angle closure glaucoma

In 90% of cases, acute angle closure glaucoma (AACG) results from pu-
pillary block in which pupillary dilation causes apposition of the lens and
the iris, resulting in obstruction of aqueous outflow from the eye [1]. Accu-
mulation of aqueous in the posterior chamber leads to a rapid elevation in
intraocular pressure beyond the normal range of 10 to 21 mmHg, which
causes pain and loss of vision [2]. Pupillary block often occurs in hyperopic
(farsighted) eyes with a shallow anterior chamber angle [1]. AACG may be
triggered by exposure to dim ambient lighting, topical mydriatics, anticho-
linergics, tricyclic antidepressants, selective serotonin reuptake inhibitors, or
adrenergic agonists as a result of their dilating effect on the pupil [3]. There
are case reports of intranasal phenylepherine [4], topiramate [5], and nebu-
lized albuterol causing AACG [6]. Other etiologies of AACG may include
peripheral anterior synechiae formed after uveits and previous ocular sur-
gery. AACG is more common in Eskimo and Asian populations, women,
patients with hyperopia, patients over 40 to 50 years of age, and in those
with a family history of the disease [3,7]. In a British study, the overall in-
cidence of AACG was found to be as high as 1 in 1000 people over the
age of 40 [8], and the peak incidence occurs between ages 55 and 70 [1].
Patients with AACG complain of an acutely painful, red eye and blurred
vision with halos around lights owing to corneal edema. Other commonly
associated symptoms include frontal headache, nausea, vomiting, and ab-
dominal pain. AACG is often misdiagnosed, particularly when systemic
symptoms, such as abdominal pain, vomiting, and headache are more
* Corresponding author. Department of Emergency Medicine, Quincy Medical Center,

114 Whitwell Street, Quincy, MA 02169.
E-mail address: [email protected] (R.A. Lowenstein).
200 DARGIN & LOWENSTEIN
prominent than ocular complaints [9,10]. Rarely, AACG may present as

painless loss of vision [11]. In approximately 50% of cases, the patient
will report similar past episodes. In these cases, the patient often describes
evening headaches owing to pupillary constriction that are relieved with
sleep [2]. Between attacks the patient is asymptomatic and the eye appears
normal [8]. Most cases are unilateral, but AACG may be bilateral, particu-
larly when medications are implicated [10].
Examination reveals circumcorneal conjunctival injection, a steamy cor-
nea, impaired visual acuity, and a mid-dilated (4–6 mm) and fixed pupil
(Fig. 1). The affected globe is tender and firm compared with the unaffected
eye [2]. An intraocular pressure greater than 40 to 50 mmHg as measured by
tonometry can cause rapid visual loss, and pressures greater than 70 mmHg
can be seen in AACG [2]. A narrow anterior chamber angle, which predis-
poses to AACG, can be confirmed by the oblique flashlight test, during
which a penlight is shone across the anterior chamber, parallel to the iris.
The anterior chamber angle is considered wide if the entire iris is illumi-
nated, and narrow if a shadow is cast across the nasal aspect of the iris
[12]. This test, which has a specificity of 69% in AACG, can help confirm
the diagnosis in the proper clinical setting [12].
AACG is an ocular emergency and prompt consultation with an ophthal-
mologist is imperative, as optic nerve atrophy and permanent loss of vision
can occur within hours if the condition is not adequately treated [9]. Reduc-
tion of intraocular pressure and preservation of vision are the primary goals
of treatment in AACG. Intraocular pressure is lowered by decreasing aque-
ous production with a topical beta blocker (timolol), alpha two agonist
(apraclonidine), and a carbonic anhydrase inhibitor (acetazolamide) [7].
Topical timolol 0.5% can be expected to lower the intraocular pressure
within 30 minutes to 1 hour. Topical beta blockers are systemically
Fig. 1. Conjunctival injection, steamy cornea, and mid-dilated pupil caused by acute angle
closure glaucoma. (From Bertolini J, Pelucio M. The red eye. Emerg Clin North Am 1995;13(3):
561–79; with permission.)
THE PAINFUL EYE 201
absorbed, and should be used cautiously if there is a history of reactive air-

way disease or cardiac conduction abnormality. Acetazolamide can be ad-
ministered as an initial dose of 500 mg orally or intravenously, but is
contraindicated in patients with a sulfa allergy. The intravenous osmotic
agent mannitol can be used as an alternative to acetazolamide [7]. A topical
corticosteroid may also be applied to reduce the inflammation associated
with AACG. In addition to decreasing aqueous production with timolol
and acetazolamide, aqueous outflow can be increased through pupillary
constriction with pilocarpine [12,13]. Topical pilocarpine 2% can be admin-
istered every 15 minutes for the first 1 to 2 hours. The miotic effect of pilo-
carpine may not be observed until the intraocular pressure first has been
reduced below 50 mmHg, at which point the ischemic paralysis of the iris
is relieved [7]. The intraocular pressure should be repeated 1 hour after ini-
tial treatment to confirm that the pressure is dropping. Medical therapy
should be continued if the intraocular pressure has not been reduced, and
definitive surgical therapy with laser iridotomy or peripheral iridectomy
may be necessary in refractory cases.
Scleritis
Scleritis, or inflammation of the sclera, is an uncommon disease, and
many ophthalmologists see only a few cases per year. The emergency physi-
cian nevertheless should be familiar with this condition on account of its po-
tential to cause visual loss and to threaten the integrity of the eye. Scleritis
occurs more commonly in white females and the average age of onset is 49
years [14]. Most cases involve the anterior (visible) portion of the sclera.
Posterior scleritis presents with a painless, red eye. Approximately 39% to
50% of patients with scleritis have an associated rheumatologic disease
[14,15]. It is important to note that scleritis may be the first presentation
of an underlying systemic disease. Infection causes scleritis in 8% of cases,
with herpes zoster being the most common etiology [15]. Scleritis can be
classified as diffuse, nodular, or necrotizing, and this distinction has impli-
cations for both treatment and prognosis.
Patients with scleritis complain of severe, boring pain that is worse with
eye movement and often interferes with normal activity or sleep. The pain
usually progresses insidiously over the course of weeks [14]. Headache
may be the most prominent symptom, making the diagnosis more difficult.
Tearing, blurred vision, and a red eye are also common symptoms. Exami-
nation reveals dilation of the deep episcleral vessels and thinning of the
sclera, resulting in a bluish discoloration of the eye (Fig. 2). Visual acuity
is impaired in 16% of patients [15] and, unlike in episcleritis, the globe is of-
ten tender to palpation. The condition is bilateral in 50% of all cases [15].
Diffuse anterior scleritis is characterized by the absence of nodules and
a lack of scleral necrosis. In nodular scleritis, there are well-localized, tender
areas of edema with dilation of the underlying vessels. In necrotizing
Fig. 2. Dilation of the deep episcleral vessels and a bluish discoloration of the eye in a patient
with scleritis. (Reprinted from Lee AG, Beaver HA, Brazis PW. Painful ophthalmologic disor-
ders and eye pain for the neurologist. Neurol Clin 2004;22(1):75–97. Plate 5; with permission.)
anterior scleritis, there is intense dilation of the deep vasculature and focal
or generalized thinning of the sclera, exposing the underlying choroid.
The eye is exquisitely tender to the touch in necrotizing scleritis, and this se-
vere form of the disease represents a threat to the integrity of the eye. The
bluish discoloration of the sclera helps differentiate scleritis from episcleritis.
In addition, episcleritis tends to cause engorgement of only the more super-
ficial vessels overlying the sclera, which are easily blanched with topical ap-
plication of phenylepherine. In contrast, the deeper vessels involved in
scleritis are unaffected by phenylepherine [14]. The distinction between scler-
itis and episcleritis has both therapeutic and prognostic implications, with
the latter generally being a less malignant process, with fewer ocular compli-
cations [15]. Episcleritis is covered in more detail in an article about red eye
that appears elsewhere in this issue.
Investigation of previously undiagnosed systemic disease, such as rheu-
matoid arthritis (RA), Wegener’s granulomatosis, relapsing polychondritis,
systemic lupus erythematosus, inflammatory bowel disease, and polyarteritis
nodosa, becomes important in scleritis [14]. RA, the most commonly impli-
cated systemic disease, accounts for up to 33% of all cases of scleritis re-
ferred to ophthalmologists [16]. Necrotizing scleritis often develops in the
setting of peripheral ulcerative keratitis associated with vasculitis. Infectious
etiologies include herpes zoster ophthalmicus, herpes simplex, Lyme disease,
and HIV. Although the history and physical examination can help to focus
testing in some cases, the painful, red eye of scleritis may be the only pre-
senting symptom of an underlying systemic disease. In such cases, a complete
blood count, blood urea nitrogen, creatinine, electrolyte panel, rheumatoid
factor, antinuclear antibody, anticytoplasmic antibodies, and urinalysis may
help to reveal an underlying systemic disease. The erythrocyte sedimentation
THE PAINFUL EYE 203
rate and C-reactive protein are useful in assessing the severity of disease and
evaluating response to treatment.
The treatment of scleritis is tailored based on the severity of disease and
should involve consultation with an ophthalmologist. Mild cases of nodular
or diffuse scleritis may respond to oral nonsteroidal anti-inflammatory
drugs (NSAIDs) (oral indomethacin 50 mg 3 times/day), with symptoms
generally resolving over the course of 1 month [15]. Oral corticosteroids
(prednisone 1 mg/kg/day) may be required in refractory anterior scleritis
and necrotizing scleritis. Other immunosuppressive drugs, such as cyclo-
phosphamide and cyclosporine, can be added at the discretion of an oph-
thalmologist or rheumatologist when the condition does not respond to
oral corticosteroids or with the goal of treating an underlying systemic dis-
ease [15]. Scleritis may be complicated by uveitis, keratitis, or glaucoma in
up to 60% of cases [15], and these conditions require appropriate treatment
when they arise. Topical corticosteroids are likely to be of little benefit in
scleritis, but may be used to treat associated anterior uveitis. Patients with
corneal or scleral perforation may require surgical intervention [14]. Most
patients with mild scleritis will have little to no change in visual acuity.
However, necrotizing scleritis is associated with a much higher incidence
of visual loss and a 21% 8-year mortality [7], underscoring the importance
of aggressive treatment and prompt referral for follow-up care.
Anterior uveitis (iritis)

The anterior uvea consists of the iris and ciliary body. Anterior uveitis
refers to inflammation of one or both of these structures. The terms iritis (in-
flammation of the iris), cyclitis (inflammation of the ciliary body), and irido-
cyclitis (inflammation of both anterior uveal structures) are more specific
anatomic descriptions of the involved structures in anterior uveitis. Anterior
uveitis is typically associated with pain, redness, blurred vision, tearing, and
photophobia. Anterior uveitis accounts for 50% to 92% of all cases of uve-
itis in Western countries [17]. In contrast, posterior uveitis, or inflammation
of the choroid, is a less common type of uveitis and is typically painless [18].
The annual incidence of uveitis is 17 in 100,000 people [18]. Anterior uveitis
occurs most commonly between the ages of 20 and 50 and rarely before the
age of 10 or after the age of 70 [17].
Examination reveals conjunctival injection primarily involving the lim-
bus. The presence of inflammatory cells and flare (protein extravasation
from inflamed blood vessels) in the anterior chamber helps to confirm the
diagnosis of anterior uveitis. A sterile hypopyon may develop in severe cases
(Fig. 3). There may be miosis of the affected eye related to ciliary spasm.
Photophobia is commonly present in the affected eye when a light is shone
in either the affected or unaffected eye. The intraocular pressure should be
measured as secondary glaucoma can result from blockage of the trabecular
meshwork by inflammatory cells or from scarring.
Fig. 3. Conjunctival injection due to anterior uveitis. Note the hypopyon in this patient with
HLA-B27-associated disease. (Reprinted from Chang J, McCluskey PJ, Wakefield D. Acute an-
terior uveitis and HLA-B27. Surv Ophthalmol 2005;50(4):364–88. Fig. 1; with permission.)
HLA-B27-associated uveitis
Approximately 60% of all cases of uveitis are idiopathic [19]. Of the iden-
tifiable etiologies of anterior uveitis, HLA-B27-associated uveitis is the most
common, accounting for 30% to 70% of cases. HLA-B27-associated ante-
rior uveitis is characterized by acute (developing over hours to days), uni-
lateral, alternating uveitis, with a high rate of recurrence [17]. The
inflammation of HLA-B27-associated uveitis is often severe, but typically
resolves within 2 to 4 months, leaving little to no visual impairment be-
tweens episodes [7]. Only half of the HLA-B27-associated cases have an as-
sociated systemic disease, such as ankylosing spondylitis, Reiter syndrome,
psoriatic arthritis, or inflammatory bowel disease [18]. The uveitis of Reiter
syndrome and ankylosing spondylitis tend to be acute and unilateral,
whereas those of psoriatic arthritis and inflammatory bowel disease are
more insidious in onset and tend to be bilateral [7]. A small percentage of
patients with HLA-B27-associated anterior uveitis develop synechiae (scar-
ring), which can block the outflow of aqueous and result in AACG.
Other noninfectious etiologies

Other causes of anterior uveitis not typically associated with HLA-B27
include trauma, sarcoidosis, juvenile rheumatoid arthritis, Behçet’s disease,
and Kawasaki’s disease. The history and physical examination can help to
establish the etiology, particularly when there are signs and symptoms of
systemic disease (Table 1). Uveitis associated with juvenile rheumatoid ar-
thritis is unique in that it is often asymptomatic and can cause progressive
visual loss if not detected on routine screening [18]. Other diseases may
mimic anterior uveitis, including lymphoma and leukemia. These so-called
Table 1
Clinical features of uveitis associated with systemic disease
Clinical features
HLA-B27 Incidence of Clinical features of systemic
Disease positive (%) uveitis (%) of uveitis disease Epidemiology
Ankylosing spondylitis 90 20–40 acute, unilateral, sacroiliitis, morning male, young adult
alternating stiffness
Reiter syndrome 60 20–40 acute, unilateral conjunctivitis, urethritis, male, young adult, white
arthritis, recent
genitourinary or enteric
THE PAINFUL EYE

infection
Psoriatic arthritis 40–50 7 insidious, bilateral arthritis, rash, nail changes male ¼ female, ages
30–55, white
Inflammatory bowel disease 35–75 3–11 insidious, bilateral diarrhea female, bimodal peak in
younger and older
adults
Behçet’s disease dd 80 acute, unilateral, hypopyon oral and genital ulcers, male ¼ female, ages
skin lesions 25–30, Asian,
Mediterranean, Middle
Eastern
Sarcoidosis dd 18 insidious, chronic, bilateral respiratory symptoms, hilar female slightly more than
adenopathy, skin lesions, male, young adult,
fever, arthritis African-American
Juvenile rheumatoid dd 5–20 asymptomatic, insidious, fever, arthritis, rash female, age younger than
arthrits bilateral 16, white
Data from Refs. [16–20].
205
‘‘masquerade syndromes,’’ which are typically malignancies that mimic

other causes of anterior uveitis, should be suspected when patients do not
respond to treatment or when uveitis is diagnosed in the elderly.
In patients with unexplained anterior uveitis, performing serologic testing
for syphilis and chest radiography to screen for sarcoidosis is a reasonable
diagnostic approach. Knowing the HLA-B27 status may provide some
prognostic information, as patients with a positive result tend to have
a more severe uveitis [7]. Idiopathic cases of anterior uveitis tend to resolve
in 6 weeks and do not necessarily require an extensive diagnostic workup as
the process tends not to recur [19].
Infectious etiologies
Anterior uveitis may be caused by infectious etiologies, including syphilis,
herpes simplex virus (HSV), and herpes zoster virus. Infectious causes
should be suspected when symptoms do not resolve with anti-inflammatory
therapy. Iritis associated with syphilis is usually acute and unilateral [18].
HSV-associated iritis usually occurs in the setting of stromal keratitis [21].
Zoster iritis may be due to viral infection of the iris itself, and often occurs
when skin lesions are observed on the tip of the nose [21].
Treatment of anterior uveitis

There is a paucity of randomized controlled trials to support the current
treatment of anterior uveitis [19]. Treatment may require a multidisciplinary
approach, including consultation with a rheumatologist and an ophthalmol-
ogist. Topical corticosteroids are used for anterior uveitis and the dosing de-
pends on the severity of disease [18]. Topical prednisolone acetate 1%
achieves a high concentration within the anterior chamber and initially
may be administered hourly in severe cases. Dosing can be slowly reduced
as a therapeutic result is achieved [7]. Cataracts and glaucoma are not
only complications of uveitis, but of treatment with long-term topical ste-
roids as well. Systemic corticosteroids and other immunosuppressive medi-
cations may be used in refractory cases or to treat a specific underlying
systemic disease. Mydriatics relieve the pain associated with ciliary spasm
and may prevent the development of adhesions between the pupil and lens.
Uveitis due to syphilis should be treated as neurosyphilis [18]. HSV-asso-
ciated iritis is treated with topical antivirals. Topical corticosteroids may
also be indicated for HSV-associated iritis, but should be used only after
consultation with an ophthalmologist, given the potential risk of exacerbat-
ing HSV keratitis with this therapy [21]. Zoster iritis usually requires long-
term therapy with topical corticosteroids, and there does not seem to be the
same risk of worsening the infection as with HSV. Zoster iritis can last for
months to years and often causes significant visual impairment, cataracts,
and glaucoma [21].
THE PAINFUL EYE 207
Optic neuritis
Optic neuritis is characterized by inflammatory demyelination of the op-
tic nerve. Although optic neuritis can be associated with many systemic or
infectious diseases, including sarcoidosis, systemic lupus erythematosus,
syphilis, postviral syndromes, lymphoma, and leukemia, it classically occurs
in the setting of multiple sclerosis [7]. In the Optic Neuritis Treatment Trial,
a landmark study of the effects of corticosteroids on optic neuritis, 38% of
patients with optic neuritis ultimately developed multiple sclerosis [22]. Op-
tic neuritis affects women more commonly than men, and the median age of
onset is at approximately 30 years [23].
Patients with optic neuritis complain of unilateral visual loss in most
cases, and there may be associated change in color perception or visual field
defects. Symptoms develop over the course of hours to days. Up to 92% of
cases will be associated with pain [24], which is often worse with eye move-
ment. Pain often begins to resolve after the first few days, as visual loss com-
mences [25]. A small number of cases involve both eyes simultaneously, and
the disease may recur in the same or opposite eye. Examination reveals vi-
sual loss, with a median acuity of 20/60 in the affected eye [24]. A visual field
defect and afferent pupillary defect are also common findings. The optic disk
may appear edematous in some patients, but up to two thirds will have
a normal-appearing optic disk [24]. Although the diagnosis of optic neuritis
is made on a clinical basis, MRI should be routinely performed primarily for
prognostic reasons: patients with one or more demyelinating lesions on
MRI at the time of optic neuritis have a significantly increased risk of being
diagnosed with multiple sclerosis over 10 years [22]. MRI of the orbits,
which reveals characteristic enhancement of the optic nerve, may also be in-
dicated in cases where the diagnosis is in question or when there is a lack of
recovery of visual acuity [25].
The acute management of patients with optic neuritis should involve ad-
mission to the hospital for intravenous methylprednisolone (250 mg every 6
hours for 3 days, followed by an oral prednisone taper), which has been shown
to improve short-term recovery from optic neuritis but has not been demon-
strated to improve long-term visual impairment [26]. Visual loss begins to im-
prove rapidly with intravenous corticosteroids, but will also improve over the
course of weeks without treatment [7]. Most patients eventually regain their
baseline visual acuity [24], but many are left with subtle visual changes that af-
fect their quality of life [7]. Patients who develop multiple sclerosis after initial
optic neuritis generally have relatively mild neurologic disability [22]. (See the
article on optic neuritis elsewhere in this issue.)
Keratitis
Keratitis is defined as inflammation of the cornea. This condition may be
infectious or noninfectious in etiology. Patients with keratitis complain of
photophobia, foreign body sensation, tearing, and exquisite pain due to the
rich sensory innervation from the ophthalmic division of the trigeminal nerve.
Herpes simplex and varicella zoster viruses, which may cause decreased cor-
neal sensation, are often less painful than other causes of keratitis [27]. The
cornea is an important refractory surface of the eye and inflammation may af-
fect visual acuity. In both infectious and noninfectious causes, the corneal in-
flammation may be superficial and involve only the epithelium or deep with
ulceration through the epithelium [27]. In other cases, the epithelium may re-
main intact while the stroma becomes inflamed. With stromal keratitis, an in-
filtrate is observed as a focal area of corneal opacity, which does not stain with
fluorescein. Infectious and noninfectious etiologies can often be determined
from a careful history and examination. Infectious keratitis may be caused
by bacteria, fungi, amoeba, and viruses. Complications of keratitis include
corneal ulcer and perforation, scarring with partial or complete visual loss,
glaucoma, and uveitis. Examination reveals conjunctival injection, which is
more pronounced at the limbus. Cells and flare in the anterior chamber may
be noted on slit lamp examination, and a hypopyon may form in more severe
cases. Corneal defects are seen as green-stained areas when fluorescein is ap-
plied to the affected eye. In superficial keratitis, scarring does not typically oc-
cur and vision is not permanently affected [7].
Noninfectious keratitis
Superficial punctate keratitis
Superficial punctate keratitis (SPK) is a condition characterized by pain,
redness, and tiny, pinpoint areas of fluorescein uptake on examination.
Noninfectious causes of SPK include Thygeson’s SPK, blepharoconjunctivi-
tis, and UV light exposure.
Thygeson’s SPK is a bilateral keratitis of unknown etiology. It is typically
insidious in onset, lasts for months to years, and affects both men and
women in the second and third decades of life. Patients experience repeated
exacerbations and remissions [7]. Thygeson’s SPK is unique form of kerati-
tis in that there is no conjunctival injection. Most patients improve with top-
ical corticosteroids without scarring or significant change in vision [7].
Chronic blepharoconjunctivitis can result in keratitis due to a hypersensi-
tivity reaction to a Staphylococcal antigen associated with this condition.
There are often infiltrates seen on the periphery of the cornea, and in severe
cases the cornea may ulcerate. Treatment of the underlying blepharocon-
junctivitis involves warm compresses and topical or systemic antibiotics
[27]. Topical steroids are indicated for associated keratitis after infectious
etiologies have been excluded.
Most cases of keratitis caused by UV light exposure occur in welders with
inadequate eye protection [28]. UV keratitis has also been described with
tanning booths, other UV lamp exposure, and sunlight reflecting off
THE PAINFUL EYE 209
snow. The onset of pain and photophobia occurs several hours after the UV
exposure [28]. Treatment of UV keratitis includes avoidance of further light
exposure and topical lubricants. In most cases, the cornea heals without
consequence, but permanent corneal damage has been reported [28].
Other causes of SPK include dry eye syndrome, mechanical trauma from
chronic eye rubbing, topical drug toxicity (tobramycin, neomycin, eye drop
preservatives, and topical antivirals), mild chemical injury, and contact lens
use [7,27]. Treatment of SPK in these cases should be directed toward the
underlying cause, as well as appropriate oral analgesics, artificial tears, or
other lubricants, such as erythromycin ointment. In contact lens wearers
with SPK, contact use should be discontinued and a topical antibiotic
with activity against Pseudomonas should be prescribed. Patients should
be reexamined daily until the cornea has healed.
Ulcerative keratitis
Extensive inflammation of the corneal epithelium may result in ulcera-
tion. Although corneal ulcers often result from infectious causes, many non-
infectious etiologies can cause this condition, such as neurotrophic keratitis,
atopic keratoconjunctivitis, vitamin A deficiency, rosacea, and collagen vas-
cular diseases [27]. When ulceration does occur, the cornea is at increased
risk of infection and scarring.
Neurotrophic keratitis results from recurrent injury to the cornea in pa-
tients with decreased corneal sensation. Neurotrophic keratitis can be
caused by HSV and VZV infection, diabetes mellitus, chemical burns,
stroke, and trigeminal nerve palsy [7]. Corneal perforation may result if
the condition goes unrecognized. Treatment is primarily focused on main-
taining eye lubrication and addressing the underlying disease process.
Atopic keratconjunctivitis is associated with allergic conjunctivitis and is
often seen in patients with a history of other allergic disease, such as atopic
dermatitis. The inflamed tarsal conjunctiva initially results in SPK, but these
areas often coalesce to form a corneal ulcer. Treatment depends on the se-
verity of disease, but may include oral antihistamines, topical steroids, top-
ical mast cell stabilizers, and topical NSAIDs [7].
Ulcerative keratitis may be seen in a wide range of collagen vascular dis-
eases, including RA, sarcoidosis, and many of the vasculitides. Ulcerative
keratitis may be the initial presentation of the underlying disease in some
cases. Approximately 20% to 30% of patients with RA have keratoconjunc-
tivitis sicca [27,29], which increases the risk of ulcerative keratitis. Corneal
involvement in RA may also present as peripheral corneal ulceration from
vasculitis [29]. Corneal involvement is common in systemic lupus erythema-
tosus (SLE) and may be a result of autoimmunity or keratoconjunctivitis
sicca. Ocular involvement in sarcoidosis may occur at any time during the
disease. Single or multiple round opacities may be observed on the cornea,
and the condition is often bilateral. The diagnosis may be difficult in the
absence of systemic evidence of sarcoidosis [27]. The vasculitides, including

Wegener’s granulomatosis and polyarteritis nodosa, and Churg-Strauss,
typically cause a peripheral ulcerative keratitis, sparing the avascular central
cornea [29]. Such corneal ulcers are susceptible to infection and this compli-
cation must be excluded before starting corticosteroid therapy. Treatment of
keratitis related to collagen vascular disease is typically directed toward the
underlying disease process and is best undertaken after consultation with
a rheumatologist and ophthalmologist.
Infectious keratitis
Bacterial
Bacterial keratitis is one of the leading causes of blindness in the develop-
ing world [7]. The relatively avascular cornea predisposes to infection, par-
ticularly when the epithelium is compromised. The incidence of bacterial
keratitis is increasing in developed countries, and contact lens use is the
most common risk factor [30], followed by ocular surface disease. In the
United States, the incidence of bacterial keratitis is 10 to 30 per 100,000
in contact lens wearers [7]. A change in the pathogens implicated in bacterial
keratitis can also be attributed to increased contact lens use: Streptococcus
pneumoniae has traditionally been the most common bacterium isolated
from corneal ulcers, but Pseudomonas, Staphylococcus aureus, and Serratia
are now the most commonly isolated organisms in contact lens wearers [7].
Although Neisseria gonorrhea, Corynebacterium diphtheriae, Shigella, and
Listeria can invade an intact cornea [31], disruption of the cornea from un-
derlying corneal disease, corneal trauma and foreign bodies, poor tear pro-
duction, contact lens use, or corneal surgery allows for adherence and
invasion of more commonly isolated bacteria. Diabetes, allergy, and topical
steroid use may also increase the risk of bacterial keratitis [30]. Advanced
HIV does not seem to increase the risk of bacterial keratitis, but patients
may have a more aggressive course once infection occurs.
Examination often reveals a corneal infiltrate, and there may be associ-
ated conjunctival injection and chemosis. Cells and flare are found in the an-
terior chamber in up to 25% of cases [30], which may be severe enough to
develop a sterile hypopyon. Familiarity with Pseudomonas keratitis has be-
come important not only because of its increasing incidence related to con-
tact lens use, but also owing to its virulence: if improperly treated, this
infection can spread rapidly, invading the entire cornea in a matter of hours.
A yellow-green discharge may be seen over the affected areas of the cornea
and ulceration may progress to perforation of the cornea. Anterior chamber
inflammation and hypopyon are commonly seen in Pseudomonas keratitis.
The vast majority of bacterial infections will respond to broad-spectrum
empiric antibiotics, but culture of corneal scrapings may be prudent when
less-common organisms are suspected, when the etiology is not clear from
THE PAINFUL EYE 211
the history, or in more severe cases. Although combination therapy with

a cephalosporin, such as cefazolin and an aminoglycoside or floroquinolone
is typically recommended, antibiotic choice should be tailored based on clin-
ical features, suspected pathogens, and local resistance patterns. Topical an-
tibiotics, which achieve high concentrations in infected tissue without
systemic side effects, are generally preferred over systemic antibiotics. Fre-
quent, repetitive dosing, (every 2 minutes for the first five doses) is usually
used as a loading dose. Antibiotics are then instilled approximately every
30 minutes for the first 24 to 36 hours depending on the severity of the in-
fection [31]. Antibiotic therapy should not be delayed for any reason, as
site-threatening complications can occur rapidly. Many patients require ad-
mission, particularly if treatment noncompliance is a concern or if there is
site-threatening infection. Large, central ulcers or extensive infiltrates can re-
sult in scarring and varying degrees of visual loss.
Viral
Common causes of viral keratitis include HSV, VZV, Epstein-Barr virus,
and adenovirus. HSV is one of the most common causes of corneal blindness
in the world. Initial infection with HSV, which often occurs in childhood, is
asymptomatic in most cases. In symptomatic cases of primary ocular infec-
tion, periorbital cutaneous vesicles are noted and there may be associated ble-
pharitis, conjunctivitis, malaise, fevers, and local lymphadenopathy [21].
Keratitis occurs in 33% to 50% of primary infections, and usually appears
1 to 2 weeks after the appearance of skin lesions. Recurrent disease accounts
for most cases of ocular HSV, and classically presents with unilateral dendritic
corneal defects (Fig. 4), but the lid and conjunctiva may be involved as well.
Within 1 to 2 years, 25% of patients will have a recurrence after an initial in-
fection [21]. Stress, trauma, surgery, and menstrual period often trigger
Fig. 4. Dendritic lesion of herpes simplex keratitis. (Reprinted from Auerback PS. Wilderness
medicine, 5th edition. Philadelphia: Mosby; 2007. Fig. 25–8; with permission.)
recurrent attacks, which lead to corneal scarring and visual loss. Examination
with fluorescein reveals superficial punctate lesions or characteristic dendrites,
which can coalesce and enlarge. Recurrent disease may also be isolated to the
stroma, appearing as a white infiltrate, which tends to be a more severe and
difficult to treat form of HSV keratitis [21]. Interestingly, there is decreased
corneal sensation associated with HSV keratitis. The diagnosis is often
made clinically, but viral culture can be performed if there is uncertainty.
Trifluridine is the drug of choice for topical treatment owing to its high de-
gree of ocular penetration. Trifluridine is administered six to eight times per
day for the first several days and then the frequency is reduced as healing oc-
curs [21]. Up to 97% of patients with dendritic lesions will heal within 2 weeks
of trifluridine therapy [21]. Oral acyclovir 400 mg five times per day is used as
alternative or adjunctive treatment to topical therapy. Topical steroids are
contraindicated in the epithelial stage, but may be used in stromal stages after
consultation with ophthalmologist. HSV keratitis can resolve spontaneously
or with treatment over 1 to 2 weeks. Most patients will have minimal change
in visual acuity, but others will be left with significant visual impairment.
VZV keratitis, similar to HSV, may result in a painful, red eye with fever
and malaise. The skin findings of VZV are painful vesicular lesions over the
ophthalmic division of the trigeminal nerve (forehead and upper eyelid), and
typically do not cross the midline. Less commonly, patients will present with
isolated corneal involvement [32]. The dendritic lesions of VZV keratitis do
not have well-stained terminal bulbs and ulceration is uncommon, in contra-
distinction to HSV keratitis [21]. Corneal sensation is often markedly im-
paired, even in mild cases [27].
VZV keratits is treated with oral acyclovir 800 mg five times per day or
oral valacyclovir 1000 mg three times per day for 7 to 10 days [32]. Therapy
is most effective when started in the first 3 days, but may have some efficacy
within 5 days of the onset of symptoms [21]. There is no clear evidence for
the efficacy of topical antiviral medications for VZV keratitis [21].
There are at least 50 types of adenovirus, and not surprisingly the spectrum
of symptoms is broad, ranging from isolated ocular involvement to ocular dis-
ease associated with pharyngitis or gastroenteritis [21]. Adenovirus is quite
contagious, and ocular disease appropriately has been termed epidemic kera-
toconjunctivitis. In corneal involvement with adenovirus, patients complain
of severe pain and often have bilateral conjunctivitis with a palpable preaur-
icular lymph node. Diffuse punctate keratitis, which typically resolves without
treatment in 7 to 10 days, is followed by subepithelial infiltrates. Topical ste-
roids may provide pain relief and improve vision at this later stage [21].
Fungal
Fungal keratitis is commonly seen in the southern United States and
tropical regions of the world. Infection with Fusarium and Aspergillus is of-
ten caused by traumatic injury from vegetable matter, and Candida albicans
THE PAINFUL EYE 213
is seen in patients with underlying eye disease [33]. Signs and symptoms are
similar to that of bacterial keratitis, and fungal infections are often treated
as bacterial until culture results are available. Topical therapy is used for
mild keratitis and systemic antifungal therapy is added in more severe cases.
Patients are usually admitted to the hospital for hourly dosing of topical an-
tifungal therapy, which may be required for several days [33]. Topical ste-
roids are contraindicated. Anterior uveitis is a common finding and
cycloplegics are often used in these cases. Improvement usually occurs
over weeks; corneal transplant is reserved for refractory cases.
Amoebic
The first case of Acanthamoeba keratitis was described in 1973 [34]. Infec-
tion with this amoeba, which is abundant in the environment, usually occurs
in young, healthy adults and the majority of cases are contact lens related.
Symptoms are similar to bacterial keratitis, although the pain seems to be
more severe and the infection progresses more slowly in the case of Ancan-
thamoeba keratitis [34]. Slit lamp examination may reveal dendritiform le-
sions that can be confused with HSV keratitis. Ancanthamoeba keratitis
should be suspected in any contact lens wearer with dendriform keratitis,
and cultures of the corneal epithelium and contact lenses should be sent
in such cases. Treatment typically involves combination therapy with topical
amoebicidal agents after consultation with an ophthalmologist.
Corneal abrasion
Patients with corneal abrasions are commonly seen in the emergency de-
partment. A corneal abrasion is a traumatic defect in the corneal epithelium,
which may result from direct mechanical trauma, contact lens related injury,
foreign bodies, or motor vehicle accidents with airbag deployment [35]. Pa-
tients complain of pain, sensitivity to light, and excessive tearing following
trauma to the eye. The patient may recollect injury from a fingernail,
makeup applicator, or excessive rubbing of the eyes. In other cases the
trauma may be so minor that it is not remembered by the patient at all.
Trauma to the eye of a machine worker or as a result of metal striking metal
should raise suspicion for penetrating globe injury. In contact lens wearers,
specific history about poorly fitting lenses and prolonged wearing should be
elicited.
Application of a short-acting topical anesthetic, such as proparacaine,
will facilitate examination of the painful eye. Visual acuity is typically nor-
mal unless the abrasion involves the visual axis or there is significant corneal
edema. There may be blepharospasm of the affected eye as a result of pho-
tophobia. Ciliary spasm may cause miosis. The cornea can appear hazy if
there is edema. Conjunctival injection is classically present and is most pro-
nounced at the limbus. The diagnosis is confirmed by green fluorescence in
damaged areas of the cornea seen under Wood’s lamp or cobalt blue light
on slit lamp examination after the application of fluorescein (Fig. 5). Con-
tact lens-related abrasions may be punctate or coalesce to form a larger,
round central abrasion. Multiple vertical abrasions suggest a foreign body
and should prompt the examiner to flip the upper eye lid for careful exam-
ination. Siedel’s sign or the presence of a hyphema on slit lamp examination
suggests penetrating trauma to the globe.
Current treatment for corneal abrasions is largely based on theoretical
benefit and general consensus rather than on rigorous study. Primary goals
of therapy are pain control, prevention of infection, and rapid healing of the
corneal epithelium. In a systematic review of five randomized controlled tri-
als, topical NSAIDs, such as ketorolac, diclofenac, and indomethacin, re-
sulted in a modest decrease in pain without a delay in wound healing or
an increased risk of infection [36]. Patients who use topical NSAIDs may
also return to work earlier and require fewer oral analgesics, including nar-
cotics. Topical NSAIDs are, however, relatively expensive. Cycloplegics
should theoretically relieve pain and photophobia related to ciliary spasm,
but a systematic review showed no clear evidence to support their use in cor-
neal abrasion [37]. Patching of the eye following a corneal abrasion has been
shown to be of no benefit in pain relief or rate of healing, and is no longer
recommended [38]. Concerns over the safety of patching have also been
raised, as this treatment leaves the patient effectively monocular, thus im-
pairing ambulation and driving. Contact lens-related abrasions in particular
are at increased risk of infection when patched, and the eye should not be
covered in these cases. Patients with contact lens-related abrasions should
be instructed to discontinue contact lens use until the defect heals and symp-
toms resolve. Oral analgesics, such as acetaminophen, ibuprofen, or opoids,
Fig. 5. Large corneal abrasion confirmed by fluorescein illumination under cobalt blue light.
(Reprinted from Goldman L, Ausiello D. Cecil text book of medicine, 22nd edition. Philadel-
phia: WB Saunders; 2004. Fig. 465–8; with permission.)
THE PAINFUL EYE 215
are typically prescribed for pain control. The decision to use oral NSAIDs
or opioids should be made on an individual patient basis. In cases where
oral analgesics are contraindicated, topical NSAIDs may be of benefit. A
topical antibiotic, such as erythromycin, is commonly prescribed four times
per day for 3 to 5 days to prevent infection, although there is no strong ev-
idence to support this practice. Because of their lubricating effect, antibiotic
ointments are generally preferred over drops. Patients with contact lens
abrasions should be treated with prophylactic topical antibiotics to cover
Pseudomonas, such as gentamycin or ciprofloxacin. There is no convincing
evidence in the literature to support tetanus prophylaxis in patients with
nonpenetrating corneal abrasions [39].
Most abrasions heal within 1 to 3 days, although defects involving greater
than half of the surface of cornea may require 4 to 5 days to fully heal [40].
Patients with small corneal abrasions will heal quickly and generally require
only a single 24-hour follow-up examination to ensure healing. Patients with
contact lens-related corneal abrasions should be reexamined daily to ensure
prompt healing and to exclude infection. Referral to an ophthalmologist is
indicated for large abrasions, defects over the visual axis, abrasions that be-
come larger or more symptomatic the next day, or for patients who develop
a corneal infiltrate or ulcer.
References
[1] Chang BM, Ritch R, Liebmann JM. Angle closure in younger patients. Ophthalmology
2003;110(10):1880–9.
[2] Khaw PT, Shah P, Elkington AR. Glaucoma-1: diagnosis. BMJ 2004;328:97–9.
[3] Gordon-Bennett P, Ung T, Stephenson C, et al. Misdiagnosis of angle closure glaucoma.
BMJ 2006;333(7579):1157–8.
[4] Zenzen CT, Eliott D, Balok EM, et al. Acute angle-closure glaucoma associated with intra-
nasal phenylephrine to treat epistaxis. Arch Ophthalmol 2004;122(4):655–6.
[5] Fraunfelder FW, Fraunfelder FT, Keates EU. Topiramate-associated acute, bilateral, sec-
ondary angle-closure glaucoma. Ophthalmology 2004;111(1):109–11.
[6] Rho D. Acute angle-closure glaucoma after albuterol nebulizer treatment. Am J Ophthalmol
2000;130(1):123–4.
[7] Yanoff M, Duker JS, Augsburger JJ, et al. Ophthalmology. 2nd edition. St. Louis (MO):
Mosby; 2004. p. 512–8, 1117–20, 1177–80, 11263–7, 1491–8, 465–91.
[8] Dayan M, Turner B, McGhee C. Acute angle closure glaucoma masquerading as systemic
illness. BMJ 1996;313(7054):413–5.
[10] Berkoff DJ, Sanchez LD. An uncommon presentation of acute angle closure glaucoma.
J Emerg Med 2005;29(1):43–4.
[11] Rosenberg CA, Adams SL. Narrow-angle glaucoma presenting as acute, painless visual im-
pairment. Ann Emerg Med 1991;20(9):1020–2.
[12] Coleman AL. Glaucoma. Lancet 1999;354(9192):1803–10.
[13] Khaw PT, Shaw P, Elkington AR. Glaucoma-2: treatment. BMJ 2004;328:156–8.
[14] Okhravi N, Odufuwa B, McCluskey P, et al. Scleritis. Surv Ophthalmol 2005;50:351–63.
[15] Jabs DA, Mudun A, Dunn JP, et al. Episcleritis and scleritis: clinical features and treatment
results. Am J Ophthalmol 2000;130:469–76.
[16] Hamideh F, Prete PE. Ophthalmologic manifestations of rheumatic diseases. Semin Arthri-
tis Rheum 2001;30(4):217–41.
[17] Chang J, McCluskey PJ, Wakefield D. Acute anterior uveitis and HLA-B27. Surv Ophthal-
mol 2005;50(4):364–88.
[18] Hajj-Ali RA, Lowder C, Mandell BF. Uveitis in the internist’s office: are a patient’s eye
symptoms serious? Cleve Clin J Med 2005;72(4):329–39.
[19] Pras E, Neumann R, Zandman-Goddard G, et al. Intraocular inflammation in autoimmune
diseases. Semin Arthritis Rheum 2004;34(3):602–9.
[20] Klippel JH. Primer on the Rheumatic Diseases. 12th edition. Atlanta (Georgia): Arthritis
Foundation; 2001. p. 245–9, 415–8, 455–8, 534–40, 233–8, 250–4.
[21] Kaufman HE. Treatment of viral diseases of the cornea and external eye. Prog Retin Eye Res
2000;19(1):69–85.
[22] Kupersmith MJ, Miller NR, Moke PS, et al. (Optic Neuritis Study Group): neurologic im-
pairment 10 years after optic neuritis. Arch Neurol 2004;61(9):1386–9.
[23] Pirko I, Blauwet LK, Lesnick TG, et al. The natural history of recurrent optic neuritis. Arch
Neurol 2004;61(9):1401–5.
[24] Balcer LJ. Optic Neuritis. N Engl J Med 2006;354(12):1273–80.
[25] Arnold AC. Evolving management of optic neuritis and multiple sclerosis. Am J Ophthalmol
2005;139(6):1101–8.
[26] Beck RW, Cleary PA, Anderson MM Jr, et al. A randomized, controlled trial of corticoste-
roids in the treatment of acute optic neuritis. N Engl J Med 1992;326:581–8.
[27] Sharma S. Keratitis. Biosci Rep 2001;21(4):419–44.
[28] Yen YL, Lin HL, Lin HJ, et al. Photokeratoconjunctivitis caused by different light sources.
Am J Emerg Med 2004;22:511–21.
[29] Messemer EM, Foster S. Vasculitic peripheral ulcerative keratitis. Surv Ophthalmol 1999;
43(5):379–96.
[30] Bourcier T, Thomas F, Borderie V, et al. Bacterial keratitis: predisposing factors, clinical and
microbiological review of 300 cases. Br J Ophthalmol 2003;87(7):834–8.
[31] O’Brien TP. Management of bacterial keratitis: beyond exorcism towards consideration of
organism and host factors. Eye 2003;17(8):957–74.
[32] Shaikh S, Ta CN. Evaluation and management of herpes zoster ophthalmicus. Am Fam Phy-
sician 2002;66(9):1723–30.
[33] Thomas PA. Fungal infections of the cornea. Eye 2003;17(8):852–62.
[34] Illingworth CD, Cook SD. Acanthamoeba keratitis. Surv Ophthalmol 1998;42(6):493–508.
[35] Duma SM, Jernigan MV, Stitzel JD, et al. The effect of frontal air bags on eye injury patterns
in automobile crashes. Arch Ophthalmol 2002;120(11):1517–22.
[36] Weaver CS, Terrell KM. Evidence-based emergency medicine. Update: do ophthalmic non-
steroidal anti-inflammatory drugs reduce the pain associated with simple corneal abrasion
without delaying healing? Ann Emerg Med 2003;41:134–40.
[37] Carley F, Carley S. Towards evidence based emergency medicine: best BETs from the Man-
chester Royal Infirmary. Mydriatics in corneal abrasion. Emerg Med J 2001;18(4):273.
[38] Turner A, Rabiu M. Patching for corneal abrasion. Cochrane Database Syst Rev 2006:
CD004764.
[39] Mukherjee P, Sivakumar A, Mackway-Jones K. Tetanus prophylaxis in superficial corneal
abrasions. Emerg Med J 2003;20(1):62–4.
[40] Wilson SA, Last A. Management of corneal abrasions. Am Fam Physician 2004;70(1):123–8.
Emerg Med Clin N Am
26 (2008) 217–231
Ophthalmologic Complications
of Systemic Disease
Jean E. Klig, MD, FAAP
Division of Pediatric Emergency Medicine, Boston University School of Medicine,
The human eye, as an organ, can offer critical clues to the presence of
systemic illness. Ocular changes are common in the early course of many
systemic infections and inflammatory diseases that may be diagnosed in
the emergency department. A careful and thorough eye examination is par-
amount during routine evaluation in the emergency department because it
can provide primary information on otherwise undetected systemic illness
and corroborative data for known problems. This article reviews various
ophthalmologic complications of systemic disease that can be detected by
an emergency department provider, as well as key findings that can be dis-
cerned with specialty consultation.
Acquired syphilis
Infection with Treponema pallidum affects most organ systems if un-
treated. In fact, acquired syphilis was a common cause of ocular inflamma-
tion before the introduction of effective antimicrobial treatment. Although
the prevalence of acquired syphilis has diminished overall in the post-antibi-
otic era, it still remains an important clinical entity [1]. Current worldwide
estimates suggest the occurrence of approximately 12 million new cases of
syphilis each year, 90% of which are in developing countries [1]. Rates of
primary and secondary syphilis infection in the United States have risen
markedly since 2000, primarily among male homosexuals. Although ocular
syphilis is now rare overall, prolonged untreated syphilis-related ocular dis-
ease can produce destructive changes in the eye; therefore, clinical suspicion
is vital to early intervention. If present, ocular syphilis will manifest during
the second or third stages of clinical illness. A key triad that should prompt
218 KLIG
clinical suspicion of syphilis infection is persistent headache, red eyes or eye

pain, and an unexplained elevated erythrocyte sedimentation rate (ESR) [2].
There are no signs that are pathognomonic of ocular syphilis, and it can
readily mimic other ocular disorders. Madarosis, or loss of the eyelashes or
eyebrows, can occur. Uveitis is the most common eye finding [3], accounting
for the red eyes or eye pain in the triad detailed previously. Eye involvement
usually is evident beyond the primary stage of syphilis, but a primary chan-
cre of the conjunctiva is possible [4]. Anterior segment eye findings that may
be evident to the emergency department physician on direct and slit lamp
examination include keratitis (interstitial or stromal) and iridocyclitis or
anterior uveitis [1,4]. Prolonged syphilitic interstitial keratitis can result in
closed angle glaucoma, which can be a presenting sign of the disease [5].
Acute iridocyclitis occurs in approximately 4% of patients with secondary
syphilis, with bilateral involvement in approximately 50% of cases [4].
A red eye–type finding that the emergency department provider may note
is dilated iris capillaries, or roseolae, often in early iridocyclitis. Roseolae
may progress to discrete papules and then to larger yellow nodules [4].
Posterior segment ocular changes in syphilis include chorioretinitis (uni-
focal, multifocal, or neuroretinitis), posterior uveitis, vaso-occlusive retinal
changes, and retinal detachment [1,4]. The emergency department provider
is most likely to detect associated visual changes and eye redness or ocular
pain on examination, although it may be possible to discern inflammatory
changes to the fundus. Neuro-ophthalmologic changes that may be appreci-
ated are an Argyll Robertson pupil (unilateral or bilateral small pupils that
accommodate to near vision but do not react to light), third and sixth cra-
nial nerve palsies, and visual field defects from brain involvement. Ophthal-
mologic consultation is important in cases of suspected ocular syphilis,
because many of the changes that occur are reversible if detected and mon-
itored during treatment. In addition to a more extensive examination of the
fundus that is performed by an ophthalmologist, additional testing via MRI,
angiography, and other modalities may be performed. Ophthalmologic con-
sultation is critical in HIV patients with evidence of ocular syphilis because
more severe eye disease and greater rates of complication are common [4]. In
turn, HIV testing should be considered in patients with suspected ocular
syphilis given the common risk factors for both diseases [3].
Varicella-zoster virus infection

Varicella and herpes zoster infections are due to the same human herpes
virus 3, also known as varicella-zoster virus (VZV). Immunization against
varicella has diminished the incidence and severity of the disease overall;
however, primary infections and reactivation as herpes zoster continue to
occur in persons who are un-immunized or immunocompromised, or as
‘‘break-through disease’’ in vaccinated patients [6]. Ocular manifestations
of primary varicella infection are generally uncommon. Nonetheless, the
OPHTHALMOLOGIC COMPLICATIONS OF SYSTEMIC DISEASE 219
following have been reported as isolated or combined findings during pri-

mary varicella illness: lid, conjunctival, and corneal vesicles; iridocyclitis;
glaucoma; cataracts; chorioretinitis; optic neuritis or atrophy; and internal
ophthalmoplegia [7]. The common viral prodrome and typical skin lesions
of varicella infection will precede or accompany any ocular involvement.
Ocular changes can also be seen as a late complication of primary varicella
illness [7].
Herpes zoster infection (shingles) results from reactivation of latent VZV
in sensory nerve ganglia, with migration down the nerves to the related der-
matome where severe pain and characteristic skin lesions result [8]. Risk fac-
tors for herpes zoster include advanced age, poor nutrition, immune
compromise, fatigue, and physical or emotional stress [9]. The lifetime
risk of herpes zoster is about 10% to 20% overall; approximately 10% to
25% of cases involve the ophthalmic division of the trigeminal nerve, which
is known as herpes zoster ophthalmicus (Fig. 1). Ocular lesions develop in
approximately half of patients with herpes zoster ophthalmicus [8]. Damage
to the eye and surrounding structures occurs owing to inflammation of sen-
sory nerves, with the potentially serious sequelae of chronic ocular inflam-
mation, vision loss, or severe pain [9]. Although acute herpes zoster
ophthalmicus typically entails the classic VZV rash in the specific derma-
tome, ocular changes can occur with minimal or no rash [8,9]. Up to one
third of patients with herpes zoster ophthalmicus also have involvement
of the nasociliary nerve that innervates the tip of the nose. This involvement
is known as Hutchinson’s sign and is a strong predictor of ocular inflamma-
tion and corneal denervation [8,10]. Patients with Hutchinson’s sign are re-
ported to have twice the incidence of ocular changes overall. Patients
Fig. 1. Herpes zoster ophthalmicus. (From Wipf JE, Paauw DS. Ophthalmologic emergencies in
the patient with diabetes. Endocrinol Metab Clin North Am 2000;29(4):825; with permission.)
220 KLIG
without Hutchinson’s sign may have eye involvement in up to one third of

cases [8,9].
External ocular manifestations of acute herpes zoster ophthalmicus that
may be observed by the emergency department provider include blepharitis,
conjunctivitis, episcleritis, and scleritis. Blepharitis can entail vesicular le-
sions on the eyelids or lid edema and inflammation. The latter may cause
ptosis on the affected side [9]. Conjunctivitis is a common early complica-
tion of herpes zoster ophthalmicus that is associated with vesicles on the
lid margin; the conjunctivae will appear red and swollen and may have pe-
techial hemorrhages [8]. Episcleritis may be seen in as many as one third of
herpes zoster ophthalmicus cases and includes erythema, pain, and swelling
of the episclera that can be masked by overlying conjunctivitis. Scleritis is
a less common but more serious late finding of herpes zoster ophthalmicus
following a week of illness, and the cornea is usually also involved
(sclerokeratitis).
Corneal involvement occurs in 65% of patients with acute herpes zoster
ophthalmicus, and a high level of suspicion for this entity is warranted if vi-
sion loss is reported by the patient or detected on visual acuity testing in the
emergency department [9]. Symptoms of eye pain or light sensitivity may
also occur. The spectrum of corneal changes in herpes zoster ophthalmicus
correlates with the progression of disease and includes epithelial keratitis,
anterior stromal keratitis, and disciform keratitis. Epithelial keratitis is usu-
ally a transient early finding of herpes zoster ophthalmicus that is initially
seen on slit lamp examination as multiple swollen lesions which stain with
fluorescein or rose bengal dye. VZV dendrites may appear later in a branch-
ing or ‘‘medusa-like’’ pattern with similar staining and examination via
Wood’s lamp or slit lamp [9]. Anterior stromal keratitis, or nummular ker-
atitis, occurs in as many as 30% of patients with herpes zoster ophthalmicus
during the second week of disease and likely reflects an antigen–antibody
response to viral proliferation in the overlying epithelium [11]. On slit
lamp examination, multiple patchy fine granular infiltrates with a halo
can be seen in the anterior stroma below areas of previous epithelial keratitis
[8,9]. Disciform keratitis is a late event in herpes zoster ophthalmicus that
occurs in about 5% of cases, usually about 3 weeks after the onset of dis-
ease. It reflects an extension of anterior stromal keratitis that can herald
chronic changes of scarring, lipid deposition, and vascularization, all of
which can compromise vision.
Anterior uveitis, or inflammation of the iris and ciliary body, occurs in
approximately 40% of patients with herpes zoster ophthalmicus. Patients
with the Hutchinson’s sign (involvement of the nasociliary nerve) are at
a higher risk for anterior uveitis. Herpes zoster ophthalmicus iritis is gener-
ally mild, with small keratic precipitates and a faint flare in the anterior
chamber that can be seen on slit lamp examination. A mild elevation in
intraocular pressure frequently occurs. Complications of iris atrophy
(20%), secondary glaucoma (10%), secondary cataracts (rare), and ischemia
of the ciliary body (phthisis bulbi, very rare) can occur [8]. Timely ophthal-
mologic referral and treatment is essential. See the article by Mueller and
McStay elsewhere in this issue for a discussion of treatment options.
Herpes zoster ophthalmicus is commonly associated with many cases of
acute retinal necrosis. Presenting symptoms include blurred vision or pain
in the affected eye. Peripheral patches of retinal necrosis may be evident
on fundoscopic examination. Emergent ophthalmologic consultation is war-
ranted because retinal detachment can occur.
Lyme disease
Lyme disease is endemic to many areas of the United States and Europe
but is an uncommon cause of significant ocular disease [12]. The rare ocular
manifestations of Lyme disease are largely known through case reports and
small case series; epidemiologic studies of patients with Lyme disease have
reported only a few cases of ocular involvement [13]. Ocular complications
of Lyme disease can be similar to those of syphilis, presenting with both
early and late findings [14]. Patients with severe ocular manifestations of
Lyme disease likely have a more chronic form of the disease [15].
Clinical findings in stage 1 Lyme disease include the erythema chronicum
migrans rash and flulike symptoms. Eye changes that may be noted in as
many as 11% of patients are conjunctivitis, periorbital edema, and mild
photophobia [14,15]. Stage 2 Lyme disease develops days to months later
and reflects clinical progression of the illness that may include a relapsing
migratory monoarthritis, neurologic disease (cranial neuropathy, meningi-
tis, or radiculopathy), and cardiac atrioventricular block. Ocular manifesta-
tions that may occur in stage 2 Lyme disease result from intraocular
inflammation that leads to granulomatous iridocyclitis with or without uve-
itis that may be seen on slit lamp examination. Further involvement of the
retina may occur as retinal vasculitis, disk edema, or choroiditis. Neuro-
ophthalmic symptoms of blurred vision, headache, Bell’s palsy, cranial neu-
ropathy, and diplopia may also be present [15]. Decreased color vision and
visual field changes can occur. Other more rare ocular complications in late
stage 2 Lyme disease are paralytic mydriasis, Horner’s syndrome, the Argyll
Robertson pupil, orbital myositis, and temporal arteritis [15].
Stage 3 Lyme disease occurs months to years afterwards and reflects per-
sistent infection that manifests as prolonged chronic or recurrent arthritis or
chronic neurologic syndromes. The severe ocular complications of Lyme
disease seen during stage 3 are episcleritis, stromal keratitis, orbital myositis,
vitreitis, or pars planitis. Episcleritis entails redness, pain, and swelling of
the episclera that can be seen on direct examination. Complaints of photo-
phobia or blurred vision can occur with stromal keratitis; scattered nummu-
lar opacities can be seen on slit lamp examination. Findings in orbital
myositis can range from pain on movement of the affected ocular muscle
to an inability to move the eye in the direction of the affected muscle.
222 KLIG
Vitreitis is evidenced as multiple inflammatory nodules that may be seen on

fundoscopic examination (‘‘snowbank exudates’’). It is referred to as pars
planitis when stromal keratitis is also present. Ophthalmologic consultation
is appropriate to confirm these findings and screen for additional rare com-
plications of Lyme disease [15].
Acquired immunodeficiency syndrome

It is estimated that since the early 1980s over 50 million people have been
infected with HIV worldwide, and more than 22 million deaths have resulted
from AIDS [16]. The advent of highly active antiretroviral therapy has pro-
duced a remarkable decline in all manifestations of HIV infection, including
ocular complications [16]. Nonetheless, ocular findings may provide impor-
tant clues to HIV-related disease.
Conjunctival microvascular disease affects as many as 75% of HIV-infected
patients. Changes of the conjunctiva can appear similar to sickle cell disease
and are best observed on the inferior perilimbal bulbar conjunctiva [17]. Slit
lamp examination may reveal capillary dilations, microaneurysms, variable
short vessel segments or isolated vessel fragments, or a granular appearance
of blood within dilated vessels [18]. Other common findings in HIV disease
include dry eye, keratoconjunctivitis sicca, and chronic allergic conjunctivitis
[17].
External ocular disease in AIDS patients manifests as opportunistic infec-
tions and tumors. The range of opportunistic infections includes viral (her-
pes zoster ophthalmicus, herpes simplex keratitis, molluscum contagiosum,
human papillomavirus), protozoal (microsporidium), and fungal (candida
and cryptococcus) sources [16–18]. Secondary bacterial infections (Staphylo-
coccus aureus, Staphylococcus epidermidis, and Pseudomonas aeruginosa)
may occur with opportunistic eye infections and can result in corneal ulcers.
A higher incidence of preseptal cellulitis is also reported in AIDS patients.
Kaposi’s sarcoma is an AIDS-defining opportunistic tumor caused by hu-
man herpesvirus 8. As many as 20% of patients with Kaposi’s sarcoma
may have ocular involvement [19]. Kaposi’s sarcoma lesions may appear
on the eyelids or conjunctivae as an irregular mass that is red to purple in
color, but they also appear similar to a hordeolum (stye) [17]. Conjunctival
Kaposi’s sarcoma lesions are possible and can be mistaken for subconjunc-
tival hemorrhages which do not resolve [16]. Squamous cell carcinomas
(associated with human papillomavirus infection) may also be seen as exter-
nal ocular manifestations of AIDS disease.
Anterior ocular segment complications of AIDS include infectious kera-
titis (due to the organisms detailed previously) and anterior uveitis. The lat-
ter affects more than half of AIDS patients [20]. Mild iridocyclitis often
results from VZV or herpes simplex virus infections, with inflammatory
changes of the iris and anterior ciliary body seen on slit lamp examination
[16]. More severe anterior uveitis occurs in association with posterior
segment infections. Symptoms may include photophobia, pain, redness, de-

creased vision, or lacrimation.
Posterior segment eye complications occur in as many as 75% of AIDS
patients and often can lead to vision impairment or blindness [19]. Retinal
microangiopathy and cytomegalovirus (CMV) retinitis are most common,
affecting 30% to 40% of AIDS patients overall [16]. The former is of
non-infectious origin and is characterized by cotton-wool spots on fundo-
scopic examination that may be asymptomatic and transient. Retinal
hemorrhages and microaneurysms may also be reported on examination
by an ophthalmologist. CMV retinitis is usually associated with low CD4
T-lymphocyte levels and severe systemic disease, although it may be
a rare presenting manifestation of AIDS [19]. Symptoms of CMV retinitis
include floaters, blurred vision, and visual field loss [16]. Ophthalmologic
consultation is needed given that retinal detachment (30%) and optic nerve
involvement (5%) are possible. The range of optic findings includes granular
retinitis, hemorrhagic retinitis, and perivascular retinitis. Other posterior eye
segment AIDS-related infections are retinitis due to other viruses (VZV, her-
pes simplex) or bacteria (ocular syphilis), choroiditis (Pneumocystis carinii,
Cryptococcus neoformans, Mycobacterium tuberculosis), and retino-
choroiditis (Toxoplasma gondii) [16,19]. Neuro-ophthalmic complications
of AIDS-related intracranial infections or tumors may also occur. Cerebral
toxoplasmosis is most common and can present with cranial nerve palsies,
visual field loss, or papilledema [16].
Reiter’s syndrome
Reiter’s syndrome is a multisystem disease that entails a symptom triad
of urethritis, conjunctivitis, and arthritis with negative serology [21,22]. It
is an uncommon disease overall that is diagnosed more frequently in men
(typically those aged more than 30 years) but can also occur in women
[23]. The onset of Reiter’s syndrome is often preceded by genitourinary in-
fection or bacterial enteric infection [22]. As many as 75% of patients with
Reiter’s syndrome test positive for HLA-B27 histocompatibility antigens.
The most consistent ocular manifestation of Reiter’s syndrome is a mild
bilateral mucopurulent conjunctivitis that is part of the diagnostic triad and
that occurs in up to 58% of patients [24]. Mucopurulent eye discharge will
be noted, and a papillary or follicular reaction is also common [24]. The con-
junctivitis may occur up to 2 weeks after the onset of symptoms of urethritis
but often precedes the onset of arthritis. Acute iritis that is detectable on slit
lamp examination develops in as many as 20% of patients with Reiter’s syn-
drome [25]. Keratitis may also be evident as subepithelial opacities (keratic
precipitates) with overlying punctate lesions [24–26]. These findings of ante-
rior uveitis are more common in patients who test positive for HLA-B27 an-
tigens [26,27]. Other eye complications of Reiter’s syndrome that have been
reported include scleritis, episcleritis, disk edema, retinal edema, and retinal
224 KLIG
vasculitis [24]. Chronic recurrent ocular inflammation may occur following

the acute eye changes and can result in posterior synechiae, glaucoma, cys-
toid macular edema, and cataracts [24].
Infectious endocarditis
Ocular complications of infectious endocarditis typically result from
some type of septic embolic event to the retina. Emergency department pro-
viders may be familiar with the finding of a Roth’s spot, which is commonly
seen in endocarditis and other diseases. A Roth’s spot is a cluster of super-
ficial retinal hemorrhages that can be seen on fundoscopic examination as
oval hemorrhages with pale centers, often located near the optic disk [28].
In endocarditis, this cluster represents red blood cells which surround in-
flammatory cells that have collected in the area in response to a septic em-
bolus [29].
A full spectrum of retinal complications may occur in patients with infec-
tious endocarditis. Most are described in case reports and include focal ret-
initis, embolic retinopathy, subretinal abscesses, choroidal septic metastasis,
choroiditis, endophthalmitis (Fig. 2), papillitis, and optic neuritis [30–36].
Clinical vigilance for symptoms of ocular pain, injection, and vision loss
is critical in patients with known or suspected endocarditis. Any of these
symptoms should prompt an immediate complete examination of the eyes
and consultation with an ophthalmologist.
Kawasaki’s disease
Mucocutaneous lymph node syndrome, or Kawasaki’s disease, is an
acute multi-organ vasculitis that primarily affects children aged 1 to 8 years.
Fig. 2. Enophthalmitis. (From Wipf JE, Paauw DS. Ophthalmologic emergencies in the patient
with diabetes. Endocrinol Metab Clin North Am 2000;29(4):821; with permission.)
It presents with fever and the following symptoms: (1) bilateral bulbar con-
junctivitis without exudates; (2) dryness of the lips and oral cavity, with red-
dening of the mucosa; (3) polymorphous truncal rash without vesicles or
crusts; (4) erythema and edema of the palms and soles, or periungual des-
quamation; and (5) acute nonpurulent cervical lymphadenopathy [37,38].
Incomplete or atypical presentations of Kawasaki’s disease are also possible
[38]. The primary ocular manifestation of Kawasaki’s disease is bilateral
bulbar conjunctivitis (often with limbal sparing); any additional inflamma-
tory eye changes are similarly bilateral [39]. A recent study of conjunctival
swabs from patients with Kaposi’s sarcoma bulbar conjunctivitis reported
a predominance of neutrophils, or ‘‘neutrophilic conjunctivitis,’’ as a cytopa-
thologic change that can be followed as Kawasaki’s disease progresses [40].
Anterior uveitis can occur in 25% to 50% of patients with Kawasaki’s
disease [38]. The initial bulbar conjunctivitis can readily progress to bilateral
iridocyclitis. Symptoms of photophobia, pain, redness, decreased vision, or
lacrimation may occur. Subconjunctival hemorrhages or circumcorneal in-
jection with a violaceous hue may be noted on examination of the eyes.
Slit lamp examination may reveal superficial punctate keratitis, keratitic pre-
cipitates, vitreous opacities, or aqueous cells and a flare response [39,41].
Posterior segment changes have also been reported in Kawasaki’s dis-
ease. Papilledema may occur, for which fluorescein angiography is needed
to detect vasculitis-related changes [39,42]. Retinal ischemia may also occur
as the Kawasaki’s disease vasculitis progresses [43]; therefore, prompt oph-
thalmologic consultation is necessary, particularly if more advanced Kawa-
saki’s disease ocular complications are suspected. Dacrocystitis has been
reported as an eye complication following resolution of acute Kawasaki’s
disease [37].
Temporal arteritis
Giant cell arteritis, or temporal arteritis, is the most common vasculitis in
patients over 50 years of age. The disease involves T cell–mediated inflam-
mation of medium-to-large caliber arteries which produces tissue destruc-
tion, vascular occlusion, and end organ ischemia [44,45]. Although giant
cell arteritis may affect the vascular supply of any organ, cranial ischemic
complications are common and often include the eyes. Patients with giant
cell arteritis have a significant risk of irreversible blindness [45]. As many
as 90% of patients with giant cell arteritis initially complain of headache,
but a wide array of symptoms is possible, including a classic symptom of
jaw pain or claudication [44]. An elevated ESR is also commonly noted,
but a normal ESR does not exclude the disease. Key symptoms of eye
involvement in giant cell arteritis include variable loss of vision, amaurosis
fugax, diplopia, and eye pain [46,47]. Amaurosis fugax has been reported to
be a significant predictor of subsequent irreversible blindness in patients
with giant cell arteritis [45].
226 KLIG
Anterior ischemic optic neuropathy is the most common cause of visual

loss in giant cell arteritis. Changes may be seen on fundoscopic examination
as a pale or chalky white swelling of the optic nerve, and splinter hemor-
rhages may also be present [44]. Diminished intraocular pressure and hypot-
ony of the ciliary body with decreased pupillary response may also be
evident [48–50]. These findings can occur in both eyes [51]. Other ocular is-
chemic lesions that can result from giant cell arteritis but may elude detec-
tion on routine examination are central retinal artery occlusion, cilioretinal
artery occlusion, posterior optic neuropathy, and choroidal ischemia [50,51].
Overall, emergency ophthalmologic consultation is essential to ensure detec-
tion of potential ocular complications of giant cell arteritis.
Giant cell arteritis–related eye changes can progress to irreversible blind-
ness within weeks without immediate treatment. Early diagnosis and a high
level of clinical suspicion for eye complications are vital to achieve the best
possible outcomes. The treatment of temporal arteritis is discussed in more
detail by Vortmann and Schneider elsewhere in this issue.
Hypertension
Hypertension, especially when longstanding or inadequately controlled,
causes a broad array of end organ damage. Ocular complications of hyper-
tension result from direct effects on the eye, as well as secondary effects of
other ophthalmologic conditions [52]. Although hypertensive eye changes
are a major risk factor in patients with diabetes, 2% to 14% of non-diabetic
patients aged more than 40 years are also at risk [53,54]. The primary effects
of hypertension on the eye cause pathologic changes to the retina through
a range that begins with vasoconstriction and progresses to leakage at the
blood–retinal barrier and arteriosclerotic changes.
Hypertensive retinopathy is classically described by four grades of retinal
change based on advanced ophthalmologic examination [55]; however, clini-
cians may observe an array of eye findings on simple fundoscopy that cor-
respond to mild, moderate, and severe hypertensive changes [52]. Mild
retinopathy entails arteriolar narrowing, often seen as arteriovenous ‘‘nick-
ing’’ or ‘‘nipping.’’ Moderate hypertensive retinopathy can entail findings of
‘‘flame’’ or ‘‘blot’’ hemorrhages, cotton-wool spots, microaneurysms, and
hard exudates [52]. All of these findings may occur with severe retinopathy,
as well as swelling of the optic disk. When blood pressure is extremely high,
increased intracranial pressure with resultant optic ischemia (hypertensive
optic neuropathy) will be evident as papilledema on fundoscopic examina-
tion [52]. Overall, hypertensive retinopathy is a well-established sign of sys-
temic vascular disease and is associated with subsequent stroke [52].
Other retinopathies that are known complications of hypertension are as
follows: retinal vein occlusion, retinal emboli, retinal artery occlusion,
retinal macroaneurysm, ischemic optic neuropathy, and diabetic re-
tinopathy. Many of these conditions have common findings, and urgent
ophthalmologic consultation is essential for a correct diagnosis and prompt

treatment. Retinal vein occlusion is a common disorder from which vision
loss can occur. Patients typically present with poor visual acuity; fundo-
scopic findings may include retinal hemorrhages, cotton-wool spots, and
edema of the macular and optic disk. Retinal emboli may be single or mul-
tiple and are often asymptomatic with transient changes, although frank ret-
inal artery occlusion can occur [52]. These emboli may be seen as punctate
pale defects on fundoscopy. Retinal emboli signal a high risk of thromboem-
bolic stroke and cardiovascular disease [52]. Retinal artery occlusion is most
common in hypertensive patients over 60 years old and presents with symp-
toms of a painless and sudden unilateral vision loss. A ‘‘cherry red spot’’
may be seen on fundoscopic examination. A retinal arterial macroaneurysm
is a more rare disorder that is somewhat unique to patients with hyperten-
sion. It can present with acute vision loss but is more likely discovered inci-
dentally on advanced ophthalmologic examination of an asymptomatic
patient. Ischemic optic neuropathy is the most common acute optic neurop-
athy of patients aged more than 50 years and is more common in patients
with diabetes. Up to 90% of cases are anterior and present with abrupt
vision loss and edema of the optic disk. Diabetic retinopathy is detailed in
the following section.
Diabetes
Emergency department providers are likely to encounter many of the
complications of type 1 and type 2 diabetes mellitus, especially as the overall
incidences of diabetes and obesity continue to rise. Ocular complications of
diabetes are no exception. Indeed, diabetic retinopathy remains the most
common cause of legal blindness in patients between 18 and 74 years of
age. Early recognition with prompt treatment can dramatically affect out-
come [56]. Other ocular complications of diabetes include vascular events
and severe eye infections.
The prevalence of diabetic retinopathy is markedly higher in patients who
have type 1 diabetes (estimated at 40% to 80%), but it also affects 20% to
30% of patients who have type 2 diabetes [56,57]. Hypertension is also a sig-
nificant risk factor for diabetic retinopathy. Intensive medical control of
blood glucose levels has been shown to significantly reduce both the inci-
dence of new retinopathy and the progression of pre-existing retinopathy.
Moreover, control of hypertension with angiotensin-converting enzyme in-
hibitors can reduce the progression of diabetic retinopathy. Despite the rou-
tine monitoring for diabetic retinopathy as a standard, diabetic patients may
still present to the emergency department with complaints of vision change.
In these cases, prompt ophthalmologic consultation is required for an opti-
mal outcome.
Three main types of diabetic retinopathy form the spectrum of progres-
sion of ocular changes: non-proliferative (background), pre-proliferative,
228 KLIG
and proliferative retinopathy [57]. Non-proliferative diabetic retinopathy

may be evident on simple fundoscopic examination as dilated veins, micro-
aneurysms temporal to the macula, and small hemorrhages in either a ‘‘dot
and blot’’ pattern (deeper) or flame shape (more superficial) [56]. More ex-
tensive ophthalmologic examination and imaging may reveal additional
findings of hard exudates, retinal edema, and arteriovenous shunting. Pre-
proliferative diabetic retinopathy entails lesions resulting from retinal ische-
mia. The lesions may be evident on simple fundoscopic examination as
multiple cotton-wool spots, vascular changes (narrowed arterioles; dilated
veins in bead, loop, or sausage-like patterns), or dark blot hemorrhages
[56]. The last change is more likely seen on further retinal examination or
fluorescein angiography, along with capillary non-perfusion filling defects,
macular edema, and intraretinal microvascular abnormalities. Proliferative
diabetic retinopathy is best detected by an ophthalmologist. Findings in-
clude retinal scars (retinitis proliferans), new vessel formation, vitreous hem-
orrhage, and retinal detachment [56].
Diabetic patients are at risk for ocular vasocclusive events, presumably
due to a hypercoaguable state [56]. Complications include central retinal ar-
tery occlusion (Fig. 3), central retinal vein occlusion, and ocular motor
nerve palsies. Central retinal artery occlusion presents with sudden near
complete or complete vision loss. Fundoscopic examination may reveal ret-
inal edema and pallor and a darkened macula; further findings are possible
on fluorescein angiography [56]. Central retinal vein occlusion presents with
a slower onset of blurred vision and variable loss of vision. Fundoscopic ex-
amination may show retinal edema, dilated and twisted retinal veins, and
retinal hemorrhages with cotton-wool spots (‘‘blood and thunder’’) [56]. Oc-
ular motor nerve palsies can result from ischemic cranial nerve injuries,
which in diabetic patients most commonly involve the oculomotor (third)
Fig. 3. Central retinal artery occlusion. (From Wipf JE, Paauw DS. Ophthalmologic emergen-
cies in the patient with diabetes. Endocrinol Metab Clin North Am 2000;29(4):819; with
permission.)
nerve. The sixth and fourth nerves can also be affected but in decreasing
order of frequency. Symptoms of third nerve palsy can include diplopia,
ptosis, and sometimes headache and eye pain; a slight anisocoria may be
noted, although the pupil is usually spared. Interestingly, ocular motor
nerve palsies usually occur in diabetic persons who do not have diabetic
retinopathy.
Severe eye infections are rare in diabetes but are uniformly destructive
when present. Two ocular infections that occur primarily in diabetic patients
are bacterial endophthalmitis (see Fig. 2) and rhinocerebral mucormycosis.
The former is a severe ocular complication that often results in blindness.
A wide array of bacterial causes is possible and includes Staphylococcus au-
reus, Escherichia coli, and Klebsiella pneumoniae. Presenting symptoms in-
clude pain, photophobia, floaters, and reduced vision. Hypopyon (pus)
can be seen in the anterior chamber on examination. Rhinocerebral mucor-
mycosis is a rare but serious fungal complication of type 1 diabetes that
presents in combination with diabetic ketoacidosis. Initial clinical symptoms
include eye pain, headache, and nasal stuffiness. Periorbital swelling and
proptosis may be noted, along with necrotic lesions on the palate and nasal
mucosa [56]. Further complications include cavernous sinus thrombosis,
internal carotid artery thrombosis, and death.
Summary
A wide array of ocular changes is possible in many systemic diseases.
Emergency department providers may encounter ophthalmologic signs or
symptoms during the evaluation of a patient with known systemic disease,
or in a patient with as yet undiagnosed illness. Careful screening of visual
acuity, a complete external eye and fundoscopic examination, as well as
slit lamp examination are needed in any patient with eye complaints and es-
pecially when progression of known systemic disease is suspected. Emergent
ophthalmology consultation is critical if any degree of vision loss is reported
or detected on screening. Many ocular complications of systemic disease
also require urgent ophthalmologic evaluation to ensure the best possible
treatment outcomes are achieved.
References
[1] Gaudio PA. Update on ocular syphilis. Curr Opin Ophthalmol 2006;17:562–6.
[2] Mikita C, Truesdell A, Katial RK. A 48-year-old woman with red eyes and a rash. Ann
Allergy Asthma Immunol 2004;93:526–31.
[3] Aldave AJ, King JA, Cunningham ET. Ocular syphilis. Curr Opin Ophthalmol 2001;12:
433–41.
[4] Kanski JJ. Acquired syphilis. In: Clinical ophthalmology. London: Butterworth; 1994.
p. 173–4.
[5] Matsuo T, Taira Y, Nagayama M, et al. Angle-closure glaucoma as a presumed presenting
sign in patients with syphilis. Jpn J Ophthalmol 2000;44:305–8.
230 KLIG
[6] Committee on Infectious Diseases, American Academy of Pediatrics. Varicella-zoster infec-

tions. In: The red book. Illinois: The American Academy of Pediatrics; 2006. p. 711–24.
[7] Fernandez de Castro LE, Al Sarraf O, Hawthorne KM, et al. Ocular manifestations after
primary varicella infection. Cornea 2006;25(7):866–7.
[8] Kanski JJ. Herpes zoster ophthalmicus. In: Clinical ophthalmology. London: Butterworth;
1994. p. 112–5.
Physician 2002;66:1723–30.
[10] Liesegang TJ. Herpes zoster virus infection. Curr Opin Ophthalmol 2004;15:531–6.
[11] Marsh RJ. Herpes zoster keratitis. Trans Ophthalmol Soc U K 1973;93:181–92.
[12] Lesser RL. Ocular manifestations of Lyme disease. Am J Med 1995;8(Suppl 4A):60–2.
[13] Karma A, Mikkila H. Ocular manifestations and treatment of Lyme disease. Curr Opin
Ophthalmol 1996;7(1):7–12.
[14] Kanski JJ. Lyme disease. In: Clinical ophthalmology. London: Butterworth; 1994. p. 175.
[15] Zaidman GW. The ocular manifestations of Lyme disease. Int Ophthalmol Clin 1997;37(2):
13–28.
[16] Moraes HV. Ocular manifestations of HIV/AIDS. Curr Opin Ophthalmol 2002;13:397–403.
[17] Chronister CL. Review of external ocular diseases associated with AIDS and HIV infection.
Optom Vis Sci 1996;73(4):225–30.
[18] Shuler JD, Engstrom RE, Holland GN. External ocular disease and anterior segment disor-
ders associated with AIDS. Int Ophthalmol Clin 1989;29:98–104.
[19] Kanski JJ. Acquired immune deficiency syndrome. In: Clinical ophthalmology. London:
Butterworth; 1994. p. 168–71.
[20] Cunningham ET Jr. Uveitis in HIV positive patients. Br J Ophthalmol 2000;84:233–5.
[21] Mahoney BP. Rheumatologic disease and associated ocular manifestations. J Am Optom
Assoc 1993;64(6):403–15.
[22] Schneider JM, Matthews JH, Graham BS. Reiter’s syndrome. Cutis 2003;71(3):198–200.
[23] Neuwelt CM, Borenstein DG, Jacobs RP. Reiter’s syndrome: a male and female disease.
J Rheumatol 1982;9(2):268–72.
[24] Kiss S, Letko E, Qamruddin S, et al. Long-term progression, prognosis, and treatment of
patients with recurrent ocular manifestations of Reiter’s syndrome. Ophthalmology 2003;
110(9):1764–9.
[25] Kanski JJ. Reiter’s syndrome. In: Clinical ophthalmology. London: Butterworth; 1994.
p. 157–8.
[26] Lowder CY, Char DH. Uveitis, a review. West J Med 1984;140(3):421–32.
[27] Ostler CR, Dawson J, Schachter J, et al. Reiter’s syndrome. Am J Ophthalmol 1971;71:
986–91.
[28] Horton JC. Roth’s spots. In: Knoop KJ, Stack LB, Storrow AB, editors. Atlas of emer-
gency medicine. 2nd edition. New York: McGraw-Hill; 2002. p. 80.
[29] Kanski JJ. Anaemias. In: Clinical ophthalmology. London: Butterworth; 1994. p. 373.
[30] Disorders of the eye. In: Kasper DL, Braunwald E, Hauser S, et al, editors. Harrison’s prin-
ciples of internal medicine. 16th edition. Iowa: McGraw-Hill; Part 2, Section 4, Chapter 25.
[31] Zayit-Soudry S, Neudorfer M, Barak A, et al. Endogenous phialemonium curvatum
endophthalmitis. Am J Ophthalmol 2005;140(4):755–7.
[32] Arcieri ES, Jorge EF, de Abrea Ferreira L, et al. Bilateral endogenous endophthalmitis
associated with infective endocarditis: case report. Braz J Infect Dis 2001;5(6):356–9.
[33] Shmuely H, Kremer I, Sagie A, et al. Candida tropicalis multifocal endophthalmitis as the
only initial manifestation of pacemaker endocarditis. Am J Ophthalmol 1997;123(4):
559–60.
[34] Verweij PE, Rademakers AJ, Koopmans PP, et al. Endophthalmitis as presenting symptoms
of group G streptococcal endocarditis. Infection 1994;22(1):56–7.
[35] Munier F, Othenin-Girard P. Subretinal neovascularization secondary to choroidal septic
metastasis from acute bacterial endocarditis. Retina 1992;12:108–12.
[36] Herschorn BJ, Brucker AJ. Embolic retinopathy due to Corynebacterium minutissimum
endocarditis. Br J Ophthalmol 1985;69:29–31.
[37] Mauriello JA, Stabile C, Wagner RS. Dacrocystitis following Kawasaki’s disease. Ophthal
Plast Reconstr Surg 1986;2(4):209–11.
[38] Committee on Infectious Diseases, American Academy of Pediatrics. Kawasaki’s disease.
In: The red book. Illinois: The American Academy of Pediatrics; 2006. p. 412–5.
[39] Ohno S, Miyajima T, Higuchi M, et al. Ocular manifestations of Kawasaki’s disease.
Am J Ophthalmol 1982;93(6):713–7.
[40] Al-abbadi MA, Abuhammour W, Harasheh A, et al. Conjunctival changes in children with
Kawasaki disease: cytopathologic characterization. Acta Cytol 2007;51(3):370–4.
[41] Jacob JK, Polomeno RC, Chad Z, et al. Ocular manifestations of Kawasaki disease.
Can J Ophthalmol 1982;17(5):199–202.
[42] Anand S, Yang YC. Optic disc changes in Kawasaki disease. J Pediatr Ophthalmol Strabis-
mus 2004;41(3):177–9.
[43] Font RL, Mehta RS, Streusand SB, et al. Bilateral retinal ischemia in Kawasaki disease.
Ophthalmology 1983;90(5):569–77.
[44] Bhatti MT, Tabandeh H. Giant cell arteritis: diagnosis and management. Curr Opin Oph-
thalmol 2001;12:393–9.
[45] Gonzalez-Gay MA, Garcia-Porrua C, Llorca J, et al. Visual manifestations of giant cell
arteritis: trends and clinical spectrum in 161 patients. Medicine 2000;79(5):283–92.
[46] Paraskevas KI, Boumpas DT, Vrentzos GE, et al. Oral and ocular/orbital manifestations of
temporal arteritis: a disease with deceptive clinical symptoms and devastating consequences.
Clin Rheumatol 2007;26(7):1044–8.
[47] Hayreh SS, Podhajsky PA, Zimmerman B. Ocular manifestations of giant cell arteritis.
Am J Ophthalmol 1998;125(4):509–20.
[48] Huna-Baron R, Mizrachi IB, Glovinsky Y. Intraocular pressure is low in eyes with giant cell
arteritis. J Neuroophthalmol 2006;26(4):273–5.
[49] Radda TM, Bardach H, Riss B. Acute ocular hypotony. Ophthalmologica 1981;182(3):
148–52.
[50] Calcagni CA, Claes CA, Maheshwari M, et al. Hypotony as a presentation of giant cell ar-
teritis. Eye 2007;21:123–4.
[51] Casson RJ, Fleming FK, Shaikh A, et al. Bilateral ocular ischemic syndrome secondary to
giant cell arteritis. Arch Ophthalmol 2001;119:306–7.
[52] Wong T, Mitchell P. The eye in hypertension. Lancet 2007;369:425–35.
[53] Yu T, Mitchell P, Berry G, et al. Retinopathy in older persons without diabetes and its re-
lationship to hypertension. Arch Ophthalmol 1998;116:83–9.
[54] Wong TY, Mitchell P. Hypertensive retinopathy. N Engl J Med 2004;351:2310–7.
[55] Kanski JJ. Hypertensive retinopathy. In: Clinical ophthalmology. London: Butterworth;
1994. p. 367–70.
[56] Wipf JE, Paauw DS. Ophthalmologic emergencies in the patient with diabetes. Endocrinol
Metab Clin North Am 2000;29(4):813–29.
[57] Kanski JJ. Diabetic retinopathy. In: Clinical ophthalmology. London: Butterworth; 1994.
p. 344–57.
Emerg Med Clin N Am
26 (2008) 233–238
Conditions Requiring Emergency

Ophthalmologic Consultation
Brendan Magauran, MD, MBA
Department of Emergency Medicine, Boston University School of Medicine,
Ophthalmologic complaints in the Emergency Department (ED) are esti-

mated to represent 3% of all visits [1]. Emergency physicians are well pre-
pared to diagnose and manage the majority of these visits. Corneal
abrasions, conjunctivitis, and conjunctival or corneal foreign bodies com-
prise 75% of ED visits [2]. The challenge for the emergency physician is to
decide which cases require the expertise of an ophthalmologist and in what
time frame. Optimally, the decision should be evidence based and in accor-
dance with clinical guidelines developed collaboratively between emergency
physicians and ophthalmologists. Hopefully, in the not too distant future,
this will be the case. This article reviews the instances where emergency con-
sultation with an ophthalmologist is warranted, with specific reference to
each article contained in this issue of Clinics.
Emergency ophthalmology consultation caveats

The availability of on-call specialists to the ED on a 24-hour a day basis
is not uniform across EDs in the United States. Specialty eye hospitals and
tertiary care centers, which also function as Level 1 Trauma Centers, for the
most part, do have around-the-clock ophthalmology coverage. Many other
hospitals, however, do not have this level of coverage or lack subspecialty
services in ophthalmology. Ophthalmology has subspecialties, including
oculo-plastics, neuro-ophthalmology, and retina. A general ophthalmologist
may feel uncomfortable with a particular request for consultation and ask
that the patient be referred to a facility with a higher level of care and oph-
thalmologic subspecialization. This is particularly true for cases involving
possible postoperative complications from eye surgeries. It is therefore
234 MAGAURAN
important for emergency medicine practitioners who find themselves in such

a situation to have referral hospitals that can be contacted to accept patients
in transfer.
Emergency diagnoses requiring emergency ophthalmology consultation

The following box contains a list of diagnoses or conditions that gener-
ally warrant an emergency ophthalmologic consultation (Box 1).
There are also cases where emergency ophthalmologic consultation is rea-
sonable, and these include patients with one good eye experiencing vision
changes, patients with a complicated ocular history, as well as cases where
the ED practitioner is unsure of a diagnosis and is concerned about vision
loss. A telephone discussion to determine the timing of the consultation
with the ophthalmologist will usually answer such questions.
Trauma
Emergency physicians are well versed in the management of trauma. The
basic ABC principle (Airway, Breathing, Circulation) in ED trauma man-
agement takes precedence over eye injuries. Evaluation for life and limb-
threatening injuries is the first priority. Most patients with multiple trauma
and an eye injury will require ophthalmologic consultation, but the timing
of this consultation is dependent on the severity of other injuries. Most
often, this consultation occurs at some point during the in-patient stay after
life threatening conditions have been addressed. This article reviews the
conditions that usually require ophthalmologic consultation from the ED.
Box 1. Conditions that generally warrant an emergency

ophthalmologic consultation
Trauma
Ruptured globe
Lid laceration through margin, nasolacrimal system,
or cannaliculus
Endophthalmitis
Angle closure glaucoma
Severe uveitis
Corneal ulceration
Acute vision loss
Temporal arteritis
Retinal detachment
Optic Neuritis
Orbital cellulitis
OPHTHALMOLOGIC CONSULTATION 235
CT scan is the imaging modality of choice in defining the extent of blunt

and penetrating trauma to the eye and orbit. Orbital fractures are not an
emergency unless there is vision loss or globe injury. Retrobulbar hemor-
rhage may occur with nondisplaced orbital fractures and may result in acute
vision loss. Penetrating injuries with globe rupture also require CT scanning
for retained foreign bodies, but sensitivity is only 75% [3]. These entities are
discussed in detail in the article by Linden and Bord elsewhere in this issue.
Direct trauma to the eyelid involving the nasolacrimal system, lid margin,
or tarsal plate requires specialized repair by either a plastic surgeon or an
ophthalmologist, depending on institutional preference and availability.
The timing of this repair should occur after any life or limb-threatening
injuries are addressed.
Endophthalmitis
Endophthalmitis represents inflammation of the aqueous or vitreous
humor. It may result from infection, trauma, or as a postoperative compli-
cation. This is generally a difficult diagnosis to make in the ED. Suspicion of
the diagnosis requires emergent ophthamologic consultation for diagnostic
evaluation, hospital admission, and surgery as necessary.
Acute angle closure glaucoma

Acute angle closure glaucoma (AACG) is generally painful and occasion-
ally associated with headache, nausea, vomiting, and abdominal pain. The
diagnosis may be difficult to establish when abdominal complaints are
prominent [4]. Intraocular pressure generally rises beyond 50 mm Hg and
may induce optic nerve atrophy if untreated. The emergency physician
can certainly initiate therapy and consult an ophthalmologist. The issue is
then response to treatment and monitoring of the intraocular pressure.
Generally, patients with a new diagnosis of AACG are admitted to the
hospital and an ophthalmologist oversees medical therapy. Cases refractory
to medical therapy may require surgical intervention. AACG is discussed in
detail in the article by Lowenstein and Dargin elsewhere in this issue.
Severe uveitis
Anterior uveitis (iridocyclitis) is inflammation of the iris (iritis) and ciliary
body (cyclitis) and accounts for the majority of uveitis in Western countries
[5]. It typically occurs in patients between the ages of 20 and 50 [5]. Inflam-
matory cells and flare in the anterior chamber associated with conjunctival
injection primarily involving the limbus help confirm the diagnosis. The
etiology of anterior uveitis is broad and may include infection, rheumato-
logic disease (HLA-B27 particularly), idiopathic, or even a malignancy
which may mimic anterior uveitis. Given the broad differential diagnosis as-
sociated with this condition, consultations with specialists in rheumatology,
236 MAGAURAN
infectious diseases, oncology, and ophthalmology may be warranted. A com-

plete discussion of uveitis is found elsewhere in this issue in the article by
Klig, entitled ‘‘Ophthalmologic Complications of Systemic Disease’’; the ar-
ticle by Mahmood and Narang, entitled ‘‘Diagnosis and Management of the
Red Eye’’; and the article by Mueler and McStay, entitled ‘‘Ocular Infection
and Inflammation’’.
Corneal ulceration
Degradation of the corneal stroma leads to ulceration of the cornea and is
associated with numerous types of infection, systemic diseases, and gluco-
corticoid usage, and may lead to permanent vision loss. The depth of the
ulceration may lead to corneal perforation with extension of the infection
into the anterior chamber. Corneal ulcerations tend to heal with scarring, re-
sulting in corneal opacification and decreased visual acuity. Ophthalmologic
consultation and diagnosis of the etiology of the ulceration may decrease
permanent damage to the cornea and resultant vision loss. The article by
Klig, entitled ‘‘Ophthalmologic Complications of Systemic Disease’’; the ar-
ticle by Mahmood and Narang, entitled ‘‘Diagnosis and Management of the
Red Eye’’; and the article by Mueler and McStay, entitled ‘‘Ocular Infection
and Inflammation’’ found elsewhere in this issue discuss this topic in greater
detail with reference to the etiology of the corneal ulceration.
Acute visual loss

Acute visual loss is a concerning complaint and can be difficult to diag-
nose. Presenting symptoms can be numerous and seemingly unrelated to
the eye. It can be a painful or painless condition. The four major diagnoses
addressed in this article include temporal arteritis, optic neuritis, central
retinal artery occlusion, and retinal detachment.
Temporal arteritis
Temporal arteritis is a diagnosis that is in the differential diagnosis for
acute vision loss. The gold standard for diagnosis is a temporal artery
biopsy positive for giant cell arteritis. The end result of this condition is
retinal ischemia and vision loss. Fundoscopic abnormalities or optic atrophy
are not helpful in establishing or eliminating the diagnosis [6]. Suspicion of
the diagnosis with prompt referral to an ophthalmologist is important, as
the administration of high dose steroids may help prevent further vision
loss [7]. Improvement in vision loss was present in only 4% of patients in
one retrospective study [8].
Optic neuritis
Patients with optic neuritis generally present with pain on eye movement
associated with unilateral vision loss, visual field defects, and change in
OPHTHALMOLOGIC CONSULTATION 237
color perception. Pain resolves as visual loss commences [9]. MRI reveals
demyelinating lesions and may be helpful in establishing the diagnosis of
multiple sclerosis. The vision loss is progressive and may occur rapidly
over several hours or more slowly over days. Treatment with steroids
generally leads to improved vision over several weeks [10]. Consultation
with neurology as well as ophthalmology is generally warranted, given the
likelihood of other conditions, such as multiple sclerosis, as well as other
etiologies of acute vision loss.

Central retinal artery occlusion (CRAO) results in a sudden painless loss
of vision. The primary cause of CRAO is embolic disease, but there are
numerous other conditions that may cause CRAO as well. Immediate oph-
thalmologic consultation is warranted as vision may be preserved with treat-
ment. Irreversible vision loss generally occurs after 4 hours of ischemia [11].
Visual acuity at presentation is predictive of final visual acuity. However, in
a 1980 experimental study of ischemic time after retinal artery occlusion,
restoration of blood flow at 100 minutes was associated with preservation
of vision [12]. That being said, the medical and surgical treatments for
CRAO have marginal benefit overall in preserving vision. Unfortunately,
spontaneous resolution of an embolus is quite rare [13].
Retinal detachment
Retinal detachment is characterized by the separation of the retina from the
underlying retinal epithelium. The incidence of retinal detachment is roughly
one to two cases per ten thousand people [14,15]. Patients complain of new
floaters, squiggly lines, or cobwebs that appear abruptly, associated with vi-
sual field loss. Examination with an ophthalmoscope is generally insufficient
as the detachment may be at the periphery of the retina where the retina is
the thinnest. The classic finding of a white billowing retinal separation is help-
ful if present, but the presence of a new visual field deficit with new ‘‘floaters’’
requires a dilated examination by an ophthalmologist [16].
The above causes of acute visual loss are all covered in more detail in the
article by Vortmann and Schneider elsewhere in this issue.
Orbital cellulitis
Cellulitis involving the orbital tissues is usually an extension of an infec-
tion involving the sinuses. Patients may present with significant pain, swell-
ing, and even proptosis, with increased risk of permanent ocular damage
and vision loss. Potentially fatal complications of orbital cellulitis include
meningitis and cavernous sinus thrombosis. CT scan can define the limits
of infection and the presence of an abscess. Admission and emergent
ophthalmologic consultation are warranted. This is discussed in detail else-
where in this issue by Mueller and McStay.
238 MAGAURAN
In summary, this article has attempted to provide guidelines to the emer-

gency physician regarding when to call for emergency ophthalmology con-
sultation. It is not intended to provide an exhaustive list of every possible
problem that may be encountered in the ED requiring the expertise of an
ophthalmologist.
References
[1] Nawar EW, Niska RW, Xu J. National hospital ambulatory medical care survey: 2005 emer-
gency department summary. Advance Data from Vital and Health Statistics 2007;386:1–32.
[2] Nash EA, Margo CE. Patterns of emergency department visits for disorders of the eye and
ocular adnexa. Arch Ophthalmol 1998;116:1222–6.
[3] Joseph D, Pieramici D, Beauchamp N. Computed tomography in the diagnosis and
prognosis of open-globe injuries. Ophthalmology 2000;107:1899–906.
[5] Chang J, McCluskey PJ, Wakefield D. Acute anterior uveitis and HLA-B27. Surv Ophthal-
mol 2005;50(4):364–88.
[6] Smetana GW, Shmerling RH. Does this patient have temporal arteritis? JAMA 2002;287(1):
92–101.
[7] Aiello PD, Trautmann JC, McPhee TG, et al. Visual prognosis in giant cell arteritis.
Ophthalmology 1993;100(4):550–5.
[8] Hayreh SS, Zimmerman B, Kardon R. Visual improvement with corticosteroid therapy in
giant cell arteritis. Report of a large study and review of literature. Acta Ophthalmol Scand
2002;80(4):355–67.
[9] Beck RW, Cleary PA, Anderson MM Jr, et al. A randomized, controlled trial of corticoste-
roids in the treatment of acute optic neuritis. N Engl J Med 1992;326:581–8.
[10] Beck RW, Gal RL, Bhatti MT, et al. Visual function more than 10 years after optic neuritis:
experience of the Optic Neuritis Treatment Trial. Am J Ophthalmol 2004;137(1):77–83
[Erratum in: Am J Ophthalmol 2004].
[11] Hayreh SS, Zimmerman MB, Kimura A, et al. Central retinal artery occlusion. Retinal
survival time. Exp Eye Res 2004;78(3):723–36.
[12] Hayreh SS, Weingeist TA. Experimental occlusion of the central artery of the retina. Retinal
tolerance time to acute ischaemia. Br J Ophthalmol 1980;64(11):818–25.
[13] Rumelt S, Dorenboim Y, Rehany U. Aggressive systematic treatment for central retinal
artery occlusion. Am J Ophthalmol 1999;128:733–8.
[14] Algvere PV, Jahnberg P, Textorius O. The Swedish retinal detachment register. I. A database
for epidemiological and clinical studies. Graefes Arch Clin Exp Ophthalmol 1999;237:
137–44.
[15] Rowe JA, Erie JC, Baratz KH, et al. Retinal detachment in Olmsted County, Minnesota,
1976 through 1995. Ophthalmology 1999;106:154–9.
[16] Shingleton BJ, O’Donoghue MW. Blurred vision. N Engl J Med 2000;343(8):556–62.

Ophthalmologic Emergencies

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Ophthalmologic Emergencies

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Emerg Med Clin N Am

In this issue of Emergency Medicine Clinics of North America, guest

Joseph H. Kahn, MD, FACEP Brendan Magauran, MD, MBA

diseases, including aﬀerent papillary defect, Argyll Robertson pupil, and

Joseph H. Kahn, MD, FACEP

The Physical Examination of the Eye

The external eye

stroma, Descemet’s layer, and the endothelium. The epithelium is highly

The anterior chamber

The extraocular muscles

The rectus muscles

The oblique muscles

Innervation of the eye

The optic nerve (II)

The oculomotor nerve (III)

The trochlear nerve (IV)

The trigeminal nerve (V)

The abducens nerve (VI)

The blood supply of the eye

Physical examination of the eye

cellulitis) or dacrocystitis, which is an infection of the tear collection system

Examination of the conjunctiva

Examination of the sclera and episclera

Examination of the uvea

Examination of the cornea

Examination of the pupils

Examination of the anterior chamber

physical examination of the eye. A goal-directed physical examination of the

Ophthalmologic emergencies account for up to 3% of visits to emergency

Visual acuity testing

Slit lamp examination

anterior chamber examination is mainly to assess the depth of the chamber

Fig. 2. Schematic representation of visualized semicircles while using applanation tonometer.

Foreign body removal

for a complete discussion of this topic). At present, CT scans are considered

Contact lens removal

of intravenous (IV) tubing to allow continuous irrigation of the eye. To

should have improved visual acuity. If the pressure continues to remain

and a 1-hour hands-on teaching experience. Of these 61 patients, the correct

Diagnosis and Management

The red eye is a clinical problem encountered on a daily basis in most

are pharyngoconjunctival fever and epidemic keratoconjunctivitis. Pharyng-

tenderness through closed lids, and preauricular lymphadenopathy may all

possibly asymptomatic, cervical/urethral chlamydial infection, laboratory

(Fig. 6). When evaluating a subconjunctival hemorrhage, one should con-

characterized by an avascular area of scleral necrosis and often by signiﬁ-

In acute presentations of scleritis, blood work may include a complete

photophobia can be a helpful distinguishing feature of anterior uveitis be-

therapy in treating noninfectious causes [15]. Topical corticosteroids (eg,

Acute angle-closure glaucoma

problems, recent laser treatment or surgery, medications), a slit lamp exam-

uveitis associated with ﬂoaters also represent serious, sight-threatening con-

Ocular Infection and Inﬂammation

Managing the inﬂamed or infected eye in the emergency setting presents

contrast to viral conjunctivitis, discharge may be purulent, and crusting

whether to treat for gonococcal conjunctivitis is based on the history, fulmi-

diagnosis also includes conjunctivitis, scleritis, keratitis, and glaucoma.

give a history of recent eye surgery, infection, new malignancy, or new

pattern. Advanced or recurrent disease may lead to stromal (subepithelial

careful so as to avoid missing the lesions. Complications include uveitis and