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,
1 Boston Medical Center Place, Boston, MA 02118, USA

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

* Corresponding author.
E-mail address: [email protected] (D.H. Dorfman).

0733-8627/08/$ - see front matter Ó 2008 Elsevier Inc. All rights reserved.
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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).
184 PRENTISS & DORFMAN

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).
 PEDIATRIC OPHTHALMOLOGY 185

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
186 PRENTISS & DORFMAN

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
 PEDIATRIC OPHTHALMOLOGY 187

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
188 PRENTISS & DORFMAN

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
 PEDIATRIC OPHTHALMOLOGY 189

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.
190 PRENTISS & DORFMAN

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).
 PEDIATRIC OPHTHALMOLOGY 191

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.
192 PRENTISS & DORFMAN

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).
 PEDIATRIC OPHTHALMOLOGY 193

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
194 PRENTISS & DORFMAN

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.
 PEDIATRIC OPHTHALMOLOGY 195

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,
196 PRENTISS & DORFMAN

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.

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