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ARTICLE

Ocular Disorders in the Newborn


Lauren C. Mehner, MD, MPH,* Jasleen K. Singh, MD*
*Department of Ophthalmology, University of Colorado School of Medicine, Aurora, CO

PRACTICE GAPS
Eye examination of the newborn is a challenging but essential component
of the comprehensive neonatal examination. Any abnormality noted on
red reflex testing or inspection of the anterior ocular structures most likely
indicates underlying ocular pathology that requires evaluation by an
ophthalmologist. Increased knowledge of the critical ocular anomalies in
newborns that can lead to vision impairment and associated morbidity and
mortality as well as how to identify these anomalies on bedside
examination is essential for all neonatal clinicians.

OBJECTIVES After completing this article, readers should be able to:

1. Recognize the importance of the eye examination as part of the


neonatal examination.
2. Describe the techniques involved in the neonatal eye examination.
3. List common congenital and acquired ocular anomalies in the newborn
period and describe their characteristics.
4. Explain indications for referral to ophthalmology and the timing of this
referral based on the clinical diagnosis.

ABSTRACT
Visual development begins at birth and continues throughout childhood.
Ocular pathology can lead to permanent visual impairment and
subsequent problems in overall development and school performance as
well as signify serious systemic disease or even life-threatening
malignancies. Prompt identification of congenital or early acquired ocular
pathology via regular bedside ocular examinations and assessments of
visual behaviors by neonatal clinicians is a critical component of newborn
care. The goal of this article is to review the components of the newborn AUTHOR DISCLOSURE Drs Mehner and
Singh have disclosed no financial
eye examination, describe key ocular findings and diagnoses that are relationships relevant to this article. This
common or critical to identify in the newborn period, and offer guidance commentary does not contain a
on necessity and timing of ophthalmology referral. discussion of an unapproved/
investigative use of a commercial
product/device.

INTRODUCTION ABBREVIATIONS
The first few months after birth are a critical period of visual development. Poor ASD anterior segment dysgenesis
visual input during this time from a variety of structural or anatomic factors can CVI cortical visual impairment
MRI magnetic resonance imaging
cause severe amblyopia (decreased vision in 1 or both eyes because of abnormal NLDO nasolacrimal duct obstruction
development of vision in infancy or childhood). Therefore, the eye examination ROP retinopathy of prematurity

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is an essential component of the newborn assessment, as sclera, and iris. Using the direct ophthalmoscope, the red re-
early diagnosis and ophthalmology referral can have a crit- flex from the retina is seen through the pupil. The test is per-
ical impact on the prognosis for many potentially blinding formed in a darkened room to facilitate maximal pupil
but treatable ocular conditions. (1) dilation without the use of dilating eye drops. The ophthalmo-
scope is held at arm’s length from the neonate and both eyes
EYE ANATOMY AND NORMAL DEVELOPMENT are viewed simultaneously to assess for symmetry in the col-
The axial length of the eyeball undergoes rapid growth af- or, brightness, and size of the red reflex. This is called the
ter birth, starting at approximately 16 mm and extending Bruckner test. Each eye can then be assessed individually for
to 20.3 mm by 18 months of age. The newborn pupil size more detail. The reflection can vary from a yellow-orange to a
is roughly 70% of the adult pupil, and the pupil response darker red depending on the pigmentation of the individual
is present by 31 weeks’ gestation. An estimated 21% of but should be symmetric. A dull, absent, white (leukocoria)
term infants have physiologic anisocoria (unequal pupil (3) or asymmetric red reflex or black spot within the reflex in-
size, typically <1 mm). Pupil asymmetry greater than 1 dicates media opacity (eg, cornea opacity, cataract) blocking
mm, poor light response, or other irregular shape (eg, co- the reflection or posterior segment abnormality (eg, tumor,
loboma) should prompt an ophthalmology referral. (2) retinal detachment) that necessitates ophthalmology referral
Visual development begins immediately after birth, with the for complete evaluation. (1) Figure 1 demonstrates the various
blink to light response as the earliest assessment of visual func- possible results of the red reflex test, and a more complete
tion. Fixation and more definitive visual behavior become evi- differential for an abnormal red reflex in an infant is demon-
dent around 6 to 8 weeks of age, though eye alignment can be strated in the Table.
quite variable in the first 3 to 4 months of age. Intermittent exo-
tropia is evident in up to 70% of term infants in the first 2 EYELIDS AND ORBITS
months after birth and decreases significantly in incidence by 4 Proptosis
months; whereas esotropia is less common at birth, typically Proptosis, or relative anterior bulging of 1 or both eyes in
small angle, and rare beyond 2 months of age. (2) the orbit, is rare in the neonatal period and typically the
Nystagmus, or involuntary rhythmic abnormal eye result of an orbital mass (vascular tumor, teratoma, der-
movements with a slow movement away from the visual moid) or craniofacial malformation, in which case other
target followed by a second movement back to the target, clinical examination findings can provide clues to the diag-
can have a wide variety of causes. Infant idiopathic nystag- nosis. Orbital imaging is often indicated and an ophthal-
mus and sensory nystagmus (caused by anatomic disor- mic examination is required to rule out compromise to
ders of the eye resulting in poor vision) are the most other ocular structures (eg, extraocular muscle restriction,
common forms seen in the neonatal period, and usually globe or optic nerve compression) that may lead to vision
manifest a few months after birth. Therefore, poor visual loss if not adequately addressed.
behavior, persistent strabismus, or abnormal eye move-
ments beyond age 3 months warrants thorough evaluation Ptosis
by an ophthalmologist. Congenital ptosis is most commonly the result of a devel-
opmental anomaly of the levator muscle and is typically
EYE EXAMINATION TECHNIQUES unilateral and sporadic. Neurologic causes of ptosis in-
The newborn eye examination consists of assessment of the clude congenital Horner syndrome (unilateral ptosis with
visual system via blink to light response; external examina- ipsilateral iris heterochromia and miosis), cranial nerve III
tion of the orbit, eyelids, and anterior segment; pupil exami- palsy (ptosis with ‘down and out’ position of the globe),
nation; and red reflex test to assess for media opacities or and Marcus Gunn jaw-winking syndrome (synkinesis of
posterior segment abnormalities. Shining the light of a direct cranial nerves III and V resulting in unilateral ptosis with
ophthalmoscope into each eye individually should illicit a eyelid excursion during chewing/sucking often seen while
blink or startle response in a newborn with normal visual feeding). (4) An eyelid mass can produce a mechanical
function. Attention should then be paid to symmetry of pupil ptosis. Severe ptosis that obstructs the visual axis is highly
size, equal constriction to light, and any abnormalities in the amblyogenic and requires urgent ophthalmology referral.
shape or appearance suggestive of an anatomic defect. Nonobstructive ptosis can still cause amblyopia because of
Evaluation of the external and anterior ocular structures in- strabismus or asymmetric refractive error and therefore
cludes inspection of the eyelids, orbits, cornea, conjunctiva, necessitates routine ophthalmic evaluation.

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Table. Differential Diagnosis for Abnormal Red Reflex
Location Diagnosis
Anterior segment  Corneal opacity
 Congenital cataract
Inherited
Persistent fetal vasculature
Metabolic
Infectious
Posterior segment (leukocoria)  Retinal detachment
Coats disease
Norrie disease
Retinopathy of prematurity
Incontinentia pigmenti
 Retinoblastoma
 Coloboma
Retina
Optic nerve
 Vitreous hemorrhage
 Infection
Toxocariasis
Endophthalmitis
 Uveitis
 Myelinated nerve fiber layer

Coloboma
Eyelid colobomas are full-thickness defects in the lid
caused by failure of closure during development (Fig 2A).
They are typically sporadic and located in the medial up-
per eyelid; however, they are associated with 2 common
syndromes that should be kept on the differential when
evaluating patients with eyelid defects (Goldenhar syn-
drome—upper lid location, Treacher Collins syndrome—
lower lid location). (5) Close monitoring of the ocular sur-
face is important because exposure can lead to corneal
scarring and vision loss if left untreated and is often the
leading factor in timing of surgical repair.

Nasolacrimal System Abnormalities


A dacryocystocele is a congenital distention of the lacrimal
sac that manifests at birth as a cystic subcutaneous nodule
with a bluish hue located inferior to the medial canthus
(Fig 2B). Risks of congenital dacryocystoceles include in-
fection, with reported rates of acute dacryocystitis (Fig 2C)
developing in 20% to 75% of patients, as well as respirato-
ry compromise and feeding difficulty caused by the intra-
nasal extension of the cyst and the obligatory nasal
breathing of neonates. (6)(7) Therefore, in addition to sys-
Figure 1. Red reflex examination: A) Normal: Child looks at light. Both red
reflections are equal. B) Unequal refraction: One red reflection is brighter temic antibiotics for infected dacryocystoceles, this condi-
than the other. C) No reflex (cataract): The presence of lens or other media tion also requires urgent referral for decompression and
opacities blocks the red reflection or diminishes it. D) Foreign body/abra-
sion (left cornea): The red reflection from the pupil will back-light corneal probing of the nasolacrimal system.
defects or foreign bodies. Movement of the examiner’s head in 1 direction In contrast to a dacryocystocele, a typical nasolacrimal
will appear to move the corneal defects in the opposite direction. E) Stra-
bismus: The corneal light reflex is temporally displaced in the misaligned duct obstruction (NLDO) presents 1 to 2 weeks after birth
right eye, indicating esotropia. (Used with permission of Alfred G. Smith, with persistent tearing and intermittent mucoid discharge of
MD, #1991.)
1 or both eyes without associated palpable nodules and low

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Figure 2. A) Right eyelid coloboma. B) Congenital dacryocystocele in a 1-week-old infant. C) Dacryocystitis in a 5-day-old infant requiring intravenous
antibiotics and surgical decompression.

risk of infection. Initial management for an NLDO con- 2 to 4 days. Largely attributed to the application of sil-
sists of observation and lacrimal sac massage; 80% or ver nitrate previously used as prophylaxis against
more of congenital NLDO cases resolve spontaneously bacterial conjunctivitis, chemical conjunctivitis is ex-
by age 12 months. (8) If not resolved by then, ophthal- ceedingly rare in developed industrialized countries af-
mology referral for surgical probing of the nasolacrimal ter the switch to erythromycin ointment or 2.5%
system to relieve the obstruction is indicated. When povidone-iodine solution (not currently approved for
making this diagnosis, clinicians should take care to rule use in the United States).
out more serious causes of persistent tearing such as 2. Neisseria gonorrhea conjunctivitis presents with bilateral
congenital glaucoma, infection, or corneal injury/foreign conjunctival injection, chemosis, significant mucopuru-
body. lent discharge, and eyelid edema 3 to 5 days after birth.
Rapid progression to corneal involvement leading to ul-
ANTERIOR SEGMENT ceration and perforation is the most feared complication,
Infection necessitating prompt diagnosis and treatment with irriga-
Neonatal conjunctivitis occurs within the first 30 days after tion with sterile saline to remove the discharge and mem-
birth by definition and is an increasingly uncommon but se- branes, topical erythromycin ointment, and intravenous
rious complication in the newborn period. All clinicians or intramuscular ceftriaxone. Hospitalization is usually re-
should be able to quickly diagnose and initiate treatment of quired to monitor for disseminated N gonorrhea infection
neonatal conjunctivitis to minimize the risk of permanent vi- as systemic complications include arthritis, meningitis,
sion loss or progression of systemic illness. Diagnostic clues and septicemia. These infants and their mothers should
include timing of onset, appearance and amount of dis- also be treated concurrently for Chlamydia.
charge, and associated maternal or newborn illness. 3. Chlamydial conjunctivitis presents 5 to 14 days after birth
with conjunctival hyperemia and scant mucoid discharge,
1. Chemical conjunctivitis presents within the first 24 with more severe cases developing chemosis, eyelid ede-
hours after birth with bilateral conjunctival injection ma, and pseudomembrane formation (Fig 3A). Because
and watery discharge and spontaneously resolves within pneumonia develops in up to 20% of these infants,

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hospitalization may be required. Treatment consists of and direct treatment; this is because other bacteria such as
oral erythromycin and topical erythromycin ointment. Staphylococcus aureus, Pseudomonas, and other pathogens can
4. Herpes simplex virus keratoconjunctivitis presents at 1 to 2 sometimes be the infective organism. Depending on the clin-
weeks of age with periocular vesicular skin lesions, eyelid ical context, polymerase chain reaction testing, blood cul-
edema, conjunctival injection, and a nonpurulent discharge. ture(s), and further evaluation for other infections may be
Corneal involvement includes microdendrites and geograph- warranted. Although the neonatal clinician is critical in mak-
ic ulcers that can be identified with fluorescein staining and ing the initial diagnosis of conjunctivitis and initiating treat-
slit-lamp examination. Systemic infection can be concurrent ment, it is beneficial to involve ophthalmology early in the
so patients should be monitored closely. Treatment consists clinical course, especially if corneal involvement is suspected.
of oral or intravenous acyclovir and topical antiviral medica-
tions, if there is corneal involvement, to minimize risk of cor- Cornea
neal scarring and subsequent vision loss. (9) Corneal clouding or opacity is associated with a wide variety
In addition to a thorough clinical examination, neonates of pathology and necessitates ophthalmology consultation to
with conjunctivitis should have conjunctival scrapings sent help determine the etiology, early treatment, and prompt
for Gram staining and culture to help identify the organism evaluation for any associated syndromes, if indicated.

Figure 3. A) Mucopurulent discharge in a 10-day-old infant with chlamydial conjunctivitis. B) Cloudy cornea in a newborn with Peters anomaly. C) Con-
genital cataract viewed by retroillumination on red reflex testing. D) Persistent fetal vasculature cataract.

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Trauma a full systemic evaluation depending on the presumed di-
Birth trauma, typically involving forceps delivery, can agnosis. (11)
cause vertical tears in Descemet membrane (posterior cor- Sclerocornea is a congenital corneal opacification result-
neal layer), resulting in unilateral corneal clouding. Most ing from ingrowth of scleral tissue into the peripheral cor-
clouding resolves within a few months, but these infants nea. It is a nonprogressive condition and may be part of
should be followed by an ophthalmologist because this the ASD spectrum or occur in isolation.
opacification can be amblyogenic if persistent and located
within the visual axis. These patients can also develop sig- Metabolic
nificant astigmatism, which can also be amblyogenic. The most common metabolic cause of corneal opacity or
clouding is mucopolysaccharidosis and its variants. The
Congenital Glaucoma clouding is a result of accumulation in the cornea of the
The classic triad of photophobia, tearing, and blepharo- metabolic pathway products and presents as bilateral dif-
spasm is diagnostic for congenital glaucoma. Persistently fuse corneal haze. If a metabolic etiology is suspected, fur-
elevated intraocular pressure results in breaks in Desce- ther systemic evaluation is indicated. Dense clouding is
met membrane (horizontal tears called Haab striae) and amblyogenic so in addition to treating the underlying dis-
corneal clouding. It also leads to optic nerve damage, in- order, the main ocular intervention is eventual corneal
creased corneal diameters, and buphthalmos (enlarged surgery.
globe) as a result of rapid axial length elongation. Neo-
nates with suspected congenital glaucoma require urgent Edema
ophthalmology referral as surgical intervention is the pri- Several congenital corneal dystrophies present at or just
mary treatment modality and prompt lowering of intraocu- after birth with varying degrees of corneal opacity or
lar pressure can prevent and even reverse damage to the clouding, usually caused by a defect in the endothelium,
optic nerve and associated vision loss. (10) or most posterior corneal layer. The pattern of opacity may
be patchy or diffuse, and usually both eyes are involved.
Corneal Ulcer Most dystrophies are hereditary and not associated with
Corneal ulcer presents as an infiltrative opacification and systemic disease. Like the metabolic disorders mentioned
associated thinning of the cornea. Most commonly, ulcers earlier, the mainstay of ocular intervention is surgery.
are the result of infection (see the Conjunctivitis section
above) and require urgent ophthalmology consultation for Dermoid
culture and initiation of topical antibiotic drops. Sterile A dermoid is an overgrowth of normal, benign tissue in
ulceration can develop from exposure related to eyelid an abnormal location. It can grow anywhere on the body,
defects, lagophthalmos, or neurotrophic cornea. including the orbit or on the ocular surface. Typically, ocu-
lar surface dermoids grow at the limbus, or the junction
Anterior Segment Dysgenesis of the cornea and sclera, and look like a white nodular le-
Anterior segment dysgenesis (ASD) encompasses various sion covering a portion of the cornea. Because these can
developmental disorders of the components of the anterior cause amblyopia if obstructing the visual axis as well as ir-
segment of the eye (cornea, iris, lens, and aqueous hu- ritation, ophthalmology referral is warranted for possible
mor). Often, the presenting clinical feature is an opacifica- surgical intervention.
tion or clouding of the cornea or other structural
abnormalities of the iris or lens visible on penlight exami- Iris Coloboma
nation. Secondary glaucoma is common in these patients. Often identified during the pupil examination, iris colobo-
The most common ASD disorder is Peters anomaly, mas present as an irregularly shaped pupil or iris tissue,
which presents with variable central corneal opacity often often with a keyhole appearance. Colobomas are a result
with iris strands adherent to the posterior cornea (Fig 3B). of incomplete closure of the embryonic fissure during the
The lens is sometimes involved as well. On the far end of early weeks of development, and typically occur in the in-
the spectrum is Peters-plus syndrome, which is a bilateral ferior nasal quadrant of the iris. They can be sporadic in
Peters anomaly with associated congenital brain defects, occurrence or associated with several genetic syndromes,
heart defects, and craniofacial anomalies. All patients with including CHARGE syndrome (colobomas, heart defects,
suspected ASD require ophthalmic evaluation and possibly atresia of the nasal choanae, restriction of development,

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genitourinary abnormalities, ear and hearing anomalies) Retinoblastoma is covered in detail in a separate article in this
and renal coloboma syndrome among many others, so a issue of NeoReviews and therefore not discussed further here.
genetics consultation is often warranted. In addition, rou-
tine ophthalmology referral is indicated to evaluate for co- Retinopathy of Prematurity
lobomas or dysgenesis in other ocular structures. Retinopathy of prematurity (ROP) is a prematurity-related
ocular complication distinguished by neovascularization of
Aniridia the retina, leading to retinal detachment and blindness in
Aniridia refers to hypoplasia or absence of iris tissue. On ex- the most severe cases. Because ROP is associated with
amination, iris tissue can range from the appearance of a lower gestational age and birthweight, the criteria for
persistently large or dilated pupil to a mere iris stump near screening include: 1) birthweight of 1,500 g or less, 2) ges-
the limbus. Roughly two-thirds of aniridia cases are isolated tational age of 30 weeks or less, or 3) infants with an un-
abnormalities inherited in a dominant genetic fashion with stable clinical course believed by their attending
variable expressivity. Important to note is that a subset of the neonatologist/pediatrician to be at high risk for ROP. (14)
sporadic cases of aniridia is part of the WAGR syndrome A detailed review of this topic can be found in a recent is-
(Wilms tumor, aniridia, genitourinary anomalies, and intel- sue of NeoReviews (15) and therefore not discussed further
lectual disability [formerly known as mental retardation]) and in this review.
therefore, these patients are at risk of developing a Wilms tu-
mor. Additional ocular disorders associated with aniridia in- Retinal Hemorrhages
Fundus hemorrhages are common among newborns, pre-
clude foveal hypoplasia, cataract, glaucoma, and corneal
sent in an estimated 20% to 34% of infants. (16)(17) The
abnormalities. Ophthalmic referral is therefore warranted in
hemorrhages can involve all 4 quadrants and multiple retinal
all cases of aniridia and visual prognosis is widely variable
layers. They are more common in vaginal deliveries (specifi-
depending on the extent of additional structural defects. (12)
cally vacuum-assisted deliveries) compared with cesarean sec-
tions and are thought to be caused by an acute rise in
Lens
intracranial pressure as the head passes through the birth ca-
Congenital cataracts can vary from a small white central dot
nal. They can be an incidental finding on dilated eye exami-
to a diffuse lens opacification visible on red reflex testing as a
nation for other reasons and do not require any intervention
black dot or smudge in the reflex (Fig 3C) to a significant
because most cases resolve within 2 weeks of the initial find-
dimming of the reflex or leukocoria because of blockage of
ing. (17) Similarly, subconjunctival hemorrhages are also a
the light by the opacified lens. Unilateral cataracts are typical-
common finding in the newborn period among both vaginal
ly sporadic. Persistent fetal vasculature is a type of unilateral
and cesarean deliveries because of elevated venous pressure
congenital cataract associated with microphthalmia, elonga-
in the head and neck from uterine contractions. (18) It is im-
tion of the ciliary processes, and stalk extending from the
portant to remember that ocular hemorrhages can also be ev-
posterior lens surface to the optic nerve (Fig 3D). Severe cases
idence of abusive head trauma in infants in the appropriate
can involve retinal detachments. Bilateral cataracts, in con-
context, and suspicion for this should be investigated with
trast, are more commonly caused by an inherited genetic mu-
the applicable team of subspecialists.
tation or associated with systemic abnormalities such as
metabolic disorders (galactosemia, hypocalcemia, etc), infec-
Retinal/Optic Nerve Colobomas
tion (eg, TORCH [toxoplasmosis, other agents, rubella, cyto- Similar to iris colobomas, colobomas of the retina or optic
megalovirus, and herpes simplex] infections), or syndromes nerve are the result of incomplete closure of the embryon-
such as trisomy 21. Although it is important to pursue the ap- ic fissure during development. The most common location
propriate evaluation in these cases, most bilateral cataracts is the inferior nasal quadrant of the involved structure. De-
have no identifiable cause. (13) pending on the size and location of the coloboma, visual
acuity can vary significantly, with early associated nystag-
POSTERIOR SEGMENT mus a poor prognostic sign for vision. Posterior segment
Retinoblastoma colobomas may be identified on newborn eye examination
Retinoblastoma is the most common primary ocular tumor as a dull or white red reflex. Numerous syndromic and ge-
in children and can affect 1 or both eyes. The most common netic abnormalities are associated with ocular colobomas
presenting sign is leukocoria on the red reflex test. so a multidisciplinary approach to evaluation and

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diagnosis should include the neonatologist or pediatrician, ophthalmologist. Early intervention with low vision serv-
ophthalmologist, geneticist, and other subspecialists, ices is important to help affected children optimize their
based on clinical examination findings. Although there is visual function and overall development.
currently no definitive treatment for ocular colobomas in
the posterior segment, these patients should be followed
Summary
by an ophthalmologist to ensure optimization of vision via
• Based on current practice guidelines, the eye
correction of refractive error, strabismus management,
examination is a critical component of the
and overall ocular health.
comprehensive newborn and infant examinations. (1)
• Studies suggest that an abnormal red reflex test,
Optic Nerve Hypoplasia
when performed correctly, is highly specific in
Optic nerve hypoplasia is characterized by a decreased
detecting ocular pathology that requires
number of optic nerve axons resulting in a nerve that is
ophthalmology referral. (22)
small and often pale or gray in appearance. It can be a
• Knowledge of the common ocular anomalies as
unilateral or bilateral finding, nonprogressive, and the as-
well as how to identify them on routine
sociated visual acuity can vary widely from 20/20 to no
examination is an essential skill for all neonatal
light perception. Because of the common association with
clinicians.
midline cerebral structural defects (absent septum pelluci-
dum, agenesis of the corpus callosum, pituitary gland dys-
function, etc), magnetic resonance imaging (MRI) is
indicated in patients with this diagnosis, as well as referral
to appropriate subspecialties such as endocrinology, de- American Board of Pediatrics
pending on the imaging findings. (19) Similarly, MRI Neonatal-Perinatal Content
findings suggestive of septo-optic dysplasia or other optic
nerve abnormality should prompt ophthalmology referral.
Specifications
• Know the normal anatomy and ophthalmologic findings
of the developing eye.
Cortical Visual Impairment
• Recognize the importance of corneal opacifications.
Cortical (cerebral) visual impairment (CVI) is currently the
• Know the syndromes associated with abnormalities of
leading cause of significant vision impairment in children in the eye including cranio-facial abnormalities, coloboma,
developed countries as a result of increased survival of pre- abnormalities of the orbit, the eyebrows, the eyelids, the
mature infants. (20) It is characterized by a decreased visual eyelashes, the cornea, the iris, and the retina.
response caused by a neurologic problem affecting the visual • Identify the conditions associated with a white pupil (ie
leukokoria).
part of the brain. This can manifest as decreased visual acu-
• Recognize the signs of and know the conditions
ity, visual field deficits, or visual processing abnormalities.
associated with congenital glaucoma.
CVI has multiple etiologic factors, all of which stem from
• Recognize the signs of, and know the conditions
brain damage occurring in the perinatal period or structural associated with, neonatal cataracts.
abnormalities in the visual processing centers of the brain. • Know the ocular findings associated with congenital
The most common cause is hypoxic-ischemic brain injury; infections.
often CVI can coexist with other neurologic disorders. Typi- • Know the benefits and complications of eye prophylaxis
cally, these children have normal findings on anatomic eye (e.g. obstructed nasolacrimal duct).

examination or an ocular condition that cannot account for • Know the causes and clinical and laboratory features of
acute neonatal infections of the eyes, including
the degree of the abnormal visual function observed. (21) ophthalmia neonatorum.
Often, visual impairment secondary to the aforemen- • Know the management and complications of acute neonatal
tioned neurologic causes is not readily apparent at birth, infections of the eyes, including ophthalmia neonatorum.
but rather signs and symptoms manifest over time. In the • Know the findings and management of the ophthalmic
absence of any structural or anatomic abnormality noted complications of eye trauma that may occur in difficult
on careful eye examination by the neonatologist or pedia- deliveries or in the "shaken baby" syndrome.
• Know the causes and management of excess tearing.
trician that would prompt sooner referral, poor visual be-
havior, persistent strabismus, or abnormal eye movements
beyond age 3 months warrants thorough evaluation by an

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12. Lee H, Khan R, O’Keefe M. Aniridia: current pathology and
References management. Acta Ophthalmol. 2008;86(7):708–715
1. Donahue SP, Baker CN; Committee on Practice and Ambulatory 13. Chan WH, Biswas S, Ashworth JL, Lloyd IC. Congenital and
Medicine, Section on Ophthalmology, American Academy of infantile cataract: aetiology and management. Eur J Pediatr. 2012;171
Pediatrics; American Association of Certified Orthoptists, American (4):625–630
Association of Pediatric Ophthalmology and Strabismus; American
14. Fierson WM; American Academy of Pediatrics Section on
Academy of Ophthalmology. Procedures for the evaluation of the
Ophthalmology; American Academy of Ophthalmology; American
visual system by pediatricians. Pediatrics. 2016;137(1)
Association for Pediatric Ophthalmology and Strabismus; American
2. Heinmiller LJ, Levin AV. Normal postnatal ocular development. In: Association of Certified Orthoptists. Screening examination of
Enzenauer RW, Levin AV, eds. The Eye in Pediatric Systemic Disease. premature infants for retinopathy of prematurity. Pediatrics. 2018;
Cham: Springer International Publishing; 2017:1–23 142(6):e20183061
3. Wan MJ, VanderVeen DK. Eye disorders in newborn infants 15. Raghuveer TS, Zackula R. Strategies to prevent severe retinopathy of
(excluding retinopathy of prematurity). Arch Dis Child Fetal Neonatal prematurity: a 2020 update and meta-analysis. NeoReviews. 2020;21
Ed. 2015;100(3):F264–F269 (4):e249–e263
4. Stein A, Kelly JP, Weiss AH. Congenital eyelid ptosis: onset and 16. Callaway NF, Ludwig CA, Blumenkranz MS, Jones JM, Fredrick DR,
prevalence of amblyopia, associations with systemic disorders, and Moshfeghi DM. Retinal and optic nerve hemorrhages in the
treatment outcomes. J Pediatr. 2014;165(4):820–4.e2 newborn infant: one-year results of the Newborn Eye Screen Test
5. Smith HB, Verity DH, Collin JR. The incidence, embryology, and Study. Ophthalmology. 2016;123(5):1043–1052
oculofacial abnormalities associated with eyelid colobomas. Eye 17. Hughes LA, May K, Talbot JF, Parsons MA. Incidence, distribution,
(Lond). 2015;29(4):492–498 and duration of birth-related retinal hemorrhages: a prospective
6. Singh S, Ali MJ. Congenital dacryocystocele: a major review. Ophthal study. J AAPOS. 2006;10(2):102–106
Plast Reconstr Surg. 2019;35(4):309–317 18. Katzman GH. Pathophysiology of neonatal subconjunctival
7. Ali MJ. Pediatric acute dacryocystitis. Ophthal Plast Reconstr Surg. hemorrhage. Clin Pediatr (Phila). 1992;31(3):149–152
2015;31(5):341–347 19. Ryabets-Lienhard A, Stewart C, Borchert M, Geffner ME. The optic
8. Sathiamoorthi S, Frank RD, Mohney BG. Spontaneous resolution nerve hypoplasia spectrum: review of the literature and clinical
and timing of intervention in congenital nasolacrimal duct guidelines. Adv Pediatr. 2016;63(1):127–146
obstruction. JAMA Ophthalmol. 2018;136(11):1281–1286 20. Kran BS, Lawrence L, Mayer DL, Heidary G. Cerebral/cortical
9. Geloneck M, Binenbaum G. Conjunctivitis of the newborn. In: visual impairment: a need to reassess current definitions of
Lambert SR, Lyons CJ, eds. Taylor and Hoyt’s Pediatric visual impairment and blindness. Semin Pediatr Neurol. 2019;31:
Ophthalmology and Strabismus. 5th ed. Philadelphia, PA: Elsevier; 25–29
2017:109–112 21. American Association of Pediatric Ophthalmology and Strabismus.
10. Weinreb RN, Grajewski AL, Papadopoulos M, Grigg J, Freedman S. Cortical visual impairment. https://aapos.org/glossary/
Childhood Glaucoma. World Glaucoma Association Consensus Series 9. cortical-visual-impairment. Accessed March 30, 2021
Amsterdam, The Netherlands: Kugler Publications; 2013 22. Subhi Y, Schmidt DC, Al-Bakri M, Bach-Holm D, Kessel L.
11. Bhandari R, Ferri S, Whittaker B, Liu M, Lazzaro DR. Peters Diagnostic test accuracy of the red reflex test for ocular pathology in
anomaly: review of the literature. Cornea. 2011;30(8):939–944 infants: a meta-analysis. JAMA Ophthalmol. 2021;139(1):33–40

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Ocular Disorders in the Newborn
Lauren C. Mehner and Jasleen K. Singh
NeoReviews 2021;22;e461
DOI: 10.1542/neo.22-7-e461

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Ocular Disorders in the Newborn
Lauren C. Mehner and Jasleen K. Singh
NeoReviews 2021;22;e461
DOI: 10.1542/neo.22-7-e461

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
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