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Cortical Visual Impairment

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DOI: 10.1542/pir.30-11-e81 · Source: PubMed

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 Cortical Visual Impairment
Luis H. Ospina
Pediatrics in Review 2009;30;e81
DOI: 10.1542/pir.30-11-e81

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 Article ophthalmology

Cortical Visual Impairment

Luis H. Ospina, MD*
Objectives After completing this article, readers should be able to:

1. Recognize cortical visual impairment (CVI) as an important cause of pediatric visual

Author Disclosure loss.
Dr Ospina has 2. Identify the most frequent causes of pediatric CVI and, more specifically, understand
disclosed no financial the role of hypoxia as a cause of central visual damage.
relationships relevant 3. Describe the different patterns and implications for future vision of hypoxic central
to this article. This visual damage occurring in preterm compared with term babies.
commentary does not 4. Know the clinical signs suggestive of CVI in children.
contain a discussion 5. Discuss the involvement of visual functions other than visual acuity (ie, cognitive
of an unapproved/ visual aspects) in CVI and correlate them with the likely affected neuroanatomic
investigative use of a substrates.
commercial product/ 6. Explain the value of using a multidisciplinary approach in rehabilitating children who
device. have CVI early in their development.

Definition
Vision loss caused by central nervous system damage often is referred to as CVI but also
may be referred to as cerebral visual impairment or neurologic visual impairment. These
terms refer to the fact that the primary defect may not necessarily be limited to the striate
(primary visual) cortex and may affect other areas subserving vision, such as the visual
associative cortex, optic radiations, and visual attention pathways. Although it is not yet
certain which of these three terms best describes the visual deficit, it is clear that use of the
label cortical blindness must be abandoned. That term not only evokes negative connota-
tions for the child and the family but is inaccurate because in almost every instance, some
degree of residual vision remains, and visual improvement can occur.

Epidemiology
CVI has become the greatest cause of pediatric visual impairment in developed countries
(1)(2)(3)(4)(5) and is becoming increasingly prevalent in developing nations. CVI is a
problem of increasing frequency for two primary reasons. First, the progress in neonatal
care has resulted in improved survival of children who have brain damage from hypoxia. As
a result, these children may suffer from CVI. (6)(7)(8) Second, the better outcomes
associated with improved treatment of other causes of pediatric vision loss such as
retinopathy of prematurity and congenital cataracts have diminished their roles as causes of
pediatric blindness. (9)
Various studies from the developed world illustrate the
preeminent place of CVI among the causes of pediatric visual
damage. Comparing current findings with previous preva-
Abbreviations lence studies, a population-based prospective study in five
Nordic countries identified an increase from 11% to 23% in
CT: computed tomography
the relative frequency of CVI as a cause of pediatric blind-
CVI: cortical visual impairment
ness. (7) Along with optic atrophy, CVI accounted for 45%
MRI: magnetic resonance imaging
of cases of pediatric blindness in this study. In a recent survey
PVL: periventricular leukomalacia
of blind children in Ireland, the most common morphologic
VEP: visual evoked potentials
diagnoses were optic atrophy, optic nerve hypoplasia, and

*Assistant Professor, Pediatric Ophthalmology and Neuro-ophthalmology, Ste-Justine Hospital, University de Montreal,
Montreal, Quebec, Canada.

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ophthalmology cortical visual impairment

cortical blindness. (4) The Blind Babies Foundation of

Northern California found that CVI was the leading
cause of visual impairment in children younger than 5
years of age. (10)

Causes and Pathogenesis

As recently emphasized by Hoyt, (5) understanding the
mechanisms involved in pediatric CVI requires consider-
ation that the pathologic processes may affect not only
the primary visual cortex but also the associative areas,
optic radiations, optic nerves, and even visual attention
pathways.
Hypoxic-ischemic brain injury is, by far, the most
common cause of pediatric CVI. Matsuba and Jan (11)
recently reported that hypoxia was the cause of CVI in
151 of 423 (35.7%) children diagnosed with CVI. Other
authors have described similar figures in smaller series.
(2)(12) The resulting pattern of injury is, to a large
extent, defined by the age at which the insult occurs and
differs in term and preterm children. (13)(14)(15) In
term infants, the areas between circulation of the anterior Figure 1. Infused computed tomography scan of a 5-week-
and middle cerebral arteries and the medial and posterior old patient who has group B Streptococcus meningitis shows
cerebral arteries most typically are affected (watershed severe bifrontal, bioccipital, and left temporoparietal cortical
zones of the cerebral cortex). The loss of vascular flow and white matter hypodensity; bifrontal subdural collections;
autoregulation induced by hypoxia leads to hypoperfu- and enlarged ventricles. Two years later, the visual behavior
was very poor.
sion of the watershed territories, resulting in infarction of
the frontal and parieto-occipital areas (parasagittal re-
gions). The striate cortex is affected frequently but not plains the visual involvement, the pathogenesis appears
exclusively. More anterior structures, such as the associa- to be much more complex. Circuits formed by these
tive occipital visual areas and temporal and parietal cor- subplate neurons are necessary for the establishment and
tices, commonly also are involved. (5) later maturation of visual connections between the thal-
Unlike term infants, preterm babies rarely suffer para- amus and the cortex. (23)
sagittal infarctions from hypoxia-ischemia. The periven- In the past, infections (Fig. 1) and hydrocephalus
tricular deep white matter, where the germinal matrix is were the most common causes of CVI. (24) In more
located, is involved when the insult occurs earlier, be- recent series, infections account for 11.8% to 15% of cases
tween 24 and 34 weeks of gestation. (16)(17) There is a of CVI. (2)(5)(11)(12) The occipital cortex is more
transient, susceptible watershed zone in the periventricu- susceptible to damage produced by Haemophilus influ-
lar white matter that later is replaced with the adult enzae, the most common organism causing CVI.
vascular configuration. (18) Capillaries in this region are (25)(26)(27)(28)(29) Pneumococci and meningococci
prone to hemorrhage from hypoxia-ischemia. (16)(18) are other causative bacteria. (28)(30) Neonatal herpes
The characteristic injury is termed periventricular leu- simplex virus also causes ocular and cerebral visual prob-
komalacia (PVL). The germinal matrix produces glial lems. (31) The onset of visual impairment typically is late
and neuronal cells that migrate eccentrically to populate in the course of the infection, (1)(29) and multiple
the cerebrum. Immature oligodendrocytes and subplate accompanying neurologic sequelae commonly occur.
neurons present around the ventricles at that moment are (32) The different mechanisms by which infection might
more vulnerable to ischemia than mature oligodendro- injure the brain include thrombophlebitis, arterial occlu-
cytes located elsewhere, which explains the specific loca- sion, hypoxic-ischemic damage, venous sinus thrombo-
tion of the damage, believed to result from free radicals sis, and hydrocephalus. (26)(27)(29)(33)
and decreased antioxidation. (19)(20)(21)(22) Hydrocephalus, which has an incidence of about 1 in
The optic radiations run precisely around the ventri- 1,000 during the first postnatal year, (34) can affect
cles, and although this anatomic location partially ex- vision by causing optic atrophy through various mecha-

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 ophthalmology cortical visual impairment

(approximately 4% of cases in two

studies). (2)(12) The damage may
be transient or permanent. Shaken
baby syndrome is a common cause
of posttraumatic CVI. Fifty percent
of the head injuries in a study pub-
lished by Groenveld and colleagues
(3) were the result of battering.
Transient vision loss in children
may occur after trivial injuries and
typically is accompanied by head-
ache, confusion, drowsiness, vom-
iting, and seizures. (39) A link with
migraine has been mentioned by
different authors, and this condi-
tion may be underdiagnosed be-
cause of the inability of young chil-
dren to communicate their
symptoms. (40) The pathophysiol-
ogy is unknown, but an abnormal
vascular response may result in va-
sospasm, ischemia, and edema.
Figure 2. Imaging results for a 10-month-old infant who has significant epileptic activity (39)(40)(41)
and poor vision. A. T2-weighted magnetic resonance imaging shows cortical dysplasia and Epilepsy and especially infantile
polymicrogyria more evident on the left hemisphere. B. Normal right hemisphere spasms can cause central visual in-
metabolism is evident on positron emission tomography-computed tomography scan. C. attention (Fig. 2). Castano and as-
The hypometabolic left hemisphere is more evident on the temporal and occipital lobes.
sociates (42) found some degree of
visual improvement in only five of
nisms, but it also can also affect the posterior visual ten children who had severe visual inattention due to
pathways that run close to the lateral ventricles. Further- infantile spasms. Despite improvements at last follow-up,
more, a combination of anterior and posterior visual all of the patients retained significant visual impairment.
involvement is frequent. A recent population-based Due to the severity of the visual and neurologic impair-
study in Sweden revealed that due to an increase in the ment, an objective measurement of visual acuity was
incidence of posthemorrhagic hydrocephalus in very pre- possible in only one of the ten patients. Visual improve-
term children, a previously decreasing incidence of hy- ment was judged on the children’s abilities to fixate and
drocephalus no longer is observed. Eighty percent of this follow. The precise cause of visual impairment in patients
population had ophthalmologic abnormalities such as who have infantile spasms is unknown, but it is likely
strabismus, optic atrophy, and refractive errors, and 33% the same mechanism that results in seizures and abnor-
had visual impairment (visual acuity ⬍0.3). (35) Houlis- mal findings on electroencephalography. (39)(40)(41)
ton and associates (36) found that more than 50% of Anticonvulsants are known to cause visual problems as
children who had CVI from hydrocephalus had higher well. (43)
cognitive visual deficits (“problems understanding and Congenital brain malformations (lissencephaly, schi-
interpreting the visual world”). (17) zencephaly, holoprosencephaly) also may be associated
Although ventricular dilatation can occlude the pos- with CVI. (44) Metabolic and neurodegenerative dis-
terior cerebral arteries, (37) chronic distention of the ease, (32) hypoglycemia, (45) hemodialysis, (46) cere-
posterior cortex is a more frequent mechanism by which brovascular accidents, and brain tumors (13) are among
hydrocephalus causes CVI. It is well known that shunt other reported causes of CVI.
malfunction can cause CVI, but paradoxically, rapid cor-
rection by shunting also occasionally can produce CVI. Diagnosis and Evaluation
(38) The diagnosis of CVI remains essentially a clinical one.
Head trauma is a significant cause of pediatric CVI (47) The possibility of CVI should be raised when there

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ophthalmology cortical visual impairment

is greater delay in visual development than in other areas who are unable to gaze directly because of eye or head
and when the degree of vision loss is unexplained by movement problems, which are not uncommon in CVI.
ocular findings. (48) Such a situation, paired with a (55) It is possible to attempt to determine whether
characteristic clinical history such as hypoxia, should alert fixation or following capacity exists, but the inherent
the clinician to the presence of CVI. In a typical case of fluctuation of vision cannot be ignored in children whose
CVI, results of the eye examination are normal, although vision can be influenced by fatigue, light conditions, or
this is not always the case. Concomitant ophthalmologic an unfamiliar environment. Therefore, obtaining an idea
problems such as optic atrophy, nystagmus, and other of a child’s daily visual behavior is more important than
conditions very frequently accompany CVI. This situa- assigning a numeric value to the visual acuity. Comments
tion is exemplified by the preterm baby who is treated from the family as to how the child functions in his or her
successfully for retinopathy of prematurity, but whose environment can be very useful.
vision remains deficient and insufficiently explained by
the ocular findings. In such a case, coexistence of some
degree of CVI, often from PVL, should be considered. Associated Neurologic and Ophthalmologic
Habits that represent adaptations to the disease fre- Abnormalities
quently are present in patients who have CVI. Most patients who have CVI have associated neurologic
(1)(49)(50) Such habits are appropriately termed “neu- deficits. Khetpal and Donahue (12) found that 65.3% of
robehavioral” signs and may aid in the diagnosis. To a children who had CVI had neurologic deficits. Whiting
large extent, many of these signs were identified and and associates (48) found that all of the patients in their
study had some degree of neurologic deficit, and Wong
described by James Jan, a pediatric neurologist who has
(56) found deficits present in all congenital cases in her
contributed enormously to the understanding of CVI in
series. Epilepsy, cerebral palsy, hemiparesis, microceph-
children. Light-gazing, a tendency to stare at bright light
aly, hydrocephalus, hearing problems, abnormal mental
(eg, the sun or fluorescent lights), is frequent even in
development, behavioral problems, myelomeningocele,
children who do not otherwise fixate. (51) Flicking the
progressive degenerative disorders, and hypotonia are
fingers in front of the eyes against the light for self-
among the reported anomalies, indicating that the dam-
stimulation sometimes is seen. (52) Enigmatically, pho-
age may not be limited to the visual pathways.
tophobia can be prominent and sometimes even coexist
(2)(11)(12)(48)(56)
with light-gazing. (53) The explanation for this phe-
Associated ocular findings, such as optic atrophy,
nomenon is unknown, but thalamic or cortical damage
nystagmus, strabismus, gaze palsy, and retinal disease,
may be responsible. also are common. (2)(5)(11)(12)(48)(56) Although the
A highly variable visual performance also is character- absence of nystagmus in a case of congenital visual inat-
istic, with such variation even seen from hour to hour. tention suggests a cerebral cause, it is now known that
(48) Children who have very limited vision may appear the presence of nystagmus should not dissuade the clini-
unexpectedly very responsive to color stimulation. (52) cian from diagnosing CVI in the appropriate setting.
Such a response possibly is due to the bilateral cerebral Nystagmus may result from concomitant anterior path-
representation of color, which is less likely to be elimi- way disease, such as optic nerve or retinal disease, or it
nated unless the lesions are very extensive. Head shaking may indicate subcortical rather than cortical damage.
often is present, (54) as is pronounced head turns to (57) In fact, nystagmus is common in PVL. (58) When
search for objects, conceivably to make use of residual optic atrophy coexists with CVI, clinical judgment is
peripheral vision. (52) Visual function also appears to be needed to define the relative contribution of each factor
better in more familiar environments. (48) to the visual limitation.
Obtaining a measurement of visual acuity can be Consideration of both the clinical presentation and
challenging in a child who suffers from accompanying neuroimaging findings often is sufficient to diagnose
neurologic deficits. If optotypes (standardized tables to CVI. Nonetheless, certain entities should be included in
test visual acuity) prove useless, preferential looking can the differential diagnosis for this disorder. The lack of
be attempted (ie, Teller Acuity Cards, in which high- interest characteristic of autism, the inability to generate
contrast gratings of different spatial frequencies are saccades (fast eye movements) in a child who has oculo-
shown beside blank areas on the same card). The child motor apraxia, or simply a delay in visual maturation may
usually prefers to fixate on the pattern of such cards, if simulate CVI, but in these cases, the diagnosis of CVI is
seen. Even this method has limitations in testing patients inappropriate.

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 ophthalmology cortical visual impairment

reduced volume of the periven-

tricular white matter; the changes
occur to a greater degree in the
posterior aspect. (16)(62)(63) The
severity of PVL has prognostic im-
plications; a more severe PVL cor-
relates with poorer future vision.
(48)(64)(65) Lambert and associ-
ates (59) also found a positive cor-
relation between the degree of in-
volvement of the optic radiation on
CT scan and MRI and a poor visual
outcome. However, they found no
correlation between visual recovery
and changes in the striate and
parastriate cortices. Casteels and as-
sociates (66) obtained similar re-
sults and described the myelination
Figure 3. T2-weighted magnetic resonance imaging for an 11-year-old patient who has pattern as useful for predicting vi-
limited vision and a history of prematurity and intraventricular hemorrhage demonstrates sual outcome.
white matter atrophy and signal abnormality (arrows) around the ventricles at the
As stated, hypoxia in term in-
occipital lobes. Compensatory enlargement of the occipital horns has resulted from
fants tends to cause frontal and
periventricular leukomalacia.
parieto-occipital infarctions. MRI
later reveals cortical thinning and
Neuroimaging decreased underlying white matter, ex vacuo dilatation of
In many circumstances, neuroimaging can support or the ventricles, ulegyria (misshapen, narrow gyri), and
confirm the diagnosis of CVI, and imaging can aid in wedge-shaped infarctions. (67)
estimating the final visual outcome. The abnormalities The location of lesions in cases of profound hypoxia
seen on computed tomography (CT) scan and magnetic from cardiac arrest and hypotension may vary with ges-
resonance imaging (MRI) for a child who has CVI are tational age. In preterm infants, the brainstem, cerebel-
diverse and range from normal to substantially altered lum, and thalami are predominantly injured, often along
anatomy of the posterior visual pathways, depending on with white matter damage. In babies born at term, the
the cause, severity, and moment of the insult. Globalized
lateral thalami, posterior putamina (Fig. 4), hippocam-
cortical atrophy, ischemic encephalopathy, PVL, and
pus, and corticospinal tracts are affected, often along
structural malformations were the most common find-
with damage to the lateral geniculate bodies and optic
ings in a study by Khetpal and Donahue. (12) Diffuse
radiations. Due to limited survivability, these patterns are
cerebral atrophy was the most prevalent CT scan abnor-
seen less frequently. (68)
mality in a study by Lambert and associates. (59)
Given the importance of neonatal hypoxia as a cause Despite the great value of MRI, in some cases, poor
of CVI, imaging findings in this condition require dis- vision does not correlate with damage seen on imaging of
cussion. First, the value of ultrasonography in evaluating the optic radiations or the primary and associative visual
preterm babies in the neonatal care unit deserves men- cortex. In these cases, it may be beneficial to use imaging
tion. This technique detects intraventricular and germi- to evaluate the damage to areas such as the frontal cortex,
nal layer hemorrhage, hemorrhagic parenchymal infarc- thalamus, inferior parietal cortex, superior colliculus, and
tions, and cystic changes. (16) The presence of cystic pulvinar, which constitute the anatomic substrate for
leukomalacia on ultrasonographic examination was visual attention and may play a role in the pathogenesis of
found to be highly predictive of CVI. (60)(61) CVI. (69)
In older children who were born preterm, MRI is the Although it is not normally necessary, functional neu-
most sensitive test for establishing damage to the roimaging (Fig. 3) may complement MRI in studying
periventricular white matter (Fig. 3). PVL is character- CVI. Silverman and colleagues (70) found large areas of
ized by atrophic dilatation of the lateral ventricles and decreased cerebral blood flow using single-photon emis-

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ophthalmology cortical visual impairment

results determined during the period of bad vision were

useful in predicting a negative outcome. Kupersmith and
Nelson (76) reported that the visually evoked response
measured promptly after the insult is useful in predicting
recovery. Good and Hou (77) found sweep VEP valu-
able in measuring visual acuity of children who had CVI
using grate images as the visual stimulus.
VEP appear to be a useful supplemental tool, but they
have limitations, and clinicians should not to rush to
predict a poor or good outcome solely on the basis of
these findings. The procedure evaluates the integrity of
the visual pathway but has limitations in accessing higher
aspects of vision.

Visual Improvement
Some improvement of vision virtually always occurs in
children who have CVI. The mechanism by which im-
provement occurs continues to be a topic of interesting
debate. In many cases, the likely explanation may be the
Figure 4. Head computed tomography scan for an 11-month- presence of residual visual potential, which gradually
old child who had prior cardiac arrest shows diffuse severe improves over time. (78) Improvement also may result
bifrontal and temporal cortical atrophy as well as lacunar from the sparing of some areas rather than total destruc-
infarcts in the basal ganglia (arrows). The occipital lobes are tion of visual cortical function at the time of injury. (48)
spared. Nonetheless, the fascinating changes observed in an-
imals make it difficult to ignore the possibility that more
sion tomography in patients who had CVI in whom MRI sophisticated responses to early damage of central vision
had revealed no abnormalities. may occur in humans. Some studies strongly support this
hypothesis. Research in cats whose visual cortex (areas
Visual Evoked Potentials (VEP) 17, 18, 19) is ablated is particularly worthy of mention.
The role of VEP in confirming the diagnosis of CVI in The results could be summarized as anatomic and phys-
children and predicting visual outcome has been ad- iologic. After cortical lesions are produced, anterograde
dressed in many studies, and the subject is not free of and retrograde tracing methods reveal a response that
controversy. Different technical methods, such as “flash” consists of increased anatomic projections from the ret-
and “sweep,” are used to record potentials. Frank and
ina through the thalamus to the posteromedial lateral
Torres (71) did not find VEP valuable in diagnosing CVI
suprasylvian extrastriate visual area in neonatal but not in
because they were unable to identify differences in re-
adult cats. Also, the young cats, unlike the adult ones,
sponse between 30 children who had cortical loss of
demonstrate a functional compensation after cortical
vision and 31 who had neurologic lesions and no visual
ablation. Single-cell neurophysiologic responses mea-
involvement. Clarke and associates (72) found a low
positive predictive value (45.1%) for flash VEP because sured at least 6 months later in the posteromedial lateral
14 of 31 children who had abnormal responses remained suprasylvian cortex achieve normality. (79)(80) Al-
impaired and low specificity (39.3%) because only 11 of though the cells in the latter area do not acquire the
28 infants who showed improvement had had normal properties of the superior striate cortex, they do develop
results on VEP. Other authors have demonstrated that a receptive field properties. (81) Similarly, young monkeys
bad VEP result does not necessarily mean a poor future whose striate cortex is damaged, unlike older ones, dem-
visual outcome. (73)(74) onstrate a capacity to respond to stimuli in the hemifield
Nevertheless, other investigators have reported better opposite the ablation as well as the ability to localize
utility of VEP in the evaluation of pediatric CVI. Taylor those stimuli with eye movements 2 to 5 years after the
and McCulloch (75) reported that abnormal flash VEP injury. (82)

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 ophthalmology cortical visual impairment

Beyond the Striate Cortex optic radiation correlated with a poor visual prognosis.
As mentioned, children afflicted with CVI seldom Other causes of CVI such as bacterial meningitis and
present with isolated lesions of the striate cortex. The epilepsy have been associated in the past with a poorer
associative areas of the occipital cortex or temporal or outcome. (44)(56) However, factors other than cause
parietal cortices frequently are affected, sometimes pre- (eg, timing of the insult, severity) must be considered for
dominantly. (69) Aside from abnormal visual acuity, each case. For example, Huo and associates (2) did not
many children who have CVI exhibit particular higher find a correlation between cause and prognosis.
visual deficits (cognitive visual disorders), and these def- More than 90% of children who have CVI remain
icits can occur in conjunction with preserved visual acu- visually handicapped despite some improvement of vi-
ity. (17)(83)(84)(85) Such deficits might result from sion. Their neurodevelopmental outcomes other than
lesions to the dorsal (parietal lobe, impaired ability to vision also remain poor. (11)
handle complex scenes) or the ventral (temporal lobe,
impaired recognition) stream systems. (8) Saidkasimova Rehabilitation and Management
and associates (86) published a series involving seven Early diagnosis and intervention are important in man-
children who had impaired perception of movement, aging pediatric CVI. (3)(90) Groups who have substan-
simultagnosia (one or some isolated elements of a visual tial experience in caring for affected patients have made
scene can be recognized, but not the whole complex specific suggestions concerning rehabilitation. Reducing
scene), visuomotor dysfunction, and impaired orienta- the amount of visual stimulation by presenting simple
tion. All exhibited some degree of periventricular white rather than crowded visual environments is believed to
matter anomaly. It is important to recognize that CVI enhance vision in children who have CVI. Cueing by
can involve much more than reduced visual acuity. (87) language, touch, and using contrasting colors are among
It has been suggested that evaluating the damage seen the many useful strategies for optimizing residual vision.
on neuroimaging to structures involved with mecha- A multidisciplinary approach for each child is essential.
nisms of visual attention may be of interest. A child who (3)
has poor vision potentially could have difficulties “choos- Because CVI almost is never an isolated problem, it is
ing” among multiple presented images because of lesions important to diagnose and treat any accompanying neu-
involving visual spatial attention (frontal lobe, globus rologic conditions. Pediatric neurologists, developmen-
pallidus, caudate, putamen, and thalamus) or selection of tal pediatricians, and occupational therapists can be of
stimulus for attention (inferior parietal cortex, superior help. Providing the best possible control of associated
colliculus, pulvinar) structures. This possibility has been seizures that may interfere with visual function by opti-
discussed in detail by Hoyt, (69) and it may apply not mizing their pharmacologic treatment also may alter
only to cases of CVI in which the imaging findings of the visual behavior. Facilitating access to special services for
striate and parastriate cortices and periventricular regions concomitant cerebral palsy may help. (2)
appear preserved, but also to more typical cases such as Sleep disorders affect children who have visual impair-
CVI from PVL. Recent evidence confirms that thalamic ment, resulting not only from deregulation of the effect
damage, for example, is frequent in PVL. (88)(89) that vision is known to have on the sleep-wake cycle, but
also from concomitant neurodevelopmental abnormali-
Prognosis ties. Detailed descriptions of the effects of melatonin and
Almost all children afflicted with CVI show some degree other treatment interventions are discussed elsewhere,
of visual improvement with time. Using different criteria, (91)(92)(93)(94)(95) but we can testify to the benefits
Matsuba and Jan (11) found overall improvement of of enrollment in a pediatric sleep clinic.
visual acuity in 46% of patients, and Huo and associates Only in limited circumstances is a treatment directed
(2) noted improvement in 60%. The level of vision at the specific cause of CVI. Two examples are hydro-
achieved depends on many factors, including the cause, cephalus or shunt blockage, in which the appropriate
age of onset, and severity and type of injury. (1) Children surgical intervention is therapeutic.
who have subcortical damage seem to have a worse The pediatric ophthalmologist or neuroophthalmolo-
prognosis. In a retrospective study, Hoyt (5) found some gist is expected to play a role not only in assisting with the
recovery of vision in 78% of children who had striate diagnosis of CVI but also in identifying associated ocular
cortex injury compared with 42% of children who had abnormalities and treating them when possible. For ex-
periventricular white matter involvement. Lambert and ample, a child who has CVI and significant myopia may
colleagues (59) found that imaging abnormality of the benefit from glasses. Every person involved in the care of

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ophthalmology cortical visual impairment

an affected child must remember that the early promo- 5. Hoyt CS. Visual function in the brain-damaged child. Eye.
tion of visual development results in better outcomes. 2003;17:369 –384
6. Haddad MA, Sei M, Sampaio MW, Kara-José N. Causes of
(90) Clinicians must direct the child as soon as possible
visual impairment in children: a study of 3,210 cases. J Pediatr
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Monique Grimard and Dr Alexandre de Saint-Sardos for
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their invaluable aid in preparing this article. oxidative injury to premyelinating oligodendrocytes in periven-
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 Cortical Visual Impairment
Luis H. Ospina
Pediatrics in Review 2009;30;e81
DOI: 10.1542/pir.30-11-e81

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