Nelson Textbook of Pediatrics, 21st Edition 2020 2

heterophoria
is found in normal individuals; it is usually asymptomatic.

Heterotropia is a misalignment of the eyes that is constant. It occurs because
of an inability of the fusional mechanism to control the deviation. Tropias may
be unilateral or may alternate between either eye, depending on the patient. In an
alternating tropia, there is no preference for fixation of either eye, and both eyes
drift with equal frequency. Because each eye is used periodically, vision usually
develops normally. A unilateral tropia is a more serious situation because only 1
eye is constantly misaligned. The undeviated eye becomes the preferred eye,
resulting in loss of vision or amblyopia of the deviated eye.
It is common in ocular misalignments to describe the type of deviation. This
helps to make decisions on the cause and treatment of the strabismus. The
prefixes eso-, exo-, hyper-, and hypo- are added to the terms phoria and tropia to
further delineate the type of strabismus. Esophorias and esotropias are inward or
convergent deviations of the eyes, commonly known as crossed eyes.
Exophorias and exotropias are divergent or outward-facing eye deviations,
walleyed being the lay term. Hyperdeviations and hypodeviations designate
upward or downward, respectively, deviations of an eye. In cases of unilateral
strabismus, the deviating eye is often part of the description of the misalignment
(left esotropia).
Diagnosis
Many techniques are used to assess ocular alignment and movement of the eyes
to aid in diagnosing strabismic disorders. In a child with strabismus or any other
ocular disorder, assessment of visual acuity is mandatory. Decreased vision in 1
eye requires evaluation for a strabismus or other ocular abnormalities, which
may be difficult to discern on a brief screening evaluation. Even strabismic
deviations of only a few degrees in magnitude, too small to be evident by gross
inspection, may lead to amblyopia and significant vision loss.
Corneal light reflex tests are perhaps the most rapid and easily performed
diagnostic tests for strabismus. They are particularly useful in children who are
uncooperative and in those who have poor ocular fixation. To perform the
Hirschberg corneal reflex test, the examiner projects a light source onto the
cornea of both eyes simultaneously as a child looks directly at the light.
Comparison should then be made of the placement of the corneal light reflex in
each eye. In straight eyes, the light reflection appears symmetric and, because of
the relationship between the cornea and the macula, slightly nasal to the center
of each pupil. If strabismus is present, the reflected light is asymmetric and
appears displaced in 1 eye. The Krimsky method of the corneal reflex test uses
prisms placed over 1 or both eyes to align the light reflections. The amount of
prism needed to align the reflections is used to measure the degree of deviation.
Although it is a useful screening test, corneal light reflex testing may not detect a
small angle or an intermittent strabismus.
Cover tests for strabismus require a child's attention and cooperation, good
eye movement capability, and reasonably good vision in each eye (Fig. 641.1 ).
If any of these are lacking, the results of these tests may not be valid. These tests
consist of the cover–uncover test and the alternate cover test. In the cover–
uncover test, a child looks at an object in the distance, preferably 6 m away. An
eye chart is commonly used for fixation in children older than 3 yr of age. For
younger children, a noise-making toy or movie helps hold their attention for the
test. As the child looks at the distant object, the examiner covers 1 eye and
watches for movement of the uncovered eye. If no movement occurs, there is no
apparent misalignment of that eye. After 1 eye is tested, the same procedure is
repeated on the other eye. When performing the alternate cover test, the
examiner rapidly covers and uncovers each eye, shifting back and forth from one
eye to the other. If the child has an ocular deviation, the eye rapidly moves as the
cover is shifted to the other eye. Both the cover–uncover test and the alternate
cover test should be performed at both distance and near fixation. The cover–
uncover test differentiates tropias, or manifest deviations, from latent deviations,
called phorias .

FIG. 641.1 The cover test. In each instance, the occluder is placed over the right eye
while the patient is viewing a fixation target and the examiner is watching for
movement of the patient's left eye. If the left eye is not aligned, it will need to move to
look at the fixation target. If there is no movement of the left eye, the test needs to be
repeated by occluding the left eye and watching for movement of the right eye. (From
Kliegman RM, Lye PS, Bordini BJ, Toth H, Basel D, editors: Nelson pediatric symptom-
based diagnosis, Philadelphia, 2018, Elsevier, Fig. 32.6, p. 567.)
Clinical Manifestations and Treatment

The etiologic classification of strabismus is complex, and the causative types
must be distinguished; there are comitant and noncomitant forms of strabismus.
Comitant Strabismus
Comitant strabismus is the most common type of strabismus. The individual
extraocular muscles usually have no defect. The amount of deviation is constant,
or relatively constant, in the various directions of gaze.
Pseudostrabismus (pseudoesotropia) is one of the most common reasons a
pediatric ophthalmologist is asked to evaluate an infant. This condition is
characterized by the false appearance of strabismus when the visual axes are
aligned accurately. This appearance may be caused by a flat, broad nasal bridge,
prominent epicanthal folds, or a narrow interpupillary distance. The observer
may see less white sclera nasally than would be expected, and the impression is
that the eye is turned in toward the nose, especially when the child gazes to
either side. Parents frequently comment that when their child looks to the side,
the eye almost disappears from view. Pseudoesotropia can be differentiated from
a true misalignment of the eyes when the corneal light reflex is centered in both
eyes and when the cover–uncover test shows no refixation movement. Once
pseudoesotropia has been confirmed, parents can be reassured that the child will
outgrow the appearance of esotropia. As the child grows, the bridge of the nose
becomes more prominent and displaces the epicanthal folds, and the medial
sclera becomes proportional to the amount visible on the lateral aspect. It is the
appearance of crossing that the child will outgrow. Some parents of children with
pseudoesotropia erroneously believe that their child has an actual esotropia that
will resolve on its own. Because true esotropia can develop later in children with
pseudoesotropia, parents and pediatricians should be cautioned that reassessment
is required if the apparent deviation does not improve.
Esodeviations are the most common type of ocular misalignment in children
and represent >50% of all ocular deviations. Congenital esotropia is a confusing
term. Few children who are diagnosed with this disorder are actually born with
an esotropia. For this reason, infants with confirmed onset earlier than 6 mo are
typically considered to have what was previously classified as congenital
esotropia, though the term infantile esotropia is perhaps a more accurate
description.
Between 2 and 4 mo of age, many infants have infantile esotropia (neonatal
misalignments), which in most resolve spontaneously. Those that resolve
without treatment do so before 10-12 wk of age and have intermittent or variable
deviations. Those most likely to benefit from active treatment have persistent
esotropia (10 wk-6 mo of age) and constant esotropia (40 PD), in combination
with a refractive error ≤ +3.00 D, and the absence of prematurity, developmental
delay, meningitis, nystagmus, eye anomalies, and incomitant or paralytic
strabismus. The evaluation is noted in Figure 641.2 .
FIG. 641.2 Work-up of infant ≥4 mo of age with esotropia. CP , Cerebral

palsy; DS , Down syndrome; PVL , periventricular leukomalacia. (From Hoyt
CS, Taylor D, editors: Pediatric ophthalmology and strabismus, ed 4,
Philadelphia, 2013, Elsevier Saunders, Fig. 74.4, p. 767.)
The characteristic angle of infantile esodeviations is large and constant (Fig.

641.3 ). Because of the large deviation, cross-fixation is frequently encountered.
This is a condition in which the child looks to the right with the left eye and to
the left with the right eye. With cross-fixation, there is no need for the eye to turn
away from the nose (abduction) as the adducting eye is used in side gaze; this

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heterophoria

is found in normal individuals; it is usually asymptomatic.

Clinical Manifestations and Treatment

FIG. 641.2 Work-up of infant ≥4 mo of age with esotropia. CP , Cerebral

The characteristic angle of infantile esodeviations is large and constant (Fig.

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