Strabismus: Kammi B. Gunton,, Barry N. Wasserman,, Caroline Debenedictis

Strabismus
Kammi B. Gunton, MD*, Barry N. Wasserman, MD,

Caroline DeBenedictis, MD
KEYWORDS
Strabismus Esotropia Exotropia Cranial nerve palsies
KEY POINTS
Defining the type of strabismus creates a framework for work-up and management.
Comitant esotropia is most commonly a childhood condition treated with glasses and
surgery.
Comitant exotropia is often a childhood condition that does require surgical correction.
Microvascular disease is the most common cause of ocular cranial nerve palsies in adult
patients.
INTRODUCTION
Patients with misaligned eyes often first present to a primary care clinician for evalu-
ation. The misalignment may be intermittently present or subtle in character, making
evaluation difficult. Strabismus is any misalignment of the visual axes and may be
referred to as squint. A basic understanding of varying types of strabismus allows bet-
ter communication with patients and parents as well as direct referral and treatment.
Although strabismus may be referred to as lazy eye, the term is used in multiple dis-
eases, such as amblyopia (decreased vision in an eye without signs of physical defect
or pathology), ptosis, and strabismus. Strabismus affects 1% to 3% of children. It is
seen more commonly in children with a history of prematurity; systemic diseases,
such as cerebral palsy; genetic syndromes; and a family history of strabismus.1
Parents are often convinced that their child has a single eye that is weak, but most
commonly 1 eye is simply the dominant, or fixating, eye. If the deviated eye is forced to
fixate by covering the dominant eye momentarily, however, then the deviation seems
to switch eyes. This demonstrates that the neuromuscular imbalance is between the
eyes and is usually not limited to 1 eye or the other. In patients with palsy of a cranial
nerve or 1 muscle, the dominant eye continues to fixate even if it has the effected
muscle.2
Department of Pediatric Ophthalmology, Wills Eye Hospital, 840 Walnut Street, Suite 1210,
Philadelphia, PA, USA
* Corresponding author.
E-mail address: [email protected]
Prim Care Clin Office Pract 42 (2015) 393–407

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394 Gunton et al
The most common form of strabismus is in the horizontal axis. An eye crossed rela-
tive to the other is called an esotropia, whereas an outward drift of 1 eye is called an
exotropia. In vertical deviations, the effected eye must be specified, because a left
hypertropia is the same as a right hypotropia. More complex forms of strabismus
have differing misalignment in various gazes. Deviations are further defined by their
comitance. Comitant deviations are the same in all directions of gaze, and incomitant
deviations vary depending on the gaze. Nevertheless, the basic approach to stra-
bismus includes defining the type of strabismus, recognizing patterns of misalign-
ment, and applying the appropriate work-up/treatment. This review covers the basic
types of comitant esotropia and exotropia and misalignments seen in cranial nerve
palsies. Special syndromes and systemic diseases that directly affect the extraocular
muscles and restrictive processes are beyond the scope of this review.
Assessment of strabismus is performed by several different techniques. Various
basic techniques are excellent for screening patients. The Hirschberg method involves
shining a beam of light at the eyes and assessing the light reflex in the pupils. If the
eyes are aligned, then the light is essentially centered within the pupil. If a child is look-
ing directly at the light, and 1 light reflex is in the center of the pupil and the other light
reflex is not, then strabismus is suspected. Another useful technique is the Bruckner
technique. With the room lights dimmed and standing a few feet from the patient, the
clinician holds the direct ophthalmoscope to his or her eye and directs the instrument
light toward the child’s face. The red reflex should be seen equally. If there is asymme-
try of the red reflex, strabismus or other ocular pathology should be suspected. If the
child is old enough to hold attention with a toy, then a cover test can be attempted.
With the toy in front of the child, 1 eye is briefly covered while observing the motion
of the uncovered eye. If the uncovered eye moves to find the toy, then strabismus
is present. If there is no strabismus, then no refixation movement of either eye should
be seen. The appropriate technique used depends on the comfort level of the clinician
and the cooperation of the patient. Ultimately, if strabismus is manifest a majority of
the time, the child loses binocularity and could become amblyopic. Adults with stra-
bismus may have disabling diplopia.
ESOTROPIA
Esotropia is a type of ocular misalignment in which the deviating eye turns in medially,
toward the nose (Fig. 1). In the first months of life, the visual and oculomotor systems
are immature and still developing. Parents may think their child’s eyes are crossed or
drifting out, but the duration of the misalignment is generally brief, and realignment is
established spontaneously. The eyes should achieve stable alignment for most
Fig. 1. Esotropia (crossing) of left eye. (Courtesy of Kammi B. Gunton, MD.)

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Strabismus 395
children by 3 months but can be delayed in children who are premature or those with
delayed visual maturation. If there is suspicion of ocular misalignment beyond
3 months of age, then referral to a pediatric ophthalmologist should be considered.
There are several types of comitant esotropia. The most common types—pseudoeso-
tropia, congenital esotropia, and accommodative esotropia—are discussed.
Pseudoesotropia
Pseudoesotropia represents a large portion of the consultations in a pediatric ophthal-
mology practice.3 The term pseudo implies a false esotropia. The ocular alignment is
normal, but the appearance of the eyes suggests crossing. Infants often have wide
nasal bridges and prominent epicanthal folds. A prominent epicanthal fold covers
the medial sclera of both eyes, making the eyes appear crossed. Referral to an
ophthalmologist to verify the alignment is appropriate. Treatment consists of an expla-
nation of the cause of the appearance of crossing with reassurance to parents that
true crossing is not present and observation because some children have pseudoeso-
tropia initially but later develop intermittent esotropia. Repeat examinations on sepa-
rate visits may reveal true strabismus.
Congenital Esotropia
Congenital esotropia is an ocular misalignment with onset in the first 6 months of life.
Any child with constant crossing of the eyes should be referred to the ophthalmologist
at presentation. Only small-angle, intermittent crossing can be observed in the first
3 months of life, because it may spontaneously resolve. In congenital esotropia, the
angle of deviation is usually large, and patients may switch the fixating eye spontane-
ously (Fig. 2). Patients typically have no significant refractive error.4,5 Several other
ocular findings may be seen in these patients. They may have latent nystagmus, a
shaking or jiggling of the eye when 1 eye is covered. They may have oblique eye mus-
cle dysfunction, which can lead to pattern esotropia. For example, a V-pattern esotro-
pia is associated with inferior oblique muscle dysfunction and has a much larger angle
of deviation in downgaze with a smaller angle of deviation in upgaze. Dissociated ver-
tical deviations also are seen in association with congenital esotropia and involve a
unilateral upward drift of the nonfixing eye. Treatment of congenital esotropia is surgi-
cal.4,6 Glasses are rarely helpful, and there is no role for eye exercises, commonly
called vision therapy.
Accommodative Esotropia
Accommodative esotropia generally occurs between 1 and 3 years of age.7,8 This type
of esotropia is associated with high hyperopic (farsighted) refractive errors. Most chil-
dren in this age group are naturally mildly hyperopic, but young people can accommo-
date or adjust their focus. They have no particular trouble with their sight for distance
or near targets. The near reflex, however, includes convergence with accommodation.
Fig. 2. Congenital esotropia (crossing) with right eye fixing on target. (Courtesy of Barry N.
Wasserman, MD.)
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396 Gunton et al
When children with accommodative esotropia with large amounts of hyperopia

accommodate, they converge and are unable to perform a fusional divergence move-
ment, which would realign the visual axis. An esotropia results (Fig. 3). Fortunately,
correction with hyperopic spectacles relaxes the accommodation and its associated
esotropia. In some patients, excessive convergence for accommodation results in
further crossing of the eyes when viewing near targets. This is referred to as a high
accommodative convergence to accommodation ratio. In these patients, a bifocal
lens further relaxes accommodation at near-target fixation and alleviates the residual
esotropia through the bifocal. Once acceptable ocular alignment is achieved in pa-
tients with accommodative esotropia, they may still require treatment of amblyopia
(poor vision in nonpreferred eye). Hyperopia classically diminishes with development
into adolescence. If treatment is delayed, residual esotropia despite spectacle correc-
tion remains. In these cases, strabismus surgery can achieve excellent ocular align-
ment.7 Early referral to an ophthalmologist for treatment with glasses can prevent
the need for surgery and amblyopia treatment.
The acute onset of comitant esotropia in older children can have a neurologic basis.
Children over 5 years old often complain of acute diplopia (double vision). In the
absence of refractive error, this type of esotropia may be associated with intracranial
pathology, including Arnold-Chiari type I malformation, pontine gliomas, and astrocy-
tomas.8 Urgent neuroimaging is indicated, as is immediate referral.
EXODEVIATIONS
An exodeviation is the outward drift of 1 eye compared with the other (Fig. 4). There
are several types of exodeviations, including pseudoexotropia, exophoria, intermittent
exotropia, constant exotropia, and convergence insufficiency. In all cases, parental
observations of the frequency of the misalignment and eye preference should be eli-
cited. A medical history of craniofacial syndromes, neurologic disorders, infections,
and trauma may predispose to exodeviations. Specific genes have not yet been iden-
tified, but there is often a family history of exotropia.
Fig. 3. Patient with accommodative esotropia. Top photo demonstrates esotropia (crossing)
of the left eye without spectacle correction. Bottom photo of same patient with aligned
eyes in glasses. (Courtesy of Barry N. Wasserman, MD.)
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Strabismus 397
Fig. 4. Exotropia (outward drift) with right eye fixing. (Courtesy of Kammi B. Gunton, MD.)
Pseudoexotropia
Pseudoexotropia occurs when the globes are properly aligned but the eyes have the
appearance of outward divergence. This can result from a wide interpupillary distance
or a positive angle kappa. Angle kappa is the angle formed between the visual and pu-
pillary axes. If the angle between these 2 axes is larger than 5 , the corneal light reflex
is nasally displaced, forming a positive angle kappa. This gives the appearance of an
exotropia. Positive angle kappa can occur in isolation or as a result of intraocular ab-
normalities, including temporal macular dragging caused by retinopathy of prematu-
rity, high myopia, or infection with macular scarring. Children with pseudoexotropia
maintain a straight head position, and there is no refixation movement with cover
testing. No treatment of the alignment is required, but amblyopia accompanying the
macular pathology must be addressed if applicable.
Exophoria
An exodeviation that is controlled by the sensory fusional mechanisms of binocular
vision is called an exophoria. Under normal viewing conditions, the eyes are in proper
alignment. If binocular fusion is disrupted, however, the exodeviation emerges. In the
absence of symptoms, no treatment is indicated. Observation is recommended
because decompensation results in progression to manifest exotropia.
Intermittent Exotropia
Intermittent exotropia is the most common form of exodeviation in childhood. The age
of onset is variable but generally occurs between 6 months and 4 years of age. The
exodeviation is intermittently present. Initially, the deviation occurs with periods of
inattention, fatigue, and stress. Parents may note intermittent squinting or children
closing 1 eye, especially in bright light. This behavior occurs to prevent diplopia
when the eye is exotropic. Bright light often disrupts fusion, allowing the exodeviation
to become manifest. Additionally, the eyes may realign with near fixation, deviating
only when the patient looks off into the distance. With progression, the deviation
may occur with near viewing. The periods of misalignment may become more frequent
and progress to constant exotropia. Patients typically have good vision in both eyes
without amblyopia and may alternate the fixing eye.
Intermittent exotropia is divided into 3 classifications. Basic exotropia is character-
ized by an exodeviation that measures the same at distance and at near. Divergence
excess is an exodeviation greater at distance than near. Lastly, convergence insuffi-
ciency exotropia is an exodeviation greater at near than at distance.
Less invasive treatments are preferred to surgical intervention if possible. Preserva-
tion of vision, binocular fusion, and proper alignment drive the treatment of exodevia-
tions. Treatment begins by correcting any underlying pathology. Significant refractive
errors should be corrected with glasses. Any amblyopia must be treated with glasses
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398 Gunton et al
and/or patching. Prisms are an option for small deviations that cause diplopia,
although they are usually reserved as a treatment in adult-onset exotropia or patients
who are not surgical candidates. Vision therapy alone has not proved to effectively
treat exotropia.9 In cases of extremely large, frequent, or constant exotropia, surgical
intervention may be undertaken. The timing of surgery is variable. Most surgeons wait
until the exotropia occurs at least 50% of the waking hours and measures greater than
14 to 16 prism diopters (PD). Surgery is undertaken earlier if the exodeviation causes
amblyopia or significantly effects binocular fusion. Surgical options include bilateral
lateral rectus recessions or unilateral recession of lateral rectus with resection of
medial rectus. Although both procedures provide good results with initial alignment,
long-term success is harder to define.10 The most common complications of stra-
bismus surgery include overcorrection, undercorrection, or recurrent strabismus after
a period of realignment.
Constant Exotropia
Constant exotropia occurs when the eyes are constantly deviated. It is more common
in older patients or in patients with decompensated intermittent exotropia, although a
congenital type exists. There are different classifications of constant exotropia.
Congenital exotropia occurs before the age of 6 months to 1 year. This is a rare form
of exotropia in healthy children. It is more common in children with neurologic disease
or craniofacial disorders. It can begin as an intermittent deviation but rapidly pro-
gresses to a constant exotropia. As in congenital esotropia, the angle of deviation in
congenital exotropia is large, usually more than 35 PDs. These children are typically
treated with early surgery to try to regain some binocularity. They should be followed
closely during the amblyogenic years to monitor the vision and new misalignments,
such as vertical deviations or esotropia.
Sensory exotropia is typically a unilateral exodeviation occurring in patients with
significantly reduced visual acuity in 1 eye. Potential causes include anisometropic
amblyopia, corneal opacities, cataracts, optic nerve hypoplasia or atrophy, and retinal
pathology. The eye with poor vision become strabismic. It can be esotropic or exo-
tropic, with exotropia occurring more commonly in older children and adults. Consec-
utive exotropia occurs after previous strabismus surgery for esotropia and recurrent
exotropia follows previous surgery for exotropia. These deviations occur in patients
with poor binocularity. Surgical treatment is required to realign the eyes, but gradual
misalignment likely recurs.
Convergence Insufficiency
Convergence insufficiency is defined as an exophoria that is greater at near than at
distance. Characteristics include asthenopia with near vision, blurred near vision,
reading problems, decreased near point of convergence, and decreased near
fusional convergence amplitudes. The options for treatment include vision therapy
exercises or prism glasses. Vision therapy is effective in convergence insuffi-
ciency.11 Prismatic correction in reading glasses may also provide relief of
symptoms.
OCULAR CRANIAL NERVE PALSIES
The 6 extraocular muscles are responsible for the coordinated motility of the eyes.
They are supplied by 3 cranial nerves. Diseases effecting any of the nerves, therefore,
result in a particular pattern of motility disturbance. Cranial nerve palsies may result in
complete or partial weakness of the corresponding muscles. In reviews of cranial
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Strabismus 399
nerve palsies, women and men seem equally effected, and 38% of patients have
associated systemic disease.12 Sixth nerve palsies are the most common of the 3, ac-
counting for 58%13 of cases; followed by third nerve palsies, 26% percent of cases;
and fourth nerve palsies, 16% percent of all cases. The motility patterns caused by
cranial nerve palsies, their causes, work-up, and finally treatment options are
reviewed.
Sixth Cranial Nerve

The sixth cranial nerve or the abducens nerve supplies the ipsilateral lateral rectus
muscle, which is responsible for abduction of the eye. Therefore, the pattern of stra-
bismus that results from palsy is an esotropia worse in gaze to the effected side
(Fig. 5). Patients often adopt a face turn away from the eye with the palsied muscle
to restore binocularity or report horizontal diplopia in side gaze depending on the com-
plete or partial nature of the palsy.
The abducens nerve originates in the pons and ascends to coordinate side gaze
with fibers in the contralateral medial rectus. Gaze palsy, therefore, results from le-
sions within the abducens nucleus. Nuclear palsies can occur from pontine infarctions
or gliomas, cerebellar tumors, and alcoholic encephalopathy. The abducens nerve
exits the pons adjacent to the V, VII, and VIII nerves. Therefore, damage to the
Fig. 5. Sixth nerve palsy right eye. Top photo reveals lack of abduction of right eye. Middle
phots shows right face turn to achieve binocularity in primary position. Bottom photo shows
normal left gaze. (Courtesy of Kammi B. Gunton, MD.)
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400 Gunton et al
fasciculus results in ipsilateral abduction weakness accompanied by ipsilateral facial

weakness and analgesia, ipsilateral Horner, and ipsilateral deafness. The cause of
fascicular palsies is most often vascular disease.
The abducens nerve then climbs along the clivus, making it vulnerable to compres-
sive lesions by basilar tumors, such as acoustic neuromas, nasopharyngeal carci-
nomas, and menigiomas. In addition, elevated intracranial pressure resulting in
downward displacement of the brainstem stretches the abducens nerve at this loca-
tion. Once the nerve pierces the dura, it travels around the inferior petrosal sinus, mak-
ing it susceptible to trauma of the temporal bone or infections of the mastoid.
Congenital lesions of the sixth nerve are rare. Etiology of sixth nerve palsies falls into
5 broad categories: idiopathic, 8% to 30%; tumors and other miscellaneous, 10% to
30%; traumatic, 3% to 30%; and vascular causes, 0% to 36%.14–17 In adults over
50 years of age, the most common cause is microvascular disease and spontaneous
resolution is common.14,18 In elderly patients, giant cell arteritis must be considered.
Without vasculopathic risk factors, neuroimaging, lumbar puncture, and blood work,
including complete blood cell count (CBC), sedimentation rate, Lyme disease, and
syphilis testing, may be indicated (Table 1).
The work-up of sixth nerve palsy depends on the age and accompanying general
health of the patient. In children, an aggressive work-up is warranted. In 1 series of
children with isolated sixth nerve palsy without papilledema or known cranial
Table 1
Work-up strategies for ocular cranial nerve palsies
Cranial Nerve Palsies Oculomotor, III Trochlear, IV Abducens, VI

Vasculopathic Pupil sparing: observe Check blood Check blood pressure,
age group, with for 5 d to rule out pressure, fasting fasting blood sugar
isolated nerve pupil involvement. blood sugar and and other parameters
palsy Check blood other parameters of diabetic control,
pressure, fasting of diabetic control, and cholesterol
blood sugar and and cholesterol levels. In
other parameters of levels. patients >65, test for
diabetic control, giant cell arteritis.
cholesterol levels. In
patients >65, test for
giant cell
arteritis.14–17
Pupil involving:
emergent MRA/
cerebral angiogram
for aneurysms18
Nonresolving, or MRI/MRA with contrast, MRI with contrast, MRI with contrast
younger patients CBC with differential, CBC with focusing on pathway
sedimentation rate, differential, tests of VI nerve and
tests for for myasthenia, increased intracranial
myasthenia14–16 fasting blood pressure, CBC with
sugar differential,
sedimentation rate,
Lyme disease
antibodies, syphilis,
thyroid testing, tests
for myasthenia gravis
Data from Refs.14–16

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Strabismus 401
pathology, tumor was found in 31% of patients.19 Any associated signs, such as
Horner, other cranial nerve involvement, nystagmus, papilledema, and contralateral
weakness, necessitate neuroimaging with MRI to assess the brainstem. In addition,
elevated intracranial pressure from any cause, including idiopathic intracranial hyper-
tension (IIH), may result in unilateral or bilateral sixth nerve palsies in as many as 60%
of cases.20 Sixth nerve palsies may also occur as postviral syndromes or in associa-
tion with multiple sclerosis.
Microvascular causes secondary to hypertension or diabetes resolve spontane-
ously over a 6- to 12-month period. Spontaneous resolution occurs in approximately
66% to 73% of patients.14,18 Traumatic causes resolve in 27% to 50% of cases.21
Lack of improvement of symptoms warrants a more aggressive work-up if microvas-
cular disease were initially suspected. Patching relieves the diplopia associated with
sixth nerve palsies. In children less than 8 years of age who are vulnerable to ambly-
opia, alternate eye patching is indicated. Prism may also relieve the symptoms in pri-
mary position, but diplopia is still likely in side gazes. Treatment is otherwise tailored to
the cause. With idiopathic etiology or nonresolving microvascular disease after
6 months, surgery to weaken the ipsilateral medial rectus, strengthen the ipsilateral
lateral rectus, or transpose the ipsilateral superior rectus or both superior and inferior
rectus to the lateral rectus may be indicated depending on the extent of the palsy.22,23
Approximately 80% of patients in 1 series achieved an acceptable range of single
vision after surgery.18
Third Cranial Nerve

The third cranial nerve controls 4 of the 6 extraocular muscles. The nerve divides when
entering into the orbit into superior and inferior divisions. The superior division controls
the superior rectus and the levator palpebrae whereas the inferior division controls the
medial rectus, inferior rectus, and inferior oblique. The pupillary constrictor fibers
travel with the inferior division as well. Therefore, complete or partial palsies of the oc-
ulomotor nerve can present with a variety of deviations. Complete palsies are more
easily recognized with complete ptosis, pupillary dilation, exotropia, and depression
of the globe (Fig. 6). Diplopia may initially be masked due to the ptosis. Pupillary dila-
tion can cause light sensitivity and poor accommodation. Incomplete palsies present
with signs and symptoms particular to the muscles effected.
The oculomotor nucleus is complex nucleus with multiple subnuclei in the brain-
stem. The fibers innervating the levator palpebrae are shared by a single midline sub-
nucleus. The fibers to the superior rectus are unique in that they cross on leaving the
nucleus. Therefore, lesions within the oculomotor nerve nucleus can lead to bilateral
ptosis and contralateral limitations in elevation. Associated tremors or spastic paraly-
sis implies a nuclear or fascicular lesion, which requires MRI to elucidate.
Within the subarachnoid space, the peripheral nerve is vulnerable to aneurysms of
the posterior communicating artery. Acute presentation of an oculomotor palsy with
pupillary involvement requires magnetic resonance angiogram (MRA)/arteriography
imaging to rule out this life-threatening aneurysm. Uncal herniation and trauma can
also effect the nerve as it pierces the dura. Pupillary fibers are effected first when palsy
occurs from a traumatic cause, because the pupillary fibers are more superficial and
located dorsal-medially within the nerve at this location.
In the cavernous sinus, the oculomotor nerve travels in the lateral dorsal wall adja-
cent to the fourth nerve. Cavernous sinus fistulas, aneurysms, tumors, pituitary
apoplexy, and infections/inflammations within the cavernous sinus can present with
accompanying palsy of any of the ocular cranial nerves.
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402 Gunton et al
Fig. 6. Third nerve palsy left eye. (A) Inability to adduct the left eye to midline. (B) Dilated
pupil with exotropia (outward drift) of left eye in primary position. (C) Normal abduction
with limited depression of left eye. (D) Same patient with eyes in primary position post–
left globe fixation surgery. (Courtesy of Kammi B. Gunton, MD.)
Within the orbit, trauma, local neoplastic processes and infections can effect either
the superior or inferior division of the third nerve. In 1 review of head injuries, approx-
imately half had some associated ocular morbidity and 11.6% of those patients had
complete or partial oculomotor nerve palsy.24 In addition, migraines may result in par-
tial oculomotor palsies. Recent studies reveal that in up to one-third of cases of
migraine, a demyelinating process or neuropathy is actually to blame for the nerve
palsy.25
The most common cause of pupillary sparing oculomotor palsy is ischemia. In pa-
tients over 50 with atherosclerotic risk factors, an isolated, pupil-sparing oculomotor
palsy is likely due to microvascular disease and neuroimaging is not required.26,27
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Strabismus 403
Ischemic damage typically resolves spontaneously within 3 to 6 months. In older

adults, testing for giant cell arteritis is indicated. A complete work-up includes a
CBC, glucose tolerance testing, sedimentation rate, blood pressure, syphilis testing,
and antinuclear antibody testing.27 In addition, close observation of the pupil is
required because evolution to pupil involvement can occur within the first 5 days of
presentation. Pupillary involvement requires emergent neuroimaging with attention
to aneurysms (MRI/MRA) and prompt referral to neurosurgeons (see Table 1).
In children, congenital causes and trauma are the most common causes of oculo-
motor palsies.27 Yet, in 1 series from Nigeria of young patients aged 20 to 29 years,
systemic disorders were found in 38% of patients presenting with cranial nerve
palsy.12 Other causes of oculomotor palsy that are not localizing include migraines;
myasthenia; postvaccination inflammation; granulomatous diseases, such as sarcoid;
meningitis from infectious causes; IIH; and multiple sclerosis.12,25,28–30
Spontaneous recovery of the third nerve can result in misdirection of the nerve fibers
into other muscles supplied by the third nerve. This is known as aberrant regeneration.
The most common misdirections are elevation of the upper eyelid with adduction or
depression of the eye, adduction of the eye on attempted downgaze, and segmental
pupillary constriction with adduction. Aberrant regeneration occurs with recovery from
trauma, aneurysm or tumors but not from microvascular insults, such as from diabetic
disease. Presence of aberrant regeneration should redirect a work-up to these other
causes.
Treatment of oculomotor palsy is directed toward the underlying causes if possible.
Six months after presentation of symptoms, strabismus surgery may be recommen-
ded if recovery is incomplete from microvascular cause. Treatment focuses on man-
aging the symptoms in primary position and incorporating the complexity of the
misalignment present. If an acceptable range of single vision cannot be achieved, oc-
clusion of 1 eye is a reasonable alternative. Possible surgical alternatives include globe
fixation procedures with various suture materials, disinsertion of the lateral rectus,
and/or superior oblique or transpositions of these muscles to allow centration of the
globe.30 Preoperative assessment of any residual function within the muscles supplied
by the oculomotor nerve also has an impact on surgical choices.
Fourth Cranial Nerve

The fourth cranial nerve, or trochlear nerve supplies the superior oblique muscle. The
superior oblique muscle’s primary function is to intort, or rotate the eye toward the
nose. This cycloduction movement occurs when the head is tilted to the side. To main-
tain stability of the visual field, the eyes perform a counter-rotation to the head. For
example, if the head tilts to the right shoulder, the right eye intorts and the left eye ex-
torts, or rotates toward the ear. The superior rectus muscle also acts as an agonist to
intort the eye. In cases of superior oblique palsy, the elevation occurring due to supe-
rior rectus activity cannot be countered by the superior oblique when intorting the eye,
and a hyperdeviation results when tilting the head toward the side of the palsied eye.
In addition, the superior oblique is a depressor and an abductor of the eye. With
palsy of the superior oblique, a hyperdeviation results. The superior oblique has the
greatest effect on the vertical position when an eye is adducted. Therefore, the degree
of diplopia in a superior oblique palsy is worse in gaze away from the eye with the pal-
sied muscle (Fig. 7).
The Parks 3-step test is used to identify which vertical muscle is the cause of a
hyperdeviation. The first step of the test is to determine the deviation with the eyes
in the primary position, which is gazing straight ahead with the head straight. A hyper-
deviation in primary position indicates a weakness in the depressors of the effected
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404 Gunton et al
Fig. 7. Fourth nerve palsy of left eye showing hyperdeviation in adducted position. (Cour-
tesy of Kammi B. Gunton, MD.)
eye or the elevators of the contralateral eye. The second step assesses which horizon-
tal gaze worsens the deviation. The vertical rectus muscles have greater effect when
an eye is abducted, and the obliques have greater vertical effect when an eye is
adducted. The third step uses head tilt to determine if the deviation is worse with intor-
sion or extorsion. The results from the 3 steps yield the causative palsied muscle.
Although the 3-step test is useful, it cannot be used in cases of restrictive processes
effecting the muscles or multiple muscle palsies.31
Vertical and torsional diplopia may result from fourth cranial nerve palsies. Patients
with congenital causes have intermittent symptoms or diplopia only in certain gazes
due to adaptive vergences, whereas patients with acquired causes usually have sud-
den onset of constant diplopia. The ability to fuse large vertical deviations is highly
suggestive of a congenital cause. Most patients also adapt a head tilt away from
the side of the palsied muscle, because the vertical deviation is smaller and more
easily controlled in this position. Old photographs showing the head tilt can support
the long-standing nature of congenital palsies.
Acquired lesions are more often due to lesions along the course of the trochlear
nerve and less frequently within the superior oblique muscle or tendon.32,33 Causes
include trauma, neoplasm, ischemia, increased intracranial pressure, aneurysm, men-
ingitis, and idiopathic. The fourth nerve also originates in the brainstem and decus-
sates on exiting. Vascular disease, trauma, and demyelinated processes may cause
injury within the brainstem resulting in contralateral superior oblique palsy, but often
accompanying symptoms occur from adjacent structures, such as the descending
sympathetic fibers, which can cause a Horner syndrome ipsilaterally.
The peripheral trochlear nerve courses around the brainstem, pierces the dura, and,
via the cavernous sinus, enters the superior orbital fissure. This long course makes it
susceptible to closed head trauma. Ischemic injury is the second most common cause
of superior oblique palsy after trauma. Inquiring about hypertension, diabetes, and
other vascular ischemic risk factors is helpful. Hydrocephalus, IIH, and tumors com-
pressing its pathway can also lead to superior oblique palsy.20 An acquired cause,
therefore, requires neuroimaging (see Table 1).
Ischemic superior oblique palsies usually spontaneously resolve within 6 months.
Treatment, therefore, requires supportive measures until resolution. These measures
include patching 1 eye, prisms within glasses to alleviate some of the symptoms, or, in
cases without resolution, surgery to balance the remaining weakness. Surgical
choices are guided by Knapp guidelines, which suggest surgery on the muscle acting
when the deviation is at its greatest. These muscles include the ipsilateral superior
oblique, inferior oblique, and superior rectus or the contralateral inferior rectus. Devi-
ations greater than 15 PDs or 7 of vertical deviation generally require 2-muscle sur-
gery.34,35 Torsion can be best addressed with surgery on the superior oblique
reservados.
Strabismus 405
muscle.36 Surgical success with fusion in primary position after surgical correction is
greater when the preoperative deviation is smaller than 15 PDs.37–39 Success rates
vary from 60% to 65% when the deviation is larger.34
SUMMARY
With a systematic approach to the strabismus patient, an appropriate work-up and

treatment plan may be instituted. Recognizing the type of misalignment and identifying
any incomitance narrows the differential substantially. Treatment options differ by the
cause and type of misalignment, and success rates also vary depending on the binoc-
ularity of the patient. Nevertheless, relieving the symptoms of asthenopia and diplopia
in patients is life altering for many.
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Strabismus

Kammi B. Gunton, MD*, Barry N. Wasserman, MD,

Prim Care Clin Office Pract 42 (2015) 393–407

Fig. 1. Esotropia (crossing) of left eye. (Courtesy of Kammi B. Gunton, MD.)

When children with accommodative esotropia with large amounts of hyperopia

OCULAR CRANIAL NERVE PALSIES

Sixth Cranial Nerve

fasciculus results in ipsilateral abduction weakness accompanied by ipsilateral facial

Cranial Nerve Palsies Oculomotor, III Trochlear, IV Abducens, VI

Data from Refs.14–16

Third Cranial Nerve

Ischemic damage typically resolves spontaneously within 3 to 6 months. In older

Fourth Cranial Nerve

With a systematic approach to the strabismus patient, an appropriate work-up and

1. McKean-Cowdin R, Cotter SA, Traczy-Hornoch K, et al. Prevalence of amblyopia

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