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Ocular motility disorders and strabismus

Last updated: January 7, 2022

Summary

Strabismus is a condition characterized by misalignment of the eyes when looking at an object. One eye
deviates (either constantly or intermittently) from the normal visual axis, which results in the inability of the
brain to fuse together the images from the right and left eye. Strabismus is classified as either concomitant
(nonparalytic) or paralytic. Concomitant strabismus primarily occurs in early childhood and manifests with a
constant angle of deviation, in which the misaligned eye follows the unaffected eye. Paralytic strabismus is
frequently acquired and is due to the functional weakness of individual extraocular muscles, which alter the
angle of deviation depending on the direction of view. Further typical features include double as well as
decreased vision. Treatment at an early stage (e.g., via occlusion treatment or surgery) is essential to prevent
complications such as amblyopia (decreased vision in an eye with no apparent structural abnormality) and
loss of binocular vision. Further complex ocular motility disorders can result from central nervous
pathologies. Internuclear ophthalmoplegia, for example, is caused by a lesion of the
medial longitudinal fasciculus and causes disturbances in horizontal eye movements.

Basic terms

Strabismus: abnormal alignment of the eyes; the visual axes of the eyes are not parallel (crossed-eyes)

Heterotropia: manifest strabismus

Esotropia: inward misalignment

Exotropia: outward misalignment

Hypertropia: upward deviation of one eye

Hypotropia: downward deviation of one eye

Cyclotropia: rotation of one eye around an anterior-posterior axis

Heterophoria: latent strabismus ; presents with the same (latent) misalignments seen in
heterotropia.

Esophoria: a type of heterophoria in which one eye has a tendency to converge towards the nose
when the other eye is shut; the opposite of exophoria

Exophoria: a type of heterophoria in which one eye has a tendency to deviate away from the nose
when the other eye is shut; the opposite of esophoria

References:[1][2]

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Muscles of the eye

See extraocular muscles in eye and orbit.

Concomitant strabismus

Definition

Strabismus in which the degree of deviation (angle between the visual axes of both the eyes) remains
constant in all directions of gaze

Etiology

Genetic

Uncorrected refractive error (particularly hyperopia and anisometropia)

Unilateral visual impairment/amblyopia (e.g., organic causes, retinal disease, retinoblastoma)

Other: perinatal lesions (e.g., preterm birth, asphyxia), cerebral damage (e.g., trauma, encephalitis)

Types

Congenital or infantile concomitant strabismus

Evident at birth or onset within 6 months of age ; does not resolve spontaneously

May manifest as esotropia or exotropia

Microstrabismus: angle of deviation < 5°; therefore frequently a late diagnosis with high risk of amblyopia

Latent strabismus: usually no clinical significance; the deviation is compensated by fixation (fusion);
decompensation and manifestation occur in situations of physical stress.

Diagnostics

Hirschberg test; : a test for determining if the eyes are in alignment. A light is shone at the eyes and the
location of the light reflex on the cornea is observed in reference to the pupil. Asymmetrical corneal
reflections on examination indicate that the visual axes are not aligned (strabismus).

Cover tests (see “Cover tests” in “Examination of the eyes”

Measurement of the angle of deviation: if necessary with the help of a tangent screen

Therapeutic options
The main goals in strabismus management are to optimize visual acuity and achieve binocularity.

Correction of refractive errors

Visual training therapy

Training to correct eye movements (e.g., in strabismus) and visual-motor deficiencies

Uses specialized computer and optical devices (e.g., lenses and prisms)

Occlusion treatment

Initiate as early as possible!

Cover the unaffected eye using a patch (occlusion) → training of the weaker eye

Duration of coverage depends on the child's age

Penalization therapy (cyclopentolate drop therapy): : apply cyclopentolate drops ; to the unaffected
eye → blurs vision → encourages monocular use of the affected eye

Botulinum toxin therapy : toxin injection into the stronger muscle → temporary and partial paralysis →
weaker muscle forced to contract → long-lasting alteration in ocular alignment

Strabismus surgery

Transposition or repositioning of muscles

Tucking or advancement to tighten muscles

Myectomy or tenectomy to loosen extraocular muscles

Complications

Disturbances of binocular vision

Amblyopia

Definition: visual decrease in one or both eyes (functional visual impairment) due to a developmental
vision disorder during early childhood

Pathophysiology: one or both eyes convey poor or mismatched visual information to the brain → brain
suppresses information from one or both eyes → disuse of the eye → lacking visual stimuli with partial
underdevelopment of the visual cortex

Forms

Deprivation amblyopia (e.g., via ptosis, cataract, occlusion)

Refractive amblyopia

Strabismus amblyopia

Failure to detect or adequately treat strabismus may result in irreversible amblyopia!

A serious underlying condition (e.g., brain tumor) should be suspected in infants with
strabismus, especially in the presence of additional ocular findings like leukocoria!

References:[3][4][5][6][7][8]

Paralytic strabismus

Definition

Strabismus caused by paresis (partial failure of action) or paralysis (total failure of action) of one or more
extraocular muscles (ophthalmoplegia)

The angle of deviation alters depending on the direction of gaze (incomitant strabismus)

Etiology
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Congenital in rare cases (e.g., overaction of the inferior oblique muscles)

Clinical features Already have an account? Log in.

Diplopia (double vision): most pronounced when looking in the direction usually enabled by the paralyzed
muscle

Often compensatory head posture

Impaired extraocular muscle function

Patients with poor visual acuity may not notice diplopia. Therefore, complete optical
(refractory) correction must be achieved before testing for strabismus!

Diagnosis

Horizontal paralytic strabismus

Exotropia → medial rectus weakness → oculomotor nerve palsy

Esotropia → lateral rectus weakness → abducens nerve palsy

Vertical paralytic strabismus: perform a 3-step Park-Bielchowsky test

Steps Inference Underlying principle

Step 1: Determine Hypertropic right eye → weakness of right eye Elevators: inferior oblique,
which eye is hypertropic depressors or left eye elevators superior rectus
in primary gaze. Hypertropic left eye → weakness of right eye Depressors: inferior rectus,
elevators or left eye depressors superior oblique

Step 2: Determine Vertical strabismus increases on looking right → Superior and

whether hypertropia
increases on the right or
left gaze.
weakness of right superior rectus, right inferior rectus muscles have
inferior rectus, left superior obliquus, or left inferior their greatest vertical action
obliquus when the eye is abducted.
Vertical strabismus increases on looking left → Superior and
weakness of right superior obliquus, right inferior inferior oblique muscles have
obliquus, left superior rectus, or left inferior rectus their greatest vertical action
when the eye is adducted.

Step 3: Determine
whether hypertropia
increases on right or left
head tilt.
Vertical strabismus increases on tilting the head Intorters: superior oblique,
towards the right shoulder → weakness of a right superior rectus
eye intorter or a left eye extorter
Extorters: inferior rectus,
Vertical strabismus increases on lilting the head inferior oblique
towards the left shoulder → weakness of a left
eye intorter or right eye extorter

Weak Step 1: Which Step 2: Vertical strabismus increases Step 3: Vertical strabismus increases
extraocular eye is with lateral gaze in this directon with head-tilt towards this shoulder
muscle hypertropic?

Right Right eye Left lateral gaze Right shoulder

superior
oblique

Left Left eye Right lateral gaze Left shoulder

superior
oblique

Treatment

Correction of refractive errors

Prismatic glasses

Treatment of underlying cause

Strabismus surgery

References:[9][10]

Internuclear ophthalmoplegia (INO)

Definition:

Damage to the medial longitudinal fasciculus (the connection between the abducens nucleus, CN VI, on
one side and the oculomotor nucleus, CN III, on the other), which leads to impaired lateral gaze.

Manifests primarily with impaired adduction of the eye ipsilateral to the lesion (ipsilateral to the
medial longitudinal fasciculus lesion)

Depending on which eye is affected, INO is classified as left, right, or bilateral

Etiology

Multiple sclerosis (MS)

INO is common in individuals with MS between 20–50 years of age [11]

Typically bilateral

Hemorrhage (common cause in older patients)

Rare causes: brain tumors, chronic alcohol and recreational drug use, encephalitis, metabolic disorders

Pathophysiology

Normally, CN VI receives a signal from the ipsilateral paramedian pontine reticular formation and sends
a signal to the contralateral CN III via the medial longitudinal fasciculus.

Activation of the CN VI ipsilateral to the lesion → activation of the ipsilateral lateral rectus →
abduction of the ipsilateral eye

Activation of the CN III contralateral to the lesion → activation of the contralateral medial rectus →
adduction of the contralateral eye

Disruption of the medial longitudinal fasciculus fibers linking the CN VI ipsilateral and the CN III
contralateral to the lesion → failure of signal transmission from CN VI to CN III → the ipsilateral
lateral rectus is activated while the contralateral medial rectus is not → abduction of the ipsilateral eye,
no adduction of contralateral eye

Firing from CN VI which fails to be transmitted to CN III is instead partially transmitted to the
lateral rectus ipsilateral to the lesion → nystagmus of the ipsilateral abducting eye

Clinical findings

Adduction limited in horizontal eye movements

Adduction is retained in convergence reaction

Dissociated nystagmus: gaze to the opposite side → nystagmus of the abducted contralateral eye

In bilateral INO: possible vertical nystagmus

Remember that internuclear ophthalmoplegia (INO) is characterized by Impaired adduction of

the eye ipsilateral to the lesion and Nystagmus on the Opposite side!

References:[12]

References

1. Khaled Mohamed MohamedKoriem. Multiple sclerosis: New insights and trends. Khaled Mohamed
MohamedKoriem. 2016 .
2. Internuclear Ophthalmoplegia (INO).
https://webeye.ophth.uiowa.edu/eyeforum/atlas/pages/INO/index.htm . Updated: January 11,
2015. Accessed: June 22, 2018.

3. Fricke L. Diagnosis and management of accommodative esotropia. Clin Exp Optom. 2006; 89 (5):
p.325-331. doi: 10.1111/j.1444-0938.2006.00059.x . | Open in Read by QxMD

4. Rutstein RP. Update on accommodative esotropia. Optometry. 2008; 79 (8): p.422-431. doi:
10.1016/j.optm.2007.11.011 . | Open in Read by QxMD

5. Engle EC. Genetic basis of congenital strabismus. Arch Ophthalmol. 2007; 125 (2): p.189-195. doi:
10.1001/archopht.125.2.189 . | Open in Read by QxMD

6. Concomitant Strabismus Definition. http://www.alpfmedical.info/visual-acuity/concomitant-

strabismus-definition.html . Updated: January 21, 2017. Accessed: March 15, 2017.
7. Optometric Clinical Practice Guideline, Care of the Patient with Strabismus: Esotropia and
Exotropia. http://www.aoa.org/documents/optometrists/CPG-12.pdf . Updated: January 1, 2010.
Accessed: March 15, 2017.

8. Wright KW, Spiegel PH . Pediatric Ophthalmology and Strabismus. Springer ; 2003

9. Martinez-Thompson JM, Diehl NN, Holmes JM, Mohney BG. Incidence, types, and lifetime risk of
adult-onset strabismus. Ophthalmology. 2014; 121 (4): p.877-882. doi:
10.1016/j.ophtha.2013.10.030 . | Open in Read by QxMD

10. Karlsson VC. A Systematic Approach to Strabismus. SLACK Incorporated ; 2009

11. Helveston EM. Understanding, detecting, and managing strabismus.. Community Eye Health. 2010;
23 (72): p.12-4.

12. Babinsky E, Sreenivasan V, Candy TR. Near heterophoria in early childhood. Invest Ophthalmol Vis
Sci. 2015; 56 (2): p.1406-1415. doi: 10.1167/iovs.14-14649 . | Open in Read by QxMD
13. 1.3 Cover test FBC3E64F-CA9C-489A-ADDD-758BF5B068.

14. 1.4 Alternating Cover Test.

15. Kaplan. USMLE Step 1 Anatomy Lecture Notes 2018. Kaplan

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