A. Epidemiology
- Affects 4% of children under 6 years
- 30-50% will develop secondary visual loss
- Early recognition and intervention allows binocular vision to develop
- Esotropic (deviating eye turns in) deviations most common
- Also called "Wandering Eye"
- May progress to amblyopia ("lazy eye") leading to vision loss
B. Classification
- Hetertropia is misalignment of eyes that is always manifest
- Heterophoria is latent malalignment
- Prefixes indicate the direction of deviation
- Eso: inward
- Exo: outward
- Hyper: upward
- Hypo: downward
C. Pathogenesis
- Reflects inability of the eye's fusional mechanism to control eye deviation
- Unilateral hetertropia results in:
- Preference for the undeviated eye AND
- Loss of vision of the deviated eye
- Alternating bilateral hetertropia
- Normal vision development
- Because each eye is used in turn
D. Clinical Findings
- May be asymptomatic or associated with squint
- Corneal Light Reflex Examination
- Requires minimal cooperation from the child
- Project a light source onto cornea of both eyes simultaneously
- If strabismus is present, the light will fall on different parts of each cornea
- Cover-Uncover Test
- Requires child's cooperation
- Patient stares at a distant object
- Examiner covers one eye and looks for movements of uncovered eye
- If no movement, then there is no misalignment of the eye
- Alternate-Cover Test
- Examiner rapidly covers and uncovers one eye like a wind-shield wiper
- If ocular deviation present, the eye rapidly moves as the cover is shifted to the other eye
- Pseudostrabismus
- Child falsely appears to have ocular misalignment or squint
- Appearance exaggerated in photographs
- Seen with flat broad nasal bridge, prominent epicanthial folds, or narrow intracanthial distance
- Corneal reflex and cover tests should differentiate true strabismus
- Facial asymmetries tend to lessen with growth
- No treatment needed
E. Non-Paralytic Strabismus
- Infantile Esotropia
- Present by 6 months
- Patching undeviated eye allows normal visual development in the other eye
- Glasses needed to correct far-sightedness
- Surgical realignment optimal before 2 ears to allow binocular vision to develop
- Accommodative Esotropia
- Occurs between 6 months and 7 years
- Most common form of strabismus
- Eye accommodates to correct a hyperopic or blurred image
- With large refractive errors, the amount of convergence may lead to esotropia
- Initially intermittent but can become constant
- Spectacle correction for hyperopic refractive error
- Reduces the need for accommodation and thus the stimulus for excessive convergence
- Bifocal spectacles sometimes needed for residual deviation at near fixation
- Eye muscle surgery needed for significant residual deviation
- Intermittent Exotropia
- Onset 6 months to 4 years
- Outward deviation of one eye when child is distance focusing
- Increased frequency with fatigue or illness
- Visual acuity and binocular vision
- Surgery needed for large or increasing frequency exotropia
- Constant Exotropia
- Rarely congenital
- Associated with neurologic disease or abnormalities of the bony orbit
- Includes association with a Couzon's syndrome
- Eye-patching to prevent visual impairment in deviated eye
- Surgery if deviation cosmetically significant
F. Paralytic Strabismus
- Third Nerve Palsy
- Fourth Nerve Palsy
- Sixth Nerve Palsy
G. Third Nerve Palsy
- Usually congenital in children
- Acquired form associations
- Intracranial neoplasm or aneurysm
- Inflammatory or infectious lesion
- Head trauma
- Post-viral syndromes
- Migraines
- Presentation
- Exotropia with downward deviation and ptosis
- Pupillary dilation may be present
- Surgical correction designed to redirect nerve fibers
- Botulinum toxin can be effective
H. Fourth Nerve Palsy
- Congenital more common
- Acquired form associated with head trauma due to long intracranial course
- Weakness of superior oblique presents with hypertropia
- Head tilts to the shoulder opposite the affected eye to minimize double vision
- Eye surgery improves ocular alignment and eliminate head tilt
I. Sixth Nerve Palsy
- Congenital deficit
- Rare condition
- Associated with Duone Retraction Syndrome
- Anomalous innervation causing a congenital disorder of ocular motility
- Acquired forms
- Self-resolving form seen in children following a febrile illness, upper respiratory infection
- Associated with ICP from hydrocephallus or intracranial mass
- Presents with markedly crossed eyes
- Ability to move affected eye laterally limited
- Head turns toward the palsied muscle to maintain binocular vision
- Surgical correction attempted
References
- Donahue SP. 2007. NEJM. 356(10):1040
- Simon JW and Kaw P. 2001. Am Fam Phys. 64(4):623