Horizontal

strabismus
A review
Chantal Boisvert OD, MD, FAAO, FAAP
Pediatric Ophthalmologist
The University of New Mexico Health
Sciences Center

Ocular alignment in
infancy
Ocular deviations during the first month of life do not

necessarily indicate an abnormality;

Adequate assessment of the eye alignment is usually

not made until the patient is ~ 3 months of age;

Infants are rarely born with their eyes aligned

Nixon et al1 observed 1,219 alert infants in newborn
nursery :
40% had straight eyes
33% had exotropia (XT)
3% had esotropia (ET)
7% were not sufficiently alert to permit classification
1

Nixon RB, Helveston EM, Miller K, et al. Incidence of strabismus in neonates. Am J

Ophthalmol 1985;100:798-801.

ESOTROPIA

Definition
Convergent misalignment of the visual axis
Pseudoesotropia
Infantile esotropia
Accommodative esotropia
6th nerve palsy

Epidemiology
Occurs in 2% of American children 1-3 years

old;

3% of school-age children, adolescents and

young adults 13 to 24 years old;

No gender difference;
Most common strabismus of childhood (60%);
Amblyopia is commonly associated with

esotropia.

Pseudoesotropia
One of the most common reasons that an

ophthalmologist is asked to evaluate an infant;

Patient is orthophoric but has an appearance of

esotropia

Flat, broad nasal bridge

Prominent medial epicanthal folds
Narrow interpupillary distance
Negative angle kappa (the eye deviates nasally to

pick up fixation as the fovea is displaced toward

the optic nerve, the line of sight is not parallel
with the corneal pupillary axis)

Pseudoesotropia
Costenbader1 found that, of 753 patients

suspected by their parents to have esotropia,

47% actually had pseudoesotropia;

Can be differentiated from a true manifest

deviation by use of the corneal light reflex

and the cover-uncover test;

When pseudostrabismus has been confirmed,

parents can be reassured that the child will

outgrow the appearance of esotropia.

Costenbader FD. Infantile esotropia. Trans Am Ophthalmol Soc 1961;59:397-419.

Look at the corneal light reflex!

http://www.aapos.org/terms_faqs/faq_list/pseudostrabismus

Infantile (or congenital)

esotropia
1-2% of all strabismus;
Equal sex distribution;
Present by 6 months of age;
Family history of strabismus is common;
Incidence is higher in patients with a history of

prematurity, cerebral palsy, hydrocephalus, and

other neurological disorders;

Maternal cigarette smoking and low birthrate have

also been linked to the development of esotropia 1.

Chew E, Remaley NA, Tamboli A, et al. Risk factors for esotropia and exotropia. Arch
Ophthalmol 1994;112:1349-1355.

Infantile (or congenital)

esotropia
Physical examination:
High incidence of amblyopia (40-72%);
Often cross-fixation (viewing targets in the right field of gaze
with the left eye and vice versa)

with equal visual acuity in

each eye;
Apparent abduction deficit (pseudoparesis);
Large angle of esodeviation (usually 30 prism
diopters);
Generally unaffected by accommodation;
Cycloplegic refractions similar to those of normal
children of the same age (low hyperopia).

Infantile (or congenital)

esotropia
Associated Findings:
Dissociated vertical deviation (DVD):
Intermittent slow deviation of nonfixing eye

consisting of upward excursion, excyclotorsion

Incidence: 46-92% (mean: 70%)
Inferior oblique overaction (IOOA):
Results in elevation of the involved eye as it

moves nasally
Incidence: 78%
Nystagmus:

Latent nystagmus: becomes manifest when one

eye is occluded

Infantile (or congenital)

esotropia
Treatment:
Nonsurgical:
Correct amblyopia before surgery (patching,

atropine eye drops)

Cross-fixation suggests equal visual acuity of both
eyes
Glasses (rarely necessary)
Surgical:
Aim for alignment within 10 prism diopters of

orthophoria
Surgical alignment earlier than age 2 is associated
with better sensory binocular fusion potential

Look at the corneal light reflex!

http://www.aapos.org/terms_faqs/esotropia

Accommodative
esotropia
Convergent deviation of the eyes associated with

activation of the accommodation reflex;

Onset 6 months to 7 years (usually between ages 2 and

3);

Often, positive family history;

May be intermittent at onset;
Associated with amblyopia (generally from

anisometropia);

May be precipitated by trauma or illness;

High hyperopia (range +3.00 to +10.00; average:

+4.00D).

Accommodative
esotropia
Treatment:
Full hyperopic correction
Treat amblyopia (patching, atropine eye drops)
If residual strabismus > 10 prism diopters:

SURGERY

Look at the corneal light reflex!

http://www.aapos.org/faq_list/accommodative_estropia

6 nerve palsy
th

Incomitant esotropia (esotropia varies in different fields of

gaze);

Congenital 6th nerve palsy is uncommon:

Usually resolves spontaneously within 6 weeks;
Thought to be caused by increased ICP associated with the

birth process;

Occurs much more frequently in childhood than in infancy;

Trauma and neoplastic disorders are the most common

cause;

~ 1/3 of the cases are associated with intracranial lesions;

Spontaneous benign lesions usually resolve over several

months.

6 nerve palsy
th

Neurologic evaluation and CT or MRI

recommended;

Surgery is indicated when spontaneous

resolution does not take place after 6

months or more of follow-up and after
exclusion of intracranial lesions.

http://www.nature.com/eye/journal/v20/n12/fig_tab/6702

EXOTROPIA

Definition
Divergent misalignment of the visual axis
Pseudoexotropia
Congenital exotropia
Intermittent exotropia
Sensory exotropia
Consecutive exotropia

Epidemiology
Less common than esotropia;
Account for 25% of strabismus in children;
Exact etiology unknown;
Most common form is intermittent

exotropia.

Pseudoexotropia
Appearance of exodeviation when the eyes

are in fact the eyes are properly aligned;

May result from the following:

Positive angle kappa (the corneal light
reflection is slightly nasal to the center of the
cornea)
Macular dragging in Retinopathy of Prematurity

(ROP)
Wide interpupillary distance (hypertelorism)

Pseudoesotropia

http://www.peds.ufl.edu/divisions/genetics/teaching/facial_dysmorpholog

Pseudoexotropia
(Hypertelorism)

Congenital exotropia
Presents before age 6 months;
Large-angle constant exotropia (>35 prism

diopters);

Often associated with neurologic impairment or

craniofacial disorders;

Amblyopia uncommon
These children usually alternate fixation;
The refractive error is similar to that of the

general population;

Treatment: Surgery early in life.

http://www.nature.com/eye/journal/v23/n6/fig_tab/eye2008183f1.html

Intermittent exotropia
The most common divergent strabismus in

childhood;

Onset usually before age 5;

Deviation becomes manifest during times of

visual inattention, fatigue, or stress;

Exposure to bright light often causes a reflex

closure of one eye;

Amblyopia is uncommon.

Intermittent exotropia
Nonsurgical treatment:
Corrective lenses are prescribed for significant

refractive errors

Myopia, astigmatism, and hyperopia >+4.00D;

Additional minus lens power (overminus spectacles)

to stimulate accomodative convergence to help

control;
Alternate daily patching;
Active orthoptic treatment to improve fusional
convergence amplitudes (if convergence
insufficiency);
Base-in prisms.

Intermittent exotropia
Surgical treatment:
Surgery for increased tropic phase, poor

recovery of fusion once tropic, increasing

ease of dissociation;
Bilateral lateral rectus recession;
3 or 4 muscle surgeries for large deviation.

Straight eyes
http://www.aapos.org/terms_faqs/faq_list/exotropia

Exotropic

Sensory exotropia
Due to vision loss or long-standing poor

vision in one eye;

Children younger than 5 with unilateral

vision loss may develop ET or XT; adults and

older children usually develop XT;

Angle of deviation may be variable and

usually increases with time.

Consecutive exotropia
Exotropia that follows previous strabismus

surgery for esotropia;

Surgery depends on many factors:

Size of deviation;
Type and amount of surgery that preceded its

development;
Presence of duction limitation;
Lateral incomitance;
Level of visual acuity in each eye.

SPECIAL FORMS OF
STRABISMUS

 Duanes retraction syndrome

Mbius syndrome
Congenital fibrosis syndrome

Duanes retraction
syndrome
Usually sporadic, may be inherited (5-10% AD);
Co-contraction of medial and lateral rectus muscles

causes retraction of the globe with secondary

narrowing of the palpebral fissure in adduction;

Characteristic upshoot and downshoot (leash

phenomenon);

Bilateral in 15 to 20%;
Higher prevalence in females;
Predilection for the left eye;
Head turn common for fusion.

Duanes retraction
syndrome
Etiology:
Abnormal innervation of lateral rectus by a

branch of CN 3;
EMG shows decreased firing of lateral rectus
during abduction and paradoxical innervation
of the lateral rectus during adduction;
Exact etiology unclear
Proposed mechanisms include hypoplasia of 6 th

nerve nucleus, midbrain pathology, fibrosis of

lateral rectus.

Duanes retraction
syndrome
3 types:
Type 1 (most common; 50-80%):
Limitation of abduction
Appears esotropic (ET)

Type 2:
Limitation of adduction
Appears exotropic (XT)

Type 3:
Limitation of abduction and adductio
ET, XT, no primary position deviation

Duanes retraction
syndrome
Associations:
Deafness;
Crocodile tears;
Syndromes (Goldenhar, Klippel-Feil, Wildervanck,

cat-eye, fetal alcohol syndrome, thalidomine

toxicity).

Treatment:
Correction of refractive error;
Amblyopia treatment;
Surgery.

http://duanes.org/

Mbius syndrome
Association of both 6th and 7th nerve palsies;
Etiology unknown (current evidence points to

brainstem defects secondary to a vascular insult in

utero);

Esotropia is the most common form of strabismus seen;

Exposure keratitis from poor lid closure;
Frequently associated with paresis of other muscles

supplied by the cranial nerves;

Various skeletal and muscle defects are common,

including absence or hypoplasia of pectoral muscles

(Poland syndrome), syndactyly, club feet, and
congenital limb amputations.

http://factoidz.com/moebius-syndrome-a-rare-disease-that-can-break-parents-

Congenital fibrosis
syndrome
Rare group of congenital disorders

characterized by varying amount of

restriction of the extraocular muscles and
replacement of the muscles by fibrous
tissue;

Etiology unknown;
Nonprogressive.

Congenital fibrosis
syndrome
Types:
General fibrosis is the more severe form (usually AD, may

be AR), involving all the extraocular muscles of both

eyes, including levator palpebrae superioris, with ptosis;
Congenital unilateral fibrosis with enophthalmos and
ptosis is nonfamilial;
Congenital fibrosis of the inferior rectus is sporadic or
familial, levator may be involved;
Strabismus fixus involves the horizontal recti with severe
esotropia;
Vertical retraction syndrome involves the superior rectus
muscle, with inability to depress the eye;

Congenital fibrosis
syndrome
Surgery is difficult and requires release of

the restricted muscles;

A good surgical result aligns the eyes in

primary position, but full ocular rotations

cannot be restored and the outcome is
unpredictable.

http://www.sarawakeyecare.com/Atlasofophthalmology/paediatric/paediatricophthalmologytpicture41congentialfibro