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.

1 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 esotropia1.

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
 6th nerve palsy

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.

 6th nerve palsy

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/6702272f1.html
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 Pseudoexotropia
(Hypertelorism)

http://www.peds.ufl.edu/divisions/genetics/teaching/facial_dysmorphology.htm
 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 Exotropic

http://www.aapos.org/terms_faqs/faq_list/exotropia
 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 6th 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-hearts/
 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/paediatricophthalmologytpicture41congentialfibrosissyndrome.htm