DESCRIPTION

• Strabismus is defined as any form of ocular misalignment. It derives from the Greek word strabismos (to squint).
• Strabismus can be intermittent or constant.
• There are many types of strabismus, which are defined by the direction of misalignment.
  • Exotropia: out-turning of eyes
  • Esotropia: in-turning of eyes
  • Hypertropia: one eye higher than the other eye
• Strabismus can be comitant (amount of misalignment is the same in all directions of gaze) or incomitant (variable angle of deviation, which is dependent on the direction of gaze).
  • Comitant strabismus is the most common form of strabismus. These children are typically developmentally normal.
  • Incomitant strabismus is less common. It is caused by paralytic strabismus such as cranial nerve palsies or restrictive strabismus such as Brown syndrome.
• Strabismus may cause permanent loss of three-dimensional vision, amblyopia (visual acuity loss), and/or ocular torticollis.
• Strabismus can result in significant psychosocial problems for children, which warrant attention and treatment.
• Patients with intermittent strabismus can also develop lifelong loss of depth perception and visual acuity. These children should be evaluated and potentially treated for their strabismus.

EPIDEMIOLOGY

RISK-FACTORS

• Low birth weight
• Maternal cigarette smoking
• Retinopathy of prematurity
• Refractive errors: high hyperopia and anisometropia
• Congenital or acquired vision loss
• Cerebral palsy
• Craniofacial syndromes
• Seizure disorders
• Developmental delays
• Hydrocephalus
• Neurologic problems—stroke, brain tumor
• Family history of strabismus

PATHOPHYSIOLOGY

• There is a limited understanding of the pathophysiology of the most common comitant strabismus. There is no specific pathologic abnormality of the cranial nerves, extraocular muscles, or orbits.
• Accommodative esotropia is a common form of strabismus in young children. It is associated with high hyperopia (farsightedness) and anisometropia (see “Refractive Error”). When a child with high hyperopia attempts to focus at any distance, he or she needs to focus his or her intraocular lens (accommodation). This focusing can trigger overconvergence of the eyes (esotropia).
• Paretic strabismus is caused by weakness of cranial nerves and their associated extraocular muscles. Examples of this type of pathology include cranial nerve palsies—III, IV, and VI; Möbius syndrome; or Duane syndrome.
• Neuromuscular diseases such as myasthenia gravis can cause strabismus with decreased extraocular muscle function.
• Restrictive strabismus is a result of muscle tightness causing a limitation in eye movement. Examples include Graves disease, Brown syndrome, or trauma to extraocular muscles.
• Sensory strabismus results from poor visual acuity in one eye.

ASSOCIATED-CONDITIONS

• Strabismus can be a sign of a vision- or life-threatening neurologic problem.
  • A physician needs to consider that retinoblastoma, brain tumor, cataract, and other conditions may initially present with ocular misalignment.
• Other ocular problems often coexist with strabismus including amblyopia, nystagmus, and refractive error.