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THE SQUINT SYNDROMES

Dany Najjar, MD

 

I. ESOTROPIA

Classification:

  1. Nonparetic (Comitant)
    1. Non-accomodative
      1. Infantile
      2. Acquired
    2. Accomodative
      1. Hyperopic
      2. High AC/A ratio
    3. Partially accommodative
  2. Paretic (Non Comitant)
  3. Pseudoesotropia

Definitions:

Paretic: due to paresis or paralysis of one or more EOM.

Comitant: the deviation is approximately the same in all gazes.

Comitant = Nonparetic ; usually more common in children

Non comitant = Paretic; usually more common in adults.


Infantile Esotropia:

- Most common type of esotropia

  • Usually occurs before the age of 6 months,
  • Comitant
  • Etiology: ? faulty innervational control involving the supranuclear pathways for convergence and divergence and their neural connections to the medial longitudinal fasciculus.
  • Hereditary: Autosomal Dominant
  • Deviation is often large (>40 prism diopters)
  • Most common error of refraction is hyperopia.
  • Frequently associated with nystagmus, overaction of the inferior obliques and DVD.
  • Alternate fixation: occurs if at various times either eye is used for fixation à  vision will be nearly equal in both eyes.
  • Cross fixation: occurs with large angle esotropia when the right eye is used for left gaze and the left eye is used for right gaze.

Treatment:

  • Correction of hyperopia
  • Treatment of amblyopia
  • Surgical treatment: Bimedial rectus recessions or medial rectus recession and lateral rectus resection.

 

Acquired Non-accomodative Esotropia:

  • Usually occurs after the age of 2 years.
  • Angle of deviation is smaller than in infantile ET but may increase with time.

 

Accomodative Esotropia:

  • There is a normal mechanism of accommodation with associated overaction of the convergence response and insufficient relative fusional divergence to maintain the eyes straight.
  • 2 types:

High Hyperopia: requiring high accommodation and therefore

convergence. Treatment: full cycloplegic correction.

High AC/A ratio: (Accomodative convergence to

accomodation). The deviation is greater at near than at

distance.

Treatment is full cycloplegic correction +/- bifocals.

 

Paretic (Noncomitant) Esotropia: (Sixth nerve Palsy)

  • More common in adults
  • Differential Diagnosis:
    1. Microvascular diseases: Diabetes and Hypertension
    2. Inflammatory and infectious CNS disorders: meningitis, raised ICP
    3. CNS tumors
    4. Head trauma
  • ET is greater at distance than at near.
  • Treatment: Wait till 6-8 weeks: Botulinum toxin.
  • > 6 months: medial rectus recession + lateral rectus resection.

 

Pseudoesotropia:

  • Occurs in patients with flat broad nasal bridge and prominent epicanthal folds.
  • Gradually disappears with age.
  • Hirschberg test differentiates it from true esotropia.

 

 

II. EXOTROPIA:

  • Less common than esotropia but incidence increases with age.
  • May progress from exophoria to intermittent exotropia to constant exotropia.
  • Possibly hereditary: Autosomal Dominant.
  • Types:
    1. Basic Exotropia: D=N
    2. Divergence Excess D>N
    3. Convergence Insufficiency N>D

 

Intermittent Exotropia:

  • Most common type.
  • Onset of deviation within the first year of age
  • Characteristic sign is closing one eye in bright light
  • Not associated with any specific error of refraction.
  • Usually not associated with amblyopia.
  • Treatment:
    1. Correction of refractive error
    2. Treatment of amblyopia if present
    3. Surgical treatment for deteriorating intermittent XT:
      a. Bilateral lateral rectus recession (if D>N)
      b. Resection of medial and recession of lateral rectus (if N>D)

       

Constant Exotropia:

  • May be present at birth or may progress from intermittent XT.
  • Sensory XT: one form of constant XT that occurs following loss of vision in one eye (ex. After trauma)
  • Associated with suppression in young age to avoid diplopia.
  • Treatment: Surgical correction.

 

A and V patterns:

  • A horizontal deviation may be different in upgaze versus downgaze, forming A and V patterns.
  • A pattern shows more esotropia (or less exo) in upgaze
  • V pattern shows more exotropia (or less eso) in downgaze.
  • A pattern is significant if greater than 10 prism diopters.
  • V pattern is significant if greater than 15 prism diopters.
  • They are frequently associated with overaction of the oblique muscles:

a. Inferior oblique for V pattern
b. Superior oblique for A pattern.


III.VERTICAL DEVIATIONS:

  • Usually occur after childhood.
  • Deviation named according to the nonfixing or "bad" eye.
  • Can be produced by systemic disorders ex. Orbital tumors, brainstem lesions, myasthenia gravis, multiple sclerosis and Grave’s disease.
  • Most common symptom is diplopia (if deviation occurs after age 6). Suppression occurs if age is less than 6 years.
  • Most are noncomitant.
  • The most commonly paretic vertical muscle is the superior oblique
  • The first muscle to be involved in Grave’s disease is the inferior rectus.
  • Maximal deviation occurs in the field of action of the paretic muscle.

 

IV. DUANE’S SYNDROME:

Characterized by :

  • Marked limitation of abduction
  • Mild limitation of adduction
  • Retraction of the globe and narrowing of the palpebral fissure on adduction
  • Upshoot or downshoot on adduction.
  • Incidence: It is more common in the left eye.
  • Pathology: Faulty innervation of the lateral rectus by fibers from the medial rectus à does not obey Sherrington’s law. This results in cocontraction of the medial and lateral rectus muscles.
  • May cause compensatory face turn to the Duane’s eye side.

                                                           


         


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