Marcus Gunn Jaw-winking Syndrome Clinical Presentation

Updated: Jul 20, 2018
  • Author: Barbara L Roque, MD, DPBO, FPAO; Chief Editor: Hampton Roy, Sr, MD  more...
  • Print
Presentation

History

Signs and symptoms of Marcus Gunn jaw-winking syndrome may include the following:

  • Mild-to-moderate blepharoptosis, usually unilateral

  • Synkinetic upper eyelid movement with jaw-winking after one of the following:

    • Mouth opening

    • Jaw movement toward the contralateral side

    • Chewing

    • Sucking

    • Jaw protrusion

    • Clenching teeth together

    • Swallowing

  • Usually, parents first notice the phenomenon while the baby is bottle-feeding or breastfeeding.

  • Jaw-winking worse in downgaze

  • Decreased vision secondary to amblyopia

  • Strabismus (see the following)

    • Vertical deviation, usually a hypotropia on the involved side

    • Horizontal deviation (rare cases)

Past ocular history may include the following:

  • Occlusion or patching therapy for amblyopia

  • Strabismus surgery

  • Periorbital trauma (suggests an aberrant third nerve regeneration if accompanied by bizarre extraocular movements and diplopia)

  • Previous eyelid surgery

  • Dry eyes (important if contemplating ptosis repair)

Past medical history may include the following:

  • Details of birth history

  • Previous reactions to anesthesia

  • Previous diagnosis of a bleeding diathesis

  • Medication history to include anticoagulants or any nonsteroidal anti-inflammatory drugs (NSAIDs)

Next:

Physical

See the list below:

  • Complete ophthalmic examination

    • Pupillary examination

    • Visual acuity (rule out amblyopia in infants and children)

    • Cycloplegic refraction (rule out anisometropia)

  • External examination

    • Extraocular motility

    • Cover test (rule out a superior rectus or double elevator palsy)

    • Bell phenomenon (can be decreased with a superior rectus or double elevator palsy)

    • Head position

      • A child may elevate the chin to see.

      • If a child does not elevate the chin in the presence of moderate-to-severe ptosis, then consider amblyopia.

    • Ptosis evaluation

      • The degree of ptosis should be assessed with the jaw immobilized in a central position and after fusion is disrupted with brief ocular occlusion. [20]

      • Vertical palpebral fissure - Widest distance between the upper and lower eyelid

      • Marginal reflex distance (MRD) - Distance from the upper eyelid margin to corneal light reflex in primary position

      • Ptosis can be quantified - Mild (less than or equal to 2 mm), moderate (3 mm), or severe (greater than or equal to 4 mm)

      • Upper eyelid crease position - Distance from the crease to the eyelid margin

      • Levator function - Good (greater than or equal to 8 mm), fair (5-7 mm), or poor (less than or equal to 4 mm)

      • Measurement of eyelid position in downgaze

      • Presence of lid lag

    • Attempt to elicit synkinesis of eyelid movement.

      • Have the infant bottle-feed.

      • An older child can chew gum.

      • Have the patient open the mouth, move the jaw from side to side, or protrude the jaw forward.

      • Jaw-wink can be quantified - Mild (less than or equal to 2 mm), moderate (3-6 mm), or severe (greater than or equal to 7 mm)

Previous
Next:

Causes

See Pathophysiology.

Previous