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Ocular System

Marcus Gunn jaw-winking syndrome (MGJWS)

Congenital condition in which coordinated synkinetic movement of the upper eyelid and the jaw occur as a result of an abnormal connection between the motor branches of the trigeminal (CN V) nerve and superior division of oculomotor nerve (CN III).

Congenital condition in which coordinated synkinetic movement of the upper eyelid and the jaw occur as a result of an abnormal connection between the motor branches of the trigeminal (CN V) nerve and superior division of oculomotor nerve (CN III).

  • Type of congenital cranial dysinnervation disorders (CCDD)

History:

MGJWS was first described by a Scottish ophthalmologist Dr.Robert Marcus Gunn in the year 1883. This syndrome was initially reported in a 15-year-old girl as unilateral ptosis associated with the upper eyelid contraction on the same side. Other names of MGJWS include Marcus-Gunn jaw winking phenomenon (MGP), Marcus Gunn ptosis, Marcus Gunn jaw winking trigemino-oculomotor synkinesis, Maxillopalpebral synkinesis, and Pterygoid-levator synkinesis.


Aetiology

Associated conditions:

  • Cleft lip/cleft palate
  • Olfactory nerve disturbance
  • CHARGE syndrome (coloboma, heart defects, choanal atresia, retardation of growth and development, genital abnormalities, and ear abnormalities)
  • Renal calculi

Clinical features

Jaw-wink reflex:

Momentary retraction/elevation of the upper eyelid (which was ptotic) upon stimulation of the ipsilateral pterygoid muscle, which is followed by a rapid return to a lower position
  • Movement of one upper eyelid in a rapid rising motion each time the jaw moves

Unilateral blepharoptosis:

Normal, low-lying upper eyelid margin

Associated features:

  • Strabismus (50-60% cases)
  • Anisometropia (5-25% cases)
  • Amblyopia (30-60%), almost always secondary to strabismus/anisometropia
    • Defined as a decrease in vision of 2 or more lines on the Snellen chart
  • Superior rectus palsy (25% cases)
  • Double elevator palsy (25% cases)
  • Morning Glory anomaly (rare)
  • Duane retraction syndrome (rare)

Differential diagnosis

Inverse Marcus Gunn phenomenon:

Ptosis in primary gaze is worsened with mouth opening as apposed to MGJWS where the ptosis improves with mouth opening

Marin Amat syndrome:

Abnormal connection between the motor branch of CN V nerve and frontal, zygomatic branches of CN VII. Here, both eyes close momentarily like blepharospasm with mouth opening secondary to the action of orbicularis oculi muscle.

Other differential diagnoses:

  • Chronic progressive external ophthalmoplegia
  • Congenital fibrosis syndrome
  • Myasthenia gravis

Management

Treatment of Marcus Gunn phenomenon is usually not needed because the condition tends to grow less noticeable with age.

  • Aggressive treatment with occlusion therapy and/or correction of anisometropia before any consideration of ptosis surgery if amblyopia is encountered.
  • In severe cases: Surgery with unilateral levator excision and frontalis brow suspension may be used

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