50 Years ago in The Journal of Pediatrics:

Article Outline

• Copyright

Smith EE, Gans ME. J Pediatr 1957; 50: 52-54

The authors reported a 1-month-old infant who was normal except for a mild right upper lid ptosis. The mother noted that during breast feeding, the drooping right upper eyelid would shoot up. This phenomenon is referred to as Marcus Gunn jaw winking. It was first described by Marcus Gunn, an English ophthalmologist, in 1883. Perhaps better known than the jaw winking phenomenon is the Marcus Gunn pupil, a test for a unilateral optic nerve disease. In either case, a hyphen should not be placed between Marcus and Gunn.

The Marcus Gunn jaw winking phenomenon characterized by the movement of 1 upper eyelid in a rapid rising motion each time the jaw moves is thought to be due to anomalous wiring of the motor division of the trigeminal nerve to the oculomotor nerve supplying the levator muscle. The wink reflex consists of a brief upper eyelid retraction or elevation to an equal or higher level than the other eye with stimulation of the ipsilateral pterygoid muscle. As in this case report, the Marcus Gunn jaw winking phenomenon is often first noted in infancy with breast or bottle feeding.

Of particular importance to the pediatrician is that the Marcus Gunn jaw winking phenomenon is often associated with a high incidence of strabismus and amblyopia. If these conditions are not diagnosed at an early age, a permanent visual impairment may result.

The Marcus Gunn jaw winking phenomenon accounts for approximately 5% of all congenital ptosis and is almost always sporadic, although rare familial presentations with an autosomal inheritance pattern have been reported. The pediatrician should make certain to test all children with ptosis to determine whether the ptosis changes with jaw movements, given that Marcus Gunn syndrome is associated with strabismus in more than 50% of cases. This phenomenon is evident at birth, and often parents are the first to notice this jaw winking as their infants feed.

If the ptosis is mild, no treatment is usually necessary. It has been postulated that this phenomenon improves as children age. More likely is that the patient adapts or learns to compensate by masking the wink response and using jaw movements to prevent wide eye fluctuations. If the ptosis is either moderate or severe, there are multiple surgical approaches to be considered that involve either unilateral or bilateral lid surgery. If surgery is contemplated, it is important to first treat any associated amblyopia.