Eyelid retraction is considered to be present if, with the eyes in primary position, the sclera is visible above the superior corneal limbus. It is usually acquired but may be present at birth. Preterm infants occasionally have a benign transient conjugate downgaze associated with upper eyelid retraction. This finding is thought to be caused by immature myelination of the vertical eye movement system and immaturity or dysfunction of the extrageniculocalcarine visual pathways. Many normal infants (80% of children 14–18 weeks of age) have an eye-popping reflex when ambient lighting levels are reduced.
Causes of eyelid retraction are listed in Table 11-2. The most common cause of eyelid retraction in adults is thyroid eye disease (see Fig 11-8). The eyelid retraction (Collier sign) in dorsal midbrain syndrome (see Chapter 7, Fig 7-5) is a less common cause. Unilateral eyelid retraction as a result of contralateral ptosis may occur in patients with levator aponeurotic defects; this phenomenon results from Hering’s law of equal innervation. Bilateral eyelid retraction can be associated with thyroid eye disease, familial periodic paralysis, Cushing syndrome, and midbrain disease, or hydrocephalus with vertical nystagmus. Unilateral eyelid retraction is caused chiefly by thyroid eye disease but may also occur from aberrant regeneration of the third nerve (see Chapter 8, on diplopia), Marcus Gunn jaw-winking syndrome (see Fig 11-10), and idiopathic levator fibrosis. Subconjunctival injections of botulinum toxin and several surgical procedures have been used to reduce the degree of eyelid retraction in patients with thyroid eye disease.
, RubinPA. Upper and lower eyelid retraction.. 2002;42(2):45–59.