Eyelid retraction is present if the sclera is visible above the superior corneal limbus when the eyes are in primary position. It is usually acquired but may be present at birth. Preterm infants occasionally have a benign transient conjugate downgaze associated with upper eyelid retraction. In many healthy infants (80% of children 14–18 weeks of age), bilateral eyelid retraction (eye-popping reflex) occurs when ambient lighting levels are reduced.
Table 11-2 lists the causes of acquired eyelid retraction. In adults, the most common cause is thyroid eye disease (Fig 11-7). The eyelid retraction (Collier sign) in dorsal midbrain syndrome (see Chapter 8, Fig 8-5) is a less common cause. Unilateral eyelid retraction can develop as a result of contralateral ptosis (pseudo–eyelid retraction); 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 CN III (see Chapter 7), Marcus Gunn jaw-winking syndrome (see Fig 11-6), idiopathic levator fibrosis, carcinomatous infiltration, or iatrogenic causes (strabismus surgery on the vertical rectus muscles or ptosis repair). Several surgical procedures have been used to reduce the degree of eyelid retraction in patients with thyroid eye disease. See BCSC Section 7, Oculofacial Plastic and Orbital Surgery, for a review of eyelid retraction treatment methods.
Table 11-2 Causes of Acquired Eyelid Retraction
Figure 11-7 Eyelid retraction from thyroid eye disease. This 73-year-old woman presented with a 6-month history of foreign-body sensation (previously treated as “dry eye syndrome”). Eyelid retraction on the left side is enhanced by severe left inferior rectus restriction.
(Courtesy of Steven A. Newman, MD.)
Excerpted from BCSC 2020-2021 series: Section 5 - Neuro-Ophthalmology. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.