Eyelid Retraction

Eyelid retraction is abnormal elevation of the upper lid margin (above the superior limbus, with scleral show) or lowering of the lower lid margin (below the inferior limbus). Normal position: the upper lid covers 1 to 2 mm of the cornea and the lower lid lies at the limbus.

• Causes:
  • Thyroid eye disease, by far the commonest cause (sympathetic overstimulation of the Muller muscle, levator fibrosis, and inflammation); about 90 percent of patients with thyroid eye disease develop upper lid retraction.
  • Neurogenic: dorsal midbrain syndrome (Collier sign), aberrant regeneration after third nerve palsy, and facial palsy (lower lid).
  • Post-surgical: after ptosis, blepharoplasty, or eyelid reconstructive surgery.
  • Mechanical: scarring of the anterior or posterior lamella, orbital fracture, and tethering.
  • Contralateral ptosis (through Hering law of equal innervation), high myopia, proptosis, and congenital retraction.
  • Drug effects (adrenergic drugs), hyperthyroidism, and cirrhosis.
• Pseudoretraction occurs with proptosis or contralateral ptosis.

• Staring or startled appearance, scleral show above or below the limbus, widened palpebral fissure, and lid lag on downgaze (von Graefe sign) in thyroid disease.
• Dry eye symptoms, exposure keratopathy, and lagophthalmos; difficulty closing the eyes.
• Thyroid eye disease: proptosis, conjunctival redness, chemosis, restricted motility, and signs of hyperthyroidism (tremor, tachycardia, weight loss).
• Dorsal midbrain syndrome: upgaze palsy, light near dissociation, convergence retraction nystagmus, and bilateral retraction.
• Unilateral retraction with contralateral ptosis improves when the ptotic lid is lifted manually.

• Measure the margin to reflex distance (MRD1 normal 4 to 5 mm; above 5.5 mm indicates retraction), the palpebral fissure height, lid lag, and corneal exposure.
• Thyroid function tests (TSH, free T4, free T3), thyroid antibodies (TSH receptor antibodies, thyroid peroxidase), and orbital imaging (CT or MRI) showing extraocular muscle enlargement.
• Neurological examination and MRI of the midbrain for dorsal midbrain signs.
• Assessment of the phenylephrine test for contralateral ptosis and Muller muscle contribution.
• Fluorescein staining of the cornea and tear function tests.

• Treat the cause: in thyroid disease achieve and maintain euthyroidism (carbimazole 10 to 40 mg daily or methimazole, with block-and-replace or titration; radioactive iodine requires steroid cover in active disease), stop smoking, and treat active inflammation (intravenous methylprednisolone 4.5 g cumulative over 12 weeks, teprotumumab 10 mg/kg then 20 mg/kg every 3 weeks for 7 infusions, or selenium 100 micrograms twice daily for 6 months in mild disease).
• Ocular surface protection: preservative free lubricants four to six times daily and ointment at night, moisture chamber goggles, and lid taping.
• Non-surgical lid lowering in the active phase: botulinum toxin A 2.5 to 5 units injected into the levator or conjunctiva (effect for 3 months); subconjunctival triamcinolone 20 mg (40 mg/mL, 0.5 mL) into the upper lid for active inflammation.
• Surgery once the disease has been stable for at least 6 months (and after orbital decompression and strabismus surgery, in that order, when needed):
  • Upper lid: recession of the Muller muscle (Mullerectomy), levator aponeurosis recession with or without spacer grafts, and full thickness blepharotomy for severe retraction.
  • Lower lid: recession of the lower lid retractors with a spacer graft (hard palate mucosa, acellular dermal matrix, or ear cartilage).
  • Lateral tarsorrhaphy for exposure.
• Post-surgical or cicatricial retraction: release of scar tissue with grafts (full thickness skin graft for the anterior lamella, mucosal graft for the posterior lamella).
• Neurogenic: treat the underlying lesion; botulinum toxin for symptomatic relief.
• Follow up: every 3 to 6 months until stable, with regular corneal assessment.
• Complications: exposure keratopathy, corneal ulcer, undercorrection or overcorrection after surgery, lagophthalmos, and cosmetic distress.