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Risk factors

• Smoking, female sex, radioactive iodine

Findings

• Soft tissue involvement: Epibulbar hyperaemia, periorbital swelling, superior limbal keratoconjunctivitis
• Eyelid retraction: widened palpebral fissure, superior and inferior “scleral show”
  • Dalrymple’s sign: superior scleral show in primary position
  • Graefe’s sign: “lid lag”, delayed/absent lowering of the upper eyelid when the gaze is lowered
  • Kocher’s sign: increased eyelid retraction during fixation, resulting in an anxious gaze
• Exophthalmos: abnormal Hertel test if >20mm and/or difference of 2-3mm between both eyes
• Exposure Keratopathy
• Compressive optic neuropathy:
  • Visual acuity may be reduced
  • RAPD
  • Colour desaturation
  • Reduced contrast sensitivity
  • Visual field defects (central or paracentral)
  • Optic disc usually unremarkable, occasionally optic disc swelling, rarely optic atrophy
• Restrictive myopathy: double vision, approx. 30 – 50% of patients develop ocular muscle palsy

Stages and therapy

1. Congestive (inflammatory) stage:

• Red, painful eyes, remission within 3 years in most cases, in the long term approx. 10% with severe eye involvement.
• Therapy depending on activity:
  • Lubricating eye drops, e.g. Lacrycon gtt, Vitamin A/Bepanthen Eye ointment
  • Consider local therapy with steroids, NSAID, Cyclosporine gtt
  • Stop smoking!
  • Selenase peroral solution 100mcg 2x daily
  • Methylprednisolone 500mg i.v. 1x weekly for 6 weeks, then 250mg i.v. 1x weekly for 6 weeks,
    • oral therapy obsolete
    • Steroid therapy should not be administered more frequently than once a year.
    • consider higher doses in severe cases
    • After steroid therapy, wait at least 3 months before radiotherapy is planned/performed, unless there is evidence of a compressive optic neuropathy.
  • Radiation therapy (a response is usually seen within 6 weeks)
  • Consider additional azathioprine or rituximab.
  • Consider teprotumumab (Cave: very expensive)
  • Surgical decompression

2. Fibrotic (quiet) stage

• Quiet eyes without active inflammation, painless restrictive myopathy possible
• Therapy: surgery in stable conditions without signs of activity for >6 months
  • In general, operations for TED are performed in the following order:
    • Orbita -> Strabismus -> Eyelids

Clinical Activity Scores (CAS)

Assessment of TED activity: 1 point each, for initial CAS only score items 1-7

• Subjective activity symptoms
  • 1 Spontaneous orbital pain
  • 2 Gaze evoked orbital pain
• Objective signs of inflammation
  • 3 Eyelid erythema
  • 4 Conjunctival redness
  • 5 Eyelid swelling
  • 6 Chemosis
  • 7 Caruncle and/or plica swelling
• Signs of progression during follow-up examinations
  • 8 Increase of ≥ 2mm in proptosis in the last 1-3 months
  • 9 Decrease in ocular motility ≥ 8° in the last 1-3 months
  • 10 Decrease of visual acuity equivalent to 1 Snellen line in the last 1-3 months

Total score progression (max. 10 points)

• Initial visit: Score ≥ 3 = active TED
• Follow-up visit: Score ≥ 4 = active TED

Work-up

• Laboratory: TSH, fT3, fT4, TRAb, TPO-Ab, Tg-Ab
• CT / MRI Orbit: typically thickened eye muscles without tendon involvement
• OCT and Visual field examination in cases of suspected optic neuropathy

Therapy at a glance

• Activity present: Severity?
  • mild: observation, smoking cessation, lubrification, optimise thyroid levels
  • moderate: steroids, radiotherapy
  • severe/malignant: steroids iv, rituximab, consider emergency decompression, teprotumumab
• No activity present: stable?
  • Not stable: wait, re-evaluation after 6 months
  • stable: sequence of surgical treatment: decompression > strabismus surgery > eyelid surgery

Sources

• EyeWiki Thyroid Ophthalmopathy
• The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7. Auflage (2016)
• Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8. Auflage (2015)