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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
- In general, operations for TED are performed in the following order:
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)