Thyroid Eye Disease (TED)

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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

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

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

Subjective activity symptoms
- 1 Spontaneous retrobulbar pain
- 2 Pain on attempted upward or downward gaze
Objective signs of inflammation
- 3 Redness of eyelids
- 4 Redness of conjunctiva
- 5 Swelling of eyelids
- 6 Swelling of conjunctiva (chemosis)
- 7 Swelling of caruncle or plica
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)

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

EyeWiki Thyroid Eye Disease
L Bartalena, G J Kahaly et al. EUGOGO, The 2021 European Group on Graves’ orbitopathy (EUGOGO) clinical practice guidelines for the medical management of Graves’ orbitopathy, European Journal of Endocrinology, Volume 185, Issue 4, Oct 2021, Pages G43–G67, https://doi.org/10.1530/EJE-21-0479
The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Kalla A. Gervasio MD, Travis J. Peck MD et al.; Wolters Kluwer; 8. Auflage (2021)
Kanski’s Clinical Ophthalmology: A Systematic Approach; John F. Salmon; Elsevier; 10. Auflage (2024)

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