1. The Eye & General Medicine Exophthalmos & thyroid eye disease A case report for a ‘Grand Round’ Good Hope Hospital, March 2003 David Kinshuck, Associate Specialist, Eye Clinic

2. Case: Thyroid eye disease (Graves) 46y female 1998 (41y) thyrotoxicosis, treated with antithyroid drugs 1999 radioactive iodine (systemic steroids 2 months) 2001 exophthalmos, active, referred 2003 stable, but expecting treatment Unhappy with appearance Sore gritty watery eyes

3. Appearance Upper lid retraction Lower lid retraction Exophthalmos (proptosis) Dry eyes Soft tissue lid swelling Symptoms Sore eyes/dry eyes/watery Appearance Optic nerve damage glaucoma

4. Pathology, basis Enlarged ocular muscles & soft tissues Glycoproteins accumulate inside cells, lipid accumulation, lymphocytes, fibrosis Pushes eye forward… exophthalmos Or presses on optic nerve (blindness) Eye movements reduced, squints

5. What is happening in the orbit

6. Receptors increase with smoking Hypothroidism increases TSH

7. Cell swells up with glcycoproteins etc

8. What do we do in eye clinic Have to identify if active, and how active, and try and predict prognosis with and without

12. What do we do in eye clinic Note symptoms/appearance Measure exophthalmos/TED group

13. What do we do in eye clinic Note symptoms/appearance Measure exophthalmos/TED group Afferent pupillary defect Test visual fields, colour vision, eye pressure, examine optic disc

14. What do we do in eye clinic Note symptoms/appearance Measure exophthalmos/TED group Afferent pupillary defect Test visual fields, colour vision, eye pressure, examine optic disc CT scan..diagnosis, especially if unilateral

15. What do we do in eye clinic Note symptoms/appearance Measure exophthalmos/TED group Afferent pupillary defect Test visual fields, colour vision, eye pressure, examine optic disc CT scan..diagnosis, especially if unilateral Diagnose/treat ‘active’disease & inactive disease..eg lid/squint surgery

16. Proposed Classification System to Assess Disease Activity in Thyroid Eye Disease. One point is given for each sign present. Pain Painful, oppressive feeling on or behind the globe Pain on attempted up, side, or down gaze Redness Redness of the eyelids Diffuse redness of the conjunctiva Swelling Chemosis Oedema of the eyelid(s) Increase proptosis of 2 mm or more during a period between 1 and 3 months Impaired function Decrease in visual acuity of 1 or more lines on the Snellen chart (using a pinhole) during a period between 1 and 3 months Decrease of eye movements in any direction equal to or more than 5 degrees during a period of time between 1 and 3 months (From Mourits et al)

17. Signs of activity: Puffiness increasing exophthalmos increasing pain increasing patient is reasonable judge optic nerve compression/field loss diplopia beginning all this early on in disease, unusual after 1 year

18. Treatment in active phase If active AND sight threatened, or proptosis disfiguring, systemic steroids (or it is reasonably clear this is likely) If activity continues despite steroids, radiotherapy to orbits Can decompress orbit surgically as alternative Soft tissue activity only, no proptosis, so no steroids Depends on ‘activity’ score

19. Can be difficult to determine whether disease is active Best results are when (severe cases) are given steroids early Don’t really know which the ‘severe’ cases are early on Threshold for steroids

20. Thyroid eye disease activity score Red=very active=proptosis/optic atrophy black=intermediate=some proptosis green=soft tissue changes that will resolve

21. Threshold for steroids

23. At onset difficult to judge outcome………………….

24. Our patient Stable appearance for 2 years Smokes, best to stop (may not make much difference at this stage) Offered referral for cosmetic surgery Lid surgery (insert ‘spacer’ material) possibly orbit surgery, more complex/risky

25. Illustrates problems in medicine (life) generally Treatments get more effective, more is known, making tremendous progress powerful treatments, side effects, often hard to decide at the time what to do sub-specialty training helps, but patients will not present to the expert initially treatment windows: leaving things late causes problems hard data lacking: cannot be trained for all problems