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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
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
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
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
What is happening in the orbit
TSH           Hypothroidism
re c e p to rs    increases TSH


   o r b ita l
fa t/m u s c le
      c e ll
  (G ra v e s
 d is e a s e )

                  Receptors
                  increase with
                  smoking
TSH
      re c e p to rs




  Cell swells up
with glcycoproteins
        etc
What do we do in eye clinic




Have to identify if active, and how active, and
 try and predict prognosis with and without
a c tiv ity


              n o n e s m o k e r , e u th y r o id




                            1 year
a c tiv ity


              a c tiv ity in c r e a s e s   T S H le v e l




                            1 year
a c tiv ity


              a c tiv ity tr ip le s 2 0 c ig s /d a y




                            1 year
What do we do in eye clinic
         Note symptoms/appearance
         Measure exophthalmos/TED group
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
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
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
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)
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
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
• 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
a c tiv ity                                    steroids




                      1 ye a r
Threshold
                                                             for
a c tiv ity                                                  steroids




   Thyroid eye
                      1 year   Red=very active=proptosis/optic atrophy
   disease activity            black=intermediate=some proptosis
   score
                               green=soft tissue changes that will resolve
Threshold
                       for
a c tiv ity            steroids




              1 year
Threshold
                       for
a c tiv ity            steroids




              1 year
Threshold
                                                            for
a c tiv ity                                                 steroids




                               1 year


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

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The Eye Clinic's Approach to Thyroid Eye Disease

  • 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. TSH Hypothroidism re c e p to rs increases TSH o r b ita l fa t/m u s c le c e ll (G ra v e s d is e a s e ) Receptors increase with smoking
  • 7. TSH re c e p to rs 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
  • 9. a c tiv ity n o n e s m o k e r , e u th y r o id 1 year
  • 10. a c tiv ity a c tiv ity in c r e a s e s T S H le v e l 1 year
  • 11. a c tiv ity a c tiv ity tr ip le s 2 0 c ig s /d a y 1 year
  • 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 a c tiv ity steroids 1 ye a r
  • 20. Threshold for a c tiv ity steroids Thyroid eye 1 year Red=very active=proptosis/optic atrophy disease activity black=intermediate=some proptosis score green=soft tissue changes that will resolve
  • 21. Threshold for a c tiv ity steroids 1 year
  • 22. Threshold for a c tiv ity steroids 1 year
  • 23. Threshold for a c tiv ity steroids 1 year 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