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Thyroid ophthalmopathy
• Self-limiting auto-immune process
• Mild to severe irreversible sight threatening
disease
• Graves' disease is the most common thyroid
abnormality
• Other associations include Hashimoto's
thyroiditis, thyroid carcinoma, primary
hyperthyroidism, and neck irradiation
EPIDEMIOLOGY
• Most common disease affecting the orbit
• Female to Male ratio 9.3:1 (mild cases) ;
1.4:1 (severe cases)
• Severe cases more frequent in >50 yr age
group
• Cigarette smoking is the strongest modifiable
risk factor
Etiology
• 80% are clinically hyperthyroid and 10% are
clinically euthyroid
• Associated with normal to abnormal thyroid
function
• In patients who are hyperthyroid, eye signs
usually develop within 18 months
PATHOGENESIS
Symptoms
SIGNS
• Eyelid Retraction (91%)
• Proptosis (62%)
• Restrictive Myopathy (42%)
• Soft Tissue Involvement
• Optic Neuropathy (6%)
LID RETRACTION
• increased sympathetic stimulation of Müller’s
muscle by thyroid hormone
• overaction of the levator muscle contracting
against a tight inferior rectus
• muscle scarring between the lacrimal gland
fascia and levator(lateral flare)
Proptosis
• Usually (90%) bilateral
• TAO is the most common cause of unilateral
and bilateral proptosis in adults
• Resulting from enlargement of the extraocular
muscles and adipose tissue, as well as orbital
fat deposits and the infiltration of orbital
tissues by GAGs and leukocytes.
• Severe proptosis prevents adequate lid
closure, and may lead to severe exposure
keratopathy and corneal ulceration.
Pseudoproptosis
• High myopia
• Congenital glaucoma (buphthalmos)
• Ipsilateral eyelid retraction
• contralateral enophthalmos
• Shallow orbit
Restrictive Myopathy
• Eye movements are restricted due to edema
in the extraocular muscles during the
infiltrative stage and the subsequent fibrosis.
• Despite expansion of the extraocular muscles
in TAO, the muscle fibers themselves are
normal.
• IR>MR>SR>LR
Clinical signs in TED
• Facial signs
–Joffroy’s sign-absent creases in the forehead on
superior gaze.
Eyelid signs
• Kocher’s sign-staring appearance
• Vigouroux sign-eyelid fullness
• Rosenbach’s sign-tremors of eyelids
• Riesman’s sign-Bruit over the eyelids
• Von graefe’s sign-lid lag on downgaze
• Dalrymple’s sign-lid retraction
• Stellwag’s sign-incomplete & infrequent blinking
• Boston sign-jerky movements of lid on downGaze
• Gellineck’s sign-abnormal pigmentation of upper
lid
• Gifford’s sign-difficulty in everting the upper lid
• Enroth ’s sign-edema of lower lid
• Griffith’s sign-lid lag on upgaze
• Goldzeiher’s sign-conjunctival injection
Extraocular movement signs
• Moebius sign-unable to converge eyes
• Ballet’s sign-restriction of one or more EOM
• Suker’s sign-poor fixation on abduction
• Jendrassik’s sign-paralysis of all EOM
CLASSIFICATION
Werners classification: NO-SPECS
• Class 0: No signs or symptoms
• Class 1: Only signs (lid retraction, stare ― lid
lag)
• Class 2: Soft tissue involvement
• Class 3: Proptosis
• Class 4: Extraocular muscle involvement
• Class 5: Corneal involvement
• Class 6: Sight loss (optic nerve involvement)
• Does not necessarily show sequential
involvement
• Inability to assess disease activity
• No prognostic or clinical implication
CLINICAL ACTIVITY SCORE
• In 1989, Mourits et al.developed the Clinical
Activity Score (CAS) for evaluating
ophthalmopathy activity.
• Found to be of value in predicting the
outcome of immunosuppressive treatment
and immunotherapy because of its high
specificity and high positive predictive value.
• CAS ≥4 were more responsive to treatment as
compared to patients with CAS<4
• 1. Pain on or behind the globe
• 2. Pain on eye movement
• 3. Redness of the eyelids
• 4. Redness of the conjunctiva
• 5. Swelling of the eyelids
• 6. Chemosis
• 7. Swollen caruncle
• 8. Increase of proptosis
• 9. Decreased eye movement
• 10. Decreased visual acuity
The VISA Classification
• Devised by Peter Dolman and Jack Rootman
• Based on four disease points
• Basic form consists of 4 sections recording
symptoms on the left and signs on the right
• Each disease activity is graded
• Objective and reproducible
• Appropriate management for patients in a
logical sequence
EUGOGO classification
EUROPEAN GROUP ON GRAVES
ORBITOPATHY
• MILD GO
– • minor lid retraction (<2 mm)
– • mild soft-tissue involvement
– • exophthalmos <3 mm above normal
– • no or intermittent diplopia
– • corneal exposure responsive to lubricants
• MODERATE GO
– • lid retraction ≥2 mm
– • moderate or severe soft-tissue involvement
– • exophthalmos ≥3 mm above normal
– • inconstant or constant diplopia
• SEVERE GO
– • DON and/or
– • corneal breakdown

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Thyroid ophthalmopathy

  • 2. • Self-limiting auto-immune process • Mild to severe irreversible sight threatening disease • Graves' disease is the most common thyroid abnormality • Other associations include Hashimoto's thyroiditis, thyroid carcinoma, primary hyperthyroidism, and neck irradiation
  • 3. EPIDEMIOLOGY • Most common disease affecting the orbit • Female to Male ratio 9.3:1 (mild cases) ; 1.4:1 (severe cases) • Severe cases more frequent in >50 yr age group • Cigarette smoking is the strongest modifiable risk factor
  • 4. Etiology • 80% are clinically hyperthyroid and 10% are clinically euthyroid • Associated with normal to abnormal thyroid function • In patients who are hyperthyroid, eye signs usually develop within 18 months
  • 6.
  • 8. SIGNS • Eyelid Retraction (91%) • Proptosis (62%) • Restrictive Myopathy (42%) • Soft Tissue Involvement • Optic Neuropathy (6%)
  • 9. LID RETRACTION • increased sympathetic stimulation of Müller’s muscle by thyroid hormone • overaction of the levator muscle contracting against a tight inferior rectus • muscle scarring between the lacrimal gland fascia and levator(lateral flare)
  • 10. Proptosis • Usually (90%) bilateral • TAO is the most common cause of unilateral and bilateral proptosis in adults • Resulting from enlargement of the extraocular muscles and adipose tissue, as well as orbital fat deposits and the infiltration of orbital tissues by GAGs and leukocytes.
  • 11. • Severe proptosis prevents adequate lid closure, and may lead to severe exposure keratopathy and corneal ulceration.
  • 12. Pseudoproptosis • High myopia • Congenital glaucoma (buphthalmos) • Ipsilateral eyelid retraction • contralateral enophthalmos • Shallow orbit
  • 13. Restrictive Myopathy • Eye movements are restricted due to edema in the extraocular muscles during the infiltrative stage and the subsequent fibrosis. • Despite expansion of the extraocular muscles in TAO, the muscle fibers themselves are normal. • IR>MR>SR>LR
  • 14. Clinical signs in TED • Facial signs –Joffroy’s sign-absent creases in the forehead on superior gaze.
  • 15. Eyelid signs • Kocher’s sign-staring appearance • Vigouroux sign-eyelid fullness
  • 16. • Rosenbach’s sign-tremors of eyelids • Riesman’s sign-Bruit over the eyelids • Von graefe’s sign-lid lag on downgaze
  • 17. • Dalrymple’s sign-lid retraction • Stellwag’s sign-incomplete & infrequent blinking • Boston sign-jerky movements of lid on downGaze • Gellineck’s sign-abnormal pigmentation of upper lid • Gifford’s sign-difficulty in everting the upper lid
  • 18. • Enroth ’s sign-edema of lower lid • Griffith’s sign-lid lag on upgaze • Goldzeiher’s sign-conjunctival injection
  • 19. Extraocular movement signs • Moebius sign-unable to converge eyes • Ballet’s sign-restriction of one or more EOM • Suker’s sign-poor fixation on abduction • Jendrassik’s sign-paralysis of all EOM
  • 21. Werners classification: NO-SPECS • Class 0: No signs or symptoms • Class 1: Only signs (lid retraction, stare ― lid lag) • Class 2: Soft tissue involvement • Class 3: Proptosis • Class 4: Extraocular muscle involvement • Class 5: Corneal involvement • Class 6: Sight loss (optic nerve involvement)
  • 22. • Does not necessarily show sequential involvement • Inability to assess disease activity • No prognostic or clinical implication
  • 23. CLINICAL ACTIVITY SCORE • In 1989, Mourits et al.developed the Clinical Activity Score (CAS) for evaluating ophthalmopathy activity. • Found to be of value in predicting the outcome of immunosuppressive treatment and immunotherapy because of its high specificity and high positive predictive value. • CAS ≥4 were more responsive to treatment as compared to patients with CAS<4
  • 24.
  • 25. • 1. Pain on or behind the globe • 2. Pain on eye movement • 3. Redness of the eyelids • 4. Redness of the conjunctiva • 5. Swelling of the eyelids • 6. Chemosis • 7. Swollen caruncle • 8. Increase of proptosis • 9. Decreased eye movement • 10. Decreased visual acuity
  • 26. The VISA Classification • Devised by Peter Dolman and Jack Rootman • Based on four disease points • Basic form consists of 4 sections recording symptoms on the left and signs on the right • Each disease activity is graded • Objective and reproducible • Appropriate management for patients in a logical sequence
  • 27.
  • 28. EUGOGO classification EUROPEAN GROUP ON GRAVES ORBITOPATHY • MILD GO – • minor lid retraction (<2 mm) – • mild soft-tissue involvement – • exophthalmos <3 mm above normal – • no or intermittent diplopia – • corneal exposure responsive to lubricants
  • 29. • MODERATE GO – • lid retraction ≥2 mm – • moderate or severe soft-tissue involvement – • exophthalmos ≥3 mm above normal – • inconstant or constant diplopia
  • 30. • SEVERE GO – • DON and/or – • corneal breakdown