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THYROID EYE
DISEASE
Dr.Sidesh Hendavitharana
(Registrar-ophthalmology)
TED,
• Also known as Gravis ophthalmopathy, Thyroid-
associated orbitopathy,Thyrotoxic exophthalmos
• Is an autoimmune inflammatory disorder.
• Characteristic clinical signs includes eye lid retraction,lid
lag,proptosis,restrictive extraocular myopathy ,compresive
optic neuropathy,exposure keratopathy and conjuctival
erythema and chemosis.
Epidemiology
• In 1996 epidemiologic study in USA,age adjusted
incidence rate for women was 16 cases per 100,000
population,whereas the rate for men was 3 cases per
100,000 population per year.
• TED affects women aprox.6 times as frequently as men.
• Development of TED is up to 7 times as likely among
smokers as among nonsmokers.
• Myasthenia Gravis occur in less than 1% of pts and is 50
times more common in patients with TED.
ETIOLOGY
• Among pts with TED,
• Approx.90%have Gravis hyperthyroidism,6% are
euthyroid,3% have hashimoto thyroiditis, and 1%
have primary hypothyroidism.
• There is evidence that cessation of smoking reduces the
risk of progression of orbitopathy.
• Increased prevalence of HLA B8,DR-3 may increase
susceptibility to TED.
• Higher frequency of HLA –DR B1-16 allele in pts with
severe extra ocular muscle involvement. (Akaishi et
al.thyroid 2008)
Pathogenesis
• Orbital fibroblast is the primary target of the inflammatory
process associated with TED.
• Orbital fibroblasts are embryologically derived from the
neural crest cells.
• A subpopulation of orbital fibroblasts appears capable of
undergoing adipocyte differentiation.
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• .
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• .
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Clinical features
• Eye lid retraction
• Soft tissue involvement
• Proptosis
• Optic neuropathy/exposure keratopathy
• strabismus
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• .
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• .
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• .
Clinical signs in TED
• Facial signs
1) Joffroy’sign-absent creases in the forehead on superior gaze
2) Eyelid signs
3) Kocher’s sign-staring appearance
4) Vigouroux sign-eyelid fullness
5) Rosenbach’s sign-tremors of eyelids
6) Riesman’s sing-bruit over the eyelids
Upper eyelid signs
1) Von graefe’s sign-lid lag on downgaze
2) Dalrymple’sign-lid retraction
3) Stellwag’s sign-incomplete and infrequent blinking
4) Grove sign-resistance to pulling the retracted upper lid
5) Boston sign-jerky movements of lid on down gaze
6) Gellineck’s sign-abnormal pigmentation of upper lid
7) Gifford’s sign-difficulty in everting the upper lid
Lower lid signs
1) Enroth’s sign-edema of lower lid
2) Grifith’s sign-lid lag on upgaze
Conjuctival signs
• Goldzeiher’s sign-conjuctival injection
Extraocular movement signs
1) Moebius sign-unable to converge eyes
2) Ballet sign-restriction of one or more EOM
3) Suker sign-poor fixation on abduction
4) Jendrassik sign-paralysis of all EOM
Pupillary signs
1) Knies sign-uneven pupillary dilatation in dim light
2) Cowen sign-jerky contraction of pupil to light
Dalrymple sign
• .
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Ballet sign
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• .
Exophthalmos(proptosis)
• Common and classical sign
• Symetrical involvement of both eyes but frequently one
eye more prominent than the other.
• Self limiting
Optic neuropathy
• Due to direct compression of optic nerve or its blood
supply-5%.
• Papilledema or optic atrophy
• Gradual progressive visual loss
• Increased intraocular pressure
Grading (werner classification)
• NO SPECS
Grade 0-No signs or symptoms
Grade 1-Only signs(lid retraction with/without lid lag and mild
proptosis)
Grade 2-Soft tissue involvement
(chemosis,grit,lacrimation,photophobia,lid or conjuctival
swelling)
Grade 3-Proptosis(min.<23mm,marked >28mm)
Grade 4-Extraocular muscle involvement (limitation of
movements and diplopia)
Grade 5-Corneal involvement (exposure keratitis)
Grade 6-loss of slight (due to involvement of optic nerve with
disc pallor or papilloedema and visual field defect)
MOURITS CLASSIFICATION
• Clinical Activity Score(CAS)to grade the inflammatory
phase of the disease
• The CAS consists of two conjunctival,eyelid and two
orbital signs.
• A sore of 4/>-active disease subjective in nature with very
large interobserver variation
Clinical Activity Score
• For initial CAS,only score items 1-7
1) Spontaneous orbital pain
2) Gaze evoked orbital pain
3) Eye lid swelling that is considered to be due to active
inflammatory phase
4) Eyelid erythema
5) Conjunctival redness that is considered to be due to
active inflammatory phase
6) Chemosis
7) Inflammation of caruncle or plica
.
• Patients assesed after follow-up can be scored out of 10
by including items 8-10.
8.Increase of >2mm in proptosis
9.Decrease in uniocular excursion in any one direction of >8 deg.
10.Decrease of acuity equivalent to 1 snellen line.
Evaluation of thyroid eye disease
•Clinical examination
 Corrected distance visual acuity
 Colour vision
 Pupillary examination
 Ocular motility
 Hertel exophthalmometry
 IOP(in primary gaze and upgaze)
 Adnexal examination
 Slit lamp examination
 Dilated fundus examination
•Laboratory studies
• T3/free T4/TPO/TRAb/TSH/TSI
•Imaging studies
• Orbital untrasonography(assesment of extraocular muscle size and reflectivity)
• CT/MRI
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• .
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• .
MANAGEMENT OF TED
• Stepwise and graded approach
• Based on pts-reported symptoms,clinical examination and
ancillary testing.
• According to the clinical activity score(CAS), TED
categories into 4 stages.
A. Mild disease-<3 score
B. Moderate disease >/=4 score
C. Severe disease
D. Refractory disease
Mild disease
• Observaton
• Pts education and lifestyle changes
• Smoking cessation
• Salt restriction
• Elevation of head of bed
• Use of sunglasses
• Ocular surface lubrication
• Establishment of a euthyroid state
• Oral seleniun
Moderate disease
• Topical cyclosporine
• Eyelid tapering at night
• Moisture goggles or chambers
• Prism glasses or selective ocular patching
• Moderate dose oral steroid therapy
Severe disease
• High dose intravenous steroid therapy
• Surgical orbital decompresion(followed by strabismus
surgery and/or eyelid surgery)
• Periocular radiotherapy
Refractory disease
• Steroid sparing immunomodulators
• Eg:rituximab,others
Thank you
• .

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Thyroid eye disease

  • 2. TED, • Also known as Gravis ophthalmopathy, Thyroid- associated orbitopathy,Thyrotoxic exophthalmos • Is an autoimmune inflammatory disorder. • Characteristic clinical signs includes eye lid retraction,lid lag,proptosis,restrictive extraocular myopathy ,compresive optic neuropathy,exposure keratopathy and conjuctival erythema and chemosis.
  • 3. Epidemiology • In 1996 epidemiologic study in USA,age adjusted incidence rate for women was 16 cases per 100,000 population,whereas the rate for men was 3 cases per 100,000 population per year. • TED affects women aprox.6 times as frequently as men. • Development of TED is up to 7 times as likely among smokers as among nonsmokers. • Myasthenia Gravis occur in less than 1% of pts and is 50 times more common in patients with TED.
  • 4. ETIOLOGY • Among pts with TED, • Approx.90%have Gravis hyperthyroidism,6% are euthyroid,3% have hashimoto thyroiditis, and 1% have primary hypothyroidism. • There is evidence that cessation of smoking reduces the risk of progression of orbitopathy. • Increased prevalence of HLA B8,DR-3 may increase susceptibility to TED. • Higher frequency of HLA –DR B1-16 allele in pts with severe extra ocular muscle involvement. (Akaishi et al.thyroid 2008)
  • 5. Pathogenesis • Orbital fibroblast is the primary target of the inflammatory process associated with TED. • Orbital fibroblasts are embryologically derived from the neural crest cells. • A subpopulation of orbital fibroblasts appears capable of undergoing adipocyte differentiation.
  • 9. Clinical features • Eye lid retraction • Soft tissue involvement • Proptosis • Optic neuropathy/exposure keratopathy • strabismus
  • 13. Clinical signs in TED • Facial signs 1) Joffroy’sign-absent creases in the forehead on superior gaze 2) Eyelid signs 3) Kocher’s sign-staring appearance 4) Vigouroux sign-eyelid fullness 5) Rosenbach’s sign-tremors of eyelids 6) Riesman’s sing-bruit over the eyelids
  • 14. Upper eyelid signs 1) Von graefe’s sign-lid lag on downgaze 2) Dalrymple’sign-lid retraction 3) Stellwag’s sign-incomplete and infrequent blinking 4) Grove sign-resistance to pulling the retracted upper lid 5) Boston sign-jerky movements of lid on down gaze 6) Gellineck’s sign-abnormal pigmentation of upper lid 7) Gifford’s sign-difficulty in everting the upper lid
  • 15. Lower lid signs 1) Enroth’s sign-edema of lower lid 2) Grifith’s sign-lid lag on upgaze
  • 16. Conjuctival signs • Goldzeiher’s sign-conjuctival injection
  • 17. Extraocular movement signs 1) Moebius sign-unable to converge eyes 2) Ballet sign-restriction of one or more EOM 3) Suker sign-poor fixation on abduction 4) Jendrassik sign-paralysis of all EOM
  • 18. Pupillary signs 1) Knies sign-uneven pupillary dilatation in dim light 2) Cowen sign-jerky contraction of pupil to light
  • 23. Exophthalmos(proptosis) • Common and classical sign • Symetrical involvement of both eyes but frequently one eye more prominent than the other. • Self limiting
  • 24. Optic neuropathy • Due to direct compression of optic nerve or its blood supply-5%. • Papilledema or optic atrophy • Gradual progressive visual loss • Increased intraocular pressure
  • 25. Grading (werner classification) • NO SPECS Grade 0-No signs or symptoms Grade 1-Only signs(lid retraction with/without lid lag and mild proptosis) Grade 2-Soft tissue involvement (chemosis,grit,lacrimation,photophobia,lid or conjuctival swelling) Grade 3-Proptosis(min.<23mm,marked >28mm) Grade 4-Extraocular muscle involvement (limitation of movements and diplopia) Grade 5-Corneal involvement (exposure keratitis) Grade 6-loss of slight (due to involvement of optic nerve with disc pallor or papilloedema and visual field defect)
  • 26. MOURITS CLASSIFICATION • Clinical Activity Score(CAS)to grade the inflammatory phase of the disease • The CAS consists of two conjunctival,eyelid and two orbital signs. • A sore of 4/>-active disease subjective in nature with very large interobserver variation
  • 27. Clinical Activity Score • For initial CAS,only score items 1-7 1) Spontaneous orbital pain 2) Gaze evoked orbital pain 3) Eye lid swelling that is considered to be due to active inflammatory phase 4) Eyelid erythema 5) Conjunctival redness that is considered to be due to active inflammatory phase 6) Chemosis 7) Inflammation of caruncle or plica
  • 28. . • Patients assesed after follow-up can be scored out of 10 by including items 8-10. 8.Increase of >2mm in proptosis 9.Decrease in uniocular excursion in any one direction of >8 deg. 10.Decrease of acuity equivalent to 1 snellen line.
  • 29. Evaluation of thyroid eye disease •Clinical examination  Corrected distance visual acuity  Colour vision  Pupillary examination  Ocular motility  Hertel exophthalmometry  IOP(in primary gaze and upgaze)  Adnexal examination  Slit lamp examination  Dilated fundus examination •Laboratory studies • T3/free T4/TPO/TRAb/TSH/TSI •Imaging studies • Orbital untrasonography(assesment of extraocular muscle size and reflectivity) • CT/MRI
  • 32. MANAGEMENT OF TED • Stepwise and graded approach • Based on pts-reported symptoms,clinical examination and ancillary testing. • According to the clinical activity score(CAS), TED categories into 4 stages. A. Mild disease-<3 score B. Moderate disease >/=4 score C. Severe disease D. Refractory disease
  • 33. Mild disease • Observaton • Pts education and lifestyle changes • Smoking cessation • Salt restriction • Elevation of head of bed • Use of sunglasses • Ocular surface lubrication • Establishment of a euthyroid state • Oral seleniun
  • 34. Moderate disease • Topical cyclosporine • Eyelid tapering at night • Moisture goggles or chambers • Prism glasses or selective ocular patching • Moderate dose oral steroid therapy
  • 35. Severe disease • High dose intravenous steroid therapy • Surgical orbital decompresion(followed by strabismus surgery and/or eyelid surgery) • Periocular radiotherapy
  • 36. Refractory disease • Steroid sparing immunomodulators • Eg:rituximab,others