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1. Thyroid Ophthalmopathy
Bipin Koirala
Master’s of Optometry & Vision Science
Himalaya Eye Institute

2. Contents
Introduction
Pathophysiology
Sign and symptoms
Treatment
Case report
References

3. Definition
Thyroid eye disease (TED) is an autoimmune disease
caused by the activation of orbital fibroblasts by
autoantibodies directed against thyroid receptors.
TED is a rare disease, which had an incidence rate of
approximately 19 in 100,000 people per year .

4. Introduction
These changes have also been called as :
Endocrine Exophthalmos
Malignant Exophthalmos
Dysthyroid Ophthalmopathy
Ocular Graves’ Disease (OGD)
Thyroid Eye Disease (TED)

5. Introduction
Incidence of Thyroid Eye Disease depending on thyroid
disorder
Hyperthyroidism-90%
Primary hypothyroidism-1%
Hashimoto thyroiditis-3%
Euthyroid -6%

6. Thyroid gland
Location: Anterior neck beneath the larynx
Endocrine gland and highly vascular and supplied by
autonomic nervous system.
Has over a million follicles that secrete thyroglobulin, a
precursor of the thyroid hormones, On activation the
thyroglobulin is re-absorbed, converted into the
thyroid hormones triiodothyronine (T3) and thyroxine
(T4),

7. Thyroid
Negative feed back
mechanism
The production of thyroid
hormones is regulated by
both the hypothalamus and
the pituitary gland.

8. Hyper Thyroid
Hyperthyroidism is a condition that results from an over-
secretion of thyroid hormones.
It is usually caused by an autoimmune disease known as
Graves’ disease,
Common causes :
Thyroiditis
Toxic adenomas,
Toxic nodular/ multinodular goiters
Thyroxine overdoses
Excess iodine dietary intake.

9. Hypo Thyroid
Hypothyroidism is due to an underactive thyroid gland and
results in a deficiency of thyroid hormones.
Thyroid hormones are essential for normal development
between birth and puberty and a deficiency causes
irreversible mental retardation.
Hypothyroidism can be caused by Hashimoto’s thyroidism,

10. Thyroid test chart
mm

11. Hyperthyroidism vs Hypothyroidsm
mm

12. Pathophysiology
TED being an autoimmune disease with orbital fibroblast as the
primary target inflammatory attack and the EOM are secondarily
involved
Also the target antigen is shared b/w thyroid follicular cells and
orbital fibroblast
The activated T cells acts on fibroblast – adipocyte lineage with in
orbit and will stimulate adipogenesis, fibroblast proliferation and
GAG synthesis as a result of which EOM are enlarged due to
infiltration and odema

13. Pathophysiology
There is an increase in the volume of the orbital contents,
particularly the muscles, which can swell to eight times their
normal size.
There may be a secondary elevation of intraorbital pressure, and
the optic nerve may be compressed.
Later muscle fibres eventually leads to fibrosis, which exerts a
tethering effect on the involved muscle, resulting in restrictive
myopathy and diplopia

14. Pathophysiology

15. Common Risk Factors
Female (4-6 times) and middle aged
Smoking (no of cigarettes per day)
H/O auto immune thyroid disorder
HLA-DR3 and HLA-B8 positive
Genetic predispsition
Life style

16. Symptoms : Ocular
Dry eyes
Ocular redness and irritation
Reduced vision
Bulged eye
Diplopia
Eyelid odema
Pain and pressure around eye
Field loss
Dyschromatopsia

17. Clinical features (lid signs)
1. Upper lid retraction (Dalrymple’s Sign)
Seen in 90% cases
Gives frightened and staring looks

18. Clinical features (lid signs)
G
2. Lid lag ( Von Graefe’s sign)
Seen in 50 % cases
Upper lid lags behind during down gaze.

19. Clinical features (lid signs)
3. Fullness of lid (Enroth’s Sign)
Puffy odematous swelling

20. Clinical features (lid signs)
4. Difficulty in Lid Eversion (Gifford’s Sign)
 Due to odematous lid swelling

21. Clinical features (lid signs)
4. Infrequent / Incomplete blinking
(Stellwag’s Sign)
5. Presence of Glabellar furrow’s

22. Clinical Features (Lid Signs)
1. Rosenbach's Sign: Fine tremors of the eyelids when closed.
2. Boston’s Sign: Jerky irregular movement of upper lid on
downward gaze.
3. Kocher’s Sign: Increased lid retraction with visual fixation
4. Abadie Sign: Spasm of the levator palpebrae superioris
muscle with retraction of the upper lid.
5. Riesman's sign: Bruit heard over the closed eye with a
stethoscope.

23. Clinical features (Conjunctiva)
Chemosis and Deep Injection of Conjunctiva
(Goldzeiher’s Sign)

24. Clinical features (Pupil)
In equality of dilation of pupil
Other Pupillary Signs:
1. Cowen’s Sign: Jerky pupillary constriction to
light.
2. Lowy’s Sign: Mydriasis with instillation of lower
concentration of adrenaline.
3. Knies’s sign: Unequal dilatation of pupil in dim
light.

25. Clinical features (EOM)
1. Möbius Sign: Inability to converge.
2. Ballet Sign: Restriction of one or more
extraocular muscles. (40 %)
3. Jendrassik's Sign: Limitation of abduction and
rotation of eyeballs.
4. Suker’s Sign: Poor fixation on abduction.

26. Clinical features (EOM)
Question 1:
What is the most commonly and initially
affected EOM??
Answer:
Inferior Rectus (Elevation defect is seen)
Medial Rectus ( Defective abduction)
@ IMSL

27. Clinical features (Facial)
1. Joffroy Sign: Absent creases in the forehead on
superior gaze.
2. Jellinek's Sign: Hyperpigmentation of the superior
eye folds.
3. Hertoge’s Sign: Loss of the lateral third of eyebrows
4. Sainton’s sign: Delayed forehead wrinkling on up
gaze.

28. Which sign is shown in clinical photo?
Answer: Joffroy Sign

29. Exophthalmos
Common Sign
Seen in 60 % cases
Bilateral (can be unequal)
Self limiting
Decreased orbital
retropulsion

30. Exophthalmos
Common Sign
Seen in 60 % cases
Bilateral (can be unequal)
Self limiting
Decreased orbital
retropulsion

31. Exposure Keratopathy and Discomfort
Gritty sensation/ lacrimation
Photophobia
Corneal exposure a/w Lagopthamos, Proptosis, Reduced blink

32. Optic Neuropathy
Seen in 60 % cases
Mainly caused due to compression of Optic nerve by enlarged
EOM orbital contents esp at Orbital apex
A/w vision loss, RAPD, Defective color vision, slow progressing
optic atrophy

33. Rare Associations
Strabismus due to muscle fibrosis
Glaucoma
Raised IOP in up gaze
Increased Episcleral Venous pressure
Increased Mucopolysaccharide deposit with in Aq. Outflow
system

34. Differential Diagnosis
Orbital or Preseptal cellulitis
Carotid-cavernous fistula
Idiopathic orbital inflammation (Pseudotumour)
TED must be ruled out in all cases of B/L proptosis

35. Disease Course and Phases
The pattern of the disease follow the Rundle’s curve which
describe the plot of orbital disease severity against time
1.Initial phase- inflammatory phase duration may last from 6 –
18 months with orbital and periorbital signs i.e. proptosis and
retraction.
2.Static phase- decrease in the inflammatory phase and
minimal improvement.
3.Quiescent phase- gradual improvement with improved
motility and retraction of the muscles

36. Disease Course (Rundle’s Curve)

37. Classification (NOSPECS) by ATA
ATA has classified Graves’ ophthalmopathy, irrespective of the
hormonal status into following classes

38. Classification (CAS) score

39. Classification (EUGOGO)

40. Investigations
Laboratory test
Thyroid hormone levels (T3, T4, TSH)
Anti thyroid antibodies.

41. Investigations (IMAGING)
USG (B SCAN)
To visualize the orbital structures and determining recti muscle
enlargement.
low cost, lack of ionizing radiation and relatively short
examination time
CT/MRI
Detects proptosis, muscle thickness, thickening of optic nerve
and anterior prolapse of the orbital septum (due to excessive
orbital fat and/or muscle swelling), erosion of wall of orbit,

42. Investigations (IMAGING)
USG (B SCAN)
CT/MRI

43. What is Coca Cola bottle sign??

44. Investigations (positional Tonometry)
Tonometry in primary gaze and upgaze is performed
An increase in intra-ocular pressure in upgaze helps in
diagnosis of subclinical cases.

45. Basic Investigations
1. Corrected visual acuity and Refraction
2. Color vision testing
3. Examination for an afferent pupillary defect
4. Extraocular muscle motility examination
5. Measurement of the lid fissure height in primary
gaze

46. Basic Investigations
1. Measurement of upper and lower scleral show,
2. Exophthalmometry to detect proptosis
3. Slit-lamp biomicroscopy to assess the tear film and
fluorescein pattern
4. Fundus examination to detect optic disc swelling or
pallor.

47. Treatment
Treatment can be classified into that of
1. Mild disease (most patients),
2. Moderate to severe active disease,
3. Post inflammatory complications.
4. The first measure taken in all cases should be the
cessation of smoking.
5. Thyroid dysfunction should also be managed
adequately

48. Treatment (Mild disease)
1. Topical artificial tear drops in the day time and
ointment at bed time are useful for relief of ocular
surface drying
2. Head elevation with three pillows during sleep to
reduce periorbital oedema.
3. Eyelid taping during sleep may alleviate mild
exposure keratopathy.
4. Oral NSAIDs may be used for periocular pain.

49. Treatment (Optometry point of view)
1. For diplopia, Fresnel prisms or occlusion therapy
may be considered.
2. Prism for restricted ocular motility
3. Base in prism for reduced convergence
4. Others are lifestyle modifications e.g. sodium
restriction to reduce water retention and tissue
edema

50. Treatment (Moderate to Severe disease)
1. Guanethidine 5% eyedrops may decrease the lid
retraction caused by overaction of Muller’s muscle.
2. Systemic steroids may be indicated in acutely
inflamed orbit with rapidly progressive chemosis
and proptosis with or without optic neuropathy
3. Radiotherapy (2000 rads given over 10 days period).
It may help in reducing orbital oedema in patients
where steroids are contraindicated

51. Treatment (Moderate to Severe disease)
5. Lateral tarsorrhaphy
Performed in patients with exposure keratopathy (with
mild to moderate proptosis) not responding to topical
artificial tears
6. Extraocular muscle surgery.
It should be carried out for left-out diplopia in primary
gaze, after the congestive phase of disease is over and
the angle of deviation is constant for the last 6
months.

52. Treatment (Post complications)
7. Orbital wall decompression
It involves the orbital floor, medial wall, and lateral
wall. In rare cases the roof of the orbit may also be
decompressed surgically.

53. PATIENT’S DETAIL
• Name: SHANTI SHARMA
• Age: 33 years Sex: Female
• Occupation: House wife
• Mode Of Presentation :
Regular eye checkup
56

54. HISTORY
CHIEF COMPLAINT(S):
• Grittiness, watering and burning Sensation
• Symoptomatic during windy days
57

55. HISTORY OF PRESENT ILLNESS
• No h/o of eyeball pain
• No h/o of photophobia
• No h/o of diplopia or polyopia and shadowing of
objects (ghost image)
• No h/o of itching of eye(s)
58

56. PAST- OCULAR HISTORY
• H/O using glasses since 1 year with
Rx
OD: -0.50 Dsph with 6/6̊
OS: - 0.50 Dsph with 6/6̊
• No other h/o of past ocular diseases and surgery
• Using tear substitute for past 1 yr
59

57. PAST- SYSTEMIC HISTORY
• No h/o of Diabetes Mellitus / Hypertension
• H/0 hypothyroidism +
• No h/o of surgical intervention for any disease
• No h/o of any other systemic illness in past
• h/o of use of systemic medication for hypothyroidism for 6 months
• Tab. Thyronorm 25 mcg - OD
60

58. PERSONAL HISTORY
• Non vegetarian by diet
• Does not consume alcohol and smoke
• House wife by proffesion
61

59. FAMILY HISTORY
• No any systemic and ocular diseases in other family
members
• No family members being syndromic or with inherited
abnormalities
60 65
33 25
62

60. General Physical Examination
• Average built, well nourished
• Weight – 54kg Height – 1.5m BMI - (21.10kg/m2)
Vitals
• Blood Pressure = 120/80 mmHg (Left arm, sitting position)
• Pulse = 82 beats per min, regular.
• Respiration rate= 18/min, regular
• Temperature: 98.6⁰F
General Examination
• No pallor, icterus, clubbing, cyanosis, lymphadenopathy or
edema. 63

61. OCULAR EXAMINATION
On the day of presentation:
RE VISUAL ACUITY LE
6/9 p UNAIDED 6/9 p
6/6 AIDED 6/6
6/6 PH 6/6
64

62. OCULAR EXAMINATION
Normal Head Posture Normal
Normal Forehead &
Eyebrows
Normal
EOM
+
+
+ +
+
+
+
+
+ +
+
+
65

63. OCULAR EXAMINATION
Puffy L. lids/Normal
Clear/Poor tear film
Eyelids / Lashes
Cornea/Tear flim
Puffy L. lids/ Normal
Clear/ Poor tear film
RIGHT EYE LEFT EYE
66

64. OCULAR EXAMINATION

65. Normal
(No congestion,
chemosis, papillary
reaction or follicles)
Conjunctiva Normal
(No congestion, chemosis,
papillary reaction or
follicles)
Normal Episclera
&
Sclera
Normal
RIGHT EYE LEFT EYE
68

66. Normal in color and
pattern
Round/Regular/Reactive
(~3.0mm)
(Direct & consensual)
Iris
Pupil
Normal in color and
pattern
Round/Regular/Reactive
(~3.0mm)
(Direct & consensual)
RIGHT EYE LEFT EYE
69

67. Normal Depth
Quiet
ANTERIOR
CHAMBER
Normal Depth
Quiet
Clear Lens Clear
Clear Vitreous Clear
RIGHT EYE LEFT EYE
70

68. Round, Pink with
sharp margin
Optic Disc Round, Pink with
sharp margin
0.2:1 Cup: Disc Ratio 0.2:1
Healthy with GFR Macula Healthy
A:V=2:3 Vessels A:V=2:3
RIGHT EYE LEFT EYE
71

69. EXAMINATIONS
10mm wet in 5
minutes
Schirmer tear
test I
10mm wet in 5
minutes
5 sec spot noted Invasive TBUT 5 sec spot noted
Normal (> 2/3 rd
cornea hidden)
Bells phenomenon Normal (> 2/3 rd
cornea hidden)
18 Hertels (97) 18
No lid lag/ no
lagophthalmos
Lid lag/
lagopthalmos
No lid lag/ no
lagophthalmos
RIGHT EYE LEFT EYE

71. TFT EXAMINATIONS
4.6 pmol/L T3 4.8 pmol/L
17.4 pmol/L T4 17 pmol/L
8 mU/L TSH 4.0 mU/L
BEFORE (6 MONTHS AGO) AFTER (PRESENT)

72. Provisional Diagnosis
• No thyroid ophthalmopathy at present
• Dry eyes
Treatment/ Suggestions
• Gtt. Optive – OU- 1dp – 4hrly- 4 weeks
• FOLLOW UP 6 months with TFT monitor

73. References
• Kanski’s clinical ophthalmology
• AK khurana ophthalmology
• Eye wiki AAO article
• Any queries???