Thyroid eye disease (TED) is an autoimmune inflammatory disorder affecting the eye muscles and surrounding tissues. It is commonly associated with Graves' disease. Symptoms include eye pain, swelling of eyelids, and issues with eye movement. Examination may reveal eyelid retraction, proptosis, and restrictive myopathy. Management involves medications like steroids to reduce inflammation during active phases, with surgery to correct eye muscle issues and proptosis during inactive phases. The goal is to improve symptoms, eye health, and appearance.

1. THYROID EYE
DISEASE
MODERATOR : DR RASHMI G
ASSOCIATE PROFESSOR
DEPARTMENT OF OPHTHALMOLOGY
PRESENTOR : DR SRI ARCHANA
JUNIOR RESIDENT
DEPARTMENT OF OPHTHALMOLOGY

2. INTRODUCTION
• Thyroid eye disease (TED) is an autoimmune inflammatory
disorder .
• It is also called as Thyroid orbitopathy, Thyroid associated
ophthalmopathy (TAO), Thyroid ophthalmopathy
• It is the commonest cause of both unilateral and bilateral
proptosis in adults

3. RISK FACTORS
 Fourth to fifth decade
 Female to Maleratio4:1
 Cigarette smoking ( modifiable risk factor)
 Treatment with radioactive iodine

4. ETIOLOGY
 Graves’ disease (90%)
 Euthyroidism (6%)
 Hyperthyroidism – other causes
(Toxic nodular goiter, Hashimoto’s thyroiditis (3%),
Factitious hyperthyroidism, Thyroid malignancy)
 Hypothyroidism (1%)

5. PATHOGENISIS
 Thyroid eye disease is an organ specific autoimmune-
mediated inflammation of the extraocular muscle and
periorbital connective tissue.
 Target antigen is shared between thyroid follicular
cells and orbital fibroblast
 Orbital fibroblast are the primary target of
inflammatory attack

6. Lymphocytic infiltration of orbital tissue
Release of cytokines (IL – 1)
Stimulation of fibroblasts, Proinflamatory genes
upregualted(IL6,IL8,PGE2)
Synthesis of Glicosaminoglycans(GAG)
Hyaluronan attracts water, GAG accumulates in fatty
connective tissue
Increase in osmotic load
Muscle odema, Proptosis, Fibrosis of muscle fibres,
Atrophy

7. PATHOGENISIS

8. GROSS PATHOLOGY
EOM
enlargement
confined to
bellies and
sparing
tendons

9. HISTOPATHOLOGY
The extraocular muscle fibres are separated by an
accumulation of granular material consisting of collagen
fibrils and glycosaminoglycans, among which hyaluronan
predominates
Focal and perivascular inflammatory
mononuclear cell
Muscle replaced by fibrous connective
tissue with a scattering of mononuclear
inflammatory cells in late disease

10. CLINICAL PRESENTATION
• Retrobulbar discomfort
• Grittiness
• Red eyes
• Lacrimation
• Photophobia
• Puffy lids

11. EYE SIGNS
FACIAL SIGN:
 Joffroy’s sign—Absent creases in the forehead on
superior gaze.
JOFFROY’S SIGN

12. EYELID SIGNS
• Kocher’s sign—Staring appearance
• Vigouroux sign—Eyelid fullness or puffiness
• Rosenbach’s sign—Tremors of eyelids (when closed)
• Riesman’s sign—Bruit over the eyelids
KOCHER’S SIGN VIGOUROUX SIGN

13. UPPER EYE LID SIGNS
• Von Graefe’s sign—Upper lid lag on downgaze
• Dalrymple’s sign—Upper eyelid retraction
• Stellwag’s sign—Incomplete and infrequent blinking
• Grove sign—Resistance to pulling the retracted upper lid
VON GRAEFE’S SIGN DALRYMPLE’S SIGN

14. • Boston sign—Uneven, jerky movements of the upper lid on
inferior gaze
• Gellinek’s sign—Abnormal pigmentation of the upper lid
• Gifford’s sign—Difficulty in everting the upper lid
• Means sign—Increase superior scleral show on upgaze
GELLINEK’S SIGN GIFFORD’S SIGN

15. LOWER EYE LID SIGNS
• Enroth’s sign—Edema of the lower lid
• Griffith’s sign—Lower lid lag on upward gaze.
ENROTH’S SIGN

16. EXTRAOCULAR MOVEMENT SIGNS
• Moebius’ sign—Unable to converge eyes
• Ballet’s sign—Restriction of one or more extra ocular muscle
• Suker’s sign—Poor fixation on abduction
• Jendrassik’s sign—Paralysis of all EOMs.
MOEBIUS’ SIGN BALLET’S SIGN

17. CONJUNCTIVAL SIGNS
• Goldzeiher’s sign—Conjunctival injection.
PUPILLARY INVOLVEMENT SIGNS
• Knies’ sign—Uneven pupillary dilatation in dim light
• Cowen’s sign—Jerky contraction of pupil to light.
KNIES’ SIGN

18. Soft tissue involvement
Periorbital and lid swelling
Chemosis
Conjunctival hyperaemia
Superior limbic
keratoconjunctivitis

19. EYELID SIGNS
 Eyelid retraction (mc clinical
feature)
 Retraction due to
- Sympathetic overstimulation of
Muller’s muscle
- Fibrotic contracture of the
levator palpebrae and inferior
rectus muscles
- Secondary overaction in
response to hypo- or
hypertropia produced by
fibrosis.

20. Marginal reflex distance (MRD)
MEASURMENT OF LID RETRACTION

21. PROPTOSIS
• Occurs in about 50%
• Axial, unilateral/bilateral
•Symmetrical or asymmetrical
•Patients < 40 years of age are
more likely to exhibit proptosis
related to fat expansion in the
apparent absence of muscle
involvement

22. PLASTIC RULER
LUEDDE’S EXOPHTHALMOMETER
HERTEL’S EXOPHTHALMOMETER
CLINICAL METHODS FOR MEASUREMENT OF PROPTOSIS
NAUGLE’S EXOPHTHALMOMTER

23. RESTRICTIVE MYOPATHY
 30% to 50% of cases
 Ocular motility initially
restricted by inflammatory
edema and later by fibrosis
 Involvement of the inferior,
medial, superior, and lateral
rectus muscle are
encountered in decreasing
frequency

24. RESTRICTIVE MYOPATHY
Elevation defect Abduction defect
Depression defect Adduction defect

25. OPTIC NEUROPATHY
 5% of cases
 Compression of optic nerve or
its blood supply at the orbital
apex by congested and enlarged
recti and swollen orbital tissue
 Visual acuity is reduced
 Colour desaturation
 Visual Field defects : central and
paracentral scotoma

26. GRADING SEVERITY
Werner’s Classification:
 Class 0- No signs and symptoms
 Class 1- Only signs
 Class 2- Soft tissue involvement
 Class 3- Proptosis
 Class 4- EOM involvement
 Class 5- Corneal involvement
 Class 6- Sight loss due to optic neuropathy
 It is not useful for determining the severity of the disease,
for prognostication, or for planning management.

27. VISA CLASSIFICATION
 It has been proposed for classifying severity of disease
 Each category has subjective and objective items, and
each category is scored both by the clinician and the
patient
 The management is decided according to the score in each
category
V—Vision, and Assessment of Optic Neuropathy
I—Inflammation
S—Strabismus
A—Appearance (Proptosis, Lid Retraction) and Exposure

28. CLINICAL ACTIVITY SCORE (CAS)
 Described to measure the activity of disease at a given
time.
 Total score is noted by giving a score of 1 for each of the
following features
 CAS >= 3/10 suggests active TED

29. EUGOGO CLASSIFICATION
 Severity of the Ophthalmopathy.
 Management depends on degree of severity of the
ophthalmopathy
 The disease is classified as
1. Mild TED: characteristics of TED have a minimum impact
on the patient's life
present with one or more of the following signs:
• Minor lid retraction ( <2 mm).
• Mild soft tissue involvement.

30.  Exophthalmos <3 mm
 Transient or no diplopia.
 Corneal exposure responsive to lubricants.
2. Moderate-to-severe TED :
- Patients without sight-threatening TED
- Eye disease has sufficient impact on daily life to justify the
risks of immunosuppression ( if active) or surgical
intervention (if inactive).
Patients usually present one or more of the following signs:
• Lid retraction (>2mm).
• Moderate or severe soft tissue involvement.

31.  Exophthalmos >3 mm
• Inconstant, or constant diplopia.
3 Sight threatening TED: patients with
• Dysthyroid optic neuropathy
• Corneal breakdown due to severe exposure
• Severe forms of frozen eye
• Choroidal folds
This category warrants immediate intervention .

32. CLINICAL COURSE
 TED is a self-limiting disease, becoming quiescent within
3–5 years of its onset
1. Congestive or Active phase :
- Lasts for 6-18 months
- Characterized by active inflammation with marked lid
edema, conjunctival chemosis and congestion and
increasing exophthalmos
2. Static phase :
majority of cases, starts to regress and most of the signs
slowly settle down.

33. 3. Fibrotic or Inactive phase:
- Also known as quiescent or burnt-out phase, ensues after
the regression phase.
- In this phase, eyes are white, however, a painless motility
defect may be present.

34. INVESTIGATION
Biochemical Profile
 Thyroid stimulating hormone
 Free and total triiodothyronine
 Free and total thyroxine
 Thyroid hormone binding ratio (THBR)
 Radioiodine uptake (RAIU)
Imaging
 Magnetic resonance imaging
 Computed tomography scanning
 Ultrasonography

35. CT ORBIT
 CT is helpful in assessing the relationship between the EOM
and optic nerve at the orbital apex and can aid in surgical
planning
 Allows identification of even minimally enlarged recti
 There is bilateral fusiform enlargement of the extra-ocular
muscles, with smooth borders and sparing of the tendons,
is the classic finding ( Coca-cola sign)

36. CT ORBIT
Coca-cola sign

37. MRI ORBIT
•MRI helps to identify the typical fusiform rectus muscle
enlargement and orbital fat expansion
•Helps in early diagnosis of compressive optic neuropathy

38. USG B - SCAN
•B scan can demonstrate inflammatory changes which occur
in orbital fat and extraocular muscles

39. MANAGEMENT
Management of thyroid eye disease includes :
 Supportive therapy during the active phase
 Management of sight-threatening conditions in severe
disease
 Functional and cosmetic rehabilitation during the
quiescent phase

40. MANAGEMENT PLAN FOR THYROID EYE
DISEASE
 Mild to moderate disease, within first year of onset:
- Stop smoking
- Lubricants
- Elevate head end of bed
- Cool compresses
 Severe sight threatening disorder at any point (optic
neuropathy, exposure keratitis):
- Systemic steroids–methylprednisolone
- Tarsorrhaphy or induction of ptosis with botulinum toxin.

41.  If severe disease is non-responsive to medical therapy
- Surgical decompression, orbital radiotherapy.
 Moderate or severe disease, stable, inactive is treated
with surgical correction.
- Orbital decompression
- Extraocular muscle surgery
- Lid correction.

42. Use of steroids:
 Benefit from intravenous methyl-prednisolone, 1 gm per
day for three days.
 A recurrent disease may need pulse doses of steroids
every three to six weeks.
 Less severe disease may respond to oral steroids(
Prednisone 1mg/kg/day then taper )
 Intra-orbital injection of long-acting steroid such as
Triamcinolone 40 mg may have some beneficial effect.

43. Use of immunosuppressants:
 Patients not responding to steroids, or intolerant of
steroids
- Methotrexate (7.5–15 mg one day a week)
- Azathioprine (initial dose 1.0 mg/kg/day, increased weekly
till minimum effective dose is achieved)
- Cyclophosphamide (0.1–0.2 mg/kg/day).
 These drugs have serious side effects, and need to be
used with careful monitoring under supervision

44. Radiotherapy:
 A dose of 2000 cGy used
 It may act by decreasing the T lymphocyte
population in the orbit.
 Radiation is contraindicated in diabetic patients, as it
can adversely affect the retinopathy.

45. SURGICAL MANAGEMENT
Orbital decompression
Extraocular muscle surgery
Eyelid surgery

46. ORBITAL DECOMPRESSION
 Expands the orbital volume to create more space for
swollen soft tissues
 Primary indications for orbital decompression
- Compressive optic neuropathy
- Excessive proptosis (can manifest as globe subluxation,
corneal ulceration, and cosmetic disfigurement)
- Steroid dependence
- Intractable pain

47.  Types
◦ Two – wall : floor and medial wall (3–7 mm)
◦ Three – wall : floor, medial and lateral wall (6–10 mm)
◦ Four – wall : floor, medial and lateral walls with part of
sphenoid (apex) and lateral part orbital roof (10–17 mm)

48. Orbital Decompression

49. STRABISMUS SURGERY
 Goal of strabismus treatment is to achieve single binocular
vision in primary and downgaze positions
 TED quiescent with stable eye muscle function without
corticosteroids for at least 6 months
 The most frequently performed surgical procedure
strabismus is recession of the restricted medial rectus or
inferior rectus muscle

50. EYELID SURGERY
 Lagophthalmos can be treated
in the active phase by
Botulinum toxin injection
 In the stable phase, levator
recession, or lid lengthening by
use of spacers
 Indications for repair
-ocular discomfort
-keratitis,
-corneal ulceration
-cosmesis.

51.  Eyelid surgery should be undertaken after orbital
decompression and extraocular muscle surgery, because
both procedures can affect eyelid position
 Upper eyelid retraction is treated by recessing Müller’s
muscle and/or the levator aponeurosis.
 Lower eyelid retraction and lower eyelid entropion are
treated by recessing the lower eyelid retractors

52. REFERENCES
 Principle and practice of ophthalmology by Albert and
Jakobie’s Volume 3 , Edition 3
 Comprehensive Ophthalmology by A K Khurana, 7th
Edition
 Kanski’s Clinical Ophthalmology 9th edition
 Postgraduate Ophthalmology by Zia Chaudhuri, First
Edition

53. THANK YOU