THYROID EYE DISEASE

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1 THYRIOD EYE DISEASE Dr. Gyanendra Lamichhane Lumbini Eye Institute Bhairahawa ,Nepal

References : 

2 References Text Book of Ophthalmology: Myron Yanoff, 2nd edition American Academy of Ophthalmology-Orbits, Eyelids, and Lacrimal system (2004-05) Duane’s ophthalmology,CD-ROM 2005 Albert and Jacobeic, CD-ROM 2003 Clinical Ophthalmology :Jack J. Kanski,5th Edition Text book Of Ophthalmology-Peymen Various Journals

Presentation layout : 

3 Presentation layout History Introduction Pathogenesis Clinical manifestations of thyroid eye disease Classification ( NOSPECS) Investigations for thyroid eye disease Treatment

History : 

4 History Earliest descriptions of hyperthyroid patients with congestive exophthalmos and infiltrative dermopathy published by: Caleb Hillier Parry in 1825; Robert James Graves in 1835 and Carl von Basedow in 1840. Parry Graves

Introduction: : 

5 Introduction: Various names Dysthyroid ophthalmopathy Thyroid orbitopathy Graves’ ophthalmopathy Endocrine ophthalmopathy Thyrotoxic exophthalmos Thyroid associated orbitopathy (TAO)

Introduction ( contd…) : 

6 Introduction ( contd…) Organ-specific autoimmune process -strongly associated with dysthyroidism. Thyroid status in TAO Grave hyperthyroidism-90% Euthyriod-6% Hashimoto thyroiditis-3% Primary hypothyrioidism-1%

Introduction (contd..) : 

7 Introduction (contd..) Both Graves' and thyroid eye disease more common in women -female to male ratio is 6:1. Clinically apparent in approximately 50% of patients with Graves' disease Requires intensive treatment or surgical intervention in only 3 to 5% of cases. Mostly affects patients aged 30-50 years.

Introduction ( contd..) : 

8 Introduction ( contd..) Two age peaks of incidence -5th and 7th decades of life. ( seen 8-80yrs) …..AAO 2006 May precede, coincide, or follow the systemic complications of dysthyroidism. Affects within 1 yr of development of hyperthyroidism Progressive but self-limiting within 3-5 years.

Introduction( contd..) : 

9 Introduction( contd..) In the United States: Incidence approximately 0.4%. 16 cases per 100,000 population per year-women and 2.9 cases per 100,000 population per year for men. In a British population: Prevalence about 2%, Incidence 3 cases per 1000 annually. Female: male -4:1

Introduction ( contd..) : 

10 Introduction ( contd..) Familial tendency with family history of thyroid disease in approximately 30% of cases. 30 to 60% of monozygotic twins develop Graves' disease when the other twin is affected. Increased risk of Graves' ophthalmopathy associated with cigarette smoking ( twice more in smokers)

Smoking : 

11 Smoking TWO hypothesis Smokers with Graves' disease have lower concentration of soluble IL- 1 receptor antagonist than do non-smokers Decreased inhibition of proinflammatory and fibrogenic effects of IL- 1 Decrease immunosuppresion Increase autoimmunity

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12 SMOKING thiocyanate expression nicotine Orbital hypoxia adrenergic stimulation of thyroid direct irritative effect localized circulatory change thyroglobulin release of thyroid antigen TAO

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13 Association : ( PARDIM) Pernicious anaemia Addison's disease Rheumatoid arthritis Diabetes mellitus Idiopathic thrombocytopenic purpura Myasthenia gravis

Pathophysiology : 

14 Pathophysiology An autoimmune-mediated inflammation of the extra ocular muscle and periorbital connective tissue. (Type II Hypersensitivity- ADCC ) ( Type V –stimulatory hypersensitivity ) Complex immunological process involving: TSH-receptor as the antigen,( Thyroid ,fibroblast and adipocytes) Fibroblast and adipocytes as the effector cells, Cytokine mediated immunologic response.

Pathophysiology : 

15 Pathophysiology Autoantibody against TSH receptor( TSH-R) in the Thyroid Cross react with TSH-R in effector cells i.e. fibroblast ,adipocytes and interstitial tissue between extra ocular muscles Inflammatory cellular infiltration ( T lymphocyte, plasma cells, macrophages and mast cells) in orbital fat,lacrimal gland and orbital interstitium

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16 Lymphocytes infiltrate in the orbit predominantly of CD4+ and CD8+ T- cells with a few B-cells. Interaction of CD4 T cells and fibroblast (orbital) Release of cytokines such as IL-1b,TNF-gamma,TGF-b Activate fibroblasts to secrete hyaluronic acid, a glycosaminoglycans. Osmotic imbibition of water ,EOM swollen upto 8 times Compressive optic neuropathy orbital fluid retention later Fibrosis of muscle intraorbital volume increased Restrictive myopathy venous obstruction Proptosis

Pathogenesis (Key Points): : 

17 Pathogenesis (Key Points): Autoimmune Disorder (IgG mediated) Enlargement of Extraocular Muscles by increase in glycosaminoglycans (GAG) Cellular Infiltration of Interstitial Tissues with lymphocytes, plasma cells, macrophages & mast cells Fibrosis Proliferation of Orbital Fat, Connective Tissue and Lacrimal Gland with retention of fluid & accumulation of GAG

Pathology : 

18 Pathology Characterized by inflammation and enlargement of orbital tissues, extra ocular muscles sparing tendon Gross examination -extra ocular muscles are enlarged, firm, rubbery, and dark red

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19 Interstitial edema Mononuclear inflammatory cellular infiltrate lymphocytes and plasma cells Normal looking muscle fiber Histopathology

Histological findings : 

20 Histological findings Progressive fibrosis late in the course of disease Intact muscle fiber Muscle replaced by fibrosis

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21 1. Acute, active, inflammatory ( congestive) Mediated predominantly by lymphocytes, fibroblasts bilateral eyelid retraction, conjunctival chemosis, exophthalmos, and optic nerve compromise. Responds to treatment

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22 2 )Chronic, stable ( Fibrotic) Hypertrophy and fibrosis of extra ocular muscles Unresponsive to any suppressive treatment White eyes

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23 Congestive phase Fibrotic phase

SYMPTOMS(OCULAR) : 

24 SYMPTOMS(OCULAR) Ocular redness and irritation Decreased vision Proptosis Diplopia Eyelid swelling Eye pain Field loss Dyschromatopsia

Symptoms of hyperthyroidism : 

25 Symptoms of hyperthyroidism Tachycardia Palpitations Nervousness Diaphoresis Heat intolerance Skeletal muscle weakness Tremor Increased appetite Weight loss Hair loss Irritability Goiter

Symptoms of hypothyroidism : 

26 Symptoms of hypothyroidism Bradycardia Drowsiness Poor mentation Muscle cramps Weight gain Dry skin Husky voice Depression Cold intolerance

Ocular Signs(PECLO) : 

27 Ocular Signs(PECLO) 1) Proptosis ( 60%) 2) Extra ocular motility disturbance (40%) 3) Eye-Lid signs (retraction-90%) 4) Conjuctival/corneal signs 5) Optic neuropathy ( 5%) All signs together- 5%

Proptosis : 

28 Proptosis Most common cause of U/L and B/L proptosis in adults / axial and frequently permanent. Unilateral proptosis reflects asymmetric muscle involvement. Decreased orbital retropulsion. Below 40- due to adipogenesis Above 70- due to muscle enlargement and minimal adipogenesis

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29 Axial and permanent in 70% May be associated with Choroidal folds

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30

Extra ocular motility dysfunction : 

31 Extra ocular motility dysfunction 30-50% cases, permanent Ballet sign (restriction of one or more extra ocular muscles) Initially due to edema , later fibrosis All 4 recti are involved but mainly IR and MR

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32 Restrictive myopathy Elevation defect Abduction defect Depression defect Adduction defect

Signs : 

33 Signs Mobius sign (poor convergence)

Eyelid signs : 

34 Eyelid signs More than 26 signs are described Upper lid retraction (Dalrymple sign) -90% with temporal flare

Mechanisms for upper lid retraction : 

35 Mechanisms for upper lid retraction Up gaze restriction Proptosis Fibrotic contracture of LPS Secondary over action of LPS-SR complex Muller muscle over action

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36 Signs of eyelid retraction Bilateral lid retraction No associated proptosis Bilateral lid retraction Bilateral proptosis Lid lag in downgaze Unilateral lid retraction Unilateral proptosis

Lid lag on down gaze (von Graefe’s sign) : 

37 Lid lag on down gaze (von Graefe’s sign)

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38 Glabellar furrows Eyelid edema

Signs : 

39 Signs Stellwag sign (incomplete and infrequent blinking) Gifford’s sign (difficulty in upper lid eversion ) Goffroy sign (absent creases in the forehead on superior gaze) Enroth’s sign (eyelid fullness)

Signs : 

40 Signs Kocher’s sign Gifford’s sign

Conjunctival signs : 

41 Conjunctival signs Deep injection over insertions of the horizontal recti Chemosis Superior limbic keratoconjunctivitis

Signs : 

42 Signs Goldhziar’s sign

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43 Corneal signs

Optic neuropathy : 

44 Optic neuropathy (5%) optic disc oedema or optic atrophy due to direct compression of the nerve or it’s blood supply May occur in absence of significant proptosis

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45 Associated with: Vision loss, Visual field loss, Early defective color vision RAPD, Orbital congestion due to extra ocular muscle enlargement, and Proptosis.

Glaucoma : 

46 Glaucoma Mechanism of the increase in IOP Elevated episcleral venous pressure due to orbital congestion, Contraction of the extra ocular muscles& restrictive myopathy (intraocular pressure may rise more than 8 mm Hg on up gaze) Chronic exposure leading to chronic inflammation and secondary angle-closure Increased mucopolysaccharide deposit within the aqueous outflow system.

Strabismus : 

47 Strabismus Common presents as hypotropia or esotropia

Clinical signs of Graves' ophthalmopathy adopted by theAmerican Thyroid Association ( ATA) : 

48 Clinical signs of Graves' ophthalmopathy adopted by theAmerican Thyroid Association ( ATA)

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49 Clinical signs do not always progress in this sequence. Disease process asymmetric between the two orbits May undergo spontaneous exacerbations and remissions. Limited prognostic value.

Investigations : 

50 Investigations Lab Studies: Serum free thyroxine (FT4) Thyroid stimulating hormone (TSH) Anti-thyroid antibodies, e.g. anti-thyroid peroxidase (TPO) antibodies , TSH-R antibody Free triiodo-thyronine (FT3)

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51 Visual fields: Central scotoma or Inferior altitudinal defect in compressive optic neuropathy; Enlarged blind spot; Paracentral scotoma; Color vision:

Imaging Studies : 

52 Imaging Studies Ultrasound Changes in extra ocular muscles, Accentuation of retro bulbar fat and Perineural inflammation of optic nerve

CT/MRI : 

53 CT/MRI INDICATIONS- not needed in every cases 1) suspected optic neuropathy 2)before decompression 3) to exclude retro bulbar tumor as a cause of proptosis

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54 FINDINGS Fusiform thickening of muscles with tendon sparing. Proptosis Thickening of optic nerve Anterior prolapse of the orbital septum Lacrimal gland enlargement without orbital erosion

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55 Increase in orbital fat volume without muscle enlargement

Management : 

56 Management 1) general management of THYROID STATUS 2) Ocular Management i) proptosis ii) optic neuropathy iii) soft tissue and exposure keratopathy iv) lid retraction V) restrictive myopathy( strabismus)

TREATMENT : 

57 TREATMENT Management of THYROID STATUS Hyperthyroidism : Antithyroid drugs e.g. carbimazole , methimazole, radio iodine etc. Hypothyroidism: Thyroxine replacement to suppress TSH. Patients should stop smoking as this may worsen ophthalmopathy.

Proptosis management : 

58 Proptosis management 1) Steroid Oral steroid indications- congestive phase ( pain/rapid progressive) start with 60-80mg/day Reduction of S/S usually occurs within 48 hours Maximal response 2-8 weeks Discontinue after about 3 months though long term maintenance may be necessary N.B- No improvement after 3 week of oral steroid ---- stop it

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59 2) Intravenous Methyl prednisolone usually reserved for compressive neuropathy Dose- 0.5 -1 gm /d for 3-5 days followed by oral prednisolone 1mg/kg for several months. mechanism- suppression of fibroblast and lymphocytes that mediates inflammation immunosuppressant anti-inflammatory Reduces local inflammation and edema

RELATED STUDIES : 

60 RELATED STUDIES Ohtsuka et al treated 41 patients with intravenous methylprednisolone (1 g/day × 3 days) followed by 3-month oral prednisolone therapy. Result -improvement in muscle hypertrophy but minimal effect on ocular motility and no significant effect on proptosis ( Japanese journal of ophthalmology 2002)

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61 Macchia et al .compared 25 patients given biweekly intravenous injections of 1 g of methylprednisolone for 6 weeks, to 26 patients treated with oral prednisone for 4 to 6 months. Results- i) patients showed significant improvement in signs and symptoms of orbital inflammation and a “slight improvement in proptosis and diplopia .”in both cases ii) intravenous pulse therapy group required additional forms of therapy at 3 months less frequently than the oral steroid group ( ACTA Ophthalmology 2002)

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62 2)Radiotherapy indications- steroid ineffective or contraindicated cases Positive response is usually seen in 6 weeks and maximal improvement in 4 months

ORBITAL RADIATION THERAPY : 

63 ORBITAL RADIATION THERAPY Mechanism -Suppression of orbital lymphocytes and fibroblasts – LYMPHOLYSIS / GAG Reduces active orbit inflammation and congestion Minimal improvement of swollen extra ocular muscles and fat. A cobalt-60 unit delivers total dose of 2000 cGy radiation in 10 fractions over 2 wk.

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64 3) Combined therapy with Radiation .immunosuppressant and low dose prednisolone is more effective than any single modality. 4) Surgical decompression

Surgical Decompression : 

65 Surgical Decompression INDICATIONS- 1) Compressive optic neuropathy (2) Exposure keratopathy ( Severe) (3) Cosmesis

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66 Exophthalmos greater than 24 mm are often candidates for orbit decompression Surgery – i)Antral-ethmoidal decompression (two wall) Most common Approach-subciliary/transconjunctival affords 3-6 mm of retro placement of the globe FLOOR / MEDIAL WALL removed

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67 Three wall decompression FLOOR /MEDIAL/ LATERAL wall 6-10mm of retro displacement Four wall decompression three wall plus lateral half of orbital roof large portion of the sphenoid at the apex 10-16mm of retro displacement

Optic neuropathy management : 

68 Optic neuropathy management initially with intravenous methyl prednisolone if ineffective Orbital decompression as stated before

LOCAL TREATMENT MEASURES : 

69 LOCAL TREATMENT MEASURES For dry eye symptoms- Artificial tears during the day, Lubricating ointment at night, Sleeping with the head of the bed elevated -decrease morning lid edema. Taping eyelids closed at night Exposure keratopathy treat with artificial tear , ointment tarsorrhaphy orbital decompression if severe proptosis

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70 Diplopia management Active phase chronic phase Simple occlusion muscle surgery Prism (primary, reading)

Strabismus Management : 

71 Strabismus Management 1) Surgery Goal : To create adequate muscle balance for fusion of images in primary and reading positions To improve the noncomitant diplopia from restrictive myopathy of extra ocular muscles. Time of surgery disease is quiescent and the angle of deviation has been stable for at least 6 months Type of surgery recession of the Inferior and/or medial rectus with adjustable sutures that can be adjustable on first post.op.day 2) Botulinum toxin injection in the involved muscle

Eyelid Surgery : 

72 Eyelid Surgery Several surgical procedures are available to improve eyelid retraction, often in combinations. The options include: (1)Lateral tarsorrhaphy, (2) Muller's and levator muscle lengthening, (3) Lower eyelid elevation, (4)Blepharoplasty with orbital fat excision.

Lid retraction management : 

73 Lid retraction management Minor degree –not required Usual sequence of surgery is Orbital decompression strabismus surgery eyelid surgery

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74 Retracted eye lid Upper lid lower lid Mullerotomy IR recession (mild retraction) LPS recession

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75 Botulinum toxin treatment To produce partial temporary denervation of restricted muscles May have a limited role in the early course of Graves' disease.

Newer modalities of treatment : 

76 Newer modalities of treatment Bromocriptine: Dopamine receptor binding agent, Inhibition of TSH, Anti–T lymphocyte action

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77 Plasmapheresis, or plasma exchange therapy Removal of humoral factors in an autoimmune process May lead to clinical improvement Effective in acute forms of Graves' orbitopathy, Rebound is likely if plasma exchange is not followed with immunosuppressive therapy.

78 Somatostatin analogue- octreotide, lanreotide inhibit ILGF -1 in fibroblast decrease GAG synthesis Pantoxyphyllin- inhibit cytokine induced GAG synthesis by fibroblast.

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