Thyroid eye disease (Thyroid orbitopathy)
I. Describe the approach to establishing the diagnosis
A. Describe the etiology of this disease
1. Euthyroid or dysthyroid concurrent disease
2. Systemic autoimmune disease with potential target cells within orbit
B. Define the relevant aspects of epidemiology of the disease
1. Associated with other systemic autoimmune disorders, notably myasthenia gravis
2. Female predominance
3. Slight familial predominance
C. List the pertinent elements of the history
1. Progressive diplopia
2. Proptosis, lid retraction with apparent proptosis
3. Corneal symptoms
4. Complaints and findings may be variable
5. Insidious or rapid visual loss
6. May exhibit signs/symptoms of hypothyroidism/hyperthyroidism
7. Early morning swelling exacerbation (dependent orbital edema)
D. Describe pertinent clinical features
1. Orbital inflammatory signs
2. Eyelid lag, eyelid retraction, lagophthalmos
3. Exposure keratopathy
4. Restrictive strabismus (esotropia/hypotropia)
5. Proptosis (resistant retropulsion)
6. Elevated intraocular pressure (IOP)
7. Anterior and posterior forms with predominantly inflammatory disease, poorly recognized and often under diagnosed
8. Loss of vision without obvious external findings may exist
9. Pattern of extraocular muscle (EOM) enlargement: Inferior > Medial > Superior > Lateral
E. Describe appropriate laboratory testing for establishing the diagnosis
1. Computed tomography
2. Magnetic resonance imaging
3. B scan ultrasonography
4. Forced duction testing
5. Gaze evoked IOP elevation
6. Endocrine evaluation
II. Define the risk factors
A. Smoking
B. Other autoimmune diseases
C. Recent treatment with radioactive iodine for hyperthyroidism
III. List the differential diagnosis
A. Dural arteriovenous malformation
B. Carotid-cavernous fistula
C. Non-specific orbital inflammation
D. Orbital tumor (lymphoma/leukemic infiltrate/single or multiple metastasis)
E. Ocular myasthenia gravis (concurrence)
F. Restrictive strabismus
G. Orbital cellulitis
H. Isolated myositis
IV. Describe patient management in terms of treatment and follow-up
A. Describe medical therapy options
1. Endocrine consultation
2. Non-steroidal anti-inflammatory drugs
3. Corticosteroids — limit the use
4. Smoking cessation
5. Role of thyroid ablation and orbitopathy progression
6. Modalities to treat symptoms such as dry eyes, e.g., lubricants
B. Describe surgical therapy options
1. Orbital decompression for optic neuropathy
a. May be a role for orbital endoscopy
b. Indications for surgical decompression
i. Optic neuropathy
ii. Severe proptosis with exposure keratopathy
iii. Unacceptable cosmesis
c. Techniques
i. Decompression of orbital apex for compressive optic neuropathy
ii. Open vs. endoscopic
iii. Orbital fat decompression
d. Strabismus surgery when motility exam is stable
C. Radiation therapy
1. Indications are controversial
2. May have a role in patients with optic neuropathy or active inflammation in the orbit
V. List the complications of treatment, their prevention and management
A. Corticosteroids
1. Immune suppression: prevent risks by limiting dose/duration
2. Skeletal bone density: therapy/monitoring
3. Endocrine: monitor hyperglycemia//immune suppression/Cushingoid
4. Vascular: monitor hypertension/cardiovascular system
5. Ocular: monitor IOP, concurrent eye disease
6. Gastrointestinal (GI): decrease additional risk factors (other GI toxic agents) GI protection
B. Surgery
1. Eyelid deformity
2. Ocular misalignment
3. EOM damage/restrictive strabismus/neuropathic damage
4. Postoperative or delayed visual loss/central retinal artery occlusion
5. Hypoesthesia
C. Abnormal pupil/accommodation loss
1. Orbital ischemia/compartment syndrome
2. Orbital cellulitis
VI. Describe disease-related complications
A. Anterior segment
1. Corneal exposure problems
B. Ocular motility
1. Typical patterns of deviation
C. Glaucoma
1. Ocular hypertension (IOP may be artificially elevated)
2. Concurrent glaucoma
D. Optic neuropathy
VII. Describe appropriate patient instructions
A. Smoking cessation
B. Report any progression in symptoms
C. Report any change in therapeutic plan by endocrinologist
Additional Resources
1. AAO, Focal Points: Thyroid-Associated Orbitopathy, Module #1, 1997.
2. AAO, Basic and Clinical Science Course. Section 5: Neuro-ophthalmology, 2004-2005.
3. Bahn RS, Heufelder AE. Pathogenesis of Graves’ ophthalmopathy. N Engl J Med. 1993;329:1468-1475.
4. Bartalena L, Marcocci C, Bogazzi F, et al. Relation between therapy for hyperthyroidism and the course of Graves’ ophthalmopathy. N Engl J Med. 1998;338:73-78.
5. Bartley GB, Fatourechi V, Kadrmas EF, et al. Long-term follow-up of Graves ophthalmopathy in an incidence cohort. Ophthalmology. 1996;103:958-962.
6. Kazim M, Trokel S, Moore S. Treatment of acute Graves orbitopathy. Ophthalmology. 1991;98:1443-1448.
7. Garrity JA, Fatourechi V, Bergstralh EJ, et al. Results of transantral orbital decompression in 428 patients with severe Graves’ ophthalmopathy. Am J Ophthalmol. 1993;116:533-547.
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13. Gorman CA, Garrity JA, Fatourechi V. A prospective, randomized, double-blind, placebo-controlled study of orbital radiotherapy for Grave's ophthalmopathy. Ophthalmology. 2001;108:1523-34.
14. Marcocci C, Bartalena L, Tanda ML. Comparison of the effectiveness and tolerability of intravenous or oral glucocorticoids associated with orbital radiotherapy in the management of severe Grave's ophthalmopathy: results of a prospective, single-blind, randomized study. J Clin Endocrinal Metab. 2001;86:3488-93.
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