What is the treatment for exophthalmos (bulging eyes) due to Graves' disease?

Treatment of Exophthalmos in Graves' Disease

For exophthalmos (bulging eyes) due to Graves' disease, the recommended treatment approach includes teprotumumab as first-line therapy for active disease, with orbital decompression surgery reserved for severe cases or those unresponsive to medical management. 1

Diagnostic Assessment

• Measure exophthalmos using an exophthalmometer to document severity and monitor progression
• Evaluate for signs of optic neuropathy (visual acuity, color vision, visual fields, pupillary exam)
• Order thyroid function tests and immune studies
• Consider orbital CT or MRI to evaluate extraocular muscle enlargement and orbital fat volume

Treatment Algorithm Based on Disease Severity

Mild Exophthalmos

1. First-line management:

   • Ocular lubricants (artificial tears, ointments) to combat exposure-related dryness 2
   • Punctal plugs to increase tear retention 2
   • Selenium supplementation (particularly in selenium-deficient patients) 2
   • Smoking cessation (critical as smoking significantly worsens disease progression) 1

2. Hyperthyroidism management:

   • Antithyroid drugs (ATDs) like methimazole or propylthiouracil are preferred 3
   • If radioiodine is used, administer steroid prophylaxis (short course of low-dose prednisone) 3

Moderate-to-Severe Active Exophthalmos

1. First-line therapy:

   • Teprotumumab (human monoclonal antibody inhibitor of IGF-IR) - demonstrated to significantly reduce proptosis and clinical activity score 2, 1

2. Alternative/adjunctive therapies:

   • High-dose intravenous glucocorticoids for inflammatory symptoms 2
   • Orbital radiation therapy in selected cases, particularly with extraocular muscle involvement 4
   • For hyperthyroidism, ATDs are preferred over radioiodine to avoid worsening ophthalmopathy 3, 5

3. For non-responders or vision-threatening cases:

   • Immediate high-dose intravenous glucocorticoids 2
   • Urgent orbital decompression surgery if optic neuropathy persists 2

Inactive/Stable Exophthalmos (≥6 months stability)

1. Rehabilitative surgical interventions (in sequential order):
   • Orbital decompression surgery (removal of one or more orbital walls to increase volume)
   • Strabismus surgery (typically 6 weeks after decompression)
   • Eyelid surgery (final step in rehabilitation) 1

Special Considerations

Management of Hyperthyroidism with Exophthalmos

• Preferred approach: Antithyroid drugs (ATDs) for patients with active TED 3, 5
• Caution with radioiodine: Associated with 15% risk of worsening ophthalmopathy; if used, concurrent prednisone prophylaxis is essential 5
• Surgical thyroidectomy: Valid option for moderate-to-severe TED when definitive treatment is needed 3

Monitoring and Follow-up

• Regular assessment of disease activity and progression
• Monitor for vision-threatening complications (corneal exposure, optic neuropathy)
• Evaluate stability of measurements before considering surgical interventions
• Typical waiting period of 6 months of stability before rehabilitative surgery 1

Common Pitfalls to Avoid

1. Using radioiodine without steroid prophylaxis in patients with active TED
2. Performing eyelid surgery before orbital decompression (can worsen corneal exposure)
3. Failing to correct hyperthyroidism, which can exacerbate ophthalmopathy
4. Delaying intervention for vision-threatening complications
5. Not addressing smoking, which significantly impairs treatment effectiveness

Remember that the treatment sequence matters significantly: orbital decompression should be performed first, followed by strabismus surgery, and finally eyelid surgery to achieve optimal outcomes 1.