What is the recommended treatment approach for a patient with thyroid orbitopathy?

Treatment of Thyroid Orbitopathy

A multidisciplinary approach combining endocrinology, oculoplastics, and neuro-ophthalmology is recommended for thyroid eye disease management, with treatment sequenced based on disease activity and severity. 1

Initial Assessment and Risk Factor Modification

Disease activity must be determined before initiating treatment, as this fundamentally changes the therapeutic approach:

• Clinical Activity Score (CAS) ≥3 indicates active inflammation requiring immunosuppressive therapy, while inactive disease requires only symptomatic management or surgical rehabilitation 2
• Smoking cessation is mandatory as it significantly increases both risk and severity of thyroid orbitopathy 2, 3
• Achieve and maintain euthyroid state rapidly through coordination with endocrinology, as adequately controlled thyroid function improves orbitopathy outcomes 3, 4
• Avoid radioactive iodine treatment during active orbitopathy as it can worsen the eye disease 4

Treatment Based on Disease Severity

Mild Disease (No Diplopia in Primary/Reading Position)

• Observation is appropriate if there is no diplopia in primary gaze or reading position 1
• Symptomatic management includes artificial tears for exposure symptoms and selenium supplementation 5, 3
• Monitor for disease progression with serial examinations 6

Moderate-to-Severe Active Disease (CAS ≥5)

• Teprotumumab is the preferred treatment for active moderate-to-severe disease, reducing proptosis and CAS scores 2
• Intravenous corticosteroids (methylprednisolone with cumulative doses around 3-4 grams over 6-12 weeks) are effective for active inflammation 5, 3
• Orbital radiation therapy may be combined with corticosteroids in select cases 3, 4
• Critical caveat: Assess for pre-existing hearing loss before teprotumumab and counsel patients about permanent hearing risk 2

Sight-Threatening Disease

• Emergency orbital decompression is required for compressive optic neuropathy or severe exposure keratopathy threatening vision 2, 3
• Do not delay surgical intervention when vision is threatened 7

Surgical Rehabilitation Sequence (After Disease Quiescence)

Surgery should be delayed until the inflammatory stage has subsided and the angle of deviation has been stable for at least 6 months, though earlier surgery in select patients can have satisfactory outcomes 1

The surgical sequence must follow this order:

1. Orbital decompression first (if needed for proptosis) 1
2. Strabismus surgery second to address diplopia, as decompression can worsen or create new strabismus 1
3. Eyelid surgery last to correct retraction, as earlier surgeries affect lid position 1, 3

Strabismus Surgery Considerations

• Recession of restricted muscles is the mainstay of surgical correction; resection is generally avoided in restrictive disease 1
• Vertical deviations are more challenging than horizontal deviations (66% vs 84% success after primary surgery) because the inferior rectus muscle is most commonly affected 1
• Systematic preoperative and intraoperative assessment of forced ductions and ocular torsion minimizes undesired outcomes 1
• Recession of inferior rectus muscle carries specific risks: lower lid retraction, consecutive hypertropia, and A-pattern exotropia in downgaze 1
• Consider orbital decompression consultation before strabismus surgery in patients with concurrent significant proptosis, as muscle recession predictably worsens proptosis 1

Technical Surgical Details

• Absorbable sutures (6-0 polyglactin) are generally preferred for muscle isolation and reattachment 1
• Non-absorbable sutures may reduce postoperative drift in large inferior rectus recessions 1
• Adjustable suture technique remains controversial: some surgeons report better success while others avoid it due to overcorrection and muscle slippage concerns 1
• Thyroid eye disease is the most common risk factor for pulled-in-two syndrome (spontaneous horizontal muscle transection during surgery), requiring careful surgical technique 1

Nonsurgical Diplopia Management

• Fresnel or ground-in prisms provide temporary relief while awaiting definitive treatment or for small residual deviations 1
• Chemodenervation during active phase may provide temporary diplopia relief and possibly reduce final misalignment 1

Provider Requirements

Management requires an experienced ophthalmologist comfortable with complex strabismus and severe restrictive disease, with orthoptists providing valuable assistance under supervision 1

Common Pitfalls to Avoid

• Do not perform strabismus surgery during active inflammation as the deviation will continue to change 1
• Do not perform eyelid surgery before strabismus repair as muscle surgery affects lid position 1
• Do not underestimate the complexity of vertical deviations in thyroid eye disease, particularly inferior rectus involvement 1
• Do not forget preoperative counseling about potential worsening of proptosis with muscle recession surgery 1