Treatment of Exophthalmos
The treatment of exophthalmos should be directed at the underlying cause, with thyroid eye disease (TED) being the most common etiology requiring a combination of medical management, orbital decompression surgery, and supportive measures to prevent vision-threatening complications. 1, 2
Diagnosis and Evaluation
Before initiating treatment, proper diagnosis is essential:
- Imaging: CT scan is the initial recommended imaging modality for suspected bilateral exophthalmos, while MRI provides superior soft tissue detail for evaluating optic nerve involvement and intracranial extension 2
- Clinical assessment: Measure exophthalmos using an exophthalmometer to document severity and monitor progression 1
- Vision assessment: Evaluate for signs of optic neuropathy (visual acuity, color vision, visual fields, pupillary exam) 1
- Thyroid function tests: Order thyroid function and associated immune studies (typically managed by endocrinology) 1
Treatment Algorithm
1. Immediate Management for Corneal Protection
- Ocular lubricants: Artificial tears and ointments to combat exposure-related dryness 1
- Punctal plugs: To increase tear retention on the ocular surface 1
- Moisture chambers/swimming goggles: For severe exposure 3
- Temporary tarsorrhaphy: In cases of severe corneal exposure not responding to conservative measures 3
2. Medical Management for Thyroid Eye Disease
- Smoking cessation: Critical as smoking significantly worsens disease progression and reduces treatment effectiveness 2
- Selenium supplementation: May reduce inflammatory symptoms in milder TED, particularly in selenium-deficient patients 1
- Teprotumumab: A human monoclonal antibody inhibitor of IGF-IR that has demonstrated effectiveness in reducing proptosis and clinical activity of TED 1
- Systemic steroids: For active inflammatory phase of TED
3. Surgical Management
Indications for Surgery:
- Compressive optic neuropathy
- Severe exposure keratopathy
- Significant proptosis causing functional or cosmetic concerns
- Restrictive strabismus causing diplopia
Surgical Options:
Orbital decompression: Removal of one or more orbital walls (floor, medial, lateral) to increase orbital volume and reduce proptosis 4
Strabismus surgery: For persistent diplopia after orbital decompression
Eyelid surgery: For correction of eyelid retraction
- Involves lengthening of anterior, posterior, or mid-eyelid lamellae 3
- Typically performed after orbital decompression and strabismus surgery
Special Considerations
Surgical sequence: When multiple procedures are needed, the recommended order is: orbital decompression first, followed by strabismus surgery, then eyelid surgery 1
Vision-threatening complications: Compressive optic neuropathy and severe corneal exposure require urgent intervention 2
Monitoring: Regular follow-up to assess disease activity, progression of exophthalmos, and development of complications 1
Pitfalls and Caveats
Strabismus risk: Orbital decompression surgery can induce or worsen strabismus, particularly when the medial wall is included 1
Timing of intervention: Surgery is typically deferred until the inflammatory phase has stabilized (usually 6 months of stable measurements), except in cases of vision-threatening complications 1
Non-thyroid causes: While TED is the most common cause (80% of bilateral cases), other etiologies such as orbital tumors, vascular malformations, sarcoidosis, and vasculitis must be considered, especially in unilateral cases 2
Superior rectus involvement: Special attention should be paid to superior rectus muscle enlargement, as it may produce reduced venous outflow from the orbit, thereby expanding orbital fat volume and worsening proptosis 5