Findings
Bilateral proptosis (Hertel >21 mm), upper lid retraction with scleral show (Dalrymple sign), lid lag on downgaze (von Graefe sign), periorbital edema/fullness, conjunctival injection, chemosis over EOM insertions.
Case 04 - Neuro-ophthalmology, orbit, and inflammation
The Nervous Patient with Prominent Eyes
55-year-old man - Feeling 'nervous' x months - Bilateral proptosis and lid retraction
Case 04Thyroid Eye Disease (Graves')
Clinical Presentation
This 55-year-old man comes to your office with complaints of feeling 'nervous' for several months. He requests an evaluation and checkup. Two photographs illustrate the physical findings.
Learner Questions
- Describe the physical findings.
- Outline your plan for workup.
- What ocular complications can develop? Explain the pathophysiology for each.
- How would you treat each complication?
Answer Framework
Complications
Exposure keratopathy (proptosis + lid retraction -> incomplete blink -> corneal desiccation). Dysthyroid optic neuropathy (apical EOM compression of CN II - emergency). Restrictive strabismus/diplopia (EOM fibrosis: IR>MR>SR>LR). Elevated IOP (EOM restriction up orbital pressure).
Workup
TSH + free T4 (suppressed in Graves'), TSI/TRAb antibodies, Hertel exophthalmometry, CT orbits (EOM belly enlargement with TENDON SPARING - distinguishes from myositis), visual fields + color vision (optic neuropathy screen), slit lamp for corneal exposure.
Treatment
Exposure: lubrication, taping, moisture chambers. Optic neuropathy: IV methylprednisolone (urgent), orbital radiation, decompression. Diplopia: observation -> prisms -> EOM recession (after >=6 months stability). All: SMOKING CESSATION (most important modifiable risk factor) + selenium.
Teprotumumab (Tepezza), FDA approved 2020
Tepezza is an IGF-1R (insulin-like growth factor-1 receptor) inhibitor and the first biologic approved specifically for active, moderate-to-severe Thyroid Eye Disease. It targets the IGF- 1R/TSHR signaling complex on orbital fibroblasts, reducing the glycosaminoglycan deposition and fibrosis that drive proptosis, EOM enlargement, and inflammation. Dosing: 8 IV infusions every 3 weeks (outpatient infusion center) Best used in: Active TED (Clinical Activity Score >=4), moderate-to-severe disease Side effects to know: Hyperglycemia (screen for DM before starting), hearing loss/tinnitus (counsel patients), muscle cramps, nausea, IBD exacerbation (use caution in IBD patients). Practical pearl: Tepezza has largely replaced IV steroids + orbital radiation as first-line for moderate-to-severe active TED at centers where it's available.
Teaching Pearl
CT shows EOM belly enlargement with TENDON SPARING in TED (vs. myositis where tendons also enlarged). EOM order: I'M SLow (Inferior > Medial > Superior > Lateral). Always assess color vision for early optic nerve compression. Smoking doubles GO severity and halves treatment response.
Original answer transcript
Findings Complications Bilateral proptosis (Hertel >21 mm), upper lid retraction with scleral show (Dalrymple sign), Exposure keratopathy (proptosis + lid retraction -> incomplete blink -> corneal lid lag on downgaze (von Graefe sign), periorbital edema/fullness, conjunctival injection, desiccation). Dysthyroid optic neuropathy (apical EOM compression of CN II - emergency). chemosis over EOM insertions. Restrictive strabismus/diplopia (EOM fibrosis: IR>MR>SR>LR). Elevated IOP (EOM restriction up orbital pressure). Workup Treatment TSH + free T4 (suppressed in Graves'), TSI/TRAb antibodies, Hertel exophthalmometry, CT Exposure: lubrication, taping, moisture chambers. Optic neuropathy: IV orbits (EOM belly enlargement with TENDON SPARING - distinguishes from myositis), methylprednisolone (urgent), orbital radiation, decompression. Diplopia: observation -> visual fields + color vision (optic neuropathy screen), slit lamp for corneal exposure. prisms -> EOM recession (after >=6 months stability). All: SMOKING CESSATION (most important modifiable risk factor) + selenium. Teprotumumab (Tepezza), FDA approved 2020 Tepezza is an IGF-1R (insulin-like growth factor-1 receptor) inhibitor and the first biologic approved specifically for active, moderate-to-severe Thyroid Eye Disease. It targets the IGF- 1R/TSHR signaling complex on orbital fibroblasts, reducing the glycosaminoglycan deposition and fibrosis that drive proptosis, EOM enlargement, and inflammation. Dosing: 8 IV infusions every 3 weeks (outpatient infusion center) Best used in: Active TED (Clinical Activity Score >=4), moderate-to-severe disease Side effects to know: Hyperglycemia (screen for DM before starting), hearing loss/tinnitus (counsel patients), muscle cramps, nausea, IBD exacerbation (use caution in IBD patients). Practical pearl: Tepezza has largely replaced IV steroids + orbital radiation as first-line for moderate-to-severe active TED at centers where it's available. TEACHING PEARL CT shows EOM belly enlargement with TENDON SPARING in TED (vs. myositis where tendons also enlarged). EOM order: I'M SLow (Inferior > Medial > Superior > Lateral). Always assess color vision for early optic nerve compression. Smoking doubles GO severity and halves treatment response.