What is the management for an adult patient with increased bilateral proptosis and no known history of orbital trauma or recent infection?

Management of Increased Bilateral Proptosis

Immediately obtain MRI orbits with and without contrast as the optimal initial imaging modality, while simultaneously consulting ophthalmology urgently to assess for vision-threatening complications including compressive optic neuropathy, corneal exposure, and elevated intraocular pressure. 1, 2

Immediate Assessment and Stabilization

The first priority is identifying vision-threatening complications that require emergent intervention:

• Assess visual acuity, pupillary responses (looking specifically for relative afferent pupillary defect), color vision, and perform fundoscopy to diagnose compressive optic neuropathy 1, 2
• Measure intraocular pressure in both eyes, as orbital congestion from proptosis commonly causes secondary glaucoma 2
• Perform fluorescein staining to identify corneal epithelial defects, particularly critical as exposure keratopathy can rapidly progress to irreversible corneal scarring and vision loss 2
• Measure exophthalmos using exophthalmometry to establish baseline measurements for tracking disease progression 1, 3

Diagnostic Imaging

MRI orbits with and without contrast is the single best initial imaging study because it provides superior soft tissue characterization for masses, inflammatory conditions (including thyroid eye disease, IgG4-related disease, and idiopathic orbital inflammatory syndrome), vascular malformations, and optic nerve pathology 1, 2

• Add CT orbits with contrast as a complementary study to assess orbital and muscle volumes, fat volumes, osseous anatomy, and to evaluate for orbital decompression planning if thyroid eye disease is suspected 1
• Obtain MRI head with and without contrast if intracranial extension or distant metastasis is suspected 1

Etiology-Specific Management

Thyroid Eye Disease (Most Common Cause)

For active moderate-to-severe thyroid eye disease with significant proptosis, teprotumumab is the most effective medical therapy, as it directly reduces proptosis, decreases clinical activity scores, improves diplopia, and reduces the need for strabismus surgery 1, 2, 3

• If teprotumumab is unavailable, contraindicated (pregnancy, inflammatory bowel disease, pre-existing hearing loss), or not suitable, use high-dose IV methylprednisolone for severe proptosis or optic neuropathy 2
• Consider urgent orbital decompression, high-dose pulse IV steroids, or orbital radiation for severe proptosis with compressive optic neuropathy 1, 2, 3
• Initiate ocular lubricants immediately to combat exposure from eyelid retraction and proptosis; punctal plugs can augment this 1, 3
• Counsel mandatory smoking cessation, as smoking significantly worsens orbitopathy 3
• Coordinate with endocrinology to optimize thyroid function, as maintaining euthyroid status is essential 1

Other Inflammatory Conditions

For idiopathic orbital inflammatory syndrome or IgG4-related disease presenting with bilateral proptosis, corticosteroids are typically first-line, though imaging cannot definitively distinguish between these entities and biopsy may be required 1

Orbital Masses or Vascular Lesions

If imaging reveals orbital masses, coordinate with oculoplastic surgery and neuro-ophthalmology for potential biopsy and definitive surgical management 1

Multidisciplinary Consultation

A multidisciplinary approach combining ophthalmology, endocrinology, oculoplastics, and neuro-ophthalmology is mandatory for optimal management of bilateral proptosis 1, 2

• Immediate ophthalmology consultation is non-negotiable for all patients with bilateral proptosis 2
• Daily ophthalmological review is necessary during acute illness to perform ocular hygiene, remove inflammatory debris, and break down conjunctival adhesions 2

Corneal Protection

Apply broad-spectrum topical antibiotic prophylaxis (quinolone preparations such as moxifloxacin or levofloxacin four times daily) when corneal fluorescein staining or frank ulceration is present 2

Critical Monitoring

• Screen for optic neuropathy at each visit with visual acuity, color vision, visual fields, pupillary examination, and fundus examination 1, 3
• Consider optical coherence tomography and automated threshold visual fields as ancillary screening tools for compressive optic neuropathy 1
• Repeat exophthalmometry measurements at follow-up visits to objectively track progression or regression 3

Common Pitfalls to Avoid

Failing to protect the cornea in patients with severe proptosis leads to irreversible corneal scarring and permanent vision loss 2

Delaying ophthalmology consultation can result in permanent blindness from compressive optic neuropathy, which is preventable with timely intervention 2

In patients with concurrent proptosis and significant strabismus from thyroid eye disease, recession of fibrotic extraocular muscles predictably worsens proptosis, warranting consultation with an orbital specialist for orbital decompression surgery prior to strabismus surgery 1