What is the treatment protocol for sympathetic ophthalmia following trauma, including medication regimen and duration, in cases where the inciting eye is eviscerated versus not removed?

Protocol for Sympathetic Ophthalmia Following Trauma from Rock

The management of sympathetic ophthalmia requires aggressive immunosuppressive therapy regardless of whether the inciting eye is eviscerated or not, with high-dose corticosteroids as first-line treatment followed by steroid-sparing immunomodulatory agents for long-term control.

Background

Sympathetic ophthalmia (SO) is a rare bilateral granulomatous panuveitis that occurs following penetrating trauma or surgery to one eye. It typically presents within the first year after trauma but can be delayed by several years 1. The condition affects both the injured eye (inciting eye) and the fellow eye (sympathizing eye).

Initial Evaluation

Key Diagnostic Features

• Bilateral granulomatous panuveitis
• History of penetrating trauma or intraocular surgery
• Yellowish-white choroidal lesions or Dalen-Fuchs nodules
• Anterior chamber and vitreous inflammation

Risk Assessment

• Median time from sensitizing event to SO occurrence: 151 days (range: 6-9100 days) 2
• Risk after open globe trauma and primary repair: 0.33% 2

Treatment Protocol

Immediate Management (Both Scenarios)

1. High-dose systemic corticosteroids

   • Oral prednisone 1-2 mg/kg/day
   • IV methylprednisolone 1 g/day for 3 days in severe cases

2. Topical therapy

   • Topical corticosteroid drops (e.g., prednisolone acetate 1% or dexamethasone 0.1%) every 1-2 hours initially 3
   • Cycloplegic agents to prevent synechiae formation and reduce pain 3

3. Ocular surface management

   • Ocular lubricants (non-preserved hyaluronate or carmellose eye drops) every 2 hours 3
   • Daily ocular hygiene to remove inflammatory debris 3

Scenario 1: Inciting Eye Eviscerated After SO Occurs

1. Systemic immunosuppression

   • Continue high-dose corticosteroids for 4-6 weeks
   • Begin tapering prednisone slowly over 3-6 months based on clinical response
   • Add steroid-sparing immunomodulatory therapy within 2-4 weeks of diagnosis:
     • First-line: Mycophenolate mofetil (1-3 g/day) or methotrexate (15-25 mg/week)
     • Second-line: Azathioprine (1-3 mg/kg/day) or cyclosporine (3-5 mg/kg/day)

2. Duration of therapy

   • Minimum treatment duration: 12-24 months after complete resolution of inflammation
   • Taper immunosuppression extremely slowly under close monitoring
   • Consider maintenance therapy at lowest effective dose for 2-5 years

3. Monitoring

   • Ophthalmic examination every 1-2 weeks initially, then monthly
   • Laboratory monitoring based on specific immunosuppressive agent used
   • Multimodal imaging (OCT, fluorescein angiography) every 3-6 months

Scenario 2: Inciting Eye Not Removed After SO Occurs

1. Systemic immunosuppression

   • More aggressive approach required
   • High-dose corticosteroids as in Scenario 1
   • Earlier introduction of steroid-sparing agents (within 1-2 weeks)
   • Consider combination therapy with two immunomodulatory agents

2. For refractory cases

   • Consider biological agents (adalimumab, infliximab)
   • High-dose short-term chlorambucil therapy for intractable cases:
     • Average duration: 11 weeks
     • Average total dose: 0.9 g 4
     • Allows discontinuation of systemic corticosteroids within 6-8 weeks

3. Duration of therapy

   • Longer duration required: minimum 24-36 months after complete resolution
   • Lifelong maintenance therapy may be necessary in some cases
   • More frequent monitoring (every 2-4 weeks initially)

4. Surgical considerations

   • Consider enucleation/evisceration of the inciting eye if:
     • Blind painful eye
     • No response to aggressive immunosuppression after 2-3 months
     • Recurrent inflammation despite maximal medical therapy

Treatment Response and Outcomes

Expected Outcomes

• With early and aggressive treatment, 50% of patients achieve visual acuity of 20/40 or better 5
• Poor visual outcomes associated with:
  • Delayed treatment
  • Glaucoma
  • Chorioretinal scars in the macula
  • Persistent uncontrolled inflammation 5

Treatment Failure

• If inflammation persists despite 4-6 weeks of high-dose corticosteroids and immunomodulatory therapy:
  1. Reassess diagnosis
  2. Consider more potent immunosuppression
  3. Evaluate for complications (e.g., cystoid macular edema, secondary glaucoma)
  4. Consider enucleation of inciting eye if still present

Special Considerations

Complications to Monitor

• Secondary glaucoma
• Cataract formation
• Cystoid macular edema
• Subretinal neovascularization
• Chorioretinal scarring

Medication Side Effects

• Monitor for steroid-related complications (diabetes, hypertension, osteoporosis)
• Regular laboratory monitoring for immunosuppressive agents
• Prophylaxis against Pneumocystis pneumonia for patients on high-dose immunosuppression

Follow-up Protocol

• Initial phase: Every 1-2 weeks
• Stable phase: Every 1-3 months
• Remission phase: Every 3-6 months
• Annual follow-up indefinitely after treatment cessation

Remember that early recognition of sympathetic ophthalmia and aggressive management with immunosuppressive therapy results in good outcomes 6. The visual prognosis for the sympathizing eye is generally better than for the inciting eye, with median final BCVA of 0.3 logMAR reported in the sympathizing eye 2.