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Symptoms ⬇

Bilateral eye pain, photophobia, decreased vision, and red eyes. A history of penetrating trauma or intraocular surgery (most commonly vitreoretinal surgery) to one eye (usually 4 to 8 weeks before, but ranges from days to decades, with 90% occurring within 1 year). Sympathetic ophthalmia is rare (the literature estimates an annual incidence of 0.03/100,000 people).

Signs ⬆ ⬇

Critical

Suspect anytime there is inflammation in the uninvolved eye after unilateral ocular trauma or surgery. Bilateral severe anterior chamber reaction with large mutton-fat KP; may have asymmetric involvement with typically more reaction in uninjured eye. Posterior segment findings include small depigmented nodules at the level of the retinal pigment epithelium (corresponding to Dalen–Fuchs nodules histopathologically) and diffuse thickening of the choroid. Signs of previous injury or surgery in one eye are usually present. In developed countries, repeated vitreoretinal procedures following ocular trauma are the most common risk factors.

Other

Nodular infiltration of the iris, peripheral anterior synechiae, neovascularization of the iris, occlusion, and seclusion of the pupil, cataract, exudative retinal detachment, and papillitis. The earliest sign may be loss of accommodation, or a mild anterior or posterior uveitis in the uninjured eye.

Differential Diagnosis ⬆ ⬇

  • VKH syndrome: Similar signs, but no history of ocular trauma or surgery. Other systemic symptoms. See 12.11, VOGT–KOYANAGI–HARADA SYNDROME.
  • Phacoantigenic (formerly phacoanaphylaxis) endophthalmitis: Severe anterior chamber reaction from injury to the lens capsule. The contralateral eye is uninvolved. See 9.12, LENS-RELATED GLAUCOMA.
  • Sarcoidosis: Often associated systemic symptoms involving the lungs or skin, elevated ACE level, or characteristic pulmonary changes on chest CT. May cause a bilateral granulomatous panuveitis. See 12.6, SARCOIDOSIS.
  • Syphilis: Positive treponemal and reflex nontreponemal testing. May cause bilateral granulomatous panuveitis. See 12.12, SYPHILIS.
  • Tuberculosis: Positive PPD or IGRA with possible characteristic findings on CXR or chest CT. May cause bilateral granulomatous panuveitis.
  • Multifocal choroiditis with panuveitis: Usually bilateral, with no history of trauma.

Work Up ⬆ ⬇

Workup
  1. History: Any prior eye surgery or injury? History of sexually transmitted disease or high-risk sexual activity? Difficulty breathing?
  2. Complete ophthalmic examination, including a dilated retinal examination.
  3. Assess for any systemic findings to rule out VKH (e.g., neurologic, skin, or auditory changes).
  4. CBC, EIA, or another treponemal testing followed by reflex, nontreponemal testing if positive.
  5. Chest radiograph and/or CT chest to evaluate for tuberculosis and sarcoidosis.
  6. IVFA or B-scan ultrasound, or both, to help confirm the diagnosis.

Treatment ⬆ ⬇

  1. Prevention: Historically enucleation of a blind, traumatized eye within 14 days of the trauma has been recommended to reduce the risk of sympathetic ophthalmia. However, this has limited support in the literature and should only be performed if the eye has been deemed unsalvageable. Once sympathetic ophthalmia develops, enucleation of the sympathizing eye appears to have no benefit.
  2. Initial treatment with high dose oral steroids (e.g. prednisone 1 mg/kg p.o. daily) with calcium/vitamin D supplementation and gastric ulcer prophylaxis may be used initially to control ocular inflammation.
  3. However long-term local or systemic immunosuppression is essential in most cases. Local control can be achieved with intravitreal steroid implants (e.g., dexamethasone 0.7 mg intravitreal implant or the fluocinolone acetonide 0.19 or 0.59 mg intravitreal implants). Inflammatory control can also be achieved with steroid-sparing systemic immunosuppression. The choice of specific local or systemic immune suppression should be made in conjunction with a uveitis specialist and individualized for each patient.
  4. Cycloplegic (e.g., cyclopentolate 1% b.i.d.) for active anterior chamber inflammation.
  5. Topical steroids for active anterior chamber inflammation (e.g., prednisolone acetate 1% q1–2h or difluprednate 0.05% q2h), which are tapered slowly as the inflammation improves.
  6. Periocular or intravitreal steroids (e.g., subconjunctival triamcinolone acetate 40 mg in 1 mL) may be used to aid in local control prior to pursuing long-term options. See Appendix 10, TECHNIQUE FOR RETROBULBAR/SUBTENON/SUBCONJUNCTIVAL INJECTIONS.

Follow Up ⬆

  1. Every 1 to 7 days initially, to monitor the effectiveness of therapy and IOP.
  2. As the condition improves, the follow-up interval may be extended to every 3 to 4 weeks.
  3. Oral steroids may be used initially to quell inflammation with rapid, sequential initiation of long-term local or steroid-sparing systemic immunosuppression to allow for tapering of oral steroids. The goal should be to slowly taper oral prednisone to 5 mg or less by 3 months to avoid the adverse effects of systemic steroids. Because of the possibility of recurrence, periodic checkups are important.
  4. The long-term prognosis in patients treated with immunosuppressive therapy can be good, with 81% of eyes seeing ≥20/40 at the final follow up.