Sympathetic ophthalmia

Synonyms: sympathetic ophthalmitis, sympathetic uveitis

What is sympathetic ophthalmia?

This is an inflammatory condition affecting both eyes that occurs after a penetrating injury (accidental or surgical) to one of the eyes. Key criteria for sympathetic ophthalmia included bilateral uveitis with 1) a history of unilateral ocular trauma or surgery and 2) an anterior chamber and vitreous inflammation or a panuveitis with choroidal involvement.¹

It is thought possibly to be an autoimmune reaction to the exposed tissue proteins within the damaged eye.

How common is sympathetic ophthalmia? (Epidemiology)

It is a very rare condition, occurring in about 3 out of every 10,000,000 cases of penetrating injury.² The incidence of patients developing sympathetic ophthalmia after traumatic eye injury was 0.19%.³ Repeated trauma gives the greatest risk. About two-thirds occur within a fortnight of the injury and 90% within the first year (range: 5 days to 66 years!).

Patients who have had vitreoretinal surgery and cyclodestructive procedures are also prone although less likely as the incidence of patients developing disease following vitreoretinal surgery is significantly lower, around 0.038%.⁴

Sympathetic ophthalmia symptoms (presentation)⁵

Any inflammation of an (uninvolved) eye following trauma to the fellow eye is suspect.

History

• Bilateral deterioration of vision.

• Painful red eyes.

• Photophobia.

• History of penetrating ocular trauma or surgery.

Examination

This reveals diffuse bilateral intraocular inflammation. Without a slit lamp, this will be seen as red eyes. There will usually be signs of previous injury to one eye.

With a slit lamp, look for 'mutton-fat' keratic precipitates (collections of inflammatory cells sitting in large clumps on the posterior surface of the cornea) and a hazy anterior chamber which indicates inflammatory activity (if you are able to focus in there, you will see inflammatory cells which look like particles of dust crossing a light shaft in a darkened room).

There is optic disc swelling and choroiditis (seen as discrete white patches on the retina). There may be peripheral anterior synechiae (the rim of the iris is stuck forwards on to the trabecular meshwork), neovascularisation of the iris and occlusion of the pupil.

Differential diagnosis^{2 5}

• Vogt-Koyanagi-Harada syndrome.

• Sarcoidosis.

• Tuberculosis.

• Syphilis.

• Phacoanaphylactic endophthalmitis (uveitis associated with a ruptured or degenerative lens capsule, histologically similar and sometimes mistaken for sympathetic ophthalmia).

Investigations⁵

Blood tests (eg FBC, VDRL and ACE levels) and CXRs may be performed to rule out possible differential diagnosis.

Multimodal imaging techniques, including B-scan ultrasonography, fluorescein angiography (injection of contrast dye to visualise the vessels at the back of the eye) and fundus autofluorescence may be performed to help confirm the diagnosis.

Optical coherence tomography (with or without angiography) are non-invasive tools for monitoring disease progress, response to treatment and recurrences.

Sympathetic ophthalmia treatment and management⁶

It is managed aggressively with steroids ± immunosuppressive agents. Cycloplegics may also be helpful for the symptoms. One study reported that the use of a fluocinolone acetonide implant provided inflammatory control and reduced the dependence on systemic immunosuppression.

Enucleation (removal of the globe) of the previously traumatised eye may need to be considered if this is blind anyway as this may improve the sympathetic ophthalmia.⁷

Complications²

They include neovascularisation of the iris (which can lead to secondary glaucoma), cataract and retinal detachment.

Prognosis

The prognosis is poor without rapid intervention; With aggressive treatment, approximately 70% of patients have improvement in visual acuity in the sympathising eye, of which 58.2% achieve visual acuity of 20/40 or better.⁵ . One study found that the presence of an exudative retinal detachment and active intraocular inflammation correlated with poorer vision in the sympathising eye.⁸ Very rarely, the uveitis follows a relatively mild and self-limiting course.

Sympathetic ophthalmia prevention

Key preventative measures include a) prompt and meticulous primary repair in cases with open globe injuries, b) removal of the injured eye when surgical repair is deemed impossible, and c) prompt control of inflammation.⁵