Synonyms: sympathetic ophthalmitis, sympathetic uveitis

This is an inflammatory condition affecting both eyes that occurs after a penetrating injury (accidental or surgical) to one of the eyes. It is thought possibly to be an autoimmune reaction to the exposed tissue proteins within the damaged eye.

Epidemiology

It is a very rare condition, occurring in about 3 out of every 10,000,000 cases of penetrating injury.¹ About two-thirds occur within a fortnight of the injury and 90% within the first year (range: 5 days to 66 years!).² The main risk is surgery - particularly retinal surgery.¹ Patients who have had vitreoretinal surgery and cyclodestructive procedures are particularly prone.³

Presentation⁴

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

• 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)⁵

Investigations

Blood tests (e.g. FBC, VDRL and ACE levels) and CXRs may be performed to rule out possible differentials and an ultrasound scan ± fluorescein angiography (injection of contrast dye to visualise the vessels at the back of the eye) may be performed to help confirm the diagnosis. One study also reported the use of tomography.⁶

Management

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

Complications

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

Prognosis

The prognosis is poor without rapid intervention; there is a reasonable chance that useful vision will be retained in those where diagnosis and appropriate treatment were prompt.¹ 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.⁹

Prevention⁴

In the irretrievably blind, traumatised eye, enucleation within 14 days of trauma can reduce the risk of developing this condition.

Document references

1. Kilmartin DJ, Dick AD, Forrester JV; Prospective surveillance of sympathetic ophthalmia in the UK and Republic of Ireland. Br J Ophthalmol. 2000 Mar;84(3):259-63. [abstract]
2. Chan C; Sympahetic ophthalmia, American Uveitis Society, 2003
3. Castiblanco CP, Adelman RA; Sympathetic ophthalmia. Graefes Arch Clin Exp Ophthalmol. 2009 Mar;247(3):289-302. Epub 2008 Sep 16. [abstract]
4. Kunimoto DY, Kanitkar KD, Makar MS; The Wills Eye Manual (4th Ed), Lippincott, Williams and Wilkins (2004)
5. Graham RH; Phacoanaphylaxis, eMedicine, Jul 2009
6. Correnti AJ, Read RW, Kimble JA, et al; Imaging of Dalen-Fuchs Nodules in a Likely Case of Sympathetic Ophthalmia by Ophthalmic Surg Lasers Imaging. 2010 Mar 9:1-3. doi: [abstract]
7. du Toit N, Motala MI, Richards J, et al; The risk of sympathetic ophthalmia following evisceration for penetrating eye injuries at Groote Schuur Hospital. Br J Ophthalmol. 2008 Jan;92(1):61-3. Epub 2007 Jun 25. [abstract]
8. Galor A, Davis JL, Flynn HW Jr, et al; Sympathetic ophthalmia: incidence of ocular complications and vision loss in the Am J Ophthalmol. 2009 Nov;148(5):704-710.e2. Epub 2009 Aug 7. [abstract]
9. Kanski J. Clinical Ophthalmology: A Systematic Approach (5th Ed) Butterworth Heinemann (2003)

Internet and further reading

• Zhang Y, Zhang MN, Jiang CH, et al; Development of sympathetic ophthalmia following globe injury. Chin Med J (Engl). 2009 Dec;122(24):2961-6. [abstract]
• Ward T; Sympathetic Ophthalmia, Chapter 16, Ophthalmic Care of the Combat Casualty, 2003; Some useful historical information

Acknowledgements