Sympathetic Ophthalmia: How Should We Respond to the Risk?

Christopher J. Rapuano, MD


June 15, 2022

Sympathetic ophthalmia (SO) is a rare bilateral granulomatous uveitic condition that occurs after an inciting event to one eye, most commonly ocular trauma or intraocular surgery. A study by Hashimoto and colleagues notes that the time from the inciting event to the onset of SO is reported to range from 5 days to 6 months in most cases with about 90% of cases of SO occurring within 1 year.

As a corneal and refractive surgeon, although I discuss potential complications of surgery with patients and families including loss of vision and blindness to the eye on which I am operating, I don't typically mention the possibility of complications to the unoperated eye. The one exception is when I am repairing a ruptured globe. And even in those cases, I don't usually discuss SO unless there is severe ocular damage and the chance of visual recovery is very low. The main reason is the rarity of this condition.

Hashimoto and colleagues used a national claims database that includes almost 100% of Japanese citizens (~126 million people) to calculate the cumulative incidence of SO over 60 months. It was found to be 0.073% after trauma, 0.060% after trabeculectomy, and 0.016% after vitrectomy. When two or more nontraumatic inciting events occurred in the year before SO, the incidence went up to 0.072%. However, when the inciting event was trauma or trauma occurred during the prior year, repeat events had an incidence of SO of 0.469% (a hazard ratio of 11.68 compared with a single inciting event).

The common teaching in ophthalmology is that SO can be prevented if the inciting eye is removed within 10-14 days of the injury. But, as stated above, there are reported cases of SO within 5 days of eye trauma, so the 10-14 day "rule" isn't absolute. As a practical matter, given the extremely low incidence of SO after routine ophthalmic surgery, prophylactic treatment is really only considered in cases of ruptured globes.

What should surgeons do? There are two main schools of thought. One is to remove badly damaged eyes with low chance of visual recovery as soon as practical after the injury, ideally as a primary enucleation/evisceration (which of those is preferable is debated) or within a few days. The rationale is that these eyes are very unlikely to recover useful vision, and therefore it is important to minimize the risk for SO in the fellow eye.

The opposite school of thought is to repair these eyes and not remove them right away. The rationale is that these eyes may recover much better vision than was predicted preoperatively, the patient and family need time to adjust psychologically to having the eye removed, there are good treatments for SO, and sometimes the inciting eye actually ends up with better vision than the sympathizing eye.

As is often the case, I don't believe there is a right or wrong answer to the question of what surgeons should do in these cases, and it depends on the specific situation. Personally, I am very reluctant to recommend a primary enucleation/evisceration for the reasons stated above. What impressed me the most from this study was that repeat surgery in eyes after trauma had an over 10-fold increased incidence of SO. So, although I remain quite hesitant to recommend primary enucleation/evisceration, the results of this study also increase my reluctance to perform additional intraocular surgery on eyes that I believe have little or minimal chance of regaining vision.


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