Should I Enucleate the Inciting Eye in a Patient With Sympathetic Ophthalmia?
Jose Cuevas
Francisco,
MD
Harvey Siy
Uy,
MD
Sympathetic ophthalmia (SO) is a bilateral, granulomatous, panuveitis precipitated by penetrating trauma to the inciting/exciting eye resulting in an inflammatory response in the uninjured, sympathizing eye. SO is a potential, bilaterally blinding complication of open globe injury or ocular surgery.1 Vision loss may result from cataract, secondary glaucoma, maculopathy, retinal detachment, optic neuritis, vasculitis, or neovascularization. It is believed that penetrating trauma allows ocular antigens to gain access to lymphatics and be presented to the systemic immune system, producing self-destructive, autoimmune, ocular sequelae.
With improvements in trauma and surgical care, SO has become a rare, though still feared, uveitic condition. The diagnosis of SO should be suspected in patients with recent history of penetrating trauma or surgery and bilateral panuveitis. The onset of SO may be insidious with symptoms manifesting a few weeks to several months or years after the inciting injury. The vast majority (90%) of cases manifest within a year of injury.
SO presents classically as granulomatous panuveitis. The range of intraocular signs include anterior uveitis (Figure 49-1) with mutton fat keratic precipitates, moderate to severe vitritis, white-yellow chorioretinal infiltrates called Dalen-Fuchs nodules (Figure 49-2), and papillitis. Systemic involvement may include Vogt-Koyanagi-Harada (VKH) syndrome-like findings such as alopecia, vitiligo, poliosis, dysacousia, and cerebrospinal fluid pleiocytosis. The vision-robbing complications of SO are legion and include cataracts; secondary glaucoma; cystoid macular edema; retinal detachment; optic neuritis; retinal vasculitis; subretinal fibrosis; and atrophy of the retina, choroid, and optic nerve (Figures 49-3 and 49-4).
Figure 49-1. Slit-lamp photograph demonstrating iris nodules and posterior synechiae in a patient with sympathetic ophthalmia.
Figure 49-2. Fundus photograph demonstrating peripheral yellow-white choroidal lesions (Dalen-Fuchs nodules) in a patient with sympathetic ophthalmia.
Figure 49-3. Fundus photograph demonstrating multiple chorioretinal scars and subretinal fibrosis following penetrating trauma in the inciting eye of a patient with sympathetic ophthalmia.
Figure 49-4. Fundus photograph demonstrating severe vitritis and inferior retinal detachment in the sympathizing, contralateral eye of the patient in Figure 49-3.
The only certain prevention for SO is enucleation, which should ideally be performed within 2 weeks of ocular injury. Mere evisceration may still lead to SO as postevisceration uveal antigens may still remain and incite an autoimmune response.
In our practice, however, I do not routinely enucleate the inciting eye as a means for preventing SO. The reasons for my approach are 1) current microsurgical techniques for ocular surgery or repair of ocular trauma have led to a decrease in the incidence of SO, 2) the advent of intraocular and systemic immunomodulatory agents have greatly improved the prognosis of SO, and 3) the inciting or exciting eyes sometimes have better visual outcomes than sympathizing eyes. In the Vietnam, Korean, and Arab-Israeli Six-Day War, no cases of SO were reported. This suggests that improved management of ocular trauma, including use of antibiotics, corticosteroids, and microsurgical repair, have decreased the risk for SO.
Enucleation remains a controversial approach. While some authors have suggested that early enucleation of the exciting eye led to improved visual prognosis, Kilmartin and associates suggested that enucleation of the inciting eye following onset of SO did not lead to a better visual outcome in the sympathizing eye nor lessened the need for immunosuppressive therapy.1,2 No clinical trials have compared the outcomes of these 2 opposite approaches.
Despite a chronic, relapsing course, good outcomes may be obtained with immunosuppressive agents or immunomodulatory therapy (IMT). Patients with acute SO should be promptly started on systemic corticosteroids (eg, prednisone 1 to 2 mg/kg/day). Using this approach, Makley and Azar reported that 65% of eyes had 20/60 or better visual acuity.3 Supplementation of systemic corticosteroids with periocular corticosteroid depot (eg, trans-septal triamcinolone acetonide 40 mg/1 mL) may provide additional, rapid immunosuppressive effect.
Severe or recurrent cases will require combination therapy with one or more immunosuppressive agents. Cyclophosphamide, cyclosporine, azathioprine, and methotrexate are frequently used corticosteroid-sparing drugs. I thoroughly discuss the potential risks and benefits of enucleation versus IMT side effects prior to initiation of any treatment and regularly monitor patients for treatment-related side effects. Co-management with an internist or rheumatologist should be considered when the ophthalmologist is not trained in the use of IMT. The introduction of biologic drugs and sustained-release ocular implants for the treatment of SO may further lead to reduced rates of enucleation.4 Despite the availability of nonsurgical methods of treatment, the long-term prognosis for SO is variable, and poor vision may be the end result despite IMT.
I reserve enucleation of the inciting eye when it is blind, painful, phthsical, or disfigured. Enucleation in these cases may provide pain relief and improved cosmesis after fitting ocular prostheses. Inadequate access to health care or poor compliance with IMT medications are factors to consider when choosing a surgical versus medical approach to treatment.
Summary
Fortunately, SO is becoming an extremely rare complication of penetrating ocular trauma. Enucleation of the inciting eye can usually be avoided by early recognition of SO and prompt, sustained institution of corticosteroids and IMT. In occasional instances, enucleation may be considered for blind, painful, and disfigured inciting eyes.
References
1. Kilmartin DJ, Dick AD, Forrester JV. Prospective surveillance of sympathetic ophthalmia in the United Kingdom and Republic of Ireland. Br J Ophthalmol. 2000;84:259-263.
2. Lubin JR, Albert DM, Weinstein M. Sixty-five years of sympathetic ophthalmia. A clinicopathologic review of 105 cases (1913-1978). Ophthalmology. 1980;87:109-121.
3. Makley TA, Azar A. Sympathetic ophthalmia: a long term follow up. Arch Ophthalmol. 1978;96:257-262.
4. Mahajan VB, Gehrs KM, Goldstein DA, Fischer DH, Lopez JS, Folk JC. Management of sympathetic ophthalmia with the fluocinolone acetonide implant. Ophthalmology. 2009;116:552-557.