BASICS

DESCRIPTION

• Typically it is a bilateral granulomatous panuveitis that occurs following penetrating injury or surgery to one eye.

• Although the trauma occurs in only one eye, the uveitis involves both eyes. The traumatized eye is commonly referred to as the “inciting” or “exciting” eye and the contralateral eye is known as the “sympathizing” eye.

EPIDEMIOLOGY

Incidence

Incidence of the disorder following trauma or surgery is very rare, occurring in 0.19% of cases following trauma and 0.007% of cases following ocular surgery.

RISK FACTORS

Occurs following penetrating injury or surgery to the eye.

Genetics

HLA-11, DR4/DRw53, DR4/DRw3, DRB1*O4, DQA1*03, and DQB1*04 have been identified in some patients.

GENERAL PREVENTION

• The avoidance of trauma

• Enucleation (removal) of a blind eye within 14 days of the trauma may prevent the development of sympathetic ophthalmia.

PATHOPHYSIOLOGY

Histological findings are the same in exciting and sympathizing eyes. Findings include a diffuse granulomatous inflammation consisting of lymphocytes (predominantly T-cells) and epithelioid cells within the uveal tract. Epithelioid cells may contain pigment. Multinucleated giant cells may also be present. In addition, clusters of epithelioid cells may accumulate between the retinal pigment epithelium and Bruch’s’ membrane. These clusters may appear yellow clinically and are known as Dalen–Fuchs nodules.

ETIOLOGY

Many studies reveal a role for T-cell mediated inflammation directed against uvea–retina proteins.

COMMONLY ASSOCIATED CONDITIONS

Trauma

DIAGNOSIS

HISTORY

• A history of trauma or surgery occurring prior to the onset of inflammation is critical to making the diagnosis.

• There is no specific laboratory test to confirm the diagnosis.

• Patients often complain of pain, redness, photophobia, and blurred vision.

PHYSICAL EXAM

• Exciting eye

• Photophobia, decreased vision, and keratic precipitates on endothelium may be seen.

• Sympathizing eye

• Symptoms are variable and may be insidious or rapid in onset. Mild anterior or posterior uveitis, ciliary flush, keratic precipitates, pain, photophobia, increased lacrimation, and blurred vision may occur.

• Both eyes

• Ciliary injection, partially dilated–poorly responsive pupil, thickened iris, clouding of vitreous, papillitis, generalized retinal edema, Dalen-Fuchs nodules (small, yellow, deposits beneath retinal pigment epithelium), peripheral choroiditis, exudative retinal detachments, and choroidal granulomas have been described.

DIAGNOSTIC TESTS & INTERPRETATION

Imaging

Fluorescein angiography reveals multiple hyperfluorescent dots at the level of the RPE that may persist in to the late phase of angiogram. Coalescence of the dye from these foci may occur in areas of exudative retinal detachment. A pattern similar to multiple evanescent white dot syndrome–-early hypofluorescence followed by dye leakage in a wreath-like pattern in the venous phase has also been described.

Pathological Findings

A diffuse granulomatous inflammation consisting of lymphocytes (predominantly T-cells) and epithelioid cells within the uveal tract. Giant cells may also be present. The choriocapillaris and retina are usually not involved.

DIFFERENTIAL DIAGNOSIS

• Sarcoidosis

• Tuberculosis

• Vogt–Koyanagi–Harada syndrome

TREATMENT

MEDICATION

First Line

Oral (1 mg/kg/day) or pulse dose corticosteroids have a rapid onset of action. However, patients who need more than 10 mg of prednisone daily to control their inflammation will require steroid-sparing agents as high-dose steroids cannot be used for long periods of time without severe side effects.

Second Line

Enucleation of the traumatized eye within 2 weeks of injury may prevent development of sympathetic ophthalmia. Removal of the traumatized eye after SO develops is controversial.

ADDITIONAL TREATMENT

General Measures

Steroid-sparing agents such as mycophenolate mofetil, azathioprine, cyclosporine, tacrolimus, and anti-TNF inhibitors have all been used with some success.

Issues for Referral

A multidisciplinary team may be required to manage the various systemic effects of steroid-sparing therapies (immunosuppression).

SURGERY/OTHER PROCEDURES

• Laser photocoagulation can be used for retinal neovascularization and does not produce postoperative inflammation.

• Fluocinolone acetonide implantation (Retisert) may help control inflammation in some patients.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patients will need very close monitoring until the disease is brought under control. Patients will need continuous follow-up with other specialists as needed.

PROGNOSIS

A majority of patients treated with corticosteroids/immunosuppression can maintain a vision of 20/60 or better.

COMPLICATIONS

• Bilateral vision loss due to ocular inflammation

• Anterior uveitis can cause cataract formation or glaucoma. Posterior segment inflammation can result in macular and peripheral retinal scaring and choroidal detachment.

Stay updated, free articles. Join our Telegram channel

Join