Sympathetic Ophthalmia

From EyeWiki
Original article contributed by: Ghazala D. O'Keefe, MD
All contributors: Ghazala D. O'Keefe, MD and Theodore Leng, MD, MS
Assigned editor: Theodore Leng, MD, MS
Review: Assigned status Update Pending by Theodore Leng, MD, MS on December 20, 2014.


Sympathetic Ophthalmia is a bilateral, granulomatous uveitis that occurs after trauma to the eye. The disease is vision-threatening and many patients still end up with significant vision loss especially if treatment is not instituted quickly. The onset can be insidious and slow or acute. Patients usually present with blurry vision, a red eye, and decreased vision. The clinical exam is significant for mutton fat keratic precipitates, serous retinal detachments and pinpoint hyperfluorescence seen on fluorescein angiography. Prevention is limited to urgent closure of the traumatized eye and enucleation within 10 days to 2 weeks after the traumatic event. Treatment is limited to corticosteroids and immunomodulators. The etiology is thought to be autoimmune but many questions still remained unanswered.

Disease Entity[edit | edit source]

Sympathetic Ophthalmia is a rare, bilateral, granulomatous uveitis due to trauma (more common) or surgery (less common) to one eye. Louis Braille, the inventor of modern Braille, was injured at the age of 3 in his right eye when he playing with a slender curved knife and is believed to have suffered from sympathetic ophthalmia. He lost vision in both eyes by the age of 5[1].

Etiology[edit | edit source]

The etiology is not well understood but is thought to be secondary to the development of an autoimmune reaction to ocular antigens that are exposed during the traumatic or surgical event. The primary mediators are thought to be T cells. Studies have shown that the initial wave of infiltrative cells is composed of CD4+ helper T cells[2] and the later wave of infiltrative cells are CD8+ cytotoxic T cells[3]. In vitro testing has shown proliferative T cell responses to uveal melanocytes in the peripheral blood of patients with sympathetic ophthalmia[4].

Risk Factors[edit | edit source]

Prior ocular trauma with delayed closure of the wound or prior ocular surgery. The interval between the time of injury and the onset of symptoms is variable and has been reported to be from 5 days to 66 years although the vast majority occur within the first year[5][6][7].

Pathology
[edit | edit source]

The injured eye is the exciting eye and the fellow eye is known as the sympathizing eye. Clinically both eyes appear the same and it is only by history that one can identify which eye is the exciting eye. Inflammation is granulomatous. The choroid is diffusely thickened with lympochytes, nests of epithelioid cells and multinucleated giant cells. The epithelioid cells and giant cells often contain pigment. The inflammatory process does not usually involve the choriocapillaris or the retina and is limited to the choroid. Dalen-Fuchs nodules, which are clusters of epithelioid cells containing pigment lying between the RPE and Bruchs membrane, are also seen. The iris can also appear thickened with nodular infiltrations [8].

Prevention[edit | edit source]

There has been debate regarding the only known means of prevention of sympathetic ophthalmia, i.e. removal of the injured eye (enucleation) within a week after the traumatizing incident. There have been arguments made that the exciting eye should be enucleated immediately after trauma [9] as well as arguments that steroid use after the onset allows for continued good vision in the contralateral eye. Occasionally, the exciting eye is the eye with better vision after the onset of sympathetic ophthalmia but it remains to be seen whether this continues to be the case as these patients are seen sooner and treated with corticosteroids and immunomodulators sooner. There is no known benefit to enucleation of the exciting eye after the onset of sympathetic ophthalmia.

Diagnosis[edit | edit source]

History[edit | edit source]

The history plays an important role in the diagnosis of sympathetic ophthalmia. Usually, the patient has a history of trauma or surgery to one eye, either recently or in the past.

Symptoms[edit | edit source]

Patients usually present with symptoms of uveitis: blurred vision, ocular discomfort, conjunctival injection. 

Clinical evaluation[edit | edit source]

Patients will have bilateral uveitis with mutton fat keratic precipitates along with variable inflammation of the choroid manifesting as vitritis, optic nerve swelling (papillitis), and choroiditis[10]. Patients classically have exudative retinal detachments that are visible on dilated fundus exam and can be captured on optical coherence tomography. These areas of serous retinal detachments correspond to pinpoint hyperfluorescence and leakage on fluorescein angiogram.

Ancillary procedures[edit | edit source]

Optical Coherence Tomography has been shown to be a reliable method of following serous retinal detachments [11]. Fluorescein angiography shows multiple areas of pinpoint hyperfluorescence which leak on later phases in areas that correspond to the serous retinal detachments seen clinically.

Differential diagnosis[edit | edit source]

The most common alternate diagnosis entertained when evaluating a patient with sympathetic ophthalmia is Vogt-Koyanagi-Harada syndrome. However, alternate diagnoses inlcude sarcoidosis, tuberculosis, syphilis and other causes of infectious granulomatous uveitis, as well as phacoanaphylactic uveitis. The infectious causes of uveitis must be ruled out prior to initiating treatment for sympathetic ophthalmia due to the risk of worsening the underlying infection with steroid use. 

Management[edit | edit source]

Corticosteroids are the mainstay of treatment. Steroids should be initiated as soon as the diagnosis is made in the absence of other contraindications. Along with corticosteroids, immunomodulators such as cyclosporine or azathioprine can be used. Patients should be followed closely and may need to be admitted if they cannot follow up or take the medications as prescribed. Patients should be seen often until they begin to stabilize and improve at which point visits can be spread out.

Prognosis[edit | edit source]

Sympathetic Ophthalmia is a serious vision-threatening disease. Half of all patients will have 20/40 or worse vision and one third of all patients will end up legally blind [12]. Complications include macular edema, CNV, and continued inflammation [13]

Additional Resources[edit | edit source]

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References[edit | edit source]

  1. Kaden, R (1977 Jan). Historic notices of Louis braille and the development of dot-writing (author's transl)]. Klinische Monatsblatter fur Augenheilkunde 170 (1): 154–8.
  2. Chan CC, Benezra D, Rodrigues MM, Palestine AG, Hsu SM, Murphree AL, Nussenblatt RB. Immunohistochemistry and electron microscopy of choroidal infiltrates and Dalen-Fuchs nodules in sympathetic ophthalmia. Ophthalmology 1985; 92:580–90.
  3. Jakobiec FA, Marboe CC, Knowles DM, Iwamoto T, Harrison W, Chang S, Coleman DJ. Human sympathetic ophthalmia. An analysis of the inflammatory infiltrate by hybridomamonoclonal antibodies, immunochemistry, and correlative electron microscopy. Ophthalmology 1983; 90:76–95.
  4. Damico F, Kiss S, Young LH. Sympathetic Ophthalmia. Semin Ophthalmol 2005;20:191–197.
  5. Lubin JR, Albert DM, Weisntein M. Sixty-five years of sympathetic ophthalmia. A clinicopathologic review of 105 cases (1913–1978). Ophthalmology 1980;87:109–112.
  6. Chan CC, Roberge RG, Whitcup SM, Nussenblatt RB. Thirty two cases of sympathetic ophthalmia. A retrospective study at the National Eye Institute Bethesda, MD from 1982–1992. Arch Ophthalmol 1995;113:597–601.
  7. Goto H, Rao NA. Sympathetic Ophthalmia and Vogt-Koyanagi-Harada syndrome. Int Ophthalmol Clin 1990;30:279–28541.
  8. Goto and Rao. Sympathetic ophthalmia and Vogt-Koyanagi-Harada syndrome. Int Ophthalmol Clin (1990) vol. 30 (4) pp. 279-85
  9. Lubin JR, Albert DM, Weisntein M. Sixty-five years of sympathetic ophthalmia. A clinicopathologic review of 105 cases (1913–1978). Ophthalmology 1980;87:109–112.
  10. Gupta V, Gupta A, Dogra MR. Posterior sympathetic ophthalmia: a single centre long-term study of 40 patients from North India. Eye. 2008;3(12):1459–1464.
  11. Chan RV, Seiff BD, Lincoff HA, Coleman DJ. Rapid recovery of sympathetic ophthalmia with treatment augmented by intravitreal steroids. Retina. 2006;3(2):243–247.
  12. Chan CC, Roberge RG, Whitcup SM, Nussenblatt RB. Thirty two cases of sympathetic ophthalmia. A retrospective study at the National Eye Institute Bethesda, MD from 1982–1992. Arch Ophthalmol 1995;113:597–601.
  13. Damico F, Kiss S, Young LH. Sympathetic Ophthalmia. Semin Ophthalmol 2005;20:191–197.