Comparison of Clinical Features and Visual Outcome between Sympathetic Ophthalmia and Vogt-Koyanagi-Harada Disease in Chinese Patients.

PURPOSE: To characterize the clinical features of sympathetic ophthalmia (SO) and compare SO and Vogt-Koyanagi-Harada (VKH) disease in Chinese patients. DESIGN: Retrospective case series. PARTICIPANTS: A total of 131 consecutive SO and 500 VKH disease patients randomly selected from among those referred to our uveitis center from April 2008 through June 2018. METHODS: History, extraocular and ocular findings, best-corrected visual acuity (BCVA), auxiliary examination findings, complications, and therapeutic effects were analyzed retrospectively in SO and VKH disease patients. MAIN OUTCOME MEASURES: Visual outcome, extraocular and ocular findings, and therapeutic effects. RESULTS: Sympathetic ophthalmia manifested as posterior uveitis (68.8%) within 2 weeks and equal involvement of anterior and posterior segment (44.4%), respectively, was observed between 2 weeks and 2 months after disease onset. Two months after disease onset, SO patients showed sunset glow fundus (51.2%) and granulomatous anterior uveitis (27.3%). Vogt-Koyanagi-Harada disease patients mainly showed posterior uveitis (100%), anterior segment involvement (92.4%) associated with posterior uveitis (84.9%), and granulomatous anterior uveitis (97.4%) accompanying sunset glow fundus (91.5%) in the 3 periods mentioned above. The frequencies of extraocular manifestations were lower in SO patients (24.4%) as compared with VKH disease patients (84.8%; P < 0.001). Best-corrected visual acuity of SO patients improved from 0.68±0.86 logarithm of the minimum angle of resolution (logMAR) to 0.47±0.78 logMAR (P = 0.01), and BCVA of VKH disease patients improved from 0.67±0.79 logMAR to 0.24±0.53 logMAR (P < 0.001) at 12 months of follow-up. A worse BCVA was noted in SO patients compared with VKH disease patients after treatment (P = 0.003). Kaplan-Meier survival analysis showed that the risk of loss of useful vision in SO patients was significantly higher than that of VKH disease patients (P < 0.001). CONCLUSIONS: Chinese SO and VKH disease patients have a different evolutionary process. The frequency of extraocular manifestations in SO patients is much lower as compared with VKH disease patients. Visual outcome is worse in SO as compared with VKH disease.

Postsurgical sympathetic ophthalmia: retrospective analysis of a rare entity.

PURPOSE: To describe clinical manifestations, management and visual outcome in postsurgical sympathetic ophthalmia (SO). METHODS: Retrospective study. RESULTS: Mean age of the patients was 41.1 years, and males were affected 1.8 times than the female. Vitrectomy and scleral buckling were the most common inciting surgeries followed by cataract surgery. Among 10 eyes with anterior uveitis, mutton-fat keratic precipitate was seen in only two eyes. Mean follow-up duration was 1556.50 ± 1470.75 days. Vision significantly improved in 11 patients (78.6%; p = 0.005). CONCLUSION: Postsurgical SO is a rare entity, but it is a bilateral blinding disease and SO following surgical intervention can have variable presentations. Rapid, effective management of postsurgical sympathetic ophthalmia can give improved visual outcomes.

Long-term, drug-free remission of sympathetic ophthalmia with high-dose, short-term chlorambucil therapy.

OBJECTIVE: To evaluate the safety and effectiveness of short-term, high-dose chlorambucil therapy in achieving long-term, drug-free remission in the treatment of sympathetic ophthalmia (SO). DESIGN: Retrospective case series. PARTICIPANTS: Sixteen patients with SO treated with high-dose, short-term chlorambucil therapy between 1970 and 2010. METHODS: Descriptive and bivariate analyses were used to characterize disease and outcomes. MAIN OUTCOME MEASURES: Months of disease-free remission, prevalence rate of relapse, and prevalence of serious treatment-related adverse events. RESULTS: Sixteen patients with SO treated with short-term, high-dose chlorambucil were identified. Patients were treated with chlorambucil for a median of 14.0 weeks (mean, 14.5 weeks; range, 12.0-19.0 weeks). Median follow-up was 98.5 months (mean, 139.1 months; range, 48-441 months) from initiation of chlorambucil therapy. Control of inflammation was achieved in 100% of patients. Thirteen patients (81.3%) maintained vision of 20/40 or better in the sympathizing eye. Four patients (25%) relapsed after a median of 83 months (mean, 131 months) after cessation of systemic therapy. Seventy-five percent of relapses were controlled with topical therapy only. Conjunctival Kaposi's sarcoma developed in 1 patient. No patient demonstrated systemic malignancy. CONCLUSIONS: Short-term, high-dose chlorambucil therapy provides sustained periods of drug-free remission. With median follow-up of more than 8 years (mean, 11.6 years; range, 4-37 years), there was a low rate of recurrence and minimal long-term serious health consequences or adverse events. Because SO may be a lifelong condition and because chlorambucil therapy may offer long-term, drug-free remission, this treatment may be worth considering early in the decision-making process for severe sight-threatening disease.

Choroidal Structural Changes in Sympathetic Ophthalmia on Swept-Source Optical Coherence Tomography.

Purpose: To analyze choroidal angioarchitecture in sympathetic ophthalmia (SO) using swept-source optical coherence tomography (SS-OCT) images.Methods: Case-control study of six patients with SO. Qualitative changes and quantitative parameters, including choroidal thickness (CT) and choroidal vascularity index (CVI), were analyzed.Results: Qualitative findings in the acute phase of SO on SS-OCT included retinal serous detachment with hyperreflective septa, choroidal folds, alterations in angioarchitecture with loss of vascular lacunae, and Dalen-Fuchs nodules. There was significantly higher CT in SO (284.05 ± 24.12 µm) compared to healthy controls (229.57 ± 46.67 µm, p = 0.04) and also increased CVI in SO (62.06 ± 2.07% vs 56.79 ± 3.15%, p = 0.006).Conclusions: CVI was significantly increased in SO, representing a novel noninvasive biomarker of disease activity. SS-OCT provides a useful qualitative and quantitative parameter, which can be potentially explored in the diagnosis and monitoring of patients with SO.

Sympathetic ophthalmia.

BACKGROUND: Sympathetic ophthalmia (SO) is a rare, bilateral, non-necrotizing, granulomatous uveitis that occurs after ocular trauma or surgical procedures to one eye threatening sight in the fellow eye. The pathophysiology is not clearly understood, but it appears that the disrupted integrity of the inciting eye leads to an autoimmune hypersensitivity reaction against the exposed ocular antigens in the injured eye as well as in the sympathizing eye. More recently, vitreoretinal surgery has been noted to be a risk factor for the development of SO. METHODS: Medline search for case reports of sympathetic ophthalmia with links to full text in English yielded articles for review of patient demographics, clinical presentation and examination, therapies and final visual acuity. RESULTS: Eighty-six patients with SO were included in this review. Sixty-two patients were male and 24 were female with an average age of 46 years. Injuries accounted for 47% of patients while ocular surgery was reported in 44% of patients with pars plana vitrectomy occurring in 21%. Most patients reported reduced vision and presented with uveitis. Ninety-five percent of them received systemic corticosteroid therapy and 75% of patients also received immunomodulators. About 70% of patients had improved visual acuity in their sympathizing eye at their last reported evaluation. CONCLUSIONS: Sympathetic ophthalmia warrants prompt evaluation and treatment to maintain a favorable visual outcome. Ocular surgeries including vitreoretinal surgery and cyclodestructive procedures have been noted to be risk factors for the development of sympathetic ophthalmia. With current medical management including corticosteroids and immunomodulators visual prognosis is relatively good.

Surgical Intervention in Inciting Eyes of Patients with Sympathetic Ophthalmia: A Case Series and Review of Literature.

Purpose: To analyze the outcomes of surgical procedures on inciting eye of patients with Sympathetic ophthalmia (SO). Methods: Retrospective study of patients with SO who underwent surgical procedures on inciting eyes between January 2000 and December 2015. Outcome measures included flare up of inflammation in either eye and change in visual acuity in the inciting eye. Results: Four SO patients underwent surgeries in their inciting eyes after adequate control of inflammation. Surgical procedures included penetrating keratoplasty, glaucoma drainage device implantation, pars plana vitrectomy, and silicon oil removal. Keratoplasty, glaucoma surgery, and silicon oil removal were well tolerated, with no flare up of disease. The patient who underwent pars plana vitrectomy, however, had a poor outcome. Conclusions: Surgical intervention in inciting eyes of patients with SO, after being adequately treated with oral steroids and immunosuppression, is a viable option for improving anatomic and functional outcomes in these eyes.

Sympathetic Ophthalmia after Vitreoretinal Surgeries: Incidence, Clinical Presentations and Outcomes of a Rare Disease.

Aim: To evaluate clinical presentation, course and outcomes in patients without a history of penetrating ocular trauma who developed Sympathetic Ophthalmia (SO) following vitreoretinal surgeries Methods: Retrospective review of clinical records of all patients diagnosed and treated as S.O was done . All cases without a previous history of trauma were included and were analyzed with respect to clinical presentations, anatomic and visual outcomes. Results: 175 cases of sympathetic ophthalmia were diagnosed and treated till June 2017. 16 of these cases had undergone a pars plana vitrecomy (PPV) in the past and had no history of prior ocular trauma. SO after vitreoetinal surgeries accounted for 9.14 percent of all cases of SO .In the same duration, till 2017,a total 41365 PPV were done. Thus 0.038 percent of PPV cases developed a SO . 10 patients were males and 6 were females. The median age at presentation was 45.7 years. The time interval from surgery to diagnosis of sympathetic ophthalmia ranged from 22 days to 4 years after undergoing a surgery. The mean visual acuity in the sympathizing eye was 1.26 logMAR (snellens equivalent of 20/320) which improved to 0.62 logMAR(snellens equivalent of 20/80) after treatment. The most common anterior segment finding was non granulomatous anterior uveitis, seen in 8 cases (50%) while neurosensory detachments were the most common posterior segment presentation (10 cases, 62.5%).12 patients had undergone more than 1 surgery (mean number of surgeries was 1.88). 10 patients had undergone a sutureless PPV (6 cases of 23 gauge and 4 cases of 25 gauge vitrectomy) while 4 patients had undergone a 20 gauge vitrectomy where all sclerotomies were sutured after surgery All patients were treated with systemic steroids and immunosuppresants and 15 out of 16 patients showed significant improvement in the final visual acuity in the sympathizing eye Conclusions: Sympathetic ophthalmia after vitreoretinal surgeries is a rare but potentially sight threatening disease occurring in 0.038 percent of all cases of Pars Plana Vitrectomy. Presence of inflammation in the fellow eye after a vitreoretinal surgery in the other eye should alert the surgeon to possibility of sympathetic ophthalmia.

Progressive, subretinal fibrosis mimicking retinal necrosis with poor visual prognosis in sympathetic ophthalmia: A rare finding.

We report a rare finding of progressive subretinal fibrosis mimicking retinal necrosis in 2 cases of sympathetic ophthalmia. Histopathology of the inciting eye and vitreous biopsy of the sympathizing eye ruled out infections and masquerades. Progression of inflammation and rapid deterioration of vision inspite of maximum immunosuppression are key findings in this variant of sympathetic ophthalmia.

Long-Term Drug-Free Remission and Visual Outcomes in Sympathetic Ophthalmia.

PURPOSE: To assess corticosteroid- and immunosuppressive therapy (IST)-free long-term remission in the treatment of patients with sympathetic ophthalmia (SO), a vision-robbing disease that can span a lifetime. METHODS: The medical records of 19 patients with SO aged 16.1 to 94.95 years (median age 58.56 years) with median follow-up of 7.10 years (mean, 6.41; range, 2.5 to 8.63 years) were retrospectively examined. RESULTS: All patients achieved control of inflammation, 13 of them for 2 years or more. Three (15.78%) of the 19 patients maintained remission without IST and corticosteroids for more than 5 years with vision of 20/25 or better in the sympathizing eye. Thirteen patients (68.42%) were inactive on IST or corticosteroids or in combination therapy. Eleven patients (57.9%) maintained visual acuity of 20/40 or better at the end of follow-up. CONCLUSION: Even with a devastating and possibly lifelong disease like sympathetic ophthalmia, long-term remission off all IST and corticosteroids, and perhaps even cure, is possible.

Abnormal increase of intraocular pressure in fellow eye after severe ocular trauma: A case report.

BACKGROUND: An ocular injury can lead to secondary glaucoma in the traumatized eye in 3% to 20% of cases. Literature on the risk of developing elevated intraocular pressure in the nontraumatized fellow eye is scant. Clinicians treating ocular traumas should also bear in mind sympathetic ophthalmia, a rare bilateral granulomatous panuveitis following accidental or surgical trauma to 1 eye. CASE REPORT: We report a case of high-pressure glaucoma of the fellow eye without any signs of uveitis. The left eye of a 24-year-old man was injured in an inadvertent movement during a free-time table-tennis match. The eye was severely crushed, leading to blindness. His right eye developed medically uncontrolled high-pressure glaucoma only 1 month after the injury. CONCLUSION: To the best of our knowledge, there are no previous reports of post-traumatic glaucoma in the nontraumatized eye after open-globe injury.

Elemental analysis of melanins from bovine hair, iris, choroid, and retinal pigment epithelium.

According to recent theory on the structure of melanin, the sulfur content of melanin is a reflection of the amount of cysteine used in its manufacture. We compared the sulfur contents of melanins from hair, choroid, iris, and retinal pigment epithelium from black-haired cattle and separately from red-haired cattle. In black-haired cattle, sulfur contents of hair and ocular melanins were all between 0.60% and 0.94%, however, in red-haired cattle, hair melanin had a sulfur content (8.66%) different from those of choroid (0.94%) or RPE (1.72%) MELANIN. One may conclude that in an individual animal, ocular melanins can have different chemical structures from hair melanins. The clinical importance of this finding is discussed with regard to sympathetic ophthalmia.

32 cases of sympathetic ophthalmia. A retrospective study at the National Eye Institute, Bethesda, Md., from 1982 to 1992.

OBJECTIVE: To examine the relationship between visual outcome and the clinical management of patients with sympathetic ophthalmia. METHODS: Thirty-two patients with sympathetic ophthalmia who were seen at the National Eye Institute, Bethesda, Md, between 1982 and 1992, were retrospectively reviewed. RESULTS: There were equal numbers of males and females. Sympathetic ophthalmia occurred after trauma in 23 patients and surgery in nine patients. Sixteen of the 32 patients had a final visual acuity of 20/40 or better; 10 patients had a visual acuity worse than 20/200. Good visual outcome was associated with early and aggressive treatment with corticosteroids, sometimes in combination with other immunosuppressive agents. Poor visual acuity was associated with glaucoma, chorioretinal scars in the macula, and persistent uncontrolled inflammation. CONCLUSION: Prompt and aggressive use of antiinflammatory therapy can improve the visual outcome of patients with sympathetic ophthalmia.

Effect of corticosteroid treatment and enucleation on the visual prognosis of sympathetic ophthalmia.

A retrospective clinicopathologic study, conducted to determine the course of disease in 30 cases of sympathetic ophthalmia with clinical follow-up of at least six months (average, 12.7 years), showed that 21 of 30 patients retained visual acuities of 20/50 or better in the sympathizing eye. The severity of inflammation graded histologically in the exciting eye did not correlate with the clinical course of final visual acuity in the sympathizing eye. Enucleation of the exciting eye within two weeks of the onset of symptoms was associated with a relatively benign clinical course (P = .008). Corticosteroid treatment in patients who underwent enucleation more than two weeks after the onset of symptoms was associated with a good visual outcome (P = .009). Early enucleation of a blind exciting eye and corticosteroid treatment appear to be important variables that influence the visual prognosis in the sympathizing eye.

[Long-term immunosuppressive treatment of sympathetic ophthalmia]. / Dlugotrwale stosowanie leczenia immunosupresyjnego w zapaleniu wspólczulnym oka.

The authoresses present the results of treatment of 5 patients with sympathetic ophthalmia caused by injury of the eye. In 3 patients the removal of the excited eye and several years lasting conservative treatment--between others by immunosuppressive compounds--led to the arrest of the inflammatory process and enabled to preserve a good visual acuity in the saved eye. In 2 persons the function of either eye however could not be saved.

The role of perivascular melanophage infiltrates in the conjunctiva in sympathetic ophthalmia.

PURPOSE: To report pathologic changes in the conjunctiva from the exciting eye in a case of sympathetic ophthalmia (SO). METHODS: Report of clinical findings and conjunctival histopathology in a patient with SO. RESULTS: A 50-year-old male developed SO, with unusual peribulbar conjunctival pigmentation in the inciting eye. Histological examination of the conjunctival biopsy revealed perivascular distribution of CD68(+) melanophages that also expressed HLA-DR, suggesting that these macrophages may act as antigen-presenting cells. In addition, increased CD4(+) and CD3(+) lymphocytes were noted in the subconjunctival space when compared to specimens of normal conjunctiva and traumatic uveal prolapse without SO, suggesting T-cell recruitment. CONCLUSIONS: These pathologic findings suggest a possible mechanism by which local antigen processing by subconjunctival melanophages may play a role in the initiation of the complex cell-mediated response seen in SO.