Cutting-edge issues in sympathetic ophthalmia approach and six case reports: a T cell-mediated autoimmune response.

Sympathetic ophthalmia is a rare and potentially devastating bilateral diffuse granulomatous panuveitis. It is caused by surgical or non-surgical eye injuries and is an uncommon and serious complication of trauma. It is diagnosed clinically and supported by imaging examinations such as ocular ultrasonography and optical coherence tomography. Its treatment consists of immunosuppressive therapy with steroids and sometimes steroid-sparing drugs, such as cyclosporine, azathioprine, cyclophosphamide, and mycophenolate mofetil. Fast and effective management with systemic immunosuppressive agents allows for disease control and achievement of good visual acuity in the sympathizing eye. By contrast, enucleation should be considered only in situations where the injured eye has no light perception or in the presence of severe trauma. In addition to a bibliographic review of this topic, we report six cases involving different immunosuppressive and surgical treatment modalities.

Long-term clinical outcomes of patients with sympathetic ophthalmia.

PURPOSE: To present the long-term clinical outcomes of patients with sympathetic ophthalmia (SO). METHODS: Retrospective review of patients' medical files between 2002 and 2022. RESULTS: Included were seven patients (four males). The mean ± SD age at presentation was 37.9 ± 22.5 years. Four patients had co-morbidities: three had diabetes mellitus type 2 and one had Turner Syndrome. Trauma was the inciting event in six patients and postoperative endophthalmitis in one patient. Decreased visual acuity (VA) was the leading symptom in the sympathizing eye and all of the patients presented with panuveitis. The mean ± SD interval between the triggering incident and the onset of SO in six cases was 4.3 ± 4.2 months. One case presented 30 years following the eye injury. Five patients underwent enucleation/evisceration of the exciting eye. The mean ± SD presenting LogMAR BCVA in the sympathizing eye was 0.57 ± 0.82, and the final LogMAR BCVA was 0.61 ± 0.95. Inflammation was completely controlled in 5 patients at a mean ± SD of 8.55 ± 9.21 months following the institution of immunomodulatory therapy, and it was partially controlled in 2 patients. VA deteriorated in all 3 diabetic patients and improved or remained stable in the 4 young and healthy patients. The mean ± SD follow-up period after achieving drug-free remission was 28 ± 22.8 months. The mean ± SD follow-up time was 6.8 ± 5.6 years. CONCLUSIONS: SO is one of the most sight-threatening conditions, affecting the healthy eye. In this cohort, the favorable visual outcome was especially seen in young and healthy individuals. Visual prognosis is directly related to prompt diagnosis and treatment.

The Epidemiology and Risk Factors for the Progression of Sympathetic Ophthalmia in the United States: An IRIS Registry Analysis.

PURPOSE: To investigate the demographic and clinical characteristics of patients with sympathetic ophthalmia (SO) and define the risk factors for its incidence following trauma and ophthalmic procedures. DESIGN: Retrospective cohort study. PARTICIPANTS: Patients in the American Academy of Ophthalmology's (Academy) IRIS Registry (Intelligent Research in Sight) who were (n=1523) or were not diagnosed with SO following a documented procedure or trauma between January 1, 2013, and December 31, 2019. METHODS: Multiple demographic and clinical factors were collected, descriptive statistics and prevalence were calculated, and multivariate linear regression models were fit to the data. MAIN OUTCOME MEASURES: Prevalence of SO, demographic and clinical characteristics, and beta coefficient (ß) estimates of demographic and clinical characteristics impacting time to SO onset after procedure (Procedure Only cohort) or trauma (Trauma cohort). RESULTS: Of 65,348,409 distinct IRIS Registry patients, 1523 (0.0023%) were diagnosed with SO between 2013 and 2019, and also had a documented preceding trauma or procedure. Of these, 927 (60.87%) were female, 1336 (87.72%) belonged to the Procedure Only cohort, and 187 (12.28%) belonged to the Trauma cohort. The prevalence of SO after trauma was 0.0207%, whereas after procedures it was 0.0124%. The highest risk of procedure-related SO was seen in patients with history of "other anterior segment" (0.122%) followed by glaucoma (0.066%) procedures, whereas the lowest prevalence was noted with cataract surgeries (0.011%). The average time to onset of SO across both cohorts combined was 527.44 (±715.60) days, with statistically significant differences between the 2 cohorts (P < .001). On average, the time to onset from inciting event to SO was shorter with increasing age, by 9.02 (95% CI: -11.96, -6.08) days for every 1-year increase. CONCLUSIONS: SO following trauma and ophthalmic procedure is potentially rarer than previously reported, as measured in this large ophthalmic medical record database. Female sex may be a risk factor for SO. Older age may be a risk factor for quicker onset. These findings can guide clinical decision-making and management.

Vision Outcomes of Long-Term Immunomodulatory and Steroid Therapy in Sympathetic Ophthalmia.

PURPOSE: To compare vision acuity outcomes of long-term steroid therapy compared with immunomodulatory therapy for treatment of sympathetic ophthalmia. DESIGN: Single-center, retrospective, comparative clinical study. METHODS: Patients with sympathetic ophthalmia treated from March 2005 to October 2022 with at least 1 year of follow-up were included. Visual acuity outcomes were compared by steroid and immunomodulatory treatment modality. RESULTS: Thirty-five patients with sympathetic ophthalmia were included in the study, with follow-up ranging from 1 to 17 years. Higher rates of vision loss correlated with longer periods of active uveitis and steroid treatment. Lower rates of vision loss correlated with longer periods of uveitis remission on immunomodulatory therapy alone and drug-free remission. Treatment with alkylating agents or combination therapy with an antimetabolite, a biologic-response modifier, and cyclosporine are more likely to result in sympathetic ophthalmia remission. CONCLUSION: Immunomodulatory therapy leads to superior vision outcomes in cases of steroid-resistant or recurrent sympathetic ophthalmia. Steroid therapy may be useful for acute or recalcitrant sympathetic uveitis but is insufficient for long-term inflammatory control. PRéCIS: This manuscript describes a retrospective analysis of vision outcomes in patients with sympathetic ophthalmia. Results indicate that long-term immunomodulatory therapy is associated with better vision outcomes than long-term steroid therapy for sympathetic ophthalmia treatment.

Material risk: vitreoretinal surgery, evisceration, enucleation and sympathetic ophthalmia-where are we currently?

Sympathetic ophthalmia (SO) is known to occur after severe penetrating eye injury, evisceration and even enucleation surgery. Recent evidence suggests that a greater risk lies after multiple vitreoretinal procedures. The risk of SO following evisceration is only minimally greater than that following enucleation surgery. This review evaluates literature on SO to date and provides figures for the risk of developing SO for the purposes of the consent process. The issue of SO and Material Risk following vitreoretinal surgery is reviewed and figures for the purposes of consent are outlined. This is of particular relevance for patients in whom the contralateral eye is and will likely remain the better seeing eye. Sympathetic ophthalmitis is known to occur after severe penetrating eye injury, after evisceration and enucleation. More recently, sympathetic ophthalmitis has been recognised to occur after vitreoretinal surgery. This article reviews the evidence on material risk when consenting patients for elective and emergency eye procedures after ocular trauma or surgery. When a globe needs to be removed because of irreparable ocular injury, previous publications dictated the procedure to be an enucleation because of the fear of an increased risk of SO after an evisceration. Perhaps the issue of material risk of sympathetic ophthalmia (SO) remains over-emphasised by ophthalmic plastic surgeons and under-recognised by vitreoretinal surgeons during the consent process for evisceration, enucleation and vitreoretinal surgery. Antecedent trauma and number of previous surgeries may actually be a more significant risk factor than the type of eye removal. Recent medicolegal cases also help us understand the importance of the discussion of this risk. We present our current understanding of the risk of SO after different procedures and suggest how this information may be included in a patient consent.

Sympathetic ophthalmia in an 85-year-old female and a 90-year-old male after a non-complicated cataract surgery: a case report.

Here, two cases involving the oldest reported patients with sympathetic ophthalmia (SO) after non-complicated cataract surgery, are reported. The first case was an 85-year-old female with bilateral decreased vision and headache. The best-corrected visual acuity (BCVA) was 0.8 in the right eye and 1.0 in the left eye at the initial visit. Pseudophakia and choroidal detachments were observed in both eyes, and retinal pigment epithelium undulation was observed by optical coherence tomography. The second case was a 90-year-old male with bilateral optic disc oedema. The BCVA was 0.09 in the right eye and 0.3 in the left eye with pseudophakic eyes. Optic disc oedema and choroidal thickening were observed in both eyes. Both patients were diagnosed with SO, and corticosteroid pulse treatments were started. The BCVA of the first case improved to 0.9 in the right eye and 1.2 in the left eye, while that of the second case reached 0.3 in the right eye and 0.6 in the left eye. No recurrence was observed in either case at 12 months after the initial visit. Both elderly patients with SO were successfully treated.

Sympathetic Ophthalmia: Demographic Characteristics, Clinical Findings, and Treatment Results.

Objectives: To evaluate the demographic characteristics, clinical findings, and treatment approach of patients with sympathetic ophthalmia (SO). Materials and Methods: The records of 14 patients with SO between 2000 and 2020 were retrospectively reviewed. The patients' Snellen best corrected visual acuity (BCVA), detailed ophthalmological examination, optical coherence tomography (OCT), enhanced depth imaging-OCT (EDI-OCT), fundus fluorescein angiography findings, and treatment approaches were recorded. Results: The study included the 14 sympathizing eyes of 14 patients with SO (7 female, 7 male). The mean age was 48.5±15.4 years (range: 28-75), and the mean follow-up duration was 55.1±48.7 months (range: 6-204). Ten patients (71%) had a history of ocular trauma and 4 (29%) had a history of ocular surgery. The time to symptom onset in the sympathizing eye after trauma or ocular surgery ranged from 15 days to 60 years. The most common posterior segment findings were optic disc edema (36%) and exudative retinal detachment (36%). In the acute period, the mean choroidal thickness value on EDI-OCT was 716.5±63.6 µm (range: 635-772) and decreased to 296±81.6 µm (range: 240-415) after treatment. Treatment with high-dose systemic corticosteroid was given to 8 patients (57%), azathioprine (AZA) to 7 (50%), AZA and cyclosporine-A combination to 7 (50%), and tumor necrosis factor-alpha inhibitors to 3 patients (21%). Recurrence was observed in 4 patients (29%) during follow-up. At last follow-up, BCVA values were better than 20/50 in 11 (79%) of the sympathizing eyes. Remission was achieved in 13 patients (93%), but 1 patient (7%) lost her vision due to acute retinal necrosis. Conclusion: SO is a bilateral inflammatory disease that presents with granulomatous panuveitis after ocular trauma or surgery. Favorable functional and anatomical results can be obtained with early diagnosis and initiation of appropriate treatment.

EIGHTY-TWO-YEAR-OLD MONOCULAR FEMALE WITH ACUTE DECREASED CENTRAL VISION.

PURPOSE: To present a case of suspected sympathetic ophthalmia in an 82-year-old monocular woman. METHODS: Case report. RESULTS: Here, we present an 82-year-old woman, status postcataract extraction with lens subluxation followed by a complicated course ultimately requiring enucleation because of a blind and painful eye in 2020, who developed contralateral choroidal lesions 6 months postenucleation along with vitritis and anterior uveitis. The lesions were suspicious for an infectious versus autoimmune etiology. Thorough systemic work-up and multimodal imaging suggest an autoimmune case. The findings in this patient suggest early identified sympathetic ophthalmia with multifocal choroiditis. This patient responded well to treatment of the acute episode with systemic corticosteroids and ultimately required steroid-sparing immunosuppression. CONCLUSION: Sympathetic ophthalmia is a rare entity classically observed after intraocular surgery and trauma. It may mimic many infectious and noninfectious uveitis entities. In this patient, sympathetic is a primary concern given her history and age of presentation with consideration for other uveitic entities.

Leopard retinopathy: An unusual presentation of sympathetic ophthalmia.

We report an unusual case of sympathetic ophthalmia (SO) in an elderly gentleman following multiple eye surgeries. He presented with diffuse granulomatous panuveitis resembling leopard retinopathy in the left eye. There was a delay in the initiation of effective treatment of his intraocular inflammation, but he responded to corticosteroid and azathioprine. The pigmentary changes in his fundus were highly unusual, and he was investigated extensively to rule out other possible causes including a search for occult malignancy. The delay in initiation of effective treatment or suboptimal therapy in SO, can lead to variable clinical picture in elderly patients. A proper screening to exclude any malignancy along with aggressive immunosuppressive therapy can achieve optimum results.

Sympathetic Ophthalmia - An Overview.

INTRODUCTION: Sympathetic ophthalmia (SO) is rare, bilateral granulomatous panuveitis that typically occurs following penetrating or perforating ocular trauma or surgery. This review aims to provide an update on the etiopathogenesis, clinical presentations, diagnosis and treatment of SO. METHODS: Reports cited in MEDLINE database, that analyzed SO in at least 5 patients, published prior to December 1st, 2021 were included. RESULTS: Initially, SO was associated with penetrating ocular trauma, however, various studies reported an increased incidence of SO after surgical procedures including vitreoretinal surgeries. Multimodal imaging including fluorescein and indocyanine green angiography, optical coherence tomography (OCT) and OCT angiography have added further insights into the understanding of SO. While pulse dose corticosteroids & immunosuppressive drugs are still the treatment of choice, TNF-α blockers & other biologic drugs represent new promising agents. CONCLUSION: There is a growing pool of evidence in understanding the pathogenesis of SO. Novel treatment options have provided better prognosis for this potentially blinding condition.

Sympathetic ophthalmia: A comprehensive update.

Sympathetic ophthalmia is a rare, bilateral, granulomatous, panuveitis following penetrating trauma or surgery to one eye. Clinical presentation commonly occurs within the first year of trauma occurrence but can be delayed by several years. It manifests as acute/chronic granulomatous uveitis with yellowish-white choroidal lesions or Dalen-Fuchs nodules. Initially, patients respond rapidly to corticosteroid therapy, but a majority require long-term use of corticosteroid-sparing agents to prevent recurrences. The purpose of this review is to elaborate on the current understanding of the pathophysiology, the importance of multimodal imaging in early diagnosis, and the role of newer immunomodulatory and biological agents in recalcitrant cases.

Sympathetic Ophthalmia - case report.

Purpose. To present the case of a 22-year-old man with a history of trauma on the right eye, followed by a sudden decrease of visual acuity on the left eye, but with a good recovery after surgical treatment. Material and methods. We reported a case of a 22-year-old patient with a sudden and painless decrease of visual acuity on the left eye, a month after a car accident, which led to the laceration of the right globe. At first, the patient received only medical treatment because he refused any surgical intervention. He had a favorable evolution during hospitalization, but he returned after a month with the same visual acuity as at his first admission. The patient accepted the medical treatment and the enucleation of the right eye, thus having a fast improvement in his visual acuity on the left eye. Conclusions. Although the enucleation was overdue, it had a strong favorable influence on the evolution of the disease. As a result of the surgery, the visual acuity has improved significantly in just a few days. Abbreviations: OCT = optical coherence tomography.

Clinical classification, visual outcomes, and optical coherence tomographic features of 48 patients with posterior sympathetic ophthalmia.

BACKGROUND: To investigate the clinical manifestations, visual outcomes and optical coherence tomographic (OCT) features of patients with posterior sympathetic ophthalmia (PSO). METHODS: We performed a retrospective review of 48 patients diagnosed with PSO between January 2013 and December 2019. We compared the clinical and OCT features among different clinical types of PSO. RESULTS: PSO could be classified into two types according to whether the fundus exhibited serous retinal detachment (SRD) or multifocal choroiditis (MFC). There were 41 patients (85.4%) with SRD and 7 patients (14.6%) with MFC. The latent period of patients with MFC was significantly longer than that of patients with SRD (P = 0.002). The final visual acuity of patients with MFC was significantly worse than that of patients with SRD (P = 0.0001). In patients with acute SRD, OCT revealed that the mean height of retinal detachment in the fovea was 528.8 ± 437.5 µm. After treatment, the retina reattached in all patients and the band structures of the outer retina were restored in most patients (92.7%). In patients with acute MFC, the OCT images revealed inflammatory lesions on the retinal pigment epithelium layer. After treatment, the OCT images showed hyperreflective fibrosis of the lesions and loss of the outer retinal band structures in all patients. CONCLUSIONS: We found that PSO could be classified according to the presence of SRD or MFC. The visual prognosis differed significantly between these types of PSO. OCT imaging is useful for clinical classification and monitoring of retinal changes after treatment.

Clinical and imaging features of sympathetic ophthalmia and efficacy of the current therapy.

PURPOSE: The purpose of the study was to clarify the clinical and imaging features of sympathetic ophthalmia (SO) and evaluate the efficacy of the current therapy. METHODS: The databases PubMed, EMBASE and Ovid up to January 2021 were searched to identify relevant studies. R software version 3.6.3 was used to perform the statistical analyses. RESULTS: Thirty-two studies involving 1067 patients were finally included. Our study found SO was male-dominated, and more than half of SO patients aged 16 to 60 years old. Ocular trauma, surgical interventions and unknown events were estimated to be inciting events in 63%, 36% and 4% of SO patients. About 35% of the patients underwent baseline enucleation, and 45% took compelled enucleation during follow-up. The most common symptoms at the first presentation were decreased vision, followed by pain and redness. The most common signs were anterior chamber cells/flare, followed by vitritis, exudative retinal detachment and Dalen-Fuch nodules. Choroidal thickening was detected in 81% of SO patients by ocular ultrasound. The most common fluorescein fundus angiography signs were disc leakage. After corticosteroid therapy became the mainstay for SO, about 76% of SO patients could get inflammation well-controlled, while 24% of them might have recurrent inflammation. Around 72% of SO patients could achieve visual improvement, and more than half of them might have a best-corrected visual acuity of 20/50 or better. CONCLUSION: SO is a complicated ocular disease with diverse clinical manifestations and imaging features. After proper anti-inflammation therapy, SO might not necessarily result in a poor prognosis.

The Risk of Sympathetic Ophthalmia after Vitreoretinal Surgery.

PURPOSE: To investigate the clinical course and outcomes of sympathetic ophthalmia (SO) and correlate these with the nature of the inciting event and the number of vitreoretinal (VR) procedures undergone by patients. DESIGN: A retrospective case review. SUBJECTS: All patients diagnosed with SO who had been treated or monitored at a single center over a 15-year period. METHODS: A search of the electronic patient record system at Moorfields Eye Hospital, London, over a 15-year period (between January 2000 and December 2015) was carried out using the search terms "sympathetic," "ophthalmia," and "ophthalmitis." Sixty-one patients with available records were identified, and data were collected from their complete electronic and paper records. MAIN OUTCOME MEASURES: The main outcome measures were best-corrected visual acuity at 1 year and at the end of follow-up and the number of VR surgical procedures preceding the diagnosis of SO. Data on patient age, sex, disease duration, ocular and systemic manifestations, ocular complications, retinal angiography, and treatment were also collected. RESULTS: There was a wide age range at presentation (2-84 years), and the length of follow-up ranged from 1 to 75 years. The first ocular event was trauma in 40 patients and surgery in 21 patients. Vitreoretinal surgery accounted for 13 of the 21 (62%) surgical first-event triggers. Twenty-three of 61 (38%) patients underwent VR surgery (1-7 operations) at some point before diagnosis. Surgical details were available for 15 patients, who had undergone a total of 25 VR procedures. Based on the surgical activity of the unit, the risk of developing SO after a single VR procedure was estimated to be 0.008%, rising to 6.67% with 7 procedures. A total of 23 (38%) patients experienced a decrease in acuity at the end of the follow-up period, vs. 9 (15%) patients experiencing an improvement and 18 (30%) remaining unchanged. CONCLUSIONS: We feel that the most significant finding in this study is the calculated risk of SO development after a single VR procedure, which was significantly lower in our cohort than that previously reported in the literature. This was seen to rise exponentially with additional procedures.

Atypical Sympathetic Ophthalmia Presenting with Ocular Pain Associated with Posterior Scleral Involvement.

PURPOSE: To describe an atypical case of sympathetic ophthalmia. Design: Case report. RESULTS: A 37 -year-old female presented a 3-day long acute left retroocular pain and photophobia, 1 month after having undergone evisceration of the fellow eye. Upon exploration, the patient presented conjunctival injection, macular retinal folds with peripapillary subretinal fluid, and hypocyanescent choroidal spots on indocyanine green angiography. A sympathetic ophthalmia with a reactive posterior scleritis involvement was diagnosed. The patient underwent treatment with prednisone, mycophenolate, and cyclosporine with slowly tapering, presenting a total recovery over the years. CONCLUSION: Sympathetic ophthalmia may present itself atypically as ocular pain with little vision loss secondarily to a mild panuveitis with reactive scleral involvement.

Incidence of Sympathetic Ophthalmia after Inciting Events: A National Database Study in Japan.

PURPOSE: To analyze the incidence of sympathetic ophthalmia (SO) after inciting events (eye trauma or intraocular surgery). DESIGN: Retrospective cohort study. PARTICIPANTS: Patients experiencing inciting events between 2012 and 2019. Onset of SO was defined as the first date of SO diagnosis. METHODS: Using a nationwide administrative claims database in Japan, we calculated the cumulative incidence of SO after inciting events stratified by sex, 10-year age groups, and a categorical variable of primary or repeated, reflecting the history of inciting events in the past year (no inciting events, inciting events without trauma, or inciting events with trauma) using the Kaplan-Meier approach. We also estimated the adjusted hazard ratio (aHR) by Cox regression. We then restricted the population to those with only 1 inciting event during the observation period to investigate the pure effect of each inciting event. MAIN OUTCOME MEASURES: Cumulative incidence of SO over 60 months. RESULTS: A total of 888 041 inciting events (704 717 patients) were eligible. The total number of SO cases was 263, and the cumulative incidence of SO was 0.044% over 60 months. Female sex was not associated with onset of SO (aHR, 1.01; 95% confidence interval [CI], 0.79-1.29; P = 0.95). The group 40 to 49 years of age showed the highest incidence of 0.104% among the age groups (aHR vs. ≥80 years of age group [0.041%], 2.44 [95% CI, 1.56-3.80]; P < 0.001). Repeated inciting events with and without trauma showed higher incidences of SO (0.469% and 0.072%, respectively) than primary inciting events (0.036%) (aHR 11.68 [7.74-17.64] and 2.21 [95% CI, 1.59-3.07], respectively); P < 0.001 and P < 0.001, respectively). The incidence of SO after vitrectomy was much lower than after trauma (0.016% vs. 0.073%), and the incidence after scleral buckling was even lower. CONCLUSIONS: The cumulative incidence of SO over 60 months was estimated to be 0.044% at minimum. Repeated inciting events, especially those with trauma, increased the risk of SO developing. Trauma was 4 to 5 times as likely to induce SO than vitrectomy. The present findings will be valuable for counseling patients about the risks of SO after trauma and before performing intraocular surgeries.