Incidence of Sympathetic Ophthalmia after Intraocular Surgery: A Systematic Review and Meta-analysis.

TOPIC: Sympathetic ophthalmia (SO) is a rare bilateral granulomatous panuveitis that can present after trauma or intraocular surgery (IOS). The incidence of SO after IOS varies among studies. The purpose of this review was to determine the incidence proportion of SO after IOS. CLINICAL RELEVANCE: The incidence proportion of SO after IOS can provide physicians and patients with information on the risk of SO during the consent process before surgery. METHODS: In this systematic review and meta-analysis, MEDLINE, EMBASE, and Cochrane databases were searched from inception to January 1, 2023 for population-based studies of SO after IOS. Two reviewers independently screened the results. Random-effects meta-analyses calculated incidence proportion. Subgroup analysis assessed SO incidence based on IOS type and technological advancements. Study quality and bias were assessed using the Newcastle-Ottawa scale and the Grades of Recommendation, Assessment, Development, and Evaluation framework. RESULTS: The final meta-analyses included 19 studies, with 118 cases of SO occurring after 505 178 inciting events. The estimated overall incidence proportion of SO after IOS was 0.061% (95% confidence interval [CI], 0.033%-0.111%; I2 = 83%), and the estimated incidence rate was 9.24 cases per 100 000 person-years (95% CI, 4.03-21.19; I2 = 88%). The average study duration across these studies was 10.8 years. Within the reviewed literature, SO after glaucoma and vitreoretinal IOS was studied most, with 9 and 6 studies, respectively. Observed differences in incidence between glaucoma (0.098%; 95% CI, 0.042%-0.232%; I2 = 40%) and vitreoretinal (0.043%; 95% CI, 0.022%-0.085%; I2 = 88%) IOS were not statistically significant (P = 0.14). Also, no significant difference was found in the incidence proportion before and after 1975, when modern intraocular surgical techniques emerged (0.060% vs. 0.058%; P = 0.98). The outcome measures showed low-certainty Grades of Recommendation, Assessment, Development, and Evaluation evidence. DISCUSSION: Sympathetic ophthalmia after IOS is rare and might not have changed over the past 5 decades. The estimated incidence proportion of SO may be useful during the consent process before surgery. Also, no significant difference may exist in the incidence of SO between glaucoma and vitreoretinal IOS, based on low-certainty evidence. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

The Ruptured Globe, Sympathetic Ophthalmia, and the 14-Day Rule.

PURPOSE: Prophylactic enucleation of a ruptured globe with no light perception within 14 days of injury to prevent sympathetic ophthalmia (SO) has been an established dictum in academic teaching for more than 100 years. This treatment strategy was originally based on observation, speculation, and careful thought, but there was never any scientific proof. This review summarizes and updates the current state of our knowledge about globe rupture and SO, examines the origin and validity of the 14-day rule, and emphasizes the importance of trying to save the traumatized eye whenever possible. METHODS: A comprehensive literature review of SO and globe rupture was performed. RESULTS: SO is a rare disorder that may potentially occur following traumatic globe rupture as well as following a variety of other intraocular surgeries. Vitreoretinal surgery may be a more common cause than trauma according to some studies. SO may still occur despite having the eye removed within 14 days of the trauma. A variety of new medications including biologic agents are now available to treat SO with improved efficacy in suppressing the associated ocular inflammation and allowing retention of some useful vision. Removing the traumatized, blind eye may have other important psychological consequences associated with it that require consideration before eye removal is carried out. Retaining the blind, phthisical, disfigured eye avoids phantom vision and phantom pain associated with enucleation as well as providing a good platform to support and move an overlying prosthetic eye. Data on the occurrence of SO following evisceration and enucleation with and without predisposing factors confirms the exceedingly low risk. CONCLUSION: Most civilian open globe injuries can be successfully repaired with modern, advanced microsurgical techniques currently available. Because of the exceedingly low risk of SO, even with the severity of open globe trauma during military conflicts being more devastating as a result of the blast and explosive injuries, today every attempt is made to primarily close the eye rather than primarily enucleate it, providing there is enough viable tissue to repair. The 14-day rule for eye removal after severe globe ruptures is not scientifically supported and does not always protect against SO, but the safe time period for prophylactic eye removal is not definitively known. In the exceptional cases where SO does occur, several new medications are now available that may help treat SO. We advocate saving the ruptured globe whenever possible and avoiding prophylactic enucleation to prevent the rare occurrence of SO. When an eye requires removal, evisceration is an acceptable alternative to enucleation in cases that do not harbor intraocular malignancy.

Sympathetic ophthalmia after 27-G pars plana vitrectomy.

BACKGROUND: Sympathetic ophthalmia (SO) is a bilateral diffuse uveitis that can arise after ocular trauma or ocular surgery in the inciting eye. Pars plana vitrectomy (PPV) is one of the risk factors for SO. Several reports have described SO developing after 23- and 25-G PPV, but none have described SO occurring after 27-G PPV. We describe herein a case of SO after 27-G PPV for rhegmatogenous retinal detachment. CASE PRESENTATION: A 42-year-old woman presented with visual disturbance in the right eye. Best-corrected visual acuity (BCVA) was 6/200 in the right eye. Fundus examination revealed off-macula retinal detachment with retinal tears at both ends of retinal lattice degeneration at the temporal-oven peripheral retina of the right eye. We therefore performed 27-G sutureless PPV on the right eye. After 12 days, the retina was reattached, and BCVA improved to 6/30 in the right eye. Fifteen days postoperatively, she experienced headache and reduced vision in both eyes. Symptoms gradually worsened, and she visited our hospital 21 days postoperatively. BCVA was 6/30 in the right eye and 6/15 in the left eye. Slit-lamp examination revealed uveitis in the anterior chambers of both eyes, and fundus examination showed papillitis and subretinal detachment at the posterior poles of both eyes. Optical coherence tomography revealed subretinal fluid in the maculae of both eyes and fluorescein angiography showed multiple hyperfluorescent leakage sites in the retinal pigment epithelium. Cerebrospinal fluid examination showed pleocytosis and human leukocyte antigen testing showed expression of the DR04 phenotype; therefore, the patient was diagnosed with SO. She was treated with steroid therapy, and her visual disturbance subsided and the subretinal fluid improved as well. Her BCVA was 6/15 for the right eye and 6/5 for the left eye 93 days after the initial surgery. CONCLUSION: The present case shows that even if the sclerotomy site of 27-G PPV is small, there is still a risk of SO occurring in the eyes of patients who underwent transconjunctival vitrectomy. Ophthalmologists should recognize SO as complication of 27-G PPV and carry out proper management as early as possible.

SYMPATHETIC OPHTHALMIA: Clinicopathologic Correlation in a Consecutive Case Series.

PURPOSE: To correlate the clinical course of sympathetic ophthalmia with the histological and immunohistochemical characteristics of the enucleated inciting eye. METHODS: A consecutive case series with baseline clinical features and subsequent histopathologic findings. RESULTS: Evaluation of the 16 enucleated inciting eyes (blind and painful) disclosed that 9 of the 16 had typical histology, fulfilling the criteria for sympathetic ophthalmia of diffuse granulomatous inflammation. Among the 16, 11 sustained previous penetrating trauma, 4 underwent previous eye surgery, and 1 patient presented with an unknown etiology. Patients with atypical histology (7 of 7) were taking corticosteroids at the time of enucleation. Only 2 of 9 patients with typical histology were taking corticosteroids at the time of enucleation. At 6 months after enucleation of the inciting eye, 4 of the 7 patients with atypical histology had a visual acuity of ≥20/40 compared with 8 of 8 patients (100%) with typical histology. On a 4-point scale (0-3+), the choroidal infiltrate of the 9 histopathologically typical eyes showed an average of 2.5+ CD68 (macrophages), 2.5+ CD20 (B cells), and 1.5+ CD3 (T cells). CONCLUSION: Histopathologic findings had minimal correlation with the clinical course of sympathetic ophthalmia. Corticosteroid treatment before enucleation may influence the pathologic confirmation of sympathetic ophthalmia. The predominance of B lymphocytes and macrophages over T lymphocytes may represent different stages of the disease process.

Incidence of hypotony and sympathetic ophthalmia following trans-scleral cyclophotocoagulation for glaucoma and a report of risk factors.

OBJECTIVE: To report the incidence and risk factors for hypotony and estimate the risk of sympathetic ophthalmia following diode laser trans-scleral cyclophotocoagulation (TSCPC). DESIGN: Retrospective study using data from a private tertiary glaucoma clinic and review of the literature. PARTICIPANTS: Seventy eyes of 70 patients with refractory glaucoma who received TSCPC treatment. METHODS: Review of the records of consecutive patients who underwent TSCPC by a single ophthalmic surgeon and review of the literature. MAIN OUTCOME MEASURES: Hypotony (including phthisis bulbi), sympathetic ophthalmia. RESULTS: Seven eyes (10%; CI 5-19%) developed hypotony and included 4 eyes that developed phthisis. Higher total energy delivered during TSCPC treatment was associated with an increased risk of hypotony: eyes that developed hypotony received a mean total energy of 192.5 ± 73.2 joules, compared to a mean of 152.9 ± 83.2 joules in hypotony-free cases. The risk of sympathetic ophthalmia estimated from a review of the published literature and current series was one in 1512, or 0.07% (CI 0.03%-0.17%). CONCLUSIONS: Total laser energy is one of several risk factors that act in a sufficient component cause-model to produce hypotony in an individual patient. The small sample size precluded inference for other individual putative risk factors but titrating laser energy may help decrease the occurrence of hypotony. The risk of sympathetic ophthalmia calculated from the literature is likely an overestimate caused by publication bias.

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.

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 - 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.

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 - 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.

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.

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.

The Incidence of Sympathetic Ophthalmia After Trauma: A Meta-analysis.

PURPOSE: Sympathetic ophthalmia (SO) is a rare, bilateral panuveitis that occurs following open globe injury (OGI), with a variable incidence reported in the literature. Our objective was to determine the incidence proportion and incidence rate of SO following OGI to help guide shared physician-patient decision making. DESIGN: Systematic review and meta-analysis. METHODS: A systematic literature search was performed using the MEDLINE, EMBASE, and Cochrane databases from inception to November 2020 for population-based studies on OGI and SO in adults and children. Two reviewers independently screened search results. Random-effects meta-analyses were performed to calculate the incidence proportion and incidence rate. The Risk Of Bias In Non-Randomized Studies - of Interventions (ROBINS-I) tool was used to assess the risk of bias. The study was registered on PROSPERO CRD42020198920. RESULTS: A total of 24 studies were utilized in the meta-analyses. After OGI, the estimated overall incidence proportion of SO was 0.19% (95% CI 0.14%-0.24%) and the incidence rate of SO was 33 per 100,000 person-years, (95% CI 19.61-56.64) with I2 of 13% and 72%, respectively. CONCLUSIONS: SO after OGI is rare. The estimated incidence proportion and incidence rate are useful when counselling patients regarding management options after OGI. Further studies are needed to examine the influence of age, the extent and location of trauma, timing of repair, and prophylactic eye removal on the incidence of SO.

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.

A case of sympathetic ophthalmia after a severe acanthamoeba keratitis.

PURPOSE: To report a case of sympathetic ophthalmia (SO) after a severe Acanthamoeba keratitis. METHODS/DESIGN: Interventional case report. RESULTS: A 59-year-old white woman, wearing contact lenses, developed a severe Acanthamoeba keratitis in the left eye, which involved the limbus, and required 8 months of intensive antiamoeba therapy; the condition resolved leaving a painful, phthisical eye with complete corneal neovascularization. Six months later, the patient presented with pain, blurred vision, and photophobia in the right eye. Slitlamp examination of the right eye revealed granulomatous uveitis. On the suspicion of an SO, treatment with high-dose topical and oral corticosteroids and immunosuppressants was started. After 3 months, the eye is stable, with a visual acuity of 20/50, and the patient is taking prednisolone 7.5 mg per day and cyclophosphamide 50 mg per day. CONCLUSIONS: [corrected] Development of SO in the absence of previous trauma or surgery is rare. Our case is the first report of a clinically diagnosed SO after an episode of severe Acanthamoeba keratitis.

Sympathetic ophthalmia: Report of a case series and comprehensive review of the literature.

PURPOSE: To evaluate the factors related to sympathetic ophthalmia (SO) in a series of patients in our ophthalmology center and previously published cases. METHODS: A retrospective and noncomparative review was performed on 16 patients with SO attending our ophthalmology center from 2013 to 2019. A total of 87 previously published cases of SO were identified by searching the Medline database from 2009 to 2019. RESULTS: Sixteen patients were included in the analysis, and six cases were induced by transscleral cyclophotocoagulation (TCP). All patients had achieved controlled inflammation at their last follow-up visit. Thirteen patients (81.3%) had improved best-corrected visual acuity (BCVA). A review of the literature revealed 87 previously reported cases of SO. Shared clinical features and treatment outcomes were summarized. CONCLUSION: Ocular therapies, including both penetrating ocular therapy and non-penetrating ocular intervention, have become increasingly prevalent risk factors for SO, and the latent period has increased compared to past reports. Visual prognosis with appropriate medical management is relatively good.

NOVEL OPTICAL COHERENCE TOMOGRAPHY FINDINGS OF A CASE OF SYMPATHETIC OPHTHALMIA AFTER 23-GAUGE VITRECTOMY.

PURPOSE: Sympathetic ophthalmia (SO) is a rare panuveitis after trauma to one eye. We present a case of SO after 23-gauge transconjunctival vitrectomy, including optical coherence tomography evidence of response to treatment. To our knowledge, this is the first SO case report to include spectral domain optical coherence tomography images of Dalen-Fuchs nodules. METHODS: Observational case report of a case of SO after two pars plana vitrectomies in the fellow eye. RESULTS: A 67-year-old woman who had an operation for her pseudophakic left eye for a macula-off retinal detachment. She had two surgeries and later on had a total retinal detachment with vision of no perception of light. Eighteen months later, she presented back with a panuveitis with vitritis in her other eye and a visual acuity of 6/18. Dalen-Fuchs denoting SO was found. Treatment was commenced, and multimodal imaging was performed. CONCLUSION: Sympathetic ophthalmia is a known complication after vitrectomy. Optical coherence tomography can be invaluable in monitoring diagnosis and progression of Dalen-Fuchs nodules.