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.

Intraocular iron injection induces oxidative stress followed by elements of geographic atrophy and sympathetic ophthalmia.

Iron has been implicated in the pathogenesis of age-related retinal diseases, including age-related macular degeneration (AMD). Previous work showed that intravitreal (IVT) injection of iron induces acute photoreceptor death, lipid peroxidation, and autofluorescence (AF). Herein, we extend this work, finding surprising chronic features of the model: geographic atrophy and sympathetic ophthalmia. We provide new mechanistic insights derived from focal AF in the photoreceptors, quantification of bisretinoids, and localization of carboxyethyl pyrrole, an oxidized adduct of docosahexaenoic acid associated with AMD. In mice given IVT ferric ammonium citrate (FAC), RPE died in patches that slowly expanded at their borders, like human geographic atrophy. There was green AF in the photoreceptor ellipsoid, a mitochondria-rich region, 4 h after injection, followed later by gold AF in rod outer segments, RPE and subretinal myeloid cells. The green AF signature is consistent with flavin adenine dinucleotide, while measured increases in the bisretinoid all-trans-retinal dimer are consistent with the gold AF. FAC induced formation carboxyethyl pyrrole accumulation first in photoreceptors, then in RPE and myeloid cells. Quantitative PCR on neural retina and RPE indicated antioxidant upregulation and inflammation. Unexpectedly, reminiscent of sympathetic ophthalmia, autofluorescent myeloid cells containing abundant iron infiltrated the saline-injected fellow eyes only if the contralateral eye had received IVT FAC. These findings provide mechanistic insights into the potential toxicity caused by AMD-associated retinal iron accumulation. The mouse model will be useful for testing antioxidants, iron chelators, ferroptosis inhibitors, anti-inflammatory medications, and choroidal neovascularization inhibitors.

Oftalmia simpática / Sympathetic ophthalmia

Resumo Relatar um caso de um paciente portador de Oftalmia Simpática (OS), com descolamento seroso da retina documentado através de tomografia de coerência óptica de domínio spectral (SD OCT), indocianina verde (ICG) e angiofluoreceinografia (AGF), que o diagnóstico foi realizado em uma consulta de rotina e iniciado tratamento clínico .

Abstract To report the case of a patient with sympathetic ophthalmia (OS), with serous detachment of retinal documented by spectral domain optical coherence tomography (OCT), indocyanine green (ICG) and angiofluorecephography (AGF). The diagnosis was made in a routine consultation and clinical treatment was initiated.

Treatment Response in Sympathetic Ophthalmia as Assessed by Widefield OCT Angiography.

The authors report the widefield montage swept-source optical coherence tomography angiography (OCTA) findings in a 36-year-old male with sympathetic ophthalmia and illustrate the presence of multiple small areas of choriocapillaris flow voids that likely correspond to areas of choriocapillaris ischemia. Using sequential imaging, the authors observed that these flow voids improved following initiating of steroid and immunosuppressive therapy and almost completely resolved following 6 months of therapy with corresponding improvement in visual acuity. This report highlights the role of widefield montage OCTA to detect these likely ischemic changes within the choriocapillaris in sympathetic ophthalmia and demonstrates that they can be used as an anatomic marker to monitor treatment response. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:726-730.].

[Sympathetic ophthalmia : Therapy with steroid-free immunosuppressant azathioprine]. / Sympathische Ophthalmie : Therapie mit steroidsparendem Immunsuppressivum Azathioprin.

This article describes the case of a 48-year-old male patient who presented with persistent inflammation and deterioration of vision to a best corrected visual acuity (BCVA) of 0.6 in the only functioning left eye. The right eye had suffered a severe penetrating ocular trauma 6 months prior to presentation. After diagnosis of a sympathetic ophthalmia a high dosage corticosteroid therapy was initiated. Due to intolerance with decompensating diabetes an immunosuppressive therapy with azathioprine was initiated. This therapy resulted in stable clinical findings with an increase in BCVA to 0.9.

Bilateral hearing impairment as the initial symptom of sympathetic ophthalmia.

IMPORTANCE: Sympathetic ophthalmia (SO), a rare bilateral panuveitis following penetrating ocular trauma or ocular surgery to one eye, shares a strikingly similar ocular pathology to that of Vogt-Koyanagi-Harada disease (VKH). Audiovestibular dysfunction is a major extraocular manifestation of VKH; however, to date, only a few cases of sympathetic ophthalmia associated with hearing loss have been reported from ophthalmologists, but not otolaryngologists. Accordingly, little is known about the audiovestibular findings in patients with SO. We herein present two cases of SO with preceding bilateral hearing loss. OBSERVATIONS: The patient in Case 1, an 80-year-old female, experienced acute bilateral hearing loss. Five days after the onset of hearing loss, she presented with sudden bilateral blurred vision. In Case 2, a 32-year-old female noticed acute bilateral hearing loss and also experienced acute bilateral blurred vision the subsequent day. Patient 1 had a history of a penetrating injury to the right eye 25 days before the onset of hearing loss, while patient 2 had previously undergone right vitreous surgery twice for the treatment of a myopic macular hole and retinal detachment 36 and 43 days prior to the current symptom onset. Both cases were diagnosed as SO based on ocular findings of bilateral panuveitis and the history of ocular insult. Patient 1 carried HLA-DR4, HLA-DR15, HLA-A33, HLA-A24, HLA-B44 and HLA-B52, and patient 2 carried HLA-DR4. Audiograms showed bilateral mild to moderate sensorineural hearing loss in both cases, with normal auditory brainstem responses and deteriorated distortion product otoacoustic emission amplitudes. In addition, the significant recruitment phenomenon observed in case 1 suggested a cochlear origin of the hearing loss. Both patients received corticosteroid therapy, and the cochlear signs and symptoms recovered within one month. CONCLUSIONS AND RELEVANCE: This is the first report to describe the comprehensive audiovestibular findings in patients with SO. In the present study, acute bilateral hearing loss developed a couple of days prior to the onset of bilateral visual loss and auditory examinations suggested a cochlear etiology in both cases.

Mitochondrial oxidative stress initiates visual loss in sympathetic ophthalmia.

The visual loss that occurs with sympathetic ophthalmia (SO) in the absence of recognizable retinal damage and inflammatory cell infiltration is an enigma. Experimental autoimmune uveoretinitis (EAU) is an animal model used to study human endogenous uveitis. Both innate and adaptive immune responses have been well studied in the photoreceptor damage mechanism of EAU. In our studies, in the early phase of EAU, proinflammatory molecules such as tumor necrosis factor (TNF)-α and inducible nitric oxide synthase (iNOS) and the subsequent mitochondrial DNA damage, mitochondrial protein alteration, and mitochondrial dysfunction by oxidative stress were observed before retinal inflammatory cell infiltration. Our recent study shows the importance of Toll-like receptors (TLRs) in the production of proinflammatory molecules and the induction of mitochondrial oxidative stress. Thus, the innate immune responses occur first with the activation of TLRs; this activation upregulates proinflammatory molecules, leading to mitochondrial oxidative stress before retinal inflammatory cell infiltration and the subsequent adaptive immune responses. Like EAU, SO also results in photoreceptor mitochondrial oxidative damage without retinal inflammatory cell infiltration. Such damage was associated with TNF-α, TNF-α receptors, and iNOS expression in the photoreceptors, suggesting that this molecular mechanism without retinal inflammatory cell infiltration may initiate photoreceptor damage in SO.

Sympathetic ophthalmia.

Sympathetic ophthalmia is a rare, bilateral granulomatous panuveitis following accidental or surgical trauma to one eye. The pathophysiology is not clearly understood, but is believed to be a T-cell mediated autoimmune response. Clinical presentation most often occurs within the first year after trauma; anteriorly it manifests as a chronic or acute uveitis with mutton-fat keratic precipitates, while posteriorly it is often accompanied by yellowish-white choroidal lesions or Dalen-Fuchs nodules. The mainstay of treatment consists of systemic anti-inflammatory agents such as oral corticosteroids, or other immunosuppressive agents. Newer treatments include intravitreal injections of steroids in combination with systemic therapy, periocular injections of steroids without systemic therapy, and injections of infliximab, an anti-vascular endothelial growth factor.

The association of systemic disorders with Vogt-Koyanagi-Harada and sympathetic ophthalmia.

BACKGROUND: The aim of this work is to determine the systemic diseases and malignancy associated with Vogt-Koyanagi-Harada (VKH) disease compared to sympathetic ophthalmia (SO). METHODS: We conducted a retrospective comparative observational clinical study where the medical records of patients with the diagnosis of VKH and SO from 1999-2009 were reviewed. The study was carried out at the King Khaled Eye Specialist Hospital and The Eye Center in Riyadh, Saudi Arabia. Investigators recorded the age, gender, history of trauma, associated systemic disorders, and ocular and systemic manifestations. Patients were examined by an ophthalmologist as well as an internist. RESULTS: A total of 316 patients were included: 256 patients had VKH and 60 patients had SO. The age range in the VKH group was 3-62 years with a mean age of 29 ± 13 years. The age range in the SO group was 4-90 years with a mean age of 36 ± 20 years. The mean follow-up period of patients with VKH was 58 ± 50 months and patients with SO was 61 ± 54 months. Out of 256 patients with VKH, there were 41 (16%) with systemic disorders. Comparatively, out of 60 patients with SO, no associated systemic autoimmune disorders or tumors were encountered. The difference between the VKH and SO groups was statistically significant (p = 0.003). CONCLUSIONS: VKH and SO are autoimmune disorders targeting melanin-bearing cells. Both diseases are characterized by immunologic dysregulation. We found a statistically significant association of systemic disorders and malignancy with VKH compared to SO. This finding may suggest that the two disorders may have different etiology with similar ocular and systemic manifestations.

Reversible retinal changes in the acute stage of sympathetic ophthalmia seen on spectral domain optical coherence tomography.

To report the changes seen in the photoreceptor layer during the acute phase of sympathetic ophthalmia. Six consecutive patients diagnosed with sympathetic ophthalmia were enrolled in the study. All 6 patients had a fundus fluorescein angiogram and spectral domain optical coherence tomography (OCT) scan carried out at presentation. The outer retinal segment was demarcated on the raster line scan between the external limiting membrane (ELM) and the retinal pigment epithelium (RPE)-choriocapillaris complex. All patients received intravenous methylprednisolone followed by oral corticosteroids 1-1.5 mg/kg/day. The serial follow-up OCT scans taken 48 h after the initiation of treatment, and 1, 2 and 12 weeks later, were studied and compared. The retina inner to the ELM did not show any remarkable structural alteration in any of the eyes. The outer retinal segment demarcated by the ELM and the RPE-choriocapillaris complex showed serous retinal detachment in all the eyes and elongation of photoreceptors could be seen in four eyes. There was a disruption to the continuity of the two inner hyper-reflective bands in all the eyes. A repeat raster line scan performed 48 h after systemic corticosteroid therapy showed a reduction in the height of the serous retinal detachment with elongation of photoreceptors and the protrusion of their apical segments into the serous detachment in 4 eyes. The serous detachment showed a progressive reduction in height following intravenous methyprednisolone therapy in all the eyes over the following week with elongated photoreceptors in all six eyes. After two weeks, none of the eyes showed any residual detachment and this corresponded with the improvement in visual acuity in all the eyes. OCT raster line scans repeated at 4 weeks showed resolution of serous detachment with normal photoreceptor layer and restoration of a third hyper-reflective band (IS/OS junction) in all the eyes. The photoreceptor layer is involved during the acute phase of sympathetic ophthalmia as indicated by the in vivo morphological changes seen on spectral domain OCT. These changes are reversible following systemic corticosteroid therapy thus supporting the hypothesis that prompt and aggressive anti-inflammatory therapy is capable of reversing the photoreceptor changes.

Histopathological changes of cornea in long-term sympathetic ophthalmitis.

PURPOSE: To report the histopathological changes occurring in the cornea in sympathetic ophthalmitis. METHODS: Observational case series of 3 patients with sympathetic ophthalmitis who were treated with penetrating keratoplasty. We report the histopathological features of the cornea. RESULTS: In all 3 cases, the epithelium was edematous and bullous with extensive loss of the Bowman layer and calcification of the superficial stroma in all the 3 eyes. The deep stroma was unremarkable in appearance. Descemet membrane showed thickening in 2 of the corneas with nodular excrescences and folds noted in 1 of the specimens. The endothelium was attenuated with some residual cells containing pigment granules in 1 case. CONCLUSIONS: Endothelial loss and development of bullous keratopathy associated with band keratopathy are predominate histopathological features of the cornea. These changes are nonspecific and reflect a chronic anterior uveitis.

Sympathetic ophthalmia: incidence of ocular complications and vision loss in the sympathizing eye.

PURPOSE: To report the frequency on presentation and subsequent incidence of ocular complications and vision loss in patients with sympathetic ophthalmia (SO) and to describe factors associated with decreased vision in the sympathizing eye. DESIGN: Multicenter retrospective case series. SETTING: Three academic tertiary care uveitis clinics. STUDY POPULATION: Eighty-five patients with SO from 1976 to 2006. OBSERVATION PROCEDURES: Review of existing medical records. MAIN OUTCOME MEASURES: Incident visual acuity (VA) loss to 20/50 or worse and 20/200 or worse and the median acuity over time. RESULTS: Twenty-six percent of patients with SO presented with a VA of 20/200 or worse in their sympathizing eye. Further development of vision loss to 20/200 or worse occurred at the rate of 10% per person-year (PY). Ocular complications were seen in the sympathizing eye in 47% of patients at presentation; further development of new complications occurred at the rate of 40%/PY. The ocular complications most often associated with decreased vision were cataract and optic nerve abnormality. Exudative retinal detachment and active intraocular inflammation were significantly associated with poorer VA in the sympathizing eye. The benefits of corticosteroids were indirectly demonstrated as their use led to more rapid disease inactivation. Fifty-nine percent of patients maintained a VA of better than 20/50 in their sympathizing eye; and 75% maintained a VA of better than 20/200. CONCLUSIONS: Although ocular complications were seen in many sympathizing eyes with SO, most patients maintained functional VA. The presence of an exudative retinal detachment and active intraocular inflammation correlated with poorer vision in the sympathizing eye.

Subretinal hypopyon in sympathetic ophthalmia.

PURPOSE: To describe a rare case of sympathetic ophthalmia with subretinal hypopyon. METHODS: Observational case report. RESULTS: A 41-year-old man was diagnosed with sympathetic ophthalmia in his left eye. Oral prednisone was started. Three days later, he developed an inferior exudative retinal detachment with an underlying hypopyon. The exudative retinal detachment and hypopyon resolved completely within 48 hours. CONCLUSIONS: A subretinal hypopyon in sympathetic ophthalmia is a rare clinical finding. It is probably the result of acute inflammation in the subretinal space.