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.

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.

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.

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.

Recurrent neovascularization of the disc in sympathetic ophthalmia.

Sympathetic ophthalmia following parsplana vitrectomy is a known complication. We describe here a case of recurrent disc neovascularization in a patient of sympathetic ophthalmia. It promptly responded to steroids initially but later recurred with inflammation.

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.].

Bilateral exudative retinal detachments as initial presentation of sympathetic ophthalmia in Turner syndrome.

CASE REPORT: Sympathetic ophthalmia typically presents as bilateral granulomatous panuveitis that occurs after penetrating ocular injury. We describe a case of bilateral exudative retinal detachments in a patient with Turner syndrome and sympathetic ophthalmia. COMMENTS: To our knowledge, this is the first report of sympathetic ophthalmia in Turner syndrome.

[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.

Sympathetic ophthalmia.

Sympathetic ophthalmia is a rare, bilateral granulomatous uveitis that occurs after either surgical or accidental trauma to one eye. The ocular inflammation in the fellow eye becomes apparent usually within 3 months after injury. Clinical presentation is an insidious or acute anterior uveitis with mutton-fat keratic precipitates. The posterior segment manifests moderate to severe vitritis, usually accompanied by multiple yellowish-white choroidal lesions. Evidence suggests that sympathetic ophthalmia represents an autoimmune inflammatory response against choroidal melanocytes mediated by T cells. Diagnosis is based on clinical findings and a history of previous ocular trauma or surgery. Other causes of granulomatous uveitis, such as Vogt-Koyanagi-Harada disease, sarcoidosis, tuberculosis, and syphilis should be considered. Treatment of sympathetic ophthalmia consists of systemic anti-inflammatory agents with high dose oral corticosteroid as the drug of choice. However, if the inflammation cannot be controlled, cyclosporine is then used. Other immunosuppressive agents, such as chlorambucil, cyclophosphamide or azathioprine, may be necessary for the control of inflammation. The role of enucleation after the diagnosis of sympathetic ophthalmia remains controversial. Visual prognosis is reasonably good with prompt wound repair and appropriate immunomodulatory therapy.

[Choroidal neovascularization associated with sympathetic ophthalmia: case report]. / Neovascularização coroidiana associada à oftalmia simpática: relato de caso.

A 7-year-old boy with sympathetic ophthalmia secondary to penetrating ocular trauma developed choroidal neovascularization in the macular region of the sympathizing eye. Posterior segment biomicroscopy disclosed a single, round, small, slightly elevated yellow-white lesion temporal to the fovea, linked to the temporal edge of the optic disc by a fibrotic band. On fluorescein angiography the round lesion showed a gradual hyperfluorescence, with late staining and leakage, and was interpreted as a fibrovascular scar from previous choroidal neovascularization. Despite intense immunosuppressive therapy, the patient's final visual acuity was 20/400. Although rarely associated with sympathetic ophthalmia, choroidal neovascularization and its cicatricial stages can occur and compromise the visual prognosis of an already debilitated eye. The best course of treatment for choroidal neovascularization in these types of cases has not yet been determined.

Cataract extraction in the sympathizing eye.

Cataract extraction was performed on the sympathizing eyes of six patients with sympathetic ophthalmia. In five patients the clinical diagnosis of sympathetic ophthalmia was supported by the results of histologic examination of the exciting eye. The postoperative period averaged 12 years, and visual acuities ranged from 20/25 to light perception. The presence of posterior synechiae posed the major complicating operative factor. Cystoid macular edema, vitreous opacity, pupillary membrane formation, and corneal opacification were associated with postoperative visual impairment in four cases. We conclude that the underlying status of the sympathizing eye at the time of cataract extraction and postoperative management are critical factors that influence ultimate visual outcome.

Laser photocoagulation for choroidal neovascularization associated with sympathetic ophthalmia.

PURPOSE: To present a case of choroidal neovascularization associated with sympathetic ophthalmia successfully treated with photocoagulation. DESIGN: Interventional case report. METHODS: A 41-year-old man with a history of penetrating injury right eye, as well as sympathetic ophthalmia left eye, developed a classic choroidal neovascular membrane that threatened the center of the fovea. RESULTS: The patient was treated with argon laser photocoagulation. He has maintained stable visual acuity without evidence of recurrence of the membrane with 4 years' follow-up. CONCLUSION: Thermal laser should be considered as a treatment option in cases of choroidal neovascularization associated with sympathetic ophthalmia.

Sympathetic ophthalmia.

A 29-year-old woman developed severe ocular manifestations of sympathetic ophthalmia on the day after enucleation of a blind, painful eye, and four weeks after a penetrating ocular injury. She was observed for one year with fundus photography, fluorescein angiography, and electrophysiologic tests. Nystagmus, a rare systemic manifestation of sympathetic ophthalmia, was noted early in the course of her disease and was accompanied by vertigo, truncal ataxia, and cerebrospinal fluid pleocytosis. Enucleation and intensive corticosteroid treatment resolved the process; however, she has experienced recurrences in the two-year follow-up period.

Cataract surgery in sympathetic ophthalmia.

PURPOSE: To analyze the results of cataract surgery in patients with sympathetic ophthalmia. SETTING: Sankara Nethralaya, Medical Research Foundation, Chennai, India. METHODS: This study comprised 66 patients (132 eyes) with sympathetic ophthalmia seen at the uveitis referral clinic between January 1990 and July 2001; 42 eyes (31.8%) had cataract. Cataract surgery was performed in 17 sympathizing eyes and 1 exciting eye (17 patients). The records of these 18 eyes were retrospectively analyzed. Three eyes had extracapsular cataract extraction (ECCE) with intraocular lens (IOL) implantation, 6 eyes had ECCE without IOL implantation, and 9 eyes had phacoemulsification with IOL implantation. The mean follow-up was 28.7 months (range 3 to 60 months). RESULTS: The causes of sympathetic ophthalmia were penetrating trauma (n = 8 eyes), ocular surgery (n = 6), perforated corneal ulcer (n = 2), and cyclocryotherapy (n = 1). The most common cataract type, present in 7 eyes (38.8%), was mixed (posterior subcapsular and posterior polar). Visual acuity improved after surgery in 13 eyes (72.2%). The main factors impairing visual recovery were submacular scar and optic atrophy, which were sequelae of the sympathetic ophthalmia. Posterior capsule opacification was noted in 14 eyes (77.7%); it was visually significant in 6 eyes. There was no significant difference in postoperative inflammation or disease reactivation between the 3 types of surgery. CONCLUSIONS: Cataract extraction in cases of sympathetic ophthalmia can be safely and successfully performed with vigilant preoperative and postoperative control of inflammation, careful surgical planning, and meticulous surgical technique. The final visual outcome, however, depends on the posterior segment complications of the disease.

Peripapillary choroidal atrophy in sympathetic ophthalmia and management with triple-agent immunosuppression.

PURPOSE: To describe the occurrence of peripapillary choroidal atrophy and experience with triple-agent immunosuppression in three cases of sympathetic ophthalmia. MATERIALS AND METHODS: Retrospective chart review of three cases of sympathetic ophthalmia with peripapillary choroidal atrophy. RESULTS: Three patients with sympathetic ophthalmia were managed with steroid pulse therapy and triple-agent immunosuppression. All three patients subsequently developed peripapillary choroidal atrophy. CONCLUSIONS: Peripapillary choroidal atrophy can occur in sympathetic ophthalmia and may indicate a severe form of inflammation which would benefit from triple-agent immunosuppression.

Sympathetic ophthalmia associated with ocular and cerebral vasculitis: an angiographic and radiologic study.

PURPOSE: To describe a case of sympathetic ophthalmia (SO) associated with ocular and cerebral vasculitis. METHODS: The authors report a 38-year-old woman who presented with bilateral uveitis 7 years after a penetrating trauma to the right eye. Ocular examination included fundus fluorescein and indocyanine green angiography. Medical history disclosed an episode of dysarthria and right-sided weakness. RESULTS: Anterior uveitis was present in the previously injured pseudophakic right eye, which harbored a piece of glass stuck in the lower temporal iris. In the left eye, vitreal inflammation with retinal sheathing and subretinal lesions was predominant. Besides retinal vasculitis, fundus angiography showed choroidal vasculitis. Neurologic studies were compatible with cerebral vasculitis. CONCLUSIONS: In SO, choroidal vasculitis that is invisible clinically can be demonstrated angiographically. Although rare, inflammation can also affect the nervous system.

[Sympathetic ophthalmitis associated with cystic epithelial invasion of the anterior chamber. A clinical case]. / Ophtalmie sympathique associée à une invasion épithéliale kystique de la chambre antérieure. Considérations sur un cas clinique.

A 9-year-old girl presented with sympathetic ophthalmitis associated with secondary glaucoma from cystic epithelial invasion of the anterior chamber. Treatment with immunosuppressants (Imuran) and corticoid therapy for eight months cured the uveitis, and surgical excision of the epithelial cyst was then undertaken. The problems related to therapy of penetrating wounds of the ocular globe are discussed.