Sympathetic ophthalmia associated with high frequent deafness. (original) (raw)
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SYMPATHETIC OPHTHALMIA: WHAT HAVE WE LEARNED
Sympathetic ophthalmia (SO) is a rare bilateral granulomatous uveitis following ocular trauma or surgery to one eye. The eye sustaining the injury is referred to as the inciting eye and the fellow eye is called the sympathizing eye. The etiology of SO has not been completely understood but the underlying pathophysiology is believed to be an autoimmune reaction against the exposed ocular antigens from the inciting eye. The time to development of SO varies from 2 weeks to 50 years with approximately 90% of patients developing the disease within 1 year of injury (1,2,3,4). Since MacKenzie and Fuchs' clinical description of the disease about a century ago enucleation of the inciting eye in an attempt to influence the course of the disease in the sympathizing eye has been a controversial issue. Most cases are diagnosed based on history and presentation, and some are confirmed by the histology (3). SO represents approximately 1-2% of all uveitis cases; the true incidence and prevalence of SO has been hard to establish however a recent prospective study in England and Ireland by Kilmartin et al estimated that SO affects 0.03/100,000 persons per year. Although earlier studies found traumatic SO to be more common Kilmartin et al demonstrated ocular surgery as the most common cause .
An Atypical Case of Sympathetic Ophthalmia After Limbal Corneal Laceration
RETINAL Cases & Brief Reports, 2017
Background/Purpose: To report a case of atypical sympathetic ophthalmia after limbal corneal laceration. Methods and Results: An 11-year-old child had a successful left eye corneal laceration repair at the temporal limbus with excision of exposed nonnecrotic iris tissue, resulting in good visual acuity of 20/80 and 20/25 on postoperative Days 1 and 7, respectively. The patient was prescribed 1 mg/kg oral prednisolone in a tapering dose as prophylaxis. On postoperative Day 21, the patient presented with acute onset decreased vision in both eyes. Visual acuity was counting fingers 3 feet in both eyes. On examination, anterior segment examination was quiet without any inflammation, anterior vitreous face showed 1+ cells, and dilated funduscopy revealed bilateral symmetrical serous retinal detachments along the posterior pole. Optical coherence tomography demonstrated separation and elevation of inner neurosensory layers from the outer segment marking presence of hyperreflective material along with subretinal fluid between detached surfaces. There was stippled hyperfluorescence along the posterior pole as seen in fluorescein angiography. With a diagnosis of sympathetic ophthalmia confirmed, oral prednisolone (2 mg/kg body weight) was instituted after which, there was gradual decrease in macular elevation with corresponding improvement in visual acuity with no recurrence for the last 6 months. Conclusion: To our knowledge, this is the first reported instance of an atypical presentation of sympathetic ophthalmia, and antecedent corticosteroid therapy would have mitigated robust anterior segment findings usually associated with the condition.
Sympathetic Ophthalmia: Incidence of Ocular Complications and Vision Loss in the Sympathizing Eye
American Journal of Ophthalmology, 2009
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. • METHODS: 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 MEA-SURES: 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. (Am J Ophthalmol 2009;148:704 -710.
Sympathetic ophthalmia: to the twenty-first century and beyond
Journal of ophthalmic inflammation and infection, 2013
Sympathetic ophthalmia is a rare bilateral granulomatous inflammation that follows accidental or surgical insult to the uvea of one eye. Onset of sympathetic ophthalmia can be insidious or acute, with recurrent periods of exacerbation. Clinical presentation shows mutton-fat keratic precipitates, choroidal infiltrations, and Dalen-Fuchs nodules. Histopathology reveals diffuse or nodular granulomatous inflammation of the uvea. Prevention and treatment strategies for sympathetic ophthalmia are currently limited to two modalities, enucleation of the injured eye and immunosuppressive therapy, aimed at controlling inflammation. The etiology and pathophysiology of the disease is still unclear but is largely thought to be autoimmune in nature. Recent insight on the molecular pathology of the disease as well as developments in imaging technology have furthered both the understanding on the autoimmune process in sympathetic ophthalmia and the targeting of prevention and treatment strategies f...
SYMPATHETIC OPHTHALMIA: Clinicopathologic Correlation in a Consecutive Case Series
Retina (Philadelphia, Pa.), 2015
To correlate the clinical course of sympathetic ophthalmia with the histological and immunohistochemical characteristics of the enucleated inciting eye. A consecutive case series with baseline clinical features and subsequent histopathologic findings. 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 sc...
Sympathetic Ophthalmia as a Major Sight-threatening Disorder
Sympathetic Ophthalma: Iris and eyeball inflammatory condition affecting both eyes that occurs after a penetrating injury as a delayed autoimmune reaction to eye injury. Patients present with pain, photophobia, paresis of accommodation, metamorphopsia and mild to significant visual loss. The granulomatous anterior uveitis is accompanied by posterior segment findings including moderate to severe vitritis, choroiditis, papillitis, perivasculitis, and yellow-white lesions of the retinal pigment epithelium (Dalen-Fuchs nodules). The inflammation can lead to serious retinal detachment and macular edema. Extraocular symptoms include headache, meningitis or cerebrospinal fluid pleocytosis, hearing loss, poliosis and vitiligo.
Sympathetic Ophthalmia: Where Do We Currently Stand on Treatment Strategies?
Clinical Ophthalmology
Sympathetic ophthalmia is a rare bilateral diffuse granulomatous panuveitis that usually results from surgical or penetrating trauma to one eye. The symptoms range from impaired near vision to pain, photophobia, and loss of visual acuity. Anterior segment manifestations include bilateral acute uveitis with mutton-fat keratic precipitates and posterior segment findings include vitritis, multifocal neurosensory retinal detachment, choroiditis, optic nerve edema, and Dalen-Fuchs nodules. The diagnosis is clinical. Ancillary investigations include fundus fluorescein angiography, indocyanine green angiography, optical coherence tomography (OCT), ultrasound B scan, and autofluorescence imaging. The management options include corticosteroids (topical and systemic) as the first line along with immunomodulatory therapy started at the presentation of the disease. Recent advances include imaging with OCT-angiography, enhanced depth imaging-OCT (EDI-OCT, choroidal vascular index/CVI), targeting IL-23/IL-17 pathway, and use of biologics for the management of this rare entity. Recent advances in early diagnosis and prompt treatment has led to improved final visual outcomes in both the sympathizing and exciting eye. This review is aimed at giving a comprehensive overview of sympathetic ophthalmia along with a special emphasis on current treatment strategies and recent advances.
Analysis of 130 Cases of Sympathetic Ophthalmia - A Retrospective Multicenter Case Series
Ocular immunology and inflammation, 2018
To analyze the demographic profile, treatment, and visual outcome of the patients with sympathetic ophthalmia (SO) in a multicenter collaborative retrospective cohort study. Medical records of the patients with SO from UK, Singapore, India were reviewed for history of ocular trauma or surgery and subsequent development of uveitis consistent with SO, presenting symptoms, treatment, and visual outcomes. A total of 130 patients were diagnosed with SO during the study period. Eighty-one (62.3%) patients were men. The mean age was 48.4 ± 15.5 years. The most common presenting symptom was blurring of vision (89.2%), followed by pain (29.2%) and floaters (23.8%). Ninety-two (70.7%) required additional immunosuppressive therapy. Thirty-six (27.9%) patients underwent enucleation of the inciting eye. SO is a potentially sight-threatening disease with high rates of visual loss. It warrants prompt evaluation and treatment. With the advances and availability in immunotherapy, the visual prognosi...
Retina, 2015
Purpose-To correlate the clinical course of sympathetic ophthalmia (SO) 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 16 had typical histology fulfilling the criteria for SO of diffuse granulomatous inflammation. Among the 16, 11 sustained previous penetrating trauma, 4 underwent previous eye surgery and one 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 time of enucleation. At 6 months after enucleation of the inciting eye, 4 of 7 patients with atypical histology had a visual acuity of ≥ 20/40 compared to 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-Histopathological findings had minimal correlation with the clinical course of SO. Corticosteroid treatment prior to enucleation may influence the pathological confirmation of SO. The predominance of B-lymphocytes and macrophages over T-lymphocytes may represent different stages of the disease process.