British Journal of Medicine & Medical Research 15(5): 1-5, 2016, Article no.BJMMR.26226 ISSN: 2231-0614, NLM ID: 101570965 SCIENCEDOMAIN international www.sciencedomain.org Sympathetic Ophthalmia as a Major Sight- threatening Disorder Mohammed S. Alkhaibari 1* 1Ministry of Health, Tabuk, Saudi Arabia. Author’s contribution The sole author designed, analyzed, interpreted and prepared the manuscript. Article Information DOI: 10.9734/BJMMR/2016/26226 Editor(s): (1) Barbara Giambene, Eye Clinic, Department of Translational Surgery and Medicine, University of Firenze, Italy. Reviewers: (1) Sadiatqudus Dupe, Federal Medical Centre Birnin Kebbi, Nigeria. (2) Seydi Okumus, Gaziantep University, Turkey. Complete Peer review History: http://sciencedomain.org/review-history/14371 Received 6th April 2016 nd Short Communication Accepted 22 April 2016 Published 28 th April 2016 ABSTRACT SO 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. The inflammation is caused by a cell-mediated immune mechanism and autoimmune inflammatory response directed against ocular self-antigens released after the initial injury. SO may occur after ocular trauma (47 to 65% of patients) or contusions. Wounds involving the ciliary body are associated with the highest risk. Surgical interventions may also trigger SO, with posterior segment surgery carrying a higher risk than anterior segment surgery. Diagnosis of SO is mainly based on patient history and clinical presentation. Imaging studies (fluorescein or indocyanine green angiography, B-scan ultrasonography and optical coherence tomography) may be useful to confirm the diagnosis. _____________________________________________________________________________________________________ *Corresponding author: E-mail: [email protected]; Alkhaibari; BJMMR, 15(5): 1-5, 2016; Article no.BJMMR.26226 Keywords: Sympathetic ophthalmia; SO; Vogt–Koyanagi–Harada syndrome; VKH; ophthalmic injuries; ocular trauma. 1. INTRODUCTION red and painful eyes with a swollen middle layer of the eye [4]. 1.1 Definition It has long been thought that uveal pigment is somehow released by trauma and incites an Sympathetic ophthalmia is defined as a rare and inflammatory reaction [5]: Specific bilateral granulomatous pan-uveitis that occurs after the uvea of one eye (inflammation of Neurogenic (extension from one eye to the the uveal tract due to chronic irritation [1] is other through the optic nerves or via the subjected to a penetrating injury due to either ciliary nerves) accidental trauma or surgery, that develops in Infectious theory: tuberculosis, the normal fellow eye [2]. It's bears remarkable Actinomyces, Rickettsia, virus parallels to VKH (Vogt– Koyanagi–Harada Combined ciliary nerve and bacterial Syndrome) but differs in being causally related to theories antecedent ocular trauma or surgery. Although Allergic or Anaphylactic theory: this response to injury can occur within a few Autoimmunity against uveal melanin, uveal days or over 60 years later, it usually arises melanocytes, retinal pigment epithelium, or between 1 and 12 months after injury [3]. retinal antigens (S & IRBP) Role of Ocular Immune Privilege The injured eye is termed the “exciting” eye and the non-injured eye is referred to the 1.5 Clinical Feature “sympathizing” eye [4]. 1.5.1 Ophthalmic [3] The term “sympathetic ophthalmia” was coined by Mackenzie in the first half of the 19 th century The earliest symptoms include limited range of [5]. accommodation and photophobia. Later there is diminished visual acuity and pain [ 1]. Bilateral 1.2 Prevalence eye pain, photophobia, decreased vision (near vision is often affected before distance vision), Difficult to measure because it has always been red eye. A history of penetrating trauma or a relatively rare disease; as a result of intraocular surgery (most commonly vitreoretinal improvements in modern surgical and medical surgery) to one eye (usually 4 to 8 weeks before, treatment, it has become even more uncommon but the range is from 5 days to 66 years, with [5]. 90% occurring within 1 year) may be elicited [6]. 1.3 Etiology • Anterior: bilateral granulomatous anterior uveitis with mutton-fat keratic precipitates Sympathetic uveitis can occur in an otherwise (KP), posterior synechiae. Bilateral severe unaffected eye even years after penetrating anterior chamber reaction with large injuries or intraocular surgery in the fellow eye, mutton-fat KP; may have asymmetric especially where there was chronic irritation. involvement with typically more reaction in Tissues in the injured eye (uveal tract, lens, and sympathetic eye. retina) act as antigens and provoke an • Posterior: vitritis, choroidal infiltration, autoimmune disorder in the unaffected eye [1]. Dalen–Fuchs nodules, macular edema, exudative retinal detachment; the inciting 1.4 Pathogenesis eye may be phthisical. Posterior segment findings include small depigmented Approximately two-thirds of SO cases occur nodules at the level of the retinal pigment within the period of two weeks to two months epithelium (Dalen–Fuchs nodules), and following injury, with 90% occurring within the thickening of the uveal tract. first year. Usually patients notice blurry vision • Complications: cataract, secondary and pain in both eyes without other symptoms glaucoma, end-stage disease (optic outside the eyes. Eye examination usually shows atrophy, chorioretinal scarring). 2 Alkhaibari; BJMMR, 15(5): 1-5, 2016; Article no.BJMMR.26226 1.5.2 Systemic [3] uveitis with mutton fat keratic precipitates along with variable inflammation of the choroid Features are the same as for VKH, but systemic manifesting as vitritis, optic nerve swelling involvement is less common. (papillitis), and choroiditis [8]. Patients classically have exudative retinal detachments that are 1.6 Sign visible on dilated fundus exam and can be captured on optical coherence tomography. Critical Suspect any inflammation in the These areas of serous retinal detachments uninvolved eye after unilateral ocular trauma. correspond to pinpoint hyperfluorescence and Signs of previous injury or surgery in one eye are leakage on fluorescein angiogram [9]. usually present. 1.8.1 Work-up [6] Nodular infiltration of the iris, peripheral anterior synechiae, neovascularization of the iris, 1. History: Any prior eye surgery or injury, occlusion and seclusion of the pupil, cataract, Venereal disease, Difficulty breathing. exudative retinal detachment, papillitis. 2. Complete ophthalmic examination, including a dilated retinal examination. The earliest sign may be loss of accommodation 3. CBC, RPR, FTA-ABS, and ACE level. or a mild anterior or posterior uveitis in the 4. Consider a chest radiograph to evaluate uninjured eye [6]. for tuberculosis or sarcoidosis. 5. IVFA or B-scan US, or both, to help 1.7 Complications confirm the diagnosis. The disorder has a chronic clinical course and 1.8.2 Pathology [4] may involve severe complications of uveitis such as secondary glaucoma, secondary cataract, The inflammatory changes in the exciting and retinal detachment, and shrinkage of the eyeball. sympathizing eyes are the same, except for Sympathetic ophthalmia can lead to blindness in features of trauma in the exciting eye. particularly severe cases. 1.8.3 Granulomatous uveitis When the injured eye is blind, prophylactic The minimal and classic changes for the enucleation is indicated before the onset of histopathologic diagnosis of sympathetic sympathetic ophthalmia in the fellow eye. An ophthalmia are diffuse lymphocytic infiltration of early sign of sympathetic ophthalmia is a limited the uveal tract with epithelioid cell nests, pigment range of accommodation with photophobia [1]. phagocytosis by the epithelioid cells, absence of necrosis, and sparing of the retina and 1.8 Diagnosis choriocapillaris by the granulomatous process. There are no tests that can tell an eye doctor The uveal tract is usually diffusely thickened without a doubt that a patient has SO. However, (massively in some) by an infiltration of a history of eye injury or surgery combined with lymphocytes in which various numbers of nests the finding of inflammation in both eyes raises of epithelioid cells displaying pigment SO as a possible diagnosis. The eye doctor will phagocytosis are present. obtain a complete history and perform a careful examination of the eyes, including tests of vision, Atypical Pathologic Features eye pressure, and inflammation in the eye, using special instruments which magnify the dilated – Focal nongranulomatous to diffuse non- eyes under bright light. Special testing such necrotizing granulomatous infiltrate as fluorescein angiograms, indocyanine green – Focal choriocapillary involvement angiography, or ultrasound may be performed. – Chorioretinal adhesions The eye doctor will likely obtain blood tests and a – Retinal detachment chest X-ray to ensure that other diseases which – Optic atrophy