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A case series of ocular disease as the primary manifestation in sarcoidosis.

Sarcoidosis is an idiopathic, systemic, non-caseating, granulomatous disease with protean clinical manifestations. This disease is highly prevalent in the southern states of United States of America, especially among African-Americans, but uncommon among Asians. Though sarcoidosis concerns physicians of virtually all specialties, it is particularly important to ophthalmologists since a significant number of patients will seek initial medical examination because of ocular disease. Out of the 262 cases of uveitis examined at the Singapore National Eye Centre over a period of two years, 9 cases were diagnosed as sarcoidosis. We report here 4 patients with active sarcoidosis who presented to us with ocular symptoms as the first and primary manifestation of the disease. All 4 patients developed symptoms and signs of systemic sarcoidosis at a later date. The ocular manifestations included simultaneous bilateral chronic granulomatous iridocyclitis, intermediate uveitis, posterior uveitis characterised by vasculitis with candle wax drippings, and optic nerve head granuloma.

Adult↗

Clinical features of human T-lymphotropic virus type 1 uveitis: a long-term follow-up.

To investigate the clinical manifestations of human T-lymphotropic virus type-1 uveitis (HU), 112 HU patients who were followed up periodically for more than one year were retrospectively analyzed with respect to their ophthalmological and systemic complications. The gender ratio (female/male ratio) of the HU patients was 2.0 and the initial complications were foggy vision in 34.5%, ocular floaters in 33.3%, and blurred vision in 15.5%. As for the ocular symptoms, the majority (78.6%) of patients were classified as intermediate uveitis with vitreous inflammation. Recurrence of uveitis episodes was seen in one half of the patients (51.8%); 12 patients had more than six uveitis episodes. The interval of uveitis episodes varied from two weeks to 10 years. Nearly one half of the patients (43.8%) had ocular complications: e.g., cataract in 22 patients, persistent vitreous opacities in 17 patients, and glaucoma in 16 patients. Although the visual prognosis was essentially good, 11 patients had poor visual prognosis (<0.1). The causes of poor vision in these patients were cataract, cystoid macular edema, epiretinal membrane, and optic nerve atrophy. Of the 112 HU patients, two developed HTLV-I-associated myelopathy (TSP/HAM) after the onset of HU, while none developed adult T-cell leukemia. Sixteen HU patients had a previous history of Graves' disease and a past history of methimazole therapy, while Graves' disease was found in another HU patient only after HU onset and methimazole was not administered before the onset of HU. The present data of long-term follow-up indicate that (1) HU causes various ocular complications and its visual prognosis can be poor, (2) TSP/HAM can be induced even after the onset of HU, and (3) methimazole is not a risk factor of HU after Graves' disease.

Adult↗

[Uveitis complicating posterior segment neovascularization. Six cases].

Subretinal or preretinal neovascularisation may be occasionally observed during the evolution of certain forms of uveitis. Subretinal neovascularisation commonly begins at the level of the chorioretinal uveitic scars. Consequently, the alteration of the retinal pigment epithelium-Bruch's membrane-choriocapillaris complex is the apparent cause. Laser photocoagulation of the neovascular front, when indicated, is the treatment of choice. In contrast, preretinal neovascularisation is observed at the active stage of uveitis. Fluorescein angiography findings suggest that it is probably correlated with the importance of inflammation since in most of the cases there is no retinal ischemia. Therefore, in order to obtain a regression of the neovascular tufts the treatment should be directed against the inflammatory process. In this study the authors report five cases of subretinal neovascularisation which developed at the level of chorioretinal post-inflammatory scars and one case of papillary neovascularisation which occurred during the active stage of an unilateral intermediate uveitis and in which new vessels regressed completely following systemic steroid treatment.

Adolescent↗

[Clinical manifestation and diagnosis of ocular borreliosis].

PURPOSE: To present severe involvements of Borrelia burgdorferi in the etiology of uveitis, optic neuritis and other ocular inflammatory conditions. METHODS: IgM and IgG antibodies for Borrelia burgdorferi were detected by ELISA. Since May 1995 we have examined 78 patients for borreliosis. RESULTS: Borreliosis was diagnosed in 11 patients (4 males, 7 females; aged 7 to 48). 7 persons remembered being bitten by a tick, 3 months to 3 years before the onset of ocular symptoms. All patients had systemic symptoms and signs. Ocular manifestations were as follows: nonspecific chronic conjunctivitis in 4 patients, keratitis in 2, diminished corneal sensation in 1, iritis in 1, intermediate uveitis and perivasculitis in 1, posterior uveitis in 3, retinal haemorrhage in 1, optic neuritis in 1, optic disc oedema in 2, sixth nerve paresis in 1 patient. CONCLUSIONS: We emphasize the importance of performing serological tests for borreliosis in patients with uveitis, inflammatory diseases of unknown etiology, optic neuritis and other neuro-ophthalmic conditions.

Adolescent↗

Uveitis in children and adolescents.

AIMS: To study the relative occurrence of uveitis (intraocular inflammation) and its causes in children and adolescents. METHODS: Patients with uveitis examined and followed during a period of 10 years were categorised by age and sex. All underwent ocular examination and an individually tailored battery of laboratory tests. The intraocular manifestations were classified according to the anatomical location of the inflammation and their most probable cause. The final diagnosis was based on typical clinical ocular and extraocular symptoms and signs and on the results of specific laboratory investigations. RESULTS: Out of 821 patients, 276 (33.1%) were 18 years of age or younger with a male to female ratio of 1 to 1. In these 276 children and adolescents, 70.3% had bilateral ocular involvement. Intermediate uveitis was the most frequent anatomical diagnosis. In many cases, symptoms were mild despite the prominent signs and marked decrease of vision. The underlying cause for the uveitis was evaluated as non-infectious in 184 cases (66.7%) and infectious in 92 cases (33.3%). A potential aetiology and/or a definite clinical diagnosis were established in 74.6% of the cases and only 25.4% of the 276 patients were classified as idiopathic. Juvenile idiopathic arthritis (JIA) was the most common systemic disease association diagnosed in 14.9% of these children. Parasite infestation was the most common infectious association. CONCLUSIONS: Uveitis in children and adolescents is not as low as previously reported. Parasite infestation on the one hand and JIA on the other hand are the most common aetiologies associated with the uveitis in these young patients.

Adolescent↗

Ultrasound biomicroscopy in peripheral retinovitreal toxocariasis.

PURPOSE: To report ultrasound biomicroscopic features in peripheral retinovitreal Toxocara uveitis. METHODS: Three patients with unilateral vitreitis suspected to be caused by peripheral toxocariasis were referred for ultrasound biomicroscopic examination of the peripheral retina, pars plana, and adjacent vitreous. RESULTS: In two of the three patients, characteristic pseudocystic transformation of the peripheral vitreous was seen. In both patients, Toxocara serology was positive and eosinophilia was present. In the third patient, no pseudocystic transformation of the peripheral vitreous was seen; however, there was the dense thickening adjacent to the pars plana usually seen by ultrasound biomicroscopy in pars planitis and corresponding to clinically visible snowbanks. The third patient had a negative Toxocara serology and later developed bilateral intermediate uveitis of the pars planitis type. In none of 48 patients with diverse inflammatory conditions of the retroiridal space that were examined by ultrasound biomicroscopy were the characteristic Toxocara-associated pseudocystic images seen. CONCLUSIONS: Pseudocystic transformation of the peripheral vitreous appears to be a rather specific and sensitive ultrasound biomicroscopic sign in patients with presumed peripheral toxocariasis that will likely aid diagnosis in difficult cases.

Adult↗

Fuchs' heterochromic uveitis and sarcoidosis.

AIMS/BACKGROUND: The aetiology of Fuchs' heterochromic uveitis (FHU) is unknown although it can occur in combination with a number of different ocular conditions. Five patients with FHU who show an association with sarcoidosis were studied. METHODS: Four patients with clinical signs compatible with FHU who had elevated serum angiotensin converting enzyme levels (sACE), and a fifth case with a normal sACE and a positive Kveim test were described. RESULTS: All five cases had iris nodules, two later developed mutton fat keratic precipitates, and one had peripheral retinal periphlebitis. Of the four cases with elevated sACE, one had respiratory function test abnormalities and an abnormal chest x ray compatible with pulmonary sarcoidosis. Another had a chorioretinal scar and developed intermediate uveitis 2 years after presentation. CONCLUSIONS: In all of these cases a diagnosis of FHU may represent a specific secondary ocular response to sarcoidosis rather than a primary idiopathic uveitis syndrome. Although FHU remains a clinical diagnosis, routine uveitis investigations should still be performed in this group of patients.

Adolescent↗

Fluocinolone acetonide sustained drug delivery device to treat severe uveitis.

PURPOSE: Uveitis is often a chronic disease requiring long-term medical therapy. In this report, we describe a pilot safety and efficacy trial of a novel sustained drug delivery system containing fluocinolone acetonide to treat patients with severe uveitis. DESIGN: Prospective, noncomparative, interventional case series PARTICIPANTS: Patients with severe uveitis. METHODS: Sustained drug delivery devices designed to release fluocinolone acetonide for at least 2.5 years were implanted through the pars plana into the vitreous cavity of seven eyes of five patients. All patients had severe uveitis not well controlled with, or intolerant to, repeated periocular corticosteroid injections, systemic corticosteroids, nonsteroidal immunosuppressive agents, or a combination thereof at the time of device implantation. Before device implantation, patients underwent complete evaluation including history, ophthalmologic examination, fluorescein angiography, visual field testing, and electroretinography. After surgery, patients were reexamined at 1 week, 2 weeks, 4 weeks, and at 1- to 3-month intervals. Visual fields, electroretinograms, and fluorescein angiography were repeated at 3- to 6-month intervals. MAIN OUTCOME MEASURES: Preoperative and postoperative visual acuity, ocular inflammation, anti-inflammatory medication use, and intraocular pressure. RESULTS: Patients had a diagnosis of Behçet's syndrome (two eyes), or idiopathic panuveitis (five eyes, including two with necrotizing retinitis, two with progressive chorioretinitis, and one with iridocyclitis and intermediate uveitis). Patients were observed an average of 10 months (range, 5-19 months). All eyes had stabilized or improved visual acuity after device implantation, and four of seven eyes had an improvement of three lines or more. The mean initial visual acuity, measured by Snellen chart, was 20/207, and the mean final visual acuity was 20/57 (P = 0.02). After surgery, at the final visit, no eye had clinically detectable inflammation, and all seven eyes had a marked reduction in systemic, topical, and periocular anti-inflammatory medication use. Four eyes had increased intraocular pressure 6 weeks to 6 months after device implantation. Intraocular pressure has been controlled on topical medications. No patient experienced intraoperative complications. CONCLUSIONS: A fluocinolone acetonide sustained drug delivery device is a promising new therapy for the treatment of severe uveitis. Intraocular pressure must be carefully monitored long after device implantation. Based on these data, a randomized study of a larger group of patients is warranted.

Adolescent↗

[Nonspecific eye autoantibodies in uveitis].

Under certain pathologic circumstances autoantigens lead to the formation of autoantibodies. In uveitis, autoimmunologic phenomena will also be discussed. We studied 149 sera of uveitis patients for various organ specific and non-organ specific autoantibodies. We found antisarcolemal autoantibodies (ASA) mainly in patients with acute iritis (59% positive) but also in panuveitis (40%) and iridocyclitis (29%). On the other hand antiendothelial antibodies (AEA) could be found in 43% of patients with chorioretinitis. Antisinusoidal antibodies (SA) have been detected in iritis and panuveitis patients more often than in the control group which show positive autoantibodies in 5% of all cases. Using ELISA we looked for antibodies against keratin, laminin and microsomes. Because antimicrosomal-, antilaminin- and antisarcolemal-antibodies recognize the same epitope, there was a good correlation of these three autoantibodies. Similar clusters of autoantibodies (ASA, AEA and SA) have been found in various infectious diseases and in chronic inflammatory diseases in which an infectious component is discussed. These results may indicate that iritis, iridocyclitis, chorioretinitis and panuveitis are secondary reactions of eye tissue following a systemic primary disease. Especially viruses are well-known for their production of autoantibodies. In patients with intermediate uveitis we could not demonstrate these autoantibodies more often than in the control group, favouring theories which believe in an autoimmune reaction against vitreous elements.

Antibodies, Antinuclear↗

Vasoproliferative tumors of the ocular fundus. Classification and clinical manifestations in 103 patients.

OBJECTIVE: To describe the clinical features of vasoproliferative tumors of the ocular fundus and to propose a comprehensive classification of these tumors. METHODS: A retrospective review of all cases that were diagnosed as acquired retinal hemangioma or vasoproliferative retinal tumor was conducted on the Ocular Oncology Service at Wills Eye Hospital, Philadelphia, Pa. RESULTS: There were 129 vasoproliferative tumors in 113 eyes of 103 patients. The tumors were classified as idiopathic in 84 eyes (74%) and secondary to preexisting ocular disease in 29 (26%). Subclassification into solitary (88 eyes), multiple (17 eyes), and diffuse (eight eyes) involvement was made. Of the 84 eyes with idiopathic tumors, 73 (87%) had solitary tumors, five (6%) had multiple tumors, and six (7%) had diffuse tumors. The lesion was located in the inferior, inferotemporal, or temporal region of the fundus in 78% and developed within 6 mm of the ora serrata retinae in 88%. Associated vitreoretinal findings included intraretinal exudation (82%), secondary exudative retinal detachment (48%), vitreous cells (46%), vitreous hemorrhage (21%), preretinal macular fibrosis (31%), and macular edema (18%). Of the 29 eyes with secondary tumors, the tumor was solitary in 15 (52%), multiple in 12 (41%), and diffuse in two (7%). The most common preexisting ocular disease included intermediate uveitis (pars planitis) in eight eyes (28%), retinitis pigmentosa in six (21%), toxoplasmic retinitis in two (7%), toxocariasis in two (7%), retinochoroidal coloboma in two (7%), and traumatic chorioretinopathy in two (7%). Retinal pigment epithelial hyperplasia was a prominent feature that was adjacent to 58% of the secondary tumors. Overall, management of the 129 tumors consisted of observation in 63 (49%), cryotherapy in 54 (42%), laser photocoagulation in seven (5%), plaque radiotherapy in three (2%), and other modes of treatment in two (2%). CONCLUSIONS: Vasoproliferative retinal tumors can be idiopathic, or they can develop secondary to congenital, inflammatory, vascular, traumatic, dystrophic, and degenerative ocular diseases. They can produce a variety of complications. Awareness and recognition of these tumors and differentiation from other retinal vascular tumors are important.

Adolescent↗

Amsler's sign associated significantly with Fuch's heterochromic cyclitis (FHC).

To investigate the frequency and diagnostic significance of Amsler's sign in patients with Fuch's heterochromic cyclitis (FHC), we performed a diagnostic AC puncture of the eyes of 31 patients with this disease and looked for the same sign in 104 patients with intermediate uveitis and 373 patients with various other types of uveitis who underwent the same procedure. AS was significantly more frequent in the FHC patients than in either of the other two groups of patients, and occurred infrequently in all other types of chronic uveitis. We therefore consider its occurrence to have important differential diagnostic value. We would also emphasize the pathophysiologic significance of AS and suggest that this aspect be investigated in the future.

Adolescent↗

Ocular toxocariasis: a presumed case of peripheral granuloma.

We report a case of peripheral Toxocara granuloma in a young boy of 17. Of the 3 forms of ocular toxocariasis, macular granuloma, endophthalmitis and peripheral granuloma, the latter is the least common and is characterized by the retention of relatively good vision. A differential diagnosis was performed between an intraocular foreign body encapsulated in fibroglial tissue, toxoplasmic retinochoroiditis, retinoblastoma, intermediate uveitis and toxocariasis. Fluorescein angiography showed not only some neovascularizations at the level of the peripheral granuloma but also manifestations of inflammatory reactions at the level of the posterior pole, i.e. diffuse leakages from retinal capillaries and parietal staining of some venous segments. Among the various laboratory tests, the most significant are eosinophilia, IgE concentration and detection of specific antibodies in serum as well as in aqueous humour.

Adolescent↗

Fuchs heterochromic cyclitis and ocular toxocariasis.

PURPOSE: To report the association of Fuchs heterochromic cyclitis (FHC) and ocular toxocariasis in a young adult. DESIGN: Observational case report. METHODS: A 26-year-old patient was referred for the management of a unilateral intermediate uveitis associated with a lower peripheral subretinal fibrotic lesion near the pars plana. Diagnosis of FHC was clinically confirmed. Laboratory examination was performed to exclude an infectious condition. RESULTS: LISA assay detected significant levels of IgG directed against Toxocara canis. Toxoplasmic serology was negative, excluding this differential diagnosis. Other examinations, including complete blood cell count, urinalysis, serum angiotensin-converting enzyme, lysosyme, chest CT scan, and syphilis serology were noncontributive. CONCLUSIONS: Previous studies have reported on the association of FHC and other parasitic conditions, such as toxoplasmosis but also on herpetic ocular infections. Serologic analysis for toxocariasis may be proposed in patients with FHC and retinal scars in the absence of toxoplasmosis.

Adult↗

Floaterectomy: combined phacoemulsification and deep anterior vitrectomy.

We describe a 1-stage surgical technique to treat vitreous floaters. Phacoemulsification is combined with a deep anterior vitrectomy through a posterior curvilinear capsulorhexis followed by implantation of a posterior chamber intraocular lens. The technique was used in 10 eyes of 6 patients. Six months postoperatively, 8 eyes had a best corrected visual acuity (BCVA) of 6/6 with complete resolution of symptoms. Two eyes of 1 patient developed cystoid macular edema that reduced final BCVA to 6/18 bilaterally. In this case, the floaters may have been the result of previously undiagnosed intermediate uveitis.

Aged↗

Medical treatment of macular edema in patients with uveitis.

PURPOSE: To determine the efficacy of medical treatment of cystoid macular edema (CME) in patients with uveitis. METHODS: Retrospective study of 40 patients (57 eyes) with uveitis and CME. Inclusion criteria were presence of CME with minimal and no macular pathology, or vascular disease which could account for CME. Patients who had undergone intraocular surgery or had visual acuity (VA) of > or = 20/40 were excluded. The diagnosis of CME was based on clinical and/or angiographic findings. Three treatment groups were defined: (1) transseptal injection of steroids (n=13 eyes); (2) systemic non steroidal anti-inflammatory drugs (NSAIDs) (n=11 eyes); both 1 and 2 (n=33 eyes). RESULTS: Overall, 79% of eyes improved 3 or more lines of Snellen VA after treatment: 51% improved 4 or more lines. The average number of lines improved was 3.8 for eyes treated with transseptal injections of steroids, 2.9 for eyes treated with NSAIDs, and 4 for eyes treated with both. For all 3 treatment groups between 60-70% of eyes improving 2 or more lines reached best VA only after a minimum of 6 months of follow up. CONCLUSIONS: CME, a vision threatening complication of uveitis, respond fairly well to medical treatment; however, the best VA is achieved after several months. The improvement in VA did not differ markedly among the three treatment groups.

Adolescent↗

Clinical applications of multifocal electroretinography.

The multifocal ERG using the m-sequence stimulation technique allows the derivation of 61 - 241 local ERG signals in a central visual field of about 60 degree diameter in a short time between 4 and 16 min. A recording in a light adapted state offers local information comparable to cone responses in the full-field ERG. Retinal functional losses due to regional disorders in outer retinal layers can be described in detail with this technique. In maculopathies decreased or absent central ERGs are found surrounded by normal ERG. The extent of the central lesion can be estimated. In diseases of the outer retina the pattern of distribution of multifocal ERG activity is similar to the the pattern of the visual field defect. In addition to decreased ERG amplitudes a delay of implicit time may be an important sign of pathology, i.e. the pronounced delay of implicit times in the periphery in retinitis pigmentosa and the implicit time delays in regions associated with retinal edema like CRVO and cystoid macula edema in intermediate uveitis. No simple correlation of the first order kernel multifocal ERG and field defects could be found in disorders of the ganglion cell layer. The multifocal ERG is therefore useful in the differential diagnosis of retinal and optic nerve diseases.

Diabetic Retinopathy↗

Mycophenolate mofetil as an immunosuppressive agent in refractory inflammatory eye disease.

PURPOSE: The aim of this study was to assess the role of mycophenolate mofetil (MMF) in refractory inflammatory eye disease. METHODS: Retrospective, noncomparative, interventional case series of all patients commenced on MMF between 1999 and 2005 for refractory inflammatory eye disease at St Paul's Eye Unit (Liverpool, UK). Main outcome measures noted were control of inflammation, steroid-sparing effect, and adverse effects of MMF therapy. RESULTS: Ten (10) patients (2 with sarcoid, 2 with intermediate uveitis, 1 with Vogt-Koyanagi Harada (VKH) syndrome, 1 with ankylosing spondylitis, 1 with juvenile chronic arthritis (JCA), and 3 with scleritis) who were unresponsive or intolerant to previous therapy and/or as a steroid-sparing agent, received 2-3 g of MMF per day for a mean period of 40.5 months (range, 3-67). Nine (9) patients had a favorable response, with diarrhea and insomnia being the main side-effects. MMF had to be withdrawn in 1 patient because of side-effects and in another because of active arthropathy (with stable uveitis). Average number of relapses was reduced from 3.1 per patient per year to 0.8 per patient per year (P < 0.005). A steroid-sparing effect was achieved in all patients. Visual acuity improved in 8 patients. CONCLUSIONS: MMF appears to be a safe and effective second- or third-line adjunct/alternative immunosuppressant in these difficult cases and works well in combination with cyclosporin A, tacrolimus, and antitumor necrosis factor (TNF) agents. It has potential as a firstor second-line agent and can be considered at a dose of 3 g/day in refractory cases.

Adult↗