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[Vitreous hemorrhage and vitreoschisis].

PURPOSE: To identify the morphologic characteristics of vitreous especially vitreoschisis in vitreous hemorrhage, aiming at discussing the mechanism and clinical importance of vitreoschisis. METHODS: Eighty-eight cases (97 eyes) of vitreous hemorrhage were observed among which 32 cases (38 eyes) resulting from PDR, 28 cases (30 eyes) resulting from retinal vasculitis and 28 cases (29 eyes) resulting from retinal vein occlusion. All cases had B-scan examination, some had color Doppler examination. Images were compared with the finding in vitre-ctomy. RESULTS: Vitreoschisis occurred in 18 cases (47.4%) in PDR group, 10 cases (33%) in retinal vasculitis group and 4 cases (13.8%) in retinal vein occlusion group. CONCLUSIONS: Vitreoschisis may occur in different kind of vitreous hemorrhage. Recognition of vitreoschisis may aid to complete vitrectomy and reduce some complication after surgery.

Adult↗

Iron chelation abrogates excessive formation of hydroxyl radicals and lipid peroxidation products in monocytes of patients with Eales' disease: direct evidence using electron spin resonance spectroscopy.

PURPOSE: Eales' disease (ED) is an idiopathic retinal vasculitis condition, which affects the retina of young adult males. Retinal changes include perivasculitis, non-perfusion and neovascularization. Disruption of blood-retinal barrier (BRB) is the common feature in intra-ocular inflammatory diseases. Disruption of BRB results in vascular hyper permeability and infiltration of circulating leukocytes into the retinal parenchyma. Monocyte (MC) activation results in oxidant thrust and subsequent tissue damage. This has been reported in various intra-ocular inflammatory diseases such as uveitis and Behcet's disease. However, there are no such reports available in ED. Hence in the present study we have investigated the role of MC activation and hydroxyl radicals (OH) production and its possible involvement in promoting the development of retinal vasculitis in patients with ED. METHODS: Twelve patients with ED and twelve healthy volunteers were recruited for the study. MC was separated from their peripheral blood. MC from patients with ED and control subjects was stimulated with phorbol-12-myristate-acetate (PMA) and OH generated was analyzed using an electron spin resonance spectrometer (ESR). Superoxide dismutase (SOD), thiobarbituric acid reactive substances (TBARS), and iron content was determined in MC to assess the oxidant thrust and antioxidant defense. RESULTS: OH generation was elevated in MC from patients with ED, which coincided with diminished SOD activity and elevated levels of iron and TBARS, when compared with healthy control subjects. OH generation was abrogated when MC from ED were co-incubated with PMA and iron chelators such as diethylenetriaminepentacetic acid (DTPA) and desferrioxamine. Iron chelation also inhibited TBARS accumulation restored SOD activity in MC of patients with ED. CONCLUSIONS: For the first time we have demonstrated the production of OH generation in MC of patients with ED using ESR. Further we have shown the beneficial effect of iron chelation in mitigating free radical mediated changes in cellular metabolism. Based on our findings, we provide further evidence for the role of oxidant thrust in promoting retinal tissue damage in patients with ED.

Adult↗

[A case of granulomatous panuveitis associated with multiple sclerosis].

BACKGROUND: Uveitis is not often reported in patients with multiple sclerosis(MS) in Japan, but retinal vasculitis and pars planitis are common findings in uveitis patients with MS in the U.S. and European countries. CASE: A 50-year-old man developed bilateral granulomatous panuveitis 10 years after initial onset of MS. FINDINGS: Examination showed bilateral anterior uveitis with mutton fat keratic precipitate and retinal vasculitis for 360 degrees. The anterior uveitis responded well to topical corticosteroid but the retinal vasculitis did not. The possibility of sarcoidosis, tuberculosis, and other diseases associated with granulomatous uveitis were excluded by systemic examinations. CONCLUSION: We encountered a rare case of granulomatous panuveitis associated with MS.

Granuloma↗

[Adamantiadis-Behçet syndrome: fluorescein angiography and choroid ischemia].

BACKGROUND: Adamantiadis-Behçet's disease is a chronically progressing multisystemic disorder. The underlying disease mechanism is an obliterative vasculitis of unknown etiology. Main clinical symptoms are oral and genital aphthous ulcers and intraocular inflammations. Additionally, cutaneous, rheumatoid, neural, gastrointestinal, or cardiovascular manifestations may be observed. Diagnosis is based on clinical features since currently no specific laboratory tests or pathognomonic histopathological features are available. Ocular changes may provide important diagnostic clues to the systemic disease. PATIENTS AND METHODS: In a retrospective fashion 196 patients of the Department of Ophthalmology of the University of Erlangen-Nürnberg between 1988 and 1998 with retinal vasculitis seen by fluorescence angiography were evaluated according to the diagnostic criteria of the "Behçet's Disease Research Committee of Japan". RESULTS: Among 196 patients there were 12 patients with Adamantiadis-Behçet's disease. Apart from retinal vasculitis, angiographic features included capillary dropout in 64% (9/14), swelling of the optic disc in 79% (11/14) and irregularly delayed areolar filling of choriocapillaris in the early phase of fluorescence angiography in 43% (6/14) of eyes. There was no statistically significant relationship between severity of the systemic disorder and the activity of the ocular disease. CONCLUSION: Apart from retinal vasculitis, 43% of eyes in patients with Adamantiadis-Behçet's disease presented a delayed choroidal filling in fluorescence angiography as a sign of choroidal involvement of occlusive vasculitis. We observed leakage of fluorescein from the optic disc, which could be due to a secondary inflammation of the ciliary circulation. Inflammatory involvement predominantly of choroidal vessels, as visualized in fluorescence angiography, may be a diagnostic lead in Adamantiadis-Behçet's disease.

Adolescent↗

Vanishing retinal arterial aneurysms: a case report.

An 18-year-old woman with retinal vasculitis developed multiple retinal arterial aneurysms over a period of 3 years. Subsequently she developed macular oedema, peripheral neovascularisation, and vitreous haemorrhage and was treated with systemic steroids, laser photocoagulation, and vitreoretinal surgery. No systemic cause for vasculitis was found. Serial fluorescein angiography demonstrated gradual resolution of the arterial aneurysms over the subsequent 7 years.

Adolescent↗

Human recombinant interferon alfa-2a for the treatment of Behçet's disease with sight threatening posterior or panuveitis.

BACKGROUND: Behçet's disease is a multisystem vasculitis of unknown origin. Standard treatment mainly comprises systemic immunosuppressive agents. Ocular involvement, mostly posterior uveitis with retinal vasculitis, leads to blindness in 20-50% of the involved eyes within 5 years. The efficacy of interferon alfa-2a was studied in patients with sight threatening posterior uveitis or retinal vasculitis. METHODS: 50 patients were included in this open, non-randomised, uncontrolled prospective study. Recombinant human interferon alfa-2a (rhIFNalpha-2a) was applied at a dose of 6 million units subcutaneously daily. Dose reduction was performed according to a decision tree until discontinuation. Disease activity was evaluated every 2 weeks by the Behçet's disease activity scoring system and the uveitis scoring system. RESULTS: Response rate of the ocular manifestations was 92% (three non-responder, one incomplete response). Mean visual acuity rose significantly from 0.56 to 0.84 at week 24 (p<0.0001). Posterior uveitis score of the affected eyes fell by 46% every week (p<0.001). Remission of retinal inflammation was achieved by week 24. Mean Behçet's disease activity score fell from 5.8 to 3.3 at week 24 and further to 2.8 at week 52. After a mean observation period of 36.4 months (range 12-72), 20 patients (40%) are off treatment and disease free for 7-58 months (mean 29.5). In the other patients maintenance IFN dosage is three million units three times weekly. CONCLUSIONS: rhIFNalpha-2a is effective in ocular Behçet's disease, leading to significant improvement of vision and complete remission of ocular vasculitis in the majority of the patients.

Adult↗

Whipple's disease complicated by a retinal Jarisch-Herxheimer reaction: a case report.

A 36 year old white man was diagnosed as having Whipple's disease after a prolonged illness of lethargy, night sweats, and weight loss associated with lymphadenopathy and splenomegaly. Biopsy specimen of an inguinal lymph node confirmed the presence of periodic acid Schiff positive macrophages and culture gave a pure growth of Corynebacterium jeikeium. Twelve hours after the introduction of oral co-trimoxazole and streptomycin the patient's condition deteriorated. He became confused, feverish, and developed florid retinal vasculitis with associated visual impairment. Both the systemic symptoms and the retinal vasculitis responded to treatment with corticosteroids and his vision returned to normal. We think this was a Jarisch-Herxheimer reaction not previously described in Whipple's disease and advise inspection of the fundi of such patients before starting treatment.

Adult↗

Electrophysiological studies on Kirisawa type uveitis (acute retinal necrosis).

Kirisawa type uveitis (acute retinal necrosis) is characterized by a necrotizing retinitis, heavy vitreous opacities, retinal vasculitis, and subsequent retinal detachment. Not only the etiology but also the clinical findings of this disease have not yet been defined. In the preceding year we have seen four patients whose clinical course and findings were similar to each other. In two of them a vitrectomy and an encircling operation have been performed before retinal detachment occurred. This treatment has found to be effective. In all 4 cases electrophysiological examinations were done at various stages of the disease. ERGs following pattern and flash stimuli varied in accordance with retinal changes, while VECPs were less strongly affected as ERGs. Results are discussed referring to fluorescein angiographic findings and ocular changes.

Adult↗

In vivo fluorescein staining of SI-30NB silicone intraocular lens.

We report a case of a 48-year-old pseudophakic woman who presented 3 weeks after Heidelberg retinal angiography using intravenous sodium fluorescein 2%. Bilateral retinal vasculitis with severe retinal ischemia and extensive capillary dropouts had been diagnosed. Anterior segment examination revealed green staining on the silicone optic of the 3-piece SI-30NB intraocular lens (IOL) (AMO), with no cell or flare in the aqueous humor. The anterior and posterior capsules were not stained. The patient did not report dark vision, double vision, or altered color vision. The anterior and posterior IOL surfaces demonstrated an autofluorescence at the time of fundus photography, which persisted to the 6-week examination although there were no symptoms. This is suggestive of deposits of sodium fluorescein on the IOL surface following angiography.

Female↗

Ocular manifestations of cat-scratch disease.

Bartonella henselae has only recently been isolated, characterized, and found to be the principal cause of cat-scratch disease (CSD). The availability of specific serologic investigations has allowed the recognition of a spectrum of ocular CSD syndromes that previously were ill defined and considered idiopathic. The primary inoculation complex causing regional lymphadenopathy is represented in the eye by Parinaud's oculoglandular syndrome; B. henselae is the most common cause. Leber's neuroretinitis has been identified for 80 years, and new data suggest that it is commonly a manifestation of CSD; the extent of the association remains to be determined. CSD optic neuritis is also described. The vitreoretinal manifestations include anterior uveitis, vitritis, pars planitis, focal retinal vasculitis, a characteristic retinal white spot syndrome, Bartonella retinitis, branch retinal arteriolar or venular occlusions, focal choroiditis, serous retinal detachments, and peripapillary angiomatous lesions. The pattern of ocular disease in AIDS-associated B. henselae infections is poorly delineated; unusual manifestations include conjunctival and retinal bacillary angiomatosis. The benefit of antimicrobial therapy for CSD in immunocompetent individuals has been difficult to establish, partly because most infections are self limited. Empirically, azithromycin, ciprofloxacin, rifampin, parenteral gentamicin, or trimethoprim-sulfamethoxazole provide the best therapeutic choices to minimize damage to the eye.

Anti-Bacterial Agents↗

Detection of varicella-zoster virus genome having a PstI site in the ocular sample from a patient with acute retinal necrosis.

We detected the virus genome in ocular samples from a 65-year-old woman with clinically diagnosed acute retinal necrosis using DNA amplification. She exhibited occlusive retinal vasculitis, confluent necrotizing retinitis, mainly peripheral, and iridocyclitis. For DNA amplification, we used recently published primers specific for varicella-zoster virus (VZV) and herpes simplex virus. Using VZV primers, we detected the VZV genome in the aqueous humor, but not in the vitreous, by amplifying a DNA fragment 642 base pairs in length. HSV DNA was not detected. After detecting the VZV genome, PstI restriction endonuclease was used because an epidemiological study found that about 25% of the VZV strains in Japan carry a mutation lacking a PstI recognition site. The VZV genome from the patient had a PstI cleavage pattern, while the positive control had a VZV genome that carried a PstI-site-less mutation. We considered our patient with acute retinal necrosis to be infected with VZV having a PstI site.

Aged↗

Ophthalmic complications of dengue.

We report 13 cases of ophthalmic complications resulting from dengue infection in Singapore. We performed a retrospective analysis of a series of 13 patients with dengue fever who had visual impairment. Investigations included Humphrey automated visual field analyzer, Amsler charting, fundus fluorescein angiography, and optical coherence tomography. Twenty-two eyes of 13 patients were affected. The mean age of patients was 31.7 years. Visual acuity varied from 20/25 to counting fingers only. Twelve patients (92.3%) noted central vision impairment. Onset of visual impairment coincided with the nadir of serum thrombocytopenia. Ophthalmologic findings include macular edema and blot hemorrhages (10), cotton wool spots (1), retinal vasculitis (4), exudative retinal detachment (2), and anterior uveitis (1). All patients recovered visual acuity to 20/30 or better with residual central scotoma by 12 weeks. These new complications suggest a widening spectrum of ophthalmic complications in dengue infection.

Adult↗

Presence of a 88 kDa Eales protein in uveitis, tuberculosis, leprosy and rheumatoid arthritis.

BACKGROUND: Eales disease (ED) is an idiopathic retinal vasculitis affecting young adult males. We have earlier reported the identification, purification and partial characterization of a novel 88 kDa protein found in the serum of patients with ED. The aim of the present study was to look for the 88 kDa protein in serum samples obtained from cases of retinal vasculitis mimicking ED and in other systemic inflammatory diseases. MATERIAL/METHODS: Serum samples from healthy volunteers and from patients with ED, uveitis, parsplanitis ocular sarcoidosis, toxoplasmosis, leprosy, diabetic retinopathy, viral hepatitis, and rheumatoid arthritis were analyzed for the presence of the 88 kDa protein by polyacralymide gel electrophoresis (PAGE). The immunological identity of the 88 kDa protein found in ED and in other diseases was investigated by Western blot. Immunohistochemistry was performed on epiretinal membranes (ERM) obtained from ED patients to localize the 88 kDa protein. RESULTS: 88 kDa protein were detected in serum samples obtained from patients with posterior uveitis, tuberculosis, leprosy and rheumatoid arthritis. The 88 kDa protein found in serum from patients with ED is immunologically identical to that found in other systemic inflammatory conditions. 88 kDa protein was localized in inflammatory cells and in nonvascular endothelium in ERMs obtained from patients with ED. CONCLUSIONS: We have identified a novel acute phase reactant, which is elaborated in ocular and systemic inflammatory conditions other than Eales disease. Further work is necessary to decipher the precise role of the 88 kDa protein in the pathophysiology of these inflammatory diseases.

Adult↗

Intraocular inflammatory reactions without focal necrotizing retinochoroiditis in patients with acquired systemic toxoplasmosis.

PURPOSE: To describe the occurrence of intraocular inflammatory reactions as the sole ophthalmic manifestation of acquired systemic toxoplasmosis. METHODS: Review of medical records for 10 patients with uveitis and evidence of recent Toxoplasma gondii infection. RESULTS: Patient ages ranged from 3 to 51 years. Ocular symptoms were present in each of eight adult patients. Inflammation was unilateral in nine patients; it manifested as vitreous humor cells and haze (10 patients), anterior chamber cells (seven patients), and retinal vasculitis (seven patients). No patient had necrotizing retinochoroiditis upon initial examination. Inflammation resolved in each of nine patients who had follow-up examinations. Foci of retinitis or inactive retinochoroidal scars were seen in four of these nine patients during follow-up examinations, at intervals of 2.0 weeks to 2.5 years after initial examination. CONCLUSIONS: Retinal vasculitis and associated inflammatory reactions may be the only ophthalmic disorder during the early stages of a newly acquired T. gondii infection. Later development of retinitis or scars consistent with toxoplasmic retinochoroiditis in the same eyes suggests that the initial, isolated inflammation may be caused by the presence of parasites in retinal tissue. These cases may have implications for understanding the original source of retinal infection in patients who have recurrent toxoplasmic retinochoroiditis and for treatment of newly acquired T gondii infection.

Adolescent↗

Human antiretinal antibodies in toxoplasma retinochoroiditis.

BACKGROUND/AIMS: Toxoplasma retinochoroiditis (TR) is an important cause of blindness and visual morbidity, affecting young adults. It has been postulated that some of the retinal damage observed in TR is due to antiretinal autoimmune mechanisms. METHODS: Humoral antiretinal autoimmunity in TR was investigated by indirect immunofluorescence (IIF) on normal human cadaveric retina and by a human retinal S-antigen ELISA. 36 patients with TR were separated on clinical grounds into those with first recurrence of disease (n = 18) or those with multiple recurrences (n = 18). Patients were also segregated into those with active (n = 28) or quiescent disease (n = 8). Serum from 16 normal controls (six with positive toxoplasma serology and 10 without) with no evidence of eye disease and 12 patients with idiopathic retinal vasculitis (IRV) were also tested. RESULTS: Sera from 34 of the 36 patients (94%) with TR demonstrated photoreceptor layer reactivity by IIF contrasting with six of 16 normal controls (p = < 0.001) and three of 12 IRV patients (p = < 0.001). Titres of antiphotoreceptor antibody were also higher among TR patients than controls. Sera from 27 of the 36 TR patients, 10 of 16 normals, and nine of 12 retinal vasculitis patients possessed anti-human retinal S-antigen antibodies at a titre of 1:400 or more as assessed by ELISA (p = > 0.05). Antiretinal autoantibody as detected by IIF did not run in parallel with S-antigen reactivity. CONCLUSIONS: The data indicate that the extent of antiretinal reactivity within TR is not accounted for by anti-S-antigen antibodies alone. This remarkably high prevalence of antiphotoreceptor antibody in TR as opposed to that found in either healthy or disease controls suggest that these antibodies may be co-pathogenic in toxoplasma retinochoroiditis.

Adult↗

[Viral vasculitis and disseminated white spots].

A case of retinal vasculitis is reported, unusual because of its occurrence in a young healthy man, and especially because of the presence of a turn-over of white spots in the mid-peripheral retina, which faded and turned into areas of pigment epithelium changes. The patient presented with anterior uveitis with raised intraocular pressure, and then with vitritis, retinitis in the peripapillary area with involvement of the optic disc, vasculitis with perivascular sheathing and vascular occlusion. Clinical and laboratory investigations remained negative. The lesions regressed with antiviral treatment (Ganciclovir) and steroids. Final visual acuity was 20/400 due to optic disc atrophy and the development of an epimacular membrane. The authors discuss the differential diagnosis of retinal vasculitis related to immunological diseases or viral infection. In conclusion, this case may constitute a mild type of acute retinal necrosis syndrome.

Fluorescein Angiography↗

Ocular complications of Rift Valley fever outbreak in Saudi Arabia.

OBJECTIVE: To report ocular complications of Rift Valley fever (RVF) during its first reported outbreak in southwest Saudi Arabia in autumn 2000. DESIGN: Cross-sectional study of patients in a referral hospital. PARTICIPANTS: One hundred forty-three consecutive patients with confirmed RVF serologic test results and ocular lesions were enrolled in the study. METHODS: Hospitalized patients (n = 30) and outpatients (n = 113) with clinical symptoms consistent with RVF, positive RVF serologic test results, and ocular abnormalities were studied. Ophthalmologic examinations, including fundus photography and fluorescein angiography, were performed. Patients were followed up at regular intervals to determine the prognosis and outcome of identified ocular abnormalities. MAIN OUTCOME MEASURES: Visual acuity at initial presentation and course of anterior and posterior segment complications. RESULTS: Among 143 patients (78% males; mean age, 53.2 years), 212 eyes were affected, comprising 47 eyes in 30 inpatients and 165 eyes in 113 outpatients. The mean interval between the onset of RVF and visual symptoms ranged from 4 to 15 days (mean, 8.8 days). Macular or paramacular retinitis was identified in all the affected eyes (n = 212) at the time of initial assessment. Lesions included retinal hemorrhages (40%), vitreous reactions (26%), optic disc edema (15%), and retinal vasculitis (7%). Anterior uveitis was present in 31% of outpatients. Fluorescein angiography of the retinitis showed early hypofluorescence with late staining of retinal lesions and blood vessels. Initial visual acuity was less than 20/200 in 80% of eyes in the outpatient group; their vision improved, deteriorated, or remained the same in 13%, 15%, or 72%, respectively. Evaluation at the last follow-up showed macular (60%) or paramacular (9%) scarring, vascular occlusion (23%), and optic atrophy (20%) in the outpatient group. CONCLUSIONS: Rift Valley fever was associated with major ocular morbidity. Ocular manifestations of RVF occurred with a relatively higher frequency than reported up to now and were not limited to severe infections. Rift Valley fever affects the uvea and posterior chorioretinal area and is associated with permanent visual loss resulting from macular and paramacular scarring, vascular occlusion, and optic atrophy. The study demonstrated for the first time that transient nongranulomatous anterior uveitis is associated with RVF.

Adolescent↗