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[The value of electroretinography in uveitis].

The involvement of the retina in the inflammation being one important factor in severe uveitis, the authors have tested the "ganzfeld" electroretinogram (ERG) in various forms of uveitis, especially in cases having opaque media. In Fuchs' syndrome and chronic anterior uveitis, the ERG is normal or only slightly altered, while important reduction of the potentials can occur even in case of good vision in intermediate uveitis, probably indicating concomitant retinal vasculitis. In Behçet's disease, ERG is mostly useful in testing the efficacy of therapy. ERG can be rather deceiving in idiopathic vasculitis, where the potentials are not always as reduced as expected. On the contrary, this test seems to allow differential diagnosis between Harada's disease and acute multifocal placoid pigment epitheliopathy. However, one must bear in mind that concurrent steroid treatment can elevate the amplitude of the potentials, a phenomenon which has to be taken into account when interpreting ERG results.

Behcet Syndrome↗

[Acute retinal necrosis: a report of 19 cases].

PURPOSE: To improve the diagnose and treatment capacity on acute retinal necrosis (ARN). METHODS: Retrospectively analyse the manifestation, examination, diagnose, treatment and prognosis of the 19 cases with ARN in our Ophthalmic Center from January, 1996 to November, 2003. RESULTS: Nineteen cases had inflammation in anterior segment, vitritis, necrotizing retinitis and retinal vasculitis, except one patient. After treatment, visual acuity had been improved in 11 eyes (45.8%). Final visual acuity were lower than 0.05 in 11 eyes. Signs were controlled in 21 eyes(87.5%). CONCLUSIONS: The diagnose of ARN should be mainly based on clinical manifestation. Early and sufficient treatment with antiviral agents, corticosteroid and prophylactic laser photocoagulation, sometimes vitrectomy are the key point to control the disease.

Acyclovir↗

[Ophthalmological involvement in rheumatic disease].

PURPOSE: The main objective of this study was to identify the prevalence of ocular manifestations in rheumatic patients admitted in a specialized clinic. METHODS: Information regarding rheumatic and ocular diseases was extracted from medical records system available in "Dr. I. Cantacuzino" Clinical Hospital from Bucharest. The prevalence of ocular involvement reported passively by rheumatologists (retrospective descriptive study of 375 different cases of rheumatic patients) was compared with the literature data. RESULTS: There were 45 cases of ocular manifestations. Keratoconjunctivitis sicca was noted in 16 patients with rheumatoid arthritis, two patients with systemic lupus erythematosus and one patient with scleroderma. Anterior uveitis was found in seven patients with ankylosing spondylitis, one patient with reactive arthritis, two patients with psoriatic arthritis and one patient with LES. Conjunctivitis was present in two patients with reactive arthritis. In LES ocular involvement also included four cases of retinal vasculitis. Complications clearly related to steroid therapy were nine cases of cataracts. One case with typical "bull's eye" maculopathy due to Hydroxychloroquine treatment was detected. CONCLUSIONS: The main conclusion of our study is that the rheumatic patients need to be referred to an ophthalmologist for the diagnosis and the optimal treatment of ocular involvement.

Aged↗

'Idiopathic' preretinal macular fibrosis in young individuals.

In order to evaluate possible etiological factors and the natural course of 'idiopathic' preretinal fibrosis in young persons, nine consecutive patients with macular pucker diagnosed under the age of 50 years were examined. There were six women and three men with the mean age of 27.2 years (range 15-50) and the mean follow-up of 4.5 years (range 1.5-10). The initial visual acuity varied from 0.25 to 1.0. The preretinal membrane was either central or paramacular. In two eyes the process was probably congenital (retinal hamartoma), one was postinflammatory (retinal vasculitis), and one was associated with sclerosis of the anomalous macular arterioles. In the others no causative factors were discovered. In seven of the nine eyes both the visual acuity and the fibrotic process remained stable, in one eye the vision improved, and in one eye deteriorated. It seems that most young persons with 'idiopathic' preretinal fibrosis of the macula can be followed up without surgical removal of the membrane.

Adolescent↗

Central retinal vein occlusion in young adults.

Central retinal vein occlusion (CRVO) is usually seen in older adults and is often associated with systemic vascular disease. CRVO can be seen in young adults, and although it is occasionally associated with a systemic disease, in the majority of cases it occurs in an otherwise healthy patient with no known systemic disease or ocular problem. Inflammation of the central retinal vein has been proposed as a cause of the occlusion in young adults and for that reason it has been called papillophlebitis. The appearance of unilateral optic disc edema, dilatation, and tortuosity of the major retinal veins with a variable amount of retinal hemorrhage in young, healthy adults with complaints of blurred vision or photopsias has been called, in addition to papillophlebitis, benign retinal vasculitis, optic disc vasculitis, nonischemic CRVO, big blind spot syndrome, and presumed phlebitis of the optic disc. An approach to the diagnostic evaluation of the young adult with CRVO is presented. Although most eyes recover vision to better than 20/40, about one-fifth have significant visual loss, and many suffer ocular sequelae. Many treatment modalities have been tried for this entity, but no conclusive evidence exists that any treatment alters its natural history.

Adolescent↗

Cyclosporin A in the treatment of posterior uveitis.

Cyclosporin A was used in nine patients with severe refractory posterior uveitis. Four patients had Behcet's disease, one had sarcoidosis, one had HLA B27 related arthritis and uveitis and three had idiopathic retinal vasculitis. The drug had beneficial ocular effects in all patients in the early stages of treatment with an improvement in the visual acuity and reduction in the severity of inflammation. The relapse rate and need for additional steroids were reduced in all patients. However, renal function deteriorated in seven patients to such an extent that the dose of Cyclosporin A had to be reduced with consequent relapse of uveitis.

Adult↗

Ocular immunopathology of Behçet's disease.

A patient developed progressive, severe, recurrent bilateral iridocyclitis, retinal vasculitis, and hemorrhagic infarction of the retina that led to blindness despite immunosuppressive therapy. Histopathology of an enucleated blind and painful eye revealed marked nongranulomatous uveitis with a predominantly CD4+ T-lymphocytic infiltration, as well as B-cell and plasma cell aggregation. Extensive expression of adhesion molecules on vascular endothelial cells were found. This finding suggests that adhesion molecules play an important role in the vasculitic process, the trademark of Behçet's disease. The ocular pathology and the therapeutic approach to Behçet's disease are briefly reviewed.

Adult↗

Acute zonal occult outer retinopathy. Donders Lecture: The Netherlands Ophthalmological Society, Maastricht, Holland, June 19, 1992.

PURPOSE: This report describes 13 patients, predominantly young women, with a syndrome characterized by rapid loss of one or more large zones of outer retinal function, photopsia, minimal funduscopic changes, and electroretinographic abnormalities affecting one or both eyes. All patients on follow-up examination had persistent visual field defects, and most had chronic photopsia and zones of pigment epithelial atrophy. Evidence is presented that these patients probably represent part of the spectrum of a single disorder that includes the multiple evanescent white-dot syndrome (MEWDS), acute idiopathic blind-spot-enlargement syndrome (AIBSES), acute macular neuroretinopathy (AMN), and the pseudo-presumed ocular histoplasmosis syndrome (P-POHS). METHODS: The medical records of these 13 patients and 2 additional patients, who developed, in addition to the features of this syndrome, funduscopic changes typical of MEWDS, AMN, and P-POHS, were reviewed and follow-up obtained. RESULTS: These patients had extensive unrewarding medical and neurological investigations because of suspected diagnoses, including central nervous system disorders, cancer-associated retinopathy, retinal vasculitis, diffuse unilateral subacute neuroretinitis, and tapetoretinal degenerations. Although most patients retained good visual acuity, all had permanent visual field loss that in some cases was severe. The cause of the disorder was not determined. No effective treatment was found. CONCLUSIONS: Acute visual loss and photopsia in these patients is probably caused by damage to large zones of the outer retina that appears unaffected ophthalmoscopically. Electroretinography is important in early diagnosis. Future investigations probably will reveal further evidence linking this disorder to MEWDS, AIBSES, AMN, and P-POHS.

Acute Disease↗

Ocular complications of Wegener's granulomatosis.

140 patients with biopsy-proven Wegener's granulomatosis were examined during a 16-year period at the Mayo Clinic. Forty patients had diverse and explosive ophthalmic involvement, including conjunctivitis, episcleritis, scleritis, corneal ulceration, uveitis, retinal vasculitis, optic neuropathy, orbital mass, orbital cellulitis, and obstruction of the nasolacrimal duct. The multiple ophthalmic and systemic complications in these 40 patients and the importance of establishing the pathologic diagnosis are discussed. Treatment with corticosteroids and immunosuppressive agents has dramatically improved the prognosis, although substantial mortality (four patients died of Wegener's granulomatosis) and ocular morbidity (three eyes were enucleated) are still associated with this disease.

Adolescent↗

[Treatment of ocular inflammatory disease with glucocorticoids and cyclophosphamide pulses].

UNLABELLED: The effectiveness, toxicity and prognostic factors influencing responses to cyclophosphamide (CP) iv pulses plus oral glucocorticosteroids (GC) in patients with GC-resistant ocular inflammatory diseases (OID) was evaluated in a cohort of 15 consecutive patients suffering from active, non-infectious OID refractory to oral GC. All patients underwent monthly evaluations with clinical, hematological, hepatic, renal and ophthalmologic tests. These included checking visual acuity and both anterior chamber and posterior segment inflammation. The overall effect evaluated by repeated measurements ANOVA demonstrated that after an average of 5 CP pulses 1-10, the group showed significant improvement regarding of visual acuity and inflammation (p < 0.000001). Amelioration was not sustained over time in patients with granulomatous uveitis. Patients with retinal vasculitis experienced rapid and sustained recovery. By the end of the follow-up period, 53% of the patients had improved, 20% remained stationary and 26% suffered visual acuity deterioration as compared with the baseline. No serious side effects were detected during treatment and follow-up. CONCLUSIONS: A combination of oral GC and iv CP pulses appears to be an effective way to treat patients suffering from noninfectious, non-granulomatous, GC-resistant OID.

Adult↗

[Childhood Behçet's disease: clinical and evolutive aspects. About 13 cases].

INTRODUCTION: Behçet's disease is a systemic vasculitis which rarely occurs in childhood. The aim of this study was to evaluate clinical characteristics and outcome of Behçet's disease in Moroccan children. MATERIAL AND METHODS: A retrospective study of 13 cases of children, 10 males and 3 females with Behçet's disease followed up between 1990 and 1998. The diagnosis of Behçet's disease was based on the criteria of the international study group for Behçet's disease. All patients were studied by a complete clinical, ophthalmological and laboratory staging and treated with appropriate therapy. RESULTS: The mean age at diagnosis of Behçet's disease was 13.9 years. Familial forms were found in 30.7% of cases. Oral aphtae were noted in all cases while genital ulcers were present in 76% of cases. Cutaneous lesions were found in only 1 case and 53.8% of children had a pathergy test. Articular involvement was found in 30.7%, neurological features in 46% and vascular manifestations in 38.4%. Only one case of intestinal involvement was noted. Ocular features (76%) were bilateral in all cases and were dominated by panuveitis complicated by macular edema ant retinal vasculitis. CONCLUSION: Behçet's disease seems to have particular characteristics in childhood. Familial forms, articular and digestive manifestations appear to be more frequent in early stages of Behçet's disease in children. Neurological and vascular involvement with panuveitis seems more frequent in the older children.

Adolescent↗

Acute retinal necrosis syndrome associated with herpes simplex keratitis.

BACKGROUND: Although acute retinal necrosis (ARN) syndrome is caused by a herpes group virus, association of herpetic keratitis with ARN syndrome is uncommon. A case of unilateral ARN syndrome with herpes simplex keratitis is discussed. METHODS: A 40-year-old man developed unilateral keratitis, necrotic retinitis, retinal vasculitis, vitritis, and iritis consistent with ARN syndrome 1 month after treatment for ipsilateral facial nerve palsy and auricular herpetic vesicles (Tolosa-Hunt syndrome). Impression cytologic examination of the corneal epithelial ulcer that developed concurrent with the intraocular findings and of the aqueous humor (obtained by paracentesis) was performed. RESULTS: Cells that reacted with anti-herpes simplex virus type 1 (HSV-1) antibody were detected on impression cytology, and DNA fragments corresponding to the HSV-1 DNA sequence were detected in the aqueous humor. CONCLUSION: This case shows that ARN syndrome may, on rare occasions, be associated with herpes keratitis secondary to HSV-1.

Adult↗

Results of vitrectomy for rare proliferative and hemorrhagic diseases.

We compiled the results of pars plana vitrectomy in rare proliferative and hemorrhagic diseases. In retinal vasculitis of unknown origin (perhaps Eales' disease), six out of seven eyes had visual improvement; in sickle cell disease, two out of three eyes improved. Three eyes with presumed toxocariasis were successfully treated. In retrolental fibroplasia (RLF), only lighter degrees of the cicatricial stage with traction detachments can be treated. Some cases may simulate Coats' disease. There are also cases which simulate the typical picture of RLF, where there is no history of prematurity or oxygen usage. Five patients with RLF were treated, three of these successfully. Cryoglobulinemia (one case) and hereditary hemorrhagic telangiectasia (one case) were successfully treated. Senile macular degeneration can also be an indication for vitrectomy (two out of three eyes were successfully treated).

Adolescent↗

Symptomatic intraocular inflammation in multiple sclerosis.

PURPOSE: The aim of this study was to determine the type and outcome of uveitis in patients with multiple sclerosis. METHODS: A retrospective study of 16 patients attending the uveitis clinic who had a clinically definite diagnosis of multiple sclerosis and symptomatic intraocular inflammation. The type of uveitis and complications present as well as the outcome of therapeutic intervention, either medical or surgical, was determined. RESULTS: Chronic, bilateral anterior uveitis was present in six patients. Secondary cataracts requiring surgery occurred in 10 eyes, and trabeculectomy was done on five eyes for secondary glaucoma. Symptomatic vitritis (visual blurring and/or visually significant floaters) occurred in eight patients and was bilateral in four. Occlusive peripheral retinal vasculitis with subsequent areas of ischaemia and neovascularization requiring panretinal laser photocoagulation developed in eight eyes, five of which also required vitrectomy. Other posterior segment complications included macular oedema (four) and epiretinal membrane involving the macula (two). Five patients required systemic steroids for control of posterior uveitis. The median visual improvement following cataract surgery or vitrectomy was 3 Snellen lines to a median visual acuity of 6/18. CONCLUSIONS: A wide range of clinical patterns of uveitis, which may be both symptomatic and sight-threatening, occur in patients with multiple sclerosis. In addition, the complications of uveitis may cause significant visual morbidity, much of which is amenable to appropriate surgical intervention. It is important that these potentially remediable conditions are recognized and treated to ensure that patients with multiple sclerosis retain optimum visual function.

Adult↗

Acute syphilitic optic neuritis.

Two patients experienced acute unilateral visual loss as a manifestation of early infectious (secondary) neurosyphilis. In both cases, optic disc swelling was preceded by the development of a characteristic rash accompanied by mild signs of meningeal inflammation. Other signs of ocular inflammation, such as uveitis or retinal vasculitis, were absent. Optic neuritis may occur as a manifestation of the meningeal inflammation that can accompany secondary syphilis. In such patients, it is important to confirm involvement of the CNS and to institute appropriate antibiotic therapy.

Acute Disease↗

Toxocara canis infection of the eye. Correlation of clinical observations with developing pathology in the primate model.

A primate model for the study of Toxocara canis infection was created by intravitreal, periocular, systemic, and intracarotid injection of viable larvae in cynomolgus monkeys. The results were as follows: (1) Intravitreal larvae caused retinal hemorrhages, perivasculitis, mild vitritis, and retinal nodules. (2) Apparently viable larvae without inflammatory reaction were found in the vitreous, retina, and optic nerves up to nine months after intravitreal inoculation. (3) Other larvae were surrounded by an acute inflammatory granuloma or a chronic fibrotic granuloma, but they did not appear to be necrotic. (4) Toxocara canis larvae apparently had the ability to move through ocular tissue, to leave the site of tissue reaction, and to exit via the optic nerve. Viable larvae were found in the retina up to 15 months after inoculation. (5) Culture fluid containing Toxocara proteins stimulated a severe retinal vasculitis. Dead larvae caused little reaction. (6) Positive aqueous and vitreous enzyme-linked immunosorbent assay titers were found in the most severely inflamed eyes, which often harbored live larvae.

Animals↗

Intravenous pulse methylprednisolone therapy in severe inflammatory eye disease.

Seventeen patients with severe ocular inflammatory disease were treated with intermittent pulse doses of intravenous methylprednisolone. There were five patients with chronic posterior uveitis, five with retinal vasculitis, three with scleritis, two with chronic anterior uveitis, one with pars planitis, and one with Mooren's ulcer. A standard protocol of intravenous administration of methylprednisolone was followed. In 15 of 17 patients receiving this therapy, visual acuity improved or stayed constant. Side effects included psychological disturbances, hypertension, and elevated glucose levels, but cessation of treatment was not necessary in any patient. Pulse methylprednisolone treatment appears to be an effective therapy for several forms of severe inflammatory eye disease and minimizes the potential side effects of more conventional regimens of corticosteroid administration.

Adolescent↗