1/377. scleritis and temporal arteritis.Thirty consecutive patients with severe scleritis or episcleritis were admitted as in-patients to the Medical ophthalmology Unit and assessed for systemic disease. There were seventeen women and thirteen men. The mean age was 53 with a median of 57 (range 23-83). Eighteen of the patients had scleritis: eleven of these had evidence of connective tissue disease and three of them had temporal arteritis. Twelve patients had episcleritis: six of them had a collagen disease and one of them developed temporal arteritis. This high incidence of temporal arteritis in association with scleritis has not been previously reported. It is important to diagnose and treat overt temporal arteritis early with parenteral steroids so that ischaemic papillopathy can be avoided. A higher incidence of collagen diseases than previously described is reported in episcleritis. It is thought that this is secondary to selection since patients with the usual self-limiting episcleritis are not normally referred for further in-patient investigation. In no patient was more than one significant diagnosis made. There was no significant medical illness in only 11% of patients with scleritis and 33% of patients with episcleritis. The majority of the non-collagen diseases (e.g. hypertension) were not previously recognized. In none of the patients with temporal arteritis was the diagnosis made before admission. It is concluded that full examination and investigation for underlying disease is indicated in both scleritis and severe episcleritis.- - - - - - - - - - ranking = 1keywords = disease (Clic here for more details about this article) |
2/377. Lymphoid lesions of the conjunctiva: relation of histopathology to clinical outcome.A retrospective clinicopathologic study of 40 patients with lymphoid lesions of the conjunctiva demonstrated the validity of current histologic criteria in predicting clinical outcome. overall histologic architecture as well as cytologic detail must be used to differentiate benign reactive lymphoid hyperplasia from lymphoma. Lesions verified clinically as being malignant had obvious malignant cytologic features. Clinical signs of surface follicularity, multifocality, and minimal elevation suggest benignancy. All the benign lesions, on histopathologic examination, were either follicular in architecture or composed of mature lymphocytes, and were generally restricted to the substantia propria. Bilaterality and clinical recurrence do not necessarily imply a malignant disease.- - - - - - - - - - ranking = 0.16666666666667keywords = disease (Clic here for more details about this article) |
3/377. Ophthalmic abnormalities in patients with cutaneous T-cell lymphoma.PURPOSE: To determine the frequency of ophthalmic abnormalities in patients with cutaneous T-cell lymphoma (mycosis fungoides and sezary syndrome) and T-cell lymphoma involving the skin and to describe the clinical course of the disease with selected examples. methods: A computerized diagnostic retrieval system was used to identify all patients with T-cell lymphoma involving the skin who were examined at the Mayo Clinic (Rochester, minnesota) between January 1, 1976 and December 31, 1990. The medical records of affected patients were reviewed. RESULTS: During the 15-year interval from 1976 through 1990, cutaneous T-cell lymphoma was diagnosed in 2,155 patients. Of these 2,155 patients, 42 (1.95%; 26 male and 16 female) had at least 1 ophthalmic abnormality attributable to the disease. The diagnoses in these 42 patients were mycosis fungoides in 19, clinical variants of T-cell lymphoma of the skin (most commonly, peripheral T-cell lymphoma) in 11, and sezary syndrome in 12. Cicatricial eyelid ectropion was the most common finding, affecting 17 (40.4%) of the 42 patients. Thirty-seven patients had findings that, although probably not a direct consequence of cutaneous T-cell lymphoma, have been cataloged in previous studies. CONCLUSION: Although ophthalmic abnormalities in patients with cutaneous T-cell lymphoma are relatively uncommon, the manifestations of the disease are diverse and frequently difficult to treat.- - - - - - - - - - ranking = 0.5keywords = disease (Clic here for more details about this article) |
4/377. The ocular manifestations of congenital infection: a study of the early effect and long-term outcome of maternally transmitted rubella and toxoplasmosis.PURPOSE: To study the spectrum of adverse ocular effects which result from maternally transmitted rubella and toxoplasma infection; further, to record the long-term visual and neurodevelopmental outcomes of these 2 major causes of fetal infection. STUDY DESIGN AND patients: A series of 55 patients with congenital infection have been studied prospectively on a long-term basis. The study group included a cohort of 34 cases with congenital rubella syndrome demonstrated by virus isolation, and 21 cases with a clinical diagnosis of congenital toxoplasmosis and serologic confirmation. All patients had specific disease-related ocular defects. Rubella patients were first identified during or following the last major rubella epidemic in 1963-1964, and some have been followed serially since that time. A separate study group of representative toxoplasmosis patients presented for examination and diagnosis at varying time periods between 1967 and 1991. OBSERVATIONS AND RESULTS: This study confirms that a broad spectrum of fetal injury may result from intrauterine infection and that both persistent and delayed-onset effects may continue or occur as late as 30 years after original infection. Many factors contribute to the varied outcome of prenatal infection, the 2 most important being the presence of maternal immunity during early gestation and the stage of gestation during which fetal exposure occurs in a nonimmune mother. RUBELLA: As a criteria of inclusion, all 34 rubella patients in this study exhibited one or more ocular defects at the time of birth or in the immediate neonatal period. Cataracts were present in 29 (85%) of the 34, of which 21 (63%) were bilateral. Microphthalmia, the next most frequent defect, was present in 28 (82%) of the 34 infants and was bilateral in 22 (65%). glaucoma was recorded in 11 cases (29%) and presented either as a transient occurrence with early cloudy cornea in microphthalmic eyes (4 patients), as the infantile type with progressive buphthalmos (1 patient), or as a later-onset, aphakic glaucoma many months or years following cataract aspiration in 11 eyes of 6 patients. Rubella retinopathy was present in the majority of patients, although an accurate estimate of its incidence or laterality was not possible because of the frequency of cataracts and nystagmus and the difficulty in obtaining adequate fundus examination. toxoplasmosis: Twenty-one patients with congenital toxoplasmosis have been examined and followed for varying time periods, 7 for 20 years or more. The major reason for initial examination was parental awareness of an ocular deviation. Twelve children (57%) presented between the ages of 3 months and 4 years with an initial diagnosis of strabismus, 9 of whom had minor complaints or were diagnosed as part of routine examinations. All cases in this study have had evidence of retinochoroiditis, the primary ocular pathology of congenital toxoplasmosis. Two patients had chronic and recurrent inflammation with progressive vitreal traction bands, retinal detachments, and bilateral blindness. Macular lesions were always associated with central vision loss; however, over a period of years visual acuity gradually improved in several patients. Individuals with more severe ocular involvement were also afflicted with the most extensive central nervous system deficits, which occurred following exposure during the earliest weeks of gestation. CONCLUSIONS: Although congenital infection due to rubella virus has been almost completely eradicated in the united states, the long-term survivors from the prevaccination period continue to experience major complications from their early ocular and cerebral defects. They may be afflicted by the persistence of virus in their affected organs and the development of late manifestations of their congenital infection. Congenital toxoplasmosis continues to be the source of major defects for 3,000 to 4,100 infants in the united states each year; the spectrum of defects is wide and may vary from blindness and severe mental retardation to minor retinochoroidal lesions of little consequence. Effective solutions for either the prevention or treatment of congenital toxoplasmosis have not been developed in this country but are under intensive and continuing investigation.- - - - - - - - - - ranking = 0.16666666666667keywords = disease (Clic here for more details about this article) |
5/377. diplopia secondary to aniseikonia associated with macular disease.OBJECTIVE: To provide an explanation for diplopia and the inability to fuse in some patients with macular disease. methods: We identified 7 patients from our practices who had binocular diplopia concurrent with epiretinal membranes or vitreomacular traction. A review of the medical records of all patients was performed. In addition to complete ophthalmologic and orthoptic examinations, evaluation of aniseikonia using the Awaya New aniseikonia Tests (Handaya Co Ltd, tokyo, japan) was performed on all patients. RESULTS: All patients were referred for troublesome diplopia. Six of the patients had epiretinal membranes and 1 had vitreomacular traction. All 7 patients had aniseikonia, ranging from 5% to 18%. In 5 of the patients the image in the involved eye was larger, and in the other 2 patients it was smaller than in the fellow eye. All patients had concomitant small-angle strabismus and at least initially did not fuse when the deviation was offset with a prism. Response to optical management and retinal surgery was variable. CONCLUSIONS: aniseikonia caused by separation or compression of photoreceptors can be a contributing factor to the existence of diplopia and the inability to fuse in patients with macular disease. Concomitant small-angle strabismus and the inability to fuse with prisms may lead the clinician to the incorrect diagnosis of central disruption of fusion. Surgical intervention does not necessarily improve the aniseikonia.- - - - - - - - - - ranking = 1keywords = disease (Clic here for more details about this article) |
6/377. A Colombian family with X-linked juvenile retinoschisis with three affected females finding of a frameshift mutation.X-linked retinoschisis (XLRS) is a vitreoretinal disease responsible for most cases of juvenile macular degeneration in males. retinoschisis carrier females generally manifest no pathological symptoms. However, a large affected family from colombia presented three affected females with typical RS phenotype similar to their 27 affected male relatives. Fundus examination as well as electroretinograms (ERG) indicate that the disease in these three affected females is as severe as in their affected male counterparts. dna sequence analysis of the XLRS1 gene in the affected members of this family indicates a single base (G) deletion at the 639 base position (639delG). This deletion causes a frameshift during translation and results in a larger (235 amino acids) than normal peptide (224 amino acids) with grossly altered discoidin domain, which is considered critical for the cellular function of the protein. The co-segregation of this gene mutation with the RS phenotype and the RS carrier status as well as its complete absence in normal controls indicates that this genetic change is responsible for the RS pathology in this family. This (639delG) is a novel RS mutation and reported here for the first time. Furthermore, the analysis of the three affected females indicates that the RS pathology in affected females (a very rare occurrence) is due to XLRS1 mutations carried on both of their X chromosomes.- - - - - - - - - - ranking = 0.33333333333333keywords = disease (Clic here for more details about this article) |
7/377. Spondylo-epiphyseal dysplasia with ocular changes: report of two "new" variants in two different families.Two families with "new" types of spondyloepiphyseal dysplasias are reported. The disease(s) are characterised by spinal changes, capital femoral epiphyseal involvement and minor changes in the first family and shortening of the hands and feet in the second.- - - - - - - - - - ranking = 0.16666666666667keywords = disease (Clic here for more details about this article) |
8/377. Neonatal asteroid hyalosis.Asteroid hyalosis, noted in 0.83% of routine eye examinations, is uncommon in younger patients and is more frequently seen in patients more than 60 years old. It has been considered to be related to an aging process, and when it occurs in younger patients, ocular disease is typically associated. We report a 4-week-old patient with down syndrome and bilateral congenital cataracts who had unilateral asteroid hyalosis.- - - - - - - - - - ranking = 0.16666666666667keywords = disease (Clic here for more details about this article) |
9/377. Cogan's syndrome: an oculo-audiovestibular disease.Typical Cogan's syndrome is a rare disease of young adults consisting of flares of interstitial keratitis and sudden onset of Meniere-like attacks (nausea, vomiting, tinnitus, vertigo and hearing loss). life-threatening aortic insufficiency develops in 10% of reported cases. Atypical Cogan's syndrome (audiovestibular dysfunction with other types of inflammatory eye disease) is associated with vasculitis in 20% of cases and has a less favourable prognosis than typical Cogan's syndrome.- - - - - - - - - - ranking = 1keywords = disease (Clic here for more details about this article) |
10/377. Double phakomatosis.In the phakomatoses--von Recklinghausen's disease, the sturge-weber syndrome, tuberous sclerosis, and von hippel-lindau disease--pathologic changes in the eye are often evident. Most cases are described as a single entity. The following is a case report of concomitant sturge-weber syndrome and von Recklinghausen's disease in which both conditions involved the eye.- - - - - - - - - - ranking = 0.5keywords = disease (Clic here for more details about this article) |
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