Extraskeletal osteosarcoma of the orbit: A clinicopathologic case report and review of literature.

Primary extraskeletal osteosarcoma (EOS) is an extremely rare malignancy. In this report, the clinical course of a 32-year-old man presenting with proptoses is described. Medical history included Hirschsprung disease (HD), horseshoe kidney, azoospermia, and vertebral anomalies. Imaging of the orbit showed an oval, well-defined heterogeneous mass adjacent to the lateral wall of the orbit. The patient underwent a lateral orbitotomy and complete excision of the mass. The mass was not attached to the bone. Histopathologic and immunohistochemical examination confirmed the diagnosis of an EOS. The patient received chemotherapy and radiotherapy and is free of the disease 3 years after the diagnosis. Genetic screening showed no mutations for both the RET proto-oncogene for HD and the p53 tumor suppressor gene for osteosarcoma.

Symptomatic bilateral choroidal metastasis from breast cancer as first clinical sign of advanced metastatic tumor disease 31 years after diagnosis.

We report a 62-year-old caucasian woman with bilateral choroidal metastases as only clinical presenting sign of advanced metastatic tumour disease. She presented with decreased vision in the left eye since 5 days. She was treated for breast cancer 31 years before. Fundoscopy and ultrasound analysis showed a large choroidal metastasis in the left eye and one asymptomatic lesion in the right eye. Systemic screening revealed multiple lung and bone metastases. Health practitioners should be aware that choroidal metastasis from breast carcinoma can present throughout life. Small asymptomatic lesions may be detected that still can be treated effectively.

Expression of chemokines and gelatinase B in sympathetic ophthalmia.

PURPOSE: To examine the expression of gelatinase B (matrix metalloproteinase-9) and the chemokines monocyte chemotactic protein-1 (CCL2/MCP-1) and stromal cell-derived factor-1 (CXCL12/SDF-1) in sympathetic ophthalmia (SO). METHODS: Five enucleated exciting eyes with a clinical diagnosis and typical histopathological findings of SO were studied by immunohistochemical techniques using a panel of monoclonal antibodies directed against gelatinase B, MCP-1, and SDF-1. In addition, a panel of monoclonal and polyclonal antibodies was used to characterize the composition of the inflammatory infiltrate. RESULTS: In all cases, the extensive uveal inflammatory infiltrate was organized as a diffuse infiltrate and as large granulomas consisting of epithelioid cells and multinucleated giant cells. CD20(+) B lymphocytes predominated in the diffuse infiltrate and CD3(+) T lymphocytes were few. The monocyte/macrophage marker CD68 was expressed in scattered inflammatory mononuclear cells and within granulomas and Dalen-Fuchs nodules. Most of the inflammatory cells were HLA-DR(+). Immunoreactivity for gelatinase B, MCP-1, and SDF-1 was observed in cells within granulomas and in scattered epithelioid cells. Immunoreactivity for MCP-1 was noted in retinal pigment epithelial cells. Endothelial cells of choriocapillaries showed weak immunoreactivity for SDF-1. CONCLUSIONS: Gelatinase B, MCP-1, and SDF-1 might have a pathogenic role in the recruitment of leucocytes into the eye in SO.

Diagnosis and treatment of cytomegalovirus iridocyclitis without retinal necrosis.

AIM: To describe the diagnostic and therapeutic management of cytomegalovirus (CMV) anterior uveitis unassociated with retinal necrosis in immunocompetent patients. METHODS: Patients referred between 2001 and 2003 for management of unilateral, chronic, recurrent uveitis associated with secondary glaucoma underwent extensive investigation including laboratory tests for herpes virus infections. Specific antiviral treatment was initiated in all cases and the level of ocular inflammation was evaluated during the follow up. RESULTS: Five patients, three men and two women, were included. Median age was 50 years old (range 30-80 years). Anterior unilateral uveitis without iris atrophy was observed in all cases. Uveitis was chronic in three cases and recurrent in two cases. Glaucoma was observed in all patients with a median intraocular pressure of 30 mm Hg (range 22-43 mm Hg). Five patients responded initially to specific anti-CMV therapy. Even though glaucoma surgery was necessary in two cases, both ocular inflammation and glaucoma were controlled in all cases. Relapses occurred in three cases after cessation of therapy, requiring prolonged maintenance therapy with valganciclovir. CONCLUSIONS: CMV infection and specific antiviral therapy should be considered in all cases of relapsing or chronic iridocyclitis and secondary glaucoma. Maintenance regimens of valganciclovir may be necessary to prevent further relapses.

An eye on inflammatory eye disease.

The purpose of this article is to outline the interaction between ophthalmologists and internists in the management of uveitis. Two issues will be addressed: 1) which strategies should the internist follow when asked to investigate a case of uveitis; and 2) in which systemic diseases should the internist order an ocular examination to rule out intraocular inflammation. The modern approach to the diagnosis of uveitis is based on the naming-meshing system popularized by Smith and Nozik. After a short history (ocular complaints, general health) an ophthalmic examination is carried out to determine the anatomic structures involved. Based on the results a uveitis is classified as anterior uveitis, intermediate uveitis, posterior uveitis, or panuveitis. Associated factors (eg, unilateral versus bilateral, acute versus chronic, granulomatous versus nongranulomatous, etc.) are also assessed. Based on this information the type of uveitis will be named (eg, acute, nongranulomatous, unilateral, anterior uveitis) and matched (meshing) to a potential list of etiologies (eg, viral iritis, HLA-B 27 associated iritis). Targeted questioning and selected medical and laboratory investigations based on the shortlist will then identify a possible cause for a particular patient's uveitis. In other words the ophthalmologist should never ask the internist to run the full battery of tests in a patient with uveitis. He rather should indicate which type of uveitis is present and what are the most likely diagnoses to be excluded. Many systemic diseases cause diffuse inflammation and are associated with uveitis. These include tuberculosis, spirochaetal diseases such as Lyme disease and syphilis, sarcoidosis, Behçet syndrome, juvenile idiopathic arthritis, and HIV infection amongst many others. Routine ophthalmic examination in patients with systemic disease may be indicated for diverse reasons: to prevent profound damage due to asymptomatic uveitis in JIA; to detect diagnostic clues in patients with febris e causa ignota; or to rule out opportunistic infections in HIV positive patients. It is clear that the information gained from routine examination in systemic disease will be greatly dependent on the prevalence of ocular involvement in a particular disease.

Myopia: more than a refractive error--LASIK and retinal dystrophies.

Three patients who had undergone laser in situ keratomileusis (LASIK) correction for myopia were first seen because of suboptimal visual acuity (VA) and night blindness and/or photophobia. After a comprehensive examination including psychophysical and electrophysiological tests, two of the three patients were shown to suffer from a progressive conerod dystrophy. The third patient had retinitis pigmentosa. These cases illustrate the need for in depth preoperative evaluation in myopic patients about to undergo LASIK when signs or problems of night blindness and/or photophobia are present.

Screening for glaucoma in a general population with the non-mydriatic fundus camera and the frequency doubling perimeter.

PURPOSE: To evaluate the usefulness of non-mydriatic fundus camera (NMFu-camera) and frequency doubling perimeter (FDP) for detecting glaucoma in a general population. METHODS: This prospective observational multicenter study consisted in screening for glaucoma in the populations of three Belgian cities. Intraocular pressure (IOP) was measured with non-contact pneumo-tonometer (NCT) and applanation tonometry (AT) if NCT IOP was > or = 17 mmHg. Visual field was screened with FDP (C-20-5) and digitized optic disc photographs (ODPs) were taken with NMFu-camera. FDP was considered abnormal if at least one defective point was found. ODPs were graded as normal or glaucomatous by consensus of three glaucoma specialists. Optic disc and visual field results were matched per eye. Subjects with known ocular hypertension and/or treated primary open angle glaucoma were excluded from the analysis. RESULTS: A total of 1620 subjects were included in the study. Their mean age was 63.2 years. AT IOP was > 21 mmHg in 8.2%. A total of 98.1% of ODPs could be interpreted. Glaucomatous optic discs were detected in 3.5% of the subjects. In this group only 24% had an AT IOP > or = 22 mmHg. FDP was abnormal in 44.5%. The sensitivity and specificity of FDP to identify patients with an optic disc graded as glaucomatous were 58.6% and 64.3% respectively. CONCLUSIONS: The combined use of the NMFu-camera and the FDP is a feasible method for an initial glaucoma mass screening. NMFu-camera may be a useful and quick method to screen for glaucomatous damage in a community. FDP in screening strategy was revealed to be not sensitive enough when setting the cut-off value at one defective test location. IOP measurements were confirmed to be a poor tool to detect glaucomatous damage.

Effect of different sterilisation methods on the properties of bioadhesive powders and ocular minitablets, and clinical evaluation.

The purpose of this study was to evaluate the influence of gamma-irradiation and dry heat sterilisation on the properties of a bioadhesive powder mixture containing ciprofloxacin and its corresponding ocular minitablets. The molecular weight characteristics of drum dried waxy maize starch (DDWM), employed as major component of the bioadhesive formulation, the decay kinetics of radicals, the rheological properties of the bioadhesive polymers and the microbial activity of ciprofloxacin were studied. The influence of the different sterilisation methods on the characteristics of the ocular minitablets was investigated by measuring the crushing strength, the friability, and the in vitro release of ciprofloxacin from the minitablets. Finally, the clinical value of the selected sterilised minitablets was evaluated in seven healthy volunteers. Both sterilisation methods similarly affected the properties of the bioadhesive formulation by inducing stable radicals and decreasing the molecular weight of DDWM, although no changes in the microbiological activity of ciprofloxacin were measured. An obvious influence of both sterilisation methods was observed in the in vitro release study. The crushing strength and friability of the minitablets were not significantly influenced by gamma-irradiation. Based on these data, gamma-irradiation was more adequate as sterilisation method for the bioadhesive ocular minitablets than dry heat sterilisation, because it affected the least the physical properties of the minitablets. Therefore, the gamma-sterilised minitablets were selected for an in vivo evaluation in seven volunteers. The concentration of ciprofloxacin in the tear film remained above its MIC value for the most common ocular pathogens for at least 8 h. Consequently, the gamma-irradiated minitablets containing ciprofloxacin can be considered as a promising formulation to treat bacterial keratitis and conjunctivitis.

Alport syndrome and conjunctival telangiectasia.

We report a patient with ocular abnormalities due to Alport syndrome. Considerable improvement of visual acuity was obtained following ocular lens extraction with foldable lens implantation in the right eye to compensate the effect of the lenticonus anterior. The presence of conjunctival telangiectasia is a new clinical finding in a patient with Alport syndrome.

Clinical and electrophysiological findings in autosomal dominant vitreoretinochoroidopathy: report of a new pedigree.

PURPOSE: To report the clinical and electrophysiological findings in a three-generation pedigree with autosomal dominant vitreoretinochoroidopathy. METHODS: Sixteen members of a three-generation pedigree with autosomal dominant vitreoretinochoroidopathy were examined clinically, including measurement of the corneal diameter. In 14 persons, Goldmann perimetry, axial length determination and electro-oculography were carried out. Electroretinography, according to ISCEV standards, was performed in 11 of 12 affected persons. RESULTS: Characteristic annular peripheral pigmentary changes were present in all affected members, as well as chorioretinal atrophy varying from a tigroid aspect to marked atrophy. Four patients presented a microcornea and shallow anterior chamber without microphthalmia. The visual fields appeared to narrow with ageing. The electro-oculography was pathological in the affected patients and normal in the unaffected. The electroretinographic amplitude responses tended to worsen with age, with maintenance of near normal latencies. CONCLUSION: The clinical presentation of autosomal dominant vitreoretinopathy is variable. Electrooculography seems to be a discriminative test. The condition may be associated with anterior segment abnormalities other than presenile cataract, such as microcornea, shallow anterior chamber and angle closure glaucoma.

The effect of duration and timing of systemic cyclosporine therapy on corneal allograft survival in a rat model.

BACKGROUND: Systemic cyclosporine A (CsA) remains a valuable treatment option in the prevention of corneal graft rejection, but the question of timing and duration of this systemic therapy remains unresolved. The effect of a pre- and postoperative dosing schedule, related to the expected moment of rejection, was examined in a rat model. METHODS: All AO (strain) recipients of PVG grafts were assigned to the following treatment groups: Group 1 (controls), groups 2-5 (a postoperative treatment regimen of CsA for 5, 10, 15 and 30 days respectively) and groups 6 and 7 (CsA preoperatively for 5 days and postoperatively for another 5 or 10 days respectively). Corneal allografts were clinically evaluated and blood CsA levels were measured at various time points. RESULTS: Untreated controls rejected their allografts after 13 days. Regression analysis showed a strongly significant positive correlation between graft survival time and duration of cyclosporine therapy. There was no difference in graft survival between groups 3 (CsA 10 days) and 4 (CsA 15 days). A pre-operative dosing schedule of CsA followed by postoperative treatment had no advantage over a solely postoperative treatment regimen. The moment of rejection was characterized by a low to undetectable CsA concentration. CONCLUSION: The present study demonstrates a significant influence of the duration of systemic CsA administration on allograft survival time. However, preoperative administration of CsA does not seem to have an additional influence on graft survival, which is in line with the biological evidence of the mechanism of action of CsA on the efferent arm of graft rejection.

Bilateral corneal melting in a patient with paraneoplastic pemphigus.

An 80-year-old man, with a solid abdominal tumor and multiple skin lesions, was admitted to the hospital because of a perforated right cornea and an impending perforation of the left. The clinical, histological, immunohistological and immunoprecipitation findings of the skin lesions were consistent with Anhalt's criteria for paraneoplastic pemphigus (PNP). The underlying malignancy proved to be an incurable peripheral neuronal shaft tumor. Both conjunctivae appeared normal. The right eye revealed a flat anterior chamber, due to a spontaneous, central corneal perforation. The central part of the left cornea had severely thinned, resulting in a descemetocele, which eventually perforated. Multiple surgical interventions were needed to restore the anterior chamber in both eyes. Although a causative association between PNP and corneal perforation could not be demonstrated, we think that corneal melting should be added to the list of ocular complications in patients with PNP.

HIV/AIDS and blindness.

Nearly 34 million people are currently living with HIV/AIDS: ocular complications are common, affecting 50% to 75% of all such patients at some point during the course of their illness. Cytomegalovirus retinitis is by far the most frequent cause of vision loss in patients with AIDS. Although the prevalence of cytomegalovirus retinitis is decreasing in industrialized countries because of the widespread availability of highly active antiretroviral therapy, between 10% and 20% of HIV-infected patients worldwide can be expected to lose vision in one or both eyes as a result of ocular cytomegalovirus infection. Less frequent but important causes of bilateral vision loss in patients with HIV/AIDS include varicella zoster virus and herpes simplex virus retinitis, HIV-related ischaemic microvasculopathy, ocular syphilis, ocular tuberculosis, cryptococcal meningitis, and ocular toxic or allergic drug reactions. At present, most patients with HIV/AIDS in developing countries who lose their vision have a very limited life expectancy. As antiretroviral therapy makes its way to these countries, however, both life expectancy and the prevalence of blindness related to HIV/AIDS can be expected to increase dramatically.

Mesopic visual acuity requirements for driving licenses in the European Union-Research Report.

In this study different methods for determining the mesopic visual acuity in candidates for driving licenses group 2 are presented (Royal Decree, 1998, European norms). Results of normal subjects are compared. Depending on the method used, visual acuity obtained under mesopic conditions, with an illuminance of 1 lux, showed a mean ranging from 0.5 to 0.65, which is clearly above the legal norm of 0.2. A comparative study of the three published official norms demonstrates that the stringent norms of 1966 do not match those of 1988 and 1998, resulting in a wider margin of acceptability, to the advantage of the candidate.

Ocular manifestations of graft versus host disease following bone marrow transplantation.

The ocular manifestations of Graft Versus Host Disease (GVHD) include keratoconjunctivitis sicca, cicatricial lagophthalmos, sterile conjunctivitis, corneal epithelial defects, corneal ulceration and melting. These manifestations are more frequent in patients with chronic GVHD than in patients with acute GVHD. The more severe ocular complications are associated with severe systemic chronic GVHD and poorer survival. Recent improvements in the systemic management of these patients have led to the more frequent recognition of the ocular problems. The high prevalence of ocular involvement and potentially severe ocular problems in GVHD patients necessitate close ophthalmic monitoring.