bluuebunny/crossref_metadata_2025 huggingface dataset:
Combined OCT retinal nerve fibre layer analysis and VEP in neuro‐ophthalmic disease
Authors: GOOD P
Published: November 2010

Purpose Ocular Coherence Tomography (OCT) has become a valuable tool in assessing retinal nerve fibre layer thickness (RNFL) in Patients with optic nerve disease. This study is designed to compare RNFL thickness n with Visual Evoked Cortical Potentials (VECP)in patients with known optic nerve disease and comparing these to a group of patients with primary open angle glaucoma (POAG).

Methods Twenty Patients (37 eyes) with clinically determined optic nerve disease underwent pattern reversal VECP and also OCT using a Spectralis OCT system. Assessment of global and segmental RNFL was made. Six Patients were diagnosed as Dominant Optic atrophy, 3 with Lebers Optic Neuropathy (LHON), 6 with Nutritional amblyopia, 3 with Anterior Ischaemic Optic Neuropathy (AION), and 2 with Demyelinating disease. These Patients were also compared to a group of 10 patients (20 eyes) with Primary Open Angle Glaucoma POAG.

Results Pattern reversal VECP were abnormal in 32/37 eyes (86%): 26/32 (81%) of these being of reduced amplitude, and 20/32 (62%) being delayed. Amongst the patients with POAG only 4/20 eyes (20%) had abnormal VECP, and none were delayed. Thinning of the RNFL occurred in 36/37 eyes (97%) with optic nerve disease; 24 (65%) had global thinning, and the remainder segmental thinning only. All of the eyes with POAG had RNFL thinning but only 6/20 eyes (30%) had global thinning. Bipolar cell thinning of the central retina was noted in 6 eyes with optic nerve disease.

Conclusion OCT is a valuable tool in the assessment of patients with optic nerve disease. Thinning of the RNFL was a more consistent finding than delay of the VECP in optic nerve disease, and a combination of VECP and OCT is helpful in the differential diagnosis of low tension glaucoma and optic nerve disease.
Exploring new strategies to record and analyse clinical electroretinograms
Authors: LACHAPELLE P , GAUVIN M , LAPORTE S , LINA JM , LITTLE JM , RACINE J , DORFMAN A
Published: November 2010

Purpose Investigate if the combination of time‐frequency domain analysis and ERG dipole rotation reveals hidden features of the normal ERG that could be instrumental in the interpretation of nearly extinguished ERG responses.

Methods Analyses were conducted on photopic ERGs (Photopic Hills: PH) obtained from normal subjects (n=75) and patients (n=65) affected with various retinopathies. A Discrete Wavelet Transform (DWT) was done on each ERGs and key descriptors (Holder exponent and wavelet coefficient maxima) were calculated. Dipole rotation was obtained by combining 11 gaze positions (0, 8, 16, 24, 32 and 40 degrees nasal or temporal to center) with 4 electrode locations [corneal (CE), lower lid (LL), external (EC) and internal canthi (IC)].

Results The Holder exponent follows a parabola, while some of the local wavelet maxima seem to follow a PH‐like like distribution (b‐wave and OPs) or a logistic growth function (a‐wave). In still recordable pathological ERGs, the wavelet maxima matched that found in normal ERGs evoked at low stimulus intensities while in nearly extinguished ERGs (<10% of normal) the wavelet coefficients were significantly lower. Irrespective of the direction of gaze, there was little variation in DTL ERGs. EC ERGs were the only ones to reverse in polarity (seen 5 degrees nasal to fixation in nasal to temporal shift).

Conclusion The parameters obtained with the DWT offers useful and reproducible tools to help identify subtle features of residual ERGs and therefore should allow for a more accurate quantification of low‐voltage ERGs responses. Finally, our results suggest that varying the gaze and electrode positions would represent a valuable addition to the recording of clinical ERGs. Funded by NSERC.
The use of the mfERG in the clinic
Authors: PALMOWSKI A
Published: November 2010

Purpose This presentation is to give a brief overview on the application of the multifocal electroretinogram (mf‐ERG) in the clinic.

Methods The use of m‐sequences as a stimulus sequence allows a high resolution topographic mapping of sensory function. The mf‐ERG can be readily applied to detect outer retinal dysfunction in the area of the macula. Different retinal layers contribute to the waveform of the mf‐ERG. This results in typical changes of the waveform, when these different layers are dysfunct.

Results MF‐ERG examples are shown, that demonstrate how this knowledge can be useful in the clinical evaluation of patients.

Conclusion The mf‐ERG is a helpful tool in the clinical evalutation of patients.
Electrophysiology of childhood retinal dystrophies
Authors: BROWN M , CHANDNA A , HAGAN R
Published: November 2010

Purpose To provide a systematic approach to the diagnosis of causes of impaired vision in childhood with particular reference to the role of electrophysiology in retinal dystrophies

Methods We have examined the contribution Electrodiagnostic Testing (EDT) can make in determining or confirming a diagnosis.

Results We have set out a systematic approach to diagnosis of both early and late onset disorders, differentiating between retinal and non‐retinal causes. Signs, symptoms, inheritance patterns and test results are tabulated against specific disorders showing the contribution EDT can make. Recent example case studies are also presented.

Conclusion Electrodiagnostic testing is of particular value in identifying and differentiating causes of poor vision where there may not be clear retinal signs in the early stages such as Leber’s congenital amaurosis, CSNB, cone dystrophy and albinism. A further role is in the differentiation of retinal from post‐retinal causes.
Phenotypic variability in association with mutation in RDS/peripherin
Authors: HOLDER GE , TRAN HV , ROBSON AG , TAN MH , RAMSDEN S , BLACK GCM , WEBSTER AR , MOORE AT
Published: November 2010

Purpose To report the clinical and electrophysiological findings associated with autosomal dominant maculopathy caused by mutations in Rds/peripherin.

Methods Fifty three individuals with autosomal dominant macular dystrophy and a confirmed molecular diagnosis of Rds/peripherin mutation were ascertained between January 2002 and December 2008. International‐standard pattern and full‐field electroretinograms (PERG; ERG) were performed in 38 cases. Electro‐oculograms (EOG) were performed in 25 cases.

Results Fourteen different mutations were identified in the Rds/peripherin gene; 4 of which were novel. Twenty four (45%) patients had the common p.Arg172 Trp allele. The mean age at the time of first symptoms and at diagnosis was 35.0+/‐ 2.4 and 47.1 +/‐ 1.5 years old [SEM] respectively. Mean LogMAR visual acuity at presentation was 0.5+/‐ 0.08 [SEM]. Fundus phenotypes included central atrophy (19 cases), pattern dystrophy (10 cases), maculopathy with flecks (5 cases), and adult vitellifom dystrophy (4 cases). Pattern ERG P50 reduction was seen in 75 of 76 eyes; the majority having undetectable or residual responses, including some cases with preserved visual acuity. ERG ranged from normal to severe reduction in both cone and rod driven responses and were not predictable either from the fundus appearance or from the specific mutation in Rds/peripherin; on addition, marked intra‐familial variation could be noted.

Conclusion Mutations in the Rds/peripherin gene result in a wide variety of fundus and functional phenotypes. The same mutation can result in profoundly different phenotypes, even within family members.
Rituximab for the treatment of refractory ocular inflammation
Authors: JAKOB E , PELLEGRIN L , SCHMIDT‐BACHER A , SCHWENGER V , MAX R , LORENZ H , MACKENSEN F
Published: November 2010

Purpose To report the efficacy of rituximab in the treatment of ocular inflammation refractory to standard immunosuppressive therapy.

Methods We retrospectively reviewed medical records of 9 patients with Wegener Granulomatosis, Behçet disease, Rheumatoid arthritis (RA) and Juvenile idiopathic arthritis (JIA) whose disease was refractory to standard immunosuppressive therapy. We recorded main outcome measures such as changes in best corrected visual acuity, patient’s complaints, intraocular or orbital inflammation, concomitant immunosuppressive therapy. All patients had been treated with at least 3 different immunosuppressive agents and corticosteroids at 1 mg/Kg/d. Rituximab was administered intravenously 1000 mg, in 2 doses, 2 weeks apart, in combination with standard treatment, repeated until ocular inflammation was controlled.

Results 6 patients were affected of Wegener disease (4 orbital granulomatosis, 1 Tolosa‐Hunt syndrome and 1 scleritis), 1 patient had scleritis and intermediate uveitis due to Behçet disease and 2 patients had iridocyclitis (RA and JIA). Over a mean follow‐up of 19 months, patients underwent a mean of 4 infusions. All patients had either stabilization or improvement of ocular inflammation. BCVA improved in 4 patients and was stable in other 4. According to European league of arthritis and rheumatism (EULAR), 8 patients were in remission and one had response to treatment. 8 patients were able to taper the immunosuppressive therapy and corticosteroids <7.5mg.

Conclusion Rituximab was effective and showed a beneficial response to treatment including induction of clinical remission of inflammation in most patients. It may be a rescue treatment in patients who are refractory to standard therapy.

Commercial interest
Pathologic epithelial and anterior corneal nerve morphology in congenital aniridic keratopathy
Authors: FAGERHOLM P , EDEN U , LAGALI N
Published: November 2010

Purpose To document corneal morphology in Swedish families with congenital aniridia.

Methods Detailed ophthalmic examinations were conducted in a number of affected and unaffected members. Digital slit lamp photography, anterior segment optical coherence tomography (ASOCT) and in‐vivo confocal microscopy (IVCM) examinations were performed bilaterally to document corneal morphology.

Results Affected family members presented with different stages of aniridic keratopathy, with a corneal appearance varying from totally transparent to opaque and highly vascularised. Increased corneal thickness in affected members, particularly those with severe keratopathy, was noted by ASOCT. By IVCM, opaque corneas were characterized by active vessels and dense inflammatory cell infiltration. In corneas with milder keratopathy, pathologic epithelial findings included epithelial pleomorphism, focal epithelial opacities, and an absence of limbal epithelial crypts and focal stromal projections at the limbus. Nerves of the anterior cornea exhibited several distinct features, including an unusually close association of subbasal nerves with epithelial cells, an unusually high subbasal nerve density with highly branched nerves, and a prominent whorl region. Additionally, abnormally dense and tortuous anterior stromal nerves, attached to stromal keratocytes, were noted in unaffected members.

Conclusion Altered epithelial morphology and a vigorous innervation of the anterior cornea were the most pronounced corneal findings in family members with milder forms of aniridic keratopathy. Further findings confirmed the known increase in corneal thickness and limbal stem cell abnormality in aniridia.
Dry eye and human tear lipid compositional, conformational and functional relationships using spectroscopy
Authors: BORCHMAN D , FOULKS GN , YAPPERT MC
Published: November 2010

Purpose Knowledge of the relationships among composition, conformation and function of tear film lipids could facilitate the development of therapies to alleviate symptoms related to meibomian gland dysfunction (MGD) and to diagnose the disease. Toward this goal, we used spectroscopic approaches to assess tear lipid composition and conformation relationships with age, sex and meibomian gland dysfunction.

Methods Spectra of meibum from 41 patients diagnosed with MGD (Md) and 27 normal donors (Mn) were acquired.

Results 1H‐NMR spectra showed cholesterol esters were found to decrease by 21% with MGD. The number of double bonds/ester increased with age and MGD which indirectly relates to tear film stability. With age, the amount of CH2 groups increased twice as much as the C=C moieties and the C=C/CH2 and CH3/CH2 ratios were related to lipid order and indirectly related to meibum delivery. With the use of MALDI‐TOF MS, we quantified and identified lipid components in Mn and Md such as cholesterol, hydrocarbons and wax esters with a sensitivity of 9 pmoles for each analyte. Sixty‐nine of the 189 resolved peaks were unique to Md spectra compared to Mn spectra and were not due to waxes. Extra peaks in Md spectra may arise from increased lipid synthesis, bacteria or cellular debris.

Conclusion It is reasonable that as the lipids become more ordered and more viscous with Md, less lipid flows out of the meibomian gland orifice and more casual lipid is present on the lid margin. The age‐ and disease‐related changes in the physical and chemical characteristics of meibum lipids suggest that the C=C/CH2 and CH3/CH2 ratios may be more important than quantity in relation to tear film stability.
Central corneal thickness in North Africa
Authors: LAZREG S , KAMMOUN H
Published: November 2010

Purpose Central corneal thickness (CCT) measure is a critical point before refractive surgery. It is well known that surgery can not be performed under a thickness of 500 microns.

Methods Pentacam pachymetry was performed in refractive surgery centers in Algeria , Tunisia and Morroco on 1615 eyes of patients originating from North Africa.

Results Most of the patients (70%) were aged from 20 to 35 years old.Thirty eight percent(38%) had a CCT between 450 and 500 microns,40% between 500 and 550 microns. Patients with Keratoconus were excluded from this study

Conclusion Publications on this subject being very poor, a study on a larger number of patients has to be conducted in order to confirm the relationship between CCT and ethnic origin.
Matrix metalloproteinase 14 overexpression reduces corneal scarring
Authors: GALIACY S , FOURNIE P , MASSOUDI D , ANCELE E , QUINTYN JC , ERRAUD A , RAYMOND‐LETRON I , ROLLING F , MALECAZE F
Published: November 2010

Purpose Corneal wound healing is an everyday preoccupation for ophthalmologists.Corneal transparency depends on the scarring quality after a traumatic corneal wound, but also after refractive corneal surgery. Cicatrisation and fibrosis formation involve epithelial/fibroblast interactions via paracrin signals inducing extracellular matrix (ECM) remodelling. The major event is fibroblast activation and differentiation into myofibroblasts. These cells have a key role in the fibrotic response. They acquire contractile properties, and synthetise a new ECM, mainly composed of type III collagen. This scar tissue is less organised than the regular stroma, thus explaining corneal opacity. ECM remodelling is a critical step which aims to digest the excess of ECM by proteolysis of type III collagen. MMP14 is a membrane‐bound fibrillar collagenase from the Matrix Metalloprotease family. We hypothesised that its overexpression in the corneal stroma during wound repair will increase ECM remodelling and thus prevent collagen deposition in the scar tissue.

Methods We developed an adeno‐associated virus‐based vector expressing murine MMP14 under the control of the CMV promoter. We evaluated MMP14 overexpression after viral transfection in a murine model of corneal wound healing. We characterised several parameters: clinical observation, histology, and wound healing markers.

Results Our preliminary results showed a decreased in oedema and corneal scar formation, associated with a decreased expression of alpha smooth actin and type III collagen.

Conclusion These results represent proof of concept that gene transfer of MMP14 can reduce scar formation, which could have therapeutic applications after corneal trauma.
Conjunctival infection by Streptomyces spp: clinical case and follow up
Authors: PORTERO A , HERRERAS JM , SECO DE GUZMAN E
Published: November 2010

Purpose Streptomyces is the largest genus of Actinobacteria belonging to Streptomycetaceae family, a group of ubiquitous Gram‐positive bacteria, which are part of the soil saprophyte flora and involved in environmental organic matter decomposition. Over 500 species of Streptomyces bacteria have been described. It is well known its capacity of production of antibiotics, antifungals and immunomodulating drugs (e.g., Streptomycin, Vancomycin, Chloramphenicol, Amphotericin B, Tetracycline, Tacrolimus and Bleomycin).

Methods a 4‐year‐old male who suffered an accidental plant prick on temporal bulbar conjunctiva of the right eye during an Indian trip. He was only diagnosed of a hyposphagma by an ophthalmologist. From that time, it was progressively forming a granulomatous lesion on the affected area. He was treated with several broad‐spectrum antibiotic eye‐drops for about three months without improvement. Hence, it was decided to perform a conjunctival biopsy. The sample was sent to the National Institute of Microbiology to be processed. The bacteriological results concluded that it was a colony of the genus Streptomyces spp. After getting the antibiogram, he started with oral and topic trimetropim‐sulfamethoxazole.

Results Three months after starting that regimen, the lesion completely disappeared. He is currently under regular controls being off of any treatments.

Conclusion Streptomyces spp systemic infection is very rare, and eye involvement, exceptional. In fact there are not cases with conjunctival involvement published in literature. Mostly patients systemically infected by these bacteria present certain degree of compromised immune system. Thus, cases in immunocompetent patients are, at least, exceptional.
The Sanger Mouse Genetics Programme: high throughput characterisation of knockout mice
Authors: GERDIN AK
Published: November 2010

Purpose The Sanger Mouse Genetics Programme (MGP) aims to make a significant impact on our understanding of the function of genes and their role in disease by generating, characterising and archiving in the order of 200 lines of knockout mice per year, including 40 lines as part of the EUMODIC consortium. The phenotyping screens employed include a wide range of assays relevant to key disease areas including diabetes, obesity, hearing and vision disorders, immune disorders, pain and motor function. The data generated by the primary screen will help to further the understanding of the interplay of genes and disease and will provide an insight into the various underlying biological pathways. All phenotyping data and biological resources generated by the programme are openly available to the scientific community.

Methods Eye morphology is routinely assessed using the Slit Lamp and Ophthalmoscope and images are collected when abnormalities are identified. Expression profiling via the lacZ reporter gene is performed for each mutant line in adults and at E14.5.

Results To date, the eye screen has been performed on over 180 mutant lines. Here we report examples of novel eye‐related abnormalities identified by the eye morphology, embryonic lethality and/or expression screens performed by the Sanger MGP. We will present how to identify a potentially interesting mouse mutant on our database and discuss the impact our knock‐out mouse models might have on your research.
The German Mouse Clinic: recent findings from the Eye Screen
Authors: PUK O , FAVOR J , SUN M , GRAW J
Published: November 2010

Purpose The purpose of this study was the large‐scale screening of different mouse mutant lines in order to detect novel models for eye disorders.

Methods The eyes of the mouse mutants were analyzed by slit lamp biomicroscopy, funduscopy, laser interference biometry, optokinetic drum, and histology.

Results In the past 12 months, 46 mouse mutant lines were investigated in the primary Eye Screen of the German Mouse Clinic (GMC). These included Csemp1 and Aey69 that exhibited irregular eye development. All tested mice of the mutant line Csemp1 unexpectedly showed white fundus flecks and significantly reduced axial eye lengths. Moreover, we additionally found strong opacities in a least a portion of the Csemp1 mutant lenses. Aey69 mice are severely microphthalmic due to a yet undefined ENU‐induced mutation. The rudimentary eyes completely lack ocular structures as iris or lens. Further significant irregularities in fat metabolism, immunology, and behaviour were detected in the GMC‐wide primary screen. Linkage studies mapped the mutated site on chromosome 3 within a 0.78 Mb spanning region between the flanking microsatellite markers D3Mit188 and D3Mit76. Among the 34 positional candidate genes, Tnrc4 (elav‐like family member 3) and Selenbp1 (selenium binding protein 1) are expressed in the eye. Sequencing studies in order to detect the causative mutation of Aey69 are in process.

Conclusion Two novel mouse models for microphthalmia were detected in the primary Eye Screen of the GMC. These mutant lines will provide further insights into molecular mechanisms behind this kind of eye disease.
Retinal blood vessel phenotyping in mice
Authors: RUBERTE J
Published: November 2010

Purpose In the retina there is a compromise between optimal visual function and optimal oxygenation. Retinal blood vessels have a relative sparse distribution and their size is small in order to minimise optical interference with the light path. Hence, the blood flow volume in the retina is relatively low. This fact, together with the high oxygen consumption of the retinal tissue, could facilitate the development of retinal hypoxia and subsequent retinopathy when the vascular bed is altered. Thus, the analysis of retinal blood vessel must be a crucial step during retinal phenotyping in mutant mice.

Methods Different technologies and methods have been used in order to analyze structure, distribution and function of retinal blood vessels, among others: retinal digest preparations, retinal whole mount immunohistochemistry, transmission and scanning electron microscopy, fluorescein and Mercox vascular injections and scanner laser ophthalmoscopy.

Results In our laboratory, morphological and topographic alterations of retinal blood vessels in Bmi1 and Sirt1 knockout mice, as well as in IGF‐1 and IL‐10 transgenic mice, have been observed and documented

Conclusion The mouse genome is fully sequenced. 99% of the coding genes present in man are also present in mouse. Moreover, the majority of disease‐related genes have been conserved since the emergence of the bony fishes about 400 million years ago. These facts and the development during the last two decades of an extensive toolbox to study the functional effects of genetic variation in mice, make them the ideal model organism for the study of human eye diseases. In this sense, morphological and functional analyses of retinal blood vessel in mutant mice could help to understand vascular gene‐based mechanisms that lead to retinopathy
Analysis of mouse eye mutants as models for human diseases
Authors: JADEJA S , SANGER MGP , MARY LYON CENTRE MRC , JACKSON I
Published: November 2010

Purpose The Eumodic (European Mouse Disease Clinic) project screens mouse knockout lines and ENU induced mutants for pathological phenotypes. Initially 2 of the strains identified with an eye defect by the Sanger MGP and a strain from the ENU mutagenesis screen at MRC Harwell have been selected for further investigation.

Methods Following the initial primary phenotyping, pathology; histology; and immunohistochemistry was carried out on ocular tissue collected from mutant and control animals to determine defects in eye structure and development. This gave an indication to the underlying cause of the defects seen, enabling further molecular biology analysis.

Results Btb/Poz Domain‐containing Protein 12 (Btbd12) is a scaffold protein required for the formation of DNA repair complexes. The mouse knockout of this gene shows corneal opacity, dilated pupils and occasional microphthalmia, modelling the phenotypes seen in human diseases of defective DNA repair. The corneas of the mutant animals exhibit increased DNA damage which is likely to be the cause of the opacification. Solute Carrier Family 9 Member 8 (Slc9a8) is a Sodium/ Hydrogen exchanger and has previously been shown to play a role in ion exchange. The Slc9a8 knockout strain appears to have retinal degeneration and the males are infertile. The ENU‐induced mutant Pedv128 exhibits defects in the retinal vasculature including defective vascular patterning and increased vascular leakage. Of particular interest is that this vascular phenotype is restricted to the eyes.

Conclusion Investigation of mouse eye mutants can result in a better understanding of the pathology and underlying causes of human diseases.
About 222 cases post cataract acute endophthalmitis: a ten year muticentric retrospective study
Authors: RYSANEK B , BILLOTTE C , DENION E , LECLERCQ R , MILAZZO S , MURAINE M , MOURIAUX F
Published: November 2010

Purpose Acute endophthalmitis is a rare but dramatic event after cataract surgery. The aim of this multicentric retrospective study is to analyse initial signs and management and then to compare our results to the guidelines of Endophthalmitis Vitrectomy Study

Methods We reviewed all cases of endophthalmitis after cataract surgery from 3 french ophthalmological university centers between 1997 to 2007. Only patients with an acute endophthalmitis were recorded. Initial visual acuity and clinical signs, microbiological results, medical or surgical management, ocular complications and final visual acuity were recorded

Results 222 patients were included in this study. The mean age was 73,3 year There was 48,9% men and 51,1% women. Vitreous biopsy or vitrectomy was realised in 117 patients. The choice of surgical treatment was statistically associated with lower initial visual acuity. Intraocular samples were taken in 95% of the patients. In 55,5% of cases, pathogens were identified in cultures. 40% of "aggressive pathogens” like staphylococcus aureus, streptococcus species and bacilles gram‐negative were found. Final visual acuity 12 months after treatment was 20/40 or better in 55,5%. Loss of red reflex at the initial examination was statistically associated with a lower final visual acuity (p<0,001). Positive bacteriological culture was statistically associated with a lower final visual acuity (p<0,001). “Aggressive pathogens” were statistically associated with ocular complications (p<0,001).

Conclusion The EVS guidelines are still valid in our experience.The most predictive criteria for final acuity in our study were the red reflex and presence and type of bacteria in acute post operative endophthalmitis
Epidemiology of uveitis in the Middle East and North Africa
Authors: KHAIRALLAH M , GARGOURI S
Published: November 2010

Purpose Numerous studies have examined the pattern of uveitis around the world. Most of them are from western countries, including the USA and countries in Europe, and Eastern Asia. The aim of this presentation is to review the epidemiological characteristics of uveitis in the the Middle East and North Africa.

Methods The epidemiologic data on uveitis available from the Middle East and North Africa were reviewed.

Results Several recent studies addressed the pattern of uveitis in different countries, including Iran, Saudi Arabia, Turkey, and Tunisia. Uveitis was most often seen in adults with a peak age at presentation in the third and fourth decades. There was no dramatic difference in gender distribution. Anterior uveitis was the most common anatomic form of uveitis, but a high rate of posterior uveitis and panuveitis was reported. A definitive or presumed specific diagnosis could be established for 57‐87% of patients. The most common infectious entities were herpetic anterior uveitis, toxoplasmosis, and tuberculosis (Saudi Arabia). The most common identifiable non‐infectious entities included Behçet’s disease and Vogt‐Koyanagi‐Harada disease.

Conclusion Herpetic infection, toxoplasmosis, and tuberculosis are the most common infectious causes of uveitis in the Middle East and North Africa. Behçet’s diease and Vogt‐Koyanagi‐Harada disease are the most common non‐infectious uveitic entities.HLA‐B27 acute anterior uveitis, ocular sarcoidosis, and juvenile idiopathic arthritis associated uveitis are less common than in western countries.
Vogt‐Koyanagi‐Harada disease
Authors: ABU EL ASRAR A
Published: November 2010

Abstract not provided
Fuchs’ uveitis in the Middle East
Authors: SOHEILIAN M
Published: November 2010

Abstract not provided
Inflammatory mediators in the aqueous humor from patients with uveitis associated with Behçet’s disease and Vogt‐Koyanagi‐Harada disease
Authors: ABU EL ASRAR A , DESCAMPS F , KANGAVE D , CAUWE B , MARTENS E , GEBOES K , OPDENAKKER G
Published: November 2010

Purpose We studied interphotoreceptor retinoid‐binding protein (IRBP), a dominant autoimmune antigen in the eye.

Methods Aqueous humour samples from 28 patients with active uveitis were analysed for immunoglobulin G (IgG) content as a marker for blood‐ocular barrier breakdown and by gelatinase B zymography for the detection of inflammation. The data were correlated with the presence of intact IRBP (approximately 140 kD) as determined by Western blot analysis and with the clinical disease activity.

Results Aqueous humour samples from control eyes and eyes with low disease activity showed positive immunoreactivity for intact IRBP. The IRBP signal weakened or disappeared with higher disease activity. Significant positive correlations were observed between disease activity and levels of gelatinase B/matrix metalloproteinase‐9 (MMP‐9) (rs=0.713; P<0.001) and IgG (rs=0.580; P=0.001). Significant negative correlations were found between levels of IRBP and disease activity (rs=‐0.520; P=0.005) and levels of MMP‐9 (rs=‐0.727; P<0.001) and of IgG (rs=‐0.834; P<0.001). Whereas neutrophil elastase converted intact IRBP into an immunoreactive 55 kD peptide in vitro, the conversion by neutrophil degranulates resembled more the in vivo context with a complete degradation of IRBP. Reversal of inflammation with immunosuppressive therapy was accompanied with reappearance of intact IRBP and disappearance of IgG and MMP‐9.

Conclusion The analysis of IRBP proteolysis is useful as a biomarker for uveitis and suggests that inhibition of proteinases might become a therapeutic strategy in an inflammatory context of a damaged blood‐ocular barrier.
Efficacy and tolerance of systemic ganciclovir as a treatment for cytomegalovirus‐associated anterior uveitis in inmunocompetent patients
Authors: ESPOSITO F , BODAGHI B
Published: November 2010

Purpose To assess the utility of systemic, intravitreal or topical ganciclovir, as a treatment for PCR‐proven cytomegalovirus (CMV)‐associated anterior uveitis.

Methods Retrospective interventional case series of 39 consecutive patients treated in a single centre between 2002 and 2009. Main outcome measures included intraocular pressure (IOP) and anterior chamber inflammation.

Results All 35 patients with at least a 12‐month follow‐up responded to the first treatment regimen. Mean IOP dropped from 32 (SD 7) to 18 (SD 2) mmHg after 1 week and remained within normal values throughout the treatment course, with progressive lowering of antihypertensive therapy. Relapses occurred in 50% of cases within one year. Mean time to relapse was 5.7 months (range: 0.5‐12). Long‐term antivirals were needed in 15% of cases whereas intolerance occurred in 15% of cases. Intravitreal ganciclovir may be proposed in patients with systemic intolerance but multiple injections are required. Long‐term topical ganciclovir was able to control the disease in selected cases.

Conclusion Systemic Ganciclovir is effective in controlling CMV‐induced chronic ocular hypertension and anterior chamber inflammation, but even prolonged treatment does not protect from relapses. Repeated short‐course regimens seem to be an appropriate strategy.
Bevacizumab induces intraocular tumour growth in mice: an intriguing paradox
Authors: VERSLUIS M , EL FILALI M , LY LV , LUYTEN GPM , VAN DER VELDEN PA , JAGER MJ
Published: November 2010

Purpose Intravitreal bevacizumab, a humanized monoclonal antibody to VEGF‐A, was originally developed as an anti‐tumour treatment, and is used to treat macular edema in radiation retinopathy. As this drug may potentially be used to treat primary uveal melanoma (UM) and/or its metastases, we analyzed the effect of bevacizumab on UM growth.

Methods Mice inoculated with B16F10 melanoma cells into the anterior chamber of the eye were monitored for tumour growth and vascularisation after bevacizumab treatment. UM cell proliferation and the expression of VEGF‐A, GLUT‐1, and HIF‐1α were analyzed in vitro.

Results Bevacizumab treatment resulted in an acceleration of intraocular tumour growth in mice, but did not induce UM cell proliferation in vitro. Incubation with bevacizumab induced VEGF‐A and GLUT‐1 mRNA expression via the HIF‐1α pathway in UM cell lines and primary cell cultures. Surprisingly, VEGF‐A expression in mice did not result in more vessels, but we did perceive more anterior chamber hemorrhages in vivo.

Conclusion Anti‐VEGF treatment with bevacizumab induces intraocular tumour growth in mice. Although bevacizumab did not induce the number of vessels in treated mice, we did observe more anterior chamber hemorrhages. One of the mechanism involved, is the induction of VEGF‐A expression in hypoxic UM cell lines and cultures through the HIF‐1α pathway, resulting in a ‘pseudohypoxic’ condition. This phenomenon has been described in other tumours and maybe the consequence of tumour adaptive or evasive resistance. The use of bevacizumab for treatment of macular edema (due to radiation retinopathy) after irradiation of UM should therefore be carefully considered.
Epistaxis or epiphora as sign for extension of a conjunctival melanoma: a series of six patients with nasolacrimal recurrence
Authors: MISSOTTEN GS , GAMBRELLE J , DE KEIZER RJW
Published: November 2010

Purpose To characterize malignant conjunctival melanomas with extension and recurrence in the nasolacrimal system

Methods Localization of the primary tumor and recurrences of 210 conjunctival melanomas treated in the Netherlands were reviewed for orbital and nasal tumors (1978‐2008). On the basis of these cases and literature data, characteristics for nasolacrimal system extension and metastasis were reviewed.

Results Six patients (3%) showed a recurrence of the primary conjunctival melanoma in the nasolacrimal system. Two of the six primary tumors were limbal tumors, the other four were diffuse tumors involving the fornix. In all six patients, the primary conjunctival melanomas were associated with primary acquired melanosis (PAM). During follow‐up period (mean 11.6 ± 3 years; median 8.7 years) two patients developed metastases, and died during follow‐up.

Conclusion Patients should be advised to contact the treating ophthalmologist in the case of symptoms of epiphora, nose obstructions and epistaxis, especially non‐bulbar and diffuse cases associated with PAM.
Trop 1 gene is overexpressed in pterygia
Authors: CALIENNO R , CURCIO C , LANZINI M , LA SORDA R , PIANTELLI M , COLASANTE M , NUBILE M
Published: November 2010

Purpose Understanding molecular and biochemical events of pterygium may allow to use less invasive treatments. Several antigens have been investigated to study the physiopathology of this disease. TROP1 protein, a cell adhesion regulatory molecule, and TROP2, a cell surface calcium signal trasducer, are expressed by a large variety of human cancers.

Methods The aim of this work is to evaluate TROP1 and 2 protein expression in primitive and recurrent of pterygia. The expression of these antigens was evaluated in limbal crypts (4 cases), corneal epithelium (2 cases), conjunctival melanoma (1 case), normal conjuntiva (4 cases) and pterygia(8 primitive and 4 recurrent). All samples were fixed in formalin, embedded in paraffin and stained by immunohistochemistry. The different expression of TROP1 and 2 was evaluated and correlated with markers normally expressed in pterygia,such as vimentin, ki67, survivin, MMP7, p63, cyclin D1 and p53.

Results No expression of TROP1 was observed in corneal epithelium and in limbal crypt, few positive cells were observed in healthy conjuntival epithelium. TROP2 was observed in some cells of limbal crypt, but was not expressed in corneal epithelium, while was positive in conjuntival epithelium. A strong TROP1 and 2 expression was detected in primary pterygia. TROP 1 and 2 positivity correlated with expression of vimentin, ki67, survivin, MMP7, p63, cyclin D1 and p53. Interestingly TROP1 positivity decreased in recurrent pterygia.

Conclusion TROP1 and TROP2 antigens can be use as marker also for ocular surface.
Malignant lymphoma of the lacrimal gland in Denmark
Authors: RASMUSSEN P , PRAUSE JU , RALFKIAER E , SJÖ LD , HEEGAARD S
Published: November 2010

Purpose To characterise clinicopathological features of lacrimal gland lymphoma.

Methods All cases of lacrimal gland lymphoma were identified searching The Danish Registry of Pathology covering the years 1975‐2009. Histological specimens were re‐evaluated with a panel of monoclonal antibodies. All patients with confirmed lymphoma had their clinical files collected.

Results Twenty‐seven patients with malignant lymphoma of the lacrimal gland were identified. Eight of the patients were males, nineteen were females, and the median age was 69 years (range 43 to 87 years). Cases were distributed as extranodal marginal zone B‐cell lymphoma 33% (9/27), follicular lymphoma 19% (5), diffuse large B‐cell lymphoma 15% (4), mantle cell lymphoma 15% (4), small lymphocytic lymphoma 7% (2) and unclassified B‐cell lymphoma 11% (3). Mass of the lacrimal gland (85%), proptosis (56%) and displacement of the globe (41%) were the most frequent clinical signs. Twenty‐two cases (81%) presented in stage I/II. One case (4%) had stage III disease and four cases (15%) presented in stage IV. Of the five cases in stage III/IV, four had lacrimal gland lymphoma as first presenting symptom. Patients in stage I/II were treated with: Radiotherapy (71%), chemotherapy (19%) and surgery (14%). Patients having widespread disease were all treated with chemotherapy alone. Complete remission was experienced by 85% of the cases but 43% of these had a relapse independent of stage, treatment and subtype. The 5‐year overall survival was 79%.

Conclusion Malignant lymphoma of the lacrimal gland is relatively rare and presents in elderly females. The distribution of subtypes of lacrimal gland lymphoma resembles the distribution of lymphoma subtypes seen in the salivary glands.
Ocular adnexal lymphoma and Helicobacter pylori gastric infection
Authors: DECAUDIN D , FERRONI A , VINCENT‐SALOMON A , VALIDIRE P , DE CREMOUX P , VALIDIRE P , PLANCHER C , ASSELAIN B , GIRODET J , MAL F , BROUSSE N , DENDALE R , LUMBROSO‐LE ROUIC L , HERMINE O , LECUIT M
Published: November 2010

Purpose There is a causal association between Helicobacter pylori (Hp) gastric infection and the development of gastric MALT lymphoma. In contrast, the link between Hp gastric infection and the development of extra‐gastric lymphoma has not been thoroughly investigated. We therefore studied the prevalence of gastric Hp infection at initial diagnosis of ophthalmologic and non‐ophthalmologic extra‐gastric lymphoma patients.

Methods Three cohorts of patients were studied: a first one of 83 patients with OAL, a second one of 101 patients with extra‐ophthalmologic extra‐gastric lymphoma, and a third one of 156 control individuals (control) without malignant lymphoma. Gastric Hp infection was investigated by histopathological analysis and Hp‐specific PCR assay on gastric biopsy tissue samples.

Results We found gastric Hp infection in 37 OAL patients (45%), in 25 extra‐ophthalmologic extra‐gastric lymphoma cases (25%), and in 18 controls individuals (12%) (p<0.0001 OAL/C and p<0.01 OAL/extra‐OAL cases). Gastritis was found in 51% and 9% of Hp‐positive and Hp‐negative lymphoma patients, respectively (p<10‐4). Gastric Hp infection only correlated with MALT/LPL lymphoma (p = 0.03).

Conclusion There is a significant association between gastric Hp infection and MALT/LPL OAL. This suggests a novel mechanism of indirect infection‐associated lymphomagenesis whereby chronic local antigen stimulation would lead to the emergence of ectopic B‐cell lymphoma.
Two cases of mantle cell lymphoma
Authors: BRISCOE D , KIDRON D , ALMALACH I
Published: November 2010

Purpose To describe the presentation of two cases of Mantle Cell Lymphoma in the Orbit

Methods Two consecutive cases of Mantle Cell Lymphoma presemted to the Orbital Clinics of Meir Medical Center and Emek Medical Center, between 2009 and 2010. Both cases underwent biopsy and a full systemic workup.

Results Both cases presented with a rapidly progressive ptosis and an anterior orbital mass. One case had primary presentation of the disease in the orbit and the other a secondary presentation on an already known systemic disease. Diagnosis was based on histopathology and bone marrow aspirates.

Conclusion Mantle Cell Lymphoma is an aggressive orbital disease and may present with a rapidly progressive ptosis.
Orbital schwannomas in Pakistan
Authors: KIANI SA
Published: November 2010

Purpose To highlight the frequency and types of origin of Orbital Schwannomas

Methods Medical Record of all the patients with orbital tumours presenting between 2000‐2009 was analysed and records showing the histopathological diagnosis of Schwannoma were identified and studied further for type and origin of Schwannomas.

Results A total of 432 patients with orbital tumours were found during last 10 years. Out of these, 10 patients were found to have Schwannomas (2.31%). Age ranged from 12 years to 50 years, while there was equal gender ratio. The commonest site of origin was roof of orbit. Six patients had origin from roof, three from intraconal region and one from floor of the orbit. Out of the tumours from roof, 5 took origin from supraorbital nerve and one from trochlear nerve involving the superior oblique muscle. The three intraconal tumours were most likely from ciliary nerves, while the one from floor was from infraorbital nerve. All the tumours except one was removed in toto. The only one where incision biopsy was taken was very close to optic nerve. Histopathologically, there were three rare variants ‐ ancient schwannomas, while the rest were classical schwannomas. There was no association of optic nerve tumours. Visual acuity was not disturbed by any of these.

Conclusion Orbital Schwannomas constitute 2.31% of orbital tumours in Pakistan. The commonest origin is roof of orbit and ancient schwannomas constitute a rare variant.
Frosted branch angiitis: clinical case and follow‐up
Authors: PORTERO A , HERRERAS JM , LOPEZ MI , CARRENO E , GARCIA C , SALA A , VALVERDE C , VICENTE TIERNO N
Published: November 2010

Purpose Frosted branch angiitis (FBA) is a disorder characterized by acute panuveitis with vasculitis. It is a rare disorder (just 60 cases worldwide published) which is slightly more frequent in males especially in the third decade of life.

Methods a 28 year‐old female was admitted due to suspected meningitis associated to visual loss in both eyes (OU). One week before, she was diagnosed of bacterial tonsillitis and got several antibiotic drugs. The admitted patient started empirical treatment with: Ceftriaxone IVand Acyclovir IV. Lab test, serologies and CT scan did not disclose any abnormality. Latex agglutination test for Neisseria meningitidis was negative, however intracellular lised bacterias were seen through the microscope informed like compatible with that agent. The patient also presented decreased visual acuities(VA): right eye (OD) 20/200 and left eye (OS) 20/120 and panuveitis in OU. Funduscopically it was seen a frosted branch angiitis in OU. Due to her antibiotic and viral therapy, Prednisone 1mg/Kg PO was added. Three weeks after, the patient left the Hospital on Famciclovir 750mg QID, Ciclosporine 300mg daily and tapering Prednisone.

Results One year later, the patient has 20/20 of VA in OU and is off of any treatment.

Conclusion FBA may disclose three conditions: an infiltrating vessels by malignant cells, a sheathing vessels secondary to an active disorder or subsequently to a previous inflammation disease. This case may be included within that last term due to an aseptic meningitis by N. meningococcus. Recognition of what kind of FBA clinical sign is will be essential to establish one certain diagnosis and the subsequently treatment.
Prevalence of and risk factors for conjunctival bacterial colonisation in adult patients undergoing elective intraocular surgery
Authors: HALACHMI‐EYAL O , LANG Y , KENESS Y , BRISCOE D , MIRON D
Published: November 2010

Purpose Bacterial colonization in the conjunctiva at the time of ocular surgery may be associated with post operative endophthalmitis. The aims of this study were to assess the risk factors for, and prevalence of bacterial colonization in the conjunctiva of adults undergoing elective intraocular surgery

Methods A prospective study was conducted in the Ophthalmology Department at the ‘Emek’ Medical Center, between May 1,2006 and August 31,2007. Conjunctival cultures were obtained from the lower fornix, prior to the application of prophylactic treatment and were processed using routine techniques. Demographic, socioeconomic and medical data of our patient cohort were obtained from all participants

Results Cultures were obtained from 501 patients (mean age 69.7 years), of whom bacteria grew in 203(40.5%). Coagulase Negative Staphylococcus (CNS) was the most prevalent bacteria isolated in 170 (34%) of the patients, followed by Streptococcus viridans and methicillin sensitive Stapylococcus aureus (2.8% each). In 175(35%) of the patients one type of bacteria was detected whereas more than one bacteria were detected in 28(5.5%). Independent risk factors for any bacterial growth were showering in the operating day (OR=1.70) and spring/summer seasons (OR=1.54). Preoperative showering (OR=1.76), spring/summer seasons (OR=1.55) and lack of formal education (OR=1.62), were significant independent risk factors for mixed‐type of bacterial growth in culture

Conclusion Bacterial growth in culture was detected in 40% of the patients of which CNS was the most prevalent bacteria. Showering and warm weather at the operating day were significant risk factors associated with one or more types of bacterial growth.
A case of bilateral idiopathic retinitis, vasculitis, aneurysms and neuroretinitis (IRVAN) treated with discontinuous immunosuppressive therapy
Authors: GONZALES S , RAMPINI A , RUBINO P , MORA P , ORSONI JG
Published: November 2010

Purpose To describe a case of bilateral Idiopathic Retinitis,Vasculitis,Aneurysms and Neuroretinitis(IRVAN)successfully treated with systemic immunomodulatory treatment including steroid, cyclosporine and methotrexate.

Methods A 47‐year‐old Italian woman was diagnosed with IRVAN at our Service.She underwent extensive general and laboratory evaluation.Periodical ophthalmologic evaluations included best correct visual acuity(BCVA)assessment,slit lamp and fundus examination.Ocular alterations were documented by fluorescein and green indocyanine angiogram,and optical coherence tomography.

Results At her first examination BCVA was 20/32 in the right eye(RE)and 20/40 in the left eye(LE).In both eyes angiography showed retinal vasculitis with arterial aneurysms and moderate ischemia,mild papillitis and macular oedema.The patient started a systemic steroid spearing immunomodulatory treatment.After 12 months of follow‐up BCVA was 20/25 in the RE and 20/20 in the LE.Imaging showed a complete resolution of the retinal and optic nerve head active inflammation in both eyes,with significant reduction of macular oedema.

Conclusion IRVAN is an isolated retinal vascular disease that can progress to severe visual loss due to ischemic sequelae.Ocular inflammation is likely related to immunitary disregulation,even if the aetiology is still not clear.In the presented case the bilateral involvement with papillitis and the progression of the visual loss in spite of previous local and systemic steroid regimen, were the reasons that convinced us to propose a systemic immunomodulatory treatment.This treatment was well tolerated, with a complete resolution after one year of follow‐up.