bluuebunny/crossref_metadata_2025_split huggingface dataset:
High‐dose chemotherapy with autologus hematopoeitic stem cell transplantation in relapsing Vitreoretinal Lymphoma, a LOC network study
Authors: Bennedjai A. , Houiller C. , Choquet S. , Cassoux N. , Guesquières H. , Marolleau J.P. , Chabrot C. , Jdid I. , Lejeune C. , Bodaghi B. , Le Hoang P. , Hoang‐Xuan K. , Soussain C. , Touitou V. 
Published: September 2017

PurposeThe management of isolated vitreoretinal lymphoma (VRL) remains controversial due to his rarity. The aim of this study was to evaluate the efficiency and safety of high‐dose chemotherapy with autologous hematopoietic stem cell transplantation (HCT‐ASCT) as a treatment of recurrent relapsing VRL.MethodsWe retrospectively studied medical records of patients included in the French LOC network database between 2011 and 2016 with isolated vitreoretinal relapse of either primary vitreoretinal lymphoma or oculocerebral lymphoma treated with HCT‐ASCT. Hematologic, neurologic and ophtalmologic datas were screened.Results32 patients (16 F/16 M), all immunocompetent, were included in the study. Median age at HCT‐ASCT was 62 (range 44–73 years). Median Karnofsky Performance status before HCT‐ASCT was 90. At diagnosis, 17/32 had primary vitreoretinous lymphoma (PVRL), 7/32 Primary Central Nervous Cells Lymphoma (PCNCL), 8/32 PVRL and PCNL. Patients had previously received a median number of 1 line of treatment including high‐dose methotrexate before HCT‐ASCT. All patients received a thiotepa‐based HCT. 81% of the patients experienced a complete response. 2 “toxic deaths” occurred. Mean follow‐up was 41.6 months (range: 5–96). 9/32 patients had recurrences. The 5‐years OS was 81%. The 5‐years OS was respectively 91% in the PRVRL groups, 85% in ocular and cerebral group and 64% in PCNCL group. The 5‐years PFS rate was 58%. The 5‐years PFS was respectively 66% in the PRVRL groups, 75% in ocular and cerebral group and 43% in PCNCL group. 4 deaths occurred overall.ConclusionsIntensive chemotherapy followed by autologous stem cell transplant is an aggressive therapeutic approach but appears to give interesting results especially in young and fit patients with recurrent refractory vitreoretinal lymphoma.
Differential hypoxic response of human choroidal and retinal endothelial cells proposes tissue heterogeneity of ocular angiogenesis
Authors: Andre H. , Mammadzada P. , Gudmundsson J. , Kvanta A. 
Published: September 2017

PurposeTo elaborate molecular differences between choroidal and retinal angiogenesis by generating and comparatively analyzing human primary choroidal and retinal endothelial cell (CEC and REC) lines.MethodsHuman CEC and REC were isolated by positive selection and cultured. Characterization was performed by immunostaining for endothelial cell (EC)‐specific markers. Total RNA and protein were extracted from normoxic or hypoxic CEC and REC cultures. qPCR arrays were used to comparatively analyze CEC and REC, and expression differences were calculated by ΔΔCt method. Angiogenesis‐related protein expression differences were investigated by western blot and proteome profiler, and were calculated by densitometry. Functional responses to hypoxia were analyzed by sprouting assay.ResultsPrimary human CEC and REC lines stained positively for all EC markers and demonstrated high purity with similar staining and morphology. Under normoxia, CEC showed significantly lower expression levels for cell proliferation and vessel maturation genes and higher expression levels for inflammation‐related genes when compared to REC. In response to hypoxia, CEC and REC displayed differential regulation for a multitude of angiogenesis‐related genes and proteins. Furthermore, within the vascular endothelial growth factor (VEGF) family, CEC showed preferential upregulation for vascular endothelial growth factor A (VEGFA) while REC upregulated placenta growth factor (PlGF) levels. Sprouting was observed in normoxia, and was significantly by hypoxia.ConclusionsDifferential normoxic and hypoxic regulation of angiogenesis‐related factors by CEC and REC outlines tissue heterogeneity of ocular angiogenesis and suggests that tissue specificity should be considered as a novel treatment modality for successfully overcoming choroidal and retinal angiogenic conditions in the clinic.
Thickness mapping of individual retinal layers and sectors by Spectralis SD‐OCT in Autosomal Dominant Optic Atrophy
Authors: Corajevic N. , Larsen M. , Rönnbäck C. 
Published: September 2017

PurposeTo assess layer‐ and location‐specific retinal thickness deficits in autosomal dominant optic atrophy (ADOA) on Spectralis SD‐OCT.MethodsThis cross‐sectional study included 41 ADOA patients with OPA1 exon 28 (2826delT) mutation (age, 8.6‐83.5 years; best‐corrected visual acuity (BCVA), 8‐89 ETDRS letters) and 55 mutation‐free first‐degree healthy control relatives (age, 8.9‐68.7 years; BCVA, 80‐99 ETRDS letters). Participants underwent routine examination and OCT with thickness analysis of the total retina, inner retinal layers and outer retinal layers. Individual segmentation was performed of the perifoveal retinal nervefiber layer (RNFL), ganglion cell layer (GCL), inner plexiform layer (IPL), inner nuclear layer, outer plexiform layer, outer nuclear layer and peripapillary RNFL. Segmentation lines were moved manually to fit optimally with the borders between the layers. Combinations of layers and sectors were tested for their diagnostic significance. Statistical analysis was corrected for age, gender, spherical equivalent, axial length and family clustering in a mixed model analysis.ResultsThe perifoveal RNFL, GCL, IPL and the peripapillary RNFL were all significantly thinner in ADOA patients than in healthy controls (p < 0.0001). No statistical difference was found for other layers. The largest deficit was found in the GCL (‐49.9%), and it reached its maximum in the “nasal inner macula” sector with a thickness deficit of ‐63%. Attenuation of the peripapillary RNFL was most prominent temporally to the optic disc (‐58.4%).ConclusionsIn ADOA, retinal ganglion cells are most prominently missing in the nasal perifoveal area of the GCL, which together with the temporal peripapillary RNFL area serves as the strongest diagnostic OCT marker of ADOA.
Swept‐Source OCTutilised to compare the choroidal thickness of the peripapillary area between patients with Parkinson′s disease and healthy subjects
Authors: Obis J. , Garcia‐Martin E. , Satue M. , Rodrigo M.J. , Cipres M. , Vilades E. , Orduna E. 
Published: September 2017

PurposeTo evaluate and compare peripapillary choroidal thickness (PPCT) in patients with Parkinson′s disease (PD) and healthy subjects using swept‐source optical coherence tomography (SS‐OCT)Methods80 healthy subjects and 40 PD patients were examined using Deep Range Imaging (DRI) OCT Triton. An optic disc 6.0 × 6.0 mm three‐dimensional scan was obtained to measure choroidal thickness. Five concentrical choroidal zones were established and used to compare PPCT between PD patients and healthy subjects: zone 1 corresponded to the optic disc area (thus not measured by the OCT), zones 2 to 5 were progressively farther from the optic discResultsPPCT was significantly thicker in PD patients compared to healthy subjects in all the zones (p ≤ 0.0001): 126.89 ± 11.23 μm vs 96.53 ± 9.55 μm in zone 2; 152.75 ± 16.00 vs 121.39 ± 12.10 μm in zone 3; 190.21 ± 12.16 vs 156.69 ± 12.44 μm in zone 4; and 213.21 ± 11.83 vs 186.64 ± 9.47 μm in zone 5. A similar pattern of choroidal thickness was observed both in PD patients and healthy subjects; it was thicker in temporosuperior region, followed by superior, temporal, nasal and inferior regionsConclusionsPD patients show an increased peripapillary choroidal thickness compared to healthy subjects in all zones. Peripapillary choroidal thickness shows a concentrical pattern both in PD patients and healthy subjects, with increasing thickness moving away from the optic disc. The new SS‐OCT could be useful to evaluate choroidal thickness, and it could be useful in clinical practice.
Choroidal changes in patients with multiple sclerosis
Authors: Rodrigo M.J. , Garcia‐Martin E. , Orduna E. , Obis J. , Cipres M. , Vilades E. , Satue M. 
Published: September 2017

PurposeTo evaluate the peripapillar choroidal thickness in patients with multiple sclerosis (MS) and to compare it with age‐ and sex‐ matched healthy controls, using a swept‐source optic coherence tomography (SS‐OCT).MethodsFifty‐one right eyes of patients with MS and 102 right eyes of healthy controls were analyzed. A 6X6 mm choroidal cube was scanned using the SS‐OCT Triton device. This OCT showed 676 measurements that were later divided in four zones, from lower to higher choroidal thickness: the zone 1 corresponds to the optic nerve head (no choroidal measurements are given), the zone 2 corresponds to choroidal thickness between 120 and 179 microns, the zone 3 between 180 and 239 microns, and finally the zone 4 corresponds to a thickness higher to 240 microns.ResultsA similar concentric choroidal thickness pattern was observed in MS patients and healthy controls. The choroidal thickness was thicker in the superior region, followed by the temporal, nasal and inferior regions. The farther from the optic nerve, the thicker choroidal tissue was quantified. However, a statistically significant decreased of the peripapillary choroidal thickness in all the concentric zones was observed in patients with MS compared to healthy controls (134.02 ± 16.59 μm in MS group vs 171.56 ± 12.43 μm in the control group in zone 2; 182.23 ± 20.52 vs 219.03 ± 17.99 μm, respectively, in zone 3; and 223.52 ± 10.70 vs 259.99 ± 10.29 μm, respectively, in zone 4; p < 0.0001).ConclusionsPatients with MS showed thinner peripapillar choroidal thickness compared to healthy controls. The SS‐OCT device could be useful equipment in clinical practice to evaluate the peripapillar choroidal tissue in such patients.
Comparative investigation of compression optic neuropathy of Grvaves orbitopahty (GO) as emergency orbital decompression
Authors: Tagami M. , Kusuhara S. , Azumi A. 
Published: September 2017

PurposeTo investigate pre‐operative clinical feature of Compression optic neuropathy of Grvaves orbitopahty(GO), which had necessities of emergency surgery as orbital decompression.DesignRetrospective,observational,case‐control study.MethodsSetting single institution. Patient population: Sevanty‐two patients were performed orbital decompression as treatment of proptosis and compression optic neuropathy with GO. Observational procedures: the following were considered potential pre‐operative clinical factors for emergency orbital decompression: patients's sex, patients's age, Disease duration, laterality of GO, laterality of surgery, treatment history of resistance of steroid pulse therapy, thyroid function, visual acuity, existence of central diplopia, asymmetry of proptosis, psychological history,smoking. Main outcome: potential pre‐operative clinical factors for emergency orbital decompressionResultsWomen,high age, long Disease duration, laterality of GO, laterality of surgery, treatment history of resistance of steroid pulse therapy, unstable thyroid function, poor visual acuity, existence of centraldiplopia significantly influenced necessities of emergency surgery as orbital decompression(p < .050). The other itmes did not significantly influence necessities of emergency surgery as orbital decompression(p > .050)ConclusionsThis study revealed potential pre‐operative clinical factors of Compression optic neuropathy of Grvaves orbitopahty(GO), which had necessities of emergency surgery as orbital decompression. This results will be helpful and deeply meaningful for physician, managing GO, planning additional surgical options
Clinical features of strabismus and nystagmus in bilateral congenital cataract
Authors: Lee S.J. , Sung Soo H. , Jung Min P. 
Published: September 2017

PurposeThe prevalence and clinical features of strabismus and nystagmus, and the factors affecting onset of strabismus and nystagmus were evaluated in the patients with bilateral congenital cataract.Methods58 patients, 116 eyes who had lens removal for the treatment of bilateral congenital cataract between January 1999 and January 2011 were evaluated. According to the preoperative and postoperative ocular alignment, patients were divided into 3 groups (Orthotropia/Orthotropia, Orthotropia/Strabismus, and Strabismus/Strabismus). Age at cataract surgery, and associations of nystagmus and primary intraocular lens (IOL) implantation with strabismus were analyzed.Results6 patients (10.3%) had strabismus preoperatively and 11 patients (19.0%) developed postoperative strabismus. Thus, total 17 patients (29.3%) had strabismus postoperatively. Exotropia was more common than esotropia both preoperatively and postoperatively. 18 patients (31.0%) had nystagmus postoperatively and sensory nystagmus was the most common type. Of the 18 patients with nystagmus, 10 patients had strabismus and exotropia was more common than esotropia. When there was nystagmus, postoperative visual acuity was poor. In the ortho/strabismus group, age at cataract sugery was significantly younger and postoperative nystagmus was more common and the rate of no primaryIOLimplantation was higher than the other groups.ConclusionsIn patients with bilateral congenital cataracts, exotropia and sensory nystagmus may be common. Age at surgery was earlier, the rate of noIOLimplantation was higher and nystagmus was more common in postoperative strabismus onset group. And if there is nystagmus, poor visual prognosis may be seen. Therefore, careful observation should be needed in these patients.
Treatment with prism glasses for overcorrection after surgery for exotropia in children
Authors: Chang H.R. 
Published: September 2017

PurposeTo determine the effect of prism glasses and factors affecting treatment in children wearing prism glasses for overcorrection after surgery for exotropia.MethodsThe medical records of 43 patients who wore prism glasses for overcorrection after exotropia surgery and were followed up more than 1 year were reviewed retrospectively. Patients with overcorrection after surgery for exotropia were treated with occlusion of one eye and correction of hyperopia, and the prism glasses were prescribed if esotropia persists. The strength of prism glasses was adjusted based on alignment. We evaluated the effect of prism glasses according to the starting age of prism glasses, type of surgery, refractive error, occlusion after wearing prism glasses and overcorrection of the strength of prism glasses.ResultsTwenty one patients (48.8%) took off prism glasses. Among 22 patients who still wear prism glasses, the strength of prism was decreasing in 10 patients, maintained in 8 patients and increased in 4 patients. Only 1 patient underwent surgery for consecutive esotropia. In patients who began to wear prism glasses under the age of 10, the rate of took off prism glasses or the rate of decreased in the strength of prism was higher than in patients who began to wear prism glasses aged 10 or older (p=0.007). All patients who underwent unilateral recession and resection took off prism glasses or decreased the strength of prism glasses (p=0.026).ConclusionsAmong 43 patients who needed prism glasses to treat overcorrection after exotropia surgery, 72% took off prism glasses or decreased the strength of prism glasses. Only 1 patient needed second surgery for consecutive esotropia. Treatment with prism glasses for overcorrection after exotropia surgery is very useful and can lower the frequency of second surgery.
Redistribution within retinal layers of the central fovea in preterms with developmental arrest
Authors: Sjöstrand J. , Popovic Z. 
Published: September 2017

PurposeThe aim of this study was to characterize thickness changes of foveal layers in preterms with or without signs of immaturity compared to control subjects through analysis of optical coherence tomography (OCT) B‐scan images.MethodsSelected eyes from 8 young adults with a history of prematurity (24‐33 weeks of gestation) and 5 controls were imaged using conventional and directional OCT. Retinal layer thickness analysis was performed at selected temporal eccentricities defined by the individual distance between two landmarks for each case, the foveal center (FC) and the foveal rim (R).ResultsThe use of an FC/R landmark transformation enabled comparisons of thickness profiles and area calculations from inter‐individual B‐scans at corresponding landmark positions in both controls and preterms. Reflectometric and manual segmentation measurements showed increased thickness of inner retinal layers (IRL) and photoreceptor cell body and axon/pedicle layers within the central fovea. Thickness and areal differences between controls and preterms with signs of developmental arrest showed a marked redistribution of IRL and the layers of photoreceptor cell bodies, axons and pedicles/synapses within the central fovea. This is in line with incomplete displacement of these layers from the foveal center. Since no changes of photoreceptor inner and outer segment thickness were observed the maturation of the photoreceptor segment layers seems to be within normal limits.ConclusionsOur landmark‐based analysis of OCT images using reflectometry and manual segmentation provides complementary findings in comparisons of normal and immature foveal structures. The thickness increase in the outer nuclear, Henle fiber, outer plexiform and post‐receptor layers in preterms with signs of arrested foveal development indicate a marked redistribution of cells.
Correlations of retinal thickness with frequency‐doubling technology perimetry in older healthy subjects
Authors: Perez Carrasco M.J. , Palomo‐Alvarez C. , Chozas‐Enrique J. , Gómez‐García S. , Ayala‐Ayerbes M. , Puell M.C. 
Published: September 2017

PurposeTo evaluate correlations of macular and peripapillary retinal nerve fiber layer (pRNFL) thickness with frequency‐doubling technology (FDT) perimetry in older healthy subjects.MethodsThe observational, transversal, and comparative study examined 65 healthy eyes of 33 young subjects (22.0 ± 2.1 years) and 32 older subjects (62.2 ± 3.9 years). All eyes underwent inner and outer macular‐segmentation (Treatment Diabetic Retinopathy Study macular regions) and peripapillary retinal nerve fiber layer (pRNFL) thickness measurements using spectral domain optical coherence tomography (iVue SD‐OCT), and the FDT testing with N‐20 full‐threshold protocol.ResultsThe fovea thickness decreased in the inner retina (p = 0.03), and increased in the outer retina (p = 0.025) in the older group. The inner macular‐segment of the 3 mm zone was thinner (p = 0.002) in the older subjects. The 5 mm zones of inner and outer macular‐segments and pRNFL thickness showed no significant differences with age. The FDT sensitivity was significantly lower in the older group, but the mean of mean deviation (MD) and pattern standard deviation (PSD) were not significantly different between age groups. PSD values were within the normal limit range (0‐6 dB) in both groups. Only the PSD of FDT showed a significant negative correlation (R = ‐0.387, p = 0.028) with the outer macular‐segment thickness of the peripheral ring in older subjects.ConclusionsThe FDT pattern standard deviation increased as the outer macular‐segment thickness of peripheral ring decreased in healthy older subjects.
Efficacy of tumour necrosis factor inhibitors in peripheral ulcerative keratitis in Granulomatosis with polyangiitis
Authors: Verly E. , De Kock J. , Leroy B.P. , Sys C. , De Schryver I. 
Published: September 2017

PurposeTo report the efficacy of TNFi (tumour necrosis factor inhibitor), as a treatment of therapy‐resistant peripheral ulcerative keratitis (PUK) in granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis.MethodsObservational report about 2 cases, known with the diagnosis of GPA, presenting with therapy‐resistant PUK.Results2 middle‐aged females, known with GPA were referred to our department with a history of chronic redness resistant to local corticosteroids. Both patients showed a unilateral limbal vasculitis with necrotizing keratitis on slit lamp examination. In the first case, the diagnosis of GPA was confirmed by positive anti‐neutrophil cytoplasmic antibodies (ANCA) and nasal septum biopsy. In the second case, the clinical presentation was considered as a viral keratitis due to recurrent unilateral inflammation. Polymerase chain reaction (PCR) could not confirm the viral aetiology. Extensive systemic evaluation revealed a cervical lymphadenopathy on PET‐CT scan. Serology was positive for ANCA. In both cases, the PUK was nonresponsive to systemic corticosteroids. In the first case, cyclophosphamide failed to control the inflammation. Because the risk of corneal perforation, a treatment with TNFi, respectively Infliximab and Adalimumab, were successfully initiated.ConclusionsPotentially lethal, it is important to diagnose and treat GPA urgently. Recently, TNFi were reported to be effective in the treatment of therapy‐resistant GPA. Both patients were refractory to conventional immunomodulatory therapy. Infliximab and adalimumab successfully controlled the inflammation with disappearance of necrotic foci. In our experience, TNFi are a safe and effective therapeutic strategy.
A case of recurrent bilateral optic oedema in tubulo‐interstitial nephritis and uveitis syndrome treated with plasmapheresis
Authors: Dhaese S. , Dehoorne J. , Willemot J.B. , Sys C. , Leroy B.P. , Vande Walle J. , De Schryver I. 
Published: September 2017

PurposeTo present a case of recurrent bilateral optic oedema in a patient with tubulo‐interstitial nephritis and uveitis syndrome (TINU) with a limited response to corticosteroids, immunosuppressive drugs and tumor necrosis factor inhibitor (TNFi). Plasmapheresis was added as rescue therapy.MethodsObservational case report about a 13‐year‐old boy diagnosed with TINU and bilateral optic oedema. An extensive general and ophthalmological work‐up confirmed the diagnosis of TINU.ResultsAt referral the patient presented with bilateral anterior uveitis and nephritis. Biomicroscopy showed a trace of anterior chamber cells. Funduscopy revealed bilateral optic oedema with neither haemorrhages nor exudates. Initially the patient responded well to systemic corticosteroids but he relapsed while tapering. Consecutive treatment consisted of azathioprine, tacrolimus and methotrexate. Due to limited response, infliximab, a TNFi, was associated. After 7 months disease‐free, the TNFi treatment failed. Finally, he responded well to plasmapheresis.ConclusionsTINU may rarely manifest with optic oedema. Although nephritis typically resolves, ocular inflammation often becomes chronic and can be therapy‐resistant. This case suggests that plasmapheresis can be an alternative in case of a therapy‐resistant TINU. A multidisciplinary approach is essential to confirm the diagnosis and initiate appropriate treatment.