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BACKGROUND: To investigate the presence of silicone oil (SO)-emulsification on the anterior iris surface with anterior segment optical coherence tomography (AS-OCT). METHODS: In this single-center cross-sectional study, vitrectomized eyes with SO tamponade that underwent AS-OCT imaging and gonioscopy examination during the postoperative follow-up visits, were reviewed. RESULTS: 45 eyes of 42 consecutive patients were included. In 35.6% of the eyes (n = 16) emulsified SO droplets were detected in the anterior chamber (AC) angle by gonioscopy and in 55.6% (n = 25) on the anterior iris surface by AS-OCT imaging. The presence of SO emulsifications in the AC-angle correlated with the presence of SO emulsifications on the anterior iris surface (OR = 13.4, 95% CI [2.179-82.130]; p = 0.005). The accuracy of the AS-OCT predicting the presence of SO in the AC-angle was 71.0% and the sensitivity was 87.5%. No significant association between SO droplets in the AC and other clinical parameters including endotamponade-duration or type of silicone oil were found. The presence of emulsified SO droplets on the anterior iris surface detected by AS-OCT was significantly correlated to postoperative IOP rise (p = 0.027). CONCLUSION: AS-OCT is a suitable method for the detection of SO on the anterior iris surface. SO droplets on the iris surface correlate with elevated postoperative IOP and with the presence of SO in the AC detected by gonioscopy, therefore AS-OCT might be used as a screening method for the detection of SO migration into the AC. TRIAL REGISTRATION NUMBER: Institutional Review Board of the Hamburg Medical Chamber (Ethik-Kommission der Ärztekammer Hamburg): 2023-300372-WF.
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BACKGROUND: Artificial intelligence (AI) has the potential to increase the affordability and accessibility of eye disease screening, especially with the recent approval of AI-based diabetic retinopathy (DR) screening programs in several countries. METHODS: This study investigated the performance, feasibility, and user experience of a seamless hardware and software solution for screening chronic eye diseases in a real-world clinical environment in Germany. The solution integrated AI grading for DR, age-related macular degeneration (AMD), and glaucoma, along with specialist auditing and patient referral decision. The study comprised several components: (1) evaluating the entire system solution from recruitment to eye image capture and AI grading for DR, AMD, and glaucoma; (2) comparing specialist's grading results with AI grading results; (3) gathering user feedback on the solution. RESULTS: A total of 231 patients were recruited, and their consent forms were obtained. The sensitivity, specificity, and area under the curve for DR grading were 100.00%, 80.10%, and 90.00%, respectively. For AMD grading, the values were 90.91%, 78.79%, and 85.00%, and for glaucoma grading, the values were 93.26%, 76.76%, and 85.00%. The analysis of all false positive cases across the three diseases and their comparison with the final referral decisions revealed that only 17 patients were falsely referred among the 231 patients. The efficacy analysis of the system demonstrated the effectiveness of the AI grading process in the study's testing environment. Clinical staff involved in using the system provided positive feedback on the disease screening process, particularly praising the seamless workflow from patient registration to image transmission and obtaining the final result. Results from a questionnaire completed by 12 participants indicated that most found the system easy, quick, and highly satisfactory. The study also revealed room for improvement in the AMD model, suggesting the need to enhance its training data. Furthermore, the performance of the glaucoma model grading could be improved by incorporating additional measures such as intraocular pressure. CONCLUSIONS: The implementation of the AI-based approach for screening three chronic eye diseases proved effective in real-world settings, earning positive feedback on the usability of the integrated platform from both the screening staff and auditors. The auditing function has proven valuable for obtaining efficient second opinions from experts, pointing to its potential for enhancing remote screening capabilities. TRIAL REGISTRATION: Institutional Review Board of the Hamburg Medical Chamber (Ethik-Kommission der Ärztekammer Hamburg): 2021-10574-BO-ff.
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Retinopatía Diabética , Glaucoma , Degeneración Macular , Humanos , Inteligencia Artificial , Retinopatía Diabética/diagnóstico , Tamizaje Masivo/métodos , Programas Informáticos , Degeneración Macular/diagnóstico , Glaucoma/diagnósticoRESUMEN
In the pursuit of identifying the underlying pathways of ocular diseases, the use of cell lines such as (retinal ganglion cell-5) RGC-5 and 661W became a valuable tool, including pathologies like retinal degeneration and glaucoma. In 2001, the establishment of the RGC-5 cell line marked a significant breakthrough in glaucoma research. Over time, however, concerns arose about the true nature of RGC-5 cells, with conflicting findings in the literature regarding their identity as retinal ganglion cells or photoreceptor-like cells. This study aimed to address the controversy surrounding the RGC-5 cell line's origin and properties by comparing it with the 661W cell line, a known cone photoreceptor model. Both cell lines were differentiated according to two prior published redifferentiation protocols under the same conditions using 500 nM of trichostatin A (TSA) and investigated for their morphological and neuronal marker properties. The results demonstrated that both cell lines are murine, and they exhibited distinct morphological and neuronal marker properties. Notably, the RGC-5 cells showed higher expression of the neuronal marker ß-III tubulin and increased Thy-1-mRNA compared with the 661W cells, providing evidence of their different properties. The findings emphasize the importance of verifying the authenticity of cell lines used in ocular research and highlight the risks of contamination and altered cell properties.
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Contaminación de Medicamentos , Glaucoma , Animales , Ratones , Diferenciación Celular , Línea Celular , Células Fotorreceptoras Retinianas Conos , Tubulina (Proteína)RESUMEN
BACKGROUND: To investigate the changes in macular cystic schisis (MCS) and sensitivity during the day in X-linked retinoschisis (XLRS) patients. METHODS: Treatment-naïve patients with genetically verified XLRS underwent best-correlated visual acuity (BCVA) testing with ETDRS charts, spectral domain optical coherence tomography, and microperimetry (MP) twice a day, at 9 a.m. and 4 p.m., to measure changes in central retinal thickness (CRT), macular volume (MV), average threshold (AT), and fixation stability parameters (P1 and P2). RESULTS: At baseline, the BCVA of the 14 eyes of 8 patients amounted 0.73 (± 0.23) LogMAR. Between timepoints, the BCVA increased in 3.21 letters (p = .021), the AV improved in 1.84 dB (p = .03, 9.73%), the CRT decreased in 24.43 µm (p = .007, - 4.05%), and the MV dropped in 0.27 µm3 (p = .016, - 2.68%). P1 and P2 did not variate. The collapse of the MCS led to the reduction of macula thickness. CRT at baseline correlated with the decrease of CRT (Spearman's ρ: - 0.83 [p = .001]). Age and change of BCVA, CRT, and AV did not correlate among one another. Eyes with disrupted ellipsoid zone showed a more prominent change in CRT (p = .050). Photoreceptor outer segment length and integrity of the external limiting membrane and cone outer segment tips were not associated with BCVA, AT, or CRT variation. CONCLUSION: Eyes of treatment-naïve XLRS patients show diurnal macular thickness and function changes. Eyes with pronounced macular thickness show a greater reduction of the MCS. These results should be taken into consideration in upcoming clinical trials in XLRS. TRIAL REGISTRATION NUMBER: Institutional Review Board of the Hamburg Medical Chamber (Ethik-Kommission der Ärztekammer Hamburg): 2020-10,328.
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BACKGROUND/AIMS: Late-infantile neuronal ceroid lipofuscinosis type 2 (CLN2) is a neurodegenerative, blinding lysosomal storage disorder. The purpose of the current study was to characterise the progression of CLN2-associated retinal degeneration in patients under intraventricular enzyme replacement therapy (ERT) with cerliponase alfa. METHODS: We analysed visual function, retinal morphology and neuropaediatric data using preferential looking test (PLT), Weill Cornell Batten Scale (WCBS), optical coherence tomography (OCT) imaging and the Hamburg Motor-Language late-infantile neuronal ceroid lipofuscinosis (LINCL) Scale (M-L scale). RESULTS: Fifty-six eyes of 28 patients had baseline PLT, WCBS and OCT. 15 patients underwent serial examinations, resulting in a total of 132 OCT scans and WCBS results, 66 Hamburg M-L scores and 49 PLT results during a mean follow-up time of 18.2 months (range 5-40). A negative correlation (r=-0.69, p<0.001) was found between central retinal thickness (CRT) values and age at examination with a maximal annual decrease of 23 µm between 56 and 80 months of age. A significant correlation was observed between PLT results and the age at examination (r=0.46, p=0.001), the WCBS scores (r=0.62; p<0.001) and CRT values (r=-0.64; p<0.001). The M-L score correlated with the ocular measurements (CRT: r=0.58, p<0.001; WCBS r=-0.64, p<0.001; PLT score: r=-0.57, p<0.001). CONCLUSION: Despite intraventricular ERT, retinal degeneration progressed in patients with CLN2 and was particularly pronounced between 56 and 80 months of age. Retina-directed therapies should therefore be initiated before or as early as possible during the phase of rapid retinal degeneration. PLT and WCBS were identified as valuable outcome measures to monitor disease progression. TRIAL REGISTRATION NUMBER: NCT04613089.
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Lipofuscinosis Ceroideas Neuronales , Degeneración Retiniana , Preescolar , Humanos , Lactante , Terapia de Reemplazo Enzimático , Lipofuscinosis Ceroideas Neuronales/diagnóstico , Lipofuscinosis Ceroideas Neuronales/tratamiento farmacológico , Lipofuscinosis Ceroideas Neuronales/complicaciones , Degeneración Retiniana/diagnóstico , Degeneración Retiniana/tratamiento farmacológico , Degeneración Retiniana/complicaciones , Tripeptidil Peptidasa 1 , Masculino , FemeninoRESUMEN
PURPOSE: Patients with CLN2 suffer from epileptic seizures, rapid psychomotor decline and vision loss in early childhood. The aim of the study was to provide longitudinal ophthalmic data of patients with confirmed genetic mutation and non-classical disease course, marked by later onset, protracted progression and prolonged life span. METHODS: Prospective, observational study to assess visual acuity, retinal features (Weil Cornell Ophthalmic Score), central retinal thickness (CRT) measured by optical coherence tomography and general disease progression (Hamburg CLN2 motor language score) in non-classical CLN2 patients. RESULTS: All patients received intracerebroventricular enzyme replacement therapy with cerliponase alfa. Mean age at last follow-up was 12.4 years; mean follow-up time 2.6 years. All cases demonstrated a stable Hamburg motor language CLN2 Score and Weill Cornell LINCL Ophthalmic Severity Score. Visual function remained stable in 4/6 patients, 2/6 patients showed a decrease, 4/6 cases had a stable CRT and 2/6 showed a reduction of CRT. One patient showed a massive macular thinning and low vision. A correlation with a specific mutation or age could not be verified. DISCUSSION: The presented longitudinal study characterizes the variable ocular involvement in non-classical CLN2 disease and contributes to the natural history description. The functional and morphologic data outline the necessity of regular ophthalmic examination. Ocular phenotyping and description of retinal degeneration in non-classical CLN2 disease.
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Lipofuscinosis Ceroideas Neuronales , Tripeptidil Peptidasa 1 , Niño , Humanos , Estudios Longitudinales , Lipofuscinosis Ceroideas Neuronales/diagnóstico , Lipofuscinosis Ceroideas Neuronales/genética , Estudios Prospectivos , Tomografía de Coherencia Óptica , Agudeza VisualRESUMEN
PURPOSE: An increasing number of posterior segment disorders is routinely managed with pars plana vitrectomy (PPV). In older, phakic patients cataract formation is expected within the first two years after surgery. For younger patients its progression is individually fluctuating. This study uses an objective quantitative measurement for lens-status-monitoring after PPV with silicone oil to derive predictions for progression and severity of post-operative lens opacification evaluated in patients with rhegmatogenous retinal detachment (RRD). METHODS: Data acquisition was performed prospectively between March 2018 and March 2021. PentacamHR® Nucleus Staging mode (PNS) was used to objectively gather data about nuclear cataracts after PPV at different time points. Data was grouped into training and test sets for a mathematical prediction model. Via backward variable selection method a mathematical formula was set up by means of which predictions about lens densitometry (LD) can be calculated. RESULTS: 20 males [58.8%] and 14 females [41.2%] matched the inclusion criteria (mean age 50.6 years [23-75; ±12.3]). Average follow-up was 8.1 months (3,4-17.4; ±3.4). Mean baseline LD of the treated and fellow eye before surgery was 11.1% (7.7%-17.6%; ±2.0) and 11.2% (7.7%-14.8%; ±1.5), respectively. Predicted LD values by the model for five pre-selected patients closely match the observed data with an average deviation of 1.06%. CONCLUSIONS: Using an objective parameter like LD delivered by the PentacamHR® PNS mode additionally to the patient's age allows us to make an individual prediction for any time after PPV with silicone oil due to RRD for all ages. The accuracy of the model was stronger influenced by baseline LD as cofactor in the equation than patient's age. The application for the prediction lens opacification [which can be accessed for free under the following link (https://statisticarium.com/apps/sample-apps/LensDensityOil/)] can help vitreoretinal surgeons for patient consultation on the possibility to combine PPV with cataract surgery.
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Catarata , Desprendimiento de Retina , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Desprendimiento de Retina/cirugía , Estudios Retrospectivos , Aceites de Silicona/efectos adversos , Agudeza Visual , Vitrectomía/efectos adversos , Vitrectomía/métodosRESUMEN
Purpose: To investigate the efficacy and safety profile of retinal tacks (RTs) in cases of retinal detachment (RD) with advanced proliferative vitreoretinopathy (PVR). Materials and Methods: In this single-center, retrospective study medical record, optical coherence tomography and ultra-widefield fundus images of patients with complex PVR-related and RT surgery were reviewed. All cases underwent 23G pars plana vitrectomy (PPV), RT implantation, retinectomy, circumferential intraoperative laser retinopexy, and silicone oil tamponade. Results: Fourteen eyes of 14 patients with complex rhegmatogenous RD with PVR were included: 7 cases showed PVR grade C type P and 7 combined grades A and P. RTs were positioned at contracted, stiffened retinal areas to achieve attachment of retinectomy borders after extensive PVR peeling. Patients underwent on an average of 1.3 PPVs (range 0-3) prior RT surgery. An average of 2.5 RTs (range 1-4) were implanted. Only in a single eye, a recurrent RD occurred. In 10 eyes, the silicone oil tamponade was still in place at the last follow-up. In 5 eyes, the silicone oil could be removed without redetachment in all of these cases (average of 31.3 weeks, range 11.4-53). No RT-related intraoperative or postoperative complications like dislocation or bleedings were observed. Conclusion: RTs have the potential to improve the treatment of complex PVR-associated RD. RT can be a useful surgical tool to reattach borders of retinectomies in advanced PVR. No RT-associated complication were observed in this study.
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BACKGROUND: Tamsulosin is one of the most commonly prescribed alpha-1A antagonists for the treatment of benign prostatic syndrome (BPS). Patients treated with tamsulosin often develop intraoperative floppy iris syndrome (IFIS) during cataract surgery. This leads to increasing miosis and an undulating iris, which increases the risk of complications significantly and can cause permanent damage. AIM OF THE WORK: The aim is to raise awareness for the effects of tamsulosin intake before cataract surgery. MATERIAL AND METHODS: We conducted a critical review of publications on the association of IFIS in cataract surgery with alpha-receptor blockers. RESULTS AND DISCUSSION: Tamsulosin has a risk of complications of up to 80â%, whereas doxazosin and alfuzosin only have a 15-20â% chance of complications. Tamsulosin therefore represents a significant risk factor for permanent secondary damage after cataract surgery. Even after discontinuing tamsulosin, IFIS was still observed after up to 3 years. Nevertheless, pausing of tamsulosin intake is recommended. An alternative preparation should therefore be preferred in patients who have not yet had cataract surgery. If patients are already pseudophakic, tamsulosin can be given without concern.
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Antagonistas de Receptores Adrenérgicos alfa 1 , Catarata , Antagonistas de Receptores Adrenérgicos alfa 1/efectos adversos , Antagonistas Adrenérgicos alfa/efectos adversos , Catarata/inducido químicamente , Humanos , Complicaciones Intraoperatorias/inducido químicamente , Enfermedades del Iris/inducido químicamente , Sulfonamidas/efectos adversos , Tamsulosina/efectos adversosRESUMEN
BACKGROUND: To evaluate the rate, risk factors, functional outcome and prognosis in eyes with retinal detachment after post-operative endophthalmitis treated with 23G Pars Plana Vitrectomy. METHODS: Electronic patient files from 2009 until 2018 were screened for the presence of an endophthalmitis. Included were 116 eyes of 116 patients. This population was evaluated for the rate of retinal detachment after 23G Pars Plana Vitrectomy for endophthalmitis following cataract surgery or intravitreal injection. The main outcome measures were retinal detachment and visual acuity. RESULTS: The reasons for endophthalmitis were previous cataract surgery in 78 patients and following intravitreal injection in 38 patients. The first clinical evidence of endophthalmitis was present in median 5 days after the triggering intervention. Twenty-five eyes (21.55%) developed a retinal detachment an average of 25 days after endophthalmitis. RD is significantly associated with preoperative visual acuity (p = 0.001). CONCLUSIONS: We emphasize the prognostic role of preoperative visual acuity in RD development of the endophthalmitis treated with 23G Pars Plana Vitrectomy.
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Endoftalmitis , Desprendimiento de Retina , Endoftalmitis/epidemiología , Endoftalmitis/etiología , Endoftalmitis/terapia , Humanos , Inyecciones Intravítreas , Desprendimiento de Retina/etiología , Desprendimiento de Retina/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Agudeza Visual , VitrectomíaRESUMEN
AIM: To investigate the safety of vitreoretinal surgery when using a soft contact lens as a temporary keratoprosthesis (TKP) in patients with severe corneal opacifications. METHODS: Three patients with simultaneous corneal and vitreoretinal pathology were treated with a soft contact lens that was used as a TKP to facilitate vitreoretinal surgery. The soft contact lens was fixated with sutures onto the globe so that no leakage was possible. RESULTS: Vitreoretinal surgery with excellent fundus view was possible in all cases. The soft contact lens allowed safe central and peripheral vitrectomy. Surgery was successful in all cases. CONCLUSION: A soft contact lens properly fixated on the globe can successfully replace a TKP. This surgical procedure has several advantages like one size fits all, low costs, and easy access to the material.
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PURPOSE: The aim of this case control study was to evaluate the prognostic value of automatically quantified retinal vessel tortuosity from fundus images and vessel density from OCT-A in Fabry disease and to evaluate the correlation of these with systemic disease parameters. METHODS: Automatically quantified perimacular retinal vessel tortuosity (MONA REVA software), acquired by fundus imaging, and perifoveal retinal vessel density, acquired by optic coherence tomography angiography (OCT-A) were compared between 26 FD patients and 26 controls. Gender and FD phenotype were analyzed to the obtained retinovascular data and correlated to the Mainz severity score index (MSSI) and plasma lyso-Gb3. RESULTS: Automatically quantified retinal vessel tortuosity indices of FD patients were significantly lower, reflecting an increased vessel tortuosity, compared to controls (p = 0.008). Males with a classical phenotype showed significantly lower retinal vessel tortuosity indices compared to males with an oligosymptomatic phenotype and females with a classical or oligosymptomatic phenotype (p < 0.001). The retinal vessel tortuosity index correlated significantly with systemic disease severity parameters [global MSSI (r = - 0.5; p < 0.01), cardiovascular MSSI (r = - 0.5; p < 0.01), lyso-Gb3 (r = - 0.6; p < 0.01)]. CONCLUSION: We advocate fundus imaging based automatically quantified retinal vessel tortuosity index over OCT-A imaging as it is a quick, non-invasive, easily assessable, objective and reproducible marker.
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Enfermedad de Fabry , Estudios de Casos y Controles , Enfermedad de Fabry/genética , Femenino , Humanos , Masculino , Pronóstico , Vasos Retinianos , Índice de Severidad de la Enfermedad , Tomografía de Coherencia Óptica/métodosRESUMEN
In this study, we examined the rate of contamination of multi-dose ophthalmic solutions in the operating theatre and the underlying risk for infection by examining the microbiological load on the tips of the dispenser bottles. A total of 245 samples of eye drop bottles were collected and analysed between June 2018 and January 2019. All were collected in the operating theatre of the University Eye Hospital Hamburg-Eppendorf. Contamination of the dropper tip occurred in 2% of the samples. Although the prevalence of contamination was low, the results of this study reveal the possibility of contamination of multi-dose eyedrops even when used by health care professionals in the controlled environment of an operating theatre. Following these results, we recommend the use of single-dose eyedrops in the pre- and intraoperative context.
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AIM: To elucidate the question of whether the ocular trauma score (OTS) and the zones of injury could be used as a predictive model of traumatic and post traumatic retinal detachment (RD) in patients with open globe injury (OGI). METHODS: A retrospective observational chart analysis of OGI patients was performed. The collected variables consisted of age, date, gender, time of injury, time until repair, mechanism of injury, zone of injury, injury associated vitreous hemorrhage, trauma associated RD, post traumatic RD, aphakia at injury, periocular trauma and OTS in cases of OGI. RESULTS: Totally 102 patients with traumatic OGI with a minimum of 12mo follow-up and a median age at of 48.6y (range: 3-104y) were identified. Final best corrected visual acuity (BCVA) was independent from the time of repair, yet a statistically significant difference was present between the final BCVA and the zone of injury. Severe trauma presenting with an OTS score I (P<0.0001) or II (P<0.0001) revealed a significantly worse BCVA at last follow up when compared to the cohort with an OTS score >III. OGI associated RD was observed in 36/102 patients (35.3%), whereas post traumatic RD (defined as RD following 14d after OGI) occurred in 37 patients (36.3%). OGI associated RD did not correlate with the OTS and the zone of injury (P=0.193), yet post traumatic RD correlated significantly with zone III injuries (P=0.013). CONCLUSION: The study shows a significant association between lower OTS score and zone III injury with lower final BCVA and a higher number of surgeries, but only zone III could be significantly associated with a higher rate of RD.
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BACKGROUND: Neuronal ceroid lipofuscinoses are hereditary lysosomal storage diseases, which lead to a progressive neurodegeneration of the brain and retina. Visual loss can be the initial symptom but can also occur later in the course of the disease. OBJECTIVE: The aim of this article is to provide ophthalmologists with an overview of the characteristic ocular alterations and the general disease course of the 13 currently known various forms of NCL. MATERIAL AND METHODS: The findings from predominantly clinical articles are reviewed and summarized. RESULTS AND CONCLUSION: Retinal degeneration plays a crucial role in this group of neurodegenerative diseases. In several forms visual decline is the initial clinical symptom in affected patients. Therefore, the ophthalmologist is the first medical expert consulted. An early diagnosis is crucial for the future personal and family planning but is also important regarding upcoming therapeutic strategies, which might be much more effective in patients with early stage disease. When the presence of retinal degeneration due to an NCL disease is suspected an immediate genetic diagnostic confirmation and collaboration with neuropediatricians is recommended.
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Lipofuscinosis Ceroideas Neuronales , Oftalmólogos , Encéfalo , Humanos , Lipofuscinosis Ceroideas Neuronales/diagnóstico , Lipofuscinosis Ceroideas Neuronales/genética , Lipofuscinosis Ceroideas Neuronales/terapia , Retina , Trastornos de la Visión/diagnóstico , Trastornos de la Visión/etiologíaRESUMEN
PURPOSE: Juvenile CLN3 disease, the most prevalent form of Batten disease, is a progressive neurodegenerative disorder resulting from mutations in the CLN3 gene. The objective of this study was to design an ophthalmic rating scale for CLN3 disease in order to quantify disease progression. DESIGN: Retrospective, cross-sectional study. METHODS: Patients underwent ophthalmic evaluations including visual testing, optical coherence tomography and fundus imaging. Patients were also assessed using the Hamburg Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) scoring system. Ophthalmic findings were divided into grades of severity ranging from 0 to 3, and the association between the extent of ocular disease and neurological function and age was assessed. RESULTS: Forty-two eyes of 21 patients were included. The mean age at the time of examination was 13.2 years (range, 5.3-21.9 years). The mean ophthalmic severity grade was 2.4 (range, 0-3). The mean neurological severity score was 9.9 (range, 4-14). Ophthalmic manifestations increased in severity with increasing age of the patients (r = -0.84; P < .001), and a strong correlation was found between the CLN3 ophthalmic rating scale score and the Hamburg JNCL score (r = 0.83; P < .001). CONCLUSIONS: Ophthalmic manifestations of CLN3 disease correlate closely with the severity of neurological symptoms and age of the patient. The newly established Hamburg CLN3 ophthalmic rating scale may serve as an objective marker of ocular disease severity and progression and may be valuable tool for the evaluation of novel therapeutic strategies for CLN3 disease.
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ADN/genética , Glicoproteínas de Membrana/genética , Chaperonas Moleculares/genética , Mutación , Lipofuscinosis Ceroideas Neuronales/complicaciones , Degeneración Retiniana/etiología , Adolescente , Niño , Preescolar , Estudios Transversales , Análisis Mutacional de ADN , Progresión de la Enfermedad , Femenino , Humanos , Masculino , Glicoproteínas de Membrana/metabolismo , Chaperonas Moleculares/metabolismo , Lipofuscinosis Ceroideas Neuronales/diagnóstico , Lipofuscinosis Ceroideas Neuronales/genética , Degeneración Retiniana/diagnóstico , Degeneración Retiniana/genética , Estudios Retrospectivos , Tomografía de Coherencia Óptica , Adulto JovenRESUMEN
OBJECTIVE: Perioperative visual loss (POVL) is a rare but serious complication in surgical disciplines, especially in spine surgery. The exact pathophysiology of POVL remains unclear, but elevated intraocular pressure (IOP) is known to be part of it. As POVL is rarely described in patients undergoing intracranial or intradural surgery, the aim of this study was to investigate the course of IOP during neurosurgical procedures with opening of the dura mater and loss of CSF. METHODS: In this prospective, controlled trial, 64 patients fell into one of 4 groups of 16 patients each. Group A included patients undergoing spine surgery in the prone position, group B patients had intracranial procedures in the prone position, and group C patients were treated for intracranial pathologies in a modified lateral position with the head rotated. In groups A-C, the dura was opened during surgery. Group D patients underwent spine surgeries in the prone position with an intact dura. IOP was measured continuously pre-, peri-, and postoperatively. RESULTS: In all groups, IOP decreased after induction of anesthesia and increased time dependently after final positioning for the operation. The maximum IOP in group A prior to opening of the dura was 28.6 ± 6.2 mm Hg and decreased to 23.44 ± 4.9 mm Hg directly after dura opening (p < 0.0007). This effect lasted for 30 minutes (23.5 ± 5.6 mm Hg, p = 0.0028); after 60 minutes IOP slowly increased again (24.5 ± 6.3 mm Hg, p = 0.15). In group B, the last measured IOP before CSF loss was 28.1 ± 5.0 mm Hg and decreased to 23.5 ± 6.1 mm Hg (p = 0.0039) after dura opening. A significant IOP decrease in group B lasted at 30 minutes (23.6 ± 6.0 mm Hg, p = 0.0039) and 60 minutes (23.7 ± 6.0 mm Hg, p = 0.0189). In group C, only the lower eye showed a decrease in IOP up to 60 minutes after loss of CSF (opening of dura, p = 0.0007; 30 minutes, p = 0.0477; 60 minutes, p = 0.0243). In group D (control group), IOP remained stable throughout the operation after the patient was prone. CONCLUSIONS: This study is the first to demonstrate that opening of the dura with loss of CSF during neurosurgical procedures results in a decrease in IOP. This might explain why POVL predominantly occurs in spinal but rarely in intracranial procedures, offers new insight to the pathophysiology of POVL, and provides the basis for further research and treatment of POVL.German Clinical Trials Register (DRKS) no.: DRKS00007590 (drks.de).
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INTRODUCTION: Idiopathic epiretinal membrane (ERM) is a proliferation of cells formed on the internal surface of the retina and may cause a slow decrease in visual acuity (VA). The aim of this study was to evaluate the individual layers of the retina by optical coherence tomography (OCT) before and after vitrectomy with ERM peeling in order to refine surgical decision-making. METHODS: Forty-seven eyes of 45 patients (aged 55-87 years) with ERM were enrolled retrospectively from a tertiary referral center. OCT examination was performed preoperatively and at a mean of 3.2 months postoperatively in all cases. Nine retinal surfaces were subjected to automated all-layer segmentation using the manufacturer's software and assessed for their correlation with VA. RESULTS: There was a significant correlation between the initial retinal morphology and change in VA postoperatively. Patients who gained at least two lines of VA had a significantly higher total retinal perimacular volume and retinal nerve fiber layer (each p < 0.0001) preoperatively. CONCLUSION: Patients with high preoperative retinal volumes and therefore increased tractive components seemed to benefit more from surgery than those with low tractive components.
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Membrana Epirretinal/cirugía , Células Ganglionares de la Retina/patología , Tomografía de Coherencia Óptica/métodos , Agudeza Visual , Vitrectomía , Anciano , Anciano de 80 o más Años , Membrana Epirretinal/diagnóstico , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Periodo Posoperatorio , Valor Predictivo de las Pruebas , Estudios RetrospectivosRESUMEN
A precise evaluation of the retinal nerve fiber layer thickness (RNFLT) is key for diagnosing and monitoring glaucoma. The Bruch's membrane opening minimum rim width (BMO-MRW) has been proposed as a reproducible assessment of the optic nerve. The BMO-MRW measures the minimum distance from the BMO to the internal limiting membrane. We propose an approach to correct the BMO-MRW using the BMO size for increased accuracy in interindividual comparisons in future studies. Eighty-one healthy patients received SPECTRALIS spectral domain optical coherence tomography measurements for the peripapillary RNFLT and BMO-MRW. We calculated a BMO size-corrected BMO-MRW using the mean BMO size of our cohort. BMO size was defined using the manufacturer-provided BMO area and manually measured BMO perimeter. We observed that the BMO-MRW correlated highly with the perimeter (r = -0.553, p < 0.0001) and the area of the BMO (r = -0.546, p < 0.0001). Using these parameters, we provided a corrected BMO size-adjusted BMO-MRW which was better correlated with the RNFLT compared to the noncorrected one (z = -3.3495, p = 0.0004). We demonstrated the dependency of the BMO-MRW on ONH size. Furthermore, we showed the superiority of the corrected BMO-MRW using either the manually measured optic nerve head perimeter or the automatically provided ONH for future studies.