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BMC Ophthalmology Jun 2024Paraproteinemic keratopathy is a rare disorder characterized by the bilateral accumulation of polychromatic deposits diffusely in all corneal layers together or not with...
BACKGROUND
Paraproteinemic keratopathy is a rare disorder characterized by the bilateral accumulation of polychromatic deposits diffusely in all corneal layers together or not with diffuse or patchy pseudo lipid deposits. We present an atypical case of paraproteinemic keratopathy which lead to an initial misdiagnosis of infectious crystalline keratopathy.
CASE PRESENTATION
a 69-year-old woman with an asymptomatic keratopathy detected during a cataract intervention. Slit-lamp examination revealed several hyper refringent subepithelial foci with fern-shaped branches, resembling crystalline keratopathy, in her left eye. Anterior segment optical coherence tomography revealed exclusively subepithelial hyperreflective lesions limited to the anterior stroma. The progressive bilateralization and progression of the condition prompted us to include other entities with crystalline corneal deposits in our differential diagnosis. Hematological analysis showed a high number of free Kappa light chains. Despite the typical clinical appearance of crystalline keratopathy, the atypical evolution and test results led us to consider that monoclonal gammopathy could be the cause of this entity.
CONCLUSIONS
Paraproteinemic keratopathy may present in its early stages as a unilateral subepithelial crystalline keratopathy. Thus, it must always be taken into account in the differential diagnosis of any crystalline keratopathy, particularly when there are no predisposing factors for an infectious crystalline keratopathy. Early recognition of this rare entity is important to address the associated potentially serious systemic disease.
Topics: Humans; Aged; Female; Diagnosis, Differential; Corneal Diseases; Paraproteinemias; Tomography, Optical Coherence
PubMed: 38898421
DOI: 10.1186/s12886-024-03487-6 -
International Journal of Ophthalmology 2024To analyze and compare the differences among ocular biometric parameters in Han and Uyghur populations undergoing cataract surgery.
AIM
To analyze and compare the differences among ocular biometric parameters in Han and Uyghur populations undergoing cataract surgery.
METHODS
In this hospital-based prospective study, 410 patients undergoing cataract surgery (226 Han patients in Tianjin and 184 Uyghur patients in Xinjiang) were enrolled. The differences in axial length (AL), anterior chamber depth (ACD), keratometry [steep K (Ks) and flat K (Kf)], and corneal astigmatism (CA) measured using IOL Master 700 were compared between Han and Uyghur patients.
RESULTS
The average age of Han patients was higher than that of Uyghur patients (70.22±8.54 63.04±9.56y, <0.001). After adjusting for age factors, Han patients had longer AL (23.51±1.05 22.86±0.92 mm, <0.001), deeper ACD (3.06±0.44 2.97±0.37 mm, =0.001), greater Kf (43.95±1.40 43.42±1.69 D, =0.001), steeper Ks (45.00±1.47 44.26±1.71 D, =0.001), and higher CA (1.04±0.68 0.79±0.65, =0.025) than Uyghur patients. Intra-ethnic male patients had longer AL, deeper ACD, and lower keratometry than female patients; however, CA between the sexes was almost similar. In the correlation analysis, we observed a positive correlation between AL and ACD in patients of both ethnicities ( =0.48, =0.44, <0.001), while AL was negatively correlated with Kf ( =-0.42, =-0.64, <0.001) and Ks ( =-0.38, =-0.66, <0.001). Additionally, Kf was positively correlated with Ks ( =0.89, =0.93, <0.001).
CONCLUSION
There are differences in ocular biometric parameters between individuals of Han ethnicity in Tianjin and those of Uyghur ethnicity in Xinjiang undergoing cataract surgery. These ethnic variances can enhance our understanding of ocular diseases related to these parameters and provide guidance for surgical procedures.
PubMed: 38895687
DOI: 10.18240/ijo.2024.06.10 -
International Journal of Ophthalmology 2024To identify genetic defects in a Chinese family with congenital posterior polar cataracts and assess the pathogenicity.
AIM
To identify genetic defects in a Chinese family with congenital posterior polar cataracts and assess the pathogenicity.
METHODS
A four-generation Chinese family affected with autosomal dominant congenital cataract was recruited. Nineteen individuals took part in this study including 5 affected and 14 unaffected individuals. Sanger sequencing targeted hot-spot regions of 27 congenital cataract-causing genes for variant discovery. The pathogenicity of the variant was evaluated by the guidelines of American College of Medical Genetics and InterVar software. Confocal microscopy was applied to detect the subcellular localization of fluorescence-labeled ephrin type-A receptor 2 (EPHA2). Co-immunoprecipitation assay was implemented to estimate the interaction between EphA2 and other lens membrane proteins. The mRNA and protein expression were analyzed by reverse transcription-polymerase chain reaction (qRT-PCR) and Western blotting assay, respectively. The cell migration was analyzed by wound healing assay. Zebrafish model was generated by ectopic expression of human /p.R957P mutant to demonstrate whether the mutant could cause lens opacity .
RESULTS
A novel missense and pathogenic variant c.2870G>C was identified in the sterile alpha motif (SAM) domain of . Functional studies demonstrated the variant's impact: reduced EPHA2 protein expression, altered subcellular localization, and disrupted interactions with other lens membrane proteins. This mutant notably enhanced human lens epithelial cell migration, and induced a central cloudy region and roughness in zebrafish lenses with ectopic expression of human /p.R957P mutant under differential interference contrast (DIC) optics.
CONCLUSION
Novel pathogenic c.2870G>C variant of in a Chinese congenital cataract family contributes to disease pathogenesis.
PubMed: 38895685
DOI: 10.18240/ijo.2024.06.04 -
International Journal of Ophthalmology 2024To compare the safety and effectiveness of eyelid treatment with the ILux-MGD Treatment System in one session versus five sessions of mechanical meibomian gland...
AIM
To compare the safety and effectiveness of eyelid treatment with the ILux-MGD Treatment System in one session versus five sessions of mechanical meibomian gland expression (MMGE) in patients with moderate to severe meibomian gland dysfunction (MGD).
METHODS
A prospective, randomized, open-label, and controlled clinical trial that compared one session of the ILux MGD Treatment System versus five sessions of MMGE in both eyes of 130 patients aged ≥18y with Ocular Surface Disease Index (OSDI) scores ≥13, total meibomian gland scores (MGS) of <15 in the lower eyelid of each eye, and non-invasive tear break-up time (NI-TBUT) <10s, who were randomized 1:1 to ILux or MMGE.
RESULTS
The mean age was 58±17.49y. Baseline total MGS scores in both treatment groups were comparable. During follow-up, there were significant differences in total MGS and per sector with <0.001. Multivariate analysis was performed using generalized estimating equations corresponding to the generalized linear model for repeated means to determine the treatment relationship with total MGS, NIBUT, and OSDI. There was a significant difference between ILux and MMGE (<0.001) at follow-up from the first to the twelfth month in MGS, NI-BUT, and OSDI scores. No adverse events were reported.
CONCLUSION
ILux treatment compared to MMGE significantly improves symptoms and signs in patients with moderate to severe MGD for one year without adverse events.
PubMed: 38895679
DOI: 10.18240/ijo.2024.06.08 -
International Journal of Ophthalmology 2024To compare high or low concentration of hyaluronic acid eye drops (HY) for dry eye syndromes (DES).
AIM
To compare high or low concentration of hyaluronic acid eye drops (HY) for dry eye syndromes (DES).
METHODS
Randomized controlled trials (RCTs) comparing various concentrations of HY were searched in PubMed, Embase, Web of Science, Cochrane, SinoMed, CNKI, Wanfang Database, CQVIP, and Chinese journals databases between inception and July 2023. Pooled standardized mean differences (SMD) or weighted mean difference (WMD) with 95% confidence intervals (CI) from RCTs evaluating Schirmer's I test (SIT), corneal fluorescein staining score (CFS), tear breakup time (TBUT), DES score (DESS), and Ocular Surface Disease Index (OSDI) were calculated. Sensitivity analysis, Egger's test and Meta-regression analysis were performed for all indicators.
RESULTS
We conducted a Meta-analysis of 10 RCTs that met the inclusion criteria, involving 1796 cases. High-concentrations group significantly improved the outcome of CFS according to random effects modelling (SMD, -3.37; 95%CI, -5.25 to -1.48; =0.0005). The rest of the results were not statistically significant, including indicators such as SIT, TBUT, DESS and OSDI.
CONCLUSION
For dry eyes with positive corneal staining, a high concentration of HY is recommended, whereas in other cases, a high concentration of HY does not offer a more pronounced advantage over a low concentration of HY in the treatment of dry eyes.
PubMed: 38895674
DOI: 10.18240/ijo.2024.06.17 -
International Journal of Ophthalmology 2024To investigate the influence of ophthalmic viscoelastic devices (OVDs) and different surgical approaches on the intraocular pressure (IOP) before and after creation of...
AIM
To investigate the influence of ophthalmic viscoelastic devices (OVDs) and different surgical approaches on the intraocular pressure (IOP) before and after creation of the curvilinear circular capsulorhexis (CCC) as a measure for anterior chamber stability during this maneuver.
METHODS
Prospective experimental WetLab study carried out on enucleated porcine eyes. IOP was measured before and after CCC with the iCare Rebound tonometer (iCare ic200; iCare Finland Oy, Vantaa, Finland). The OVDs used were a cohesive one [Z-Hyalin, Carl Zeiss Meditec AG, Germany; hyaluronic acid (HA)] and a dispersive [Z-Celcoat, Carl Zeiss Meditec AG, Germany; hydroxy propylmethylcellulosis (HPMC)]. The CCC was created using Utrata forceps or 23 g microforceps in different combinations with the OVDs.
RESULTS
Using the Utrata forceps the IOP dropped from 63.65±6.44 to 11.25±3.63 mm Hg during the CCC. The use of different OVDs made no difference. Using the 23 g microforceps the IOP dropped from 65.35±8.15 to 36.55±6.09 mm Hg. The difference between IOP drop using either Utrata forceps or 23 g microforceps was highly significant regardless of the OVD used.
CONCLUSION
Using the sideport for the creation of the capsulorhexis leads to a lesser drop in IOP during this maneuver compared to the main incision in enucleated porcine eyes. The use of different OVD has no significant influence on IOP drop.
PubMed: 38895666
DOI: 10.18240/ijo.2024.06.22 -
Diagnostics (Basel, Switzerland) May 2024To clinically evaluate how dry eye symptoms in preoperatively diagnosed dry eye patients change with the use of sodium hyaluronate- and dexpanthenol-containing eye drops...
BACKGROUND
To clinically evaluate how dry eye symptoms in preoperatively diagnosed dry eye patients change with the use of sodium hyaluronate- and dexpanthenol-containing eye drops (HYLO CARE (HC), URSAPHARM Arzneimittel GmbH, Saarbruecken, Germany) after cataract surgery. The aim of the study was not to compare different eye drops but to implement standard treatment in patients with dry eye undergoing cataract surgery. The impact of treatment was evaluated using Symptom Assessment Tools for Dry Eye.
METHODS
In this prospective, single-center, open-label clinical trial, 49 patients undergoing cataract surgery were included who showed signs and symptoms of dry eye disease assessed by the Symptom Assessment in Dry Eye (Visual Analogue Scale (VAS)) questionnaire, Ocular Surface Disease Index (OSDI), and fluorescein tear break-up Time (TBUT). Patients were instructed to apply HC three to four times a day for 5 weeks in the operated eye in addition to the standard postoperative topical anti-inflammatory regimen. The primary endpoint was the change in TBUT. Secondary endpoints were the assessment of the subjective symptoms (VAS), corrected distance visual acuity (CDVA), and slit-lamp examination including the corneal staining score, Schirmer test, and intraocular pressure.
RESULTS
At 5 weeks after operation, the mean TBUT increased from 6.42 ± 1.57 s (s) to 7.81 ± 1.83 s in the per-protocol (PP) population ( > 0.001) and from 6.33 ± 1.64 s to 7.71 ± 2.05 s in the intention-to-treat (ITT) population ( < 0.001). There was a statistically significant decrease in all scores ( < 0.05) from the VAS questionnaire except for the tearing score ( = 0.062) at 5 weeks after operation. The mean total corneal staining score also decreased statistically significantly from 8.85 ± 2.49 before operation to 5.61 ± 3.37 at 5 weeks after operation on a 15-point scale.
CONCLUSIONS
Controlled standardized dry eye treatment (with HC) improved tear film stability, ocular surface defects, and subjective symptoms of dry eye disease in patients 5 weeks after undergoing cataract surgery. Both the patient and physician assessments indicated high efficacy, tolerability, and a reliable safety profile, as indicated by the low number of at least possibly related adverse events (AE), suggesting its beneficial role in the postoperative management of the ocular surface (OS) in patients with dry eye symptoms prior to and after cataract surgery.
PubMed: 38893624
DOI: 10.3390/diagnostics14111097 -
Cancers May 2024Helium ion therapy (HRT) is a promising modality for the treatment of pediatric tumors and those located close to critical structures due to the favorable biophysical...
Helium ion therapy (HRT) is a promising modality for the treatment of pediatric tumors and those located close to critical structures due to the favorable biophysical properties of helium ions. This in silico study aimed to explore the potential benefits of HRT in advanced juvenile nasopharyngeal angiofibroma (JNA) compared to proton therapy (PRT). We assessed 11 consecutive patients previously treated with PRT for JNA in a definitive or postoperative setting with a relative biological effectiveness (RBE) weighted dose of 45 Gy (RBE) in 25 fractions at the Heidelberg Ion-Beam Therapy Center. HRT plans were designed retrospectively for dosimetric comparisons and risk assessments of radiation-induced complications. HRT led to enhanced target coverage in all patients, along with sparing of critical organs at risk, including a reduction in the brain integral dose by approximately 27%. In terms of estimated risks of radiation-induced complications, HRT led to a reduction in ocular toxicity, cataract development, xerostomia, tinnitus, alopecia and delayed recall. Similarly, HRT led to reduced estimated risks of radiation-induced secondary neoplasms, with a mean excess absolute risk reduction of approximately 30% for secondary CNS malignancies. HRT is a promising modality for advanced JNA, with the potential for enhanced sparing of healthy tissue and thus reduced radiation-induced acute and long-term complications.
PubMed: 38893114
DOI: 10.3390/cancers16111993 -
Journal of Clinical Medicine Jun 2024: The Carlevale lens (FIL SSF, Soleko IOL Division, Italy) is a new lens for suture-less scleral fixation. This paper aimed to systematically review articles on this... (Review)
Review
: The Carlevale lens (FIL SSF, Soleko IOL Division, Italy) is a new lens for suture-less scleral fixation. This paper aimed to systematically review articles on this lens, the surgical techniques used for its implantation, complications and outcomes. : This systematic review was performed following the PRISMA guidelines. The search string used was "Carlevale" AND "scleral fixation" from inception until March 2024. For completeness, either case-control studies, case reports or case series written in English were included. The authors used the Newcastle-Ottawa scale for the case-control studies and the JBI Critical Appraisal Checklist for case reports and case series. : Twenty-nine articles were included. Scleral fixation with Carlevale lens can be performed by creating scleral flaps or, alternatively, by using scleral pockets. The two sclerotomies must be diametrically opposed, and are preferably created by 25-gauge trocars. A pars plana vitrectomy should be performed every time, and the design of the lens should be suitable for self-anchoring to the sclera; the most accredited strategy to achieve this is to avoid scleral sutures. There were only a few intraoperative and postoperative complications reported; vitreous hemorrhages were the most frequent intraoperative events, while the most relevant postoperative complications were vitreous hemorrhages, cystoid macular oedema and transient variations in the intraocular pressure. Excellent results have been obtained in terms of BCVA, IOL centration and stability, mean intraocular pressure, postoperative spherical equivalent, separation between anterior and posterior chamber and the distance of the IOL from anterior chamber structures. : The foldable hydrophilic design of the Carlevale lens has shown good effectiveness, IOL stability and few intra and post-operative complications.
PubMed: 38892997
DOI: 10.3390/jcm13113287