Retinoblastoma (RB) is the most common pediatric intraocular malignancy, yet the role of N6-methyladenosine (m6A) regulators in RB progression remains unclear. This study investigated the function and mechanism of the m6A reader YTHDC1 in RB invasion. The RNA-sequencing dataset GSE97508 from Gene Expression Omnibus was analyzed to identify differentially expressed m6A regulators between invasive and non-invasive RB. Their expression was next validated by RT-qPCR, Western blot, and immunohistochemistry. Functional assays including CCK-8, EdU, and Transwell assays were performed to evaluate the effects of YTHDC1 knockdown or overexpression on RB cells in vitro and in vivo. SQSTM1 was predicted to be a downstream target by bioinformatics and experimental validation. RNA stability and RIP-qPCR assays were performed to examine the regulation of SQSTM1 mRNA by YTHDC1. Autophagic flux was evaluated by LC3B Western blotting and mCherry-GFP-LC3B fluorescence imaging. YTHDC1 was significantly downregulated in invasive RB. YTHDC1 knockdown enhanced, whereas its overexpression suppressed, RB cell proliferation and invasion. YTHDC1 promoted SQSTM1 expression by stabilizing its mRNA. Knockdown of SQSTM1 in RB cells promoted cell proliferation, migration, and invasion, and partially counteracted the inhibition of cell function caused by YTHDC1 overexpression. Knockdown of YTHDC1 or SQSTM1 enhanced autophagic flux, and knockdown of SQSTM1 led to a substantial reduction of the mTOR pathway activity. This study highlights the critical role of YTHDC1 and SQSTM1 in the invasive progression of RB. Their downregulation promoted autophagy in RB cells, providing preliminary evidence of their involvement in RB progression.
Background: Nasal microbiota is a key factor in the development of chronic rhinosinusitis (CRS). Its influence on orbital autoimmune diseases remains unknown. This study aims to investigate the influence of CRS-related nasal dysbiosis on extraocular muscle (EOM) microbiota in patients with or without idiopathic orbital myositis (IOM).Methods: Sixteen subjects were recruited, including 4 cases in each study group [IOM(+) CRS(+) group, IOM(–) CRS(+) group, IOM(+) CRS(–) group, IOM(–) CRS(–) group]. The EOM tissues and corresponding incision tissues were sampled and processed with metagenomic next-generation sequencing (mNGS). The nasal swaps from middle meatus were collected and analyzed with 16s rRNA sequencing. The sequencing data were calculated with Shannon index for microbial diversity, principal component analysis for microbial abundance and linear discriminant analysis effect size for dominant species.Results: A total of 42 species of bacteria, 3 species of fungi and no virus were detected. Both microbial diversity and microbial abundance differed between the EOM and incision samples in IOM(–) CRS(+) and IOM(–) CRS(–) subjects, with Staphylococcus predominant in the EOM samples and Micrococcus luteus predominant in the incision samples. The microbiota also differed between EOM and swap samples in the IOM(–)CRS(+) subjects, and the most distinguishable species in the swab samples was Cutibacterium acnes. Further analysis of the swab samples confirmed nasal dysbiosis in the CRS(+) subjects with Staphylococcus haemolyticus representating as the dominant species. The microbiota of EOM samples differed between the IOM(–)CRS(+) and IOM(–)CRS(–) subjects, resulting into preponderance of Cutibacterium acnes in the IOM(–)CRS(+) subjects. Small discrepancies of EOM microbiota were detected between the IOM(+)CRS(+) and IOM(+)CRS(–) subjects, but data analysis showed no statistical significance. The above findings demonstrate an alteration of EOM microbiota in CRS patients, providing evidence that nasal dysbiosis may play a role in regulating microbial communities in EOM.Conclusions: This study provided a reference spectrum of EOM microbiota in IOM patients and offered insights into the nasal microbiota’s influence on orbital autoimmune conditions.(Trial registration: Chinese Clinical Trial Registry, ChiCTR2100047731. Registered 24 June 2021 - Retrospectively registered, https://www.chictr.org.cn/showproj.aspx?proj=127896)
Orbital solitary fibrous tumor is extremely rare, and the therapeutic role of radiotherapy remains poorly defined. This study aimed to compare long-term prognosis between surgery plus radiotherapy and surgery alone in complicated orbital solitary fibrous tumor (OSFT), thereby providing evidence-based insights for clinical management. In this retrospective cohort study, we analyzed OSFT patients treated over a decade (January 2013-December 2023). Inclusion criteria encompassed cases with tumor recurrence or histopathological confirmation of anaplastic /malignant variants. By excluding benign cases without recurrence, we could specifically examine the impact of radiotherapy on disease control in complicated OSFTs. Clinico-demographic parameters including age, gender, histopathological subtype, surgical intervention, and radiotherapy administration were collected. Patients were stratified into two treatment arms: surgery alone (n = 19) versus surgery with adjuvant radiotherapy (n = 15). Kaplan-Meier methodology was employed to evaluate recurrence-free survival (RFS) and overall survival (OS), with between-group comparisons using log-rank tests. Cox proportional hazards models were implemented for prognostic factor analysis. From 60 screened OSFT cases, 34 met inclusion criteria: 13 anaplastic, 11 malignant, and 10 recurrent tumors at presentation. The surgery combining radiotherapy cohort demonstrated superior 3-year (88.9
Background:Early lacrimal sac neoplasms are often misdiagnosed as lacrimal duct obstruction, delaying appropriate treatment, accelerating tumor progression, and worsening prognosis. By systematically reviewing and summarizing these characteristics, we aimed to assist ophthalmologists and otolaryngologists in accurately diagnosing and managing early-stage lacrimal sac neoplasms. Methods:In this retrospective study, patients presenting with epiphora were diagnosed with either lacrimal sac neoplasms or mucoceles. Diagnostic assessments included computed tomography (CT), enhanced magnetic resonance imaging (MRI), and pathological analysis. Results:The study comprised 9 patients (2 male, 7 female) with lacrimal sac neoplasms; 44% had malignancies, and the remaining 56% exhibited benign conditions. There was no statistically significant difference in mean tumor volume or margin characteristics between patients with neoplasms and those with mucoceles. There was no statistically significant difference in the mean width of the nasolacrimal canal between patients with neoplasms (6.49±3.68 mm) and mucoceles (5.50±1.88 mm) (P=0.44). However, the depth of the nasolacrimal canal was significantly greater in the neoplasm group (5.08±3.21 mm) compared to the mucocele group (2.85±0.96 mm) (P=0.042). Notably, lacrimal sac mass extended above the medial canthal tendon in 8 neoplasm patients, unlike any in the mucocele group, demonstrating distinct pathological presentations. Additionally, the diseased eyes in the neoplasm group showed significantly greater nasolacrimal canal width and depth compared to the contralateral eyes (all P<0.05), whereas the mucocele group showed only slightly increased nasolacrimal canal width compared with the contralateral eyes. Conclusions:The findings suggest that patients with a lacrimal sac mass extending above the medial canthal tendon and a nasolacrimal canal deeper than the contralateral side should undergo contrast-enhanced MRI for accurate diagnosis and treatment planning.
The prevalence of vision disorders and eye cancer risk, driven by global population aging, necessitates precise ophthalmological disease screening, yet standardized and robust approaches for risk stratification remain absent. Here, we present SonoEye, a vision-language ultrasound foundation model for eye disease risk stratification, pre-trained on 215,356 image-text pairs from 70,452 patients using contrastive learning and fine-tuned with consensus-confirmed patient-level labels. The model achieves 98.3% screening sensitivity and a mean accuracy of 96.3% in differentiating 18 diseases. We established the Eye Reporting and Data System (Eye-RADS), a 4-tier risk stratification framework (normal, low-vision risk, high-vision risk, and tumor risk), demonstrating excellent Cohen's kappa agreement across internal (0.808) and external (0.677-0.685) test cohorts. Notably, incorporating age significantly improved Eye-RADS performance in elderly but not younger populations, indicating age-related features in eye care. Through image-text alignment, SonoEye generates structured clinical reports with interpretable attention-based visualizations, advancing automated vision risk stratification, particularly for aging populations in resource-limited settings.
Background:The optimal treatment approach for lacrimal gland adenoid cystic carcinoma (LGACC) remains controversial. Objectives:We aim to demonstrate the value of radiotherapy (RT) in the multidisciplinary treatment of LGACC. Design:This was a retrospective cohort study. Methods:This study was conducted on 90 patients with LGACC treated from 2002 to 2023. We compared the overall survival (OS), progression-free survival (PFS), recurrence-free survival (RFS), and distant metastasis-free survival (DMFS) between those treated with surgery alone versus surgery plus postoperative radiotherapy (PORT). In addition, the ipsilateral visual outcome and ocular complications were evaluated. Results:The addition of radiotherapy significantly improved the 5-year RFS (63.4% vs 31.1%, p = 0.014) and PFS (56.8% vs 31.1%, p = 0.036) while showing no improvement in OS (79.7% vs 85.6%, p = 0.98) and DMFS (67.3% vs 70.1%, p = 0.70) compared to surgery alone. Further analysis indicated that, compared to the photon-RT group, the proton/carbon-ion group showed no significant differences in 5-year OS (p = 0.7), RFS (p = 0.37), PFS (p = 0.45), and DMFS (p = 0.57). Univariate (odds ratio (OR) = 0.14, 95% confidence interval (CI): 0.04-0.49, p = 0.002) and multivariate (OR = 0.16, 95% CI: 0.04-0.62, p = 0.008) logistic regression indicated that radiotherapy was a protective factor for local recurrence. Furthermore, among the long follow-up of 35 patients accepting eye-sparing surgery plus PORT, 13 patients (37.1%) had best-corrected visual acuity (⩾20/40) and 14 (40.0%) had severe vision loss (<20/200); furthermore, dry eye disease (100%, 35/35) and cataract progression (45.7%,16/35) are the most common ocular complications. Conclusion:Surgery plus radiotherapy is a safe and effective multidisciplinary treatment in improving local control with tolerable long-term ocular toxicity, but of limited impact on OS for LGACC.
Background This case presents a rare form of thyroid-associated ophthalmopathy with idiopathic choroidal folds, unlinked to optic neuropathy—an unusual finding in thyroid-related eye disease. Instead, bilateral nuclear cataracts and lens dislocation were identified as the primary causes of vision loss, emphasizing the importance of considering multiple, less common contributors in such cases. The decision to begin with cataract surgery exemplifies a tailored, effective approach to multifactorial vision loss, offering insights for cases with overlapping ocular issues. Given recent associations between COVID-19 vaccination and ocular symptoms like choroidal folds and scleritis, posterior scleritis was considered a possible explanation. This underscores the need for further investigation into atypical presentations of thyroid-associated eye disease. Case presentation: A 54-year-old male developed bilateral vision loss and choroidal folds one year following COVID-19 vaccination. Initially diagnosed with thyroid-associated ophthalmopathy, he underwent an adequate course of steroid pulse therapy with no visual improvement. Further assessment revealed bilateral cataracts and lens dislocation. Computed tomography identified mild extraocular muscle thickening but no orbital apex crowding, ruling out compressive optic neuropathy. Lens surgery subsequently restored his vision and normalized his visual fields. However, two months postoperatively, he experienced recurrent blurred vision and restricted movement in the right eye. Magnetic resonance imaging confirmed extraocular muscle enlargement, leading to a diagnosis of compressive optic neuropathy, and tocilizumab therapy was initiated. Following treatment, the patient demonstrated improved vision, reduced muscle thickness, and enhanced motility. Conclusions This case suggests that initial choroidal folds may be due to a combination of vaccine-related side effects and elevated orbital pressure, underscoring the potential for COVID-19 vaccination to cause atypical ocular symptoms.
BackgroundMany patients with uveal melanoma (UM) cannot receive laboratory analysis due to eye-preserving treatment, biopsy risks or costs. The study is to evaluate correlation between tumor shape and a series of metastasis risks in UM, and to assess the predictive value of tumor shape classification.MethodsFour hundred thirty-nine UM patients undergoing enucleation were included in the study. Standardized echography was utilized to document selected tumor characteristics. Tumors were categorized into five distinct shape groups: mushroom, dome, lobulated, diffuse, and irregular. Clinical data, tumor thickness, largest basal diameter (LBD), American Joint Committee on Cancer (AJCC) stage, pathological results, and survival status were collected and comparatively analyzed across the different tumor shapes. Survival analysis was carried out with both Cox hazard regression and Kaplan–Meier log rank test.ResultsThe 439 UM cases were classified as mushroom in 164 (37.4%), dome in 129 (29.4%), lobulated in 62 (14.1%), diffuse in 11 (2.5%) and irregular in 73 (16.6%). Significant differences were observed in tumor thickness, LBD, cell type, ciliary body involvement (CBI), extraocular extension, and AJCC stage across these shape categories. Regardless of tumor size and AJCC stage, mushroom-shaped melanoma exhibited the most favorable prognosis, irregular-shaped melanoma demonstrated the worst prognosis.ConclusionTumor shape could be defined noninvasively and dependably using echography. Shape classification in UM provides an independent variable to improve the clinical prognostication.
To quantitatively investigate the pathological subtypes of lacrimal gland adenoid cystic carcinoma (LGACCs), the tumor immune microenvironment in each pathological subtype, and the relation to survival. In this retrospective study, the tumor subtype was determined by H E staining. Multiplex immunochemistry was performed to define specific immune cells. The tumor immune microenvironment (TIME) was sketched by sequential image scanning and reconstructed by a cytometry platform. Eighteen patients with adequate paraffin blocks diagnosed with LGACC from 2012 to 2021 were included in this study. Thirteen patients out of the eighteen patients (72.2
To compare the narrowest diameter of the bony nasolacrimal canal (BNLC) in a Chinese population with and without primary acquired nasolacrimal duct obstruction (PANDO). Eighty-one patients with PANDO and forty-two non-PANDO patients underwent computed tomography (CT) covering the full length of their nasolacrimal canals. The narrowest transverse diameters of the BNLC were measured in axial CT images. The narrowest BNLC diameter was significantly shorter in PANDO patients than non-PANDO patients (4.20 ± 0.87 mm vs. 4.95 ± 0.82 mm, p < 0.001), especially in patients aged 40 years and older (4.20 ± 0.84 mm vs. 5.11 ± 0.83 mm, p < 0.001). When males and females were analyzed separately, the differences between PANDO patients and controls remained significant (4.32 ± 0.82 mm vs. 5.70 ± 0.64 mm, p < 0.001, male; 4.18 ± 0.88 mm vs. 4.74 ± 0.75 mm, p < 0.001, female). Generally, in the Chinese population, males have a larger minimum transverse diameter compared to females (5.70 ± 0.64 mm vs. 4.74 ± 0.75 mm, p < 0.001). Furthermore, the narrowest transverse diameter of the BNLC remarkably increased with age (r = 0.432, p < 0.0001). BNLC stenosis may be an important etiological factor for PANDO in the Chinese population.
ObjectivesTransforming growth factor β1 (TGFβ1)-interleukin 11 (IL11) is a newly found critical signaling pathway in fibrotic diseases such as Graves’ orbitopathy (GO). It has now been confirmed that enhanced glycolysis plays a key role in the pathogenesis of GO. However, little is known about the relationship between glycolysis and IL11-mediated fibrosis in GO. This study aimed to identify the relationship between glycolysis and TGFβ1-IL11 signaling pathway and investigate the role of IL11 in glycolysis-facilitated fibrosis in GO.MethodsOrbital connective tissues were collected from GO and control patients. Primary orbital fibroblasts (OFs) were cultured from clinical tissues. Patient-derived xenografts were established via intraorbital transplantation of GO orbital tissue in humanized NCG mice. Protein levels were measured using Capillary Western Immunoassay (WES). Small interfering RNA (siRNA) was used to construct transfected OF strains. Lactate production was measured to assess glycolysis status. Animal models were assessed by T2-weighted magnetic resonance (MR) scan. Immunohistochemistry staining was applied to patients’ orbital connective tissues.ResultsOrbital connective tissues were collected from GO patients. Immunohistochemical (IHC) staining of GO tissues revealed the phenomenon of pyruvate dehydrogenase kinase 2 (PDK2)-enhanced glycolysis and upregulated IL11-IL11Rα pathway. In vitro experiments showed successful induction of fibrosis of patient-derived orbital fat/connective tissues, which could be alleviated by dichloroacetic acid (DCA). MRI images and analysis of hematoxylin and eosin (HE) and Masson-stained section demonstrated enhanced glycolysis in GO, facilitating fibrosis of the orbital tissue. Targeting PDK2 decreased IL11 expression to suppress fibrosis. In vivo experiment confirmed anti-fibrotic effect of inhibition of glycolysis.ConclusionsPDK2-enhanced glycolysis exacerbates fibrosis via IL11-IL11Rα signaling pathway, shedding light on a potential therapeutic role of metabolic modulators such as DCA in GO treatment.
OBJECTIVE:To evaluate the effectiveness of administering additional intravitreal topotecan injections (plus two) in reducing the recurrence rate in patients with advanced retinoblastoma (RB). METHODS AND ANALYSIS:This retrospective, non-comparative and interventional study encompassed 26 eyes of 25 patients with RB, treated between January 2019 and April 2024. All eyes in this series had received previous intravenous chemotherapy and/or intra-arterial chemotherapy. Patients received intravitreal topotecan (30 µg/0.1 mL) and/or intravitreal melphalan (20-30 µg/0.1 mL) injections every 3-4 weeks until vitreous seeds regression was achieved. Subsequently, two additional injections of topotecan (30 µg/0.1 mL) were administered after complete regression to consolidate subclinical residuals. The control of vitreous seeds, ocular toxicity assessment and clinical characteristics was observed. RESULTS:The median age of the patients was 26 months, with 57.7% belonging to group D and 42.3% to group E of RB classification. A total of 111 injections were administered in 26 eyes, with a median of 4 injections per eye. Complete vitreous seeds regression was achieved in 100% of eyes, and eye salvage was successful in 24 of 26 eyes (92.3%). There were no recurrences of vitreous seeds. Enucleation was performed in two eyes: one due to phthisis bulbi and one due to optic nerve tumour recurrence. No cases of systemic metastasis or death were reported. CONCLUSION:Administering additional intravitreal topotecan injections (plus two) following the complete regression of vitreous seeds may contribute to reducing the recurrence rate in patients with RB. This approach enhances the long-term efficacy of intravitreal injections, thereby contributing to higher eye salvage rates.However, the retrospective, non-randomised design and small sample size remain limitations. Multicentre prospective studies are warranted to validate these findings and refine treatment protocols.
PURPOSE:To explore the levels of cytokines in the aqueous humor (AH) of patients diagnosed with retinoblastoma (RB) who received intravitreal melphalan (IVM). METHODS:A total of 28 eyes from 27 patients diagnosed with RB who received IVM were enrolled, along with 14 eyes from 14 patients with congenital cataracts as controls. The clinical features of the patients were recorded before and after IVM. 27 cytokines concentrations in AH samples collected from these patients were measured by a suspension array system. RESULTS:The levels of IL-8, IL-17A, Eotaxin, G-CSF, and PDGF-BB increased significantly following IVM administration in RB patients (p<0.05). Post IVM treatments, the levels of IL-5, IL-8, IL-9, IL-12p70, IL-15, IL-17A, G-CSF, IP-10, MIP-1α, PDGF-BB, TNF-α, and VEGF were higher in eyes that developed ocular toxicity than those eyes didn't (p<0.05). The ocular toxicity group received a higher cumulative dose and mean per dose of intravitreal melphalan. The elevated cytokines subsequent to IVM were associated with the number of treatments and cumulative dose of intravitreal melphalan. CONCLUSIONS:The results revealed a notable increase in cytokine levels subsequent to IVM interventions. Elevated cytokine concentrations in the eyes of RB patients post-IVM may be associated with ocular toxicities.
Introduction:Tocilizumab (TCZ) has been demonstrated to be effective in treating thyroid-associated ophthalmopathy (TAO); however, its efficacy in hormone-resistant dysthyroid optic neuropathy (DON) remains unclear. This study aims to identify baseline and post-treatment indicators that can predict the necessity for surgical intervention following TCZ therapy. Methods:Thirty-one hormone-resistant DON patients treated with TCZ were categorized into surgery (n=15, 7 males, 8 females) and non-surgery (n=16, 8 males, 8 females) groups based on their post-TCZ surgical status. Retrospective comparisons between the two groups were performed using pre- and post-treatment ophthalmic assessments, biomarkers, and orbital magnetic resonance imaging (MRI) scans. The predictive value of identified variables was evaluated through receiver operating characteristic (ROC) curve analysis and logistic regression analysis. Results:The surgery group exhibited a significantly higher baseline thyroid-stimulating hormone receptor antibody (TRAb) (P=0.001) and a positive change in the maximal signal intensity ratio of extraocular muscle to temporalis muscle (SIR(EOM/temporalis)MAX), whereas the non-surgery group demonstrated a negative change (P<0.001). A TRAb cut-off value of ≤5.07 IU/L predicted non-surgery with 93.3% sensitivity and 81.2% specificity, while a SIR(EOM/temporalis)MAX cut-off value of ≤-1.83 had 86.7% sensitivity and 87.5% specificity. The area under the curve was 0.846 for TRAb and 0.863 for SIR(EOM/temporalis)MAX. Multivariate regression confirmed SIR(EOM/temporalis)MAX change (P=0.017) as an independent predictor of surgical intervention. Linear regression revealed a significant correlation between SIR(EOM/temporalis)MAX change and TCZ dosage in the non-surgery group (P=0.005), but not in the surgery group. Discussion:The percentage change in SIR(EOM/temporalis)MAX following TCZ treatment and baseline TRAb can serve as predictors of the necessity for surgical intervention in hormone-resistant DON. These indicators assist in the personalization of TCZ therapy and in the avoidance of unnecessary surgical procedures.
The manuscript reports a unique case of untreated choroidal melanoma with obvious exudation, easily confused with pigment epithelial adenomas.
PurposeThis study aimed to investigate the cytokine profile in the aqueous humor of uveal melanoma (UM) and explore the role of cytokines in tumor progression and radiation response.MethodsAqueous humor samples were collected from patients with UM who had undergone enucleation or radiotherapy. Cytokine levels in these samples were measured using the Human 48-Plex Luminex assay, and statistical analyses were performed to evaluate the correlations with clinicopathological parameters.ResultsThirty-six patients with UM were enrolled in this study: 17 in the enucleation group and 19 in the radiotherapy group. Significant differences in cytokine levels were observed between the two groups. The enucleation group exhibited higher levels of basic fibroblast growth factor (basic-FGF), interleukin-2 (IL-2), interleukin-2 receptor alpha (IL-2Rα), interleukin-12 subunit p40 (IL-12(P40)), macrophage colony-stimulating factor (M-CSF), macrophage migration inhibitory factor (MIF) than the radiotherapy group. Correlation analysis revealed significant associations between several cytokines and clinicopathological features, including tumor height, diameter, and treatment strategy. Tumor height was correlated with cytokines such as interleukin-8 (IL-8), M-CSF, and stem cell growth factor-beta (SCGF-β), while tumor diameter showed negative correlations with beta-nerve growth factor (β-NGF) and tumor necrosis factor-beta (TNF-β). Cytokines, including basic-FGF, IL-2, and MIF, were positively associated with radiation complications, while cytokines such as β-NGF, interleukin-12 subunit p70 (IL-12(P70)), and vascular endothelial growth factor (VEGF) exhibited negative correlations with post-radiation duration. Interleukin-5 (IL-5) was the only cytokine linked to subretinal fluid, and multiple cytokines, such as M-CSF and MIF, were correlated with the tumor cell type. Multiple regression analysis confirmed that these cytokines, including interferon-gamma (IFN-γ), interleukin-1 receptor antagonist (IL-1ra) and VEGF, were significantly associated with tumor characteristics and treatment response.ConclusionThese findings suggest the potential role of cytokines in the tumor microenvironment of UM. The analysis points to these cytokines as possible biomarkers for tumor progression and treatment response. Further exploration of cytokines in the aqueous humor may enhance our understanding of UM and provide insights for managing post-treatment complications.
Background To examine the clinical features and imaging findings of early-stage lacrimal sac neoplasms. Methods In this retrospective study, patients presenting with epiphora were diagnosed with either lacrimal sac neoplasms or mucoceles. Diagnostic assessments included computed tomography (CT), enhanced magnetic resonance imaging (MRI), and pathological analysis. Results The study comprised nine patients (2 male; 7 female) with lacrimal sac neoplasms, 44% of whom had malignancies while 56% exhibited benign conditions. There was no statistically significant difference in the mean width of the nasolacrimal canal between patients with neoplasms (6.49 ± 3.68 mm) and mucoceles (5.50 ± 1.88 mm, P = 0.44). However, the depth of the nasolacrimal canal was significantly greater in the neoplasm group (5.08 ± 3.21 mm) compared to the mucocele group (2.85 ± 0.96 mm, P = 0.05). Notably, lacrimal sac mass extended above the medial canthal tendon in eight neoplasm patients, unlike any in the mucocele group, demonstrating distinct pathological presentations. Additionally, the diseased eyes in the neoplasm group showed significantly greater nasolacrimal canal width (6.49 mm) compared to the contralateral eyes (3.01 mm, P = 0.01) and a slightly deeper depth (5.08 mm vs. 2.66 mm, P = 0.04). Conclusions Findings suggest that a lacrimal sac mass extending above the medial canthal tendon warrants a CT scan. Patients with a nasolacrimal canal wider than the contralateral side should undergo enhanced MRI for accurate diagnosis and treatment planning.
PurposeTo evaluate changes in retinal microvascular density and choroidal vascularity in patients with retinoblastoma (RB) after intra-arterial chemotherapy (IAC).DesignRetrospective clinical cohort study.MethodsThis study included 12 unilateral RB eyes treated with IAC (RB tumour), 12 contralateral normal eyes (RB fellow), and 12 healthy controls. The macular retinal thickness and retinal microvascular structure, including foveal avascular zone (FAZ) area, the macular and peripapillary superficial vessel density (SVD) and deep vessel density (DVD), were measured by optical coherence tomography angiography (OCTA). The choroidal thickness (ChT) and choroidal vascularity, including total choroidal area (TCA), luminal area (LA), stromal area (SA) and Choroidal Vascularity Index (CVI), were measured by spectral-domain optical coherence tomography (SD-OCT). A comparison among the three groups was conducted, while the correlations among the parameters were analyzed.ResultsBetween the three cohorts, the foveal retinal thickness, the SVD, DVD, ChT, TCA, LA, SA and CVI were significantly lower in RB tumour compared to RB fellow and the control eyes (all P<0.01). There were no significant differences in the parameters between the contralateral and control eyes. The correlation analyses indicated a significant negative correlation between the total melphalan dose and foveal and parafoveal DVD, ChT, and LA.ConclusionsThe retinal microvascular density and choroidal vascularity were lower in unilateral RB treated with IAC, and seemed to be related to the total melphalan dose. Moreover there were no measurable changes in the contralateral eyes.
Importance:Adjuvant therapy is an important and effective treatment for retinoblastoma. However, there is a lack of head-to-head clinical trials comparing 3 vs 6 cycles of CEV chemotherapy (carboplatin, etoposide, and vincristine) for enucleated unilateral retinoblastoma with high-risk pathological features. Objective:To assess whether 3 cycles of CEV chemotherapy is noninferior to 6 cycles for enucleated unilateral retinoblastoma with high-risk pathological features. Design, Setting, and Participants:This double-center, randomized, open-label, noninferiority trial was conducted at 2 premier eye centers in China and included 187 patients who had undergone enucleation for unilateral retinoblastoma with high-risk pathological features (massive choroidal infiltration, retrolaminar optic nerve invasion, or scleral infiltration) between August 2013 and March 2024. The final date of follow-up was March 21, 2024. Interventions:Patients were randomly assigned to receive either 3 (n = 94) or 6 (n = 93) cycles of CEV chemotherapy regimen after enucleation. Main Outcomes and Measures:The primary end point was disease-free survival, with a noninferiority margin of 12%. Secondary end points encompassed overall survival, safety, economic burden, and the quality of life of children. Results:All 187 patients (median [IQR] age, 25.0 [20.0-37.0] months; 83 [44.4%] female) completed the trial. Median (IQR) follow-up was 79.0 (65.5-102.5) months. Five-year disease-free survival was 90.4% for the 3-cycle group vs 89.2% for the 6-cycle group (difference, 1.2% [95% CI, -7.5% to 9.8%]), which met the noninferiority criterion (P = .003 for noninferiority). The 6-cycle group experienced a higher frequency of adverse events, greater reduction in quality of life scores, and increased costs compared with the 3-cycle group. Conclusions and Relevance:Among patients with unilateral pathologic high-risk retinoblastoma, 3 cycles of CEV chemotherapy resulted in 5-year disease-free survival that was noninferior to 6 cycles of CEV chemotherapy. Trial Registration:ClinicalTrials.gov Identifier: NCT01906814.