Abstract Background Ciliary body tumor is extremely rare and treatment is challenging. The aim of this study is to present our experience in treating this rare entity, especially large tumors with more than 5 clock hours of involvement, and to evaluate the surgical outcomes and complications of local resection via partial lamellar sclerouvectomy in four cases of ciliary body tumors in China. Methods Four patients with ciliary body tumors underwent partial lamellar sclerouvectomy between October 2019 and April 2023 in Shanghai General Hospital, China. Tumor features, histopathologic findings, complications, visual acuity, and surgical outcomes were reviewed at a mean follow-up of 20.8 months. Results Four patients with a mean age of 31.8 years were included in this study. The histopathological diagnosis was adenoma of non-pigmented ciliary epithelium (ANPCE), schwannoma, and multiple ciliary body pigment epithelial cysts. The mean largest tumor base diameter was 6.00 mm (range: 2.00–10.00) and the mean tumor thickness was 3.50 mm (range: 2.00–5.00). Preoperative complications included cataract in 3 (75%) eyes, lens dislocation in 2 (50%), and secondary glaucoma in 1 (25%). Temporary ocular hypotonia was observed in one case and no other postoperative complications were observed. At a mean follow-up of 20.8 months, the best corrected visual acuity increased in 3 eyes and was stable in 1 eye. Tumor recurrence was absent in all eyes. All patients were alive at the end of follow-up. Conclusions Local tumor resection via PLSU is useful in the treatment of ciliary body tumors, including large tumors occupying more than five clock hours of pars plicata. Surgery-related complications were manageable with adequate preoperative assessment and careful operation during surgery.

Purpose: To evaluate the effectiveness and safety of diazepam in maintaining stable intraoperative blood pressure (BP) in hypertensive patients undergoing vitrectomy under nerve block anesthesia.Methods: A total of 180 hypertensive patients undergoing vitrectomy with nerve block anesthesia were randomized into two groups. The intervention group was given oral diazepam 60 min before operation, while the control group was given oral placebo 60 min before operation. The primary outcome is the effective rate of intraoperative BP control, defined as systolic blood pressure (SBP) during the operation maintained < 160 mmHg at all timepoints. The logistic regression model will be performed to analyze the compare risk factors for ineffective BP control.Results: The effective rate of intraoperative SBP control in the diazepam group was significant higher than that in the placebo group from 15 min to 70 min of the surgery (P < 0.05). The proportion of patients with SBP >= 180 mmHg at any timepoint from operation to 1 h postoperation was higher in the placebo group (12.22%) than in the diazepam group (2.22%) (P = 0.0096). We observed that the change in SBP from baseline consistently remained higher in the placebo group than in the diazepam group. In the logistic regression analysis, age, years of diagnosed hypertension and SBP 1h before surgery were significant risk factors for ineffective BP control.Conclusion: This study provides robust evidence supporting the effectiveness of oral diazepam as a pre-surgery intervention in maintaining stable blood pressure during vitrectomy in hypertensive patients.

AIM: To evaluate the efficacy and safety of perfluoro-n-octane (PFO) for ophthalmic surgery versus F-Octane as an intraoperative tamponade in pars plana vitrectomy (PPV) in management of retinal detachment. METHODS: This multicenter, prospective, randomized, double-masked, parallel-controlled, non-inferiority trial was conducted in three ophthalmology clinical centers in China. Patients with retinal detachment, who were eligible for PPV were consecutively enrolled. Participants were assigned to PFO for ophthalmic surgery or F-Octane for intraocular tamponade in a 1:1 ratio. Best-corrected visual acuity (BCVA), intraocular pressure (IOP) measurement, and dilated fundus examination were performed preoperatively and at 1, 7±1, 28±3d postoperatively. The primary outcome was complete retinal reattachment rate at postoperative day one. The non-inferiority margin was set at 9.8%. The secondary outcomes included intraoperative retinal reattachment rate, and mean changes in IOP and BCVA from baseline to 1, 7±1, 28±3d postoperatively, respectively. Safety analyses were presented for all randomly assigned participates in this study. RESULTS: Totally 124 eligible patients completed the study between Mar. 14, 2016 and Jun. 7, 2017. Sixty of them were randomly assigned to the PFO for ophthalmic surgery group, and 64 were assigned to the F-Octane group. Baseline characteristics were comparable between the two groups. Both groups achieved 100% retinal reattachment at postoperative day one (difference 0, 95%CI: -6.21% to 5.75%, P=1). The pre-defined noninferiority criterion was met. No significant difference was observed in intraoperative retinal reattachment rate (difference 1.77%, P=0.61), mean changes in IOP (difference 0.36, -0.09, 2.22 mm Hg at 1, 7±1, 28±3d postoperatively, with all P>0.05) and BCVA (difference 0.04, -0.02, 0.06 logMAR at 1, 7±1, 28±3d postoperatively, all P>0.05) between the two groups. No apparent adverse events related to the utilization of PFO were reported. CONCLUSION: In patients with retinal detachment undergoing PPV, PFO for ophthalmic surgery is non-inferior to F-Octane as an intraocular tamponade, and both are safe and well-tolerated.

Introduction: This study aimed to compare anatomical outcomes of air and perfluoropropane gas (C3F8) tamponade in pars plana vitrectomy for the treatment of rhegmatogenous retinal detachment (RRD). Methods: In this retrospective study, data were gathered from 578 patients (578 eyes) with RRD. The follow-up records of all 578 patients that underwent primary vitrectomy for RRD with air or C3F8 were examined and analyzed. Surgical outcomes of the two groups were compared. Results: A total of 342 eyes were treated with air and 236 with C3F8. The mean follow-up period was 37.65 ± 2.33 months. Baseline and preoperative clinical characteristics were similar between groups, but the period to intraocular bubble disappearance (p < 0.0001), intraocular pressure on the first postoperative day (p < 0.0001), number of cases with intraocular pressure >21 mm Hg within 3 days post-surgery (p < 0.0001), and the number with intraocular pressure >21 mm Hg during follow-up (p = 0.0002) differed significantly between groups. Primary reattachment rates for air and C3F8 groups were 95.03% and 95.34%, respectively. Clinical characteristics were similar in those with and without successful reattachment, and the frequency of new or unclosed breaks was similar between the two groups. There was no significant difference in two groups according to the presence or absence of inferior retinal breaks and inferior detached quadrants. Univariate and multivariate logistic regression identified no risk factor for surgical failure. Conclusions: Air showed equivalent effects to C3F8, with a shorter period to intraocular bubble disappearance, less risk of postoperative intraocular hypertension, and less expense.

PURPOSE:To compare three different internal limiting membrane (ILM) peeling techniques, including standard ILM peeling, fovea-sparing ILM peeling, and inverted ILM flap (ILMF), in the treatment of myopic traction maculopathy with high risk of postoperative macular hole development.METHOD:This retrospective cohort study enrolled 101 eyes suffering from lamellar macular hole combined with myopic traction maculopathy in 98 consecutive patients who underwent vitrectomy with either standard ILM peeling, fovea-sparing ILM peeling, or ILMF from July 2017 to August 2020. All patients were followed up for at least 12 months after surgery. Best-corrected visual acuity, macular anatomical outcomes, and postoperative full-thickness macular hole (FTMH) formation were evaluated.RESULTS:No significant differences were found among the three surgical groups in baseline characteristics. 12 months after surgery, the mean best-corrected visual acuity was significantly improved ( P < 0.001) and showed no significant differences among groups ( P = 0.452). None of the eyes in the ILMF group, five eyes (15.6%) in the standard ILM peeling group, and six eyes (17.1%) in the fovea-sparing ILM peeling group developed a postoperative FTMH ( P = 0.026). Logistic regression showed that the ILM peeling technique was an independent influencing factor for FTMH formation (OR = 0.209, P = 0.014).CONCLUSION:Compared with the standard ILM peeling or fovea-sparing ILM peeling technique, the ILMF technique resulted in similar visual outcomes but a relatively low incidence of postoperative FTMH in the treatment of lamellar macular hole combined with myopic traction maculopathy. Inverted ILM flap is an effective technique for treating myopic traction maculopathy with high risk of postoperative FTMH development.

As a type of local anaesthesia, retrobulbar nerve block is often used in vitrectomy, with patients remaining conscious during the operation. The increase in systolic blood pressure (SBP) caused by tension and fear during the operation—especially in patients with a history of hypertension—can negatively impact the safety of the procedure, resulting in suprachoroidal haemorrhage or retinal haemorrhage. Diazepam has a sedative effect and can relieve tension during surgery. This study aims to evaluate the efficacy and safety of diazepam for intraoperative BP stabilisation in hypertensive patients under retrobulbar anaesthesia during surgery. This single-centre, double-blind, randomised controlled and parallel clinical trial will include 180 hypertensive patients who will undergo vitrectomy with nerve block anaesthesia. Study participants will be randomly allocated in a 1:1 ratio to intervention (patients receiving oral diazepam before the operation) and control (patients receiving oral placebo before the operation) groups. The primary outcome is the effective rate of intraoperative BP control (systolic BP during operation maintained at <160mmHg at all timepoints). The secondary outcomes are the proportion of patients with SBP ≥180 mmHg at any timepoint from operation to 1 h post-operation, the change of mean systolic blood pressure and mean heart rate during operation from baseline, as well as the number of patients with intraoperative and post-operative adverse reactions within 12 weeks of surgery. The logistic regression model will be performed to compare the outcomes. This study will evaluate the efficacy and safety of diazepam for intraoperative BP stabilisation in hypertensive patients under nerve block anaesthesia during surgery. The results of this trial will reveal whether diazepam has a significant effect on intraoperative BP stability in patients with a history of hypertension who require vitrectomy. If the results of this trial are significant, a large-scale multi-centre clinical trial can be designed. Chinese Clinical Trial Registry (ChiCTR) ChiCTR2100041772 . Registered on 5 January 2021.

INTRODUCTION:The maculopathy in highly myopic eyes is complex. Its clinical diagnosis is a huge workload and subjective. To simply and quickly classify pathologic myopia (PM), a deep learning algorithm was developed and assessed to screen myopic maculopathy lesions based on color fundus photographs.METHODS:This study included 10,347 ocular fundus photographs from 7606 participants. Of these photographs, 8210 were used for training and validation, and 2137 for external testing. A deep learning algorithm was trained, validated, and externally tested to screen myopic maculopathy which was classified into four categories: normal or mild tessellated fundus, severe tessellated fundus, early-stage PM, and advanced-stage PM. The area under the precision-recall curve, the area under the receiver operating characteristic curve (AUC), sensitivity, specificity, accuracy, and Cohen's kappa were calculated and compared with those of retina specialists.RESULTS:In the validation data set, the model detected normal or mild tessellated fundus, severe tessellated fundus, early-stage PM, and advanced-stage PM with AUCs of 0.98, 0.95, 0.99, and 1.00, respectively; while in the external-testing data set of 2137 photographs, the model had AUCs of 0.99, 0.96, 0.98, and 1.00, respectively.CONCLUSIONS:We developed a deep learning model for detection and classification of myopic maculopathy based on fundus photographs. Our model achieved high sensitivities, specificities, and reliable Cohen's kappa, compared with those of attending ophthalmologists.

Purpose: To investigate the characteristics of macular outward scleral height (MOSH) in different grades of myopic tractional maculopathy (MTM) and explore the risk factors for MTM. Methods: A total of 188 eyes (188 participants) with high myopia were divided into the no MTM (nMTM) group and the MTM group, which was further graded into foveoschisis, foveal detachment, full-thickness macular hole, and macular hole with retinal detachment. Swept-source optical coherence tomography was used to measure the MOSH. Results: No significant differences were found in axial length between the nMTM and MTM groups (p = 0.295). The MOSH was significantly higher in the MTM group (p < 0.001), which was identified as a risk factor for MTM (OR = 1.108, p < 0.001). The proportion of eyes with severe atrophic myopic maculopathy (AMM) was higher in the MTM group (28.48%) (p = 0.003). The macular hole with foveoschisis (MH/FS+) subgroup presented a higher average MOSH (p = 0.012) and more severe AMM (p = 0.009) than the macular hole without foveoschisis (MH/FS−) subgroup. Conclusion: MOSH would be more suitable for estimating MTM occurrence than axial length. The grading of AMM helps to evaluate the severity of MTM. The categorization of MH/FS− as a distinct grade from MH/FS+ might be preferable.

Background To investigate the long-term surgical outcomes and prognostic factors of foveal detachment (FD) in pathological myopia. Methods This retrospective observational study included 59 patients with FD (61 eyes) who underwent pars plana vitrectomy at Shanghai General Hospital between June 2017 and July 2018 with follow-up for at least 24 months. Comprehensive ophthalmic examinations, including best-corrected visual acuity (BCVA) and swept-source optical coherence tomography, were assessed. Preoperative myopic maculopathy was evaluated according to the ATN classification. Results FD completely resolved in 59 of 61 eyes (96.7%). Mean duration of retinal reattachment was 12.10 ± 8.10 months. Mean logMAR BCVA improved from 1.34 ± 0.52 to 0.83 ± 0.43 at 24 months postoperatively ( P < 0.001). Secondary macular hole occurred in 8 eyes (13.1%) with a mean period of 3.4 ± 4.1 weeks after primary surgery. In regression analyses, baseline myopic atrophy maculopathy (MAM) ( B = 0.213, P = 0.005) and vitreomacular traction (VMT) ( B = 0.292, P = 0.007) were adverse prognostic factors for postoperative BCVA. A more severe MAM revealed a delay in retinal reattachment ( B = 5.670, P = 0.002). FD eyes with VMT (OR = 1.309, P = 0.003) or outer lamellar macular hole (O-LMH) (OR = 1.369, P < 0.001) were risk factors for postoperative secondary macular hole. Conclusions Vitrectomy was effective in the long-term for treating FD. Careful consideration is needed for those with VMT or O-LMH due to the high risk of secondary macular hole after vitrectomy. FD eyes with more severe MAM tended to have poorer postoperative BCVA and extended periods of retinal reattachment.

Introduction Vitrectomy combined with internal limiting membrane (ILM) peeling, flap or tamponade is widely used in the treatment of macular diseases, such as macular hole (MH) and high myopia macular hole retinal detachment (HMMHRD). However, movement of the ILM to a suitable position to prevent displacement is a difficult operation. Improving visual function after surgery remains controversial. Compared with ILM, the thicker and more flexible lens capsule is easy to obtain and operate. Previous studies have confirmed the effectiveness of lens capsule flap in the treatment of MH. This study aims to evaluate the efficacy and safety of vitrectomy combined with lens capsule flap transplantation in the treatment of HMMHRD.Methods and analysis This single-centre, single-blind, prospective, randomised clinical trial will include 54 patients with HMMHRD who will first undergo phacoemulsification and intraocular lens implantation and then vitrectomy combined with lens capsule flap transplantation (experimental group) or ILM tamponade (control group). Study participants will be randomly allocated in a 1:1 ratio to experimental and control groups. Follow-up will be conducted 1, 3 and 7 days and 1, 3 and 6 months after surgery in both groups. Necessary examinations will be performed at each follow-up visit. Measurement outcomes include postoperative situation of macular hole closure, best-corrected visual acuity, macular retinal function and macular retinal sensitivity. The primary outcome is type I closure rate of MH 6 months after operation. Intergroup comparisons of the proportions of patients with type I closure of MH will be performed with Fisher’s exact test.Ethics and dissemination Full ethics approval for this study was obtained from the Ethics Committee of Shanghai General Hospital, Shanghai Jiaotong University, Shanghai, China. The outcomes of the trial will be disseminated through peer-reviewed journals and at scientific conferences.Trial registration number ChiCTR2200057836.

Additional file 2. RDH5 mutation results.

Purpose: To investigate the morphologic features and identify the risk factors of myopic choroidal neovascularization (CNV). Methods: Eighty-eight eyes of 69 consecutive patients with myopic CNV were included in this study. About 109 eyes of 78 pathologic myopia patients without myopic CNV were randomly selected as the control group. Morphologic features and parameters including scleral thickness (ST), choroidal thickness (CT), posterior staphyloma height and the presence of scleral perforating vessels were obtained and measured by swept-source optical coherence tomography (SS-OCT). Binary logistic regression analysis was performed to identify the risk factors for myopic CNV. Results: Patients with myopic CNV had relatively shorter axial length (P < 0.001) and thicker sclera (P < 0.001) compared to those without. After adjusting age, gender and axial length, thick sclera (OR = 1.333, P < 0.001 per 10-μm increase) and thin choroid (OR = 0.509, P < 0.001 per 10-μm increase) were associated with the presence of myopic CNV. Scleral perforating vessels were detected in the area of myopic CNV in 78.67% of the subjects. Conclusions: A relatively thicker sclera and a thinner choroid are the biological indicators for myopic CNV on SS-OCT. Scleral perforating vessels may also play a pivotal role in the formation of myopic CNV.

Purpose: Takayasu arteritis (TA) is a systemic granulomatous large vessel vasculitis that involves mainly the aorta and its primary branches, and occurs most commonly in young females. Ocular manifestations of TA include small vessels dilation, microaneurysm, arteriovenous anastomosis, retinal ischemia and retinopathy. However, no specific and effective treatments for Takayasu retinopathy is applied. This case aimed to demonstrate the role of anti-VEGF (vascular endothelial growth factor) therapy in treating Takayasu retinopathy. Observations: We herein reported an 18-year-old Asian woman who presented with typical wreath-like arteriovenous anastomosis around the disc in the right eye and vitreous hemorrhage in the left eye. The stenosis and occlusion of bilateral subclavian arteries, carotid arteries and other proximal arteries on angiography confirmed the diagnosis of TA. Meanwhile, elevated ESR and CRP revealed that TA was in the active stage. We applied anti-VEGF therapy in treating Takayasu retinopathy specially to inhibit neovascularization. Additionally, vitreous extraction was conducted in the left eye after the treatment of anti-VEGF therapy. Conclusions and importance: This is the first report of effective application of anti-VEGF therapy in inhibiting wreath-like arteriovenous anastomosis and improving vitrectomy in TA.

Background Retinopathy of prematurity (ROP), a major cause of significant visual morbidity and blindness in preterm infants, is closely related to pathological angiogenesis. The aim of the study is to evaluate the effect of a new 12-aa peptide (named peptide CW-703) from human insulin-like growth factor-2, against angiogenesis in ROP. Methods In order to evaluate the inhibitory effect of CW-703 on the proliferation, migration, tube formation and apoptosis of human umbilical vein endothelial cells (ScienCell) in vitro, we used MTS assays, a modified Boyden chamber, Matrigel system and TUNEL assays. Effects in vivo were assayed using chorioallantoic membrane assays and oxygen-induced retinopathy (OIR) models in mice. We also performed eletrophysiological and histologic examinations to evaluate the possible toxicity of the peptide. Real-time PCR, ELISA and western blotting were used to elucidate the mechanism of CW-703. Results CW-703 inhibited angiogenesis in vitro by suppressing endothelial cell proliferation, migration and tube formation. CW-703 also prevented angiogenesis in chicken chorioallantoic membrane assays and OIR assays in mice. No evident functional or morphologic abnormalities in neuroretina after CW-703 injection were revealed in electrophysiological tests and histological examinations. Moreover, we elucidated that CW-703 competed for binding to IGF-1R and inhibited angiogenesis by inhibiting IGF-1R/PI3K/AKT activation and downregulating vascular endothelial growth factor expression. Conclusion The novel peptide CW-703 may act as an effective inhibitor of ocular pathologic angiogenesis, especially in treating ROP.

PURPOSE:To identify the clinical characteristics and pathogenic genes among a Chinese family with angioid streaks and to assess a novel splicing mutation at the transcriptional and translational levels.METHODS:Consenting family members were clinically evaluated, and blood samples were collected for targeted exome capture sequencing and/or Sanger sequencing. The two affected siblings were assessed by multimodal fundus imaging. ABCC6 splicing patterns were analysed by RNA identification and quantification using the proband's peripheral blood mononuclear cells. Minigene experiments were performed to verify the university. Plasmids expressing the transcripts were transfected into HEK293 cells to assess protein translation. Bioinformatic analyses were also performed to predict the splicing patterns and the functional consequences of the mutation.RESULTS:The two siblings were trans-compound heterozygous pseudoxanthoma elasticum (PXE) patients with the same genotype (c.3703C>T and c.1177-2A>G for ABCC6) but different phenotypes. We identified several ABCC6 alternative splicing transcripts that were not previously reported. The novel splicing mutation c.1177-2A>G led to the upregulation of three transcripts, one using a cryptic splice acceptor in the upstream region of the intron, another using a cryptic splice acceptor in the downstream exon, and the third stimulating non-canonical U12-type splicing. All the transcripts were successfully translated in vitro.CONCLUSION:The genotype-phenotype correlation of PXE is poorly understood. The novel ABCC6 splicing mutation c.1177-2A>G results in multiple splicing patterns. Endogenous U2 to U12 conversion may occur in humans in a disease state. Peripheral blood mononuclear cells can be reliably used to study ABCC6 RNA.

Many diseases are associated with angiogenesis. Therefore, inhibition of angiogenesis is deemed as a treatment for those diseases. To date, many angiogenesis inhibitors are from large plasma proteins. In this study we identified a 21-aa peptide (named peptide ZY1) from human placenta growth factor-1 and it may serve as a potent angiogenesis inhibitor. Our study demonstrated ZY1 inhibited angiogenesis in vitro by suppressing proliferation, migration and tube formation of HUVECs. The in vivo inhibition activity of ZY1 was observed in chicken chorioallantoic membrane assays and tumor-bearing mouse models. Moreover, we found ZY1 inhibited angiogenesis by decreasing the expression of HIF-1β and subsequently reducing its downstream molecule VEGF. In conclusion, peptide ZY1 can inhibit angiogenesis and may serve as an anti-angiogenesis drug candidate.

Objective. To observe the fellow eye in patients undergoing surgery on one eye for treating myopic traction maculopathy. Methods. 99 fellow eyes of consecutive patients who underwent unilateral surgery to treat MTM were retrospectively evaluated. All patients underwent thorough ophthalmologic examinations, including age, gender, duration of follow-up, refraction, axial length, intraocular pressure, lens status, presence/absence of a staphyloma, and best-corrected visual acuity (BCVA). Fundus photographs and SD-OCT images were obtained. When feasible, MP-1 microperimetry was performed to evaluate macular sensitivity and fixation stability. Results. At an average follow-up time of 24.7 months, 7% fellow eyes exhibited partial or complete MTM resolution, 68% stabilized, and 25% exhibited progression of MTM. Of the 38 eyes with “normal” macular structure on initial examination, 11% exhibited disease progression. The difference in progression rates in Groups 2, 3, and 4 was statistically significant. Refraction, axial length, the frequency of a posterior staphyloma, chorioretinal atrophy, initial BCVA, final BCVA, and retinal sensitivity all differed significantly among Groups 1–4. Conclusions. Long axial length, chorioretinal atrophy, a posterior staphyloma, and anterior traction contribute to MTM development. Patients with high myopia and unilateral MTM require regular OCT monitoring of the fellow eye to assess progression to myopic pre-MTM. For cases exhibiting one or more potential risk factors, early surgical intervention may maximize the visual outcomes.