Objective: To evaluate whether the nailfold microcirculation is associated with retinal microcirculation in healthy subjects. Methods: Fifty subjects without systematic and ocular diseases were enrolled. Thickness of peripapillary retinal nerve fiber layer (RNFL), vessel density (VD) of radial peripapillary capillaries (RPCs), and superficial capillary VD in macular zone were measured with optical coherence tomography angiography (OCTA) in left eyes. Nailfold microcirculation, including capillary density, avascular zones, dilated capillaries, and hemorrhages was examined on the fourth digit of each subject’s non-dominant (left) hand with nailfold capillaroscopy (NFC). Results: After adjustment for relatively systemic factors, multivariate regression analyses showed a significant direct relationship between RNFL thickness and nailfold capillary density (OR = 1.09; p = 0.046). RNFL thickness and RPCs VD were negatively correlated with nailfold avascular zones (OR = 0.855; p = 0.007; OR = 0.596; p = 0.010). Superficial VD of parafovea was negatively associated with dilated nailfold capillaries (OR = 0.794; p = 0.012). Conclusion: In healthy subjects, nailfold capillary lower density and abnormalities are associated with reduced RNFL thickness and retinal VD. The results provide a theoretical foundation for relevant studies on ocular diseases with microvascular abnormalities and could contribute to pathogenesis understanding in the future. NFC and OCTA have the potential to identify risk factors and improve accuracy of the early diagnosis and treatment of ocular diseases, even systemic diseases with any microvascular component in clinical practice. Clinical Trail Registration: http://www.chictr.org.cn/index.aspx, identifier ChiCTR 1800017875.

Importance: This is the first comprehensive comparison between ab interno approach and ab externo approach for microcatheter‐assisted trabeculotomy in primary congenital glaucoma with clear cornea.Background: Ab externo microcatheter‐assisted trabeculotomy is considered to be the gold standard for primary congenital glaucoma. The novel ab interno approach would intuitively be similar to ab externo approach for microcatheter‐assisted trabeculotomy in eyes with clear cornea; however, there is no evidence yet.Design: Non‐contemporary comparison of retrospective case series.Participants: Fifty‐eight and fifty‐seven consecutive primary congenital glaucoma eyes with clear corneas underwent ab interno and ab externo microcatheter‐assisted trabeculotomy, respectively.Methods: Retrospective comparison of clinical outcomes.Main Outcome Measures: Surgical success was defined as a postoperative intraocular pressure of ≤21 mmHg with at least a 30% reduction from preoperative IOP with or without the use of anti‐glaucoma medication (qualified and complete success, respectively).Results: The qualified success rate (87.9% vs 82.2%, P = .40) and complete success rates (81.0% vs 73.3%, P = .32) were comparable between groups. Complications were minimal and comparable. Less than 360° trabeculotomy (P = .009) and a higher number of previous surgeries (P = .03) were both associated with worse surgical outcomes.Conclusions and Relevance: Ab interno approach provide good and comparable outcomes as ab externo approach for microcatheter‐assisted trabeculotomy in primary congenital glaucoma eyes with clear cornea. It should be considered as the initial choice in these patients with the benefit of sparing conjunctiva. Successful trabeculotomy with either technique during the first attempt is critical to overall success and underscores the need for proficiency before independent practice.

Background: Autosomal recessive bestrophinopathy (ARB) is caused by homozygous or compound heterozygous mutations in the BEST1 gene and always accompanied with refractory angle-closure glaucoma (ACG). The exact mechanism for the pan-ocular abnormalities in ARB is still unknown and the management of ACG in these cases is challenging. Case presentation: A 26-year-old patient with a novel autosomal–recessively inherited c.1 A > G variant in BEST1 diagnosed as ARB and ACG, presented as widespread vitelliform deposits in the posterior pole, retinoschisis in the macula, vitreoretinal interface abnormalities, shallow anterior chamber depth (ACD) and angle closure with uncontrolled intraocular pressure (IOP). Combined phacoemulsification, intraocular lens implantation and goniosynechialysis did not improve any clinical presentation. However, low dose transscleral cyclophotocoagulation with subsequent vitreous liquefaction effectively lowered IOP, deepened ACD, and rehabilitated retinoschisis in both eyes. Conclusions: This case implied vitreous condition may play a pathogenic role in formation of retinoschisis and ACG in the patients with ARB. Treatments that induce vitreous liquefaction could be used to treat young ACG patients with ARB or other kinds of ACG to avoid vision-threatening post-operative complications.

Background To explore the outcomes and mechanisms of intraocular pressure (IOP) control using low-dose transscleral cyclophotocoagulation (LDTSCP) followed by phacoemulsification in patients with prolonged acute primary angle closure (APAC). Methods Patients with prolonged APAC refractory to all other treatment modalities were prospectively recruited, and underwent LDTSCP (10 shots, 2 s duration, 120° treatment with the energy starting at 1500 mW and titrated to the level with audible burst but not exceeding 2000 mW) and anterior chamber paracentesis 1 week prior to phacoemulsification with intraocular lens implantation and viscogoniosynechiolysis. Postoperative IOP, vision, anatomic changes on anterior segment optical coherence tomography and complications were recorded. Results Twenty eyes with prolonged APAC were recruited. Median follow-up was 12 months (range 9–18), at which point the vision in all eyes had improved and IOP was ≤17 mm Hg on no antiglaucoma medications. Following LDTSCP at postoperative day (POD) 1, IOP decreased in all eyes to a median 15 mm Hg (range: 6–28 mm Hg). Post-LDTSCP supraciliary effusion (SCE) occurred in 90% of eyes on POD1 or POD7 and ciliary body defect (CBD) was detected in 30% of eyes and resolved in all cases by postoperative month 1. Lower post-LDTSCP IOP was associated with more number of bursts (r=−0.558, p=0.011) and higher grade of SCE (r=−0.877, p<0.001), but not with total energy (p=0.240). Eyes with CBD (p=0.018) and a higher number of bursts (r=0.657, p=0.002) had higher grade SCE. Conclusions LDTSCP-induced SCE may explain the post-LDTSCP IOP reduction seen in eyes with prolonged APAC. LDTSCP instead of traditional more extensive treatment, was sufficient to provide a relatively safe and effective bridge therapy prior to phacoemulsification. Trial registration number Chinese Clinical Trials Registry (ChiCTR1900023567).

To retrospectively report a case of Weill-Marchesani syndrome 4 (WMS4) with compound heterozygous variants of ADAMTS17 gene. The patient was a 7-year-old boy with progressively worsening eyesight and intermittent elevated intraocular pressure (IOP) for two years. His IOPs were temporarily controlled using anti-glaucoma drugs. At presentation he had a shallow anterior chamber, lens subluxation, spherophakia and extensive synechial angle closure with high myopia in both eyes. Ultrasound biomicroscopy (UBM) identified thickened zonule fibers and anteriorly rotated, flat and slender ciliary processes, both of which worsened and were accompanied by obvious iris bombe after miosis. Gene testing showed compound heterozygosity of a maternal submicroscopic deletion on chromosome 15q26.3 (0.774 Mb) affecting the sequences of ADAMTS17, LYSMD4 and CERS3 as well as a paternal nonsense variant (c.1051_1053delAAGinsTAA, P.K351X) in the ADAMTS17 gene in the proband. The diagnosis of WMS4 was confirmed by genetic testing. Phacoemulsification (Phaco), intraocular lens (IOL) implantation, and irido-zonulo-hyaloid-vitrectomy (IZHV) combined with Ahmed Glaucoma Valve (AGV) implantation as a staged or one-stage surgery effectively lowered IOP, deepened ACD, improved visual acuity, and resolved the configuration of the ciliary processes in both eyes. Recessive ADAMTS17 variants are associated with WMS4. We report here compound heterozygous variants in ADAMTS17 causing WMS4, and anatomically highlighted the possible pathophysiology for its clinical phenotype. A modified surgical approach with Phaco, IOL implantation, and IZHV combined with AGV implantation could be used to treat these complicated cases.

Purpose: To explore stable and sensitive indicators for clinical diagnosis of acute angle closure (AAC) secondary to lens subluxation (LS) through quantitative analysis of CASIA 2 imaging. Design: A prospective cross-sectional study. Setting: Clinical practice. Participants: 23 patients with unilateral acute angle closure secondary to lens subluxation and 23 cataract patients without lens subluxation were recruited. Lens subluxation was confirmed by ultrasound biomicroscope diagnosis. The contralateral eyes without LS served as fellow control group. The cataract eyes without LS were enrolled in blank control group. Intervention: Participants underwent ophthalmologic examinations including slit-lamp biomicroscope, best corrected visual acuity, intraocular pressure, central corneal thickness measurement, axial length, gonioscopy, ultrasound biomicroscope and 360-degree anterior chamber and crystalline lens scan protocols of CASIA 2 system. Main outcome measures: Automated circumferential anterior segment and lens morphological parameters under anterior segment optical coherence tomography were analyzed via three-dimensional analysis. Significant differences were found in the front and back radius of the lens, the front and back radius of steep curvature of the lens, lens thickness, lens decentration, lens diameter, iris-trabecular contact (ITC) index, ITC area, anterior chamber depth (ACD), lens vault (LV), and iris volume between LS and controls. Among these parameters, LV, the anterior radius of steep curvature of the lens and ACD demonstrated the highest prediction power (AUC = 0.87, 0.89, and 0.86, respectively). The prediction power of tilt/axis was much higher in the Gaussian Naive Bayes model (AUCs = 0.90) than in the logistic model (AUCs = 0.74). Combination of LV_mean, LV_std, tilt and tilt axis in Gaussian Naive Bayes model presented as most stable and excellent diagnostic markers for AAC secondary to LS (AUCs = 0.98). The combination of markers including lens tilt and lens vault in the mathematic model facilitate clinical work as it not only provides novel diagnostic indications and possible prompt treatment for AAC secondary to lens subluxations, but also enhances our understanding of the pathogenic role of zonulopathy in angle closure glaucoma.