Abstract: Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, and juvenile idiopathic associated uveitis (JIA-U) is the most frequently noted extra-articular manifestation. JIA-U can present asymptomatically and lead to ocular complications, so regular screening and monitoring are needed to prevent potentially sight-threatening sequelae. Topical glucocorticoids such as prednisolone acetate are usually the first line of treatment for anterior uveitis associated with JIA-U, but long-term use may be associated with cataract, ocular hypertension and glaucoma. Disease modifying anti-rheumatic drugs (DMARDs) such as methotrexate allow tapering of the corticosteroids to prevent long-term complications. Biologic therapies have been increasingly used as targeted therapies for JIA-U, particularly monoclonal antibodies targeting the proinflammatory cytokine TNF-α such as adalimumab and infliximab. One recent, multicenter, prospective, randomized clinical trial provided evidence of the efficacy of adalimumab with methotrexate for JIA-U compared to methotrexate alone. Another clinical trial studying the interleukin-6 inhibitor tocilizumab for JIA-U showed promise in tapering topical corticosteroids. Additionally, JAK inhibitors are emerging biologic therapies for JIA-U in patients refractory to TNF-α inhibitors, with a clinical trial assessing the efficacy of baricitinib for JIA-U underway. While clinical trials on these novel biologics are limited, further investigation of these agents may provide additional therapeutic options for JIA-U.

Background: To evaluate a fully automated vascular density (VD), skeletal density (SD) and fractal dimension (FD) method for the longitudinal analysis of retinal vein occlusion (RVO) eyes using projection-resolved optical coherence tomography angiography (OCTA) images and to evaluate the association between these quantitative variables and the visual prognosis in RVO eyes.

Methods: Retrospective longitudinal observational case series. Patients presenting with RVO to Creteil University Eye Clinic between October 2014 and December 2018 and healthy controls were retrospectively evaluated. Group 1 consisted of central RVO (CRVO) eyes, group 2 consisted of eyes with branch RVO (BRVO) and group 3 of healthy control eyes. OCTA acquisitions (AngioVue RTVue XR Avanti, Optovue, Inc., Freemont, CA) were performed at baseline and last follow up visit. VD, SD, and FD analysis were computed on OCTA superficial and deep vascular complex (SVC, DVC) images at baseline and final follow up using an automated algorithm. Logistic regression was performed to find if and which variable (VD, SD, FD) was predictive for the visual outcome.

Results: Forty-one eyes, of which 21 consecutive eyes of 20 RVO patients (13 CRVO in group 1, 8 BRVO in group 2), and 20 eyes of 20 healthy controls were included. At the level of SVC, VD and FD were significantly lower in RVO eyes compared to controls (P<0.0001 and P=0.0008 respectively). Best-corrected visual acuity (BCVA) at last follow-up visit was associated with baseline VD (P=0.013), FD (P=0.016), and SD (P=0.01) at the level of the SVC, as well as with baseline FD at the DVC level (P=0.046).

Conclusions: Baseline VD, SD, and FD are associated with the visual outcome in RVO eyes. These parameters seem valuable biomarkers and may help improve the evaluation and management of RVO patients.

Background: Using a randomized controlled trial (RCT), to assess the efficacy of the folded technique of self-adherent wrap to eyes after orbital tumour extirpation and compare it with the classic technique.

Methods: A single-centre, prospective, randomized, controlled study was conducted among 128 patients who underwent orbital tumour extirpation in this study. The folded and classic techniques of applying self-adherent wraps were randomly allocated to patients (1:1). The primary endpoint was the interface pressure on the affected eye. Secondary efficacy endpoints were the interface pressure above and below the ear of the affected side, above the ear of the non-affected side, and discomfort scores. Postoperative complications were observed for 24 hours.

Results: The interface pressure with the folded technique on the affected eye was neither inferior nor superior to the classic technique (1.33±0.07 vs. 1.41±0.09 mmHg, P=0.480). Most importantly, the pressure at three other points outside of the affected eye, including above and below the ear of the affected side, and above the ear of the non-affected side, were significantly higher when using the classic technique than when using the folded technique (P=0.041, 0.019, and 0.047, respectively). Discomfort scores were higher in the classic technique group than in the folded technique group (2.93±0.30 vs.1.52±0.19, P≤0.001).

Conclusions: Findings demonstrated the advantages of using folded technique to apply self-adherent wrap for wounds after orbital tumour extirpation with lower interface pressure outside of the affected eye and patient discomfort scores, without influencing pressure on the affected eye comparing with the classic technique.

Abstract: The rare disease of chronic infantile neurological cutaneous and articular (CINCA) syndrome, is caused by the over-secretion of interleukin (IL)-1β due to a gain-of-function NLRP3 gene mutation in the autosomal chromosome which often involves in eyes. In this report, we studied a 9-year-old girl with CINCA. The eyes were also involved and presented bilateral papilledema. Genetic testing revealed that the symptoms were caused by a novel gene mutation site (c.913G>A, p. D305N) in conservative domain exon-3 of NLRP3 which is gain-function gene of CINCA. The patient had the characteristic facial features, frontal fossa and saddle nose, manifested the generalized urticaria-like skin rash at two weeks after birth, periodic fever 6 months after birth, sensorineural deafness at 7 years old, and bilateral papilledema, aseptic meningitis and knee arthropathy at 9 years old. White cell counts, C-reactive protein increased and intracranial pressure raised to 300 mmH2O. The meningeal thickening enhanced by gadolinium in magnetic resonance imaging (MRI). Based on clinical features and genetic test, the girl was diagnosed bilateral papilledema secondary to CINCA and administered prednisone and lowered intracranial pressure medicine to resolve symptoms. With 3-year follow-up, patient had no inflammatory flare-up with visual acuity improvement. The finding of novel genetic mutation site (p. D305N) in NLRP3 gene expanded genotype spectrum associated with CINCA. This case also expanded the cause spectrum of papilledema and it highlighted systemic disease history for patients with bilateral papilledema.

Abstract: Anthropometry can analyze the size, weight, and proportion of the human body objectively and quantitatively to supplement the visual assessment. Various non-invasive three-dimensional (3D) anthropometric techniques have been applied to assess soft tissues’ 3D morphology in the clinical practice. Among them, non-invasive stereophotogrammetry and laser scanning techniques are becoming increasingly popular in craniofacial surgery and plastic surgery. They have been applied for craniofacial growth estimation and morphometric investigation, genetic and acquired malformation diagnosis, as well as orthodontic or surgical treatment arrangement and outcome evaluation. However, few studies have been published for assessing the 3D morphology of soft tissues in the periorbital region. This paper reviews the studies involving the application and evaluation of the increasingly popular 3D photogrammetry in the periorbital region. These studies proposed detailed and standardized protocols for three-dimensionally assessing linear, curvilinear, angular, as well as volumetric measurements, and verified its high reliability in the periorbital region (even higher than caliper-derived direct measurements). In the future, reliable and accurate 3D imaging techniques, as well as standardized analyzing protocols, may find applications in following up morphological growth, preoperatively diagnosing and assessing patient periorbital conditions, planning surgical procedures, postoperatively evaluating treatment outcomes of a specific procedure, and comparing the differences in surgical results between various procedures, studies, as well as populations.

Abstract: Glaucoma is a group of eye diseases that seriously threaten human visual health. Increased intraocular pressure is the main clinical manifestation and diagnostic basis of glaucoma and is directly related to increased resistance to aqueous circulation channels. The trabecular meshwork (TM) is a multi-layer spongy tissue that filters aqueous humor. Its structure changes and the filtering capacity decreases, leading to an increase in intraocular pressure. Surgical methods for TM are constantly updated. Compared with traditional glaucoma surgical techniques, such as external trabeculectomy, the development of a new surgical technique—minimally invasive glaucoma surgery (MIGS)—enables the operation to reduce intraocular pressure efficiently while further reducing damage to the eye. MIGS achieves the purpose of surgery mainly by optimizing the TM outflow pathway, uveoscleral outflow pathway, and subconjunctival outflow pathway. A new surgical instrument, the Kahook Dual Blade, appears to optimize the TM outflow pathway in the surgical technique. The Kahook Dual Blade is a new type of angle incision instrument. Because of its unique double-edged design, in the process of goniotomy, it can effectively reduce the damage to the anterior chamber angle structure and accurately remove the appropriate amount of TM so that the aqueous humor can flow out smoothly. Kahook Dual Blade goniotomy has the advantages of avoiding complications and foreign body sensation caused by intraocular implants. The operation time is relatively short, the surgical technique is easy to master, and the TM resection scope can be determined based on the patient’s condition. It can be used to treat some clinically meaningful glaucoma. This article is organized as follows. We present the following article following the Narrative Review reporting checklist.

Abstract: In a comprehensive literature review, PubMed, Embasem and Web of Science were searched for studies examining targeted therapy of ocular malignant melanomas to present and discuss targeted therapy treatment options of ocular tumors, mainly conjunctival and uveal melanoma (UM). Conjunctival malignant melanomas showed similarities in clinical and genetic aspects with cutaneous melanomas. Many therapies with checkpoint inhibitors already established for cutaneous melanomas may be a treatment option for conjunctival malignant melanomas with shared traits. Existing targeted therapies are for example checkpoint inhibitors like pembrolizumab or nivolumab. As a corollary, due to marked differences in clinics and genetics between UMs and conjunctival melanomas (CMs) or cutaneous melanomas, it has remained elusive whether the available possibilities of molecular targeted therapy will be an option for the therapy of metastasizing UMs. Possible novel ways of treating UM are being explored. Fotemustine or the inoculation of dendritic cells with tumorous RNA or sunitinib in combination with cisplatin and or tamoxifen may be used in future to treat UM. While CM are treatable using targeted therapies, UM have not been researched enough to find working targeted therapy options. Further research has to be done in order to find acceptable treatment options.