Abstract: Our increase in knowledge of the pathophysiology of non-infectious uveitis (NIU) and other immune-mediated diseases has been mirrored over the last two decades by the expansion of therapeutic options in the realm of immunosuppressive medications. Principal among these advances is the emergence of biologics, which offer the promise of targeted therapy and the hope of reduced toxicity when compared to corticosteroids and “standard” immunosuppression. Among the biologics, monoclonal antibodies blocking tumor necrosis factor alpha (TNF-α) have been shown to be a very effective therapeutic target for uveitis and many associated systemic inflammatory diseases. Multiple TNF blockers have shown benefit for uveitis, and in 2016, adalimumab became the first biologic and non-corticosteroid immunosuppressive to obtain Food and Drug Administration (FDA) approval in the treatment of NIU. Although effective, TNF blockers are not universally so, and safety concerns such as infection and demyelinating disease must be carefully considered and ruled out prior to their use, especially in patients with intermediate uveitis with which multiple sclerosis is a known association. Ongoing study has identified novel targets for regulation in the treatment of immune-mediated and inflammatory diseases. Interferons, interleukin and Janus kinase inhibitors in addition to antibodies targeting T cell and B cell activation highlight the expanding field of treatment modalities in NIU. Ongoing study will be required to better determine the safety and efficacy of biologics in the armamentarium of immunosuppressive treatments for NIU.

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.

Abstract: Bioengineered materials are used as a substitute in many fields of medicine, especially in plastic surgery and in burns. In ophthalmic plastic surgery they can be used for covering large tissue defects or as a tarsal plate substitute, in cases when it is not possible to use conventional surgical techniques. We have searched PubMed and Web of Science scientific databases. We can generally categorize skin substitutes by the type of tissue used—we distinguish autografts, allografts, and xenografts. There are also completely synthetic substitutes. The aim of our article was to summarize the current state of knowledge and to sum up all the clinical applications of bioengineered materials in the periocular region. There are only a few scientific articles about this topic and lack of prospective randomized studies aimed on use of bioengineered materials in periocular region. Nevertheless, there are many articles describing successful case reports or case reports series. According to literature, bioengineered materials are the most commonly used in big traumas or large surgical defects, especially in oculoplastic tumour surgery. Bioengineered dermal substitutes are not frequently used in the periocular region. Dermal substitutes are useful, when it is not possible to close the defect with any other conventional surgical technique.

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.