PurposeTo report the first simultaneous onset of bilateral acute depigmentation of the iris (BADI) in two siblings.ObservationsTwo sisters presented with bilateral ocular pain, redness and light sensitivity. Examination revealed bilateral circulating pigment in the anterior chamber with pigment dusting on backs of the corneas, patchy iris depigmentation and heavy pigment deposition in the angle. Both patients had recently suffered from upper respiratory tract infections. Bilateral visual acuities were preserved and no transillumination defects were observed. The patients were diagnosed with BADI. Both cases were successfully controlled with topical corticosteroids and anti-glaucoma drops as well as topical glanciclovir gel.Conclusions and ImportanceTo date, there had been no published reports of BADI in the Middle East and Africa. This is the first observation of this entity in these regions. Moreover it is the first occurrence of BADI in two immediate siblings simultaneously. We also report the rare asymmetrical presentation with BADI in one of our patients. These observations point to the possibility of genetic factors underlying BADI as well as an infectious cause behind the etiology.

The possible involvement of subtypes of dopamine (DA) receptors in pecking induced by apomorphine (APO) in chicks was studied. D-1/D-2 agonist APO dose-dependently induced pecking in chicks. The APO response was decreased in animals pretreated with either the D-2 receptor antagonist sulpiride or the D-1 receptor antagonist SCH 23390. The inhibitory effects of both antagonists were also dose dependent. The pecking induced by APO was completely inhibited in animals pretreated with a combination of SCH 23390 and sulpiride and was potentiated with reserpine. Single administration of D-2 agonist quinpirole or D-1 agonist SKF 38393 did not induced pecking, although quinpirole, but not SKF 38393 caused considerable response in reserpine or reserpine + alpha-methyl-p-tyrosine (AMPT)-treated animals. When quinpirole was administered with SKF 38393, a slight pecking response was shown. This was also potentiated in reserpine or reserpine + AMPT-treated chicks. The results may indicate that both D-1 and D-2 DA receptors are involved in pecking induced by APO, and reserpine treatment caused the sensitization of the D-2 receptors for the induction of pecking in chicks.

Introduction:The aim of this paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry for paediatric and adult patients with non-infectious uveitis (NIU). Methods: This is a physician-driven, population-and electronic-based registry implemented for both retrospective and prospective collection of real-world demographics, clinical, laboratory, instrumental and socioeconomic data of patients with uveitis and other non-infectious inflammatory ocular diseases recruited through the AIDA Network. Data recruitment, based on Luca Cantarini and Claudia Fabiani contributed equally to this manuscript.

Purpose To assess the safety and efficacy of combining nasal goniotomy with temporal trabeculotomy in the management of primary congenital glaucoma. Design Case series Methods Fifteen eyes of eleven children (3–12 months old at presentation) were enrolled in this study after the establishment of PCG diagnosis based on the criteria placed by the World Glaucoma Association. Combined nasal goniotomy and temporal trabeculotomy were done on each eye in an attempt to perform almost 360 degrees circumferential angle surgery without disturbing the superior 180 degrees of conjunctiva to preserve it for future filtering glaucoma surgeries. Results By first month, average IOP was 10.5 ± 4.3 mmHg with a 65.3% reduction from average pre-operative IOP. Almost the same percentage of reduction was maintained at 3rd,6th and 12th months postoperative visits with average IOP of 11.9 ± 4.65, 11.8 ± 2.77 and 13 ± 2.82 mmHg (60.7%, 61.1% and 57.2% reduction from pre-operative average). According to success rates, complete success has been achieved in all eyes but one (93.3%), with minor complications in 4 out of 15 eyes (26.6%) that did not affect IOP outcome. Nine out of fifteen eyes completed 18 months post-operative follow-up visits with a successfully, maintained target average IOP of 13.3 ± 3.0 mmHg (57.2% reduction). All of the mean IOP readings during post-operative follow-up period were significantly lower when compared to pre-operative IOP ( p < 0.0001). Only one eye had persistently elevated IOP of 26 mmHg at 1st and 3rd months post-operatively that necessitated a subsequent subscleral trabeculectomy which succeeded in controlling the pressure bringing it down to 15 mmHg on topical medications. Conclusion Nasal goniotomy – temporal trabeculotomy (NGTT) is the combination of two well established surgeries that exploits the advantages of circumferential angle surgery while sparing the superior conjunctiva completely for future surgeries if needed. This new procedure was safe and effective in lowering IOP by an average of 60% from pre-operative IOP with a sustained effect till 18 months post-operatively. We believe that this surgery might be added to the armentarium of the surgical management of infantile glaucomas with other circumferential angle surgeries.

Introduction: This article points out the design, methods, development and deployment of the international registry promoted by the AutoInflammatory Disease Alliance (AIDA) Network with the aim to define and assess paediatric and adult patients with immunemediated scleritis. Methods: This registry collects both retrospective and prospective real-world data from patients with non-infectious scleritis through the Research Electronic Data Capture (REDCap) tool and aims to promote knowledge and reallife evidence from patients enrolled worldwide; the registry also allows the collection of Luca Cantarini and Claudia Fabiani contributed equally.

Waardenburg syndrome (WS) is a genetic disorder resulting in anomalies of derivatives of neural crest cells during development. Patients tend to have variable degrees of pigmentary defects affecting skin, hair, and irides in addition to hearing loss and possible systemic neurological associations. Elevation of the intraocular pressure has been reported in several adult patients with WS. We report the first case of WS to be associated with juvenile open-angle glaucoma in a 20-year-old Egyptian man thus expanding the spectrum of the types of glaucoma that can coexist with the syndrome.

Described as early as Hippocrates in his “Third Book of Endemic Diseases,” Behçet's Disease (BD), also known as “The Silk Road Disease” following its initial demographics, consists of a triad of recurrent oro-genital ulcers and associated uveitis. Current demographics and rising percentages of patients seen far beyond the Silk Road in Ocular Inflammatory Disease and Uveitis Clinics list BD uveitis as one of the frontliners of non-infectious autoinflammatory eye diseases. Clinical features of BD and juvenile-onset BD are detailed alongside various approaches in classification and suggested algorithms for diagnosis that are outlined in this review. With the ongoing Human Microbiome Project and studies such as the MAMBA study, the role of the human microbiome in BD is highlighted in the pathophysiology of BD to include the current research and literature perspective. Furthermore, with the advancement of recent diagnostic and investigative techniques, especially in the field of Optical Coherence Tomography (OCT), disease-related characteristics are updated to encompass SD, EDI and OCT-angiography characteristics of BD. Having entered the era of biologic therapy, the role of various specific cytokine-blocking biologic drugs, such as TNF-α inhibitors (e.g., adalimumab, infliximab), interferon α-2a inhibitors, IL-6 and IL-1 inhibitors are presented and contrasted alongside the conventional immunosuppressant drugs and the classic old gold standard: corticosteroids (systemic or local). Finally, with the ongoing SARS-CoV-2 pandemic, it was not possible to conclude the review without reviewing the latest evidence-based literature reporting BD morbidity in this era, the observed pattern and treatment recommendations as well as those related to reported post-vaccine complications and emergence of BD.

To evaluate the retinal vasculature in psoriasis patients and detect if metabolic syndrome is an additional risk factor. This cross-sectional analytic study was carried out on 80 eyes of 80 subjects; 28 eyes with psoriasis only (PS group), 12 eyes with additional metabolic syndrome to psoriasis (PMS group) and 40 eyes healthy controls (HS). The retinal capillary plexuses were evaluated by OCTA. The disease activity was evaluated by the Psoriasis Area and Severity Index (PASI) score and extent. The superficial capillary plexus (SCP) vascular density was significantly lower in PS group than HS while in PMS it was significantly lower only in whole image and superior and temporal perifoveal areas (p-value = 0.020, 0.030, 0.001 respectively). The changes correlated with the disease duration. The vascular density of the deep capillary plexus (DCP) was significantly lower in both PS and PMS groups (p-value < 0.001). Psoriatic patients are at a higher risk of developing retinal vascular complications even without evident clinical ocular disease. It was noted that the presence of metabolic syndrome contributes as an additional risk factor in possible visual loss secondary to ischemic changes that are likely to start in the DCP and progress to involve all levels.