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Importance: Pediatric blepharokeratoconjunctivitis (PBKC) is a chronic, sight-threatening inflammatory ocular surface disease. Due to the lack of unified terminology and diagnostic criteria, nonspecific symptoms and signs, and the challenge of differentiation from similar ocular surface disorders, PBKC may be frequently unrecognized or diagnosed late., Objective: To establish a consensus on the nomenclature, definition, and diagnostic criteria of PBKC., Design, Setting, and Participants: This quality improvement study used expert panel and agreement applying the non-RAND modified Delphi method and open discussions to identify unified nomenclature, definition, and definitive diagnostic criteria for PBKC. The study was conducted between September 1, 2021, and August 14, 2022. Consensus activities were carried out through electronic surveys via email and online virtual meetings., Results: Of 16 expert international panelists (pediatric ophthalmologists or cornea and external diseases specialists) chosen by specific inclusion criteria, including their contribution to scientific leadership and research in PBKC, 14 (87.5%) participated in the consensus. The name proposed was "pediatric blepharokeratoconjunctivitis," and the agreed-on definition was "Pediatric blepharokeratoconjunctivitis is a frequently underdiagnosed, sight-threatening, chronic, and recurrent inflammatory eyelid margin disease associated with ocular surface involvement affecting children and adolescents. Its clinical spectrum includes chronic blepharitis, meibomitis, conjunctivitis, and corneal involvement ranging from superficial punctate keratitis to corneal infiltrates with vascularization and scarring." The diagnostic criteria included 1 or more suggestive symptoms accompanied by clinical signs from 3 anatomical regions: the eyelid margin, conjunctiva, and cornea. For PBKC suspect, the same criteria were included except for corneal involvement., Conclusions and Relevance: The agreements on the name, definition, and proposed diagnostic criteria of PBKC may help ophthalmologists avoid diagnostic confusion and recognize the disease early to establish adequate therapy and avoid sight-threatening complications. The diagnostic criteria rely on published evidence, analysis of simulated clinical cases, and the expert panel's clinical experience, requiring further validation with real patient data analysis.

Purpose: To explore the tolerability of automated conjunctival hyperemia quantification in children with blepharokeratoconjunctivitis (BKC) and its agreement with clinical activity grading and to explore the Children's Health Utility 9D (CHU9D) as a measure of health-related quality of life in children with BKC., Methods: We enrolled 63 children, 31 with BKC and 32 without ocular surface inflammation, with a median [interquartile range (IQR)] age of 10.6 (7.2-13.9) years for BKC and 11.4 (9.5-13.8) years for healthy volunteers. Two masked observers graded the ocular surface images. The children indicated discomfort during imaging on a 5-point Likert scale. Using nonparametric tests, we explored the interobserver agreement and the agreement of automated redness index (RI) measurements of limbal and bulbar conjunctival hyperemia with clinician assessment. The children also completed the 9-item CHU9D., Results: The children tolerated imaging well: median (IQR) Likert value of 0 ("comfortable") (0-0) in healthy volunteers and 1 ("a little bit uncomfortable") (0-2) in mild/moderate BKC. In children with BKC, the median (IQR) bulbar RI was 1.3 (0.8-1.6) and the median limbal RI was 0.7 (0.3-1.1). In healthy volunteers, the median bulbar RI was 0.8 (0.55-1.1; P = 0.162) and the median limbal RI was 0.3 (0.2-0.4; P = 0.02). The agreement between RI and clinical grading was high. There was no significant difference between the mean CHU9D utility score between the 2 groups [0.89 (SD 0.08) vs. 0.92 (SD 0.07); P = 0.15]., Conclusions: Automated conjunctival hyperemia quantification is feasible in children with ocular surface inflammation and may prove useful for long-term monitoring and as an objective outcome measure in clinical trials.

Purpose: To evaluate non-contact infrared meibography and anterior segment optical coherence tomography (AS-OCT) to detect meibomian gland (MG) and corneal changes in children with blepharokeratoconjunctivitis (BKC)., Methods: We acquired infrared meibography images of upper and lower lids and AS-OCT corneal scans. One masked observer graded meiboscore, full/partial MG dropout, and measured total corneal volume and differential corneal volume per quadrant and central corneal thickness (CCT)., Results: We enrolled 63 children, 31 with BKC and 32 without ocular surface inflammation; median (interquartile range) age BKC 10.6 (7.2-13.9) years, healthy volunteers (HV) 11.4 (9.5-13.8) years. Likert scale scores for meibography and OCT indicate no to low discomfort. Meiboscores for upper and lower lids as well as the total meiboscore were significantly higher in children with BKC than in HV. Subscores for full and partial MG dropout were also significantly higher in children with BKC than in healthy volunteers. There was no statistically significant difference between upper and lower lid for meiboscore nor full/partial MG dropout scores. The corneal volume in the superior quadrant was significantly higher in children with BKC than in HV, whereas the corneal volume in the nasal and inferior quadrants was significantly lower., Conclusions: Non-contact imaging technologies objectively demonstrate damage to meibomian glands and changes in corneal volume secondary to BKC. The tests are well tolerated by children with mild/moderate ocular surface inflammation and can detect changes without the requirement for routine eversion of the upper lid. These parameters may be useful both for clinical follow-up and clinical trials., (© 2019 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To describe a pragmatic approach to the medical management of blepharokeratoconjunctivitis in children, based on published evidence and clinical experience., Methods: The authors used the Delphi consensus method to explore the preferred management patterns of four senior clinicians at one institution to reach agreement on indications and dosage schedules for commonly used treatments. Four iterations were created, with electronic questionnaires distributed via an online survey platform. Initial questions were based on recent systematic reviews and clinical experience. After each round, a facilitator summarized the responses and fed these back to the expert participants, together with an invitation to complete the next round of questions., Results: Typical and specific eyelid, corneal, and conjunctival disease features influenced management decisions, and treatments were targeted toward specific findings in these tissues rather than to overall disease severity. Active keratitis was considered the main indication for high potency steroids, systemic antibiotics, and possibly systemic immunomodulators. Other indications for systemic antibiotics were chronic active blepharitis and recurrent troublesome chalazia. Oral antibiotics were used for their anti-inflammatory and antimicrobial properties. There was little agreement on the role of dietary modification, topical lubricants, and preference for oral or topical antibiotics., Conclusions: Detailed clinical assessment of eyelids and ocular surface allows targeted treatment. Research is needed to clarify disease mechanisms and to optimize treatment strategies. [J Pediatr Ophthalmol Strabismus. 2017;54(3):156-162.]., (Copyright 2017, SLACK Incorporated.)

Background: Blepharokeratoconjunctivitis (BKC) is a type of inflammation of the surface of the eye and eyelids that involves changes of the eyelids, dysfunction of the meibomian glands, and inflammation of the conjunctiva and cornea. Chronic inflammation of the cornea can lead to scarring, vascularisation and opacity. BKC in children can cause significant symptoms including irritation, watering, photophobia and loss of vision from corneal opacity, refractive error or amblyopia.Treatment of BKC is directed towards modification of meibomian gland disease and the bacterial flora of lid margin and conjunctiva, and control of ocular surface inflammation. Although both topical and systemic treatments are used to treat people with BKC, this Cochrane review focuses on topical treatments., Objectives: To assess and compare data on the efficacy and safety of topical treatments (including antibiotics, steroids, immunosuppressants and lubricants), alone or in combination, for BKC in children from birth to 16 years., Search Methods: We searched CENTRAL (which contains the Cochrane Eyes and Vision Trials Register) (2016, Issue 6), Ovid MEDLINE, Ovid MEDLINE In-Process and Other Non-Indexed Citations, Ovid MEDLINE Daily, Ovid OLDMEDLINE ( January 1946 to 11 July 2016), Embase (January 1980 to 11 July 2016), the ISRCTN registry (www.isrctn.com/editAdvancedSearch), ClinicalTrials.gov (www.clinicaltrials.gov) and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en). We did not use any date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 11 July 2016. We searched the reference lists of identified reports and the Science Citation Index to identify any additional reports of studies that met the inclusion criteria., Selection Criteria: We searched for randomised controlled trials that involved topical treatments in children up to 16 years of age with a clinical diagnosis of BKC. We planned to include studies that evaluated a single topical medication versus placebo, a combination of treatments versus placebo, and those that compared two or multiple active treatments. We planned to include studies in which participants received additional treatments, such as oral antibiotics, oral anti-inflammatories, warm lid compresses and lid margin cleaning., Data Collection and Analysis: Two review authors independently screened the results of the literature search (titles and abstracts) to identify studies that met the inclusion criteria of the review and applied standards as expected for Cochrane reviews. We graded the certainty of the evidence using GRADE., Main Results: We included one study from the USA that met the inclusion criteria. In the study, 137 children aged zero to six years old with blepharoconjunctivitis were randomised to treatment in one of four trial arms (loteprednol etabonate/tobramycin combination, loteprednol etabonate alone, tobramycin alone or placebo) for 15 days, with assessments on days 1, 3, 7 and 15. We judged the study to be at high risk of attrition bias and bias due to selective outcome reporting. The study did not report the number of children with improvement in symptoms nor with total or partial success as measured by changes in clinical symptoms.All children showed a reduction in blepharoconjunctivitis grade score, but there was no evidence of important differences between groups. Visual acuity was not fully reported but the authors stated that there was no change in visual acuity in any of the treatment groups. The study reported ocular and non ocular adverse events but was underpowered to detect differences between the groups. Ocular adverse events were as follows: loteprednol/tobramycin 1/34 (eye pain); loteprednol 4/35 (eye pain, conjunctivitis, eye discharge, eye inflammation); tobramycin 0/34; placebo (vehicle) 0/34. The evidence was limited for all these outcomes and we judged it to be very low certainty.There was no information on clinical signs (aside from grade score), disease progression or quality of life., Authors' Conclusions: There is no high-quality evidence of the safety and efficacy of topical treatments for BKC, which resulted in uncertainty about the indications and effectiveness of topical treatment. Clinical trials are required to test efficacy and safety of current and any future treatments. Outcome measures need to be developed which can capture both objective clinical and patient-reported aspects of the condition and treatments.

Introduction: Childhood blepharokeratoconjunctivitis is a common lid margin inflammation with secondary ocular surface disease. Its etiology is unclear and there are no randomized controlled trials to support the superiority of any treatment option. Areas covered: We searched the following databases; Cochrane Central Register of Controlled Trials, Ovid MEDLINE and affiliated Ovid databases, EMBASE, the ISRCTN registry, Clinical- Trials.gov and the World Health Organization International Clinical Trials Registry Platform. Due to the paucity of pediatric data we also considered information from articles focused on adults. Expert commentary: Treatment is based on the assumption that the mechanisms of BKC and rosacea keratitis are the same: meibomian gland dysfunction, bacterial colonisation of the lid margin, delayed type hypersensitivity, Demodex folliculorum, genetic predisposition and Toll-like receptors inducing release of pro-inflammatory cytokines. Generally accepted grading scales are needed. Randomized clinical trials are needed to evaluate treatment options. The effects of antibiotics, immunomodulators, osmoprotectants and essential fatty acids need further investigation. [ABSTRACT FROM PUBLISHER]