Morales-Mancillas, Nallely R; Velazquez-Valenzuela, Fabiola; Kinoshita, Shigeru; Suzuki, Tomo; Dahlmann-Noor, Annegret H; Dart, John KG; Hingorani, Melanie; ... Rodriguez-Garcia, Alejandro; + view all Morales-Mancillas, Nallely R; Velazquez-Valenzuela, Fabiola; Kinoshita, Shigeru; Suzuki, Tomo; Dahlmann-Noor, Annegret H; Dart, John KG; Hingorani, Melanie; Ali, Asim; Fung, Simon; Akova, Yonca A; Doan, Serge; Gupta, Noopur; Hammersmith, Kristin M; Tan, Donald TH; Paez-Garza, J Homar; Rodriguez-Garcia, Alejandro; - view fewer (2023) Definition and Diagnostic Criteria for Pediatric Blepharokeratoconjunctivitis. JAMA Ophthalmology , 142 (1) pp. 39-47. 10.1001/jamaophthalmol.2023.5750.

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Abstract

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.

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