Definition and Diagnostic Criteria for Pediatric Blepharokeratoconjunctivitis

Author:

Morales-Mancillas Nallely R.1,Velazquez-Valenzuela Fabiola2,Kinoshita Shigeru3,Suzuki Tomo45,Dahlmann-Noor Annegret H.67,Dart John K. G.8,Hingorani Melanie78,Ali Asim9,Fung Simon10,Akova Yonca A.11,Doan Serge12,Gupta Noopur13,Hammersmith Kristin M.14,Tan Donald T. H.151617,Paez-Garza J. Homar1,Rodriguez-Garcia Alejandro2

Affiliation:

1. Tecnologico de Monterrey, School of Medicine and Health Sciences, Institute of Ophthalmology and Visual Sciences, Pediatric and Strabismus Service, Monterrey, Mexico

2. Tecnologico de Monterrey, School of Medicine and Health Sciences, Institute of Ophthalmology and Visual Sciences, Cornea, External Disease and Ocular Immunology Service, Monterrey, Mexico

3. Department of Frontier Medical Science and Technology for Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan

4. Department of Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan

5. Department of Ophthalmology, Kyoto City Hospital Organization, Kyoto, Japan

6. National Institute of Health Research’s Biomedical Research Centre at Moorfields Eye Hospital and the UCL Institute of Ophthalmology, London, United Kingdom

7. Children’s Service, Moorfields Eye Hospital National Health Service Foundation Trust, London, United Kingdom

8. Corneal Service, Moorfields Eye Hospital National Health Service Foundation Trust, London, United Kingdom

9. Department of Ophthalmology & Vision Sciences, Hospital for Sick Children, Toronto, Ontario, Canada

10. Department of Ophthalmology, University of California, Los Angeles

11. Department of Ophthalmology, Bayındır Hospital, Ankara, Turkey

12. Department of Ophthalmology, Fondation Ophtalmolologique A. de Rothschild, Paris, France

13. Cornea, Cataract & Refractive Surgery Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

14. Cornea Service, Wills Eye Hospital, Jefferson Health, Philadelphia, Pennsylvania

15. Eye & Cornea Surgeons, Eye & Retina Surgeons, Camden Medical and Mount Elizabeth Novena Specialist Centre, Singapore

16. Singapore Eye Research Institute, Singapore National Eye Centre, Singapore

17. Department of Ophthalmology and Visual Science, Duke-NUS Graduate Medical School, Singapore

Abstract

ImportancePediatric 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.ObjectiveTo establish a consensus on the nomenclature, definition, and diagnostic criteria of PBKC.Design, Setting, and ParticipantsThis 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.ResultsOf 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 RelevanceThe 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.

Publisher

American Medical Association (AMA)

Subject

Ophthalmology

Reference75 articles.

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