Definition and Diagnostic Criteria for Pediatric Blepharokeratoconjunctivitis

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.

Figure 1.. Diagnostic Criteria for Definitive Pediatric Blepharokeratoconjunctivitis (PBKC) and PBKC Suspect

The criteria for confirming a diagnosis of PBKC require 1 or more suggestive symptoms (or history) plus 1 or more clinical signs from each of 3 anatomical regions: the eyelid margin, the conjunctiva, and the cornea. PBKC suspect comprises the same criteria for definitive diagnosis except for corneal involvement. Both categories may or may not have skin involvement, identified by adding an -Sk suffix (PBKC-Sk or SPBKC-Sk). ^aSubepithelial infiltrates are primarily located inferiorly or in the interpalpebral zone of the limbus and peripheral cornea. They may present as multiple nummular stromal lesions or as large, dense, and highly vascularized paracentral or central stromal opacities with or without an epithelial defect. ^bSuperficial corneal vascularization may arise in the inferior and peripheral cornea, frequently following a pathway toward the infiltrates. It can be localized or may cover extensive areas.

Figure 2.. Spectrum of the Eyelid Margin Manifestations of Pediatric Blepharokeratoconjunctivitis (PBKC)

A, Meibomitis, meibomian gland dysfunction, and telangiectasia in the upper eyelid. B, Chalazia and meibomitis in the upper eyelid. C, Active external chalazion with meibomitis and collarette secretions in the anterior upper eyelid margin.

Figure 3.. Spectrum of the Conjunctival Manifestations of Pediatric Blepharokeratoconjunctivitis (PBKC)

A, Upper eyelid external chalazion and marked bulbar conjunctival hyperemia. B, Follicular and faint papillary conjunctival reaction in recurrent PBKC. C, Marked conjunctival hyperemia and multiple inferior limbal rounded and elevated corneal infiltrates with micropannus.

Figure 4.. Characteristic Pediatric Blepharokeratoconjunctivitis (PBKC) Acute Corneal Findings

A, Multiple perilimbal early active stromal infiltrates. B, Extensive peripheral stromal infiltration and paracentral phlyctenules with considerable superficial vascularization invading the temporal cornea. C, Scattered corneal infiltrates associated with upper corneal vascularization.

Figure 5.. Characteristic Pediatric Blepharokeratoconjunctivitis (PBKC) Chronic Corneal Findings

A, Late central and paracentral corneal infiltrates and leukoma formation. B, Inferior corneal vessel ingrowth and meibomitis in the central upper eyelid margin. C, Subepithelial stromal cellular infiltration with vascularization (phlycten) associated with meibomitis on the upper eyelid margin, which are extension of the phlycten.