Clinical Characteristics and Therapeutic Outcomes of Pediatric Blepharokeratoconjunctivitis

Abstract

Purpose:The purpose of this study was to determine the clinical characteristics, disease course, therapeutic outcomes, and prognostic factors for pediatric patients with blepharokeratoconjunctivitis (BKC).Methods:A retrospective medical chart review was performed for patients aged 15 years or younger who had been diagnosed with BKC between 2004 and 2020 at 2 tertiary hospitals in Korea. The following data were collected: demographics, medical history, ocular findings, geometric profiling of corneal lesion, medical management, and outcomes.Results:A total of 137 patients (90 female and 47 male) were included. The patients’ mean age was 8.3 ± 3.8 years at disease onset. Both eyes were involved in 57.7% of cases. The most common corneal lesion was corneal neovascularization (77.4%), followed by clinically visible corneal infiltration (51.8%) and stromal scarring (43.1%). Most of the corneal lesions involved a single quadrant, most commonly the inferior quadrant. After treatment, disease remission was achieved in 95% of patients, and visual acuities improved from 0.2 ± 0.3 logarithm of minimal angle of resolution at disease presentation to 0.1 ± 0.3 logarithm of minimal angle of resolution at final follow-up (P = 0.001). Recurrence occurred in 52.6% of patients. Cylinder power was significantly higher in patients with recurrence than in those without. The number of cases of recurrence was positively associated with final cylinder power.Conclusions:Although the treatment induced disease remission in 95% of children with BKC, recurrence occurred in 52.6% of those cases. Because recurrence resulted in significant astigmatism, careful observation for recurrence and prompt management are warranted for preservation of vision in pediatric patients with BKC.

Definition and Diagnostic Criteria for Pediatric Blepharokeratoconjunctivitis

“…Therefore, we agreed to name it pediatric blepharokeratoconjunctivitis, a term enclosing the age population and the ocular surface structures affected by the disease. Although wording prevalence was not a selection criterion, the first word (age population affected) is the most used in relevant literature reports describing this entity . The second one (ocular surface structures affected) is also mentioned in most relevant publications on the subject (eTable 1 in Supplement 1).…”

“…Such infiltrates can produce significant opacity and scarring and may or may not present with epithelial defects (Figure 5). 6,17,26 On the other hand, skin changes may occur in patients with PBKC, leading to the widespread use of the terminology child- 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.…”

“…Although wording prevalence was not a selection criterion, the first word (age population affected) is the most used in relevant literature reports describing this entity. [1][2][3][4]14,16,17,26,[67][68][69][70][71] The second one (ocular surface structures affected) is also mentioned in most relevant publications on the subject (eTable 1 in Supplement 1). [1][2][3][4]7,9,14,16,17,23,26,[55][56][57]60,[67][68][69][70][71][72] For the definition, we included the clinical course of the disease, its primary pathogenic manifestation, age group, potential visual consequences, and the affected ocular structures to delineate the most critical features that distinguish PBKC.…”

“…17 A recent study found corneal neovascularization (77.4%), stromal infiltrates (51.8%), and scarring (43.1%) as the most frequent corneal findings, reducing vision at presentation to 0.2 logMAR and producing amblyopia in 8.8% of patients. 26 Since its initial description, 27 PBKC has received many different names and definitions (eTable 1 in Supplement 1). Furthermore, because PBKC shows a broad clinical spectrum, diverse clinical course, and overlapping symptoms and signs with other ocular surface disorders, there is no agreement on its nomenclature, definition, and diagnostic criteria, promoting the potential for substantial visual loss related to misdiagnosis or late diagnosis.…”

Letter Regarding: Clinical Characteristics and Therapeutic Outcomes of Pediatric Blepharokeratoconjunctivitis

“…We have read the report by Moon et al 1 on pediatric blepharokeratoconjunctivitis (PBKC) therapeutic outcomes in a large retrospective cohort study with great interest. We congratulate the authors on their contribution to our knowledge of such a complex sight-threatening ocular surface inflammatory disease and would like to make some observations.…”