PBKC is an underdiagnosed chronic and recurrent inflammatory disorder with significant ocular surface morbidity during childhood. [1, 2, 8, 11, 20–22] PBKC usually presents in children aged 6.2 ± 2.4 years [2, 6, 8, 20], and self-limits between 9.5 to 13 years. [2, 21] This reported age of self-limitation is like the mean age reported in our study, which excluded patients with active disease (10.39 ± 3.81 years). Although some series do not report a significant predisposition to the disease by gender [2, 23], others have reported a female predominance. [6, 9, 22] No clear gender predisposition was found in these series (p = 0.461).
Corneal involvement has been reported in up to 55 to 81% of PBKC eyes [1, 8, 22]; hence, severe visual impairment is potentially related to corneal inflammation and scarring-induced refractive alterations and opacity. Therefore, considering that this sight-threatening disorder affects childhood and taking advantage of the ease and speed of acquiring a complete visual system analysis with the OPD Scan-III device, we decided to assess the eyes of PBKC children with corneal involvement and compare them with a healthy control group. Furthermore, although the eye's optical aberration analysis has recently become an important issue in ocular surface diseases, we still lack normative values in children. [24, 25]
Limited evidence exists regarding ocular surface disorders and the impact of optical aberrations on visual outcomes. [26, 27] In this study, PBKC patients had a significantly lower (0.24 LogMAR) CDVA than the control group (0.07 LogMAR). Teo et al. [22] reported that up to one-third of PBKC eyes might have poor vision (< 0.3 LogMAR) at presentation. Likewise, Jones et al. [8] reported a median monocular CDVA of 0.28 LogMAR in affected eyes at presentation. They demonstrated that a 2-year lag between disease onset and presentation could result in a 0.06 LogMAR-unit reduction in visual acuity, emphasising the disease can result in significant visual loss. [8] Further evidence has shown that the diagnosis of PBKC is usually delayed up to more than three years, correlating with worse visual outcomes. [1, 6]
The PBKC group had significantly higher astigmatism than the control group. In contrast, Doan et al. [21] found a mean astigmatism of 0.80 ± 0.89D in PBKC patients; 52% had astigmatism of at least 0.75D attributable to corneal scarring. Also, a significant increase in cornea astigmatism and ectasia pattern with no changes in average K values has been reported in adult patients with phlyctenular keratitis.[28] However, the average keratometry values did not differ between our study's PBKC and control groups.
Zhang et al. [24] studied 1,634 healthy children's eyes between 5 and 17 years old, finding a considerable variability in the presence of total HOAs. The mean total HOAs in the preschool age (5–7 years) was 0.338 ± 0.125, during the scholar age (8–12 years) was 0.353 ± 0.224, and during adolescence (13–17 years) was 0.400 ± 0.250. [24] In our healthy control population, including children between 6 to 16 years of age, the mean HOAs value was 0.41 ± 0.18, like previous studies on Asian and Oceanic healthy children. [24, 25, 29].
Chan et al. [30] reported the effect of corneal involvement in children with vernal keratoconjunctivitis, concluding that chronic inflammation on the ocular surface produced by this chronic allergic disease induces wavefront optical aberrations consisting of higher levels of HOAs RMS values and coma. We also found a significantly higher value of total HOAs in PBKC eyes than in healthy controls (Fig. 2).
The presence of leucoma, corneal vascularization, phlyctenula, and pannus formation demonstrated a higher risk of presenting elevated HOAs (Table 3). The association between these corneal manifestations and the increased likelihood of developing HOAs might be explained because these lesions may affect the visual axis and, therefore, often involve the physiologic pupil size studied by OPD-Scan. We could not relate peripheral stromal infiltrates, focal corneal thinning, peripheral ulcerative keratitis (PUK), and corneal perforation to increased HOAs because a limited number of eyes were suffering from these clinical manifestations. In addition to the peripheral location of corneal lesions that were not included in the OPD-Scan analysis.
Finally, OPD-Scan includes simulated analyses of the eye's optical quality and visual performance. [31] The MTF is an optical bench measurement used to evaluate the performance of an optical system, and aberrations contribute to a poor MTF value. [32] However, while RMS error and MTF are strongly correlated, it is not perfect, and in aberrated eyes, the interrelation of RMS and MTF is strongly dependent on the relative magnitudes of all Zernike coefficients. [33] Nevertheless, there was a significant relationship between reduced MTF and prominent HOAs in the PBKC group in this study (Fig. 1). On the other hand, there was no difference in the distortion level of a simulated point source of light measured by the Strehl ratio from the PSF analysis between groups, which is probably related to the localization of the peripherical corneal lesions in the PBKC and the presence of lower-order aberration in the control group.
Limitations encountered during the planning and execution of this study were the difficulty in evaluating visual acuity by contrast sensitivity and glare test in younger children, which would have improved our knowledge of the effect of PBKC corneal lesions on visual performance. Also, the frequency of recurrence or persistent inflammation hinders the availability of OPD-Scan analysis in PBKC patients. Moreover, although the device possesses an excellent eye visual fixation system and a quick data acquisition, it is always challenging to achieve children´s cooperation to perform a reliable OPD-Scan analysis. Also, longer follow-up periods and a larger series would be ideal for establishing the long-term effect of the HOAs on these children´s visual acuity.
In conclusion, this study addresses for the first time the visual impact of optical aberrations caused by PBKC, describing the corneal findings leading to increased HOAs. It demonstrates that astigmatism and HOAs are frequently observed in PBKC, with a higher impact on asymmetric disease, reducing visual acuity and quality. Wavefront aberrometry is a valuable tool to evaluate the eye´s optical system, allowing a fast and accurate analysis in chronic BKC children and a comprehensive assessment of the affected eye's optical quality by clinicians. Such information is crucial for making therapeutic decisions in the visual rehabilitation stage of these patients.