Purpose

Assess epidermolysis bullosa (EB)-related corneal pathology using anterior segment optical coherence tomography (AS-OCT) and correlate imaging with clinical metrics in EB patients versus age-matched controls.

Methods

EB patients and controls were recruited during an EB conference (July 2018) and at Tufts Medical Center (June–August 2019). Subjects completed a questionnaire, had best corrected visual acuity (BCVA) tested, and underwent AS-OCT scanning. Stromal and epithelial thickness were measured. Depth, length, and type of the three largest lesions were assessed by a masked examiner using a novel pathology grading system. Multivariate analysis of AS-OCT findings and clinical metrics was performed.

Results

62 EB patients and 60 age-matched controls were enrolled. Mean BCVA was 1.8 lines worse in patients (p < 0.001). Vision loss was associated with increased stromal thickness. Discrete lesions were seen in 60.2% of patient eyes, averaging 1.71 ± 1.75 lesions in patients and 0.14 ± 0.42 in controls (p < 0.001). Mean primary lesion depth was 151.88 ± 97.49 μm in patients. Patients showed significant stromal thickening versus controls and lesions were most common in the periphery and inferiorly. Differences in frequency and duration of abrasions and severity of pain were all statistically and clinically significant in patients versus controls (p < 0.001).

Conclusions

AS-OCT can visualize and quantify differences in the corneas of EB patients compared with age-matched controls. Novel findings include quantification of average depth, length, and severity of discrete lesions, and sparing of the superior quadrant from stromal thickening in EB patients. These results support use of AS-OCT and a questionnaire in clinical trials for new EB therapies.

中文翻译：

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大疱表皮松解术的前段光谱域光学相干断层扫描

目的

使用前段光学相干断层扫描（AS-OCT）评估大疱性表皮松解（EB）相关的角膜病理，并将EB患者与年龄匹配的对照组的影像学与临床指标相关联。

方法

在EB会议（2018年7月）和塔夫茨医疗中心（2019年6月至2019年8月）期间招募了EB患者和对照组。受试者完成了问卷，测试了最佳矫正视力（BCVA），并接受了AS-OCT扫描。测量基质和上皮厚度。戴面具的检查员使用新型病理分级系统，可以评估三个最大病变的深度，长度和类型。进行了AS-OCT结果和临床指标的多变量分析。

结果

招募了62名EB患者和60名年龄匹配的对照。患者的平均BCVA差1.8行（p <0.001）。视力下降与基质厚度增加有关。在60.2％的患者眼中可见离散的病变，患者的平均病变为1.71±1.75，对照组为0.14±0.42（p <0.001）。患者的平均原发灶深度为151.88±97.49μm。与对照相比，患者显示出明显的间质增厚，并且病变最常见于周围和下部。与对照组相比，擦伤频率和持续时间以及疼痛严重程度的差异在统计学和临床​​上均具有统计学意义（p <0.001）。

结论

与年龄匹配的对照组相比，AS-OCT可以可视化和量化EB患者角膜的差异。新颖的发现包括对EB患者的平均深度，长度和离散病变的严重程度进行定量，并保留了由间质增厚引起的上象限。这些结果支持在新的EB疗法的临床试验中使用AS-OCT和问卷。