Vesaluoma M H, Linna T U, Sankila E M, Weiss J S, Tervo T M
Helsinki University Eye Hospital, Finland.
Ophthalmology. 1999 May;106(5):944-51. doi: 10.1016/S0161-6420(99)00514-X.
To analyze corneal morphology in Schnyder crystalline corneal dystrophy (SCCD) in vivo.
Observational case series.
Five eyes of four patients of various belonging to the same family were examined.
The eyes were examined using in vivo confocal microscopy (CM).
The corneal morphology including keratocytes and stromal extracellular matrix, as well as basal epithelial/subepithelial nerves is, described.
The right eye of a 48-year-old male patient had been treated with anterior keratectomy and the left eye with phototherapeutic keratectomy (PTK). The right eye presented with increased stromal reflectivity owing to accumulation of extracellular matrix and large subepithelial crystalline deposits. Far fewer crystals could be observed in the left eye. The haze, however, was increased, either because of the dystrophy or the excimer laser treatment. The anterior keratocytes appeared irregular, and the subepithelial nerves were undetectable in both eyes. His 78-year-old mother showed more advanced changes with dense crystals, highly fibrotic stroma, and severely damaged corneal innervation. The partly irregular anterior keratocytes of the 9- and 7-year-old children contained intracellular deposits, although the corneas were clinically clear with only subtle subepithelial crystalline formation. Accumulation of similar reflective material was also observed in association with the prominent subepithelial nerves.
In the early stages of SCCD, highly reflective deposits accumulate intracellularly and around anterior keratocytes and along subepithelial nerves. With time, the normal corneal architecture becomes disturbed by large extracellular crystalline deposits and accumulation of highly reflective extracellular matrix resulting in central opacity and disruption of the subepithelial nerve plexus. Furthermore, neural regeneration after keratectomy appears delayed in SCCD.
在体分析施奈德结晶性角膜营养不良（SCCD）的角膜形态。
观察性病例系列。
对来自同一家族的4例患者的5只眼进行检查。
使用共聚焦显微镜（CM）对眼睛进行检查。
描述角膜形态，包括角膜细胞和基质细胞外基质，以及基底上皮/上皮下神经。
一名48岁男性患者的右眼接受了前板层角膜切除术，左眼接受了光治疗性角膜切削术（PTK）。右眼由于细胞外基质积聚和大量上皮下结晶沉积物而呈现基质反射率增加。左眼观察到的晶体要少得多。然而，由于营养不良或准分子激光治疗， haze增加。前角膜细胞出现不规则，两只眼睛均未检测到上皮下神经。他78岁的母亲表现出更严重的变化，有密集的晶体、高度纤维化的基质和严重受损的角膜神经支配。9岁和7岁儿童的部分不规则前角膜细胞含有细胞内沉积物，尽管临床上角膜清晰，仅有轻微的上皮下结晶形成。在突出的上皮下神经周围也观察到类似反射物质的积聚。
在SCCD的早期阶段，高反射性沉积物在细胞内、前角膜细胞周围以及上皮下神经周围积聚。随着时间的推移，正常的角膜结构会受到大的细胞外结晶沉积物和高反射性细胞外基质积聚的干扰，导致中央混浊和上皮下神经丛的破坏。此外，SCCD患者角膜切除术后的神经再生似乎延迟。
