Benito-Pascual B, Arriola-Villalobos P, Díaz-Valle D, Benítez Del Castillo-Sánchez J M
Hospital Clínico San Carlos, Madrid, España.
Hospital Clínico San Carlos, Madrid, España.
Arch Soc Esp Oftalmol (Engl Ed). 2018 Oct;93(10):470-475. doi: 10.1016/j.oftal.2018.06.004. Epub 2018 Jul 18.
Polychromatic corneal dystrophy is an unusual pre-descemet dystrophy, about which there are very few publications. The findings are presented in a case series of four patients with polychromatic corneal dystrophy, using a slit lamp, specular biomicroscopy, and confocal microcospy.
Four women, between 36 and 72 year-old, with the diagnosis of polychromatic corneal dystrophy in routine reviews. None reported visual symptoms or ocular history of interest. Anterior biomicroscopy showed multiple and small multicoloured brilliant opacities in the posterior area of the corneal stroma, with normal epithelium and anterior stroma. The opacities were bilateral and distributed throughout the entire cornea. Direct family members were examined, but none of them showed opacities. In the specular biomicroscopy, a normal endothelium, with pre-descemet hypereflective particles, was observed. With confocal microscopy, there were no abnormalities in epithelium, Bowman layer, or sub-basal nervous plexus. In two cases, the anterior stroma showed hyper-reflective keratocytes and with small hypereflective particles among them. In the middle stroma, hyper-reflective keratocytes were seen in the four cases, two of them showed tiny hypereflective particles, and in the other two there were abnormal keratocytes with prominent cytoplasmic processes. Posterior stroma in the four cases showed a lot of hypereflective keratocytes and hypereflective particles of different sizes. These particles prevented examining the endothelium.
Polychromatic corneal dystrophy has typical signs that allow it to be diagnosed and characterised. Although the biomicroscopy image only seems to show alterations in the posterior stroma, confocal microscopy shows that the dystrophy affects the entire corneal stroma.
多色性角膜营养不良是一种罕见的后弹力层前营养不良,关于它的出版物非常少。本文通过裂隙灯、镜面反射显微镜和共聚焦显微镜,展示了一组4例多色性角膜营养不良患者的研究结果。
4名年龄在36至72岁之间的女性,在常规检查中被诊断为多色性角膜营养不良。她们均未报告视觉症状或有意义的眼部病史。眼前节显微镜检查显示角膜基质后部有多个小的多色性明亮混浊,上皮和前部基质正常。混浊为双侧性,分布于整个角膜。对直系亲属进行了检查,但他们均未出现混浊。在镜面反射显微镜检查中,观察到内皮正常,后弹力层前有高反射颗粒。共聚焦显微镜检查显示,上皮、Bowman层或基底神经丛无异常。在2例中,前部基质显示高反射的角膜细胞,其中有小的高反射颗粒。在4例患者的中部基质中均可见高反射的角膜细胞,其中2例有微小的高反射颗粒,另外2例有异常的角膜细胞,其细胞质突起明显。4例患者的后部基质均显示有大量高反射的角膜细胞和不同大小的高反射颗粒。这些颗粒妨碍了对内皮的检查。
多色性角膜营养不良有典型的体征,可据此进行诊断和特征描述。虽然眼前节显微镜图像似乎仅显示后部基质的改变,但共聚焦显微镜显示该营养不良影响整个角膜基质。