PDF(Pubmed)
Abstract：
BACKGROUND: A report of a Brittle cornea syndrome (BCS) case with bluish scleral discoloration, keratoglobus, and myopia based on multimodal imaging modalities including in vivo confocal microscopy (IVCM), high-definition optical coherence tomography (HD-OCT) and scheimpflug corneal densitometry analysis.
METHODS: A 36-year-old Chinese female patient presented with significant bluish discoloration of the sclera in both eyes, extreme corneal thinning with increased corneal curvature, increased central corneal densitometry, and nystagmus. She also had scoliosis, severe osteoporosis, and thyroid disease.
CONCLUSIONS: Timely diagnosis, early detection, and detailed follow-up are essential for BCS. There has been no report of a BCS evaluation performed by IVCM and corneal densitometry methods thus far in the literature. Furthermore, multimodal imaging can offer a more comprehensive view of BCS and contribute to a deeper understanding of the disease. Interestingly, this is a rare case of BCS in an adult with good vision, an intact cornea, and nystagmus.
METHODS: A 36-year-old Chinese female patient presented with significant bluish discoloration of the sclera in both eyes, extreme corneal thinning with increased corneal curvature, increased central corneal densitometry, and nystagmus. She also had scoliosis, severe osteoporosis, and thyroid disease.
CONCLUSIONS: Timely diagnosis, early detection, and detailed follow-up are essential for BCS. There has been no report of a BCS evaluation performed by IVCM and corneal densitometry methods thus far in the literature. Furthermore, multimodal imaging can offer a more comprehensive view of BCS and contribute to a deeper understanding of the disease. Interestingly, this is a rare case of BCS in an adult with good vision, an intact cornea, and nystagmus.
摘要：
背景：报告了脆性角膜综合征（BCS）伴蓝色巩膜变色的病例，keratoglobus,和基于多模态成像模式的近视，包括体内共聚焦显微镜（IVCM），高清光学相干断层扫描（HD-OCT）和scheimpflug角膜密度测定分析。
方法：一名36岁的中国女性患者，双眼巩膜呈明显的蓝色变色，角膜极度变薄，角膜曲率增加，中央角膜密度测定增加，和眼球震颤.她也有脊柱侧弯,严重的骨质疏松症,甲状腺疾病。
结论：及时诊断，早期发现,详细的跟进对于BCS至关重要。迄今为止，文献中还没有关于通过IVCM和角膜密度测定法进行BCS评估的报道。此外，多模态成像可以提供更全面的BCS视图，并有助于更深入地了解疾病。有趣的是,这是一个罕见的BCS在一个有良好视力的成年人，完整的角膜,和眼球震颤.
方法：一名36岁的中国女性患者，双眼巩膜呈明显的蓝色变色，角膜极度变薄，角膜曲率增加，中央角膜密度测定增加，和眼球震颤.她也有脊柱侧弯,严重的骨质疏松症,甲状腺疾病。
结论：及时诊断，早期发现,详细的跟进对于BCS至关重要。迄今为止，文献中还没有关于通过IVCM和角膜密度测定法进行BCS评估的报道。此外，多模态成像可以提供更全面的BCS视图，并有助于更深入地了解疾病。有趣的是,这是一个罕见的BCS在一个有良好视力的成年人，完整的角膜,和眼球震颤.
