关键词: Affection héréditaire de cornée Classification Corneal dystrophy Dystrophie de cornée Dystrophie endothéliale Dystrophie granulaire Dystrophie stromale Dystrophie épithéliale Endothelial dystrophy Epithelial dystrophy Genetic Granular dystrophy Génétique Hereditary corneal condition Keratectomy Keratoplasty Kératectomie Kératoplastie Stromal dystrophy

Mesh : Cogan Syndrome / classification diagnosis therapy Corneal Diseases / classification diagnosis etiology therapy Corneal Dystrophies, Hereditary / classification diagnosis therapy Diagnosis, Differential Humans Keratoplasty, Penetrating Lasers, Excimer Photorefractive Keratectomy

来  源:   DOI:10.1016/j.jfo.2017.02.004   PDF(Sci-hub)

Abstract:
Degenerative or hereditary corneal diseases are sometimes difficult to discriminate. Corneal dystrophies affect approximately 0.09 % of the population. They are identified by the IC3D classification based on their phenotype, genotype and evidence gathered for their diagnosis. Practically, the ophthalmologist manages functional symptoms, such as recurrent erosions, visual loss and amblyopia, photophobia, foreign body sensation, and sometimes pain and aesthetic concerns. Medical treatments consist of drops to promote healing, ointments, hyperosmotic agents and bandage contact lenses. Less invasive surgical treatments are used as second line therapy (phototherapeutic keratectomy, lamellar keratectomy). More invasive procedures may eventually be utilized (lamellar or penetrating keratoplasty). Anterior lamellar or endothelial keratoplasty are now preferred to penetrating keratoplasty, although the latter still remains the only possible option in some cases. Some rare dystrophies require coordinated and comprehensive medical care.
摘要:
退行性或遗传性角膜疾病有时难以区分。角膜营养不良影响约0.09%的人口。它们根据其表型通过IC3D分类进行识别,基因型和为他们的诊断收集的证据。实际上,眼科医生管理功能性症状,比如经常性的糜烂,视力丧失和弱视,畏光,异物感,有时疼痛和美学问题。药物治疗包括促进愈合的滴剂,软膏,高渗剂和绷带隐形眼镜。微创手术治疗被用作二线治疗(光疗角膜切除术,板层角膜切除术)。最终可以使用更多的侵入性程序(层状或穿透性角膜移植术)。前板层或内皮角膜移植术现在优于穿透性角膜移植术,尽管在某些情况下,后者仍然是唯一可能的选择。一些罕见的营养不良需要协调和全面的医疗护理。
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