Bourges J-L
Université Paris Descartes, Sorbonne Paris Cité, 15, rue de l'École-de-Médecine, 75006 Paris, France; Ophthalmology Unit, Ophthalmology Service, Hôtel-Dieu, Hôpitaux Universitaires Paris Centre, Assistance publique-Hôpitaux de Paris, 1, place du Parvis-Notre-Dame, 75004 Paris, France; Équipe 17, Inserm UMRS 1138, Centre de Recherche des Cordeliers, 15, rue de l'École-de-Médecine, 75006 Paris, France.
J Fr Ophtalmol. 2017 Jun;40(6):e177-e192. doi: 10.1016/j.jfo.2017.05.003. Epub 2017 Jun 3.
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. In practice, 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.
退行性或遗传性角膜疾病有时难以鉴别。角膜营养不良影响约0.09%的人口。它们根据其表型、基因型以及诊断所收集的证据通过IC3D分类来识别。在实践中,眼科医生处理诸如反复角膜糜烂、视力丧失和弱视、畏光、异物感,有时还有疼痛和美观问题等功能症状。药物治疗包括促进愈合的滴眼液、眼膏、高渗剂和绷带式隐形眼镜。侵入性较小的手术治疗用作二线治疗(光治疗性角膜切削术、板层角膜切除术)。最终可能会采用侵入性更强的手术(板层或穿透性角膜移植术)。尽管在某些情况下穿透性角膜移植术仍是唯一可行的选择,但现在前板层或内皮角膜移植术比穿透性角膜移植术更受青睐。一些罕见的营养不良需要协调和全面的医疗护理。