Mertsch S, Alder J, Dua H S, Geerling G
Univ.-Klinik für Augenheilkunde, Heinrich-Heine-Universität Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland.
Univ.-Klinik für Augenheilkunde, Pius-Hospital Oldenburg, Medizinischer Campus Universität Oldenburg, Oldenburg, Deutschland.
Ophthalmologe. 2019 Feb;116(2):109-119. doi: 10.1007/s00347-018-0823-9.
Neurotrophic keratopathy (NK) is a degenerative corneal disease that is based on an impairment of the corneal innervation. The damage to the sensory innervation, which is delivered through the 1st branch of the trigeminal nerve (ophthalmic nerve), can occur throughout the entire length of the nerve from the nucleus in the brainstem, e.g. caused by brain tumors, to the terminal nerve fibers in the cornea, caused for example by refractive corneal surgery (e. g. LASIK). Due to the loss of the sensory innervation, a reduced lacrimation and a reduction in the secretion of trophic factors occur. This in turn inhibits the regeneration potential of the corneal epithelium. In the most severe cases of the disease, the reduction or loss of lacrimation, together with the impaired regeneration potential of the epithelial cells, can lead to persistent epithelial defects, ulcers and corneal perforation. The NK has a prevalence of 5 or fewer individuals per 10,000 and is classified as a rare, i. e. orphan disease (ORPHA137596). A fundamental understanding of the pathogenesis and epidemiology of NK supports the early diagnosis and therefore the initiation of a specific treatment.
神经营养性角膜病变(NK)是一种基于角膜神经支配受损的退行性角膜疾病。通过三叉神经第一分支(眼神经)传递的感觉神经支配损伤,可发生在从脑干核团起的整个神经长度范围内,例如由脑肿瘤引起,也可发生在角膜的终末神经纤维,例如由屈光性角膜手术(如准分子激光原位角膜磨镶术)引起。由于感觉神经支配丧失,泪液分泌减少,营养因子分泌也减少。这反过来又抑制了角膜上皮的再生潜能。在该疾病最严重的情况下,泪液分泌减少或丧失,加上上皮细胞再生潜能受损,可导致持续性上皮缺损、溃疡和角膜穿孔。NK的患病率为每10000人中5人或更少,被归类为罕见病,即孤儿病(孤儿病编号:ORPHA137596)。对NK发病机制和流行病学的基本了解有助于早期诊断,从而启动特异性治疗。