Pathogenesis and epidemiology of neurotrophic keratopathy

Neurotrophic keratopathy (NK) is adegenerative corneal disease that is based on an impairment of the corneal innervation. The damage to the sensory innervation, which is delivered through the 1stbranch 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, areduced lacrimation and areduction 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 aprevalence of 5 or fewer individuals per 10,000 and is classified as arare, i.e. orphan disease (ORPHA137596). Afundamental understanding of the pathogenesis and epidemiology of NK supports the early diagnosis and therefore the initiation of aspecific treatment.