Zusammenfassung
Die neurotrophe Keratopathie (NK) ist eine degenerative Hornhauterkrankung, die auf einer Beeinträchtigung der kornealen Innervation beruht. Die Schädigung der sensiblen Innervation, die durch den 1. Ast des N. trigeminus (N. ophthalmicus) erfolgt, kann über die gesamte Länge des Nervenverlaufs erfolgen, ausgehend vom Kern im Hirnstamm z. B. durch einen Hirntumor, bis hin zu den terminalen Nervenfasern in der Kornea, z. B. verursacht durch refraktive Hornhautchirurgie (z. B. Laser-in-situ-Keratomileusis [LASIK]). Bedingt durch den Verlust der sensiblen Innervation kommt es zu einer verminderten Tränensekretion und einer Reduktion in der Ausschüttung trophischer Faktoren. Dieses wiederum inhibiert das Regenerationspotenzial des kornealen Epithels. Die Reduktion bzw. der Verlust der Tränensekretion gepaart mit dem verschlechterten Regenerationspotenzial der Epithelzellen kann in schwersten Fällen der Erkrankung zu persistierenden Epitheldefekten, Ulzera bis hin zur Perforation der Hornhaut führen. Die NK weist eine Prävalenz von 5 oder weniger Betroffenen pro 10.000 auf und wird als seltene/Orphan-Erkrankung (ORPHA137596) eingestuft. Ein grundlegendes Verständnis der Pathogenese und Epidemiologie der NK unterstützt eine frühzeitige Diagnose und damit die Einleitung einer spezifischen Therapie.
Abstract
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.
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S. Mertsch und J. Alder geben an, dass kein Interessenkonflikt besteht. H.S. Dua und G. Geerling geben Tätigkeiten als Berater und Vortragende für Dompé Farmaceutici an. G. Geerling hat Mittel für die Durchführung eines selbst initiierten Forschungsprojektes von Dompé Farmaceutici erhalten.
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Mertsch, S., Alder, J., Dua, H.S. et al. Pathogenese und Epidemiologie der neurotrophen Keratopathie. Ophthalmologe 116, 109–119 (2019). https://doi.org/10.1007/s00347-018-0823-9
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DOI: https://doi.org/10.1007/s00347-018-0823-9