Abstract
BackgroundNeurotrophic keratopathy (NK) is an orphan disease, with an estimated prevalence of 1–5/10,000. No data regarding the incidence exists. The primary aim was to evaluate incidence and prevalence of NK at a tertiary referral center in Germany, and the secondary aim was to analyze demographic parameters, etiology, and clinical features and therapeutic outcomes.Methods and materialAll patients treated for NK with serum eye drops (SED), amnionic membrane transplantation (AMT), or penetrating keratoplasty (PK) in 2013–2017 were identified. Age, sex, etiology of NK, visual acuity, disease stage, treatment, and visual acuity were analyzed. Incidence and prevalence of NK in our hospital and the overall population of the city were calculated.ResultsIn 63 eyes of 60 patients (56.7% male; 68 ± 16 years), the most common underlying diseases were herpetic infections (23.8%), neurological causes (19%), and diabetes mellitus (14.3%). The annual incidence of NK in our tertiary referral center ranges between 5/10,000 and 3/10,000, the prevalence between 9/10,00 and 22/10,000. In all patients treated with corneal ulcers, the prevalence was up to 27% (2706/10,000). The incidence in the overall population is estimated at 0.1–0.3/10,000, the prevalence at 0.2–0.5/10,000 to 0.5/10,000.ConclusionBased on our assessment, the prevalence of NK in the overall population is lower than estimated before. However, in patients with corneal ulcers, the percentage of NK is comparably high. The disease may still be underdiagnosed due to the variety of underlying disorders and unknown comorbidities. Thus, in cases of therapy-refractive superficial keratopathy or ulcerations, NK should be considered more frequently.
Highlights
Neurotrophic keratopathy (NK) is a corneal condition in which impaired corneal innervation by the ophthalmic branch of the trigeminal nerve leads to reduced corneal healing, epithelial defects, corneal ulceration, melting, and even perforation possibly resulting in a serious loss of visual acuity or even blindness [1, 2]
The clinical records of patients who had been treated with serum eye drops (SED), amnionic membrane transplantation (AMT), or penetrating keratoplasty (PK) for a first episode of NK during the years 2013–2017 were retrospectively analyzed
A total of 1849 ± 157 individuals were treated as inpatients and 92 ± 11 were treated for a corneal ulcer (H16.0)
Summary
Neurotrophic keratopathy (NK) is a corneal condition in which impaired corneal innervation by the ophthalmic branch of the trigeminal nerve leads to reduced corneal healing, epithelial defects, corneal ulceration, melting, and even perforation possibly resulting in a serious loss of visual acuity or even blindness [1, 2].NK is an orphan disease (ORPHA:137596, www.orpha.net) and epidemiological data on this condition are limited. The estimation of the overall prevalence of 1–5/10,000, respectively 1/2380 in Europe, as stated on www.orpha.net, is mainly based on the prevalence of triggering pathomechanisms such as herpetic infections or iatrogenic damage after neurosurgical intervention [3,4,5]. Since this estimate does not include other possibly underlying conditions and causes such as e.g. diabetes mellitus or neurodegenerative diseases as multiple sclerosis, the actual prevalence is assumed to be higher [3, 4]. In cases of therapy-refractive superficial keratopathy or ulcerations, NK should be considered more frequently
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