Incidence, prevalence, and outcome of moderate to severe neurotrophic keratopathy in a German tertiary referral center from 2013 to 2017

Background Neurotrophic 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 material All 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. Results In 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. Conclusion Based 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.

Neurotrophic keratopathy and Wallenberg – Zakharchenko syndrome: a clinical case

BACKGROUND: Degenerative changes of cornea after transection of the trigeminal nerve were first described by F. Magendie in 1824. Neurotrophic keratopathy is considered to be an orphan disease which lately has been recognized more and more often. According to literature data, neurotropchic keratopathy affects 5 individuals in 10,000. The diagnosis is difficult due to the lack of information about this condition, the rare occurrence and the presence of a large number of etiological factors. AIM: To determine the causes of the neurotrophic keratopathy development and the treatment tactics in a patient with a neurological disease. The article presents a case of neurotrophic keratopathy in a patient with Wallenberg Zakharchenko syndrome. Because of the fact that neurotrophic keratopathy was diagnosed late and the correct treatment did not start in time, further progression of the pathological process in the cornea could not be avoided. Periodic recurrence of neurotrophic keratopathy is associated with an underlying chronic neurological disease. CONCLUSIONS: Neurotrophic keratopathy requires early diagnosis. In certain clinical cases, for the successful treatment of this pathology, it is necessary to prescribe systemic therapy.

Long-term clinical outcome and satisfaction survey in patients with neurotrophic keratopathy after treatment with cenegermin eye drops or amniotic membrane transplantation

Purpose Neurotrophic keratopathy (NK) is a degenerative corneal disease caused by damage of trigeminal innervation. The purpose of this study is to evaluate the clinical outcomes and patient-reported satisfaction of treatment with amniotic membrane transplantation (AMT) or cenegermin eye drops in patients with NK. Methods Clinical charts of patients with NK treated with AMT (group A) or cenegermin eye drops (group B), with at least 12 months of follow-up, were reviewed for demographics, medical history, corneal healing, and disease recurrence. Patient satisfaction was evaluated by a newly developed questionnaire investigating patient’s appreciation of treatment of NK (2 items) and satisfaction with NK treatment outcomes (5 items). Results At the end of treatment, complete corneal healing was observed in 13/15 (86%) patients in group A and in 23/24 (96%) in group B. At 12 months follow-up, 6/13 patients (46%) in group A and 3/23 patients (13%) in group B showed recurrence of NK (p = 0.037). Survival analysis showed that group B remained recurrence free for a significantly longer period of time than the group A (p = 0.028). Patients in group B showed a significantly higher satisfaction when compared with patients in group A (total score: 65.7 ± 15.7 vs 47.4 ± 12.8, p = 0.003), both in terms of patients’ appreciation of treatment (78.3 ± 15.9 vs 52.2 ± 30, p = 0.020) and satisfaction with treatment outcomes (60.7 ± 21 vs 45.4 ± 13.3, p = 0.037). Conclusions Treatment of NK with cenegermin was associated with long-term maintenance of corneal integrity and a higher degree of patient satisfaction.

Corneal neurotisation for neurotrophic keratopathy with a sural nerve autograft: a case report

Neurotrophic keratopathy is a corneal disease characterised by reduced corneal sensation. Corneal neurotization is the transfer of healthy donor nerve tissue to the cornea to restore sensation. An 11-year-old male presented with reduced Mackie Stage 1 neurotrophic keratopathy from de-bulking of a cerebellopontine angle arachnoid cyst. He underwent minimally invasive indirect corneal neurotization with a sural nerve autograft to ipsilateral supratrochlear nerve and cornea. Close and objective post-operative monitoring of donor sites, the cornea, visual acuity, and tear production clearly demonstrate the efficacy of this technique, and the timeline of clinical improvement.

Expert consensus on the identification, diagnosis, and treatment of neurotrophic keratopathy

Background Neurotrophic keratopathy (NK) is a relatively uncommon, underdiagnosed degenerative corneal disease that is caused by damage to the ophthalmic branch of the trigeminal nerve by conditions such as herpes simplex or zoster keratitis, intracranial space-occupying lesions, diabetes, or neurosurgical procedures. Over time, epithelial breakdown, corneal ulceration, corneal melting (thinning), perforation, and loss of vision may occur. The best opportunity to reverse ocular surface damage is in the earliest stage of NK. However, patients typically experience few symptoms and diagnosis is often delayed. Increased awareness of the causes of NK, consensus on when and how to screen for NK, and recommendations for how to treat NK are needed. Methods An 11-member expert panel used a validated methodology (a RAND/UCLA modified Delphi panel) to develop consensus on when to screen for and how best to diagnose and treat NK. Clinicians reviewed literature on the diagnosis and management of NK then rated a detailed set of 735 scenarios. In 646 scenarios, panelists rated whether a test of corneal sensitivity was warranted; in 20 scenarios, they considered the adequacy of specific tests and examinations to diagnose and stage NK; and in 69 scenarios, they rated the appropriateness of treatments for NK. Panelist ratings were used to develop clinical recommendations. Results There was agreement on 94% of scenarios. Based on this consensus, we present distinct circumstances when we strongly recommend or may consider a test for corneal sensitivity. We also present recommendations on the diagnostic tests to be performed in patients in whom NK is suspected and treatment options for NK. Conclusions These expert recommendations should be validated with clinical data. The recommendations represent the consensus of experts, are informed by published literature and experience, and may improve outcomes by helping improve diagnosis and treatment of patients with NK.