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

Reza Dana,Sarah N Gibbs,Jay S Pepose,Arvind Saini,Pedram Hamrah,Stephen Pflugfelder,Cathleen M Mccabe,Christopher J Rapuano,Michael S Broder,Marjan Farid,Preeya K Gupta,Lisa Nijm,Paul Karpecki

doi:10.1186/s12886-021-02092-1

Abstract

BackgroundNeurotrophic 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.MethodsAn 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.ResultsThere 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.ConclusionsThese 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.

Highlights

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
NK can be classified into three stages using the Mackie classification, from the relatively mild Stage 1 and moderate Stage 2, to the more severe Stage 3 [4, 5]
The panel disagreed on only 6% of the 735 scenarios after the group discussion compared to 40% in the pre-meeting ratings (Table 1)

Summary

Introduction

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. Dua et al [1] suggested a modification of the Mackie classification, incorporating corneal esthesiometry findings, which may be more clinically relevant by including specific reference to severity and prognosis They define mild disease as epithelial changes only with no epithelial defect, manifestations of superficial punctate keratopathy and tear film instability, and a reduced or absent sensation in one or more quadrants of the cornea; moderate disease as epithelial defect with corneal anesthesia; and severe disease as stromal involvement, corneal ulcer or perforation, and corneal anesthesia. As patients typically experience few symptoms in the earlier stages, diagnosis is often delayed

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