Neurotrophic keratopathy

Neurotrophic keratopathy (also known as neurotrophic keratitis or neurotrophic corneal ulcer) is a degenerative disease of the cornea caused by impairment of corneal innervation, most commonly due to damage to the trigeminal nerve (cranial nerve V). The loss of corneal sensation leads to a breakdown of the corneal epithelium, impaired healing, and progressive corneal damage that can ultimately result in corneal perforation and vision loss. The condition primarily affects the eye, specifically the corneal surface, but its underlying causes can involve the central or peripheral nervous system. Neurotrophic keratopathy is classified into three stages of increasing severity. Stage 1 is characterized by superficial punctate keratopathy with corneal epithelial irregularities and dryness. Stage 2 involves persistent epithelial defects, typically presenting as a smooth-bordered oval ulcer most often located in the upper half of the cornea. Stage 3 is the most severe, featuring deep corneal ulceration with stromal melting that can progress to corneal perforation. Patients may experience decreased visual acuity, redness, and paradoxically may report little or no pain due to the reduced corneal sensitivity that defines the condition. The causes of neurotrophic keratopathy are diverse and include herpes simplex or herpes zoster keratitis, surgical damage to the trigeminal nerve (such as during procedures for trigeminal neuralgia or acoustic neuroma), chemical burns, diabetes mellitus, multiple sclerosis, and congenital conditions such as familial dysautonomia (Riley-Day syndrome). Treatment depends on the stage of disease and ranges from preservative-free artificial tears and therapeutic contact lenses in early stages to surgical interventions such as tarsorrhaphy or amniotic membrane transplantation in advanced cases. Cenegermin (recombinant human nerve growth factor) eye drops represent a significant therapeutic advance, having been approved specifically for the treatment of neurotrophic keratitis, and have demonstrated the ability to promote corneal healing by restoring neurotrophic support to the corneal surface.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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