Neurotrophic keratopathy in childhood: advances in understanding of pathogenesis and management

Neurotrophic keratopathy (NK) is an uncommon corneal disorder caused by trigeminal nerve dysfunction, leading to loss of ocular surface sensation, impaired corneal epithelial maintenance, and possible progressive stromal lysis. NK is of added potential visual significance in children on account of the risk of amblyopia resulting from stromal opacification. Unlike acquired NK in adult-onset disease, NK in childhood is frequently congenital or inherited, linked to genetic pain insensitivity syndromes, cranial dysinnervation disorders or broader developmental anomalies. Visual function can be well preserved in affected eyes in many children with supportive management or specific medical and surgical interventions directed at modulating sensory nerve function. A modification of the existing classification of NK stages is proposed to incorporate those eyes in which there is no detectable corneal sensation but otherwise normal eye examination, a phenotype not infrequently encountered in early NK in childhood.