title: Congenital Corneal Anesthesia and Neurotrophic Keratitis: Diagnosis and Management.
abstract: Neurotrophic keratitis (NK) is a rare degenerative disease of the cornea caused by an impairment of corneal sensory innervation, characterized by decreased or absent corneal sensitivity resulting in epithelial keratopathy, ulceration, and perforation. The aetiopathogenesis of corneal sensory innervation impairment in children recognizes the same range of causes as adults, although they are much less frequent in the pediatric population. Some extremely rare congenital diseases could be considered in the aetiopathogenesis of NK in children. Congenital corneal anesthesia is an extremely rare condition that carries considerable diagnostic and therapeutic problems. Typically the onset is up to 3 years of age and the cornea may be affected in isolation or the sensory deficit may exist as a component of a congenital syndrome, or it may be associated with systemic somatic anomalies. Accurate diagnosis and recognition of risk factors is important for lessening long-term sequelae of this condition. Treatment should include frequent topical lubrication and bandage corneal or scleral contact lenses. Surgery may be needed in refractory cases. The purpose of this review is to summarize and update data available on congenital causes and treatment of corneal hypo/anesthesia and, in turn, on congenital NK.
authors.affiliation: Department of Biology, College of Science and Technology, Temple University, 1900 N. 12 Street, Philadelphia, PA 19122, USA.
authors.forename: Flavio
authors.initials: F
authors.lastname: Mantelli
authors.affiliation: Department of Sense Organs, Sapienza University, Viale del Policlinico 155, 00186 Rome, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Nardella
authors.affiliation: Neonatology Unit, Università Cattolica del Sacro Cuore Roma-Gemelli, Largo Agostino Gemelli 8, 00168 Rome, Italy.
authors.forename: Eloisa
authors.initials: E
authors.lastname: Tiberi
authors.affiliation: Cornea and Ocular Surface Unit, Ospedale San Raffaele IRCCS, Via Olgettina 60, 20132 Milan, Italy.
authors.forename: Marta
authors.initials: M
authors.lastname: Sacchetti
authors.affiliation: Department of Sense Organs, Sapienza University, Viale del Policlinico 155, 00186 Rome, Italy.
authors.forename: Alice
authors.initials: A
authors.lastname: Bruscolini
authors.affiliation: Department of Sense Organs, Sapienza University, Viale del Policlinico 155, 00186 Rome, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Lambiase
mesh_terms: D003317:Corneal Dystrophies, Hereditary / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D019317:Evidence-Based Medicine; D006801:Humans; D007634:Keratitis / Q000151:congenital / Q000175:diagnosis* / Q000628:therapy*; D016896:Treatment Outcome; D020433:Trigeminal Nerve Diseases / Q000151:congenital / Q000175:diagnosis* / Q000628:therapy*