Unilateral ulceration of the cornea secondary to congenital trigeminal nerve agenesis.

PURPOSE: To report a case of unilateral ulceration of the cornea secondary to congenital trigeminal nerve agenesis. METHODS: Case report. RESULTS: We report the case of a 7-month-old boy referred to our department for left infectious keratitis. An impassive reaction to eyedrops and bandage contact lenses was noted during the follow-up examinations and corneal sensitivity remained negative. Mouth ulcers and self-injury in the left nostril were detected during these follow-up consultations. These symptoms were secondary to self-mutilation and associated with anesthesia in the distribution of all divisions of the left trigeminal nerve. Congenital trigeminal nerve agenesis was confirmed by magnetic resonance imaging slices at age 2. CONCLUSIONS: Magnetic resonance imaging can confirm congenital nerve agenesis, a cause of childhood neurotrophic keratitis. Radiologic confirmation of agenesis allows elimination of other diagnoses.