Congenital trigeminal anesthesia in two siblings and their long-term follow-up

PURPOSE: To alert ophthalmologists to congenital trigeminal anesthesia as a cause of corneal scarring and amblyopia and its effective treatment with t arsorrhaphies, METHODS: Case reports. A 2-month-old infant presented with bilateral cornea l erosions and complete corneal anesthesia. Her sister presented at age 3 y ears with a corneal ulcer and corneal hypoesthesia (sensation markedly decr eased). The father and paternal grandmother of the siblings also had cornea l hypoesthesia. RESULTS: Further investigation of the infant revealed bilateral hearing los s, swallowing difficulties, and decreased sensation in the trigeminal nerve distribution. A diagnosis of congenital trigeminal anesthesia was made. Th e corneal erosions of the patient resolved with bilateral two-thirds width tarsorrhaphies. The girl continues to do well now at 10 years of age with o cular lubrication and superficial corneal scar removal. Her older sister in itially required antibiotic ointment for her corneal ulcer but now requires only ocular lubrication for congenital trigeminal anesthesia. CONCLUSION: This study describes the earliest reported use of tarsorrhaphie s in an infant with congenital trigeminal anesthesia. The presence of this condition in her sister and relatives makes it one of the few reports of co ngenital trigeminal anesthesia in more than two generations. Early recognit ion of this condition is essential in the preservation of useful vision. (A m J Olphthalmol 2000;129:96-98. (C) 2000 by Elsevier Science Inc. All right s reserved.)