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 tarsorrhaphies. METHODS: Case reports. A 2-month-old infant presented with bilateral corneal erosions and complete corneal anesthesia. Her sister presented at age 3 years with a corneal ulcer and corneal hypoesthesia (sensation markedly decreased). The father and paternal grandmother of the siblings also had corneal hypoesthesia. RESULTS: Further investigation of the infant revealed bilateral hearing loss, swallowing difficulties, and decreased sensation in the trigeminal nerve distribution. A diagnosis of congenital trigeminal anesthesia was made. The 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 ocular lubrication and superficial corneal scar removal. Her older sister initially 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 tarsorrhaphies 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 congenital trigeminal anesthesia in more than two generations. Early recognition of this condition is essential in the preservation of useful vision.