The congenital insensitivity-to-pain syndrome (analgesia congenita): report of a case.

The congenital insensitivity-to-pain syndrome is one of several entities of sensory neuropathies in which pain sensation is absent from birth. This report describes a female child with the syndrome, who in all other aspects was normal. The most severe oral consequence of her disorder was self-inflicted exfoliation at an early age of all single-rooted primary teeth and one primary molar (a total of 13 teeth). The child was monitored from age 8. The primary aim in management was to monitor the eruption of the permanent teeth and occlusal development. At eruption of the mandibular incisors a protective splint was constructed to prevent their sharp edges injuring the tongue and the oral mucosa. The child was also instructed not to agitate her newly erupted teeth. Further occlusal development of the permanent dentition was uneventful.