[ORO-DENTO-FACIAL MANIFESTATIONS IN PATIENTS WITH FAMILIAL DYSAUTONOMIA]

The oro-dento-facial features and dysfunctions of children with hereditary sensory and autonomic neuropathy type III (HSAN III), known as familial dysautonomia (FD) or Riley-Day syndrome, were first described in the scientific literature in 1949. They include: dental trauma, dental and soft tissue selfmutilation, normal dental age, normal sequence and timing of eruption and exfoliation of teeth, smaller tooth size, different and disproportional tooth components, normal alveolar bone height, small jaws, mild crowding and malocclusion. These individuals have different craniofacial morphology from accepted norms, but resemble norms of their ethnic origin. The subjects often display gray, pale, shiny faces with an asymmetric suffering expression; frontal bossing, with eventual hypertelorism and narrow lips; a low dental caries rate; drooling, and genuine hypersalivation. They may have changes in salivary composition and content, which influence plaque and calculus accumulation and increase the risk of gingival and periodontal diseases. They also have difficulty in controlling oral muscles, a progressive decrease in the number of tongue fungiform papillae, accompanied by a reduced number of taste buds and specific dysgeusia, but a normal sense of smell.