Abnormal enamel and pulp dimensions in familial dysautonomia

In familial dysautonomia (FD), a hereditary autonomic and sensory neuropathy, somatic growth is impaired. This study was conducted to explore the possibility that tooth dimensions are altered as a consequence of neural crest dysfunction known to be present in FD. Enamel, dentin, pulp, and tooth size measurements of mandibular primary and permanent molars from FD patients were compared with those of healthy controls. It was found that although tooth size in the FD patients was smaller than normal, the enamel was thicker on the occlusal table, while the pulp chamber was smaller and disproportional to tooth size. Our results suggest distorted tooth dimensions rather than a generalized growth arrest as observed in other hereditary syndromes, such as Down's or Crouzon.