Because of the medical characteristics of this inheritable disorder, t
he treatment of these patients can be fraught with potential pitfalls.
Oral manifestations can include taurodontism of the pulp chambers, re
trognathia, palatal constriction with attendant posterior crossbite, f
rontal tongue posturing, and mouth breathing relating to airway restri
ction from choanal atresia. The authors describe their treatment of a
patient three years of age with a tracheostomy.