title: A clinical and histological study of dental defects in a 10-year-old girl with pseudoxanthoma elasticum and amelogenesis imperfecta.
abstract: BACKGROUND
  The prominent dental feature of a 10-year-old girl was severely hypoplastic enamel in permanent teeth.
  
  
  METHODS
  Severe dental defects were detected in a 10-year-old female patient affected by pseudoxanthoma elasticum and amelogenesis imperfecta. An orthopantomographic examination revealed a reduction of enamel thickness on the crown of all erupted and unerupted teeth, agenesis of the maxillary right second premolar, delayed eruption of mandibular first premolars, and the presence of large calcifications in all tooth pulp chambers. A detailed histological analysis of permanent mandibular first molars showed that pulp calcifications presented a concentric laminate organization and merged to almost completely obliterate the pulp chamber. Osteodentine was visible all along the pulpal surface of the radicular dentine. Broad resorption areas were present in the outermost dentine at both coronal and radicular levels. Radicular resorption areas presented a typical rectangular form and were filled with acellular cementum. Cementum thickness was highly increased on the root surface. Apposition of cellular cementum-like tissue was also observed on the coronal dentine surface.
  
  
  CONCLUSIONS
  Before treating patients affected by amelogenesis imperfecta and/or pseudoxanthoma elasticum, paediatric dentists should be aware of the presence of pulp calcifications that add to the complexity of endodontic procedures.
authors.affiliation: Département d'Odontologie Pédiatrique, Faculté d'Odontologie, Université Lyon1, Université de Lyon, Lyon, France. morrier@sante.univ-lyon1.fr
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Morrier
authors.forename: Annick
authors.initials: A
authors.lastname: Romeas
authors.forename: Emilie
authors.initials: E
authors.lastname: Lacan
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Farges
mesh_terms: D000567:Amelogenesis Imperfecta / Q000150:complications* / Q000473:pathology; D002648:Child; D003784:Dental Pulp Calcification / Q000473:pathology*; D003804:Dentin / Q000473:pathology; D019229:Dentition, Permanent; D004708:Endodontics / Q000379:methods; D005260:Female; D006801:Humans; D008963:Molar / Q000473:pathology*; D011561:Pseudoxanthoma Elasticum / Q000150:complications* / Q000473:pathology