Picnodisostose e suas repercussões orofaciais: relato de caso

Abstract

Picnodysostosis is an extremely rare syndrome in which there is a mutation in Cathepsin K enzyme that encodes osteoclasts interfering directly in bone remodeling due to deficiency in the organic matrix’s resorption. Thus, there are important orofacial repercussions that need to be known and studied. The objective of this study is to report a case of Picnodysostosis describing their physical, clinical and radiographic features with a dental approach and related to the challenges and possibilities of orthodontic treatment in these patients, based in literature. A 9 years and half old, male and syndrome patient, who reached the pediatric dentistry clinic of the Federal University of Ceará with dental follow-up aim was described in this study. Clinical procedures of oral adequation, molding, confection and installation of fixed orthodontic appliance were performed. There has not been yet a defined protocol reported in literature that ensures dentists about dental and orthopedic movement in patients with this type of dysostosis. Therefore, this is a matter that is understudied in the orthodontic and orthopedic field, which needs further research, once it is typical to the syndrome’s patients the great need of this type of treatment.