Pycnodysostosis: Characteristics of teeth, mouth and jaws

Author:

Ferlias Nikolaos1ORCID,Gjørup Hans2,Doherty Mia Aagaard34,Pedersen Thomas Klit15

Affiliation:

1. Department of Dentistry and Oral Health, Section of Orthodontics Aarhus University Aarhus Denmark

2. Department of Oral and Maxillofacial Surgery, Center for Oral Health in Rare Diseases Aarhus University Hospital Aarhus Denmark

3. Childhood Cancer Research Group Danish Cancer Research Institute Copenhagen Denmark

4. Department of Pediatrics and Adolescent Medicine Aarhus University Hospital Aarhus Denmark

5. Department of Oral and Maxillofacial Surgery Aarhus University Hospital Aarhus Denmark

Abstract

AbstractObjectivesTo describe the clinical and radiographic oro‐dental characteristics of patients with pycnodysostosis (PDO).Materials & MethodsA short interview and clinical examination of seven patients with PDO were performed as well as assessment of the temporomandibular joints and masticatory muscles using the diagnostic criteria for temporomandibular disorders, DC‐TMD form. A full set of records were taken including photos and intraoral scan. Finally, existing cone beam computed tomography (CBCT) images and radiographs were also studied.ResultsAll patients presented with bimaxillary micrognathia, five had a convex profile, and two had a straight profile. In addition, posterior open bite, Angle Class III molar relation with accompanying anterior crossbite and a grooved median palate were common findings. No patient showed symptoms of temporomandibular disorder (TMD) apart from some clicking. Finally, the main radiographic findings were the obtuse mandibular angle, the frontal bossing, the elongation of the coronoid/condylar process and the presence of hypercementosis with obliterated pulp chambers.ConclusionThe examined patients with PDO were characterized by dental crowding, malocclusion (anterior crossbite, posterior open bite), hypercementosis, obliterated pulp chambers and deviations in mandibular morphology. In conclusion, patients with PDO have a specific need for dental and orthodontic monitoring with focus on crowding and posterior open bite. The patients will benefit from a long‐term orthodontic plan including extractions.

Publisher

Wiley

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