6533b82ffe1ef96bd12947c7
RESEARCH PRODUCT
Clinical and radiographic features of pycnodysostosis: A case report
Luciano Barreto SilvaPâmella Recco ÁLvaresPriscila Prosini Da FonteFernando-antônio GomesAna-paula SobralJosé Alcides Almeida De ArrudaCleomar Donizeth RodriguesMárcia Maria Fonseca Da Silveirasubject
medicine.medical_specialtyOral Medicine and Pathologymedicine.diagnostic_testNasal bridgebusiness.industryRadiographyCase ReportPhysical examination030206 dentistryDiseaseProminent cheeks:CIENCIAS MÉDICAS [UNESCO]medicine.diseaseDermatologyMidface hypoplasia03 medical and health sciencesFrontal Bossing0302 clinical medicineUNESCO::CIENCIAS MÉDICASPycnodysostosismedicinebusinessGeneral Dentistry030217 neurology & neurosurgerydescription
Pycnodysostosis is a rare disorder that was first described in 1962; however, it was only in 1996 that the defective gene was discovered, which led to a better understanding of this disease. This study reports and discuss a case of pycnodysostosis. In addition, a search of articles published in PubMed-Medline was performed. The case was a 13-year-old girl who was referred to a private clinic for dental treatment. Clinical examination showed midface hypoplasia, prominent cheeks, a high nasal bridge, beaked nose, spoon-shaped fingers, frontal bossing, open fontanelles and dental alterations, findings compatible with pycnodysostosis. Patients with this disease also suffer from fractures because of bone hardness with almost no elasticity, a fact that requires special care particularly in the case of children and adolescents. The diagnosis of pycnodysostosis is made based on clinical and radiographic findings. Clinicians should be aware of this disorder to provide adequate dental treatment. Key words:Pycnodysostosis, developmental bone disease, imaging diagnosis.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2017-10-01 | Journal of Clinical and Experimental Dentistry |