title: Clinical aspects, imaging features, and considerations on bisphosphonate-related osteonecrosis risk in a pediatric patient with osteogenesis imperfecta.
abstract: Osteogenesis imperfecta (OI) is a rare hereditary condition caused by changes in collagen metabolism. It is classified into four types according to clinical, genetic, and radiological criteria. Clinically, bone fragility, short stature, blue sclerae, and locomotion difficulties may be observed in this disease. OI is often associated to severe dental problems, such as dentinogenesis imperfecta (DI) and malocclusions. Radiographically, affected teeth may have crowns with bulbous appearance, accentuated constriction in the cementoenamel junction, narrowed roots, large root canals due to defective dentin formation, and taurodontism (enlarged pulp chambers). There is no definitive cure, but bisphosphonate therapy is reported to improve bone quality; however, there is a potential risk of bisphosphonate-related osteonecrosis of the jaw. In this study we report a case of OI in a male pediatric patient with no family history of OI who was receiving ongoing treatment with intravenous perfusion of bisphosphonate and who required dental surgery. In addition, we discussed the clinical and imaging findings and briefly reviewed the literature.
authors.affiliation: Division of Oral Radiology, Department of Clinical Dentistry, Federal University of Ceará, Rua Alexandre Baraúna 949, 60430-160 Fortaleza, CE, Brazil.
authors.forename: Fábio Wildson Gurgel
authors.initials: FW
authors.lastname: Costa
authors.affiliation: Division of Stomatology and Oral Radiology, Department of Clinical Dentistry, Federal University of Ceará, Rua Alexandre Baraúna 949, 60430-160 Fortaleza, CE, Brazil.
authors.forename: Filipe Nobre
authors.identifier: 0000-0001-6345-8156
authors.initials: FN
authors.lastname: Chaves
authors.affiliation: Division of Stomatology, Department of Clinical Dentistry, Federal University of Ceará, Rua Alexandre Baraúna 949, 60430-160 Fortaleza, CE, Brazil.
authors.forename: Alexandre Simões
authors.initials: AS
authors.lastname: Nogueira
authors.affiliation: Division of Oral and Maxillofacial Surgery, Department of Oral and Maxillofacial Surgery, Walter Cantídio University Hospital, Federal University of Ceará, Rua Alexandre Baraúna 949, 60430-160 Fortaleza, CE, Brazil.
authors.forename: Francisco Samuel
authors.initials: FS
authors.lastname: Rodrigues Carvalho
authors.affiliation: Division of Oral Pathology and Stomatology, Department of Clinical Dentistry, Federal University of Ceará, Rua Alexandre Baraúna 949, 60430-160 Fortaleza, CE, Brazil.
authors.forename: Karuza Maria Alves
authors.initials: KM
authors.lastname: Pereira
authors.affiliation: Division of Oral Radiology, Department of Clinical Dentistry, Federal University of Ceará, Rua Alexandre Baraúna 949, 60430-160 Fortaleza, CE, Brazil.
authors.forename: Lúcio Mitsuo
authors.initials: LM
authors.lastname: Kurita
authors.affiliation: Division of Oral and Maxillofacial Surgery, Department of Oral and Maxillofacial Surgery, Federal University of Rio Grande do Norte, Campus Universitário Lagoa Nova, 59078-900 Natal, RN, Brazil.
authors.forename: Rodrigo Rodrigues
authors.initials: RR
authors.lastname: Rodrigues
authors.affiliation: Division of Pediatric Dentistry, Department of Clinical Dentistry, Federal University of Ceará, Rua Alexandre Baraúna 949, 60430-160 Fortaleza, CE, Brazil.
authors.forename: Cristiane Sá Roriz
authors.initials: CS
authors.lastname: Fonteles