Dental manifestations of rare skeletal disorders: Diagnosis and treatment for the oral health care provider.

BACKGROUND

It is essential that health care providers be familiar with the etiology, manifestation, and management of rare skeletal diseases to ensure positive patient outcomes. Dentists play a critical role in the multidisciplinary care of these patients. This review highlights several rare skeletal disorders that profoundly affect bone mineralization, craniofacial growth, and tooth development.

TYPES OF STUDIES REVIEWED: The authors review scoping review articles, position papers, case reports, and treatment protocols describing effects of rare skeletal disorders that affect skeletal and dental hard tissues. The relationships between the diagnoses and implications for various aspects of dental treatment are highlighted.

RESULTS

Examples of the skeletal disorders highlighted include cleidocranial dysplasia, which is characterized in part by hypoplastic or absent clavicles, delayed exfoliation, and eruption of teeth. Osteogenesis imperfecta associated with dentinogenesis imperfecta has intrinsic discoloration of the teeth, which are more prone to fracture. Osteoporosis secondary to chronic systemic disease can affect oral health care due to skeletal fragility and therapy-related alterations in bone metabolism, features that overlap with other rare skeletal disorders. Patients with X-linked hypophosphatemia exhibit persistent osteomalacia and dental hard-tissue changes. Hypophosphatasia has a heterogeneous manifestation, with effects on the skeleton and dental hard tissues.

CONCLUSIONS AND PRACTICAL IMPLICATIONS: New therapies and management of rare skeletal disorders affect the craniofacial complex and oral health care needs of patients. Clinicians should consider the etiology and skeletal and dental manifestations when addressing the treatment needs of these patients. Working with an interdisciplinary and intradisciplinary approach to care must be considered to create the best treatment outcomes.