Oral manifestations and rehabilitation of a patient with osteogenesis imperfecta

Abstract

Introduction Osteogenesis imperfecta is a rare heritable connective tissue disorder characterized by increased fragility of the bony tissue. The incidence of orofacial alterations associated with osteogenesis imperfecta is variable and includes dentinogenesis imperfecta, malocclusions, hypoplasia of the jaws, delayed dental development and structural abnormalities of the teeth.

Case outline A 22-year-old female was referred to the Clinic for Pediatric and Preventive Dentistry for dental treatment. Enlarged head, triangular-shaped face, mandibular prognathism with excessive maxillary hypoplasia, lowered vertical occlusal dimension were present features. The intraoral findings included dentinogenesis imperfecta with Kennedy’s class IV in the upper jaw and class II in the lower jaw. Panoramic radiograph revealed abnormalities in the crown and root shape, obliteration of the pulp chamber and severe deficiency of alveolar bone mass. Overall treatment involved five phases: I – preventive and prophylactic treatment, II – direct restauration of five teeth with glass ionomer cement, III – extraction of severely damaged teeth, IV – prosthodontic rehabilitation with removable partial dentures, V – maintenance and follow-up phase.

Conclusion Low prevalence and wide variety of signs and symptoms make dental treatment of osteogenesis imperfecta overly complex and challenging. Nevertheless, it is essential to improve craniofacial and dental function along with facial aesthetic.

Keywords: osteogenesis imperfecta; rare diseases; dentinogenesis imperfecta; partial dentures