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 girl 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 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.
Familial dentinogenesis imperfecta, blue sclerae, and wormian bones without fractures: another type of osteogenesis imperfecta?
