1 Chapter 33 Oral-Facial Aspects of Osteogenesis Imperfecta Copyright 2014 Elsevier Inc. All rights reserved. 2 FIGURE 33.1 Typical shade of gray as seen in the dentition of a 13-year-old patient with OI. Notice the negative anterior overjet indicative of a Class III malocclusion. This fi gure is reproduced in color in the color plate section. 3 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE 33.2 Spontaneous fracture of a permanent molar with DI. Notice the color of the underlying dentin. This figure is reproduced in color in the color plate section. 4 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE 33.3 The primary incisor on the left belongs to a child with OI. The primary incisor on the right belongs to a healthy child. However, not all OI patients have teeth that are smaller than normal. This figure is reproduced in color in the color plate section. 5 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE 33.4 The pan-oral radiograph shows the bulbous crowns of the teeth with calcified canals. The child is 14 years old. The mandibular second molars have just erupted and have large pulpal spaces. Pulpal spaces calcify very quickly once they erupt. The pulpal spaces of the maxillary second and third molars are wide and the molars are impacted. 6 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE 33.5 There is an evident lack of eruption of the posterior teeth. The second primary molars are retained with full roots and the permanent bicuspids below show their twisted roots. 7 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE 33.6 Retained primary teeth and underlying succedaneous teeth in an OI patient who received IV bisphosphonates. 8 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE 33.7 Lateral cephalometric radiograph and tracing of an OI type III patient presenting with severe lateral open-bite and retrusive maxilla. 9 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE 33.8 Adolescent with type I OI with agenesis of the permanent maxillary right canine, but no DI. This fi gure is reproduced in color in the color plate section. (From ref. 48 ) 10 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE 33.9 Same individual as in Figure 33.8 post-orthodontic treatment. This figure is reproduced in color in the color plate section. (From ref. 48 ) 11 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE Young OI type 1 patient presenting with a moderate malocclusion and DI. This figure is reproduced in color in the color plate section. 12 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE Lateral cephalometric radiograph and tracing of a patient with type I OI showing moderately abnormal findings in the anteroposterior plane. Vertical plane measurements are within normal limits. The tendency towards a Class III skeletal malocclusion is present with compensation of the incisor angulations to have a positive anterior overjet. This figure is reproduced in color in the color plate section. 13 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE Intra-oral photo showing retrusion of the maxilla, forwards rotation of the mandible, anterior cross-bite (negative anterior overjet), and DI. This figure is reproduced in color in the color plate section. 14 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE Eight-year-old male patient presenting with the typical facial pattern associated with OI type III. Triangular face, bossing of the forehead, low-set ears and maxillary retrusion. This figure is reproduced in color in the color plate section. 15 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE Adult with type IV OI with mandibular prognathism (Angle Class III malocclusion), and DI. This figure is reproduced in color in the color plate section. (From ref. 48 ) 16 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE Same individual as in Figure post-orthodontic treatment and orthognathic surgery of the maxilla and mandible. This figure is reproduced in color in the color plate section. (From ref. 48 ) 17 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE Mandibular arch of a 13-year-old OI type III female showing an enlarged intermolar distance resulting in a posterior cross-bite. This figure is reproduced in color in the color plate section. 18 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE Craniofacial reconstruction from a cone-beam CT scan of a type III OI patient following orthodontic and prosthetic treatment. This figure is reproduced in color in the color plate section. 19 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE Lateral cephalometric radiograph and tracing of an OI type III patient presenting with severe lateral open-bite and retrusive maxilla. Cephalometric measurements are markedly abnormal horizontally and vertically. This figure is reproduced in color in the color plate section. 20 Copyright 2014 Elsevier Inc. All rights reserved. FIGURE Periapical radiographs of an OI type III patient. After 18 months of treatment and slow orthodontic movement, no evidence of root resorption is evident. This figure is reproduced in color in the color plate section.