Advantages of cone beam computed tomography (CBCT) in the orthodontic treatment planning of cleidocranial dysplasia patients: a case report

Domenico Dalessandri,Ingrid Tonni,Corrado Paganelli,Francesca Zotti,Maria Grazia Piancino,Pietro Bracco,Laura Laffranchi

doi:10.1186/1746-160x-7-6

Abstract

Our aim was to discuss, by presenting a case, the possibilities connected to the use of a CBCT exam in the dental evaluation of patients with Cleidocranial Dysplasia (CCD), an autosomal dominant skeletal dysplasia with delayed exfoliation of deciduous and eruption of permanent teeth and multiple supernumeraries, often impacted. We think that CBCT in this patient was adequate to accurately evaluate impacted teeth position and anatomy, resulting thus useful both in the diagnostic process and in the treatment planning, with an important reduction in the radiation dose absorbed by the patient.

Highlights

Cleidocranial dysplasia (CCD), known as cleidocranial dysostosis or Marie-Sainton syndrome, is a disorder that affects most prominently those bones derived from endochondral and intramembranous ossification and it’s characterized by defective development of the cranial bones and by the complete or partial absence of the clavicles
All other cases were studied with traditional dental radiology using the tube shift method, that taking two conventional radiographs permit to locate the impacted tooth location comparing the movement of this tooth respectively to the way in which the radiograph was taken
In Cleidocranial Dysplasia (CCD) patients the use of reconstructed 3D images obtained by a CBCT exam for diagnosis and treatment planning has only scarcely been documented until now, so no evidence-based conclusion can be made based on the current literature

Summary

Introduction

Cleidocranial dysplasia (CCD), known as cleidocranial dysostosis or Marie-Sainton syndrome, is a disorder that affects most prominently those bones derived from endochondral and intramembranous ossification and it’s characterized by defective development of the cranial bones and by the complete or partial absence of the clavicles. Diagnosis is based on clinical and radiographic findings, that include imaging of the cranium, thorax, pelvis and hands These patients presents a delayed ossification of the skull fontanels and a premature closing of the coronal suture that leads to a frontal, parietal and occipital bossing of the skull; a short stature, occasionally accompanied by a spinal scoliosis; a wide and flat nasal bridge due to hypertelorism; different anomalies of pubis and hipbone, with flat feet and knock knees; a brachycephaly with an high arched palate and sometimes cleft palate; a prolonged retention of deciduous teeth and several impacted permanent successors and supernumerary elements, sometimes accompanied. These supernumerary, associated with a diminished alveolar bone resorption, lead to the impaction or the ectopic location of the permanent teeth [6,7]

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Discussion

Conclusion