Interdisciplinary Management of the Patient with Cleidocranial Dysplasia: A Long-Term Treatment. Part I.

Author(s): Zuzana Weberová, Romana Koberová Ivan?aková.

Cleidocranial dysplasia (CCD) – is an autosomal dominant skeletal dysplasia with a prevalence of 1 in 1 million individuals. This rare disorder is associated with clavicular hypoplasia or aplasia, delayed closure of fontanels, brachycephalic skull, delayed exfoliation of primary teeth, delayed eruption of permanent teeth, multiple supernumerary teeth, and morphologic abnormalities of maxilla and mandible. Only a few successfully treated cases have been reported because of the diagnosis's rarity and the treatment's complexity. In the past, the primary treatment was prosthetic replacement. In the first part, we describe the complex therapy we aligned and included into the dental arch permanent teeth up to the dental age of 12. The second part will focus on the second and third molars and the necessity of orthognathic surgery. The patient was 8 years and 5 months old when first presented in the clinic and has been treated for another 10 years by the interdisciplinary team comprised of the pediatric dentist, the orthodontist, and the maxillofacial surgeon. The report describes the clinical and radiographic findings as clinical, surgical, and orthodontic interventions. Treating such cases requires long-term management, close cooperation of specialists, and proper dental age assessment