Endodontic treatment of patient with cleidocranial dysostosis: a case report

Aim: Cleidocranial dysostosis (CCD) is a rare congenital bone disorder with an autosomal dominant hereditary inheritance pattern. This disorder can be caused by mutation in the transcription factor CBFA1 (RUNX2). The CBFA1 gene is essential for membranous as well as endochondral bone formation. The estimated prevalence of CCD is one per million, but it is most likely underdiagnosed because of the relative lack of medical complications in comparison to other skeletal dysplasias. CCD is reported in all ethnic groups, and there is no sex predilection. Clinically, CCD is characterized by hypoplastic/aplastic clavicles, brachycephalic skull, midface hypoplasia, delayed closure of fontanelles and moderately short stature. The most common dentoalveolar characteristics of CCD are: over-retained deciduous teeth with unresorbed roots, supernumerary teeth, retarded eruption, reduced height of the lower third of the face and a skeletal Class III tendency due to an underdevelopment of the maxilla and to an upward and forward mandibular rotation, seriously delayed root development of the permanent teeth. The multidisciplinary treatment procedure consists of a surgical phase followed by an orthodontic treatment, in which extrusion forces are applied. Methods: A 18-years-old male student presented at Gemelli Hospital in June 2016 because of pain in the upper right back-molar region. Clinical and radiographic examinations revealed an extensive cavity in the upper right first molar and impactation of numerous teeth. In addition, supernumerary teeth were evident at orthopantomography. During treatment, an accidental pulp exposure occurred, so an interim medication with calcium hydroxide was performed. After 1 week, the patient keep on being symptomatic and returned for a recall evaluation, reporting pain and sensitivity. After local anesthesia (3% mepivacaine with noradrenaline 1:100.000) and rubber dam isolation, the pulp chamber was accessed using a spherical diamond bur and further refined with Endo Z bur. The working length was established with a Root ZX apex locator. The root canals were instrumented with NiTi rotary files, using copious irrigation with 2.5 % sodium hypochlorite. The MB2 canal was deeply calcified, so an interim root canal medication was performed with a pellet embedded with 17% EDTA and calcium oxide cement. The next appointment, under 2,5x loop magnification, the MB2 canal was rinsed by copious irrigation with 17% EDTA and instrumented until working length. The root canal was dried with sterilized paper points and then filled with Thermafil gutta-percha cones and AH Plus sealer. After 5 days, coronal restoration was performed with composite resin. Results: Our follow up plan consisted in a clinical visit and a control radiography of the tooth. After 9 months of follow up, the patient reports the complete remission of symptomatology attributable to the tooth treated. Conclusions: As seen, there are many dental implications of this pathology. Therefore, it is very important to approach it with a correct multidisciplinary protocol. Since these patients have a great number of impacted teeth, it is fundamental to preserve their erupted teeth. In fact, keeping permanent teeth as much healthy as possible, enables to choose among different treatment possibilities in order to improve everyday life of our patients. In our case, an erupted tooth can be very important for the orthodontic treatment, since it may help during the orthodontic traction of impacted teeth after surgical exposure. In conclusion, the dental condition of these patients has to be analyzed carefully, because even a single tooth can give us the opportunity to improve oral condition of our patients.