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Indicates sessions with recordings available for purchase.The Multidisciplinary Approach in the Treatment of Patients with Cleidocranial Dysplasia to Achieve a Functional Aesthetic Outcome
Cleidocranial dysplasia is a hereditary congenital disorder, where there is delayed ossification of midline structures. The incidence is estimated at 1:200,000. Patients develop often with a short stature, with hypoplastic facial bones and resultant prognathic mandible. They also develop many supernumerary permanent teeth that affect eruption and alignment of the secondary dentition. The successful management of these patients is dependent on a multidisciplinary assessment and comprehensive treatment planning. The timing of intervention is critical to accommodate for alveolar growth and development and eruption of the permanent dentition. Research has shown that variation in the type of mutation of the Runx2 gene in these patients may affect treatment outcome. By adopting a carefully planned multidisciplinary approach in the treatment of these patients, the aim is to achieve an optimal functional and esthetic outcome. The principles of treatment are focused around surgical intervention, orthodontic alignment and prosthodontic rehabilitation governed by the type and severity of dental anomalies present. Early surgical intervention includes removal of supernumerary teeth and the retained deciduous dentition to facilitate alveolar growth and encourage eruption of the permanent dentition. Delayed eruption proves to be problematic in the timed management of cleidocranial dysplasia patients and often requires surgical intervention in combination with orthodontic traction to facilitate eruption of the permanent dentition. In cases where orthodontic traction was unsuccessful, patients were considered for autotransplantation or surgical removal of ectopic teeth. Orthodontic treatment is focused on alignment and eruption through traction and is usually governed by the age of the patient and timing of surgical intervention. The need for prosthetic rehabilitation is case dependent and serves two main purposes: first, to provide an intermediate restorative prosthesis during patient development and, second, to facilitate long-term fixed or removable restorations. This was required in patients presenting with severe displacement of the dentition, resulting in edentulous spaces that could not solely be managed with orthodontics. Treatment involved the use of dental implant-supported prostheses, often coupled with bone augmentation. We present a case series of the management of 10 cleidocranial dysplasia patients who have undergone a multidisciplinary treatment regimen. All the patients have been managed using a combination of the three main multidisciplinary interventions specifically tailored to the severity of each individual case. The aim is to provide patients with a functional dentition, as well as restoring their smile and facial contour. Below is a preoperative radiograph of one of the patients who presented with severe dental anomalies. Cleidocranial dysplasia presents complex challenges for successful dental rehabilitation not only as a result of multiple dental anomalies but also the reduced quantity and density of bone available. A carefully planned multidisciplinary approach with early intervention has shown to be successful in the long-term rehabilitation of these patients. Current research is being conducted by the GSTT craniofacial development team looking into the mRNA pathway for follicular development and eruption in these patients. Outcomes of this may be beneficial for future management of patients with this condition. References 1. Cohen M: Biology of RUNX2 and Cleidocranial Dysplasia. J Craniofac Surg 24: 130-133, 2013. 2. Angle A, Rebellato J: Dental tea management for a patient with cleidocranial dystosis. Am J Orthod Dentofacial Orthop 128(1): 110-117, 2005