Multidisciplinary prosthetic rehabilitation of an adult patient with cleidocranial dysplasia by using a rapid external distraction device: A clinical report.

The multidisciplinary treatment of a 41-year-old man with cleidocranial dysplasia is described. A rapid external distraction device was used to reposition the maxilla before the prosthodontic rehabilitation.

Conservative orthodontic and multidisciplinary approaches for patients with cleidocranial dysplasia in late adolescence or young adulthood.

This case series describes conservative orthodontic and multidisciplinary approaches for treating two patients diagnosed with cleidocranial dysplasia in late adolescence and young adulthood. Most of the impacted permanent teeth erupted spontaneously within 3 to 4 years after surgical extraction of the deciduous and supernumerary teeth. The remaining unerupted permanent teeth were facilitated with traction or extracted followed by implantation or restoration. Repositioning of the maxilla and mandible via orthognathic surgery was also applied to correct skeletal and occlusal discrepancies and lead to satisfying results.

Nuss procedure for pectus excavatum in a patient with cleidocranial dysplasia.

Cleidocranial dysplasia is an autosomal skeletal disorder resulting from delayed or abnormal ossification of bony growth. Pectus excavatum independently presented in a 9-year-old boy with cleidocranial dysplasia and was corrected using the Nuss procedure. There were no perioperative complications, and the post-operative course was uneventful. Although there were concerns regarding extraordinary late consolidation or remodeling of the bony thorax, placement of a Nuss plate for 5 years and 6 months improved the patient's concave deformity without re-depression.

Orthognathic Surgery in Cleidocranial Dysplasia.

ABSTRACT: Optimal surgical correction of the craniofacial manifestations of cleidocranial dysplasia (CCD) has not been established due to the rarity of the condition. A 27-year-old female with CCD is presented. She underwent virtual surgical planning (VSP) followed by LeFort-I disimpaction, bone grafting, bilateral sagittal-split osteotomy, genioplasty, submental lipectomy, and targeted facial fat grafting. The patient necessitated 15-mm of vertical maxillary disimpaction centrally, stabilized with wide maxillary plates and interpositional allogenic fibula grafts. Six-month postoperative examination demonstrated improved appearance and functional symptoms. Skeletal relationships were normalized on computed tomography (CT) and there was minimal change between immediate and 6-month postoperative CT measurements, demonstrating a stable result. Orthognathic surgery used to establish dentofacial harmony in patients with CCD can test the extremes of single-stage facial skeletal expansion. Use of VSP, wide maxillary plates, and interpositional bone grafts can help optimize maxillary expansion and stability, while concurrent fat redistribution optimizes facial aesthetics.

Combined Orthodontic-Surgical Sequential Treatment of Cleidocranial Dysplasia: A Case Report With 7-Year Follow-up and Review of the Literature.

Cleidocranial dysplasia (CCD) is a rare hereditary disorder characterized by skeletal malformations and dental abnormalities. Mutations of the transcription factor RUNX2 are responsible for the pathogenesis of CCD. We present a case of a 10-year-old boy with CCD, presenting with hypoplastic clavicles, delayed closure of the fontanelles, retarded exfoliation of the deciduous teeth, retarded eruption of the permanent teeth, and multiple impacted supernumerary teeth. Based on the clinical and radiographic examination results showing abnormalities of the bones and teeth, a diagnosis was reached easily, but it was difficult to achieve a complete curative effect. We carried out a highly organized schedule of treatment, including extraction of the deciduous and supernumerary teeth, partial resection of alveolar bone, distraction of impacted teeth, and orthodontic surgery. After 7-year follow-up, the patient has achieved acceptable occlusion and midfacial appearance. The main objectives of this study were to present the diagnosis and treatment of CCD and to emphasize the benefits of combined orthodontic-surgical sequential treatment.

The Treatment Strategy of Cleidocranial Dysplasia: Combined Orthodontic and Orthognathic Treatment.

Cleidocranial dysplasia is a rare autosomal-dominant condition that affects ossification. The main symptoms of this cleidocranial dysplasia patient include craniofacial abnormalities, hypoplasia of clavicles, narrow thorax, retarded exfoliation of deciduous teeth, retarded eruption of permanent teeth, and multiple impacted supernumerary teeth. The aim of this treatment was to correct the craniofacial abnormalities and provide an adequate functional reconstruction of the occlusion. The patient was treated with a multidisciplinary therapeutic protocol including removal of supernumerary teeth, surgical exposure of impacted teeth, orthodontic, and orthognathic surgery. After 6 years' follow-up, the treatment objectives of acceptable occlusion, normal function, and acceptable profile were achieved.

Combined surgical-orthodontic treatment of patients with cleidocranial dysplasia: case report and review of the literature.

OBJECTIVES: To study the present treatment situation and investigate a better orthodontic approach for patients with cleidocranial dysplasia (CCD) through systematically reviewing the published cases and to conclude the surgical-orthodontic treatment experience of cleidocranial dysplasia. METHODS: A comprehensive search for studies published through to April 10, 2018 was conducted using the Pubmed, Web of Science, and Embase databases. The CCD cases treated with the approach combining surgical exposure and orthodontic treatment were concluded. RESULTS: Eight papers and 9 finished cases were included to be compared with the present case. The age of cases ranged from 9 to 28 years. Clearing the way of eruption path in early age can facilitate the spontaneous eruption of impacted teeth. For adults, combined surgical-orthodontic treatment can achieve a nearly complete dentition and stable occlusal contact, but it is time consuming and needs surgical assistance. The combination of orthognathic surgery can reduce the difficulty of orthodontic treatment and treatment duration, as well as achieve a better facial profile. CONCLUSION: Surgical exposure combined with orthodontic traction is an effective treatment for patient with CCD. Patient's age, demand, economic circumstances, and status of permanent dentition should be considered when making treatment plan.

Clinical and radiological findings in a severe case of cleidocranial dysplasia.

Cleidocranial dysplasia (CCD) is a rare congenital autosomal dominant condition, causing hypoplasia of the clavicle, abnormal formation of teeth, skeletal and craniofacial bones. CCD is caused by the mutation of RUNX2/CBFA1 present in the short arm of chromosome 6 at position 21.1, a transcription factor essential for the formation of teeth, cartilage and bone. Patients with CCD show the classical features of excessive mobility of the shoulder bone, lack of resorption of the deciduous teeth, failure to erupt permanent teeth, multiple impacted and supernumerary teeth, and open fontanelle and sutures of the skull. In this article we report a case of CCD in a 16-year-old male patient, with an aim to highlight the clinical, radiological and recommended treatment guidelines.

Therapeutic Protocol for Orthosurgical Management of Class III Malocclusion in Patients With Cleidocranial Dysostosis.

Cleidocranial dysostosis (CCD) is a congenital skeletal disorder with significant manifestations in facial and dental development. Patients are affected with CCD present maxillary deficiency, late dental eruption, and supernumerary teeth. Early and multidisciplinary approach is necessary to treat CCD patients, especially to manage dental eruption and Class III malocclusion with maxillary deficiency. Several orthodontic and surgical interventions are performed to enable traction and extraction of teeth. Yet the maxillary deficiency may be protracted followed by orthodontic dental compensation. On the other hand, it is important to note that CCD patients' treatment is closely related to the severity of transversal and sagittal deformities, as well as the discrepancies in the lower third of the face. In this context, patients with facial impairment highly affected by CCD may need ortho-surgical decompensation to reach more aesthetic outcomes. The present study reports a case of a 14-year-old young patient affected by CCD. Clinically, the patient presented Class III malocclusion, maxillary deficiency, short lower facial third, posterior crossbite, and anterior open bite leading to facial disharmony. The patient underwent treatment in 2 stages: the interceptive approach aimed to transversally expand the maxilla and promote its protraction; and the corrective phase combined with the orthognathic surgery treated the patients' main complains; the anterior open bite, unerupted teeth, and chin prominence. The treatment approach applied in the clinical report allowed the correction of the malocclusion and facial profile satisfying completely the patient's expectations.

Surgical Management and Evaluation of the Craniofacial Growth and Morphology in Cleidocranial Dysplasia.

Cleidocranial dysplasia (CCD, MIM 119600) is a rare autosomal dominant disorder affecting bone, cartilage, craniofacial growth, and tooth formation leading to supernumerary teeth. Few reports delineate the genotype-phenotype correlations related to the variations in craniofacial morphology and patterning of the dentition and the complexity of treating patient's malocclusion. Successful management of the craniofacial deformities in patients with CCD requires a multidisciplinary team of healthcare specialists. Approximately 70% of patients are due to point mutations in RUNX2 and <20% due to copy number variations with the remainder unidentified. There is no literature to date, describing the orthognathic management of CCD patients with deletion in one of the RUNX2 alleles. The purpose of this study was to evaluate the craniofacial morphology and dental patterning in a 14-year-old Caucasian female with CCD resulting from a novel microdeletion of RUNX2 in 1 allele. The CCD patient with RUNX2 haploinsufficiency due to microdeletion had decreased craniofacial bone and ankyloses in the permanent dentition. An altered extraction protocol of supernumerary teeth was followed in this patient. Craniofacial growth and morphologic analysis demonstrated atypical skull shape, persistent metopic suture, and decreased mandibular size.

Cleidocranial dysplasia: diagnosis, surgical and orthodontic planning and interventions in a pediatric patient / Displasia cleidocraneal: diagnóstico, planificación quirúrgica y ortodóncica e intervenciones en un paciente pediátrico

Cleidocranial Dysplasia (CCD) is an autosomal dominant bone disorder caused by a defect in the CBFA1 gene and characterized by skeletal, craniofacial and orodental anomalies. This paper describes the main aspects of a case of CCD from diagnosis and planning to the first step of the interventions. An 11-year-old male patient sought the pediatric dentistry clinic of the Federal University of Paraná (Brazil) with the complaint of the prolonged retention of nearly all his primary teeth. Clinical and imaging exams led to the diagnosis of CCD and the treatment plan was outlined. The first step consisted of the extraction of 4 primary, 2 permanent and 2 supernumerary teeth from the maxilla, followed by palate separation, traction of the impacted teeth and reverse traction of the maxilla. The patient remains in treatment. Clinical follow up as well as the awareness and motivation of the family are important factors in such cases.

La displasia cleidocraneal (CCD) es un trastorno óseo, autosómico dominante, causado por un defecto en el gen CBFA1 y se caracteriza por anomalías esqueléticas, craneofaciales y bucodentales. En este trabajo se describen los principales aspectos de un caso de CCD, desde el diagnóstico y la planificación para la primera etapa de las intervenciones. Un paciente varón de 11 años de edad, concurrió a la Clínica de Odontología Pediátrica de la Universidad Federal de Paraná (Brasil) con un problema de retención prolongada de casi todos sus dientes de leche. Se describen los exámenes clínicos y de imagen dirigidos al diagnóstico de la CCD y el plan de tratamiento. La primera etapa consistió en la extracción de cuatro dientes primarios, dos dientes permanentes y dos dientes supernumerarios del maxilar, seguido de separación del paladar, tracción de los dientes afectados y tracción inversa del maxilar. El paciente permanece en tratamiento. El seguimiento clínico, así como la concientización y motivación de la familia son factores importantes en este tipo de casos.

Histological examination and clinical evaluation of the jawbone of an adult patient with cleidocranial dysplasia: a case report.

OBJECTIVES: Cleidocranial dysplasia (CCD) is a rare congenital malformation syndrome, inherited autosomal-dominantly. During a course of treatment including surgical, implantological and restorative procedures, an opportunity arose to histologically examine biopsies of the maxilla and mandible of a CCD patient 47 years of age. CASE REPORT: The aim of this case report is to present the results of the histological evaluation of the alveolar bone and the surgical pretreatment for and placement of six implants each in the maxilla and the mandible. The implants were inserted in a minimally invasive procedure using 3D template guidance. Following uneventful healing of the implants, ceramically veneered bridges were cemented on individual titanium abutments. Since the patient had not received orthodontic treatment in childhood-which would have been the treatment modality of choice-this implantological and prosthodontic approach was necessary. Biopsies were taken from the maxilla and the mandible before placing the implants. Histological evaluation showed bone with strong, coarsely interconnecting trabeculae, especially in the maxilla. Both the bone and the gingiva otherwise exhibited a normal structure without pathological features or anomalies. CONCLUSION: The clinical parameters and histological evaluation of this one clinical case suggest that the concepts familiar from general oral implantology in terms of surgical and prosthetic procedures can be adopted for older patients with CCD.

Orthognathic surgery in patients with cleidocranial dysplasia.

The aim of this study was to report the orthodontic-surgical approach of a 21-year-old female patient diagnosed with cleidocranial dysplasia. An orthognathic surgery was performed in the maxilla and mandible during the same procedure to correct an existing dentofacial deformity (class III malocclusion). In addition, malar prostheses were used to correct midface deficiency. After surgical intervention, orthodontic treatment continued in order to promote stability, function, and aesthetics. Cases of cleidocranial dysplasia treated with the defined criteria can bring aesthetic and functional benefits to the patient.

Early dental treatments for patients with cleidocranial dysplasia.

OBJECTIVE: To explore the early dental interventional strategies for adolescent patients and a child patient with cleidocranial dysplasia (CCD). METHODS: Surgical exposure using the apically repositioned flap technique combined with orthodontic traction was used in the adolescent patients whose ideal treatment time for initiating treatment was missed. For the child patient whose ideal treatment time for initiating treatment was not missed, the simple surgical exposure method was carried out in order to promote the eruption of the impacted incisors. RESULTS: All the impacted maxillary incisors of the three CCD patients were successfully positioned into a proper alignment either through the two stages of crown exposure and the elastic traction or simple surgical exposure. CONCLUSION: Crown exposure surgery combined with light force orthodontic traction provides an effective approach to treat the typical dental abnormalities of adolescent CCD patients. Simple surgical exposure was also an effective way for a child CCD patient for whom the most ideal time for initiation of treatment was not missed.

Restorative treatment strategies for patients with cleidocranial dysplasia.

OBJECTIVE: To develop a suitable treatment strategy for patients with cleidocranial dysplasia (CCD) who miss the optimal early treatment stage. MATERIALS AND METHODS: This study enrolled 15 patients with CCD who had all missed the optimal treatment stage and were diagnosed with CCD through clinical examinations and genetic tests. Based on the chief complaints and requirements of the patients, three different therapeutic schedules were devised for these patients. Schedules I (periodontal and endodontic treatments) and II (periodontal, endodontic and prosthodontic treatments) were used for patients with low requirements, whereas Schedule III (multidisciplinary strategy, including periodontal, endodontic, surgical, orthodontic and prosthodontic treatments) was used for patients with high requirements. RESULTS: Schedules I, II and III were used in five, seven and three patients, respectively. Schedule III treatments produced the best outcomes in terms of occlusion and esthetics. CONCLUSIONS: Schedule III based on a comprehensive multidisciplinary therapy is an ideal restorative therapeutic strategy and can achieve good outcomes for patients with CCD who missed the optimal treatment stage.

A novel anchorage technique for transnasal traction in rigid external maxillary distraction.

We describe an effective technique for anchorage of transnasal traction in the management of maxillary rotation during external distraction.

Differing therapeutic approaches to cleidocranial dysplasia (CCD).

Cleidocranial dysplasia (CCD) (MIM 119600) is a genetic skeletal disorder characterised by skeletal alterations at numerous bone segments (cranium, clavicles, pelvis) and a typical hyperdontia. It is a hereditary disease of the dominant autosomal type with wide variability of expression. The constant presence of numerous supernumerary teeth poses two orders of problems. On one hand, if these supplementary teeth are impacted, they may constitute a mechanical obstacle to the eruption of normal teeth. If on the contrary they have erupted, they cause aesthetic and functional damage. Surgical therapy is indispensable to restore a correct architecture to the alveolar-dental arches, and it is recommended during childhood to avoid or at least reduce complications in adulthood. Two cases of cleidocranial dysplasia are here reported. The adult patient (Case 1), reached our observation with a poor oral situation, manifested relapsing odontogenic abscesses at several impacted supernumerary teeth. The surgical treatment was complex and invasive, requiring percutaneous access in order to remove a tooth embedded in the mandible and the extraction of almost all residual teeth. This outcome was avoided in the second case, through combined surgical-orthodontic treatment that entailed extraction of the supernumerary teeth and subsequent orthodontic treatment, with restoration of a normal occlusion. In conclusion, management of patients suffering from very rare pathologies should be carefully evaluated in relation to clinical characteristics and possible local and systemic complications. The aim of this study is to illustrate two cases managed in a paradigmatically opposite way. It is paramount for the surgeon to intercept cases of hyperodontia typical of CCD at an early phase. It is possible, through correct surgical-orthodontic treatment, not only to avoid local and general complications, but also to achieve proper occlusion.

Correction of depressed forehead with BoneSource in cleidocranial dysplasia.

Cleidocranial dysplasia is a well-documented rare congenital disorder of the bone characterized by abnormalities of the skull, clavicle, and dentition. Despite numerous observations, there are still comparatively few reports regarding patients with cleidocranial dysplasia that focus on the impact of reconstruction of these defects on facial aesthetics. We report a 19-year-old woman with opened metopic and sagittal sutures and delayed closure of the anterior fontanelle. Through bicoronal incision and pericranial elevation, the defect was fully exposed. BoneSource was used to fill the cranial defect and was contoured to the desired cosmetic outcome. During a 24-month follow-up period, no absorption or recurrence was seen, and the patient was satisfied.