ABSTRACT
Objective: To summarize the clinical characteristics of patients with cleidocranial dysplasia (CCD) and analyze their treatment methods. Methods: From January 2000 to December 2020, patients with CCD who completed comprehensive treatment in the Department of Orthodontics and the First Dental Clinic, School and Hospital of Stomatology, China Medical University were retrospectively analyzed. A total of 14 CCD patients [7 males and 7 females, aged (16.1±4.5) years] were collected. There were 153 impacted permanent teeth in this study. In addition to the teeth that needed to be extracted due to special conditions, 147 impacted teeth were pulled into the dentition using closed traction. Patients were divided into adolescent group (≥12 years and<18 years, 10 patients) and adult group (≥18 years, 4 patients). Failure rate of traction was compared between the two groups. Factors affecting the success rate of closed traction such as vertical position of teeth (high, middle and low) and horizontal position of the teeth (palatal, median and buccal) were analyzed. Results: The incidence of maxillary impacted teeth [69.3% (97/140)] was higher than that of mandibular impacted teeth [40% (56/140)]. The difference was statistically significant (χ2=24.22, P<0.001). The supernumerary teeth were mainly located in the premolar area 61.4% (21/44), and most of them were in the palatal region of the permanent teeth 95.5% (42/44). They were generally located at the same height or the occlusal side of the corresponding permanent teeth. The success rate of closed traction was 93.9% (138/147). The success rate in the adolescent group [98.2% (108/110)] was higher than that in the adult group [81.1% (30/37)], and the difference was significant (χ2=14.09, P<0.05). Failure after closed traction of 9 teeth was found totally, including 7 second premolars. The success rate of traction in impacted second premolars at different vertical (χ2=11.44, P<0.05) and horizontal (χ2=9.71, P<0.05) positions in alveolar bone was different significantlly. The success rates of the second premolars were high (15/16), middle (12/13), low (2/7), and lingual palatine (10/17), median (19/19), lip-buccal (0/0), respectively. Conclusions: The closed traction of impacted teeth in patients with CCD was effective, and the age was the main variable affecting the outcome. The success rate of traction in impacted second premolars located in low position vertically or in palatal position was low, which required close observation during treatment.
Subject(s)
Adolescent , Adult , Child , Female , Humans , Male , Young Adult , Bicuspid , Cleidocranial Dysplasia/therapy , Mandible , Retrospective Studies , Tooth, Supernumerary/surgeryABSTRACT
Introducción: La disostosis cleidocraneal es un trastorno genético raro con patrón hereditario. Sus rasgos patognomónicos son la aplasia clavicular, fontanelas y suturas abiertas, múltiples anomalías dentales. Su origen se relaciona con alteraciones en el gen RUNX2, importante para la síntesis de CBFA1, que a su vez funciona como un conformador óseo y un diferenciador de osteoblastos. Caso clínico: Paciente de 11 años con características clínicas de CCD, se corroboran los antecedentes genéticos hereditarios y alteraciones dentales relacionados con disostosis cleidocraneal. Resultado: A tres años de tratamiento se observa mejor definición facial, la erupción de piezas permanentes retenidas y mejor función masticatoria. Conclusión: Mejorar la calidad de vida del paciente con tratamientos interceptivos y el conocimiento de las alteraciones causadas por el síndrome, así como el trabajo interdisciplinario (AU)
Introduction: Cleidocranial dysostosis is a rare genetic disorder with a hereditary pattern. Its pathognomonic features are clavicular aplasia, fontanelles and open sutures, multiple dental anomalies. Its origin is related to alterations in the RUNX2 gene, important for the synthesis of CBFA1, which in turn functions as a bone conformer and an osteoblast differentiator. Clinical case: Patient with eleven years old with clinical characteristics of CCD, hereditary genetic background, and dental alterations related to cleidocranial dysostosis are corroborated. Result: After three years of treatment, the facial definition is better, the eruption of permanent pieces retained and better chewing function. Conclusion: Improve the quality of life of the patient with interceptive treatments and the knowledge of the alterations caused by the syndrome, as well as the interdisciplinary work (AU)
Subject(s)
Humans , Female , Child , Orthodontics, Interceptive , Tooth Abnormalities/therapy , Cleidocranial Dysplasia/therapy , Genetic Diseases, Inborn , Patient Care Team , Quality of Life , Schools, Dental , Tooth Eruption , MexicoABSTRACT
La disostosis cleidocraneal es una enfermedad esqueletal rara que se caracteriza por retardo en la osificación cranel, hipoplaisa o aplasia clavicular y anomalías dentarias. En esta publicación se mostrarán dos casos en que se involucra la genética y las enfermedades del sistema óseo y dentario, corresopndientes a dos miembros de la misma familia. Se detallan la etiología y patogenia, así como los hallazgos clínicos y radiográficos.
Subject(s)
Humans , Male , Female , Adolescent , Middle Aged , Tooth, Impacted/surgery , Tooth, Impacted , Cleidocranial Dysplasia/complications , Cleidocranial Dysplasia/therapy , Cleidocranial Dysplasia/etiology , Cleidocranial Dysplasia/pathologyABSTRACT
Paciente de 24 anos, sexo feminino, portadora de sinais característicos da Displasia Cleidocraniana foi submetida a exames clínico e radiográfico, os quais evidenciaram anormalidades na aparência física e constituição esquelética da mesma, confirmando a suspeita diagnóstica. O tratamento proposto foi excisão cirúrgica de numerosos dentes inclusos, para viabilizar posterior reabilitação protética. A peça cirúrgica foi encaminhada para exame histopatológico, para avaliar a presença de lesão cística e possíveis distúrbios na constituição morfológica dos dentes removidos. Diante da raridade desta síndrome, este artigo objetiva relatar os aspectos gerais da doença e a terapêutica utilizada
Subject(s)
Humans , Female , Craniofacial Abnormalities/etiology , Craniofacial Abnormalities/pathology , Maxillofacial Abnormalities/pathology , Cleidocranial Dysplasia , Cleidocranial Dysplasia/therapy , Bone Diseases/etiology , Bone Diseases/pathology , Dentigerous Cyst/pathology , Dentigerous CystABSTRACT
A 19-year old patient reported with mid face deficiency, un-erupted teeth and functional bite problems, his history revealed that he has another elder brother with the same birth condition. His chief concern was functional class III bite problems as well as poor aesthetics. He was clinically examined thoroughly to diagnose and label his condition. On examination, it was seen that he was able to join his shoulders [hypoplastic clavicles], mid face deficiency and relative mandibular prognathism, while hypertelorism was also noticed. On intra-oral examination, a high maxillary arch with all permanent teeth were found to be fully erupted except 3rd molars, right and left canines, incisors and left 1st premolar. The right central incisor was partially erupted. Left deciduous canine was retained. In the mandibular arch, relatively less permanent teeth were erupted. The teeth found missing were 3rd molars, all premolars except left 2nd premolar, right lateral incisor and both canines. The right 2nd deciduous molar, right and left deciduous canines and right deciduous lateral incisors were found retained. The crowns of all permanent 1st molars were grossly carious but without involvement of the pulp. The oral hygiene was not satisfactory with generalized plaque and calculus. After initial periodontal, conservative and extraction treatment, patient was ready for the dentures. On radiographic assessment [OPG], un-erupted teeth were present along with five supernumerary teeth in the lower arch and one in the upper arch