Multiplanar imaging in a familial case of cleidocranial dysplasia and various orthodontic-surgical protocols approved for its treatment

S. Buch, R. Castelino, S. Babu, M. Baba
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Abstract

Introduction: Cleidocranial dysplasia (CCD) is a rare autosomal-dominant disorder that results in defective ossification. The gene responsible for pathogenesis has been mapped to the short arm of chromosome 6p21 core-binding factor alpha-1 or runt-related transcription factor-2. The condition being a complex congenital disorder with numerous skeletal and dental anomalies, its management depends considerably on the patient's ambitions, age, economic status and permanent dentition status, endodontic and periodontal health. Case Report: A 17-year-old female reported with complaint of misaligned teeth and desired orthodontic treatment. The patient had been diagnosed with CCD during childhood but, seemingly, with no previous management. Family wise patient's mother presented with features of CCD but no previous health records. A familial case report of CCD is presented with a brief explanation of various orthodontic-surgical methods carried out for its treatment Conclusion: CCD is a rare disorder, with no universally acknowledged treatment plan; hence, it is appropriate to involve a multidisciplinary team for its management.
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家族性锁骨颅骨发育不良病例的多平面成像和各种批准的正畸手术治疗方案
引言:裂颅发育不良(CCD)是一种罕见的常染色体显性遗传疾病,可导致骨化缺陷。负责发病机制的基因已被定位到染色体6p21核心结合因子α-1或runt相关转录因子-2的短臂上。这种情况是一种复杂的先天性疾病,有许多骨骼和牙齿异常,其治疗在很大程度上取决于患者的雄心、年龄、经济状况和恒牙列状况、牙髓和牙周健康。病例报告:一名17岁的女性报告了牙齿错位和需要正畸治疗的投诉。该患者在儿童时期被诊断为CCD,但似乎之前没有任何治疗。家庭型患者的母亲表现出CCD的特征,但之前没有健康记录。本文报告了CCD的家族性病例,并简要介绍了各种正畸手术治疗方法。结论:CCD是一种罕见的疾病,没有公认的治疗方案;因此,让一个多学科团队参与其管理是合适的。
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