CLINICAL CASE OF CRANIOCLAVICAL DYSOSTOSIS SYNDROME: CLINICAL AND X-RAY MANIFESTATIONS

S. Chuykin, N. Davletshin, O. Chuykin, N. Makusheva, K. Kuchuk, A. Bilak
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Abstract

Objectives. Clinical case in the practice of an oral and maxillofacial surgeon: a child with cranioclavicular dysostosis. This cider was first described in 1898 by P. Marie and P. Sainton. H. Scheuthauer described the pathological manifestations of skeletal anomalies in more detail. Steithauer–Marie–Sainton syndrome, or cranioclavicular dysostosis, involves aplasia or hypoplasia of the clavicles, delayed dentition, supernumerary teeth, and other skeletal abnormalities. In this syndrome, skeletal abnormalities include clavicular aplasia, or hypoplasia, bell-shaped chest, enlarged cranial vault with a convex frontal bone, open fontanelle, brachydactyly, pelvic hypoplasia, short stature, severe abnormal development of teeth with changes in occlusion, that is, this is a fairly severe hereditary pathology . Retention and dystopia of permanent teeth are associated with the presence of one or more supernumerary teeth on the upper and lower jaws. Purpose. Conducting an anthropometric, dental, and x-ray examination of a child with cranioclavicular dysostosis. Methodology. The article presents photographs of the child, pediatric and dental examination data, plain radiography of the chest and collarbones, orthopantomography of the upper and lower jaw, and a treatment plan for cranioclavicular dysostosis. Currently, due to a problem with teething, she consulted a dentist; after an examination, the child was referred to a maxillofacial surgeon. Conclusions. Cranioclavical dysostosis syndrome is a complex congenital pathology, the complex rehabilitation of which should involve not only doctors of all dental specialties, but also maxillofacial surgeons, orthopedists, pediatricians, neurologists, speech therapists, and medical psychologists.
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颅锁骨发育不良综合征临床病例:临床和X光表现
目标。口腔颌面外科医生的临床病例:一名颅锁关节发育不良的儿童。1898 年,P. Marie 和 P. Sainton 首次描述了这种畸形。H. Scheuthauer 更详细地描述了骨骼异常的病理表现。Steithauer-Marie-Sainton综合征,或称颅锁关节发育不良,包括锁骨发育不全或发育不良、牙齿发育迟缓、超常牙齿和其他骨骼异常。在这种综合征中,骨骼畸形包括锁骨增生或发育不良、钟形胸、颅穹增大且额骨外凸、囟门开放、腕骨发育不良、骨盆发育不良、身材矮小、牙齿发育严重异常且咬合发生变化,也就是说,这是一种相当严重的遗传性病症。恒牙滞留和阻生与上下颌存在一颗或多颗超常牙齿有关。 研究目的对一名患有颅锁关节发育不良的儿童进行人体测量、牙科和 X 光检查。 方法。文章介绍了患儿的照片、儿科和牙科检查数据、胸部和锁骨的X光平片、上颚和下颚的X光正位片以及颅锁关节发育不良的治疗方案。目前,由于长牙问题,她咨询了牙医;经过检查后,孩子被转诊到颌面外科医生处。 结论颅锁关节发育不良综合征是一种复杂的先天性疾病,其复杂的康复治疗不仅需要所有牙科专业的医生参与,还需要颌面外科医生、矫形外科医生、儿科医生、神经科医生、语言治疗师和医学心理学家的参与。
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