КЛИНИЧЕСКИЙ СЛУЧАЙ СИНДРОМА ЧЕРЕПНО-КЛЮЧИЧНОГО ДИЗОСТОЗА: КЛИНИЧЕСКИЕ И РЕНГЕНОЛОГИЧЕСКИЕ ПРОЯВЛЕНИЯ

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.