[Special dental management in patients with Williams syndrome].

INTRODUCTION: The Williams syndrome (WS) is a rare genetic disease. The disorder is characterized by symptoms, such as facial dysmorphism, defects within the masticatory organ and cardiovascular system as well as intellectual and physical disability. Moreover, the disorder may also affect vision or hearing, gastrointestinal and urogenital system and skin. Limited cooperation with children suffering from WS is related not only from their young age and fear of dental treatment but also with hypersensitivity to sounds, which is one of the symptoms of this disease. Therefore, there is a need to carry out special dental treatment of such patients. AIM: The aim of the paper is to describe the case of a patient with Williams syndrome. CASE REPORT: The girl showed characteristic findings of the disease such as heart defect and typical facial features (known as " elfin face" with full lips, wide mouth, long and smooth philtrum, prominent forehead and malocclusion). A radiological examination revealed lack of few permanent premolars and incisor. In addition, the patient had many teeth with active caries process and its complications. Therefore, comprehensive dental therapeutic procedures were carried out. At the beginning of the treatment the sensitivity to high-pitched sounds as well as the significant anxiety of the child before dental intervention were the problem. CONCLUSIONS: In individuals with congenital syndromes it is necessary to prepare the patient for dental prophylaxis and therapeutic approach with regard to a variety of physical and intellectual limitations. In addition, there is a need for systematic dental care from their infancy.