Oral health care in patient with Williams Syndrome

William Syndrome is characterized by multiple anomalies including peculiar facial and oral features. A precocious maxillofacial and dental examination is mandatory in order to follow facial development and tooth eruption. The clinical examination often reveals dental anomalies in the form of amelogenesis imperfecta, morphology (microdontia) and number anomalies (dental agenesis). The risk of Early Childhood Caries is increased by gastroesophageous reflux, a frequent finding in the first year of life of this children. Orthopaedic-orthodontic treatment is often required to address bone growth and to correct the position of the teeth both for function and for aesthetic reasons. The cooperation level and the presence of systemic diseases have to be considered before the treatment planning. A correct psychological approach can ameliorate the compliance even in the patient with mental delay, but a certain degree of collaboration is required for positioning orthodontic devices. A careful analysis of the general health of the patient is necessary before dental therapy: antimicrobial prophylaxis, to prevent infective endocarditis is needed in patient with congenital heart diseases (75%); renal function and blood pressure have to be monitorized before administrating drugs (antibiotics, antiflogistics and sedative). A strict collaboration with the paediatrist and other specialists is needed to plan a safe and successful therapy. Parents must be involved in the planning of dental treatment and correctly informed and motivated about oral hygiene technique, as their collaboration is necessary to maintain oral health of their child after the treatment. In this presentation, the authors report their experience in dental treatment in children with Williams Syndrome describing their clinical approach from a complete anamnesis to the treatment plan, in order to propose guidelines for oral health care in this patients. References: Axelsson S., Bjornland T., Kjaer I., Heidberg A., Storhaug K., Dental characteristics in Williams syndrome: a clinical and radiographic evaluation, Acta Odontol Scand. 2003 Jun;61(3):129-36 Hertzberg J, Nakisbendi L, Needleman HL, Pober B Williams syndrome-oral presentation of 45 cases Pediatr Dent. 1994 Jul-Aug;16(4):262-7 Mass E, Belostoky L., Craniofacial morphology of children with Williams syndrome, Cleft Palate Craniofac J, 1993 May;30(3):343-9. Oncag V., Gumbay S., Parlar A., Williams Syndrome, J Clin Ped Dent, 1995; 19(4): 301- 4