Dental treatment of 2 years old patient with Williams Syndrome with 1 year follow-up period. Case report
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Introduction Williams syndrome (WS) is a multisystemic rare genetic disorder caused by deletion of 26–28 genes in the long arm of chromosome 7. To confirm the clinical diagnosis of Williams syndrome use the technique known as fluorescent in situ hybridisation (FISH). Background A male patient with WS aged 1.8 years was reported to LSMU Kaunas Clinics from a general dentist. Patient was diagnosed with WS through the FISH test. Extra-oral examination revealed typical features specific to WS such as the high forehead, big mouth, large ear buds, short nose, wide nose tip, long filter. Intra-oral examination revealed diastemas, enamel hypoplasia of all teeth, except upper and lower left canines, caries lesions were found in all first primary molars, while the second primary molars were not erupted. The chief complaint was fragility and break down of teeth structure. Case Report(s) Firstly, non-operative dental treatment such as diet counselling, oral hygiene instructions, plaque removal and fluoride varnish (Bifluoride 10, VOCO) application was performed. Later operative treatment (direct Fuji IX GP(GC) restorations and stainless steel crowns (3 M)) based on aesthetic rehabilitation was performed under general anaesthesia. Follow-up Patient did not report any complaints during 1 year follow-up period. Clinic findings showed a good quality of restorations and the second primary molars had erupted without lesions after 1-year follow-up. Conclusions A high prevalence of tooth abnormalities and occlusal disorders in patients with WS requires an early complex dental treatment planning and regular follow-up.