Williams Syndrome - Review of Clinical Features and It’s Medical Considerations in Dental Treatment

Abstract

Williams syndrome is a congenital disorder that involves multiple system. The condition is characterized by a number of developmental and physical abnormalities. The cause of this disorder is a micro deletion from the long arm of chromosome 7 at 7q11.23. The deleted portion of the chromosome 7q11.23 includes the ELN gene that codes for the structural protein elastin and therefore in the affected individuals arteries may become narrowed. Hypocalcaemia is also found in the affected individuals. Typical facial, oral, and dental features are characteristic of this syndrome. Presence of congenital heart defects and the risk of developing cardiac arrest make dental treatment challenging for such patients. This article narrates the etiology, pathogenesis, and clinical features including oral features of patients with Williams syndrome. A note on role of dental evaluation in diagnosis of Williams syndrome and medical consideration in the dental treatment of patients with Williams syndrome has also been presented.