Contemporary Clinical Dentistry (Jan 2015)

Dental management of patient with Williams Syndrome - A case report

  • Daniel Wong,
  • Srinivas Sulugodu Ramachandra,
  • Ashish Kumar Singh

DOI
https://doi.org/10.4103/0976-237X.161908
Journal volume & issue
Vol. 6, no. 3
pp. 418 – 420

Abstract

Read online

Williams syndrome is a multisystemic rare genetic disorder caused by deletion of 26-28 genes in the long arm of chromosome 7. It is characterized by developmental and physical abnormalities including congenital cardiovascular abnormalities, mental retardation, neurological features, growth deficiency, genitourinary manifestations, gastrointestinal problems, musculoskeletal problems, unique behavioral characteristics, and dental problems. Dental abnormalities include malocclusion, hypodontia, malformed teeth, taurodontism, pulp stones, increased space between teeth, enamel hypoplasia, and high prevalence of dental caries. Authors report a 17-year-old female patient with underlying Williams syndrome. Oral features and problems seen in the patient are listed. Malocclusion and screwdriver shaped teeth were noticed. Generalized widening of the periodontal ligament space with vital teeth was seen. This finding has not been reported in cases of Williams syndrome earlier. Precautions taken during dental treatment in patients with Williams syndrome are also discussed.

Keywords