Child Dental Patient with WILLIAMS SYNDROME

Clinical cases

Case report

DI, female, age 13

General dg.:     Williams-Beuren syndrome
Dental dg.:     mixed dentition, with retention of deciduous teeth,         microdontia, teeth agenesis  



Characteristic features:

  • “elfin” appearance, thick lips, saddle nose, anteverted nostrils (blue eyes)
  • highly sociable, talkative


Clinical findings:

  • Retention of 6 primary teeth – suspicion of multiple agenesis – X-ray needed
  • Malocclusion
  • Poor hygiene


Session 1:

  • professional cleaning
  • extraction of 52 (topical anaesthesia)


Panoramic Xray (taken after extraction of 52) confirms agenesis (probably 14, 24).
Hypodontia and microdontia permits the upper deciduous canines to remain on the arch, while one upper premolar is missing on  boths sides (14/15?; 24/25?).
Extraction of 62 and 55, 65 are planned in order to make place for the existing premolars to emerge. 53, 63 shall be kept.
After extractions patient did not show for follow-ups.