Region Jnkpings ln Odontologiska Institutionen

Orthodontic treatment strategies in five patients with Down syndrome, congenital heart defect, and oligodontia

Norderyd J*, Kling Karlberg M


Tooth agenesis is about 10 times more common in individuals with Down syndrome (DS) than in the general population (Russell & Kjaer 1995). About half of the children with DS are born with a congenital heart defect. Oligodontia is a rare condition that can be considered an oral disability.

Materials and methods

Four girls and one boy with Down syndrome and congenital heart defect were missing 6 – 12 permanent teeth, third molars excluded. Oral habilitation was planned with a multidisciplinary team approach. The patients' ability to cope with dental treatment varied greatly. Different behavioural management techniques including conscious sedation with N2O/O2 and general anaesthesia were used.


Two patients underwent only serial extractions of primary teeth. Two patients had serial extractions, surgical removal of impacted teeth, and orthodontic treatment with fixed appliances. One patient had serial extractions, surgical exposure of teeth, and is now undergoing planning for maxillofacial surgery. In three of the patients, primary second molars with no permanent successors were saved.


When planning orthodontic treatment for patients with intellectual and medical disability and oligodontia, a multidisciplinary approach was necessary. The treatment chosen depended mostly on the individual's ability to cope with dental treatment, but alternative orthodontic solutions were also made concerning the risk of inducing endocarditis. Ethical considerations were essential.

Uppdaterad: 2013-04-03
Anna Thofelt, Avdelningen för odontologisk radiologi Jönköping, Folktandvården