Region Jnkpings ln Odontologiska Institutionen
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Prader-Willi syndrome and oligodontia – presentation of a case treated by autotransplantation

S Kvint, J Norderyd*, B Bergendal
 

Objectives of investigation

Single hypodontia is fairly common among Scandinavian children, 6 – 10%, while multiple aplasia is rare. When a larger number of teeth is missing numeral considerations have to be made when planning oral habilitation. Attention has to be paid to dental and oral conditions as well as to the growing child's psychological and physical development. Treatment planning for a child that, apart from oligodontia, also has a medical or psychiatric diagnose demands even further considerations.

Prader-Willi syndrome is a rare condition (1 in 25 000 births) caused by a chromosomal defect. Some of the characteristics are obesity, muscular hypotonia, mental retardation, short stature, hypogonadism, and small hands and feet.

In this report a multidisciplinary approach in the planning and performance of oral habilitation in a boy with two rare disorders, Prader Willi syndrome and oligodontia, is presented.

Materials and methods

An 8-year-old boy with Prader Willi syndrome and agenesis of all eight permanent premolars was referred to a multidisciplinary group of specialist dentists at the Institute for Postgradutate Dental Education in Jönköping, Sweden, in order to plan oral habilitation. Involved in the strategic decisions were specialists in paediatric dentistry, orthodontics, prosthodontics and oral and maxillofacial surgery.

Results

Autotransplantation of the second permanent molars to the premolar regions was performed in two sessions; at the ages of 10 and 11 years. The surgical treatment was carried out under general anesthesia. The boy is now 14 years old and the transplanted teeth are vital and well functioning.

Conclusions

This case shows a favourable outcome of oral habilitation in a patient with a genetic syndrome and multiple agenesis. The young boy has now a functional occlusion without having received active orthodontic treatment or any prosthetic replacement of missing teeth. Early referrals and a multidisciplinary approach are important and beneficial factors when planning treatment in patients with oligodontia, and even more so in children with a chronic condition or disability.

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