Craniofacial morphology and dental age in children with Silver-Russell syndrome.

OBJECTIVES

This investigation is a part of a multidisciplinary descriptive evaluation of the Silver-Russell syndrome (SRS). The aim of this study was to describe the craniofacial morphology, occlusion and dental age in children with the SRS.

DESIGN

A descriptive literature-controlled study.

SETTING AND SAMPLE POPULATION: Sixteen children diagnosed as having SRS, 10 boys and six girls, aged 4.4-14.5 years, were referred from different parts of Sweden to the Queen Silvia Children's Hospital, Göteborg University.

EXPERIMENTAL VARIABLE: Facial morphology was measured on lateral and postero-anterior radiographs. Occlusion, tooth eruption and palatal height were measured on casts, and dental maturity was evaluated on orthopantomograms.

OUTCOME MEASURE: Linear and angular measurements were obtained from lateral radiographs and the ratios of the linear measurements from the postero-anterior radiographs. The degree of tooth calcification shown on orthopantomograms was taken as a measure of dental maturity. Biometric measurements were taken and the degree of tooth eruption was recorded from the dental casts. The SRS children were compared with reference groups with t-test and z-scores.

RESULTS

Overall, SRS children were found to have smaller linear facial dimensions and deviations in the facial proportions, such as a small retropositioned, and steeply inclined maxilla and mandible, and a proportionally larger anterior facial height in relation to the posterior facial height. In 40% of them a smaller facial height or length on one side (facial asymmetry) was correlated to the smaller side of the body. The frequency of malocclusions was higher, and the palatal height showed a tendency towards an increase. Dental maturity was within normal limits, while the time of tooth eruption was slightly delayed.

CONCLUSIONS

The deviating facial morphology described above is a part of the syndrome, which is characterized by short stature, growth hormone deficiency and asymmetries of the body. The higher percentage of malocclusions in the SRS children might lead to a greater need of orthodontic treatment.