Silver-Russell syndrome: phenotype features and oral health status.

Background: Silver-Russell Syndrome is a rare malformation syndrome with a variable clinical and genetic presentation. Its incidence is estimated at 1:70.000-1:100.000 births. Since the diagnosis of Silver-Russell syndrome is based primarily on the identification of clinical features, studies assessing the craniofacial/dental changes present in this group of patients are important. The aim of the study was to evaluate phenotype features and oral health status in patients with Silver-Russell syndrome.

Results: In the extraoral examination, patients with SRS were found to have a triangular facial shape, facial asymmetry, low-set, protruding ears, narrow lips and downward-facing mouth angles. In intraoral examination, reduced tongue dimensions, cleft palate and gothic palate were observed. There were no statistically significant differences in Plaque Index values between the groups. Gingival Index values were significantly higher in the Silver-Russell syndrome. The prevalence of caries was also higher in the group of subjects with Silver-Russell syndrome.

Conclusions: Patients with Silver-Russell syndrome present themselves with features that affect oral health. Prompt orthodontic and dental intervention in children with SRS can help normalize oral function and facial appearance.