Dental and craniofacial anomalies in Silver-Russell Syndrome

AIM: This was to describe the orofacial findings in the patients affected by Silver-Russell Syndrome and to show the dentist’s role in preventing severe dentofacial and general medical consequences. BACKGROUND: The Silver-Russell Syndrome is a growth disorder with prenatal onset, postnatal growth deficiency, arms, legs, head or truncal asymmetry, short stature, microdontia, craniofacial disproportion with small triangular face and downturned corners of the mouth, and clinodactylous fifth fingers. The Syndrome is genetically heterogenous: maternal uniparental disomy of chromosome 7 has been demonstrated in 70% of cases. The possibility that submicroscopic duplications including GRB10 are a more general causes of Silver-Russell Syndrome is still being investigated. METHODS: Dental assessments were made of a group of children affected by Silver-Russell syndrome and compared with a group of normal children. RESULTS: Mandibular body size and dental age were significantly smaller than in healthy children of the same height: these situations support the possibility of physiological growth hormone deficiency. The treatment with recombinant growth hormone (rhGH) had a significant influence on acceleration on stature gain, but did not have a significant influence on tooth formation and development. CONCLUSION: The focus of approach in Silver-Russell Syndrome should be to reduce negative parent-child interactions and reduce parental anxiety about feeding, growth and weight. Interdisciplinary dental and medical approach is the only able to reach the best long term results.