Unusual dental findings in a girl with Russell–Silversyndrome

Abstract

Introduction: Russell–Silver syndrome (RSS) is a congenital disor-der of unknown aetiology characterised by pre-natal and post-natalgrowth retardation, short stature, limb asymmetry, facial dysmor-phism and oral abnormalities. This case report describes thegeneral features, and in particular the dental findings and theirsignificance, in a Chinese girl with RSS who has been followed upat our dental hospital over the past eighteen years.Clinical management: This patient was referred to our hospital at13 months of age because of the presence of an unrepaired cleftpalate and failure to thrive. Characteristically, her body weight andheight were consistently below the 3rd percentile, with her headcircumference on the 25th percentile. She also exhibited mentalretardation and bilateral hearing loss. Examination revealedfrontal bossing with triangular facies and a cleft of the palate.Furthermore, she presented with down-turned corners of themouth and small maxillary and mandibular dimensions. Followingclosure of the palate, her dental development was reviewed andfound to be delayed in both the primary and permanent dentitions.Severe crowding and tilting of the teeth were subsequently noted inaddition to a double tooth in the primary dentition. Panoramicradiographs have revealed two congenitally missing pre-molars,impacted mandibular first permanent molar, a macrodonticmandibular incisor and some teeth with bulbous crowns andshort, fused roots.Conclusion: Few reports describe unusual dental findings inpatients with RSS. Nevertheless, the presence of dental anomalies,especially short roots along with intellectual impairment can limittreatment options, especially orthodontic therapy.