Silver Russell Syndrome: A Case Report and Review
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Abstract
Silver–Russell syndrome (SRS) is a rare congenital genomic imprinting disorder characterized by prenatal and postnatal growth retardation, craniofacial dysmorphism, body asymmetry, and multisystem involvement. Due to marked phenotypic variability and nonspecific clinical findings, diagnosis is often challenging, particularly in the absence of molecular confirmation. Reports focusing on dental and orofacial manifestations of SRS are limited. A 6-year-old boy presented with multiple carious teeth and malocclusion. General examination revealed short stature, slender build, triangular facial appearance with frontal bossing, downturned corners of the mouth, low-set auricles, and clinodactyly of the fifth finger. Intraoral examination showed early mixed dentition, hypodontia, mandibular crowding, and deep palatal vault. Radiographic evaluation demonstrated missing permanent tooth buds, delayed skeletal maturation, clinodactyly, and characteristic craniofacial disproportion. Based on clinical and radiographic findings, a diagnosis of Silver–Russell syndrome was established. Dental management and parent counselling were done. Early recognition of the craniofacial and dental features of SRS by pediatric dentists is crucial for timely diagnosis and multidisciplinary management, which can significantly improve functional outcomes and quality of life.
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