Pierre Robin Sequence, from conception to realization

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Pierre Robin Sequence (PRS) comprises a combination of craniofacial defects, including micro/retrognathia, glossoptosis, and respiratory obstruction. Cleft palate is often associated with PRS, however it is not required for diagnosis. The diagnostic criteria for PRS is poorly defined with a large variation in phenotypic presentation. Multiple theories exist regarding the etiology and pathogenesis of PRS, including mechanical, neurological maturation, and rhombencephalic dysneurulation. PRS can be classified into isolated PRS, syndromic PRS, and associated PRS. Defining the classification can aid in determining a treatment plan. Management is largely dependent upon the infant's phenotypic presentation and may require respiratory support with possible surgical intervention to maintain airway patency. Implications for the caregiver focus on identifying the PRS phenotype, obtaining a thorough prenatal history, developing a multidisciplinary approach to care, and ensuring growth and development are supported. Through thorough management positive outcomes are achievable in the infant with PRS.

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