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KMID : 1144020090050020092
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Journal of Korean Association for Disability and Oral Health
2009 Volume.5 No. 2 p.92 ~ p.95
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PIERRE ROBIN SEQUENCE: A CASE REPORT AND REVIEW OF THE LITERATURE
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Lee Jung-Eun
Song Je-Seon
Kim Seong-Oh
Lee Jae-Ho
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Abstract
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In 1923, Pierre Robin, a French stomatologist, first reported the association of micrognathia with glossoptosis. As more than 80% of all Pierre Robin cases accompanied with other syndrome such as Stickler syndrome, velocardiofacial syndrome, fetal alcohol syndrome, Treacher Collins syndrome, etc, it is called Pierre Robin sequence(PRS). PRS is described in the literature as a triad of anomalies characterized by micrognathia, glossoptosis and cleft palate. Clinically, the triad consists of airway obstruction and feeding difficulty, which are more frequent and severe in the neonatal period. In this case, a 14-month old boy with PRS was referred to the department of pediatric dentistry, Yonsei university dental hospital, who resolved airway obstruction by lateral positioning and feeding problem by percutaneous endocutaneous gastrotomy insertion. PRS is a developmental disorder as well as an anatomic obstructive disorder, therefore it should be dealt with by a multidisciplinary team.
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KEYWORD
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Pierre Robin sequence, Airway obstruction, Lateral positioning
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