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Book

Pierre Robin Syndrome

Diana Baxter  1 , Anthony L. Shanks
In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.
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Book

Pierre Robin Syndrome

Diana Baxter et al.
Free Books & Documents

Excerpt

Pierre Robin sequence (PRS) is characterized by the clinical triad of micrognathia (mandibular hypoplasia), glossoptosis (downward displacement of the tongue), and upper airway obstruction. Clinically, this results in a small, underdeveloped mandible that causes the base of the tongue to fall back into the throat and can ultimately lead to upper airway compromise. It is commonly associated with cleft palate. These findings may be part of a syndrome or isolated. The sequence was first described in 1891. However, Pierre Robin first published a case of an infant with these characteristics in 1923.

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Conflict of interest statement

Disclosure: Diana Baxter declares no relevant financial relationships with ineligible companies.

Disclosure: Anthony Shanks declares no relevant financial relationships with ineligible companies.

References

    1. Hsieh ST, Woo AS. Pierre Robin Sequence. Clin Plast Surg. 2019 Apr;46(2):249-259. - PubMed
    1. Gangopadhyay N, Mendonca DA, Woo AS. Pierre robin sequence. Semin Plast Surg. 2012 May;26(2):76-82. - PMC - PubMed
    1. Izumi K, Konczal LL, Mitchell AL, Jones MC. Underlying genetic diagnosis of Pierre Robin sequence: retrospective chart review at two children's hospitals and a systematic literature review. J Pediatr. 2012 Apr;160(4):645-650.e2. - PubMed
    1. Karempelis P, Hagen M, Morrell N, Roby BB. Associated syndromes in patients with Pierre Robin Sequence. Int J Pediatr Otorhinolaryngol. 2020 Apr;131:109842. - PubMed
    1. Giudice A, Barone S, Belhous K, Morice A, Soupre V, Bennardo F, Boddaert N, Vazquez MP, Abadie V, Picard A. Pierre Robin sequence: A comprehensive narrative review of the literature over time. J Stomatol Oral Maxillofac Surg. 2018 Nov;119(5):419-428. - PubMed

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