Pierre Robin Sequence.

Affiliation

Pediatric Plastic Surgery, Craniofacial Program, Rhode Island Hospital, The Warren Alpert Medical School of Brown University, 2 Dudley Street, Suite 180, Providence, RI 02905, USA. Electronic address: [Email]

Abstract

Pierre Robin sequence consists of clinical triad of micrognathia, glossoptosis, and airway compromise with variable inclusion of cleft palate. Evaluation of airway obstruction includes physical examination, polysomnography for obstruction events, and a combination of nasoendoscopy and bronchoscopy to search for synchronous obstructive lesions. A multidisciplinary approach is required given the high rate of syndromic disease. Management of airway obstruction and feeding starts with nonsurgical maneuvers, such as prone and lateral positioning, nasopharyngeal stenting, and continuous positive airway pressure. Surgical management includes mandibular distraction and tongue-lip adhesion. Subglottic obstruction and central sleep apnea may best be treated with tracheostomy.

Keywords

Cleft palate,Distraction,Glossoptosis,Micrognathia,Pierre Robin sequence,

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