Advances in Pierre Robin sequence

Pierre Robin sequence (PRS) is classically described as a triad of micrognathia, glossoptosis, and airway obstruction. About 58% ~ 90% infants of Pierre Robin sequence are associated with a wide U-shaped cleft palate. The pathogenesis of PRS is not clear. PRS is not a syndrome in itself, but rather a sequence of disorders which are related to several other craniofacial anomalies and may appear in conjunction with a syndromic diagnosis. The micrognathia leads to glossoptosis, which in turn results in airway obstruction and inability to feed, and then results in hyoxemia, gastroesophageal reflux and severe malnutrition. In the most severe case it may lead to weight loss and death. Infants with PRS should be evaluated by a multidisciplinary team to assess the anatomic findings, delineate the source of airway obstruction, and address airway and feeding issues. Treatment decisions should focus on the airway obstruction and feeding issues. Positioning will resolve the airway obstruction in 70% of cases. In the correct position, most children will also be able to feed normally. If the infant continues to show evidence of desaturation, then placement of a nasopharyngeal tube is indicated. A proportion of PRS infants do not respond to conservative measures and will require operative treatment. Tongue-lip adhesion and distraction osteogenesis of the mandible and tracheostomy are usually be used. Prior to considering any surgical procedure, the clinician should first rule out any sources of obstruction below the base of the tongue. A polysomnography also be necessary to monitor the sleep apnea. Key words: Pierre Robin sequence; Micrognathia; Airway obstruction