International Journal of Pediatric Otorhinolatyngology, 22 (1991) 29-37 0 1991 Elsevier Science Publishers B.V. 016.5-5876/91/$03.50 29 PEDOT 00722 Management of airway obstruction in the Pierre Robin sequence Bruce Benjamin and Paul Walker Royal Alexandra Hospital for Children, Camperdown, N.S. W 2050 (Australia i (Received 8 June 1990) (Revised version received 25 January 1991) (Accepted 30 January 1991) Key words: Pierre Robin sequence; Airway obstruction; Tracheotomy Abstract A lo-year retrospective study of the management of airway obstruction in 26 infants with Pierre Robin sequence was made. It was not possible when the infant was first seen to assess the future severity of the airway obstnlction but later 3 distinct groups could be identified according to the airway management which had become necessary. The deaths from respiratory obstruction indicate the prime importance of airway management. Laryngoscopy for intubation or endoscopic evaluation was often difficult and sometimes could not be achieved. ‘Awake intubation’ without general anesthesia has proved to be safer and less difficult using a special purpose slotted laryngoscope. Airway management should be individualized following the progressive sequence of posturing in the prone posi- tion, nasopharyngeal tube, endotracheal intubation and tracheotomy until success- ful control is achieved as indicated by the clinical features and pulse oximetry. Introduction The diagnostic criteria for the Pierre Robin sequence (PRS) are micrognathia, glossoptosis, and incomplete cleft palate [21], although an occasional patient may not have one of these features. PRS presents as an isolated anomaly, as part of a syndrome or as Stickler’s syndrome. Estimates of incidence range from 1 : 2000 to 1 : 50000 births [lo]. Upper airway obstruction in infants with PRS is common and usually consid- ered to be due to mechanical factors [6,11]. The severity is less in the prone Correspondence: B. Benjamin, 231 Macquarie Street, Sydney, N.S.W. 2050, Australia