Late presentation of upper airway obstruction in Pierre Robin sequence

A retrospective review was carried out of 11 consecutive patients with the Pierre Robin sequence referred to a tertiary paediatric referral centre ove r a five year period from 1993 to 1998. Ten patients were diagnosed with si gnificant upper airway obstruction; seven of these presented late at betwee n 24 and 51 days of age. Failure to thrive occured in six of these seven in fants at the time of presentation, and was a strong indicator of the severi ty of upper airway obstruction. Growth normalised on treatment of the upper airway obstruction with nasopharyngeal tube placement. All children had be en reviewed by either an experienced general paediatrician or a neonatologi st in the first week of life, suggesting that clinical signs alone are insu fficent to alert the physician to the degree of upper airway obstruction or that obstruction developed gradually after discharge home. The use of poly somnography greatly improved the diagnostic accuracy in assesssing the seve rity of upper airway obstruction and monitoring the response to treatment. This report highlights the prevalence of late presentation of upper airway obstruction in the Pierre Robin sequence and emphasises the need for close prospective respiratory monitoring in this condition. Objective measures su ch as polysomnography should be used, as clinical signs alone may be an ina dequate guide to the degree of upper airway obstruction.