Pierre Robin sequence: An institutional experience in the multidisciplinary management of airway, feeding and serous otitis media challenges

ObjectivesTo evaluate the course and prognosis of airway obstruction, feeding difficulties and hearing abnormalities in patients with Pierre Robin sequence (PRS).MethodsA retrospective review was conducted, of 69 patients with PRS, attending between 1991 and 2010 at the Children's University Hospital in Dublin. Data regarding airway management, nutritional status and hearing difficulties was collected prospectively.ResultsAirway obstruction requiring intervention other than positional therapy was seen in 39% (27) patients. Fifty nine percent (16/27) of these patients, who failed positional therapy, were successfully managed with a nasopharyngeal airway. Following failed intervention with nasopharyngeal airways, two patients had airway maintenance achieved with a successful glossopexy procedure. One patient had an adequate airway achieved with nasal continuous positive airway pressure. Eight patients (12%) required a surgical tracheostomy. Of those who required a tracheostomy, six patients had isolated PRS, one patient had PRS in association with Stickler syndrome and one patient had Nager acro-facial dystosis. Duration of tracheostomy tube ranged from 10 to 19 months, mean 13 months.Seventy percent (48 patients) required supplementary feeding in the form of nasogastric (NG) or gastrostomy tube. Forty-four patients were successfully managed with a temporary NG tube. One patient required a prolonged NG tube, and three required a gastrostomy tube. Twenty-one (30%) patients were successfully managed with a specialised Haberman bottle. Twenty-four patients (35%) who had their airway managed successfully by positional therapy, still required supplemental feeding.Thirty-one patients (45%) demonstrated a conductive hearing loss at some stage, which affected their speech and language development. Twenty-four patients (35%) required tympanostomy tube insertion once, while 7 (10%) of patients required ventilation tube insertion twice or more.ConclusionAirway management in the majority of PRS can be successfully achieved by conservative methods. Even in the presence of an adequate airway, many patients will require supplemental feeding. Early audiological assessment is necessary as many patients will need tympanostomy tube placement to ensure adequate speech and language development.