Aims To describe the airway and nutritional management strategies used in a regional cohort of infants with Pierre Robin Sequence (PRS), their associated syndromes and anomalies, and their subsequent outcomes regarding growth, development and mortality.
Methods Setting: Regional Cleft-Lip and Palate Network. A tertiary referral Service. Retrospective case note study.
Participants 55 sequential infants with Pierre Robin Sequence (as defined by the triad of micrognathia, Cleft-Palate and Glossoptosis) identified between January 2003 and December 2009; including 4 infants who transferred out of area and 4 infants who died.
Interventions Airway management consisted of Positioning, Nasopharyngeal Airway, Continuous Positive Airway Pressure, Ventilation and Tracheostomy. Nutritional management consisted of oral feeding, nasogastric tube feeding and Percutaneous Endoscopic Gastrostomy.
Main outcomes: Weight and growth percentile as measured at birth and cleft palate surgery. Grommet insertion. Developmental Delay. Mortality.
Results 13 (23.6%) of infants had syndromic PRS (sPRS), 17 (30.9%) had other associated anomalies and 25 (45.4%) had isolated PRS (iPRS) (n = 55). The majority of infants had their airways managed with prone positioning 40 (73%) (n = 55).
The majority of infants 26 (55%) (n = 47) fell to a lower growth percentile by the time of cleft-palate surgery (figure 1).
13 (24%) had documented developmental delay (n = 55). There were 4 deaths (Mortality rate 78 per 1000 PRS births) (n = 55).
Conclusions Infants with sPRS and aPRS appear at greater risk of death than infants with iPRS. Airway management with positioning alone appears to be associated with a higher rate of mortality and failure to thrive in this cohort than other published techniques; its use should be reviewed. However there is inconsistent data reporting in the published literature. Future studies should include a published mortality rate; use standard definitions for iPRS, aPRS and sPRS; document weight gain in terms of growth percentiles and ideally use polysomnography to determine airway obstruction. Given that the practise of positioning is widespread and data inconsistent a national study including the above may be necessary to provide accurate data.