Management and Outcomes of Neonates with Treacher Collins and Nager Syndromes.

Abstract

To compare management and outcomes of infants with mandibulofacial dysostosis syndromes (Treacher Collins and Nager syndromes) admitted to neonatal intensive care units (NICUs) to infants with other causes of micrognathia. The Children's Hospitals Neonatal Database from 2010 to 2023 was queried for infants with diagnoses of Treacher Collins syndrome (TCS), Nager syndrome (NS), and other infants in NICUs with micrognathia (n = 4210). We identified 103 infants with TCS and 11 with NS to compare with the micrognathia cohort (n = 4210). Compared with infants with micrognathia, those with TCS were more likely to undergo tracheostomy (54% vs 11%) and gastrostomy tube placement (67% vs 35%) and were less likely to undergo mandibular distraction (9.7% vs 28.2%). The hospital mortality rate in TCS was lower than micrognathia cohort (1.9% vs 7.2%). Apgar scores were similar for TCS and micrognathia cohorts (6 and 8 vs 7 and 8, at 1 and 5 minutes, respectively) but lower for NS (2 and 6). Infants with NS had the highest rate of intubation at birth (91%) and tracheostomy placement (72.7%), and a higher mortality rate than TCS (27.3% vs 1.9%). Hospital length of stay was longer in TCS (47.5 days) and NS (43 days) than the micrognathia cohort (37 days). Infants with mandibulofacial dysostosis (TCS and NS) were more likely to have a tracheostomy and gastrostomy tube, and less likely to undergo mandibular distraction than infants with micrognathia from other causes. NS was most severe with highest mortality rate and lowest Apgar scores. Despite a higher rate of tracheostomy and longer length of stay, the mortality rate for TCS remained low.