The Journal of pediatrics · 2025
Management and Outcomes of Neonates with Treacher Collins and Nager Syndromes.
СтатьяСравнительное исследованиеПоддержка NIH: внешняя программаНазвание и аннотация: Перевод в очереди
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Wenger Tara L, Wild K Taylor, Zaniletti Isabella, Zackai Elaine H, Lioy Janet, Resnick Cory M, Chaudhari Bimal P, Rottgers S Alex, Goldstein Jeffrey, Vyas Raj, Ahmad Irfan, Coghill Carl H, Gogcu Semsa, Lai Kuan-Chi, Cielo Christopher M, Padula Michael A, Children's Hospitals Neonatal Consortium Micrognathia Focus Group
Аннотация
OBJECTIVE: 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.
STUDY DESIGN: 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).
RESULTS: 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).
CONCLUSIONS: 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.
STUDY DESIGN: 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).
RESULTS: 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).
CONCLUSIONS: 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.