目的:从三级护理中心住院的多中心队列中描述先天性小颌畸形婴儿的疾病谱和护理负担。
方法:从2010年到2020年,查询了儿童医院新生儿数据库中被诊断为小颌畸形的婴儿。人口统计,遗传综合征的存在,和裂隙状态进行了总结。结果包括死亡,住院时间,新生儿手术,出院时的喂养和呼吸支持。
结果:分析包括3236例先天性小颌畸形患儿。1266年发现腭裂(39.1%)。在新生儿住院期间,有256例(7.9%)被诊断出与小颌畸形相关的遗传综合征。住院的中位数(四分位距)为35(16,63)天。住院期间的死亡(n=228,6.8%)与无left裂有关(4.4%,p<0.001)和母亲黑人种族(11.6%,p<0.001)。在新生儿住院期间,1289(39.7%)接受了手术以纠正气道阻塞,1059(32.7%)接受了胃造口管放置。在出院时,1035(40.3%)只口服。与住院时间和出院时存在饲管相关的中心之间存在显着差异(两者p<0.001)。
结论:先天性小颌畸形住院的婴儿具有显著的疾病负担,通常接受手术干预,通常在出院时需要管喂食。我们确定了基于种族和中心之间的差异。制定循证指南可以改善新生儿护理。
OBJECTIVE: To describe the spectrum of disease and burden of care in infants with congenital
micrognathia from a multicenter cohort hospitalized at tertiary care centers.
METHODS: The Children\'s Hospitals Neonatal Database was queried from 2010 through 2020 for infants diagnosed with
micrognathia. Demographics, presence of genetic syndromes, and cleft status were summarized. Outcomes included death, length of hospitalization, neonatal surgery, and feeding and respiratory support at discharge.
RESULTS: Analysis included 3,236 infants with congenital
micrognathia. Cleft palate was identified in 1266 (39.1%). A genetic syndrome associated with
micrognathia was diagnosed during the neonatal hospitalization in 256 (7.9%). Median (IQR) length of hospitalization was 35 (16, 63) days. Death during the hospitalization (n = 228, 6.8%) was associated with absence of cleft palate (4.4%, P < .001) and maternal Black race (11.6%, P < .001). During the neonatal hospitalization, 1289 (39.7%) underwent surgery to correct airway obstruction and 1059 (32.7%) underwent gastrostomy tube placement. At the time of discharge, 1035 (40.3%) were exclusively feeding orally. There was significant variability between centers related to length of stay and presence of a feeding tube at discharge (P < .001 for both).
CONCLUSIONS: Infants hospitalized with congenital
micrognathia have a significant burden of disease, commonly receive surgical intervention, and most often require tube feedings at hospital discharge. We identified disparities based on race and among centers. Development of evidence-based guidelines could improve neonatal care.