METHODS: Active surveillance of RS throughout UK/Ireland via the British Paediatric Surveillance Unit and nationally commissioned cleft services. Clinical data were collected at initial notification and 12-month follow-up.
RESULTS: 173 infants with RS were identified, of which 47% had additional congenital anomalies or an underlying syndrome (non-isolated RS). Two-thirds (n = 119) required an airway intervention other than prone positioning: non-surgical in 84% and surgical (tracheostomy) in 16%. Nasopharyngeal airway (NPA) was the most common intervention, used in 83% (n = 99) for median 90 days (IQR 136). Surgical UAO management was associated with prolonged hospital admission, higher prevalence of neurodevelopmental delay (NDD), lower weight-for-age z-scores, and delayed oral feeding. These findings were not attributable to a higher prevalence of non-isolated RS in this group. Although more commonly associated with non-isolated RS, growth faltering was also identified in 48%, and NDD in 18%, of cases of isolated RS.
CONCLUSIONS: In UK/Ireland, most infants with RS are managed with NPA, and tracheostomy is reserved for refractory severe UAO. Clinical outcomes and duration of use indicate that NPA is a safe and feasible first-line approach to UAO. Longitudinal assessment of neurodevelopment and growth is imperative, including in children with isolated RS. Current variations in practice reinforce the need for evidence-based treatment guidelines.
方法:通过英国儿科监测单位和国家委托的唇裂服务对整个英国/爱尔兰的RS进行积极监测。在初次通知和12个月随访时收集临床数据。
结果:确定了173名患有RS的婴儿,其中47%患有其他先天性异常或潜在综合征(非孤立性RS)。三分之二(n=119)需要俯卧位以外的气道干预:84%的非手术和16%的手术(气管造口术)。鼻咽气道(NPA)是最常见的干预措施,用于83%(n=99)的中位90天(IQR136)。手术UAO管理与住院时间延长有关,神经发育迟缓(NDD)患病率较高,较低的体重年龄z分数,和延迟口服喂养。这些发现并非归因于该组中非孤立RS的患病率较高。虽然更常见的与非孤立RS相关,48%的人也发现增长步履蹒跚,NDD占18%,孤立RS的病例。
结论:在英国/爱尔兰,大多数患有RS的婴儿都使用NPA进行管理,气管造口术保留用于难治性重度UAO。临床结果和使用持续时间表明NPA是UAO的安全可行的一线方法。神经发育和生长的纵向评估势在必行,包括孤立RS的儿童。实践中的当前变化加强了对循证治疗指南的需求。