Abstract：
OBJECTIVE: There is currently no consensus about managing upper airway obstruction (UAO) in infants with Robin sequence (RS), in terms of treatment efficacy or clinical outcomes. This study describes UAO management in UK/Ireland, and explores relationships between patient characteristics, UAO management, and clinical outcomes in the first 2 years of life.
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.
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.
摘要：
目的：目前对于使用Robin序列（RS）治疗婴儿上呼吸道阻塞（UAO）尚无共识，在治疗效果或临床结果方面。这项研究描述了UAO在英国/爱尔兰的管理，并探索患者特征之间的关系，UAO管理，和头2年的临床结果。
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。
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。
