Pediatric Airway Unit, Division of Pediatric Surgery, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Madrid, E-28041, Spain.
J Pediatr Surg. 2012 Aug;47(8):1512-5. doi: 10.1016/j.jpedsurg.2012.01.075.
The purpose of this study is to assess the incidence of airway anomalies in children with severe craniofacial syndromes and to establish the role of bronchoscopy in the care of these patients.
Consecutive children with craniofacial syndromes, including both bony deformities of the skull and face, in which a bronchoscopy was performed between 1995 and 2010 were retrospectively reviewed.
Thirty-six patients (22 boys, 14 girls; mean age, 39 months) were studied. Craniofacial synostosis was present in 21 patients (Crouzen syndrome, 11; Apert syndrome, 7, Pfeiffer syndrome, 3) and craniofacial dysostosis in 15 (Goldenhart syndrome, 8; Treacher Collins syndrome, 7). In 30 patients (83.3%), bronchoscopy was performed because of respiratory symptoms (apneic episodes, 22; respiratory distress, 13; stridor, 6; cyanosis, 1) and, in the remaining 6 (asymptomatic children), during guided tracheal intubation before a surgical procedure. Airway anomalies were found in 69.4% of patients (70% in symptomatic patients). Management consisted of tracheotomy in 13 patients, adenoidectomy/tonsillectomy in 13, glossopexy in 5, antireflux surgery in 3, and supraglottoplasty in 2.
Airway anomalies occurred in 70% of children with severe craniofacial syndromes and respiratory symptoms. Bronchoscopy should be performed routinely in this selected group of patients, and the entire airway must be examined. Treatment should be tailored to each individual patient.
本研究旨在评估严重颅面综合征儿童气道异常的发生率，并确定支气管镜在这些患者治疗中的作用。
回顾性分析了 1995 年至 2010 年间连续患有颅面综合征（包括颅骨和面骨的骨畸形）且行支气管镜检查的儿童。
共研究了 36 名患者（22 名男孩，14 名女孩；平均年龄 39 个月）。21 名患者存在颅面骨缝早闭（Crouzen 综合征 11 例，Apert 综合征 7 例，Pfeiffer 综合征 3 例），15 名患者存在颅面骨发育不良（Goldenhar 综合征 8 例，Treacher Collins 综合征 7 例）。30 名患者（83.3%）因呼吸症状（呼吸暂停发作 22 例，呼吸困难 13 例，喘鸣 6 例，发绀 1 例）进行了支气管镜检查，其余 6 名（无症状儿童）则在手术前进行气管插管时进行了支气管镜检查。70%的有症状患者存在气道异常。13 例患者接受气管切开术，13 例患者接受腺样体切除术/扁桃体切除术，5 例患者接受悬雍垂成形术，3 例患者接受抗反流手术，2 例患者接受声门上成形术。
70%有严重颅面综合征和呼吸症状的儿童存在气道异常。支气管镜检查应常规用于该组有选择的患者，必须检查整个气道。治疗应根据每个患者的具体情况制定。
