Jacobs I N, Gray R F, Todd N W
Division of Otolaryngology, Emory University School of Medicine, Atlanta, Ga., USA.
Arch Otolaryngol Head Neck Surg. 1996 Sep;122(9):945-50. doi: 10.1001/archotol.1996.01890210025007.
To analyze the complex nature of upper airway obstruction (UAO) and the response to surgery in children with Down syndrome.
Retrospective medical chart review of all patients with Down syndrome who had UAO during a 5-year period.
Academic tertiary care children's hospital.
Seventy-one pediatric patients with Down syndrome who had significant UAO. Thirty-four patients had pulmonary artery hypertension; 44 patients had multiple sites of airway obstruction. The obstructive problems included lymphoid hyperplasia, macroglossia, narrow nasopharynx, laryngomalacia, congenital subglottic stenosis, tracheobronchomalacia, and tracheal stenosis.
Children with Down syndrome and UAO underwent surgical procedures including tonsillectomy, adenoidectomy, tonsillar pillar plication, uvulopalatopharyngoplasty, anterior tongue reduction, tonguehyoid suspension, laryngotracheoplasty, and tracheotomy.
Postoperative symptoms found on medical chart review, or parental telephone survey, or both, and results of postoperative diagnostic tests. Patients were grouped as "improved" or having "significant residual symptoms."
Twenty-seven of the 55 surgical patients had mild obstructive symptoms, and most improved after tonsil or adenoid surgery, or both. The remaining patients were younger and had more severe symptoms, multiple sites of obstruction, and a high incidence of cardiac disease. Eleven (39%) of the 28 patients in this group had significant residual symptoms after surgery. Four children are tracheotomy-dependent. Five deaths occurred; 3 were attributable to the upper airway.
Upper airway obstruction in children with Down syndrome often is a complex process with multifocal causes. Residual symptoms of airway obstruction are common after surgery. A comprehensive and individualized approach is important in the management of UAO in Down syndrome.
分析唐氏综合征患儿上气道梗阻（UAO）的复杂性质及其对手术的反应。
对5年内患有UAO的所有唐氏综合征患者进行回顾性病历审查。
学术性三级儿童医院。
71例患有严重UAO的唐氏综合征儿科患者。34例患者患有肺动脉高压；44例患者有多个气道梗阻部位。梗阻问题包括淋巴组织增生、巨舌症、鼻咽狭窄、喉软化、先天性声门下狭窄、气管支气管软化和气管狭窄。
患有唐氏综合征和UAO的儿童接受了手术，包括扁桃体切除术、腺样体切除术、扁桃体柱折叠术、悬雍垂腭咽成形术、前部舌体缩小术、舌骨悬吊术、喉气管成形术和气管切开术。
通过病历审查、家长电话调查或两者兼有的方式发现的术后症状，以及术后诊断测试结果。患者被分为“改善”或“有明显残留症状”两组。
55例手术患者中有27例有轻度梗阻症状，大多数患者在扁桃体或腺样体手术或两者兼做后症状改善。其余患者年龄较小，症状更严重，有多个梗阻部位，且心脏病发病率高。该组28例患者中有11例（39%）术后有明显残留症状。4名儿童依赖气管切开术。发生了5例死亡；3例归因于上气道。
唐氏综合征患儿的上气道梗阻通常是一个多病因的复杂过程。术后气道梗阻残留症状很常见。在唐氏综合征患者UAO的管理中，采用全面且个性化的方法很重要。
