Department of Paediatric Anaesthesia, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899.
Singapore Med J. 2011 Oct;52(10):726-9.
Congenital tracheal stenosis is a rare but life-threatening obstructive airway disease. It usually presents in early infancy and requires surgical intervention. This study is a review of our experience in the management of congenital tracheal stenosis in children at KK Women's and Children's Hospital, Singapore.
All children who had undergone tracheoplasty for congenital tracheal stenosis between January 1999 and December 2008 were included. The patients' medical records were retrieved from our database, and the demographic data, comorbidities, clinical presentation and management, surgery performed, postoperative complications, final outcomes and follow-up were reviewed.
A total of 11 children aged 12 days to six years underwent surgery for congenital long-segment tracheal stenosis, of which ten (90.9 percent) had associated cardiac and vascular anomalies and nine (81.8 percent) had left pulmonary artery sling predominance. Five (45.4 percent) children had associated bronchopulmonary abnormalities. All the patients underwent slide tracheoplasty with concomitant repair of congenital heart defects. Overall mortality was 45.4 percent (n is 5), including one late mortality due to an unrelated cause. With the exception of one, the follow-up of all patients was complete at this writing. One patient had mild residual tracheal stenosis and another had bilateral bronchomalacia.
This is the largest case series of congenital long-segment tracheal stenosis reported in Southeast Asia to date. Slide tracheoplasty with concomitant repair of cardiac lesions is currently the preferred management for long-segment stenosis. Management of such patients remains a challenge for clinicians and the patients' families, and requires a multidisciplinary approach.
先天性气管狭窄是一种罕见但危及生命的气道阻塞性疾病。它通常在婴儿早期出现，需要手术干预。本研究回顾了新加坡 KK 妇女儿童医院治疗儿童先天性气管狭窄的经验。
纳入 1999 年 1 月至 2008 年 12 月期间接受气管成形术治疗的所有先天性气管狭窄患儿。从我们的数据库中检索患儿的病历，回顾其人口统计学数据、合并症、临床表现和治疗、手术方式、术后并发症、最终结果和随访情况。
共有 11 名 12 天至 6 岁的患儿因先天性长段气管狭窄接受手术治疗，其中 10 例（90.9%）合并心脏和血管异常，9 例（81.8%）合并左肺动脉吊带优势。5 例（45.4%）患儿合并支气管肺异常。所有患儿均行滑动气管成形术，同时修复先天性心脏缺陷。总体死亡率为 45.4%（n=5），包括 1 例因非相关原因导致的晚期死亡。除 1 例外，所有患儿的随访均在本次报告完成。1 例患儿存在轻度残余性气管狭窄，另 1 例患儿存在双侧支气管软化。
这是迄今为止东南亚报道的最大例先天性长段气管狭窄病例系列。滑动气管成形术联合心脏病变修复是目前治疗长段狭窄的首选方法。此类患者的管理仍然是临床医生和患者家属的挑战，需要多学科方法。
