Fetal Care Center at Connecticut Children's and the Division of Pediatric General, Thoracic, and Fetal Surgery, Department of Surgery, University of Connecticut School of Medicine, Hartford, Connecticut, USA.
Fetal Care Center of Dallas and Medical City Children's Hospital, Dallas, Texas, USA.
Fetal Diagn Ther. 2024;51(4):411-418. doi: 10.1159/000537820. Epub 2024 Mar 27.
Unilateral congenital high airway obstruction syndrome (CHAOS) is caused by a complete obstruction of a mainstem bronchus with resulting hyperinflation and accelerated growth of one lung, severe mediastinal shift, and hydrops. Spontaneous perforation of the atresia has been observed in CHAOS which allows hydrops to resolve but hyperinflation, mediastinal shift and a critical airway obstruction persists as the perforation is usually pinhole-sized.
We present a case of unilateral CHAOS presenting at 26 2/7 weeks with observed-to-expected total lung volume (O/E TLV) of 203% with spontaneous perforation occurring at 28 weeks with resolution of hydrops but persistence of hyperinflation and mediastinal shift with an O/E TLV of 60.5% on 34 5/7 weeks' magnetic resonance imaging (MRI), successfully managed in a 35 5/7 weeks, 1,670 gm, growth restricted baby, by venoarterial extracorporeal membrane oxygenation (VA ECMO) and resection of the tracheobronchial atresia and tracheobronchoplasty on day of life 5. The baby was separated from ECMO on post-op day 12, required tracheostomy for positive end expiratory pressure for tracheomalacia at 4 months.
At 2 years of age, she has met all developmental milestones, has been weaned to room air tracheostomy collar, and has been anticipating tracheal decannulation. There is persistent bronchiectasis in the hyperinflated right lung but no malacia. This is the first reported survivor of mainstem bronchial atresia suggesting the importance of preservation of the hyperplastic lung and airway reconstruction to normal long-term outcome.
单侧先天性高位气道阻塞综合征（CHAOS）是由主支气管完全阻塞引起的，导致单侧肺过度充气和加速生长、严重纵隔移位和水肿。在 CHAOS 中已经观察到闭锁的自发性穿孔，这使得水肿得以缓解，但由于穿孔通常只有针孔大小，因此仍然存在过度充气、纵隔移位和严重气道阻塞。
我们报告了一例单侧 CHAOS 病例，该病例在 26 周 2 天出现，观察到的预期总肺容量（O/E TLV）为 203%，在 28 周时出现自发性穿孔，水肿缓解，但仍存在过度充气和纵隔移位，O/E TLV 为 60.5%，在 34 周 5 天的磁共振成像（MRI）上，在一个 35 周 5 天、体重 1670 克、生长受限的婴儿中成功地通过静脉动脉体外膜肺氧合（VA ECMO）和切除气管支气管闭锁和气管支气管成形术进行管理。婴儿在出生后第 5 天从 ECMO 中分离出来，在第 4 个月因气管软化症需要使用正压呼气末气道进行气管造口术。
在 2 岁时，她已经达到了所有的发育里程碑，已经从 ECMO 脱机，使用气管造口套管呼吸空气，并且已经在预期进行气管拔管。过度充气的右肺仍然存在支气管扩张，但没有软化。这是首例主支气管闭锁存活的报告，提示保留过度增生的肺和气道重建对正常长期结局的重要性。
