兵庫県立こども病院小児集中治療科Department of Pediatric Critical Care Medicine, Hyogo Prefectural Kobe Children’s Hospital ◇ Hyogo, Japan
先天性気管狭窄症（CTS）は完全気管輪を伴った気管内腔の狭小化により重篤な呼吸障害を来し得る難治性の疾患である．スライド気管形成術の普及によりその治療成績は著しく向上したが，窒息などの急変対応，手術手技や周術期管理，術後フォローアップの難しさから，国内でCTSの管理や治療に取り組んでいる施設は限られている．一方，先天性心疾患（CHD）において呼吸と循環は相互に密接に関連しているため，気道病変の合併は病態をより複雑かつ重症化させ，診療や治療に難渋することが多い．CTSは稀な疾患であるがCHDの合併は比較的多く，小児循環器診療に携わる医療従事者にとってCTSに関する理解を深めることは重要である．本稿ではCTSについて概説し，CHD合併に焦点を当てながら外科的治療を含めた周術期管理の要点をまとめ，施設間ネットワークやチーム医療によるCTS管理の普及と発展を期待する．
Congenital tracheal stenosis (CTS), characterized by the narrowing of the tracheal tract due to complete tracheal rings of the cartilage, is a rare but potentially life-threatening disease that often leads to severe respiratory failure in children. CTS is often found in conjunction with congenital heart diseases (CHD). Although slide tracheoplasty has demonstrated huge improvement in clinical outcomes of treating CTS and has recently emerged as the dominant surgical technique, some hospitals in Japan are still challenged by the amount of work required to manage and treat CTS cases. Generally, CTS in patients with CHD makes the condition more complex and severe due to the sensitive and crucial link between the pulmonary and systemic circulations, such as cardiopulmonary interaction. Therefore, acquiring sufficient knowledge on CTS is highly essential for the attending medical staff in pediatric cardiology. This article reviews and summarizes the clinical presentation and perioperative management of CTS, emphasizing its association with the CHD.
Key words: congenital tracheal stenosis; congenital heart disease; slide tracheoplasty; perioperative management; cardiopulmonary interaction
© 2021 特定非営利活動法人日本小児循環器学会© 2021 Japanese Society of Pediatric Cardiology and Cardiac Surgery
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This page was last modified on 2021-08-03T20:08:36.000+09:00
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