Congenital tracheobronchial stenosis

详细信息    查看全文

• 作者：Richard J. Hewitt ; BSc ; DOHNS ; FRCS^a ; ^{richard.hewitt@gosh.nhs.uk" class="auth_mail" title="E-mail the corresponding author} ; Colin R. Butler ; BSc ; MRCS^a ; ^b ; ^c ; Elizabeth F. Maughan ; MRCS^b ; ^c ; Martin J. Elliott ; MD ; FRCS ; FRSA^d
• 关键词：Trachea ; Congenital disease ; Long-segment tracheal stenosis ; Slide tracheoplasty ; Transplantation ; Stem cell
• 刊名：Seminars in Pediatric Surgery
• 出版年：2016
• 出版时间：June 2016
• 年：2016
• 卷：25
• 期：3
• 页码：144-149
• 全文大小：595 K

文摘

Congenital tracheobronchial stenosis is a rare disease characterized by complete tracheal rings that can affect variable lengths of the tracheobronchial tree. It causes high levels of morbidity and mortality both due to the stenosis itself and to the high incidence of other associated congenital malformations. Successful management of this complex condition requires a highly individualized approach delivered by an experienced multidisciplinary team, which is best delivered within centralized units with the necessary diverse expertise. In such settings, surgical correction by slide tracheoplasty has become increasingly successful over the past 2 decades such that long-term survival now exceeds 88%, with normalization of quality of life scores for patients with non-syndrome-associated congenital tracheal stenosis. Careful assessment and planning of treatment strategies is of paramount importance for both successful management and the provision of patients and carers with accurate and realistic treatment counseling.