Congenital tracheobronchial stenosis.

Abstract:

: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.

journal_name

Semin Pediatr Surg

authors

Hewitt RJ,Butler CR,Maughan EF,Elliott MJ

doi

10.1053/j.sempedsurg.2016.02.007

subject

Has Abstract

pub_date

2016-06-01 00:00:00

pages

144-9

issue

3

eissn

1055-8586

issn

1532-9453

pii

S1055-8586(16)00021-4

journal_volume

25

pub_type

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