title: The management of congenital tracheal stenosis.
abstract: This paper reviews current concepts and results in the management of congenital tracheal stenosis (CTS). Diagnostic options are considered and the requirements for successful management defined. Chief amongst these is a multi-disciplinary approach with individualised patient management. Severe long-segment CTS represents the biggest challenge to clinicians and the worst problems for affected families. Near-death episodes are frequent in affected infants and some cannot be ventilated and require ECMO. Associated cardiovascular anomalies are frequent. Patients require immediate resuscitation and transfer to a specialist unit. After careful assessment, accurate diagnosis and discussion, primary resection and end-to-end repair with a slide technique should always be the first option, with concomitant repair of associated cardiac anomalies. If this is impossible because of the severity of the lesion, some form of patch tracheoplasty will be indicated. Cardiopulmonary bypass is often required. Patches include pericardium, autograft trachea, carotid artery, cartilage, and allograft trachea. Mortality ranges from 0 to 30% in the literature, which largely comprises single-centre long-term experience. Recurrence is common and can be managed by stenting and tracheal homograft implantation. Long-term quality of life of survivors is little reported but seems good. Physiological data are lacking. To improve results, we suggest a treatment algorithm to rationalise care.
authors.affiliation: The Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, UK. elliom1@gosh.nhs.uk
authors.forename: Martin
authors.initials: M
authors.lastname: Elliott
authors.forename: Derek
authors.initials: D
authors.lastname: Roebuck
authors.forename: Clair
authors.initials: C
authors.lastname: Noctor
authors.forename: Clare
authors.initials: C
authors.lastname: McLaren
authors.forename: Ben
authors.initials: B
authors.lastname: Hartley
authors.forename: Quen
authors.initials: Q
authors.lastname: Mok
authors.forename: Catherine
authors.initials: C
authors.lastname: Dunne
authors.forename: Nick
authors.initials: N
authors.lastname: Pigott
authors.forename: Chirag
authors.initials: C
authors.lastname: Patel
authors.forename: Alpesh
authors.initials: A
authors.lastname: Patel
authors.forename: Colin
authors.initials: C
authors.lastname: Wallis
mesh_terms: D000402:Airway Obstruction / Q000209:etiology / Q000517:prevention & control; D002315:Cardiopulmonary Bypass / Q000379:methods; D006801:Humans; D013517:Otorhinolaryngologic Surgical Procedures / Q000379:methods; D010348:Patient Care Team; D011183:Postoperative Complications / Q000517:prevention & control; D012307:Risk Factors; D055502:Secondary Prevention; D014135:Tracheal Stenosis / Q000150:complications / Q000151:congenital* / Q000601:surgery*