Congenital tracheobronchial stenosis.
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.
Department of Ear, Nose and Throat Surgery, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. Electronic address: richard.hewitt@gosh.nhs.uk.
Richard J
RJ
Hewitt
Department of Ear, Nose and Throat Surgery, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK; Department of Paediatric Surgery, Stem Cell and Regernerative Medicine Group, UCL Institute of Child Health, London, UK; Lungs for Living Research Centre, UCL Respiratory, Division of Medicine, University College London, London, UK.
Colin R
CR
Butler
Department of Paediatric Surgery, Stem Cell and Regernerative Medicine Group, UCL Institute of Child Health, London, UK; Lungs for Living Research Centre, UCL Respiratory, Division of Medicine, University College London, London, UK.
Elizabeth F
EF
Maughan
Department of Cardiothoracic Surgery, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Martin J
MJ
Elliott
D001980:Bronchi / Q000002:abnormalities* / Q000196:embryology / Q000601:surgery; D001982:Bronchial Diseases / Q000175:diagnosis / Q000196:embryology / Q000235:genetics / Q000601:surgery; D001999:Bronchoscopy; D003251:Constriction, Pathologic / Q000175:diagnosis / Q000196:embryology / Q000235:genetics / Q000601:surgery; D004452:Echocardiography; D006801:Humans; D019651:Plastic Surgery Procedures / Q000379:methods; D041623:Tomography, Optical Coherence; D014057:Tomography, X-Ray Computed; D014132:Trachea / Q000601:surgery; D014135:Tracheal Stenosis / Q000175:diagnosis / Q000196:embryology / Q000235:genetics / Q000601:surgery; D016896:Treatment Outcome
Congenital disease; Long-segment tracheal stenosis; Slide tracheoplasty; Stem cell; Trachea; Transplantation