Current Management and Outcome of Tracheobronchial Malacia and Stenosis Presenting to the Paediatric Intensive Care Unit

Intensive Care Med 52001) 27: 722±729 DOI 10.1007/s001340000822 NEONATAL AND PEDIATRIC INTENSIVE CARE David P.Inwald Current management and outcome Derek Roebuck Martin J.Elliott of tracheobronchial malacia and stenosis Quen Mok presenting to the paediatric intensive care unit Abstract Objective: To identify fac- but was not related to any other fac- Received: 10 July 2000 Final Revision received: 14 Oktober 2000 tors associated with mortality and tor. Patients with stenosis required a Accepted: 24 October 2000 prolonged ventilatory requirements significantly longer period of venti- Published online: 16 February 2001 in patients admitted to our paediat- latory support 5median length of Springer-Verlag 2001 ric intensive care unit 5PICU) with ventilation 59 days) than patients tracheobronchial malacia and with malacia 539 days). stenosis diagnosed by dynamic con- Conclusions: Length of ventilation Dr Inwald was supported by the Medical Research Council. This work was jointly trast bronchograms. and bronchographic diagnosis did undertaken in Great Ormond Street Hos- Design: Retrospective review. not predict survival. The only factor pital for Children NHSTrust, which re- Setting: Tertiary paediatric intensive found to contribute significantly to ceived a proportion of its funding from the care unit. mortality was the presence of com- NHSExecutive; the views expressed in this Patients: Forty-eight cases admitted plex cardiac and/or syndromic pa- publication are those of the authors and not to our PICU over a 5-year period in thology. However, patients with necessarily those of the NHSexecutive. whom a diagnosis of tracheobron- stenosis required longer ventilatory chial malacia or stenosis was made support than patients with malacia. by dynamic contrast bronchography D.P.Inwald 5)) 51994±1999). Key words Tracheal stenosis ´ Portex Anaesthesia, Intensive Therapy Interventions: Conservative man- Bronchomalacia ´ Tracheomalacia ´ and Respiratory Medicine Unit, Institute of Child Health, agement, tracheostomy and long- Tracheobronchomalacia ´ 30 Guilford Street London WC1N 1EH, term ventilation, surgical correction, Bronchogram ´ Ventilation ´ UK internal or external airway stenting. Mortality E-mail: [email protected] Measurements and results: Record- Phone: +44-20-74059200 ing of clinical details, length of inva- Fax: +44-20-78298634 sive ventilation and appearance at M.J.Elliott contrast bronchography. Five Department of Cardiothoracic Surgery, groups of patients were defined: iso- Great Ormond Street Hospital for lated primary airway pathology Children NHSTrust, Great Ormond Street, 5n = 7), ex-premature infants London WC1N 3JH, UK 5n = 11), vascular rings 5n = 9), complex cardiac and/or syndromic pa- D.Roebuck Department of Diagnostic Imaging, thology 5n = 17) and tracheo- Great Ormond Street Hospital for oesophageal fistulae 5n = 4). The Children NHSTrust, Great Ormond Street, overall mortality was 29%. Median London WC1N 3JH, UK length of invasive ventilation in survivors was 38 days and in patients Q.Mok who died 45. Mortality was highest Paediatric Intensive Care Unit, Great Ormond Street Hospital for in the patients with complex cardiac Children NHSTrust, Great Ormond Street, and/or syndromic pathology London WC1N 3JH, UK 5p = 0.039 Cox regression analysis) 723 Introduction bronchus intermedius and peripheral bronchi). A pressure monitor was connected to the airway circuit via a Y connector and when Tracheobronchomalacia is a condition of dynamic air- collapse was found positive end-expiratory pressure 5PEEP) was applied and opening pressures noted. Airways were defined as way collapse during expiration. In tracheal stenosis, stenosed when the narrowing was fixed and unaffected by PEEP fixed airway narrowing causes airway obstruction in in- up to 25 cmH2O and malacic when the narrowing was dynamic spiration and in expiration. These conditions, first de- and improved with positive airway pressure. Infants with tracheal scribed in 1952 [1], may be primary, when cartilaginous stenosis with distal malacia were classified as tracheal stenosis for rings are congenitally malformed, or secondary, due to the purposes of this analysis since the numbers of patients with degeneration of previously normal cartilage. In neona- both forms of pathology was small 5n = 10). Our bronchograms are not stored as cine-films or videos but as static images, so no at- tal chronic lung disease, development of the tracheo- tempt was made at retrospective blinded severity scoring as this bronchial tree may be abnormal and the cartilage may was felt to be unlikely to yield any useful information. be weak [2]. Extrinsic airway compression by a vascular Data were analysed using SPSS 8.0 for Windows 5SPSS, UK). ring or by enlarged great vessels in the presence of a Analysis of survival and length of invasive ventilation was per- large shunt can result in permanent airway narrowing formed using Cox regression analysis. The Kruskal-Wallis test was [3]. Tracheo-oesophageal fistulae are often associated used to determine whether differences in the length of follow-up in survivors were significant. with malacia of a portion of the trachea at the point of insertion of the fistula. Airway abnormalities may de- velop in recurrent aspiration syndromes and in acquired cartilaginous disorders such as relapsing polychondritis. Results The severity of disease depends on the length, location and degree of narrowing in the affected airway seg- Of the 48 patients, 19 were female. The median age ments. at diagnosis was 137 days 5interquartile range Although primary isolated tracheobronchial malacia 62±213 days). The number of patients in each group rarely requires ventilatory support and is often self-lim- was as follows: isolated primary airway pathology 57), iting, infants with tracheobronchial malacia or stenosis ex-premature infants 511), vascular rings 59), complex with abnormal bronchograms who require ventilatory cardiac and/or syndromic pathology 517) and tracheo- support have been reported to have a high morbidity oesophageal fistulae 54). The overall mortality was and mortality, often related to airway obstruction and 14/48 529 %). Median length of follow-up in survivors respiratory failure [4]. This has not been our experience. was 439 days and was not significantly different between We therefore reviewed children admitted to our inten- any of the groups 5p = 0.635, Kruskal-Wallis test). Out- sive care unit with tracheobronchial malacia and stenos- come data is presented related to diagnosis and to oper- is, diagnosed at contrast bronchography, to determine ative procedure 5Tables 1 and 2). which factors affected outcome. Isolated primary airway pathology Materials and methods Seven patients with primary airway pathology were Following local ethics committee approval, the notes of 48 pa- identified. The median age at diagnosis was 66 days. tients, who had been admitted to our intensive care unit between One term infant was referred for surgical management 1994±1999 and in whom a diagnosis of tracheobronchial malacia of congenital subglottic stenosis, tracheobronchomala- or stenosis had been made at contrast bronchography, were re- cia being diagnosed when weaning difficulties were en- viewed. Information collected included underlying diagnosis, length of invasive ventilation around the time of diagnosis, surgical countered following surgery. Invasive ventilation was interventions, mortality, time between diagnosis and the present defined in this group as the time from first intubation day for survivors and time between diagnosis and death for those for airway obstruction to death or weaning to either who died. Underlying diagnostic categories were defined as isolat- extubation or continuous positive airway pressure ed primary airway pathology or airway pathology associated with 5CPAP) or biphasic positive airway pressure 5BiPAP) neonatal chronic lung disease, complex cardiac and/or syndromic via a tracheostomy. The median length of invasive venti- pathology, vascular rings or tracheo-oesophageal fistulae. Radiographic data were also reviewed. Bronchography was lation of survivors was 60 days. Five patients had isolat- performed as previously described [5]. The patients were lightly se- ed tracheobronchomalacia and two had tracheal stenos- dated, intubated and were breathing spontaneously. The endotra- is with airway malacia distal to the stenotic segment. cheal tube was maintained in a high position with the tip in the sub- Two patients with mild tracheobronchomalacia were ex- glottic region. Opacity was obtained by bolus injections of 0.5±2 ml tubated, one after a cricoid split procedure for congeni- of contrast medium 5Omnipaque, Nycomed, Birmingham, UK), tal subglottic stenosis, the other requiring no interven- injected into the airway via the endotracheal tube through a 3.7 Fr angiography catheter with a single end-hole. Contrast dis- tion. The latter patient was excluded from the statistical persal was obtained by hand ventilation. Up to five affected sites analysis as he had severe combined immune deficiency were identified 5trachea, left main bronchus, right main bronchus, and died from graft versus host disease following bone 724 Table 1 Outcome by primary surgical procedure Procedure No. Diagnosis Further procedures Invasive ventilation Dead Alive Still 5median, days, ventilated survivors only) Aortopexy 2 Tracheomalacia None 38 0 2 0 Division of vascular 1 Tracheal stenosis None 14 0 1 0 ring Slide tracheoplasty 4 Tracheal stenosis Tracheostomy

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