NATIONAL INSTITUTE FOR HEALTH AND CLINICAL EXCELLENCE

Interventional procedure consultation document

Thoracoscopic aortopexy for severe primary tracheomalacia

1 Provisional recommendations

1.1 Current evidence on the safety and efficacy of thoracoscopic aortopexy for severe primary tracheomalacia is limited to small case series and reports. Therefore, clinicians wishing to undertake this procedure should make special arrangements for clinical governance, consent and for audit or research.

1.2 Clinicians wishing to undertake thoracoscopic aortopexy for severe primary tracheomalacia should take the following actions.

• Inform the clinical governance leads in their Trusts.
• Ensure that parents or carers understand the uncertainty about the procedure?s safety and efficacy. They should explain the alternative management options and the likely need for additional treatments. They should provide parents or carers with clear written information. Use of the Institute?s information for patients (‘Understanding NICE guidance?) is recommended (available from www.nice.org.uk/IPGXXXpublicinfo). [[details to be completed at publication]]
• Audit and review clinical outcomes of all patients having thoracoscopic aortopexy for severe primary tracheomalacia (see section 3.1).

1.3 Patient selection and treatment should be carried out by a multidisciplinary team which should include a surgeon experienced in paediatric thoracoscopic procedures.

2  The procedure

2.1 Indications

2.1.1 Primary tracheomalacia is a congenital condition in which the tracheal wall is weak. When the weakness is severe, the trachea can collapse during inspiration, obstructing the normal airflow. It is often associated with congenital abnormalities of the oesophagus. Symptoms may include breathing problems, such as coughing, wheezing, high-pitched breathing (stridor), respiratory tract infections and reflex apnoea/bradycardia (‘dying spells?). In addition there may be feeding difficulties. Mild to moderate symptoms in infants usually improve with age. Treatments for infants with this condition include the use of humidified air, chest physiotherapy and medication to control infection. Supplemental oxygen is sometimes required and Continuous Positive Airway Pressure (CPAP) may be used to treat short-term respiratory distress.

2.1.2 Surgery may be required if conservative management fails or if symptoms are severe (such as in reflex apnoea/bradycardia). Surgical options include open aortopexy, segmental tracheal resection, tracheostomy and endoluminal or extraluminal stenting of the trachea.

2.2 Outline of the procedure

2.2.1 Thoracoscopic aortopexy is performed under general anaesthesia. A telescope and other instruments are inserted into the mediastinum through small incisions on the chest wall and used to visualise the operative field and to carry out the procedure. The left lobe of the thymus is moved aside or removed to expose the aortic arch. Polypropylene sutures are passed through the sternum, into the mediastinum and around the aortic arch. These are tightened so that the aortic arch, and the trachea that lies behind it, are both pulled forward, making the tracheal wall less likely to collapse on inspiration. Adequacy of the procedure can be verified by intraoperative bronchoscopy, which shows an enlarged tracheal lumen.

2.3 Efficacy

2.3.1 In a case series of 6 children, there were two recurrences of life-threatening events after thoracoscopic aortopexy (at 2 and 4 weeks respectively) after which both children underwent a repeat procedure. At a mean follow-up of 27 months, all 6 children were described as ‘doing well? with no further life-threatening events.

2.3.2  A case report stated that 2 children improved ‘dramatically? after the procedure and all stridor disappeared. Oxygen saturation improved from less than 85–90% before surgery to 96–100% postoperatively. Both children gained weight and were well at 17 and 27 months? follow-up.

2.3.3  A second case report of a child treated with thoracoscopic aortopexy stated that a follow-up bronchoscopy 7 months after surgery showed the child had a patent airway with no significant collapse. At 22 months? follow-up the child was well, no longer fatigued easily with activity and had experienced no more apnoea/brachycardia spells.

2.3.4 A third case report of a child treated with thoracoscopic aortopexy reported appropriate growth and no feeding or respiratory difficulties at follow-up (period not stated). For more details, refer to the sources of evidence (see appendix).

2.3.5 The Specialist Advisers listed key efficacy outcomes as improvement of symptoms (cyanotic episodes, stridor and lower respiratory tract infections), the ability to breathe unsupported, the degree of correction as assessed at bronchoscopy and growth (assessed from standard growth charts).

2.4 Safety

2.4.1 The case series of 6 children reported that no adverse events occurred. Two of the three case reports stated that there were no complications associated with the procedure and one stated that there was ‘no notable blood loss?. One case report stated that extubation was delayed because of upper airway oedema (resolved with steroid treatment). For more details, refer to the sources of evidence (see appendix).

2.4.2  The Specialist Advisers listed potential adverse events as life-threatening haemorrhage which cannot be controlled without opening the chest, temporary or permanent injury to one or both phrenic nerves, pneumothorax and mediastinitis.  

2.5 Other comments

2.5.1 The Committee noted that the National Commissioning Group has commissioned Great Ormond Street Hospital to provide a service for children who have complex tracheal abnormalities, with which severe tracheomalacia is often associated.

3 Further information

3.1 This guidance requires that clinicians undertaking the procedure make special arrangements for audit. The Institute has identified relevant audit criteria and is developing an audit tool (which is for use at local discretion), which will be available when the guidance is published.

Bruce Campbell
Chairman, Interventional Procedures Advisory Committee
August 2007
Appendix: Sources of evidence

The following document, which summarises the evidence, was considered by the Interventional Procedures Advisory Committee when making its provisional recommendations.

‘Interventional procedure overview of Thoracoscopic aortopexy for severe primary tracheomalacia, April 2007?

Available from: www.nice.org.uk/ip396overview