Surgery in Patients with Obstructive Airway Disease Due to Intrinsic Tracheal Malformation and/or Vascular Ring: Long-Term Results in 114 Single-Centre Patients

Objectives: Obstruction of airways and occurs in association with vascular ring or intrinsic tracheal malformations. In some patients only reconstruction of trachea/and or bronchi will improve the clinical situation and CPB is needed.

Methods: In this retrospective single center analysis we included all patients presenting in the period between 1995 and 2017 with severe respiratory failure due to airway obstruction/compression caused by 1) intrinsic malformations of the trachea leading to respiratory failure or 2) vascular and/or congenital heart defects associated with respiratory failure. All patients underwent bronchoscopy, imaging was performed by CT or MRI and in selected cases by cardiac catheterization. Follow-up data were obtained from follow-up examinations.

Results: A total of 114 patients, aged 0.8 years [10 days to 49 years] (Median [range]) presenting with vascular ring (78 out of 114), intrinsic tracheal malformations (n = 24/114), pulmonary sling (n = 12/114) were enrolled. 33/114 patients showed additional cardiac malformations including VSD (n = 15/33), Fallot/DORV 4/33, aortic arch hypoplasia (1/33) or complex CHD (13/33).

Surgical technics included (1) decompression of airway by transection of ligaments or vascular remnants (n = 82), (2) partial resection of stenotic segments of the trachea (n = 19), and (3) slide plasty (n = 9). In selected cases external re-expansion of trachea was used (n = 4). CPB was used in 75/114 patients and was a pre-requisite for all patients where tracheal procedures were needed or whenever intracardiac repair was performed.

At follow-up period of 2 [0.1–22] years 104 patients showed clinical improvement or—if ventilator dependent—could be weaned successfully. 3 syndromic patients died 2.5 month to 1 year after the operation from neurological complications. In-hospital mortality (7 patients) was due to multiorgan failure in six syndromic patients and due to mediastinitis after leakage of tracheal anastomosis in one patient.

Conclusion: Relief of vascular compression and/or reconstruction of the trachea and main bronchi can be performed safely in patients with severe airway malformations by using CPB and modern reconstructive technics. However, pre-existing syndromal disease and co-existing multimorbidity must be taken into account in decision making.