Complete cartilaginous rings – a rare and severe tracheal stenosis in children. minimally invasive and surgical treatment
DOI:
https://doi.org/10.15574/PS.2018.58.66Keywords:
congenital tracheal stenosis, complete cartilaginous rings, pulmonary artery sling, tracheoplasty, resection of trachea, stentingAbstract
Objective – optimization of disease management in children with congenital tracheal stenosis, taking into account risk factors, associated defects and application of minimally invasive and surgical techniques.Material and methods. The study included 9 patients with congenital tracheal stenosis associated with complete cartilaginous rings, aged from 3 weeks to 20 months (on average 7.28±2.07 months) during the period of 2011–2017. The associated defects were found in 7 (77.8%) patients: pulmonary artery sling (n=4), double vascular ring and total abnormal pulmonary venous drainage (TAPVD) (n=1), aberrant right subclavian artery (n=1), ventricular septal defect (VSD) (n=2), right lung agenesis (n=1), small bowel atresia (n=1). Diagnosis of tracheal stenosis was based on CT and tracheobronchoscopy. Surgical treat ment (group I, n=5) included tracheal resection (n=2), slide tracheoplasty (n=2) or double vascular ring division (n=1), associated with pulmonary artery reimplantation (n=4) and VSD closure (n=1). Minimally invasive correction of tracheal stenosis (group II, n=4) consisted of its dilation and stenting, which in one case was performed during the operation of VSD closure in a child with right lung agenesis. Cardiopulmonary bypass was applied in 5 cases.
Results. In the group of minimally invasive correction of tracheal stenosis, 100% of patients survived, in the surgical correction group – 40% (p=0.02). Total mortality associated with correction of tracheal stenosis was 33.3% (n=3). Long-term follow-up showed the restoration of the tracheal lumen in all surviving patients. Complications were observed with the stenting of the trachea, which were as follows: pneumomediastinum (n=1) and granulation in the stent area (n=1).
Conclusions. Minimally invasive treatment options for congenital tracheal stenosis are reasonable in newborns and high-risk children. Resection and tracheoplasty of the thoracic trachea should be performed under cardiopulmonary bypass. Associated intracardiac defects and pulmonary artery sling are subject to simultaneous correction. A unique complex combination of birth defects – a double vascular ring, TAPVD and complete cartilaginous tracheal rings – should be corrected in a more forced pattern, including the correction of intracardiac haemodynamics as well as decompression of the respiratory tract.
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