• Complete cartilaginous rings – a rare and severe tracheal stenosis in children. Minimally invasive and surgical treatment
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Complete cartilaginous rings – a rare and severe tracheal stenosis in children. Minimally invasive and surgical treatment

PAEDIATRIC SURGERY.UKRAINE.2018.1(58):66-74; doi 10.15574/PS.2018.58.66

Rudenko E. O.
Bogomolets National Medical University, Kyiv, Ukraine
National Children’s Specialized Hospital «OKHMATDYT», Kyiv, Ukraine

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.
Key words: congenital tracheal stenosis, complete cartilaginous rings, pulmonary artery sling, tracheoplasty, resection of trachea, stenting.

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