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

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.

1. Krivchenya DU, Lazorishinets VV, Yemets IN et al. 2012. Double vascular ring: pulmonary sling and double aortic arch associated with total anomalous pulmonary venous drainage and complete tracheal rings. Neonatology, Surgery and Perinatal Medicine. II, 1(3):120-125

2. Patent 56491, Ukraine, IPC A61B17/00 Method of formation of vascular anastomosis DU Krivchenya, VV Lazorishinets, EO Rudenko, Ya P Truba, VG Karpenko, RV Zhezhera; owner Bogomolets National Medical University, Amosov National Institute of Cardiovascular Surgery; No 201011621; application 30.09.2010; publication 10.01.2011, Bulletin 1

3. Anton-Pacheco JL, Cano I, Garcia A et al. 2003. Patterns of management of congenital tracheal stenosis. J Pediatr Surg 38(10):1452-1458. https://doi.org/10.1016/S0022-3468(03)00495-0

4. Antón-Pacheco JL, Morante R. 2017. Operative or non-operative treatment of congenital tracheal stenosis: is there something new?. Journal of thoracic disease. 9(12): 4878-4880. https://doi.org/10.21037/jtd.2017.11.75; PMid:29312681 PMCid:PMC5757067

5. Backer CL, Mavroudis C, Dunham ME et al. 1998. Repair of congenital tracheal stenosis with a free tracheal autograft. The Journal of thoracic and cardiovascular surgery. 115(4): 869-874. https://doi.org/10.1016/S0022-5223(98)70368-X

6. Backer CL, Holinger LD. 2010. A History of Pediatric Tracheal Surgery. World Journal for Pediatric and Congenital Heart Surgery. 1(3): 344-363. https://doi.org/10.1177/2150135110381602; PMid:23804890

7. Backer CL, Kelle AM, Mavroudis C et al. 2009. Tracheal reconstruction in children with unilateral lung agenesis or severe hypoplasia. The Annals of thoracic surgery. 88(2): 624-631. https://doi.org/10.1016/j.athoracsur.2009.04.111; PMid:19632424

8. Backer CL, Mavroudis C, Holinger LD. 2002. Repair of congenital tracheal stenosis. In Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual. 5(1): 173-186. WB Saundershttps://doi.org/10.1053/pcsu.2002.29718; PMid:11994878

9. Brown SB, Hedlund GL, Glasier CM et al. 1987. Tracheobronchial stenosis in infants: successful balloon dilation therapy. Radiology. 164: 475-478. https://doi.org/10.1148/radiology.164.2.3602388; PMid:3602388

10. Cheng W, Manson DE, Forte V et al. 2006. The role of conservative management in congenital tracheal stenosis: an evidence-based long-term follow-up study. Journal of pediatric surgery. 41(7): 1203-1207. https://doi.org/10.1016/j.jpedsurg.2006.03.046; PMid:16818049

11. Chiu PP, Kim PC. 2006. Prognostic factors in the surgical treatment of congenital tracheal stenosis: a multicenter analysis of the literature. Journal of pediatric surgery. 41(1): 221-225. https://doi.org/10.1016/j.jpedsurg.2005.10.043; PMid:16410137

12. Elliott M, Roebuck D, Noctor C et al. 2003. The management of congenital tracheal stenosis. International journal of pediatric otorhinolaryngology. 67: S183-S192. https://doi.org/10.1016/j.ijporl.2003.08.023

13. Fiore AC, Brown JW, Weber TR et al. 2005. Surgical treatment of pulmonary artery sling and tracheal stenosis. Ann Thorac Surg. 79: 38-46. https://doi.org/10.1016/j.athoracsur.2004.06.005; PMid:15620911

14. Forsen Jr JW, Lusk RP, Huddleston CB. 2002. Costal cartilage tracheoplasty for congenital long-segment tracheal stenosis. Archives of Otolaryngology–Head & Neck Surgery. 128(10): 1165-1171. https://doi.org/10.1001/archotol.128.10.1165; PMid:12365888

15. Grillo HC, Wright CD, Vlahakes GJ et al. 2002. Management of congenital tracheal stenosis by means of slide tracheoplasty or resection and reconstruction, with long-term follow-up of growth after slide tracheoplasty. The Journal of thoracic and cardiovascular surgery. 123(1): 145-152. https://doi.org/10.1053/jtc.2002.v123.p0145; https://doi.org/10.1067/mtc.2002.119068; PMid:11782768

16. Herrera P, Caldarone C, Forte V et al. 2007. The current state of congenital tracheal stenosis. Pediatr. Surg. Int. 23: 1033-1044. https://doi.org/10.1007/s00383-007-1945-3; PMid:17712567

17. Hoffer M, Tom L, Wetmore R et al. 1994. Congenital tracheal stenosis. The otolaryngologist’s perspective. Arch Otolaryngol Head Neck Surg. 120: 449-453. https://doi.org/10.1001/archotol.1994.01880280077015; PMid:8166979

18. Hong X, Zhou G, Liu Y et al. 2015. Management of pulmonary artery sling with tracheal stenosis: LPA re-implantation without tracheoplasty. Int J Clin Exp Med. 8: 2741-2747. PMid:25932228 PMCid:PMC4402875

19. Hraška V, Photiadis J, Haun C et al. 2009. Pulmonary artery sling with tracheal stenosis. Multimedia Manual of Cardio-Thoracic Surgery. 9(0123): mmcts.2008.003343. https://doi.org/10.1510/mmcts.2008.003343; PMid:24412825.

20. Laberge JM, Puligandla P. 2008. Congenital malformations of the lungs and airways. In: Pediatric Respiratory Medicine (Taussig LM, Landau LI eds) Philadelphia: Mosby: 907-942. https://doi.org/10.1016/B978-032304048-8.50068-2

21. Maeda K, Ono S, Tazuke Yu et al. 2013. Long-term outcomes of congenital tracheal stenosis treated by metallic airway stenting. J Ped Surg. 48(2): 293-296. https://doi.org/10.1016/j.jpedsurg.2012.11.002; PMid:23414854

22. Maeda K, Yasufuku M, Yamamoto T. 2001. A new approach to the treatment of congenital tracheal stenosis: balloon tracheoplasty and expandable metallic stenting. Journal of pediatric surgery. 36(11): 1646-1649. https://doi.org/10.1053/jpsu.2001.27940; PMid:11685692

23. Manning PB, Rutter MJ, Border WL. 2008. Slide tracheoplasty in infants and children: risk factors for prolonged postoperative ventilator support. Ann Thorac Surg. 85: 1187-1192. https://doi.org/10.1016/j.athoracsur.2007.11.019; PMid:18355493

24. Monnier P. 2011. Congenital Tracheal Anomalies. Pediatric Airway Surgery.  Berlin Heidelberg Springer-Verlag: 157-179. https://doi.org/10.1007/978-3-642-13535-4

25. Munzon GB, Martinez-Ferro M. 2012. Pediatric tracheal stenosis and vascular rings. Toraks Cerrahisi Bülteni. 3: 206. https://doi.org/10.5152/tcb.2012.30; https://doi.org/10.5152/tcb.2012.31

26. Oshima Y, Yamaguchi M, Yoshimura N et al. 2008. Management of pulmonary artery sling associated with tracheal stenosis. Ann Thorac Surg. 86: 1334-1338. https://doi.org/10.1016/j.athoracsur.2008.04.020; PMid:18805188

27. Rutter MJ. 2006. Evaluation and management of upper airway disorders in children. In Seminars in pediatric surgery. 15(2): 116-123. WB Saunders. https://doi.org/10.1053/j.sempedsurg.2006.02.009; PMid:16616315

28. Speggiorin S, Torre M, Roebuck DJ et al. 2012. A new morphologic classification of congenital tracheobronchial stenosis. The Annals of thoracic surgery. 93(3): 958-961. https://doi.org/10.1016/j.athoracsur.2011.12.019; PMid:22364985

29. Weber TR, Connors RH, Tracy TF. 1991. Congenital tracheal stenosis with unilateral pulmonary agenesis. Ann Surg. 213: 70-74. https://doi.org/10.1097/00000658-199101000-00012; PMid:1985541 PMCid:PMC1358313

30. Wright CD, Grillo HC, Wain JC et al. 2004. Anastomotic complications after tracheal resection: prognostic factors and management. J Thorac Cardiovasc Surg. 128: 731-739. https://doi.org/10.1016/j.jtcvs.2004.07.005; https://doi.org/10.1016/S0022-5223(04)00977-8

31. Yang JH, Jun TG, Sung K et al. 2007. Repair of long-segment congenital tracheal stenosis. Journal of Korean medical science. 22(3): 491-496. https://doi.org/10.3346/jkms.2007.22.3.491; PMid:17596659 PMCid:PMC2693643