Combined Surgical and Endoscopic Approach for Ring–Sling Complex

Abstract

Background Left pulmonary artery (LPA) sling (PAS) is a vascular ring, which is frequently associated with long-segment tracheal stenosis (TS). Mortality rate in operated children is still high, especially in cases of severe tracheal hypoplasia and/or associated congenital heart defects (CHDs). We report our experience of treatment and follow-up in a pediatric cohort of patients affected by PAS with severe tracheobronchial involvement.

Methods From 2005 to 2017, we enrolled 11 children diagnosed with PAS and congenital TS requiring surgical intervention. Echocardiography, computed tomography, and bronchoscopy were performed in all patients. Associated CHD were present in 5 (45%) patients. Tracheal reconstruction techniques included slide tracheoplasty (7/11; 63%), slide tracheoplasty and costal cartilage graft (2/11; 18%), and Hazekamp technique (2/11; 18%).

Nine patients underwent LPA direct reimplantation and concomitant tracheoplasty; concomitant surgical repair for CHD was performed in three children.

Results Over a mean follow-up of 30 months (range: 3–75 months), a late mortality of 18% was registered; no early death occurred. Good flow through LPA could be documented in all patients. Ten children required operative bronchoscopies (mean: 16/patients) aimed at stent positioning/removal, treatment of granulomas, and tracheobronchial dilatation.

Conclusions Severe tracheobronchial stenosis and associated CHD were the main determinants for hospitalization time, intensive assistance, and repeated endoscopic procedures.

Patients affected by PAS/TS complex require a careful management at high-specialized centers providing multidisciplinary team.

Respiratory endoscopy may play a central role both in preoperatory assessment and in postoperative management of patients showing severe tracheobronchial involvement.

Keywords

congenital heart disease - CHD - pulmonary arteries - trachea

• References

• 1 Backer CL, Russell HM, Kaushal S, Rastatter JC, Rigsby CK, Holinger LD. Pulmonary artery sling: current results with cardiopulmonary bypass. J Thorac Cardiovasc Surg 2012; 143 (01) 144-151
• 2 Xie J, Juan YH, Wang Q. , et al. Evaluation of left pulmonary artery sling, associated cardiovascular anomalies, and surgical outcomes using cardiovascular computed tomography angiography. Sci Rep 2017; 7: 40042
• 3 Chao YC, Peng CC, Lee KS, Lin SM, Chen MR. The association of congenital tracheobronchial stenosis and cardiovascular anomalies. Int J Pediatr Otorhinolaryngol 2016; 83: 1-6
• 4 Fiore AC, Brown JW, Weber TR, Turrentine MW. Surgical treatment of pulmonary artery sling and tracheal stenosis. Ann Thorac Surg 2005; 79 (01) 38-46 , discussion 38–46
• 5 Burden RJ, Shann F, Butt W, Ditchfield M. Tracheobronchial malacia and stenosis in children in intensive care: bronchograms help to predict outcome. Thorax 1999; 54 (06) 511-517
• 6 Tsang V, Murday A, Gillbe C, Goldstraw P. Slide tracheoplasty for congenital funnel-shaped tracheal stenosis. Ann Thorac Surg 1989; 48 (05) 632-635
• 7 Beierlein W, Elliott MJ. Variations in the technique of slide tracheoplasty to repair complex forms of long-segment congenital tracheal stenoses. Ann Thorac Surg 2006; 82 (04) 1540-1542
• 8 Hazekamp MG, Koolbergen DR, Kersten J, Peper J, de Mol B, König-Jung A. Pediatric tracheal reconstruction with pericardial patch and strips of autologous cartilage. Eur J Cardiothorac Surg 2009; 36 (02) 344-351 , discussion 351
• 9 Hong X, Zhou G, Liu Y, Liu Y, Wang H, Feng Z. Management of pulmonary artery sling with tracheal stenosis: LPA re-implantation without tracheoplasty. Int J Clin Exp Med 2015; 8 (02) 2741-2747
• 10 Chiu PP, Kim PC. Prognostic factors in the surgical treatment of congenital tracheal stenosis: a multicenter analysis of the literature. J Pediatr Surg 2006; 41 (01) 221-225 , discussion 221–225
• 11 Li X, Cheng LC, Cheung YF, Lun KS, Chau KT, Chiu SW. Management of symptomatic congenital tracheal stenosis in neonates and infants by slide tracheoplasty: a 7-year single institution experience. Eur J Cardiothorac Surg 2010; 38 (05) 609-614
• 12 Hewitt RJ, Butler CR, Maughan EF, Elliott MJ. Congenital tracheobronchial stenosis. Semin Pediatr Surg 2016; 25 (03) 144-149
• 13 Arcieri L, Pak V, Poli V. , et al. Tracheal surgery in children: outcome of a 12-year survey. Interact Cardiovasc Thorac Surg 2018; 26 (04) 660-666
• 14 Manning PB, Rutter MJ, Lisec A, Gupta R, Marino BS. One slide fits all: the versatility of slide tracheoplasty with cardiopulmonary bypass support for airway reconstruction in children. J Thorac Cardiovasc Surg 2011; 141 (01) 155-161
• 15 Kocyildirim E, Kanani M, Roebuck D. , et al. Long-segment tracheal stenosis: slide tracheoplasty and a multidisciplinary approach improve outcomes and reduce costs. J Thorac Cardiovasc Surg 2004; 128 (06) 876-882
• 16 Oshima Y, Yamaguchi M, Yoshimura N. , et al. Management of pulmonary artery sling associated with tracheal stenosis. Ann Thorac Surg 2008; 86 (04) 1334-1338
• 17 Yong MS, d'Udekem Y, Brizard CP. , et al. Surgical management of pulmonary artery sling in children. J Thorac Cardiovasc Surg 2013; 145 (04) 1033-1039
• 18 Elliott M, Hartley BE, Wallis C, Roebuck D. Slide tracheoplasty. Curr Opin Otolaryngol Head Neck Surg 2008; 16 (01) 75-82
• 19 Butler CR, Speggiorin S, Rijnberg FM. , et al. Outcomes of slide tracheoplasty in 101 children: a 17-year single-center experience. J Thorac Cardiovasc Surg 2014; 147 (06) 1783-1789
• 20 Hofferberth SC, Watters K, Rahbar R, Fynn-Thompson F. Evolution of surgical approaches in the management of congenital tracheal stenosis: single-center experience. World J Pediatr Congenit Heart Surg 2016; 7 (01) 16-24
• 21 Antón-Pacheco JL. Tracheobronchial stents in children. Semin Pediatr Surg 2016; 25 (03) 179-185
• 22 Serio P, Fainardi V, Leone R. , et al. Tracheobronchial obstruction: follow-up study of 100 children treated with airway stenting. Eur J Cardiothorac Surg 2014; 45 (04) e100-e109
• 23 Cheng W, Manson DE, Forte V. , et al. The role of conservative management in congenital tracheal stenosis: an evidence-based long-term follow-up study. J Pediatr Surg 2006; 41 (07) 1203-1207
• 24 Kwak JG, Kim WH, Min J, Lee C, Jang W, Lee CH. Is tracheoplasty necessary for all patients with pulmonary artery sling and tracheal stenosis?. Pediatr Cardiol 2013; 34 (03) 498-503
• 25 Arcieri L, Giordano R, Murzi B. To do or not to do? The management dilemma of congenital tracheal stenosis in the setting of the ring-sling complex. J Thorac Dis 2017; 9 (12) 4896-4898
• 26 Brouns M, Jayaraju ST, Lacor C. , et al. Tracheal stenosis: a flow dynamics study. J Appl Physiol (1985) 2007; 102 (03) 1178-1184