Congenital tracheal stenosis is a rare disease that benefits from no established surgical procedure due to insufficient experience and a lack of large-scale research.1 If patients develop symptoms early in infancy, surgical repair can be a considerable challenge because the size of the airway is so small that it can be easily obstructed by post-operative oedema.2 If the length of stenotic segment is short, segmental resection and end-to-end anastomosis can be performed, with high success rates.3–6 However, for cases involving the long segment of the trachea, it has been suggested that resection of more than 30% of the tracheal length may lead to excessive anastomotic tension followed by recurrent stenosis or fatal separation.6,7 Long-segment tracheal stenosis is often associated with other anomalies such as pulmonary vascular sling, intracardiac lesion and right-sided aortic arch, which do not occur with with short-segment stenosis.8–10 For these reasons, the management of long-segment tracheal stenosis in infancy still reports significant morbidity and mortality rates.11,12 Various surgical techniques have been suggested for the surgical treatment of this disorder, including rib-cartilage tracheoplasty, peri-cardial patch tracheoplasty, tracheal autograft, and slide tracheoplasty.2,6,11,13–18 Recently, successful results have been reported in several studies.10,11,14,17,19 Nonetheless, there still remains much debate as to the appropriate surgical procedure due to the rarity of the disease and the lack of long-term follow-up data.2,14,17
The limitation on the trachea length that can be resected safely necessitates the introduction of various patch materials for tracheal augmentation. For the patch materials, costal cartilage13 and autologous pericardium have been reported.15 Jaquiss and associates showed favourable results for rib-cartilage tracheoplasty with a low rate of post-operative problems and no operative mortality.14 Backer and associates described an 83% survival rate using peri-cardial patch tracheoplasty.19 Due to its rigidity, cartilage graft cannot offer an airtight suture line. In contrast, peri-cardial patch has an advantage due to its pliability, which allows for an airtight suture line.14
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- Maeda M, Grillo HC, Effect of tension on tracheal growth after resection and anastomosis in puppies, J Thorac Cardiovasc Surg, 1973;65:658–68.
- Berdon WE, Baker DH, Wung JT, et al., Complete cartilage-ring tracheal stenosis associated with anomalous left pulmonary artery: the ring-sling complex, Radiology, 1984;152:57–64.
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- Chiu PP, Kim PC, Prognostic factors in the surgical treatment of congenital tracheal stenosis: a multicentre analysis of the literature, J Pediatr Surg, 2006;41:221–5.
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- Idriss FS, DeLeon SY, Ilbawi MN, et al., Tracheoplasty with pericardial patch for extensive tracheal stenosis in infants and children, J Thorac Cardiovasc Surg, 1984;88:527–36.
- Cosentino CM, Backer CL, Idriss FS, et al., Pericardial patch tracheoplasty for severe tracheal stenosis in children: intermediate results, J Pediatr Surg, 1991;26:879–84.
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- Yamaguchi M, Oshima Y, et al., Concomitant repair of congenital tracheal stenosis and complex cardiac anomaly in small children, J Thorac Cardiovasc Surg, 1990;100:181–7.
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- Tsugawa C, Kimura K, Muraji T, et al., Congenital stenosis involving a long segment of the trachea: further experience in reconstructive surgery, J Pediatr Surg, 1988;23:471–5.
- Ito H, et al., Bilateral independent high-frequency jet ventilation for intra-operative airway management of repair of congenital tracheal stenosis, Anaesth Intensive Care, 2006;34:683–4.
- 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;41:1203–7.