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BackgroundCongenital tracheal stenosis (CTS) is a rare but life-threatening malformation of the trachea. Surgical reconstruction is the treatment of choice in symptomatic cases which is highly risky and is rarely performed in extremely premature infants. With this, reporting a case of CTS managed by tracheal reconstructive surgery under ECMO in a baby weighing 1.47 kg at 32 + 1 WOG was the first ever case in China.Case presentationA premature newborn with a very low birth weight (VLBW) was admitted to our institute for breathing difficulties, requiring mechanical ventilation, and experienced two unsuccessful attempts of extubation. The team performed tracheal reconstructive surgery supported by ECMO after identifying lower tracheal stenosis through a bronchoscopy examination. One month after the surgery, oxygen support was able to discontinue. The patient's entire hospitalization was incredibly challenging, marked by hemodynamic instability with persistent anemia, and disseminated intravascular coagulation (DIC), which were managed with great care. Despite the difficult stay, a follow-up bronchoscopy revealed no obstruction or tracheal stenosis, leading to a successful discharge.ConclusionAdvancements in diagnostic techniques and innovative management methods have made diagnosing and treating CTS easier, even in premature infants. Our case is the first in China to successfully undergo tracheal reconstructive surgery supported by ECMO, inspiring future achievements in the medical field.

Congenital tracheal stenosis can lead to symptomatic airway obstruction in children and often mandates surgical correction. Over the past half-century, numerous tracheal reconstruction techniques have been developed, including tracheal resection with end-to-end anastomosis (for short-segment complete tracheal stenosis), patch tracheoplasty, slide tracheoplasty, and homograft and autograft augmentation repairs. However, operative management of congenital tracheal stenosis is often complicated by the presence of congenital heart disease, the most common of which is pulmonary artery sling. When present concomitantly, combined repair of both defects is feasible and is currently the preferred approach. Questions have been raised about the optimal timing and sequence of surgery, and some have advocated staged repair for patients with complex associated cardiac lesions. However, evidence from the past two decades suggests that concomitant repair can be performed with excellent results. The current standard of care involves the use of cardiopulmonary bypass to simultaneously repair the tracheal defect using slide tracheoplasty and all associated cardiac anomalies. Advances in operative techniques and extracorporeal circulation, progressive understanding of the pathological basis of combined congenital tracheal and cardiac disease, and a multidisciplinary approach to patient care have all contributed to the successful outcomes seen in the modern era. This article describes the combined surgical correction of tracheal stenosis and double-outlet right ventricle-tetralogy of Fallot type in an infant, provides a detailed step-by-step description for performing a slide tracheoplasty along with various other less favored tracheoplasty techniques, and reviews the current literature discussing such combined repairs., Competing Interests: Conflicts of Interest: The series “Management of Congenital Heart Disease” was commissioned by the editorial office without any funding or sponsorship. RAM serves as the unpaid editorial board member of Journal of Thoracic Disease from Apr 2019 to Mar 2021 and the unpaid Guest Editor of the series. AS has no other conflicts of interest to declare., (2020 Journal of Thoracic Disease. All rights reserved.)

Background: Congenital tracheal stenosis (CTS) is a rare and life-threatening disease in children. Although pulmonary artery sling (PA sling) complicated by CTS sometimes occurs, there are few reports detailing the management of CTS with PA sling. The purpose of this retrospective study was to determine the appropriate indications for surgical intervention for CTS complicated by PA sling., Methods: We evaluated 42 patients (19 males and 23 females) with the median age of 9.9±3.3 months (range, 5-34 months) with CTS complicated by PA sling who were treated at our hospital between 2005 and 2018. Twenty-eight patients received both a slide tracheoplasty and PA re-implantation, and 14 patients were managed conservatively for CTS. Among the latter, nine patients received PA re-implantation only, and five were managed conservatively without any surgery. We determined the surgical indications by retrospectively comparing the DLR value [tracheal diameter (mm)/stenotic length ratio], history of ventilator respiration, mortality rate, and post-operative course of patients at a single institution., Results: The cut-off value for the DLR was determined to be 5.9 (sensitivity: 0.929, specificity: 0.714) by using the ROC curve (AUC 0.89, P<0.05)., Conclusions: A DLR value under 5.9 may serve as a new surgical indication for CTS complicated by PA sling., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare., (2019 Journal of Thoracic Disease. All rights reserved.)

Congenital tracheal stenosis (CTS) is rare lesions frequently associated with pulmonary sling (PS). Despite the recent improvement in the results by the introduction of slide tracheoplasty (ST) and multidisciplinary approach, surgical management remains difficult and several authors, for this reason, debate for a conservative approach in these cases. Preoperative planning, require hence the use of the most innovative technology to gain the best possible result. This report describes a complicated, unsuccessful surgically treated case of CTS associated to PS. Based on this result and in order to improve the outcomes, we have built a 3D model of patient's trachea simulating preoperative surgical planning. We have hypnotized that having the model preoperatively the choice of surgical technique would have been different. Since that case, 3D models are introduced in our practice and built before tracheal or cardiac cases considered to have complex anatomy. Future investigations are required but at the moment this experience confirms the utility of 3D model in the evaluation of children considered for tracheal surgery., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare.

Congenital tracheal stenosis (CTS) is a rare disorder almost always diagnosed in infancy due to respiratory failure and other cardiopulmonary abnormalities. We experienced a 42-year-old female undiagnosed with CTS until difficult intubation upon surgery. Chest X-ray and computed tomography (CT) images revealed bronchial narrowing, which could already be seen prior to intubation, but was left unnoticed. Difficult airway management is a potentially lethal airway emergency. This life-threatening situation is preventable with the appropriate awareness. We report this clinically valuable case for the safety of future patient care. In English and Japanese literature, there are only 12 reported cases of CTS diagnosed in the adult. Ours and six previous cases were discovered with difficult intubation, a preventable life-threatening airway emergency. Pre-intubation images should be examined carefully for the possibility of CTS, as its frequency may be underestimated. Moreover, in treatment resistant recurrent asthmatic episodes, CTS should be kept in mind., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare.