Management Strategies for Congenital Heart Disease Comorbid with Airway Anomalies in Children.

OBJECTIVE: To assess management strategies for pediatric patients with the challenging combination of congenital heart diseases (CHDs) and airway anomalies. STUDY DESIGN: Patients diagnosed with CHD and airway anomalies in the Pediatric Cardiac Surgery Centre of Fuwai Hospital from January 2016 to December 2020 were included in this retrospective study. Patients were divided into three groups based on different management, including the conservative group, the slide group (slide tracheoplasty), and the suspension group (suspension with external stenting). Patients' data and computed tomography measurements from medical records were reviewed. RESULTS: A total of 139 patients were included in the cohort; 107 had conservative airway treatment (conservative group), 15 had slide tracheoplasty (slide group), and 17 had tracheal suspension operation (suspension group). The top three associated intracardiac anomalies were ventricular septal defect (n = 34, 24%), pulmonary artery sling (n = 22, 16%), and tetralogy of Fallot (n = 15, 11%). Compared with patients with conservative airway management (100 minutes [median], 62-152 [IQR]), the extra airway procedure prolonged cardiopulmonary bypass duration, with 202 minutes (IQR, 119-220) for the slide group and 150 minutes (IQR, 125-161) for the suspension group. Patients who underwent slide tracheoplasty required prolonged mechanical ventilation (129 minutes [median], 56-328 [IQR]). Of the total cohort, 6 in-hospital deaths, all in the conservative group, and 8 mid-to long-term deaths, with 6 in the conservative group, occurred. CONCLUSIONS: Both conservative and surgical management of CHD patients with airway anomalies have promising outcomes. Extra tracheobronchial procedures, especially the slide tracheoplasty, significantly prolonged cardiopulmonary bypass duration. Based on multidisciplinary team assessment, individualized management strategies should be developed for these patients.

Long- term outcomes of congenital tracheal stenosis after slide tracheoplasty.

PURPOSE: Long-term outcomes of slide tracheoplasty in patients with congenital tracheal stenosis (CTS) have rarely been reported. This study aimed to clarify the long-term outcomes of CTS after slide tracheoplasty. METHODS: The medical records of 33 patients who underwent slide tracheoplasty for CTS at our institution between January 2005 and July 2018, with a follow-up duration > 5 years, were retrospectively reviewed. Patients' characteristics, perioperative condition, operative management, postoperative course, tracheal stenosis rates and growth data, were collected from medical records. RESULTS: The median operative age, minimum tracheal diameter, length of stenosis, duration of hospital stays, and follow-up duration were 8 months, 2.4 mm, 35 mm, 39 days, and 90 months, respectively. One patient died of bleeding in the right lung at 126 months postoperatively. Among the 10 patients requiring postoperative tracheostomy, seven were successfully decannulated at a median of 65 months postoperatively. Tracheal stenosis rates improved postoperatively and were subsequently maintained. Growth impairment and psychomotor delay were observed in 9 and 16 patients, respectively with significant differences found only in cases with genetic abnormalities and not in tracheal stenosis severity. CONCLUSION: Slide tracheoplasty for CTS leads to favorable long-term outcomes. However, various associated anomalies may influence growth and psychomotor development, emphasizing the importance of adequate support.

[Thoracoscopic tracheal resection in a child under extracorporeal membrane oxygenation]. / Torakoskopicheskaya rezektsiya trakhei u rebenka v usloviyakh ekstrakorporal'noi membrannoi oksigenatsii.

Congenital tracheal stenosis in children is a rare, severe and life-threatening respiratory tract malformation characterized by respiratory failure. We have performed 74 surgical interventions in these patients under extracorporeal membrane oxygenation between 2013 and 2022. In this article, we present surgical treatment of a newborn with congenital tracheal stenosis. For the first time in the world, the patient underwent thoracoscopic resection and reconstruction of the trachea under extracorporeal membrane oxygenation.

Incidence and risk factor of vocal cord paralysis following slide tracheoplasty for congenital tracheal stenosis: a retrospective observational study.

BACKGROUND: Slide tracheoplasty for congenital tracheal stenosis (CTS) has been shown to improve post-operative outcomes, but the incidence and risk factors of vocal cord paralysis (VCP) following slide tracheoplasty remain unclear. This study aimed to review our experience of slide tracheoplasty for CTS with a focus on post-operative VCP. METHODS: Twenty-eight patients, who underwent tracheal reconstruction with or without cardiovascular repair at Kobe Children's Hospital between June, 2016 and March, 2020 were enrolled in this retrospective observational study. They were divided into two groups based on the presence of a pulmonary artery sling (PA sling). Perioperative variables were compared between the two groups. RESULTS: Twenty-one of the 28 patients underwent concomitant repair for associated cardiovascular anomalies, including 15 patients with PA sling. The overall incidence of VCP following slide tracheoplasty was 28.6%. The incidences of VCP were 46.7% in patients with CTS and PA sling, which were 14.3% in CTS patients without cardiovascular anomalies. The only risk factor associated with VCP following slide tracheoplasty was a concomitant repair for PA sling. Post-operatively, the duration of nasogastric tube feeding in patients with VCP was significantly longer than that in patients without VCP. CONCLUSIONS: The incidence of VCP following slide tracheoplasty for CTS was high, especially in concomitant repair cases for PA sling. Routine screening and evaluation of VCP soon after post-operative extubation is required for its appropriate management.

Surgical management of congenital tracheal stenosis associated with complex cardiovascular anomalies.

PURPOSE: Although surgical outcomes have improved in patients with congenital tracheal stenosis (CTS), the management of such patients with complex cardiovascular anomalies (CVAs) remains a challenge. This study aimed to clarify the clinical features of this combination. METHODS: Medical records of 14 patients with complex CVAs who had undergone slide tracheoplasty for CTS between May 2016 and February 2022 were retrospectively reviewed. Complex CVAs were defined as CVAs without left pulmonary artery sling and simple cardiac shunts. Data collected included age and body weight at tracheal reconstruction, preoperative respiratory support, surgery details, and mortality. RESULTS: The median age and body weight at tracheal reconstruction were 3.5 (range, 1-17) months and 4.7 (range, 2.3-8.2) kg, respectively. Simultaneous repair of CTS and CVAs was performed in eight patients and staged repair in six patients. Patients who underwent simultaneous repair required preoperative respiratory support more frequently than those who underwent staged repair (8/8 [100%] vs 2/6 [33.3%]; P = 0.015). No mortality occurred. CONCLUSIONS: Patients with complex CVAs often require tracheal reconstruction in early infancy. Surgical management strategy for this combination was generally decided on the basis of the severity of respiratory symptoms due to CTS.

Successful surgical treatment of congenital tracheal stenosis combined with tracheal bronchus and left pulmonary artery sling: a 10-year single-institution experience.

PURPOSE: Radical surgery for congenital tracheal stenosis (CTS) is technically demanding. CTS combined with tracheal bronchus (TB) and pulmonary artery (PA) sling is a particularly challenging condition. We herein report our successfully modified surgical techniques for CTS combined with TB and PA sling. METHODS: Nine patients treated at our institution from July 2010 to December 2020 for CTS with TB and PA sling were enrolled. The patients' characteristics, operative results, and clinical outcomes were reviewed and analyzed retrospectively. RESULTS: The mean age at the operation and body weight were 8.0 ± 4.4 months old and 6.5 ± 0.8 kg, respectively. The mean tracheal diameter and length of the stenotic lesion were 3.2 ± 1.0 mm (mean stenosis rate 46.2%) and 25.4 ± 4.9 mm, respectively. All cases were complicated with PA sling at bifurcation stenosis with tracheobronchomalacia. All patients underwent modified posterior-anterior slide tracheoplasty with an inverted Y-shaped incision at the bifurcation and repositioning of the PA. The mean postoperative intubation period was 25.0 ± 32.1 days. There were no major intraoperative or postoperative complications, including hypoxic-ischemic encephalopathy. The mean hospital stay was 92.2 ± 73.4 days. All patients were discharged home without tracheostomy or oxygen support. CONCLUSION: Our slide tracheoplasty technique for CTS with TB and PA sling achieved excellent outcomes. LEVEL OF EVIDENCE: Level IV.

[Surgical tracheoplasty for children with congenital tracheal stenosis undergoing previous balloon dilatation or tracheal metal stent placement: a series of 9 cases].

Objective: To examine the outcomes of Slide tracheoplasty for the children with severe congenital tracheal stenosis received previous repeated balloon dilatation or metal stent placement under endoscopy. Methods: A retrospective study was conducted in 9 children with congenital tracheal stenosis undergoing previous interventional therapy under tracheoscopy and later received Slide tracheoplasty due to obvious respiratory symptoms at Department of Cardiac Surgery, Qilu Children's Hospital of Shandong University between February 2017 and July 2021. There were 7 males and 2 females with a median age at operation of 72.4 months (range: 13.3 to 98.9 months), and the median weight was 19.0 kg (range: 9.0 to 33.0 kg). Among the 9 patients, 2 patients began to receive repeated balloon dilatation (more than 3 times) 17.8 and 51.8 months ago respectively. One patient received metal stents placement into the trachea for 4 days and the other 6 children for median 56.8 months (range: 21.6 to 74.2 months). Complete tracheal cartilage rings and long segmental stenosis were present. in all 9 children. Operative details and outcome measures, including the need for endoscopic airway intervention and mortality, were collected. Results: Slide tracheoplasty was performed in all cases. Two patients with repeated balloon dilatation had different thickness of tracheal wall, local scar hyperplasia and irregular lumen. Among them, 1 case had obvious local calcification of tracheal wall, which was difficult to suture. The metal stent in one patient with short time of placement was completely removed. However, only part of the metal stents could be removed due to the long placement time in the other 6 cases. There was no operative death in the 9 children. The median postoperative tracheal intubation time was 25.3 hours (range: 17.4 to 74.5 hours). A silicone stent was placed in the trachea of 1 child due to obvious respiratory symptoms. Follow-up of median 11 months (range: 1 to 23 months) showed that no death occurred after discharge and all children had basically normal activity tolerance with no obvious respiratory symptoms. Conclusions: Slide tracheoplasty is feasible for children undergoing prior balloon dilatation or metal stents placement. Previously repeated balloon dilatation or metal stent placement under endoscopy increased the difficulty of slide tracheoplasty, the metal stent could not be completely removed after a long time.

Histological features of complete tracheal rings in congenital tracheal stenosis.

PURPOSE: Congenital tracheal stenosis is a disease in which complete tracheal cartilage rings (CTCR) cause airway narrowing. Although tracheal cartilage malformation has been suggested as a cause of CTCR, no histological studies have been performed. Here, we report a comparison of the tissues from CTCR and normal tracheal cartilage. METHODS: Thirty-one infants who underwent slide tracheoplasty at our institution from May 2016 to August 2019 were included. Tissues from ten autopsy cases without tracheal lesions were used as controls. The survey items were tracheal cartilage cell density, cartilage thickness, and chondrocyte findings. RESULTS: The median cartilage cell density from cases was 23/125 × 125 µm2 and from controls was 23.5/125 × 125 µm2 (p = 0.90). The median cartilage thickness from cases was 689 µm and from controls was 840 µm (p = 0.11). Comparing the ventral and dorsal sides of the CTCR tissues, the cell density was significantly different (median ventral 23/125 × 125 µm2; median dorsal 19.5/125 × 125 µm2; p = 0.034). There were no significant findings in the chondrocytes of the CTCR tissues. CONCLUSION: CTCR tissues did not differ in cartilage density and thickness from normal tracheal cartilage.

Non-operative management of congenital tracheal stenosis: criteria by computed tomography.

OBJECTIVES: Whether to perform surgical or conservative treatment for congenital tracheal stenosis (CTS) is controversial. Thus, the computed tomography (CT) criteria for conservative treatment of CTS were investigated. METHODS: From 2005 to 2017, 28 CTS cases were included. The operative cases and preoperative death cases constituted the required intervention group (group I), and the non-operative surviving cases constituted the observation group (group O). The diameter of the tracheal narrowest part (DTNP) on CT was evaluated as a criterion for non-operative follow-up. RESULTS: Chest CT was performed 19 times in 19 group I cases and 18 times in 9 group O cases. The median age of the patients that underwent CT scan examinations was 3.4 months (range 0-25 months) in group I and 22 months (range 0-60 months) in group O. The cut-off values of the non-operative criteria were 40.8% (AUC: 0.82, p < .01) normal for age of the trachea's narrowest part, and 41.6% normal for body weight (AUC: 0.92, p < .01), respectively. CONCLUSIONS: DTNP is 40% and more of the normal diameter appears necessary for non-surgical management. The present study suggests that the criteria for conservative management of CTS are that the DTNP is not less than 40% of the normal tracheal diameter, with a few symptoms.

[Congenital tracheal stenosis: from clinic to treatment.] / La sténose trachéale congénitale: de la clinique au traitement.

Congenital tracheal stenosis is a rare malformation of the fibrino-cartilaginous tracheal skeleton, frequently associated with cardiac malformations. The symptoms and the age of presentation varie according to the severity of the stenosis. There is a risk of airway obstruction. The diagnosis is based on endoscopy and surgery is usually required.

La sténose trachéale congénitale est une malformation rare du squelette fibro-cartilagineux de la trachée, fréquemment associée à des malformations cardiaques. Le tableau clinique et l'âge de présentation varient en fonction de la sévérité de la sténose. Il existe un risque d'obstruction des voies aériennes. Le diagnostic repose sur l'endoscopie et le traitement est chirurgical.

Temporary tracheal stenting using endovascular prosthesis in the management of severe refractory stenosis following slide tracheoplasty.

OBJECTIVES: To evaluate the role of endovascular stents in temporary tracheal stenting and endoscopic treatment strategy for patients with severe refractory tracheal stenosis following slide tracheoplasty. METHODS: Two patients had severe tracheal stenosis after slide tracheoplasty for long segment congenital tracheal stenosis. Both these patients were managed endoscopically and an endovascular prosthesis was used to temporarily stent the trachea. Short and long term results were evaluated clinically and with repeated endoscopies. RESULTS: The 2 patients had an excellent recovery immediately after the stent placement and continue to have an optimal, age-appropriate stable tracheal diameter after stent removal. CONCLUSION: Endovascular stents can be temporarily used to stent the trachea in the endoscopic treatment strategy to correct refractory severe tracheal stenosis following slide tracheoplasty.

Pitfalls in the management of congenital tracheal stenosis: is conservative management feasible?

PURPOSE: Congenital tracheal stenosis (CTS) is rare and challenging. Complete tracheal rings cause a wide spectrum of airway-obstructing lesions and varying degrees of respiratory distress. Although surgical reconstruction is the primary option for symptomatic CTS, sometimes an appropriate management strategy may be difficult due to other anomalies. We aimed to identify pitfalls in the management of CTS. METHODS: We retrospectively reviewed the records of patients with CTS during the last 10 years in our institution. RESULTS: Sixteen pediatric patients were diagnosed with CTS. Of the 16 patients, 12 (75.0%) had cardiovascular anomalies including seven left pulmonary artery sling. Six patients with dyspnoea caused by CTS and three patients with difficult intubations due to CTS underwent tracheoplasty. Four patients underwent only cardiovascular surgery without tracheoplasty. Three asymptomatic patients were followed up without undergoing any surgical procedure. We repeatedly discussed management of four patients with especially complex pathophysiology at multidisciplinary meetings. Right ventricular outflow tract obstruction, tracheobronchial malacia, increased pulmonary blood flow, and pulmonary aspiration due to gastroesophageal reflux presumably accounted for their severe respiratory distress, and we forewent their tracheal reconstruction. CONCLUSION: The management of CTS should be individualized, and conservative management is a feasible option in selected cases.

Congenital tracheal malformations.

Congenital malformations of the trachea include a variety of conditions that cause respiratory distress in neonates and infants. A number of anomalies are self-limiting while others are life-threatening and require immediate therapy. The prevalence of congenital airway malformations has been estimated to range between 0.2 and 1 in 10,000 live births. The most frequent congenital tracheal malformations are: tracheomalacia, congenital tracheal stenosis, laryngotracheal cleft and tracheal agenesis. The management of congenital tracheal malformations is complex and requires an individualized approach delivered by a multidiscipilinary team within centralized units with the necessary expertise.

Early and late outcomes after surgical management of congenital vascular rings.

Persistent respiratory or feeding problems in children may be associated with a congenital vascular ring. Surgical management is fairly standardized, but long-term outcomes are not well described. This study aims to investigate clinical presentation, surgical treatment, and risk factors for early mortality and late outcome. Our database revealed 62 surgically treated vascular ring patients between 1993 and 2014. Double aortic arch was the most common diagnosis (53%). Median age at operation was 1 year. Symptoms were mainly respiratory (89%) and feeding problems (32%). Median extubation time and hospital stay were 4 h and 5 days. Mean follow-up was 7.8 ± 5.8 years. Early mortality was 8% and was related to anatomical diagnosis, concomitant anomalies, and a need for preoperative intubation. Freedom from residual symptoms at 1 and 6 months was 63 and 82%, respectively. Freedom from inhalation therapy at the last follow-up was 82% and was influenced by a type of vascular ring and preoperative ventilation. Dysphagia symptoms always disappeared. CONCLUSION: Surgical relief of tracheoesophageal compression is commonly effective in vascular ring anomalies. Respiratory symptoms necessitating chronic inhalation therapy only persist in a minority of children. Patients with double aortic arch are at increased risk to remain symptomatic, particularly with infectious exacerbations.

Management of congenital tracheal stenosis in the neonatal period.

PURPOSE: Few reports have focused on the management of congenital tracheal stenosis (CTS) in the neonatal period. The aim of this study was to determine appropriate management strategies for CTS in the neonatal period. METHODS: The medical records of eight neonatal patients with CTS at a single institution between January 2007 and December 2016 were retrospectively reviewed. RESULTS: Three patients with frequent ventilatory insufficiency despite assisted ventilation underwent surgical intervention (balloon tracheoplasty: n = 1, slide tracheoplasty: n = 2). Ventilatory insufficiency improved after surgery in all three patients. One patient who underwent slide tracheoplasty died due to non-airway-related causes. Observation or conservative management was performed in five patients with minimal respiratory symptoms or stable ventilation under assisted ventilation. All five patients were safely managed non-operatively in the neonatal period. CONCLUSION: Depending on the severity of ventilatory insufficiency, there are two management strategies for CTS in the neonatal period. Surgical intervention, such as balloon tracheoplasty or slide tracheoplasty, is indicated for patients with unstable ventilatory status despite assisted ventilation. Observation or conservative management is a more suitable option for neonates with stable ventilation.

Rare Upper Airway Anomalies.

A broad spectrum of congenital upper airway anomalies can occur as a result of errors during embryologic development. In this review, we will describe the clinical presentation, diagnosis, and management strategies for a few select, rare congenital malformations of this system. The diagnostic tools used in workup of these disorders range from prenatal tests to radiological imaging, swallowing evaluations, indirect or direct laryngoscopy, and rigid bronchoscopy. While these congenital defects can occur in isolation, they are often associated with disorders of other organ systems or may present as part of a syndrome. Therefore workup and treatment planning for patients with these disorders often involves a team of multiple specialists, including paediatricians, otolaryngologists, pulmonologists, speech pathologists, gastroenterologists, and geneticists.

Surgical intervention strategies for congenital tracheal stenosis associated with a tracheal bronchus based on the location of stenosis.

PURPOSE: The aim of this study was to determine the appropriate surgical intervention strategies for congenital tracheal stenosis (CTS) associated with a tracheal bronchus based on the location of stenosis. METHODS: The medical records of 13 pediatric patients with CTS associated with a tracheal bronchus at a single institution between January 2006 and December 2015 were retrospectively reviewed. RESULTS: Type 1: tracheal stenosis above the right upper lobe bronchus (RULB) (n = 1). One patient underwent slide tracheoplasty and was successfully extubated. Type 2: tracheal stenosis below the RULB (n = 7). Tracheal end-to-end anastomosis was performed before 2014, and one patient failed to extubate. Posterior-anterior slide tracheoplasty was performed since 2014, and all three patients were successfully extubated. Type 3: tracheal stenosis above the RULB to the carina (n = 5). One patient underwent posterior-anterior slide tracheoplasty and was successfully extubated. Two patients with left-right slide tracheoplasty and another two patients with tracheal end-to-end anastomosis for the stenosis below the RULB could not be extubated. CONCLUSION: Tracheal end-to-end anastomosis or slide tracheoplasty can be selected for tracheal stenosis above the RULB according to the length of stenosis. Posterior-anterior slide tracheoplasty appears feasible for tracheal stenosis below the RULB or above the RULB to the carina.